Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience.

PURPOSE: Although achalasia is a rare disorder in children, its symptom may mimic common childhood diseases. This study aimed to assess the diagnosis and management of achalasia in children and adolescents in a Brazilian single center during a 12-year period. METHODS: Patients with achalasia were identified from a database built during the period of January 2000-January 2012 from a Pediatric Gastroenterology reference center. Information regarding demographic data, clinical symptoms, diagnosis, treatment, and long-term follow-up were described. RESULTS: Thirteen patients were studied; median age was 7 (1-14) years. Most frequent symptoms were vomiting (84.6 %) and dysphagia (69.2 %). Weight loss occurred in 46.0 % of patients and chronic cough in 46.1 %. Associated disorders were Down's syndrome, Allgrove syndrome, and congenital central hypoventilation syndrome. Achalasia was misdiagnosed with anorexia nervosa. Six patients were previously treated as having gastroesophageal reflux disease and asthma. Five patients had pneumatic balloon dilation as initial therapy whereas five had esophageal myotomy. Finally, 11 patients had surgical therapy with a favorable follow-up. CONCLUSION: Achalasia symptoms may mimic common diseases in children, and therefore, may delay the diagnosis. This study emphasizes the importance of the clinical symptoms for the diagnosis of achalasia, mainly in those cases with associated disorders.

Transforming growth factor beta1 (TGF-ß1) levels in a rat model of induced pleural empyema.

PURPOSE: To evaluate the concentration of transforming growth factor beta 1 (TGFB1) levels in a rat pleural effusion obtained by inoculation of intrapleural bacteria or turpentine through thoracentesis. METHODS: Thirty-Nine Wistar rats were divided into three groups: Staphylococcus aureus (SA, n = 17); Streptococcus pneumoniae (SP, n = 12); and turpentine (control, n = 10). Pleural fluid was collected through ultrasound-guided thoracentesis 12 h, 24 h, and 36 h after instillation of bacteria or turpentine. Levels of TGFB1 were measured in pleural fluid. RESULTS: At 12 h, mean TGFB1concentrations were 5.3450 pg/mL in the SA group, 5.3449 pg/mL in the SP group, and 5.3450 pg/mL in controls. At 24 h, they were 4.6700 pg/mL in the SA group, 4.6700 pg/mL in the SP group, and 4.6700 pg/mL in controls. At 36 h, they were 4.6699 pg/mL in the SA group and in control. No difference was observed among the groups in mean TGFB1concentration (p = 0.12); however, a significant intragroup reduction in mean TGFB1 was observed between 12 and 24 h (p < 0.01). CONCLUSION: The transforming growth factor beta 1 concentrations were not useful as a diagnostic tool or an early marker of infected pleural effusion.

Accuracy of complement activation product levels to detect infected pleural effusion in rats.

BACKGROUND: Pleural empyema is a well-known complication of pneumonia. If treatment is delayed, empyema may increase morbidity and mortality in affected patients. Therefore, the identification of empyema biomarkers in parapneumonic pleural effusion is desirable. Previous research has suggested complement activation products as candidate empyema markers. OBJECTIVE: To compare the levels of complement activation products C3a, C5a, and C5b9 in pleural effusion induced by Staphylococcus aureus (SA), Streptococcus pneumoniae (SP), or turpentine (control). METHODS: Thirty-nine male Wistar rats (mean weight 414 g; 290-546 g) were allocated as follows: 17 animals in the SA group, 12 in the SP group, and 10 in the control group. Bacteria or turpentine were injected into the pleural space. After 12 hr, intrapleural fluid was collected using ultrasound-guided thoracentesis. Levels of complement activation products were determined using ELISA kits. RESULTS: Two SA and one SP animals died before 12 hr. Mean levels were as follows: C3a: 1066.82 µg/ml (937.29-1196.35 µg/ml) in SA, 1188.28 µg/ml (1095.65-1280.92 µg/ml) in SP, and 679.13 µg/ml (601.29-756.98 µg/ml) in controls (P < 0.001); C5a: 55.727 ng/ml (41.22-70.23 ng/ml) in SA, 520.107 ng/ml (278.92-761.3 ng/ml) in SP, and 5.268 ng/ml (1.68-8.85 ng/ml) in controls (P < 0.001); C5b9: 15.02 ng/ml (13.1-16.94 ng/ml) in SA, 16.63 ng/ml (14.37-18.9 ng/ml) in SP, and 14.05 ng/ml (9.8-18.29 ng/ml) in controls (P = 0.692). ROC analysis revealed an area under the curve of 0.987 (95% CI: 0.953-1) for C3a; 1 (1-1) for C5a; and 0.757 for C5b9 (0.523-0.990). CONCLUSIONS: In the present rat model, complement activation fragments C3a and C5a accurately detected infected pleural effusion. Pediatr Pulmonol. 2017;52:757-762. © 2017 Wiley Periodicals, Inc.

Transforming growth factor beta1 (TGF-ß1) levels in a rat model of induced pleural empyema

Abstract Purpose: To evaluate the concentration of transforming growth factor beta 1 (TGFB1) levels in a rat pleural effusion obtained by inoculation of intrapleural bacteria or turpentine through thoracentesis. Methods: Thirty-Nine Wistar rats were divided into three groups: Staphylococcus aureus (SA, n = 17); Streptococcus pneumoniae (SP, n = 12); and turpentine (control, n = 10). Pleural fluid was collected through ultrasound-guided thoracentesis 12 h, 24 h, and 36 h after instillation of bacteria or turpentine. Levels of TGFB1 were measured in pleural fluid. Results: At 12 h, mean TGFB1concentrations were 5.3450 pg/mL in the SA group, 5.3449 pg/mL in the SP group, and 5.3450 pg/mL in controls. At 24 h, they were 4.6700 pg/mL in the SA group, 4.6700 pg/mL in the SP group, and 4.6700 pg/mL in controls. At 36 h, they were 4.6699 pg/mL in the SA group and in control. No difference was observed among the groups in mean TGFB1concentration (p = 0.12); however, a significant intragroup reduction in mean TGFB1 was observed between 12 and 24 h (p < 0.01). Conclusion: The transforming growth factor beta 1 concentrations were not useful as a diagnostic tool or an early marker of infected pleural effusion.

Hamartoma fibroso infantil – tumor raro em crianças / Fibrous hamartoma of infancy: a rare tumor in children

O Hamartoma Fibroso Infantil (HFI) é um tumor raro de característica benigna com representação histológica trifásica própria. Acomete tipicamente crianças de até 2 anos, predominantemente do sexo masculino. Apresenta usualmente diâmetro menor de 5 cm, ocorrendo classicamente em áreas como a parede torácica, braços, axilas e região inguinal, porém já foram descritos acometimentos de estruturas genitais, cabeça, pescoço e estruturas distais. Em sua avaliação, patologias como fibrolipoma, lipoblastoma, fibrosarcoma e rabdomiosarcoma devem sempre ser consideradas como diagnóstico diferencial. Atualmente, em sua abordagem, exames de imagem como ultrassonografia e ressonância nuclear magnética vem tornando-se importantes ferramentas no processo investigativo e preparatório à terapêutica empregada, que baseia-se na excisão da lesão. Apresentamos a seguir um relato de caso de criança com 4 meses de idade com lesão expansiva em braço esquerdo diagnosticada como HFI após investigação, excisão e análise histopatológica.

Fibrous hamartoma of infancy (FHI) is a rare, benign tumor with a characteristic three-phase histological pattern. It typically affects children up to two years of age, predominantly male. It is usually smaller than 5 cm in diameter, occurring classically in areas such as the chest wall, arms, underarms and groin, but it may as well affect genital structures, head, neck and distal structures. In its evaluation, disorders like fibrolipoma, lipoblastoma, fibrosarcoma and rhabdomyosarcoma should always be considered as a differential diagnosis. Currently, in its approach, imaging tests such as ultrasound and magnetic resonance imaging have become important tools in the investigative and preparatory process for the treatment to be used, which is based on excision of the lesion. The following is a case report of a 4-month old with an expansive lesion in the left arm diagnosed as FHI after investigation, excision and histopathology.