Open AccessWolfram syndrome: A rare case report
Author(s) -
Arjun Padmanabhan,
Aditi Parihar,
Urmi S C Vartak,
Nitin M Gadgil
Publication year - 2019
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.217
H-Index - 31
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/ijpm.ijpm_397_18
Subject(s) - medicine , autopsy , diabetic nephropathy , reflux , pancreas , anuria , diabetes insipidus , nephrectomy , kidney , oliguria , surgery , urology , disease , renal function
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.[1],[2].