Akash Shukla1, Pratin Bhatt2, Deepak Kumar Gupta2, Tejas Modi2, Jatin Patel2, Amit Gupte2, Megha Meshram2, Shobna Bhatia2. 1. Department of Gastroenterology, KEM Hospital and Seth GS Medical College, Multistory Building, 11th floor, Parel, Mumbai, Maharashtra, 400012, India. drakashshukla@yahoo.com. 2. Department of Gastroenterology, KEM Hospital and Seth GS Medical College, Multistory Building, 11th floor, Parel, Mumbai, Maharashtra, 400012, India.
Abstract
There are limited data on clinical profile of adolescent patients with Budd-Chiari syndrome (BCS). We studied clinical, radiological, thrombophilia profile and treatment outcomes in adolescent patients with BCS. METHODS: Forty-three consecutive patients of BCS with onset of symptoms during adolescence (10-19 years) were enrolled in the study. 129 randomly selected adult patients with BCS and 36 children with BCS formed the two control groups. The clinical history, physical examination, laboratory tests, thrombophilic disorders, radiological features and treatment outcomes of adolescents were compared to adults and children. RESULTS: In adolescents, ascites (25/43 vs. 110/129, p = 0.0004) and thrombophilic disorders (16/43 vs. 93/129 p < 0.0001) were less frequent than adults. More adolescents (14/43) presented with hepatomegaly alone without ascites than adults (9/129, p < 0.001) or children (1/36, p = 0.005). Adolescents had lower Clichy scores [3.75 (1.2)] than adults [4.72 (1.3), p < 0.0001) or children [4.43 (1.7), p = 0.041]. JAK-2 V617F mutation was the most common thrombophilic disorder in adolescents (5/43) and more common than children (0/36, p = 0.043). Response to therapy was better in adolescents (74.4%) than children (52.8%, p = 0.038), but similar to adults (63.56%, p = 0.13). CONCLUSION: During adolescence, patients with BCS present less commonly with ascites and may present with hepatomegaly alone. JAK-2 V617F mutation is the most common thrombophilic disorder during adolescence; though thrombophilic disorders are less common in adolescents than adults. Response to therapy is similar to adults, but better than children.
There are limited data on clinical profile of adolescent patients with Budd-Chiari syndrome (BCS). We studied clinical, radiological, thrombophilia profile and treatment outcomes in adolescent patients with BCS. METHODS: Forty-three consecutive patients of BCS with onset of symptoms during adolescence (10-19 years) were enrolled in the study. 129 randomly selected adult patients with BCS and 36 children with BCS formed the two control groups. The clinical history, physical examination, laboratory tests, thrombophilic disorders, radiological features and treatment outcomes of adolescents were compared to adults and children. RESULTS: In adolescents, ascites (25/43 vs. 110/129, p = 0.0004) and thrombophilic disorders (16/43 vs. 93/129 p < 0.0001) were less frequent than adults. More adolescents (14/43) presented with hepatomegaly alone without ascites than adults (9/129, p < 0.001) or children (1/36, p = 0.005). Adolescents had lower Clichy scores [3.75 (1.2)] than adults [4.72 (1.3), p < 0.0001) or children [4.43 (1.7), p = 0.041]. JAK-2 V617F mutation was the most common thrombophilic disorder in adolescents (5/43) and more common than children (0/36, p = 0.043). Response to therapy was better in adolescents (74.4%) than children (52.8%, p = 0.038), but similar to adults (63.56%, p = 0.13). CONCLUSION: During adolescence, patients with BCS present less commonly with ascites and may present with hepatomegaly alone. JAK-2 V617F mutation is the most common thrombophilic disorder during adolescence; though thrombophilic disorders are less common in adolescents than adults. Response to therapy is similar to adults, but better than children.
Authors: J B Dilawari; P Bambery; Y Chawla; U Kaur; S R Bhusnurmath; H S Malhotra; G K Sood; S K Mitra; S K Khanna; B S Walia Journal: Medicine (Baltimore) Date: 1994-01 Impact factor: 1.889
Authors: Sarwa Darwish Murad; Aurelie Plessier; Manuel Hernandez-Guerra; Federica Fabris; Chundamannil E Eapen; Matthias J Bahr; Jonel Trebicka; Isabelle Morard; Luc Lasser; Joerg Heller; Antoine Hadengue; Philippe Langlet; Helena Miranda; Massimo Primignani; Elwyn Elias; Frank W Leebeek; Frits R Rosendaal; Juan-Carlos Garcia-Pagan; Dominique C Valla; Harry L A Janssen Journal: Ann Intern Med Date: 2009-08-04 Impact factor: 25.391