Diagnostic Accuracy of Direct Immunofluorescence Test on Paraffin-Embedded Blocks in Comparison with Frozen Section Blocks in Renal Biopsies.

Background: In several published research, the evaluation of renal disorders using immunofluorescence on formalin-fixed, paraffin-embedded (FFPE) tissue sections versus immunofluorescence on frozen sections was compared. Each technique's accuracy varies greatly. This study's objective was to assess IF-P as a potential replacement for IF-F in the diagnosis of renal biopsy specimens. Materials and Methods: To show immunoglobulin IgA, IgG, IgM, and C3 immune deposits, proteinase K digestion of paraffin-embedded renal biopsy was standardized and used in 51 renal biopsies. Sensitivity, specificity, false-positive, and false-negative values were calculated. Results: IF-P showed a sensitivity of 93.1%, 76.9%, 63.6%, and 33.3%, and a specificity of 100%, 97.3%, 95%, and 100% for IgG, IgA, IgM, and C3, respectively. Compared to cases that had both routine IF and IF-P, 50 of 51 showed either the same amount of staining for the diagnostic immunoglobulin/complement or a small amount of difference. In most of the cases (49 of 51), diagnostic findings were found. Conclusion: IF-P is a sensitive and precise approach for assessing immune deposits in renal tissue biopsies. We come to the conclusion that IF-P serves as a beneficial salvage immunohistochemistry method for renal biopsies that do not contain enough cortical tissue for IF-F.

ROHHAD Syndrome an Inconspicuous Cause of Hypernatremia, A Case Report.

Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) syndrome is a rare, life threatening disease with unknown etiology. Dysnatremia is a common finding in these patients. Here we present a 12-year-old boy with multiple admissions due to hypernatremia and was repeatedly misdiagnosed. An eventual diagnosis of ROHHAD syndrome was made by integration of the previous ignored findings of sleep apnea and obesity. The diagnosis of this rare but potentially fatal syndrome should be considered in patients with dysnatremia associated with obesity and sleep apnea disorders.

Concomitant BK virus infection and visceral Leishmaniasis in a pediatric liver transplant recipient.

BACKGROUND: Solid organ transplant recipients are vulnerable to various unusual infections. Visceral Leishmaniasis (VL) is a protozoal opportunistic infection, which may affect the immune-suppressed hosts and solid organ transplant recipients. The BK virus infection is an evolving challenge in kidney transplant recipients. However, there are very few reports of BK virus (BKV) nephropathy involving the native kidney in liver transplant recipients. To the best of our knowledge, this is the first report of the simultaneous occurrence of these rare infections in a liver transplant recipient. CASE REPORT: The patient was a 9-year-old girl, a case of liver transplantation who presented with the incidental finding of proteinuria, azotemia, and cytopenia. Investigations revealed that she had concomitant BKV nephropathy and visceral leishmaniasis. Both infections were successfully treated. CONCLUSION: BK virus should be considered as a cause of nephropathy in liver transplant recipients. The presenting features of fever, cytopenia, and splenomegaly in a post-transplant patient should remind of unusual infections such as VL other than the common post-transplant conditions.

Acute necrotizing glomerulonephritis associated with COVID-19 infection: report of two pediatric cases.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is thought to cause kidney injury via a variety of mechanisms. The most common reported kidney injury following COVID-19 infection is acute tubular injury (ATI); however, the procoagulant state induced by the virus may also damage the kidneys. CASE-DIAGNOSIS/TREATMENT: Herein, we report two cases of acute necrotizing glomerulonephritis (GN) with fibrinoid necrosis in the context of COVID-19 infection. The one with more chronic features in the kidney biopsy progressed to permanent kidney failure but the second one had an excellent response to glucocorticoid pulse therapy with subsequent normal kidney function at 2-month follow-up. CONCLUSIONS: Both reported cases had an acute presentation of kidney injury with positive nasopharyngeal PCR test for COVID-19. Based on the data review by the researchers, this is the first report of acute necrotizing GN associated with COVID-19 infection.

A novel frame shift mutation in STIM1 gene causing primary immunodeficiency.

Immunodeficiency 10 is an autosomal recessive disorder presenting with iris hypoplasia, muscular hypotonia and nonprogressive myopathy, recurrent bacterial infections, autoimmune hemolytic anemia, hypohidrosis and nail dysplasia caused by the mutation of stromal interaction molecule 1 gene (STIM1). Herein, we present a new case of STIM1 mediated immunodeficiency, carrying a novel frameshift mutation. Our patient presented with nephrotic syndrome, hypotonia, myopathy, recurrent bacterial infections, thrombocytopenia and autoimmune hemolytic anemia. She is now 23 months old and is on steroid, cyclosporine and monthly IVIG. She has had no recent significant infections and is receiving rehabilitation therapy to improve her motor skills. Rare genetic syndromes should be suspected in patients of consanguineous parents, who present with a set of different manifestations. Gathering all the patient's manifestations together and looking them as one disease should be encouraged.

Prevalence of Renal Dysfunction Among Pediatric Liver Transplant Recipients.

INTRODUCTION: Renal dysfunction is among the common and considerable complications after liver transplantation and is principally attributable to immunosuppressive medications .The purpose of this study was to define the prevalence of hypertension and renal dysfunction among pediatric liver transplant recipients. METHODS: 46 pediatric liver transplant recipients were assessed for hypertension utilizing ambulatory blood pressure monitoring (ABPM), and glomerular, and tubular renal function at the transplant clinic of Shiraz University of Medical Sciences. Results were analyzed using SPSS 19 and P value < .05 was considered statistically significant. RESULTS: The mean age of the patients was 12.2 ± 3.3 years and 24 of them were female. Considering ABPM measurements 20 patients (43.5%) were hypertensive, 37% were systolic and 36.6% were diastolic non-dippers respectively. eGFR was calculated based on different formulations and Cystatin C-based equation estimated lower GFR than Cr-based equation. Micro-albuminuria was noticed in 26.1%. Additional parameters of tubular dysfunction included hyperuricosuria (4.3%), hypercalciuria (6.5%), abnormal fractional excretion of Mg (FeMg) (43.5%), abnormal tubular reabsorption of phosphate (TRP) (4.3%), and abnormal fractional excretion of uric acid (FEUA) in 13% of the patients. We noticed a statistically significant negative correlation between hypercalciuria, microalbuminuria, FeMg (P < .05) and GFR. CONCLUSION: Renal function impairment and hypertension are frequent complications amongst pediatric liver transplant recipients. Using Cyctatin C instead of Cr based formula for GFR estimation, and blood pressure monitoring by ABPM and regular screening of renal function are essential measures for recognition and treatment of renal dysfunction in these patients.

Recurrence of nephrotic syndrome following kidney transplantation in a child with congenital nephrotic syndrome.

Recurrence of original disease is a common threat in the field of transplantation. Recurrence of nephrotic syndrome is not common in children with congenital nephrotic syndrome (CNS). We report a case of a female child with CNS who presented with nephrotic state at first month of age and became dialysis dependent at 17 months of age. After seven months of continuous ambulatory peritoneal dialysis, she received a kidney from a deceased donor. Eight months after transplantation, she presented with a full-blown feature of nephrotic syndrome. She responded well to rituximab.

Nephritic-nephrotic syndrome as a presentation of BK virus infection.

BK virus (BKV) is increasingly found as an important cause of allograft nephropathy. Nephrotic syndrome is not a usual manifestation of BKV nephropathy. Here, we report a 12-year-old boy, a case of end-stage renal disease due to nephronophthisis, who got the kidney transplanted from a 16-year-old cadaver, and after 18 months of uneventful transplantation on triple immunosuppressive therapy (mycophenolate mofetil (MMF), cyclosporin and prednisolone), presented with nephrotic feature (edema, heavy proteinuria, hypoalbuminemia and hyperlipidemia). Kidney biopsy was in favor of BKV infection and eventually ended in graft failure.