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5%). The model proposed by Guldberg to determine a genotype/phenotype correlation was applied to four classical PKU patients with two identified mutations. In three of them, the predicted moderate/moderate or moderate PKU phenotype did not coincide with the actual diagnosis. The prediction coincided with the diagnosis of one classic PKU patient. The estimated incidence of PKU for Mato Grosso, Brazil, was 133,342 live births from 2003 to 2015. CONCLUSION The only mutations found in the analyzed samples were the IVS10nt-11G>A, V388M, and R261Q. The genotype/phenotype correlation only occurred in four (5.3%) patients.OBJECTIVE To present a case of bilateral gynecomastia in a prepubertal boy with autism spectrum disorder, diagnosed with myotonic dystrophy type 1. CASE DESCRIPTION A 12-year-old boy with autism spectrum disorder presented at a follow-up visit with bilateral breast growth. There was a family history of gynecomastia, cataracts at a young age, puberty delay, and myotonic dystrophy type 1. The physical examination showed that he had bilateral gynecomastia with external genitalia Tanner stage 1. Neurologic examination was regular, without demonstrable myotonia. The analytical study revealed increased estradiol levels and estradiol/testosterone ratio. After excluding endocrine diseases, the molecular study of the dystrophia myotonica protein kinase gene confirmed the diagnosis of myotonic dystrophy type 1. COMMENTS A diagnosis of prepubertal gynecomastia should include an investigation for possible underlying diseases. This case report highlights the importance of considering the diagnosis of myotonic dystrophy type 1 in the presence of endocrine and neurodevelopmental manifestations.OBJECTIVE To evaluate the intake of ultra-processed foods by children under 24 months of age from the city of Montes Claros and identify factors associated with this consumption. METHODS This is a population-based cross-sectional study with data collected from households through interviews. A questionnaire assessed the sociodemographic conditions of the family, maternal and child characteristics, and food consumption. We adopted a multivariate model to identify factors associated with the intake of ultra-processed foods. RESULTS A total of 545 children participated in this study, of whom 74.3% consumed some kind of ultra-processed food. The factors most strongly associated with this consumption were children older than six months, infants who were not breastfed, households with up to three residents, and the main caregiver of the child being someone other than the mother. CONCLUSIONS Children under 24 months start consuming ultra-processed products at an early age, replacing foods considered natural and healthy. This study can contribute to guide health professionals in counseling families about feeding in the first years of life, emphasizing the proper introduction of complementary feeding and discouraging the consumption of ultra-processed products.OBJECTIVE To report on a case of malignant hyperthermia in a child after a magnetic resonance imaging of the skull was performed using sevoflurane anesthesia. CASE DESCRIPTION A 3-year-old boy admitted to the pediatric intensive care unit after presenting clinical and laboratory findings consistent with unspecified viral meningoencephalitis. While the patient was sedated, a magnetic resonance imaging of the skul was performed using propofol followed by the administration of sevoflurane through a laryngeal mask in order to continue anesthesia. Approximately three hours after the start of the procedure, the patient presented persistent tachycardia, tachypnea, generalized muscular stiffness and hyperthermia. With a diagnostic hypothesis of malignant hyperthermia, dantrolene was then administered, which immediately induced muscle stiffness, tachycardia, tachypnea and reduced body temperature. COMMENTS Malignant hyperthermia is a rare pharmacogenetic syndrome characterized by a severe hypermetabolic reaction after the administration of halogenated inhalational anesthetics or depolarizing muscle relaxants such as succinylcholine, or both. Although it is a potentially fatal disease, the rapid administration of continued doses dantrolene has drastically reduced the morbidity and mortality of the disease.OBJECTIVE To verify the association between participation in physical activity (PA) and the clustering of health risk behaviors (HRB) in adolescents of both genders. METHODS A cross-sectional study involving 862 adolescents (11 to 17 years old) enrolled in 14 randomly selected public schools from Curitiba, Paraná, Brazil. Participation in PA, screen time, consumption of fruit, vegetables, cigarettes and alcoholic beverages were the criteria evaluated. Multinomial logistic regression tested the association between participation in PA and clustering of HRB, and results are expressed Odds Ratio (OR) with 95% confidence intervals (95%CI). RESULTS Adolescents with high participation in PA had lower odds of clustering 2-3 HRB (OR 0.38, 95%CI 0.21-0.68; p less then 0.01) and 4-5 HRB (OR 0.29; 95%IC 0.16-0.53; p less then 0.01). Boys with high participation in PA had lower chances of clustering 2-3 HRB (OR 0.31, 95%CI 0.13-0.75; p=0.01), and girls had lower odds of clustering 2-3 HRB (OR 0.41; 95%CI 0.17-0.99; p=0.04) and 4-5 HRB (OR 0.25; 95%CI 0.10-0.61; p less then 0.01). CONCLUSIONS High participation in PA was inversely associated with the clustering of HRB in adolescents.The yellow fever is a systemic disease that was under control due to the effective campaigns against the vector and promotion of vaccines programs. However, since 1999, outbreaks appeared because of inefficient control of the vector, and led to the need of amplifying the immunization in large scale against the yellow fever virus, and consequently, raising the risk of adverse reactions to the vaccine. We report a case of previously healthy infant, who was referred to our care service, after 3 days with fever, chills, nausea and vomits, he received support therapy and was discharged from the hospital. After 24 hours of supportive measures, he was discharge. The patient returned to our service with general condition decline, strabismus, inability to control of cervical musculature and reduced force of the legs. The patient vaccine had received all vaccines from the calendar, and he was vaccinated for yellow fever 20 days before symptoms. During the hospitalization, liquor was collected, and ceftriaxone and aciclovir were administered. After negative cultures from the liquor, the antibiotics were suspended. The computed tomography of patient's brain showed no alterations. Research for antibodies against yellow fever was requested, being positive for IgM in the liquor, and confirming the neurotropic disease associated with the yellow fever vaccine. On the fifth day of hospitalization, the patient showed improvement on the strabismus, cervical tonus, and musculature force. On the tenth day of hospitalization, patient showed complete improvement, and his laboratory exams no alterations. Subsequently, patient was discharged. The vaccine against yellow fever is safe, efficient and highly recommended, however it is not completely free from serious adverse reactions, including death.OBJECTIVE To characterize storage and disposal practices associated with expired medicines in home pharmacies of Primary Care users. METHODS Cross-sectional study based on data collected from 423 users of 15 Primary Care units located in a Brazilian city, between August 2014 and July 2016. Data were collected via face-to-face interviews. Categorical (demographic and socioeconomic characteristics) and continuous variables were expressed as proportions and means and standard deviations, respectively . Storage behaviors and disposal practices associated with unused and expired medicines were described as frequencies. RESULTS Most (83%) interviewees were female and approximately 70% had completed high school. The kitchen was the most common medicine storage place (58.6%). Approximately 75% of participants reported inappropriate medicine disposal practices. CONCLUSION This study revealed high rates of inappropriate medicine disposal practices with direct impacts on pharmacological treatment and the environment. Continuing education of healthcare professionals and the general public is required to raise awareness about proper medicine use and disposal.OBJECTIVE To describe antiretroviral treatment regimens prescribed and their compliance with the Clinical Protocol and Therapy Guidelines of the Ministry of Health for the management of HIV infection. METHODS Observational and descriptive study. Secondary data of the state of Paraná (Brazil) on drugs, treatment regimens, lines of treatment and number of individuals on treatment, from January to June 2018, were accessed at the Antiretroviral Agents Logistic Control System. Combinations of antiretroviral drugs (treatment regimens) were compared according to the current Clinical Protocol and Therapy Guidelines and non-compliances were classified and quantified. RESULTS In Paraná, 35,127 individuals with HIV were treated with 253 different treatment regimens. Of the prescribed regimens, 19.1% were first-line, 27.4% second-line and 48.5% third-line. Among non-compliances, the most prevalent were absence of association of protease inhibitors and ritonavir (42.8%), low efficacy triple therapy (36.9%), double therapy (26.1%), monotherapy (20.3%), and triple therapy of nucleoside analog reverse transcriptase inhibitors (17.1%). CONCLUSION Most individuals receiving HIV treatment in the state of Paraná are on treatment regimens established in the current Clinical Protocol and Therapy Guidelines, which contributes to successful therapy. However, associations not provided by the current Clinical Protocol and Therapy Guidelines were identified in the initial treatment lines, which could lead to ineffectiveness, virologic failure and viral resistance.OBJECTIVE To analyze the obstacles and challenges faced by managers and coordination professionals in their practices in municipal coordinating centers. selleck chemicals llc METHODS An exploratory descriptive study with a qualitative focus, applied in 40 managers and coordination professionals, from September 2017 to November 2018, with semi-structured interviews, resulting in two categories of analysis limiting factors and factors that facilitate the management and operationalization of the Brazilian Unified Health System (SUS) coordinating sector. RESULTS Analyzing the statements, we found evidence of the following limiting factors failure in the criteria of referral, unavailability of beds, high demand, systemic difficulties in relation to the coordinating system, procedures of difficult scheduling and execution, increased repressed demand for elective procedures and difficulties in the flow of information between primary care and coordination. In the category of facilitating factors, the most significant possibilities were expansion of the capability to know the user's reality, improvement in primary care and increase in health financial resources, health training and education and restructuring, in addition to reorganizing internal coordinating procedures. CONCLUSION The limiting factors of coordination show the need to promote actions that offer all SUS users full access to health services.