Growth retardation is a significant adverse effect of imatinib in children with CML. The failure to gain appropriate height was most discernible when imatinib was initiated in the prepubertal period. Etiology and remedial measures need to be investigated.
Coeliac disease (CD) is a chronic inflammatory, multi-system disorder with protean manifestations which has been linked to various auto-immune-mediated disorders. Dilated cardiomyopathy (DCM) is a rare extra-intestinal manifestation that is being recognised increasingly in patients with CD. Two cases of CD are described, an 18-year-old boy and a 13-year-old girl, both of whom presented with rapid onset of congestive heart failure and severe left ventricular systolic dysfunction. Upper limb venous thrombosis and recurrent haemoptysis secondary to pulmonary haemosiderosis in the second case were the other unusual features. The importance of CD screening of patients with DCM and pulmonary haemosiderosis is emphasised.
cluding a severe and aggressive course of disease, predominant colonic involvement and a higher incidence of familial IBD compared to PIBD other than VEOIBD (6-17 years). 1 The incidence of PIBD is on the rise globally. 2 A complex interplay of genetic and changing environmental factors has been implicated for the rising incidence although specific factors are yet to be completely understood. 2-6 Most of the epidemiological data however are from the West including North America, Canada, and Europe. 3 A few recent studies from Asia also suggest a rising trend of PIBD. Interestingly the phenotype appears to be distinct from that of the West including a milder ulcerative colitis (UC) and a higher perianal
Objectives:
Disconnected pancreatic duct syndrome (DPDS) is frequently encountered in cases with walled off necrosis (WON). The impact of DPDS on the outcomes of pancreatic fluid collections (PFCs) is not well known. In this study, we aim to evaluate the incidence of DPDS and its clinical impact on the outcomes of endoscopic ultrasound (EUS)-guided drainage of PFC in children.
Methods:
All children with symptomatic WON who underwent EUS-guided drainage using metal stents were included in the study. At 4 weeks, pancreatic ductal anatomy was evaluated, and metal stents removed. All the children were followed at regular intervals and evaluated for the recurrence of PFC and the development of new-onset diabetes.
Results:
A total of 32 children (28 boys, median age 15 years) underwent EUS-guided drainage of WON. Resolution of WON was documented in all children at 4 weeks. Pancreatic ductal anatomy using magnetic resonance retrograde cholangiopancreatography and endoscopic retrograde pancreatography was available in 30 (93.7%) children. Of these, DPDS was documented in 25 (83.3%) children including proximal disconnection in 12 and distal disconnection in 13 children. Recurrent PFC and new-onset diabetes were found in 5 (20%) and 2 (8%) children with DPDS, respectively. Of the 5 recurrences of PFC, endoscopic reintervention was required in 3 children.
Conclusions:
Majority of the children with DPDS do not develop a symptomatic recurrence of PFC after the removal of cystogastric stents. DPDS may be a risk factor for the development of new-onset diabetes. However, future prospective studies are needed.
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