The diagnosis of a non-surgical cause of delayed passage of meconium in a neonate may be challenging to the pediatric surgeon. The usefulness of determining trypsin activity (TA) in stool and duodenal aspirate for the diagnosis of cystic fibrosis (CF) and the existence of an entity called transient deficiency of trypsin (TTD) was assessed in 49 neonates over a 14-month period. TA was determined by a gelatin liquefaction technique. An absence of TA was considered if gelatin liquefaction was seen at a dilution of less than 1:100. Neonates with negative activity in both samples were started on supplementary pancreatic enzymes (pancreatin) for 1 month. TA was retested in the stool after stopping pancreatin for 1 week. Neonates with a return of TA were considered to have TTD; those who showed persistent TA deficiency at re-evaluation were investigated for CF by sweat iontophoresis. Twenty-one neonates had negative TA (in 1 only stool was tested); 13 could be re-evaluated (4 died, 4 were lost to follow-up). Nine were found to have TTD as a stool or duodenal sample showed the presence of TA; 3 of 4 patients with persistent negative TA were confirmed to have CF. One patient with normal sweat chloride is awaiting genetic studies. Determination of TA by gelatin liquefaction is a simple, rapid, inexpensive, and reliable (sensitivity 100%, negative predictive value 100%) means to differentiate non-surgical causes of intestinal obstruction such as neonatal and postoperative TTD and CF.
BACKGROUND Cytopenia (bicytopenia / pancytopenia) in paediatric age group patients presents with variable clinical features from pallor, fever to organomegaly. Causes vary from megaloblastic anaemia to fatal leukaemias. The purpose of the study was to evaluate the etiological and clinico-haematological profile in children with bicytopenia and pancytopenia. METHODS The present retrospective study was carried out in the section of haematology, Department of Pathology of Shri Guru Ram Rai Institute of Health and Medical Sciences, Dehradun. All paediatric cases (up to 18 yrs.) with bone marrow examination, that were presented as bicytopenia or pancytopenia by routine haematological investigations were included in the study. RESULTS A total of 126 cases were included in the study, out of which, bone marrow aspiration was done in all 126 cases and trephine biopsy was done in only 78 cases. In our study, bicytopenia and pancytopenia was seen in 57.9 % and 42.1 % cases respectively. Most cases were recorded in 2nd decade. Pallor and fever were frequently observed clinical features in both cytopenias. Splenomegaly, lymphadenopathy and hepatomegaly were observed more in bicytopenia (34.2 %, 28.8 % and 27.4 % respectively). Bleeding and petechial rash were more common in pancytopenia (30.2 % and 20.8 % respectively). Anaemia and thrombocytopenia (67.1 %) were commonest combinations of bicytopenia followed by anaemia and leucopenia (26.0 %) and thrombocytopenia and leucopenia (6.8 %). CONCLUSIONS Bone marrow aspiration and trephine biopsy are important diagnostic tools in evaluating the cases of cytopenia. Both procedures are complementary to each other. KEY WORDS Bicytopenia, Pancytopenia, Megaloblastic Anaemia, Leukemia, Children
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