Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection

Heart transplantation represents a valuable therapeutical option for patients with congenital heart disease and end-stage heart failure. We report the case of a young adult patient with a situs inversus and additional complex congenital malformations of the heart who underwent several prior palliative interventions, a biventricular repair being impossible. Orthotopic cardiac transplantation with several technical modifications was performed successfully at the age of 19 years.     

Asymptomatic renal infarction, due to fibromuscular dysplasia, in a young woman with 11 years of follow-up

We report a 27-year-old woman with renovascular hypertension, renal infarction, and hepatic artery aneurysm due to fibromuscular dysplasia. The patient was first noted to have renal artery aneurysm and hepatic artery aneurysm at the age of 17. The renal infarction was asymptomatic and was incidentally detected by magnetic resonance imaging (MRI) examination. Because of the rather peripheral location of the aneurysms, percutaneous transluminal renal artery angioplasty was considered inappropriate. This case suggests the need for long-term and periodical follow-up of patients with fibromuscular dysplasia.     

Metabolic syndrome and the development of chronic kidney disease among 118 924 non-diabetic Taiwanese in a retrospective cohort

Aim:   Metabolic syndrome (MetS) is a common risk factor for cardiovascular and chronic kidney disease (CKD) in Western populations; however, no prospective studies have examined MetS as a risk factor for CKD in Chinese adults.
Methods:   The incidence of CKD and the prospective link between MetS (defined by two criteria: modified Adult Treatment Panel III (ATP-III) and the International Diabetes Federation (IDF)) and CKD among 118 924 Taiwanese participants without baseline diabetes, aged 20–74 years with a mean 3.7 years follow up, was examined. CKD was measured by using estimated glomerular filtration rate or dipstick proteinuria (1+). The association between MetS or combination patterns of MetS abnormalities and CKD was evaluated using Cox models with adjustment for confounders.
Results:   The incidence of CKD was 288/10 000 person-years (95% confidence interval (CI), 283–293). The findings showed that central obesity (OB), high blood pressure (BP) and high triglyceride were considered to be the major metabolic events in the study cohort. Incidences and hazard ratios (HR) on CKD had evidently increasing trends with the number of MetS components. The multivariable-adjusted HR for CKD associated with ATP-III-MetS was 1.30 (95% CI, 1.24–1.36). Equivalent HR for IDF-MetS were 1.37 (95% CI, 1.30–1.44). The associations were still observed when analyzing by stratifying incident diabetes and adjusting hypertension status.
Conclusion:   MetS induces an increased risk for CKD independent of baseline confounding factors and subsequent incident diabetes modified the associations lightly. The mechanism through which MetS may cause CKD in this population likely is the development of multiple metabolic pathogenic processes together.     

Automated peritoneal dialysis as the modality of choice: a single-center, 3-year experience with 458 children in Mexico

Automated peritoneal dialysis (APD) has been considered as the ideal dialysis modality for pediatric patients. This study reports the 3-year APD experience with 458 end-stage renal disease (ESRD) children who started APD in a single pediatric center in Mexico City between June 2003 and June 2006. By June 2003, there were 310 patients being treated with continuous ambulatory peritoneal dialysis (CAPD). At that time, these patients were gradually switched to APD, with priority being given to those prescribed more than four exchanges per day, younger than 6 years of age, or presenting complications [hernias or decreased ultrafiltration (UF)]. An improvement of daily UF was observed when the patients were switched from CAPD (590 ± 340 ml/day) to APD (846 ± 335 ml/day). The presence of edema decreased (from 67% to 8%) as well as the percentage of patients requiring antihypertensive drugs (from 83% to 38%), the peritonitis rate improved from one episode every 35 patient/month to one episode every 47 patient/month, the total number of hospitalizations decreased (from 384 to 51), and 85% of children attended school. While waiting for renal transplant, APD is the dialysis modality of choice for ESRD children at the La Raza Medical Center in Mexico City. Dr. Divino Filho is an employee of Baxter Health Care and holds stock in the company.     

A Phase I/II Randomized Open-Label Multicenter Trial of Efalizumab, a Humanized Anti-CD11a, Anti-LFA-1 in Renal Transplantation

Leukocyte function associated antigen-1 (LFA-1) has a multifaceted role in the immune response, including adhesion and trafficking of leukocytes, stabilizing the immune synapse of the MHC-TCR complex and providing costimulation signals. Monoclonal antibodies to the CD11a chain of LFA-1 have been seen to result in effective immunosuppression in experimental models. Efalizumab, a humanized IgG1 anti-CD11a, is approved for use in psoriasis and may provide effective immunosuppression in organ transplantation. Thirty-eight patients undergoing their first living donor or deceased renal transplant were randomized to receive efalizumab 0.5 or 2 mg/kg weekly subcutaneously for 12 weeks. Patients were maintained on full dose cyclosporine, mycophenolate mofetil and steroids or half dose cyclosporine, sirolimus and prednisone. At 6 months following transplant patient survival was 97% and graft survival was 95%. Clinical biopsy-proven acute rejection in the first 6 months after transplantation was confirmed in 4 of 38 patients (11%). Three patients (8%) developed post transplant lymphoproliferative disease, all treated with the higher dose efalizumab and full dose cyclosporine. The two doses of efalizumab resulted in comparable saturation and modulation of CD11a. This phase II trial suggests that efalizumab may warrant further investigation in transplantation.     

Placental growth factor and placental protein 13 in patients with Balkan endemic nephropathy,a worldwide disease

Abstract

Background: Balkan endemic nephropathy (BEN) is a chronic tubulointerstitial kidney disease occurring in people living in along the tributaries of the Danube River. The aim of the study was to determine serum level and urinary excretion of placental growth factor (PlGF) and placental protein 13 (PP13) in patients with BEN. Methods: Thirty patients with BEN from the South Morava River region of Serbia and 18 controls were studied. Age of patients was 74?yr (53–87) and 73?yr (66–83) in controls. Results: In patients with BEN, serum creatinine was significantly higher than in controls (129.7 vs. 83.2?µmol/L, respectively), but GFR was lower in patients than in controls (40.7 vs. 54.6?mL/min). Serum PlGF was significantly higher in BEN patients than in controls (9.90 vs. 6.80?pg/mL), urinary excretion being significantly lower in patients (0.20 vs. 0.90?pg/mmol creat.). Serum PP13 was significantly lower in BEN patients (208.2 vs. 291.0?pg/mL). Urinary excretion of PP13 was also significantly lower in BEN patients than in controls (32.5 vs. 182.5?pg/mmol creat). In multivariate regression analysis BEN, sex and age were significant determinants of the observed changes in PlGF and PP13. Conclusion: Important changes of PlGF and PP13 in patients with BEN were demonstrated, where kidney disease, female sex, and the age have been significant determinants.     

Antiidiotypic PTH antibodies as a cause of elevated immunoreactive parathyroid hormone in idiopathic hypoparathyroidism,a second case: Another manifestation of autoimmune endocrine disease?

Summary A 69-year-old man became hypocalcemic under medical observation. The hypocalcemia occurred in the presence of circulating immunoreactive parathyroid hormone (PTH). Common causes of secondary hyperparathyroidism were excluded, as was PTH resistance using PTH infusions. The immunoreactive PTH was examined in detail. PTH immunoreactivity (1) was not retained on a C₁₈ SPE-column, suggesting unusual molecular or physicochemical properties, unlike bona fide PTH; (2) was precipitated with 15% PEG, indicating a molecular size far in excess of native PTH; (3) had an apparent molecular size similar to immunoglobulins on size exclusion chromatography; (4) was retained on affinity chromatography with both Protein A and anti-hlgG antibodies. These data lead us to conclude that the immunoreactive PTH was due to antiidiotypic PTH autoantibodies. No significant quantities of true PTH were found in the patient's serum suggesting that his hypoparathyroidism was a result of PTH deficiency. Autoimmunity might explain the occurrence of both processes if an arrested antiidiotypic antibody cascade is assumed.     

Acute West Nile Virus Meningoencephalitis Diagnosed Via Metagenomic Deep Sequencing of Cerebrospinal Fluid in a Renal Transplant Patient

Solid organ transplant patients are vulnerable to suffering neurologic complications from a wide array of viral infections and can be sentinels in the population who are first to get serious complications from emerging infections like the recent waves of arboviruses, including West Nile virus, Chikungunya virus, Zika virus, and Dengue virus. The diverse and rapidly changing landscape of possible causes of viral encephalitis poses great challenges for traditional candidate‐based infectious disease diagnostics that already fail to identify a causative pathogen in approximately 50% of encephalitis cases. We present the case of a 14‐year‐old girl on immunosuppression for a renal transplant who presented with acute meningoencephalitis. Traditional diagnostics failed to identify an etiology. RNA extracted from her cerebrospinal fluid was subjected to unbiased metagenomic deep sequencing, enhanced with the use of a Cas9‐based technique for host depletion. This analysis identified West Nile virus (WNV). Convalescent serum serologies subsequently confirmed WNV seroconversion. These results support a clear clinical role for metagenomic deep sequencing in the setting of suspected viral encephalitis, especially in the context of the high‐risk transplant patient population.