Coeliac crisis mimicking nephrotic syndrome in a post-partum patient

Background: Coeliac crisis is a life-threatening presentation of coeliac disease. Severe diarrhoea, weight loss, electrolyte imbalances and malnutrition are prominent features. Although mainly a disease of childhood, it can on the rare occasion be diagnosed in adults. Case presentation: A 25-year-old female with severe generalised oedema, lower extremity weakness, hypokalemia and profound hypoalbuminemia was referred with an initial diagnosis of nephrotic syndrome. Three months previously she had given birth to a healthy child following an uneventful pregnancy. She did not have proteinuria. She had a history of diarrhoea with gluten-containing food since childhood but lacked a formal diagnosis of coeliac disease. A duodenal biopsy confirmed the suspected diagnosis. Coeliac crisis was diagnosed with life-threatening multisystem involvement. Introduction of a gluten-free diet abolished all disease symptoms and ameliorated laboratory parameters at six months’ follow-up. Conclusion: Coeliac crisis is a rare, yet dangerous presentation of coeliac disease in adults. As this case suggests, it can present with generalised oedema and hypoalbuminemia mimicking nephrotic syndrome. Rapid diagnosis is the key to successful treatment

Unexpected Late Response to Ofatumumab in Adult Post-Transplantation Recurrent Focal Segmental Glomerulosclerosis, Case Report

© 2022 Elsevier Inc.Background: Idiopathic focal segmental glomerulosclerosis is an important cause of kidney failure in adults, which is associated with a high risk of disease recurrence after transplantation. Plasmapheresis, rituximab, immunoadsorption, and high-dose cyclosporine are used to treat post-transplant recurrent focal segmental glomerulosclerosis (rFSGS). However, the response rate is variable, and few options remain for unresponsive patients. Case Report: We present a 44-year-old man with an early post-transplant rFSGS. After peritransplant plasmapheresis, rituximab, and abatacept treatments failed, we employed ofatumumab. After 9 months without apparent benefit, we observed an unexpected partial remission thereafter, without severe side effects. Furthermore, remission has been sustained in 30-month follow-up. Conclusions: We believe ofatumumab can be considered an alternative for patients with plasmapheresis and rituximab-resistant post-transplant rFSGS

Vitamin D receptor polymorphisms and bone health after kidney transplantation

Background/aim: Bone disease is one of the most prominent complications after kidney transplantation. Bone diseases include osteoporosis, persistent secondary hyperparathyroidism, and avascular necrosis (AVN). We investigated the relationship between the polymorphisms of the vitamin D receptor (VDR) gene and bone diseases occurring after kidney transplantation. Materials and methods: The study consists of 234 kidney allograft recipients with a minimum follow-up of five years after kidney transplantation. Patients with glomerular filtration rates less than 30 mL/min/1.73m2, a history of parathyroidectomy, bisphosphonate use pre- or post-transplantation, and cinacalcet use posttransplantation excluded. We evaluated associations between the polymorphisms of the VDR gene (BsmI, TaqI, ApaI, FokI, and Cdx2), the first-year bone mineral density (BMD) scores, persistent secondary hyperparathyroidism, and AVN. Results: Patients with low BMD scores were significantly younger (P = 0.03) and had higher intact parathormone (iPTH) levels (P = 0.03). Cdx2 TT genotype significantly increases the risk of low BMD scores (OR: 3.34, P = 0.04). Higher phosphate levels were protective against abnormal BMD scores (OR: 0.53; P = 0.03). Patients with persistent hyperparathyroidism had significantly longer dialysis vintage and higher pretransplantation iPTH levels (P = 0.02 and P < 0.001, respectively). Cdx2, CT/TT, and ApaI CA/AA genotypes significantly increase the risk of persistent hyperparathyroidism (OR: 6.81, P < 0.001, OR: 23.32, P < 0.001, OR:4.01, P = 0.02, and OR: 6.30, P = 0.01; respectively). BsmI CT/TT genotypes were found to increase AVN risk with an HR of 3.48 (P = 0.03). Higher hemoglobin levels were also found to decrease AVN risk with an HR of 0.76 (P = 0.05). Conclusion: Certain VDR gene polymorphisms are associated with a higher risk for bone diseases after kidney transplantation