A Case of Hearing Impairment with Renal Dysfunction.

Hearing impairment in a patient with renal failure is an important clue towards etiologic diagnosis of kidney disease. Variety of hereditary diseases, developmental defects, and toxins involve these two organs. However, additional retinopathy is seen in quite a few diseases which include Alport's syndrome and Muckle-Wells syndrome (MWS). We are reporting a case of middle-aged woman with childhood-onset of hearing impairment who presented with renal failure and was diagnosed to have renal amyloidosis on kidney biopsy but without any light chain restriction. During evaluation for live donor kidney transplant, her brother was also found to have hearing impairment and retinopathy however with normal renal function and urinalysis. Genetic testing of both of them was done for panel of mutations related to hereditary amyloidosis which revealed NLRP3 mutation in both. This mutation is characteristic of MWS which can lead to secondary amyloidosis and renal failure.

Surgical nuances in the separation of craniopagus twins - Our experience and a follow up of 15 years.

Craniopagus twins are conjoined twins fused at the cranium. This is the rarest anomaly seen in conjoined twins and craniopagus twins account for 2- 6% of conjoined twins. Conjoined twins are also extremely rare, with the anomaly seen in about 10-20 subjects per billion births. A female preponderance has been noted. Craniopagus twins can be classified into complete or partial, depending on whether or not they have shared dural venous sinuses. They can be further classified into angular or total depending on the alignment of the inter-twin longitudinal axis. Surgical separation of these cases can be an elective procedure or an emergency, mandated by the death of one of the twins. Surgical separation of craniopagus twins is a complex exercise needing detailed evaluation and planning. For the successful management of these twins, a multi-disciplinary approach involving neurosurgeons, plastic surgeons, anesthetists, radiologists, pediatric critical care specialists and ancillary staff is mandatory. We present a case of partially successful elective separation of partial angular craniopagus twins performed in 2002.The surviving twin was managed conservatively for a cerebrospinal fluid leak. The patient subsequently developed a pseudomeningocele, necessitating re-exploration, excision of the gliotic tissue, and repair of the dura and the overlying scalp flap. The patient has a skull defect for which cranioplasty has been deferred. The child is neurologically intact. The post-operative evaluation and the detailed periodic neurological assessment till date (with a follow up of 15 years) have been presented in this study.

Spectrum of renal involvement in hematolymphoid neoplasms: Renal biopsy findings of 12 cases.

Spectrum of causes for renal dysfunction in patients with hematolymphoid malignancy (excluding plasma cell dyscrasia) is varied. A retrospective evaluation of "native" renal biopsies referred to our institute during the period from January 2010 to December 2013 revealed 12 cases. Age ranged between 7 and 69 (median 54.5) years. All patients were males. The neoplasms included non-Hodgkin lymphoma, chronic lymphocytic leukemia, acute lymphoblastic leukemia, Burkitt's lymphoma, intravascular lymphoma, Hodgkin lymphoma and chronic myeloid leukemia. Proteinuria was noted in 66% of the patients (nephrotic range in 5, subnephrotic range in 3). Renal insufficiency was noted in 100% patients. Malignancy-related kidney injury was noted in 75% of the cases. Renal histology showed lymphomatous infiltration (8), membranoproliferative glomerulonephritis (MPGN) (3), intracapillary monoclonal deposit disease (1) and intravascular lymphoma (1). Distribution of lymphomatous infiltration was diffuse in 50% and focal in 50%. We observed that renal dysfunction was predominantly a direct effect, that is, lymphomatous invasion. Paraneoplastic glomerulopathic changes occur in the form of MPGN. Proteinuria of >2 g/day correlated with glomerular disease.

Primary hyperparathyroidism in a child.

Primary hyperparathyroidism is rare in children. We report a 12-year-old girl who presented with recurrent renal calculi, muscular weakness and inability to walk; was diagnosed to have parathyroid adenoma and underwent parathyroidectomy.

Burden and predictors of hypertension in India: results of SEEK (Screening and Early Evaluation of Kidney Disease) study.

BACKGROUND: Hypertension (HTN) is one of the major causes of cardiovascular morbidity and mortality. The objective of the study was to investigate the burden and predictors of HTN in India. METHODS: 6120 subjects participated in the Screening and Early Evaluation of Kidney disease (SEEK), a community-based screening program in 53 camps in 13 representative geographic locations in India. Of these, 5929 had recorded blood pressure (BP) measurements. Potential predictors of HTN were collected using a structured questionnaire for SEEK study. RESULTS: HTN was observed in 43.5% of our cohort. After adjusting for center variation (p < 0.0001), predictors of a higher prevalence of HTN were older age ≥ 40 years (p < 0.0001), BMI of ≥ 23 Kg/M2 (p < 0.0004), larger waist circumference (p < 0.0001), working in sedentary occupation (p < 0.0001), having diabetes mellitus (p < 0.0001), having proteinuria (p < 0.0016), and increased serum creatinine (p < 0.0001). High school/some college education (p = 0.0016), versus less than 9th grade education, was related with lower prevalence of HTN. Of note, proteinuria and CKD were observed in 19% and 23.5% of HTN subjects. About half (54%) of the hypertensive subjects were aware of their hypertension status. CONCLUSIONS: HTN was common in this cohort from India. Older age, BMI ≥ 23 Kg/M2, waist circumference, sedentary occupation, education less, diabetes mellitus, presence of proteinuria, and raised serum creatinine were significant predictors of hypertension. Our data suggest that HTN is a major public health problem in India with low awareness, and requires aggressive community-based screening and education to improve health.

Sunitinib induced nephrotic syndrome and thrombotic microangiopathy.

Sunitinib is an oral, multitargeted receptor tyrosine kinase inhibitor of targets such as vascular endothelial growth factor and platelet derived growth factor receptor. It is used for the treatment of metastatic renal cell carcinoma (RCC). Use of sunitinib has been associated with renal dysfunction and nephrotic syndrome. However, simultaneous occurrence of nephrotic syndrome and renal dysfunction in a patient treated with sunitinib is rare. We report a case of metastatic RCC treated with sunitinib for 22 months who presented with nephrotic syndrome and renal dysfunction. Renal biopsy was diagnostic of thrombotic microangiopathy with diffuse effacement of podocytic foot process.

Infective endocarditis as a cause of fever in hemodialysis patients.

Vascular access infections are common in maintenance hemodialysis patients especially with dual lumen cuffed catheter. Persistent infections may lead to valvular seeding and the development of infective endocarditis. Though antibiotic therapy may often suffice, many patients may require surgical correction which carries a high risk of mortality. However appropriate preoperative therapy may considerably reduce the risk of surgery in maintenance hemodialysis patients.