Rathika D. Shenoy

Thrombocytopenia in Hepatitis A—An Atypical Presentation

Acute hepatitis due to hepatitis A virus (HAV) is usually a benign self-limiting disease during childhood. Autoimmune manifestations are rare with acute HAV, especially in children. We report an 8-year-old boy with severe thrombocytopenia as an initial manifestation of acute HAV infection. Bone marrow examination suggested peripheral destruction of platelets. His clinical course was complicated by persistent severe thrombocytopenia and progressive cholestasis requiring prednisolone therapy.

TRANSIENT MYELOPROLIFERATIVE DISORDER AND EOSINOPHILIC PERICARDIAL EFFUSION IN A DOWN SYNDROME NEONATE

Transient myeloproliferative disorder seen in neonates with Down syndrome is often thought to have a benign course. The authors describe the clinical and laboratory profile of a neonate with Down phenotype and transient myeloproliferative disorder with pericardial effusion as co-morbidity. Pericardial fluid analysis showed eosinophils. Pericardial effusion resolved with prednisolone therapy. Regression in hepatosplenomegaly with clearance of blasts was seen by third week of illness. The clinical course suggested a benign infiltration of the pericardium. Presence of eosinophils supports the differentiating capability of the blast cells in transient myeloproliferative disorders.

Mediastinal enteric cyst in a neonate

Mediastinal enteric cysts are relatively uncommon, and patients tend to present at a later age compared to those with duplications in other areas of alimentary canal. The tendency of enteric cyst to enlarge and produce airway obstruction is sufficient reason for early surgical removal. We report on a case of mediastinal enteric cyst in a neonate with respiratory distress for its early presentation and management. The embryological basis and anatomical issues relating to duplication cysts of the gastrointestinal tract is discussed.

Dandy-Walker malformation, occipital meningoencephalocele, meso-axial polydactyly and bifid hallux.

Introduction Dandy–Walker malformation (DWM), consisting of the triad of cystic dilatation of the fourth ventricle, cerebellar vermian hypoplasia and hydrocephalus, was first described in 1887 by Sutton. Since then the association of DWM with polysyndactyly together with other systemic and central nervous system anomalies have been designated as specific syndromes, often with overlapping features. We report a case of DWM, occipital meningoencephalocele, meso-axial polydactyly (PD) and bifid halluces with distinct radiographic and neuroimaging features.

Autoantibody profile in a cohort of South Indian children with Kawasaki disease

Objective: There is no clear data on autoantibody levels in Kawasaki Disease (KD) especially from the Indian Subcontinent. Aim: To look for the presence of organ nonspecific and organ specific antibodies to strengthen the search for an autoimmune cause of KD. We tested the presence of antinuclear antibody (ANA) and antithyroid microsomal antibody (TMA) in children with KD, 6 months after the acute phase. Anti Neutrophil Cytoplasmic Antibody (pANCA, cANCA), Anti Endothelial Cytoplasm Antibody (AECA) and Anti Smooth Muscle Antibody (SMA) was additionally tested in those with elevated titers of ANA and/or TMA. Materials and Methods: Prospective case-control study of 24 children with KD on follow up and an equal number of age and sex matched controls. Historical data about acute phase of illness was obtained from the medical records. After obtaining institutional ethics committee clearance and informed consent from the parents, blood was tested for ANA and TMA by the indirect immunofluorescence method (IIF), using a kit developed by Euroimmun. Positive samples were additionally tested for pANCA, cANCA, AECA and SMA. Relationship of autoantibody elevation and clinical course in the cases was determined. Results: The age of the study group was 4 ΁ 3.2 years. Incomplete KD was seen in 12.5% of the cases. Five cases (21%) had cardiac involvement. All but one with mitral and tricuspid regurgitation resolved after the acute phase of the disease. Only her ANA was elevated. Two children (8%) positive for TMA did not show any cardiac abnormalities. Further antibody testing was negative. All three children with elevated autoantibodies were females. (P value = 0.02: statistically significant). Conclusion: Elevated autoantibodies in three (12.5%) children after the acute phase may suggest the role of autoimmunity in the etiopathogenesis of KD, even though our observations were not statistically significant.