Hereditary epidermolysis bullosa: clinical-epidemiological profile of 278 patients at a tertiary hospital in São Paulo, Brazil

Abstract Background Epidermolysis bullosa (EB) is a group of rare hereditary diseases, characterized by fragility of the skin and mucous membranes. Epidemiological data on EB in Brazil are scarce. Objectives To describe epidemiological aspects of patients with EB diagnosed in the Dermatology Department of a tertiary hospital, from 2000 to 2022. Methods An observational and retrospective study was conducted through the analysis of medical records. The evaluated data included clinical form, sex, family history, consanguinity, age at diagnosis, current age, time of follow-up, comorbidities, histopathology and immunomapping, presence of EB nevi and squamous cell carcinomas (SCC), cause of and age at death. Results Of 309 patients with hereditary EB, 278 were included. The most common type was dystrophic EB (DEB), with 73% (28.4% dominant DEB, 31.7% recessive DEB and 12.9% pruriginous DEB). Other types were junctional EB with 9.4%, EB simplex with 16.5% and Kindler EB with 1.1%. Women accounted for 53% and men for 47% of cases. Family history was found in 35% and consanguinity in 11%. The mean age at diagnosis was 10.8 years and the current age was 26 years. The mean time of follow-up was nine years. Esophageal stenosis affected 14%, dental alterations affected 36%, malnutrition 13% and anemia 29%. During diagnostic investigation, 72.6% underwent histopathological examination and 92% underwent immunomapping. EB nevi were identified in 17%. Nine patients had SCC. Eleven patients died. Study limitations Insufficient data included to medical records, loss to follow-up, and unavailability of genetic testing. Conclusions In this study, dystrophic EB predominated and the need for multidisciplinary care for comorbidities and complications was highlighted.

Advantages of whole-exome sequencing over immunomapping in 67 Brazilian patients with epidermolysis bullosa

Abstract Background Epidermolysis bullosa (EB) is characterized by skin fragility and blistering. In Brazil, the diagnosis is usually obtained through immunomapping, which involves a skin biopsy. Most recently, whole exome sequencing (WES) has become an important tool for the diagnosis of the subtypes of EB, providing information on prognosis as well as allowing appropriate genetic counseling for the families. Objective To compare the results of immunomapping and molecular analysis and to describe the characteristics of a Brazilian cohort of patients with EB. Methods Patients were submitted to clinical evaluation and WES using peripheral blood samples. WES results were compared to those obtained from immunomapping testing from skin biopsies. Results 67 patients from 60 families were classified: 47 patients with recessive dystrophic EB (DEB), 4 with dominant DEB, 15 with EB simplex (EBS), and 1 with junctional EB (JEB). Novel causative variants were: 10/60 (16%) in COL7A1 associated with recessive DEB and 3 other variants in dominant DEB; one homozygous variant in KRT5 and another homozygous variant in PLEC, both associated with EBS. Immunomapping was available for 59 of the 67 patients and the results were concordant with exome results in 37 (62%), discordant in 13 (22%), and inconclusive in 9 patients (15%). Study limitations Even though EB is a rare disease, for statistical purposes, the number of patients evaluated by this cohort can still be considered limited; other than that, there was a significant difference between the proportion of types of EB (only one case with JEB, against more than 50 with DEB), which unfortunately represents a selection bias. Also, for a small subset of families, segregation (usually through Sanger sequencing) was not an option, usually due to deceased or unknown parent status (mostly the father). Conclusion Although immunomapping has been useful in services where molecular studies are not available, this invasive method may provide a misdiagnosis or an inconclusive result in about 1/3 of the patients. This study shows that WES is an effective method for the diagnosis and genetic counseling of EB patients.

Consensus on the therapeutic management of atopic dermatitis ‒ Brazilian Society of Dermatology: an update on phototherapy and systemic therapy using e-Delphi technique

Abstract This publication is an update of the "Consensus on the therapeutic management of atopic dermatitis - Brazilian Society of Dermatology" published in 2019, considering the novel, targeted-oriented systemic therapies for atopic dermatitis. The initial recommendations of the current consensus for systemic treatment of patients with atopic dermatitis were based on a recent review of scientific published data and a consensus was reached after voting. The Brazilian Society of Dermatology invited 31 experts from all regions of Brazil and 2 international experts on atopic dermatitis who fully contributed to the process. The methods included an e-Delphi study to avoid bias, a literature search and a final consensus meeting. The authors added novel approved drugs in Brazil and the indication for phototherapy and systemic therapy for AD. The therapeutical response to systemic treatment is hereby reported in a suitable form for clinical practice and is also part of this updated manuscript.

Bullous lesions following phototherapy in a newborn

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.

Morbidity and survival after perioperative chemotherapy in gastric cancer: a study using the becker's classification and regression / Morbidade e sobrevida após quimioterapia periooperatória no câncer gástrico: um estudo utilizando a classificação de becker e a regressão linfonodal

ABSTRACT BACKGROUND: Gastric cancer is an aggressive neoplasm with a poor prognosis. The multimodal approach with perioperative chemotherapy is currently the recommended treatment for patients with locally advanced gastric cancer. This treatment induces a histopathological response expressed either through the degree of regression of the primary tumor or of the lymph nodes or through yTNM staging. Despite its advantages, there are still doubts regarding the effects of chemotherapy on postoperative morbidity and mortality. AIMS: This study aims to evaluate the impact of perioperative chemotherapy and its effect on anatomopathological results and postoperative morbidity and on patient survival. METHODS: This is an observational retrospective study on 134 patients with advanced gastric cancer who underwent perioperative chemotherapy and curative radical surgery. The degree of histological regression of the primary tumor was evaluated according to Becker's criteria; the proportion of regressed lymph nodes was determined, and postoperative complications were evaluated according to the Clavien-Dindo classification. Survival times were compared between the groups using Kaplan-Meier curves and the Mantel-Cox log-rank test. RESULTS: In all, 22.3% of the patients were classified as good responders and 75.9% as poor responders. This variable was not correlated with operative morbidity (p=1.68); 64.2% of patients had invaded lymph nodes and 46.3% had regressed lymph nodes; and 49.4% had no lymphatic invasion and 61.9% had no signs of venous invasion. Postoperative complications occurred in 30.6% of the patients. The group of good responders had an average survival of 56.0 months and the group of poor responders had 34.0 months (p=0.17). CONCLUSION: Perioperative chemotherapy induces regression in both the primary tumor and lymph nodes. The results of the operative morbidity were similar to those described in the literature. However, although the group of good responders showed better survival, this value was not significant. Therefore, further studies are needed to evaluate the importance of the degree of lymph node regression and its impact on the survival of these patients.

RESUMO RACIONAL: O cancer gástrico é uma neoplasia com mau prognóstico. A abordagem multimodal com quimioterapia-perioperatória é o tratamento recomendado para os pacientes com cancer gástrico localmente avançando. Este tratamento induz uma resposta histopatológica manifestado pelo grau de regressão do tumor primário, dos gânglios linfáticos e do estadiamento ypTNM. Apesar de suas vantagens, ainda há dúvidas quanto aos efeitos da quimioterapia na morbimortalidade pós-operatória. OBJETIVOS: Avaliar o impacto da quimioterapia perioperatória e o seu efeito nos resultados anatomopatológicos, na morbidade pós-operatória e na sobrevida. MÉTODOS: Estudo observacional-retrospetivo com 134 doentes com cancer gástrico avançado, que se submeteram a quimioterapia perioperatória e cirurgia radical curativa. O grau de regressão histológico do tumor primário foi avaliado de acordo com os critérios de Becker. A proporção de gânglios regredidos foi determinada e as complicações pós-operatórias foram avaliadas com a classificação de Clavien-Dindo. Os tempos de sobrevida foram comparados entre os grupos por meio das curvas de Kaplan-Meier e do teste Mantel-Cox Log Rank. RESULTADOS: 22,3% dos doentes foram classificados como bons-respondedores e 75,9% como maus-respondedores. Esta variável e a morbidade pós-operatória não estavam relacionadas. 64,2% dos doentes apresentaram invasão ganglionar e 46,3% tinham regressão ganglionar, 49,4% não tinham invasão linfática e 61,9% não tinham sinais de invasão venosa. As complicações pós-operatórias ocorreram em 30,6% dos pacientes. O grupo dos bons respondedores apresentou uma sobrevida mediana de 56,0 meses e o grupo dos maus respondedores 34,0 meses. CONCLUSÕES: A quimioterapia perioperatória induz a regressão quer do tumor primário e dos gânglios-linfáticos. Os resultados da morbidade pós-operatória foram semelhantes aos descritos na literatura. Apesar do grupo dos bons-respondedores apresentar melhor sobrevida, este valor não foi significativo. Assim, são necessários mais estudos que avaliem a importância do grau de regressão ganglionar e o seu impacto na sobrevida.

Pediatric dermatoses pattern at a Brazilian reference center

Abstract Objective: The aim of this study was to identify the pattern of pediatric dermatoses of patients evaluated at a dermatologic clinic of a reference center in Brazil and to compare these results to similar surveys conducted in other countries. Methods: A retrospective study was performed of patients up to 18 years old, evaluated at a dermatologic clinic between January 1, 2017 and December 31, 2017. Variables collected for analysis included age, gender, dermatological diagnosis, multidisciplinary follow-up, hospitalization, and complementary exams. Results: A total of 2330 patients were included for analysis, with a mean age of 9.7 years. 295 patients were diagnosed with more than one skin disease, leading to a total of 2668 diagnoses. Skin diseases were organized into categories and inflammatory dermatoses corresponded to the largest group (31.2%), mostly due to atopic dermatitis (18.3%). The other main categories were: genodermatoses (14.2%), infectious diseases (12.6%), adnexal disorders (12.5%), cysts and neoplasms (10.7%), and vascular disorders (7.0%). Fifty-six patients needed to be admitted to the dermatology ward; 25 of them (44.6%) for management of worsening of the skin disease, mainly atopic dermatitis, psoriasis, and drug reactions. There were 885 biopsies performed in 38.0% of the subjects and 751 patients (32.2%) required multidisciplinary care; most of them had some genodermatoses. Conclusions: Dermatologic disorders are very common in the pediatric age group and differ from those in adults, suffering influence from cultural, ethnic, socioeconomic, and environmental factors. Knowing the magnitude and distribution of these dermatoses is important to better plan healthcare policies.

CHILD syndrome: successful treatment of skin lesions with topical lovastatin and cholesterol lotion

Abstract: CHILD syndrome (Congenital Hemidysplasia, Ichthyosiform erythroderma, Limb Defects) is a rare X-linked dominant disease. The authors report a 2-month-old patient presenting with typical features of CHILD syndrome that was treated with a topical solution containing cholesterol and lovastatin, with complete clearance of her CHILD nevus. The changes in skin lipid metabolism that explain the CHILD ichthyosiform nevus and their correction through topical application of cholesterol and lovastatin are discussed.

Consensus on the treatment of hidradenitis suppurativa - Brazilian Society of Dermatology

Abstract: Hidradenitis suppurativa is a chronic immune mediated disease of universal distribution that causes great damage to the quality of life of the affected individual, whose prevalence is estimated at 0.41% in the Brazilian population. The objective of this work was update on physiopathogenesis, diagnosis and classification of hidradenitis suppurativa and to establish therapeutic recommendations in the Brazilian reality. It was organized as a work group composed of eight dermatologists from several institutions of the country with experience in the treatment of hidradenitis suppurativa and carried out review on the topic. Recommendations were elaborated and voted by modified Delphi system and statistical analysis of the results was performed. The Brazilian consensus on the clinical approach of hidradenitis suppurativa had the support of the Brazilian Society of Dermatology.

Consensus on the diagnostic and therapeutic management of chronic spontaneous urticaria in adults - Brazilian Society of Dermatology

Abstract: Background: Urticarias are frequent diseases, with 15% to 20% of the population presenting at least one acute episode in their lifetime. Urticaria are classified in acute ( ≤ 6 weeks) or chronic (> 6 weeks). They may be induced or spontaneous. Objectives: To verify the diagnostic and therapeutic recommendations in chronic spontaneous urticaria (CSU), according to the experience of Brazilian experts, regarding the available guidelines (international and US). Methods: A questionnaire was sent to Brazilian experts, with questions concerning diagnostic and therapeutic recommendations for CSU in adults. Results: Sixteen Brazilian experts answered the questionnaire related to diagnosis and therapy of CSU in adults and data were analyzed. Final text was written, considering the available guidelines (International and US), adapted to the medical practices in Brazil. Diagnostic work up in CSU is rarely necessary. Biopsy of skin lesion and histopathology may be indicated to rule out other diseases, such as, urticarial vasculitis. Other laboratory tests, such as complete blood count, CRP, ESR and thyroid screening. Treatment of CSU includes second-generation anti-histamines (sgAH) at licensed doses, sgAH two, three to fourfold doses (non-licensed) and omalizumab. Other drugs, such as, cyclosporine, immunomodulatory drugs and immunosuppressants may be indicated (non-licensed and with limited scientific evidence). Conclusions: Most of the Brazilian experts in this study partially agreed with the diagnostic and therapeutic recommendations of the International and US guidelines. They agreed with the use of sgAH at licensed doses. Increase in the dose to fourfold of sgAH may be suggested with restrictions, due to its non-licensed dose. Sedating anti-histamines, as suggested by the US guideline, are indicated by some of the Brazilian experts, due to its availability. Adaptations are mandatory in the treatment of CSU, due to scarce or lack of other therapeutic resources in the public health system in Brazil, such as omalizumab or cyclosporine.

Consensus on the therapeutic management of atopic dermatitis - Brazilian Society of Dermatology

Abstract: BACKGROUND: Atopic dermatitis is a highly prevalent inflammatory and pruritic dermatosis with a multifactorial etiology, which includes skin barrier defects, immune dysfunction, and microbiome alterations. Atopic dermatitis is mediated by genetic, environmental, and psychological factors and requires therapeutic management that covers all the aspects of its complex pathogenesis. OBJECTIVES: The aim of this article is to present the experience, opinions, and recommendations of Brazilian dermatology experts regarding the therapeutic management of atopic dermatitis. METHODS: Eighteen experts from 10 university hospitals with experience in atopic dermatitis were appointed by the Brazilian Society of Dermatology to organize a consensus on the therapeutic management of atopic dermatitis. The 18 experts answered an online questionnaire with 14 questions related to the treatment of atopic dermatitis. Afterwards, they analyzed the recent international guidelines on atopic dermatitis of the American Academy of Dermatology, published in 2014, and of the European Academy of Dermatology and Venereology, published in 2018. Consensus was defined as approval by at least 70% of the panel. RESULTS/CONCLUSION: The experts stated that the therapeutic management of atopic dermatitis is based on skin hydration, topical anti-inflammatory agents, avoidance of triggering factors, and educational programs. Systemic therapy, based on immunosuppressive agents, is only indicated for severe refractory disease and after failure of topical therapy. Early detection and treatment of secondary bacterial and viral infections is mandatory, and hospitalization may be needed to control atopic dermatitis flares. Novel target-oriented drugs such as immunobiologicals are invaluable therapeutic agents for atopic dermatitis.

Adult female acne: a guide to clinical practice

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.

Tuberous sclerosis complex: review based on new diagnostic criteria

Abstract: Tuberous sclerosis complex is a multisystemic, autosomal dominant genetic disorder with complete penetrance, that can evolve with hamartomas in multiple organs, such as skin, central nervous system, kidney and lung. Due to the wide phenotypic variability, the disease is often not recognized. Tuberous sclerosis complex affects one in 10,000 newborns and most patients are diagnosed during the first 15 months of life. The diagnostic criteria for tuberous sclerosis were reviewed in 2012, at the second International Tuberous Sclerosis Complex Consensus Conference. The diagnosis is based on genetic criteria, by the identification of inactivating pathogenic mutation of tumor suppressor genes TSC1 and TSC2, and clinical criteria, including cutaneous, renal, pulmonary, cardiac and neurological manifestations. The treatment of tuberous sclerosis complex consists, mainly, in management of the symptoms caused by hamartomas and in prevention of organ failure. Multidisciplinary approach is recommended, in order to obtain better clinical outcomes.

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Abstract: Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

Avaliação da aplicabilidade do modelo de Gail como preditor de risco de câncerde mama em mulheres baianas / Evaluation of the applicability of Gail model as a predictor of breast cancer risk in women from Bahia

Objetivo: Avaliar a acurácia do modelo de Gail em mulheres baianas. Métodos: Estudo de casocontrole, com 64 casos e 64 controles. Este estudo foi conduzido em duas clínicas de Salvador, Bahia, que atendem pacientes conveniados aos planos de saúde: o Núcleo de Oncologia da Bahia e o Núcleo da Mama. Foram calculados dois valores do índice de Gail para cada paciente, com etnia referida e com etnia desconhecida. Os valores encontrados foram comparados entre os grupos caso e controle, comanálise de significância pelo teste t de Student. Também foi calculado qual o percentual de pacientes do grupo caso que apresentavam alto (?1,67%) e baixo risco (<1,67%). Resultados: As  médias dos valores do índice de Gail do grupo controle foram superiores às médias dos valores nos casos,independente de qual risco foi avaliado (em cinco anos ou ?life-time?) e da etnia utilizada (autoreferida ou desconhecida). Quando utilizada a etnia auto-referida, apenas 26,7% das pacientes com câncer de mama teriam sido identificadas como alto risco pelo modelo, e, quando utilizada a etniadesconhecida, apenas 34,7%. Conclusão: Na presente amostra com mulheres baianas, o modelo de Gail não foi um bom preditor para o desenvolvimento de câncer de mama.

Purpose: To evaluate the accuracy of Gail model applied to women from Bahia. Methods: It is a casecontrol study, with 64 cases and 64 controls. This study was lead in two clinics in Salvador, Bahia, Brazil, that attend patients with health insurance: Núcleo de Oncologia da Bahia and Núcleo da Mama. Two values of Gail model were calculated for each patient, one using self-reported ethnicity and other using ethnicity as unknown. The values were compared between case and control groups, using significance analysis by Student t test. It was also calculated which percentage of patients in case group were at high-risk (?1.67%) or low risk (<1.67). Results: The mean values of the Gail model was higher in the control group than the mean values in the case group, regardless of which risk was assessed (in five years or life-time) and of ethnicity was used (self-reported or unknown). When used the self-reported ethnicity for calculation, only 26.7% of women with breast cancer would have been identified as high-risk by the Gail model, and when used unknown ethnicity, only 34.7% of these would have been identified. Conclusion: In the present sample, the Gail model was nota good predictor for the development of breast cancer in womens from Bahia.

Perfil epidemiológico dos subtipos moleculares de carcinoma ductal da mama em população de pacientes em Salvador, Bahia / Epidemiological profile of molecular subtypes of breast ductal carcinoma in patients population in Salvador, Bahia

Objetivo: Avaliar o perfil dos subtipos moleculares do câncer de mama em uma população de pacientes de duas clínicas privadas de Salvador, Bahia: o Núcleo da Mama e o Núcleo de Oncologia da Bahia. Metodologia: Estudo de corte transversal que incluiu pacientes do sexo feminino com diagnóstico aná- tomo-patológico de carcinoma invasivo sem tipo especial (ductal invasor). Foram excluídas as pacientes com história de câncer prévio, exceto câncer de pele não melanoma; com dados clínicos anteriores ao tratamento sistêmico indisponíveis e sem laudo de imuno-histoquímica. Foi realizada classificação do câncer de mama com base no subtipo molecular em quatro padrões principais: luminal A, luminal B, HER2+ e triplo-negativo. Frequências e proporções foram estabelecidas para cada um dos subtipos moleculares. Outros dados clínicos e laboratoriais também foram colhidos para estabelecer o perfil da população estudada. Resultados: Foram recrutadas 59 pacientes entre dezembro de 2012 e maio de 2014, com idade média de 56,9 anos. O subtipo molecular mais comum na amostra foi o luminal B, com 35 casos (59,3%), seguido do luminal A, (11 casos; 18,6%), triplo-negativo (6 casos, 10,2%) e HER2+ (7 casos; 11,9%). Conclusões: Corroborando outros estudos realizados em população brasileira, o subtipo luminal B é o mais comum encontrado nesta amostra de pacientes de Salvador, Bahia. O achado ajuda a confirmar o padrão atípico do país em relação ao descrito na literatura internacional, com importantes implicações terapêuticas e prognósticas.

Objective: The aim of this study is to evaluate breast cancer molecular subtype distribution in a female population of two private centers in Salvador, Bahia, Brazil: Núcleo da Mama e o Núcleo de Oncologia da Bahia. Methodology: This is a cross-sectional study involving female patients with pathological diagnosis of invasive ductal carcinoma not otherwise specified (NOS). Patients with history of previous cancer except non-melanoma skin câncer; clinical data prior to systemic treatment unavailable and those without immunohistochemical report were excluded from the study. Breast cancer classification was based on four main molecular subtype patterns: luminal A, luminal B, HER2 + and triple negative. Frequencies and proportions were established for each of the molecular subtypes. Other clinical and laboratory data were also collected to determine baseline characteristics of the population studied. Results: We recruited 59 patients between December 2012 and May 2014 with an average age of 56.9 years. The most common molecular subtype of the sample was luminal B subtype, with 35 cases (59.3%), followed by luminal A (11 cases; 18.6%), triple negative (6 cases, 10.2%) and HER2+ (7 cases; 11.9%). Conclusions: Similar to other studies with Brazilian population samples, luminal B subtype is the most common in this sample of patients in Salvador, Bahia. The finding helps confirm the atypical pattern of the country in relation to that described in international literature, with important therapeutic and prognostic implications.

Avaliação da satisfação profissional de funcionários em um hospital público de reconhecida efetividade assistencial / Evaluation of job satisfaction of employees of a public hospital care recognized effectiveness

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. .

Propranolol for treatment of infantile hemangiomas / Tratamento de hemangiomas infantis com propranolol

Infantile hemangioma is the most common vascular tumor in early childhood. Propranolol has been successfully used recently in a limited number of children with Infantile hemangioma. We present 6 cases of Infantile hemangioma, at a single dermatological center, which responded to oral propranolol with good results.

O hemangioma da infância é o tumor vascular mais comum nessa faixa etária. Mais recentemente, propranolol oral tem sido usado com sucesso em um número limitado de crianças com hemangioma da infância. Nós apresentamos 6 casos de hemangioma da infância, provenientes de um único centro dermatológico, que apresentaram boa resposta ao tratamento com propranolol oral.

A study of pent-up demand in ophthalmology: Divinolândia Hospital/Unicamp / Estudo da demanda reprimida em Oftalmologia: hospital de Divinolândia/Unicamp

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: 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.