Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative

Patients with rare and complex diseases such as congenital adrenal hyperplasia (CAH) often receive fragmented and inadequate care unless efforts are coordinated among providers. Translating the concepts of the medical home and comprehensive health care for individuals with CAH offers many benefits for the affected individuals and their families. This manuscript represents the recommendations of a 1.5 day meeting held in September 2009 to discuss the ideal goals for comprehensive care centers for newborns, infants, children, adolescents, and adults with CAH. Participants included pediatric endocrinologists, internal medicine and reproductive endocrinologists, pediatric urologists, pediatric surgeons, psychologists, and pediatric endocrine nurse educators. One unique aspect of this meeting was the active participation of individuals personally affected by CAH as patients or parents of patients. Representatives of Health Research and Services Administration (HRSA), New York-Mid-Atlantic Consortium for Genetics and Newborn Screening Services (NYMAC), and National Newborn Screening and Genetics Resource Center (NNSGRC) also participated. Thus, this document should serve as a "roadmap" for the development phases of comprehensive care centers (CCC) for individuals and families affected by CAH.

Risk factors for inadequate prenatal care use in the metropolitan area of Aracaju, Northeast Brazil

Abstract Background The aim of prenatal care is to promote good maternal and foetal health and to identify risk factors for adverse pregnancy outcomes in an attempt to promptly manage and solve them. Although high prenatal care attendance is reported in most areas in Brazil, perinatal and neonatal mortalities are disproportionally high, raising doubts about the quality and performance of the care provided. The objective of the present study was to evaluate the adequacy of prenatal care use and the risk factors involved in inadequate prenatal care utilization in the metropolitan area of Aracaju, Northeast Brazil. Methods A survey was carried out with puerperal women who delivered singleton liveborns in all four maternity hospitals of Aracaju. A total of 4552 singleton liveborns were studied. The Adequacy of Prenatal Care Utilization Index, modified according to the guidelines of the Prenatal Care and Birth Humanization Programme, was applied. Socioeconomic, demographic, biological, life style and health service factors were evaluated by multiple logistic regression. Results: Prenatal care coverage in Aracaju was high (98.3%), with a mean number of 6.24 visits. Prenatal care was considered to be adequate or intensive in 66.1% of cases, while 33.9% were considered to have inadequate usage. Age < 18 to 34 years at delivery, low maternal schooling, low family income, two or more previous deliveries, maternal smoking during pregnancy, having no partner and prenatal care obtained outside Aracaju were associated with inadequate prenatal care use. In contrast, private service attendance protected from inadequate prenatal care use. Conclusion Prenatal care coverage was high. However, a significant number of women still had inadequate prenatal care use. Socioeconomic inequalities, demographic factors and behavioural risk factors are still important factors associated with inadequate prenatal care use.

Weight and neuro-psycho-motor development in children discharged from neonatal intensive care unit

Background: Low birth weight affects child growth and development, requiring the intensive use of health services. There are conversely proportional associations between prematurity and academic performance around the world. In this study we evaluated factors involved in weight and neuropsychomotor profile in one and two years old discharged from Intensive Care Units (ICU). Methods/Design: We investigated 203 children from the ICU who were followed for 24 +/- 4 months. The research was conducted by collecting data from medical records of patients in a Follow-up program. We investigated the following variables: inadequate weight at one year old; inadequate weight at two years old and a severe neurological disorder at two years old. Results: We observed increase of almost 20% in the proportion of children which weighted between the 10th and 90th percentiles and decrease of around 40% of children below the 15th percentile, from one to two years old. In almost 60% of the cases neuropsychomotor development was normal at 2 years old, less than 15% of children presented abnormal development. Variables that remained influential for clinical outcome at 1 and 2 years old were related to birth weight and gestational age, except for hypoglycemia. Neurological examination was the most influential variable for severe neurological disturbance. Conclusion: Hypoglycemia was considered a new fact to explain inadequate weight. The results, new in Brazil and difficult in terms of comparison, could be used to identify risk factors and for a better approach of newborn discharged from ICUs.

Evaluation of inadequate anti-retroviral treatment in patients with HIV/AIDS

Introduction: Since the emergence of antiretroviral therapy, the survival of patients infected with human immunodeficiency virus has increased. Non-adherence to this therapy is directly related to treatment failure, which allows the emergence of resistant viral strains. Methods: A retrospective descriptive study of the antiretroviral dispensing records of 229 patients from the Center for Health Care, University Hospital, Federal University of Juiz de Fora, Brazil, was conducted between January and December 2009. Results: The study aimed to evaluate patient compliance and determine if there was an association between non-adherence and the therapy. Among these patients, 63.8% were men with an average age of 44.0 +/- 9.9 years. The most used treatment was a combination of 2 nucleoside reverse transcriptase inhibitors with 1 non-nucleoside reverse transcriptase inhibitor (55.5%) or with 2 protease inhibitors (28.8%). It was found that patients taking lopinavir/ritonavir with zidovudine and lamivudine had a greater frequency of inadequate treatment than those taking atazanavir with zidovudine and lamivudine (85% and 83.3%, respectively). Moreover, when the combination of zidovudine/lamivudine was used, the patients were less compliant (chi(2) = 4.468, 1 degree of freedom, p = 0.035). Conclusions: The majority of patients failed to correctly adhere to their treatment; therefore, it is necessary to implement strategies that lead to improved compliance, thus ensuring therapeutic efficacy and increased patient survival.