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Sample records for clinical experience congenital

  1. Congenital Portosystemic Shunt: Our Experience

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    Tiziana Timpanaro

    2015-01-01

    Full Text Available Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II. In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down’s syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis.

  2. Clinical Experience of Prosthodontics Treatment on Congenital Missing Teeth%口腔修复治疗先天性缺牙的临床体会

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    徐尤学

    2014-01-01

    目的:观察对先天性缺牙患者进行口腔修复治疗的临床方法和效果。方法:2011年3月~2013年3月我院收治的先天性缺牙67例患者进行口腔修复,观察临床效果。结果:治疗后患者咀嚼功能平均分和语言功能平均分高于治疗前;患者对治疗满意度为98.51%,结果有统计学意义(P<0.05)。结论:对先天性缺牙患者积极的进行口腔修复治疗,临床效果较好,不良反应及并发症较少,治疗满意度较高,值得推广。%Objective:To observe the clinical approach and effect of the clinical experience of prosthodontics treatment on congenital missing teeth. Method:Observing clinical effect of 67 patients with congenital missing teeth from March,2011 to March,2013 in the hospital. Results:After treatment,the average score of masticatory function and language function points were higher than the average pre-treatment; patient treatment satisfaction was 98.51%,and the results had statistically significant meaning(P <0.05). Conclusion:For patients with congenital missing teeth,dental restorative treatment has good clinical effect,with fewer adverse reactions and complications,and higher treatment satisfaction. So it is worthy of promotion.

  3. Congenital myopathies: clinical and immunohistochemical study.

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    Thaha, Fazil; Gayathri, N; Nalini, A

    2011-01-01

    Congenital myopathies (CMs), a group of relatively non-progressive disorders presents with weakness and hypotonia of varying severity, morphologically recognized by specific structural abnormalities within the myofiber. This report presents the clinical and Histopathological features of 40 patients with CMs. Centronuclear myopathy was the commonest (40%) followed by congenital fiber type disproportion (37.5%). Other less common CMs included: myotubular myopathy (5%), nemaline myopathy (5%), central core disease (5%), multicore disease (2.5%) and congenital myopathy with tubular aggregate (5%). Immunolabeling to desmin corresponded to morphological changes within the myofibers while vimentin was negative in all the patients. There is no combined role of these proteins in the disease process. PMID:22234203

  4. Our experience in congenital pouch colon

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    Gharpure Vivek

    2007-01-01

    Full Text Available Congenital pouch colon is considered to be a malformation seen only in north india. we carried out a prospective study of congenital pouch colon from 1991 to 2005 to determine what interventions are most suited for the short and long term management of this compex malformation. anatomical details, procedures and continence outcomes were recorded. patients were managed in a private and public hospital by a single surgeon. 17 patients could be enrolled in the study. we could reconstruct 16/17 patients. with aggressive bowel management, 9/14 patients could achieve continence.

  5. Clinical, chromosomal and endocrine studies for congenital adrenal hyperplasia

    International Nuclear Information System (INIS)

    Severe forms of congenital adrenal hyperplasia are potentially fatal if unrecognized and untreated. The aim of this study was to clarify the clinical presentation together with the chromosomal and laboratory associations in this syndrome. Twenty four patients diagnosed as congenital adrenal hyperplasia were referred from Children's Hospital, Cairo University, Egypt, for hormonal and chromosomal workup. The age ranged from eight months to 19 years with mean age of 3.18 years. Twenty two patients were diagnosed as classic congenital adrenal hyperplasia (CAH) syndrome. Severe salt wasting form was present in ten patients whereas simple virilisation was the presenting manifestation in twelve patients. Two patients presented as late onset congenital adrenal hyperplasia (LOCAH). The sex of rearing was female in 18 cases and male in six cases. Genitography and sonography confirmed the presence of female internal organs in all cases. Advanced bone age was evident by radiographic studies. Although the karyotyping was 46,XX in all cases, the diagnosed correct sex was delayed in six cases. Serum concentrations of 17-hydroxyprogesterone (17.OH.P), dehydroepiandrosterone sulfate (DHEAS), delta, 4-androstenedione (D4A), testosterone (T) and 11-deoxycortisol were all elevated as compared to controls. It was found that the adrenal androgens DHEAS, D4A and T were more elevated in salt losers when compared to simple virilising patients. However, this difference was statistically non-significant. The present study demonstrates that the clinical examination and laboratory investigations are necessary for the early detection and treatment of these cases to avoid major medical and psychological problems for the patients and their parents

  6. Clinical, Chromosomal and Endocrine Studies for Congenital Adrenal Hyperplasia

    International Nuclear Information System (INIS)

    Several forms of congenital adrenal hyperplasia are potentially fatal if unrecognized and untreated. The aim of this study is to throw light on the clinical presentation together with chromosomal and laboratory associations in this syndrome. Twenty four patients diagnosed as congenital adrenal hyperplasia were referred from the Diabetic Endocrine Metabolic Pediatric Unit [DEMPU], Children's Hospital, Cairo University for hormonal and chromosomal workup. Twenty two patients were diagnosed as classic congenital adrenal hyperplasia (CAH) syndrome. Sever salt wasting form was present in ten patients whereas simple virilization was the presenting manifestation in twelve patients. Two patients presented as late onset congenital adrenal hyperplasia (LOCAH). The mean age was 3.18 years, ranging from eight months to 19 years. The sex of rearing was Female in 18 cases and male in six cases. Genitography and sonography confirmed the presence of female internal organs in all cases. Advanced bone age was evident by radiographic studies. Although the karyotyping was (46,XX) in all cases, the correct sex diagnosis was delayed in 6 cases. Serum concentrations of 17-hydroxyprogesterone (17.OH.P); Dehydroepiandrosterone sulfate (DHEAS); Delta,4-androstenedione (D 4 A); Testosterone and 11-deoxycortisol were all elevated in relation to controls. We found that the adrenal androgens DHEAS, delta 4A, and T were more elevated in salt losers when compared to simple virilizing patients. However, this difference was not of statistical significance. The present study demonstrates that clinical examination and laboratory investigations are necessary for early detection and treatment of hese cases to avoid major medical and psychological problems for the patients and their parents.

  7. CONGENITAL CRYPTORCHIDISM - ITS ANATOMICAL ASPECTS, EMBRYOGENESIS AND CLINICAL CONSEQUENCES

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    Kalyan Chakravarthi

    2013-01-01

    The testes are a pair of male reproductive organs. The testicular variations are unusual and can result in multiple clinical conditions. Congenital cryptorchidism is a condition in which one or both the testes have not passed down the scrotal sac. During a routine cadaveric dissection of a middle aged male cadaver, in the left side of the pelvic part of the abdomen an unusual undescended testis was noted near the deep inguinal ring. Additional to this we also noted a thick band of ligament co...

  8. Ullrich Congenital Muscular Dystrophy (UCMD: Clinical and Genetic Correlations

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    Bita BOZORGMEHR

    2013-08-01

    Full Text Available How to Cite This Article: Bozorgmehr B, Kariminejad A, Nafissi Sh, Jebelli B, Andoni U, Gartioux C, Ledeuil C, Allamand Y, Richard P, Kariminejad MH. Ullrich Congenital Muscular Dystrophy (UCMD:Clinical and Genetic Correlations. Iran J Child Neurol. 2013 Summer; 7(3: 15-22.  Objective:Ullrich congenital muscular dystrophy (UCMD corresponds to the severe end of the clinical spectrum of neuromuscular disorders caused by mutations in the genes encoding collagen VI (COL VI. We studied four unrelated families with six affected children that had typical UCMD with dominant and recessive inheritance.Materials & MethodsFour unrelated Iranian families with six affected children with typical UCMD were analyzed for COLVI secretion in skin fibroblast culture and the secretion of COLVI in skin fibroblast culture using quantitative RT–PCR (Q-RT-PCR, and mutation identification was performed by sequencing of complementary DNA.ResultsCOL VI secretion was altered in all studied fibroblast cultures. Two affected sibs carried a homozygous nonsense mutation in exon 12 of COL6A2, while another patient had a large heterozygous deletion in exon 5-8 of COL6A2. The two other affected sibs had homozygote mutation in exon 24 of COL6A2, and the last one was homozygote in COL6A1.ConclusionIn this study, we found out variability in clinical findings and genetic inheritance among UCMD patients, so that the patient with complete absence of COLVI was severely affected and had a large heterozygous deletion in COL6A2. In contrast, the patients with homozygous deletion had mild to moderate decrease in the secretion of COL VI and were mildly tomoderately affected.References1. Voit T. Congenital Muscular Dystrophies Brain Dev 1998;20(2: 65-74.2. Ullrich OZ Ges. Scleroatonic Muscular Dystrophy. NeurolPsychiatr 1930;126:171-201.3. Ullrich O. Monatsschr. Kinderheilkd 1930;47:502-10.4. Mercuri E, Yuva Y, Brown SC, Brockington M, Kinali M, Jungbluth H, et al. Collagen VI involvement in

  9. Congenital choledochal cysts in adults: twenty-five-year experience

    Institute of Scientific and Technical Information of China (English)

    LIU Ying-bin; CAO Li-ping; PENG Shu-you; WANG Jian-wei; Khagendra Raj Devkota; JI Zhen-ling; LI Jiang-tao; WANG Xu-an; MA Xiao-ming; CAI Wei-long; KONG Ying

    2007-01-01

    Background Choledochal cyst is rare in western countries. The relatively high incidence of coexistent hepatobiliary disease increases the difficulty of the surgical management of choledochal cyst. Here we analyze the diagnosis and treatment of congenital bile duct cyst in 122 Chinese adults.Methods The clinical data of 122 patients with congenital choledochal cysts admitted from 1981 to 2006 were analyzed.Results Clinical symptoms in most cases were nonspecific, resulting in delayed diagnosis. Sixty-one patients (50%) had coexistent pancreatobiliary disease. Among the 122 patients, 119 patients underwent ultrasonic examination;ERCP/MRCP was performed in 63 cases and CT in 102 cases. Abnormal pancreatobiliary duct junction was found in 48 patients. Sixteen patients had malignant lesions in the bile duct, arising in 11 of them from incomplete choledochal cyst that underwent various operations including cystenterostomy or cystojejunostomy. There was significant difference between the patients who underwent incomplete cyst resection and complete cyst resection in malignancy rate of bile duct (Chi square test, P= 0.000; odds ratio, 7.800; 95 % confidence interval, 2.450 to 24.836).Conclusions ERCP, CT and MRCP had proved their great values in the classification of the disease. Cyst excision with Roux-en-Y hepaticojejunostomy is recommended as the treatment of choice for patients with type Ⅰ or type Ⅳ cysts. For type Ⅴ cyst (Caroli's disease) with recurrent cholangitis, liver transplantation should be considered.

  10. Congenital hydrocephalus in clinical practice : A genetic diagnostic approach

    NARCIS (Netherlands)

    Verhagen, J. M. A.; Schrander-Stumpel, C. T. R. M.; Krapels, P. C.; de Die-Smulders, C. E. M.; van Lint, F. H. M.; Willekes, C.; Weber, J. W.; Gavilanes, A. W. D.; Macville, M. V. E.; Stegmann, A. P. A.; Engelen, J. J. M.; Bakker, J.; Vos, Y. J.; Frints, S. G. M.

    2011-01-01

    Congenital hydrocephalus is a common and often disabling disorder. The etiology is very heterogeneous. Little is known about the genetic causes of congenital hydrocephalus. A retrospective survey was performed including patients with primary congenital hydrocephalus referred to the Department of Cli

  11. Cardiomyopathy in congenital and acquired generalized lipodystrophy: a clinical assessment.

    Science.gov (United States)

    Lupsa, Beatrice C; Sachdev, Vandana; Lungu, Andreea O; Rosing, Douglas R; Gorden, Phillip

    2010-07-01

    Lipodystrophy is a rare disorder characterized by loss of adipose tissue and low leptin levels. This condition is characterized by severe dyslipidemia, insulin resistance, diabetes mellitus, and steatohepatitis. Another phenotypic feature that occurs with considerable frequency in generalized lipodystrophy is cardiomyopathy. We report here the cardiac findings in a cohort of patients with generalized congenital and acquired lipodystrophy, and present a literature review of the cardiac findings in patients with generalized lipodystrophy. We studied 44 patients with generalized congenital and acquired lipodystrophy, most of them enrolled in a clinical trial of leptin therapy. Patients underwent electrocardiograms and transthoracic echocardiograms to evaluate their cardiac status. We followed these patients for an extended time period, some of them up to 8 years. Evaluation of our cohort of patients with generalized lipodystrophy shows that cardiomyopathy is a frequent finding in this population. Most of our patients had hypertrophic cardiomyopathy, and only a small number had features of dilated cardiomyopathy. Hypertrophic cardiomyopathy was more frequent in patients with seipin mutation, a finding consistent with the literature. The underlying mechanism for cardiomyopathy in lipodystrophy is not clear. Extreme insulin resistance and the possibility of a "lipotoxic cardiomyopathy" should be entertained as possible explanations.

  12. The clinical and radiological observation of congenital syphilis

    International Nuclear Information System (INIS)

    Congenital syphilis is transmitted through the placenta by the infected mother after 16 weeks of gestation. Since the incidence of syphilis is again on the increase, syphilis remains at the present time a public problem of major and increasing proportions. Recently, congenital syphilis has different formas of presentation. The authors observed clinically and radiologically 27 cases of congenital syphilis in the neonates and infants treated at the pediatric ward of Chonnam National University Hospital from Jan. 1977 to Mar. 1982. The results are as follows: 1. The number of observed patients was 27 cases, 22 cases in male, 5 cases in female. 2. Onset of first clinical symptoms and signs was within the 4th week life in 14 cases (52%), 1-2 months in 6 cases (22%), 2-3 months in 4 cases (15%), 3-4 months in 3 cases (11%). All cases was within 6 months. 3. The order of the frequency of common clinical manifestations was hepatomegaly (96%), splenomegaly (78%), skin lesion (63%), anemia (63%), nasal snuffle (56%). 4. Of 11 cases with known birth weight, 10 cases were low birth weight. 5. The serologic test (VDRL slide test) of 27 tested caes revealed reactive response in 26 cases, non-reactive response in 1 case, and that of syphilitic mothers except one revealed reactive in 23 cases, non- reactive in 3 cases. 6. Roentgenographic syphilitic bony changes were detected in 26 cases (96%), of 27 studied cases, osteochondritis was present in 24 cases (89%), periostitis in 21 case (78%), osteomyelitis in 11 cases (41%). 7. The most common sites affected were as follows. Radius and ulna were the most frequently affected, the next were in order of tibia and fibula. Considering osteochondiritis only, the distal end of radius and ulna (78% respectively) and proximal end of tibra (67%) were the most frequently affected sites, the proximal end of femur (33%) was least frequently affected site. 8. On chest films of 27 case, osseous changes (mainly periostitis) of clavicle were noted

  13. The clinical and radiological observation of congenital syphilis

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    Nah, Byung Sik; Chung, Ung Ki [Chonnam National University College of Medicine, Kwangju (Korea, Republic of)

    1983-03-15

    Congenital syphilis is transmitted through the placenta by the infected mother after 16 weeks of gestation. Since the incidence of syphilis is again on the increase, syphilis remains at the present time a public problem of major and increasing proportions. Recently, congenital syphilis has different formas of presentation. The authors observed clinically and radiologically 27 cases of congenital syphilis in the neonates and infants treated at the pediatric ward of Chonnam National University Hospital from Jan. 1977 to Mar. 1982. The results are as follows: 1. The number of observed patients was 27 cases, 22 cases in male, 5 cases in female. 2. Onset of first clinical symptoms and signs was within the 4th week life in 14 cases (52%), 1-2 months in 6 cases (22%), 2-3 months in 4 cases (15%), 3-4 months in 3 cases (11%). All cases was within 6 months. 3. The order of the frequency of common clinical manifestations was hepatomegaly (96%), splenomegaly (78%), skin lesion (63%), anemia (63%), nasal snuffle (56%). 4. Of 11 cases with known birth weight, 10 cases were low birth weight. 5. The serologic test (VDRL slide test) of 27 tested caes revealed reactive response in 26 cases, non-reactive response in 1 case, and that of syphilitic mothers except one revealed reactive in 23 cases, non- reactive in 3 cases. 6. Roentgenographic syphilitic bony changes were detected in 26 cases (96%), of 27 studied cases, osteochondritis was present in 24 cases (89%), periostitis in 21 case (78%), osteomyelitis in 11 cases (41%). 7. The most common sites affected were as follows. Radius and ulna were the most frequently affected, the next were in order of tibia and fibula. Considering osteochondiritis only, the distal end of radius and ulna (78% respectively) and proximal end of tibra (67%) were the most frequently affected sites, the proximal end of femur (33%) was least frequently affected site. 8. On chest films of 27 case, osseous changes (mainly periostitis) of clavicle were noted

  14. Abnormal Congenital Location of Stapes’ Superstructure: Clinical and Embryological Implications

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    Vânia Henriques

    2016-01-01

    Full Text Available Congenital middle ear malformations are rare. Most part of them are usually associated with other malformations, such as aural atresia, microtia, and dysmorphic craniofacial features. A clinical case of a 24-year-old male with a right-sided conductive hearing loss since his childhood, without craniofacial malformation, is presented. He was proposed for exploratory tympanotomy under the suspicious diagnosis of otosclerosis. The surgery revealed an abnormal location of stapes’ superstructure, which was attached to the promontory and had an isolated and mobile osseous footplate in the oval window. A stapes prosthesis was inserted and resulted in closure of the air-bone gap by 25 dB. A review of the literature was also performed using MEDLINE. Two theories diverge on the embryologic origin of the stapes. Our findings seem to be in favour of the theory that defines two different embryologic origins to the stapes.

  15. Congenital duodenal obstruction in neonates:a decade's experience from one center

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    Qing-Jiang Chen; Zhi-Gang Gao; Jin-Fa Tou; Yun-Zhong Qian; Min-Ju Li; Qi-Xing Xiong; Qiang Shu

    2014-01-01

    Background: Congenital duodenal obstruction (CDO) is one of the most common anomalies in newborns, and accounting for nearly half of all cases of neonatal intestinal obstruction. This study aimed to review our single-center experience in managing congenital duodenal obstruction while evaluate the outcomes. Methods: We conducted a retrospective analysis of the records of all neonates dianogsed with congenital duodenal obstruction admitted to our center between January 2003 and December 2012. We analyzed demographic criteria, clinical manifestations, associated anomalies, radiologic findings, surgical methods, postoperative complications, and fi nal outcomes. Results: The study comprised 287 newborns (193 boys and 94 girls). Birth weight ranged from 950 g to 4850 g. Fifty-three patients were born prematurely between 28 and 36 weeks' gestation. Malrotation was diagnosed in 174 patients, annular pancreas in 66, duodenal web in 55, duodenal atresia or stenosis in 9, preduodenal portal vein in 2, and congenital band compression in 1. Twenty patients had various combinations of these conditions. Presenting symptoms included bilious vomiting, dehydration, and weight loss. X-rays of the upper abdomen demonstrated the presence of a typical doublebubble sign or air-fl uid levels in 68.64% of patients, and confi rmatory upper and/or lower gastrointestinal contrast studies were obtained in 64.11%. Multiple associated abnormalities were observed in 50.52% of the patients. Various surgical approaches were used, including Ladd's procedure, duodenoplasty, duodenoduodenostomy, duodenojejunostomy, or a combination of these. Seventeen patients died postoperatively and 14 required re-operation. Conclusions: Congenital duodenal obstruction is a complex entity with various etiologies and often includes multiple concomitant disorders. Timely diagnosis and aggressive surgery are key to improving prognosis. Care should be taken to address all of the causes of duodenal obstruction and

  16. [Clinical evaluation of congenital cholesteatoma of the middle ear].

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    Kikuchi, Masahiro; Yamamoto, Etsuo; Shinohara, Shogo; Shiomi, Yousaku; Fujiwara, Keizo; Shiomi, Yoshiko; Watanabe, Futoshi; Tanabe, Makito

    2003-08-01

    We conducted a retrospective study to identify the clinical features and surgical observations of congenital cholesteatoma. Sixty patients were diagnosed and underwent surgery for congenital cholesteatoma between April 1987 and May 2002. All diagnoses were made on the basis of two operative findings: 1. the tympanic membrane manifested neither retraction, perforation, nor granulation. 2. the tympanic membrane was not continuous with the cholesteatoma. In this series, congenital cholesteatoma accounted for 7% of all cholesteatomas (853 ears). The patient age ranged from 2 to 48 years. The male to female ratio was 4:1. Seventeen patients had multiple cholesteatoma. Fifty-three patients exhibited closed-type cholesteatomas, while the remaining 7 patients had open-type cholesteatomas that had formed as a flat surface of the epidermis. Patients with open-type cholesteatomas presented with a much more pronounced conductive hearing loss and ossicular erosion or malformation. Twenty-two patients with relatively small cholesteatomas were analyzed to estimate the origin of their cholesteatomas. Of the 22 patients, 13 had anterior superior quadrant (ASQ-type) and 9 had posterior superior quadrant (PSQ-type) cholesteatomas. The mean age at the time of detection was older in the PSQ-type group than in the ASQ-type group and the frequency of ossicular erosion or malformation was more prominent in the PSQ-type group than in the ASQ-type group. The primary site of origin was thought to be the portion between the tympanic ostium of the auditory canal and the semicanal for tensor tympani in the ASQ-type group and near the incudostapedial joint in the PSQ-type group. A planned staged procedure was performed in 29 patients, 15 patients (52%) had residual lesions situated mostly on the oval window, the round window, an exposed facial nerve or an exposed lateral semicircular canal. The frequency of residual lesions in patients who presented with extended, multiple cholesteatoma and

  17. Spectrum of congenital myopathies: A single centre experience

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    Megha S Uppin

    2013-01-01

    Full Text Available Background: Congenital myopathies (CMs are rare and they are clinically and genetically heterogeneous. Muscle biopsy is characterized by structural abnormality that is diagnostic. There are few studies from India. Materials and Methods: This is a retrospective study of 12 years. The demographic data, clinical features and laboratory data of patients diagnosed as CMs on muscle biopsy were retrieved from medical records. The slides were reviewed for morphological and structural abnormalities using the following stains hematoxylin and eosin, modified Gomori trichrome, masson trichrome, periodic acid schiff, adenosine triphosphatase preincubated at pH 9.4, 4.6 and 4.3, nicotinamide adenine dinucleotide tetrazolium reductase, succinic dehydrogenase and cytochrome c oxidase. Immunohistochemistry was performed with dystrophin, sarcoglycans and desmin wherever necessary. Results: There were 50 patients with CMs: Centronuclear myopathy (23, myotubular myopathy (3 and central core disease (CCD (8, nemaline myopathy (5, congenital fiber type proportion (10 and desmin related myopathy with arrythmogenic right ventricular cardiomyopathy (ARVD (1. Of the 50 patients, 30 (60% presented in the first decade of life. Proximal muscle weakness and hypotonia were the common presenting features. Type 1 atrophy and predominance were seen in most cases on muscle biopsy. CCD had one patient with high creatine phosphokinase levels, biopsy in one patient showed both rods and cores, in the other limb girdle muscular dystrophy like picture and one biopsy showed uniform type 1 fibers. There was one desmin related myopathy with ARVD, who had cardiac transplantation and both skeletal and cardiac muscle showed characteristic rimmed vacuoles and inclusions positive for desmin. Conclusion: CMs are rare and the diagnosis can only be established on muscle biopsy. Defining the specific CMs helps the clinician in counseling the patient and family.

  18. 机器人辅助先天性心脏病矫正术的临床经验%Clinical experiences on correction of congenital heart diseases with robotic technology: a report of 160 cases

    Institute of Scientific and Technical Information of China (English)

    杨明; 高长青; 肖苍松; 王刚; 王加利; 吴扬

    2012-01-01

    目的 总结机器人手术系统在先天性心脏病矫正术中应用的临床经验.方法 回顾性分析2007年1月至2012年5月解放军总医院心血管外科160例接受机器人辅助下、先天性心脏病(简称先心病)矫正术患者的临床资料,其中男74例,女86例;年龄11~62(35±12)岁.继发孔型房间隔缺损130例,原发孔型房间隔缺损1例,膜周部室间隔缺损21例,二尖瓣前叶裂7例,二尖瓣前叶裂+左房黏液瘤1例.采用外周体外循环技术;于右侧胸壁打3个直径为0.8 cm、1个直径1.5 cm的小孔后,机器人辅助下完成心内畸形矫正.术中食管超声引导建立体外循环及评估手术效果,术后常规随访.收集手术时间、体外循环时间、术后复查和随访资料并进行统计学分析.结果 全组患者成功接受机器人辅助下心脏手术,无术式转化.其中76例房间隔缺损于心脏不停跳下完成.心脏停跳下房缺修补术的主动脉阻断时间随手术例数增加明显下降;心脏不停跳下房缺修补术手术时间随手术例数的增加逐步缩短.随访1 ~61个月,平均(29±16)个月,未见残余分流和恶性心律失常等并发症的发生.结论 机器人技术可安全地应用于部分简单先心病矫正,是一种可供选择的微创术式.%Objective To summarize the clinical experiences on correction of congenital heart disease with robotic technology at a single center.Methods Between January 2007 and May 2012,this retrospective study recruited 160 consecutive patients undergoing robotic surgery for congenital heart diseases.There were 74 males and 86 females with a median age of 35 years ( range:11 - 62 ). The procedures included secundum-type atrial septal defect repair ( n =130),ostium primum defect repair ( n =1 ),perimembranous ventricular septal defect repair ( n =21 ),mitral valve repair for anterior leaflet cleft ( n =7 ) and mitral valve repair plus left atrial myxoma resection ( n =1 ).Cardiopulmonary

  19. Use of an Administrative Database to Determine Clinical Management and Outcomes in Congenital Heart Disease

    OpenAIRE

    Gutgesell, Howard P.; Hillman, Diane G.; McHugh, Kimberly E.; Dean, Peter; Matherne, G. Paul

    2011-01-01

    We review our 16-year experience using the large, multi-institutional database of the University HealthSystem Consortium to study management and outcomes in congenital heart surgery for hypoplastic left heart syndrome, transposition of the great arteries, and neonatal coarctation. The advantages, limitations, and use of administrative databases by others to study congenital heart surgery are reviewed.

  20. Transgressive first clinical experiences

    DEFF Research Database (Denmark)

    Jensen, Carsten Juul; Jeppesen, Lise Kofoed; Drachmann, Merete

    2014-01-01

    . The nursing students’ learning seems to be oriented towards socialization in the clinic as a workplace. This means that the nursing students seek to deal with overwhelming experiences concerning the naked bodies of patients and death, useful application of theoretical knowledge, the path from novice...... to advanced beginner, and adjusting to the workplace community. The conclusion is that the learning of nursing students during their first clinical in-service placement appears informal and not founded on evident best practice.......This Study seeks to comprehend learning experiences of nursing students during their first clinical in-service placement. This Paper is part of a longitudinal development project interviewing the Student Nurse after each one of the five clinical in-service placements and then one year after...

  1. Clinical features of adult patients with Eisenmenger syndrome associated with different types of congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    陈果

    2013-01-01

    Objective To explore the clinical features and hemodynamics of adult patients with Eisenmenger syndrome in different types of congenital heart diseases (CHD) .Methods Patients with Eisenmenger syndrome with different types of CHD diagnosed by right heart

  2. Diagnosis and treatment of Congenital choledochal cyst:20 years'experience in China

    Institute of Scientific and Technical Information of China (English)

    Liu-BJn Shi; Shu-You Peng; Xing-Kai Meng; Cheng-Hong Peng; Ying-Bin Liu; Xiao-Peng Cheni; Zhen-Ling Ji; De-Tong Yang; Huai-Ren Chen

    2001-01-01

    AIM To summarize the experience of diagnosis and treatment of congenital choledochal cyst in the past 20years ( 1980 2000).``METHODS The clinical data of 108 patients admitted from 1980 to 2000 were analyzed retrospectively.RESULTS Abdominal pain, jaundice and abdominal mass were presented in most child cases. Clinical symptoms in adult cases were non-specific, resulting in delayed diagnosis frequently. Fifty-seven patients (52.7%) had coexistent pancreatiobiliary disease. Carcinoma of the biliary duct occurred in 18 patients (16.6%). Ultrasonic examination was undertaken in 94 cases, ERCP performed in 46 cases and CT in 71 cases. All of the cases were correctly diagnosed before operation. Abnormal pancreatobiliary duct junction was found in .39 patients.Before 1985 the diagnosis and classification of congenital choledochal cyst were established by ultrasonography preoperatively and confirmed during operation, the main procedures were internal drainage by cyst enterostomy.After 1985, the diagnosis was established by ERCP and CT. and cystectomy with Roux-en-Y hepaticojejunostomy was the conventional procedures. In 1994, we reported a new and simplified operative procedure in order to reduce the risk of choledochal cyst malignancy. Postoperative complication was mainly retrograde infection of biliary tract, which could be controlled by the administration of antibiotics, there was no perioperative mortality.``CONCLUSION The concept in diagnosis and treatment of congenital choledochal cyst has obviously been changed greatly. CT and ERCP were of great help in the classification of the disease. Currently, cystectomy with Roux-en-Y hepaticojejunostomy is strongly recommended as the choice for patients with type I and type Ⅳ cysts.Piggyback orthotopic liver transplantation is indicated in type \\ cysts (Carolis disease) with frequently recurrent cholangitis.``

  3. CLINICAL STUDY OF CONGENITAL HEART DISEASE IN INFANTS IN TERTIARY CARE HOSPITAL

    OpenAIRE

    Sandeep V.Harshangi; Laxmi Nagaraj Itagi; Venkatesh Patil; Vijayanath.V

    2013-01-01

    Congenital heart disease are primarily disease of neonates, infants and children. The burden of CHD in India is likely to be enormous due to very high birth rate. The reported incidence is 8-10/ 1000 live births. Objective was to know the pattern of clinical presentation of various congenital heart disease in 0-1 year age group. To study the different form of cyanotic and acyanotic congenital heart disease in present cases. 50 cases of CHD proven by 2D echocardiography were studied for 2 ye...

  4. Congenital dislocation of the patella – clinical case☆

    Science.gov (United States)

    Miguel Sá, Pedro; Raposo, Filipa; Santos Carvalho, Manuel; Alegrete, Nuno; Coutinho, Jorge; Costa, Gilberto

    2015-01-01

    Congenital patellar dislocation is a rare condition in which the patella is permanently dislocated and cannot be reduced manually. The patella develops normally as a sesamoid bone of the femur. This congenital dislocation results from failure of the internal rotation of the myotome that forms the femur, quadriceps muscle and extensor apparatus. It usually manifests immediately after birth, although in some rare cases, the diagnosis may be delayed until adolescence or adulthood. Early diagnosis is important, thereby allowing surgical correction and avoiding late sequelae, including early degenerative changes in the knee. A case of permanent dislocation of the patella is presented here, in a female child aged seven years. PMID:26962496

  5. Congenital posterolateral diaphragmatic hernia : pathophysiological studies and clinical picture

    NARCIS (Netherlands)

    A.P. Bos (Albert)

    1993-01-01

    textabstractCongenital diaphragmatic hernias are classified according to the location of the defect: posterolateral hernia with or without a sac (Bochdalek-type), parasternal hernia through the foramen of Morgagni, central hernia, and diaphragmatic eventration. The so-called hiatal hernia has a diff

  6. Auricular reconstruction of congenital microtia: personal experience in 225 cases.

    Science.gov (United States)

    Anghinoni, M; Bailleul, C; Magri, A S

    2015-06-01

    Microtia is a congenital disease with various degrees of severity, ranging from the presence of rudimentary and malformed vestigial structures to the total absence of the ear (anotia). The complex anatomy of the external ear and the necessity to provide good projection and symmetry make this reconstruction particularly difficult. The aim of this work is to report our surgical technique of microtic ear correction and to analyse the short and long term results. From 2000 to 2013, 210 patients affected by microtia were treated at the Maxillo-Facial Surgery Division, Head and Neck Department, University Hospital of Parma. The patient population consisted of 95 women and 115 men, aged from 7 to 49 years. A total of 225 reconstructions have been performed in two surgical stages basing of Firmin's technique with some modifications and refinements. The first stage consists in fabrication and grafting of a three-dimensional costal cartilage framework. The second stage is performed 5-6 months later: the reconstructed ear is raised up and an additional cartilaginous graft is used to increase its projection. A mastoid fascial flap together with a skin graft are then used to protect the cartilage graft. All reconstructions were performed without any major complication. The results have been considered satisfactory by all patients starting from the first surgical step. Low morbidity, the good results obtained and a high rate of patient satisfaction make our protocol an optimal choice for treatment of microtia. The surgeon's experience and postoperative patient care must be considered as essential aspects of treatment. PMID:26246664

  7. Key aspects congenital infection

    Directory of Open Access Journals (Sweden)

    Yu. V. Lobzin

    2014-01-01

    Full Text Available The key questions to solve the problem of congenital infection in the Russian Federation are: using in national practice over world accepted terminology adapted to the recommendations of the World Health Organization; representation of the modern concepts of an infectious process in the classification of congenital infections; scientific development and introducing in clinical practice the «standard case definitions», applied to different congenital infections; optimization of protocols and clinical guidelines for diagnosis, treatment and prevention of congenital infections; improvement a knowledge in the infectious disease for all  pecialists involved in the risk assessment of congenital infections, manage pregnancy and children. Based on our experience and analysis of publications, the authors suggest possible solutions.

  8. Clinical experiences of delayed primary anastomosis for type I congenital esophageal atresia%Ⅰ型食管闭锁延期根治手术临床经验总结

    Institute of Scientific and Technical Information of China (English)

    沈淳; 郑珊; 李凯; 刘小舟; 朱海涛; 孙松; 肖现民

    2014-01-01

    Objective To summarize our experiences of delayed primary anastomosis for type Ⅰcongenital esophageal atresia.Methods A retrospective review was performed for 7 patients with typeⅠ congenital esophageal atresia.There were 5 females and 2 males with a birth weight of 2.170 to 2. 615 kilograms.All had a prenatal diagnosis of hydramnios.And esophageal atresia was suspected in 5 cases by ultrasonic examinations.There were congenital heart disease (n=4)and congenital anal-recta malformation (n=2).Gastrostomy was performed after birth and the gap between proximal and distal esophagus measured radiologically. Second surgery was performed by end-to-end esophageal anastomosis at weeks 8-16.The correlation was analyzed between the growth body weight and the growth of blind esophagus during delayed operation period.The intra-operative blind end distance was observed and different operative approaches were selected. Then follow-ups were conducted for postoperative complications,long-term growth and development,including feedings.Results During 8-16 weeks,7 cases grew well with an average growth weight of 2.98 kg.The esophageal growth of two blind sides was 1-2.5 cm.Esophageal growth length and weight gain had no significant correlation (R=0.171,P=0.713).And there was no obvious correlation with two surgical intervals (R=0. 084,P=0.084).During second surgery,after a full separation of two blind esophageal sides,the gap was 3个椎体;生后8~16周行经胸食管-食管端端吻合术。分析延期手术食管断端生长与体质量的相关性;观察术中断端距离与采用不同手术方式的判断;随访术后并发症和生长发育、喂养情况。结果7例8~16周体质量平均增长2.98 kg,造影示两断端生长1.0~2.5 cm,生长长度与体质量增长无明显相关性(R=0.171,P=0.713);与两次手术间隔无明显相关性(R=0.084,P=0.858)。根治手术充分游离两断端,3例距离<1.0 cm者完成食管-食管端端吻合;3例距离1

  9. Congenital hypothyroidism in Calabria: epidemiological and clinical aspects

    Directory of Open Access Journals (Sweden)

    Mariangiola Baserga

    2009-12-01

    Full Text Available Neonatal screening of congenital hypothyroidism (CH, carried out on all newborns 3rd -5th day of life, has a very important priority because the incidence of this disease is the highest of all congenital diseases involving brain damage which can be preventable with early specific treatment. In recent years, the values of TSH that were considered pathological were modified and the TSH cut-off, the title of hormone to be taken as the limit of significance for determining a subject suffering from CH, has been progressively lowered. In Calabria the introduction of the new value of TSH cut-off on blood spot, has led to a considerable increase in the frequency of CH, particularly in the case of the thyroid in situ normal ultrasound and of the diagnosis of transitional forms of CH.

  10. Congenital adrenal hyperplasia, CYP21 deficiency, screening and clinical aspects

    OpenAIRE

    Nordenström, Anna

    2001-01-01

    Congenital adrenal hyperplasia (CAH) is a group of recessively inherited disorders. More than 90% of all cases of CAB are caused by 21-hydroxylase deficiency. This enzyme deficiency results in reduced ability to synthesize cortisol and aldosterone and at the same time increased secretion of androgens. There is a wide spectrum of severity of the disease. The most severe forms of CAH are life-threatening, with the risk of a salt crisis in the neonatal period. CAH has special i...

  11. Ocular manifestation of congenital toxoplasmosis, clinical implication - case report.

    Science.gov (United States)

    Modrzejewska, Monika; Patalan, Jacek; Kulik, Urszula; Czeszyńska, Maria Beata

    2016-01-01

    The aim of this case report was to present extremely severe, ophthalmic complications in form of rare, congenital toxoplasmatic bilateral defect of eye-balls concomitant with advanced uveitis, microphthalmia and eye-multistructural developmental abnormalities leading to irreversible visual disability. The ocular diagnosis was confirmed in Ret-Cam II and ultrasonography and it was accompanied with congenital multiorgan lesions including hepato-splenomegaly, thrombocytopenia, leukomalacia, hydrocephalus and ventriculomegaly with neurological symptoms. Serology, PCR of cerebro-spinal fluid and cord blood confirmed the presence of congenital Toxoplasma gondii infection in the infant. The authors took the effort of insightful analysis for the causes of applied treatment failure in mother during pregnancy, analyzing the inefficacy of Spiromycin therapy in pregnant woman and evaluating false-negative result of amniocentesis for Toxoplasma gondii presence. Among many issues concerning anti-toxoplasmatic treatment in mother and infant presented in this article, the need for multiple repetition of toxoplasmatic tests should be underlined including amniotic fluid PCR and ultrasonography which can add much important data for correct diagnosis. The authors indicate that the lack of benefits from conservative therapy in case of suspected Toxopalsma gondii suggestion lead to dramatic multiorgan complications, especially ophthalmo-neurologic, leading to irreversible visual disability. PMID:27306134

  12. Outcomes of Congenital Cystic Lung Malformations in Children and Their Management According to Clinical Progression

    Directory of Open Access Journals (Sweden)

    Galyagina N.А.

    2014-06-01

    Full Text Available The aim of the investigation was to analyze the outcomes of congenital cystic lung malformations in children in accordance with clinical manifestations in pre- and postnatal period, and justify the management of patients. Materials and Methods. We analyzed retrospectively 45 cases of congenital cystic lung malformations treated over a period from 2005 to 2012 in two main children hospitals in Nizhny Novgorod. Results. Congenital cystic adenomatoid malformation (CCAM is the most common congenital cystic lesion of the lung (60%. Nearly half children with this pathology have no respiratory signs in neonatal period, and in 22.2% cases there are no manifestations in early childhood either. Three cases were found to have spontaneous regression of CCAM. Cystic adenomatoid malformation and solitary congenital cysts have the most favorable prognosis if prenatal lung lesions are early detected and there are no associated congenital malformations. Only 13% children (28.8% required surgical resection of cystic lung malformations. The survival rate of children with cystic lung malformations after surgery was 92.3%. Conclusion. Favorable outcomes of pregnancies with cystic lung diseases constituted the majority (86.7% of cases. Operative treatment is indicated if there are respiratory failure signs or recurrent respiratory infections.

  13. Congenital portosystemic shunts: Imaging findings and clinical presentations in 11 patients

    Energy Technology Data Exchange (ETDEWEB)

    Konstas, Angelos A., E-mail: akonstas@partners.org [Department of Radiology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit St, Boston, MA 02114 (United States); Digumarthy, Subba R.; Avery, Laura L. [Department of Radiology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit St, Boston, MA 02114 (United States); Wallace, Karen L. [Department of Radiology, Mount Auburn Hospital and Harvard Medical School, 330 Mount Auburn St, Cambridge, MA 02138 (United States); Lisovsky, Mikhail; Misdraji, Joseph [Department of Pathology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit St, Boston, MA 02114 (United States); Hahn, Peter F. [Department of Radiology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit St, Boston, MA 02114 (United States)

    2011-11-15

    Objective: To evaluate the clinical anatomy and presentations of congenital portosystemic shunts, and determine features that promote recognition on imaging. Materials and methods: Institutional review board approval was obtained for this HIPAA-compliant study. The requirement for written informed consent was waived. Radiology reports were retrospectively reviewed from non-cirrhotic patients who underwent imaging studies from January 1999 through February 2009. Clinical sources reviewed included electronic medical records, archived images and histopathological material. Results: Eleven patients with congenital portosystemic shunts were identified (six male and five female; age range 20 days to 84 years). Seven patients had extrahepatic and four patients had intrahepatic shunts. All 11 patients had absent or hypoplastic intrahepatic portal veins, a feature detected by CT and MRI, but not by US. Seven patients presented with shunt complications and four with presentations unrelated to shunt pathophysiology. Three adult patients had four splenic artery aneurysms. Prospective radiological evaluation of five adult patients with cross-sectional imaging had failed prospectively to recognize the presence of congenital portosystemic shunts on one or more imaging examinations. Conclusions: Congenital portosystemic shunts are associated with splenic artery aneurysms, a previously unrecognized association. Portosystemic shunts were undetected during prospective radiologic evaluation in the majority of adult patients, highlighting the need to alert radiologists to this congenital anomaly.

  14. Transfer to Adult Care--Experiences of Young Adults with Congenital Heart Disease.

    Science.gov (United States)

    Asp, Ann; Bratt, Ewa-Lena; Bramhagen, Ann-Cathrine

    2015-01-01

    More than 90% of children born with congenital heart disease survive into adulthood due to successes of cardiac surgery and medical management. Interviews with 16 young adults with congenital heart disease to explore their experiences of transfer from pediatric to adult care were performed. The analysis identified five themes; Feeling secure during the transfer process, Experiencing trust in the care, Expecting to be involved, Assuming responsibility for one's health is a process and Lack of knowledge leads to uncertainty. In conclusion; a structured and gradual transfer process was necessary to enable the informants to shoulder the responsibility for self-care.

  15. Experience of a single center with congenital hepatic fibrosis:A review of the literature

    Institute of Scientific and Technical Information of China (English)

    Ali; Shorbagi; Yusuf; Bayraktar

    2010-01-01

    Congenital hepatic fibrosis(CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts.It is one of the fibropolycystic diseases,which also include Caroli disease,autosomal dominant polycystic kidney disease,and autosomal recessive polycystic kidney disease. Clinically it is characterized by hepatic fibrosis,portal hypertension,and renal cystic disease.CHF is known to occur in association with a ran...

  16. 2013 update on congenital heart disease, clinical cardiology, heart failure, and heart transplant.

    Science.gov (United States)

    Subirana, M Teresa; Barón-Esquivias, Gonzalo; Manito, Nicolás; Oliver, José M; Ripoll, Tomás; Lambert, Jose Luis; Zunzunegui, José L; Bover, Ramon; García-Pinilla, José Manuel

    2014-03-01

    This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters in assessing the systemic right ventricle, problems in patients with repaired tetralogy of Fallot and indication for pulmonary valve replacement, and confirmation of the role of specific factors in the selection of candidates for Fontan surgery. The most recent publications in clinical cardiology include a study by a European working group on correct diagnostic work-up in cardiomyopathies, studies on the cost-effectiveness of percutaneous aortic valve implantation, a consensus document on the management of type B aortic dissection, and guidelines on aortic valve and ascending aortic disease. The most noteworthy developments in heart failure and transplantation include new American guidelines on heart failure, therapeutic advances in acute heart failure (serelaxin), the management of comorbidities such as iron deficiency, risk assessment using new biomarkers, and advances in ventricular assist devices.

  17. Congenital cytomegalovirus infection in pregnancy: a review of prevalence, clinical features, diagnosis and prevention.

    Science.gov (United States)

    Naing, Zin W; Scott, Gillian M; Shand, Antonia; Hamilton, Stuart T; van Zuylen, Wendy J; Basha, James; Hall, Beverly; Craig, Maria E; Rawlinson, William D

    2016-02-01

    Human cytomegalovirus (CMV) is under-recognised, despite being the leading infectious cause of congenital malformation, affecting ~0.3% of Australian live births. Approximately 11% of infants born with congenital CMV infection are symptomatic, resulting in clinical manifestations, including jaundice, hepatosplenomegaly, petechiae, microcephaly, intrauterine growth restriction and death. Congenital CMV infection may cause severe long-term sequelae, including progressive sensorineural hearing loss and developmental delay in 40-58% of symptomatic neonates, and ~14% of initially asymptomatic infected neonates. Up to 50% of maternal CMV infections have nonspecific clinical manifestations, and most remain undetected unless specific serological testing is undertaken. The combination of serology tests for CMV-specific IgM, IgG and IgG avidity provide improved distinction between primary and secondary maternal infections. In pregnancies with confirmed primary maternal CMV infection, amniocentesis with CMV-PCR performed on amniotic fluid, undertaken after 21-22 weeks gestation, may determine whether maternofetal virus transmission has occurred. Ultrasound and, to a lesser extent, magnetic resonance imaging are valuable tools to assess fetal structural and growth abnormalities, although the absence of fetal abnormalities does not exclude fetal damage. Diagnosis of congenital CMV infection at birth or in the first 3 weeks of an infant's life is crucial, as this should prompt interventions for prevention of delayed-onset hearing loss and neurodevelopmental delay in affected infants. Prevention strategies should also target mothers because increased awareness and hygiene measures may reduce maternal infection. Recognition of the importance of CMV in pregnancy and in neonates is increasingly needed, particularly as therapeutic and preventive interventions expand for this serious problem. PMID:26391432

  18. Coil embolization of a congenital intrahepatic arterioportal fistula: increasing experience in management

    International Nuclear Information System (INIS)

    Congenital intrahepatic arterioportal fistula (IAPF) is a rare condition and there is limited experience of transcatheter embolization. We report here the transcatheter coil embolization of an incidentally found, asymptomatic congenital IAPF in a 16-month-old patient. After demonstrating that the IAPF was fed by two branches of a dilated left hepatic artery and drained into an aneurysm of the left portal vein, the feeding arteries were superselectively catheterized and occluded by microcoils in a single session. Follow-up was uneventful for 1 year. With respect to this and previously reported cases, we believe that embolization of feeding arteries is a safe and effective method that should be the primary choice of treatment; however, further cases should be reported to confirm this belief. (orig.)

  19. Interruption or congenital stenosis of the inferior vena cava: Prevalence, imaging, and clinical findings

    Energy Technology Data Exchange (ETDEWEB)

    Koc, Zafer [Baskent University, School of Medicine, Department of Radiology, Adana (Turkey)]. E-mail: koczafer@gmail.com; Oguzkurt, Levent [Baskent University, School of Medicine, Department of Radiology, Adana (Turkey)

    2007-05-15

    Objective: To present the prevalence, clinical, and imaging findings of interruption or congenital stenotic lesions of the inferior vena cava (IVC), associated malformations, and their clinical relevance. Materials and methods: Between March 2004 and March 2006, 7972 patients who had undergone consecutive routine abdominal multidetector row computed tomography were analyzed for interruption or stenotic lesion of the IVC. Results: Prevalence of interruption (n = 8) or congenital stenosis (n = 4) of the IVC occurred in 12 (0.15%) of 7972 patients. Four patients with interruption and four patients with congenital stenosis of the IVC were symptomatic with DVT (n = 4), leg swelling (n = 4), leg pain (n = 2), lower extremity varices (n = 2), hepatic vein thrombosis (n = 1), and hematochezia (n = 1). All four of the asymptomatic patients were from the interruption group, and these patients had interrupted IVC with well-developed azygos/hemiazygos continuation. Eight symptomatic patients did not have a well-developed azygos/hemiazygos continuation, and drainage of lower extremity was mainly from collateral veins. Additional findings in eight symptomatic patients were abdominal venous collaterals (n = 8), venous aneurysm (n = 2), lower extremity varices (n = 2), varicocele (n = 2), and pelvic varices (n = 1). Conclusion: Interruption or stenosis of the IVC are rare on routine abdominal CT examinations and may cause different clinical findings depending on the variant drainage patterns or collaterals. Interrupted IVC is commonly asymptomatic if associated with well-developed azygos/hemiazygos continuation, whereas commonly symptomatic if well-developed azygos/hemiazygos continuation is not present.

  20. On writing from clinical experience.

    Science.gov (United States)

    Scharff, J S

    2000-01-01

    Papers that present the life of the analytic session offer material through which analysts can together study analytic process and therapeutic action and arrive at consensus on how to improve psychoanalytic theory and practice. But some analysts have been deterred from publishing clinical material of that kind because of concerns about preserving confidentiality, protecting the therapeutic relationship, reporting accurately, being scrutinized, worrying about losing their colleagues' support, and not feeling authorized to present their views. Here conscious, preconscious, and unconscious constraints against writing and publishing are explored, and an example is given of successful self-analysis of a writing inhibition. The debate over the ethics of writing is reviewed and an argument made that detailed clinical description is useful in advancing analytic understanding. Finally, a clinical example shows how the analysand usefully analyzes the experience of reading what the analyst has written, and how the analyst's self-analysis may be promoted in resonance with the analysand's experience.

  1. Clinical and molecular characterization of two patients with palmoplantar keratoderma-congenital alopecia syndrome type 2.

    Science.gov (United States)

    Castori, M; Morlino, S; Sana, M E; Paradisi, M; Tadini, G; Angioni, A; Malacarne, M; Grammatico, P; Iascone, M; Forzano, F

    2016-08-01

    Palmoplantar keratoderma-congenital alopecia (PPKCA) syndrome is a rare genodermatosis, with two clinically recognizable forms: dominant (Type 1) and recessive (Type 2). Reports of only 18 patients have been published to date, and the molecular basis of the condition is unknown. We describe two cases with PPKCA Type 2 (PPKCA2), comprising a novel patient, originally reported as an example of autosomal ichthyosis follicularis-atrichia-photophobia syndrome, and the 6-year follow-up of a previously published case. Extensive molecular studies of both patients excluded mutations in all the known genes associated with PPK and partially overlapping syndromes. The striking similarities between these two patients confirm PPKCA2 as a discrete genodermatosis, of which the main features are congenital and universal alopecia, diffuse keratosis pilaris, facial erythema, and a specific PPK with predominant involvement of the fingertips and borders of the hands and feet, with evolution of sclerodactyly, contractures and constrictions. Clinical follow-up of these patients has demonstrated progressive worsening of the hand involvement and attenuation of facial erythema. PMID:27339777

  2. Low-dose fetal CT for evaluation of severe congenital skeletal anomalies: preliminary experience

    Energy Technology Data Exchange (ETDEWEB)

    Victoria, Teresa; Epelman, Monica; Johnson, Ann M.; Kramer, Sandra; Jaramillo, Diego [Children' s Hospital of Philadelphia, Diagnostic Imaging, Philadelphia, PA (United States); Bebbington, Michael [Children' s Hospital of Philadelphia, Center for Fetal Diagnosis and Treatment, Philadelphia, PA (United States); Wilson, R.D. [University of Calgary, Obstetrics and Gynecology, Calgary (Canada)

    2012-01-15

    Congenital skeletal abnormalities compose a heterogeneous and complex group of conditions that affect bone growth and development and result in various anomalies in shape and size of the skeleton. Prenatal sonographic diagnosis of these anomalies is challenging because of the relative rarity of each skeletal dysplasia, the multitude of differential diagnoses encountered when the bony abnormalities are identified, lack of precise molecular diagnosis and the fact that many of these disorders have overlapping features and marked phenotypic variability. The following review is a preliminary summary of our experience at the Children's Hospital of Philadelphia (CHOP) using low-dose fetal CT in the evaluation of severe fetal osseous abnormalities. (orig.)

  3. Population-based evaluation of a suggested anatomic and clinical classification of congenital heart defects based on the International Paediatric and Congenital Cardiac Code

    Directory of Open Access Journals (Sweden)

    Goffinet François

    2011-10-01

    Full Text Available Abstract Background Classification of the overall spectrum of congenital heart defects (CHD has always been challenging, in part because of the diversity of the cardiac phenotypes, but also because of the oft-complex associations. The purpose of our study was to establish a comprehensive and easy-to-use classification of CHD for clinical and epidemiological studies based on the long list of the International Paediatric and Congenital Cardiac Code (IPCCC. Methods We coded each individual malformation using six-digit codes from the long list of IPCCC. We then regrouped all lesions into 10 categories and 23 subcategories according to a multi-dimensional approach encompassing anatomic, diagnostic and therapeutic criteria. This anatomic and clinical classification of congenital heart disease (ACC-CHD was then applied to data acquired from a population-based cohort of patients with CHD in France, made up of 2867 cases (82% live births, 1.8% stillbirths and 16.2% pregnancy terminations. Results The majority of cases (79.5% could be identified with a single IPCCC code. The category "Heterotaxy, including isomerism and mirror-imagery" was the only one that typically required more than one code for identification of cases. The two largest categories were "ventricular septal defects" (52% and "anomalies of the outflow tracts and arterial valves" (20% of cases. Conclusion Our proposed classification is not new, but rather a regrouping of the known spectrum of CHD into a manageable number of categories based on anatomic and clinical criteria. The classification is designed to use the code numbers of the long list of IPCCC but can accommodate ICD-10 codes. Its exhaustiveness, simplicity, and anatomic basis make it useful for clinical and epidemiologic studies, including those aimed at assessment of risk factors and outcomes.

  4. Congenital Anomalies in Infant with Congenital Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Zahra Razavi

    2012-09-01

    Full Text Available bjective: Congenital hypothyroidism is characterized by inadequate thyroid hormone production in newborn infants. Many infants with CH have co-occurring congenital malformations. This is an investigation on the frequency and types of congenital anomalies in infants with congenital hypothyroidism born from May 2006-2010 in Hamadan, west province of Iran.Methods: The Iranian neonatal screening program for congenital hypothyroidism was initiated in May 2005. This prospective descriptive study was conducted in infants diagnosed with congenital hypothyroidism being followed up in Pediatric Endocrinology Clinicof Besat Hospital, a tertiary care centre in Hamadan. Cases included all infants with congenital hypothyroidism diagnosed through newborn screening program or detected clinically. Anomalies were identified by clinical examination, echocardiography, and X-ray of the hip during the infant’s first year of life.Results: A total of 150 infants with biochemically confirmed primary congenital hypothyroidism (72 females and 78 males were recruited during the period between May 2006-2010. Overall, 30 (20% infants had associated congenital anomalies. The most common type of anomaly was Down syndrome. Seven infants (3.1% had congenital cardiac anomalies such as: ASD (n=3, VSD (n=2, PS (n =1, PDA (n=1. Three children (2.6% had developmental displasia of the hip (n=3.Conclusion: The overall frequency of Down syndrome, cardiac malformation and other birth defect was high in infants with CH. This reinforces the need to examine all infants with congenital hypothyroidism for the presence of associated congenital anomalies.

  5. Psychophysical measures of visual function and everyday perceptual experience in a case of congenital stationary night blindness.

    Science.gov (United States)

    Cammack, Jocelyn; Whight, John; Cross, Vinette; Rider, Andrew T; Webster, Andrew R; Stockman, Andrew

    2016-01-01

    An appreciation of the relation between laboratory measures of visual deficit and everyday perceptual experience is fundamental to understanding the impact of a visual condition on patients and so to a fuller characterization of the disorder. This study aims to understand better the interpretative processes by which modified sensory information is perceived by a patient with congenital stationary night blindness and the adaptive strategies that are devised to deal with their measurable visual loss. Psychophysical measurements of temporal resolution, spectral sensitivity, and color discrimination were conducted on a 78-year-old male patient with the condition, who was also interviewed at length about the ways in which his diagnosis affected his daily life. Narrative analysis was employed to identify the relation between his subjective perceptual experiences and functional deficits in identifiable components of the visual system. Psychophysical measurements indicated a complete lack of rod perception and substantially reduced cone sensitivity. Two particular effects of this visual loss emerged during interviews: 1) the development of navigational techniques that relied on light reflections and point sources of light and 2) a reluctance to disclose the extent of visual loss and resulting lifelong psychosocial consequences. This study demonstrates the valuable complementary role that rich descriptive patient testimony can play, in conjunction with laboratory and clinical measurements, in more fully characterizing a disorder and in reaching a more complete understanding of the experience of vision loss. It also evidences the particular suitability of filmmaking techniques as a means of accessing and communicating subjective patient experience. PMID:27601873

  6. Congenital bronchopulmonary malformations: A single-center experience and a review of literature

    Directory of Open Access Journals (Sweden)

    Kumar Basant

    2008-01-01

    Full Text Available Purpose: To present a single-center experience with 25 cases of bronchopulmonary malformations and the review the literature. Materials and Methods: We conducted a retrospective analysis of the medical records of patients with congenital bronchopulmonary malformations who were operated between July 1997 and July 2007 in our institute; we examined the modes of presentations, management, and outcome. Outcome of all patients was assessed over a short follow-up period (average 1.8 months. Results: Out of 25 patients, 18 (72% were male and 7 (28% were female. Age of patients ranged from 1 day to 11 years. The histopathological diagnosis was congenital cystic adenomatoid malformations [CCAM; n = 14 (56%], congenital lobar emphysema [CLE; n = 5 (20%], pulmonary sequestrations [PS; n = 3 (12%], and bronchogenic cysts [BC; n = 3 (12%]. Antenatal diagnosis was available in only 2 (8% patients. The common presenting symptoms were respiratory distress and chest infections. Lobectomy was the procedure of choice . Mortality was 16% (n = 4; M: F = 3: 1. Two patients died because of overwhelming sepsis, one from compromised cardiac function, and one from aspiration which might possibly have been prevented. Conclusion: Patients with progressive respiratory distress due to these anomalies may require urgent surgical intervention regardless of age. The surgical outcome is favorable, with manageable complications. Plain x-ray chest and CT of thorax are usually sufficient for diagnosis and planning of treatment. Pathological diagnosis may differ from the imaging diagnosis. Mortality is found to be more in neonates. Apart from initial stabilization, resection of lesion and careful postoperative care is necessary to reduce mortality and morbidity.

  7. Thromboprophylaxis and Outcomes for Total Joint Arthroplasty in Congenital Bleeding Disorders: A Single-Center Experience.

    Science.gov (United States)

    Holderness, Britt M; Goto, Yuika; McKernan, Laurel; Bernini, Philip; Ornstein, Deborah L

    2016-09-01

    Total joint arthroplasty (TJA) improves the quality of life for patients with end-stage osteoarthritis but is associated with an increased risk of venous thromboembolism (VTE), thus pharmacologic thromboprophylaxis is recommended for most patients. Patients with congenital bleeding disorders may develop severe arthropathies due to repeated hemarthroses and derive similar benefit from TJA as the general population. No guidelines for pharmacologic thromboprophylaxis in this population exist, however, as the risks and benefits are not well defined. We undertook the current study to assess the safety and efficacy of pharmacologic VTE prophylaxis in patients with congenital bleeding disorders undergoing TJA. We retrospectively reviewed the medical records of patients with bleeding disorders who underwent TJA at our academic institution between 1987 and 2012. We identified 28 patients who underwent 38 TJA procedures. Low-molecular-weight heparin (LMWH) was administered in 29 procedures (76%) and was discontinued early in 3 procedures (2 patients) due to nonjoint bleeding. No symptomatic VTE was identified, and no joint or deep wound infections were seen. Twenty-two patients accounting for 31 procedures were contacted to discuss their experience with TJA. All reported decreased pain, and 97% reported improved function after the surgery. Impressively, 97% stated that they would choose to have the surgery again. These results confirm the benefit of TJA in patients with congenital bleeding disorders and end-stage arthropathies and suggest that LMWH thromboprophylaxis is safe. No patient in our cohort developed symptomatic VTE, whether or not thromboprophylaxis was administered, thus necessity of thromboprophylaxis remains an unanswered question. PMID:27099385

  8. Defense mechanisms in congenital and acquired facial disfigurement: a clinical-empirical study.

    Science.gov (United States)

    van den Elzen, Marijke E P; Versnel, Sarah L; Perry, J Christopher; Mathijssen, Irene M J; Duivenvoorden, Hugo J

    2012-04-01

    It is of clinical interest to investigate the degree to which patients with facial disfigurement use defense styles. Therefore, 59 adults born with rare facial clefts, 59 patients with facial deformities acquired at an adult age, and a reference group of 141 adults without facial disfigurements completed standardized questionnaires. There was a significant difference between the group with and the group without disfigurements on immature defense styles, with the disfigured group using the immature style more frequently. There was a trend for the nondisfigured group to use more mature defense styles. No difference between congenital and acquired groups was seen on individual types of defense style. Self-esteem had the strength to differentiate mature and immature defense styles within our disfigured groups. The association of low self-esteem and the utilization of immature defense styles suggests that professional help may tailor treatment on discussing immature defense style and problems triggering or maintaining this style. PMID:22456586

  9. Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Hye Jin; Kim, Woo Sun; Cheon, Jung-Eun; Shin, Su-Mi; Kim, In-One; Yeon, Kyung Mo [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Yoo, So-Young [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea); Park, Kwi-Won; Jung, Sung-Eun [Seoul National University Children' s Hospital, Department of Pediatric Surgery, Seoul (Korea)

    2010-08-15

    Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF. To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula. We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children. CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months. Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed. (orig.)

  10. The clinical experience of cardiopulmonary bypass in pediatric congenital heart disease complicated by infective endocarditis%小儿先心病并感染性心内膜炎手术治疗中的体外循环技术

    Institute of Scientific and Technical Information of China (English)

    汪力; 皮名安; 龚立; 王涛

    2011-01-01

    目的 总结2002年1月至2011年4月作者收治的15例先天性心脏病合并感染性心内膜炎( infective endocarditis,IE)患儿手术治疗中的体外循环经验(cardiopulmonary bypass,CPB).方法 15例患儿均采用浅低温(28℃~30℃)、较高流量[2.4~3.2 L/(min·m2)]灌注.手术方式为畸形矫治(ASD/VSD修补、右室流出道重建)、赘生物摘除、瓣膜整形或置换术.心肌保护采用4℃冷含血晶体保护液顺行灌注.结果 15例患儿CPB总时间85~ 180 min,主动脉阻断时间40~120 min,均顺利脱离CPB.均获痊愈.1例术后2年死于肺部感染及心功能衰竭.结论 CPB中HCT及胶体渗透压的良好控制、采用平衡超滤结合改良超滤、肝素化后监测ACT、CPB中加入敏感抗生素等对患儿术后恢复至关重要.%Objective To summarize retrospectively the clinical experience of cardiopulmonary bypass (CPB)in pediatric congenital heart disease (CHD) complicated by infective endocarditis (IE) from January 2002 to April 2011(15 cases). Methods CPB was performed with Shallow low temperature(28℃ ~30℃) and high flow rate [2. 4 ~3.21/(min·M2) ] perfusion. Operation method for Correcting the heart abnormality (ASD/VSD repaired., Right ventricular outflow reconstructed), excrescence excision, valve plastic or replacement. 4℃ cold crystalloid with blood was perfused by antegrade perfusion for myocardial protection. Result-s Among the total time CPB 85 ~ 180 min, aortic blocking time 40 ~ 120 min, 15 cases of patients were smoothly disconnected from CPB. All cases recovered completely. 1 case died in lyear due to heart failure. Conclusion The important factors in CPB are the good adjustment in colloid osmotic pressure( COP) and hem-atocrit( HCT) ,Balance ultrafiltration( BUF) and modified ultrafiltration( MUF) .monitor of activated clotting time (ACT) .sensitive antibiotics used in priming liquid.

  11. Sociodemographic and clinical profile of children with congenital heart disease assisted at a hospital institution

    Directory of Open Access Journals (Sweden)

    Mirna Albuquerque Frota

    2014-06-01

    Full Text Available Objective: To characterize the sociodemographic and clinical profile of children with congenital heart disease assisted at a hospital institution. Methods: A descriptive, crosssectional study conducted in the pediatric cardiology outpatient clinic of a hospital in Fortaleza, CE, Brazil, in the period from March to August 2012. It comprised a nonrandom convenience sample of 80 parents of children with heart disease aged 5 to 12 years. Information was obtained from primary data of medical records and through questionnaires covering the parent’s sociodemographic characteristics, family data and the children’s clinical aspects. Results: Regarding socioeconomic data, 77 (96.2% caregivers were female, 50 (62.5% were married or in a stable relationship, 39 (48.7% had 10-12 years of formal education, and 69 (82.6% reported a household income between 1 and 2 minimum wages. In the sample of children studied, 43 (53.8% were female. The median age of the children was 8.4 years, ranging from 5-13 years. As to the age at the heart disease diagnosis, 44 (55% occurred under the age of 6 months, regardless of the type of cardiopathy. It was detected that 19 (23.75% were related to the disease. Conclusion: Children’s caregivers were predominantly their mothers, with low income and satisfactory schooling. Early diagnosis was predominant, mainly among the children with cyanotic cardiopathy. doi:10.5020/18061230.2014.p239

  12. Determination of the prevalence of congenital heart disease in the patients admitted to the heart clinic

    Institute of Scientific and Technical Information of China (English)

    Shokoufeh Ahmadipour; Behzad Mohammadpour Ahranjani; Sara Daeichin; Zahra Mirbeig Sabzevari

    2015-01-01

    Objective:To investigate the prevalence of congenital heart disease (CHD) among the patients who refferred to the heart clinic so as to make an early and correct diagnosis. Methods: In this descriptive-cross sectional study, all the patients admitted to the heart clinic who had symptoms or signs ofCHDwere included. The data were collected in one year based on the medical records. The main variables consisted of age, gender, history of folic acid consumption by the mother in pregnancy, clinical signs, symptoms and so on. Results: Among the 763 admitted patients, 498 were males and the rest were females. Infants were the most common group and teenagers were the least one. The most common findings for which the patients had been referred were chest pain and a murmur heard during a normal physical examination. Based on the echocardiography findings, ventricular and atrial septal defects were the most common ones. The history of folic acid consumption was negative in 168 mothers within their pregnancy. Conclusions: Since the causes and risk factors in the incidence ofCHD in children are numerous, we recommended that the information about these diseases should be given to the community and strengthen the referral system, design registration system ofCHD set up in the country round.

  13. Congenital Esophageal Atresia Surgery Treatment Experience%先天性食管闭锁外科治疗经验

    Institute of Scientific and Technical Information of China (English)

    侯可峰; 薛枫; 窦欣欣; 王培倩

    2015-01-01

    目的:总结分析先天性食管闭锁的诊断及外科治疗,观察其治疗效果,总结经验。方法对我院2011年1月~2014年11月收治的16例先天性食管闭锁型患儿的临床资料进行回顾分析,对食管闭锁的诊治、术后主要并发症的影响因素及诊断治疗进行分析。结果16例患儿全部康复出院,中仅有1例发生吻合口漏,未见明显吻合口狭窄。结论食管闭锁的分型是影响治疗效果的重要因素,外科吻合技术的提高是手术成功的关键。%Objective To Summarize and analyze the diagnosis and surgical treatment of congenital esophageal atresia, observe the treatment effect, summarize the experience. Methods The clinical data of our hospital in 2011January to 2014 year in November 16 cases of congenital esophageal atresia were analyzed retrospectively, analyzed main inlfuencing operation of esophageal atresia, diagnosis and treatment of complications of diagnostic and treatment factors. Results 16 patients were discharged from the hospital, only 1 cases of anastomotic leakage, no anastomotic stenosis. Conclusion Esophageal atresia type is an important factor to affect the treatment effect, surgical technique is the key to improve the success of operation.

  14. The Burden of Care: Mothers’ Experiences of Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Sakinne Sabzevari

    2016-10-01

    Full Text Available Background: Mothers play a key role in caring for their sick children. Their experiences of care were influenced by culture, rules, and the system of health and care services. There are few studies on maternal care of children with congenital heart disease. Also, each of them has studied a particular aspect of care. The present research aimed to understand care experiences of mothers of children with congenital heart disease. Methods: A conventional content analysis was used to obtain rich data. The goal of content analysis is “to provide knowledge and deeper understanding of the phenomenon under the study”. The study was conducted in Kerman, Iran in 2014, on mothers of children with CHD. The purposive sampling technique was used to select the participants. Participants were 14 mothers of children with CHD and one father and one nurse of open heart surgery unit, from two hospitals affiliated with Kerman University of Medical Sciences. Eighteen semi-structured interviews were constructed. Data were analyzed using conventional content analysis. MAXQDA 2007 software (VERBI GmbH, Berlin, Germany was used to classify and manage the coding. Constant comparative method was done for data analysis. The reliability and validity of the findings, including the credibility, confirm ability, dependability, and transferability, were assessed. Results: According to the content analysis, the main theme was the catastrophic burden of child care on mothers that included three categories: 1 the tension resulting from the disease, 2 involvement with internal thoughts, and 3 difficulties of care process Conclusion: The results of this study may help health care professionals to provide supportive and educational packages to the patients, mothers and Family members until improving the management of patient’s care.

  15. The Burden of Care: Mothers’ Experiences of Children with Congenital Heart Disease

    Science.gov (United States)

    Sabzevari, Sakinne; Nematollahi, Monirsadat; Mirzaei, Tayebeh; Ravari, Ali

    2016-01-01

    ABSTRACT Background: Mothers play a key role in caring for their sick children. Their experiences of care were influenced by culture, rules, and the system of health and care services. There are few studies on maternal care of children with congenital heart disease. Also, each of them has studied a particular aspect of care. The present research aimed to understand care experiences of mothers of children with congenital heart disease. Methods: A conventional content analysis was used to obtain rich data. The goal of content analysis is “to provide knowledge and deeper understanding of the phenomenon under the study”. The study was conducted in Kerman, Iran in 2014, on mothers of children with CHD. The purposive sampling technique was used to select the participants. Participants were 14 mothers of children with CHD and one father and one nurse of open heart surgery unit, from two hospitals affiliated with Kerman University of Medical Sciences. Eighteen semi-structured interviews were constructed. Data were analyzed using conventional content analysis. MAXQDA 2007 software (VERBI GmbH, Berlin, Germany) was used to classify and manage the coding. Constant comparative method was done for data analysis. The reliability and validity of the findings, including the credibility, confirm ability, dependability, and transferability, were assessed. Results: According to the content analysis, the main theme was the catastrophic burden of child care on mothers that included three categories: 1) the tension resulting from the disease, 2) involvement with internal thoughts, and 3) difficulties of care process Conclusion: The results of this study may help health care professionals to provide supportive and educational packages to the patients, mothers and Family members until improving the management of patient’s care. PMID:27713900

  16. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease.

    Science.gov (United States)

    Zijlstra, Willemijn M H; Douwes, Johannes M; Ploegstra, Mark-Jan; Krishnan, Usha; Roofthooft, Marcus T R; Hillege, Hans L; Ivy, D Dunbar; Rosenzweig, Erika B; Berger, Rolf M F

    2016-09-01

    Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013). According to this classification, 24% of 134 children were classified into group 1, 14% into group 2, 19% into group 3, and 30% into group 4; 11% could not be classified. Types of CHD and hemodynamic profile differed between groups, with the highest right atrial pressure in group 4 (P classification. Pediatric PAH-CHD is a heterogeneous condition frequently associated with extracardiac, developmental factors that are believed to affect disease development. The Nice CHD classification identifies groups with specific patient/disease characteristics. However, a substantial proportion of children could not be classified. Group 3 forms a distinct disease entity. Its prognostic value could not be determined because of the low number of events. The Nice CHD classification supports clinical characterization of PAH-CHD; however, further refinement is needed to classify all children with PAH-CHD. PMID:27683607

  17. Update Review and Clinical Presentation in Congenital Insensitivity to Pain and Anhidrosis

    Science.gov (United States)

    Pérez-López, L. M.; Cabrera-González, M.; Gutiérrez-de la Iglesia, D.; Ricart, S.; Knörr-Giménez, G.

    2015-01-01

    Introduction. Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV is an extremely rare syndrome. Three clinical findings define the syndrome: insensitivity to pain, impossibility to sweat, and mental retardation. This pathology is caused by a genetic mutation in the NTRK1 gene, which encodes a tyrosine receptor (TrkA) for nerve growth factor (NGF). Methods. The consultation of a child female in our center with CIPA and a tibia fracture in pseudoarthrosis encouraged us to carefully review literature and examine the therapeutic possibilities. A thorough review of literature published in Pubmed was done about CIPA and other connected medical issues mentioned in the paper. Conclusions. The therapeutic approach of CIPA remains unclear. The preventive approach remains the only possible treatment of CIPA. We propose two new important concepts in the therapeutic approach for these patients: (1) early surgical treatment for long bone fractures to prevent pseudoarthrosis and to allow early weight bearing, decreasing the risk of further osteopenia, and (2) bisphosphonates to avoid the progression of osteopenia and to reduce the number of consecutive fractures. PMID:26579324

  18. Tension-type headache as the unique pain experience of a patient with congenital insensitivity to pain.

    Science.gov (United States)

    Danziger, N; Willer, J-C

    2005-10-01

    Congenital insensitivity to pain (CIP) is a rare clinical syndrome characterized by dramatic impairment of pain perception since birth and is generally caused by a hereditary sensory and autonomic neuropathy (HSAN) with loss of the small-calibre, nociceptive nerve fibres. We report the case of a 32-year-old woman with CIP and a presumptive diagnosis of HSAN type V, who experienced physical pain for the first and unique time in her life shortly after the sudden loss of her brother. This patient had sustained innumerable painless injuries during childhood, including bone fractures and severe burns. The only pain she ever felt consisted in an intense headache, which took place in a context of strong emotional overload and anxiety, 3 weeks after her younger brother died suddenly in a car accident. The description of this inaugural episode of headache fulfilled the diagnostic criteria of episodic tension-type headache. This case strongly suggests that the transcription of the grief of bereavement into physical pain may sometimes occur independently of the peripheral mechanisms of nociception and despite the lack of previous pain experience. In the light of recent experimental data showing that the same neural mechanisms that regulate physical pain may also control the expression of separation distress and the feeling of social exclusion, this unique case helps to better understand why some patients may feel physically hurt after the loss of someone they love. PMID:16154693

  19. Maturation and experience in action representation: Bilateral deficits in unilateral congenital amelia.

    Science.gov (United States)

    Philip, B A; Buckon, C; Sienko, S; Aiona, M; Ross, S; Frey, S H

    2015-08-01

    Congenital unilateral absence of the hand (amelia) completely deprives individuals of sensorimotor experiences with their absent effector. The consequences of such deprivation on motor planning abilities are poorly understood. Fourteen patients and matched controls performed two grip selection tasks: 1) overt grip selection (OGS), in which they used their intact hand to grasp a three-dimensional object that appeared in different orientations using the most natural (under-or over-hand) precision grip, and 2) prospective grip selection (PGS), in which they selected the most natural grip for either the intact or absent hand without moving. For the intact hand, we evaluated planning accuracy by comparing concordance between grip preferences expressed in PGS vs. OGS. For the absent hand, we compared PGS responses with OGS responses for the intact hand that had been phase shifted by 180°, thereby accounting for mirror symmetrical biomechanical constraints of the two limbs. Like controls, amelic individuals displayed a consistent preference for less awkward grips in both OGS and PGS. Unexpectedly, however, they were slower and less accurate for PGS based on either the intact or the absent hand. We conclude that direct sensorimotor experience with both hands may be important for the typical development or refinement of effector-specific internal representations of either limb. PMID:26092768

  20. Republication: Two Premature Neonates of Congenital Syphilis with Severe Clinical Manifestations.

    Science.gov (United States)

    Akahira-Azuma, Moe; Kubota, Mai; Hosokawa, Shinichi; Kaneshige, Masao; Yasuda, Noriko; Sato, Noriko; Matsushita, Takeji

    2015-09-01

    Congenital syphilis (CS) is a public health burden in both developing and developed countries. We report two cases of CS in premature neonates with severe clinical manifestations; Patient 1 (gestational age 31 weeks, birth weight 1423 g) had disseminated idiopathic coagulation (DIC) while Patient 2 (gestational age 34 weeks and 6 days, birth weight 2299 g) had refractory syphilitic meningitis. Their mothers were single and had neither received antenatal care nor undergone syphilis screening. Both neonates were delivered via an emergency cesarean section and had birth asphyxia and transient tachypnea of newborn. Physical examination revealed massive hepatosplenomegaly. Laboratory testing of maternal and neonatal blood showed increased rapid plasma reagin (RPR) titer and positive Treponema pallidum hemagglutination assay. Diagnosis of CS was further supported by a positive IgM fluorescent treponemal antibody absorption test and large amounts of T. pallidum spirochetes detected in the placenta. Each neonate was initially treated with ampicillin and cefotaxime for early bacterial sepsis/meningitis that coexisted with CS. Patient 1 received fresh frozen plasma and antithrombin III to treat DIC. Patient 2 experienced a relapse of CS during initial antibiotic treatment, necessitating parenteral penicillin G. Treatment was effective in both neonates, as shown by reductions in RPR. Monitoring of growth and neurological development through to age 4 showed no evidence of apparent delay or complications. Without adequate antenatal care and maternal screening tests for infection, CS is difficult for non-specialists to diagnose at birth, because the clinical manifestations are similar to those of neonatal sepsis and meningitis. Ampicillin was insufficient for treating CS and penicillin G was necessary. PMID:26543391

  1. Clinical countermeasures against congenital heart disease in adults%成人先天性心脏病临床对策

    Institute of Scientific and Technical Information of China (English)

    徐仲英

    2013-01-01

    Congenital heart disease is common among the newborns, and the incidence rate is about 0. 8%. Over the past 30 years,great progress has been made in the diagnosis and treatment of congenital heart disease in children. As a result,many children with such diseases now survive to adulthood. In the United States alone, the population of adults with congenital heart disease, either surgically corrected or uncorrected, is estimated to be increasing at a rate of about 5 percent every year; in 2008 there had be almost 1 million such patients. Currently in China, although there is no statistical data of incidence of adult congenital heart disease,the number of such patients must be huge. This article discusses congenital heart disease from two aspects: acyanotic and cyanotic;and clinical countermeasures against each type will be described.%先天性心脏病比较常见的,在新生儿中发生率约0.8%.在过去的30年,儿童先天性心脏病诊断和治疗取得了巨大进步,许多孩子因此可以存活到成年.仅在美国,经矫治或未矫治的先天性心脏病成人数,预计将以每年约5%的速度增长,2008已达近100万例患者.中国还没有成人先天性心脏病的统计数据,但患者数目必须是巨大的.文章从紫绀型和紫绀型2个方面阐述常见成人先天性心脏病的临床对策.

  2. Genetic and epigenetic factors at COL2A1 and ABCA4 influence clinical outcome in congenital toxoplasmosis.

    Directory of Open Access Journals (Sweden)

    Sarra E Jamieson

    Full Text Available BACKGROUND: Primary Toxoplasma gondii infection during pregnancy can be transmitted to the fetus. At birth, infected infants may have intracranial calcification, hydrocephalus, and retinochoroiditis, and new ocular lesions can occur at any age after birth. Not all children who acquire infection in utero develop these clinical signs of disease. Whilst severity of disease is influenced by trimester in which infection is acquired by the mother, other factors including genetic predisposition may contribute. METHODS AND FINDINGS: In 457 mother-child pairs from Europe, and 149 child/parent trios from North America, we show that ocular and brain disease in congenital toxoplasmosis associate with polymorphisms in ABCA4 encoding ATP-binding cassette transporter, subfamily A, member 4. Polymorphisms at COL2A1 encoding type II collagen associate only with ocular disease. Both loci showed unusual inheritance patterns for the disease allele when comparing outcomes in heterozygous affected children with outcomes in affected children of heterozygous mothers. Modeling suggested either an effect of mother's genotype, or parent-of-origin effects. Experimental studies showed that both ABCA4 and COL2A1 show isoform-specific epigenetic modifications consistent with imprinting. CONCLUSIONS: These associations between clinical outcomes of congenital toxoplasmosis and polymorphisms at ABCA4 and COL2A1 provide novel insight into the molecular pathways that can be affected by congenital infection with this parasite.

  3. Congenital syphilis in the newborn.

    OpenAIRE

    Chawla, V.; Pandit, P B; Nkrumah, F K

    1988-01-01

    We studied 53 newborn babies with congenital syphilis. The common clinical features seen were low birth weight, hepatosplenomegaly, anaemia, jaundice, and symmetrical superficial desquamation of the skin affecting palms and soles. The presence of these clinical signs is highly suggestive of early congenital syphilis. Hydrops fetalis without rhesus or ABO isoimmunisation should always arouse the suspicion of congenital syphilis.

  4. [Congenital myasthenic syndromes: difficulties in the diagnosis, course and prognosis, and therapy--The French National Congenital Myasthenic Syndrome Network experience].

    Science.gov (United States)

    Eymard, B; Stojkovic, T; Sternberg, D; Richard, P; Nicole, S; Fournier, E; Béhin, A; Laforêt, P; Servais, L; Romero, N; Fardeau, M; Hantaï, D

    2013-02-01

    Congenital myasthenic syndromes (CMS) are a heterogeneous group of disorders caused by genetic defects affecting neuromuscular transmission and leading to muscle weakness accentuated by exertion. Three different aspects have been investigated by members of the national French CMS Network: the difficulties in making a proper diagnosis; the course and long-term prognosis; and the response to therapy, especially for CMS that do not respond to cholinesterase inhibitors. CMS diagnosis is late in most cases because of confusion with other entities such as: congenital myopathies, due to the frequent presentation in patients of myopathies such as permanent muscle weakness, atrophy and scoliosis, and the abnormalities of internal structure, diameter and distribution of fibers (type I predominance, type II atrophy) seen on biopsy; seronegative autoimmune myasthenia gravis, when CMS is of late onset; and metabolic myopathy, with the presence of lipidosis in muscle. The long-term prognosis of CMS was studied in a series of 79 patients recruited with the following gene mutations: CHRNA; CHRNE; DOK7; COLQ; RAPSN; AGRN; and MUSK. Disease-course patterns (progressive worsening, exacerbation, stability, improvement) could be variable throughout life in a given patient. DOK7 patients had the most severe disease course with progressive worsening: of the eight wheelchair-bound and ventilated patients, six had mutations of this gene. Pregnancy was a frequent cause of exacerbation. Anticholinesterase agents are the first-line therapy for CMS patients, except for cases of slow-channel CMS, COLQ and DOK7. In our experience, 3,4-DAP was a useful complement for several patients harboring CMS with AChR loss or RAPSN gene mutations. Ephedrine was given to 18 patients (eight DOK7, five COLQ, four AGRN and one RAPSN). Tolerability was good. Therapeutic responses were encouraging even in the most severely affected patients, particularly with DOK7 and COLQ. Salbutamol was a good alternative in

  5. Validity of Sildenafil Test in Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease According to Clinical and Echocardiographic Parameters

    OpenAIRE

    Akbar Shahmohammadi; Paridokht Nakhostin Davari; Mohammad Yusof Aarabi Mogaddam; Akbar Molaei; Mahmood Meraji

    2009-01-01

    Background: Pulmonary arterial hypertension is a complication of most congenital heart diseases. We sought to assess the effect of sildenafil on patients suffering from pulmonary arterial hypertension in association with congenital heart disease on the basis of clinical and echocardiographic parameters and compare the catheterization and treatment results so as to evaluate the predictive value of sildenafil on the operability of patients. Methods: After primary echocardiography, 21 patients w...

  6. Radiological case: congenital scoliosis

    OpenAIRE

    Macedo, F.

    2010-01-01

    ABSTRACT We present a case of a two month old infant with clinical suspicion of congenital torticollis because of lateral flexion of the head and neck since birth. There was no response to physiotherapy and the neck ultrasound was normal. An x-ray of the cervical and dorsal spine showed congenital scoliosis with failure of formation in certain vertebras. The diagnosis of congenital scoliosis must be considered in cases of abnormal tilting of the head and neck.

  7. DISTRIBUTION OF CONGENITAL HEART DISEASES AT TERTIARY CARE CENTER: SINGLE CENTER EXPERIENCE

    Directory of Open Access Journals (Sweden)

    Vaishali

    2016-02-01

    Full Text Available BACKGROUND The current study was undertaken at a tertiary care center, Bhopal, MP, India, with the objective of establishing frequency of occurrence of congenital heart diseases by echocardiography. MATERIALS AND METHOD 10,000 consecutive cases undergoing Echo Color Doppler in the Cardiology Department Hamidia Hospital, Bhopal, between 1st Jan 2009 and July 2011 were analysed. Echo CD was performed by consultant cardiologist using Acuson Aspen Color Doppler machine following the ASE guidelines. RESULTS AND CONCLUSIONS In the present study out of 10,000consecutive cases undergoing echo 648 were identified to having congenital heart diseases. Isolated VSD (30.5%, isolated ASD (23.6% PDA (9% and TOF (8.3% were commonest defect observed. Most common congenital heart disease found in the present study is VSD and is most prevalent in males and is highest among 0-5 yrs.

  8. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease

    NARCIS (Netherlands)

    Zijlstra, Willemijn M H; Douwes, Johannes M; Ploegstra, Mark-Jan; Krishnan, Usha; Roofthooft, Marcel; Hillege, Hans L; Ivy, D Dunbar; Rosenzweig, Erika B; Berger, Rolf M F

    2016-01-01

    Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore

  9. [From congenital glaucoma to chronic open angle glaucoma in adulthood: a clinical and genetic continuum].

    Science.gov (United States)

    Dufier, Jean-Louis; Rozet, Jean-Michel; Kaplan, Josseline; Roche, Olivier

    2013-01-01

    Congenital glaucoma, a true hydrocephalus of the eye, is defined by ocular hypertension resulting in buphthalmos in children up to three years old, the elasticity of the eye wall allowing its expansion. Juvenile glaucoma in teenagers and chronic glaucoma in adults do not alter the external aspect of the eye, as the eyeball has lost its elasticity. However, chronic ocular hypertension always causes ischemic excavation of the optic nerve head, leading to insidious amputation of the visual field and, potentially, blindness. Like most ophthalmological disorders, the different types of glaucoma have been shown to be genetically determined, and alterations in several genes have been identified. These altered genes can be expressed more or less early in life, suggesting a role of modifier genes. The role of CYP1B1 alterations in classic primary congenital glaucoma is well known, as is the role of PITX2, FOXC1, PAX6 and LOXC1 alterations in secondary congenital glaucoma due to iridogoniodysgenesis, and of MYOC alterations in the genesis of chronic glaucoma in adulthood. An outbred family carrying CYP1B1 mutations in the compound heterozygous state includes two sibs with primary congenital glaucoma and two others who developed chronic glaucoma in adulthood. PMID:24672985

  10. Clinical and histological responses of congenital melanocytic nevi after single treatment with Q-switched lasers

    NARCIS (Netherlands)

    Grevelink, JM; vanLeeuwen, RL; Anderson, RR; Byers, HR

    1997-01-01

    Background: Laser irradiation of congenital melanocytic nevi is a controversial treatment. Recurrence of lesions after laser treatment appears to be the rule, and the effects of laser irradiation on cellular biological behavior and the possible mutagenic responses of nevomelanocytes that have receiv

  11. New clinical molecular diagnostic methods for congenital and inherited heart disease

    NARCIS (Netherlands)

    Jongbloed, Jan Dh; Pósafalvi, Anna; Kerstjens-Frederikse, Wilhelmina S; Sinke, Richard J; van Tintelen, J Peter

    2011-01-01

    IMPORTANCE OF THE FIELD: For patients with congenital and inherited heart disorders, causative mutations are often not identified owing to limitations of current screening techniques. Identifying the mutation is of major importance for genetic counseling of patients and families, facilitating the di

  12. New clinical experience with tramadol.

    Science.gov (United States)

    Sunshine, A

    1994-01-01

    The analgesic efficacy of tramadol has been recently reassessed as part of a new clinical development programme to support an application for registration in the USA. This article reviews the results of single dose and short term studies of oral tramadol 50, 75, 100 and 150 mg in various acute pain conditions. In a double-blind single dose study conducted in 161 patients with severe pain following caesarean section, tramadol 75 and 150 mg and the combination of paracetamol 650 mg with dextropropoxyphene napsylate 100 mg were shown to be effective and statistically superior to placebo. The results from this and 17 other similar studies in patients with pain after surgery (n = 1594) or dental extraction (n = 1859) including other comparators were included in a pooled analysis. Tramadol 100 mg was the optimal single dose for acute pain and tramadol 50 mg showed similar analgesic efficacy to codeine 60 mg. Multiple dose short term studies (n = 520) with tramadol 50, 75 and 100 mg demonstrated a statistically significant and dose-dependent reduction in the consumption of either ibuprofen or morphine as escape medication. New pharmacokinetic data show that steady-state plasma tramadol concentrations reached after oral administration of 50 mg doses every 6 hours are similar to those obtained after administration of a 100 mg single oral dose (250 micrograms/L). This rationale is supported by the results of long term studies in which the average daily dose of tramadol was approximately 250 mg. PMID:7517826

  13. Natural orifice surgery: initial clinical experience

    OpenAIRE

    Horgan, Santiago; Cullen, John P; Talamini, Mark A.; Mintz, Yoav; Ferreres, Alberto; Jacobsen, Garth R.; Sandler, Bryan; Bosia, Julie; Savides, Thomas; Easter, David W.; Savu, Michelle K.; Ramamoorthy, Sonia L.; Whitcomb, Emily; Agarwal, Sanjay; Lukacz, Emily

    2009-01-01

    Background Natural orifice translumenal endoscopic surgery (NOTES) has moved quickly from preclinical investigation to clinical implementation. However, several major technical problems limit clinical NOTES including safe access, retraction and dissection of the gallbladder, and clipping of key structures. This study aimed to identify challenges and develop solutions for NOTES during the initial clinical experience. Methods Under an Institutional Review Board (IRB)-approved protocol, patients...

  14. Clinical and neuroimaging profile of congenital brain malformations in children with spastic cerebral palsy

    International Nuclear Information System (INIS)

    Purpose: Analysis of the incidence of congenital brain malformations in children with spastic cerebral palsy (CP) in a hospital based study. Material and Methods: The present study included 74 boys and 56 girls with spastic tetraplegia, diplegia, and hemiplegia CP. Magnetic resonance imaging MRI findings were analyzed in children with CP. Results: Significant abnormalities relevant to the CP were evident on MRI in 124 (95.3%) subjects. Periventicular leukomalacia (PVL) was detected more frequently in children with spastic diplegia than in patients with tetraplegia or hemiplegia. Cerebral atrophy was found more often in the tetraplegic group compared to the diplegic patients. Porencephalic cysts were detected more often in children with spastic hemiplegia. Congenital brain anomalies were evident in 15 (10.7%) children with spastic CP. Brain malformations included: schizencephaly (5), agenesis corpus callosum (4), polymicrogyria (2), holoprosencephaly (2) and lissencephaly (2). Intractable epilepsy and mental retardation were observed more often in children with brain anomalies. Twelve patients with congenital brain malformations were born at term and three born at preterm. Conclusions: Neuroimaging results in children with CP may help determine the etiology and make better prognosis of CP. (authors)

  15. A clinical comparative study of anatomic parameters before and after total hip replacement on congenital dysplasia.

    Science.gov (United States)

    Huang, Ziqiang; Zhou, Yonggang; Chai, Wei; Ji, Weiping; Cui, Guopeng; Ma, Miaoqun; Zhu, Yin

    2016-07-01

    [Purpose] To study preoperative and postoperative hip circumference data of various types of congenital dysplasia of the hip treated with total hip replacement, including the femoral offset, femoral neck length, height, and hip abductor arm parameters. [Subjects and Methods] This study included seventy-eight cases of congenital dysplasia of the hip (I-III type). Furthermore, four parameters were measured, including the preoperative and postoperative femoral offset. Statistical data analysis was performed using the SPSS 13.0 software. [Results] The femoral offset was 33.3 ± 8.4 mm (preoperative) and 39.1 ± 7.1 mm (postoperative). The femoral head height was 59.5 ± 8.7 mm (preoperative) and 68.8 ± 11.0 mm (postoperative). The femoral neck length was 50.8 ± 10.8 mm (preoperative) and 61.5 ± 10.4 mm (postoperative). The hip abductor arm was 54.3 ± 9.6 mm (preoperative) 64.7 ± 10.1 mm (postoperative). The preoperative and postoperative parameters showed statistical differences. Furthermore, no significant differences were evidenced when comparing the postoperative hip parameters with the normal data parameters. [Conclusion] Total hip replacement on congenital dysplasia of the hip could lead to the rebuilt of an almost normal physiological anatomy for each hip case (type I-III). PMID:27512242

  16. Early therapeutic experience with the endothelin antagonist BQ-123 in pulmonary hypertension after congenital heart surgery

    OpenAIRE

    Prendergast, B; Newby, D; Wilson, L.; Webb, D; Mankad, P

    1999-01-01

    OBJECTIVE—To assess the effect of endothelin type A (ETA) receptor antagonism in infants with pulmonary hypertension following corrective surgery for congenital heart disease.
DESIGN—Open label, preliminary study.
SETTING—Tertiary paediatric cardiothoracic surgical centre.
PATIENTS—Three infants (aged 3 weeks, 7 weeks, and 8 months) with postoperative pulmonary hypertension unresponsive to conventional treatment, including inhaled nitric oxide.
INTERVENTIONS—Patients received incremental intr...

  17. Canadian experience with structured clinical examinations.

    OpenAIRE

    Grand'Maison, P.; Lescop, J; Brailovsky, C. A.

    1993-01-01

    The use of structured clinical examinations to improve the evaluation of medical students and graduates has become significantly more common in the past 25 years. Many Canadian medical educators have contributed to the development of this technique. The Canadian experience is reviewed from the introduction of simulated-standardized patients and objective-structured clinical examinations to more recent developments and the use of such examinations for licensure and certification.

  18. Congenital Pseudoarthrosis of Medial Malleolus in A Young Soccer Player - Diagnosis in Clinical setting of Ankle Sprain

    OpenAIRE

    Giuliano Cerulli; Fantasia Fabiano; Potalivo Gabriele; Placella Giacomo; Sebastiani Enrico

    2014-01-01

    Introduction: We report a case of a young female soccer player affected by congenital medial bilateral malleolus pseudoarthrosis and os subfibulare. Congenital pseudoarthrosis is the failure of the bones to fuse prior or at birth. The etiology is still unknown, although frequency is high in subjects affected by neurofibromatosis or correlated syndromes, so it has been suggested that these congenital disorders may be the cause of congenital pseudoarthrosis. Case Report: Our patient, a 16-y...

  19. Clinical trial comparing autogenous fascia lata sling and Gore-Tex suspension in bilateral congenital ptosis

    Directory of Open Access Journals (Sweden)

    Elsamkary MA

    2016-03-01

    Full Text Available Mahmoud Ahmed Elsamkary,1 Maged Maher Salib Roshdy1,2 1Ophthalmology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt; 2Pediatric Ophthalmology Department, Al Watany Eye Hospital, Cairo, Egypt Purpose: To study the effect of autogenous fascia lata sling (AFLS versus Gore-Tex suspension (GTS regarding the functional and aesthetic outcomes in patients with bilateral congenital ptosis. Patients and methods: A prospective comparative randomized single-center study enrolled 110 patients with bilateral congenital ptosis. One group (n=55 underwent AFLS and the second group (n=55 underwent GTS. Exclusion criteria were good levator function, absent Bell’s phenomenon, and abnormal ocular motility. Follow-up period was 2 years. Functional outcome was measured from digital photos by analysis of upper eyelid margin position relative to the superior limbus and classified as very good (<3 mm, good (3–5 mm, poor (>5 mm, and recurrent. Aesthetic outcome was assessed in terms of lid contour, symmetry of eyelid height, and lid crease presence. Complications were also reported. Results: Failure rate (recurrence and complications was less in AFLS (P=0.035. Symmetrical lid height and good contour were more frequently attained by AFLS (P=0.007 and 0.047, respectively. However, the frequency of very good, good, poor, recurrence, lagopthalmos, ectropion, infection, and formed lid crease individually showed no statistically significant difference (P=0.252, 0.482, 1, 0.489, 0.438, 1, 0.618, and 0.506, respectively. Conclusion: AFLS is a better choice in surgery for patients with bilateral congenital ptosis because it has fewer complications and a lesser recurrence rate than GTS. Keywords: ptosis surgery, surgical outcomes, ptosis recurrence, lagophthalmos

  20. Early experiences of accredited clinical informatics fellowships.

    Science.gov (United States)

    Longhurst, Christopher A; Pageler, Natalie M; Palma, Jonathan P; Finnell, John T; Levy, Bruce P; Yackel, Thomas R; Mohan, Vishnu; Hersh, William R

    2016-07-01

    Since the launch of the clinical informatics subspecialty for physicians in 2013, over 1100 physicians have used the practice and education pathways to become board-certified in clinical informatics. Starting in 2018, only physicians who have completed a 2-year clinical informatics fellowship program accredited by the Accreditation Council on Graduate Medical Education will be eligible to take the board exam. The purpose of this viewpoint piece is to describe the collective experience of the first four programs accredited by the Accreditation Council on Graduate Medical Education and to share lessons learned in developing new fellowship programs in this novel medical subspecialty. PMID:27206458

  1. The clinical experiences of dyslexic healthcare students

    Energy Technology Data Exchange (ETDEWEB)

    Murphy, Fred [Directorate of Radiography, School of Health Care Professions, University of Salford, Allerton Building, Salford, Greater Manchester M6 6PU (United Kingdom)], E-mail: f.j.murphy@salford.ac.uk

    2009-11-15

    This paper reflects on the experiences of healthcare students with dyslexia in order to raise awareness of the potential challenges for dyslexic student radiographers and their clinical educators. With widening participation policies it is likely that the number of student radiographers with specific learning difficulties such as dyslexia will continue to increase. A review of the literature associated with dyslexia in healthcare education was performed in order to provide an overview of the current position. Although Higher Education Institutions (HEIs) have embraced the support and learning opportunities for dyslexic students at university, evidence would suggest that this is not reflected in the clinical departments. The current literature strongly suggests that since the risk of errors with clinical information is far more significant within the clinical placement, there is an immediate requirement for greater understanding, robust support and risk assessment systems. This review considers the problems experienced by dyslexic students, coping strategies they employ and the possible implications for clinical radiography education.

  2. The clinical experiences of dyslexic healthcare students

    International Nuclear Information System (INIS)

    This paper reflects on the experiences of healthcare students with dyslexia in order to raise awareness of the potential challenges for dyslexic student radiographers and their clinical educators. With widening participation policies it is likely that the number of student radiographers with specific learning difficulties such as dyslexia will continue to increase. A review of the literature associated with dyslexia in healthcare education was performed in order to provide an overview of the current position. Although Higher Education Institutions (HEIs) have embraced the support and learning opportunities for dyslexic students at university, evidence would suggest that this is not reflected in the clinical departments. The current literature strongly suggests that since the risk of errors with clinical information is far more significant within the clinical placement, there is an immediate requirement for greater understanding, robust support and risk assessment systems. This review considers the problems experienced by dyslexic students, coping strategies they employ and the possible implications for clinical radiography education.

  3. Congenital Methemoglobinemia Type II-Clinical Improvement with Short-Term Methylene Blue Treatment.

    Science.gov (United States)

    Cooper, Monica S; Randall, Melinda; Rowell, Margaret; Charlton, Margaret; Greenway, Anthea; Barnes, Chris

    2016-03-01

    We report a case of prophylactic management with methylene blue (MB) in an almost 4-year-old male with congenital methemoglobinemia type II. He has a CYB5R3 compound heterozygote mutation, causing a cytochrome-b(5) reductase deficiency. Since the MB treatment regimen has commenced, his methemoglobin level has been significantly lower. He has shown modest behavioral improvements (as assessed on the Achenbach behavior report scales). There have been no iatrogenic side effects. These findings are encouraging for symptomatic improvement with regular prophylactic MB treatment but represent a single case report, which must be interpreted with caution.

  4. Congenital Ocular Motor Apraxia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-06-01

    Full Text Available The clinical and neuroradiological findings, and long-term intellectual prognosis in 10 patients (4 boys and 6 girls with congenital ocular motor apraxia (COMA are reviewed by researchers at Tottori University, Yonago, Japan.

  5. Congenital hyperinsulinism in Ukraine

    DEFF Research Database (Denmark)

    Globa, E.; Zelinska, N.; Flanagan, S.;

    2015-01-01

    Background: Congenital hyperinsulinism (CHI) has not been studied in the Ukraine. Objective and hypotheses: We investigated the genetic aetiology and treatment of patients with CHI. Method: Routine clinical and laboratory investigations were performed in children with hypoglycaemia. Genetic testi...

  6. Congenital Ocular Motor Apraxia

    OpenAIRE

    J Gordon Millichap

    2007-01-01

    The clinical and neuroradiological findings, and long-term intellectual prognosis in 10 patients (4 boys and 6 girls) with congenital ocular motor apraxia (COMA) are reviewed by researchers at Tottori University, Yonago, Japan.

  7. Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea

    Directory of Open Access Journals (Sweden)

    Bo Kyung Jin

    2013-03-01

    Full Text Available Purpose: The use of implantable cardioverter defibrillators (ICDs to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. Methods: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Results: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5±5.4 years (range, 2 to 22 years. The follow-up duration was 28.9±20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1, cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years. Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia.During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. Conclusion: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

  8. Cord blood thyrotropin screening for congenital hypothyroidism. Three years' experience on the Island of Saint Lucia.

    Science.gov (United States)

    Sajous, C; Goto, M; Fitzgerald, M; Anderson, C L; Craft, W; Hurley, R M; Zeller, W P

    1991-01-01

    Cord blood thyrotropin (TSH) screening for congenital primary hypothyroidism has been in effect on the island of St. Lucia for the past three years. Umbilical cord blood samples are obtained on Guthrie filter paper and then transported 3,000 miles to Loyola University of Chicago and delivered to the Illinois State Metabolic Screening Laboratory. There TSH is measured by radioimmunoassay (RIA). After three years, 1,789 newborns have been screened, and the mean value is 6.23 +/- 0.13 microIU per ml. This mean value is less than previously reported by us in 1986 (10.23 +/- 0.29 microIU per ml).13 It is concluded that this screening service continues to be possible far removed from the population under observation. No case of primary hypothyroidism has been detected. Our decreased mean TSH value is due to the new method currently used by the Illinois State Metabolic Screening Laboratory. Congenital hypothyroidism will not be missed provided internal controls are established and rigidly observed. PMID:1781667

  9. Congenital cystic lesions of the lungs: The perils of misdiagnosis - A single-center experience

    Directory of Open Access Journals (Sweden)

    V Shankar Raman

    2015-01-01

    Full Text Available Background: A majority of cystic lesions in the western world are detected antenatally, whereas, the diagnosis in our setup occurs once the child becomes symptomatic. Surgical management is primarily dictated by the presence of symptoms, recurrent infection, and rarely by the potential risk of malignant transformation. Materials and Methods: A retrospective analysis was carried out on all consecutive patients with cystic lung lesions managed at our center from January 2000 through June 2011 for antenatal diagnosis, presentation, diagnostic modalities, treatment, and complications. Results: Forty cystic lung lesions were identified. Only 8% were antenatally detected. Out of 40, the final diagnosis was congenital cystic adenomatoid malformation in 19, congenital lobar emphysema in 11, and bronchogenic cysts and pulmonary sequestration in five each. Of these, 20% had received a course of prior antitubercular therapy and 30% had an intercostal drain inserted prior to referral to our center. Postoperative morbidity in the form of bronchopleural fistula, pneumothorax, and non-expansion of the residual lung was noted in 10% of the patients. Conclusion: Antenatal diagnosis of these lesions is still uncommon in third world countries. Prior to referral to a pediatric surgical center a large number of patients received antitubercular drugs and an intercostal drain insertion, due to incorrect diagnosis.

  10. Vibrotactile masking experiments reveal accelerated somatosensory processing in congenitally blind braille readers.

    Science.gov (United States)

    Bhattacharjee, Arindam; Ye, Amanda J; Lisak, Joy A; Vargas, Maria G; Goldreich, Daniel

    2010-10-27

    Braille reading is a demanding task that requires the identification of rapidly varying tactile patterns. During proficient reading, neighboring characters impact the fingertip at ∼100 ms intervals, and adjacent raised dots within a character at 50 ms intervals. Because the brain requires time to interpret afferent sensorineural activity, among other reasons, tactile stimuli separated by such short temporal intervals pose a challenge to perception. How, then, do proficient Braille readers successfully interpret inputs arising from their fingertips at such rapid rates? We hypothesized that somatosensory perceptual consolidation occurs more rapidly in proficient Braille readers. If so, Braille readers should outperform sighted participants on masking tasks, which demand rapid perceptual processing, but would not necessarily outperform the sighted on tests of simple vibrotactile sensitivity. To investigate, we conducted two-interval forced-choice vibrotactile detection, amplitude discrimination, and masking tasks on the index fingertips of 89 sighted and 57 profoundly blind humans. Sighted and blind participants had similar unmasked detection (25 ms target tap) and amplitude discrimination (compared with 100 μm reference tap) thresholds, but congenitally blind Braille readers, the fastest readers among the blind participants, exhibited significantly less masking than the sighted (masker, 50 Hz, 50 μm; target-masker delays, ±50 and ±100 ms). Indeed, Braille reading speed correlated significantly and specifically with masking task performance, and in particular with the backward masking decay time constant. We conclude that vibrotactile sensitivity is unchanged but that perceptual processing is accelerated in congenitally blind Braille readers.

  11. Challenges in the management of congenital heart disease in Vietnam: A single center experience

    Directory of Open Access Journals (Sweden)

    Vu Minh Phuc

    2015-01-01

    Full Text Available Vietnam, in Asia, is a low middle-income country with a relatively large population to cater to. Not many know about Vietnam, or its healthcare sector especially the field of pediatric cardiology and congenital heart disease. In contrast to the developed world, congenital heart disease (CHD is not diagnosed early. Since most of the patients visit the hospital only in later stages of the disease there are many complications during the operation and post-operatively. But during the past 5 years (from 2009, there has been major improvement in the treatment of CHD, both by intervention and surgery. At present, all kinds of CHD, both simple and complex are being successfully treated in our country. Today in Vietnam, all children under 6 years of age have health insurance coverage, under which almost all operations and catheter interventions are done free in government hospitals. It is helping many patients, especially those from the poor socioeconomic background. However, the present infrastructure is inadequate and a long waiting list has accumulated for treatment of CHD.

  12. Challenges in the management of congenital heart disease in Vietnam: A single center experience

    International Nuclear Information System (INIS)

    Vietnam, in Asia, is a low middle-income country with a relatively large population to cater to. Not many know about Vietnam, or its healthcare sector especially the field of pediatric cardiology and congenital heart disease. In contrast to the developed world, congenital heart disease (CHD) is not diagnosed early. Since most of the patients visit the hospital only in later stages of the disease there are many complications during the operation and post-operatively. But during the past 5 years (from 2009), there has been major improvement in the treatment of CHD, both by intervention and surgery. At present, all kinds of CHD, both simple and complex are being successfully treated in our country. Today in Vietnam, all children under 6 years of age have health insurance coverage, under which almost all operations and catheter interventions are done free in government hospitals. It is helping many patients, especially those from the poor socioeconomic background. However, the present infrastructure is inadequate and a long waiting list has accumulated for treatment of CHD

  13. 足后内侧松解胫前肌外移治疗小儿先天性马蹄内翻足的体会%Medial Foot after Neurolysis and Anterior Tibial Shift in Clinical Treatment of Children with Congenital Clubfoot Experience

    Institute of Scientific and Technical Information of China (English)

    杨新民

    2014-01-01

    Objective To investigate the tibialis anterior muscle transfer ef ect in the treatment of children with congenital clubfoot. Methods 61 cases with 106 foot for foot posteromedial release and anterior tibial shift on congenital talipes equinovarus in children. Results 4 years of fol ow-up in 18 cases 34 feet 52 feet, 6 years of fol ow-up, the appearance was satisfactory, ankle and foot activities basical y normal, flat foot can walk, check the children to wear shoes with no significant dif erence in normal children sole. The total excellent and good rate was 93%.Conclusion Anterior tibial muscle tendon transfer in treatment of children with congenital talipes equinovarus, think age is smal , strong ability of tarsal bone remodeling, the relationship between easy to return to normal, quickly into the normal morphological development.%目的:探讨胫前肌外移治疗小儿先天性马蹄内翻足的疗效。方法对小儿先天性马蹄内翻足61例106只足行足后内侧松解胫前肌外移。结果随访4年18例34只足,随访6年52只足,外形满意,踝关节和足的各项活动基本正常,足能放平走路,检查患儿鞋底磨损与正常儿童鞋底无明显差别,总优良率93%。结论胫前肌外移治疗小儿先天性马蹄内翻足,认为年龄小,骨塑形能力强,跗骨间关系容易恢复正常,较快进入正常形态发育。

  14. [Congenital toxoplasmosis: clinical manifestation, treatment and follow-up] [Article in Italian] • Il neonato con toxoplasmosi congenita: clinica, terapia e follow-up

    Directory of Open Access Journals (Sweden)

    Lina Bollani

    2014-01-01

    Full Text Available Toxoplamosis is a parasitic zoonosis which occurs worldwide, but is prevalent in Europe, South America and Africa. When infection occurs for the first time during pregnancy, mother to child transmission of the parasite can cause congenital toxoplasmosis. Rate of congenital infection ranges from less than 0.1 to approximately 1 per 1,000 live births. The risk of transmission depends on the gestational age at the time of maternal infection. A diagnosis of congenital toxoplasmosis is usually considered in infants who present: hydrocephalus, chorioretinitis, and intracranial calcifications, but this triade is very rare. Approximately 85% of the infants with congenital toxoplasmosis are clinically normal at birth; however, sequelae of infection may become apparent only months or even years later. Chorioretinitis is the main complication of congenital toxoplasmosis, late onset retinal lesions and relapse can appear many years after birth, but the overall ocular prognosis is satisfactory when infection is identified and treated accordingly. Fortunately, serious neonatal forms and severe neurological impairment have become rare, but prompt treatment of children with convulsions, abnormal muscle tone, hydrocephalus, may improve the prognosis and result in almost normal outcome. For infants who have congenital toxoplasmosis, treatment soon after birth for 1 year with pyrimetamine, sulfadiazine and leukoverin led to remarkable resolution of serious, active disease. A long follow-up is necessary to assess the long-term outcome of children and young adults with congenital toxoplasmosis, that is favourable for the majority of cases. Epidemiological surveillance needs to be improved in order to determine the effectiveness of prevention programs.Articoli Selezionati del “3° Convegno Pediatrico del Medio Campidano” · Guspini · 25 Maggio 2013Guest Editor: Roberto Antonucci

  15. Congenital Pseudoarthrosis of Medial Malleolus in A Young Soccer Player – Diagnosis in Clinical setting of Ankle Sprain

    Directory of Open Access Journals (Sweden)

    Giuliano Cerulli

    2014-01-01

    Full Text Available Introduction: We report a case of a young female soccer player affected by congenital medial bilateral malleolus pseudoarthrosis and os subfibulare. Congenital pseudoarthrosis is the failure of the bones to fuse prior or at birth. The etiology is still unknown, although frequency is high in subjects affected by neurofibromatosis or correlated syndromes, so it has been suggested that these congenital disorders may be the cause of congenital pseudoarthrosis. Case Report: Our patient, a 16-year-old female, high level soccer player, was referred to us following a right ankle sprain during a match. She reported no medical history of tibia-tarsus joint injuries or disease. Pain, swelling and functional impairment were noted immediately after the accident. Standard radiographs in the emergency department revealed a displaced fracture of the medial malleolus and the presence of os subfibularis. The patient was transferred to our Traumatology and Orthopaedic Department to undergo malleolus ostheosynthesis. Before surgery swelling, functional impairment and intense pain at the medial malleolus level were confirmed. However, there was no radiological opening of ankle, instability or pronation pain; furthermore the flexion-extension was preserved with slight pain. Twenty-four hours later a considerable remission of symptoms was evident with increased range of motion and reduction in the swelling and post-traumatic edema. A radiograph on the left ankle to compare with that of the right ankle was necessary to overcome the discrepancy between the radiological diagnosis and the clinical examination. The radiographic results of both medial malleoli were comparable although on the left the os subfibularis was absent. Since the diagnosis of fracture by the association between the radiographs and the symptomatology was doubtful, a bilateral CT was performed. The scan revealed a medial bilateral malleolus pseudoarthrosis and an accessory right subfibularis nucleus

  16. Identification of congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    For many reasons an accurate and straightforward identification of congenital deafblindness can be difficult. This article reports on the assessment procedures and experience in Denmark where medical examinations were combined with functional assessments performed through direct observation. The ...

  17. Clinical results of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries

    Institute of Scientific and Technical Information of China (English)

    FAN Xiang-ming; ZHU Yao-bin; SU Jun-wu; ZHANG Jing; LI Zhi-qiang; XU Yao-qiang; LI Xiao-feng

    2013-01-01

    Background Congenital heart defects with intractable hypoplasia of the pulmonary arteries without intercourse or with intercourse stenosis is unsuitable for surgical correction or regular palliative procedures.We reported our experience with combined palliative procedures for congenital heart defects with intractable hypoplasia pulmonary arteries.Methods From 2001 to 2012,a total of 41 patients with cyanotic congenital heart defects and intractable hypoplasia of the pulmonary arteries underwent surgical procedures.From among them,31 patients had pulmonary atresia with ventricular septal defect (VSD) and the other 10 cases had complicated congenital heart defects with pulmonary stenosis.Different kinds of palliative procedures were performed according to the morphology of the right and left pulmonary arteries in every patient.If the pulmonary artery was well developed,a Glenn procedure was performed.A modified Blalock-Taussi9 shunt or modified Waterston shunt was performed if pulmonary arteries were hypoplastic.If the pulmonary arteries were severely hypoplastic,a Melbourne shunt was performed.Systemic pulmonary artery shunts were performed bilaterally in 25 cases.A systemic-pulmonary shunt was performed on one side and a Glenn procedure was performed contralaterally in 16 cases.Major aortopulmonary collateral arteries were unifocalized in six cases,ligated in two cases and interventionally embolized in two cases.There was one early death because of cardiac arrest and the hospital mortality was 2.4%.Results Five patients suffered from postoperative low cardiac output syndrome,three had perfusion of the lungs,and two pulmonary infections.Systemic pulmonary shunts were repeated after the original operation in three cases due to the occlusion of conduits.The mean follow-up time was 25 months.The pre-and the post-operation left pulmonary indices were (8.13±3.68) vs.(14.9±6.21) mm2/m2.The pre-and post-operation right pulmonary indices were (12.7±8.13) vs.(17.7±7

  18. First year clinical tutorials: students’ learning experience

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    Burgess A

    2014-11-01

    Full Text Available Annette Burgess,1 Kim Oates,2 Kerry Goulston,2 Craig Mellis1 1Central Clinical School, Sydney Medical School, The University of Sydney, Sydney, NSW, Australia; 2Sydney Medical School, The University of Sydney, Sydney, NSW, Australia Background: Bedside teaching lies at the heart of medical education. The learning environment afforded to students during clinical tutorials contributes substantially to their knowledge, thinking, and learning. Situated cognition theory posits that the depth and breadth of the students' learning experience is dependent upon the attitude of the clinical teacher, the structure of the tutorial, and the understanding of tutorial and learning objectives. This theory provides a useful framework to conceptualize how students' experience within their clinical tutorials impacts their knowledge, thinking, and learning. Methods: The study was conducted with one cohort (n=301 of students who had completed year 1 of the medical program at Sydney Medical School in 2013. All students were asked to complete a three-part questionnaire regarding their perceptions of their clinical tutor's attributes, the consistency of the tutor, and the best features of the tutorials and need for improvement. Both quantitative and qualitative data were collected and analyzed using descriptive statistics. Results: The response rate to the questionnaire was 88% (265/301. Students perceived that their tutors displayed good communication skills and enthusiasm, encouraged their learning, and were empathetic toward patients. Fifty-two percent of students reported having the same communications tutor for the entire year, and 28% reported having the same physical examination tutor for the entire year. Students would like increased patient contact, greater structure within their tutorials, and greater alignment of teaching with the curriculum. Conclusion: Situated cognition theory provides a valuable lens to view students' experience of learning within the

  19. Comparison of Two Methods for Upper Lid Fascia Lata Sling in Congenital Blepharoptosis: a Randomized Clinical Trial

    Directory of Open Access Journals (Sweden)

    Abbas Bagheri

    2008-12-01

    Full Text Available

    PURPOSE: To compare the results of two different methods of upper lid sling with autogenous fascia lata in the treatment of congenital ptosis. METHODS: In a randomized clinical trial, patients with congenital upper lid ptosis and poor levator function (< 4mm were randomly assigned to two different methods of upper lid sling: group A, bitriangular fascia sling (modified Crawford method and group B, monotriangular fascia sling (modified Fox method. RESULTS:  This study included 30 upper eyelids (15 eyelids in each surgical group of 19 patients (8 unilateral and 11 bilateral cases with congenital ptosis. Mean increase in eyelid fissure height was 2.7±2.3 mm in group A and 3.4±2.2 mm in group B, respectively. Change in eyelid fissure in both groups was significant (P < 0.001, paired t-test but intergroup difference was not (P=0.4, independent sample t-test. Early complications such as corneal epithelial defects and entropion, and late complications such as undercorrection were comparable in the two groups. No patient experienced recurrent ptosis requiring reoperation in either group. CONCLUSION:  The monotriangular method of upper lid fascia sling can be used instead of the more popular bitriangular method. Advantages include less need for fascial tissue, less periocular scar formation and a shorter period of anesthesia.

  20. Congenital myasthenia gravis.

    Science.gov (United States)

    Nizamani, Noor Bakht; Talpur, Khalid Iqbal; Memon, Mariya Nazish

    2013-07-01

    Congenital myasthenia gravis is caused by genetic mutations affecting neuromuscular transmission, characterized by muscle weakness usually starting in childhood. A two and a half years old male child presented with bilateral ptosis and hoarseness of voice. The symptoms progressed giving the clinical impression of congenital myasthenia gravis. A series of tests were done including Ice Pack Test, acetylcholine receptor antibody test, trial of steroids and finally neostigmine test which confirmed the diagnosis. This case illustrates the challenges in diagnosing congenital myasthenia gravis and highlights the potential benefits of neostigmine test in its diagnosis. PMID:23823963

  1. Disorder of Sexual Development and Congenital Heart Defect in 47XYY: Clinical Disorder or Coincidence?

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    Hanane Latrech

    2015-01-01

    Full Text Available Background. 47XYY syndrome is a rare sex chromosome variation characterized by an additional Y chromosome. Most patients with 47XYY karyotype have normal phenotype. This disorder seems associated with a higher risk of developing behavioral and cognitive problems, tall stature, and infertility in adulthood. Sexual development disorder is a rare finding. We report a first case with an abnormal left coronary artery originating from the pulmonary artery in a 47XYY patient. Case. A one-month-old child was referred for ectopic testis and micropenis. Physical examination revealed facial dysmorphia, micropenis, and curvature of the penis with nonpalpable testis. Laboratory tests showed decreased total testosterone and anti-Mullerian hormone (AMH levels. Blood karyotyping revealed a 47XYY chromosomal formula. At the age of 3 months, the patient developed dyspnea and tachycardia. Echocardiography revealed an anomalous left coronary artery from pulmonary artery with left ventricular dysfunction requiring surgical revascularization by direct reimplantation of the left coronary artery system. Our second case was a 3-year-old child referred for hypospadias with nonpalpable left testicle. Physical examination showed hypertelorism. Blood karyotyping revealed a 47XYY chromosomal formula. Conclusion. To our knowledge, this is the first case of 47XYY syndrome associated with this congenital heart malformation and a sexual development disorder.

  2. Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report.

    Science.gov (United States)

    Takahashi, H; Ohkuchi, A; Kuwata, T; Usui, R; Takahashi, S; Matsubara, S

    2016-01-01

    To characterise congenital mesoblastic nephroma (CMN), with special emphasis on polyhydramnios and the neonatal prognosis, we summarise 31 CMN patients (30 reported patients and the present patient). CMN was detected at a median of 30 weeks' gestation, and infants were delivered at a median of 34 weeks' gestation. Of 27 patients with available data, 19 (70%) had polyhydramnios, of which 8 required amnio- drainage. Women with amnio-drainage gave birth significantly earlier (30.4 weeks' gestation) than those without polyhydramnios (36.7 weeks' gestation). Thus, CMN was frequently associated with polyhydramnios and this polyhydramnios was associated with a significant increase in the risk of preterm birth. Of 20 patients with available data, the affected-side kidney was 'compressed' in 16 and 'replaced' in 4: polyhydramnios was present in a half vs 100%, respectively, suggesting that a 'replaced' kidney may suggest a more aggressive tumour and may be associated with a poorer prognosis. Univariate analysis showed that early gestational week at diagnosis was the only feature significantly associated with poor prognosis. Thus, polyhydramnios, 'replaced' kidney and early gestational week at diagnosis, may indicate poor prognosis, to which obstetricians should pay attention. PMID:26467634

  3. Congenital lobar emphysema

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    Tural-Kara, Tuğçe; Özdemir, Halil; Çiftçi, Ergin; İnce, Erdal

    2016-01-01

    Congenital lobar emphysema is a rare disease, which is characterized by pulmoner hyperinflation. Depending on the degree of bronchial obstruction, the clinical presentation may be variable. We report a rare case with congenital lobar emphysema in a 38-days-old male infant who presented with severe respiratory distress and hypertension. Air trapping in the left upper lung and significant mediastinal shift to the right were observed on the chest x-ray. Emphysematous changes were detected on the thorax computed tomography and considered as congenital lobar emphysema. The upper left lobectomy was successfully performed by pediatric surgeons. On postoperative follow up, no sign of respiratory distress occurred and the patient was normotensive. In this report, a case with congenital lobar emphysema, which is a rare cause of respiratory distress and hypertension is discussed. PMID:27381542

  4. Congenital histiocytosis X

    International Nuclear Information System (INIS)

    Congenital histiocytosis X involving multiple organs is a rare disease that causes rapid mortality in intrauterine and neanatal life. The diagnosis of histiocytosis X (Litterer-Siwe disease should be considered in a neonate with vesiculated crusting skin lesions. We present clinical, radiographic and histopathological findings in a neonate with congenital histiocytosis who died of respiratory failure due to diffuse infilteration of lungs with histiocytic cells. Congenital histiocytosis X is a rare form of Langerhans cell histiocytosis. We report on an infant with congenital histiocytosis X who died within 10 days of birth due to diffuse infiltration of multiple organ systems with Langerhans histiocytic cells. To our knowledge, this is the first case of the radiographic illustration of progressive lung involvement in an infant with histiocytosis X. (orig.)

  5. Correlation of biochemical markers and clinical signs of hyperandrogenism in women with polycystic ovary syndrome (PCOS) and women with non-classic congenital adrenal hyperplasia (NCAH)

    OpenAIRE

    Nana Kvashilava; Jenara Kristesashvili; Diana Chanukvadze

    2012-01-01

    Background: Polycystic ovary syndrome (PCOS) is the most common cause of hyperandrogenism in women. Non-classic congenital adrenal hyperplasia (NCAH) is very close to PCOS. The diagnosis of hyperandrogenism is not based on the finding of decreased or increased levels of a single hormone. Objective: In our paper, we are going to test correlation between clinical signs and biochemical markers of hyperandrogenism. Materials and Methods: In this prospective study, we calculated free testosterone ...

  6. [Prevalence of congenital heart diseases in Koranic schools (daara) in Dakar: a cross-sectional study based on clinical and echocardiographic screening in 2019 school children].

    Science.gov (United States)

    Bodian, M; Ngaïdé, A A; Mbaye, A; Sarr, S A; Jobe, M; Ndiaye, M B; Kane, A D; Aw, F; Gaye, N D; Ba, F G; Bah, M B; Tabane, A; Dioum, M; Diagne, D; Diao, M; Diack, B; Sarr, M; Kane, A; Bâ, S A

    2015-02-01

    Congenital heart diseases are one of the major cardiovascular diseases in developing countries. Most prevalence studies were based on clinical examination of children with echocardiographic confirmation of suspected cases and underestimate its prevalence. The objective of this study was to investigate the prevalence of congenital heart disease in "daara" (Koranic schools) in the city of Dakar and its suburbs on the basis of clinical examination and Doppler echocardiography in school children. This cross-sectional survey was carried out from 9(th) August to 24(th) December 2011, and included a population of 2019 school children aged 5 to 18 years in 16 selected "daaras" under the Academic Inspectorate of Dakar and its suburbs. Anamnestic, clinical and echocardiographic data were recorded in a validated questionnaire. A p heart diseases were detected being a prevalence of 8.9 per 1 000 (95 % CI: 1.8 to 7.9). This included 6 cases of inter-atrial septal aneurysm, 5 cases of peri-membranous ventricular septal defects, 4 cases of patent ductusarteriosus and 3 cases of tetralogy of Fallot. Factors correlated with the presence of congenital heart disease were ageless than 8 (p rheumatic valvular disease, a prevalence of 4.9 per 1 000 (95% CI: 2.4 to 9.1). Our study shows a high prevalence of congenital heart diseases, which is almost identical to the WHO estimates and that ultrasound screening is more sensitive than clinical screening. Reducing the prevalence of these diseases requires implementation of appropriate policies, focusing on awareness and early detection. PMID:25516291

  7. Congenital and perinatal complications of chikungunya fever: a Latin American experience

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    Jaime R. Torres

    2016-10-01

    Conclusions: This study presents the largest number of symptomatic neonates with CHIKF analyzed so far in any region and is the first involving infection with the Asian genotype of CHIKV. Although the clinical manifestations found were similar to those reported previously, the percentage of neurological complications was lower. The CFR was comparatively high. Chikungunya represented a substantial risk for neonates born to symptomatic parturients during the chikungunya outbreak in the Americas Region, with important clinical and public health implications.

  8. Self-Reported Health Experiences of Children Living with Congenital Heart Defects: Including Patient-Reported Outcomes in a National Cohort Study

    Science.gov (United States)

    Tadic, Valerija; Hogan, Ailbhe; Bull, Catherine; Rahi, Jugnoo Sangeeta; Dezateux, Carol

    2016-01-01

    Background Understanding children’s views about living with congenital heart defects (CHDs) is fundamental to supporting their successful participation in daily life, school and peer relationships. As an adjunct to a health and quality of life outcomes questionnaire, we asked school-age children who survived infant heart procedures to describe their experiences of living with CHDs. Methods In a UK-wide cohort study, children aged 10 to 14 years with CHDs self-completed postal questionnaires that included an open question about having a ‘heart problem’. We compared the characteristics of children with more and less severe cardiac diagnoses and, through collaborative inductive content analysis, investigated the subjective experiences and coping strategies described by children in both clinical severity groups. Results Text and/or drawings were returned by 436 children (246 boys [56%], mean age 12.1 years [SD 1.0; range 10–14]); 313 had less severe (LS) and 123 more severe (MS) cardiac diagnoses. At the most recent hospital visit, a higher proportion of the MS group were underweight (more than two standard deviations below the mean for age) or cyanosed (underweight: MS 20.0%, LS 9.9%; cyanosed: MS 26.2%, LS 3.5%). Children in the MS group described concerns about social isolation and feeling ‘different’, whereas children with less severe diagnoses often characterised their CHD as ‘not a big thing’. Some coping strategies were common to both severity groups, including managing health information to avoid social exclusion, however only children in the LS group considered their CHD ‘in the past’ or experienced a sense of survivorship. Conclusions Children’s reported experiences were not dependent on their cardiac diagnosis, although there were clear qualitative differences by clinical severity group. Children’s concerns emphasised social participation and our findings imply a need to shift the clinical focus from monitoring cardiac function to

  9. Clinical Experience in TCM Treatment of Insomnia

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    @@ Insomnia is a sleeping disorder that affects 1 in 10 Americans and around 50% of the seniors. It is often more prevalent in women. Since September 11, 2005 these estimations have increased. Insomnia can affect not only your energy level and mood, but your health as well because sleep helps bolster your immune system. Insomnia is characterized by: 1) difficulty in falling asleep; 2) waking up frequently during the night with difficulty of returning to sleep; 3) waking up too early in the morning; and 4) with unrefreshing sleep, the patient has a low spirit, palpation, poor memory, viscera function disorder. All these seriously affect the patient's life and work. The following is an account of the authors' clinical TCM experience in treating insomnia.

  10. Radiological imaging of congenital hand anomalies - a 6-year single-centre experience and what the hand surgeons want to know

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    Gerety, E.L.; Hopper, M.A. [Cambridge University Hospitals NHS Foundation Trust, Department of Radiology, Cambridge (United Kingdom); Grant, I. [Cambridge University Hospitals NHS Foundation Trust, Department of Plastic Surgery, Cambridge (United Kingdom)

    2014-12-19

    Congenital hand anomalies present a rare but important physical and emotional challenge for children and parents. Radiological imaging is important for accurate diagnosis, to aid decision making and to monitor changes in the growing hand. The goal of any treatment is to help the child achieve his/her maximum potential, to provide a useful hand with attention to cosmesis. We investigated the range of congenital hand anomalies imaged in a tertiary referral centre. We examined the timing of imaging and the key clinical questions. The radiology imaging system was searched retrospectively for radiographs of congenital hand anomalies over a 6-year period. The images were reviewed and patient demographics, diagnosis and other imaging recorded. Over 6 years, 85 patients had imaging. Twenty-three patients had bilateral problems and 11 had recognised syndromes. The most common abnormalities imaged were duplicated thumbs (28 %), followed by syndactyly (18 %). Children were first imaged as early as 1 day old, with the median age of initial imaging 12 months. Thumb duplication and syndactyly are the most common conditions for which radiographs are requested at our hospital, although overall syndactyly is considered the most common congenital hand anomaly. For a variety of reasons, children are often imaged very early, before review by the Specialist in Children's Hand Surgery (despite surgery being unlikely before 1 year of age.) We discuss the classification systems and specific issues that hand surgeons want to know from the radiologists. (orig.)

  11. Early detection of congenital syphilis

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    Nagalakshmi Chowdhary

    2014-01-01

    Full Text Available Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson′s teeth.

  12. Congenital Pulmonary Alveolar Proteinosis

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    Saber Hammami

    2013-01-01

    Full Text Available Pulmonary alveolar proteinosis (PAP is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the current standard treatment for PAP patients. We report a new congenital case of PAP.

  13. Clinical experience with the Sarns centrifugal pump.

    Science.gov (United States)

    Curtis, J J; Walls, J T; Demmy, T L; Boley, T M; Schmaltz, R A; Goss, C F; Wagner-Mann, C C

    1993-07-01

    Since October 1986, we have had experience with 96 Sarns centrifugal pumps in 72 patients (pts). Heparinless left atrial to femoral artery or aorta bypass was used in 14 pts undergoing surgery on the thoracic aorta with 13 survivors (93%). No paraplegia or device-related complications were observed. In 57 patients, the Sarns centrifugal pump was used as a univentricular (27 pts) or biventricular (30 pts) cardiac assist device for postcardiotomy cardiogenic shock. In these patients, cardiac assist duration ranged from 2 to 434 h with a hospital survival rate of 29% in those requiring left ventricular assist and 13% in those requiring biventricular assist. Although complications were ubiquitous in this mortally ill patient population, in 5,235 pump-hours, no pump thrombosis was observed. Hospital survivors followed for 4 months to 6 years have enjoyed an improved functional class. We conclude that the Sarns centrifugal pump is an effective cardiac assist device when used to salvage patients otherwise unweanable from cardiopulmonary bypass. Partial left ventricular bypass using a centrifugal pump has become our procedure of choice for unloading the left ventricle and for maintenance of distal aortic perfusion pressure when performing surgery on the thoracic aorta. This clinical experience with the Sarns centrifugal pump appears to be similar to that reported with other centrifugal assist devices.

  14. Congenital bipartite atlas with hypodactyly in a dog: clinical, radiographic and CT findings.

    Science.gov (United States)

    Wrzosek, M; Płonek, M; Zeira, O; Bieżyński, J; Kinda, W; Guziński, M

    2014-07-01

    A three-year-old Border collie was diagnosed with a bipartite atlas and bilateral forelimb hypodactyly. The dog showed signs of acute, non-progressive neck pain, general stiffness and right thoracic limb non-weight-bearing lameness. Computed tomography imaging revealed a bipartite atlas with abaxial vertical bone proliferation, which was the cause of the clinical signs. In addition, bilateral hypodactyly of the second and fifth digits was incidentally found. This report suggests that hypodactyly may be associated with atlas malformations. PMID:24635705

  15. Nurses' experiences of clinical commissioning group boards.

    Science.gov (United States)

    Allan, Helen; O'Driscoll, Mike; Savage, Jan; Lee, Gay; Dixon, Roz

    2016-06-15

    Aim To explore the experience of governing body nurses appointed to clinical commissioning group (CCG) boards; how they perform their responsibilities; and their perceived effectiveness in ensuring safe, patient-centred care and the factors that influence their effectiveness. Method This was a small pilot study using a mixed methods approach. There were four phases of the study: literature review, qualitative data collection (interviews), quantitative data collection (survey), and final data analysis. Findings In the early stages of the formation of CCGs, few governing body nurses had relevant experience to meet the needs of a strategic role, and many of these nurses had no proper job description, too little time to carry out their responsibilities, little management support, and unequal access to training, development, formal support or supervision compared to GP colleagues. Two working patterns or models of work of governing body nurses emerged: the full-time integrated executive statutory role and the part-time non-executive statutory role. Quality and quality assurance were the most frequently cited roles or responsibilities of governing body nurses in CCGs, and their highest priority was to improve the population's health. Conclusion The role of governing body nurse has emerged at a time of organisational change, and following extensive criticism of nursing and nurses in the media. Nurses' roles and experiences are affected by these contextual events and by the emerging structures and diversity of CCGs. Further research is required into the leadership role of governing body nurses, succession planning, and the effectiveness of their relationships with other senior nurses. PMID:27305258

  16. A clinical and experimental overview of sirenomelia: insight into the mechanisms of congenital limb malformations

    Directory of Open Access Journals (Sweden)

    Carlos Garrido-Allepuz

    2011-05-01

    Full Text Available Sirenomelia, also known as sirenomelia sequence, is a severe malformation of the lower body characterized by fusion of the legs and a variable combination of visceral abnormalities. The causes of this malformation remain unknown, although the discovery that it can have a genetic basis in mice represents an important step towards the understanding of its pathogenesis. Sirenomelia occurs in mice lacking Cyp26a1, an enzyme that degrades retinoic acid (RA, and in mice that develop with reduced bone morphogenetic protein (Bmp signaling in the caudal embryonic region. The phenotypes of these mutant mice suggest that sirenomelia in humans is associated with an excess of RA signaling and a deficit in Bmp signaling in the caudal body. Clinical studies of sirenomelia have given rise to two main pathogenic hypotheses. The first hypothesis, based on the aberrant abdominal and umbilical vascular pattern of affected individuals, postulates a primary vascular defect that leaves the caudal part of the embryo hypoperfused. The second hypothesis, based on the overall malformation of the caudal body, postulates a primary defect in the generation of the mesoderm. This review gathers experimental and clinical information on sirenomelia together with the necessary background to understand how deviations from normal development of the caudal part of the embryo might lead to this multisystemic malformation.

  17. Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results

    Directory of Open Access Journals (Sweden)

    Lorier Gabriel

    2001-01-01

    Full Text Available OBJECTIVE: To analyze late clinical evolution after surgical treatment of children, with reparative and reconstructive techniques without annular support. METHODS: We evaluated 21 patients operated upon between 1975 and 1998. Age 4.67±3.44 years; 47.6% girls; mitral insufficiency 57.1% (12 cases, stenosis 28.6% (6 cases, and double lesion 14.3% (3 cases. The perfusion 43.10±9.50min, and ischemia time were 29.40±10.50min. The average clinical follow-up in mitral insufficiency was 41.52±53.61 months. In the stenosis group (4 patients was 46.39±32.02 months, and in the double lesion group (3 patients, 39.41±37.5 months. The echocardiographic follow-up was in mitral insufficiency 37.17±39.51 months, stenosis 42.61±30.59 months, and in the double lesion 39.41±37.51 months. RESULTS: Operative mortality was 9.5% (2 cases. No late deaths occurred. In the group with mitral insufficiency, 10 (83.3% patients were asymptomatic (p=0.04. The majorit y with mild reflux (p=0.002. In the follow-up of the stenosis group, all were in functional class I (NYHA; and the mean transvalve gradient varied between 8 and 12mmHg, average of 10.7mmHg. In the double lesion group, 1 patient was reoperated at 43 months. No endocarditis or thromboembolism were reported. CONCLUSION: Mitral stenosis repair has worse late results, related to the valve abnormalities and associated lesions. The correction of mitral insufficiency without annular support showed good long-term results.

  18. Anoftalmia clínica bilateral associada à hidrocefalia congênita em cão Bilateral clinical anophthalmia associated with congenital hydrocephalus in the dog

    Directory of Open Access Journals (Sweden)

    Mariana Isa Poci Palumbo

    2011-07-01

    Full Text Available A ausência completa do bulbo ocular é muito rara em cães e gatos, enquanto a hidrocefalia é comumente observada como distúrbio congênito em cães de raças miniatura ou braquicefálicas, com menos de um ano de idade. O presente trabalho relata a ocorrência de anoftalmia clínica bilateral associada à hidrocefalia congênita em um cão da raça poodle, sendo este o primeiro relato de caso da associação dessas alterações no Brasil.The complete absence of the eyeball is rare in dogs and cats, and hydrocephalus is commonly seen as a congenital disorder in toy or brachycephalic dogs before one year old. This paper describes for the first time in Brazil the occurrence of bilateral clinical anophthalmia associated to congenital hydrocephalus in a dog.

  19. Clinical features of different clinical forms of childhood congenital hepatic ifbrosis%儿童先天性肝纤维化临床分型及特征

    Institute of Scientific and Technical Information of China (English)

    吴欣; 杜霄壤; 丁金芳; 吴孟晋; 罗生强; 冯兴中

    2016-01-01

    ObjectiveTo compare the clinical features of children with different clinical forms of congenital hepatic ifbrosis (CHF), and provides a description of the characteristics of childhood CHF.MethodsSixty children with CHF between January 2002 and June 2015 were enrolled, including 26 children with portal hypertensive CHF (PH CHF), 3 children with cholangitic CHF, 30 children with combined portal hypertensive and cholangitic CHF (mixed CHF), and 1 child with latent forms of CHF. The medical data of 26 children with PH CHF and 30 children with mixed CHF, including gender, age, clinical manifestations, physical signs, laboratory tests and imaging characteristics, were retrospectively studied.ResultsFever, jaundice and hepatomegaly were more frequently noted in children with mixed CHF than in those with PH CHF (P0.05);混合型患儿凝血酶原活动度、白细胞计数、血小板计数、血小板平均体积、丙氨酸转氨酶、天冬氨酸转氨酶、碱性磷酸酶、ν谷氨酰转肽酶、亮氨酸氨基肽酶和总胆汁酸水平高于门脉高压型患儿,国际标准化比值和白蛋白水平低于门脉高压型患儿(P<0.05)。结论儿童CHF中以门脉高压型和混合型常见,门脉高压型和混合型均以肝硬化、肝脾肿大等门脉高压表现明显,但混合型常伴肝损伤。

  20. Congenital nystagmus and negative electroretinography

    Directory of Open Access Journals (Sweden)

    Roussi M

    2011-04-01

    Full Text Available Mirella Roussi, Hélène Dalens, Jean Jacques Marcellier, Franck BacinDepartment of Ophthalmology, Clermont-Ferrand University, Clermont-Ferrand, FranceAbstract: Congenital nystagmus is a pathologic oculomotor state appearing at about three to four months of age. The precise diagnosis requires detailed clinical examination and electrophysiological findings. This case report presents two male patients with congenital nystagmus examined longitudinally from the age of six months until 17-18 years of age. Clinical and electrophysiological protocols were detailed. The first results showed electronegative electroretinography in the two cases and examination combined with electroretinographic findings helped us to make the diagnosis of Congenital Night Stationary Blindness (CSNB. This diagnosis was confirmed by genetic studies. CSNB is interesting to study because through electrophysiological findings, it enables a better understanding of the physiology of neural transmission in the outer part of the retina.Keywords: Congenital nystagmus, negative electroretinography, congenital night stationary blindness

  1. A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients.

    Science.gov (United States)

    Costello, John P; Weiderhold, Allison; Louis, Clauden; Shaughnessy, Conner; Peer, Syed M; Zurakowski, David; Jonas, Richard A; Nath, Dilip S

    2015-06-01

    The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

  2. Transmyocardial laser revascularization. Early clinical experience

    Directory of Open Access Journals (Sweden)

    Oliveira Sérgio Almeida de

    1999-01-01

    Full Text Available OBJECTIVE: To analyze the initial clinical experience of transmyocardial laser revascularization (TMLR in patients with severe diffuse coronary artery disease. METHODS: Between February, 1998 and February, 1999, 20 patients were submitted to TMLR at the Heart Institute (InCor, University of São Paulo Medical School, Brazil, isolated or in association with conventional coronary artery bypass graft (CABG. All patients had severe diffuse coronary artery disease, with angina functional class III/IV (Canadian Cardiovascular Society score unresponsive to medical therapy. Fourteen patients were submitted to TMLR as the sole therapy, whereas 6 underwent concomitant CABG. Fifty per cent of the patients had either been previously submitted to a CABG or to a percutaneous transluminal coronary angioplasty (PTCA. Mean age was 60 years, ranging from 45 to 74 years. RESULTS: All patients had three-vessel disease, with normal or mildly impaired left ventricular global function. Follow-up ranged from 1 to 13 months (mean 6.6 months, with no postoperative short or long term mortality. There was significant symptom improvement after the procedure, with 85% of the patients free of angina, and the remaining 15 % of the patients showing improvement in functional class, as well as in exercise tolerance. CONCLUSION: This novel technique can be considered a low risk alternative for a highly selected group of patients not suitable for conventional revascularization procedures.

  3. Diagnosis of Congenital Myasthenic Syndromes

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-02-01

    Full Text Available Clinical and neurophysiological data of 11 patients (4 males, 7 females with congenital myasthenic syndromes (CMS diagnosed between 1994 and 2000 are reported from Great Ormond Street Hospital, London, UK.

  4. Genetics Home Reference: congenital hyperinsulinism

    Science.gov (United States)

    ... Z, Arya VB, Hussain K. Hyperinsulinaemic hypoglycaemia:genetic mechanisms, diagnosis and management. J Clin Res Pediatr Endocrinol. ... Shyng SL, Stanley CA. Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel ...

  5. Ullrich Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Goknur Haliloglu

    2011-09-01

    Full Text Available ObjectiveUllrich congenital muscular dystrophy is a rather severe type of congenitalmuscular dystrophy with early onset features related to motor development.In general it is inherited in autosomal recessive principles, however in theWestern world mostly seen with de novo dominant mutations in the collagenVI genes. Milder form of the condition is the Bethlem myopathy. There may beoverlap forms in the clinic resembling the Ehler-Danlos syndrome. There hasbeen some radical efforts for cure especially through the apoptosis cascades.Key words: Ullrich congenital muscular dystrophy, collgen VI genes, Bethlemmyopathy, autophagy.

  6. Ullrich Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Goknur Haliloglu

    2011-06-01

    Full Text Available ObjectiveUllrich congenital muscular dystrophy is a rather severe type of congenital muscular dystrophy with early onset features related to motor development.In general it is inherited in autosomal recessive principles, however in the Western world mostly seen with de novo dominant mutations in the collagen VI genes. Milder form of the condition is the Bethlem myopathy. There may be overlap forms in the clinic resembling the Ehler-Danlos syndrome. There has been some radical efforts for cure especially through the apoptosis cascades.

  7. Prenatal diagnosis and clinical significance of fetal congenital heart disease%胎儿先天性心脏病产前诊断与临床意义

    Institute of Scientific and Technical Information of China (English)

    叶林

    2012-01-01

    Congenital heart disease is one of the most common birth defects.In early pregnancy,congenital heart disease screening methods mainly include neck translucency thickness measurement,ductus venosus flow measurement and fetal echocardiography.In the second trimester fetal heart disease most directly by fetal ultrasound examination for diagnosis of heartbeat,besides cardiac anatomic abnormalities,arrhythmia the diagnosis and assessment of heart function also are the important examination content.Focusing on more congenital heart disease related gene researches can provide reliable basis for prenatal diagnosis.Through the fetal congenital heart disease screening intervention,physicians can help early formulation of clinical decision making,alleviate the burden of the families,which has obvious social and economic benefits.%先天性心脏病是最常见的出生缺陷之一.孕早期先天性心脏病筛查的方法主要有颈后透明层厚度测量、静脉导管血流测量以及直接进行胎儿超声心动图检查等.孕中期绝大部分的胎儿心脏病可以直接通过胎儿超声心动图检查获得诊断,除心脏解剖结构畸形外,心律失常的诊断以及心功能的评估也是重要检查内容.加强先天性心脏病的相关基因研究,也能够为产前诊断提供可靠依据.对胎儿先天性心脏病进行筛查干预,能够帮助医生早期制定临床决策,缓解家庭负担,有明显的社会效益.

  8. Ten novel FBN2 mutations in congenital contractural arachnodactyly : Delineation of the molecular pathogenesis and clinical phenotype

    NARCIS (Netherlands)

    Gupta, PA; Putnam, EA; Carmical, SG; Kaitila, [No Value; Steinmann, B; Child, A; Danesino, C; Metcalfe, K; Berry, SA; Chen, E; Delorme, CV; Thong, MK; Ades, LC; Milewicz, DM

    2002-01-01

    Congenital contractural arachnodactyly (CCA) is an autosomal dominant condition that shares skeletal features with Marfan syndrome (MFS), but does not have the ocular and cardiovascular complications that characterize MFS. CCA and MFS result from mutations in highly similar genes, FBN2 and FBN1, res

  9. Consensus Statement : Chromosomal Microarray Is a First-Tier Clinical Diagnostic Test for Individuals with Developmental Disabilities or Congenital Anomalies

    NARCIS (Netherlands)

    Miller, David T.; Adam, Margaret P.; Aradhya, Swaroop; Biesecker, Leslie G.; Brothman, Arthur R.; Carter, Nigel P.; Church, Deanna M.; Crolla, John A.; Eichler, Evan E.; Epstein, Charles J.; Faucett, W. Andrew; Feuk, Lars; Friedman, Jan M.; Hamosh, Ada; Jackson, Laird; Kaminsky, Erin B.; Kok, Klaas; Krantz, Ian D.; Kuhn, Robert M.; Lee, Charles; Ostell, James M.; Rosenberg, Carla; Scherer, Stephen W.; Spinner, Nancy B.; Stavropoulos, Dimitri J.; Tepperberg, James H.; Thorland, Erik C.; Vermeesch, Joris R.; Waggoner, Darrel J.; Watson, Michael S.; Martin, Christa Lese; Ledbetter, David H.

    2010-01-01

    Chromosomal microarray (CMA) is increasingly utilized for genetic testing of individuals with unexplained developmental delay/intellectual disability (DD/ID), autism spectrum disorders (ASD), or multiple congenital anomalies (MCA). Performing CMA and G-banded karyotyping on every patient substantial

  10. Congenital Pulmonary Alveolar Proteinosis

    OpenAIRE

    Saber Hammami; Khaled Harrathi; Khaled Lajmi; Samir Hadded; Chebil Ben Meriem; Mohamed Néji Guédiche

    2013-01-01

    Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the curr...

  11. Congenital CMV Infection

    Science.gov (United States)

    ... infect the baby. This can happen when a pregnant woman experiences a first-time infection, a reinfection with a different CMV strain (variety), ... passed their newborn hearing test. Diagnosis Congenital CMV ... newborn baby’s saliva, urine, or blood. Such specimens must be collected for ...

  12. Histidine-tryptophan-ketoglutarate solution decreases mortality and morbidity in high-risk patients with severe pulmonary arterial hypertension associated with complex congenital heart disease: an 11-year experience from a single institution

    Science.gov (United States)

    Li, X.W.; Lin, Y.Z.; Lin, H.; Huang, J.B.; Tang, X.M.; Long, X.M.; Lu, W.J.; Wen, Z.K.; Liang, J.; Li, D.Y.; Zhao, X.F.

    2016-01-01

    Cardioplegic reperfusion during a long term ischemic period interrupts cardiac surgery and also increases cellular edema due to repeated solution administration. We reviewed the clinical experiences on myocardial protection of a single perfusion with histidine-tryptophan-ketoglutarate (HTK) for high-risk patients with severe pulmonary arterial hypertension associated with complex congenital heart disease. This retrospective study included 101 high-risk patients undergoing arterial switch operation between March 2001 and July 2012. We divided the cohort into two groups: HTK group, myocardial protection was carried out with one single perfusion with HTK solution; and St group, myocardial protection with conventional St. Thomas' crystalloid cardioplegic solution. The duration of cardiopulmonary bypass did not differ between the two groups. The mortality, morbidity, ICU stay, post-operative hospitalization time, and number of transfusions in HTK group were lower than those in St group (P<0.05). Univariate and multivariate analysis showed that HTK is a statistically significant independent predictor of decreased early mortality and morbidity (P<0.05). In conclusion, HTK solution seems to be an effective and safe alternative to St. Thomas' solution for cardioplegic reperfusion in high-risk patients with complex congenital heart disease. PMID:27191607

  13. Congenitally Deaf Children's Care Trajectories in the Context of Universal Neonatal Hearing Screening: A Qualitative Study of the Parental Experiences

    Science.gov (United States)

    Hardonk, Stefan; Desnerck, Greetje; Loots, Gerrit; Van Hove, Geert; Van Kerschaver, Erwin; Sigurjonsdottir, Hanna Bjorg; Vanroelen, Christophe; Louckx, Fred

    2011-01-01

    The objective of this study is to examine the early care trajectories of congenitally deaf children from a parental perspective, starting with universal neonatal hearing screenings. The analysis using a three-dimensional care trajectory concept is aimed at developing a basic typology of postscreening care trajectories. Children with…

  14. Dengue fever outbreak: a clinical management experience

    International Nuclear Information System (INIS)

    To determine the frequency of dengue as a cause of fever and compare the clinical and haematological characteristics of Dengue-probable and Dengue-proven cases. All patients with age above 14 years, who were either hospitalized or treated in medical outdoor clinic due to acute febrile illness, were evaluated for clinical features of Dengue Fever (DF), Dengue haemorrhagic fever (DHF) and Dengue Shock Syndrome (DSS). Patients showing typical clinical features and haematological findings suggestive of Dengue fever (As per WHO criteria) were evaluated in detail for comparison of probable and confirmed cases of Dengue fever. All other cases of acute febrile illness, not showing clinical features or haematological abnormalities of Dengue fever, were excluded. The clinical and laboratory features were recorded on SPSS 11.0 programme and graded where required, for descriptive and statistical analysis. Out of 5200 patients with febrile illness, 107 (2%) presented with typical features of DF, 40/107 (37%) were Dengue-proven while 67/107 (63%) were Dengue-probable. Out of Dengue-proven cases, 38 were of DF and 2 were of DHF. Day 1 temperature ranged from 99-105 degreeC (mean 101 degree C). Chills and rigors were noticed in 86 (80%), myalgia in 67%, headache in 54%, pharyngitis in 35%, rash in 28%, and bleeding manifestations in 2% cases. Hepatomegaly in 1(0.5%), lymphadenopathy in 1 (0.5%) and splenomegaly in 12 (11.2%) cases. Leucopoenia (count 40 U/L in 57% cases. Frequency of clinically suspected dengue virus infection was 107 (2%), while confirmed dengue fever cases were 40 (0.8%) out of 5200 fever cases. Fever with chills and rigors, body aches, headache, myalgia, rash, haemorrhagic manifestations, platelet count, total leukocyte count, and ALT, are parameters to screen the cases of suspected dengue virus infection, the diagnosis cannot be confirmed unless supported by molecular studies or dengue specific IgM. (author)

  15. Congenital imprinting disorders

    DEFF Research Database (Denmark)

    Eggermann, Thomas; Netchine, Irène; Temple, I Karen;

    2015-01-01

    Imprinting disorders (IDs) are a group of eight rare but probably underdiagnosed congenital diseases affecting growth, development and metabolism. They are caused by similar molecular changes affecting regulation, dosage or the genomic sequence of imprinted genes. Each ID is characterised...... their common underlying (epi)genetic aetiologies, and their basic pathogenesis and long-term clinical consequences remain largely unknown. Efforts to elucidate the aetiology of IDs are currently fragmented across Europe, and standardisation of diagnostic and clinical management is lacking. The new consortium...... EUCID.net (European network of congenital imprinting disorders) now aims to promote better clinical care and scientific investigation of imprinting disorders by establishing a concerted multidisciplinary alliance of clinicians, researchers, patients and families. By encompassing all IDs and establishing...

  16. Congenital dilatation of the large and segmental intrahepatic bile ducts (Caroli's disease in two Golden retriever littermates : clinical communication

    Directory of Open Access Journals (Sweden)

    R.D. Last

    2006-06-01

    Full Text Available Two, sibling, male Golden retriever puppies, 13 weeks of age, were presented with congenital biliary cysts of the liver involving both hepatic and segmental bile ducts, as well as bilateral polycystic kidney disease. Ultrasonography of the livers of both pups demonstrated segmental cystic lesions that were contiguous with the bile ducts. Histopathology revealed cystic ectatic bile duct hyperplasia and dysplasia with variable portal fibrosis in the liver, while in the kidneys there were radially arranged, cylindrically dilated cysts of the collecting ducts, which extended through the medulla and cortex. This pathology was compatible with that of congenital dilatation of the large and segmental bile ducts (Caroli's disease described in humans, dogs and rats. In humans Caroli's disease has an autosomal recessive inheritance pattern, while in rats activation of the MEK5/ERK cascade initiates the biliary dysgenesis of Caroli's disease in this species. However, the exact mode of inheritance and pathogenesis of Caroli's disease in dogs is as yet unknown. Previous reports on congenital hepatic cystic diseases of the dog have described Caroli's disease like lesions in various breeds, but these are believed to be the 1st reported cases in the Golden retriever breed.

  17. Congenital Leukemia

    OpenAIRE

    Raj, Aishwarya; Talukdar, Sewali; Das, Smita; Gogoi, Pabitra Kumar; Das, Damodar; Bhattacharya, Jina

    2013-01-01

    Congenital leukemia is a rare but a well-documented disease in which leukemic process is detected at birth or very shortly thereafter (Philip McCoy and Roy Overton, Commun Clin Cytom 22:85–88, 1995). These leukemias represent approximately 0.8 % of all childhood leukemias. We present a case of congenital acute myeloid leukemia manifesting from the very first day of birth. Diagnosis of acute myeloid leukemia was suspected by the presence of blasts in the peripheral blood smear and was confirme...

  18. Mesenchymal stem cells: from experiment to clinic

    Directory of Open Access Journals (Sweden)

    Otto William R

    2011-09-01

    Full Text Available Abstract There is currently much interest in adult mesenchymal stem cells (MSCs and their ability to differentiate into other cell types, and to partake in the anatomy and physiology of remote organs. It is now clear these cells may be purified from several organs in the body besides bone marrow. MSCs take part in wound healing by contributing to myofibroblast and possibly fibroblast populations, and may be involved in epithelial tissue regeneration in certain organs, although this remains more controversial. In this review, we examine the ability of MSCs to modulate liver, kidney, heart and intestinal repair, and we update their opposing qualities of being less immunogenic and therefore tolerated in a transplant situation, yet being able to contribute to xenograft models of human tumour formation in other contexts. However, such observations have not been replicated in the clinic. Recent studies showing the clinical safety of MSC in several pathologies are discussed. The possible opposing powers of MSC need careful understanding and control if their clinical potential is to be realised with long-term safety for patients.

  19. Microfollicular thyroid adenoma and congenital goitrous hypothyroidism.

    OpenAIRE

    Alabbasy, A J; Delbridge, L.; Eckstein, R; Cowell, C.; Silink, M

    1992-01-01

    Three patients with congenital goitrous hypothyroidism are reported. They were treated with adequate thyroxine replacement and developed well defined microfollicular thyroid adenomas despite being euthyroid clinically and biochemically throughout their clinical course. Patients with congenital goitrous hypothyroidism appear to be at increased risk of developing thyroid adenoma in childhood despite the use of replacement thyroxine treatment in physiological doses.

  20. The experience of black parents/caretakers with the births and care of a child with profound congenital defects

    Directory of Open Access Journals (Sweden)

    M.S. Mabaso

    1990-09-01

    Full Text Available The reaction of Black families to the birth and care of a baby with profound congenital defects was researched using twenty case studies. It was found that the families went through stages of the grieving process, that they shifted from the Western/Christian viewpoint to the traditional viewpoint in their struggle to cope and that they find the existing services grossly inadequate.

  1. Congenital bronchoesophageal fistula in adults

    Institute of Scientific and Technical Information of China (English)

    Bao-Shi Zhang; Nai-Kang Zhou; Chang-Hai Yu

    2011-01-01

    AIM: To study the clinical characteristics, diagnosis and surgical treatment of congenital bronchoesophageal fistulae in adults. METHODS: Eleven adult cases of congenital bronchoesophageal fistula diagnosed and treated in our hospital between May 1990 and August 2010 were reviewed. Its clinical presentations, diagnostic methods, anatomic type, treatment, and follow-up were recorded. RESULTS: Of the chief clinical presentations, nonspecific cough and sputum were found in 10 (90.9%), recurrent bouts of cough after drinking liquid food in 6 (54.6%), hemoptysis in 6 (54.6%), low fever in 4 (36.4%), and chest pain in 3 (27.3%) of the 11 cases, respectively. The duration of symptoms before diagnosis ranged 5-36.5 years. The diagnosis of congenital bronchoesophageal fistulae was established in 9 patients by barium esophagography, in 1 patient by esophagoscopy and in 1 patient by bronchoscopy, respectively. The congenital bronchoesophageal fistulae communicated with a segmental bronchus, a main bronchus, and an intermediate bronchus in 8, 2 and 1 patients, respectively. The treatment of congenital bronchoesophageal fistulae involved excision of the fistula in 10 patients or division and suturing in 1 patient. The associated lung lesion was removed in all patients. No long-term sequelae were found during the postoperative follow-up except in 1 patient with bronchial fistula who accepted reoperation before recovery. CONCLUSION: Congenital bronchoesophageal fistula is rare in adults. Its most useful diagnostic method is esophagography. It must be treated surgically as soon as the diagnosis is established.

  2. Insomnia: clinical experience with zolpidem (sanval

    Directory of Open Access Journals (Sweden)

    Yakov Iosifovich Levin

    2010-01-01

    Full Text Available The paper describes the present view of the problem of insomnia and gives a classification of sleep disorders and basic methods for their drug and non-drug therapy. Emphasis is placed on the role of the objective sleep study - polysomnography. The use of the current hypnotics belonging to a three Zs group and the minimization of administration of benzodiazepines are most important in pharmacotherapy for insomnia. The results of a clinical polysomnographic study of the effect of Zolpidem (Sanval in patients with insomnia are presented. The subjective evaluation of the beneficial effect of a 10-day course of Sanval is confirmed by the objective studies of the sleep pattern undergoing positive changes in the most important indicators, such as the process of falling asleep, the time of intrasleep awakenings, and the duration of Δ-sleep. The high safety and good tolerability of Sanval permit the latter to be assessed as an effective agent for the treatment of insomnia.

  3. Digital mammography: Experiences in clinical application

    International Nuclear Information System (INIS)

    Target: In 1989 in the Kantonal Hospital of Lucerne conventional film-screen mammography was replaced by digital mammography. With the support of a retrospective study, it was checked whether or not digital mammography represents an equally valid diagnostic procedure in daily routine. Methods: 1204 patients were examined using digital mammography. A reevaluation of these patients was carried out using clinical and r[iological routine controls. [ditionally a r[iological and histological examination was performed in 127 cases in which excisional biopsies h[ been done, paying particular attention to detail perception. Results: The sensitivity of digital mammography achieved a total of 85%, whereas the accuracy was 81%. With [ditional use of ultrasound and galactography the sensitivity attained 91%. By reevaluation the sensitivity amounted to 87%, the accuracy remaining at 81%. The positive predictive value was especially high with 76% and 77%. Conclusion: Digital mammography offers satisfactory diagnostic performance. (orig.)

  4. Congenital Anomalies in Infant with Congenital Hypothyroidism

    OpenAIRE

    Zahra Razavi; Alireza Yavarikia; Saadat Torabian

    2012-01-01

    bjective: Congenital hypothyroidism is characterized by inadequate thyroid hormone production in newborn infants. Many infants with CH have co-occurring congenital malformations. This is an investigation on the frequency and types of congenital anomalies in infants with congenital hypothyroidism born from May 2006-2010 in Hamadan, west province of Iran.Methods: The Iranian neonatal screening program for congenital hypothyroidism was initiated in May 2005. This prospective descriptive study wa...

  5. Mental and behavioural disorders among people with congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2011-01-01

    95 congenitally deafblind adults. Seventy-four percent were found to have a mental and/or behavioral diagnose. Mental retardation was found among 34%, psychosis among 13%. Mental and behavioral disorders, especially with symptoms of psychosis and mental retardation, are common among people......The population of people with congenital deafblindness faces challenges concerning communication and mobility. Due to the significance of the sensory loss it is difficult to diagnose mental and behavioral disorders. This article investigates the prevalence of mental and behavioral disorders among...... with congenital deafblindness. Clinical experience is needed, as well as cross-disciplinary cooperation and specialized diagnostic methods together with a observation and intervention period in order to be able to assess and differentiate mental and behavioral symptoms from sensory deprivation in people...

  6. Cartap hydrochloride poisoning: A clinical experience

    Directory of Open Access Journals (Sweden)

    Hari K Boorugu

    2012-01-01

    Full Text Available Cartap hydrochloride, a nereistoxin analog, is a commonly used low toxicity insecticide. We describe a patient who presented to the emergency department with alleged history of ingestion of Cartap hydrochloride as an act of deliberate self-harm. The patient was managed conservatively. To our knowledge this is the first case report of Cartap hydrochloride suicidal poisoning. Cartap toxicity has been considered to be minimal, but a number of animal models have shown significant neuromuscular toxicity resulting in respiratory failure. It is hypothesized that the primary effect of Cartap hydrochloride is through inhibition of the [ 3 H]-ryanodine binding to the Ca 2+ release channel in the sarcoplasmic reticulum in a dose-dependent manner and promotion of extracellular Ca 2+ influx and induction of internal Ca 2+ release. This results in tonic diaphragmatic contraction rather than paralysis. This is the basis of the clinical presentation of acute Cartap poisoning as well as the treatment with chelators namely British Anti Lewisite and sodium dimercaptopropane sulfonate.

  7. Eosinophilic gastroenteritis: Clinical experience with 15 patients

    Institute of Scientific and Technical Information of China (English)

    Ming-Jen Chen; Cheng-Hsin Chu; Shee-Chan Lin; Shou-Chuan Shih; Tsang-En Wang

    2003-01-01

    AIM: To evaluate the clinic features of eosinophilicgastroenteritis and to examine the diagnosis, treatment,long-term outcome of this disease.METHODS: Charts with a diagnosis of eosinophilicgastroenteritis from 1984 to 2002 at Mackay Memorial Hospital were reviewed retrospectively. There were 15 patients diagnosed with eosinophilic gastroenteritis. The diagnosis was established in 13 by histologic evaluation of endoscopic biopsy or operative specimen and in 2 by radiologic imaging and the presence of eosinophilic ascites.RESULTS: All the patients had gastrointestinal symptoms and 12 (80 %) had hypereosinophilia (absolute eosinophil count 1 008 to 31 360/cm3). The most common symptoms were abdominal pain and diarrhea. Five of the 15 patients had a history of allergy. Seven patients had involvement of the mucosa, 2 of muscularis, and 6 of subserosa. One with a history of seafood allergy was successfully treated with an elimination diet. Another patient improved spontaneously after fasted for several days. The remaining 13 patients were treated with oral prednisolone, 10 to 40 mg/day initially,which was then tapered. The symptoms in all the patients subsided within two weeks. Eleven of the 15 patients were followed up for more than 12 months (12 to 104 months,mean 48.7), of whom 5 had relapses after discontinuing steroids (13 episodes). Two of these patients required longterm maintenance oral prednisolone (5 to 10 mg/day).CONCLUSION: Eosinophilic gastroenteritis is a rare condition of unclear etiology characterized by relapses and remissions. Short courses of corticosteroids are the mainstay of treatment, although some patients with relapsing disease require long-term low-dose steroids.

  8. Ameloblastoma: Our clinical experience with 68 cases

    Directory of Open Access Journals (Sweden)

    Benjamin Fomete

    2014-01-01

    Full Text Available Introduction: In this environment, previous workers have reported on the challenges of managing large sized ameloblastoma of the jaws with less than adequate facilities. The aim of this review is to present the management of 68 cases of ameloblastoma with emphasis on surgical care. Materials and Methods: Retrospective survey of case notes of patients with histopathologic diagnosis of ameloblastoma (using the criteria of Barnes et al., 2005 seen between January 2006 and August 2010 at the Maxillofacial Unit, Ahmadu Bello University Teaching Hospital, Shika-Zaria, Nigeria was undertaken. Data collected includes histopathological diagnosis, age, gender, clinical information on site of lesion, form of intubation and surgical procedure performed. Results: Out of 94 patients, 68 with histological diagnosis of ameloblastoma (59 mandibular and 9 maxillary were operated within the study period. The remainder (26 was not treated in hospital. Among 68 patients treated, more were males (38 than females (30, giving a male to female ratio of 1.3:1. The age range was between 14 and 74 years (mean-standard deviation. The duration of the symptoms ranged from 7 months to 24 years, most were follicular ameloblastoma (n = 13 followed by acanthomatous type (n = 7. Endotracheal intubation was the most common (n = 55 followed by fiber optic laryngoscopy (n = 8. The surgical approach most used was extended Risdon with intraoral (n = 24 followed by extended Risdon with lip split and intraoral (n = 17. Segmental resection (en block formed the bulk of our procedures (n = 22 followed by subtotal mandibulectomy (n = 16. Conclusion: The treatment of ameloblastoma remains controversial. Its destructive nature has left patients with wide defects difficult to reconstruct.

  9. CLINICAL EXPERIENCE WITH METABOLIC THERAPY FOR BRAIN ISCHEMIA

    Directory of Open Access Journals (Sweden)

    M. Kh. Shurdumova

    2013-01-01

    Full Text Available The paper describes clinical experience with metabolic therapy, including neuroprotective drugs and antioxidants, for cerebrovascular diseases.It gives the results of basic Russian and foreign clinical studies of ethylmethylhydroxypyridoxine succinate and choline alfoscerate and discusses their efficacy and routes of administration.

  10. CLINICAL EXPERIENCE WITH METABOLIC THERAPY FOR BRAIN ISCHEMIA

    Directory of Open Access Journals (Sweden)

    M. Kh. Shurdumova

    2014-07-01

    Full Text Available The paper describes clinical experience with metabolic therapy, including neuroprotective drugs and antioxidants, for cerebrovascular diseases.It gives the results of basic Russian and foreign clinical studies of ethylmethylhydroxypyridoxine succinate and choline alfoscerate and discusses their efficacy and routes of administration.

  11. Lagged Syndesmotic Fixation: Our Clinical Experience.

    Science.gov (United States)

    Kwaadu, Kwasi Yiadom; Fleming, Justin James; Salmon, Trudy

    2015-01-01

    Ankle fractures are very common, and although algorithms are in place for osseous management, consensus has not been reached regarding treatment of associated ligamentous injuries. Although tibiofibular syndesmotic stabilization can be done using different forms of fixation, the biomedical literature has long emphasized the risk of long-term restriction of ankle mobility with the use of lagged transfixation. However, when reduction cannot be maintained with positional fixation, we found that lagging the syndesmotic screw helped to maintain the reduction without causing functional restriction. In this report, we describe our experience with patients who had undergone lagged tibiofibular transfixation and were available for short- to intermediate-term follow-up to assess ankle function. A total of 31 patients (32.63% of 95 consecutive patients) were available at a mean of 34.87 (range 18 to 52) months to complete the American Orthopedic Foot and Ankle Society ankle-hindfoot questionnaire. The mean score was 88.38 (range 42 to 100) points at a mean follow-up interval of 34.87 (range 18 to 52) months. Of 31 patients, 19 had an AOFAS score of 90 points, 9 an AOFAS score of 80 to 89 points, 2 an AOFAS score of 60 to 69 points, and 1 an AOFAS score of ankle kinematics than positional syndesmotic fixation. PMID:25736445

  12. Neonatal Sludge: A finding of congenital hypothyroidism

    OpenAIRE

    Kurtoğlu, Selim; Çoban, Dilek; Akın, Mustafa Ali; Akın, Leyla; Yıkılmaz, Ali

    2010-01-01

    Congenital hypothyroidism is one of the most urgent diseases of the neonate. When diagnosed and treated at an early stage, its most important complication, mental retardation, is preventable. The signs of congenital hypothyroidism are nonspecific in neonates. Only 5% of the cases have characteristic clinical findings. One of the most important and earliest signs is prolonged jaundice during the neonatal period. We report herein a case of congenital hypothyroidism, who presented with icterus a...

  13. [Congenital aniridia].

    Science.gov (United States)

    Chiruţa, Daria; Stan, Cristina

    2014-01-01

    Aniridia is a rare congenital, hereditary, bilateral disease which is associated with various systemic and ocular defects. We present the case of a 61 year old patient who was admitted in the hospital of ophthalmology Cluj Napoca, for the symptoms caused by the ocular defects associated with aniridia. In this case, aniridia is autosomal dominant transmitted with incomplete penetrance and it is not accompanied by any systemic defects. The disease also affects three of her sons and two nephews of the patient.

  14. Congenital diplopodia

    Energy Technology Data Exchange (ETDEWEB)

    Brower, Jason S.; Wootton-Gorges, Sandra L.; Costouros, John G.; Boakes, Jennette; Greenspan, Adam [University of California, Davis, Department of Radiology, 4860 Y. Street, Suite 3100, CA 95817, Davis (United States)

    2003-11-01

    Diplopodia, or duplicated foot, is a rare congenital anomaly. It differs from polydactyly in that supernumerary metatarsal and tarsal bones are present as well as extra digits. Only a few cases of this anomaly have been reported in the literature to date. We present a newborn male without intrauterine teratogen exposure who was born with a duplicate foot of the left lower extremity and imperforate anus. (orig.)

  15. Congenital Thrombocytopenia

    Institute of Scientific and Technical Information of China (English)

    王兆钺

    2011-01-01

    @@ Platelets are essential for normal hemostasis.Platelets adhere to damaged blood vessels, and then aggregate and promote activation of coagulation factors, resulting to ceasing bleeding.Both quantitative and qualitative abnormalities of platelets can cause bleeding problems.Among them, immune thrombocytopenias are the most common conditions.However, congenital thrombocytopenias are often neglected because of their relative rarity and complex laboratory tests.That causes misdiagnosis and unnecessary and potentially harmful treatments for many patients.

  16. Clinical and Applied Experience in Rehabilitation Counselor Education

    Science.gov (United States)

    Tschopp, Molly K.; Chronister, Julie A.

    2008-01-01

    Applied training of pre-practicum, practicum, and internship are important gateway experiences for rehabilitation counselors-in-training. Counselor educators and supervisors must be aware of requirements and expectations of counselor-in-training supervision and common ethical issues specific to these clinical experiences of rehabilitation…

  17. COMPARISON OF COUPLES REFERRED AND NOT REFERRED FOR GENETIC-COUNSELING IN A GENETIC CLINIC AFTER THE BIRTH OF A CHILD WITH A CONGENITAL ANOMALY - A STUDY IN A POPULATION IN THE NORTHEASTERN NETHERLANDS

    NARCIS (Netherlands)

    CORNEL, MC; VANESSEN, AJ; TENKATE, LP

    1992-01-01

    After the birth of a child with a congenital anomaly, parents have many questions about cause, prognosis, and recurrence risk. An important means of transmitting such information is referral to a genetic clinic. We were interested in knowing what determines whether or not parents are referred for ge

  18. Color Doppler Ultrasound Screening of Infants with Congenital Heart Disease Clinical Analysis%彩超筛查婴幼儿先天性心脏病临床分析

    Institute of Scientific and Technical Information of China (English)

    秦旭红; 王博; 余蓝

    2015-01-01

    目的 探讨彩超筛查婴幼儿先天性心脏病价值.方法 对800例婴儿临床资料进行回顾性分析,均为出生后42d给予彩色多普勒超声检查,掌握先天性心脏病发生情况.结果 对800例婴儿临床资料分析可知,共检出先天性心脏病患儿10例.结论 彩超筛查婴幼儿先天性心脏病能够在胎儿产前畸形筛查早发现早治疗.提高临床治疗的有效率,值得临床大力推广.%Objective To investigate the value of color Doppler ultrasound in screening infants with congenital heart disease.MethodsThe clinical data of 800 infants were analyzed retrospectively, and al of the patients were born with 42d after color Doppler ultrasound examination, and mastered the occurrence of congenital heart disease.ResultsThe clinical data of 800 infants were analyzed, and 10 cases of congenital heart disease were detected.Conclusion Color Doppler ultrasound screening for congenital heart disease in infants and early detection of prenatal malformation screening early detection and treatment. Improve the clinical treatment efficiency, it is worthy of clinical promotion.

  19. Congenital Cleft Hand

    OpenAIRE

    Aritamur, Ayhan; Cakmak, Mehmet; Taser, Omer; Berk, Hasan

    2004-01-01

    Congenital cleft hand deformity, which is also known with such names as cleft hand, lobster claw hand and Ectrodactyiy, is characterized by the absence of one or two fingers in the mid portion of the hand. A case of bilateral cleft hand deformity four years old, which is considerabiy rare, was reconstructed surgically. The result obtained has been presented. Because no sufficient experience has accumulated due to the fact that it is observed considerably rare, the therapeutical principales re...

  20. Validity of Sildenafil Test in Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease According to Clinical and Echocardiographic Parameters

    Directory of Open Access Journals (Sweden)

    Akbar Shahmohammadi

    2009-06-01

    Full Text Available Background: Pulmonary arterial hypertension is a complication of most congenital heart diseases. We sought to assess the effect of sildenafil on patients suffering from pulmonary arterial hypertension in association with congenital heart disease on the basis of clinical and echocardiographic parameters and compare the catheterization and treatment results so as to evaluate the predictive value of sildenafil on the operability of patients. Methods: After primary echocardiography, 21 patients were selected for the final study with a diagnosis of moderate-to-large ventricular septal defect and pulmonary artery hypertension. They were divided into 3 age groups: younger than one year, 1 to 2 years, and older than 2 years. Before and one hour after the consumption of sildenafil, the patients had their oxygen saturation and blood pressure measured. Additionally, the patients underwent echocardiography and cardiac catheterization. The patients’ operability was determined on the basis of their clinical condition and their response to oxygen inhalation in the catheterization room. Finally, the results of the drug response and final treatment were analyzed statistically. Results: The 21 patients, who had ventricular septal defect and pulmonary arterial hypertension, were comprised of 8 (38% boys and 13 (62% girls. The patients aged from 2.5 to 204 months (mean 30 months. It was clear that the younger patients had a more positive response to the drug. All the patients who had a positive response to the drug were considered operable after catheterization and all of them had a positive response to treatment. There was no significant correlation between operable/inoperable conditions and response to treatment (P value=0.262, while there was a very significant correlation between response to treatment and response to drug (P value=0.005. Conclusion: According to the results of this study and given the low cost and availability of sildenafil and its oral

  1. Clinical Positioning Space: Residents' Clinical Experiences in the Outpatient Oncology Clinic.

    Science.gov (United States)

    Williams, Lars H; Christensen, Mette K; Rytter, Carsten; Musaeus, Peter

    2015-09-01

    In this article, we present a case study of residents' clinical experiences and communication in outpatient oncology consultations. We apply positioning theory, a dynamic alternative to role theory, to investigate how oncology residents and patients situate themselves as persons with rights and duties. Drawing from seven qualitative interviews and six days of observation, we investigate the residents' social positioning and their conversations with patients or supervisors. Our focus is on how (a) relational shifts in authority depend on each situation and its participants; (b) storylines establish acts and positions and narratively frame what participants can expect from a medical consultation viewed as a social episode; and (c) the positioning of rights and duties can lead to misunderstandings and frustrations. We conclude that residents and patients locate themselves in outpatient conversations as participants who jointly produce and are produced by patients' and nurses' storylines about who should take responsibility for treatment. PMID:25288406

  2. Parenteral nutrition combined with rice soup can be a safe and effective intervention for congenital chylous ascites.

    Science.gov (United States)

    Cao, Yi; Yan, Weihui; Lu, Lina; Tao, Yijing; Lu, Wei; Chen, Yingwei; Tang, Qingya; Cai, Wei

    2016-01-01

    Congenital chylous ascites in the neonatal period is a rare entity. Total parenteral nutrition (TPN), medium chain triglyceride (MCT)-based diet, octreotide and repeated paracentesis are regarded as appropriate medical treatment for congenital chylous ascites, and surgery is recommended when conservative therapy has failed. We present two cases in which ascites were confirmed via an abdominal sonogram and diagnostic paracentesis. In our clinical experience, rice soup combined with PN can be a safe and effective intervention. PMID:27440699

  3. Congenital lipodystrophies and dyslipidemias.

    Science.gov (United States)

    Prieur, Xavier; Le May, Cedric; Magré, Jocelyne; Cariou, Bertrand

    2014-09-01

    Lipodystrophies are rare acquired and genetic disorders characterized by the selective loss of adipose tissue. One key metabolic feature of patients with congenital inherited lipodystrophy is hypertriglyceridemia. The precise mechanisms by which the lack of adipose tissue causes dyslipidemia remain largely unknown. In recent years, new insights have arisen from data obtained in vitro in adipocytes, yeast, drosophila, and very recently in several genetically modified mouse models of generalized lipodystrophy. A common metabolic pathway involving accelerated lipolysis and defective energy storage seems to contribute to the dyslipidemia associated with congenital generalized lipodystrophy syndromes, although the pathophysiological changes may vary with the nature of the mutation involved. Therapeutic management of dyslipidemia in patients with lipodystrophy is primarily based on specific approaches using recombinant leptin therapy. Preclinical studies suggest a potential efficacy of thiazolidinediones that remains to be assessed in dedicated clinical trials.

  4. Congenital intestinal lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Popović Dušan Đ.

    2011-01-01

    Full Text Available Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  5. Pre-clinical medical student experience in a pediatric pulmonary clinic

    Directory of Open Access Journals (Sweden)

    Thomas G. Saba

    2015-11-01

    Full Text Available Objective: Our objective was to evaluate the educational value of introducing pre-clinical medical students to pediatric patients and their families in a subspecialty clinic setting. Methods: First- and second-year medical students at the University of Michigan seeking clinical experience outside of the classroom attended an outpatient pediatric pulmonary clinic. Evaluation of the experience consisted of pre- and post-clinic student surveys and post-clinic parent surveys with statements employing a four-point Likert scale as well as open-ended questions. Results: Twenty-eight first-year students, 6 second-year students, and 33 parents participated in the study. Post-clinic statement scores significantly increased for statements addressing empathic attitudes, confidence communicating with children and families, comfort in the clinical environment, and social awareness. Scores did not change for statements addressing motivation, a sense of team membership, or confidence with career goals. Students achieved their goals of gaining experience interacting with patients, learning about pulmonary diseases, and observing clinic workflow. Parents felt that they contributed to student education and were not inconvenienced. Conclusions: Students identified several educational benefits of exposure to a single pediatric pulmonary clinic. Patients and families were not inconvenienced by the participation of a student. Additional studies are warranted to further investigate the value of this model of pre-clinical medical student exposure to subspecialty pediatrics.

  6. Congenital hypothyroidism presenting with postpartum bradycardia

    International Nuclear Information System (INIS)

    Congenital hypothyroidism is a clinical condition characterized by lack of thyroid hormone because of thyroid gland developmental and thyroid hormone biosynthesis disorders. The most common cause of permanent hypothyroidism is congenital factors. Prompt diagnosis is critical. However, overt signs of hypothyroidism are rarely present at birth, and 95% of affected babies are asymptomatic. Hypoxemia, apnea, acidosis, increased intracranial pressure, vagal stimulus and central nerve system abnormalities represent the most common causes of bradycardia in the neonate. Bradycardia associated with congenital hypothyroidism is very rare. In this paper, a case of severe congenital hypothyroidism, induced by maternal blocker antibodies, who presented with bradycardia, is reported. (author)

  7. Clinical Needs Finding: Developing the Virtual Experience, A Case Study

    Science.gov (United States)

    Mittal, Vaishali; Thompson, Megan; Altman, Stuart M; Taylor, Peter; Summers, Alexander; Goodwin, Kelsey; Louie, Angelique Y

    2013-01-01

    We describe an innovative program at the University of California, Davis for students to engage in clinical needs finding. Using a team-based approach, students participated in clinical rotations to observe firsthand the needs of clinicians at the university affiliated medical center. The teams were asked to develop documentary-style videos to capture key experiences that would allow future viewers to use the videos as “virtual” clinical rotations. This was conceived as a strategy to allow students in prohibitively large classes, or students in programs at institutions without associated medical or veterinary school programs, to experience clinical rotations and perform needs assessments. The students' perspectives on the experience as well as instructor analysis of best practices for this type of activity are presented and discussed. We found that the internship experience was valuable to the students participating, by not only introducing the practice of needs finding but for increasing the students' confidence in the practice of engineering design and their ability to work independently. The videos produced were of such high quality that instructors from other institutions have requested copies for instructional use. Virtual clinical rotations through video experiences may provide a reasonable substitute for students who do not have the ability to participate in rotations in person. PMID:23483373

  8. Congenital hypoaldosteronism.

    Science.gov (United States)

    Sethupathi, Vanathi; Vijayakumar, M; Janakiraman, Lalitha; Nammalwar, B R

    2008-08-01

    Congenital hypoaldosteronism due to an isolated aldosterone biosynthesis defect is rare. We report a 4 month old female infant who presented with failure to thrive, persistent hyponatremia and hyperkalemia. Investigations revealed normal serum 17 hydroxy progesterone and cortisol. A decreased serum aldosterone and serum 18 hydroxy corticosterone levels with a low 18 hydroxy corticosterone: aldosterone ratio was suggestive of corticosterone methyl oxidase type I deficiency. She was started on fludrocortisone replacement therapy with a subsequent normalization of electrolytes. Further molecular analysis is needed to ascertain the precise nature of the mutation.

  9. Bone conduction hearing in congenital aural atresia.

    Science.gov (United States)

    Zhang, Lichun; Gao, Na; Yin, Yanbo; Yang, Lin; Xie, Youzhou; Chen, Ying; Dai, Peidong; Zhang, Tianyu

    2016-07-01

    Previous researches focusing on BC hearing mechanisms proved that the two routes, (1) EAC sound radiation and (2) inertial of ossicular chain, partially contribute to normal BC hearing. Therefore, the BC hearing for those patients with congenital aural atresia should partially decrease theoretically due to their abnormal anatomy. However, there are not many studies which mention these patients' BC hearing up till now. The objective of this study is to investigate congenital aural atresia patient's BC hearing by analysis of pre-surgical audiogram and to study their potential BC hearing mechanisms using animal modeling and their ABR measurements. The study methoed involves analyzing 75 patients' pre-operative audiogram. Then we produced an animal model by surgery to measure their BC hearing threshold changes. Clinical data showed that those patients had some BC hearing loss; and there were 25 cases (25/75, 33.3 %) which present with typical Carhart's Notch. The animal experiments proved that inertia of ossicular chain contribute to partial BC hearing, which demonstrated that the inertia produced more affects on high frequencies by comparing with low frequencies. The patients with congenital aural atresia present BC hearing loss, which could be mainly ascribed to the absence of inertia of ossicular chain. PMID:26205153

  10. Community based clinical program: the Medunsa physiotherapy students` experience

    Directory of Open Access Journals (Sweden)

    N. P. Taukobong

    2004-02-01

    Full Text Available Backgound: The aim of community based clinical training is tproduce graduates who are responsive to the health needs of their communit It is envisaged that upon completion of training graduates would go back an serve their respective communities following exposure to community need Program evaluation should therefore allow students to express the inadequacie and strengths of the program.Aim: To evaluate the community-based clinical program through student's experiences.Methodology: A qualitative research design was used. End of block students reports for both third (8 and fourth (15 year physiotherapy students (n = 23 were used to collect the data. Responses in the reports were grouped into the following categories for purpose of data analysis: feeling about the block, suggestion/s and supervision.Results: The students described the community based clinical program as an unique learning experience which equipped them with the understanding of life within communities. Sixty five percent (65% expressed satisfaction with the supervision given. The main complaints were amounts of paper work involved and clinical workload.Conclusion: The student's experiences indicated that the community-based clinical program within the MEDUNSA physiotherapy department realizes the goal of community-based clinical training as determined by WHO, except for inclusion of some multi-professional approaches and adaptation of the supervision provided.

  11. Congenital myasthenic syndrome due to mutation in CHRNE gene with clinical worsening and thymic hyperplasia attributed to association with autoimmune-myasthenia gravis.

    Science.gov (United States)

    Santos, Ernestina; Moreira, Isabel; Coutinho, Ester; Gonçalves, Guilherme; Lopes, Carlos; Lopes Lima, José; Leite, M Isabel

    2015-12-01

    We report a patient with congenital myasthenic syndrome (CMS) due to mutation in CHRNE with symptoms since the age of 4; mild to moderate fatigable weakness involved mainly ocular, bulbar and limb muscles; functional impact of the disease in their development and physical activity was modest. By the age of 34, the patient experienced gradual worsening of fatigue with dyspnoea and pronounced limb weakness, requiring significant increase of pyridostigmine. Further, a remarkable and sustained clinical improvement followed thymectomy with hyperplastic thymus. Despite of the absence of detectable antibodies to acetyl-choline receptor (AChR) (including clustered-AChR), muscle-specific kinase and low-density lipoprotein receptor-related protein-4 antibodies in the serum obtained nine years after thymectomy, the clinical, genetic and histological features are in keeping with the extremely rare association of two rare neuromuscular junction disorders - CMS and myasthenia gravis (MG). The inexistence of other conditions that could potentially associate with thymic hyperplasia also supports the diagnosis of MG. PMID:26363966

  12. Diagnostic value and clinical problems of MR imaging in congenital anomalies of the central nervous system, 2. Spinal dysraphisms

    Energy Technology Data Exchange (ETDEWEB)

    Oi, Shizuo; Urui, Seishiro; Asano, Noboru; Masumura, Michio; Shose, Yoshiteru; Matsumoto, Satoshi

    1987-06-01

    Spina bifida and associated congenital anomalies in the central nervous system were evaluated by means of MRI, and the results compared with those obtained by conventional diagnostic procedures. Using the two-dimensional Fourier transform technique, a three-radiofrequency-pulse sequence (inversion recovery: IR 2100/500; spin-echo: SE 2100/40 or 2100/80) was routinely applied. Compared with X-ray CT, MR proved to be more accurate in the detection of the pathoanatomical relation between the lesion and the spinal cord, or that between the spinal dysraphic state and associated intracranial anomalies. MRI was also superior in the anatomical diagnosis of a spinal lipoma, a tethered cord, syringobulbia, syringomyelia, the Chiari anomaly, and so forth. The most considerable disadvantage of MRI in the diagnosis of the spina bifida is the poor information it provides about the bifid spine itself, but this information may be obtained by the use of conventional diagnostic procedures. Also, a regular-conducting MRI system is still insufficient to demonstrate the precise location of the canda equina nerve roots, especially in relation to a lipoma, although the spin-echo MR myelographic technique was helpful in demarcating the major structures, such as the lipoma and the cord. In syringomyelia and syringobulbia, further invasive study in analyzing the fluid dynamics is needed to determine the proper operative procedure. It was emphasized in this study that MRI is an extremely valuable diagnostic tool also in the diagnosis of spinal dysraphism, especially in the detection of a pathoanatomical structure, but can also be expected to be improved so as to make possible finer anatomical analysis and provide a higher quality of information on the fluid dynamics, at least so as to indicate operative procedures without any invasive methods. (J.P.N.).

  13. Diagnostic value and clinical problems of MR imaging in congenital anomalies of the central nervous system, 2

    International Nuclear Information System (INIS)

    Spina bifida and associated congenital anomalies in the central nervous system were evaluated by means of MRI, and the results compared with those obtained by conventional diagnostic procedures. Using the two-dimensional Fourier transform technique, a three-radiofrequency-pulse sequence (inversion recovery: IR 2100/500; spin-echo: SE 2100/40 or 2100/80) was routinely applied. Compared with X-ray CT, MR proved to be more accurate in the detection of the pathoanatomical relation between the lesion and the spinal cord, or that between the spinal dysraphic state and associated intracranial anomalies. MRI was also superior in the anatomical diagnosis of a spinal lipoma, a tethered cord, syringobulbia, syringomyelia, the Chiari anomaly, and so forth. The most considerable disadvantage of MRI in the diagnosis of the spina bifida is the poor information it provides about the bifid spine itself, but this information may be obtained by the use of conventional diagnostic procedures. Also, a regular-conducting MRI system is still insufficient to demonstrate the precise location of the canda equina nerve roots, especially in relation to a lipoma, although the spin-echo MR myelographic technique was helpful in demarcating the major structures, such as the lipoma and the cord. In syringomyelia and syringobulbia, further invasive study in analyzing the fluid dynamics is needed to determine the proper operative procedure. It was emphasized in this study that MRI is an extremely valuable diagnostic tool also in the diagnosis of spinal dysraphism, especially in the detection of a pathoanatomical structure, but can also be expected to be improved so as to make possible finer anatomical analysis and provide a higher quality of information on the fluid dynamics, at least so as to indicate operative procedures without any invasive methods. (J.P.N.)

  14. Early clinical experience: do students learn what we expect?

    NARCIS (Netherlands)

    Helmich, E.; Bolhuis, S.; Laan, R.F.J.M.; Koopmans, R.T.C.M.

    2011-01-01

    CONTEXT: Early clinical experience is thought to contribute to the professional development of medical students, but little is known about the kind of learning processes that actually take place. Learning in practice is highly informal and may be difficult to direct by predefined learning outcomes.

  15. A qualitative study of constructive clinical learning experiences.

    Science.gov (United States)

    van der Hem-Stokroos, H H; Daelmans, H E M; van der Vleuten, C P M; Haarman, H J Th M; Scherpbier, A J J A

    2003-03-01

    Little is known about the effectiveness of clinical education. A more educational structure is considered to be potentially beneficial. The following structured components were added to a surgical clerkship: logbooks, an observed student-patient encounter, individual appraisals, feedback on patient notes, and (case) presentations by students. The authors organized two focus-group sessions in which 19 students participated to explore their perceptions about effective clinical learning experiences and the newly introduced structured components. The analysis of the transcripts showed that observation and constructive feedback are key features of clinical training. The structured activities were appreciated and the results show the direction to be taken for further improvement. Learning experiences depended vastly on individual clinicians' educational qualities. Students experienced being on call, assisting in theatre and time for self-study as instructive elements. Recommended clerkship components are: active involvement of students, direct observation, selection of teachers, a positive learning environment and time for self-study. PMID:12745517

  16. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  17. Congenital granular cell epulis.

    Science.gov (United States)

    Conrad, Rachel; Perez, Mia C N

    2014-01-01

    Congenital granular cell epulis is a rarely reported lesion of unknown histogenesis with a strong predilection for the maxillary alveolar ridge of newborn girls. Microscopically, it demonstrates nests of polygonal cells with granular cytoplasm, a prominent capillary network, and attenuated overlying squamous epithelium. The lesion lacks immunoreactivity for S-100, laminin, chromogranin, and most other markers except neuron-specific enolase and vimentin. Through careful observation of its unique clinical, histopathologic, and immunohistochemical features, this lesion can be distinguished from the more common adult granular cell tumor as well as other differential diagnoses.

  18. Chromosomal microarray analysis as a first-tier clinical diagnostic test: Estonian experience.

    Science.gov (United States)

    Zilina, Olga; Teek, Rita; Tammur, Pille; Kuuse, Kati; Yakoreva, Maria; Vaidla, Eve; Mölter-Väär, Triin; Reimand, Tiia; Kurg, Ants; Ounap, Katrin

    2014-03-01

    Chromosomal microarray analysis (CMA) is now established as the first-tier cytogenetic diagnostic test for fast and accurate detection of chromosomal abnormalities in patients with developmental delay/intellectual disability (DD/ID), multiple congenital anomalies (MCA), and autism spectrum disorders (ASD). We present our experience with using CMA for postnatal and prenatal diagnosis in Estonian patients during 2009-2012. Since 2011, CMA is on the official service list of the Estonian Health Insurance Fund and is performed as the first-tier cytogenetic test for patients with DD/ID, MCA or ASD. A total of 1191 patients were analyzed, including postnatal (1072 [90%] patients and 59 [5%] family members) and prenatal referrals (60 [5%] fetuses). Abnormal results were reported in 298 (25%) patients, with a total of 351 findings (1-3 per individual): 147 (42%) deletions, 106 (30%) duplications, 89 (25%) long contiguous stretches of homozygosity (LCSH) events (>5 Mb), and nine (3%) aneuploidies. Of all findings, 143 (41%) were defined as pathogenic or likely pathogenic; for another 143 findings (41%), most of which were LCSH, the clinical significance remained unknown, while 61 (18%) reported findings can now be reclassified as benign or likely benign. Clinically relevant findings were detected in 126 (11%) patients. However, the proportion of variants of unknown clinical significance was quite high (41% of all findings). It seems that our ability to detect chromosomal abnormalities has far outpaced our ability to understand their role in disease. Thus, the interpretation of CMA findings remains a rather difficult task requiring a close collaboration between clinicians and cytogeneticists.

  19. E-health stakeholders experiences with clinical modelling and standardizations.

    Science.gov (United States)

    Gøeg, Kirstine Rosenbeck; Elberg, Pia Britt; Højen, Anne Randorff

    2015-01-01

    Stakeholders in e-health such as governance officials, health IT-implementers and vendors have to co-operate to achieve the goal of a future-proof interoperable e-health infrastructure. Co-operation requires knowledge on the responsibility and competences of stakeholder groups. To increase awareness on clinical modeling and standardization we conducted a workshop for Danish and a few Norwegian e-health stakeholders' and made them discuss their views on different aspects of clinical modeling using a theoretical model as a point of departure. Based on the model, we traced stakeholders' experiences. Our results showed there was a tendency that stakeholders were more familiar with e-health requirements than with design methods, clinical information models and clinical terminology as they are described in the scientific literature. The workshop made it possible for stakeholders to discuss their roles and expectations to each other.

  20. Congenital Aortic Stenosis and Aneurysms

    NARCIS (Netherlands)

    D. van der Linde (Denise)

    2013-01-01

    textabstractDue to improvements in pediatric cardio-thoracic surgery, anesthesia and diagnostics over the past decades, the number of adult patients with congenital heart disease (CHD) is growing. This causes an increasing demand in clinical practice for insight in long term outcome in both non-oper

  1. Congenital granular-cell myoblastoma.

    Science.gov (United States)

    Cussen, L J; MacMahon, R A

    1975-04-01

    The clinical and pathologic features of congenital granular-cell myoblastoma in five infant girls are reported. One lesion, treated expectantly, progressively decreased in size and after 3 yr and 9 mo could not be detected, while two lesions which were imcompletely excised did not recur. It is suggested that congenital granular-cell myoblastoma is caused by an intrauterine stimulus, and that this stimulus may possible be production of estrogen by the fetus. Congential granular-cell myoblastoma should be treated expectantly or by limited excision, and has an excellent prognosis. PMID:164527

  2. Analysis of Clinical Characteristics of Children with Congenital Esophageal Atresia%先天性食管闭锁患儿的临床特征分析

    Institute of Scientific and Technical Information of China (English)

    王睿; 李碧香; 周崇高

    2011-01-01

    [目的]观察先天性食管闭锁患儿的临床高危因素,手术治疗疗效以及临床并发症,为临床诊治提供依据.[方法]回顾性分析本院90例先天性食管闭锁患儿的临床资料,分析先天性食管闭锁分型高危因素,手术治疗疗效和并发症等因素.多因素采用logistic回归分析.[结果]本组患儿治愈率为88.9%(72/81),未愈率占11.1%.患儿年龄、住院天数与疗效无相关(P>0.05).Ⅲ型和Ⅴ型食管闭锁术后疗效比较差异有统计学意义(P<0.05).合并症的情况与预后无关(P>0.05).食管上下盲端距离越近,疗效越好,差异有统计学意义(P<0.05).患儿术后并发症与疗效有相关性(P<0.05).[结论]患儿败血症、出生体重、伴随畸形、术后并发症及食管上下盲端的距离都与先天性食管闭锁患儿的术后疗效有关.%[Objective]To observe the clinical high-risk factors, surgical efficacy and complications of children with congenital esophageal atresia. [Methods]The clinical data of 90 cases of congenital esophageal atre-sia were analyzed retrospectively. The multiple factors such as high-risk factors, surgical efficacy and complications were analyzed by using logistic regression analysis. [Results] The curative rate was 88. 9% (72/81) and non-curative rate was 11. 1%. Age and hospitalization day had no correlation with treatment efficacy( P > 0. 05). There was significant difference in the efficacy between the surgery of type Ⅲ esophageal atresia and type Ⅳ esophageal atresia( P <0. 05). The complications had no correlation with the prognosis( P >0. 05). The narrower the esophagus, the better the curative effect, and the differences had statistically significance( P <0. 05). Postoperative complications were obviously related with the curative effect (P < 0. 05). [Conclusion]Sepsis, birth weight, accompanied malformation, postoperative complications and the distance between up and down blind-end of esophagus are

  3. Experience of 2 dental clinics registered to ISO 9002.

    Science.gov (United States)

    Casas, Michael J; Kenny, David J; Johnston, Douglas H

    2003-04-01

    This paper describes the 3-year experience of managing 2 hospital-based dental clinics registered to ISO 9002:1994; it also examines the revision of previous quality management standards in 2 separate institutions to prepare for registration under the new ISO 9001:2000 standard. Daily equipment and process checks, combined with internal audits, were the backbone of the quality system at both locations. Corrective and protective actions had been underused, because of the partial duplication produced by 2 different institutionally mandated risk management and incident reporting systems. ISO 9002 registration provided both dental clinics with responsive quality systems, emphasizing patient satisfaction and providing measurable continuous quality improvement.

  4. [The congenital afibrinogenemia: case report].

    Science.gov (United States)

    Brahem, Imen; Charfeddine, Bassem; Chraiti, Haythem; Ben Abdallah, Jihene; Ben Othmen, Leila; Neffati, Souhir; Ali Smach, Mohamed; Ltaief, Affef; Ksourri, Monia; Dridi, Hedi; Limem, Khalifa

    2010-01-01

    The deficiency in factor I or fibrinogen is a largely unknown genetic disease. It is a rare condition inherited as an autosomal recessive, whose clinical events are variable, ranging from moderate to minimal bleeding or cataclysmic hemorrhage. We report a case of congenital afibrinogenemia in a 17 years-old patient hospitalized in surgical ICU for hemoperitoneum medium abundance discovered by abdominal ultrasound performed before a picture of abdominopelvic pain lasting for 24 hours. Exploration led to the diagnosis of congenital afibrinogenemia with favorable evolution with a contribution of factor deficient. Through this case we raise the problem of congenital afibrinogenemia in diagnosis and the peculiarities of its management. PMID:20870582

  5. Clinical Analysis of 19 young children with congenital imperforated anus operated with Perine-um-anoplasty%会阴肛门成形术治疗先天性无肛19例临床分析

    Institute of Scientific and Technical Information of China (English)

    黄德铨; 侯艳梅; 许璟; 康健; 琚晓; 廖波; 袁可

    2011-01-01

    目的 分析和评估会阴肛门成形术治疗先天性中低位无肛患儿的疗效.方法 回顾性分析2003年10月至2010年8月我科收治的19例中低位无肛畸形儿采用会阴肛门成形术治疗的临床资料.结果 术后随访3个月至3年,19例患儿肛门功能及排便功能正常.结论 会阴肛门成形术治疗中低位无肛疗效是确切的,值得临床推广.%Objective To analyze and evaluate the efficacy of treating intermediate and low congenital imperforated anus with Perineum-anoplasty. Methods From Oct. 2003 to Aug. 2010, 19 young children with intermediate and low congenital imperforated anus were operated with Perineum-anoplasty. Results The cases had been followed up for 3 to 36 months. Anal function and defecation were normal with satisfactory.Conclusion The efficacy of treating congenital intermediate and low congenital imperforated anus with Perineum-anoplasty is accurate, and it is worth promoting in the clinic.

  6. Nursing preceptors' experiences of two clinical education models.

    Science.gov (United States)

    Mamhidir, Anna-Greta; Kristofferzon, Marja-Leena; Hellström-Hyson, Eva; Persson, Elisabeth; Mårtensson, Gunilla

    2014-08-01

    Preceptors play an important role in the process of developing students' knowledge and skills. There is an ongoing search for the best learning and teaching models in clinical education. Little is known about preceptors' perspectives on different models. The aim of the study was to describe nursing preceptors' experiences of two clinical models of clinical education: peer learning and traditional supervision. A descriptive design and qualitative approach was used. Eighteen preceptors from surgical and medical departments at two hospitals were interviewed, ten representing peer learning (student work in pairs) and eight traditional supervision (one student follows a nurse during a shift). The findings showed that preceptors using peer learning created room for students to assume responsibility for their own learning, challenged students' knowledge by refraining from stepping in and encouraged critical thinking. Using traditional supervision, the preceptors' individual ambitions influenced the preceptorship and their own knowledge was empathized as being important to impart. They demonstrated, observed and gradually relinquished responsibility to the students. The choice of clinical education model is important. Peer learning seemed to create learning environments that integrate clinical and academic skills. Investigation of pedagogical models in clinical education should be of major concern to managers and preceptors. PMID:24512652

  7. 小儿先天性重度肾积水的外科治疗分析%Clinical efficacy of surgical treatment of severe congenital hydronephrosis

    Institute of Scientific and Technical Information of China (English)

    董武

    2014-01-01

    目的:探讨外科治疗小儿先天性重度肾积水的临床疗效。方法回顾性分析36例小儿先天性重度肾积水患者的临床资料,分析手术后患肾形态和功能及临床疗效。结果随访时患儿患侧肾实质平均厚度以及肾实质平均面积较术前显著增加(P0.05)。对12例患儿术前术后肾脏功能进行比较,随访时健侧患侧肾滤过分数均显著增加(P0.05). Preoperative and postoperative renal function were compared on 12 patients that the results showed ipsilateral kidney filtration fraction significantly increased during follow-up (P<0.01), but still significantly lower than the contralateral kidney(P<0.01). During follow-up,the average of renal parenchyma surface without urinary tract infection was signif-icantly higher than that in children with urinary tract infection group(P<0.01). Conclusion Surgical treatment of chil-dren with severe congenital hydronephrosis shows better clinical efficacy.

  8. Congenital Heart Disease in Pregnancies Complicated by Maternal Diabetes Mellitus : An International Clinical Collaboration, Literature Review, and Meta-Analysis Angeborene Herzfehlbildungen bei mütterlichem Typ-1-Diabetes in der Schwangerschaft. Internationale klinische Multicenterstudie, Literaturübersicht und Metaanalyse

    NARCIS (Netherlands)

    L.A. Lisowski; P.M. Verheijen; J.A. Copel; C.S. Kleinman; S. Wassink; G.H.A. Visser; E.J. Meijboom

    2010-01-01

    PURPOSE: : Investigation of the incidence and distribution of congenital structural cardiac malformations among the offspring of mothers with diabetes type 1 and of the influence of periconceptional glycemic control. METHODS: : Multicenter retrospective clinical study, literature review, and meta-an

  9. Clinical Experience of Medical Students at University Sains Malaysia

    OpenAIRE

    Quah, Ban Seng; Malik, Alam Sher; Simpson, Hamish

    2000-01-01

    Experience of acute medical, surgical conditions, and clinical procedures of undergraduate students were assessed via a questionnaire survey during the final week of the 1993/1998 programme at the School of Medical Sciences, Univestiti Sains Malaysia. Individual performances were assessed by a scoring system. One hundred and twenty four students responded, (response rate 97%). More than 90% had seen myocardial infarction, cerebrovascular accident, pneumonia, respiratory distress, gastroenteri...

  10. Influence of light source and clinical experience on shade matching

    OpenAIRE

    GÁSPÁRIK, CRISTINA; TOFAN, ALINA; CULIC, BOGDAN; BADEA, MÎNDRA; DUDEA, DIANA

    2014-01-01

    Introduction Shade is one of the most important attributes when evaluating the success of a restoration. Several factors can influence the visual shade matching process, and therefore the outcome of the final restoration. Objectives 1. to assess the importance of clinical experience on shade matching accuracy; 2. to assess the influence of two standard light sources on the ability to match shade tabs; 3. to identify the area in a VITA Classical shade guide where matching errors are most likel...

  11. DNA-abzymes in autoimmune diseases in clinic and experiment

    Directory of Open Access Journals (Sweden)

    T E Naumova

    2003-01-01

    Full Text Available DNA-abzymes enzymes in autoimmune diseases in clinic and experiment T.E. Naumova, O.M. Durova, A.G. Gabibov, Z.S. Alekberova, S. V. Suchkov DNA-hydrolyzing autoantibodies (AAB or DNA-abzymes can be found in autoimmune diseases in clinic and experiment. Technology of serum express screening for presence of DNA abzymes is described. Comparative study of DNA-hydrolising activity in patients with different forms of systemic and organ-specific autoimmune diseases was performed. Blood of clinically healthy donors was usually free of IgG DNA-abzymes. DNA-abzymes were most often revealed in patients with systemic lupus erythematosus (SLE and rheumatoid arthritis (RA less often in patients with organ-specific forms of autoimmune disturbances. The results of the study confirm the hypothesis of autoimmune origin of IgG DNA abzymes and demonstrate the possibility to use them in clinical practice for monitoring to disease activity in SLE and RA.

  12. Clinical decision-making: physicians' preferences and experiences

    Directory of Open Access Journals (Sweden)

    White Martha

    2007-03-01

    Full Text Available Abstract Background Shared decision-making has been advocated; however there are relatively few studies on physician preferences for, and experiences of, different styles of clinical decision-making as most research has focused on patient preferences and experiences. The objectives of this study were to determine 1 physician preferences for different styles of clinical decision-making; 2 styles of clinical decision-making physicians perceive themselves as practicing; and 3 the congruence between preferred and perceived style. In addition we sought to determine physician perceptions of the availability of time in visits, and their role in encouraging patients to look for health information. Methods Cross-sectional survey of a nationally representative sample of U.S. physicians. Results 1,050 (53% response rate physicians responded to the survey. Of these, 780 (75% preferred to share decision-making with their patients, 142 (14% preferred paternalism, and 118 (11% preferred consumerism. 87% of physicians perceived themselves as practicing their preferred style. Physicians who preferred their patients to play an active role in decision-making were more likely to report encouraging patients to look for information, and to report having enough time in visits. Conclusion Physicians tend to perceive themselves as practicing their preferred role in clinical decision-making. The direction of the association cannot be inferred from these data; however, we suggest that interventions aimed at promoting shared decision-making need to target physicians as well as patients.

  13. Genetics Home Reference: congenital hypothyroidism

    Science.gov (United States)

    ... Help Me Understand Genetics Home Health Conditions congenital hypothyroidism congenital hypothyroidism Enable Javascript to view the expand/collapse ... Genetic Testing Registry: Congenital hypothyroidism Genetic Testing Registry: Hypothyroidism, ... Encyclopedia: Congenital Hypothyroidism These resources ...

  14. Genetic and Epigenetic Factors at COL2A1 and ABCA4 Influence Clinical Outcome in Congenital Toxoplasmosis

    OpenAIRE

    JAMIESON, S.E.; de Roubaix, L. A.; Cortina-Borja, M.; Tan, H K; Mui, E. J.; Cordell, H J; Kirisits, M. J.; Miller, E. N.; Peacock, C. S.; Hargrave, A. C.; Coyne, J J; Boyer, K.; Bessieres, M H; Buffolano, W.; Ferret, N

    2008-01-01

    Background: Primary Toxoplasma gondii infection during pregnancy can be transmitted to the fetus. At birth, infected infants may have intracranial calcification, hydrocephalus, and retinochoroiditis, and new ocular lesions can occur at any age after birth. Not all children who acquire infection in utero develop these clinical signs of disease. Whilst severity of disease is influenced by trimester in which infection is acquired by the mother, other factors including genetic predisposition may ...

  15. Low-dose prospective ECG-triggering dual-source CT angiography in infants and children with complex congenital heart disease: first experience

    Energy Technology Data Exchange (ETDEWEB)

    Cheng, Zhaoping; Wang, Ximing; Duan, Yanhua; Wu, Lebin; Wu, Dawei; Chao, Baoting; Liu, Cheng; Xu, Zhuodong [Shandong University, Shandong Medical Imaging Research Institute, Jinan, Shandong (China); Li, Hongxin; Liang, Fei [Shandong Provincial Hospital, Department of Cardiovascular Surgery, Jinan, Shandong (China); Xu, Jian; Chen, Jiuhong [Siemens. Ltd. China, CT Research Collaboration, Beijing (China)

    2010-10-15

    To explore the clinical value of low-dose prospective ECG-triggering dual-source CT (DSCT) angiography in infants and children with complex congenital heart disease (CHD) compared with transthoracic echocardiography (TTE). Thirty-five patients (mean age: 16 months, range: 2 months to 6 years; male 15; mean weight: 12 kg) underwent low-dose prospective ECG-triggering DSCT angiography and TTE. Surgeries were performed in 29 patients, and conventional cardiac angiography (CCA) was performed in 8 patients. The accuracy was calculated based on the surgical and/or CCA findings. The overall imaging quality was evaluated on a five-point scale. A total of 146 separate cardiovascular deformities were confirmed. DSCT missed three atrial septal defects and a patent ductus arteriosus. The accuracy of DSCT angiography and TTE was 97.3% (142/146) and 92.5% (135/146), respectively. Overall test parameters for DSCT angiography and TTE were similar (sensitivity, 97.3% vs 92.5%; specificity, 99.8% vs 99.8%). The average subjective image quality score was 4.3 {+-} 0.7. The mean effective dose was 0.38 {+-} 0.09 mSv. Prospective ECG-triggering DSCT angiography with a very low effective radiation dose allows the accurate diagnosis of anomalies in infants and children with complex CHD compared with TTE. It has great promise to become a commonly used second-line technique for complex CHD. (orig.)

  16. Comprehensive Clinical and Molecular Assessment of 32 Probands With Congenital Contractural Arachnodactyly : Report of 14 Novel Mutations and Review of the Literature

    NARCIS (Netherlands)

    Callewaert, Bert L.; Loeys, Bart L.; Ficcadenti, Anna; Vermeer, Sascha; Landgren, Magnus; Kroes, Hester Y.; Yaron, Yuval; Pope, Michael; Foulds, Nicola; Boute, Odile; Galan, Francisco; Kingston, Helen; Van der Aa, Nathalie; Salcedo, Iratxe; Swinkels, Marielle E.; Wallgren-Pettersson, Carina; Gabrielli, Orazio; De Backer, Julie; Coucke, Paul J.; De Paepe, Anne M.

    2009-01-01

    Beals-Hecht syndrome or congenital contractural arachnodactyly (CCA) is a rare, autosomal dominant connective tissue disorder characterized by crumpled ears, arachnodactyly, contractures, and scoliosis. Recent reports also mention aortic root dilatation, a finding previously thought to differentiate

  17. Comprehensive clinical and molecular assessment of 32 probands with congenital contractural arachnodactyly: report of 14 novel mutations and review of the literature.

    NARCIS (Netherlands)

    Callewaert, B.L.; Loeys, B.L.; Ficcadenti, A.; Vermeer, S.; Landgren, M.; Kroes, H.Y.; Yaron, Y.; Pope, M.; Foulds, N.; Boute, O.; Galan, F.; Kingston, H.; Aa, N. van der; Salcedo, I.; Swinkels, M.E.; Wallgren-Pettersson, C.; Gabrielli, O.; Backer, J. de; Coucke, P.J.; Paepe, A.M. De

    2009-01-01

    Beals-Hecht syndrome or congenital contractural arachnodactyly (CCA) is a rare, autosomal dominant connective tissue disorder characterized by crumpled ears, arachnodactyly, contractures, and scoliosis. Recent reports also mention aortic root dilatation, a finding previously thought to differentiate

  18. 婴幼儿先天性心脏病术后高热的护理体会%Nursing experience of infants with fever after congenital heart disease surgery

    Institute of Scientific and Technical Information of China (English)

    孙佳

    2015-01-01

    本文探讨了婴幼儿先天性心脏病(简称:先心病)术后高热的护理对策及临床效果.对100例婴幼儿先天性心脏病术后高热患者,通过一系列的物理和药物的方法,所有患者都得到了有效的降温,临床效果较满意.因此,对婴幼儿先天性心脏病术后高热给予一系列的护理对策具有较好的临床效果.%In this paper,the author explored the nursing countermeasure and clinical effect of infants with fever after congenital heart disease surgery.100 infants with fever after congenital heart disease surgery were given a series of physical and drug methods,and all patients had been effectively cooled.The clinical effect was satisfactory.Thus,a series of nursing countermeasures for infants with fever after congenital heart disease surgery have a better clinical effect.

  19. 小儿先天性心脏病封堵术后的护理体会%Nursing Experience of Children with Congenital Heart Disease after Transcatheter Closure of Congenital Heart Disease

    Institute of Scientific and Technical Information of China (English)

    曾珍; 郭文馨

    2015-01-01

    目的:探讨小儿先天性心脏病封堵术后护理要点。方法回顾性分析我科自2014年1月~2015年5月共50例小儿先天性心脏病封堵术的案例。结果手术成功,术后恢复快,无严重并发症。结论患者年龄小,体重低,护理难度大,规范有效的护理能有效预防并发症的发生,促进术后恢复。%Objective To explore the nursing points of the patients with congenital heart disease in children. Methods A retrospective analysis of 50 cases of congenital heart disease in children from January 2014 to May 2015, a total of cases of congenital heart disease. Results The operation was successful, and the recovery was fast and no serious complication. Conclusion Patients with low age, low weight, dif iculty of nursing, standardized and ef ective nursing can ef ectively prevent the occur ence of complications, promote postoperative recovery.

  20. Adult congenital heart disease

    OpenAIRE

    Morphet, John AM

    2006-01-01

    One million people over the age of 20 suffer from congenital heart disease in the United States. These adult patients can slip through the cracks of our medical system; many are too old to be cared for in most pediatric institutions by pediatric cardiologists and, unfortunately, most adult cardiologists are not trained in congenital heart disease. Therefore, it is important to identify the common lesions in adult congenital heart disease and how they should be managed. Acyanotic congenital he...

  1. Feasibility of a Team Approach to Complex Congenital Heart Defect Neurodevelopmental Follow-Up: Early Experience of a Combined Cardiology/Neonatal Intensive Care Unit Follow-Up Program.

    Science.gov (United States)

    Chorna, Olena; Baldwin, H Scott; Neumaier, Jamie; Gogliotti, Shirley; Powers, Deborah; Mouvery, Amanda; Bichell, David; Maitre, Nathalie L

    2016-07-01

    Infants with complex congenital heart disease are at high risk for poor neurodevelopmental outcomes. However, implementation of dedicated congenital heart disease follow-up programs presents important infrastructure, personnel, and resource challenges. We present the development, implementation, and retrospective review of 1- and 2-year outcomes of a Complex Congenital Heart Defect Neurodevelopmental Follow-Up program. This program was a synergistic approach between the Pediatric Cardiology, Cardiothoracic Surgery, Pediatric Intensive Care, and Neonatal Intensive Care Unit Follow-Up teams to provide a feasible and responsible utilization of existing infrastructure and personnel, to develop and implement a program dedicated to children with congenital heart disease. Trained developmental testers administered the Ages and Stages Questionnaire-3 over the phone to the parents of all referred children at least once between 6 and 12 months' corrected age. At 18 months' corrected age, all children were scheduled in the Neonatal Intensive-Care Unit Follow-Up Clinic for a visit with standardized neurological exams, Bayley III, multidisciplinary therapy evaluations and continued follow-up. Of the 132 patients identified in the Cardiothoracic Surgery database and at discharge from the hospital, a total number of 106 infants were reviewed. A genetic syndrome was identified in 23.4% of the population. Neuroimaging abnormalities were identified in 21.7% of the cohort with 12.8% having visibly severe insults. As a result, 23 (26.7%) received first-time referrals for early intervention services, 16 (13.8%) received referrals for new services in addition to their existing ones. We concluded that utilization of existing resources in collaboration with established programs can ensure targeted neurodevelopmental follow-up for all children with complex congenital heart disease. PMID:27220370

  2. 3500例婴幼儿泪囊炎的疗效分析%The clinical effect of 3500 cases with congenital dacryocystitis

    Institute of Scientific and Technical Information of China (English)

    刘淑玲; 李瑞宏; 姜洋; 李箐; 李莹

    2011-01-01

    Objective To analyze che feature of the bacteria spectrum of congenital dacryocystitis; and to discuss the clinical effccacy of the three-stage mcthod to providd evidences for clinical practice. Design Retrospective case series. Participants 3500 cases with congenital dacryocystitis from Tangshan Matemity and Child Care Hospital. Methods The 3500 patients were treated with three-step treatment. The first step: pushing down on the lump; the second step: probing the lacrimal passage when it is useless only pushing the lump. The third step: comprehensive therapy. Bacterial culture of the discharge from the conjunctive sac were performed in 60 cases who were selected randomly. The clinical effecacy and the bacteria spectrum were observed. Main Outcome Measures The clinical effecacy and the bacteria sepctrum. Results In 3500 cases, 98.9% of patients were less than one-year old (3.7±0.7months).There were 8% of cases cured by only one step therapy. There were 90% of cases cured by two step therapy. And there were only 20% of cases cured by three-step therapy. All the patients were cured by three-step therapy with no more infection of lacrimal sac except for one case who was an older child suffered recurrent blockage of the lacrimal passage. The 60 bacterial cunures of discharges randomly getting from the dacryocystitis patients' conjunctive sac show that siaphylococcus aurous, Burt's bacteria produce acid clay, and pseudomonas aeruginosa were the most common pathogenic bacterium. Conclusion Dacryocystitis commonly attracts infants less than 4 months old, with one eye involved. Although it shows diversity of the bacterial culture, the most common pathogenic bacterium is staphylococcus aurous, Burt ' s bacteria produce acid clay, and pseudomonas aeruginosa. It is effective management of congenital dacryocystitis with the lacrimal passage probing combined with antihiotic eye drops.%目的 总结婴幼儿泪囊炎细菌学特征和临床治疗效果,为此类疾

  3. Developing a leadership pipeline: the Cleveland Clinic experience.

    Science.gov (United States)

    Hess, Caryl A; Barss, Christina; Stoller, James K

    2014-11-01

    The complexity of health care requires excellent leadership to address the challenges of access, quality, and cost of care. Because competencies to lead differ from clinical or research skills, there is a compelling need to develop leaders and create a talent pipeline, perhaps especially in physician-led organizations like Cleveland Clinic. In this context, we previously reported on a cohort-based physician leadership development course called Leading in Health Care and, in the current report, detail an expanded health care leadership development programme called the Cleveland Clinic Academy (CCA). CCA consists of a broad suite of offerings, including cohort-based learning and 'a la carte' half- or full-day courses addressing specific competencies to manage and to lead. Academy attendance is optional and is available to all physicians, nurses, and administrators with the requisite experience. Course selection is guided by competency matrices which map leadership competencies to specific courses. As of December 2012, a total of 285 course sessions have been offered to 6,050 attendees with uniformly high ratings of course quality and impact. During the past 10 years, Cleveland Clinic's leadership and management curriculum has successfully created a pipeline of health care leaders to fill executive positions, search committees, board openings, and various other organizational leadership positions. Health care leadership can be taught and learned. PMID:25082312

  4. Congenital peritoneal encapsulation

    Institute of Scientific and Technical Information of China (English)

    Diana; Teixeira; Vítor; Costa; Paula; Costa; Carlos; Alpoim; Pinto; Correia

    2015-01-01

    Peritoneal encapsulation(PE) is a rare congenital malformation, characterized by a thin accessory peritoneal membrane which covers all or part of the small bowel, forming an accessory peritoneal sac. Most cases areasymptomatic and diagnosed incidentally during surgery and/or autopsy. Clinical presentation with intestinal obstruction is extremely rare and we report a case. A 25-year-old male, referred to emergency department with diffuse abdominal pain, crampy, with 8 h evolution, associated with nausea, vomiting and constipation in the last 48 h. The abdominal examination revealed an asymmetric and fixed distension, with hard consistency on palpation of lower abdominal quadrants. The abdominal radiography reveals a small bowel distension and fluid levels. Submitted to laparoscopic surgery that recourse to conversion because there is a total peritoneal encapsulation of the small bowel. After opening the peritoneal sac, we find a rotation of mesentery, at its root, conditioning twisting of small bowel and consequently occlusion. Uneventful postoperative with discharged at the 6th day. The PE is a very rare congenital anomaly characterized by abnormal bowel back into the abdominal cavity in the early stages of development. Your knowledge becomes important because, although rare, it might be diagnosis in patients with intestinal obstruction, in the absence of other etiologic factors.

  5. Congenital fiber type disproportion.

    Science.gov (United States)

    Kissiedu, Juliana; Prayson, Richard A

    2016-04-01

    Type I muscle fiber atrophy in childhood can be encountered in a variety of neuromuscular disorders. Congenital fiber type disproportion (CFTD) is one such condition which presents as a nonprogressive muscle weakness. The diagnosis is often made after excluding other differential diagnostic considerations. We present a 2-year-9-month-old full term boy who presented at 2 months with an inability to turn his head to the right. Over the next couple of years, he showed signs of muscle weakness, broad based gait and a positive Gower's sign. He had normal levels of creatine kinase and normal electromyography. A biopsy of the vastus lateralis showed a marked variation in muscle fiber type. The adenosine triphosphate (ATP)-ase stains highlighted a marked type I muscle atrophy with rare scattered atrophic type II muscle fibers. No abnormalities were observed on the nicotinamide adenine dinucleotide (NADH), succinate dehydrogenase (SDH) or cytochrome oxidase stained sections. Ragged red fibers were not present on the trichrome stain. Abnormalities of glycogen or lipid deposition were not observed on the periodic acid-Schiff or Oil-Red-O stains. Immunostaining for muscular dystrophy associated proteins showed normal staining. Ultrastructural examination showed a normal arrangement of myofilaments, and a normal number and morphology for mitochondria. A diagnosis of CFTD was made after excluding other causes of type I atrophy including congenital myopathy. The lack of specific clinical and genetic disorder associated with CFTD suggests that it is a spectrum of a disease process and represents a diagnosis of exclusion. PMID:26526626

  6. Congenital extrahepatic portosystemic shunts

    Energy Technology Data Exchange (ETDEWEB)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S. [Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, M5G 1X8, Toronto, Ontario (Canada)

    2003-09-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  7. Clinical application of the"hybrid approach"in the treatment of congenital heart disease%“镶嵌”技术在先天性心脏病临床治疗中的应用

    Institute of Scientific and Technical Information of China (English)

    姜明泽(综述); 游昕(审校)

    2014-01-01

    With growing number of operation with congenital heart disease ( CHD) ,its methods of treatment are paid more and more attention by the surgeons .The article briefly introduced the concept ,development history ,the main clinical applications ,complica-tions,treatment, comprehensive evaluation and prospect of "hybrid approach".%随着先天性心脏病( congenital heart disease ,CHD)手术数量的增加,其治疗方法的改进也日益受到重视。文中简要介绍CHD“镶嵌”技术( hybrid approach )的概念、发展历史、主要临床应用、并发症及处理等内容,并对这一技术进行综合评价及展望。

  8. Isolated congenital heart block in undifferentiated connective tissue disease and in primary Sjögren’s syndrome: a clinical study of 81 pregnancies in 41 patients

    Directory of Open Access Journals (Sweden)

    S. Todesco

    2011-09-01

    Full Text Available Objective: To study the incidence and the features of congenital heart block (CHB in patients with undifferentiated connective tissue disease (UCTD and primary Sjögren’s syndrome (pSS. Methods: We studied 81 pregnancies of 41 women attending the Outpatients’ Clinic of the Rheumatology Unit of University Hospital of Padova from July 1989 to March 2004. Twenty five of these (61% were affected with UCTD and 16 (39% with pSS. Serologic inclusion criteria was anti-Ro/La positivity, assessed by counterimmunoelectrophoresis and ELISA. Results: CHB was found in 2 out of the 46 (4,3% pregnancies followed by our Staff and in 2 out of the 35 (5,7% included in the retrospective part of the study. In 3 cases CHB was a 3rd degree block, causing pregnancy termination in 2. The only 2nd degree block was identified in one patient at the 22nd week of gestation and treated with dexamethasone and plasma-exchange. All of the women were positive to 52 kd and 60 kd Ro autoantibodies. CHB mothers had higher titer antibodies to 52 kd Ro protein than did the mothers with healthy infants (P = 0,026. Electrocardiographic abnormalities at birth were found in 3 out of 29 asymptomatic infants. One presented sinus bradycardia, the second abnormalities of ventricular repolarization, both regressed spontaneously, while the third ventricular extrasystoles which continue even now at 5 months. Conclusion: These results showed that in UCTD and pSS there is a higher incidence of CHB than that reported in Systemic Lupus Erythematosus. Electrocardiographic screening in all infants born to mothers with anti-Ro/La antibodies would seem an important measure to identify those with irreversible heart conduction abnormalities.

  9. Solitary ulcerated congenital giant juvenile xanthogranuloma

    Directory of Open Access Journals (Sweden)

    Su Yuen Ng

    2015-01-01

    Full Text Available A 3-month-old female patient with a giant ulcerated nodule over the back since birth was diagnosed as congenital giant juvenile xanthogranuloma (JXG based on clinical and histopathological examination. Congenital giant JXG with ulceration at birth is a rare presentation of JXG and commonly misdiagnosed. This case emphasizes the importance of being aware of the myriad presentations of JXG in order to make a correct diagnosis and avoid unnecessary investigations or treatment.

  10. Running a postmortem service - a business case and clinical experience

    International Nuclear Information System (INIS)

    The purpose of the postmortem examination is to offer answers to explain the cause and manner of death. In the case of perinatal, infant and paediatric postmortem examinations, the goal is to identify unsuspected associated features, to describe pathogenic mechanisms and new conditions, and to evaluate the clinical management and diagnosis. Additionally, the postmortem examination is useful to counsel families regarding the probability of recurrence in future pregnancies and to inform family planning. Worldwide the rate of paediatric autopsy examinations has significantly declined during the last few decades. Religious objections to postmortem dissection and organ retention scandals in the United Kingdom provided some of the impetus for a search for non-invasive alternatives to the traditional autopsy; however, until recently, imaging studies remained an adjunct to, rather than a replacement for, the traditional autopsy. In 2012, Sheffield Children's Hospital National Health Service Foundation Trust set up the service provision of minimally invasive fetal, perinatal and neonatal autopsy, while a postmortem imaging service has been running in Melbourne, Australia, since 2008. Here we summarise the essentials of a business case and practical British and Australian experiences in terms of the pathological and radiologic aspects of setting up a minimally invasive clinical service in the United Kingdom and of developing a clinical postmortem imaging service as a complementary tool to the traditional autopsy in Australia. (orig.)

  11. Running a postmortem service - a business case and clinical experience

    Energy Technology Data Exchange (ETDEWEB)

    Cohen, Marta C.; Whitby, Elspeth; Fink, Michelle A.; Collett, Jacquelene M.; Offiah, Amaka C. [Western Bank, Academic Unit of Child Health, Sheffield Children' s NHS Foundation Trust, University of Sheffield, Sheffield (United Kingdom)

    2015-04-01

    The purpose of the postmortem examination is to offer answers to explain the cause and manner of death. In the case of perinatal, infant and paediatric postmortem examinations, the goal is to identify unsuspected associated features, to describe pathogenic mechanisms and new conditions, and to evaluate the clinical management and diagnosis. Additionally, the postmortem examination is useful to counsel families regarding the probability of recurrence in future pregnancies and to inform family planning. Worldwide the rate of paediatric autopsy examinations has significantly declined during the last few decades. Religious objections to postmortem dissection and organ retention scandals in the United Kingdom provided some of the impetus for a search for non-invasive alternatives to the traditional autopsy; however, until recently, imaging studies remained an adjunct to, rather than a replacement for, the traditional autopsy. In 2012, Sheffield Children's Hospital National Health Service Foundation Trust set up the service provision of minimally invasive fetal, perinatal and neonatal autopsy, while a postmortem imaging service has been running in Melbourne, Australia, since 2008. Here we summarise the essentials of a business case and practical British and Australian experiences in terms of the pathological and radiologic aspects of setting up a minimally invasive clinical service in the United Kingdom and of developing a clinical postmortem imaging service as a complementary tool to the traditional autopsy in Australia. (orig.)

  12. Congenital TORCH infections of the brain--CT manifestation (with analysis of 7 cases)

    International Nuclear Information System (INIS)

    To study the neuropathologic changes and CT manifestations in congenital TORCH infection of the brain. Analysis of 7 cases of congenital TORCH infection of the neonates and infants demonstrated by serum examination was performed. There were congenital toxoplasmosis 3 cases, congenital syncytial virus infection 1 case, congenital rubella virus infection 1 case, congenital cytomegalovirus infection 2 cases, and congenital herpes simplex virus infection 1 case. Cerebral hypoplasia, ventricular dilatation or hydrocephalus, subependymal and parenchymal calcifications, microcephalic focal cortical migration anomalies, schizencephaly polymicrogyria, et al, were demonstrated by CT with congenital TORCH infection. The earlier the infection, the more severe the brain developmental anomalies. The extent and appearance of calcification in brain were related to the degree, extent and course of TORCH infection. Basal ganglia calcification of unknown cause in infant was suggestive of congenital TORCH infection. Typical CT manifestations together with clinical picture may suggest congenital TORCH infection, while serological test can be diagnostic

  13. 43例新生儿先天性感染TORCH的临床分析%Clinical analysis of 43 congenital TORCH infections in newborns

    Institute of Scientific and Technical Information of China (English)

    孙西锋; 孙肖伟; 常立军; 于亮

    2010-01-01

    目的 对先天性感染TORCH的新生儿进行临床分析.方法 用ELASA法检测血清中TORCH(弓形体、风疹病毒、巨细胞病毒、单纯疱疹病毒)IgM.结果 2004年1月至2008年6月本院新生儿重症监护室共收治1352例,其中43例为TORCH感染,巨细胞病毒感染率为48.8%;风疹病毒感染占32.6%;单纯疱疹病毒感染占18.6%,未发现有弓形体感染者.其临床特点主要为听力异常、高胆红素血症和肝功能异常、神经系统损伤、心肌损伤、血小板减少、先天性心脏病等.结论 新生儿TORCH感染可造成多器官损伤,危害性大,对可疑患儿进行筛查是必要的,预防应从母亲怀孕期开始%Objective To search for the early clinical manifestations as evidences for prevention and treatment TORCH infections in newborns as early as possible. Methods ELASA was performed to screen specific anti-TORCH ( Toxoplasma gondlii, Cystomegalovirus, Rubella virus, Herpes simplex virus ) Ig-M antibodies.RESULTS Totally 1352 in-patients who were treated in Neonatal Intemaive Care Unit(NICU) of our hospital from January 2007 to June 2008 were retrospectively studied,43 of them had TORCH infections. Cytomegalovirus, rubella and herpes simples virus infections accounted for 48.8% ,32.6% and 18.6%, respectively. None of them had toxoplasma infection. The main clinical manifestations of them were hearing impairment, hyperbilirubinemias and liver dysfuntion,impairment of neurologic system, myocardial impairment, thrombocytopenia, and congenital heart disease. Conclusion TORCH infections can cause multiorgan lesions. Rubella vaccine inoculation, serological screening during pregnancy and early period of newborn, intervention and treatment in the early period are most important.

  14. A new extra-abdominal channel alternative to the mitrofanoff principle: experimental and preliminary clinical experience

    Directory of Open Access Journals (Sweden)

    Antonio Macedo Jr.

    2009-04-01

    Full Text Available INTRODUCTION: The appendix is the gold-standard channel for the Mitrofanoff principle in pediatric urology, but the search for alternatives is justified considering it may not be available or preferably used for colonic stomas (Malone antegrade continence enema. The aim of this study is to report on technical feasibility of a new approach for creating catheterizable channels in a rabbit model and to present our preliminary clinical experience. MATERIAL AND METHODS: We configured a tube from two rectangular skin flaps 1x4 cm opposite each other in the middle line of the lower inferior abdomen. The channel was anastomosed to the bladder dome with embedding sutures to create a valvular mechanism. The experimental study consisted of 12 rabbits, divided in 4 groups according to the sacrifice schedule at 2, 4, 8 and 12 weeks. At 30th postoperative day, an urodynamic evaluation was performed to record continence of the stoma. A histological analysis of the specimens stained with hematoxylin-eosin, Masson trichrome and Picrosirius red was also done in group 2 (sacrifice at 4 weeks postoperatively. We used this method in 3 patients with congenital non-neurogenic bladder disease presenting with massive residual volumes without compliance deficits. RESULT: The technique proved feasible in all animals, 9 of 12 could be easily catheterized and underwent urodynamic study. No stoma leakage was observed in 7 animals at high bladder pressures (> 50 cm H20 and only 2 animals had some leakage at 40 cm H20. Urodynamics performed through the stoma showed urethral leakage at 20 cm H20, therefore demonstrating the efficacy of the valvular mechanism. Histological analysis confirmed good integration between the tube and the bladder. Mean follow-up of the clinical series (3 patients was 7.2 months. Two patients remained continent up to 4 hours, whereas 1 patient had some leakage after 2 hours. CONCLUSION: We were able to confirm feasibility of a new extra

  15. Clinicians’ experiences of becoming a clinical manager: a qualitative study

    Directory of Open Access Journals (Sweden)

    Spehar Ivan

    2012-11-01

    Full Text Available Abstract Background There has been an increased interest in recruiting health professionals with a clinical background to management positions in health care. We know little about the factors that influence individuals’ decisions to engage in management. The aim of this study is to explore clinicians’ journeys towards management positions in hospitals, in order to identify potential drivers and barriers to management recruitment and development. Methods We did a qualitative study which included in-depth interviews with 30 clinicians in middle and first-line management positions in Norwegian hospitals. In addition, participant observation was conducted with 20 of the participants. The informants were recruited from medical and surgical departments, and most had professional backgrounds as medical doctors or nurses. Interviews were analyzed by systemic text condensation. Results We found that there were three phases in clinicians’ journey into management; the development of leadership awareness, taking on the manager role and the experience of entering management. Participants’ experiences suggest that there are different journeys into management, in which both external and internal pressure emerged as a recurrent theme. They had not anticipated a career in clinical management, and experienced that they had been persuaded to take the position. Being thrown into the position, without being sufficiently prepared for the task, was a common experience among participants. Being left to themselves, they had to learn management “on the fly”. Some were frustrated in their role due to increasing administrative workloads, without being able to delegate work effectively. Conclusions Path dependency and social pressure seems to influence clinicians’ decisions to enter into management positions. Hospital organizations should formalize pathways into management, in order to identify, attract, and retain the most qualified talents. Top managers

  16. Congenital contractural arachnodactyly (Beals syndrome).

    OpenAIRE

    Viljoen, D

    1994-01-01

    Congenital contractural arachnodactyly (CCA) is an autosomal dominant disorder akin to, but usually less severe than, Marfan syndrome. The clinical features are marfanoid habitus, arachnodactyly, crumpled ears, camptodactyly of the fingers and adducted thumbs, mild contractures of the elbows, knees, and hips, and mild muscle hypoplasia especially of the calf muscles. Many patients have kyphoscoliosis and mitral valve prolapse and, very occasionally, aortic root dilatation and ectopia lentis h...

  17. Clinical application of imaging diagnosis in complicated congenital heart disease%复杂性先天性心脏病影像学诊断的临床应用

    Institute of Scientific and Technical Information of China (English)

    罗凯(综述); 付炜; 郑景浩(审校)

    2014-01-01

    With the continuous development of imaging techniques,patients with congenital heart dis-ease,especially in complicated congenital cardiac malformations,have more choices for detection methods of ear-ly diagnosis. Nowadays,the widely used diagnostic imaging methods included echocardiography,CT,MRI,cardi-ac catheterization,and prenatal ultrasound examination. Clinicians should choose the appropriate means of ima-ging test depending on the situation. This article will focus on the clinical application of imaging diagnosis in complicateol congenital heart disease.%随着影像学技术的不断发展,先天性心脏病,尤其是复杂性先天心脏畸形的早期诊断方法有了更多选择。目前广泛用于先天性心脏病心影像学诊断的检查主要包括超声心动图、CT、MRI、心导管检查以及产前超声检查等。临床医生应该根据患者不同情况选择合适的检查手段,该文将对近年来几种影像检查在临床上如何选择应用做一综述。

  18. General Practitioners’ responses to global climate change - lessons from clinical experience and the clinical method

    Directory of Open Access Journals (Sweden)

    Blashki Grant

    2012-08-01

    Full Text Available Abstract Background Climate change is a global public health problem that will require complex thinking if meaningful and effective solutions are to be achieved. In this conceptual paper we argue that GPs have much to bring to the issue of climate change from their wide-ranging clinical experience and from the principles underpinning their clinical methods. This experience and thinking calls forth particular contributions GPs can and should make to debate and action. Discussion We contend that the privileged experience and GP way of thinking can make valuable contributions when applied to climate change solutions. These include a lifetime of experience, reflection and epistemological application to first doing no harm, managing uncertainty, the ability to make necessary decisions while possessing incomplete information, an appreciation of complex adaptive systems, maintenance of homeostasis, vigilance for unintended consequences, and an appreciation of the importance of transdisciplinarity and interprofessionalism. Summary General practitioners have a long history of public health advocacy and in the case of climate change may bring a way of approaching complex human problems that could be applied to the dilemmas of climate change.

  19. Psychometric validation of satisfaction with simulated clinical learning experience evaluation – corrections (SSCLEE-C

    Directory of Open Access Journals (Sweden)

    Desiree A. Díaz

    2016-03-01

    Originality/value: The Satisfaction with Simulated Clinical Learning Experience Evaluation – Corrections (SSCLEE-C is the only instrument available for ongoing assessment of correctional nurse satisfaction with simulated clinical learning experiences.

  20. Diagnosis and treatment of congenital heart defects

    International Nuclear Information System (INIS)

    The Clinic for Congenital Heart Defects in Bad Oeynhausen, Germany, specializes in the interdisciplinary care of children and adolescents with congenital heart defects. The available expertise covers all aspects of diagnosis and treatment, as well as clinical research at the highest level. The clinic has a complete array of imaging systems, including a Philips Integris BV5000 Biplane cath lab system, Philips Gyroscan Intera CV MR system, ultrasound and PET systems. The range of available equipment makes it possible to select the most appropriate imaging modality for each situation, with particular reference to the need to minimize the overall radiation exposure. (orig.)

  1. Mental retardation, congenital heart defect, cleft palate, short stature, and facial anomalies: A new X-linked multiple congenital anomalies/mental retardation syndrome: Clinical description and molecular studies

    Energy Technology Data Exchange (ETDEWEB)

    Hamel, B.C.J.; Mariman, E.C.M.; Beersum, S.E.C. van; Ropers, H.H. [University Hospital, Nijmegen (Netherlands); Schoonbrood-Lenssen, A.M.J. [Institute for the Mentally Retarded, Echt (Netherlands)

    1994-07-15

    We report on two brothers and their two maternal uncles with severe mental retardation, congenital heart defect, cleft or highly arched palate, short stature and craniofacial anomalies consisting of microcephaly, abnormal ears, bulbous nose, broad nasal bridge, malar hypoplasia, and micro-gnathia. Three of the four patients died at an early age. The mother of the two brothers had an atrial septal defect. She is assumed to be manifesting carrier of a mutant gene, which is expressed in her two sons and two brothers. By multipoint linkage analysis it is found that the most likely location of the responsible gene is the pericentromeric region Xp21.3-q21.3 with DMD and DXS3 as flanking markers. Maximum information is obtained with marker DXS453 (Z = 1.20 at {theta} = 0.0). 24 refs., 12 figs., 1 tab.

  2. 新生儿先天性心脏病54例%Clinical Analysis on 54 Cases of Congenital Heart Disease in Newborn Infants

    Institute of Scientific and Technical Information of China (English)

    唐俊海; 熊高洁; 刘漫君; 夏春波; 郑楚

    2012-01-01

    Objective lo explore the clinical charactenstics ol the newborn congenital heart disease(CHD) in order to provide rele-rences for earlier diagnosis. Methods The pathologic type, clinical manifestations and auxiliary examinations of 54 newborn infants with CHD (31 cases were male and 23 cases were female;30 -41 weeks oldjthe weight was 1 200 -3 920 g) were analyzed,adopting color doppler ul-trasonography was used to detect the left ventricular long axis, aorta short axis, four - chamber heart ( parastemal, apical, xiphoid) , and if necessary, the aortic arch suprasternal fossa was performed confirm the presence of cardiovascular abnormalities. Results The non - cyanosis CHD accounted for 75.93% ,which had 8 pathological types,and most of them were atrial septal defect,patent ductus arteriosus and ventricular septal defect;the cyanosis CHD accounted for 24.07% ,which expressed in 7 pathological types,mainly in the form of aorta transition and tetralogy of fallot. Their common clinical manifestations were heart murmur, cyanosis and shortness of breath. Cyanosis CHD incidence rate was 62. 50% in premature infants,7. 89% in full - term infants,and there was a significant difference between them( P < 0. 01 ). Conclusions Clinical manifestations of newborn CHD are not typical and the early diagnosis is difficult, so attention should be paid to the suspected cases, and the diagnosis of CHD should be performed by auxiliary examinations as early as possible.%目的 分析新生儿先天性心脏病(CHD)的临床特点,为早期诊断提供依据.方法 收集本院2005年5月- 2010年8月54例CHD患儿.男31例,女23例;胎龄30~41周;出生体质量1200~3920g.分析CHD患儿的病理类型、病史、临床表现和辅助检查等情况.采用心脏彩色多普勒超声诊断仪探查其左心室长轴、大动脉短轴、四腔心(胸骨旁、心尖、剑突下),必要时胸骨上窝探查其主动脉弓,证实其心血管畸形存在.结果

  3. High Prevalence of Associated Birth Defects in Congenital Hypothyroidism

    OpenAIRE

    Alok Sachan; V. Suresh; D Rajasekhar; V. Vanaja; Harinarayan, C. V.; Rajagopal, G.; P Amaresh Reddy

    2010-01-01

    Aim. To identify dysmorphic features and cardiac, skeletal, and urogenital anomalies in patients with congenital hypothyroidism. Patients and Methods. Seventeen children with congenital primary hypothyroidism were recruited. Cause for congenital hypothyroidism was established using ultrasound of thyroid and T99mc radionuclide thyroid scintigraphy. Malformations were identified by clinical examination, echocardiography, X-ray of lumbar spine, and ultrasonography of abdomen. Results. Ten (59...

  4. CONGENITAL DISLOCATION OF RIGHT HIP JOINT: IMPORTANCE OF DYNAMIC ASSESSMENT

    OpenAIRE

    Pranita viveki; R. G. Viveki

    2014-01-01

    Congenital Dislocation of Hip (CDH), is one of the most common congenital diseases in the orthopedic field. It is also known as Developmental Dysplasia of Hip. The condition can be diagnosed by clinical, ultrasonographic and radiological examination. Here we are reporting two days old male baby with congenital dislocation of right hip joint. The goal of treatment is to obtain a reduction to provide an optimal environment for femoral head and acetabular development. Early diagnosis is the mo...

  5. Neuropathological Changes and Clinical Features of Autism Spectrum Disorder Participants Are Similar to that Reported in Congenital and Chronic Cerebral Toxoplasmosis in Humans and Mice

    Science.gov (United States)

    Prandota, Joseph

    2010-01-01

    Anatomic, histopathologic, and MRI/SPET studies of autistic spectrum disorders (ASD) patients' brains confirm existence of very early developmental deficits. In congenital and chronic murine toxoplasmosis several cerebral anomalies also have been reported, and worldwide, approximately two billion people are chronically infected with T. "gondii"…

  6. 先天性甲状腺功能减低症患儿的临床护理分析%Analysis of Clinical Nursing of Children With Congenital Hypothyroidism

    Institute of Scientific and Technical Information of China (English)

    霍丽春; 韩丽萍; 王红梅; 曹香雪

    2015-01-01

    Objective To investigate clinical nursing in children with congenital hypothyroidism.Methods From January 2014 to March 2015,30 children with congenital hypothyroidism,nursing measures were analyzed.Results 30 children with clinical treatment and intensive nursing had improved and discharged.Conclusion Children with congenital hypothyroidism disease should be Early, adequate, long-term and lifelong treatment. Children feel the warmth,received attention,through clinical treatment and intensive nursing,caring and encouraging,fuled of confidence, maintained a good mental state, and promoting recovery.%目的:探讨儿童先天性甲状腺功能减低症的临床护理。方法对2014年1月~2015年3月收治的先天性甲状腺功能减低症患儿30例的临床护理措施进行分析。结果30例患儿经临床治疗和精心护理均好转出院。结论先天性甲状腺功能减低症患儿应早期、足量、长期、终身治疗。通过临床治疗和精心护理及关怀和鼓励,使患儿感到温暖受重视,从而充满自信心,保持良好的心理状态,以促进康复。

  7. Dasatinib first-line: Multicentric Italian experience outside clinical trials.

    Science.gov (United States)

    Breccia, Massimo; Stagno, Fabio; Luciano, Luigiana; Abruzzese, Elisabetta; Annunziata, Mario; D'Adda, Mariella; Maggi, Alessandro; Sgherza, Nicola; Russo-Rossi, Antonella; Pregno, Patrizia; Castagnetti, Fausto; Iurlo, Alessandra; Latagliata, Roberto; Cedrone, Michele; Di Renzo, Nicola; Sorà, Federica; Rege-Cambrin, Giovanna; La Nasa, Giorgio; Scortechini, Anna Rita; Greco, Giovanna; Franceschini, Luca; Sica, Simona; Bocchia, Monica; Crugnola, Monica; Orlandi, Esther; Guarini, Attilio; Specchia, Giorgina; Rosti, Gianantonio; Saglio, Giuseppe; Alimena, Giuliana

    2016-01-01

    Dasatinib was approved for the treatment of chronic phase (CP) chronic myeloid leukemia (CML) patients in first line therapy based on the demonstration of efficacy and safety reported in patients enrolled in clinical trials. We describe a multicentric Italian "real-life" experience of dasatinib used as frontline treatment outside clinical trials. One hundred and nine patients (median age 54 years) were treated from January 2012 to December 2013. Increased incidence of high risk patients were detected according to stratification (26% according to Sokal score, 19% according to Euro score and 16% according to EUTOS) when compared to company sponsored studies. Median time from diagnosis to start of dasatinib was 18 days. Ten patients received unscheduled starting dose (6 patients 50mg and 4 patients 80 mg QD), whereas 99 patients started with 100mg QD. At 3 months, 92% of patients achieved a BCR-ABL ratio less than 10%. At 6 months, the rate of CCyR was 91% and the rate of MR3 was 40%, with 8% of the patients reaching MR4.5. Ninety-three patients were evaluable at 12 months: the rate of MR3 was 62%, with MR4.5 being achieved by 19% of the patients. At a median follow-up of 12 months, 27 patients (24.7%) were receiving the drug at reduced dose. Two patients (1.8%) experienced a lymphoid blast crisis and the overall incidence of resistance was 8%. As regards safety, the major side effects recorded were thrombocytopenia, neutropenia and pleural effusions, which occurred in 22%, 10% and 8% of patients, respectively. Present results, achieved in a large cohort of patients treated outside clinical trials, further confirm the efficacy and safety of dasatinib as firstline treatment in CML. PMID:26643920

  8. Clinical experience of medical students at university sains malaysia.

    Science.gov (United States)

    Quah, B S; Malik, A S; Simpson, H

    2000-01-01

    Experience of acute medical, surgical conditions, and clinical procedures of undergraduate students were assessed via a questionnaire survey during the final week of the 1993/1998 programme at the School of Medical Sciences, Univestiti Sains Malaysia. Individual performances were assessed by a scoring system. One hundred and twenty four students responded, (response rate 97%). More than 90% had seen myocardial infarction, cerebrovascular accident, pneumonia, respiratory distress, gastroenteritis, coma, and snake bite. Less than 33% had witnessed acute psychosis, diabetic ketoacidosis, acute hepatic failure, status epilepticus, near drowning, hypertensive encephalopathy, acute haemolysis or child abuse.Acute surgical/obstetrics cases, seen by >90% students, included fracture of long bones, head injury, acute abdominal pain, malpresentation and foetal distress. Less than 33% had observed epistaxis, sudden loss of vision, peritonitis or burns. Among operations only herniorrhaphy, Caesarian section, internal fixation of fracture and cataract extraction were seen by >80% students. The main deficits in clinical procedures are in rectal and vaginal examinations, urine collection and microscopic examinations. The performance of individual students, assessed by a scoring system, showed 15 students had unacceptably low scores (181.4/230, 70%) and 5 had superior scores (197.6/230, 80%). PMID:22844212

  9. Dabigatran in Secondary Stroke Prevention: Clinical Experience with 106 Patients

    Directory of Open Access Journals (Sweden)

    Alicia DeFelipe-Mimbrera

    2014-01-01

    Full Text Available Introduction. Our aim was to analyze our clinical experience with dabigatran etexilate in secondary stroke prevention. Methods. We retrospectively included patients starting dabigatran etexilate for secondary stroke prevention from March 2010 to December 2012. Efficacy and safety variables were registered. Results. 106 patients were included, median follow-up of 12 months (range 1–31. Fifty-six females (52.8%, mean age 76.4 (range 50–95, SD 9.8, median CHADS2 4 (range 2–6, CHA2DS2-VASc 5 (range 2–9, and HAS-BLED 2 (range 1–5. Indication for dabigatran etexilate was ischemic stroke in 101 patients and acute cerebral hemorrhage (CH due to warfarin in 5 (4.7%. Dabigatran etexilate 110 mg bid was prescribed in 71 cases (67% and 150 mg bid was prescribed in the remaining. Seventeen patients (16% suffered 20 complications during follow-up. Ischemic complications (10 were 6 transient ischemic attacks (TIA, 3 ischemic strokes, and 1 acute coronary syndrome. Hemorrhagic complications (10 were CH (1, gastrointestinal bleeding (6, mild hematuria (2, and mild metrorrhagia (1, leading to dabigatran etexilate discontinuation in 3 patients. Patients with previous CH remained uneventful. Three patients died (pneumonia, congestive heart failure, and acute cholecystitis and 9 were lost during follow-up. Conclusions. Dabigatran etexilate was safe and effective in secondary stroke prevention in clinical practice, including a small number of patients with previous history of CH.

  10. Medical academia clinical experiences of Ward Round Teaching curriculum

    Directory of Open Access Journals (Sweden)

    Fariba Haghani

    2014-01-01

    Full Text Available Background: Medical students spend most of their time in hospital wards and it is necessary to study clinical educational opportunities. This study was aimed to explore faculty members′ experience on Ward Round Teaching content. Methods and Materials: This qualitative study was conducted by purposive sampling with the maximum variation of major clinical departments faculty members in Isfahan University of Medical Sciences (n = 9. Data gathering was based on deep and semi-structured interviews. Data gathering continued till data saturation.Data was analyzed through the Collaizzi method and validated. Strategies to ensure trustworthiness of data (credibility, dependability, conformability, transferability were employed (Guba and Lincoln. Results: Basic codes extracted from the analyzed data were categorized into two main themes and related subthemes, including (1 tangible teachings (analytic intelligence, technical intelligence, legal duties and (2 implied teachings (professionalism, professional discipline, professional difficulties. Conclusion: Ward round teaching is a valuable opportunity for learners to learn not only patient care aspects but also ethical values. By appropriate planning, opportunities can be used to teach capabilities that are expected of general practitioners.

  11. Congenital hypothyroidism: current perspectives

    Directory of Open Access Journals (Sweden)

    Dayal D

    2015-07-01

    Full Text Available Devi Dayal, Rajendra Prasad Department of Pediatrics, Pediatric Endocrinology and Diabetes Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India Abstract: Congenital hypothyroidism (CH, the most common pediatric endocrine disorder with an estimated prevalence of 1:2,000 to 1:4,000, is an under-recognized problem in countries without routine newborn screening (NBS programs. Thyroid dysgenesis (TD is the most common cause of primary CH accounting for approximately 85% of all cases; most of the remaining patients have dyshormonogenesis. Transient CH and CH with eutopic gland, are increasingly being identified after introduction of routine NBS. The clinical features of CH are often subtle resulting in delayed diagnosis and eventually poor intellectual outcome. In developed countries, detection by NBS and early initiation of treatment has largely eliminated the intellectual disability caused by this disorder. The lower screening thyroid stimulating hormone (TSH cutoff and changes in birth demographics in some countries have been associated with an increase in the reported incidence of CH. However, the additional cases detected by the lower TSH cutoff tend to have either milder or transient hypothyroidism. Diagnosis of CH is made on the basis of serum concentrations of TSH and thyroxine (T4. Thyroid ultrasound, radionuclide scintigraphy, serum thyroglobulin (TG levels and specific genetic tests help ascertaining the exact etiological diagnosis. Non-availability of later tests should not deter the pediatrician from initiation of treatment. Age at initiation of treatment and starting dose of levothyroxine are critical factors that determine the long-term outcome. Higher doses of levothyroxine at 10–15 µg/kg/day are required in infants, with titration based on T4 and TSH levels, which are repeated frequently. Coexistence of other congenital anomalies in children with CH adds to the morbidity. Approximately 70% of babies

  12. Traumatic effects of political repression in Chile: a clinical experience.

    Science.gov (United States)

    Cordal, Margarita Díaz

    2005-10-01

    The author examines psychic trauma resulting from human rights violations in Chile. Starting from trauma theories developed by authors such as Ferenczi, Winnicott and Stolorow, she posits the relevance of the subject's emotionally significant environment in the production of the traumatic experience. She describes the characteristics of the therapeutic process on the basis of a clinical case. She emphasizes the need to recognize the damage that may be produced within the reliable link between patient and analyst, pointing out the risk of retraumatization if analysts distance themselves and apply 'technique' rigorously, leaving out their own subjective assessments. Therapists must maintain their focus on the conjunction of the patient's intersubjective context and inner psychic world both when exploring the origin of the trauma and when insight is produced. The author posits repetition in the transference as an attempt at reparation, at finding the expected response from the analyst that will help patients assemble the fragments of their history and achieve, as Winnicott would put it, a feeling of continuity in the experience of being.

  13. Genetics of congenital hypothyroidism

    OpenAIRE

    Park, S.; Chatterjee, V

    2005-01-01

    Congenital hypothyroidism is the most common neonatal metabolic disorder and results in severe neurodevelopmental impairment and infertility if untreated. Congenital hypothyroidism is usually sporadic but up to 2% of thyroid dysgenesis is familial, and congenital hypothyroidism caused by organification defects is often recessively inherited. The candidate genes associated with this genetically heterogeneous disorder form two main groups: those causing thyroid gland dysgenesis and those causin...

  14. Digital breast tomosynthesis (DBT): initial experience in a clinical setting

    International Nuclear Information System (INIS)

    false-positive findings. The side-by-side feature analysis showed higher conspicuity scores for tomosynthesis compared to conventional 2D for cancers presenting as spiculated masses and distortions. Conclusion: Tomosynthesis is a promising new technique. Our preliminary clinical experience shows that there is a potential for increasing the sensitivity using this new technique, especially for cancers manifesting as spiculated masses and distortions

  15. How Clinical Instructors Can Enhance the Learning Experience of Physical Therapy Students in an Introductory Clinical Placement

    Science.gov (United States)

    Cole, Beverley; Wessel, Jean

    2008-01-01

    Purpose: There is little understanding of how physical therapy students are influenced by clinical instructors (CIs) particularly at the outset of their clinical learning. The purpose of this study was to evaluate physical therapy students' perceptions of their learning experiences during an introductory clinical placement. Methods: Subjects were…

  16. [Radiological evaluation of congenital tumors].

    Science.gov (United States)

    Aguado del Hoyo, A; Ruiz Martín, Y; Lancharro Zapata, Á; Marín Rodríguez, C; Gordillo Gutiérrez, I

    2015-01-01

    In this article, we consider tumors that are diagnosed during pregnancy or in the first three months of life. This is a heterogeneous group of neoplasms with special biological and epidemiological characteristics that differentiate them from tumors arising in children or adults. In the last two decades, the prenatal detection of congenital tumors has increased due to the generalized use of prenatal sonographic screening. Advances in imaging techniques, especially in fetal magnetic resonance imaging, have enabled improvements in the diagnosis, follow-up, clinical management, and perinatal treatment of these tumors. This image-based review of the most common congenital tumors describes their histologic types, locations, and characteristics on the different imaging techniques used.

  17. Impact of the International Quality Improvement Collaborative on outcomes after congenital heart surgery: A single center experience in a developing economy

    Directory of Open Access Journals (Sweden)

    Rakhi Balachandran

    2015-01-01

    Full Text Available Background: The International Quality Improvement Collaborative (IQIC for Congenital Heart Surgery in Developing Countries was initiated to decrease mortality and major complications after congenital heart surgery in the developing world. Objective: We sought to assess the impact of IQIC on postoperative outcomes after congenital heart surgery at our institution. Methods: The key components of the IQIC program included creation of a robust worldwide database on key outcome measures and nurse education on quality driven best practices using telemedicine platforms. We evaluated 1702 consecutive patients ≤18 years undergoing congenital heart surgery in our institute from January 2010-December 2012 using the IQIC database. Preoperative variables included age, gender, weight at surgery and surgical complexity as per the RACHS-1 model. The outcome variables included, in- hospital mortality, duration of ventilation, intensive care unit (ICU stay, bacterial sepsis and surgical site infection. Results: The 1702 patients included 771(45.3% females. The median age was 8 months (0.03-216 and the median weight was 6.1Kg (1-100. The overall in-hospital mortality was 3.1%, Over the three years there was a significant decline in bacterial sepsis (from 15.1%, to 9.6%, P < 0.001, surgical site infection (11.1% to 2.4%, P < 0.001 and duration of ICU stay from 114(8-999 hours to 72 (18-999 hours (P < 0.001 The decline in mortality from (4.3% to 2.2% did not reach statistical significance. Conclusions: The inclusion of our institution in the IQIC program was associated with improvement in key outcome measures following congenital heart surgery over a three year period.

  18. A Short Version of the Revised ‘Experience of Close Relationships Questionnaire’: Investigating Non-Clinical and Clinical Samples

    OpenAIRE

    Wongpakaran, Tinakon; Wongpakaran, Nahathai

    2012-01-01

    Aim: This study seeks to investigate the psychometric properties of the short version of the revised ‘Experience of Close Relationships’ questionnaire, comparing non-clinical and clinical samples. Methods: In total 702 subjects participated in this study, of whom 531 were non-clinical participants and 171 were psychiatric patients. They completed the short version of the revised ‘Experience of Close Relationships’ questionnaire (ECR-R-18), the Perceived Stress Scale-10(PSS-10), the Rosenberg ...

  19. Non-invasive prenatal chromosomal aneuploidy testing--clinical experience: 100,000 clinical samples.

    Directory of Open Access Journals (Sweden)

    Ron M McCullough

    Full Text Available OBJECTIVE: As the first laboratory to offer massively parallel sequencing-based noninvasive prenatal testing (NIPT for fetal aneuploidies, Sequenom Laboratories has been able to collect the largest clinical population experience data to date, including >100,000 clinical samples from all 50 U.S. states and 13 other countries. The objective of this study is to give a robust clinical picture of the current laboratory performance of the MaterniT21 PLUS LDT. STUDY DESIGN: The study includes plasma samples collected from patients with high-risk pregnancies in our CLIA-licensed, CAP-accredited laboratory between August 2012 to June 2013. Samples were assessed for trisomies 13, 18, 21 and for the presence of chromosome Y-specific DNA. Sample data and ad hoc outcome information provided by the clinician was compiled and reviewed to determine the characteristics of this patient population, as well as estimate the assay performance in a clinical setting. RESULTS: NIPT patients most commonly undergo testing at an average of 15 weeks, 3 days gestation; and average 35.1 years of age. The average turnaround time is 4.54 business days and an overall 1.3% not reportable rate. The positivity rate for Trisomy 21 was 1.51%, followed by 0.45% and 0.21% rate for Trisomies 18 and 13, respectively. NIPT positivity rates are similar to previous large clinical studies of aneuploidy in women of maternal age ≥ 35 undergoing amniocentesis. In this population 3519 patients had multifetal gestations (3.5% with 2.61% yielding a positive NIPT result. CONCLUSION: NIPT has been commercially offered for just over 2 years and the clinical use by patients and clinicians has increased significantly. The risks associated with invasive testing have been substantially reduced by providing another assessment of aneuploidy status in high-risk patients. The accuracy and NIPT assay positivity rate are as predicted by clinical validations and the test demonstrates improvement in the

  20. Centralization of a Regional Clinical Microbiology Service: The Calgary Experience

    Directory of Open Access Journals (Sweden)

    Deirdre L Church

    1999-01-01

    Full Text Available Diagnostic laboratory services in Alberta have been dramatically restructured over the past five years. In 1994, Alberta Health embarked on an aggressive laboratory restructuring that cut back approximately 30% of the overall monies previously paid to the laboratory service sector in Calgary. A unique service delivery model consolidated all institutional and community-based diagnostic testing in a company called Calgary Laboratory Services (CLS in late 1996. CLS was formed by a public/private partnership between the Calgary Regional Health Care Authority (CRHA and MDS-Kasper Laboratories. By virtue of its customer service base and scope of testing, CLS provides comprehensive regional laboratory services to the entire populace. Regional microbiology services within CLS have been successfully consolidated over the past three years into a centralized high volume laboratory (HVL. Because the HVL is not located in a hospital, rapid response laboratories (RRLs are operated at each acute care site. Although the initial principle behind the proposed test menus for the RRLs was that only procedures requiring a clinical turnaround time of more than 2 h stay on-site, many other principles had to be used to develop and implement an efficient and clinically relevant RRL model for microbiology. From these guiding principles, a detailed assessment of the needs of each institution and extensive networking with user groups, the functions of the microbiology RRLs were established and a detailed implementation plan drawn up. The experience at CLS with regards to restructuring a regional microbiology service is described herein. A post-hoc analysis provides the pros and cons of directing and operating a regionalized microbiology service.

  1. Non-immune hydrops fetalis: Clinical experience in newborn infants

    Directory of Open Access Journals (Sweden)

    Pejić Katarina

    2011-01-01

    Full Text Available Introduction. Non-immune hydrops fetalis is a condition of excessive accumulation of extravascular fluid without identifiable circulating antibody to erythrocytes membrane antigens. In newborn infants it is characterized by skin oedema and pleural, pericardial or peritoneal effusion. In the era of routine Rh immunization for the prevention of foetal erythroblastosis, non-immune pathophysiologic mechanisms are presented in 76-87% of all hydropic newborns. Non-immune hydrops fetalis can be associated with numerous and various disorders. The mortality rate may exceed 50%. This study was aimed at presenting our clinical experience in treating newborn infants with non-immune hydrops fetalis. Material and methods. A retrospective-prospective study included newborn infants with non-immune hydrops fetalis, who were treated in the Neonatal Intensive Care Unit of Mother and Child Health Institute of Serbia between January 1, 2001 and October 31, 2010. All valid data about aetiology, diagnosis, clinical course and outcome were recorded. Results. The diagnosis of non-immune hydrops fetalis was made in 11 newborns. The etiologic diagnosis was established in 8 patients: anaemia due to fetomaternal transfusion in 4 patients and conatal cytomegalovirus infection, intracranial haemorrhage, isolated pulmonary lymphangiectasia and diffuse skin and mediastinal lymphangiomatosis in the remaining 4 patients. Conclusion. Non-immune hydrops of newborn infant is associated with a high mortality rate and requires complex diagnostic and therapeutic procedures. An optimal management of neonates with non-immune hydrops fetalis demands a multidisciplinary approach to the treatment in a neonatal intensive care unit.

  2. Exploring masculinity and marginalization of male undergraduate nursing students' experience of belonging during clinical experiences.

    Science.gov (United States)

    Sedgwick, Monique G; Kellett, Peter

    2015-03-01

    Aggressive recruitment strategies used in Canadian undergraduate nursing programs have enjoyed only moderate success, given that male students represent a small percentage of the student population. To determine whether there were gender differences in their sense of belonging, undergraduate nursing students (n = 462) in southern Alberta were surveyed using the Belongingness Scale-Clinical Placement Experience questionnaire. No significant gender differences were found on two of the subscales. However, male students demonstrated significantly lower scores on the efficacy subscale (p = 0.02). This finding suggests that some men experience feelings of marginalization and discrimination. Nurse educators and students are encouraged to explore their worldviews related to gendered performances and teaching practices that create bias. Practice environments are encouraged to deinstitutionalize policies and procedures that accentuate femininities of care. Finally, men entering into the nursing profession are encouraged to reflect on how their gender performance may facilitate or detract from their feelings of belonging.

  3. Pediatric congenital vertebral artery arteriovenous malformation.

    Science.gov (United States)

    Shownkeen, Harish; Bova, Davide; Chenelle, Andrew G; Origitano, Thomas C

    2003-05-01

    Vertebral arteriovenous fistulas are rare in children and the congenital form has been seldom reported in the literature. Prior to using endovascular therapy techniques, only surgery was the main treatment. The most common endovascular treatment is through the use of detachable balloons. This report describes the clinical and radiological findings of a congenital vertebral artery fistula in a 20-month-old child. Balloons could not be safely employed; therefore, embolization was performed with Guglielmi detachable microcoils. We review the history and treatment of these lesions, their clinical presentation, and imaging features, including their outcome, with particular attention to the pediatric population. PMID:12695869

  4. Pediatric congenital vertebral artery arteriovenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Shownkeen, Harish; Chenelle, Andrew G.; Origitano, Thomas C. [Department of Neurologcal Surgery, Loyola University Medical Center, 2160 S. First Avenue, Maywood, IL 60153 (United States); Bova, Davide [Department of Radiology, Loyola University Medical Center, 2160 S. First Avenue, Maywood, IL 60153 (United States)

    2003-05-01

    Vertebral arteriovenous fistulas are rare in children and the congenital form has been seldom reported in the literature. Prior to using endovascular therapy techniques, only surgery was the main treatment. The most common endovascular treatment is through the use of detachable balloons. This report describes the clinical and radiological findings of a congenital vertebral artery fistula in a 20-month-old child. Balloons could not be safely employed; therefore, embolization was performed with Guglielmi detachable microcoils. We review the history and treatment of these lesions, their clinical presentation, and imaging features, including their outcome, with particular attention to the pediatric population. (orig.)

  5. High Prevalence of Associated Birth Defects in Congenital Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Alok Sachan

    2010-01-01

    Full Text Available Aim. To identify dysmorphic features and cardiac, skeletal, and urogenital anomalies in patients with congenital hypothyroidism. Patients and Methods. Seventeen children with congenital primary hypothyroidism were recruited. Cause for congenital hypothyroidism was established using ultrasound of thyroid and T99mc radionuclide thyroid scintigraphy. Malformations were identified by clinical examination, echocardiography, X-ray of lumbar spine, and ultrasonography of abdomen. Results. Ten (59% patients (6 males and 4 females had congenital malformations. Two had more than one congenital malformation (both spina bifida and ostium secundum atrial septal defect. Five (29% had cardiac malformations, of whom three had only osteum secundum atrial septal defect (ASD, one had only patent ductus arteriosus (PDA, and one patient had both ASD and PDA. Seven patients (41% had neural tube defects in the form of spina bifida occulta. Conclusion. Our study indicates the need for routine echocardiography in all patients with congenital hypothyroidism.

  6. Radiochemotherapy of malignant glioma in adults. Clinical experiences

    Energy Technology Data Exchange (ETDEWEB)

    Kortmann, R.D.; Jeremic, B.; Plasswilm, L.; Bamberg, M. [Dept. for Radiation Oncology, Univ. of Tuebingen (Germany); Weller, M. [Dept. of Neurology, Univ. of Tuebingen (Germany)

    2003-04-01

    Background: Standard treatment in patients with malignant glioma consists of surgery and postoperative radiotherapy. A high early recurrence rate, particularly in glioblastoma, has led to the investigation of additional chemotherapy. Material and Methods: Recent results of radiochemotherapy published in the literature were reviewed with respect to outcome in phase II and III trials. Based on these experiences, aspects of future strategies were discussed. Results: 3 decades of intensive research had, unfortunately, little impact on the overall results. While early prospective studies established adjuvant nitrosoureas, particularly BCNU, as suitable adjuvant to surgery and postoperative radiotherapy, further studies largely concentrated on combined chemotherapeutic protocols, mostly procarbazine, CCNU and vincristine (PCV), which was shown to prolong survival in anaplastic astrocytoma. The recent MRC study, however, showed no effect for adjuvant PCV in grade III and IV malignant glioma. Only in high-grade glioma with an oligodendroglial component, additional chemotherapy may be of a decisive benefit. The introduction of newer drugs such as paclitaxel, temozolomide, or gemcitabine demonstrated no decisive advantage. Different modes of application and sequencing of radiotherapy and chemotherapy are presently actively investigated, but failed to substantially improve outcome. Conclusions: Therefore, search for newer and more effective drugs continues, as well as for ''optimal'' administration and sequencing, especially from the standpoint of accompanying acute and late toxicity. Finally, recent endeavors focused on basic research such as angiogenesis, migration and invasion, or induction of cell differentiation, but these strategies are still away from broader clinical investigation. (orig.)

  7. Single photon emission computed tomography: A clinical experience

    International Nuclear Information System (INIS)

    In the past decade, single photon emission computed tomography (SPECT) has evolved from an experimental technique used only in academic settings to a routine clinical examination performed in many community hospitals. Responding to reports of increased diagnostic efficacy, many nuclear medicine physicians have chosen to make SPECT imaging a routine technique for bone, liver, spleen, heart, and brain imaging. However, the enthusiasm for SPECT is not universal. Most nuclear medicine physicians continue to rely primarily on planar imaging, with little or no routine use of SPECT. This milieu has left many physicians asking themselves the following practical questions: Can SPECT be done easily in my hospital? Will not doing SPECT reduce the competitiveness of my nuclear medicine laboratory? The authors' experience at an institution heavily committed to SPECT for over 5 years may be helpful in answering these types of questions. The first rotating gamma camera at the Milwaukee Regional Medical Center was installed in late 1981. At present the authors have eight gamma cameras, of which four routinely perform SPECT examinations. Between 1981 and 1986, over 4,000 SPECT examinations have been performed

  8. Normothermic donor heart perfusion: current clinical experience and the future.

    Science.gov (United States)

    Messer, Simon; Ardehali, Abbas; Tsui, Steven

    2015-06-01

    Following the first successful heart transplant in 1967, more than 100,000 heart transplants have been carried out worldwide. These procedures have mostly relied on cold ischaemic preservation of the donor heart because this simple technique is inexpensive and relatively reliable. However, the well-known limitations of cold ischaemic preservation imposes significant logistical challenges to heart transplantation which put a ceiling on the immediate success on this life-saving therapy, and limits the number of donor hearts that can be safely transplanted annually. Although the theoretical advantages of normothermic donor heart perfusion have been recognised for over a century, the technology to transport donor hearts in this state has only been developed within the last decade. The Organ Care System (OCS) which is designed and manufactured by TransMedics Inc. is currently the only commercially available device with this capability. This article reviews the history of normothermic heart perfusion and the clinical experience with the TransMedics OCS to date. We have also attempted to speculate on the future possibilities of this innovative and exciting technology. PMID:24853906

  9. Congenital symmastia revisited

    DEFF Research Database (Denmark)

    Sillesen, Nanna H; Hölmich, Lisbeth R; Siersen, Hans Erik;

    2012-01-01

    Symmastia is defined as medial confluence of the breast. The term 'symmastia' is modified from Greek (syn meaning 'together', and mastos meaning 'breast') and was first presented by Spence et al. in 1983. Two forms of symmastia exist: an iatrogenic and a congenital version. Congenital symmastia i...

  10. Congenital Hepatic Fibrosis

    Directory of Open Access Journals (Sweden)

    MH Antikchi

    2010-09-01

    Full Text Available Congenital hepatic fibrosis (CHF is a rare disease that primarily involves hepatobiliary and renal systems. It is characterized by hepatic fibrosis, portal hypertension and renal cystic disease. We present a 22 years old man with fever, abdominal pain, icterus and hematemesis. On complete work up of the patient and liver with kidney biopsy, the diagnosis was congenital hepatic fibrosis.

  11. Congenital Insensitivity to Pain

    Directory of Open Access Journals (Sweden)

    Praveen Kumar B,

    2011-01-01

    Full Text Available Congenital Insensitivity to Pain belongs to the family of Hereditary Sensory and Autonomic Neuropathies (HSAN. It is a rare disorder of unknown etiology associated with loss of pain sensation. Cognition and sensation is otherwise normal and there is no detectable physical abnormality. We report a case of Congenital Insensitivity to Pain in a 3 year old female child.

  12. Cultural awareness: Enhancing clinical experiences in rural Appalachia.

    Science.gov (United States)

    Presley, Chaundel

    2013-01-01

    Students often work with clients from a cultural group different than their own. This is especially true for students completing clinical practica in Appalachia, where there is a culture unique to that geographic area. To prepare for this unique setting, common cultural scenarios experienced in the clinical setting must be addressed to help provide culturally appropriate patient care while developing required clinical competencies. Although applicable to most nursing students, the author discusses culturally specific approaches to clinical care of clients from Appalachia, specifically applied to nurse practitioner students, preceptors, and clinical faculty.

  13. Lymphangiosarcoma in a 3.5-year-old Bullmastiff bitch with vaginal prolapse, primary lymph node fibrosis and other congenital defects : clinical communication

    Directory of Open Access Journals (Sweden)

    J.H. Williams

    2005-06-01

    broad ligament and the ventral rectal serosa, and the 'prolapsed' tissue was found to be expanded vaginal wall. The bitch was euthanased and necropsied, Histological examination confirmed lymphangiosarcomatous invasion of the submucosal and muscular layers of the retroperitoneal, traumatised, prolapsed part of the vagina, the urethra and the ventral rectal wall. The broad ligament was diffusely invaded with tumour which had proliferated into the caudal abdominal space, and 3 small intra-trabecular foci of tumour were found in the right popliteal lymph node near the hilus. Mitotic figures were generally scarce. There was mild subcutaneous oedema of the ventral trunk extending from the axillae to the inner proximal thighs, which had not been evident clinically, and the lymph nodes (peripheral more so than internal microscopically showed marked trabecular and perivascular fibrosis especially in hilar regions. Other congenital defects were hepatic capsular and central venous fibrosis with lymphatic duplication and dilatation in all areas of connective tissue, ventrally-incongruous half-circular tracheal rings, and multifocal renal dysplasia affecting the right kidney. There was locally-extensive subacute pyelonephritis of the left kidney.

  14. Three-year experience with the Sophono in children with congenital conductive unilateral hearing loss: tolerability, audiometry, and sound localization compared to a bone-anchored hearing aid.

    Science.gov (United States)

    Nelissen, Rik C; Agterberg, Martijn J H; Hol, Myrthe K S; Snik, Ad F M

    2016-10-01

    Bone conduction devices (BCDs) are advocated as an amplification option for patients with congenital conductive unilateral hearing loss (UHL), while other treatment options could also be considered. The current study compared a transcutaneous BCD (Sophono) with a percutaneous BCD (bone-anchored hearing aid, BAHA) in 12 children with congenital conductive UHL. Tolerability, audiometry, and sound localization abilities with both types of BCD were studied retrospectively. The mean follow-up was 3.6 years for the Sophono users (n = 6) and 4.7 years for the BAHA users (n = 6). In each group, two patients had stopped using their BCD. Tolerability was favorable for the Sophono. Aided thresholds with the Sophono were unsatisfactory, as they did not reach under a mean pure tone average of 30 dB HL. Sound localization generally improved with both the Sophono and the BAHA, although localization abilities did not reach the level of normal hearing children. These findings, together with previously reported outcomes, are important to take into account when counseling patients and their caretakers. The selection of a suitable amplification option should always be made deliberately and on individual basis for each patient in this diverse group of children with congenital conductive UHL.

  15. An eight-year clinic experience with clozapine use in a Parkinson's disease clinic setting.

    Directory of Open Access Journals (Sweden)

    Nawaz Hack

    Full Text Available BACKGROUND: To examine our eight year clinic-based experience in a Parkinson's disease expert clinical care center using clozapine as a treatment for refractory psychosis in Parkinson's disease (PD. METHODS: The study was a retrospective chart review which covered eight years of clozapine registry use. Statistical T-tests, chi-square, correlations and regression analysis were used to analyze treatment response for potential associations of age, disease duration, and Hoehn & Yahr (H&Y score, and degree of response to clozapine therapy. RESULTS: There were 36 participants included in the analysis (32 PD, 4 parkinsonism-plus. The characteristics included 30.6% female, age 45-87 years (mean 68.3±10.15, disease duration of 17-240 months (mean 108.14±51.13 and H&Y score of 2 to 4 (mean 2.51±0.51. The overall retention rate on clozapine was 41% and the most common reasons for discontinuation were frequent blood testing (28%, nursing home (NH placement (11% and leucopenia (8%. Responses to clozapine across the cohort were: complete (33%, partial (33%, absent (16%, and unknown (16%. Age (r = -0.36, p0.05. CONCLUSIONS: This single-center experience highlights the challenges associated with clozapine therapy in PD psychosis. Frequent blood testing remains a significant barrier for clozapine, even in patients with therapeutic benefit. Surprisingly, all patients admitted to a NH discontinued clozapine due to logistical issues of administration and monitoring within that setting. Consideration of the barriers to clozapine therapy will be important to its use and to its continued success in an outpatient setting.

  16. Laser Ablation Of Atrial Fibrillation: Mid-term Clinical Experience

    Directory of Open Access Journals (Sweden)

    Li Poa, MD

    2009-08-01

    Full Text Available Background: Atrial Fibrillation is known to account for one third of all the strokes caused in the US in the population above the age of 70. Patients treated with the surgical Cox MAZE operation have been shown to have a 150 fold decrease in the incidence of stroke over an 18 year period. However, the original Cox MAZE although extremely successful in treating atrial fibrillation and decreasing the incidence of strokes was not performed widely because of complexity and invasiveness of the procedure. A variety of alternative energy based curative ablation strategies are now available for more minimally invasive therapeutic management of atrial fibrillation (AF. In this communication, we report our clinical experience in AF therapy utilizing laser energy ablation technology. Methods: Fifty two consecutive AF patients underwent concomitant or isolated ablation prior to any coexisting cardiac procedures that included CABG (coronary artery bypass surgery, MV (mitral valve or AV (aortic valve repairs. All patients had an epicardially based ablation pattern with basic lesions being en bloc box type pulmonary vein isolation which included the antral surface of the left atrium, directed ganglionectomies of the the right anterior and inferior ganglions, posteriomedial ablation of the IVC ( inferior vena cava, and a right isthmus ablation. Twenty seven patients had ligation of their left atrial appendage, 14 patients had resection of the ligament of Marshall, and three patients had endocardial placed lesions of a mitral annular connecting type lesion. In order to maintain the patients in normal sinus rhythm (NSR, electrical cardioversion and anti-arrhythmic drugs were employed as required. Results: At a median follow-up of 250 days, 44 of the total 52 patients (84.6% exhibited NSR.. No complications or mortality were reported due to the laser procedure. Conclusion: Laser ablation was successfully and safely used for endocardial and epicardial AF ablation

  17. Enhancing quantity and quality of clinical experiences in a baccalaureate nursing program.

    Science.gov (United States)

    Andresen, Kathleen; Levin, Pamela

    2014-01-01

    Nursing programs encounter barriers to clinical education, which may include limited clinical capacity for nursing students. Congestion of clinical placements compounded by multiple external influences prompted a need to develop an alternative approach for meeting program standards pertaining to clinical education. A curriculum improvement project was implemented within a school of nursing with the primary goal of expanding clinical learning opportunities while maintaining program quality. The unique aspect of this project was a comprehensive evaluative design, including qualitative responses from students, faculty, and clinical site stakeholders, as well as standardized student test scores. Augmenting the tools and processes for evaluation of clinical learning required collaboration from the faculty. Project outcomes include expanded clinical capacity, increased variety of clinical learning experiences, and improved quality of the clinical experiences. Collaborative partnerships yielded valuable lessons, which have implications for other nursing programs challenged with clinical placements. PMID:25205733

  18. Congenital anomalies associated with hypothyroidism.

    OpenAIRE

    Bamforth, J S; Hughes, I; Lazarus, J; John, R.

    1986-01-01

    Seven of the 34 infants identified through the Welsh Hypothyroid Screening Programme have additional congenital abnormalities. Two infants have a previously undescribed syndrome, two have chromosomal abnormalities, two have congenital heart disease, and one has a myelomeningocoele. Congenital hypothyroidism often seems to be associated with other congenital abnormalities.

  19. Impact of Simulation and Clinical Experience on Self-efficacy in Nursing Students: Intervention Study.

    Science.gov (United States)

    Kimhi, Einat; Reishtein, Judith L; Cohen, Miri; Friger, Michael; Hurvitz, Nancy; Avraham, Rinat

    2016-01-01

    This study compared the effect of simulation and clinical experience timing on self-confidence/self-efficacy for the nursing process. Using a randomized, double-crossover design, self-efficacy was measured 3 times. Although self-efficacy was significantly higher at time 1 for students who had clinical experience, there was no difference between the groups at the end of the course (time 2). Thus, simulation increased self-confidence/self-efficacy equivalently if placed either before or after clinical experience.

  20. 先天性心脏病的临床护理分析%Analysis of Clinical Nursing of Congenital Heart Disease

    Institute of Scientific and Technical Information of China (English)

    2013-01-01

    Objective To summarize the nursing points of interventional treatment of congenital heart disease, improve the quality of nursing care. Methods Selected in our hospital from 2011 December to 2013 January 24 cases of children with congenital heart disease, interventional nursing before operation for treatment of children with congenital heart disease in children, nursing and postoperative care and cooperation during the operation. Results 26 patients were successfully completed the intervention, returned to the wards. Conclusion Preoperative, intraoperative cooperation and nursing is a prerequisite for interventional therapy smoothly, and postoperative nursing is the key of interventional therapy in children with safety discharge.%  目的总结先天性心脏病介入治疗的护理要点,提高护理质量。方法选取我院2011年12月至2013年1月24例先天性心脏病患儿,对先天性心脏病患儿行介入治疗的患儿行术前护理,术中配合与护理及术后护理。结果26例患儿顺利完成介入治疗术,返回病房。结论术前护理、术中配合与护理是保障介入治疗顺利进行的前提,而术后护理是介入治疗术患儿安全出院的关键。

  1. Congenital Triangular Alopecia.

    Science.gov (United States)

    Yin Li, Vincent Chum; Yesudian, Paul Devakar

    2015-01-01

    Congenital triangular alopecia (CTA) also known as temporal triangular alopecia is a benign noncicatricial pattern of hair loss. It typically affects the frontotemporal region and rarely involves the temporoparietal or occipital scalp. It is a nonprogressive disorder that presents as a triangular, oval or lancet-shaped patch of alopecia. CTA can manifest at birth or develop later in life. The exact etiology of this condition remains unknown. Rarely, it may be associated with other disorders such as Down's syndrome and phakomatosis pigmentovascularis. The diagnosis is based on its distinct clinical appearance. Histologically, hair follicles are miniaturized and replaced by sparse vellus hair follicles. Tricoscopy using a polarized light handheld dermatoscope can be a useful diagnostic tool. CTA is often asymptomatic and remains unchanged throughout the life. No treatment is required. Surgical intervention with follicular unit hair transplantation can provide a satisfactory cosmetic result. In this paper, we have identified 126 cases of CTA in the published literature cited on PubMed between 1905 and 2015. From the available evidence, 79% of patients with CTA presented with unilateral hair loss, 18.5% with bilateral involvement and rarely, with occipital alopecia (2.5%). There was no gender predilection. These figures are entirely consistent with previously published data. Physicians should remember to consider CTA as a potential diagnosis in any patient presenting with a nonscarring alopecia in order to avoid unnecessary investigations and treatments. PMID:26180448

  2. Congenital parotid fistula

    Directory of Open Access Journals (Sweden)

    Shiggaon Natasha

    2014-01-01

    Full Text Available Parotid fistula is a cause of great distress and embarrassment to the patient. Parotid fistula is most commonly a post-traumatic situation. Congenital parotid salivary fistulas are unusual entities that can arise from accessory parotid glands or even more infrequently, from normal parotid glands through an aberrant Stensen′s duct. The treatment of fistulous tract is usually surgical and can be successfully excised after making a skin incision along the skin tension line around the fistula opening. This report describes a case of right accessory parotid gland fistula of a 4-year-old boy with discharge of pus from right cheek. Computed tomography (CT fistulography and CT sialography demonstrated fistulous tract arising from accessory parotid gland. Both CT fistulography and CT sialography are very helpful in the diagnosis and surgical planning. In this case, superficial parotidectomy is the treatment of choice. A detailed history, clinical and functional examination, proper salivary gland investigations facilitates in correct diagnosis followed by immediate surgical intervention helps us to restore physical, psychological health of the child patient.

  3. Congenital parotid fistula.

    Science.gov (United States)

    Natasha, Shiggaon

    2014-01-01

    Parotid fistula is a cause of great distress and embarrassment to the patient. Parotid fistula is most commonly a post-traumatic situation. Congenital parotid salivary fistulas are unusual entities that can arise from accessory parotid glands or even more infrequently, from normal parotid glands through an aberrant Stensen's duct. The treatment of fistulous tract is usually surgical and can be successfully excised after making a skin incision along the skin tension line around the fistula opening. This report describes a case of right accessory parotid gland fistula of a 4-year-old boy with discharge of pus from right cheek. Computed tomography (CT) fistulography and CT sialography demonstrated fistulous tract arising from accessory parotid gland. Both CT fistulography and CT sialography are very helpful in the diagnosis and surgical planning. In this case, superficial parotidectomy is the treatment of choice. A detailed history, clinical and functional examination, proper salivary gland investigations facilitates in correct diagnosis followed by immediate surgical intervention helps us to restore physical, psychological health of the child patient. PMID:25231049

  4. 先天性多指畸形X线表现与临床分型对照分析%Analysis of radiographic manifestations of congenital polydactyly and clinical classification

    Institute of Scientific and Technical Information of China (English)

    王亚军; 李胜; 余永桂

    2013-01-01

    Objective:To investigate the radiographic manifestations and their significance for clinical classification of congenital polydactyly,in order to provide improvement of its classification. Methods: Wassel and Stelling Twrek classifica tion was used as the basis of study,the radiographic manifestations and clinical materials of 85 cases with congenital poly dactyly (a total of 91 sick hands) were recruited,mainly to study the incidence of gender,type,location,characteristics and radiographic findings of congenital polytactyly. According to the radiographic manifestations, suggestion for further lm provement of classification was attempted. Results; All patients had been correctly diagnosed by radiography before surgery. Congenital polytactyly occurred at one hand accounts for 92. 94% (79/85 cases) ,mainly the right hand (the ratio of inci dence of left and right hands was approximately 0. 65: 1). The incidence of males was higher than that of females with the ratio as 3. 7 :1. Preaxial polydactyly was more common than Postaxial polydactyly,with the ratio being 9. 1:1. According to Wassel and Stelling Twrek classification,the most common type was Type IV with the incidence being approximately 51. 22% (42/82 cases) in preaxial polydactyly patients. However,there were about 4. 88% (4/82 cases) polydactyly patients which could not be classified yet. Conclusion:Congenital polydactyly is frequently seen. Preoperative radiographic examina tion is helpful not only for the classification but also for surgical planning. Yet improvement of clinical classification needs further study.%目的:探讨先天性多指畸形的X线表现及临床分型意义,总结经验并尝试性对分类方法进行完善.方法:以Wassel分类法和Stelling-Twrek分类法为依据,回顾性分析85例先天性多指畸形(91只病手)的X线表现及临床资料,主要观察先天性多指畸形的发病性别、类型、部位、特点及X线表现,依据本组病例X线表现尝试性对分

  5. Congenital mesoblastic nephroma: case report

    OpenAIRE

    POLAT, Mesut; Arisoy, Resul; Erdoğdu, Emre; ANGIN, A.Doğukan; TUĞRUL, Ahmet Semih

    2014-01-01

    Aim: Aim of the report is discuss the case who was prenatally diagnosed as congenital mesoblastic nephroma and  postnatal managenent. Case: We reported a case of 27 year old gravida 3, parity 1, abortion 1 referred to our clinic with the diagnosis of preterm labour and polihydramniosis at 34. gestational week. The ultrasonografic examination of the patient, with no antenatal follow up before, revealed a fetal biometry of 33 weeks and polyhydramniosis. A 63x66 mm solid mass with reguler border...

  6. Nursing Experience of Congenital Heart Disease in Infants and Children with Pulmonary Infection%小儿先天性心脏病合并肺部感染的护理体会

    Institute of Scientific and Technical Information of China (English)

    夏伟

    2015-01-01

    通过38例先心病并肺部感染患儿的护理工作总结护理经验,并通过治疗护理前后患儿BNP水平,评价治疗护理疗效。结果显示精心的护理是使患儿尽快恢复的重要措施,其中保持呼吸道通畅、病情观察是关键,健康教育、生活护理和心理护理是重点。%To investigate the nursing experience of congenital heart disease in infants and children with pulmonary infection by summarizing the 38 cases of nursing experience in children with congenital heart disease and pulmonary infection.In addition,level of brain natriuretic peptide was compared before and after treatment/nursing. Meticulous care is the important measure to recover as soon as possible,and keep the air way open and observe the condition are critical, health education,life care and psychological care are the key factors.

  7. Current advance in genetics and clinical phenotype of Leber congenital amaurosis%Leber先天性黑矇致病基因及相关临床表型研究进展

    Institute of Scientific and Technical Information of China (English)

    王淑然

    2013-01-01

    Leber congenital amaurosis (LCA) is one of the main inherited retinal diseases causing congenital blindness.LCA is also characterized by genetic heterogeneity and variable clinical phenotypes.Recent years,a lot of molecular genetic studies related with its pathogenesis have been performed.So far,20 causative genes have been identified that account for LCA.Some correlations between genotype and clinical phenotype have also been found.Those specific clinical manifestations may help to identify the mutant gene that causes the LCA.This review summarized the causal genes,their roles in the pathogenesis of LCA,coupled with relationship between specific gene and Corresponding phenotype,which will assist the clinician in patient diagnosis and counseling.%Leber先天性黑矇(LCA)是导致先天性盲的主要遗传性视网膜疾病,具有遗传异质性与临床表型多样性的特点.近年来其分子遗传学研究成为国内外热点,相继明确了20个与LCA相关的致病基因.多项研究表明LCA的基因型和临床表型之间存在关联,了解不同致病基因对应的临床表型特点有助于致病基因的筛查.就当前发现的LCA致病基因、可能的发病机制以及特定基因型与临床表型的关系进行综述,以期有助于临床诊断和咨询.

  8. Computer-assisted radiographic calculation of spinal curvature in brachycephalic "screw-tailed" dog breeds with congenital thoracic vertebral malformations: reliability and clinical evaluation.

    Directory of Open Access Journals (Sweden)

    Julien Guevar

    Full Text Available The objectives of this study were: To investigate computer-assisted digital radiographic measurement of Cobb angles in dogs with congenital thoracic vertebral malformations, to determine its intra- and inter-observer reliability and its association with the presence of neurological deficits. Medical records were reviewed (2009-2013 to identify brachycephalic screw-tailed dog breeds with radiographic studies of the thoracic vertebral column and with at least one vertebral malformation present. Twenty-eight dogs were included in the study. The end vertebrae were defined as the cranial end plate of the vertebra cranial to the malformed vertebra and the caudal end plate of the vertebra caudal to the malformed vertebra. Three observers performed the measurements twice. Intraclass correlation coefficients were used to calculate the intra- and inter-observer reliabilities. The intraclass correlation coefficient was excellent for all intra- and inter-observer measurements using this method. There was a significant difference in the kyphotic Cobb angle between dogs with and without associated neurological deficits. The majority of dogs with neurological deficits had a kyphotic Cobb angle higher than 35°. No significant difference in the scoliotic Cobb angle was observed. We concluded that the computer assisted digital radiographic measurement of the Cobb angle for kyphosis and scoliosis is a valid, reproducible and reliable method to quantify the degree of spinal curvature in brachycephalic screw-tailed dog breeds with congenital thoracic vertebral malformations.

  9. [Congenital Esophageal Atresia].

    Science.gov (United States)

    Suzuki, Makoto; Kuwano, Hiroyuki

    2015-07-01

    In this report, we describe the esophageal atresia in terms of current surgical management on the basis of our experience and literatures. Traditionally, infants with esophageal atresia have presented shortly after birth because of an inability to pass an orogastric tube, respiratory distress, or an inability to tolerate feeding. And also, an isolated trachea-esophageal fistula (TEF) usually cases coughing, recurrent pneumonia, or choking during feedings. To ignore these symptoms is to risk a delayed diagnosis. The condition may be associated with other major congenital anomalies such as those seen in the vertebral, anal, cardiac, tracheo-esophageal, renal/radial (VACTER) association, or it may be an isolated defect. Therapeutic strategies for esophageal atresia are a prevention of pulmonary complication by TEF closing and an early establishment of enteral alimentation. We promptly repair healthy infants without performing a gastrostomy and delay repair in infants with high-risk factors such as associated severe cardiac anomaly and respiratory insufficiency. Esophageal atresia has been classically approached through a thoracotomy. The disadvantages of such a thoracotomy have been recognized for a long time, for example winged scapula, elevation of fixation of shoulder, asymmetry of the chest wall, rib fusion, scoliosis, and breast and pectoral muscle maldevelopment. To avoid such disadvantages, thoracoscopic repair was recently reported. PMID:26197921

  10. Congenital heart disease

    Science.gov (United States)

    ... blood sugar level. Certain genes may play a role in congenital heart disease. Many family members may be affected. Talk to your provider about genetic counseling and screening if you have a family history ...

  11. Congenital nephrotic syndrome

    Science.gov (United States)

    The disorder often leads to infection, malnutrition, and kidney failure. It can lead to death by age 5, and many children die within the first year. Congenital nephrotic syndrome may be controlled in some cases with early ...

  12. Congenital adrenal hyperplasia

    Science.gov (United States)

    ... to treat congenital adrenal hyperplasia do not usually cause side effects such as obesity or weak bones, because the doses replace the hormones that the child's body cannot make. It is important for parents ...

  13. Photoacoustic Imaging in Oncology: Translational Preclinical and Early Clinical Experience.

    Science.gov (United States)

    Valluru, Keerthi S; Wilson, Katheryne E; Willmann, Jürgen K

    2016-08-01

    Photoacoustic imaging has evolved into a clinically translatable platform with the potential to complement existing imaging techniques for the management of cancer, including detection, characterization, prognosis, and treatment monitoring. In photoacoustic imaging, tissue is optically excited to produce ultrasonographic images that represent a spatial map of optical absorption of endogenous constituents such as hemoglobin, fat, melanin, and water or exogenous contrast agents such as dyes and nanoparticles. It can therefore provide functional and molecular information that allows noninvasive soft-tissue characterization. Photoacoustic imaging has matured over the years and is currently being translated into the clinic with various clinical studies underway. In this review, the current state of photoacoustic imaging is presented, including techniques and instrumentation, followed by a discussion of potential clinical applications of this technique for the detection and management of cancer. (©) RSNA, 2016. PMID:27429141

  14. TREATMENT OF HYPERTENSION IN PREGNANCY: GUIDELINES AND CLINICAL EXPERIENCE

    Directory of Open Access Journals (Sweden)

    O. V. Gaisenok

    2014-01-01

    Full Text Available Topical issues of the treatment of hypertension in pregnancy are presented. Examples from clinical practice are discussed as well as possible medical treatment of hypertension in pregnant women taking into account actual recommendations.

  15. Childhood victimisation and developmental expression of non-clinical delusional ideation and hallucinatory experiences: victimisation and non-clinical psychotic experiences

    OpenAIRE

    Lataster, Tineke; van Os, Jim; Drukker, Marjan; Henquet, Cécile; Feron, Frans; Gunther, Nicole; Myin-Germeys, Inez

    2006-01-01

    Victimisation in childhood may be associated with adult psychosis. The current study examined this association in the crucial developmental period of early adolescence and investigated whether (1) unwanted sexual experiences, and (2) being bullied, were associated with non-clinical delusional ideation and hallucinatory experiences in a general population sample of 14 year olds.

  16. Developing a leadership pipeline: the Cleveland Clinic experience

    OpenAIRE

    Hess, Caryl A.; Barss, Christina; Stoller, James K

    2014-01-01

    The complexity of health care requires excellent leadership to address the challenges of access, quality, and cost of care. Because competencies to lead differ from clinical or research skills, there is a compelling need to develop leaders and create a talent pipeline, perhaps especially in physician-led organizations like Cleveland Clinic. In this context, we previously reported on a cohort-based physician leadership development course called Leading in Health Care and, in the current report...

  17. Congenital oesophageal hiatal hernia in a pug

    Directory of Open Access Journals (Sweden)

    Keeley B

    2008-06-01

    Full Text Available Abstract Congenital sliding, oesophageal or type I hiatal hernia was diagnosed in a five-month-old pug puppy presented for evaluation of dyspnoea post feeding. The diagnosis was confirmed using plain film radiography. Surgical reduction of the hernia followed by plication of the oesophageal hiatus, oesophagopexy and left flank gastropexy permitted restoration of normal function. At 12 months of age, the dog was asymptomatic. This article describes the diagnosis and treatment of a congenital type 1 oesophageal hiatal herniation with unusual clinical presentation.

  18. Congenital myasthenic syndromes and transient myasthenia gravis.

    Science.gov (United States)

    Gajda, Anna; Szabó, Hajnalka; Gergev, Gyurgyinka; Karcagi, Veronika; Szabó, Nóra; Endreffy, Emoke; Túri, Sándor; Sztriha, László

    2013-05-30

    Hypotonia in the neonatal period and early infancy is a common clinical finding. It can be caused by various heterogeneous disorders of different origin which might lead to diagnostic difficulties. Disorders of the neuromuscular junction, such as congenital myasthenic syndromes and neonatal transient myasthenia gravis are among the aetiologies. We report on a case of congenital myasthenia caused by mutation in the long cytoplasmic loop of the epsilon subunit of the acetylcholine receptor and a neonate of a myasthenic mother diagnosed with transient myasthenia gravis. PMID:23909021

  19. Congenital bilateral perisylvian syndrome with hydrocephalus

    Directory of Open Access Journals (Sweden)

    Salih Hattapoğlu

    2012-12-01

    Full Text Available Congenital bilateral perisylvian syndrome (CBPS, whichis seen by indications of mental retardasyon, epilepsi,speech disorder and pseudobulbar palsy, is a diseasewhich comes up with genetic and non-genetic reasons.Revealing characteristic indications (like polymicrogyriawith MR imaging and clinic indications contributes makingdiagnosis. In present paper, we aimed to present 18month girl case report who diagnosed as CBPS with hydrocephaliindication. J Clin Exp Invest 2012; 3(4: 552-554Key words: Epilepsy, congenital bilateral perisylviansyndrome, polymicrogyria, hydrocephalus, pseudobulbarpalsy

  20. Congenital tumors of the central nervous system

    Energy Technology Data Exchange (ETDEWEB)

    Severino, Mariasavina [G. Gaslini Children' s Hospital, Department of Neuroradiology, Genoa (Italy); Schwartz, Erin S. [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Thurnher, Majda M. [Medical University of Vienna, Department of Radiology, Vienna (Austria); Rydland, Jana [MR Center, St. Olav' s Hospital HF, Trondheim (Norway); Nikas, Ioannis [Agia Sophia Children' s Hospital, Imaging Department, Athens (Greece); Rossi, Andrea [G. Gaslini Children' s Hospital, Department of Neuroradiology, Genoa (Italy); G. Gaslini Children' s Hospital, Department of Pediatric Neuroradiology, Genoa (Italy)

    2010-06-15

    Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into ''definitely congenital'' (present or producing symptoms at birth), ''probably congenital'' (present or producing symptoms within the first week of life), and ''possibly congenital'' (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors

  1. Congenital laryngeal anomalies,

    OpenAIRE

    Rutter, Michael J.

    2014-01-01

    Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the ma...

  2. Congenital chylous ascites

    OpenAIRE

    Romańska-Kita, Justyna; Borszewska-Kornacka, Maria Katarzyna; Dobrzańska, Anna; Rudzińska, Iwona; Czech-Kowalska, Justyna; Wawrzoniak, Tomasz

    2011-01-01

    Summary Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity.

  3. Congenital Cystic Lung Diseases

    Directory of Open Access Journals (Sweden)

    Aditi Jain

    2013-01-01

    Full Text Available Congenital cystic diseases of the lung are a rare but significant cause of morbidity in children and young adults presenting with respiratory distress and repeated chest infections. They consist of cystic adenomatoid malformation, bronchogenic cyst, pulmonary sequestration, and congenital lobar emphysema. Surgical treatment is a safe and an effective method of treatment. Chest X-ray and computed tomography are the key imaging modalities used for diagnosis.

  4. How to Conduct Clinical Qualitative Research on the Patient's Experience

    Science.gov (United States)

    Chenail, Ronald J.

    2011-01-01

    From a perspective of patient-centered healthcare, exploring patients' (a) preconceptions, (b) treatment experiences, (c) quality of life, (d) satisfaction, (e) illness understandings, and (f) design are all critical components in improving primary health care and research. Utilizing qualitative approaches to discover patients' experiences can…

  5. Clinical and molecular genetic analysis of a Chinese family with congenital X-linked adrenal hypoplasia caused by novel mutation 1268delA in the DAX-1 gene.

    Science.gov (United States)

    Zhang, Zhe; Feng, Ye; Ye, Dan; Li, Cheng-jiang; Dong, Feng-qin; Tong, Ying

    2015-11-01

    Congenital X-linked adrenal hypoplasia (AHC) is a rare disease characterized by primary adrenal insufficiency before adolescence and by hypogonadotropic hypogonadism (HHG) during adolescence. In this paper, we present a Chinese family with AHC. Two brothers, misdiagnosed with adrenal insufficiency of unknown etiology at the age of 9, were correctly diagnosed with AHC when delayed puberty, HHG, and testicular defects were observed. We investigated the clinical features and identified the dosage-sensitive sex reversal AHC critical region of the X chromosome gene 1 (DAX-1) mutation in this kindred. Direct sequencing of the DAX-1 gene revealed that the two siblings have a novel mutation (1268delA) of which their mother is a heterozygous carrier. This mutation causes a frameshift and a premature stop codon at position 436, encoding a truncated protein. It is important to increase knowledge of the mutational spectrum in genes related to this disease, linking phenotype to genotype. PMID:26537215

  6. An Enzymatic Clinical Chemistry Laboratory Experiment Incorporating an Introduction to Mathematical Method Comparison Techniques

    Science.gov (United States)

    Duxbury, Mark

    2004-01-01

    An enzymatic laboratory experiment based on the analysis of serum is described that is suitable for students of clinical chemistry. The experiment incorporates an introduction to mathematical method-comparison techniques in which three different clinical glucose analysis methods are compared using linear regression and Bland-Altman difference…

  7. Congenital scoliosis: an up-to-date.

    Science.gov (United States)

    Burnei, G; Gavriliu, S; Vlad, C; Georgescu, I; Ghita, R A; Dughilă, C; Japie, E M; Onilă, A

    2015-01-01

    Congenital scoliosis represents a spinal malformation due to defects of formation, segmentation or mixed ones. It is characterized by a longitudinal and rotational imbalance. 54 patients were analyzed and 39 out of them were operated by various approaches with anterior and posterior instrumentations during 2000 and 2012. The impossibility to appoint some patients encountered in the daily practice into the known classifications, allowed us to purpose two categories of congenital scoliosis related to the predominance of spinal deviances in the coronal and transversal views. No certain etiology of congenital scoliosis has been identified until today. The susceptibility of some polygenic defects is obvious due to the presence of a sum of defects associated to most of the congenital scoliosis cases and the rarity of the presence of a unique defect. The diagnosis requires a thorough clinical and imaging examination in order to establish an individualized therapeutic strategy. The treatment of congenital scoliosis is different from the adolescent idiopathic one. Therapeutic criteria are significantly different. It is essential to assess the difference in growth of the concavity related to the convexity when choosing a particular procedure. The magnitude of the curve and the progressive rate are fundamental issues to the surgeon.

  8. Sporotrichosis in childhood: clinical and therapeutic experience in 25 patients.

    Science.gov (United States)

    Bonifaz, Alexandro; Saúl, Amado; Paredes-Solis, Vanessa; Fierro, Leonel; Rosales, Alejandra; Palacios, Carolina; Araiza, Javier

    2007-01-01

    Sporotrichosis in an uncommon mycoses in childhood and is generally associated with injuries received as a consequence of farm work. We undertook a retrospective study of sporotrichosis in children and adolescents seen over a 10-year period, focusing on their clinical, epidemiologic, and mycologic features as well as treatment. We included 25 children with a mean age of 9.3 years. Most of those affected were schoolchildren (84%) from rural areas. The main clinical variety of sporotrichosis seen was the lymphocutaneous form (64%), followed by the fixed cutaneous form (36%), and one instance of the disseminated cutaneous form. Most lesions were located on the upper limbs (40%) and the face (36%). Sporothrix schenckii was isolated in all patients and 24 of 25 had a positive sporotrichin skin test. Nineteen patients were treated and cured clinically and mycologically with potassium iodide, three were cured with itraconazole and one with heat therapy.

  9. Clinical treatment discussion of neonatal congenital megacolon combined with enterocolitis%新生儿先天性巨结肠合并小肠结肠炎的临床治疗

    Institute of Scientific and Technical Information of China (English)

    谢文雅

    2015-01-01

    目的:探讨新生儿先天性巨结肠合并小肠结肠炎的临床治疗方法。方法:2013年2月-2014年10月收治新生儿先天性巨结肠合并小肠结肠炎患儿60例,随机分为观察组和对照组,各30例。对照组为采用保守治疗方法的患儿,观察组为保守治疗无效行肠造瘘术手术治疗的患儿,对比和分析两组患者的治疗效果。结果:观察组治愈率86.7%,对照组治愈率96.6%,观察组的治愈率高于对照组,差异具有统计学意义(P<0.05)。结论:对先天性巨结肠合并小肠结肠炎新生儿采取肠造瘘术治疗效果好,值得临床参考。%Objective:To explore clinical treatment method of neonatal congenital megacolon combined with enterocolitis.Methods:60 children patients with neonatal congenital megacolon combined with enterocolitis were selected from February 2013 to October 2014.They were randomly divided into the observation group and the control group with 30 cases in each.The control group was the children with conservative treatment method,the observation group was the children with enterostomy operation treatment after invalid conservative treatment.The treatment effects of patients in two groups were compared and analyzed.Results:The cure rate of the observation group was 86.7%,the cure rate of the control group was 96.6%,the cure rate of the observation group was higher than that of the control group,the difference was statistically significant(P<0.05).Conclusion:Using enterostomy in the treatment of neonatal congenital megacolon combined with enterocolitis has good effect.It is worthy of clinical use and reference.

  10. 先天性白癜风73例临床特征分析与治疗总结%Clinical features and therapy of 73 cases of congenital vitiligo

    Institute of Scientific and Technical Information of China (English)

    韦超; 马翠玲; 高天文; 李春英

    2012-01-01

    目的:总结分析先天性白癜风患者的临床特点及治疗方法,比较先天性白癜风与儿童白癜风的异同点.方法:对本院2000年1月~2011年12月诊治的73例先天性白癜风患者的临床资料进行回顾性分析.结果:73例先天性白癜风患者男女比例为0.92:1.神经节段型占15.1%,病情处于进展期者占56.2%,伴发晕痣者3例,有阳性家族史者占21.9%,治疗后好转或稳定者占86.7%.结论:①白癜风并非完全后天发病,出生时即可发生;②日晒可能是一种诱发加重因素;③有阳性家族史者符合多基因遗传规律;④联合治疗有较好的效果.%Objective To investigate the clinical characteristics of congenital vitiligo and find out its effective treatment, compared with a control group of vitiligo in children. Methods The clinical data and therapeutic conditions of 73 patients diagnosed with congenital vitiligo from 2000 to 2011 were retrospectively analyzed. Results 73 cases of congenital vitiligo were evaluated. Males constituted 47.9% (35) of the group and female 52.1%(38). Segmental vitiligo was in 11 (15.1%) patients. Condition in progress was present in 41 (56.2%) patients. Family history of vitiligo was present in 16 (21.9%) patients. Halo nevus was showed in 3 patients. The proportion of patients with improved condition after treatment was 86.7%. Conclusion ①Vitiligo can onset while born, not all acquired. ②Solarization may be an aggressive factor. ③lt is accorded with polygene heredity rule in family vitiligo. ④Combination therapy has good effect.

  11. Dr. Lin Zhu's Clinical Experience in Treating Mental Disorders

    Institute of Scientific and Technical Information of China (English)

    Ma Xueqing; Wang Xinzhong

    2005-01-01

    @@ Mental disorders are mainly caused by the emotional factors. Chief physician Lin Zhu, a famous TCM doctor in Beijing, is very good at treating this kind of disorders. The following is a summary of Prof. Lin's experience in this aspect.

  12. Differences in Clinical Experiences of ADN and BSN Students.

    Science.gov (United States)

    Oermann, Marilyn H.

    1998-01-01

    In a study of 211 associate degree (AD) and 204 baccalaureate nursing students, AD students reported significantly higher stress in clinical practice. Stress for both groups increased as they progressed. Instructors were the predominant source of stress. Students had the most difficulty coping with the demands of patient care and the clinical…

  13. Clinical Experience in TCM Treatment of Chronic Cervicitis

    Institute of Scientific and Technical Information of China (English)

    周宜强; 范宏宇

    2002-01-01

    @@ Chronic cervicitis is a common disease in the female reproductive system, which may be the inducing factor for carcinoma of uterine cervix. It is clinically manifested by sticky and foul leukorrhagia, contact hemorrhage, pain in the lower limbs or lumbosacral region, dysmenorrhea and infertility.

  14. Managing University Clinical Partnership: Learning from International Experience

    Science.gov (United States)

    Davies, Stephen; Smith, Tom

    2004-01-01

    Dialogue between the leaders of academic clinical organisations in different countries has revealed that the core elements of the partnership between universities and health care systems are remarkably consistent across national boundaries. There is now an impetus to move beyond analysis of common challenges and towards strategies for success that…

  15. Multiple congenital epulis in newborn - A rare presentation

    Directory of Open Access Journals (Sweden)

    Sahu Sandeep

    2009-01-01

    Full Text Available A newborn infant with multiple congenital oral swellings can be a striking sight for both parents and healthcare professionals involved in neonatal care. Neumann first described congenital epulis of a newborn in 1871, hence it is also known as Neumann′s tumor. Congenital epulis occurs at birth and has a predilection for females. Congenital epulis clinically appears as a pedunculated protuberant mass. In cases with large lesions, mechanical, oral, and nasal obstruction can impair fetal deglutition or interfering with feeding and / or respiratory impairment. We are reporting a case of a six-day-old, 3.5 kg newborn female, who had reported to our institution with multiple congenital oral swellings arising from the maxilla and mandible.

  16. Postnatal Cardiac Autonomic Nervous Control in Pediatric Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Ineke Nederend

    2016-04-01

    Full Text Available Congenital heart disease is the most common congenital defect. During childhood, survival is generally good but, in adulthood, late complications are not uncommon. Abnormal autonomic control in children with congenital heart disease may contribute considerably to the pathophysiology of these long term sequelae. This narrative review of 34 studies aims to summarize current knowledge on function of the autonomic nervous system in children with a congenital heart defect. Large scale studies that measure both branches of the nervous system for prolonged periods of time in well-defined patient cohorts in various phases of childhood and adolescence are currently lacking. Pending such studies, there is not yet a good grasp on the extent and direction of sympathetic and parasympathetic autonomic function in pediatric congenital heart disease. Longitudinal studies in homogenous patient groups linking autonomic nervous system function and clinical outcome are warranted.

  17. Juvenile nasopharyngeal angiofibroma: Clinical diagnosis and treatment experience

    Directory of Open Access Journals (Sweden)

    Sladoje Radmila

    2002-01-01

    Full Text Available Juvenile nasopharyngeal angiofibroma is an infrequent epi-pharyngeal tumor necessitating particular diagnostic and therapeutic procedures in comparison to other benign epipharyngeal tumors due to its expansive growth tendency. Our retrospective study is aimed at presenting clinical casuistry of the tumor in order to evaluate modern diagnostic and therapeutic possibilities. The study included 13 male patients, aged 13-24 years, who were hospitalized, diagnostically assessed and surgically treated at the Institute of Otorhinolaryngology and Maxillofacial Surgery, Clinical Centre of Serbia over the period 1990 - June 2001. The following parameters were analyzed: sex, age groups, preoperative symptoms of the disease, diagnostic methods, embolization, local tumor spreading, number and time of tumor relapses and surgical approach.

  18. Clinical course of ectopic pregnancy: A single-center experience

    OpenAIRE

    Aqueela Ayaz; Sameh Emam; Mian Usman Farooq

    2013-01-01

    OBJECTIVES: The objective was to highlight the frequency, clinical profile, and predisposing factors of ectopic pregnancy (EP) in a general hospital. MATERIALS AND METHODS: This descriptive study was conducted at the Obstetrics and Gynaecology department of Hera General hospital, Makkah, Saudi Arabia, from July 1, 2009 to December 29, 2010. Data were collected on chief medical complaints, sociodemographic characteristics, past obstetrics and gynecological history, management done, and outcome...

  19. The First Experience of Clinical Practice on Psychology Students’ Imaginary

    OpenAIRE

    Sueli Regina Gallo-Belluzzo; Elisa Corbett; Tania Maria Jose Aiello-Vaisberg

    2013-01-01

    Considering the academic development of the psychologist as a complex process which articulates the transmission of scientific knowledge and changes in imaginative activity, we psychoanalytically investigate the collective imaginary of Psychology students regarding the first clinical consultation. We conducted a group interview with 52 undergraduate students, using the Thematic Story-Drawing Procedure as a way to open a dialogical field. The material obtained, through the psychoanalytical met...

  20. Knowledge Management: Experiences in Academic and Clinic Environments

    Directory of Open Access Journals (Sweden)

    Martha Torres Narváez

    2014-05-01

    Full Text Available This article describes the knowledge management practices derived from clinical research, and some hospitals and academic institutions in Colombia. The profile of these institutions and the variables involved in knowledge management and research are presented. Objective: Exploring the practices that some Colombian institutions have to build or strengthen their capacity to con-duct clinical research. Materials and methods: We conducted an exploratory study by consulting a convenience sample, the use of specific practices in knowledge management. In a documentary analysis on knowledge management. The information was obtained of primary sources by semi-structured interview applied to selected key players. Results: In the institutions that participated in the study, it was observed as determinants in knowledge management: structure and strategic management, intellectual capital, technological resources, and the continuity and sustainability of the processes. Conclusion: Include research processes in services delivery institutions is criti¬cal to improving the quality of health care and encourage their intellectual capital. Colombia has institutions working with determination to the generation of knowledge from clinical practice.

  1. Improving biological relevancy of transcriptional biomarkers experiments by applying the MIQE guidelines to pre-clinical and clinical trials.

    Science.gov (United States)

    Dooms, M; Chango, A; Barbour, E; Pouillart, P; Abdel Nour, A M

    2013-01-01

    The "Minimum Information for the Publication of qPCR Experiments" (MIQE [3]) guidelines are very much targeted at basic research experiments and have to our knowledge not been applied to qPCR assays carried out in the context of clinical trials. This report details the use of the MIQE qPCR app for iPhone (App Store, Apple) to assess the MIQE compliance of one clinical and five pre-clinical trials. This resulted in the need to include 14 modifications that make the guidelines more relevant for the assessment of this special type of application. We also discuss the need for flexibility, since while some parameters increase experimental quality, they also require more reagents and more time, which is not always feasible in a clinical setting. PMID:22910527

  2. A Comparison of a Traditional Clinical Experience to a Precepted Clinical Experience for Baccalaureate-Seeking Nursing Students in Their Second Semester

    OpenAIRE

    Linda Dune; Renae Schumann; Kristin Ownby; David Kohne

    2012-01-01

    The shortage of nursing faculty has contributed greatly to the nursing workforce shortage, with many schools turning away qualified applicants because there are not enough faculty to teach. Despite the faculty shortage, schools are required to admit more students to alleviate the nursing shortage. Clinical groups in which preceptors are responsible for student learning extend faculty resources. Purpose. To determine the effectiveness of an alternative clinical experience (preceptorship). Meth...

  3. Radiologic imaging of congenital gastrointestinal anomalies in infants

    Directory of Open Access Journals (Sweden)

    Leny Zabidi

    2014-11-01

    Full Text Available Background Congenital gastrointestinal anomalies may manifest signs or symptoms in the first few days of life, most commonly in the form of obstructions. Radiologic imaging plays an important role in diagnosis confirmation and surgical correction plans. Most cases may be diagnosed by plain radiographs alone, but CT scans and MRI may be needed to make accurate diagnoses, especially in difficult cases. Objective To report radiologic imaging findings in infants with congenital gastrointestinal anomalies. Methods For this retrospective, cross-sectional study we took secondary data from medical records of infants with congenital gastrointestinal anomalies in Dr. Kariadi Hospital, Semarang, Indonesia from January 2010 – June 2011. Diagnosis of congenital anomalies was confirmed by clinical manifestation and radiologic imaging. Radiologic findings were reviewed by a single radiologist on duty at that time. Data is presented in the form of frequency distribution. Results Subjects consisted of 50 males and 23 females. The most common complaints were vomiting in 14 subjects (19%, abdominal distension in 31 subjects (43%, and fecal passage dysfunction in 28 subjects (38%. Radiologic imaging of subjects with congenital gastrointestinal anomalies revealed the following conditions: anal atresia in 28 subjects (38%, congenital megacolon in 21 subjects (29%, esophageal atresia in 14 subjects (19%, duodenal atresia in 9 subjects (12%, and pyloric atresia in 1 subject (2%. Conclusion Using radiologic imaging of infants with congenital gastrointestinal anomalies, the most to least common conditions found were anal atresia, congenital megacolon, esophageal atresia, duodenal atresia, and pyloric atresia.

  4. Association Between Medicare Summary Star Ratings for Patient Experience and Clinical Outcomes in US Hospitals

    Directory of Open Access Journals (Sweden)

    Stephen Trzeciak MD, MPH

    2016-03-01

    Full Text Available Objective: In 2015, the Centers for Medicare and Medicaid Services (CMS released new summary star ratings for US hospitals based on patient experience. We aimed to test the association between CMS patient experience star ratings and clinical outcomes. Methods: We analyzed risk-adjusted data for more than 3000 US hospitals from CMS Hospital Compare using linear regression. Results: We found that better patient experience was associated with favorable clinical outcomes. Specifically, a higher number of stars for patient experience had a statistically significant association with lower rates of many in-hospital complications. A higher patient experience star rating also had a statistically significant association with lower rates of unplanned readmissions to the hospital within 30 days. Conclusion: Better patient experience according to the CMS star ratings is associated with favorable clinical outcomes. These results support the inclusion of patient experience data in the framework of how hospitals are paid for services.

  5. Medical students' emotional development in early clinical experience: a model.

    Science.gov (United States)

    Helmich, Esther; Bolhuis, Sanneke; Laan, Roland; Dornan, Tim; Koopmans, Raymond

    2014-08-01

    Dealing with emotions is a critical feature of professional behaviour. There are no comprehensive theoretical models, however, explaining how medical students learn about emotions. We aimed to explore factors affecting their emotions and how they learn to deal with emotions in themselves and others. During a first-year nursing attachment in hospitals and nursing homes, students wrote daily about their most impressive experiences, explicitly reporting what they felt, thought, and did. In a subsequent interview, they discussed those experiences in greater detail. Following a grounded theory approach, we conducted a constant comparative analysis, collecting and then interpreting data, and allowing the interpretation to inform subsequent data collection. Impressive experiences set up tensions, which gave rise to strong emotions. We identified four 'axes' along which tensions were experienced: 'idealism versus reality', 'critical distance versus adaptation', 'involvement versus detachment' and 'feeling versus displaying'. We found many factors, which influenced how respondents relieved those tensions. Their personal attributes and social relationships both inside and outside the medical community were important ones. Respondents' positions along the different dimensions, as determined by the balance between attributes and tensions, shaped their learning outcomes. Medical students' emotional development occurs through active participation in medical practice and having impressive experiences within relationships with patients and others on wards. Tensions along four dimensions give rise to strong emotions. Gaining insight into the many conditions that influence students' learning about emotions might support educators and supervisors in fostering medical students' emotional and professional development. PMID:23949724

  6. Clinical analysis of 9 cases of complicated pneumomediastinum in infants with congenital heart disease after open heart surgery.%婴幼儿先心病术后并发纵膈气肿临床分析

    Institute of Scientific and Technical Information of China (English)

    仇杰; 费忠化; 刘宏生

    2012-01-01

    Objective To explore the common causes, diagnosis and treatment approaches in infants with congenital heart disease complicated pneumomediastinum after open heart surgery. Methods The diagnosis and treatment approaches of complicated pneumomediastinum of 9 infants with congenital heart disease after open-heart surgery from January 2011 to February 2012 in our hospital were retrospectively studied. Results 5 cases recovered in all patients, 3 cases treatment ineffective ( can' t maintain the circulation and abandoned treatment) , 1 case died due to the ineffective control of serious infection. Conclusions Congenital heart disease complicated pneumomediastinum in children after open heart surgery has great influences on heart-lung capability, and may complicated with mediastinal infection. Otherwise, because of it s variation in clinical manifestations, misdiagnosis and a high mortality rate were likely to happen. With an early diagnosis and treatment, the effect goes well.%目的 探讨婴幼儿先心病术后合并纵隔气肿的常见病因、诊断和治疗方法.方法回顾性分析作者于2011年1月至2012年2月收治的9例婴幼儿心内直视手术后并发纵隔气肿患儿临床资料.结果 本组患儿中5例痊愈,3例治疗效果欠佳,不能维持循环功能,家属放弃治疗,1例因严重感染未得到有效控制而死亡.结论 小儿先心病术后合并纵隔气肿,对心肺功能影响大,并可并发纵膈感染,临床表现多样,易发生误诊或漏诊,死亡率高,如能早期诊断,及时治疗,效果良好.

  7. Clinical evaluation of advancement flap surgical technique for congenital concealed penis%渐进性皮瓣转移技术治疗先天性隐匿阴茎

    Institute of Scientific and Technical Information of China (English)

    陈杰; 徐卯升; 耿红全; 徐国锋; 咸华

    2009-01-01

    目的 通过总结103例先天性隐匿阴茎的临床资料,探讨渐进性皮瓣转移技术应用于先天性隐匿阴茎的诊治经验.方法 2006年7月至2008年9月,采用渐进性皮瓣转移技术治疗先天性隐匿阴茎患儿103例,年龄为6个月至12.3岁,所有病例均符合先天性隐匿阴茎的诊断,其中3例伴有明显肥胖.结果平均随访1.5年(6个月至2.5年).所有患儿阴茎外观均较满意,阴茎显露好,无包皮臃肿现象.术后1例冈阴茎根部皮肤附着不佳仍有同缩,2例患儿在阴茎根部切口处轻度裂开.结论 渐进性皮瓣转移技术治疗先天性隐匿阴茎,术后阴茎外观接近包茎术后表现,术后再回缩等并发症少.渐进性皮瓣转移技术可以作为治疗先天性隐匿阴茎的常规术式.%Objective To summarize the clinical data of 103 children with congenital concealed penis treated with advancement flap surgical technique. Methods From July 2006 to September 2008, 103 children with congenital concealed penis underwent penial plasty using advancement flap surgical technique. The patients aged from 6 months to 12. 3 years. Three patients were extremely obese. Resuits The mean follow-up period was 1.5 years. All patients gained good postoperative cosmetic appearance with satisfactory exposure of penis and no malformation of prepuce. Only one patient underwent a reoperation for recurrent retraction caused by unstable attachment at the radix of penis. Two patients suffered slight disruption of wound at the radix of penis. Conclusions Flap surgical technique can provide immediate and excellent cosmetic results with a low incidence of complications. The advancement flap surgical technique can be adopted as a regular procedure for treating children with congenital concealed penis.

  8. 小儿先天性双肾积水35例临床分析%Bilateral congenital hydronephrosis in children: clinical analysis of 35 cases

    Institute of Scientific and Technical Information of China (English)

    张恒; 卢根生; 李新; 沈文浩; 熊恩庆; 方强; 宋波; 周占松

    2013-01-01

    Objective To explore the mechanism and therapy of bilateral congenital hydronephrosis in children. Methods The medical history, radiographic studies, urodynamic results and therapies of 35 children were analyzed retrospectively. Results Among the 35 patients with bilateral hydronephrosis, 32 cases were lower urinary tract dysfunction and 3 cases were upper urinary tract obstruction, residual u-rine volume of 25 cases exceed 100 mL and bladder storage pressure of 28 cases were more than 40 cmH2O. The 16 cases with posterior ure-thral valve accepted valvectomy, 10 cases with neurogenic bladder treated by self-cleaning catheterization and sacral nerve stimulation,4 cases accepted colliculectomy, 2 cases with phimosis accepted circumcision, 1 case with bilateral ureteropelvic junction stenosis accepted ureteropel-vic stricture plasty,2 cases with bilateral lower ureteral obstruction received ureterovesical reimplantation. All patients were followed up for more than six months and found hydronephrosis significantly alleviated. Conclusion Vesical pressure over safe pressure result from lower u-rinary tract voiding dysfunction is the most common reason of bilateral congenital hydronephrosis in children. To depress the vesical pressure until lower than the safe pressure in time is crucial for the treatment of patients with bilateral congenital hydronephrosis.%目的 探讨小儿先天性双肾积水的发病机制及治疗策略.方法 回顾分析35例小儿先天性双肾积水的尿动力学、泌尿影像、肾脏功能检查及外科治疗的临床资料.结果 下尿路排尿功能障碍32例,上尿路梗阻3例;25例残余尿量超过100mL,28例膀胱储尿期压力大于40 cmH2O.16例行后尿道瓣膜切除术;10例神经源性膀胱行自身清洁导尿及骶神经电刺激治疗,4例行精阜切除术,2例行包皮环切术,1例双侧肾盂输尿管交界部狭窄行离断成形术,2例行输尿管膀胱再植术.术后随访6个月以上,双肾

  9. Pathophysiology, clinical manifestation and management of angioedema - our experience

    Directory of Open Access Journals (Sweden)

    Aleksić Aleksandra

    2015-01-01

    Full Text Available Introduction. Angioedema is characterized by subcutaneous and/or submucosal swelling usually localized to the lips, eyelids, tongue, oral cavity, larynx and pharynx. Various types of angioedema, caused by different pathophysiologic mechanisms, can have the same or very similar clinical picture and require different diagnostic and therapeutic procedures. The immediate threat to life as a result of rapidly developed edema of the pharynx and larynx with airway obstruction requires endotracheal intubation or emergency tracheotomy. Standard therapy, which includes epinephrine, second-generation antihistamines and steroids, is not effective in the treatment of all types of angioedema. Objective. On the basis of the clinical presentation and course of angioedema, this retrospective study was aimed at contributing to a better understanding of the etiopathogenesis of the disease and at helping determine the most effective available treatment modalities. Methods. This retrospective study included patients treated under the diagnosis of angioedema of the upper aerodigestive tract between 2000 and 2012 in the Department of Otorhinolaryngology, Clinical Center of Banja Luka. Results. A total of 76 subjects were included in the study. The average age was 62.8 years. There were 40 (52.6% male and 36 (47.4% female patients. The largest number of patients (44.7% had type II angioedema. Almost half of the patients or 36 patients (47.4% were on treatment with an angiotensinconverting enzyme inhibitor (ACEi, but there was no statistically significant difference under the total number of patients (p=0.678. Conclusion. Better understanding of pathophysiologic mechanisms and the adoption of diagnostic protocols contributes to more effective treatment of angioedema.

  10. Clinical spectrum of hypopituitarism in India: A single center experience

    Directory of Open Access Journals (Sweden)

    Abhay Gundgurthi

    2012-01-01

    Full Text Available Objectives: There is paucity of information regarding clinical profile of hypopituitarism from India. We report the clinical profile of hypopituitarism from a tertiary center in North India. Materials and Methods: This study was carried out in patients attending our endocrine center between January 2010 and December 2011. All new patients were studied prospectively and those registered before January 2010 retrospectively. Relevant clinical, hormonal, and imaging data were collected. Dynamic testing for pituitary functions was carried out as necessary. Hormonal deficiencies were defined as per prevailing recommendations. Results: This study included 113 subjects. The mean age was 38.6 ± 17.8 years (range, 4 - 76 years. There were 78 (69% males and 35 females (31%. There were 22 subjects aged ≤18 years (childhood and adolescence and 91 adults (>18 years. Visual disturbances were the most common presenting complaint (33%, though headache was the most common symptom (81%. Fifteen percent presented with pituitary apoplexy. Tumors comprised of 84% of cases. Hypogonadism (97% was the most common abnormality seen followed by hypothyroidism (83.2%, hypoadrenalism (79.6%, growth hormone deficiency (88.1% of the 42 patients tested, and diabetes insipidus (13.3%. Panhypopituitarism was seen in 104 (92% patients. There were no cases of hypopituitarism secondary to traumatic brain injury, subarachnoid hemorrhage, central nervous system infections, or cranial irradiation to extrasellar tumors. Conclusion: The most common cause of hypopituitarism at tertiary care center is pituitary tumors and the commonest presenting complaint is visual symptoms. Panhypopituitarism is present in 92% cases.

  11. Antiarrhythmic treatment with flecainide (Tambocor). Clinical experience from 107 patients

    DEFF Research Database (Denmark)

    Frandsen, F; Pless, P; Mickley, H;

    1990-01-01

    The long-term clinical effect of oral flecainide treatment was evaluated in 107 pts (10-82 yrs). Indications for treatment were: atrial fibrillation 38%, atrial flutter 16%, ventricular tachycardia 24%, ventricular ectopic beats 10% and supraventricular tachycardia 12%. Daily flecainide dosage...... pts due to: insufficient effect in 28, side effects in 17 and for other reasons in 5. The side effects indicating flecainide withdrawal (pts) were: cerebral symptoms (4), gastrointestinal complaints (2), bradyarrhythmias (2), heart failure (3) and suspected pro-arrhythmia (4). (Ventricular tachycardia...

  12. Lokomat: Clinical training and experience in a neurorehabilitation hospital

    DEFF Research Database (Denmark)

    Riberholt, Christian Gunge

    2014-01-01

    This presentation aims to give insight into the daily work of walking rehabilitation of patients with severe acquired brain injury (ABI) using the Lokomat© system. The lokomat system offers a high number of repetitions (steps) pr. training session with less physical stress on therapists compared to...... literature lack randomized controlled trials in ABI. Furthermore few trials have specifically investigated the most optimal training strategy for different groups of neurological patients This presentation aims at highlighting some of the strategies and clinical challenges using an evidence-based approach to...

  13. 乙状结肠代阴道成形术治疗先天性无阴道的疗效分析%Clinical effect of sigmoid colon vaginoplasty in treatment of congenital absence of vagina

    Institute of Scientific and Technical Information of China (English)

    唐素蓉; 藤青芳; 张晓丹

    2014-01-01

    Objective To discuss the clinical effect of sigmoid colon vaginoplasty in the treatment of patients with congenital absence of vagina. Methods 4 patients with the congenital absence of vagina treated in this hospital from February 2011 to August 2013 received the treatment of sigmoid colon vaginoplasty. Their average operation time, intraoperative hematoma volume, postoperative complications and postoperative vaginal structure and function were observed. Results 4 patients with the congenital absence of vagina who received the sigmoid colon vaginoplasty were all successful in the operation, with the average operation time of (140.5±26.5)min and average hematoma volume of (118.4±4.5)mL. The vagina formed by the sigmoid colon surgery was close to the normal one in the shape and patients were satisfied with the sex life without any intraoperative or postoperative complication. Conclusion After receiving the sigmoid colon vaginoplasty, patients with the congenital absence of vagina have their operation-formed vaginas basically similar to normal ones in the structure and function. It is characterized by the safe operation, short operation time, limited hematoma volume and little complications, which is worthy to be popularized in the clinical practice.%目的:研究探讨应用乙状结肠代阴道成形术治疗先天性无阴道患者的临床疗效。方法对我院2011年2月~2013年8月收治的先天性无阴道患者4例进行分析,采用乙状结肠代阴道成形术治疗,观察4例采用乙状结肠代阴道成形术的平均手术时间、手术时出血量、手术后的并发症以及手术后阴道的结构和功能的情况。结果采用乙状结肠代阴道成形术治疗的4例先天性无阴道患者手术均获得成功,而且手术的平均时间为(140.5±26.5)min,平均出血量为(118.4±4.5)mL。用乙状结肠手术成的阴道在形态上与正常女性阴道接近,并且患者性生活感觉满意,没有发生手

  14. Congenital Cataract Screening.

    Science.gov (United States)

    Rajavi, Zhale; Sabbaghi, Hamideh

    2016-01-01

    Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year) is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender. PMID:27621790

  15. Congenital tracheobronchial stenosis.

    Science.gov (United States)

    Hewitt, Richard J; Butler, Colin R; Maughan, Elizabeth F; Elliott, Martin J

    2016-06-01

    Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Successful management of this complex condition requires a highly individualized approach delivered by an experienced multidisciplinary team, which is best delivered within centralized units with the necessary diverse expertise. In such settings, surgical correction by slide tracheoplasty has become increasingly successful over the past 2 decades such that long-term survival now exceeds 88%, with normalization of quality of life scores for patients with non-syndrome-associated congenital tracheal stenosis. Careful assessment and planning of treatment strategies is of paramount importance for both successful management and the provision of patients and carers with accurate and realistic treatment counseling. PMID:27301600

  16. Congenital cataract screening

    Directory of Open Access Journals (Sweden)

    Zhale Rajavi

    2016-01-01

    Full Text Available Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  17. Congenital Cataract Screening

    Science.gov (United States)

    Rajavi, Zhale; Sabbaghi, Hamideh

    2016-01-01

    Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year) is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender. PMID:27621790

  18. Conducting Family Nursing in Heart Failure outpatient clinics: Nurses experiences

    DEFF Research Database (Denmark)

    Voltelen, Barbara; Konradsen, Hanne; Østergaard, Birte

    Aim: This study aimed to explore what was documented during structured Family Nursing (FN) conversations with patients diagnosed with Heart Failure and their families, and to gain knowledge about the nurses’ experiences conducting FN. Background: Patients with HF face many challenges, and so do...... throughout the FN intervention and a Focus group interview with 6 nurses who were conducting the conversations. Content analyses of all text material dealt with both manifest and latent content, and were analyzed through a deductive and inductive process. Results: Enabling bonding emerged as the overall...... theme for the FN conversations, and was made possible through two subthemes; “strengthening family bonds” and “creating an enabling context for FN”. Conducting the FN conversations was challenging due to the use of new communication skills. Optimal setting, experience, retraining and professional...

  19. Clinical experiences in fungal keratitis caused by Acremonium

    Directory of Open Access Journals (Sweden)

    Kim SJ

    2014-01-01

    Full Text Available Seong-Jae Kim,1,2 Yong-Wun Cho,1 Seong-Wook Seo,1,2 Sun-Joo Kim,2,3 Ji-Myong Yoo1,21Department of Ophthalmology, 2Gyeongsang Institute of Health Science, 3Department of Laboratory Medicine, Gyeongsang National University, College of Medicine, Jinju, KoreaPurpose: To report the predisposing risk factors, clinical presentation, management, and therapeutic outcomes of fungal keratitis caused by Acremonium.Methods: This is a retrospective study of cases with Acremonium fungal keratitis that presented to our tertiary referral center between January 2006 and August 2012. Patient demographic and clinical details were determined and reported.Results: Five cases of fungal keratitis from Acremonium species were identified in five patients (three males, two females. The mean age of the patients was of 73.4±5.46 years, with a mean follow-up time of 124±72 days. All patients had a history of corneal trauma with vegetable matter. Four cases were unresponsive to initial treatment (0.2% fluconazole, 0.15% amphotericin B and required topical 5% natamycin, and, in two out of five cases, topical 1% voriconazole.Conclusion: The most common risk factors for Acremonium fungal keratitis was ocular trauma. When a corneal lesion is found to be unresponsive to the initial treatment, we should consider adding or substituting topical natamycin or voriconazole for treatment.Keywords: Acremonium, fungal keratitis, natamycin, prognosis, voriconazole

  20. Nursing experience in clinical endovascular treatment for renal artery aneurysms

    International Nuclear Information System (INIS)

    Objective: To discuss the standardized clinical nursing measures for patients receiving endovascular treatment of renal artery aneurysms. Methods: The clinical data of 9 patients with renal artery aneurysm, who were admitted to authors' hospital during the period from Jan. 2010 to Aug. 2011 and received endovascular treatment, were retrospectively analyzed. The related nursing points as well as the received endovascular. Results: A total of 9 cases with renal artery aneurysm were treated nursing measures were summarized. Results: A total of 9 cases with renal artery aneurysm were with interventional management, including embolization (n = 6), stent implantation (n = 2) and stent implantation together with coil embolization (n = 1). The mean hospitalization time was (10±2) days. Postoperative retention of urine was observed in one patient and postoperative retroperitoneal hemorrhage occurred in another patient. Neither nursing-related nor operation-related complications occurred. Conclusion: Standardized perioperative nursing care for patients with renal artery aneurysm can surely help enhance the patient's tolerance to the surgery, and effectively prevent the complications. (authors)

  1. [First clinical experiences with ceramic ball attachments for overdentures].

    Science.gov (United States)

    Büttel, Adrian E; Schmidli, Fredy; Marinello, Carlo P; Lüthy, Heinz

    2008-01-01

    In this prospective clinical study on 40 patients with similar clinical conditions (edentulous jaw with 2 interforaminal implants) commercially available ceramic ball attachments (ruby) were compared to commercial titanium ball attachments. The primary aim of the study was to measure the wear of the ball attachments after being 1 year in function. However, in the course of the study already after 7 to 12 months multiple failures with ceramic ball attachments occurred. Twelve (28%) of 43 ceramic ball attachments had to be replaced, mostly because of fractures (8) of the ceramic ball. It seems that ceramic ball attachments of the investigated design are not able to withstand normal intraoral stresses. The short-term susceptibility to fractures didn't allow to examine the ceramic-inherent features such as compressive strength and wear resistance. Furthermore, a secure connection between a titan base and a ceramic ball seems to be challenging. Based on these results, in implant-retained removable prosthesis the use of metal-based retainers is still recommended, although during maintenance a higher wear has to be expected. This wear can be compensated by either activating or changing the matrix or the patrix. PMID:18293602

  2. Translating comparative effectiveness research into clinical practice: the UK experience.

    Science.gov (United States)

    Walley, Tom

    2012-01-22

    Comparative effectiveness research (CER) is not new but its potential to improve the effectiveness of healthcare has not yet been exploited in the US. Other countries such as the UK have more experience of this. Key points of the UK experience are summarized here and some possible pointers for the US are drawn. These include the following: how to go beyond the evidence and apply judgements to make recommendations with authority and in a timely manner; how to implement these recommendations; how to identify suitable topics; and how to be open and transparently fair to all stakeholders. The quality of the science of CER is key but this needs developing, and not just in biomedical or statistical terms but also in how to understand public expectations, and how to implement its recommendations. A key issue is the role of health economics, which seems to have been marginalized by the CER legislation, but perhaps this is more apparent than real. Clearly this is a matter for much further debate. It is hard to see how CER can deliver its potential without active consideration of both benefits and costs. Although other countries have more experience of this than does the US, the context for such work is always very specific and the US will have to find its own way, while trying to avoid some of the errors made elsewhere. PMID:22268389

  3. Congenital laryngeal anomalies,

    Directory of Open Access Journals (Sweden)

    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  4. Congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    1995-01-01

    950283 Surgical treatment of congenital coronaryartery fistula.CAO Qingheng(曹庆亨),et al.DeptCardiovasc Surg,Shanghai Chest Hosp,Shanghai,200030.Shanghai Med J 1995;18(1):10-12.From October 1957 through December 1990,twenty-five patients with congenital coronary artery fistula(CCAF),including 3 cases complicated with giantcoronary artery aneurysms,underwent surgical repair.The ages ranged from 4 to 47 years (mean 19.8years).CCAF originated from the right coronaryartery in 17 cases (68.0%) and terminated into RA,RV,pulmonary artery (PA) or LV,in 8 cases (32.

  5. Congenital spinal malformations

    International Nuclear Information System (INIS)

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.)

  6. Congenital preduodenal portal vein

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang Jin; Hwang, Mi Soo; Huh, Young Soo; Park, Bok Hwan [College of Medicine, Youngnam University, Gyeongsan (Korea, Republic of)

    1991-03-15

    Congenital preduodenal portal vein, first reported by Knight in 1921, is an extremely rare congenital anomaly in which the portal vein passes anteriorly to the duodenum rather than posteriorly in its normal location. It is of surgical significance because it may cause difficulties in operations involving the gall bladder, biliary duct, or duodenum. Recently, we experienced 2 cases of preduodenal portal vein. One was found during surgical exploration for the diagnosis and correction of malrotation of the bowels and the other in a 3 day-old male newborn associated with dextrocardia, situs inversus, and duodenal obstruction by diaphragm. We report these 2 cases with a review of the literature.

  7. Clinical Experience in Acupuncture Treatment of Allergic Rhinitis

    Institute of Scientific and Technical Information of China (English)

    ZHANG Yan-qiu

    2009-01-01

    Objective:To observe the clinical effects of acupuncture for allergic rhinitis.Methods:The body acupuncture, auricular seed-embedding and microwave irradiation were adopted for treatment of allergic rhinitis due to various causative factors, such as cold and insufficiency of the lung-qi weakening the body resistance, insufficiency of the spleen-qi with lucid yang failing to rise, insufficiency of the kidney-yang failing to warm the body surface, and the heat accumulated in the lung channels giving invading the nose.Results:After treatment, the symptoms and signs disappeared in all illustrative cases, with no recurrence found after a one-year follow-up.Conclusion:Acupuncture may help to improve the blood rheology indexes with an increased volume of blood flow, and regulate the immunological function of the human body, thus giving therapeutic effects for allergic rhinitis.

  8. Clinical Experience in Application of the Point Wangu

    Institute of Scientific and Technical Information of China (English)

    杨西永

    2002-01-01

    Wangu (GB 12) is a point of the Gallbladder Channel of Foot Shaoyang, located in the depression posteroinferior to the temporal process. And it is a hui point of Foot Taiyang and Shaoyang Channels, with the effects of eliminating pathogenic wind and heat, and tranquilizing the mind. This point is located superior to the attachment point of the sternocleidomastoid muscle, in which there distribute the stem of the lesser occipital nerve, and branches of the posterior auricular artery and vein. It can be needled perpendicularly 0.5-1 cun deep. In clinical practice, the author has applied the point for treatment of various diseases, and obtained quite satisfactory therapeutic results. The following is a report of some illustrative cases.

  9. Selecting clinical diagnoses: logical strategies informed by experience.

    Science.gov (United States)

    Stanley, Donald Edward; Campos, Daniel G

    2016-08-01

    This article describes reasoning strategies used by clinicians in different diagnostic circumstances and how these modes of inquiry may allow further insight into the evaluation and treatment of patients. Specifically, it aims to make explicit the implicit logical considerations that guide a variety of strategies in the diagnostic process, as exemplified in specific clinical cases. It focuses, in particular, in strategies that clinicians use to move from a large set of possible diagnoses initially suggested by abductive inferences - the process of hypothesis generation that creates a diagnostic space - to a narrower set or even to a single 'best' diagnosis, where the criteria to determine what is 'best' may differ according to different strategies. Experienced clinicians should have a diversified kit of strategies - for example, Bayesian probability or inference to a lovely explanation - to select from among previously generated hypotheses, rather than rely on any one approach every time.

  10. Experience in the Clinical Application of Naokong (GB19)

    Institute of Scientific and Technical Information of China (English)

    陆汎

    2005-01-01

    @@ Naokong (GB19) is located 1.5 cun above Fengchi (GB20) and at the level with Naohu (GV17) in the depression of the lateral side of the external occipital protuberance. It belongs to the Gallbladder Channel of Foot Shaoyang, and is a crossing point of the Foot Shaoyang Channel and the Yangwei Channel. As is said in A Collection of Gems in Acupuncture and Moxibustion, Naokong (GB19) is indicated for emaciation due to over strain and stress, fever, neck rigidity, unbearable pain in the head, heavy eyes and palpitation as well as xenophthalmia and rhinalgia caused by epileptic seizure in severe cases. In clinical practice, the author has adopted Naokong (GB 19) as the main point in treating some obstinate diseases and obtained quite good therapeutic effects.

  11. CLINICAL AND MICROBIOLOGICAL STUDY OF OTOMYCOSIS : OUR EXPERIENCE

    Directory of Open Access Journals (Sweden)

    Venugopal

    2015-06-01

    Full Text Available Otomycosis is fungal infection of external auditory canal, which is common throughout the world. Otomycosis may be refractory to treatment prescribed and hence challenges the clinician. AIM: To identify the organism causing otomycosis and to explore speci fic predisposing factors and treatment. MATERIALS AND METHODS: This is a descriptive study of cases of otomycosis attending ENT outpatient department of Govt. Medical College, Thiruvananthapuram which were evaluated clinically and microbiologically for one year. RESULT AND CONCLUSION: The commonest predisposing factor for otomycosis is trauma due to self - cleaning and water entering the ear. Common in females. Common presentation is itching of ear and ear ache. Commonest organism isolated is Aspergillus Nige r. Usually respond to treatment with topical antifungal agent especially clotrimazole. Most of the cases responded satisfactorily with topical clotrimazole.

  12. [Subantral augmentation with porous titanium in experiment and clinic].

    Science.gov (United States)

    Sirak, S V; Shchetinin, E V; Sletov, A A

    2016-01-01

    The article discusses the use of porous titanium for subantral augmentation. Experimental study was conducted on 12 yearling rams. Subantral augmentation using porous titanium was performed in 33 patients. In the control group consisting of 14 patients calcium phosphates and bone collagen based agents ("Bio-Оss" and "Collost") were used. In the main and control groups 46 and 32 implant were placed, respectively. Pilot histological and clinical studies proved that the granules of porous titanium are biocompatible with bone tissue, provide the optimal surface microrelief, thus creating good conditions for adhesion, expansion and migration of osteoforming cells, have negligible kinetics of resorption, are porous to ensure effective neovascularization of de novo formed bone tissue. Porous titanium is an effective alternative material for subantral bone augmentation for dental implantation and reconstructive operations on the maxillary sinus.

  13. [Subantral augmentation with porous titanium in experiment and clinic].

    Science.gov (United States)

    Sirak, S V; Shchetinin, E V; Sletov, A A

    2016-01-01

    The article discusses the use of porous titanium for subantral augmentation. Experimental study was conducted on 12 yearling rams. Subantral augmentation using porous titanium was performed in 33 patients. In the control group consisting of 14 patients calcium phosphates and bone collagen based agents ("Bio-Оss" and "Collost") were used. In the main and control groups 46 and 32 implant were placed, respectively. Pilot histological and clinical studies proved that the granules of porous titanium are biocompatible with bone tissue, provide the optimal surface microrelief, thus creating good conditions for adhesion, expansion and migration of osteoforming cells, have negligible kinetics of resorption, are porous to ensure effective neovascularization of de novo formed bone tissue. Porous titanium is an effective alternative material for subantral bone augmentation for dental implantation and reconstructive operations on the maxillary sinus. PMID:26925568

  14. Congenital granular cell lesion: A rare tumor of new born

    Directory of Open Access Journals (Sweden)

    Rajesh Kumar

    2013-01-01

    Full Text Available Congenital granular cell tumor (CGCT, or congenital epulis, is an uncommon benign soft tissue lesion that usually arises from the alveolar mucosa of neonates and may cause respiratory and feeding problems. The histogenesis and clinical history of the lesion remains obscure. Treatment involves surgical excision and recurrences are rare. The present report describes a case of congenital granular cell lesion (CGCL in the anterior segment of maxillary alveolar ridge of a 2-month-old female. This lesion was causing feeding problem and was excised under local anesthesia, with no recurrence even after 3 years.

  15. Congenital anomalies of the coronary sinus: A pictorial essay

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jung Eun; Kwon, Se Hwan; Oh, Joo Hyeong [Dept. of Radiology, College of Medicine, Kyung Hee University, Seoul (Korea, Republic of)

    2013-07-15

    Congenital anomalies involving the coronary sinus (CS) tend to receive relatively little attention because they rarely cause clinical symptoms or disturbances of cardiac function. However, as imaging modalities have been developed over time, the detailed anatomy of the heart, including CS anomalies, can now be evaluated more precisely. The purpose of this pictorial review is to illustrate multi-detector computed tomography findings of various congenital anomalies of the CS. The cardiac venous system and its embryologic development are also described in detail to familiarize radiologists with various congenital anomalies of the CS.

  16. Congenital anomalies of the coronary sinus: A pictorial essay

    International Nuclear Information System (INIS)

    Congenital anomalies involving the coronary sinus (CS) tend to receive relatively little attention because they rarely cause clinical symptoms or disturbances of cardiac function. However, as imaging modalities have been developed over time, the detailed anatomy of the heart, including CS anomalies, can now be evaluated more precisely. The purpose of this pictorial review is to illustrate multi-detector computed tomography findings of various congenital anomalies of the CS. The cardiac venous system and its embryologic development are also described in detail to familiarize radiologists with various congenital anomalies of the CS.

  17. The Clinical Effect of Rehabilitation Education on 48 Cases of Congenital Down Children%康复教育对48例先天愚型儿童的临床影响

    Institute of Scientific and Technical Information of China (English)

    安春梅

    2015-01-01

    ObjectiveTo investigate the clinical effect of rehabilitation education on the children gotten type down .Methods48 cases children with congenital down selected as the research group; the control group was 48 cases which were the same disease at the period. The research group were recieved rehabilitation education but Control group weren’t, compared the social life of two groups .ResultsThe children of control group without health education in motor, cognitive, ifne motor, speech and so on social adaptation ability is lower than the research group, two groups of data was difference (P<0.05). Conclusion The rehabilitation education for children congenital down, it can effectively improve their cognitive, language, sports, social adaptability and ifne motor skills, clinical effect is remarkable, and rehabilitation education has clinical value.%目的:探讨康复教育对先天愚型儿童的临床影响。方法选取先天愚型患儿48例为研究对象,设为研究组;同期选取同症患儿48例作为参照对象,设为对照组;对照组并未进行康复教育,研究组进行康复教育,对比两组社会生活能力。结果未进行健康教育的对照组儿童在运动、认知、精细运动、言语及社会适应能力等方面均低于研究组,两组数据对比具有差异性(P<0.05)。结论康复教育对于先天愚型儿童而言,其能够有效改善其认知、语言、运动、社会适应能力和精细动作等能力,临床效果显著。

  18. Experience with the Implementation of Clinical Pharmacy Services and Processes in a University Hospital in Belgium.

    Science.gov (United States)

    Somers, Annemie; Claus, Barbara; Vandewoude, Koen; Petrovic, Mirko

    2016-03-01

    This article summarizes the experience with the development of clinical pharmacy services in the Ghent University Hospital in Belgium. Implementation of clinical pharmacy services in Belgian hospitals has not been evident because these activities were initially not structurally financed. The aim is to describe the strengths and weaknesses of the clinical pharmacy development process, and the milestones that enhanced the progress. Furthermore, the organisation of clinical pharmacy in the Ghent University Hospital is explained, including back- and front-office activities, seamless pharmaceutical care and medication safety improvement. Some working methods, procedures and tools are explained for different clinical pharmacy services. In particular, the clinical pharmacy projects for geriatric patients as well as the preparation of clinical pharmacy services for the accreditation process are explained. We also reflect on the organisation model and the future development of clinical pharmacy, taking into consideration facilitators and potential barriers. PMID:26922733

  19. Experience with the Implementation of Clinical Pharmacy Services and Processes in a University Hospital in Belgium.

    Science.gov (United States)

    Somers, Annemie; Claus, Barbara; Vandewoude, Koen; Petrovic, Mirko

    2016-03-01

    This article summarizes the experience with the development of clinical pharmacy services in the Ghent University Hospital in Belgium. Implementation of clinical pharmacy services in Belgian hospitals has not been evident because these activities were initially not structurally financed. The aim is to describe the strengths and weaknesses of the clinical pharmacy development process, and the milestones that enhanced the progress. Furthermore, the organisation of clinical pharmacy in the Ghent University Hospital is explained, including back- and front-office activities, seamless pharmaceutical care and medication safety improvement. Some working methods, procedures and tools are explained for different clinical pharmacy services. In particular, the clinical pharmacy projects for geriatric patients as well as the preparation of clinical pharmacy services for the accreditation process are explained. We also reflect on the organisation model and the future development of clinical pharmacy, taking into consideration facilitators and potential barriers.

  20. EPID based in vivo dosimetry system: clinical experience and results.

    Science.gov (United States)

    Celi, Sofia; Costa, Emilie; Wessels, Claas; Mazal, Alejandro; Fourquet, Alain; Francois, Pascal

    2016-01-01

    Mandatory in several countries, in vivo dosimetry has been recognized as one of the next milestones in radiation oncology. Our department has implemented clinically an EPID based in vivo dosimetry system, EPIgray, by DOSISOFT S.A., since 2006. An analysis of the measurements per linac and energy over a two-year period was performed, which included a more detailed examination per technique and treat-ment site over a six-month period. A comparison of the treatment planning system doses and the doses estimated by EPIgray shows a mean of the differences of 1.9% (± 5.2%) for the two-year period. The 3D conformal treatment plans had a mean dose difference of 2.0% (± 4.9%), while for intensity-modulated radiotherapy and volumetric-modulated arc therapy treatments the mean dose difference was -3.0 (± 5.3%) and -2.5 (± 5.2%), respectively. In addition, root cause analyses were conducted on the in vivo dosimetry measurements of two breast cancer treatment techniques, as well as prostate treatments with intensity-modulated radiotherapy and volumetric-modulated arc therapy. During the breast study, the dose differences of breast treatments in supine position were correlated to patient setup and EPID positioning errors. Based on these observations, an automatic image shift correc-tion algorithm is developed by DOSIsoft S.A. The prostate study revealed that beams and arcs with out-of-tolerance in vivo dosimetry results tend to have more complex modulation and a lower exposure of the points of interest. The statistical studies indicate that in vivo dosimetry with EPIgray has been successfully imple-mented for classical and complex techniques in clinical routine at our institution. The additional breast and prostate studies exhibit the prospects of EPIgray as an easy supplementary quality assurance tool. The validation, the automatization, and the reduction of false-positive results represent an important step toward adaptive radiotherapy with EPIgray. PMID:27167283

  1. Clinical spectrum and outcome of pulmonary nocardiosis: 5-year experience

    Science.gov (United States)

    Singh, Akashdeep; Chhina, Deepinder; Soni, RK; Kakkar, Chandan; Sidhu, US

    2016-01-01

    Background: Pulmonary nocardiosis is a rare but a life-threatening infection caused by Nocardia spp. The diagnosis is often missed and delayed resulting in delay in appropriate treatment and thus higher mortality. Aim: In this study, we aim to evaluate the clinical spectrum and outcome of patients with pulmonary nocardiosis. Methods: A retrospective, 5-year (2009–2014) review of demographic profile, risk factors, clinical manifestations, imaging findings, treatment, and outcome of patients with pulmonary nocardiosis admitted to a tertiary care hospital. Results: The median age of the study subjects was 54 years (range, 16–76) and majority of them (75%) were males. The risk factors for pulmonary nocardiosis identified in our study were long-term steroid use (55.6%), chronic lung disease (52.8%), diabetes (27.8%), and solid-organ transplantation (22.2%). All the patients were symptomatic, and the most common symptoms were cough (91.7%), fever (78%), and expectoration (72%). Almost two-third of the patients were initially misdiagnosed and the alternative diagnosis included pulmonary tuberculosis (n = 7), community-acquired pneumonia (n = 5), lung abscess (n = 4), invasive fungal infection (n = 3), lung cancer (n = 2), and Wegener's granulomatosis (n = 2). The most common radiographic features were consolidation (77.8%) and nodules (56%). The mortality rate for indoor patients was 33% despite treatment. Higher mortality rate was observed among those who had brain abscess (100.0%), HIV positivity (100%), need for mechanical ventilation (87.5%), solid-organ transplantation (50%), and elderly (age > 60 years) patients (43%). Conclusion: The diagnosis of pulmonary nocardiosis is often missed and delayed resulting in delay in appropriate treatment and thus high mortality. A lower threshold for diagnosing pulmonary nocardiosis needs to be exercised, in chest symptomatic patients with underlying chronic lung diseases or systemic immunosuppression, for the early diagnosis

  2. Student Pharmacists’ Clinical Interventions in Advanced Pharmacy Practice Experiences at a Community Nonteaching Hospital

    OpenAIRE

    Shogbon, Angela O.; Lundquist, Lisa M.

    2014-01-01

    Objective. To assess student pharmacists’ clinical interventions in advanced pharmacy practice experiences (APPEs) at a community nonteaching hospital and evaluate completed interventions based on the type of documentation method used.

  3. 42 CFR 482.80 - Condition of participation: Data submission, clinical experience, and outcome requirements for...

    Science.gov (United States)

    2010-10-01

    ... center. (3) A pancreas transplant center is not required to comply with the clinical experience... pancreas transplants performed at the center. (4) A center that is requesting initial Medicare approval...

  4. The significance of clinical experience on learning outcome from resuscitation training-a randomised controlled study

    DEFF Research Database (Denmark)

    Jensen, Morten Lind; Lippert, Freddy; Hesselfeldt, Rasmus;

    2008-01-01

    CONTEXT: The impact of clinical experience on learning outcome from a resuscitation course has not been systematically investigated. AIM: To determine whether half a year of clinical experience before participation in an Advanced Life Support (ALS) course increases the immediate learning outcome ...... but statistically significant impact on the retention of learning, but not on the immediate learning outcome Udgivelsesdato: 2008/12/4......CONTEXT: The impact of clinical experience on learning outcome from a resuscitation course has not been systematically investigated. AIM: To determine whether half a year of clinical experience before participation in an Advanced Life Support (ALS) course increases the immediate learning outcome...... and retention of learning. MATERIALS AND METHODS: This was a prospective single blinded randomised controlled study of the learning outcome from a standard ALS course on a volunteer sample of the entire cohort of newly graduated doctors from Copenhagen University. The outcome measurement was ALS...

  5. Minimally invasive transforaminal lumbar interbody fusion—indications and clinical experience

    Directory of Open Access Journals (Sweden)

    Akshay Hari

    2016-01-01

    Conclusions: Our clinical experience along with a review of the medical literature indicates that TLIF can be effectively and safely performed in a minimally invasive fashion for a wide variety of indications.

  6. Summary on Clinical Experience of Acupuncture Treating Dry Eye Syndromes

    Institute of Scientific and Technical Information of China (English)

    马晓芃; 杨玲; 莫文权; 施征; 赵粹英

    2009-01-01

    @@ Dry eye syndromes(DES)refer to discomfort in the eye region,visual disturbance,poor stability of lacrimal film,inflammation of eve surface and its potential iniury due to many factors of abnonnal lacrimation[1].Patients with this condition can experience a dry sensation in the eyes,foreign body sensation,burning and itching sensation in the eyes,and blurred vision.The lingering pathological change can lead to decreased transparency of the cornea and hypopsia,affecting the work,study and life,and even resulting in blindness.With popularization of computers,and change in lifestyles,the incidence of DES rises gradually and tends to occur in young age.

  7. [Brain-Computer Interface: the First Clinical Experience in Russia].

    Science.gov (United States)

    Mokienko, O A; Lyukmanov, R Kh; Chernikova, L A; Suponeva, N A; Piradov, M A; Frolov, A A

    2016-01-01

    Motor imagery is suggested to stimulate the same plastic mechanisms in the brain as a real movement. The brain-computer interface (BCI) controls motor imagery by converting EEG during this process into the commands for an external device. This article presents the results of two-stage study of the clinical use of non-invasive BCI in the rehabilitation of patients with severe hemiparesis caused by focal brain damage. It was found that the ability to control BCI did not depend on the duration of a disease, brain lesion localization and the degree of neurological deficit. The first step of the study involved 36 patients; it showed that the efficacy of rehabilitation was higher in the group with the use of BCI (the score on the Action Research Arm Test (ARAT) improved from 1 [0; 2] to 5 [0; 16] points, p = 0.012; no significant improvement was observed in control group). The second step of the study involved 19 patients; the complex BCI-exoskeleton (i.e. with the kinesthetic feedback) was used for motor imagery trainings. The improvement of the motor function of hands was proved by ARAT (the score improved from 2 [0; 37] to 4 [1; 45:5] points, p = 0.005) and Fugl-Meyer scale (from 72 [63; 110 ] to 79 [68; 115] points, p = 0.005). PMID:27188145

  8. [Cystinic nephrolythiasis: clinical experience and new diagnostic and therapeutic perspectives].

    Science.gov (United States)

    Gentili, Anna; Ria, Paolo; Lupo, Antonio; Fabris, Antonia

    2016-01-01

    Cystinuria is an inherited autosomal recessive disease with a prevalence 1:7000 and typical age of onset in the second decade of life. This nephrolithiasis is not always well known and well studied and for this reason it is often underdiagnosed. Cystinuria is characterized by increased urinary excretion of cystine and dibasic amino acids (lysine, ornithine, arginine) caused by defective transport of these amino acids across the luminal membrane of proximal tubule and small intestine cells. Two mutated genes responsible of this tubular defect are SLC3A1 on chromosome 2 and SLC7A9 on chromosome 19. Clinical manifestations of cystinuria are essentially those related to stones formation and their movement across the urinary tract, like flank pain/abdomen pain and hematuria, as occurred in other nephrolithiasis types. Diagnosis is based on biochemical urine analysis, stone analysis and imaging. Genetic study of this disease may be a new and stimulating approach to better understand the defects and identify new therapeutic targets. A wider knowledge and a more detailed approach to cystinuria may help to ameliorate patients quality of life, to prevent recurrences and complications and to develop more specific and adequate treatments. PMID:27374390

  9. CLINICAL STUDY AND MANAGEMENT OF INCISIONAL HERNIAS: OUR EXPERIENCE

    Directory of Open Access Journals (Sweden)

    Narayanaswamy

    2013-11-01

    Full Text Available ABSTRACT: BACKGROUND: Incisional hernia, by definition represents a breakdown or loss of continuity of a fascial closure. Surgical management of incision al hernias has evolved over the last century. This study was performed to review clinical profile and management of incisional hernia in our institute. AIMS AND OBJECTIVES: To analyze the etiopathogenesis of incisional hernia with respect to patient variab le factors, types of surgical intervention. MATERIALS AND METHODS: This a prospective study conducted at our institute between February 2009 and January 2011(24 months. 100 patients were included and followed up for immediate post - operative complications. OBSERVATIONS AND RESULTS: Incisional hernia was found to occur more often in 31 - 40yr age group,and mostly in females. Most commonly occurred following gynecological operations, lower abdominal incisions, post operative wound infection. Most patients notic ed the incisional hernia only 1 to 5 years after the index surgery. Laparoscopic hernioplasty was the most commonly performed surgery. CONCLUSION: In Incisional hernias the choice of operative technique is crucial Incisional hernias occur more often in fem ales as they are more likely to undergo lower abdominal surgeries. Mesh repair is considered superior to anatomical repair alone and we recommend Laparoscopic Hernioplasty as the first line of treatment. KEYWORDS : Incisional hernia, ventral hernia, post op erative hernia, mesh repair,Laparoscopic Hernioplasty

  10. Student service learning and dementia: bridging classroom and clinical experiences.

    Science.gov (United States)

    Corwin, Melinda; Owen, Donna; Perry, Carolyn

    2008-01-01

    University students in speech-language pathology and nursing were involved in a community service learning project with residents of a long-term care facility who had dementia. Nursing students were asked to interact and converse with residents to the best of their ability (control group, n = 28). Speech-language pathology students were instructed to design a personalized, multi-modality "connection kit" for residents based on the tenets of cognitive linguistic stimulation and facilitative styles of interaction, which were included within course content (experimental group, n = 25). Post-project surveys were administered in an attempt to answer the following research question: Do students perceive benefits from participating in a service learning project involving long-term care residents with dementia? Results revealed that the experimental/trained group of speech-language pathology students reported greater academic and clinical benefits compared to the control group of nursing students. Overall implications were that students benefited from a service learning project, especially when explicit instruction was provided.

  11. Clinical experience with apixaban in atrial fibrillation: implications of AVERROES

    Directory of Open Access Journals (Sweden)

    De Caterina R

    2011-07-01

    Full Text Available Raffaele De CaterinaInstitute of Cardiology and Center of Excellence on Aging, G d’Annunzio University, Chieti, G Monasterio Foundation, Pisa, ItalyAbstract: Atrial fibrillation is an extremely common arrhythmia, which substantially increases the risk of stroke and thromboembolism. Prevention of stroke and thromboembolism is therefore an important part of the management of atrial fibrillation. Guidelines until now have recommended that patients with atrial fibrillation receive some form of antithrombotic therapy, ie, a vitamin K antagonist or aspirin, with a preference for anticoagulants in most cases. However, current treatments are suboptimal, and despite the recommendations, many patients do not receive adequate thromboprophylaxis, because they are considered, for various reasons, “unsuitable” to receive a vitamin K antagonist. In this patient population, apixaban, a new oral anticoagulant inhibiting activated coagulation factor X, administered in fixed doses and without anticoagulation monitoring, has undergone testing against aspirin in the recently published AVERROES trial. This paper addresses the strengths and limitations of this trial and the practical relevance of the new clinical information it provides.Keywords: atrial fibrillation, apixaban, thromboprophylaxis 

  12. The First Experience of Clinical Practice on Psychology Students’ Imaginary

    Directory of Open Access Journals (Sweden)

    Sueli Regina Gallo-Belluzzo

    2013-09-01

    Full Text Available Considering the academic development of the psychologist as a complex process which articulates the transmission of scientific knowledge and changes in imaginative activity, we psychoanalytically investigate the collective imaginary of Psychology students regarding the first clinical consultation. We conducted a group interview with 52 undergraduate students, using the Thematic Story-Drawing Procedure as a way to open a dialogical field. The material obtained, through the psychoanalytical method, resulted in the creation/gathering of four affective-emotional meaning fields: “I came, I saw and I conquered”, “I know that I (do not know”, “I survived and I will save” and “I am and I do”, from which we see an emotionally immature imaginary about the meeting with the patient, since students are more self-centered than concerned with the patient. The overall situation indicates the need for care regarding student academic development, in order to encourage a more mature approach toward the suffering of the other.

  13. Congenital dyserythropoietic anemia type 1 with a novel mutation in the CDAN1 gene previously diagnosed as congenital hemolytic anemia.

    Science.gov (United States)

    Fujino, Hisanori; Doisaki, Sayoko; Park, Young-Dong; Hama, Asahito; Muramatsu, Hideki; Kojima, Seiji; Sumimoto, Shinichi

    2013-05-01

    The congenital dyserythropoietic anemias (CDAs) are a heterogeneous group of genetic disorders of red cell production. They are characterized by ineffective erythropoiesis and dyserythropoiesis. Here, we present the clinical description and mutation analysis of a Japanese female with CDA type 1. She has long been diagnosed with unclassified congenital hemolytic anemia from the neonatal period. However, bone marrow morphology and genetic testing of the CDAN1 gene at the age of 12 years confirmed the afore-mentioned diagnosis. Thus, we should be aware of the possibility of CDA if the etiology of congenital anemia or jaundice cannot be clearly elucidated.

  14. Clinical application of ultrasound biomicroscopy in the treatment of congenital corneal opacities%超声生物显微镜在先天性角膜混浊诊疗中的应用价值

    Institute of Scientific and Technical Information of China (English)

    李东芳; 赵军; 李滢; 臧新杰; 应良

    2012-01-01

    Objective To investigate the clinical application of ultrasound biomicroscopy in the treatment of congenital corneal opacities.Methods Medical records of 20 eyes (15 patients) with congenital corneal opacity treated at our hospital from July 2004 to November 2011 were retrospectively reviewed.Best corrected visual acuity testing,intraocular pressure testing,slit-lamp anterior segment examination,fundus examination,slit-lamp microscopic photography,B scan examination,and ultrasound biomicroscopy were performed for analysis of complications of congenital corneal opacity and selection of surgical approaches.Results The ultrasound biomicroscopic examination showed that 5 eyes had no Descemet's membrane and corneal endothelium,20 eyes had anterior synechia,5 eyes had aniridia,3 eyes had loss of lens cortex,13 eyes had cataract,14 eyes had closed angle,and 3 eyes had pupillary membrane.14 of 20 eyes received surgical treatment,including penetrating keratoplasty combined with cataract extraction and trabeculectomy (5 eyes),penetrating keratoplasty combined with pupil angioplasty (3 eyes),penetrating keratoplasty combined with cataract extraction (3 eyes),penetrating keratoplasty combined with trabeculectomy (2 eyes),and lamellar keratoplasty (1 eye).Conclusions Ultrasound biomicroscopy is important to guide the diagnosis and treatment of congenital corneal opacity.%目的 探讨超声生物显微镜在治疗先天性角膜混浊的临床应用.方法 回顾分析2004年7月至2011年11月于我院收治的先天性角膜混浊患者15例20只眼,常规行最佳矫正视力检查、眼压检查、裂隙灯眼前节检查、眼底检查、大体照相、B型超声检查、超声生物显微镜检查,分析先天性角膜混浊的并发症及手术方式情况.结果 根据超声生物显微镜检查结果,先天性角膜混浊患者伴有角膜无后弹力膜和内皮层者5只眼,虹膜前粘连(Peter异常)者20只眼,虹膜缺损者5只眼,晶体皮质丢失者3

  15. Experience of cardiopulmonary bypass in congenital heart disease surgery of low weight infants%低体重婴幼儿先心病手术的体外循环经验

    Institute of Scientific and Technical Information of China (English)

    黄东娇; 李传在

    2014-01-01

    目的:总结本院5年来10kg以下婴幼儿先天性心脏病的体外循环管理经验。方法:2009年7月-2014年7月收治先天性心脏病患儿123例,根据畸形复杂程度,采用浅低温或中低温体外循环,重视各种脏器的保护。结果:全组体外循环时间25~188 min,主动脉阻断时间10~123 min,转流中Hct 0.25~0.30,均顺利脱机。术后死亡5例。结论:对于婴幼儿先天性心脏病的 ECC 管理力求做到精细,能够减轻体外循环对婴幼儿的生理干扰,促进术后恢复,减少术后并发症及死亡率。%Objective:To summarize the management experience on cardiopulmonary bypass in congenital heart disease surgery of infants below 10kg weight in recent 5 years.Methods:123 infants with congenital heart disease were selected from July 2009 to July 2014,according to the complicated degree of deformity,we used extracorporeal circulation of mild hypothermia or in low temperature,we attached great importance to the protection of various organs.Results:the cardiopulmonary bypass time of the whole group was 25~188min,aortic cross clamping time was 10~123min,Hct in bypass was 0.25~0.30,all of them were successfully offline.5 cases were death in postoperative.Conclusion:For the ECC management of infants with congenital heart disease we must strive to do fine,so that can reduce the physiological disturbance of extracorporeal circulation on infants and promote postoperative recovery and reduce postoperative complications and mortality.

  16. The South African Military Nursing College Pupil Enrolled Nurses’ experiences of the clinical learning environment

    OpenAIRE

    Ernestina M. Caka; Sebi Lekalakala-Mokgele

    2013-01-01

    The study focused on the clinical learning experiences of Pupil Enrolled Nurses (PENs) within the military health service. The purpose of the research was to explore and describe the learning experiences of PENs within the Military health clinical learning environment. An explorative, descriptive, contextual design which is qualitative in nature was used to guide the study. The military as a training institution prides itself on preparing nurses both as soldiers and nurses, this could be both...

  17. Eating disorders - knowledge, attitudes, management and clinical experience of Norwegian dentists

    OpenAIRE

    Johansson, Ann-Katrin; Johansson, Anders; Nohlert, Eva; Norring, Claes; Åstrøm, Anne N; Tegelberg, Åke

    2015-01-01

    Background The purpose of this study was to investigate knowledge, attitudes and clinical experience with regard to patients with eating disorders (ED) among Norwegian dentists. Methods In 2010, a questionnaire was sent to all dentists in Norway (N = 4282) comprising 33 questions related to demographics of the participating dentists, their knowledge of ED (general and oral health aspects), clinical experience, attitudes and perceived management preferences. Results The participation rate was ...

  18. Eating disorders - knowledge, attitudes, management and clinical experience of Norwegian dentists

    OpenAIRE

    Johansson, Ann-Katrin; Johansson, Anders; Nohlert, Eva; Norring, Claes; Åstrøm, Anne N; Tegelberg, Åke

    2015-01-01

    Background: The purpose of this study was to investigate knowledge, attitudes and clinical experience with regard to patients with eating disorders (ED) among Norwegian dentists. Methods: In 2010, a questionnaire was sent to all dentists in Norway (N = 4282) comprising 33 questions related to demographics of the participating dentists, their knowledge of ED (general and oral health aspects), clinical experience, attitudes and perceived management preferences. Results: The participation rate w...

  19. Congenital temporal triangular alopecia.

    OpenAIRE

    Bargman, H

    1984-01-01

    Congenital temporal triangular alopecia is a form of nonscarring alopecia that, as its name suggests, is present at birth. Four cases are reported. One patient underwent hair transplantation, which was successful and might be useful in other patients. Cases occurring in a father and his son suggest for the first time a genetic link.

  20. CONGENITAL PATELLA LUXATION

    OpenAIRE

    Cakmak, Mehmet; Taser, Omer; Domanic, Unsal; Temelli, Yener; Karamehmetoglu, Mahmut

    2004-01-01

    Five cases of congenital patella luxation which is rarely seen, have been presented. The diagnosis and treatment problems of this disease have been discussed in our cases with literature on the subject. It was concluded the method of the plastic of Williams-Picat quadriceps performed in the unification with the method of Krogius is the most appropriate form of treatment.

  1. Severe congenital neutropenia

    DEFF Research Database (Denmark)

    Borregaard, Niels

    2014-01-01

    In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutr...... neutropenia (SCN)....

  2. Congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    970296 Evaluating the degree of pulmonary vascularlesions in congenital heart disease with selective pul-monary angiography. PAN Shiwei(潘世伟), et al.Fuwai Hosp, CAMS & PUMC, Beijing, 100037. Chin JCardiol 1997; 25(1): 39-41. Objective: To evaluate the degree of pulmonary vas-

  3. Congenital Heart Information Network

    Science.gov (United States)

    ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright ©1996 - 2016 C.H.I.N. All rights reserved TX4-390-685 Original site design and HTML by Panoptic Communications

  4. Congenital Lumbar Hernia

    Directory of Open Access Journals (Sweden)

    Sanjay Sharma

    2008-01-01

    Full Text Available Lumbar hernia is a rare hernia. It constitutes less than one percent of all abdominal hernias. It can becongenital or acquired. Acquired can occur either spontaneously or after surgery or trauma. Only 300cases of lumbar hernia are reported till date. We report a case of congenital lumbar hernia in one month oldmale baby

  5. Congenital Absence of Tibia

    Directory of Open Access Journals (Sweden)

    Sudesh Sharma, Saleem Mir, Vikrant Sharma, Irshad Dar, Rafee

    2002-10-01

    Full Text Available Congenital absence of tibia is a rare anomaly. We repol1 a case who presented at the age of 3 years withabsence of tibia right side with associated anomolies and was managed by reconstruction of the kneeand ankle joints b transfer of fibula

  6. Clinical experience in the use of marginal donor hearts

    Institute of Scientific and Technical Information of China (English)

    XIE Ai-ni; DONG Nian-guo; ZHANG Kai-lun; XIA Jia-hong; XIAO Shi-liang; SUN Zong-quan

    2011-01-01

    Background Although heart transplantation has become a standard therapy for end-stage heart disease, there are few published studies regarding the use of transplant organs from marginal donors. Here we describe the clinical outcome we have obtained using marginal donor hearts.Methods We analyzed 21 cases of orthotropic heart transplantation for end-stage heart disease performed in our department between September 2008 and July 2010. Of these patients, six received hearts from marginal donors and the remainder received standard-donor hearts. The two groups were compared in terms of both mortality and the incidence of perioperative complications such as infection, acute rejection, and right heart insufficiency.Results The 1-year survival rate of both groups was 100%. Only one death was recorded in standard-donor group during follow-up. Patients who received marginal donor hearts (83%) experienced more early complications than did the standard-donor-heart group (13%), but the mortality of the two groups was the same. The duration of post-ICU stay was greater in the marginal donor group than in the standard-donor group, (35.5±17.4) days and (21.7±2.6) days, respectively (P <0.05).Conclusions The use of marginal donor hearts increases the number of patients who can receive and benefit from transplants. However, it may introduce an increased risk of early complications, thus care should be taken both in the choice of patients who will receive marginal donor hearts and in the perioperative treatment of those for whom the procedure is performed.

  7. Clinical experience of surgical intervention for severe acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Xu Yuan; Shao Qinshu; Yang Jin; Yu Xiaojun; Xu Ji

    2014-01-01

    Background The controversy on the treatment strategy for severe acute pancreatitis (SAP) has never stopped for the past century.Even now surgical procedures play a decisive role in the treatment of SAP,especially in managing the related complications,but the rational indications,timing,and approaches of surgical intervention for SAP are still inconclusive.Methods Clinical data of 308 SAP patients recruited during January 2000-January 2013,including 96 conservatively treated cases plus 212 surgically intervened cases,were comparatively analyzed.Based on the initial surgical intervention time,the surgical intervention group was split into two:early intervention group (within 2 weeks) 103 cases,and late intervention group (after 2 weeks) 109 cases.Results In the conservative treatment group,the cure rate was 82.29% (79/96),the death rate was 13.54% (13/96),and 4 cases self-discharged,while in the surgical intervention group,the cure rate was 84.43% (179/212) and the death rate was 10.85% (23/212) with 10 cases self-discharged.The difference was of no statistical significance between these two groups (P >0.05).In surgical intervention group,the death rate 15.53% (16/103) in the early surgical intervention group was higher than that of late surgical intervention group 6.42% (7/109),and the difference was statistically significant (P <0.05).Conclusions Both conservative treatment and surgical intervention play important roles in the treatment of SAP,and the indication,timing,and procedure should be strictly followed.Surgery earlier than 2 weeks after onset of the disease is not recommended in patients with necrotizing pancreatitis only when there are specific indications,such as multiple organ failure,which does not improve despite active treatment,and in those who develop abdominal compartment syndrome.

  8. Clinical trial experience using erythropoietin during radiation therapy

    International Nuclear Information System (INIS)

    Oncologists have several reasons for trying to maintain or increase hemoglobin levels in their patients during therapy. Relief of the symptoms of anemia, including fatigue and dyspnea, are traditional, well-accepted indications. A newer rationale is to enhance the efficacy of radiation therapy and/or chemotherapy in controlling tumors. A laboratory animal study found that administration of recombinant human erythropoietin (rHuEPO) increased intratumoral median oxygen levels and diminished the proportion of measurements in the very low (<3 mm Hg) range. Hemoglobin level is a strong independent prognostic factor for tumor control by radiation therapy. The hemoglobin level at the end of radiation therapy is a stronger prognostic factor than is the hemoglobin level at the start of therapy. Numerous clinical trials have utilized rHuEPO during radiation with or without concurrent chemotherapy. All 4 trials which enrolled patients with low hemoglobin levels (<12 to 13.5 g/dl) found that rHuEPO significantly increased hemoglobin within 2 weeks and that hemoglobin levels continued to rise until the end of rHuEPO treatment. rHuEPO was efficacious in limiting the decrease in hemoglobin and use of packed red blood cell transfusion in the one reported trial in which it was used in patients with initially normal hemoglobin levels during intensive concurrent radiation and chemotherapy. One trial found a statistically significant improvement in complete pathologic response rate after neoadjuvant chemoradiotherapy with the use of rHuEPO. rHuEPO has a potentially large role to play in the care of the cancer patient. (orig.)

  9. Methyldibromo glutaronitrile: clinical experience and exposure-based risk assessment.

    Science.gov (United States)

    Zachariae, Claus; Rastogi, Suresh; Devantier, Charlotte; Menné, Torkil; Johansen, Jeanne Duus

    2003-03-01

    In the year 2000, the level of methyldibromo glutaronitrile (MDGN) allergy in dermatology clinics in Europe exceeded the level of allergies to all other preservatives, with a prevalence of 3.5%. In the present study, cases of primary sensitization and elicitation to MDGN due to cosmetic products were collected over an 8-month period at the Department of Dermatology, Gentofte University Hospital. The aim was to identify the products related to hand eczema, assess exposure to MDGN in these products and relate the findings to results from a newly developed updated risk assessment model for contact allergy. Out of 24 patients with a positive patch test to MDGN, 17 patients with hand eczema were identified. In 11 of these patients, cosmetic products used in relation to the onset of the disease were shown to contain MDGN (65%). In 8 of these 11 cases, primary sensitization was probable, 5 due to hand/body lotions and 3 due to lotions and/or liquid hand soap. Chemical analysis of 12 products showed that lotions contained 149-390 ppm of MDGN, liquid hand soap 144-399 ppm, a rinsing cream 293 ppm and shampoos 78-79 ppm. The shampoo exposure was not of certain relevance to the eczema. Applying the newly developed updated risk assessment model showed that the concentrations of MDGN in lotions of 149-390 ppm exceeded the calculated maximum acceptable exposure level for MDGN, which would be expected to lead to sensitization in consumers using such products, as seen in the current study. The present cases and updated exposure-based risk assessment process add to the evidence and need for re-defining safe-use concentrations of MDGN in cosmetic products.

  10. Continuous intra-arterial blood gas monitoring. A clinical experience.

    Science.gov (United States)

    Paolillo, G; Tosoni, A; Mariani, M A; Venturino, M

    1994-01-01

    Miniaturized sensors, based upon the principles of optical fluorescence, can measure in vivo the pH, pCO2 value and pO2 value of blood. In this report we studied continuous intra-arterial blood gas monitoring in 27 patients undergoing cardiac surgery (no. 16 coronary artery by-pass grafting, no. 2 valvular surgery) and major vascular surgery (no. 9 abdominal aortic aneurysms). Total duration of continuous intra-arterial blood gas monitoring was 677 hours, with a ratio of 25.0 +/- 14.8 hours/patient (range 4-96 hours). The in vitro values of pH, pCO2 and pO2 were compared to simultaneous records from the fiberoptic sensor for each of the 283 arterial blood gas samples obtained, by means of linear regression and Bland-Altman method, in order to test the correlation and the agreement between the two methods of measuring. For pH average bias was -0.023 and intersensor precision was 0.028, with a strong correlation (R = 0.92; p sensor precision was 2.65, with a slight decrease in correlation (R = 0.89; p < 0.001) and agreement. For pO2 average bias was -2.69 and the intersensor precision was 12.16, with a strong correlation (R = 0.97; p < 0.001) and agreement. In addition, we tested the reliability of the system for values of pO2 above 100 mmHg and we found a strong correlation (R = 0.96; p20.001) and agreement even for these clinical conditions, largely out of physiologic parameters. This study demonstrates the feasibility and reliability of continuous intra-arterial three-component PB 3300 (Puritan Bennett) blood gas monitoring. PMID:7800182

  11. Clinical course of ectopic pregnancy: A single-center experience

    Directory of Open Access Journals (Sweden)

    Aqueela Ayaz

    2013-01-01

    Full Text Available Objectives: The objective was to highlight the frequency, clinical profile, and predisposing factors of ectopic pregnancy (EP in a general hospital. Materials and Methods: This descriptive study was conducted at the Obstetrics and Gynaecology department of Hera General hospital, Makkah, Saudi Arabia, from July 1, 2009 to December 29, 2010. Data were collected on chief medical complaints, sociodemographic characteristics, past obstetrics and gynecological history, management done, and outcome of management. Data were analyzed using Microsoft Office Excel (version 2007. Results: Out of total 7564 pregnancies, 44 (0.58% patients were diagnosed as EP. Out of 44, 22 (50% patients presented within 24 h of onset of symptoms. Mean age was 28 ± 7 years. Multigravida were predominant in 25 (57%, and 21 (48% had gestational age of 6-8 weeks at the time of presentation; the common presenting features were amenorrhea (41, 93.2%, abdominal pain (39, 88.6%, and tenderness (38, 86%. Previous pelvic surgery (13, 29.5%, infertility treatment (11, 25%, and pelvic inflammatory disease (10, 22.7% were the common predisposing factors. Twenty-five (57% presented with ruptured EP and were operated within 24 h, and the remaining were kept under observation till further diagnosis. After confirming the diagnosis, 12/19 underwent laparoscopy, whereas 7/19 received medical treatment. Surgery confirmed fallopian tube pregnancies in 35 (94.5%. No mortality was observed. Conclusion: Previous pelvic surgeries were the major etiological factor for EP. Other factors were infertility treatment and pelvic inflammatory disease. The most common site of EP was fallopian tubes.

  12. Clinical Experience with Chitosan Matrix and Cultured Fibroblasts for Burns

    Directory of Open Access Journals (Sweden)

    Gaziza Danlybayeva

    2014-12-01

    Full Text Available Introduction. Burns are an important public health challenge due to the frequency of getting burns in day-to-day life, occupational hazards, and catastrophes. Treatment of burns is complex and is associated with high morbidity and mortality. Duration and complexity of burn treatment require finding new ways of curing and rehabilitating burns. The result of burn treatment plays a significant role in post-traumatic status of a patient and his or her consequent adaptation in society. Chitosan is a natural safe non-toxic product compatible with human tissues, characterized by hydrosorbid, anticoagulant, antibacterial, and wound healing features. The study aims to  show a clinical application of chitosan-pectin scaffold with cultured human skin fibroblasts in the treatment of deep burns.Methods. The substrate was prepared by dissolving 3% chitosan in 0.5N acetic acid, which was then mixed with 3% solution of pectin dissolved in distillated water. Chitosan film was formed in a Petri dish for 20-24 hours at 20-25 °C. After drying the film, cultured allogeneic fibroblasts (patent number RK-25091 were seeded on its surface.Results. The results from an in vitro culture study showed that human allogeneic fibroblasts could adhere well and grow on the selected scaffold with a typical morphology. During autodermoplasty surgery, cultured allogeneic fibroblasts were applied on granulating wounds of 9 patients with IIIA to IVB degree burns and limited donor resources. Wounds treated with the fibroblast-seeded scaffold among all patients provided the highest level of re-epithelialization (day 5, in comparison to cell-free scaffold (day 7 and untreated surface of wounds (day 10.Conclusion. Our results indicate the potential use of chitosan for wound healing due to its allogenic fibroblast adherence to scaffolding as well as high epithelization. This warrants further studies on chitosan for use in wounds resulting from third and fourth degree burns.

  13. Respiratory dynamic CT of the lung: initial clinical experience

    International Nuclear Information System (INIS)

    We applied spiral CT to evaluate the dynamic changes of regional ventilation of the lung in normal subjects and abnormal patients. This study includes normal subjects (n = 5) and patients with chronic obstructive pulmonary disease (n = 4), small air-way disease (n = 3), diffuse panbronchiolitis (n = 4), and tracheobronchial tuberculosis (n = 2). Time-continuous scan data at a fixed level during forced vital capacity maneuver (10-12 seconds) were obtained and images were reconstructed retrospectively by using 0.67 second scan data per image. The reconstructed images were displayed in a cine mode. Time-density curves were plotted and were correlated with clinical diagnosis. In normal subjects, mean attenuation difference between full inspiration and full expiration was 145.8 HU and mean time interval between 20% expiration and 80% expiration was 2.04 seconds. In chronic obstructive lung disease, mean attenuation difference between full inspiration and full expiration was 21.2 HU and mean time interval between 20% expiration and 80% expiration was 3.63 seconds. In small air-way disease, mosaic-pattern hyperlucency and normal portion of lung showed mean attenuation differences between 20% expiration and 80% expiration to be 49.8 HU and 167.0 HU, respectively. In diffuse panbronchiolitis, centrilobular region and normal portion of lung showed mean attenuation differences between 20% expiration and 80% expiration to be 35.4 HU and 79.3 HU, respectively. Respiratory dynamic CT is an updated technique which enable imaging of the functional status of the lung parenchyma. It may be useful in differentiation and quantitation of variable obstructive lung diseases

  14. Psychotic experiences in a mental health clinic sample : implications for suicidality, multimorbidity and functioning

    NARCIS (Netherlands)

    Kelleher, I.; Devlin, N.; Wigman, J. T. W.; Kehoe, A.; Murtagh, A.; Fitzpatrick, C.; Cannon, M.

    2014-01-01

    Background Recent community-based research has suggested that psychotic experiences act as markers of severity of psychopathology. There has, however, been a lack of clinic-based research. We wished to investigate, in a clinical sample of adolescents referred to a state-funded mental health service,

  15. Students' Experiences of Clinic-Based Learning during a Final Year Veterinary Internship Programme

    Science.gov (United States)

    Matthew, Susan M.; Taylor, Rosanne M.; Ellis, Robert A.

    2010-01-01

    This study investigated veterinary students' experiences of clinic-based learning (CBL) during a comprehensive final year internship programme. Open-ended surveys (n = 93) were used to gather qualitative data about students' conceptions of what is learned during CBL and their approaches to learning in clinics. Phenomenography was used for detailed…

  16. Children's Views Matter Too! A Pilot Project Assessing Children's and Adolescents' Experiences of Clinical Psychology Services

    Science.gov (United States)

    Gordon, Michael; Russo, Kate

    2009-01-01

    This pilot study explored the experiences and understanding of clinical psychology practices and services of children and adolescents attending clinical psychology outpatient appointments. Fifteen young participants took part in the study. A content analysis indicated that young children and adolescents have an appropriate understanding of the…

  17. Experiences of Student Speech-Language Pathology Clinicians in the Initial Clinical Practicum: A Phenomenological Study

    Science.gov (United States)

    Nelson, Lori A.

    2011-01-01

    Speech-language pathology literature is limited in describing the clinical practicum process from the student perspective. Much of the supervision literature in this field focuses on quantitative research and/or the point of view of the supervisor. Understanding the student experience serves to enhance the quality of clinical supervision. Of…

  18. Clinical and hematological features of congenital dyserythropoietic anemia type Ⅰ%先天性红细胞生成异常性贫血Ⅰ型临床及血液学特征

    Institute of Scientific and Technical Information of China (English)

    王慧君; 张凤奎; 张莉; 周康; 井丽萍; 杨栋林; 李洪强; 刘庆国; 茹永新; 储榆林

    2009-01-01

    Objective To analyze the clinical and laboratory features of patients with congenital dys-erythropoietic anemia type Ⅰ (CDA-Ⅰ), and improve the clinical diagnostic accuracy. Methods The clin-ical and hematological features of 5 patients diagnosed as CDA-Ⅰ in our hospital between July 2002 and July 2007 were analyzed retrospectively, and the related literatures was reviewed. Results Five CDA-Ⅰ pa-tients, 1 male and 4 females, all had a long history of varied degree of chronic anemia. One patient had con-genital malformations, 3 jaundice and 4 hepatosplenomegaly. Bone marrow specimens invariably showed hy-percellularity due to erythroid hyperplasia with megaloblastoid changes, irregularly shaped nuclear, and chro-matin bridges in 0.2% to 0.6% of all erythroblasts. All the 5 patients' bone marrow erythroblasts showed spongy heterochromatin appearances(swiss-cheese) with electron microscopy examination. There was no mor-phologic abnormality in the granulocytes and megakaryocytes. Serum ferritin levels were increased in 3/4 pa-tients. One patient had been misdiagnosed as hereditary spherocytosis and performed splenectomy in the local hospital with no improvement in Hb level. Conclusions CDA-Ⅰ is a rare congenital anemia characterized by ineffective erythropoiesis, jaundice, hepatosplenomegaly and iron overload, and may be misdiagnosed. Keeping these manifestations in mind should avoid misdiagnosis.%目的 分析先天性红细胞生成异常性贫血Ⅰ型(CDA-Ⅰ)的临床及实验室特征,以提高对该病的认识.方法 对5例CDA-Ⅰ患者的临床表现、血液学检查结果及药物治疗反应结合文献进行同顾性分析.结果 5例患者中男1例,女4例,中位年龄31(17~39)岁.均幼年发病,长期贫血,1例发育畸形,3例伴有黄疸,4例脾肿大.骨髓有核细胞增生明显活跃,红系比例增高,巨幼样变,幼红细胞间可见核间桥;粒系、巨核系细胞形态无特殊改变.幼红细胞胞核呈瑞士奶酪样特

  19. Stem Cell Therapy for Cardiovascular Disorders - Our Clinical Experience

    Directory of Open Access Journals (Sweden)

    Jayakrishnan AG

    2011-01-01

    Full Text Available Background: Autologous Bone Marrow stem Cell transplantation is a viable therapeutic option for patients with end stage heart failure due to cardiomyopathy of varied etiology as there are only limited treatment options other than cardiac transplantation. The rationale behind the application of stem cells in these patients include • Stem cells directly replace the affected cells by differentiation into the damaged cell type • Stem cells also exert Paracrine effects by secre tion of growth factors (VGEF,FGF-1to stimu late local cell growth•In addition to the above, stem cells release signaling factors which recruit stem cells from elsewhere by modulating the immune system.Materials and Methods: In this presentation we describe our study on a series of 13 patients who received isolated and expanded CD 34 cells from the bone marrow. Seven had ischemic dysfunction, three had dilated cardiomyopathy and three had primary pulmonary hypertension. Five patients received the stem cells via intracoronary injection, three directly into the myocardium and three intrapulmonary. Results: All patients showed functional improvement of the myocardium recorded by non-invasive investigations and improvement in the quality of life. Follow up period ranged from 6 months to 2 years. Conclusion: Our experience with bone marrow derived stem cells in patients with cardiomyopathy has been encouraging. More studies are planned in the future.

  20. Facilitative and obstructive factors in the clinical learning environment: Experiences of pupil enrolled nurses

    Directory of Open Access Journals (Sweden)

    Eucebious Lekalakala-Mokgele

    2015-02-01

    Full Text Available Background: The clinical learning environment is a complex social entity that influences student learning outcomes in the clinical setting. Students can experience the clinical learning environment as being both facilitative and obstructive to their learning. The clinical environment may be a source of stress, creating feelings of fear and anxiety which in turn affect the students’ responses to learning. Equally, the environment can enhance learning if experienced positively.Objectives: This study described pupil enrolled nurses’ experiences of facilitative and obstructive factors in military and public health clinical learning settings.Method: Using a qualitative, contextual, exploratory descriptive design, three focus group interviews were conducted until data saturation was reached amongst pupil enrolled nurses in a military School of Nursing.Results: Data analysed provided evidence that acceptance by clinical staff and affordance of self-directed learning facilitated learning. Students felt safe to practise when they were supported by the clinical staff. They felt a sense of belonging when the staff showed an interest in and welcomed them. Learning was obstructed when students were met with condescending comments. Wearing of a military uniform in the public hospital and horizontal violence obstructed learning in the clinical learning environment.Conclusion: Students cannot have effective clinical preparation if the environment is not conducive to and supportive of clinical learning, The study shows that military nursing students experience unique challenges as they are trained in two professions that are hierarchical in nature. The students experienced both facilitating and obstructing factors to their learning during their clinical practice. Clinical staff should be made aware of factors which can impact on students’ learning. Policies need to be developed for supporting students in the clinical learning environment.

  1. Congenital cutaneous histiocytosis in a piglet.

    Science.gov (United States)

    Hélie, P; Kiupel, M; Drolet, R

    2014-07-01

    A 2-week-old crossbred male piglet with numerous congenital, variably sized macules, plaques, and papules distributed all over the body was submitted for necropsy. Significant gross and histological lesions were restricted to the skin. On light microscopic examination, these cutaneous lesions corresponded to dermal and/or subcutaneous masses composed of spindle-shaped to round cells that multifocally contained hemosiderin; epidermotropism was not observed. Immunohistochemically, the neoplastic cells were strongly positive for CD204; moderately positive for CD163, lysozyme, and vimentin; and negative for Mac 387, α-1-antitrypsin, S-100 protein and E-cadherin; frozen tissues were not available for CD1a and CD11c. Transmission electron microscopic examination of sections from formalin-fixed tissues did not reveal Birbeck's granules. The clinical, morphological, and immunohistochemical results were consistent with a congenital cutaneous histiocytosis of non-Langerhans cell origin. The condition most resembled juvenile xanthogranuloma in humans, a generally skin-limited non-Langerhans histiocytic disorder that can be congenital. Cutaneous and/or systemic histiocytic disorders are well characterized in dogs and have been described in cats, and a case with some similarities to ours has been reported in a neonatal piglet, but this is to our knowledge the first immunohistochemically supported report of histiocytosis in the pig and congenital histiocytosis in animals.

  2. 新型泪道探通针治疗先天性泪道阻塞临床分析%Clinical analysis of congenital nasolacrimal duct obstruction treated with a new type of probe

    Institute of Scientific and Technical Information of China (English)

    张莉; 方旺; 王莉; 陈凌燕; 肖诗艺; 张越骊

    2012-01-01

    目的:根据患儿年幼、头部易摆动、针头难固定易滑脱、组织娇嫩、不宜探针反复进出泪道操作等特点,探讨新型泪道探通针治疗婴幼儿先天性泪道阻塞(nasolacrimal duct obstruction,NLDO)的临床疗效.方法:用新型泪道探通针对眼科门诊2 568例2 771眼先天性NLDO患者进行泪道探通,并观察疗效.结果:所有患者手术成功,其中一次探通成功2 722眼(98.23%),二次探通成功49眼(1.77%),除极少数患儿有轻度眼睑水肿外,无任何严重并发症发生.结论:新型泪道探通针设计合理、操作方便、安全实用,对泪道无损伤、独立包装、消毒彻底、能避免交叉感染、价格低廉、临床应用效果良好,尤其适合婴幼儿患者,值得推广使用.%·AIM:To study the clinical effect of a new type of probe to treat congenital nasolacrimal duct obstruction(NLDO) according to the characteristics of the infant, such as age, head not being easily fixed, tissue tenderness and not suitable for manipulation repeatedly. ·METHODS:All 2 568 infants(2 771 eyes) who have congenital NLDO Received probing of lacrimal passages with the new type probing needle and were observed in the clinic service. ·RESULTS: All eyes could be probed successfully, especially 2 722 eyes (98.23%) could be probed successfully one time and 49 eyes (1.77%) were two times. There were not any severe complications but gentle palpebral edema. ·CONCLUSION: New one-time probing needle which are reasonable designed are convenient, safe and practical to use. They have no hurt to lacrimal passage. At the same time, they can be packed and sterilized independently to avoid cross contamination. This new type, low price needle which is particularly suitable for infant has good therapeutic effect and deserves to be spread to use.

  3. Clinical trial experience using erythropoietin during radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Lavey, R.S. [Radiation Oncology Program, Childrens Hospital Los Angeles, Univ. of Southern California, CA, Los Angeles (United States)

    1998-12-01

    Oncologists have several reasons for trying to maintain or increase hemoglobin levels in their patients during therapy. Relief of the symptoms of anemia, including fatigue and dyspnea, are traditional, well-accepted indications. A newer rationale is to enhance the efficacy of radiation therapy and/or chemotherapy in controlling tumors. A laboratory animal study found that administration of recombinant human erythropoietin (rHuEPO) increased intratumoral median oxygen levels and diminished the proportion of measurements in the very low (<3 mm Hg) range. Hemoglobin level is a strong independent prognostic factor for tumor control by radiation therapy. The hemoglobin level at the end of radiation therapy is a stronger prognostic factor than is the hemoglobin level at the start of therapy. Numerous clinical trials have utilized rHuEPO during radiation with or without concurrent chemotherapy. All 4 trials which enrolled patients with low hemoglobin levels (<12 to 13.5 g/dl) found that rHuEPO significantly increased hemoglobin within 2 weeks and that hemoglobin levels continued to rise until the end of rHuEPO treatment. rHuEPO was efficacious in limiting the decrease in hemoglobin and use of packed red blood cell transfusion in the one reported trial in which it was used in patients with initially normal hemoglobin levels during intensive concurrent radiation and chemotherapy. One trial found a statistically significant improvement in complete pathologic response rate after neoadjuvant chemoradiotherapy with the use of rHuEPO. rHuEPO has a potentially large role to play in the care of the cancer patient. (orig.) [Deutsch] In der Onkologie bestehen zahlreiche Gruende, die Haemoglobinkonzentration der Patienten waehrend der Therapie zu halten oder sogar anzuheben. Als anerkannte Indikation gilt hierbei die Besserung anaemiebedingter Symptome wie Muedigkeit und Dyspnoe, wobei jedoch neuere Ergebnisse darauf hinweisen, dass auch die Effizienz der Strahlen- und

  4. Congenital pseudarthrosis of clavicle, differential diagnosis pathology

    International Nuclear Information System (INIS)

    The congenital pseudarthrosis of clavicle is a rare entity, frequently appearing without association to other pathologies and does not cause important limitations in the children. It can confuse with other traumatic pathologies like clavicle fracture. Most of the patients complain about the aesthetics and few times for pain. The treatment is generally surgical there is controversy about of carrying out surgery. We reported two clinical cases with pseudoarthrosis of the right clavicle that they received surgical treatment with satisfactory results.

  5. Nutrition in neonatal congenital heart disease

    OpenAIRE

    Morgan CT; Shine AM; McMahon CJ

    2013-01-01

    Conall T Morgan,1 Anne Marie Shine,2 Colin J McMahon1 1Department of Pediatric Cardiology, 2Department of Clinical Nutrition and Dietetics, Our Lady's Children's Hospital Crumlin, Dublin, Republic of Ireland Abstract: There are 40,000 infants born in the USA with congenital heart disease annually. Achievement of adequate oral nutrition is difficult in this population. Malnutrition is common. Single ventricle physiology, the risk of necrotizing enterocolitis, and cardiopulmonary bypass...

  6. Nutrition in neonatal congenital heart disease

    OpenAIRE

    McMahon, Colin

    2013-01-01

    Conall T Morgan,1 Anne Marie Shine,2 Colin J McMahon1 1Department of Pediatric Cardiology, 2Department of Clinical Nutrition and Dietetics, Our Lady's Children's Hospital Crumlin, Dublin, Republic of Ireland Abstract: There are 40,000 infants born in the USA with congenital heart disease annually. Achievement of adequate oral nutrition is difficult in this population. Malnutrition is common. Single ventricle physiology, the risk of necrotizing enterocolitis, and cardiopulmona...

  7. Aphallia:A Rare Congenital Anomaly

    OpenAIRE

    Alpa Gupta; Alpa Gupta, Harendra Gupta*

    2008-01-01

    Aphallia or absence of penis is a very rare congenital anomaly. Clinical presentation is diagnostic, butimmediate and long term management poses great dilemma. The issue like gender reassignment needsparental counseling. Due to social reasons parents have difficulty in giving consent for the classicalmanagement of gender reassignment. However with better penile reconstruction techniques, now there ishope for such parents who want to bring up their child as a boy.We present such a rare case wi...

  8. Congenital disorders of glycosylation with neonatal presentation

    OpenAIRE

    Resende, Catarina; Carvalho, Carmen; Alegria, Artur; Oliveira, Dulce; Quelhas, Dulce; Bandeira, Anabela; Proença, Elisa

    2014-01-01

    Congenital disorders of glycosylation (CDG) are a group of hereditary diseases characterised by deficiency of enzymes involved in proteins glycosylation. We describe the clinical case of a neonate with CDG type 1a, nowadays designated phosphomannomutase 2 (PMM2)-CDG. Physical examination showed an abnormal facies, axial hypotonia, abnormal fat distribution, inverted nipples, non-palpable testicles and arachnodactyly. Progressive multiple system organ involvement and worsening of hypertrophic ...

  9. Ultrasonographic diagnosis and clinical prognosis of fetal congenital diseases of the lung%胎儿肺部病变产前超声表现与临床预后

    Institute of Scientific and Technical Information of China (English)

    姜小力; 邓学东; 凌晨; 唐亚奇; 梁泓

    2012-01-01

    examination is a reliable method for the diagnosis of fetal congenital lung disease , and enable the prediction of clinical prognosis of the affected fetuses. If the fetus had heart and mediustinum displacement , hydrops or chromosomal abnormalities , termination should be considered.%目的 探讨胎儿肺部病变超声声像图特征及临床预后与转归.方法 对49例肺部病变胎儿的产前超声声像图特征及临床随访资料进行回顾性分析.结果 49例肺部病变胎儿产前超声表现及临床结局:(1)先天性肺囊腺瘤畸形21例(42.9%,21/49),超声表现为混合性回声内见多个大小不等的无回声区或增强回声团块.其中15例引产后病理证实为先天性肺囊腺瘤畸形,1例出生后新生儿死亡,5例于孕37周前肺部肿块消失.(2)隔离肺12例(24.5%,12/49),超声主要表现为胎儿胸腔内均质增强回声的楔形包块.其中3例引产后病理证实为隔离肺;3例于孕36周前肺部肿块消失;6例活产后经MRI或手术病理证实病灶仍存在.(3)先天性膈疝9例(18.4%,9/49),超声表现为胸腔内见腹腔脏器.其中7例引产(1例合并18-三体),1例出生后新生儿死亡,1例活产,产后手术存活.(4)胸腔积液5例(10.2%,5/49),超声表现为胸腔内无回声区,其中4例引产(分别合并有21-三体、胎儿水肿、Turner综合征、大量腹腔积液),1例于孕34周时胸腔积液消失.(5)肺缺如2例(4.1%,2/49),超声表现为肺叶缺失,其中左、右肺叶缺如各1例,均选择引产.结论 超声检查是检出胎儿肺部病变的可靠方法,可从超声观察角度预测肺内病变胎儿的临床预后与转归;胎儿纵隔和心脏移位、胎儿水肿或合并染色体异常是终止妊娠的指征.

  10. Transitions from Clinical Experiences to Clinical Questions and then Research: Songwriting with Bereaved Pre-adolescent Children

    Directory of Open Access Journals (Sweden)

    Melina Roberts

    2006-11-01

    Full Text Available The purpose of this article is to describe the author’s personal transition from her clinical experiences working with bereaved children and adolescents, to clinical questions that arose during this time, and then to her current phase whereby she has commenced research to examine songwriting with bereaved children. This article includes relevant literature that discusses bereavement in childhood, songwriting in music therapy, and music therapy support for bereaved children and adolescents. A description about the author’s current research, that integrates the three areas highlighted in the literature, is also discussed.

  11. Nursing students’ perception of clinical learning experiences as provided by the nursing staff in the wards

    Directory of Open Access Journals (Sweden)

    N. R. C. TIakula

    1993-05-01

    Full Text Available A descriptive survey was carried out, using convenience and systematic sampling in order to better understand the manner in which student nurses perceive their clinical experience in the hospital. Data were collected from 80 subjects in 4 nursing colleges using a critical incident technique. Positive and negative experiences are described,

  12. Comprehensive Experiment--Clinical Biochemistry: Determination of Blood Glucose and Triglycerides in Normal and Diabetic Rats

    Science.gov (United States)

    Jiao, Li; Xiujuan, Shi; Juan, Wang; Song, Jia; Lei, Xu; Guotong, Xu; Lixia, Lu

    2015-01-01

    For second year medical students, we redesigned an original laboratory experiment and developed a combined research-teaching clinical biochemistry experiment. Using an established diabetic rat model to detect blood glucose and triglycerides, the students participate in the entire experimental process, which is not normally experienced during a…

  13. A Mandala: A Diagram of the Clinical Education Experience in Athletic Training

    Science.gov (United States)

    Cernohous, Steve; West, Sharon

    2007-01-01

    Objective: The objective of this paper is to present the practical use of a Mandala that: 1) provides opportunities for athletic training students to explore, reflect on and appreciate their clinical experiences; 2) provides educators with a model to understand and value athletic training student experiences; 3) organizes and captures factors and…

  14. Radiography students' clinical placement experiences in MRI: A phenomenological study

    International Nuclear Information System (INIS)

    Purpose: The purpose of this study was to gain an insight into radiography students' clinical practice experiences within an MRI unit of a large teaching hospital in Malta. The underlying objectives of the study were to identify and explore the descriptions and meanings of the MRI clinical experiences of students. Methods: Five students, independently undergoing their clinical placements in MRI were asked to write a diary about their daily experiences. Each student then participated in a follow up interview. Data analysis was based on Husserls' phenomenological approach. Results: Eight themes emerged from the data analysed. Students described their experience as one mainly based on observation, where, during their placements, they observed radiographers at work. In this respect, students described their experience as lacking ‘hands on practice’. Students indicated that they felt uncomfortable, unsupervised and unwelcome most of the time. They also expressed the need to feel useful and part of the MRI team during their placement and so they tried to help the team in areas such as patient screening. This helped them build confidence. Learning in MRI was specifically based on the university tasks prescribed and possibly there was no motivation to learn more. The students also described their experience as varied and different when compared to other placements. Conclusion: This study provides a rare insight into radiography students' placement experiences in MRI and should enable the supervising radiographer and educator to obtain a deeper appreciation of the clinical placement experience

  15. Sequencing of Simulation and Clinic Experiences in an Introductory Pharmacy Practice Experience

    OpenAIRE

    Leon, Nicholas; Hajjar, Emily; DeSevo Bellottie, Gina

    2015-01-01

    Objective. To examine how the intrasemester sequencing of a simulation component, delivered during an ambulatory care introductory pharmacy practice experience (IPPE), affects student performance on a series of 3 assessments delivered during the second professional (P2) year.

  16. 41例Ⅲ型先天性食管闭锁Ⅰ期手术治疗%Experience of treatment of 113 cases with congenital esophageal atresia

    Institute of Scientific and Technical Information of China (English)

    马良龙; 张泽伟; 高展; 应力阳; 李建华; 朱雄凯; 林茹; 俞建根

    2009-01-01

    Objective To evaluate the outcome of patients with congenital esophageal atresia. Methods The medical records of 113 patients with congenital esophageal atresia were reviewed, 41 were identified with type Ⅲ and underwent one-stage operation. The ratio of surgery treatment in all patients was 36.28%. Among them, 38 patients (92.68% ) had type Ⅲ B and 3 patients (7.32% ) had type ⅢA. Gastrostomy was done at first. Then the baby was positioned left recumbent position. Right posterial-lateral incision was made. The azygous vein was ligated and cut. The esophageal tracheal fistula was dissected, cut and sutured by interrupted suture with 5-0 prolene. The thoracic segments of the esophagus were dissected. End to end anastomosis was done by interrupted suture with 5-0 prolene. 2 to 4 relaxation suture of adventitia of esophagus and medias-tinal pleura were done. Thoracic space drainage tube was localized through next costal space, the tip was lay nearby the anastomosis. The neonates were monitored in the neonate ICU. Results Six of the 41 patients died after operation, with a mortality rate of 14.63%. The other 35 patients survived (85.37% ). Twelve cases that operated after 2007 were all survived after operation. Anastomotic striction and anastomotic leakage were the most common postoperative complications, 41. 46% and 12.20% .respectively. Two cases of anastomotic leakage died, 2 cases who were redone esophageal anastomosis survived, 1 case recovered under expectant treatment. Conclusion The outcome of one-stage repair of esophageal atresia is inspiring. Anastomotic leakage is a high-risk factor lead to death, but it is not the determinant factor. The important thing to improve outcome of one-stage repairmen of esophageal atresia depends on early diagnosis, early operation and better management preopera-tive and postoperative.%目的 探讨先天性食管闭锁Ⅰ期根治手术治疗的效果.方法 1994年11月至2008年6月收治先天性食管闭锁113

  17. Imaging tissue hypoxia: clinical and pre-clinical experience with {sup 123}IAZA

    Energy Technology Data Exchange (ETDEWEB)

    Wiebe, L.I. [University of Alberta, Edmonton (Canada). Noujaim Institute for Pharmaceutical Oncology Research, Faculty of Pharmacy and Pharmaceutical Sciences

    1997-10-01

    The molecular mechanisms that underline the selective binding of iodazomycin arabinoside, IAZA, and related nitromidazoles are reviewed as a basis for interpretation of preclinical and clinical data for hypoxic binding of radioiodinated IAZA. Clinical data are presented for {sup 123}IAZA uptake in a number of pathologies including metastatic tumours, peripheral vascular disease in diabetes, muscle stress and rheumatoid arthritis. The results of studies to determine the influence of tumour type on uptake of {sup 123} I-IAZA in patients with a variety of deep-seated solid tumours will be presented. Correlations of hypoxia-dependent binding with {sup 99m}Tc-HMPAO perfusion images will be reviewed and early correlations of uptake to treatment response in cancer will be presented. Unusual features of {sup 123}I-IAZA biodistribution will also be discussed together with detailed pharmacokinetic and radiation dosimetry data for `2{sup 123}I- IAZA in normal volunteers 27 refs., 1 fig.

  18. Imaging tissue hypoxia: clinical and pre-clinical experience with 123IAZA

    International Nuclear Information System (INIS)

    The molecular mechanisms that underline the selective binding of iodazomycin arabinoside, IAZA, and related nitromidazoles are reviewed as a basis for interpretation of preclinical and clinical data for hypoxic binding of radioiodinated IAZA. Clinical data are presented for 123IAZA uptake in a number of pathologies including metastatic tumours, peripheral vascular disease in diabetes, muscle stress and rheumatoid arthritis. The results of studies to determine the influence of tumour type on uptake of 123 I-IAZA in patients with a variety of deep-seated solid tumours will be presented. Correlations of hypoxia-dependent binding with 99mTc-HMPAO perfusion images will be reviewed and early correlations of uptake to treatment response in cancer will be presented. Unusual features of 123I-IAZA biodistribution will also be discussed together with detailed pharmacokinetic and radiation dosimetry data for '2123I- IAZA in normal volunteers

  19. Utilization of the Nursing Process to Foster Clinical Reasoning During a Simulation Experience

    Directory of Open Access Journals (Sweden)

    Amanda Lambie

    2015-11-01

    Full Text Available Nursing practice includes complex reasoning and multifaceted decision making with minimal standardized guidance in how to evaluate this phenomenon among nursing students. Learning outcomes related to the clinical reasoning process among novice baccalaureate nursing students during a simulation experience were evaluated. Nursing process records were utilized to evaluate and foster the development of clinical reasoning in a high-fidelity medical-surgical simulation experience. Students were unable to describe and process pertinent patient information appropriately prior to the simulation experience. Students’ ability to identify pertinent patient cues and plan appropriate patient care improved following the simulation. The learning activity afforded a structured opportunity to identify cues, prioritize the proper course of nursing interventions, and engage in collaboration among peers. The simulation experience provides faculty insight into the students’ clinical reasoning processes, while providing students with a clear framework for successfully accomplishing learning outcomes.

  20. Rare combination of congenital heart disease and pulmonary alveolar proteinosis.

    Science.gov (United States)

    Tanaka, Yuki; Miyamoto, Takashi; Yoshitake, Shuichi; Naito, Yuji; Kobayashi, Tomio

    2015-10-01

    Here, we describe a case of total anomalous pulmonary venous return with coarctation of the aorta that was diagnosed as pulmonary alveolar proteinosis at autopsy in a male infant. Surgical repair was performed at 1 day of age, but the infant died on postoperative day 51 due to respiratory insufficiency without any evidence of pulmonary venous obstruction. He had been unexpectedly diagnosed with pulmonary alveolar proteinosis and pulmonary hypoplasia on autopsy. Congenital pulmonary alveolar proteinosis is a serious condition with a high mortality rate, which should be considered in the differential diagnosis in patients with a clinical picture of pulmonary venous obstruction, because most patients are unable to survive without proper treatment. In this report, we address specific issues that should be discussed in such cases based on our recent experience. PMID:26310609

  1. A novel GJA8 mutation (p.V44A causing autosomal dominant congenital cataract.

    Directory of Open Access Journals (Sweden)

    Yanan Zhu

    Full Text Available To examine the mechanism by which a novel connexin 50 (Cx50 mutation, Cx50 V44A, in a Chinese family causes suture-sparing autosomal dominant congenital nuclear cataracts.Family history and clinical data were recorded and direct gene sequencing was used to identify the disease-causing mutation. The Cx50 gene was cloned from a human lens cDNA library. Connexin protein distributions were assessed by fluorescence microscopy. Hemichannel functions were analyzed by dye uptake assay. Formation of functional channels was assessed by dye transfer experiments.Direct sequencing of the candidate GJA8 gene revealed a novel c.131T>C transition in exon 2, which cosegregated with the disease in the family and resulted in the substitution of a valine residue with alanine at codon 44 (p. V44A in the extracellular loop 1 of the Cx50 protein. Both Cx50 and Cx50V44A formed functional gap junctions, as shown by the neurobiotin transfer assay. However, unlike wild-type Cx50, Cx50V44A was unable to form open hemichannels in dye uptake experiments.This work identified a unique congenital cataract in the Chinese population, caused by the novel mutation Cx50V44A, and it showed that the V44A mutation specifically impairs the gating of the hemichannels but not the gap junction channels. The dysfunctional hemichannels resulted in the development of human congenital cataracts.

  2. 先天性肝纤维化不同分型的临床特征--75例分析%Congenital hepatic fibrosis:clinical features of different clinical types in 75 patients

    Institute of Scientific and Technical Information of China (English)

    吴欣; 周超; 罗生强

    2014-01-01

    目的:比较病理证实为先天性肝纤维化(CHF)患者4种临床类型的临床、影像表现及病理特点。方法回顾性分析75例 CHF 患者的4种临床类型的临床和病理资料,并进行临床、影像表现与病理特点比较。结果75例患者中男44例,女31例,发病年龄2~55岁,平均年龄(19.88±11.59)岁。门脉高压型38例,其中脾大25例,肝大8例,腹水15例,食管静脉曲张20例,上消化道出血和黑便14例,合并 Caroli’s 病13例,肾脏病变13例;WBC、Hb、PLT 下降,肝功能正常。胆管炎型4例,无脾肿大、腹水、上消化道出血等症状,合并肾脏病变1例;血常规正常,ALT、AST、ALP、GGT 和LAP 明显升高,与隐匿型和门脉高压型相比,差异有统计学意义(P <0.05)。门脉高压和胆炎管混合型30例,其中脾大21例,肝大11例,腹水10例,食管静脉曲张14例,上消化道出血和黑便8例,合并 Caroli’s 病11例,肾脏病变10例,Hb 下降,与隐匿型相比差异有统计学意义(P <0.05),ALT、AST、ALP、GGT 和 LAP 升高,与隐匿型和门脉高压型相比,差异有统计学意义(P <0.05)。门脉高压型和混合型中 Hb 下降与上消化道出血呈负相关性。PAL 在各类型中差异大,差异有统计学意义(P <0.05)。结论在 CHF 中,肝脏合成功能正常。胆管炎型表现为慢性胆汁淤积性肝病特点,以肝功能异常及 ALP、GGT、LAP 等梗阻性酶谱升高为主,症状常不典型;门脉高压型是最常见类型,以门脉高压症及肝功能正常为特点;混合型在 CHF 中并不少见,其兼有胆管炎型和门脉高压1型两者的特点。隐匿型常无特征性表现。PAL 可能是一个有效判断 CHF 预后的指标。%Objective To describe the clinical,biochemical and histological characteristics in 75 patients with pathologically proved congenital hepatic fibrosis (CHF

  3. Expertise in Clinical Psychology. The Effects of University Training and Practical Experience on Expertise in Clinical Psychology

    OpenAIRE

    Vollmer, Sabine; Spada, Hans; Caspar, Franz; Burri, Salome

    2013-01-01

    How do university training and subsequent practical experience affect expertise in clinical psychology? To answer this question we developed methods to assess psychological knowledge and the competence to diagnose, construct case conceptualizations, and plan psychotherapeutic treatment: a knowledge test and short case studies in a first study, and a complex, dynamically evolving case study in the second study. In our cross-sectional studies, psychology students, trainees in a certified postgr...

  4. The clinical application of CT in congenital Wart disease%CT在先天性心脏病中的临床应用概况

    Institute of Scientific and Technical Information of China (English)

    司彪

    2011-01-01

    With the technical developmenr of compured tomography (CT) in software and hardware. CT was used for clinic more and more widely. The full text reviewed relevant literatures ro sum up the survey of clinical application of CT in' congénital heart disease.%随着计算机体层成像(computed tomography. CT)软硬件技术的发展.CT在临床中的应用越来越广泛.本文通过有关文献的复习.总结CT在先天性心脏病中的临床应用概况.

  5. Haemodynamic findings on cardiac CT in children with congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo [University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Asan Medical Center, Seoul (Korea, Republic of)

    2011-02-15

    In patients with congenital heart disease, haemodynamic findings demonstrated on cardiac CT might provide useful hints for understanding the haemodynamics of cardiac defects. In contrast to morphological features depicted on cardiac CT, such haemodynamic findings on cardiac CT have not been comprehensively reviewed in patients with congenital heart disease. This article describes normal haemodynamic phenomena of cardiovascular structures and various abnormal haemodynamic findings with their mechanisms and clinical significance on cardiac CT in patients with congenital heart disease. (orig.)

  6. Congenital diaphramatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Fetal Care Center of Cincinnati, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States)

    2012-01-15

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  7. Clinical observation different operative method in treatment of children with congenital blepharoptosis%不同手术方法治疗儿童先天性重度上睑下垂的临床观察

    Institute of Scientific and Technical Information of China (English)

    于亚东

    2012-01-01

    目的 观察不同的手术方式矫治儿童重度先天性上睑下垂的治疗效果.方法 36例47眼,应用额肌筋膜瓣悬吊术组18例24眼,自体阔筋膜悬吊术组18例23眼,比较两组术后疗效.结果 随访6个月,满意率比较两组无明显差异性(P>0.05).结论 两种方法各有优缺点,对治疗儿童重度上睑下垂均有效.%Objective To observe the clinical effect of different surgical treatment in children severe congenital blepharoptosis. Methods Totally 36 cases of 47 eyes,used forehead myofascial disc sling operation group 18 cases of 24eye,rich autologous fascia of sling operation 18 cases 23 eye,Compared postoperative curative effect of two groups. Results Follow - up 6 months,satisfaction rate was compared between the two groups has no obvious difference. Conclusions Two kinds of method have its advantages and disadvantages,to treating children severe blepharoptosis are valid.

  8. Congenital nasal lipoma

    International Nuclear Information System (INIS)

    The authors describe a rare case of congenital lipoma of a nose and nasopharynx in a 7 months old girl. The tumor, about 7 x 2 cm was situated in the right nasal cavity and the nasopharynx. The tumor caused complete obstruction of the right side of the nose. After CT diagnosis the tumor was excised from intranasal approach. Histological examination disclosed lipoma. The duration of follow up was 10 months without any sight of recurrence. (author)

  9. CONGENITAL ANTERIOR TIBIOFEMURAL SUBLUXATION

    Directory of Open Access Journals (Sweden)

    A. Shahla

    2008-06-01

    Full Text Available Congenital anterior tibiofemoral subluxation is an extremely rare disorder. All reported cases accompanied by other abnormalities and syndromes. A 16-year-old high school girl referred to us with bilateral anterior tibiofemoral subluxation as the knees were extended and reduced at more than 30 degrees flexion. Deformities were due to tightness of the iliotibial band and biceps femuris muscles and corrected by surgical release. Associated disorders included bilateral anterior shoulders dislocation, short metacarpals and metatarsals, and right calcaneuvalgus deformity.

  10. Congenital Triangular Alopecia

    OpenAIRE

    Yin Li, Vincent Chum; Yesudian, Paul Devakar

    2015-01-01

    Congenital triangular alopecia (CTA) also known as temporal triangular alopecia is a benign noncicatricial pattern of hair loss. It typically affects the frontotemporal region and rarely involves the temporoparietal or occipital scalp. It is a nonprogressive disorder that presents as a triangular, oval or lancet-shaped patch of alopecia. CTA can manifest at birth or develop later in life. The exact etiology of this condition remains unknown. Rarely, it may be associated with other disorders s...

  11. Congenital ocular motor apraxia

    OpenAIRE

    Carrasquinho, S; Teixeira, S.; Cadete, A; Bernardo, M.; Pêgo, P; Prieto, I.

    2008-01-01

    PURPOSE: Congenital ocular motor apraxia is a rare disease characterized by defective or absent voluntary and optically induced horizontal saccadic movements. Jerky head movements or thrusts on attempted lateral gaze are a compensatory sign. Most affected children have delayed motor and speech development. Cases associated with systemic diseases, neurologic maldevelopment, metabolic deficits, and chromosomal abnormalities have been described. METHODS: Case report and review of the scienti...

  12. Congenital syphilis surveillance

    OpenAIRE

    Antonella Marangoni; Alessandra Moroni; Elisabetta Tridapalli; Maria Grazia Capretti; Antonietta D’Antuono; Marina Biagi; Sanzio Ruscello; Franca Savioli; Roberto Cevenini

    2011-01-01

    Congenital syphilis (CS) is mainly a consequence of the lack of antenatal care and control of sexually transmitted infections.The bedrock of the prevention of CS is syphilis diagnosis by serological screening during pregnancy.Current Italian guidelines suggest that all the pregnant women should be tested in the first trimester. Due to the frequently absence of specific signs of infection at birth, laboratory tests are often the only method for a correct CS diagnosis. The aim of this study was...

  13. Mutations in CFTR gene and clinical correlation in Argentine patients with congenital bilateral absence of the vas deferens Correlación de las características clínicas con mutaciones del gen CFTR en pacientes argentinos con ausencia bilateral congénita de vasos deferentes

    OpenAIRE

    Levy, Estrella M; Patricia Granados; Vanesa Rawe; Santiago Brugo Olmedo; María C Luna; Eduardo Cafferata; Omar H Pivetta

    2004-01-01

    Congenital bilateral absence of the vas deferens (CBAVD) is a form of male infertility in which mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene have been identified. Here we identify different mutations of CFTR and the poly-T variant of intron 8 (IVS8) in Argentine patients and analyze sweat test values and clinical characteristic related to Cystic Fibrosis (CF). For counseling purposes the two most frequent mutations in Argentine CF population: DF508 and G542...

  14. Congenital portosystemic shunts with and without gastrointestinal bleeding - case series

    Energy Technology Data Exchange (ETDEWEB)

    Gong, Ying; Chen, Jun; Chen, Qi; Ji, Min; Pa, Mier; Qiao, Zhongwei [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China); Zhu, Hui [Fudan University Shanghai Cancer Center, Department of Radiology, Shanghai (China); Zheng, Shan [Children' s Hospital of Fudan University, Department of Surgery, Shanghai (China)

    2015-12-15

    The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation. To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5). We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt. A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts. Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high. (orig.)

  15. Congenital self-healing reticulohistiocytosis - an important diagnostic challenge

    DEFF Research Database (Denmark)

    Jensen, Marie Louise Slott; Bygum, Anette; Clemmensen, Ole;

    2011-01-01

    Aim:  To present current and new knowledge on congenital self-healing reticulohistiocytosis, a benign variant of cutaneous Langerhans cell histiocytosis presenting with skin lesions in the neonatal period. Methods:  We describe and photo document two cases of this rare disease and review the lite......Aim:  To present current and new knowledge on congenital self-healing reticulohistiocytosis, a benign variant of cutaneous Langerhans cell histiocytosis presenting with skin lesions in the neonatal period. Methods:  We describe and photo document two cases of this rare disease and review......, multidisciplinary assessment and follow up are essential. Conclusion:  Our data document how easily the diagnosis congenital self-healing reticulocytosis may be missed and emphasize the importance and value of instant clinical photographing at the neonatal unit and the use of teledermatology whenever congenital...

  16. Congenital lobar emphysema. A rare cause of hypertension.

    Science.gov (United States)

    Tural-Kara, Tuğçe; Özdemir, Halil; Çiftçi, Ergin; İnce, Erdal

    2016-07-01

    Congenital lobar emphysema is a rare disease, which is characterized by pulmoner hyperinflation. Depending on the degree of bronchial obstruction, the clinical presentation may be variable. We report a rare case with congenital lobar emphysema in a 38-days-old male infant who presented with severe respiratory distress and hypertension. Air trapping in the left upper lung and significant mediastinal shift to the right were observed on the chest x-ray. Emphysematous changes were detected on the thorax computed tomography and considered as congenital lobar emphysema. The upper left lobectomy was successfully performed by pediatric surgeons. On postoperative follow up, no sign of respiratory distress occured and the patient was normotensive. In this report, a case with congenital lobar emphysema, which is a rare cause of respiratory distress and hypertension is discussed. PMID:27381542

  17. CONGENITAL DISLOCATION OF RIGHT HIP JOINT: IMPORTANCE OF DYNAMIC ASSESSMENT

    Directory of Open Access Journals (Sweden)

    Pranita viveki

    2014-11-01

    Full Text Available Congenital Dislocation of Hip (CDH, is one of the most common congenital diseases in the orthopedic field. It is also known as Developmental Dysplasia of Hip. The condition can be diagnosed by clinical, ultrasonographic and radiological examination. Here we are reporting two days old male baby with congenital dislocation of right hip joint. The goal of treatment is to obtain a reduction to provide an optimal environment for femoral head and acetabular development. Early diagnosis is the most crucial aspect of the treatment of children with congenital dislocation of hip. If dislocation remains undiagnosed or neglected, the secondary pathological changes take place. Education of primary care colleagues, in making the diagnosis and prompt referral for management is recommended.

  18. Congenital adrenal hyperplasia: Case report.

    Directory of Open Access Journals (Sweden)

    Jaime Avaria E.

    2013-04-01

    Full Text Available INTRODUCTION: Congenital adrenal hyperplasia (CAH is an autosomal recessive disease whose main cause is the deficiency of 21-hydroxylase, an enzyme involved in the synthesis of cortisol and aldosterone. There are two forms of CAH, a classical and nonclassical form, being the first objective of analysis in the clinical case. Its clinical manifestations vary in severity, depending on the level of hormone deficiency. Within the classic is described the salt-wasting form, whose consequences are androgen excess and insufficiency of cortisol and mineralocorticoids. So this may manifest as a sex differentiation disorder (virilization of the external genitalia if the fetus is female and adrenal insufficiency. For diagnosis are considered the family history, clinical manifestations, measuring 17-hydroxyprogesterone levels and detection of genetic alteration. CASE REPORT: Patient with a family history of a brother with HSC brother, born with a disorder of sexual differentiation and is discharged with legal male sex. After three months develops adrenal insufficiency and was diagnosed with classical HSC salt-wasting form and determined female karyotype. DISCUSSION: The Pillars of the HSC are handling genetic counseling in families at risk, prenatal treatment with dexamethasone, postnatal glucocorticoid therapy and surgical treatment of disorders of the external genitalia, along with new research based therapy gene and the use of stem cells, requiring this way an integral view of HSC.

  19. CONGENITAL DUODENAL OBSTRUCTIONS

    Directory of Open Access Journals (Sweden)

    S.G. Aprodu

    2005-07-01

    Full Text Available The purpose of this study is to analyze a cohort of 46 cases of congenital duodenal obstruction, operated on between 1996 and 2002, 23 of them being diagnosed in neonatal period. In one case, the diagnosis was made antenatally, by ultrasonography. There were 15 males and 8 females, 17 with duodenal atresia and 6 with duodenal diaphragmatic stenosis. Surgery was performed in all cases, consisting in lateral duodeno-duodenal anastomosis in 5 cases and "diamond-shape" duodeno-duodenal anastomosis in 18 cases. The survival rate in this study was 69.5%. 12 cases (52,1% had other congenital pathologies: trisomy 21 (6 cases, multiple ileal atresia (2 cases, dextrocardy (2 cases, omphalocel (1 case, situs inversus (1 case. The complications of surgery were: anastomotic leaking with peritonitis, biliary fistula, intestional adhesions with occlusion. Congenital duodenal obstruction (midgut volvulus, atresia, stenosis remains a challenging issue for pediatric surgeons, especially in our country, due to limited possibilities of quick diagnosis and treatment of associated anomalies.

  20. Congenital myasthenic syndromes in childhood: diagnostic and management challenges.

    Science.gov (United States)

    Kinali, M; Beeson, D; Pitt, M C; Jungbluth, H; Simonds, A K; Aloysius, A; Cockerill, H; Davis, T; Palace, J; Manzur, A Y; Jimenez-Mallebrera, C; Sewry, C; Muntoni, F; Robb, S A

    2008-09-15

    The Congenital Myasthenic Syndromes (CMS), a group of heterogeneous genetic disorders of neuromuscular transmission, are often misdiagnosed as congenital muscular dystrophy (CMD) or myopathies and present particular management problems. We present our experience of 46 children with CMS, referred to us between 1992-2007 with provisional diagnoses of congenital myopathy (22/46), CMS or limb-girdle myasthenia (9/46), central hypotonia or neurometabolic disease (5/46), myasthenia gravis (4/46), limb-girdle or congenital muscular dystrophy (4/46) and SMA (2/46). Diagnosis was often considerably delayed (up to 18y4 m), despite the early symptoms in most cases. Diagnostic clues in the neonates were feeding difficulties (29/46), hypotonia with or without limb weakness (21/46), ptosis (19/46), respiratory insufficiency (12/46), contractures (4/46) and stridor (6/46). Twenty-five children had delayed motor milestones. Fatigability developed in 43 and a variable degree of ptosis was eventually present in 40. Over the period of the study, the mainstay of EMG diagnosis evolved from repetitive nerve stimulation to stimulation single fibre EMG. The patients were studied by several different operators. 66 EMGs were performed in 40 children, 29 showed a neuromuscular junction abnormality, 7 were myopathic, 2 had possible neurogenic changes and 28 were normal or inconclusive. A repetitive CMAP was detected in only one of seven children with a COLQ mutation and neither of the two children with Slow Channel Syndrome mutations. Mutations have been identified so far in 32/46 children: 10 RAPSN, 7 COLQ, 6 CHRNE, 7 DOK7, 1 CHRNA1 and 1 CHAT. 24 of 25 muscle biopsies showed myopathic changes with fibre size variation; 14 had type-1 fibre predominance. Three cases showed small type-1 fibres resembling fibre type disproportion, and four showed core-like lesions. No specific myopathic features were associated with any of the genes. Twenty children responded to Pyridostigmine treatment alone

  1. Clinical analysis of early complications in patients with severe pulmonary hypertension undergoing congenital heart surgery%先天性心脏病伴重度肺动脉高压术后早期并发症分析

    Institute of Scientific and Technical Information of China (English)

    张文波; 张希; 孙培吾; 殷胜利; 王治平; 唐白云

    2009-01-01

    Objective A clinical analysis of early complications in patients with severe pulmonary hypertension(PH)undergoing congenital heart surgery with cardiopulmonary bypass(CPB)is reported.Methods A retrospective study of 45 patients with severe PH undergoing congenital heart surgery with CPB in our hospital from June 2003 to June 2009 was completed.The cause of death and the major complications in the early postoperative period were discussed.Results The major complications were severe hypoxemia,anoxic spell and low cardiac output.The mortality was 6.67%.The causes of death were all low cardiac output,which manifested decompensation of right ventricular function.By multivariate regression,Hypothermic CPB(OR=0.56;95%CI 0.35-0.89)was a risk factor for severe hypoxemia.Conclusion Early postoperative complications were mainly due to impaired function of heart and lungs,which based upon postoperative PH.Selective postoperative monitoring for pulmonary arterial pressure and right heart function,and prolonged mechanical ventilation for high risk patients may help to guide treatment and to prevent and cure early complications in these patients.%目的 探讨先天性心脏病(CHD)伴重度肺动脉高压(PH)体外循环(CPB)术后早期并发症及处理.方法 对本科2003年6月至2009年6月行CPB手术的45例CHD伴重度PH患者进行回顾分析,讨论术后早期主要并发症和死亡原因.结果 术后早期主要并发症有严重低氧血症(13.33%)、缺氧发作(11.11%)、低心排(6.67%).住院死亡率6.67%,死亡原因均为低心排,主要为右心室功能失代偿.多因素分析显示,低温CPB(OR=0.56;95%CI 0.35-0.89)是严重低氧血症的一个危险因素.结论 术后早期并发症主要由术后PH为基础的心肺功能不全所导致,对术后高危患者动态监测肺动脉压、右心功能和延长呼吸机支持,有助于防治并发症.

  2. Clinical use of dexmedetomidine in pediatric patients with congenital heart disease%右美托咪啶在先天性心脏病患儿中的临床应用

    Institute of Scientific and Technical Information of China (English)

    陆海松; 王晓凑; 晏馥霞

    2011-01-01

    背景 右美托咪啶(dexmedetomidine,DEX)是新一代高选择性α2肾上腺素能受体激动剂,具有镇静、镇痛和抗交感作用.虽然DEX还未被批准用于18岁以下患者,但已有许多在小儿心脏外科使用的文献报道.目的 总结DEX在先天性心脏病(先心病)患儿中的临床应用近况.内容 除最常用于围术期重症监护治疗外,DEX在小儿心脏外科还可用于各种诊疗时的镇静、作为麻醉辅助用药、治疗快速型室上性心动过速和治疗阿片药及苯二氮萆类药物的撤药反应.趋向 DEX的安全范围较宽,对呼吸影响小,特别是对心肺功能严重受损的重症先心病患儿能发挥独特的优势.%Background Dexmedetomidine(DEX)is a novel highly selective α2-adrenoceptor agonist with sedative,analgesic,and anxiolytic effects.Although it has not been approved by the US FDA for pediatric patients under 18 years old,there are a lot of literatures focusing on its using in pediatric patients with congenital heart disease.Objective This paper discusses the application of DEX in children with congenital heart disease in detail.Content The clinical applications of DEX in cardiac surgery include sedative in pediatric intensive care unit,sedation during non-invasive and invasive procedures,as part of multimodal anesthetic regimen,treatment of supraventricular tachyarrhymias and prevention of drug withdrawal.Trend DEX is a safe chug while using in critically ill patients,with advantage of wide dose range and minimal respiratory depression.

  3. 先天性眼外肌纤维化一家系临床分析及手术治疗%Clinical features and surgical treatment on a family with congenital fibrosis of the extraocular muscles

    Institute of Scientific and Technical Information of China (English)

    张剑飞; 王亚丽; 陈静; 乔珊丽

    2014-01-01

    AIM: To investigate the clinical characteristics, surgical outcome and curative effect of congenital fibrosis of the extraocular muscles ( CFEOM) . METHODS: The eye exam of members in a Chinese family with CFEOM includes visual acuity, intraocular pressure, dilated fundus exam, extraocular muscle function test, orbital CT scan, and ultrasound. We did extraocular muscle surgery or frontalis suspension procedure for affected subjects in the family. RESULTS: The incidence of CFEOM in this family was 31%. All patients were affected bilateraly with symptom of congenital eye movement disorder, ptosis, hypotropia, perverted convergence on upgaze and chin up head position. As the age grows, the diseases worsen unobviously. No other systemic disorder was found. Surgical treatment improved the anomalous head position although the ocular movement disorder preserved. CONCLUSION: The pattern of inheritance in our serial patients are autosomal dominant. Surgery can improve chin up head position and cosmetic appearance. However, the eye movement deficiency cannot be improved.%目的:探讨家族性先天性眼外肌纤维化的临床特点、手术治疗方法及疗效。  方法:对先天性眼外肌纤维化家系成员进行眼部的各项检查,包括:视力、眼压、眼底、眼外肌功能、眼眶CT、双眼B超等检查,并对部分患者行斜视矫正术及额肌悬吊术。  结果:该家系眼外肌纤维化发病率为31%。该家系各患者均双眼受累,自幼表现为眼球运动障碍、上睑下垂,眼球位于下转位,向正前方注视时伴有异常辐辏,向前注视抬下颌。随年龄增长病情加重不明显。其他全身系统器官未见异常。经手术治疗,下颌上抬及外观可获得明显改善,眼球运动改善不明显。  结论:该家系具有常染色体显性遗传特征。通过手术治疗可改善头位及外观。眼球运动无明显改善。

  4. 早期干预对先天性甲状腺功能低下儿童的临床疗效观察%Clinical curative effect observation of early intervention on children with congenital hypothyroidism

    Institute of Scientific and Technical Information of China (English)

    钱晔

    2015-01-01

    Objective:To explore the influence for clinical curative effect of early intervention on children with congenital hypo-thyroidism.Methods:36 cases with congenital hypothyroidism were selected,they were randomly divided into the observation group and the control group with 18 cases in each,two groups were given regular treatment,the control group was given routine nursing, the observation group was given early nursing intervention,we compared the efficacy of the two groups.Results:In the observation group,for children at the age of one,MDI were (11.54 ± 10.5),PDI were (113.5 ± 9.6),height were (76.9 ± 3.4)cm,weight were (10.8±2.0)kg,head circumference were (47.5±2.1)cm,in the control group,for children at the age of one,MDI were (106.5±8.1), PDI were (105.3±8.5),height were (74.5±3)cm,weight were (9.5±1.5)kg,head circumference were (45.2±2)cm,the two groups had statistical significance(P<0.05).Conclusion:Early intervention can significantly improve the intelligence development in-dex(MDI),psychomotor development index(PDI),height,weight,head circumference,improve children's intellectual development and quality of life for children with congenital hypothyroidism.%目的:探讨早期干预对先天性甲状腺功能低下儿童临床疗效的影响。方法:收治先天性甲状腺功能低下患儿36例,随机分成观察组和对照组各18例,两组患儿均给予正规治疗,对照组给予常规护理,观察组进行早期护理干预,比较两组的疗效。结果:观察组患儿1岁时MDI(115.4±10.5),PDI(113.5±9.6),身高(76.9±3.4)cm,体重(10.8±2.0)kg,头围(47.5±2.1)cm;对照组患儿1岁时MDI(106.5±8.1),PDI(105.3±8.5),身高(74.5±3.0)cm,体重(9.5±1.5)kg,头围(45.2±2.0)cm;两组比较,差异有统计学意义(P<0.05)。结论:早期干预可以明显提高先天性甲状腺功能低下儿童智力发育指数(MDI)、心理运动发育指数(PDI)、身高、体重、头围,提高患儿的智力发育和生活质量。

  5. Congenital agenesis of seminal vesicle

    Institute of Scientific and Technical Information of China (English)

    Hong-Fei Wu; Di Qiao; Li-Xin Qian; Ning-Hong Song; Ning-Han Feng; Li-Xin Hua; Wei Zhang

    2005-01-01

    Congenital agenesis of the seminal vesicle (CASV) is frequently associated with congenital absence of the vas deferens (CAVD) or ipsilateral congenital vasoureteral communication. We reported two cases of a rare condition that the vas deferens open ectopically into Mullerian duct cyst associated with agenesis of the ipsilateral seminal vesicle. The diagnosis was confirmed by vasography. Transurethral unroofing of the Mullerian duct cyst was performed in both patients with favourable results, however, assisted reproductive technology (ART) was still necessary for them to father children.

  6. Evaluation of Adults With Congenital Heart Disease.

    Science.gov (United States)

    Graziani, Francesca; Delogu, Angelica Bibiana

    2016-03-01

    The clinical approach to adults with congenital heart diseases (ACHDs) is unique in cardiovascular medicine because these patients encompass a broad range of presentations. Each patient, despite having similar diagnosis, will be anatomically and physiologically unlike others within ACHD population, in relation to the type of repair, age at repair, associated defects, with specific long-term risk factors and complications. Furthermore, as many patients will not complain of symptoms, clinical evaluation and diagnostic testing must also be based on the underlying main diagnostic category, with complete standardized lesion-specific clinical protocols, investigating all known risk factors specific for each congenital heart disease and performed as part of screening for significant long-term complications. The first part of this review will focus on clinical history, physical examination, and the most important diagnostic testing in ACHD population. The second part of the article will focus on some clinical issues we have to face in our daily practice, such as heart failure, cyanosis, and pulmonary hypertension. Furthermore, as survival rates of ACHD population continue to improve and patients with this condition live longer, we will briefly report on a new clinical concern regarding the impact of acquired morbidities like coronary artery disease that appear to be of greater importance in defining outcome in older patients with ACHD. PMID:26957402

  7. A systematic review of evidence on the links between patient experience and clinical safety and effectiveness

    OpenAIRE

    Doyle, Cathal; Lennox, Laura; Bell, Derek

    2013-01-01

    Objective To explore evidence on the links between patient experience and clinical safety and effectiveness outcomes. Design Systematic review. Setting A wide range of settings within primary and secondary care including hospitals and primary care centres. Participants A wide range of demographic groups and age groups. Primary and secondary outcome measures A broad range of patient safety and clinical effectiveness outcomes including mortality, physical symptoms, length of stay and adherence ...

  8. Clinical Engeneering Experience at the Hospital of the State University of Londrina

    OpenAIRE

    Ernesto Fernando Ferreyra Ramírez

    2002-01-01

    This paper describes the four-year experience of implementation of Clinical Engineering services at the Hospital of the State University of Londrina (HURNP/UEL). It was performed by the Electrical Engineering Department (DEEL), through a project involving lecturers and students from the Electrical and Civil Engineering Courses of the same university. The main objectives were the formation of human resources in the Clinical Engineering area and a positive contribution to the healthcare service...

  9. Journey through the clinics - The experience of a woman with BRCA2.

    Science.gov (United States)

    Abernethy, Kathy

    2015-12-01

    Being told that you carry the gene abnormality for breast cancer is hard enough, then being told that you need surgery that will render you menopausal at a young age makes life even harder. Trying to navigate through the NHS, the genetics service, the gynaecology clinics, the gynaecology surgery, primary care services and finally menopause clinics has highlighted the need for cohesive and consistent advice for such women. This woman reports on her personal, generally positive, experience of this journey. PMID:26612439

  10. Practical Experience with Continuous Subcutaneous Insulin Infusion Therapy in a Pediatric Diabetes Clinic

    OpenAIRE

    O'Connell, Michele A.; Cameron, Fergus J.

    2008-01-01

    Continuous subcutaneous insulin infusion therapy (CSII) is an increasingly popular form of intensive insulin administration in pediatric patients. The use of CSII commenced at our large tertiary referral diabetes clinic as recently as 2002. In the intervening years, demand and enthusiasm from both patients and physicians alike have resulted in a steady ongoing increase in CSII use at our clinic. We currently have >200 active patients using insulin pump therapy. This article reviews our experi...

  11. Genetics Home Reference: critical congenital heart disease

    Science.gov (United States)

    ... Home Health Conditions critical congenital heart disease critical congenital heart disease Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Critical congenital heart disease (CCHD) is a term that refers to a ...

  12. Genetics Home Reference: severe congenital neutropenia

    Science.gov (United States)

    ... Home Health Conditions severe congenital neutropenia severe congenital neutropenia Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Severe congenital neutropenia is a condition that causes affected individuals to ...

  13. Genetics Home Reference: congenital leptin deficiency

    Science.gov (United States)

    ... Genetics Home Health Conditions congenital leptin deficiency congenital leptin deficiency Enable Javascript to view the expand/collapse ... Print All Open All Close All Description Congenital leptin deficiency is a condition that causes severe obesity ...

  14. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... Health Conditions Fukuyama congenital muscular dystrophy Fukuyama congenital muscular dystrophy Enable Javascript to view the expand/collapse boxes. ... All Open All Close All Description Fukuyama congenital muscular dystrophy is an inherited condition that predominantly affects the ...

  15. Congenital cystic lesions of lung in the paediatric population: A 5-year single institutional study with review of literature

    Directory of Open Access Journals (Sweden)

    Shibsankar Barman

    2015-01-01

    Full Text Available Background: The aim was to evaluate the clinical presentation, investigation modalities, operative management, pathology, outcome (morbidity and mortality and short term follow-up of congenital cystic lesions of the lung. Materials and Methods: This is a retrospective study. Study period was 5 years (December 2008-November 2013 in the Department of paediatric surgery, Nil Ratan Sircar Medical College. Study population: Total number = 10 patients. Age range: 2 days-7 years. (Neonate-4. Male and female ratio = 1:1. Result: Among 10 cases of lung cyst four having congenital lobar emphysema, four having congenital pulmonary airway malformation, one sequestration and one teratoma. All patients have undergone surgical excision in terms of lobectomy or excision of the lesion. Post-operative histopathology confirmed the diagnosis. Recovery was uneventful. Conclusion: Although our experience is limited, operative management of lung cysts seems to be safe with rewarding results. However we are yet to encounter many of the other varieties of the cysts found in the lung, which may be associated with other congenital anomalies and have an impact on prognosis.

  16. Congenital lobar emphysema: Pitfalls in diagnosis

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    Abhishek Chinya

    2016-01-01

    Full Text Available Congenital lobar emphysema (CLE is a rare but life-threatening congenital anomaly leading to respiratory distress in early childhood. Diagnosis requires a strong clinical suspicion. We report a case of a 31/2-month-old infant who was initially diagnosed with pneumonia requiring multiple hospital admissions. After computed tomography of the thorax, a diagnosis on CLE was made. The child was planned for surgery in the next available routine operation theatre. However, suddenly in the evening, she developed respiratory distress and needed emergency surgical intervention. The child improved dramatically after surgery, and the postoperative period was uneventful. Early diagnosis and treatment in such cases can lead to dramatic results.

  17. Congenital lobar emphysema: Pitfalls in diagnosis.

    Science.gov (United States)

    Chinya, Abhishek; Pandey, Prince Raj; Sinha, Shandip Kumar; Sarin, Yogesh Kumar

    2016-01-01

    Congenital lobar emphysema (CLE) is a rare but life-threatening congenital anomaly leading to respiratory distress in early childhood. Diagnosis requires a strong clinical suspicion. We report a case of a 31/2-month-old infant who was initially diagnosed with pneumonia requiring multiple hospital admissions. After computed tomography of the thorax, a diagnosis on CLE was made. The child was planned for surgery in the next available routine operation theatre. However, suddenly in the evening, she developed respiratory distress and needed emergency surgical intervention. The child improved dramatically after surgery, and the postoperative period was uneventful. Early diagnosis and treatment in such cases can lead to dramatic results. PMID:27185998

  18. CONGENITAL DYSERYTHROPOIETIC ANEMIA: AN ETIOPATHOLOGICAL STUDY

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    Vinod Kumar

    2015-12-01

    Full Text Available AIMS To evaluate the role of blood and bone marrow findings in the diagnosis of congenital dyserythropoietic anaemias type I and type II. SETTINGS AND DESIGN Dyserythropoietic anaemia is suspected when there is a suboptimal reticulocyte response for the degree of anaemia. Congenital dyserythropoetic anaemias are a group of rare hereditary disorders of hematopoiesis. It is believed that CDA is often under diagnosed. Knowledge of CDA workup in a patient of haemolytic anaemia is often rewarding. MATERIALS AND METHODS All the cases diagnosed as CDA at a tertiary paediatric centre were reviewed with respect to clinical and serological data, peripheral smear and bone marrow study findings. The haematological changes in CDA were analyzed with respect to the diagnosis. RESULTS AND CONCLUSION The light microscopic findings of the peripheral smear and bone marrow aspirate were found to be highly specific for the diagnosis of CDA. Significant differences were also noted between type I and type II CDA.

  19. Congenital epulides: A rare case report

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    Saurabh Kumar

    2014-01-01

    Full Text Available Congenital epulis (CE or "Granular cell epulis" also previously termed as "Neumann′s tumor" is a benign growth arising from the mucosa of the gingiva, typically seen as a mass protruding from the infant′s mouth, often interfering with respiration and feeding. These tumors generally present as a single mass arising from the upper alveolus. We report a rare case of two congenital epulides arising from the maxillary and mandibular alveolus in a day old female infant, which was surgically excised, allowing for early initiation of breast feeding. The tumor cells stained negative for S100 protein hence differentiating from other granular cell tumors. The clinical presentation, differential diagnosis in regard to the various neonatal oral swellings and the management of CE has been discussed.

  20. Congenital Malalignment of the Great Toenail.

    Science.gov (United States)

    Fierro-Arias, Leonel; Morales-Martínez, André; Zazueta-López, Rosa María; Ramírez-Dovala, Silvia; Bonifaz, Alexandro; Ponce-Olivera, Rosa María

    2015-01-01

    Congenital malalignment of the great toenail (CMA) is a disorder of the anatomic orientation of the ungual apparatus, in which the longitudinal axis of the nail plate is not parallel with the axis of the distal phalanx but is deflected sideways. This disorder is understood to arise from multiple factors. Although many theories have been proposed about its origin, its pathogenesis is not fully known. Besides the cosmetic impact, this disorder causes such problems in the medium and long term as onychocryptosis and difficulty in motion. Some cases may regress spontaneously, although persistent cases may require a specialized surgical approach. Congenital malalignment of the great toenail is poorly understood and described medical condition that is often treated incorrectly; thus, reviewing the subject is important. A symptombased clinical classification system is proposed to guide diagnosis and treatment modality decisions.

  1. Clinical Outcome and Safety of Multilevel Vertebroplasty: Clinical Experience and Results

    Energy Technology Data Exchange (ETDEWEB)

    Mailli, Leto, E-mail: lmailli@hotmail.com; Filippiadis, Dimitrios K.; Brountzos, Elias N.; Alexopoulou, Efthymia; Kelekis, Nikolaos; Kelekis, Alexios [Attikon University Hospital, Second Department of Radiology, Athens University School of Medicine (Greece)

    2013-02-15

    To compare safety and efficacy of percutaneous vertebroplasty (PVP) when treating up to three vertebrae or more than three vertebrae per session. We prospectively compared two groups of patients with symptomatic vertebral fractures who had no significant response to conservative therapy. Pathologic substrate included osteoporosis (n = 77), metastasis (n = 24), multiple myeloma (n = 13), hemangioma (n = 15), and lymphoma (n = 1). Group A patients (n = 94) underwent PVP of up to three treated vertebrae (n = 188). Group B patients (n = 36) underwent PVP with more than three treated vertebrae per session (n = 220). Decreased pain and improved mobility were recorded the day after surgery and at 12 and 24 months after surgery per clinical evaluation and the use of numeric visual scales (NVS): the Greek Brief Pain Inventory, a linear analogue self-assessment questionnaire, and a World Health Organization questionnaire. Group A presented with a mean pain score of 7.9 {+-} 1.1 NVS units before PVP, which decreased to 2.1 {+-} 1.6, 2.0 {+-} 1.5 and 2.0 {+-} 1.5 NVS units the day after surgery and at 12 and 24 months after surgery, respectively. Group B presented with a mean pain score of 8.1 {+-} 1.3 NVS units before PVP, which decreased to 2.2 {+-} 1.3, 2.0 {+-} 1.5, and 2.1 {+-} 1.6 NVS units the day after surgery and at 12 and 24 months after surgery, respectively. Overall pain decrease and mobility improvement throughout the follow-up period presented no statistical significance neither between the two groups nor between different underlying aetiology. Reported cement leakages presented no statistical significance between the two groups (p = 0.365). PVP is an efficient and safe technique for symptomatic vertebral fractures independently of the vertebrae number treated per session.

  2. Informed Practice: Students' Clinical Experiences in the Undergraduate Phase of an Accelerated Physician Assistant Program.

    Science.gov (United States)

    Dereczyk, Amy; DeWitt, Rachel

    2016-06-01

    This qualitative study explored the clinical experiences of students in an accelerated physician assistant (PA) program. The participants were either certified nursing assistants (CNAs) or emergency medical technicians-basic (EMTs-B). The study was designed to elicit (1) how the participants perceived their older patients and (2) how the participants' experiences might affect their own future communications, bedside manner, and clinical preparedness as PAs. This study used a focus group to explore students' clinical experiences before the graduate phase of their accelerated PA program. Five female and 2 male PA students (N = 7) participated in the study. All participants were 23 years old and worked as either a CNA or an EMT-B. Results fell into 2 basic themes: informing practice and forming relationships. Regarding the first theme, participants felt that their experience as entry-level health care providers allowed them to improve their communication skills and bedside manner and to provide greater comfort to patients. Regarding the second theme, participants gained appreciation for older people and began to recognize the knowledge deficits and learning needs of their patients. The results suggested that a student's clinical experience as a CNA or an EMT-B before entering a PA program has a positive effect on the student's personal and professional development. The participants acquired greater appreciation and respect for older patients and members of the health care team. PMID:27123599

  3. Use of the experience sampling method in the context of clinical trials

    Science.gov (United States)

    Verhagen, Simone J W; Hasmi, Laila; Drukker, Marjan; van Os, J; Delespaul, Philippe A E G

    2016-01-01

    Objective The experience sampling method (ESM) is a structured diary technique to appraise subjective experiences in daily life. It is applied in psychiatric patients, as well as in patients with somatic illness. Despite the potential of ESM assessment, the improved logistics and its increased administration in research, its use in clinical trials remains limited. This paper introduces ESM for clinical trials in psychiatry and beyond. Methods ESM is an ecologically valid method that yields a comprehensive view of an individual's daily life. It allows the assessment of various constructs (eg, quality of life, psychopathology) and psychological mechanisms (eg, stress-sensitivity, coping). These constructs are difficult to assess using cross-sectional questionnaires. ESM can be applied in treatment monitoring, as an ecological momentary intervention, in clinical trials, or in single case clinical trials. Technological advances (eg, smartphone applications) make its implementation easier. Results Advantages of ESM are highlighted and disadvantages are discussed. Furthermore, the ecological nature of ESM data and its consequences are explored, including the potential pitfalls of ambiguously formulated research questions and the specificities of ESM in statistical analyses. The last section focuses on ESM in relation to clinical trials and discusses its future use in optimising clinical decision-making. Conclusions ESM can be a valuable asset in clinical trial research and should be used more often to study the benefits of treatment in psychiatry and somatic health. PMID:27443678

  4. Clinical accompaniment: the critical care nursing students’ experiences in a private hospital

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    N. Tsele

    2000-09-01

    Full Text Available The quality of clinical accompaniment of the student enrolled for the post-basic diploma in Medical and Surgical Nursing Science: Critical Care Nursing (General is an important dimension of the educational/learning programme. The clinical accompanist/mentor is responsible for ensuring the student’s compliance with the clinical outcomes of the programme in accordance with the requirements laid down by the Nursing Education Institution and the South African Nursing Council. The purpose of this study was to explore and describe the experiences of the students enrolled for a post-basic diploma in Medical and Surgical Nursing Science: Critical Care Nursing (General, in relation to the clinical accompaniment in a private hospital in Gauteng. An exploratory, descriptive and phenomenological research design was utilised and individual interviews were conducted with the ten students in the research hospital. A content analysis was conducted and the results revealed both positive and negative experiences by the students in the internal and external worlds. The recommendations include the formulation of standards for clinical accompaniment of students. the evaluation of the quality of clinical accompaniment of students and empowerment of the organisation, clinical accompanists/mentors and clinicians.

  5. Updating Standard Procedures for Diagnosis and Treatment of Congenital Rubella Case

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    W. Buffolano

    2013-06-01

    Full Text Available Congenital Rubella is the dramatic consequence of rubella during gestation. A combined strategy of Measles and Rubella universal vaccination on children and selective vaccination of susceptible women has been shown effective in the elimination of congenital rubella requiring an incidence of <1 case of CRS per 100,000 live births. Verification processes of rubella elimination require that physicians early and appropriately diagnose all cases of congenital rubella, including those unpatent at birth. The paper highlights clinical and laboratory aspects channeling diagnosis of congenital rubella infection or syndrome even after the first year of life, and the short- and long-term management criteria.

  6. Molecular and Genetic Studies of Congenital Myopathies

    Science.gov (United States)

    2015-10-26

    Central Core Disease; Centronuclear Myopathy; Congenital Fiber Type Disproportion; Multiminicore Disease; Myotubular Myopathy; Nemaline Myopathy; Rigid Spine Muscular Dystrophy; Undefined Congenital Myopathy

  7. The South African Military Nursing College Pupil Enrolled Nurses’ experiences of the clinical learning environment

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    Ernestina M. Caka

    2013-06-01

    Full Text Available The study focused on the clinical learning experiences of Pupil Enrolled Nurses (PENs within the military health service. The purpose of the research was to explore and describe the learning experiences of PENs within the Military health clinical learning environment. An explorative, descriptive, contextual design which is qualitative in nature was used to guide the study. The military as a training institution prides itself on preparing nurses both as soldiers and nurses, this could be both challenging and exasperating for students, as the scopes are diverse. Being notably very hierarchical, the military’s rules constantly take precedence over nursing rules. For the duration of nursing training, students are allocated in the clinical learning area to acquire competencies such as problem solving, cognitive and psychomotor skills (Kuiper & Pesut 2003:383. Students learn how to merge theory and practice and apply theories in the practical sense. This is however, not done in isolation from the military codes, as they are intertwined. Attendance of military parades and drills are incorporated during this phase. This could create missed opportunities from the clinical learning as students are expected to leave the clinical setting for this purpose. Three focus group sessions were conducted and the experiences of the students, as narrated by themselves, yielded valuable insights. The researcher wrote field notes and assisted with the management of the audio tapes for easy retrieval of information. Data was analysed by the researcher, independent of the cocoder. Two themes relating to the PENs’ learning experiences emerged from the data analysed: (1 facilitators of clinical learning, (2 and barriers to clinical learning. The findings obtained depicted those factors which facilitated and obstructed student learning. These findings made it possible for the researcher to make recommendations concerning positive interventions which could be taken to

  8. Women's Management of Recurrent Bacterial Vaginosis and Experiences of Clinical Care: A Qualitative Study.

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    Jade Bilardi

    Full Text Available Few data are available on how women manage recurring bacterial vaginosis (BV and their experiences of the clinical care of this condition. This study aimed to explore women's recurrent BV management approaches and clinical care experiences, with a view to informing and improving the clinical management of BV.A descriptive, social constructionist approach was chosen as the framework for the study. Thirty-five women of varying sexual orientation who had experienced recurrent BV in the past 5 years took part in semi-structured interviews.The majority of women reported frustration and dissatisfaction with current treatment regimens and low levels of satisfaction with the clinical management of BV. Overall, women disliked taking antibiotics regularly, commonly experienced adverse side effects from treatment and felt frustrated at having symptoms recur quite quickly after treatment. Issues in clinical care included inconsistency in advice, misdiagnosis and inappropriate diagnostic approaches and insensitive or dismissive attitudes. Women were more inclined to report positive clinical experiences with sexual health physicians than primary care providers. Women's frustrations led most to try their own self-help remedies and lifestyle modifications in an attempt to treat symptoms and prevent recurrences, including well-known risk practices such as douching.In the face of considerable uncertainty about the cause of BV, high rates of recurrence, unacceptable treatment options and often insensitive and inconsistent clinical management, women are trying their own self-help remedies and lifestyle modifications to prevent recurrences, often with little effect. Clinical management of BV could be improved through the use of standardised diagnostic approaches, increased sensitivity and understanding of the impact of BV, and the provision of evidence based advice about known BV related risk factors.

  9. Phenotype-genotype discordance in congenital malformations with communication disorders resembling trisomy 18 (Edwards syndrome)

    OpenAIRE

    Pruszewicz, Antoni; Wiskirska-Woźnica, Bożena; Wojnowski, Waldemar; Czerniejewska, Hanna; Jackowska, Joanna; Jarmuż, Małgorzata; Szyfter, Krzysztof; Leszczyńska, Małgorzata

    2014-01-01

    Patient: Female, 6 Final Diagnosis: Phenotype-genotype discordance in congenital malformations with communication disorders resembling trisomy 18 (Edwards syndrome) Symptoms: — Medication: — Clinical Procedure: — Specialty: Otolaryngology Objective: Congenital defects Background: Communication process disorders are very frequent in rare cases of chromosomal aberrations (deletions, insertions, and trisomies) such as Down syndrome (trisomy 21), Turner syndrome, Edwards syndrome (trisomy 18), or...

  10. An Exploration of Dental Students' Assumptions About Community-Based Clinical Experiences.

    Science.gov (United States)

    Major, Nicole; McQuistan, Michelle R

    2016-03-01

    The aim of this study was to ascertain which assumptions dental students recalled feeling prior to beginning community-based clinical experiences and whether those assumptions were fulfilled or challenged. All fourth-year students at the University of Iowa College of Dentistry & Dental Clinics participate in community-based clinical experiences. At the completion of their rotations, they write a guided reflection paper detailing the assumptions they had prior to beginning their rotations and assessing the accuracy of their assumptions. For this qualitative descriptive study, the 218 papers from three classes (2011-13) were analyzed for common themes. The results showed that the students had a variety of assumptions about their rotations. They were apprehensive about working with challenging patients, performing procedures for which they had minimal experience, and working too slowly. In contrast, they looked forward to improving their clinical and patient management skills and knowledge. Other assumptions involved the site (e.g., the equipment/facility would be outdated; protocols/procedures would be similar to the dental school's). Upon reflection, students reported experiences that both fulfilled and challenged their assumptions. Some continued to feel apprehensive about treating certain patient populations, while others found it easier than anticipated. Students were able to treat multiple patients per day, which led to increased speed and patient management skills. However, some reported challenges with time management. Similarly, students were surprised to discover some clinics were new/updated although some had limited instruments and materials. Based on this study's findings about students' recalled assumptions and reflective experiences, educators should consider assessing and addressing their students' assumptions prior to beginning community-based dental education experiences. PMID:26933101

  11. Prevalence of Congenital Malformations

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    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  12. Congenital acute megakaryocytic leukemia

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    N B Mathur

    2011-01-01

    Full Text Available Congenital leukemia (CL is an extremely rare disorder in the newborn, significant proportion of which is of myeloid origin, primarily of M4 or M5 morphology. As compared to pediatric leukemia, CL is a more aggressive disease. Acute myeloid leukemia (AML-M7 or acute megakaryocytic leukemia is a rare type of AML with an incidence of 0.5 per million per year. Median age of presentation is 6 years, and children may present with a broad variety of symptoms including low-grade fever, diarrhea, easy bruising, failure to gain weight and life-threatening conditions.

  13. Congenital anterior urethral diverticulum.

    Science.gov (United States)

    Singh, Sanjeet Kumar; Ansari, Ms

    2014-09-01

    Congenital anterior urethral diverticulum (CAUD) may be found all along the anterior urethra and may present itself at any age, from infant to adult. Most children with this condition present with difficulty in initiating micturition, dribbling of urine, poor urinary stream, or urinary tract infection. A careful history will reveal that these children never had a good urinary stream since birth, and the telltale sign is a cystic swelling of the penile urethra. In this paper, we present two cases of CAUD that were managed by excision of the diverticulum with primary repair. PMID:26328174

  14. Managing congenital hyperinsulinism: improving outcomes with a multidisciplinary approach

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    Ackermann AM

    2015-07-01

    Full Text Available Amanda M Ackermann, Andrew A Palladino Division of Endocrinology and Diabetes, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Abstract: Congenital hyperinsulinism (CHI is the most common cause of persistent hypoglycemia in pediatric patients and is associated with significant risk of hypoglycemic seizures and developmental delays. CHI results from mutations in at least nine genes that play a role in regulating beta-cell insulin secretion. Thus, patients with CHI have dysregulated insulin secretion that is unresponsive to blood glucose level. Each different genetic etiology of CHI is associated with particular clinical characteristics that affect management decisions. Given the broad phenotypic spectrum and relatively rare prevalence of CHI, it is important that patients with CHI be evaluated by clinicians experienced with CHI and the multiple subspecialty services that are necessary for the management of the disorder. In this review, we summarize the pathophysiology and genetic causes of CHI and then focus primarily on the most common genetic cause (mutations in the ATP-gated potassium [KATP] channel for further discussion of diagnosis, medical and surgical management, and potential acute and chronic complications. We provide insight from relevant published studies and reports, in addition to anecdotal information from our center’s clinical experience in caring for over 400 patients with CHI. Careful assessment of each patient’s individual pathophysiology is necessary to determine the appropriate treatment regimen, and continued close follow-up and monitoring of disease- and treatment-related complications are essential. Although significant improvements have been made in the past several years with regard to diagnosis and management, given the continued high morbidity rate in patients with CHI, improved diagnostic techniques and new therapeutic options would be welcomed. Keywords: hypoglycemia, hyperinsulinism, beta

  15. Commissioning and initial experience with the first clinical gantry-mounted proton therapy system.

    Science.gov (United States)

    Zhao, Tianyu; Sun, Baozhou; Grantham, Kevin; Rankine, Leith; Cai, Bin; Goddu, Sreekrishna M; Santanam, Lakshmi; Knutson, Nels; Zhang, Tiezhi; Reilly, Michael; Bottani, Beth; Bradley, Jeffrey; Mutic, Sasa; Klein, Eric E

    2016-01-01

    The purpose of this study is to describe the comprehensive commissioning process and initial clinical experience of the Mevion S250 proton therapy system, a gantry-mounted, single-room proton therapy platform clinically implemented in the S. Lee Kling Proton Therapy Center at Barnes-Jewish Hospital in St. Louis, MO, USA. The Mevion S250 system integrates a compact synchrocyclotron with a C-inner gantry, an image guidance system and a 6D robotic couch into a beam delivery platform. We present our commissioning process and initial clinical experience, including i) CT calibration; ii) beam data acquisition and machine characteristics; iii) dosimetric commissioning of the treatment planning system; iv) validation through the Imaging and Radiation Oncology Core credentialing process, including irradiations on the spine, prostate, brain, and lung phantoms; v) evaluation of localization accuracy of the image guidance system; and vi) initial clinical experience. Clinically, the system operates well and has provided an excellent platform for the treatment of diseases with protons. PMID:27074470

  16. Major congenital defects. Relevant risk factors. Cienfuegos. 2000-2005.

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    Vivian Vázquez Martínez

    2008-04-01

    Full Text Available Background: Major congenital defects have an impact on perinatal mortality and morbidity with economic and social consequences. Objective: To determine the relevant risk factors linked to the occurrence of congenital defects in Cienfuegos between 2000 and 2005. Methods: A case-control study was carried out. Women with fetus or newborns with major congenital defects formed the case group (255 while the control group consisted of a similar number of healthy children selected each year by a randomized simple sample. The variables gathered by the Cuban Congenital Defects Records were studied. Chi-Square method was used with 95% of accuracy and a reliance rate of 95%; logistic regression was used with the Wald test. Results: There was a predominance of Central Nervous System defects (28, 6%. The most relevant risk factors were a history of congenital defects, smoking habits, and acute infection during the first three months, as well as belong to the third birth on. Conclusion: The most clinical relevant risks factors were the congenital defect history and the acute infection during the firs three months.

  17. Congenital Malformation Prevalence in Cluj District between 2003-2007

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    Ştefan I. ŢIGAN

    2009-12-01

    Full Text Available Introduction: Congenital anomalies represent a significant cause of premature birth, of child morbidity and mortality. From 200000 new born per year, over 10000 presented malformations. Epidemiologic studies have shown that the incidence of malformations is increasing and varies upon geographic features, race and gender. Perinatal mortality is generated in 66.66% of cases by congenital malformations, illnesses from perinatal period and the rest of them is generated by the birth. Material and Method: The study was retrospective and was carried on for a period of five years (2003-2007 based on medical records and on laboratory results, (especially those for TORCH screening: toxoplasmosis, rubella, cytomegalovirus, and herpes virus. Results: Major structural anomalies were present at 39.51% (388 cases of 982 patients which were registered in Genetic Pathology Center from Pediatric Clinics I, Cluj-Napoca. Diagnosed abnormalities included: congenital malformations of circulatory, respiratory, digestive, central nervous system, congenital malformations of skeletal system, Down syndrome, which is consistent with results of other studies showing that the most common are heart abnormalities (33.06%, followed in descending order of frequency by urinary, genital, CNS, skin, oral-facial cleft and digestive anomalies. Conclusions: Early detection of major malformation during early pregnancy can indicate for medical termination of pregnancy to reduce the high morbidity and mortality of neonates due to congenital malformations. So proper and timely counselling, regular antenatal care with folate supplementation especially during the most sensitive period of embryogenesis is essential to avoid major congenital malformation for future pregnancy.

  18. Perceptions of Co-Teaching in the Clinical Experience: How Well Is It Working?

    Science.gov (United States)

    Kinne, Lenore J.; Ryan, Carol; Faulkner, Shawn A.

    2016-01-01

    This study examined the perceptions of teacher candidates, cooperating teachers, and university supervisors in the first year of state-mandated co-teaching in the clinical experience. Study results suggest the need (a) to emphasize the importance of the teacher candidate exerting leadership, (b) to develop and communicate specific criteria for…

  19. Why Clinical Experience and Mentoring Are Replacing Student Teaching on the Best Campuses. A White Paper

    Science.gov (United States)

    Fraser, James W.; Watson, Audra M.

    2014-01-01

    Woodrow Wilson Senior Fellow James W. Fraser and Audra Watson, the Foundation's Director of Mentoring and Induction Strategy, take a look at emerging trends in clinical preparation for new teachers. This new white paper is based on experience with the Woodrow Wilson Teaching Fellowships, and includes observations from some of the colleges and…

  20. Using a Web-Based Database to Record and Monitor Athletic Training Students' Clinical Experiences

    Science.gov (United States)

    Brown, Kirk W.; Williams, Lisa; Janicki, Thomas

    2008-01-01

    Objective: The purpose of this article is to introduce a documentation recording system employing the Microsoft Structured Query Language (MS-SQL) database used by the Athletic Training Education Program (ATEP) for recording and monitoring of athletic training student (ATS) clinical experiences and hours. Background: Monitoring ATSs clinical…

  1. Preservice Teachers' Reflection on Clinical Experiences: A Comparison of Blog and Final Paper Assignments

    Science.gov (United States)

    Harland, Darci J.; Wondra, Joshua D.

    2011-01-01

    This study focused on the depth of reflection in the writing of preservice teachers who completed end-of-the-semester reflective papers or reflective blogs for undergraduate education courses associated with clinical experiences. Coders rated the depth of reflection as one of four categories: non-reflection, understanding, reflection, or critical…

  2. Clinical experience with mycophenolate mofetil in systemic autoimmune conditions refractory to common immunosuppressive therapies

    OpenAIRE

    Bandelier, Cédric; Guerne, Pierre-André; Genevay, Stéphane; Finckh, Axel; Gabay, Cem

    2009-01-01

    OBJECTIVES: Standard therapies against inflammatory rheumatic diseases consist of immunosuppressive drugs with high toxicities and many side effects. Except in the treatment of systemic lupus erythematosus with renal involvement, controlled studies with mycophenolate mofetil (MMF) are lacking in other autoimmune and inflammatory systemic diseases. Here we describe our clinical experience with MMF in several unusual indications. METHODS: We collected data including serological findings, advers...

  3. Comprehensive experiment-clinical biochemistry: determination of blood glucose and triglycerides in normal and diabetic rats.

    Science.gov (United States)

    Jiao, Li; Xiujuan, Shi; Juan, Wang; Song, Jia; Lei, Xu; Guotong, Xu; Lixia, Lu

    2015-01-01

    For second year medical students, we redesigned an original laboratory experiment and developed a combined research-teaching clinical biochemistry experiment. Using an established diabetic rat model to detect blood glucose and triglycerides, the students participate in the entire experimental process, which is not normally experienced during a standard clinical biochemistry exercise. The students are not only exposed to techniques and equipment but are also inspired to think more about the biochemical mechanisms of diseases. When linked with lecture topics about the metabolism of carbohydrates and lipids, the students obtain a better understanding of the relevance of abnormal metabolism in relation to diseases. Such understanding provides a solid foundation for the medical students' future research and for other clinical applications.

  4. Social justice as a framework for undergraduate community health clinical experiences in the United States.

    Science.gov (United States)

    Boutain, Doris M

    2008-01-01

    Educating future registered nurses for social justice is an urgent, yet complex undertaking in undergraduate education. Although the need for social justice education is often highlighted, few articles describe practical teaching strategies for ensuring that undertaking. The purpose of this article is to illustrate how a curricular focus on social justice framed and supported the development of a clinical evaluation tool for undergraduate community health clinical experiences. First, social justice is defined and its relationship to baccalaureate nursing education explained. Then a description is provided of how social justice was highlighted in the vision, curriculum, and community health clinical evaluation tool of a College of Nursing. The article subsequently showcases the content and evaluation of students' journal entries about social justice. The development of the social justice component presented in this article may be useful to nurse educators striving to match theory and practice in the evaluation of social justice in students' community health experience.

  5. Catarata congênita: aspectos diagnósticos, clínicos e cirúrgicos em pacientes submetidos a lensectomia Congenital cataract: diagnostic, clinic and surgical features in patients submitted to lensectomy

    Directory of Open Access Journals (Sweden)

    Márcia Lima Santos Oliveira

    2004-12-01

    Full Text Available OBJETIVO: Estabelecer o perfil clínico-terapêutico e resultados cirúrgicos de crianças submetidas a lensectomia. MÉTODOS: Estudo retrospectivo de 89 olhos operados de 62 crianças do Setor de Catarata Congênita do Departamento de Oftalmologia da UNIFESP-EPM. RESULTADOS: Entre as 62 crianças, 30,64% apresentaram causa infecciosa, 19,36% genética e 50% idiopática. Estrabismo foi encontrado em 54,83% das crianças e 27,42% apresentaram alterações sistêmicas. Quanto às condições gestacionais, 22,58% das mães não realizaram adequadamente o pré-natal. Consangüinidade foi observada em 17,74% dos casais e catarata congênita em 9,68% dos familiares. A suspeita diagnóstica foi realizada pelas mães em 72,58% dos casos, sendo leucocoria o principal sinal. A idade da suspeita diagnóstica variou de imediatamente após o nascimento até 15 meses (média = 1,34 meses, ao passo que a idade de atendimento no serviço variou de 8 dias a 20 meses (média = 5,84 meses. Opacidade secundária de eixo visual foi a principal complicação cirúrgica, ocorrendo em 19,1% dos olhos operados. Com relação à adesão ao tratamento pós-operatório, 45,16% apresentaram dificuldades quanto ao uso de oclusivo, óculos ou colírios. CONCLUSÃO: A rubéola persiste como uma das principais causas de catarata congênita em nosso meio, sendo necessário maior atenção às medidas preventivas A suspeita diagnóstica inicial da catarata é realizada principalmente pelas mães (72,58% e até os 3 meses de idade (93,44% período ideal para a recuperação visual. Há, porém, demora considerável entre a suspeita e o atendimento no serviço especializado. A realização tardia da cirurgia, juntamente com insatisfatória adesão ao tratamento pós-cirúrgico, compromete os resultados visuais finais.PURPOSE: To analyze the clinical and therapeutic profiles of children with congenital cataract submitted to lensectomy. METHODS: Retrospective study of 89 eyes of 62

  6. Trastornos congénitos de la glicosilación de las proteínas: patogenia y aspectos clínicos Congenital disorders of glycosylation of proteins. Pathogeny and clinical features

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    N. Higuera

    2011-01-01

    Full Text Available Objetivo. Este trabajo se propone actualizar los conocimientos a cerca de los trastornos congénitos de la glicosilación de las proteínas (CDG, en los aspectos referentes a la patogenia y manifestaciones clínicas. Desarrollo. Realizamos una revisión de la literatura considerandola evolución histórica de estas enfermedades, las bases bioquímicas y genéticas que permiten una clasificación, así como las manifestaciones clínicas; se hace especial hincapié en la descripción de la variante CDG Ia, la forma más frecuente, de la que se describen las tres etapas evolutivas: infantil multisistémica, infantil tardía y del adulto; de forma más breve se comentan las características de las variantes CDG Ib y CDG Ic. Conclusiones. Los CDG constituyen una patología emergente que dada su heterogeneidad clínica debe sospecharse en todo paciente con un cuadro neurológico inexplicable, en particular si junto a retraso psicomotor, hipotonía y epilepsia, asocia alteraciones hepáticas o de la coagulación, así como en casos de hipoplasia cerebelosa u olivo pontocerebelosa de aparición en el período neonatal. Objective. This work aims to provide an up-date on the knowledge regarding congenital disorders of glycosylation (CDG of proteins in aspects on pathogeny and clinical manifestations. Development. We performed a review of the literature, considering the historical course of these diseases, biochemical and genetic bases that permit their classification and the clinical manifestations. Special emphasis is placed on the description of the CDG Ia variant, the most frequent form, describing the three evolutive states: infantile multisystem late-infantile and adult. Briefly, we comment on the characteristics of the CDG Ib and CDG Ic variants. Conclusions. CDG is an emerging disease that, given its clinical heterogeneity, should be suspected in all patients with an unexplainable neurological picture, especially if it is accompanied by psychomotor

  7. Deficiencia congénita de complemento: C3 y C4: Comunicación de un caso clínico Congenital deficiency of the C3 and C4 fractions of complement: A clinical report

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    ALEXIS STRICKLER P

    2006-06-01

    Full Text Available La deficiencia congénita del 3er constituyente del Complemento (C3 es extremadamente rara, y se expresa clínicamente como un defecto de la inmunidad humoral. Se comunica un caso de deficiencia C3 y C4 en un lactante de sexo femenino de 1 año de edad, hijo de padres consanguíneos, que presentó un cuadro de meningoencefalitis aguda de etiología no precisada, con secuela neurológica severa e infecciones bacterianas recurrentes, respiratorias y urinarias, septicemia y osteomielitis, con respuesta parcial a antimicrobianos. El estudio de inmunidad humoral y celular (subpoblaciones linfocitarias, inmunoglobulinas séricas y subclases de IgG fue normal, demostrándose déficit de C3 y C4 con CH50 ausente en la niña y cifras bajas de C3 y C4, cercanas al 50% del valor normal en ambos padresCongenital deficiency of C3 fraction of the complement is a very rare condition. Clinically it is expressed as a deficiency of the humoral immunity. We report a case of C3 and C4 deficiency in a 1 year old infant girl. Her parents have a high consanguinity. She presented an acute meningoencephalitis of unknown etiology, and she evolved with severe neurological damage, and recurrent respiratory and urinary bacterial infections, sepsis and osteomielitis, with partial response to antimicrobials. The tests to investigate humoral and cellular immune response (lymphocyte subpopulations, serum immunoglobulins and subtypes of IgG were normal. The patient had a deficit of C3 and C4, mainly C3, with absence of CH50. Both of her parents had C3 and C4 about 50% of normal values, and CH50 slightly under the normal values

  8. [Genetics of congenital deafness].

    Science.gov (United States)

    Faundes, Víctor; Pardo, Rosa Andrea; Castillo Taucher, Silvia

    2012-10-20

    Congenital deafness is defined as the hearing loss which is present at birth and, consequently, before speech development. It is the most prevalent sensor neural disorder in developed countries, and its incidence is estimated between 1-3 children per 1,000 newborns, of which more than 50% are attributable to genetics causes. Deafness can be classified as syndromic or non-syndromic. In the first case, it is associated with outer ear malformations and/or systemic findings. More than 400 syndromes accompanied of deafness have been described, which represent about 30% of cases of congenital hearing loss. The remaining percentage corresponds to non-syndromic cases: 75-85% are autosomal recessive, 15-24% are autosomal dominant, and 1-2% are X-linked. The evaluation of a child with deafness requires a multidisciplinary collaboration among specialists, who must coordinate themselves and give information to the affected family. The aims of establishing a diagnosis are to predict other manifestations that may suggest some syndrome and to anticipate their management, as well as to perform genetic counseling to parents and affected individuals.

  9. The experience of graduated midwifery students about clinical education: A phenomenological study

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    R. Shahoei

    2013-06-01

    Full Text Available Introduction: Improvement and promotion of the quality of clinical education requires continuous assessment of the current situation, and also identifying the strengths and weaknesses. Students' views and ideas as learner can help future planning. This study aims to identify the experiences of midwifery graduates about factors affecting their clinical learning. Methods: A qualitative study using phenomenology approach was conducted. Ten midwifery graduates were selected based on purposive sampling and then interviewed. Data were analyzed by thematic analysis. Results: The extracted conceptual codes were classified into several main concepts. There were two main themes factors facilitating learning and factors preventing learning, and seven sub themes performance of instructor, pre-clinical training, students satisfaction, lack of peripheral facilities, lack of coordination of educational planning and behaviors of health care personnel. Conclusion: Trained human resources and equipment for midwifery educational planning are needed to provide a supportive learning atmosphere and promote the quality of clinical learning.

  10. Congenital Unilateral Hypoplasia of Depressor Anguli Oris

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    Seckin O. Ulualp

    2012-01-01

    Full Text Available Objectives. Asymmetric facial appearance may originate from abnormalities of facial musculature or facial innervation. We describe clinical features of congenital hypoplasia of depressor anguli oris muscle in a child. Material and Methods. Chart of a 10-month-old female referred to a tertiary care pediatric hospital for assessment of facial paralysis was reviewed. Data included relevant history and physical examination, diagnostic work up, and management. Results. The child presented with asymmetric movement of lower lip since birth. Asymmetry of lower lip was more pronounced when she smiled and cried. Rest of the face movement was symmetric. On examination, the face appeared symmetric at rest. The child had inward deviation of right lower lip when she smiled. Facial nerve function, as determined by frowning/forehead, wrinkling, eye closure, nasolabial fold depth, and tearing, was symmetric. Magnetic resonance imaging of the temporal bones and internal auditory canals were within normal limits. Echocardiogram did not show cardiac abnormality. Auditory brainstem response showed no abnormality. Conclusions. Congenital hypoplasia of depressor anguli oris is a rare anomaly that causes asymmetric crying face. Pediatricians and otolaryngologists need to be cognizant of cardiac, head and neck, and central nervous system anomalies associated with congenital unilateral hypoplasia of depressor anguli oris.

  11. Belongingness: a montage of nursing students' stories of their clinical placement experiences.

    Science.gov (United States)

    Levett-Jones, Tracy; Lathlean, Judith; McMillan, Margaret; Higgins, Isabel

    2007-04-01

    The psychological and social sciences literature is replete with assertions that human beings are fundamentally and pervasively motivated by the need to belong. This paper reports on some of the findings from the qualitative phase of a mixed-method, multi-site study that explored nursing students' experience of belongingness while on clinical placements. Students from Australia and the United Kingdom were interviewed to identify factors that impact upon and are consequences of belongingness. A montage of participants' stories is used to illustrate some of the key features of clinical workplaces that are conducive to the development of belongingness. Contextual factors and interpersonal dynamics were seen to have a significant bearing on students' experiences. Clinical leaders/managers who were welcoming, accepting and supportive, and nursing staff who were inclusive and encouraging, facilitated students' perception of being valued and respected as members of the nursing team. Additionally, the provision of consistent, quality mentorship was identified as important to students' feelings of connectedness and fit. The experience of belongingness, in turn, enhanced students' potential for learning and influenced their future career decisions. Alternatively, alienation resulted from unreceptive and unwelcoming clinical environments and from the dissonance created when students' personal and professional values did not articulate with those evident in practice environments. Consequently, distress, detachment and disengagement occurred and students' capacity and motivation for learning was negatively impacted. PMID:17563325

  12. 女性生殖管道发育异常225例临床分析%Clinical analysis of 225 women with congenital uterine malformation

    Institute of Scientific and Technical Information of China (English)

    王世军; Mandakini Oli; 蒋励; 王建六; 魏丽惠

    2008-01-01

    目的 探讨子宫发育异常的发病情况、临床特征、诊治方法及其对生育的影响.方法 选取1990年3月-2005年1月北京大学人民医院收治的子宫发育异常患者225例的临床资料,进行回顾性分析,总结不同类型子宫发育异常的构成比、临床特征、诊治方法及对生育的影响.结果 (1)225例子宫发育异常患者中,中隔子宫125例,占55.6%,其中122例(97.6%,122/125)患者妊娠,但影响其妊娠结局,出现流产、胎位异常、早产、前置胎盘、胎膜早破等并发症.(2)双子宫51例,占22.7%(51/225);其中50例(98%,50/51)患者妊娠,对妊娠无明显影响;5例(10%,5/51)合并泌尿系统畸形.(3)残角子宫26例(11.6%,26/225),发生残角子宫妊娠4例(15%,4/26),残角子宫同侧输卵管妊娠1例(4%,1/26);2例(8%,2/26)合并泌尿系统畸形;合并不孕4例(15%,4/26).(4)双角子宫14例,占6.2%(14/225),无不孕患者,出现早产、胎膜早破等并发症;1例(7%,1/14)合并泌尿系统畸形.(5)其他类型:鞍状子宫4例(1.8%,4/225),无不孕患者;无子宫3例(1.3%,3/225),均以原发闭经就诊;单角子宫2例(0.9%,2/225),均合并不孕.结论 子宫发育异常中以中隔子宫最多见.中隔子宫、双子宫、双角子官、残角子宫、鞍状子宫对生育无明显影响,但在妊娠期可出现不同的并发症.子宫发育异常常合并泌尿系统畸形.无症状的子宫发育异常,可不予处理.%Objective To investigate the prevalence, clinical features, diagnosis, treatment and the influence on procreation of uterine malformation. Methods Totally 225 women with uterine malformation referred to our hospital from Mar 1990 to Jan 2005 were involved in this retrospective analysis.The constituent ratio, clinical feature, diagnosis, treatment and the influence on procreation were analyzed.Results (1) Among 225 cases of uterine malformation, 125 cases (55.6%) were septate uterus; 122(97.6%, 122/125 ) of these patients became pregnant, but

  13. Genetic counseling in the adult with congenital heart disease: what is the role?

    Science.gov (United States)

    Burchill, Luke; Greenway, Steven; Silversides, Candice K; Mital, Seema

    2011-08-01

    New discoveries using high-resolution methods for detecting genetic aberrations indicate that the genetic contribution to congenital heart disease has been significantly underestimated in the past. DNA diagnostics have become more accessible and genetic test results are increasingly being used to guide clinical management. Adult congenital heart disease specialists seeking to counsel adults with congenital heart disease about the genetic aspects of their condition face the challenge of keeping abreast of new genetic techniques and discoveries. The emphasis of this review is on the genetic basis of structural cardiovascular defects. A framework for identifying adult congenital heart disease patients most likely to benefit from genetic testing is suggested, along with a summary of current techniques for genetic testing. The clinical and ethical challenges associated with genetic counseling are highlighted. Finally, emerging technologies and future directions in genetics and adult congenital heart disease are discussed.

  14. Evaluation of pharmacy students’ clinical interventions on a general medicine practice experience

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    Jones JD

    2011-03-01

    Full Text Available As colleges of pharmacy prepare a new generation of practitioners, it is important that during practice experiences students learn the impact of clinical interventions. For over ten years, pharmacy students have been a vital part of the multidisciplinary team at the military treatment facility. The overall impact of the student interventions on patient care has not been evaluated. To evaluate the impact, the students began documenting their clinical interventions in Medkeeper RxInterventions™, an online database. The program is used to document faculty and fourth year pharmacy students’ pharmaceutical interventions.Objective: The objective of this study was to analyze the interventions completed by fourth year pharmacy students during a general medicine advanced pharmacy practice experience at a military treatment facility.Methods: The students completing their general medicine advanced pharmacy practice experience at the military treatment facility are responsible for self reporting all interventions made during clinical rounds into the Medkeeper RxIntervention™ database. The researchers retrospectively collected and analyzed interventions made from June 2008 to June 2009.Results: The total number of interventions recorded by 8 fourth year pharmacy students was 114. Students averaged a number of 14.3 interventions during an eight week practice experience. Students spent an average of 5 minutes per intervention. Ninety- five percent of the interventions were accepted.Conclusion: Fourth year pharmacy students’ recommendations were accepted at a high rate by resident physicians. The high acceptance rate may have the ability to positively impact patient care.

  15. Optically-guided frameless linac-based radiosurgery for brain metastases: clinical experience

    OpenAIRE

    Nath, Sameer K; Lawson, Joshua D.; Wang, Jia-Zhu; Simpson, Daniel R.; Newman, C. Benjamin; Alksne, John F.; Mundt, Arno J.; Murphy, Kevin T.

    2009-01-01

    The purpose of this study was to describe our clinical experience using optically-guided linear accelerator (linac)-based frameless stereotactic radiosurgery (SRS) for the treatment of brain metastases. Sixty-five patients (204 lesions) were treated between 2005 and 2008 with frameless SRS using an optically-guided bite-block system. Patients had a median of 2 lesions (range, 1–13). Prescription dose ranged from 14 to 22 Gy (median, 18 Gy) and was given in a single fraction. Clinical and radi...

  16. Clinical Engeneering Experience at the Hospital of the State University of Londrina

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    Ernesto Fernando Ferreyra Ramírez

    2002-01-01

    Full Text Available This paper describes the four-year experience of implementation of Clinical Engineering services at the Hospital of the State University of Londrina (HURNP/UEL. It was performed by the Electrical Engineering Department (DEEL, through a project involving lecturers and students from the Electrical and Civil Engineering Courses of the same university. The main objectives were the formation of human resources in the Clinical Engineering area and a positive contribution to the healthcare services offered by the HURNP for the community in the surroundings of Londrina – Paraná State – Brazil.

  17. Clinical effects of breast implant on congenital mammary dysplasia with mild pigeon chest deformity%先天乳腺不发育伴轻度鸡胸行隆乳术治疗的临床效果评价

    Institute of Scientific and Technical Information of China (English)

    许柯; 刘飞; 郭正东; 林海波; 杨卫国; 洪浩; 梁智

    2010-01-01

    Objective To evaluate the clinical effect of breast implant on congenital mammary dysplasia with mild pigeon chest deformity. Methods From January 2003 to July 2009,10 cases of female mammary dysplasia (between 20 to 31 years of age) underwent breast implant surgery. Subpectoral placement and transaxillary incision were selected. The surgeon marked the range of the operation on the skin, made a 3-4 cm incision in the armpit, separated the tissue until the pectoral lateral margin, cut the pecto-ralis fascia and bluntly created a suitable pocket under the pectoralis major for the implant. After the implant was placed in the pocket, the incision was closed. Results Ten cases of breast implant surgery did not pose the complications of local skin necrosis, infection, implant shift, heart and lungs dysfunction after one year follow-up. The appearance of anterior chest wall deformity was markedly improved. Conclusions The application of breast implant surgery in the treatment of congenital mammary dysplasia with mild pigeon chest deformity should be promoted, because of the double surgical effect of easy performing, minimal surgical damage, perfect breast shape and concealed deformity.%目的 评价隆乳术治疗先天乳腺不发育、乳腺发育不良及胸大肌发育不良伴轻度鸡胸畸形临床效果.方法 对10例先天乳腺不发育、乳腺发育不良及胸大肌发育不良伴轻度鸡胸的女性行隆乳术.先在体表标出剥离范围及畸形部位,经双腋窝切口,直达皮下组织,向内分离至胸大肌外侧缘,于胸大肌后间隙,置入硅凝胶乳房假体.结果 10例于隆乳术后,随访1年,均未见发生局部感染、假体移位、心肺功能异常等并发症,前胸壁鸡胸畸形外观有明显改善.结论 应用隆乳术治疗先天性乳腺不发育、乳腺发育不良及胸大肌发育不良并伴有轻度鸡胸,操作方便、手术损伤小、效果满意.既美化乳房外观又掩饰了鸡胸畸形,具有双重手术效果,值得推广.

  18. 海南省新生儿先天性甲状腺功能减低症筛查情况及治疗分析%Screening and clinical analysis of neonates with congenital hypothyroidism in Hainan province

    Institute of Scientific and Technical Information of China (English)

    黄晓燕; 黄兹丹; 向伟

    2012-01-01

    Objective To summarize the screening results of neonates with congenital hypothyroidism (CH) in Hainan province from Jan. 2010 to Dec. 2011, and to analyze the clinical efficacy of the treatment. Methods The screening procedure was based on the determination of thyroid stimulating hormone (TSH) in dried blood spots obtained by heel puncture 72 hours after birth. Neonates with CH were treated with levothyroxine (L-T4). The thyroid function, condition of growth and intelligence status were investigated to evaluate the efficacy of therapy during the follow-up. Results A total of 158 524 neonates were screened, of which 1 570 positive cases were called back for diagnosis test and fifty cases of CH were confirmed with an average incidence rate of 1/3 170. During the follow-up, the thyroid function, growing development and intelligence of the forty-three patients who insisted on treatment had gone back to normal. Conclusion Neonates with CH confirmed by neonatal screening have chance of receiving early treatment and achieving satisfactory prognosis. Therefore, neonates screening and follow-up study are worthy to be improved and popularized.%目的 总结海南省2010-2011年先天性甲状腺功能减低症(Congenital hypothyroidism,CH)的筛查情况及治疗情况.方法 采集出生72 h新生儿158 524例的足跟血滴于,滤纸上,采用时间分辨荧光免疫测试法测定促甲状腺素(TSH),召回阳性者进一步测定静脉血甲状腺功能,明确诊断.确诊者立即开始给予左旋甲状腺素片替代治疗,定期检测甲状腺功能,并进行生长发育评估及智力测试,以评估疗效.结果 初筛阳性患儿1507例,确诊先天性甲状腺功能减低症50例,两年来发病率为1/3170.其中43例坚持治疗随访中,目前监测甲状腺功能正常,体格生长发育及智力测试均与正常同年龄小儿相当.结论 新生儿筛查可使先天性甲状腺减低症患儿尽早得到有效治疗,改善预后.因此应加大新生

  19. Systematic reviews of animal experiments demonstrate poor human clinical and toxicological utility.

    Science.gov (United States)

    Knight, Andrew

    2007-12-01

    The assumption that animal models are reasonably predictive of human outcomes provides the basis for their widespread use in toxicity testing and in biomedical research aimed at developing cures for human diseases. To investigate the validity of this assumption, the comprehensive Scopus biomedical bibliographic databases were searched for published systematic reviews of the human clinical or toxicological utility of animal experiments. In 20 reviews in which clinical utility was examined, the authors concluded that animal models were either significantly useful in contributing to the development of clinical interventions, or were substantially consistent with clinical outcomes, in only two cases, one of which was contentious. These included reviews of the clinical utility of experiments expected by ethics committees to lead to medical advances, of highly-cited experiments published in major journals, and of chimpanzee experiments--those involving the species considered most likely to be predictive of human outcomes. Seven additional reviews failed to clearly demonstrate utility in predicting human toxicological outcomes, such as carcinogenicity and teratogenicity. Consequently, animal data may not generally be assumed to be substantially useful for these purposes. Possible causes include interspecies differences, the distortion of outcomes arising from experimental environments and protocols, and the poor methodological quality of many animal experiments, which was evident in at least 11 reviews. No reviews existed in which the majority of animal experiments were of good methodological quality. Whilst the effects of some of these problems might be minimised with concerted effort (given their widespread prevalence), the limitations resulting from interspecies differences are likely to be technically and theoretically impossible to overcome. Non-animal models are generally required to pass formal scientific validation prior to their regulatory acceptance. In contrast

  20. Experimenting clinical pathways in general practice: a focus group investigation with Italian general practitioners

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    Lucia Zannini

    2012-07-01

    Full Text Available Background. Clinical governance is considered crucial in primary care. Since 2005, clinical pathways have been experimentally implemented at the Local Health Authority of Monza Brianza (ASLMB, Italy, to develop general practitioners’ (GPs care of patients affected by some chronic diseases. The experimentation was aimed at introducing clinical governance in primary care, increasing GPs’ involvement in the care of their patients, and improving both patients’ and professionals’ satisfaction. In the period 2005-2006, 12% of the 763 employed GPs in the ASLMB were involved in the experiment, while this percentage increased to 15-20% in 2007-2008. Design and Methods. Twenty-four GPs were purposively sampled, randomly divided into two groups and asked to participate in focus groups (FGs held in 2008, aimed at evaluating their perception of the experiment. The FGs were audio-recorded, dialogues were typed out and undergone to a thematic analysis, according to the Interpretative Phenomenological Approach. Results. Four major themes emerged: i clinical pathways can result in GPs working in a more efficient and effective fashion; ii they can assure higher levels of both patient and professional satisfaction, since they sustain a caring approach and strengthen the GPs’ role; iii nevertheless, clinical pathways increase the bureaucratic workload and problems can arise in relationships among GPs and the LHA; iv the implementation of clinical pathways can be improved, especially by reducing bureaucracy and by assuring their continuity. Conclusions. Managerial aspects should be considered with care in order to experimentally introduce clinical pathways in general practice, and continuity of the experimentation should be guaranteed to improve GPs’ adherence and commitment.

  1. Early Visual Deprivation Severely Compromises the Auditory Sense of Space in Congenitally Blind Children

    Science.gov (United States)

    Vercillo, Tiziana; Burr, David; Gori, Monica

    2016-01-01

    A recent study has shown that congenitally blind adults, who have never had visual experience, are impaired on an auditory spatial bisection task (Gori, Sandini, Martinoli, & Burr, 2014). In this study we investigated how thresholds for auditory spatial bisection and auditory discrimination develop with age in sighted and congenitally blind…

  2. The clinical assessment and surgical treatment of congenital scoliosis combined with the Sprengel's deformity%先天性脊柱侧凸伴高肩胛症的临床评估与手术

    Institute of Scientific and Technical Information of China (English)

    汪飞; 钱邦平; 邱勇

    2012-01-01

    @@ 前言 先天性脊柱侧凸(congenital scoliosis,CS)伴高肩胛症(Congenital elelvation of the scapula,Sprengel'sdeformity)是一种少见而复杂的先天性发育畸形.Tsirikos等[1]分析了537例先天性脊柱畸形患者的肋骨、胸廓及肩胛畸形,在497例CS患者中43例(8.6%)合并高肩胛畸形.Cavendish等[2]报道的100例先天性高肩胛症患者中,有39例(39%)伴CS;与单纯的先天性高肩胛症或CS相比,CS伴高肩胛症的临床评估及手术策略更加复杂,现对这种复杂畸形的临床评估与手术策略作一综述.%Congenital scoliosis (CS) combined with the Sprengel's deformity was a rare and complicated congenital deformity. Although the pathogenesis of this complicated deformity was still unclear, some existing studies revealed that Sprengel's deformity occurred most frequently in congenital scoliosis with unilateral failure of vertebral segmentation in cervicothoracic or thoracic segments. According to the relationship between the congenital scoliosis and the Sprengel's deformity, the complicated congenital deformity could be divided into 3 types: the elevated shoulder occurred on the convex side of the scoliosis, on the concave side of the scoliosis and CS combined with bilateral Sprengel's deformity. A thorough physical examination and comprehensive using of three-dimensional computed tomography (CT) and magnetic resonance imaging (MRI) were very important for the assessment of this complicated deformity. The Sprengel's deformity occurred on the convex side of upper thoracic congenital scoliosis usually required one-stage surgery to elevate scapula and correct the spinal deformity. The one-stage reduction of the elevated scapula was not necessarily needed when the Sprengel's deformity occurred on the concave side of the scoliosis, because the elevated scapula could partially compensate the cosmetic deformity and minimize shoulder asymmetry.

  3. An exploration of student midwives' experiences of the Objective Structured Clinical Examination assessment process.

    Science.gov (United States)

    Barry, Maebh; Noonan, Maria; Bradshaw, Carmel; Murphy-Tighe, Sylvia

    2012-08-01

    This paper reports on a qualitative descriptive study that explored student midwives' experiences of the Objective Structured Clinical Examination assessment process for obstetric emergencies within a university setting. The development of fundamental clinical skills is an important component in preparing students to meet the responsibilities of a midwife. There is an international concern that the transfer of midwifery education into universities may impact on the development of midwifery clinical skills. Objective Structured Clinical Examinations (OSCEs) have the potential to promote integration and consolidation of skills prior to clinical placement. Twenty six students (n=36) from two midwifery programmes (BSc and Higher Diploma) participated in four focus groups and Burnard's (2006) framework was used for data analysis. Three main themes emerged following analysis: preparation for the OSCE assessment, the OSCE process and learning through simulating practice. Preparation for the OSCE's which included lectures, demonstrations, and practice of OSCE's facilitated by lecturers and by the students themselves, was considered central to the process. Learning via OSCEs was perceived to be more effective in comparison to other forms of assessment and prepared students for clinical practice. Positive aspects of the process and areas for improvement were identified. Using OSCE's increased the depth of learning for the students with the steps taken in preparation for the OSCE's proving to be a valuable learning tool. This study adds to the evidence on the use of OSCE's in midwifery education. PMID:21999901

  4. Congenital Self-Healing Reticulohistiocytosis

    Science.gov (United States)

    Lee, Young H.; Talekar, Mala K.; Chung, Catherine G.; Bell, Moshe D.

    2014-01-01

    Congenital self-healing reticulohistiocytosis, also known as congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease, is a Langerhans cell histiocytosis. It is characterized by skin lesions in the newborn period in an otherwise healthy infant that show a Langerhans cell infiltrate in the skin on histological analysis. These findings subsequently spontaneously involute. This report describes two newborns who presented at birth with differing presentations of congenital self-healing reticulohistiocytosis. A review of the disorder, including diagnosis and evaluation, is presented. PMID:24578781

  5. Congenital hypothyroidism: Screening dilemma

    Directory of Open Access Journals (Sweden)

    Meena P Desai

    2012-01-01

    Full Text Available Primary sporadic congenital hypothyroidism (CH is the most common cause of hypothyroidism infancy early childhood in iodine sufficient region. Screening for neonatal CH began in 1970s. The rationale and reason for neonatal screening for CH (NSCH are well established. It is mandatory in most developed countries along with the screen for metabolic disorder. The possibility of measuring TSH and thyroid hormones in cord blood paved the way for newborn screening (NS for CH. Worldwide it is estimated that 25% of the live born population of 130 million babies undergo NSCH. Klein et al., by 1972 had shown improved CNS prognosis in CH treated by age 3 months. NSCH has largely eradicated the severe irreversible neurodevelopmental damage and reversed the chances of growth failure in infancy and early childhood.

  6. Congenital Goitre in Goats

    Directory of Open Access Journals (Sweden)

    A. H. Cheema, A. Shakoor and A. H. Shahzad

    2010-01-01

    Full Text Available One full-term, dead foetus was successfully removed from a 5-year old, crossbred black and white goat. The goat was stall-fed with green fodder and it delivered two dead foetuses in the previous pregnancy. The foetus had a large swelling in the cranio-ventral neck region. Upon cutting skin, the swelling revealed extremely enlarged thyroid gland having two asymmetrical lobes with the right lobe was 8.10 x 15.0 cm and the left 5.5 x 8.6 cm in size. The skin was devoid of hair, pale-white and thickened with myxedema. Histologically, the enlarged thyroid consisted of colloid goitre and the lungs were oedematous. This case of congenital goitre was unusual and differed from the reported cases in two aspects viz 1 the two lobes were enlarged but unequal and 2 histologically goitre was colloid instead of usual hyperplastic type.

  7. Giant Congenital Melanocytic Nevus

    DEFF Research Database (Denmark)

    Rasmussen, Bo Sonnich; Henriksen, Trine Foged; Kølle, Stig-Frederik Trojahn;

    2015-01-01

    % versus 44% required unplanned additional surgery, respectively. Complications were noted in 25% and 67% of the patients, respectively. Cosmetic result was satisfying in 76% of patients without difference between the groups. No malignant transformation was found during a mean follow-up of 11 years......Giant congenital melanocytic nevi (GCMN) occur in 1:20,000 livebirths and are associated with increased risk of malignant transformation. The treatment of GCMN from 1981 to 2010 in a tertiary referral center was reviewed evaluating the modalities used, cosmetic results, associated complications......, and malignant transformation. Of 35 patients, 25 underwent surgery. Curettage was most frequently used (64%) followed by excision and tissue expansion (20%). Six percent of the patients treated with curettage, and 78% of the patients who received excision surgery required more than 1 planned procedure, and 25...

  8. Virus-induced congenital malformations in cattle.

    Science.gov (United States)

    Agerholm, Jørgen S; Hewicker-Trautwein, Marion; Peperkamp, Klaas; Windsor, Peter A

    2015-01-01

    Diagnosing the cause of bovine congenital malformations (BCMs) is challenging for bovine veterinary practitioners and laboratory diagnosticians as many known as well as a large number of not-yet reported syndromes exist. Foetal infection with certain viruses, including bovine virus diarrhea virus (BVDV), Schmallenberg virus (SBV), blue tongue virus (BTV), Akabane virus (AKAV), or Aino virus (AV), is associated with a range of congenital malformations. It is tempting for veterinary practitioners to diagnose such infections based only on the morphology of the defective offspring. However, diagnosing a virus as a cause of BCMs usually requires laboratory examination and even in such cases, interpretation of findings may be challenging due to lack of experience regarding genetic defects causing similar lesions, even in cases where virus or congenital antibodies are present. Intrauterine infection of the foetus during the susceptible periods of development, i.e. around gestation days 60-180, by BVDV, SBV, BTV, AKAV and AV may cause malformations in the central nervous system, especially in the brain. Brain lesions typically consist of hydranencephaly, porencephaly, hydrocephalus and cerebellar hypoplasia, which in case of SBV, AKAV and AV infections may be associated by malformation of the axial and appendicular skeleton, e.g. arthrogryposis multiplex congenita. Doming of the calvarium is present in some, but not all, cases. None of these lesions are pathognomonic so diagnosing a viral cause based on gross lesions is uncertain. Several genetic defects share morphology with virus induced congenital malformations, so expert advice should be sought when BCMs are encountered. PMID:26399846

  9. Hyperpyrexia associated with congenital Long QT Syndrome

    Directory of Open Access Journals (Sweden)

    Nuriye Tarakci

    2014-08-01

    Full Text Available Congenital long QT syndrome (CLQTS is a genetic disorder presented with prolonged QT interval. In these patients, risk of sudden cardiac death due to ventricular tachyarrhythmias is high. Bradycardia may exhibit as a result of intrauterine fetal atrioventricular block, sinus bradycardia, tachycardia in these patient. Prolonged QT interval and multisystem involvement such as sensorineural hearing loss, muscle paralysis, immune deficiency, syndactyly have been reported in these patient . We have detected hyperpyrexia without clinical immunodeficiency and infection in our patient. To our knowledge, our patient is the first case in the literature . [Cukurova Med J 2014; 39(4.000: 909-903

  10. NonClassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Azziz Ricardo

    2010-05-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and "on-time" puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  11. Virtual Surgery in Congenital Heart Disease

    DEFF Research Database (Denmark)

    Sørensen, Thomas Sangild; Mosegaard, Jesper; Kislinskiy, Stefan;

    2014-01-01

     Teaching, diagnosing, and planning of therapy in patients with complex structural cardiovascular heart disease require profound understanding of the three-dimensional (3D) nature of cardiovascular structures in these patients. To obtain such understanding, modern imaging modalities provide high...... et al., Cardiol Young 13:451–460, 2003). In combination with the availability of virtual models of congenital heart disease (CHD), techniques for computer- based simulation of cardiac interventions have enabled early clinical exploration of the emerging concept of virtual surgery (Sorensen et al...

  12. Urogenital tract anomalies in children with congenital anorectal malformation

    NARCIS (Netherlands)

    J.W. Hoekstra

    1991-01-01

    textabstractThe term 'imperforate anus' covers a variety of congenital anorectal malformations ranging in severity from anal stenosis to cloacal exstrophy. The clinical picture of the anorectal malformation has been known for thousands of years, during which many attempts have been made to find an e

  13. Experience from two decades of the Cambridge Rapid Access Neurology Clinic.

    Science.gov (United States)

    Axinte, Laura T; Fiddes, Barnaby D; Donaghy, Alastair; Whyte, Adam; Allen, Chris; Sawcer, Stephen J; Adam, Robert J; Stacpoole, Sybil R L

    2015-10-01

    We report on the evolution of the rapid access neurology clinic (established in 1995) at Addenbrooke's Hospital, Cambridge. Annualised attendance data demonstrate an ever increasing demand, with primary headache disorders now accounting for more than 40% of referrals. Secondary causes of headache (including intracranial tumours, idiopathic intracranial hypertension, carotid or vertebral artery dissection and subdural haematomas) remain infrequent. In all such cases, there were additional diagnostic clues. The number of patients referred with problems related to chronic neurological diseases has fallen considerably, reflecting the roles of specialist nurses and clinics. Imaging investigation of choice shifted from computerised tomography scan (45 to 16%) towards magnetic resonance imaging (17 to 47%). Management is increasingly on an outpatient basis, often without the need for a follow-up appointment. The experience presented here should inform further development of rapid access neurology clinics across the UK and suggests the need for acute headache services, in line with those for transient ischaemic attack and first seizure.

  14. The Return of Congenital Rickets, Are We Missing Occult Cases?

    Science.gov (United States)

    Elidrissy, Abdelwahab T H

    2016-09-01

    Congenital rickets is the term given to fetus born with clinical features of rickets, but those born with biochemical evidence of rickets without obvious clinical features still can be considered occult congenital rickets. Some of the affected babies with this disease have the intrauterine rachitic environment, but a calcium trans-placental pump prevents the fetus from having clinical features of rickets. They may present with hypocalcemia few days after birth or later with more florid features of rickets. Congenital rickets cases born with florid features reported over the last 40 years are few and can be divided into two groups. The first due to severe maternal osteomalacia in which their bones were so decalcified to have enough calcium to be pumped to their fetus. Another group in which newborn babies were hypocalcemic due to other maternal diseases as malabsorption, celiac disease, pre-eclampsia, and prematurity. All inherited rickets cases per se, or as part of other syndromes can be considered congenital rickets. Most cases seen in our region are due to maternal vitamin D deficiency with symptoms becoming obvious when the infants are breastfed, or may present with hypocalcemic convulsions or craniotabes. This is a review of congenital rickets with the aim of shedding light on this potentially acute disease that needs more attention and awareness in the neonatal period to avoid rare serious complications as cardiomyopathy or myelofibrosis and the complications of hypocalcemic convulsions. Congenital rickets cases seen simulate a tip of an ice-burg and its prevention is an important issue, especially with the tremendous urbanization with tall buildings living in sun-deprived flats as the commonest type of residence leading to the increasing incidence of maternal osteomalacia and rickets.

  15. The Return of Congenital Rickets, Are We Missing Occult Cases?

    Science.gov (United States)

    Elidrissy, Abdelwahab T H

    2016-09-01

    Congenital rickets is the term given to fetus born with clinical features of rickets, but those born with biochemical evidence of rickets without obvious clinical features still can be considered occult congenital rickets. Some of the affected babies with this disease have the intrauterine rachitic environment, but a calcium trans-placental pump prevents the fetus from having clinical features of rickets. They may present with hypocalcemia few days after birth or later with more florid features of rickets. Congenital rickets cases born with florid features reported over the last 40 years are few and can be divided into two groups. The first due to severe maternal osteomalacia in which their bones were so decalcified to have enough calcium to be pumped to their fetus. Another group in which newborn babies were hypocalcemic due to other maternal diseases as malabsorption, celiac disease, pre-eclampsia, and prematurity. All inherited rickets cases per se, or as part of other syndromes can be considered congenital rickets. Most cases seen in our region are due to maternal vitamin D deficiency with symptoms becoming obvious when the infants are breastfed, or may present with hypocalcemic convulsions or craniotabes. This is a review of congenital rickets with the aim of shedding light on this potentially acute disease that needs more attention and awareness in the neonatal period to avoid rare serious complications as cardiomyopathy or myelofibrosis and the complications of hypocalcemic convulsions. Congenital rickets cases seen simulate a tip of an ice-burg and its prevention is an important issue, especially with the tremendous urbanization with tall buildings living in sun-deprived flats as the commonest type of residence leading to the increasing incidence of maternal osteomalacia and rickets. PMID:27245342

  16. Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease.

    Science.gov (United States)

    Miura, Yukiko; Saito, Takefumi; Fujita, Kazutaka; Tsunoda, Yoshiya; Tanaka, Toru; Takoi, Hiroyuki; Yatagai, Yohei; Rin, Shigen; Sekine, Akimasa; Hayashihara, Kenji; Nei, Takahito; Azuma, Arata

    2014-10-20

    Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenidone, which is an antifibrotic agent, in five patients with scleroderma-related interstitial lung disease. All patients demonstrated an increase in vital capacity.

  17. Experiences by student nurses during clinical placement in psychiatric units in a hospital

    OpenAIRE

    W.J.C. Van Rhyn; M.R. Gontsana

    2004-01-01

    An exploratory study was conducted with the aim of discovering and describing experiences of psychiatric nursing students during clinical placement in a psychiatric unit. For the purpose of the study an unstructured interview was conducted with each participant during their first placement in a psychiatric unit to identify the factors experienced as stressful. The results indicated that all eight participants experienced average to high stress. Sources of stress identified included, among oth...

  18. Clinical outcome of parotidectomy with reconstruction: Experience of a regional head and neck cancer unit

    OpenAIRE

    Eyituoyo Okoturo; Anslem Osasuyi

    2016-01-01

    Background: Salivary gland pathologies represent a histologically diverse group of benign and malignant neoplasms. Currently, World Health Organization recognizes 13 benign and 24 malignant variants of all salivary gland neoplasms. Surgery continues to remain the main-stay for treatment of parotid gland neoplasms. The aim of this study was to document our experiences of the patients treated for parotid tumors and find out if any compelling variable predicted the relative clinical outcomes. Ma...

  19. Job preferences among clinical officers in public sector facilities in rural Kenya: a discrete choice experiment

    OpenAIRE

    Takemura, Toshio; Kielmann, Karina; Blaauw, Duane

    2016-01-01

    Background Clinical officers (COs), a mid-level cadre of health worker, are the backbone of healthcare provision in rural Kenya. However, the vacancy rate for COs in rural primary healthcare facilities is high. Little is known about factors motivating COs’ preferences for rural postings. Methods A discrete choice experiment (DCE) questionnaire was used with 57 COs at public health facilities in nine districts of Nyanza Province, Kenya. The questionnaire was developed on the basis of formative...

  20. Experiences of Newly Admitted Nursing Students in Theoretical and Clinical Practice

    OpenAIRE

    Lekkas, Elena; Mwanakayaya, Naomi; Uzoka, Zeal; Ogie-Osahon, Cynthia

    2016-01-01

    The clinical and classroom environment provides opportunities for student learning and widens the scope of them acquring competencies in the nursing profession. During the first year of nursing school, any unpleasant or delightful encounter by students in these environments of study may influence their learning outcome. The aim of this study was to find out the experiences of the first year nursing students in classroom and practice area as well as its consequence on their wellbeing and the c...