A pediatric case of relapsed pulmonary alveolar proteinosis despite successful whole lung lavage / 소아과

Pulmonary alveolar proteinosis (PAP) is a rare disease in children characterized by intra-alveolar accumulation of surfactant proteins, which severely reduces gaseous exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. Herein, we present a pediatric case of PAP treated with WLL. An 11-year-old boy was admitted with the chief complaint of a dry cough lasting 6 months. He developed symptoms of dyspnea on exertion and had difficulty in climbing stairs. He was ultimately diagnosed with PAP through video-assisted thoracoscopic lung biopsy. As first-line of treatment for PAP, he underwent therapeutic WLL for each of his lungs on separate days. After a brief recovery, his symptoms gradually worsened; therefore, he underwent a second WLL. This is the first pediatric case of PAP relapse despite successful WLL in Korea.

The Effect of Histologic Chorioamnionitis on the Development of Respiratory Distress Syndrome and Chronic Lung Disease in Preterm Infants / 소아과

PURPOSE: Histologic chorioamnionitis may play a role in the development of respiratory distress syndrome(RDS) and chronic lung disease(CLD) independently or through its association with preterm birth. We investigated the relationship between histologic chorioamnionitis and clinical complications including, RDS and CLD, of preterm infants. METHODS: Clinical data were collected retrospectively from 478 preterm infants(gestational period< or =34 weeks) who were admitted to the neonatal intensive care unit(NICU) in Seoul National University Children's Hospital from January 1993 to December 2000. RESULTS: Histologic chorioamnionitis(CA) was observed in 210 of 478 infants(44%). Lower gestational period was detected in CA(+) group(31+1 +/- 2+2 weeks vs. 30+1+/-2+3 weeks). CA(+) group had decreased incidence of RDS(38.4% vs. 28.1%)[odds ratio, OR 0.35(P=0.0004, 95% confidence intervals, CI 0.19-0.63)], and increased incidence of CLD(7.5% vs. 13.3%)[OR 1.95(P=0.047, 95% CI 1.01-3.79)] combined much more "atypical CLD"(10.5% vs. 55.6%). CA(+) group had decreased incidence of patent ductus arteriosus(33.3% vs. 25.4%)[OR 0.37(P=0.003, 95% CI 0.19-0.71)]. There was no difference between the two groups in birth weight. CONCLUSION: It is suggested that intrauterine infections and fetal inflammatory responses might play a role in the outcome of preterm infants, and histologic chorioamnionitis is an isolated risk factor in the development of RDS and CLD of the preterm infants.

Classification and Risk Factors for Chronic Lung Disease(CLD) of Prematurity: Classical CLD Versus Atypical CLD

PURPOSE: We tried to classify the different type of CLD and assess the risk factors for classical CLD and atypical CLD. METHODS: Retrospective cohort analysis was done in 120 preterm infants with birth weights less than 1,500 g who were admitted to NICU in Seoul National University Children's Hospital between Jan. 1993 and Dec. 1998 and survived more than 28 days of life. RESULTS: CLD occurred in 44 of all infants(37%). The total subjects were classified into severe respiratory distress syndrome(RDS) group, mild RDS group, and non-RDS group. Multivariative logistic regression analysis was done for the assessment of risk factors for CLD in each groups. The analysis revealed that in severe RDS group, the significant risk factors for CLD were short gestational duration[OR 3.1(per 1 week decrement), 95% CI 1.4-7.0], male sex(OR 11, 95% CI 1.0-121), and poor response to surfactant(initial poor response to surfactant or relapse of RDS after initial good response to surfactant, OR 15, 95% CI 1.3-168). In non-RDS group, the significant risk factors for CLD were male sex(OR 8.9, 95% CI 1.5-51), chorioamnionitis(OR 7.5, 95% CI 1.4-38), and high mean airway pressure during the first 72 hours of life[OR 2.1(per 1 cmH2O increment), 95% CI 1.3-3.3]. CONCLUSIONS: It could be suggested that the poor response to surfactant of RDS might be one of the etiologic factors of classical CLD which occurs following severe RDS, and chorioamnionits might be one of the etiologic factors of atypical CLD which occurs without a history of RDS. Therefore CLD might be an etiologically heterogeneous disease entity.

Effects of Cisapride on QTc Interval and QT Dispersion in Preterm Infants

PURPOSE: Cisapride, a prokinetic agent, is widely used in preterm infants with feeding intolerance or gastroesophageal reflux. Although cisapride is regarded as a safe drug, increased QTc interval or ventricular arrhythmia has been reported in adults and neonates. So we prospectively examined the effects of cisapride on the QTc interval and QT dispersion in preterm infants. METHODS: QTc interval and QT dispersion were determined just before and 4.9+/-1.7days after the start of the cisapride treatment in 15 preterm infants with cisapride between April 1, 1998 and August 31, 1998. RESULTS: Cisapride significantly increased QTc interval(P<0.05), and this had no correlation with birthweight or gestational age. Three(20%) of the 15 cases were found to have QTc interval above 0.450, but they were clinically asymptomatic. QT dispersion did not increase significantly. CONCLUSION: Cisapride use in preterm infants is associated with an increase in QTc interval. High dose or longterm use of cisapride in preterm infants should be used cautiously, and when used, monitoring the QTc interval should be considered.

Association of Early Postnatal Neutropenia and Development of Bronchopulmonary Dysplasia in Preterm Infants

PURPOSE: To see if a similar relationship exists between the decreased number of circulating neutrophils and the development of bronchopulmonary dysplasia (BPD) in preterm infants, we tried to test the hypothesis that claims that preterm infants, who develop BPD, have decreased number of circulating neutrophils than those who do not develop BPD. METHODS: A retrospective cohort study was conducted in 167 preterm infants from August 1995 to July 1997, who were admitted in the neonatal intensive care unit (NICU) of Seoul National University Children's Hospital. RESULTS: BPD was diagnosed in 16% (27/167) of preterm infants. We compared the clinical characteristics of the study population according to the presence or absence of BPD. Compared to non-BPD group, the BPD group had a lower gestational age (29.4 +/- 2.7weeks versus 32.7 +/- 1.7 weeks), lower birth weight (1,240 +/- 486g versus 1,780 +/- 420g), lower incidence of prenatal steroid use (2/27 versus 41/140), decreased number of circulating neutrophils (3,622 +/- 4,866/microliter versus 7,586 +/- 4,545/microliter) at 1 day of life. After adjusting for the variables of the above risk factors, neutropenia (<2,500/microliter) in the peripheral blood increased the odds ratio of developing BPD (OR : 46.3, 95% CI : 17.3-117.2). CONCLUSION: Early postnatal neutropenia might be an important risk factor for the development of BPD and lung injury responsible for the development of BPD might begin at the early postnatal period.

Association of Early Postnatal Neutropenia and Development of Bronchopulmonary Dysplasia in Preterm Infants

PURPOSE: To see if a similar relationship exists between the decreased number of circulating neutrophils and the development of bronchopulmonary dysplasia (BPD) in preterm infants, we tried to test the hypothesis that claims that preterm infants, who develop BPD, have decreased number of circulating neutrophils than those who do not develop BPD. METHODS: A retrospective cohort study was conducted in 167 preterm infants from August 1995 to July 1997, who were admitted in the neonatal intensive care unit (NICU) of Seoul National University Children's Hospital. RESULTS: BPD was diagnosed in 16% (27/167) of preterm infants. We compared the clinical characteristics of the study population according to the presence or absence of BPD. Compared to non-BPD group, the BPD group had a lower gestational age (29.4 +/- 2.7weeks versus 32.7 +/- 1.7 weeks), lower birth weight (1,240 +/- 486g versus 1,780 +/- 420g), lower incidence of prenatal steroid use (2/27 versus 41/140), decreased number of circulating neutrophils (3,622 +/- 4,866/microliter versus 7,586 +/- 4,545/microliter) at 1 day of life. After adjusting for the variables of the above risk factors, neutropenia (<2,500/microliter) in the peripheral blood increased the odds ratio of developing BPD (OR : 46.3, 95% CI : 17.3-117.2). CONCLUSION: Early postnatal neutropenia might be an important risk factor for the development of BPD and lung injury responsible for the development of BPD might begin at the early postnatal period.

Influence of Phototherapy on Incidence of Patent Ductus Arteriosus in Very Low Birth Weight Infants

PURPOSE: Patent ductus arteriosus (PDA) is a common disease in very low birth weight infants (VLBWI). Hemodynamically significant PDA increases the morbidity and mortality of premature infants. Based on experimental model, light inhibits the constriction of immature piglet's ductal rings. No specific mechanism adequately explains the effect of light on the relaxation of PDA. Several hypotheses, including activation of photosensitive metabolites, alterations in receptors, or alterations in prostaglandin metabolism, have been postulated. The purpose of this study was to evaluate the influence of phototherapy on incidence of PDA in VLBWI. Mehtods : Sixty-three infants with birth weights less than 1,500 gm from March 1994 to February 1996 who were admitted in NICU of Seoul National University Children's Hospital were included. Thirty-four infants from March 1995 to February 1996 were shielded with aluminium foils on left chest during phototherapy (Shield group) and twenty-nine infants from March 1994 to February 1995 were not shielded (No shield group : control group). We investigated the incidence and the perinatal risk factors of PDA. RESULTS: 1) The incidence of PDA was 18% in shield group and 41% in control group. There was statistically significant between the two groups (P<0.05). 2) There was not statistically significant between two groups with gestational age, birth weight, sex, delivery mode, etc. 3) The perinatal risk factors which were statistically significant were group and presence of respiratory distress syndrome (RDS), and use of artificial surfactant. With linear logistic regression analysis, only group (OR=8.3, 95% CI=1.17-58.69) and presence of RDS (OR=21.3, 95% CI=1.39-329.81) were proved to be related to the occurrence of PDA. CONCLUSIONS: We conclude that chest shielding during phototherapy is a simple and inexpensive method to decrease the incidence of PDA.