RESUMO
OBJECTIVES: To evaluate the diagnostic value of transbronchial biopsy (TBB), video-assisted thoracoscopy (VAT), and open lung biopsy (OLB) in immunocompetent children with chronic, diffuse infiltrates; to identify factors that may predict diagnosis in children requiring biopsy; to determine whether age, number of biopsies, or type of procedure are associated with diagnostic yield in children undergoing transthoracic biopsy; and to compare morbidity of VAT with that of OLB. STUDY DESIGN: As part of a prospective, descriptive study to define the clinical spectrum of pediatric interstitial lung disease, 30 immunocompetent children required TBB, VAT, and/or OLB for diagnosis of diffuse infiltrates. We reviewed and analyzed the following clinical variables: age; preoperative diagnosis; type of procedure; number of lobes undergoing biopsies; durations of surgery, chest tube insertion, and hospitalization; tissue diagnosis; and complications. RESULTS: Specific diagnoses were made in 50%, 60%, and 53% of patients undergoing TBB, VAT, and OLB, respectively. A variety of rare disorders was found, and tissue diagnosis confirmed the preoperative diagnosis in 25% of all procedures. For patients who underwent transthoracic biopsy, patient age of greater than 24 months was significantly associated with increased diagnostic yield, but the number of lobes biopsied and type of procedure were not. VAT was associated with shorter operating time, chest tube placement, and hospitalization when compared with OLB. The complications of VAT and OLB were comparable. CONCLUSION: Lung biopsy is an important tool for the diagnosis of interstitial lung disease in immunocompetent children, but the diagnosis of many children, particularly those aged 2 years or younger, remains uncertain.
Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Toracoscopia , Adolescente , Fatores Etários , Biópsia , Criança , Pré-Escolar , Doença Crônica , Humanos , Lactente , Recém-Nascido , Doenças Pulmonares Intersticiais/imunologia , Estudos ProspectivosRESUMO
To study the potential role of endothelin-1, a potent endothelium-derived vasoconstrictor peptide, in the pathophysiology of persistent pulmonary hypertension of the newborn (PPHN), we measured arterial concentrations of immunoreactive endothelin-1 (irET-1) in 24 neonates with PPHN. Secondary diagnoses included meconium aspiration syndrome (13 patients), sepsis (2), congenital diaphragmatic hernia (1), asphyxia (1), pulmonary hemorrhage (1), aspiration of blood (1), and respiratory distress syndrome (1). Compared with irET-1 levels in umbilical cord blood in normal infants (15.1 +/- 4.1 pg/ml; mean +/- SEM) and in newborn infants with hyaline membrane disease who were supported by mechanical ventilation (11.8 +/- 1.2 pg/ml), infants with PPHN had markedly elevated circulating irET-1 levels (27.6 +/- 3.6 pg/ml; p < 0.01 vs cord blood, hyaline membrane disease). Infants with severe PPHN requiring extracorporeal membrane oxygenation (ECMO) therapy had higher irET-1 levels than infants with milder disease (31.0 +/- 4.7 for ECMO-treated infants vs 21.2 +/- 2.0 for non-ECMO-treated infants; p < 0.05). In patients treated without ECMO, irET-1 progressively decreased during the following 3 to 5 days, paralleling clinical improvement. In contrast, irET-1 concentrations remained elevated in infants with severe PPHN during ECMO therapy. We conclude that circulating irET-1 levels are elevated in newborn infants with PPHN, are positively correlated with disease severity, and decline with resolution of disease in patients who do not require ECMO therapy. Whether endothelin-1 contributes directly to the pathophysiology of PPHN or is simply a marker of disease activity remains speculative.
Assuntos
Anticorpos/sangue , Endotelinas/imunologia , Síndrome da Persistência do Padrão de Circulação Fetal/sangue , Análise de Variância , Oxigenação por Membrana Extracorpórea , Feminino , Sangue Fetal/química , Humanos , Doença da Membrana Hialina/sangue , Doença da Membrana Hialina/epidemiologia , Recém-Nascido , Masculino , Síndrome da Persistência do Padrão de Circulação Fetal/epidemiologia , Síndrome da Persistência do Padrão de Circulação Fetal/terapia , Radioimunoensaio , Fatores de TempoRESUMO
To evaluate the impact of early pancreatic insufficiency on growth and nutritional status in cystic fibrosis, we studied 49 infants identified by a newborn screening program. Pancreatic insufficiency, determined by increased 72-hour fecal fat excretion, was present in 59% (23/39) of infants at diagnosis (7.0 +/- 0.8 weeks; mean +/- SEM). Before initiation of pancreatic enzyme replacement, growth and nutritional status of pancreatic-insufficient (n = 16) and pancreatic-sufficient (n = 13) infants were compared. Pancreatic-insufficient infants gained less weight from birth to diagnosis (13.4 +/- 3.4 vs 22.3 +/- 4.0 gm/day; p = 0.05), had decreased triceps skin-fold thicknesses (4.5 +/- 0.3 vs 6.1 +/- 0.4 mm; p less than 0.005), and had lower blood urea nitrogen (3.07 +/- 0.42 vs 4.62 +/- 0.65 mg/dl; p = 0.02) and albumin (2.99 +/- 0.14 vs 3.54 +/- 0.14 gm/dl; p less than 0.01) levels despite higher gross calorie (154 +/- 8 vs 116 +/- 13 kcal/kg per day; p less than 0.01) and protein intakes (2.81 +/- 0.21 vs 2.14 +/- 0.33 gm/kg per day; p = 0.03). Fecal nitrogen loss was correlated with fat loss (r = 0.79; p less than 0.001). Fat malabsorption was present in 79% (30/38) and 92% (33/36) of infants tested at 6 months and 12 months of age, respectively, indicating that pancreatic insufficiency persists and increases in frequency throughout infancy. We conclude that pancreatic insufficiency is prevalent in young infants with cystic fibrosis and has a significant impact on growth and nutrition.