Most of the gastric outlet obstruction symptoms like vomiting and abdominal distension were caused by congenital anatomical abnormality in a neonate. Abnormal structures associated with congenital gastric outlet obstruction have been categorized by its site and extent of obstruction. We report one case of persisting vomiting in a premature infant caused by serosal fibrous band in gastric outlet lesion, excluded from the category of congenital gastric outlet obstruction.
Fibrosis ; Gastric Outlet Obstruction* ; Humans ; Infant, Newborn ; Infant, Premature ; Vomiting

Protein C (PROC) deficiency is caused by mutations in the PROC gene on chromosome 2q14.3. Patients with PROC deficiency typically present distinguished purpura, intracerebral and intravascular coagulopathy, and ophthalmologic complications. Here, we report a rare severe form of PROC deficiency resulting from a compound heterozygosity in PROC. The patient was a 5-day-old female neonate born at 39 weeks of gestation with a birth weight of 2,960 g. She was transferred to our hospital with running a fever at 38.5℃ and with dark red patches on her feet. At admission, a complete blood count showed no specific findings, but levels of PROC and protein S were abnormally low (1% and 68%, respectively). Magnetic resonance imaging revealed intracerebral hemorrhaging and parenchymal damage with dysplasia of the brain. Ophthalmologic examination revealed vitreous hemorrhaging with retinal detachment. Genetic testing revealed a missense mutation (Arg211Trp) and a frameshift mutation (Gly239Serfs*8) in PROC, inherited from the father and mother, respectively. The patient recovered from purpura after undergoing ventriculoperitoneal shunting and treatment with fresh frozen plasma, warfarin sodium, and PROC concentrate. This is the first report of severe neonatal PROC deficiency with purpura fulminans, vitreous hemorrhage, and intracerebral hemorrhage confirmed via PROC genetic testing, which identified a rare compound heterozygosity of PROC.
Birth Weight ; Blood Cell Count ; Brain ; Cerebral Hemorrhage ; Diagnosis* ; Fathers ; Female ; Fever ; Foot ; Frameshift Mutation ; Genetic Testing ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging ; Mothers ; Mutation, Missense ; Plasma ; Pregnancy ; Protein C Deficiency* ; Protein C* ; Protein S ; Purpura ; Purpura Fulminans ; Retinal Detachment ; Running ; Ventriculoperitoneal Shunt ; Vitreous Hemorrhage ; Warfarin

PURPOSE: Antenatal steroid (AS) may result in lower insensible water loss (IWL), and higher urine output (UO) in early life. We examined if the postnatal fluid balance differed between infants exposed to AS or not (control) in VLBW infants. METHODS: Fifty-four VLBW infants were grouped into AS (n=24) or control (n=30). Fluid intake, UO, IWL and maximal % of weight loss on day 1, day 2, day 3 and day 7 after birth were analyzed. Daily maintenance fluid was determined in each infants by calculation of insensible water loss (IWL=[intake-output]-Delta wt) and UO. RESULTS: Fluid intake (AS vs control; 117.2+/-33.9 vs 126.0+/-29.6 mL/kg/d, P=0.315), IWL (28.1+/-23.7 vs 21.1+/-20.5 P=0.248), UO and maximal % of weight loss on day 7 were not different between groups: similar findings were observed on day 1, day 2, and day 3 after birth. Neonatal morbidities and clinical relevant factors were not different between groups. The duration of assisted ventilation was shorter in the AS than in the control (10.8+/-9.2 vs 27.6+/-26.2, P=0.002). However, the difference disappeared after adjustment for RDS severity and oxygenation index. CONCLUSION: VLBW infants exposed to AS did not have an alteration in postnatal fluid balance during the first week of life, when given fluid based on needs reflected by IWL and UO. The decreased need for assisted ventilation in the AS group may be related to the effects of steroid on fetal lung fluid absorption or maturity, but not on postnatal fluid balance.
Absorption ; Humans ; Infant* ; Infant, Very Low Birth Weight* ; Lung ; Oxygen ; Parturition ; Ventilation ; Water Loss, Insensible ; Water-Electrolyte Balance* ; Weight Loss

Isolated obstruction of the splenic vein leads to segmental portal hypertension, which is mainly originated from pancreatic disease, such as chronic pancreatitis, pancreatic pseudocyst, pancreatic cancer. The clinical manifestation are devoid of ascites and encephalopathy, but presented with gastric variceal bleeding. We experienced 27-year-old male patient who complained of hematochezia and melena. After work-up with CT and angiography, we performed operation with the impression of pancreatic tumor associated with splenic vein occlusion. It resulted nonfunctioning pancreatic endocrine tumor. Often called islet cell tumor, pancreatic endocrine tumor has various names according to secreted hormones, such as insulinoma, Zollinger-Ellison syndrome and glucagonoma. It was classified as nonfunctioning tumor if there were any evidence of hormone secretion by radioimmunoassay and immunohistochemistry. Surgical excision and adjuvant medical therapy is the mainstay of therapy. We performed tumor resection and splenectomy for control of gastric variceal bleeding due to splenic vein occlusion.
Adenoma, Islet Cell ; Adult ; Angiography ; Ascites ; Esophageal and Gastric Varices* ; Gastrointestinal Hemorrhage ; Glucagonoma ; Humans ; Hypertension, Portal ; Immunohistochemistry ; Insulinoma ; Male ; Melena ; Pancreas* ; Pancreatic Diseases ; Pancreatic Neoplasms ; Pancreatic Pseudocyst ; Pancreatitis, Chronic ; Radioimmunoassay ; Splenectomy ; Splenic Vein ; Zollinger-Ellison Syndrome

PURPOSE: Preterm formula used in Korea, theoretically does not supply the recommended mineral intake for optimal bone metabolism in very low birth weight infants(Formula 500-700 mg Ca/L, 300~370mg P/L, and 440IU of vitamin D/L). The purpose of this study is to identify th e possible etiologic factors and clinical course of rickets in these infants. METHODS: We recently identified radiographic rickets or osteopenia in 16 VLBW infants over a 30-month period from January 1990, to July 1992. We performed a retrospective case analysis to evaluate the clinical features, nutritional status, biochemical and radiological findings o f metabolic disease in preterm infants. RESULTS: Mean gestational age and birth weight were 29+/-2.1wks, and 1172+/-245gm. All infants received parenteral nutrition and 11 infants needed assisted ventilation. Enteral feeding w as started at a mean age of 9.4+/-11.0d and mean total calorie intakes were 62+/-16.2kcal/kg /d in the first 2wks and 111+/-26.9kcal/kg/d at 2-4 wks of age. Oral Ca/P intakes were severely restricted during the first month of life, and they were about 20% of recommended intakes of Ca /P for VLBW infants. At diagnosis of active rickets, serum Ca was high in 19% and serum P wa s low in 69% of infants. Serum alkaline phosphatase was increased in 56% and serum 25-hydroxyvitamin D was low in 67%. Active rickets was diagnosed at mean age of 38+/-14.7 d; 12 infants had overt rickets(grade 2), including 3 infants with fractures(grade 3). Healing rickets was diagnosed on follow-up at mean age of 67.3+/-22.0 days. Thus, metabolic bone disease of VLB W infants was associated with low mineral intakes compared to recommended intakes, and signs of P deficiency occurred at about 2 wks of age and persisted to 8 wks of age; hypercalcemia occurred initially, and these biochemical abnormalities precede the radiological abnormalities about 2 or 3 weeks. CONCLUSIONS: Many factors are related to the development of metabolic hone disease of prematurity; one of the most important factor in our study was nutritional deficiency, especially m ineral(phosphorus) and vitamin D. In preterm VLBW infants whose adequate enteral feeding is restricted, mineral(calcium and phosphorus) and vitamin D supplementation would be needed t o reduce the development of metabolic bone disease in preterm infants.
Alkaline Phosphatase ; Birth Weight ; Bone Diseases, Metabolic* ; Diagnosis ; Enteral Nutrition ; Follow-Up Studies ; Gestational Age ; Humans ; Hypercalcemia ; Infant ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Korea ; Malnutrition ; Metabolic Diseases ; Metabolism ; Nutritional Status ; Parenteral Nutrition ; Retrospective Studies ; Rickets ; Ventilation ; Vitamin D ; Vitamins

BACKGROUND: Severe acute lung injury(ALI), also known as the adult respiratory distress syndrome(ARDS), is a heterogenous nature of dynamic and explosive clinical synrome that exacts a mortality of approximately 50%. Endotoxin(ETX) is an abundant component of the outer membrane of gram-negative bacteria capable of inducing severe lung injury in gram-negative sepsis and gram-negative bacterial pneumonia, which are among the most common predisposing causes of ARDS. The influx of PMNs into airway tissue is a pathological hallmark of LPS-induced lung injury. And th3re is a substantial evidence suggesting that cytokines are important mediators of lung injury in gram-negative sepsis. However, the kinetics of phagocytes and cytokines by an exact time sequence and their respective pathogenic importance remain to be elucidated. This study was performed to investigate the role of phagocytes and proinflammatory cytokines in ETX-induced ALl through a time course of changes in the concentration of protein, TNFa and IL-6, and counts of total and its differential cells in BALF. The consecutive histologic findings were also evaluated. METHOD: The experimental animals, healthy male Sprague-Dawley, weighted 200+/-50g, were divided into controland ALI-group. ALI was induced by an intravenous administration of ETX, 5mg/kg. Above mentioned all parameters were examined at 0(control), 3, 6, 24, 72 h after administration of ETX. TNFa and IL-6 conc. in BALE were measured by a bioassay. RESULTS: The protein concentration and total leukocyte count(TC) in BALF was significantly increased at 3h compared to controls(p<0.05). The protein conc. was significantly elavated during observation period, but TC was significantly decreased at 72h(p<0.05 vs. 24h). There was a close relationship between TC and protein cone. in BALF(r = 0.65, p <0.001). The PMN and monocyte count was well correlated with TC in BALF, and the correlation of PMN(r=0.97, p<0.001) appeared to be more meaningful than that of monoeyte(r = 0.61, p<0.001). There was also a significant correlation between protein cone. and PMN or monocyte count in BALF(PMN vs. monocyte r = 0.55, p<0.005 vs. r = 0.64, p<0.001). The count of monocyte was significantly elavated during observation period though a meaningful reduction of PMN count in BALF at 72h, this observation suggested that monocyte may, at least, partipate in the process of lung injury steadly. In this sudy, there was no relationship between IL-6 and TNFt conc., and TNFa but not IL-6 was correlated with TC(r 0.61, p <0.05) and monocyte(r = 0.67, p<0.05) in BALF only at 3, 6h after ETX introduced. In particular, the IL-6 cone. increased earlier and rapidly peaked than TNFz cone. in BALF. In histologic findings, the cell counts of lung slices were increased from 3 to 72h(p<0.001 vs. NC). Alveolar wallthickness was increased from 6 to 24h(p<0.001 vs. NC). There was a significant correlation between the cell counts of lung slices and alveolar wall-thickness(r= 0.61, p<0.001). This result suggested that the cellular infiltrations might be followed by the alterations of interstitium, and the edematous change of alveolar wall might be most rapidly recovered to its normal condition in the process of repair. CONCLUSION: We concluded that although the role of PMIN is partly certain in ETX-induced ALI, it is somewhat inadequate to its known major impact on ALL Alveolar macrophage and/or non-immune cells such as pulmonary endothelial or epithelial cells, may be more importantly contributed to the initiation and perpetual progression of ETX-induced ALI. The IL-6 in ETX-induced ALI was independent to TNFa, measured by a bioassay in BALF. The early rise in IL-6 in BALF implies multiple origins of the IL-6.
Acute Lung Injury* ; Administration, Intravenous ; Adult ; Animals ; Biological Assay ; Cell Count ; Cytokines ; Epithelial Cells ; Gram-Negative Bacteria ; Humans ; Interleukin-6 ; Kinetics ; Leukocytes ; Lung ; Lung Injury ; Macrophages, Alveolar ; Male ; Membranes ; Monocytes ; Mortality ; Phagocytes* ; Pneumonia, Bacterial ; Rats, Sprague-Dawley ; Sepsis

PURPOSE: Pulmonary function is decreased in varying degrees in healthy premature infants as well as those with bronchopulmonary dysplasia. The evaluation of pulmonary function in infants is finally standardized after strenuous efforts, but it has not yet been in Korea. In this study, we aimed at the evaluation of the utility of pulmonary function tests in premature infants with chronic lung disease by objectively measuring pulmonary function and by analyzing the risk factors that may decrease lung function. METHODS: Fifty-four premature infants born in Severance Hospital were selected. Among the 54 infants, 31 were male and 23 were female, and their mean age was 5.6+/-3.7 years. Exhalyser was used to measure tidal volume and functional residual capacity, and then their change after the inhalation of bronchodilators was evaluated. There was no test related complication in all subjects. RESULTS: Among the 54 subjects, 22 were at the gestational age of <28 weeks, 25 were at the gestational age between 28 and 33 weeks, and 7 were at the gestational age between 33 and 37 weeks. As for birth weight, 23 had extreme low birth weight, 23 had very low birth weight, and 8 had low birth weight. The delta functional residual capacities (FRCs) before and after the inhalation of bronchodilator were significantly increased in infants with younger gestational age (P<0.05) and lower birth weight (P<0.05). There was a significant negative correlation between gestational age and birth weight, and a significant positive correlation with the duration of ventilator care and that of oxygen therapy. The delta FRC before the inhalation of bronchodilator was significantly lower in infants with lower birth weight, and the tidal volume before the inhalation of bronchodilator correlated negatively with the duration of ventilator care. CONCLUSION: The reversibility of FRC is increased in premature infants with lower birth weight, younger gestational age, and longer duration of ventilator care and oxygen therapy. The reversibility of FRC may be a useful parameter of pulmonary function that can be safely measured in premature infants with chronic lung disease.
Birth Weight ; Bronchodilator Agents ; Bronchopulmonary Dysplasia ; Female ; Functional Residual Capacity ; Gestational Age ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Inhalation ; Korea ; Lung ; Lung Diseases ; Male ; Oxygen ; Respiratory Function Tests ; Risk Factors ; Tidal Volume ; Ventilators, Mechanical

BACKGROUND AND OBJECTIVES: Idiopathic dilated cardiomyopathy (IDC) is a disease entity with no known specific curative measures. However, significant improvement in the left ventricular (LV) systolic function, during the management course for IDC, is frequently observed. In this study we tried to ascertain associated factors for the improvement of the LV function in patients with IDC. SUBJECTS AND METHODS: Thirty-three patients, newly diagnosed as IDC between Jan. 1999 and Jan. 2001, on whom a 6-month follow-up echocardiography was performed, were included in the study. Improvement in the LV systolic function was defined as an increase in the LV ejection fraction greater than 10% from the baseline. The subjects were divided into two groups; the improved group (IG) and the unimproved group (UG). The clinical characteristics and management methods were evaluated, and compared between the two groups. RESULTS: Seventeen patients (M/F:15/2) were included in the IG, and 16 (M/F:7/9) were included in the UG. There were no significant differences in the baseline LV systolic function (IG:23.4+/-1.5% vs. UG:28.9+/-2.3%), age, NYHA functional class or resting heart rate on admission between the two groups. b-blocker therapy (p=0.002), the absence of diabetes mellitus (p=0.046) and male sex (p=0.007), were all significantly associated with an improvement in the LV systolic function from the univariate analyses. With the multivariate analyses, only b-blocker therapy was significantly associated with an improvement in the LV systolic function. The 6-month event-free survival rate was significantly better in the IG compared with UG (94+/-5% vs. 63+/-13%, p=0.031). CONCLUSION: b-blocker therapy exerts a considerable effecs on the improvement in the LV systolic function of patients with IDC.
Adrenergic beta-Antagonists ; Cardiomyopathy, Dilated* ; Diabetes Mellitus ; Disease-Free Survival ; Echocardiography ; Follow-Up Studies ; Heart Failure ; Heart Rate ; Humans ; Male ; Multivariate Analysis

Granular cell tumor(formerly named to be granular cell myoblastoma) was first described by Abrikossoff in 1926 and is a rare tumor thought to be of Schwann cell origin. It can occur at any soft tissue of the body, but most cases are found at the tongue, skin, breast and GI tract. Only 6% of them occur in respiratory system. We report two cases of asymptomatic granular cell tumor of the bronchus that were detected incidentally by bronchoscopy. One patient had aspiration pneumonia, the other had immotile cilia syndrome. The former patient was simply observed and bronchoscopic extirpation of the tumor was done in the latter patient.
Breast ; Bronchi* ; Bronchoscopy* ; Ciliary Motility Disorders ; Gastrointestinal Tract ; Granular Cell Tumor* ; Humans ; Pneumonia, Aspiration ; Respiratory System ; Skin ; Tongue