Wolfram syndrome: A rare case report

We present an autopsy case of a 19 year old male admitted for breathlessness and oliguria. He was diabetic since 7 years of age and was on insulin. Patient was on testosterone and anti hypertensives. He was diagnosed of hypocontractile bladder and congenital bilateral megaureter with vesico-ureteric reflux 2 years back. History of hemiparesis 2 years back. CT scan of the brain showed a right fronto- parietal healed infarct. At autopsy, bilateral kidneys showed coarse granularity and scarring. Pelvicalyceal system and both ureters were dilated. A right sided intrabdominal testes was identified. On histology, kidney showed features of diabetic nephropathy and pancreas showed decreased number of islet cells. Correlating the clinical, laboratory and autopsy parameters, our case satisfies the EURO-WABB criteria (1major+2minor) for diagnosis of Wolfram Syndrome, even though genetic confirmation could not be done.

Thymoma: a pathological study of 50 cases.

BACKGROUND: A combination of epithelial cells and lymphocytes results in a varied histomorphology of thymomas and consequent varied classification systems. AIM: To correlate the Marino and Muller-Hermelink (MMH) classification with the invasive behaviour of thymomas. SETTING AND DESIGN: Retrospective analysis. MATERIALS AND METHOD: Thymomas encountered in the past 21 years were re-classified with the MMH classification and correlated with Masaoka's staging and clinical presentation. RESULTS: The thymomas formed 91% of the primary thymic epithelial tumours. Predominantly cortical thymomas (n=21) and cortical thymomas (n=22) were the common subtypes and 60% and 77% of these, respectively, were in stages II or III. Cystic change, necrosis or haemorrhage played no role in predicting invasive behaviour. Cortical epithelium correlated well with the presence of para-thymic syndromes, especially myasthenia gravis. CONCLUSION: MMH classification is easy to apply. Cortical thymomas in stage I should be followed up for possible recurrence.

Deafness in children--an analysis.

This study was conducted at the Government Deaf and Dumb School, Thanjavur to ascertain the possible etiological factors. The social outlook of the parents of these children was analysed. Boys were more often affected (60.2%). Consanguinity was observed in 65.4% parents; 23.1% had one or more affected sibling and 14.1% had affected relatives. Parents detected the hearing disability in their children between 3-6 months of age in about 79% cases. Syphilis and chickenpox were the common antenatal associated infections. Streptomycin, chloroquine and abortificients were used during early pregnancy. Eight were preterm and 9 were asphyxiated at birth. A total of 11.5% had purulent ear discharge, and 2 had ear injury. Parents were committed to help the children to be self dependent.

Delinquency in children.

Delinquency among children is not a problem only in Western countries. Psychosocial analysis of inmates of an approved school revealed that broken home was the reason for the emotional stress which triggered antisocial behaviour. Children want to be with their parents inspite of the injustice done to them.