Primary Budd‐Chiari Syndrome in Children

Nobre, Susana; Khanna, Rajeev; Bab, Natalie; Kyrana, Eirini; Height, Sue; Karani, John; Kane, Pauline; Heaton, Nigel; Dhawan, Anil

doi:10.1097/mpg.0000000000001587

Cited by 29 publications

(15 citation statements)

References 14 publications

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“…The histological features were, however, similar to what are documented 26 . Even though pediatric case series of BCS have been documented across continents, yet reports of children with hepatic presentations of Niemaan‐Pick Disease are quite scant from Asia‐Pacific region 27–29 …”

Section: Discussionsupporting

confidence: 58%

Clinicohistological correlation of etiological spectrum of chronic liver disease diagnosed during noncirrhotic stages in children: Can need of liver biopsy be obviated?

et al. 2020

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Background and Aim: Limited data exist regarding the etiological spectrum of the subset of chronic liver diseases (CLDs) diagnosed in noncirrhotic states in children. Our primary objective was to study the clinicoetiological profile of CLDs detected in noncirrhotic stages in children younger than 12 years of age. The secondary objective was to find the hepatic histological correlation of provisional diagnosis by different ranks of doctors. Methods: This was an observational epidemiological study, cross-sectional in design, conducted in a tertiary-care setting over a 2-year period. Results: Thirty-seven cases were enrolled, with a mean ± SD age of 8 ± 4.1 years and a male:female ratio of 1.8:1. Etiologies noted were Wilson disease (n = 8), autoimmune hepatitis (n = 4), secondary hemochromatosis (n = 4), chronic hepatitis B (n = 3), chronic hepatitis C (n = 2), non-alcoholic steatohepatitis (n = 2), progressive familial intrahepatic cholestasis (n = 2), extrahepatic biliary atresia (n = 2), Alagille syndrome (n = 1), galactosemia (n = 1), Gaucher disease (n = 1), Niemann-Pick disease (n = 1), and Budd-Chiari syndrome (n = 1), with an inconclusive diagnosis in five children. Relevant investigations were ordered more frequently by the specialist consultant (SC) and super specialist (SS) combined in comparison with the senior resident (SR) and junior resident (JR) together. (P = 0.0013). Irrelevance of the tests ordered was significantly higher in the junior tier (JR and SR; SR > JR) in contrast to the senior tier of doctors (SC and SS) (P < 0.01). The clinicohistological correlation of an etiological diagnosis significantly differed between the junior and senior ranks of physicians. We noted that an ideal clinical acumen could help to avoid liver biopsy for etiological diagnosis in 78.3% (29/37) of the study population. Conclusion: Interpretation of clinical presentation by the senior set of doctors is preferable, which could obviate the need for liver biopsy regarding diagnosis in a proportion of pediatric CLD patients.

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Section: Discussionsupporting

confidence: 58%

Clinicohistological correlation of etiological spectrum of chronic liver disease diagnosed during noncirrhotic stages in children: Can need of liver biopsy be obviated?

et al. 2020

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“…The management decision depends on several factors including local expertise, presentation of patient, site of blockage and structural anatomy. In the present era, most of the BCS are managed with radiological intervention [28], however, the risk of endothelial damage and the of a new thrombosis was a limitation for this therapeutic option in our patient. Prothrombotic states need to be worked up carefully.…”

Section: Normal Valuesmentioning

confidence: 83%

Pediatric Budd-Chiari Syndrome Secondary to Antiphospholipid Syndrome Running Title: Pediatric Budd-Chiari Syndrome

Vera¹,

Martínez²

2022

JCR

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Background: Budd-Chiari syndrome is a rare entity in childhood. Antiphospholipid syndrome is an autoimmune disease characterized by the presence of thrombosis or obstetrical complications. The presentation of both diseases is rare. Currently there are few reports in the literature of this association. Case Report: A 15-year-old male patient presented with generalized pallor, Raynaud phenomenon and pain abdomen. Imaging studies reveal portal vein and splenic vein thrombosis corroborating with angiography. Based on the thrombosis presentation, Raynaud phenomenon, aTTP prolongation, thrombocytopenia and complement consumption, diagnosis of antiphospholipid syndrome was made. He continued treatment with anticoagulation, in addition to steroid and azathioprine, presenting adequate clinical evolution. Conclusion: The seronegative antiphospholipid syndrome represents a diagnostic challenge. It´s essential to maintain a suspected diagnosis of antiphospholipid syndrome as a cause of Budd-Chiari syndrome, even in it´s seronegative presentation.

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“…Nobre et al (18) have reported on a case series of 7 children with BCS from the UK, which included 2 liver transplants and 1 meso-caval shunt. No surgical shunts or transplants for chronic liver disease were performed in this series.…”

Section: Discussionmentioning

confidence: 99%

Budd-Chiari Syndrome—A Single Center Experience From the United Kingdom

Mann

Ikram

Modin

et al. 2023

Journal of Pediatric Gastroenterology &Amp; Nutrition

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Pediatric Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single center retrospective review. Fourteen cases were identified; 6 of 14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.

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Primary Budd‐Chiari Syndrome in Children

Cited by 29 publications

References 14 publications

Clinicohistological correlation of etiological spectrum of chronic liver disease diagnosed during noncirrhotic stages in children: Can need of liver biopsy be obviated?

Clinicohistological correlation of etiological spectrum of chronic liver disease diagnosed during noncirrhotic stages in children: Can need of liver biopsy be obviated?

Pediatric Budd-Chiari Syndrome Secondary to Antiphospholipid Syndrome Running Title: Pediatric Budd-Chiari Syndrome

Budd-Chiari Syndrome—A Single Center Experience From the United Kingdom

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