Abstract Objectives: To measure Quality of Life (QoL) and costs of Heart Failure (HF) outpatients in Brazil as an introduction to the Value-Based Health Care (VBHC) concept. Materials and methods: Cross-sectional study, patients with HF, with ejection fraction <50%, were recruited from three hospitals in Brazil. Two QoL (36-Item Short Form Survey [SF-36] and Minnesota Living with Heart Failure Questionnaire [MLHFQ]) and two anxiety/depression questionnaires were applied. SF-36 scores were stratified by domains. Treatment costs were calculated using the Time-Driven Activity-Based Costing (TDABC) method. Results were stratified by NYHA functional class and sex. Results: From October 2018 to January 2021, 198 patients were recruited, and the median MLHFQ (49.5 [IQR 21.0, 69.0]) and SF-36 scores demonstrated poor QoL, worse at higher NYHA classes. A third of patients had moderate/severe depression and anxiety symptoms, and women had higher anxiety scores. Mean costs of outpatient follow-up were US$ 215 ± 238 for NYHA I patients, US$ 296 ± 399 for NYHA II and US$ 667 ± 1012 for NYHA III/IV. Lab/exam costs represented 30% of the costs in NYHA I, and 74% in NYHA III/IV (US $ 63.26 vs. US$ 491.05). Conclusion: Patients with HF in Brazil have poor QoL and high treatment costs; both worsen as the NYHA classification increases. It seems that HF has a greater impact on the mental health of women. Costs increase mostly related to lab/exams. Accurate and crossed information about QoL and costs is essential to drive care and reimbursement strategies based on value.

Abstract Phenylketonuria (PKU) requires tight control to prevent neurocognitive impairment but reports show that patients may present mild cognitive defects related to higher impulsivity. We hypothesize that chronic intervention may influence the parents and child bonding and the child´s resources to face problems. To describe the PKU parenting styles perceived by the children (PS) and their coping strategies (CS) assessing their relationship with impulsivity, 30 early diagnosed and adequately treated PKU children and 30 non PKU aged-paired controls (CG) were compared. The Argentine Children´s Coping Questionnaire, Argentine Scale Perception of the Relationship with Parents, WISC IV Comprehension Subtest, and CPT II test were administered. PKU PS were based on control: strict to pathologic in the mother and acceptable in the father (both p<0.05 vs. CG). Children significantly sought greater support and showed less emotional control when facing conflicts. These characteristics positively correlated with maternal control r:.383 and r:.398 (both p<0.05). Impulsivity was higher in PKU (p<0.05) but didn´t associate with PS or CS. Maternal strict control wasn´t linked to the higher impulsivity found (possibly neurobiologically based). Nevertheless, if both factors are present, patients may develop a psychological and/or behavioral trait of greater dependency and impulsivity that must be considered in their follow-up.

Abstract Phenylketonuria (PKU, OMIM 261600) is predominantly caused by mutations in the PAH gene. One hundred and three Argentine PKU patients were studied by Sanger sequencing; 101 were completely characterized (90.3% were compound heterozygotes). Fifty-four different pathogenic variants were identified. Mutations were distributed all along the PAH gene but concentrated in exon 7 (26%), 12 (12%), 11 (10%), and 6 (10%). 77% were missense, and 77% affected the enzyme catalytic domain, nine mutations accounted for 57% of 179 studied alleles: p.Arg261Gln (Allele frequency(AF):10.6%), c.1066-11G>A (AF:9,5%), p.Arg408Trp (AF:8,3%), p.Tyr414Cys (AF:5,5%), p.Ala403Val, p.Val388Met, and p.Arg158Gln (AF: 5% each), p.Leu48Ser, and p.Ile65Thr (AF:4% each). The predicted phenotype was assigned by Guldberg´s arbitrary value (AV) and compared with the clinical phenotype based in tolerance to Phe intake. 29.1% (n:30) were hyperphenylalaninemias, 18.5% (n:19) mild-PKU, 27.2% (n:28) moderate-PKU and 25.2 % (n:26) classical-PKU. Genotype/phenotype correlation was statistically significant (p<0.001) Overall concordance was 62,5%: 93.3% in hyperphenylalaninemia, 64.7% in mild-PKU and 65.4% in classical patients. The moderate-PKU showed a weak concordance (17%) with milder AV prediction than clinical assessment. 74% of discordant moderate patients harbored p.Arg261Gln, and p.Val388Met. Our cohort is highly heterogeneous, with predominant Mediterranean influence (mainly Spanish), but with differences with other Latin-American countries.

Abstract: Introduction: Sleep quality is commonly impaired in medical disorders, and the HIV-positive population is particularly vulnerable to complaint from sleep disturbances. Objective: To determine the prevalence of poor sleep quality and the factors associated with it, in a population of HIV positive Mexican out patients. Method: A cross sectional study was done. A sample of 367 HIV-positive subjects over 18 years completed a set of self-administered questionnaires: the Pittsburgh Sleep Quality Index (PSQI), the Epworth Sleepiness Scale, the Athens Insomnia Scale, and the Beck Depression Inventory. Sociodemographic and clinical data were recorded. Results: Of the respondents, 82.8% (n = 304) were male. The mean age of the patients was 36.6 (SD = 9.4) years, and the mean educational level was 12.2 (SD = 3.8) years. According to the PSQI, 58.6% (n = 215) had poor sleep quality. Poor sleep quality was more common in those who had lived longer with HIV, had started their antiretroviral treatment later, had suboptimal antiretroviral adherence, had a CD4 cell count <200 cells/µL, reported illicit drug use, had concomitant medications, or had insomnia, sleepiness, or depressive symptoms. In multivariate analyses, poor sleep quality was associated with depressive symptoms, illicit drug use, a CD4 count <200 cells/µL, and time elapsed since HIV diagnosis. Discussion and conclusion: Poor sleep quality is common in HIV patients. Early identification of patients with poor sleep quality through vigilance for factors associated with it might facilitate prompt treatment.

Resumen: Introducción: La población seropositiva es particularmente vulnerable a presentar alteraciones del sueño. Objetivo: Determinar la prevalencia y los factores asociados a la mala calidad del sueño en un grupo de pacientes con VIH en la Ciudad de México. Método: Estudio transversal. Se incluyó una muestra de 367 personas con VIH mayores de 18 años. Todos los pacientes contestaron el Índice de Calidad del Sueño de Pittsburgh (PSQI), la Escala de Somnolencia de Epworth, la Escala de Atenas de Iinsomnio y el Inventario de Depresión de Beck. Se recabaron datos sociodemográficos y clínicos. Resultados: El 82.8% (n = 304) de los encuestados eran varones. La edad media fue de 36.6 (DE = 9.4), con escolaridad promedio de 12.2 (DE = 3.8) años. El 58.6% (n = 215) tuvieron una mala calidad del sueño. La mala calidad del sueño fue más común en aquellos con mayor tiempo de haber sido diagnosticados, con mayor retardo en el inicio y adherencia subóptima al tratamiento antirretroviral, con recuento de linfocitos T-CD4+ <200 células/µL, con uso ilícito de drogas y uso de medicamentos concomitantes, o con presencia de insomnio, somnolencia y síntomas depresivos. En el análisis multivariado, la mala calidad del sueño se asoció con síntomas depresivos, uso de drogas ilícitas, un recuento de linfocitos T-CD4+ <200 células/µL y el tiempo desde el diagnóstico de VIH. Discusión y conclusión: La mala calidad del sueño es común en pacientes con VIH. Su identificación temprana a través de la vigilancia de los factores asociados con ella puede facilitar el tratamiento oportuno.

Congenital hypothyroidism (CH), as any chronic disease, has an impact on the parent-child relationship and on the child's resources to cope with conflicting situations. Objectives. To describe parenting styles according to the perception of children with CH and their coping strategies. Population and methods. Children aged 9-10 years who had CH detected by newborn screening and had received adequate treatment and a group without CH (control group). The Argentine Coping Questionnaire, the Argentine Scale for the Perception of Parent Relations, and the comprehension subtest of the Wechsler Intelligence Scale for Children III (WISC III) were used. Results were compared using a multivariate analysis of variance (MANOVA). Results. Sixty children with CH were included; they perceived that their mothers exercised a strict control and that their fathers showed more acceptance. They sought more support and became paralyzed more often in conflicting situations than the 60 children without CH. Conclusion. These findings may be associated with a higher level of dependence. They should be taken into consideration in CH care.

El hipotiroidismo congénito (HC) como enfermedad crónica impacta en la vinculación padres-hijo y en recursos del niño para afrontar situaciones conflictivas. Objetivos. Describir estilos parentales desde la percepción del hijo con HC y sus estrategias de afrontamiento. Población y métodos. Niños de entre 9 y 10 años con HC detectado por pesquisa neonatal y adecuadamente tratado y un grupo sin HC (grupo control). Se utilizó el cuestionario argentino de afrontamiento y la escala argentina de percepción de la relación con los padres y el subtest comprensión de la Wechsler Intelligence Scale for Children III (WISC III). Se compararon los resultados con el análisis multivariante de la varianza (multivariate analysis of variance; MANOVA, por sus siglas en inglés). Resultados. Se incluyeron 60 niños con HC; percibían a su madre con una modalidad de control estricto y a su padre con más aceptación. Buscaban mayor apoyo y se paralizaban más ante situaciones conflictivas que los 60 niños sin patología. Conclusión. Estos hallazgos podrían asociarse a mayor dependencia. Deben considerarse en la atención del HC.

El hipotiroidismo congénito afecta a 1:2000-3000 recién nacidos detectados por pesquisa neonatal. Las oxidasas duales, DUOX1 y 2, generan agua oxigenada, lo que constituye un paso crítico en la síntesis hormonal. Se han comunicado mutaciones en el gen DUOX2 en casos de hipotiroidismo congénito transitorio y permanente. Se describen dos hermanos con hipotiroidismo congénito detectados por pesquisa neonatal, con glándula tiroides eutópica y tiroglobulina elevada. Recibieron levotiroxina hasta su reevaluación en la infancia con suspensión del tratamiento. Su función tiroidea fue normal y se consideró el cuadro como transitorio por un posible defecto de organificación. Ambos pacientes eran heterocigotos compuestos para una mutación en el exón 9 del alelo paterno (c.1057_1058delTT, p.F353PfsX36 o p.F353fsX388) y otra en el exón 11 del alelo materno (c.1271T>G, p.Y425X) del gen DUOX2. Nuestro hallazgo confirma que la magnitud del defecto de DUOX2 no se relaciona con el número de alelos afectados, lo que sugiere mecanismos compensadores en la generación de peróxido.

Introducción. Los niños con hipotiroidismo congénito (HC) detectados por pesquisa neonatal y tratados adecuadamente presentarían defectos cognitivos leves. Objetivos. Evaluar el coeficiente intelectual de niños con HC e identificar la presencia de déficits cognitivos específicos. Población y métodos. Se seleccionó un grupo de 60 niños con HC, de entre 9 y 10 años, detectados por pesquisa neonatal y tratados adecuadamente desde el primer mes de vida, y se comparó con un grupo control de 60 niños sin HC de la misma edad. Fueron criterios de inclusión la ausencia de patología intercurrente, concurrencia a jornada escolar simple y padres con nivel escolar mínimo de secundaria completa. En entrevistas individuales, se administraron la escala de inteligencia Wechsler para niños, tercera edición, figura compleja de Rey, test Woodcock-Muñoz revisado, Conners Continuous Performance Test II, test Illinois de aptitudes psicolingüísticas, test de fluidez verbal, test de cubos de Knox, Trail Making Test, test de caras y test de los 5 dígitos. Se realizó el análisis estadístico con pruebas t de Student (muestras independientes) ajustado por Bonferroni (p < 0,002). Resultados. Aun dentro del rango normal promedio, hubo diferencias significativas entre grupos en el coeficiente intelectual total y de ejecución (tamaño del efecto pequeño y moderado, respectivamente). Los niños hipotiroideos presentaron, en el área de ejecución, significativamente menor desempeño en velocidad de procesamiento, tiempos de reacción, atención, flexibilidad cognitiva, visoconstrucción y memoria a largo plazo. No hubo diferencia significativa entre grupos en el área verbal. Conclusiones. Los niños hipotiroideos congénitos, sin discapacidad mental, presentaron defectos cognitivos leves, que deben ser tenidos en cuenta para su atención integral.

Abstract The phenylalanine (PHE)-restricted diet has improved in quality and diversity over time and has proven to be effective in all patients. Nevertheless, this treatment imposes a heavy social and economic burden to patient and family and impacts quality of life. Sustained adherence to PHE restriction is difficult to maintain. Moreover, even patients with phenylketonuria (PKU) with normal intelligence quotient (IQ) have lower IQ than matched individuals without PKU and can have deficits in multiple other aspects of neuropsychological function, including cognitive and executive function, working memory. They can also have behavior problems, depression, and low self-esteem. In recent years, alternative treatments for PKU have been developed and their use has been indicated for some patients who are candidates for options besides traditional treatment. Sapropterindihydrochloride, large neutral amino acids, and glycomacropeptide are alternative treatment options in use for selected patients. The aim of this article is to review the current knowledge of these new approaches to PKU treatment.

Tuberculosis is currently one of the most important re-emerging infections worldwide. Up to 20% of cases are extra-thoracic. However, the presence of parotid tuberculosis is uncommon even in developing countries where the incidence of this disease is higher. The case of an 83 year-old patient is presented who had a tumour in the parotid region of 4 weeks onset, and with no previous personal history. Clinical examination revealed a mass of approximately 3 × 2 cm in the left parotid. It was not painful on palpation and there was no evidence of cervical lymph node involvement. After inconclusive radiological examination and fine needle aspiration, an excision of the lesion was performed, and the specimen sent for a PCR study. This confirmed the diagnosis of parotid tuberculosis, and after receiving additional tuberculosis treatment the patient was discharged due to remission of the disease.

Hoy en día la tuberculosis es una de las más importantes infecciones reemergentes en el mundo. Hasta un 20% de las formas de presentación son extratorácicas. Sin embargo, la presencia de tuberculosis a nivel parotídeo es infrecuente incluso en países subdesarrollados, donde la incidencia de esta enfermedad es mayor. Presentamos un paciente de 83 años que acude por tumoración en región parotídea de 4 semanas de evolución sin antecedentes personales. La exploración clínica revela una masa en cola de parótida izquierda de aproximadamente 3 × 2 cm, no dolorosa a la palpación y sin evidencia de adenopatías a nivel cervical. Tras el estudio radiológico y punción con aguja fina, no se obtienen datos concluyentes, por lo que se procede a realizar exéresis. Se envía muestra para estudio de PCR, se confirma diagnóstico de tuberculosis parotídea. Tras recibir quimioterapia antituberculosa complementaria, el paciente es dado de alta por remisión de la enfermedad.

Resumo: O objetivo deste trabalho foi avaliar o efeito do tratamento de sementes com inseticidas sobre o manejo de Dichelops melacanthus e a produtividade da soja e do milho, cultivados em sucessão. O estudo foi realizado em campo, nas safras 2012/2013 (I) e 2013/2014 (II). Avaliaram-se os inseticidas imidacloprido, tiametoxam, tiodicarbe, fipronil e abamectina. Determinaram-se: a densidade populacional do percevejo, a produtividade de soja e milho, e a intensidade da injúria em milho. A densidade do percevejo permaneceu abaixo de um inseto por metro quadrado, na maior parte do ciclo da soja. Os picos populacionais foram observados nas primeiras semanas, após a emergência do milho, e atingiram 2,2 (safra I) e 6,7 (safra II) percevejos por metro quadrado. Na cultura da soja, os inseticidas não reduziram a densidade populacional do percevejo. Na cultura do milho, o imidacloprido reduziu a densidade do percevejo em 23,2% (safra I) e 38,8% (safra II), e a injúria em 61,8% (safra I) e 26,4% (safra II). O tiametoxam reduziu a densidade dos insetos em 27,8% (safra II) e a injúria em 42,7% (safra I). O tratamento de sementes com inseticidas não proporciona aumento de produtividade à soja e ao milho, portanto, não se justifica sua utilização nas condições deste estudo.

Abstract: The objective of this work was to evaluate the effect of seed treatment with insecticides on the management of Dichelops melacanthus on the yield of soybean and corn, grown in succession. The study was carried out in a field, in the 2012/2013 (I) and 2013/2014 (II) crop seasons. The evaluated insecticides were: imidacloprid, thiamethoxam, thiodicarb, fipronil, and abamectin. The following were determined: stink bug population density, soybean and corn yield, and corn injury. Population density remained below one stink bug per square meter, in most of the soybean cycle. Population peaks were observed in the first weeks, after corn emergence, and they reached 2.2 (crop season I) and 6.7 (crop season II) stink bugs per square meter. In the soybean crop, the insecticides did not reduce the stink bug population density. In the corn crop, imidacloprid reduced the stink bug density in 23.2% (crop season I) and 38.8% (crop season II), and injury in 61.8% (crop season I) and 26.4% (crop season II). Thiamethoxam reduced the insect population density in 27.8% (crop season II) and injury in 42.7% (crop season I). Seed treatment with insecticides does not provide increase for soybean and corn yields, therefore, their use is not justified in this study's conditions.

Este estudo objetivou relatar as relações entre trabalhadores e usuários e suas implicações para a continuidade do cuidado. MÉTODOS: Trata-se de uma pesquisa qualitativa, desenvolvida a partir de um estudo de caso, de fevereiro a julho de 2012. Os sujeitos foram trabalhadores de saúde e usuários de uma Unidade de Saúde da Família. Realizaram-se observação participante, entrevistas e buscas em documentos e registros. RESULTADOS: Evidenciaram-se momentos em que os usuários são generalizados pela equipe, que desconsidera sua singularidade; isso desqualifica o usuário, revelando a fragilidade da integralidade da atenção. CONCLUSÃO: Há implicações das relações colonialistas que consideram usuários como objetos, afetando a continuidade do cuidado. Descortinou-se a participação de enfermeiros neste processo, reforçando a necessidade de se aprofundar a reflexão acerca da prática profissional.

OBJECTIVE: To report on the relationships between the workers and service users and the implications of these for continuity of care. METHODS: Qualitative research, undertaken based on a case study, between February and July 2012. The subjects were health workers and the users of a Family Health Unit(FHU). Participant observation, interviews and document and record searches were made. RESULTS: Instances were evidenced in which the team generalizes the service users, which disregards their uniqueness; this dismisses the service user, revealing the fragility of the comprehensiveness of the care. CONCLUSION: There are implications of colonialist relationships which consider the service users as objects, affecting the continuity of the care. The nurses' participation in the processwas revealed, emphasizing the need to deepen reflection in regard to professional practice.

OBJETIVO: Relatar las relaciones entre trabajadores y usuarios y sus implicaciones para la continuidad de la atención. MÉTODOS: Es una investigación cualitativa, desarrollada a partir de un estudio de caso, de Febrero a Julio de 2012. Los sujetos fueron trabajadores de salud y usuarios de una Unidad Salud de la Familia. Se realizó la observación participante, entrevistas y búsquedas en documentos y registros. RESULTADOS: Fueron evidenciados momentos donde los usuarios son generalizados por el equipo, que desconsidera su singularidad; descalifica el usuario, revelando fragilidad de la atención integral. CONCLUSIÓN: Hay implicaciones de las relaciones coloniales que consideran usuarios como objetos, afectando la continuidad de la atención. Fue revelado la participación de enfermeros en este proceso, reforzando la necesidad de mayor reflexión sobre la práctica profesional.

OBJECTIVE: To report on the relationships between the workers and service users and the implications of these for continuity of care. METHODS: Qualitative research, undertaken based on a case study, between February and July 2012. The subjects were health workers and the users of a Family Health Unit(FHU). Participant observation, interviews and document and record searches were made. RESULTS: Instances were evidenced in which the team generalizes the service users, which disregards their uniqueness; this dismisses the service user, revealing the fragility of the comprehensiveness of the care. CONCLUSION: There are implications of colonialist relationships which consider the service users as objects, affecting the continuity of the care. The nurses' participation in the processwas revealed, emphasizing the need to deepen reflection in regard to professional practice.

OBJETIVO: Relatar las relaciones entre trabajadores y usuarios y sus implicaciones para la continuidad de la atención. MÉTODOS: Es una investigación cualitativa, desarrollada a partir de un estudio de caso, de Febrero a Julio de 2012. Los sujetos fueron trabajadores de salud y usuarios de una Unidad Salud de la Familia. Se realizó la observación participante, entrevistas y búsquedas en documentos y registros. RESULTADOS: Fueron evidenciados momentos donde los usuarios son generalizados por el equipo, que desconsidera su singularidad; descalifica el usuario, revelando fragilidad de la atención integral. CONCLUSIÓN: Hay implicaciones de las relaciones coloniales que consideran usuarios como objetos, afectando la continuidad de la atención. Fue revelado la participación de enfermeros en este proceso, reforzando la necesidad de mayor reflexión sobre la práctica profesional.

Este estudo objetivou relatar as relações entre trabalhadores e usuários e suas implicações para a continuidade do cuidado. MÉTODOS: Trata-se de uma pesquisa qualitativa, desenvolvida a partir de um estudo de caso, de fevereiro a julho de 2012. Os sujeitos foram trabalhadores de saúde e usuários de uma Unidade de Saúde da Família. Realizaram-se observação participante, entrevistas e buscas em documentos e registros. RESULTADOS: Evidenciaram-se momentos em que os usuários são generalizados pela equipe, que desconsidera sua singularidade; isso desqualifica o usuário, revelando a fragilidade da integralidade da atenção. CONCLUSÃO: Há implicações das relações colonialistas que consideram usuários como objetos, afetando a continuidade do cuidado. Descortinou-se a participação de enfermeiros neste processo, reforçando a necessidade de se aprofundar a reflexão acerca da prática profissional.

Mutações ativadoras no gene receptor de TSH (TSHR) foram identificadas como a base molecular do hipertireoidismo congênito não autoimune. Descrevemos os achados clínicos e a caracterização molecular de uma menina que apresentou hipertireoidismo autoimune grave desde o nascimento, nascida de uma mãe com desordem tiroidiana autoimune. Tratada com metilmercaptoimidazol e β-bloqueadores, ela permaneceu com hipertiroidismo e necessitou de tiroidectomia total. A fim de caracterizar a presença da mutação ativadora, foram analisados toda a sequência codificadora assim como os extremos dos íntrons e éxons do gene TSHR. A paciente era heterozigota para a mutação p.Ser281Asn e para o polimorfismo p.Asp727Glu. Essa mutação recorrente, p.Ser281Asn, caracterizada in vitro pelo aumento da produção basal de cAMP, é a única variante genética ativadora da linhagem germinativa descrita até hoje no domínio extracelular do receptor de TSH. Interessantemente, a mãe da paciente apresentou hipertireoidismo sem qualquer mutação ativadora do gene TSHR. Embora o hipertireoidismo congênito não autoimune seja uma condição rara, ele deve ser investigado quando há persistência da doença grave, mesmo em um recém-nascido de uma mãe com hipertireoidismo autoimune, a fim de se diferenciar o caso da forma mais comum da doença autoimune congênita. Arq Bras Endocrinol Metab. 2012;56(8):513-8

Activating mutations in the TSH Receptor (TSHR) gene have been identified as the molecular basis for congenital non-autoimmune hyperthyroidism. We describe the clinical findings and molecular characterization in a girl who presented severe non-autoimmune hyperthyroidism since birth, born to a mother with autoimmune thyroid disease. She was treated with methylmercaptoimidazol and β-blockers, but remained hyperthyroid and required total thyroidectomy. To characterize the presence of an activating mutation, the whole coding sequence and intron-exon boundaries of TSHR gene were analyzed. The patient was heterozygous for p.Ser281Asn mutation and p.Asp727Glu polymorphism. This recurrent mutation, p.Ser281Asn, characterized in vitro by increased basal production of cAMP, is the unique germline activating gene variant described so far in the extracellular domain of TSH receptor. Interestingly, the patient's mother presented hyperthyroidism but without any TSHR gene activating mutation. Although congenital non-autoimmune hyperthyroidism is a rare condition, it should be investigated when severe disease persists, even in a newborn from an autoimmune hyperthyroid mother, in order to differentiate it from the more common congenital autoimmune disease. Arq Bras Endocrinol Metab. 2012;56(8):513-8

INTRODUÇÃO: A colestase neonatal causada por doenças endócrinas é pouco frequente e reconhecida. Existe um atraso no encaminhamento dos pacientes a um endocrinologista pediátrico. OBJETIVO: Caracterizamos a colestase em recém-nascidos com deficiências congênitas de hormônio hipofisário (DCHH) e sua resolução após a terapia de reposição hormonal (TRH). SUJEITOS E MÉTODOS: Dezesseis pacientes (12 do sexo masculino) foram incluídos; sete com DCHH, e cinco com hipocortisolismo central isolado. RESULTADOS: O início da colestase ocorreu aos 18 dias de vida (variação 2-120). Dez e nove pacientes apresentaram elevação das transaminases e γGT, respectivamente. A consulta com um endocrinologista aconteceu aos 32 dias (variação 1-72). A remissão da colestase ocorreu em uma idade mediana de 65 dias, enquanto a remissão das enzimas hepáticas aconteceu aos 90 dias. Na coorte isolada, o hipocortisolismo foi uma desordem transitória. CONCLUSÃO: A colestase causada por deficiências hormonais foi completamente resolvida após a introdução da TRH. O hipocortisolismo pode ser uma causa transitória da colestase e precisa ser reavaliado após a remissão da colestase.

INTRODUCTION: Neonatal cholestasis due to endocrine diseases is infrequent and poorly reco-gnized. Referral to the pediatric endocrinologist is delayed. OBJECTIVE: We characterized cholestasis in infants with congenital pituitary hormone deficiencies (CPHD), and its resolution after hormone replacement therapy (HRT). SUBJECTS AND METHODS: Sixteen patients (12 males) were included; eleven with CPHD, and five with isolated central hypocortisolism. RESULTS: Onset of cholestasis occurred at a median age of 18 days of life (range 2-120). Ten and nine patients had elevated transaminases and γGT, respectively. Referral to the endocrinologist occurred at 32 days (range 1 - 72). Remission of cholestasis occurred at a median age of 65 days, whereas liver enzymes occurred at 90 days. In our cohort isolated, hypocortisolism was a transient disorder. CONCLUSION: Cholestasis due to hormonal deficiencies completely resolved upon introduction of HRT. Isolated hypocortisolism may be a transient cause of cholestasis that needs to be re-evaluated after remission of cholestasis.

The aim of this study was to identify knowledge and practice about the strategy of Integrated Management of Childhood Illness (IMCI) in the professional practice of graduates of the School of Nursing at University of São Paulo (EEUSP). It was developed a descriptive study from a quantitative approach. Online questionnaire, created and managed by the editor Dream Weaver, was sent to 396 graduate students from 2003-2007. From this total, 62 (16.6%) were labeled as non-existent addresses and 61 (15.4%) questionnaires were filled. The descriptive analysis was performed using SPSS version 16.0. Most respondents were women (91.8%) and the participant ages concentrated between 25 and 29 years old (65.6%). Two-thirds had made at least one graduate course, mostly in hospitals. Only one-third worked or works in primary care. Two ex-students correctly answered all the questions about knowledge, one of them with training in IMCI. The strategy was remembered as part of the undergraduate curriculum for almost all graduates (95.1%) and useful for 86.9%. For 45.9%, the workload was insufficient for their learning and practice and 37.7% did not feel confident to apply the strategy in professional practice. The results showed that almost all participants remembered the strategy in the undergraduate curriculum and most consider it useful. On the other hand, more than one third did not feel confident to apply it, and little of the theoretical content has been consolidated. This data suggests the need for a revision of the teaching methodology adopted.

Con el objetivo de identificar conocimiento acerca de la estrategia de Atención Integrada a las Enfermedades Prevalentes de la Infancia (AIEPI), consolidadas en la práctica profesional de egresados de la Escuela de Enfermería de la Universidad de São Paulo (EEUSP), se desarrolló un estudio descriptivo de enfoque cuantitativo. Consistió en el envío de cuestionario en línea, creado y administrado por el editor Dream Weaver, a 396 estudiantes graduados en el período 2003-2007. De este total, 62 (15,6%) regresó por e-mail no existentes y se obtuvo retorno de 61 (15,4%) cuestionarios completados. El análisis se realizó mediante SPSS versión 16.0. La mayoría de los encuestados fueron mujeres (91,8%) de edades entre 25-29 años (65,6%). Dos tercios habían hecho al menos un curso de postgrado, principalmente en área hospitalario. Sólo un tercio trabajaba o tenía trabajado en la atención primaria. Dos ex-alumnos respondieran correctamente a todas las preguntas de conocimiento, uno de ellos el único con capacitación postgrado en AIEPI. La estrategia fue recordada como parte del currículo de pregrado por casi todos los graduados (95,1%) y referida como útil por 86,9%. Para 45,9%, el volumen de contenido fuera insuficiente para su aprendizaje y 37,7% no se sentirán seguros para aplicar la estrategia en la práctica profesional. Los resultados mostraron que casi todos recordaban de la estrategia en el currículo de pregrado y la gran mayoría considera que es útil, pero más de un tercio no se sentía seguro y poco contenido teórico se ha consolidado. Estos datos sugieren la necesidad de revisión de la metodología de enseñanza utilizada.

Com o objetivo de identificar conhecimentos adquiridos sobre a estratégia de Atenção Integrada às Doenças Prevalentes na Infância (AIDPI) e consolidados na prática profissional de egressos da Escola de Enfermagem da Universidade de São Paulo (EEUSP), desenvolveu-se um estudo descritivo de abordagem quantitativa. Constituiu-se no envio de questionário online, criado e gerenciado pelo editor Dream Weaver, a 396 egressos de 2003-2007. Desse total, 62 (15,6%) retornaram por endereços inexistentes e obteve-se retorno de 61 (15,4%) questionários preenchidos. As análises foram realizadas com uso do programa SPSS versão 16.0. A maioria dos respondentes foram mulheres (91,8%) com idade entre 25-29 anos (65,6%). Dois terços haviam feito pelo menos um curso de aperfeiçoamento, em sua maioria na área hospitalar. Apenas um terço atua ou atuou na atenção básica. Dois egressos responderam corretamente todas as questões sobre conhecimento, sendo um deles o único que referiu capacitação em AIDPI. A estratégia foi lembrada como parte do currículo da graduação por quase todos os egressos (95,1%) e útil para 86,9% Para 45,9%, a carga horária foi insuficiente para seu aprendizado e 37,7% não se sentiram seguros para aplicar a estratégia na vida profissional. Os resultados evidenciaram que quase a totalidade se lembra da estratégia no currículo da graduação e a grande maioria a considera útil, porém mais de um terço não se sentia seguro e pouco do conteúdo teórico foi consolidado. Esses dados sugerem a necessidade de revisão da metodologia de ensino empregada.