BACKGROUND Choledochal cysts are rare congenital dilatations of the biliary tract with an incidence of approximately 1 in 1000 live births. Most cases present with jaundice or other obstructive symptoms and are treated by surgical resection of the cyst and re-anastomosis. A liver biopsy at the time of surgery is mandatory to study the histological changes including fibrosis and to grade these changes to determine prognosis. We wanted to study the histology of liver biopsies received along with choledochal cysts, grade the hepatocellular damage and fibrosis, and correlate these findings with clinical presentation of the patients. METHODS 40 liver biopsies received along with choledochal cysts in paediatric patients were assessed for histological changes using 7 parameters including liver fibrosis and scored semi-quantitatively according to established scoring systems. These scores were correlated with the clinical data, age at surgery, sex of the patient, type of choledochal cyst, clinical features, and the results were analysed for their role in predicting patient prognosis. RESULTS We found that all the cases showed histological variations in the liver biopsy. Age of children showed an inverse relationship with histological scoring. Infants showed higher scores in comparison to older children. Fibrosis of the liver was also higher in Infants. Most of the cases presented with clinical jaundice. Patients with jaundice, pain abdomen, fever and acholic stools showed higher scores. We found that the sex of the patient and type of choledochal cysts were independent of the scores. CONCLUSIONS Younger age and more severe clinical presentation warrant an urgent surgical intervention in paediatric choledochal cyst. Histological assessment of the liver is mandatory to grade the hepatocellular changes including fibrosis and suggest prognosis and follow up of patients. KEYWORDS Liver Histology, Grading, Paediatric Choledochal Cyst
AIMSTo evaluate the role of blood and bone marrow findings in the diagnosis of congenital dyserythropoietic anaemias type I and type II. SETTINGS AND DESIGNDyserythropoietic anaemia is suspected when there is a suboptimal reticulocyte response for the degree of anaemia. Congenital dyserythropoetic anaemias are a group of rare hereditary disorders of hematopoiesis. It is believed that CDA is often under diagnosed. Knowledge of CDA workup in a patient of haemolytic anaemia is often rewarding. MATERIALS AND METHODSAll the cases diagnosed as CDA at a tertiary paediatric centre were reviewed with respect to clinical and serological data, peripheral smear and bone marrow study findings. The haematological changes in CDA were analyzed with respect to the diagnosis. RESULTS AND CONCLUSIONThe light microscopic findings of the peripheral smear and bone marrow aspirate were found to be highly specific for the diagnosis of CDA. Significant differences were also noted between type I and type II CDA.
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