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.

Glomerular Diseases Associated with Malignancies: Histopathological Pattern and Association with Circulating Autoantibodies

Aim: Glomerular diseases (GD) associated with malignancies (AM, GDAM) have unique features, which are important to recognize, in the light of the progress made in cancer therapy. We aimed to describe the clinical and histopathological characteristics of patients with GDAM in relation to the presence of circulating autoantibodies, pointing to potential immune pathogenic pathways connecting cancer to GD. Materials and Methods: The included patients were studied retrospectively on the basis of a kidney biopsy proving GD and a related biopsy to establish the diagnosis of AM. We recorded patients’ demographics, serological and laboratory parameters, histopathological findings, and the type of malignancy, GD, and therapy. Results: In total, 41 patients with GDAM, with a mean age of 63.1 (±10.7) years, were studied. In 28 (68.3%) cases, GD was associated with a solid tumor, and in 13 (31.7%) patients with a lymphoid malignancy. The most frequent histopathological pattern was membranous nephropathy (43.9%). Overall, at the time of GD diagnosis, 17% of the patients were positive for antinuclear antibodies (ANA), and 12.2% for antineutrophil cytoplasmic autoantibodies (ANCA), all against myeloperoxidase (MPO). In addition, 93.3% of the patients who had membranous nephropathy were negative for transmembrane glycoprotein M-type phospholipase A2 receptor (PLA2R) antibody. Sixteen patients (39.0%) presented with acute nephritic syndrome, of whom five (31.25%) developed rapidly progressive glomerulonephritis. In a mean follow-up time of 36.1 (±28.3) months, nine (21.95%) patients ended up with end-stage kidney disease, and eight (19.5%) died. Conclusion: We found that 3.2% of patients who underwent a native kidney biopsy in our institution during the past decade, for any reason, were identified as having some type of GD associated with a malignancy. Serology indicated a significant presence of ANA or MPO-ANCA antibodies in patients with nephritic syndrome and the absence of PLA2R antibodies in patients with membranous nephropathy.

Rapidly progressing glomerulonephritis in children

Rapidly progressing glomerulonephritis in children is a rare but the most aggressive form of glomerulonephritis. This disease is clinically characterized by acute nephritic syndrome with rapidly progressive renal failure, morphologically it is characterized by the presence of extracapillary cellular or fibro-cellular crescent in more than 50% of glomeruli. Currently the literature describes only a few cases of clinical course of glomerulonephritis in children. The article presents the researchers’ observation of rapidly progressive glomerulonephritis type I (anti-BMC+) in a 12-year-old girl. Despite immunosuppressive therapy combined with plasmapheresis, the authors observed the progress of the disease resulting in chronic kidney disease stage V. The child received renal replacement therapy with peritoneal dialysis. The course was complicated by the development of severe hyperparathyroidism.

Post-streptococcal glomerulonephritis

Post-streptococcal glomerulonephritis is a complication of Streptococcal infections that is responsible for classic acute nephritic syndrome, mostly seen in children. This is an acute nephritis associated with prominent fluid retention and oedema, hypertension and haematuria. Serum complement levels are diagnostically helpful as C3 levels are characteristically very low. However, many cases are much less severe and may pass unrecognized, only being identified by screening for dipstick haematuria. In children recovery is the rule but in adults, often with comorbid conditions, the prognosis is significantly worse. Management centres on loop diuretics plus treatment of the infection if still present, and additional hypotensive agents if required. Severe cases may require dialysis. High-dose corticosteroids have often been given in severe crescentic disease but there is no evidence that they are effective. In children, recovery of renal function is often excellent, though long-term studies now suggest that it may represent a risk factor for the development of chronic kidney disease. When it occurs in developed societies it is often in older patients with comorbid conditions and atypical presentations. Resolution may be less complete than in children.

Clinical profile and outcome of acute nephritic syndrome in children from a tertiary care centre in south India: a descriptive study

Background: Acute nephritic syndrome (ANS) is major cause of morbidity in developing countries. This study is an attempt to evaluate the clinical characteristics, complications and outcome of acute nephritic syndrome.Methods:This hospital based descriptive study was conducted at a tertiary care hospital in Kerala, South India. 103 children with ANS were analysed. Detailed clinical examination and relevant laboratory investigations were done. These children were followed up for 1 year.Results: Out of 103 patients studied 64% were male and 36% were female. The peak age group was 6 to 8 years. Skin infection was the most common predisposing condition (68.9%). Hypertension was present in 60.1% of patients. 26.2% of patients developed complications. Of these Acute renal failures was the most commonly encountered complication (18.4%). Proteinuria (87.4%) and microscopic hematuria (80.6%) were the most consistent features. 82.5 % patients had low C3 at the time of diagnosis. Majority of patients with low C3 level had positive ASO titre. (p = 0.014). At 3month follow up C3 became normal in 95.2% of patients. At 3 months’ majority of patients with normal C3 had incomplete recovery. (p = 0.010). At the end of 12m, microscopic heamaturia was present in 4 patients, persistent hypertension in 2 patients, 11 patients had proteinuria. These patients are kept under long term follow up.Conclusions:Complications and morbidity is significantly high during the acute phase in ANS. This study highlights the need for long term follow up of these patients.

Wilson’s Disease Presenting With Haematuria and Swelling

Wilson’s disease gene located in Chromosime 3 is also expressed in Kidneys. So Wilson’s disease may have renal manifestations either as primary even or secondarily as Hepato renal syndrome. Patients commonly manifest as Fanconi syndrome or Urolithiasis. Haematuria and proteinuria is a rare manifestation. Here we are presenting a case who initially presented with haematuria and proteinuria (Acute nephritic syndrome) which masked features of Wilson’s disease and late diagnosis. JCMCTA 2012; 23(1): 51-52