FNAB and Xpert MTB/RIF enable a rapid diagnosis in pediatric mycobacterial lymphadenitis, expediting appropriate treatment and potentially preventing morbidity and mortality.
Aim Immunoglobulin‐associated mesangiocapillary glomerulonephritis is currently the most common biopsy‐confirmed glomerulonephritis in Cape Town, South Africa. We aimed to determine the outcome of patients with a biopsy‐confirmed diagnosis of immunoglobulin‐associated mesangiocapillary glomerulonephritis at our centre. Methods A retrospective cohort study of adult patients was conducted from January 1, 2000 to December 31, 2016. The endpoint was a composite of doubling of creatinine and/or end‐stage renal disease and/or death. Cox univariable and multivariable proportional hazards models were used to examine the association between the composite endpoint and predictor variables. Survival curves were made with the use of Kaplan‐Meier estimates. Results A total of 70 patients were included in the study and their median duration of follow‐up was 30.4 months. Forty‐eight (68.6%) patients reached the composite endpoint. The proportion reaching this endpoint at 1, 3 and 5 years were 37.5%, 64.6% and 81.3%, respectively. Cox multivariable proportional hazards model identified a serum creatinine concentration > 200 μmol/L at the time of biopsy, moderate to severe interstitial fibrosis, ≥50% crescents and cyclophosphamide therapy as predictors of the composite endpoint. Conclusion Immunoglobulin‐associated mesangiocapillary glomerulonephritis remains a common glomerular pathological diagnosis in our setting and has poor outcomes. This may partially be explained by late presentation. Future research needs to focus on identifying the possible cause(s) of this common glomerular disease so that more targeted therapeutic approaches can be offered.
Atypical haemolytic-uraemic syndrome (aHUS) is a rare cause of thrombotic microangiopathy (TMA). The typical form occurs most frequently in children following infection with Shiga-like toxin-producing bacteria, whereas in the atypical form genetic mutations affecting complement regulatory proteins involved in the alternative complement pathway may be identified. The clinical features of aHUS may mimic other causes of TMA such as thrombotic thrombocytopenic purpura and malignant hypertension. We present a case of a 67-year-old woman who presented with a TMA and discuss the diagnostic challenges that were confronted due to the many overlapping clinical and laboratory features of the different causes of this syndrome. Clinicians should be aware of the varied clinical manifestations of TMAs to ensure early diagnosis and initiation of appropriate treatment.
Introduction: HIV-associated kidney diseases continue to be a major problem in South Africa. Objectives: We aimed to determine the kidney outcomes of immune-complex associated mesangiocapillary glomerulonephritis (MCGN) in patients with and without HIV. Patients and Methods: A retrospective cohort study was conducted on all adult patients with a kidney biopsy diagnosis of immune-complex associated MCGN from 1 January 2000 to 31 December 2016. We compared the proportion of HIV-positive and HIV-negative patients that reached the composite endpoint of either doubling of the serum creatinine or end-stage kidney disease. Cox proportional hazards models were employed to examine the association between the composite endpoint and predictor variables. Results: A total of 79 patients were included of which 20 (25.3%) were HIV-positive. Twentyfour patients (30.4%) reached the composite endpoint. The cumulative proportions reaching the composite endpoint at one and four years were 25.3% and 30.4% with no difference between HIVpositive and HIV-negative patients (45.0% versus 25.4%, respectively; P= 0.10). Multivariable Cox proportional hazards model identified estimated glomerular filtration rate at biopsy (hazard ratio [HR] = 0.92; 95% confidence interval [CI]: 0.84-1.00, P=0.04) and proteinuria at follow-up (HR = 1.60; 95% CI: 1.21-2.11, P<0.01) as predictors of the composite endpoint at one-year. On survival analysis, there was no difference in the composite endpoint for HIV status (P=0.09; log-rank). Conclusion: Immune-complex associated MCGN continues to be a common histopathological pattern of injury at our center. Due to late presentation, kidney outcomes remain poor, regardless of HIV status.
We report a unique primary cervical neoplasm in a 44-yr-old woman which we believe, based on the morphology and immunophenotype, represents an extremely unusual small cell variant of paraganglioma. This represents the first report of a primary cervical paraganglioma. Following chemoradiation treatment, the tumor underwent malignant transformation into an S100 and SOX10 positive sarcoma, morphologically and immunohistochemically resembling a malignant peripheral nerve sheath tumor, which we believe represents a sarcoma derived from the sustentacular cells of the paraganglioma. Mutational analysis detected a nonsense mutation of NF1 gene in the sarcoma. This further supports the diagnosis as both somatic and germline NF1 mutations have been associated with paragangliomas and malignant peripheral nerve sheath tumors. Targeted RNA sequencing (ARCHER, expanded sarcoma panel) covering many known genes implicated in sarcoma development, did not reveal any other molecular alteration (fusion or internal tandem duplication).
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