No abstract available.
Thanatophoric Dysplasia*

Abdominal distension is not an uncommon symptom in the neonate; it is indistinguishable from Hirschsprung disease by symptoms and X-ray findings. In three patients, severe abdominal distension was found at early infancy and improved with conservative treatment without relapse. The findings were different from those of Hirschsprung disease. Immaturity or poor coordination of peristaltic movement is postulated as the cause. With maturation such problems can normalize. However the pathogenesis remains unclear and further investigation is needed to improve our understanding.
Hirschsprung Disease ; Humans ; Infant, Newborn* ; Recurrence

PURPOSE: Obesity has recently emerged as a significant health problem in the pediatric population, and the prevalence of non-alcoholic fatty liver disease is increasing in tandem with a significant rise in childhood obesity. Therefore, this study was conducted to clarify the risk factors of non-alcoholic steatohepatitis in obese children. METHODS: We enrolled 84 obese children who visited the pediatric obesity clinic at Yeung-Nam university hospital. The patients were divided into two groups based on their alanine aminotransferase (ALT) level (separated at 40 IU/L), and the mean of ages, total cholesterol levels, HDL-cholesterol levels, LDL-cholesterol levels, triglyceride (TG) levels, as well as the mean obesity index, and body fat percentage of the two groups were then compared. RESULTS: When the mean of ages (10.5+/-1.6 vs. 10.7+/-2.0 years), total cholesterol levels (183.0+/-29.1 vs. 183.7+/-31.3 mg/dL), HDL-cholesterol levels (53.0+/-10.2 vs. 55.7+/-13.0 mg/dL), LDL-cholesterol levels (113.4+/-30.2 vs. 113.0+/-30.0 mg/dL), triglyceride levels (99.4+/-62.9 vs. 114.2+/-47.3 mg/dL), obesity indexes (44.7+/-12.2 vs. 47.9+/-15.1%), and body fat percentages (32.7+/-5.0 vs. 34.0+/-4.8%) of group 1 (ALT< or =40 IU/L) were compared with those of group 2 (ALT> or =41 IU/L), no significant differences were observed (p>0.05). However, hypertriglyceridemia (TG> or =110 mg/dL) was more frequent in group 2 than in group 1 (p=0.023). CONCLUSION: TG may be an important risk factor in non-alcoholic steatohepatitis and further study regarding the risk factors in non-alcoholic steatohepatitis is required.
Adipose Tissue ; Alanine Transaminase ; Child ; Cholesterol ; Fatty Liver* ; Humans ; Hypertriglyceridemia ; Obesity ; Pediatric Obesity* ; Prevalence ; Risk Factors* ; Triglycerides

PURPOSE: Obesity has recently emerged as a significant health problem in the pediatric population, and the prevalence of non-alcoholic fatty liver disease is increasing in tandem with a significant rise in childhood obesity. Therefore, this study was conducted to clarify the risk factors of non-alcoholic steatohepatitis in obese children. METHODS: We enrolled 84 obese children who visited the pediatric obesity clinic at Yeung-Nam university hospital. The patients were divided into two groups based on their alanine aminotransferase (ALT) level (separated at 40 IU/L), and the mean of ages, total cholesterol levels, HDL-cholesterol levels, LDL-cholesterol levels, triglyceride (TG) levels, as well as the mean obesity index, and body fat percentage of the two groups were then compared. RESULTS: When the mean of ages (10.5+/-1.6 vs. 10.7+/-2.0 years), total cholesterol levels (183.0+/-29.1 vs. 183.7+/-31.3 mg/dL), HDL-cholesterol levels (53.0+/-10.2 vs. 55.7+/-13.0 mg/dL), LDL-cholesterol levels (113.4+/-30.2 vs. 113.0+/-30.0 mg/dL), triglyceride levels (99.4+/-62.9 vs. 114.2+/-47.3 mg/dL), obesity indexes (44.7+/-12.2 vs. 47.9+/-15.1%), and body fat percentages (32.7+/-5.0 vs. 34.0+/-4.8%) of group 1 (ALT< or =40 IU/L) were compared with those of group 2 (ALT> or =41 IU/L), no significant differences were observed (p>0.05). However, hypertriglyceridemia (TG> or =110 mg/dL) was more frequent in group 2 than in group 1 (p=0.023). CONCLUSION: TG may be an important risk factor in non-alcoholic steatohepatitis and further study regarding the risk factors in non-alcoholic steatohepatitis is required.
Adipose Tissue ; Alanine Transaminase ; Child ; Cholesterol ; Fatty Liver* ; Humans ; Hypertriglyceridemia ; Obesity ; Pediatric Obesity* ; Prevalence ; Risk Factors* ; Triglycerides

Background: Cross-sex hormone therapy (CHT) changes the physical characteristics of transgender women to match their gender identity and expression. This study aimed to determine the effects of feminizing cross-sex hormones on body composition, bone mineral density (BMD) and muscle strength in transgender women. Methods: A prospective observational study assessed 11 participants who underwent feminizing CHT. Dual energy X-ray absorptiometry (DXA), and handgrip strength were measured before CHT and after 6-months of CHT. Fat mass, lean body mass (LBM), and BMD were measured by DXA and handgrip strength was measured by hand-dynamometer. Results: Regional body fat in the trunk, legs, and gynoid region increased by 18%, 27.4%, and 27.2%, respectively after 6 months of CHT. Total body fat increased by 16.2%, while the fat mass ratio decreased by 7.2%. Although body fat increased, the android/gynoid fat ratio decreased; BMD in the lumbar spine significantly increased by 3.9% (P=0.0051), but changes in the femoral neck (P=0.1969) and total femur (P=0.4769) were not significant. Changes in LBM ranged from -3% (trunk) to -8% (arm region). Right-hand grip strength also significantly decreased by 7.7% (P=0.0467). Conclusions After 6 months of CHT, transgender women showed a general increase in fat mass and a decreased in overall LBM and handgrip strength. Increase in fat mass percentage were more remarkable in gynoid region, leading to a more “female” body fat distribution.

Tongue tumors of pediatric patients are the most common soft tissue tumor of pediatric oral and maxillofacial tumors. There are many kind of pediatric tongue lesions such as hemangioma, lymphangioma, papilloma, and cyst. Most of these lesions are benign, but malignant tumors of tongue may be occurred. Therefore, malignancy should be ruled out. Sometimes, tongue lesions are present with dyspnea, dysphagia, dysarthria, bleeding, or cosmetic problem. We experienced a case of chronic inflammatory mass on posterior 1/3 of the tongue in 4 month old female patient. Swallowing difficulty and respiratory distress symptom occurred because of tongue mass effect. The tongue mass was confirmed as granulation tissue by microscopic examination. After excision of tongue mass, she had no problem with swallowing and breathing.
Deglutition ; Deglutition Disorders ; Dysarthria ; Dyspnea ; Female ; Granulation Tissue* ; Hemangioma ; Hemorrhage ; Humans ; Infant* ; Lymphangioma ; Papilloma ; Respiration ; Tongue*

This study was performed to elucidate the mechanism of improved oxygenation after surfactant replacement therapy in respiratory distress syndrome (RDS) of the newborn infants. In 26 newborns with RDS, end tidal-CO2 tension (PetCO2), arterial blood gas analysis and pulmonary function tests were measured at baseline, 30 min, 2 hr and 6 hr after surfactant administration. The changes in dead space/tidal volume ratio (VD/VT ratio=(PaCO2-PetCO2)/PaCO2), oxygenation index and arterial-alveolar partial pressure difference for oxygen ((A-a)DO2) were elucidated and correlated with pulmonary mechanics. Oxygenation index and (A-a)DO2 improved, and VD/VT ratio decreased progressively after surfactant administration, becoming significantly different from the baseline at 30 min and thereafter with administration of surfactant. Pulmonary mechanics did not change significantly during the observation period. VD/VT ratio showed close correlation with OI and (A-a)DO2, but not with pulmonary mechanics. These results suggest that decreased physiologic dead space resulting from the recruitment of atelectatic alveoli rather than improvement in pulmonary mechanics is primarily responsible for the improved oxygenation after surfactant therapy in the RDS of newborn.
Airway Resistance ; Human ; Infant, Newborn ; Lung/physiopathology* ; Lung Compliance ; Pulmonary Gas Exchange ; Pulmonary Surfactants/therapeutic use* ; Respiratory Dead Space* ; Respiratory Distress Syndrome/physiopathology ; Respiratory Distress Syndrome/drug therapy* ; Tidal Volume*

PURPOSE: This study was designed to assess the effects of cyclic parenteral nutrition(CP) on parenteral nutrition induced liver diseases. METHODS: Before and after CP, data were collected on diagnosis, age, duration of parenteral nutrition, macronutrients and biochemical parameters. Initially parenteral nutrition was cycled off for 1-2 hours and the off-time was advanced by 0.5-1 hour daily over 1 week. Blood and urine glucose were monitored during procedure. RESULTS: Data on 6 sets of CP in 4 patients, among whom 2 patients had undertaken CP 2 times, respectively, were analyzed. The mean age was approximately 4 months, ranging from 2 to 11 months. Underlying diseases were as follows : 2 cases of microvillous inclusion disease, 1 case of protracted diarrhea of infancy and 1 case of feeding intolerance. The mean duration of parenteral nutrition before CP were 38.6 days, and that of CP was 41.6 days. During CP, the mean total caloric intake of each patient was 107kcal/kg/day, and the mean weight gain was 6.0g/kg/day. After CP, the biochemical parameters changed as follows : bilirubin was decreased in 4 cases, not changed in 1 case but increased in 1 case who had sepsis during CP period; the level of ALT was decreased in 2 cases but increased in 2 cases and not changed in 2 cases. Overall, CP was tolerated well although 3 cases had hypoglycemia(serum glucose concentration less than 40mg/dL) at the initial CP period. CONCLUSION: CP has potential beneficial metabolic effects on total parenteral nutrition induced cholestasis with minimal complications.
Bilirubin ; Cholestasis* ; Cytomegalovirus Infections ; Diagnosis ; Diarrhea ; Energy Intake ; Glucose ; Humans ; Infant* ; Liver Diseases ; Parenteral Nutrition* ; Parenteral Nutrition, Total* ; Sepsis ; Weight Gain

PURPOSE: This study was designed to assess the effects of cyclic parenteral nutrition(CP) on parenteral nutrition induced liver diseases. METHODS: Before and after CP, data were collected on diagnosis, age, duration of parenteral nutrition, macronutrients and biochemical parameters. Initially parenteral nutrition was cycled off for 1-2 hours and the off-time was advanced by 0.5-1 hour daily over 1 week. Blood and urine glucose were monitored during procedure. RESULTS: Data on 6 sets of CP in 4 patients, among whom 2 patients had undertaken CP 2 times, respectively, were analyzed. The mean age was approximately 4 months, ranging from 2 to 11 months. Underlying diseases were as follows : 2 cases of microvillous inclusion disease, 1 case of protracted diarrhea of infancy and 1 case of feeding intolerance. The mean duration of parenteral nutrition before CP were 38.6 days, and that of CP was 41.6 days. During CP, the mean total caloric intake of each patient was 107kcal/kg/day, and the mean weight gain was 6.0g/kg/day. After CP, the biochemical parameters changed as follows : bilirubin was decreased in 4 cases, not changed in 1 case but increased in 1 case who had sepsis during CP period; the level of ALT was decreased in 2 cases but increased in 2 cases and not changed in 2 cases. Overall, CP was tolerated well although 3 cases had hypoglycemia(serum glucose concentration less than 40mg/dL) at the initial CP period. CONCLUSION: CP has potential beneficial metabolic effects on total parenteral nutrition induced cholestasis with minimal complications.
Bilirubin ; Cholestasis* ; Cytomegalovirus Infections ; Diagnosis ; Diarrhea ; Energy Intake ; Glucose ; Humans ; Infant* ; Liver Diseases ; Parenteral Nutrition* ; Parenteral Nutrition, Total* ; Sepsis ; Weight Gain

PURPOSE: This study was carried out to elucidate the effects of nitric oxide synthase(NOS) inhibitor, NG-monomethyl-L-arginine(L-NMMA) and nitric oxide precursor, L-arginine(L-Arg) on cerebral hemodynamics and energy metabolism during reoxygenation-reperfusion(RR) after hypoxia-ischemia(HI) in newborn piglets. METHODS: Twenty-eight newborn piglets were divided into 4 groups; Sham normal control(NC), experimental control(EC), L-NMMA(HI & RR with L-NMMA), and L-Arg(HI & RR with L-Arg) groups. HI was induced by occlusion of bilateral common carotid arteries and simultaneously breathing with 8 percent oxygen for 30 mins, and followed RR by release of carotid occlusion and normoxic ventilation for one hour. All groups were monitored with cerebral hemodynamics and cytochrome aa3 (Cyt aa3) using near infrared spectroscopy(NIRS). Na+, K(+)-ATPase activity, lipid peroxidation products, and tissue high energy phosphate levels were determined biochemically in the cerebral cortex. RESULTS: In experimental groups, mean arterial blood pressure, PaO2, and pH decreased, and base excess and blood lactate level increased after HI compared to NC group(P<0.05). These variables subsequently returned to baseline after RR except pH. There were no differences among the experimental groups. In NIRS, oxidized hemoglobin(HbO2) decreased and hemoglobin(Hb) increased during HI(P<0.05) but returned to base line immediately after RR; 40 min after RR, the HbO2 had decreased significantly compared to NC group(P<0.05). Changes of Cyt aa3 decreased significantly compared to NC after HI and recovered at the end of the experiment. Significantly reduced cerebral cortical cell membrane Na+, K(+)-ATPase activity and increased lipid peroxidation products(P<0.05) were not improved with L-NMMA or L-Arg. CONCLUSION: These findings suggest that NO is not involved in the mechanism of HI and RR brain damage during the early acute phase of RR.
Anoxia ; Arginine* ; Arterial Pressure ; Brain ; Carotid Artery, Common ; Cell Membrane ; Cerebral Cortex ; Electron Transport Complex IV ; Energy Metabolism* ; Hemodynamics* ; Humans ; Hydrogen-Ion Concentration ; Hypoxia-Ischemia, Brain* ; Infant, Newborn* ; Ischemia ; Lactic Acid ; Lipid Peroxidation ; Nitric Oxide ; omega-N-Methylarginine* ; Oxygen ; Perfusion ; Respiration ; Ventilation