Changes to bone mineral density, the trabecular bone score and hip structural analysis following parathyroidectomy: a case report.

BACKGROUND: Reduction in bone mineral density (BMD) measured by dual-energy X-ray absorptiometry (DXA) occurs in secondary hyperparathyroidism associated with chronic kidney disease. BMD generally increases following parathyroidectomy, however longitudinal changes to other DXA-derived parameters, the trabecular bone score (TBS) and hip structural analysis (HSA), have not been described. Postoperative calcium requirements and positive calcium balance raise concerns for an increased risk of vascular calcification. This case illustrates the dramatic increase in BMD that can follow parathyroidectomy in a patient on dialysis, and for the first time demonstrates improvements to HSA parameters and to the TBS. CASE PRESENTATION: A 30-year old woman on haemodialysis underwent subtotal parathyroidectomy for secondary hyperparathyroidism. She developed a post-operative 'hungry bone syndrome' requiring substantial calcium and calcitriol supplementation. Six months post-parathyroidectomy, BMD increased by 42% at the lumbar spine, 30% at the femoral neck and 25% at the total proximal femur, with increases sustained over the following 18 months. The TBS increased by 8%. HSA showed a 63% increase in femoral neck cortical thickness and 38% reduction in the buckling ratio, consistent with increased femoral neck stability. The abdominal aortic vascular calcification score (0-24) increased from zero 8-years pre-parathyroidectomy to 2/24 at 18-months post-parathyroidectomy. CONCLUSION: BMD losses incurred by secondary hyperparathyroidism recover rapidly after parathyroidectomy, particularly at sites of trabecular bone. Bone architectural parameters, measured as the TBS and by HSA, also improve. Greater BMD gains may be associated with higher post-operative calcium requirements. While bone is the major reservoir for post-parathyroidectomy calcium supplementation, positive calcium balance may contribute to vascular calcification risk.

A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizumab.

BACKGROUND: Pregnancy-related Atypical Haemolytic Uremic Syndrome (P-aHUS) is a rare condition affecting genetically predisposed women during pregnancy. It is often difficult to diagnose and has a significant impact on maternal and foetal outcomes. It is characterised by microangiopathic haemolytic anaemia and kidney injury from thrombotic microangiopathy. CASE PRESENTATION: A 27-year-old female of Lebanese descent presented at 36 weeks' gestation with foetal death in-utero (FDIU) with placental abruption on a background of previously normal antenatal visits. She was coagulopathic and anaemic with anuric acute kidney injury, requiring emergency Caesarean section, intubation and dialysis. Her coagulopathy rapidly resolved, however, her anaemia and renal dysfunction persisted. A diagnosis of P-aHUS was made, and she was empirically treated with Eculizumab. Her ADAMTS13 level was normal, effectively excluding thrombotic thrombocytopenic purpura. Within 2 weeks of treatment her haematological parameters improved, and her renal function began to recover and within 2 months she became dialysis independent. CONCLUSION: This case highlights the challenges of a timely diagnosis of P-aHUS from other pregnancy-related diseases. Although our patient is dialysis-independent, her risk of relapse remains high with subsequent pregnancies. Currently we are awaiting her genetic sequencing to complete her assessment for underlying mutations and are determining the safest approach to a future planned pregnancy.

Association between Aortic Calcification, Cardiovascular Events, and Mortality in Kidney and Pancreas-Kidney Transplant Recipients.

BACKGROUND: Cardiovascular (CV) disease is the leading cause of death in kidney and simultaneous pancreas-kidney (SPK) transplant recipients. Assessing abdominal aortic calcification (AAC), using lateral spine x-rays and the Kaupilla 24-point AAC (0-24) score, may identify transplant recipients at higher CV risk. METHODS: Between the years 2000 and 2015, 413 kidney and 213 SPK first transplant recipients were scored for AAC at time of transplant and then followed for CV events (coronary heart, cerebrovascular, or peripheral vascular disease), graft-loss, and all-cause mortality. RESULTS: The mean age was 44 ± 12 years (SD) with 275 (44%) having AAC (26% moderate: 1-7 and 18% high: ≥8). After a median of 65 months (IQR 29-107 months), 46 recipients experienced CV events, 59 died, and 80 suffered graft loss. For each point increase in AAC, the unadjusted hazard ratios (HR) for CV events and mortality were 1.11 (95% CI 1.07-1.15) and 1.11 (1.08-1.15). These were similar after adjusting for age, gender, smoking, transplant type, dialysis vintage, and diabetes: aHR 1.07 (95% CI 1.02-1.12) and 1.09 (1.04-1.13). For recipients with high versus no AAC, the unadjusted and fully-adjusted HRs for CV events were 5.90 (2.90-12.02) and 3.51 (1.54-8.00), for deaths 5.39 (3.00-9.68) and 3.38 (1.71-6.70), and for graft loss 1.30 (0.75-2.28) and 1.94 (1.04-3.27) in age and smoking history-adjusted analyses. CONCLUSION: Kidney and SPK transplant recipients with high AAC have 3-fold higher CV and mortality risk and poorer graft outcomes than recipients without AAC. AAC scoring may be useful in assessing and targeted risk-lowering strategies.

Health-related quality of life following kidney and simultaneous pancreas kidney transplantation.

INTRODUCTION: Kidney and simultaneous pancreas kidney (SPK) transplant recipients are younger and fitter than most other dialysis patients, but are also more vulnerable in areas of social, emotional and physical interaction. Few studies have tracked their post-transplant health-related quality of life (HRQoL). AIM: To assess HRQoL following kidney and SPK transplantation, with comparison to dialysis patients, people with multiple co-morbidities and general population data. METHODS: Patients completed the Kidney Disease Quality of Life Short Form (KDQOL-SF™) 1.3 to assess their pre-transplant HRQoL within 4 weeks of transplantation and 12 months later. Demographic and laboratory data were collected on participating patients and on non-participating patients at both time-points. RESULTS: Of 118 patients who completed the baseline KDQOL-SF™, 75 (57 kidney and 18 SPK) completed the 1 year survey. Compared to baseline, 12 months HRQoL scores improved in all domains except for work status, exceeded those of patients on dialysis and, except for emotional wellbeing and mental health, exceeded the scores of people with multiple co-morbidities. For female transplant recipients, 12 months HRQoL scores were not statistically different from similarly aged women in the general population. Male transplant recipients had similar scores for bodily pain and energy/fatigue, but lower scores in other domains. Compared to kidney-only transplant recipients, SPK recipients achieved higher scores in work and sleep domains. CONCLUSION: Improvements in most HRQoL domains occur within 1 year of kidney or SPK transplantation, and women achieve similar HRQoL to women in the general population. These data are encouraging for patients contemplating transplant listing.

Cryoglobulinemic Glomerulonephritis Associated With Nodal and Renal Infiltration by T-Cell Lymphoma of T-Follicular Helper Phenotype: A Case Report.

We present a unique case of cryoglobulinemic glomerulonephritis associated with nodal and renal infiltration by T-cell lymphoma of T-follicular helper phenotype. The patient presented with transient neurologic symptoms, severe nephritic syndrome with nephrotic-range proteinuria, and acute kidney injury. He had elevated double-stranded DNA levels, low complement levels, detectable cryoglobulin, and detectable immunoglobulin M (IgM) paraprotein. The kidney biopsy showed cryoglobulinemic glomerulonephritis with a membranoproliferative pattern and diffuse interstitial infiltrates on light microscopy; IgM, C3 but weak IgG, C1q, and negative C4d staining on immunofluorescence; and deposits with organized substructures on electron microscopy. Positron emission tomography showed diffuse uptake in bilaterally enlarged kidneys and a localized group of lymph nodes. Subsequent lymph node biopsy revealed Epstein-Barr virus-negative nodal T-cell lymphoma, which was also proven in renal tissue. The association between T-cell lymphoma, autoantibodies, and cryoglobulinemia may represent a paraneoplastic phenomenon. His renal prognosis has been excellent, but overall prognosis and survival is dictated by the clinical course of T-cell lymphoma.

Efficacy of intraperitoneal calcium for hungry bone syndrome following parathyroidectomy: A case report.

A man with hyperparathyroidism secondary to kidney failure on peritoneal dialysis underwent a parathyroidectomy with half-gland reimplantation complicated by severe hungry bone syndrome resulting in severe hypocalcaemia, hypotension and QT prolongation on ECG. He was initially managed with oral calcium and intravenous (IV) calcium chloride. Despite standard supportive treatment, attempts to wean IV therapy were unsuccessful. We report the novel use of intraperitoneal calcium to facilitate the weaning of IV calcium and discharge from hospital. A subsequent peritoneal membrane adequacy study did not demonstrate loss of peritoneal membrane adequacy.

Health-related quality of life of patients awaiting kidney and simultaneous pancreas-kidney transplants.

AIM: Patients undergoing kidney and simultaneous pancreas-kidney (SPK) transplants are younger and fitter than the general dialysis population. Intuitively these patients might have better quality of life (QOL) than the general dialysis population, but their QOL scores are not well characterized. The aim of this study was to compare QOL of patients about to undergo kidney or SPK transplants with Australian dialysis outcomes and practice patterns (DOPPS) data and multiple comorbidity and age-adjusted general population data. METHODS: Patients attending Westmead Hospital for transplants from August 2009 to December 2011 were invited to complete the Kidney Disease QOL-SF(™) 1.3 (KDQOL-SF(™) 1.3) questionnaire regarding their immediate pretransplant QOL. This QOL instrument is predictive of hospitalizations and mortality. The questionnaire was completed within 4 weeks of transplantation. RESULTS: Of 180 patients seen within 4 weeks of transplantation 95 (53%) responded, with no differences from non-responders in age, sex, comorbidities or perioperative complications. Compared with DOPPS, these patients had better physical function and less pain, but significantly lower scores for role physical (CI: -19 to -4, P=0.004) and role emotional (CI: -17 to -2, P=0.018). Patients undergoing SPK transplants reported even poorer general health, energy, social support and function. Patients had lower emotional and social function than people with multiple comorbidities, with whom they shared poor general and mental health and vitality. Scores were markedly lower than the general population except for bodily pain (female). CONCLUSION: Younger, fitter patients are more vulnerable to effects of their illness on social, emotional and physical interactions and may benefit from targeted support.

Aortic vascular calcification is inversely associated with the trabecular bone score in patients receiving dialysis.

INTRODUCTION: Progressive chronic kidney disease (CKD) confers a marked increase in risk for vascular calcification, cardiovascular disease, fracture and mortality, with likely contributing factors including dysregulated bone metabolism and mineral homeostasis. In general population studies, increased vascular calcification is directly related to mortality and inversely related to bone mineral density (BMD) measured by dual-energy X-ray absorptiometry (DXA). In patients with CKD, abnormalities in turnover, mineralization and bone volume reduce the ability of DXA to predict fracture. The trabecular bone score (TBS) obtained from lumbar spine DXA images, provides a surrogate measure of microarchitectural integrity not captured by BMD. This study aimed to examine the association of the TBS to prevalent abdominal aortic calcification (AAC) in patients with CKD receiving dialysis. METHODS: We performed a cross-sectional study of dialysis patients awaiting transplantation. All patients underwent laboratory testing, lateral spinal radiographs including the abdominal aorta, DXA imaging and TBS assessment. AAC scores were determined using the Kauppila method. Correlations and linear regression models were used to determine predictors of AAC scores. RESULTS: 146 patients (60% male, mean age 48 ±â€¯13 years) were included, of whom 49% had prevalent calcification with an AAC score ≥ 1. Of those with calcification, the mean AAC score was 7 ±â€¯5.5 and 42 patients had scores ≥ 6, considered to indicate severe AAC. TBS values corresponding to intermediate or high risk for fracture (<1.31) were present in 35% of patients. TBS values correlated inversely to AAC scores (ß = -0.206, p = 0.013) and remained significant in multivariable linear regression, adjusting for age, BMI and time on dialysis (-0.160, p = 0.031). There was no significant correlation of AAC scores to any BMD parameter. CONCLUSION: There is a high prevalence of AAC in relatively young dialysis patients awaiting transplantation and their AAC scores are inversely related to the TBS but not to DXA-derived BMD parameters. In patients with CKD on dialysis, TBS assessment reflects microarchitectural abnormalities of bone not captured by DXA. The inverse relationship of TBS to vascular calcification may provide insights into bone-vascular interactions in CKD.