Successful Treatment of Bilateral Renal Mucormycosis With Isavuconazole: A Case Report.

Isolated renal mucormycosis (IRM) is a rare disease with high mortality, more commonly seen in immunocompromised patients. Management has traditionally included antifungal drugs with or without nephrectomy. We present the case of a 34-year-old female with a past medical history of type 1 diabetes mellitus and intravenous heroin use who presented with fever, flank pain, hematuria, and vomiting. She was found to have an oliguric acute kidney injury (AKI) with a serum creatinine (Cr) of 2.5 mg/dL. CT showed bilateral emphysematous pyelonephritis and ureteral cultures grew Rhizopus species. Amphotericin B was started before being switched to isavuconazole due to worsening AKI, and hemodialysis was only required transiently. Rather than the traditional approach to treatment, a conservative approach that preserved kidney function was utilized, and the patient was successfully treated with six months of isavuconazole.

Late-onset Fabry disease: the cardiac sequela.

We describe a patient with Fabry disease (FD) who initially presented with atrial fibrillation without left ventricular hypertrophy (LVH) 14 years before being correctly diagnosed with FD. In the interim, he survived a myocardial infarction complicated by ventricular fibrillation, and his severe LVH was misdiagnosed as sarcomeric hypertrophic cardiomyopathy. In the following 4 years, he developed proteinuric kidney disease, neuropathy, sensorineural hearing loss and gastrointestinal symptoms. The patient was eventually readmitted for an overt heart failure (HF) exacerbation and was seen by an HF cardiologist. The constellation of systemic findings led to further diagnostic testing, including an endomyocardial biopsy, tests to determine alpha-galactosidase A enzyme activity and α-galactosidase A gene (GLA) analysis. The results of the patient's tests were consistent with FD and he was started on enzyme replacement therapy. To our knowledge, this is the first detailed description of a late-onset phenotype of FD with c.146 G>C GLA variant. In addition, this case serves as a potent reminder to pay meticulous attention to 'red flags' accompanying LVH.

Improving Primary Care Delivery for Patients Receiving Maintenance Hemodialysis.

The beneficial impact of primary care, focused on all aspects of a patient's health (rather than a disease-specific focus) is well established. Recognized benefits include greater receipt of preventive care and counseling, lower use of emergency care and hospitalization for ambulatory care-sensitive conditions, and decreased early mortality. Although the importance of primary care and care coordination at the primary care/specialty interface is well recognized, the role of primary care within traditional and emerging care models for patients receiving in-center maintenance hemodialysis remains ill-defined. In this perspective article, we will describe: (1) the role of primary care for patients receiving maintenance hemodialysis and the current evidence regarding the receipt of primary care among these patients; (2) the key challenges to delivery of primary care in these complex cases, including suboptimal care coordination between nephrology and primary care providers, the intensity of dialysis care, and the limited capacity of nephrologists and primary care providers to meet the broad health needs of hemodialysis patients; (3) potential strategies for improving the delivery of primary care for patients receiving hemodialysis; and (4) future research requirements to improve primary care delivery for this high-risk population.

Effect of intensive blood pressure on the progression of non-diabetic chronic kidney disease at varying degrees of proteinuria.

The ideal blood pressure (BP) target for renoprotection is uncertain in patients with non-diabetic chronic kidney disease (CKD), especially considering the influence exerted by pre-existing proteinuria. In this pooled analysis of landmark trials, we coalesced individual data from 5001 such subjects randomized to intensive versus standard BP targets. We employed multivariable regression to evaluate the relationship between follow-up systolic blood pressure (SBP) and diastolic blood pressure (DBP) on CKD progression (defined as glomerular filtration rate decline by 50% or end-stage renal disease), focusing on the potential for effect modification by baseline proteinuria or albuminuria. The median follow-up was 3.2 years. We found that SBP rather than DBP was the primary predictor of renal outcomes. The optimal SBP target was 110-129 mm Hg. We observed a strong interaction between SBP and proteinuria such that lower SBP ranges were significantly linked with progressively lower CKD risk in grade A3 albuminuria or ≥0.5-1 g/day proteinuria (relative to SBP 110-119 mm Hg, the adjusted HR for SBP 120-129 mm Hg, 130-139 mm Hg, and 140-149 mm Hg was 1.5, 2.3, and 3.3, respectively; all p<0.05). In grade A2 microalbuminuria or proteinuria near 0.5 g/day, a non-significant but possible connection was seen between tighter BP and decreased CKD (aforementioned HRs all <2; all p>0.05), while in grade A1 albuminuria or proteinuria <0.2 g/day no significant association was apparent (HRs all <1.5; all p>0.1). We conclude that in non-diabetic CKD, stricter BP targets <130 mm Hg may help limit CKD progression as proteinuria rises.

Kidney Physiology in Pregnancy.

A woman's body undergoes a myriad of changes throughout the course of a normal gestation. The kidneys play a central role in driving adjustments that guarantee maternal and fetal well-being, including a dramatic increase in glomerular filtration rate, alterations in tubular function, and changes in electrolyte and acid/base handling. Early in gestation, systemic vasodilation, driven by both a change in quantity of and response to various hormones, leads to increased renal blood flow and glomerular filtration rate. Vasodilation also results in activation of the renin-angiotensin-aldosterone axis, which combined with changing tubular handling causes alterations in total body stores of electrolytes and total body water, resulting in a lower serum sodium concentration. In addition, mild proteinuria, glucosuria, and a decrease in serum calcium and magnesium are common. The primary acid/base change seen in pregnancy is a mild respiratory alkalosis due to progesterone effects. This article provides an overview of the current understanding of the healthy response of the kidneys to pregnancy, an understanding of which is key to caring for the pregnant patient, and early identification of alterations that may indicate underlying kidney pathology in pregnancy.

Racial and Ethnic Disparities in Pregnancy-Related Acute Kidney Injury.

Background: Pregnancy-related AKI (PR-AKI) is increasing in the United States. PR-AKI is associated with adverse maternal outcomes. Disparities in racial/ethnic differences in PR-AKI by race have not been studied. Methods: This was a retrospective cohort study using the National Inpatient Sample (NIS) from 2005 to 2015. We identified patients who were admitted for a pregnancy-related diagnosis using the Neomat variable provided by the NIS database that indicates the presence of a maternal or neonatal diagnosis code or procedure code. PR-AKI was identified using ICD codes. Survey logistic regression was used for multivariable analysis adjusting for age, medical comorbidities, socioeconomic factors, and hospital/admission factors. Results: From 48,316,430 maternal hospitalizations, 34,001 (0.07%) were complicated by PR-AKI. Hospitalizations for PR-AKI increased from 3.5/10,000 hospitalizations in 2005 to 11.8/10,000 hospitalizations in 2015 with the largest increase seen in patients aged ≥35 and black patients. PR-AKI was associated with higher odds of miscarriage (adjusted odds ratio [aOR], 1.64; 95% CI, 1.34 to 2.07) and mortality (aOR, 1.53; 95% CI, 1.25 to 1.88). After adjustment for age, medical comorbidities, and socioeconomic factors, blacks were more likely than whites to develop PR-AKI (aOR, 1.17; 95% CI, 1.04 to 1.33). On subgroup analyses in hospitalizations of patients with PR-AKI, blacks and Hispanics were more likely to have preeclampsia/eclampsia compared with whites (aOR, 1.29; 95% CI, 1.01 to 1.65; and aOR, 1.69; 95% CI, 1.23 to 2.31, respectively). Increased odds of mortality in PR-AKI compared with whites were only seen in black patients (aOR, 1.61; 95% CI, 1.02 to 2.55). Conclusions: The incidence of PR-AKI has increased and the largest increase was seen in older patients and black patients. PR-AKI is associated with miscarriages, adverse discharge from hospital, and mortality. Black and Hispanic patients with PR-AKI were more likely to have adverse outcomes than white patients. Further research is needed to identify factors contributing to these discrepancies.

Natural language processing of electronic health records is superior to billing codes to identify symptom burden in hemodialysis patients.

Symptoms are common in patients on maintenance hemodialysis but identification is challenging. New informatics approaches including natural language processing (NLP) can be utilized to identify symptoms from narrative clinical documentation. Here we utilized NLP to identify seven patient symptoms from notes of maintenance hemodialysis patients of the BioMe Biobank and validated our findings using a separate cohort and the MIMIC-III database. NLP performance was compared for symptom detection with International Classification of Diseases (ICD)-9/10 codes and the performance of both methods were validated against manual chart review. From 1034 and 519 hemodialysis patients within BioMe and MIMIC-III databases, respectively, the most frequently identified symptoms by NLP were fatigue, pain, and nausea/vomiting. In BioMe, sensitivity for NLP (0.85 - 0.99) was higher than for ICD codes (0.09 - 0.59) for all symptoms with similar results in the BioMe validation cohort and MIMIC-III. ICD codes were significantly more specific for nausea/vomiting in BioMe and more specific for fatigue, depression, and pain in the MIMIC-III database. A majority of patients in both cohorts had four or more symptoms. Patients with more symptoms identified by NLP, ICD, and chart review had more clinical encounters. NLP had higher specificity in inpatient notes but higher sensitivity in outpatient notes and performed similarly across pain severity subgroups. Thus, NLP had higher sensitivity compared to ICD codes for identification of seven common hemodialysis-related symptoms, with comparable specificity between the two methods. Hence, NLP may be useful for the high-throughput identification of patient-centered outcomes when using electronic health records.