Recurrent DHA nephropathy in renal allograft-revisiting clinicopathological aspects of a rare entity.

BACKGROUND: Adenine phosphoribosyltransferase (APRT) enzyme deficiency is a rare autosomal recessive disorder of purine metabolism affecting mainly the kidneys. It can present at any age with varying degrees of acute and chronic renal damage. Though xanthine dehydrogenase inhibitors offer effective control over the disease process, delay in diagnosis and treatment often lead to compromised function of native and even graft kidneys. METHODS: We have done a retrospective search of records of renal biopsies reported at our center during the 5-year period from 2014 to 2018 to identify biopsies with 2,8-dihydroxyadenine crystal deposits. The demographic, clinical, and histopathological findings in these cases were studied and reviewed in the light of available literature. RESULTS: Of 9059 renal biopsies received during the study period, 3 cases had the rare 2,8- dihydroxyadenine (DHA) crystals. All of them were diagnosed for the first time on allograft biopsies. CONCLUSION: A high index of clinical suspicion together with the characteristic microscopic appearance of crystals on renal biopsy and urine microscopy can clinch the diagnosis of this rare disease. Hence, improving awareness about this entity among clinicians and pathologists is extremely important.

A Study of Clinical Presentation and Correlative Histopathological Patterns in Renal Parenchymal Disease.

Suspicion and subsequent detection of renal disease is by an assessment of the urinalysis and renal function in the clinical context. Our attempt in this study is to correlate initial presenting features of urinalysis and renal function to the final histopathological diagnosis. A retrospective analysis of 1059 native kidney biopsies performed from January 2002 to June 2015 at Amrita Institute of Medical Sciences was conducted. Correlative patterns between urinalysis, renal function, and final histopathological diagnosis were studied. Five hundred and eleven (48%) patients had nephrotic syndrome. Out of these, 193 (38%) had pure: nephrotic syndrome, 181 (35.8%) had associated microhematuria, 110 (21.7%) had microhematuria and renal failure, and 27 (5.3%) had only associated renal failure. Minimal change disease (MCD) (30%), membranous nephropathy (30%), and IgA nephropathy (29%) were the major diseases in the respective groups. Five hundred and five (47.6%) patients had subnephrotic proteinuria. Out of these, 29 (5.6%) had only subnephrotic proteinuria, 134 (27%) had additional microhematuria, 300 (59%) had subnephrotic proteinuria, microhematuria, and renal failure, and 42 (8%) had subnephrotic proteinuria with renal failure. Lupus Nephritis (45% and 40%) and IgA Nephropathy (32% and 21%) were the major disorders in the subgroups respectively. Forty-two patients (3.7%) were biopsied for isolated renal failure with bland urinary sediment. Cast nephropathy and acute interstitial nephritis were the major diseases. Out of 89 patients with diabetes who were biopsied, 15 (16.8%) had diabetic nephropathy, 45 (50.5%) had no diabetic nephropathy, and 29 (32.5%) had diabetic nephropathy along with a non-diabetic renal disease. Postinfectious glomerulonephritis was the major glomerular disease. IgA nephropathy (22.2%) and membranous nephropathy (15.5%) were the major diseases in patients with diabetes with no diabetic nephropathy. In our population, MCD and membranous nephropathy formed the majority of diseases in biopsied nephrotic syndrome. Added microhematuria did not seem to decrease the incidence of either disease on the whole. We found a significant number of patients with membranous nephropathy with nephrotic syndrome, microhematuria, and additional renal failure. IgA nephropathy formed a majority of cases with nephrotic syndrome, microhematuria, and renal failure. The presence of renal failure regardless of other abnormalities in urinalysis showed a trend toward IgA nephropathy. Membranous nephropathy may have a more varied presentation than was originally thought and IgA nephropathy presenting as nephrotic syndrome may not be uncommon. MCD is the major subgroup of diseases in the pediatric population and presents both as nephrotic syndrome as well as nephrotic syndrome with microhematuria. Thus, urinalysis and renal failure may be a valuable tool in assessing renal disease.

Plaque Like Giant Dermatofibroma: A Case Report.

Dermatofibroma, also known as benign fibrous histiocytoma, is a soft-tissue tumour that usually occurs in the mid-adult life and shows a slight female predominance. Giant dermatofibroma, a very rare clinical variant, is characterised by its unusually large size, benign biological behaviour despite its large size and same histopathological characteristics as conventional dermatofibroma. We report a 63-year-old woman who presented with a large tumour on the scapular region which showed histopathological features of benign dermatofibroma.

Mn-doped zinc sulphide nanocrystals for immunofluorescent labeling of epidermal growth factor receptors on cells and clinical tumor tissues.

The field of molecular detection and targeted imaging has evolved considerably with the introduction of fluorescent semiconductor nanocrystals. Manganese-doped zinc sulphide nanocrystals (ZnS:Mn NCs), which are widely used in electroluminescent displays, have been explored for the first time for direct immunofluorescent (IF) labeling of clinical tumor tissues. ZnS:Mn NCs developed through a facile wet chemistry route were capped using amino acid cysteine, conjugated to streptavidin and thereafter coupled to biotinylated epidermal growth factor receptor (EGFR) antibody utilizing the streptavidin-biotin linkage. The overall conjugation yielded stable EGFR antibody conjugated ZnS:Mn NCs (EGFR ZnS:Mn NCs) with a hydrodynamic diameter of 65 ± 15 nm, and having an intense orange-red fluorescence emission at 598 nm. Specific labeling of EGF receptors on EGFR(+ve) A431 cells in a co-culture with EGFR(-ve) NIH3T3 cells was demonstrated using these nanoprobes. The primary antibody conjugated fluorescent NCs could also clearly delineate EGFR over-expressing cells on clinical tumor tissues processed by formalin fixation as well as cryopreservation with a specificity of 86% and accuracy of 88%, in comparison to immunohistochemistry. Tumor tissues labeled with EGFR ZnS:Mn NCs showed good fluorescence emission when imaged after storage even at 15 months. Thus, ZnS nanobioconjugates with dopant-dependent and stable fluorescence emission show promise as an efficient, target-specific fluorophore that would enable long term IF labeling of any antigen of interest on clinical tissues.

Disseminated peritoneal leiomyosarcomas after laparoscopic "myomectomy" and morcellation.

Herein is reported a case of disseminated peritoneal leiomyosarcoma arising shortly after laparoscopic myomectomy and specimen retrieval with an electromechanical morcellator. The topography of the sarcomas suggests morcellation as a contributing factor. This case shows that caution should be exercised when selecting patients for laparoscopic myomectomy and stresses the need for a thorough pathologic examination of the specimen retrieved.

Thrombotic microangiopathy with severe renal failure in adenocarcinoma.

Kidney disease frequently complicates malignancy and its treatment. The spectrum of renal disease in cancers includes acute kidney injury, chronic kidney disease and tubular disorders. Thrombotic microangiopathy (TMA) is an uncommon initial clinical presentation of malignancies. Renal failure is an extremely rare feature of cancer-associated TMA syndromes in the absence of chemotherapy. Here, we report a patient who presented to the hospital for the first time with TMA and severe renal failure requiring hemodialysis and was diagnosed with gastric adenocarcinoma.