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1.
Eur J Pediatr Surg ; 21(4): 238-41, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21480163

RESUMO

BACKGROUND/PURPOSE: Retinoid-mediated signal transduction plays a crucial role in the embryonic development of various organs. We previously reported that retinoic acid induced anorectal malformations (ARM) in mice. GDF11 is a TGFß superfamily molecule and is cleaved and activated by proprotein convertase subtilisin/kexin 5 (PCSK5). PCSK5 (PC5/6) mutations result in an abnormal expression of Hlxb9 and Hox genes, which include known GDF11 targets that are necessary for caudal development in vertebrate embryos. To determine a possible role of the retinoid-mediated signaling pathway in the pathogenesis of ARM, we investigated whether all-trans retinoic acid (ATRA) affected the expression patterns of PCSK5 and GDF11 in ARM-treated mouse embryos. METHODS: Pregnant ICR-Slc mice were administered 100 mg/kg ATRA by gavage on embryonic day (E) 9.0. Embryos were harvested between days E12 and E18, and mid-sagittal sections of the hindgut region were prepared for immunohistochemistry using antibodies against PCSK5 (PC5/6) and GDF11 (GDF8/11). RESULTS: Over 95% of the embryos treated with ATRA showed ARM, with rectourethral fistula or rectocloacal fistula, and a short tail. Furthermore, most of these embryos exhibited sacral malformations, tethered spinal cords, and presacral masses resembling those malformations found in caudal regression syndrome. By E14, normal mouse embryos formed a rectum and anus, and the somites behind the hindgut were positive for PC5/6 and GDF8/11. In contrast, in ARM embryos, the somites behind the hindgut were negative for PC5/6 and GDF8/11. CONCLUSION: ATRA treatment affected the caudal development in mouse embryos, resulting in anorectal, sacral, and spinal malformations, and inhibited PCSK5 and GDF11 expression in the hindgut region. These findings indicate that the expression of PCSK5 and GDF11, which plays a crucial role in the organogenesis of the hindgut, was disturbed in the hindgut region when retinoid-mediated signaling was disrupted. This study offers a new insight into the pathogenesis of ARM in mice as affected by the interaction between ATRA and PCSK5/GDF11.


Assuntos
Anus Imperfurado/embriologia , Proteínas Morfogenéticas Ósseas/metabolismo , Colo/embriologia , Fatores de Diferenciação de Crescimento/metabolismo , Pró-Proteína Convertase 5/metabolismo , Tretinoína/efeitos adversos , Anormalidades Múltiplas/embriologia , Anormalidades Múltiplas/metabolismo , Anormalidades Múltiplas/patologia , Animais , Malformações Anorretais , Anus Imperfurado/metabolismo , Anus Imperfurado/patologia , Colo/anormalidades , Colo/metabolismo , Feminino , Imuno-Histoquímica , Camundongos , Camundongos Endogâmicos ICR , Gravidez , Fístula Retal/embriologia , Fístula Retal/metabolismo , Transdução de Sinais , Cauda/anormalidades , Cauda/embriologia , Tretinoína/administração & dosagem , Tretinoína/metabolismo
2.
Eur J Pediatr Surg ; 20(5): 325-9, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20623446

RESUMO

AIMS: In patients with an anomalous arrangement of the pancreaticobiliary duct (AAPBD), clinical presentations may differ between infants and older children. The optimal timing of surgery remains controversial, particularly in early infancy. The aim of this study was to evaluate the clinicopathological features and clinical outcomes using comparative methods between infants and older cases. MATERIALS AD METHODS: From 1983 to 2007, a total of 85 consecutive children with AAPBD were treated at our institute. They included 46 with the cystic type, 33 with the fusiform type, and 6 with the non-dilatation type. These patients were divided into 2 age groups: "infant" (n=9), <12 months old; and "older", >1 year old (n=76). A retrospective study was performed. RESULTS: Mean age was 5.2 months (range, 8 days-11 months) in the infant group and 5.2 years (range, 1.2-17.3 years) in the older group. Jaundice was significantly more frequent in the infant group ( P<0.05), whereas abdominal pain was more common in the older group ( P<0.001). Bleeding tendencies such as cranial hemorrhage or bloody stools were noted in only 3 infants. In terms of liver histology, liver cirrhosis was observed in 2 infants, one of whom was a 3-month-old girl with severe jaundice resulting in living-donor liver transplantation, despite bile drainage. A single postoperative death occurred due to an adenocarcinoma arising in a choledochal cyst in a 12-year-old girl. CONCLUSIONS: Problems characteristic of infantile AAPBD were a severe bleeding tendency and irreversible liver cirrhosis, which could develop as young as 3 months old. The surgical recommendation for infantile AAPBD is thus early surgery before the age of 3 months to prevent liver failure.


Assuntos
Ductos Biliares/anormalidades , Ductos Pancreáticos/anormalidades , Fatores Etários , Idade de Início , Ductos Biliares/patologia , Colangite/cirurgia , Cisto do Colédoco/diagnóstico , Dilatação Patológica , Feminino , Humanos , Lactente , Recém-Nascido , Cirrose Hepática/epidemiologia , Masculino , Estudos Retrospectivos
3.
Eur J Pediatr Surg ; 20(4): 222-5, 2010 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-20383819

RESUMO

BACKGROUND/PURPOSE: Meconium peritonitis (MP) may induce prolonged cholestasis after laparotomy. In this study, we investigated the postoperative clinical course of MP retrospectively and discuss the relationship between MP and the development of obstructive jaundice, including biliary atresia (BA). PATIENTS AND METHODS: Between 1979 and 2008, 23 infants with MP underwent laparotomy at our institution. Eleven of the 23 infants (47.8%) developed obstructive jaundice postoperatively. The medical charts of these 11 infants were reviewed. RESULTS: The causative disease underlying MP included jejunoileal atresia in 10 and cloacal anomaly in 1. Of these 11 infants, 4 had acholic stools. Nine of the 11 improved with conservative management including an expectant approach, choleretic agents, and exchange blood transfusion. To differentiate the diagnosis from BA, open cholangiography was required in 2 cases following negative HIDA scintigraphy and a small gallbladder on ultrasonography. One of these 2 cases was diagnosed as BA and underwent hepatic portoeneterostomy simultaneously, after which the infant became jaundice free. CONCLUSIONS: Postoperative cholestasis after MP was a transient condition in most cases. However, ultrasonography and HIDA scintigraphy should be performed to differentiate BA in infants with MP who show prolonged jaundice with acholic stools.


Assuntos
Icterícia Obstrutiva/etiologia , Laparotomia/efeitos adversos , Peritonite/cirurgia , Portoenterostomia Hepática/métodos , Colangiografia , Diagnóstico Diferencial , Feminino , Seguimentos , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Icterícia Obstrutiva/diagnóstico , Icterícia Obstrutiva/cirurgia , Masculino , Mecônio , Estudos Retrospectivos , Resultado do Tratamento
4.
Eur J Pediatr Surg ; 17(2): 96-9, 2007 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-17503301

RESUMO

BACKGROUND/PURPOSE: The anomalous arrangement of the pancreaticobiliary duct (AAPBD) is one theory used to explain the etiology of biliary atresia. We investigated whether AAPBD could be involved and evaluated its significance for the etiology of biliary atresia. MATERIALS AND METHODS: Of 43 patients with biliary atresia, the area between the common bile duct and the duodenum could be visualized by operative cholangiogram in 5 patients with an uncorrectable type of biliary atresia. Three of the 5 showed an anomalous arrangement of the pancreaticobiliary duct. In these 3 patients, the type of anomalous arrangement of the pancreaticobiliary duct and the length of the common channel were studied by operative cholangiogram. Histological findings of the gallbladder and the common bile duct were examined in addition to the measurement of the serum amylase levels. RESULTS: All 3 patients showed AAPBD with the P-C type of pancreaticobiliary junction. The length of the common channel ranged from 7 mm to 12 mm. Two of the 3 cases did not show an elevated serum amylase level. Epithelial hyperplasia of the gallbladder was observed in one patient, while the other two showed no hyperplasia. Inflammatory changes in the mucosa of the gallbladder and the common bile duct were not remarkable in these 3 patients. CONCLUSIONS: From these results it seems that AAPBD in biliary atresia might not be an etiological factor for atresia of the extrahepatic bile duct, but might be an associated anomaly in biliary atresia. Other factors should be examined to clarify the etiological factor leading to lumenal obstruction of the extrahepatic bile duct.


Assuntos
Ductos Biliares/anormalidades , Atresia Biliar/etiologia , Ductos Pancreáticos/anormalidades , Ductos Biliares Extra-Hepáticos/anormalidades , Atresia Biliar/patologia , Colangiografia , Feminino , Humanos , Lactente , Masculino
5.
Eur J Pediatr Surg ; 17(2): 100-3, 2007 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-17503302

RESUMO

PURPOSE: Despite improvements in the surgical management of biliary atresia (BA), it is still difficult to maintain good bile flow. In the present study, we examined steroid therapy and determined the appropriate dose to achieve freedom from jaundice after hepatoportoenterostomy (HPE) in the uncorrectable type of BA. METHODS: A retrospective clinical analysis was done in 23 of 29 (79 %) cases who had become jaundice-free after undergoing HPE with steroid therapy between 1988 and 2004. A correlation between the total or mean steroid dose and the postoperative jaundice period (serum total bilirubin > 1.0 mg/dl) was evaluated using linear regression analysis. The regimen was as follows: prednisolone was given intravenously, starting with 3 to 5 mg/kg/day, and then gradually tapered with repetition until freedom from jaundice was achieved. RESULTS: Age at HPE was 72 +/- 20 days (mean +/- SD), and the postoperative jaundice period was 108 +/- 68 days. Total and mean steroid doses were 118 +/- 73 mg/kg and 1.31 +/- 0.8 mg/kg/day, respectively. There was no correlation between the total steroid dose and the period of jaundice. However, there was a significant correlation between the mean steroid dose and the period of jaundice (p = 0.021). CONCLUSION: A high mean dose of steroids could shorten the jaundice period after HPE in the uncorrectable type of BA.


Assuntos
Atresia Biliar/cirurgia , Glucocorticoides/uso terapêutico , Portoenterostomia Hepática , Prednisolona/uso terapêutico , Feminino , Glucocorticoides/administração & dosagem , Humanos , Masculino , Prednisolona/administração & dosagem , Estudos Retrospectivos
6.
Eur J Pediatr Surg ; 17(1): 2-5, 2007 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-17407013

RESUMO

AIM OF THE STUDY: Although a bleeding tendency as a first symptom is a critical condition in congenital biliary dilatation (CBD), the clinical details of this symptom remain unclear. We assessed this condition in children with CBD in this paper. MATERIALS AND METHODS: Sixty-five children with CBD were treated at our institute between 1983 and 2004. The children, initially presenting with bleeding manifestations such as intracranial hemorrhage and bloody stools, were defined as the bleeding group, and the remaining children with digestive symptoms such as abdominal pain and vomiting were defined as the digestive group. The clinical features were compared between these two groups. RESULTS: In 6 of the 65 cases, bleeding manifestations were noted (9.2 %). All six had cystic-type choledochal dilatation. The mean age of the bleeding group was significantly younger than that of the digestive group, and bleeding was more frequent, especially in infants less than 12 months of age. In a laboratory study, the bleeding group showed a more prolonged blood coagulation time than the digestive group did. Serum amylase and lipase levels in the bleeding group were almost normal, while those in the digestive group were significantly higher. The direct bilirubin level in the bleeding group was significantly higher than that in the digestive group. CONCLUSIONS: Disturbed blood coagulation due to vitamin K deficiency related to cholestasis results in a bleeding tendency in children with CBD. Therefore, pediatric surgeons should be aware of this rare but critical condition which can be prevented by rapid and precise treatment with vitamin K supplementation.


Assuntos
Doenças dos Ductos Biliares/congênito , Doenças dos Ductos Biliares/diagnóstico , Hemorragia/etiologia , Dor Abdominal/etiologia , Adolescente , Antifibrinolíticos/uso terapêutico , Doenças dos Ductos Biliares/complicações , Ductos Biliares Extra-Hepáticos/patologia , Criança , Pré-Escolar , Dilatação Patológica , Feminino , Hemorragia/tratamento farmacológico , Humanos , Lactente , Recém-Nascido , Masculino , Vitamina K/uso terapêutico
7.
Eur J Pediatr Surg ; 15(6): 399-403, 2005 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-16418956

RESUMO

BACKGROUND: Congenital diaphragmatic hernia (CDH) still has a high mortality because of accompanying lung hypoplasia and persistent pulmonary hypertension. Although prognostic parameters based on perinatal measurements have been proposed, our ability to accurately predict the surgical results remains insufficient. METHODS: We treated 55 infants with CDH from 1981 to 2004. Among them, 46 patients presented respiratory distress within the first 24 hours of life. Results of surgical treatment in the 46 infants were retrospectively correlated with gender, birth weight, gestational age at diagnosis, laterality, cardiac anomalies, diaphragmatic defect area, contents of herniated viscera, and the great vessel diameters measured by echocardiography. RESULTS: Out of 46 CDH neonates, 27 (58.7 %) survived and 19 (41.3 %) died aged 3 to 17 days. Non survivors had a significantly larger diaphragmatic defect and more frequent liver herniation. Out of possible predictive parameters studied, an index of the main pulmonary artery (cross-sectional area/diaphragmatic defect area ratio) most closely correlated with the surgical outcomes. CONCLUSIONS: The postoperative prognosis of CDH infants does not depend only on pulmonary hypoplasia, but also on other factors including the magnitude of abdominal visceral herniation. In this series of patients, the most reliable prognostic predictor was a clinical index reflecting the degree of both pulmonary hypoplasia and diaphragmatic maldevelopment.


Assuntos
Hérnia Diafragmática/mortalidade , Hérnias Diafragmáticas Congênitas , Feminino , Hérnia Diafragmática/patologia , Humanos , Recém-Nascido , Masculino , Prognóstico , Artéria Pulmonar/patologia
8.
Eur J Pediatr Surg ; 12(5): 345-7, 2002 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-12469265

RESUMO

Recently a few articles have been published concerning the long-term follow-up of vaginoplasty of cloaca. However, no postoperative evaluation has been fully described and, in particular, the late complications are still unknown. We report a case of tuboovarian abscess after colonic vaginoplasty for high cloacal anomaly in a 13-year-old girl. She required a left salpingo-oophorectomy and postoperatively showed regular menstruation. Therefore we stress that tubo-ovarian abscess is one of the important late complications after colonic vaginoplasty for high cloacal anomaly.


Assuntos
Abscesso/etiologia , Cloaca/anormalidades , Cloaca/cirurgia , Ooforite/etiologia , Complicações Pós-Operatórias , Salpingite/etiologia , Abscesso/cirurgia , Adolescente , Anus Imperfurado/cirurgia , Feminino , Procedimentos Cirúrgicos em Ginecologia , Humanos , Ooforite/cirurgia , Procedimentos de Cirurgia Plástica , Salpingite/cirurgia , Vagina/anormalidades , Vagina/cirurgia
9.
Br J Surg ; 89(7): 865-9, 2002 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-12081735

RESUMO

BACKGROUND: In an anomalous arrangement of the pancreaticobiliary duct (AAPBD), two-way regurgitation, including pancreaticobiliary reflux and biliopancreatic reflux (BPR), might occur. Although pancreaticobiliary reflux has been shown by high levels of pancreatic enzymes in bile, there have been few reports of clinical evidence of BPR in AAPBD. Recently, spiral computed tomography combined with intravenous infusion cholangiography (IVC-SCT) has been reported to be useful for visualizing the biliary anatomy, which may allow a new insight into bile flow dynamics in AAPBD. METHODS: Fifteen paediatric patients with AAPBD were examined before operation using both IVC-SCT and endoscopic retrograde cholangiopancreatography. Visualization of the main pancreatic duct on IVC-SCT defined the presence of BPR. Blood samples were also analysed when the initial symptom was strongly present. RESULTS: BPR was observed in six of 15 patients. Pancreatic enlargement and hyperamylasaemia were significantly more frequent in patients without BPR than in those with BPR. Levels of enzyme markers of cholestasis were also higher in the former group. CONCLUSION: The present study, using IVC-SCT, has identified clinical evidence of BPR in AAPBD. Further studies are necessary to determine the correlation between BPR and clinical manifestations.


Assuntos
Ductos Biliares/anormalidades , Bile/fisiologia , Ductos Pancreáticos/anormalidades , Adolescente , Amilases/sangue , Refluxo Biliar/diagnóstico por imagem , Refluxo Biliar/etiologia , Criança , Pré-Escolar , Colangiopancreatografia Retrógrada Endoscópica/métodos , Feminino , Humanos , Lactente , Masculino , Ductos Pancreáticos/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos
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