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Background: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction., Objectives: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia., Materials and Methods: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1-430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4-333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed., Results: Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9-25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2-10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6-75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications., Conclusion: Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.

Background: There is paucity of data on long-term probability of remission in chronic idiopathic thrombocytopenic purpura (ITP). Aim was to study the course and factors influencing remission of chronic ITP. Chronic ITP was defined as thrombocytopenia persisting >6 months following initial diagnosis., Procedures: Case-records of children with chronic ITP, aged <14 years, were reviewed in this retrospective study (1987-2006)., Results: Two hundred seventy children were followed. Median age at diagnosis was 6 years. Median duration of follow up was 30 months (range 6-166). Isolated thrombocytopenia (even if <10 x 10(9)/L) in the absence of "significant" bleeds, by itself was not considered an indication for drug therapy. Sixty-seven (24.8%) children attained complete remission (CR) over a median period of 18 months (range 7-120). The probabilities of remission at 5 years for males and females were 24% and 39.6%, respectively (P = 0.01). The probability of achieving remission at 10 years in children <8 and > or =8 years was 51.2% and 34%, respectively (P = 0.02). The probability of remission at 5 years for children who received some treatment, versus no treatment was 31.4% and 27%, respectively (P = 0.8). Nine of 18 children, who underwent splenectomy, achieved CR. Intracranial hemorrhage (ICH) occurred in 11 (4%) cases. The time of occurrence of ICH from onset of symptoms varied from 6 to 55 months., Conclusions: The predicted spontaneous remission rate with chronic ITP was 30% and 44% at 5 and 10 years, respectively. Platelet count at diagnosis and the treatment administered did not influence remission outcomes. Age <8 years and female gender were predictors of a favorable outcome., (Copyright 2009 Wiley-Liss, Inc.)