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  1. Atresia pulmonar con comunicación interventricular

    Directory of Open Access Journals (Sweden)

    Tomasa Centella Hernández

    2014-04-01

    Full Text Available La atresia pulmonar con comunicación interventricular es una cardiopatía congénita cianótica, severa y rara, de alta complejidad, que se caracteriza por la ausencia de conexión entre el ventrículo derecho y las arterias pulmonares. Coexiste con una comunicación interventricular. El flujo hacia el territorio pulmonar puede realizarse a través del ductus arterioso o de colaterales sistémico-pulmonares. La dificultad de esta cardiopatía viene determinada por los diferentes niveles de interrupción desde el ventrículo derecho hasta el territorio pulmonar, y por la diferencia anatómica de las fuentes del flujo hacia dicho territorio, lo que determina diferentes tipos de abordaje quirúrgico.

  2. Morfología ecocardiográfica de la atresia pulmonar con septum interventricular intacto, estudio de dos decenios

    Directory of Open Access Journals (Sweden)

    Adel Eladio González Morejón

    2013-03-01

    Full Text Available Introducción: la atresia pulmonar con septum interventricular intacto es una malformación cardiovascular que representa el 1 % de las cardiopatías congénitas observadas en vida extrauterina, y, dados sus resultados desfavorables, constituye un verdadero reto para la medicina contemporánea. Objetivo: la investigación condujo a la aplicación de pautas clasificatorias, a la caracterización del tracto de salida atrésico, al estudio morfológico ventricular derecho, a la valoración del anillo tricuspídeo y a la determinación de la presencia de anomalías en la circulación coronaria. Métodos: se estudiaron 43 pacientes con diagnóstico confirmado de la entidad remitidos al Cardiocentro Pediátrico "William Soler" entre enero de 1992 y noviembre de 2011. Se practicó a cada caso el examen ecocardiográfico bidimensional y doppler con codificación en colores. Resultados y conclusiones: se corroboró el predominio de la variante morfológica valvular de la entidad y la existencia de niveles moderados o severos de hipoplasia ventricular derecha en asociación con capacitancia volumétrica limitada de dicha cámara, con hipodesarrollo valvular tricuspídeo y con presencia de circulación coronaria anómala sinusoides dependiente. El foramen oval permeable constituyó el defecto septal interatrial más vinculado a la enfermedad, y se evidenciaron diversas anomalías estructurales del aparato tricuspídeo en conjunción o no con el hipodesarrollo anular imperante.

  3. ATRESIA BILIER

    Directory of Open Access Journals (Sweden)

    Julinar Julinar

    2009-09-01

    Full Text Available AbstrakAtresia bilier merupakan penyakit yang jarang terjadi dan penyababnya belum diketahui secara pasti. Karakteristik dari penyakit ini adalah terjadinya inflamasi progresif pada duktus bilier sehingga terjadi obstruksi ekstrahepatal yang akhirnya dapat menyebabkan fibrosis dan sirosis hepar. Atresia bilier ada 2 tipe yaitu: 1. Syndromic atau fetal, disertai beberapa kelainan kongenital (10-20%. 2. non syndromic, tanpa disertai kelainan kongenital yang lain (80-90%. Atresia bilier akan berakibat fatal tanpa penanganan yang cepat. Kelainan ini dapat ditangani dengan metode operasi Kasai prosedure yang dapat mengalirkan kembali aliran empedu hampir 80% jika dilakukan secepatnya, gold periode >60 hari. Diagnosis dini sangat penting untuk keberhasilan operasi Kasai. Pada penulisan ini akan dilaporkan sebuah kasus atresia bilier tipe fetal, seorang anak laki-laki berusia 58 hari, dengan keluhan tampak kuning sejak usia 3 minggu disertai dengan buang air besar berwarna pucat, buang air kecil berwarna seperti teh pekat. Diagnosis ditegakkan berdasarkan gejala klinis, laboratorium, USG dan biopsi hepar yang sangat mendukung diagnosis atresia bilier. Operasi Kasai tidak efektif karena disertai dengan komplikasi kholangitis yang akhirnya menyebabkan sirosis hepatis pada 5 bulan kehidupan.Kata kunci : Atresia bilier, Kasai procedure, kholangitis, sirosis hapatisAbstractBiliary atresia is a disease of unknown etiology, characterized by progressive fibro inflammatory of the bile duct and liver that result obstruction of extrahepatic bile duct, leading to the fIbrosis and liver cirrhosis. It has two form of biliary atresia : 1. syndromic of fetal biliary atresia (10-20% with various congenital anomalies, 2. non syndromic biliary atresia (80-90% with isolated anomaly. In this case we report on an infant with the second form of biliary atresia, with diagnosis and operation was not based on liver biopsy, but on clinical features, laboratorium finding and USG

  4. Pulmonary atresia

    Science.gov (United States)

    ... another type of congenital heart defect called a patent ductus arteriosus (PDA). Pulmonary atresia may occur with ... known way to prevent this condition. All pregnant women should get routine prenatal care. Many congenital defects ...

  5. Atresia biliar: una enfermedad grave

    OpenAIRE

    Ramonet, Margarita; Ciocca, Mirta; Alvarez, Fernando

    2014-01-01

    La atresia biliar es una grave enfermedad que se manifiesta en los recién nacidos, y se desconoce su causa. La inflamación y destrucciónprogresiva de los conductos biliares conducen a la aparición de ictericia, coluria y acolia entre la segunda y sexta semana de vida. Como existen múltiples causas de colestasis neonatal en esta etapa de la vida, es necesario realizar un diagnóstico y derivación precoz para ofrecer un tratamiento quirúrgico, con el fin de restablecer el flujo biliar. Alrededor...

  6. Congenital pyloric atresia: clinical features, diagnosis, associated ...

    African Journals Online (AJOL)

    Epidermolysis bullosa was seen in eight (40%) patients and multiple intestinal atresias in five (25%). Three patients had associated esophageal atresia. Pyloric diaphragm was the most common and seen in 13 patients including double diaphragms in two followed by pyloric atresia with a gap in four and pyloric atresia ...

  7. Surgery for congenital choanal atresia.

    Science.gov (United States)

    Cedin, Antonio C; Atallah, Alvaro N; Andriolo, Régis B; Cruz, Oswaldo L; Pignatari, Shirley N

    2012-02-15

    Congenital choanal atresia is a rare abnormality characterized by unilateral or bilateral lack of patency of the posterior end of the nasal cavity. With an incidence of 1:5000 to 1:8000 births, it is twice as prevalent in females as it is in males. Surgical procedures aim to provide adequate functional choanal patency and a low rate of restenosis, avoid harm to any structure in development, enable shorter surgery and hospitalization times, and minimize morbidity and mortality. To evaluate the effectiveness and safety of the available surgical techniques for the treatment of congenital choanal atresia in patients with unilateral and bilateral atresia. We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ISRCTN and additional sources for published and unpublished trials. The date of the search was 31 January 2011. We planned to include parallel randomized or quasi-randomized controlled trials testing surgical approaches for the treatment of congenital atresia (irrespective of gender and age) that evaluated normal/adequate respiratory function (self reported or preserved nasal airway) and restenosis as the main primary outcomes. We did not consider reoperation and non-congenital atresia (e.g. traumatic, iatrogenic atresias) for inclusion. Three review authors independently assessed the titles and abstracts of the identified articles to determine potential relevance. For dichotomous and continuous variables, we planned to calculate risk ratios (relative risks; RR) and mean differences (MD) with 95% confidence intervals (CI), respectively. We planned to use the random-effects model since we were expecting substantial clinical and methodological heterogeneity. No randomized controlled trials were identified. From the 120 reports retrieved using our search strategy, 46 primary studies had the

  8. MRI in distal vaginal atresia

    International Nuclear Information System (INIS)

    Hugosson, C.; Jorulf, H.; Bakri, Y.

    1991-01-01

    Magnetric resonance imaging in two young females with abdominal pain revealed vaginal atresia with massive hematocolpos but a normal cervix and uterine body. Information obtained with MRI was superior to ultrasound and CT and is suggested as the examination of choice prior to surgical correction. (orig.)

  9. The Combination of Gastroschisis, Jejunal Atresia, and Colonic Atresia in a Newborn

    Directory of Open Access Journals (Sweden)

    Zachary Bauman

    2015-01-01

    Full Text Available We encountered a rare case of gastroschisis associated with jejunal atresia and colonic atresia. In our case, the jejunal atresia was not discovered for 27 days after the initial abdominal wall closure. The colonic atresia was not discovered for 48 days after initial repair of the gastroschisis secondary to the rarity of the disorder. Both types of atresia were repaired with primary hand-sewn anastomoses. Other than the prolonged parenteral nutrition and hyperbilirubinemia, our patient did very well throughout his hospital course. Based on our case presentation, small bowel atresia and colonic atresia must be considered in patients who undergo abdominal wall closure for gastroschisis with prolonged symptoms suggestive of bowel obstruction. Our case report also demonstrates primary enteric anastomosis as a safe, well-tolerated surgical option for patients with types of intestinal atresia.

  10. Oesophageal atresia: triumph and tragedy.

    Science.gov (United States)

    Rickham, P P; Stauffer, U G; Cheng, S K

    1977-04-01

    An enormous amount has been written about oesophageal atresia during the last 30 years. This is not surprising because it is not so long ago that the condition was uniformly fatal, and even today, a generation after the first successful operations, many problems associated with its management have not been completely solved. This lecture discusses past and present management, past and present results and future prospects of infants suffering from this malformation.

  11. Biliary atresia: the Canadian experience.

    Science.gov (United States)

    Schreiber, Richard A; Barker, Collin C; Roberts, Eve A; Martin, Steven R; Alvarez, Fernando; Smith, Lesley; Butzner, J Decker; Wrobel, Iwona; Mack, David; Moroz, Stanley; Rashid, Mohsin; Persad, Rabin; Levesque, Dominique; Brill, Herbert; Bruce, Garth; Critch, Jeff

    2007-12-01

    To determine the outcomes of Canadian children with biliary atresia. Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed. 349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS). This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.

  12. Epidemiology of small intestinal atresia in Europe

    DEFF Research Database (Denmark)

    Best, Kate E; Tennant, Peter W G; Addor, Marie-Claude

    2012-01-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe....

  13. Histopathologic profile of esophageal atresia and ...

    African Journals Online (AJOL)

    dUMAss Memorial Pathology, UMass Memorial Medical Center, Worcester, ..... 2 Yokoi A, Nishijima E. Long-term complications of esophageal atresia. Nihon ... long upper esophageal pouch and short gap, associated with left congenital.

  14. Sirenomelia with oesophageal atresia: a rare association.

    Science.gov (United States)

    Sathe, Pragati Aditya; Ghodke, Ratnaprabha Kundlikrao; Kandalkar, Bhuvaneshwari Mahendra

    2014-02-01

    We are reporting a rare case of sirenomelia with oesophageal atresia. Sirenomelia is a lethal sporadic defect of which lower gastrointestinal tract anomalies are characteristic findings. Respiratory and upper gastrointestinal tract malformations like oesophageal atresia occur in about 20-35% of cases. Though its occurrence has been described, it has been reported only rarely. This report aims at describing this uncommon association along with its histological features.

  15. [Extrahepatic biliary atresia: diagnostic methods].

    Science.gov (United States)

    Cauduro, Sydney M

    2003-01-01

    To emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct relationship with the surgical re-establishment of the biliary flow before the second month of life. To discuss several complementary methods with the aim of selecting the ones that present better evidence, and avoiding delays in diagnosis and worse prognostic. Bibliographical researching regarding the period of 1985-2001, in Medline and MdConsult, using the following key words: neo-natal cholestasis; extrahepatic biliary atresia; neo-natal hepatitis. National and foreign articles were also elected based on the bibliography of consulted publications, and when necessary, for better understanding of the theme, opinions emitted in theses and textbooks were referred. The revision of the consulted bibliography led to the assumption that early diagnosis of EHBA and surgical treatment to reestablish the biliary flow up to 60 days of life are fundamental in order to achieve good results. Among several complementary methods of diagnosis, cholangiography by MR, US and the hepatic biopsy are the ones that provide the largest success indexes. The referring of patients bearers of EHBA to centers of references in Brazil, is still made tardily, probably due to lack of enlightenment of the doctors of primary attention, allied to bureaucratic and technological difficulties. The experience in England in relation to the "Yellow Alert" program, allowed that the number of children referred to surgical treatment before the 60 days of life increased significantly. Among the complementary methods, the MR cholangiography, ultrasonography and hepatic biopsy should be used, depending on the technological resources of the diagnosis units.

  16. Prenatal diagnosis of horseshoe lung and esophageal atresia

    International Nuclear Information System (INIS)

    Goldberg, Shlomit; Ringertz, Hans; Barth, Richard A.

    2006-01-01

    We present a case of horseshoe lung (HL) and esophageal atresia suspected prenatally on US imaging and confirmed with fetal MRI. Prenatal diagnosis of HL and esophageal atresia allowed for prenatal counseling and informed parental decisions. (orig.)

  17. Prenatal diagnosis of horseshoe lung and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Goldberg, Shlomit; Ringertz, Hans [Stanford University School of Medicine, Radiology Department, Stanford, CA (United States); Barth, Richard A. [Stanford University School of Medicine, Radiology Department, Stanford, CA (United States); Lucile Packard Children' s Hospital, Radiology, Palo Alto, CA (United States)

    2006-09-15

    We present a case of horseshoe lung (HL) and esophageal atresia suspected prenatally on US imaging and confirmed with fetal MRI. Prenatal diagnosis of HL and esophageal atresia allowed for prenatal counseling and informed parental decisions. (orig.)

  18. Choanal atresia in siblings; Case report | Kaitesi | East and Central ...

    African Journals Online (AJOL)

    Choanal atresia is an uncommon and often poorly recognized cause of unilateral or bilateral nasal obstruction. This report describes the case of bilateral choanal atresia in two consecutive siblings and describes the methods of treatment offered.

  19. Emergencies in neonatal management: jaundice and biliary atresia

    OpenAIRE

    Clemente, Maria Grazia; Dessanti, Antonio

    2016-01-01

    Biliary atresia is a severe and progressive inflammatory process of unknown cause, which initially involves the extrahepatic bile ducts but which quickly proceeds towards the intrahepatic bile tree leading rapidly to biliary cirrhosis. Biliary atresia is the major reason for liver transplantation during childhood. The extrahepatic bile ducts in biliary atresia become connective fibrotic cords which is irreversibly damaged.

  20. Pyloric atresia: a challenge in an underdeveloped country | Sagna ...

    African Journals Online (AJOL)

    Pyloric atresia is a rare congenital malformation. We report a case in a 5-day newborn with pyloric atresia type C. Authors emphasize the diagnostic difficulties and therapeutic challenges in a resource-limited country. Keywords: Pyloric atresia, newborn, rare congenital malformation ...

  1. Wind sock deformity in rectal atresia

    International Nuclear Information System (INIS)

    Hosseini, Seyed M V; Ghahramani, Farhad; Shamsaeefar, Alireza; Razmi, Tannaz; Zarenezhad, Mohammad

    2009-01-01

    Rectal atresia is a rare anorectal deformity. It usually presents with neonatal obstruction and it is often a complete membrane or severe stenosis. Windsock deformity has not been reported in rectal atresia especially, having been missed for 2 years. A 2-year-old girl reported only a severe constipation despite having a 1.5-cm anal canal in rectal examination with scanty discharge. She underwent loop colostomy and loopogram, which showed a wind sock deformity of rectum with mega colon. The patient underwent abdominoperineal pull-through with good result and follow-up. This is the first case of the wind sock deformity in rectal atresia being reported after 2 years of age. (author)

  2. Aortic atresia with normal sized left ventricle

    Directory of Open Access Journals (Sweden)

    Priya Jagia

    2016-01-01

    Full Text Available Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best of our knowledge, use of multi-detector computed tomography in aortic atresia with well developed left ventricle has not been reported in literature till date.

  3. Outcomes of surgical management of intestinal atresias

    African Journals Online (AJOL)

    2013-11-22

    Nov 22, 2013 ... nutrition and neonatal surgical intensive care services are the norm. Thus, outcome of management in ... Social Sciences (SPSS 15.0 version, SPSS Inc, Chicago Ill) was used for data entry and analysis. ..... Excellent long‑term outcome for survivors of apple peel atresia. J Pediatr Surg 2002;37:61‑5. 17.

  4. Dysphagia in children with repaired oesophageal atresia

    NARCIS (Netherlands)

    Coppens, C.H.; Engel-Hoek, L. van den; Scharbatke, H.E.; Groot, S.A. de; Draaisma, J.M.T.

    2016-01-01

    Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral

  5. Double sigmoid atresia with meconium pseudocyst masquerading ...

    African Journals Online (AJOL)

    8В 6cm dark cystic lesion was present in the left iliac fossa, which was delivered intact ... of Pediatric Surgery. Unauthorized reproduction of this article is prohibited. .... sigmoid-colon atresia: the perforated web variety. APSP J Case Rep. 2010 ...

  6. Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

    International Nuclear Information System (INIS)

    Githu, Tangayi; Merrow, Arnold C.; Lee, Jason K.; Garrison, Aaron P.; Brown, Rebeccah L.

    2014-01-01

    Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination. (orig.)

  7. Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

    Energy Technology Data Exchange (ETDEWEB)

    Githu, Tangayi [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Radiology of Huntsville, P.C., Huntsville, AL (United States); Merrow, Arnold C.; Lee, Jason K. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Garrison, Aaron P. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States); Akron Children' s Hospital, Pediatric Surgery, Akron, OH (United States); Brown, Rebeccah L. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States)

    2014-03-15

    Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination. (orig.)

  8. Chronic Pulmonary Aspergillosis Complicating Bronchial Atresia

    Directory of Open Access Journals (Sweden)

    Mazen O. Al-Qadi

    2014-01-01

    Full Text Available Bronchial atresia is a rare pulmonary developmental anomaly characterized by the presence of a focal obliteration of a segmental or lobar bronchial lumen. The lung distal to the atretic bronchus is typically emphysematous along with the presence of mucus filled ectatic bronchi (mucoceles. BA is usually asymptomatic but pulmonary infections can rarely develop in the emphysematous lung distal to the atretic bronchus. We present a unique case of chronic pulmonary aspergillosis (CPA in a patient with BA with no evidence of immune dysfunction. The patient was treated initially with voriconazole and subsequently underwent surgical excision of the involved area. On follow-up, she has done extremely well with no evidence for recurrence. In summary, we describe the first case of chronic pulmonary aspergillosis in an immunocompetent patient with bronchial atresia.

  9. Etiological study on isolated esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Szendrey, T; Danyi, G; Czeizel, A

    1985-01-01

    A study group of 160 index patients with isolated esophageal atresia, a control group of 160 matched healthy controls, and the first-degree relatives of patients and controls were examined; epidemiological, family planning, teratological, and genetic data were obtained by personal interview in the study and control groups. One half of the index patients were male. Intrauterine growth retardation, a higher proportion of mothers under 19 or over 30 years of age, and less skilled professions of the parents were found in the study group. There were more extramarital conceptions, more pregnancies in spite of the use of contraceptive pills, and more delayed conceptions in index patients mothers. The teratogens studied did not have an obvious pathological effect here. The sib occurrence of isolated esophageal atresia was 0.43%, which did not correspond well to the expected figure of 1.34% based on the polygenic model.

  10. Dysphagia in children with repaired oesophageal atresia

    OpenAIRE

    Coppens, C.H.; Engel-Hoek, L. van den; Scharbatke, H.E.; Groot, S.A. de; Draaisma, J.M.T.

    2016-01-01

    Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral and pharyngeal dysphagia based on videofluoroscopic swallow study (VFSS) findings in a limited number of children in this cohort. Medical records of 111 patients, born between January 1996 and July ...

  11. Aortic atresia with normal sized left ventricle

    OpenAIRE

    Priya Jagia; Arun Sharma; Saurabh K Gupta; Munish Guleria

    2016-01-01

    Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best ...

  12. Ultrasonographic findings of type IIIa biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seung Seob; Kim, Myung Joon; Lee, Mi Jung; Yoon, Choon Sik; Han, Seok Joo; Koh, Hong [Dept. of Radiology, Research Institute of Radiological Science, Severance Hospital, Yensei University College of Medicine, Seoul (Korea, Republic of)

    2014-12-15

    To describe the ultrasonographic (US) findings of type IIIa biliary atresia. We retrospectively reviewed a medical database of patients pathologically confirmed to have biliary atresia, Kasai type IIIa, between January 2002 and May 2013 (n=18). We evaluated US findings including the visible common bile duct (CBD), triangular cord thickness, gallbladder size and shape, and subcapsular flow on color Doppler US; laboratory data; and pathological hepatic fibrosis grades. We divided them into two groups-those with visible (group A) and invisible (group B) CBD on US-and compared all parameters between the two groups. CBD was visible on US in five cases (27.8%; group A) and invisible in 13 cases (72.2%; group B). US was performed at an earlier age in group A than in group B (median, 27 days vs. 60 days; P=0.027) with the maximal age of 51 days. A comparison of the US findings revealed that the triangular cord thickness was smaller (4.1 mm vs. 4.9 mm; P=0.004) and the gallbladder length was larger (20.0 mm vs. 11.7 mm; P=0.021) in group A. The gallbladder shape did not differ between the two groups, and the subcapsular flow was positive in all cases of both groups. There was no significant difference in the laboratory data between the two groups. Upon pathological analysis, group A showed low-grade and group B showed low- to high-grade hepatic fibrosis. When CBD is visible on US in patients diagnosed with type IIIa biliary atresia, other US features could have a false negative status. A subcapsular flow on the color Doppler US would be noted in the type IIIa biliary atresia patients.

  13. Primary Segmental Volvulus Mimicking Ileal Atresia

    Science.gov (United States)

    Rao, Sadashiva; B Shetty, Kishan

    2013-01-01

    Neonatal intestinal volvulus in the absence of malrotation is a rare occurrence and rarer still is the intestinal volvulus in absence of any other predisposing factors. Primary segmental volvulus in neonates is very rare entity, which can have catastrophic outcome if not intervened at appropriate time. We report two such cases, which were preoperatively diagnosed as ileal atresia and intraoperatively revealed to be primary segmental volvulus of the ileum. PMID:26023426

  14. Apple-peel atresia presenting as foetal intestinal obstruction ...

    African Journals Online (AJOL)

    Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA) is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed ...

  15. ATRESIA CONGÉNITA DEL OÍDO Y SU MANEJO

    Directory of Open Access Journals (Sweden)

    Dr. Daniel Orfila

    2016-11-01

    Los implantes cocleares siguen siendo los más usados y en las que se tiene mayor experiencia. Las prótesis de conducción ósea implantables o semi implantables cambiaron el manejo de las atresias y malformaciones de oído externo y medio. Pese a lo prometedor que se visualiza el presente y futuro con el uso de estos dispositivos, siempre se debe tener presente que requieren de un acto quirúrgico para su implantación y que no están exentas de complicaciones, por lo cual se debe elegir juiciosamente la prótesis a usar.

  16. Relación entre el peso al nacer y la mortalidad por atresia esofágica

    Directory of Open Access Journals (Sweden)

    Roberto Reyes Rodríguez

    Full Text Available Introducción: la historia del tratamiento quirúrgico de la atresia esofágica con fístula traqueoesofágica es extraordinaria, y cubre 270 años desde la primera descripción hasta el primer sobreviviente. Existen varias clasificaciones que ayudan a determinar el pronóstico de estos niños, entre las que se encuentra la de Waterston, la de Montreal y la de Spitz. La mejoría en la supervivencia no solo se debe al tratamiento quirúrgico, sino a los avances en los cuidados intensivos neonatales, particularmente el apoyo ventilatorio y nutricional que requieren estos pacientes. Los niños con mayor riesgo de muerte son aquellos con peso al nacimiento menor de 1 500 g, con malformaciones cardiacas o anomalías cromosómicas. Objetivo: determinar la influencia del peso al nacer en la mortalidad de estos pacientes. Métodos: se realizó un estudio observacional, descriptivo transversal de todos los casos diagnosticados de atresia esofágica con o sin fístula traqueoesofágica, en el periodo comprendido desde enero de 2000 hasta diciembre de 2011, en el Hospital Pediátrico Docente Provincial "José Luis Miranda", de Santa Clara, Cuba. Resultados: de los 32 pacientes estudiados, el 46,9 % pesó menos de 2 500 g. Los pacientes con un peso inferior a 2 500 g, tienen 2,2 veces más probabilidades de morir, que los que pesan más de 2 500 g. Conclusiones: el bajo peso al nacer continúa siendo significativo como predictor de mortalidad en los recién nacidos cubanos con atresia esofágica.

  17. Feeding Difficulties in Children with Esophageal Atresia.

    Science.gov (United States)

    Mahoney, Lisa; Rosen, Rachel

    2016-06-01

    The current available literature evaluating feeding difficulties in children with esophageal atresia was reviewed. The published literature was searched through PubMed using a pre-defined search strategy. Feeding difficulties are commonly encountered in children and adults with repaired esophageal atresia [EA]. The mechanism for abnormal feeding includes both esophageal and oropharyngeal dysphagia. Esophageal dysphagia is commonly reported in patients with EA and causes include dysmotility, anatomic lesions, esophageal outlet obstruction and esophageal inflammation. Endoscopic evaluation, esophageal manometry and esophograms can be useful studies to evaluate for causes of esophageal dysphagia. Oropharyngeal dysfunction and aspiration are also important mechanisms for feeding difficulties in patients with EA. These patients often present with respiratory symptoms. Videofluoroscopic swallow study, salivagram, fiberoptic endoscopic evaluation of swallowing and high-resolution manometry can all be helpful tools to identify aspiration. Once diagnosed, management goals include reduction of aspiration during swallowing, reducing full column reflux into the oropharynx and continuation of oral feeding to maintain skills. We review specific strategies which can be used to reduce aspiration of gastric contents, including thickening feeds, changing feeding schedule, switching formula, trialing transpyloric feeds and fundoplication. Copyright © 2015 Elsevier Ltd. All rights reserved.

  18. Extrahepatic biliary atresia in a border collie.

    Science.gov (United States)

    Schulze, C; Rothuizen, J; van Sluijs, F J; Hazewinkel, H A; van den Ingh, T S

    2000-01-01

    Progressive lameness and leg pain were the predominant clinical signs in a 17-week-old male border collie presented for examination. On clinical investigation, extrahepatic cholestasis in association with rickets due to inadequate vitamin D resorption was diagnosed. The dog was treated parenterally with vitamin D and a cholecystoduodenostomy was performed. At 25 days postsurgery the lameness had resolved and bone structure was radiographically normal. However, at six weeks postsurgery, the dog's condition deteriorated rapidly and euthanasia was finally performed at eight weeks postsurgery. At postmortem examination, Toxocara canis nematodes were found to have invaded the biliary system via the anastomosis between the gallbladder and duodenum, causing biliary and hepatic toxocariasis. The cause of the primary extrahepatic cholestasis was atresia of the common bile duct at the hepatic end. The liver tissue showed microscopic lesions of chronic extrahepatic cholestasis as well as acute inflammation associated with the nematode invasion. There was no postmortem evidence of bone lesions. Extrahepatic biliary atresia is extremely rare in animals and has not been described before in dogs. In contrast, it represents the most common cause of congenital cholestasis in children, occurring in approximately one per 10,000 to 15,000 live births.

  19. Postoperative follow-up studies in biliary atresia using radioisotope

    Energy Technology Data Exchange (ETDEWEB)

    Kanto, Kei; Ishida, Haruo; Hayashi, Akira; Kamagata, Shoichiro; Sanbonmatsu, Toru; Matsufuji, Hiroshi; Ishii, Katsumi

    1988-09-01

    With increasing numbers of long survival patients in biliary atresia, associated diseases such as liver cirrhosis and portal hypertension seem to be more important in their course. We use liver scintigraphy, hepatobiliary scintigraphy and transrectal portal scintigraphy as the follow-up study. Three studies generally correlate the present state of the patients, but there seems to be dissociation in the group of cirrhosis without icterus which are encountered most often in biliary atresia. That can be seen in hepatobiliary scintigraphy especially. So we emphasis that to choose several isotope studies are essential in determination of the postoperative state in biliary atresia.

  20. Anaesthesia for oesophageal atresia with or without tracheo ...

    African Journals Online (AJOL)

    and a reduced closing pressure of the cardiac sphincter.5 An abnormality of ... Tetralogy of Fallot, double outlet right ventricle, tricuspid atresia, atrial septal defect ..... prostaglandins in ductus arteriosus-dependent physiology and a measure of ...

  1. Dysphagia in Children with Esophageal Atresia: Current Diagnostic Options

    OpenAIRE

    Rayyan, Maissa; Allegaert, Karel; Omari, Taher; Rommel, Nathalie

    2015-01-01

    Dysphagia or swallowing disorder is very common (range, 15-52%) in patients with esophageal atresia. Children present with a wide range of symptoms. The most common diagnostic tools to evaluate esophageal dysphagia, such as upper barium study and manometry, aim to characterize anatomy and function of the esophageal body and the esophagogastric junction (EGJ). Using these technologies, a variety of pathological motor patterns have been identified in children with esophageal atresia. However, t...

  2. Evolución a largo plazo de 55 pacientes operados de atresia esofágica

    Directory of Open Access Journals (Sweden)

    Ruperto Llanes Céspedes

    2003-06-01

    Full Text Available Los pacientes operados de atresia esofágica constituyen un reto para los médicos involucrados en su seguimiento. Se realizó un estudio retrospectivo de las historias clínicas de 55 pacientes vivos después de operarse de atresia esofágica, en un período de 15 años, donde se evaluaron las complicaciones encontradas a largo plazo, con el objetivo de identificarlas y conocer su tratamiento. La presencia de reflujo gastroesofágico en 30 niños (54,54 %, la dismotilidad esofágica en 28 (50,90 % y las manifestaciones respiratorias recurrentes en 26 pacientes (47,27 % fueron las más frecuentes, seguidas de las estenosis de la sutura en 11 (20 %, retardo del crecimiento y desarrollo en 10 (18,18 % y la recurrencia de la fístula en 2 niños (3,63 %. Se concluye que en el seguimiento a largo plazo de los pacientes operados de atresia esofágica se deben realizar el diagnóstico precoz y el tratamiento de múltiples complicaciones que definen su calidad de vida.The patients operated for esophageal atresia become a challenge for physicians involved in their follow-up. A retrospective study of the medical histories of 55 live patients after they had been operated for esophageal atresia in a period of 15 years was undertaken where long-term complications were evaluated to identify them and find out their treatment. Gastroesophageal reflux in 30 children (54,54 %, esophageal dismotility in 28 children (50,90 % and recurrent respiratory manifestations in 26 patients (47,27 % were the most frequent complications followed by suture stenosis in 11 (20 %, retarded growth and development in 10 (18,18 % and recurrent tracheoesophageal fistula in 2 children. It is concluded that early diagnosis and treatment of multiple complications, which define the quality of life of patients, should be made in the long-term follow-up of patients operated for esophageal atresia.

  3. [Bile duct atresia: outline for a solution].

    Science.gov (United States)

    Broto, J; Asensio, M; Gil Vernet, J M; Marhuenda, C; Boix Ochoa, J

    2000-07-01

    Biliary atresia continues to be a serious and relatively rare disease (1/50,000 newborns) and whose long-term prognosis has changed drastically since the appearance of liver transplant (LT) as a therapeutic weapon. The combination of two factors, early diagnosis and correct application of Kasai's surgical technique, is essential to obtain acceptable results and sufficient biliary drainage allowing the children to overcome the critical 7 kg barrier and place them in the lesser morbi-mortality range in relation to a possible LT. But we must keep in mind that despite its critics, Kasai's technique can guarantee, both in our own experience and in the literature, ten years survival percentages over 50% with correct hepatic function, as well as clinical normality and a quality of life clearly superior to first years post-LT. We present the evolution of a group of 20 patients affected with biliary atresia, diagnosed in our center since 1985, the year when pediatric LT began to be used as a therapeutic procedure in this country. We valued the age of intervention, technique, immediate and long-term results and the evolution and necessity of LT. All 20 patients were analyzed individually, and they currently have an age range from 2-14 years and were all operated by Kasai's technique. We classified the patients as having good, regular or poor results with regards to biliary flow, normalization of billirubin levels and clinical evolution. Sixteen patients presented biliary flow of such an extent that 14 of them, classified as good, completely normalized the billirubin levels. Two others, presently aged 14 and 8 years respectively, present average levels of 2.5-5.5 mg/100 ml and are classified as regular in a situation of advisable transplant, although with an acceptable hepatic function. Only one case, the first in the poor group, did not initially present biliary elimination and died at age six months while on the waiting list. Three other cases in the same group presented

  4. Atresia biliar: a experiência Brasileira

    Directory of Open Access Journals (Sweden)

    Elisa de Carvalho

    2010-12-01

    Full Text Available OBJETIVO: Avaliar as características epidemiológicas, clínicas e prognósticas de crianças com atresia biliar. MÉTODOS: Dados sobre portoenterostomia, transplante hepático (TxH, idade no último seguimento e sobrevida foram coletados dos prontuários de pacientes acompanhados em seis centros no Brasil (1982-2008 e comparados em relação às décadas do procedimento cirúrgico. RESULTADOS: Dos 513 pacientes, 76,4% foram submetidos a portoenterostomia [idade: 60,0-94,7 (82,6±32,8 dias] e 46,6% foram submetidos a TxH. Em 69% dos casos, o TxH foi realizado após a portoenterostomia, enquanto em 31% dos casos o TxH foi realizado como cirurgia primária. Os pacientes da região Nordeste foram submetidos a portoenterostomia mais tardiamente do que as crianças das regiões Sul (p = 0,008 e Sudeste (p = 0,0012, embora, mesmo nas duas últimas regiões, a idade no momento da portoenterostomia tenha sido superior ao desejável. Ao longo das décadas, houve aumento progressivo do número de TxH realizados. A sobrevida global foi de 67,6%. A sobrevida aumentou nas últimas décadas (anos 1980 versus 1990, p = 0,002; anos 1980 versus 2000, p 90 dias, respectivamente. Os pacientes transplantados apresentaram taxas de sobrevida mais elevadas (88,3%. A sobrevida de 4 anos com fígado nativo foi de 36,8%, inversamente correlacionada à idade no momento da portoenterostomia (54, 33,3, 26,6% para 90 dias, respectivamente. CONCLUSÕES: Este estudo multicêntrico demonstrou que o encaminhamento tardio das crianças portadoras de atresia biliar ainda é um problema no Brasil, influenciando a sobrevida destes pacientes. Estratégias que proporcionam o encaminhamento precoce estão sendo desenvolvidas com o objetivo de reduzir a necessidade de transplante hepático nos primeiros anos de vida.

  5. Duodenal Derotation and Extent Tapering Jejunoplasty as Primary Repair for Neonates With High Jejunal Atresia

    Directory of Open Access Journals (Sweden)

    Chih-Cheng Luo

    2010-10-01

    Conclusion: In very proximal high atresia, the extent of tapering is limited by the proximity of the ligament of Treitz. Duodenal derotation provides better access to the high atresia. The results of this limited experience suggest that the DDETJ procedure could provide an alternative therapy in patients with high jejunal atresia.

  6. Intestinal smooth muscle response to chronic obstruction : possible applications in jejunoileal atresia.

    Science.gov (United States)

    Cloutier, R

    1975-02-01

    Hyperplasia is the main change occurring in intestinal smooth muscle above a chronic obstruction and explains the functional obstruction seen in the proximal bowel of a jejunoileal atresia. With an experimental model in dogs, this hyperplasia has been shown to be reversible. However, changes are extreme in atresia, and experiments in animals with induced atresia will best evaluate various kinds of treatment.

  7. Immunological gap in the infectious animal model for biliary atresia.

    Science.gov (United States)

    Czech-Schmidt, G; Verhagen, W; Szavay, P; Leonhardt, J; Petersen, C

    2001-11-01

    Extrahepatic biliary atresia (EHBA), the etiology of which still remains unclear, occurs exclusively in newborns and has recently been simulated in an animal model. It is possible to trigger an EHBA corresponding to the human disease by means of intraperitoneal infection of newborn Balb/c mice with rhesus rotavirus (RRV). The aim of the present study was to determine the conditions and circumstances for inducing biliary atresia in this model focusing on first-line immunological aspects. Newborn as well as pregnant Balb/c mice were intraperitoneally infected with RRV. The highest incidence of cholestasis (86%) was achieved by infection with 10(6) PFU/ml RRV within the first 12 h postpartum, resulting in EHBA with a lethality of 100%. However, the later the newborn mouse is infected, the less likelihood there is that EHBA is triggered. Additionally, the incidence of biliary atresia in this model depends on the quantity of the virus that is given intraperitoneally. However, the development of biliary atresia is not correlated to the virus in the liver. The antepartum infection of pregnant mice does not induce EHBA in the offspring. Female mice that are immunized against RRV protect their newborns from developing RRV-induced cholestasis and EHBA. This protection is transmitted transplacentally and not by breast milk. It is obvious that a temporary immunological gap is essential for virally induced EHBA. Further studies should focus on specific parameters of the immune system of newborn mice in this biliary atresia model. Copyright 2001 Academic Press.

  8. Incidence of hepatotropic viruses in biliary atresia.

    Science.gov (United States)

    Rauschenfels, Stefan; Krassmann, Miriam; Al-Masri, Ahmed N; Verhagen, Willem; Leonhardt, Johannes; Kuebler, Joachim F; Petersen, Claus

    2009-04-01

    Biliary atresia (BA) is the most frequent indication for paediatric liver transplantation. We tested the hypothesis of a viral aetiology of this disease by screening liver samples of a large number of BA patients for the common human hepatotropic viruses. Moreover, we correlated our findings to the expression of Mx protein, which has been shown to be significantly up-regulated during viral infections. Seventy-four liver biopsies (taken during Kasai portoenterostomy) were tested by polymerase chain reaction (PCR) for DNA viruses (herpes simplex virus [HSV], Epstein-Barr virus [EBV], varicella zoster virus [VZV], cytomegalovirus [CMV], adenovirus, parvovirus B19 and polyoma BK) and RNA viruses (enteroviruses, rotavirus and reovirus 3). Mx protein expression was assessed by immunohistochemistry. Virus DNA/RNA was found in less than half of the biopsies (8/74 CMV, 1/74 adenovirus; 21/64 reovirus, 1/64 enterovirus). A limited number presented with double infection. Patients that had detectable viral RNA/DNA in their liver biopsies were significantly older than virus-free patients (P = 0.037). The majority (54/59) of the liver biopsies showed expression of Mx proteins in hepatocytes, bile ducts and epithelium. Our data suggest that the known hepatotropic viruses do not play a major role in the aetiology and progression of BA. Their incidence appears to be, rather, a secondary phenomenon. Nonetheless, the inflammatory response in the livers of BA patients mimics that observed during viral infections.

  9. Esophageal heterotopic gastric mucosa in esophageal atresia

    Directory of Open Access Journals (Sweden)

    Lachlan J.R. Harrison

    2018-05-01

    Full Text Available Heterotopic gastric mucosa (HGM is occasionally found at endoscopy in the proximal esophagus of adults and children, when it manifests as an asymptomatic small island of reddish pink mucosa just below the upper esophageal sphincter. There are few reports of esophageal HGM detected by endoscopy after repair of esophageal atresia (EA with tracheo-esophageal fistula (TEF. We report a child with multiple patches of HGM in the proximal and distal esophagus seen at endoscopy after EA/TEF repair. No obvious symptoms were related to the HGM and she remains under endoscopic surveillance. The incidence of esophageal HGM may be increased in patients with EA and its distribution can be more extensive than a simple “inlet patch”. There is evidence to suggest that esophageal HGM increases the risk of developing Barrett's esophagus and has a malignant potential. Heterotopic gastric mucosa extends the spectrum of potential pathologies affecting the esophagus in patients with EA/TEF and supports current international guidelines for endoscopic surveillance of these patients. Keywords: Tracheo-esophageal fistula, Ectopic mucosa, Esophageal malignancy

  10. Appendicitis with appendicular atresia: a rare presentation

    Science.gov (United States)

    Masood, Irfan; Majid, Zain; Rafiq, Ali; Fatima, Saba; Siddiqui, Osama Bin Zia

    2015-01-01

    Acute appendicitis is the most common acute surgical condition; making appendectomy the most commonly performed emergency surgical procedure in the world. Anomalies of the appendix are relatively uncommon. However, their presence may alter the course of pre-operative diagnosis and the surgical treatment provided, leading to medico-legal issues in certain cases as well. We hereby present the case of a 17 year-old female who had the suggestive signs, symptoms and investigations of appendicular lump. She was managed according to the Ochsner-Sherren regimen and then underwent interval open appendectomy 6 weeks later. During the procedure, the findings of a 5 cm long appendix were noted. The base of the appendix was attached to the caecum, however there was complete mucosal discontinuity between the base and the remaining portion of the appendix. A fibrous strand connected the two blind ending parts together. After thorough literature search, the authors concluded that this is only the fourth reported case of appendicular atresia ever to have been reported. Considering the rarity of this finding we feel this could be of valuable interest to surgeons and readers alike PMID:26090015

  11. Atresia biliar: continuamos operando tarde Biliary atresia: we still operate too late

    Directory of Open Access Journals (Sweden)

    Carlos O. Kieling

    2008-10-01

    Full Text Available OBJETIVO: Analisar a idade na cirurgia de crianças com atresia biliar e a sobrevida sem necessidade de transplante de fígado. O estudo foi realizado no Hospital de Clínicas de Porto Alegre. MÉTODOS: Foram revisados os prontuários dos pacientes operados entre 1982 e 2007, residentes no Rio Grande do Sul. RESULTADOS: Dos 112 casos estudados de crianças com atresia biliar, 38 (33,9% ocorreram de 1982 a 1989, 46 (41,1% de 1990 a 1999 e 28 (25,0% a partir de 2000. Em 12 (10,7% casos, não foi realizada a portoenterostomia. A idade na cirurgia variou de 25 a 297 dias (mediana: 80,5; IIQ25-75: 61,3-109,0 dias; em 20,5% dos casos, a idade das crianças foi menor do que 60 dias. Não houve diferença na idade, no momento do diagnóstico, entre as 3 décadas. Os pacientes do interior do estado (mediana: 87,0; IIQ25-75: 69,0-115,0 dias foram encaminhados significativamente (p = 0,007 mais tarde do que os da região metropolitana de Porto Alegre (RS (mediana: 68,0; IIQ25-75: 55,5-98,0 dias. A proporção de pacientes com menos de 60 dias foi significativamente menor (p = 0,013 nos oriundos do interior. A sobrevida com fígado nativo do total dos pacientes foi de 46,2% em 2 anos, diminuindo progressivamente até 15,3% em 20 anos. Os pacientes operados com menos de 60 dias tiveram maior sobrevida com fígado nativo (log rank OBJECTIVE: To analyze the age at surgery for children with biliary atresia and their survival periods without need for liver transplantation. The study was performed at Hospital de Clínicas de Porto Alegre, in Porto Alegre, state of Rio Grande do Sul, Brazil. METHODS: The medical records of patients operated between 1982 and 2007 who were residents of the state of Rio Grande do Sul were reviewed. RESULTS: Of the 112 cases of children with biliary atresia studied, 38 (33.9% occurred between 1982 and 1989, 46 (41.1% between 1990 and 1999 and 28 (25.0% after 2000. Portoenterostomy was not performed for 12 cases (10.7%. Age at surgery

  12. [Colorimetric card use for early detection visual biliary atresia].

    Science.gov (United States)

    Reyes-Cerecedo, Alicia; Flores-Calderón, Judith; Villasis-Keever, Miguel Á; Chávez-Barrera, José A; Delgado-González, Elba E

    2018-01-01

    Bile duct atresia (BVA) is a condition that causes obstruction to biliary flow, not corrected surgically, causes cirrhosis and death before 2 years of age. In Mexico from 2013 the visual colorimetric card (VVC) was incorporated for the timely detection of BVA to the National Health Card (NHC). The aim of this study was to evaluate the impact of VCT for the detection of BVA before and after the use of NHC incorporation. Ambispective, analytical observational study. We included patients with AVB treated in two pediatric hospitals of third level care. We compared the age of reference, diagnosis and surgery before and after incorporation of the TCV. In addition, a questionnaire was made to the parents to know their perception about the TCV. In 59 children, there were no differences in age at diagnosis (75 vs 70 days) and age at surgery (84 vs 90 days) between the pre and post-implementation period of the VVC. The questionnaire showed that 10 (30%) of the parents received information about the use of the VVC and 13 (38%) identified the abnormal evacuations. This study did not show changes in time for the timely detection of BVA by using VVC. Therefore, it is necessary to reinforce the program in the three levels of care in our country. La atresia de vías biliares (AVB) es una condición que provoca obstrucción al flujo biliar, y de no corregirse quirúrgicamente, provoca cirrosis y la muerte antes de los 2 años de edad. En México, a partir del año 2013 se incorporó la tarjeta colorimétrica visual (TCV) para la detección oportuna de la AVB a la Cartilla Nacional de Salud (CNS). El objetivo de este estudio fue evaluar el impacto de la TCV para la detección de AVB antes y después de su incorporación a la CNS. Estudio ambispectivo, observacional y analítico. Se incluyeron pacientes con AVB atendidos en dos hospitales pediátricos de tercer nivel de atención. Se compararon la edad de referencia, el diagnóstico y la cirugía antes y después de la incorporaci

  13. Imaging findings of bronchial atresia in fetuses, neonates and infants

    Energy Technology Data Exchange (ETDEWEB)

    Alamo, Leonor; Meuli, Reto [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Diagnostic and Interventional Radiology, Lausanne (Switzerland); Vial, Yvan [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Obstetrics and Gynecology, Lausanne (Switzerland); Gengler, Carole [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Pathology, Lausanne (Switzerland)

    2016-03-15

    Congenital lung malformations are increasingly detected before birth. However, bronchial atresia is rarely identified in utero and not always recognized in neonates. There are two types of atresia: (1) proximal, located at the level of the mainstem or the proximal lobar bronchi, which is extremely rare and usually lethal during pregnancy, causing a tremendous volume increase of the distal involved lung with secondary hypoplasia of the normal lung, and (2) peripheral, located at the segmental/subsegmental bronchial level, which may present as an isolated lesion or as part of a complex congenital malformation. Prenatal findings are mostly nonspecific. Postnatal exams show overinflated lung areas and focal bronchial dilations. The typical fluid-filled bronchoceles are not always observed in neonates but develop progressively in the first months of life. This pictorial essay describes the spectrum of imaging findings of bronchial atresia in fetuses, neonates and infants. (orig.)

  14. Mammalian follicular development and atresia: role of apoptosis.

    Science.gov (United States)

    Asselin, E; Xiao, C W; Wang, Y F; Tsang, B K

    2000-01-01

    The regulation of follicular development and atresia is a complex process and involves interactions between endocrine factors (gonadotropins) and intraovarian regulators (sex steroids, growth factors and cytokines) in the control of follicular cell fate (i.e. proliferation, differentiation and programmed cell death). Granulosa and theca cells are key players in this fascinating process. As atresia is the fate of most follicles, understanding of how these physiological regulators participate in determining the destiny of the follicle (to degenerate or to ovulate) at cellular and subcellular levels is fundamental. This short review summarizes the role of intraovarian modulators of programmed cell death in the induction of atresia during follicular development. Copyright 2000 S. Karger AG, Basel

  15. Post-operative abdominal CT scanning in extrahepatic biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Day, D L; Mulcahy, P F; Letourneau, J G; Dehner, L P

    1989-07-01

    A retrospective review of the abdominal CT scans of 26 children with extrahepatic biliary atresia was performed, and the results were correlated with available surgical and pathologic data. Associated congenital anomalies or acquired abnormalities were identified in these patients. Congenital anomalies included polysplenia, venous anomalies and bowel stenosis. Acquired abnormalities developed secondary to cirrhosis, portal hypertension, intrahepatic biliary duct dilatation, and hepatic ischemia. Despite frequent episodes of ascending cholangitis in these children, no hepatic abscesses were identified by CT or by pathologic examination. In conclusion, abdominal CT scanning of children with extrahepatic biliary atresia can define congenital and acquired abnormalities and provide important anatomic data for the surgeons before liver transplantation. (orig.).

  16. Apple-peel atresia presenting as foetal intestinal obstruction

    Directory of Open Access Journals (Sweden)

    Ashok Yadavrao Kshirsagar

    2011-01-01

    Full Text Available Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed signs of intestinal obstruction on day one after birth, for which an exploratory laparotomy was performed. Type 3 JIA was found for which resection of atretic segments with jejuno-ascending colon anastomosis was preformed.

  17. Biliary atresia and cerebellar hypoplasia in polysplenia syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Vanderdood, Kurt; Op de Beeck, Bart; Desprechins, Brigitte; Osteaux, Michel [Department of Radiology, Free University Brussels, AZ-VUB, Laarbeeklaan 101, 1090 Brussels (Belgium)

    2003-09-01

    We report a 3.5-month-old boy with polysplenia syndrome who demonstrated hemiazygos continuation of the inferior vena cava, extrahepatic biliary atresia, multiple splenunculi, bowel malrotation, and the rare finding of brainstem and cerebellar hypoplasia. A possible pathogenesis for cerebellar hypoplasia in this syndrome is suggested after review of the literature. The importance of seeking associated anomalies in biliary atresia, which may be possible indicators of polysplenia syndrome, is stressed since these patients need appropriate management when surgery is considered. (orig.)

  18. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras Extrahepatic biliary atresia: current concepts and future directions

    OpenAIRE

    Elisa de Carvalho; Cláudia Alexandra Pontes Ivantes; Jorge A. Bezerra

    2007-01-01

    OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed) pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal in...

  19. Long survival ( 21 years) after portoenterostomy for biliary atresia: A ...

    African Journals Online (AJOL)

    Long term survival for decades after portoenterostomy (Kasai procedure) for biliary atresia is rare and the association of portoenterostomy with liver cirrhosis is well known. Not much attention was given in the evaluation of the imaging features of cirrhosis caused by portoenterostomy as received by other known usual ...

  20. Biliary Atresia – An Easily Missed Cause of Jaundice amongst ...

    African Journals Online (AJOL)

    Back ground: Biliary atresia is characterized by biliary obstruction, it has an incidence of 1:15000 and presents with jaundice, acholic stools / dark urine and hepatomegaly. This disease rapidly leads to liver cirrhosis and liver failure if untreated surgically. The main objective was to establish the epidemiology of patients ...

  1. The "boomerang" malleus-incus complex in congenital aural atresia.

    Science.gov (United States)

    Mukherjee, S; Kesser, B W; Raghavan, P

    2014-01-01

    "Boomerang" malleus-incus fusion deformity is identified on axial high-resolution CT in a subset of patients with congenital aural atresia, and it is associated with an absent incudostapedial joint and stapes capitulum and attachment of the incus to the tympanic segment of the facial nerve canal. Twelve patients with this deformity were identified on a retrospective review of imaging from a cohort of 673 patients with congenital aural atresia, with surgical confirmation in 9 of these patients. Eight of 9 patients underwent partial ossicular replacement prosthesis reconstruction with improvement in hearing outcome. We hypothesize that the boomerang anomaly represents a more severe ossicular anomaly than is normally seen in congenital aural atresia, arising from an arrest earlier in the embryonic development of the first and second branchial arch. This has potentially important implications for surgical planning because hearing outcomes with placement of prosthesis may not be as good as with conventional atresia surgery, in which reconstruction is performed with the patient's native ossicular chain. © 2014 by American Journal of Neuroradiology.

  2. Ulcerative Colitis in Colonic Interposition for Esophageal Atresia

    OpenAIRE

    Arshad, Hafiz Muhammad Sharjeel; Tetangco, Eula; Elkhatib, Imad

    2016-01-01

    A 38-year-old male with a history of colonic interposition for esophageal atresia as an infant presented with dysphagia and abdominal pain. On the basis of endoscopy findings, pathology, and response to therapy, he was found to have ulcerative colitis of the colonic conduit.

  3. Thirty-four years' experience with biliary atresia in Denmark

    DEFF Research Database (Denmark)

    Kvist, N; Davenport, M

    2011-01-01

    Biliary atresia (BA) is a rare disease in Denmark (population ∼ 5.5 million) and there has been some controversy on how smaller countries should manage such conditions to obtain the best possible outcomes. The aim of this study was to evaluate the efficacy of primary surgery (i. e., Kasai portoen...

  4. CASE REPORT CASE Unusual case of pulmonary valve atresia

    African Journals Online (AJOL)

    atresia with a ventricular septal defect (VSD) and anomalous origin of the left pulmonary artery arising from ... Anomalous origin of either pulmonary artery branch from the aorta has been reported. The association of ... Department of Radiology, Pretoria Academic Hospital and University of. Pretoria. F Takawira, MB ChB, FC ...

  5. Unusual case of pulmonary valve atresia | Steyn | SA Journal of ...

    African Journals Online (AJOL)

    We report on a 12-year-old boy with a rare form of pulmonary valve atresia with a ventricular septal defect (VSD) and anomalous origin of the left pulmonary artery arising from the aortic arch. He also has an absent right pulmonary artery, ... severe PHT of the left lung. South African Journal of Radiology Vol. 12 (1) 2008: pp.

  6. Bilateral Choanal Atresia: Highlighting The Role Of Computerised ...

    African Journals Online (AJOL)

    A neonate delivered at UCH Ibadan was diagnosed to have bilateral choanal atresia being membranous on the right side and bony on the left side by computed tomography. She had surgical correction of the right sided obstruction but died on the seventh post operative day due to septicemia. The literature has been ...

  7. Brain Oxygenation During Thoracoscopic Repair of Long Gap Esophageal Atresia

    NARCIS (Netherlands)

    Stolwijk, Lisanne J; van der Zee, David C; Tytgat, Stefaan; van der Werff, Desiree; Benders, Manon J N L; van Herwaarden, Maud Y A; Lemmers, Petra M A

    2017-01-01

    Background: Elongation and repair of long gap esophageal atresia (LGEA) can be performed thoracoscopically, even directly after birth. The effect of thoracoscopic CO2-insufflation on cerebral oxygenation (rScO2) during the consecutive thoracoscopic procedures in repair of LGEA was evaluated.

  8. Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case ...

    African Journals Online (AJOL)

    Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case report and literature ... skin over the right leg from the knee joint up to the middle of the right foot. ... Examination indicated no signs of child abuse; the parents refused an ...

  9. Repair of oesophageal atresia with tracheo- oesophageal fistula ...

    African Journals Online (AJOL)

    Right thoracotomy for oesophageal atresia (OA) with dextrocardia is technically challenging due to the heart being in the operative field, and also due to the possibility of right-sided aortic arch. We report a neonate with long- gap OA with tracheo-oesophageal fistula (TOF), dextrocardia, and left-sided aortic arch who was.

  10. European biliary atresia registries: summary of a symposium

    DEFF Research Database (Denmark)

    Petersen, C.; Harder, D.; Abola, Z.

    2008-01-01

    , centralization of surgery). In conclusion, EBAR represents the first attempt at an overall evaluation of the outcome of BA from a pan-European perspective. The natural history and outcome of biliary atresia is of considerable relevance to a European population. It is essential that there is further support...

  11. Anaesthesia for oesophageal atresia with or without tracheo ...

    African Journals Online (AJOL)

    Oesophageal atresia, with or without tracheo-oesophageal fistula, is one of the most challenging conditions with which the anaesthesiologist has to deal during the perioperative period. The patients are usually in their first few days of life, and might be premature with inherent airway problems. This is then compounded by ...

  12. Unilateral pulmonary veins atresia: evaluation by computed tomography; Atresia unilateral das veias pulmonares: avaliacao por tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Eifer, Diego Andre; Arsego, Felipe Veras, E-mail: felipesoarestorres@gmail.com [Hospital de Clinicas de Porto Alegre (HCPA), RS (Brazil). Serv. de Radiologia; Torres, Felipe Soares [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Escola de Medicina

    2013-11-15

    Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient. (author)

  13. Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia.

    Science.gov (United States)

    Kim, Minbum

    2015-08-01

    The occurrence of oval window atresia is a rare anomaly with conductive hearing loss. Traditional atresia surgeries involve challenging surgical techniques with risks of irreversible inner ear damage. Recent reports on Bonebridge (Medel, Innsbruck, Austria), a novel implantable bone conduction hearing aid system, assert that the device is safe and effective for conductive hearing loss. We present a case of Bonebridge implantation in an eight-year-old girl with bilateral oval window atresia.

  14. BIRTH INJURY RELATED UNILATERAL ANTERIOR NARES PARTIAL FIBROUS ATRESIA: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Jayagar

    2016-02-01

    Full Text Available Congenital atresia of anterior nares has been rarely reported and it may co-exist along with posterior choanal atresia. (1,2 In our case, birth injury caused by forceps delivery has caused unilateral anterior nares partial atresia. Twenty eight years old male patient came with the complaints of left nostril blocked sensation since birth, aggravated with attacks of upper respiratory tract infections. On examination he had left anterior nares partial atresia caused by fibrous bands as a result of birth injury due to instrumental delivery. Rhinoplasty performed to open-up left nostril and patient relieved of his symptoms and also on cosmetic appearance

  15. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras

    OpenAIRE

    Carvalho,Elisa de; Ivantes,Cláudia Alexandra Pontes; Bezerra,Jorge A.

    2007-01-01

    OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed) pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal in...

  16. Congenital esophageal atresia with tracheo-esophageal fistula

    International Nuclear Information System (INIS)

    Rhee, Chung Sik

    1970-01-01

    Three cases of esophageal atresia with tracheo-esophageal fistula. 1). Case 1: A female infant birth Wt. 1.95 kg , Apgar Score 10, Skeletal anomalies, was delivered after a pregnancy compeicated by hydroamnious on Aug. 17, 1970. The family history was not contributory. 2) Case 2: A male infant birth Wt. 2.8 kg , Apgar Score 8, was forcep delivered after a pregnancy on Feb. 8, 1970. This infant is twin. The family history was not contributory. 3) Case 3: A female infant birth Wt. 2.22 kg , Apgar Score 10, was C-section after a pregnancy on May, 16. 1970. The family history was not contributory. All cases: After 24 hours 5% glucose solution was given and immediately vomited and some of it regurgitating through the nose and mouth with associated cyanosis and dyspnea. A catheter was inserted through the nose into the esophagus under diagnosis of the esophageal atresia

  17. A newborn with duodenal atresia and a gastric perforation

    Directory of Open Access Journals (Sweden)

    Akcora Bulent

    2010-01-01

    Full Text Available Congenital duodenal atresia complicated by gastric perforation (GP is a very rare and a very mortal condition. Only three newborns could be cured in the reported 13 cases. We report a successfully treated newborn with this complicated disease. A 2-day-old male was hospitalized with prediagnosis of duodenal obstruction. Twelve hours later, significant abdominal distention occurred promptly. At laparotomy, GP and preampullary duodenal atresia were detected. Gastrorrhaphy and duodenoduodenostomy were performed in the same operation. The patient was discharged on the 15th postoperative day. This complicated disease can be treated by early diagnosis and surgical intervention. We choose one-stage operation because of the clean peritoneal cavity. However, generalized peritonitis may require two-stage operation in delayed cases.

  18. Pregnancy Complicated by Portal Hypertension Secondary to Biliary Atresia

    Directory of Open Access Journals (Sweden)

    O. E. O'Sullivan

    2013-01-01

    Full Text Available Biliary atresia is a rare idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extrahepatic biliary ducts. As the disease is progressive all cases will develop portal fibrosis, cirrhosis, and portal hypertension with the sequelae of varices, jaundice, and eventually liver failure requiring a transplant. Survival rates have improved considerably with many females living well in to be childbearing age. Due to the complexity of the disease these pregnancies are considered, high risk. We report the antenatal, intrapartum, and postpartum managements of a pregnancy complicated by biliary atresia. Furthermore, we highlight the importance of a multidisciplinary team approach in optimizing obstetric care for this high risk group.

  19. MRI in children following surgery for anal and rectal atresia

    International Nuclear Information System (INIS)

    Krahe, T.; Herold, A.; Doelken, W.; Hoecht, B.; Wuerzburg Univ.

    1989-01-01

    MRI of the pelvis was performed in 17 children following surgical correction of anal and rectal atresias and in five children without ano-rectal malformations. A muscle score was used to characterize the muscles of the pelvic floor and their relationship to the rectum. There was close agreement between the MRI muscle score and clinical continence. MRI provided additional information that should improve continence following conservative and surgical treatment. (orig.) [de

  20. Congenital Pyloric Atresia; a report of two cases

    International Nuclear Information System (INIS)

    Tayeb, Maaen; Khogeer, Suzie; Fallatah, Amna; Hamchou, Mustafa A.

    2005-01-01

    Congenital pyloric atresia (CPA) is a very rare malformation that constitutes less than 1% of all gas upper gastrointestinal atresias. It is a unique malformation that is commonly seen as an isolated lesion, but can also occur in association with other genetically determined conditions such as hereditary multiple intestinal atresias (HMIA). This is a report of two cases of isolated isolated CPA, outlining aspects of diagnosis and management. A 2-day-old female, a product of 35-weeks gestation via a low cesarean delivery due to a transverse lie to a 25-year-old mother who had gestational insulin dependent diabetes and polyhydraminos was referred because of non-bile stained vomiting. Her abdominal x-ray showed dilated stomach with no gas distally. Gastrograffin meal confirmed the diagnosis of gastric outlet obstruction. She was found to have pyloric artesia. This was excised via longitudinal incision in the pylorus, which was then closed transversely. Subsequently she did well. A 1-year -old female was evaluated because of persistent non-bile stained vomiting. Abdominal x-ray showed dilated stomach with no gas distally and barium meal confirmed the diagnosis of gastric outlet obstruction. She was operated and on during surgery was found to have congenital pyloric atresia. This was excised via longitudinal incsion in the pylorus. She did well. CPA is divided in three types: 1) Pyloric membrane 2) Pyloric artesia without a gap 3) Pyloric artesia with a gap. Both our patients have pyloric diaphagrams. The treatment of CPA is surgical and depends on the type of arteseia. For those with pyloric diaphagram or pyloric artesia without a gap the treatment is excision of diphagaram. This is also of importance in case there is more than one diaphagram. For those with pyloric artesia with a gap, if the gap is short, they should be treated with a Finny or Heineke-Mickulicz pyloroplasty, but if the gap is long then a gastroduodenostomy becomes the treatment of choice

  1. Evaluation of the angiographic findings in pulmonary atresia

    International Nuclear Information System (INIS)

    Choe, Kyu Ok; Sul, Jun Hee; Lee, Seung Kyu; Cho, Bum Koo; Hong, Pill Whoon

    1986-01-01

    We studied the angiographic findings in 65 patients with congenital pulmonary atresia, ages 4 days to 14 years (mean 3.3 yrs), from 1981 to 1986 at Severance Hospital Yonsei University. 1. 6 had pulmonary atresia with an intact interventricular septum, 38 had it with cardiac anomaly Renodynamically simulating TOF, and 21 associated with more complicated cardiac anomalies. 2. In the group with an intact ventricular septum, 5 showed confluent intrapericardial pulmonary artery, all segmental pulmonary arteries connected to intrapericardial artery. 3. In the group simulating TOF, aorta arose from RV with or without overriding in 35. In 27 patients with confluent intrapericardial pulmonary artery, 23 had more than 10 segmental pulmonary arteries connected to intrapericardial artery and 5 had severely hypoplastic hilar pulmonary arteries. In 11 with non confluent intrapericardial pulmonary artery, 4 had more than 10 segmental pulmonary arteries connected to central pulmonary artery and 9 had severely hypoplastic hilar pulmonary arteries. 4. In the group associated with more complicated cardiac anomaly, included 8 patients with atrioventricular discordance, 7 with univentricular heart and 6 with tricuspid atresia. In 17 patients with confluent intrapericardial artery, 16 had more than 10 segmental pulmonary arteries connected to intrapericardial artery, one showed severe hypoplasia of hilar pulmonary arteries. In another 4 with non confluence, no one showed more than 10 segmental arteries connected to intrapericardial or hilar pulmonary artery.

  2. False Computed Tomography Findings in Bilateral Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Elsheikh, Ezzeddin

    2016-01-01

    Full Text Available Introduction Choanal atresia (CA is a challenging surgical problem defined as a failure in the development of communication between the nasal cavity and nasopharynx. Objective The objective of this study is to describe computed tomography (CT findings in cases with bilateral choanal atresia. Methods The study involved performing axial and coronal non-contrast CT scanning with 2–3 mm sections on14 neonates that had bilateral CA. We used fiberoptic nasal endoscopy to confirm the diagnosis. We evaluated coronal CT to study the skull base area in such neonates. Results This study included 14 neonates with bilateral CA; with mean age of 7 ± 3.5 days. Mixed atretic plates were found in 12 (85.7% cases while two (14.3% had pure bony atresia. Isolated CA was detected in 9 cases (64.3% and 5 (35.7% cases had associated anomalies. Coronal CT showed soft tissue density in the nasal cavity that appeared to extend through an apparent defect in the nasal roof (cribriform plate, falsely diagnosed by radiologists as associated encephalocele. At the time of surgical repair, all patients showed thick tenacious mucous secretions in both nasal cavities and revealed no encephalocele. Nasal roof remained intact in all cases. Conclusion The thick secretion of bilateral CA could give a false encephalocele appearance on the CT. It is highly recommended to perform proper suction of the nasal cavity of suspected CA cases just before CT scanning.

  3. Dysphagia in Children with Esophageal Atresia: Current Diagnostic Options.

    Science.gov (United States)

    Rayyan, Maissa; Allegaert, Karel; Omari, Taher; Rommel, Nathalie

    2015-08-01

    Dysphagia or swallowing disorder is very common (range, 15-52%) in patients with esophageal atresia. Children present with a wide range of symptoms. The most common diagnostic tools to evaluate esophageal dysphagia, such as upper barium study and manometry, aim to characterize anatomy and function of the esophageal body and the esophagogastric junction (EGJ). Using these technologies, a variety of pathological motor patterns have been identified in children with esophageal atresia. However, the most challenging part of diagnosing patients with esophageal dysphagia lies in the fact that these methods fail to link functional symptoms such as dysphagia with the esophageal motor disorders observed. A recent method, called pressure-flow analysis (PFA), uses simultaneously acquired impedance and manometry measurements, and applies an integrated analysis of these recordings to derive quantitative pressure-flow metrics. These pressure-flow metrics allow detection of the interplay between bolus flow, motor patterns, and symptomatology by combining data on bolus transit and bolus flow resistance. Based on a dichotomous categorization, flow resistance at the EGJ and ineffective esophageal bolus transit can be determined. This method has the potential to guide therapeutic decisions for esophageal dysmotility in pediatric patients with esophageal atresia. Georg Thieme Verlag KG Stuttgart · New York.

  4. Treatment strategies in the management of jejunoileal and colonic atresia

    Directory of Open Access Journals (Sweden)

    Chadha Rajiv

    2006-01-01

    Full Text Available BACKGROUND/PURPOSE: The purpose of this prospective study was to review the operative findings, treatment strategies, as well as the results of management of 46 consecutive cases of jejunoileal and colonic atresia, managed over a 2-year period. MATERIALS AND METHODS: There were 42 patients with jejunoileal atresia (JIA and 4 with colonic atresia (CA. The 4 group types were: type I-membranous (n=20, type II- blind ends separated by a fibrous cord (n=6, type IIIa- blind ends with a V-shaped mesenteric defect (n=10, type IIIb- apple-peel atresia (n=4 and type IV- multiple atresias (n=6. Primary surgery for JIA consisted of resection with a single anastomosis (n=37, anastomosis after tapering jejunoplasty (n=3, multiple anastomosis (n=1 and a Bishop-Koop ileostomy (n=1. For CA, resection with primary anastomosis was performed. A single end-to-oblique anastomosis after adequate resection of dilated proximal bowel, was the preferred surgical procedure. In the absence of facilities for administering TPN, early oral/nasogastric (NG tube feeding was encouraged. In patients with anastomotic dysfunction, conservative treatment of the obstruction followed after its resolution by gradually increased NG feeds, was the preferred treatment protocol. RESULTS: Late presentation or diagnosis with hypovolemia, electrolyte imbalance, unconjugated hyperbilirubinemia (n=25 and sepsis (n=6, were significant preoperative findings. After resection and anastomosis, significant shortening of bowel length was seen in 16 patients (34.7%. Postoperative complications included an anastomotic leak (n=3, a perforation proximal to the anastomosis in 1 and anastomotic dysfunction in 5 patients. Full oral or NG tube feeding was possible only by the 13th to 31st postoperative day (POD, after the primary surgery in patients with anastomotic dysfunction and those undergoing reoperation. Overall, 38 patients survived (82.6%. Mortality was highest in patients with type IIIb or type IV

  5. Fertility, pregnancy, and delivery after biventricular repair for pulmonary atresia with an intact ventricular septum

    NARCIS (Netherlands)

    Drenthen, Willem; Pieper, Petronella G.; Roos-Hesselink, Jollen W.; Zoon, Nicole; Voors, Adrlaan A.; Mulder, Barbara J. M.; van Dijk, Arie P. J.; Vliegen, Hubert W.; Sollie, Krystyna M.; Ebels, Tjark; van Veldhuisen, Dirk J.

    2006-01-01

    The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34

  6. Fertility, pregnancy, and delivery after biventricular repair for pulmonary atresia with an intact ventricular septum.

    NARCIS (Netherlands)

    Drenthen, W.; Pieper, P.G.; Roos-Hesselink, J.W.; Zoon, N.; Voors, A.A.; Mulder, B.J.M.; Dijk, A.P.J. van; Vliegen, H.W.; Sollie, K.M.; Ebels, T.; Veldhuisen, D.J. van

    2006-01-01

    The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34

  7. Development of infantile hypertrophic pyloric stenosis in patients treated for oesophageal atresia. A case report

    DEFF Research Database (Denmark)

    Qvist, N; Rasmussen, L; Hansen, L P

    1986-01-01

    Two cases of infantile hypertrophic pyloric stenosis (IHPS) developed in 74 patients treated for oesophageal atresia. Treatment of oesophageal atresia is frequently followed by vomiting and failure to thrive due to gastrooesophageal reflux or anastomotic stricture. The diagnose of IHPS must...

  8. Duodenal atresia with 'apple-peel configuration' of the ileum and ...

    African Journals Online (AJOL)

    According to the current understanding, duodenal atresia is considered to be a primary malformation resulting from the errors in recanalisation in early gestation. We report a rare case of duodenal atresia with apple-peel configuration of remaining small bowel with absent superior mesenteric artery in a preterm child, which ...

  9. Role of Multislice Computed Tomography and Local Contrast in the Diagnosis and Characterization of Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Khaled Al-Noury

    2011-01-01

    Full Text Available Objective. To illustrate the role of multislice computed tomography and local contrast instillation in the diagnosis and characterization of choanal atresia. To review the common associated radiological findings. Methods. We analyzed 9 pediatric patients (5 males and 4 females with suspected choanal atresia by multislice computed tomography. We recorded the type of atresia plate and other congenital malformations of the skull. Results. Multislice computed tomography with local contrast installed delineated the posterior choanae. Three patients had unilateral mixed membranous and bony atresia. Three patients had unilateral pure bony atresia. Only 1 of 7 patients have bilateral bony atresia. It also showed other congenital anomalies in the head region. One patient is with an ear abnormality. One patient had congenital nasal pyriform aperture stenosis. One of these patients had several congenital abnormalities, including cardiac and renal deformities and a hypoplastic lateral semicircular canal. Of the 6 patients diagnosed to have choanal atresia, 1 patient had esophageal atresia and a tracheoesophageal fistula. The remaining patients had no other CHARGE syndrome lesions. Conclusions. Local Contrast medium with the application of the low-dose technique helps to delineate the cause of the nasal obstruction avoiding a high radiation dose to the child.

  10. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo; Park, Sang-Hyub; Koo, Hyun Jung; Cho, Young Hoon; Lee, Eunsol [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea, Republic of)

    2014-08-15

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  11. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    International Nuclear Information System (INIS)

    Goo, Hyun Woo; Park, Sang-Hyub; Koo, Hyun Jung; Cho, Young Hoon; Lee, Eunsol

    2014-01-01

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  12. [Laparoscopic Kasai portoenterostom: present and future of biliary atresia treatment].

    Science.gov (United States)

    Ayuso, L; Vila-Carbó, J J; Lluna, J; Hernández, E; Marco, A

    2008-01-01

    Kasai's operation has proved its value in surgical treatment of biliary atresia (BA). Its laparoscopic approach is a new challenge for pediatric surgeons, with all the potential advantages of minimally invasive surgery. The aim of the present study has been to report our experience in laparoscopic management of five patients with biliary atresia. The average of age of five patients with biliary atresia, three boys and two girls was 58 days (range 40-64). Pre and postoperative management included antibiotic prophylaxis and choleretic treatment. Laparoscopic procedure was accomplished using one umbilical 10-mm trocar and two additional 5-mm trocars. We carried out the same technique in all the patients except in one of them with a total situs inversus and who compelled us to modify the original procedure. All five patients underwent a laparoscopic procedure, conversion was not necessary. The mean surgical time was 3 hours and 40 minutes (range: 5:30 y 3:10). There were not intra operative complications and all of them had a satisfactory recovery, except for the patient with situs inversus, who suffered a small bowel volvulus 9 days after the operation, leading us to perform an extensive bowel resection. All the patients, except this one, showed signs of adequate bile flow, with disappearance of clinical cholestasis. Biochemistry test became normal. Besides the certain advantages compared with conventional surgical procedures (lower surgical damage, diminished post-operative recovery), laparoscopic management of BA, allows a better exposure of the porta hepatis without hepatic mobilization so it shows similar or better preliminary results than conventional techniques. The advantages of laparoscopic portoenterostomy are yet to be proved whenever liver transplantation is indicated.

  13. Definitive management of isolated esophageal atresia: Experience at NICH Karachi

    Directory of Open Access Journals (Sweden)

    Jan Iftikhar

    2006-01-01

    Full Text Available Background: Definitive treatment of isolated esophageal atresia (IEA is still controversial. A study was conducted to review cases of IEA in our department with a view to evaluate the long term results of definitive surgery in these patients. Materials and Methods: Nine consecutive patients with IEA were included in the study. All babies initially underwent cervical esophagostomy and feeding gastrostomy. One baby also had anorectal atresia and needed a colostomy. In 6 babies, end esophagostomy was performed while in 3 babies, lateral esophagostomy was performed. Of the 6 babies with end esophagostomy, two underwent jejunal Interpositioning, two had serial extra-thoracic lengthening and two reverse gastric tube interpositiong. Three babies with lateral esophagostomy were planned for delayed primary repair. Results: Both babies with jejunal interpositiong initially did well; serious gastro-esophageal reflux occurred in one baby who needed antireflux surgery. At 6 years follow-up both children had redundant Jejunum. Recurrent Respiratory tract infection remained a problem in one child while other is doing well. Of the two babies with extrathoracic lengthening it was possible to perform end to end anastomosis in one baby but in the other extrathoracic lengthening did not work and gastric transpositioning was performed. Of the two babies with reverse gastric tube interpositioning one developed esophageal stenosis at the site of anastomosis and is on serial dilatations. Other died due to anastomotic leak and mediastinitis. In all the three babies with lateral esophagostomy spontaneous lengthening of esophagus was noted. End to end anastomosis was possible in two of these babies who are doing well. One is waiting definitive surgery. Number of complications, hospital stay and cost of surgery was least in patients with lateral esophagostomy. Conclusion: Various methods of definitive treatment have been proposed for isolated esophageal atresia but each

  14. Biliary atresia: lessons learned from the voluntary German registry.

    Science.gov (United States)

    Leonhardt, J; Kuebler, J F; Leute, P J; Turowski, C; Becker, T; Pfister, E-D; Ure, B; Petersen, C

    2011-03-01

    Aim of the study was to carry out a 5-year survey of German patients with biliary atresia (BA) and to launch a discussion regarding the feasibility of voluntary registries in unregulated healthcare systems. A retrospective analysis of German BA patients born between 2001 and 2005, based on data collected from the voluntary European Biliary Atresia Registry (EBAR), was carried out and supplemented by data from all BA patients who underwent liver transplantation at the only 4 pediatric transplantation centers (pLTx) in Germany which are so far not registered at EBAR. Survival rates were calculated using Kaplan-Meier analysis and compared by Cox regression to determine the predictive value of age at surgery and the influence of the center size (fewer or more than 5 patients/study period) on overall survival and survival with native liver. A critical review of the 148 German EBAR charts revealed that 11 patients (7.4%) had no biliary atresia. The remaining 137 patients from EBAR together with 46 BA patients who underwent LTx without prior registration at EBAR were evaluated with a median follow-up of 39 months (range: 25-85 months). 29 hospitals performed a total of 159 Kasai procedures, but only 7 centers treated 5 or more patients (116 patients, range: 5-68), and 22 hospitals performed less than 5 KP (43 patients, range: 1-4). Primary LTx was performed in 21 patients (11.5%) and 3 patients died without surgical intervention. 16 patients were lost to follow-up (8.7%). Overall survival after 2 years was 83.3% (139 patients), including 105 patients (63%) who had undergone LTx and 34 patients (20.3%) with native liver. 28 patients died (16.7%), 8 after LTx (5.8%). The experience of the center was the only factor with a significant predictive value for jaundice-free survival with native liver (p=0.001). 25% of all German BA patients were not registered at EBAR, and 29 clinics were involved in the surgical management of BA patients. Therefore a new approach consisting of

  15. Tracheal Atresia with Segmental Esophageal Duplication: An Unusual Anatomic Arrangement.

    Science.gov (United States)

    Gaerty, Kirsten; Thomas, Joseph T; Petersen, Scott; Tan, Edwin; Kumar, Sailesh; Gardener, Glenn; Armes, Jane

    2016-01-01

    An unusual anatomic configuration of segmental tracheal agenesis/atresia with esophageal duplication on autopsy in a fetus that demised in utero at 29 weeks is reported. The mother was scanned initially for a cardiac anomaly at 20 weeks and on follow-up scan at 27 weeks had polyhydramnios and underwent amnioreduction. The final autopsy diagnosis was vertebral, ano-rectal, cardiac, tracheoesophageal, renal, and limb malformations (VACTERL). We discuss the autopsy findings along with the embryological mechanisms and compare the configuration with Floyd's classification for tracheal agenesis. The difficulties in prenatal diagnosis are discussed.

  16. Abnormalities of intrahepatic bile ducts in extrahepatic biliary atresia.

    Science.gov (United States)

    Raweily, E A; Gibson, A A; Burt, A D

    1990-12-01

    The infantile cholangiopathies are a group of conditions associated with neonatal jaundice, which include extrahepatic biliary atresia, paucity of intra-hepatic bile ducts and disorders associated with persistence of fetal biliary structures, the so-called ductal plate malformations. Although previously regarded as distinct entities, it has recently been suggested that they may represent parts of a disease spectrum in which the principal process is one of bile duct destruction, the morphological manifestations in individual cases being influenced by the stage of intra-uterine development at which such injury occurs and by the site within the biliary system at which there is maximum damage. To further examine this concept, we have studied liver biopsy specimens from 37 neonates with extrahepatic biliary atresia, with particular reference to abnormalities of the intrahepatic bile ducts. Paucity of intrahepatic ducts, defined as a bile duct: portal tract ratio of less than 0.9, was identified in six cases (16.2%). In eight cases (21.6%) we found concentric tubular ductal structures similar to those observed in ductal plate malformations. In one case, both abnormalities could be demonstrated. Our findings support the concept that there is overlap between the various types of infantile cholangiopathy.

  17. Management of Acquired Atresia of the External Auditory Canal.

    Science.gov (United States)

    Bajin, Münir Demir; Yılmaz, Taner; Günaydın, Rıza Önder; Kuşçu, Oğuz; Sözen, Tevfik; Jafarov, Shamkal

    2015-08-01

    The aim was to evaluate surgical techniques and their relationship to postoperative success rate and hearing outcomes in acquired atresia of the external auditory canal. In this article, 24 patients with acquired atresia of the external auditory canal were retrospectively evaluated regarding their canal status, hearing, and postoperative success. Acquired stenosis occurs more commonly in males with a male: female ratio of 2-3:1; it seems to be a disorder affecting young adults. Previous ear surgery (13 patients, 54.2%) and external ear trauma (11 patients, 45.8%) were the main etiological factors of acquired ear canal stenosis. Mastoidectomy (12/13) and traffic accidents (8/11) comprise the majority of these etiological factors. Endaural incision is performed in 79.2% and postauricular incision for 20.8% of cases during the operation. As types of surgical approach, transcanal (70.8%), transmastoid (20.8%), and combined (8.4%) approaches are chosen. The atretic plate is generally located at the bony-cartilaginous junction (37.5%) and in the cartilaginous canal (33.3%); the bony canal is involved in a few cases only. Preserved healthy canal skin, split- or full-thickness skin grafts, or pre- or postauricular skin flaps are used to line the ear canal, but preserved healthy canal skin is preferred. The results of surgery are generally satisfactory, and complications are few if surgical principles are followed.

  18. Congenital aplasia of the optic chiasm and esophageal atresia: a case report

    Directory of Open Access Journals (Sweden)

    Madonia Maurizio

    2011-08-01

    Full Text Available Abstract Introduction The complete absence of the chiasm (chiasmal aplasia is a rare clinical condition. Hypoplasia of the optic nerve and congenital nystagmus are almost invariably associated characteristics. Microphthalmos or anophthalmos are common features in chiasmal aplasia, while central nervous system abnormalities are less frequent. Esophageal atresia can be isolated or syndromic. In syndromic cases, it is frequently associated with cardiac, limb, renal or vertebral malformations and anal atresia. More rarely, esophageal atresia can be part of anophthalmia-esophageal-genital syndrome, which comprises anophthalmia or microphthalmia, genital abnormalities, vertebral defects and cerebral malformations. Here, a previously unreported case of chiasmal aplasia presenting without microphthalmos and associated with esophageal atresia is described. Case presentation Aplasia of the optic chiasm was identified in a Caucasian Italian 8-month-old boy with esophageal atresia. An ultrasound examination carried out at 21 weeks' gestation revealed polyhydramnios. Intrauterine growth retardation, esophageal atresia and a small atrial-septal defect were subsequently detected at 28 weeks' gestation. Repair of the esophageal atresia was carried out shortly after birth. A jejunostomy was carried out at four months to facilitate enteral feeding. The child was subsequently noted to be visually inattentive and to be neurodevelopmentally delayed. Magnetic resonance imaging revealed chiasmal aplasia. No other midline brain defects were found. His karyotype was normal. Conclusion If achiasmia is a spectrum, our patient seems to depict the most severe form, since he appears to have an extremely severe visual impairment. This is in contrast to most of the cases described in the literature, where patients maintain good--or at least useful-- visual function. To the best of our knowledge, the association of optic nerve hypoplasia, complete chiasmal aplasia, esophageal

  19. Branchial cleft anomaly, congenital heart disease, and biliary atresia: Goldenhar complex or Lambert syndrome?

    Science.gov (United States)

    Cohen, J; Schanen, N C

    2000-01-01

    The features of Goldenhar complex have been well-described and classically include branchial arch abnormalities, epibulbar dermoid and vertebral abnormalities. We have identified an infant with these features in association with complex congenital heart disease and intrahepatic biliary atresia. Although Lambert described an autosomal recessive disorder with an association of biliary atresia and branchial arch abnormalities, none of those cases had epibulbar dermoid. Diagnostic considerations in this case include inclusion of biliary atresia as a new feature in the expanding spectrum of the Goldenhar complex, versus Lambert syndrome with epibulbar dermoid.

  20. Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia

    DEFF Research Database (Denmark)

    Madsen, Stine Skipper; Kvist, Nina; Thorup, Jørgen

    2015-01-01

    INTRODUCTION: Biliary atresia is the leading cause of liver transplantation in children. It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four children are born every year with biliary atresia. Early correction of biliary obstruction is essential to prevent fatal biliary...... cirrhosis. The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of children with elevated level of serum bilirubin after two weeks of age. Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 μmol/l, and/or more than 20% of total bilirubin...

  1. Análisis estructural e inmunohistoquímico de la atresia folicular de Vanellus chilenis (Charadriidae e Himantopus melanurus (Recurvirostridae

    Directory of Open Access Journals (Sweden)

    Mirian Bulfon

    2011-07-01

    Full Text Available Se estudiaron los aspectos estructurales e inmunohistoquímicos de la atresia folicular en el ovario de Vanellus chilenis e Himantopus melanurus. Fueron utilizadas cinco hembras adultas de cada especie en fase de recrudescencia gonadal; las gónadas se extrajeron, pesaron, fijaron y procesaron con la técnica de inclusión en parafina. Las secciones se colorearon con hematoxilina-eosina, tricrómico de Mallory, reacción nuclear de Feulgen y para la marcación de células apoptóticas se usó la técnica de TUNEL. De acuerdo a las características morfohistológicas de los folículos atrésicos analizados, en ambas aves fueron identificados dos tipos de atresia: a no bursting o pared folicular intacta que comprende la atresia lipoidal (ovocitos primordiales y lipoglandular (folículos previtelogénicos y vitelogénicos pequeños y b bursting con ruptura de la pared folicular (folículos vitelogénicos mayores de 500 mm. En la fase gonadal estudiada se observaron folículos atrésicos lipoidales y lipoglandulares, siendo escasos los folículos bursting. La apoptosis, se detectó al inicio de la involución en las células granulosas de los folículos atrésicos lipoglandulares con la reacción de Feulgen y se corroboró con la técnica de TUNEL. Por el contrario los estadios finales de los diferentes tipos involutivos de las dos especies se caracterizaron por una notoria necrosis. En base a estos resultados se infiere que, la muerte celular es un mecanismo fisiológico normal en la remodelación del ovario de las aves estudiadas y que los procesos de apoptosis y necrosis están estrechamente relacionados con la involución de los folículos ováricos de estas aves.

  2. Seasonal prevalence and intensity of follicular atresia in Baltic cod Gadus morhua callarias L

    DEFF Research Database (Denmark)

    Kraus, Gerd; Tomkiewicz, Jonna; Diekmann, R.

    2008-01-01

    of the physical disector method and volume fraction (Delesse principle). Atretic oocytes were observed in 32% of the ovaries. Prevalence of atresia was independent of female size, but increased significantly with declining female condition from prespawning and through the spawning stages. The relative intensity...... of atresia, i.e. number of atretic oocytes in relation to normally developed vitellogenic oocytes, was low amounting to 1.4% on average. Similar to prevalence, relative intensity of atresia differed significantly between maturity stages and increased with decreasing female condition. The population egg loss......In the present study, 307 ovaries of eastern Baltic cod Gadus morhua callarias sampled during the prespawning and spawning season 2000 were analysed histologically to estimate the seasonal prevalence and intensity of atresia. The number of atretic oocytes per ovary was estimated using a combination...

  3. Biliary atresia with hyaline cartilage at the porta hepatis: a novel ...

    African Journals Online (AJOL)

    Biliary atresia is an important cause of liver disease and morbidity in infants with ... hypoglycemia, nocturnal feed, constipation, or previous hospitalization was present. ... A clinical diagnosis of neonatal cholestasis (BA) was considered.

  4. Epidemiology of small intestinal atresia in Europe: a register-based study.

    LENUS (Irish Health Repository)

    Best, Kate E

    2012-09-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe.

  5. Gastric diverticulum causing gastric outlet obstruction in the setting of duodenal atresia

    Directory of Open Access Journals (Sweden)

    Devashis Mukherjee

    2018-04-01

    Full Text Available Duodenal obstruction due to duodenal atresia occurs in 1 in 10,000 live births and is the most common type of intestinal obstruction in neonates [1–3]. Gastric outlet obstruction in the newborn period from causes other than hypertrophic pyloric stenosis is very uncommon [3]. Potential etiologies include gastric volvulus, antral web, and duplication cysts. Gastric diverticula in the infant is even more rare, with only a few case reports published, and only one describes a gastric diverticulum in the presence of a duodenal atresia [4–8]. In this report, we describe the first case of a gastric outlet obstruction due to a gastric diverticulum in the presence of duodenal atresia. Keywords: Duodenal atresia, Gastric diverticulum, Gastric outlet obstruction

  6. Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence

    DEFF Research Database (Denmark)

    Bjørsum-Meyer, Thomas; Herlin, Morten; Qvist, Niels

    2016-01-01

    Background: The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac...... defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases. Furthermore, we aimed to collect existing knowledge...... in the embryopathogenesis and genetics in order to discuss a possible link between the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome. Case presentation: Our first case was a white girl...

  7. Ovarian Follicular Atresia of Ewes during Spring Puerperium

    Directory of Open Access Journals (Sweden)

    Radoslava Vlčková

    2012-01-01

    Full Text Available The distribution of healthy and atretic follicles on the ovarian surface of improved Valachian ewes 17, 24, and 32 days postpartum is reported in this study. The number of healthy follicles was higher on day 24 postpartum and their mean diameter tended to increase to day 32 (P<0.05 with the greatest diameter of 5 mm. 78–81% of atretic follicles ≥3 mm in diameter was observed where apoptosis began in the follicular cells situated at the follicular cavity. The early atretic follicles are characterized by the presence of mitotic pictures. In one ewe 24 days postpartum, small regressive follicular cysts were observed. Contracting atresia is characterized by thickening of the theca interna even to 190 μm. Progesterone and oestradiol-17β concentrations were maintained at relatively low levels, but with no significant difference between the days postpartum.

  8. Persistent bronchography in a newborn with esophageal atresia

    Directory of Open Access Journals (Sweden)

    Giuseppe De Bernardo, MD

    2016-06-01

    Full Text Available Esophageal atresia (EA with tracheoesophageal fistula occurs in about 1:2,500 births. We report a case of persistent bronchography in a newborn with EA and distal tracheoesophageal fistula. A large amount of barium sulfate was injected for mistake by a tube directly in the right bronchus to evaluate the patency of the esophagus. The infant showed signs of respiratory distress; he was intubated and transported at children's Hospital Santobono Pausilipon. Here, it was performed a chest X-ray that confirmed the opacification of the right bronchial tree, and it was suspected an EA type 3b. The literature recommends that: evaluation of the patency of the esophagus, with an iodinate contrast medium, should be done in a pediatric specialized center for high risk of lung aspiration.

  9. Long gap esophageal atresia: lengthening technique and primary anastomosis.

    Science.gov (United States)

    Hadidi, Ahmed T; Hosie, Stuart; Waag, Karl-Ludwig

    2007-10-01

    The treatment of long gap esophageal atresia remains a major surgical challenge. The authors describe a modification of a lengthening technique based on tissue expansion to avoid sutures cutting through the esophagus. Between January 2004 and August 2006, 4 patients did not respond to stretching, and underwent this modified esophageal lengthening technique using silastic tubes. RESULTS AND FOLLOW-UP: All infants recovered and have an intact esophagus. All infants developed gastroesophageal reflux. Thal antireflux procedure was performed in the first infant. The other 3 patients were managed conservatively. Follow-up ranged between 6 and 34 months. The tissue expansion principle can be successfully applied in the esophagus through external traction. Silastic tube fixation at esophageal ends may help to apply even traction and avoid sutures cutting through the esophageal tissue.

  10. Dysmotility in Esophageal Atresia: Pathophysiology, Characterization, and Treatment

    Science.gov (United States)

    Faure, Christophe; Righini Grunder, Franziska

    2017-01-01

    Esophageal dysmotility is almost universal after esophageal atresia (EA) repair and is mainly related to the developmental anomaly of the esophagus. Esophageal dysmotility is involved in the pathophysiology of numerous symptoms and comorbidities associated with EA such as gastroesophageal reflux disease, aspiration and respiratory complications, and symptoms of dysphagia and feeding disorders. High-resolution esophageal manometry (HREM) has facilitated the characterization of the dysmotility, but there is an incomplete correlation between symptoms and manometrical patterns. Impedance coupled to HREM should help to predict the clinical outcome and therefore personalize patient management. Nowadays, the management of esophageal dysmotility in patients with EA is essentially based on treatment of associated inflammation related to peptic or eosinophilic esophagitis. PMID:28620599

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

    Directory of Open Access Journals (Sweden)

    A O Akuma

    2013-01-01

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

  12. The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

    OpenAIRE

    Rommel, Nathalie; Rayyan, Maissa; Scheerens, Charlotte; Omari, Taher

    2017-01-01

    Infants and children with esophageal atresia commonly present with swallowing dysfunction or dysphagia. Dysphagia can lead to a range of significant consequences such as aspiration pneumonia, malnutrition, dehydration, and food impaction. To improve oral intake, the clinical diagnosis of dysphagia in patients with esophageal atresia should focus on both the pharynx and the esophagus. To characterize the complex interactions of bolus flow and motor function between mouth, pharynx, and esophagu...

  13. Dysphagia among Adult Patients who Underwent Surgery for Esophageal Atresia at Birth

    Directory of Open Access Journals (Sweden)

    Valérie Huynh-Trudeau

    2015-01-01

    Full Text Available BACKGROUND: Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and/or anatomical anomalies.

  14. Coexistence of bronchial atresia and bronchogenic cyst: diagnostic criteria and embryologic considerations

    International Nuclear Information System (INIS)

    Kuhn, C.; Kuhn, J.P.

    1992-01-01

    We report a case in a neonate of concurrent bronchial atresia and bronchogenic cyst. An accurate, noninvasive, preoperative diagnosis of this unusual combination of anomalies was made by ultrafast computed tomography (UFCT). This case supports the hypothesis that bronchial atresia results from an event occurring in the 5th-6th week of embryological development, rather than after the 16th week as previously believed. (orig.)

  15. Atresia of large ovarian follicles of the rat

    Directory of Open Access Journals (Sweden)

    Maria Słomczyn´ ska

    2011-08-01

    Full Text Available In the rat, at the beginning of pregnancy a cohort of antral follicles develops until the preovulatory stage. However, these follicles, differentiating in the hyperprolactinemic milieu, produce only small amount of estradiol, do not ovulate and undergo rapid degeneration. They constitute an interesting physiological model of atresia. In the present study, we analysed the development and subsequent degeneration of such follicles. The study was performed on Wistar female rats killed in succession between days 1-9 of pregnancy. Excised ovaries were submitted to a routine histological procedure. Paraffin sections were subjected to hematoxylin and eosin staining or in situ DNA labelling. Histological and TUNEL staining revealed that the investigated group of follicles grew slower than that on the corresponding days of the estrous cycle and reached a preovulatory size and morphological appearance on day 5 of pregnancy. They did not ovulate and between days 6 and 9 of pregnancy an increasing number of apoptotic cells appeared within these follicles. They were localized predominantly in the antral granulosa layer, especially near the cumulus oophorus complex (COC and in the region linking the COC with the follicular wall. The COC and the theca layer were much less affected. In late stages of atresia, also cumulus cells became apoptotic but degenerating oocytes did not exhibit positive TUNEL staining. Only limited number of the theca cells have undergone apoptosis and generally they were not hypertrophied. Our findings indicate that much smaller than normal amount of intrafollicular estradiol was sufficient to support a normal, according to the morphological criteria, although slower development of antral follicles to the late preovulatory stage.

  16. Histopathologic observations of anorectal abnormalities in anal atresia.

    Science.gov (United States)

    Meier-Ruge, W A; Holschneider, A M

    2000-01-01

    Over the years from 1992 to 1997, 41 anorectal malformations (ARM) with histopathologic alterations were investigated to determine which morphologic abnormalities of the distal rectum accompany ARMs. Three other cases showed normal neuromuscular morphology; 9 further cases could not be evaluated owing to scanty biopsies. All resected specimens were caudocranially coiled and cryostat cut at -20 degrees C into serial sections, which were stained with a lactic dehydrogenase, succinic dehydrogenase, nitroxide synthase, and acetylcholinesterase reaction as well as hemalum and sirius red. Ten low, 15 intermediate, and 10 high forms of anal atresia (AA) were studied. In addition, six cloacal abnormalities were investigated. In 7 cases (17%) (5 intermediate, 2 low AAs), the characteristics of Hirschsprung's disease were observed. Oligoneuronal hypoganglionosis of the myenteric plexus proximal to the anal floor was diagnosed in 7 AAs (12%). In 10 children with high-type AA and resection of 1-5 cm distal rectum and in all cloacal anomalies (n = 6) defects of the muscularis propria were seen in the rectal-atresia sac. These defects were characterized by hypoplasia of the circular-muscle layer and/or the internal anal sphincter (IAS). Intestinal neuronal dysplasia of the submucous plexus was most frequently observed (12%) in high-type AA. A correlation between innervation anomalies or anomalies of the muscularis propria and the type of fistula could not be seen. In conclusion, all cases with high-type AA and cloacal anomalies were characterized by anomalies of the muscularis propria and/or IAS but this was not the case in intermediate and low-type AAs. Anomalies of the enteric nervous system were diagnosed in 60% of AAs.

  17. Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators

    Directory of Open Access Journals (Sweden)

    Gürkan Altun

    2013-01-01

    Full Text Available Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein′s anomaly, Uhl′s anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.

  18. miR-26b promotes granulosa cell apoptosis by targeting ATM during follicular atresia in porcine ovary.

    Directory of Open Access Journals (Sweden)

    Fei Lin

    Full Text Available More than 99% of ovarian follicles undergo atresia in mammals, but the mechanism of follicular atresia remains to be elucidated. In this study, we explored microRNA (miRNA regulation of follicular atresia in porcine ovary. A miRNA expression profile was constructed for healthy, early atretic, and progressively atretic follicles, and the differentially expressed miRNAs were selected and analyzed. We found that miR-26b, which was upregulated during follicular atresia, increased the number of DNA breaks and promoted granulosa cell apoptosis by targeting the ataxia telangiectasia mutated gene directly in vitro.

  19. Supra-transumbilical laparotomy (STL approach for small bowel atresia repair: Our experience and review of the literature

    Directory of Open Access Journals (Sweden)

    Ernesto Leva

    2013-01-01

    Full Text Available Background: Supra-Transumbilical Laparotomy (STL has been used in paediatric surgery for a broad spectrum of abdominal procedures. We report our experience with STL approach for small bowel atresia repair in newborns and review previous published series on the topic. Patients and Methods: Fourteen patients with small bowel atresia were treated via STL approach at our Institution over a 5-year period and their charts were retrospectively reviewed. Results: STL procedure was performed at mean age of 3.1 day. No malrotation disorders were detected with pre-operative contrast enema. Eight patients (54.1% presented jejunal atresia, five (35.7% ileal atresia, and one (7.1% multiple ileal and jejunal atresias. Standard repair with primary end-to-back anastomosis was performed in all but one patient. In the newborn with multiple atresia, STL incision was converted in supra-umbilical transverse incision due to difficulty of exposition. After surgery, one patient developed anastomotic stricture, and another developed occlusion due to adhesions: Both infants required second laparotomy. No infections of the umbilical site were recorded, and cosmetic results were excellent in all patients. Conclusions: Increasing evidence suggests that STL approach for small bowel atresia is feasible, safe and provides adequate exposure for small bowel atresia surgery. When malrotation and colonic/multiple atresia are pre-operatively ruled out, STL procedure can be choosen as first approach.

  20. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras Extrahepatic biliary atresia: current concepts and future directions

    Directory of Open Access Journals (Sweden)

    Elisa de Carvalho

    2007-04-01

    Full Text Available OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal indicação de transplante hepático na faixa etária pediátrica. Quanto à etiologia, o citomegalovírus, o reovírus e o rotavírus têm sido os agentes mais estudados como possíveis desencadeadores da obstrução imunomediada da árvore biliar. A resposta imune, especialmente o predomínio da resposta TH1 e do interferon gama, a susceptibilidade genética e as alterações do desenvolvimento embrionário da árvore biliar são aspectos que podem participar na etiopatogênese da obliteração das vias biliares extra-hepáticas. Ainda hoje, o único tratamento disponível é a portoenterostomia, cujos resultados são melhores quando realizada nos primeiros 2 meses de vida. Em relação ao prognóstico, as crianças não tratadas vão a óbito na totalidade, por complicações relacionadas à hipertensão portal e à cirrose hepática, e mesmo os casos tratados necessitam, em sua maioria, do transplante hepático. CONCLUSÃO: A atresia das vias biliares extra-hepáticas continua sendo a principal indicação de transplante hepático em crianças, e a mudança deste panorama depende de um melhor entendimento da etiopatogenia da obstrução biliar nos diferentes fenótipos da doença. Investigações futuras a respeito do papel do interferon gama e de outras citocinas são necessárias para avaliar se esses aspectos seriam potenciais alvos de intervenção terapêutica.OBJECTIVE: To provide an updated review on extrahepatic biliary atresia, focusing

  1. Ultrasonographic differentiation of biliary atresia and neonatal hepatitis: Reestablishment of size criteria of the gallbladder

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Woo Sun; Cheon, Jung Eun; Koh, Young Hwan; Kim, In One; Yeon, Kyung Mo [Seoul National University College of Medicine and Institude of Radiation Medicion, Seoul (Korea, Republic of)

    2001-12-15

    To reestablish the size criterion of the gallbladder on ultrasonography (US) for the differentiation diagnosis of biliary atresia from neonatal hepatitis. Abdominal US ws performed in 201 patients with jaundice and 40 patients without evidence of jaundice or hepatobiliary illness (all with the age less than 4 months). US was performed in fasting (fasting for at least 4 hours) to measure the length of the gallbladder and calculated the area of the gallbladder lumen. The morphology of the gallbladder was classified into three types: normal, elongated and atretic. To evaluate the contractibility of the gallbladder, the length of the gallbladder and area of the gallbladder lumen was again measured 1 hour after feeding. The final diagnosis included biliary atresia in 79 patients and neonatal hepatitis in 83 patients. Differences in the length, area, and morphology of the gallbladder were statistically significant among three groups, the normal group, neonatal hepatitis group and biliary atresia group (length and area of gallbladder; normal group>neonatal hepatitis>biliary atresia). The differences in the length and area of gallbladder between pre- and postmeal state were statistically significant in the normal and neonatal hepatitis groups whereas those of biliary atresia were not significant (p=0.85). When the empirical size criterion of the gallbladder (<15 mm in length) was applied, the sensitivity, specificity and diagnostic accuracy for the differential diagnosis of biliary atresia from hepatitis were 52%, 82%, and 67%, respectively. Meanwhile, if the area criterion(<30 mm{sup 2} in area) was applied, the sensitivity, the specificity and diagnostic accuracy were 67%, 85%, and 75%, respectively. Ultrasonographic evaluation of the morphology as well as size of the gallbladder are helpful in the differential diagnosis of biliary atresia from neonatal hepatitis. Therefore, since the measurement of the area of gallbladder lumen on US reflect both size and morphology of

  2. Impact of unilateral conductive hearing loss due to aural atresia on academic performance in children.

    Science.gov (United States)

    Kesser, Bradley W; Krook, Kaelyn; Gray, Lincoln C

    2013-09-01

    This study evaluates the effect of unilateral conductive hearing loss secondary to aural atresia on elementary school children's academic performance. Case control survey and review of audiometric data. One hundred thirty-two surveys were mailed to families of children with aural atresia, and 48 surveys were sent to families of children with unilateral sensorineural hearing loss (SNHL) to identify rates of grade retention, use of any resource, and behavioral problems. Audiometric data of the cohort were tabulated. Of the 40 atresia patients, none repeated a grade, but 65% needed some resources: 12.5% currently use a hearing aid, 32.5% use(d) a frequency-modulated system in school, 47.5% had an Individualized Education Plan, and 45% utilized speech therapy. Compared to the unilateral SNHL group and a cohort of children with unilateral SNHL in an earlier study, children with unilateral atresia were less likely to repeat a grade. Children in both unilateral atresia and SNHL groups were more likely to utilize some resource in the academic setting compared to the unilateral SNHL children in the prior study. Unilateral conductive hearing loss due to aural atresia has an impact on academic performance in children, although not as profound when compared to children with unilateral SNHL. The majority of these children with unilateral atresia utilize resources in the school setting. Parents, educators, and health care professionals should be aware of the impact of unilateral conductive hearing loss and offer appropriate habilitative services. Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  3. Diagnostic Accuracy 99mTc-DISIDA Scintigraphy in Biliary Atresia

    International Nuclear Information System (INIS)

    Hyun, In Young; Lee, Dong Soo; Lee, Kyung Han; Kim, Jong Ho; Chung, June Key; Suh, Jung Key; Lee, Myung Chul; Koh, Chang Soon

    1994-01-01

    We evaluated the diagnostic accuracy of 99m Tc-DISIDA scintigraphy as a mean of differentiating biliary atresia from neonatal hepatitis. 99m Tc-DISIDA scintigraphy was visually interpreted by assessing the presence or absence of radioactivity in the intestine or gall bladder. In patients without intestinal radioactivity, we measured the hepatic retention index and the hepatic uptake index. The hepatic retention index was expressed as the amount of change of liver activity from 5 minutes to 30 minutes postinjection. The hepatic uptake index was graded visually with 5 minute images using the following scoring scheme :grade 0 (normal hepatic uptake), grade 1 (decreased hepatic up take), grade 2 (hepatic uptake equal to cardiac uptake), and grade 3 (hepatic uptake less than cardiac uptake). Age, total bilirubin, and hepatic uptake index were compared between the biliary atresia and the neonatal hepatitis group, between neonatal hepatitis patients with and without intestinal radioactivity, and between the biliary atresia and neonatal hepatitis patients with absent intestinal radioactivity. The results were as follows : l) None of the 30 biliary atresia patients showed intestinal radioactivity, while 31/40 neonatal hepatitis patients showed intestinal radioactivity. The sensitivity, specificity, and accuracy of the presence of intestinal radioactivity or the diagnosis of biliary atresia was 100%, 78%, and 87%, respectively. 2) In patients with absent intestinal radioactivity the mean hepatic retention index was 1.5 ± 0.6 in the 16 biliary atresia patient,s, and 1.1 ± 0.2 in the 7 neonatal hepatitis patients(p 99m Tc-DISIDA scintigraphy is accurate in the differential diagnosis of biliary atresia and neonatal hepatitis. In patients without intestinal radioactivity, the hepatic retention index and hepatic uptake index, along with the patients age and total bilirubin level may supplement diagnosis and improve diagnostic accuracy.

  4. Immediate primary anastomosis for isolated oesophageal atresia: A single-centre experience

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    Ibrahim Uygun

    2015-01-01

    Full Text Available Background: Isolated oesophageal atresia without tracheo-oesophageal fistula represents a major challenge for most paediatric surgeons. Here, we present our experience with six neonates with isolated oesophageal atresia who successfully underwent immediate primary anastomosis using multiple Livaditis circular myotomy. Materials and Methods: All six neonates were gross type A isolated oesophageal atresia (6%, from among 102 neonates with oesophageal atresia, treated between January 2009 and December 2013. Five neonates were female; one was male. The mean birth weight was 2300 (range 1700-3100 g. Results: All six neonates successfully underwent immediate primary anastomosis using multiple myotomies (mean 3; range 2-4 within 10 (median 3 days after birth. The gap under traction ranged from 6 to 7 cm. One neonate died of a major cardiac anomaly. Another neonate was lost to follow-up after being well for 3 months. Three anastomotic strictures were treated with balloon dilatation, and four anastomotic leaks were treated conservatively. The mean duration of follow-up was 33 months. Conclusions: To treat isolated oesophageal atresia, an immediate primary anastomosis can be achieved using multiple myotomies. Although, this approach is associated with high complication rates, as are other similar approaches, these complications can be overcome.

  5. Readability and quality assessment of websites related to microtia and aural atresia.

    Science.gov (United States)

    Alamoudi, Uthman; Hong, Paul

    2015-02-01

    Many parents and children utilize the Internet for health-related information, but the quality of these websites can vary. The objective of this study was to assess the quality and readability of microtia and aural atresia related websites. The search engine Google was queried with the terms 'microtia' and 'aural atresia.' The first 30 results were evaluated, and those websites containing original information written in English were reviewed. Quality of content was assessed with the DISCERN instrument, and readability was assessed with the Flesch-Kincaid Reading Grade Level (FKGL) and the Flesch Reading Ease Score (FRES) tests. Each website was also reviewed for ownership and the date of last update. Sixteen microtia and 14 aural atresia websites were included for full review. The mean DISCERN score for microtia websites was 54.4 (SD=8.3), and for aural atresia websites it was 47.6 (SD=10.7), which indicates 'good' and 'fair' quality of content, respectively. Readability assessments showed an average reading level requiring a grade 10 education on FKGL, and only one microtia (6.3%) and one aural atresia (7.1%) websites were deemed to be at 'reasonable' reading level on FRES. High-quality websites that are considered easily comprehensible to the general public were lacking. Since parents and children may use websites when making treatment decisions, physicians should be aware of the quality of health information pertaining to their area of expertise available on the Internet. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  6. Association of Nasal Nostril Stenosis with Bilateral Choanal Atresia: A Case Report

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    Shahin Abdollahifakhim

    2014-01-01

    Full Text Available Introduction: Neonatal nasal airway obstruction induces various degrees of respiratory distress. The management of this disease, including surgical repair, will depend on the severity and location of the obstruction. We describe here a case of congenital nasal nostril stenosis that required surgical repair for stenting of both nares after coanal atresia repair.   Case Report: A 2 days old female newborn referred to neonatal department of Tabriz Children’s Hospital affiliated to the University of Medical Sciences of Tabriz, Iran on the 3rd of December, 2011 immediately after birth with respiratory distress due to bilateral coanal atresia and nasal hypoplasia with very small nostrils. CT scan showed normal brain and bilateral choanal atresia with normal size Pyriform apertures.   Conclusion: Nasal obstruction can lead to airway compromise and respiratory distress. Congenital bony nasal deformities are being recognized as an important cause of newborn airway obstruction. Nasal hypoplasia is seen in many craniofacial syndromes. Although our patient had hypoplastic nostrils with respiratory distress due to bilateral coanal atresia, correction of hypoplastic nostrils was necessary for completing the operation of choanal atresia.

  7. Postoperative Course and Complications after Pull-through Vaginoplasty for Distal Vaginal Atresia.

    Science.gov (United States)

    Mansouri, Roshanak; Dietrich, Jennifer E

    2015-12-01

    To report the usual postoperative course and complications after pull-through vaginoplasty for isolated distal vaginal atresia. Retrospective chart review at Texas Children's Hospital of all patients who were diagnosed with isolated distal vaginal atresia and underwent pull-through vaginoplasty during the study time frame. None. Postoperative complications such as vaginal stenosis or infection and postoperative vaginal diameter. Sixteen patients were identified and charts were reviewed. Patients were initially evaluated by pelvic magnetic resonance imaging and found to have distended hematometrocolpos with distal vaginal atresia. All patients underwent pull-through vaginoplasty with similar operative techniques. The average distance from the perineum to the level of the obstruction was 1.84 ± 1.2 cm. Two patients, both with obstructions at greater than 3 cm, experienced stricture formation postoperatively. Four patients (25%) experienced postoperative vaginitis. One patient (6.25%) experienced a postoperative urinary tract infection. Two groups (3 cm or less versus greater than 3 cm) were compared, and the presence of stricture was statistically different based on mean centimeters from perineum prior to pull-through vaginoplasty (P = .038). Distal vaginal atresia is managed with pull-through vaginoplasty. Atresias that extend greater than 3 cm from the perineum are at increased risk for vaginal stricture formation and should be followed to monitor for their formation. Other complications are infrequent and minor. Copyright © 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  8. Ultrasound-guided percutaneous cholecysto-cholangiography for the exclusion of biliary atresia in infants

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Kyung Min; Ryeom, Hun Kyu; Choe, Byung Ho; Kim, Kap Cheol; Kim, Jong Yeol; Lee, Jong Min; Kim, Hye Jeong; Lee, Hee Jung [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    2006-08-15

    The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder.

  9. Ultrasound-guided percutaneous cholecysto-cholangiography for the exclusion of biliary atresia in infants

    International Nuclear Information System (INIS)

    Shin, Kyung Min; Ryeom, Hun Kyu; Choe, Byung Ho; Kim, Kap Cheol; Kim, Jong Yeol; Lee, Jong Min; Kim, Hye Jeong; Lee, Hee Jung

    2006-01-01

    The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder

  10. Current concept of the treatment of biliary atresia.

    Science.gov (United States)

    Miyano, T; Fujimoto, T; Ohya, T; Shimomura, H

    1993-01-01

    Hepatic portoenterostomy (Kasai operation) for the patient with biliary atresia (BA) can restore the bile flow in approximately 80% of children operated on before 60 days of life [1]. However, in terms of long-term survival, according to a recent nationwide survey among the major pediatric centers in Japan, only 325 of 2013 patients had more than 10 years' survival, and only 157 patients (7.8%) remained jaundice-free with normal liver function [2]. About 20% of BA cases without jaundice are generally able to survive for long periods; and most of those patients have portal hypertension or abnormal liver function [3-5]. As the results of liver transplantation have improved, controversy has arisen over the optimal care of these children [4, 6, 7]. Some investigators have claimed that transplantation is the favored primary therapy for most patients with BA [8]. We are thus at a turning point concerning the primary therapy of BA, which makes it necessary to determine the exact indications for the Kasai portoenterostomy and the timing of liver transplantation. This paper describes our strategy for the optimal treatment of BA patients based on our 117 patients who have had various form of portoenterostomy.

  11. Metabonomics reveals metabolite changes in biliary atresia infants.

    Science.gov (United States)

    Zhou, Kejun; Xie, Guoxiang; Wang, Jun; Zhao, Aihua; Liu, Jiajian; Su, Mingming; Ni, Yan; Zhou, Ying; Pan, Weihua; Che, Yanran; Zhang, Ting; Xiao, Yongtao; Wang, Yang; Wen, Jie; Jia, Wei; Cai, Wei

    2015-06-05

    Biliary atresia (BA) is a rare neonatal cholestatic disorder caused by obstruction of extra- and intra-hepatic bile ducts. If untreated, progressive liver cirrhosis will lead to death within 2 years. Early diagnosis and operation improve the outcome significantly. Infants with neonatal hepatitis syndrome (NHS) present similar symptoms, confounding the early diagnosis of BA. The lack of noninvasive diagnostic methods to differentiate BA from NHS greatly delays the surgery of BA infants, thus deteriorating the outcome. Here we performed a metabolomics study in plasma of BA, NHS, and healthy infants using gas chromatography-time-of-flight mass spectrometry. Scores plots of orthogonal partial least-squares discriminant analysis clearly separated BA from NHS and healthy infants. Eighteen metabolites were found to be differentially expressed between BA and NHS, among which seven (l-glutamic acid, l-ornithine, l-isoleucine, l-lysine, l-valine, l-tryptophan, and l-serine) were amino acids. The altered amino acids were quantitatively verified using ultraperformance liquid chromatography-tandem mass spectrometry. Ingenuity pathway analysis revealed the network of "Cellular Function and Maintenance, Hepatic System Development and Function, Neurological Disease" was altered most significantly. This study suggests that plasma metabolic profiling has great potential in differentiating BA from NHS, and amino acid metabolism is significantly different between the two diseases.

  12. Transjugular Intrahepatic Portosystemic Shunts in Children with Biliary Atresia

    International Nuclear Information System (INIS)

    Huppert, Peter E.; Goffette, Pierre; Astfalk, Wolfgang; Sokal, Emil M.; Brambs, Hans-Juergen; Schott, Ullrich; Duda, Stephan H.; Schweizer, Paul; Claussen, Claus D.

    2002-01-01

    Purpose: We retrospectively evaluated the technical and long-term clinical results of transjugularintrahepatic portosystemic shunts (TIPS) in children with portal hypertension and biliary atresia (BA). Methods: Nine children with BA and recurrent bleeding from esophagogastric and/or intestinal varices were treated by TIPS at the age of 34-156 months and followed-up in two centers. Different types of stents were used. Results: Shunt insertion succeeded in all patients, but in two a second procedure was necessary. Seven procedures lasted more than 3 hr, mainly due to difficult portal vein puncture.Variceal bleeding ceased in all patients; however, 16 reinterventions were performed in eight patients for clinical reasons (n =11) and sonographically suspected restenosis (n =5). Four patients underwent successful liver transplantation 4-51 months after TIPS and five are in good clinical conditions 64-75 months after TIPS. Conclusions: TIPS in children with BA is technically difficult, mainly due to periportal fibrosis and small portal veins. Frequency of reinterventions seems to be higher compared with adults

  13. The Modified Kimura's Technique for the Treatment of Duodenal Atresia

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    Biagio Zuccarello

    2009-01-01

    Full Text Available Background/Purpose. Kimura's diamond-shaped-duodenoduodenostomy (DSD is a known technique for the correction of congenital intrinsic duodenal obstruction. We present a modification of the technique and review the advantages of this new technique. Methods. From 1992 to 2006, 14 newborns were treated for duodenal atresia. We inverted the direction of the duodenal incisions: a longitudinal incision was made in the proximal duodenum while the distal was opened by transverse incision. Results. Our “inverted-diamond-shaped-duodenoduodenostomy” (i-DSD allowed postoperative oral feeding to start on days 2 to 3, peripheral intravenous fluids discontinuity on days 3 to 8 (median values 3.6; time to achieve full oral feeds on days 8 to 12 (median values 9.4; the length of hospitalisation ranged from 10 and 14 days (median value 11.2. No complications related to the anastomosis, by Viz leakage, dehiscence, biliary stasis, or stenosis were observed. Conclusions. The i-DSD provides a safe procedure to protect the ampulla of Vater from injury and avoids any formation of a blind loop. The results show that patients who have i-DSD achieve full oral feeds in a very short time period and, consequently, the length of hospitalisation is also significantly reduced.

  14. Successful Outcome and Biliary Drainage in an Infant with Concurrent Alpha-1-Antitrypsin Deficiency and Biliary Atresia

    Directory of Open Access Journals (Sweden)

    Andrew W. Wang

    2017-01-01

    Full Text Available We describe the rare instance of concomitant biliary atresia and alpha-1-antitrypsin deficiency and the first documented successful portoenterostomy in this scenario. The potential for dual pathology must be recognized and underscores that prompt diagnosis of biliary atresia, despite concomitant alpha-1-antitrypsin deficiency, is essential to afford potential longstanding native liver function.

  15. Reoperation of Anastomotic Stricture after Oesophageal Atresia Repair: An Uncommon Event

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    A L Azakpa

    2017-01-01

    Full Text Available Oesophageal atresia is a common malformation in which the survival rate in developed countries is around 90%, while its mortality remains very high in developing countries. Oesophageal stricture post-oesophageal atresia repair is traditionally treated by non-surgical approach. However, surgical resection of the oesophageal stricture may be necessary after the failure of dilations. We report one case of refractory oesophageal stricture post-EA repair in a 3-year-old girl, who underwent oesophageal atresia Type III repair at 11-day-old. We performed an end-to-end oesophageal anastomosis with tracheal oesophageal fistula closure by extra-pleural approach. The patient was lost to follow-up for 3 years. She was seen later for anastomotic oesophageal stricture with the failure of oesophageal dilatations. Surgical resection of oesophageal stricture was performed with end-to-end oesophageal anastomosis.

  16. Atresia biliar extra-hepática: métodos diagnósticos Extrahepatic biliary atresia: diagnostic methods

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    Sydney M. Cauduro

    2003-04-01

    Full Text Available OBJETIVO: enfatizar a importância do diagnóstico precoce da atresia biliar extra-hepática e sua relação direta com o restabelecimento cirúrgico do fluxo biliar antes do segundo mês de vida, discutindo os diversos métodos complementares utilizados, objetivando selecionar os de maior evidência, evitando retardo diagnóstico e, conseqüentemente, piora do prognóstico. MÉTODO: pesquisa bibliográfica referente ao período de 1985 a 2001, no Medline e MdConsult, através das palavras-chaves: colestase neonatal, atresia biliar extra-hepática e hepatite neonatal. Também foram selecionados artigos nacionais e estrangeiros a partir da bibliografia das publicações consultadas e, quando necessário, para melhor entendimento do tema, opiniões emitidas em teses e livros-textos foram referidas. RESULTADOS: a revisão da bibliografia consultada permite afirmar que o diagnóstico precoce das AVBEH e tratamento cirúrgico para restabelecer o fluxo biliar até 60 dias de vida são fundamentais na obtenção de bons resultados. Entre os diversos métodos complementares de diagnóstico, a colangiografia por RM, US e a biópsia hepática são os que proporcionam os maiores índices de acerto. CONCLUSÕES: o encaminhamento de pacientes portadores de AVBEH para centros de referências no Brasil ainda é efetuado tardiamente, provavelmente pela falta de esclarecimento dos médicos de atenção primária, aliada às dificuldades burocráticas e tecnológicas. A experiência da Inglaterra em relação ao programa "Alerta Amarelo", permitiu que o número de crianças encaminhadas para tratamento cirúrgico antes dos 60 dias de vida aumentasse significativamente. Entre os métodos complementares, a colangiografia por RM, a US e a biópsia hepática deverão ser utilizados, dependendo dos recursos tecnológicos das unidades de diagnóstico.OBJECTIVE: to emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct

  17. Congenital external auditory canal atresia and stenosis: temporal bone CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Hoon; Kim, Bum Soo; Jung, So Lyung; Kim, Young Joo; Chun, Ho Jong; Choi, Kyu Ho; Park, Shi Nae [College of Medicine, Catholic Univ. of Korea, Seoul (Korea, Republic of)

    2002-04-01

    To determine the computed tomographic (CT) findings of atresia and stenosis of the external auditory canal (EAC), and to describe associated abnormalities in surrounding structures. We retrospectively reviewed the axial and coronal CT images of the temporal bone in 15 patients (M:F=8:7;mean age, 15.8 years) with 16 cases of EAC atresia (unilateral n=11, bilateral n=1) and EAC stenosis (unilateral n=3). Associated abnormalities of the EAC, tympanic cavity, ossicles, mastoid air cells, eustachian tube, facial nerve course, mandibular condyle and condylar fossa, sigmoid sinus and jugular bulb, and the base of the middle cranial fossa were evaluated. Thirteen cases of bony EAC atresia (one bilateral), with an atretic bony plate, were noted, and one case of unilateral membranous atresia, in which a soft tissue the EAC. A unilateral lesion occurred more frequently on the right temporal bone (n=8, 73%). Associated abnormalities included a small tympanic cavity (n=8, 62%), decreased mastoid pneumatization (n=8, 62%), displacement of the mandibular condyle and the posterior wall of the condylar fossa (n=7, 54%), dilatation of the Eustachian tube (n=7, 54%), and inferior displacement of the temporal fossa base (n=8, 62%). Abnormalities of ossicles were noted in the malleolus (n=12, 92%), incus (n=10, 77%) and stapes (n=6, 46%). The course of the facial nerve was abnormal in four cases, and abnormality of the auditory canal was noted in one. Among three cases of EAC stenosis, ossicular aplasia was observed in one, and in another the location of the mandibular condyle and condylar fossa was abnormal. In the remaining case there was no associated abnormality. Atresia of the EAC is frequently accompanied by abnormalities of the middle ear cavity, ossicles, and adjacent structures other than the inner ear. For patients with atresia and stenosis of this canal, CT of the temporal bone is essentially helpful in evaluating these associated abnormalities.

  18. microRNA-222 modulates liver fibrosis in a murine model of biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Shen, Wen-jun; Dong, Rui; Chen, Gong, E-mail: chengongzlp@hotmail.com; Zheng, Shan

    2014-03-28

    Highlights: • The RRV infected group showed cholestasis, retardation and extrahepatic biliary atresia. • miR-222 was highly expressed, and PPP2R2A was inhibited in the murine biliary atresia model. • miR-222 profoundly modulated the process of fibrosis in the murine biliary atresia model. • miR-222 might represent a potential target for improving biliary atresia prognosis. - Abstract: microRNA-222 (miR-222) has been shown to initiate the activation of hepatic stellate cells, which plays an important role in the pathogenesis of liver fibrosis. The aim of our study was to evaluate the role of miR-22 in a mouse model of biliary atresia (BA) induced by Rhesus Rotavirus (RRV) infection. New-born Balb/c mice were randomized into control and RRV infected groups. The extrahepatic bile ducts were evaluated. The experimental group was divided into BA group and negative group based on histology. The expression of miR-222, protein phosphatase 2 regulatory subunit B alpha (PPP2R2A), proliferating cell nuclear antigen (PCNA) and phospho-Akt were detected. We found that the experimental group showed signs of cholestasis, retardation and extrahepatic biliary atresia. No abnormalities were found in the control group. In the BA group, miR-222, PCNA and Akt were highly expressed, and PPP2R2A expression was significantly inhibited. Our findings suggest that miR-222 profoundly modulated the process of fibrosis in the murine BA model, which might represent a potential target for improving BA prognosis.

  19. microRNA-222 modulates liver fibrosis in a murine model of biliary atresia

    International Nuclear Information System (INIS)

    Shen, Wen-jun; Dong, Rui; Chen, Gong; Zheng, Shan

    2014-01-01

    Highlights: • The RRV infected group showed cholestasis, retardation and extrahepatic biliary atresia. • miR-222 was highly expressed, and PPP2R2A was inhibited in the murine biliary atresia model. • miR-222 profoundly modulated the process of fibrosis in the murine biliary atresia model. • miR-222 might represent a potential target for improving biliary atresia prognosis. - Abstract: microRNA-222 (miR-222) has been shown to initiate the activation of hepatic stellate cells, which plays an important role in the pathogenesis of liver fibrosis. The aim of our study was to evaluate the role of miR-22 in a mouse model of biliary atresia (BA) induced by Rhesus Rotavirus (RRV) infection. New-born Balb/c mice were randomized into control and RRV infected groups. The extrahepatic bile ducts were evaluated. The experimental group was divided into BA group and negative group based on histology. The expression of miR-222, protein phosphatase 2 regulatory subunit B alpha (PPP2R2A), proliferating cell nuclear antigen (PCNA) and phospho-Akt were detected. We found that the experimental group showed signs of cholestasis, retardation and extrahepatic biliary atresia. No abnormalities were found in the control group. In the BA group, miR-222, PCNA and Akt were highly expressed, and PPP2R2A expression was significantly inhibited. Our findings suggest that miR-222 profoundly modulated the process of fibrosis in the murine BA model, which might represent a potential target for improving BA prognosis

  20. First branchial cleft sinus presenting with cholesteatoma and external auditory canal atresia.

    Science.gov (United States)

    Yalçin, Sinasi; Karlidağ, Turgut; Kaygusuz, Irfan; Demirbağ, Erhan

    2003-07-01

    First branchial cleft abnormalities are rare. They may involve the external auditory canal and middle ear. We describe a 6-year-old girl with congenital external auditory canal atresia, microtia, and cholesteatoma of mastoid and middle ear in addition to the first branchial cleft abnormalities. Clinical features of the patient are briefly described and the embryological relationship between first branchial cleft anomaly and external auditory canal atresia is discussed. The surgical management of these lesions may be performed, both the complete excision of the sinus and reconstructive otologic surgery.

  1. Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia

    DEFF Research Database (Denmark)

    Madsen, Stine Skipper; Kvist, Nina; Thorup, Jørgen

    2015-01-01

    . This percentage value has caused diagnostic trouble over the years. The objective of the present study was to investigate the possibility of changing the recommendations. METHODS: This was a retrospective analysis of the medical records of children operated for biliary atresia in the 1993-2012 period. RESULTS......: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion. FUNDING: none. TRIAL REGISTRATION...

  2. Missed diagnosis of atresia of the right pulmonary artery in woman with left-sided pneumothorax

    DEFF Research Database (Denmark)

    Dagnegård, Hanna; Ryom, Philip

    2016-01-01

    Isolated pulmonary atresia is an uncommon condition, which can go undiagnosed for a long time in asymptomatic patients. Sometimes, diagnosis can be made at pregnancy due to respiratory symptoms. There is no known increased risk of pneumothorax. We here present a case where a second-time pregnant...... woman with an unknown atresia of the right pulmonary artery received a left-sided pneumothorax. The diagnosis was initially missed in spite of adequate imaging and the condition progressed to respiratory stop. We describe the course of diagnostics and the chosen strategy of treatment....

  3. Scintigraphic hepatobiliary function studies in newborn infants to diagnose biliary hypoplasia or atresia

    International Nuclear Information System (INIS)

    Askari-Sabi, Z.

    1987-01-01

    The results obtained from scintigraphic hepatobiliary function studies, intraoperative cholangiography and histological examinations in a total of 17 infants suspected of having biliary atresia were compared and analysed with reference to the clinical signs and symptoms observed. In most cases, the individual diagnostic procedures led to consistent findings, even though there were some variations in the clinical picture. Patient outcome is largely determined by the site of atresia, due to which fact surgical correction should be carried out as soon as possible, in any case before the 8th week post partum. (TRV) [de

  4. Reverse gastric tube oesophageal substitution for staged repair of oesophageal atresia and tracheo-oesophageal fistula

    Directory of Open Access Journals (Sweden)

    Christopher Olusanjo Bode

    2014-01-01

    Full Text Available The management of oesophageal atresia and tracheo-oesophageal atresia (OATOF is very challenging. While in developed countries survival of patients with this condition has improved, the outcome in many developing countries has been poor. Primary repair through a thoracotomy (or video-assisted thoracoscopic surgery where available is the gold standard treatment of OATOF. However, in our setting where patients typically present late and with minimum support resources such as Neonatal Intensive Care Unit and total parenteral nutrition; staged repair may be the only hope of survival of these patients and this communication highlights the essential steps of this mode of treatment.

  5. Anomalous pancreatic ductal system allowing distal bowel gas with duodenal atresia

    Directory of Open Access Journals (Sweden)

    Shruti Sevak

    2017-11-01

    Bypass of the atretic duodenal segment through an anomalous pancreatic ductal system is a rare anomaly described in the literature in only a handful of cases. This case report highlights the importance of considering duodenal atresia and pancreaticobiliary enteric bypass in the differential diagnosis of neonates presenting with partial duodenal obstruction. On ultrasound, the presence of gas in the biliary tree or pancreatic duct should alert the physician to the possibility of duodenal atresia with congenital pancreaticobiliary duct anomalies that allow for bypass of enteric contents, including air, into more distal bowel, thereby creating a gas pattern aptly described as double bubble with distal gas.

  6. Congenital bronchial atresia coexistent with intralobar pulmonary sequestration: a case report

    International Nuclear Information System (INIS)

    Han, Young Min; Ku, Ja Hong; Lee, Dong Keun; Chung, Kyung Ho; Kim, Chong Soo; Sohn, Myung Hee; Choi, Ki Chul

    1995-01-01

    Bronchial atresia coexistent with intralobar pulmonary sequestration is so rare that only two cases have been reported in the literature. We report a case of congential bronchial atresia coexistent with intralobar pulmonary sequestation in a 51-year-old woman. Computed tomography showed the branching mass with hyperinflation of adjacent pulmonary parenchyma in the medial segment of the right middle lobe and a large thin-walled cystic mass with air-fluid levels in the medial basal segment of the right lower lobe. Selective inferior phrenic arteriography showed two aberrant arteries supplying the large cystic mass in the right lower lobe. The venous drainage was through the right pulmonary vein

  7. Congenital bronchial atresia coexistent with intralobar pulmonary sequestration: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Han, Young Min; Ku, Ja Hong; Lee, Dong Keun; Chung, Kyung Ho; Kim, Chong Soo; Sohn, Myung Hee; Choi, Ki Chul [Chonbuk National University Medical School, Jeonju (Korea, Republic of)

    1995-02-15

    Bronchial atresia coexistent with intralobar pulmonary sequestration is so rare that only two cases have been reported in the literature. We report a case of congential bronchial atresia coexistent with intralobar pulmonary sequestation in a 51-year-old woman. Computed tomography showed the branching mass with hyperinflation of adjacent pulmonary parenchyma in the medial segment of the right middle lobe and a large thin-walled cystic mass with air-fluid levels in the medial basal segment of the right lower lobe. Selective inferior phrenic arteriography showed two aberrant arteries supplying the large cystic mass in the right lower lobe. The venous drainage was through the right pulmonary vein.

  8. Factors Influencing Time-to-diagnosis of Biliary Atresia.

    Science.gov (United States)

    Harpavat, Sanjiv; Lupo, Philip J; Liwanag, Loriel; Hollier, John; Brandt, Mary L; Finegold, Milton J; Shneider, Benjamin L

    2018-06-01

    Diagnosing biliary atresia (BA) quickly is critical, because earlier treatment correlates with delayed or reduced need for liver transplantation. However, diagnosing BA quickly is also difficult, with infants usually treated after 60 days of life. In this study, we aim to accelerate BA diagnosis and treatment, by better understanding factors influencing the diagnostic timeline. Infants born between 2007 and 2014 and diagnosed with BA at our institution were included (n = 65). Two periods were examined retrospectively: P1, the time from birth to specialist referral, and P2, the time from specialist referral to treatment. How sociodemographic factors associate with P1 and P2 were analyzed with Kaplan-Meier curves and Cox proportional hazard models. In addition, to better characterize P2, laboratory results and early tissue histology were studied. P1 associated with race/ethnicity, with shorter times in non-Hispanic white infants compared to non-Hispanic black and Hispanic infants (P = 0.007 and P = 0.004, respectively). P2 associated with referral age, with shorter times in infants referred after 30, 45, or 60 days of life (P P2 in infants referred ≤30 days is that aminotransferase levels were normal or near-normal. However, despite reassuring laboratory values, tissue histology in early cases showed key features of BA. Our findings suggest 2 opportunities to accelerate BA diagnosis and treatment. First, to achieve prompt referrals for all races/ethnicities, universal screening strategies should be considered. Second, to ensure efficient evaluations independent of age, algorithms designed to detect early features of BA can be developed.

  9. Eosinophilic esophagitis in patients with esophageal atresia and chronic dysphagia.

    Science.gov (United States)

    Kassabian, Sirvart; Baez-Socorro, Virginia; Sferra, Thomas; Garcia, Reinaldo

    2014-12-21

    Esophageal atresia (EA) is defined as a discontinuity of the lumen of the esophagus repaired soon after birth. Dysphagia is a common symptom in these patients, usually related to stricture, dysmotility or peptic esophagitis. We present 4 cases of patients with EA who complained of dysphagia and the diagnosis of Eosinophilic esophagitis (EoE) was made, ages ranging from 9 to 16 years. Although our patients were on acid suppression years after their EA repair, they presented with acute worsening of dysphagia. Esophogastroduodenoscopy and/or barium swallow did not show stricture and biopsies revealed elevated eosinophil counts consistent with EoE. Two of 4 patients improved symptomatically with the topical steroids. It is important to note that all our patients have asthma and 3 out of 4 have tested positive for food allergies. One of our patients developed recurrent anastomotic strictures that improved with the treatment of the EoE. A previous case report linked the recurrence of esophageal strictures in patients with EA repair with EoE. Once the EoE was treated the strictures resolved. On the other hand, based on our observation, EoE could be present in patients without recurrent anastomotic strictures. There appears to be a spectrum in the disease process. We are suggesting that EoE is a frequent concomitant problem in patients with history of congenital esophageal deformities, and for this reason any of these patients with refractory reflux symptoms or dysphagia (with or without anastomotic stricture) may benefit from an endoscopic evaluation with biopsies to rule out EoE.

  10. Endotoxin and CD14 in the progression of biliary atresia

    Directory of Open Access Journals (Sweden)

    Chen Ching-Mei

    2010-12-01

    Full Text Available Abstract Background Biliary atresia (BA is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/or extra-hepatic bile ducts. However, the mechanisms contributing to the pathogenesis of BA remain uncertain. Because of decreased bile flow, infectious complications and damaging endotoxemia occur frequently in patients with BA. The aim of this study was to investigate endotoxin levels in patients with BA and the relation of these levels with the expression of the endotoxin receptor, CD14. Methods The plasma levels of endotoxin and soluble CD14 were measured with a pyrochrome Limulus amebocyte lysate assay and enzyme-linked immunosorbent assay in patients with early-stage BA when they received the Kasai procedure (KP, in patients who were jaundice-free post-KP and followed-up at the outpatient department, in patients with late-stage BA when they received liver transplantation, and in patients with choledochal cysts. The correlation of CD14 expression with endotoxin levels in rats following common bile duct ligation was investigated. Results The results demonstrated a significantly higher hepatic CD14 mRNA and soluble CD14 plasma levels in patients with early-stage BA relative to those with late-stage BA. However, plasma endotoxin levels were significantly higher in both the early and late stages of BA relative to controls. In rat model, the results demonstrated that both endotoxin and CD14 levels were significantly increased in liver tissues of rats following bile duct ligation. Conclusions The significant increase in plasma endotoxin and soluble CD14 levels during BA implies a possible involvement of endotoxin stimulated CD14 production by hepatocytes in the early stage of BA for removal of endotoxin; whereas, endotoxin signaling likely induced liver injury and impaired soluble CD14 synthesis in the late stages of BA.

  11. Respiratory problems in children with esophageal atresia and tracheoesophageal fistula.

    Science.gov (United States)

    Porcaro, Federica; Valfré, Laura; Aufiero, Lelia Rotondi; Dall'Oglio, Luigi; De Angelis, Paola; Villani, Alberto; Bagolan, Pietro; Bottero, Sergio; Cutrera, Renato

    2017-09-05

    Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient's needs and harmonize the care. A retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children's Hospital (Rome, Italy) between 2010 and 2015. 69/105 (66%) children reported lower respiratory symptoms with a mean age onset of 2.2 ± 2.5 years and only 63/69 (91%) performed specialist assessment at Respiratory Unit. Recurrent pneumonia (33%) and wheezing (31%) were the most reported symptoms. The first respiratory evaluation was performed after surgically correction of gastroesophageal reflux (GER) at mean age of 3.9 ± 4.2 years. Twenty nine patients have undergone to chest CT with contrast enhancement detecting localized atelectasis (41%), residual tracheal diverticulum (34%), bronchiectasis (31%), tracheal vascular compression (21%), tracheomalacia (17%) and esophageal diverticulum (14%). Fifty three patients have undergone to airways endoscopy detecting tracheomalacia (66%), residual tracheal diverticulum (26%), recurrent tracheoesophageal fistula (19%) and vocal cord paralysis (11%). Our study confirms that respiratory symptoms often complicate EA and TEF; their persistence despite medical and surgical treatment of GER means that other etiological hypothesis must be examined and that a complete respiratory diagnostic work up must be considered.

  12. Aspiration Risk and Respiratory Complications in Patients with Esophageal Atresia.

    Science.gov (United States)

    Kovesi, Thomas

    2017-01-01

    Chronic, long-term respiratory morbidity (CRM) is common in patients with a history of repaired congenital esophageal atresia, typically associated with tracheoesophageal fistula (EA/TEF). EA/TEF patients are at high risk of having aspiration, and retrospective studies have associated CRM with both recurrent aspiration and atopy. However, studies evaluating the association between CRM in this population and either aspiration or atopy have reported conflicting results. Furthermore, CRM in this population may be due to other related conditions as well, such as tracheomalacia and/or recurrent infections. Aspiration is difficult to confirm, short of lung biopsy. Moreover, even within the largest evidence base assessing the association between CRM and aspiration, which has evaluated the potential relationship between gastroesophageal reflux and asthma, findings are contradictory. Studies attempting to relate CRM to prior aspiration events may inadequately estimate the frequency and severity of previous aspiration episodes. There is convincing evidence documenting that chronic, massive aspiration in patients with repaired EA/TEF is associated with the development of bronchiectasis. While chronic aspiration is likely associated with other CRM in patients with repaired EA/TEF, this does not appear to have been confirmed by the data currently available. Prospective studies that systematically evaluate aspiration risk and allergic disease in patients with repaired EA/TEF and document subsequent CRM will be needed to clarify the causes of CRM in this population. Given the prevalence of CRM, patients with repaired EA/TEF should ideally receive regular follow-up by multidisciplinary teams with expertise in this condition, throughout both childhood and adulthood.

  13. International survey on the management of esophageal atresia.

    Science.gov (United States)

    Zani, Augusto; Eaton, Simon; Hoellwarth, Michael E; Puri, Prem; Tovar, Juan; Fasching, Guenter; Bagolan, Pietro; Lukac, Marija; Wijnen, Rene; Kuebler, Joachim F; Cecchetto, Giovanni; Rintala, Risto; Pierro, Agostino

    2014-02-01

    Because many aspects of the management of esophageal atresia (EA) are still controversial, we evaluated the practice patterns of this condition across Europe. A survey was completed by 178 delegates (from 45 [27 European] countries; 88% senior respondents) at the EUPSA-BAPS 2012. Approximately 66% of respondents work in centers where more than five EA repairs are performed per year. Preoperatively, 81% of respondents request an echocardiogram, and only 43% of respondents routinely perform preoperative bronchoscopy. Approximately 94% of respondents prefer an open approach, which is extrapleural in 71% of respondents. There were no differences in use of thoracoscopy between Europeans (10%) and non-Europeans (11%, p = nonsignificant). Approximately 60% of respondents measure the gap intraoperatively. A transanastomotic tube (90%) and chest drain (69%) are left in situ. Elective paralysis is adopted by 56% of respondents mainly for anastomosis tension (65%). About 72% of respondents routinely request a contrast study on postoperative day 7 (2-14). Approximately 54% of respondents use parenteral nutrition, 40% of respondents start transanastomotic feeds on postoperative day 1, and 89% of respondents start oral feeds after postoperative day 5. Pure EA: 46% of respondents work in centers that repair two or more than two pure EA a year. About 60% of respondents opt for delayed primary anastomosis at 3 months (1-12 months) with gastrostomy formation without esophagostomy. Anastomosis is achieved with open approach by 85% of respondents. About 47% of respondents attempt elongation of esophageal ends via Foker technique (43%) or with serial dilations with bougies (41%). Approximately 67% of respondents always attempt an anastomosis. Gastric interposition is the commonest esophageal substitution. Many aspects of EA management are lacking consensus. Minimally invasive repair is still sporadic. We recommend establishment of an EA registry. Georg Thieme Verlag KG Stuttgart · New

  14. Cervicovaginal atresia with hematometra: restoring menstrual and sexual function by utero-coloneovaginoplasty.

    Science.gov (United States)

    Kisku, Sundeep; Varghese, Lilly; Kekre, Aruna; Sen, Sudipta; Karl, Sampath; Mathai, John; Thomas, Reju Joseph; Barla, Ravi Kishore

    2014-10-01

    Cervicovaginal atresia is a rare Mullerian anomaly. The management of cervicovaginal atresia has evolved from historical recommendations of hysterectomy to various reconstructive procedures more recently. The latter carries a risk of significant morbidity and unknown fertility. We present our experience in the management of this complex anomaly. Twenty patients with cervicovaginal atresia were operated in our hospital from January 2004 through December 2013. The details of their anatomical variations and functional outcomes were analyzed. Eighteen out of twenty patients had cervical agenesis. Two patients had cervical hypoplasia. All patients underwent utero-coloneovaginoplasty. Post operatively, all patients have regular menstrual cycles. One patient is married, sexually active and has satisfactory coital function. One patient had a bowel anastomotic leak that required a diversion ileostomy. Two patients developed mild stenosis. One patient has mild neovaginal mucosal prolapse. No patient has developed pyometra. Patients with cervicovaginal atresia need to be counselled about the various reconstructive options available and the potential risks. Social and economic factor play a significant role in determining the plan of management. For patients from conservative societies, utero-coloneovaginoplasty provides a safe conduit for the passage of menstrual flow and coitus, at the cost of permanent infertility.

  15. A newborn with duodenal atresia and a gastric perforation | Akçora ...

    African Journals Online (AJOL)

    ... postoperative day. This complicated disease can be treated by early diagnosis and surgical intervention. We choose one-stage operation because of the clean peritoneal cavity. However, generalized peritonitis may require two-stage operation in delayed cases. Key words: Duodenal atresia, gastric perforation, newborn ...

  16. Gastric serosal tear due to congenital pyloric atresia: A rare anomaly ...

    African Journals Online (AJOL)

    Congenital pyloric atresia (CPA) is a very rare malformation with unknown aetiology. It has has numerous complications including gastric perforation, aspiration pneumonia. Gastric perforations in newborns occur by three mechanisms: trauma, ischaemia, or spontaneous. Here, we report a newborn with CPA presenting with ...

  17. Use of TC 99-IDA in the differential diagnosis of bile ducts atresia in the newborn

    International Nuclear Information System (INIS)

    Sosky, R.A.; Arias Coehl, S.; Jara York, J.; Calabro, M.A.

    1984-01-01

    Two newborn with jaundice, acholia, coluria, elevated bilirubinemia and alchaline fosfatase were studied at the Nuclear Medicine Unit at the IICS with Tc 99-IDA. Differential diagnosis between bile ducts atresia and neonatal hepatitis can be done with this method. This technique is reliable, low radiation dose to patient and no adverse reaction occurs with this radiopharmaceutical

  18. Congenital bronchopulmonary foregut malformation initially diagnosed as esophageal atresia type C: challenging diagnosis and treatment

    NARCIS (Netherlands)

    Boersma, Doeke; Koot, Bart G.; van der Griendt, Erik Jonas; van Rijn, Rick R.; van der Steeg, Alida F.

    2012-01-01

    Communicating bronchopulmonary foregut malformations are extremely rare congenital malformations, characterized by a communicating fistula between an isolated part of the respiratory system and the esophagus or the stomach. In this article, we present a case of esophageal atresia type C, later

  19. MicroRNAs: New Insight in Modulating Follicular Atresia: A Review

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    Tesfaye Worku

    2017-02-01

    Full Text Available Our understanding of the post-transcriptional mechanisms involved in follicular atresia is limited; however, an important development has been made in understanding the biological regulatory networks responsible for mediating follicular atresia. MicroRNAs have come to be seen as a key regulatory actor in determining cell fate in a wide range of tissues in normal and pathological processes. Profiling studies of miRNAs during follicular atresia and development have identified several putative miRNAs enriched in apoptosis signaling pathways. Subsequent in vitro and/or in vivo studies of granulosa cells have elucidated the functional role of some miRNAs along with their molecular pathways. In particular, the regulatory roles of some miRNAs have been consistently observed during studies of follicular cellular apoptosis. Continued work should gradually lead to better understanding of the role of miRNAs in this field. Ultimately, we expect this understanding will have substantial benefits for fertility management at both the in vivo or/and in vitro levels. The stable nature of miRNA holds remarkable promise in clinical use as a diagnostic tool and in reproductive medicine to solve the ever-increasing fertility problem. In this review, we summarize current knowledge of the involvement of miRNAs in follicular atresia, discuss the challenges for further work and pinpoint areas for future research.

  20. Pyloric atresia: A report of ten patients | Ksia | African Journal of ...

    African Journals Online (AJOL)

    Pyloric atresia (PA) is uncommon. It occurs in 1:100000 live births. Neonates usually present soon after birth with copious non-bilious vomiting. The treatment is surgical and its prognosis is poor, especially, when it is associated with epidermolysis bullosa (EB). The aim of this study was to evaluate the clinical presentation, ...

  1. Diagnosis and treatment of gastroesophageal reflux in patients with esophageal atresia

    NARCIS (Netherlands)

    J.H.L.J. Bergmeijer (Jan Hein)

    2002-01-01

    textabstractIn the last two decades, surgical treatment of children born with esophageal atresia has become a standard procedure. Postoperative mortality- now negligible in those born at term without other severe anomalies- mainly relates to patients with associated severe congenital cardiac

  2. Hepatocellular carcinoma in the native liver of a 38-year-old female patient with biliary atresia

    Directory of Open Access Journals (Sweden)

    Yutaka Kanamori

    2015-11-01

    Full Text Available We report a rare case of hepatocellular carcinoma in native liver in a case of biliary atresia. The patient was a 38-year-old female with three children who had an aggressive tumor, resulting in her subsequent death. We also review 14 reports, published previously in the English language medical literature, concerning hepatocellular carcinoma originating from native liver in biliary atresia cases and discuss the possible etiology, and propose more careful follow up for the patients with biliary atresia who suffer from repetitive cholangitis and/or experience the child delivery.

  3. Physical development and cognitive performance in a monozygotic twins for biliary atresia: Report of a case and literature reviewing

    Directory of Open Access Journals (Sweden)

    Yang Chen

    2016-08-01

    Full Text Available A case of monozygotic twins, one of whom suffered from biliary atresia and the other one was apparently healthy, is described herein. Children with biliary atresia appear to be vulnerable to developmental lags in the physical and cognitive performance, due to malnutrition and deteriorated liver function. In our case where the child with biliary atresia, who underwent a successful kasai operation, has achieved comparable growth development and cognitive performance in comparision with her sister, which suggest that the early diagnosis and timly kasai operation could allow children with biliary artesia to grow well in the short-term following time.

  4. Hepatic ADC value correlates with cirrhotic severity of patients with biliary atresia

    International Nuclear Information System (INIS)

    Mo, Yuan Heng; Jaw, Fu Shan; Ho, Ming Chih; Wang, Yung Cheng; Peng, Steven Shinn Forng

    2011-01-01

    Introduction: At least 40% of survivors of biliary atresia have progressive cirrhosis even after undergoing Kasai operation. The values of hepatic apparent diffusion coefficient and apparent-diffusion-coefficient-related indices were applied to biliary atresia patients and correlated with cirrhotic severity scores of model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte, and Child-Pugh systems. Materials and methods: Thirty-three biliary atresia patents (mean = 1140, 61–4314 days of age) received magnetic resonance image examinations due to complications of biliary atresia from April 2008 to August 2009. Two non-breath-hold diffusion weighted imaging sequences were performed with motion-probing gradients in three directions with two b values: 0/100 and 0/500 s/mm 2 ; 1000 ms/61.1 ms, time to repeat/time to echo; number of excitation, 1.0; 8 mm section thickness; 40 cm × 40 cm field of view; 128 × 256 matrix in all biliary atresia patients and 18 control subjects. We used the Spearman rank correlation test to analyze the relationship among the scores of model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte and Child-Pugh scores and right hepatic apparent diffusion coefficients, apparent diffusion coefficient using b factor of 500-albumin product and alanine transaminase/apparent diffusion coefficient with b factor of 500 ratio. Results: The right hepatic apparent diffusion coefficient using b factor of 100, apparent diffusion coefficient with b factor of 500 and product of apparent diffusion coefficient with b factor of 500-albumin level were significantly negatively correlated (p ≤ 0.0125) with model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte, and Child-Pugh scores of biliary atresia patients. The ratio of alanine transaminase level/right hepatic apparent diffusion coefficient with b factor of 500 was also significantly (p ≤ 0

  5. The Role of ARF6 in Biliary Atresia.

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    Mylarappa Ningappa

    Full Text Available Altered extrahepatic bile ducts, gut, and cardiovascular anomalies constitute the variable phenotype of biliary atresia (BA.To identify potential susceptibility loci, Caucasian children, normal (controls and with BA (cases at two US centers were compared at >550000 SNP loci. Systems biology analysis was carried out on the data. In order to validate a key gene identified in the analysis, biliary morphogenesis was evaluated in 2-5-day post-fertilization zebrafish embryos after morpholino-antisense oligonucleotide knockdown of the candidate gene ADP ribosylation factor-6 (ARF6, Mo-arf6.Among 39 and 24 cases at centers 1 and 2, respectively, and 1907 controls, which clustered together on principal component analysis, the SNPs rs3126184 and rs10140366 in a 3' flanking enhancer region for ARF6 demonstrated higher minor allele frequencies (MAF in each cohort, and 63 combined cases, compared with controls (0.286 vs. 0.131, P = 5.94x10-7, OR 2.66; 0.286 vs. 0.13, P = 5.57x10-7, OR 2.66. Significance was enhanced in 77 total cases, which included 14 additional BA genotyped at rs3126184 only (p = 1.58x10-2, OR = 2.66. Pathway analysis of the 1000 top-ranked SNPs in CHP cases revealed enrichment of genes for EGF regulators (p<1 x10-7, ERK/MAPK and CREB canonical pathways (p<1 x10-34, and functional networks for cellular development and proliferation (p<1 x10-45, further supporting the role of EGFR-ARF6 signaling in BA. In zebrafish embryos, Mo-arf6 injection resulted in a sparse intrahepatic biliary network, several biliary epithelial cell defects, and poor bile excretion to the gall bladder compared with uninjected embryos. Biliary defects were reproduced with the EGFR-blocker AG1478 alone or with Mo-arf6 at lower doses of each agent and rescued with arf6 mRNA.The BA-associated SNPs identify a chromosome 14q21.3 susceptibility locus encompassing the ARF6 gene. arf6 knockdown in zebrafish implicates early biliary dysgenesis as a basis for BA, and also

  6. Esophageal atresia: long-term interdisciplinary follow-up

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    Lidia B. Giúdici

    2016-07-01

    Full Text Available Background: We provide protocolized interdisciplinary follow-up to babies born with Esophageal Atresia (EA. There are few reports in Argentina about follow-up of EA patients.Objective: To describe outcomes in follow-up of EA patients at 1, 3 and 6 years old and to compare outcomes at age 1 with those at age 6.Methods: Prospective, longitudinal, analytic study of the cohort of babies born with EA, admitted to the follow-up program from 11/01/03 to 10/31/14. Follow-up includes: growth (weight > 10th centile, WHO, neurology-psychomotor development, audiology, vision, genetic, mental health, surgical reintervention, phonostomatology, language, pulmonology, re-hospitalization for clinical causes, lost to follow-up. Outcomes were described at age 1, 3 and 6. We included all EA patients who had reached age 1 at the start of this study.Results: 27 babies were admitted; 30% had long-gap EA; 18% presented VACTERL association; 23 children met inclusion criteria. Genetics  was assessed in 18 newborns (78%; a chromosomal map was performed in 11 babies; 3 had an abnormal karyotype. Mental health: 5/14 of the assessed children showed problems. Phonostomatology: 11 newborns checked (6 required treatment, 4 recovered at age 1. Pulmonologist evaluated 18 babies (7 with recurrent wheezing, 6 with moderate tracheomalacia. Gastroenterology and endoscopy: 80% presented gastroesophageal reflux (GER grade 3-4, and 50% showed a pathologic pHmetry. Lost to follow-up: age 1, 2 (8%; age 3, 3 (17%; age 6, 3 (23%. Normal outcomes observed are the following. Age 1 – growth: 81%; neurologic-psychomotor developmental index (NPDI: 76%; audiology: 95%; vision: 85%; language: 62%; re-hospitalization for clinical causes: 38%; surgical reinterventions: 47%. Age 3 – growth: 78%; NPDI: 50%; audiology: 93%; vision: 93%; language: 43%; re-hospitalization: 35%; surgical reinterventions: 14%. Age 6 – growth: 50%; NPDI: 30%; audiology: 90%; vision: 40%; language: 50%; re

  7. Equol inhibits growth, induces atresia, and inhibits steroidogenesis of mouse antral follicles in vitro

    International Nuclear Information System (INIS)

    Mahalingam, Sharada; Gao, Liying; Gonnering, Marni; Helferich, William; Flaws, Jodi A.

    2016-01-01

    Equol is a non-steroidal estrogen metabolite produced by microbial conversion of daidzein, a major soy isoflavone, in the gut of some humans and many animal species. Isoflavones and their metabolites can affect endogenous estradiol production, action, and metabolism, potentially influencing ovarian follicle function. However, no studies have examined the effects of equol on intact ovarian antral follicles, which are responsible for sex steroid synthesis and further development into ovulatory follicles. Thus, the present study tested the hypothesis that equol inhibits antral follicle growth, increases follicle atresia, and inhibits steroidogenesis in the adult mouse ovary. To test this hypothesis, antral follicles isolated from adult CD-1 mice were cultured with vehicle control (dimethyl sulfoxide; DMSO) or equol (600 nM, 6 μM, 36 μM, and 100 μM) for 48 and 96 h. Every 24 h, follicle diameters were measured to monitor growth. At 48 and 96 h, the culture medium was subjected to measurement of hormone levels, and the cultured follicles were subjected to gene expression analysis. Additionally, follicles were histologically evaluated for signs of atresia after 96 h of culture. The results indicate that equol (100 μM) inhibited follicle growth, altered the mRNA levels of bcl2-associated X protein and B cell leukemia/lymphoma 2, and induced follicle atresia. Further, equol decreased the levels of estradiol, testosterone, androstenedione, and progesterone, and it decreased mRNA levels of cholesterol side-chain cleavage, steroid 17-α-hydroxalase, and aromatase. Collectively, these data indicate that equol inhibits growth, increases atresia, and inhibits steroidogenesis of cultured mouse antral follicles. - Highlights: • Equol exposure inhibits antral follicle growth. • Equol exposure increases follicle atresia. • Equol exposure inhibits sex steroid hormone levels. • Equol exposure inhibits mRNA levels of certain steroidogenic enzymes.

  8. Equol inhibits growth, induces atresia, and inhibits steroidogenesis of mouse antral follicles in vitro

    Energy Technology Data Exchange (ETDEWEB)

    Mahalingam, Sharada, E-mail: mahalin2@illinois.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States); Gao, Liying, E-mail: lgao@uiuc.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States); Gonnering, Marni, E-mail: mgonne2@illinois.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States); Helferich, William, E-mail: helferic@illinois.edu [Department of Food Science and Human Nutrition, University of Illinois, 905 S. Goodwin, Urbana, IL 61801 (United States); Flaws, Jodi A., E-mail: jflaws@illinois.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States)

    2016-03-15

    Equol is a non-steroidal estrogen metabolite produced by microbial conversion of daidzein, a major soy isoflavone, in the gut of some humans and many animal species. Isoflavones and their metabolites can affect endogenous estradiol production, action, and metabolism, potentially influencing ovarian follicle function. However, no studies have examined the effects of equol on intact ovarian antral follicles, which are responsible for sex steroid synthesis and further development into ovulatory follicles. Thus, the present study tested the hypothesis that equol inhibits antral follicle growth, increases follicle atresia, and inhibits steroidogenesis in the adult mouse ovary. To test this hypothesis, antral follicles isolated from adult CD-1 mice were cultured with vehicle control (dimethyl sulfoxide; DMSO) or equol (600 nM, 6 μM, 36 μM, and 100 μM) for 48 and 96 h. Every 24 h, follicle diameters were measured to monitor growth. At 48 and 96 h, the culture medium was subjected to measurement of hormone levels, and the cultured follicles were subjected to gene expression analysis. Additionally, follicles were histologically evaluated for signs of atresia after 96 h of culture. The results indicate that equol (100 μM) inhibited follicle growth, altered the mRNA levels of bcl2-associated X protein and B cell leukemia/lymphoma 2, and induced follicle atresia. Further, equol decreased the levels of estradiol, testosterone, androstenedione, and progesterone, and it decreased mRNA levels of cholesterol side-chain cleavage, steroid 17-α-hydroxalase, and aromatase. Collectively, these data indicate that equol inhibits growth, increases atresia, and inhibits steroidogenesis of cultured mouse antral follicles. - Highlights: • Equol exposure inhibits antral follicle growth. • Equol exposure increases follicle atresia. • Equol exposure inhibits sex steroid hormone levels. • Equol exposure inhibits mRNA levels of certain steroidogenic enzymes.

  9. Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence: two case reports and a review of the literature.

    Science.gov (United States)

    Bjørsum-Meyer, Thomas; Herlin, Morten; Qvist, Niels; Petersen, Michael B

    2016-12-21

    The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases. Furthermore, we aimed to collect existing knowledge in the embryopathogenesis and genetics in order to discuss a possible link between the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome. Our first case was a white girl delivered by caesarean section at 37 weeks of gestation; our second case was a white girl born at a gestational age of 40 weeks. A co-occurrence of vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome was diagnosed in both cases. We performed a systematic literature search in PubMed ((VACTERL) OR (VATER)) AND ((MRKH) OR (Mayer-Rokitansky-Küster-Hauser) OR (mullerian agenesis) OR (mullerian aplasia) OR (MURCS)) without limitations. A similar search was performed in Embase and the Cochrane library. We added two cases from our local center. All cases (n = 9) presented with anal atresia and renal defect. Vertebral defects were present in eight patients. Rectovestibular fistula was confirmed in seven patients. Along with the uterovaginal agenesis, fallopian tube aplasia appeared in five of nine cases and in two cases ovarian involvement also existed. The co-occurrence of the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal

  10. Fatores relacionados ao prognóstico da atresia biliar pós-portoenterostomia Factors related to the post-portoenterostomy prognosis of biliary atresia

    Directory of Open Access Journals (Sweden)

    Jorge Luiz dos Santos

    2002-01-01

    Full Text Available Objetivo: o estudo está envolvido com o prognóstico da atresia biliar pós-portoenterostomia, presença de anomalias congênitas associadas à doença e de malformação de placa ductal, área de fibrose hepática e, sobretudo, com a idade dos pacientes por ocasião da cirurgia. O presente estudo verificou numa amostra de atresia biliar as implicações prognósticas destes fatores. Métodos: foram avaliados 47 pacientes com atresia biliar, em estudo de corte transversal. O material histológico dos casos foi marcado com anticorpo anticitoqueratina 19 e CAM 5.2 por método imunoistoquímico, para o estudo das estruturas biliares, e corado com picrossírius para avaliação da área de fibrose. O estudo das estruturas biliares foi realizado por dois patologistas e pelo primeiro autor deste estudo, "cegos" quanto à evolução dos casos. A mensuração da área de fibrose foi quantitativa. Os dados dos pacientes em relação à idade, ocorrência de óbito ou realização de transplante hepático foram pesquisados nos prontuários. Resultados: a idade por ocasião da portoenterostomia variou entre 24 e 251 dias de vida (90,4 + 44,8 dias, e em 32 casos (72% a evolução pôde ser acompanhada. Os 9 casos (19% com anomalias congênitas extra-hepáticas associadas não diferiram quanto ao prognóstico em relação ao restante da amostra. A idade por ocasião da portoenterostomia influenciou o prognóstico (p=0,016. A área de fibrose foi diferente entre pacientes operados com menos de 60 dias de vida e os operados com mais de 90 dias (p= 0,023, mas não influenciou a evolução dos casos. Tampouco a presença de malformação de placa ductal influiu no prognóstico. Conclusões: a idade por ocasião da portoenterostomia foi o único fator que afetou o prognóstico dos casos de atresia biliar. É necessário maior número de pacientes para avaliar a influência da presença de anomalias congênitas extra-hepáticas associadas sobre a evolução p

  11. MINERALIZACION OSEA EN PACIENTES CON INSUFICIENCIA HEPÁTICA

    OpenAIRE

    Muzzo B, Santiago; Cordero, Jaime; Ceresa, Sergio; Buchel, Erwin; Gajardo L, Hector; Leiva B, Laura

    2004-01-01

    La insuficiencia hepática (IH) crónica altera la mineralización ósea (MO) de acuerdo a la etiología de la IH, debido a los problemas nutricionales agregados y por el tratamiento post transplante con inmunosupresores y glucocorticoides. El objetivo del presente estudio fue conocer la mineralización ósea en niños con insuficiencia hepática secundaria a atresia de vías biliares y la dinámica ósea. La mineralización ósea se determinó en columna lumbar (L2-L4) mediante un densitómetro Lunar radiol...

  12. Atresia Hymenalis with Haematometrocolpos: A Benefit of Teaching a Case Series and Review of the Literature

    LENUS (Irish Health Repository)

    Whitla, L

    2017-09-01

    Imperforate hymen or atresia hymenalis is a rare clinical presentation, the condition is estimated to be present in 0.5\\/1000 females1. It is the most common obstructive anomaly of the female genital tract, and results from failure of canalization of the vaginal plate in utero. Atresia hymenalis can present in neonates as bulging hymen due to accumulation of secretions by the uterovaginal mucosa2,3. Usually the imperforate hymen is asymptomatic until menarche, when haematometrocolpos results in symptoms such as abdominal pain, abdominal mass, urinary retention and constipation. Many cases are diagnosed in the evaluation of primary amenorrhoea or recurrent abdominal pain2,3. We present 4 cases diagnosed over a 7 month period in our ED.

  13. Radiological evaluation of pulmonary atresia: An analysis of cineangiography in 32 cases

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Ho; Yeon, Kyung Mo; Park, Jae Hyung; Han, Man Chung; Yoon, Yong Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1983-06-15

    Total 32 cases of pulmonary atresia were diagnosed radiographically at Seoul National University Hospital from March, 1979 to August 1982. Some characteristic radiological findings were analyzed in chest PA and cineangiographies. The results were as follows: 1. In the evaluation of chest PA, cardiomegaly was noticed in 16 cases, dextrocardia in 8 cases, elevated cardiac apex in 7 cases and right-sided aortic arch in 6 cases. The pulmonary vascularties were mildly decreased in 20 cases, markedly decreased in 9 cases and decreased with reticular pattern in 3 cases. 2. As final diagnoses after cineangiography, pulmonary atresia was associated with Tetralogy of Fallot variant in 17 cases, transposition of great vessels in 7 cases, single ventricle in 5 cases, tricuspid atresia in 2 cases and intact ventricular septum in 1 case. 3. The classification according to the pattern of pulmonary artery is main pulmonary trunk with PDA(Type Ia) in 10 cases, pulmonary arterial confluence with PDA (Type Ib) in 10 cases, no pulmonary arterial confluence with PDA (Type Ic) in 5 cases, main pulmonary trunk without PDA (Type IIa) in 0 case, pulmonary arterial confluence without PDA (Type IIb) in 5 cases, and no pulmonary arterial confluence without PDA ( Type IIc) in 2 cases. 4. Pulmonary wedge venography was done and successful in 8 cases. Among them, confluence between right and left pulmonary arteries was noticed in 5 cases. 5. Biventricular cineangiograpy and/or pulmonary wedge venography, if necessary, is essential for the accurate diagnosis of pulmonary atresia to demonstrate detailed anatomy of pulmonary artery.

  14. Radiological evaluation of pulmonary atresia: An analysis of cineangiography in 32 cases

    International Nuclear Information System (INIS)

    Lee, Dong Ho; Yeon, Kyung Mo; Park, Jae Hyung; Han, Man Chung; Yoon, Yong Soo

    1983-01-01

    Total 32 cases of pulmonary atresia were diagnosed radiographically at Seoul National University Hospital from March, 1979 to August 1982. Some characteristic radiological findings were analyzed in chest PA and cineangiographies. The results were as follows: 1. In the evaluation of chest PA, cardiomegaly was noticed in 16 cases, dextrocardia in 8 cases, elevated cardiac apex in 7 cases and right-sided aortic arch in 6 cases. The pulmonary vascularties were mildly decreased in 20 cases, markedly decreased in 9 cases and decreased with reticular pattern in 3 cases. 2. As final diagnoses after cineangiography, pulmonary atresia was associated with Tetralogy of Fallot variant in 17 cases, transposition of great vessels in 7 cases, single ventricle in 5 cases, tricuspid atresia in 2 cases and intact ventricular septum in 1 case. 3. The classification according to the pattern of pulmonary artery is main pulmonary trunk with PDA(Type Ia) in 10 cases, pulmonary arterial confluence with PDA (Type Ib) in 10 cases, no pulmonary arterial confluence with PDA (Type Ic) in 5 cases, main pulmonary trunk without PDA (Type IIa) in 0 case, pulmonary arterial confluence without PDA (Type IIb) in 5 cases, and no pulmonary arterial confluence without PDA ( Type IIc) in 2 cases. 4. Pulmonary wedge venography was done and successful in 8 cases. Among them, confluence between right and left pulmonary arteries was noticed in 5 cases. 5. Biventricular cineangiograpy and/or pulmonary wedge venography, if necessary, is essential for the accurate diagnosis of pulmonary atresia to demonstrate detailed anatomy of pulmonary artery

  15. Sirenomelia and ndash; Mermaid Syndrome with Oesophageal Atresia: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Veena Raja

    2015-09-01

    Full Text Available Sirenomelia or Mermaid syndrome is a very rare congenital deformity in which legs are fused together and commonly associated with abnormal kidney development, genital, and rectal abnormalities. In this present case, sirenomelia was associated with oesophageal atresia, which is a rare association and occurs in about 20-35% of cases. [J Interdiscipl Histopathol 2015; 3(3.000: 113-116

  16. Multidetector Computed Tomography Assessment in Biliary Atresia for the Diagnosis of Portosystemic Collaterals before Liver Transplant

    International Nuclear Information System (INIS)

    Cadavid A, Lina; Barber, Ignasi; Bueno, Javier

    2011-01-01

    Introduction: Chronic liver disease increases portal vein pressure and modifies splanchnic circulation. This is particularly significant in infants with biliary atresia. Large collaterals steal portal flow and increase the risk of post transplant portal vein thrombosis. Objective: to describe different types of portosystemic collaterals prior to liver transplantation with low-dose multidetector CT (MDCT) in patients with biliary atresia. Material and methods: 13 patients with severe liver dysfunction due to biliary atresia underwent low-dose 64-MDCT before liver transplantation (effective tube current ranged from 20 to 120 mAs according to weight, with a kilo voltage of 80-120 for all CT). Hepatic arterial and portal venous phases were performed after IV contrast administration [1.5-2 ml/kg]. The mean age of the study group was1 year (range, 4 months to 3.6 years). Two radiologists reviewed the CT images to determine the grade and types of the portosystemic collaterals. Results: A total of 16 CT scans were obtained.the most common portosystemic collaterals found were esophageal (11), gastric submucosal (8), gastric adventitial (7, splenic (7), hemorrhoidal (10), mesenteric [dilated or tortuous branches of the inferior mesenteric vein (8)], retroperitoneal varices [gastro renal shunt (10), splenorenal shunt (4)] and dilated or tortuous left gastric vein (13). Conclusion: MDCT provides important information on venous system patency, presence of varices, and location of venous shunts in pediatric patients with biliary atresia going to liver transplant. in addition, it is critical to detect collaterals that are not evident on ultrasound in order to avoid the steal phenomenon that may lead to portal vein thrombosis and graft failure.

  17. Split thickness skin graft for cervicovaginal reconstruction in congenital atresia of cervix.

    Science.gov (United States)

    Zhang, Xuyin; Han, Tiantian; Ding, Jingxin; Hua, Keqin

    2015-10-01

    To introduce a new technique that combines laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in patients with congenital atresia of the cervix. Video article introducing a new surgical technique. University hospital. A 16-year-old patient with congenital cervical atresia, vaginal dysgenesis, and ovarian endometrial cyst. An original technique of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft for cervicovaginal reconstruction. A midline incision at the vaginal introitus was made, and a 9-cm canal was made between the bladder and the rectum using sharp and blunt dissection along the anatomic vaginal route, with the aid of laparoscopy to ensure correct orientation. A 14 × 12 cm split thickness skin graft was harvested from the right lateral thigh. By laparoscopy, the level of the lowest pole of the uterine cavity was exposed and the cervix was incised by shape dissection. The proximal segment of the harvested skin to the lower uterine segment was secured, and the distal segment was sutured with the upper margin of vulva vaginally. Surgical technique reports in anonymous patients are exempted from ethical approval by the Institutional Review Board. The patient gave consent to use the video in the article. The procedure was successfully completed. Since February 2013, our experiences of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in 10 patients with congenital atresia of cervix were positive, with successful results and without complications or cervical, or vaginal stenosis. Our technique is feasible and safe for congenital atresia of cervix, with successful results and without complications or cervical or vaginal stenosis. Copyright © 2015 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  18. Long-term pulmonary function in esophageal atresia-A case-control study

    DEFF Research Database (Denmark)

    Pedersen, Rikke N; Markøv, Simone; Kruse-Andersen, Søren

    2017-01-01

    BACKGROUND: Esophageal atresia (EA) is a congenital anomaly associated with substantial pulmonary morbidity throughout childhood. AIM: The aim of this study was to evaluate pulmonary complications among 59 five to 15-year-old children and adolescents with surgically corrected congenital EA. METHO...... ventilatory impairment occurring in EA is probably due to poor lung growth after thoracotomy. No single factor predicted ventilatory impairment in children and adolescents with EA. Pediatr Pulmonol. 2016; 9999:XX-XX. © 2016 Wiley Periodicals, Inc....

  19. Specific echocardiographic findings useful for the diagnosis of common pulmonary vein atresia

    Directory of Open Access Journals (Sweden)

    Hiroyuki Nagasawa

    2015-12-01

    Full Text Available In this paper, we report a case of common pulmonary vein atresia, which is a very rare disease characterized by cyanosis, heart failure and pulmonary hypertension. Reverse flow in the pulmonary artery at end-diastole as well as in the isthmus of the aorta from early systole to end-diastole detected by echocardiography were found to be specific features useful in diagnosing the disease.

  20. A functional study on small intestinal smooth muscles in jejunal atresia

    Directory of Open Access Journals (Sweden)

    Preeti Tyagi

    2016-01-01

    Full Text Available Aim: The present study was aimed to assess the contractile status of neonatal small intestinal smooth muscle of dilated pre-atretic part of intestinal atresia to resolve debatable issues related to mechanisms of persistent dysmotility after surgical repair. Materials and Methods: A total of 34 longitudinally sectioned strips were prepared from pre-atretic dilated part of freshly excised 8 jejunal atresia type III a cases. Spontaneous as well as acetylcholine- and histamine-induced contractions were recorded in vitro by using organ bath preparations. Chemically evoked contractions were further evaluated after application of atropine (muscarinic blocker, pheniramine (H1 blocker, and lignocaine (neuronal blocker to ascertain receptors and neuronal involvement. Histological examinations of strips were made by using Masson trichrome stain to assess the fibrotic changes. Results: All 34 strips, except four showed spontaneous contractions with mean frequency and amplitude of 5.49 ± 0.26/min and 24.41 ± 5.26 g/g wet tissue respectively. The response to ACh was nearly twice as compared to histamine for equimolar concentrations (100 μM. ACh (100 μM induced contractions were attenuated (by 60% by atropine. Histamine (100 μM-induced contractions was blocked by pheniramine (0.32 μM and lignocaine (4 μM by 74% and 78%, respectively. Histopathological examination showed varying degree of fibrotic changes in muscle layers. Conclusions: Pre-atretic dilated part of jejunal atresia retains functional activity but with definitive histopathologic abnormalities. It is suggested that excision of a length of pre-atretic part and early stimulation of peristalsis by locally acting cholinomimetic or H1 agonist may help in reducing postoperative motility problems in atresia patients.

  1. A functional study on small intestinal smooth muscles in jejunal atresia.

    Science.gov (United States)

    Tyagi, Preeti; Mandal, Maloy B; Gangopadhyay, Ajay N; Patne, Shashikant C U

    2016-01-01

    The present study was aimed to assess the contractile status of neonatal small intestinal smooth muscle of dilated pre-atretic part of intestinal atresia to resolve debatable issues related to mechanisms of persistent dysmotility after surgical repair. A total of 34 longitudinally sectioned strips were prepared from pre-atretic dilated part of freshly excised 8 jejunal atresia type III a cases. Spontaneous as well as acetylcholine- and histamine-induced contractions were recorded in vitro by using organ bath preparations. Chemically evoked contractions were further evaluated after application of atropine (muscarinic blocker), pheniramine (H1 blocker), and lignocaine (neuronal blocker) to ascertain receptors and neuronal involvement. Histological examinations of strips were made by using Masson trichrome stain to assess the fibrotic changes. All 34 strips, except four showed spontaneous contractions with mean frequency and amplitude of 5.49 ± 0.26/min and 24.41 ± 5.26 g/g wet tissue respectively. The response to ACh was nearly twice as compared to histamine for equimolar concentrations (100 μM). ACh (100 μM) induced contractions were attenuated (by 60%) by atropine. Histamine (100 μM)-induced contractions was blocked by pheniramine (0.32 μM) and lignocaine (4 μM) by 74% and 78%, respectively. Histopathological examination showed varying degree of fibrotic changes in muscle layers. Pre-atretic dilated part of jejunal atresia retains functional activity but with definitive histopathologic abnormalities. It is suggested that excision of a length of pre-atretic part and early stimulation of peristalsis by locally acting cholinomimetic or H1 agonist may help in reducing postoperative motility problems in atresia patients.

  2. The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Nathalie Rommel

    2017-06-01

    Full Text Available Infants and children with esophageal atresia commonly present with swallowing dysfunction or dysphagia. Dysphagia can lead to a range of significant consequences such as aspiration pneumonia, malnutrition, dehydration, and food impaction. To improve oral intake, the clinical diagnosis of dysphagia in patients with esophageal atresia should focus on both the pharynx and the esophagus. To characterize the complex interactions of bolus flow and motor function between mouth, pharynx, and esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus.

  3. The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia.

    Science.gov (United States)

    Rommel, Nathalie; Rayyan, Maissa; Scheerens, Charlotte; Omari, Taher

    2017-01-01

    Infants and children with esophageal atresia commonly present with swallowing dysfunction or dysphagia. Dysphagia can lead to a range of significant consequences such as aspiration pneumonia, malnutrition, dehydration, and food impaction. To improve oral intake, the clinical diagnosis of dysphagia in patients with esophageal atresia should focus on both the pharynx and the esophagus. To characterize the complex interactions of bolus flow and motor function between mouth, pharynx, and esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA) method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus.

  4. Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

    Directory of Open Access Journals (Sweden)

    Abdel-Aziz Mosaad

    2012-06-01

    Full Text Available Abstract Background Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis. Case presentation A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma. Conclusions Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology.

  5. MRI-based decision tree model for diagnosis of biliary atresia.

    Science.gov (United States)

    Kim, Yong Hee; Kim, Myung-Joon; Shin, Hyun Joo; Yoon, Haesung; Han, Seok Joo; Koh, Hong; Roh, Yun Ho; Lee, Mi-Jung

    2018-02-23

    To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice. We retrospectively reviewed features of MRI and ultrasonography (US) performed in infants with jaundice between January 2009 and June 2016 under approval of the institutional review board, including the maximum diameter of periportal signal change on MRI (MR triangular cord thickness, MR-TCT) or US (US-TCT), visibility of common bile duct (CBD) and abnormality of gallbladder (GB). Hepatic subcapsular flow was reviewed on Doppler US. We performed conditional inference tree analysis using MRI findings to generate a decision tree model. A total of 208 infants were included, 112 in the BA group and 96 in the non-BA group. Mean age at the time of MRI was 58.7 ± 36.6 days. Visibility of CBD, abnormality of GB and MR-TCT were good discriminators for the diagnosis of BA and the MRI-based decision tree using these findings with MR-TCT cut-off 5.1 mm showed 97.3 % sensitivity, 94.8 % specificity and 96.2 % accuracy. MRI-based decision tree model reliably differentiates BA in infants with jaundice. MRI can be an objective imaging modality for the diagnosis of BA. • MRI-based decision tree model reliably differentiates biliary atresia in neonatal cholestasis. • Common bile duct, gallbladder and periportal signal changes are the discriminators. • MRI has comparable performance to ultrasonography for diagnosis of biliary atresia.

  6. The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

    Science.gov (United States)

    Rommel, Nathalie; Rayyan, Maissa; Scheerens, Charlotte; Omari, Taher

    2017-01-01

    Infants and children with esophageal atresia commonly present with swallowing dysfunction or dysphagia. Dysphagia can lead to a range of significant consequences such as aspiration pneumonia, malnutrition, dehydration, and food impaction. To improve oral intake, the clinical diagnosis of dysphagia in patients with esophageal atresia should focus on both the pharynx and the esophagus. To characterize the complex interactions of bolus flow and motor function between mouth, pharynx, and esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA) method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus. PMID:28680874

  7. Decisional Conflict in Parents Considering Bone-Anchored Hearing Devices in Children With Unilateral Aural Atresia.

    Science.gov (United States)

    Graham, M Elise; Haworth, Rebecca; Chorney, Jill; Bance, Manohar; Hong, Paul

    2015-12-01

    The benefits of bone-anchored hearing devices (BAHD) in children with unilateral aural atresia are controversial. We sought to determine whether there is parental decisional conflict surrounding elective placement of BAHD for this indication. Caregivers of pediatric patients with unilateral aural atresia and normal contralateral ear undergoing percutaneous BAHD consultation were enrolled. All consultations were carried out by one pediatric otolaryngologist in a consistent manner. After consultation, the participants completed a demographics form and the Decisional Conflict Scale (DCS) questionnaire. Twenty-three caregivers of 15 male (65.2%) and 8 female (34.8%) children (mean age 5.65 years) participated. The overall median DCS score was 15.63 (standard error = 4.21). Significant decisional conflict (DCS score ≥ 25) was found in 10 participants (43.5%). The median DCS score in the group choosing surgery was 5.47, and it was 23.44 in those who did not choose surgery (Mann-Whitney U = 39, Z = -1.391, P = .164). The median DCS score for mothers and fathers was 25 and 3.91, respectively. Many parents experienced significant decisional conflict when considering percutaneous BAHD surgery in children with unilateral aural atresia in our study population. Future research should explore the impact of decisional conflict on health outcomes. © The Author(s) 2015.

  8. BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

    Directory of Open Access Journals (Sweden)

    Thais Costa Nascentes QUEIROZ

    2014-03-01

    Full Text Available Context Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that pre­sents with biliary obstruction exclusively in the neonatal period. Objectives To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Methods Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Results Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001. On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15, but difference was found when comparing the age at surgery (P = 0.002. Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Conclusions Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

  9. Value of sup(99m)Tc-diethyl-IDA scintigraphy for the diagnosis of biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Bourdelat, D.; Gruel, Y.; Guibert, L.; Babut, J.M. (Hopital Pontchaillou 35 - Rennes (France)); Bourguet, P.; Herry, J.Y. (Centre Eugene Marquis, CHR Pontchaillou, 35 - Rennes (France))

    1983-04-01

    With reference to three cases, the value of cholescintigraphy for the diagnosis of biliary atresia is underscored. With this procedure, surgical indications can be determined in neonates with jaundice persisting beyond the physiologic period. Clolescintigraphy can be repeated as it is easy to perform, safe and well tolerated. sup(99m)Tc-diethyl-IDA (technetium 99m labelled N-(2,6 diethyl-acetanilide)-iminodiacetic acid) scintigraphy was carried out in eight neonates. This procedure helped to outrule the diagnosis of biliary atresia in 5 cases (1 choledochal cyst, 1 alpha-1-antitrypsin deficiency and 3 neonatal hepatitis'). Scintigraphic images and time/activity curves generated simultaneously from equal surfaces over the heart and liver were analyzed separately. A good uptake by the liver (rapid decrease in the cardiac curve) with subsequent retention (no decrease in the hepatic curve) is suggestive of biliary atresia. No activity is detected in the biliary ducts or intestinal tract. Post-operatively, cholescintigraphy is a useful tool for controling the efficiency of the surgical procedure.

  10. Spastic quadriplegia in Down syndrome with congenital duodenal stenosis/atresia.

    Science.gov (United States)

    Kurosawa, Kenji; Enomoto, Keisuke; Tominaga, Makiko; Furuya, Noritaka; Sameshima, Kiyoko; Iai, Mizue; Take, Hiroshi; Shinkai, Masato; Ishikawa, Hiroshi; Yamanaka, Michiko; Matsui, Kiyoshi; Masuno, Mitsuo

    2012-06-01

    Down syndrome is an autosomal chromosome disorder, characterized by intellectual disability and muscle hypotonia. Muscle hypotonia is observed from neonates to adulthood in Down syndrome patients, but muscle hypertonicity is extremely unusual in this syndrome. During a study period of nine years, we found three patients with severe spastic quadriplegia among 20 cases with Down syndrome and congenital duodenal stenosis/atresia (3/20). However, we could find no patient with spastic quadriplegia among 644 cases with Down syndrome without congenital duodenal stenosis/atresia during the same period (0/644, P quadriplegia among 17 patients with congenital duodenal stenosis/atresia without Down syndrome admitted during the same period to use as a control group (0/17, P quadriplegia in patients with Down syndrome. Long-term survival is improving, and the large majority of people with Down syndrome are expected to live well into adult life. Management and further study for the various problems, representing a low prevalence but serious and specific to patients with Down syndrome, are required to improve their quality of life. © 2012 The Authors. Congenital Anomalies © 2012 Japanese Teratology Society.

  11. Biliary atresia: evaluation on two distinct periods at a reference pediatric service.

    Science.gov (United States)

    Queiroz, Thais Costa Nascentes; Ferreira, Alexandre Rodrigues; Fagundes, Eleonora Druve Tavares; Roquete, Mariza Leitão Valadares; Penna, Francisco José

    2014-01-01

    Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

  12. New successful one-step surgical repair for apple peel atresia

    Directory of Open Access Journals (Sweden)

    Machmouchi M

    2011-11-01

    Full Text Available Mahmoud MachmouchiDepartment of Pediatrics, Royal Commission Hospital, Jubail, Saudi ArabiaAbstract: A new successful surgical approach in two identical twins delivered with intestinal atresia, “apple peel” type is reported. This technique consists of: (1 an end-to-end oblique primary anastomosis with single layer inverted 5/0 Vicryl® sutures (Ethicon, Inc, Somerville, NJ; (2 proximal tube jejunostomy using Foley catheter, size 10 French, inserted through a stab wound in the left upper quadrant and entering the proximal dilated loop at about 10 cm proximal from its blind end (site of anastomosis; and (3 transanastomotic stenting using feeding tube, size 6 French, exteriorized in conjunction with the Foley catheter and reaching the lumen of the distal loop for more than 20 cm. The postoperative course was uneventful and progressive oral feed became ad libitum around day 40 postoperative. This procedure is simple, performed in one stage, and responds to the most obligatory requirements of this congenital malformation.Keywords: proximal jejunal atresia, intestinal atresia, surgical repair, anastomosis

  13. Acute iliofemoral venous thrombosis in patients with atresia of the inferior vena cava can be treated successfully with catheter-directed thrombolysis

    DEFF Research Database (Denmark)

    Broholm, Rikke; Jørgensen, Maja; Just, Sven

    2011-01-01

    To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT).......To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT)....

  14. Formation of intestinal atresias in the Fgfr2IIIb-/- mice is not associated with defects in notochord development or alterations in Shh expression.

    Science.gov (United States)

    Reeder, Amy L; Botham, Robert A; Franco, Marta; Zaremba, Krzysztof M; Nichol, Peter F

    2012-09-01

    The etiology of intestinal atresia remains elusive but has been ascribed to a number of possible events including in utero vascular accidents, failure of recanalization of the intestinal lumen, and mechanical compression. Another such event that has been postulated to be a cause in atresia formation is disruption in notochord development. This hypothesis arose from clinical observations of notochord abnormalities in patients with intestinal atresias as well as abnormal notochord development observed in a pharmacologic animal model of intestinal atresia. Atresias in this model result from in utero exposure to Adriamycin, wherein notochord defects were noted in up to 80% of embryos that manifested intestinal atresias. Embryos with notochord abnormalities were observed to have ectopic expression of Sonic Hedgehog (Shh), which in turn was postulated to be causative in atresia formation. We were interested in determining whether disruptions in notochord development or Shh expression occurred in an established genetic model of intestinal atresia and used the fibroblast growth factor receptor 2IIIb homozygous mutant (Fgfr2IIIb-/-) mouse model. These embryos develop colonic atresias (100% penetrance) and duodenal atresias (42% penetrance). Wild-type and Fgfr2IIIb-/- mouse embryos were harvested at embryonic day (E) 10.5, E11.5, E12.5, and E13.5. Whole-mount in situ hybridization was performed on E10.5 embryos for Shh. Embryos at each time point were harvested and sectioned for hematoxylin-eosin staining. Sections were photographed specifically for the notochord and resulting images reconstructed in 3-D using Amira software. Colons were isolated from wild-type and Fgfr2IIIb-/- embryos at E10.5, then cultured for 48 hours in Matrigel with FGF10 in the presence or absence of exogenous Shh protein. Explants were harvested, fixed in formalin, and photographed. Fgfr2IIIb-/- mouse embryos exhibit no disruptions in Shh expression at E10.5, when the first events in atresia

  15. Meconial peritonitis in a rare association of partial ileal apple-peel atresia with small abdominal wall defect

    Directory of Open Access Journals (Sweden)

    V. Insinga

    2014-06-01

    Full Text Available Intestinal atresia type III B (apple peel and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation. Paralytic ileus and infections are the main causes of morbidity and mortality at neonatal age. In our case, in spite of the mild phenotype, prognosis has been complicated by the onset of functional bowel obstruction, caused by chemical peritonitis resulting from contact with either amniotic fluid and meconium.

  16. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar Angiographic study of pulmonary circulation in tetralogy of Fallot with pulmonary atresia

    Directory of Open Access Journals (Sweden)

    Marco Aurélio Santos

    2005-02-01

    Full Text Available OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1 veia pulmonar encunhada, 2 colaterais aortopulmonares, 3 aorta torácica e 4 ductus arteriosus e/ou shunt sistêmico pulmonar. RESULTADOS: Dos 56 pacientes, 15 tinham o suprimento sangüíneo pulmonar através de colaterais aortopulmonares, em 36 o suprimento sangüíneo pulmonar era feito isoladamente pelo ductus arteriosus e em 5 pelo ductus arteriosus e colaterais aortopulmonares. Conforme a presença ou ausência de estruturas vasculares que compõem a circulação pulmonar na tetralogia de Fallot com atresia pulmonar e do tipo de perfusão vascular pulmonar, os doentes foram classificados em 6 tipos. CONCLUSÃO: Em função da grande complexidade e extrema variabilidade do suprimento sangüíneo pulmonar na tetralogia de Fallot com atresia pulmonar torna-se possível, com este tipo de abordagem, a obtenção de informações, suficientemente necessárias, para o correto manuseio clínico-cirúrgico.OBJECTIVE: To identify the types of pulmonary vascular blood supply in tetralogy of Fallot with pulmonary atresia by use of hemodynamic study. METHODS: Fifty-six patients with tetralogy of Fallot and pulmonary atresia, and ages ranging from 20 days to 4 years, underwent cineangiocardiographic study with contrast medium injections in the following vascular structures: 1 wedged pulmonary vein; 2 aortopulmonary collaterals; 3 thoracic aorta; and 4 ductus arteriosus or systemic-pulmonary shunt. RESULTS: In the 56 patients studied, pulmonary blood was supplied as follows: in 15, by aortopulmonary collaterals; in 36, only by the ductus arteriosus; and in 5, by the ductus

  17. Uterovaginal Anastomosis for Cases of Cryptomenorrhea Due to Cervical Atresia with Vaginal Aplasia: Benefits and Risks.

    Science.gov (United States)

    Zayed, M; Fouad, R; Elsetohy, K A; Hashem, A T; AbdAllah, A A; Fathi, A I

    2017-12-01

    The objective of this study was to assess short-term benefits and risks of utero-vaginal anastomosis done for cases of cryptomenorrhea due to cervical atresia with vaginal aplasia. Prospective study. Surgical procedures were done between December 2013 and September 2015 at the department of Obstetrics and Gynecology, Cairo University Hospital. Five patients who had cryptomenorrhea due to cervical atresia associated with vaginal aplasia were included. Utero-vaginal anastomoses were performed in 2 stages; a stage of McIndoe vaginoplasty and a stage of excision of the atretic cervical tissue and anastomosing the uterus to the neovagina. Follow-up was done by gynecological and ultrasound examination in a duration ranged from 12 to 36 months. Occurrence of regular menstrual flow and relief of the severe cyclic pain. All patients had relief of the severe cyclic pain. Four patients had regular menstrual flow. One patient developed occlusion of the track after 1 year and needed dilatation once. Three patients developed low vaginal stenosis without occlusion of the track. One patient had rectal injury repaired without causing postoperative morbidity. Uterovaginal anastomosis is a promising conservative management option for cervical atresia with vaginal aplasia, which has benefits but is not free of risks. Long-term follow-up is still needed to judge its feasibility. We recommend performing McIndoe vaginoplasty as a starting stage before the anastomosis preferably in a separate setting. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  18. Transcriptome profiling of the theca interna from bovine ovarian follicles during atresia.

    Directory of Open Access Journals (Sweden)

    Nicholas Hatzirodos

    Full Text Available The theca interna is a specialized stromal layer that envelops each growing ovarian follicle. It contains capillaries, fibroblasts, immune cells and the steroidogenic cells that synthesize androgens for conversion to estradiol by the neighboring granulosa cells. During reproductive life only a small number of follicles will grow to a sufficient size to ovulate, whereas the majority of follicles will undergo regression/atresia and phagocytosis by macrophages. To identify genes which are differentially regulated in the theca interna during follicular atresia, we undertook transcriptome profiling of the theca interna from healthy (n = 10 and antral atretic (n = 5 bovine follicles at early antral stages (<5 mm. Principal Component Analyses and hierarchical classification of the signal intensity plots for the arrays showed primary clustering into two groups, healthy and atretic. A total of 543 probe sets were differentially expressed between the atretic and healthy theca interna. Further analyses of these genes by Ingenuity Pathway Analysis and Gene Ontology Enrichment Analysis Toolkit software found most of the genes being expressed were related to cytokines, hormones and receptors as well as the cell cycle and DNA replication. Cell cycle genes which encode components of the replicating chromosome complex and mitotic spindle were down-regulated in atretic theca interna, whereas stress response and inflammation-related genes such as TP53, IKBKB and TGFB1 were up-regulated. In addition to cell cycle regulators, upstream regulators that were predicted to be inhibited included Retinoblastoma 1, E2 transcription factor 1, and hepatocyte growth factor. Our study suggests that during antral atresia of small follicles in the theca interna, arrest of cell cycle and DNA replication occurs rather than up- regulation of apoptosis-associated genes as occurs in granulosa cells.

  19. Pulmonary ventilation and perfusion abnormalities and ventilation perfusion imbalance in children with pulmonary atresia or extreme tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Dowdle, S.C.; Human, D.G.; Mann, M.D. (Univ. of Cape Town (South Africa))

    1990-08-01

    Xenon-133 lung ventilation and perfusion scans were done preoperatively after cardiac catheterization and cineangiocardiography in 19 children; 6 had pulmonary atresia with an intact ventricular septum and hypoplastic right ventricle, 4 pulmonary atresia with associated complex univentricular heart, and 9 extreme Tetralogy of Fallot. The four patients with discrepancies in the sizes of the left and right pulmonary arteries on angiography had marked asymmetry of pulmonary perfusion and ventilation-perfusion imbalance on scintigraphy. Similar degrees of asymmetry and imbalance were present in 6 of the 15 children with equal-size pulmonary vessels. Asymmetry of pulmonary perfusion and ventilation-perfusion imbalance were associated with a poor prognosis.

  20. Pulmonary ventilation and perfusion abnormalities and ventilation perfusion imbalance in children with pulmonary atresia or extreme tetralogy of Fallot

    International Nuclear Information System (INIS)

    Dowdle, S.C.; Human, D.G.; Mann, M.D.

    1990-01-01

    Xenon-133 lung ventilation and perfusion scans were done preoperatively after cardiac catheterization and cineangiocardiography in 19 children; 6 had pulmonary atresia with an intact ventricular septum and hypoplastic right ventricle, 4 pulmonary atresia with associated complex univentricular heart, and 9 extreme Tetralogy of Fallot. The four patients with discrepancies in the sizes of the left and right pulmonary arteries on angiography had marked asymmetry of pulmonary perfusion and ventilation-perfusion imbalance on scintigraphy. Similar degrees of asymmetry and imbalance were present in 6 of the 15 children with equal-size pulmonary vessels. Asymmetry of pulmonary perfusion and ventilation-perfusion imbalance were associated with a poor prognosis

  1. Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report

    Directory of Open Access Journals (Sweden)

    Madhavi Nori

    2013-01-01

    Full Text Available Extrahepatic biliary atresia (EHBA is an uncommon cause of neonatal jaundice. Antenatal Magnetic Resonance Imaging (MRI diagnosis of EHBA has not been published to the best of our knowledge till date. EHBA with cystic component is likely to be mistaken for choledochal cyst. A case that was antenatally predicted and postnatally confirmed by surgery and histopathology is being reported. All imaging signs are analyzed herewith. Imaging helps in the prediction of EHBA and also helps in early postnatal surgical referral which in turn improves the results of Kasai′s portoenterostomy.

  2. Right ventricular hypoplasia syndrome. Atresia of the right atrioventricular ostium (X-ray examination)

    International Nuclear Information System (INIS)

    Golonzko, R.R.; Chernova, M.P.; Berishvili, I.I.; Bulchinskij, S.A.

    1982-01-01

    Data on routine radiologic examination of 51 patients aged 3 days to 3 years with the right atrio-ventricular ostium atresia were presented. The evidence obtained was compared with angiographic and, in part of cases, with morphometric findings. All patients were divided in two groups, depending on pulmonary circulation functions. Characteristic radiologic features of the valvular defect were revealed: ''quadrangle'' heart configuration; increase of right auricle; ''sheared off'' profile of the right ventricle, absence of increase of leftauricle in children up to 1 year

  3. Pulmonary atresia with intact ventricular septum and agenesis of the ductus arteriosus in a pup

    International Nuclear Information System (INIS)

    Brown, D.J.; Patterson, D.F.

    1989-01-01

    A 7-week-old Wire Fox Terrier was admitted with pulmonary atresia with intact ventricular septum. The right ventricle and tricuspid valve were hypoplastic, and venous return to the right atrium reached the left side through an atrial septal defect. Oxygenation was via hyperplastic bronchial arteries. There was no evidence of the ductus arteriosus. Physical examination, plain and contrast radiography, and electrocardiography were performed. Clinical findings for this combination of defects were similar to those of more common defects (tetralogy of Fallot, patent ductus arteriosus

  4. Case report: Isolated unilateral pulmonary vein atresia diagnosed on 128-slice multidetector CT

    Directory of Open Access Journals (Sweden)

    Rashmi Dixit

    2011-01-01

    Full Text Available Unilateral pulmonary venous atresia is an uncommon entity that is generally believed to be congenital. Most patients present in infancy or childhood with recurrent chest infections or hemoptysis. Pulmonary angiography is usually used for definitive diagnosis. However, the current multislice CT scanners may obviate the need for pulmonary angiography. We report two cases diagnosed using 128-slice CT angiography. On the CT angiography images both these cases demonstrated absent pulmonary veins on the affected side, with a small pulmonary artery and prominent bronchial or other systemic arterial supply.

  5. Dysphagia among adult patients who underwent surgery for esophageal atresia at birth.

    Science.gov (United States)

    Huynh Trudeau, Valérie; Maynard, Stéphanie; Terzic, Tatjana; Soucy, Geneviève; Bouin, Mickeal

    2015-03-01

    Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and⁄or anatomical anomalies. To determine the motor and anatomical causes of dysphagia. A total of 41 adults, followed at the Esophageal Atresia Clinic at Hôpital Saint-Luc (Montreal, Quebec), were approached to particpate in the present prospective study. Evaluation was completed using upper endoscopy, manometry and barium swallow for the participants who consented. The medical charts of respondents were systematically reviewed from the neonatal period to 18 years of age to assess medical and surgical history. All 41 patients followed at the clinic consented and were included in the study. Dysphagia was present in 73% of patients. Esophagogastroduodenoscopy was performed in 32 patients: hiatal hernia was present in 62% (n=20); esophageal diverticulum in 13% (n=4); macroscopic Barrett esophagus in 31% (n=10); and esophagitis in 19% (n=6). Histological esophagitis was present in 20% and intestinal metaplasia in 10%. There were no cases of dysplagia or adenocarcinoma. Esophageal manometry was performed on 56% of the patients (n=23). Manometry revealed hypomotility in 100% of patients and included an insufficient number of peristaltic waves in 96%, nonpropagating peristalsis in 78% and low-wave amplitude in 95%. Complete aperistalsis was present in 78%. The lower esophageal sphincter was abnormal in 12 (52%) patients, with incomplete relaxation the most common anomaly. Of the 41 patients, 29 (71%) consented to a barium swallow, which was abnormal in 13 (45%). The anomalies found were short esophageal dilation in 28%, delay in esophageal emptying in 14%, diverticula in 14% and stenosis in 7

  6. Atresia biliar extra-hepática: métodos diagnósticos

    OpenAIRE

    Cauduro Sydney M.

    2003-01-01

    OBJETIVO: enfatizar a importância do diagnóstico precoce da atresia biliar extra-hepática e sua relação direta com o restabelecimento cirúrgico do fluxo biliar antes do segundo mês de vida, discutindo os diversos métodos complementares utilizados, objetivando selecionar os de maior evidência, evitando retardo diagnóstico e, conseqüentemente, piora do prognóstico. MÉTODO: pesquisa bibliográfica referente ao período de 1985 a 2001, no Medline e MdConsult, através das palavras-chaves: colestase ...

  7. Should patients with esophageal atresia be submitted to esophageal substitution before they start walking?

    Science.gov (United States)

    Tannuri, U; Tannuri, A C A

    2011-01-01

    Esophagocoloplasty and gastric transposition are two major methods for esophageal substitution in children with esophageal atresia, and there is broad agreement that these operations should not be performed before the children start walking. However, there are some reported advantages of performing such operations in the first months of life or in the neonatal period. In this study, we compared our experience with esophageal substitution procedures performed in walking children with esophageal atresia, with the outcomes of children who had the operation before the third month of life reported in the literature. The purpose of this study was to establish if we have to wait until the children start walking before indicating the esophageal replacement procedure. From February 1978 to October 2009, 129 children with esophageal atresia underwent esophageal replacement in our hospital (99 colonic interpositions and 30 gastric transpositions). The records of these patients were reviewed for data regarding demographics, complications (leaks, graft failures, strictures, and graft torsion), and mortality and compared with those reported in the two main articles on esophageal replacement in the neonatal period or in patients less than 3 months of age. The main complication of our casuistic was cervical anastomosis leakage, which sealed spontaneously in all except in four patients. One patient of the esophagocoloplasty group developed graft necrosis and three patients in the gastric transposition group had gastric outlet obstruction, secondary to axial torsion of the stomach placed in the retrosternal space. The long-term outcome of the patients in both groups was considered good to excellent in terms of normal weight gain, absence of dysphagia, and other gastrointestinal symptoms. The comparisons of the main complications and mortality rates in walking children with esophageal substitutions performed in the first months of life showed that the incidences of cervical

  8. Sonographic diagnosis of fetal intestinal volvulus with ileal atresia: a case report.

    Science.gov (United States)

    Yu, Wang; Ailu, Cai; Bing, Wang

    2013-05-01

    Fetal intestinal volvulus is a rare life-threatening condition usually manifesting after birth with most cases being associated with intestinal malrotation. It appears on prenatal sonography (US) as a twisting of the bowel loops around the mesenteric artery, leading to mechanical obstruction and ischemic necrosis of the bowel. We report a case of intrauterine intestinal volvulus with ileal atresia, suspected when US revealed a typical "whirlpool" sign at 37 weeks' gestation, with a segment of markedly distended bowel loops and small amount of fetal ascites. Copyright © 2012 Wiley Periodicals, Inc.

  9. Successful pregnancy after uterovaginal anastomosis in patients with congenital atresia of cervix uteri.

    Science.gov (United States)

    Prorocic, M; Vasiljevic, M; Tasic, L; Brankovic, S

    2012-01-01

    We present a case of successful pregnancy after effective uterovaginal anastomosis in a 26-years-old patient with congenital atresia of the cervix uteri. She spontaneously achieved pregnancy after four years of uterovaginal anastomosis. Gestation was at the eighth lunar month and the delivery was done by cesarean section due to rapidly progressing fetal asphyxia. The patient gave birth to a live healthy male, weighing 1,950 g, with an Apgar score of 5 and 8 at 1 and 5 min, respectively. The postoperative course was uneventful, and leakage of lochia was normal.

  10. Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys.

    Science.gov (United States)

    Chadha, Rajiv; Puri, Manju; Saxena, Rahul; Agarwala, Surendrakumar; Puri, Archana; Choudhury, Subhasis Roy

    2013-04-01

    This report describes a girl with congenital pouch colon (CPC), uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract.

  11. Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida Sacrococcygeal agenesis association and anal atresia in mixed breed cats

    Directory of Open Access Journals (Sweden)

    Felipe Purcell de Araújo

    2009-09-01

    Full Text Available O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

  12. Outcomes in patients undergoing robotic reconstructive uterovaginal anastomosis of congenital cervical and vaginal atresia.

    Science.gov (United States)

    Zhang, Ying; Chen, Yisong; Hua, Keqin

    2017-09-01

    To introduce our experience of robotic surgery of reconstructive uterovaginal anastomosis and operative outcomes in congenital cervical and vaginal atresia patients. Clinical observation and follow-up of four patients with congenital cervical and vaginal atresia who underwent robotic reconstruction of cervix and vagina by SIS (small intestinal submucosa, SIS) graft. Average patient age was 13.8 ± 2.2. Patients complained of severe periodic abdominal pain. Diagnosis was made according to clinical characteristics, physical examination, MRI and classified by ESHRE/ESGE system. All patients underwent reconstruction of cervix and vagina by uterovaginal anastomosis by SIS graft. Average operation time was 232.5 ± 89.2 min, average blood loss was 225.0 ± 95.7 mL. After surgery, all patients have regular menstruation without pain. Average follow up was 12 months, average vagina length was 8.9 ± 0.3 cm, average vagina width was 2.9 ± 0.1 cm. Robotic assisted reconstruction of cervix and vagina is feasible from our experience, enlarged cases and additional studies are required. © 2017 The Authors The International Journal of Medical Robotics and Computer Assisted Surgery Published by John Wiley & Sons Ltd.

  13. Application of RI hepatogram to evaluate liver cirrhosis in biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Ogawa, Tomio; Suruga, Keijiro; Miyano, Takeshi; Arai, Takeo; Shimomura, Hiroshi; Nagase, Katsuya; Iida, Susumu; Arakawa, Yoshiya

    1985-01-01

    RI hepatgram with sup(99m)TcO/sub 4/ was developed for the evaluation of the changes in hepatic circulation in liver cirrhosis in 41 cases of pediatric liver diseases including 25 cases of biliary atresia. After bolus intravenous injection of 1-5 mCi of sup(99m)TcO/sub 4/ radioactive count over the abdomen was measured by a scinticamera for ten minutes. Time activity curve of a ROI on the liver right lobe was drawn for ten minutes. The count reached to plateau about one minute after injection and gradually decreased thereafter. The ratio of the counts of eight minutes to the plateau was calculated. It ranged from 49% to 98%, and this ratio correlated well with the physical and laboratory findings of liver cirrhosis. There is a significant correlation between the ratio and liver fibrosis. Most of the cases (4/5) that showed over 80% were accompanied by gastrointestinal bleeding due to portal hypertension. This method can be used for postoperative follow-up of liver cirrhosis of biliary atresia to predict G-I bleeding as it is easily performed, almost noninvasive and easily repeated. (author).

  14. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

    Directory of Open Access Journals (Sweden)

    Go Miyano

    2015-01-01

    Full Text Available We describe herein a case of unilateral pulmonary agenesis (PA with oesophageal atresia (EA/tracheoesophageal fistula (TEF that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  15. [The application of vibrant sound bridge in microtia whose reconstructive external auditory canal occurred atresia].

    Science.gov (United States)

    Zhao, Shouqin; Gong, Shusheng; Han, Demin; Chen, Shubin; Li, Yi; Ma, Xiaobo; Liu, Haihong

    2012-05-01

    To investigate the effect of vibrant sound bridge implantation in microtia whose reconstructive external auditory canal occurred atresia. Three cases (2 males and 1 female) of microtia had underwent hearing reconstruction operation (Include the external ear canal reconstructive surgery and tympanoplasty). The age ranged from 15 to 18 years and the average age was 17 years. All the 3 cases suffered from conductive hearing loss with the air-bone gap ranging from 51.6 to 65.0 dB HL and the average value being 56. 3 dB HL. All the 3 cases underwent vibrant sound bridge implantation, including the floating mass transducer implanted in the head of stapes in 2 cases and in the niche of round window in 1 case. The postoperative hearing level improved from 21.6 to 52.5 dB HL with an average of 32.2 dB HL. There were no complications such as vertigo, tinnitus and facial paralysis. Through vibrant sound bridge implantation, the hearing level of microtia whose reconstructive external auditory canal occurred atresia was improved effectively.

  16. Karyotype analysis of a male exhibiting Meckel's diverticulum and aural atresia

    Energy Technology Data Exchange (ETDEWEB)

    Frizzell, B.; Hicks, M.F. (David Lipscomb Univ., Nashville, TN (United States))

    Patau's Syndrome is caused by inheritance of an extra chromosome 13. It is characterized primarily by severe mental retardation, cleft palate, and retarded growth. Most fetuses expressing Patau's Syndrome spontaneously abort, and those that are born usually die before one year. Both Meckel's diverticulum and aural atresia are defects found in patients with Patau's at levels higher than those in the general population. An otherwise asymptomatic male expressing only Meckel's diverticulum and aural atresia has a female sibling whose son expressed Patau's syndrome. Twenty percent of patients with Patau's show a translocation of part of chromosome 13 to another D chromosome. If a translocation were the cause of the expression of Patau's in this family, it is possible that the normal male inherited a balanced translocation and the Patau's male received an unbalanced translocation. A karyotype analysis of the non-Patau's male was done to determine if such a translocation were present.

  17. Subdural hemorrhage: A unique case involving secondary vitamin K deficiency bleeding due to biliary atresia.

    Science.gov (United States)

    Miyao, Masashi; Abiru, Hitoshi; Ozeki, Munetaka; Kotani, Hirokazu; Tsuruyama, Tatsuaki; Kobayashi, Naho; Omae, Tadaki; Osamura, Toshio; Tamaki, Keiji

    2012-09-10

    Extrahepatic biliary atresia (EHBA) is a rare disease characterized by progressive and obliterative cholangiopathy in infants and is one of the major causes of secondary vitamin K deficiency bleeding (VKDB) due to cholestasis-induced fat malabsorption. Breast feeding increases the tendency of bleeding in EHBA patients because breast milk contains low amounts of vitamin K. A 2-month-old female infant unexpectedly died, with symptoms of vomiting and jaundice prior to death. She had been born by uncomplicated vaginal delivery and exhibited normal growth and development with breastfeeding. There was no history of trauma. She received vitamin K prophylaxis orally. In an emergency hospital, a CT scan showed a right intracranial hematoma and mass effect with midline shift to the left. In the postmortem examination, severe atresia was observed in the whole extrahepatic bile duct. Histologically, cholestasis, periductal fibrosis, and distorted bile ductules were noted. The gallbladder was not identified. A subdural hematoma and cerebellar tonsillar herniation were found; however, no traumatic injury in any part of the body was observed. Together, these findings suggest that the subdural hemorrhage was caused by secondary vitamin K deficiency resulting from a combination of cholestasis-induced fat malabsorption and breastfeeding. Subdural hemorrhage by secondary VKDB sometimes occurs even when vitamin K prophylaxis is continued. This case demonstrated that intrinsic factors, such as secondary VKDB (e.g., EHBA, neonatal hepatitis, chronic diarrhea), should also be considered in infant autopsy cases presenting with subdural hemorrhage. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  18. Congenital Left Circumflex Coronary Artery Atresia Detected by 64-Slice Computed Tomography: A Case Report

    Directory of Open Access Journals (Sweden)

    Chen-Yuan Liu

    2007-06-01

    Full Text Available A variety of coronary artery disorders, including intramyocardial coronary segments and coronary artery anomalies, can result in sudden cardiac death, especially in young adults. The detection of structural coronary artery abnormalities is important in the management of patients at risk of sudden cardiac death. Coronary artery anomalies occur in about 1% of the population. Congenital absence of left circumflex coronary artery (LCX is a very rare vascular anomaly, and few cases have been reported in the literature, with a frequency of only 0.003% in all patients who underwent coronary angiography. Although coronary catheterization is the gold standard for the evaluation of coronary arterial patency disease, noninvasive computed tomography (CT is considered the diagnostic method of choice for the detection and evaluation of coronary artery anomaly. Herein, we report the case of a 17-year-old girl who presented with exertional dyspnea and chest pain and who was studied at our emergency department with the final diagnosis of LCX atresia detected by 64-slice CT. She may be the first case of congenital LCX atresia proved by multislice CT.

  19. A two-center comparative study of gastric pull-up and jejunal interposition for long gap esophageal atresia

    NARCIS (Netherlands)

    Gallo, Gabriele; Zwaveling, S; Van Der Zee, David C.; Bax, Klaas N.; De Langen, Zacharias J.; Hulscher, Jan B F

    2015-01-01

    Purpose When restoration of the anatomical continuity in case of long gap esophageal atresia (LGEA) is not feasible, esophageal replacement surgery becomes mandatory. The aim of this paper is to critically compare the experience of two tertiary referral centers in The Netherlands performing either

  20. Adults with corrected oesophageal atresia: is oesophageal function associated with complaints and/or quality of life?

    NARCIS (Netherlands)

    Deurloo, J.A.; Klinkenberg, E.C.; Ekkelkamp, S.; Heij, H.A.; Aronson, D.C.

    2008-01-01

    The aim of this study was to evaluate oesophageal function after correction of oesophageal atresia in adults, and to investigate the association between complaints, oesophageal function and quality of life (QoL). Twenty-five adults were included who participated in previous follow-up studies, during

  1. Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016

    DEFF Research Database (Denmark)

    Pakarinen, Mikko P; Johansen, Lars Søndergaard; Svensson, Jan F

    2017-01-01

    therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >3 patients and diagnostic protocol without routine liver biopsy...

  2. Endoscopic Assessment of Children with Esophageal Atresia: Lack of Relationship of Esophagitis and Esophageal Metaplasia to Symptomatology

    Directory of Open Access Journals (Sweden)

    Julie Castilloux

    2010-01-01

    Full Text Available BACKGROUND: Late complications of esophageal atresia (EA, particularly esophagitis and Barrett’s esophagus, are increasingly being recognized. With the exception of patients with dysphagia associated with esophageal stricture, it is unknown whether patient symptomatology can predict endoscopic findings.

  3. Oesophageal atresia in premature infants: an analysis of morbidity and mortality over a period of 20 years

    NARCIS (Netherlands)

    Deurloo, J. A.; Smit, B. J.; Ekkelkamp, S.; Aronson, D. C.

    2004-01-01

    Aim: To determine the morbidity and mortality of premature infants born with oesophageal atresia (OA) and to evaluate historical changes in morbidity and mortality over time. Methods: Retrospective analysis of morbidity and mortality of all patients admitted for OA, with or without

  4. The Triangular Sign, a Useful Diagnostic Marker for Biliary Atresia: A Case Series of Three Irish Infants

    LENUS (Irish Health Repository)

    Smith, A

    2018-06-01

    The triangular cord (TC) sign is the appearance of a triangular shaped echogenic density visualised immediately cranial to the portal vein bifurcation on ultrasonographic examination. Several studies have reported that this ultrasonographic sign is a reliable and helpful marker in identifying Biliary Atresia (BA).

  5. Radiological Findings in a Case of Multiple Focal Nodular Hyperplasia Associated with Portal Vein Atresia and Portopulmonary Hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Lee, In Joon; Jeong, Sook Hyang; Choi, Jin Woo; Park, Hee Sun; Lee, Kyoung Ho; Kim, Hae Ryoung [Seoul National University Bundang Hospital, Seoul (Korea, Republic of)

    2008-08-15

    We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities

  6. Combined atresia of one left-sided and one right-sided cardiac valve in a premature newborn.

    Science.gov (United States)

    Roberts, William C; Sing, Alan C; Guileyardo, Joseph M

    2017-10-01

    Described herein is the heart of a 2-day-old newborn, the product of a 25-week gestation, with atresia of two cardiac valves, one on the right side and one on the left side, apparently a previously undescribed entity.

  7. Sphingosine-1-phosphate and ceramide are associated with health and atresia of bovine ovarian antral follicles.

    Science.gov (United States)

    Hernández-Coronado, C G; Guzmán, A; Espinosa-Cervantes, R; Romano, M C; Verde-Calvo, J R; Rosales-Torres, A M

    2015-02-01

    The follicle destiny towards ovulation or atresia is multi-factorial in nature and involves outcries, paracrine and endocrine factors that promote cell proliferation and survival (development) or unchain apoptosis as part of the atresia process. In several types of cells, sphingosine-1-phospate (S1P) promotes cellular proliferation and survival, whereas ceramide (CER) triggers cell death, and the S1P/CER ratio may determine the fate of the cell. The aim of present study was to quantify S1P and CER concentrations and their ratio in bovine antral follicles of 8 to 17 mm classified as healthy and atretic antral follicles. Follicles were dissected from cow ovaries collected from a local abattoir. The theca cell layer, the granulosa cells and follicular fluid were separated, and 17β-estradiol (E2) and progesterone (P4) concentrations were measured in the follicular fluid by radioimmunoassay. Based on the E2/P4 ratio, the follicles were classified as healthy (2.2±0.3) or atretic (0.2±0.3). In both follicular compartments (granulosa and theca cell layer), sphingolipids were extracted and S1P and CER concentrations were quantified by HPLC (XTerra RP18; 5 µm, 3.0×150 mm column). Results showed that in both follicular compartments, S1P concentrations were higher in healthy antral follicles than in atretic antral follicles (P<0.05). The concentration of CER in the granulosa cells was higher in atretic antral follicles than in healthy antral follicles, but no differences were observed in the theca cell layer. The S1P/CER ratio in both follicular compartments was also higher in healthy antral follicles. Interestingly, in these follicles, there was a 45-fold greater concentration of S1P than CER in the granulosa cells (P<0.05), whereas in the theca cell layer, S1P had only a 14-fold greater concentration than CER when compared with atretic antral follicles. These results suggest that S1P plays a role in follicle health, increasing cellular proliferation and survival. In

  8. Symptomatic paroxysmal atrial fibrillation in a patient with unilateral pulmonary vein atresia

    Directory of Open Access Journals (Sweden)

    Sharmila Sehli

    2015-01-01

    Full Text Available A 52-year-old man with symptomatic paroxysmal atrial fibrillation was offered an atrial fibrillation (AF ablation procedure. His echocardiogram indicated that he had no structural heart disease. A cardiac computed tomographic (CT scan showed enlargement of the right pulmonary veins, absence of the left pulmonary veins, a prominent left atrial appendage, and a hypoplastic left lung. Cardiac CT with an electroanatomic mapping system confirmed a prominent left atrial appendage and the absence of the left pulmonary veins. Due to the limited number of patients with this condition, information about ablation remains very limited, and his ablation was deferred. Unilateral pulmonary vein atresia is a rare condition in adults which results from failure of incorporation of the common pulmonary vein into the left atrium. This case demonstrates the clinical importance of preprocedural imaging prior to AF ablation.

  9. External auditory canal atresia of probable congenital origin in a dog.

    Science.gov (United States)

    Schmidt, K; Piaia, T; Bertolini, G; De Lorenzi, D

    2007-04-01

    A nine-month-old Labrador retriever was referred to the Clinica Veterinaria Privata San Marco because of frequent headshaking and downward turning of the right ear. Clinical examination revealed that there was no external acoustic meatus in the right ear. Computed tomography confirmed that the vertical part of the right auditory canal ended blindly, providing a diagnosis of external auditory canal atresia. Cytological examination and culture of fluid from the canal and the bulla revealed only aseptic cerumen; for this reason, it was assumed that the dog was probably affected by a congenital developmental deformity of the external auditory canal. Reconstructive surgery was performed using a "pull-through" technique. Four months after surgery the cosmetic and functional results were satisfactory.

  10. IL-6, TNF-α, IL-10, and nutritional status in pediatric patients with biliary atresia.

    Science.gov (United States)

    Wilasco, Maria Ines de Albuquerque; Uribe-Cruz, Carolina; Santetti, Daniele; Fries, Gabriel Rodrigo; Dornelles, Cristina Toscani Leal; Silveira, Themis Reverbel da

    The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child-Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry. IL-6, TNF-α, and IL-10 were significantly higher in the cirrhosis group when compared with the control group (2.4 vs. 0.24 (patresia, IL-6 could be used as a possible supporting biomarker of deficient nutritional status and elevated IL-10 levels could be used as a possible early-stage supporting biomarker of deteriorating nutritional status. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  11. Radiological aspects of bronchial atresia: report of three cases and review of the literature

    International Nuclear Information System (INIS)

    Neu, Alexandre da Silva; Menezes, Roger Eliandro; Maciel, Antonio Carlos; Castro, Rogerio Fernandes Peixoto de

    2003-01-01

    Bronchial atresia is a rare congenital abnormality that usually shows a juxta-hilar mass with distal radiolucency on conventional x-ray films. Most patients are young and have no symptoms. Computed tomography usually confirms the diagnosis, allowing conservative management of the asymptomatic cases. The authors report three cases of bronchial obstruction. One of the patients was of particular importance and interest, a 45-year-old female patient with symptoms of respiratory infection, due to the association with a bulky bronchocele. The diagnosis was made by analyzing the findings of conventional x-ray films and computed tomography, and the comparison with previous conventional x-ray films and linear tomography, which had already demonstrated abnormalities. A literature review on the clinical and radiological findings of this pulmonary malformation is presented. (author)

  12. Repair of esophageal atresia with proximal fistula using endoscopic magnetic compression anastomosis (magnamosis) after staged lengthening.

    Science.gov (United States)

    Dorman, Robert M; Vali, Kaveh; Harmon, Carroll M; Zaritzky, Mario; Bass, Kathryn D

    2016-05-01

    We describe the treatment of a patient with long-gap esophageal atresia with an upper pouch fistula, mircogastria and minimal distal esophageal remnant. After 4.5 months of feeding via gastrostomy, a proximal fistula was identified by bronchoscopy and a thoracoscopic modified Foker procedure was performed reducing the gap from approximately 7-5 cm over 2 weeks of traction. A second stage to ligate the fistula and suture approximate the proximal and distal esophagus resulted in a gap of 1.5 cm. IRB and FDA approval was then obtained for endoscopic placement of 10-French catheter mounted magnets in the proximal and distal pouches promoting a magnetic compression anastomosis (magnamosis). Magnetic coupling occurred at 4 days and after magnet removal at 13 days an esophagram demonstrated a 10 French channel without leak. Serial endoscopic balloon dilation has allowed drainage of swallowed secretions as the baby learns bottling behavior at home.

  13. Use of a Palmaz stent for tracheomalacia: case report of an infant with esophageal atresia.

    Science.gov (United States)

    Tazuke, Y; Kawahara, H; Yagi, M; Yoneda, A; Soh, H; Maeda, K; Yamamoto, T; Imura, K

    1999-08-01

    A male infant with congenital cardiac anomalies and esophageal atresia with tracheoesophageal fistula (EA-TEF) showed intractable respiratory symptoms after delayed primary repair of EA-TEF. Computed tomography demonstrated that the trachea was compressed by the enlarged aorta. Artificial ventilation was necessary even after aortopexy performed at 2 months of age. At 140 days of age, an expandable metallic stent (Palmaz stent) was inserted through a rigid bronchoscope into the trachea underfluoroscopic control. His respiratory status improved dramatically, and he was extubated in 18 hours. Although the follow-up period has been 9 months, the short-term result is satisfactory. The expandable metallic stent placement should be considered in patients with EA-TEF who show intractable respiratory symptoms caused by tracheomalacia.

  14. “Parenteral Nutrition Supplementation in Biliary Atresia Patients Listed for Liver Transplantation”

    Science.gov (United States)

    Sullivan, Jillian S; Sundaram, Shikha S; Pan, Zhaoxing; Sokol, Ronald J

    2011-01-01

    Objective To determine the impact of parenteral nutrition (PN) on outcomes in biliary atresia (BA) patients listed for liver transplantation (LTx). Study Design We retrospectively reviewed charts of all BA patients from 1990 through 2010 at our institution, s/p hepatoportoenterostomy, ≤ 36 months old, and listed for LTx. Initiation of PN was based on clinical indications. Results 25 PN and 22 non-PN subjects (74% female) were studied. Median PN initiation age was 7.7 months, mean duration 86 days, and mean PN energy supplied 77 kcal/kg/day. Prior to PN, triceps skinfold thickness (TSF) and mid-arm circumference (MAC) Z-scores were decreasing. After PN, TSF (p=0.003) and MAC (pnutritional status in malnourished BA patients awaiting LTx, which is associated with post-LTx outcomes comparable to those not requiring PN. PMID:21987426

  15. Incidental Finding of Inferior Vena Cava Atresia Presenting with Deep Venous Thrombosis following Physical Exertion

    Directory of Open Access Journals (Sweden)

    Shalini Koppisetty

    2015-01-01

    Full Text Available Inferior vena cava atresia (IVCA is a rare but well described vascular anomaly. It is a rare risk factor for deep venous thrombosis (DVT, found in approximately 5% of cases of unprovoked lower extremity (LE DVT in patients <30 years of age. Affected population is in the early thirties, predominantly male, often with a history of major physical exertion and presents with extensive or bilateral DVTs. Patients with IVC anomalies usually develop compensatory circulation through the collateral veins with enlarged azygous/hemizygous veins. Despite the compensatory circulation, the venous drainage of the lower limbs is often insufficient leading to venous stasis and thrombosis. We describe a case of extensive and bilateral deep venous thrombosis following physical exertion in a thirty-six-year-old male patient with incidental finding of IVCA on imaging.

  16. Atresia coli in a Japanese black calf diagnosed by a barium sulphate enema contrast radiograph in the standing position: a case report

    International Nuclear Information System (INIS)

    Abouelnasr, K.; Ishii, M.; Inokuma, H.; Kobayashi, Y.; Lee, K.; Yamada, K.

    2012-01-01

    A three day-old Japanese black calf was admitted with a history of abdominal distension and absence of defecation. Dilated loops of the small intestine and hypoplasia of the colon and rectum was observed upon a contrast radiographic examination in the standing position. At necropsy atresia coli with undeveloped rectum and patent anal opening was found. We conclude that a contrast radiograph in the standing position is useful for diagnosing atresia coli in such cases

  17. Surgical Outcomes in Esophageal Atresia and Tracheoesophageal Fistula: A Comparison between Primary and Delayed Repair

    Directory of Open Access Journals (Sweden)

    H Davari

    2006-01-01

    Full Text Available Background: The purpose of this study was to investigate outcomes of surgical repair of esophageal atresia (EA or tracheoesophageal fistula (TEF in newborns, with respect to incidence of death and other complications in early or late operations. Methods: Charts of all 80 infants with EA/TEF, operated in Alzahra hospital (A tertiary hospital of Isfahan University of Medical Sciences from 2002 to 2004 were reviewed. Patients were designed in two groups as, primary and delayed repair groups. Patients demographics, frequency of associated anomalies, and details of management and outcomes were studied. Results: There were 48 male and 32 female patients with a frequency of 28(35% preterm infant and mean birth weight of 2473±595 g. Overall survival rate was 71.2%. Mortality rate in delayed repair group was significantly higher than the other one (22.5% vs. 6.3% but with matching, according to full term/preterm proportion, the significant differences were failed. Female sex and being preterm were the most powerful predictors of death (nearly odds ratio=7 for both. Conclusion: in this study mortality and complications rates are higher in delayed repair than early one, although our data proposed that in absence of sever life threatening anomalies the most important factor for death is gestational age and female sex, and primary repair is opposed to it. Although mortality rate and complications are equal in two strategies, with matching cases for being preterm, but primary repair stays the better choice due to economic considerations. Keywords: tracheoesophageal fistula, esophageal atresia, delayed repair, primary repair, outcome

  18. 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.)

  19. Silencing of the rotavirus NSP4 protein decreases the incidence of biliary atresia in murine model.

    Directory of Open Access Journals (Sweden)

    Jiexiong Feng

    Full Text Available Biliary atresia is a common disease in neonates which causes obstructive jaundice and progressive hepatic fibrosis. Our previous studies indicate that rotavirus infection is an initiator in the pathogenesis of experimental biliary atresia (BA through the induction of increased nuclear factor-kappaB and abnormal activation of the osteopontin inflammation pathway. In the setting of rotavirus infection, rotavirus nonstructural protein 4 (NSP4 serves as an important immunogen, viral protein 7 (VP7 is necessary in rotavirus maturity and viral protein 4 (VP4 is a virulence determiner. The purpose of the current study is to clarify the roles of NSP4, VP7 and VP4 in the pathogenesis of experimental BA. Primary cultured extrahepatic biliary epithelia were infected with Rotavirus (mmu18006. Small interfering RNA targeting NSP4, VP7 or VP4 was transfected before rotavirus infection both in vitro and in vivo. We analyzed the incidence of BA, morphological change, morphogenesis of viral particles and viral mRNA and protein expression. The in vitro experiments showed NSP4 silencing decreased the levels of VP7 and VP4, reduced viral particles and decreased cytopathic effect. NSP4-positive cells had strongly positive expression of integrin subunit α2. Silencing of VP7 or VP4 partially decreased epithelial injury. Animal experiments indicated after NSP4 silencing, mouse pups had lower incidence of BA than after VP7 or VP4 silencing. However, 33.3% of VP4-silenced pups (N = 6 suffered BA and 50% of pups (N = 6 suffered biliary injury after VP7 silencing. Hepatic injury was decreased after NSP4 or VP4 silencing. Neither VP4 nor VP7 were detected in the biliary ducts after NSP4. All together, NSP4 silencing down-regulates VP7 and VP4, resulting in decreased incidence of BA.

  20. Pulmonary Atresia

    Science.gov (United States)

    ... Rounds Seminar Series & Daily Conferences Fellowships and Residencies School of Perfusion Technology Education Resources Library & Learning Resource Center CME Resources THI Journal THI Cardiac Society Register for the Cardiac Society ...

  1. Biliary Atresia

    Science.gov (United States)

    ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ...

  2. Prevention of vitamin K deficiency bleeding in breastfed infants: lessons from the Dutch and Danish biliary atresia registries

    DEFF Research Database (Denmark)

    Hasselt, P.M. van; Koning, T.J. de; Vries, E. de

    2008-01-01

    OBJECTIVE: Newborns routinely receive vitamin K to prevent vitamin K deficiency bleeding. The efficacy of oral vitamin K administration may be compromised in infants with unrecognized cholestasis. We aimed to compare the risk of vitamin K deficiency bleeding under different prophylactic regimens...... in infants with biliary atresia. PATIENTS AND METHODS: From Dutch and Danish national biliary atresia registries, we retrieved infants who were either breastfed and received 1 mg of oral vitamin K at birth followed by 25 microg of daily oral vitamin K prophylaxis (Netherlands, 1991-2003), 2 mg of oral...... vitamin K at birth followed by 1 mg of weekly oral prophylaxis (Denmark, 1994 to May 2000), or 2 mg of intramuscular prophylaxis at birth (Denmark, June 2000-2005) or were fed by formula. We determined the absolute and relative risk of severe vitamin K deficiency and vitamin K deficiency bleeding...

  3. Duodenal atresia in an infant with triple-X syndrome: a new associated malformation in 47,XXX.

    Science.gov (United States)

    Rolle, Udo; Linse, Barbara; Glasow, Simone; Sandig, Klaus Rainer; Richter, Thomas; Till, Holger

    2007-08-01

    An association between the triple-X syndrome (47,XXX) and gastrointestinal malformations is extremely rare. Most 47,XXX patients present with a normal phenotype, but genitourinary malformations have been described. We report a case of a child with 47,XXX and duodenal atresia. Antenatal ultrasound scan showed a dilated fetal stomach and upper part of the duodenum (double bubble phenomenon) at 31 weeks of gestation in a 31-year-old woman with polyhydramnion. The amniotic fluid karyotype showed 47,XXX. After a scheduled delivery, duodenal atresia was confirmed and treated with duodeno-duodenostomy. The possible association of gastrointestinal and genitourinary tract anomalies requires a detailed postnatal clinical investigation and ultrasonographic examination of the abdomen, retroperitoneum, and pelvis on all triple-X syndrome patients. 2007 Wiley-Liss, Inc.

  4. Gaseous distention of the hypopharynx and cervical esophagus with nasal CPAP: a mimicker of pharyngeal perforation and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Walor, David; Berdon, Walter; Holt, Peter D.; Fox, Matthew [Columbia University Medical Center, Department of Radiology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States); Anderson, Nicole [Columbia University Medical Center, Department of Neonatology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States)

    2005-12-01

    Nasal continuous positive airway pressure (CPAP) has been used since 1975 as the initial treatment for respiratory distress syndrome (RDS) in very premature infants. Gaseous distention of the abdomen (CPAP belly) is a common secondary effect of CPAP. Gaseous distention of the hypopharynx is also common. To determine the incidence of hypopharyngeal distention in infants on CPAP. We performed a retrospective review of the chest radiographs of 57 premature infants treated with CPAP during a 4-week period to find the presence and degree of hypopharyngeal distention. Of the 57 radiographs, 14 (25%) revealed gaseous distention of the hypopharynx and/or cervical esophagus. On occasion, this raised concern for pharyngeal perforation or esophageal atresia. Awareness that CPAP-related hypopharyngeal distention is common should help radiologists avoid erroneous consideration of esophageal atresia or hypopharyngeal perforation. (orig.)

  5. Gaseous distention of the hypopharynx and cervical esophagus with nasal CPAP: a mimicker of pharyngeal perforation and esophageal atresia

    International Nuclear Information System (INIS)

    Walor, David; Berdon, Walter; Holt, Peter D.; Fox, Matthew; Anderson, Nicole

    2005-01-01

    Nasal continuous positive airway pressure (CPAP) has been used since 1975 as the initial treatment for respiratory distress syndrome (RDS) in very premature infants. Gaseous distention of the abdomen (CPAP belly) is a common secondary effect of CPAP. Gaseous distention of the hypopharynx is also common. To determine the incidence of hypopharyngeal distention in infants on CPAP. We performed a retrospective review of the chest radiographs of 57 premature infants treated with CPAP during a 4-week period to find the presence and degree of hypopharyngeal distention. Of the 57 radiographs, 14 (25%) revealed gaseous distention of the hypopharynx and/or cervical esophagus. On occasion, this raised concern for pharyngeal perforation or esophageal atresia. Awareness that CPAP-related hypopharyngeal distention is common should help radiologists avoid erroneous consideration of esophageal atresia or hypopharyngeal perforation. (orig.)

  6. Di(2-ethylhexyl) phthalate inhibits antral follicle growth, induces atresia, and inhibits steroid hormone production in cultured mouse antral follicles

    Energy Technology Data Exchange (ETDEWEB)

    Hannon, Patrick R., E-mail: phannon2@illinois.edu; Brannick, Katherine E., E-mail: kbran@illinois.edu; Wang, Wei, E-mail: Wei.Wang2@covance.com; Gupta, Rupesh K., E-mail: drrupesh@yahoo.com; Flaws, Jodi A., E-mail: jflaws@illinois.edu

    2015-04-01

    Di(2-ethylhexyl) phthalate (DEHP) is a ubiquitous environmental toxicant found in consumer products that causes ovarian toxicity. Antral follicles are the functional ovarian units and must undergo growth, survival from atresia, and proper regulation of steroidogenesis to ovulate and produce hormones. Previous studies have determined that DEHP inhibits antral follicle growth and decreases estradiol levels in vitro; however, the mechanism by which DEHP elicits these effects is unknown. The present study tested the hypothesis that DEHP directly alters regulators of the cell cycle, apoptosis, and steroidogenesis to inhibit antral follicle functionality. Antral follicles from adult CD-1 mice were cultured with vehicle control or DEHP (1–100 μg/ml) for 24–96 h to establish the temporal effects of DEHP on the follicle. Following 24–96 h of culture, antral follicles were subjected to gene expression analysis, and media were subjected to measurements of hormone levels. DEHP increased the mRNA levels of cyclin D2, cyclin dependent kinase 4, cyclin E1, cyclin A2, and cyclin B1 and decreased the levels of cyclin-dependent kinase inhibitor 1A prior to growth inhibition. Additionally, DEHP increased the mRNA levels of BCL2-associated agonist of cell death, BCL2-associated X protein, BCL2-related ovarian killer protein, B-cell leukemia/lymphoma 2, and Bcl2-like 10, leading to an increase in atresia. Further, DEHP decreased the levels of progesterone, androstenedione, and testosterone prior to the decrease in estradiol levels, with decreased mRNA levels of side-chain cleavage, 17α-hydroxylase-17,20-desmolase, 17β-hydroxysteroid dehydrogenase, and aromatase. Collectively, DEHP directly alters antral follicle functionality by inhibiting growth, inducing atresia, and inhibiting steroidogenesis. - Highlights: • DEHP inhibits antral follicle growth by dysregulating cell cycle regulators. • DEHP induces antral follicle atresia by dysregulating apoptosis regulators. • DEHP

  7. Large right ventricular sinusoids in an infant with aorta-left ventricular tunnel and proximal right coronary artery atresia.

    Science.gov (United States)

    Chen, Peter C; Spinner, Joseph A; Heinle, Jeffrey S

    2018-07-01

    We report a 1-month-old infant diagnosed with an aorta-left ventricular tunnel, ventricular septal defect, and right coronary atresia with right ventricular sinusoids. The patient's anatomy and physiology did not indicate right-ventricular-dependent coronary circulation, and therefore right ventricular decompression could be performed without compromising coronary perfusion during surgical correction. A detailed understanding of the coronary anatomy is critical in managing this defect when coronary anomalies are present.

  8. Efficacy of Lower-Extremity Venous Thrombolysis in the Setting of Congenital Absence or Atresia of the Inferior Vena Cava

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    Ganguli, Suvranu; Kalva, Sanjeeva; Oklu, Rahmi; Walker, T. Gregory; Datta, Neil; Grabowski, Eric F.; Wicky, Stephan

    2012-01-01

    Purpose: A rare but described risk factor for deep venous thrombosis (DVT), predominately in the young, is congenital agenesis or atresia of the inferior vena cava (IVC). The optimal management for DVT in this subset of patients is unknown. We evaluated the efficacy of pharmacomechanical catheter-directed thrombolysis (PCDT) followed by systemic anticoagulation in the treatment of acute lower-extremity DVT in the setting of congenital IVC agenesis or atresia. Materials and Methods: Between November of 2005 and May of 2010, six patients (three women [average age 21 years]) were referred to our department with acute lower-extremity DVT and subsequently found to have IVC agenesis or atresia on magnetic resonance imaging. A standardized technique for PCDT (the Angiojet Rheolytic Thrombectomy System followed by the EKOS Microsonic Accelerated Thrombolysis System) was used for all subjects. Successful thrombolysis was followed by systemic heparinization with transition to Coumadin or low molecular-weight heparin and compression stockings. Subjects were followed-up at 1, 3, and then every 6 months after the procedure with clinical assessment and bilateral lower-extremity venous ultrasound. Results: All PCDT procedures were technically successful. No venous stenting or angioplasty was performed. The average thrombolysis time was 28.6 h (range 12–72). Two patients experienced heparin-induced thrombocytopenia, and one patient developed a self-limited knee hemarthrosis, No patients were lost to follow-up. The average length of follow-up was 25.8 ± 20.2 months (range 3.8–54.8). No incidence of recurrent DVT was identified. There were no manifestations of postthrombotic syndrome. Conclusions: PCDT followed by systemic anticoagulation and the use of compression stockings appears to be safe and effective in relatively long-term follow-up treatment of patients who present with acute DVT and IVC agenesis or atresia.

  9. Di(2-ethylhexyl) phthalate inhibits antral follicle growth, induces atresia, and inhibits steroid hormone production in cultured mouse antral follicles

    International Nuclear Information System (INIS)

    Hannon, Patrick R.; Brannick, Katherine E.; Wang, Wei; Gupta, Rupesh K.; Flaws, Jodi A.

    2015-01-01

    Di(2-ethylhexyl) phthalate (DEHP) is a ubiquitous environmental toxicant found in consumer products that causes ovarian toxicity. Antral follicles are the functional ovarian units and must undergo growth, survival from atresia, and proper regulation of steroidogenesis to ovulate and produce hormones. Previous studies have determined that DEHP inhibits antral follicle growth and decreases estradiol levels in vitro; however, the mechanism by which DEHP elicits these effects is unknown. The present study tested the hypothesis that DEHP directly alters regulators of the cell cycle, apoptosis, and steroidogenesis to inhibit antral follicle functionality. Antral follicles from adult CD-1 mice were cultured with vehicle control or DEHP (1–100 μg/ml) for 24–96 h to establish the temporal effects of DEHP on the follicle. Following 24–96 h of culture, antral follicles were subjected to gene expression analysis, and media were subjected to measurements of hormone levels. DEHP increased the mRNA levels of cyclin D2, cyclin dependent kinase 4, cyclin E1, cyclin A2, and cyclin B1 and decreased the levels of cyclin-dependent kinase inhibitor 1A prior to growth inhibition. Additionally, DEHP increased the mRNA levels of BCL2-associated agonist of cell death, BCL2-associated X protein, BCL2-related ovarian killer protein, B-cell leukemia/lymphoma 2, and Bcl2-like 10, leading to an increase in atresia. Further, DEHP decreased the levels of progesterone, androstenedione, and testosterone prior to the decrease in estradiol levels, with decreased mRNA levels of side-chain cleavage, 17α-hydroxylase-17,20-desmolase, 17β-hydroxysteroid dehydrogenase, and aromatase. Collectively, DEHP directly alters antral follicle functionality by inhibiting growth, inducing atresia, and inhibiting steroidogenesis. - Highlights: • DEHP inhibits antral follicle growth by dysregulating cell cycle regulators. • DEHP induces antral follicle atresia by dysregulating apoptosis regulators. • DEHP

  10. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

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    Holmqvist, C.; Hochbergs, P.; Bjoerkhem, G.; Brockstedt, S.; Laurin, S.

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5±4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD

  11. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

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    Holmqvist, C.; Hochbergs, P. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology; Bjoerkhem, G. [Univ. Hospital, Lund (Sweden). Dept of Paediatrics; Brockstedt, S.; Laurin, S. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5{+-}4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.

  12. 2q24 deletion in a 9-month old girl with anal atresia, hearing impairment, and hypotonia.

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    Zhao, Peiwei; Mao, Bing; Cai, Xiaonan; Jiang, Jun; Liu, Zhisheng; Lin, Jun; He, Xuelian

    2018-06-01

    Deletion of 2q24.2 is a rare cytogenetic aberration in patients, exhibiting heterogeneous clinical features, and common phenotypes included developmental delay, intellectual disability, hypotonia, and mild dysmorphic features. Hearing impairment and anal atresia are rarely described. Here we described a 9-month-old female patient with hypotonia in all four limbs, developmental delay, and intellectual disability. In addition, congenital anal atresia was diagnosed and treated after birth, and hearing impairment was found in right ear. Single nucleotide polymorphisms (SNP) array detected a 5.2 Mb deletion on 2q24.2q24.3, including 19 genes (ITGB6; TBR1; SLC4A10; KCNH7 SCN3A; SCN2A et al.). Among these genes, it is affirmative that TBR1 is a causative gene for intellectual disability; however, the pathogenic genes of other phenotypes remain unclear. We briefly review the knowledge of genes likely involved in these clinical features, including hearing impairment, anal atresia, and developmental delay. Copyright © 2018 Elsevier B.V. All rights reserved.

  13. Three-dimensional assessment of the temporal bone and mandible deformations in patients with congenital aural atresia.

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    Fu, Yaoyao; Li, Chenlong; Dai, Peidong; Zhang, Tianyu

    2017-10-01

    To investigate the deformations of temporal bone and mandible combined with congenital aural atresia. A total of 158 patients with congenital aural atresia were included in the study. The raw CT data of the temporal bone was imported into MIMICS v 12 and threshold dissection, region growing and three-dimensional (3D) calculation were used to calculate 3D models. The 3D characteristics of the temporal bone and upper part of mandible were assessed. The tympanic part of the temporal bone was all undeveloped. Of all the patients included, 14 patients were found to have severe maxillofacial malformations. Among them, 2 cases have floating arch, 4 cases have interrupted arch, 5 cases have mandibular processes hypoplasia and 3 cases have interrupted arch combined with severe maxillary malformation. Ten of the 14 patients were suffered from dysplasia of the mastoid part of the temporal bone as well. Maxillofacial malformations may sometimes coexist with congenital aural atresia. Otolaryngologists should not neglect the coexisted maxillofacial malformations and give timely referral to maxillofacial surgeons. Copyright © 2017. Published by Elsevier B.V.

  14. Surgical treatment of choanal atresia with transnasal endoscopic approach with stentless single side-hinged flap technique: 5 year retrospective analysis

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    Carmelo Saraniti

    Full Text Available Abstract Introduction: Choanal atresia is a rare congenital malformation of the nasal cavity characterized by the complete obliteration of the posterior choanae. In 67% of cases choanal atresia is unilateral, affecting mainly (71% the right nasal cavity. In contrast to the unilateral form, bilateral choanal atresia is a life-threatening condition often associated with respiratory distress with feeding and intermittent cyanosis exacerbated by crying. Surgical treatment remains the only therapeutic option. Objective: To report our experience in the use of a transnasal endoscopic approach with stentless single side-hinged flap technique for the surgical management of choanal atresia. Methods: A 5 year retrospective analysis of surgical outcomes of 18 patients treated for choanal atresia with a transnasal technique employing a single side-hinged flap without stent placement. All subjects were assessed preoperatively with a nasal endoscopy and a Maxillofacial computed tomography scan. Results: Ten males and eight females with a mean age at the time of surgery of 20.05 ± 11.32 years, underwent surgery for choanal atresia. Fifteen subjects (83.33% had a bony while 3 (26.77% a mixed bony-membranous atretic plate. Two and sixteen cases suffered from bilateral and unilateral choanal atresia respectively. No intra- and/or early postoperative complications were observed. Between 2 and 3 months after surgery two cases (11.11% of partial restenosis were found. Only one of these presented a relapse of the nasal obstruction and was subsequently successfully repaired with a second endoscopic procedure. Conclusion: The surgical technique described follows the basic requirements of corrective surgery and allows good visualization, evaluation and treatment of the atretic plate and the posterior third of the septum, in order to create the new choanal opening. We believe that the use of a stent is not necessary, as recommended in case of other surgical techniques

  15. Check-list for the assessment of functional impairment in children with congenital aural atresia.

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    Montino, Silvia; Agostinelli, Anna; Trevisi, Patrizia; Martini, Alessandro; Ghiselli, Sara

    2017-11-01

    Congenital Aural Atresia (CAA) is a deformity of the external ear and it is commonly associated with malformations of middle and inner ear and, in some cases, with other facial deformities. Very few assessment measures exist for evaluating the functional impairment in children with CAA. Purpose of this study is to introduce and describe an assessment Checklist, (nominated FOS Checklist) that covers feeding abilities (F), oralmotor skills (O), communication/language development (S) in children with CAA. FOS wants to offer a range of assessment providing a profile of the child in comparison to hearing peers and it aims to make clinicians able to identify additional problems and areas of difficulties as well as specific abilities and skills. Secondary, we want to investigate the presence of correlations between disorders and side of CAA. a new Checklist (FOS Checklist) was administered to 68 children with CAA. Feeding abilities are age-adequate in 94,3% of all patients. 54,4% of all patients are in need for further assessment of their oral-motor skills; delays in language development were found in 44,1% of cases. Orofacial development delays have been observed in 57.2% of subjects among the bilateral CAA group, in 53.9% among the right CAA group and in 53.4% among the left CAA group. Patients referred for further language evaluation were 42,9% in the bilateral CAA group, 33.3% in the right CAA group and 33.3% in the left CAA group. According to the χ 2 analysis, referral for further assessment is independent from side of aural atresia. Subjects with bilateral CAA are more likely to be referred for further assessment, both for oral motor aspects and for speech perception and language development. However, there is not a significant statistical difference between the performances of children with bilateral or unilateral CAA. FOS Checklist is simple, reliable and time effective and can be used in everyday clinical practice. FOS enable clinicians to identify additional

  16. Pediatric Eating Assessment Tool-10 as an indicator to predict aspiration in children with esophageal atresia.

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    Soyer, Tutku; Yalcin, Sule; Arslan, Selen Serel; Demir, Numan; Tanyel, Feridun Cahit

    2017-10-01

    Airway aspiration is a common problem in children with esophageal atresia (EA). Pediatric Eating Assessment Tool-10 (pEAT-10) is a self-administered questionnaire to evaluate dysphagia symptoms in children. A prospective study was performed to evaluate the validity of pEAT-10 to predict aspiration in children with EA. Patients with EA were evaluated for age, sex, type of atresia, presence of associated anomalies, type of esophageal repair, time of definitive treatment, and the beginning of oral feeding. Penetration-aspiration score (PAS) was evaluated with videofluoroscopy (VFS) and parents were surveyed for pEAT-10, dysphagia score (DS) and functional oral intake scale (FOIS). PAS scores greater than 7 were considered as risk of aspiration. EAT-10 values greater than 3 were assessed as abnormal. Higher DS scores shows dysphagia whereas higher FOIS shows better feeding abilities. Forty patients were included. Children with PAS greater than 7 were assessed as PAS+ group, and scores less than 7 were constituted as PAS- group. Demographic features and results of surgical treatments showed no difference between groups (p>0.05). The median values of PAS, pEAT-10 and DS scores were significantly higher in PAS+ group when compared to PAS- group (p<0.05). The sensitivity and specificity of pEAT-10 to predict aspiration were 88% and 77%, and the positive and negative predictive values were 22% and 11%, respectively. Type-C cases had better pEAT-10 and FOIS scores with respect to type-A cases, and both scores were statistically more reliable in primary repair than delayed repair (p<0.05). Among the postoperative complications, only leakage had impact on DS, pEAT-10, PAS and FOIS scores (p<0.05). The pEAT-10 is a valid, simple and reliable tool to predict aspiration in children. Patients with higher pEAT-10 scores should undergo detailed evaluation of deglutitive functions and assessment of risks of aspiration to improve safer feeding strategies. Level II (Development of

  17. IL-6, TNF-α, IL-10, and nutritional status in pediatric patients with biliary atresia,

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    Maria Ines de Albuquerque Wilasco

    Full Text Available Abstract Objectives: The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. Methods: The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child–Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry. Results: IL-6, TNF-α, and IL-10 were significantly higher in the cirrhosis group when compared with the control group (2.4 vs. 0.24 (p < 0.001, 0.21 vs. 0.14 (p = 0.007, and 0.65 vs. 0.36 (p = 0.004, respectively. IL-6 and IL-10 were positively correlated with disease severity (0.450 [p = 0.001] and 0.410; [p = 0.002], respectively. TNF-α did not show a significant correlation with disease severity (0.100; p = 0.478. Regarding nutritional evaluation, IL-6 was negatively correlated with the standard deviation score of height-for-age (−0.493; p < 0.001 and of triceps skinfold thickness-for-age (−0.503; p < 0.001, respectively. IL-10 exhibited a negative correlation with the standard deviation score of height-for-age (−0.476; p < 0.001 and the standard deviation score of triceps skinfold thickness-for-age (−0.388; p = 0.004. TNF-α did not show any significance in both anthropometric parameters (−0.083 (p = 0.555 and −0.161 (p = 0.253. Conclusion: The authors suggest that, in patients with cirrhosis secondary to biliary atresia, IL-6 could be used as a possible supporting biomarker of deficient nutritional status and elevated IL-10 levels could be used as a possible early-stage supporting biomarker of deteriorating nutritional status.

  18. Prevalence of Malnutrition and Feeding Difficulties in Children With Esophageal Atresia.

    Science.gov (United States)

    Menzies, Jessica; Hughes, Jennifer; Leach, Steven; Belessis, Yvonne; Krishnan, Usha

    2017-04-01

    Growth and feeding problems have been described in children with esophageal atresia (EA). Ongoing gastrointestinal and respiratory complications such as Gastroesophageal reflux disease, esophageal dysmotility, strictures, and respiratory infections may contribute. The aim of the study was to document the prevalence of malnutrition and feeding difficulties and examine predictive factors, which may influence feeding and growth in children attending a multidisciplinary EA clinic in Sydney, Australia. A retrospective review of 75 children, ages 0 to 16 years, who attended a multidisciplinary EA clinic between 2011 and 2014. Data on demographics, comorbidities, nutrition, and mealtime behaviors were collected from their initial clinic appointment. Factors that may affect on growth and mealtime behaviors were identified and analyzed. Nine percent of children were malnourished and 9% were stunted. Infants, children with prior fundoplication, at risk of aspiration, or those who had surgery in the first year of life additional to EA repair were significantly more likely to be malnourished (P children required texture modification at their meals, with parental concern being the most common reason. Younger children were less likely to be eating age-appropriate textures (P = 0.04) which improved after 5 years of age. Poor growth and inability to manage age-appropriate textures are often present in children with EA, particularly in the younger years. This highlights the need for early intervention in a specialist multidisciplinary EA clinic in which dietetics and speech pathology are available.

  19. The accumulation of regulatory T cells in the hepatic hilar lymph nodes in biliary atresia.

    Science.gov (United States)

    Sakamoto, Naoya; Muraji, Toshihiro; Ohtani, Haruo; Masumoto, Kouji

    2017-10-01

    A proposed etiopathogenesis of biliary atresia (BA) involves T-cell-mediated inflammatory bile duct damage and progressive hepatic fibrosis. Pediatric surgeons often observe swelling of the hepatic hilar lymph nodes during the Kasai procedure. Given the importance of regulatory mechanisms in immune responses, the present study was designed to analyze the quantitative changes of regulatory T cells (T reg cells) in the hepatic hilar lymph nodes (hepatic hilar LNs) and peripheral blood (PB) in BA. The hepatic hilar LNs and PB obtained during the Kasai procedure were analyzed by flow cytometry. The ratios of total and active Tregs to the total CD4 + cells in the PB and the hepatic hilar LNs were compared. In patients with BA, the ratios of both the total and active T reg cells in the hepatic hilar LNs were higher than those in the PB (total T reg cells: PB vs. LN; P hilar lymph nodes of BA patients. This finding could shed light on the pathogenesis of BA.

  20. Intramural Ganglion Structures in Esophageal Atresia: A Morphologic and Immunohistochemical Study

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    Biagio Zuccarello

    2009-01-01

    Full Text Available Introduction and Aim. Disorders of esophageal motility causing dysphagia and gastroesophageal reflux are frequent in survivors to esophageal atresia (EA and distal tracheoesophageal fistula (TEF. The aim of the present study was to investigate the histologic and immunohistochemical features in both esophageal atretic segments to further understand the nature of the motor disorders observed in these patients. Material and Methods. Esophageal specimens from 12 newborns with EA/TEF and 5 newborns dead of unrelated causes were examined. The specimens were fixed in 5% buffered formalin, included in paraffin and cut in 5 micron sections that were stained with hematoxilin and eosin (H and E, and immunohistochemical stainings for Actin, S-100 protein, Neurofilament, Neuron-Specific-Enolase, Chromogranin A and Peripherin were evaluated under the microscope. Results. In controls, the distribution of the neural elements was rather homogenous at both levels of the esophagus. In contrast, the atretic segments showed quantitative and qualitative differences between them with sparser nervous tissue in the distal one in comparison with the proximal one and with controls. Conclusions. These results further support the assumption that histomorphological alterations of the muscular and nervous elements within the esophageal wall might contribute to esophageal dysmotility in patients surviving neonatal operations for EA/TEF.

  1. Scottish outcomes for extra hepatic biliary atresia post-rationalisation of services.

    Science.gov (United States)

    Tayler, Rachel; Barclay, Andrew R; Rogers, Pam; Mcintyre, Karen; Russell, Richard K; Devadason, David; Bisset, W Mike; Ling, Simon C; McGrogan, Paraic

    2013-05-01

    To evaluate the outcome of Scottish children with extra hepatic biliary atresia (EHBA) since rationalisation of Kasai services to three English centres in 2002 (The 'Group A' centres). All Scottish children with EHBA diagnosed between 2002 and 2009 were identified via the Scottish Society of Paediatric Gastroenterology, Hepatology and Nutrition (SSPGHAN) clinicians. A case-note review was conducted with demographics, presentation and outcome data recorded. These data were compared with historical Scottish data and data published previously by the supraregional liver units. 25 patients were identified, of whom 22 were referred for Kasai in the group A centres, and of whom 19 had a Kasai. 2 year transplant-free survival (TFS) was significantly lower in the SSPGHAN 2002-2009 group than the group A centres in (1) (6/18 (33%) vs 36/57 (63%), p=0.023). These postrationalisation data are disappointing. The emphasis for care will now focus on improved communication between, primary care, general paediatricians and surgical centres through regional and national managed clinical networks, aiming to improve future outcomes for Scottish children with BA.

  2. Bone Health in a Nonjaundiced Population of Children with Biliary Atresia

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    Rachel A. Kramer

    2009-01-01

    Full Text Available Objectives. To assess bone health in a cohort of nonjaundiced children with biliary atresia (BA and the effect of growth and development on bone outcomes. Methods. Children ages one to eighteen years receiving care from Children's Hospital of Philadelphia were recruited. Each child was seen once and assessed for growth, pubertal development, concurrent medications, bilirubin, ALT, albumin, vitamin D status, bone mineral density (BMD, and bone mineral content (BMC of the lumbar spine and whole body. Results. BMD declined significantly with age, and upon further analysis with a well-phenotyped control cohort, it was found that BMC was significantly decreased for both lumbar spine and whole body, even after adjustment for confounding variables. An age interaction was identified, with older subjects having a significantly greater impairment in BMC. Conclusions. These preliminary results demonstrate that children with BA, including those without jaundice, are likely to have compromised bone health even when accounting for height and puberty, which are common confounding factors in chronic disease. Further investigation is needed to identify the determinants of poor bone mineral status and to develop strategies to prevent osteoporosis later in life.

  3. Preterm Infants With Biliary Atresia: A Nationwide Cohort Analysis From The Netherlands.

    Science.gov (United States)

    van Wessel, Daan B E; Boere, Thomas; Hulzebos, Christian V; de Kleine, Ruben H J; Verkade, Henkjan J; Hulscher, Jan B F

    2017-10-01

    Biliary atresia (BA) occurs in 0.54 of 10.000 of overall live births in the Netherlands. BA has an unfavorable prognosis: Netherlands. Retrospective study including Dutch preterm infants treated for BA. Parameters included gestational age, congenital anomalies, age at KPE, days between first symptoms, and KPE and referral interval (first hospital to KPE). Outcome parameters were clearance of jaundice (COJ) and (transplant-free) survival. Data are presented as medians (ranges). Included 28 preterm infants (13 boys/15 girls) between March 1988 and December 2015. The incidence of BA was 1.06 of 10.000 preterm live births. Gestational age was 34.8 (27.3-36.9) weeks. Congenital anomalies were present in 11 of 28 (39%) infants. Time between first symptoms and KPE was 57 (9-138) days. Referral interval was 28 (8-86) days. Age at KPE was 70 (35-145) days. COJ was achieved in 23% of cases. Four-year transplant-free survival rate was 21%. Four-year overall survival was 61%. BA has a higher incidence in the preterm population compared to the overall BA population. The outcome of BA in preterm infants is poor, regarding COJ and (transplant-free) survival. We speculate that timely recognition of BA-related signs and symptoms in preterm infants will improve prognosis.

  4. Duodenal Atresia: Open versus MIS Repair—Analysis of Our Experience over the Last 12 Years

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    Salvatore Fabio Chiarenza

    2017-01-01

    Full Text Available Objective. Duodenal atresia (DA routinely has been corrected by laparotomy and duodenoduodenostomy with excellent long-term results. We revisited the patients with DA treated in the last 12 years (2004–2016 comparing the open and the minimally invasive surgical (MIS approach. Methods. We divided our cohort of patients into two groups. Group 1 included 10 patients with CDO (2004–09 treated with open procedure: 5, DA; 3, duodenal web; 2, extrinsic obstruction. Three presented with Down’s syndrome while 3 presented with concomitant malformations. Group 2 included 8 patients (2009–16: 1, web; 5, DA; 2, extrinsic obstruction. Seven were treated by MIS; 1 was treated by Endoscopy. Three presented with Down’s syndrome; 3 presented with concomitant malformations. Results. Average operating time was 120 minutes in Group 1 and 190 minutes in Group 2. In MIS Group the visualization was excellent. We recorded no intraoperative complications, conversions, or anastomotic leakage. Feedings started on 3–7 postoperative days. Follow-up showed no evidence of stricture or obstruction. In Group 1 feedings started within 10–22 days and we have 1 postoperative obstruction. Conclusions. Laparoscopic repair of DA is one of the most challenging procedures among pediatric laparoscopic procedures. These patients had a shorter length of hospitalization and more rapid advancement to full feeding compared to patients undergoing the open approach. Laparoscopic repair of DA could be the preferred technique, safe, and efficacious, in the hands of experienced surgeons.

  5. Treatment and Outcome for Children with Esophageal Atresia from a Gender Perspective

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    Julia Ekselius

    2017-01-01

    Full Text Available Background. Besides the incidence of esophageal atresia (EA being higher in males, no other gender-specific differences in EA have been reported. The aim of this study was to search for gender-specific differences in EA. Methods. A retrospective study was conducted at a tertiary center for pediatric surgery. The medical charts of infants born with EA were reviewed. 20 girls were identified, and 20 boys were selected as matched controls with respect to concomitant malformations. Their treatment and outcome were evaluated. Results. Polyhydramnios was more common in pregnancies with boys, 40%, versus girls, 10%, with EA (p<0.01. In total, 36 (90% children had patent ductus arteriosus, without any gender difference (18 and 18, resp., p=1. The distribution of days at the different levels of care was not equally distributed between boys and girls. Boys with EA had significantly more postoperative days (median 5 days in the ward than girls (median 5 and 2 days, resp., p=0.04. No other gender-specific differences in surgical treatment, complications, or symptoms at follow-up were identified. Conclusion. Polyhydramnios appears to be more frequent in pregnancies with boys than girls with EA. In this study, boys have longer stays than girls at the pediatric surgery ward.

  6. Treatment and Outcome for Children with Esophageal Atresia from a Gender Perspective.

    Science.gov (United States)

    Ekselius, Julia; Salö, Martin; Arnbjörnsson, Einar; Stenström, Pernilla

    2017-01-01

    Besides the incidence of esophageal atresia (EA) being higher in males, no other gender-specific differences in EA have been reported. The aim of this study was to search for gender-specific differences in EA. A retrospective study was conducted at a tertiary center for pediatric surgery. The medical charts of infants born with EA were reviewed. 20 girls were identified, and 20 boys were selected as matched controls with respect to concomitant malformations. Their treatment and outcome were evaluated. Polyhydramnios was more common in pregnancies with boys, 40%, versus girls, 10%, with EA ( p patent ductus arteriosus, without any gender difference (18 and 18, resp., p =1). The distribution of days at the different levels of care was not equally distributed between boys and girls. Boys with EA had significantly more postoperative days (median 5 days) in the ward than girls (median 5 and 2 days, resp., p =0.04). No other gender-specific differences in surgical treatment, complications, or symptoms at follow-up were identified. Polyhydramnios appears to be more frequent in pregnancies with boys than girls with EA. In this study, boys have longer stays than girls at the pediatric surgery ward.

  7. Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome.

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    Wu, E T; Chen, H L; Ni, Y H; Lee, P I; Hsu, H Y; Lai, H S; Chang, M H

    2001-07-01

    Bacterial cholangitis (BC) is a common complication in patients with biliary atresia (BA) and is characterized by fever, acholic stools and positive blood cultures. The diagnosis is often empirical because the yield of blood cultures is low. It is difficult to differentiate BC from other febrile episodes. In order to characterize the clinical and laboratory features of BC in patients with BA, identify risk factors, and correlate cholangitis with outcome, 37 patients with BA from 1993 to 1998 who underwent a Kasai operation in our hospital were studied. The follow-up period ranged from 6 to 59 months. A total of 107 febrile episodes were documented in these patients. The diagnostic criteria for cholangitis were fever, increased jaundice, or acholic stools. The clinical features, laboratory data, results of bacterial cultures, and outcomes were analyzed retrospectively. A total of 107 febrile episodes, including 78 bouts of cholangitis and 29 non-cholangitis infections, were found in 34 patients. Patients with BC had higher postoperative bilirubin levels (P = 0.02) and less frequent use of prophylactic antibiotics (P = 0.05) than those with non-cholangitis infections. Abnormal white blood cell counts (> 12,000 or Acinetobacter baumanni, and Salmonella typhi. The sensitivity tests justified empirical therapy with ceftriaxone. The effectiveness of prophylactic trimethoprim-sulfamethoxazole or neomycin warrants further studies. BC was a highly prevalent postoperative complication in patients with BA, especially those with inadequate bile drainage. It significantly affected early mortality. Aggressive and complete treatment with empirical ceftriaxone was appropriate.

  8. Distribution of Interstitial Cells of Cajal in the Esophagus of Fetal Rats with Esophageal Atresia

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    Caner Isbir

    2016-04-01

    Full Text Available Aim: Scarcity of the interstitial cells of Cajal (ICC is related to motility disorders. In the study, we aimed to evaluate the number and density of ICCs in the fetal rat esophagus in the adriamycin - esophageal atresia (EA model. Material and Method: Rat fetuses were divided into three groups as a control, adriamycin group without EA and adriamycin group with EA. Four doses of adriamycin, 2 mg/kg each, were injected intraperitoneally to the adriamycin group rats between on 6 and 9 days of gestation. The presence of ICCs in the esophagus of the rat fetuses was determined by using an immunohistochemistry technique (c-kit, CD117. The average numbers of ICCs were calculated with microscopic evaluation by using a visual scoring system (range1 to 3. Results: Seven fetuses were included in each group. The ICCs score 3 distributions of fetuses were 5 (72% fetuses in the control group, 3 (43% fetuses in the adriamycin group without EA, 1 (14% fetus in the adriamycin group with EA. It have been found that there was a marked reduction of ICCs distribution in the adriamycin group with EA compared to control group (p 0.05. Discussion: ICCs density was significantly decreased in the rat fetuses with EA compared to the fetuses without EA. These findings support the idea that ICCs density may be congenitally abnormal in EA. This may be led to dismotility seen in the operated esophagus due to EA.

  9. The role of splenectomy before liver transplantation in biliary atresia patients.

    Science.gov (United States)

    Takahashi, Yoshiaki; Matsuura, Toshiharu; Yanagi, Yusuke; Yoshimaru, Koichiro; Taguchi, Tomoaki

    2016-12-01

    There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score. The mean age was 17.5±7.0years (range 11-31years), and the male-to-female ratio was 1:1. The platelet and leukocyte levels increased after splenectomy and returned to normal levels one month postoperatively. The mean MELD score after splenectomy was significantly decreased after splenectomy: 10±2.1 vs 7.6±1.8. In particular, PT-INR improved. Five patients underwent liver transplantation because of hepatopulmonary syndrome and repeated bouts of cholangitis, whereas the remaining five patients did not undergo liver transplantation because of improvements in the liver function (the mean follow-up period was 56months). The postoperative complications included portal vein thrombosis and intestinal perforation, but the patient survival rates remained at 100%. After splenectomy, both pancytopenia and the liver function clearly improved. Splenectomy should therefore be a treatment option for patients with hypersplenism before liver transplantation. Retrospective Comparative Study - Level III. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Esophageal atresia with or without tracheophgeal fistula: success and failure in 94 cases

    International Nuclear Information System (INIS)

    Al-Salem, Ahmed H.; Tayeb, M.; Khogair, S.; Roy, A.; Al-Jishi, N.; Alsenan, K.; Shaban, H.; Ahmad, M.

    2006-01-01

    The management of newborns with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) has evolved considerably over the years. Currently an overall survival of 85% to 90% has been reported from developed countries. In developing countries, several factors contribute to higher mortalities rates. We describe our experience with 94 consecutive cases of FA with or without TEF. We retrospectively studied 94 patients with EA with or without TEF treated at our hospital over a period of 15 years. Medical records were reviewed for age at diagnosis, sex, birth weight, associated anomalies, aspiration pneumonia, method of diagnosis, treatment, postoperative complications and outcome. Ninety-four newborns (55 males and 39 females) with EA/TEF were treated at our hospital. Their mean birth weight was 2.2 kg (700g to 3800g). Age at diagnosis ranged from birth to 7 days. At the time of admission 37 (39.4%) had aspiration pneumonia. Associated anomalies were seen in 46 (49%) patients. Thirteen patients had major associated anomalies that contributed to mortality. Postoperative complications were similar to those from developed countries but overall operative mortality (30.8%) was high. The overall mortality was high but excluding major congenital malformations, sepsis was the most frequent cause of death. Factors contributing to mortality included prematurely, delay in diagnosis with an increased incidence of aspiration pneumonia and a shortage of qualified nurses. To improve overall outcome, factors contributing to sepsis should be evaluated and efforts and efforts should be made to overcome them. (author)

  11. Aspectos radiológicos da atresia brônquica: relato de três casos e revisão da literatura Radiological aspects of bronchial atresia: report of three cases and review of the literature

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    Alexandre da Silva Neu

    2003-01-01

    Full Text Available A atresia brônquica é uma anomalia congênita rara que usualmente produz massa justa-hilar com hipertransparência distal à radiografia simples. A maioria dos pacientes é jovem e não apresenta sintomas. A tomografia computadorizada confirma o diagnóstico, permitindo o manejo conservador nos casos assintomáticos. Os autores relatam três casos de atresia brônquica. Um caso, de particular interesse, estava associado a volumosa broncocele em paciente do sexo feminino, de 45 anos de idade, que apresentou quadro sintomático de infecção respiratória. O diagnóstico foi estabelecido por meio dos achados da radiologia convencional, da tomografia computadorizada e da comparação com radiografias convencionais e tomografia linear prévios, que já demonstravam alterações. Realizou-se uma revisão sobre os achados clínicos e radiológicos dessa malformação.Bronchial atresia is a rare congenital abnormality that usually shows a juxta-hilar mass with distal radiolucency on conventional x-ray films. Most patients are young and have no symptoms. Computed tomography usually confirms the diagnosis, allowing conservative management of the asymptomatic cases. The authors report three cases of bronchial obstruction. One of the patients was of particular importance and interest, a 45-year-old female patient with symptoms of respiratory infection, due to the association with a bulky bronchocele. The diagnosis was made by analyzing the findings of conventional x-ray films and computed tomography, and the comparison with previous conventional x-ray films and linear tomography, which had already demonstrated abnormalities. A literature review on the clinical and radiological findings of this pulmonary malformation is presented.

  12. Paciente con esquizofrenia tratado con ziprasidona + clozapina

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    Pol Yanguas E.

    2013-05-01

    Full Text Available P es un paciente diagnosticado de esquizofrenia, sigue en un piso tutelado un programa de rehabilitación, está medicado con clozapina 500 mg/día y ziprasidona 280 mg/ día. Padece hipercolesterolemia, tabaquismo y sus hábitos alimenticios no son buenos. La medicación que utiliza desde 2007 hasta ahora se refleja en la tabla 1. El último tratamiento se le introdujo el 7 de agosto de 2012, habiendo presentado un electro cardiograma (ECG normal, pero con ligera taquicardia ventricular y prolactinemia de 44,8 ng/ml (valores normales: 2-18 ng/ml.

  13. Combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in patients with congenital atresia of cervix.

    Science.gov (United States)

    Zhang, Xuyin; Han, Tiantian; Ding, Jingxin; Hua, Keqin

    2015-01-01

    The aim of this study was to introduce a new technique which is combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in patients with congenital atresia of cervix and to evaluate the feasibility and the safety of it. This is a prospective observational study of 10 patients with congenital atresia of cervix who underwent combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft for cervicovaginal reconstruction from February 2013 to August 2014 in our hospital. All of the surgical procedures were carried out by the same operation team. Patient data were collected including operating time, estimated blood loss, hospital stay post-surgery, complications, total cost, and median vaginal length at 3 month, resumption of menstruation, vaginal stenosis and stricture of the cervix postoperatively. The operative procedure lasted 237±46 (175-380) min. The estimated blood loss was 160±76 (50-300) ml. The hospital stay post-surgery was 12±2 (9-18) days. None of the patients had complications or required a blood transfusion. The mean total cost was $3352±1025. The average vaginal length at 3 month was 8.3±1.1 (8-10) cm. All patients had resumption of menstruation. The patients were followed for a mean of 5±2 (1-10) months. Cervical or vaginal stenosis did not occur in any of the patients. Our experiences of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in10 patients with congenital atresia of cervix were positive, with successful results and without complications, and cervical or vaginal stenosis.

  14. Congenital hernia of the umbilical cord associated with extracelomic colonic atresia and perforation of gut in a newborn

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    Kamalesh Pal

    2014-01-01

    Full Text Available Congenital hernia of the umbilical cord (CHUC is a rare congenital entity compared to more common post-natally occurring umbilical hernia. Although recognized as a distinct entity since 1920s, CHUC is often misdiagnosed as a small omphalocele, resulting in its underreporting. We present the first case report of CHUC associated with extracelomic colonic atresia, complicated by perinatal perforation in a newborn. We also discuss the differentiating features from other anterior abdominal wall defects such as omphalocele and gastroschisis including its embryogenesis.

  15. Tratamento cirúrgico da atresia de vias biliares: a experiência do hospital municipal jesus, 1997-2000

    OpenAIRE

    Jesus, Lisieux Eyer de; Monteiro, Paulo Cesar Costa

    2001-01-01

    OBJETIVO: Analisar a indicação, resultados e limitações da portoenterostomia para o tratamento da atresia de vias biliares em hospital infantil terciário no Rio de Janeiro-RJ/Brasil. MÉTODO: Foram estudados prospectivamente oito pacientes submetidos à portoenterostomia do tipo Kasai 1, num período de três anos (1997-2000). Todos foram submetidos à antibioticoterapia profilática, uso de colerético, reavaliação médica mensal nos primeiros seis meses de seguimento e reavaliação laboratorial, ult...

  16. A Rare Coincidence of Infantile Hypertrophic Pyloric Stenosis and Esophageal Atresia with TracheoEsophageal Fistula: A Case Report

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    Yesim Coskun

    2017-10-01

    Full Text Available The coincidence of Infantile Hypertrophic Pyloric Stenosis (IHPS and Esophageal Atresia (EA with Tracheo-Esophageal Fistula (TEF is rare. Although, vomiting and regurgitation in operated cases of EA and TEF are attributed to Gastroesophageal Reflux (GER and the stricture of the anastomosis, it may be also associated with IHPS as well. We report a case of 3-hour-old female infant, who had EA and TEF operation and diagnosed to have IHPS at 9th week of age. Early diagnosis and treatment can prevent complications.

  17. Expanding the cardiac spectrum of Noonan syndrome with RIT1 variant: Left main coronary artery atresia causing sudden death.

    Science.gov (United States)

    Ramond, Francis; Duband, Sébastien; Croisille, Pierre; Cavé, Hélène; Teyssier, Georges; Adouard, Véronique; Touraine, Renaud

    2017-06-01

    Noonan syndrome is a well-known genetic condition associating congenital heart defects, short stature, and distinctive facial features. Pulmonary valve stenosis and hypertrophic cardiomyopathy are the most frequent cardiac abnormalities, the latter being associated with a higher mortality. Here we report for the first time, a case of congenital left main coronary artery atresia in a Noonan syndrome associated with RIT1 variant, leading to unrescued sudden death. This case-report supports the already-suspected severity of the RIT1-related Noonan syndrome compared to average Noonan syndrome, and should encourage clinicians to be very cautious with these patients. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  18. Telomere Length in Peripheral Blood Leukocytes Is Associated with Severity of Biliary Atresia.

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    Wanvisa Udomsinprasert

    Full Text Available The purpose of this study was to investigate the association of telomere length in peripheral blood leukocytes with the severity of biliary atresia (BA.One hundred and fourteen BA patients and 114 age-matched healthy controls were enrolled. Relative telomere length (RTL was assessed using a quantitative real-time polymerase chain reaction. Multivariate regression analysis was used to estimate RTL as an independent risk factor of BA. Receiver operating characteristic curve analysis was used to calculate the accuracy of biomarkers in the prediction of liver cirrhosis.BA patients had significantly shorter telomeres than healthy controls (p < 0.0001. The RTL in BA patients with jaundice was considerably lower than that of patients without jaundice (p = 0.005. Moreover, RTL was markedly shorter in patients with cirrhosis (F4, as compared to patients with mild fibrosis (F2 and non-fibrosis (F0-F1, p < 0.0001. Logistic regression analysis indicated that short RTL was associated with a higher risk of liver cirrhosis in BA. Tertile analysis showed a dose-response effect for this association (p trend < 0.0001. Additionally, RTL in BA children revealed a negative correlation with age (r = -0.50, p < 0.001. We noted an association between reduction of RTL and liver stiffness scores, adjusted for age and gender (b = -0.01, p < 0.0001. Short RTL can be employed to distinguish cirrhosis patients from non-cirrhosis patients (AUC = 0.78. Further analysis showed a linear correlation between leukocyte RTL and liver RTL in BA patients (r = 0.83, p < 0.001.The findings of this study provide evidence that telomere shortening is associated with an elevated risk of liver cirrhosis in BA.

  19. Association between Promoter Hypomethylation and Overexpression of Autotaxin with Outcome Parameters in Biliary Atresia.

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    Wanvisa Udomsinprasert

    Full Text Available Biliary atresia (BA is a progressive fibroinflammatory liver disease. Autotaxin (ATX has a profibrotic effect resulting from lysophosphatidic acid activity. The purpose of this study was to examine ATX expression and ATX promoter methylation in peripheral blood leukocytes and liver tissues from BA patients and controls and investigate their associations with outcome parameters in BA patients.A total of 130 subjects (65 BA patients and 65 age-matched controls were enrolled. DNA was extracted from circulating leukocytes and liver tissues of BA patients and from and age-matched controls. ATX promoter methylation status was determined by bisulfite pyrosequencing. ATX expression was analyzed using quantitative real-time polymerase chain reaction and enzyme-linked immunosorbent assay.Decreased methylation of specific CpGs were observed at the ATX promoter in BA patients. Subsequent analysis revealed that BA patients with advanced stage had lower methylation levels of ATX promoter than those with early stage. ATX promoter methylation levels were found to be associated with hepatic dysfunction in BA. In addition, ATX expression was significantly elevated and correlated with a decrease in ATX promoter methylation in BA patients compared to the controls. Furthermore, promoter hypomethylation and overexpression of ATX were inversely associated with jaundice status, hepatic dysfunction, and liver stiffness in BA patients.Accordingly, it has been hypothesized that ATX promoter methylation and ATX expression in peripheral blood may serve as possible biomarkers reflecting the progression of liver fibrosis in postoperative BA. These findings suggest that the promoter hypomethylation and overexpression of ATX might play a contributory role in the pathogenesis of liver fibrosis in BA.

  20. Operative intercostal chest drain is not required following extrapleural or transpleural esophageal atresia repair.

    Science.gov (United States)

    Paramalingam, Saravanakumar; Burge, David M; Stanton, Michael P

    2013-08-01

    Approximately half of the United Kingdom patients undergoing esophageal atresia (OA) repair have an operative intercostal chest drain (ICD) placed (2008 British Association of Pediatric Surgeons Congenital Anomalies Surveillance Study data). We reviewed our experience of OA repairs to evaluate if an ICD placement is necessary. Patients with OA/distal tracheoesophageal fistula (TOF), treated between January 1990 and January 2010, were identified by retrospective review of a prospectively maintained electronic database and patient case notes. A total of 112 consecutive patients were identified, of whom 107 were included (73 male). Five were excluded as no case notes were available. Median birth weight was 2,597 g (range 924 to 4,245 g) and median gestational age was 38 weeks (27 to 41 weeks). Median age at discharge was 22 days (3 to 440 days) and median follow-up was 3.5 years (0 to 18 years). Patients were analyzed in two groups-group 1 (n = 73) had an extrapleural (EP) repair, of which 23 had a pleural breach and group 2 (n = 34) had a purposeful transpleural (TP) approach (surgeon preference). Eleven patients (10%) had an operative ICD, of which six patients were in group 1 and five in group 2. These 11 patients had an uncomplicated postoperative course and all operative ICD were removed within 48 hours of surgery. Of the 96 patients that did not have an operative ICD, only 2 (2%) required postoperative intervention. One patient, in group 2, had a postoperative ICD inserted for a simple pneumothorax at 12 hours and removed at 48 hours. The other patient, in group 1, had a clinically detected anastomotic leak after 48 hours and required operative repair. An operative ICD is not required following OA/distal TOF repair, whether the approach is EP or TP. ICD that were electively placed (in 10%) served no clinical purpose. Georg Thieme Verlag KG Stuttgart · New York.

  1. Ultrasonographic diagnosis of biliary atresia based on a decision-making tree model

    International Nuclear Information System (INIS)

    Lee, So Mi; Cheon, Jung Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun Hye; Kim, In One; You, Sun Kyoung

    2015-01-01

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology

  2. Ultrasonographic Diagnosis of Biliary Atresia Based on a Decision-Making Tree Model.

    Science.gov (United States)

    Lee, So Mi; Cheon, Jung-Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun-Hae; Cho, Hyun-Hye; Kim, In-One; You, Sun Kyoung

    2015-01-01

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.

  3. Susceptibility to experimental biliary atresia linked to different hepatic gene expression profiles in two mouse strains.

    Science.gov (United States)

    Leonhardt, Johannes; Kuebler, Joachim F; Turowski, Carmen; Tschernig, Thomas; Geffers, Robert; Petersen, Claus

    2010-02-01

    To compare hepatic gene expression during the development of experimental biliary atresia (BA) in two different mouse strains. Balb/c mice and C57Black/6 (Black/6) mice were infected with rhesus rotavirus (RRV) postpartum, clinical signs of BA and survival were noted. Liver sections were assessed for cluster of differentiation antigen (CD) 3, CD4 and CD8 expression, and the hepatic virus load was determined. Second, mice of both strains were sacrificed three days after infection. Isolated hepatic RNA was subjected to gene expression analysis using Affymetrix Gene Chip MOE 430 2.0. The incidence of BA was significantly lower in Black/6 mice compared to Balb/c mice (13.5% vs. 67%, P < 0.05). The mean virus titers were higher in mice with BA compared to mice without BA. Different gene profiles three days after virus infection were noted, with differential expression of 201 genes, including those regulating apoptosis, nucleic acid binding, transport function and particularly the immune response (chemokine C-C motif ligand 2, toll-like receptor 3, CD antigen 14, chemokine (C-X-C motif) ligands 10 and 11). This correlated with a significant increase of CD4 positive cells only in Balb/c mice with BA compared to healthy mice (13.5 vs. 5.0; P < 0.05). Black/6 mice did not exhibit any significant increase of CD3 or CD4 leukocytes despite cholestasis. The different susceptibility to experimental BA was associated with an increase of CD4 T-cells in the liver of Balb/c mice, which is linked to different gene profiles at the onset of bile duct obstruction.

  4. Pattern and survival of biliary atresia patients; Experience in Southern Nigeria

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    Philemon E Okoro

    2013-01-01

    Full Text Available Background: Biliary atresia (BA has been a challenge to surgeons worldwide. Beyond the revolutionary surgical technique popularised by Kasai, liver transplantation has added renewed hope in the long-term outcome. In Nigeria, where late presentation is very common, there is need to assess the long-term results of the treatment options available to us. Aim: We aimed to evaluate the presentation and management outcome of BA and the long-term survival of BA patients seen in our practice. Materials and Methods: Cases of BA seen between January 2007 and December 2011 in three tertiary health facilities in South East Nigeria were included. Data obtained included age at presentation, clinical features, treatment offered and age at the time of death. Analysis was with the SPSS 17.0. Results: Twenty four patients comprising 10 (41.7% males and 14 (58.3% females were included in the study. The mean age of presentation was 4.02 (±214 months; range 1.75-11.0 months. Fifteen (62.5% patients had surgery while 9 (37.5% received medical treatment only. The mean age at death was 14.2 (±8.1 months; range 2.5-30 months. Conclusion: BA poses a daunting challenge in our practice. Outcome of treatment is still discouraging. We identified late presentation, lack of facilities to make early diagnosis, lack of adequately trained manpower to manage these children and lack of post-operative care and support for patients as the major challenges in the management of BA children in our region.

  5. Structural airway abnormalities contribute to dysphagia in children with esophageal atresia and tracheoesophageal fistula.

    Science.gov (United States)

    Baxter, Katherine J; Baxter, Lauren M; Landry, April M; Wulkan, Mark L; Bhatia, Amina M

    2018-01-31

    Long-term dysphagia occurs in up to 50% of repaired esophageal atresia and tracheoesophageal fistula (EA/TEF) patients. The underlying factors are unclear and may include stricture, esophageal dysmotility, or associated anomalies. Our purpose was to determine whether structural airway abnormalities (SAA) are associated with dysphagia in EA/TEF. We conducted a retrospective chart review of children who underwent EA/TEF repair in our hospital system from 2007 to 2016. Children with identified SAA (oropharyngeal abnormalities, laryngeal clefts, laryngomalacia, vocal cord paralysis, and tracheomalacia) were compared to those without airway abnormalities. Dysphagia outcomes were determined by the need for tube feeding and the modified pediatric Functional Oral Intake Scale (FOIS) at 1 year. SAA was diagnosed in 55/145 (37.9%) patients with EA/TEF. Oropharyngeal aspiration was more common in children with SAA (58.3% vs. 36.4%, p=0.028). Children with SAA were more likely to require tube feeding both at discharge (79.6% vs. 48.3%, pesophageal stricture, the presence of SAA remained a significant risk factor for dysphagia (OR 4.17 (95% CI 1.58-11.03)). SAA are common in children with EA/TEF and are associated with dysphagia, even after accounting for gestational age, esophageal gap and stricture. This study highlights the need for a multidisciplinary approach, including early laryngoscopy and bronchoscopy, in the evaluation of the EA/TEF child with dysphagia. Level II retrospective prognostic study. Copyright © 2018. Published by Elsevier Inc.

  6. Contractile profile of esophageal and gastric fundus strips in experimental doxorubicin-induced esophageal atresia

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    F.A. Capeto

    2015-05-01

    Full Text Available Esophageal atresia (EA is characterized by esophageal and gastric motility changes secondary to developmental and postsurgical damage. This study evaluated the in vitro contractile profile of the distal esophagus and gastric fundus in an experimental model of EA induced by doxorubicin (DOXO. Wistar pregnant rats received DOXO 2.2 mg/kg on the 8th and 9th gestational days. On day 21.5, fetuses were collected, sacrificed, and divided into groups: control, DOXO without EA (DOXO-EA, and DOXO with EA (DOXO+EA. Strips from the distal esophagus and gastric fundus were mounted on a wire myograph and isolated organ-bath system, respectively, and subjected to increasing concentrations of carbamylcholine chloride (carbachol, CCh. The isolated esophagus was also stimulated with increasing concentrations of KCl. In esophagus, the concentration-effect curves were reduced in response to CCh in the DOXO+EA and DOXO-EA groups compared to the control group (P0.05. In response to KCl, the distal esophagus samples in the three groups were not statistically different with regard to Emax or EC50 values (P>0.05. No significant difference was noted for EC50 or Emax values in fundic strips stimulated with CCh (P>0.05. In conclusion, exposure of dams to DOXO during gestation inhibited the contractile behavior of esophageal strips from offspring in response to CCh but not KCl, regardless of EA induction. The gastric fundus of DOXO-exposed offspring did not have altered contractile responsiveness to cholinergic stimulation.

  7. Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation.

    Science.gov (United States)

    Lien, Tien-Hau; Bu, Ling-Nan; Wu, Jia-Feng; Chen, Huey-Ling; Chen, An-Chyi; Lai, Ming-Wei; Shih, Hsiang-Hung; Lee, I-Hsien; Hsu, Hong-Yuan; Ni, Yen-Hsuan; Chang, Mei-Hwei

    2015-05-01

    Recurrent cholangitis may aggravate cholestatic liver cirrhosis in biliary atresia (BA) after the Kasai operation. This pilot study aimed to investigate whether Lactobacillus casei rhamnosus has the prophylactic efficacy for recurrent cholangitis in comparison with the conventional neomycin prophylaxis. Twenty jaundice-free patients with BA ages 0 to 3 years who underwent a Kasai operation were enrolled and randomized into 2 groups with 10 patients each: neomycin (25 mg · kg · day for 4 days/wk) and L casei rhamnosus (8 × 10 colony-forming unit per day) groups. The treatment duration was 6 months. Bacterial stool cultures were performed before treatment and 1, 3, and 6 months after starting treatment. In addition, 10 patients with BA with similar status but without prophylaxis served as the historical control group. In the Lactobacillus group, 2 patients (20%, mean 0.03 ± 0.07 episodes per month) developed cholangitis during the study period, with the same frequency as in the neomycin group and significantly lower than that in the control group (80%, P = 0.005, mean 0.22 ± 0.16 episodes per month). The mean change in body weight z score during the 6 months in the Lactobacillus group was 0.97 ± 0.59, which was significantly better than that in the control group (-0.01 ± 0.79, P = 0.006). In bacterial stool cultures, the Lactobacillus and Escherichia coli populations significantly increased and decreased, respectively, in the Lactobacillus group. The use of L casei rhamnosus was as effective as neomycin in preventing cholangitis in patients with BA who underwent Kasai operation, and therefore could be considered as a potential alternative prophylactic regimen.

  8. Ultrasonographic diagnosis of biliary atresia based on a decision-making tree model

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    Lee, So Mi; Cheon, Jung Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun Hye; Kim, In One; You, Sun Kyoung [Dept. of Radiology, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2015-12-15

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.

  9. Th-17 cells infiltrate the liver in human biliary atresia and are related to surgical outcome.

    Science.gov (United States)

    Hill, Richard; Quaglia, Alberto; Hussain, Munther; Hadzic, Nedim; Mieli-Vergani, Giorgina; Vergani, Diego; Davenport, Mark

    2015-08-01

    Biliary atresia (BA), a cholangiopathy of unknown etiology is associated with intrahepatic mononuclear cell infiltrate. An abnormal reaction to viral exposure has been hypothesized in some cases. We aimed to investigate the nature of the CD4+ hepatic infiltrate in defined clinical variants of BA by quantification of inflammatory cell components. Liver biopsies of infants obtained at Kasai portoenterostomy (KPE) were stained immunohistochemically using monoclonal antibodies to Tbet, GATA-3, FOXP3 and interleukin (IL) 17, identifying Th-1, Th-2, Tregs and Th-17 cells respectively. T cells were counted with the aid of a graticule. Data are reported as median (range) of cells per high-power-field (×400) and compared using nonparametric statistical tests with P≤0.05 regarded as significant. Liver biopsies from BA (n=37) and age-matched cholestatic controls (e.g. alpha-1-anti trypsin deficiency, Alagilles syndrome, n=12) were investigated. BA infants were divided into three groups: cytomegalovirus IgM +ve (CMV; n=9); BA splenic malformation (BASM; n=9) and isolated BA (IBA; n=19). All T-cell subsets were present in the portal tracts, with an overrepresentation of Th-1 (PTh-17 (PTh-2 (P=0.94) or Tregs (P=0.15), compared to controls. Th-1 cells predominated in the CMV group; (18 [7-37] vs. 3 [0-14] [BASM] and vs. 5 [3-23] [IBA]; PTh-17 cells. The degree of Th-1 cell infiltrate inversely correlated with platelet count (rS=-0.49; PTh-17 cells were fewer (6 [2-11] vs. 11 [8-20]; P=0.02) in infants who cleared their jaundice (n=15, Th-17 cells infiltrate the liver in BA and are associated with a worse surgical outcome; a Th-1 profile predominates in CMV-associated BA. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Characterization of the upper pouch tracheo-oesophageal fistula in oesophageal atresia.

    Science.gov (United States)

    Summerour, Virginia; Stevens, Paul S; Lander, Anthony D; Singh, Michael; Soccorso, Giampiero; Arul, G Suren

    2017-02-01

    A small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF. A retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed. Eight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28-39) weeks, and median birth weight was 1647g (range 1100-3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n=2), at the time of thoracotomy when mobilizing the upper pouch (n=3), or during subsequent bronchoscopy for symptoms post OA repair (n=2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus. Upper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature. level IV. Copyright © 2016. Published by Elsevier Inc.

  11. Predictive factors for complications in children with esophageal atresia and tracheoesophageal fistula.

    Science.gov (United States)

    Shah, R; Varjavandi, V; Krishnan, U

    2015-04-01

    The objective of this study was to describe the incidence of complications in children with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) at a tertiary pediatric hospital and to identify predictive factors for their occurrence. A retrospective chart review of 110 patients born in or transferred to Sydney Children's Hospital with EA/TEF between January 1999 and December 2010 was done. Univariate and multivariate regression analyses were performed to identify predictive factors for the occurrence of complications in these children. From univariate analysis, early esophageal stricture formation was more likely in children with 'long-gap' EA (odds ratio [OR] = 16.32). Patients with early strictures were more likely to develop chest infections (OR = 3.33). Patients with severe tracheomalacia were more likely to experience 'cyanotic/dying' (OR = 180) and undergo aortopexy (OR = 549). Patients who had gastroesophageal reflux disease were significantly more likely to require fundoplication (OR = 10.83) and undergo aortopexy (OR = 6.417). From multivariate analysis, 'long-gap' EA was a significant predictive factor for late esophageal stricture formation (P = 0.007) and for gastrostomy insertion (P = 0.001). Reflux was a significant predictive factor for requiring fundoplication (P = 0.007) and gastrostomy (P = 0.002). Gastrostomy insertion (P = 0.000) was a significant predictive factor for undergoing fundoplication. Having a prior fundoplication (P = 0.001) was a significant predictive factor for undergoing a subsequent aortopexy. Predictive factors for the occurrence of complications post EA/TEF repair were identified in this large single centre pediatric study. © 2014 International Society for Diseases of the Esophagus.

  12. Dental management of pediatric patients affected by pulmonary atresia with ventricular septal defect: A scoping review.

    Science.gov (United States)

    Garrocho-Rangel, A; Echavarría-García, A-C; Rosales-Bérber, M-A; Flores-Velázquez, J; Pozos-Guillén, A

    2017-07-01

    Congenital Heart Diseases (CHD) involves a wide range of pathological conditions, such as Pulmonary Atresia with Ventricle Septal Defect (PA/VSD). This disorder leads to the systemic circulation of oxygen-poor blood (cyanosis), with associated features and consequences in the oral cavity. Using scoping review methodology for screening and article selection, the primary objectives of this paper were as follows: first, to pose a research question; second, to identify relevant studies in order to answer the research question; third, to select and retrieve the studies; fourth, to chart the critical data, and finally, to collate, summarize, and report the results from the most important articles on the dental management of children affected with PA/VSD. Relevant articles (Randomized Controlled Trials [RCT], reviews, observational studies, and clinical case reports) published over a 10-year period were identified and retrieved from four Internet databases: PubMed; Embase/Ovid; Cochrane Library, and Google Scholar. By title and abstract screening and after removing duplicates, 24 articles were finally included in the present scoping review. According to the extracted data, the following are the most important clinical issues to be considered when treating children with PA/VSD in the dental setting: prevalence of dental caries; prevention of dental disease (oral hygiene and diet); bacteremia and infective endocarditis risk, and child behavior control and treatment under general anesthesia. Pediatric Dentists should bear in mind that early diagnosis and treatment, together a long-term follow-up of children with PA/VSD, continue to be the best approaches for achieving enhanced patient psychological well-being and, in consequence, their good quality of life.

  13. Congenital anorectal atresia: MR imaging of late post-operative appearances in adult patients with anal incontinence

    International Nuclear Information System (INIS)

    Gartner, Louise; Peiris, Chand; Marshall, Michele; Taylor, Stuart A.; Halligan, Steve

    2013-01-01

    To describe the MR imaging findings in adults presenting with anal incontinence following pull-through perineoplasty for anorectal atresia. 15 adults (12 male, 3 female; age 22-52 years) with anal incontinence following a prior perineal pull-through procedure as an infant for anorectal atresia were identified retrospectively. MR imaging was performed using either an endoanal coil or body coil. MR images were reviewed by three observers who noted whether pelvic floor and sphincter muscles were present and, if so, whether they were thinned or not. Data were tabulated and raw frequencies determined. Images were unavailable for one patient, leaving 14 for analysis. Anal stenosis prevented endoanal coil placement in 5. The pull-through was anatomically correct in 12 (86 %) patients but was misdirected in 2. Thinned muscle was seen in 11 (79 %) patients. External sphincter thinning was commonest (present in 10 patients), with levator plate thinning least common (present in 4 patients). Only one patient had thinning of all muscle groups. MR imaging may be used to determine the extent and quality of residual pelvic floor and anal sphincter muscle in adults who have functional disability following pull-through perineoplasty for anorectal agenesis. (orig.)

  14. Anatomic measurements of the posterior tympanum related to the round window vibroplasty in congenital aural atresia and stenosis patients.

    Science.gov (United States)

    Chen, Keguang; Yin, Dongming; Lyu, Huiying; Yang, Lin; Zhang, Tianyu; Dai, Peidong

    2016-01-01

    With the aggravation of the external auditory canal malformation, the size of extra-niche fossa became smaller, providing concrete data and valuable information for the better design, selecting and safer implantation of the transducer in the area of round window niche. Three-dimensional measurements and assessments before surgery might be helpful for a safer surgical approach and implantation of a vibrant soundbridge. The aim of this study was to investigate whether differences exist in the morphology of the posterior tympanum related to the round window vibroplasty among congenital aural atresia (CAA), congenital aural stenosis (CAS), and a normal control group, and to analyze its effect on the round window implantation of vibrant soundbridge. CT images of 10 normal subjects (20 ears), 27 CAS patients (30 ears), and 25 CAA patients (30 ears) were analyzed. The depth and the size of outside fossa of round window niche related to the round window vibroplasty (extra-niche fossa)and the distances between the center of round window niche and extra-niche fossa were calculated based on three-dimensional reconstruction using mimics software. Finally, the data were analyzed statistically. The size of extra-niche fossa in the atresia group was smaller than in the stenosis group (p < 0.05); furthermore, the size of extra-niche fossa in the stenosis group was smaller than that of the control group (p < 0.05). There was no statistically significant difference of the depth of extra-niche fossa among different groups.

  15. THE RESTRICTED SURGICAL RELEVANCE OF MORPHOLOGIC CRITERIA TO CLASSIFY SYSTEMIC-PULMONARY COLLATERAL ARTERIES IN PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT

    NARCIS (Netherlands)

    DERUITER, MC; GITTENBERGERDEGROOT, AC; BOGERS, AJJC; ELZENGA, NJ

    1994-01-01

    Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic

  16. Videojuego con Realidad Virtual

    OpenAIRE

    González Mora, César

    2017-01-01

    El objetivo del proyecto es el desarrollo de un videojuego deportivo que utilice realidad mixta. El videojuego se podrá utilizar con dispositivos de tipo cardboard, y utilizará realidad aumentada para la interacción del jugador con el videojuego. En el desarrollo se utilizará el motor Unity para conseguir una aplicación multiplataforma, y la librería Vuforia para implementar realidad mixta.

  17. Sistemas integrados con Arduino

    OpenAIRE

    EL YAKOUTI, MOHAMMED

    2017-01-01

    Design of a robot prototype remotely controllable from Bluetooth using Arduino. Control and testing of sensors and events interacting with Arduino and Bluetooth. Diseño de un prototipo de robot controlable remotamente con Bluetooth utilizando Arduino. Control y verificación de los sensores y eventos que interactúan mediante el Arduino y el Bluetooth. El Yakouti, M. (2017). Sistemas integrados con Arduino. http://hdl.handle.net/10251/89274. TFGM

  18. An assessment of quality of life of operated cases of esophageal atresia in the community

    Directory of Open Access Journals (Sweden)

    Harshjeet Singh Bal

    2016-01-01

    Full Text Available Aims: To evaluate the outcome of the operated children of esophageal atresia (EA focusing on their early and late morbidity and mortality and quality of life (QoL of survivors. Settings and Design: A cross-sectional follow-up with retrospective analysis of available medical and surgical records of children who underwent repair for EA. Materials and Methods: The medical records of the children who underwent repair for EA during the period from 2000 to 2011 at the Christian Medical College Hospital, Vellore, were collected retrospectively. Patients with parents were invited to visit the hospital for follow-up and nutritional status, digestive and respiratory symptoms, status of associated anomalies and QoL assessment of children done. QoL assessment was done using the PedsQL™ 4.0 generic core scales questionnaire comprising 4 scale scores: physical, emotional, social functioning, and school functioning. Mean scores are calculated based on a 5-point response scale for each item and transformed to a 0-100 scale with a higher score representing better QoL. Statistical Analysis Used: Statistical Package for Social Sciences (SPSS version 16 using Chi-square or Fisher′s exact test. Results: Of 79 patients operated during the said period, there were 10 deaths and a total of 69 (87% children survived. Of the 66 patients available for follow-up, we interviewed 30 parents and children while for the remaining 36 children, out-patients charts were reviewed retrospectively. Mean follow-up duration was 3.56 years. The height and weight for age measurement showed 47% and 56% of children respectively as below the 5 th percentile. Main problems faced by operated EA children were of the respiratory (26% and gastroesophageal (36% tracts. In spite of the mentioned problems faced, the overall QoL of this group appeared good. In 23 of 30 patients, who answered PedsQL™ , more than 70% had scores >85 out of 100 in QoL scoring. Conclusions: While survivals of the

  19. Investigando con personas con dificultades de aprendizaje

    Directory of Open Access Journals (Sweden)

    Borja González Luna

    2013-12-01

    Full Text Available El artículo muestra los orígenes de lo que Walmsley (2008 denomina «investigación inclusiva». Para comprender qué se entiende por investigación inclusiva tenemos que remontarnos a los debates epistemológicos sobre las metodologías cuantitativas y cualitativas, acontecidos en la década de los 90, en torno a la revista Disability & Society. A partir de una síntesis de dichos debates, focalizados en el ámbito de la «discapacidad intelectual y del desarrollo», se exponen dos estrategias de colaboración con dicha población: a una aproximación etnográfica (de trabajo grupal, y b una aproximación biográfica (de trabajo individual. A continuación se esboza un posible diseño de trabajo de campo que intenta superar el paradigma cualitativo «clásico» con el objetivo de incluir a dicho colectivo más allá del rol de «sujetos de la investigación». Para finalizar se recoge el debate sobre la accesibilidad de los resultados de la investigación a los participantes en dichas investigaciones, y con ello la necesaria innovación en el ámbito de las «devoluciones» de los resultados, cuando se trata de incluir a personas que presentan limitaciones para la comprensión del lenguaje abstracto oral y/o escrito.

  20. The effects of the environmental antiandrogen vinclozolin on the induction of granulosa cell apoptosis during follicular atresia in pigs.

    Science.gov (United States)

    Knet, Malgorzata; Tabarowski, Zbigniew; Slomczynska, Maria; Duda, Malgorzata

    2014-06-01

    The aim of this study was to investigate whether the androgens testosterone and dihydrotestosterone (DHT) and the antiandrogenic fungicide vinclozolin (Vnz) exert proapoptotic effects on porcine granulosa cells (GCs), and to examine the roles of these compounds in follicular atresia. Granulosa cells isolated from pig follicles were cultured for 24 hours, and then exposed to 0.1 μM testosterone, 0.1 μM DHT, 14 μM Vnz, or the equivalent concentrations of testosterone and Vnz or DHT and Vnz for a further 24 hours. Apoptosis and necrosis of the GCs were determined via Hoechst staining and flow cytometry analyses of annexin V-stained cells. Whole porcine follicles were also exposed to the same compounds and combinations of compounds for 24 hours. The sections were stained with hematoxylin and eosin for morphologic assessments, and a Terminal deoxynucleotidyl Transferase Biotyn-dUTP Nick-End Labeling (TUNEL) assay was performed to determine the number of apoptotic cells. The progesterone and estradiol concentrations secreted into the culture media by isolated GCs and follicles were also measured. Exposure to the androgens resulted in an increased number of apoptotic GCs both in vitro and in the organotypic model. Vinclozolin exposure increased and decreased the number of necrotic and apoptotic GCs, respectively. Furthermore, compared with control follicles, those exposed to testosterone, DHT, or Vnz displayed enhanced atresia, and coadministration of Vnz attenuated the promotive effect of these androgens on atresia. Estradiol secretion was stimulated by the combination of testosterone and Vnz, whereas exposure to Vnz alone reduced it. Progesterone production declined after the combined addition of androgens and the antiandrogen. In summary, Vnz caused massive necrosis of GCs in vitro and induced apoptosis of GCs in whole follicles. The androgens testosterone and DHT enhanced these effects. The results presented here suggest that selective destruction of porcine

  1. Anuloplastia de homoenxerto pulmonar criopreservado com anel de Delrin na atresia pulmonar com comunicação interventricular Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre Croti

    2007-05-01

    Full Text Available Criança de seis anos portadora de atresia pulmonar com comunicação interventricular, submetida a correção total com um ano, empregando monoválvula de pericárdio bovino na reconstrução da via de saída do ventrículo direito. Evoluiu com importante regurgitação valvar pulmonar (RVP e disfunção do ventrículo direito. Na reoperação foi implantado homoenxerto pulmonar criopreservado (HPC com anuloplastia, utilizando anel de Delrin com o intuito de evitar distorção geométrica do conduto. Após dois anos, o ecocardiograma, semelhante ao pós-operatório imediato, demonstra RVP discreta e função ventricular direita normal, sugerindo que essa manobra pode ser utilizada como coadjuvante para otimizar o resultado do implante do HPC.A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.

  2. Giochiamo con i robot

    Directory of Open Access Journals (Sweden)

    Andrea Bonarini

    2009-01-01

    Full Text Available "Giochiamo con i robot" e' un laboratorio interattivo per grandi e piccini realizzato per l'edizione 2007 del Festival della Scienza di Genova. Lungo un percorso che va dalla telerobotica alla robotica evolutiva, il laboratorio sviluppa il tema di dare intelligenza ai robot. Questo percorso, le cui tappe sono le varie installazioni, si conclude nella "bottega" dove e' possibile costruire e programmare i propri robot o smontare e modificare quelli esposti durante il percorso didattico. I visitatori sono coinvolti in attivita' ludiche grazie alle quali possonoentrare in contatto con alcune delle idee potenti della robotica,

  3. disegnare con ... Alberto Pratelli

    Directory of Open Access Journals (Sweden)

    Roberto Mingucci

    2017-12-01

    Full Text Available Con questa breve intervista ad Alberto Pratelli, (non a caso scelto per aprire questa nuova rubrica intendia-mo inaugurare un dialogo con personalità significati-ve del Disegno di Architettura, che consenta riflessioni dedicate alle sue varie dimensioni, oggi più che mai da approfondire. La suggestione a farlo, viene da un’idea di Pablo Rodri-guez Navarro ed abbiamo quindi pensato di avviarla proprio in questo numero, che Pablo ha accettato di curare su un tema a lui particolarmente caro.

  4. Pulmonary arteriography by digital subtraction angiographic method in cyanotic heart disease with pulmonary stenosis or pulmonary atresia

    International Nuclear Information System (INIS)

    Kobayashi, Junjiro; Hirose, Hajime; Nakano, Susumu

    1985-01-01

    Pulmonary arteriography was performed by digital subtraction angiographic (DSA) method in 10 patients with cyanotic heart disease associated with pulmonary stenosis or pulmonary atresia. Ten patients consisted of five patients with tetralogy of Fallot, three with single ventricle and pulmonary stenosis, and two with pseudotruncus arteriosus. Hepato-clavicular position was taken in four patients. Pulmonary artery and its main branches were opacified and recognized clearly, and their diameter could be measured accurately with a small amount of contrast medium. There was a good correlation between the diameter of pulmonary artery measured by DSA and that measured by conventional pulmonary arteriography. DSA is a useful method for evaluating the size and the stenosis of pulmonary artery especially in small cyanotic infants. (author)

  5. Custom-made covered transjugular intrahepatic portosystemic shunt (TIPS) in an infant with trisomy 22 and biliary atresia

    International Nuclear Information System (INIS)

    Chlapoutaki, Chrysanthi Emmanouil; Franchi-Abella, Stephanie; Pariente, Daniele; Habes, Dalila

    2009-01-01

    We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results. (orig.)

  6. Custom-made covered transjugular intrahepatic portosystemic shunt (TIPS) in an infant with trisomy 22 and biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Chlapoutaki, Chrysanthi Emmanouil; Franchi-Abella, Stephanie; Pariente, Daniele [Bicetre Hospital University Paris XI, Assistance Publique Hopitaux de Paris, Department of Paediatric Radiology, Paris (France); Habes, Dalila [Bicetre Hospital University Paris XI, Assistance Publique Hopitaux de Paris, Pediatric Hepatology and National Reference Center for Biliary Atresia, Paris (France)

    2009-07-15

    We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results. (orig.)

  7. Duodenal atresia in 17q12 microdeletion including HNF1B: a new associated malformation in this syndrome.

    Science.gov (United States)

    Quintero-Rivera, Fabiola; Woo, Jennifer S; Bomberg, Eric M; Wallace, W Dean; Peredo, Jane; Dipple, Katrina M

    2014-12-01

    Deletions of chromosome 17q12 [OMIM 614527] encompass a wide range of phenotypes, including renal cysts, diabetes mellitus, pancreatic structural abnormalities, genital tract anomalies, developmental delay, learning difficulties, and more recently, autism spectrum disorder and schizophrenia. To date, gastrointestinal malformations have not been fully characterized in this syndrome. In this case report, we describe a four-year-old girl with a 17q12 microdeletion who was born with duodenal atresia, bilateral renal cysts, left kidney dysplasia, a midline cystic structure at the conus medullaris, and dysmorphic features. Both the patient and her affected father were found to have a deletion of 17q12, which encompasses the HNF1B (hepatocyte nuclear factor beta). It is hypothesized that HNF1B may play a role in intestinal differentiation and development. Our clinical report further expands the pre-and post-natal presentation of this rare microdeletion syndrome. © 2014 Wiley Periodicals, Inc.

  8. Estado nutricional de pacientes com atresia biliar e hepatite autoimune e relação com os níveis séricos de vitaminas A, D e E

    OpenAIRE

    Saron, Margareth L. G.; Godoy, Helena T.; Hessel, Gabriel

    2009-01-01

    CONTEXT: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients...

  9. The natural insect peptide Neb-colloostatin induces ovarian atresia and apoptosis in the mealworm Tenebrio molitor.

    Science.gov (United States)

    Czarniewska, Elżbieta; Rosiński, Grzegorz; Gabała, Elżbieta; Kuczer, Mariola

    2014-01-30

    The injection of Neb-colloostatin into T. molitor females causes gonadoinhibitory effects on ovarian development. This peptide inhibits intercellular space formation (patency) in follicular epithelium and results in slowed vitellogenesis, delayed ovulation, reduced number of eggs laid and presumably cell death in the terminal follicles. However, as does the form of cell death in the terminal follicle, the mode of action of Neb-colloostatin remains unknown. We tested Neb-colloostatin for a sterilizing effect on females of Tenebrio molitor. We report that injection of nanomolar doses of Neb-colloostatin induce ovarian follicle atresia in 4-day old females during their first gonadotropic cycle. Light microscope observations revealed morphological changes in the ovary: after Neb-colloostatin injection the terminal oocytes are significantly smaller and elicit massive follicle resorption, but the control terminal follicles possess translucent ooplasm in oocytes at different stages of vitellogenesis. A patency is visible in follicular epithelium of the control vitellogenic oocytes, whereas peptide injection inhibits intercellular space formation and, in consequence, inhibits vitellogenesis. Confocal and electron microscope examination showed that peptide injection causes changes in the morphology indicating death of follicular cells. We observed F-actin cytoskeleton disorganization, induction of caspase activity, changes in chromatin organization and autophagic vacuole formation. Moreover, the apical cytoplasm of follicular cells is filled with numerous free ribosomes, probably indicating a higher demand for protein biosynthesis, especially in preparation for autophagic vacuole formation. On the other hand, the process of polyribosomes formation is inhibited, indicating the contributing effect of this hormone. Neb-colloostatin induces atresia in the mealworm ovary. Degeneration of T. molitor follicles includes changes in morphology and viability of follicular cells, and

  10. The natural insect peptide Neb-colloostatin induces ovarian atresia and apoptosis in the mealworm Tenebrio molitor

    Science.gov (United States)

    2014-01-01

    Background The injection of Neb-colloostatin into T. molitor females causes gonadoinhibitory effects on ovarian development. This peptide inhibits intercellular space formation (patency) in follicular epithelium and results in slowed vitellogenesis, delayed ovulation, reduced number of eggs laid and presumably cell death in the terminal follicles. However, as does the form of cell death in the terminal follicle, the mode of action of Neb-colloostatin remains unknown. Results We tested Neb-colloostatin for a sterilizing effect on females of Tenebrio molitor. We report that injection of nanomolar doses of Neb-colloostatin induce ovarian follicle atresia in 4-day old females during their first gonadotropic cycle. Light microscope observations revealed morphological changes in the ovary: after Neb-colloostatin injection the terminal oocytes are significantly smaller and elicit massive follicle resorption, but the control terminal follicles possess translucent ooplasm in oocytes at different stages of vitellogenesis. A patency is visible in follicular epithelium of the control vitellogenic oocytes, whereas peptide injection inhibits intercellular space formation and, in consequence, inhibits vitellogenesis. Confocal and electron microscope examination showed that peptide injection causes changes in the morphology indicating death of follicular cells. We observed F-actin cytoskeleton disorganization, induction of caspase activity, changes in chromatin organization and autophagic vacuole formation. Moreover, the apical cytoplasm of follicular cells is filled with numerous free ribosomes, probably indicating a higher demand for protein biosynthesis, especially in preparation for autophagic vacuole formation. On the other hand, the process of polyribosomes formation is inhibited, indicating the contributing effect of this hormone. Conclusion Neb-colloostatin induces atresia in the mealworm ovary. Degeneration of T. molitor follicles includes changes in morphology and

  11. Review of oesophageal atresia and tracheoesophageal fistula in hospital sultanah bahiyah, alor star. Malaysia from january 2000 to december 2009.

    Science.gov (United States)

    Narasimman, S; Nallusamy, M; Hassan, S

    2013-01-01

    Oesophageal atresia (EA) and tracheoesophageal fistula (TEF) is one of the congenital anomaly occurring in the newborns with the incidence of 1 in 2500 births seen worldwide. A retrospective review of newborns admitted to Hospital Sultanah Bahiyah (HSB) from 1st January 2000 to 31st December 2009 was done. The objective was to look at the influence of birth weight, time of surgical intervention, presence of other congenital anomaly and presence of preoperative pneumonia to the immediate outcome (mortality) of the surgery. There were 47 patients with oesophageal atresia, out of which 26 (55%) were males and 21 (45%) females. The distribution of patients by race were 34 Malays (72%), 9 Chinese (19%) and 4 Indians (9%). The birth weight of the babies range from 0.8 kg to 4.0 kg and there was a significant association with the outcome of the surgery (p< 0.05). Most of the babies (20) were operated within 24 hours of presentation but there was no significant association to the outcome. 23 (49%) of them were born with congenital malformation and there was a significant association with the outcome of the surgery (p<0.05). Based on the chest roentgenogram, 20 (43%) of them had pneumonia with significant association with the outcome (p<0.05). The mortality rate is 23% and the causes of death were pneumonia (36%), renal failure (18%), cardiac malformation (18%) and multiple congenital malformations (28%). The outcome of EA and TEF is determined mainly by birth weight, congenital malformations and presence of preoperative pneumonia in HSB.

  12. Esophageal atresia associated with anorectal malformation: Is the outcome better after surgery in two stages in a limited resources scenario?

    Directory of Open Access Journals (Sweden)

    Sunita Singh

    2012-01-01

    Full Text Available Aims: To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF associated with anorectal malformation (ARM can be improved by doing surgery in 2 stages. Materials and Methods : A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed. Results: Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11 were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70 neonates had VACTERL association and 8.6% (6/70 neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70. The survival was 45% (9/20 in neonates operated in a single stage and 53.3% (16/30 when operated in 2 stages (P = 0.04. Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively. The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively. This was further supported by stepwise logistic regression analysis. Conclusions: In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control.

  13. Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study.

    Science.gov (United States)

    Ng, Vicky L; Sorensen, Lisa G; Alonso, Estella M; Fredericks, Emily M; Ye, Wen; Moore, Jeff; Karpen, Saul J; Shneider, Benjamin L; Molleston, Jean P; Bezerra, Jorge A; Murray, Karen F; Loomes, Kathleen M; Rosenthal, Philip; Squires, Robert H; Wang, Kasper; Arnon, Ronen; Schwarz, Kathleen B; Turmelle, Yumirle P; Haber, Barbara H; Sherker, Averell H; Magee, John C; Sokol, Ronald J

    2018-05-01

    To assess neurodevelopmental outcomes among participants with biliary atresia with their native liver at ages 12 months (group 1) and 24 months (group 2), and to evaluate variables predictive of neurodevelopmental impairment. Participants enrolled in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with either the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition. Scores (normative mean = 100 ± 15) were categorized as ≥100, 85-99, and Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition, scales) was analyzed using logistic regression. There were 148 children who completed 217 Bayley Scales of Infant and Toddler Development, 3rd edition, examinations (group 1, n = 132; group 2, n = 85). Neurodevelopmental score distributions significantly shifted downward compared with test norms at 1 and 2 years of age. Multivariate analysis identified ascites (OR, 3.17; P = .01) and low length z-scores at time of testing (OR, 0.70; P cognitive/language impairment at 1 year of age. An unsuccessful hepatoportoenterostomy was predictive of both physical/motor (OR, 4.88; P cognitive/language impairment (OR, 4.76; P = .02) at 2 years of age. Participants with biliary atresia surviving with native livers after hepatoportoenterostomy are at increased risk for neurodevelopmental delays at 12 and 24 months of age. Those with unsuccessful hepatoportoenterostomy are >4 times more likely to have neurodevelopmental impairment compared with those with successful hepatoportoenterostomy. Growth delays and/or complications indicating advanced liver disease should alert clinicians to the risk for neurodevelopmental delays, and expedite appropriate interventions. Clinicaltrials.gov: NCT00061828 and NCT00294684. Copyright © 2018 Elsevier Inc. All rights reserved.

  14. Conversando con Oriol Bohigas

    OpenAIRE

    Redondo Domínguez, Ernesto; Moya Sala, Joaquim

    2015-01-01

    [EN] Interview with Oriol Bohigas [ES] Entrevista con Oriol Bohigas Redondo Domínguez, E.; Moya Sala, J. (2015). Conversando con… Oriol Bohigas. EGA. Revista de Expresión Gráfica Arquitectónica. 20(26):22-35. doi:10.4995/ega.2015.4061 22 35 20 26

  15. DR Con o:

    African Journals Online (AJOL)

    which could fall under the Ugandan influence. The con-. flict in the ..... The Congolese people and international community within SADC, the AU ..... ments and make peace among themselves. However, one ... friends overnight.There is a great ...

  16. fertilizada con diferentes abonos

    Directory of Open Access Journals (Sweden)

    Jorge Alberto Elizondo-Salazar

    2007-01-01

    Full Text Available Producción y calidad de la biomasa de morera (Morus alba fertilizada con diferentes abonos. Se llevó a cabo un experimento en la Estación Experimental “Alfredo Volio Mata” de la Universidad de Costa Rica con el fi n de evaluar la aplicación de 150 kg de N/ha/año proveniente de dos abonos orgánicos: lombriabono y compostaje; y de un fertilizante químico, sobre la producción y calidad de la biomasa de morera. El periodo experimental comprendió un ciclo de 12 meses, iniciando en julio del 2003 y fi nalizando en julio del 2004. Se utilizó una plantación de morera de 12 años de establecida con una densidad de siembra de 27.777 plantas/ ha. Se empleó un diseño de bloques completos al azar con cuatro tratamientos: dos abonos orgánicos, nitrato de amonio (33,5% N y un control. Las plantas se podaron a 0,6 m sobre el nivel del suelo al inicio del ensayo. Durante el periodo experimental, las plantas fueron podadas consecutivamente cada 90 días. Las hojas y los tallos fueron separados y analizados para determinar el contenido de materia seca y proteína cruda. La producción de materia seca fue 23% superior y el contenido de proteína cruda fue signifi cativamente mayor con el nitrógeno químico, mientras que el contenido de materia seca fue menor. No se encontraron diferencias signifi cativas entre el tratamiento control y los tratamientos orgánicos.

  17. Successful vaginal delivery at term after vaginal reconstruction with labium minus flaps in a patient with vaginal atresia: A rare case report.

    Science.gov (United States)

    Liu, Yu; Wang, Yi-Feng

    2017-07-01

    We report a case of successful vaginal delivery after vaginal reconstruction with labium minus flaps in a 23-year-old patient with congenital vaginal atresia. The patient primarily presented with amenorrhea and cyclic abdominal pain; transabdominal ultrasonography revealed an enlarged uterus due to hematometra and absence of the lower segment of the vagina. Eight years ago, she had undergone an unsuccessful attempt at canalization at a local hospital. Upon referral to our hospital, she underwent vaginal reconstruction with labium minus flaps. Four months after this procedure, she became pregnant and, subsequently, successfully and safely vaginally delivered a healthy female baby weighing 3250 g at 38 +1 weeks' gestation. The delivery did not involve perineal laceration by lateral episiotomy. To the best of our knowledge, this is the first reported case of successful vaginal delivery at term after vaginal reconstruction with labium minus flaps in a patient with vaginal atresia. © 2017 Japan Society of Obstetrics and Gynecology.

  18. Characterization of patients with esophageal atresia of the Servicio de Cuidados Intensivos Neonatales of the Hospital Nacional de Ninos, during the period from January 2008 to December 2012

    International Nuclear Information System (INIS)

    Ramirez Gutierrez, Hannia

    2013-01-01

    A population of 61 patients discharged of the Servicio de Cuidados Intensivos Neonatales of the Hospital Nacional de Ninos with the diagnosis of esophageal atresia, associated anomalies, medical-surgical management and complications was describing during the period from 2008 to 2012. The patients had complementary studies such as chest x-ray, esophagogram, echocardiogram and gastrointestinal transit. Esophageal atresia can be diagnosed prenatally by ultrasound. According to the study, the absence of prenatal ultrasound data in the files and failure to perform a routine morphological ultrasound to pregnant women to rule out malformations or defects in prenatal care, were considered as possible causes for the poor prenatal diagnosis. The most frequent Gross AE is type C, as noted in world literature. Cardiac malformations are the most frequent. Early and late complications according to the type of surgical approach (extrapleural and transpleural) were compared and described. The most frequent surgical complications were presented in the transpleural surgical approach [es

  19. Acurácia diagnóstica do espessamento ecogênico periportal à ultra-sonografia e da histopatologia no diagnóstico diferencial da atresia biliar Accuracy of echogenic periportal enlargement image in ultrasonographic exams and histopathology in differential diagnosis of biliary atresia

    Directory of Open Access Journals (Sweden)

    Mariza L. V. Roquete

    2008-08-01

    Full Text Available OBJETIVOS: Definir a sensibilidade, especificidade e a acurácia do espessamento ecogênico periportal à ultra-sonografia e da histopatologia hepática, isolados ou em conjunto, na distinção diagnóstica entre atresia biliar e as colestases intra-hepáticas. MÉTODOS: Trata-se de estudo retrospectivo realizado entre janeiro de 1990 e dezembro de 2004. Foram analisados 51 casos de atresia biliar e 45 com colestase intra-hepática. A histopatologia foi realizada por uma patologista de forma cega. O espessamento ecogênico periportal foi pesquisado na ultra-sonografia como único sinal diagnóstico de atresia biliar. Foram calculados os índices de sensibilidade, especificidade e acurácia do espessamento ecogênico periportal e da histologia isoladamente ou associados. O padrão-ouro utilizado para o diagnóstico de atresia biliar foi o aspecto da via biliar extra-hepática à laparotomia. RESULTADOS: O espessamento ecogênico periportal revelou sensibilidade de 49%, especificidade de 100% e acurácia de 72,5%. A histopatologia compatível com obstrução biliar extra-hepática conferiu sensibilidade de 90,2%, especificidade de 84,6% e acurácia de 87,8%. O espessamento ecogênico periportal e a histopatologia isolados ou associados proporcionaram sensibilidade de 93,2%, especificidade de 85,7% e acurácia de 90,3%. CONCLUSÕES: A evidência do espessamento ecogênico periportal na ultra-sonografia é indicação de laparotomia. Se o espessamento ecogênico periportal é negativo, está indicada a biopsia hepática; se a histopatologia revelar sinais de atresia biliar, impõe-se a laparotomia exploradora. Nos casos de espessamento ecogênico periportal negativo com histopatologia de hepatite neonatal ou de outras colestases intra-hepáticas, recomenda-se o acompanhamento ou o tratamento clínico conforme o diagnóstico.OBJECTIVES: To define the sensitivity, specificity and accuracy of the ultrasound triangular cord sign and hepatic

  20. Remarkable case of uncorrected type IC tricuspid atresia with adaptive pulmonary trunk dilatation to allow prolonged survival: Case report and CT fly-through

    Directory of Open Access Journals (Sweden)

    Zeke J. McKinney

    2017-06-01

    Full Text Available A remarkable case of a surgically uncorrected Type IC (no great artery transposition, no pulmonary stenosis tricuspid atresia surviving to adulthood is presented. This is a case of an adult female of 30 years of age with an atretic tricuspid valve, an atrial septal defect, a large ventricular septal defect, and a dilated pulmonary trunk. Surgical correction was never conducted on this heart with a significant congenital cardiac defect, and yet the individual survived into a fourth decade. Without surgical correction, survival to adulthood in tricuspid atresia is exceedingly rare. Survival depends on a high degree of ventricular shunting with limited pulmonary outflow obstruction. The resistance of this obstruction must be both low enough to maintain increased pulmonary blood flow and high enough to prevent systemic-level pressures upon the pulmonary vasculature. The unique finding of a significantly dilated pulmonary trunk is described, which presumably allowed this individual to survive to adulthood. This complex physiology is described and augmented with high-resolution images of this cardiac specimen. These were made possible by the application of a plastination process prior to obtaining high-resolution multi-slice computed tomography imaging studies. The result is a study comprised of multiplanar and three-dimensional data detailing the anatomic features of the specimen, which were then used to create reconstructions of the specimen for visualization of the anatomy. An understanding of the development and morphology of tricuspid atresia is essential for successful surgical correction in new patients with this congenital malformation. Keywords: Tricuspid atresia, Pulmonary blood flow, Pulmonary hypertension, Plastination, CT

  1. Anal Atresia Associated to Urethrorectal Fistula in a Giant Anteater Myrmecophaga Tridactyla at the Ocarro´S Bio Park (Villavicencio-Colombia

    Directory of Open Access Journals (Sweden)

    Rosa María Viviana Gómez-Carrillo

    2013-05-01

    Full Text Available Congenital malformations have been found in humans and in some domestic species, however in wild species, reports are limited. The knowledge of these illnesses in wild fauna has not been documented; neither it is epidemiological behavior or casuistic level. The case presented in this article was presented in a neonatal female who belongs to the Myrmecophaga tridactyla species. Specifically, this wild animal was born in the Ocarros Biopark, Villavicencio- Colombia, their final diagnosis was type I anal atresia.

  2. pacientes con falla cardiaca

    Directory of Open Access Journals (Sweden)

    Diana Marcela Achury Saldaña

    2007-01-01

    Full Text Available Objetivo: determinar la adherencia al tratamiento de pacientes con falla cardiaca hospitalizados, al aplicar un plan educativo quefomenta el autocuidado.Método: estudio cuasiexperimental (entrevistas enfermera-paciente realizado entre diciembre de 2004 y mayo de 2006, con unamuestra de 50 pacientes seleccionados por conveniencia. Se diseñó un instrumento para evaluar los comportamientos de los pacientes,con base en algunos resultados de la adherencia y sus respectivos indicadores de la taxonomía NOC (Nursing out comes classification. Laadherencia al tratamiento fue medida en dos momentos: el primero durante la hospitalización, seguido de la aplicación del plan educativoantes del alta, que proporcionaba información en el manejo de su enfermedad desde una dimensión física, psicológica y social quepromueve el autocuidado; y el segundo un mes después del alta en su domicilio.Resultados: diferencias estadísticamente significativas (P=0,0001 que demuestran cómo mediante la capacitación al paciente enel manejo de su tratamiento farmacológico y no farmacológico, el establecimiento de una sana relación entre el profesional de enfermeríay el paciente, y la participación de la familia, se logra una total adherencia al tratamiento.Conclusiones: para lograr una adherencia total del paciente con falla cardiaca al tratamiento es necesario un proceso educativo y unseguimiento continuo y personalizado que motive permanentemente al paciente y se le reconozca el papel protagónico en su cuidado y manejo de la enfermedad.

  3. Dientes supernumerarios y atresia maxilar: Tratamiento orto-quirúrgico en Odontopediatría. Relato de un caso clínico: 5 años de seguimiento

    Directory of Open Access Journals (Sweden)

    MS Sampaio

    Full Text Available Se describe a los dientes supernumerarios o hiperodoncia como el aumento del número de órganos dentarios comparado con la dentición normal. Todos los dientes supernumerarios deben ser extraídos, respetando el momento ideal para su remoción quirúrgica, evaluando siempre el momento oportuno para intervenir. En el presente trabajo las autoras presentan un caso clínico de una paciente de sexo femenino, que tuvo un seguimiento durante cinco años a partir de los 6 meses de edad asistida en el Curso de Extensión universitaria Bebé Clínica de la Universidad Federal de Río Grande del Sur. Durante sus visitas periódicas de revisión odontológica correspondientes al programa de la Bebé Clínica, se diagnosticó clínicamente la presencia de mordida cruzada anterior, atresia maxilar y radiográficamente la presencia de dos elementos supernumerarios. Frente a este diagnóstico, es necesario un planteamiento multidisciplinario, o sea, orto-quirúrgico para la realización del tratamiento, destacando una vez más, la importancia de la atención odontológica del bebé y el diagnóstico precoz de posibles alteraciones como en el caso clínico en cuestión; la presencia de elementos supernumerarios y maloclusión dentaria

  4. Cementos con cenizas volantes

    Directory of Open Access Journals (Sweden)

    Ossa M., Mauricio

    1984-03-01

    additions of 20 and 30% .

    Casi la generalidad de los estudios realizados sobre cementos con adición de cenizas volantes se refieren a sus características y comportamiento en pastas, morteros y hormigones, siempre en relación con aquéllos del cemento portland. Esta vez, se desarrolló un trabajo experimental orientado a relacionar entre sí los cementos con adiciones de cenizas volantes y de puzolana natural. Para ello se fabricaron a escala de laboratorio cementos de ambos tipos, empleando como materias primas comunes clinker y yeso y, como variables, diferentes porcentajes de las dos adiciones, que cumplieron previamente los requisitos normalizados en cuanto a sus actividades puzolánicas. La calidad de los cementos fabricados resultó adecuada y concordante con la del cemento portland-puzolánico obtenido a escala industrial con los mismos clinker, yeso y puzolana natural de este estudio. Posteriormente, se determinaron las características de los cementos experimentales y se confeccionaron morteros normales para la realización de ensayos físicos y mecánicos. Los resultados de ensayos indicaron que los cementos con adición de cenizas volantes (CCV requieren menos agua para consistencia normal, presentan tiempos de fraguado mayores y expansiones en autoclave menores que los cementos con adición de puzolana (CP. Los calores de hidratación a 7 y 28 días de edad fueron aproximadamente similares para ambos tipos de cemento. En morteros normales, los cementos CCV mostraron menor retracción de secado, mayor retentividad y mayor fluidez (para igual cantidad de agua que los cementos CP. En los ensayos de exudación se observó que ésta depende más de la finura que el tipo de adición. Finalmente, los ensayos mecánicos señalaron que las resistencias a compresión y flexotracción de los morteros con cementos CCV son menores a edades inferiores que 14 días (del orden de 5 a 10% a un día de edad, pero que a partir de entonces pasan a ser mayores que las de

  5. competencia con China

    Directory of Open Access Journals (Sweden)

    Elena de la Paz Hernández Águila

    2007-01-01

    Full Text Available El artículo ofrece un diagnóstico del desempeño de la industria mexicana del calzado desde la década de los ochenta hasta la actualidad. Analiza la problemática que ha enfrentado esta rama industrial a partir del proceso de apertura comercial y de la competencia en su mercado interno con productos provenientes de países asiáticos, particularmente China. Problematiza al respecto los retos y las perspectivas que a mediano plazo enfrentará este sector empresarial y sobre las posibilidades de competir en el mercado globalizado.

  6. Construir con Madera

    OpenAIRE

    Olabe-Velasco, F. (Fermín); Val-Hernández, Y. (Yolanda); Varela-de-la-Cruz, P. (Perla); Cabrero-Ballarín, J.M. (José Manuel)

    2010-01-01

    Guía divulgativa ‘Construir con madera’, elaborada por la Cátedra Madera de la Universidad de Navarra y el Gobierno de Navarra. La publicación pretende explicar de forma sencilla los beneficios y posibilidades de este material en la construcción, tanto en lo que respecta a su resistencia, comportamiento frente al fuego, durabilidad, capacidad de aislamiento, propiedades acústicas, estética, respeto al medio ambiente y sostenibilidad como fuente de energía. A modo de ejemplo, en la ...

  7. Nutritional status of patients with biliary atresia and autoimmune hepatitis related to serum levels of vitamins A, D and E Estado nutricional de pacientes com atresia biliar e hepatite autoimune e relação com os níveis séricos de vitaminas A, D e E

    Directory of Open Access Journals (Sweden)

    Margareth L. G. Saron

    2009-03-01

    Full Text Available CONTEXT: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients underwent anthropometric evaluation, alimentary inquiry and determination of serum levels of vitamins A, D and E by high performance liquid chromatography. The Mann-Whitney test, Spearman correlation coefficients and variance analysis (ANOVA were utilized for data treatment, regarding significant difference if PCONTEXTO: As doenças hepáticas crônicas podem induzir à má absorção de lipídios e vitaminas lipossolúveis e levar ao comprometimento do estado nutricional. OBJETIVOS: Avaliar o estado nutricional e relacionar com os níveis séricos de vitaminas (A, D e E e a gravidade da doença em pacientes com atresia biliar e hepatite autoimune na faixa etária pediátrica. MÉTODOS: O estudo foi transversal controlado e foram avaliados os pacientes com hepatite autoimune e atresia biliar e um grupo controle pareado por sexo e idade. Foi realizada avaliação antropométrica, aplicação do inquérito alimentar e determinação dos níveis séricos das vitaminas A, D e E pela técnica de cromatografia líquida de alta eficiência. Foram empregados os testes de Mann-Whitney, o coeficiente de correlação de Spearman e análise de variância (ANOVA, sendo considerada diferença significativa se P<0,05. RESULTADOS: O déficit nutricional mais grave foi observado nos pacientes com atresia biliar, principalmente com colestase. Em relação às vitaminas, no grupo controle, constatou-se que os níveis séricos das vitaminas A e E variaram com a idade. Os n

  8. Entrevista con Giovanni Levi

    Directory of Open Access Journals (Sweden)

    Monica Oliveira

    2017-06-01

    Full Text Available En esta entrevista, Giovanni Levi - como un conocedor del tema de Familia - realiza una importante evaluación sobre el actual estado de las investigaciones realizadas en el Brasil y em el exterior. Con estilo franco, agudo y lucido critica las visiones tradicionales y sus ilusiones ypropone nuevos conceptos y métodos. La historia de la familia debería ceder espacio para el estudio de las redes relacionales o de los mundos relacionales. De la misma forma, la historia cuantitativa debería abrir espacio para el estudio de las cualidades. Ya con relación a la historia de las elites, tan estudiada y reproducida en una diversidad de trabajos, que deberíase mirar en otra perspectiva. Es decir, no mirar a las reglas sociales predeterminadas, sino a los desvíos y a las variaciones. Levi defiende que los historiadores deben trascender a los documentos que se encuentran fácilmente y que pueden fortalecer perspectivas deformadas y esequilibradas de la sociedad. Para él, los historiadores deben esforzarse por estudiar a aquellos grupos que dejaron pocos rastros documentales. En ese esfuerzo existiría una nueva mirada sobre la historia de la familia.

  9. Entrevista con Patricia Ariza

    Directory of Open Access Journals (Sweden)

    Esperanza Londoño La Rotta

    2017-01-01

    Full Text Available Pensamiento, Palabra y Obra entrevista a una artista, feminista y activista política, quien como mujer y artista ha permitido pensar el arte más allá de un simple espectáculo. Toda una vida dedicada al teatro y a darle voz, a través de sus obras, a víctimas del conflicto colombiano, defensora de derechos humanos; además de hacer evidente en su vida y a través de la plataforma “Artistas por la paz”, las múltiples relaciones que se pueden establecer entre el arte, la construcción de paz y la resolución de conflictos. Hablamos en su casa, en medio del calor de la bienvenida con Patricia Ariza, directora del festival alternativo de teatro, de Mujeres en Escena y de la Corporación Colombiana de Teatro, entre otras muchas actividades que voluntariamente su espíritu libertario ha asumido. Esta entrevista se realizó antes del 2 de octubre, pero con la revisión de los acuerdos que propició el plebiscito ganado por una ínfima minoría por el no, sigue siendo vigente este planteamiento.

  10. Experiencia inicial en la cirugía cardiovascular con circulación extracorpórea en recién nacidos prematuros con un peso menor de 2.500 gramos

    Directory of Open Access Journals (Sweden)

    Christian Kreutzer

    2010-01-01

    Full Text Available RESUMENIntroducciónNo obstante el progreso sostenido en cirugía cardiovascular neonatal en los últimos 30 años,los recién nacidos prematuros con cardiopatías congénitas presentan un desafío adicional alequipo multidisciplinario interviniente debido a la frecuente asociación de comorbilidades ybajo peso. A diferencia de las estrategias históricas diferidas de diferir la cirugía, desde 2007llevamos a cabo cirugías correctoras sin importar el peso ni la edad en pacientes sintomáticoscon el propósito de mejorar su estado cardíaco para que pudieran superar las comorbilidadesasociadas.ObjetivoComunicar la experiencia inicial en cirugía cardiovascular (CCV con circulación extracorpórea(CEC en neonatos de menos de 2.500 gramos.Material y métodosDe mayo de 2007 a mayo de 2009, 11 neonatos fueron intervenidos con CEC. La edad mediafue de 24 días (4 a 90 días, la edad gestacional media fue de 34 semanas (32 a 37 y el pesomedio, de 2,27 kg (1,7 a 2,5. Previo a la intervención, siete pacientes estaban con asistenciarespiratoria mecánica (ARM y nueve con inotrópicos. Los diagnósticos fueron comunicacióninterventricular (n = 4, uno con coartación de la aorta grave, anomalía total del retornovenoso pulmonar (n = 2, transposición de grandes arterias (n = 2, atresia pulmonarcon septum íntegro (n = 1, atresia pulmonar con comunicación interventricular (n = 1 ehipoplasia del ventrículo izquierdo (n = 1. Se utilizó hipotermia profunda a 18 °C de temperaturarectal con paro circulatorio total intermitente (n = 8 o flujo completo a 28 °C detemperatura rectal (n = 3. En 10 pacientes se realizó cirugía correctora biventricular y enuno se efectuó cirugía de Norwood.ResultadosEn 8 pacientes se dejó el esternón abierto, con un tiempo medio al cierre de 3,5 días (3 a 5.Tres pacientes requirieron factor VII por presentar sangrado incoercible. El tiempo mediode ARM fue de 7,5 días (2 a 20. Las complicaciones más comunes fueron

  11. Sonic hedgehog (SHH) and glioblastoma-2 (Gli-2) expressions are associated with poor jaundice-free survival in biliary atresia.

    Science.gov (United States)

    Jung, Hae Yoen; Jing, Jin; Lee, Kyoung Bun; Jang, Ja-June

    2015-03-01

    Biliary atresia (BA) causes biliary obstruction in neonates. Although the Kasai operation can successfully treat certain BA cases, many patients exhibit recurrent jaundice and secondary biliary cirrhosis requiring liver transplantation. Consequently, studies of the prognostic factors of the Kasai operation are needed. Accordingly, sonic hedgehog (SHH) pathway expression at the extrahepatic bile duct (EHBD), an important bile duct repair mechanism, will be investigated via immunohistochemistry in patients with BA to examine the association with post-Kasai operation prognosis. Fifty-seven EHBD specimens were obtained during Kasai operations from 1992 to 2009. The SHH, patched (PTCH), and glioblastoma-2 (Gli-2) immunohistochemical staining results were analyzed quantitatively. Overall, 57.9% of patients had bile flow normalization after the Kasai operation; 43.1% did not. High preoperative serum total bilirubin, direct bilirubin, and aspartate aminotransferase levels were associated with sustained jaundice post-Kasai operation, as was an age ≥65days at the time of surgery (all pjaundice relapse. Strong Gli-2 and SHH expression in the EHBD might be a poor prognostic factor in Kasai operation-treated patients with BA. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. Heterozygous deletion of FOXA2 segregates with disease in a family with heterotaxy, panhypopituitarism, and biliary atresia.

    Science.gov (United States)

    Tsai, Ellen A; Grochowski, Christopher M; Falsey, Alexandra M; Rajagopalan, Ramakrishnan; Wendel, Danielle; Devoto, Marcella; Krantz, Ian D; Loomes, Kathleen M; Spinner, Nancy B

    2015-06-01

    Biliary atresia (BA) is a pediatric cholangiopathy with unknown etiology occurring in isolated and syndromic forms. Laterality defects affecting the cardiovascular and gastrointestinal systems are the most common features present in syndromic BA. Most cases are sporadic, although reports of familial cases have led to the hypothesis of genetic susceptibility in some patients. We identified a child with BA, malrotation, and interrupted inferior vena cava whose father presented with situs inversus, polysplenia, panhypopituitarism, and mildly dysmorphic facial features. Chromosomal microarray analysis demonstrated a 277 kb heterozygous deletion on chromosome 20, which included a single gene, FOXA2, in the proband and her father. This deletion was confirmed to be de novo in the father. The proband and her father share a common diagnosis of heterotaxy, but they also each presented with a variety of other issues. Further genetic screening revealed that the proband carried an additional protein-altering polymorphism (rs1904589; p.His165Arg) in the NODAL gene that is not present in the father, and this variant has been shown to decrease expression of the gene. As FOXA2 can be a regulator of NODAL expression, we propose that haploinsufficiency for FOXA2 combined with a decreased expression of NODAL is the likely cause for syndromic BA in this proband. © 2015 WILEY PERIODICALS, INC.

  13. Serum Mac-2 binding protein glycosylation isomer predicts grade F4 liver fibrosis in patients with biliary atresia.

    Science.gov (United States)

    Yamada, Naoya; Sanada, Yukihiro; Tashiro, Masahisa; Hirata, Yuta; Okada, Noriki; Ihara, Yoshiyuki; Urahashi, Taizen; Mizuta, Koichi

    2017-02-01

    Mac-2 Binding Protein Glycosylation Isomer (M2BPGi) is a novel fibrosis marker. We examined the ability of M2BPGi to predict liver fibrosis in patients with biliary atresia. Sixty-four patients who underwent living donor liver transplantation (LDLT) were included [median age, 1.1 years (range 0.4-16.0), male 16 patients (25.0 %)]. We examined M2BPGi levels in serum obtained the day before LDLT, and we compared the value of the preoperative M2BPGi levels with the histological evaluation of fibrosis using the METAVIR fibrosis score. Subsequently, we assessed the ability of M2BPGi levels to predict fibrosis. The median M2BPGi level in patients with BA was 6.02 (range, 0.36-20.0), and 0, 1, 1, 11, and 51 patients had METAVIR fibrosis scores of F0, F1, F2, F3, and F4, respectively. In patients with F4 fibrosis, the median M2BPGi level was 6.88 (quartile; 5.235, 12.10), significantly higher than that in patients with F3 fibrosis who had a median level of 2.42 (quartile; 1.93, 2.895, p F4 fibrosis. M2BPGi is a novel fibrosis marker for evaluating the status of the liver in patients with BA, especially when predicting grade F4 fibrosis.

  14. Detection of Epstein Barr Virus by Chromogenic In Situ Hybridization in cases of extra-hepatic biliary atresia

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    Farahmand Fatemeh

    2008-04-01

    Full Text Available Abstract Introduction Extra-hepatic biliary atresia (EHBA is an important cause of neonatal cholestasis. Several infectious agents have been proposed as etiologic factors such as Rotavirus and Reovirus. There is limited data on the role of Epstein Barr virus (EBV infection in EHBA, so we decided to study the presence of EBV virus in a series of 16 proven EHBA cases by Chromogenic in situ hybridization (CISH technique. Methods In the current study a total of 16 liver wedge biopsies of proven cases of EHBA were selected in a period of 4 years. CISH staining for EBV-encoded RNA (EBER transcript was performed. Results The review of H&E-stained slides of liver biopsies revealed fibrosis and marked ductular proliferation. In CISH-stained slides, EBV trace was observed in hepatocytes in two cases and in biliary epithelium in one case of EHBA. Discussion Considering the association of hepatitis with the Epstein-Barr virus in later life, it is likely that EBV hepatitis and its complications occur in the neonatal/perinatal period. Since EHBA is a relatively rare disease, a similar study on wedge biopsies of this number of proven cases of EHBA has not been performed to date. Current observation proposes the need for a study of larger series and employing other methods for confirming the etiologic role of EBV in EHBA cases.

  15. Amniotic fluid stem cells prevent follicle atresia and rescue fertility of mice with premature ovarian failure induced by chemotherapy.

    Science.gov (United States)

    Xiao, Guan-Yu; Liu, I-Hsuan; Cheng, Chun-Chun; Chang, Chia-Chun; Lee, Yen-Hua; Cheng, Winston Teng-Kuei; Wu, Shinn-Chih

    2014-01-01

    Chemotherapy used to treat cancer may cause irreversible premature ovarian failure (POF). Of late, amniotic fluid stem cells (AFSCs) provide a novel source for regenerative medicine because of their primitive stage, low immunogenicity, and easy accessibility. In this study, we isolated AFSCs from transgenic mice that ubiquitously express enhanced green fluorescence protein (EGFP). These AFSCs exhibited morphologies, immunophenotypes, and mesoderm trilineage differentiation potentials similar to mesenchymal stem cells (MSCs). Further, AFSCs proliferated faster than MSCs and expressed OCT4, a marker for pluripotency. To investigate their potential in recovering fertility in POF model, AFSCs were transplanted into the ovaries of mice with POF six weeks post induction using chemotherapeutic drugs, busulfan and cyclophosphamide. AFSCs could rescue the reproductive ability of mice with POF by preventing follicle atresia and sustaining the healthy follicles. Notably, the transplanted AFSCs did not differentiate into granulosa and germline cells in vivo. After one month, the decreased numbers of transplanted AFSCs accompanied with the reduced beneficial effects indicated that the therapeutic efficacy were directly from AFSCs. These findings demonstrated the therapeutic effects of AFSCs and suggested the promise of AFSCs for treating infertility and POF caused by chemotherapy.

  16. Amniotic fluid stem cells prevent follicle atresia and rescue fertility of mice with premature ovarian failure induced by chemotherapy.

    Directory of Open Access Journals (Sweden)

    Guan-Yu Xiao

    Full Text Available Chemotherapy used to treat cancer may cause irreversible premature ovarian failure (POF. Of late, amniotic fluid stem cells (AFSCs provide a novel source for regenerative medicine because of their primitive stage, low immunogenicity, and easy accessibility. In this study, we isolated AFSCs from transgenic mice that ubiquitously express enhanced green fluorescence protein (EGFP. These AFSCs exhibited morphologies, immunophenotypes, and mesoderm trilineage differentiation potentials similar to mesenchymal stem cells (MSCs. Further, AFSCs proliferated faster than MSCs and expressed OCT4, a marker for pluripotency. To investigate their potential in recovering fertility in POF model, AFSCs were transplanted into the ovaries of mice with POF six weeks post induction using chemotherapeutic drugs, busulfan and cyclophosphamide. AFSCs could rescue the reproductive ability of mice with POF by preventing follicle atresia and sustaining the healthy follicles. Notably, the transplanted AFSCs did not differentiate into granulosa and germline cells in vivo. After one month, the decreased numbers of transplanted AFSCs accompanied with the reduced beneficial effects indicated that the therapeutic efficacy were directly from AFSCs. These findings demonstrated the therapeutic effects of AFSCs and suggested the promise of AFSCs for treating infertility and POF caused by chemotherapy.

  17. Multidetector CT and MRI of ostial atresia of the coronary sinus, associated collateral venous pathways and cardiac anomalies

    International Nuclear Information System (INIS)

    Shum, J.S.F.; Kim, S.M.; Choe, Y.H.

    2012-01-01

    Aim: To analyse the multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) findings in patients with atresia of the coronary sinus orifice (CSA). Materials and methods: MDCT findings of 15 consecutive adult patients with CSAs were retrospectively analysed. The patients underwent contrast-enhanced electrocardiography-gated MDCT (n = 13) or both CT and MRI (n = 2). Results: The mean size of the coronary sinus (CS) was 14.2 mm (range 5.5–24 mm) and 11 patients (73.3%) showed CS dilatation (diameter ≥12 mm). The mean length of the atretic CS segment was 2.9 mm (range 0–8 mm). Different forms of venous collateral pathways were observed in the CSA patients. Nine (60%) of the 15 CSA patients had communication between the right atrium (RA; n = 6) or LA (n = 5) and CS via intraseptal veins; six patients (40%) had persistent left superior caval veins; communications were also observed between the CS and RA (n = 4) or LA (n = 4); two patients had collateral venous pathways between dilated cardiac veins with RA; two patients had unroofing of the CS as outlet channels. Nine patients (60%) had cardiac anomalies: coronary artery fistula to the pulmonary artery (n = 6) or left ventricular base and CS (n = 1), atrial septal defects (n = 2), and a ventricular septal defect (n = 1). Conclusion: CSA patients have venous collateral pathways and a high incidence of associated cardiovascular anomalies such as coronary artery fistulae and atrial septal defects.

  18. Comparison of environmental risk factors for esophageal atresia, anorectal malformations, and the combined phenotype in 263 German families.

    Science.gov (United States)

    Zwink, N; Choinitzki, V; Baudisch, F; Hölscher, A; Boemers, T M; Turial, S; Kurz, R; Heydweiller, A; Keppler, K; Müller, A; Bagci, S; Pauly, M; Brokmeier, U; Leutner, A; Degenhardt, P; Schmiedeke, E; Märzheuser, S; Grasshoff-Derr, S; Holland-Cunz, S; Palta, M; Schäfer, M; Ure, B M; Lacher, M; Nöthen, M M; Schumacher, J; Jenetzky, E; Reutter, H

    2016-11-01

    Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and anorectal malformations (ARM) represent the severe ends of the fore- and hindgut malformation spectra. Previous research suggests that environmental factors are implicated in their etiology. These risk factors might indicate the influence of specific etiological mechanisms on distinct developmental processes (e.g. fore- vs. hindgut malformation). The present study compared environmental factors in patients with isolated EA/TEF, isolated ARM, and the combined phenotype during the periconceptional period and the first trimester of pregnancy in order to investigate the hypothesis that fore- and hindgut malformations involve differing environmental factors. Patients with isolated EA/TEF (n = 98), isolated ARM (n = 123), and the combined phenotype (n = 42) were included. Families were recruited within the context of two German multicenter studies of the genetic and environmental causes of EA/TEF (great consortium) and ARM (CURE-Net). Exposures of interest were ascertained using an epidemiological questionnaire. Chi-square, Fisher's exact, and Mann-Whitney U-tests were used to assess differences between the three phenotypes. Newborns with isolated EA/TEF and the combined phenotype had significantly lower birth weights than newborns with isolated ARM (P = 0.001 and P studies. © 2015 International Society for Diseases of the Esophagus.

  19. Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

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    Tae Jun Park

    2014-03-01

    Full Text Available Hepatopulmonary syndrome (HPS is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE, and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%. Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

  20. Hepatic tumours in children with biliary atresia: Single-centre experience in 13 cases and review of the literature

    International Nuclear Information System (INIS)

    Yoon, H.J.; Jeon, T.Y.; Yoo, S.-Y.; Kim, J.H.; Eo, H.; Lee, S.-K.; Kim, J.S.

    2014-01-01

    Aim: To establish the risks of developing of hepatic tumours and to investigate their clinical and imaging findings in children with biliary atresia (BA) after Kasai portoenterostomy (Kasai). Materials and methods: Among 157 children who had undergone Kasai for BA over an 18 year period, patients who had newly developed hepatic tumours were identified. Patient demographics, clinical features, and imaging findings were retrospectively reviewed. Results: Three male and 10 female patients (mean age 3.9 years) all (8%, of 157) had single hepatic tumours, which were confirmed in 10 explanted and three non-explanted livers. Ten (77%) were benign and three (23%) were malignant. Of the benign hepatic tumours, focal nodular hyperplasia (FNH; n = 6) was the most common, followed by regenerative nodules (n = 3) and adenoma (n = 1). All FNH appeared in young children <1 year of age and showed a subcapsular location, bulging contour, and lack of central scar. Malignant tumours included two hepatocellular carcinomas and one cholangiocarcinoma. Conclusion: Hepatic tumours developed in approximately 8% of children with BA after Kasai. Although benign tumours, including FNHs and regenerative nodules, were more common than malignant tumours, screening with alpha-foetoprotein (AFP) levels and regular imaging studies are the mainstay of malignant tumour detection

  1. Endoscopic Stenting and Clipping for Anastomotic Stricture and Persistent Tracheoesophageal Fistula after Surgical Repair of Esophageal Atresia in an Infant

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    Mohammed Amine Benatta

    2014-01-01

    Full Text Available Anastomotic stricture (AS and recurrent tracheoesophageal fistula (TEF are two complications of surgical repair of esophageal atresia (EA. Therapeutic endoscopic modalities include stenting, tissue glue, and clipping for TEF and endoscopic balloon dilation bougienage and stenting for esophageal strictures. We report herein a two-month infant with both EA and TEF who benefited from a surgical repair for EA, at the third day of life. Two months later he experienced deglutition disorders and recurrent chest infections. The esophagogram showed an AS and a TEF confirmed with blue methylene test at bronchoscopy. A partially covered self-expanding metal type biliary was endoscopically placed. Ten weeks later the stent was removed. This allows for easy passage of the endoscope in the gastric cavity but a persistent recurrent fistula was noted. Instillation of contrast demonstrated a fully dilated stricture but with a persistent TEF. Then we proceeded to placement of several endoclips at the fistula site. The esophagogram confirmed the TEF was obliterated. At 12 months of follow-up, he was asymptomatic. Stenting was effective to alleviate the stricture but failed to treat the TEF. At our knowledge this is the second case of successful use of endoclips placement to obliterate recurrent TEF after surgical repair of EA in children.

  2. Congenital bronchial atresia (CBA). A critical review of CBA as a disease entity and presentation of a case series

    International Nuclear Information System (INIS)

    Pedicelli, Giovacchino; Ciarpaglini, Luisa Laura; De Santis, Marcello; Leonetti, Clara

    2005-01-01

    Purpose. To analyse the state of the art of diagnostic imaging in the rare disease entity known as congenital bronchial atresia (CBA) and to suggest new guidelines for diagnosis. Materials and methods. From January 2002 to December 2003 we examined 6 patients, 4 males and 2 females. Four of them complained of relapsing bronchitis; one patient was admitted with a diagnosis of pulmonary abscess. All patients underwent chest X-ray and MDCT. Results. Five patients presented at chest x-ray the typical association of hilar mass and distal parenchymal hyperinfIation that raised the suspicion of CBA; the remaining patient presented an area of parenchymal translucency that prompted us to perform MDCT, which showed severe stenosis of the segmental bronchus. CBA involved the left upper lobe (LUL) in 4 patients and the right upper lobe (RUL) in the remaining 2. In 50% of cases there were associated anomalies: distal bronchiectasis, bronchogenic cyst, anomaly of branching of bronchial tree and vascular structure. Conclusions. CBA is a relatively rare, generally oligo symptomatic malformation. Knowledge of the signs present at chest X-ray can prompt the radiologist to perform a CT scan. MDCT with multiplanar reconstruction is fundamental for the characterisation, localisation and study of the distribution of the lesion, helping to make a precise diagnosis. Surgery is seldom required and at any rate only performed in case of complications [it

  3. preescolares desnutridos con madres con obesidad y sin obesidad

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    Viridiana Vanessa Conzuelo-González

    2009-01-01

    Full Text Available El primer objetivo fue conocer cuántos menores de cinco años con diferentes grados de desnutrición tienen una madre con sobrepeso/obesidad/ en una comunidad indígena que vive en extrema pobreza y bajo condiciones de migración masculina internacional. El segundo fue comparar tres variables socionutricionales (ingreso familiar, educación de la madre y adecuación nutrimental de la dieta diaria entre estos hogares y los hogares con desnutrición infantil y madres sin obesidad. Se realizó un estudio transversal (2006-2007, en la comunidad mazahua de San Francisco Tepeolulco, Municipio de Temascalcingo; que incluyó a 85 hogares integrados por preescolares con desnutrición inscritos al programa Oportunidades. Se determinó el estado nutrición de los preescolares con indicadores antropométricos y se obtuvo el IMC de las madres de estos infantes. Se aplicó una encuesta socionutricional, incluida el recordatorio de 24 horas, y complementado con la observación participante (cualitativa. Se encontró que 83% de las madres mazahuas presentaron sobrepeso u obesidad. El estado de nutrición de los preescolares con madres con obesidad presentó un porcentaje mayor de desnutrición (76%. En la variable género, se encontró que 54% de los niños con madres con obesidad tenía baja talla. Al relacionar el nivel educativo de la madre, esta variable resultó ser estadísticamente significativa (p=0.015, donde el analfabetismo está más relacionado con la desnutrición infantil que tienen madres de bajo y/o peso normal. La elevada prevalencia de hogares conformados con preescolares con desnutrición y madres con obesidad, es un síntoma más de la pobreza en zonas indígenas en México, con bajo índice de desarrollo humano.

  4. Circulação coronária dependente do ventrículo direito na atresia pulmonar com septo interventricular íntegro. Ausência da origem das artérias coronárias da aorta Right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Absence of origin of the coronary arteries from the aorta

    Directory of Open Access Journals (Sweden)

    Ivan Romero Rivera

    1998-08-01

    Full Text Available São descritos os aspectos clínicos, ecocardiográficos e angiográficos de um neonato de sexo masculino, com cinco dias de vida e diagnóstico de atresia pulmonar com septo interventricular íntegro. Tanto o ecocardiograma como a aortografia mostraram ausência da origem das artérias coronárias da aorta. O ecocardiograma bidimensional e, posteriormente, a ventriculografia direita identificaram as artérias coronárias, originando-se no ventrículo direito. Não houve contrastação retrógrada da aorta ou do tronco pulmonar quando contrastadas as artérias coronárias. Este é o primeiro caso relatado com diagnóstico ecocardiográfico pré angiografia, e é um exemplo da necessidade de se avaliar as artérias coronárias em pacientes com atresia pulmonar e septo ventricular íntegro.This report describes the clinical, echocardiographic and angiographic aspects of a five-day old boy with pulmonary atresia and intact ventricular septum. Both the echocardiogram and the aortography did not show any coronary arteries arising from the aorta. Two-dimensional echocardiography was able to identify the coronary arteries originating from the right ventricle and so did the right ventricular angiogram. No retrograde flow into the aorta or pulmonary trunk was identified after opacification of the coronary arteries. As far as we know this is the first case diagnosed by echocardiography, and is a vivid example of the necessity of identifying the coronary arteries in patients with pulmonary atresia and intact ventricular septum.

  5. A Point Mutation in the Rhesus Rotavirus VP4 Protein Generated through a Rotavirus Reverse Genetics System Attenuates Biliary Atresia in the Murine Model.

    Science.gov (United States)

    Mohanty, Sujit K; Donnelly, Bryan; Dupree, Phylicia; Lobeck, Inna; Mowery, Sarah; Meller, Jaroslaw; McNeal, Monica; Tiao, Greg

    2017-08-01

    Rotavirus infection is one of the most common causes of diarrheal illness in humans. In neonatal mice, rhesus rotavirus (RRV) can induce biliary atresia (BA), a disease resulting in inflammatory obstruction of the extrahepatic biliary tract and intrahepatic bile ducts. We previously showed that the amino acid arginine (R) within the sequence SRL (amino acids 445 to 447) in the RRV VP4 protein is required for viral binding and entry into biliary epithelial cells. To determine if this single amino acid (R) influences the pathogenicity of the virus, we generated a recombinant virus with a single amino acid mutation at this site through a reverse genetics system. We demonstrated that the RRV mutant (RRV VP4-R446G ) produced less symptomatology and replicated to lower titers both in vivo and in vitro than those seen with wild-type RRV, with reduced binding in cholangiocytes. Our results demonstrate that a single amino acid change in the RRV VP4 gene influences cholangiocyte tropism and reduces pathogenicity in mice. IMPORTANCE Rotavirus is the leading cause of diarrhea in humans. Rhesus rotavirus (RRV) can also lead to biliary atresia (a neonatal human disease) in mice. We developed a reverse genetics system to create a mutant of RRV (RRV VP4-R446G ) with a single amino acid change in the VP4 protein compared to that of wild-type RRV. In vitro , the mutant virus had reduced binding and infectivity in cholangiocytes. In vivo , it produced fewer symptoms and lower mortality in neonatal mice, resulting in an attenuated form of biliary atresia. Copyright © 2017 American Society for Microbiology.

  6. Acurácia diagnóstica do espessamento ecogênico periportal à ultra-sonografia e da histopatologia no diagnóstico diferencial da atresia biliar

    OpenAIRE

    Roquete,Mariza L. V.; Ferreira,Alexandre R.; Fagundes,Eleonora D. T.; Castro,Lúcia P. F.; Silva,Rogério A. P.; Penna,Francisco J.

    2008-01-01

    OBJETIVOS: Definir a sensibilidade, especificidade e a acurácia do espessamento ecogênico periportal à ultra-sonografia e da histopatologia hepática, isolados ou em conjunto, na distinção diagnóstica entre atresia biliar e as colestases intra-hepáticas. MÉTODOS: Trata-se de estudo retrospectivo realizado entre janeiro de 1990 e dezembro de 2004. Foram analisados 51 casos de atresia biliar e 45 com colestase intra-hepática. A histopatologia foi realizada por uma patologista de forma cega. O es...

  7. autorregulado con estudiantes universitarios

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    Jairo Andrés Montes

    2005-01-01

    Full Text Available El propósito del presente estudio es describir la forma en la que se presentan los procesos de aprendizaje autorregulado con un grupo de estudiantes (22 estudiantes de tercer semestre de Psicología de la PUJ, Cali, en el evento de preparación para la presentación un examen. Asimismo se describen las correlaciones que ocurren entre las distintas fases de dicho proceso de autorregulación del aprendizaje. Para conseguir los objetivos propuestos se ha hecho uso de una observación de desempeño en tiempo real, es decir, de la observación durante una sesión de preparación de examen de los estudiantes, en la cual se emplearon protocolos verbales para dar cuenta de lo que «pasaba por su mente» mientras estudiaban. Una entrevista semi-estructurada y una prueba objetiva. Los resultados fueron analizados a la luz del modelo mixto de procesamiento de información y constructivismo abordado por Winne(1998. Como resultado se encontró una relación significativa entre los niveles de desempeño en el proceso de ARR y el resultado del examen. Igualmente se encontraron bajos niveles de regulación en una parte importante de la muestra y un desfase significativo entre conocimiento declarativo de ARR y desempeño en el mismo

  8. Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum

    Energy Technology Data Exchange (ETDEWEB)

    Seguela, Pierre-Emmanuel [Centre Chirurgical Marie Lannelongue, Department of Pediatric Cardiology, Le Plessis-Robinson (France); Hopital des Enfants, Toulouse Cedex 9 (France); Houyel, Lucile; Piot, Jean-Dominique [Centre Chirurgical Marie Lannelongue, Department of Pediatric Cardiology, Le Plessis-Robinson (France); Loget, Philippe [Centre Hospitalier Universitaire de Rennes, Department of Pathology, Rennes (France); Paul, Jean-Francois [Centre Chirurgical Marie Lannelongue, Department of Pediatric Radiology, Le Plessis-Robinson (France)

    2011-08-15

    We report the case of a newborn with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation. He died several weeks after a Blalock-Taussig procedure because of a progressive stenosis of the main coronary artery. We present echocardiographic and dual-source CT images of the stenosis, with autopsy correlation. To our knowledge, CT images of this quality have never been reported in a newborn. This case illustrates the extreme difficulty in prognosticating the outcome for these patients and underlines the need for a detailed neonatal coronary mapping to assess right ventricle-dependent coronary circulation. (orig.)

  9. Dioxin exposure reduces the steroidogenic capacity of mouse antral follicles mainly at the level of HSD17B1 without altering atresia

    Energy Technology Data Exchange (ETDEWEB)

    Karman, Bethany N., E-mail: bklement@illinois.edu; Basavarajappa, Mallikarjuna S., E-mail: mbshivapur@gmail.com; Hannon, Patrick, E-mail: phannon2@illinois.edu; Flaws, Jodi A., E-mail: jflaws@illinois.edu

    2012-10-01

    2,3,7,8-Tetrachlorodibenzo-p-dioxin (TCDD) is a potent ovarian toxicant. Previously, we demonstrated that in vitro TCDD (1 nM) exposure decreases production/secretion of the sex steroid hormones progesterone (P4), androstenedione (A4), testosterone (T), and 17β-estradiol (E2) in mouse antral follicles. The purpose of this study was to determine the mechanism by which TCDD inhibits steroidogenesis. Specifically, we examined the effects of TCDD on the steroidogenic enzymes, atresia, and the aryl hydrocarbon receptor (AHR) protein. TCDD exposure for 48 h increased levels of A4, without changing HSD3B1 protein, HSD17B1 protein, estrone (E1), T or E2 levels. Further, TCDD did not alter atresia ratings compared to vehicle at 48 h. TCDD, however, did down regulate the AHR protein at 48 h. TCDD exposure for 96 h decreased transcript levels for Cyp11a1, Cyp17a1, Hsd17b1, and Cyp19a1, but increased Hsd3b1 transcript. TCDD exposure particularly lowered both Hsd17b1 transcript and HSD17B1 protein. However, TCDD exposure did not affect levels of E1 in the media nor atresia ratings at 96 h. TCDD, however, decreased levels of the proapoptotic factor Bax. Collectively, these data suggest that TCDD exposure causes a major block in the steroidogenic enzyme conversion of A4 to T and E1 to E2 and that it regulates apoptotic pathways, favoring survival over death in antral follicles. Finally, the down‐regulation of the AHR protein in TCDD exposed follicles persisted at 96 h, indicating that the activation and proteasomal degradation of this receptor likely plays a central role in the impaired steroidogenic capacity and altered apoptotic pathway of exposed antral follicles. -- Highlights: ► TCDD disrupts steroidogenic enzymes in mouse antral follicles. ► TCDD particularly affects the HSD17B1 enzyme in mouse antral follicles. ► TCDD does not affect atresia ratings in mouse antral follicles. ► TCDD decreases levels of the proapoptitic factor Bax in mouse antral follicles.

  10. [Medium-term outcome, follow-up, and quality of life in children treated for type III esophageal atresia].

    Science.gov (United States)

    Lepeytre, C; De Lagausie, P; Merrot, T; Baumstarck, K; Oudyi, M; Dubus, J-C

    2013-10-01

    The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good. Copyright © 2013. Published by Elsevier SAS.

  11. Prognostic impact of hepatobiliary scintigraphy in diagnosis and postoperative follow-up of newborns with biliary atresia

    International Nuclear Information System (INIS)

    Rossmueller, B.; Porn, U.; Dresel, S.; Hahn, K.; Schuster, T.; Lang, T.

    2000-01-01

    Aim: To investigate the prognostic relevance of hepatobiliary scintigraphy (HBS) in newborns suffering from biliary atresia (BA) for establishing the primary diagnosis and in the postoperative follow-up after portoenterostomy (Kasai). Methods: Twenty newborns with direct hyperbilirubinemia and 6 children after operative treatment of BA (Kasai) underwent HBS with Tc-99m-DEIDA. In patients without intestinal drainage, hepatocellular extraction was estimated visually and calculated semiquantitatively by means of liver/heart-ratio 5 min p.i. Results: 10/20 patients with hyperbilirubinemia did not display biliary drainage; 6 had BA, 3 intrahepatic hypoplasia, and one showed a bile plug syndrom. 4/6 with BA but none of the 4 children with diagnoses other than BA presented with a good extraction. All of the 4 children with BA, who had either pre- or postoperatively a bad extraction, needed liver transplantation due to liver failure. Both of the two newborns with BA and favourable outcome after Kasai had a good extraction in the preoperative HBS and demonstrated good intestinal drainage in the postoperative scan. Conclusion: HBS rules out BA with high accuracy by demonstrating drainage of bile into the intestine. In newborns without drainage a good extraction favours the diagnosis of BA. In newborns with BA a bad extraction seems to indicate a poor postoperative prognosis after Kasai operation. HBS might therefore help to select those children who will not benefit from portoenterostomy. Postoperatively, HBS can easily and quickly confirm the successful hepatobiliary anastomosis by demonstrating biliary drainage into the intestine. (orig.) [de

  12. Identification of rare heterozygous missense mutations in FANCA in esophageal atresia patients using next-generation sequencing.

    Science.gov (United States)

    Feng, Yu; Chen, Runsen; Da, Min; Qian, Bo; Mo, Xuming

    2018-06-30

    Esophageal atresia and tracheoesophageal fistula (EA/TEF) are relatively common malformations in newborns, but the etiology of EA/TEF remains unknown. Fanconi anemia (FA) complementation group A (FANCA) is a key component of the FA core complex and is essential for the activation of the DNA repair pathway. The middle region (amino acids 674-1208) of FANCA is required for its interaction with FAAP20. We performed targeted sequencing of this binding region of FANCA (exons 23-36) in 40 EA/TEF patients. We also investigated the effect of the p.A958V mutation on the protein-protein interaction between FANCA and FAAP20 using an in vitro binding assay and co-immunoprecipitation. Immunolocalization analysis was performed to investigate the subcellular localization of FANCA, and tissue sections and immunohistochemistry were used to explore the expression of FANCA. We identified four rare missense variants in the FANCA binding region. FANCA mutations were significantly overrepresented in EA/TEF patients compared with 4300 control subjects from the NHLBI-ESP project (Fisher's exact p = 2.17 × 10 -5 , odds ratio = 31.75). p.A958V, a novel de novo mutation in the FANCA gene, was identified in one patient with EA/TEF. We provide further evidence that the p.A958V mutation reduces the binding affinity of FANCA for FAAP20. Interestingly, the p.A958V mutation impaired the nuclear localization of the FANCA protein expressed in HeLa cells. We found that FANCA was more highly expressed in stratified squamous epithelium than in smooth muscle. In conclusion, mutations in the FANCA gene are associated with EA/TEF in humans. Copyright © 2018. Published by Elsevier B.V.

  13. Efecto de un tratamiento agudo con norgestomet en la dinámica folicular de vacas Cebú x Holstein sincronizadas con implantes de norgestomet y eCG

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    R. Páez

    2005-01-01

    Full Text Available El objetivo del presente estudio fue evaluar el efecto de una inyección de norgestomet en la dinámica folicular de vacas Cebú x Holstein sincronizadas con implantes de norgestomet. Se utilizaron 50 vacas en lactación con más de 60 días posparto, las cuales recibieron un implante de 3 mg de norgestomet y una dosis luteolítica de PGF2a (día cero. El día cinco, a 25 vacas (grupo Norg, se les administraron 1.8 mg de norgestomet intramuscular (IM y 25 vacas (grupo testigo, recibieron 3 ml de solución salina. El noveno día se retiró el implante, se realizó un destete temporal por 72 h y los animales fueron divididos al azar dentro de sus tratamientos para recibir (n = 23 o no (n = 27 500 UI de eCG. Posteriormente, las vacas fueron observadas durante cinco días en forma continua para la detección de signos de estro. Se realizaron diariamente ecografías de los ovarios desde el día de la inserción del implante hasta la presentación de estro. El tratamiento agudo con norgestomet provocó recambio del folículo dominante (FD en 40% (10/25 de las vacas, contra 16% (4/25 del grupo testigo (P 0.05 el porcentaje de vacas que presentaron estro ni el tiempo de presentación del mismo. Se concluye que el tratamiento agudo con norgestomet, durante la sincronización del estro con progestágenos, provoca atresia y recambio folicular. Sin embargo, este efecto depende de la etapa de desarrollo del folículo dominante al momento del tratamiento.

  14. Interventional catheter methods for complex tetralogy of fallot and 'Critical' PS/pulmonary atresia with intact interventional septum

    International Nuclear Information System (INIS)

    Issenberg, H.J.

    2005-01-01

    TOF is generally accepted as the index lesion used for evaluating the overall quality of a congenital cardiac program. To achieve good results , the evaluation and management of TOF requires an intimate collaboration between all members of the cardiac team involved in the care of these children, especially the interventional catheterizer and the surgeon. Each program has to develop its own approach, relying on the collective strengths of their staff. Any weak link in this chain will often have a profoundly negative effect upon the patient , which may be difficult to correct later. Whereas the cathlab was the sole source of the anatomic and physiologic data for surgical planning, much of the information, especially intracardiac anatomy and physiology, can now be acquired with echocardiography, and if necessary, enhanced with MRI and CT. The role of diagnostic angiography is reserved for the elucidation of complex and small vascular structures beyond the resolution and specificity of less invasive methods, such as pulmonary artery branches, coronary arteries and arterial collaterals. This anatomy becomes particularly important when staging procedures for TOF in the infant with small pulmonary arteries or atresia, when is the greatest potential for enhancing arterial size and vascular bed growth. Our approach is to perform early correction which will insure antegrade flow into the small pulmonary arteries, by opening the outflow tract (patch or conduit), and when feasible, closing the VSD. Aortic to pulmonary arterial shunts are avoided. Direct access into the pulmonary arteries allows additional catheter-based interventions. Which may include balloon dilation and stenting, which avoiding any deformity from peripheral shunts. Aortic collaterals perfusing lung segments competing with antegrade flow into the branch pulmonary arteries may need Giannturco coil occlusion. Those with small pulmonary arteries may require multiple ballooning/stenting interventions to increase

  15. Puentes con vigas pretensadas

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    Editorial, Equipo

    1965-07-01

    Full Text Available This paper describes one of the three bridges which Hidrocivil, S. A., has built in Catalonia (northern Spain, over the river Ripoll. The other two bridges are very similar to this one, both in construction and design, and show only minor adjustments to the local topography. The contracting firm proposed several alterations in the prefabrication and constructional procedure, in relation to the initial project, and these changes were accepted. The main feature of these projects is the use of prestressed beams, built at the workshop in sections, and joined together by means of sixty 7 mm cables in each beam. As the shear forces are more acute at the joints, the end of each section has a kind of diaphragm, to provide a large contact area, and hence greater surface to transmit the shear forces. The methods of construction are also of interest. Briefly, they involve building the bridge piles, and use these to support a provisional structure with transversal movement. This provisional structure, in turn, served as platform for two bridge cranes, which lifted the girders to their final location. After the first span was completed, the deck was concreted and the auxiliary structure pushed forward to the next span, to repeat the same operations. This arrangement saved the use of provisional framework.En este trabajo se describe uno de los tres puentes que Hidrocivil, S. A., ha construido.—previo concurso— en la región catalana; concretamente, el que salva el río Ripoll. Los otros dos no han sido objeto de descripción general por ser muy similares, en lo que a ejecución y concepción se refiere, con la única variante que presentan las características topográficas locales. La empresa propuso ciertas variantes— que fueron aceptadas— en la prefabricación y métodos de construcción. El interés de estas obras se centra en el empleo de vigas pretensadas, prefabricadas en taller por trozos, y solidarizados en el mismo mediante las operaciones

  16. Violencia con el anciano

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    Rita Campillo Motilva

    2002-08-01

    Full Text Available La violencia doméstica es tan antigua como la humanidad misma y se reconocen la violencia infantil, contra la mujer y al anciano, fundamentalmente; siendo este último grupo una población en ascenso por las mayores expectativas de vida de los últimos años. Como resultado de ello, el número de casos de abuso en el anciano se incrementará y el impacto de este abuso sobre la salud debe ser considerado de forma adecuada. La gama de maltratos es variadísima e incluye el abuso físico, emocional, financiero, sexual, por negligencia, negación a brindarle ayuda y otras formas más. Los ancianos con deterioro cognitivo son los más vulnerables. El médico en la atención primaria de salud es un pilar importante en la prevención y educación de este problema.Domestic violence is as old as humanity itself. Child, women and elderly abuse are mainly recognized. The elderly group is increasing due to the higher life expectancy experimented during the last years. As a result, the number of battered elderly will grow and the impact of this abuse on health should be adequately considered. The range of abuse is very wide and it includes physical, emotional, financial and sexual abuse, negligence, rejection to give assistance and others. The elderly with cognitive deterioration are the most vulnerable. The physician at the primary health care level is an important milestone in the prevention and education of this problem.

  17. Clinical benefit of liver stiffness measurement at 3 months after Kasai hepatoportoenterostomy to predict the liver related events in biliary atresia.

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    Seung Min Hahn

    Full Text Available BACKGROUND: The progression of hepatic fibrosis may result in decompensated hepatic failure with cirrhosis, liver related events (LRE such as ascites, variceal bleeding, and death after successful and timely Kasai hepatoportoenterostomy (HPE in biliary atresia. The aim of this study is to suggest clinical benefit of the liver stiffness measurement (LSM using transient elastography at 3 months after the Kasai operation to predict LRE. METHODS: Between January 2007 and December 2011, 69 eligible biliary atresia patients who underwent Kasai HPE and performed transient elastography before and 3 months after HPE were included. The occurrences of LRE were analyzed for all patients. All patients were divided into 2 groups (with and without LRE for comparison. Multivariate analysis was used to detect the independent risk factors of LRE. The area under the receiver operation characteristics curve (AUROC was used to establish the LSM optimal cutoff value of 3 months after Kasai operation in predicting LRE. RESULTS: LSM value, aminotransferase, albumin, bilirubin, and PT-INR significantly differed among the two groups. Multivariate analysis demonstrated LSM value as the most powerful independent factor of the development of LRE. The cut-off value of 19.9 kPa was calculated to be optimal for predicting LRE development with total sensitivity and specificity of 1.804. AUROC resulted in 0.943, with sensitivity of 85.3% and specificity of 95.2%. CONCLUSIONS: The LSM value of 3 months after Kasai HPE can be a useful predictor of LRE development.

  18. Melanins as biomarkers of ovarian follicular atresia in the catfish Heteropneustes fossilis: biochemical and histochemical characterization, seasonal variation and hormone effects.

    Science.gov (United States)

    Kumar, Ravi; Joy, Keerikkattil P

    2015-06-01

    Follicular atresia is a common feature of the vertebrate ovary that occurs at different stages of folliculogenesis and ovarian regression. It has physiological significance to maintain homeostasis and control fecundity, and ensure removal of post-ovulatory follicular remnants for preparing the ovary for the next cycle. Pigments appear late in the atretic process as indigestible waste formed out of the degradation of the oocytes, follicle wall and granulocytes. In the present study, pigment accumulation was demonstrated by Schmorl's and Perls' staining methods in the atretic ovarian follicles of Heteropneustes fossilis during follicular development and regression. Melanins were characterized spectrophotometrically for the first time in fish ovary. The predominant form is eumelanin, followed by pheomelanin and alkali-soluble melanin. Melanins showed significant seasonal variations with levels low in gonad resting phase, increasing to the peak in the post-spawning phase. The concentration of melanins increased time-dependently in post-ovulated ovary after human chorionic gonadotropin treatment. In the spawning phase, in vitro incubation of ovary slices with estradiol-17β or dexamethasone for 8 or 16 h decreased both eumelanin and pheomelanin levels time-dependently. The alkali-soluble melanin showed a significant decrease only in the dexamethasone group at 16 h. The results show that melanin assay can be used as a biomarker of follicular atresia in fish ovary, natural or induced by environmental toxicants.

  19. Acne polimorfo: tratamiento con Implacen

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    Rubén Pérez Armas

    1995-06-01

    Full Text Available Se realiza el estudio de 40 pacientes con acné polimorfo, los que fueron atendidos en la Consulta de Dermatología del Hospital Provincial Clinicoquirúrgico Docente "Celia Sánchez Manduley", en el período comprendido de enero de 1988 a diciembre de 1989. Se revisa la literatura médica sobre los diversos métodos y medicamentos utilizados en la terapéutica de esta dermatosis. Se describe el esquema de tratamiento empleado con implacén en 30 pacientes; los 10 restantes se trataron con placebo; se compara dicho esquema con los tradicionales y se observan mejores resultados con nuestro estudio. Se destaca la ausencia de recaídas, así como el resultado del tratamiento de acuerdo con el sexo.A study was performed in 40 patients presenting with polymorphic acne who were attended in the Dermatology Department of "Celia Sánchez Manduley" Clinicosurgical and Teaching Hospital from January, 1988 to December, 1989. A review of the literature was made seeking for the different methods and drugs used for the treatment of this dermatosis. The treatment schedule with the use of implacen in 30 patients is described. Such therapeutic schedule was compared with traditional ones and better results were observed with the use of implacen. The fact that there were no relapses is highlighted, as well as the result of treatment according to sex.

  20. Anastomotic Strictures after Esophageal Atresia Repair: Incidence, Investigations, and Management, Including Treatment of Refractory and Recurrent Strictures

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    Renato Tambucci

    2017-05-01

    Full Text Available Improved surgical techniques, as well as preoperative and postoperative care, have dramatically changed survival of children with esophageal atresia (EA over the last decades. Nowadays, we are increasingly seeing EA patients experiencing significant short- and long-term gastrointestinal morbidities. Anastomotic stricture (AS is the most common complication following operative repair. An esophageal stricture is defined as an intrinsic luminal narrowing in a clinically symptomatic patient, but no symptoms are sensitive or specific enough to diagnose an AS. This review aims to provide a comprehensive view of AS in EA children. Given the lack of evidence-based data, we critically analyzed significant studies on children and adults, including comments on benign strictures with other etiologies. Despite there is no consensus about the goal of the luminal diameter based on the patient’s age, esophageal contrast study, and/or endoscopy are recommended to assess the degree of the narrowing. A high variability in incidence of ASs is reported in literature, depending on different definitions of AS and on a great number of pre-, intra-, and postoperative risk factor influencing the anastomosis outcome. The presence of a long gap between the two esophageal ends, with consequent anastomotic tension, is determinant for stricture formation and its response to treatment. The cornerstone of treatment is endoscopic dilation, whose primary aims are to achieve symptom relief, allow age-appropriate capacity for oral feeding, and reduce the risk of pulmonary aspiration. No clear advantage of either balloon or bougie dilator has been demonstrated; therefore, the choice is based on operator experience and comfort with the equipment. Retrospective evidences suggest that selective dilatations (performed only in symptomatic patients results in significantly less number of dilatation sessions than routine dilations (performed to prevent symptoms with equal long

  1. Tratamento cirúrgico da atresia de vias biliares: a experiência do hospital municipal jesus, 1997-2000

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    Lisieux Eyer de Jesus

    Full Text Available OBJETIVO: Analisar a indicação, resultados e limitações da portoenterostomia para o tratamento da atresia de vias biliares em hospital infantil terciário no Rio de Janeiro-RJ/Brasil. MÉTODO: Foram estudados prospectivamente oito pacientes submetidos à portoenterostomia do tipo Kasai 1, num período de três anos (1997-2000. Todos foram submetidos à antibioticoterapia profilática, uso de colerético, reavaliação médica mensal nos primeiros seis meses de seguimento e reavaliação laboratorial, ultra-sonográfica e através de endoscopia digestiva alta em prazos programados. RESULTADOS: Em cinco pacientes foi possível obter drenagem biliar estável e satisfatória por > 6 meses pós-operatórios. Quatro faleceram no período de observação (um por insuficiência hepática, um por colangite, dois por hemorragias digestivas, inclusive os três pacientes em que não foi obtida drenagem biliar satisfatória. Quatro persistem em bom estado geral, anictéricos, um deles com fibrose hepática residual grave e provável indicação futura de transplante hepático. Detectamos problemas graves com relação ao encaminhamento tardio de doentes para tratamento e para disponibilizar transplante hepático quando necessário. CONCLUSÕES: Os resultados da portoenterostomia são compensadores quando é possível obter bom débito biliar no pós-operatório. Em pacientes em que não é obtida boa drenagem biliar o pós-operatório é tormentoso e o óbito é esperado até o segundo ano de vida sem o uso de transplante hepático. É necessário disponibilizar transplante hepático pediátrico em nosso meio e conscientizar a comunidade médica para o encaminhamento precoce de bebês ictéricos além dos primeiros 15 dias de vida para investigação e tratamento em unidades especializadas.

  2. Histopathological and Follicular Atresia Assessment of Rat’s Ovarian Tissue Following Experimental Chronic Spinal Cord Injury

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    Sam Zarbakhsh

    2017-06-01

    Full Text Available Background One of the important side effects in spinal cord injuries in both genders is sexual dysfunction. This study aimed at investigating histopathological changes of ovaries in the chronic phase after spinal cord injury. Methods Animals (adult female rats were divided to the following groups, Control (Co, Sham (Sh, and Spinal Cord Injury (SCI; each group contained 18 rats. The spinal cord of SCI animals was transected by the bilateral laminectomy method on level T9, while the rats of the sham group were incised in the same location without any spinal cord injury. Animals were sacrified at 7th, 14th and 21st day postsurgical intervention, then the body weight, and the weight and volume of ovaries were measured. The slices were stained by hematoxylin and eosin and periodic acid Schiff methods. The histomorphometric changes of the diameter of the follicle and ovum, and the thickness of granulosa layer were measured in different kinds of follicles including, Unilaminar Primary Follicle (UPF, Multilaminar Primary Follicle (MPF, Secondary Follicle (SF, and Tertiary Follicle (TF. Results The results showed that the animal weights were decreased in three SCI groups (P = 0.018. In the SCI groups, the diameter of follicle and ovum and the thickness of granulosa layer were significantly decreased in different kinds of follicles (P = 0.012. Also the thickness of zona pellucida and theca interna were significantly decreased in UPF, SF, and TF in the SCI groups on 14th and 21st day (P = 0.024. The histopathologic examination revealed widespread ovarian follicle atresia in the SCI groups on the 14th and 21st day, including numerous cell debris and inflammatory cells in the antrum atretic follicles. The ovarian stroma showed edema, fibrosis, hypercellularity, and vasodilation in the SCI group, compared to the sham or control groups. Conclusions The histopathlogic data indicated that after spinal cord injury many histologic parameter changes occurred and

  3. con mala calidad de vida

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    Agustín Martín-Rodríguez

    2007-01-01

    Full Text Available En este estudio ex post facto se ha analizado si los familiares de pacientes con mala calidad de vida presentan diferencias en las variables clínicas de personalidad y relaciones familiares en función de que el paciente haya estado o no ingresado en una Unidad de Cuidados Intensivos. Seleccionamos dos grupos: 29 familiares de pacientes traumatizados graves transcurridos cuatro años de su ingreso en una UCI de Traumatología y con mala calidad de vida (debido a secuelas físicas y/o psicológicas tras el ingreso, tales como traumatismos craneoencefálicos, politraumatismos y tetraplejias traumáticas y 32 familiares de pacientes con mala calidad de vida con cuatro años de evolución de su enfermedad física (hipertensión, diabetes, artritis reumatoide y síndrome de intestino irritable que no han estado ingresados en la UCI. Para alcanzar nuestro objetivo empleamos una Encuesta Psicosocial y los siguientes instrumentos: Cuestionario de Análisis Clínico, Escala de Clima Social en la Familia y Escala de Adaptación Psicosocial de la Enfermedad. Los resultados mostraron que los familiares de pacientes con mala calidad de vida que estuvieron ingresados en la UCI hace cuatro años, presentan diferencias significativas en las variables agitación y expresividad comparados con los familiares de pacientes con mala calidad de vida que no han estado ingresados en la UCI.

  4. Pregnenolone co-treatment partially restores steroidogenesis, but does not prevent growth inhibition and increased atresia in mouse ovarian antral follicles treated with mono-hydroxy methoxychlor

    Energy Technology Data Exchange (ETDEWEB)

    Craig, Zelieann R., E-mail: zelieann@illinois.edu; Hannon, Patrick R., E-mail: phannon2@illinois.edu; Flaws, Jodi A., E-mail: jflaws@illinois.edu

    2013-11-01

    Mono-hydroxy methoxychlor (mono-OH MXC) is a metabolite of the pesticide, methoxychlor (MXC). Although MXC is known to decrease antral follicle numbers, and increase follicle death in rodents, not much is known about the ovarian effects of mono-OH MXC. Previous studies indicate that mono-OH MXC inhibits mouse antral follicle growth, increases follicle death, and inhibits steroidogenesis in vitro. Further, previous studies indicate that CYP11A1 expression and production of progesterone (P{sub 4}) may be the early targets of mono-OH MXC in the steroidogenic pathway. Thus, this study tested whether supplementing pregnenolone, the precursor of progesterone and the substrate for HSD3B, would prevent decreased steroidogenesis, inhibited follicle growth, and increased follicle atresia in mono-OH MXC-treated follicles. Mouse antral follicles were exposed to vehicle (dimethylsulfoxide), mono-OH MXC (10 μg/mL), pregnenolone (1 μg/mL), or mono-OH MXC and pregnenolone together for 96 h. Levels of P{sub 4}, androstenedione (A), testosterone (T), estrone (E{sub 1}), and 17β-estradiol (E{sub 2}) in media were determined, and follicles were processed for histological evaluation of atresia. Pregnenolone treatment alone stimulated production of all steroid hormones except E{sub 2}. Mono-OH MXC-treated follicles had decreased sex steroids, but when given pregnenolone, produced levels of P{sub 4}, A, T, and E{sub 1} that were comparable to those in vehicle-treated follicles. Pregnenolone treatment did not prevent growth inhibition and increased atresia in mono-OH MXC-treated follicles. Collectively, these data support the idea that the most upstream effect of mono-OH MXC on steroidogenesis is by reducing the availability of pregnenolone, and that adding pregnenolone may not be sufficient to prevent inhibited follicle growth and survival. - Highlights: • Mono-OH MXC inhibited antral follicle steroidogenesis, growth, and survival. • Pregnenolone partially restored steroidogenesis

  5. Pregnenolone co-treatment partially restores steroidogenesis, but does not prevent growth inhibition and increased atresia in mouse ovarian antral follicles treated with mono-hydroxy methoxychlor

    International Nuclear Information System (INIS)

    Craig, Zelieann R.; Hannon, Patrick R.; Flaws, Jodi A.

    2013-01-01

    Mono-hydroxy methoxychlor (mono-OH MXC) is a metabolite of the pesticide, methoxychlor (MXC). Although MXC is known to decrease antral follicle numbers, and increase follicle death in rodents, not much is known about the ovarian effects of mono-OH MXC. Previous studies indicate that mono-OH MXC inhibits mouse antral follicle growth, increases follicle death, and inhibits steroidogenesis in vitro. Further, previous studies indicate that CYP11A1 expression and production of progesterone (P 4 ) may be the early targets of mono-OH MXC in the steroidogenic pathway. Thus, this study tested whether supplementing pregnenolone, the precursor of progesterone and the substrate for HSD3B, would prevent decreased steroidogenesis, inhibited follicle growth, and increased follicle atresia in mono-OH MXC-treated follicles. Mouse antral follicles were exposed to vehicle (dimethylsulfoxide), mono-OH MXC (10 μg/mL), pregnenolone (1 μg/mL), or mono-OH MXC and pregnenolone together for 96 h. Levels of P 4 , androstenedione (A), testosterone (T), estrone (E 1 ), and 17β-estradiol (E 2 ) in media were determined, and follicles were processed for histological evaluation of atresia. Pregnenolone treatment alone stimulated production of all steroid hormones except E 2 . Mono-OH MXC-treated follicles had decreased sex steroids, but when given pregnenolone, produced levels of P 4 , A, T, and E 1 that were comparable to those in vehicle-treated follicles. Pregnenolone treatment did not prevent growth inhibition and increased atresia in mono-OH MXC-treated follicles. Collectively, these data support the idea that the most upstream effect of mono-OH MXC on steroidogenesis is by reducing the availability of pregnenolone, and that adding pregnenolone may not be sufficient to prevent inhibited follicle growth and survival. - Highlights: • Mono-OH MXC inhibited antral follicle steroidogenesis, growth, and survival. • Pregnenolone partially restored steroidogenesis in mono-OH MXC

  6. Tratamiento con implantes Leader-Nano en paciente con oligodoncia

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    Salvador Javier Santos Medina

    2015-03-01

    Full Text Available Los implantes dentales de titanio han revolucionado el mundo de la rehabilitación desde su surgimiento. De manera particular, el empleo de implantes de carga inmediata acorta el tiempo quirúrgico y protésico, con el consiguiente bienestar estético. Se presenta el caso de una paciente femenina de 32 años de edad, con antecedentes de oligodoncia de ambos incisivos laterales superiores y portadora de prótesis parcial acrílica. Fue atendida por el equipo multidisciplinario de implantes en la Clínica Estomatológica Docente “3 de Octubre” y se le realizó tratamiento de rehabilitación integral con implantes Leader-Nano y prótesis fija con corona acrílica sobre dichos implantes. La implantología fue satisfactoria en la paciente; la mejoría estética y funcional, así como la satisfacción de la paciente, fueron los principales logros obtenidos

  7. con dietas suplementadas con Cromo-L-metionina

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    Ram\\u00F3n Garc\\u00EDa-Castillo

    2006-01-01

    Full Text Available Un total de 48 cerdos (Sus scrofa domesticus; 24 machos castrados y 24 hembras cruzados (Yorkshire, Hampshire, Duroc y Landrace de 3,5 a 4,0 meses de edad y 60,0 ± 5,0 kg PV en finalización. Se alimentaron con dietas isoproteícas (14,5 % PC e isoenergéticas (3.400 kcal EM/kg de MS, adicionadas con Cr-L-metionina (MiCroPlex® (0, 200, 400 y 600 ppb. El experimento tuvo una duración de 45 días y se realizó de agosto a noviembre del 2002 en las instalaciones de la Universidad Autónoma Agraria Antonio Narro, localizada en Saltillo, Coahuila, México. Al tener los animales aproximadamente 95 kg PV, se tomó muestra de 15 ml de sangre por cada animal para determinar la concentración de glucosa, ácido úrico, creatinina, urea, proteinas totales y colesterol. Se aplicó un diseño completamente al azar con arreglo factorial 2 x 4; dos para el factor sexo y cuatro para nivel de cromo. Los metabolitos en suero no fueron afectados (P>0,05 por el factor sexo. La glucosa en suero disminuyó (P<0,05 y el colesterol incrementó (P<0,05 con cromo en la dieta. Se concluye que el Cr incrementa el metabolismo de glucosa y disminuye el de colesterol, con lo cual puede haber energía disponible para síntesis de proteína la cual es necesaria para el crecimiento de los animales

  8. Intestinal atresia, encephalocele, and cardiac malformations in infants with 47,XXX: Expansion of the phenotypic spectrum and a review of the literature.

    Science.gov (United States)

    Bağci, Soyhan; Müller, Andreas; Franz, Axel; Heydweiller, Andreas; Berg, Christoph; Nöthen, Markus M; Bartmann, Peter; Reutter, Heiko

    2010-01-01

    Identification of the 47,XXX karyotype often occurs adventitiously during prenatal fetal karyotyping in cases of advanced maternal age. Although most females with 47,XXX appear healthy at birth, various types of congenital malformations have been reported, of which urinary tract anomalies are the most frequent. We report on 2 newborns with 47,XXX and congenital cardiac defects, one of whom had duodenal atresia and the other an occipital encephalocele. This expands the spectrum of malformations reported in association with the triple-X syndrome. We also present a review of the literature on non-urinary tract malformations in females with 47,XXX. We conclude that prenatal identification of the 47,XXX karyotype is an indication for detailed fetal ultrasonography which should include examination of multiple organ systems. Such prenatal screening for possible associated congenital malformations should help to ensure optimal perinatal clinical management of 47,XXX cases. 2010 S. Karger AG, Basel.

  9. 3D Printing Provides a Precise Approach in the Treatment of Tetralogy of Fallot, Pulmonary Atresia with Major Aortopulmonary Collateral Arteries.

    Science.gov (United States)

    Anwar, Shafkat; Rockefeller, Toby; Raptis, Demetrios A; Woodard, Pamela K; Eghtesady, Pirooz

    2018-02-03

    Patients with tetralogy of Fallot, pulmonary atresia, and multiple aortopulmonary collateral arteries (Tet PA MAPCAs) have a wide spectrum of anatomy and disease severity. Management of these patients can be challenging and often require multiple high-risk surgical and interventional catheterization procedures. These interventions are made challenging by complex anatomy that require the proceduralist to mentally reconstruct three-dimensional anatomic relationships from two-dimensional images. Three-dimensional (3D) printing is an emerging medical technology that provides added benefits in the management of patients with Tet PA MAPCAs. When used in combination with current diagnostic modalities and procedures, 3D printing provides a precise approach to the management of these challenging, high-risk patients. Specifically, 3D printing enables detailed surgical and interventional planning prior to the procedure, which may improve procedural outcomes, decrease complications, and reduce procedure-related radiation dose and contrast load.

  10. Balloon dilatation in children for oesophageal strictures other than those due to primary repair of oesophageal atresia, interposition or restrictive fundoplication

    International Nuclear Information System (INIS)

    Fasulakis, Stephen; Andronikou, Savvas

    2003-01-01

    Balloon dilatation of the oesophagus in children has been performed predominantly for treating strictures, which are the result of primary repair of oesophageal atresia, interposition surgery or restrictive Nissen's fundoplication. Reports of the use of this technique for alternative causes of stricture are few. To report our experience and success with balloon dilatation of strictures due to caustic ingestion, achalasia, oesophagitis, congenital stenosis, and epidermolysis bullosa (EB) and to make comparisons with our treatment of patients with primary repair of oesophageal atresia (OA), as well as with reports in the English language literature. Retrospective review of fluoroscopically guided balloon oesophageal dilatation procedures in 19 patients over a 5-year period, and comparison of those performed for OA repair complications with those due to other diseases. The average radiation dose, per procedure, was calculated by a medical physicist. Ten patients had strictures as a result of primary repair of OA. Three patients had stricture as a result of EB, two from achalasia, two from caustic injury, one due to an oesophageal web and one from reflux oesophagitis. Our results show that the technique can also be curative for the last group and that it may be used intermittently to alleviate symptoms in ongoing diseases. We have not experienced any complications and have also calculated that, even with prolonged use of multiple procedures, the radiation exposure is comparable to other radiological techniques. Patients with alternative causes for oesophageal stricture may be treated to resolution within 2 years using balloon dilatation. Ongoing diseases such as EB require ongoing dilatation, but balloon dilatation of strictures has been successful in alleviating swallowing difficulty. Patients with stricture from OA repair sometimes need ongoing dilatation. Radiation exposure for multiple procedures, over an extended period, is comparable to that from a single

  11. Balloon dilatation in children for oesophageal strictures other than those due to primary repair of oesophageal atresia, interposition or restrictive fundoplication

    Energy Technology Data Exchange (ETDEWEB)

    Fasulakis, Stephen [Royal Children' s Hospital, Melbourne (Australia); Andronikou, Savvas [Royal Children' s Hospital, Melbourne (Australia); Department of Paediatric Radiology, Red Cross Children' s Hospital, Klipfontein Road, Rondebosch, 7700, Cape Town (South Africa)

    2003-10-01

    Balloon dilatation of the oesophagus in children has been performed predominantly for treating strictures, which are the result of primary repair of oesophageal atresia, interposition surgery or restrictive Nissen's fundoplication. Reports of the use of this technique for alternative causes of stricture are few. To report our experience and success with balloon dilatation of strictures due to caustic ingestion, achalasia, oesophagitis, congenital stenosis, and epidermolysis bullosa (EB) and to make comparisons with our treatment of patients with primary repair of oesophageal atresia (OA), as well as with reports in the English language literature. Retrospective review of fluoroscopically guided balloon oesophageal dilatation procedures in 19 patients over a 5-year period, and comparison of those performed for OA repair complications with those due to other diseases. The average radiation dose, per procedure, was calculated by a medical physicist. Ten patients had strictures as a result of primary repair of OA. Three patients had stricture as a result of EB, two from achalasia, two from caustic injury, one due to an oesophageal web and one from reflux oesophagitis. Our results show that the technique can also be curative for the last group and that it may be used intermittently to alleviate symptoms in ongoing diseases. We have not experienced any complications and have also calculated that, even with prolonged use of multiple procedures, the radiation exposure is comparable to other radiological techniques. Patients with alternative causes for oesophageal stricture may be treated to resolution within 2 years using balloon dilatation. Ongoing diseases such as EB require ongoing dilatation, but balloon dilatation of strictures has been successful in alleviating swallowing difficulty. Patients with stricture from OA repair sometimes need ongoing dilatation. Radiation exposure for multiple procedures, over an extended period, is comparable to that from a single

  12. Hearing improvement with softband and implanted bone-anchored hearing devices and modified implantation surgery in patients with bilateral microtia-atresia.

    Science.gov (United States)

    Wang, Yibei; Fan, Xinmiao; Wang, Pu; Fan, Yue; Chen, Xiaowei

    2018-01-01

    To evaluate auditory development and hearing improvement in patients with bilateral microtia-atresia using softband and implanted bone-anchored hearing devices and to modify the implantation surgery. The subjects were divided into two groups: the softband group (40 infants, 3 months to 2 years old, Ponto softband) and the implanted group (6 patients, 6-28 years old, Ponto). The Infant-Toddler Meaning Auditory Integration Scale was used conducted to evaluate auditory development at baseline and after 3, 6, 12, and 24 months, and visual reinforcement audiometry was used to assess the auditory threshold in the softband group. In the implanted group, bone-anchored hearing devices were implanted combined with the auricular reconstruction surgery, and high-resolution CT was used to assess the deformity preoperatively. Auditory threshold and speech discrimination scores of the patients with implants were measured under the unaided, softband, and implanted conditions. Total Infant-Toddler Meaning Auditory Integration Scale scores in the softband group improved significantly and approached normal levels. The average visual reinforcement audiometry values under the unaided and softband conditions were 76.75 ± 6.05 dB HL and 32.25 ± 6.20 dB HL (P hearing devices is effective for auditory development and hearing improvement in infants with bilateral microtia-atresia. Wearing softband bone-anchored hearing devices before auricle reconstruction and combining bone-anchored hearing device implantation with auricular reconstruction surgery may bethe optimal clinical choice for these patients, and results in more significant hearing improvement and minimal surgical and anesthetic injury. Copyright © 2017 Elsevier B.V. All rights reserved.

  13. A case report of bicornis bicollis uterus with unilateral cervical atresia: an unusual aetiology of chronic debilitating pelvic pain in a Cameroonian teenager.

    Science.gov (United States)

    Dohbit, Julius Sama; Meka, Esther; Tochie, Joel Noutakdie; Kamla, Igor; Mwadjie, Darolles; Foumane, Pascal

    2017-06-02

    Congenital uterine anomalies like bicornis or bicornuate uterus are relatively rare in sub-Saharan Africa. They are associated with an increased rate of spontaneous abortion, preterm delivery, and infertility. The occurrence of bicornis bicollis uterus with unilateral cervical atresia is exceptional and its management is controversial. We hereby report a rare cause of chronic pelvic pain in a Cameroonian teenager due to unilateral obstructive hematometra and hematosalpinx in the non-communicating horn of a bicornis bicollis uterus. A 13-year-old premenarchal non-virgin female presented with chronic and severe cyclical crampy pelvic pain. On clinical examination, she had a perforated hymen, a single vagina, and one uterine cervix. A two-dimensional pelvic ultrasonography revealed hematometra but missed out the underlying anomaly. Failure to drain the hematometra by serial cervical dilatations prompted an exploratory laparotomy which revealed: bicornis bicollis uterus with a right rudimentary uterine horn communicating with the vagina and a left non-communicating uterine horn distended by hematometra due to a homolateral cervical atresia. She underwent utero-vaginal canalization and a left hemi-hysterotomy with drainage of the hematometra. The postoperative period was uneventful. Regular cyclic menses occurred thereafter beginning at the first postoperative month. She had complete resolution of symptoms without recurrence after six months. Due to the risk of compromised fertility from bicornis uterus and the diagnostic challenges akin to resource-limited settings, we highlight the need for a high index of suspicion by healthcare providers when faced with chronic pelvic pain in premenarchal adolescents.

  14. congenital Biliary atresia

    African Journals Online (AJOL)

    embryonic form), comprising 10-35% of cases2. The pathology of the extrahepatic biliary system widely varies in these ... hepatic duct, with cystic structures found in the porta hepatis. .... Nelson Textbook of. Paediatrics 15th edition : Chapter 302.

  15. Recubrimiento de acero con polidopamina

    OpenAIRE

    Carrasco Rodríguez, Javier

    2013-01-01

    Se ha obtenido recubrimientos de polidopamina en acero mecánicamente resistentes y con tiempos de obtención relativamente pequeños a través de la polimerización de la dopamina bajo diferentes condiciones.

  16. Santiago, una ciudad con temor

    Directory of Open Access Journals (Sweden)

    Enrique Oviedo S.

    1999-04-01

    Full Text Available El objetivo general de este artículo es evaluar los efectos de la inseguridad ciudadana en el uso del espacio público. Dicha evaluación exige analizar dos relaciones que se establecen en el ámbito de la violencia: la relación entre victimización y percepción de inseguridad; y la que se establece entre actitudes sociales y resolución pacífica de conflictos nacionales. Para ello, se analizaron las variables victimización, percepción de inseguridad, uso del espacio físico, actitudes hacia el sistema institucional político y social y hacia la resolución de conflictos nacionales, y las posibles relaciones entre ellas. Los datos para realizar el estudio se obtuvieron por medio de una encuesta que se llevó a cabo con 1 200 personas de 18 y 70 años de edad residentes en la ciudad de Santiago. Los resultados indican que Santiago es una ciudad de habitantes con temor y que el aumento de la percepción de inseguridad de sus habitantes contrasta con el hecho de que las tasas de victimización se hayan mantenido, más o menos, constantes en los años que precedieron a la encuesta. El temor se relaciona con el abandono del espacio público físico y sociopolítico, así como con el refugio en los espacios y la vida privados. La actitud de resolver los conflictos por medios no pacíficos es frecuente y se asocia en mayor medida con la inseguridad, la actitud negativa hacia la democracia y la falta de expectativas sobre el futuro del país. Los resultados de este estudio respaldan la idea de que para superar el temor la gente tiende a adaptarse a la realidad adoptando una postura conformista, homogeneizando las creencias y los comportamientos, y sobreestimando la fuerza como medio para resolver las diferencias.

  17. Santiago, una ciudad con temor

    Directory of Open Access Journals (Sweden)

    Oviedo S. Enrique

    1999-01-01

    Full Text Available El objetivo general de este artículo es evaluar los efectos de la inseguridad ciudadana en el uso del espacio público. Dicha evaluación exige analizar dos relaciones que se establecen en el ámbito de la violencia: la relación entre victimización y percepción de inseguridad; y la que se establece entre actitudes sociales y resolución pacífica de conflictos nacionales. Para ello, se analizaron las variables victimización, percepción de inseguridad, uso del espacio físico, actitudes hacia el sistema institucional político y social y hacia la resolución de conflictos nacionales, y las posibles relaciones entre ellas. Los datos para realizar el estudio se obtuvieron por medio de una encuesta que se llevó a cabo con 1 200 personas de 18 y 70 años de edad residentes en la ciudad de Santiago. Los resultados indican que Santiago es una ciudad de habitantes con temor y que el aumento de la percepción de inseguridad de sus habitantes contrasta con el hecho de que las tasas de victimización se hayan mantenido, más o menos, constantes en los años que precedieron a la encuesta. El temor se relaciona con el abandono del espacio público físico y sociopolítico, así como con el refugio en los espacios y la vida privados. La actitud de resolver los conflictos por medios no pacíficos es frecuente y se asocia en mayor medida con la inseguridad, la actitud negativa hacia la democracia y la falta de expectativas sobre el futuro del país. Los resultados de este estudio respaldan la idea de que para superar el temor la gente tiende a adaptarse a la realidad adoptando una postura conformista, homogeneizando las creencias y los comportamientos, y sobreestimando la fuerza como medio para resolver las diferencias.

  18. The Role of DmCatD, a Cathepsin D-Like Peptidase, and Acid Phosphatase in the Process of Follicular Atresia in Dipetalogaster maxima (Hemiptera: Reduviidae), a Vector of Chagas' Disease

    Science.gov (United States)

    Leyria, Jimena; Fruttero, Leonardo L.; Nazar, Magalí; Canavoso, Lilián E.

    2015-01-01

    In this work, we have investigated the involvement of DmCatD, a cathepsin D-like peptidase, and acid phosphatase in the process of follicular atresia of Dipetalogaster maxima, a hematophagous insect vector of Chagas’ disease. For the studies, fat bodies, ovaries and hemolymph were sampled from anautogenous females at representative days of the reproductive cycle: pre-vitellogenesis, vitellogenesis as well as early and late atresia. Real time PCR (qPCR) and western blot assays showed that DmCatD was expressed in fat bodies and ovaries at all reproductive stages, being the expression of its active form significantly higher at the atretic stages. In hemolymph samples, only the immunoreactive band compatible with pro-DmCatD was observed by western blot. Acid phosphatase activity in ovarian tissues significantly increased during follicular atresia in comparison to pre-vitellogenesis and vitellogenesis. A further enzyme characterization with inhibitors showed that the high levels of acid phosphatase activity in atretic ovaries corresponded mainly to a tyrosine phosphatase. Immunofluorescence assays demonstrated that DmCatD and tyrosine phosphatase were associated with yolk bodies in vitellogenic follicles, while in atretic stages they displayed a different cellular distribution. DmCatD and tyrosine phosphatase partially co-localized with vitellin. Moreover, their interaction was supported by FRET analysis. In vitro assays using homogenates of atretic ovaries as the enzyme source and enzyme inhibitors demonstrated that DmCatD, together with a tyrosine phosphatase, were necessary to promote the degradation of vitellin. Taken together, the results strongly suggested that both acid hydrolases play a central role in early vitellin proteolysis during the process of follicular atresia. PMID:26091289

  19. pacientes con insuficiencia renal terminal

    Directory of Open Access Journals (Sweden)

    Karen Herrera Herrera

    2011-01-01

    Full Text Available La presente investigación fundamenta en la clínica psicoanalítica el estudio de dos casos de tres personas diagnosticadas con IRT que reciben tratamiento de hemodiálisis, en razón a que dadas las características y el aumento de los reportes que se presentan, ya esto se considera un problema de salud pública. El objetivo principal es describir las características dinámicas del proceso de duelo en pacientes con IRT en un centro de terapia renal de la ciudad de Cartagena. El procedimiento metodológico empleó un diseño de tipo cualitativo; la investigación se desarrolló con un diseño clínico mediante el estudio de casos, y fundamentada en la hermenéutica psicoanalítica. Todo esto respaldado en la historia clínica, la entrevista semiestructurada individual y familiar, los test proyectivos, test del dibujo de la figura humana Machover y TAT de Murray, para la debida integración de los análisis. Se concluye que predominan funciones fallidas de los progenitores y que son individuos provenientes de familias psicosomáticas, que utilizan la enfermedad para obtener un beneficio secundario.

  20. con bajo peso al nacer

    Directory of Open Access Journals (Sweden)

    Adriana Mora Antó

    2005-01-01

    Full Text Available Esta investigación dio cuenta de la relación entre el estilo de funcionamiento familiar, los patrones de crianza y las edades de desarrollo evolutivo en niños, nacidos con bajo peso. El estudio descriptivo correlacional se realizó con 41 niños y sus madres, aplicándose cuestionarios sobre funcionamiento familiar, prácticas de crianza y desarrollo infantil. Los resultados señalaron la existencia de un funcionamiento familiar caracterizado por una cohesión amalgamada y una adaptabilidad caótica, una disciplina complaciente, falta de control y de límites claros en la díada madre-hijo. Se trataba de familias monoparentales, donde la temprana edad de concepción, el madresolterismo y el apoyo de la familia extensa eran constantes. Las edades evolutivas registradas indicaron un desarrollo inferior a la edad cronológica, en la mayor parte de los casos; sin embargo, éstas tendieron a ser superiores al compararlas con la edades reales de los infantes. No se encontró una correlación estadísticamente significativa entre la edad de desarrollo y los diferentes factores del funcionamiento familiar para algunos de los rangos de edad considerados; sin embargo, no se lo descartó por completo, especialmente en lo referente al optimismo familiar

  1. Challenging surgical dogma in the management of proximal esophageal atresia with distal tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium.

    Science.gov (United States)

    Lal, Dave R; Gadepalli, Samir K; Downard, Cynthia D; Ostlie, Daniel J; Minneci, Peter C; Swedler, Ruth M; Chelius, Thomas H; Cassidy, Laura; Rapp, Cooper T; Billmire, Deborah; Bruch, Steven; Burns, R Carland; Deans, Katherine J; Fallat, Mary E; Fraser, Jason D; Grabowski, Julia; Hebel, Ferdynand; Helmrath, Michael A; Hirschl, Ronald B; Kabre, Rashmi; Kohler, Jonathan; Landman, Matthew P; Leys, Charles M; Mak, Grace Z; Raque, Jessica; Rymeski, Beth; Saito, Jacqueline M; St Peter, Shawn D; von Allmen, Daniel; Warner, Brad W; Sato, Thomas T

    2017-06-01

    Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF. The Midwest Pediatric Surgery Consortium conducted a multicenter, retrospective study examining selected outcomes on infants diagnosed with proximal EA with distal TEF who underwent primary repair over a 5-year period (2009-2014), with a minimum 1-year follow up, across 11 centers. 292 patients with proximal EA and distal TEF who underwent primary repair were reviewed. The overall mortality was 6% and was significantly associated with the presence of congenital heart disease (OR 4.82, p=0.005). Postoperative complications occurred in 181 (62%) infants, including: anastomotic stricture requiring intervention (n=127; 43%); anastomotic leak (n=54; 18%); recurrent fistula (n=15; 5%); vocal cord paralysis/paresis (n=14; 5%); and esophageal dehiscence (n=5; 2%). Placement of a transanastomotic tube was associated with an increase in esophageal stricture formation (OR 2.2, p=0.01). Acid suppression was not associated with altered rates of stricture, leak or pneumonia (all p>0.1). Placement of interposing prosthetic material between the esophageal and tracheal suture lines was associated with an increased leak rate (OR 4.7, p24h were used in 193 patients (66%) with no difference in rates of infection, shock or death when compared to antibiotic use ≤24h (all p>0.3). Hospital volume was not associated with postoperative complication rates (p>0.08). Routine postoperative esophagram obtained on day 5 resulted in no delayed/missed anastomotic leaks or a difference in anastomotic leak rate as compared to esophagrams obtained on day 7

  2. Quantitative analysis of pulmonary artery and pulmonary collaterals in preoperative patients with pulmonary artery atresia using dual-source computed tomography

    International Nuclear Information System (INIS)

    Yin Lei; Lu, Bin; Han Lei; Wu Runze; Johnson, Laura; Xu Zhongying; Jiang Shiliang; Dai Ruping

    2011-01-01

    Objective: To evaluate the value of dual-source computed tomography (DSCT) in quantitatively measuring pulmonary arteries and major aortopulmonary collateral vessels in comparison with conventional angiographic (CA) on preoperative patients with pulmonary artery atresia and ventricular septal defect (PAA-VSD). Materials and methods: Twenty PAA-VSD patients who had complete imaging data of DSCT, CA and echocardiography (ECHO) studies were retrospectively analyzed. Using final clinical diagnosis as the standard, results of DSCT, CA and ECHO on the detection of cardiac malformations, measurement of diameters of pulmonary artery and collateral vessel, as well as the values of McGoon ratio, pulmonary arterial index (PAI) and total neopulmonary arterial index (TNPAI) were derived and compared. Results: In 20 patients, 51 of 54 (94.4%) cardiac malformations were visualized by DSCT, whereas 42 (77.8%) by ECHO (p = 0.027). Fourteen cases with aortopulmonary collateral vessels were all (100%) detected by DSCT, whereas 5 cases (35.7%) by ECHO (p = 0.001), and 13 cases (92.9%) by CA (p = 0.995). Sixteen cases with confluence of native pulmonary arteries were diagnosed by DSCT, whereas 10 cases by CA (p = 0.024). Measurement of the diameters of pulmonary arteries, collateral vessels, and descending aorta at the level of diaphragm were correlated well between DSCT and CA (r = 0.95-0.99). McGoon ratio (DSCT = 1.18 ± 0.60, CA = 1.23 ± 0.64), PAI (DSCT = 130.96 ± 99.38 mm 2 /m 2 , CA = 140.91 ± 107.87 mm 2 /m 2 ) and TNPAI (DSCT = 160.31 ± 125.62 mm 2 /m 2 , CA = 169.14 ± 122.81 mm 2 /m 2 ) were calculated respectively, without significant differences between DSCT and CA by paired t-tests (all p > 0.05). Conclusion: DSCT was efficient for evaluating and measuring native pulmonary artery and aortopulmonary collateral vessels prior to surgical procedures in PAA-VSD patients. Combined with echocardiography, DSCT showed potential to replace CA for evaluating pulmonary artery

  3. Conversando con...Momoyo Kaijima

    OpenAIRE

    Gómez Alonso, Carlos; Álvarez Isidro, Eva; Torres Barchino, Ana

    2017-01-01

    [ES] Momoyo Kaijima es profesora en la Facultad de Arte y Diseño de la Universidad de Tsukuba en la Prefectura de Ibaraki y profesora visitante en la ETH de Zürich, en Royal Academy of Fine Arts, en Rice School of Architecture y en Harvard GSD. A lo largo de los años, Atelier Bow Wow ha colaborado con Krešimir Rogina, arquitecto de Zagreb y socio de la firma internacional Penezic&Rogina, en la realización del Grožnjan International Summer School of Architecture, siendo Rogina el nexo indispen...

  4. Food irradiation - pros and cons

    International Nuclear Information System (INIS)

    1983-01-01

    The use of ionising radiation for food preservation is a much-disputed topic, both among experts and among consumers. Pros and cons of this issue were discussed in detail at the consumers' forum. Professor Dr. Johannes Friedrich Diehl, Director of the Institute for Biochemistry of the Food Research Centre, Karlsruhe, is a well-known supporter of the new method of food preservation; he sees advantages in the radiopreservation of food because, for example, losses due to inedibility are reduced, the danger of salmonellosis is decreased, just as the use of chemicals. He thinks this method to be without danger to health, shown by many years of experience. Opponents to food irradiation like Prof. Dr. Konrad Pfeilsticker, Professor for food science and food chemistry at the Bonn University deem the method to be unnecessary and raise the problem of qualitative changes caused in the food. In the course of the discussions, the pros and cons seemed to balance each other out. (orig./AJ) [de

  5. De paseo con los Bourbaki

    Directory of Open Access Journals (Sweden)

    Miquel Escudero

    2012-04-01

    Full Text Available Setenta y siete años después de la fundación del grupo Bourbaki, procedemos a una reflexión que puede ser útil para los estudiantes que acaso no sepan de su existencia, ni tan siquiera los de matemáticas. Sería interesante conocer que a este grupo se le debe el signo del vacío como conjunto. Con todo lo discutible que sea el método Bourbaki en su reinterpretación de la matemática, no cabe duda de su importante repercusión hasta el punto de que ha marcado una época. Hay un antes y un después tras su irrupción, ningún matemático de primera fila de la segunda mitad del siglo XX fue ajeno a su influjo, para encabezarlo o para reprobarlo. Comenzaron como una juvenil extravagancia, pero repleta de conocimientos y con decidida voluntad de aprehender el rigor de forma exhaustiva.

  6. Tratamiento del paciente con artrosis

    Directory of Open Access Journals (Sweden)

    Francisco Vargas Negrín

    2014-01-01

    Full Text Available El manejo terapéutico del paciente con artrosis tiene como objetivo disminuir la sintomatología dolorosa e inflamatoria, mejorar la capacidad funcional del paciente y la aplicación de intervenciones terapéuticas eficaces y lo más seguras posibles. Un enfoque centrado en el paciente implica su participación activa en el diseño del plan terapéutico y en la toma de decisiones informadas oportunas en todas las etapas de la enfermedad. La educación terapéutica, la actividad física y el ejercicio terapéutico junto con el control de peso, en caso de sobrepeso u obesidad, constituyen el núcleo central del tratamiento. Los autocuidados individuales y por los familiares son fundamentales en el control del día a día del paciente. El uso de terapias físicas, ayudas técnicas (bastón, etc. y de fármacos tipo analgésicos simples, opioides y antiinflamatorios tiene evidencias demostradas en el control del dolor, mejora la funcionalidad y la calidad de vida del paciente y una clara recomendación de uso en el tratamiento de la artrosis. La cirugía conservadora y la de reemplazo articular se indican en los casos en los que no se logran los objetivos terapéuticos en casos concretos.

  7. Cons ICARUS, TIGER and Fascism

    Directory of Open Access Journals (Sweden)

    Janez Vrečko

    2010-12-01

    Full Text Available Like the scientists of their time, Russian artists in the 1920s considered gravity the central problem – a view which points to the close harmony between modern physics and the avant-garde. It was only with the constructivist movement that the Icarus revolution grasped the principles of the “mobile philosophy” (3.651 which was almost at the same time recognised by modern physics as well. The static view of the world became obsolete, space and time were no longer absolute values. It was necessary to transcend Euclidean geometry, shake off the political ʻshackles on one’s hands’ and surrender to Lisicki’s imaginary space, where “At 2000 metres in the air / there is no more perspective” (Integrals 276.  Kosovel’s Icarus project accorded with Tatlin’s, and both of them accorded with the quintessential aims of the constructivist movement. It is no accident that Kosovel wished to name one of his poetry collections The Dream of Icarus. Poems on the Icarus theme, such as “Cons Icarus”, “Evacuation of the Spirit”, “Eh, Hey”, “A Heart in Alcohol” etc. belong to the group of Kosovel’s conses which follow his “mobile philosophy” (3.650 and “letters growing into space” (Int. 282.  The question “Man, do you want up in the air?” (Int. 128 will remain a question until the moment when man is finally ready to transcend the existing boundary and dive “beyond”. Hence Kosovel’s clear-cut contrast between the “green windows of an illuminated / express on a viaduct”, which moves horizontally and is, like a water current, subject to the earth’s gravity, and “the spirit in space”, whose direction of motion is “the perpendicular of the spirit”, atectonicity. “The spirit burns in space”: fire is an element that knows vertical movement alone, the only one of the elements to outgrow and transcend the earth’s gravity, therefore it is associated with another mythological figure important for

  8. Microcephaly, microtia, preauricular tags, choanal atresia and developmental delay in three unrelated patients: a mandibulofacial dysostosis distinct from Treacher Collins syndrome.

    Science.gov (United States)

    Wieczorek, Dagmar; Gener, Blanca; González, Ma Jesús Martínez; Seland, Saskia; Fischer, Sven; Hehr, Ute; Kuechler, Alma; Hoefsloot, Lies H; de Leeuw, Nicole; Gillessen-Kaesbach, Gabriele; Lohmann, Dietmar R

    2009-05-01

    Treacher Collins syndrome (TCS, OMIM 154500) is a well-defined mandibulofacial dysostosis characterized by symmetric facial anomalies consisting of malar hypoplasia, coloboma of the lower eyelid, dysplastic ears, micrognathia, cleft palate and deafness. Other mandibulofacial dysostoses (MDs) such as Toriello (OMIM 301950), Bauru (OMIM 604830), Hedera-Toriello-Petty (OMIM 608257), and Guion-Almeida (OMIM 610536) syndromes are less well characterized and much rarer. Here we describe three unrelated patients showing clinical features overlapping with TCS, but who in addition have developmental delay, microcephaly and a distinct facial gestalt. Because of the distinct ear anomalies and the hearing loss a HOXA2 mutation was taken into account. CHARGE syndrome was discussed because of ear anomalies, choanal atresia, and developmental delay in our patients. But mutational analyses including sequencing of the TCOF1, the HOXA2, and the CHD7 genes, deletion screening of the TCOF1 gene as well as genomewide array analyses revealed normal results. We suggest that these three patients have a new type of mandibulofacial dysostosis. As all three cases are sporadic and both sexes are affected the pattern of inheritance might be autosomal dominant or autosomal recessive. Identification of additional patients will allow to further delineate the phenotype, to assign the inheritance pattern and to identify the molecular basis.

  9. Hepatic ADC map as an adjunct to conventional abdominal MRI to evaluate hepatic fibrotic and clinical cirrhotic severity in biliary atresia patients

    International Nuclear Information System (INIS)

    Peng, Steven Shinn-Forng; Jeng, Yung-Ming; Hsu, Wen-Ming; Ho, Ming-Chih; Yang, Justin Cheng-Ta

    2015-01-01

    Hepatic apparent diffusion coefficient (ADC) values and ADC-related indices were correlated with the Mayo risk score for primary biliary cirrhosis (MRSPBC) and METAVIR scores of liver specimens to determine the clinical and pathological significance of diffusion-weighted magnetic resonance imaging (DWMRI). Thirty-two patients with biliary atresia (BA; mean age 461 days, range 11-4616 days) received magnetic resonance examinations from March 2009 to August 2013. A free-breathing DWMRI sequence was performed with the single-shot echo-planar imaging technique with b = 0 and 500 s/mm 2 in all 32 BA patients and 24 controls. We used the ordinal logistic regression test and Spearman rank correlation test to analyse the relationships between the MRSPBC and METAVIR fibrosis scores and right liver-to-psoas ADC ratios (LTPARs). BA patients had significantly lower LTPARs in both hepatic lobes than controls (p < 0.01). Right LTPARs, showing moderate intraobserver agreement (intraclass correlation coefficient = 0.736) and interobserver reliability (intraclass correlation coefficient = 0.659), were negatively correlated with MRSPBC and METAVIR fibrosis scores (R 2 = 0.398, p = 0.024 and R 2 = 0.628, p < 0.001, respectively). Right LTPARs may be used for long-term follow-up of cirrhosis severity in BA patients. (orig.)

  10. Pre-operative computed tomography evaluation of congenital aural atresia in children; Bilan tomodensitometrique pre-operatoire des aplasies majeures de l`oreille chez l`enfant

    Energy Technology Data Exchange (ETDEWEB)

    Lumbroso, C.; Sebag, G.; Argyropoulou, M.; Manach, Y.; Lallemand, D. [Hopital Necker-Enfants-Malades, 75 - Paris (France)

    1995-04-01

    To describe computed tomography (CT) findings in congenital aural atresia (CAA) and to illustrate the impact of these findings in the preoperative evaluation. A narrow bony external auditory canal (EAC) was present in 24% of the cases. In one of these cases, the EAC contained a cholesteatoma and was consequently a clear indication of surgery. An hyperpneumatized mastoid (22%), a posterior position of the temporo mandibular joint (16%), and a bulging medial temporal fossa (12%) made the operation much more difficult. The tympanic cavity was small in 68% of the cases, normal in 28% and absent in 4% of the cases without any detectable ossicular chain. Ossicular chain anomalies were present in 91% of the cases. This consisted most frequently of a fusion of the malleus and the incus (76%) with or without fusion of the tympanic wall (33%). Lateral and anterior displacement of the descending portion of the facial nerve was present in 62%. Oval and round windows were normal in 86% of the cases. A soft tissue opacity (33%) in the tympanic cavity made it difficult to evaluate the stapes, the incudo stapedial articulation, and the facial nerve. Simultaneous abnormalities of inner ear were exceptional. (authors). 9 refs., 8 figs.

  11. Oesophageal elongation with traction sutures (FOKER procedure in a newborn baby with long-gap oesophageal atresia (LGEA: Maybe too early, maybe too dangerous?

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    Holger Till

    2013-01-01

    Full Text Available In children with long gap oesophageal atresia (LGEA, the FOKER technique (oesophageal elongation with traction sutures has been criticized for its high complication rate. We advocate analysing such problems to increase the safety in the future. The present case report will focus on timing. A female newborn (3000 g with LGEA (gap of 5 cm was delivered in an outward hospital. On day two of life, she received traction sutures on both pouches. By day five, all sutures had torn out, and a primary anastomosis was attempted. However, it leaked severely. Thus, on day ten, the oesophagus was approached from the neck converting the proximal end into a spit fistula and closing the distal end blindly. Furthermore, the gastro-oesophageal (GE- junction was wrapped with a Teflon sling. When the baby arrived in our institution, she suffered from cavernous oesophageal masses extending from the thoracic inlet down to the diaphragm and fistulas draining them into the neck as well as into the right lung. Moreover, the Teflon sling had dislodged allowing for GE-reflux. In several stages, the oesophageal remnants were resected without any complications. Finally, Prof. Alaa Hamza performed a colonic interposition, which is working well today. In conclusion, the present case aims to caution paediatric surgeons to apply traction sutures for oesophageal elongation in newborns with LGEA.

  12. Macrophage Migration Inhibitory Factor (MIF) Gene Promotor Polymorphism Is Associated with Increased Fibrosis in Biliary Atresia Patients, but Not with Disease Susceptibility.

    Science.gov (United States)

    Sadek, Khaled H; Ezzat, Sameera; Abdel-Aziz, Samira A; Alaraby, Hanaa; Mosbeh, Asmaa; Abdel-Rahman, Mohamed H

    2017-09-01

    Two polymorphisms, rs755622 and rs5844572, in the promoter region of the macrophage migration inhibitory factor (MIF) gene influence the basal and/or induced transcriptional activity and have been linked to several inflammatory and autoimmune diseases. The aim of this study was to investigate the association between these two polymorphisms and disease susceptibility in patients with biliary atresia (BA). Allele frequencies of rs755622 and rs5844572 were assessed in 60 Egyptian infants with a confirmed diagnosis of BA. DNA was extracted from archival material. For the rs755622, samples were tested using Taqman real-time PCR, and for the rs5844572, samples were tested using fluorescence-based genotyping. The allele frequency in the general population was assessed in 141 healthy adults from the same geographical location. No statistical differences were observed in the allele frequencies of either rs755622 or rs5844572 between BA patients and controls. The homozygous and heterozygous short repeats (5/5, or 5/X) of rs5844572 were observed more frequently (16/28, 57.1%) in BA patients with mild to moderate fibrosis compared with those with marked fibrosis (10/32, 31.3%). The difference was statistically significant (P  =  0.032). In conclusion, we observed no association between MIF rs755622 and rs5844572 polymorphisms and susceptibility to BA; however, the rs5844572 could be linked to the rate of progression of the disease and extent of fibrosis. © 2017 John Wiley & Sons Ltd/University College London.

  13. Hepatic ADC map as an adjunct to conventional abdominal MRI to evaluate hepatic fibrotic and clinical cirrhotic severity in biliary atresia patients

    Energy Technology Data Exchange (ETDEWEB)

    Peng, Steven Shinn-Forng [National Taiwan University, Department of Medical Imaging, Medical College and Hospital, Taipei (China); Jeng, Yung-Ming [National Taiwan University, Department of Pathology, Medical College and Hospital, Taipei (China); Hsu, Wen-Ming; Ho, Ming-Chih [National Taiwan University, Department of Surgery, Medical College and Hospital, Taipei, Taiwan (China); Yang, Justin Cheng-Ta [National Taiwan University, Department of Medical Imaging, Medical College and Hospital, Taipei (China); National Taiwan University, Institute of Biomedical Engineering, College of Engineering and College of Medicine, Taipei (China)

    2015-10-15

    Hepatic apparent diffusion coefficient (ADC) values and ADC-related indices were correlated with the Mayo risk score for primary biliary cirrhosis (MRSPBC) and METAVIR scores of liver specimens to determine the clinical and pathological significance of diffusion-weighted magnetic resonance imaging (DWMRI). Thirty-two patients with biliary atresia (BA; mean age 461 days, range 11-4616 days) received magnetic resonance examinations from March 2009 to August 2013. A free-breathing DWMRI sequence was performed with the single-shot echo-planar imaging technique with b = 0 and 500 s/mm{sup 2} in all 32 BA patients and 24 controls. We used the ordinal logistic regression test and Spearman rank correlation test to analyse the relationships between the MRSPBC and METAVIR fibrosis scores and right liver-to-psoas ADC ratios (LTPARs). BA patients had significantly lower LTPARs in both hepatic lobes than controls (p < 0.01). Right LTPARs, showing moderate intraobserver agreement (intraclass correlation coefficient = 0.736) and interobserver reliability (intraclass correlation coefficient = 0.659), were negatively correlated with MRSPBC and METAVIR fibrosis scores (R{sup 2} = 0.398, p = 0.024 and R{sup 2} = 0.628, p < 0.001, respectively). Right LTPARs may be used for long-term follow-up of cirrhosis severity in BA patients. (orig.)

  14. Fare astronomia con piccoli telescopi

    CERN Document Server

    Gainer, Michael K

    2007-01-01

    Non sono necessariamente richiesti strumenti mastodontici per produrre risultati scientificamente validi nel campo dell’astronomia. Anche l’astrofilo dotato di un piccolo telescopio, con un diametro di soli 8-9 cm, può contribuire alla scienza del cielo realizzando utili osservazioni del Sole, della Luna, dei pianeti, delle comete, degli asteroidi, delle stelle doppie o variabili, delle nebulose e degli ammassi stellari. Il manuale di M.K. Gainer spiega quale sia la dotazione minima (un piccolo telescopio, un computer, una semplice fotocamera digitale), come utilizzarla, e quali siano le tecniche appropriate da adottare nelle osservazioni. Offre inoltre schemi per interpretare e ridurre i dati raccolti, nonché schede da compilare e da spedire ai centri di raccolta internazionali. Questo libro è il passaporto grazie al quale l’astrofilo può entrare a pieno titolo nel mondo affascinante della scienza astronomica.

  15. Depresion en pacientes con alteraciones del tiroides

    OpenAIRE

    Radanovic-Grguric´, Ljiljana; Filakovic´, Pavo; Barkic´, Jelena; Mandic´, Nikola; Karner, Ivan; Smoje, Juraj

    2003-01-01

    Nuestro estudio fue realizado en un grupo de 53 mujeres con disfunción tiroidea y 28 mujeres con depresión mayor. Empleamos la Escala de la Depresión de Hamilton, la Escala de Autoevaluación de la Depresión de Zung y la Escala sobre la Impresión Clínica Global. Los resultados del estudio demuestran que la mayoría de los pacientes con disfunción tiroidea se mostraron clínicamente significativos en cuanto al trastorno depresivo. Los episodios depresivos son más frecuentes en pacientes con hipot...

  16. Aprende Ajedrez con Rey - Parte 2

    OpenAIRE

    ESTÉVEZ MONTERO, RAÚL; Lloret Mauri, Jaime

    2016-01-01

    Es una pieza audiovisual creada con el objeto de atraer la atención de los niños de muy corta edad con el ajedrez y familiarizarlos con todas sus piezas y movimientos. Es una animación dirigida a un público infantil presentada por dibujos animados en 2D, en la que se ha intentado respetar en todo momento el argot de la comunidad ajedredística. En este video se presenta la segunda parte. Estévez Montero, R.; Lloret Mauri, J. (2016). Aprende Ajedrez con Rey - Parte 2. http://hdl.handle.net/1...

  17. Aprende Ajedrez con Rey - Parte 1

    OpenAIRE

    ESTÉVEZ MONTERO, RAÚL; Lloret Mauri, Jaime

    2016-01-01

    Es una pieza audiovisual creada con el objeto de atraer la atención de los niños de muy corta edad con el ajedrez y familiarizarlos con todas sus piezas y movimientos. Es una animación dirigida a un público infantil presentada por dibujos animados en 2D, en la que se ha intentado respetar en todo momento el argot de la comunidad ajedredística. En este video se presenta la primera parte. Estévez Montero, R.; Lloret Mauri, J. (2016). Aprende Ajedrez con Rey - Parte 1. http://hdl.handle.net/1...

  18. Utilização da distração osteogênica mediana sagital para tratamento da atresia mandibular Mid-sagital mandibular distraction osteogenesis for treatment of lower arch crowding

    Directory of Open Access Journals (Sweden)

    Luiz Guilherme Martins Maia

    2007-06-01

    Full Text Available OBJETIVO: relatar o tratamento de uma paciente com apinhamento severo decorrente de uma atresia maxilomandibular, tratada por meio da Distração Osteogênica da Sínfise Mandibular (DOSM. RESULTADOS: neste caso, observamos bom engrenamento dentário, diminuição do corredor bucal escuro, correção do apinhamento, boa forma de arcos e boa estética facial, num período de tempo bastante satisfatório, sem trazer dano ao tecido periodontal adjacente. CONCLUSÃO: pacientes com arcos atrésicos e apinhamento dentário podem ser beneficiados com a DOSM que, quando bem indicada, surge como uma nova forma de tratamento para casos limítrofes de apinhamento dentário, nos quais as alternativas convencionais oferecem limitações, tais como o achatamento do perfil facial, o aumento do corredor bucal e a instabilidade.OBJECTIVE: To report the treatment of a patient with severe dental crowding resulting from a maxillary mandibular atresia, treated by the osteogenic extraction of the mandible symphysis (DOSM. RESULTS: In this case there was good dental engagement, a reduction of the dark oral aisle, a correction of the crowding, good dental arch form and good facial esthetics, in a very satisfactory period of time without causing damage to the adjacent periodontal tissues. CONCLUSION: Patients with atresia arches and dental crowding can be benefited by a DOSM that when well indicated, appears as a new form of treatment in cases of maximum borderline dental crowding, in which the conventional alternatives have limitations such as flattening of the facial profile, increase in the oral aisle and instability.

  19. Plantas cubanas con efecto antiinflamatorio

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    Ada Ivis Regalado Veloz

    Full Text Available La actividad antiinflamatoria suscita gran interés científico en el área farmacológica, debido a que muchas enfermedades en su evolución cursan por procesos inflamatorios (artritis reumatoide, ateroesclerosis, cáncer, diabetes, gota, asma, dermatitis, trastornos neurodegenerativos y diversas dolencias menores. Las enfermedades inflamatorias constituyen un problema de salud importante, debido a la falta de medicamentos eficaces y seguros para su uso por periodos prolongados. Hoy en día se trabaja en la búsqueda de alternativas de antiinflamatorios más seguros, en el que las plantas medicinales, una de las formas más antiguas de tratamiento, constituyen una elección a considerar. En este trabajo se realizó una revisión bibliográfica, sobre especies de plantas que crecen en Cuba que le reportan propiedades farmacológicas como antinflamatorios. En la revisión de la literatura se utilizó la base de datos Medline (vía PubMed, así como revistas nacionales desde el periodo de 2000 hasta el presente, con las palabras claves "inflamación" y "plantas cubanas antiinflamatorias" o "actividad antiinflamatoria" y "plantas medicinales".

  20. Hospitalidad, con y sin papeles

    Directory of Open Access Journals (Sweden)

    Ana Paula Penchaszadeh

    Full Text Available Resumen El objetivo de este artículo es vincular el trabajo sobre el archivo de Jacques Derrida con la experiencia de la hospitalidad. Se intentará mostrar que, por un lado, se trata siempre de los papeles, de la legitimidad que éstos otorgan o no tanto a nivel filosófico (deseo de poseer los papeles que autoricen tal o cual decisión interpretativa, como a nivel político ("tener papeles" como el principio básico de todo derecho a tener derechos, de todo derecho a la comunidad. Mas también, por otro lado, se intentará pensar aquello que arruina la idea misma de tener o no tener (papeles, la idea de propiedad, aquello que hace imposible fundar una decisión o identidad en última instancia y, por ende, una soberanía, una frontera. La hospitalidad, la llegada inminente del otro, representa un desafío político y ético para la filosofía: pues no se trata de un saber, sino de una experiencia transformando el sustrato del nos-otros, del ser común.

  1. Itinerari Musicali con la Wiild

    Directory of Open Access Journals (Sweden)

    Elisabetta Nanni

    2013-03-01

    Full Text Available La Wiild, acronimo di Wiimote Lavagna Digitale, è uno strumento didattico che utilizza il telecomando della Wii, il famoso gioco della Nintendo, insieme a un software libero, rendendolo così estremamente versatile. Non vincolato a software proprietario, il suo utilizzo è legato alla capacità dell’insegnante di ripartire dalla didattica, dalle risorse selezionate e dall’epistemologia di ogni singola disciplina, trovando così nel proprio contesto un ruolo per le tecnologie. Il contributo presenta ipotesi di lavoro per l’educazione musicale nella scuola secondaria di primo grado che si sviluppano sia attraverso lo studio del rapporto suono/segno con affinità pittoriche e successiva codificazione grafica, sia attraverso un’attività di laboratorio in cui co-costruire percorsi storico-musicali. La Wiild diventerà davvero utile ed efficace nel momento in cui, affiancando le risorse selezionate dal docente, verrà utilizzata senza essere notata, giocando un ruolo di strumento tecnologico «normale e trasparente».

  2. Importância do diagnóstico fetal de atresia de esôfago para a suspeição clínica de síndrome de Edwards

    Directory of Open Access Journals (Sweden)

    Luciano Vieira Targa

    2012-04-01

    Full Text Available Resumo Nosso objetivo é relatar um feto cuja confirmação diagnóstica de atresia de esôfago através da ressonância nuclear magnética (RNM levou à suspeita de síndrome de Edwards. A gestante apresentava 28 anos e veio inicialmente à consulta na medicina fetal com 24 semanas de gravidez. Possuía ultrassom fetal com polidrâmnio, artéria umbilical única e não visualização da bolha gástrica. A RNM fetal evidenciou a presença de uma estrutura similar a uma bolsa dilatada ao nível torácico, na topografia da porção proximal do esôfago, o que confirmou o diagnóstico de atresia de esôfago. Neste momento, suspeitou-se do diagnóstico de cromossomopatia, em especial, de síndrome de Edwards. Contudo, o exame de cariótipo não pôde ser realizado no momento. A ecocardiografia fetal de pouco antes do nascimento mostrou uma tetralogia de Fallot. A criança nasceu de parto cesáreo, com 33 semanas de gestação, pesando 1460 g. Observou-se na sua avaliação a presença de dismorfias como camptodactilia dos dedos das mãos e calcâneos proeminentes. A avaliação cariotípica confirmou a suspeita de síndrome de Edwards. Os exames complementares também confirmaram a presença de atresia de esôfago com fístula traqueoesofágica. A síndrome de Edwards é uma anormalidade cromossômica relativamente comum caracterizada por um espectro clínico bastante amplo e uma limitada sobrevida. A RNM fetal, por sua vez, tem o potencial de complementar a avaliação realizada pelo US na elucidação de algumas malformações maiores fetais, tal como a atresia de esôfago. Estas possuem o potencial de serem marcadores para anormalidades cromossômicas, tal como a síndrome de Edwards.

  3. Bases tratadas con cemento, en California

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    Chinchilla, M.

    1962-05-01

    Full Text Available El uso de bases tratadas con cemento para autopistas se inició en el Estado de California en 1938, empleándose para carreteras con determinadas condiciones de tráfico. Inicialmente, se especificó el uso obligatorio de plantas mezcladoras para asegurar el debido control de las proporciones adecuadas.

  4. Interactions between Th1 cells and Tregs affect regulation of hepatic fibrosis in biliary atresia through the IFN-γ/STAT1 pathway.

    Science.gov (United States)

    Wen, Jie; Zhou, Ying; Wang, Jun; Chen, Jie; Yan, Wenbo; Wu, Jin; Yan, Junkai; Zhou, Kejun; Xiao, Yongtao; Wang, Yang; Xia, Qiang; Cai, Wei

    2017-06-01

    Regulatory T cells (Tregs) and CD4 + T helper (Th) cells have important roles in bile duct injury of biliary atresia (BA). However, their impacts on liver fibrosis are undefined. Between 2013 and 2016, 146 patients with various stages of BA were enrolled in this study. Peripheral blood, liver biopsy and lymph node samples were collected. Flow cytometry, magnetic cell sorting and immunostaining were used to characterize lymphocytes from BA patients. Deficiency of Tregs was observed along with increased Th1, Th2 and Th17 frequencies in the peripheral blood and livers of BA patients. The levels of peripheral and intrahepatic Th1 cells positively correlated with the stage of liver fibrosis. Furthermore, Th1 cells were located in close proximity to activated hepatic stellate cells (HSCs) and areas of fibrosis in BA livers. In culture, Th1 cells accelerated the proliferation and secretion of profibrogenic markers of HSCs through the IFN-γ/STAT1 pathway. Of note, Tregs blocked the Th1-stimulated effects on HSCs by inhibiting Th1-induced activation of STAT1. Consistent with the results of in vitro study, intrahepatic IFN-γ/STAT1 levels increased in relation to the severity of liver fibrosis in BA patients, and the altered balance between MMP2 and TIMP1 expressions in livers may contribute to increased deposition of extracellular matrix and fibrosis. Finally, to identify the effects of Th1 cells on Tregs, we demonstrated that Th1 cells upregulated the proportion of aTreg cells by secreting IFN-γ cytokine. Thus, aberrant Th1 immune responses in BA promote the proliferation and secretion of HSCs through the IFN-γ/STAT1 pathway. The regulation of HSCs by the interactions between Tregs and Th1 cells might be part of the mechanism underlying progressive liver fibrosis and may be a suitable target for therapy.

  5. Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: A Congenital Heart Surgeons Society study

    Science.gov (United States)

    Karamlou, Tara; Poynter, Jeffrey A.; Walters, Henry L.; Rhodes, Jonathan; Bondarenko, Igor; Pasquali, Sara K.; Fuller, Stephanie M.; Lambert, Linda M.; Blackstone, Eugene H.; Jacobs, Marshall L.; Duncan, Kim; Caldarone, Christopher A.; Williams, William G.; McCrindle, Brian W.

    2013-01-01

    Background A bias favoring biventricular (BV) repair exists regarding choice of repair pathway for patients with pulmonary atresia with intact ventricular septum (PAIVS). We sought to determine the implications of moving borderline candidates down a BV route in terms of late functional health status (FHS) and exercise capacity (EC). Methods Between 1987 and 1997, 448 neonates with PAIVS were enrolled in a multi-institutional study. Late EC and FHS were assessed following repair (mean 14 years) using standardized exercise testing and 3 validated FHS instruments. Relationships between FHS, EC, morphology, and 3 end states (ie, BV, univentricular [UV], or 1.5-ventricle repair [1.5V]) were evaluated. Results One hundred two of 271 end state survivors participated (63 BV, 25 UV, and 14 1.5V). Participants had lower FHS scores in domains of physical functioning (P < .001) compared with age- and sex-matched normal controls, but scored significantly higher in nearly all psychosocial domains. EC was higher in 1.5V-repair patients (P = .02), whereas discrete FHS measures were higher in BV-repair patients. Peak oxygen consumption was low across all groups, and was positively correlated with larger initial tricuspid valve z-score (P < .001), with an enhanced effect within the BV-repair group. Conclusions Late patient-perceived physical FHS and measured EC are reduced, regardless of PAIVS repair pathway, with an important dichotomy whereby patients with PAIVS believe they are doing well despite important physical impediments. For those with smaller initial tricuspid valve z-score, achievement of survival with BV repair may be at a cost of late deficits in exercise capacity, emphasizing that better outcomes may be achieved for borderline patients with a 1.5V- or UV-repair strategy. PMID:23374986

  6. Prognos is of living donor liver transplantation performed during different periods in infants with biliary atresia: a single-center experience with 101 cases

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    HUA Xiangwei

    2014-01-01

    Full Text Available ObjectiveTo compared the prognosis between infants with biliary atresia (BA who underwent living donor liver transplantation (LDLT during 2006-2009 and 2010-2012 in the Department of Liver Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine and to evaluate the prognos is of LDLT performed during different periods. MethodsA retrospective analysis was performed on the clinical data of 101 infants with BA who underwent LDLT from October 2006 to December 2012. These patients were divided into 2006-2009 group and 2010-2012 group according to the year the procedure was performed. The two groups were compared in terms of preoperative general data, intraoperative status, and treatment outcome. The Kaplan-Meier method was used for survival analysis; measurement data were analyzed by t test, and enumeration data were analyzed by Fisher exact probability test and chi-square test. ResultsThe preoperative general data showed no significant difference between the two groups. Compared with the 2006-2009 group, the 2010-2012 group had significantly less intraoperative blood loss (t=2.05, P=0.04, a significantly shorter cold ischemia time of donor liver (t=3.25, P<0.01, and significantly lower incidence of postoperative biliary complications (χ2=4.27, P=0.04, pulmonary infection (χ2=4.47, P=0.03, and acute rejection (P=0.03. The 2010-2012 group had significantly improved survival compared with the 2006-2009 group; the 1 and 2 year cumulative survival rates for the 2010-2012 group were 884% and 88.4%, respectively, versus 84.4% and 75% for the 2006-2009 group. ConclusionThe development of surgical techniques, improvement in perioperative management, and standardized follow-up can significantly increase the success rate of surgery and survival rate and reduce postoperative complications in BA patients.

  7. Near-infrared fluorescence cholangiography with indocyanine green for biliary atresia. Real-time imaging during the Kasai procedure: a pilot study.

    Science.gov (United States)

    Hirayama, Yutaka; Iinuma, Yasushi; Yokoyama, Naoyuki; Otani, Tetsuya; Masui, Daisuke; Komatsuzaki, Naoko; Higashidate, Naruki; Tsuruhisa, Shiori; Iida, Hisataka; Nakaya, Kengo; Naito, Shinichi; Nitta, Koju; Yagi, Minoru

    2015-12-01

    Hepatoportoenterostomy (HPE) with the Kasai procedure is the treatment of choice for biliary atresia (BA) as the initial surgery. However, the appropriate level of dissection level of the fibrous cone (FC) of the porta hepatis (PH) is frequently unclear, and the procedure sometimes results in unsuccessful outcomes. Recently, indocyanine green near-infrared fluorescence imaging (ICG-FCG) has been developed as a form of real-time cholangiography. We applied this technique in five patients with BA to visualize the biliary flow at the PH intraoperatively. ICG was injected intravenously the day before surgery as the liver function test, and the liver was observed with a near-infrared camera system during the operation while the patient's feces was also observed. In all patients, the whole liver fluoresced diffusely with ICG-containing stagnant bile, whereas no extrahepatic structures fluoresced. The findings of the ICG fluorescence pattern of the PH after dissection of the FC were classified into three types: spotty fluorescence, one patient; diffuse weak fluorescence, three patients; and diffuse strong fluorescence, one patient. In all five patients, the feces evacuated after HPE showed distinct fluorescent spots, although that obtained before surgery showed no fluorescence. One patient with diffuse strong fluorescence who did not achieve JF underwent living related liver transplantation six months after the initial HPE procedure. Four patients, including three cases involving diffuse weak fluorescence and one case involving spotty fluorescence showed weak fluorescence compared to that of the surrounding liver surface. We were able to detect the presence of bile excretion at the time of HPE intraoperatively and successfully evaluated the extent of bile excretion using this new technique. Furthermore, the ICG-FCG findings may provide information leading to a new classification and potentially function as an indicator predicting the clinical outcomes after HPE.

  8. Posterior Tracheopexy for Severe Tracheomalacia Associated with Esophageal Atresia (EA: Primary Treatment at the Time of Initial EA Repair versus Secondary Treatment

    Directory of Open Access Journals (Sweden)

    Hester F. Shieh

    2018-01-01

    Full Text Available PurposeWe review outcomes of posterior tracheopexy for tracheomalacia in esophageal atresia (EA patients, comparing primary treatment at the time of initial EA repair versus secondary treatment.MethodsAll EA patients who underwent posterior tracheopexy from October 2012 to September 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores, and persistent airway intrusion were collected. Indication for posterior tracheopexy was the presence of clinical symptoms, in combination with severe tracheomalacia as identified on bronchoscopic evaluation, typically defined as coaptation in one or more regions of the trachea. Secondary cases were usually those with chronic respiratory symptoms who underwent bronchoscopic evaluation, whereas primary cases were those found to have severe tracheomalacia on routine preoperative dynamic tracheobronchoscopy at the time of initial EA repair.ResultsA total of 118 patients underwent posterior tracheopexy: 18 (15% primary versus 100 (85% secondary cases. Median (interquartile range age was 2 months (1–4 months for primary (22% type C and 18 months (8–40 months for secondary (87% type C cases (p < 0.001. There were statistically significant improvements in most clinical symptoms postoperatively for primary and secondary cases, with no significant differences in any postoperative symptoms between the two groups (p > 0.1. Total tracheomalacia scores improved significantly in primary (p = 0.013 and secondary (p < 0.001 cases. Multivariable Cox regression analysis indicated no differences in persistent airway intrusion requiring reoperation between primary and secondary tracheopexy adjusting for imbalances in age and EA type (p = 0.67.ConclusionPosterior tracheopexy is effective in treating severe tracheomalacia with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy. With no significant differences in outcomes between primary and

  9. Classificação anatômica e correção cirúrgica da atresia pulmonar com comunicação interventricular Anatomical classification and surgical repair of the pulmonary atresia with ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre CROTI

    2001-12-01

    Full Text Available OBJETIVO: Analisar as características anatômicas, o resultado das técnicas empregadas na correção cirúrgica de acordo com o número de procedimentos, assim como a mortalidade em cada grupo da classificação de Barbero-Marcial para atresia pulmonar com comunicação interventricular. CASUÍSTICA E MÉTODOS: De janeiro de 1990 a novembro de 1999, 73 pacientes que foram submetidos a estudo cineangiocardiográfico previamente à primeira intervenção cirúrgica, foram analisados. As características anatômicas das artérias pulmonares e artérias colaterais sistêmico-pulmonares, assim como as técnicas cirúrgicas que propiciaram tratamento paliativo, "paliativo definitivo" e definitivo foram estudadas. As causas de mortalidade também foram descritas. RESULTADOS: Dezenove pacientes apresentavam os segmentos pulmonares supridos por artérias pulmonares (grupo A, 45 por artérias pulmonares e artérias colaterais sistêmico-pulmonares (grupo B e 9 somente por artérias colaterais sistêmico-pulmonares (grupo C. O grupo A apresentou maior proporção de tratamentos definitivos, o grupo B maior proporção de paliativos e o grupo C, maior proporção de "paliativos definitivos" (pOBJECTIVE: To analyze the morphological aspects, the surgical results obtained according to the number of procedures, and the mortality in each group of Barbero-Marcial´s classification of the pulmonary atresia with ventricular septal defect. MATERIAL E METHODS: From January 1990 to November 1999, 73 patients submitted to cardiac catheterization and detailed pulmonary angiographic study before the first surgical intervention were analyzed. The anatomical characteristics of the pulmonary arteries and major aorticopulmonary collaterals, as the surgical techniques of definitive, palliative and "definitive palliative" were studied. The causes of mortality were also described. RESULTS: Nineteen patients had all the pulmonary segments supplied by pulmonary arteries (group

  10. Fuerza manual de adultos con discapacidad intelectual

    Directory of Open Access Journals (Sweden)

    Ruth Cabeza Ruiz

    2017-09-01

    Full Text Available Objetivo. Presentar una descripción de la fuerza de prensión manual de hombres y mujeres con discapacidad intelectual (DI y comparar los resultados con valores de referencia de otras personas con y sin discapacidad intelectual. Método. El presente trabajo es un estudio transversal observacional, financiado por la Fundación SAMU, en el que se evaluaron a 122 personas con DI (86 hombres y 36 mujeres durante el desarrollo de unas jornadas de carácter recreativo en las que participaron varias asociaciones de atención a este colectivo. La batería de test utilizada fue el Alpaha-Fit Test Battery for Adults. Resultados. Se presentan los resultados relacionados con las variables de fuerza del miembro superior (Hand Grip Strength por grupos de edad (20-24, 25- 29, 30-34, 35-39, 40-44, 45-49, 50-54, 55-59 años. Los datos muestran valores que oscilan desde los 31 kg en los hombres más jóvenes con DI hasta los 13.3 kg del grupo más maduro de mujeres. Estos hallazgos son similares a los valores de referencia de población con DI española. Sin embargo, son muy inferiores a los obtenidos por la población sin discapacidad de la misma edad. Conclusión. Los resultados evidencian el menor rendimiento de las personas con DI en pruebas de fuerza de prensión manual por lo que se hace evidente la necesidad de llevar a cabo programas de ejercicio físico o deporte con las personas con DI.

  11. Experiencias de haber crecido con un padre/madre con trastorno mental severo (TMS)

    OpenAIRE

    Vivanco B, Gabriela; Grandón F, Pamela

    2016-01-01

    Introducción. La experiencia de vivir con personas que presentan un Trastorno Mental Severo (TMS) es difícil para las familias, en especial para los hijos quienes han sido poco estudiados. El objetivo de la investigación fue conocer cómo la experiencia de haber vivido con un padre o madre con un trastorno mental severo influyó en la infancia, adolescencia y adultez joven de sus hijos e hijas. Método. Se analizan las experiencias de convivencia con un padre/madre con TMS en 10 hijos (6 hombres...

  12. El maltrato en las personas con discapacidad

    OpenAIRE

    Revuelta, Lucerga

    2014-01-01

    El maltrato no solo se realiza por acción sino también por omisión, la indiferencia hacia la persona con discapacidad es una forma de maltrato muy frecuente. Por ejemplo, ignorar y desatender las necesidades de la persona con discapacidad o, al contrario, la sobreprotección son maneras de maltrato. Cuando a un niño con discapacidad el padre o cuidador le hace todo, el niño se siente agredido pues le están incapacitando más de lo que su enfermedad ya lo hace.

  13. Hiperalgesia asociada al tratamiento con opioides

    OpenAIRE

    A. Gil Martín; M. Moreno García; J. Sánchez-Rubio Ferrández; T. Molina García

    2014-01-01

    La hiperalgesia inducida por opioides es una reacción paradójica caracterizada por una percepción intensificada de dolor relacionada con el uso de estos medicamentos en ausencia de progresión de la enfermedad o de síndrome de retirada. A diferencia de los casos de tolerancia, definida como pérdida de potencia analgésica durante el uso prolongado de opioides, no se produce mejoría con el escalado de dosis. La hiperalgesia inducida por opioides se ha manifestado en pacientes con dosis de manten...

  14. Tratamiento conservador en pacientes con retinoblastoma bilateral

    Directory of Open Access Journals (Sweden)

    Juan C. Suárez

    2008-11-01

    Full Text Available OBJETIVO: comparar el tratamiento convencional del retinoblastoma bilateral, usado hasta hace algunos años, consistente en radioterapia o enucleación bilateral, con el tratamiento conservador actual que incluye termoterapia transpupilar (TTT o TTT/quimioterapia al menos en un ojo, en niños con diagnóstico de retinoblastoma bilateral. DISEÑO: estudio retrospectivo descriptivo. MUESTRA: 20 pacientes con diagnóstico de retinoblastoma bilateral que consultaron al Hospital Universitario San Vicente de Paúl, de Medellín, Colombia, entre 1997 y 2007. MÉTODO: se hizo enucleación del ojo con el tumor de mayor tamaño. En el otro ojo se hizo tratamiento con TTT, con el láser diodo (810 nm, spot amplio, solo o combinado con otras terapias. RESULTADOS: se dividió a los pacientes en dos grupos: 16 pacientes (32 ojos en el grupo 1 tratados conservadoramente y 4 pacientes (8 ojos en el grupo 2 con tratamiento convencional. El rango de edad fue de 1-72 meses en el grupo 1 y de 1-12 meses en el grupo 2. El tiempo de seguimiento fue de 7-67 meses para el grupo 1 y de 13-73 meses para el grupo 2. En el grupo 1 se hizo enucleación de 16 ojos (50%, radioterapia externa de uno (3,1%, quimioterapia más termoterapia de 5 (15,6% y quimioterapia más termoterapia más crioterapia de 10 (31,3%. En todos los pacientes se logró preservar al menos un ojo. En el grupo 2, se enuclearon 7 ojos (87,5% y se hizo radioterapia externa más enucleación en un paciente (12.5%. Además, todos los pacientes recibieron quimioterapia. CONCLUSIÓN: la terapia conservadora actual consistente en tratamiento local (termoterapia, crioterapia o braquiterapia y quimiorreducción permite preservar al menos un ojo y en algunos casos de los dos, muchas veces con buena agudeza visual, en niños con retinoblastoma bilateral; se evitan así la enucleación bilateral y la radioterapia externa usada en el tratamiento convencional con todos sus efectos secundarios. La enucleación contin

  15. Paciente con tumor de cuerpo carotideo

    Directory of Open Access Journals (Sweden)

    Mariuska Forteza Sáez

    Full Text Available Los tumores de cuerpo carotideo (paragangliomas son neoplasias altamente vascularizadas, muy poco frecuentes y generalmente benignas, originadas en los quimiorreceptores del cuerpo carotideo. Se presenta el caso de un paciente de 54 años, con aumento de volumen cervical derecho, asintomático, con estudio preoperatorio y angiografía realizados por tomografía axial computarizada, que resultan compatibles con tumor de cuerpo carotideo. Se realiza disección subadventicial, informando la biopsia paraganglioma. El tumor fue completamente resecado, sin evidencia de recurrencia y sin complicaciones.

  16. Presa con revestimiento asfáltico

    Directory of Open Access Journals (Sweden)

    Proudfit, D. P.

    1960-07-01

    Full Text Available La presa denominada Montgomery se halla situada sobre el río South Platte River, a unos 8 km aguas arriba de la ciudad de Alma, del Estado de Colorado (EE. UU., y en la ladera oriental de la cordillera Continental Divide. El cuerpo o dique de esta presa está constituido por piedra, revestida con una capa de hormigón asfáltico en el paramento de contacto con el agua.

  17. Trastornos temporomandibulares en pacientes con maloclusiones

    Directory of Open Access Journals (Sweden)

    Luis Soto Cantero

    Full Text Available Introducción: existe una prevalencia de trastornos oclusales en gran medida relacionados con la presencia de disfunción temporomandibular. Objetivo: determinar el grado de disfunción temporomandibular según el Índice de Maglione en correspondencia con la prevalencia de maloclusiones en el área de salud del policlínico "Turcios Lima". Métodos: se realizó un estudio observacional descriptivo, de corte transversal, en la consulta de ortodoncia, de junio del 2008 a junio del 2009. De un universo constituido por los 280 pacientes que acudieron al servicio de ortodoncia en el período señalado con presencia de maloclusiones, se seleccionó una muestra de 84 pacientes por muestreo aleatorio simple, teniendo en cuenta los criterios de inclusión y exclusión realizados por criterios de expertos. Resultados: presentaban disfunción temporomandibular 74 pacientes y dentro de ellos 44 (52,4 % tenían disfunción grado II (Moderada. El 97 % de los pacientes con relación molar de clase II, presentaban disfunción temporomandibular. El 42,9 % de los pacientes con una maloclusión, presentaban disfunción grado I. El 60 % de los pacientes con dos maloclusiones presentaban disfunción grado II y el 66,7 % de los pacientes con tres maloclusiones, presentaban disfunción grado III. La mayor cantidad de pacientes tuvieron disfunción grado II (Moderada. Conclusiones: el mayor por ciento de los pacientes con disfunciones presentó una clase II molar y a medida que aumentó el número de maloclusiones aumentó también la severidad de la disfunción.

  18. RadCon: A Radiological Consequences Model

    International Nuclear Information System (INIS)

    Crawford, J.; Domel, R.U.

    2000-05-01

    RadCon estimates the dose received by user selected groups in the population from an accidental release of radionuclides to the environment. The exposure pathways considered are external exposure from the cloud and ground and internal exposure from inhalation and ingestion of contaminated food. Atmospheric dispersion modelling is carried out externally to RadCon.Given a two dimensional time varying air and ground concentration of radioactive elements, RadCon allows the user to: view the air and ground concentration over the affected area, select optional parameters and calculate the dose to people,display the results to the user, and change the parameter values. RadCon offers two user interfaces: 1) the standard graphical user interface which is started using Java DoseApp at the command line, or by setting up a shortcut to this command (particularly when RadCon is installed on a PC) and 2) the text based interface used to generate information for the model inter-comparison exercise . This is initiated using Java BIOMASS at the command line, or an equivalent shortcut. The text based interface was developed for research purposes and is not generally available. Appendices A, B and C provide a summary of instructions on setting up RadCon. This will generally be carried out by the computer support personnel

  19. Adolescente femenino con granulomatosis de Wegener fulminante

    Directory of Open Access Journals (Sweden)

    S C Scheffler-Mendoza

    2017-01-01

    Full Text Available Adolescente femenino de 13 años de edad, originaria de Guerrero, grupo étnico mixteco, padres analfabetos y con poco entendimiento del español. Sin otros antecedentes de importancia para el padecimiento. Inició un mes previo con la aparición de una pápula hipercrómica en párpado superior derecho que en los 10 días previos al ingreso aumentó de volumen generando proptosis. Al interrogatorio se negó dolor, lagrimeo, fiebre, o pérdida de peso. Ingresó con fiebre (39.0°C, palidez generalizada, proptosis derecha con exposición de córnea y conjuntiva, eritema periorbitario, movimientos oculares y agudeza visual disminuidos (i.e. contaba dedos a 1 m, pupila con tendencia a midriasis, hiperreactividad a la luz, y fondo de ojo con papila hiperémica edematosa y tortuosidad peripapilar. El resto de exploración no mostró datos relevantes. Se hospitalizó para iniciar tratamiento antimicrobiano parenteral de amplio espectro (i.e. ceftriaxona y vancomicina y abordar proptosis unilateral.

  20. Interleukin 17, Produced by γδ T Cells, Contributes to Hepatic Inflammation in a Mouse Model of Biliary Atresia and Is Increased in Livers of Patients.

    Science.gov (United States)

    Klemann, Christian; Schröder, Arne; Dreier, Anika; Möhn, Nora; Dippel, Stephanie; Winterberg, Thomas; Wilde, Anne; Yu, Yi; Thorenz, Anja; Gueler, Faikah; Jörns, Anne; Tolosa, Eva; Leonhardt, Johannes; Haas, Jan D; Prinz, Immo; Vieten, Gertrud; Petersen, Claus; Kuebler, Joachim F

    2016-01-01

    Biliary atresia (BA) is a rare disease in infants, with unknown mechanisms of pathogenesis. It is characterized by hepatobiliary inflammatory, progressive destruction of the biliary system leading to liver fibrosis, and deterioration of liver function. Interleukin (IL) 17A promotes inflammatory and autoimmune processes. We studied the role of IL17A and cells that produce this cytokine in a mouse model of BA and in hepatic biopsy samples from infants with BA. We obtained peripheral blood and liver tissue specimens from 20 patients with BA, collected at the time of Kasai portoenterostomy, along with liver biopsies from infants without BA (controls). The tissue samples were analyzed by reverse transcription quantitative polymerase chain reaction (PCR), in situ PCR, and flow cytometry analyses. BA was induced in balb/cAnNCrl mice by rhesus rotavirus infection; uninfected mice were used as controls. Liver tissues were collected from mice and analyzed histologically and by reverse transcriptase PCR; leukocytes were isolated, stimulated, and analyzed by flow cytometry and PCR analyses. Some mice were given 3 intraperitoneal injections of a monoclonal antibody against IL17 or an isotype antibody (control). Livers from rhesus rota virus-infected mice with BA had 7-fold more Il17a messenger RNA than control mice (P = .02). γδ T cells were the exclusive source of IL17; no T-helper 17 cells were detected in livers of mice with BA. The increased number of IL17a-positive γδ T cells liver tissues of mice with BA was associated with increased levels of IL17A, IL17F, retinoid-orphan-receptor C, C-C chemokine receptor 6, and the IL23 receptor. Mice that were developing BA and given antibodies against IL17 had lower levels of liver inflammation and mean serum levels of bilirubin than mice receiving control antibodies (191 μmol/L vs 78 μmol/L, P = .002). Liver tissues from patients with BA had 4.6-fold higher levels of IL17 messenger RNA than control liver tissues (P = .02

  1. Preoperative evaluation of the congenital aural atresia on computed tomography; an analysis of the severity of the deformity of the middle ear and mastoid

    Energy Technology Data Exchange (ETDEWEB)

    Tasar, Mustafa [Gulhane Medical School, Department of Radiology, Etlik-Ankara (Turkey)]. E-mail: tasarmus@yahoo.com; Yetiser, Sertac [Gulhane Medical School, Department of ORL and HNS (Turkey); Yildirim, Duzgun [Gulhane Medical School, Department of Radiology, Etlik-Ankara (Turkey); Bozlar, Ugur [Gulhane Medical School, Department of Radiology, Etlik-Ankara (Turkey); Tasar, M. Aysin [Ankara Hospital, Department of Pediatrics (Turkey); Saglam, Mutlu [Gulhane Medical School, Department of Radiology, Etlik-Ankara (Turkey); Ugurel, M. Sahin [Gulhane Medical School, Department of Radiology, Etlik-Ankara (Turkey); Battal, Bilal [Gulhane Medical School, Department of Radiology, Etlik-Ankara (Turkey); Ucoz, Taner [Gulhane Medical School, Department of Radiology, Etlik-Ankara (Turkey)

    2007-04-15

    Objective: To compare the development of temporal bone in normal and atretic ears and to assess some radiological landmarks that could be important in the hearing restoration interventions in such patients. Material and methods: Thirty-five patients with 40 atretic external ears were evaluated with temporal bone CT and compared to a control group of 40 normal ears retrospectively. Using comparable slice levels in all patients, the course and the caliper of the facial canal, the surface area of the incus and malleus, the level of mastoid aeration, the location and anteroposterior diameters of the jugular bulb and sigmoid sinus, the direction and the caliber of the tympanic bony part of the Eustachian tube, area of the middle ear cavity, distance from facial nerve to incudomalleolar joint, to the vestibule and to the jugular bulb were included in the assessment. Non-parametric and parametric statistical tests were used for comparison. Results: In atretic ears middle ear sectional area was found to be smaller at the equivalent plane as compared to control subjects (mean area index: 19.3 mm{sup 2} versus 47.4 mm{sup 2}). Mastoid aeration was low in general and the ossicles in the atretic ears were hypoplastic (mean ossicular sectional area: 8.3 mm{sup 2} versus 11 mm{sup 2}). The distance from the jugular bulb to the facial nerve was significantly lower (mean: 6.2 mm versus 6.8 mm) (p < 0.05) in the atretic ears. Facial canal caliber, distance from the facial canal to the incudomalleolar joint and distance from the facial canal to the vestibule in the atretic ears (means: 1.49, 2.93 and 1.82, respectively) did not show statistically significant difference from the control subjects (means: 1.44, 2.91 and 1.83, respectively) (p > 0.05 for all). Conclusion: External ear atresia is significantly associated with middle ear and mastoid abnormalities. The ossicles were underdeveloped which always have to be considered during reconstructive surgery. Radiologically, in the

  2. RadConEd: A Graphical Data Editor for the Radiological Consequences Model, RadCon

    International Nuclear Information System (INIS)

    Crawford, J.; Domel, R.U.

    2000-05-01

    This document describes the application, RadConEd, which has been designed and implemented to enable users of the RadCon system to update these parameter files. The RadCon system is written in the Java programming language, and as such provides portability across computer platforms. The software described in this report was developed in line with the portability requirements of RadCon, thus providing a uniform user interface across computer platforms and bypassing the need of using system editors. In addition a number of data integrity measures were implemented

  3. Enfermedad coronaria en pacientes con psoriasis

    Directory of Open Access Journals (Sweden)

    Walter Masson

    2013-10-01

    Full Text Available Comunicaciones previas asociaron la psoriasis con la enfermedad coronaria. Desconocemos si en nuestro país o región existe dicha asociación. Se realizó un estudio transversal analizando los datos de la historia clínica electrónica de un sistema de salud de Buenos Aires. Analizamos todos los pacientes mayores de 18 años con diagnóstico de psoriasis entre el 1 de enero de 2003 y el 31 de julio de 2011 y los comparamos con un grupo control, en una relación 2:1, obtenido en forma aleatoria del mismo sistema de salud, apareados por edad y sexo. Determinamos la prevalencia de los factores de riesgo cardiovascular y de enfermedad coronaria. Analizamos la asociación entre la enfermedad coronaria y la psoriasis mediante análisis uni y multivariado. Se incluyeron 3 833 sujetos (1 286 pacientes con psoriasis y 2 547 controles. La prevalencia de hipertensión arterial (50% vs. 38%, p < 0.001, tabaquismo (25% vs. 17%, p < 0.001, diabetes (12% vs. 8%, p < 0.001 y enfermedad coronaria (4.98% vs. 3.06%, p = 0.003 fue mayor en los sujetos con psoriasis en comparación con el grupo control. Independientemente de la edad, la presencia de diabetes, hipertensión arterial o tabaquismo, hubo una asociación significativa entre la enfermedad coronaria y la psoriasis (OR 1.48, IC95% 1.04-2.11, p = 0.03. En conclusión, en esta población de Buenos Aires, los pacientes con psoriasis tuvieron una mayor prevalencia de diabetes, hipertensión arterial, tabaquismo y enfermedad coronaria. La asociación entre la psoriasis y la enfermedad coronaria fue independiente de los factores de riesgo explorados.

  4. Mincle Signaling Promotes Con-A Hepatitis

    Science.gov (United States)

    Greco, Stephanie H.; Torres-Hernandez, Alejandro; Kalabin, Aleksandr; Whiteman, Clint; Rokosh, Rae; Ravirala, Sushma; Ochi, Atsuo; Gutierrez, Johana; Salyana, Muhammad Atif; Mani, Vishnu R.; Nagaraj, Savitha V.; Deutsch, Michael; Seifert, Lena; Daley, Donnele; Barilla, Rocky; Hundeyin, Mautin; Nikifrov, Yuriy; Tejada, Karla; Gelb, Bruce E.; Katz, Steven C.; Miller, George

    2016-01-01

    Concanavalin-A (Con-A) hepatitis is regarded as a T cell-mediated model of acute liver injury. Mincle is a C-type lectin receptor (CLR) that is critical in the immune response to mycobacteria and fungi, but does not have a well-defined role in pre-clinical models of non-pathogen mediated inflammation. Since Mincle can ligate the cell death ligand SAP130, we postulated that Mincle signaling drives intrahepatic inflammation and liver injury in Con-A hepatitis. Acute liver injury was assessed in the murine Con-A hepatitis model using C57BL/6, Mincle−/−, and Dectin-1−/− mice. The role of C/EBPβ and HIF-1α signaling was assessed using selective inhibitors. We found that Mincle was highly expressed in hepatic innate inflammatory cells and endothelial cells in both mice and humans. Furthermore, sterile Mincle ligands and Mincle signaling intermediates were increased in the murine liver in Con-A hepatitis. Most significantly, Mincle deletion or blockade protected against Con-A hepatitis whereas Mincle ligation exacerbated disease. Bone marrow chimeric and adoptive transfer experiments suggested that Mincle signaling in infiltrating myeloid cells dictates disease phenotype. Conversely, signaling via other CLRs did not alter disease course. Mechanistically, we found that Mincle blockade decreased the NF-κβ related signaling intermediates, C/EBPβ and HIF-1α, both of which are necessary in macrophage-mediated inflammatory responses. Accordingly, Mincle deletion lowered production of nitrites in Con-A hepatitis and inhibition of both C/EBPβ and HIF1-α reduced the severity of liver disease. Our work implicates a novel innate immune driver of Con-A hepatitis and, more broadly, suggests a potential role for Mincle in diseases governed by sterile inflammation. PMID:27559045

  5. Mincle Signaling Promotes Con A Hepatitis.

    Science.gov (United States)

    Greco, Stephanie H; Torres-Hernandez, Alejandro; Kalabin, Aleksandr; Whiteman, Clint; Rokosh, Rae; Ravirala, Sushma; Ochi, Atsuo; Gutierrez, Johana; Salyana, Muhammad Atif; Mani, Vishnu R; Nagaraj, Savitha V; Deutsch, Michael; Seifert, Lena; Daley, Donnele; Barilla, Rocky; Hundeyin, Mautin; Nikifrov, Yuriy; Tejada, Karla; Gelb, Bruce E; Katz, Steven C; Miller, George

    2016-10-01

    Con A hepatitis is regarded as a T cell-mediated model of acute liver injury. Mincle is a C-type lectin receptor that is critical in the immune response to mycobacteria and fungi but does not have a well-defined role in preclinical models of non-pathogen-mediated inflammation. Because Mincle can ligate the cell death ligand SAP130, we postulated that Mincle signaling drives intrahepatic inflammation and liver injury in Con A hepatitis. Acute liver injury was assessed in the murine Con A hepatitis model using C57BL/6, Mincle(-/-), and Dectin-1(-/-) mice. The role of C/EBPβ and hypoxia-inducible factor-1α (HIF-1α) signaling was assessed using selective inhibitors. We found that Mincle was highly expressed in hepatic innate inflammatory cells and endothelial cells in both mice and humans. Furthermore, sterile Mincle ligands and Mincle signaling intermediates were increased in the murine liver in Con A hepatitis. Most significantly, Mincle deletion or blockade protected against Con A hepatitis, whereas Mincle ligation exacerbated disease. Bone marrow chimeric and adoptive transfer experiments suggested that Mincle signaling in infiltrating myeloid cells dictates disease phenotype. Conversely, signaling via other C-type lectin receptors did not alter disease course. Mechanistically, we found that Mincle blockade decreased the NF-κβ-related signaling intermediates C/EBPβ and HIF-1α, both of which are necessary in macrophage-mediated inflammatory responses. Accordingly, Mincle deletion lowered production of nitrites in Con A hepatitis and inhibition of both C/EBPβ and HIF-1α reduced the severity of liver disease. Our work implicates a novel innate immune driver of Con A hepatitis and, more broadly, suggests a potential role for Mincle in diseases governed by sterile inflammation. Copyright © 2016 by The American Association of Immunologists, Inc.

  6. Variables socionutricionales de hogares mazahuas integrados por preescolares desnutridos con madres con obesidad y sin obesidad

    Directory of Open Access Journals (Sweden)

    Conzuelo González, Viridiana Vanessa

    2009-01-01

    Full Text Available El primer objetivo fue conocer cuántos menores de cinco años con diferentes grados de desnutrición tienen una madre con sobrepeso/obesidad/ en una comunidad indígena que vive en extrema pobreza y bajo condiciones de migración masculina internacional. El segundo fue comparar tres variables socionutricionales (ingreso familiar, educación de la madre y adecuación nutrimental de la dieta diaria entre estos hogares y los hogares con desnutrición infantil y madres sin obesidad. Se realizó un estudio transversal (2006-2007, en la comunidad mazahua de San Francisco Tepeolulco, Municipio de Temascalcingo; que incluyó a 85 hogares integrados por preescolares con desnutrición inscritos al programa Oportunidades. Se determinó el estado nutrición de los preescolares con indicadores antropométricos y se obtuvo el IMC de las madres de estos infantes. Se aplicó una encuesta socionutricional, incluida el recordatorio de 24 horas, y complementado con la observación participante (cualitativa. Se encontró que 83% de las madres mazahuas presentaron sobrepeso u obesidad. El estado de nutrición de los preescolares con madres con obesidad presentó un porcentaje mayor de desnutrición (76%. En la variable género, se encontró que 54% de los niños con madres con obesidad tenía baja talla. Al relacionar el nivel educativo de la madre, esta variable resultó ser estadísticamente significativa (p=0.015, donde el analfabetismo está más relacionado con la desnutrición infantil que tienen madres de bajo y/o peso normal. La elevada prevalencia de hogares conformados con preescolares con desnutrición y madres con obesidad, es un síntoma más de la pobreza en zonas indígenas en México, con bajo índice de desarrollo humano.

  7. Epilepsia de la infancia con paroxismos occipitales

    Directory of Open Access Journals (Sweden)

    Albia Pozo Alonso

    1999-09-01

    Full Text Available Se reporta que la epilepsia de la infancia con paroxismos occipitales constituye un síndrome epiléptico muy bien definido; se incluye en el grupo de las epilepsias idiopáticas relacionadas con localización. Los ataques comienzan habitualmente con síntomas visuales, en ocasiones seguidos por ataques hemiclónicos, parciales complejos y tónicoclónicos generalizados. El electroencefalograma interictal muestra complejos de puntaonda de localización occipital y temporal posterior que desaparecen con la apertura de los ojos. Se presenta una paciente de 8 años de edad que reúne los criterios clínicos y electroencefalográficos de la epilepsia de la infancia con paroxismos occipitales y se enfatiza en la evolución supuestamente benigna de dicho síndrome.Is is reported that epilepsy of childhood with occipital paroxysms is an epileptic syndrome that is well defined. It is included in the group of related idiopathic epilepsies with localization. Seizures habitually begin with visual symptoms, which are ocassionally followed by hemiclonic, complex partial, and generalized tonicclonic seizures. The interictal EEG shows spike-wave complexes of occipital and temporal posterior localization that are suppressed by eye opening. A patient aged 8 in whom the clinical and electroencephalographic criteria of epilepsy of childhood with occipital paroxysms are present is studied in this paper. Emphasis is made on the apparently benign evolution of this syndrome.

  8. Guia de tomada de decisão em cardiopatias congênitas em página Web na Internet: modelo Atresia Tricúspide Guidelines to decision - making in congenital heart disease on the WEB interface: Tricuspid Atresia model

    Directory of Open Access Journals (Sweden)

    Ruy Guilherme Rodrigues CAL

    1999-04-01

    Full Text Available Estimulada pelo desafio de gerenciar toda a informação envolvida na formação do conhecimento na área de saúde, a Informática Médica desenvolveu-se em larga escala em todos os países. O recente sucesso da rede Internet como veículo de distribuição de informação incentiva a elaboração de programas médicos para utilização através dessa rede. A elaboração de um programa de apoio à decisão - para cardiopatias congênitas em forma de documento em hipertexto de World-Wide Web - apresentado pela Internet possibilitaria o aproveitamento das características de processamento e armazenamento distribuído dessa rede. Este projeto teve como objetivos: criar um modelo de guia de tomada de decisão em cirurgia cardíaca pediátrica usando como base a Atresia Tricúspide; avaliar o grau de complexidade da criação deste modelo e os benefícios pelo emprego de interface em página WEB; testar sua validação com os casos de 16 pacientes tratados na Universidade Federal de São Paulo, no período de 1980 a março de 1997. Com a utilização de hardware adequado e da linguagem de programação HTML, o programa foi desenvolvido com a utilização de 7 telas de fluxograma, conjunto de textos e 37 ilustrações. Durante a elaboração deste modelo foi possível constatar a possibilidade de fácil desenvolvimento e rápida atualização. O resultado da validação mostrou concordância significativa (91,66% com as indicações cirúrgicas realizadas pelos especialistas, na Universidade Federal de São Paulo.Stimulated by the challenge of updating and organizing all the information acquired in the health field, computer medicine has developed on a wide global scale. The recent success of the Internet for distribution of information has created a need for the production and distribution of medical programs for use via this network. The creation of a decision-making program through the World Wide Web for congenital heart diseases could provide

  9. Aspectos Psicosociales Relacionados con el TEPT en Pacientes con Cáncer de Mama

    OpenAIRE

    Hernández Moreno, Fresia Paloma; Landero Hernández, René

    2015-01-01

    El objetivo del estudio fue analizar la relación del estrés, la depresión y las estrategias de afrontamiento con el Trastorno de Estrés Postraumático (TEPT) en una muestra de pacientes con cáncer de mama. Se realizó con una muestra no probabilística de 52 mujeres con cáncer de mama. Se encontró una relación entre TEPT y estrés (r= .344, p= .014), TEPT y depresión (rs=.346, p= .013). El TEPT correlacionó con evitación cognitiva (r= .437, p= .001), TEPT y desesperanza (rs= .437, p= .001) y TEPT...

  10. Desarrollo de aleaciones multifuncionales con base titanio

    OpenAIRE

    Pareja, Ramiro

    2007-01-01

    El Laboratorio de Materiales Nano-estructurados y Multifuncionales del Departamento de Física de la Universidad Carlos III de Madrid se dedica al desarrollo, procesado y caracterización de aleaciones con propiedades mejores que las preparadas por métodos convencionales. La mejora de las propiedades se consigue produciendo las aleaciones con un tamaño de grano nanométrico o ultrafino, o introduciendo una dispersión de nano-partículas de óxido en la matriz. El grupo está interesado en algún tip...

  11. TIROTOXICOSIS GESTACIONAL: PATOLOGIA CON RIESGO VITAL

    OpenAIRE

    Valdés R.,Enrique; Pilasi M.,Carlos; Núñez U.,Tatiana

    2003-01-01

    Se presenta un caso clínico con diagnóstico final de Tirotoxicosis gestacional que debuta con una complicación excepcional, insuficiencia cardíaca congestiva e hipertensión pulmonar severa. Se presenta la experiencia del Hospital Clínico de la Universidad de Chile, proponiendo que su diagnóstico y tratamiento oportunos son la base del pronóstico de esta patología de riesgo vital para el binomio madre-hijo

  12. Amplificadores con transistores. Estudio y dimensionado

    OpenAIRE

    Lubiano García, Adrián

    2017-01-01

    Este trabajo es un estudio de las distintas configuraciones de los amplificadores con transistores vistos en la asignatura de Electrónica Analógica del tercer curso del Grado en Ingeniería en Electrónica Industrial y Automática de la Escuela de Ingenierías Industriales de la Universidad de Valladolid. En este trabajo se mostrarán los pasos seguidos en la creación de una aplicación con Visual Basic para la realización de los ejercicios de las distintas configuraciones, así...

  13. Galaxias australes con núcleo doble

    Science.gov (United States)

    Gimeno, G.; Díaz, R.; Carranza, G.

    Se estudia una muestra de galaxias australes con núcleo doble a partir de una búsqueda extensiva en la literatura. Se analizan las características morfológicas, fotométricas y espectroscópicas de la muestra. Para algunas galaxias se han realizado observaciones con el espectrógrafo multifunción (EMF) de la Estación Astrofísica de Bosque Alegre a partir de las cuales se determinaron parámetros cinemáticos.

  14. Niveis sericos das vitaminas lipossoluveis (A, D e E) em pacientes com atresia biliar e hepatite auto-imune e a relação com o estado nutricional e indicadores clinicos e laboratoriais

    OpenAIRE

    Margareth Lopes Galvão Saron

    2008-01-01

    Resumo: As doenças hepáticas crônicas podem induzir à má-absorção de lipídios e vitaminas lipossolúveis e levar ao comprometimento do estado nutricional. O objetivo da pesquisa foi determinar os níveis séricos de vitaminas lipossolúveis (A, D e E) em crianças e adolescentes com atresia biliar (AB) e hepatite auto-imune (HAI) e verificar a relação com o estado nutricional e indicadores laboratoriais e clínicos. O estudo foi transversal e controlado e foram avaliados os pacientes com HAI (n=25...

  15. Prognostic impact of hepatobiliary scintigraphy in diagnosis and postoperative follow-up of newborns with biliary atresia; Prognostische Wertigkeit der hepatobiliaeren Funktionszintigraphie in Diagnostik und Nachsorge der Gallengangsatresie

    Energy Technology Data Exchange (ETDEWEB)

    Rossmueller, B.; Porn, U.; Dresel, S.; Hahn, K. [Muenchen Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin; Schuster, T. [Muenchen Univ. (Germany). Kinderchirurgische Klinik; Lang, T. [Muenchen Univ. (Germany). Kinderklinik

    2000-01-01

    Aim: To investigate the prognostic relevance of hepatobiliary scintigraphy (HBS) in newborns suffering from biliary atresia (BA) for establishing the primary diagnosis and in the postoperative follow-up after portoenterostomy (Kasai). Methods: Twenty newborns with direct hyperbilirubinemia and 6 children after operative treatment of BA (Kasai) underwent HBS with Tc-99m-DEIDA. In patients without intestinal drainage, hepatocellular extraction was estimated visually and calculated semiquantitatively by means of liver/heart-ratio 5 min p.i. Results: 10/20 patients with hyperbilirubinemia did not display biliary drainage; 6 had BA, 3 intrahepatic hypoplasia, and one showed a bile plug syndrom. 4/6 with BA but none of the 4 children with diagnoses other than BA presented with a good extraction. All of the 4 children with BA, who had either pre- or postoperatively a bad extraction, needed liver transplantation due to liver failure. Both of the two newborns with BA and favourable outcome after Kasai had a good extraction in the preoperative HBS and demonstrated good intestinal drainage in the postoperative scan. Conclusion: HBS rules out BA with high accuracy by demonstrating drainage of bile into the intestine. In newborns without drainage a good extraction favours the diagnosis of BA. In newborns with BA a bad extraction seems to indicate a poor postoperative prognosis after Kasai operation. HBS might therefore help to select those children who will not benefit from portoenterostomy. Postoperatively, HBS can easily and quickly confirm the successful hepatobiliary anastomosis by demonstrating biliary drainage into the intestine. (orig.) [German] Ziel der Studie war es, die diagnostische Wertigkeit und die prognostische Aussagekraft der hepatobiliaeren Funktionsszintigraphie (HB-FS) in der Primaerdiagnostik der Gallengangsatresie (GG-Atresie) und bei postoperativen Kontrollen nach Portoenterostomie (Kasai-OP) zu ueberpruefen. Methoden: 20 Neugeborene (Alter: 3-119 d) mit

  16. Relationship between hepatic CTGF expression and routine blood tests at the time of liver transplantation for biliary atresia: hope or hype for a biomarker of hepatic fibrosis

    Directory of Open Access Journals (Sweden)

    Haafiz A

    2011-04-01

    Full Text Available Allah Haafiz1, Christian Farrington1, Joel Andres1, Saleem Islam21Hepatology and Liver Transplantation, Division of Pediatric Gastroenterology, Hepatology and Nutrition, 2Division of Pediatric Surgery, University of Florida College of Medicine, Gainesville, FL, USABackground: Progressive hepatic fibrosis (HF is a prominent feature of biliary atresia (BA, the most common indication for liver transplantation (LT in children. Despite its importance in BA, HF is not evaluated in routine patient care because the invasiveness of liver biopsy makes histologic monitoring of fibrosis unfeasible. Therefore, the identification of noninvasive markers to assess HF is desirable especially in children.Purpose: The main goal of this pilot project was to establish an investigational framework correlating hepatic expression of fibrogenic markers with routine blood tests in BA.Methods: Using liver explants from patients with BA (n = 26, immune-expression of connective tissue growth factor (CTGF, a key fibrogenic cytokine was determined using horseradish-labeled antibodies. Expression intensities of lobular (L-CTGF and portal (P-CTGF CTGF were determined by using ImageJ software. These CTGF intensities were correlated with blood tests performed at the time of LT. Correlation coefficients were determined for each blood test variable versus mean L-CTGF and P-CTGF expression intensities. A P-value of less than 0.05 was considered statistically significant.Results: All patients had end-stage liver disease and persistent cholestasis at the time of LT. Kendall tau (t rank correlation coefficient for L-CTGF and white blood cell (WBC was inversed (—0.52; P ≤ 0.02. Similar but statistically nonsignificant inverse relationships were noted between L-CTGF and prothrombin time (PT (—0.15; P ≤ 0.4, international normalized ratio (INR (—0.14; P ≤ 0.5, and platelet count (—0.36; P ≤ 0.09. Inversed (t rank correlation coefficients were also evident between P

  17. Relationship between hepatic CTGF expression and routine blood tests at the time of liver transplantation for biliary atresia: hope or hype for a biomarker of hepatic fibrosis.

    Science.gov (United States)

    Haafiz, Allah; Farrington, Christian; Andres, Joel; Islam, Saleem

    2011-01-01

    Progressive hepatic fibrosis (HF) is a prominent feature of biliary atresia (BA), the most common indication for liver transplantation (LT) in children. Despite its importance in BA, HF is not evaluated in routine patient care because the invasiveness of liver biopsy makes histologic monitoring of fibrosis unfeasible. Therefore, the identification of noninvasive markers to assess HF is desirable especially in children. The main goal of this pilot project was to establish an investigational framework correlating hepatic expression of fibrogenic markers with routine blood tests in BA. Using liver explants from patients with BA (n = 26), immune-expression of connective tissue growth factor (CTGF), a key fibrogenic cytokine was determined using horseradish-labeled antibodies. Expression intensities of lobular (L-CTGF) and portal (P-CTGF) CTGF were determined by using ImageJ software. These CTGF intensities were correlated with blood tests performed at the time of LT. Correlation coefficients were determined for each blood test variable versus mean L-CTGF and P-CTGF expression intensities. A P-value of less than 0.05 was considered statistically significant. All patients had end-stage liver disease and persistent cholestasis at the time of LT. Kendall tau (τ) rank correlation coefficient for L-CTGF and white blood cell (WBC) was inversed (-0.52; P ≤ 0.02). Similar but statistically nonsignificant inverse relationships were noted between L-CTGF and prothrombin time (PT) (-0.15; P ≤ 0.4), international normalized ratio (INR) (-0.14; P ≤ 0.5), and platelet count (-0.36; P ≤ 0.09). Inversed (τ) rank correlation coefficients were also evident between P-CTGF expression and gamma-glutamyl transpeptidase (GGT), PT, INR, and platelet count. Pearson correlation coefficients for combinational analysis of standardized total bilirubin (TB), alkaline phosphatase, GGT, and platelet count with L-CTGF (0.33; P = 0.3) and P-CTGF (0.06; P = 0.8), were not significant. Similar

  18. IoT con Raspberry Pi

    OpenAIRE

    García Muelas, Carlos

    2015-01-01

    Estudio de diferentes plataformas software y hardware del internet de las cosas. Aplicación real con Raspberry Pi. Estudi de diferents plataformes programari i maquinari de l'internet de les coses. Aplicació real amb Raspberry Pi. Bachelor thesis for the Telecommunication Technologies program on Telematics.

  19. en personas con el VIH/SIDA

    Directory of Open Access Journals (Sweden)

    Ximena Palacios Espinosa

    2006-01-01

    Full Text Available El objetivo de este estudio fue hallar la relación de la intención e ideación suicida con algunas variables sociodemográficas, las características de la enfermedad, el tratamiento antirretroviral y los aspectos piscoafectivos en personas con el VIH/SIDA que residen en Bogotá (Colombia y pertenecientes a una fundación. Para ello, se tomó una muestra de 75 pacientes con VIH positivo a quienes se evaluó a través de una entrevista semiestructurada y dos instrumentos: el inventario de depresión de Beck (IBD y la escala de Ideación Suicida (SSI. Los resultados mostraron que solo 29 personas tenían ideación suicida y 10 intención suicida: De los 10 solo 2 sujetos tenían intención después del diagnóstico. Se encontró una relación estadística significativa para ideación suicida e intención suicida con edad, estrato socioeconómico, estado civil, orientación sexual, las características de la enfermedad, el tratamiento antirretroviral y los aspectos piscoafectivos.

  20. La relazione empatica con i robot

    NARCIS (Netherlands)

    Marti, P.; Iacono, I.; Tittarelli, M.

    2013-01-01

    È possibile instaurare una relazione empatica tra un essere umano e un robot? Il presente studio valuta le soluzioni di design adottate nella progettazione di una maschera empatica, implementata su un tablet, che consente all’anziano di dialogare nell’ambiente domestico con un robot che lo assiste

  1. Sarcoma de Kaposi en paciente con SIDA

    Directory of Open Access Journals (Sweden)

    Jesús Ramón León Polanco

    2015-01-01

    Full Text Available Se presenta el caso de un paciente masculino de 33 años de edad, con antecedentes de VIH-SIDA desde hace 10 años, que se mantiene en tratamiento con antirretrovirales. Durante todo este tiempo ha presentado varios episodios de infecciones respiratorias, incluyendo tuberculosis pulmonar 5 años atrás. Acude a consulta refiriendo edemas en miembros inferiores acompañado de lesiones en piel de color violáceo de un año de evolución, previamente interpretado como linfangitis rebelde al tratamiento y que se extendió a la cara interna de los muslos y a los miembros inferiores. Con pérdida de peso, no prurito en las lesiones, fiebre, lesiones en la mucosa oral. Se determinó hemoglobina 89 g/L, leucocitos 4,5 x 109 /L, se estudiaron las funciones hepática y renales resultando normales. Radiografías de tórax y ultrasonido abdominal normales. Se realizó estudio anatomopatológico de piel que informó Sarcoma de Kaposi. Se impuso tratamiento con quimioterapia

  2. Las construcciones con esqueleto en madera

    Directory of Open Access Journals (Sweden)

    Heinz Leser S.

    1984-06-01

    Estos sistemas se emplean preferentemente en viviendas prefabricadas y edificios de pequeña envergadura. Finalmente, consideramos las construcciones con "esqueleto en madera", cuyo campo de aplicación principal está en las edificaciones de mediana extensión: escuelas, centros comunitarios, pequeñas industrias o bloques habitacionales.

  3. Conversazioni autobiografiche con Albino Sacco-Casamassima

    Directory of Open Access Journals (Sweden)

    Orazio Maria Valastro

    2004-03-01

    Full Text Available Nel 1948 venne Olivetti a Matera. Olivetti venne a Matera con un certo ambasciatore americano che praticamente aveva preparato o stava dando una mano per il piano Marschall, venne a Matera e mi vollero incontrare. Io sono stato chiamato dalla piazza, stavo nella piazza, era il mese di luglio, una cosa di questo genere, e andai in un bar, un bar ad angolo mi ricordo in Via Don Minzioni, dove questo Olivetti mi disse 'ma lei che cosa fa coi sassi?' 'Che cos'è questa storia?' Gli raccontai un po' che cosa facevo io con questi amici, avevamo fatto questo, insomma tutto quello che avevamo fatto nei sassi e che volevamo preparare ancora per presentare a qualcuno un piano di revisionamento dei sassi. Mi ricordo, era la prima volta che avevo visto Adriano Olivetti, ne avevo sentito parlare però non avevo mai visto quest'uomo con gli occhi celesti, minuto, coi capelli bianchi, riccioluto, con una cravatta bianca, cosa che mi fece impressione, una specie di angelo.

  4. Procesamiento de argumentos en personas con afasia

    Directory of Open Access Journals (Sweden)

    Luis Martínez

    2012-06-01

    Full Text Available En las personas con afasia se alteran diversos procesos lingüísticos. Entre las alteraciones más comunes tenemos aquellas que se producen en el procesamiento sintáctico, lo cual es evidente en el procesamiento de los argumentos. Investigaciones previas realizadas a sujetos con afasia reflejan un procesamientodeficiente de argumentos, lo cual se hace más evidente a medida que el número de argumentos aumenta. Se ha encontrado que mientras mayor es la complejidad en la estructura de la oración, los sujetos afásicos manifiestan mayores dificultades en el procesamiento de la misma. El presente estudio indaga en las dificultades que presentan los sujetos adultos con trastornos afásicos hablantes de español en el procesamiento de argumentos durante tareas de descripción. Este trabajo corrobora la evidencia de que los sujetos con afasia manifiestan un manejo deficiente en el procesamiento de argumentos en las tareas señaladas.

  5. Coriocarcinoma con metástasis pulmonar

    Directory of Open Access Journals (Sweden)

    Vicia Sánchez Abalos

    2014-05-01

    Full Text Available Se presenta el caso clínico de una fémina de 44 años de edad, con 32 semanas de embarazo, la cual fuera ingresada en la Unidad de Cuidados Intensivos del Hospital General Docente "Dr. Juan Bruno Zayas Alfonso" de Santiago de Cuba, por presentar insuficiencia respiratoria aguda como consecuencia de una sepsis. La paciente fue tratada con cefalosporina de tercera generación y ventilación mecánica no invasiva, pero se mantuvieron las características gasométricas de hipoxemia y una mala reacción terapéutica, por lo que se requirió instrumentación de las vías respiratorias y soporte hemodinámico, sin lograr regresión del cuadro clínico, lo cual condujo a un paro cardiorrespiratorio y, con ello, a la muerte. La necropsia mostró un coriocarcinoma del endometrio con metástasis pulmonar

  6. Con la testa fra le nuvole

    Directory of Open Access Journals (Sweden)

    André Greboge

    2015-06-01

    Full Text Available Partitura de Con la testa fra le nuvole, composta em 2015 para piano e harpa de pedais. Este memorial apresenta as notas sobre a obra e a organização do processo composicional, baseada em um insight composicional a partir da ideia de complexidade apresentada por James Tenney em Meta Meta-hodos (1988.

  7. REDUCCIÓN DE ÓXIDOSDE NITROGENO CON CATALIZADORES ZEOLÍTICOS INTERCAMBIADOS CON COBALTO

    Directory of Open Access Journals (Sweden)

    Enrique Alexis García Moya

    2012-10-01

    Full Text Available Se presenta un estudio sobre la reducción catalítica selectiva (RCS de NOx con metano en presencia de oxígeno en exceso con varios catalizadores a base de las zeolitas mordenita, ferrierita y ZSM-5 intercambiadas con diferentes cargas de cobalto y con las correspondientes zeolitas en forma acida. Cuando la mezcla reactiva contenía mayormente NO2 en lugar de NO, los catalizadores ácidos mostraron las más altas velocidades de formación de N2 en condiciones secas. Las mayores actividades se obtuvieron con los catalizadores Co-mordenita, siguiendo en orden de actividad los catalizadores Co-Ferrierita y Co-ZSM-5. El catalizador Co-Mordenita más activo se ensayó con una mezcla reactiva donde predominó el NO en lugar del NO2 bajo condiciones secas e hidrotérmicas y en presencia de SO2. Con la adición de 8% de agua a la mezcla de reacción se observó desactivación reversible, especialmente a bajas temperaturas. La adición de 60 ppm de SO2 disminuyó la velocidad de reacción aproximadamente a la mitad debido posiblemente al envenenamiento de algunos sitios activos.

  8. Esophageal Atresia and Tracheoesophageal Fistula

    Science.gov (United States)

    ... Kids and Teens Pregnancy and Childbirth Women Men Seniors Your Health Resources Healthcare Management End-of-Life Issues Insurance & Bills Self Care Working With Your Doctor Drugs, Procedures & Devices Over-the- ...

  9. Manejo de conductos radiculares con curvatura marcada

    Directory of Open Access Journals (Sweden)

    Jorge Mario Nuñez Duran

    2013-10-01

    Full Text Available Las variaciones anatómicas en dientes con conductos que poseen dilaceraciones hacen del tratamiento de endodonciaun reto o una complicación relativa, en donde el clínico debe desarrollar toda su habilidad y el uso de instrumentosespeciales que faciliten u optimicen la terapia de conductos radiculares. La toma de radiografías previas con diferentesangulaciones y el estudio minucioso de ellas, darán una ayuda fundamental para conocer más detalladamente estacomplicación anatómica, que en algunos casos es muy frecuente. El uso de instrumental más flexible y resistentepermite llegar con mayor precisión y seguridad a la parte apical sin que se corra el riesgo de fracturar los instrumentos,limpiándolos y modelándolos en toda su extensión para poder obturarlos de una manera correcta.El uso de limas de menor calibre, precurvadas, permite acceder a la porción dilacerada de la raíz. Este es un métodoeficaz para prevenir la perdida de longitud de trabajo y evitar así las perforaciones de las raíces durante la preparación.Dentro de los errores más comunes durante la instrumentación de los conductos está la perdida de longitud detrabajo, junto con la transportación del foramen apical, ya sea de manera externa o interna. En el presente artículose menciona el uso de nuevas y mejores técnicas usadas para poder evitar este tipo de errores y se muestra un casoclínico del manejo de un molar superior con una curvatura de 40 grados que fue preparado y obturado exitosamentecon el sistema Protaper (Dentsply- maillefer.

  10. Variables socionutricionales de hogares mazahuas integrados por preescolares desnutridos con madres con obesidad y sin obesidad

    OpenAIRE

    Viridiana Vanessa Conzuelo-González

    2009-01-01

    El primer objetivo fue conocer cuántos menores de cinco años con diferentes grados de desnutrición tienen una madre con sobrepeso/obesidad/ en una comunidad indígena que vive en extrema pobreza y bajo condiciones de migración masculina internacional. El segundo fue comparar tres variables socionutricionales (ingreso familiar, educación de la madre y adecuación nutrimental de la dieta diaria) entre estos hogares y los hogares con desnutrición infantil y madres sin obesidad. Se realizó un estud...

  11. Terapias alternativas con animales para niños con necesidades especiales

    OpenAIRE

    Cea-Chueca, Aihnoa

    2014-01-01

    El presente trabajo de fin de grado consiste en la realización de un análisis y descripción de la literatura acerca de las distintas terapias animales, destinadas a niños con necesidades especiales: cómo y por qué surgieron, en qué consisten las terapias, cómo podemos utilizarlas, etc. Las terapias alternativas con animales se conocen como una alternativa terapéutica, donde se utiliza a un animal como co-terapeuta para poder desarrollar diferentes capacidades de los niños con necesidades espe...

  12. Variables socionutricionales de hogares mazahuas integrados por preescolares desnutridos con madres con obesidad y sin obesidad

    OpenAIRE

    Viridiana Vanessa Conzuelo-González; Ivonne Vizcarra-Bordi

    2009-01-01

    El primer objetivo fue conocer cuántos menores de cinco años con diferentes grados de desnutrición tienen una madre con sobrepeso/obesidad/ en una comunidad indígena que vive en extrema pobreza y bajo condiciones de migración masculina internacional. El segundo fue comparar tres variables socionutricionales (ingreso familiar, educación de la madre y adecuación nutrimental de la dieta diaria) entre estos hogares y los hogares con desnutrición infantil y madres sin obesidad. Se realizó un est...

  13. LA FORMACION DE LOS DOCENTES EN LA INTERVENCION CON ALUMNOS/AS CON DEFICIT DE ATENCION CON HIPERACTIVIDAD (TDAH

    Directory of Open Access Journals (Sweden)

    Francisco D. Fernández Martín

    2004-06-01

    Full Text Available La presencia del Trastorno por d.ficit de atención con hiperactividad (TDAH en algunos/as de nuestros/as alumnos/as ocasiona serios problemas para que estos/as se adapten al contexto escolar y sociofamiliar en el que se desenvuelven, debido a la presencia de una serie de dificultades: hiperactividad, dificultades para mantener la atenci.n, autorregular la conducta, controlarse, etc.

  14. Esporotricosis cutánea diseminada con compromiso articular en una mujer con diabetes tipo 2

    Directory of Open Access Journals (Sweden)

    Sendy Solorzano

    Full Text Available La esporotricosis diseminada es una presentación infrecuente de esta micosis. Aunque ha sido descrita en sujetos inmunocompetentes, con frecuencia existe compromiso de la inmunidad mediada por células T. Reportamos el caso de una mujer con diabetes tipo 2 no controlada que desarrolló esporotricosis cutánea diseminada. El curso de la enfermedad presentó varias complicaciones que incluyeron hepatitis secundaria a itraconazol y artritis de la rodilla con cultivo positivo para Sporothrix schenckii durante el tratamiento con solución saturada de ioduro de potasio. La discusión del caso incluye aspectos de la patogénesis de la esporotricosis diseminada y el manejo de la infección y sus complicaciones.

  15. Riesgo familiar total en familias con mujeres diagnosticadas con neoplasia de mama

    Directory of Open Access Journals (Sweden)

    Nancy Cecilia Charrys-Bravo

    2017-07-01

    Full Text Available Objetivo: Determinar el riesgo familiar total de las familias con mujeres diagnosticadas con cáncer de mama, que asisten a un centro de salud oncológico en la ciudad de Barranquilla. Materiales y Métodos: El estudio fue de tipo descriptivo, transversal con abordaje cuantitativo. Se utilizó el instrumento Riesgo Familiar Total RFT 5-33. El universo estuvo conformado por familias con mujeres diagnosticadas con cáncer en mama. La muestra fue de 41 familias que integraron a 154 miembros; se abordó el estudio de manera censal, y no por medio de una muestra, porque el acceso a la información de la totalidad de las familia fue viable. Resultados: Los resultados mostraron que las familias, en su mayoría, son de tipo 2. El 68 % de las pacientes categorizan sus familias como amenazadas, el 5 % como familias de alto riesgo y un 27 % de las familias con un bajo riesgo. Conclusiones: Los hallazgos encontrados en esta investigación son importantes para las familias, lo cual permitirá establecer acciones y actividades que logren orientar e implementar procesos de atención específicos con el propósito de cuidar a las familias para que se mantengan sanos en un nivel de bajo riesgo; además, desarrollar controles y seguimiento a aquellas familias que se encuentran en un riesgo alto de amenazas, mediante acciones de promoción y prevención de la enfermedad de una manera amplia. Por lo anterior, se deben emprender programas más agresivos de prevención y promoción, especialmente con las familias que asisten en busca de apoyo médico para este padecimiento; de esta forma, se podrán diagnosticar los casos de forma temprana y proceder al respectivo tratamiento.

  16. Materiales nanocumpuestos basados en LDPE relleno con nanotubos de carbono con potenciales propiedades bactericidas

    OpenAIRE

    Benigno Escribano, Erika

    2015-01-01

    En este trabajo, se ha seleccionado como material de estudio polietileno de baja densidad, LDPE, ya que es un polímero con múltiples aplicaciones en diversos campos. Se busca como objetivo principal, preparar y caracterizar nuevos materiales basados en LDPE con potenciales propiedades antibacterianas. Para ello, se van a estudiar dos posibles maneras de conseguirlo: La primera de ellas, consiste en realizar un procesado mecánico sobre el polietileno, concretamente, una molienda del alta en...

  17. Rabdomiolisis asociada al tratamiento con valaciclovir

    Directory of Open Access Journals (Sweden)

    Héctor Enríquez Gómez

    2012-03-01

    Full Text Available La rabdomiolisis es un efecto secundario asociado a un buen número de fármacos, entre ellos los antivirales. Describimos un caso de rabdomiolisis secundaria a valaciclovir, efecto no descrito anteriormente en laliteratura, en un contexto clínico muy especial. Se trata de un varón queingresa por un golpe de calor secundario a la realización de ejercicio físicoextremo. Durante su ingreso se comienza tratamiento con valaciclovir porun herpes nasolabial, observándose en los días siguientes una marcadaelevación de las cifras de CPK que desaparece con la suspensión del mismo. Proponemos, por tanto, que ante la administración de valaciclovir ensituaciones que puedan comportar daño mitocondrial habría que realizarmonitorización estrecha de los niveles de CPK

  18. Experiencias y aprendizajes con juegos cooperativos

    Directory of Open Access Journals (Sweden)

    Evelyn Cerdas Agüero

    2013-03-01

    Full Text Available El presente trabajo tiene como objetivo presentar experiencias y aprendizajes que  han logrado algunos grupos de docentes con los que se ha trabajado por medio de talleres de juegos cooperativos para la paz. Los talleres se han facilitado desde la actividad de extensión Aula Activa: juegos cooperativos para la Educación para la Paz que se ha implementado en el  Instituto de Estudios Latinoamericanos (IDELA desde el 2006. En este trabajo  nos enfocamos en el trabajo con docentes del Ministerio de Educación Pública (MEP de Costa Rica, proceso que deja una serie de aprendizajes actitudinales, de valores, habilidades y cognocitivos.

  19. Comunicándonos con las cosas

    Directory of Open Access Journals (Sweden)

    Miguel Delgado

    2009-09-01

    Full Text Available Tras la Web 2.0 y gracias a la colaboración entre las personas, Internet sigue creciendo y evolucionando, pero esta vez a través de los objetos. La siguiente evolución pretende que las personas puedan interactuar con las cosas de una forma inteligente mediante tecnologías ampliamente utilizadas como el teléfono móvil e Internet, y tecnologías que permitan etiquetar objetos.

  20. Bacteriemia en pacientes internados con celulitis

    Directory of Open Access Journals (Sweden)

    Juan S. Lasa

    2012-08-01

    Full Text Available La celulitis es una inflamación aguda de la dermis y tejido celular subcutáneo de causa bacteriana, que generalmente complica a heridas, úlceras y dermatosis, aunque de manera frecuente no existe sitio de entrada. Se recomienda la realización de cultivo de punción de piel y partes blandas (PPB. Los hemocultivos raramente dan resultados positivos. El objetivo de este trabajo fue determinar la prevalencia de bacteriemia en pacientes internados en nuestra institución con diagnóstico de celulitis. Se analizaron retrospectivamente los registros clínicos de los pacientes con este diagnóstico al ingreso entre junio de 2007 y marzo de 2010. Se evaluaron los datos poblacionales, presencia de comorbilidades, y resultados de los cultivos. En ese período, se internaron 140 pacientes con diagnóstico de celulitis y a todos ellos se les realizó hemocultivo y cultivos de PPB. Setenta y cuatro eran varones (52.8%. La edad promedio: 47.5 ± 19.7 años (rango 16-94. El 40% tuvo cultivos positivos de PPB, en los que el Staphylococcus aureus meticilino resistente (SAMR fue el germen más frecuentemente aislado (35.7%; la prevalencia de bacteriemia fue del 8.6%, en donde el germen más frecuente fue Streptoccocus Beta hemolítico, grupo G (33% del total de hemocultivos positivos. La bacteriemia se asoció significativamente a mayor estadía hospitalaria (10.5 ± 8.9 vs. 4.9 ± 6, p = 0.004. Se asoció con mayor riesgo de hemocultivo positivo a ser diabético, tener cultivo de PPB positivo, consumo de alcohol y/o enfermedad pulmonar obstructiva crónica.

  1. ORIENTACIONES CURRICULARES CON EL ENFOQUE CTS

    Directory of Open Access Journals (Sweden)

    Miguel Corchuelo

    2010-11-01

    Full Text Available Considerando que el propósito de la educación con un enfoque CTS propende por abordar problemas contextualizados socialmente, se busca construir una propuesta desde la práctica, con el fi n de fortalecer la formación para la participación ciudadana. Encontramos que abordar el tratamiento de las relaciones entre deporte, nutrición humana y motricidad convierte al cuerpo humano en un objeto de estudio vigente. En la ciudad de Popayán (Cauca, Colombia se vive una de las más altas tasas de bulimia y anorexia en adolescentes del país, al igual que de consumo de drogas ilícitas. Además, el bajo rendimiento de los deportistas caucanos en los últimos Juegos Nacionales, los casos de dopaje en atletas olímpicos, entre otros, abre interrogantes sobre la formación integral de nuestros estudiantes. Con el fi n de acercar la escuela a estas problemáticas locales y nacionales, se asume un trabajo interdisciplinario basado en la resolución de problemas socialmente relevantes. En esta oportunidad compartiremos los resultados del camino recorrido en torno a la pregunta: “El deporte: ¿emancipación o alienación?”.

  2. Aprendiendo química con golosinas

    Directory of Open Access Journals (Sweden)

    José Manuel Rodríguez Rodríguez

    2011-01-01

    Full Text Available Las golosinas (caramelos, chicles, nubes, palotes, papas fritas, etc. nos gustan a casi todos y gozan de una imagen positiva, a pesar de sus efectos secundarios si no se consumen con moderación. Aprovechándonos de esa buena imagen y de la gran variedad de golosinas que existen en el mercado, hemos diseñado una serie de experimentos con los que podemos explicar distintos tipos de reacciones químicas (ácido-base, oxidación reducción, endotérmicas¿, la actuación de un indicador e, incluso, podemos comparar cantidades de vitamina C. En todas las actividades se persigue explicar algún aspecto de la Química que, en general, el público desconoce, captando su atención, por ejemplo, mediante cambios de color espectaculares y con las golosinas como protagonistas. Así mismo, estas experiencias pueden emplearse como recursos didácticos para alumnos de 4º E.S.O. y Bachillerato, apoyando los contenidos teóricos del currículum.

  3. Calidad de vida, satisfacción con el tratamiento y bienestar emocional en pacientes con diabetes LADA

    OpenAIRE

    Granado Casas, Minerva

    2014-01-01

    Introducción: La calidad de vida, satisfacción con el tratamiento y bienestar emocional en pacientes con diabetes mellitus están relacionados con el control metabólico, las complicaciones y algunas variables socioeconómicas. Actualmente, no existe ningún estudio científico que analice la calidad de vida, satisfacción con el tratamiento y bienestar emocional en pacientes con diabetes LADA. Objetivos: Los objetivos principales fueron conocer la calidad de vida, satisfacción con el tratamient...

  4. "Sonríe con salud"

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    Rosa María González Ramos

    Full Text Available Introducción: los programas educativos juegan un papel importante ya que permiten modificar hábitos y conductas perjudiciales para la salud. Las personas de la tercera edad deben adquirir los conocimientos necesarios para realizar el auto cuidado de la salud bucal mejorando así su calidad de vida. Objetivo: evaluar los resultados del programa "Sonríe con salud". Métodos: se realizó una investigación descriptiva de corte transversal, con un universo de 74 personas de 60 años y más. Las variables estudiadas fueron: la edad, sexo. Se aplicó una encuesta. Las respuestas se agruparon atendiendo a si poseían o no, conocimientos acerca de la relación entre salud bucal y salud general, las principales enfermedades buco-dentales, realización del autoexamen bucal, efecto del tabaquismo y alcoholismo, ingestión de los alimentos azucarados, calientes, picantes y la importancia de la higiene bucal. La recolección de la información, la realizaron los autores empleando como fuente primaria el cuestionario aplicado en la primera etapa de la investigación. Se utilizaron los valores absolutos y el porcentaje en el análisis de los resultados. Resultados: el grupo de edad de 70 a 79 años fueron los más representados (36,5 % y el sexo femenino con un 59,5 %. Se elevaron los conocimientos relacionados con el efecto perjudicial del alcoholismo al 91,9 % y el tabaquismo al 95,9 %; acerca de la ingestión de alimentos azucarados se elevan al 97,7 % al igual que los alimentos calientes y picantes en un 90,5 %, relativo al autoexamen bucal se superan los conocimientos de su realización al 78,4 % y referente a la higiene bucal se elevan al 91,9 %. Conclusiones: el programa educativo "sonríe con salud" resultó eficaz, al elevar el nivel de conocimientos de los ancianos que recibieron las actividades previstas en el programa.

  5. Acogida de los solicitantes de asilo con discapacidad en Europa

    OpenAIRE

    Beduschi-Ortiz, Ana

    2010-01-01

    Con respecto a la acogida de los solicitantes de asilo en la Unión Europea, podemos encontrar disposiciones para la protección de las personas con discapacidad en una amplia gama de fuentes reguladoras.

  6. Novas técnicas cirúrgicas para o tratamento da atresia pulmonar com comunicação interventricular e anomalias de artérias pulmonares incluindo o assim chamado truncus tipo IV New surgical techniques for treatment of pulmonary atresia with ventricular septal defect and pulmonary arteries anomalies including the so-called tipo IV truncus

    Directory of Open Access Journals (Sweden)

    Miguel Barbero-Marcial

    1987-04-01

    Full Text Available Entre janeiro de 1975 e outubro de 1986, 42 pacientes com atresia pulmonar e comunicação interventricular, com idade entre 2 e 18 anos, foram submetidos a correção parcial, ou total. Foram divididos em: tipo A com todos os segmentos broncopulmonares conectados às artérias pulmonares (AP's, 34 pacientes; tipo B com alguns dos segmentos broncopulmonares conectados às AP's, 6 pacientes; tipo C com todos os segmentos broncopulmonares conectados às colaterais sistêmico-pulmonares, 2 pacientes. A correção foi planejada em uma a três etapas. No tipo A, 17 foram corrigidos em uma etapa, com três óbitos; em 9, na primeira etapa, as AP's foram reconstruídas e o Blalock (BT, realizado, tendo ocorrido um óbito. Em 2, a segunda etapa de correção total foi realizada, sem óbitos. No tipo B, a primeira etapa de unificação das colaterais intra ou extra-hilares foi realizada em 6 casos, sem óbitos; em 2, a segunda etapa da correção total foi realizada, com um óbito. No tipo C, 2 pacientes foram operados; 1 em três etapas; a primeira constou de construção de segmento arterial intermediário entre as artérias lobares e o BT; a segunda compreendeu unificação das colaterais contralaterais e a terceira, restabelecimento da continuidade ventrículo direito - circulação pulmonar; o paciente teve boa evolução. No segundo caso, a correção foi realizada após somente uma intervenção prévia. A evolução foi satisfatória. Estudos hemodinâmicos seriados foram realizados em 32 pacientes. As técnicas propostas permitem obter condições para correção total com adequada relação pós-operatória das pressões ventrículo direito - ventrículo esquerdo.Fourty-two patients with pulmonary atresia and interventricular septal defect were submited to a partial or total correction, between January 1975 and October 1986, with a range of 2 months to 18 years of age. Three groups were identified: Group A: 34 patients with all bronco

  7. The pulmonary vascular blood supply in the pulmonary atresia with ventricular septal defect and its implications in surgical treatment O suprimento sangüíneo vascular pulmonar na atresia pulmonar com comunicação interventricular e suas implicações no tratamento cirúrgico

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre Croti

    2003-10-01

    Full Text Available OBJECTIVE: With base in the studies cineangiocardiography of pacients with pulmonary atresia (PA with ventricular septal defect (VSD, to identify in the groups proposed by BARBERO MARCIAL, subgroups with similar morphological characteristics, to measure their central pulmonary arteries (CPA and major aortopulmonary collateral arteries (MAPCA, thereby establishing their implications in surgical treatment. METHOD: Sixty three patients were classified in groups A (15, B (40 and C (8 between january 1990 and june 2001. Patients with complete cineangiocardiograms prior to the first surgical intervention were included in this study, being calculated the pulmonary arterial index (PAI, the major aortopulmonary collateral arterial index (MAPCAI and the total neopulmonary arterial index (TNPAI = PAI + MAPCAI. Surgical treatment was considered palliative (PT, definitively palliative (DPT and definitive (DT. RESULTS: Nine subgroups were identified, A (A1 and A2, B (B1, B2, B3, B4 and B5 and C (C1 and C2. In group A, the PAI of patients for DT was higher than for PT patients (p=0,0092. In group B, the TNPAI of DT patients was greater than for PT patients (p=0,0959. In group C, the MAPCAI in DPT patients was lower than in PT and DT patients. In the group A was not mortality, in the group B was of 17,5% and in the group C was of 12,5%. CONCLUSIONS:Among the groups A, B e C was possible to identifiy nine subgroups, the morphologic and morphometric characteristics allowed to suggest the surgical treatment in the patients of the group A had larger chance of TD, the group B of TP and the group C of TPD. The mortality presented larger correlation with the morphologic characteristics that with the morphometric.OBJETIVO: Com base nos estudos cineangiocardiográficos de pacientes portadores de atresia pulmonar (AP com comunicação interventricular (CIV, identificar nos grupos propostos pela classificação de BARBERO MARCIAL, subgrupos com suprimento sang

  8. Factores asociados con la irregularidad de la ingesta de Dapsona en pacientes con lepra: Dapsona en pacientes con lepra

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    Luis Carlos Orozco Vargas

    2013-04-01

    Full Text Available Introducción: Conocer los factores asociados al cumplimiento del tratamiento en pacientes con lepra, es muy importante para prevenir la resistencia del Mycobacterium leprae y garantizar la cura bacteriológica de estas personas. La prueba de orina para Dapsona, presente en el régimen autoadministrado, es un método sencillo para establecer la regularidad del tratamiento. Objetivo: Explorar los factores asociados a la irregularidad de la ingesta del tratamiento antileproso. Métodos: Estudio de corte transversal de los enfermos que recibieron tratamiento antileproso en un centro dermatológico. La irregularidad se estableció con la prueba de dapsonuria. Se consideró irregular el que presentó la prueba negativa. Las variables sospechosas de influir en la irregularidad se analizaron con regresión logística exacta. Resultados: En el modelo final del análisis multivariado se encontraron cinco variables asociadas, entre éstas sobresalen como factores de riesgo, la ausencia de discapacidad, OR 28.56 (IC90% 1.2-2.1 y la entrega de tratamiento para tiempos mayores a un mes, por cada mes OR 3.41 (IC90% 1.4-9.2 y como factor protector, la aceptación familiar de la enfermedad OR 0.008 (IC90% 0.001-0.24. Conclusión: Aunque es posible que el pequeño tamaño de muestra no haya permitido detectar algunos factores de riesgo informados en otras investigaciones, la mayoría de esos estudios no han realizado análisis multivariado por lo cual es posible que muchos de los factores informados en la literatura no tengan importancia. Salud UIS 2013; 45 (1: 7-14

  9. 9 CFR 319.301 - Chili con carne with beans.

    Science.gov (United States)

    2010-01-01

    ... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Chili con carne with beans. 319.301 Section 319.301 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE, DEPARTMENT OF AGRICULTURE... Dehydrated Meat Food Products § 319.301 Chili con carne with beans. Chili con carne with beans shall contain...

  10. Insuficiencia renal aguda relacionada con medicamentos en pacientes hospitalizados

    Directory of Open Access Journals (Sweden)

    Lujan Iavecchia

    2015-11-01

    Conclusiones: La mitad de los episodios de IRA intrahospitalaria se relacionaron con medicamentos. Los pacientes con IRA relacionada con medicamentos presentaron más antecedentes patológicos cardiovasculares, pero menos factores de riesgo de IRA y una menor mortalidad.

  11. Tumor germinal mixto con componentes de disgerminoma y coriocarcinoma de ovario en mujer adolescente con ataxiatelangiectasia

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    Eduardo Augusto Gálvez-Cuitiva

    2015-11-01

    días. Se suspendió la quimioterapia porque desarrolló choque séptico que puso en riesgo su vida. Por su enfermedad de base permaneció bajo vigilancia. Actualmente, 17 meses después, la paciente vive sin actividad tumoral.  Conclusión: existe asociación entre ataxia-telangiectasia, leucemia y linfomas. Se describe el caso clínico de una paciente con ataxia-telangiectasia y tumor germinal mixto con componente de disgerminoma y coriocarcinoma de ovario. Se propone establecer un tratamiento especial para estos pacientes inmunocomprometidos, con alto riesgo de cáncer pero, a la vez, de no tolerar los esquemas habituales.

  12. Tratamiento de la obesidad con productos naturales

    Directory of Open Access Journals (Sweden)

    Lina Neri Espinosa Pire

    2016-12-01

    Full Text Available Los crecientes índices de obesidad alrededor del mundo, provocados principalmente por los excesos en la ingesta y la vida sedentaria, constituye uno de los grandes problemas de salud pública en nuestras sociedades. Entre otras formas como dietas y tratamientos que surgen cada día para combatir la obesidad, el uso de plantas medicinales conocido como fitoterapia, ha despertado gran interés. Existen ciertas plantas que poseen propiedades adelgazantes, sin embargo, lo mejor es ponerse en manos de especialistas en este campo, para recibir una correcta asesoría de acuerdo a las necesidades personales. Las plantas medicinales y los productos que de ellas se obtienen, constituyen remedios eficaces para combatir la obesidad, pues no tienen efectos adversos. No obstante el tratamiento debe combinarse con un cambio de hábitos alimentarios y actividad física y no conviene utilizarlos irresponsablemente, pues no todos tienen los mismos beneficios y efectos. La eficacia de la aplicación de plantas con la finalidad de bajar de peso ha sido comprobada en numerosos ensayos clínicos que se han realizado con determinadas especies vegetales. Las plantas medicinales se han utilizado tradicionalmente en forma de infusión, sin embargo esta forma de administración no permite controlar efectivamente las dosis administradas, por lo que es preferible hacerlo en preparados como cápsulas o comprimidos. Cada planta deberá administrarse de una manera específica y es importante que los preparados se tomen siempre en el mismo horario para potenciar su efectividad.

  13. Factores prenatales relacionados con la prematuridad

    Directory of Open Access Journals (Sweden)

    Guillermo Arias Macías

    2001-03-01

    Full Text Available Se estudiaron 130 madres y sus recién nacidos prematuros con su correspondiente grupo control, nacidos en el Hospital Ginecoobstétrico Provincial Docente "Justo Legón Padilla", de Pinar del Río, desde febrero hasta julio de 1998, con el objetivo de analizar algunos factores perinatales relacionados con la prematuridad. Se analizaron las variables: edad materna, paridad, peso al inicio del embarazo, talla materna, período intergenésico, edad gestacional, oligoamnios, hipertensión arterial materna y sexo del niño. Se demostró que la edad materna inferior a 17 años, la baja talla materna, el oligoamnio, la sepsis ovular y el período intergenésico de más de 5 años tuvieron resultados significativos para el nacimiento de pretérminos.130 mothers and their premature infants with their corresponding control group delivered at "Justo Legón Padilla" Provincial Gynecoobstetric Teaching Hospital, in Pinar del Río, from February to July, 1998, were studied aimed at analyzing some perinatal factors connected with prematurity. The following variables were analyzed: maternal age, parity, weight at the beginning of pregnancy, maternal height, intergenesic period, gestational age, oligohydramnios, maternal hypertension and sex of the child. It was proved that maternal age under 17, low maternal height, the oligohydramnios, vulvar sepsis and the intergenesic period of more than 5 years had significant results for the birth of preterm infants.

  14. Rescate articular con colgajo anterolateral del muslo

    Directory of Open Access Journals (Sweden)

    F.J. García-Bernal

    2015-06-01

    Full Text Available La preservación del nivel de amputación tanto de la extremidad superior como de la inferior, puede mejorar la función residual y el proceso de rehabilitación protésica de la misma. Ambas van a estar condicionadas por la longitud restante de la extremidad, la presencia o no de articulaciones operativas y la calidad de la cobertura del muñón. Presentamos 2 casos de amputaciones. El primero es un caso agudo de amputación traumática de extremidad superior a la altura del tercio proximal de antebrazo, que asocia avulsión cutánea circular desde el tercio medio del brazo y con articulación de codo conservada. El segundo, es la secuela de una amputación infracondílea de extremidad inferior por osteosarcoma que presenta fístulas cutáneas con drenaje supurativo por osteomielitis en el muñón tibial. En ambos pacientes realizamos cobertura con colgajo anterolateral de muslo anastomosado a la arteria radial en el caso de la extremidad superior, y a la arteria genicular descendente en la extremidad inferior. En los dos casos el postoperatorio transcurrió sin complicaciones, logrando preservar las articulaciones del codo y de la rodilla respectivamente, así como la posterior rehabilitación protésica. Consideramos que el colgajo anterolateral del muslo permite aportar tejido de buena calidad como cobertura del muñón de amputación. Dadas las características del tejido aportado y su volumen, es idóneo tanto para cobertura de defectos agudos como de déficits de almohadillado en casos crónicos.

  15. Proyecto innovador: “con+ciencia”

    OpenAIRE

    Fernández Arroyo, Jorge; Rodríguez Marín, Fátima; González Medina, Sara

    2013-01-01

    En esta comunicación se expone la experiencia de observación sobre el funcionamiento del proyecto Con+ciencia en los cursos de 5º y 6º de Primaria (tercer ciclo) del centro C.E.I.P. “Príncipe de Asturias” (Sevilla) durante el mes de marzo del 2013. Nuestra participación ha buscado conocer en profundidad este proyecto, observar su aplicación real en el aula y sus implicaciones en el alumnado y el profesorado.

  16. Riego subsuperficial con aguas residuales tratadas

    OpenAIRE

    Crespi, Raúl; Camacho, Emilio; Polo, María José

    2009-01-01

    La reutilización de aguas residuales tratadas para riego por goteo subsuperficial (RGS) de cultivos extensivos, es una práctica reconocida mundialmente, siendo la obturación de emisores un serio problema en la operación de estos sistemas. Durante el ciclo agrícola 2002/03, se realizó un ensayo experimental usando dos tipos de agua: limpia (AL) y residual tratada (AR) en un sistema mixto combinando un reactor biológico con laguna de maduración y tres tipos de protectores del emisor: micro dosi...

  17. Biorremediacion de suelos contaminados con hidrocarburos

    OpenAIRE

    Ortiz, E.; Núñez, R.; Fonseca, E.; Oramas, J.; Almazán, V.; Cabranes, Y.; Miranda, A.; Barbán, O.; Martínez, C.; Díaz, Y.; Borges, G.

    2005-01-01

    En la actualidad los suelos contaminados con hidrocarburos representan el 70% del total de los eco-sistemas impactados. A partir del aislamiento, selección y conservación de bacterias marinas degra-dadoras de hidrocarburos y productoras de sustancias tensioactivas se formuló un producto denomi-nado BIOIL-FC. Este producto ha sido aplicado satisfactoriamente en derrames de hidrocarburos en el mar. En el presente trabajo fue evaluada la factibilidad del uso de los procesos de biorremediación “e...

  18. Los juegos de Borges con el tiempo

    Directory of Open Access Journals (Sweden)

    Paulína Šišmišová

    2012-12-01

    Full Text Available La preocupación por el tiempo es una de las constantes de la obra del escritor argentino Jorge Luis Borges. Los «juegos con el tiempo» forman parte indispensable de sus estrategias narrativas, tal como lo documenta también su célebre cuento «Borges y yo». En nuestra contribución nos dedicaremos a estudiar la problemática del tiempo en Borges bajo dos aspectos: el tiempo como recurso de configuración del texto narrativo y el tiempo como categoria metafísica.

  19. Tratamiento de aguas contaminadas con cianuro

    OpenAIRE

    Aguilar Roca, Fernando; Aguilar Roca, Fernando; Aguilar Roca, Fernando

    2011-01-01

    El presente trabajo “Tratamiento de aguas contaminadas con cianuro" se divide en tres partes: química del contaminante, procesos de descontaminación estudiados y un procedimiento basado en una investigación reciente. Principales propiedades físico-químicas del ion cianuro y de sus principales compuestos, cianuros simples o complejos, en medio acuoso. Métodos y procesos que existen para la descontaminación de efluentes cianurados, tanto los que se dan de forma natural (degradación natu...

  20. Las personas con discapacidad en el cine

    OpenAIRE

    Monjas, M.I.; et al.

    2005-01-01

    Una forma de comprobar cómo se conceptualiza a las personas con discapacidad es analizar la imagen que de ellas se refleja en distintos soportes como pueden ser libros, prensa y medios de comunicación. El presente trabajo se centra en el estudio de películas cuyos protagonistas tienen alguna discapacidad. A tal efecto, y después de revisar una parte de la producción cinematográfica, se han seleccionado cincuenta películas para proceder a su análisis. Los resultados obtenidos evidencian la esc...

  1. Estudio de viabilidad de cajones marítimos con hormigón con fibras

    OpenAIRE

    Ramos Marquès, Òscar Gonçal

    2017-01-01

    En este TFM se estudia la viabilidad de sustituir la armadura pasiva por fibras metálicas o plásticas en cajones para obras portuarias. Para ello, en primer lugar, se estudia el procedimiento constructivo de un cajón y las propiedades de los hormigones con fibras. Una vez analizado el estado del conocimiento se procede a escoger un cajón representativo, ya construido con armadura pasiva. El cajón escogido es el cajón tipo 3 del dique est...

  2. Pulpotomía con electrocauterio en pacientes con hemofilia A

    OpenAIRE

    Carbonell Rodriguez, Rosario

    2017-01-01

    Rehabilita la salud oral de un niños con Hemofilia A, empleando el Electrocauterio como una alternativa en la terapia pulpar, específicamente en la pulpotomía, para lo cual se realiza un minucioso examen clínico y anamnesis, que respaldados con exámenes complementarios, permite determinar el plan de tratamiento. La rehabilitación integral se llevó a cabo, previo consentimiento informado del padre, en sala de operaciones bajo anestesia general, debido a la condición sistémica del niño. Incl...

  3. SATISFACCIÓN CON EL TRATAMIENTO EN PACIENTES DE ATENCIÓN PRIMARIA CON ARTROSIS

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    Carmen Llanos Val Jiménez

    2017-01-01

    Full Text Available Fundamentos: Al igual que en otros problemas crónicos, en el abor - daje del paciente con artrosis se tiende a un modelo de toma de decisiones compartidas, en el que el punto de vista del paciente debe ser considerado en la elección del tratamiento. El objetivo de este trabajo fue evaluar la sa - tisfacción con el tratamiento en pacientes diagnosticados de osteoartrosis y comprobar si existen diferencias entre diferentes opciones farmacológicas, así como determinar si existe asociación con el estado funcional y las carac - terísticas clínicas y sociodemográficas. Métodos: Se realizó un estudio observacional transversal en una muestra de 487 pacientes seleccionados mediante muestreo consecutivo. La variable principal fue el nivel de satisfacción con el tratamiento (cuestionario ARTS. El estado funcional de los pacientes fue evaluado mediante la escala WO - MAC. Otras variables fueron: características del tratamiento, adherencia te - rapéutica, eventos adversos, y variables clínicas y sociodemográficas. Resultados: En el cuestionario ARTS los pacientes, en un rango entre 28 y 87, obtuvieron una puntuación media de 65,3 (DE: 9,9. La puntuación no fue significativamente diferente en consumidores de 1, 2 o más fármacos. Entre quienes consumían un solo fármaco, no hubo diferencias entre los di - ferentes tipos de fármacos. En el estado funcional se obtuvo una puntuación media de 30,2 puntos (DE: 20,8 y se observó una débil correlación negativa con el nivel de satisfacción (r= - 0,252; p<0,001. Mediante regresión lineal múltiple, se observó mayor puntuación en la escala ARTS (p<0,05 en pa - cientes con menor puntuación en la escala WOMAC, mayor edad y ausencia de eventos adversos. Conclusión : En pacientes con osteoartritis se observa un nivel mode - rado de satisfacción con el tratamiento farmacológico, condicionado por su situación funcional, sus características sociodemográficas y por la presencia de eventos

  4. Síndrome disejecutivo en personas con dependencia alcohólica con/sin recidivas

    OpenAIRE

    Fidalgo Martínez, Eva

    2015-01-01

    [ES]En la mayoría de las culturas el alcohol es el depresor del SNC utilizado con más frecuencia y el responsable de una morbilidad y una mortalidad considerables. En la actualidad, el consumo de bebidas alcohólicas constituye un importante problema de salud pública, especialmente en España, que es uno de los países con mayor consumo de alcohol por habitante y año. El objetivo general del estudio es determinar la existencia de diferencias significativas entre dos grupos clínicos de sujetos...

  5. Intervención con menores con adicciones desde Proyecto Hombre

    OpenAIRE

    Pardo-Esteban, Belén; Escalza-González, Amaia

    2015-01-01

    En el citado seminario informaremos del programa de atención y prevención de las toxicomanías y otras adicciones, llevado a cabo por la Asociación Proyecto hombre. A continuación, trataremos la formación en adicciones y toxicomanía con los estudiantes, mediante charla, cuestionarios sobre conocimientos básicos en prevención de toxicomanía en menores y los diferentes programas de intervención posibles aducados a los perfiles de los usuarios. Terminaremos con un debate sobre los temas tra...

  6. Experimentos en Antioquia con Fertilizantes para Papa.

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    Yepes Y. Emilio

    1949-03-01

    Full Text Available Con el fin de determinar la adecuada aplicación de cal. y la economía de la adición de Nitrógeno, Fósforo y Potasio, se realizaron varios experimentos de fertilizantes para papa en dos regiones productoras de Antioquia. Los experimentos se realizaron por el sistema de "Parcelas divididas y del análisis estadístico de los resultados pueden sacarse las siguientes conclusiones: 1. Es notable la deficiencia de Fósforo en ambos suelos, y hubo respuesta significativa a una aplicación de 120 kilos por hectárea. 2. Los suelos responden a una aplicación moderada de Nitrógeno y Potasio, pero puede lograrse una economía importante en las cantidades de estos elementos. 3. Parece que la aplicación de dos toneladas de cal por cuadra es suficiente, y no hay razón para aconsejar cantidades mayores. 4. Los resultados obtenidos demuestran que sí es posible la experimentación con fertilizante para papa, y sería de desear que se ampliara la experimentación en las demás regiones productoras.

  7. Polimiositis con incapacidad ventilatoria e insuficiencia respiratoria

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    O. Lopez Gaston

    2003-10-01

    Full Text Available Las miopatías inflamatorias son un grupo de enfermedades caracterizadas por debilidad muscular proximal y las causas mayores incluyen la polimiositis (PM, dermatomiositis y miositis por cuerpos de inclusión. Las complicaciones respiratorias son comunes y adquieren importancia por la incidencia sobre el pronóstico. Las tres formas de compromiso frecuentes son la enfermedad pulmonar intersticial, la neumopatía aspirativa y la incapacidad ventilatoria (IV por debilidad de los músculos respiratorios, que en su forma severa es de ocurrencia excepcional. Se presenta una paciente con IV sin compromiso del parénquima pulmonar, que requirió asistencia respiratoria mecánica por 4 meses con «destete» exitoso.The inflammatory diseases of muscle are a group of disorders characterized by proximal muscle weakness. Most cases fall into three major diagnostic categories, polymyositis (PM, dermatomyositis and inclusion body myositis. Respiratory complications are a common feature and are an important cause of morbidity and mortality. The three main types of pulmonary involvement are interstitial lung disease, aspiration pneumonia and ventilatory incapacity (VI due to muscle weakness. There are few reported cases in which mechanical ventilation has been used in patients with PM and VI in absense of lung disease. We present a patient with PM and VI due to muscle weakness who underwent therapy maintenance with mechanical ventilator and was weaned 4 months later.

  8. Optimización con Solver

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    Sánchez Álvarez , I.

    1998-01-01

    Full Text Available La relevancia de los problemas de optimización en el mundo empresarial ha generado la introducción de herramientas de optimización cada vez más sofisticadas en las últimas versiones de las hojas de cálculo de utilización generalizada. Estas utilidades, conocidas habitualmente como «solvers», constituyen una alternativa a los programas especializados de optimización cuando no se trata de problemas de gran escala, presentado la ventaja de su facilidad de uso y de comunicación con el usuario final. Frontline Systems Inc es la empresa que desarrolla el «solver» de Excel, si bien existen asimismo versiones para Lotus y Quattro Pro con ligeras diferencias de uso. En su dirección de internet (www.frontsys.com se puede obtener información técnica sobre las diferentes versiones de dicha utilidad y diversos aspectos operativos del programa, algunos de los cuales se comentan en este trabajo.

  9. con enfermedad crónica

    Directory of Open Access Journals (Sweden)

    María del Refugio Zavala Rodríguez

    2007-01-01

    Full Text Available El propósito del estudio fue determinar el grado de correlación entre los niveles de fortaleza para la salud y de ansiedad-rasgo, en unamuestra de 200 pacientes integrada por medio de muestreo no probabilístico; los criterios de inclusión fueron: adultos de ambos génerosdiagnosticados con enfermedad crónica, con estudios de enseñanza básica y orientada. El marco teórico que guió la investigación es elModelo de Adaptación de Roy. El diseño fue descriptivo, transversal, correlacional. Se utilizó el cuestionario de fortaleza de S. Pollock y laescala de autoevaluación de ansiedad-rasgo de Spielberger; los instrumentos presentaron una consistencia interna aceptable con unAlpha de Cronbach de ,852 y ,813 respectivamente. La información se recabó de cuatro instituciones de salud ubicadas en los municipiosde Tampico-Cd. Madero Tamaulipas, México. El análisis de los datos se efectuó mediante estadística descriptiva e inferencial, se utilizó elpaquete estadístico SPSS versión 13. El rango de edad que predominó fue de 45 a 64 años (54%; el 55% fue del sexo femenino, el 48%cuenta con estudios de primaria, cerca del 90% padece de una enfermedad metabólica. El coeficiente de correlación de Spearman entrela fortaleza para la salud y la ansiedad-rasgo fue significativo (Œ,570, valor Œp,000. En la mayoría de la población participante se encontróun nivel moderado tanto de fortaleza (83% como de ansiedad (73%. El análisis de regresión múltiple indicó que la relación significativaentre estas dos variables no es afectada por la edad, el género o los años de evolución de la enfermedad crónica. Los datos de este estudiomuestran evidencia de que en los pacientes con enfermedades crónicas a mayor nivel de fortaleza menor nivel de ansiedad-rasgo. Estasituación invita a desarrollar más estudios de estos fenómenos, en la disciplina de enfermería, que sirvan como sustento teórico-prácticopara el diseño o la mejora de

  10. Reducción de color con cloruro de magnesio en soluciones con colorantes comerciales

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    Mercedes Lucero Chávez

    2017-01-01

    Full Text Available Se experimentó con diferentes concentraciones de cloruro de magnesio (MgCl2 como coagulante para reducir el color en soluciones con colorantes comerciales: negro, azul y café. Los parámetros analizados fueron pH, color, demanda química de oxígeno ( dqo y sólidos suspendidos totales (sst de acuerdo con las Normas Mexicanas. Como prueba complementaria se agregó carbón activado a las muestras para reducir el color residual después de la coagulación-floculación. La reducción de los valores de color y dqo fue mayor cuando se adicionó más cantidad de MgCl2 (1.8 g/L. Los porcentajes de reducción fueron ≥90% para el color y >50% para la dqo. Los sst aumentaron con la adición de MgCl2. El carbón activado eliminó el color residual.

  11. Queratitis ulcerativa periférica bilateral en paciente con artritis reumatoide: tratamiento con infliximab

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    Vicente Aldasoro-Cáceres

    2017-01-01

    Discusión: La PUK es una enfermedad rara, pero a tener en cuenta en los pacientes con AR. En la mayoría de los casos, el tratamiento de fondo debe ser intensificado; en este sentido deben considerarse los tratamientos biológicos por su eficacia y efecto rápido de acción.

  12. ESTUDIO DE LA DIGESTION HUMANA CON ALUMNOS CON DIFICULTADES DE APRENDIZAJE

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    Mª Begoña Carretero Gómez

    2009-01-01

    Full Text Available En esta experiencia se han seleccionado una serie de actividades relacionadas con la digestión con el fin de lograr una reestructuración de ideas y cambios conceptuales, y así con la conseguir que el alumnado aprenda a construir sus propios conocimientos acerca de cómo y donde se dan los procesos de digestión necesarios para la nutrición del organismo. El trabajo se ha llevado a cabo con alumnos de 3º de Educación Secundaria Obligatoria, pertenecientesal programa de diversificación curricular. Hemos querido hacer la ciencia más cercana para que comprendan que algunos procesos tienen su explicación científica y que la ciencia forma parte de nuestro día a día, fomentando el interés del estudiante así como su implicación en el proceso de enseñanza-aprendizaje. Por ello hemos trabajado a base de pequeñas investigaciones, donde es el alumno el que marca su ritmo de trabajo, investiga y hace sus aportaciones. Han aprendido a trabajar de una forma meticulosa donde el rigor esfundamental, contagiándose del afán investigador de manera que al realizar las experiencias se han aproximado al trabajo científico a la vez que aclaran y reestructuran conocimientos.

  13. FORTIFICACIÓN DE PULPA DE UCHUVA CON CALCIO, OLIGOFRUCTOSA Y VITAMINA C, ESTABILIZADA CON HIDROCOLOIDE

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    ALBA LUCIA DUQUE

    2014-06-01

    Full Text Available La pulpa de uchuva fortificada con calcio, oligofructosa y vitamina C es un alimento funcional con grandes beneficios para la salud. Para ello se le adicionó componentes biológicamente activos, como ácidos grasos, fibra alimentaria y antioxidantes. En este trabajo se estudió la estabilidad de la pulpa enriquecida en diferentes formulaciones, teniendo en cuenta los criterios de incorporación de calcio y vitamina C (20% del valor diario recomendado VDR y para la oligofructosa (fibra dietaría en relación 1:1 con el calcio. Los resultados obtenidos mostraron que la pulpa de uchuva fortificada con CMC, ologofructosa, calcio y vitamina C presentó en promedio una estabilidad de 87,7±10,0; una viscosidad de 22,8±7,9; una aw de 0,9863±0; pH de 3,3±0,1; acidez de 1,6±0,1; grados Brix de 13,3±0,7; una densidad de 1,32±0 g/mL y las mejores respuestas sensoriales en cuanto a color, aroma, fluidez y sabor.

  14. Terapia regenerativa con plasma rico en plaquetas en pacientes con quemaduras

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    María del Carmen Franco Mora

    2015-10-01

    Full Text Available Se realizó un estudio comparativo, longitudinal y prospectivo de 60 adultos con quemaduras profundas, atendidos en el Hospital General docente "Dr. Juan Bruno Zayas Alfonso" de Santiago de Cuba, desde febrero de 2013 hasta igual mes de 2014, para evaluar la efectividad de la terapia regenerativa con plasma rico en plaquetas para la cicatrización de estas lesiones. La muestra fue dividida en 2 grupos de 30 integrantes cada uno: a los del primero se les aplicó plasma rico en plaquetas y a los del segundo sulfadiazina de plata. Se utilizaron las frecuencias absolutas y el porcentaje como medidas de resumen para variables cualitativos, así como la media aritmética y la desviación estándar para las cuantitativas. Con el proceder aplicado la cicatrización se completó en un tiempo menor, de manera que se demostró la efectividad de esta alternativa terapéutica en relación con el tratamiento convencional

  15. Efectos del contacto piel con piel del recién nacido con su madre

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    Camila Lucchini Raies

    Full Text Available Antecedentes: el contacto piel con piel ha sido una práctica ancestral en toda cultura, que debido a la institucionalización de la atención del parto, disminuyó en occidente. Se retomó en el marco de la atención humanizada del parto. Objetivo principal: determinar los efectos del contacto piel con piel del recién nacido con su madre. Metodología: revisión bibliográfica de 26 estudios. Límites de búsqueda: new borns, humans, clinical trials, de 10 años de publicación, en bases de datos: Pubmed, ProQuest, CINHAL, Lilacs. Resultados principales: Se observaron beneficios en las variables: duración de lactancia materna, comportamiento neurosensorial, regulación de temperatura, como alternativa al cuidado estándar, disminución en días hospitalización y niveles de cortisol, favorecedor de la interacción madre-hijo y desarrollo infantil. Conclusión principal: el contacto piel con piel brinda múltiples beneficios a recién nacidos. Se presenta como alternativa de cuidado segura, factible de implementar.

  16. Estudio observacional prospectivo con insulina detemir en pacientes con diabetes mellitus tipo 2 mal controlados que inician por primer vez tratamiento con insulina (estudio SOLVE

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    Domingo Orozco-Beltrán

    2016-02-01

    Conclusiones: En esta cohorte de pacientes con diabetes mellitus tipo 2 recientemente insulinizados, la insulina detemir (una vez al día mejoró el control glucémico, con baja incidencia de hipoglucemia y una reducción significativa del peso.

  17. BIOMASA Y RENDIMIENTO DE FRIJOL CON POTENCIAL EJOTERO EN UNICULTIVO Y ASOCIADO CON GIRASOL

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    J. Garduño-González

    2009-01-01

    Full Text Available El objetivo del presente estudio fue estimar la producción de biomasa, rendimiento y algunos de sus componentes de dos cultivares de frijol (Phaseolus vulgaris L. con potencial ejotero ('Acerado' y 'Criollo', en unicultivo y asociado con dos cultivares de girasol (Helianthus annuus L. ('Sunbright' y 'Victoria'. El estudio se realizó durante el ciclo primavera verano 2006 bajo condiciones de temporal en Tenancingo, Méx. Las variables evaluadas fueron: índice de área foliar (IAF, tasa de asimilación neta (TAN, biomasa total (BT, diámetro de vaina (DV, longitud de vaina (LV, número de vainas·m-2 (NV y rendimiento de vaina·m-2 (RV; las cuales fueron analizadas en arreglo factorial, bajo un diseño de bloques completos al azar con cuatro repeticiones. Para estimar el grado de asociación, entre las variables estudiadas y el rendimiento de vainas, se realizó un análisis de correlación lineal simple. En el factor cultivares se detectaron diferencias estadísticas significativas para la TAN y DV; para el factor sistemas de siembra hubo diferencias significativas para BT, DV, NV y RV. La interacción cultivares * sistema de siembra fue significativa en todas las variables, excepto en la TAN. En ese sentido, las asociaciones 'Criollo' + 'Sunbright', 'Acerado' + 'Victoria' y 'Acerado' en unicultivo, presentaron el mayor RV. Las variables IAF, BT y NV se correlacionaron positiva y significativamente con el rendimiento de vaina. Los resultados indican que 'Criollo' y 'Acerado', asociados con 'Sunbright' y 'Victoria', respectivamente, responden positivamente a la asociación, constituyendo una buena opción para los productores de la región de Tenancingo, México.

  18. Incidencia y factores asociados con las reacciones adversas del tratamiento antirretroviral inicial en pacientes con VIH

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    Juan Astuvilca

    2007-07-01

    Full Text Available La alta incidencia de reacciones adversas medicamentosas (RAMA al tratamiento antirretroviral de gran actividad (TARGA en pacientes con VIH/SIDA puede afectar la calidad de vida y adherencia al tratamiento. Objetivos: Determinar la incidencia de RAMA del TARGA inicial e identificar los factores asociados con la ocurrencia de RAMA al recibir dicha terapia. Materiales y métodos: Se realizó un estudio de cohorte histórica con todos los pacientes VIH (+ mayores de 18 años que recibieron TARGA por primera vez en el Hospital Nacional Arzobispo Loayza, con un seguimiento de 360 días desde la primera prescripción. Se recabó las RAMA de las historias clínicas y tarjetas de control. Resultados: Se incluyeron 353 pacientes, se encontró una incidencia acumulada de 66,7% de efectos adversos al TARGA inicial y una densidad de incidencia de 9,1 eventos de RAMA por 10 personas año de seguimiento (IC95%: 8,1-10,1. Anemia (23,4%, náuseas (20,6% y rash (17,2% fueron las RAMA más frecuentes. El uso de drogas (OR 2,40; IC95% 1,01-5,67; consumo de alcohol (OR 0.32; IC95%: 0,19-0,55 y estadio SIDA (OR 0,20; IC95%: 0,04-0,95 estuvieron asociadas con la presencia de RAMA. Conclusiones: Existe un alta incidencia de RAMA, siendo la anemia la más frecuente. El uso de drogas es un factor de riesgo para presentar RAMA.

  19. Hidroxilación de fenol con catalizadores ZSM-5 modificado con cobre

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    César Augusto Caro

    2005-01-01

    Full Text Available Se sintetizaron varios catalizadores Cu-ZSM-5 con diferentes relaciones Si/Al y Si/Cu. Usando metilamina o hidróxido de sodio como agente mineralizante. Los catalizadores se caracterizaron por DRX, IR, análisis BET, UV-VIS y análisis elemental. El desempeño catalítico de los catalizadores sintetizados se evaluó en la hidroxilación de fenol con H2O2. Se encontró que la relación catecol (CAT/(hidroquinona (HQ + p-benzoquinona (PBQ aumentó con el contenido de agua, presentó un máximo cuando la relación en peso de agua/fenol fue de 53/1 y se favoreció a altas temperaturas, con el aumento del contenido de aluminio o con la disminución en el contenido de catalizador. El contenido de cobre no fue un factor determinante para la conversión ni para la selectividad en la hidroxilación de fenol. La producción de la p-benzoquinona (PBQ, producto de oxidación de la hidroquinona, fue mínimo cuando se usaron las siguientes condiciones: 80 ºC, fenol: 1 mmol, relación molar fenol/H2O2 de 3/1, catalizador: 20 mg, agua: 5 g, tiempo de reacción: 4 h.

  20. Los profesores de alumnos con altas habilidades

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    Cándido GENOVARD

    2010-01-01

    Full Text Available En este trabajo analizamos las características de los profesores expertos para alumnos de altas habilidades. Se analizan los antecedentes del tema y el proceso instruccional para atender la diversidad de estos alumnos. Se destaca el valor de las interacciones entre profesor¿ alumnos y los estilos de enseñar y aprender. Asimismo, recogemos pautas de acción y recursos instruccionales a utilizar en el aula para la enseñanza de estos alumnos. No hay un profesor ideal para los alumnos con altas habilidades, pero los docentes deben conocer qué son y cómo funcionan los procesos de enseñanza¿aprendizaje y las variables psicológicas, de contenidos y contextuales implicadas en éstos.