Recurrent nephrolithiasis and loss of kidney function: a cohort study.

PURPOSE: To evaluate whether symptomatic recurrent nephrolithiasis leads to loss of kidney function. METHODS: Adults who presented to the Emergency Department at least twice with symptomatic and radiologically confirmed nephrolithiasis were retrospectively recruited. Primary endpoint was the change in glomerular filtration rate (GFR) between baseline and at the time of data collection. Secondary endpoints include GFR slope defined as the mean rate of change in GFR from baseline to the end of the study period. RESULTS: 240 patients had recurrent symptomatic nephrolithiasis. Median follow-up was 5.4 years. The median age of first acute presentation was 51.6 years and the median baseline serum creatinine (bsCr) was 85.5 umol/l. 17.5% (n = 42) had worsening GFR, with the average change in GFR of - 8.64 ml/min/1.73 m2 per year. Four patients progressed to ESKD requiring haemodialysis. 14.5% (n = 35) had calcium oxalate stones. Univariate analysis showed older patients (p < 0.001), more symptomatic stone episodes (p < 0.001) and non-calcium-containing stones (p < 0.001) were strongly associated with deteriorating kidney function. Age (p = 0.002) and number of acute stone episodes (p = 0.011) were significant predictive factors when unadjusted to co-morbidities. Age (p = 0.018) was the only predictive factor of worsening GFR when adjusted for co-morbidities. Average mean GFR slope was - 2.83/min/1.73 m2 per year. CONCLUSIONS: Recurrent symptomatic nephrolithiasis is associated with loss of kidney function, in older patients, increased episodes of symptomatic nephrolithiasis and non-calcium-containing stones. Age is the only predictive factor for progression to chronic kidney disease in this subgroup.

Gadolinium-Induced Acute Graft Pancreatitis in a Simultaneous Pancreas-Kidney Transplant Recipient.

Gadolinium-induced acute pancreatitis is a rare phenomenon associated with the administration of gadolinium-based contrast agents. Only five cases of gadolinium-induced acute pancreatitis have been reported worldwide in patients with native pancreas and none with a pancreatic graft. We present a 32-year-old woman with prior history of simultaneous pancreas-kidney transplant who presented with generalized abdominal pain associated with systemic inflammatory response syndrome requiring admission to the intensive care unit. This occurred within 48 hours after having a magnetic resonance imaging (MRI) with gadolinium for investigation of subacute left optic atrophy. She was noted to have a marked rise in serum lipase, and the computed tomography findings were consistent with acute graft pancreatitis. Other causes of pancreatitis were ruled out, and she was managed conservatively with aggressive hydration, bowel rest, and analgesia with good recovery. This is the first reported case of gadolinium-induced acute graft pancreatitis occurring in a simultaneous pancreas-kidney transplant recipient. Clinicians should consider this rare differential diagnosis as a cause of graft pancreatitis in patients who have received gadolinium-based contrast agents.

Isolated Mesenteric Calciphylaxis With Ischemic Colitis in a Hemodialysis Patient Without Active Cutaneous Calciphylaxis: A Case Report of Calcific Uremic Arteriolopathy.

Calciphylaxis, also known as calcific uremic arteriolopathy, is a devastating systemic disease most commonly associated with chronic kidney failure. Its hallmark histopathologic features of small-vessel calcification, intimal hyperplasia, and microthrombi lead to microvascular occlusion and tissue necrosis. Clinically, it typically presents with painful cutaneous lesions that may be distal or proximal, with proximal lesions associated with higher mortality. Visceral involvement in this disease process is rare and in such case reports, all patients have coincident active cutaneous lesions. We present a case of a man in his 40s receiving hemodialysis presenting with mesenteric calciphylaxis complicated by ischemic colitis without active cutaneous lesions. Treatment consisted of sodium thiosulfate, vitamin K, and surgical resection. He previously had penile calciphylaxis treated with 3 months of sodium thiosulfate therapy and optimization of his serum calcium, phosphate, and parathyroid hormone levels. His penile calciphylaxis healed 12 months before his presentation with mesenteric calciphylaxis. This is the first known case report of isolated mesenteric calciphylaxis. It raises a number of clinical dilemmas, including duration of sodium thiosulfate use, monitoring for disease activity, and suitability for future kidney transplantation.

Multiple cutaneous leiomyomas leading to discovery of novel splice mutation in the fumarate hydratase gene associated with HLRCC.

Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a rare autosomal dominant condition, which manifests as cutaneous leiomyomas (CL), uterine fibroids and renal cell cancer (RCC). We describe the case of a 53-year-old woman who presented with multiple CL with a novel heterozygous canonical splice site mutation in intron 9 of the fumarate hydratase (FH) gene IVS 9-1 G>C (NM_000143.3:c 1391-1 G>C) that was not detected on initial screening of a mutation hotspot but was picked up on sequencing the remaining exons and splice site junctions. This report highlights the importance of clinical suspicion in the diagnosis of HLRCC in the absence of a family or personal history of cancer and despite initial genetic testing being negative.

Preventive behaviours towards influenza A(H1N1)pdm09 and factors associated with the intention to take influenza A(H1N1)pdm09 vaccination.

PURPOSE: (i) To determine knowledge of, and self-protecting preventive behaviours towards influenza A(H1N1)pdm09 and (ii) to identify the factors influencing intention to take influenza A(H1N1)pdm09 vaccination among the study population. MATERIALS AND METHODS: This is a cross-sectional survey carried out in Mantin Town, a semi-urban area of Malaysia. A structured questionnaire consisted of sociodemographic characteristics, knowledge of pandemic influenza symptoms, mode of transmission, self-protecting preventive behaviours, and intention to receive the influenza A(H1N1)pdm09 vaccine was used for face-to-face interviews with the household members. RESULTS: Of 230 who heard about pandemic influenza A(H1N1), 86% had misconception about mode of transmission of influenza A(H1N1)pdm09, and 52% had sufficient self-protecting behaviours. A majority (58.3%; 134/230) had intended to receive the vaccine. In the multivariate analysis, the intention to get vaccinated was significantly higher among 'those who trusted in efficacy of vaccine for prevention of influenza A(H1N1)pdm09' (p<0.001), 'those who were equipped with higher education level' (p=0.015) and 'those who worry about themselves contracting illness' (p=0.008). CONCLUSIONS: Our findings highlight the need to scale up the community's knowledge regarding influenza A(H1N1)pdm09. Recognizing the factors affecting the acceptance of vaccination documented in this study will allow decision makers to devise effective and efficient vaccination strategies.