Bronchiolitis Obliterans with Finger Clubbing after Measles Infection / 소아알레르기및호흡기학회지

A 5-year-old Korean boy was admitted with dyspnea, intermittent cyanosis and finger clubbing. He was diagnosed to be suffering measles pneumonia 3 years ago. Plain radiograph showed collapse of the right lung, mediastinal shifting and infiltration on both lower lung fields. High resolution computerized tomography revealed a central bronchiectasis with mosaic hyperinflation. All of the clinical information indicated that the severity of illness score was 4 of bronchiolitis obliterans (BO). The bronchoalveolar lavage cellular profile showed predominant neutrophilia. BO with finger clubbing has been rarely reported previously in childhood. We report a case of BO with finger clubbing in children with regard to clinical presentation of measles complicated pneumonia.

A Case of Lennox-Gastaut Syndrome due to 3-Methylcrotonyl CoA Carboxylase Deficiency / 대한소아신경학회지

3-Methylcrotonyl-CoA carboxylase(MCC) is a biotin-dependent enzyme involved in the leucine metabolism. We describe a patient with MCC deficiency who manifested with Reye syndrome-like illness with status epilepticus, metabolic acidosis, hypoglycemia, hyperammonemia, elevated liver enzymes and neurologic impairments after a viral gastroenteritis and then suffered from Lennox-Gastaut syndrome. Urinary organic acid analysis revealed increased excretions of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. This patient was managed with a leucine restriction diet and supplementation of biotin and carnitine, which was not so effective. He suffered from neurologic sequelae such as Lennox-Gastaut syndrome, motor and cognitive impairements.

Pulmonary Function of Patients with Adolescent Idiopathic Scoliosis / 소아과

PURPOSE: To evaluate the patterns of preoperative pulmonary function tests(PFTs) and their correlation with postoperative pulmonary impairment in patients with adolescent idiopathic scoliosis(AIS), who had orthopaedic operation with or without rib resection thoracoplasty. METHODS: The case records and radiographs of 79 patients(12, boys and 67, girls) of a mean age of 13.3 years(7-15 years) with AIS who underwent orthopaedic operations at the author's institute between 1997 and 2003 were reviewed. RESULTS: The average preoperative coronal Cobb measurement was 56 (range, 32-81 ). Thirty-one patients(39%) had normal values of of PFTs. Forty five patients(57%) with severe scoliosis(Cobb's angle 65-90 ) showed mild to moderate decrease on PFTs with FVC(73%), FEV1(79%), FEV25-75%(58 %). The severity of Cobb's angle were correlated with the decrease in FVC, FEV1, FEV25-75%(P< 0.05). Forty-three patients had thoracoplasty. Twenty-two(51%) of them had pleural effusions. Eleven patients among them had chest tube inserted postoperatively. But, none of the patients had any increased requirement of postoperative ventilatory support. The incidence of postoperative pulmonary effusion in patients with thoracoplasty was a 17-fold increase, compared to the patients without thoracoplasty(O.R.=17, chi-squared-test, P<0.05). CONCLUSION: The fall in FVC and FEV1 of patients with mild to moderate scoliosis(Cobb's angle, below 65 ) was mild and was unlikely to be of clinical significance. It appeared that patients with severe AIS had mild to moderate restrictive patterns with reduced lung volumes. Performance of the thoracoplasty was the important risk factor for postoperative pulmonary complications in patients underwent surgical operation.

Fatty Acids Composition in Breast Milk and Its Relationship with Infant Body Weight Gain / 대한소아내분비학회지

PURPOSE:Recent studies proposed that polyunsaturated fatty acids (PUFA) of the omega6 series (linoleic acid, LA) compared to the omega 3 series (linolenic acid, LNA) may be a potent promoters of adipogenesis during the gestation/lactation period. Increased ratio of LA/LNA may stimulate adipose tissue development during gestation/lactation period and may subsequently lead to chilhood obesity. The purpose of this study is to evaluate fatty acids composition of the breast milk in Korean lactating women and to analyze the relationship between LA/LNA and the body weight gain of the infants. METHODS:Fifty eight healthy postpartum lactating women and their healthy 58 breast feeding infants were studied. At 11.3+/-3.9 days of postpartum, breast milk were taken and analyzed by gas-liquid chromatography with one-step methylation method. RESULTS:The total fat contents in breast milk were 3.2+/-1.3%. The total fat contents in breast milk were not related to maternal BMI or Kaup index of infants. The percentage of PUFA was 24.9+/-6.0%. Among these PUFA, the percentage of LA and LNA was 20.3+/-5.8% and 2.2+/-0.8%, respectively. LA/ LNA ratio was 10.2+/-2.5. There were no significant correlations between LA/LNA ratio and infant weight gain during the first 2 months of life. CONCLUSION: Short term weight gain of infant was not related to the LA/LNA ratio in the breast milk. Further studies on the long term effects on fat percent and body weight of infants would be needed.