ABSTRACT
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].
Subject(s)
Brain/pathology , Wolfram Syndrome/diagnostic imaging , Autopsy , Brain/diagnostic imaging , Diabetes Complications , Diabetic Nephropathies , Fatal Outcome , Humans , Kidney/pathology , Male , Pancreas/cytology , Pancreas/pathology , Paresis , Urinary Bladder/pathology , Young AdultSubject(s)
Graft Rejection/immunology , Immunity, Cellular , Kidney Transplantation/adverse effects , Nephritis, Interstitial/microbiology , Opportunistic Infections/microbiology , Tuberculosis/microbiology , Adult , Antitubercular Agents/therapeutic use , Biopsy , Female , Graft Rejection/diagnosis , Humans , Immunocompromised Host , Immunosuppressive Agents/adverse effects , Nephrectomy , Nephritis, Interstitial/diagnosis , Nephritis, Interstitial/immunology , Opportunistic Infections/diagnosis , Opportunistic Infections/immunology , Treatment Outcome , Tuberculosis/diagnosis , Tuberculosis/immunologyABSTRACT
Diabetes and hypertension are at present the major causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide. The stages 0-5 of CKD are defined according to the estimated glomerular filtration rate. The term chronic renal failure (CRF) typically corresponds to CKD stages 3-5. Cardiovascular disease is the main cause of morbidity and mortality in patients of CRF and ESRD. This study was undertaken to analyze the age and sex incidence, clinical features, etiology, pathology of various organs in detail, and causes of death of CRF patients. All autopsies performed on known cases of CRF and those who were diagnosed as CRF at autopsy at a tertiary care hospital in India over a 7-year period were studied. The highest number of cases of CRF fell within the 56-65 years age group with a male/female ratio of 1.38:1. Oliguria and anasarca were the most common presenting features. Chronic pyelonephritis was the most common cause of CRF in our study, followed by hypertension, diabetes, and chronic glomerulonephritis. Other causes included amyloidosis, autosomal poly- cystic kidney disease, and ischemic and multiple myeloma. Most common cause of death found was cardiovascular, followed by infections, cerebrovascular, metabolic, and other causes.
