Infantile generalized hypertrichosis caused by topical minoxidil.

Rare cases of hypertrichosis have been associated with topically applied minoxidil. We present the first reported case in the Brazilian literature of generalized hypertrichosis affecting a 5-year-old child, following use of minoxidil 5%, 20 drops a day, for hair loss. The laboratory investigation excluded hyperandrogenism and thyroid dysfunction. Topical minoxidil should be used with caution in children.

Pitted keratolysis.

Pitted keratolysis is a skin disorder that affects the stratum corneum of the plantar surface and is caused by Gram-positive bacteria. A 30-year-old male presented with small punched-out lesions on the plantar surface. A superficial shaving was carried out for scanning electron microscopy. Hypokeratosis was noted on the plantar skin and in the acrosyringium, where the normal elimination of corneocytes was not seen. At higher magnification (x 3,500) bacteria were easily found on the surface and the described transversal bacterial septation was observed.

Infantile generalized hypertrichosis caused by topical minoxidil

Abstract Rare cases of hypertrichosis have been associated with topically applied minoxidil. We present the first reported case in the Brazilian literature of generalized hypertrichosis affecting a 5-year-old child, following use of minoxidil 5%, 20 drops a day, for hair loss. The laboratory investigation excluded hyperandrogenism and thyroid dysfunction. Topical minoxidil should be used with caution in children.

Pitted keratolysis

Abstract Pitted keratolysis is a skin disorder that affects the stratum corneum of the plantar surface and is caused by Gram-positive bacteria. A 30-year-old male presented with small punched-out lesions on the plantar surface. A superficial shaving was carried out for scanning electron microscopy. Hypokeratosis was noted on the plantar skin and in the acrosyringium, where the normal elimination of corneocytes was not seen. At higher magnification (x 3,500) bacteria were easily found on the surface and the described transversal bacterial septation was observed.

Three-dimensional aspects of superficial disseminated porokeratosis with scanning electron microscopy

The three-dimensional findings of the surface and from a cross section from a case of disseminated superficial porokeratois using scanning electron microscopy are reported. On the surface of the skin, irregular keratin with a serpiginous distribution was seen. A gross aspect of keratin in the hyperkeratotic wall was also observed and compared to the normal area, in which the release of corneocytes seemed normal. The cross-sectional imaging easily identified the cornoid lamella, with compact keratin surrounded by normal stratum corneum.

Three-dimensional aspects of superficial disseminated porokeratosis with scanning electron microscopy.

The three-dimensional findings of the surface and from a cross section from a case of disseminated superficial porokeratois using scanning electron microscopy are reported. On the surface of the skin, irregular keratin with a serpiginous distribution was seen. A gross aspect of keratin in the hyperkeratotic wall was also observed and compared to the normal area, in which the release of corneocytes seemed normal. The cross-sectional imaging easily identified the cornoid lamella, with compact keratin surrounded by normal stratum corneum.

Perception of disease severity in adult patients with cystic fibrosis.

OBJECTIVE: To evaluate the perception of disease severity in patients with cystic fibrosis (CF), investigating its relationship with clinical score, radiographic score, respiratory function tests, adherence to treatment and perception of self-care practices. METHODS: Prospective, cross-sectional study involving CF patients treated in a program for adults with CF. The perception of disease severity, adherence to treatment and reported self-care practices were evaluated by means of questionnaires. Clinical data, Shwachman-Kulczycki clinical score, Brasfield radiographic score and spirometry were obtained for all of the patients. RESULTS: Of the 38 patients studied, 3 (7.9%) patients rated their perception of health status as well below average; 5 (13.2%), as below average; 15 (39.5%), as average; 10 (26.3%), as above average; and 5 (13.2%), as well above average. The perception of disease severity correlated significantly with clinical score (r = 0.43, p = 0.007), FVC (r = 0.34, p = 0.034), FEV1 (r = 0.38, p = 0.019) and self-care practices (r = 0.33, p = 0.044), but not with degree of adherence (r = -0.03, p = 0.842) and radiographic score (r = 0.33, p = 0.51). CONCLUSIONS: The perception of disease severity correlated with objective measurements of disease severity (clinical score and respiratory function tests) and with reported self-care practices, but not with adherence to treatment.

Percepção da gravidade da doença em pacientes adultos com fibrose cística / Perception of disease severity in adult patients with cystic fibrosis

OBJETIVO: Avaliar a percepção da gravidade da doença em pacientes com fibrose cística (FC), investigando sua relação com escore clínico, escore radiológico, testes de função pulmonar, adesão ao tratamento e percepção de autocuidado. MÉTODOS: Estudo transversal, prospectivo, envolvendo pacientes com FC atendidos em um programa para adultos com FC. A percepção da gravidade da doença, a adesão ao tratamento e o relato de autocuidado foram avaliados por questionários. Foram obtidos de todos os pacientes dados clínicos, escore clínico de Shwachman-Kulczycki, escore radiológico de Brasfield e espirometria. RESULTADOS: De 38 pacientes estudados, 3 (7,9 por cento) relataram percepção de sua saúde como muito abaixo da média; 5 (13,2 por cento), como abaixo da média; 15 (39,5 por cento), como na média; 10 (26.3 por cento), como acima da média; e 5 (13,2 por cento), como muito acima da média. A percepção da gravidade da doença correlacionou-se significativamente com o escore clínico (r = 0,43, p = 0,007), CVF (r = 0,34, p = 0,034), VEF1 (r = 0,38, p = 0,019) e com relato de autocuidado (r = 0,33, p = 0,044), mas não com o grau de adesão (r = -0,03, p = 0,842) e escore radiológico (r = 0,33, p = 0,51). CONCLUSÕES: A percepção da gravidade da doença se relacionou com medidas objetivas de gravidade da doença (escore clínico e testes de função pulmonar) e com relato de autocuidado, mas não com a adesão ao tratamento.

OBJECTIVE: To evaluate the perception of disease severity in patients with cystic fibrosis (CF), investigating its relationship with clinical score, radiographic score, respiratory function tests, adherence to treatment and perception of self-care practices. METHODS: Prospective, cross-sectional study involving CF patients treated in a program for adults with CF. The perception of disease severity, adherence to treatment and reported self-care practices were evaluated by means of questionnaires. Clinical data, Shwachman-Kulczycki clinical score, Brasfield radiographic score and spirometry were obtained for all of the patients. RESULTS: Of the 38 patients studied, 3 (7.9 percent) patients rated their perception of health status as well below average; 5 (13.2 percent), as below average; 15 (39.5 percent), as average; 10 (26.3 percent), as above average; and 5 (13.2 percent), as well above average. The perception of disease severity correlated significantly with clinical score (r = 0.43, p = 0.007), FVC (r = 0.34, p = 0.034), FEV1 (r = 0.38, p = 0.019) and self-care practices (r = 0.33, p = 0.044), but not with degree of adherence (r = -0.03, p = 0.842) and radiographic score (r = 0.33, p = 0.51). CONCLUSIONS: The perception of disease severity correlated with objective measurements of disease severity (clinical score and respiratory function tests) and with reported self-care practices, but not with adherence to treatment.

Adesão ao tratamento em pacientes com fibrose cística / Adherence to treatment in patients with cystic fibrosis

OBJETIVOS: Avaliar a adesão auto-relatada ao tratamento na fibrose cística (FC), estabelecendo associações com características da doença e com a adesão percebida pelos profissionais de saúde. MÉTODOS: Foi realizado estudo transversal, prospectivo, com pacientes atendidos por um programa para adultos com FC. O grau da adesão foi avaliado por questionário. Os pacientes foram divididos em grupo com elevada adesão e grupo com moderada/baixa adesão. Foram obtidos dados clínicos, medida do escore clínico de Shwachman-Kulczycki, medida do escore radiológico de Brasfield e espirometria. RESULTADOS: De 38 pacientes estudados, 31 (81,6 por cento) foram classificados como tendo elevada adesão e 7 (18,4 por cento) como moderada/baixa adesão. Houve correlação entre o escore de adesão auto-relatada e o escore clínico (r = -0,36, p = 0,028). O escore de adesão auto-relatada foi maior (mediana = 0,79) que o percebido pelo profissional (mediana = 0,71, p = 0,003). Adesão elevada foi auto-relatada em 84,2 por cento para a fisioterapia respiratória, em 21,1 por cento para a atividade física, em 65,8 por cento para dieta, em 96,3 por cento para enzimas pancreáticas, em 79,4 por cento para as vitaminas, em 76,7 por cento para o antibiótico inalatório e em 79,4 por cento para a dornase alfa. CONCLUSÕES: A adesão auto-relatada dos pacientes atendidos por um programa de adultos com FC foi elevada. Menor adesão foi observada com a dieta e a atividade física. O escore de adesão auto-relatada se correlacionou inversamente com o escore clínico. A adesão auto-relatada foi maior que a percebida pelos profissionais.

OBJECTIVES: To evaluate the self-reported degree of adherence to treatment in patients with cystic fibrosis (CF), investigating associations with characteristics of the disease and with the degree of adherence perceived by health professionals. METHODS: This was a prospective, cross-sectional study involving patients with CF monitored at a Program for Adults with CF. The degree of adherence was evaluated using a questionnaire. Patients were divided into two groups: greater degree of adherence and moderate/poor degree of adherence. Clinical data, Shwachman-Kulczycki clinical score, Brasfield radiographic score and spirometry data were obtained for all patients. RESULTS: Out of 38 patients studied, 31 (81.6 percent) were classified as presenting a greater degree of adherence and 7 (18.4 percent) as presenting a moderate/poor degree of adherence. The self-reported patient adherence score correlated with the clinical score (r = -0.36, p = 0.028). The self-reported patient adherence score (median = 0.79) was higher than that perceived by health professionals (median = 0.71, p = 0.003). A greater degree of adherence was self-reported for respiratory therapy (by 84.2 percent), exercise (by 21.1 percent), prescribed diet (by 65.8 percent), pancreatic enzymes (by 96.3 percent), vitamins (by 79.4 percent), inhaled antibiotic therapy (by 76.7 percent) and inhaled DNase (by 79.4 percent). CONCLUSIONS: Self-reported adherence of patients attending a Program for Adults with CF was good. The self-reported patient adherence score correlated inversely with the clinical score. Self-reported patient adherence was greater than the adherence perceived by health professionals.

[Adherence to treatment in patients with cystic fibrosis]. / Adesão ao tratamento em pacientes com fibrose cística.

OBJECTIVES: To evaluate the self-reported degree of adherence to treatment in patients with cystic fibrosis (CF), investigating associations with characteristics of the disease and with the degree of adherence perceived by health professionals. METHODS: This was a prospective, cross-sectional study involving patients with CF monitored at a Program for Adults with CF. The degree of adherence was evaluated using a questionnaire. Patients were divided into two groups: greater degree of adherence and moderate/poor degree of adherence. Clinical data, Shwachman-Kulczycki clinical score, Brasfield radiographic score and spirometry data were obtained for all patients. RESULTS: Out of 38 patients studied, 31 (81.6%) were classified as presenting a greater degree of adherence and 7 (18.4%) as presenting a moderate/poor degree of adherence. The self-reported patient adherence score correlated with the clinical score (r = -0.36, p = 0.028). The self-reported patient adherence score (median = 0.79) was higher than that perceived by health professionals (median = 0.71, p = 0.003). A greater degree of adherence was self-reported for respiratory therapy (by 84.2%), exercise (by 21.1%), prescribed diet (by 65.8%), pancreatic enzymes (by 96.3%), vitamins (by 79.4%), inhaled antibiotic therapy (by 76.7%) and inhaled DNase (by 79.4%). CONCLUSIONS: Self-reported adherence of patients attending a Program for Adults with CF was good. The self-reported patient adherence score correlated inversely with the clinical score. Self-reported patient adherence was greater than the adherence perceived by health professionals.

Doppler echocardiogram, oxygen saturation and submaximum capacity of exercise in patients with cystic fibrosis.

STUDY OBJECTIVES: To determine the relationship between pulmonary arterial systolic pressure (PASP) and submaximum capacity of exercise, using the six-minute walk test (6MWT) in patients with cystic fibrosis (CF), and to investigate the relation between echocardiographic findings and results of 6MWT, clinical scores, chest radiograph scores and lung function tests. DESIGN: This was a prospective cross-sectional study in patients with CF (16 years and older) with clinical stability, attending the Adult CF Program at the Hospital de Clínicas de Porto Alegre. The patients had Doppler echocardiography and performed a 6MWT. As well as pulmonary function tests and chest roentgenograms, and a clinical score was obtained for all patients. RESULTS: The study included 39 patients with a mean age of 23.7+/-6.3 years. There were no significant correlation between the distance walked and PASP, diameter of the right ventricle (DRV) and pulmonary acceleration time (p>0.05). We observed a significant correlation among PASP and the SpO(2) at rest (r=-0.73; p<0.001), SpO(2) at the end of the 6MWT (r= -0.45; p=0.006), clinical score (r= -0.55; p=0.001), chest radiograph score (r= -0.33; p=0.049), FEV1 (r= -0.63; p< 0.001), and FVC (r=-0.55; p=0.001). Right ventricular outflow tract flow acceleration time (Ac T) was significantly correlated only with the FEV1 (r=0.32; p=0.047). RVD was significantly correlated with SpO(2) at rest (r= -0.44; p=0.005) and clinical score (r= -0.38; p=0.017). The SpO(2) at rest was the single best predictor of PASP and this effect was independent of the relationship between other independent variables (p=0.001). The declining pulmonary function was significantly associated with PASP (p<0.001), SpO(2) at rest (p=0.001), SpO(2) at the end of the 6MWT (p=0.007) and difference between peripheral oxygen saturation at resting and at the end of the 6MWT (p=0.025). CONCLUSION: The PASP was not significantly correlated with the distance walked during the 6MWT in patients with CF. The PASP was strongly correlated with oxygen status at rest. The SpO(2) at rest was the best predictor of PASP. Also, PASP was strongly correlated with Shwachman-Kulczycki score, FEV(1), and FVC in this population.