When the 1996-2000 and 2001-2011 periods were compared, a significant reduction in CFR was observed in the latter, as well as a change in the clinical profile of the pediatric inpatients at the institute. These findings may be related to the improvement in the socio-economical status of the population. Penicillin use did not influence CFR.

Pulmonary assessment should be part of the preoperative investigation of pediatric patients with chronic liver disease undergoing liver transplantation, as it allows the identification of pulmonary alterations that influence candidacy for transplantation and survival. To describe pulmonary changes found in pediatric patients who were candidates for liver transplantation. Retrospective study of 17 pediatric liver transplant candidates undergoing preoperative pulmonary evaluation assessing pulmonary clinical data, arterial blood gas analysis, CXR, respiratory function test by spirometry, pulmonary scintigraphy, and CEE. Ten patients presented normal chest roentgenograms. The most common radiographic change was interstitial infiltrate in the lung bases. Of the five patients with PaO(2) <70 mmHg, four had cyanosis and dyspnea and two were diagnosed with HPS with intrapulmonary shunt evidenced by contrast echocardiogram. Two patients presented with intrapulmonary shunt but without hypoxemia. Spirometry was normal in six patients, restrictive disturbance was evidenced in one patient, obstructive in three, and combined in two. The most common scintigraphic change was heterogeneous pulmonary perfusion. Pulmonary assessment should be performed routinely in pediatric patients prior to liver transplantation, even in asymptomatic patients. Pulmonary assessment may indicate changes such as HPS that can increase postoperative morbidity/mortality.

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ResumoObjetivo: Alertar os pediatras para uma síndrome cuja principal característica é a anomalia da caixa torácica que evolui, geralmente, para insuficiência respiratória e óbito nos primeiros dois anos de vida.Métodos: Relato de três casos de pacientes com características clínicas e radiológicas compatíveis com distrofia torácica asfixiante (síndrome de Jeune): caixa torácica pequena, estreita e desenvolvimento anormal de costelas. Faz-se a seguir uma breve revisão da literatura.Resultados: Os três pacientes acompanhados encontram-se em idade pré-escolar, contrapondo-se à maioria dos casos da literatura, cujas evoluções são geralmente insatisfatórias. Até o momento não apresentaram complicações pulmonares graves ou insuficiência respiratória.Conclusões: Embora salientando a raridade da síndrome, esta deve ser suspeitada no diagnóstico diferencial das anomalias da caixa torácica. Ressalta-se o grau variável de acometimento pulmonar e evolução da doença, embora dados de literatura revelem, em sua maioria, mau prognóstico.J. pediatr. (Rio J.). 1998; 74(4):333-337-: distrofia torácica asfixiante, síndrome de Jeune, anomalias da caixa torácica. AbstractObjective: To drive pediatrician's attention to a syndrome wwich has as its typical clinical presentation a thoracic cage abnormality which evolves to respiratory distress and death during the first two years of life.Methods: We describe three cases of patients with clinical and radiographic findings characteristic of asphyxiating thoracic dystrophy (Jeune's Syndrome): short and narrow thoracic cage and abnormal development of the ribs. A brief review of the literature on the subject is also included.Results: The three patients are older then two years of age. They did not present life threatening pulmonary complication nor respiratory distress, in contrast to most of the cases reported in the literature.Conclusions: Even though the number of occurrences of this syndrome is quite low, it must be consider when conducting a differential diagnostic related to thoracic cage abnormalities. It is important to emphasize the degree of variability of the pulmonary lesions and the progress of the disease. However, most articles on the subject point to a grim outcome. J. pediatr. (Rio J.). 1998; 74(4):333-337-: asphyxiating thoracic dystrophy, Jeune's syndrome, thoracic cage abnormalities.