Crescentic postinfectious glomerulonephritis in an adult patient with juvenile nasopharyngeal angiofibroma

Crescentic glomerulonephritis is usually associated with an acute nephritic syndrome with rapidly declining renal function. Postinfectious cases usually have a higher possibility of recovery. Juvenile nasopharyngeal angiofibroma (JNA) is a rare, locally aggressive tumour affecting mostly young men. A 28-year-old man presented with recurrent JNA initially excised 2 years prior. The patient was initially managed as a case of airway obstruction and pneumonia. He developed tea-coloured urine, oedema and acute kidney failure requiring dialysis while awaiting surgery. Urine and immunological studies (low C3, negative antineutrophil cytoplasmic antibody and antinucleosomal antibody and high antistreptolysin O) suggested a nephritic aetiology. Nasopharyngeal swab cultures of the mass revealed gram-negative organisms. Kidney biopsy showed diffuse proliferative glomerulonephritis compatible with a postinfectious glomerulonephritis with 77% cellular crescents. The mass was excised with histopathology consistent with JNA. The patient was eventually discharged off dialysis.

Description of Renal Biopsy in Frequently Relapsing and Corticosteroid Non Responsive Nephrotic Syndrome in Childhood

A study of 71 children with nephrotic syndrome admitted to the Department of Child Health, Medical School, Sriwijaya University Palembang General Hospital between November 1981 and November 1983 has been reported. Of the 71 children, there were 50 (70.4%) boys and 21 (29.6%) girls, in which 31 were less than 6 years of age. During the first eight weeks complete rem1sswn was found in 66 patients. The other five were steroid resistant. Among the 66 patients who responded to steroid, 61 had remission with the first 4 weeks. Further observation up to two years, 3 patients were still in remission, 36 had one relapse and 22 had frequent relapses. The other 5 patients, who had remission on the second 4 weeks, one had one relapse, two had frequent relapses and the other two were not available for follow up. Renal biopsy was performed in 31 patients. The representative results of the renal biopsy were found in only 20 patients : Eleven patients showed minimal change nephrotic syndrome (MCNS). Ten of these patients had frequent relapses while one patient was steroid resistant. Two had renal insufficiency. Three patients had focal glomerulosclerosis (FGS); two of them were frequently relapsing patients and the other one had steroid resistant. Four patients had mesangial proliferative glomerulonephritis (Mes. PGN). All of them had frequent relapses. One patient with diffuse proliferative glomerulonephritis (DPGN) was steroid resistant. One patient with crescentic glomerulonephritis (CrGN) was also steroid resistant.

Postinfectious Glomerulonephritis with Crescents in an Elderly Diabetic Patient after Acute Gastroenteritis: Case Report

Postinfectious glomerulonephritis (PIGN) is primarily a disease of childhood. It occurs after upper respiratory tract infection or skin infections. Streptococcus is the most common causative agent, but in the elderly, staphylococcus is the main culprit. In adults, PIGN is more common in immunocompromised patients, particularly diabetics and alcoholics. Here, we report the case of an elderly diabetic male who presented with severe acute kidney injury with active urinary sediment after acute gastroenteritis. Additional analyses revealed a very low serum C3 level and a normal serum C4 level. Renal biopsy showed diffuse proliferative glomerulonephritis with crescents. Direct immunofluorescence showed mesangial and capillary wall staining for C3 and IgG (2+, mesangial and segmental capillary wall, granular). Renal electron microscopy showed subepithelial hump-like electron-dense deposits. The role of steroid in the treatment of PIGN is controversial and there is no standard protocol, but our patient responded very well to steroid as he did not require hemodialysis after 2 weeks of initiation of steroid therapy. We should be aware of an atypical presentation of PIGN in elderly to ensure correct diagnosis.

Various faces of the same disease: membranous nephropathy in pregnancy - A case series

Introduction. Pregnancies in women with membranous nephropathy (MN) are usually complicated by increased proteinuria and superimposed preeclampsia, and this frequently results in poor pregnancy outcomes. The aim of this paper is to present case series of pregnant women with MN and different fetal and maternal outcomes. Outline of cases. Case 1 presents a 25-year-old woman with MN, who had relapsed nephrotic syndrome in early pregnancy with proteinuria of 4.14 g/day and serum albumin of 30 g/L accompanied by hypertension. Due to a missed abortion, the pregnancy was terminated. Three months later her proteinuria was still increased, measuring 3 g/day. Case 2 presents a 29-year-old woman with a history of diffuse proliferative glomerulonephritis, who conceived with proteinuria below 0.5 g/day. The proteinuria ranged between 1 and 2 g/day from the 32nd until the 38th gestational week, when she delivered a healthy neonate. After delivery, the woman underwent a kidney biopsy, which revealed MN. Case 3 presents a 25-year-old woman with MN, whose proteinuria was 1 g/day at the time of conception, but in the 35th gestational week proteinuria of 4.2 g/day was noticed. In the 36th gestational week, increased proteinuria was detected, and a cesarean section was performed with favorable neonatal outcome. After two weeks her proteinuria dropped to 0.6 g/day. Conclusion. Pregnancies in women with MN associated with low-grade proteinuria at the time of conception may have a favorable perinatal outcome. Such pregnancies require multidisciplinary management by both obstetricians and nephrologists, and team decision regarding the best timing of delivery.

New-onset lupus nephritis after male-to-female sex reassignment surgery

We report an original case of a 27-year-old transgender woman who developed lupus nephritis after male-to-female sex reassignment surgery. The patient had been taking hormones to induce feminization since the age of 18. She was admitted with malar “butterfly” rash, anasarca and hypertension, associated with an increase in serum creatinine (1.7 mg/dl). Renal involvement was characterized by nephritic and nephrotic syndrome. Autoantibody tests were positive for antinuclear antibodies and anti-double-stranded DNA, and complement levels were markedly reduced. Renal biopsy demonstrated diffuse proliferative glomerulonephritis and granular immune complexes deposits with a “full-house” pattern at the immunofluorescence level. The induction treatment was realized with corticosteroid and cyclophosphamide and maintenance immunosuppression phase with mycophenolate, obtaining complete remission. We speculated that lupus nephritis was induced by estrogens and antiandrogen therapy and gonadectomy. In the present case, we discuss the role of sex hormones in systemic lupus erythematosus onset and review the cases linked to transgender patients.

Leishmaniasis and trypanosomiasis

Visceral leishmaniasis, also known as kala-azar, has an insidious onset with constitutional features. Subsequently the intense parasitism of the reticuloendothelial system causes hepatosplenomegaly, anaemia, leucopenia, and thrombocytopaenia as well as hypergammaglobulinaemia. Kidney involvement manifests with proteinuria up to 1 g/24 hours, micro/macrohaematuria, and leucocyturia. Kidney involvement is generally mild and reversible with the treatment of infection. Biopsy appearances of diffuse proliferative glomerulonephritis, mesangial proliferation, and occasionally focal necrotizing glomerulonephritis with crescents have been described. Defects of urinary concentration and acidification have also been observed. Acute kidney injury (AKI) may be seen in one-third of patients and is associated with increased mortality.Trypanosomiasis has two forms. It causes sleeping sickness in Africa (T. brucei, transmitted by tsetse flies) or Chagas disease in South America (T. cruzei, transmitted by reduvid bugs). There is no direct association of these conditions with nephropathy, although there is in experimental models. AKI may occur, typically as a manifestation of multi-organ failure in African trypanosomiasis. APOL1 genotypes that confer susceptibility to FSGS are protective against T. brucei infection.

Histopathological audit of renal biopsy in Nepalese children: two center experience

Background: Renal biopsy is an invaluable tool which provides histopathological description of renal disease in terms of severity of lesion and helps in formulating the long term plan. However such studies with histopathological description of renal biopsy among Nepalese children are scarce.Material and Methods: This was a retrospective study conducted among all children aged less than 18 years who underwent renal biopsy at two tertiary centers in Nepal between July 2015 and December 2017.Results: All 72 renal biopsies were done with Bard 18G spring loaded gun. Nephrotic syndrome and mixed nephritic-nephrotic features were the commonest indications for renal biopsy. IgA nephropathy was the most frequent histopathological diagnosis (20.8%) which was mainly observed in children of age group 11-18 years followed by minimal change disease (16.7%) and diffuse proliferative glomerulonephritis (16.7%). Majority of children with recurrent gross hematuria (6/7) or mixed nephritic-nephrotic features (6/8) had IgA nephropathy. Focal segmental glomerulosclerosis (6/8) was commonest in children with steroid resistant nephrotic syndrome and nephrotic syndrome with atypical features. Majority of children (10/12) with diffuse proliferative glomerulonephritis had features of poststreptococcal glomerulonephritis and 6/12 had crescents in glomeruli. Lupus nephritis (13.9%) was the commonest secondary cause of glomerular pathology and was observed predominantly in 11-18 years age.Conclusion: Renal biopsy is safe and not associated with clinically significant complications. Nephrotic syndrome was the commonest indication for renal biopsy and minimal change disease predominated. IgA nephropathy was the commonest histological diagnosis overall, as well as in children who presented with recurrent gross hematuria or mixed nephritic-nephrotic features.