Incidence and consequences of varicella in children treated for cancer in Guatemala.

BACKGROUND: Varicella-zoster virus infection is associated with significant morbidity and mortality in immune-compromised children, despite treatment with antiviral agents. Universal varicella vaccine programs have significantly decreased this risk in many highincome countries, but in most low-income and middleincome countries, the burden of varicella in children treated for malignancy is poorly defined. METHODS: We retrospectively reviewed records of children at the National Unit of Pediatric Oncology (UNOP) in Guatemala diagnosed with varicella between January 2009 and March 2013 in order to calculate incidence of varicella and evaluate morbidity, mortality, treatment interruption, and cost. RESULTS: Fifty-nine cases of varicella were identified. Incidence was 23.4 cases per 1000 person-years (p-y). 66.1% of cases occurred in children with leukemia (median age 5.2 years; interquantile range 3.4-7 years) and 41.0% of these occurred during maintenance therapy. Source of exposure was identified for 14/59 (23.7%) children. Most were hospitalized (71.2%) and given intravenous acyclovir (64.4%). Eight (13.6%) children required critical care, and two (3.4%) died from disseminated varicella with multiorgan failure. Chemotherapy was delayed or omitted due to varicella in 50%. No significant differences in outcomes based on nutritional and immunologic status were detected. The minimum average cost of treatment per episode was 598.75 USD. CONCLUSIONS: Varicella is a significant problem in children treated for cancer in Guatemala, where effective post-exposure prophylaxis is limited. In the absence of universal varicella vaccination, strategies to improve recognition of exposure and the future use of novel inactivated vaccines currently under investigation in clinical trials could mitigate this burden.

Low awareness of nonalcoholic fatty liver disease among patients at high metabolic risk.

GOALS: To assess awareness of nonalcoholic fatty liver disease (NAFLD) as a disease entity among individuals with and without metabolic risk factors in an outpatient clinical setting, and to evaluate interest in patient-centered education on NAFLD. BACKGROUND: NAFLD is the most common chronic liver disease in the United States with up to 30% of the adult population affected. Individuals with metabolic risk factors, particularly, insulin resistance, diabetes, and overweight/obesity, have a high prevalence of NAFLD estimated up to 70%, yet little is known about the understanding and perceptions of NAFLD in these high-risk patients. STUDY: A self-administered paper questionnaire was given to 368 adult patients presenting to an outpatient endocrinology clinic from February 2012 to October 2012. RESULTS: A total of 302 surveys were completed for a response rate of 82%. Overall, 18% of all respondents reported awareness of NAFLD. Even among patients with self-reported major risk factors for NAFLD (overweight/obese, insulin resistant, or both overweight/obese and insulin resistant), the rates of awareness of NAFLD were low (19%, 23%, and 24%, respectively). A majority of survey respondents expressed interest in receiving patient-centered education on NAFLD (73%). CONCLUSIONS: Among high metabolic risk individuals there is low awareness of NAFLD. The majority of those surveyed expressed interest in learning about NAFLD. These findings suggest opportunities to raise public awareness of NAFLD, particularly among patients at high metabolic risk, and to provide education to high-risk individuals with the goal of implementing early prevention strategies and optimizing care.

The relationship between thyroid dose and diagnosis of primary hypothyroidism in pediatric brain tumor patients receiving craniospinal irradiation.

PURPOSE: The aim of this work is to determine if a relationship exists between thyroid dose and incidence of primary hypothyroidism (PH) in children undergoing craniospinal irradiation (CSI). METHODS: A total of 22 patients received CSI with evaluable thyroid dose information. All patients received concurrent chemotherapy and 21 patients (95%) received adjuvant chemotherapy. Median follow-up was 42.9 months. RESULTS: The incidence of PH in our cohort was 59% at a median time after radiotherapy of 3.5 years (range: 8 months to 7.5 years). Mean thyroid dose appeared to best predict for PH, with a median of 2080 cGy for patients with PH versus 1736 cGy for children without PH (p=0.057). There was no association between the rate of PH and sex, age, CSI dose, minimum thyroid dose and maximum thyroid dose. CONCLUSIONS: A relationship may exist between the mean thyroid dose and incidence of PH in patients undergoing CSI. Thus, new strategies to protect the thyroid gland may be warranted.

Respiratory and polysomnographic values in 3- to 5-year-old normal children at higher altitude.

STUDY OBJECTIVES: To determine polysomnographic parameter differences in children living at higher altitude to children living near sea level. DESIGN AND SETTING: Prospective study of non-snoring, normal children recruited from various communities around Denver, CO. In-lab, overnight polysomnograms were performed at a tertiary care children's hospital. All children required residence for greater than one year at an elevation around 1,600 meters. PARTICIPANTS: 45 children (62% female), aged 3-5 years, 88.9% non-Hispanic white with average BMI percentile for age of 47.8% ± 30.7%. MEASUREMENTS AND RESULTS: Standard sleep indices were obtained and compared to previously published normative values in a similar population living near sea level (SLG). In the altitude group (AG), the apnea-hypopnea index was 1.8 ± 1.2 and the central apnea-hypopnea index was 1.7 ± 1.1, as compared to 0.9 ± 0.8 and 0.8 ± 0.7, respectively, (P ≤ 0.005) in SLG. Mean end-tidal CO2 level in AG was 42.3 ± 3.0 mm Hg and 40.6 ± 4.6 mm Hg in SLG (P = 0.049). The ≥ 4% desaturation index was 3.9 ± 2.0 in AG compared to 0.3 ± 0.4 in SLG (P < 0.001). Mean periodic limb movement in series index was 10.1 ± 12.3 in AG and 3.6 ± 5.4 in SLG (P = 0.001). CONCLUSION: Comparison of altitude and sea level sleep studies in healthy children reveals significant differences in central apnea, apneahypopnea, desaturation, and periodic limb movement in series indices. Clinical providers should be aware of these differences when interpreting sleep studies and incorporate altitude-adjusted normative values in therapeutic-decision making algorithms.

Identifying practice gaps to optimize medical care for patients with nonalcoholic fatty liver disease.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is a leading cause of chronic liver disease. Primary care providers (PCPs), in contrast to gastroenterology/hepatology (GI/Hep) providers, are the first medical contact for the majority of patients with, or at risk for, NAFLD. PCP awareness of and facility with NAFLD is critical for management of these patients. AIM: The purpose of this study was to assess understanding and practice patterns of PCPs and non-GI/Hep subspecialty providers with respect to the diagnosis and management of NAFLD. METHODS: We administered an online, 61-question survey to 479 providers in internal medicine, family medicine, endocrinology, cardiology, and obstetrics and gynecology (ObGyn) across three health systems: academic medical center, safety-net health system and managed care health system. RESULTS: There were 246 respondents (51 %), with the majority (87 %) being PCPs (internal medicine, family medicine, ObGyn). Only 31 % of providers identified NAFLD as a clinically important diagnosis in their practice. Although 65 % of providers reported some degree of facility in diagnosing NAFLD, less than half (47 %) were comfortable managing NAFLD. Only 33 % refer patients with suspected NAFLD to GI/Hep. Subspecialists in endocrinology and cardiology reported greater clinical concern over NAFLD and were more likely (67 %) to refer patients with suspected NAFLD to GI/Hep. CONCLUSIONS: The majority of providers do not identify NAFLD as a clinically important diagnosis and do not refer to GI/Hep. However, 83 % expressed a need for education on NAFLD. Our data reveal practice gaps within NAFLD care and identify opportunities for targeted education to guide PCPs in the evaluation and management of NAFLD.

Prevalence of cardiovascular events in patients with autosomal dominant polycystic kidney disease.

BACKGROUND: This study evaluates the prevalence of cardiovascular events in autosomal dominant polycystic kidney disease (ADPKD) patients. METHODS: We distributed surveys to 1,439 subjects from our ADPKD research database. In total, 426 subjects completed and returned surveys; 7 of these were from children and were excluded from the study. RESULTS: The patients who responded were female (63.2%), nonHispanic (88.1%) and white (93.6%). The mean age of the total group was 53.2 ± 13.7 years; 82.8% had a family history of ADPKD and 32.5% had reached end-stage renal disease (ESRD). With respect to cardiovascular risk factors, 86.6% were hypertensive with a mean age at diagnosis of 36.9 ± 12.9 years and hypertension was significantly more prevalent in males. In addition, 19.6% of the subjects were obese, 20.8% were smokers, 8.7% had diabetes, 45.7% had high cholesterol and 17.8% were sedentary. The most prevalent self-reported cardiovascular events were arrhythmias (25.9%), evidence of peripheral vascular disease (16.5%), heart valve problems (14.4%), cardiac enlargement (9.5%), stroke or cerebral bleeding (7.5%), myocardial infarction (6%) and brain aneurysm (5.0%). The most commonly used antihypertensive medications were renin-angiotensin inhibitors used by 75% of ADPKD patients. Older ADPKD patients and those at ESRD had a significantly higher incidence of cardiovascular events. CONCLUSION: These findings support the high prevalence of cardiovascular risk factors and events in ADPKD patients which contribute to a greater mortality risk. Due to the prevalence of cardiovascular risk factors in the ADPKD population, early diagnosis and clinical intervention are recommended.

Exercise for people in early- or mid-stage Parkinson disease: a 16-month randomized controlled trial.

BACKGROUND: Exercise confers short-term benefits for individuals with Parkinson disease (PD). OBJECTIVE: The purpose of the study was to compare short- and long-term responses among 2 supervised exercise programs and a home-based control exercise program. DESIGN: The 16-month randomized controlled exercise intervention investigated 3 exercise approaches: flexibility/balance/function exercise (FBF), supervised aerobic exercise (AE), and home-based exercise (control). SETTING: This study was conducted in outpatient clinics. PATIENTS: The participants were 121 individuals with PD (Hoehn & Yahr stages 1-3). INTERVENTIONS: The FBF program (individualized spinal and extremity flexibility exercises followed by group balance/functional training) was supervised by a physical therapist. The AE program (using a treadmill, bike, or elliptical trainer) was supervised by an exercise trainer. Supervision was provided 3 days per week for 4 months, and then monthly (16 months total). The control group participants exercised at home using the National Parkinson Foundation Fitness Counts program, with 1 supervised, clinic-based group session per month. MEASUREMENTS: Outcomes, obtained by blinded assessors, were determined at 4, 10, and 16 months. The primary outcome measures were overall physical function (Continuous Scale-Physical Functional Performance [CS-PFP]), balance (Functional Reach Test [FRT]), and walking economy (oxygen uptake [mL/kg/min]). Secondary outcome measures were symptom severity (Unified Parkinson's Disease Rating Scale [UPDRS] activities of daily living [ADL] and motor subscales) and quality of life (39-item Parkinson's Disease Quality of Life Scale [PDQ-39]). RESULTS: Of the 121 participants, 86.8%, 82.6%, and 79.3% completed 4, 10, and 16 months, respectively, of the intervention. At 4 months, improvement in CS-PFP scores was greater in the FBF group than in the control group (mean difference=4.3, 95% confidence interval [CI]=1.2 to 7.3) and the AE group (mean difference=3.1, 95% CI=0.0 to 6.2). Balance was not different among groups at any time point. Walking economy improved in the AE group compared with the FBF group at 4 months (mean difference=-1.2, 95% CI=-1.9 to -0.5), 10 months (mean difference=-1.2, 95% CI=-1.9 to -0.5), and 16 months (mean difference=-1.7, 95% CI=-2.5 to -1.0). The only secondary outcome that showed significant differences was UPDRS ADL subscale scores: the FBF group performed better than the control group at 4 months (mean difference=-1.47, 95% CI=-2.79 to -0.15) and 16 months (mean difference=-1.95, 95% CI=-3.84 to -0.08). LIMITATIONS: Absence of a non-exercise control group was a limitation of the study. CONCLUSIONS: Findings demonstrated overall functional benefits at 4 months in the FBF group and improved walking economy (up to 16 months) in the AE group.