ABSTRACT
We report a four year old boy who presented with liver failure secondary to anti-thrombin III deficiency related Budd Chiari syndrome. He was treated with TIPSS (transjugular intrahepatic porto systemic shunt) which reversed the encephalopathy, normalised the liver function and improved growth, pre-empting the need for a liver transplantation. This is the first reported case of TIPSS in a child with a fulminant presentation of Budd-Chiari Syndrome.
Subject(s)
Budd-Chiari Syndrome , Portasystemic Shunt, Transjugular Intrahepatic , Budd-Chiari Syndrome/diagnosis , Budd-Chiari Syndrome/diagnostic imaging , Budd-Chiari Syndrome/surgery , Child, Preschool , Hepatic Veins/diagnostic imaging , Hepatic Veins/surgery , Humans , Liver/pathology , Liver/surgery , Male , Phlebography , Portal Vein/diagnostic imaging , Portal Vein/surgeryABSTRACT
OBJECTIVES: To study the prevalence of joint hypermobility in children from Mumbai, India and to study its association with malnutrition. METHODS: In a cross-sectional field study from September '02 to February '03 in Mumbai, 829 children of the lower urban socio-economic strata, between 3 and 19 years of age were evaluated independently by two observers for hypermobility using the Beighton 9-point scoring system. A score of >or= 4/9 was considered positive. Their nutritional status was stratified using standard Indian growth charts and hypermobility was quantified in various nutritional groups. Musculoskeletal symptoms were assessed by a questionnaire given to parents. Standard tests of significance (Chi square test, p<0.05-significant) were applied. RESULTS: 58.7% of the population studied, had a Beighton score >or= 4/9. There was a declining prevalence of joint hypermobility noted with increasing age. Near equal sex incidence was noted. A higher incidence of finger signs was noted in comparison to elbow hyperextension, knee hyperextension and hands-to-floor. 26% of the hypermobile population had musculoskeletal symptoms as compared with 17.2% of the non-hypermobile population (p<0.05). A positive Beighton score was found in 452/734 (61.5%) children with Grade 3 and 4 malnutrition in comparison to 35/95 (36.8%) children with normal nutrition or mild grades (Grade 1 and 2) of malnutrition (p<0.05). In the group with Grade 3 and 4 malnutrition, 26.1% of those hypermobile had musculoskeletal symptoms in comparison to 17.7% of their non-hypermobile counterparts (p<0.05). CONCLUSIONS: In our study population: 1. A high prevalence of hypermobility using Beighton's score was noted; 2. Finger signs of the Beighton score were more common than the other signs; 3. Moderate and severe malnutrition were associated with hypermobility; 4. Musculoskeletal symptoms were linked to joint hypermobility; 5. Moderate and severely malnourished hypermobile children were more likely to have musculoskeletal symptoms as compared to their non-hypermobile counterparts.