Clinical and biochemical manifestations of lecithin-cholesterol acyltransferase (LCAT) deficiency include an abnormal lipid profile (characterized by hypercholesterolemia with markedly decreased high-density lipoprotein cholesterol [HDL-C] and hypertriglyceridemia), corneal opacities, hematologic abnormalities (normochromic anemia of varying intensity), splenomegaly, variable early coronary artery disease and nephropathy (initially proteinuria followed by progressive deterioration of renal function). We presented a patient with nephrotic syndrome, which renal biopsy revealed classic features of LCAT deficiency. To our knowledge, the present case is the first reported case of LCAT deficiency presenting with symptoms related to nephrotic syndrome in a patient with no obvious family history without any corneal deposits and normal HDL-C levels.
View Article and Find Full Text PDFBackground: Renal biopsy is a well-established diagnostic modality for the assessment of kidney diseases in children. It can provide diagnostic precision and prognostic value and guide in therapeutic options for many renal diseases.
Objectives: This report describes the indication, histopathological patterns, and epidemiology of renal diseases in children in India.
The kidneys are involved in significant number of patients with multiple myeloma (MM) who can present with acute or chronic renal failure, nephritic syndrome, non-nephrotic proteinuria or tubular function defects. To assess the clinical profile of kidney involvement preceding diagnosis of multiple myeloma Renal involvement in 29 cases with MM admitted over an 18-month period to our tertiary care center was retrospectively examined. Diagnosis of MM was confirmed by two or more of the following four features: lytic bone lesions, serum or urine monoclonal peak, Bence-Jones proteinuria, and greater than 20% plasma cells in bone marrow.
View Article and Find Full Text PDFBackground: To determine the knowledge, attitudes and practices regarding organ donation in western India.
Methods: Convenience sampling was used to generate a sample of 250; 200 interviews were successfully completed and used for analysis. Data collection was carried out via face to face interviews based on a pre-tested questionnaire in selected public areas of Ahmedabad, Gujarat state of India.
Despite heightened international interest in performing living donor kidney paired donation (KPD) transplantation after the publication of a research protocol by Ross and colleagues in 1997, only a few hundred have been performed worldwide. The major obstacle is that many individuals in end-stage renal disease are of blood type O and can only receive an organ from a donor of blood type O, whereas blood type O donors are "universal donors" and will be able to donate directly with an intended recipient of any blood type unless there is a positive crossmatch. To overcome this, patients with compatible but non-HLA identical donors over 45 years of age should be approached for inclusion in KPD program especially O blood group donors.
View Article and Find Full Text PDFTacrolimus, a calcineurin inhibitor, is a potent immunosuppressive agent used by a majority of transplanters across the globe. Its adverse effects include nephrotoxicity, neurotoxicity, new onset diabetes after transplant, gastro-intestinal toxicity, hepatotoxicity, and thrombotic microangiopathy. Tacrolimus-induced hepatotoxicity is a very uncommon side effect.
View Article and Find Full Text PDFPosterior reversible encephalopathy syndrome (PRES) is a clinicoradiographic entity of heterogenous etiologies, which are grouped together because of similar findings on neuroimaging studies, associated with similar symptom complex of headache, vision loss, altered mentation, and seizures. In this report, we describe a case of PRES in setting of postobstructive diuresis in a 5-year-old male child, whose solitary functioning kidney was obstructed by a 1.6-cm radio-opaque stone, who after percutaneous nephrostomy (PCN) diversion developed persistent hypocalcemia which persisted despite maximum replacement by iv calcium gluconate drip, and the child developed repeated generalized tonic clonic convulsions and became unconscious for 4 days.
View Article and Find Full Text PDFPlasmodium vivax infection is increasingly a major public health burden and the second most frequent human malaria. Higher levels of clinical severity and chloroquine resistance are major factors responsible for such increases. Malarial glomerular injury is uncommon and mainly observed in Plasmodium malariae-infected patients.
View Article and Find Full Text PDFRTx is best treatment for children with ESRD. Data scarcity on DDRTx outcome in children prompted us to review our experience. This study was undertaken to evaluate patient/graft survival, function vis-a-vis SCr, rejection episodes, and mortality in DDRTx performed in 37 children between 1998 and 2011.
View Article and Find Full Text PDFWe report a case of diabetic end-stage renal disease patient who presented with a right common carotid artery jugular arteriovenous fistula as a complication of the insertion of a polyurethane double-lumen hemodialysis catheter into the right internal jugular vein .On physical examination of the neck, a pulsating mass with a palpable thrill and a bruit was noted in the right subclavicular region. The diagnosis was confirmed by color doppler ultrasonography of the neck and carotid angiography.
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