ABSTRACT
BACKGROUND AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. The aim of this study was to characterize PAH in pediatric patients by evaluating the patients demographics, clinical and hemodynamic variables, treatments, and outcomes. SUBJECTS AND METHODS: Sixty-five patients who were diagnosed with PAH at Seoul National University Children's Hospital between January 1985 and August 2007 were retrospectively reviewed. RESULTS: There was no difference in gender distribution (males, 33; females, 32). The mean age at the time of diagnosis was 5.7+/-5.2 years and the mean follow-up period was 6.3+/-5.5 years. The major causes of PAH were congenital heart disease (CHD) in 32 patients (49.2%) and idiopathic PAH in 11 patients (16.9%). The most common presenting symptom was dyspnea in 44 patients (67.7%). The mean cardiothoracic ratio was 58.9+/-8.3%, which decreased to 55.9+/-8.3% after vasodilator therapy (p=0.011). The mean pulmonary arterial pressure at the time of cardiac catheterization was 59.7+/-18.7 mmHg and the mean pulmonary vascular resistance was 14.9+/-9.7 wood units . m2. Forty-three of 65 patients (66.2%) had vasodilator therapy (prostacycline, sildenafil, and bosentan). A statistically significant decrease in tricuspid valve regurgitation velocity (4.8+/-0.8 m/sec vs. 3.6+/-1.0 m/sec, p=0.001), and an increase in diastolic dimension of the left ventricle (28.3+/-12.1 mm vs. 33.2+/-10.2 mm, p=0.021) on echocardiography before and after vasodilator therapy was demonstrated. The 5-, 10-, and 15-year survival rates were 96%, 92% and 65%, respectively. Six of 65 patients (9.2%) with PAH died. There was no significant correlation between outcome and the immediate response to the vasodilators. CONCLUSION: PAH is a devastating disease which is rare in children. PAH in children has a variable pattern cause and progression, the confirmation of which requires analysis of detailed registries from nationwide hospitals.
Subject(s)
Child , Female , Humans , Arterial Pressure , Cardiac Catheterization , Cardiac Catheters , Demography , Dyspnea , Echocardiography , Follow-Up Studies , Heart Defects, Congenital , Heart Diseases , Heart Ventricles , Hemodynamics , Hypertension , Hypertension, Pulmonary , Piperazines , Prognosis , Purines , Rare Diseases , Registries , Retrospective Studies , Sulfones , Survival Rate , Tricuspid Valve Insufficiency , Vascular Resistance , Vasodilator Agents , Wood , Sildenafil CitrateABSTRACT
PURPOSE: The purpose of this study was to determine those factors which could contribute to the development of necrotizing enterocolitis(NEC) in fullterm. METHODS: We retrospectively reviewed the medical record of 20 full-terms with NEC(> or = modified Bell's staging criteria IIa) who were admitted to the Neonatal Intensive Care Unit of Il Sin Christian hospital from January 1998 through July 2005, and for each case, the next 2 healthy newborns were matched as controls. RESULTS: Mean gestational age and birth weight in the fullterm with NEC group was 38.42 weeks and 2,915 g; in the healthy fullterm without NEC group, it was 38.61 weeks and 3,148 g. When compared with the control group, NEC infants had a significantly higher frequency of chorioamnionitis, protracted diarrhea. As for Apgar score at 1 min <7, respiratory problem, congenital heart disease. there were no differences in frequency of preeclampsia, maternal diabetes, maternal drug abuse, meconium-stained amniotic fluid, polycythemia or exchange transfusion. CONCLUSION: Most of these full term infants have a predisposing factor before developing NEC. Our study suggested that NEC in fullterm infants was significantly associated with protracted diarrhea, and congenital heart disease.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Pregnancy , Amniotic Fluid , Apgar Score , Birth Weight , Causality , Chorioamnionitis , Diarrhea , Enterocolitis, Necrotizing , Gestational Age , Heart Defects, Congenital , Intensive Care, Neonatal , Medical Records , Polycythemia , Pre-Eclampsia , Retrospective Studies , Risk Factors , Substance-Related DisordersABSTRACT
PURPOSE: To evaluate various sepsis screening tests, individually and in combination, to formulate a guideline for the diagnosis of neonatal sepsis. METHODS: The study was a retrospective cohort study. It took place at the neonatal intensive care unit of the Paediatric Department, Il Sin Christian Hospital, Busan, Korea, over a period of 68 months from 1st, April, 2001 to 31st, December, 2005. This study evaluated 100 neonates having clinical features of sepsis and 100 normal asymptomatic neonates and used screening tests including C-reactive protein (CRP), total leukocyte count (TLC), absolute neutrophil count (ANC), immature neutrophils to total neutrophil count ratio (I/T ratio), thrombocytopenia, degenerative changes in the neutrophils and gastric aspirate cytology (GAC) for the diagnosis of neontal sepsis. RESULTS: The sensitivity of CRP and ANC was high. CRP had 86 percent sensitivity for group-A (proven sepsis) and 74 percent sensitivity for group-B (probable sepsis) and 94 percent specificity for group-A, B. ANC had sensitivity of 72 percent for group-A and 62 percent for group-B and 86 percent specificity for group-A, B. For group-A, sensitivity, specificity of GAC for polymorphs was 74 percent and 94 percent respectively. As for sensitivity, specificity of platelet count for group-A was 64 percent and 89 percent respecively. The sensitivity, specificity and predictive values (PV) of the individual tests and different test combinations were also calculated for group-A and B. CONCLUSION: For the detection of culture negative cases in neonatal sepsis, screening tests including CRP, TLC, ANC, thrombocytopenia, cytoplasmic vacuolization in the neutrophils and GAC for polymorphs have high sensitivity. A combination of three tests has higher sensitivity.