Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations.

Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection and monitoring of hepatobiliary complications in CF were published in 1999. The CF Foundation assembled a committee to evaluate research advances and formulate revised guidelines for CF-associated liver disease. A committee of hepatologists, gastroenterologists, pulmonologists, pharmacists, nurses, dietitians, individuals with CF, and the parents of a child with CF devised "population, intervention, comparison, and outcome" questions regarding hepatobiliary disease in CF. PubMed literature searches were performed for each population, intervention, comparison, and outcome question. Recommendations were voted on with 80% agreement required to approve a recommendation. Public comment on initial recommendations was solicited prior to the formulation of final recommendations. Thirty-one population, intervention, comparison, and outcome questions were assembled, 6401 manuscripts were title screened for relevance, with 1053 manuscripts undergoing detailed full-text review. Seven recommendations were approved for screening, 13 for monitoring of existing disease, and 14 for treatment of CF-associated hepatobiliary involvement or advanced liver disease. One recommendation on liver biopsy did not meet the 80% threshold. One recommendation on screening ultrasound was revised and re-voted on. Through a multidisciplinary committee and public engagement, we have assembled updated recommendations and guidance on screening, monitoring, and treatment of CF-associated hepatobiliary involvement and advanced liver disease. While research gaps remain, we anticipate that these recommendations will lead to improvements in CF outcomes through earlier detection and increased evidence-based approaches to monitoring and treatment.

Monitoreo continuo de glucosa durante la gestación en pacientes con diabetes mellitus tipo 2 pregestacional

La diabetes pregestacional requiere un control glicémico estricto durante el embarazo. Los dispositivos de monitoreo continuo de glucosa (MCG) miden niveles de glucosa intersticial sin necesidad de punción capilar. Se estudió 4 gestantes con diabetes mellitus tipo 2 pregestacional con la ayuda del MCG durante 2 semanas de su gestación. Ellas tuvieron sesiones nutricionales semanales y controles médicos con un endocrinólogo. El promedio de nivel de glucosa osciló entre 82 y 171 mg/dL. El MCG permitió cambios tempranos en el tratamiento de una paciente con hipoglicemia. Todas las pacientes manifestaron que el MCG les ayudó en la selección de sus alimentos. En conclusión, el MCG ayudó en el reconocimiento de carbohidratos y en el reajuste del tratamiento. El MCG tuvo buena aceptación de su uso.

Pregestational diabetes requires strict glycemic control during pregnancy. Continuous glucose monitoring (CGM) devices measure interstitial glucose levels without the need for capillary puncture. Four pregnant women with pregestational type 2 diabetes mellitus were studied with the aid of CGM during 2 weeks of their gestation. They had weekly nutritional sessions and medical controls with an endocrinologist. The average glucose level ranged from 82 to 171 mg/dL. The CGM allowed early changes in the treatment of one patient with hypoglycemia. All patients stated that the GCM helped them in their food selection. In conclusion, the GCM helped in carbohydrate recognition and treatment readjustment. The CGM was well accepted for use.

Assessment of Household Food Insecurity During a Medical Mission to Chincha, Peru.

Introduction Food insecurity directly influences health outcomes and is an important consideration for medical missions seeking to address chronic disease, particularly those serving disaster-prone communities. The region of Peru in which we held an inaugural mission is vulnerable to developing food insecurity following natural disasters. We, therefore, sought to evaluate food insecurity to understand the community's needs and inform future public health efforts. Methods In this cross-sectional pilot study, a convenience sample representing the households of patients attending a student-run health fair at the community medical center in Chincha, Peru was assessed for food insecurity. An adult female (n = 30) of each randomly selected family attending the fair was asked to complete the Household Food Security Survey (HFSS) developed by the US Department of Agriculture. The survey items were aggregated into a single, continuous food security scale reflecting the severity of hunger within a household. Results Two-thirds of respondents (n = 20) acknowledged anxiety about having enough food at home over the past 12 months, making it the most common concern. Nearly three in five respondents were concerned about their ability to provide a balanced diet. We found that 16.7% of all households were food insecure with severe hunger, 26.7% were food insecure with moderate hunger, 30% were food insecure without hunger, and 26.7% were food secure. Conclusion Nearly three-quarters of families attending our clinic experience some degree of food insecurity. Families with children were disproportionately affected. The high levels of food insecurity many years after a natural disaster support the development of future social programs such as food pantries. We intend to continue our partnership in Chincha and perform the HFSS survey on a periodic basis to monitor hunger.

Hepatic enzyme ALT as a marker of glucose abnormality in men with cystic fibrosis.

AIM: Cystic fibrosis (CF) patients are at high risk of developing CF-related diabetes (CFRD). In non-CF patients, liver disease, specifically steatosis and non-alcoholic fatty liver disease (NAFLD), is strongly associated with type 2 diabetes. We compared glycemic status and metabolic profiles in CF patients according to a biomarker of hepatic injury, alanine aminotransferase (ALT). METHODS: We conducted a cross-sectional study among 273 adult CF patients recruited from the Montreal CF Cohort. A 2-hour oral glucose tolerance test (OGTT) was performed to collect glucose and insulin measures every 30 minutes. Fasting ALT levels and anthropometric measures were also obtained. Patients were categorized into 2 groups based on ALT cut-off of 25 U/L. RESULTS: Patients in the high ALT group were mostly men (83%), had higher mean weight and BMI (p<0.001) and showed elevated glucose levels throughout OGTT (p≤0.01). When stratified by sex, only men with high ALT showed significantly higher weight (p<0.001), higher glycemic values at 60, 90 and 120 minutes of OGTT (p≤0.01), higher frequency of de novo CFRD (20.5% vs 8.2%, p = 0.04) as well as lower insulin sensitivity than men with normal ALT (p = 0.03). ALT levels were strongly associated with HOMA-IR in CFRD patients (p = 0.001, r2 = 0.28). CONCLUSIONS: Adult CF men with higher ALT show an increased frequency of dysglycemia and de novo CFRD, lower insulin sensitivity and higher eight. Our data suggests that ALT levels could be an interesting tool to guide targeted diabetes screening, particularly among CF men. Prospective studies are needed to confirm these observations.

The Yin and Yang Between Plasma Glucose Levels and Cortisol Replacement Therapy in Schmidt's Syndrome.

Objective: To illustrate how steroid replacement in adrenal insufficiency can influence the development of hypoglycemia in a patient with type 1 diabetes mellitus (T1D). Methods: We describe the case of a 36-year-old female patient with T1D and Addison's disease (Schmidt's syndrome) on multiple daily insulin injections who presented with recurrent hypoglycemia despite being on physiological replacement doses of hydrocortisone. Results: With the assistance of continuous glucose monitoring technology, a pattern of nocturnal hypoglycemia was clearly identified. The patient was taking her hydrocortisone 15 mg in the morning and 5 mg in the early afternoon. With the short half-life of oral hydrocortisone, the evening decline in plasma cortisol concentration led to an increased susceptibility to recurrent evening and nocturnal hypoglycemia. Hypoglycemic episodes were resolved when her morning hydrocortisone dose was changed and prednisolone was added to a later time in the evening. Conclusion: Patients with Schmidt's syndrome can be susceptible to nocturnal hypoglycemia with inadequate steroid replacement. Identifying patients at risk for hypoglycemia in Schmidt's syndrome provides an opportunity for precision management beyond the manipulation of antihyperglycemic agents.

Cost-effectiveness of genotype-guided warfarin therapy for anticoagulation in elderly patients with atrial fibrillation.

BACKGROUND: In patients with atrial fibrillation (AF), anticoagulation with warfarin decreases the risk of embolic stroke by >50%. Identification of genetic polymorphisms in enzymes involved in the metabolism of warfarin can partially predict the maintenance dose and thus potentially decrease the incidence of bleeding episodes secondary to warfarin overdose. OBJECTIVES: The objectives of this study were to evaluate the potential clinical and economic outcomes of genotype-guided warfarin therapy in elderly patients newly diagnosed with AF and to identify a threshold in bleeding risk at which such therapy may be cost-effective. METHODS: A decision tree was designed to represent the medical decision (pharmacogenetic testing or not) and the main clinical outcomes (embolic stroke, bleeding). Event rates of embolic stroke and bleeding complications were based on data from previously published clinical trials and an observational study, respectively; costs were from a third-party payer perspective; and utilities were from the patient perspective. It was assumed that use of pharma-cogenetic testing would not lead the clinician to make any potentially harmful modifications to the regimen. RESULTS: This analysis found that any reduction in major bleeding as a result of pharmacogenetic testing would lead to improved utility. The higher costs of pharmacogenetic testing compared with no testing would be immediately offset by any reduction in major bleeding. CONCLUSIONS: In this decision analysis, genotype-guided warfarin therapy for anticoagulation in elderly patients with AF was potentially cost-effective, and its benefits were closely related to efficacy in preventing bleeding events. Clinical trials testing the efficacy of genotype-guided warfarin therapy are warranted.

Diátesis atópica: prevalencia en escolares de Santiago de Surco / Atopic diatesis: prevalence in schoolchildren of Santiago de Surco

Entre los meses de marzo y julio de 1995 se llevó a cabo un estudio epidemiológico, de corte transversal en el distrito de Santiago de Surco, Lima con el objeto de determinar la prevalencia de dermatitis atópica (DA) en escolares de 13 y 14 años. Para lograr esto se realizó un muestreo aleatorio simple de la población en estudio, conformada por 7,300 niños. Se obtuvo una muestra de 3,270 escolares, quienes respondieron de manera directa a un cuestionario ISAAC (International Study of Asthma and Allergies in Childhood). La tasa de respuesta fue del 97.4 por ciento con un nivel de inconsistencia que variaba entre 0.6 por ciento y 2.3 por ciento. Se encontró que 14.9 por ciento reportaron la presencia de síntomas sugerentes de DA alguna vez en la vida, y de éstos 60.1 por ciento reportaron haberlos presentado durante el último año. El 16.1 por ciento de los niños encuestados afirmaron haber tenido diagnóstico de eczema, encontrándose una asociación estadística altamente significativa entre este último y el reporte de síntomas (p menor 0.001). Finalmente se encontró una prevalencia de DA de 7.9 por ciento: Concluimos que la DA es una patología frecuente en la población estudiada, siendo necesario nuevos estudios similares que permitan conocer la repercusión de esta enfermedad en nuestra población