Immunoglobulin M (IgM) Nephropathy is a primary glomerulonephritis, characterized by predominant IgM deposition on direct immunofluorescence (DIF) microscopy. Long considered to be similar to minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS), it is now considered to be a separate entity with unique epidemiology, clinical presentation, biopsy findings, treatment response and prognosis. Although steroid is the first line treatment, there is a high degree of steroid resistance. Other immunosuppressive egents have not been quite effective. Rituximab is an emerging treatment option. We present a 40-year-old man with IgM Nephropathy in whom Rituximab proved to be a highly effective treatment.
Background: Asia has greatest growth rate for number of chronic diseases and end-stage organ failure, but has the lowest rate of organ transplant, particularly deceased donor. This survey was conducted to assess knowledge, awareness, and attitude regarding deceased organ-tissue donation among health care professionals (HCP) in Bangladesh. Methods: This was a cross-sectional observational study, done in six tertiary hospitals of Dhaka from January to June 2022. A questionnaire constructed by the Asian Society of Transplantation was used for data collection. Statistical analysis was performed by SAS software. Results: A total of 446 HCP participated. There were nurses (42.0%), medical students (29.0%), doctors (21.0%), and others (8.0%). The mean age of participants was 28 years; 44.0% were male participants and 56.0% were female. About 92.6% participants heard about organ-tissue donation, but majority (75.2%) never participated in educational programs and only 8.3% knew it well. About 72.3% HCP showed positive attitude towards organ-tissue donation. Nonetheless, 24.0% strongly wished to donate and only 0.9% registered as donor. The main reason for not willing to donate were never thought about it (29.0%) and lack of information (27.0%). The principle cause of not registering was not knowing the procedure (34.4%). Majority advised outdoor campaigns, educational activities, and TV commercials as necessary sources for activating brain death donation. However, most reliable source for information chosen was TV (53.7%), Internet, and newspaper. Majority felt that when promoting life-sharing, the essential message that should be delivered is giving new lives to patients. About 85.0% participants knew the difference between brainstem death and vegetative state, 75.0% considered brain death as death and 46.4% assumed one brain death donor can save five persons lives. Conclusions: There was lack of knowledge, awareness, and attitude about deceased organ-tissue donation among HCP in Bangladesh. More educational program is needed to improve deceased organ transplantation.
Background: CMV is the one of the most common viral infections after kidney transplantation with significant morbidity and mortality. Currently sero-positivity for CMV IgG before solid organ transplantation is the laboratory test of choice for stratifying the risk of CMV reactivation after transplantation. Test for CMV specific T cell responses have been proposed to change the current risk stratification strategy using CMV assays. We therefore tested QuantiFERON cytomegalovirus assay for evaluation of CMV reactivity among renal transplant recipient and donor in Bangladesh. Methods: A retrospective study was done with 326 subjects from 2017 to 2021 in Kidney Foundation Hospital and Research Institute, Bangladesh. Data analyzed by statistical analysis software. Results: In this study for assaying CMV reactivity by QuantiFERON cytomegalovirus assay, 326 subjects were included. The mean age was 38.5 (SD, 11.9) years, 140 (42.9%) were female, 186 (57.1%) were male. Among these 191 (58.6%) were recipients with mean age 34.3 (SD, 10.7) years and 135 (41.4%) were donors with mean age 44.4 (SD, 11.0) years. Among recipient 68 (20.86%) was reactive and 123 (37.7%) was non-reactive to QuantiFERON cytomegalovirus assay. Among donor 65 (19.94%) was reactive and 70 (21.47 %) was non-reactive to the assay. Recipients were significantly less reactive to donors (P<0.02). In our study younger people were less reactive to this assay (P<0.001) which can be explained by recipients being of younger age. In our study it has also been shown that most of the recipients being male 145 (76%) and donor being female 94 (70%). Conclusions: This is the baseline study for evaluation of CMV reactivity in kidney recipients and donors of Bangladesh. This knowledge will be used for stratifying the risk of CMV reactivation after transplantation.
Moyamoya disease is a cerebrovascular condition characterized by progressive occlusion of the cerebral vessels, particularly in the circle of Willis. This report describes the case of a 12-year-old boy presenting with a history of recurrent right-sided weakness over a period of seven years. Magnetic resonance imaging revealed evidence of both old and recent infarcts, as well as encephalomalacic changes. The diagnosis was confirmed by magnetic resonance angiography, which demonstrated severe stenosis in both internal carotid arteries and the presence of significant collateral formation. In Bangladesh, surgical revascularization for Moyamoya disease had not been previously attempted, and due to financial constraints, the patient’s family opted for conservative management with anti-platelet therapy and regular follow-ups. Although a hereditary component is often presumed in Moyamoya disease, no such familial history was identified in this case. Additionally, no associations with immunological, infectious, hematological, vascular, or congenital syndromes were found. Mortality rates for Moyamoya disease are approximately 10% in adults and 4.3% in children, with a significant proportion of affected individuals experiencing cognitive decline. However, the patient in this case maintained intact cognitive function, and with diligent follow-up and anticoagulation therapy, it was anticipated that his functional capacity would remain stable.
Infections are common complications in kidney transplant recipients owing to the lifelong immunosuppression. Cytomegalovirus (CMV) and varicella zoster virus (VZV) infections are quite common in the posttransplant period. Coinfection with both however has been reported only once. The immunomodulatory effect of CMV makes their interaction with other organisms like VZV potentially sinister. This is a case of a female who developed coinfection with HZV and CMV in the first month following a live related kidney transplantation. A 32-year-old female, with a presumptive diagnosis of glomerulonephritis, hypertension and chronic kidney (CKD) underwent live-related kidney transplantation from her mother following a period of hemodialysis for 8 months. There was one haplotype match and both B and T cell cross matches were negative. CMV DNA was undetected in both donor and recipient prior to transplantation. Immunosuppression consisted of prednisolone, mycophenolate sodium and tacrolimus. The surgery went well, yielding a urine output of 4-6 L/day. However, the serum creatinine did not reach baseline, with a nadir of 2 mg/dL. On the postoperative day (POD) 25, she developed low grade fever, which was followed 2 days later by pain and vesicular eruptions involving the dermatomal distribution of the ophthalmic division of the trigeminal nerve (V1) on the left, resembling herpes zoster ophthalmicus. CMV polymerase chain reaction (PCR) yielded 300 copies/mL. Treatment was immediately started with oral Acyclovir, which was later switched to oral Ganciclovir, along with acyclovir ointment and ganciclovir eye gel. The patient's fever subsided and the skin lesions resolved over a period of 2 weeks. Serum creatinine came down to baseline. Infections after kidney transplant is often difficult to diagnose, manage and cure, especially in the immediate posttransplant period. In our case, early diagnosis and treatment resulted in a good outcome.
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