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Topic: Pediatric Nephrology. Background: Home hemodialysis (HD) is increasingly used in adults with lower all-cause mortality, morbidity and cost, and better blood pressure (BP) control compared to conventional HD or peritoneal dialysis (PD). The pediatric literature is sparse on this topic, but does support an improved quality of life and linear growth in addition to benefits reported in adults. However barriers to use include anxiety over the transfer of treatment burden and logistical concerns. We report here the youngest patient to date in the literature who received frequent home HD for 4 months until transplant with an improvement in school attendance, growth, and BP control and no complications. Methods: The patient had ARPKD with unilateral nephrectomy in infancy, had a living related donor (LRD) kidney transplant at 18 months, lost to rejection after 2 years. He was on PD from age 3.5-7, briefly on HD during the time of a cadaveric liver transplant at age 5. At age 7 the PD catheter eroded into his small bowel and he converted to in-center HD. He was fluid and nutrition restricted, and uncontrolled BP led to PRES. The family traveled 9 hours a week for in-center HD and he was on full homebound. On transfer to our medical care he was 8 years old and 22.5-kg, mildly wasted and moderately stunted, with BP >99th percentile. He was converted to a frequent home HD prescription with NxStage machine, 6 days a week, 2.5 hour treatments, QB 150- 200 ml/hr, RenaFlo II HF 400 dialyzer. Clearance was more than adequate and is represented in figure 1. Starting at week 1 of home HD, BP medications were weaned to PRN. He achieved adequate gain of weight 8.7 g/day and height 0.6 cm/month. By week 3 he was able to attend school 3-4 days per week for the first time in a year. During the 4 months on frequent home HD his nutrition and BP were optimized, and with antibody reduction successfully received a high-risk LRD kidney transplant. Conclusions: Our limited experience has shown that frequent home HD is safe and successful in the appropriate pediatric patient. It provided improved clearance and allowed school attendance, better BP control, and nutrition until transplant. Larger studies are needed to evaluate efficacy of frequent home versus incenter HD in pediatric patients. [ABSTRACT FROM AUTHOR]

Bioaerosol measurements were made in 45 Quad Cities, Illinois housesfollowing the 1993 floods of the Mississippi and Rock Rivers. Least squares log transformed means for fungal concentrations (in cfu m-3) were 1,449 in basements, 1,174 in living rooms, 1,234 in kitchens, 1,464 in first floor bedrooms, 1,197 in second floor bedrooms, and 2,590 outside. Mean least squares log transformed bacterial concentrations were 887 cfu m-3 in basements, 878 cfu m-3 in living rooms and 697 cfu m-3 outdoors. Levels of fungi and bacteria were not notably elevated in the basements or other rooms of the formerly flooded houses. Levels of fungi and bacteria indoors were significantly less in houses with dehumidifiers and in homes with natural gas odors. Air-conditioning during warm periods significantly reduced airborne concentrations of fungi. [ABSTRACT FROM AUTHOR]

Introduction: There is a paucity of information about risk behaviors in adolescents with chronic kidney disease (CKD). We designed this study to assess the prevalence of risk behaviors among teens with CKD in the United States and to investigate any associations between risk behavior and patient or disease characteristics., Methods: After informed consent, adolescents with CKD completed an anonymous, confidential, electronic web-based questionnaire to measure risk behaviors within five domains: sex, teen driving, alcohol and tobacco consumption, illicit drug use, and depression-related risk behavior. The reference group was composed of age-, gender-, and race-matched US high school students., Results: When compared with controls, teens with CKD showed significantly lower prevalence of risk behaviors, except for similar use of alcohol or illicit substances during sex (22.5% vs. 20.8%, p =0.71), feeling depressed for ≥2 weeks (24.3% vs. 29.1%, p =0.07), and suicide attempt resulting in injury needing medical attention (36.4% vs. 32.5%, p =0.78). Furthermore, the CKD group had low risk perception of cigarettes (28%), alcohol (34%), marijuana (50%), and illicit prescription drug (28%). Use of two or more substances was significantly associated with depression and suicidal attempts ( p < 0.05) among teens with CKD., Conclusions: Teens with CKD showed significantly lower prevalence of risk behaviors than controls. Certain patient characteristics were associated with increased risk behaviors among the CKD group. These data are somewhat reassuring, but children with CKD still need routine assessment of and counselling about risk behaviors., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2019 Nianzhou Xiao et al.)

Pheochromocytoma (PCC) and paraganglioma (PGL) are rare chromaffin cell tumors which secrete catecholamines and form part of the family of neuroendocrine tumors. Although a rare cause of secondary hypertension in pediatrics, the presentation of hypertension in these patients is characteristic, and treatment is definitive. The gold standard for diagnosis is via measurement of plasma free metanephrines, with imaging studies performed for localization, identification of metastatic lesions and for surgical resection. Preoperative therapy with alpha-blocking agents, beta blockers, and potentially tyrosine hydroxylase inhibitors aid in a safe pre-, intra- and postoperative course. PCC and PGL are inherited in as much as 80% of pediatric cases, and all patients with mutations should be followed closely given the risk of recurrence and malignancy. While the presentation of chromaffin cell tumors has been well described with multiple endocrine neoplasia, NF1, and Von Hippel-Lindau syndromes, the identification of new gene mutations leading to chromaffin cell tumors at a young age is changing the landscape of how clinicians approach such cases. The paraganglioma-pheochromocytoma syndromes (SDHx) comprise familial gene mutations, of which the SDHB gene mutation carries a high rate of malignancy. Since the inheritance rate of such tumors is higher than previously described, genetic screening is recommended in all patients, and lifelong follow-up for recurrent tumors is a must. A multidisciplinary team approach allows for optimal health-care delivery in such children. This review serves to provide an overview of pediatric PCC and PGL, including updates on the preferred methods of imaging, guidelines on gene testing as well as management of hypertension in such patients.

Asthma is a clinically heterogeneous genetic disease, and its pathogenesis is incompletely understood. Genome-wide association studies link ORM (yeast)-Like protein isoform 3 [corrected] (ORMDL3), a member of the ORM gene family, to nonallergic childhood-onset asthma. Orm proteins negatively regulate sphingolipid (SL) synthesis by acting as homeostatic regulators of serine palmitoyl-CoA transferase (SPT), the rate-limiting enzyme of de novo SL synthesis, but it is not known how SPT activity or SL synthesis is related to asthma. The present study analyzes the effect of decreased de novo SL synthesis in the lung on airway reactivity after administration of myriocin, an inhibitor of SPT, and in SPT heterozygous knockout mice. We show that, in both models, decreased de novo SL synthesis increases bronchial reactivity in the absence of inflammation. Decreased SPT activity affected intracellular magnesium homeostasis and altered the bronchial sensitivity to magnesium. This functionally links decreased de novo SL synthesis to asthma and so identifies this metabolic pathway as a potential target for therapeutic interventions.