ABSTRACT A male infant presented with progressive jaundice immediately after birth. Fecal acholia and choluria associated with extensive bullous skin lesions in his trunk, abdomen, and upper and lower limbs developed during phototherapy. Several diagnostic hypotheses were presented, including neonatal porphyria, hemochromatosis, Alagille syndrome, and neonatal lupus. A 24-hour urine sample for the dosage of urinary porphyrins was collected, showing high results (1823.6µg in 100mL). At 50 days of life, fluorescence spectroscopy using a Wood’s lamp revealed simultaneous bright red fluorescence of urine-stained diapers and sample blood. A definitive diagnosis of congenital erythropoietic porphyria was made following identification of a mutation of the uroporphyrinogen synthetases III gene on genetic testing. The patient was subsequently maintained in a low light environment since then, resulting in improvement of the lesions. Congenital erythropoietic porphyria is a disease of the group of porphyrias that presents shortly after birth with blistering occurring in regions exposed to the sun or other ultraviolet light. Atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth may also be observed. There is no specific treatment, and prophylaxis comprising a total avoidance of sunlight is generally recommended. A high degree of suspicion is required for diagnosis. An early diagnosis can lead to less damage. Here, we present the case of a newborn with congenital erythropoietic porphyria diagnosed after presenting with bullous lesions secondary to phototherapy.

Abstract: Background: Acne in women is often associated with anxiety and depression, and may persist from adolescence as well as manifest for the first time in adulthood. Genetic and hormonal factors contribute to its etiopathogenesis, and maintenance treatment is required, usually for years, due to its clinical evolution. Objective: To develop a guide for the clinical practice of adult female acne. Methods: A team of five experts with extensive experience in acne conducted a literature review of the main scientific evidence and met to discuss the best practices and personal experiences to develop a guide containing recommendations for the clinical practice of adult female acne. Results: The group of specialists reached consensus on the main guidelines for clinical practice, providing detailed recommendations on clinical picture, etiopathogenesis, laboratory investigation and treatment of adult female acne. Conclusion: Different from teenage acne, adult female acne presents some characteristics and multiple etiopathogenic factors that make its management more complex. This guide provides recommendations for best clinical practices and therapeutic decisions. However, the authors consider that additional studies are needed in order to provide more evidence for adult female acne to be better understood.

Objetivo: Avaliar a satisfação no trabalho de profissionais não médicos, a fim de identificar possíveis falhas do serviço e propor modificações para melhoria. Métodos: Foi realizado um estudo transversal e descritivo com 14 funcionários do Centro Oftalmológico no Hospital Regional de Divinolândia, São Paulo. Utilizou-se como instrumento de pesquisa, um questionário autoaplicável desenvolvido pelos autores. Os dados coletados foram tabulados e apresentados na forma de tabelas. Resultados: Relataram ótimo nível de satisfação com o trabalho desempenhado (71,4%). O fator que mais agradou os funcionários no ambiente de trabalho foi o bom relacionamento com os colegas (35,6%). Consideraram como sendo ótimo o relacionamento com a equipe médica e com os pacientes (71,4%). Referiram como fator mais desagradável o fato de o espaço físico ser muito pequeno (35,7%). A maioria dos funcionários (64,3%) trabalhava na instituição há mais de 10 anos. Conclusão: Encontrou-se elevado grau de satisfação dos funcionários com a instituição, com a equipe médica e com os pacientes.

Objective: To evaluate job satisfaction of non-medical professionals in order to identify potential service failures and propose changes for improvements. Methods: A cross-sectional, descriptive study of 14 employees in the Ophthalmologic Center Divinolândia Regional Hospital in São Paulo state. Was used as a research tool, a self-administered questionnaire developed by the authors. The collected data were tabulated and presented in tables. Results: Reported great satisfaction with the work performed (71,4%). The factor that most pleased the employees in the workplace was the good relationship with colleagues (35.6 %). Considered as great the relationship with the medical staff and the patients (71,4%). Mentioned as the most unpleasant factor in the workplace, the fact that the physical space is very small (35.7%). Most employees (64.3 %) worked in the institution for more than 10 years. Conclusion: We found a high satisfactory degree of employee satisfaction with the institution, with medical staff and patients.

OBJETIVO: Avaliar a fila de espera pelo atendimento oftalmológico detectando os problemas oculares; estudar o agravo que esta espera pode acarretar ao paciente; verificar a eficácia na triagem dos casos de urgência e averiguar a possibilidade de aumento da capacidade de atendimento clínico e cirúrgico. MÉTODOS Foi realizada pesquisa retrospectiva e descritiva dos dados obtidos durante 12 mutirões de atendimento oftalmológico. Os mutirões foram realizados com a intenção de diminuir a fila de espera e atender as urgências que surgiram nos dias de atendimento; ocorreram aos sábados durante os meses de junho a agosto no ano de 2006, em 16 municípios da região do Conderg(Consórcio de Desenvolvimento da Região de Governo de São João da Boa Vista). RESULTADOS: Das 2.000 vagas disponibilizadas, foram utilizadas 1.743 (87,1%) dos encaminhados. Nos diagnósticos realizados se destacam os vícios de refração com 683 casos, correspondendo a 39,1 % seguido de catarata com 296, correspondendo a 20,9. Das 238 cirurgias indicadas, 54,6% foram de facectomia. Foram detectados 35 casos (2,0%) considerados como urgência. CONCLUSÃO: Nos diagnósticos realizados durante os mutirões de atendimento à demanda reprimida, destacaram-se os vícios de refração e catarata; que somados representaram a maioria dos problemas detectados. O Hospital de Divinolândia tem recursos humanos e materiais para atender a demanda gerada, e não absorvida pelo SUS local. O encaminhamento imediato das urgências pelos triadores dos postos de saúde mostrou-se eficaz.

OBJECTIVE: To assess the waiting time for eye care identifying the number of patients with each complaint; to investigate how the waiting time may worsen the patient's condition; to check the screening of urgent cases for effectiveness; and to devise means of increasing the medical-surgical care capacity. METHODS: A retrospective descriptive survey was conducted using data obtained on 12 occasions during collaborative team visits to provide eyecare services. These initiatives were designed to decrease the waiting time and to treat urgent cases that occurred on each occasion; eyecare services were provided every Saturday, in the period from June to August 2006, in 16 cities of the region covered by Conderg (Consortium for the Development of the São João da Boa Vista Administrative Region). RESULTS: Referrals used 1,743 (87.1%) of the 2,000 places available. The most frequent diagnoses were refractive errors, with 683 cases, corresponding to 39.1% of the total, followed by cataracts, with 296 cases, corresponding to 20.9%. Of the 238 surgeries indicated, 54.6% were phakectomies. Thirty-five (2.0%) cases were considered urgent. CONCLUSION: The most common diagnoses made during the team visits to manage the excess demand for eyecare were refractive errors and cataracts, which, together, accounted for the majority of the cases. The Divinolândia Hospital has the necessary human and material resources to meet the demand left unattended by the local SUS network. Immediate referral of urgent cases by the primary units' screeners proved effective.

OBJETIVO: Estudar e comparar o aspecto dos cabelos de portadores das síndromes de Chédiak-Higashi e Griscelli-Prunieras, tanto na microscopia óptica convencional quanto com luz polarizada. MÉTODO: Cabelos de dois doentes portadores da síndrome de Chédiak-Higashi e de dois portadores da síndrome de Griscelli-Prunieras foram obtidos e estudados tanto à microscopia convencional quanto com luz polarizada. RESULTADOS: Na microscopia óptica convencional, os cabelos dos doentes portadores da síndrome de Chédiak-Higashi mostraram grânulos de melanina regulares, com distribuição homogênea e de maior tamanho em comparação aos presentes no cabelo normal. À microscopia de luz polarizada notou-se aspecto brilhante e refringência policromática. Diferentemente, os cabelos dos doentes portadores da síndrome de Griscelli-Prunieras apresentaram à microscopia convencional, grânulos de melanina irregulares e maiores do que os presentes no cabelo normal e os presentes nos cabelos dos doentes portadores da síndrome de Chédiak-Higashi, preferencialmente próximos à medula das hastes pilosas. À microscopia de luz polarizada apresentaram aspecto monotonamente esbranquiçado. CONCLUSÃO: O exame dos cabelos pela microscopia convencional nas síndromes de Chédiak-Higashi e Griscelli-Prunieras revela diferenças sutis no reconhecimento dessas doenças. No presente trabalho apresentamos evidência de que o exame das hastes pilosas com microscopia de luz polarizada - não descrito previamente - contribui na diferenciação de ambas doenças sugerindo que esse seja um método diagnóstico útil na distinção entre as síndromes de Chédiak-Higashi e Griscelli Prunieras, especialmente nos casos em que estudos moleculares mais sofisticados não estejam disponíveis.

PURPOSE: To study and compare the appearance of hairs from patients with Chédiak-Higashi and Griscelli-Prunieras syndromes under light and polarized light microscopy. METHOD: Hairs from 2 Chédiak-Higashi and 2 Griscelli-Prunieras patients were obtained and examined under normal and polarized light microscopy. RESULTS: Under light microscopy, hairs from Chédiak-Higashi patients presented evenly distributed, regular melanin granules, larger than those seen in normal hairs. Under polarized light microscopy, shafts exhibited a bright and polychromatic refringence appearance. In contrast, hair from Griscelli-Prunieras patients, under light microscopy, exhibited bigger and irregular melanin granules, distributed mainly near the medulla. Under polarized light microscopy, shafts appeared monotonously white. CONCLUSION: Light microscopic examination of hair shafts of patients with Chédiak-Higashi or Griscelli-Prunieras syndrome reveals subtle differences that are useful in identifying both disorders, but not in distinguishing between them. We provide evidence that polarized light microscopy of hair shafts, an approach that has not been previously described, aids in differentiating between these syndromes. We propose hair study by polarized light microscopy as a helpful complementary diagnostic method for differential diagnosis between CHS and GPS, especially when the more sophisticated molecular studies are not available.