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Sample records for failure neonatal hemochromatosis

  1. Neonatal hemochromatosis and patent ductus venosus: clinical course and diagnostic pitfalls

    International Nuclear Information System (INIS)

    Tsai, Andy; Paltiel, Harriet J.; Sena, Laureen M.; Kim, Heung Bae; Fishman, Steven J.; Alomari, Ahmad I.

    2009-01-01

    Neonatal hemochromatosis is a rare metabolic disorder characterized by excessive iron deposition within the liver leading to hepatic failure and portal hypertension. We describe the clinical course and imaging findings in three infants with neonatal hemochromatosis associated with patent ductus venosus. We paid special attention to the diagnostic challenges encountered in these patients in order to emphasize some of the potential diagnostic pitfalls. We conducted a comprehensive search of our radiology database of the last 10 years (1999-2008) for the keywords ''neonatal hemochromatosis.'' Medical records and imaging studies of various modalities were reviewed. Three neonates were found to have neonatal hemochromatosis; all of them were associated with patent ductus venosus. Two of these patients were referred to our tertiary center for embolization of an inaccurately diagnosed hepatic vascular malformation. Two patients underwent successful liver transplantation and one died shortly after referral. The awareness and inclusion of neonatal hemochromatosis in the differential diagnosis of newborns with liver failure and patent ductus venosus has critical treatment implications. (orig.)

  2. Neonatal hemochromatosis and patent ductus venosus: clinical course and diagnostic pitfalls

    Energy Technology Data Exchange (ETDEWEB)

    Tsai, Andy; Paltiel, Harriet J.; Sena, Laureen M. [Children' s Hospital Boston and Harvard Medical School, Department of Radiology, Boston, MA (United States); Kim, Heung Bae; Fishman, Steven J. [Children' s Hospital Boston and Harvard Medical School, Department of Surgery, Boston, MA (United States); Alomari, Ahmad I. [Children' s Hospital Boston and Harvard Medical School, Department of Radiology, Boston, MA (United States); Children' s Hospital Boston, Division of Vascular and Interventional Radiology, Boston, MA (United States)

    2009-08-15

    Neonatal hemochromatosis is a rare metabolic disorder characterized by excessive iron deposition within the liver leading to hepatic failure and portal hypertension. We describe the clinical course and imaging findings in three infants with neonatal hemochromatosis associated with patent ductus venosus. We paid special attention to the diagnostic challenges encountered in these patients in order to emphasize some of the potential diagnostic pitfalls. We conducted a comprehensive search of our radiology database of the last 10 years (1999-2008) for the keywords ''neonatal hemochromatosis.'' Medical records and imaging studies of various modalities were reviewed. Three neonates were found to have neonatal hemochromatosis; all of them were associated with patent ductus venosus. Two of these patients were referred to our tertiary center for embolization of an inaccurately diagnosed hepatic vascular malformation. Two patients underwent successful liver transplantation and one died shortly after referral. The awareness and inclusion of neonatal hemochromatosis in the differential diagnosis of newborns with liver failure and patent ductus venosus has critical treatment implications. (orig.)

  3. Neonatal hemochromatosis. Case series from Bahrain.

    Science.gov (United States)

    Isa, Hasan M; Mohamed, Afaf M

    2013-12-01

    To review clinical presentations, diagnosis, response to treatment, and outcome of infants with neonatal hemochromatosis (NH). This is a retrospective review of all cases admitted to the Pediatric Department at Salmaniya Medical Center, Manama, Bahrain between March 2008 and May 2011. The diagnosis was based on serum iron and ferritin, alpha-fetoprotein levels (AFP), liver and buccal biopsies, and abdominal MRI scan. Ten patients (8 males and 2 females) were diagnosed with NH. Two patients were intrauterine growth restriction (IUGR) and 6 were preterm. The median birth weight was 1.700 grams. The median age at presentation was 16 days, and at diagnosis was 23 days. Two patients had positive consanguinity. Clinical presentations of the infants were hepatosplenomegaly (n=5), ascites (n=3), and hypoglycemia (n=6). All patients had raised ferritin levels, prolonged prothrombin time, and 9 patients had high serum iron and serum AFP. Abdominal MRI showed iron overload in the liver (n=8). Liver biopsies showed evidence of hemochromatosis (n=3). Buccal biopsies stained positive for iron (n=1). Eight patients received antioxidant therapy and survived. Two patients passed away. Neonatal hemochromatosis is a rare liver disease of newborns with a spectrum of clinical severity. Elevated serum ferritin and AFP support the diagnosis after excluding other causes of neonatal liver failure. The use of antioxidant therapy helps to improve the outcome.

  4. Hemochromatosis

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    Onur Albayrak

    2009-08-01

    Full Text Available Hemochromatosis is the most common form of iron overload disease. Iron builds up in the body’s organs and damages them. Hemochromatosis is presented with arthropathy, cirrhosis of the liver, melanoderma, heart failure, diabetes mellitus and other endocrine deficiencies. There are two type of this disease. Primary hemochromatosis is known as an inherited disease. Herediter hemochromatosis is caused by the mutations of HFE gene. The most common mutations are H63D (Histidine- Aspartat and C282Y (Cysteine-Tyrosine on the gene. Secondary hemochromatosis is caused by anemia, alcoholism, and some of the other disorders. [Archives Medical Review Journal 2009; 18(4.000: 268-275

  5. Hereditary hemochromatosis (HFE) genotypes in heart failure: relation to etiology and prognosis

    DEFF Research Database (Denmark)

    Møller, Daniel Vega; Pecini, Redi; Gustafsson, Finn

    2010-01-01

    It is believed that hereditary hemochromatosis (HH) might play a role in cardiac disease (heart failure (HF) and ischemia). Mutations within several genes are HH-associated, the most common being the HFE gene. In a large cohort of HF patients, we sought to determine the etiological role...... and the prognostic significance of HFE genotypes....

  6. Molecular basis of HFE-hemochromatosis

    OpenAIRE

    Vujić, Maja

    2014-01-01

    Iron-overload disorders owing to genetic misregulation of iron acquisition are referred to as hereditary hemochromatosis (HH). The most prevalent genetic iron overload disorder in Caucasians is caused by mutations in the HFE gene, an atypical MHC class I molecule. Recent studies classified HFE/Hfe-hemochromatosis as a liver disease with the primarily failure in the production of the liver iron hormone hepcidin in hepatocytes. Inadequate hepcidin expression signals for excessive iron absorptio...

  7. Failure to thrive among neonates, associated factors and early neonatal outcome

    International Nuclear Information System (INIS)

    Thomas, Erica; Manji, Karim; Mpembeni Rose

    2005-01-01

    Failure to thrive or growth failure is an important feature of problems prevalent in the neonate. It remains one of the greatest challenges for the practicing pediatrician and it is a common pathway or outcome of several different underlaying infant and maternal conditions. To determine the prevalence, possible causes and early neonatal outcome of failure to thrive among young infants admitted to the Neonatal Unit in this hospital. A cross-sectional descriptive hospital based study, was carried for 10 months from April 2001 to January 2002 at the Neonatal Unit at Muhimbili National Hospital. (author)

  8. Hemochromatosis

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now Hemochromatosis Back Hemochromatosis affects ...

  9. Molecular basis of HFE-hemochromatosis

    Directory of Open Access Journals (Sweden)

    Maja eVujic Spasic

    2014-03-01

    Full Text Available Iron-overload disorders owing to genetic misregulation of iron acquisition are referred to as hereditary hemochromatosis (HH. The most prevalent genetic iron overload disorder in Caucasians is caused by mutations in the HFE gene, an atypical MHC class I molecule. Recent studies classified HFE/Hfe-hemochromatosis as a liver disease with the primarily failure in the production of the liver iron hormone hepcidin in hepatocytes. Inadequate hepcidin expression signals for excessive iron absorption from the diet and iron deposition in tissues causing multiple organ damage and failure. This review focuses on the molecular actions of the HFE/Hfe and hepcidin in maintaining systemic iron homeostasis and approaches undertaken so far to combat iron overload in HFE/Hfe-HH. In the light of the recent investigations, novel roles of extra-hepatocytic Hfe are discussed raising a question to the relevance of the multipurpose functions of Hfe for the understanding of HH associated pathologies.

  10. Hereditary Hemochromatosis (HFE genotypes in heart failure: Relation to etiology and prognosis

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    Torp-Pedersen Christian

    2010-07-01

    Full Text Available Abstract Background It is believed that hereditary hemochromatosis (HH might play a role in cardiac disease (heart failure (HF and ischemia. Mutations within several genes are HH-associated, the most common being the HFE gene. In a large cohort of HF patients, we sought to determine the etiological role and the prognostic significance of HFE genotypes. Methods We studied 667 HF patients (72.7% men with depressed systolic function, enrolled in a multicentre trial with a follow-up period of up to 5 years. All were genotyped for the known HFE variants C282Y, H63D and S65C. Results The genotype and allele frequencies in the HF group were similar to the frequencies determined in the general Danish population. In multivariable analysis mortality was not predicted by C282Y-carrier status (HR 1.2, 95% CI: 0.8-1.7; H63D-carrier status (HR 1.0, 95% CI: 0.7-1.3; nor S65C-carrier status (HR 1.2, 95% CI: 0.7-2.0. We identified 27 (4.1% homozygous or compound heterozygous carriers of HFE variants. None of these carriers had a clinical presentation suggesting hemochromatosis, but hemoglobin and ferritin levels were higher than in the rest of the cohort. Furthermore, a trend towards reduced mortality was seen in this group in univariate analyses (HR 0.4, 95% CI: 0.2-0.9, p = 0.03, but not in multivariate (HR 0.5, 95% CI: 0.2-1.2. Conclusion HFE genotypes do not seem to be a significant contributor to the etiology of heart failure in Denmark. HFE variants do not affect mortality in HF.

  11. Musculoskeletal disease burden of hereditary hemochromatosis.

    Science.gov (United States)

    Sahinbegovic, Enijad; Dallos, Tomáš; Aigner, Elmar; Axmann, Roland; Manger, Bernhard; Englbrecht, Matthias; Schöniger-Hekele, Maximilian; Karonitsch, Thomas; Stamm, Tanja; Farkas, Martin; Karger, Thomas; Stölzel, Ulrich; Keysser, Gernot; Datz, Christian; Schett, Georg; Zwerina, Jochen

    2010-12-01

    To determine the prevalence, clinical picture, and disease burden of arthritis in patients with hereditary hemochromatosis. In this cross-sectional observational study of 199 patients with hemochromatosis and iron overload, demographic and disease-specific variables, genotype, and organ involvement were recorded. The prevalence, intensity, and localization of joint pain were assessed, and a complete rheumatologic investigation was performed. Radiographs of the hands, knees, and ankles were scored for joint space narrowing, erosions, osteophytes, and chondrocalcinosis. In addition, the number and type of joint replacement surgeries were recorded. Joint pain was reported by 72.4% of the patients. Their mean ± SD age at the time of the initial joint symptoms was 45.8 ± 13.2 years. If joint pain was present, it preceded the diagnosis of hemochromatosis by a mean ± SD of 9.0 ± 10.7 years. Bony enlargement was observed in 65.8% of the patients, whereas synovitis was less common (13.6%). Joint space narrowing and osteophytes as well as chondrocalcinosis of the wrist and knee joints were frequent radiographic features of hemochromatosis. Joint replacement surgery was common, with 32 patients (16.1%) undergoing total joint replacement surgery due to severe OA. The mean ± SD age of these patients was 58.3 ± 10.4 years at time of joint replacement surgery. Female sex, metacarpophalangeal joint involvement, and the presence of chondrocalcinosis were associated with a higher risk of early joint failure (i.e., the need for joint replacement surgery). Arthritis is a frequent, early, and severe symptom of hemochromatosis. Disease is not confined to involvement of the metacarpophalangeal joints and often leads to severe damage requiring the replacement of joints. Copyright © 2010 by the American College of Rheumatology.

  12. Acute renal failure: Nephrosonographic findings in asphyxiated neonates

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    Mohd. Ashraf

    2011-01-01

    Full Text Available To determine the incidence of acute renal failure (ARF and nephrosonographic findings among asphyxiated neonates, and to correlate this with uric acid levels and the severity of hypoxic encephalopathy, we studied 80 full-term appropriate-for-date singleton neonates with perinatal asphyxia, and 30 healthy full-term neonates as controls from March 2006 to February 2007. A detailed history, thorough clinical examination along with investigations, including urine examination, 24-h urine collection, ultrasonography of abdomen and cranium, serum electrolytes, blood urea nitrogen, serum creatinine, and serum uric acid were obtained. ARF developed in 45% (36/80 of the asphyxiated neonates. Forty-eight (60% neonates showed significant elevation of blood urea and 41 (51.3% neonates had significant elevation of serum creatinine than the control group (P < 0.001. Sixty-two (77.5% neonates developed significant elevation of serum uric acid levels, and nephrosonography revealed hyperechogenicity in all of them, while only two among the healthy neonates showed the raised uric acid levels (P < 0.001. Nonoliguric renal failure was seen 28/36 (77.8% of the neonates with ARF, whereas eight (22.2% neonates had oliguric renal failure. Eight (27.8% patients among ARF patients maintained abnormal biochemical parameters after 2 weeks, and of whom four patients died after variable lengths of time with a mortality rate of 11.11%. Kidneys are the most common organs involved in perinatal asphyxia, and uric acid might be a causative factor for failure in addition to hypoxic insult. Routine use of kidney function test, along with abdominal ultrasonography form an important screening tool to detect any additional morbidity in these patients.

  13. Genetics Home Reference: hereditary hemochromatosis

    Science.gov (United States)

    ... Type 1 hemochromatosis results from mutations in the HFE gene, and type 2 hemochromatosis results from mutations in ... about the genes associated with hereditary hemochromatosis HAMP HFE HJV PNPLA3 SLC40A1 TFR2 Related Information What is a gene? What is a gene mutation and how do ...

  14. Maternal drugs and neonatal renal failure

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    M Sahay

    2014-01-01

    Full Text Available Maternal use of drugs during pregnancy may cause irreversible renal failure in the newborn. This report highlights the adverse effect of telmisartan during the last trimester of pregnancy. The neonate presented with oliguric renal failure and the renal histology showed proximal tubular dysgenesis.

  15. Panhypopituitarism Due to Hemochromatosis

    OpenAIRE

    Mesut Özkaya; Kadir Gis; Ali Çetinkaya

    2013-01-01

    Hemochromatosis is an iron storage disease. Panhypopituitarism is a clinical condition in which the anterior pituitary hormones are deficient. Herein, we report a rare case of panhypopituitarism due to hemochromatosis. Turk Jem 2013; 17: 125-6

  16. Diagnosis and management of hereditary hemochromatosis.

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    Salgia, Reena J; Brown, Kimberly

    2015-02-01

    Hereditary hemochromatosis is a rare genetic disorder that can have significant clinical consequences. Hemochromatosis is associated with iron overload, and can initially be recognized through laboratory testing for serum ferritin and transferrin saturation. Genetic testing for the HFE mutation can be performed in patients with elevated iron indices and a suspicion for hemochromatosis or liver disease. The main pathway resulting in iron overload is through altered hepcidin levels. Treatment of patients with the clinical phenotype of hereditary hemochromatosis is commonly through phlebotomy for removal of excess iron stores. This article highlights the current information and data regarding the diagnosis and management of hemochromatosis. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Neonate With Severe Heart Failure Related to Vein of Galen Malformation

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    Meng-Yu Chen

    2010-08-01

    Full Text Available We report a full-term female neonate who presented with respiratory distress and severe heart failure soon after birth. Heart failure secondary to perinatal infection was initially suspected. Subsequent echocardiography revealed aortic runoff, which led to consideration of an intracranial vascular abnormality. Ultrasound and magnetic resonance imaging of the brain confirmed a diagnosis of vein of Galen aneurismal malformation (VGAM. Endovascular coil embolization of the vascular anomaly was performed, resulting in improvement of heart failure. VGAM should be considered in the differential diagnosis of neonatal congestive heart failure with a structurally normal heart. Urgent endovascular embolization and aggressive medical treatment of heart failure improve prognosis in neonatal VGAM.

  18. Monocyte transferrin-iron uptake in hereditary hemochromatosis

    International Nuclear Information System (INIS)

    Sizemore, D.J.; Bassett, M.L.

    1984-01-01

    Transferrin-iron uptake by peripheral blood monocytes was studied in vitro to test the hypothesis that the relative paucity of mononuclear phagocyte iron loading in hereditary hemochromatosis results from a defect in uptake of iron from transferrin. Monocytes from nine control subjects and 17 patients with hemochromatosis were cultured in the presence of 59Fe-labelled human transferrin. There was no difference in 59Fe uptake between monocytes from control subjects and monocytes from patients with hemochromatosis who had been treated by phlebotomy and who had normal body iron stores. However, 59Fe uptake by monocytes from iron-loaded patients with hemochromatosis was significantly reduced compared with either control subjects or treated hemochromatosis patients. It is likely that this was a secondary effect of iron loading since iron uptake by monocytes from treated hemochromatosis patients was normal. Assuming that monocytes in culture reflect mononuclear phagocyte iron metabolism in vivo, this study suggests that the relative paucity of mononuclear phagocyte iron loading in hemochromatosis is not related to an abnormality in transferrin-iron uptake by these cells

  19. Maternal and neonatal outcomes of respiratory failure during pregnancy

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    Chen-Yiu Hung

    2018-05-01

    Full Text Available Background: Obstetric patients comprise a limited portion of intensive care unit patients, but they often present with unfamiliar conditions and exhibit the potential for catastrophic deterioration. This study evaluated the maternal and neonatal outcomes of respiratory failure during pregnancy. Methods: Information on 71 patients at >25 weeks gestation in the ICU with respiratory failure was recorded between 2009 and 2013. The characteristics and outcomes of mothers and fetuses were determined through a retrospective chart review and evaluated using Student's t test, chi-square test, and Fisher's exact test. Results: The leading causes of respiratory failure were postpartum hemorrhage and severe preeclampsia in the obstetric causes group and pneumonia in the nonobstetric causes group during pregnancy and the peripartum period. The non-obstetric causes group exhibited a higher incidence of acute respiratory distress syndrome and renal replacement therapy as well as requiring more ventilator days. The patients in the obstetric causes group showed significant improvement after delivery in the partial pressure of arterial oxygen to the fraction of inspired oxygen and peak inspiratory pressure decrease. Both groups exhibited high incidences of neonatal respiratory distress syndrome. Neonatal complications resulting from meconium aspiration syndrome (MAS and sepsis were more common in the non-obstetric causes group; however, neurological development impairment was more common in the obstetric causes group. Conclusion: Obstetric cause was associated with longer ventilator free days and fewer episodes of ARDS after delivery. Neonatal complications resulting from different etiologies of respiratory failure were found to differ. Keywords: Acute respiratory distress syndrome, Neonatal, Obstetric, Outcome, Respiratory failure

  20. Hemochromatosis Patients as Voluntary Blood Donors

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    Tara E Power

    2004-01-01

    Full Text Available The present study was designed to investigate hemochromatosis patients' suitability as blood donors as well as their perceptions and experience with the current public donation system. Participants were gathered from a list of current hemochromatosis patients (n=120 and members of the Canadian Hemochromatosis Society (n=1000. Of the 1120 surveys mailed out to these groups, 801 surveys were returned completed. The sample respondents had a mean age of 57.44 years (SD=12.73; range 19 to 87 years, and 57% were men. It was found that 20% (160 of the respondents have donated blood since their diagnosis; however, only 12% of the respondents indicated that they use voluntary blood donation as a means of maintaining their iron levels. Forty per cent of the respondents indicated that they had been refused from voluntary donation. Despite the fact that in May 2001 the Canadian Blood Services, in collaboration with the Canadian Hemochromatosis Society, began a promotion campaign to encourage hemochromatosis patients to become voluntary blood donors, the present study found that 15% of the respondents reported having been refused from the voluntary blood donation service due to the diagnosis of hemochromatosis. With respect to quality of life, it was found that individuals who donate blood were generally healthier with respect to physical functioning and bodily pain, however, these findings may indicate that hemochromatosis patients who are healthier are better able to donate at public blood banks, rather than that voluntary blood donation has an effect on the donors' physical functioning over phlebotomy clinic users. These study findings suggest that although there may be other medical factors limiting individuals from donating, hemochromatosis patients are interested in being voluntary blood donors and this potential resource is currently under-used.

  1. Hemochromatosis

    Science.gov (United States)

    ... medical center. Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you. Screening Family Members for Hemochromatosis Parents, grandparents, ...

  2. [Heart failure as early manifestation of neonatal hyperthyroidism. Case report].

    Science.gov (United States)

    Alvarado S, Jorge Luis; Fernández V, Zhirly Andrea

    2014-04-01

    Neonatal hyperthyroidism is usually a self-limited condition frequently associated with transplacental passage of thyroid stimulating antibodies secondary to maternal autoimmune disorders. To timely detect mothers with this medical antecedents decreases the risk for fetal adverse events. To report a case of neonatal hyperthyroidism associated with intrauterine growth restriction and heart failure. A 36 week-old newborn with birth weight of 1,240 g. Symptoms were tachycardia, distal coldness, exophthalmos, hepatomegaly and tremors. Echocardiogram ruled out structural heart disorders. Due to maternal symptoms suggestive of hyperthyroidism, TSH tests were performed showing 0.01 ulU/ml, free T4 7.7 ng/dl, so the diagnosis of neonatal hyperthyroidism was confirmed. It was treated with methimazole and propanol, alleviating the symptoms and decreasing the levels of free T4. To know the maternal history helps identify and manage neonatal complications of hyperthyroidism. Heart failure and other cardiopulmonary disorders are determinants of mortality during early neonatal period. High-risk newborns should receive follow up assessments.

  3. [Molecular genetic analysis and clinical aspects of patients with hereditary hemochromatosis].

    Science.gov (United States)

    Lange, U; Teichmann, J; Dischereit, G

    2014-08-01

    The purpose of the study was to perform a molecular genetic analysis and to document clinical aspects in patients with hereditary hemochromatosis. The study included 33 outpatients (23 males average age 50.6 years and 10 females average age 60.6 years) with a disorder of iron metabolism (transferrin saturation > 75 %) as confirmation of hemochromatosis who were subjected to molecular genetic and clinical analyses. A homozygous mutation of the hemochromatosis (HFE) gene (C282YY) was detected in 63.6 %, a compound heterozygous mutation (C282Y/H63D) in 30.3% and no mutation of the HFE gene was detected in 6.1 %. The following organ manifestations could be objectified: arthralgia (78.8 %), liver disease (39.9 %), skin hyperpigmentation (30.3 %), osteoporosis (24.2 %), diabetes mellitus (24.2 %) and cardiomyopathy (12.1 %). Comparison between patients with heterozygous and homozygous hemochromatosis revealed the following differences: compound heterozygote patients presented less frequently with osteoarthritis of the metacarpophalangeal (MCP) joints and hands (85.7 %/71.4 % homozygotes vs. 60 %/60 % heterozygotes). Osteoarthritis of the shoulder joints and osteoporosis as well as hypothyroidism were more frequent in compound heterozygote patients, whereas osteoarthritis of the knee and hip joints as well as liver disease were more common in homozygote patients. No differences between both groups were seen with respect to the clinical manifestations of cardiomyopathy and diabetes mellitus. Prevalent causes of death in hereditary hemochromatosis are heart failure, liver disease (cirrhosis and hepatocellular carcinoma) and portal hypertension. Therefore, an early diagnosis, adequate therapy and genetic screening of family members are of great importance. Medicinal treatment will only effectively prevent deleterious organ involvement and subsequent complications if initiated at an early stage. Furthermore, an overview of the current data is given.

  4. Acute renal failure in asphyxiated term neonates

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    Pejović Biljana

    2002-01-01

    Full Text Available INTRODUCTION Acute renal failure (ARF is a frequent clinical condition in neonatal intensive care units (NICU. The leading cause of neonatal ARF is perinatal asphyxia (PS. The aim of this study was to examine the relationship between the degree of PS and the severity of ARF in term neonates. METHODS A prospective survey of 31 term neonates with Ps and but without congenital malformations or sepsis was performed in NICU of the regional Hospital of Gynaecology and Obstetrics in Belgrade (average number of deliveries about 6000 per year. ARF was diagnosed in the first 7 days of life when plasma creatinine was above 133 μmοΙ/L for at least 48 hours while maternal renal function was normal. The degree of PS was determined according to Apgar score (AS at 1 min. The severe PS was defined as AS < 3 and moderate PS as AS 4-6. RESULTS Twenty neonates (64% had oliguric ARF with urine output of 0.37 ±0.16 ml/kg/h while the others had nonoliguric ARF with urine output of 2.4 ± 0.7 ml/kg/h. Most of neonates with oliguric ARF (65% had severe perinatal asphuxia while in those with nonoliguric ARF moderate perinatal asphyxia predominated (73%. DISCUSSION During hypoxic-ischaemic events many organs are injured, and the most vulnerable ones are kidneys and central nervous system. Our results showed a strong connection between perinatal asphyxia and A, which was in accordance with the results of other studies. Neonates with severe perinatal asphyxia had serious impairment of renal function, which was confirmed with strong correlation between Apgar score and plasma creatinine. In neonates with oliguric ARF, but not in those with nonoliguric ARF, the highly positive linear correlations were found between AS and urinary output (r = 0.77; p < 0.01, plasma creatinine (r = 0.78; p < 0.01, fractional excretion of sodium (r = 0.76; p < 0.01, and index of renal failure (r = 0.80; p < 0.01. Only in oliguric neonates with severe perinatal asphyxia (31 % the outcome was

  5. Maternal and neonatal outcomes of respiratory failure during pregnancy.

    Science.gov (United States)

    Hung, Chen-Yiu; Hu, Han-Chung; Chiu, Li-Chung; Chang, Chih-Hao; Li, Li-Fu; Huang, Chung-Chi; Kao, Chuan-Chi; Cheng, Po-Jen; Kao, Kuo-Chin

    2018-05-01

    Obstetric patients comprise a limited portion of intensive care unit patients, but they often present with unfamiliar conditions and exhibit the potential for catastrophic deterioration. This study evaluated the maternal and neonatal outcomes of respiratory failure during pregnancy. Information on 71 patients at >25 weeks gestation in the ICU with respiratory failure was recorded between 2009 and 2013. The characteristics and outcomes of mothers and fetuses were determined through a retrospective chart review and evaluated using Student's t test, chi-square test, and Fisher's exact test. The leading causes of respiratory failure were postpartum hemorrhage and severe preeclampsia in the obstetric causes group and pneumonia in the nonobstetric causes group during pregnancy and the peripartum period. The non-obstetric causes group exhibited a higher incidence of acute respiratory distress syndrome and renal replacement therapy as well as requiring more ventilator days. The patients in the obstetric causes group showed significant improvement after delivery in the partial pressure of arterial oxygen to the fraction of inspired oxygen and peak inspiratory pressure decrease. Both groups exhibited high incidences of neonatal respiratory distress syndrome. Neonatal complications resulting from meconium aspiration syndrome (MAS) and sepsis were more common in the non-obstetric causes group; however, neurological development impairment was more common in the obstetric causes group. Obstetric cause was associated with longer ventilator free days and fewer episodes of ARDS after delivery. Neonatal complications resulting from different etiologies of respiratory failure were found to differ. Copyright © 2017. Published by Elsevier B.V.

  6. Hereditary Hemochromatosis

    Science.gov (United States)

    ... hemochromatosis occurs when a child inherits a mutated HFE gene from his or her parents. This mutated gene ... a special test to look for an abnormal HFE gene. This test would tell their doctor if they ...

  7. Molecular basis of HFE-hemochromatosis.

    Science.gov (United States)

    Vujić, Maja

    2014-01-01

    Iron-overload disorders owing to genetic misregulation of iron acquisition are referred to as hereditary hemochromatosis (HH). The most prevalent genetic iron overload disorder in Caucasians is caused by mutations in the HFE gene, an atypical MHC class I molecule. Recent studies classified HFE/Hfe-HH as a liver disease with the primarily failure in the production of the liver iron hormone hepcidin in hepatocytes. Inadequate hepcidin expression signals for excessive iron absorption from the diet and iron deposition in tissues causing multiple organ damage and failure. This review focuses on the molecular actions of the HFE/Hfe and hepcidin in maintaining systemic iron homeostasis and approaches undertaken so far to combat iron overload in HFE/Hfe-HH. In the light of the recent investigations, novel roles of extra-hepatocytic Hfe are discussed raising a question to the relevance of the multipurpose functions of Hfe for the understanding of HH-associated pathologies.

  8. An unfortunate case of acquired hemochromatosis: a case report review of the clinical presentation, diagnosis, management, and prognosis

    Directory of Open Access Journals (Sweden)

    Tariq A

    2016-12-01

    Full Text Available Anam Tariq,1 Kevin Westra,2 Arben Santo3 1Department of Internal Medicine, Pinnacle Health Internal Medicine, 2Department of Gastroenterology, Harrisburg Gastroenterology, Harrisburg, PA, 3Department of Pathology, Virginia College of Osteopathic Medicine-Virginia Tech, Blacksburg, VA, USA Background: While blood transfusions are commonly used for prophylaxis and treatment for acute chest syndromes and strokes in sickle cell patients, accumulation of excess iron resulting in secondary hemochromatosis remains a rare disease. Chelation is the mainstay for preventing and treating iron overload to deter potential end-organ damages; it is rare when therapy fails. Case report: A 52-year-old African American woman with chronic anemia secondary to sickle cell anemia and history of multiple blood transfusions presented with elevated serum ferritin (8000 ng/mL and bilirubin (16.8 mg/dL. She had no previous personal or family history of liver disease. A magnetic resonance cholangiopancreatography (MRCP and a liver biopsy confirmed the secondary hemochromatosis with marked fibrosis and 4+ iron deposits, but since she was therapeutically on deferasirox, her treatment regimen involved only closer monitoring. Her hemochromatosis led to readmission within a year for rapid progression of cardiac and hepatic failure. Conclusion: Since chronically transfused sickle cell patients are at a significantly higher risk of mortality due to the secondary hemochromatosis and end-stage organ damage, knowledge of prophylactic iron chelation is important. Minimizing unnecessary transfusions should be strongly emphasized to reduce the sequelae as iron burden remains a threat. The effectiveness of iron-chelating therapy is best monitored via periodic magnetic resonance imaging, liver transaminases, bilirubin, creatinine, ferritin, and cardiac function tests. Despite the prophylactic treatment and quarterly blood work, in this case the initial presentation did not correlate with

  9. Scrub typhus causing neonatal hepatitis with acute liver failure-A case series.

    Science.gov (United States)

    Vajpayee, Shailja; Gupta, R K; Gupta, M L

    2017-05-01

    Neonatal hepatitis with acute liver failure due to varied etiology including various infections is reported in the past. Scrub typhus as a cause of neonatal hepatitis has rarely been reported in literature. A high index of clinical suspicion is required for early diagnosis and timely treatment. Severity and prognosis of the disease varies widely because several different strains of Orientia tsutsugamushi exist with different virulence. Delayed diagnosis can result in complication and significant morbidity and mortality. Here, we report three cases of neonatal hepatitis with acute liver failure caused by scrub typhus to increase awareness.

  10. Non-coding keratin variants associate with liver fibrosis progression in patients with hemochromatosis.

    Directory of Open Access Journals (Sweden)

    Pavel Strnad

    Full Text Available BACKGROUND: Keratins 8 and 18 (K8/K18 are intermediate filament proteins that protect the liver from various forms of injury. Exonic K8/K18 variants associate with adverse outcome in acute liver failure and with liver fibrosis progression in patients with chronic hepatitis C infection or primary biliary cirrhosis. Given the association of K8/K18 variants with end-stage liver disease and progression in several chronic liver disorders, we studied the importance of keratin variants in patients with hemochromatosis. METHODS: The entire K8/K18 exonic regions were analyzed in 162 hemochromatosis patients carrying homozygous C282Y HFE (hemochromatosis gene mutations. 234 liver-healthy subjects were used as controls. Exonic regions were PCR-amplified and analyzed using denaturing high-performance liquid chromatography and DNA sequencing. Previously-generated transgenic mice overexpressing K8 G62C were studied for their susceptibility to iron overload. Susceptibility to iron toxicity of primary hepatocytes that express K8 wild-type and G62C was also assessed. RESULTS: We identified amino-acid-altering keratin heterozygous variants in 10 of 162 hemochromatosis patients (6.2% and non-coding heterozygous variants in 6 additional patients (3.7%. Two novel K8 variants (Q169E/R275W were found. K8 R341H was the most common amino-acid altering variant (4 patients, and exclusively associated with an intronic KRT8 IVS7+10delC deletion. Intronic, but not amino-acid-altering variants associated with the development of liver fibrosis. In mice, or ex vivo, the K8 G62C variant did not affect iron-accumulation in response to iron-rich diet or the extent of iron-induced hepatocellular injury. CONCLUSION: In patients with hemochromatosis, intronic but not exonic K8/K18 variants associate with liver fibrosis development.

  11. Heart Failure

    Science.gov (United States)

    ... Other diseases. Chronic diseases — such as diabetes, HIV, hyperthyroidism, hypothyroidism, or a buildup of iron (hemochromatosis) or ... transplantation or support with a ventricular assist device. Prevention The key to preventing heart failure is to ...

  12. Hereditary hemochromatosis: genetic complexity and new diagnostic approaches.

    NARCIS (Netherlands)

    Swinkels, D.W.; Janssen, M.C.H.; Bergmans, J.; Marx, J.J.M.

    2006-01-01

    Since the discovery of the hemochromatosis gene (HFE) in 1996, several novel gene defects have been detected, explaining the mechanism and diversity of iron-overload diseases. At least 4 main types of hereditary hemochromatosis (HH) have been identified. Surprisingly, genes involved in HH encode for

  13. Learning about Hereditary Hemochromatosis

    Science.gov (United States)

    Skip to main content Learning About Hereditary Hemochromatosis Enter Search Term(s): Español Research Funding An Overview Bioinformatics Current Grants Education and Training Funding Extramural Research ...

  14. Evaluation of a workplace hemochromatosis screening program.

    Science.gov (United States)

    Stave, G M; Mignogna, J J; Powell, G S; Hunt, C M

    1999-05-01

    Hemochromatosis is a common inherited disorder of iron metabolism with significant health consequences for the employed population. Although screening for hemochromatosis has been recommended, workplace screening programs remain uncommon. In the first year of a newly initiated corporate screening program, 1968 employees were tested. The screening algorithm included measurement of serum iron and transferrin and subsequent ferritin levels in those employees with elevated iron/transferrin ratios. Thirteen percent of men and 21% of women had elevated iron/transferrin ratios. Of these, 14 men and 2 women had elevated ferritin levels. Of these 16, three had liver biopsies and all three have hemochromatosis. The cost of the screening program was $27,850. The cost per diagnosis was $9283 and the cost per year of life saved was $928. These costs compare very favorably with other common workplace screening programs. Several barriers to obtaining definitive diagnoses on all patients with a positive screening result were identified; strategies to overcome these barriers would further enhance the cost effectiveness of the program. We conclude that workplace hemochromatosis screening is highly cost effective and should be incorporated into health promotion/disease prevention programs.

  15. Hereditary hemochromatosis: An opportunity for gene therapy

    Directory of Open Access Journals (Sweden)

    FERNANDO EZQUER

    2006-01-01

    Full Text Available Levels of body iron should be tightly controlled to prevent the formation of oxygen radicals, lipoperoxidation, genotoxicity, and the production of cytotoxic cytokines, which result in damage to a number of organs. Enterocytes in the intestinal villae are involved in the apical uptake of iron from the intestinal lumen; iron is further exported from the cells into the circulation. The apical divalent metal transporter-1 (DMT1 transports ferrous iron from the lumen into the cells, while the basolateral transporter ferroportin extrudes iron from the enterocytes into the circulation. Patients with hereditary hemochromatosis display an accelerated transepithelial uptake of iron, which leads to body iron accumulation that results in cirrhosis, hepatocellular carcinoma, pancreatitis, and cardiomyopathy. Hereditary hemochromatosis, a recessive genetic condition, is the most prevalent genetic disease in Caucasians, with a prevalence of one in 300 subjects. The majority of patients with hereditary hemochromatosis display mutations in the gene coding for HFE, a protein that normally acts as an inhibitor of transepithelial iron transport. We discuss the different control points in the homeostasis of iron and the different mutations that exist in patients with hereditary hemochromatosis. These control sites may be influenced by gene therapeutic approaches; one general therapy for hemochromatosis of different etiologies is the inhibition of DMT1 synthesis by antisense-generating genes, which has been shown to markedly inhibit apical iron uptake by intestinal epithelial cells. We further discuss the most promising strategies to develop gene vectors and deliver them into enterocytes

  16. Rare HFE variants are the most frequent cause of hemochromatosis in non-c282y homozygous patients with hemochromatosis.

    Science.gov (United States)

    Hamdi-Rozé, Houda; Beaumont-Epinette, Marie-Pascale; Ben Ali, Zeineb; Le Lan, Caroline; Loustaud-Ratti, Véronique; Causse, Xavier; Loreal, Olivier; Deugnier, Yves; Brissot, Pierre; Jouanolle, Anne-Marie; Bardou-Jacquet, Edouard

    2016-12-01

    p.Cys282Tyr (C282Y) homozygosity explains most cases of HFE-related hemochromatosis, but a significant number of patients presenting with typical type I hemochromatosis phenotype remain unexplained. We sought to describe the clinical relevance of rare HFE variants in non-C282Y homozygotes. Patients referred for hemochromatosis to the National Reference Centre for Rare Iron Overload Diseases from 2004 to 2010 were studied. Sequencing was performed for coding region and intronic flanking sequences of HFE, HAMP, HFE2, TFR2, and SLC40A1. Nine private HFE variants were identified in 13 of 206 unrelated patients. Among those, five have not been previously described: p.Leu270Argfs*4, p.Ala271Valfs*25, p.Tyr52*, p.Lys166Asn, and p.Asp141Tyr. Our results show that rare HFE variants are identified more frequently than variants in the other genes associated with iron overload. Rare HFE variants are therefore the most frequent cause of hemochromatosis in non-C282Y homozygote HFE patients. Am. J. Hematol. 91:1202-1205, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  17. Severe acute haemorrhagic liver failure in a neonate with a favourable spontaneous outcome

    International Nuclear Information System (INIS)

    Cavet, Madeleine; Balu, Marie; Garel, Catherine; Ducou le Pointe, Hubert; Mitanchez, Delphine; Alexandre, Marie; Renolleau, Sylvain; Pariente, Daniele

    2008-01-01

    Acute liver failure in neonates is rare and is frequently associated with an unfavourable outcome. There is no curative treatment other than liver transplantation. Screening for viral, metabolic, toxic or vascular disease is essential to assess the prognosis and to guide specific treatment. Hepatic haemorrhage in neonates is often associated with bacterial infection, trauma and coagulopathies. We present a unique case of neonatal acute liver failure and multifocal massive haemorrhagic intrahepatic lesions of traumatic origin, documented by US and MRI. The patient made a spontaneous recovery. Clinical, biological and imaging outcome was excellent despite the apparent severity of the initial features. The only possible aetiology was a difficult caesarean delivery for mild fetal macrosomia. (orig.)

  18. Severe acute haemorrhagic liver failure in a neonate with a favourable spontaneous outcome

    Energy Technology Data Exchange (ETDEWEB)

    Cavet, Madeleine; Balu, Marie; Garel, Catherine; Ducou le Pointe, Hubert [Universite Pierre et Marie Curie Paris VI, Service de Radiologie, Hopital d' enfants Armand-Trousseau, Paris (France); Mitanchez, Delphine; Alexandre, Marie [Universite Pierre et Marie Curie Paris VI, Service de Neonatologie, Hopital d' enfants Armand-Trousseau, Paris (France); Renolleau, Sylvain [Universite Pierre et Marie Curie Paris VI, Service de Reanimation, Hopital d' enfants Armand-Trousseau, Paris (France); Pariente, Daniele [Hopital de Bicetre, Service de Radiologie Pediatrique, Paris (France)

    2008-10-15

    Acute liver failure in neonates is rare and is frequently associated with an unfavourable outcome. There is no curative treatment other than liver transplantation. Screening for viral, metabolic, toxic or vascular disease is essential to assess the prognosis and to guide specific treatment. Hepatic haemorrhage in neonates is often associated with bacterial infection, trauma and coagulopathies. We present a unique case of neonatal acute liver failure and multifocal massive haemorrhagic intrahepatic lesions of traumatic origin, documented by US and MRI. The patient made a spontaneous recovery. Clinical, biological and imaging outcome was excellent despite the apparent severity of the initial features. The only possible aetiology was a difficult caesarean delivery for mild fetal macrosomia. (orig.)

  19. Hemochromatosis mutations in the general population

    DEFF Research Database (Denmark)

    Andersen, Rolf Vaern; Tybjaerg-Hansen, Anne; Appleyard, Merete

    2004-01-01

    The progression rate of iron overload in hereditary hemochromatosis in individuals in the general population is unknown. We therefore examined in the general population iron overload progression rate in C282Y homozygotes. Using a cohort study of the Danish general population, The Copenhagen City...... saturation and ferritin levels increased slightly in male and female C282Y homozygotes. None of the C282Y homozygotes developed clinically overt hemochromatosis. In conclusion, individuals in the general population with C282Y homozygosity at most demonstrate modest increases in transferrin saturation...

  20. Preliminary investigation of bottlenose dolphins (Tursiops truncatus) for hfe gene-related hemochromatosis.

    Science.gov (United States)

    Phillips, Brianne E; Venn-Watson, Stephanie; Archer, Linda L; Nollens, Hendrik H; Wellehan, James F X

    2014-10-01

    Hemochromatosis (iron storage disease) has been reported in diverse mammals including bottlenose dolphins (Tursiops truncatus). The primary cause of excessive iron storage in humans is hereditary hemochromatosis. Most human hereditary hemochromatosis cases (up to 90%) are caused by a point mutation in the hfe gene, resulting in a C282Y substitution leading to iron accumulation. To evaluate the possibility of a hereditary hemochromatosis-like genetic predisposition in dolphins, we sequenced the bottlenose dolphin hfe gene, using reverse transcriptase-PCR and hfe primers designed from the dolphin genome, from liver of affected and healthy control dolphins. Sample size included two case animals and five control animals. Although isotype diversity was evident, no coding differences were identified in the hfe gene between any of the animals examined. Because our sample size was small, we cannot exclude the possibility that hemochromatosis in dolphins is due to a coding mutation in the hfe gene. Other potential causes of hemochromatosis, including mutations in different genes, diet, primary liver disease, and insulin resistance, should be evaluated.

  1. Hemochromatosis and diabetes mellitus: case report and literature review

    Directory of Open Access Journals (Sweden)

    Karina Biavatti

    2008-12-01

    Full Text Available Hemochromatosis is a disorder characterized by iron storage amended. The acquired form of the disease can be caused by iron overload, alcoholism, infection by  C virus hepatitis, non-alcoholic hepatitis and chronic liver disease. The hereditary form can be caused by different mutations, being the C282Y and H63D the most frequent, 83% of cases are homozigotous for C282Y and 4% are compound heterozygous (C282Y/H63D. Hemochromatosis is a condition that can affect several organs, including: heart, joints, liver, hypothalamus, pituitary, pancreas and gonads. The aim of this study was to report a case of hemochromatosis and review the literature, with special attention to the association of hemochromatosis and diabetes mellitus. Patient 53 years, male presented to the doctor with arthralgia metacarpophalangeal, ankles, knees, coxofemoral right, and cervical and lumbar, complaints of fatigue and weight loss. Between 3 brothers, one of them had a diagnosis of hereditary hemochromatosis, with PCR demonstrating homozygous for C282Y. Labs: GOT 128 U/L, ALT 231 U/L, alkaline phosphatase 258 U/L, abdominal ultrasound with hepatomegaly and spleen at the upper limit of normal. Liver biopsy demonstrated portal fibrosis extension with hemosiderosis intense. It also made the diagnosis of diabetes mellitus. The research confirmed the same mutation of the changing family: homozygous for C282Y

  2. Prediction of extubation failure for neonates with respiratory distress syndrome using the MIMIC-II Clinical Database

    NARCIS (Netherlands)

    Mikhno, A.; Ennett, C.M.

    2012-01-01

    Extubation failure (EF) is an ongoing problem in the neonatal intensive care unit (NICU). Nearly 25% of neonates fail their first extubation attempt, requiring re-intubations that are associated with riskfactors and financial costs. We identified 179 mechanically ventilated neonatal patients that

  3. Acute liver failure in a term neonate after repeated paracetamol administration

    OpenAIRE

    Bucaretchi, Fabio; Fernandes, Carla Borrasca; Branco, Maira Migliari; Capitani, Eduardo Mello De; Hyslop, Stephen; Caldas, Jamil Pedro S.; Moreno, Carolina Araujo; Porta, Gilda

    2014-01-01

    Objective: Severe hepatotoxicity caused by paracetamol is rare in neonates. We report a case of paracetamol-induced acute liver failure in a term neonate. Case description: A 26-day-old boy was admitted with intestinal bleeding, shock signs, slight liver enlargement, coagulopathy, metabolic acidosis (pH=7.21; bicarbonate: 7.1mEq/L), hypoglycemia (18mg/dL), increased serum aminotransferase activity (AST=4,039IU/L; ALT=1,087IU/L) and hyperbilirubinemia (total: 9.57mg/dL; direct: 6.18mg/dL)...

  4. Vein of Galen Aneurysmal Malformation in Neonates Presenting With Congestive Heart Failure

    Directory of Open Access Journals (Sweden)

    Andjenie Madhuban MD

    2016-03-01

    Full Text Available The authors report the case of a neonate presenting with signs of a congenital cardiac disease. Echocardiography showed a structural normal heart, right-to-left ductal flow, a dilated superior caval vein, and reversed diastolic flow in the proximal descending aorta. Brain magnetic resonance imaging showed a vein of Galen arteriovenous malformation. This highlights the importance of considering an intracranial cause in the differential diagnosis of neonatal congestive heart failure.

  5. Prognostic factors in neonatal acute renal failure

    International Nuclear Information System (INIS)

    Chevalier, R.L.; Campbell, F.; Brenbridge, A.N.

    1984-01-01

    Sixteen infants, 2 to 35 days of age, had acute renal failure, a diagnosis based on serum creatinine concentrations greater than 1.5 mg/dL for at least 24 hours. Eight infants were oliguric (urine flow less than 1.0 mL/kg/h) whereas the remainder were nonoliguric. To determine clinical parameters useful in prognosis, urine flow rate, duration of anuria, peak serum creatinine, urea (BUN) concentration, and nuclide uptake by scintigraphy were correlated with recovery. Nine infants had acute renal failure secondary to perinatal asphyxia, three had acute renal failure as a result of congenital cardiovascular disease, and four had major renal anomalies. Four oliguric patients died: three of renal failure and one of heart failure. All nonoliguric infants survived with mean follow-up serum creatinine concentration of 0.8 +/- 0.5 (SD) mg/dL whereas that of oliguric survivors was 0.6 +/- 0.3 mg/dL. Peak serum creatinine concentration did not differ between those patients who were dying and those recovering. All infants who were dying remained anuric at least four days and revealed no renal uptake of nuclide. Eleven survivors were anuric three days or less, and renal perfusion was detectable by scintigraphy in each case. However, the remaining survivor (with bilateral renal vein thrombosis) recovered after 15 days of anuria despite nonvisualization of kidneys by scintigraphy. In neonates with ischemic acute renal failure, lack of oliguria and the presence of identifiable renal uptake of nuclide suggest a favorable prognosis

  6. Prognostic factors in neonatal acute renal failure

    Energy Technology Data Exchange (ETDEWEB)

    Chevalier, R.L.; Campbell, F.; Brenbridge, A.N.

    1984-08-01

    Sixteen infants, 2 to 35 days of age, had acute renal failure, a diagnosis based on serum creatinine concentrations greater than 1.5 mg/dL for at least 24 hours. Eight infants were oliguric (urine flow less than 1.0 mL/kg/h) whereas the remainder were nonoliguric. To determine clinical parameters useful in prognosis, urine flow rate, duration of anuria, peak serum creatinine, urea (BUN) concentration, and nuclide uptake by scintigraphy were correlated with recovery. Nine infants had acute renal failure secondary to perinatal asphyxia, three had acute renal failure as a result of congenital cardiovascular disease, and four had major renal anomalies. Four oliguric patients died: three of renal failure and one of heart failure. All nonoliguric infants survived with mean follow-up serum creatinine concentration of 0.8 +/- 0.5 (SD) mg/dL whereas that of oliguric survivors was 0.6 +/- 0.3 mg/dL. Peak serum creatinine concentration did not differ between those patients who were dying and those recovering. All infants who were dying remained anuric at least four days and revealed no renal uptake of nuclide. Eleven survivors were anuric three days or less, and renal perfusion was detectable by scintigraphy in each case. However, the remaining survivor (with bilateral renal vein thrombosis) recovered after 15 days of anuria despite nonvisualization of kidneys by scintigraphy. In neonates with ischemic acute renal failure, lack of oliguria and the presence of identifiable renal uptake of nuclide suggest a favorable prognosis.

  7. Predicting Factors of INSURE Failure in Low Birth Weight Neonates with RDS; A Logistic Regression Model

    Directory of Open Access Journals (Sweden)

    Bita Najafian

    2015-02-01

    Full Text Available Background:Respiratory Distress syndrome is the most common respiratory disease in premature neonate and the most important cause of death among them. We aimed to investigate factors to predict successful or failure of INSURE method as a therapeutic method of RDS.Methods:In a cohort study,45 neonates with diagnosed RDS and birth weight lower than 1500g were included and they underwent INSURE followed by NCPAP(Nasal Continuous Positive Airway Pressure. The patients were divided into failure or successful groups and factors which can predict success of INSURE were investigated by logistic regression in SPSS 16th version.Results:29 and16 neonates were observed in successful and failure groups, respectively. Birth weight was the only variable with significant difference between two groups (P=0.002. Finally logistic regression test showed that birth weight is only predicting factor for success (P: 0.001, EXP[β]: 0.009, CI [95%]: 1.003-0.014 and mortality (P: 0.029, EXP[β]: 0.993, CI [95%]: 0.987-0.999 of neonates treated with INSURE method.Conclusion:Predicting factors which affect on success rate of INSURE can be useful for treating and reducing charge of neonate with RDS and the birth weight is one of the effective factor on INSURE Success in this study.

  8. Predicting Factors of INSURE Failure in Low Birth Weight Neonates with RDS; A Logistic Regression Model

    Directory of Open Access Journals (Sweden)

    Bita Najafian

    2015-02-01

    Full Text Available Background:Respiratory Distress syndrome is the most common respiratory disease in premature neonate and the most important cause of death among them. We aimed to investigate factors to predict successful or failure of INSURE method as a therapeutic method of RDS. Methods:In a cohort study,45 neonates with diagnosed RDS and birth weight lower than 1500g were included and they underwent INSURE followed by NCPAP(Nasal Continuous Positive Airway Pressure. The patients were divided into failure or successful groups and factors which can predict success of INSURE were investigated by logistic regression in SPSS 16th version. Results:29 and16 neonates were observed in successful and failure groups, respectively. Birth weight was the only variable with significant difference between two groups (P=0.002. Finally logistic regression test showed that birth weight is only predicting factor for success (P: 0.001, EXP[β]: 0.009, CI [95%]: 1.003-0.014 and mortality (P: 0.029, EXP[β]: 0.993, CI [95%]: 0.987-0.999 of neonates treated with INSURE method. Conclusion:Predicting factors which affect on success rate of INSURE can be useful for treating and reducing charge of neonate with RDS and the birth weight is one of the effective factor on INSURE Success in this study.

  9. Hereditary hemochromatosis

    Directory of Open Access Journals (Sweden)

    Stephen A. Geller

    2015-03-01

    Full Text Available Hereditary hemochromatosis (HH is the most commonly identified autosomal recessive genetic disorder in the white population, characterized by increased intestinal iron absorption and secondary abnormal accumulation in parenchymal organs, not infrequently accompanied by functional impairment. This entity is associated with mutations of the HFE gene (located on the short arm of chromosome 6 at location 6p22.2; closely linked to the HLA-A3 locus, which encodes the HFE protein, a membrane protein thought to regulate iron absorption by affecting the interaction between transferrin receptor and transferrin.

  10. Diagnosis of hepatic iron overload: a family study illustrating pitfalls in diagnosing hemochromatosis.

    Science.gov (United States)

    Schranz, Melanie; Talasz, Heribert; Graziadei, Ivo; Winder, Thomas; Sergi, Consolato; Bogner, Klaus; Vogel, Wolfgang; Zoller, Heinz

    2009-03-01

    Recent identification of genetic variants in iron storage disease has changed the classification system and diagnostic algorithms for hemochromatosis. Clinical diagnosis of the disease requires phenotypic evidence of iron overload because the commonly disease-associated HFE genotypes have an incomplete penetrance. Furthermore, approximately 20% of patients with a clinical diagnosis of hemochromatosis have no disease-associated genotype, which underlines the importance of clear phenotypic criteria of hemochromatosis. A diagnosis of hemochromatosis cannot be made even in patients with liver cirrhosis simply on the basis of genetic testing that indicates that iron overload is the cause of the disease and not its consequence. Proper diagnosis requires integration of clinical presentation, family history, and the results of biochemical and histopathologic tests. Here we propose a rational diagnostic algorithm for hepatic iron overload syndromes and illustrate potential pitfalls by presenting a family study in a pedigree with rare HFE variants (H63D and E168Q), in cis on the same chromosome. Although the clinical suspicion of hemochromatosis was confirmed by histology, chemical analysis of liver tissue revealed a normal hepatic iron concentration, which is compatible with the genetic finding of 1 normal and 1 doubly mutated allele. In conclusion, clinical suspicion of hemochromatosis and elevated serum iron parameters should prompt HFE genotyping for C282Y and H63D. Should they be uninformative, further genetic tests should be recommended only if iron overload in liver tissue has been confirmed chemically.

  11. Rational hemochromatosis therapy with erythrocytapheresis under low doses of erythropoietin

    OpenAIRE

    Brückl, Dorothea

    2016-01-01

    Summary: Hereditary hemochromatosis with an incidence of 1:200 – 1:400 is the most common hereditary disease. It is characterized by increased iron absorption and deposition in various organs, leading to organ damage and even death. To date, phlebotomy (bloodletting) is the standard therapy of iron-overload in hemochromatosis. However, this treatment is long lasting, frequently intolerable and/or remains limited due the development of significant anemia. All these difficulties might be ov...

  12. The prevalence of primary hereditary hemochromatosis in central Anatolia.

    Science.gov (United States)

    Karaca, Halit; Güven, Kadri; Önal, Müge; Gürsoy, Şebnem; Başkol, Mevlüt; Özkul, Yusuf

    2013-01-01

    Hereditary hemochromatosis is an autosomal recessive disorder associated with the HFE genes. Early identification and diagnosis is important as end stage organ damage may occur if treatment is delayed.. This study aimed to identify the prevalence of hereditary hemochromatosis in Kayseri and surroundings known as Central Anatolia. 2304 participants (1220 males, 1084 females) who were older then the age of 17 were included in the study conducted between December 2005 and December 2006 in Kayseri, Turkey. Transferin saturation was measured from overnight fasting blood samples. Serum iron, total iron binding capacity, and transferin saturation were measured. Serum ferritin levels and hereditary hemochromatosis genetic analysis were also performed after an overnight fasting blood samples from participants whose transferin saturation results were more than 50% in man and more than 45% in women. The homozygote C282Y mutation and heterozygote C282Y mutation prevalences were found as 0.08% (1/1220) and 0.08% (1/1220) in male participants, respectively. The heterozygote H63D mutation prevalence was found in 0.09% (1/1084) of female participants. Calculated prevalences in general population are as follows; The homozygote C282Y mutation prevalence is 0.043% (1/2304), the heterozygote C282Y mutation prevalence is 0.043% (1/2304) and the heterozygote H63D mutation prevalence is 0.043% (1/2304). The prevalence of hereditary hemochromatosis in Central Anatolia is 0.043% (1/2304). Because of the relatively low frequency, population screening studies are not cost-effective.

  13. FAMILY ANAMNESIS OF CHILDREN WITH MUTATION OF THE INHERITED HEMOCHROMATOSIS

    Directory of Open Access Journals (Sweden)

    S.I. Polyakova

    2010-01-01

    Full Text Available The inherited burdened is studied on diseases, associated with an overload iron in 41 children with frequent mutations of the inherited hemochromatosis (IG of a 1 type (C282y, H63d, S65c. Control group was made by 27 children with undiscovered frequent mutations of NG. Frequencies of iron-associated diseases are compared for 560 members of families which have children with mutations of IG and 390 members of families which have children without IG mutations. Some features of medical-genealogical anamnesis, which can be conditioned of siderosis, are exposed, and indirectly specify in the presence of mutations in the gene of HFE. So, the high frequency of oncologic diseases, diabetes mellitus, hepatocirrhosis and deaths of relatives under the age of 50 years are the foundation for research of exchange of iron and holding of molecular-genetic research of the inherited hemochromatosis. Key words: inherited hemochromatosis, heredity, children. (Pediatric Pharmacology. – 2010; 7(3:52-56

  14. [Molecular genetic diagnostics and screening of hereditary hemochromatosis].

    Science.gov (United States)

    Zlocha, J; Kovács, L; Pozgayová, S; Kupcová, V; Durínová, S

    2006-06-01

    Hereditary hemochromatosis is considered one of the most common hereditary diseases in population of Caucasian origin. In recent years, a candidate gene for HLA-linked hemochromatosis, HFE, has been cloned, and a single G-to-A mutation resulting in a cysteine-to-tyrosine substitution (C282Y) has been identified in up to 80% of study patients with type 1 hereditary hemochromatosis. The purpose of the paper was to confirm the importance of genetic testing for HFE mutations in making the diagnosis of hemochromatosis and find out a suitable diagnostic algorithm for the indication of this form of diagnostics in patients suspected of hereditary hemochromatosis. The examination of C282Y mutation was conducted in 500 subjects. The most frequent indications for DNA analysis were hepatopathy of unknown ethiology, liver cirrhosis, diabetes mellitus, bronze skin pigmentation in connection with high serum iron concentration, elevated transferrin saturation and elevated serum ferritin levels. In our group of patients, 29 homozygotes and 75 heterozygotes for C282Y mutation were identified, 10 patients carried both C282Y and H63D mutations of HFE gene (compound heterozygotes), whereas in 386 subjects the mutation was not found. The genotype-phenotype correlation showed that 22 homozygotes had liver affection proved by imaging and/or histologic methods. Except the liver disorders, the most common symptoms of these patients were type 2 diabetes mellitus or glucose tolerance disorder (10 patients), arthritis or joint pain (9 patients) and cardiovascular disorders, such as cardiomyopathy (2 patients). Bronze skin pigmentation was present in 9 homozygotes. Transferin saturation values were significantly higher in homozygotes for C282Y mutation as compared to C282Y heterozygotes (p diagnostics of this severe, but in early recognition curable disease. Early detection and phlebotomy treatment prior to the onset of cirrhosis can reduce morbidity and normalize life expectancy. It is readily

  15. R2*-relaxometry of the pancreas in patients with human hemochromatosis protein associated hereditary hemochromatosis

    Energy Technology Data Exchange (ETDEWEB)

    Henninger, B., E-mail: benjamin.henninger@i-med.ac.at [Department of Radiology, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck (Austria); Rauch, S. [Department of Radiology, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck (Austria); Zoller, H. [Department of Internal Medicine, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck (Austria); Plaikner, M.; Jaschke, W.; Kremser, C. [Department of Radiology, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck (Austria)

    2017-04-15

    Highlights: • MRI with R2* relaxometry is suitable to detect iron overload of the pancreas. • Pancreatic iron overload can be present in HFE associated hereditary hemochromatosis. • R2* relaxometry of the pancreas should then be performed when liver iron is present. • It can be omitted in cases with no sign of hepatic iron. - Abstract: Purpose: To evaluate pancreatic iron in patients with human hemochromatosis protein associated hereditary hemochromatosis (HHC) using R2* relaxometry. Materials and methods: 81 patients (58 male, 23 female; median age 49.5, range 10–81 years) with HHC were retrospectively studied. All underwent 1.5 T magnetic resonance imaging (MRI) of the abdomen. A fat-saturated multi-gradient echo sequence with 12 echoes (TR = 200 ms; TE-initial 0.99 ms; Delta-TE 1.41 ms; 12 echoes; flip-angle: 20°) was used for the R2* quantification of the liver and the pancreas. Parameter maps were analyzed using regions of interest (3 in the liver and 2 in the pancreas) and R2* values were correlated. Results: 59/81 patients had a liver R2* ≥ 70 1/s of which 10/59 patients had a pancreas R2* ≥ 50 1/s. No patient presented with a liver R2* < 70 1/s and pancreas R2* ≥ 50 1/s. All patients with pancreas R2* values ≥ 50 1/s had liver R2* values ≥ 70 1/s. ROC analysis resulted in a threshold of 209.4 1/s for liver R2* values to identify HFE positive patients with pancreas R2* values ≥ 50 1/s with a median specificity of 78.87% and a median sensitivity of 90%. Conclusion: In patients with HHC R2* relaxometry of the pancreas should be performed when liver iron overload is present and can be omitted in cases with no sign of hepatic iron.

  16. R2*-relaxometry of the pancreas in patients with human hemochromatosis protein associated hereditary hemochromatosis

    International Nuclear Information System (INIS)

    Henninger, B.; Rauch, S.; Zoller, H.; Plaikner, M.; Jaschke, W.; Kremser, C.

    2017-01-01

    Highlights: • MRI with R2* relaxometry is suitable to detect iron overload of the pancreas. • Pancreatic iron overload can be present in HFE associated hereditary hemochromatosis. • R2* relaxometry of the pancreas should then be performed when liver iron is present. • It can be omitted in cases with no sign of hepatic iron. - Abstract: Purpose: To evaluate pancreatic iron in patients with human hemochromatosis protein associated hereditary hemochromatosis (HHC) using R2* relaxometry. Materials and methods: 81 patients (58 male, 23 female; median age 49.5, range 10–81 years) with HHC were retrospectively studied. All underwent 1.5 T magnetic resonance imaging (MRI) of the abdomen. A fat-saturated multi-gradient echo sequence with 12 echoes (TR = 200 ms; TE-initial 0.99 ms; Delta-TE 1.41 ms; 12 echoes; flip-angle: 20°) was used for the R2* quantification of the liver and the pancreas. Parameter maps were analyzed using regions of interest (3 in the liver and 2 in the pancreas) and R2* values were correlated. Results: 59/81 patients had a liver R2* ≥ 70 1/s of which 10/59 patients had a pancreas R2* ≥ 50 1/s. No patient presented with a liver R2* < 70 1/s and pancreas R2* ≥ 50 1/s. All patients with pancreas R2* values ≥ 50 1/s had liver R2* values ≥ 70 1/s. ROC analysis resulted in a threshold of 209.4 1/s for liver R2* values to identify HFE positive patients with pancreas R2* values ≥ 50 1/s with a median specificity of 78.87% and a median sensitivity of 90%. Conclusion: In patients with HHC R2* relaxometry of the pancreas should be performed when liver iron overload is present and can be omitted in cases with no sign of hepatic iron.

  17. Predicting Factors of INSURE Failure in Low Birth Weight Neonates with RDS; A Logistic Regression Model

    OpenAIRE

    Bita Najafian; Aminsaburi Aminsaburi; Seyyed Hassan Fakhraei; Abolfazl afjeh; Fatemeh Eghbal; Reza Noroozian

    2015-01-01

    Background:Respiratory Distress syndrome is the most common respiratory disease in premature neonate and the most important cause of death among them. We aimed to investigate factors to predict successful or failure of INSURE method as a therapeutic method of RDS. Methods:In a cohort study,45 neonates with diagnosed RDS and birth weight lower than 1500g were included and they underwent INSURE followed by NCPAP(Nasal Continuous Positive Airway Pressure). The patients were divided into failu...

  18. Introduction of Molecular Diagnosis of Hemochromatosis Type 1 in Cuba

    Directory of Open Access Journals (Sweden)

    Ismael Aramís Cervera García

    2013-06-01

    Full Text Available Background: hemochromatosis type 1 is an autosomal recessive genetic disorder, which should be diagnosed during its preclinical phase in order to prevent severe organ damage. Objective: to establish the diagnosis of hemochromatosis type 1 in Cuba, and calculate its frequencies in patients with hepatopathies. Methods: an analytic cross-sectional study was conducted including 65 patients with liver disease, who were referred to the laboratory of Molecular Biology of the National Medical Genetics Center by clinical geneticists. A PCR-RFLP analysis was used for detecting the C282Y and H63D mutations in the HFE gene. Results: PCR-RFLP analysis was standardized for the detection of C282Y and H63D mutations. Frequencies of C282Y and H63D mutations in the HFE gene in patients with hepatopathies were 6.3% and 18.2% respectively. Conclusions: molecular diagnosis of C282Y and H63D mutations in the HFE gene causing hemochromatosis type 1 contributed to the identification of 28 carriers in the 65 patients who were studied, as well as a homozygous individual for the H63D mutation, which shows the high prevalence of these mutations in Cuban patients with liver disease.

  19. Plasma Endothelin-1 Levels in Preterm Neonatal Infants with Acute Respiratory Failure

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    D. V. Dmitriyev

    2008-01-01

    Full Text Available Increased pulmonary vascular resistance in preterm infants is associated with acute respiratory failure (ARF and at the same time endothelin-1 (E-1 plays an important role in neonatal pulmonary vascular responsiveness. Methods. Endothelin-1 levels were measured in two blood samples in 12 preterm infants with ARF and in 12 controls (at 32.2±1.3 and 29.8±1.2 weeks of gestation, respectively by enzyme immunoassay. For this, the first and second blood samples were taken at 18 to 40 hours after birth. Results. The plasma level of E-1 in the first sample did not differ between the neonates of both groups. In the second sample, significantly higher E-1 concentrations were observed in the newborns with ARF than in the controls. In the first sample, E-1 concentration were higher than in the second one in both groups (p<0.001. There was a significant positive correlation between the second E-1 sample and the SNAPPE 2 scale rating (r=0.38; p=0.02. The plasma level of E-1 in the first sample did not differ in both groups (11.9 and 12.2 pg/ml, respectively. Conclusion. Neonates with and without ARF had the similar plasma E-1 levels in the first sample, by taking into account the fact that the E-1 levels were higher in ARF than in the controls at 18 to 40 hour after birth. Increased vascular resistance in ARF may be associated with the high level of E-1. Key words: endothe-lin-1, acute respiratory failure.

  20. Does bilirubin protect against hemochromatosis gene (HFE) related mortality?

    NARCIS (Netherlands)

    Alizadeh, Behrooz Z.; Njajou, Omer T.; Houwing-Duistermaat, Jeanine J.; de Jong, Gerard; Vergeer, Jeannette M.; Hofman, Albert; Pols, Huibert A.P.; van Duijn, Cornelia M.

    2004-01-01

    Serum bilirubin is an important antioxidant that is found at increased levels in hereditary hemochromatosis patients. We hypothesized that increased levels of serum bilirubin may play a protective role against oxidative stress induced by iron overload in carriers of mutations in the hereditary

  1. Placental Inflammatory Changes and Bacterial Infection in Premature Neonates with Respiratory Failure

    Directory of Open Access Journals (Sweden)

    S. A. Perepelitsa

    2012-01-01

    Full Text Available Objective: to reveal a relationship of placental inflammatory changes to bacterial infection in premature neonates with respiratory failure. Material and methods. Bronchoalveolar aspirate was bacteriologically studied in 157 premature neonates with respiratory distress syndrome (NRDS; the total and differential leukocyte counts were measured in their peripheral blood. The levels of the cytokines IL-1^3, IL-4, IL-6, and TNF-a were studied in different biological fluids of mothers and their babies; the placentas were also morphologically examined. Results. An analysis of bacterial cultures from the tracheobronchial tree revealed no growth of bacterial microflora in 61.8% of cases, Enterococcus faecalis and Staphylococcus epidermidis were isolated in 6.4 and 8.3% of the infants, respectively; Staphylococcus haemolyticus, Staphylococcus capitis, Enterobacter agglomerans, and hemolytic group A Streptococcus were seen in 1.9% each; moreover, 1.3% of the newborn infants were found to have Bacillus spp., Staphylococcus aureus, Escherichia coli, Acinetobacter spp., and Serratia marcescens. Other microorganisms and a microbial association were encountered in 8.9% of cases. Placental morphological examination revealed different inflammatory changes concurrent with chronic and acute placental insufficiency. The investigation demonstrated that the maternal peripheral plasma levels of IL-1^, IL-4, IL-6, and TNF-a were within the physiological range at the end of the first period of delivery. The amniotic fluid displayed elevated IL-6 and TNF-a concentrations and normal IL-4 and IL-1e levels, suggesting that there was an intrauterine inflammatory process. Conclusion. Premature birth is associated with various placental inflammatory changes, which causes intrauterine stimulation of macrophages in the chorionic villi. Specific immune defense mechanisms that prevent the development of a fetal infectious process, i.e. the maternal infectious process, may induce

  2. Hereditary hemochromatosis and risk of ischemic heart disease

    DEFF Research Database (Denmark)

    Ellervik, Christina; Tybjaerg-Hansen, Anne; Grande, Peer

    2005-01-01

    BACKGROUND: We tested the hypothesis that the hereditary hemochromatosis genotypes C282Y/C282Y, C282Y/H63D, or C282Y/wild-type are risk factors for ischemic heart disease (IHD) and myocardial infarction (MI). METHODS AND RESULTS: We performed a prospective study of 9178 individuals from the Danish...

  3. R2*-relaxometry of the pancreas in patients with human hemochromatosis protein associated hereditary hemochromatosis.

    Science.gov (United States)

    Henninger, B; Rauch, S; Zoller, H; Plaikner, M; Jaschke, W; Kremser, C

    2017-04-01

    To evaluate pancreatic iron in patients with human hemochromatosis protein associated hereditary hemochromatosis (HHC) using R2* relaxometry. 81 patients (58 male, 23 female; median age 49.5, range 10-81 years) with HHC were retrospectively studied. All underwent 1.5T magnetic resonance imaging (MRI) of the abdomen. A fat-saturated multi-gradient echo sequence with 12 echoes (TR=200ms; TE-initial 0.99ms; Delta-TE 1.41ms; 12 echoes; flip-angle: 20°) was used for the R2* quantification of the liver and the pancreas. Parameter maps were analyzed using regions of interest (3 in the liver and 2 in the pancreas) and R2* values were correlated. 59/81 patients had a liver R2*≥70 1/s of which 10/59 patients had a pancreas R2*≥50 1/s. No patient presented with a liver R2*pancreas R2*≥50 1/s. All patients with pancreas R2* values≥50 1/s had liver R2* values≥70 1/s. ROC analysis resulted in a threshold of 209.4 1/s for liver R2* values to identify HFE positive patients with pancreas R2* values≥50 1/s with a median specificity of 78.87% and a median sensitivity of 90%. In patients with HHC R2* relaxometry of the pancreas should be performed when liver iron overload is present and can be omitted in cases with no sign of hepatic iron. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. Acute liver failure in a term neonate after repeated paracetamol administration

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    Fabio Bucaretchi

    2014-03-01

    Full Text Available Objective: Severe hepatotoxicity caused by paracetamol is rare in neonates. We report a case of paracetamol-induced acute liver failure in a term neonate. Case description: A 26-day-old boy was admitted with intestinal bleeding, shock signs, slight liver enlargement, coagulopathy, metabolic acidosis (pH=7.21; bicarbonate: 7.1mEq/L, hypoglycemia (18mg/dL, increased serum aminotransferase activity (AST=4,039IU/L; ALT=1,087IU/L and hyperbilirubinemia (total: 9.57mg/dL; direct: 6.18mg/dL after receiving oral paracetamol (10mg/kg/dose every 4 hours for three consecutive days (total dose around 180mg/kg; serum concentration 36-48 hours after the last dose of 77µg/ mL. Apart from supportive measures, the patient was successfully treated with intravenous N-acetylcysteine infusion during 11 consecutive days, and was discharged on day 34. The follow-up revealed full recovery of clinical and of laboratory findings of hepatic function. Comments: The paracetamol pharmacokinetics and pharmacodynamics in neonates and infants differ substantially from those in older children and adults. Despite the reduced rates of metabolism by the P-450 CYP2E1 enzyme system and the increased ability to synthesize glutathione - which provides greater resistance after overdoses -, it is possible to produce hepatotoxic metabolites (N-acetyl-p-benzoquinone that cause hepatocellular damage, if glutathione sources are depleted. Paracetamol clearance is reduced and the half-life of elimination is prolonged. Therefore, a particular dosing regimen should be followed due to the toxicity risk of cumulative doses. This report highlights the risk for severe hepatotoxicity in neonates after paracetamol multiple doses for more than two to three days.

  5. Visão atual da hemocromatose hereditária Current approach to hereditary hemochromatosis

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    Rodolfo Delfini Cançado

    2010-01-01

    Full Text Available A hemocromatose hereditária (HH está relacionada a diversos distúrbios do metabolismo do ferro que ocasionam sua sobrecarga tecidual. A HH clássica está associada às mutações do gene HFE (homozigose para C282Y ou duplo heterozigose para C282Y/H63D, sendo encontrada quase exclusivamente em descendentes do norte Europeu. A hemocromatose hereditária, quando não relacionada ao gene HFE, é causada por mutações de outros genes, recentemente identificados, envolvidos no metabolismo do ferro. Hepcedina é o hormônio regulador do ferro que inibe a ferroportina, proteína exportadora de ferro dos enterócitos e dos macrófagos; um defeito na expressão do gene da hepcedina ou na sua função costuma ser a causa da maioria dos tipos de hemocromatose hereditária. Os alvos acometidos pela HH são órgãos e tecidos - fígado, coração, pâncreas, articulações e pele -, sendo a cirrose e o diabetes melito os sinais tardios da doença em pacientes com expressivo aumento da concentração hepática de ferro. Pacientes com diagnóstico estabelecido de hemocromatose hereditária e sobrecarga de ferro devem ser tratados com flebotomia para a obtenção de depleção do ferro do organismo; em seguida, com flebotomia de manutenção. As causas mais frequentes de morte por hemocromatose hereditária são câncer hepático, cirrose, miocardiopatia e diabete; entretanto, pacientes submetidos à depleção do ferro de maneira satisfatória e antes do desenvolvimento da cirrose ou da diabete podem ter sobrevida normal.Hereditary hemochromatosis refers to several inherited disorders of the iron metabolism that lead to tissue iron overload. Classical hereditary hemochromatosis is associated with mutations of the HFE gene (C282Y homozygotes or C282Y/H63D compound heterozygotes and is almost exclusively found in populations of northern European descent. Non-HFE-associated hereditary hemochromatosis is caused by mutations in other recently identified genes

  6. Changes of iron concentrations in skin and plasma of patients with hemochromatosis along therapy

    International Nuclear Information System (INIS)

    Pinheiro, T.; Alves, L.C.; Neres, M.; Pinheiro, T.; Barreiros, A.; Fleming, R.; Silva, J.N.; Filipe, P.; Silva, R.

    2009-01-01

    Skin as a manageable organ can provide direct or indirect information of tissue iron overload resulting from inherited disorders as hemochromatosis. Patients with hemochromatosis were evaluated at three consecutive phases along the therapy programme. Nuclear microprobe techniques were used to assess skin iron and Total Reflection X-ray Fluorescence to determine the plasma iron concentrations. Results showed that iron pools were differently correlated at the three therapy phases. These variations highlighted the value of skin iron content to assess organ iron deposition and therapy efficacy. Skin iron content can be used for a better management of patients with iron overload pathologies. (author)

  7. Association of mutations in the hemochromatosis gene with shorter life expectancy

    DEFF Research Database (Denmark)

    Bathum, L; Christiansen, L; Nybo, H

    2001-01-01

    BACKGROUND: To investigate whether the frequency of carriers of mutations in the HFE gene associated with hereditary hemochromatosis diminishes with age as an indication that HFE mutations are associated with increased mortality. It is of value in the debate concerning screening for hereditary...... hemochromatosis to determine the significance of heterozygosity. METHODS: Genotyping for mutations in exons 2 and 4 of the HFE gene using denaturing gradient gel electrophoresis in 1784 participants aged 45 to 100 years from 4 population-based studies: all 183 centenarians from the Danish Centenarian Study, 601...... in the distribution of mutations in exon 2 in the different age groups. CONCLUSIONS: In a high-carrier frequency population like Denmark, mutations in HFE show an age-related reduction in the frequency of heterozygotes for C282Y, which suggests that carrier status is associated with shorter life expectancy....

  8. Molecular Diagnostic and Pathogenesis of Hereditary Hemochromatosis

    Directory of Open Access Journals (Sweden)

    Paulo C. J. L. Santos

    2012-02-01

    Full Text Available Hereditary hemochromatosis (HH is an autosomal recessive disorder characterized by enhanced intestinal absorption of dietary iron. Without therapeutic intervention, iron overload leads to multiple organ damage such as liver cirrhosis, cardiomyopathy, diabetes, arthritis, hypogonadism and skin pigmentation. Most HH patients carry HFE mutant genotypes: homozygosity for p.Cys282Tyr or p.Cys282Tyr/p.His63Asp compound heterozygosity. In addition to HFE gene, mutations in the genes that encode hemojuvelin (HJV, hepcidin (HAMP, transferrin receptor 2 (TFR2 and ferroportin (SLC40A1 have been associated with regulation of iron homeostasis and development of HH. The aim of this review was to identify the main gene mutations involved in the pathogenesis of type 1, 2, 3 and 4 HH and their genetic testing indication. HFE testing for the two main mutations (p.Cys282Tyr and p.His63Asp should be performed in all patients with primary iron overload and unexplained increased transferrin saturation and/or serum ferritin values. The evaluation of the HJV p.Gly320Val mutation must be the molecular test of choice in suspected patients with juvenile hemochromatosis with less than 30 years and cardiac or endocrine manifestations. In conclusion, HH is an example that genetic testing can, in addition to performing the differential diagnostic with secondary iron overload, lead to more adequate and faster treatment.

  9. Hemochromatosis C282Y gene mutation as a potential susceptibility ...

    African Journals Online (AJOL)

    G.M. Mokhtar

    2017-08-12

    Aug 12, 2017 ... Background: Hereditary hemochromatosis is the most frequent cause of primary iron overload that is associated with HFE gene's mutation especially the C282Y mutation. The interaction between hemoglo- bin chain synthesis' disorders and the C282Y mutation may worsen the clinical picture of beta-.

  10. Peritoneal dialysis in the neonatal intensive care unit. Management of acute renal failure after a severe subgaleal hemorrhage.

    Science.gov (United States)

    Coe, Kristi; Lail, Candace

    2007-08-01

    Acute renal failure is common in the neonatal intensive care unit but is often not recognized in its early phases, when it is potentially reversible. The typical patient with acute renal failure is premature, but many term infants are also at risk. One such group is those with severe bleeding, such as a subgaleal hemorrhage. In these cases, hypovolemia can quickly progress to ischemia, which affects many organs but has profound effects on the kidney. In term infants, acute renal failure is most commonly diagnosed in those with perinatal depression. This article presents a unique case of an infant with subgaleal and intracranial bleeding that resulted in acute renal failure requiring peritoneal dialysis in the hopes of the eventual restoration of kidney function.

  11. Neonatal Death and Heart Failure in Mouse with Transgenic HSP60 Expression

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    Tsung-Hsien Chen

    2015-01-01

    Full Text Available Mitochondrial heat shock proteins, such as HSP60, are chaperones responsible for the folding, transport, and quality control of mitochondrial matrix proteins and are essential for maintaining life. Both prosurvival and proapoptotic roles have been proposed for HSP60, and HSP60 is reportedly involved in the initiation of autoimmune, metabolic, and cardiovascular diseases. The role of HSP60 in pathogenesis of these diseases remains unclear, partly because of the lack of mouse models expressing HSP60. In this study we generated HSP60 conditional transgenic mice suitable for investigating in vivo outcomes by expressing HSP60 at the targeted organ in disease models. Ubiquitous HSP60 induction in the embryonic stage caused neonatal death in mice at postnatal day 1. A high incidence of atrial septal defects was observed in HSP60-expressing mice, with increased apoptosis and myocyte degeneration that possibly contributed to massive hemorrhage and sponge-like cardiac muscles. Our results showed that neonatal heart failure through HSP60 induction likely involves developmental defects and excessive apoptosis. The conditional HSP60 mouse model is useful for studying crucial biological questions concerning HSP60.

  12. Hemochromatosis: abnormalities of bones and joints: a case report and literature review

    International Nuclear Information System (INIS)

    Farao, S.R.F.; Pereira, E.M.; Harima, H.A.; Rocha Correa Fernandes, A. da; Pavin, A.E.

    1989-01-01

    The authors report a case of a 49 years-old male patient with emphasis in the arthropathy of hemochromatosis. The arthropathy was the first manifestation: the patient had been complaining of pain on the right hip for eight years. The other specific clinical manifestations: diabetes, abnormal pigmentation appeared after six years. The roentgenographic features of bone and joint involvement include abnormalites at metacarpophalangeal joints with osteophytes on the metacarpal heads and in the hip, joint space narrowing, was seen. In the knee involvement is characterized by subchondral cyst and osteophytosis. Laboratory analysis are: serum iron = 191 mg/dl (normal value: 50-150 mg/dl), ferritin > 400 ng/ml (normal value: 42-26 ng/ml). Iron within the parenchymal cells of the liver cirrhosis was detected by hepatic biopsy. Hemochromatosis was pathologically characterized by tissue damage produced by iron deposition. (author) [pt

  13. HFE C282Y/H63D Compound Heterozygotes Are at Low Risk of Hemochromatosis-Related Morbidity

    OpenAIRE

    Gurrin, Lyle C.; Bertalli, Nadine A.; Dalton, Gregory W.; Osborne, Nicholas J.; Constantine, Clare C.; McLaren, Christine E.; English, Dallas R.; Gertig, Dorota M.; Delatycki, Martin B.; Nicoll, Amanda J.; Southey, Melissa C.; Hopper, John L.; Giles, Graham G.; Anderson, Gregory J.; Olynyk, John K.

    2009-01-01

    The risk of hemochromatosis-related morbidity is unknown among HFE compound heterozygotes (C282Y/H63D). We used a prospective population-based cohort study to estimate the prevalence of elevated iron indices and hemochromatosis-related morbidity for compound heterozygotes. In all, 31,192 subjects of northern European descent were genotyped for HFE C282Y and H63D. An HFE-genotype stratified random sample of 1,438 subjects, followed for an average of 12 years to a mean age of 65 years, complete...

  14. Whole Blood Polymerase Chain Reaction in a Neonate with Disseminated Herpes Simplex Virus Infection and Liver Failure

    Directory of Open Access Journals (Sweden)

    Jennifer A. Scoble

    2013-10-01

    Full Text Available A late preterm neonate born by cesarean section with intact membranes presented at 9 days of life with shock and liver failure. Surface cultures were negative but whole blood polymerase chain reaction was positive for herpes simplex virus type 2, underscoring the value of this test in early diagnosis of perinatally acquired disseminated herpes simplex virus infection without skin lesions.

  15. Anti-Obesity and Pro-Diabetic Effects of Hemochromatosis

    OpenAIRE

    Abbas, Mousa Al; Abraham, Deveraprabu; Kushner, James P.; McClain, Donald A.

    2014-01-01

    Objective Levels of tissue iron contribute to determining diabetes risk, but little is known about the effects of higher iron levels on weight, nor on the interaction of weight and iron overload on diabetes risk. We therefore examined the effect of iron on body mass index and diabetes in individuals with iron overload from hereditary hemochromatosis (HH), compared to non-HH siblings and historical controls. Methods Chart reviews were performed on a cohort of adults (age ≥40, N=101) with the c...

  16. Prevalence of H63D, S65C, and C282Y hereditary hemochromatosis gene variants in Madeira Island (Portugal).

    Science.gov (United States)

    Spínola, Carla; Brehm, António; Spínola, Hélder

    2011-01-01

    Hereditary HFE Hemochromatosis is an inherited disorder of iron metabolism that results from mutations in the HFE gene. Almost all patients with hereditary hemochromatosis show a C282Y mutation in homozygosity or in compound heterozygosity with H63D. Also, the mutation S65C has been shown to be associated to a milder iron overload. Since allele and genotype frequencies of these three variants of the HFE gene vary between populations, the determination of their prevalence in Madeira Island will clarify the population susceptibility to hereditary hemochromatosis. One hundred and fifty-four samples from Madeira Island were genotyped for the three most common HFE gene mutations, H63D, C282Y, and S65C, by polymerase chain reaction followed by restriction fragment length polymorphism analysis. Results have shown a prevalence of 20.5%, 0.33%, and 1% for H63D, C282Y, and S65C, respectively. Accordingly to our estimates, both genotypes associated to hereditary hemochromatosis, C282Y homozygotes and C282/H63D compound heterozygotes, could be present in Madeira Island population in 1,648 individuals, which represents 0.65% of the total population.

  17. Computational Profiling of Deleterious Non-Synonymous SNP’s in HFE

    Directory of Open Access Journals (Sweden)

    S. Sarath Raj

    2017-12-01

    Full Text Available Liver cirrhosis describes a condition where scar tissue gradually replaces healthy cells in liver. The main causes are sustained, excessive alcohol consumption, viral hepatitis B and C, and fatty liver disease - however, there are other possible causes. Hemochromatosis is most common form of iron overload disease. Three types of hemochromatosis are primary hemochromatosis, also called hereditary hemochromatosis; secondary hemochromatosis; and neonatal hemochromatosis. The HFE gene helps regulate the amount of iron absorbed from food and inherited genetic defects or mutation in HFE [C282Y] cause primary hemochromatosis. Computational approach is sought to determine other similar mutations in this gene. In-silico tools such as SIFT, Polyphen 2.0, and PROVEAN were employed to determine the various deleterious ns-SNPs of HFE that may influence cystic fibrosis.

  18. Iron storage disease (hemochromatosis) and hepcidin response to iron load in two species of pteropodid fruit bats relative to the common vampire bat.

    Science.gov (United States)

    Stasiak, Iga M; Smith, Dale A; Ganz, Tomas; Crawshaw, Graham J; Hammermueller, Jutta D; Bienzle, Dorothee; Lillie, Brandon N

    2018-07-01

    Hepcidin is the key regulator of iron homeostasis in the body. Iron storage disease (hemochromatosis) is a frequent cause of liver disease and mortality in captive Egyptian fruit bats (Rousettus aegyptiacus), but reasons underlying this condition are unknown. Hereditary hemochromatosis in humans is due to deficiency of hepcidin or resistance to the action of hepcidin. Here, we investigated the role of hepcidin in iron metabolism in one species of pteropodid bat that is prone to iron storage disease [Egyptian fruit bat (with and without hemochromatosis)], one species of pteropodid bat where iron storage disease is rare [straw-colored fruit bat (Eidolon helvum)], and one species of bat with a natural diet very high in iron, in which iron storage disease is not reported [common vampire bat (Desmodus rotundus)]. Iron challenge via intramuscular injection of iron dextran resulted in significantly increased liver iron content and histologic iron scores in all three species, and increased plasma iron in Egyptian fruit bats and straw-colored fruit bats. Hepcidin mRNA expression increased in response to iron administration in healthy Egyptian fruit bats and common vampire bats, but not in straw-colored fruit bats or Egyptian fruit bats with hemochromatosis. Hepcidin gene expression significantly correlated with liver iron content in Egyptian fruit bats and common vampire bats, and with transferrin saturation and plasma ferritin concentration in Egyptian fruit bats. Induction of hepcidin gene expression in response to iron challenge is absent in straw-colored fruit bats and in Egyptian fruit bats with hemochromatosis and, relative to common vampire bats and healthy humans, is low in Egyptain fruit bats without hemochromatosis. Limited hepcidin response to iron challenge may contribute to the increased susceptibility of Egyptian fruit bats to iron storage disease.

  19. Predicting C282Y Homozygote Genotype for Hemochromatosis Using Serum Ferritin and Transferrin Saturation Values from 44,809 Participants of the HEIRS Study

    Directory of Open Access Journals (Sweden)

    Andrew Lim

    2014-01-01

    Full Text Available INTRODUCTION: The simultaneous interpretation of serum ferritin level and transferrin saturation has been used as a clinical guide to diagnose genetic hemochromatosis. The Hemochromatosis and Iron Overload Screening (HEIRS Study screened 101,168 North American participants for serum ferritin level and transferrin saturation, and C282Y genotyping for the HFE gene.

  20. Noninvasive Positive Pressure Ventilation or Conventional Mechanical Ventilation for Neonatal Continuous Positive Airway Pressure Failure

    Directory of Open Access Journals (Sweden)

    Zohreh Badiee

    2014-01-01

    Full Text Available Background: The aim of this study was to assess the success rate of nasal intermittent positive pressure ventilation (NIPPV for treatment of continuous positive airway pressure (CPAP failure and prevention of conventional ventilation (CV in preterm neonates. Methods: Since November 2012 to April 2013, a total number of 55 consecutive newborns with gestational ages of 26-35 weeks who had CPAP failure were randomly assigned to one of the two groups. The NIPPV group received NIPPV with the initial peak inspiratory pressure (PIP of 16-20 cmH 2 O and frequency of 40-60 breaths/min. The CV group received PIP of 12-20 cmH 2 O and frequency of 40-60 breaths/min. Results: About 74% of newborns who received NIPPV for management of CPAP failure responded to NIPPV and did not need intubation and mechanical ventilation. Newborns with lower postnatal age at entry to the study and lower 5 min Apgar score more likely had NIPPV failure. In addition, treatment failure was higher in newborns who needed more frequent doses of surfactant. Duration of oxygen therapy was 9.28 days in CV group and 7.77 days in NIPPV group (P = 0.050. Length of hospital stay in CV group and NIPPV groups were 48.7 and 41.7 days, respectively (P = 0.097. Conclusions: NIPPV could decrease the need for intubation and mechanical ventilation in preterm infants with CPAP failure.

  1. A Mouse Model of Cardiomyopathy Induced by Mutations in the Hemochromatosis HFE Gene.

    Science.gov (United States)

    Djemai, Haidar; Thomasson, Rémi; Trzaskus, Yvan; Mougenot, Nathalie; Meziani, Amira; Toussaint, Jean-François; Noirez, Philippe; Vitiello, Damien

    2017-07-01

    The heart is 1 of the organs most affected by hereditary hemochromatosis (HH). The clinical impact of cardiomyopathy in patients with HH requires a particular diagnosis and less invasive treatments. We developed a model of cardiomyopathy in knockout (KO) mice for the high-Fe (HFE) gene and assessed left ventricular (LV) function and structure from 7-20 months. Male wild-type (WT) heterozygous and KO SV129 mice for the HFE gene were used in this study. Twenty-four mice were used to assess LV function and structure by echocardiography at 7, 14, 18, and 20 months. Evaluations of LV function and structure and myocardial fibrosis were performed at 7 and 20 months. The percent decrease of LV thickness-to-radius ratio between 7 and 20 months was higher in KO mice compared with WT mice (-30.2% ± 5.3% vs -10.5% ± 4.9%; P HFE-related hemochromatosis. Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  2. Total and cause-specific mortality by elevated transferrin saturation and hemochromatosis genotype in individuals with diabetes - two general population studies

    DEFF Research Database (Denmark)

    Ellervik, Christina; Mandrup-Poulsen, Thomas; Tybjærg-Hansen, Anne

    2013-01-01

    ObjectiveMortality is increased in patients with hereditary hemochromatosis, in individuals from the general population with increased transferrin saturation(TS), and also in patients with diabetes type 1 and increased TS from a highly specialised diabetes clinic. Thus, we have recommended targeted...... and hemochromatosis genotype(HFE) C282Y/C282Y in individuals with diabetes(type 1,N=118;type 2,N=3228;total,N=3346).ResultsThe cumulative survival was reduced in individuals with diabetes with TS≥50% vs....

  3. Neonatal varicella pneumonia, surfactant replacement therapy

    Directory of Open Access Journals (Sweden)

    Mousa Ahmadpour-kacho

    2015-12-01

    Full Text Available Background: Chickenpox is a very contagious viral disease that caused by varicella-zoster virus, which appears in the first week of life secondary to transplacental transmission of infection from the affected mother. When mother catches the disease five days before and up to two days after the delivery, the chance of varicella in neonate in first week of life is 17%. A generalized papulovesicular lesion is the most common clinical feature. Respiratory involvement may lead to giant cell pneumonia and respiratory failure. The mortality rate is up to 30% in the case of no treatment, often due to pneumonia. Treatment includes hospitalization, isolation and administration of intravenous acyclovir. The aim of this case report is to introduce the exogenous surfactant replacement therapy after intubation and mechanical ventilation for respiratory failure in neonatal chickenpox pneumonia and respiratory distress. Case Presentation: A seven-day-old neonate boy was admitted to the Neonatal Intensive Care Unit at Amirkola Children’s Hospital, Babol, north of Iran, with generalized papulovesicular lesions and respiratory distress. His mother has had a history of Varicella 4 days before delivery. He was isolated and given supportive care, intravenous acyclovir and antibiotics. On the second day, he was intubated and connected to mechanical ventilator due to severe pneumonia and respiratory failure. Because of sever pulmonary involvement evidenced by Chest X-Ray and high ventilators set-up requirement, intratracheal surfactant was administered in two doses separated by 12 hours. He was discharged after 14 days without any complication with good general condition. Conclusion: Exogenous surfactant replacement therapy can be useful as an adjunctive therapy for the treatment of respiratory failure due to neonatal chickenpox.

  4. Therapeutic Depletion of Iron Stores Is Not Associated with a Reduced Hemoglobin Mass in a Hemochromatosis Patient

    Directory of Open Access Journals (Sweden)

    Nina Wrobel

    2016-08-01

    Full Text Available Introduction: Hereditary hemochromatosis features a dysregulated iron absorption leading to iron overload and organ damage. The regulation of total hemoglobin mass during depletion of iron deposits by therapeutic phlebotomy has not been studied. Case Presentation: The initial ferritin level of the 52-year-old male subject was 1,276 μg/l. Despite successful depletion of iron stores (ferritinmin: 53 μg/l through phlebotomies, total hemoglobin mass stabilized at the pretherapy level. However, regeneration of total hemoglobin mass was accelerated (up to 10.8 g/day. Conclusion: In this hemochromatosis patient, the total hemoglobin mass was not altered in the long term, but regeneration was accelerated, possibly due to elevated body iron content.

  5. Usefulness of Magnetic Resonance Imaging for the Diagnosis of Hemochromatosis with Severe Hepatic Steatosis in Nonalcoholic Fatty Liver Disease.

    Science.gov (United States)

    Nozaki, Yuichi; Sato, Noriko; Tajima, Tsuyoshi; Hasuo, Kanehiro; Kojima, Yasushi; Umemoto, Kumiko; Mishima, Saori; Mikami, Shintaro; Nakayama, Tomohiro; Igari, Toru; Akiyama, Junichi; Imamura, Masatoshi; Masaki, Naohiko; Yanase, Mikio

    2016-01-01

    The ratio of the number of patients with non-alcoholic steatohepatitis (NASH) to the total number of patients with liver dysfunction has increased in many countries around the world. Liver dysfunction is also caused by multiple blood transfusions in patients with leukemia and other hematological diseases, with liver dysfunction often accompanied by secondary hemochromatosis. This study describes a 25-year-old man with secondary hemochromatosis combined with NASH. Magnetic resonance imaging was useful for visualizing the distributions of both iron and fat in the liver of this patient in order to make a differential diagnosis and to evaluate the effect of treatment.

  6. Obstetrical and neonatal outcomes following unsuccessful external cephalic version: a stratified analysis amongst failures, successes, and controls.

    Science.gov (United States)

    Balayla, Jacques; Dahdouh, Elias M; Villeneuve, Sophie; Boucher, Marc; Gauthier, Robert J; Audibert, François; Fuchs, Florent

    2015-03-01

    Though on average one out of every two external cephalic versions (ECV) fails to rotate the breech fetus, little is known about the outcomes of pregnancies in which ECV is unsuccessful. The objective of the present study is to compare obstetrical and neonatal outcomes following failure of ECV, relative to cases of breech controls without an attempt at ECV. We conducted a retrospective, population-based, cohort study using the CDC's Birth Data files from the US for the year 2006. We stratified the cohort according to fetal presentation and ECV status: success, failure, and no ECV (controls). The effect of failure of ECV on the risk of several neonatal and obstetrical outcomes was estimated using logistic regression analysis, adjusting for relevant confounders. We analyzed a total of 4 273 225 births, out of which 183 323 (4.3%) met inclusion criteria. Relative to breech controls, failed ECV occurred more frequently amongst Caucasian, college-educated, married women bearing a female fetus. Compared to no ECV, failure of ECV was associated with increased odds of PROM (aOR, 1.75; 95% CI, 1.60-1.90), elective cesarean delivery (aOR, 1.53; 95% CI, 1.36-1.72), cesarean delivery in labor (aOR, 1.38; 95% CI, 1.21-1.57), abnormal fetal heart tracing (aOR, 1.78; 95% CI, 1.50-2.11), assisted ventilation at birth (aOR, 1.50; 95% CI, 1.27-1.78), 5-min APGAR scores <7 (aOR, 1.35; 95% CI, 1.20-1.51), and NICU admission (aOR, 1.48; 95% CI, 1.20-1.82). The delayed spontaneous fetal restitution rate was 13%. When stratifying controls with regards to trial of labor status, the increased risk of failed ECV persisted for cesarean delivery, NICU admission, assisted ventilation and abnormal fetal tracing, independently of whether a trial of labor took place. Relative to breech controls without attempt at ECV, failure of ECV to restitute cephalic presentation appears to be associated with an increased risk of adverse perinatal and obstetrical outcomes.

  7. An overview of medical ECMO for neonates.

    Science.gov (United States)

    Fletcher, Kathryn; Chapman, Rachel; Keene, Sarah

    2018-03-01

    Extracorporeal membrane oxygenation (ECMO), a life-saving therapy for respiratory and cardiac failure, was first used in neonates in the 1970s. The indications and criteria for ECMO have changed over the years, but it continues to be an important option for those who have failed other medical therapies. Since the Extracorporeal Life Support Organization (ELSO) Registry was established in 1989, more than 29,900 neonates have been placed on ECMO for respiratory failure, with 84% surviving their ECMO course, and 73% surviving to discharge or transfer. In this chapter, we will review the basics of ECMO, patient characteristics and criteria, patient management, ECMO complications, special uses of neonatal ECMO, and patient outcomes. Copyright © 2018 Elsevier Inc. All rights reserved.

  8. Acute heart failure associated with congenital complete atrioventricular block due to neonatal lupus: case series report

    International Nuclear Information System (INIS)

    Velasquez F, Carlos J; Correa, Cindy; Molina, Monica; Fernanda, Luisa; Velasquez, Nataly; Diaz, Luis H

    2010-01-01

    Neonatal lupus (NL) defines a set of clinical syndromes characterized by maternal autoantibodies against the RNA protein complex (Ro/SSA or La/SSB) that cross the placenta and potentially lead to fetal tissue damage. Little is known about other cardiac manifestations of NL different from congenital heart block (CHB), as heart failure (HF). Four cases of LN with BCC and acute HF at the first three days of life on average were reported. No complications or deaths were reported at 4,9 months of follow-up. All infants had anti-Ro high titles at the time of diagnosis.

  9. Neonatal outcomes and operative vaginal delivery versus cesarean delivery.

    LENUS (Irish Health Repository)

    Contag, Stephen A

    2010-06-01

    We compared outcomes for neonates with forceps-assisted, vacuum-assisted, or cesarean delivery in the second stage of labor. This is a secondary analysis of a randomized trial in laboring, low-risk, nulliparous women at >or=36 weeks\\' gestation. Neonatal outcomes after use of forceps, vacuum, and cesarean were compared among women in the second stage of labor at station +1 or below (thirds scale) for failure of descent or nonreassuring fetal status. Nine hundred ninety women were included in this analysis: 549 (55%) with an indication for delivery of failure of descent and 441 (45%) for a nonreassuring fetal status. Umbilical cord gases were available for 87% of neonates. We found no differences in the base excess (P = 0.35 and 0.78 for failure of descent and nonreassuring fetal status) or frequencies of pH below 7.0 (P = 0.73 and 0.34 for failure of descent and nonreassuring fetal status) among the three delivery methods. Birth outcomes and umbilical cord blood gas values were similar for those neonates with a forceps-assisted, vacuum-assisted, or cesarean delivery in the second stage of labor. The occurrence of significant fetal acidemia was not different among the three delivery methods regardless of the indication.

  10. [Risk factors for neonatal pulmonary hemorrhage in the neonatal intensive care unit of a municipal hospital].

    Science.gov (United States)

    Fan, Jie; Hei, Ming-Yan; Huang, Xi-Lin; Li, Xiao-Ping

    2017-03-01

    To investigate the risk factors for neonatal pulmonary hemorrhage (NPH) in the neonatal intensive care unit (NICU) of a municipal hospital, and to provide a basis for the early identification and treatment of NPH. A total of 112 neonates who were admitted to the NICU of Shaoyang Central Hospital of Hunan Province and diagnosed with NPH were enrolled as the case group. A nested case-control method was used to select, as a control group (n=224), the neonates who underwent the treatment with an assisted mechanical ventilator and did not experience pulmonary hemorrhage. Univariate analysis and unconditional logistic regression analysis were used to identify the high risk factors for NPH. The univariate analysis showed that compared with the control group, the case group had significantly higher incidence rates of gestational diabetes and cholestasis in mothers, cesarean delivery, gestational age <34 weeks, 5-minute Apgar score ≤5, birth weight <2 500 g, heart failure and disseminated intravascular coagulation (DIC) before the development of NPH, partial pressure of oxygen/fraction of inspired oxygen (oxygenation index, OI) ≤100, and a reduction in mean platelet volume. The multivariate logistic regression analysis showed that DIC, heart failure, and OI ≤100 were independent risk factors for NPH (OR=33.975, 3.975, 1.818 respectively; P<0.05). Heart failure, OI ≤100, and DIC are risk factors for the development of NPH in the NICU of the municipal hospital.

  11. Acute renal failure in critically ill newborns increases the risk of death: a prospective observational study from India.

    Science.gov (United States)

    Patel, Ankur; Sharma, Deepak; Shastri, Sweta; Sharma, Pradeep

    2016-09-01

    To determine the incidence and risk factors of acute renal failure (ARF) in hospitalized critically ill neonates and analyze outcome of all neonates with renal failure in relation to risk factors. In this prospective observational study 815 infants were enrolled. Renal profile (blood urea and serum creatinine) was done after 12 h of life (or at the time of admission for outborn babies) and then every 12 hourly. Daily 24 h urine output was evaluated. Incidence of renal failure in critically ill neonates was 10.67%. Out of 87 ARF neonates 52 (60%) expired. Mortality in the renal failure group was significantly higher in comparison to control group (p renal failure was more common than oliguric renal failure, but mortality in the oliguric renal failure group was more. Neonatal sepsis was the most common cause of ARF. Eight neonates underwent peritoneal dialysis (PD) out of which there were seven neonatal deaths. Prognosis of neonates with ARF requiring PD was very poor. It can thus be concluded that the health care personal should do rapid diagnosis of ARF in neonates with potential risk factors and also goal at an early and effective treatment of these risk factors in neonates with ARF.

  12. Dosing antibiotics in neonates: review of the pharmacokinetic data.

    Science.gov (United States)

    Rivera-Chaparro, Nazario D; Cohen-Wolkowiez, Michael; Greenberg, Rachel G

    2017-09-01

    Antibiotics are often used in neonates despite the absence of relevant dosing information in drug labels. For neonatal dosing, clinicians must extrapolate data from studies for adults and older children, who have strikingly different physiologies. As a result, dosing extrapolation can lead to increased toxicity or efficacy failures in neonates. Driven by these differences and recent legislation mandating the study of drugs in children and neonates, an increasing number of pharmacokinetic studies of antibiotics are being performed in neonates. These studies have led to new dosing recommendations with particular consideration for neonate body size and maturation. Herein, we highlight the available pharmacokinetic data for commonly used systemic antibiotics in neonates.

  13. Identification of novel mutations in HFE, HFE2, TfR2, and SLC40A1 genes in Chinese patients affected by hereditary hemochromatosis.

    Science.gov (United States)

    Wang, Yongwei; Du, Yali; Liu, Gang; Guo, Shanshan; Hou, Bo; Jiang, Xianyong; Han, Bing; Chang, Yanzhong; Nie, Guangjun

    2017-04-01

    Hereditary hemochromatosis (HH) is a group of inherited iron-overload disorders associated with pathogenic defects in the genes encoding hemochromatosis (HFE), hemojuvelin (HJV/HFE2), hepcidin (HAMP), transferrin receptor 2 (TfR2), and ferroportin (FPN1/SLC40A1) proteins, and the clinical features are well described. However, there have been only a few detailed reports of HH in Chinese populations. Thus, there is insufficient patient information for population-based analyses in Chinese populations or comparative studies among different ethical groups. In the current work, we describe eight Chinese cases of hereditary hemochromatosis. Gene sequencing results revealed eight mutations (five novel mutations) in HFE, HFE2, TfR2, and SLC40A1 genes in these Chinese HH patients. In addition, we used Polymorphism Phenotyping v2 (Polyphen), Sorting Intolerant From Tolerant (SIFT), and a sequence alignment program to predict the molecular consequences of missense mutations.

  14. The implementation of neonatal peritoneal dialysis in a clinical setting.

    Science.gov (United States)

    Unal, Sevim; Bilgin, Leyla; Gunduz, Mehmet; Uncu, Nermin; Azili, Mujdem Nur; Tiryaki, Tugrul

    2012-10-01

    To investigate etiology, outcome and complications related to neonatal peritoneal dialysis (PD). Neonates treated with PD in our neonatal intensive care unit during 2007-2010 were analyzed retrospectively. Among 4036 hospitalized neonates; 20 neonates (0.5%) who underwent 21 cycles of PD [7 preterm, 13 term; 13 female, 7 male] were included. The mean birth weight was 2930.2 ± 720.6 g (1120-4570), mean gestational age was 37.5 ± 3.5 weeks (27-41). The etiologic disorders included inborn errors of metabolism (propionic acidemia, methylmalonic acidemia, citrullinemia, glutaric aciduria type 2, maple syrup urine disease, 10), or acute renal failure secondary to perinatal asphyxia (4), sepsis (2), prematurity (2), hypoplastic left heart syndrome (1), kernicterus (1). The complications included peritonitis (2), early leakage (4), hemorrhage (1), catheter removal (3) and occlusion (2). The mortality rate was 50%. The gestational ages and birth weights of surviving neonates were higher (p neonates, chronic renal failure (1), severe (4) and moderate neuromotor impairment (2) developed within 4-43 months. PD, although invasive, is an effective therapy in neonates. The complexity and invasiveness of the procedure is probably responsible for high rate of complications and mortality. If appropriate catheter selection and technique in the placement should be done, PD might improve outcome.

  15. Hemochromatosis (HFE gene mutations in Brazilian chronic hemodialysis patients

    Directory of Open Access Journals (Sweden)

    F.V. Perícole

    2005-09-01

    Full Text Available Patients with chronic renal insufficiency (CRI have reduced hemoglobin levels, mostly as a result of decreased kidney production of erythropoietin, but the relation between renal insufficiency and the magnitude of hemoglobin reduction has not been well defined. Hereditary hemochromatosis is an inherited disorder of iron metabolism. The importance of the association of hemochromatosis with treatment for anemia among patients with CRI has not been well described. We analyzed the frequency of the C282Y and H63D mutations in the HFE gene in 201 Brazilian individuals with CRI undergoing hemodialysis. The analysis of the effects of HFE mutations on iron metabolism and anemia with biochemical parameters was possible in 118 patients of this study (hemoglobin, hematocrit, ferritin levels, transferrin saturation, and serum iron. A C282Y heterozygous mutation was found in 7/201 (3.4% and H63D homozygous and heterozygous mutation were found in 2/201 (1.0% and 46/201 (22.9%, respectively. The allelic frequencies of the HFE mutations (0.017 for C282Y mutation and 0.124 for H63D mutation did not differ between patients with CRI and healthy controls. Regarding the biochemical parameters, no differences were observed between HFE heterozygous and mutation-negative patients, although ferritin levels were not higher among patients with the H63D mutation (P = 0.08. From what we observed in our study, C282Y/H63D HFE gene mutations are not related to degrees of anemia or iron stores in CRI patients receiving intravenous iron supplementation (P > 0.10. Nevertheless, the present data suggest that the H63D mutation may have an important function as a modulating factor of iron overload in these patients.

  16. Clinical penetrance in hereditary hemochromatosis: estimates of the cumulative incidence of severe liver disease among HFE C282Y homozygotes.

    Science.gov (United States)

    Grosse, Scott D; Gurrin, Lyle C; Bertalli, Nadine A; Allen, Katrina J

    2018-04-01

    Iron overload (hemochromatosis) can cause serious, symptomatic disease that is preventable if detected early and managed appropriately. The leading cause of hemochromatosis in populations of predominantly European ancestry is homozygosity of the C282Y variant in the HFE gene. Screening of adults for iron overload or associated genotypes is controversial, largely because of a belief that severe phenotypes are uncommon, although cascade testing of first-degree relatives of patients is widely endorsed. We contend that severe liver disease (cirrhosis or hepatocellular cancer) is not at all uncommon among older males with hereditary hemochromatosis. Our review of the published data from a variety of empirical sources indicates that roughly 1 in 10 male HFE C282Y homozygotes is likely to develop severe liver disease during his lifetime unless iron overload is detected early and treated. New evidence from a randomized controlled trial of treatment allows for evidence-based management of presymptomatic patients. Although population screening for HFE C282Y homozygosity faces multiple barriers, a potentially effective strategy for increasing the early detection and prevention of clinical iron overload and severe disease is to include HFE C282Y homozygosity in lists of medically actionable gene variants when reporting the results of genome or exome sequencing.

  17. State of the art. Neonatal respiratory failure.

    Science.gov (United States)

    Parker, L A

    1999-12-01

    Advances in ventilatory management of respiratory distress in the newborn have made dramatic strides during the last decade. Innovative treatments such as PTV, HFV, liquid ventilation, and NO therapy are just beginning to have an impact on the care of neonates in the NICU. These treatment modalities should continue to have an effect on the care of the newborn infant well into the future.

  18. Intestinal Obstruction in Early Neonatal Period: A 3-Year Review Of ...

    African Journals Online (AJOL)

    BACKGROUND: Failure to pass meconium by a full-term neonate within the first 24 hours should raise a suspicion of bowel obstruction. The objective of this study was to determine pattern of presentation, diagnosis and outcome of management of intestinal obstruction in the early neonatal period in the Neonatal Care Unit ...

  19. Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

    Directory of Open Access Journals (Sweden)

    Hyun Jin Kim

    2017-03-01

    Full Text Available Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9.7% and 12,910 ng/mL (normal range, 20–320 ng/mL, respectively. The 24-hour urine C-peptide level was normal with negative antiglutamic acid decarboxylase antibody. Subsequently, metformin and an iron-chelating agent were administered. However, an intensive insulin regimen was necessary 2 years after the onset of diabetes. Subject 2, who was diagnosed with severe aplastic anemia at 2 years of age, received multiple blood transfusions until she underwent haploidentical PBSCT at 13 years of age. At 11 years of age, she developed diabetes mellitus with a high serum ferritin level (12,559.8 ng/mL. She is currently 18 years old and has been treated with an intensive insulin regimen and estrogen/progesterone replacement therapy because of hypogonadotropic hypogonadism. It is presumed that the loss of insulin secretory capacity and insulin resistance played a role in the pathogenesis of diabetes mellitus due to hemochromatosis in these cases.

  20. Urinary excretion of biomarkers of oxidatively damaged DNA and RNA in hereditary hemochromatosis

    DEFF Research Database (Denmark)

    Broedbaek, Kasper; Poulsen, Henrik E; Weimann, Allan

    2009-01-01

    Oxidatively generated damage to nucleic acids is considered to play a significant role in carcinogenesis, and it has been shown that people with hereditary hemochromatosis are at increased risk of cancer. In this study we used a new refined liquid chromatography-tandem mass spectrometry method...... of the iron overload seen in this disease. By this mechanism cellular damage resulting in end organ damage, typically seen in the liver of such patients, may be mediated....

  1. Prevalence of C282Y, H63D, and S65C mutations in hereditary HFE-hemochromatosis gene in Lithuanian population.

    Science.gov (United States)

    Kucinskas, Laimutis; Juzenas, Simonas; Sventoraityte, Jurgita; Cedaviciute, Ruta; Vitkauskiene, Astra; Kalibatas, Vytenis; Kondrackiene, Jurate; Kupcinskas, Limas

    2012-04-01

    HFE-hemochromatosis is a common autosomal recessive disease caused by HFE gene mutations and characterized as iron overload and failure of different organs. The aim of this study was to determine the prevalence of C282Y (c.845 G>A), H63D (c.187 C>G), and S65C (c.193A>T) alleles of HFE gene in the Lithuanian population. One thousand and eleven healthy blood donors of Lithuanian nationality were examined in four different ethnic Lithuanian regions to determine HFE gene alleles and genotype frequencies. The samples of DNA were analyzed for the presence of restriction fragment length polymorphism and validated by DNA sequencing. Among 1,011 blood donors tested, the frequency of C282Y, H63D, and S65C alleles were 2.6%, 15.9%, and 1.9%, respectively. One third of the tested subjects (n = 336) had at least one of the C282Y or H63D HFE gene mutations. The screening of Lithuanian blood donors has detected 13 (1.3%) subjects with a genotype C282Y/C282Y or C282Y/H63D responsible for the development of HFE-hemochromatosis. The prevalence of C282Y mutation was significantly higher among the inhabitants of Zemaitija (Somogitia) at the Baltic Sea area (5.9%) in comparison to the regions of continental part of Lithuania (2.4% in Dzukija, 2.3% in Aukstaitija, and 2% in Suvalkija, p HFE gene mutations in ethnic Lithuanians showed that the frequencies of H63D, C282Y, and S65C of HFE gene alleles are similar to the other North-Eastern Europeans, especially in the Baltic region (Estonia, Latvia), Poland, and part of Russia (Moscow region).

  2. Absorption of manganese and iron in a mouse model of hemochromatosis.

    Directory of Open Access Journals (Sweden)

    Jonghan Kim

    Full Text Available Hereditary hemochromatosis, an iron overload disease associated with excessive intestinal iron absorption, is commonly caused by loss of HFE gene function. Both iron and manganese absorption are regulated by iron status, but the relationships between the transport pathways of these metals and how they are affected by HFE-associated hemochromatosis remain poorly understood. Loss of HFE function is known to alter the intestinal expression of DMT1 (divalent metal transporter-1 and Fpn (ferroportin, transporters that have been implicated in absorption of both iron and manganese. Although the influence of HFE deficiency on dietary iron absorption has been characterized, potential effects on manganese metabolism have yet to be explored. To investigate the role of HFE in manganese absorption, we characterized the uptake and distribution of the metal in Hfe (-/- knockout mice after intravenous, intragastric, and intranasal administration of (54Mn. These values were compared to intravenous and intragastric administration of (59Fe. Intestinal absorption of (59Fe was increased and clearance of injected (59Fe was also increased in Hfe(-/- mice compared to controls. Hfe (-/- mice displayed greater intestinal absorption of (54Mn compared to wild-type Hfe(+/+ control mice. After intravenous injection, the distribution of (59Fe to heart and liver was greater in Hfe (-/- mice but no remarkable differences were observed for (54Mn. Although olfactory absorption of (54Mn into blood was unchanged in Hfe (-/- mice, higher levels of intranasally-instilled (54Mn were associated with Hfe(-/- brain compared to controls. These results show that manganese transport and metabolism can be modified by HFE deficiency.

  3. HLA haplotype map of river valley populations with hemochromatosis traced through five centuries in Central Sweden.

    Science.gov (United States)

    Olsson, K Sigvard; Ritter, Bernd; Hansson, Norbeth; Chowdhury, Ruma R

    2008-07-01

    The hemochromatosis mutation, C282Y of the HFE gene, seems to have originated from a single event which once occurred in a person living in the north west of Europe carrying human leukocyte antigen (HLA)-A3-B7. In descendants of this ancestor also other haplotypes appear probably caused by local recombinations and founder effects. The background of these associations is unknown. Isolated river valley populations may be fruitful for the mapping of genetic disorders such as hemochromatosis. In this study, we try to test this hypothesis in a study from central Sweden where the haplotyope A1-B8 was common. HLA haplotypes and HFE mutations were studied in hemochromatosis patients with present or past parental origin in a sparsely populated (1/km(2)) rural district (n = 8366 in the year of 2005), in central Sweden. Pedigrees were constructed from the Swedish church book registry. Extended haplotypes were studied to evaluate origin of recombinations. There were 87 original probands, 36 females and 51 males identified during 30 yr, of whom 86% carried C282Y/C282Y and 14% C282Y/H63D. Of 32 different HLA haplotypes A1-B8 was the most common (34%), followed by A3-B7 (16%), both in strong linkage disequilibrium with controls, (P females. River valley populations may contain HLA haplotypes reflecting their demographic history. This study has demonstrated that the resistance against recombinations between HLA-A and HFE make HLA haplotypes excellent markers for population movements. Founder effects and genetic drift from bottleneck populations (surviving the plague?) may explain the commonness of the mutation in central Scandinavia. The intergenerational time difference >30 yr was greater than expected and means that the age of the original mutation may be underestimated.

  4. Neonatal hypoxia, hippocampal atrophy, and memory impairment: evidence of a causal sequence.

    Science.gov (United States)

    Cooper, Janine M; Gadian, David G; Jentschke, Sebastian; Goldman, Allan; Munoz, Monica; Pitts, Georgia; Banks, Tina; Chong, W Kling; Hoskote, Aparna; Deanfield, John; Baldeweg, Torsten; de Haan, Michelle; Mishkin, Mortimer; Vargha-Khadem, Faraneh

    2015-06-01

    Neonates treated for acute respiratory failure experience episodes of hypoxia. The hippocampus, a structure essential for memory, is particularly vulnerable to such insults. Hence, some neonates undergoing treatment for acute respiratory failure might sustain bilateral hippocampal pathology early in life and memory problems later in childhood. We investigated this possibility in a cohort of 40 children who had been treated neonatally for acute respiratory failure but were free of overt neurological impairment. The cohort had mean hippocampal volumes (HVs) significantly below normal control values, memory scores significantly below the standard population means, and memory quotients significantly below those predicted by their full scale IQs. Brain white matter volume also fell below the volume of the controls, but brain gray matter volumes and scores on nonmnemonic neuropsychological tests were within the normal range. Stepwise linear regression models revealed that the cohort's HVs were predictive of degree of memory impairment, and gestational age at treatment was predictive of HVs: the younger the age, the greater the atrophy. We conclude that many neonates treated for acute respiratory failure sustain significant hippocampal atrophy as a result of the associated hypoxia and, consequently, show deficient memory later in life. © The Author 2013. Published by Oxford University Press.

  5. Incidência de insuficiência renal aguda na Unidade de Terapia Intensiva Neonatal de um hospital paulista Incidencia de insuficiencia renal crónica aguda en la Unidad de Cuidados Intensivos Neonatal de un hospital de Sao Paulo Incidence of acute renal failure in the Neonatal Intensive Care Unit of a hospital in São Paulo

    Directory of Open Access Journals (Sweden)

    Renato Ribeiro Nogueira Ferraz

    2009-01-01

    .OBJECTIVES: To describe the incidence of acute renal failure (ARF in the neonatal intensive care unit (NICU of a hospital in São Paulo and to verify the use of the "risk of renal failure, injury to the kidney, failure of kidney function, loss of kidney function and end-stage renal failure (RIFLE" classification for the allocation of the neonates. METHODS: Review of medical records of neonates from April 4 to April 25, 2008. RESULTS: Of the 19 admissions in the NICU, 10% were diagnosed as ARF according to the RIFLE classification. The neonates diagnosed with ARF were referred to the dialysis service. CONCLUSION: Although this study had a very small sample size, the findings indicate that ARF represents 10% of the primary diagnosis among our sample of neonates admitted to the NICU. Large and longer studies are necessary to evaluate the incidence of ARF in the NICU.

  6. Tmprss6 is a genetic modifier of the Hfe-hemochromatosis phenotype in mice

    Science.gov (United States)

    Whittlesey, Rebecca L.; Andrews, Nancy C.

    2011-01-01

    The hereditary hemochromatosis protein HFE promotes the expression of hepcidin, a circulating hormone produced by the liver that inhibits dietary iron absorption and macrophage iron release. HFE mutations are associated with impaired hepatic bone morphogenetic protein (BMP)/SMAD signaling for hepcidin production. TMPRSS6, a transmembrane serine protease mutated in iron-refractory iron deficiency anemia, inhibits hepcidin expression by dampening BMP/SMAD signaling. In the present study, we used genetic approaches in mice to examine the relationship between Hfe and Tmprss6 in the regulation of systemic iron homeostasis. Heterozygous loss of Tmprss6 in Hfe−/− mice reduced systemic iron overload, whereas homozygous loss caused systemic iron deficiency and elevated hepatic expression of hepcidin and other Bmp/Smad target genes. In contrast, neither genetic loss of Hfe nor hepatic Hfe overexpression modulated the hepcidin elevation and systemic iron deficiency of Tmprss6−/− mice. These results indicate that genetic loss of Tmprss6 increases Bmp/Smad signaling in an Hfe-independent manner that can restore Bmp/Smad signaling in Hfe−/− mice. Furthermore, these results suggest that natural genetic variation in the human ortholog TMPRSS6 might modify the clinical penetrance of HFE-associated hereditary hemochromatosis, raising the possibility that pharmacologic inhibition of TMPRSS6 could attenuate iron loading in this disorder. PMID:21355094

  7. A family with hereditary hemochromatosis carrying HFE gene splice site mutation: a case report

    Directory of Open Access Journals (Sweden)

    NING Huibin

    2017-01-01

    Full Text Available ObjectiveTo investigate a new type of HFE gene mutation in a family with hereditary hemochromatosis (HH. MethodsThe analysis of HFE gene was performed for one patient with a confirmed diagnosis of HH and five relatives. Blood genomic DNA was extracted and PCR multiplication was performed for the exon and intron splice sequences of related HFE, HJV, HAMP, transferrin receptor 2 (TfR2, and SLC40A1 genes. After agarose gel electrophoresis and purification, bi-directional direct sequencing was performed to detect mutation sites. ResultsThe proband had abnormal liver function and increases in serum iron, total iron binding capacity, serum ferritin, and transferrin saturation, as well as T→C homozygous mutation in the fourth base of intron 2 in the intervening sequence of the exon EXON2 of HFE gene (IVs 2+4T→C, C/C homozygous, splicing, abnormal. There were no abnormalities in HJV, HAMP, TfR2, and SLC40A1 genes. The proband′s son had the same homozygous mutation, three relatives had heterozygous mutations, and one relative had no abnormal mutations. ConclusionGene detection plays an important role in the diagnosis of hemochromatosis, and IVs 2+4T→C mutation may be a new pathogenic mutation for HH in China.

  8. Neonatal Bartter Syndrome in association with congenital adrenal hyperplasia in a neonate - a rare combination.

    Science.gov (United States)

    Hussain, Shabbir

    2016-05-01

    Neonatal Bartter syndrome (NBS) is an autosomal recessive renal tubulopathy characterized by hypokalaemic, hypochloraemic metabolic alkalosis associated with increased urinary loss of sodium, potassium, calcium and chloride. There is hyperreninaemia and hyperaldosteronaemia but normotension. Congenital adrenal hyperplasia (CAH), another autosomal recessive condition, may present in the neonatal period with vomiting, hypovolaemia, failure to gain weight or ambiguous genitalia. We report a case of NBS and CAH combination in a neonate. A male neonate born at term was admitted with history of recurrent vomiting and dehydration episodes. Investigations revealed electrolytes imbalance, metabolic alkalosis, raised aldosterone and renin levels suggestive of NBS. He was treated successfully and discharged. He was re-admitted with the same symptoms. Further evaluation confirmed the presence of CAH as well. We report this case because of the rarity of this combination (NBS plus CAH) and to the best of our knowledge this is the first such case report from Pakistan.

  9. Neonatal Cholestasis - Single Centre Experience in Central India.

    Science.gov (United States)

    Jain, Mayank; Adkar, Sagar; Waghmare, Chandrashekhar; Jain, Jenisha; Jain, Shikhar; Jain, Kamna; Passi, Gouri Rao; Vinay, Rashmi Shad; Soni, M K

    2016-01-01

    Neonatal cholestasis syndrome (NCS) is a major cause of morbidity and mortality in infants. The disorder has rarely been studied in centers from Central India. To study the prevalence, clinical presentation and etiology of NCS at a tertiary referral center in Central India. The study was carried out at a tertiary referral center in Central India. The study is a descriptive study. The records of all patients with suspected NCS treated in the Department of Pediatrics from 2007-2012 were analyzed. One hundred and sixty-eight children had a provisional diagnosis of NCS. The complete records of 100 children were available for the study. The median age of presentation was 78 days (range 15-270 days). The male: female ratio was 1.17:1. The clinical features noted were- jaundice (100/100,100%), failure to thrive (73,73%), organomegaly (68, 68%), acholic stools (38,38%), abdominal distention (52,52%) and poor feeding (29, 29%). The etiology as confirmed by investigations is as follows- neonatal hepatitis (20,20%), idiopathic neonatal hepatitis (18,18%), biliary atresia (41,41%), sepsis (14,14%) and others (7,7%). The proportion of NCS in our group of patients was 1.2 per 1000 patients. Jaundice, organomegaly and failure to thrive are the common presentations. Biliary atresia, neonatal hepatitis and idiopathic neonatal hepatitis were the common etiological factors at our center.

  10. Frequency of the Hemochromatosis Gene (HFE Variants in a Jordanian Arab Population and in Diabetics from the Same Region

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    Asem Alkhateeb

    2009-01-01

    Full Text Available Hereditary HFE-linked hemochromatosis is a frequent recessive disorder among individuals of northern European ancestry. The clinical characteristic of this disease is the gradual accumulation of iron in internal organs, which ultimately may lead to organ damage and death. Three allelic variants of HFE gene have been correlated with hereditary hemochromatosis: C282Y is significantly associated with hereditary hemochromatosis in populations of Celtic origin, H63D and S65C are associated with milder form of iron overload. In this study we performed mutation analysis to identify allele frequency of the three variants of HFE gene in Jordanian Arab population, to assess deviations of these frequencies from those detected elsewhere, and to determine if there is an increased frequency of these variants in a diabetic population (Type 2 diabetes from the same area. DNA was extracted from blood samples of 440 individuals attending King Abdullah University Hospital for ambulatory services. We used polymerase chain reaction (PCR to amplify exons 2 and 4 of the HFE gene then restriction fragment length polymorphism (RFLP method to detect the variants. There were neither homozygous nor heterozygous for C282Y variant. For the H63D variant, 0.68% were homozygous and 21.1% were heterozygous. For the S65C variant, there were no homozygous and 0.23% were heterozygous. Allelic frequencies were, 0%, 11.25%, and 0.11% for C282Y, H63D, and S65C, respectively. Our samples were subdivided into two categories of type 2 diabetic (89 cases and controls (blood donors, 204 cases and compared with regard to the H63D variant. Both groups did not have homozygous H63D variant. H63D heterozygous in diabetics were 23.60% and in blood donor controls 22.55%. Allelic frequency of the mutant H63D allele was 11.80% in diabetics and 11.27% for the blood donor controls. This is the first study to show the frequency of the three hemochromatosis gene variants in Jordan with the interesting

  11. Absorption of Manganese and Iron in a Mouse Model of Hemochromatosis

    Science.gov (United States)

    Kim, Jonghan; Buckett, Peter D.; Wessling-Resnick, Marianne

    2013-01-01

    Hereditary hemochromatosis, an iron overload disease associated with excessive intestinal iron absorption, is commonly caused by loss of HFE gene function. Both iron and manganese absorption are regulated by iron status, but the relationships between the transport pathways of these metals and how they are affected by HFE-associated hemochromatosis remain poorly understood. Loss of HFE function is known to alter the intestinal expression of DMT1 (divalent metal transporter-1) and Fpn (ferroportin), transporters that have been implicated in absorption of both iron and manganese. Although the influence of HFE deficiency on dietary iron absorption has been characterized, potential effects on manganese metabolism have yet to be explored. To investigate the role of HFE in manganese absorption, we characterized the uptake and distribution of the metal in Hfe −/− knockout mice after intravenous, intragastric, and intranasal administration of 54Mn. These values were compared to intravenous and intragastric administration of 59Fe. Intestinal absorption of 59Fe was increased and clearance of injected 59Fe was also increased in Hfe−/− mice compared to controls. Hfe −/− mice displayed greater intestinal absorption of 54Mn compared to wild-type Hfe+/+ control mice. After intravenous injection, the distribution of 59Fe to heart and liver was greater in Hfe −/− mice but no remarkable differences were observed for 54Mn. Although olfactory absorption of 54Mn into blood was unchanged in Hfe −/− mice, higher levels of intranasally-instilled 54Mn were associated with Hfe−/− brain compared to controls. These results show that manganese transport and metabolism can be modified by HFE deficiency. PMID:23705020

  12. HFE C282Y/H63D compound heterozygotes are at low risk of hemochromatosis-related morbidity.

    Science.gov (United States)

    Gurrin, Lyle C; Bertalli, Nadine A; Dalton, Gregory W; Osborne, Nicholas J; Constantine, Clare C; McLaren, Christine E; English, Dallas R; Gertig, Dorota M; Delatycki, Martin B; Nicoll, Amanda J; Southey, Melissa C; Hopper, John L; Giles, Graham G; Anderson, Gregory J; Olynyk, John K; Powell, Lawrie W; Allen, Katrina J

    2009-07-01

    The risk of hemochromatosis-related morbidity is unknown among HFE compound heterozygotes (C282Y/H63D). We used a prospective population-based cohort study to estimate the prevalence of elevated iron indices and hemochromatosis-related morbidity for compound heterozygotes. In all, 31,192 subjects of northern European descent were genotyped for HFE C282Y and H63D. An HFE-genotype stratified random sample of 1,438 subjects, followed for an average of 12 years to a mean age of 65 years, completed questionnaires and gave blood. Clinical examinations were blinded to HFE genotype. A total of 180 (84 males) clinically examined C282Y/H63D participants were compared with 330 (149 males) controls with neither HFE mutation; 132 (65 males) and 270 (122 males), respectively, had serum iron measures at both timepoints. Mean serum ferritin (SF) and transferrin saturation (TS) were significantly greater for male and female compound heterozygotes than for wild-types at baseline and follow-up (all P females who were premenopausal at baseline, where SF was similar in both genotype groups. For subjects with serum measures from both baseline and follow-up, mean SF and TS levels did not change significantly for men or for postmenopausal women, but for premenopausal women SF levels increased from 43 to 109 microg/L for compound heterozygotes and from 35 to 64 microg/L for wild-types (both P female compound heterozygotes had a similar prevalence of hemochromatosis-related morbidity to wild-types. One of 82 males and zero of 95 females had documented iron overload-related disease. For male compound heterozygotes, mean iron indices do not change during middle age but for female compound heterozygotes menopause results in increased mean SF. Although compound heterozygotes might maintain elevated iron indices during middle age, documented iron overload-related disease is rare.

  13. [Predictive study of HBsAg in different stages of neonatal venous blood on failure of blocking HBV mother to infant transmission].

    Science.gov (United States)

    Yi, Wei; Li, Ming-Hui; Hu, Yu-Hong; Liu, Feng; Zhang, Yang-Li; Liu, Xue-Jing; Hao, Hong-Xiao; Song, Shu-Jing; Liu, Ying; Li, Xing-Hong; Sun, Ji-Yun; Liu, Min; Cheng, Jun; Xie, Yao

    2011-10-01

    In this study, we discuss the predictive value of different content of HBsAg in different stages of neotal venous blood on failure of blocking mother to infant transmission of HBV. 150 infants born of chronically HBV infected mothers who were positive of both HBsAg and HBeAg and who also had a HBV DNA virus load above 10(5) copies/ml were enrolled. These infants were given hepatitis B virus immune globin (HBIG) 200 IU immediately after birth and were given hepatitis B vaccine 10 or 20 microg at brith, 1 month and 6 months after birth. HBV serological index of these infants were test at birth, 1 month and 7 months after birth respectively. Different content of HBsAg in different stages of neonatal venus blood were analyzed to predict the failure of blocking mother to infant transmission of HBV. 11 infants failed in blocking of HBV mother to infant transmission. The positive rate of HBsAg at birth, 1 month and 7 months after birth were 41.26%, 10.49% and 7.69% respectively, and were 97.90%, 65.73% and 13.29% of HBeAg. The positive predictive value of HBsAg > or = 0.05 and HBsAg > or = 1 IU/ml at birth were 18.64% and 70% respectively, and were 73.33% and 100% one month after birth. Infants with HBsAg > or = 1 IU/ml at birth should be suspicious of failure on blocking HBV mother-to-infant transmission and it should be more credible if the infant has HBsAg > or = 1 IU/ml one month after birth. How to improve the blocking rate of neonates who were positive of HBsAg at birth and one month after birth should be the focus of our future research.

  14. Prevalence of alpha-1 antitrypsin deficiency and hereditary hemochromatosis gene mutations in Algarve, Portugal

    OpenAIRE

    Barreto da Silva, Marta; Gaio, Vânia; Fernandes, Aida; Mendonça, Francisco; Horta Correia, Filomena; Beleza, Álvaro; Gil, Ana Paula; Bourbon, Mafalda; Vicente, A.M.; Dias, Carlos Matias

    2012-01-01

    Alpha-1 antitrypsin (AAT) deficiency and hereditary hemochromatosis (HH) are two of the most fatal genetic disorders in adult life, affecting million individuals worldwide. They are often under-diagnosed conditions and diagnosis is only made when the patient is already in the advanced stages of damage. AAT deficiency results from mutations in one highly pleiomorphic gene located on chromosome 14, SERPINA 1, being Z and S mutations the most relevant clinically. These mutations will lead to an ...

  15. Neonatal cholestasis - Single centre experience in Central India

    Directory of Open Access Journals (Sweden)

    Mayank Jain

    2016-01-01

    Full Text Available Background: Neonatal cholestasis syndrome (NCS is a major cause of morbidity and mortality in infants. The disorder has rarely been studied in centers from Central India. Objectives: To study the prevalence, clinical presentation and etiology of NCS at a tertiary referral center in Central India. Materials and Methods: The study was carried out at a tertiary referral center in Central India. The study is a descriptive study. The records of all patients with suspected NCS treated in the Department of Pediatrics from 2007−2012 were analyzed. Results: One hundred and sixty-eight children had a provisional diagnosis of NCS. The complete records of 100 children were available for the study. The median age of presentation was 78 days (range 15−270 days. The male: female ratio was 1.17:1. The clinical features noted were- jaundice (100/100,100%, failure to thrive (73,73%, organomegaly (68, 68%, acholic stools (38,38%, abdominal distention (52,52% and poor feeding (29, 29%. The etiology as confirmed by investigations is as follows- neonatal hepatitis (20,20%, idiopathic neonatal hepatitis (18,18%, biliary atresia (41,41%, sepsis (14,14% and others (7,7%. Conclusions: The proportion of NCS in our group of patients was 1.2 per 1000 patients. Jaundice, organomegaly and failure to thrive are the common presentations. Biliary atresia, neonatal hepatitis and idiopathic neonatal hepatitis were the common etiological factors at our center.

  16. Primary hemochromatosis: anatomic and physiologic characteristics of the cardiac ventricles and their response to phlebotomy

    International Nuclear Information System (INIS)

    Dabestani, A.; Child, J.S.; Henze, E.; Perloff, J.K.; Schon, H.; Figueroa, W.G.; Schelbert, H.R.; Thessomboon, S.

    1984-01-01

    M-mode and 2-dimensional echocardiography and gated equilibrium blood pool imaging (rest and exercise) were used in 10 patients with primary hemochromatosis to characterize the spectrum of pathophysiologic abnormalities of the cardiac ventricles and to determine the response to chronic therapeutic phlebotomy. Dilated and restrictive cardiomyopathic patterns were identified in 1 patient each, but our data do not permit conclusions on when in the natural history a given pattern becomes overt. On entry into study, 3 patients had normal ventricles and 7 had ventricular abnormalities on echocardiography and blood pool angiography. In 2 of the latter patients, biventricular dysfunction and increased left ventricular (LV) mass normalized after phlebotomy; 1 patient achieved a normal LV response to exercise. Of the 4 patients with isolated abnormal LV ejection fraction responses to exercise, the EF normalized in 2 after phlebotomy. In 1 patient, isolated right ventricular enlargement and dysfunction (echocardiographic and radionuclide imaging) normalized after phlebotomy. Thus, primary hemochromatosis can effect LV and RV size and function; clinically occult cardiac involvement can be identified by echocardiography and equilibrium blood pool imaging; therapeutic phlebotomy can ameliorate or reverse the deleterious effects of excess cardiac iron deposition which appears to exert its harm, at least in part, by a mechanism other than irreversible connective tissue replacement

  17. Neonatal thyrotoxicosis presenting as persistent pulmonary hypertension

    Science.gov (United States)

    Obeid, Rawad; Kalra, Vaneet Kumar; Arora, Prem; Quist, Felix; Moltz, Kathleen C; Chouthai, Nitin Shashikant

    2012-01-01

    Neonatal hyperthyroidism is a rare condition caused either by transplacental passage of thyroid-stimulating immunoglobulins from a mother with Graves’ disease or by activating mutations of the thyrotropin receptors and α-subunit of G-protein. The clinical features may vary. We report a case of neonatal thyrotoxicosis in an infant born to a mother with Graves’ disease, who presented with cardiorespiratory failure and persistent pulmonary hypertension (PPHN). PPHN resolved with specific antithyroid treatment and extracorporeal membrane oxygenation was not required. PMID:22669869

  18. HFE Cys282Tyr homozygotes with serum ferritin concentrations below 1000 microg/L are at low risk of hemochromatosis.

    Science.gov (United States)

    Allen, Katrina J; Bertalli, Nadine A; Osborne, Nicholas J; Constantine, Clare C; Delatycki, Martin B; Nisselle, Amy E; Nicoll, Amanda J; Gertig, Dorota M; McLaren, Christine E; Giles, Graham G; Hopper, John L; Anderson, Gregory J; Olynyk, John K; Powell, Lawrie W; Gurrin, Lyle C

    2010-09-01

    Hemochromatosis gene (HFE)-associated hereditary hemochromatosis (HH) is a genetic predisposition to iron overload and subsequent signs and symptoms of disease that potentially affects approximately 80,000 persons in Australia and almost 1 million persons in the United States. Most clinical cases are homozygous for the Cys282Tyr (C282Y) mutation in the HFE gene, with serum ferritin (SF) concentration >1000 microg/L as the strongest predictor of cirrhosis. The optimal treatment regimen for those with SF concentrations above the normal range but aged 40-69 years. An HFE-stratified random sample of 1438 participants including all C282Y homozygotes with iron studies 12 years apart were examined by physicians blinded to participants' HFE genotype. All previously undiagnosed C282Y homozygotes (35 male, 67 female) and all HFE wild-types (131 male, 160 female) with baseline and follow-up SF concentrations age when disease would be expected to have developed. These observations have implications for the management of C282Y homozygotes.

  19. Causes of Acute Intranatal and Postnatal Hypoxia in Neonatal Infants

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    S. A. Perepelitsa

    2012-01-01

    Full Text Available Objective: to study the causes of acute intranatal hypoxia and reveal a relationship of placental changes to respiratory failure (RF in newborn infants. Subjects and methods. The investigation included 252 neonates with the complicated course of an early neonatal period. Their gestational age was 26 weeks to 40 weeks, birth weight varied from 850 g to 4100 g. 95.3% of the newborn infants were born with a low Apgar score and RF, which required mechanical ventilation immediately after birth. The neonatal status was clinically evaluated; the values of blood gas composition and acid-base balance were recorded; the pathogen was discharged from the tracheobronchial tree; chest X-ray survey and placental morphological examination were performed. Results. The main cause of neonatal respiratory failure is chronic intrauterine hypoxia caused by placental inflammatory changes and fetal-placental blood circulatory disorders, which gives rise to preterm delivery, cerebral hemodynamic disorders, and neonatal amniotic fluid aspiration. Bacteriological examination of tracheobronchial aspirations showed that no microflora growth occured in the majority of the newborns acute intranatal hypoxia. Enterococcus faecalis and Staphylococcus epidermidis were isolated in 12.3% and 8.7%, respectively. Growth of в-hemolytic streptococcus was observed in 2.8% of cases. The rate of microbial association specific only for rate premature infants with neonatal respiratory distress syndrome (NRDS was 4.8%. Conclusion. Placental changes causing fetal-placental circulatory disorders were ascertained to be responsible for acute intranatal and postnatal neonatal hypoxia. Placental inflammatory changes occurred in the majority of cases, as confirmed by bacteriological examinations of neonatal infants. Isolation of the varying microbial flora in infants with RF to a greater extent is, indicative of the infectious process occurring in the maternal body. Key words: acute intranatal

  20. Combined segregation and linkage analysis of genetic hemochromatosis using affection status, serum iron, and HLA.

    OpenAIRE

    Borecki, I B; Lathrop, G M; Bonney, G E; Yaouanq, J; Rao, D C

    1990-01-01

    Characterizing the distribution of parameters of iron metabolism by hemochromatosis genotype remains an important goal vis-à-vis potential screening strategies to identify individuals at genetic risk, since a specific marker to detect the abnormal gene has not been identified as yet. In the present investigation, we analyze serum iron values in ascertained families using a method which incorporates both segregation of the clinical affection status and the HLA linkage information to identify t...

  1. HEMOCHROMATOSIS

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    Frijo jose

    2011-11-01

    Full Text Available 36 year old male with no history of blood transfusion in the past, presented with features of left heart failure and presyncope. He had sparse facial, axillary and pubic hair, hyperpigmentation of skin, palms, palate and history of impotence.

  2. Neonatal marfan syndrome: report of two cases.

    Science.gov (United States)

    Ghandi, Yazdan; Zanjani, Keyhan S; Mazhari-Mousavi, Seyed-Eshagh; Parvaneh, Nima

    2013-02-01

    Neonatal Marfan syndrome is a rare and severe phenotype of this disease. A poor prognosis is anticipated due to the high probability of congestive heart failure, and mitral and tricuspid regurgitations with suboptimal response to medical therapy and difficulties in surgical management at an early age. We present two consecutive patients with this disease who are the first reported cases from Iran to the best of our knowledge. Unfortunately both of them died shortly after diagnosis. Neonatal Marfan syndrome is reported from Iran and has a poor prognosis like the patients reported from elsewhere.

  3. Neonatal Graves' Disease with Maternal Hypothyroidism.

    Science.gov (United States)

    Akangire, Gangaram; Cuna, Alain; Lachica, Charisse; Fischer, Ryan; Raman, Sripriya; Sampath, Venkatesh

    2017-07-01

    Neonatal Graves' disease presenting as conjugated hyperbilirubinemia is a diagnostic challenge because the differential includes a gamut of liver and systemic diseases. We present a unique case of neonatal Graves' disease in a premature infant with conjugated hyperbilirubinemia born to a mother with hypothyroidism during pregnancy and remote history of Graves' disease. Infant was treated with a combination of methimazole, propranolol, and potassium iodide for 4 weeks. Thyroid function improved after 8 weeks of treatment with full recovery of thyroid function, disappearance of thyroid-stimulating antibodies, and resolution of failure to thrive and conjugated hyperbilirubinemia. This case provides several clinical vignettes as it is a rare, severe, presentation of an uncommon neonatal disease, signs, symptoms, and clinical history presented a diagnostic challenge for neonatologists and endocrinologists, normal newborn screen was misleading, and yet timely treatment led to a full recovery.

  4. Appendicoumbilical Fistula: A Rare Reason for Neonatal Umbilical Mass

    OpenAIRE

    Cevik, M.; Boleken, M. E.; Kadıoglu, E.

    2011-01-01

    The normal umbilicus is a simple structure, but the intrauterine development of the umbilicus is highly complex. Neonatal umbilical mass anomalies usually represent failure of obliteration of the vitelline duct or the allantois which results in persistence of remnants, which can lead to a wide variety of disorders. In this paper, we present a case of an appendicoumbilical fistula in a neonate along with the differential diagnosis and management options. Embryologic explanation of the etiology...

  5. Inhaled PGE1 in neonates with hypoxemic respiratory failure: two pilot feasibility randomized clinical trials.

    Science.gov (United States)

    Sood, Beena G; Keszler, Martin; Garg, Meena; Klein, Jonathan M; Ohls, Robin; Ambalavanan, Namasivayam; Cotten, C Michael; Malian, Monica; Sanchez, Pablo J; Lakshminrusimha, Satyan; Nelin, Leif D; Van Meurs, Krisa P; Bara, Rebecca; Saha, Shampa; Das, Abhik; Wallace, Dennis; Higgins, Rosemary D; Shankaran, Seetha

    2014-12-12

    Inhaled nitric oxide (INO), a selective pulmonary vasodilator, has revolutionized the treatment of neonatal hypoxemic respiratory failure (NHRF). However, there is lack of sustained improvement in 30 to 46% of infants. Aerosolized prostaglandins I2 (PGI2) and E1 (PGE1) have been reported to be effective selective pulmonary vasodilators. The objective of this study was to evaluate the feasibility of a randomized controlled trial (RCT) of inhaled PGE1 (IPGE1) in NHRF. Two pilot multicenter phase II RCTs are included in this report. In the first pilot, late preterm and term neonates with NHRF, who had an oxygenation index (OI) of ≥15 and <25 on two arterial blood gases and had not previously received INO, were randomly assigned to receive two doses of IPGE1 (300 and 150 ng/kg/min) or placebo. The primary outcome was the enrollment of 50 infants in six to nine months at 10 sites. The first pilot was halted after four months for failure to enroll a single infant. The most common cause for non-enrollment was prior initiation of INO. In a re-designed second pilot, co-administration of IPGE1 and INO was permitted. Infants with suboptimal response to INO received either aerosolized saline or IPGE1 at a low (150 ng/kg/min) or high dose (300 ng/kg/min) for a maximum duration of 72 hours. The primary outcome was the recruitment of an adequate number of patients (n = 50) in a nine-month-period, with fewer than 20% protocol violations. No infants were enrolled in the first pilot. Seven patients were enrolled in the second pilot; three in the control, two in the low-dose IPGE1, and two in the high-dose IPGE1 groups. The study was halted for recruitment futility after approximately six months as enrollment targets were not met. No serious adverse events, one minor protocol deviation and one pharmacy protocol violation were reported. These two pilot RCTs failed to recruit adequate eligible newborns with NHRF. Complex management RCTs of novel therapies for persistent pulmonary

  6. Losartan in combination with propranolol slows the aortic root dilatation in neonatal Marfan syndrome

    Directory of Open Access Journals (Sweden)

    Lu-Hang Liu

    2018-04-01

    Full Text Available Neonatal Marfan syndrome, in contrast to classical Marfan syndrome, is characterized by rapidly progressive multi-valvular cardiac disease and death from congestive heart failure, typically within the first year of life. Due to the rarity of this condition, treatment for neonatal Marfan syndrome has not been well studied. In this report, a combination of losartan and propranolol reduced the aortic root dilatation rate after three months of losartan therapy. Genetic analysis in this patient revealed a mutation in exon 25 of the FBN1 gene, which typically results in a shorter life expectancy. However, the patient's heart failure was controlled by losartan, propranolol and other anti-congestive medications, which may have prolonged his survival. Key Words: FBN1, losartan, neonatal Marfan syndrome

  7. Implementation of Bubble CPAP in a Rural Ugandan Neonatal ICU.

    Science.gov (United States)

    McAdams, Ryan M; Hedstrom, Anna B; DiBlasi, Robert M; Mant, Jill E; Nyonyintono, James; Otai, Christine D; Lester, Debbie A; Batra, Maneesh

    2015-03-01

    Respiratory distress is a leading cause of neonatal death in low-income and middle-income countries. CPAP is a simple and effective respiratory support modality used to support neonates with respiratory failure and can be used in low-income and middle-income countries. The goal of this study was to describe implementation of the Silverman-Andersen respiratory severity score (RSS) and bubble CPAP in a rural Ugandan neonatal NICU. We sought to determine whether physicians and nurses in a low-income/middle-income setting would assign similar RSS in neonates after an initial training period and over time. We describe the process of training NICU staff to use the RSS to assist in decision making regarding initiation, titration, and termination of bubble CPAP for neonates with respiratory distress. Characteristics of all neonates with respiratory failure treated with bubble CPAP in a rural Ugandan NICU from January to June 2012 are provided. Nineteen NICU staff members (4 doctors and 15 nurses) received RSS training. After this, the Spearman correlation coefficient for respiratory severity scoring between doctor and nurse was 0.73. Twenty-one infants, all CPAP, with 17 infants starting on the day of birth. The majority of infants (16/21, 76%) were preterm, 10 (48%) were CPAP, 5.2 ± 2.3 after 2-4 h of CPAP, 4.9 ± 2.7 after 12-24 h of CPAP, and 3.5 ± 1.9 before CPAP was discontinued. Duration of treatment with CPAP averaged 79 ± 43 h. Approximately half (11/21, 52%) of infants treated with CPAP survived to discharge. Implementing bubble CPAP in a low-income/middle-income setting is feasible. The RSS may be a simple and useful tool for monitoring a neonate's respiratory status and for guiding CPAP management. Copyright © 2015 by Daedalus Enterprises.

  8. Neonatal Marfan Syndrome: Report of a Case with an Inherited Splicing Mutation outside the Neonatal Domain.

    Science.gov (United States)

    Le Gloan, Laurianne; Hauet, Quentin; David, Albert; Hanna, Nadine; Arfeuille, Chloé; Arnaud, Pauline; Boileau, Catherine; Romefort, Bénédicte; Benbrik, Nadir; Gournay, Véronique; Joram, Nicolas; Baron, Olivier; Isidor, Bertrand

    2016-02-01

    We report a child and her mother affected by Marfan syndrome. The child presented with a phenotype of neonatal Marfan syndrome, revealed by acute and refractory heart failure, finally leading to death within the first 4 months of life. Her mother had a common clinical presentation. Genetic analysis revealed an inherited FBN1 mutation. This intronic mutation (c.6163+3_6163+6del), undescribed to date, leads to exon 49 skipping, corresponding to in-frame deletion of 42 amino acids (p.Ile2014_Asp2055del). FBN1 next-generation sequencing did not show any argument for mosaicism. Association in the same family of severe neonatal and classical Marfan syndrome illustrates the intrafamilial phenotype variability.

  9. Neonatal Marfan syndrome: Report of two cases.

    Science.gov (United States)

    Jurko, Tomas; Jurko, Alexander; Minarik, Milan; Micieta, Vladimir; Tonhajzerova, Ingrid; Kolarovszka, Hana; Zibolen, Mirko

    2017-07-01

    Marfan syndrome is rarely diagnosed in the neonatal period because of variable expression and age-dependent appearance of clinical signs. The prognosis is usually poor due to high probability of congestive heart failure, mitral and tricuspid regurgitations with suboptimal response to medical therapy and difficulties in surgical management. The authors have studied two cases of Marfan syndrome in the newborn period. Two cases of neonatal Marfan syndrome, one male and one female, were diagnosed by characteristic physical appearance. Both infants had significant cardiovascular abnormalities diagnosed by ultrasonography. Genetic DNA analysis in the second case confirmed the mutations in the fibrillin-1 gene located on chromosome 15q21 which is responsible for the development of Marfan syndrome. The boy died at six weeks of age with signs of rapidly progressive left ventricular failure associated with pneumonia. The second infant was having only mild signs of congestive heart failure and has been treated with beta blockers. At the age of 4 years her symptoms of congestive heart failure had worsened due to progression of mitral and tricuspid insufficiency and development of significant cardiomegaly. Mitral and tricuspid valvuloplasy had to be done at that time. Early diagnosis of Marfan syndrome in the newborn period can allow treatment in the early stages of cardiovascular abnormalities and may improve the prognosis. It also helps to explain to the family the serious health problem of their child.

  10. The hemochromatosis protein HFE 20 years later: An emerging role in antigen presentation and in the immune system.

    Science.gov (United States)

    Reuben, Alexandre; Chung, Jacqueline W; Lapointe, Réjean; Santos, Manuela M

    2017-09-01

    Since its discovery, the hemochromatosis protein HFE has been primarily defined by its role in iron metabolism and homeostasis, and its involvement in the genetic disease termed hereditary hemochromatosis (HH). While HH patients are typically afflicted by dysregulated iron levels, many are also affected by several immune defects and increased incidence of autoimmune diseases that have thereby implicated HFE in the immune response. Growing evidence has supported an immunological role for HFE with recent studies describing HFE specifically as it relates to MHC I antigen presentation. Here, we present a comprehensive overview of the relationship between iron metabolism, HFE, and the immune system to better understand the origin and cause of immune defects in HH patients. We further describe the role of HFE in MHC I antigen presentation and its potential to impair autoimmune responses in homeostatic conditions, a mechanism which may be exploited by tumors to evade immune surveillance. Overall, this increased understanding of the role of HFE in the immune response sets the stage for better treatment and management of HH and other iron-related diseases, as well as of the immune defects related to this condition. © 2017 The Authors. Immunity, Inflammation and Disease Published by John Wiley & Sons Ltd.

  11. Genetic disruption of NRF2 promotes the development of necroinflammation and liver fibrosis in a mouse model of HFE-hereditary hemochromatosis.

    Science.gov (United States)

    Duarte, Tiago L; Caldas, Carolina; Santos, Ana G; Silva-Gomes, Sandro; Santos-Gonçalves, Andreia; Martins, Maria João; Porto, Graça; Lopes, José Manuel

    2017-04-01

    In hereditary hemochromatosis, iron deposition in the liver parenchyma may lead to fibrosis, cirrhosis and hepatocellular carcinoma. Most cases are ascribed to a common mutation in the HFE gene, but the extent of clinical expression is greatly influenced by the combined action of yet unidentified genetic and/or environmental modifying factors. In mice, transcription factor NRF2 is a critical determinant of hepatocyte viability during exposure to acute dietary iron overload. We evaluated if the genetic disruption of Nrf2 would prompt the development of liver damage in Hfe -/- mice (an established model of human HFE-hemochromatosis). Wild-type, Nrf2 -/- , Hfe -/- and double knockout (Hfe/Nrf2 -/- ) female mice on C57BL/6 genetic background were sacrificed at the age of 6 (young), 12-18 (middle-aged) or 24 months (old) for evaluation of liver pathology. Despite the parenchymal iron accumulation, Hfe -/- mice presented no liver injury. The combination of iron overload (Hfe -/- ) and defective antioxidant defences (Nrf2 -/- ) increased the number of iron-related necroinflammatory lesions (sideronecrosis), possibly due to the accumulation of toxic oxidation products such as 4-hydroxy-2-nonenal-protein adducts. The engulfment of dead hepatocytes led to a gradual accumulation of iron within macrophages, featuring large aggregates. Myofibroblasts recruited towards the injury areas produced substantial amounts of collagen fibers involving the liver parenchyma of double-knockout animals with increased hepatic fibrosis in an age-dependent manner. The genetic disruption of Nrf2 promotes the transition from iron accumulation (siderosis) to liver injury in Hfe -/- mice, representing the first demonstration of spontaneous hepatic fibrosis in the long term in a mouse model of hereditary hemochromatosis displaying mildly elevated liver iron. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

  12. The Southern French registry of genetic hemochromatosis: a tool for determining clinical prevalence of the disorder and genotype penetrance.

    Science.gov (United States)

    Aguilar-Martinez, Patricia; Bismuth, Michael; Blanc, François; Blanc, Pierre; Cunat, Severine; Dereure, Olivier; Dujols, Pierre; Giansily-Blaizot, Muriel; Jorgensen, Christian; Konate, Amadou; Larrey, Dominique; Le Quellec, Alain; Mura, Thibault; Raingeard, Isabelle; Ramos, Jeanne; Renard, Eric; Rousseau, Florence; Schved, Jean-François; Picot, Marie-Christine

    2010-04-01

    Despite great progress in understanding the mechanisms underlying genetic hemochromatosis, data on the prevalence and the penetrance of the disorder are conflicting. A registry of patients with genetic hemochromatosis was established in the South of France and a regional health network was developed to allow the inclusion of all the diagnosed patients. C282Y homozygous patients classified in stages 2 (biological iron overload), 3 and 4 (clinical manifestations of iron overload, stage 4 being the more severe) according to the classification of the French National Authority for Health were included in the registry over a 6-year period. A total of 352 symptomatic C282Y homozygotes were identified, resulting in a total prevalence of 1.83 per 10,000 (95% CI: 1.63 to 2.02) in subjects over 20 years and 2.40 per 10,000 (95% CI, 2.15 to 2.65) among subjects of European descent. Among Europeans, the total calculated penetrance was 15.8% in stage 2 or higher, 12.1% in stage 3 or 4 and 2.9% in stage 4. The penetrance was slightly higher in males (18.7%) than in females (13.2%). It was 19.9% for individuals over 40 years of age (24.1% and 16.3% in males and females, respectively) with a maximum of 31% in subjects between 50 and 54 years old. Among 249 patients with complete records, 24% were in stage 2, the majority (58%) were in stage 3, and 18% in stage 4. There was a higher proportion of males, and excessive alcohol intake was more prevalent in stage 4 than in stages 2 and 3 combined. A French Mediterranean regional hemochromatosis registry with strict inclusion criteria is a useful tool for characterizing the history of this disease, particularly for the most severely affected patients, as defined by the disease severity classification. The total prevalence of symptomatic C282Y homozygotes in the region was found to be low. However, clinical penetrance (stages 3 and 4) was not negligible.

  13. A novel technique in airway management of neonates with occipital encephalocele.

    Science.gov (United States)

    Rangaswamy, N; Pramanik, A K

    2014-11-01

    Airway stabilization in neonates with occipital encephalocele (OE) is critical during surgery or if they develop hypoxic-respiratory failure. Endotracheal intubation can be challenging due to difficulty in positioning the head in a patient with large occipital mass. We describe a novel technique for positioning neonates with large OE using a commonly used hospital apparatus which facilitated appropriate positioning of the baby and successful endotracheal intubation with ease and no additional staff.

  14. Appendicoumbilical Fistula: A Rare Reason for Neonatal Umbilical Mass

    Directory of Open Access Journals (Sweden)

    M. Cevik

    2011-01-01

    Full Text Available The normal umbilicus is a simple structure, but the intrauterine development of the umbilicus is highly complex. Neonatal umbilical mass anomalies usually represent failure of obliteration of the vitelline duct or the allantois which results in persistence of remnants, which can lead to a wide variety of disorders. In this paper, we present a case of an appendicoumbilical fistula in a neonate along with the differential diagnosis and management options. Embryologic explanation of the etiology was discussed with the possible association with different forms of malpositioning and rotation of the gut.

  15. Appendicoumbilical fistula: a rare reason for neonatal umbilical mass.

    Science.gov (United States)

    Cevik, M; Boleken, M E; Kadıoglu, E

    2011-01-01

    The normal umbilicus is a simple structure, but the intrauterine development of the umbilicus is highly complex. Neonatal umbilical mass anomalies usually represent failure of obliteration of the vitelline duct or the allantois which results in persistence of remnants, which can lead to a wide variety of disorders. In this paper, we present a case of an appendicoumbilical fistula in a neonate along with the differential diagnosis and management options. Embryologic explanation of the etiology was discussed with the possible association with different forms of malpositioning and rotation of the gut.

  16. Comparison between late-presenting and isolated neonatal ...

    African Journals Online (AJOL)

    Results: Isolated neonatal cases presented more often with acute respiratory symptoms (n=25; P= 0.016) and failure to thrive (n= 38; P= 0.03). Late-presenting cases presented more often with chronic respiratory symptoms (n=14; P= 0.0044) or gastrointestinal symptoms (n=12; P= 0.006). Thirty-five cases with minor or ...

  17. Three cases of neonatal tetanus in Papua New Guinea lead to development of national action plan for maternal and neonatal tetanus elimination.

    Science.gov (United States)

    Datta, Siddharta Sankar; Barnabas, Roland; Sitther, Adeline; Guarenti, Laura; Toikilik, Steven; Kariwiga, Grace; Sui, Gerard Pai

    2013-01-01

    Maternal or neonatal tetanus causes deaths primarily in Asia and Africa and is usually the result of poor hygiene during delivery. In 2011, three neonatal tetanus cases were investigated in Papua New Guinea, and all three cases were delivered at home by untrained assistants. The babies were normal at birth but subsequently developed spasms. A neonatal tetanus case must be viewed as a sentinel event indicating a failure of public health services including immunization, antenatal care and delivery care. The confirmation of these cases led to the drafting of the Papua New Guinea National Action Plan for Maternal and Neonatal Tetanus Elimination. This included three rounds of a tetanus toxoid supplementary immunization campaign targeting women of childbearing age (WBCA) and strengthening of other clean delivery practices. The first immunization round was conducted in April and May 2012, targeting 1.6 million WBCA and achieved coverage of 77%. The government of Papua New Guinea should ensure detailed investigation of all neonatal tetanus cases reported in the health information system and perform subprovincial analysis of tetanus toxoid coverage following completion of all three immunization rounds. Efforts also should be made to strengthen clean delivery practices to help eliminate maternal and neonatal tetanus in Papua New Guinea.

  18. Three cases of neonatal tetanus in Papua New Guinea lead to development of national action plan for maternal and neonatal tetanus elimination

    Directory of Open Access Journals (Sweden)

    Grace Kariwiga

    2013-06-01

    Full Text Available Maternal or neonatal tetanus causes deaths primarily in Asia and Africa and is usually the result of poor hygiene during delivery. In 2011, three neonatal tetanus cases were investigated in Papua New Guinea, and all three cases were delivered at home by untrained assistants. The babies were normal at birth but subsequently developed spasms. A neonatal tetanus case must be viewed as a sentinel event indicating a failure of public health services including immunization, antenatal care and delivery care. The confirmation of these cases led to the drafting of the Papua New Guinea National Action Plan for Maternal and Neonatal Tetanus Elimination. This included three rounds of a tetanus toxoid supplementary immunization campaign targeting women of childbearing age (WBCA and strengthening of other clean delivery practices. The first immunization round was conducted in April and May 2012, targeting 1.6 million WBCA and achieved coverage of 77%. The government of Papua New Guinea should ensure detailed investigation of all neonatal tetanus cases reported in the health information system and perform sub-provincial analysis of tetanus toxoid coverage following completion of all three immunization rounds. Efforts also should be made to strengthen clean delivery practices to help eliminate maternal and neonatal tetanus in Papua New Guinea.

  19. Surfactant from neonatal to pediatric ICU: bench and bedside evidence.

    Science.gov (United States)

    Boet, A; Brat, R; Aguilera, S S; Tissieres, P; De Luca, D

    2014-12-01

    Surfactant is a cornerstone of neonatal critical care for the treatment of respiratory distress syndrome of preterm babies. However, other indications have been studied for various clinical conditions both in term neonates and in children beyond neonatal age. A high degree of evidence is not yet available in some cases and this is due to the complex and not yet totally understood physiopathology of the different types of pediatric and neonatal lung injury. We here summarise the state of the art of the bench and bedside knowledge about surfactant use for the respiratory conditions usually cared for in neonatal and pediatric intensive care units. Future research direction will also be presented. On the whole, surfactant is able to improve oxygenation in infection related respiratory failure, pulmonary hemorrhage and meconium aspiration syndrome. Bronchoalveolar lavage with surfactant solution is currently the only means to reduce mortality or need for extracorporeal life support in neonates with meconium aspiration. While surfactant bolus or lavage only improves the oxygenation and ventilatory requirements in other types of postneonatal acute respiratory distress syndrome (ARDS), there seems to be a reduction in the mortality of small infants with RSV-related ARDS.

  20. Bubble CPAP versus ventilator CPAP in preterm neonates with early onset respiratory distress--a randomized controlled trial.

    Science.gov (United States)

    Tagare, Amit; Kadam, Sandeep; Vaidya, Umesh; Pandit, Anand; Patole, Sanjay

    2013-04-01

    Bubble continuous positive airway pressure (BCPAP) is a low cost nasal CPAP delivery system with potential benefits to developing nations. To compare the efficacy and safety of BCPAP with ventilator-derived CPAP (VCPAP) in preterm neonates with respiratory distress. In a randomized controlled trial, preterm neonates with Silverman-Anderson score ≥ 4 and oxygen requirement >30% within first 6 h of life were randomly allocated to BCPAP or VCPAP. Proportion of neonates with success or failure was compared. In all, 47 of 57 (82.5%) neonates from BCPAP group and 36 of 57 (63.2%) neonates from the VCPAP group completed CPAP successfully (p = 0.03). Neonates who failed CPAP had higher Silverman-Anderson score (p neonates with early onset respiratory distress, with comparable safety.

  1. HFE mutations and hemochromatosis in Danish patients admitted for HFE genotyping

    DEFF Research Database (Denmark)

    Koefoed, P; Dalhoff, K; Dissing, J

    2002-01-01

    Analysis of the common C282Y and H63D mutations in the HFE gene is widely used to diagnose hereditary hemochromatosis (HH). The aim of this study was to evaluate the efficiency with which different hospitals and general practitioners select patients for HH genotype and to determine the distribution...... of HFE mutations in such patients. Nine hundred unrelated patients from Danish hospitals and general practitioners (group A) and 69 consecutive patients from a specialized liver unit (group B) were examined for HFE substitutions using multiplex real-time polymerase chain reaction. In group A we found 13...... in the H63D homozygotes or S65C heterozygotes. Moreover, 7 wild-type patients, 2 C282Y heterozygote patients and one H63D heterozygote patient fulfilled the criteria for HH. The significant enrichment of HH among associated genotype samples submitted for HFE testing indicates that the clinical selection...

  2. Eighty percent of French sport winners in Olympic, World and Europeans competitions have mutations in the hemochromatosis HFE gene.

    Science.gov (United States)

    Hermine, Olivier; Dine, Gérard; Genty, Vincent; Marquet, Laurie-Anne; Fumagalli, Gabriela; Tafflet, Muriel; Guillem, Flavia; Van Lierde, Françoise; Rousseaux-Blanchi, Marie-Philippe; Palierne, Christian; Lapostolle, Jean-Claude; Cervetti, Jean-Pierre; Frey, Alain; Jouven, Xavier; Noirez, Philippe; Toussaint, Jean-François

    2015-12-01

    The HFE gene encodes a protein involved in iron homeostasis; individuals with mutations in both alleles develop hemochromatosis. 27% of the French population is heterozygous for mutations in this gene. We found that 80% of the French athletes who won international competitions in rowing, Nordic skiing and judo display mutations in one allele of HFE, thus demonstrating the existence of a favourable phenotype linked to this heterozygosity. Copyright © 2015. Published by Elsevier B.V.

  3. Anti-obesity and pro-diabetic effects of hemochromatosis.

    Science.gov (United States)

    Abbas, Mousa Al; Abraham, Deveraprabu; Kushner, James P; McClain, Donald A

    2014-10-01

    Levels of tissue iron contribute to determining diabetes risk, but little is known about the effects of higher iron levels on weight, and on the interaction of weight and iron overload on diabetes risk. Therefore, the effect of iron on body mass index and diabetes in individuals with iron overload from hereditary hemochromatosis (HH), compared to non-HH siblings and historical controls was examined. Chart reviews were performed on a cohort of adults (age ≥40, N = 101) with the common C282Y/C282Y HFE genotype, compared to wild type siblings (N = 32) and comparable NHANES cohorts, with respect to body mass index and diabetes status. Males with HH have lower body mass index (BMI) than control siblings. Females had a trend toward decreased BMI that was not significant, possibly related to decreased degrees of iron overload. In both males and females, increased rates of diabetes were seen, especially in the overweight or obese. High tissue iron levels may be both pro- and anti-diabetic. The prevalence of obesity and diabetes in HH is likely dependent upon the degree of iron overload, caloric intake, and other genetic and environmental factors, contributing to the observed heterogeneity in the frequency of disease-related morbidities in HH. Copyright © 2014 The Obesity Society.

  4. Autoimmune Conditions in 235 Hemochromatosis Probands with HFE C282Y Homozygosity and Their First-Degree Relatives

    Directory of Open Access Journals (Sweden)

    James C. Barton

    2015-01-01

    Full Text Available We performed a retrospective study of autoimmune conditions (ACs in 235 hemochromatosis probands at diagnosis by analyzing age, sex, ACs, history of first-degree family members with ACs (FH, diabetes, heavy ethanol consumption, elevated serum ALT/AST, nonalcoholic fatty liver disease, viral hepatitis, cirrhosis, iron removed to achieve iron depletion (QFe, and positivity for human leukocyte antigen (HLA haplotypes A∗01, B∗08; A∗02, B∗44; A∗03, B∗07; A∗03, B∗14; and A∗29, B∗44. There were 138 men (58.7%. Median followup was 19.6 y. One or more of 19 ACs were diagnosed in each of 35 probands (14.9%. Prevalences of Hashimoto’s thyroiditis, rheumatoid arthritis, and ankylosing spondylitis were 8.1% (95% CI: [5.1, 12.5], 1.7% [0.6, 4.6], and 0.0085 [0.0015, 0.0337], respectively. Eighteen probands (7.7% had a FH. Eight probands with ACs had 9 family members with ACs. In a logistic regression, ACs were less likely in men (odds ratio (OR 0.3 [0.1, 0.6] and more likely in probands with a FH (OR 4.1 [1.4, 11.8]. Overall ACs risk was not significantly associated with QFe or HLA haplotypes. Estimated survival of probands with and without ACs did not differ significantly. We conclude that ACs are common in hemochromatosis probands, especially women and probands with a FH.

  5. Immunological unresponsiveness of the neonatal ruminant to gastrointestinal helminths

    International Nuclear Information System (INIS)

    Soulsby, E.J.L.

    1981-01-01

    Parasitic gastro-enteritis of domestic ruminants is a disease syndrome which is most usually seen in young animals in their first grazing season. Although this may be due, in part, to greater susceptibility of young animals to the pathogenic effects of parasitic infection, there is also good evidence that young animals are less able to mount a satisfactory protective immune response or a response which will reject an existing infection. This phenomenon is exemplified by Haemonchus contortus and Trichostrongylus spp. infection in sheep, but the phenomenon is recognized in other species including neonatal rodents (e.g. rats infected with Nippostrongylus brasiliensis) and has been demonstrated in neonatal cattle infected with Taenia saginata. The present consideration will deal mainly with the failure of lambs to mount an effective immune response to gastrointestinal nematodes during the neonatal period. (author)

  6. CT findings in neonatal hypothermia

    International Nuclear Information System (INIS)

    Schulman, H.; Laufer, L.; Berginer, J.; Hertzanu, Y.; Hershkowitz, E.; Berenstein, T.; Sofer, S.; Maor, E.

    1998-01-01

    Background. Newborn infants are particularly prone to hypothermia, a condition with a high mortality. Objective. To study the CT brain patterns in infants with hypothermia and neurological symptoms. Materials and methods. We reviewed the brain CT of nine infants with neonatal hypothermia, multiple organ failure, seizures and coma. Results. Two infants had normal CT scans, acutely and at follow-up, and were clinically normal at follow-up. In seven infants, CT showed diffuse cerebral oedema, with reversal of the normal density relationship between grey and white matter and a relative increased density of the thalami, brainstem and cerebellum - the 'reversal sign'. In six surviving infants with severe developmental delay, follow-up CT revealed cerebral atrophy with multicystic encephalomalacia. Conclusions. The 'reversal sign' has been described in the abused child, birth asphyxia and anoxia due to drowning. Neonatal hypothermia is offered as a further cause. (orig.)

  7. Coinheritance of hereditary spherocytosis and reversibility of cirrhosis in a young female patient with hereditary hemochromatosis.

    Science.gov (United States)

    Höblinger, A; Erdmann, C; Strassburg, C P; Sauerbruch, T; Lammert, F

    2009-04-16

    Here we report a 33-years-old woman with hereditary spherocytosis and hemochromatosis due to homozygosity for the C282Y mutation of the HFE gene. The coinheritance of both conditions led to severe iron overload and liver cirrhosis at young age. The patient was treated by repeated phlebotomy, and reversibility of cirrhosis was documented by transient elastography. This report discusses the pathophysiology of iron accumulation in patients with hemolytic anemia combined with HFE C282Y homozygosity. The case indicates that patients with hematological disorders characterized by increased erythropoetic activity should be screened for HFE mutations.

  8. Research gaps in neonatal HIV-related care

    Directory of Open Access Journals (Sweden)

    Mary-Ann Davies

    2015-05-01

    Full Text Available The South African prevention of mother to child transmission programme has made excellentprogress in reducing vertical HIV transmission, and paediatric antiretroviral therapyprogrammes have demonstrated good outcomes with increasing treatment initiation inyounger children and infants. However, both in South Africa and across sub-Saharan African,lack of boosted peri-partum prophylaxis for high-risk vertical transmission, loss to followup,and failure to initiate HIV-infected infants on antiretroviral therapy (ART before diseaseprogression are key remaining gaps in neonatal HIV-related care. In this issue of the Southern African Journal of HIV Medicine, experts provide valuable recommendations for addressingthese gaps. The present article highlights a number of areas where evidence is lacking toinform guidelines and programme development for optimal neonatal HIV-related care.

  9. Evidence that the ancestral haplotype in Australian hemochromatosis patients may be associated with a common mutation in the gene.

    OpenAIRE

    Crawford, D H; Powell, L W; Leggett, B A; Francis, J S; Fletcher, L M; Webb, S I; Halliday, J W; Jazwinska, E C

    1995-01-01

    Hemochromatosis (HC) is a common inherited disorder of iron metabolism for which neither the gene nor biochemical defect have yet been identified. The aim of this study was to look for clinical evidence that the predominant ancestral haplotype in Australian patients is associated with a common mutation in the gene. We compared indices of iron metabolism and storage in three groups of HC patients categorized according to the presence of the ancestral haplotype (i.e., patients with two copies, ...

  10. Neonatal Hearing screening in tafila

    International Nuclear Information System (INIS)

    Rashed, K.A.

    2007-01-01

    To measure the true prevalence of hearing impairment in neonates in Tafila, Jordan. This retrospective study was carried out at Prince Zeid Hospital, Taflia, Jordan through analysis of data of all births from January 2005 and January 2006. Transient evoked otoacoustic emissions (TEOAE) were measured via the application of echoprobe to both ears. There were two groups of births that were analysed statistically. Hearing impaired neonates were those with two fails or more in each ear. Normal ones were those with 3 pass or more. Of the 1788 babies in the study group, 1622 (90.7%) were enrolled in the study with 9.3% loss rate. 1512 babies were examined on the 2 day of birth, 2 of them had hearing impairment with a rate of 1.2/1000. 110 babies were screened on the day of discharge from the nursery, one of them with hearing defect with a rate of 5.9/1000. Thus, true prevalence of hearing impaiment or failure was 1.7/1000. We conclude that screening for hearing impairment in the neonatal period is easy, informative and the true prevalence of hearing impairment in Tafila is similar to that in different parts of the world. (author)

  11. Multimodality Renal Failure in a Patient with OEIS Complex

    Directory of Open Access Journals (Sweden)

    Jonathan D. Santoro

    2015-10-01

    Full Text Available Omphalocele-exstrophy of the bladder-imperforate anus-spinal defect (OEIS complex is a rare constellation of clinical abnormalities with wide phenotypic presentation. We describe a case of a preterm neonate with OEIS complex with acute renal failure, and the challenges in diagnosis and management of this patient as renal failure can be a multifactorial process when encountered with this rare complex.

  12. Infection control for SARS in a tertiary neonatal centre.

    Science.gov (United States)

    Ng, P C; So, K W; Leung, T F; Cheng, F W T; Lyon, D J; Wong, W; Cheung, K L; Fung, K S C; Lee, C H; Li, A M; Hon, K L E; Li, C K; Fok, T F

    2003-09-01

    The Severe Acute Respiratory Syndrome (SARS) is a newly discovered infectious disease caused by a novel coronavirus, which can readily spread in the healthcare setting. A recent community outbreak in Hong Kong infected a significant number of pregnant women who subsequently required emergency caesarean section for deteriorating maternal condition and respiratory failure. As no neonatal clinician has any experience in looking after these high risk infants, stringent infection control measures for prevention of cross infection between patients and staff are important to safeguard the wellbeing of the work force and to avoid nosocomial spread of SARS within the neonatal unit. This article describes the infection control and patient triage policy of the neonatal unit at the Prince of Wales Hospital, Hong Kong. We hope this information is useful in helping other units to formulate their own infection control plans according to their own unit configuration and clinical needs.

  13. CT findings in neonatal hypothermia

    Energy Technology Data Exchange (ETDEWEB)

    Schulman, H.; Laufer, L.; Berginer, J.; Hertzanu, Y. [Department of Radiology, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, P. O. Box 151, Beer-Sheva 84101 (Israel); Hershkowitz, E.; Berenstein, T.; Sofer, S. [Pediatric Intensive Care Unit, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva (Israel); Maor, E. [Department of Pathology, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva (Israel)

    1998-06-01

    Background. Newborn infants are particularly prone to hypothermia, a condition with a high mortality. Objective. To study the CT brain patterns in infants with hypothermia and neurological symptoms. Materials and methods. We reviewed the brain CT of nine infants with neonatal hypothermia, multiple organ failure, seizures and coma. Results. Two infants had normal CT scans, acutely and at follow-up, and were clinically normal at follow-up. In seven infants, CT showed diffuse cerebral oedema, with reversal of the normal density relationship between grey and white matter and a relative increased density of the thalami, brainstem and cerebellum - the `reversal sign`. In six surviving infants with severe developmental delay, follow-up CT revealed cerebral atrophy with multicystic encephalomalacia. Conclusions. The `reversal sign` has been described in the abused child, birth asphyxia and anoxia due to drowning. Neonatal hypothermia is offered as a further cause. (orig.) With 6 figs., 1 tab., 13 refs.

  14. Influence of weight at enterostomy reversal on surgical outcomes in infants after emergent neonatal stoma creation.

    Science.gov (United States)

    Talbot, Lindsay J; Sinyard, Robert D; Rialon, Kristy L; Englum, Brian R; Tracy, Elizabeth T; Rice, Henry E; Adibe, Obinna O

    2017-01-01

    Neonates after emergent enterostomy creation frequently require reversal at low weight because of complications including cholestasis, dehydration, dumping, failure to thrive, and failure to achieve enteral independence. We investigated whether stoma reversal at low weight (stoma type, reversal indication, operative details, and complications were examined. Patients were categorized by weight at reversal of less than 2kg, 2.01-2.5kg, 2.51-3.5kg, and greater than 3.5kg. Data were analyzed using univariable and multivariable regression with significance level of pstoma reversal may be acceptable when required for progression of neonatal care. Level III, Treatment Study (Retrospective comparative study). Copyright © 2017. Published by Elsevier Inc.

  15. Pulmonary hypertension and right heart failure due to severe hypernatremic dehydration.

    Science.gov (United States)

    Chiwane, Saurabh; Ahmed, Tageldin M; Bauerfeld, Christian P; Chauhan, Monika

    2017-07-01

    Neonates are at risk of developing hypernatremic dehydration and its associated complications, such as stroke, dural sinus thrombosis and renal vein thrombosis. Pulmonary hypertension has not been described as a complication of hypernatremia. We report a case of a seven-day-old neonate with severe hypernatremic dehydration who went on to develop pulmonary hypertension and right heart failure needing extracorporeal membrane oxygenation (ECMO). Normal or high anion gap metabolic acidosis commonly accompanies hypernatremic dehydration. The presence of acidosis and/or hypoxia can delay the normal drop in pulmonary vascular resistance (PVR) after birth, causing pulmonary hypertension and right ventricular failure. A high index of suspicion is paramount to diagnose pulmonary hypertension and aggressive correction of the acidosis and hypoxia is needed. In the presence of severe right ventricular failure, ECMO can be used as a bridge to recovery while underlying metabolic derangements are being corrected.

  16. Fatal Neonatal Herpes Simplex Infection Likely from Unrecognized Breast Lesions.

    Science.gov (United States)

    Field, Scott S

    2016-02-01

    Type 1 herpes simplex virus (HSV-1) is very prevalent yet in rare circumstances can lead to fatal neonatal disease. Genital acquisition of type 2 HSV is the usual mode for neonatal herpes, but HSV-1 transmission by genital or extragenital means may result in greater mortality rates. A very rare scenario is presented in which the mode of transmission was likely through breast lesions. The lesions were seen by nurses as well as the lactation consultant and obstetrician in the hospital after delivery of the affected baby but not recognized as possibly being caused by herpes. The baby died 9 days after birth with hepatic failure and disseminated intravascular coagulation. Peripartum health care workers need to be aware of potential nongenital (including from the breast[s]) neonatal herpes acquisition, which can be lethal. © The Author(s) 2015.

  17. Losartan in combination with propranolol slows the aortic root dilatation in neonatal Marfan syndrome.

    Science.gov (United States)

    Liu, Lu-Hang; Lin, Shan-Miao; Lin, Dar-Shong; Chen, Ming-Ren

    2018-04-01

    Neonatal Marfan syndrome, in contrast to classical Marfan syndrome, is characterized by rapidly progressive multi-valvular cardiac disease and death from congestive heart failure, typically within the first year of life. Due to the rarity of this condition, treatment for neonatal Marfan syndrome has not been well studied. In this report, a combination of losartan and propranolol reduced the aortic root dilatation rate after three months of losartan therapy. Genetic analysis in this patient revealed a mutation in exon 25 of the FBN1 gene, which typically results in a shorter life expectancy. However, the patient's heart failure was controlled by losartan, propranolol and other anti-congestive medications, which may have prolonged his survival. Copyright © 2017. Published by Elsevier B.V.

  18. A prospective population-based study of maternal, fetal, and neonatal outcomes in the setting of prolonged labor, obstructed labor and failure to progress in low- and middle-income countries.

    Science.gov (United States)

    Harrison, Margo S; Ali, Sumera; Pasha, Omrana; Saleem, Sarah; Althabe, Fernando; Berrueta, Mabel; Mazzoni, Agustina; Chomba, Elwyn; Carlo, Waldemar A; Garces, Ana; Krebs, Nancy F; Hambidge, K; Goudar, Shivaprasad S; Dhaded, S M; Kodkany, Bhala; Derman, Richard J; Patel, Archana; Hibberd, Patricia L; Esamai, Fabian; Liechty, Edward A; Moore, Janet L; Koso-Thomas, Marion; McClure, Elizabeth M; Goldenberg, Robert L

    2015-01-01

    This population-based study sought to quantify maternal, fetal, and neonatal morbidity and mortality in low- and middle-income countries associated with obstructed labor, prolonged labor and failure to progress (OL/PL/FTP). A prospective, population-based observational study of pregnancy outcomes was performed at seven sites in Argentina, Guatemala, India (2 sites, Belgaum and Nagpur), Kenya, Pakistan and Zambia. Women were enrolled in pregnancy and delivery and 6-week follow-up obtained to evaluate rates of OL/PL/FTP and outcomes resulting from OL/PL/FTP, including: maternal and delivery characteristics, maternal and neonatal morbidity and mortality and stillbirth. Between 2010 and 2013, 266,723 of 267,270 records (99.8%) included data on OL/PL/FTP with an overall rate of 110.4/1000 deliveries that ranged from 41.6 in Zambia to 200.1 in Pakistan. OL/PL/FTP was more common in women aged 3500g, and women with a BMI >25 (RR 1.4, 95% CI 1.3 - 1.5), with the suggestion of OL/PL/FTP being less common in preterm deliveries. Protective characteristics included parity of ≥3, having an infant birth vaginally were more likely to become infected, to have an infected neonate, to hemorrhage in the antepartum and postpartum period, and to die, have a stillbirth, or have a neonatal demise. Women with OL/PL/FTP were far more likely to deliver in a facility and be attended by a physician or other skilled provider than women without this diagnosis. Women with OL/PL/FTP in the communities studied were more likely to be primiparous, younger than age 20, overweight, and of higher education, with an infant with birthweight of >3500g. Women with this diagnosis were more likely to experience a maternal, fetal, or neonatal death, antepartum and postpartum hemorrhage, and maternal and neonatal infection. They were also more likely to deliver in a facility with a skilled provider. CS may decrease the risk of poor outcomes (as in the case of antepartum hemorrhage), but unassisted vaginal

  19. Swiss cheese ventricular septal defect with myocarditis - A rare coexistence in a neonate

    Directory of Open Access Journals (Sweden)

    A R Saboo

    2012-01-01

    Full Text Available Myocarditis is defined as acute inflammation of the myocardium, usually following a non-specific flu-like illness, and encompasses a wide range of clinical presentations ranging from mild or subclinical disease to heart failure. We report a 12-day-old healthy full-term neonate who presented with abrupt onset of congestive cardiac failure (CCF following a viral prodrome. Examination revealed persistent sinus tachycardia, lymphocytosis, gross cardiomegaly, nonspecific electrocardiogram changes with echocardiography showing Swiss cheese ventricular septal defect (VSD. VSD alone very rarely presents as early-onset cardiac failure in the absence of other precipitating factors like anemia, sepsis, hypoglycemia etc. Myocarditis, however, can mimic VSD and can present as fulminant cardiac failure in an otherwise healthy newborn. Myocarditis is usually diagnosed based on circumstantial evidence such as a recent viral infection and the sudden onset of cardiac dysfunction while ruling out other diagnostic possibilities. Elevated troponin T level is one of the most crucial noninvasive diagnostic modalities. Several trials have concluded that levels >0.055 ng/ml are statistically significant for diagnosing myocarditis in children. In our case an abrupt onset of cardiac failure following a viral prodrome and markedly elevated cardiac troponin T without sepsis and in the presence of normal coronary anatomy clinched the diagnosis of myocarditis. An early and aggressive treatment for CCF along with regular long-term follow-up plays a key role in the management of myocarditis. Role of high-dose Intravenous immunoglobulin in myocarditis has been studied by many trials with different outcomes. This is the first case report showing coexistence of VSD with myocarditis in a neonate presenting as early-onset acute cardiac failure. The report highlights the importance of screening for myocarditis in all previously normal babies presenting primarily with cardiogenic

  20. Transient spontaneous remission in congenital MLL-AF10 rearranged acute myeloid leukemia presenting with cardiorespiratory failure and meconium ileus.

    Science.gov (United States)

    Gyárfás, Tobias; Wintgens, Juergen; Biskup, Wolfgang; Oschlies, Ilske; Klapper, Wolfram; Siebert, Reiner; Bens, Susanne; Haferlach, Claudia; Meisel, Roland; Kuhlen, Michaela; Borkhardt, Arndt

    2016-12-01

    Neonatal leukemia is a rare disease with an estimated prevalence of about one to five in a million neonates. The majority being acute myeloid leukemia (AML), neonatal leukemia can present with a variety of symptoms including hyperleucocytosis, cytopenia, hepatosplenomegaly, and skin infiltrates. Chromosomal rearrangements including mixed lineage leukemia (MLL) translocations are common in neonatal AML. A female neonate born at 34 weeks gestation presented with cardiorespiratory failure, hepatosplenomegaly, pancytopenia, and coagulopathy. She required intensive care treatment including mechanical ventilation, high-dose catecholamine therapy, and multiple transfusions. Small intestinal biopsy obtained during laparotomy for meconium ileus revealed an infiltrate by an undifferentiated monoblastic, MLL-rearranged leukemia. No other manifestations of leukemia could be detected. After spontaneous clinical remission, lasting 5 months without any specific treatment, the patient presented with leukemia cutis and full-blown monoblastic leukemia. MLL-AF10-rearranged AML could be re-diagnosed and successfully treated with chemotherapy and hematopoietic stem cell transplantation. Our patient exhibited a unique manifestation of neonatal MLL-AF10 rearranged AML with cardiorespiratory failure and intestinal infiltration. It highlights the importance of leukemia in the differential diagnosis of neonatal distress, congenital hematological abnormalities, and skin lesions.

  1. Therapeutic Erythrocytapheresis in the Initial Treatment of Hereditary Hemochromatosis

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    Vít Řeháček

    2012-01-01

    Full Text Available Background: The current treatment of hereditary hemochromatosis (HH consists of performing periodic whole blood phlebotomies. Erythrocytapheresis (EA can remove up to three times more red blood cells per single procedure and could thus have a clinical benefit. A prospective study of 30 consecutive cases of HH were included in a periodic EA program. Methods and patients: EA were performed using a discontinuous flow cell separators. The protocol consisted of a bimonthly EA until normalization of the serum ferritin was reached. The aim was to reduce the total erythrocyte volume by 25–35%, eventually, to adjust the amount so that hematocrit would not drop below 0.25. Results: 530 ± 101 ml of erythrocytes were removed (median 517, range 116–761 ml. Iron depletion (ferritin < 20 μg/l was achieved in all patients after a mean 6.9 ± 7.6 months, median 5 months, range 1–36 months and a mean 14 EA sessions. The procedures were well tolerated and there were no severe side-effects. Conclusions: We conclude that HH patients treated with EA achieved iron depletion quickly under good conditions of tolerance. The efficacy, speed, tolerability, and more favorable schedule of an EA program facilitate treatment of HH.

  2. Neonatal Death

    Science.gov (United States)

    ... Home > Complications & Loss > Loss & grief > Neonatal death Neonatal death E-mail to a friend Please fill in ... cope with your baby’s death. What is neonatal death? Neonatal death is when a baby dies in ...

  3. A Rare Case of Neonatal Hypophosphatasia: A Case Report

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    Nasim Pouralizadeh

    2018-03-01

    Full Text Available Hypophosphatasia is a rare hereditary disorder of bone metabolism.In this article, we presented the case of a male infant with a soft skull and short, deformed limbs at birth, followed by seizures and respiratory distress during admission in the neonatal intensive care unit (NICU. Prenatal ultrasound showed limb hypoplasia, skull hypomineralization, and polyhydramnios. Seizures occurred on day nine of admission. The neonate was intubated after pneumonia on day 12of birth and died due to the same cause and respiratory failure on day 14. Clinical presentation and low alkaline phosphatase (ALP confirmed the diagnosis of hypophosphatasia. The disorder covers a spectrum of severe neonatal type with severe hypomineralization to various adult types with osteomalacia and dental problems. Prenatal hypophosphatasia is diagnosed based on the clinical signs, including soft skull, short limbs, breathing difficulty, seizures, respiratory distress, laboratory results (low ALP and high pyridoxal 5-phosphate, and radiographic findings (hypomineralization and metaphyseal dysplasia.

  4. Effect of C282Y genotype on self-reported musculoskeletal complications in hereditary hemochromatosis.

    Science.gov (United States)

    Camacho, António; Funck-Brentano, Thomas; Simão, Márcio; Cancela, Leonor; Ottaviani, Sébastien; Cohen-Solal, Martine; Richette, Pascal

    2015-01-01

    Arthropathy that mimics osteoarthritis (OA) and osteoporosis (OP) is considered a complication of hereditary hemochromatosis (HH). We have limited data comparing OA and OP prevalence among HH patients with different hemochromatosis type 1 (HFE) genotypes. We investigated the prevalence of OA and OP in patients with HH by C282Y homozygosity and compound heterozygosity (C282Y/H63D) genotype. A total of 306 patients with HH completed a questionnaire. Clinical and demographic characteristics and presence of OA, OP and related complications were compared by genotype, adjusting for age, sex, body mass index (BMI), current smoking and menopausal status. In total, 266 of the 306 patients (87%) were homozygous for C282Y, and 40 (13%) were compound heterozygous. The 2 groups did not differ by median age [60 (interquartile range [IQR] 53 to 68) vs. 61 (55 to 67) years, P=0.8], sex (female: 48.8% vs. 37.5%, P=0.18) or current smoking habits (12.4% vs. 10%, P=0.3). As compared with compound heterozygous patients, C282Y homozygous patients had higher median serum ferritin concentration at diagnosis [1090 (IQR 610 to 2210) vs. 603 (362 to 950) µg/L, P<0.001], higher median transferrin saturation [80% (IQR 66 to 91%) vs. 63% (55 to 72%), P<0.001]) and lower median BMI [24.8 (22.1 to 26.9) vs. 26.2 (23.5 to 30.3) kg/m2, P<0.003]. The overall prevalence of self-reported OA was significantly higher with C282Y homozygosity than compound heterozygosity (53.4% vs. 32.5%; adjusted odds ratio [aOR] 2.4 [95% confidence interval 1.2-5.0]), as was self-reported OP (25.6% vs. 7.5%; aOR 3.5 [1.1-12.1]). Patients with C282Y homozygosity may be at increased risk of musculoskeletal complications of HH.

  5. ACUTE RENAL FAILURE IN THE NEWBORNS HOSPITALIZED AT THE INTENSIVE CARE UNIT, UNIVERSITY CLINICAL CENTRE TUZLA

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    Zulić Evlijana

    2015-03-01

    Full Text Available Introduction: Reasons for acute renal failure in hospitalized infants were sepsis, hypovolemia, asphyxia, respiratory distress syndrome, surgical interventions and congenital heart defects. The aim of this study was to determine the frequency and and main etiologies, and early outcome of neonatal acute renal failure. Materials and Methods: At Intensive Care Unit, Clinical Center Tuzla, from 15. 01. 2013 to 15. 01. 2015 in 21 newborn was diagnosed renal failure, based on the amount of excreted urine and serum creatinine. Results: The prevalence of renal failure was 6.84%, with a higher incidence of female. 33.3% of infants were term neonates. Oliguria was diagnosed in 71.4% of newborns. Sepsis was the most common predisposing factor for the development of renal failure, associated with high mortality. Other causes of renal failure were perinatal hypoxia, RDS, surgical interventions and congenital heart defects. There was a positive correlation between the gestational age of the newborn and serum creatinine. Discussion: Early prevention of risk factors with rapid diagnosis and effective treatment, can affect further outcome of acute renal failure in infants.

  6. Leucine is a major regulator of muscle protein synthesis in neonates

    Science.gov (United States)

    Approximately 10 % of infants born in the United States are of low birth weight. Growth failure during the neonatal period is a common occurrence in low birth weight infants due to their inability to tolerate full feeds, concerns about advancing protein supply, and high nutrient requirements for gro...

  7. Neonatal Hyperglycemia due to Transient Neonatal Diabetes Mellitus in Puerto Rico

    OpenAIRE

    Fargas-Berríos, N.; García-Fragoso, L.; García-García, I.; Valcárcel, M.

    2015-01-01

    Neonatal hyperglycemia is a metabolic disorder found in the neonatal intensive care units. Neonatal diabetes mellitus (NDM) is a very uncommon cause of hyperglycemia in the newborn, occurring in 1 in every 400,000 births. There are two subtypes of neonatal diabetes mellitus: permanent neonatal diabetes mellitus (PNDM) and transient neonatal diabetes mellitus (TNDM). We describe a term, small for gestational age, female neonate with transient neonatal diabetes mellitus who presented with poor ...

  8. Mutations in the HFE, TFR2, and SLC40A1 genes in patients with hemochromatosis.

    Science.gov (United States)

    Del-Castillo-Rueda, Alejandro; Moreno-Carralero, María-Isabel; Cuadrado-Grande, Nuria; Alvarez-Sala-Walther, Luis-Antonio; Enríquez-de-Salamanca, Rafael; Méndez, Manuel; Morán-Jiménez, María-Josefa

    2012-10-15

    Hereditary hemochromatosis causes iron overload and is associated with a variety of genetic and phenotypic conditions. Early diagnosis is important so that effective treatment can be administered and the risk of tissue damage avoided. Most patients are homozygous for the c.845G>A (p.C282Y) mutation in the HFE gene; however, rare forms of genetic iron overload must be diagnosed using a specific genetic analysis. We studied the genotype of 5 patients who had hyperferritinemia and an iron overload phenotype, but not classic mutations in the HFE gene. Two patients were undergoing phlebotomy and had no iron overload, 1 with metabolic syndrome and no phlebotomy had mild iron overload, and 2 patients had severe iron overload despite phlebotomy. The patients' first-degree relatives also underwent the analysis. We found 5 not previously published mutations: c.-408_-406delCAA in HFE, c.1118G>A (p.G373D), c.1473G>A (p.E491E) and c.2085G>C (p.S695S) in TFR2; and c.-428_-427GG>TT in SLC40A1. Moreover, we found 3 previously published mutations: c.221C>T (p.R71X) in HFE; c.1127C>A (p.A376D) in TFR2; and c.539T>C (p.I180T) in SLC40A1. Four patients were double heterozygous or compound heterozygous for the mutations mentioned above, and the patient with metabolic syndrome was heterozygous for a mutation in the TFR2 gene. Our findings show that hereditary hemochromatosis is clinically and genetically heterogeneous and that acquired factors may modify or determine the phenotype. Copyright © 2012. Published by Elsevier B.V.

  9. Hemorrhage is the most common cause of neonatal mortality in patients with sacrococcygeal teratoma.

    Science.gov (United States)

    Kremer, Marijke E B; Wellens, Lianne M; Derikx, Joep P M; van Baren, Robertine; Heij, Hugo A; Wijnen, Marc H W A; Wijnen, René M H; van der Zee, David C; van Heurn, L W Ernest

    2016-11-01

    A small percentage of neonates with sacrococcygeal teratoma die shortly after birth from hemorrhagic complications. The incidence of and risk factors associated with hemorrhagic mortality are unknown. In this multicenter study we determined the incidence of early death in neonates born with SCT and evaluated potential risk factors for hemorrhagic mortality. 235 children with SCT treated from 1970 to 2010 in the Netherlands were retrospectively included. The following candidate risk factors for hemorrhagic mortality were examined: sex, prematurity, Altman type, tumor volume, tumor histology, necessity of emergency operation and time of diagnosis. Eighteen patients (7.7%) died at a median age of 163.5days (range 1.7-973days). Nine patients died of a malignancy. Nine others (3.8%) died postnatally (age 1-27days), six even within two days after birth. In seven of these nine patients death was related to tumor-hemorrhage and/or circulatory failure. Risk factors for hemorrhagic mortality were prematurity, tumor volume>1000cm 3 and performance of an emergency operation. Hemorrhagic mortality of neonates with SCT is relatively high (3.8%) representing almost 70% of the overall mortality in the neonatal period. High-output cardiac failure, internal tumor hemorrhage and perioperative bleeding were the most common causes of early death and were all strongly associated with larger tumor sizes. II (Retrospective study). Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Feasibility of nitric oxide administration by neonatal helmet-CPAP: a bench study.

    Science.gov (United States)

    Trevisanuto, Daniele; Doglioni, Nicoletta; Micaglio, Massimo; Zanardo, Vincenzo

    2007-09-01

    Inhaled nitric oxide (NO) may have a role in the treatment of preterm infants with respiratory failure. We evaluated the feasibility of administering NO therapy by a new continuous positive airway pressure (CPAP) system (neonatal helmet-CPAP). While maintaining a constant total flow of 8, 10, and 12 l.min(-1), NO concentrations were progressively increased to 5, 10, 20, and 40 p.p.m. in the neonatal helmet-CPAP pressure chamber (5 cmH2O). NO, NO2, and O2 concentrations were measured in the pressure chamber and the immediate external environment. In the chamber, NO2 levels remained low (neonatal helmet-CPAP system. This method allows the delivery of accurate NO levels and high O2 concentrations avoiding NO2 accumulation. Further experimental and clinical studies are needed.

  11. Assessment of maternal smoking status during pregnancy and the associations with neonatal outcomes.

    Science.gov (United States)

    Bakker, Rachel; Kruithof, Claudia; Steegers, Eric A P; Tiemeier, Henning; Mackenbach, Johan P; Hofman, Albert; Jaddoe, Vincent W V

    2011-12-01

    Single assessment of smoking during pregnancy may lead to misclassification due to underreporting or failure of smoking cessation. We examined the percentage of mothers who were misclassified in smoking status based on single assessment, as compared with repeated assessment, and whether this misclassification leads to altered effect estimates for the associations between maternal smoking and neonatal complications. This study was performed in 5,389 mothers participating in a prospective population-based cohort study in the Netherlands. Smoking status was assessed 3 times during pregnancy using questionnaires. Information on birth weight and neonatal complications was obtained from hospital records. For categorizing mothers per smoking status, Cohen's Kappa coefficient was .86 (p pregnancy, 1.7% (70 of 4,141) and 33.7% (217 of 643), respectively, were reclassified to continued smoking based on repeated assessment. Younger, shorter lower educated mothers who had non-European ethnicity experienced more stress, consumed more alcohol, and did not use folic acid supplements had higher risk of underreporting their smoking status or failure of smoking cessation. Marginal differences were found on the associations of maternal smoking with neonatal complications between single or repeated assessment. Our results suggest that single assessment of smoking during pregnancy leads to underestimation of the continued smoking prevalence, especially among mothers who reported quitting smoking in first trimester. However, this underestimation does not materially change the effect estimates for the associations between maternal smoking and neonatal outcomes.

  12. Population-based analysis of the frequency of HFE gene polymorphisms: Correlation with the susceptibility to develop hereditary hemochromatosis.

    Science.gov (United States)

    Katsarou, Martha-Spyridoula; Latsi, Rosana; Papasavva, Maria; Demertzis, Nikolaos; Kalogridis, Thodoris; Tsatsakis, Aristides M; Spandidos, Demetrios A; Drakoulis, Nikolaos

    2016-07-01

    Hereditary hemochromatosis (HH) is an autosomal recessive genetic disease, characterized by increased dietary iron absorption. Due to the absence of an effective excretory mechanism, the excess iron in the body may accumulate resulting in toxic effects. The HFE gene also affects the activity of hepcidin, a hormone which acts as a negative regulator of iron metabolism. In this study, we performed a population-based analysis of the distribution of three hemochromatosis-related polymorphisms in the HFE gene (rs1800562, rs1799945 and rs1800730). DNA from 1,446 non‑related individuals of Greek ethnicity was collected and analyzed, either from whole blood or buccal swabs. The frequency distribution of these HFE gene polymorphisms was then determined. The results revealed that in our Greek population cohort (gr) the frequencies of each polymorphism were as follows: rs1800562: GG (wild‑type)=97.0%, GA=1.5%, AA=1.5%; rs1799945: CC (wild‑type)=74.4%, CG=23.4%, GG=2.2%; rs1800730: AA (wild‑type)=98.1%, AT=1.5% and TT=0.4%. No association between the HFE polymorphisms rs1800562, rs1799945 and rs1800730 and gender could be established. As regards the rs1800562 polymorphism, the A allele (mutant) was ~1.8‑fold more frequent in the European population (eur) than in the Greek population [(gr)=2,3%<(eur)=4%]. As for the rs1799945 polymorphism, the G allele (mutant) was 1.2‑fold more frequent in the European population than in the Greek population [(gr)=13,9%<(eur)=17%]. As regards the rs1800730 polymorphism, the T allele (mutant) was ~1.7‑fold more frequent in the European population than in the Greek population [(gr)=1.2%<(eur)=2%]. However, these pathogenic mutations were found more frequently in the Greek population compared to the global population (gl) [rs1800562: (gl)=1%<(gr)=2,3%; rs1799945: (gl)=7%<(gr)=13,9%; rs1800730: (gl)=<1%<(gr)=1.2%]. This suggests that the Greek population may differ genetically from the northern European population

  13. ACUTE RENAL FAILURE IN TERM NEWBORN FOLLOWING PERINATAL ASPHYXIA

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    Emina Hadzimuratovic

    2017-04-01

    Full Text Available Introduction: Perinatal asphyxia (PA results in hypoxic damage to almost all organs, kidneys being most frequently (40% affected. Objectives: was to determine the incidence of acute renal failure (ARF in term neonates with PA and to correlate it with severity of hypoxic ischemic encephalopathy (HIE. Materials and methods: This prospective study of 54 term neonates with PA was performed in tertiary level neonatal intensive care unit at Pediatric Clinic Sarajevo from June 2014 to June 2016. The severe PA was defined as 5. minute Apgar score 1.5 mg/dl (>133 micromol/L on 3rd day of life or urine output 6 hrs beyond 24 hrs of life. Results. Out of 54 neonates with PA, 22 (40.74 % had ARF. Most of them (63.6% had non-oliguric ARF with mean renal output of 2.2 ± 0.5 ml/kg/h. Eight neonates (36.4% had oliguric ARF with mean renal output of 0.35 ± 0.6 ml/kg/h. Most of the neonates with oliguric ARF (63.4% had severe PA while in those with non-oliguric ARF moderate PA was predominant. ARF was highest in the neonates with HIE III (85.71 %. (Figure 1.. This showed that as HIE stage progressed, more renal dysfunction was seen in asphyxiated babies and this difference in incidence was found statistically significant (p<0.05. Conclusions. Neonates with severe PA had more frequent ARF and the predominant type of renal involvement was non oliguric. Neonates with HIE stage II and III had significantly higher incidence of ARF.

  14. Hepatobiliary scan in neonatal Jaundice

    International Nuclear Information System (INIS)

    Nahar, Nurun; Hasan, Mizanul; Karim, M.A.

    2002-01-01

    Jaundice is more or less common in newborn babies. Through physiological jaundice is most common cause of neonatal jaundice, possibility of obstructive jaundice especially biliary atresia should be kept in mind. Early diagnosis of biliary atresia followed by surgical treatment can save baby's life. Otherwise death is inevitable due to liver failure. Hepatobiliary scan is the imaging study of choice in neonatal jaundice especially when there is persistent conjugated hyperbilirubinaemia. Total 27 newborn babies of suspected biliary atresia, aged 14 days to 4 months were referred to Institute of Nuclear Medicine for Hepatobiliary scan. All of them had high serum bilirubin ranged from 6.0 mg/dl with an average of 9.35 ng/dl serum bilirubin level. Ultrasonography of hepatobiliary system was performed in 14 cases showing normal sized liver in 4 cases and hepatomegaly in 10 cases. Hepatobiliary scan was done with 99m Tc-Mebrofenin (Br IDA) after preparing the baby with phenobarbitone for 3-5 days. 20 (67%) cases were scan positive suggesting biliary atresia (BA) and 7(27%) cases were scan negative. In BA there will be increased hepatic uptake of the radionuclide without any significant excretion even in 24 hours delayed images. Presence of radiotracer in the bowel exclude the diagnosis of BA. Early diagnosis of biliary atresia is very important because in this condition surgery should be performed early (within 60 days of life). Studies suggest that hepatobiliary scan after hepatic stimulation with phenobarbitone for a period of 3-5 days is highly accurate for differentiating biliary atresia from other causes of neonatal jaundice. It is very important to perform hepatobiliary scan in a case of neonatal jaundice to exclude biliary atresia for the sake of baby's life.(author)

  15. The management of neonatal acute and chronic renal failure: A review.

    Science.gov (United States)

    Coulthard, Malcolm G

    2016-11-01

    Most babies with chronic renal failure are identified antenatally, and over half that are treated with peritoneal dialysis receive kidney transplants before school age. Most infants that develop acute renal failure have hypotension following cardiac surgery, or multiple organ failure. Sometimes the falls in glomerular filtration and urine output are physiological and reversible, and sometimes due to kidney injury, but (illogically) it is now common to define them all as having 'acute kidney injury'. Contrary to widespread opinion, careful interpretation of the plasma creatinine concentrations can provide sensitive evidence of early acute renal failure. Conservative management frequently leads to under-nutrition or fluid overload. Acute peritoneal dialysis is often technically fraught in very small patients, and haemotherapies have been limited by vascular access and anticoagulation requirements, the need to blood-prime circuits, and serious limitations in regulating fluid removal. Newer devices, including the Nidus, have been specifically designed to reduce these difficulties. Crown Copyright © 2016. Published by Elsevier Ireland Ltd. All rights reserved.

  16. Neonatal Hyperglycemia due to Transient Neonatal Diabetes Mellitus in Puerto Rico.

    Science.gov (United States)

    Fargas-Berríos, N; García-Fragoso, L; García-García, I; Valcárcel, M

    2015-01-01

    Neonatal hyperglycemia is a metabolic disorder found in the neonatal intensive care units. Neonatal diabetes mellitus (NDM) is a very uncommon cause of hyperglycemia in the newborn, occurring in 1 in every 400,000 births. There are two subtypes of neonatal diabetes mellitus: permanent neonatal diabetes mellitus (PNDM) and transient neonatal diabetes mellitus (TNDM). We describe a term, small for gestational age, female neonate with transient neonatal diabetes mellitus who presented with poor feeding tolerance and vomiting associated with hyperglycemia (385 mg/dL), glycosuria, and metabolic acidosis within the first 12 hours of life. The neonate was treated with intravenous insulin, obtaining a slight control of hyperglycemia. An adequate glycemia was achieved at 5 weeks of life. The molecular studies showed complete loss of maternal methylation at the TND differentially methylated region on chromosome 6q24. The etiology of this neonate's hyperglycemia was a hypomethylation of the maternal TND locus. A rare cause of neonatal diabetes mellitus must be considered if a neonate presents refractory hyperglycemia. To our knowledge, this is the first case reported in Puerto Rico of transient neonatal mellitus due to the uncommon mechanism of maternal hypomethylation of the TND locus. Its prevalence in Puerto Rico is unknown.

  17. Pulmonary Changes in Preterm Neonates with Hyaline Membrane Disease (a Clinicomorphological Study

    Directory of Open Access Journals (Sweden)

    A. M. Golubev

    2009-01-01

    Full Text Available Objective: to reveal lung morphological changes in preterm neonatal infants with hyaline membrane disease (HMD in the use of exogenous surfactants and artificial ventilation. Materials and methods. Case histories and autopsy protocols were analyzed in 90 preterm neonates who had died from severe respiratory failure. All the neonates were divided into 4 groups: 1 20 (22.2% infants who had received the exogenous surfactant Curosurf in the combined therapy of HMD; 2 19 (21.1% babies with HMD who had taken Surfactant BL; 3 25 (27.8% surfactant-untreated infants who had died from HMD; 4 26 (28.9% very preterm neonates with extremely low birth weight who had died within the first hour of life. The lungs were histologically and morphometrically examined. Results. The study demonstrated the specific course of HMD when exogenous surfactants and artificial ventilation were used. The contributors to the development of the disease are intranatal amniotic fluid aspiration and intranatal fetal hypoxia. Conclusion. Artificial ventilation and the use of exogenous surfactants do not block the generation of hyaline membranes. The latter differ in formation time, form, and location. The differences in a cell response to hyaline membranes were found in the neonatal infants receiving exogenous surfactants. The characteristic morphological signs of the disease for all the neonates enrolled in the study are alveolar and bronchial epithelial damages and microcirculatory disorders. Key words: preterm neonatal infants, hyaline membrane disease, exogenous surfactants, artificial ventilation, histology, morphometry.

  18. Glucose and Intermediary Metabolism and Astrocyte-Neuron Interactions Following Neonatal Hypoxia-Ischemia in Rat.

    Science.gov (United States)

    Brekke, Eva; Berger, Hester Rijkje; Widerøe, Marius; Sonnewald, Ursula; Morken, Tora Sund

    2017-01-01

    Neonatal hypoxia-ischemia (HI) and the delayed injury cascade that follows involve excitotoxicity, oxidative stress and mitochondrial failure. The susceptibility to excitotoxicity of the neonatal brain may be related to the capacity of astrocytes for glutamate uptake. Furthermore, the neonatal brain is vulnerable to oxidative stress, and the pentose phosphate pathway (PPP) may be of particular importance for limiting this kind of injury. Also, in the neonatal brain, neurons depend upon de novo synthesis of neurotransmitters via pyruvate carboxylase in astrocytes to increase neurotransmitter pools during normal brain development. Several recent publications describing intermediary brain metabolism following neonatal HI have yielded interesting results: (1) Following HI there is a prolonged depression of mitochondrial metabolism in agreement with emerging evidence of mitochondria as vulnerable targets in the delayed injury cascade. (2) Astrocytes, like neurons, are metabolically impaired following HI, and the degree of astrocytic malfunction may be an indicator of the outcome following hypoxic and hypoxic-ischemic brain injury. (3) Glutamate transfer from neurons to astrocytes is not increased following neonatal HI, which may imply that astrocytes fail to upregulate glutamate uptake in response to the massive glutamate release during HI, thus contributing to excitotoxicity. (4) In the neonatal brain, the activity of the PPP is reduced following HI, which may add to the susceptibility of the neonatal brain to oxidative stress. The present review aims to discuss the metabolic temporal alterations observed in the neonatal brain following HI.

  19. Congenital hypopituitarism and renal failure

    Directory of Open Access Journals (Sweden)

    Gaurav Atreja

    2011-01-01

    Full Text Available Congenital hypopituitarism is potentially fatal in the newborn period but treatable if the diagnosis is made early. We report a neonate who presented with hypothermia and severe hypoglycemia. He also had undescended testis and micropenis. Initial screening revealed panhypopituitarism, which was corrected promptly. He developed renal failure due to initial cardiovascular compromise related to hypotension but recovered quickly with standard management. Magnetic resonance imaging revealed absent stalk of anterior pituitary.

  20. Precipitating factors of porphyria cutanea tarda in Brazil with emphasis on hemochromatosis gene (HFE) mutations. Study of 60 patients.

    Science.gov (United States)

    Vieira, Fatima Mendonça Jorge; Nakhle, Maria Cristina; Abrantes-Lemos, Clarice Pires; Cançado, Eduardo Luiz Rachid; Reis, Vitor Manoel Silva dos

    2013-01-01

    Porphyria cutanea tarda is the most common form of porphyria, characterized by the decreased activity of the uroporphyrinogen decarboxylase enzyme. Several reports associated HFE gene mutations of hereditary hemochromatosis with porphyria cutanea tarda worldwide, although up to date only one study has been conducted in Brazil. Investigation of porphyria cutanea tarda association with C282Y and H63D mutations in the HFE gene. Identification of precipitating factors (hepatitis C, HIV, alcoholism and estrogen) and their link with HFE mutations. An ambispective study of 60 patients with PCT was conducted during the period from 2003 to 2012. Serological tests for hepatitis C and HIV were performed and histories of alcohol abuse and estrogen intake were investigated. HFE mutations were identified with real-time PCR. Porphyria cutanea tarda predominated in males and alcohol abuse was the main precipitating factor. Estrogen intake was the sole precipitating factor present in 25% of female patients. Hepatitis C was present in 41.7%. All HIV-positive patients (15.3%) had a history of alcohol abuse. Allele frequency for HFE mutations, i.e., C282Y (p = 0.0001) and H63D (p = 0.0004), were significantly higher in porphyria cutanea tarda patients, compared to control group. HFE mutations had no association with the other precipitating factors. Alcohol abuse, hepatitis C and estrogen intake are prevalent precipitating factors in our porphyria cutanea tarda population; however, hemochromatosis in itself can also contribute to the outbreak of porphyria cutanea tarda, which makes the research for HFE mutations necessary in these patients.

  1. NEONATAL COMPLICATIONS OF PREMATURE RUPTURE OF MEMBRANES

    Directory of Open Access Journals (Sweden)

    F. Nili AA. Shams Ansari

    2003-07-01

    Full Text Available Premature rupture of membranes (PROM is one of the most common complications of pregnancy that has a major impact on neonatal outcomes. With respect to racial, nutritional and cultural differences between developed and developing countries, this study was conducted to detect the prevalence of neonatal complications following PROM and the role of the duration of rupture of membranes in producing morbidities and mortalities in these neonates in our hospital. Among 2357 pregnant women, we found 163 (6.91% cases of premature rupture of the fetal membranes in Tehran Vali-e-Asr Hospital during April 2001 to April 2002. Route of delivery was cesarean section in 65.6% of women. Urinary tract infection occured in 1.8%, maternal leukocytosis and fever in 20.2% and 5.5%, chorioamnionitis in 6.1%, fetal tachycardia in 1.2% and olygohydramnios in 4.9%. Gestational age in 138 (86% of neonates was less than 37 completed weeks. Thirty five infants (21.47% had respiratory distress syndrome and 33 (20.245% had clinical sepsis. Pneumonia in 6 (3.7% and skeletal deformity in 7 (4.294% were seen. Rupture of membrane of more than 24 hours duration occurred in 71 (43.6% of the patients. Comparison of morbidities between two groups of neonates and their mothers according to the duration of PROM (less and more than 24 hours showed significant differences in NICU admission, olygohydramnios, maternal fever, leukocytosis and chorioamnionitis rates (p24 hr of PROM with an odds ratio of 2.68 and 2.73, respectively. Positive blood and eye cultures were detected in 16 cases during 72 hours of age. Staphylococcus species, klebsiella, E.coli and streptococcus were the predominant organisms among positive blood cultures. Mortality was seen in 18 (11% of neonates because of respiratory failure, disseminated intravascular coagulation, septic shock, and a single case of congenital toxoplasmosis. In this study, the prevalence of prematurity, sepsis and prolonged rupture of membrane

  2. Transient neonatal diabetes or neonatal hyperglycaemia: A case ...

    African Journals Online (AJOL)

    Transient neonatal diabetes and neonatal hyperglycaemia both present in the neonatal period with features of hyperglycaemia, dehydration and weight loss. Differentiating these conditions clinically is difficult. We describe the case of a 13 day old female whom we managed recently who could have had either condition.

  3. Neonatal Hyperglycemia due to Transient Neonatal Diabetes Mellitus in Puerto Rico

    Directory of Open Access Journals (Sweden)

    N. Fargas-Berríos

    2015-01-01

    Full Text Available Neonatal hyperglycemia is a metabolic disorder found in the neonatal intensive care units. Neonatal diabetes mellitus (NDM is a very uncommon cause of hyperglycemia in the newborn, occurring in 1 in every 400,000 births. There are two subtypes of neonatal diabetes mellitus: permanent neonatal diabetes mellitus (PNDM and transient neonatal diabetes mellitus (TNDM. We describe a term, small for gestational age, female neonate with transient neonatal diabetes mellitus who presented with poor feeding tolerance and vomiting associated with hyperglycemia (385 mg/dL, glycosuria, and metabolic acidosis within the first 12 hours of life. The neonate was treated with intravenous insulin, obtaining a slight control of hyperglycemia. An adequate glycemia was achieved at 5 weeks of life. The molecular studies showed complete loss of maternal methylation at the TND differentially methylated region on chromosome 6q24. The etiology of this neonate’s hyperglycemia was a hypomethylation of the maternal TND locus. A rare cause of neonatal diabetes mellitus must be considered if a neonate presents refractory hyperglycemia. To our knowledge, this is the first case reported in Puerto Rico of transient neonatal mellitus due to the uncommon mechanism of maternal hypomethylation of the TND locus. Its prevalence in Puerto Rico is unknown.

  4. New-Onset Neonatal Pulmonary Hypertension Associated with a Rhinovirus Infection

    Directory of Open Access Journals (Sweden)

    Nishit Patel

    2012-01-01

    Full Text Available A 3.5-week-old male neonate who developed an upper and lower respiratory tract rhinovirus infection that was temporally associated with the development of severe pulmonary hypertension is described. Rhinovirus has not previously been associated with pulmonary hypertension. This child developed severe pulmonary hypertension with right ventricular failure, requiring mechanical ventilation, nitric oxide inhalation and, eventually, extracorporeal membrane oxygenation.

  5. Molecular and cellular characterization of cardiac overload-induced hypertrophy and failure

    NARCIS (Netherlands)

    Umar, Soban

    2009-01-01

    In neonatal rat ventricular cardiomyocytes (NRVCs), we activated integrins by RGD to test whether integrin stimulation produced hypertrophy. Effect of RGD was compared with pro-hypertrophic effects of phenylephrine (chapter 2). Ventricular failure is associated with disturbed collagen turnover.

  6. BRAF V600E-Positive Multisite Langerhans Cell Histiocytosis in a Preterm Neonate

    Directory of Open Access Journals (Sweden)

    Sara V. Bates

    2013-10-01

    Full Text Available Hemorrhagic pustules with a “blueberry muffin” appearance accompanied by respiratory failure in a neonate present a challenging differential diagnosis that includes infections and neoplasms. We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH, positive for the oncogenic BRAF V600E mutation, in a preterm neonate. Infants with LCH pose a diagnostic challenge due to their heterogeneous presentations. This case is unusual in that the newborn presented with severe multiorgan involvement. Due to the rare incidence, wide spectrum of clinical manifestations, and high mortality rate, clinicians must maintain a high index of suspicion for LCH.

  7. Comparison between late-presenting and isolated neonatal congenital diaphragmatic hernias

    Directory of Open Access Journals (Sweden)

    Christos Plataras

    2011-01-01

    Full Text Available Purpose: Late-presenting posterolateral congenital diaphragmatic hernias (CDH are anatomically similar to isolated neonatal CDH but are diagnosed and treated after the first month of life. We aim to characterise the clinical manifestations and short-term postoperative course of this entity and compare it with isolated CDH of the neonatal period. Materials and Methods: In the 30-year period from 1980 to 2010, 116 children with CDH were treated at the Aghia Sophia Children′s Hospital, Athens, Greece. Twenty-three (19% of these children were late-presenting cases, being diagnosed between the ages of 1 month and 4 years. Ninety-three were neonatal cases, of whom 22 (24% were excluded due to severe associated anomalies, leaving 71 cases of isolated neonatal CDH. We compared these two groups of patients with regard to preoperative symptoms, postoperative hospital stay, time to complete feeding, overall complication rate, and reoperation rate. Results: Isolated neonatal cases presented more often with acute respiratory symptoms (n=25; P= 0.016 and failure to thrive (n= 38; P= 0.03. Late-presenting cases presented more often with chronic respiratory symptoms (n=14;P= 0.0044 or gastrointestinal symptoms (n=12; P= 0.006. Thirty-five cases with minor or serious complications were reported in the neonatal group, whereas only five complications were observed in the late-presenting group (P= 0.028. We did not record any recurrences or reoperations in the late-presenting group, but we had two recurrences and three reoperations in the neonatal group. Time to full feeds and postoperative hospital stay was shorter in the late-presenting group. Conclusions: Our data demonstrate differences between the two groups in preoperative symptoms and short-term postoperative complications and short-term outcome. Late-presenting cases of CDH had a greater number of chronic symptoms preoperatively, more favorable postoperative outcomes, and less recurrences and reoperations.

  8. Neonatal Bartter syndrome associated with ileal atresia and cystic fibrosis

    Directory of Open Access Journals (Sweden)

    A O Akuma

    2013-01-01

    Full Text Available A rare case of neonatal Bartter syndrome presenting with severe hyperkalemia is reported in a preterm child born to consanguineous parents. This child also had ileal atresia, and meconium plugs were found at laparotomy. The diagnosis of cystic fibrosis was subsequently made on genetic testing. Despite full intensive care management and surgical interventions, he died of respiratory failure after 70 days. This is the first reported case of such conglomeration of pathologies in a newborn child. Second, in highlighting this case we want clinicians to be aware that a subtype of neonatal Bartter syndrome can present with initial hyperkalemia so that an erroneous diagnosis of pseudohypoaldosteronism is not made when this is seen in combination with hyperkalemia and hyperrenin hyperaldosteronism.

  9. Stent Placement in a Neonate with Sano Modification of the Norwood using Semi-Elective Extracorporeal Membrane Oxygenation

    Directory of Open Access Journals (Sweden)

    Mustafa Gulgun

    Full Text Available Abstract Extracorporeal membrane oxygenation (ECMO is a well-established tool of cardiopulmonary circulatory support for cardiopulmonary failure in children and adults. It has been used as a supportive strategy during interventional procedures in neonates with congenital heart disease. Herein, we describe a neonate with hypoplastic left heart syndrome who underwent stenting of the Sano shunt and left pulmonary artery after Norwood Sano operation using intra-procedural ECMO support. The use of ECMO as a bridge to recovery might be a feasible and reasonably safe adjunctive approach in the treatment of complications in selective case of neonates having undergone the Norwood Sano procedure.

  10. The neonatal brain

    International Nuclear Information System (INIS)

    Flodmark, O.

    1987-01-01

    The clinical examination of the CNS in the neonate is often difficult in cases of complex pathology. Diagnostic imaging of the neonatal brain has become extremely useful and in the last decade has developed in two main directions: CT and US. MR imaging has been used recently with varying success in the diagnosis of pathology in the neonatal brain. Despite technical difficulties, this imaging method is likely to become increasingly important in the neonate. The paper examines the normal neonatal brain anatomy as seen with the different modalities, followed by pathologic conditions. Attention is directed to the common pathology, in asphyxiated newborns, the patholphysiology of intraventicular hemorrhage and periventricular leukomalacia in the preterm neonate, and hypoxic-ischemic brain injury in the term neonate. Pitfalls, artifacts, and problems in image interpretation are illustrated. Finally, the subsequent appearance of neonatal pathology later in infancy and childhood is discussed

  11. Neonatal hypertension.

    Science.gov (United States)

    Sharma, Deepak; Farahbakhsh, Nazanin; Shastri, Sweta; Sharma, Pradeep

    2017-03-01

    Neonatal hypertension (HT) is a frequently under reported condition and is seen uncommonly in the intensive care unit. Neonatal HT has defined arbitrarily as blood pressure more than 2 standard deviations above the base as per the age or defined as systolic BP more than 95% for infants of similar size, gestational age and postnatal age. It has been diagnosed long back but still is the least studied field in neonatology. There is still lack of universally accepted normotensive data for neonates as per gestational age, weight and post-natal age. Neonatal HT is an important morbidity that needs timely detection and appropriate management, as it can lead to devastating short-term effect on various organs and also poor long-term adverse outcomes. There is no consensus yet about the treatment guidelines and majority of treatment protocols are based on the expert opinion. Neonate with HT should be evaluated in detail starting from antenatal, perinatal, post-natal history, and drug intake by neonate and mother. This review article covers multiple aspects of neonatal hypertension like definition, normotensive data, various etiologies and methods of BP measurement, clinical features, diagnosis and management.

  12. Impact of prolonged leucine supplementation on protein synthesis and lean growth in neonatal pigs

    Science.gov (United States)

    Most low-birth weight infants experience extrauterine growth failure due to reduced nutrient intake as a result of feeding intolerance. The objective of this study was to determine whether prolonged enteral leucine supplementation improves lean growth in neonatal pigs fed a restricted protein diet. ...

  13. Etiologies of Prolonged Unconjugated Hyperbilirubinemia in Neonates Admitted to Neonatal Wards

    Directory of Open Access Journals (Sweden)

    Mohammad Kazem Sabzehei

    2015-12-01

    Full Text Available Background: Jaundice is a common condition among neonates. Prolonged unconjugated hyperbilirubinemia occurs when jaundice persists beyond two weeks in term neonates and three weeks in preterm neonates. This study aimed to determine the etiologies of prolonged unconjugated hyperbilirubinemia in infants admitted to the neonatal ward of Besat Hospital in Hamadan, Iran. Methods: This study was conducted on all infants diagnosed with prolonged unconjugated hyperbilirubinemia during 2007-2012 in the neonatal ward of Besat Hospital in Hamadan, Iran. Demographic characteristics of infants, physical examination and laboratory findings were collected and analyzed to determine the etiologies of neonatal hyperbilirubinemia. Results: In total, 100 infants diagnosed with neonatal hyperbilirubinemia were enrolled in this study, including 49 male and 51 female neonates with mean age of 20±1 days and mean bilirubin level of 17.5±4.0 mg/dL. Main causes of hyperbilirubinemia were urinary tract infection, ABO incompatibility, hypothyroidism and glucose-6-phosphate dehydrogenase deficiency in 14%, 5%, 6% and 5% of neonates, respectively. Moreover, unknown etiologies, such as breastfeeding, were detected in 70% of the studied infants. Conclusion: According to the results of this study, determining the main causes of prolonged unconjugated hyperbilirubinemia in neonates is of paramount importance. In the majority of cases, neonatal hyperbilirubinemia is associated with physiological factors, such as breastfeeding.

  14. The Study of Pulmonary Complication of Neonatal Mechanical Ventilation in NICU

    Directory of Open Access Journals (Sweden)

    M.K. Sabzeie

    2016-01-01

    Full Text Available Introduction & Objective: The main indication of mechanical ventilation is in the treatment of neonates with respiratory failure. With the increased use of mechanical ventilation, its complications have increased too. The aim of this study was to evaluate the prevalence of complications and short-term improvement in infants undergoing mechanical ventilation in the neonatal intensive care unit (NICU. Materials & Methods: In this prospective-analytic study, all infants requiring mechanical ventilation and admitted in the neonatal intensive care unit of Fatemiyeh and Be’sat hospitals, have been evaluated for one year (2012. Their data included: neonatal age, sex, gestational age, birth weight, weight at admission, diagnosis, length of hospitalization, disease outcome (improvement-died, need for mechanical ventilation, complications and culture results (blood, endotracheal tube, urine, CSF insert in check list. The data were analysed by SPSS and c2 statistical test. Results: In this study, a total of 114 infants hospitalized in intensive care unit and needed mechanical ventilation was studied of whom 72 were male and 42 were female. The mean of gestational age in the admitted neonates was 32.9 ± 0.85 weeks. The majority of neonates (80.70% were undergoing mechanical ventilation with respiratory distress syndrome (RDS. 67% of neonates were suffering from complications of mechanical ventilation. The prevalent complication was seen in the neonates was narrowing or obstruction of the endotracheal tube (52.63%. 47.37% of infants died and respiratory distress syndrome was the common cause of death in these neonates (46.29%. In our study, there was significant relationship between resuscitation at birth (P=0.002, time required for mechanical ventilation (P=0.0000 and Apgar score (P=0.0000 and complications of mechanical ventilation. Conclusions: The results show that the high prevalence of pulmonary complications is associated with mechanical

  15. Knowledge, attitudes and practices of neonatal staff concerning neonatal pain management

    Directory of Open Access Journals (Sweden)

    Sizakele L.T. Khoza

    2014-11-01

    Full Text Available Background: Neonatal pain management has received increasing attention over the past four decades. Research into the effects of neonatal pain emphasises the professional, ethical and moral obligations of staff to manage pain for positive patient outcomes. However, evaluation studies continuously report evidence of inadequate neonate pain management and a gap between theory and practice. Objective: This study reviewed current practice in neonatal pain management to describe the knowledge, attitudes and practices of nurses and doctors regarding pain management for neonates in two academic hospitals. Method: A non-experimental, prospective quantitative survey, the modified Infant Pain Questionnaire, was used to collect data from 150 nurses and doctors working in the neonatal wards of two academic hospitals in central Gauteng. Results: The response rate was 35.33% (n = 53, most respondents being professional nurses (88.68%; n = 47 working in neonatal intensive care units (80.77%; n = 42; 24 (45.28% had less than 5 years’ and 29 respondents 6 or more years’ working experience in neonatal care. A review of pain management in the study setting indicated a preference for pharmacological interventions to relieve moderate to severe pain. An association (p < 0.05 was found between pain ratings on 5 procedures and frequency of administration of pharmacological pain management. Two-thirds of respondents (64% reported that there were no pain management guidelines in the neonatal wards in which they worked. Conclusion: The interventions to manage moderate neonatal pain are in line with international guidelines. However, neonatal pain management may not occur systematically based on prior assessment of neonatal pain, choice of most appropriate intervention and evaluation. This study recommends implementation of a guideline to standardise practice and ensure consistent and adequate pain management in neonates.

  16. Neonatal retinoblastoma

    Directory of Open Access Journals (Sweden)

    Tero T Kivelä

    2017-01-01

    Full Text Available From 7% to 10% of all retinoblastomas and from 44% to 71% of familial retinoblastomas in developed countries are diagnosed in the neonatal period, usually through pre- or post-natal screening prompted by a positive family history and sometimes serendipitously during screening for retinopathy of prematurity or other reasons. In developing countries, neonatal diagnosis of retinoblastoma has been less common. Neonatal retinoblastoma generally develops from a germline mutation of RB1, the retinoblastoma gene, even when the family history is negative and is thus usually hereditary. At least one-half of infants with neonatal retinoblastoma have unilateral tumors when the diagnosis is made, typically the International Intraocular Retinoblastoma Classification (Murphree Group B or higher, but most germline mutation carriers will progress to bilateral involvement, typically Group A in the fellow eye. Neonatal leukokoria usually leads to the diagnosis in children without a family history of retinoblastoma, and a Group C tumor or higher is typical in the more advanced involved eye. Almost all infants with neonatal retinoblastoma have at least one eye with a tumor in proximity to the foveola, but the macula of the fellow eye is frequently spared. Consequently, loss of reading vision from both eyes is exceptional. A primary ectopic intracranial neuroblastic tumor known as trilateral retinoblastoma is no more common after neonatal than other retinoblastoma. For many reasons, neonatal retinoblastoma may be a challenge to eradicate, and the early age at diagnosis and relatively small tumors do not guarantee the preservation of both eyes of every involved child. Oncology nurses can be instrumental in contributing to better outcomes by ensuring that hereditary retinoblastoma survivors receive genetic counseling, by referring families of survivors to early screening programs when they are planning for a baby, and by providing psychological and practical support

  17. Octreotide for the treatment of chylothorax in neonates.

    LENUS (Irish Health Repository)

    Das, Animitra

    2012-02-01

    BACKGROUND: Routine care for chylothorax in neonate includes either conservative or surgical approaches. Octreotide, a somatostatin analogue, has been used for the management of patients with refractory chylothorax not responding to conservative management. OBJECTIVES: To assess the efficacy and safety of octreotide in the treatment of chylothorax in neonates. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library), MEDLINE and EMBASE (to March 7, 2010). We assessed the reference lists of identified trials and abstracts from the annual meetings of the Pediatric Academic Societies published in Pediatric Research (2002 to 2009) without language restrictions. SELECTION CRITERIA: We planned to include randomised or quasi-randomised controlled trials of octreotide in the treatment of congenital or acquired chylothorax in term or preterm neonates, with any dose, duration or route of administration. DATA COLLECTION AND ANALYSIS: Data on primary (amount of fluid drainage, respiratory support, mortality) and secondary outcomes (side effects) were planned to be collected and analysed using mean difference, relative risk and risk difference with 95% confidence intervals. MAIN RESULTS: No randomised controlled trials were identified. Nineteen case reports of 20 neonates with chylothorax in whom octreotide was used either subcutaneously or intravenously were identified. Fourteen case reports described successful use (resolution of chylothorax), four reported failure (no resolution) and one reported equivocal results following use of octreotide. The timing of initiation, dose, duration and frequency of doses varied markedly. Gastrointestinal intolerance and clinical presentations suggestive of necrotizing enterocolitis and transient hypothyroidism were reported as side effects. AUTHORS\\' CONCLUSIONS: No practice recommendation can be made based on the evidence identified in this review. A prospective registry of

  18. Neonatal Marfan syndrome caused by an exon 25 mutation of the fibrillin-1 gene.

    Science.gov (United States)

    Elçioglu, N H; Akalin, F; Elçioglu, M; Comeglio, P; Child, A H

    2004-01-01

    Neonatal Marfan syndrome caused by an exon 25 mutation of the Fibrillin-1 gene: We describe a male infant with severe arachnodactyly, hypermobility of the fingers, flexion contractures of elbows, wrists, hips, and knees, microretrognathia, crumpled ears, rockerbottom feet, loose redundant skin, and lens dislocations. Cardiac valve insufficiency and aortic dilatation resulted in cardiac failure, decompensated with digitalisation and death occurred at the age of 4 months. This case represents the severe end of the clinical spectrum of Marfan syndrome, namely neonatal Marfan syndrome. Molecular diagnostic analyses confirmed a de novo exon 25 mutation in the FBN1 gene.

  19. The infectious disease blood safety risk of Australian hemochromatosis donations.

    Science.gov (United States)

    Hoad, Veronica; Bentley, Peter; Bell, Barbara; Pathak, Praveen; Chan, Hiu Tat; Keller, Anthony

    2016-12-01

    It has been suggested that blood donors with hereditary hemochromatosis may pose an increased infectious disease risk and adversely affect recipient outcomes. This study compares the infectious disease risk of whole blood (WB) donors enrolled as therapeutic (T) donors to voluntary WB donors to evaluate the safety of blood products provided by the T donors. This was a retrospective cohort study of all WB donations at the Australian Red Cross Blood Service who donated between January 1, 2011, and December 31, 2013, comparing a yearly mean of 11,789 T donors with 107,773 total donations and a yearly mean of 468,889 voluntary WB donors with 2,584,705 total donations. We compared postdonation notification of infectious illnesses, bacterial contamination screening results, and positive tests for blood borne viruses in T and WB donors. Rates of transfusion-transmissible infections in donations destined for component manufacture were significantly lower in therapeutic donations compared to voluntary donations (8.4 vs. 21.6 per 100,000 donations). Bacterial contamination (43.0 vs. 45.9 per 100,000 donations) and postdonation illness reporting (136.2 vs. 110.8 per 100,000 donations) were similar in both cohorts. The Australian therapeutic venisection program enables T donors to provide a safe and acceptable source of donated WB that has a low infectious disease risk profile. © 2016 AABB.

  20. Home-based Care Needs of Preterm Infants Discharged Early from the Neonatal Intensive Care Unit: A Descriptive Qualitative Study

    Directory of Open Access Journals (Sweden)

    Mahboobeh Namnabati

    2017-12-01

    Conclusion: The enhancement of the nurses' knowledge about the needs of the preterm neonates with early discharge would result in the improvement of their abilities in the relevant domain. Accordingly, these nurses could help the mothers to prevent the incidence of several complications in the neonates, such as readmissions to the NICU. More importantly, these measures could prevent from the consequences of failure to fulfil these needs emerging in the later stages of life.

  1. Reduced size liver transplantation from a donor supported by a Berlin Heart.

    Science.gov (United States)

    Misra, M V; Smithers, C J; Krawczuk, L E; Jenkins, R L; Linden, B C; Weldon, C B; Kim, H B

    2009-11-01

    Patients on cardiac assist devices are often considered to be high-risk solid organ donors. We report the first case of a reduced size liver transplant performed using the left lateral segment of a pediatric donor whose cardiac function was supported by a Berlin Heart. The recipient was a 22-day-old boy with neonatal hemochromatosis who developed fulminant liver failure shortly after birth. The transplant was complicated by mild delayed graft function, which required delayed biliary reconstruction and abdominal wall closure, as well as a bile leak. However, the graft function improved quickly over the first week and the patient was discharged home with normal liver function 8 weeks after transplant. The presence of a cardiac assist device should not be considered an absolute contraindication for abdominal organ donation. Normal organ procurement procedures may require alteration due to the unusual technical obstacles that are encountered when the donor has a cardiac assist device.

  2. Multilocular Hepatic Abscess Formation and Sepsis due to Yersinia enterocolitica in a Patient with Hereditary Hemochromatosis and Type 2 Diabetes Mellitus

    Directory of Open Access Journals (Sweden)

    Matthias Sauter

    2017-11-01

    Full Text Available Infection with Yersinia enterocolitica (YE typically presents with mild gastroenteritis without systemic infection. However, systemic YE infection has been described in states of iron overload. We present the case of a patient with sepsis with hepatic abscesses due to YE infection. Workup revealed a past diagnosis of diabetes mellitus and hemochromatosis which had been untreated for the previous 5 years due to patient refusal. This case highlights risk factors for systemic infection with YE. A high degree of suspicion for YE infection is warranted in patients with iron overload, diabetes mellitus, or immunosuppression.

  3. Could Neonatal Hypernatremia Dehydration Influence Hearing Status?

    Directory of Open Access Journals (Sweden)

    Hassan Boskabadi

    2014-01-01

    Full Text Available Introduction: Neonatal hypernatremia dehydration (NHD is a dangerous condition in neonates, which is accompanied by acute complications (renal failure, cerebral edema, and cerebral hemorrhage and chronic complications (developmental delay. Children begin learning language from birth, and hearing impairment interferes with this process. We assessed the hearing status of infants with hypernatremia dehydration.   Materials and Methods: In a case-control study in 110 infants presenting at the Ghaem Hospital (Mashhad, Iran between 2007 and 2011, we examined the incidence of hearing impairment in infants suffering from hypernatremia dehydration (serum sodium >150 mEq/L in comparison with infants with normal sodium level (serum sodium ≤150 mEq/L.   Results: Three of 110 cases examined in the study group showed a transient hearing impairment. A mean serum sodium level of 173mg/dl was reported among hearing-impaired infants.   Conclusion:  Transient hearing impairment was higher in infants with hypernatremia; although this difference was not significant (P>0.05. Hearing impairment was observed in cases of severe hypernatremia.  

  4. Foetal and neonatal thyroid disorders.

    Science.gov (United States)

    Radetti, G; Zavallone, A; Gentili, L; Beck-Peccoz, P; Bona, G

    2002-10-01

    recently identified are represented by TSH receptor mutations leading to constitutively activated TSH receptor. Infants born to mothers with Graves' history may develop neonatal thyrotoxicosis. Foetal/neonatal disease is due to transplacental thyrotrophin receptor stimulating antibodies (TRAb) passage. It's extremely important recognizing and treating Graves' disease in mothers as soon as possible, because a thyrotoxic state may have adverse effects on the outcome of pregnancy and both on the foetus and newborn. Thyrotoxic foetuses may develop goitre, tachycardia, hydrops associated with heart failure, growth retardation, craniosynostosis, increased foetal motility and accelerated bone maturation. Neonatal Graves' disease tends to resolve spontaneously within 3-12 weeks as maternal thyroid stimulating immunoglobulins are cleared from the circulation but subsequent development may be impaired by perceptual motor difficulties. Hashimoto's thyroiditis is a very common autoimmune thyroid disease. In presence of maternal Hashimoto's thyroiditis, there are usually no consequences on foetal thyroid, even if antiTPO and antiTg antibodies can be found in the newborn due to transplacental passage. However there are some literature reports describing foetal and neonatal hyperthyroidism in the affected mothers' offspring.

  5. 足月新生儿呼吸衰竭368例临床分析及存活儿随访观察%Clinical analysis of neonatal respiratory failure in 368 term neonates and follow-up observation of the survivals

    Institute of Scientific and Technical Information of China (English)

    任青; 赵丽丽; 许平; 张勇军

    2015-01-01

    Objective To analyze the clinical characteristics of neonatal respiratory failure (NRF) in term neonates hospitalized in neonatal intensive care unit (NICU) of Liaocheng people's hospital,and to follow-up the quality of life in NRF term neonates who needed endotracheally intubated and normal frequency mechanical ventilation.Methods From January 2010 to December 2011 in NICU of Liaocheng people' s hospital,the clinical data of the NRF neonates who received mechanical ventilation were summarized at the same time.Physical development,neurological development and the times of respiratory infection of the survival NRF in term neonates who needed endotracheally intubated and normal frequency mechanical ventilation were followed up for 18 months after discharge.Results There were 368 cases of term neonates with NRF who received mechanical ventilation during admission from 2010 to 2011 in NICU.(1)Clinical features of NRF were:① Complications:154 cases(41.8%) with kidney injury,54 cases(14.7%) with pulmonary hypertension,7 cases (1.9%) with pneumothorax.② Etiology:Main primary diseases including 143 cases of neonatal respiratory distress syndrome (RDS) and (or) severe wet lung disease (38.9%),102 cases of pneumonia (27.7%),and 72 cases of asphyxia (19.6%).③ Prognosis:244 neonates (66.3%) had complete recovery,43 neonates (11.7%) had clinical improvement,16 neonates (4.3%)deceased and 60 neonates(17.7%)with parental abandon of treatments.④ Hospitalization was (12.1 ± 0.3)d.(2) Follow up:Among all of the 186 cases of NRF in term neonates who needed endotracheally intubated and normal frequency mechanical ventilation,118 cases(63.4%) were cured,81 cases were followed up for 18 months after discharge.The follow-up rate was 68.6%.Sixty-seven cases(82.7%) were completely normal,14 cases (17.3%) were with adverse neurological outcome,in which 6 cases(7.4%) with mental abnormality,8 cases(9.9%) with abnormal movement,One case (1.2%) with

  6. Technological advances in extracorporeal membrane oxygenation for respiratory failure.

    Science.gov (United States)

    Rehder, Kyle J; Turner, David A; Bonadonna, Desiree; Walczak, Richard J; Rudder, Robert J; Cheifetz, Ira M

    2012-08-01

    Extracorporeal membrane oxygenation (ECMO) for neonatal and pediatric cardiac and/or respiratory failure is well established, and its use for adult respiratory failure is rapidly increasing. Management strategies developed over the past 30 years coupled with significant recent technological advances have led to improved ECMO survival. These new technologies are expanding the potential applications for ECMO in exciting ways, including new patient populations and the ability to make ECMO mobile for both intra- and inter-hospital transport. In this article, we highlight some of the recent technological advances and their impact on the utilization of ECMO in increasingly diverse patient populations.

  7. Ventilatory management in neonates. Science or art?

    Science.gov (United States)

    Mariani, G L; Carlo, W A

    1998-03-01

    Conventional mechanical ventilation continues to be the standard mode of support for neonates with respiratory failure. Controversies regarding the selection of optimal ventilatory strategies still abound. A deep understanding of physiologic concepts as well as a critical appraisal of the literature is needed to optimize the ventilatory management of the newborn. Principles of gas exchange, pulmonary mechanics and control of breathing are reviewed in the context of their relevance during mechanical ventilation. The application of these concepts to the ventilatory strategies for the management of infants with respiratory distress is presented, and current controversies are emphasized.

  8. Advancing Neurologic Care in the Neonatal Intensive Care Unit with a Neonatal Neurologist

    Science.gov (United States)

    Mulkey, Sarah B.; Swearingen, Christopher J.

    2014-01-01

    Neonatal neurology is a growing sub-specialty area. Given the considerable amount of neurologic problems present in the neonatal intensive care unit, a neurologist with expertise in neonates is becoming more important. We sought to evaluate the change in neurologic care in the neonatal intensive care unit at our tertiary care hospital by having a dedicated neonatal neurologist. The period post-neonatal neurologist showed a greater number of neurology consultations (Pneurology encounters per patient (Pneurology became part of the multi-disciplinary team providing focused neurologic care to newborns. PMID:23271754

  9. Lack of association of C282Y and H63D mutations in the hemochromatosis (HFE) gene with diabetes mellitus type 2 in a case-control study of women in Brazil.

    Science.gov (United States)

    Gomes, K B; Carvalho, M G; Coelho, F F; Rodrigues, I F; Soares, A L; Guimarães, D A; Fernandes, A P

    2009-10-27

    Hereditary hemochromatosis is one of the most common autosomal recessive diseases; it is characterized by excess absorption of iron. Clinically, the major challenge is to diagnose increased iron deposition before irreversible tissue damage has occurred. C282Y and H63D are the main mutations related to hereditary hemochromatosis; these mutations have been reported to be associated with increased risk of developing diabetes mellitus type 2 (DM2). We investigated whether these mutations are associated with increased risk for the development of DM2 in women in Brazil. Seventy-two women with clinical diagnosis of DM2 under treatment with hypoglycemic agents and a control group composed of 72 women with no clinical history of diabetes were studied. The C282Y and H63D mutations were determined by PCR-RFLP. Significant differences were not observed for C282Y and H63D, when we compared diabetic and non-diabetic women. We suggest that mutations C282Y and H63D in the HFE gene are not significant risk factors for the development of DM2 in Brazilian women.

  10. Protein synthesis in skeletal muscle of neonatal pigs is enhanced by administration of β-hydroxy-β-methylbutyrate

    OpenAIRE

    Wheatley, Scott M.; El-Kadi, Samer W.; Suryawan, Agus; Boutry, Claire; Orellana, Renán A.; Nguyen, Hanh V.; Davis, Steven R.; Davis, Teresa A.

    2013-01-01

    Many low-birth-weight infants experience failure to thrive. The amino acid leucine stimulates protein synthesis in skeletal muscle of the neonate, but less is known about the effects of the leucine metabolite β-hydroxy-β-methylbutyrate (HMB). To determine the effects of HMB on protein synthesis and the regulation of translation initiation and degradation pathways, overnight-fasted neonatal pigs were infused with HMB at 0, 20, 100, or 400 μmol·kg body wt−1·h−1 for 1 h (HMB 0, HMB 20, HMB 100, ...

  11. Neonatal hypocalcemia, neonatal seizures, and intellectual disability in 22q11.2 deletion syndrome

    Science.gov (United States)

    Cheung, Evelyn Ning Man; George, Susan R.; Andrade, Danielle M.; Chow, Eva W. C.; Silversides, Candice K.; Bassett, Anne S.

    2015-01-01

    Purpose Hypocalcemia is a common endocrinological condition in 22q11.2 deletion syndrome. Neonatal hypocalcemia may affect neurodevelopment. We hypothesized that neonatal hypocalcemia would be associated with rare, more severe forms of intellectual disability in 22q11.2 deletion syndrome. Methods We used a logistic regression model to investigate potential predictors of intellectual disability severity, including neonatal hypocalcemia, neonatal seizures, and complex congenital heart disease, e.g., interrupted aortic arch, in 149 adults with 22q11.2 deletion syndrome. Ten subjects had moderate-to-severe intellectual disability. Results The model was highly significant (P < 0.0001), showing neonatal seizures (P = 0.0018) and neonatal hypocalcemia (P = 0.047) to be significant predictors of a more severe level of intellectual disability. Neonatal seizures were significantly associated with neonatal hypocalcemia in the entire sample (P < 0.0001), regardless of intellectual level. There was no evidence for the association of moderate- to-severe intellectual disability with other factors such as major structural brain malformations in this sample. Conclusion The results suggest that neonatal seizures may increase the risk for more severe intellectual deficits in 22q11.2 deletion syndrome, likely mediated by neonatal hypocalcemia. Neonatal hypocalcemia often remains unrecognized until the postseizure period, when damage to neurons may already have occurred. These findings support the importance of early recognition and treatment of neonatal hypocalcemia and potentially neonatal screening for 22q11.2 deletions. PMID:23765047

  12. Incidence of acute kidney injury in the neonatal intensive care unit

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    Doaa Youssef

    2015-01-01

    Full Text Available The aim of this work is to study the incidence of acute kidney injury (AKI in neonates admitted to the neonatal intensive care unit (NICU over a six-month period from September 2011 to March 2012. This prospective study was performed on 250 neonates admitted to the NICU at the Children′s Hospital, Faculty of Medicine, Zagazig University. All neonates were subjected to detailed history taking, including pre-natal, natal and post-natal history, with stress on symptoms suggestive of AKI. All neonates were examined thoroughly and the following investigations were performed: Blood urea nitrogen (BUN, serum creatinine, sodium, potassium, calcium, complete blood count, C-reactive protein, arterial blood gases, urine sodium and urine creatinine. AKI was diagnosed in 27 cases (10.8%, including 12 females and 15 males. 40.7% of the AKI cases were born after full-term pregnancy while 59.3% were pre-term babies. 29.6% of the AKI cases had oliguria, and there was male sex predominance, with a male-female ratio of 1.3:1. The cause of AKI was pre-renal in 96.3% and intrinsic renal in 3.7% of the cases. The predisposing factors for AKI were sepsis in 63% of the cases, respiratory distress syndrome in 55.6%, mechanical ventilation in 51.9%, peri-natal asphyxia in 18.5%, dehydration in 14.8%, surgical operation in 11.1%, congenital heart disease in 7.4%, sub-galeal hematoma in 3.7%, polycythemia in 3.7% and intra-ventricular hemorrhage in 3.7% of the cases. Our data suggest that pre-renal failure was the most common form of AKI in our patients. Early recognition of risk factors such as sepsis, peri-natal asphyxia or peri-operative problems and rapid effective treatment of contributing conditions will reduce the incidence of AKI in the neonatal period.

  13. Incidence of acute kidney injury in the neonatal intensive care unit.

    Science.gov (United States)

    Youssef, Doaa; Abd-Elrahman, Hadeel; Shehab, Mohamed M; Abd-Elrheem, Mohamed

    2015-01-01

    The aim of this work is to study the incidence of acute kidney injury (AKI) in neonates admitted to the neonatal intensive care unit (NICU) over a six-month period from September 2011 to March 2012. This prospective study was performed on 250 neonates admitted to the NICU at the Children's Hospital, Faculty of Medicine, Zagazig University. All neonates were subjected to detailed history taking, including pre-natal, natal and post-natal history, with stress on symptoms suggestive of AKI. All neonates were examined thoroughly and the following investigations were performed: Blood urea nitrogen (BUN), serum creatinine, sodium, potassium, calcium, complete blood count, C-reactive protein, arterial blood gases, urine sodium and urine creatinine. AKI was diagnosed in 27 cases (10.8%), including 12 females and 15 males. 40.7% of the AKI cases were born after full-term pregnancy while 59.3% were pre-term babies. 29.6% of the AKI cases had oliguria, and there was male sex predominance, with a male-female ratio of 1.3:1. The cause of AKI was pre-renal in 96.3% and intrinsic renal in 3.7% of the cases. The predisposing factors for AKI were sepsis in 63% of the cases, respiratory distress syndrome in 55.6%, mechanical ventilation in 51.9%, peri-natal asphyxia in 18.5%, dehydration in 14.8%, surgical operation in 11.1%, congenital heart disease in 7.4%, sub-galeal hematoma in 3.7%, polycythemia in 3.7% and intra-ventricular hemorrhage in 3.7% of the cases. Our data suggest that pre-renal failure was the most common form of AKI in our patients. Early recognition of risk factors such as sepsis, peri-natal asphyxia or peri-operative problems and rapid effective treatment of contributing conditions will reduce the incidence of AKI in the neonatal period.

  14. Phenobarbital for Neonatal Seizures: Response Rate and Predictors of Refractoriness.

    Science.gov (United States)

    Spagnoli, Carlotta; Seri, Stefano; Pavlidis, Elena; Mazzotta, Silvia; Pelosi, Annalisa; Pisani, Francesco

    2016-10-01

    Background Phenobarbital is the first-line choice for neonatal seizures treatment, despite a response rate of approximately 45%. Failure to respond to acute anticonvulsants is associated with poor neurodevelopmental outcome, but knowledge on predictors of refractoriness is limited. Objective To quantify response rate to phenobarbital and to establish variables predictive of its lack of efficacy. Methods We retrospectively evaluated newborns with electrographically confirmed neonatal seizures admitted between January 1999 and December 2012 to the neonatal intensive care unit of Parma University Hospital (Italy), excluding neonates with status epilepticus. Response was categorized as complete (cessation of clinical and electrographic seizures after phenobarbital administration), partial (reduction but not cessation of electrographic seizures with the first bolus, response to the second bolus), or absent (no response after the second bolus). Multivariate analysis was used to identify independent predictors of refractoriness. Results Out of 91 newborns receiving phenobarbital, 57 (62.6%) responded completely, 15 (16.5%) partially, and 19 (20.9%) did not respond. Seizure type (p = 0.02), background electroencephalogram (EEG; p ≤ 0.005), and neurologic examination (p  ≤  0.005) correlated with response to phenobarbital. However, EEG (p  ≤  0.02) and seizure type (p  ≤  0.001) were the only independent predictors. Conclusion Our results suggest a prominent role of neurophysiological variables (background EEG and electrographic-only seizure type) in predicting the absence of response to phenobarbital in high-risk newborns. Georg Thieme Verlag KG Stuttgart · New York.

  15. Transient neonatal myasthenia gravis due to a mother with ocular onset of anti-muscle specific kinase myasthenia gravis.

    Science.gov (United States)

    Lee, Ju-Yeun; Min, Ju-Hong; Han, Sueng-Han; Han, Jinu

    2017-07-01

    We describe a 27-year-old pregnant female with new onset of conjugate gaze deficit during the third trimester of pregnancy. Repetitive nerve stimulation tests, neostigmine tests, and acetylcholine receptor antibody assays were all negative. The patient delivered a normal healthy baby at a local clinic via cesarean section. The baby became hypotonic and had respiratory failure several minutes after birth. The result of acetylcholine receptor antibody was negative in the neonate. The neonate became healthy spontaneously and was extubated after 21 days of ventilation care. Two months after delivery, the mother developed ptosis and generalized symptoms and subsequent workup revealed she was muscle specific kinase (MuSK) antibody positive. The neonate was presumed to have an anti-MuSK-mediated transient neonatal myasthenia gravis. Although MuSK antibody testing is rarely indicated in ocular myasthenia gravis, MuSK antibody testing is necessary in pregnant women who are presumed ocular myasthenia gravis to warn occurrence of transient neonatal myasthenia gravis. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Neonatal Bartter syndrome with cholelithiasis and hydrocephalus: Rare association.

    Science.gov (United States)

    Özdemir, Özmert Ma; Çıralı, Ceren; Yılmaz Ağladıoğlu, Sebahat; Evrengül, Havva; Tepeli, Emre; Ergin, Hacer

    2016-09-01

    Neonatal Bartter syndrome (NBS) is a rare autosomal recessive renal tubular disorder. This disease is characterized by hypokalemia, hypochloremia, and metabolic alkalosis that is often associated with failure to thrive and recurrent episodes of dehydration. The combination of BS and cholelithiasis in an infant is very rare. Herein, we report a premature male infant with NBS who developed cholelithiasis and hydrocephalus on clinical follow up. We recommend that periodic routine hepatobiliary ultrasonograpic screening for cholelithiasis should be performed in patients with NBS. © 2016 Japan Pediatric Society.

  17. Increased Retinal Expression of the Pro-Angiogenic Receptor GPR91 via BMP6 in a Mouse Model of Juvenile Hemochromatosis.

    Science.gov (United States)

    Arjunan, Pachiappan; Gnanaprakasam, Jaya P; Ananth, Sudha; Romej, Michelle A; Rajalakshmi, Veeranan-Karmegam; Prasad, Puttur D; Martin, Pamela M; Gurusamy, Mariappan; Thangaraju, Muthusamy; Bhutia, Yangzom D; Ganapathy, Vadivel

    2016-04-01

    Hemochromatosis, an iron-overload disease, occurs as adult and juvenile types. Mutations in hemojuvelin (HJV), an iron-regulatory protein and a bone morphogenetic protein (BMP) coreceptor, underlie most of the juvenile type. Hjv(-/-) mice accumulate excess iron in retina and exhibit aberrant vascularization and angiomas. A succinate receptor, GPR91, is pro-angiogenic in retina. We hypothesized that Hjv(-/-) retinas have increased BMP signaling and increased GPR91 expression as the basis of angiomas. Expression of GPR91 was examined by qPCR, immunofluorescence, and Western blot in wild-type and Hjv(-/-) mouse retinas and pRPE cells. Influence of excess iron and BMP6 on GPR91 expression was investigated in ARPE-19 cells, and wild-type and Hjv(-/-) pRPE cells. Succinate was used to activate GPR91 and determine the effects of GPR91 signaling on VEGF expression. Signaling of BMP6 was studied by the expression of Smad1/5/8 and pSmad4, and the BMP-target gene Id1. The interaction of pSmad4 with GPR91 promoter was studied by ChIP. Expression of GPR91 was higher in Hjv(-/-) retinas and RPE than in wild-type counterparts. Unexpectedly, BMP signaling was increased, not decreased, in Hjv(-/-) retinas and RPE. Bone morphogenetic protein 6 induced GPR91 in RPE, suggesting that increased BMP signaling in Hjv(-/-) retinas was likely responsible for GPR91 upregulation. Exposure of RPE to excess iron and succinate as well as BMP6 and succinate increased VEGF expression. Bone morphogenetic protein 6 promoted the interaction of pSmad4 with GPR91 promoter in RPE. G-protein-coupled receptor 91 is a BMP6 target and Hjv deletion enhances BMP signaling in retina, thus underscoring a role for excess iron and hemochromatosis in abnormal retinal vascularization.

  18. Risk Factors for Neonatal Mortality Among Very Low Birth Weight Neonates

    Directory of Open Access Journals (Sweden)

    Fatemeh Nayeri

    2013-05-01

    Full Text Available The objective of this study is to determine risk factors causing increase in very low birth way (VLBW neonatal mortality. The medical files of all neonates weighing ≤1500 g, born in Vali-e-Asr hospital (2001-2004 were studied. Two groups of neonates (living and dead were compared up to the time of hospital discharge or death. A total of 317 neonates were enrolled. A meaningful relationship existed between occurrence of death and low gestational age (P=0.02, low birth weight, lower than 1000 g (P=0.001, Apgar score <6 at 5th minutes (P=0.001, resuscitation at birth (P=0.001, respiratory distress syndrome (P=0.001 need for mechanical ventilation (P=0.001, neurological complications (P=0.001 and intraventricular hemorrhage (P=0.001. Regression analysis indicated that each 250 g weight increase up to 1250 g had protective effect, and reduced mortality rate. The causes of death of those neonates weighting over 1250 g should be sought in factors other than weight. Survival rate was calculated to be 80.4% for neonates weighing more than 1000 g. The most important high risk factors affecting mortality of neonates are: low birth weight, need for resuscitation at birth, need for ventilator use and intraventricular hemorrhage.

  19. [Persistent pulmonary hypertension in a neonate caused by blood aspiration following vaginal blood loss].

    Science.gov (United States)

    Krüse-Ruijter, M F; Zimmermann, L J I

    2007-07-14

    A preterm neonate, with a gestational age of 30 1/7 weeks, was born after a period of prolonged rupture of the membranes and a retroplacental haematoma causing vaginal bleeding. During admission to the neonatal intensive-care unit, mechanical ventilation was indicated because of acute respiratory failure following blood aspiration, which was causing oxygenation and ventilation problems. Endotracheal surfactant was administered and, because of persistent pulmonary hypertension of the newborn (PPHN), NO-inhalation therapy was started. A quick recovery was seen and two days post partum the patient could be extubated. Blood aspiration may cause acute respiratory problems and PPHN, with quick recovery after effective mechanical ventilation, surfactant and NO-inhalation therapy.

  20. Hiperbilirrubinemia neonatal agravada Aggravated neonatal hyperbilirubinemia

    Directory of Open Access Journals (Sweden)

    Ana Campo González

    2010-09-01

    Full Text Available INTRODUCCIÓN. La mayoría de las veces la ictericia en el recién nacido es un hecho fisiológico, causado por una hiperbilirrubinemia de predominio indirecto, secundario a inmadurez hepática e hiperproducción de bilirrubina. El objetivo de este estudio fue determinar el comportamiento de la hiperbilirrubinemia neonatal en el Hospital Docente Ginecoobstétrico de Guanabacoa en los años 2007 a 2009. MÉTODOS. Se realizó un estudio descriptivo y retrospectivo de 173 recién nacidos que ingresaron al Departamento de Neonatología con diagnóstico de hiperbilirrubinemia agravada. RESULTADOS. La incidencia de hiperbilirrubinemia neonatal agravada fue del 3,67 % y predominó en hermanos con antecedentes de ictericia (56,65 %. El tiempo de aparición fue de 48 a 72 h (76,87 % y entre los factores agravantes se hallaron el nacimiento pretérmino y el bajo peso al nacer. La mayoría de los pacientes fueron tratados con luminoterapia (90,17 %. CONCLUSIÓN. La hiperbilirrubinemia neonatal agravada constituye un problema de salud. Los factores agravantes son la prematuridad y el bajo peso al nacer. La luminoterapia es una medida terapéutica eficaz para su tratamiento.INTRODUCTION. Most of times jaundice in newborn is a physiological fact due to hyperbilirubinemia of indirect predominance, secondary to liver immaturity and to bilirubin hyperproduction. The aim of present of present study was to determine the behavior of neonatal hyperbilirubinemia in the Gynecology and Obstetrics Teaching Hospital of Guanabacoa municipality from 2007 to 2009. METHODS. A retrospective and descriptive study was conducted in 173 newborn patients admitted in the Neonatology Department diagnosed with severe hyperbilirubinemia. RESULTS. The incidence of severe neonatal hyperbilirubinemia was of 3,67% with predominance in brothers with a history of jaundice (56,65%. The time of appearance was of 48 to 72 hrs (76,87% and among the aggravating factors were the preterm birth and

  1. Oxygen saturation index and severity of hypoxic respiratory failure.

    Science.gov (United States)

    Rawat, Munmun; Chandrasekharan, Praveen K; Williams, Ashley; Gugino, Sylvia; Koenigsknecht, Carmon; Swartz, Daniel; Ma, Chang Xing; Mathew, Bobby; Nair, Jayasree; Lakshminrusimha, Satyan

    2015-01-01

    The oxygenation index (OI = mean airway pressure, MAP × FiO2 × 100 : PaO2) is used to assess the severity of hypoxic respiratory failure (HRF) and persistent pulmonary hypertension of the newborn (PPHN). An indwelling arterial line or arterial punctures are necessary to obtain PaO2 for the calculation of OI. Oxygenation can be continuously and noninvasively assessed using pulse oximetry. The use of the oxygen saturation index (OSI = MAP × FiO2 × 100 : SpO2) can be an alternate method of assessing the severity of HRF. To evaluate the correlation between OSI and OI in the following: (1) neonates with HRF and (2) a lamb model of meconium aspiration syndrome. Human neonates: a retrospective chart review of 74 ventilated late preterm/term neonates with indwelling arterial access and SpO2 values in the first 24 h of life was conducted. OSI and OI were calculated and correlated. Lamb model: arterial blood gases were drawn and preductal SpO2 was documented in 40 term newborn lambs with asphyxia and meconium aspiration. OI and OSI were calculated and correlated with pulmonary vascular resistance (PVR). Mean values of OSI and OI showed a correlation coefficient of 0.952 in neonates (mean value of 308 observations in 74 neonates) and 0.948 in lambs (mean value of 743 observations in 40 lambs). In lambs, with increasing PVR, there was a decrease in OI and OSI. OSI correlates significantly with OI in infants with HRF. This noninvasive measure may be used to assess the severity of HRF and PPHN in neonates without arterial access. © 2015 S. Karger AG, Basel

  2. Operative vaginal delivery in case of persistent occiput posterior position after manual rotation failure: a 6-month follow-up on pelvic floor function.

    Science.gov (United States)

    Guerby, Paul; Parant, Olivier; Chantalat, Elodie; Vayssiere, Christophe; Vidal, Fabien

    2018-05-21

    To compare the short- and long-term perineal consequences (at 6 months postpartum) and short-term neonatal consequences of instrumental rotation (IR) to those induced by assisted delivery (AD) in the occiput posterior (OP) position, in case of manual rotation failure. A prospective observational cohort study; tertiary referral hospital including all women presenting with persistent OP position who delivered vaginally after manual rotation failure with attempted IR or AD in OP position from September 2015 to October 2016. Maternal and neonatal outcomes of all attempted IR deliveries were compared with OP operative vaginal deliveries. Main outcomes measured were pelvic floor function at 6 months postpartum including Wexner score for anal incontinence and ICIQ-FLUTS for urinary symptoms. Perineal morbidity comprised severe perineal tears, corresponding to third and fourth degree lacerations. Fetal morbidity parameters comprised low neonatal Apgar scores, acidaemia, major and minor fetal injuries and neonatal intensive care unit admissions. Among 5265 women, 495 presented with persistent OP positions (9.4%) and 111 delivered after manual rotation failure followed by AD delivery: 58 in the IR group and 53 in the AD in OP group. The incidence of anal sphincter injuries was significantly reduced after IR attempt (1.7% vs. 24.5%; p < 0.001) without increasing neonatal morbidity. At 6 months postpartum, AD in OP position was associated with higher rate of anal incontinence (30% vs. 5.5%, p = 0.001) and with more urinary symptoms, dyspareunia and perineal pain. OP operative deliveries are associated with significant perineal morbidity and pelvic floor dysfunction at 6 months postpartum.

  3. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... Ammonia, which is produced by your body when proteins are digested, is one of the toxins that’s ... as Hemochromatosis, Wilson disease and Alpha-1 antitrypsin deficiency Liver failure is a life-threatening condition that ...

  4. Protein synthesis in skeletal muscle of neonatal pigs is enhanced by administration of Beta-hydroxy-Beta-methylbutyrate

    Science.gov (United States)

    Many low-birth-weight infants experience failure to thrive. The amino acid leucine stimulates protein synthesis in skeletal muscle of the neonate, but less is known about the effects of the leucine metabolite Beta-hydroxy-Beta-methylbutyrate (HMB). To determine the effects of HMB on protein synthesi...

  5. Neonatal hyperthyroidism: neonatal clinical course of two brothers born to a mother with Graves-Basedow disease, before and after total thyroidectomy.

    Science.gov (United States)

    Zuppa, A A; Sindico, P; Savarese, I; D'Andrea, V; Fracchiolla, A; Cota, F; Romagnoli, C

    2007-04-01

    About 1-2% of infants born to mothers with Graves' disease or Hashimoto's thyroiditis develop neonatal hyperthyroidism because of transplacental passage of IgG stimulating TSH receptors (TRAb). To evaluate the effect of maternal total thyroidectomy on neonatal clinical course. We describe two brothers born to a mother with Graves' disease, before and after total thyroidectomy. The first child showed persistent tachycardia, the presence of TRAb and a laboratory pattern of hyperthyroidism. Lugol's solution was started and then propylthiouracil was added. Digitalis, furosemide and diazepam were necessary for treatment of heart failure, hypertension and irritability. On the 70th day of life, hormone serum levels normalized and treatment was interrupted. TRAb normalized by the third month of life. The second infant was born 2 years after the mother underwent total thyroidectomy. In spite of a laboratory pattern of hyperthyroidism and positivity to TRAb, he showed only considerable weight loss, and no therapy was required. TRAb may persist after total thyroidectomy: clinical and instrumental follow-up of the newborn is recommended.

  6. Efficacy of clonidine versus phenobarbital in reducing neonatal morphine sulfate therapy days for neonatal abstinence syndrome. A prospective randomized clinical trial.

    Science.gov (United States)

    Surran, B; Visintainer, P; Chamberlain, S; Kopcza, K; Shah, B; Singh, R

    2013-12-01

    To compare the efficacy of clonidine versus phenobarbital in reducing morphine sulfate treatment days for neonatal abstinence syndrome (NAS). Prospective, non-blinded, block randomized trial at a single level III NICU (Neonatal Intensive Care Unit). Eligible infants were treated with a combination of medications as per protocol. Primary outcome was treatment days with morphine sulfate. Secondary outcomes were the mean total morphine sulfate dose, outpatient phenobarbital days, adverse events and treatment failures. A total of 82 infants were eligible, of which 68 were randomized with 34 infants in each study group. Adjusting for covariates phenobarbital as compared with clonidine had shorter morphine sulfate treatment days (-4.6, 95% confidence interval (CI): -0.3, -8.9; P=0.037) with no difference in average morphine sulfate total dose (1.1 mg kg(-1), 95% CI: -0.1, 2.4; P=0.069). Post-discharge phenobarbital was continued for an average of 3.8 months (range 1 to 8 months). No other significant differences were noted. Phenobarbital as adjunct had clinically nonsignificant shorter inpatient but significant overall longer therapy time as compared with clonidine.

  7. Management of neonatal abstinence syndrome in neonates born to opioid maintained women.

    Science.gov (United States)

    Ebner, Nina; Rohrmeister, Klaudia; Winklbaur, Bernadette; Baewert, Andjela; Jagsch, Reinhold; Peternell, Alexandra; Thau, Kenneth; Fischer, Gabriele

    2007-03-16

    Neonates born to opioid-maintained mothers are at risk of developing neonatal abstinence syndrome (NAS), which often requires pharmacological treatment. This study examined the effect of opioid maintenance treatment on the incidence and timing of NAS, and compared two different NAS treatments (phenobarbital versus morphine hydrochloride). Fifty-three neonates born to opioid-maintained mothers were included in this study. The mothers received methadone (n=22), slow-release oral morphine (n=17) or buprenorphine (n=14) throughout pregnancy. Irrespective of maintenance treatment, all neonates showed APGAR scores comparable to infants of non-opioid dependent mothers. No difference was found between the three maintenance groups regarding neonatal weight, length or head circumference. Sixty percent (n=32) of neonates required treatment for NAS [68% in the methadone-maintained group (n=15), 82% in the morphine-maintained group (n=14), and 21% in the buprenorphine-maintained group (n=3)]. The mean duration from birth to requirement of NAS treatment was 33 h for the morphine-maintained group, 34 h for the buprenorphine-maintained group and 58 h for the methadone-maintained group. In neonates requiring NAS treatment, those receiving morphine required a significantly shorter mean duration of treatment (9.9 days) versus those treated with phenobarbital (17.7 days). Results suggest that morphine hydrochloride is preferable for neonates suffering NAS due to opioid withdrawal.

  8. Neonatal Acid-Base Status in Fetuses with Abnormal Vertebro- and Cerebro-Placental Ratios.

    Science.gov (United States)

    Morales-Roselló, José; Khalil, Asma; Ferri-Folch, Blanca; Perales-Marín, Alfredo

    2015-01-01

    A low cerebro-placental ratio (CPR) at term suggests the existence of failure to reach growth potential (FRGP) with a higher risk of poor neonatal acid-base status. This study aimed to evaluate whether similar findings were also seen in the vertebral artery (vertebro-placental ratio, VPR), supplying 30% of the cerebral flow. We studied term fetuses classified into groups according to birth weight (BW), CPR and VPR. BW was expressed in centiles and ratios in multiples of the median (MoM). Subsequently, associations with neonatal pH values were evaluated by means of regression curves and Mann-Whitney tests. VPR MoM correlated with BW centiles (p < 0.0001, R2 = 0.042) and its distribution resembled that of CPR MoM (p < 0.001). When both arteries were compared, adequate-for-gestational-age (AGA) fetuses with either low CPR or low VPR had lower neonatal venous pH values (p < 0.05, p < 0.01, respectively). However, in case of small-for-gestational-age (SGA) fetuses, only those with low VPR had significantly lower neonatal arterial and venous pH values (p < 0.05). Blood flow in the vertebral artery mimics that in the middle cerebral artery supporting the FRGP model. Both CPR and VPR identify AGA fetuses with lower neonatal pH values, but only VPR identifies SGA with lower pH values. Hypoxemia might be reflected as a generalized cerebral vasodilation demonstrated as low CPR and VPR.

  9. Neonatal pain

    Science.gov (United States)

    Walker, Suellen M

    2014-01-01

    Effective management of procedural and postoperative pain in neonates is required to minimize acute physiological and behavioral distress and may also improve acute and long-term outcomes. Painful stimuli activate nociceptive pathways, from the periphery to the cortex, in neonates and behavioral responses form the basis for validated pain assessment tools. However, there is an increasing awareness of the need to not only reduce acute behavioral responses to pain in neonates, but also to protect the developing nervous system from persistent sensitization of pain pathways and potential damaging effects of altered neural activity on central nervous system development. Analgesic requirements are influenced by age-related changes in both pharmacokinetic and pharmacodynamic response, and increasing data are available to guide safe and effective dosing with opioids and paracetamol. Regional analgesic techniques provide effective perioperative analgesia, but higher complication rates in neonates emphasize the importance of monitoring and choice of the most appropriate drug and dose. There have been significant improvements in the understanding and management of neonatal pain, but additional research evidence will further reduce the need to extrapolate data from older age groups. Translation into improved clinical care will continue to depend on an integrated approach to implementation that encompasses assessment and titration against individual response, education and training, and audit and feedback. PMID:24330444

  10. Neonatal and pediatric extracorporeal membrane oxygenation in developing Latin American countries.

    Science.gov (United States)

    Kattan, Javier; González, Álvaro; Castillo, Andrés; Caneo, Luiz Fernando

    To review the principles of neonatal-pediatric extracorporeal membrane oxygenation therapy, prognosis, and its establishment in limited resource-limited countries in Latino America. The PubMed database was explored from 1985 up to the present, selecting from highly-indexed and leading Latin American journals, and Extracorporeal Life Support Organization reports. Extracorporeal membrane oxygenation provides "time" for pulmonary and cardiac rest and for recovery. It is used in the neonatal-pediatric field as a rescue therapy for more than 1300 patients with respiratory failure and around 1000 patients with cardiac diseases per year. The best results in short- and long-term survival are among patients with isolated respiratory diseases, currently established as a standard therapy in referral centers for high-risk patients. The first neonatal/pediatric extracorporeal membrane oxygenation Program in Latin America was established in Chile in 2003, which was also the first program in Latin America to affiliate with the Extracorporeal Life Support Organization. New extracorporeal membrane oxygenation programs have been developed in recent years in referral centers in Argentina, Colombia, Brazil, Mexico, Perú, Costa Rica, and Chile, which are currently funding the Latin American Extracorporeal Life Support Organization chapter. The best results in short- and long-term survival are in patients with isolated respiratory diseases. Today extracorporeal membrane oxygenation therapy is a standard therapy in some Latin American referral centers. It is hoped that these new extracorporeal membrane oxygenation centers will have a positive impact on the survival of newborns and children with respiratory or cardiac failure, and that they will be available for an increasing number of patients from this region in the near future. Copyright © 2016 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  11. Neonatal and pediatric extracorporeal membrane oxygenation in developing Latin American countries

    Directory of Open Access Journals (Sweden)

    Javier Kattan

    Full Text Available Abstract Objective: To review the principles of neonatal-pediatric extracorporeal membrane oxygenation therapy, prognosis, and its establishment in limited resource-limited countries in Latino America. Sources: The PubMed database was explored from 1985 up to the present, selecting from highly-indexed and leading Latin American journals, and Extracorporeal Life Support Organization reports. Summary of the findings: Extracorporeal membrane oxygenation provides “time” for pulmonary and cardiac rest and for recovery. It is used in the neonatal-pediatric field as a rescue therapy for more than 1300 patients with respiratory failure and around 1000 patients with cardiac diseases per year. The best results in short- and long-term survival are among patients with isolated respiratory diseases, currently established as a standard therapy in referral centers for high-risk patients. The first neonatal/pediatric extracorporeal membrane oxygenation Program in Latin America was established in Chile in 2003, which was also the first program in Latin America to affiliate with the Extracorporeal Life Support Organization. New extracorporeal membrane oxygenation programs have been developed in recent years in referral centers in Argentina, Colombia, Brazil, Mexico, Perú, Costa Rica, and Chile, which are currently funding the Latin American Extracorporeal Life Support Organization chapter. Conclusions: The best results in short- and long-term survival are in patients with isolated respiratory diseases. Today extracorporeal membrane oxygenation therapy is a standard therapy in some Latin American referral centers. It is hoped that these new extracorporeal membrane oxygenation centers will have a positive impact on the survival of newborns and children with respiratory or cardiac failure, and that they will be available for an increasing number of patients from this region in the near future.

  12. Evaluation of Nutritional Status in a Teaching Hospital Neonatal Intensive Care Unit

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    Mohammadreza Rafati

    2015-02-01

    Full Text Available Background: Extrauterine growth restriction remains a common and serious problem in newborns especially who are small, immature, and critically ill. Very low birth weight infants (VLBW had 97% and 40% growth failure at 36 weeks and 18-22 months post-conceptual age respectively. The postnatal development of premature infants is critically dependent on an adequate nutritional intake that mimics a similar gestational stage. Deficient protein or amino acid administration over an extended period may cause significant growth delay or morbidity in VLBW infants. The purpose of this study was to evaluate current nutritional status in the neonatal intensive care unit in a teaching hospital. Methods: During this prospective observational study, the nutritional status of 100 consecutive critically ill neonates were evaluated by anthropometric and biochemical parameters in a tertiary neonatal intensive care unit. Their demographic characteristics (weight, height and head circumference, energy source (dextrose and lipid and protein were recorded in the first, 5th, 10th, 15th and 20th days of admission and blood samples were obtained to measure serum albumin and prealbumin. The amount of calorie and protein were calculated for all of preterm and term neonates and compared to standard means separately. Results: The calorie and amino acids did not meet in the majority of the preterm and term neonates and mean daily parenteral calorie intake was 30% or lower than daily requirements based on neonates’ weight. Mortality rate was significantly higher in neonates with lower serum albumin and severity of malnutrition but not with serum prealbumin concentration. Conclusion: Infants were studied did not receive their whole of daily calorie and protein requirements and it is recommended early and enough administration of calorie source (dextrose, lipids and amino acids. Prealbumin was a more benefit biochemical parameter than albumin to evaluate short term nutrition

  13. Maternal or neonatal infection: association with neonatal encephalopathy outcomes.

    Science.gov (United States)

    Jenster, Meike; Bonifacio, Sonia L; Ruel, Theodore; Rogers, Elizabeth E; Tam, Emily W; Partridge, John Colin; Barkovich, Anthony James; Ferriero, Donna M; Glass, Hannah C

    2014-07-01

    Perinatal infection may potentiate brain injury among children born preterm. The objective of this study was to examine whether maternal and/or neonatal infection are associated with adverse outcomes among term neonates with encephalopathy. This study is a cohort study of 258 term newborns with encephalopathy whose clinical records were examined for signs of maternal infection (chorioamnionitis) and infant infection (sepsis). Multivariate regression was used to assess associations between infection, pattern, and severity of injury on neonatal magnetic resonance imaging, as well as neurodevelopment at 30 mo (neuromotor examination, or Bayley Scales of Infant Development, second edition mental development index encephalopathy, chorioamnionitis was associated with a lower risk of brain injury and adverse outcomes, whereas signs of neonatal sepsis carried an elevated risk. The etiology of encephalopathy and timing of infection and its associated inflammatory response may influence whether infection potentiates or mitigates injury in term newborns.

  14. Tuberculosis neonatal

    OpenAIRE

    Pastor Durán, Xavier

    1986-01-01

    PROTOCOLOS TERAPEUTICOS. TUBERCULOSIS NEONATAL 1. CONCEPTO La tuberculosis neonatal es la infección del recién nacido producida por el bacilo de Koch. Es una situación rara pero grave que requiere un diagnóstico precoz y un tratamiento enérgico..

  15. Minimally Invasive Epicardial Pacemaker Implantation in Neonates with Congenital Heart Block.

    Science.gov (United States)

    Costa, Roberto; Silva, Katia Regina da; Martinelli Filho, Martino; Carrillo, Roger

    2017-10-01

    Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB. Among these, 12 (75.0%) had congenital heart defects associated with CCAVB. The patients had a mean age of 4.7 ± 5.3 days and nine (56.3%) were female. Bipolar steroid-eluting epicardial leads were implanted in all patients through a minimally invasive subxiphoid approach and fixed on the diaphragmatic ventricular surface. The pulse generator was placed in an epigastric submuscular position. All procedures were successful, with no perioperative complications or early deaths. Mean operating time was 90.2 ± 16.8 minutes. None of the patients displayed pacing or sensing dysfunction, and all parameters remained stable throughout the follow-up period of 4.1 ± 3.9 years. Three children underwent pulse generator replacement due to normal battery depletion at 4.0, 7.2, and 9.0 years of age without the need of ventricular lead replacement. There were two deaths at 12 and 325 days after pacemaker implantation due to bleeding from thrombolytic use and progressive refractory heart failure, respectively. Epicardial pacemaker implantation through a subxiphoid approach in neonates with CCAVB is technically feasible and associated with excellent surgical outcomes and pacing lead longevity.

  16. Prevalence and descriptive analysis of congenital heart disease in parturients: obstetric, neonatal, and anesthetic outcomes.

    Science.gov (United States)

    Warrick, Christine M; Hart, Jan E; Lynch, Anne M; Hawkins, Joy A; Bucklin, Brenda A

    2015-09-01

    The study objectives are to (1) assess prevalence of congenital heart disease (CHD), (2) describe outcomes of pregnancies in women with CHD, (3) compare outcomes in women with and without CHD, and (4) characterize neonatal outcomes in pregnancies complicated by CHD. This was a retrospective cohort study of women who delivered at the University of Colorado Hospital. Diagnosis of CHD was identified based on history of cardiac disease, pulmonary disease, or subacute bacterial endocarditis prophylaxis during labor and confirmed with echocardiogram when available. Comprehensive retrospective review of anesthetic, obstetric, and neonatal outcomes was performed. University of Colorado Hospital. 18,226 women. Medical record review. Valvular abnormalities, New York Heart Failure Association classification scores, types of CHD, maternal age, race, gravidity, parity, maternal prepregnancy body mass index, cigarette use, type of delivery, type of analgesia used, early initiation of neuraxial analgesia, arrhythmias, need for peripartum diuretics, prolonged maternal hospital stay, preterm birth, small for gestational age, neonatal CHD, neonatal or maternal intensive care unit (ICU) admissions, and maternal or neonatal death. We identified 117 pregnancies in 110 women with CHD. Parturients with CHD were more likely to have operative vaginal delivery (P neonatal ICU admissions (P = .003), and had prolonged hospital stays. Occurrence of CHD in neonates was 6%. Moderate-to-severe valvular disease was associated with increased rates of operative vaginal delivery, early initiation of neuraxial labor analgesia, cardiac complications (including arrhythmia and use of diuretics), prolonged hospital stay, and maternal ICU admission. However, most deliveries and births were uncomplicated; and there were one case each of maternal mortality and fetal death after birth. Operative abdominal deliveries and neonatal ICU admissions are more common in women with CHD, but these pregnancies are

  17. Effect of Hereditary Hemochromatosis Gene H63D and C282Y Mutations on Iron Overload in Sickle Cell Disease Patients

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    Yunus Kasım Terzi

    2016-12-01

    Full Text Available Objective: Hemochromatosis is an autosomal recessive disease that is one of the most important reasons for iron overload. Sickle cell disease is a hemoglobinopathy that occurs as a result of a homozygous mutation in the hemoglobin gene. Erythrocyte transfusion is frequently used in the treatment of this disease. Iron overload as a result of transfusion is important in the mortality and morbidity of sickle cell anemia patients as well as in other hemoglobinopathies. In this study, the effect of hemochromatosis gene (HFE p.H63D and p.C282Y mutations on transfusion-related cardiac and liver iron overload in sickle cell disease patients who carry homozygous hemoglobin S mutation has been investigated. Materials and Methods: This is a prospective single-center crosssectional study in patients with homozygous hemoglobin S mutation between the years 2008 and 2013. The patients were divided into two groups. The first group (group A, n=31 was receiving chelation therapy and the second group (group B, n=13 was not. Direct and indirect iron loads were analyzed by magnetic resonance imaging and biochemically, respectively. HFE gene mutations were analyzed by polymerase chain reaction-restriction fragment length polymorphism method. Statistical analyses were performed by independent samples t-test. Results: p.H63D mutation was detected in 10 (32.3% patients in group A and in only 1 patient (7.7% in group B. When the 2 groups were compared for iron overload, iron deposition in the liver was significantly higher in group B (p=0.046. In addition, in group A, iron deposition was significantly higher in HFE mutation carriers compared to patients without the mutation (p=0.05. Conclusion: Results of this study showed that HFE gene mutations are important in iron deposition in the liver in patients with sickle cell disease.

  18. A Rare Disorder with Common Clinical Presentation: Neonatal Bartter Syndrome.

    Science.gov (United States)

    Hussain, Shabbir; Tarar, Saba Haider; Al-Muhaizae, Muhammad

    2015-04-01

    Bartter syndrome is an autosomal recessive renal tubulopathy that presents with hypokalemic, hypochloremic metabolic alkalosis associated with increased urinary loss of sodium, potassium, calcium and chloride. There is hyperreninemia and hyperaldosteronemia but normotension. A full term male neonate was referred at 20-day of age with features of sepsis and respiratory distress. He was evaluated and managed as case of septicemia with all supportive paraphernalia including mechanical ventilation. Investigations revealed electrolytes imbalance and metabolic alkalosis suggestive of Neonatal Bartter Syndrome (NBS). Raised aldosterone and renin levels confirmed the diagnosis. Electrolyte imbalance was corrected with fluids and indomethacin, treated successfully, discharged and parents counseled. He was thriving well at 9 months of age. Another 2 months old male baby presented with recurrent episodes of lethargy with dehydration and failure to gain weight. Investigations confirmed the diagnosis of NBS. He was also successfully treated with same medication. We report these 2 cases because of the rarity of NBS, presentation of which may mimic common illnesses like sepsis and gastroenteritis.

  19. Neonatal doses from X ray examinations by birth weight in a neonatal intensive care unit

    Energy Technology Data Exchange (ETDEWEB)

    Ono, K.; Akahane, K.; Aota, T.; Hada, M.; Takano, Y.; Kai, M.; Kusama, T

    2003-07-01

    The aim of this study was to investigate the frequency and type of X ray examinations performed on neonates classified according to their birth weight in a neonatal intensive care unit (NICU). In this study, the radiology records of 2408 neonates who were admitted to the NICU of Oita Prefectural Hospital between January 1994 and September 1999 were investigated. This study revealed that the neonates with earlier gestational ages and lower birth weights required longer NICU stays and more frequent X ray examinations made using a mobile X ray unit. The average number of X ray examinations performed on neonates of less than 750 g birth weight was 26 films per neonate. In regard to computed tomography and fluoroscopy, no significant relationship was found between the birth weight and number of X rays. This study revealed that the entrance-surface dose per neonate was dependent upon the birth weight, while the maximum dose was not dependent upon the birth weight. The average neonatal dose in the NICU was predominantly from computed tomography and fluoroscopy. The individual dose varied widely among neonates. (author)

  20. Neonatal doses from X ray examinations by birth weight in a neonatal intensive care unit

    International Nuclear Information System (INIS)

    Ono, K.; Akahane, K.; Aota, T.; Hada, M.; Takano, Y.; Kai, M.; Kusama, T.

    2003-01-01

    The aim of this study was to investigate the frequency and type of X ray examinations performed on neonates classified according to their birth weight in a neonatal intensive care unit (NICU). In this study, the radiology records of 2408 neonates who were admitted to the NICU of Oita Prefectural Hospital between January 1994 and September 1999 were investigated. This study revealed that the neonates with earlier gestational ages and lower birth weights required longer NICU stays and more frequent X ray examinations made using a mobile X ray unit. The average number of X ray examinations performed on neonates of less than 750 g birth weight was 26 films per neonate. In regard to computed tomography and fluoroscopy, no significant relationship was found between the birth weight and number of X rays. This study revealed that the entrance-surface dose per neonate was dependent upon the birth weight, while the maximum dose was not dependent upon the birth weight. The average neonatal dose in the NICU was predominantly from computed tomography and fluoroscopy. The individual dose varied widely among neonates. (author)

  1. Hemochromatosis C282Y gene mutation as a potential susceptibility factor for iron-overload in Egyptian beta-thalassemia patients

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    G.M. Mokhtar

    2018-04-01

    Full Text Available Background: Hereditary hemochromatosis is the most frequent cause of primary iron overload that is associated with HFE gene’s mutation especially the C282Y mutation. The interaction between hemoglobin chain synthesis’ disorders and the C282Y mutation may worsen the clinical picture of beta-thalassemia major (β-TM. Aim: To establish the prevalence of the C282Y mutations in Egyptian β-TM patients and to address its adverse effects. Methods: Two-hundred and five β-TM patients were recruited and divided into two groups based on their serum ferritin (SF; group I (N = 125 (SF ≤ 2500 ng/dl and group II (N = 80 (SF > 2500 ng/dl. All patients were subjected to clinical and laboratory assessment with special emphasis on iron overload complications. Genotyping was assessed by polymerase chain reaction for detection of C282Y mutation in HFE gene. Results: The C282Y mutation was not detected in the studied β-TM neither in homozygous nor heterozygous state. There were several iron overload complications including cardiac complication (9.1%, liver disease (36.6%, delayed puberty (56.6%, primary (35.71% and secondary amenorrhea (21.42%, short stature (27.3%, diabetes (3.4%, neutropenia (9.7%, arthralgia (10.2%, gastrointestinal (21.1%, depression (2.9% and others (12.05%. Group I showed a statistically significant lower rate of taking iron-rich diet when compared to group II. Group II showed significant longer mean duration of disease, higher total transfusion rate per life, lower mean HbF% level, higher mean HbA% level, and higher rate of elevated liver enzymes than patients with SF ≤ 2500 ng/dl. Conclusion: The C282Y mutation was not detected in the studied cohort of Egyptian β-TM patients neither in homozygous nor heterozygous state in spite of manifestations of iron overload complications. Keywords: Beta-thalassemia major, Hereditary hemochromatosis, The C282Y mutation, Iron overload complications, Egyptian

  2. Severe anemia and hydrops in a neonate with parvovirus B19 infection: a case report

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    Negar Sajjadian

    2013-12-01

    Full Text Available Background: Anemia at the time of birth may cause some problem like asphyxia, heart failure shock or even death in a neonate. Different etiologies can be considered for this problem. Parvovirus B19, as a viral organism, can cause hydrops fetalis and neonatal anemia and consequent complications. We present here a case of newborn infant with severe anemia who had human parvovirus B19 infection.Case Presentation: A male newborn with gestational age of 36 week was born from a mother with poor prenatal care and history of contact with domestic animal. The neonate was very pale with Apgar score 2 at 1 min and received resuscitation, mechanical ventilation and repeated blood transfusion The hemoglobin level was significantly low. Analysis was made based on the clinical presentations. According to the case history, physical and laboratory findings, neonatal severe anemia induced by parvovirus B19 infection was suggested and Laboratory work up documented his infection with parovirus B19.Conclusion: Parvovirus B19 (B19 virus is the smallest single strand linear DNA virus in animal viruses, which is the only strain of parvovirus that is pathogenic in humans. Human parvovirus B19 may cross the placenta and result in fetal infection, morbidity and death. Parvovirus is an uncommon cause of neonatal anemia and hydrops fetalis so this etiology must be considered in differential diagnosis of anemia at birth.

  3. Neonatal neurosonography

    Energy Technology Data Exchange (ETDEWEB)

    Riccabona, Michael, E-mail: michael.riccabona@klinikum-graz.at

    2014-09-15

    Paediatric and particularly neonatal neurosonography still remains a mainstay of imaging the neonatal brain. It can be performed at the bedside without any need for sedation or specific monitoring. There are a number of neurologic conditions that significantly influence morbidity and mortality in neonates and infants related to the brain and the spinal cord; most of them can be addressed by ultrasonography (US). However, with the introduction of first CT and then MRI, neonatal neurosonography is increasingly considered just a basic first line technique that offers only orienting information and does not deliver much relevant information. This is partially caused by inferior US performance – either by restricted availability of modern equipment or by lack of specialized expertise in performing and reading neurosonographic scans. This essay tries to highlight the value and potential of US in the neonatal brain and briefly touching also on the spinal cord imaging. The common pathologies and their US appearance as well as typical indication and applications of neurosonography are listed. The review aims at encouraging paediatric radiologists to reorient there imaging algorithms and skills towards the potential of modern neurosonography, particularly in the view of efficacy, considering growing economic pressure, and the low invasiveness as well as the good availability of US that can easily be repeated any time at the bedside.

  4. Socioeconomic factors and adolescent pregnancy outcomes: distinctions between neonatal and post-neonatal deaths?

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    Flick Louise H

    2005-07-01

    Full Text Available Abstract Background Young maternal age has long been associated with higher infant mortality rates, but the role of socioeconomic factors in this association has been controversial. We sought to investigate the relationships between infant mortality (distinguishing neonatal from post-neonatal deaths, socioeconomic status and maternal age in a large, retrospective cohort study. Methods We conducted a population-based cohort study using linked birth-death certificate data for Missouri residents during 1997–1999. Infant mortality rates for all singleton births to adolescent women (12–17 years, n = 10,131; 18–19 years, n = 18,954 were compared to those for older women (20–35 years, n = 28,899. Logistic regression was used to estimate adjusted odds ratios (OR and 95% confidence intervals (CI for all potential associations. Results The risk of infant (OR 1.95, CI 1.54–2.48, neonatal (1.69, 1.24–2.31 and post-neonatal mortality (2.47, 1.70–3.59 were significantly higher for younger adolescent (12–17 years than older (20–34 years mothers. After adjusting for race, marital status, age-appropriate education level, parity, smoking status, prenatal care utilization, and poverty status (indicated by participation in WIC, food stamps or Medicaid, the risk of post-neonatal mortality (1.73, 1.14–2.64 but not neonatal mortality (1.43, 0.98–2.08 remained significant for younger adolescent mothers. There were no differences in neonatal or post-neonatal mortality risks for older adolescent (18–19 years mothers. Conclusion Socioeconomic factors may largely explain the increased neonatal mortality risk among younger adolescent mothers but not the increase in post-neonatal mortality risk.

  5. Neonatal adrenal hemorrhage presenting as late onset neonatal jaundice

    OpenAIRE

    Qureshi, Umar Amin; Ahmad, Nisar; Rasool, Akhter; Choh, Suhail

    2009-01-01

    Clinical manifestations of adrenal hemorrhage vary depending on the degree and rate of hemorrhage, as well as the amount of adrenal cortex compromised by hemorrhage. We report here a case of neonatal adrenal hemorrhage that presented with late onset neonatal jaundice. The cause of adrenal hemorrhage was birth asphyxia.

  6. Citrin deficiency: a novel cause of failure to thrive that responds to a high-protein, low-carbohydrate diet.

    Science.gov (United States)

    Dimmock, David; Kobayashi, Keiko; Iijima, Mikio; Tabata, Ayako; Wong, Lee-Jun; Saheki, Takeyori; Lee, Brendan; Scaglia, Fernando

    2007-03-01

    The proband was born at 36 weeks, appropriate for gestational age, to nonconsanguineous white parents. There was no evidence of hyperbilirubinemia or intrahepatic cholestasis in the neonatal period, and she had normal newborn screen results. She presented with 3 episodes of life-threatening bleeding and anemia. The diagnostic evaluation for her bleeding diathesis revealed an abnormal clotting profile with no biochemical evidence for hepatocellular damage. She was incidentally noted to have severe growth deceleration that failed to respond to 502 kJ/kg (120 kcal/kg) per day of protein-hydrolyzed formula. An extensive diagnostic workup for failure to thrive, which was otherwise normal, included plasma amino acid analysis that revealed hyperglutaminemia and citrulline levels within the reference range. Testing of a repeat sample revealed isolated hypercitrullinemia. No argininosuccinic acid was detected. Her ammonia level and urine orotic acid were within the reference ranges. Subsequent plasma amino acid analysis exhibited a profile suggestive of neonatal intrahepatic cholestasis caused by citrin deficiency with elevations in citrulline, methionine, and threonine. Western blotting of fibroblasts demonstrated citrin deficiency, and a deletion for exon 3 was found in the patient's coding DNA of the SLC25A13 gene. On the basis of the experience with adults carrying this condition, the patient was given a high-protein, low-carbohydrate diet. The failure to thrive and bleeding diathesis resolved. When compliance with the dietary prescription was relaxed, growth deceleration was again noted, although significant bleeding did not recur. This is the first report of an infant of Northern European descent with citrin deficiency. The later age at presentation with failure to thrive and bleeding diathesis and without obvious evidence of neonatal intrahepatic cholestasis expands the clinical spectrum of citrin deficiency. This case emphasizes the importance of continued dietary

  7. Exposure assessment of neonates in israel to x-ray radiation during hospitalization at neonatal intensive care unit

    International Nuclear Information System (INIS)

    Datz, H.

    2005-03-01

    Nowadays nearly 10% of all births in western countries are premature. In the last decade, there has been an increase of 45% in the number of neonates that were born in Israel. At the same time, the survival of neonates, especially those with very low birth weight, VLBW, (less than 1,500 gr), has increased dramatically. Diagnostic radiology plays an important role in the assessment and treatment of neonates requiring intensive care. During their prolonged and complex hospitalization, these infants are exposed to multiple radiographic examinations involving X-ray radiation. The extent of the examinations that the infant undergoes depends on its birth weight, gestational age and its medical problems, where most of the treatment effort is focused especially on VLBW neonates. Most of the diagnostic X-ray examinations taken during the hospitalization of neonates in the neonatal intensive care unit (NICU) consist of imaging of the respiratory and gastrointestinal systems, namely, the chest and abdomen. The imaging process is done using mobile X-ray units located at the NICUs. Due to their long hospitalization periods and complex medical condition, all neonates, and neonates with VLBW in particular, are exposed to a much higher level of diagnostic radiation, compared to normal newborns. The goal of this research was to assess the extent of the exposure of neonates in Israel to X-ray radiation during their hospitalization at the neonatal intensive care unit. Five NICUs, located at different geographical zones in Israel and treating 20% of all newborns in Israel every year, participated in this research. The research was conducted in three phases: Phase I: Collection of information on radiographic techniques and exposure parameters (e.g. kV, mAs, focus to skin distance (FSD), examination borders). 499 X-ray examinations (from 157 neonates) were evaluated for necessary and unnecessary exposure of the neonate's organs to X-ray radiation during these examinations. Phase II

  8. Comparing the Efficacy of Face Mask CPAP with Nasopharyngeal CPAP for Neonatal Transport after Delivery

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    Manizheh Mostafa-Gharehbaghi

    2013-09-01

    Full Text Available Background: Preterm infants have respiratory failure and complications because surfactant in alveolus is low. CPAP (Continuous positive airway pressure is a method for respiratory support in pre-term neonates and is provided by different equipment and methods. This study aims to compare two different routes of CPAP delivery in preterm newborn infants and to determine the need for surfactant replacement therapy in two groups. Materials and Methods: This is a randomized controlled clinical trial. Eighty four preterm infants delivered in Al-Zahra Hospital with gestational age 28-32 weeks were enrolled in this study from January 2012 to September 2012. They were randomly allocated in two groups. After initial stabilization in delivery room, forty two infants transferred to neonatal intensive care unit (NICU with face mask CPAP and 42 infant with nasopharyngeal CPAP and continued nasal CPAP in the NICU in both groups. All infants were followed for developing respiratory distress and need for surfactant replacement therapy and oxygen dependency till discharge.Results: The neonates that treated with two methods of CPAP delivery were similar with respect to gestation age, birth weight and other demographic characteristics. Twenty three neonates (65.5% in face mask group and 15 neonates (39.5% in nasopharyngeal CPAP group need surfactant replacement therapy (p=0.08.Conclusion: Mask CPAP or nasopharyngeal CPAP can used in preterm infants after delivery for neonatal transfer to NICU. This study showed no method of CPAP delivery is preferable to other in decreasing the need for surfactant therapy.

  9. A Case Report of Primary Cardiac Tumor in A Neonate

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    Sh. Rejaei

    2008-04-01

    Full Text Available Introduction: Primary cardiac tumors are extremely rare in infants and children . Most primary cardiac tumors in pediatric age group are benign, and less than 10% of such tumors are malignant. Many of these tumors are asymptomatic and incidentally diagnosed. The clinical manifestations are very different and includes direct cardiac effect, systemic effect , and embolic phenomena. Every infant or child with an unusual cardiac murmur, unexplained congestive heart failure, or arrhythmia should be evaluated for cardiac tumors. Echocardiography has contributed significantly to the evaluation of these patients. Surgery is the only treatment for primary cardiac tumors that require intervention with a relatively good prognosis. Case Report: The patient was a 20 days old neonate presented with severe congestive heart failure. Evaluation of the patient showed primary cardiac tumor in the left atrium and ventricle. We recommended surgical removal of the tumor but her parents denied surgical intervention at all. Conclusion: After about one year follow up, congestive heart failure symptoms were controlled and the tumor size was decreased.

  10. Effect of Intravenous Administration of Contrast Media on Serum Creatinine Levels in Neonates.

    Science.gov (United States)

    Bedoya, Maria A; White, Ammie M; Edgar, J Christopher; Pradhan, Madhura; Raab, Elisabeth L; Meyer, James S

    2017-08-01

    material). Similar findings were observed for GFRs. None of the neonates developed nephrogenic systemic fibrosis. Conclusion In the absence of known renal failure, neonates receiving standard inpatient care do not appear to be at increased risk for developing renal toxicity due to administration of intravenous iodine- and gadolinium-based contrast material. © RSNA, 2017.

  11. INFLUENCE OF FETOPLACENTAL INSUFFICIENCY ON THE FORMATION OF PERINATAL PATHOLOGY IN PRETERM NEONATES

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    N. P. Molokanova

    2013-01-01

    Full Text Available Aim: to identify features of early neonatal period in preterm infants of women with fetoplacental insufficiency (FPI. Patients and methods: we conducted a retrospective study of early neonatal period in 76 preterm infants with gestational age from 28 to 36 weeks. Of these, 31 were born by women with FPI (study group and 45 neonates from mothers without complications during pregnancy (control group. We analyzed course of pregnancy and delivery in both groups. Infant’s conditions were assessed using Apgar and Silverman Score. Neurological status, severity of respiratory and cardio-vascular failure were take into account in the first 7 days of life. Results: women with FPI had significantly higher levels of total extragenital pathology, hypertension was found only in mothers of the main group. The extent of Doppler abnormalities in most cases was light, while the most severe 3d grade was detected only in 4 women (12,9%, which had caused the birth of infants in severe asphyxia. Pathology of respiratory and central nervous system was dominated in preterm infants of both groups. Conclusions: the number of infants with serious conditions born by mothers with FPI is significantly higher than that determined by the deterioration of the utero-placental blood flow. Preterm neonates in women with FPI form a risk group of perinatal asphyxia.

  12. Neonatal pain management

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    Tarun Bhalla

    2014-01-01

    Full Text Available The past 2-3 decades have seen dramatic changes in the approach to pain management in the neonate. These practices started with refuting previously held misconceptions regarding nociception in preterm infants. Although neonates were initially thought to have limited response to painful stimuli, it was demonstrated that the developmental immaturity of the central nervous system makes the neonate more likely to feel pain. It was further demonstrated that untreated pain can have long-lasting physiologic and neurodevelopmental consequences. These concerns have resulted in a significant emphasis on improving and optimizing the techniques of analgesia for neonates and infants. The following article will review techniques for pain assessment, prevention, and treatment in this population with a specific focus on acute pain related to medical and surgical conditions.

  13. Thyroid function in neonatal hypothyroidism.

    Science.gov (United States)

    Dussault, J H; Letarte, J; Guyda, H; Laberge, C

    1976-10-01

    Various aspects of the thyroid function have been measured in 28 cases of neonatal hypothyroidism detected by means of the Quebec Screening Program for Metabolic Diseases. In all instances the T4 value in the blood of filter paper spot was below 2 SD of the mean of the day, averaging 0.39 +/- 0.04 ng/40 mul (mean +/- SEM) of eluted blood. The T4 value of a second similar sample averaged 0.22 +/- 0.04 ng/mul of eluted blood; this value was significantly lower than the first one. The serum T4 concentration was decreased in all the infants, whereas three of them had a normal serum TSH concentration. At least three groups of patients could be identified: (1) patients with primary thyroid failure, (2) those with secondary or tertiary hypothyrodism, and (3) those with abnormal synthesis of thyroid hormone.

  14. Lethal neonatal short-limbed dwarfism

    International Nuclear Information System (INIS)

    Kim, Ok Hwa; Yim, Chung Ik; Bahk, Yong Whee

    1986-01-01

    We have detailed our experiences on 6 cases of neonatal lethal short-limbed dwarfism and reviewed the articles. They include, achondrogenesis, thanatophoric dysplasia, asphyxiating thoracic dysplasia, osteogenesis imperfect a congenita, and hypophosphatasia lethals. Five babies were born alive but died soon after birth and one was a stillbirth. The main cause of failure to thrive was respiratory insufficiency. Each case was having quite characteristic radiologic findings, even if the general appearances were similar to the achondroplasts clinically. Precise diagnosis is very important for genetic counselling of the parents and alarm to them the possibility of bone dysplasias to the next offsprings. For this purpose, the radiologists play major role for the correct diagnosis. We stress that when the baby is born with short-limbed dwarfism, whole body radiogram should be taken including lateral view and postmortem radiogram is also very precious.

  15. Lethal neonatal short-limbed dwarfism

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok Hwa; Yim, Chung Ik; Bahk, Yong Whee [Catholic Medical College, Seoul (Korea, Republic of)

    1986-02-15

    We have detailed our experiences on 6 cases of neonatal lethal short-limbed dwarfism and reviewed the articles. They include, achondrogenesis, thanatophoric dysplasia, asphyxiating thoracic dysplasia, osteogenesis imperfect a congenita, and hypophosphatasia lethals. Five babies were born alive but died soon after birth and one was a stillbirth. The main cause of failure to thrive was respiratory insufficiency. Each case was having quite characteristic radiologic findings, even if the general appearances were similar to the achondroplasts clinically. Precise diagnosis is very important for genetic counselling of the parents and alarm to them the possibility of bone dysplasias to the next offsprings. For this purpose, the radiologists play major role for the correct diagnosis. We stress that when the baby is born with short-limbed dwarfism, whole body radiogram should be taken including lateral view and postmortem radiogram is also very precious.

  16. Causes of Neonatal Mortality in the Neonatal Intensive Care Unit of Taleghani Hospital

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    Ali Hossein Zeinalzadeh

    2017-09-01

    Full Text Available Background: Neonatal survival is one of the most important challenges today. Over 99% of neonatal mortalities occur in the developing countries, and epidemiologic studies emphasize on this issue in the developed countries, as well. In this study, we attempted to investigate the causes of neonatal mortality in Taleghani Hospital, Tabriz, Iran.Methods: In this cross-sectional study, we studied causes of neonatal mortality in neonatal intensive care unit (NICU of Taleghani Hospital, Tabriz, Iran, during 2013-2014. Data collection was performed by the head nurse and treating physician using a pre-designed questionnaire. Most of the data were extracted from the neonatal records. Information regarding maternal underlying diseases and health care during pregnancy was extracted from mothers' records.Results: A total of 891 neonates were admitted to NICU of Taleghani Hospital of Tabriz, Iran, during 2013-2014, 68 (7.5% of whom died. Among these cases, 37 (%54.4 were male, 29 (29.4% were extremely low birth weight, and 16 (23.5% weighed more than 2.5 kg. The main causes of mortality were congenital anomalies (35.3%, prematurity (26.5%, and sepsis (10.3%, respectively.Conclusion: Congenital anomaly is the most common cause of mortality, and the pattern of death is changing from preventable diseases to unavoidable mortalities

  17. Developments in neonatal care and nursing responses.

    Science.gov (United States)

    Healy, Patricia; Fallon, Anne

    This article reviews the origins and evolution of neonatology and considers the role of the neonatal nurse within this specialty. Neonatal nurses are a vital part of the neonatal team that provides care for sick babies. The nursing care required by sick babies and their families on a neonatal unit can be variable and complex. The past century has seen significant changes in the role of the neonatal nurse. This has come about through dramatic technological developments on neonatal units, an increased understanding of neonatal physiology and pathology, changes in the education of neonatal nurses, and active and ongoing clinical research within the specialty. The resulting significant advances in neonatal care, including that provided by neonatal nurses, have made a crucial and steadfast contribution to marked improvements in neonatal outcomes.

  18. Neonatal irradiation nephropathy in the growing dog. I. Renal morphological and functional adaptations following neonatal, sublethal, whole-body irradiation

    International Nuclear Information System (INIS)

    Wilke, W.L.; Phemister, R.D.; Jaenke, R.S.

    1979-01-01

    Sixty beagles were used to study the effects of exposure to 330 R 60 Co γ radiation (bilateral, whole-body) at 2 days of age on renal functional and morphological development in the growing dog. A significant deficit in grams kidney per kilogram body weight was found in irradiated dogs at 50 days of age (P < 0.05), but not at 125 or 200 days of age. Glomerular filtration rate (GFR) per kilogram body weight and GFR per gram kidney were not significantly different between irradiated and nonirradiated dogs at 50, 125, or 200 days of age, but blood urea nitrogen (BUN) was significantly elevated in irradiated dogs throughout this period (P < 0.05). The fractional distribution of intracortical renal blood flow, as determined by radiolabeled microspheres, to the outermost cortex was found to be reduced in irradiated animals at all ages evaluated (P < 0.05). The fractional blood flow to the outermost renal cortex was negatively correlated with BUN in both irradiated (P < 0.05) and nonirradiated (P < 0.05) animals. Based on prior demonstrations of reductions in nephron numbers following similar irradiation, these data indicate increases in mean single nephron GFR and nephronal hypertrophy in the kidneys of the neonatally irradiated dog. The renal functional and morphological adaptations are sufficient to maintain adequate renal function in growing, neonatally irradiated dogs. The BUN elevations in irradiated dogs are believed to be related to changes in intracortical renal blood flow, rather than indicating renal insufficiency. The possible importance of the functional and morphological adaptations to the subsequent development of chronic renal failure in neonatally irradiated animals is discussed

  19. Incidence and risk factors of acute kidney injury among the critically ill neonates

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    Ayman A El-Badawy

    2015-01-01

    Full Text Available Acute kidney injury (AKI is a complex disorder with clinical manifestations ranging from mild dysfunction to complete kidney failure. The published literature on the incidence and outcome of AKI in the critically ill neonatal population is scarce. The aim of this study was to evaluate the types, the associated risk factors and short-term outcome of AKI in the critically ill neonates. A cohort study was conducted including 100 critically ill neonates successively admitted to the Neonatal Intensive Care Unit. The inclusion criteria were a gestational age ≥28 weeks and body weight ≥1 kg. Exclusion criteria included those with multiple congenital anomalies or on drugs altering glomerular filtration rate or AKI developing postoperatively. Neonates were evaluated for the development of AKI [creatinine >1.5 mg/dL and/or blood urea nitrogen (BUN >20 mg/dL] and were assigned as group A (who developed AKI and group B (who did not develop AKI. Forty-one patients developed AKI (group A among whom nine (22% showed oliguric AKI. The most common risk factors among group A patients were sepsis (75.6% and nephrotoxic drug administration (75.6%, followed by shock (39%. There were no statistically significant differences between both groups except for male sex predominance and necrotizing enterocolitis (NEC, which were significantly higher among group A (P <0.05. Use of continuous positive airway pressure (CPAP ventilation was significantly higher in neonates without AKI (13.6% vs 0.0%, P = 0.02. The mortality rate among group A reached 51.2%. Various risk factors including gender, gestational age, birth weight, shock, NEC, sepsis, nephrotoxic drugs, oliguria and mechanical ventilation were studied as regards outcome of group A, and all factors except gender and oliguria proved to be significantly higher in deceased neonates. Male sex and NEC were important risk factors for developing AKI that was predominantly non-oliguric. CPAP ventilation may have a

  20. Patterns of admission and factors associated with neonatal mortality among neonates admitted to the neonatal intensive care unit of University of Gondar Hospital, Northwest Ethiopia

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    Demisse AG

    2017-05-01

    Full Text Available Abayneh Girma Demisse, Fentahun Alemu, Mahlet Abayneh Gizaw, Zemene Tigabu School of Medicine, College of Medicine and Health Science, University of Gondar, Gondar, Ethiopia Introduction: The neonatal period is a highly vulnerable time for an infant completing many of the physiologic adjustments required for life outside the uterus. As a result, there are high rates of morbidity and mortality. The three major causes of mortality in developing countries include prematurity, infection, and perinatal asphyxia. The aim of this study was to identify the patterns of neonatal admission and factors associated with mortality among neonates admitted at the Neonatal Intensive Care Unit (NICU of University of Gondar Hospital.Materials and methods: A retrospective cross-sectional study was conducted among all admitted neonates in the NICU of University of Gondar referral hospital from December 1, 2015 to August 31, 2016. Information was extracted retrospectively during admission from patient records and death certificates, using a pretested questionnaire. The data were entered and analyzed using SPSS version 20, and p-values <0.05 were considered statistically significant.Results: A total of 769 neonates was included in the study. There were 448 (58.3% male neonates, and 398 (51.8% neonates were rural residents. More than two-thirds of the 587 deliveries (76.3% were performed in tertiary hospitals. Neonatal morbidity included hypothermia 546 (71%, sepsis 522 (67.9%, prematurity 250 (34.9%, polycythemia 242 (31.5%, hypoglycemia 142 (18.5, meconium aspiration syndrome 113 (14.7%, and perinatal asphyxia 96 (12.5%. The overall mortality was 110 (14.3%; 95% confidence interval [CI]: 11.9–16.9 of which 69 (62.7% deaths occurred in the first 24 hours of age. In the multivariate analysis, mortality was associated with perinatal asphyxia (adjusted odds ratio [AOR]: 5.97; 95% CI: 3.06–11.64, instrumental delivery (AOR: 2.99; 95% CI: 1.08–8.31, and early onset

  1. Volume and leak measurements during neonatal CPAP in neonates

    OpenAIRE

    Fischer, Hendrik S.

    2011-01-01

    As yet, little is known about the effects of air leakages during CPAP in newborns. The present doctoral dissertation investigates tidal volume and leak measurements during nasal continuous positive airway pressure in neonates using a commercial ventilatory device. Investigations include in vitro studies, modelling and computer simulation as well as a clinical randomized cross-over trial in neonates.

  2. Efficacy and safety of bubble CPAP in neonatal care in low and middle income countries: a systematic review.

    Science.gov (United States)

    Martin, Simone; Duke, Trevor; Davis, Peter

    2014-11-01

    Forty per cent of global child deaths occur in the neonatal period. Low and middle income countries need effective and simple methods to improve hospital-based neonatal care. Bubble continuous positive airway pressure (CPAP) may have a role in improving the quality of respiratory support in hospitals in low and middle income countries. To examine the evidence for the efficacy and safety of bubble CPAP in neonates with respiratory distress in low and middle income settings. A systematic search (1946-March 2014) was performed of Pubmed, Ovid MEDLINE, Web of Science, Google Scholar and the references of relevant articles. Articles meeting inclusion criteria (CPAP for respiratory distress in infants CPAP compared with oxygen therapy, followed by mechanical ventilation if required, reduced the need for mechanical ventilation by 30%-50%. In another three trials comparing bubble CPAP with ventilator CPAP, mortality and complication rates were similar, while meta-analysis of CPAP failure in these same trials showed a lower failure rate in the bubble CPAP groups (p CPAP is safe and reduces the need for mechanical ventilation. Further research into the efficacy of bubble CPAP in low-income and middle-income countries is needed. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  3. Telemedicine in Neonatal Home Care

    DEFF Research Database (Denmark)

    Holm, Kristina Garne; Brødsgaard, Anne; Zachariassen, Gitte

    2016-01-01

    participatory design and qualitative methods. Data were collected from observational studies, individual interviews, and focus group interviews. Two neonatal units participated. One unit was experienced in providing neonatal home care with home visits, and the other planned to offer neonatal home care......BACKGROUND: For the majority of preterm infants, the last weeks of hospital admission mainly concerns tube feeding and establishment of breastfeeding. Neonatal home care (NH) was developed to allow infants to remain at home for tube feeding and establishment of breastfeeding with regular home...... visits from neonatal nurses. For hospitals covering large regions, home visits may be challenging, time consuming, and expensive and alternative approaches must be explored. OBJECTIVE: To identify parental needs when wanting to provide neonatal home care supported by telemedicine. METHODS: The study used...

  4. Neonatal Informatics: Transforming Neonatal Care Through Translational Bioinformatics

    Science.gov (United States)

    Palma, Jonathan P.; Benitz, William E.; Tarczy-Hornoch, Peter; Butte, Atul J.; Longhurst, Christopher A.

    2012-01-01

    The future of neonatal informatics will be driven by the availability of increasingly vast amounts of clinical and genetic data. The field of translational bioinformatics is concerned with linking and learning from these data and applying new findings to clinical care to transform the data into proactive, predictive, preventive, and participatory health. As a result of advances in translational informatics, the care of neonates will become more data driven, evidence based, and personalized. PMID:22924023

  5. Maternal haemoglobin and short-term neonatal outcome in preterm neonates.

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    Elodie Savajols

    Full Text Available To determine whether there is a significant association between maternal haemoglobin measured before delivery and short-term neonatal outcome in very preterm neonates.We included prospectively all live births occurring from 25 to 32+6 weeks of gestation in a tertiary care centre between January 1(st 2009 and December 31(st 2011. Outborn infants and infants presenting with lethal malformations were excluded. Three hundred and thirty-nine mothers and 409 infants met the inclusion criteria. For each mother-infant pair a prospective record of epidemiologic data was performed and maternal haemoglobin concentration recorded within 24 hours before delivery was retrospectively researched. Maternal haemoglobin was divided into quartiles with the second and the third one regarded as reference as they were composed of normal haemoglobin values. Short-term outcome was defined as poor in case of death during hospital stay and/or grades III/IV intraventricular haemorrhage and/or periventricular leukomalacia and/or necessity of ventriculoperitoneal shunt.The global rate of poor short-term neonatal outcome was 11.4% and was significantly associated with low maternal haemoglobin values. This association remained significant after adjustment for antenatal corticosteroids therapy, gestational age, parity, mechanism of preterm birth, mode of delivery and birth weight (aOR = 2.97 CI 95% [1.36-6.47]. There was no relation between short-term neonatal outcome and high maternal haemoglobin concentration values.We show that low maternal haemoglobin concentration at delivery is an independent risk factor for poor short-term neonatal outcome in very preterm neonates. This study is one of the first to show such an association within the preterm population.

  6. Obstructive choledocholithiasis requiring intervention in a three week old neonate: A case report and review of the literature

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    Lindsay E. Peters

    2016-01-01

    Full Text Available The discovery of cholelithiasis in neonates is often incidental, however obstructing common bile duct stones are rare. Herein we report the case of a 3 week old neonate who presented with obstructive choledocholithiasis. The patient was treated conservatively with antibiotics and ursodeoxycholic acid but did not improve. He was therefore taken to surgery for cholecystectomy and stone extraction. The operation was successful and his transaminases and bilirubin levels declined. Trials of conservative management can be attempted in asymptomatic infants with choledocholithiasis. However, failure of the stone to pass or ongoing signs of cholecystitis should be met with operative intervention to remove the obstruction.

  7. Neonates need tailored drug formulations.

    Science.gov (United States)

    Allegaert, Karel

    2013-02-08

    Drugs are very strong tools used to improve outcome in neonates. Despite this fact and in contrast to tailored perfusion equipment, incubators or ventilators for neonates, we still commonly use drug formulations initially developed for adults. We would like to make the point that drug formulations given to neonates need to be tailored for this age group. Besides the obvious need to search for active compounds that take the pathophysiology of the newborn into account, this includes the dosage and formulation. The dosage or concentration should facilitate the administration of low amounts and be flexible since clearance is lower in neonates with additional extensive between-individual variability. Formulations need to be tailored for dosage variability in the low ranges and also to the clinical characteristics of neonates. A specific focus of interest during neonatal drug development therefore is a need to quantify and limit excipient exposure based on the available knowledge of their safety or toxicity. Until such tailored vials and formulations become available, compounding practices for drug formulations in neonates should be evaluated to guarantee the correct dosing, product stability and safety.

  8. Efficacy of a low-cost bubble CPAP system in treatment of respiratory distress in a neonatal ward in Malawi.

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    Kondwani Kawaza

    Full Text Available Respiratory failure is a leading cause of neonatal mortality in the developing world. Bubble continuous positive airway pressure (bCPAP is a safe, effective intervention for infants with respiratory distress and is widely used in developed countries. Because of its high cost, bCPAP is not widely utilized in low-resource settings. We evaluated the performance of a new bCPAP system to treat severe respiratory distress in a low resource setting, comparing it to nasal oxygen therapy, the current standard of care.We conducted a non-randomized convenience sample study to test the efficacy of a low-cost bCPAP system treating newborns with severe respiratory distress in the neonatal ward of Queen Elizabeth Central Hospital, in Blantyre, Malawi. Neonates weighing >1,000 g and presenting with severe respiratory distress who fulfilled inclusion criteria received nasal bCPAP if a device was available; if not, they received standard care. Clinical assessments were made during treatment and outcomes compared for the two groups.87 neonates (62 bCPAP, 25 controls were recruited. Survival rate for neonates receiving bCPAP was 71.0% (44/62 compared with 44.0% (11/25 for controls. 65.5% (19/29 of very low birth weight neonates receiving bCPAP survived to discharge compared to 15.4% (1/13 of controls. 64.6% (31/48 of neonates with respiratory distress syndrome (RDS receiving bCPAP survived to discharge, compared to 23.5% (4/17 of controls. 61.5% (16/26 of neonates with sepsis receiving bCPAP survived to discharge, while none of the seven neonates with sepsis in the control group survived.Use of a low-cost bCPAP system to treat neonatal respiratory distress resulted in 27% absolute improvement in survival. The beneficial effect was greater for neonates with very low birth weight, RDS, or sepsis. Implementing appropriate bCPAP devices could reduce neonatal mortality in developing countries.

  9. Efficacy of a low-cost bubble CPAP system in treatment of respiratory distress in a neonatal ward in Malawi.

    Science.gov (United States)

    Kawaza, Kondwani; Machen, Heather E; Brown, Jocelyn; Mwanza, Zondiwe; Iniguez, Suzanne; Gest, Al; Smith, E O'Brian; Oden, Maria; Richards-Kortum, Rebecca R; Molyneux, Elizabeth

    2014-01-01

    Respiratory failure is a leading cause of neonatal mortality in the developing world. Bubble continuous positive airway pressure (bCPAP) is a safe, effective intervention for infants with respiratory distress and is widely used in developed countries. Because of its high cost, bCPAP is not widely utilized in low-resource settings. We evaluated the performance of a new bCPAP system to treat severe respiratory distress in a low resource setting, comparing it to nasal oxygen therapy, the current standard of care. We conducted a non-randomized convenience sample study to test the efficacy of a low-cost bCPAP system treating newborns with severe respiratory distress in the neonatal ward of Queen Elizabeth Central Hospital, in Blantyre, Malawi. Neonates weighing >1,000 g and presenting with severe respiratory distress who fulfilled inclusion criteria received nasal bCPAP if a device was available; if not, they received standard care. Clinical assessments were made during treatment and outcomes compared for the two groups. 87 neonates (62 bCPAP, 25 controls) were recruited. Survival rate for neonates receiving bCPAP was 71.0% (44/62) compared with 44.0% (11/25) for controls. 65.5% (19/29) of very low birth weight neonates receiving bCPAP survived to discharge compared to 15.4% (1/13) of controls. 64.6% (31/48) of neonates with respiratory distress syndrome (RDS) receiving bCPAP survived to discharge, compared to 23.5% (4/17) of controls. 61.5% (16/26) of neonates with sepsis receiving bCPAP survived to discharge, while none of the seven neonates with sepsis in the control group survived. Use of a low-cost bCPAP system to treat neonatal respiratory distress resulted in 27% absolute improvement in survival. The beneficial effect was greater for neonates with very low birth weight, RDS, or sepsis. Implementing appropriate bCPAP devices could reduce neonatal mortality in developing countries.

  10. Increased MMP-9 and TIMP-1 in mouse neonatal brain and plasma and in human neonatal plasma after hypoxia-ischemia: a potential marker of neonatal encephalopathy.

    Science.gov (United States)

    Bednarek, Nathalie; Svedin, Pernilla; Garnotel, Roselyne; Favrais, Géraldine; Loron, Gauthier; Schwendiman, Leslie; Hagberg, Henrik; Morville, Patrice; Mallard, Carina; Gressens, Pierre

    2012-01-01

    To implement neuroprotective strategies in newborns, sensitive and specific biomarkers are needed for identifying those who are at risk for brain damage. We evaluated the effectiveness of matrix metalloproteinases (MMPs) and their naturally occurring tissue inhibitors of metalloproteinases (TIMPs) in predicting neonatal encephalopathy (NE) damage in newborns. Plasma MMP-9 and TIMP-1 levels were upregulated as early as 1 h after the HI insult but not did not show such elevations after other types of injury (ibotenate-induced excitotoxicity, hypoxia, lipopolysaccharide-induced inflammation), and brain levels reflected this increase soon thereafter. We confirmed these results by carrying out plasma MMP-9 and TIMP-1 measurements in human newborns with NE. In these infants, protein levels of MMP-9 and TIMP-1 were found to be elevated during a short window up to 6 h after birth. This feature is particularly useful in identifying newborns in need of neuroprotection. A second peak observed 72 h after birth is possibly related to the second phase of energy failure after a HI insult. Our data, although preliminary, support the use of MMP-9 and TIMP-1 as early biomarkers for the presence and extent of perinatal brain injury in human term newborns. We first used a mouse model of neonatal HI injury to explore mechanistic aspects such as the time course of these markers after the hypoxia-ischemia event, and the correlation between the levels of these candidate markers in brain and plasma.

  11. Clinical case of using discrete plasmapheresis in infant with early neonatal sepsis and hemolytic disease

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    S. V. Aborin

    2017-01-01

    Full Text Available Currently in the literature there are insufficient data on the use of efferent hemocorrection methods in neonatal practice. The basic principle of this method is the removal of plasma containing endotoxins and exotoxins and other pathological substances, replacing it with donor plasma, colloid and crystalloid solutions. The therapeutic effect of plasmapheresis includes detoxification, anti-inflammatory and immunomodulatory effects by removing toxins and removal of circulating immune complexes, inflammatory mediators, and activated structures of the complement system. Discrete plasmapheresis in neonatal practice may be used in any body mass of the patient. This article describes a clinical case of successful application of discrete plasmapheresis in full-term newborn baby is in critical condition. The severity was due to severe early neonatal sepsis, development of multiple organ failure and accompanying RH-conflict. After two sessions of plasmapheresis was observed a positive clinical effect in reducing intoxication, systemic inflammatory response syndrome. Reducing the level of bilirubin is allowed to avoid the operation replacement blood transfusion.

  12. Neonatal Nursing

    OpenAIRE

    Crawford, Doreen; Morris, Maryke

    1994-01-01

    "Neonatal Nursing" offers a systematic approach to the nursing care of the sick newborn baby. Nursing actions and responsibilities are the focus of the text with relevant research findings, clinical applications, anatomy, physiology and pathology provided where necessary. This comprehensive text covers all areas of neonatal nursing including ethics, continuing care in the community, intranatal care, statistics and pharmokinetics so that holistic care of the infant is described. This book shou...

  13. Neonatal Outcomes of Rh-Negative Pregnancies in a Tertiary Level Neonatal Intensive Care Unit: A Prospective Study

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    Chacham

    2016-07-01

    Full Text Available Background Rhesus incompatibility is a preventable cause for severe neonatal hyperbilirubinemia, hydrops fetalis and still births. The prevalence of the Rh-negative blood group among Indian woman varies from 2% - 10%. Despite declining the incidence of Rhesus incompatibility, due to availability of anti-D immunoglobulin, and improved antenatal care of the Rh-negative pregnant woman, it still accounts for a significant proportion of neonatal hyperbilirubinemia and neuro-morbidity. The prevalence of Rh-negative women having Rh-positive neonates is 60%. Objectives This study aimed to estimate the incidence of Rh iso-immunization and evaluate the outcomes of Rh iso-immunized neonates. Methods This prospective observational study was conducted in a tertiary level neonatal intensive care unit, Princess Esra hospital, Deccan college of medical sciences, Hyderabad, Telangana, India. Consecutive intramural and extramural neonates admitted to neonatal intensive care unit with the Rh-negative mother’s blood group and hyperbilirubinemia were enrolled. Neonates born to Rh+ve mothers were excluded. Neonatal gestational age, birth weight, age at admission, duration of phototherapy, duration of hospitalization, neonatal examination and investigations were recorded in a predesigned, pretested performa. Results A total of 90 neonates were born to Rh-negative mothers, of which 70% (63 had the Rh-positive blood group and 30% had the Rh-negative blood group. Of these 63 neonates, 48 (76.2% had hyperbilirubinemia and 43 neonates (68.3% had significant hyperbilirubinemia (total serum bilirubin > 15mg/dL. Among them, 2%, 75% and 23% were born to primi, multi and grandmutli, respectively. Also, 14.5% of the neonates were large for dates (LFD, 75% appropriate for dates (AFD and 10.5% were small for dates (SFD. Premature and SFD neonates had higher incidence of hyperbilirubinemia. Significantly higher incidence of jaundice occurred within 72 hours of life. The mean

  14. Analysis of HLA-A antigens and C282Y and H63D mutations of the HFE gene in Brazilian patients with hemochromatosis

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    Bittencourt P.L.

    2002-01-01

    Full Text Available The hemochromatosis gene, HFE, is located on chromosome 6 in close proximity to the HLA-A locus. Most Caucasian patients with hereditary hemochromatosis (HH are homozygous for HLA-A3 and for the C282Y mutation of the HFE gene, while a minority are compound heterozygotes for C282Y and H63D. The prevalence of these mutations in non-Caucasian patients with HH is lower than expected. The objective of the present study was to evaluate the frequencies of HLA-A antigens and the C282Y and H63D mutations of the HFE gene in Brazilian patients with HH and to compare clinical and laboratory profiles of C282Y-positive and -negative patients with HH. The frequencies of HLA-A and C282Y and H63D mutations were determined by PCR-based methods in 15 male patients (median age 44 (20-72 years with HH. Eight patients (53% were homozygous and one (7% was heterozygous for the C282Y mutation. None had compound heterozygosity for C282Y and H63D mutations. All but three C282Y homozygotes were positive for HLA-A3 and three other patients without C282Y were shown to be either heterozygous (N = 2 or homozygous (N = 1 for HLA-A3. Patients homozygous for the C282Y mutation had higher ferritin levels and lower age at onset, but the difference was not significant. The presence of C282Y homozygosity in roughly half of the Brazilian patients with HH, together with the findings of HLA-A homozygosity in C282Y-negative subjects, suggest that other mutations in the HFE gene or in other genes involved in iron homeostasis might also be linked to HH in Brazil.

  15. SERUM SODIUM CHANGES IN NEONATES RECEIVING PHOTOTHERAPY FOR NEONATAL HYPERBILIRUBINEMIA

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    Sunil Kumar

    2015-07-01

    Full Text Available BACKGROUND : Neonates receiving phototherapy have side effects like hypocalcemia and electrolyte changes. Our study is hereby intended to study the serum sodium changes due to phototherapy. AIMS : To evaluate the serum sodium changes in neonates receiving phototherapy f or neonatal hyperbilirubinemia. SETTINGS AND DESIGN : A prospective hospital based comparative study conducted on neonates admitted in the Neonatal Intensive Care Unit receiving phototherapy. METHODS AND MATERIAL : A predesigned proforma has aided the enroll ment of 252 newborns into the study. Serum bilirubin and serum sodium were determined before and after termination of phototherapy. The first samples were considered as controls. A comparative study was made between before and after phototherapy groups to determine the incidence of serum sodium imbalances. STATISTICAL ANALYSIS USED : Proportions will be compared using chi - square test. All data of various groups will be tabulated and statistically analyzed using suitable statistical tests (Student's t test. RESULTS : Male to Female ratio was 1.45 : 1. Incidence of low birth weight babies was 23% and preterm was 20.2%. Mean birth weight and gestational age was 2.84±0.51 kg and 38.44±1.98 wks respectively. Mean duration of phototherapy was 37.65±11.06 hrs. The incidence of hyponatremia post phototherapy found to be 6% which was more in low birth weight (LBW babies (17.2% , p48 hrs (p<0.001. Even the decline in mean serum sodium values after phototherapy found to be statistically significant. CONCLUSION : Our study shows that neonates u nder phototherapy are at higher risk of hyponatremia. This risk is greater in premature and LBW babies and hence this group of babies should be closely monitored for changes in serum sodium and should be managed accordingly.

  16. Neonatal Vaccination: Challenges and Intervention Strategies.

    Science.gov (United States)

    Morris, Matthew C; Surendran, Naveen

    2016-01-01

    While vaccines have been tremendously successful in reducing the incidence of serious infectious diseases, newborns remain particularly vulnerable in the first few months of their life to life-threatening infections. A number of challenges exist to neonatal vaccination. However, recent advances in the understanding of neonatal immunology offer insights to overcome many of those challenges. This review will present an overview of the features of neonatal immunity which make vaccination difficult, survey the mechanisms of action of available vaccine adjuvants with respect to the unique features of neonatal immunity, and propose a possible mechanism contributing to the inability of neonates to generate protective immune responses to vaccines. We surveyed recent published findings on the challenges to neonatal vaccination and possible intervention strategies including the use of novel vaccine adjuvants to develop efficacious neonatal vaccines. Challenges in the vaccination of neonates include interference from maternal antibody and excessive skewing towards Th2 immunity, which can be counteracted by the use of proper adjuvants. Synergistic stimulation of multiple Toll-like receptors by incorporating well-defined agonist-adjuvant combinations to vaccines is a promising strategy to ensure a protective vaccine response in neonates. © 2016 S. Karger AG, Basel.

  17. Critically ill neonates displayed stable vital parameters and reduced metabolic acidosis during neonatal emergency airborne transport in Sweden.

    Science.gov (United States)

    Frid, Ingrid; Ågren, Johan; Kjellberg, Mattias; Normann, Erik; Sindelar, Richard

    2018-02-26

    This study evaluated the medical quality of acute airborne transports carried out by a neonatal emergency transport service in a Swedish healthcare region from 2012 to 2015. The transport charts and patient records of all infants transported to the regional centre were reviewed for transport indications and vital parameters and outcomes. We identified 187 acute airborne transports and the main indications for referral were therapeutic hypothermia after perinatal asphyxia, extremely preterm birth and respiratory failure. There were 37 deaths, but none of these occurred during transport and none of the deaths that occurred within 24 hours after transport were found to be related to the transport per se. No differences were found in vital parameters or ventilator settings before and after transport, except for an improvement in blood pH (7.22 ± 0.13 versus 7.27 ± 0.13, mean ± SD, p < 0.01), due to a decrease in base deficit (-8.0 ± 6.8 versus -5.4 ± 6.3 mmol, p < 0.001), while the partial pressure of carbon dioxide remained unchanged. During air transport, critically ill neonates displayed stable vital parameters and reduced metabolic acidosis. No transport-related mortality was found, but the high number of extremely preterm infants transported indicates the potential for improving in-utero transport. ©2018 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  18. Characteristics of gene mutation in Chinese patients with hereditary hemochromatosis

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    LYU Tingxia

    2016-08-01

    Full Text Available ObjectiveTo investigate the characteristics of gene mutation in Chinese patients with hereditary hemochromatosis (HH. MethodsA total of 9 patients with HH who visited Beijing Friendship Hospital, Capital Medical University from January 2013 to December 2015 were enrolled. The genomic DNA was extracted, and PCR amplification and Sanger sequencing were performed for all the exons of four genotypes of HH, i.e., HFE (type Ⅰ, HJV (type ⅡA, HAMP (type ⅡB, TFR2 (type Ⅲ, and SLC40A1 (type Ⅳ to analyze gene mutations. A total of 50 healthy subjects were enrolled as control group to analyze the prevalence of identified gene mutations in a healthy population. ResultsOf all patients, 2 had H63D mutation of HFE gene in type Ⅰ HH, 1 had E3D mutation of HJV gene in type ⅡA HH, 2 had I238M mutation of TFR2 gene in type Ⅲ HH, and 1 had IVS 3+10 del GTT splice mutation of SLC40A1 gene in type Ⅳ HH. No patients had C282Y mutation of HFE gene in type Ⅰ HH which was commonly seen in European and American populations. Five patients had no missense mutation or splice mutation. In addition, it was found in a family that a HH patient had E3D mutation of HJV gene, H63D mutation of HFE gene, and I238M mutation of TFR2 gene, but the healthy brother and sister carrying two of these mutations did not had the phenotype of HH. ConclusionHH gene mutations vary significantly across patients of different races, and non-HFE-HH is dominant in the Chinese population. There may be HH genes which are different from known genes, and further investigation is needed.

  19. Evaluation of serial urine viral cultures for the diagnosis of cytomegalovirus infection in neonates and infants.

    Science.gov (United States)

    Chisholm, Karen M; Aziz, Natali; McDowell, Michal; Guo, Frances P; Srinivas, Nivedita; Benitz, William E; Norton, Mary E; Gutierrez, Kathleen; Folkins, Ann K; Pinsky, Benjamin A

    2014-01-01

    Cytomegalovirus (CMV) is the most common cause of congenital infection worldwide. Urine viral culture is the standard for CMV diagnosis in neonates and infants. The objectives of this study were to compare the performance of serial paired rapid shell vial cultures (SVC) and routine viral cultures (RVC), and to determine the optimal number of cultures needed to detect positive cases. From 2001 to 2011, all paired CMV SVC and RVC performed on neonates and infants less than 100 days of age were recorded. Testing episodes were defined as sets of cultures performed within 7 days of one another. A total of 1264 neonates and infants underwent 1478 testing episodes; 68 (5.4%) had at least one episode with a positive CMV culture. In episodes where CMV was detected before day 21 of life, the first specimen was positive in 100% (16/16) of cases. When testing occurred after 21 days of life, the first specimen was positive in 82.7% (43/52) of cases, requiring three cultures to reach 100% detection. The SVC was more prone to assay failure than RVC. Overall, when RVC was compared to SVC, there was 86.0% positive agreement and 99.9% negative agreement. In conclusion, three serial urine samples are necessary for detection of CMV in specimens collected between day of life 22 and 99, while one sample may be sufficient on or before day of life 21. Though SVC was more sensitive than RVC, the risk of SVC failure supports the use of multimodality testing to optimize detection.

  20. Identification of priorities for medication safety in the neonatal intensive care unit via failure mode and effect analysis

    Directory of Open Access Journals (Sweden)

    Ali Vafaee Najar

    2016-06-01

    Conclusion: FMEA is an effective proactive risk-assessment tool, used to help multidisciplinary teams to understand the healthcare process and identify the possible errors. In addition, it helps prioritize remedial interventions for patients and enhance the safety of drug prescription in neonates.

  1. Therapies for neonates with congenital malformations admitted to a neonatal unit

    Directory of Open Access Journals (Sweden)

    Maria Vera Lúcia Moreira Leitão Cardoso

    2015-03-01

    Full Text Available The aim of this study was to characterize the treatments applied to newborns with congenital malformation hospitalized in a neonatal unit and to identify whether there is an association among the treatments used and the type of malformation. A descriptive, prospective and quantitative study was developed in a public institution in Fortaleza, Ceará, Brazil. Data were collected using the medical records of 30 neonates with congenital malformations. The incidence of malformations was higher among females, regardless of the mother’s age, gestational age or weight at birth; malformations of the central nervous and musculoskeletal systems prevailed. The treatments used varied according to the clinical evolution of the neonate. The data collected did not present statistical significance when associated with the variable of congenital malformation and the treatments used (p>0.05. The treatments are not directly related to the type of malformation, but to the clinical condition of the neonate.

  2. Intraoperative fluid therapy in neonates

    African Journals Online (AJOL)

    Differences from adults and children in physiology and anatomy of neonates inform our ... is based on energy expenditure indexed to bodyweight.2 Energy ... fragile and poorly keratinised.5 ... neonates means that very conservative fluid regimes in neonates ..... I make an estimation of insensible loss from the skin, viscera,.

  3. Pneumoperitoneum without Intestinal Perforation in a Neonate: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Prabhavathi Gummalla

    2017-01-01

    Full Text Available Pneumoperitoneum in a preterm neonate usually indicates perforation of the intestine and is considered a surgical emergency. However, there are cases of pneumoperitoneum with no evidence of rupture of the intestine reported in the literature. We report a case of pneumoperitoneum with no intestinal perforation in a preterm neonate with respiratory distress syndrome who was on high frequency oscillatory ventilation (HFOV. He developed bilateral pulmonary interstitial emphysema with localized cystic lesion, likely localized pulmonary interstitial emphysema, and recurrent pneumothoraces. He was treated with dexamethasone to wean from the ventilator. Pneumoperitoneum developed in association with left sided pneumothorax following mechanical ventilation and cardiopulmonary resuscitation. Pneumoperitoneum resolved after the pneumothorax was resolved with chest tube drainage. He died from acute cardiorespiratory failure. At autopsy, there was no evidence of intestinal perforation. This case highlights the fact that pneumoperitoneum can develop secondary to pneumothorax and does not always indicate intestinal perforation or require exploratory laparotomy.

  4. Neonatal Tele-Homecare

    DEFF Research Database (Denmark)

    Holm, Kristina Garne

    Neonatal homecare (NH) implies that parents manage tube feeding and care of their preterm infant at home supported by home visits from neonatal nurses, to monitor infant growth and the well-being of the family. Home visits are costly and time consuming in rural areas. The overall aim of this study...

  5. [Neonatal tumours and congenital malformations].

    Science.gov (United States)

    Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

    2008-06-01

    The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data

  6. Dengue neonatal en el Perú: Reporte de un caso Neonatal dengue in Peru: a case report

    Directory of Open Access Journals (Sweden)

    Hermann Silva Delgado

    2011-03-01

    Full Text Available Se presenta el caso de un neonato de sexo femenino, a término, producto de madre fallecida al séptimo día de su puerperio, por falla multiorgánica debido a dengue grave confirmado por detección de antígeno NS1 e IgM. La recién nacida (RN no tuvo complicaciones, pero a partir del cuarto día de vida desarrolló fiebre, ictericia, manifestaciones de extravasación de plasma, hepatomegalia, ascitis, plaquetopenia y otros signos de síndrome de respuesta inflamatoria sistémica. Su evolución final fue favorable con tratamiento instaurado. Se demuestra con PCR en tiempo real, la presencia del virus dengue serotipo 2 en sangre de la RN, confirmándose el primer caso de dengue neonatal reportado en el Perú.We present the case of a full-term female newborn, whose mother died seven days postpartum from multi-organ failure due to severe dengue confirmed by NS1 antigen detection and positive IgM. The newborn did not have any complication, but at the fourth day of life she developed fever, jaundice, signs of plasma leakage, thrombocytopenia, hepatomegaly, ascitis, and others signs of systemic inflammation response syndrome. She fully recovered with supportive treatment. The RT-PCR test of a peripheral blood sample revealed a positive result for the dengue virus serotype 2, confirming the first case of neonatal dengue reported in Peru.

  7. Neonatal mortality in Utah.

    Science.gov (United States)

    Woolley, F R; Schuman, K L; Lyon, J L

    1982-09-01

    A cohort study of neonatal mortality (N = 106) in white singleton births (N = 14,486) in Utah for January-June 1975 was conducted. Using membership and activity in the Church of Jesus Christ of Latter-day Saints (LDS or Mormon) as a proxy for parental health practices, i.e., tobacco and alcohol abstinence, differential neonatal mortality rates were calculated. The influence of potential confounding factors was evaluated. Low activity LDS members were found to have an excess risk of neonatal death five times greater than high activity LDS, with an upper bound of a two-sided 95% confidence interval of 7.9. The data consistently indicate a lower neonatal mortality rate for active LDS members. Non-LDS were found to have a lower rate than either medium or low activity LDS.

  8. Predictors of positive blood culture and deaths among neonates with suspected neonatal sepsis in a tertiary hospital, Mwanza- Tanzania

    Directory of Open Access Journals (Sweden)

    Jeremiah Seni

    2010-06-01

    Full Text Available Abstract Background Neonatal sepsis is a significant cause of morbidity and mortality in neonates. Appropriate clinical diagnosis and empirical treatment in a given setting is crucial as pathogens of bacterial sepsis and antibiotic sensitivity pattern can considerably vary in different settings. This study was conducted at Bugando Medical Centre (BMC, Tanzania to determine the prevalence of neonatal sepsis, predictors of positive blood culture, deaths and antimicrobial susceptibility, thus providing essential information to formulate a policy for management of neonatal sepsis. Methods This was a prospective cross sectional study involving 300 neonates admitted at BMC neonatal unit between March and November 2009. Standard data collection form was used to collect all demographic data and clinical characteristics of neonates. Blood culture was done on Brain Heart Infusion broth followed by identification of isolates using conventional methods and testing for their susceptibility to antimicrobial agents using the disc diffusion method. Results Among 770 neonates admitted during the study period; 300 (38.9% neonates were diagnosed to have neonatal sepsis by WHO criteria. Of 300 neonates with clinical neonatal sepsis 121(40% and 179(60% had early and late onset sepsis respectively. Positive blood culture was found in 57 (47.1% and 92 (51.4% among neonates with early and late onset neonatal sepsis respectively (p = 0.466. Predictors of positive blood culture in both early and late onset neonatal sepsis were inability to feed, lethargy, cyanosis, meconium stained liquor, premature rupture of the membrane and convulsion. About 49% of gram negatives isolates were resistant to third generation cephalosporins and 28% of Staphylococcus aureus were found to be Methicillin resistant Staphylococcus aureus (MRSA. Deaths occurred in 57 (19% of neonates. Factors that predicted deaths were positive blood culture (p = 0.0001, gram negative sepsis (p = 0.0001 and

  9. NEONATAL TOBACCO SYNDROME

    Directory of Open Access Journals (Sweden)

    R.A.Kireev

    2008-12-01

    Full Text Available The objective of the research is to study neonatal adaptation in new-born children from the tobacco abused mothers. A comparative analysis of clinical and neuroendochnal status and lipid metabolism in new-born children from smoking and non-smoking mothers was carried out Neonatal adaptation disorders were revealed in new-born children from the smoking mothers.

  10. Pelayanan Kesehatan Ibu dan Kematian Neonatal

    Directory of Open Access Journals (Sweden)

    Desy Fitri Yani

    2013-03-01

    Full Text Available Indonesia bersama seluruh negara berkembang berupaya mencapai kesepakatan Millenium Development Goals (MDGs dengan salah satu sasaran menurunkan angka kematian neonatal dari 20 per 1.000 kelahiran hidup menjadi 15 per 1.000 kelahiran hidup. Penelitian ini bertujuan mengetahui hubungan pelayanan kesehatan ibu dengan kematian neonatal di Kabupaten Lampung Timur tahun 2011. Penelitian dengan desain studi kasus kontrol ini mengamati kasus ibu yang mengalami kematian neonatal dan kontrol ibu yang tidak mengalami kematian neonatal. Analisis multivariat menemukan pelayanan antenatal dan pertolongan persalinan berhubungan secara signifikan dengan kematian neonatal, setelah mengendalikan variabel umur ibu dan riwayat kehamilan (OR = 16,32; nilai p = 0,000; dan (OR = 18,36; nilai p = 0,31. Bayi yang dilahirkan dari Ibu dengan pelayanan antenatal tidak lengkap berisiko mengalami kematian neonatal 16,32 dan 18,36 kali lebih besar daripada bayi yang dilahirkan. Ibu dengan pelayanan antenatal lengkap dan penolong persalinan profesional. Tidak ada hubungan penolong persalinan dengan kematian neonatal, setelah mengontrol variabel pelayanan antenatal, umur ibu, riwayat kehamilan, riwayat penyakit, dan riwayat persalinan. Disarankan meningkatkan kualitas pelayanan antenatal dengan memerhatikan faktor umur ibu dan riwayat persalinan, mengembangkan kegiatan audit maternal perinatal serta meningkatkan keterampilan petugas penolong persalinan. All developing countries including Indonesia seek to reach agreement the Millennium Development Goals (MDG’s. It is objectives include reducing neonatal mortality by 25 percent from 20 per 1,000 live birth to 15 per 1,000 live births. This study aimed to determine the relationship of maternal health services with neonatal mortality in East Lampung District in 2011. This study used case control design to compare between the groups of mother whom have neonatal deaths (cases and neonatal life (control in East Lampung District in

  11. Interpretation of neonatal chest radiography

    International Nuclear Information System (INIS)

    Yoon, Hye Kyung

    2016-01-01

    Plain radiographs for infants in the neonatal intensive care unit are obtained using the portable X-ray equipment in order to evaluate the neonatal lungs and also to check the position of the tubes and catheters used for monitoring critically-ill neonates. Neonatal respiratory distress is caused by a variety of medical or surgical disease conditions. Clinical information about the gestational week, respiratory symptoms, and any events during delivery is essential for interpretation of the neonatal chest radiographs. Awareness of common chest abnormality in the prematurely born or term babies is also very important for chest evaluation in the newborn. Furthermore, knowledge about complications such as air leaks and bronchopulmonary dysplasia following treatment are required to accurately inform the clinicians. The purpose of this article was to briefly review radiographic findings of chest diseases in newborns that are relatively common in daily practice

  12. Interpretation of neonatal chest radiography

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hye Kyung [Dept. of Radiology, Kangwon National University Hospital, Chuncheon (Korea, Republic of)

    2016-05-15

    Plain radiographs for infants in the neonatal intensive care unit are obtained using the portable X-ray equipment in order to evaluate the neonatal lungs and also to check the position of the tubes and catheters used for monitoring critically-ill neonates. Neonatal respiratory distress is caused by a variety of medical or surgical disease conditions. Clinical information about the gestational week, respiratory symptoms, and any events during delivery is essential for interpretation of the neonatal chest radiographs. Awareness of common chest abnormality in the prematurely born or term babies is also very important for chest evaluation in the newborn. Furthermore, knowledge about complications such as air leaks and bronchopulmonary dysplasia following treatment are required to accurately inform the clinicians. The purpose of this article was to briefly review radiographic findings of chest diseases in newborns that are relatively common in daily practice.

  13. Radiation doses and risks to neonates undergoing common radiographic examinations in the neonatal intensive care unit

    International Nuclear Information System (INIS)

    McParland, B.J.; Lee, R.

    1996-01-01

    Neonates in the-Neonatal Intensive Care Unit (NICU) can receive large numbers of radiographs owing to the clinical conditions they may present. More neonatal radiation dosimetry data are required for three fundamental reasons: (1.) to aid in the establishment of reference dose levels for interinstitutional comparisons; (2.) to improve childhood cancer risk estimates following neonatal exposure; and (3.) to indicate appropriate directions for dose reduction. This paper describes an investigation of two different NICU radiological techniques with significantly different neonate doses. While patient-matched images taken with both techniques were assessed in a blind review, this component of the study is beyond the scope of this paper and is not discussed here. (author)

  14. Alteration in adenylate cyclase response to aminergic stimulation following neonatal x-irradiation

    International Nuclear Information System (INIS)

    Chronister, R.B.; Palmer, G.C.; Gerbrandt, L.

    1980-01-01

    X-irradiation of the rat neonatal hippocampus produces severe alterations in the architectonic features of the mature hippocampus. The most prominent alteration is a marked depletion of the granule cells of the dentate gyrus, with a subsequent realignment of CA 4 cells. The present data also show that norepinephrine (NE), dopamine and histamine stimulation of adenylate cyclase activity is severely attenuated in the hippocampi of irradiated animals. This failure suggests that the NE fibers of irradiated subjects, although normal in content of NE, are not functional in some of their NE-effector actions

  15. Comparing the Efficacy of Face Mask CPAP with Nasopharyngeal CPAP for Neonatal Transport after Delivery

    OpenAIRE

    Manizheh Mostafa-Gharehbaghi; Ali Peirovifar; Bahram Karimi

    2013-01-01

    Background: Preterm infants have respiratory failure and complications because surfactant in alveolus is low. CPAP (Continuous positive airway pressure) is a method for respiratory support in pre-term neonates and is provided by different equipment and methods. This study aims to compare two different routes of CPAP delivery in preterm newborn infants and to determine the need for surfactant replacement therapy in two groups. Materials and Methods: This is a randomized controlled clinical tri...

  16. RISK FACTORS IN NEONATAL ANAEROBIC INFECTIONS

    Directory of Open Access Journals (Sweden)

    M. S. Tabib

    2008-06-01

    Full Text Available Anaerobic bacteria are well known causes of sepsis in adults but there are few studies regarding their role in neonatal sepsis. In an attempt to define the incidence of neonatal anaerobic infections a prospective study was performed during one year period. A total number of 400 neonates under sepsis study were entered this investigation. Anaerobic as well as aerobic cultures were sent. The patients were subjected to comparison in two groups: anaerobic culture positive and anaerobic culture negative and this comparison were analyzed statistically. There were 7 neonates with positive anaerobic culture and 35 neonates with positive aerobic culture. A significant statistical relationship was found between anaerobic infections and abdominal distention and pneumonia. It is recommended for those neonates with abdominal distention and pneumonia refractory to antibiotic treatment to be started on antibiotics with anaerobic coverage.

  17. Determinants of neonatal mortality in Indonesia.

    Science.gov (United States)

    Titaley, Christiana R; Dibley, Michael J; Agho, Kingsley; Roberts, Christine L; Hall, John

    2008-07-09

    Neonatal mortality accounts for almost 40 per cent of under-five child mortality, globally. An understanding of the factors related to neonatal mortality is important to guide the development of focused and evidence-based health interventions to prevent neonatal deaths. This study aimed to identify the determinants of neonatal mortality in Indonesia, for a nationally representative sample of births from 1997 to 2002. The data source for the analysis was the 2002-2003 Indonesia Demographic and Health Survey from which survival information of 15,952 singleton live-born infants born between 1997 and 2002 was examined. Multilevel logistic regression using a hierarchical approach was performed to analyze the factors associated with neonatal deaths, using community, socio-economic status and proximate determinants. At the community level, the odds of neonatal death was significantly higher for infants from East Java (OR = 5.01, p = 0.00), and for North, Central and Southeast Sulawesi and Gorontalo combined (OR = 3.17, p = 0.03) compared to the lowest neonatal mortality regions of Bali, South Sulawesi and Jambi provinces. A progressive reduction in the odds was found as the percentage of deliveries assisted by trained delivery attendants in the cluster increased. The odds of neonatal death were higher for infants born to both mother and father who were employed (OR = 1.84, p = 0.00) and for infants born to father who were unemployed (OR = 2.99, p = 0.02). The odds were also higher for higher rank infants with a short birth interval (OR = 2.82, p = 0.00), male infants (OR = 1.49, p = 0.01), smaller than average-sized infants (OR = 2.80, p = 0.00), and infant's whose mother had a history of delivery complications (OR = 1.81, p = 0.00). Infants receiving any postnatal care were significantly protected from neonatal death (OR = 0.63, p = 0.03). Public health interventions directed at reducing neonatal death should address community, household and individual level factors

  18. Experience of nasal continuous positive airway pressure (cpap) by infant flow driver in a neonatal unit of a developing country

    International Nuclear Information System (INIS)

    Iqbal, A.; Waqar, T.; Safdar, C.A.; Iqbal, T.

    2014-01-01

    Objective: To study the safety and efficacy of nasal continuous positive airways pressure by infant flow drivers in neonates admitted with respiratory problems. Study Design: Quasi-experimental study. Place and Duration of Study: This study was conducted at CMH Lahore from April 2012 to March 2013. Subjects and Methods: All infants who were treated with nasal continuous positive airway pressure (nCPAP) for various indications at neonatal intensive care unit (NICU) of CMH Lahore were evaluated for gestational, age, weight, gender, indications and duration on nCPAP, pre-defined outcomes, complications and length of hospital stay. Efficacy was defined as the ability to manage an infant on nCPAP alone thus avoiding the need for mechanical ventilation. Results: During the study period, 343 neonates were admitted in NICU, forty five neonates were placed on nCPAP. Mean gestational age was 33.85+ 3 weeks. Mean weight was 2043 + 770 grams. Main indications for applying nCPAP were respiratory distress syndrome (48.9%) and neonatal pneumonia (17.8%). Most common complication was abdominal distension (6.7%). Out of 45 infants placed on nCPAP, 32 (71.1%) were managed on nCPAP alone while 13 (28.9%) needed mechanical ventilation after nCPAP failure. Conclusion: Nasal CPAP by an infant flow driver is a useful method to manage respiratory distress in neonates. It reduces the need for mechanical ventilation and can be used as first line respiratory support before mechanical ventilation. (author)

  19. NEONATAL CONJUNCTIVITIS AND ITS DRUG SENSITIVITY PATTERN

    Directory of Open Access Journals (Sweden)

    Kavitha Thulukkanam

    2017-05-01

    Full Text Available BACKGROUND Neonatal conjunctivitis is eye discharge in neonates and it is a common infection in neonates in the first month of life. Worldwide, the incidence of neonatal conjunctivitis varies from 1% to 33% depending on the socioeconomic status of the people in the region. In India, the incidence varies from region to region from 0.9 to 35%. MATERIALS AND METHODS Single center prospective study. All neonates of age 0-30 days delivered and referred from neonatal ward of Chengalpattu Medical College and Hospital with complaints of eye swelling, redness, conjunctival discharge to the eye department for treatment are included in the study. RESULTS In this study, age group from 0-7 days were most commonly affected by neonatal conjunctivitis. Both eyes were commonly affected together. Neonates born by both LSCS and normal vaginal delivery were almost equally affected. The most common organism found in culture and Gram staining was staphylococci. CONCLUSION Neonatal conjunctivitis is more likely to be acquired postnatally. It acquires during the 1 st week and responds well to local application of antibiotics, which covers common causative bacteria.

  20. Neonatal tetanus mortality in coastal Kenya

    DEFF Research Database (Denmark)

    Bjerregaard, P; Steinglass, R; Mutie, D M

    1993-01-01

    In a house-to-house survey in Kilifi District, Kenya, mothers of 2556 liveborn children were interviewed about neonatal mortality, especially from neonatal tetanus (NNT). The crude birth rate was 60.5 per 1000 population, the neonatal mortality rate 21.1 and the NNT mortality rate 3.1 per 1000 li...... indicates that over the past decade the surveyed area has greatly reduced neonatal and NNT mortality. Possible strategies for accelerated NNT control have been identified by the survey....

  1. Specialist teams for neonatal transport to neonatal intensive care units for prevention of morbidity and mortality.

    Science.gov (United States)

    Chang, Alvin S M; Berry, Andrew; Jones, Lisa J; Sivasangari, Subramaniam

    2015-10-28

    Maternal antenatal transfers provide better neonatal outcomes. However, there will inevitably be some infants who require acute transport to a neonatal intensive care unit (NICU). Because of this, many institutions develop services to provide neonatal transport by specially trained health personnel. However, few studies report on relevant clinical outcomes in infants requiring transport to NICU. To determine the effects of specialist transport teams compared with non-specialist transport teams on the risk of neonatal mortality and morbidity among high-risk newborn infants requiring transport to neonatal intensive care. We used the standard search strategy of the Cochrane Neonatal Review Group to search the Cochrane Central Register of Controlled Trials (CENTRAL 2015, Issue 7), MEDLINE (1966 to 31 July 2015), EMBASE (1980 to 31 July 2015), CINAHL (1982 to 31 July 2015), conference proceedings, and the reference lists of retrieved articles for randomised controlled trials and quasi-randomised trials. randomised, quasi-randomised or cluster randomised controlled trials. neonates requiring transport to a neonatal intensive care unit. transport by a specialist team compared to a non-specialist team. any of the following outcomes - death; adverse events during transport leading to respiratory compromise; and condition on admission to the neonatal intensive care unit. The methodological quality of the trials was assessed using the information provided in the studies and by personal communication with the author. Data on relevant outcomes were extracted and the effect size estimated and reported as risk ratio (RR), risk difference (RD), number needed to treat for an additional beneficial outcome (NNTB) or number needed to treat for an additional harmful outcome (NNTH) and mean difference (MD) for continuous outcomes. Data from cluster randomised trials were not combined for analysis. One trial met the inclusion criteria of this review but was considered ineligible owing to

  2. Correction of Neonatal Hypovolemia

    Directory of Open Access Journals (Sweden)

    V. V. Moskalev

    2007-01-01

    Full Text Available Objective: to evaluate the efficiency of hydroxyethyl starch solution (6% refortane, Berlin-Chemie versus fresh frozen plasma used to correct neonatal hypovolemia.Materials and methods. In 12 neonatal infants with hypoco-agulation, hypovolemia was corrected with fresh frozen plasma (10 ml/kg body weight. In 13 neonates, it was corrected with 6% refortane infusion in a dose of 10 ml/kg. Doppler echocardiography was used to study central hemodynamic parameters and Doppler study was employed to examine regional blood flow in the anterior cerebral and renal arteries.Results. Infusion of 6% refortane and fresh frozen plasma at a rate of 10 ml/hour during an hour was found to normalize the parameters of central hemodynamics and regional blood flow.Conclusion. Comparative analysis of the findings suggests that 6% refortane is the drug of choice in correcting neonatal hypovolemia. Fresh frozen plasma should be infused in hemostatic disorders. 

  3. Haloperidol aggravates transverse aortic constriction-induced heart failure via mitochondrial dysfunction

    OpenAIRE

    Yasuharu Shinoda; Hideaki Tagashira; Md. Shenuarin Bhuiyan; Hideyuki Hasegawa; Hiroshi Kanai; Kohji Fukunaga

    2016-01-01

    Haloperidol is an antipsychotic drug that inhibits the dopamine D2 receptor among others. Haloperidol also binds the sigma-1 receptor (σ1R) and inhibits it irreversibly. A serious outcome of haloperidol treatment of schizophrenia patients is death due to sudden cardiac failure. Although the cause remains unclear, we hypothesized that these effects were mediated by chronic haloperidol inhibition of cardiac σ1R. To test this, we treated neonatal rat cardiomyocytes with haloperidol, exposed them...

  4. Phototherapy and exchange transfusion for neonatal ...

    African Journals Online (AJOL)

    The purpose of this document is to address the current lack of consensus regarding the management of hyperbilirubinaemia in neonates in South Africa. If left untreated, severe neonatal hyperbilirubinaemia may cause kernicterus and ultimately death and the severity of neonatal jaundice is often underestimated clinically.

  5. Intrahepatic cholangiocarcinoma: impact of genetic hemochromatosis on outcome and overall survival after surgical resection.

    Science.gov (United States)

    Sulpice, Laurent; Rayar, Michel; Boucher, Eveline; Pele, Fabienne; Pracht, Marc; Meunier, Bernard; Boudjema, Karim

    2013-03-01

    The influence of genetic hemochromatosis (GH) on outcomes following surgical resections for intrahepatic cholangiocarcinoma (ICC) has not been evaluated. All patients with ICC who underwent a surgical resection between January 1997 and August 2011 were analyzed retrospectively. Risk factors were assessed by univariate and multivariate analyses. Eighty-seven patients were analyzed; 16 of these patients (18.4%) had GH. Among the 71 non-GH patients, 52 (73.2%) and 19 (26.8%) had normal or cirrhotic parenchyma, respectively. There was no significant difference in survival between the GH and non-GH patients. A univariate analysis showed that major hepatectomy (P = 0.012), intraoperative blood transfusion (P = 0.007), tumor size >5 cm (P = 0.006), several nodules (P < 0.001), and microvascular invasion (P = 0.04) were significantly associated with poor survival. A multivariate analysis showed that intraoperative blood infusion (HR 0.37; CI 95% [0.19; 0.71]) and more than one nodule (HR 2.5; CI 95% [1.06; 5.8]) were associated with a lower survival rate. Although the incidence of GH was high in our series, the presence of GH did not affect the outcomes after a liver hepatectomy for ICC. GH does not appear to increase recurrences or worsen the overall and disease-free survival. Copyright © 2013 Elsevier Inc. All rights reserved.

  6. Role of massage therapy on reduction of neonatal hyperbilirubinemia in term and preterm neonates: a review of clinical trials.

    Science.gov (United States)

    Garg, Bhawan Deep; Kabra, Nandkishor S; Balasubramanian, Haribalakrishna

    2017-09-13

    Neonatal hyperbilirubinemia (NNH) is one of the leading causes of admissions in nursery throughout the world. It affects approximately 2.4-15% of neonates during the first 2 weeks of life. To evaluate the role of massage therapy for reduction of NNH in both term and preterm neonates. The literature search was done for various randomized control trials (RCTs) by searching the Cochrane Library, PubMed, and EMBASE. This review included total of 10 RCTs (two in preterm neonates and eight in term neonates) that fulfilled inclusion criteria. In most of the trials, Field massage was given. Six out of eight trials reported reduction in bilirubin levels in term neonates. However, only one trial (out of two) reported significant reduction in bilirubin levels in preterm neonates. Both trials in preterm neonates and most of the trials in term neonates (five trials) reported increased stool frequencies. Role of massage therapy in the management of NNH is supported by the current evidence. However, due to limitations of the trials, current evidences are not sufficient to use massage therapy for the management of NNH in routine practice.

  7. Incidence and Outcome of CPAP Failure in Preterm Infants.

    Science.gov (United States)

    Dargaville, Peter A; Gerber, Angela; Johansson, Stefan; De Paoli, Antonio G; Kamlin, C Omar F; Orsini, Francesca; Davis, Peter G

    2016-07-01

    Data from clinical trials support the use of continuous positive airway pressure (CPAP) for initial respiratory management in preterm infants, but there is concern regarding the potential failure of CPAP support. We aimed to examine the incidence and explore the outcomes of CPAP failure in Australian and New Zealand Neonatal Network data from 2007 to 2013. Data from inborn preterm infants managed on CPAP from the outset were analyzed in 2 gestational age ranges (25-28 and 29-32 completed weeks). Outcomes after CPAP failure (need for intubation CPAP using adjusted odds ratios (AORs). Within the cohort of 19 103 infants, 11 684 were initially managed on CPAP. Failure of CPAP occurred in 863 (43%) of 1989 infants commencing on CPAP at 25-28 weeks' gestation and 2061 (21%) of 9695 at 29-32 weeks. CPAP failure was associated with a substantially higher rate of pneumothorax, and a heightened risk of death, bronchopulmonary dysplasia (BPD) and other morbidities compared with those managed successfully on CPAP. The incidence of death or BPD was also increased: (25-28 weeks: 39% vs 20%, AOR 2.30, 99% confidence interval 1.71-3.10; 29-32 weeks: 12% vs 3.1%, AOR 3.62 [2.76-4.74]). The CPAP failure group had longer durations of respiratory support and hospitalization. CPAP failure in preterm infants is associated with increased risk of mortality and major morbidities, including BPD. Strategies to promote successful CPAP application should be pursued vigorously. Copyright © 2016 by the American Academy of Pediatrics.

  8. Opinions regarding neonatal resuscitation training for the obstetric physician: a survey of neonatal and obstetric training program directors.

    Science.gov (United States)

    Bruno, C J; Johnston, L; Lee, C; Bernstein, P S; Goffman, D

    2018-04-01

    Our goal was to garner opinions regarding neonatal resuscitation training for obstetric physicians. We sought to evaluate obstacles to neonatal resuscitation training for obstetric physicians and possible solutions for implementation challenges. We distributed a national survey via email to all neonatal-perinatal medicine fellowship directors and obstetrics & gynecology residency program directors in the United States. This survey was designed by a consensus method. Ninety-eight (53%) obstetric and fifty-seven (51%) neonatal program directors responded to our surveys. Eighty-eight percent of neonatologists surveyed believe that obstetricians should be neonatal resuscitation program (NRP) certified. The majority of surveyed obstetricians (>89%) believe that obstetricians should have some neonatal resuscitation training. Eighty-six percent of obstetric residents have completed training in NRP, but only 19% of obstetric attendings are NRP certified. Major barriers to NRP training that were identified include time, lack of national requirement, lack of belief it is helpful, and cost. Most obstetric attendings are not NRP certified, but the majority of respondents believe that obstetric providers should have some neonatal resuscitation training. Our study demonstrates that most respondents support a modified neonatal resuscitation course for obstetric physicians.

  9. The effect of high risk pregnancy on duration of neonatal stay in neonatal intensive care unit.

    Science.gov (United States)

    Afrasiabi, Narges; Mohagheghi, Parisa; Kalani, Majid; Mohades, Gholam; Farahani, Zahra

    2014-08-01

    High risk pregnancies increase the risk of neonatal mortality and morbidity. In order to identify the influence of pregnancy complications on the period of neonatal stay in Neonatal Intensive Care Units (NICUs), an analysis has been carried out in our center. In a cross-sectional-descriptive analytical study, the data including NICU length of stay was gathered from 526 medical records of neonates. We also assessed their maternal complications such as premature rapture of membranes (PROM), urinary tract infection (UTI), preeclampsia, oligohydramnios, and twin/triplet pregnancy. Finally we analyzed the relation between variables by SPSS statistics software version 19. The level of significance was considered PUTI (P=0.02), multiple gestation (P=0.03), and oligohydramnios (P=0.003). We found a positive correlation between numbers of gestation and length of NICU stay (P=0.03). A positive correlation existed between neonatal complication and length of NICU stay (P<0.001). By increasing maternal health level and prenatal care services, neonatal outcome can be improved and length of stay in NICUs decreased.

  10. The prevalence of neonatal jaundice and risk factors in healthy term neonates at National District Hospital in Bloemfontein

    Science.gov (United States)

    2018-01-01

    Background Neonatal jaundice affects one in two infants globally. The jaundice is the result of an accumulation of bilirubin as foetal haemoglobin is metabolised by the immature liver. High serum levels of bilirubin result in lethargy, poor feeding and kernicterus of the infant. Aim The main aim of this article was to determine the prevalence of neonatal jaundice and secondly to explore its risk factors in healthy term neonates. Setting Maternity ward, National District Hospital, Bloemfontein, South Africa. Methods In this cross-sectional study, mothers and infants were conveniently sampled after delivery and before discharge. The mothers were interviewed and their case records were reviewed for risk factors for neonatal jaundice and the clinical appearance and bilirubin levels of the infants were measured with a non-invasive transcutaneous bilirubin meter. Results A total of 96 mother-infant pairs were included in the study. The prevalence of neonatal jaundice was 55.2%; however, only 10% of black babies who were diagnosed with jaundice appeared clinically jaundiced. Normal vaginal delivery was the only risk factor associated with neonatal jaundice. Black race and maternal smoking were not protective against neonatal jaundice as in some other studies. Conclusion More than half (55.2%) of healthy term neonates developed neonatal jaundice. As it is difficult to clinically diagnose neonatal jaundice in darker pigmented babies, it is recommended that the bilirubin level of all babies should be checked with a non-invasive bilirubin meter before discharge from hospital or maternity unit as well as during the first clinic visit on day 3 after birth.

  11. Neonatal maternal deprivation response and developmental changes in gene expression revealed by hypothalamic gene expression profiling in mice.

    Directory of Open Access Journals (Sweden)

    Feng Ding

    Full Text Available Neonatal feeding problems are observed in several genetic diseases including Prader-Willi syndrome (PWS. Later in life, individuals with PWS develop hyperphagia and obesity due to lack of appetite control. We hypothesized that failure to thrive in infancy and later-onset hyperphagia are related and could be due to a defect in the hypothalamus. In this study, we performed gene expression microarray analysis of the hypothalamic response to maternal deprivation in neonatal wild-type and Snord116del mice, a mouse model for PWS in which a cluster of imprinted C/D box snoRNAs is deleted. The neonatal starvation response in both strains was dramatically different from that reported in adult rodents. Genes that are affected by adult starvation showed no expression change in the hypothalamus of 5 day-old pups after 6 hours of maternal deprivation. Unlike in adult rodents, expression levels of Nanos2 and Pdk4 were increased, and those of Pgpep1, Ndp, Brms1l, Mett10d, and Snx1 were decreased after neonatal deprivation. In addition, we compared hypothalamic gene expression profiles at postnatal days 5 and 13 and observed significant developmental changes. Notably, the gene expression profiles of Snord116del deletion mice and wild-type littermates were very similar at all time points and conditions, arguing against a role of Snord116 in feeding regulation in the neonatal period.

  12. Bartter syndrome: presentation in an extremely premature neonate.

    Science.gov (United States)

    Flores, F X; Ojeda, F J; Calhoun, D A

    2013-08-01

    Reports of Bartter syndrome in premature neonates are rare. We describe the presentation and clinical course of a neonate born at 25.6 weeks estimated gestational age with polyuria, hyponatremia, hypokalemia and hypercalciuria ,who was diagnosed with neonatal Bartter syndrome. The evaluation, diagnosis and management of neonatal Bartter syndrome in this premature neonate are discussed.

  13. Neonates with reduced neonatal lung function have systemic low-grade inflammation

    DEFF Research Database (Denmark)

    Chawes, Bo L.K.; Stokholm, Jakob; Bønnelykke, Klaus

    2015-01-01

    Background: Children and adults with asthma and impaired lung function have been reported to have low-grade systemic inflammation, but it is unknown whether this inflammation starts before symptoms and in particular whether low-grade inflammation is present in asymptomatic neonates with reduced...... lung function. ObjectiveWe sought to investigate the possible association between neonatal lung function and biomarkers of systemic inflammation.  Methods: Plasma levels of high-sensitivity C-reactive protein (hs-CRP), IL-1β, IL-6, TNF-α, and CXCL8 (IL-8) were measured at age 6 months in 300 children.......  Results: The neonatal forced expiratory volume at 0.5 seconds was inversely associated with hs-CRP (β-coefficient, −0.12; 95% CI, −0.21 to −0.04; P approach, including hs-CRP, IL-6...

  14. Analysis of In-hospital Neonatal Death in the Tertiary Neonatal Intensive Care Unit in China: A Multicenter Retrospective Study

    Institute of Scientific and Technical Information of China (English)

    Chen-Hong Wang; Li-Zhong Du; Xiao-Lu Ma; Li-Ping Shi; Xiao-Mei Tong; Hong Liu; Guo-Fang Ding

    2016-01-01

    Background:Globally,the proportion of child deaths that occur in the neonatal period remains a high level of 37-41%.Differences of cause in neonate death exist in different regions as well as in different economic development countries.The specific aim of this study was to investigate the causes,characteristics,and differences of death in neonates during hospitalization in the tertiary Neonatal Intensive Care Unit (NICU) of China.Methods:All the dead neonates admitted to 26 NICUs were included between January 1,2011,and December 31,2011.All the data were collected retrospectively from clinical records by a designed questionnaire.Data collected from each NICU were delivered to the leading institution where the results were analyzed.Results:A total of 744 newborns died during the l-year survey,accounting for 1.2% of all the neonates admitted to 26 NICUs and 37.6% of all the deaths in children under 5 years of age in these hospitals.Preterm neonate death accounted for 59.3% of all the death.The leading causes of death in preterm and term infants were pulmonary disease and infection,respectively.In early neonate period,pulmonary diseases (56.5%) occupied the largest proportion ofpreterm deaths while infection (27%) and neurologic diseases (22%) were the two main causes of term deaths.In late neonate period,infection was the leading cause of both preterm and term neonate deaths.About two-thirds of neonate death occurred after medical care withdrawal.Of the cases who might survive if receiving continuing treatment,parents' concern about the long-term outcomes was the main reason of medical care withdrawal.Conclusions:Neonate death still accounts for a high proportion of all the deaths in children under 5 years of age.Our study showed the majority of neonate death occurred in preterm infants.Cause of death varied with the age of death and gestational age.Accurate and prompt evaluation of the long-term outcomes should be carried out to guide the critical

  15. Determinants of neonatal mortality in Indonesia

    Directory of Open Access Journals (Sweden)

    Agho Kingsley

    2008-07-01

    Full Text Available Abstract Background Neonatal mortality accounts for almost 40 per cent of under-five child mortality, globally. An understanding of the factors related to neonatal mortality is important to guide the development of focused and evidence-based health interventions to prevent neonatal deaths. This study aimed to identify the determinants of neonatal mortality in Indonesia, for a nationally representative sample of births from 1997 to 2002. Methods The data source for the analysis was the 2002–2003 Indonesia Demographic and Health Survey from which survival information of 15,952 singleton live-born infants born between 1997 and 2002 was examined. Multilevel logistic regression using a hierarchical approach was performed to analyze the factors associated with neonatal deaths, using community, socio-economic status and proximate determinants. Results At the community level, the odds of neonatal death was significantly higher for infants from East Java (OR = 5.01, p = 0.00, and for North, Central and Southeast Sulawesi and Gorontalo combined (OR = 3.17, p = 0.03 compared to the lowest neonatal mortality regions of Bali, South Sulawesi and Jambi provinces. A progressive reduction in the odds was found as the percentage of deliveries assisted by trained delivery attendants in the cluster increased. The odds of neonatal death were higher for infants born to both mother and father who were employed (OR = 1.84, p = 0.00 and for infants born to father who were unemployed (OR = 2.99, p = 0.02. The odds were also higher for higher rank infants with a short birth interval (OR = 2.82, p = 0.00, male infants (OR = 1.49, p = 0.01, smaller than average-sized infants (OR = 2.80, p = 0.00, and infant's whose mother had a history of delivery complications (OR = 1.81, p = 0.00. Infants receiving any postnatal care were significantly protected from neonatal death (OR = 0.63, p = 0.03. Conclusion Public health interventions directed at reducing neonatal death should

  16. The challenges of neonatal magnetic resonance imaging

    International Nuclear Information System (INIS)

    Arthurs, Owen J.; Graves, Martin J.; Lomas, David J.; Edwards, Andrea; Austin, Topun

    2012-01-01

    Improved neonatal survival rates and antenatal diagnostic imaging is generating a growing demand for postnatal MRI examinations. Neonatal brain MRI is now becoming standard clinical care in many settings, but with the exception of some research centres, the technique has not been optimised for imaging neonates and small children. Here, we review some of the challenges involved in neonatal MRI, including recent advances in overall MR practicality and nursing practice, to address some of the ways in which the MR experience could be made more neonate-friendly. (orig.)

  17. Susceptibility weighted imaging of the neonatal brain

    International Nuclear Information System (INIS)

    Meoded, A.; Poretti, A.; Northington, F.J.; Tekes, A.; Intrapiromkul, J.; Huisman, T.A.G.M.

    2012-01-01

    Susceptibility weighted imaging (SWI) is a well-established magnetic resonance technique, which is highly sensitive for blood, iron, and calcium depositions in the brain and has been implemented in the routine clinical use in both children and neonates. SWI in neonates might provide valuable additional diagnostic and prognostic information for a wide spectrum of neonatal neurological disorders. To date, there are few articles available on the application of SWI in neonatal neurological disorders. The purpose of this article is to illustrate and describe the characteristic SWI findings in various typical neonatal neurological disorders.

  18. Susceptibility weighted imaging of the neonatal brain

    Energy Technology Data Exchange (ETDEWEB)

    Meoded, A.; Poretti, A. [Division of Pediatric Radiology and Division of Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, MD (United States); Northington, F.J. [Division of Neonatology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD (United States); Tekes, A.; Intrapiromkul, J. [Division of Pediatric Radiology and Division of Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, MD (United States); Huisman, T.A.G.M., E-mail: thuisma1@jhmi.edu [Division of Pediatric Radiology and Division of Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, MD (United States)

    2012-08-15

    Susceptibility weighted imaging (SWI) is a well-established magnetic resonance technique, which is highly sensitive for blood, iron, and calcium depositions in the brain and has been implemented in the routine clinical use in both children and neonates. SWI in neonates might provide valuable additional diagnostic and prognostic information for a wide spectrum of neonatal neurological disorders. To date, there are few articles available on the application of SWI in neonatal neurological disorders. The purpose of this article is to illustrate and describe the characteristic SWI findings in various typical neonatal neurological disorders.

  19. Birth risk indicators for maternal and neonatal health: Songkla Center Hospital perspective.

    Science.gov (United States)

    Kaewsuksai, Peeranan; Chandeying, Verapol

    2012-02-01

    The aim of the present study was to examine the maternal and neonatal birth risk indicator and their relationship with the outcome of pregnancy. This retrospective descriptive study was conducted in a selective month of 2008, 2009, and 2010. The birth risk indicators of maternal and neonatal health were collected from the medical records. There were 385, 349 and 334 deliveries in a selective month of 2008, 2009, and 2010. There was neither maternal mortality, nor cardiovascular failure in the present study period. Three main indication of inductions of labor were premature rupture of membrane (up to 4.0%), diabetes mellitus (up to 2.0%), and postdate (up to 1.3%). The first two conditions had statistical significance in September 2009 (p = 0.0334 and 0.0053 respectively). Whereas, the three major indications of cesarean section were previous cesarean section (12.5 to 21.9%), failure to progress due to protracted/arrest of labor pattern with/without rupture of membrane and augmented labor (2.4 to 7.5%), and fetal distress (1.1 to 4.2%). The rates of low birth weight, less than 2,500 grams, were varied from 5.2 to 6.9%. The respiratory distress syndrome (RDS) related to repeat cesarean section was encountered up to 3.6%, as well as the RDS related to induction of labor was up to 1.6%. The birth risk indicators reflect the outcome of pregnancy, however the development of additional key indicators for perinatal health care outcome are required.

  20. [Prevention of Neonatal Group B Sreptococcal Infection. Spanish Recommendations. Update 2012. SEIMC/SEGO/SEN/SEQ/SEMFYC Consensus Document].

    Science.gov (United States)

    Alós Cortés, Juan Ignacio; Andreu Domingo, Antonia; Arribas Mir, Lorenzo; Cabero Roura, Luis; de Cueto López, Marina; López Sastre, José; Melchor Marcos, Juan Carlos; Puertas Prieto, Alberto; de la Rosa Fraile, Manuel; Salcedo Abizanda, Salvador; Sánchez Luna, Manuel; Sanchez Pérez, María José; Torrejon Cardoso, Rafael

    2013-03-01

    Group B streptococci (GBS) remain the most common cause of early onset neonatal sepsis. In 2003 the Spanish Societies of Obstetrics and Gynaecology, Neonatology, Infectious Diseases and Clinical Microbiology, Chemotherapy, and Family and Community Medicine published updated recommendations for the prevention of early onset neonatal GBS infection. It was recommended to study all pregnant women at 35-37 weeks gestation to determine whether they were colonised by GBS, and to administer intrapartum antibiotic prophylaxis (IAP) to all colonised women. There has been a significant reduction in neonatal GBS infection in Spain following the widespread application of IAP. Today most cases of early onset GBS neonatal infection are due to false negative results in detecting GBS, to the lack of communication between laboratories and obstetric units, and to failures in implementing the prevention protocol. In 2010, new recommendations were published by the CDC, and this fact, together with the new knowledge and experience available, has led to the publishing of these new recommendations. The main changes in these revised recommendations include: microbiological methods to identify pregnant GBS carriers and for testing GBS antibiotic sensitivity, and the antibiotics used for IAP are updated; The significance of the presence of GBS in urine, including criteria for the diagnosis of UTI and asymptomatic bacteriuria in pregnancy are clarified; IAP in preterm labour and premature rupture of membranes, and the management of the newborn in relation to GBS carrier status of the mother are also revised. These recommendations are only addressed to the prevention of GBS early neonatal infection, are not effective against late neonatal infection. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  1. Ictericia Neonatal

    OpenAIRE

    Blanco de la Fuente, María Isabel

    2014-01-01

    El motivo que ha llevado a la realización de este trabajo fin de grado sobre el tema de la ICTERICIA NEONATAL se debe a la elevada frecuencia de su aparición en la población. Un porcentaje elevado de RN la padecen al nacer siendo, en la mayor parte de los casos, un proceso fisiológico resuelto con facilidad debido a una inmadurez del sistema hepático y a una hiperproducción de bilirrubina. La ictericia neonatal es la pigmentación de color amarillo de la piel y mucosas en ...

  2. Post-neonatal Tetanus in a PICU of a Developing Economy: Intensive Care Needs, Outcome and Predictors of Mortality.

    Science.gov (United States)

    Angurana, Suresh Kumar; Jayashree, Muralidharan; Bansal, Arun; Singhi, Sunit; Nallasamy, Karthi

    2018-02-01

    To evaluate pediatric intensive care unit (PICU) needs, outcome and predictors of mortality in post-neonatal tetanus. Review of 30 consecutive post-neonatal tetanus cases aged 1 months to 12 years admitted to a PICU in north India over a period of 10 years (January 2006 to December 2015). Chronic suppurative otitis media was the commonest portal of entry. All received tetanus toxoid, human tetanus immunoglobulin (HTIG) and appropriate antibiotics; 7 (23.3%) received intrathecal HTIG. Common complications were respiratory failure, rhabdomyolysis, autonomic dysfunction, acute kidney injury and healthcare-associated infections. PICU needs were as follows: ventilation; benzodiazepine, morphine and magnesium sulfate infusion; neuromuscular blockers, inotropes, tracheostomy and renal replacement therapy. Mortality rate was 40%; severity Grade IIIb, autonomic dysfunction, use of vasoactive drugs and those who did not receive intrathecal HTIG were significantly associated with mortality. Post-neonatal tetanus is associated with high mortality, and PICU needs include management of spasms, autonomic dysfunction and complications and cardiorespiratory support. © The Author [2017]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com

  3. Laser Photoradiation Therapy For Neonatal Jaundice

    Science.gov (United States)

    Hamza, Mostafa; Hamza, Mohammad

    1987-04-01

    This paper describes our leading experience in the clinical application of laser in the treatment of neonatal jaundice. Currently, the irradiation of jaundiced infants during neonatal life to fluorescent light is the most common treatment of neonatal hyperbilirubinemia. The authors have investigated the photodegradation of bilirubin by laser in vitro and in Gunn rats before embarking on its clinical application in the treatment of jaundice in the new born child. This work was done to study the theraputic effect of laser compared to the currently used phototherapy in the treatment of neonatal jaundice. We selected 16 full term neonates with jaundice to be the subject of this study. The neonates of the study were devided into two groups. The first group was treated with continuous phototherapy . The second group recieved photoradiation therapy with gas laser The laser used was a CW argon-ion laser tuned to oscillate at 488.0 nm wavelength. This wavelength selection was based on our previous studies on the effect of laser irradiation of Gunn rats at different wavelengths. Comparison of the results of both methods of treatment will be reported in detail. The advantages and limitations of laser photoradiation therapy for neonatal jaundice will be discussed.

  4. MRI of neonatal encephalopathy

    International Nuclear Information System (INIS)

    Khong, P.L.; Lam, B.C.C.; Tung, H.K.S.; Wong, V.; Chan, F.L.; Ooi, G.C.

    2003-01-01

    We present the magnetic resonance imaging (MRI) findings in neonatal encephalopathy, including hypoxic-ischaemic encephalopathy, perinatal/neonatal stroke, metabolic encephalopathy from inborn errors of metabolism, congenital central nervous system infections and birth trauma. The applications of advanced MRI techniques, such as diffusion-weighted imaging and magnetic resonance spectroscopy are emphasized

  5. Neonatal resuscitation: advances in training and practice

    Directory of Open Access Journals (Sweden)

    Sawyer T

    2016-12-01

    Full Text Available Taylor Sawyer, Rachel A Umoren, Megan M Gray Department of Pediatrics, Division of Neonatology, Neonatal Education and Simulation-based Training (NEST Program, University of Washington School of Medicine, Seattle, WA, USA Abstract: Each year in the US, some four hundred thousand newborns need help breathing when they are born. Due to the frequent need for resuscitation at birth, it is vital to have evidence-based care guidelines and to provide effective neonatal resuscitation training. Every five years, the International Liaison Committee on Resuscitation (ILCOR reviews the science of neonatal resuscitation. In the US, the American Heart Association (AHA develops treatment guidelines based on the ILCOR science review, and the Neonatal Resuscitation Program (NRP translates the AHA guidelines into an educational curriculum. In this report, we review recent advances in neonatal resuscitation training and practice. We begin with a review of the new 7th edition NRP training curriculum. Then, we examine key changes to the 2015 AHA neonatal resuscitation guidelines. The four components of the NRP curriculum reviewed here include eSim®, Performance Skills Stations, Integrated Skills Station, and Simulation and Debriefing. The key changes to the AHA neonatal resuscitation guidelines reviewed include initial steps of newborn care, positive-pressure ventilation, endotracheal intubation and use of laryngeal mask, chest compressions, medications, resuscitation of preterm newborns, and ethics and end-of-life care. We hope this report provides a succinct review of recent advances in neonatal resuscitation. Keywords: neonatal resuscitation, Neonatal Resuscitation Program, NRP, simulation, deliberate practice, debriefing, eSIM

  6. Acute symptomatic neonatal seizures in preterm neonates: etiologies and treatments.

    Science.gov (United States)

    Pisani, Francesco; Spagnoli, Carlotta

    2017-12-15

    Acute symptomatic neonatal seizures in preterm newborns are a relevant clinical challenge due to the presence of many knowledge gaps. Etiology-wise, acute symptomatic seizures have an age-specific epidemiology, with intraventricular hemorrhage and its complications representing the first cause in extremely and very preterm neonates, whereas other etiologies have similar occurrence rates as in full-term infants. Specific treatment strategies for the premature neonates are not yet available. Studies suggest a similarly low response rate with even more unfavorable prognosis than in full-term infants. Pharmacodynamic and pharmacokinetic changes are likely under way during the preterm period, with the potential to affect both effectiveness and safety of antiepileptic drugs in these patients. However, due to the lack of clear evidence to guide prioritization of second-line drugs, off-label medications are frequently indicated by review papers and flow-charts, and are prescribed in clinical practice. We therefore conclude by exploring potential future lines of research. Copyright © 2017 Elsevier Ltd. All rights reserved.

  7. Procedural pain in neonatal units in Kenya.

    Science.gov (United States)

    Kyololo, O'Brien Munyao; Stevens, Bonnie; Gastaldo, Denise; Gisore, Peter

    2014-11-01

    To determine the nature and frequency of painful procedures and procedural pain management practices in neonatal units in Kenya. Cross-sectional survey. Level I and level II neonatal units in Kenya. Ninety-five term and preterm neonates from seven neonatal units. Medical records of neonates admitted for at least 24 h were reviewed to determine the nature and frequency of painful procedures performed in the 24 h period preceding data collection (6:00 to 6:00) as well as the pain management interventions (eg, morphine, breastfeeding, skin-to-skin contact, containment, non-nutritive sucking) that accompanied each procedure. Neonates experienced a total of 404 painful procedures over a 24 h period (mean=4.3, SD 2.0; range 1-12); 270 tissue-damaging (mean=2.85, SD 1.1; range 1-6) and 134 non-tissue-damaging procedures (mean=1.41, SD 1.2; range 0-6). Peripheral cannula insertion (27%) and intramuscular injections (22%) were the most common painful procedures. Ventilated neonates and neonates admitted in level II neonatal units had a higher number of painful procedures than those admitted in level I units (mean 4.76 vs 2.96). Only one procedure had a pain intensity score documented; and none had been performed with any form of analgesia. Neonates in Kenya were exposed to numerous tissue-damaging and non-tissue-damaging procedures without any form of analgesia. Our findings suggest that education is needed on how to assess and manage procedural pain in neonatal units in Kenya. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  8. The value of neonatal autopsy.

    LENUS (Irish Health Repository)

    Hickey, Leah

    2012-01-01

    Neonatal autopsy rates were in decline internationally at the end of the last century. Our objective was to assess the current value of neonatal autopsy in providing additional information to families and healthcare professionals.

  9. Bupivacaine versus lidocaine analgesia for neonatal circumcision

    OpenAIRE

    Stolik-Dollberg, Orit C; Dollberg, Shaul

    2005-01-01

    Abstract Background Analgesia for neonatal circumcision was recently advocated for every male infant, and its use is considered essential by the American Academy of Pediatrics. We compared the post-operative analgesic quality of bupivacaine to that of lidocaine for achieving dorsal penile nerve block (DPNB) when performing neonatal circumcision. Methods Data were obtained from 38 neonates following neonatal circumcision. The infants had received DPNB analgesia with either lidocaine or bupivac...

  10. Parents' experiences with neonatal home care following initial care in the neonatal intensive care unit: a phenomenological hermeneutical interview study.

    Science.gov (United States)

    Dellenmark-Blom, Michaela; Wigert, Helena

    2014-03-01

    A descriptive study of parents' experiences with neonatal home care following initial care in the neonatal intensive care unit. As survival rates improve among premature and critically ill infants with an increased risk of morbidity, parents' responsibilities for neonatal care grow in scope and degree under the banner of family-centred care. Concurrent with medical advances, new questions arise about the role of parents and the experience of being provided neonatal care at home. An interview study with a phenomenological hermeneutic approach. Parents from a Swedish neonatal (n = 22) home care setting were extensively interviewed within one year of discharge. Data were collected during 2011-2012. The main theme of the findings is that parents experience neonatal home care as an inner emotional journey, from having a child to being a parent. This finding derives from three themes: the parents' experience of leaving the hospital milieu in favour of establishing independent parenthood, maturing as a parent and processing experiences during the period of neonatal intensive care. This study suggests that neonatal home care is experienced as a care structure adjusted to incorporate parents' needs following discharge from a neonatal intensive care unit. Neonatal home care appears to bridge the gap between hospital and home, supporting the family's adaptation to life in the home setting. Parents become empowered to be primary caregivers, having nurse consultants serving the needs of the whole family. Neonatal home care may therefore be understood as the implementation of family-centred care during the transition from NICU to home. © 2013 John Wiley & Sons Ltd.

  11. An agent based architecture for high-risk neonate management at neonatal intensive care unit.

    Science.gov (United States)

    Malak, Jaleh Shoshtarian; Safdari, Reza; Zeraati, Hojjat; Nayeri, Fatemeh Sadat; Mohammadzadeh, Niloofar; Farajollah, Seide Sedighe Seied

    2018-01-01

    In recent years, the use of new tools and technologies has decreased the neonatal mortality rate. Despite the positive effect of using these technologies, the decisions are complex and uncertain in critical conditions when the neonate is preterm or has a low birth weight or malformations. There is a need to automate the high-risk neonate management process by creating real-time and more precise decision support tools. To create a collaborative and real-time environment to manage neonates with critical conditions at the NICU (Neonatal Intensive Care Unit) and to overcome high-risk neonate management weaknesses by applying a multi agent based analysis and design methodology as a new solution for NICU management. This study was a basic research for medical informatics method development that was carried out in 2017. The requirement analysis was done by reviewing articles on NICU Decision Support Systems. PubMed, Science Direct, and IEEE databases were searched. Only English articles published after 1990 were included; also, a needs assessment was done by reviewing the extracted features and current processes at the NICU environment where the research was conducted. We analyzed the requirements and identified the main system roles (agents) and interactions by a comparative study of existing NICU decision support systems. The Universal Multi Agent Platform (UMAP) was applied to implement a prototype of our multi agent based high-risk neonate management architecture. Local environment agents interacted inside a container and each container interacted with external resources, including other NICU systems and consultation centers. In the NICU container, the main identified agents were reception, monitoring, NICU registry, and outcome prediction, which interacted with human agents including nurses and physicians. Managing patients at the NICU units requires online data collection, real-time collaboration, and management of many components. Multi agent systems are applied as

  12. Neonatal tetanus associated with skin infection.

    Science.gov (United States)

    Maharaj, M; Dungwa, N

    2016-08-03

    A 1-week-old infant was brought to a regional hospital with a history of recurrent seizures following lower abdominal septic skin infection. She was found to have neonatal tetanus, and a spatula test was positive. The tetanus infection was associated with a superficial skin infection, common in neonates. Treatment included sedatives (diazepam, chlorpromazine, phenobarbitone and morphine), muscle relaxants, antibiotics and ventilation in the neonatal intensive care unit. Intrathecal and intramuscular immunoglobulin were given, and the wound was treated. The infant recovered, with no seizures by the 16th day from admission, and was off the ventilator by the 18th day. This was shorter than the usual 3 - 4 weeks for neonates with tetanus at the hospital. The question arises whether tetanus immunisation should be considered in infants with skin infections, which frequently occur in the neonatal period.

  13. Perinatal pharmacology: applications for neonatal neurology.

    Science.gov (United States)

    Smits, Anne; Allegaert, Karel

    2011-11-01

    The principles of clinical pharmacology also apply to neonates, but their characteristics warrant a tailored approach. We focus on aspects of both developmental pharmacokinetics (concentration/time relationship) and developmental pharmacodynamics (concentration/effect relationship) in neonates. We hereby aimed to link concepts used in clinical pharmacology with compound-specific observations (anti-epileptics, analgosedatives) in the field of neonatal neurology. Although in part anecdotal, we subsequently illustrate the relevance of developmental pharmacology in the field of neonatal neurology by a specific intervention (e.g. whole body cooling), specific clinical presentations (e.g. short and long term outcome following fetal exposure to antidepressive agents, the development of new biomarkers for fetal alcohol syndrome) and specific clinical needs (e.g. analgosedation in neonates, excitocytosis versus neuro-apoptosis/impaired synaptogenesis). Copyright © 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  14. Dense pulmonary opacification in neonates treated with extracorporeal membrane oxygenation

    Energy Technology Data Exchange (ETDEWEB)

    Schlesinger, A.E.; Cornish, J.D.; Null, D.M.

    1986-09-01

    Chest radiographic findings in three neonates with respiratory failure secondary to meconium aspiration treated with extracorporeal membrane oxygenation (ECMO) are described. The degree of pulmonary opacification on the chest radiographs failed to correlate with the patients' clinical status as measured by the arterial oxygen levels but correlated well with the peak airway pressure (PAP) and continuous positive airway pressure (CPAP) settings on the mechanical ventilator. Because a variable portion of the arterial blood oxygenation is performed by the extracorporeal membrane oxygenator and unusually large fluctuations in airway pressure settings can occur in these patients while on ECMO, it is important to realize that the chest radiography may not be an accurate predictor of the patients' clinical status.

  15. Dense pulmonary opacification in neonates treated with extracorporeal membrane oxygenation

    International Nuclear Information System (INIS)

    Schlesinger, A.E.; Cornish, J.D.; Null, D.M.

    1986-01-01

    Chest radiographic findings in three neonates with respiratory failure secondary to meconium aspiration treated with extracorporeal membrane oxygenation (ECMO) are described. The degree of pulmonary opacification on the chest radiographs failed to correlate with the patients' clinical status as measured by the arterial oxygen levels but correlated well with the peak airway pressure (PAP) and continuous positive airway pressure (CPAP) settings on the mechanical ventilator. Because a variable portion of the arterial blood oxygenation is performed by the extracorporeal membrane oxygenator and unusually large fluctuations in airway pressure settings can occur in these patients while on ECMO, it is important to realize that the chest radiography may not be an accurate predictor of the patients' clinical status. (orig.)

  16. Geographical Accessibility to Obstetric and Neonatal Care and its Effect on Early Neonatal Mortality in Colombia, 2012-2014

    Directory of Open Access Journals (Sweden)

    Diego Fernando Rojas Gualdrón

    2017-04-01

    Full Text Available Introduction: The distribution of health resources influences early neonatal mortality, granting access to obstetric care which is a major public health problem. However, the geographical dimension of this influence has not been studied in Colombia. Objective: To describe the geographical accessibility to obstetric and neonatal care beds and its association with early neonatal mortality in Colombia and its municipalities. Method:An ecological study at municipal level was carried out. Ordinary least squares (OLS regression and a geographically weighted regression (GWR were used to explore statistical and spatial associations. Results: The municipalities in Colombia with Higher mortality tend to have lower geographical accessibility to obstetric and neonatal beds after controlling the fertility and economic characteristics of these municipalities. This association is significant only in municipalities of the west coast. The strength of this association decreases in inner municipalities. Discussion: The centralization of obstetric and neonatal beds in major municipalities around the central region leaves municipalities with high risk of mortality underserved. The decentralization of obstetric and neonatal healthcare resources is a mandatory issue in order to reduce geographical disparities in mortality and to improve neonatal survival, and a healthy beginning of life.

  17. Clinical practice: neonatal resuscitation. A Dutch consensus

    NARCIS (Netherlands)

    van den Dungen, F.A.M.; van Veenendaal, M.B.; Mulder, A.L.M.

    2010-01-01

    The updated Dutch guidelines on Neonatal Resuscitation assimilate the latest evidence in neonatal resuscitation. Important changes with regard to the 2004 guidelines and controversial issues concerning neonatal resuscitation are reviewed, and recommendations for daily practice are provided and

  18. Study of Relationship between Hypernatremia in Neonates and Way of Maternal Breast Feeding

    Directory of Open Access Journals (Sweden)

    H Boskabadi

    2014-04-01

    Full Text Available Introduction: The extreme reduction in breast milk intake during the first days of life leads to weight loss, kidney failure and hypernatremia. The aim of this study was to examine the relationship between hypernatremia in neonates and way of maternal breastfeeding in hospitalized infants in Ghaem Hospital of Mashhad.   Materials and Methods: After obtaining parental consent, 687 infants referred to the neonatal ward and clinic of Ghaem hospital of Mashhad participated in this cross-sectional study. First, complete history of the mother and baby regarding pregnancy and delivery problems, way of breastfeeding, and the first time of lactation beginning were obtained. Then neonates were divided into two groups of normal and hypernatremia (sodium≥150 mg/dl according to the amount of blood sodium level. Data was analyzed using correlation tests, chi-square, t test and Mann-Whitney tests with SPSS software (version 11.5.   Results: According to the findings of this study, the average age (P=0.911, Apgar scores (P=0.192, time of the first lactation (P=0.081 and breast feeding duration (P=o.108 showed no statistically significant difference between normal and hypernatremia groups. But the admission weight (P=0.011, times of lactation (P=0.108, breast-feeding status (P=0.001, let down reflex in mother’s breast (0.001, kind of nutrition (P=o.oo1, breast filling after childbirth and lactation (P=0.000, and breast softening after breast-feeding (P=0.000, urination frequency (P=0.000, defecation frequency (P=0.000 and duration of maternal hospitalization (P=0.007 showed statistically significant difference between the groups.   Conclusion: Neonatal weight control, times of lactation, lactation status, breast changes during breast feeding and frequency of urination and defecation may be effective in the early detection of the reduced breast milk intake and the control of the related complications.   Key Words: Breast Feedinf, Breast Milk

  19. Neonatal and infantile acne vulgaris: an update.

    Science.gov (United States)

    Serna-Tamayo, Cristian; Janniger, Camila K; Micali, Giuseppe; Schwartz, Robert A

    2014-07-01

    Acne may present in neonates, infants, and small children. Neonatal and infantile acne vulgaris are not considered to be rare. The presentation of acne in this patient population sometimes represents virilization and may portend later development of severe adolescent acne. Neonatal and infantile acne vulgaris must be distinguished from other cutaneous disorders seen in newborns and infants. Infantile acne tends to be more pleomorphic and inflammatory, thus requiring more vigorous therapy than neonatal acne.

  20. Providing safe surgery for neonates in sub-Saharan Africa.

    Science.gov (United States)

    Ameh, Emmanuel A; Ameh, Nkeiruka

    2003-07-01

    Advances in neonatal intensive care, total parenteral nutrition and improvements in technology have led to a greatly improved outcome of neonatal surgery in developed countries. In many parts of sub-Saharan Africa, however, neonatal surgery continues to pose wide-ranging challenges. Delivery outside hospital, delayed referral, poor transportation, and lack of appropriate personnel and facilities continue to contribute to increased morbidity and mortality in neonates, particularly under emergency situations. Antenatal supervision and hospital delivery needs to be encouraged in our communities. Adequate attention needs to be paid to providing appropriate facilities for neonatal transport and support and training of appropriate staff for neonatal surgery. Neonates with surgical problems should be adequately resuscitated before referral where necessary but surgery should not be unduly delayed. Major neonatal surgery should as much as possible be performed by those trained to operate on neonates. Appropriate research and international collaboration is necessary to improve neonatal surgical care in the environment.

  1. PLACENTAL WEIGHT AND ITS ASSOCIATION WITH MATERNAL AND NEONATAL CHARACTERISTICS

    Directory of Open Access Journals (Sweden)

    M Asgharnia

    2008-12-01

    Full Text Available "nPlacenta plays a vital role in normal fetal development and failure of placenta to gain weight and insufficiency of its function can result in fetal disorders. We performed this study to determine placental weight and factors associated with low weight placentas. In a longitudinal cross-sectional study, women with single pregnancy, and gestational age between 37-42 weeks were studied. The subjects were categorized in high (> 750 g, normal (330-750 g, and low placental weights (< 330 g. The placental weight, birth weight, maternal age, gestational age, parity, pre-eclampsia, history of maternal diabetes, delivery approaches, infants' gender; and Apgar score in 5th minutes after delivery were examined. One thousand-eighty eight pregnant women were included in the study. The mean and standard deviation for maternal ages and gestational ages at deliveries were 25.35 ± 5.6 and 247.51 ± 9.56 days, respectively. The mean and standard deviation of neonates' weights at birth and placental weights were 3214.28 ± 529 and 529.72 ± 113 g, respectively. The prevalences of low and high placental weights were 2% and 2.8%, respectively. There were statistically significant relationships between placental weight and birth weight, fetal distress, Apgar score, maternal diabetes, pre-eclampsia and approaches of deliveries (α = 0.05. Our findings indicate that placental weight can be associated with important variables influencing some maternal and neonatal outcomes and placental weight lower than 330 g can be a warning sign. Careful attention to placenta growth during pregnancy, for example by ultrasonography, can guide physicians to assess neonatal health.

  2. EMQN best practice guidelines for the molecular genetic diagnosis of hereditary hemochromatosis (HH)

    Science.gov (United States)

    Porto, Graça; Brissot, Pierre; Swinkels, Dorine W; Zoller, Heinz; Kamarainen, Outi; Patton, Simon; Alonso, Isabel; Morris, Michael; Keeney, Steve

    2016-01-01

    Molecular genetic testing for hereditary hemochromatosis (HH) is recognized as a reference test to confirm the diagnosis of suspected HH or to predict its risk. The vast majority (typically >90%) of patients with clinically characterized HH are homozygous for the p.C282Y variant in the HFE gene, referred to as HFE-related HH. Since 1996, HFE genotyping was implemented in diagnostic algorithms for suspected HH, allowing its early diagnosis and prevention. However, the penetrance of disease in p.C282Y homozygotes is incomplete. Hence, homozygosity for p.C282Y is not sufficient to diagnose HH. Neither is p.C282Y homozygosity required for diagnosis as other rare forms of HH exist, generally referred to as non-HFE-related HH. These pose significant challenges when defining criteria for referral, testing protocols, interpretation of test results and reporting practices. We present best practice guidelines for the molecular genetic diagnosis of HH where recommendations are classified, as far as possible, according to the level and strength of evidence. For clarification, the guidelines' recommendations are preceded by a detailed description of the methodology and results obtained with a series of actions taken in order to achieve a wide expert consensus, namely: (i) a survey on the current practices followed by laboratories offering molecular diagnosis of HH; (ii) a systematic literature search focused on some identified controversial topics; (iii) an expert Best Practice Workshop convened to achieve consensus on the practical recommendations included in the guidelines. PMID:26153218

  3. The cost-effectiveness of screening for hereditary hemochromatosis in Germany: a remodeling study.

    Science.gov (United States)

    Rogowski, Wolf H

    2009-01-01

    Genetic tests for hereditary hemochromatosis (HH) are currently included in the German ambulatory care reimbursement scheme but only for symptomatic individuals and the offspring of HH patients. This study synthesizes the most current evidence to examine whether screening in the broader population is cost-effective and to identify the best choice of initial and follow-up screening tests. A probabilistic decision-analytic model was constructed to calculate cost per life year gained (LYG) for HH screening among male Caucasians aged 30. Three strategies were considered in both the general population and male offspring of HH patients: phenotypic (transferrin saturation, TS), genotypic (C282Y mutation), and sequential (genotype if TS is elevated) screening. The incremental cost-effectiveness of sequential screening among male offspring, sequential population-wide screening, and genotypic screening is 41000, 124000, and 161000 Eero/LYG, respectively. All other strategies were subject to simple or extended dominance. The results are subject to high uncertainty. The most influential parameters in the deterministic one-way sensitivity analysis are discounting of life years gained and the adherence of patients to preventive phlebotomy. The current German policy of only screening at-risk individuals is consistent with health economic decision making based on typically accepted thresholds. However, conducting the DNA test after the first elevated TS result is more cost-effective than waiting for a second TS result as recommended by the German guidelines. Further empirical work regarding adherence to long-term prevention recommendations and explicit and well-justified guidance for the choice of discount rates in German economic evaluation are needed.

  4. Neonatal pressure ulcers: prevention and treatment

    Directory of Open Access Journals (Sweden)

    García-Molina P

    2017-09-01

    Full Text Available Pablo García-Molina,1,2 Alba Alfaro-López,1 Sara María García-Rodríguez,1 Celia Brotons-Payá,1 Mari Carmen Rodríguez-Dolz,1,2 Evelin Balaguer-López1,2 1Department of Nursing, University of Valencia, 2Research Group of Pediatric Nutrition, INCLIVA Foundation, Valencia, Spain Abstract: Health professionals should be prepared to respond to the needs of hospitalized neonates. The health team must consider multiple situations, where the neonate is at risk of having an adverse effect. One of the main interventions that health professionals must practice when interacting with hospitalized newborns is skin care. Neonates often suffer from diaper rash or intravenous drugs extravasation. Recently, hospitalized neonates and especially those in an unstable clinical situation are also at a risk of developing pressure ulcers. The presence of a pressure ulcer in a neonate can lead to serious problems to survival (eg, sepsis, clinical instability. This is the reason why, with this literature review, we attempt to answer questions from health professionals caring for neonates about the prevention and treatment of pressure ulcers. Keywords: infant, pressure ulcer, treatment, prevention, wound, assessment

  5. Imaging approach to persistent neonatal jaundice

    International Nuclear Information System (INIS)

    Kirks, D.; Coleman, R.E.; Filston, H.C.; Rosenberg, E.R.; Merten, D.F.

    1984-01-01

    Fifteen patients with persistent neonatal jaundice were evaluated by sonography and radionuclide scintigraphy. The sonographic features of both neonatal hepatitis and biliary atresia are nonspecific. Hepatobiliary scintigraphy after phenobarbital pretreatment in patients with neonatal hepatitis demonstrates normal hepatic extraction and delayed tracer excretion into the gastrointestinal tract. If there is neonatal hepatitis with severe hepatocellular damage, the hepatic extraction of tracer activity is decreased and excretion may be delayed or absent. Patients under 3 months of age with biliary atresia have normal hepatic extraction of tracer with no excretion into the gastrointestinal tract. Sonography in patients with a choledochal cyst shows a cystic mass in the porta hepatis with associated bile-duct dilatation. Hepatobiliary scintigraphy confirms that the choledochal cyst communicates with the biliary system. Initial sonography demonstrates hepatobiliary anatomy; subsequent phenobarbital-enhanced radionuclide scintigraphy determines hepatobiliary function. An expedient diagnostic approach is recommended for the evaluation of persistent neonatal jaundice

  6. A PEARL Study Analysis of National Neonatal, Early Neonatal, Late Neonatal, and Corrected Neonatal Mortality Rates in the State of Qatar during 2011: A Comparison with World Health Statistics 2011 and Qatar's Historic Data over a Period of 36 Years (1975-2011).

    Science.gov (United States)

    Rahman, Sajjad; Al Rifai, Hilal; El Ansari, Walid; Nimeri, Nuha; El Tinay, Sarrah; Salameh, Khalil; Abbas, Tariq; Jarir, Rawia A; Said, Nawal; Taha, Samer

    2012-10-01

    To prospectively ascertain Qatar's national Neonatal Mortality Rate (NMR), Early Neonatal Mortality Rate (ENMR), and Late Neonatal Mortality Rate (LNMR) during 2011, compare it with recent data from high-income countries, and analyze trends in Qatar's NMR's between 1975 and 2011 using historic data. A National prospective cohort-study. National data on live births and neonatal mortality was collected from all public and private maternity facilities in Qatar (1(st) January-December 31(st) 2011) and compared with historical neonatal mortality data (1975-2010) ascertained from the database of maternity and neonatal units of Women's Hospital and annual reports of Hamad Medical Corporation. For inter country comparison, country data of 2009 was extracted from World Health Statistics 2011 (WHO) and the European Perinatal Health report (2008). A total of 20583 live births were recorded during the study period. Qatar's national NMR during 2011 was 4.95, ENMR 2.7, LNMR 2.2, and cNMR 3.33. Between 1975 and 2011, Qatar's population increased by 10-fold, number of deliveries by 7.2 folds while relative risk of NMR decreased by 87% (RR 0.13, 95% CI 0.10-0.18, P<0.001), ENMR by 91% (RR 0.09, 95% CI 0.06-0.12, P<0.001) and LNMR by 58% (RR 0.42, 95% CI 0.23-0.74, P=0.002). The comparable ranges of neonatal mortality rates from selected high-income West European countries are: NMR: 2-5.7, ENMR 1.5-3.8, and LNMR 0.5-1.9. The neonatal survival in the State of Qatar has significantly improved between 1975 and 2011. The improvement has been more marked in ENMR than LNMR. Qatar's current neonatal mortality rates are comparable to most high-income West European countries. An in-depth research to assess the correlates and determinants of neonatal mortality in Qatar is indicated.

  7. Neonatal orbital abscess

    Directory of Open Access Journals (Sweden)

    Khalil M Al-Salem

    2014-01-01

    Full Text Available Orbital complications due to ethmoiditis are rare in neonates. A case of orbital abscess due to acute ethmoiditis in a 28-day-old girl is presented. A Successful outcome was achieved following antimicrobial therapy alone; spontaneous drainage of the abscess occurred from the lower lid without the need for surgery. From this case report, we intend to emphasize on eyelid retraction as a sign of neonatal orbital abscess, and to review all the available literature of similar cases.

  8. Abdominal surgery in neonatal foals.

    Science.gov (United States)

    Bryant, James E; Gaughan, Earl M

    2005-08-01

    Abdominal surgery in foals under 30 days old has become more common with improved neonatal care. Early recognition of a foal at risk and better nursing care have increased the survival rates of foals that require neonatal care. The success of improved neonatal care also has increased the need for accurate diagnosis and treatment of gastrointestinal, umbilical, and bladder disorders in these foals. This chapter focuses on the early and accurate diagnosis of specific disorders that require abdominal exploratory surgery and the specific treatment considerations and prognosis for these disorders.

  9. Feasibility Study of NMR Based Serum Metabolomic Profiling to Animal Health Monitoring: A Case Study on Iron Storage Disease in Captive Sumatran Rhinoceros (Dicerorhinus sumatrensis).

    Science.gov (United States)

    Watanabe, Miki; Roth, Terri L; Bauer, Stuart J; Lane, Adam; Romick-Rosendale, Lindsey E

    2016-01-01

    A variety of wildlife species maintained in captivity are susceptible to iron storage disease (ISD), or hemochromatosis, a disease resulting from the deposition of excess iron into insoluble iron clusters in soft tissue. Sumatran rhinoceros (Dicerorhinus sumatrensis) is one of the rhinoceros species that has evolutionarily adapted to a low-iron diet and is susceptible to iron overload. Hemosiderosis is reported at necropsy in many African black and Sumatran rhinoceroses but only a small number of animals reportedly die from hemochromatosis. The underlying cause and reasons for differences in susceptibility to hemochromatosis within the taxon remains unclear. Although serum ferritin concentrations have been useful in monitoring the progression of ISD in many species, there is some question regarding their value in diagnosing hemochromatosis in the Sumatran rhino. To investigate the metabolic changes during the development of hemochromatosis and possibly increase our understanding of its progression and individual susceptibility differences, the serum metabolome from a Sumatran rhinoceros was investigated by nuclear magnetic resonance (NMR)-based metabolomics. The study involved samples from female rhinoceros at the Cincinnati Zoo (n = 3), including two animals that died from liver failure caused by ISD, and the Sungai Dusun Rhinoceros Conservation Centre in Peninsular Malaysia (n = 4). Principal component analysis was performed to visually and statistically compare the metabolic profiles of the healthy animals. The results indicated that significant differences were present between the animals at the zoo and the animals in the conservation center. A comparison of the 43 serum metabolomes of three zoo rhinoceros showed two distinct groupings, healthy (n = 30) and unhealthy (n = 13). A total of eighteen altered metabolites were identified in healthy versus unhealthy samples. Results strongly suggest that NMR-based metabolomics is a valuable tool for animal health

  10. Feasibility Study of NMR Based Serum Metabolomic Profiling to Animal Health Monitoring: A Case Study on Iron Storage Disease in Captive Sumatran Rhinoceros (Dicerorhinus sumatrensis.

    Directory of Open Access Journals (Sweden)

    Miki Watanabe

    Full Text Available A variety of wildlife species maintained in captivity are susceptible to iron storage disease (ISD, or hemochromatosis, a disease resulting from the deposition of excess iron into insoluble iron clusters in soft tissue. Sumatran rhinoceros (Dicerorhinus sumatrensis is one of the rhinoceros species that has evolutionarily adapted to a low-iron diet and is susceptible to iron overload. Hemosiderosis is reported at necropsy in many African black and Sumatran rhinoceroses but only a small number of animals reportedly die from hemochromatosis. The underlying cause and reasons for differences in susceptibility to hemochromatosis within the taxon remains unclear. Although serum ferritin concentrations have been useful in monitoring the progression of ISD in many species, there is some question regarding their value in diagnosing hemochromatosis in the Sumatran rhino. To investigate the metabolic changes during the development of hemochromatosis and possibly increase our understanding of its progression and individual susceptibility differences, the serum metabolome from a Sumatran rhinoceros was investigated by nuclear magnetic resonance (NMR-based metabolomics. The study involved samples from female rhinoceros at the Cincinnati Zoo (n = 3, including two animals that died from liver failure caused by ISD, and the Sungai Dusun Rhinoceros Conservation Centre in Peninsular Malaysia (n = 4. Principal component analysis was performed to visually and statistically compare the metabolic profiles of the healthy animals. The results indicated that significant differences were present between the animals at the zoo and the animals in the conservation center. A comparison of the 43 serum metabolomes of three zoo rhinoceros showed two distinct groupings, healthy (n = 30 and unhealthy (n = 13. A total of eighteen altered metabolites were identified in healthy versus unhealthy samples. Results strongly suggest that NMR-based metabolomics is a valuable tool for

  11. Clinical value of periventricular low-intensity areas detected by fluid attenuated inversion recovery (FLAIR). Relationships between perinatal vital parameter and neonatal MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kadowaki, Sachiko; Iwata, Osuke; Tamura, Masanori [Nagano Children' s Hospital, Toyoshina (Japan)] (and others)

    2002-01-01

    A follow-up study was performed to assess the correlation among the incidence of periventricular low intensities (PVLI) on MRI (magnetic resonance imaging) FLAIR (fluid attenuated inversion recovery) imaging, clinical evidence of perinatal insults that may cause white matter damage, and the outcome of the infants. We evaluated periventricular white matter lesions of 329 neonates whose MRI were obtained before two months corrected age. The detective rate of periventricular abnormalities on FLAIR imaging was significantly higher than that of T1-T2 weighted imaging. The most typical lesion detected on FLAIR imaging was periventricular low intensities (PVLI), frequently observed in the neonates with a history of preterm labour, very low birth weight, birth asphyxia and severe respiratory failure. Although we could not characterize the risk factors of PVLI, the incidence of PVLI had a strong correlation with the scores of motor and developmental tests at 12 and 36-months corrected age. In conclusion, FLAIR imaging, detecting the border zone damage of white matter, would be a strong tool to pick out neonates at high risk of neurological disturbances from those without clinical evidence of neurological insults in the neonatal period. (author)

  12. The study of thrombocytopenia in sick neonates

    International Nuclear Information System (INIS)

    Aman, I.; Hassan, K.A.; Ahmad, T.M.

    2004-01-01

    Objective: To determine the number of cases and manifestations of thrombocytopenia in sick neonates. Subjects and Methods: A total of 365 neonates from 0-28 days of age admitted with different clinical problems irrespective of birth weight and gestational age were evaluated for thrombocytopenia. These neonates were categorized into five different groups (A-E), which were of neonatal infections, asphyxia neonatorum, preterm and smallness for gestational age, jaundice and miscellaneous respectively. Results: Out of 365 cases, 88 were found to have thrombocytopenia (platelet counts < 150,000 per mm/sup 3/) which was 24.1% of the total. In group A (neonatal infections), out of 152 neonates, 62 had low platelet counts (40.78%). In group B (neonatal asphyxia), out of 90 only 11 had thrombocytopenia (12.2%). In group C (preterm and small for gestational age), out of 60 cases only 9 had thrombocytopenia. In group D (jaundice), all 33 cases had normal platelet counts. In group E (miscellaneous), out of 30 cases only 6 had thrombocytopenia. The common manifestations in thrombocytopenic babies were petechiae and bruises followed by gastrointestinal hemorrhages. The percentage of manifest thrombocytopenia cases was 56.8% and of occult thrombocytopenia 43.1 %. Conclusion: The leading causes of thrombocytopenia in sick neonates are infections, asphyxia, complicated pre- maturity and smallness for gestational age. Apart from the platelet counts the bleeding mainfestations also depend upon the underlying ailments. (author)

  13. Estimation of adult and neonatal RBC lifespans in anemic neonates using RBCs labeled at several discrete biotin densities.

    Science.gov (United States)

    Kuruvilla, Denison J; Widness, John A; Nalbant, Demet; Schmidt, Robert L; Mock, Donald M; An, Guohua; Veng-Pedersen, Peter

    2017-06-01

    Prior conclusions that autologous neonatal red blood cells (RBC) have substantially shorter lifespans than allogeneic adult RBCs were not based on direct comparison of autologous neonatal vs. allogeneic adult RBCs performed concurrently in the same infant. Biotin labeling of autologous neonatal RBCs and allogeneic adult donor RBCs permits concurrent direct comparison of autologous vs. allogeneic RBC lifespan. RBCs from 15 allogeneic adult donors and from 15 very-low-birth-weight (VLBW) neonates were labeled at separate biotin densities and transfused simultaneously into the 15 neonates. Two mathematical models that account for the RBC differences were employed to estimate lifespans for the two RBC populations. Mean ± SD lifespan for adult allogeneic RBC was 70.1 ± 19.1 d, which is substantially shorter than the 120 d lifespan of both autologous and adult allogeneic RBC in healthy adults. Mean ± SD lifespan for neonatal RBC was 54.2 ± 11.3 d, which is only about 30% shorter than that of the adult allogeneic RBCs. This study provides evidence that extrinsic environmental factors primarily determine RBC survival (e.g., small bore of the capillaries of neonates, rate of oxygenation/deoxygenation cycles) rather than factors intrinsic to RBC.

  14. Reanimación neonatal: actualización Neonatal resuscitation: up-date

    OpenAIRE

    E. Burón Martínez; A. Pino Vázquez

    2009-01-01

    Las últimas recomendaciones en reanimación neonatal fueron publicadas en el año 2005 por el ERC (European Resuscitation Council), el grupo ILCOR (International Liaison Committee on Resuscitation) y la AHA (American Heart Association). En nuestro país estas normas fueron difundidas por el grupo de RCP Neonatal de la Sociedad Española de Neonatología, introduciendo algunas adaptaciones y ampliando algunos capítulos como la reanimación del recién naci...

  15. Pentraxin 3 concentrations of the mothers with preterm premature rupture of membranes and their neonates, and early neonatal outcome.

    Science.gov (United States)

    Akin, Mustafa Ali; Gunes, Tamer; Coban, Dilek; Ozgun, Mahmut Tuncay; Akgun, Hulya; Kurtoglu, Selim

    2015-07-01

    Pentraxin 3 (PTX3) is an acute phase reactant which has been used to detect intra-amniotic infections (IAI) in pregnancy, but the prognostic value of PTX3 concentrations on neonates has not been studied. We aimed to investigate the relationship between maternal PTX3-neonatal PTX3 concentrations and early neonatal outcome. The mothers diagnosed with preterm prelabor rupture of membranes (PPROM) (n = 28) and their preterm infants (n = 28) were included in the study. PTX3 concentrations were studied in plasma in the maternal peripheral blood and umbilical/peripheral vein in the neonates. The relationship between the mPTX3-nPTX3 concentrations and neonatal outcome were investigated using non-parametric tests and binary logistic regression analysis. The mean mPTX3 concentration was 10.35 ± 7.82 μg/L. Ten (35.7%) of all mothers were within the normal range and 18 (64.3%) in high percentile (≥ 97.5 percentile). There was no relation between mPTX3 concentrations and clinical or histologic chorioamnionitis, latency of PPROM, and early neonatal outcome. Mean nPTX3 concentrations was 9.18 ± 7.83 μg/L and high nPTX3 concentrations were detected in five (17.8%) neonates. nPTX3 concentrations were inversely correlated with gestational age and correlated with rate of intraventricular hemorrhage (IVH) and mortality. Neonates with high nPTX3 concentrations also have lowered APGAR scores, increased rate of respiratory distress syndrome, clinical sepsis, IVH, necrotizing enterocolitis and prolonged NICU stay. High PTX3 concentrations of the newborns are associated with some worsened early neonatal outcome including lower gestational age at delivery, increased rate of IVH and mortality. Maternal PTX3 concentrations are not an adequate marker in defining clinical or histologic chorioamnionitis and early neonatal outcome.

  16. Diffusion Weighted Imaging of the Neonatal Brain

    NARCIS (Netherlands)

    J. Dudink (Jeroen)

    2010-01-01

    textabstractAlthough in the last decades advances in fetal and neonatal medicine have reduced mortality in neonatal intensive care units in the Western world, the morbidity due to brain injury remains high. Patterns of neonatal brain injury can be roughly divided in (1) term and (2) preterm

  17. Right paratesticular abscess mimicking neonatal testicular torsion ...

    African Journals Online (AJOL)

    U.O. Ezomike

    Abstract. The clinical presentation of neonatal paratesticular abscess may closely resemble that of, neonatal testicular torsion and the use of scrotal ultrasonography to differentiate the two has low, sensitivity. We propose early operative treatment of suspected neonatal testicular torsion to salvage, the testicle in cases of ...

  18. Malaria parasite positivity among febrile neonates | Enyuma ...

    African Journals Online (AJOL)

    Background: Malaria, earlier considered rare in neonates, has been reported with increasing frequency in the last decade. Neonatal malaria diagnosis is challenging because the clinical features are non-specific, variable and also overlap with bacterial infection. Aim: To determine the prevalence of neonatal malaria and ...

  19. [Cost effectiveness and budget impact analysis of inhaled nitric oxide in a neonatal unit from the perspective of the public health system].

    Science.gov (United States)

    Kilchemmann Fuentes, Carlos; Vallejos Vallejos, Carlos; Román Navarro, Andrés

    Inhaled nitric oxide (iNO) is currently the first-line therapy in severe hypoxaemic respiratory failure of the newborn. Most of regional neonatal centres in Chile do not have this therapeutic alternative. To determine the cost effectiveness of inhaled nitric oxide in the treatment of respiratory failure associated with pulmonary hypertension of the newborn compared to the usual care, including the transfer to a more complex unit. A clinical decision tree was designed from the perspective of Chilean Public Health Service. Incremental cost effectiveness rates (ICER) were calculated, deterministic sensitivity analysis was performed, and probabilistic budget impact was estimated using: TreeAge Pro Healthcare 2014 software. The iNO option leads to an increase in mean cost of $ 11.7 million Chilean pesos (€15,000) per patient treated, with an ICER compared with the usual care of $23 million pesos (€30,000) in case of death or ECMO avoided. By sensitising the results by incidence, it was found that from 7 cases and upwards treated annually, inhaled nitric oxide is less costly than the transfer to a more complex unit. From the perspective of a Chilean regional hospital, incorporating inhaled nitric oxide into the management of neonatal respiratory failure is the optimal alternative in most scenarios. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. Home delivery and neonatal mortality in North Carolina.

    Science.gov (United States)

    Burnett, C A; Jones, J A; Rooks, J; Chen, C H; Tyler, C W; Miller, C A

    1980-12-19

    Neonatal mortality examined by place and circumstances of delivery in North Carolina during 1974 through 1976 with attention given to home delivery. Planned home deliveries by lay-midwives resulted in three neonatal deaths per 1,000 live births; planned home deliveries without a lay-midwife, 30 neonatal deaths per 1,000 live births; and unplanned home deliveries, 120 neonatal deaths per 1,000 live births. The women babies were delivered by lay-midwives were screened in county health departments and found to be medically at low risk of complication, despite having demographic characteristics associated with high-risk of neonatal mortality. Conversely, the women delivered at home without known prenatal screening or a trained attendant had low-risk demographic characteristics but experienced a high rate of neonatal mortality. Planning, prenatal screening, and attendant-training were important in differentiating the risk of neonatal mortality in this uncontrolled, observational study.

  1. Diagnostic imaging in neonatal stroke; Bildgebende Diagnostik des Neonatal stroke

    Energy Technology Data Exchange (ETDEWEB)

    Kuhle, S.; Ipsiroglu, O.; Weninger, M. [Universitaetsklinik fuer Kinder- und Jugendheilkunde, Wien (Austria). Abt. fuer Neonatologie, angeborene Stoerungen und Intensivmedizin; Puig, S.; Prayer, D. [Universitaetsklinik fuer Radiodiagnostik, Wien (Austria)

    2000-01-01

    A cerebral artery infarction is an important differential diagnosis in the newborn with neurological abnormalities. Based on clinical data, its incidence is estimated to be 1 in 4000 newborns. Since the course is often subclinical, the true incidence is probably higher. Diagnosis: Cerebral ultrasound and Doppler sonography as readily available screening tools play a central role in the initial diagnosis of neonatal cerebral infarction. Definitive diagnosis is made by computed tomography or magnetic resonance imaging. Beside symptomatic anticonvulsive therapy, treatment aims at the prevention of secondary ischemic injury. Discussion: Three term infants with different clinical courses of neonatal stroke are presented to sensitize the clinician and the radiologist for this probably underdiagnosed entity. The role of imaging modalities in the diagnosis and follow-up of neonatal cerebral infarction is discussed. (orig.) [German] Ein Infarkt im Stromgebiet der Zerebralarterien stellt eine wichtige Differentialdiagnose bei neurologischen Auffaelligkeiten in der Neonatalperiode dar. Die Inzidenz wird anhand von klinischer Daten auf 1:4000 Lebendgeborene geschaetzt. Da der Verlauf oft subklinisch ist, liegt die wahre Inzidenz wahrscheinlich hoeher. Diagnose: Bei der Diagnosestellung kommen dem Schaedelultraschall und der Doppelsonographie als leicht verfuegbaren Screening-Methoden eine zentrale Rolle zu. Die definitive Diagnose wird, je nach Verfuegbarkeit, mittels Computertomographie oder Kernspintomographie gestellt. Die Behandlung ist neben der symptomatischen (antikonvulsiven) Therapie auf die Vermeidung von ischaemischen Sekundaerschaeden gerichtet. Diskussion: Wir wollen mit der vorliegenden Arbeit anhand von 3 Kindern mit verschiedenen klinischen Verlaeufen eines sog. Neonatal stroke den Stellenwert der bildgebenden Verfahren bei der Diagnostik und Verlaufskontrolle aufzeigen und die Sensibilitaet fuer dieses vermutlich unterdiagnostizierte Krankheitsbild erhoehen

  2. Surfactant therapy and antibiotics in neonates with meconium aspiration syndrome: a systematic review and meta-analysis.

    Science.gov (United States)

    Natarajan, C K; Sankar, M J; Jain, K; Agarwal, R; Paul, V K

    2016-05-01

    Meconium aspiration syndrome (MAS), a common cause of respiratory failure in neonates, is associated with high mortality and morbidity. The objectives of this review were to evaluate the effects of administration of (a) surfactant-either as lung lavage (SLL) or bolus surfactant (BS) and (b) antibiotics on mortality and severe morbidities in neonates with MAS. We searched the following databases: MEDLINE via PubMed, Cochrane CENTRAL, WHOLIS and CABI using sensitive search strategies. We included eight studies on use of surfactant and three studies on use of antibiotics. Neither SLL nor BS reduced the risk of mortality in neonates with MAS (relative risk (RR) 0.38, 95% confidence interval (CI) 0.09 to 1.57; and RR 0.80, 95% CI 0.39 to 1.66, respectively). Both SLL and BS reduced the duration of hospital stay (mean difference -2.0, 95% CI -3.66 to -0.34; and RR -4.68, 95% CI -7.11 to -2.24 days, respectively) and duration of mechanical ventilation (mean difference -1.31, 95% CI -1.91 to -0.72; and mean difference 5.4, 95% CI -9.76 to -1.03 days). Neonates who received BS needed extracorporeal membrane oxygenation (ECMO) less often than the controls (RR 0.64, 95% CI 0.46 to 0.91). Use of antibiotics for MAS did not result in significant reduction in the risk of mortality, sepsis or duration of hospital stay. Surfactant administration either as SLL or BS for MAS was found to reduce the duration of mechanical ventilation and hospital stay; BS also reduced the need for ECMO. Administration of antibiotics did not show any significant clinical benefits in neonates with MAS and no evidence of sepsis. Given the limited number of studies and small number of neonates enrolled, there is an urgent need to generate more evidence on the efficacy and cost-effectiveness of these two treatment modalities before recommending them in routine clinical practice.

  3. A Study On Neonatal Mortality In Jamnagar District Of Gujarat

    Directory of Open Access Journals (Sweden)

    Yadav Sudha

    1998-01-01

    Full Text Available Research question: Which are the maternal, socio-demographic and neonatal attributes responsible for neonatal mortality in rural areas of Gujarat? Objectives: (i To know various maternal, socio-demographic and neonatal factors responsible for neonatal mortality in rural areas of Gujarat (ii To estimate neonatal mortality rate in the area. Setting: Rural areas of six Primary Health Centers of Jamnagar district of Gujarat State. Study design: Community based cohort study. Sample size: Population of 40512 Participants: Members of the family in which neonatal deaths occurred. Outcome variable: Neonatal mortality Analysis: Sample proportions. Results: Neonatal mortality rate on the basis of follow-up of births during one year was found to be 47.27 per thousand live births. The major maternal and socio-demographic factors responsible for neonatal mortality were; maternal age, illiteracy, lack of antenatal care, closely spaced pregnancies, delivery conducted at home, delivery conducted untrained personnel and delayed initiation of breast feeding. The major neonatal factors responsible for mortality in neonates were; low birth weight, prematurity, first order of birth, early phase of neonatal period, male gender of the child. The leading causes of neonatal mortality were found to be prematurity, birth asphyxia, neonatal infections and congenital anomalies.

  4. [Epidemiology of nosocomial infections in neonates].

    Science.gov (United States)

    Lachassinne, E; Letamendia-Richard, E; Gaudelus, J

    2004-03-01

    Epidemiology of nosocomial infections in neonates has to be described according to our definitions (early onset GBS diseases excluded) and according to levels of care. Nosocomial risk exists in maternity departments (3% in postnatal beds), incidence rates are 7.5-12.7% or 1.3-8.5 per 1000 days in neonatal care units and 14.2% or 11.7 per 1000 days in neonatal intensive care units (NICU). Gram-positive cocci bloodstream infections are the most common nosocomial infections in NICU but viral gastroenteritis are more frequent in neonatal care units. Risk factors are low birthweight, small gestational age and intravascular catheter in NICU, and for viral nosocomial infections, visits and winter outbreaks.

  5. Potentially harmful excipients in neonatal medicines

    DEFF Research Database (Denmark)

    Nellis, Georgi; Metsvaht, Tuuli; Varendi, Heili

    2015-01-01

    OBJECTIVES: We aimed to describe administration of eight potentially harmful excipients of interest (EOI)-parabens, polysorbate 80, propylene glycol, benzoates, saccharin sodium, sorbitol, ethanol and benzalkonium chloride-to hospitalised neonates in Europe and to identify risk factors for exposure....... METHODS: All medicines administered to neonates during 1 day with individual prescription and demographic data were registered in a web-based point prevalence study. Excipients were identified from the Summaries of Product Characteristics. Determinants of EOI administration (geographical region......, gestational age (GA), active pharmaceutical ingredient, unit level and hospital teaching status) were identified using multivariable logistical regression analysis. RESULTS: Overall 89 neonatal units from 21 countries participated. Altogether 2095 prescriptions for 530 products administered to 726 neonates...

  6. [Lactose intolerance in neonates with non-infectious diarrhea].

    Science.gov (United States)

    Su, Hui-Min; Jiang, Yi; Hu, Yu-Lian; Yang, Hui; Dong, Tian-Jin

    2016-04-01

    To investigate the development of lactose intolerance in neonates with non-infectious diarrhea and its association with diarrhea, and to evaluate the diagnostic values of fecal pH value and urine galactose determination for neonatal lactase deficiency. Seventy hospitalized neonates who developed non-infectious diarrhea between October 2012 and June 2015 were enrolled as the diarrhea group, and 162 hospitalized neonates without non-infectious diarrhea were enrolled as the non-diarrhea group. Test paper was used to determine fecal pH value. The galactose oxidase method was used to detect urine galactose. The neonates with positive galactose oxidase were diagnosed with lactase deficiency, and those with lactase deficiency and diarrhea were diagnosed with lactose intolerance. According to the results of urine galactose detection, 69 neonates in the diarrhea group who underwent urine galactose detection were classified into lactose intolerance group (45 neonates) and lactose tolerance group (24 neonates), and their conditions after treatment were compared between the two groups. The follow-up visits were performed for neonates with diarrhea at 3 months after discharge. Fecal pH value and positive rate of urine galactose (65% vs 54%) showed no significant differences between the diarrhea and non-diarrhea groups (P>0.05). Fecal pH value showed no significant difference between the lactose intolerance and lactose tolerance groups (P>0.05), while the neonates in the lactose intolerance group had a significantly longer time to recovery of defecation than those in the lactose tolerance group (Plactose intolerance tends to occur. Determination of fecal pH value has no significance in the diagnosis of lactose intolerance in neonates with diarrhea.

  7. Frequency of the hemochromatosis HFE mutations C282Y, H63D, and S65C in blood donors in the Faroe Islands

    DEFF Research Database (Denmark)

    Milman, Nils; á Steig, Torkil; Koefoed, Pernille

    2004-01-01

    on the HFE gene was assessed by genotyping using the polymerase chain reaction (PCR) technique and calculated from direct allele counting. We found no C282Y homozygous subjects; 28 (14.0%) subjects were C282Y heterozygous and four subjects were C282Y/H63D compound heterozygous (2.0%). The C282Y allele......The aim of the study was to assess the frequencies of the hereditary hemochromatosis HFE mutations C282Y, H63D, and S65C in the population in the Faroe Islands. The series comprised 200 randomly selected blood donors of Faroese heritage. The frequency of the C282Y, H63D, and S65C mutations.......6%. Screening of larger groups of the Faroese population for HFE mutations especially C282Y should be considered in order to establish the penetrance....

  8. Surgical procedures performed in the neonatal intensive care unit on critically ill neonates: feasibility and safety

    International Nuclear Information System (INIS)

    Mallick, M.S.; Jado, A.M.; Al-Bassam, A.R.

    2008-01-01

    Transferring unstable, ill neonates to and from the operating rooms carries significant risks and can lead to morbidity. We report on our experience in performing certain procedures in critically ill neonates in the neonatal intensive care unit (NICU). We examined the feasibility and safety for such an approach. All surgical procedures performed in the NICU between January 1999 and December 2005 were analyzed in terms of demographic data, diagnosis, preoperative stability of the patient, procedures performed, complications and outcome. Operations were performed at beside in the NICU in critically ill, unstable neonates who needed emergency surgery, in neonates of low birth weight (<1000 gm) and in neonates on special equipments like higher frequency ventilators and nitrous oxide. Thirty-seven surgical procedures were performed including 12 laparotomies, bowel resection and stomies, 7 repairs of congenital diaphragmatic hernias, 4 ligations of patent ductus arteriosus and various others. Birth weights ranged between 850 gm and 3500 gm (mean 2000 gm). Gestational age ranged between 25 to 42 weeks (mean, 33 weeks). Age at surgery was between 1 to 30 days (mean, 30 days). Preoperatively, 19 patients (51.3%) were on inotropic support and all were intubated and mechanically ventilated. There was no mortality related to surgical procedures. Postoperatively, one patient developed wound infection and disruption. Performing major surgical procedures in the NICU is both feasible and safe. It is useful in very low birth weight, critically ill neonates who have definite risk attached to transfer to the operating room. No special area is needed in the NICU to perform complication-free surgery, but designing an operating room within the NICU will be ideal. (author)

  9. Hemochromatosis (HFE) gene mutations and response to chloroquine in porphyria cutanea tarda.

    Science.gov (United States)

    Stölzel, Ulrich; Köstler, Erich; Schuppan, Detlef; Richter, Matthias; Wollina, Uwe; Doss, Manfred O; Wittekind, Christian; Tannapfel, Andrea

    2003-03-01

    To examine the role of hemochromatosis (HFE) gene mutations, which are associated with porphyria cutanea tarda (PCT), in the therapeutic response to chloroquine. We retrospectively analyzed a database (Excel version 2001 [Microsoft Excel, Redmond, Wash]; date range of search, 1985-1999) of chloroquine-treated patients with PCT on whether HFE mutations (C282Y and H63D) might have influenced the clinical response, urinary porphyrin excretion, liver enzyme activities, and serum iron markers. Serum samples and corresponding complete sets of data before and after therapy were available in 62 of 207 patients with PCT who were treated exclusively with chloroquine. Academic teaching hospital. For treatment, low-dose chloroquine diphosphate, 125 to 250 mg twice weekly, was used during a median time of 16 months (range, 12-26 months). Of the 62 German patients with PCT, 37 (60%) carries HFE mutations. Chloroquine therapy was accompanied by clinical remission and reduced urinary porphyrin excretion (P<.001) in the 24 patients (39%) with HFE wild type as well as in 35 HFE heterozygous patients with PCT (56%). Decreases of serum iron markers following chloroquine therapy were limited to patients with PCT and HFE wild type. All patients homozygous for the C282Y mutation (3 [5%] of 62) had high serum iron, ferritin, and transferrin saturation and failed to respond to chloroquine treatment. The therapeutic response to chloroquine was not compromised by C282Y heterozygosity and compound heterozygosity of HFE mutations. Because HFE C282Y homozygotes (+/+) did not respond to chloroquine and a decrease in serum iron concentration was limited to patients with PCT and HFE wild type, phlebotomy should be first-line therapy in patients with PCT and HFE mutations.

  10. Efficacy of a new technique - INtubate-RECruit-SURfactant-Extubate - "IN-REC-SUR-E" - in preterm neonates with respiratory distress syndrome: study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Vento, Giovanni; Pastorino, Roberta; Boni, Luca; Cota, Francesco; Carnielli, Virgilio; Cools, Filip; Dani, Carlo; Mosca, Fabio; Pillow, Jane; Polglase, Graeme; Tagliabue, Paolo; van Kaam, Anton H.; Ventura, Maria Luisa; Tana, Milena; Tirone, Chiara; Aurilia, Claudia; Lio, Alessandra; Ricci, Cinzia; Gambacorta, Alessandro; Consigli, Chiara; D'Onofrio, Danila; Gizzi, Camilla; Massenzi, Luca; Cardilli, Viviana; Casati, Alessandra; Bottino, Roberto; Pontiggia, Federica; Ciarmoli, Elena; Martinelli, Stefano; Ilardi, Laura; Colnaghi, Mariarosa; Matassa, Piero Giuseppe; Vendettuoli, Valentina; Villani, Paolo; Fusco, Francesca; Gazzolo, Diego; Ricotti, Alberto; Ferrero, Federica; Stasi, Ilaria; Magaldi, Rosario; Maffei, Gianfranco; Presta, Giuseppe; Perniola, Roberto; Messina, Francesco; Montesano, Giovanna; Poggi, Chiara; Giordano, Lucio; Roma, Enza; Grassia, Carolina; Ausanio, Gaetano; Sandri, Fabrizio; Mescoli, Giovanna; Giura, Francesco; Garani, Giampaolo; Solinas, Agostina; Lucente, Maria; Nigro, Gabriella; del Vecchio, Antonello; Petrillo, Flavia; Orfeo, Luigi; Grappone, Lidia; Quartulli, Lorenzo; Scorrano, Antonio; Messner, Hubert; Staffler, Alex; Gargano, Giancarlo; Balestri, Eleonora; Nobile, Stefano; Cacace, Caterina; Meli, Valerio; Dallaglio, Sara; Pasqua, Betta; Mattia, Loretta; Gitto, Eloisa; Vitaliti, Marcello; Re, Maria Paola; Vedovato, Stefania; Grison, Alessandra; Berardi, Alberto; Torcetta, Francesco; Guidotti, Isotta; di Fabio, Sandra; Maranella, Eugenia; Mondello, Isabella; Visentin, Stefano; Tormena, Francesca

    2016-01-01

    Although beneficial in clinical practice, the INtubate-SURfactant-Extubate (IN-SUR-E) method is not successful in all preterm neonates with respiratory distress syndrome, with a reported failure rate ranging from 19 to 69 %. One of the possible mechanisms responsible for the unsuccessful IN-SUR-E

  11. Radiologic findings of neonatal sepsis

    International Nuclear Information System (INIS)

    Kim, Sam Soo; Han, Dae Hee; Choi, Guk Myeong; Jung, Hye Won; Yoon, Hye Kyung; Han, Bokyung Kim; Lee, Nam Yong

    1997-01-01

    To review the simple radiographic and sonographic findings in infants with neonatal sepsis. We retrospectively analyzed simple chest and abdominal radiographs, and brain sonograms in 36 newborn infants (preterm : term=23 :13). With neonatal sepsis diagnosed by blood culture and clinical manifestations. Pulmonary parenchymal infiltrate excluding respiratory distress syndrome and pulmonary edema or atelectasis was found in 22 infants (61%). Paralytic ileus, hepatosplenomegaly, and necrotizing enterocolitis were present in 18(50%), 9(25%), and 1(3%) infants, respectively, while skeletal changes suggesting osteomyelitis were found in three. Brain sonography was performed in 29 infants and in four, abnormalities were seen ; these comprised three germinal matrix hemorrhages and one intraparenchymal hemorrhage. In six patients(17%) radiologic examinations revealed no abnormality. In patients with neonatal sepsis, pulmonary infiltrates and paralytic ileus were common abnormalities. Although these were nonspecific, radiologic findings may be used to supplement clinical and laboratory findings in diagnosing neonatal sepsis and planning its treatment

  12. Radiologic findings of neonatal sepsis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Sam Soo; Han, Dae Hee; Choi, Guk Myeong; Jung, Hye Won [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of); Yoon, Hye Kyung; Han, Bokyung Kim; Lee, Nam Yong [Sansung Medical Center, Seoul (Korea, Republic of)

    1997-06-01

    To review the simple radiographic and sonographic findings in infants with neonatal sepsis. We retrospectively analyzed simple chest and abdominal radiographs, and brain sonograms in 36 newborn infants (preterm : term=23 :13). With neonatal sepsis diagnosed by blood culture and clinical manifestations. Pulmonary parenchymal infiltrate excluding respiratory distress syndrome and pulmonary edema or atelectasis was found in 22 infants (61%). Paralytic ileus, hepatosplenomegaly, and necrotizing enterocolitis were present in 18(50%), 9(25%), and 1(3%) infants, respectively, while skeletal changes suggesting osteomyelitis were found in three. Brain sonography was performed in 29 infants and in four, abnormalities were seen ; these comprised three germinal matrix hemorrhages and one intraparenchymal hemorrhage. In six patients(17%) radiologic examinations revealed no abnormality. In patients with neonatal sepsis, pulmonary infiltrates and paralytic ileus were common abnormalities. Although these were nonspecific, radiologic findings may be used to supplement clinical and laboratory findings in diagnosing neonatal sepsis and planning its treatment.

  13. Neonatal hypothermia in sub-Saharan Africa: a review.

    Science.gov (United States)

    Onalo, R

    2013-01-01

    Hypothermia is a major factor in neonatal morbidity and mortality in developing countries. High prevalence of hypothermia has been reported widely even from warmer tropical countries. In spite of the World Health Organization's recommendation of maintenance of warm chain in newborn care, hypothermia continues to be a common neonatal condition which has remained under-recognized, under-documented, and poorly-managed. This review aims at providing the incidence of and risk factors for neonatal hypothermia as well as provides a pathophysiological overview and management options for neonates with the condition in sub-Saharan Africa. All available published literature on neonatal hypothermia was searched electronically and manually. The principal electronic reference libraries and sites searched were PubMed, Embase, Ajol, Cochrane Reference Libraries and Google Scholar. The search terms used included 'neonatal hypothermia,' 'Cold stress in newborn' 'thermal care of the newborn,' 'neonatal thermogenesis,' 'neonatal cold injury,' among others. Pertinent books and monographs were accessed. Data in formats inaccessible to the reviewer were excluded. Neonatal hypothermia is a major condition of public health importance in countries of sub- Saharan Africa. Awareness of the burden of the disease is still low in some communities. Risk factors for neonatal hypothermia in the region include poverty, home delivery, low birthweight, early bathing of babies, delayed initiation of breastfeeding and inadequate knowledge among health workers. Low-tech facilities to prevent heat losses and provide warmth are available in sub-Saharan Africa and are thus recommended as well as continuous efforts at sensitizing caregivers on the thermal needs of newborns.

  14. Mitochondrial dysfunction in alveolar and white matter developmental failure in premature infants.

    Science.gov (United States)

    Ten, Vadim S

    2017-02-01

    At birth, some organs in premature infants are not developed enough to meet challenges of the extra-uterine life. Although growth and maturation continues after premature birth, postnatal organ development may become sluggish or even arrested, leading to organ dysfunction. There is no clear mechanistic concept of this postnatal organ developmental failure in premature neonates. This review introduces a concept-forming hypothesis: Mitochondrial bioenergetic dysfunction is a fundamental mechanism of organs maturation failure in premature infants. Data collected in support of this hypothesis are relevant to two major diseases of prematurity: white matter injury and broncho-pulmonary dysplasia. In these diseases, totally different clinical manifestations are defined by the same biological process, developmental failure of the main functional units-alveoli in the lungs and axonal myelination in the brain. Although molecular pathways regulating alveolar and white matter maturation differ, proper bioenergetic support of growth and maturation remains critical biological requirement for any actively developing organ. Literature analysis suggests that successful postnatal pulmonary and white matter development highly depends on mitochondrial function which can be inhibited by sublethal postnatal stress. In premature infants, sublethal stress results mostly in organ maturation failure without excessive cellular demise.

  15. iNICU - Integrated Neonatal Care Unit: Capturing Neonatal Journey in an Intelligent Data Way.

    Science.gov (United States)

    Singh, Harpreet; Yadav, Gautam; Mallaiah, Raghuram; Joshi, Preetha; Joshi, Vinay; Kaur, Ravneet; Bansal, Suneyna; Brahmachari, Samir K

    2017-08-01

    Neonatal period represents first 28 days of life, which is the most vulnerable time for a child's survival especially for the preterm babies. High neonatal mortality is a prominent and persistent problem across the globe. Non-availability of trained staff and infrastructure are the major recognized hurdles in the quality care of these neonates. Hourly progress growth charts and reports are still maintained manually by nurses along with continuous calculation of drug dosage and nutrition as per the changing weight of the baby. iNICU (integrated Neonatology Intensive Care Unit) leverages Beaglebone and Intel Edison based IoT integration with biomedical devices in NICU i.e. monitor, ventilator and blood gas machine. iNICU is hosted on IBM Softlayer based cloud computing infrastructure and map NICU workflow in Java based responsive web application to provide translational research informatics support to the clinicians. iNICU captures real time vital parameters i.e. respiration rate, heart rate, lab data and PACS amounting for millions of data points per day per child. Stream of data is sent to Apache Kafka layer which stores the same in Apache Cassandra NoSQL. iNICU also captures clinical data like feed intake, urine output, and daily assessment of child in PostgreSQL database. It acts as first Big Data hub (of both structured and unstructured data) of neonates across India offering temporal (longitudinal) data of their stay in NICU and allow clinicians in evaluating efficacy of their interventions. iNICU leverages drools based clinical rule based engine and deep learning based big data analytical model coded in R and PMML. iNICU solution aims to improve care time, fills skill gap, enable remote monitoring of neonates in rural regions, assists in identifying the early onset of disease, and reduction in neonatal mortality.

  16. Viral infection and antiviral therapy in the neonatal intensive care unit.

    Science.gov (United States)

    Barford, Galina; Rentz, Alison C; Faix, Roger G

    2004-01-01

    Viral diseases are leading causes of mortality and morbidity among infants requiring care in the neonatal intensive care unit (NICU), with ongoing discoveries of new viral pathology likely to add to the burdens posed. Many viral diseases in NICU infants are undiagnosed or appreciated only late in the course because of subtle or asymptomatic presentation, confusion with bacterial disease, and failure to consider viral disease. We present an overview of viral disease in NICU infants, with emphasis on pharmacologic agents currently employed for prophylaxis and treatment of such diseases. Advances in molecular biology and popular demand to develop antiviral agents for viral diseases (eg, human immunodeficiency virus) offer great promise for the future.

  17. Combined therapy in gastro-esophageal reflux disease of term neonates resistant to conservative therapy and monotherapy: a clinical trial

    Directory of Open Access Journals (Sweden)

    Peymaneh Alizadeh Taheri

    2018-05-01

    Full Text Available Background: Gastroesophageal reflux disease (GERD is one of the most common problems in neonates. The main clinical manifestations of neonatal GERD are frequent regurgitation or vomiting associated with irritability, crying, anorexia or feeding refusal, failure to thrive, arching of the back and sleep disturbance.Aims: As no study has compared metoclopramide plus ranitidine with metoclopramide plus omeprazole in the management of neonatal GERD resistant to conservative and monotherapy, this study was carried out.Study design: This study was a randomized clinical trial of term neonates with GERD resistant to conservative and monotherapy admitted to the neonatal ward of Bahrami Children Hospital during 2013-2015. Totally, 116 term neonates (mean age 10.53 ± 8.17 days; girls 50.9% were randomly assigned to a double blind trial with either oral omeprazole plus metoclopramide (group A or oral ranitidine plus metoclopramide (group B. The changes of the symptoms and signs were recorded after one week and one month.Results: There was no significant difference in demographic and baseline characteristics between the two groups. The response rate of “omeprazole plus metoclopramide” was significantly higher than “ranitidine plus metoclopramide” (93.74% ± 7.28% vs. 75.43% ± 23.24%, p = 0.028. All clinical manifestations recovered significantly in group A while the response rate of irritability and wheezing was not significant in group B (primary outcome. There were no side effects in either group after one week and one month of treatment (secondary outcome.Conclusions: The response rate was > 70% in each group, but it was significantly higher in group A (> 90%. Combination of each acid suppressant with metoclopramide led to higher response rate in comparison with monotherapy used before intervention.

  18. 21 CFR 880.5270 - Neonatal eye pad.

    Science.gov (United States)

    2010-04-01

    ... DEVICES GENERAL HOSPITAL AND PERSONAL USE DEVICES General Hospital and Personal Use Therapeutic Devices § 880.5270 Neonatal eye pad. (a) Identification. A neonatal eye pad is an opaque device used to cover... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Neonatal eye pad. 880.5270 Section 880.5270 Food...

  19. Neonatal bacteriemia isolates and their antibiotic resistance pattern in neonatal insensitive care unit (NICU at Beasat Hospital, Sanandaj, Iran.

    Directory of Open Access Journals (Sweden)

    Parvin Mohammadi

    2014-05-01

    Full Text Available Bacteremia continues to result in significant morbidity and mortality, particularly among neonates. There is scarce data on neonatal bacteremia in among Iranian neonates. In this study, we determined neonatal bacteremia isolates and their antibiotic resistance pattern in neonatal insensitive care unit at Beasat hospital, Sanandaj, Iran. During one year, all neonates admitted to the NICU were evaluated. Staphylococcal isolates were subjected to determine the prevalence of MRS and mecA gene. A total of 355 blood cultures from suspected cases of sepsis were processed, of which 27 (7.6% were positive for bacterial growth. Of the 27 isolates, 20 (74% were Staphylococcus spp as the leading cause of bacteremia. The incidence of Gram negative bacteria was 04 (14.8%. The isolated bacteria were resistant to commonly used antibiotics. Maximum resistance among Staphylococcus spp was against Penicillin, and Ampicillin. In our study, the isolated bacteria were 7.5 % Vancomycin and Ciprofloxacin sensitive. Oxacillin disk diffusion and PCR screened 35% and 30% mec a positive Staphylococcus spp. The spectrum of neonatal bacteremia as seen in NICU at Beasat hospital confirmed the importance of pathogens such as Staphylococcus spp. Penicillin, Ampicillin and Cotrimoxazol resistance was high in theses isolates with high mecA gene carriage, probably due to antibiotic selection.

  20. Reduced nephron endowment in the neonates of Indigenous Australian peoples.

    Science.gov (United States)

    Kandasamy, Y; Smith, R; Wright, I M R; Lumbers, E R

    2014-02-01

    Rates of chronic kidney disease (CKD) among Indigenous groups in Australia exceed non-Indigenous rates eight-fold. Using kidney volume as a surrogate for nephron number, we carried out a study to determine if Indigenous neonates have a smaller kidney volume (and thus a reduced nephron number) from birth compared with non-Indigenous neonates. We recruited term and preterm neonates (Indigenous) and 39 term (13 Indigenous) neonates. TKV of Indigenous neonates was significantly lower at 32 weeks [12.0 (2.0) v. 15.4 (5.1) ml; P=0.03] and 38 weeks CA [18.6 (4.0) v. 22.6 (5.9) ml; P=0.04] respectively. Term Indigenous neonates also had smaller kidney volumes compared with non-Indigenous neonates. Despite a smaller kidney volume (and reduced nephron number), Indigenous neonates did not have a significantly lower eGFR. Indigenous neonates achieve similar eGFRs to Non-Indigenous neonates, presumably through a higher single nephron filtration rate. This places Indigenous neonates at a greater risk of long-term kidney damage later in life.

  1. Neonatal herpes in Denmark 1977-1991

    DEFF Research Database (Denmark)

    Fonnest, G; de la Fuente Fonnest, I; Weber, Tom

    1997-01-01

    BACKGROUND: To prevent neonatal herpes, women in labor with genital herpes infection are still delivered by Cesarean section. This policy is currently being debated. The aim of this study was to determine the incidence of neonatal herpes in Denmark and to evaluate the prevention practice. METHODS...... herpes recurrence. Four infants had a serious infection in spite of Cesarean section. This study does not support a policy of Cesarean section in case of maternal recurrent herpes simplex infection at delivery.......BACKGROUND: To prevent neonatal herpes, women in labor with genital herpes infection are still delivered by Cesarean section. This policy is currently being debated. The aim of this study was to determine the incidence of neonatal herpes in Denmark and to evaluate the prevention practice. METHODS......: All newborns with perinatal herpes in Denmark 1977-1991 were identified from hospital-records. RESULTS: Of 862,298 deliveries 136 possible cases were found but only 30 (22%) fulfilled the criteria for neonatal herpes. The incidence increased from 2.36 to 4.56 per 100,000 live births during 1977...

  2. Incidence of congenital heart disease among neonates in a neonatal unit of a tertiary care hospital

    International Nuclear Information System (INIS)

    Hussain, S.; Sabir, M.U.

    2014-01-01

    Objectives: To determine the incidence and pattern of various congenital heart disease in a neonatal unit of a tertiary care hospital. Methods: The prospective study was carried out in the neonatal unit of Combined Military Hospital, Rawalpindi, from September 2008 to August 2011. All 5800 neonates admitted with gestational age of >28 weeks irrespective of birthweight were included in the study. Neonatologist/Paediatrician carried out the neonatal examination during the first 12 hours of life. Neonates suspected of having congenital heart disease were further evaluated by pulse oxymetry, X-ray chest and echocardiography to ascertain final diagnosis and type of lesion. Data was collected on a predesigned proforma containing information regarding gender, mode of delivery, gestational age, weight at birth, family history, and associated malformations. SPSS 16 was used for statistical analysis. Results: Of the 5800 neonates, 87 (1.5%) were found to have congenital heart disease with an incidence of 15/1000. There was a male preponderance. Most common lesion was ventricular septal defect 27(31.3%), followed by atrial septal defect 20 (22.9%), patent ductus arteriosus 13 (14.94%), tetralogy of fallot 06 (6.89%), transposition of great arteries 04 (4.59%), Pulmonary stenosis 05 (5.79%) and 03(3.44%) had atrioventricular canal defects. Conclusion: Congenital heart disease is a common congenital anomaly. Its incidence varies from centre to centre due to different factors like nature of the sample, method of detection and early examination by a neonatologist/paediatrician. In this study a higher incidence is reported because it was carried out in a tertiary care unit, which is a referral hospital and all the neonates admitted in the unit were included in the study. (author)

  3. Parenteral Lipid Dose Restriction With Soy Oil, Not Fish Oil, Preserves Retinal Function in Neonatal Piglets.

    Science.gov (United States)

    Lansing, Marihan; Sauvé, Yves; Dimopoulos, Ioannis; Field, Catherine J; Suh, Miyoung; Wizzard, Pamela; Goruk, Susan; Lim, David; Muto, Mitsuru; Wales, Paul; Turner, Justine

    2018-03-13

    A dietary supply of docosahexaenoic acid (DHA) and arachidonic acid (AA) is critical for neonatal retinal development. Both are absent/minimal in parenteral nutrition (PN) using soy-oil emulsions ([SO] Intralipid®) traditionally used for neonatal intestinal failure. In contrast, fish-oil emulsions ([FO] Omegaven®) are enriched in DHA/AA. The aim of this study was to compare retinal function and fatty acid content in neonatal piglets fed PN with SO or FO. Two-5-day-old piglets were randomly allocated to SO (n = 4) or FO (n = 4), provided at equivalent doses (5g/kg/d). After 14 days of PN, retinal function was assessed by electroretinography and retinas were harvested for fatty acid content analysis. Sow-fed piglets served as a reference (REF). Light flash-elicited stoppage of cone and rod dark-currents (a-waves) and the ensuing postsynaptic activation of cone and rod ON bipolar cells (b-waves) were comparable between SO and REF. Responses recorded from FO were subnormal (P DHA content was similar in both groups (FO, 14.59% vs SO, 12.22%; P = 0.32); while AA was lower in FO (FO, 6.01% vs SO, 8.21%; P = .001). Paradoxically, FO containing more DHA and AA did not preserve retinal function when compared with the same low dose of SO. This may be due to the reduced AA enrichment in the retina with FO treatment. Further investigation into the ideal amounts of DHA and AA for optimal neonatal retinal function is required. © 2018 American Society for Parenteral and Enteral Nutrition.

  4. Improved outcome in neonatal short bowel syndrome using parenteral fish oil in combination with ω-6/9 lipid emulsions.

    Science.gov (United States)

    Angsten, Gertrud; Finkel, Yigael; Lucas, Steven; Kassa, Ann-Marie; Paulsson, Mattias; Lilja, Helene Engstrand

    2012-09-01

    Newborn infants with short bowel syndrome (SBS) represent a high-risk group of developing intestinal failure-associated liver disease (IFALD), which may be fatal. However, infants have a great capacity for intestinal growth and adaptation if IFALD can be prevented or reversed. A major contributing factor to IFALD may be the soybean oil-based intravenous lipid emulsions used since the introduction of parenteral nutrition (PN) 40 years ago. This retrospective study compares the outcome in 20 neonates with SBS treated with parenteral fish oil (Omegaven) in combination with ω-6/9 lipid emulsions (ClinOleic) with the outcome in a historical cohort of 18 patients with SBS who received a soybean oil-based intravenous lipid emulsion (Intralipid). Median gestational age was 26 weeks in the treatment group and 35.5 weeks in the historical group. All patients were started on PN containing Intralipid that was switched to ClinOleic/Omegaven in the treatment group at a median age of 39 gestational weeks. In the treatment group, direct bilirubin levels were reversed in all 14 survivors with cholestasis (direct bilirubin >50 umol/L). Median time to reversal was 2.9 months. Only 2 patients died of liver failure (10%). In the historical cohort, 6 patients (33%) died of liver failure, and only 2 patients showed normalization of bilirubin levels. Parenteral fish oil in combination with ω-6/9 lipid emulsions was associated with improved outcome in premature neonates with SBS. When used instead of traditional soybean-based emulsions, this mixed lipid emulsion may facilitate intestinal adaptation by increasing the IFALD-free period.

  5. Simultaneous occurrence of fetal and neonatal alloimmune thrombocytopenia and neonatal neutropenia due to maternal neutrophilic autoantibodies

    DEFF Research Database (Denmark)

    Taaning, Ellen; Jensen, Lise; Varming, Kim

    2012-01-01

    Foetal and neonatal alloimmune thrombocytopenia (FNAIT) and neonatal neutropenia caused by maternal autoantibodies against neutrophils are rare disorders. We describe a newborn with severe thrombocytopenia and intracerebral bleeding caused by maternal anti-HPA-3a alloantibodies and mild neutropenia...

  6. Intracranial complications of Serratia marcescens infection in neonates.

    Science.gov (United States)

    Madide, Ayanda; Smith, Johan

    2016-03-15

    Even though Serratia marcescens is not one of the most common causes of infection in neonates, it is associated with grave morbidity and mortality. We describe the evolution of brain parenchymal affectation observed in association with S. marcescens infection in neonates. This retrospective case series details brain ultrasound findings of five neonates with hospital-acquired S. marcescens infection. Neonatal S. marcescens infection with or without associated meningitis can be complicated by brain parenchymal affectation, leading to cerebral abscess formation. It is recommended that all neonates with this infection should undergo neuro-imaging more than once before discharge from hospital; this can be achieved using bedside ultrasonography.

  7. Status of Neonatal Pain Assessment and Management in Jordan.

    Science.gov (United States)

    Abdel Razeq, Nadin M; Akuma, Akuma O; Jordan, Sue

    2016-08-01

    Current pain assessment and management in neonates need to be fully described before neonatal pain care can be optimized. This study's purpose was to report neonatal nurses' knowledge, existing pain assessment practice, and pharmacological pain management of neonates in Jordan. A cross-sectional descriptive study was conducted. Eighteen neonatal intensive care units in Jordan were included in the study. One hundred eighty-four neonatal nurses participated. Questionnaires were distributed by and returned to the neonatal intensive care units' managers between June and August 2014. Descriptive and inferential statistics were used to present study results. Of 240 questionnaires distributed, 184 useable responses were returned. Nurses' knowledge regarding neonates' neurological development, nociception, and need for neonatal pain management was suboptimal. The analgesics most commonly used to treat neonatal pain were acetaminophen (52%) and lidocaine (45%). Benzodiazepines, phenobarbitone, and muscles relaxants were also used. Most nurses (54%-97%) reported that pain emanating from most painful procedures was never or rarely treated. Circumcision, lumbar punctures, and chest tube insertion were assigned the highest pain scores (≥9), but were rarely accompanied by analgesia. Pain assessment scales were more likely to be used, and procedural pain was more likely to be treated, in private hospitals than public hospitals. Neonates who require special care still suffer unnecessary pain that could be avoided and managed by following best practice recommendations. Disparities between developed and developing countries in quality of neonatal pain care appear to exist. Resources for education and routine care are needed to address these discrepancies. Copyright © 2016 American Society for Pain Management Nursing. Published by Elsevier Inc. All rights reserved.

  8. NEONATES (BIRTH – 1 MONTH)

    African Journals Online (AJOL)

    Chantel

    Major defects in neonatal skin have serious consequences for the health of the baby, but fortunately these are very rare. Early diagnosis of the genodermatoses can be life-saving. Birthmarks, also known as naevi, are usually first noted in neonates, and can be very alarming for parents. A variety of infections can.

  9. Neonatal Listeriosis

    Directory of Open Access Journals (Sweden)

    Shih-Yu Chen

    2007-01-01

    Full Text Available In Western developed countries, Listeria monocytogenes is not an uncommon pathogen in neonates. However, neonatal listeriosis has rarely been reported in Taiwan. We describe two cases collected from a single medical institute between 1990 and 2005. Case 1 was a male premature baby weighing 1558 g with a gestational age of 31 weeks whose mother had fever with chills 3 days prior to delivery. Generalized maculopapular rash was found after delivery and subtle seizure developed. Both blood and cerebrospinal fluid culture collected on the 1st day yielded L. monocytogenes. In addition, he had ventriculitis complicated with hydrocephalus. Neurologic development was normal over 1 year of follow-up after ventriculoperitoneal shunt operation. Case 2 was a 28-weeks' gestation male premature baby weighing 1180 g. Endotracheal intubation and ventilator support were provided after delivery due to respiratory distress. Blood culture yielded L. monocyto-genes. Cerebrospinal fluid showed pleocytosis but the culture was negative. Brain ultrasonography showed ventriculitis. Sudden deterioration with cyanosis and bradycardia developed on the 8th day and he died on the same day. Neonatal listeriosis is uncommon in Taiwan, but has significant mortality and morbidity. Early diagnosis of perinatal infection relies on high index of suspicion in perinatal health care professionals. [J Formos Med Assoc 2007;106(2:161-164

  10. A new 500 kb haplotype associated with high CD8+ T-lymphocyte numbers predicts a less severe expression of hereditary hemochromatosis

    Directory of Open Access Journals (Sweden)

    Mascarenhas Cláudia

    2008-11-01

    Full Text Available Abstract Background Hereditary Hemochromatosis(HH is a common genetic disorder of iron overload where the large majority of patients are homozygous for one ancestral mutation in the HFE gene. In spite of this remarkable genetic homogeneity, the condition is clinically heterogeneous, varying from a severe disease to an asymptomatic phenotype with only abnormal biochemical parameters. The recent recognition of the variable penetrance of the HH mutation in different large population studies demands the need to search for new modifiers of its phenotypic expression. The present study follows previous observations that MHC class-I linked genetic markers, associated with the setting of CD8+ T-lymphocyte numbers, could be clinically relevant modifiers of the phenotypic expression in HH, and aimed to find new markers that could be used as more reliable prognostic variables. Methods Haplotype analysis, including seven genetic markers within a 1 Mb region around the microsatellite D6S105 was performed in a group of 56 previously characterized C282Y homozygous Portuguese patients. Parameters analyzed in this study were total body iron stores, clinical manifestations related with HH and immunological parameters (total lymphocyte numbers, CD4+ and CD8+ T-lymphocyte numbers. An independent group of 10 C282Y homozygous patients from Vancouver, Canada, were also included in this study and analyzed for the same parameters. Results A highly conserved ancestral haplotype defined by the SNP markers PGBD1-A, ZNF193-A, ZNF165-T (designated as A-A-T was found associated with both abnormally low CD8+ T-lymphocyte numbers and the development of a severe clinical expression of HH. In a small proportion of patients, another conserved haplotype defined by the SNP markers PGBD1-G, ZNF193-G, ZNF165-G (designated as G-G-G was found associated with high CD8+ T-lymphocyte numbers and a milder clinical expression. Remarkably, the two conserved haplotypes defined in Portuguese

  11. Hemochromatosis enhances tumor progression via upregulation of intracellular iron in head and neck cancer.

    Directory of Open Access Journals (Sweden)

    Michelle Lenarduzzi

    Full Text Available Despite improvements in treatment strategies for head and neck squamous cell carcinoma (HNSCC, outcomes have not significantly improved; highlighting the importance of identifying novel therapeutic approaches to target this disease. To address this challenge, we proceeded to evaluate the role of iron in HNSCC.Expression levels of iron-related genes were evaluated in HNSCC cell lines using quantitative RT-PCR. Cellular phenotypic effects were assessed using viability (MTS, clonogenic survival, BrdU, and tumor formation assays. The prognostic significance of iron-related proteins was determined using immunohistochemistry.In a panel of HNSCC cell lines, hemochromatosis (HFE was one of the most overexpressed genes involved in iron regulation. In vitro knockdown of HFE in HNSCC cell lines significantly decreased hepcidin (HAMP expression and intracellular iron level. This in turn, resulted in a significant decrease in HNSCC cell viability, clonogenicity, DNA synthesis, and Wnt signalling. These cellular changes were reversed by re-introducing iron back into HNSCC cells after HFE knockdown, indicating that iron was mediating this phenotype. Concordantly, treating HNSCC cells with an iron chelator, ciclopirox olamine (CPX, significantly reduced viability and clonogenic survival. Finally, patients with high HFE expression experienced a reduced survival compared to patients with low HFE expression.Our data identify HFE as potentially novel prognostic marker in HNSCC that promotes tumour progression via HAMP and elevated intracellular iron levels, leading to increased cellular proliferation and tumour formation. Hence, these findings suggest that iron chelators might have a therapeutic role in HNSCC management.

  12. Hemochromatosis Enhances Tumor Progression via Upregulation of Intracellular Iron in Head and Neck Cancer

    Science.gov (United States)

    Lenarduzzi, Michelle; Hui, Angela B. Y.; Yue, Shijun; Ito, Emma; Shi, Wei; Williams, Justin; Bruce, Jeff; Sakemura-Nakatsugawa, Noriko; Xu, Wei; Schimmer, Aaron; Liu, Fei-Fei

    2013-01-01

    Introduction Despite improvements in treatment strategies for head and neck squamous cell carcinoma (HNSCC), outcomes have not significantly improved; highlighting the importance of identifying novel therapeutic approaches to target this disease. To address this challenge, we proceeded to evaluate the role of iron in HNSCC. Experimental Design Expression levels of iron-related genes were evaluated in HNSCC cell lines using quantitative RT-PCR. Cellular phenotypic effects were assessed using viability (MTS), clonogenic survival, BrdU, and tumor formation assays. The prognostic significance of iron-related proteins was determined using immunohistochemistry. Results In a panel of HNSCC cell lines, hemochromatosis (HFE) was one of the most overexpressed genes involved in iron regulation. In vitro knockdown of HFE in HNSCC cell lines significantly decreased hepcidin (HAMP) expression and intracellular iron level. This in turn, resulted in a significant decrease in HNSCC cell viability, clonogenicity, DNA synthesis, and Wnt signalling. These cellular changes were reversed by re-introducing iron back into HNSCC cells after HFE knockdown, indicating that iron was mediating this phenotype. Concordantly, treating HNSCC cells with an iron chelator, ciclopirox olamine (CPX), significantly reduced viability and clonogenic survival. Finally, patients with high HFE expression experienced a reduced survival compared to patients with low HFE expression. Conclusions Our data identify HFE as potentially novel prognostic marker in HNSCC that promotes tumour progression via HAMP and elevated intracellular iron levels, leading to increased cellular proliferation and tumour formation. Hence, these findings suggest that iron chelators might have a therapeutic role in HNSCC management. PMID:23991213

  13. Impact of HFE genetic testing on clinical presentation of hereditary hemochromatosis: new epidemiological data

    Directory of Open Access Journals (Sweden)

    Ka Chandran

    2005-06-01

    Full Text Available Abstract Background Hereditary hemochromatosis (HH is a common inherited disorder of iron metabolism in Northern European populations. The discovery of a candidate gene in 1996 (HFE, and of its main mutation (C282Y, has radically altered the way to diagnose this disease. The aim of this study was to assess the impact of the HFE gene discovery on the clinical presentation and epidemiology of HH. Methods We studied our cohort of 415 patients homozygous for the C282Y allele and included in a phlebotomy program in a blood centre in western Brittany, France. Results In this cohort, 56.9% of the patients were male and 21.9% began their phlebotomy program before the implementation of the genetic test. A significant decrease in the sex ratio was noticed following implementation of this DNA test, from 3.79 to 1.03 (p -5, meaning that the proportion of diagnosed females relatives to males greatly increased. The profile of HH patients at diagnosis changed after the DNA test became available. Serum ferritin and iron values were lower and there was a reduced frequency of clinical signs displayed at diagnosis, particularly skin pigmentation (20.1 vs. 40.4%, OR = 0.37, p Conclusion This study highlights the importance of the HFE gene discovery, which has simplified the diagnosis of HH and modified its clinical presentation and epidemiology. This study precisely measures these changes. Enhanced diagnosis of HFE-related HH at an early stage and implementation of phlebotomy treatment are anticipated to maintain normal life expectancy for these patients.

  14. Imaging findings of neonatal adrenal disorders

    International Nuclear Information System (INIS)

    Yoon, Hye Kyung; Han, Bo Kyung; Lee, Min Hee

    1999-01-01

    In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surrounded by a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenal gland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenal hemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenal hemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findings in neonates with various adrenal disorders

  15. Imaging findings of neonatal adrenal disorders

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hye Kyung; Han, Bo Kyung; Lee, Min Hee [Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-01-01

    In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surrounded by a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenal gland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenal hemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenal hemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findings in neonates with various adrenal disorders.

  16. Neonatal Near Miss: the need for a standard definition and appropriate criteria and the rationale for a prospective surveillance system

    Directory of Open Access Journals (Sweden)

    Juliana P. Santos

    2015-12-01

    Full Text Available In Latin American, there is currently a regional action with the main purposes of putting the concept of severe neonatal morbidity in practice and formulating proposals for interventions. A general overview of neonatal health conditions, including morbidity and mortality, is provided to update regional knowledge on the topic. An example of the development and implementation of the concept of maternal near miss is also provided, followed by results from a systematic review covering all previously published studies on Neonatal Near Miss. Finally, some proposals for building a common concept on the topic and for launching a prospective surveillance study are presented. A Neonatal Near Miss is a neonate who had a severe morbidity (organ dysfunction or failure but who survived this condition within the first 27 days of life. The pragmatic criteria recommended to be used are as follows: birth weight below 1700 g, Apgar score below 7 at 5 minutes of life and gestational age below 33 weeks. As a proxy for organ dysfunction, the following management criteria are also confirmed: parenteral therapeutic antibiotics; nasal continuous positive airway pressure; any intubation during the first 27 days of life; phototherapy within the first 24 h of life; cardiopulmonary resuscitation; the use of vasoactive drugs, anticonvulsants, surfactants, blood products and steroids for refractory hypoglycemia and any surgical procedure. Although this study starts from a regional perspective, this topic is clearly globally relevant. All nations, especially low and middle-income countries, could benefit from the proposed standardization.

  17. Triagem auditiva neonatal: incidência de deficiência auditiva neonatal sob a perspectiva da nova legislação paulista Neonatal auditory screening: the incidence of neonatal hearing impairment in the context of the new São Paulo legislation

    Directory of Open Access Journals (Sweden)

    Khalil Fouad Hanna

    2010-06-01

    Full Text Available OBJETIVOS: identificar a incidência de recém-nascidos com deficiência auditiva, em maternidade particular da cidade de São Paulo. MÉTODOS: estudo de coorte transversal, realizado no período de 2004 a 2008, em maternidade localizada na zona sul da cidade de São Paulo, com 20.615 recém-nascidos de ambos os sexos, sem indicadores de risco para deficiência auditiva e submetidos à triagem auditiva neonatal. O teste foi realizado por intermédio das Emissões Otoacústicas Evocadas Transientes (EOAET. Os pacientes que falharam nas EOAET nas duas fases foram encaminhados para a realização do Potencial Evocado Auditivo do Tronco Encefálico (PEATE para a confirmação da deficiência auditiva neonatal. Empregou-se o Teste Exato de Fischer e o nível de significância adotado foi de 0,05 oupOBJECTIVES: to determine the incidence of hearing impairment in newborns, at a private maternity hospital in the city of São Paulo. METHODS: a cross-sectional cohort study was carried out covering the period between 2004 and 2008, at a maternity hospital located in the southern zone of the city of São Paulo, including 20,615 newborns of both sexes, with no risk factors for hearing impairment and who had undergone neonatal auditory screening. The test was carried out using the Evoked Transient Otoacoustic Emissions test. Patients who failed both phases of this test were referred to do a Brainstem Auditory Evoked Potential test to confirm the presence of neonatal auditory deficiency. Fischer 's exact test was used with a level of significance of 0.05 orp<0.05. RESULTS: the incidence of neonatal hearing impairment found in this study was 1.2/1000. CONCLUSION: state legislation allows neonatal auditory screening to be more effective in achieving early detection of neonatal hearing impairment. Neonatal auditory screening prevents future impairment of oral development and language acquisition in a social, professional and educational context.

  18. The neonate was born with holoprosencephaly

    Directory of Open Access Journals (Sweden)

    reza saeidi

    2014-12-01

    Full Text Available holoprosencephaly is a rare congenital brain malformation resulting from failure of diverticulation and cleavage of primitive prosencephalon which occurs at 4 - 8th week of gestation and is usually associated with multiple midline facial anomalies. it is the most common forebrain developmental anomaly in humans with prevalence of 1/16,000 in live borns, an incidence as high as 1:250 in conceptuses, and a worldwide distribution6. The etiology of HPE is very heterogeneous. First, this pathology can be caused by environmental or metabolic factors. The only formally recognized environmental factors are insulin-dependent diabetes mellitus (1% risk of HPE and maternal alcoholism with a risk that cumulates with smoking . Clinical expression is variable, extending in unbroken sequence from a small brain with a single cerebral ventricle and cyclopia to clinically unaffected carriers in familial holoprosencephaly. Here. we report a boy 39 weeks neonatal case of holoprosencephaly with Antenatal ultrasonographic diagnosis, with microcephaly, hypotelorism, flat nose, a single nostril, a midline cleft lip and palate microcephaly.

  19. Collectively Facilitated Behavior of the Neonate Caterpillars of Cactoblastis cactorum (Lepidoptera: Pyralidae

    Directory of Open Access Journals (Sweden)

    Terrence D. Fitzgerald

    2016-10-01

    Full Text Available The behavioral biology of the first instar larva of Cactoblastis cactorum was studied from the time of eclosion until the colony penetrated and initiated excavation of the host plant. Hatching from an egg stick was asynchronous, requiring 20 h for the entire cohort to eclose at 50%–70% RH and significantly longer at a lower range of RHs. On eclosion, neonates aggregated in an arena at the base of their egg stick and did not attempt to excavate the cladode until an average of 25 caterpillars had collected, approximately 15 h after the onset of egg hatch. Typically only a single entrance hole was formed, limiting the active process of excavating to one or a few individuals at-a-time until the host was fully penetrated and enlarged internally. Olfactometer tests showed that the neonates are strongly attracted to volatile chemicals released when caterpillars chewed into the cladode, accounting for the strong fidelity of the whole cohort to the initial site of penetration. In one instance, the caterpillars were observed to deal with an explosive release of mucilage by imbibing the liquid until the flooded zone was drained and the caterpillars could reenter the plant through the original entrance hole. Once inside the cladode, marked individuals adopted a regular cycle of defecating at the surface at a mean interval of approximately 10 min when followed for 35 successive cycles. Blanket spraying cladodes with a mandibular gland extract prior to hatching led to the independent dispersal of neonates and a failure to form an arena. When the cladode was impenetrable at the site of eclosion, the active cohort of unfed neonates set off together in search of a new site, marking and following a persistent trail that allowed late-to-eclose caterpillars to join their departed siblings. The adaptive significance of these observations is discussed in the context of the life history of the caterpillar.

  20. Selected Abstracts of the 2nd Congress of joint European Neonatal Societies (jENS 2017; Venice (Italy; October 31-November 4, 2017; Session "Neonatal Pulmonology, Neonatal Respiratory Support, Resuscitation"

    Directory of Open Access Journals (Sweden)

    --- Various Authors

    2017-10-01

    Full Text Available Selected Abstracts of the 2nd Congress of joint European Neonatal Societies (jENS 2017; Venice (Italy; October 31-November 4, 201758th ESPR Annual Meeting, 7th International Congress of UENPS, 3rd International Congress of EFCNIORGANIZING INSTITUTIONSEuropean Society for Paediatric Research (ESPR, European Society for Neonatology (ESN, Union of European Neonatal & Perinatal Societies (UENPS, European Foundation for the Care of Newborn Infants (EFCNIORGANIZING COMMITTEELuc Zimmermann (President of ESPR, Morten Breindahl (President of ESN, Manuel Sánchez Luna (President of UENPS, Silke Mader (Chairwoman of the Executive Board and Co-Founder of EFCNISCIENTIFIC COMMITTEEVirgilio P. Carnielli (Congress President Chair, Pierre Gressens (Past Scientific President, Umberto Simeoni, Manon Benders, Neil Marlow, Ola D. Saugstad, Petra Hüppi, Agnes van den HoogenSession "Neonatal Pulmonology, Neonatal Respiratory Support, Resuscitation"ABS 1. HOW SAFE IS NASAL CANNULAE OXYGEN THERAPY DURING NEONATAL TRANSPORT? • A. Brunton, J. O’Shea, A. JacksonABS 2. WEANING PREMATURE INFANTS FROM NASAL CONTINUOUS POSITIVE AIRWAY PRESSURE – CURRENT PRACTICE IN GERMANY • A. Kidszun, C. Arnold, J. Winter, C. Whybra-Trümpler, E. MildenbergerABS 3. TIMING OF KEY EVENTS IN NEONATAL RE­SUSCITATION • A. Heathcote, J. Jones, P. ClarkeABS 4. COMPARING NON-INVASIVE VENTILATION METHODS IN RESPIRATORY SUPPORT OF PRETERM NEONATES WITH RESPIRATORY DISEASES • A.S. Farhat, A. Mohammadzadeh, G.A. Mamuri, R. Saeidi, S. NoorizadehABS 5. PREDICTORS AND OUTCOMES OF CONTINU­OUS POSITIVE AIRWAY PRESSURE AND NA­SAL HIGH-FLOW TREATMENT FAILURE IN PRE­TERM INFANTS: A SECONDARY ANALYSIS OF A RANDOMIZED TRIAL • B. Manley, C. Roberts, D. Frøisland, L. Doyle, P. Davis, L. OwenABS 6. NEONATAL NON-INVASIVE RESPIRATORY SUPPORT APPROACH: A TWO COUNTRIES COMPARISON • I. Bresesti, S. Zivanovic, G. Lista, C. RoehrABS 7. PREDICTORS OF EARLY CPAP FAILURE IN VERY LOW BIRTH WEIGHT PRETERM

  1. Resistencia a antimicrobianos y evaluación del tratamiento combinado en la septicemia neonatal Resistance to antimicrobials and combination therapy assessment in neonatal sepsis

    Directory of Open Access Journals (Sweden)

    María Espino Hernández

    2003-04-01

    these strains, 94.5% were resistant to several anti biotics. Four strains of coagulase-negative Staphylococcus spp. and one strain of Enterococcus faecium were resistant to vancomycin (minimal inhibitory concentration = 128 mg/mL. We noted a high probability of clinical efficacy (P < 0.01 with anti biotic combinations having a synergistic effect in vitro, whereas antagonic reactions were seen in close association with a highly significant probability of clinical therapeutic failure. DISCUSSION: The main cause of neonatal sepsis in the Neonatal Intensive Care Unit at "América Arias" Hospital in Havana, Cuba, were strains of Staphylococcus resistant to methycillin and other antibiotics. In seriously ill patients (according to policy established at this institution, the first line of treatment is a combination of an aminoglycoside antibiotic and a beta-lactamic antibiotic. This study showed that the clinical efficacy of combination antibiotic therapy is not determined by the resistance pattern of the strain and that good clinical results can be attained, in all likelihood, when a synergistic effect can be shown in vitro.

  2. Fatores de risco para o desenvolvimento de sepse neonatal precoce em hospital da rede pública do Brasil Risk factors for early-onset neonatal sepsis in Brazilian public hospital short-title: early-onset neonatal sepsis

    Directory of Open Access Journals (Sweden)

    Ana Paula Goulart

    2006-06-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: O conhecimento dos fatores de risco associados à sepse neonatal precoce em unidade de neonatologia, inserida na realidade de nosso sistema de saúde, no sentido de se detectar, prevenir e adotar medidas específicas e reduzir as taxas de mortalidade nessa faixa etária. O objetivo deste estudo foi determinar os fatores de risco associados a sepse neonatal precoce em hospital de referência em neonatologia ligado à rede pública de saúde. MÉTODO: Foi realizado um estudo observacional, prospectivo, tipo caso-controle. Foram incluídos os recém-nascidos com diagnóstico de sepse precoce e como controle, recém-nascidos sem infecção neonatal nascido na mesma data do recém-nascido considerado como caso. Foram incluídos 50 casos e três controles para cada caso, resultando em amostra total de 200 pacientes. Foi considerada estatisticamente significativa a associação quando p BACKGROUND AND OBJECTIVES: The determination of the risk factors to early-onset neonatal sepsis in our country is essential to prevent and reduce the mortality associated with this syndrome. Thus, the objective of this study was to determine the frequency and associated risk factors to early-onset neonatal sepsis in public hospital in Southern Brazil. METHODS: Observational, case-control study. Were included neonates with diagnostic of early-onset neonatal sepsis and as controls, neonates without neonatal infection. Were included 50 cases and 3 controls for each case resulting in a total sample of 200 patients. Associations were considered significant when p < 0.05. RESULTS: The sepsis frequency was 50.3 per 1000 born-alive. Risk factors associated to the development of neonatal sepsis were prematurity (OR 9.33; p < 0.001, low birth weight (OR 11.74; p < 0.001, maternal infection (OR 2.28; p = 0.009, mother with history of previous infant with neonatal sepsis (OR 6.43; p = 0.035 and rupture of the membranes more than 18 hours before delivery

  3. Clinical Pharmacology of Paracetamol in Neonates: A Review

    Directory of Open Access Journals (Sweden)

    Gian Maria Pacifici, MD, PhD

    2015-12-01

    Paracetamol clearance is lower in neonates than in children and adults. After metabolic conversion, paracetamol is subsequently eliminated by the renal route. The main metabolic conversions are conjugation with glucuronic acid and with sulphate. In the urine of neonates sulphated paracetamol concentration is higher than the glucuronidated paracetamol level, suggesting that sulfation prevails over glucuronidation in neonates. A loading dose of 20 mg/kg followed by 10 mg/kg every 6 hours of intravenous paracetamol is suggested to achieve a compartment concentration of 11 mg/L in late preterm and term neonates. Aiming for the same target concentration, oral doses are similar with rectal administration of 25 to 30 mg/kg/d in preterm neonates of 30 weeks’ gestation, 45 mg/kg/d in preterm infants of 34 weeks’ gestation, and 60 mg/kg/d in term neonates are suggested. The above-mentioned paracetamol doses for these indications (pain, fever are well tolerated in neonates, but do not result in a significant increase in liver enzymes, and do not affect blood pressure and have limited effects on heart rate. In contrast, the higher doses suggested in extreme preterm neonates to induce closure of the patent ductus arteriosus have not yet been sufficiently evaluated regarding efficacy or safety. Moreover, focussed pharmacovigilance to explore the potential causal association between paracetamol exposure during perinatal life and infancy and subsequent atopy is warranted.

  4. Clinical pharmacokinetics of aminoglycosides in the neonate: a review.

    Science.gov (United States)

    Pacifici, Gian Maria

    2009-04-01

    Sepsis is common in neonates and is a major cause of morbidity and mortality. Sixty percent of preterm neonates receive at least one antibiotic, and 43% of the antibiotics administered to these neonates are aminoglycosides. The clearance (Cl), serum half-life (t(1/2)), and volume of distribution (Vd) of aminoglycosides change during the neonatal life, and the pharmacokinetics of aminoglycosides need to be studied in neonates in order to optimise therapy with these drugs. The aim of this work is to review the published data on the pharmacokinetics of aminoglycosides in order to provide a critical analysis of the literature that can be a useful tool in the hands of physicians. The bibliographic search was performed electronically using PubMed, as the search engine, through July 11th, 2008. Firstly, a Medline search was performed with the keywords "pharmacokinetics of aminoglycosides in neonates" with the limit of "human". Other Medline searches were performed with the keywords "pharmacokinetics of ... in neonates" followed by the name of the aminoglycosides: amikacin, gentamicin, netilmicin and tobramycin. In addition, the book Neofax: A Manual of Drugs Used in Neonatal Care by Young and Mangum (Thomson Healthcare, 2007) was consulted. The aminoglycosides are mainly eliminated by the kidney, and their elimination rates are reduced at birth. As a consequence Cl is reduced and t(1/2) is prolonged in the neonate as compared to more mature infants. The high body-water content of the neonate results in a large Vd of aminoglycosides as these drugs are fairly water soluble. Postnatal development is an important factor in the maturation of the neonate, and as postnatal age proceeds, Cl of aminoglycosides increases. The maturation of the kidney governs the pharmacokinetics of aminoglycosides in the infant. Cl and t(1/2) are influenced by development, and this must be taken into consideration when planning a dosage regimen with aminoglycosides in the neonate. Aminoglycosides

  5. Influence of iron status on risk of maternal or neonatal infection and on neonatal mortality with an emphasis on developing countries

    NARCIS (Netherlands)

    Brabin, Loretta; Brabin, Bernard J.; Gies, Sabine

    2013-01-01

    Infection is a major cause of neonatal death in developing countries. This review investigates whether host iron status affects the risk of maternal and/or neonatal infection, potentially contributing to neonatal death, and summarizes the iron acquisition mechanisms described for pathogens causing

  6. Neonatal follow-up program: Where do we stand?

    Science.gov (United States)

    2012-01-01

    Neonatal follow-up program (NFP) is becoming the corner stone of standard, high quality care provided to newborns at risk of future neuorodevelopmental delay. Most of the recognized neonatal intensive care units in the developed countries are adopting NFP as part of their mandatory care for the best long term outcome of high risk infants, especially very low birth weight (VLBW) infants. Unfortunately, in the developing and in underdeveloped countries, such early detection and intervention programs are rarely existing, mainly because of the lack of awareness of and exposure to such programs in spite of the increasing numbers of surviving sick newborns due to advancement in neonatal care in these countries. This is a review article to explore the Neonatal follow-up programs looking at historical development, benefts and aims, and standard requirements for successful program development that can be adopted in our countries. In conclusion, proper Neonatal follow-up programs are needed to improve neonatal outcome. Therefore all professionals working in the feld of neonatal care in developing countries should cooperate to create such programs for early detection and hence early intervention for any adverse long term outcome in high-risk newborn infants PMID:27493326

  7. Intrapartum FHR monitoring and neonatal CT brain scan

    International Nuclear Information System (INIS)

    Takahashi, Yoshiki; Ukita, Masahiko; Nakada, Eizo

    1982-01-01

    The effect of fetal distress on the neonatal brain was investigated by neonatal CT brain scan, FHR monitoring and mode of delivery. This study involved 11 cases of full term vertex delivery in which FHR was recorded by fetal direct ECG during the second stage labor. All infants weighed 2,500 g or more. FHR monitoring was evaluated by Hon's classification. Neonatal brain edema was evaluated by cranial CT histgraphic analysis (Nakada's method). 1) Subdural hemorrhage was noted in 6 of 7 infants delivered by vacuum extraction or fundal pressure (Kristeller's method). 2) Intracranial hemorrhage was demonstrated in all of 3 infants with 5-min. Apgar score 7 or less. 3) Two cases with prolonged bradycardia and no variability had intraventricular or intracerebral hemorrhage which resulted in severe central nervous system damage. 4) The degree of neonatal brain edema correlated with 5-min. Apgar score. 5) One case with prolonged bradycardia and no variability resulted in severe neonatal brain edema. Four cases with variable deceleration and increased variability resulted in mild neonatal brain edema. Two cases with late deceleration and decreased variability resulted in no neonatal brain edema. (author)

  8. Drug binding properties of neonatal albumin

    DEFF Research Database (Denmark)

    Brodersen, R; Honoré, B

    1989-01-01

    Neonatal and adult albumin was isolated by gel chromatography on Sephacryl S-300, from adult and umbilical cord serum, respectively. Binding of monoacetyl-diamino-diphenyl sulfone, warfarin, sulfamethizole, and diazepam was studied by means of equilibrium dialysis and the binding data were analyzed...... by the method of several acceptable fitted curves. It was found that the binding affinity to neonatal albumin is less than to adult albumin for monoacetyl-diamino-diphenyl sulfone and warfarin. Sulfamethizole binding to the neonatal protein is similarly reduced when more than one molecule of the drug is bound...

  9. Hypothermia for neonatal hypoxic-ischemic encephalopathy: NICHD Neonatal Research Network contribution to the field.

    Science.gov (United States)

    Shankaran, Seetha; Natarajan, Girija; Chalak, Lina; Pappas, Athina; McDonald, Scott A; Laptook, Abbot R

    2016-10-01

    In this article, we summarize the NICHD Neonatal Research Network (NRN) trial of whole-body hypothermia for neonates with hypoxic-ischemic encephalopathy in relation to other randomized controlled trials (RCTs) of hypothermia neuroprotection. We describe the NRN secondary studies that have been published in the past 10 years evaluating clinical, genetic, biochemical, and imaging biomarkers of outcome. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Delivery practices, hygiene, birth attendance and neonatal infections ...

    African Journals Online (AJOL)

    Background: Drawing attention to home birth conditions and subsequent neonatal infections is a key starting point to reducing neonatal morbidity which are a main cause of mortality in sub-Saharan Africa. Objectives: To determine the proportion of respiratory, ophthalmic, and diarrhoeal infections in neonates; the proportion ...

  11. Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus.

    Science.gov (United States)

    Morel, Nathalie; Lévesque, Kateri; Maltret, Alice; Baron, Gabriel; Hamidou, Mohamed; Orquevaux, Pauline; Piette, Jean-Charles; Barriere, François; Le Bidois, Jérôme; Fermont, Laurent; Fain, Olivier; Theulin, Arnaud; Sassolas, François; Hauet, Quentin; Guettrot-Imbert, Gaëlle; Georgin-Lavialle, Sophie; Deligny, Christophe; Hachulla, Eric; Mouthon, Luc; Le Jeunne, Claire; Ravaud, Philippe; Le Mercier, Delphine; Romefort, Bénédicte; Villain, Elisabeth; Bonnet, Damien; Costedoat-Chalumeau, Nathalie

    2017-12-01

    Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM (P=0.0199; HR=3.13 [95% CI: 1.20-8.16]), non-European origin (P=0.0052; HR=4.10 [95% CI: 1.81-9.28]) and pacemaker implantation (P=0.0013; HR=5.48 [95% CI: 1.94-15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth-4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months-22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM (P=0.27; HR=1.65 [95% CI: 0.63-4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. Neurodevelopmental outcome after neonatal perforator stroke

    NARCIS (Netherlands)

    Ecury-Goossen, Ginette M; van der Haer, Marit; Smit, Liesbeth S; Feijen-Roon, Monique; Lequin, Maarten; de Jonge, Rogier C J; Govaert, Paul; Dudink, Jeroen

    AIM: To assess outcome after neonatal perforator stroke in the largest cohort to date. METHOD: Survivors from a cohort of children diagnosed with neonatal perforator stroke using cranial ultrasound or magnetic resonance imaging were eligible for inclusion. Recovery and Recurrence Questionnaire

  13. Oral Dextrose Gel Reduces the Need for Intravenous Dextrose Therapy in Neonatal Hypoglycemia.

    Science.gov (United States)

    Rawat, Munmun; Chandrasekharan, Praveen; Turkovich, Stephen; Barclay, Nancy; Perry, Katherine; Schroeder, Eileen; Testa, Lisa; Lakshminrusimha, Satyan

    2016-01-01

    Newborn infants with risk factors may require intravenous (IV) dextrose for asymptomatic hypoglycemia. Administration of IV dextrose and transfer to the neonatal intensive care unit (NICU) may interfere with parent-infant bonding. To study the effect of implementing dextrose gel supplement with feeds in late preterm/term infants affected by asymptomatic hypoglycemia on reducing IV dextrose therapy. A retrospective study was conducted before and after dextrose gel use: 05/01/2014 to 10/31/2014 and 11/01/2014 to 04/30/2015, respectively. Asymptomatic hypoglycemic (blood glucose level dextrose gel (200 mg/kg of 40% dextrose) along with feeds. Transfer to the NICU for IV dextrose was considered treatment failure. Dextrose gel with feeds increased the blood glucose level in 184/250 (74%) of asymptomatic hypoglycemic infants compared to 144/248 (58%) with feeds only (p dextrose decreased from 35/1,000 to 25/1,000 live births (p dextrose gel with feeds reduced the need for IV fluids, avoided separation from the mother and promoted breastfeeding. Neonates who failed dextrose gel therapy were more likely to be large for gestational age, delivered by cesarean section and had lower baseline blood glucose levels.

  14. Bupivacaine versus lidocaine analgesia for neonatal circumcision

    Directory of Open Access Journals (Sweden)

    Stolik-Dollberg Orit C

    2005-05-01

    Full Text Available Abstract Background Analgesia for neonatal circumcision was recently advocated for every male infant, and its use is considered essential by the American Academy of Pediatrics. We compared the post-operative analgesic quality of bupivacaine to that of lidocaine for achieving dorsal penile nerve block (DPNB when performing neonatal circumcision. Methods Data were obtained from 38 neonates following neonatal circumcision. The infants had received DPNB analgesia with either lidocaine or bupivacaine. The outcome variable was the administration by the parents of acetaminophen during the ensuing 24 hours. Results Seventeen infants received lidocaine and 19 received bupivacaine DPNB. Ten infants in the lidocaine group (59% were given acetaminophen following circumcision compared to only 3 (16% in the bupivacaine group (P 2 = 20.6; P = 0.006. Conclusion DPNB with bupivacaine for neonatal circumcision apparently confers better analgesia than lidocaine as judged by the requirement of acetaminophen over the ensuing 24-hour period.

  15. Structural Connectivity Asymmetry in the Neonatal Brain

    OpenAIRE

    Ratnarajah, Nagulan; Rifkin-Graboi, Anne; Fortier, Marielle V.; Chong, Yap Seng; Kwek, Kenneth; Saw, Seang-Mei; Godfrey, Keith M; Gluckman, Peter D.; Meaney, Michael J.; Qiu, Anqi

    2013-01-01

    Asymmetry of the neonatal brain is not yet understood at the level of structural connectivity. We utilized DTI deterministic tractography and structural network analysis based on graph theory to determine the pattern of structural connectivity asymmetry in 124 normal neonates. We tracted white matter axonal pathways characterizing interregional connections among brain regions and inferred asymmetry in left and right anatomical network properties. Our findings revealed that in neonates, small-...

  16. A community based study of failure to thrive in Israel.

    Science.gov (United States)

    Wilensky, D S; Ginsberg, G; Altman, M; Tulchinsky, T H; Ben Yishay, F; Auerbach, J

    1996-01-01

    OBJECTIVE: To examine the characteristics of infants suffering from failure to thrive in a community based cohort in Israel and to ascertain the effect of failure to thrive on their cognitive development. METHODS: By review of records maintained at maternal and child health clinics in Jerusalem and the two of Beit Shemesh, epidemiological data were obtained at age 15 months on a cohort of all babies born in 1991. For each case of failure to thrive, a matched control was selected from the same maternal and child health clinic. At age 20 months, cognitive development was measured, and at 25 months a home visit was carried out to assess maternal psychiatric status by questionnaire, and the HOME assessment was performed to assess the home environment. RESULTS: 3.9% of infants were found to have fallen below the third centile in weight for at least three months during the first year of life. Infants with failure to thrive did not differ from the general population in terms of obstetric or neonatal complications, birth order, or parents' ethnic origin, age, or years of education. The infants with failure to thrive did have lower birthweights and marginally smaller head circumferences at birth. Developmental assessment at 20 months of age showed a DQ of 99.7 v 107.2 in the matched controls, with 11.5% having a DQ below 80, as opposed to only 4.6% of the controls. No differences were found in maternal psychiatric problems as measured by a self report questionnaire. There were, however, significant differences in subscales of the HOME scale. CONCLUSIONS: (1) Infants who suffered from failure to thrive had some physiological predispositions that put them at risk; (2) failure to thrive may be an early marker of families providing suboptimal developmental stimulation. PMID:8869197

  17. Neonatal transport practices in Ibadan, Nigeria | Abdulraheem | Pan ...

    African Journals Online (AJOL)

    Introduction: Neonatal transport involves moving sick neonates in optimal conditions to ensure good outcomes. It is well organized in most developed countries but receives little attention in developing countries where the highest burden of neonatal mortality exists and a large number of newborns require referrals daily for ...

  18. Comparison of blood lead levels of mothers and cord blood in intrauterine growth retarded neonates and normal term neonates

    International Nuclear Information System (INIS)

    Iranpour, R.; Besharati, Amir A.; Nasseri, F.; Hashemipour, M.; Kelishadi, R.; Balali-Mood, M.

    2007-01-01

    Objective was to compare the blood lead levels of mothers and cord blood in intrauterine growth retarded (IUGR) neonates and normal term neonates. From April 2005, we carried out a cross-sectional, prospective study in Isfahan University of Medical Sciences, Isfahan, Iran. Blood lead levels were measured in the umbilical cord and maternal venous blood samples in the 32 mother-infant pairs with IUGR full term neonates and 34 mother-infant pairs with normal full term neonates. Blood-lead levels were analyzed by atomic absorption spectrometry. The mean lead concentration in neonates of IUGR and normal groups was not significantly different (107.47+- 16.75 versus 113.08+-19.08 ug/L, p=0.2). The mean lead concentration in mothers of IUGR group was lower than normal groups, but this difference was not significant (124.56+-19.71 versus 135.26+-26.91 ug/L, p=0.07). Maternal lead levels were strongly related with related with cord blood in both IUGR and normal groups (r=0.8, p 100ug/L by the centers for disease control; however, this was not statistically different between the groups. Our results indicate that the mean lead level was not higher in IUGR neonates, and the whole blood lead was not related to the birth weight. In addition, maternal and cord blood lead levels were strongly correlated, and there were remarkable lead burdens on both the mothers and their neonates in this industrial area. (author)

  19. Ethical issues in neonatal research involving human subjects.

    Science.gov (United States)

    Fleischman, Alan R

    2016-06-01

    Research involving critically ill neonates creates many ethical challenges. Neonatal clinical research has always been hard to perform, is very expensive, and may generate some unique ethical concerns. This article describes some examples of historical and modern controversies in neonatal research, discusses the justification for research involving such vulnerable and fragile patients, clarifies current federal regulations that govern research involving neonates, and suggests ways that clinical investigators can develop and implement ethically grounded human subjects research. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. The sonographic features of neonatal appendicitis: A case report.

    Science.gov (United States)

    Si, Shu-Yu; Guo, Yi-Yi; Mu, Jian-Feng; Yan, Chao-Ying

    2017-11-01

    Neonatal appendicitis is extremely rare, and preoperative diagnosis is challenging. This study aimed to investigate the utility of ultrasound for the diagnosis of neonatal appendicitis. Four cases of neonatal appendicitis were included in this case series. One was a female infant and the other 3 were male infants; they were aged from 10 to 17 days. Neonatal appendicitis. Four newborns in our hospital were diagnosed with neonatal appendicitis by abdominal ultrasound. Their sonographic features were summarized and compared with surgical and pathological findings. In these infants, abdominal ultrasound demonstrated ileocecal bowel dilatation, intestinal and bowel wall thickening, and localized encapsulated effusion in the right lower quadrant and the abscess area, which was assumed to surround the appendix. Ultrasound is helpful for the diagnosis of neonatal appendicitis.

  1. Involvement of parents in care. Telemedicin neonatal homecare

    DEFF Research Database (Denmark)

    Holm, K. G.; Brødsgaard, Anne; Zachariassen, G.

    2015-01-01

    INTRODUCTION Neonatal homecare (NH) is an alternative to hospitalization for healthy preterm infants dependent only on tube feeding. NH implies parents managing tube feeding while establishing breastfeeding at home with support from neonatal nurses offering home visits regularly, until breastfeed......INTRODUCTION Neonatal homecare (NH) is an alternative to hospitalization for healthy preterm infants dependent only on tube feeding. NH implies parents managing tube feeding while establishing breastfeeding at home with support from neonatal nurses offering home visits regularly, until...... breastfeeding is established. Home visits are time consuming and challenging for neonatal units covering big geographical areas and therefore replacements for home visits should be considered. The aim of this study is to identify parent’s needs when receiving NH and thereby identify requirements...

  2. Prenatal cocaine exposure and neonatal/infant outcomes.

    Science.gov (United States)

    Cambell, Shelly

    2003-01-01

    Illegal drug use throughout the nation is a problem of epidemic proportion. Of particular concern is drug use among pregnant women. In most cases, these women have little hope of achieving a better life for themselves or their children. Illegal drugs, cocaine in particular, can have devastating effects on the neonate. These effects can last well into childhood and can exhibit themselves in academic, social, and family situations. Challenges for the neonatal nurse include early identification of these infants and use of available resources. This article addresses prenatal cocaine use and support services for drug-dependent women, effects of cocaine during the neonatal period, possible neonatal and infant outcomes, and implications for nursing practice.

  3. Time for a neonatal-specific consensus definition for sepsis.

    Science.gov (United States)

    Wynn, James L; Wong, Hector R; Shanley, Thomas P; Bizzarro, Matthew J; Saiman, Lisa; Polin, Richard A

    2014-07-01

    To review the accuracy of the pediatric consensus definition of sepsis in term neonates and to determine the definition of neonatal sepsis used. The review focused primarily on pediatric literature relevant to the topic of interest. Neonatal sepsis is variably defined based on a number of clinical and laboratory criteria that make the study of this common and devastating condition very difficult. Diagnostic challenges and uncertain disease epidemiology necessarily result from a variable definition of disease. In 2005, intensivists caring for children recognized that as new drugs became available, children would be increasingly studied and thus, pediatric-specific consensus definitions were needed. Pediatric sepsis criteria are not accurate for term neonates and have not been examined in preterm neonates for whom the developmental stage influences aberrations associated with host immune response. Thus, specific consensus definitions for both term and preterm neonates are needed. Such definitions are critical for the interpretation of observational studies, future training of scientists and practitioners, and implementation of clinical trials in neonates.

  4. Evidence that the ancestral haplotype in Australian hemochromatosis patients may be associated with a common mutation in the gene

    Energy Technology Data Exchange (ETDEWEB)

    Crawford, D.H.G.; Powell, L.W.; Leggett, B.A. [Univ. of Queensland (Australia)] [and others

    1995-08-01

    Hemochromatosis (HC) is a common inherited disorder of iron metabolism for which neither the gene nor biochemical defect have yet been identified. The aim of this study was to look for clinical evidence that the predominant ancestral haplotype in Australian patients is associated with a common mutation in the gene. We compared indices of iron metabolism and storage in three groups of HC patients categorized according to the presence of the ancestral haplotype (i.e., patients with two copies, one copy, and no copies of the ancestral haplotype). We also examined iron indices in two groups of HC heterozygotes (those with the ancestral haplotype and those without) and in age-matched controls. These analyses indicate that (i) HC patients with two copies of the ancestral haplotype show significantly more severe expression of the disorder than those with one copy or those without, (ii) HC heterozygotes have partial clinical expression, which may be influenced by the presence of the ancestral haplotype in females but not in males, and (iii) the high population frequency of the HC gene may be the result of the selective advantage conferred by protecting heterozygotes against iron deficiency. 18 refs., 3 tabs.

  5. CT of the neonatal head

    International Nuclear Information System (INIS)

    Mohan, S.; Rogan, E.A.; Batty, R.; Raghavan, A.; Whitby, E.H.; Hart, A.R.; Connolly, D.J.A.

    2013-01-01

    Computed tomography (CT) is used less often than other techniques on neonatal units. However, in the acute setting, CT can be invaluable in diagnosing or excluding potentially life-threatening conditions and guiding initial management in neonates. Common indications for scanning include trauma, suspected non-accidental injury, infection, or an acute hypoxic or metabolic event. The aim of this review is to provide an overview of the normal neonatal head at CT and compare this to the common pathological abnormalities. Several key features of each condition will be highlighted. It is important to note that some pathological conditions can have overlapping features at CT and, therefore, the clinical history and additional investigations are also of key importance in determining the diagnosis

  6. [Congenital myotonic dystrophy in a Neonatal Intensive Care Unit: case series].

    Science.gov (United States)

    Domingues, Sara; Alves Pereira, Clara; Machado, Angela; Pereira, Sandra; Machado, Leonilde; Fraga, Carla; Oliveira, Abílio; Vale, Isabel; Quelhas, Ilídio

    2014-02-01

    Steinert myotonic dystrophy is a multisystemic disease, autosomal dominant, with a wide spectrum of severity and clinical manifestations. The most severe form is one that manifests in the neonatal period, called congenital myotonic dystrophy. This condition is distinguished by overall hypotonia at birth and respiratory function compromise. Complications are frequent, mainly psychomotor development delay, growth failure, food difficulties and constipation. It is associated with a poor prognosis, with an overall mortality of up to 50% of severely affected children. We present five patients with congenital myotonic dystrophy in order to describe clinical manifestations, diagnosis, treatment and prognosis. Existing data in the literature on psychomotor development, complications and prognosis of survivors with congenital myotonic dystrophy are scarce. In our case studies, we have found significant chronic psychomotor limitations.

  7. Neonatal sepsis: Highlighting the principles of diagnosis and ...

    African Journals Online (AJOL)

    Neonatal sepsis is a clinical syndrome consisting of nonspecific symptoms and signs of infection, accompanied by a bacteraemia in the first 28 days of life. The risk of neonatal sepsis and death increases with decreasing birth weight and gestational age. South African data have reported the overall incidence of neonatal ...

  8. Maternal Risk Factors for Neonatal Necrotizing Enterocolitis

    Science.gov (United States)

    March, Melissa I.; Gupta, Munish; Modest, Anna M.; Wu, Lily; Hacker, Michele R.; Martin, Camilia R.; Rana, Sarosh

    2015-01-01

    Objective This study aimed to investigate the relationship between maternal hypertensive disease and other risk factors and the neonatal development of necrotizing enterocolitis (NEC). Methods This was a retrospective case control study of infants with NEC from 2008 to 2012. The primary exposure of interest was maternal hypertensive disease, which has been hypothesized to put infants at risk for NEC. Other variables collected included demographics, pregnancy complications, medications, and neonatal hospital course. Data was abstracted from medical records. Results 28 cases of singleton neonates with NEC and 81 matched controls were identified and analyzed. There was no significant difference in the primary outcome. Fetuses with an antenatal diagnosis of growth restriction were more likely to develop NEC (p=0.008). Infants with NEC had lower median birth weight than infants without NEC (p=0.009). Infants with NEC had more late-onset sepsis (p=0.01) and mortality before discharge (p=0.001). Conclusions The factors identified by this case-control study that increased the risk of neonatal NEC included intrauterine growth restriction and lower neonatal birth weight. The primary exposure, hypertensive disease, did not show a significantly increased risk of neonatal NEC, however there was a nearly two-fold difference observed. Our study was underpowered to detect the observed difference. PMID:25162307

  9. [Neonatal cholestasis

    Science.gov (United States)

    Roquete, M L

    2000-07-01

    OBJECTIVE: To warn pediatricians about the early recognition of cholestasis in newborns and infants. METHODS: A bibliographic research about cholestasis was performed using Medline, and emphasizing the most relevant publications of the last 30 years. RESULTS: The concept of cholestasis and the causes of cholestatic tendency in newborns and infants are described. Several causes of intra and extrahepatic cholestasis are reported as well. In this review, only the diseases with diagnostic, therapeutic or prognostic peculiarities are commented, including extrahepatic biliary atresia, idiopathic neonatal hepatitis, galactosemia, and Alagille s syndrome. Furthermore, several resources are discussed for the diagnosis of cholestasis. CONCLUSIONS: The establishment of the diagnosis of cholestasis through the detection of hyperbilirubinemia in newborns who present jaundice after 14 days of life is a goal that could change the prognosis of several diseases responsible for neonatal cholestasis.

  10. Study on the relationship between enkephalin and neonatal asphyxia

    International Nuclear Information System (INIS)

    Hu Dian; Jin Zhijun; Xiong Ying; Cao Liping; Gu Hang; Hong Xinru; Wang Chenghai

    2003-01-01

    Objective: To evaluate the role of enkephalin in the neonatal asphyxia. Methods: Near-term pregnant rabbits were asphyxiated. The rabbit fetuses were delivered by cesarean section and were asphyxiated with the 1 min Apgar scores ranging at 2 to 7. All the neonatal rabbits delivered by asphyxiated mother rabbits were randomized into four groups: neonatal asphyxia untreated (asphyxia group), neonatal asphyxia treated with ICI 174864 ( ICI group), neonatal asphyxia treated with enkephalin anti-serum (anti-serum group), neonatal asphyxia treated with normal rabbit serum (serum group). A group of intact neonatal rabbits delivered by nonasphyxiated mother rabbits were used as control group. All the study neonatal rabbits were scored according to the respiration, heart beat, skin color, muscle tone, reflexes at 1, 5, 10, 15 and 30 min after cesarean delivery. The concentration of enkephalin was measured by radioimmunoassay. Results: 1) The levels of enkephalin in hypothalamus, pituitary and peripheral blood in asphyxia group were (635.2±57.6), (452.7±37.5) and (297.6±28.4) ng/L, respectively; in the control group, (185.7±29.6), (150.9±21.4) and (121.5±19.9) ng/L, respectively. The levels of enkephalin in asphyxia group were significantly higher than that in the control group (P 0.05). Conclusions: The neonatal asphyxia was associated with enkephalin. The changes of enkephalin levels may play an important role in the pathophysiological changes in neonatal asphyxia; delta-receptors may play certain role in conducting the enkephalin effect

  11. Length of stay and cost analysis of neonates undergoing surgery at a tertiary neonatal unit in England.

    Science.gov (United States)

    Shetty, S; Kennea, N; Desai, P; Giuliani, S; Richards, J

    2016-01-01

    Introduction There is a lack of knowledge on the average length of stay (LOS) in neonatal units after surgical repair of common congenital anomalies. There are few if any publications reporting the activity performed by units undertaking neonatal surgery. Such activity is important for contracting arrangements, commissioning specialist services and counselling parents. The aim of this study was to describe postnatal LOS for infants admitted to a single tertiary referral neonatal unit with congenital malformations requiring surgery. Methods Data on nine conditions were collected prospectively for babies on the neonatal unit over a five-year period (2006-2011). For those transferred back to their local unit following surgery, the local unit was contacted to determine the total LOS. Only those babies who had surgery during their first admission to our unit and who survived to discharge were included in the study. Cost estimates were based on the tariffs agreed for neonatal care between our trust and the London specialised commissioning group in 2011-2012. Results The median LOS for the conditions studied was: gastroschisis 35 days (range: 19-154 days), oesophageal atresia 33 days (range: 9-133 days), congenital diaphragmatic hernia 28 days (range: 7-99 days), intestinal atresia 24 days (range: 6-168 days), Hirschsprung's disease 21 days (range: 15-36 days), sacrococcygeal teratoma 17 days (range: 12-55 days), myelomeningocoele 15.5 days (range: 8-24 days), anorectal malformation 15 days (range: 6-90 days) and exomphalos 12 days (range: 3-228 days). The total neonatal bed day costs for the median LOS ranged from £8,701 (myelomeningocoele) to £23,874 (gastroschisis). The cost of surgery was not included. Conclusions There is wide variation in LOS for the same conditions in a single neonatal unit. This can be explained by different types and severity within the same congenital anomalies, different surgeons and other clinical confounders (eg sepsis, surgical

  12. [Clinical efficacy of flomoxef in neonatal bacterial infection].

    Science.gov (United States)

    Sakata, H; Hirano, Y; Maruyama, S

    1993-03-01

    One hundred and seventy one neonates were treated with flomoxef (FMOX) and the clinical efficacy and safety were evaluated. The ages of the patients ranged from 0 to 28 days, and their body weights from 450 to 4300 g. Dose levels were 12.4 to 24.9 mg/kg every 8 or 12 hours for 1 to 10 days. Fifty two patients who responded to the FMOX treatment included 5 neonates with sepsis, 17 with suspected sepsis, 9 with urinary tract infections, 12 with pneumonia, 8 with intrauterine infections, and 1 with omphalitis. The other neonates could not be retrospectively diagnosed as bacterial infections. Of 52 patients, clinical results were excellent in 15, good in 34, fair in 1, and poor in 2. And the FMOX treatment was effective in 13 out of 14 patients in which causative bacteria were identified. The drug was well tolerated, but 6 neonates out of 33 over 5 days old had diarrhea. From these results, empiric treatment with FMOX against neonatal bacterial infection was as clinically useful as that of combination with ampicillin and gentamicin or cefotaxime and ampicillin in our neonatal intensive care unit. But, as this study did not include neonate with meningitis, efficacy to meningitis was not evaluated.

  13. Neonatal Intrathoracic Stomach without Gastric Volvulus.

    Science.gov (United States)

    Bokka, Sriharsha; Mohanty, Manoj Kumar

    2016-10-01

    Intrathoracic stomach is a rare and serious congenital abnormality. The anomaly may be complicated by gastric volvulus and can lead to ischemic gastric infarction in the neonate. If diagnosed antenatally, neonatal management can be planned in advance so as to reduce morbidity. This anomaly must be differentiated from the more common congenital diaphragmatic hernia, as associated pulmonary hypoplasia is common in the latter and rare with gastric herniation. We report a case of intrathoracic stomach in a neonate without volvulus, fortunately a rare entity which was managed operatively, and the child has been under regular follow-up.

  14. Teaching Adult Rats Spinalized as Neonates to Walk Using Trunk Robotic Rehabilitation: Elements of Success, Failure, and Dependence.

    Science.gov (United States)

    Udoekwere, Ubong I; Oza, Chintan S; Giszter, Simon F

    2016-08-10

    Robot therapy promotes functional recovery after spinal cord injury (SCI) in animal and clinical studies. Trunk actions are important in adult rats spinalized as neonates (NTX rats) that walk autonomously. Quadrupedal robot rehabilitation was tested using an implanted orthosis at the pelvis. Trunk cortical reorganization follows such rehabilitation. Here, we test the functional outcomes of such training. Robot impedance control at the pelvis allowed hindlimb, trunk, and forelimb mechanical interactions. Rats gradually increased weight support. Rats showed significant improvement in hindlimb stepping ability, quadrupedal weight support, and all measures examined. Function in NTX rats both before and after training showed bimodal distributions, with "poor" and "high weight support" groupings. A total of 35% of rats initially classified as "poor" were able to increase their weight-supported step measures to a level considered "high weight support" after robot training, thus moving between weight support groups. Recovered function in these rats persisted on treadmill with the robot both actuated and nonactuated, but returned to pretraining levels if they were completely disconnected from the robot. Locomotor recovery in robot rehabilitation of NTX rats thus likely included context dependence and/or incorporation of models of robot mechanics that became essential parts of their learned strategy. Such learned dependence is likely a hurdle to autonomy to be overcome for many robot locomotor therapies. Notwithstanding these limitations, trunk-based quadrupedal robot rehabilitation helped the rats to visit mechanical states they would never have achieved alone, to learn novel coordinations, and to achieve major improvements in locomotor function. Neonatal spinal transected rats without any weight support can be taught weight support as adults by using robot rehabilitation at trunk. No adult control rats with neonatal spinal transections spontaneously achieve similar changes

  15. Magnetic resonance imaging of neonatal brain. Assessment of normal and abnormal findings

    International Nuclear Information System (INIS)

    Hasegawa, Koh; Kadono, Naoko; Kawase, Shohji; Kihara, Minako; Matsuo, Yasutaka; Yoshioka, Hiroshi; Kinugasa, Akihiko; Sawada, Tadashi

    1994-01-01

    To establish the normal MRI appearance of the neonatal brain, magnetic resonance imaging (MRI) was performed on 124 neonates who admitted to our neonatal intensive care unit. Degree of myelination, ventricular size, width of the extracerebral space and focal lesion in the brain were evaluated to investigate the relationship between MRI findings of neonatal brain and the neurological prognosis. 85 neonates underwent MRI both at neonatal period and at the corrected age of one year. The change of abnormal MRI findings was evaluated. 19 neonates had abnormal neurological outcome on subsequent examinations. Delayed myelination, ventriculomegaly and large extracerebral space were seen in 13, 7 and 9 neonates respectively. 4, 3 and 5 neonates out of them showed abnormal neurological prognosis respectively. Of the 19 neonates with focal lesion in MRI, 2 had parenchymal hematoma in the brain, 2 had subdural hematoma, 5 had chronic hematoma following subependymal hemorrhage, 6 had cystic formation following subependymal hemorrhage, 2 had subcortical leukomalacia, one had periventricular leukomalacia and one had cyst in the parenchyma of cerebellum. 4 neonates of 19 with focal lesion in MRI showed abnormal development. Of the neonates who had abnormal neurological prognosis, 7 neonates showed no abnormal finding in MRI at neonatal period. 3 of them had mild mental retardation. MRI shows promise in the neonatal period. It facilitates recognition of abnormalities of neonatal brain and may be used to predict abnormal neurologic outcome. However physiological change in the brain of neonates, especially of premature neonates, should be considered on interpreting these findings. Awareness of developmental features should help to minimize misinterpretation of normal changes in the neonatal brain. (author)

  16. Magnetic resonance imaging of neonatal brain. Assessment of normal and abnormal findings

    Energy Technology Data Exchange (ETDEWEB)

    Hasegawa, Koh; Kadono, Naoko; Kawase, Shohji; Kihara, Minako; Matsuo, Yasutaka; Yoshioka, Hiroshi; Kinugasa, Akihiko; Sawada, Tadashi (Kyoto Prefectural Univ. of Medicine (Japan))

    1994-11-01

    To establish the normal MRI appearance of the neonatal brain, magnetic resonance imaging (MRI) was performed on 124 neonates who admitted to our neonatal intensive care unit. Degree of myelination, ventricular size, width of the extracerebral space and focal lesion in the brain were evaluated to investigate the relationship between MRI findings of neonatal brain and the neurological prognosis. 85 neonates underwent MRI both at neonatal period and at the corrected age of one year. The change of abnormal MRI findings was evaluated. 19 neonates had abnormal neurological outcome on subsequent examinations. Delayed myelination, ventriculomegaly and large extracerebral space were seen in 13, 7 and 9 neonates respectively. 4, 3 and 5 neonates out of them showed abnormal neurological prognosis respectively. Of the 19 neonates with focal lesion in MRI, 2 had parenchymal hematoma in the brain, 2 had subdural hematoma, 5 had chronic hematoma following subependymal hemorrhage, 6 had cystic formation following subependymal hemorrhage, 2 had subcortical leukomalacia, one had periventricular leukomalacia and one had cyst in the parenchyma of cerebellum. 4 neonates of 19 with focal lesion in MRI showed abnormal development. Of the neonates who had abnormal neurological prognosis, 7 neonates showed no abnormal finding in MRI at neonatal period. 3 of them had mild mental retardation. MRI shows promise in the neonatal period. It facilitates recognition of abnormalities of neonatal brain and may be used to predict abnormal neurologic outcome. However physiological change in the brain of neonates, especially of premature neonates, should be considered on interpreting these findings. Awareness of developmental features should help to minimize misinterpretation of normal changes in the neonatal brain. (author).

  17. Maternal education and age: inequalities in neonatal death.

    Science.gov (United States)

    Fonseca, Sandra Costa; Flores, Patricia Viana Guimarães; Camargo, Kenneth Rochel; Pinheiro, Rejane Sobrino; Coeli, Claudia Medina

    2017-11-17

    Evaluate the interaction between maternal age and education level in neonatal mortality, as well as investigate the temporal evolution of neonatal mortality in each stratum formed by the combination of these two risk factors. A nonconcurrent cohort study, resulting from a probabilistic relationship between the Mortality Information System and the Live Birth Information System. To investigate the risk of neonatal death we performed a logistic regression, with an odds ratio estimate for the combined variable of maternal education and age, as well as the evaluation of additive and multiplicative interaction. The neonatal mortality rate time series, according to maternal education and age, was estimated by the Joinpoint Regression program. The neonatal mortality rate in the period was 8.09‰ and it was higher in newborns of mothers with low education levels: 12.7‰ (adolescent mothers) and 12.4‰ (mother 35 years old or older). Low level of education, without the age effect, increased the chance of neonatal death by 25% (OR = 1.25, 95%CI 1.14-1.36). The isolated effect of age on neonatal death was higher for adolescent mothers (OR = 1.39, 95%CI 1.33-1.46) than for mothers aged ≥ 35 years (OR = 1.16, 95%CI 1.09-1.23). In the time-trend analysis, no age group of women with low education levels presented a reduction in the neonatal mortality rate for the period, as opposed to women with intermediate or high levels of education, where the reduction was significant, around 4% annually. Two more vulnerable groups - adolescents with low levels of education and older women with low levels of education - were identified in relation to the risk of neonatal death and inequality in reducing the mortality rate.

  18. The Tuning of Human Neonates' Preference for Speech

    Science.gov (United States)

    Vouloumanos, Athena; Hauser, Marc D.; Werker, Janet F.; Martin, Alia

    2010-01-01

    Human neonates prefer listening to speech compared to many nonspeech sounds, suggesting that humans are born with a bias for speech. However, neonates' preference may derive from properties of speech that are not unique but instead are shared with the vocalizations of other species. To test this, thirty neonates and sixteen 3-month-olds were…

  19. Neonatal Intestinal Obstruction-Four Year Experience

    Directory of Open Access Journals (Sweden)

    D. Rathore

    2015-06-01

    Full Text Available Aim of study: To study the aetiology and frequency, sex incidence, age of presentation, management and outcome of neonatal intestinal obstruction. Material and Methods: This prospective study of 316 neonates with intestinal obstruction was conducted over a period of 4 years from November 2009 to October 2013 at single institute. These cases were managed by various surgical procedures. Their epidemiology, day of presentation, associated anomalies and outcomes were studied. Results: A total of 316 neonates (277 males and 39 females were operated for intestinal obstruction. 268(84.81% neonates presented in the 1st week of life. Imperforate anus occurred in 206 (65.19%.Small bowel atresia accounted for23 (7.27% cases while duodenal atresia was seen in19 (6.01% patients. Infantile hypertrophic pyloric stenosis and Malrotation each occurred in 14 (4.43% patients; Hirschsprung’s disease in 18(5.69%, Necrotising Enterocolitis in 12(3.79%, Meconium disease of newborn in 9(2.85% while colonic atresia was seen in one (0.3% patient. Colostomy was performed in 145(45.88%, Pouchostomy in 15(4.74% and Cutback anoplasty in 56(17.72% patients. Ramsted’s Pyloromyotomy in 13(4.11%% neonates, Laparoscopic Pyloromyotomy in 1(0.3%,Kimura’s Duodenoduodenostomy in 19(6.01% ,End to Back anastomosis in 24(7.59% , End to End anastomosis in 7(2.21% , Multiple anastomosis in 2(0.6% , Enterotomy with irrigation in 7(2.21% , Ladd’s procedure in 14(4.43% , ,Single stage transanal pull through in 8(2.53% , Ileostomy in 2(0.6% , Single stage Abdominoperineal pull through in 2(0.6%, Levelling colostomy in 6(1.89% ,Peritoneal drain insertion under Local anaesthesia in 5(1.58% . Overall mortality was 13.60%. Conclusion: Intestinal Obstruction is the most common surgical emergency in neonatal period. Early and accurate diagnosis is paramount for proper patient management. The etiology, mode of presentation, morbidity and outcome of surgery of intestinal obstruction in

  20. Managing neonatal bowel obstruction: clinical perspectives

    Directory of Open Access Journals (Sweden)

    Desoky SM

    2018-02-01

    Full Text Available Sarah M Desoky,1 Ranjit I Kylat,2 Unni Udayasankar,1 Dorothy Gilbertson-Dahdal1 1Department of Medical Imaging, University of Arizona College of Medicine, Tucson, AZ, USA; 2Division of Neonatal-Perinatal Medicine, Department of Pediatrics, University of Arizona College of Medicine, Tucson, AZ, USA Abstract: Neonatal intestinal obstruction is a common surgical emergency and occurs in approximately 1 in 2,000 live births. The causes of obstruction are diverse with varied embryological origins, and some underlying etiologies are not yet well described. Some findings of neonatal bowel obstruction can be detected prenatally on ultrasound imaging. The obstruction is classified as “high” when the level of obstruction is proximal to the ileum, and “low” when the level of obstruction is at the ileum or colon. Early diagnosis of the type of intestinal obstruction and localization of the obstructive bowel segment guides timely and appropriate management of the underlying pathologic entity. Neonatal bowel obstructions are ideally managed at specialized centers with a large volume of neonatal surgery and dedicated pediatric surgical and anesthesia expertise. Although surgical intervention is necessary in most cases, initial management strategies often target underlying metabolic, cardiac, or respiratory abnormalities. Imaging plays a key role in early and accurate diagnosis of the abnormalities. When bowel obstruction is suspected clinically, initial imaging workup usually involves abdominal radiography, which may direct further evaluation with fluoroscopic examination such as upper gastrointestinal (UGI contrast study or contrast enema. This article provides a comprehensive review of clinical and radiological features of common and less common causes of intestinal obstruction in the neonatal age group, including esophageal atresia, enteric duplication cysts, gastric volvulus, congenital microgastria, hypertrophic pyloric stenosis, duodenal atresia

  1. Pattern and prevalence of neonatal thrombocytopenia in Port Harcourt, Nigeria

    Directory of Open Access Journals (Sweden)

    Zaccheaus A Jeremiah

    2010-04-01

    Full Text Available Zaccheaus A Jeremiah1, Justina E Oburu21Hematology and Blood Transfusion Science Unit, Department of Medical Laboratory Sciences, College of Health Sciences, Niger Delta University, Wilberforce Island, Bayelsa State, Nigeria; 2Department of Hematology and Blood Transfusion University of Port Harcourt Teaching Hospital, Port Harcourt, NigeriaBackground: In Port Harcourt, evidence-based guidelines for platelet transfusion therapy in neonatal patients are yet to be defined and the prevalence and pattern of neonatal thrombocytopenia has not yet been reported.Methods: Platelet counts of 132 neonatal patients admitted into the special care baby unit (SCBU at the University of Port Harcourt Teaching Hospital in Nigeria were assessed using the International Committee on Standards in Hematology (ICSH approved manual procedures for hemocytometry.6Study design: This is a cross sectional study carried out on neonates to determine the prevalence and pattern of neonatal thrombocytopenia.Results: The median platelet count of the neonates was 97.0 × 109/L (interquartile range [IQR] 50–152 while the mean age was 61.7 hours (range 1–336 hours. The overall prevalence of neonatal thrombocytopenia was 53.0%. Mild thrombocytopenia (platelet count 51–100 × 109/L was found in 39.4% of the neonates, 12.1% had moderate thrombocytopenia (platelet count 30–50 × 109/L, while severe thrombocytopenia (platelet count <30 × 109/L was detected in 1.5% of the neonates. Of these, 84.84% of the cases occurred within 72 hours (early onset. The most common clinical diagnosis among the neonates was severe birth asphyxia (33.3%, followed by neonatal jaundice (19.7%, neonatal sepsis (16.7%, low birth weight (13.6%, anemia and bleeding (6.1%, and other clinical conditions (10.6%. There was no association between clinical diagnosis and thrombocytopenia (Fisher’s exact test = 10.643; P = 0.923.Conclusion: There is a high prevalence of early onset neonatal thrombocytopenia

  2. A mortalidade neonatal em 1998, no município de Botucatu - SP La mortalidad neonatal en 1998, en el município de Botucatu-SP The neonatal mortality in 1998 at the municipality of Botucatu-SP

    Directory of Open Access Journals (Sweden)

    Kátia Poles

    2000-07-01

    Full Text Available Considerando que a mortalidade neonatal é indicador da qualidade da assistência prestada à gestante, ao parto e ao recém-nascido, realizamos o presente trabalho, cujo objetivo foi identificar as causas e o índice de mortalidade neonatal durante o ano de 1998 em Botucatu-SP. O coeficiente de mortalidade neonatal obtido foi de 8,3/1000 nascidos vivos e o coeficiente de mortalidade neonatal precoce foi de 7,3/1000 nascidos vivos, confirmando a importância dos óbitos na primeira semana de vida. Aproximadamente três quartos dos óbitos puderam ser classificados como reduzíveis por diagnóstico e tratamento precoces, reduzíveis por adequada atenção ao parto ou parcialmente reduzíveis por adequado controle da gravidez, evidenciando que para se reduzir os índices de morte neonatal, deveremos investir na melhoria da qualidade da assistência prestada à gestante, à parturiente e ao neonato.Considerando que la mortalidad neonatal es un indicador de la calidad de la asistencia ofrecida en la gestación, al parto y al recién- nacido, realizamos el presente trabajo, con el objetivo de estudiar la mortalidad neonatal durante el año de 1998 en Botucatu-SP. El coeficiente de mortalidad neonatal encontrado fue 8,3/1000 nacidos vivos y el coeficiente de mortalidad neonatal precoz fue de 7,3/1000 nacidos vivos, confirmando la importancia de las defunciones en la primera semana de vida. Los resultados mostraron que aproximadamente tres cuartos de las defunciones pueden ser reducidas con el control del embarazo, con el diagnóstico y tratamiento precoz o con la adecuada atención al parto, evidenciando que para reducir los índices de mortalidad neonatal, deberemos invertir en la mejoría de la calidad de la asistencia ofrecida en la gestación, en el parto y al recién-nacido.Considering that neonatal mortality is an indicator of the quality of the care provided to pregnant women, at childbirth as well as to the new born, authors developed the

  3. Diagnostic imaging in neonatal stroke

    International Nuclear Information System (INIS)

    Kuhle, S.; Ipsiroglu, O.; Weninger, M.

    2000-01-01

    A cerebral artery infarction is an important differential diagnosis in the newborn with neurological abnormalities. Based on clinical data, its incidence is estimated to be 1 in 4000 newborns. Since the course is often subclinical, the true incidence is probably higher. Diagnosis: Cerebral ultrasound and Doppler sonography as readily available screening tools play a central role in the initial diagnosis of neonatal cerebral infarction. Definitive diagnosis is made by computed tomography or magnetic resonance imaging. Beside symptomatic anticonvulsive therapy, treatment aims at the prevention of secondary ischemic injury. Discussion: Three term infants with different clinical courses of neonatal stroke are presented to sensitize the clinician and the radiologist for this probably underdiagnosed entity. The role of imaging modalities in the diagnosis and follow-up of neonatal cerebral infarction is discussed. (orig.) [de

  4. Neonatal maxillary orthopedics: past to present

    NARCIS (Netherlands)

    Kuijpers-Jagtman, A.M.; Prahl, C.; Berkowitz, S.

    2013-01-01

    Neonatal maxillary orthopedics was introduced in the treatment protocol for cleft lip and palate in the 1950s of the last century. A wide range of appliances has been designed with pin-retained active appliances at one end of the spectrum and passive appliances at the other. Although neonatal

  5. Neonatal seizures : Aetiology by means of a standardized work-up

    NARCIS (Netherlands)

    Loman, Annemiek M. W.; ter Horst, Henk J.; Lambrechtsen, Florise A. C. P.; Lunsing, Roelineke J.

    Neonatal seizures are an alarming symptom and are frequent in neonates. It is important to find the cause of neonatal seizures to start a specific treatment and to give a meaningful prognosis. The aim of this study is to investigate the incidence of different aetiologies of neonatal seizures in our

  6. Tei index in neonatal respiratory distress and perinatal asphyxia

    OpenAIRE

    Ahmed Anwer Attia Khattab

    2015-01-01

    Cardiovascular compromise is a common complication of neonatal respiratory distress and perinatal asphyxia. Tei index is a Doppler-derived index for the assessment of overall left ventricular function that combines systolic and diastolic time intervals. Aim: Assess the role of MPI versus cardiac troponin I as early indicator of hypoxic cardiac damage in neonates with respiratory distress or perinatal asphyxia. The present work was conducted on forty neonates, 15 with neonatal respiratory dist...

  7. Bayesian automated cortical segmentation for neonatal MRI

    Science.gov (United States)

    Chou, Zane; Paquette, Natacha; Ganesh, Bhavana; Wang, Yalin; Ceschin, Rafael; Nelson, Marvin D.; Macyszyn, Luke; Gaonkar, Bilwaj; Panigrahy, Ashok; Lepore, Natasha

    2017-11-01

    Several attempts have been made in the past few years to develop and implement an automated segmentation of neonatal brain structural MRI. However, accurate automated MRI segmentation remains challenging in this population because of the low signal-to-noise ratio, large partial volume effects and inter-individual anatomical variability of the neonatal brain. In this paper, we propose a learning method for segmenting the whole brain cortical grey matter on neonatal T2-weighted images. We trained our algorithm using a neonatal dataset composed of 3 fullterm and 4 preterm infants scanned at term equivalent age. Our segmentation pipeline combines the FAST algorithm from the FSL library software and a Bayesian segmentation approach to create a threshold matrix that minimizes the error of mislabeling brain tissue types. Our method shows promising results with our pilot training set. In both preterm and full-term neonates, automated Bayesian segmentation generates a smoother and more consistent parcellation compared to FAST, while successfully removing the subcortical structure and cleaning the edges of the cortical grey matter. This method show promising refinement of the FAST segmentation by considerably reducing manual input and editing required from the user, and further improving reliability and processing time of neonatal MR images. Further improvement will include a larger dataset of training images acquired from different manufacturers.

  8. Neonatal tetanus elimination in Pakistan: progress and challenges.

    Science.gov (United States)

    Lambo, Jonathan A; Nagulesapillai, Tharsiya

    2012-12-01

    Pakistan is one of the 34 countries that have not achieved the neonatal tetanus (NT) global elimination target set by the World Health Organization (WHO). NT, caused by Clostridium tetani, is a highly fatal infection of the neonatal period. It is one of the most underreported diseases and remains a major but preventable cause of neonatal and infant mortality in many developing countries. In 1989, the World Health Assembly called for the elimination of NT by 1995, and since then considerable progress has been made using the following strategies: clean delivery practices, routine tetanus toxoid (TT) immunization of pregnant women, and immunization of all women of childbearing age with three doses of TT vaccine in high-risk areas during supplementary immunization campaigns. This review presents the activities, progress, and challenges in achieving NT elimination in Pakistan. A review of the literature found TT vaccination coverage in Pakistan ranged from 60% to 74% over the last decade. Low vaccination coverage, the main driver for NT in Pakistan, is due to many factors, including demand failure for TT vaccine resulting from inadequate knowledge of TT vaccine among reproductive age females and inadequate information about the benefits of TT provided by health care workers and the media. Other factors linked to low vaccination coverage include residing in rural areas, lack of formal education, poor knowledge about place and time to get vaccinated, and lack of awareness about the importance of vaccination. A disparity exists in TT vaccination coverage and antenatal care between urban and rural areas due to access and utilization of health care services. NT reporting is incomplete, as cases from the private sector and rural areas are underreported. To successfully eliminate NT, women of reproductive age must be made aware of the benefits of TT vaccine, not only to themselves, but also to their families. Effective communication strategies for TT vaccine delivery and

  9. Differing impact of the deletion of hemochromatosis-associated molecules HFE and transferrin receptor-2 on the iron phenotype of mice lacking bone morphogenetic protein 6 or hemojuvelin.

    Science.gov (United States)

    Latour, Chloé; Besson-Fournier, Céline; Meynard, Delphine; Silvestri, Laura; Gourbeyre, Ophélie; Aguilar-Martinez, Patricia; Schmidt, Paul J; Fleming, Mark D; Roth, Marie-Paule; Coppin, Hélène

    2016-01-01

    Hereditary hemochromatosis, which is characterized by inappropriately low levels of hepcidin, increased dietary iron uptake, and systemic iron accumulation, has been associated with mutations in the HFE, transferrin receptor-2 (TfR2), and hemojuvelin (HJV) genes. However, it is still not clear whether these molecules intersect in vivo with bone morphogenetic protein 6 (BMP6)/mothers against decapentaplegic (SMAD) homolog signaling, the main pathway up-regulating hepcidin expression in response to elevated hepatic iron. To answer this question, we produced double knockout mice for Bmp6 and β2-microglobulin (a surrogate for the loss of Hfe) and for Bmp6 and Tfr2, and we compared their phenotype (hepcidin expression, Bmp/Smad signaling, hepatic and extrahepatic tissue iron accumulation) with that of single Bmp6-deficient mice and that of mice deficient for Hjv, alone or in combination with Hfe or Tfr2. Whereas the phenotype of Hjv-deficient females was not affected by loss of Hfe or Tfr2, that of Bmp6-deficient females was considerably worsened, with decreased Smad5 phosphorylation, compared with single Bmp6-deficient mice, further repression of hepcidin gene expression, undetectable serum hepcidin, and massive iron accumulation not only in the liver but also in the pancreas, the heart, and the kidneys. These results show that (1) BMP6 does not require HJV to transduce signal to hepcidin in response to intracellular iron, even if the loss of HJV partly reduces this signal, (2) another BMP ligand can replace BMP6 and significantly induce hepcidin expression in response to extracellular iron, and (3) BMP6 alone is as efficient at inducing hepcidin as the other BMPs in association with the HJV/HFE/TfR2 complex; they provide an explanation for the compensatory effect of BMP6 treatment on the molecular defect underlying Hfe hemochromatosis in mice. © 2015 by the American Association for the Study of Liver Diseases.

  10. Neonatal Sepsis: past, present and future; a review article | Tripathi ...

    African Journals Online (AJOL)

    Sepsis is the most common cause of neonatal mortality. As per National Neonatal Perinatal Database (NNPD) 2002-2003, the incidence of neonatal sepsis in India was 30 per 1000 live birth. It is 3% among intramural babies and 39.7% among extramural admissions. The early manifestations of neonatal sepsis are vague ...

  11. Long-term use of neonatal helmet-CPAP: a case report.

    Science.gov (United States)

    Doglioni, N; Micaglio, M; Zanardo, V; Trevisanuto, D

    2009-12-01

    In a recent short-term physiological study, we demonstrated a new continuous positive airway pressure (CPAP) system (neonatal helmet-CPAP) that could be a feasible device for managing preterm infants needing continuous distending pressure with better tolerability than nasal-CPAP. However, its application for a long-term period has never been reported in neonates. Here, we describe the use of neonatal helmet-CPAP in a neonate with persistent pulmonary hypertension of the newborn. Twenty minutes after neonatal helmet-CPAP placement, the baseline post-ductal tcSaO2 (66%) and alveolar-arterial gradient O2 improved from 66% and 648 mmHg to 100% and 465 mmHg, respectively. The neonatal helmet-CPAP was applied for 48 hours and was well-tolerated by the patient without complications. Long-term use of neonatal helmet-CPAP appears feasible and well-tolerated. Comparative trials are needed.

  12. Two denominators for one numerator: the example of neonatal mortality.

    Science.gov (United States)

    Harmon, Quaker E; Basso, Olga; Weinberg, Clarice R; Wilcox, Allen J

    2018-06-01

    Preterm delivery is one of the strongest predictors of neonatal mortality. A given exposure may increase neonatal mortality directly, or indirectly by increasing the risk of preterm birth. Efforts to assess these direct and indirect effects are complicated by the fact that neonatal mortality arises from two distinct denominators (i.e. two risk sets). One risk set comprises fetuses, susceptible to intrauterine pathologies (such as malformations or infection), which can result in neonatal death. The other risk set comprises live births, who (unlike fetuses) are susceptible to problems of immaturity and complications of delivery. In practice, fetal and neonatal sources of neonatal mortality cannot be separated-not only because of incomplete information, but because risks from both sources can act on the same newborn. We use simulations to assess the repercussions of this structural problem. We first construct a scenario in which fetal and neonatal factors contribute separately to neonatal mortality. We introduce an exposure that increases risk of preterm birth (and thus neonatal mortality) without affecting the two baseline sets of neonatal mortality risk. We then calculate the apparent gestational-age-specific mortality for exposed and unexposed newborns, using as the denominator either fetuses or live births at a given gestational age. If conditioning on gestational age successfully blocked the mediating effect of preterm delivery, then exposure would have no effect on gestational-age-specific risk. Instead, we find apparent exposure effects with either denominator. Except for prediction, neither denominator provides a meaningful way to define gestational-age-specific neonatal mortality.

  13. Hypertension in the Neonatal Period: An Update.

    Science.gov (United States)

    Pillai, Anish; Sharma, Deepak; Kadam, Pratichi

    2016-01-01

    There has been a growing interest among neonatologists and paediatricians regarding identification and evaluation of hypertension in the neonatal period. Despite the emergent normative data on blood pressure values in term and preterm neonates over the last two decades, there is still controversy regarding correct definition and classification of hypertension. This article will discuss the current definitions, available normative data and etiology of neonatal hypertension. There is paucity of records in terms of efficacy of antihypertensive drugs in this specific population and management is usually experience based, causing considerable heterogeneity amongst different units. This review article will also cover the evaluation, management, outcomes and follow up of neonatal hypertension with latest advances in this field. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  14. May maternal lifestyle have an impact on neonatal glucose levels?

    Science.gov (United States)

    Hoirisch-Clapauch, Silvia; Porto, Maria Amelia S; Nardi, Antonio E

    2016-02-01

    Neonatal glucose levels correlate negatively with umbilical cord levels of C-peptide, a polypeptide secreted with insulin. In other words, neonatal hypoglycemia results from excessive insulin secretion from fetal/neonatal beta cells. Given that insulin causes fat to be stored rather than to be used for energy, one would expect that chronic hyperinsulinemia would result in large-for-gestational-age neonates. The finding that many small-for-gestational-age neonates have hypoglycemia suggests that the stimulus for insulin production occurs close to delivery. We postulated that a potent stimulation of maternal insulin production close to delivery would also provide a potent stimulus for fetal and neonatal insulin production, causing neonatal hypoglycemia. This study has evaluated 155 mothers with markers of excessive insulin production (such as acanthosis or grade III obesity), or with situations characterized by increased insulin requirements (such as an invasive bacterial infection or use of systemic corticosteroid within a week before delivery; or sedentariness or high-carbohydrate intake within 24h before delivery) and their 158 neonates who were screened for glycemic levels at 1, 2 and 4h after birth. The minimum glucose level was correlated to the maternal parameters, and to classical predictors of neonatal hypoglycemia, such as low-birth weight and preterm delivery. The only independent predictors were sedentariness and high-carbohydrate intake within 24h before delivery. The risk of neonatal hypoglycemia increased five-fold with sedentariness, 11-fold with high-carbohydrate intake, and 329-fold with both risk factors. The risk of neonatal hypoglycemia seems to be highly influenced by maternal lifestyle within 24h before delivery. Controlled randomized trials may help determine whether a controlled carbohydrate diet combined with regular physical activity close to delivery can prevent neonatal hypoglycemia and all its severe complications to the newborn

  15. Neonatal Platelet Transfusions and Future Areas of Research.

    Science.gov (United States)

    Sola-Visner, Martha; Bercovitz, Rachel S

    2016-10-01

    Thrombocytopenia affects approximately one fourth of neonates admitted to neonatal intensive care units, and prophylactic platelet transfusions are commonly administered to reduce bleeding risk. However, there are few evidence-based guidelines to inform clinicians' decision-making process. Developmental differences in hemostasis and differences in underlying disease processes make it difficult to apply platelet transfusion practices from other patient populations to neonates. Thrombocytopenia is a risk factor for common preterm complications such as intraventricular hemorrhage; however, a causal link has not been established, and platelet transfusions have not been shown to reduce risk of developing intraventricular hemorrhage. Platelet count frequently drives the decision of whether to transfuse platelets, although there is little evidence to demonstrate what a safe platelet nadir is in preterm neonates. Current clinical assays of platelet function often require large sample volumes and are not valid in the setting of thrombocytopenia; however, evaluation of platelet function and/or global hemostasis may aid in the identification of neonates who are at the highest risk of bleeding. Although platelets' primary role is in establishing hemostasis, platelets also carry pro- and antiangiogenic factors in their granules. Aberrant angiogenesis underpins common complications of prematurity including intraventricular hemorrhage and retinopathy of prematurity. In addition, platelets play an important role in host immune defenses. Infectious and inflammatory conditions such as sepsis and necrotizing enterocolitis are commonly associated with late-onset thrombocytopenia in neonates. Severity of thrombocytopenia is correlated with mortality risk. The nature of this association is unclear, but preclinical data suggest that thrombocytopenia contributes to mortality rather than simply being a proxy for disease severity. Neonates are a distinct patient population in whom

  16. Normal lactate concentration range in the neonatal brain.

    Science.gov (United States)

    Tomiyasu, Moyoko; Aida, Noriko; Shibasaki, Jun; Tachibana, Yasuhiko; Endo, Mamiko; Nozawa, Kumiko; Shimizu, Eiji; Tsuji, Hiroshi; Obata, Takayuki

    2016-11-01

    Lactate peaks are occasionally observed during in vivo magnetic resonance spectroscopy (MRS) scans of the neonatal brain, even in healthy patients. The purpose of this study was to investigate the normal range of neonatal brain lactate concentration, as a definitive normal range would be clinically valuable. Using a clinical 3T scanner (echo/repetition times, 30/5000ms), single-voxel MRS data were obtained from the basal ganglia (BG) and centrum semiovale (CS) in 48 healthy neonates (postconceptional age (PCA), 30-43weeks), nine infants (age, 1-12months old), and 20 children (age, 4-15years). Lactate concentrations were calculated using an MRS signal quantification program, LCModel. Correlations between regional lactate concentration and PCA (neonates), or age (all subjects) were investigated. Absolute lactate concentrations of the BG and CS were as follows: neonates, 0.77mM (0-2.02) [median (range)] and 0.77 (0-1.42), respectively; infants, 0.38 (0-0.79) and 0.49 (0.17-1.17); and children, 0.17 (0-0.76) and 0.22 (0-0.80). Overall, subjects' lactate concentrations decreased significantly with age (Spearman: BG, n=61, ρ=-0.38, p=0.003; CS, n=68, ρ=-0.57, p<0.001). However, during the neonatal period no correlations were detected between lactate concentration in either region and PCA. We determined normal ranges of neonatal lactate concentration, which may prove useful for diagnostic purposes. Further studies regarding changes in brain lactate concentration during development would help clarify the reasons for higher concentrations observed during the neonatal period, and contribute to improvements in diagnoses. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Sonographic features of neonatal mastitis and breast abscess.

    Science.gov (United States)

    Borders, Heather; Mychaliska, George; Gebarski, K Stiennon

    2009-09-01

    Neonatal mastitis and neonatal breast abscess are uncommon. Although well described in the pediatric and surgical literature, there is a paucity of reports describing their sonographic features. To describe and illustrate the sonographic features of neonatal mastitis and neonatal breast abscess. We reviewed the medical database of a large children's health-care center from 2000 through 2008 for patients presenting in the first 8 weeks of life with mastitis. The findings were correlated with clinical presentation and course, laboratory findings and clinical outcome. Four neonates (three girls and one boy) presented with mastitis. They all had prominent breast buds on the affected side with poorly defined margins, slightly more echogenic focally or diffusely compared to normal with hyperemia on color flow Doppler US. The surrounding subcutaneous tissue was thick and echogenic. Two abscesses presented as avascular areas without color flow on Doppler US, subtly increased through-transmission and surrounding hyperemia. One abscess was of increased echogenicity while the other was anechoic. Neonatal mastitis and breast abscess are unusual diseases that should be appropriately treated with antibiotics and drainage to avoid generalized sepsis, breast hypoplasia, and scarring. US is useful in distinguishing mastitis from breast abscess and guiding treatment options.

  18. Perinatal transport: problems in neonatal intensive care capacity.

    Science.gov (United States)

    Gill, A B; Bottomley, L; Chatfield, S; Wood, C

    2004-05-01

    To assess the quantity and nature of transfers within the Yorkshire perinatal service, with the aim of identifying suitable outcome measures for the assessment of future service improvements. Collection of data on perinatal transfers from all neonatal and maternity units located in the Yorkshire region of the United Kingdom from May to November 2000. Expectant mothers (in utero transfers) and neonates (ex utero transfers). None Quantification of in utero and ex utero transfers; the reasons for and resources required to support transfers; the nature of each transfer (acute, specialist, non-acute, into or out of region). In the period studied, there were 800 transfers (337 in utero; 463 ex utero); 306 transfers were "acute" (80% of transfers in utero), 214 because of specialist need, and 280 "non-acute". Some 37% of capacity transfers occurred from the two level 3 units in the region. Of 254 transfers out of the 14 neonatal units for intensive care, 44 (17.3%) were transferred to hospitals outside the normal neonatal commissioning boundaries. The study highlights a continuing apparent lack of capacity within the neonatal service in the Yorkshire region, resulting in considerable numbers of neonatal and maternal transfers.

  19. Pharmacokinetics of cephalosporins in the neonate: a review

    Directory of Open Access Journals (Sweden)

    Gian Maria Pacifici

    2011-01-01

    Full Text Available The aim of this work was to review the published data on the pharmacokinetics of cephalosporins in neonates to provide a critical analysis of the literature as a useful tool for physicians. The bibliographic search was performed for articles published up to December 3, 2010, using PubMed. In addition, the book Neofax: A Manual of Drugs Used in Neonatal Care by Young and Mangum was consulted. The cephalosporins are mainly eliminated by the kidneys, and their elimination rates are reduced at birth. As a consequence, clearance is reduced and t1/2 is more prolonged in the neonate than in more mature infants. The neonate's substantial body water content creates a large volume of distribution (Vd of cephalosporins, as these drugs are fairly water soluble. Postnatal development is an important factor in the maturation of the neonate, and as postnatal age proceeds, the clearance of cephalosporins increases. The maturation of the kidney governs the pharmacokinetics of cephalosporins in the infant. Clearance and t1/2 are influenced by development, and this must be taken into consideration when planning a cephalosporin dosage regimen for the neonate.

  20. Neonatal malaria complicated by hypoglycaemia and ...

    African Journals Online (AJOL)

    There is no established and widely accepted guidelines for clinical management of severe neonatal malaria. The aim of this paper is to raise the alertness of physicians regarding the occurrence of severe malaria in the neonatal period and to describe the treatment modality we adopted (in the absence of an internationally ...

  1. Cost Analysis of Treating Neonatal Hypoglycemia with Dextrose Gel.

    Science.gov (United States)

    Glasgow, Matthew J; Harding, Jane E; Edlin, Richard

    2018-04-03

    To evaluate the costs of using dextrose gel as a primary treatment for neonatal hypoglycemia in the first 48 hours after birth compared with standard care. We used a decision tree to model overall costs, including those specific to hypoglycemia monitoring and treatment and those related to the infant's length of stay in the postnatal ward or neonatal intensive care unit, comparing the use of dextrose gel for treatment of neonatal hypoglycemia with placebo, using data from the Sugar Babies randomized trial. Sensitivity analyses assessed the impact of dextrose gel cost, neonatal intensive care cost, cesarean delivery rate, and costs of glucose monitoring. In the primary analysis, treating neonatal hypoglycemia using dextrose gel had an overall cost of NZ$6863.81 and standard care (placebo) cost NZ$8178.25; a saving of NZ$1314.44 per infant treated. Sensitivity analyses showed that dextrose gel remained cost saving with wide variations in dextrose gel costs, neonatal intensive care unit costs, cesarean delivery rates, and costs of monitoring. Use of buccal dextrose gel reduces hospital costs for management of neonatal hypoglycemia. Because it is also noninvasive, well tolerated, safe, and associated with improved breastfeeding, buccal dextrose gel should be routinely used for initial treatment of neonatal hypoglycemia. Australian New Zealand Clinical Trials Registry: ACTRN12608000623392. Copyright © 2018 Elsevier Inc. All rights reserved.

  2. Fellowship Training in the Emerging Fields of Fetal-Neonatal Neurology and Neonatal Neurocritical Care.

    Science.gov (United States)

    Smyser, Christopher D; Tam, Emily W Y; Chang, Taeun; Soul, Janet S; Miller, Steven P; Glass, Hannah C

    2016-10-01

    Neonatal neurocritical care is a growing and rapidly evolving medical subspecialty, with increasing numbers of dedicated multidisciplinary clinical, educational, and research programs established at academic institutions. The growth of these programs has provided trainees in neurology, neonatology, and pediatrics with increased exposure to the field, sparking interest in dedicated fellowship training in fetal-neonatal neurology. To meet this rising demand, increasing numbers of training programs are being established to provide trainees with the requisite knowledge and skills to independently deliver care for infants with neurological injury or impairment from the fetal care center and neonatal intensive care unit to the outpatient clinic. This article provides an initial framework for standardization of training across these programs. Recommendations include goals and objectives for training in the field; core areas where clinical competency must be demonstrated; training activities and neuroimaging and neurodiagnostic modalities which require proficiency; and programmatic requirements necessary to support a comprehensive and well-rounded training program. With consistent implementation, the proposed model has the potential to establish recognized standards of professional excellence for training in the field, provide a pathway toward Accreditation Council for Graduate Medical Education certification for program graduates, and lead to continued improvements in medical and neurological care provided to patients in the neonatal intensive care unit. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Two Neonates with Congenital Hydrocolpos

    Directory of Open Access Journals (Sweden)

    Vydehi Murthy

    2013-01-01

    Full Text Available Introduction. Neonatal hydrocolpos is a rare condition. Hydrocolpos is cystic dilatation of the vagina with fluid accumulation due to a combination of stimulation of secretary glands of the reproductive tract and vaginal obstruction. The differential for a neonatal presentation of lower abdominal mass includes urogenital anomalies, Hirschsprung’s, disease or sacrococcygeal teratoma. Prenatal diagnosis and early newborn imaging studies leads to early detection and treatment of these cases. Case. We report here two cases of neonatal hydrocolpos with prenatal diagnosis of lower abdominal mass. Postnatally, ultrasound, MRI imaging, and cystoscopy confirmed large cystic mass as hydrocolpos with distal vaginal obstruction. Both patients had enlarged renal system secondary to mass effect. Conclusion. High index of suspicion for hydrocolpos in a newborn presenting with fetal diagnosis of infraumbilical abdominal mass will facilitate timely intervention and prevention of complications.

  4. [Spectrum of congenital malformations observed in neonates of consanguineous parents].

    Science.gov (United States)

    Pinto Escalante, D; Castillo Zapata, I; Ruiz Allec, D; Ceballos Quintal, J M

    2006-01-01

    Consanguineous unions occur in all populations around the world. Couples related as second cousins or closer have been observed with deleterious effect. Among the clinical effects of parental consanguinity, the incidence of offspring with congenital malformations (CM) increases approximately two-fold. A hospital database of neonates with CM was searched to select neonates with parental consanguinity and two control groups. One control group consisted of healthy neonates and the other control group consisted of neonates with CM but without parental consanguinity. Both control groups consisted of the first neonate of the same sex to be born after a consanguineous neonate with CM. Family, sociodemographic and anthropometric variables, as well as the severity of the malformations, were compared between the two groups with CM. Neonates with CM were grouped into five categories: Major multiple CM, minor multiple CM, isolated major CM, isolated minor CM, and specific diseases. The indigenous Mayan subpopulation was also analyzed. Among 1117 neonates with CM, parental consanguinity was found in 21. Parental consanguinity was also found in 8 neonates in the group of healthy controls (OR 2.4 [1.05-5.95]). The most common form of consanguinity was between second cousins and was more frequent in the Mayan subpopulation. Major multiple CM were more frequent among consanguineous than among nonconsanguineous couples. No association was found between the severity of CM and the degree of relationship. The prevalence of consanguinity found in neonates with CM and healthy controls (1.9 % and 0.8 %) was similar to that found in other Latin populations. A higher prevalence was found in the Mayan population. Mayor multiple CM were more frequent among the neonates of consanguineous than among nonconsanguineous couples.

  5. Neonatal hypoglycaemia: learning from claims

    OpenAIRE

    Hawdon, Jane M; Beer, Jeanette; Sharp, Deborah; Upton, Michele

    2016-01-01

    Objectives Neonatal hypoglycaemia is a potential cause of neonatal morbidity, and on rare but tragic occasions causes long-term neurodevelopmental harm with consequent emotional and practical costs for the family. The organisational cost to the NHS includes the cost of successful litigation claims. The purpose of the review was to identify themes that could alert clinicians to common pitfalls and thus improve patient safety. Design The NHS Litigation Authority (NHS LA) Claims Management Syste...

  6. Preeclampsia; short and long-term consequences for mother and neonate.

    Science.gov (United States)

    Bokslag, Anouk; van Weissenbruch, Mirjam; Mol, Ben Willem; de Groot, Christianne J M

    2016-11-01

    Preeclampsia is a common pregnancy specific disease, that presents with hypertension and a variety of organ failures, including malfunction of kidneys, liver and lungs. At present, the only definitive treatment of preeclampsia is end the pregnancy and deliver the neonate and placenta. For women with mild preeclampsia in the preterm phase of pregnancy, expectant management is generally indicated to improve fetal maturity, often requiring maternal medical treatment. Last decades, more evidence is available that the underlying mechanism of preeclampsia, endothelial disease, is not limited to pregnancy but increases cardiovascular risk in later life. In this review, we present the most recent insight in preeclampsia with focus on impact on the fetus, short and long-term outcome of offspring's, and long-term outcome of women with a history of preeclampsia. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  7. Effects of maternal hypertension on the neonatal haemogram in ...

    African Journals Online (AJOL)

    Haematocrit and white blood cell differentials were done manually. Data were analysed using SPSS version 16. Results A total of 200 neonates were recruited, comprising 100 neonates of mothers with hypertensive disorders of pregnancy and 100 neonates of normotensive mothers. The mean haematocrit was significantly ...

  8. Causes and Clinical Outcomes in Neonates with Acute Abdomen ...

    African Journals Online (AJOL)

    Causes and Clinical Outcomes in Neonates with Acute Abdomen Requiring Surgery at ... Neonatal surgery is challenging, particularly in the emergency setting whereby ... Other causes included peritonitis (6.9%) and abdominal wall defects (4.7%). ... accounting for 13 cases (30.2%) and mortality was 34.9 % ( 15 neonates).

  9. Severe hyperglycaemia due to neonatal sepsis - A case report ...

    African Journals Online (AJOL)

    Neonatal sepsis is a clinical syndrome characterized by signs and symptoms of infection with or without accompanying bacteremia in the first month of life. The clinical signs of neonatal sepsis are neither specific nor uniform. Neonatal sepsis may present with fever, hypotonia, respiratory distress, apnea and hyperglycaemia.

  10. Neonatal Jaundice

    DEFF Research Database (Denmark)

    Maimburg, Rikke Damkjær; Væth, Michael; Schendel, Diana

    2008-01-01

    In a previous study, we found that infants transferred to a neonatal ward after delivery had an almost twofold increased risk of being diagnosed with infantile autism later in childhood in spite of extensive controlling of obstetric risk factors. We therefore decided to investigate other reasons ...

  11. Predictors of early neonatal mortality at a neonatal intensive care ...

    African Journals Online (AJOL)

    admin

    mortality and they have been the reasons for the wide variation in mortality rates among the health facilities reporting. Objective: ... A study in Indonesia about determinants of neonatal ..... antenatal visit, frequency of visits and administration of.

  12. Design of a Functional Training Prototype for Neonatal Resuscitation

    Directory of Open Access Journals (Sweden)

    Sivaramakrishnan Rajaraman

    2014-11-01

    Full Text Available Birth Asphyxia is considered to be one of the leading causes of neonatal mortality around the world. Asphyxiated neonates require skilled resuscitation to survive the neonatal period. The project aims to train health professionals in a basic newborn care using a prototype with an ultimate objective to have one person at every delivery trained in neonatal resuscitation. This prototype will be a user-friendly device with which one can get trained in performing neonatal resuscitation in resource-limited settings. The prototype consists of a Force Sensing Resistor (FSR that measures the pressure applied and is interfaced with Arduino® which controls the Liquid Crystal Display (LCD and Light Emitting Diode (LED indication for pressure and compression counts. With the increase in population and absence of proper medical care, the need for neonatal resuscitation program is not well addressed. The proposed work aims at offering a promising solution for training health care individuals on resuscitating newborn babies under low resource settings.

  13. Neonatal morbidity associated with shoulder dystocia maneuvers.

    Science.gov (United States)

    Spain, Janine E; Frey, Heather A; Tuuli, Methodius G; Colvin, Ryan; Macones, George A; Cahill, Alison G

    2015-03-01

    We sought to examine neonatal morbidity associated with different maneuvers used among term patients who experience a shoulder dystocia. We conducted a retrospective cohort study of all women who experienced a clinically diagnosed shoulder dystocia at term requiring obstetric maneuvers at a single tertiary care hospital from 2005 through 2008. We excluded women with major fetal anomaly, intrauterine death, multiple gestation, and preterm. Women exposed to Rubin maneuver, Wood's screw maneuver, or delivery of the posterior arm were compared to women delivered by McRoberts/suprapubic pressure only, which served as the reference group. The primary outcome was a composite morbidity of neonatal injury (defined as clavicular or humeral fracture or brachial plexus injury) and neonatal depression (defined as Apgar dystocia, defined as time from delivery of fetal head to delivery of shoulders. Among the 231 women who met inclusion criteria, 135 were delivered by McRoberts/suprapubic pressure alone (57.9%), 83 women were exposed to Rubin maneuver, 53 women were exposed to Wood's screw, and 36 women were exposed to delivery of posterior arm. Individual maneuvers were not associated with composite morbidity, neonatal injury, or neonatal depression after adjusting for nulliparity and duration of shoulder dystocia. We found no association between shoulder dystocia maneuvers and neonatal morbidity after adjusting for duration, a surrogate for severity. Our results demonstrate that clinicians should utilize the maneuver most likely to result in successful delivery. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Serum zinc status of neonates with seizure

    Directory of Open Access Journals (Sweden)

    Olia Sharmeen

    2016-08-01

    Full Text Available Background: Seizure is a common neurological disorder in neonatal age group!. Primary metabolic derangement is one of the important reason behind this convulsion during this period. Among primary metabolic derangement hypoglycemia, is most common followed by bypocalcaemia, hypomagnesaemia, low zinc status etc. As causes of many cases of convul­sion remain unknown in neonate. Objectives: To see the zinc status in the sera of neonate with convulsion. So that if needed early intervention can be taken up and thereby prevent complications. Method: A total of 50 neonates (1-28 days who had convulsion with no apparent reasons of convulsion were enrolled as cases and 50 healthy age and sex matched neonates were enrolled as controls. After a quick clinical evaluation serum zinc status was estimated from venous blood by atomic absorption method in Chemistry Division, Atomic Energy Centre. Low zinc was considered if serum value was <0.7mg/L. Results: Among a total of 50 cases 6% had low zinc value & 2% of controls also had low zinc level. The mean serwu zinc level of cases and controls were 1.57±0.95 and 2.37±1.06 mmol/1 respectively (p<0.01. Conclusion: From the study it is seen that low zinc value is an important cause of neonatal seizure due to primary metabolic abnormalities. So early recognition and treatment could save these babies from long term neurological sequelies.

  15. Risk factors for hearing loss in neonates

    Directory of Open Access Journals (Sweden)

    Ni Luh Putu Maharani

    2016-11-01

    Full Text Available Background An estimated 6 of 1,000 children with live births suffer from permanent hearing loss at birth or the neonatal period. At least 90% of cases occur in developing countries. Hearing loss should be diagnosed as early as possible so that intervention can be done before the age of 6 months. Objective To determine risk factors for hearing loss in neonates. Methods We performed a case-control study involving 100 neonates with and without hearing loss who were born at Sanglah Hospital, Denpasar from November 2012 to February 2013. Subjects were consisted of 2 groups, those with hearing loss (case group of 50 subjects and without hearing loss (control group of 50 subjects. The groups were matched for gender and birth weight. We assessed the following risk factors for hearing loss: severe neonatal asphyxia, hyperbilirubinemia, meningitis, history of aminoglycoside therapy, and mechanical ventilation by Chi-square analysis. The results were presented as odds ratio and its corresponding 95% confidence intervals. Results Seventy percent of neonates with hearing loss had history of aminoglycoside therapy. Multivariable analysis revealed that aminoglycoside therapy of 14 days or more was a significant risk factor for hearing loss (OR 2.7; 95%CI 1.1 to 6.8; P=0.040. There were no statistically significant associations between hearing loss and severe asphyxia, hyperbilirubinemia, meningitis, or mechanical ventilation. Conclusion Aminoglycoside therapy for >=14 days was identified as a risk factor for hearing loss in neonates.

  16. Altered surfactant homeostasis and recurrent respiratory failure secondary to TTF-1 nuclear targeting defect.

    Science.gov (United States)

    Peca, Donatella; Petrini, Stefania; Tzialla, Chryssoula; Boldrini, Renata; Morini, Francesco; Stronati, Mauro; Carnielli, Virgilio P; Cogo, Paola E; Danhaive, Olivier

    2011-08-25

    Mutations of genes affecting surfactant homeostasis, such as SFTPB, SFTPC and ABCA3, lead to diffuse lung disease in neonates and children. Haploinsufficiency of NKX2.1, the gene encoding the thyroid transcription factor-1 (TTF-1)--critical for lung, thyroid and central nervous system morphogenesis and function--causes a rare form of progressive respiratory failure designated brain-lung-thyroid syndrome. Molecular mechanisms involved in this syndrome are heterogeneous and poorly explored. We report a novel TTF-1 molecular defect causing recurrent respiratory failure episodes in an infant. The subject was an infant with severe neonatal respiratory distress syndrome followed by recurrent respiratory failure episodes, hypopituitarism and neurological abnormalities. Lung histology and ultrastructure were assessed by surgical biopsy. Surfactant-related genes were studied by direct genomic DNA sequencing and array chromatine genomic hybridization (aCGH). Surfactant protein expression in lung tissue was analyzed by confocal immunofluorescence microscopy. For kinetics studies, surfactant protein B and disaturated phosphatidylcholine (DSPC) were isolated from serial tracheal aspirates after intravenous administration of stable isotope-labeled (2)H(2)O and (13)C-leucine; fractional synthetic rate was derived from gas chromatography/mass spectrometry (2)H and (13)C enrichment curves. Six intubated infants with no primary lung disease were used as controls. Lung biopsy showed desquamative interstitial pneumonitis and lamellar body abnormalities suggestive of genetic surfactant deficiency. Genetic studies identified a heterozygous ABCA3 mutation, L941P, previously unreported. No SFTPB, SFTPC or NKX2.1 mutations or deletions were found. However, immunofluorescence studies showed TTF-1 prevalently expressed in type II cell cytoplasm instead of nucleus, indicating defective nuclear targeting. This pattern has not been reported in human and was not found in two healthy controls and

  17. Altered surfactant homeostasis and recurrent respiratory failure secondary to TTF-1 nuclear targeting defect

    Directory of Open Access Journals (Sweden)

    Carnielli Virgilio P

    2011-08-01

    Full Text Available Abstract Background Mutations of genes affecting surfactant homeostasis, such as SFTPB, SFTPC and ABCA3, lead to diffuse lung disease in neonates and children. Haploinsufficiency of NKX2.1, the gene encoding the thyroid transcription factor-1 (TTF-1 - critical for lung, thyroid and central nervous system morphogenesis and function - causes a rare form of progressive respiratory failure designated brain-lung-thyroid syndrome. Molecular mechanisms involved in this syndrome are heterogeneous and poorly explored. We report a novel TTF-1 molecular defect causing recurrent respiratory failure episodes in an infant. Methods The subject was an infant with severe neonatal respiratory distress syndrome followed by recurrent respiratory failure episodes, hypopituitarism and neurological abnormalities. Lung histology and ultrastructure were assessed by surgical biopsy. Surfactant-related genes were studied by direct genomic DNA sequencing and array chromatine genomic hybridization (aCGH. Surfactant protein expression in lung tissue was analyzed by confocal immunofluorescence microscopy. For kinetics studies, surfactant protein B and disaturated phosphatidylcholine (DSPC were isolated from serial tracheal aspirates after intravenous administration of stable isotope-labeled 2H2O and 13C-leucine; fractional synthetic rate was derived from gas chromatography/mass spectrometry 2H and 13C enrichment curves. Six intubated infants with no primary lung disease were used as controls. Results Lung biopsy showed desquamative interstitial pneumonitis and lamellar body abnormalities suggestive of genetic surfactant deficiency. Genetic studies identified a heterozygous ABCA3 mutation, L941P, previously unreported. No SFTPB, SFTPC or NKX2.1 mutations or deletions were found. However, immunofluorescence studies showed TTF-1 prevalently expressed in type II cell cytoplasm instead of nucleus, indicating defective nuclear targeting. This pattern has not been reported in human

  18. Opiate v CNS depressant therapy in neonatal drug abstinence syndrome.

    Science.gov (United States)

    Kandall, S R; Doberczak, T M; Mauer, K R; Strashun, R H; Korts, D C

    1983-04-01

    Paregoric and phenobarbital, administered randomly in 153 passively addicted neonates, initially appeared to control neonatal abstinence signs equally well. However, seven of the 62 phenobarbital-treated newborns had abstinence-associated seizures within the first month of life, while none of 49 paregoric-treated neonates had seizures. Forty-two neonates initially requiring no specific pharmacotherapy for abstinence signs were born to mothers taking less methadone hydrochloride just before delivery. Five of those 42 neonates, however, had seizures within the first 14 days of life. Seizure occurrence could not be predicted from analysis of early abstinence patterns. We consider paregoric to be the treatment of choice for the neonatal abstinence syndrome. Phenobarbital use should be monitored with serum drug levels and modification of recommended dosage regimens considered.

  19. Fetal neonatal hyperthyroidism: diagnostic and therapeutic approachment

    Science.gov (United States)

    Kurtoğlu, Selim; Özdemir, Ahmet

    2017-01-01

    Fetal and neonatal hyperthyroidism may occur in mothers with Graves’ disease. Fetal thyrotoxicosis manifestation is observed with the transition of TSH receptor stimulating antibodies to the fetus from the 17th–20th weeks of pregnancy and with the fetal TSH receptors becoming responsive after 20 weeks. The diagnosis is confirmed by fetal tachycardia, goiter and bone age advancement in pregnancy and maternal treatment is conducted in accordance. The probability of neonatal hyperthyroidism is high in the babies of mothers that have ongoing antithyroid requirement and higher antibody levels in the last months of pregnancy. Clinical manifestation may be delayed by 7–17 days because of the antithyroid drugs taken by the mother. Neonatal hyperthyroidism symptoms can be confused with sepsis and congenital viral infections. Herein, the diagnosis and therapeutic approach are reviewed in cases of fetal neonatal hyperthyroidism. PMID:28439194

  20. Colonization with hospital flora and its associated risk factors in neonates hospitalized in neonatal ward of a teaching center in Isfahan, Iran.

    Science.gov (United States)

    Shirani, Kiana; Mostafavizadeh, Kamyar; Ataei, Behrouz; Akhani, Khatere

    2018-01-01

    Due to the high incidence and prevalence of infection in neonatal ward, especially Neonatal Intensive Care Units (NICUs) reported by different studies and the important role of colonization with hospital germs in the development of nosocomial infections, we intended to evaluate the risk of colonization with hospital germs in neonates and its associated risk factors. This cross-sectional, descriptive-analytical study was conducted in 2016 in a Teaching Center in Iran. In total, 51 neonates were selected based on the inclusion criteria, and after recording their information in a checklist, samples were taken by swab from outer ear, axilla, and groin for culture. Neonates with negative culture from mentioned regions were enrolled in the study. The swab samples again were taken and sent for culture from mentioned regions in at least 3 days after hospitalization. Culture results from first and second sampling were collected and analyzed statistically. This study was conducted on 51 neonates. The mean gestational age among the neonates ranged from 35.25 (Week) ± 2.98. 22 girls (43.1%) and 29 boys (56.9%), most of them were born by cesarean. Based on the results of logistic regression, a significant association was found between the occurrence of colonization of hospital flora and the place hospitalization of the newborns (odds ratio (OR): 4.750; 95% confidence interval (CI): 1.26-17.85). This study revealed that the only risk factors of colonization with hospital flora in neonates are the type of delivery and place of hospitalization. Based on findings of the study, it is recommended to focus on efforts in increasing the rate of natural birth as well as improving conditions of infection control in NICUs to reduce the number of incidences of colonization with hospital flora in neonates.

  1. Severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia.

    Science.gov (United States)

    Scott, Angela; Glover, Jason; Skoda-Smith, Suzanne; Torgerson, Troy R; Xu, Min; Burroughs, Lauri M; Woolfrey, Ann E; Fleming, Mark D; Shimamura, Akiko

    2015-11-01

    Aplastic anemia in the neonate is rare. We report a case of severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia. This report highlights the importance of considering SCID early in the evaluation of neonatal aplastic anemia prior to the development of infectious complications. © 2015 Wiley Periodicals, Inc.

  2. Selected Abstracts of the 2nd Congress of joint European Neonatal Societies (jENS 2017; Venice (Italy; October 31-November 4, 2017; Session "Neonatal Infectious Diseases/Immunology"

    Directory of Open Access Journals (Sweden)

    --- Various Authors

    2017-10-01

    és López, M.J. Cobas Pazos, Y. Lopez Lozano, M.D. Sanchez-Redondo Sanchez-Gabriel, A. Martinez GimenoABS 29. NEONATAL SEPSIS: FREQUENCY, RISK FACTORS AND OUTCOME IN A LEVEL II NICU UNIT • L. Blaga, M. Matyas, C. Vidra, M. Hasmasanu, G. Abrudan, G. ZaharieABS 30. LUMBAR PUNCTURE SUCCESS & FAILURE IN A TERTIARY NEONATAL UNIT – A PROSPECTIVE COHORT STUDY • A. Marshall, M. Sadarangani, C. Roehr, M. AnthonyABS 31. THE EARLY ONSET RISK CALCULATOR TO EVALUATE EARLY-ONSET SEPSIS IN INFANTS BORN ≥ 35 WEEKS GESTATION  • S. Stokes, R. Cuzzilla, AJ. Daley, SE. JacobsABS 32. HOSPITALISATION FOR RESPIRATORY SYNCYTIAL VIRUS (RSV INFECTION IN PREMATURE INFANTS BORN IN MULTIPLE BIRTHS • R. Thwaites, S. Buchan, C. Morris, B. Rodgers-Gray, J. Fullarton, J. CouttsABS 33. ANTIBIOTICS FOR EARLY ONSET NEONATAL SEPSIS: AUDITING PRACTICE AND ANALYSING COST • O. Kazeem, M. Saunders, R. Roy, C. TremlettABS 34. DYSFUNCTIONAL INFLAMMATION; THE SIGNIFICANCE OF TLR2 SIGNALING IN HYPOXIC NEONATES PREDISPOSED TO NEURAL ENCEPHALOPATHY • B. Wright, K. Lynne, M. Eleanor, M. Omer, N. A Taher, M. O’Dea, A. Melorz, A. MorenoABS 35. NEONATAL THYMIC CHARACTERISTICS IN A RAT MODEL OF INTRAUTERINE GROWTH RESTRICTION • J.B. Armengaud, K. Nardou, F. Pre-Curto, F. Candotti, F. Angelini, M. Hofer, U. Simeoni, C. YzydorczykABS 36. IMPACT OF MICROBIAL-ASSOCIATED INTRA-AMNIOTIC INFLAMMATION AND “STERILE” INTRA-AMNIOTIC INFLAMMATION ON SHORT-TERM NEONATAL OUTCOME IN WOMEN WITH PRETERM LABOR AND INTACT MEMBRANES • A. Herranz Barbero, R. Amoedo Blanco, M. Izquierdo Renau, C. Murillo, C. Rueda, J. Ponce, T. CoboABS 37. VARIABILITY IN IMMUNOLOGICAL RESPONSE TO OROPHARYNGEAL COLOSTRUM ADMINISTRATION IN PRETERM NEWBORN BASED ON SEX • E. Martin-Alvarez, L. Serrano-Lopez, J. Díaz-Castro, M. Peña-Caballero, M.B. Sánchez-Martínez, F. Martín-Peregrina, J.J. Ochoa, J.A. Hurtado-SuazoABS 38. PCT, IL-6 AND IL-8, BUT NOT HA ARE USEFUL FOR DIAGNOSTIC UTILITY IN NEONATES AT RISK THAT FULFILL THE

  3. Pattern of neonatal septicemia in a Malaysian maternity hospital.

    Science.gov (United States)

    Boo, N Y; Wong, Y H; Lim, V K

    1989-09-01

    Over a 12 months period, out of 25,411 livebirths, 155 neonates (6.1 per 1000 livebirths) had proven septicemia by blood culture. The mortality rate was 26.5%. Septicemia was more common among the very low birthweight and preterm neonates of gestation of 30 weeks or less. 45.8% of the septicemia occurred during the first 48 hours of life. Staphylococcus epidermidis was the most common causative organism. However, mortality was highest among neonates who acquired multiresistant nosocomial infection during the later part of neonatal life.

  4. Neonatal hypocalcemia and its relation to vitamin D and calcium supplementation.

    Science.gov (United States)

    Elsary, Asmaa Y; Elgameel, Alkassem A; Mohammed, Wael S; Zaki, Osman M; Taha, Shaimaa A

    2018-03-01

    To assess the prevalence of hypocalcemia in outpatient clinic neonates and its relation to vitamin D and calcium supplementation.  Methods: This cross-sectional analytical study was conducted at the University Teaching Hospital from May to October 2016. Data were collected from 100 neonates by interviewing mothers using a structured questionnaire; which included socio-demographic information, maternal and neonatal history; in addition to investigations of serum calcium total and ionized and serum vitamin D level. Results: The prevalence of hypocalcemia was 76%, late hypocalcemia represent 52% of hypocalcemic neonates. The prevalence of hypovitaminosis D was 38%. Hypocalcemia was found more prevalent among neonates with no history of vitamin D supplementation (98.7%), no history of maternal calcium supplementation (57.9%), while they had a history of neonatal jaundice on phototherapy (46.1%) which increased to 53.8% with late hypocalcemia. Conclusion: Neonatal hypocalcemia is widely prevalent in Fayoum governorate with significant association with a history of neonatal jaundice on phototherapy, not receiving maternal calcium or neonatal vitamin D supplementation.

  5. Palliative care in the neonatal unit: neonatal nursing staff perceptions of facilitators and barriers in a regional tertiary nursery.

    Science.gov (United States)

    Kilcullen, Meegan; Ireland, Susan

    2017-05-11

    Neonatology has made significant advances in the last 30 years. Despite the advances in treatments, not all neonates survive and a palliative care model is required within the neonatal context. Previous research has focused on the barriers of palliative care provision. A holistic approach to enhancing palliative care provision should include identifying both facilitators and barriers. A strengths-based approach would allow barriers to be addressed while also enhancing facilitators. The current study qualitatively explored perceptions of neonatal nurses about facilitators and barriers to delivery of palliative care and also the impact of the regional location of the unit. The study was conducted at the Townsville Hospital, which is the only regional tertiary neonatal unit in Australia. Semi-structured interviews were conducted with a purposive sample of eight neonatal nurses. Thematic analysis of the data was conducted within a phenomenological framework. Six themes emerged regarding family support and staff factors that were perceived to support the provision of palliative care of a high quality. Staff factors included leadership, clinical knowledge, and morals, values, and beliefs. Family support factors included emotional support, communication, and practices within the unit. Five themes emerged from the data that were perceived to be barriers to providing quality palliative care. Staff perceived education, lack of privacy, isolation, staff characteristics and systemic (policy, and procedure) factors to impact upon palliative care provision. The regional location of the unit also presented unique facilitators and barriers to care. This study identified and explored facilitators and barriers in the delivery of quality palliative care for neonates in a regional tertiary setting. Themes identified suggested that a strengths-approach, which engages and amplifies facilitating factors while identified barriers are addressed or minimized, would be successful in

  6. Colestase neonatal prolongada: estudo prospectivo

    Directory of Open Access Journals (Sweden)

    PRADO Elizabeth Teixeira Mendes Livramento

    1999-01-01

    Full Text Available Em razão da urgência de se decidir por um tratamento clínico ou por uma intervenção cirúrgica imediata, o estudo da colestase neonatal prolongada envolve dois objetivos básicos: o diagnóstico diferencial entre atresia biliar e hepatite neonatal e a pesquisa dos agentes etiológicos associados. Desta maneira, através de estudo prospectivo desenvolvido na década de 1970, foram avaliadas 77 crianças portadoras de colestase neonatal prolongada para estabelecer o diagnóstico diferencial entre atresia biliar e hepatite neonatal e, numa segunda fase, 108 crianças, visando esclarecer a etiopatogenia da colestase neonatal prolongada. Os resultados do diagnóstico diferencial revelaram que, dos 18 atributos avaliados, apenas oito mostraram-se bons indicadores de atresia biliar, em ordem decrescente: ductos proliferados (espaço-porta, fibrose (espaço-porta, colestase (espaço-porta, cor das fezes -- acolia, hepatomegalia, colestase canalicular (lóbulo, infiltrado (espaço-porta, células gigantes (lóbulo. Estes oito atributos foram então compostos, mediante uma ponderação, em um único indicador de grande poder discriminativo, capaz de decidir o diagnóstico diferencial em 99% dos casos. Quanto à etiopatogenia, registrou-se: vírus rubéola 0%, vírus herpes simples 0%, listeriose 0%, citomegalovirose 2,2%, vírus hepatite B 2,4%, toxoplasmose 2,8%, deficiência de alfa-1-antitripsina 13,1%, sífilis 21,1 %, auto-anticorpos hepáticos 58,4%. O trabalho desenvolvido mostra que as 8 variáveis mais decisivas, como indicadoras diferenciais entre atresia biliar e hepatite neonatal, permanecem como índices fundamentais, auxiliando, em conjunto com novos métodos diagnósticos, na composição de uma estratégia multifatorial cada vez menos invasiva e mais precisa. O estudo da etiopatogenia, dependente das condições epidemiológicas locais e da época, com a introdução de novos métodos diagnósticos, torna-se atualmente cada vez mais

  7. Frequency of neonatal abstinence syndrome (NAS and type of the narcotic substance in neonates born from drug addicted mothers

    Directory of Open Access Journals (Sweden)

    Fatemeh Nayeri

    2015-02-01

    Full Text Available Abstract Background and objective: NAS is a combination of signs and symptoms that due to physical and mental dependency, develops in neonates born from drug addicted mothers. The onset of NAS varies in accordance with the type, amount, frequency and duration of substance used. Because of diverse and unclear pattern of substance abuse in Iranian addicted pregnant mothers in comparison with western countries, this multi-center study has been designed to evaluate NAS in neonates born from drug addicted mothers. Material and method: A cross sectional study was carried out on newborns of narcotic addicted mothers during the first six months of 2008. The newborn’s status and clinical signs were checked by physical examination and scored by the Finnegan scoring system. Results: In this study 100 neonates born from narcotic addicted mothers were examined; the most used narcotic was crack (36%. 60% of neonates showed signs of NAS. The most prevalent signs of NAS were increased muscle tonicity (60%/7, irritability (59%/6 and increased moro reflex (51%/8. Neonates born from crack abusers, in comparison with other drugs, were significantly at risk of NAS (100% vs.87%, p

  8. Development and validation of Neonatal Satisfaction Survey--NSS-13.

    Science.gov (United States)

    Hagen, Inger H; Vadset, Tove B; Barstad, Johan; Svindseth, Marit F

    2015-06-01

    The purpose of this study was to develop and validate a survey to investigate parents' satisfaction with neonatal wards in a population of parents of children with a gestation age of ≥24 weeks to 3 months after full-term birth. We explored the literature and conducted three focus groups: two with expert health personnel and one with parents. We tested the survey in a parent population (N = 105) and report the different stages in the validation process along with the full survey, the Neonatal Satisfaction Survey - 13 categories (NSS-13). We found 13 subcategories in the Neonatal Satisfaction Survey. The subcategories measure parents' satisfaction with neonatal units based on staff, admission, nurses, anxiety, siblings (parents' perceptions of caring for the siblings of the newborn), information, timeout, doctors, facilities, nutrition, preparation for discharge, trust and visitors. Each subcategory showed acceptable internal consistency. The full version of the Neonatal Satisfaction Survey presents 69 items, and each subcategory contains two to eleven items. The Neonatal Satisfaction Survey seems suitable to measure parents' satisfaction with neonatal units and can be used in full, but it can also measure subcategories. Parents' satisfaction with neonatal units can be used to improve the quality in such wards. We consider this study as the first in a series to validate the NSS-13. The full survey with subcategories is presented in this paper. © 2014 Nordic College of Caring Science.

  9. Prevalence and pattern of congenital malformations among neonates in the neonatal unit of a teaching hospital

    International Nuclear Information System (INIS)

    Hussain, S.; Sabir, M.; Tarar, S. H.; Mushtaq, R.; Asghar, I.; Chattha, M. N.

    2014-01-01

    Objective: To determine the prevalence and pattern of congenital malformations among neonates in a teaching hospital. Methods: The prospective hospital-based study was conducted over a period of 18 months in the neonatal unit of Combined Military Hospital, Kharian, from September 2011 to February 2013. All neonates from newborn to 28 days of age admitted to the unit irrespective of their condition comprised the study population. Neonatal examination was done by the Registrar at the time of admission followed by neonatologist/paediatrician. Information regarding gender, weight, gestational age, mode of delivery, consanguinity, maternal age, antenatal visit record and family history were recorded on a predesigned proforma. After clinical examination, if required, relevant investigations like ultrasonography, radiology, echocardiography, laboratory and genetic studies were done to confirm diagnosis. Data was statistically analysed by using SPSS 20. Results: Out of 3,210 total admissions, 226 (7%) neonates were congenitally malformed. Of them, 130 (57.52 %) were male and 96 (42.47 %) females. Among different body systems affected, anomalies related to the central nervous system were 46(20.35%) musculoskeletal 42(18.58%), genitourinary 34 (15.04%), cardiovascular system 30 (13.27%), ear, eye, face, neck 27(11.94%), digestive system 19 (8.40%), syndromes and skin 14 (6.19%) each. Conclusion: Congenital Malformations are not rare in our community and central nervous system is the most commonly affected system. Healthcare managers must stress upon primary prevention in the form of vaccination, nutrition and drugs to decrease preventable share of congenital malformations. (author)

  10. Exercise training restores cardiac protein quality control in heart failure.

    Directory of Open Access Journals (Sweden)

    Juliane C Campos

    Full Text Available Exercise training is a well-known coadjuvant in heart failure treatment; however, the molecular mechanisms underlying its beneficial effects remain elusive. Despite the primary cause, heart failure is often preceded by two distinct phenomena: mitochondria dysfunction and cytosolic protein quality control disruption. The objective of the study was to determine the contribution of exercise training in regulating cardiac mitochondria metabolism and cytosolic protein quality control in a post-myocardial infarction-induced heart failure (MI-HF animal model. Our data demonstrated that isolated cardiac mitochondria from MI-HF rats displayed decreased oxygen consumption, reduced maximum calcium uptake and elevated H₂O₂ release. These changes were accompanied by exacerbated cardiac oxidative stress and proteasomal insufficiency. Declined proteasomal activity contributes to cardiac protein quality control disruption in our MI-HF model. Using cultured neonatal cardiomyocytes, we showed that either antimycin A or H₂O₂ resulted in inactivation of proteasomal peptidase activity, accumulation of oxidized proteins and cell death, recapitulating our in vivo model. Of interest, eight weeks of exercise training improved cardiac function, peak oxygen uptake and exercise tolerance in MI-HF rats. Moreover, exercise training restored mitochondrial oxygen consumption, increased Ca²⁺-induced permeability transition and reduced H₂O₂ release in MI-HF rats. These changes were followed by reduced oxidative stress and better cardiac protein quality control. Taken together, our findings uncover the potential contribution of mitochondrial dysfunction and cytosolic protein quality control disruption to heart failure and highlight the positive effects of exercise training in re-establishing cardiac mitochondrial physiology and protein quality control, reinforcing the importance of this intervention as a non-pharmacological tool for heart failure therapy.

  11. Brain injuries due to neonatal hypoglycemia: case report

    International Nuclear Information System (INIS)

    Kim, Dae Bong; Song, Chang Joon; Chang, Mae Young; Youn, Hyae Won

    2003-01-01

    Although hypoglycemia may be common among neonates, brain injuries resulting from isolated neonatal hypoglycemia are rare. The condition may cause neurological symptoms such as stupor, jitteriness, and seizures, though in their absence, diagnosis delayed or difficult. Hypoglycemia was diagnosed in a three-day-old neonate after he visited the emergency department with loose stool, poor oral intake, and decreased activity, first experienced two days earlier. Two days after his visity, several episodes of seizure occurred. T2 and diffusion-weighted magnetic resonance (MR) scanning, performed at 11 days of age, revealed bilateral and symmetrical high signal intensity lesions in occipital, parietal, and temporal lobes. We report the MR findings of hypoglycemic encephalopathy in a neonate

  12. Quantifying Neonatal Sucking Performance: Promise of New Methods.

    Science.gov (United States)

    Capilouto, Gilson J; Cunningham, Tommy J; Mullineaux, David R; Tamilia, Eleonora; Papadelis, Christos; Giannone, Peter J

    2017-04-01

    Neonatal feeding has been traditionally understudied so guidelines and evidence-based support for common feeding practices are limited. A major contributing factor to the paucity of evidence-based practice in this area has been the lack of simple-to-use, low-cost tools for monitoring sucking performance. We describe new methods for quantifying neonatal sucking performance that hold significant clinical and research promise. We present early results from an ongoing study investigating neonatal sucking as a marker of risk for adverse neurodevelopmental outcomes. We include quantitative measures of sucking performance to better understand how movement variability evolves during skill acquisition. Results showed the coefficient of variation of suck duration was significantly different between preterm neonates at high risk for developmental concerns (HRPT) and preterm neonates at low risk for developmental concerns (LRPT). For HRPT, results indicated the coefficient of variation of suck smoothness increased from initial feeding to discharge and remained significantly greater than healthy full-term newborns (FT) at discharge. There was no significant difference in our measures between FT and LRPT at discharge. Our findings highlight the need to include neonatal sucking assessment as part of routine clinical care in order to capture the relative risk of adverse neurodevelopmental outcomes at discharge. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  13. Efferent inhibition of otoacoustic emissions in preterm neonates

    Directory of Open Access Journals (Sweden)

    Renata Mota Mamede Carvallo

    2015-10-01

    Full Text Available ABSTRACT INTRODUCTION: Abnormalities in auditory function of newborns may occur not only because of preterm birth, but also from the use of medications and from diseases related to prematurity. OBJECTIVE: To analyze the inhibitory effect from stimulation of the olivocochlear efferent system on transient evoked otoacoustic emissions in preterm neonates, comparing these data with those from full-term neonates. METHODS: This was a prospective, cross-sectional, contemporary cohort study with 125 neonates, pooled into two groups: full-term (72 full-term neonates, 36 females and 36 males, born at 37-41 weeks of gestational age; and preterm (53 neonates, 28 males and 25 females, born at ≤36 weeks of gestational age, evaluated at the corrected gestational age of 37-41 weeks. Otoacoustic emissions were recorded using linear and nonlinear click-evoked stimuli, with and without contralateral stimulation. RESULTS: The inhibitory effect of the efferent pathway in otoacoustic emissions was different (p = 0.012 between groups, and a mean reduction of 1.48 dB SPL in full-term births and of 1.02 dB SPL in preterm births was observed for the non-linear click-evoked stimulus. CONCLUSION: The results suggest a reduced inhibitory effect of the olivocochlear efferent system on otoacoustic emissions in preterm neonates.

  14. The Use of Neuraxial Catheters for Postoperative Analgesia in Neonates: A Multicenter Safety Analysis from the Pediatric Regional Anesthesia Network.

    Science.gov (United States)

    Long, Justin B; Joselyn, Anita S; Bhalla, Tarun; Tobias, Joseph D; De Oliveira, Gildasio S; Suresh, Santhanam

    2016-06-01

    Currently, there is limited evidence to support the safety of neuraxial catheters in neonates. Safety concerns have been cited as a major barrier to performing large randomized trials in this population. The main objective of this study is to examine the safety of neuraxial catheters in neonates across multiple institutions. Specifically, we sought to determine the incidence of overall and individual complications encountered when neuraxial catheters were used for postoperative analgesia in neonates. This was an observational study that used the Pediatric Regional Anesthesia Network database. Complications and adverse events were defined by the presence of at least 1 of the following intraoperative and/or postoperative factors: catheter malfunction (dislodgment/occlusion), infection, block abandoned (unable to place), block failure (no evidence of block), vascular (blood aspiration/hematoma), local anesthetic systemic toxicity, excessive motor block, paresthesia, persistent neurologic deficit, and other (e.g., intra-abdominal misplacement, tremors). Additional analyses were performed to identify the use of potentially toxic doses of local anesthetics. The study cohort included 307 neonates with a neuraxial catheter. There were 41 adverse events and complications recorded, resulting in an overall incidence of complications of 13.3% (95% confidence interval, 9.8%-17.4%). Among the complications, catheter malfunction, catheter contamination, and vascular puncture were common. None of the complications resulted in long-term complications and/or sequelae, resulting in an estimated incidence of any serious complications of 0.3% (95% confidence interval, 0.08%-1.8%). There were 120 of 307 patients who received intraoperative and/or postoperative infusions consistent with a potentially toxic local anesthetic dose in neonates. The incidence of potentially toxic local anesthetic infusion rates increased over time (P = 0.008). Neuraxial catheter techniques for intraoperative

  15. Inflammatory and vascular placental lesions are associated with neonatal amplitude integrated EEG recording in early premature neonates.

    Directory of Open Access Journals (Sweden)

    Dorit Paz-Levy

    Full Text Available Placental histologic examination can assist in revealing the mechanism leading to preterm birth. Accumulating evidence suggests an association between intrauterine pathological processes, morbidity and mortality of premature infants, and their long term outcome. Neonatal brain activity is increasingly monitored in neonatal intensive care units by amplitude integrated EEG (aEEG and indices of background activity and sleep cycling patterns were correlated with long term outcome. We hypothesized an association between types of placental lesions and abnormal neonatal aEEG patterns.To determine the association between the placental lesions observed in extreme preterm deliveries, and their neonatal aEEG patterns and survival.This prospective cohort study included extreme premature infants, who were born ≤ 28 weeks of gestation, their placentas were available for histologic examination, and had a continues aEEG, soon after birthn = 34. Infants and maternal clinical data were collected. aEEG data was assessed for percentage of depressed daily activity in the first 3 days of life and for sleep cycling. Associations of placental histology with clinical findings and aEEG activity were explored using parametric and non-parametric statistics.Twenty two out of the 34 newborns survived to discharge. Preterm prelabor rupture of membranes (PPROM or chorioamnionitis were associated with placental lesions consistent with fetal amniotic fluid infection (AFI or maternal under perfusion (MUP (P < 0.05. Lesions consistent with fetal response to AFI were associated with absence of SWC pattern during the 1st day of life. Fetal-vascular-thrombo-occlusive lesions of inflammatory type were negatively associated with depressed cerebral activity during the 1st day of life, and with aEEG cycling during the 2nd day of life (P<0.05. Placental lesions associated with MUP were associated with depressed neonatal cerebral activity during the first 3 days of life (P = 0

  16. Post-cardiotomy extracorporeal cardiopulmonary resuscitation in neonates with complex single ventricle: analysis of outcomes.

    Science.gov (United States)

    Polimenakos, Anastasios C; Wojtyla, Patrice; Smith, Pamela J; Rizzo, Vincent; Nater, Melissa; El Zein, Chawki F; Ilbawi, Michel N

    2011-12-01

    Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We sought to review outcomes of neonates with functional single ventricle (FSV) receiving post-cardiotomy ECPR. Forty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2009) were identified. Twenty-seven were neonates. Review of records and survival analysis were conducted. Of 27 neonates receiving post-cardiotomy ECMO 20 had FSV. Fourteen had ECPR. Ten underwent Norwood operation (NO) for hypoplastic left heart syndrome (HLHS). Four had FSV other than HLHS. Three underwent Damus-Kay-Stansel or modified NO with systemic-to-pulmonary shunt (SPS) and one SPS with anomalous pulmonary venous connection repair. Mean age and weight were 7.8 ± 2.9 days and 3.44 ± 1.78 kg, respectively. ECMO median duration was 6 days (interquartile range (IQR) 3-14). Survival to ECMO discontinuation was 79% (11 of 14 patients) and at hospital discharge was 57% (8 of 14 patients). The most common cause of death was multi-organ failure (four of six deaths). At last follow-up (median: 11 months (1-34)) 43% of patients were alive. CPR mean duration for patients with favorable versus unfavorable outcome was 38.6 ± 6.3 versus 42.1 ± 7.7 min (p = 0.12). Previously reported determinants for poorer prognosis in conventional non-rescue ECMO (such as pre-ECMO pH0.05). ECMO support in neonates with FSV requiring ECPR can result in favorable outcome in more than half of patients at hospital discharge. Aggressive strategy toward timely application of ECPR is justified. Expeditious ECPR deployment after proper patients' selection, refinement of CPR quality and use of adjunctive neuroprotective interventions, such as induced hypothermia, might further improve outcomes. Copyright © 2011 European Association for

  17. Prevalence of neonatal septicaemia in the University of Port ...

    African Journals Online (AJOL)

    Background : Septicaemia is a major cause of morbidity and mortality in the neonatal period. Early detection of neonatal septicaemia is often hampered by its subtle and nonspecific symptoms and signs thus a high index of suspicion is needed. Objectives: To determine the prevalence of neonatal sept ic a emia , ident i fy ...

  18. The first occurrence of a CTX-M ESBL-producing Escherichia coli outbreak mediated by mother to neonate transmission in an Irish neonatal intensive care unit.

    LENUS (Irish Health Repository)

    O'Connor, Ciara

    2017-01-05

    Escherichia coli (E. coli) comprise part of the normal vaginal microflora. Transfer from mother to neonate can occur during delivery resulting, sometimes, in neonatal bacterial disease. Here, we aim to report the first outbreak of CTX-M ESBL-producing E. coli with evidence of mother-to-neonate transmission in an Irish neonatal intensive care unit (NICU) followed by patient-to-patient transmission.

  19. Bloodstream Infections in a Neonatal Intensive Care Unit

    OpenAIRE

    Mehmet Sah Ipek

    2016-01-01

    Aim: To determine the pattern of bloodstream infections (BSIs) and antimicrobial susceptibility of pathogens in a neonatal intensive care unit (NICU).Material and Method: Positive hemoculture of neonates diagnosed with nosocomial sepsis from March 2011 to March 2014 in the NICU of Diyarbakir Maternity and Children%u2019s Hospital, in the southeastern region of Anatolia, Turkey, were retrospectively reviewed. Results: A total of 148 pathogens were isolated in 142 neonates. The most common micr...

  20. Photodegradation of riboflavin in neonates

    International Nuclear Information System (INIS)

    Sisson, T.R.

    1987-01-01

    The biologically most important flavins are riboflavin and its related nucleotides, all highly sensitive to light. It is because of its photoreactivity and its presence in almost all body fluids and tissues that riboflavin assumes importance in phototherapy of neonatal jaundice. The absorption maxima of both bilirubin and riboflavin in the body are nearly identical: 445-450 (447) nm. In consequence, blue visible light will cause photoisomerization of bilirubin accompanied by photodegradation of riboflavin. This results in diminished erythrocyte glutathione reductase, which indicates generalized tissue riboflavin deficiency and red cell lysis. Single- and double-strand breaks in intracellular DNA have occurred with phototherapy. This light exposure of neonates may result also in alterations of bilirubin-albumin binding in the presence of both riboflavin and theophylline (the latter frequently given to prevent neonatal apnea). Many newborns, especially if premature, have low stores of riboflavin at birth. The absorptive capacity of premature infants for enteral riboflavin is likewise reduced. Consequently, inherently low stores and low intake of riboflavin plus phototherapy for neonatal jaundice will cause a deficiency of riboflavin at a critical period for the newborn. Supplementation to those infants most likely to develop riboflavin deficiency is useful, but dosage, time, and mode of administration to infants undergoing phototherapy must be carefully adjusted to avoid unwanted side effects