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.

PURPOSE: Adenoid hypertrophy is a physical alteration that may affect speech, and a speech disorder can have other negative effects on a child's life. Airway obstruction leads to constricted oral breathing and causes postural alterations of several oro-facial structures, including the mouth, tongue, and hyoid bone. The postural modifications may affect several aspects of speech production. METHODS: In this study, we compared articulation errors in 19 children with adenoid hypertrophy (subject group) to those of 33 children with functional articulation disorders independent of anatomical problems (control group). RESULTS: The mean age of the subject group was significantly higher (P=0.016). Substitution was more frequent in the subject group (P=0.003; odds ratio [OR], 1.80; 95% confidence interval [CI], 1.23-2.62), while omission was less frequent (P<0.001; OR, 0.43; 95% CI, 0.27-0.67). Articulation errors were significantly less frequent in the palatal affricative in the subject group (P=0.047; OR, 0.25; 95% CI, 0.07-0.92). The number of articulation errors in other consonants was not different between the two groups. Nasalization and aspiration were significantly more frequent in the subject group (P=0.007 and 0.014; OR, 14.77 and 0.014; 95% CI, [1.62-135.04] and NA, respectively). Otherwise, there were no differences between the two groups. CONCLUSION: We identified the characteristics of articulation errors in children with adenoid hypertrophy, but our data did not show the relationship between adenoid hypertrophy and oral motor function that has been observed in previous studies. The association between adenoid hypertrophy and oral motor function remains doubtful.
Adenoids* ; Airway Obstruction ; Articulation Disorders ; Child* ; Humans ; Hyoid Bone ; Hypertrophy* ; Mouth ; Mouth Breathing ; Odds Ratio ; Respiration ; Tongue

No abstract available.

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*

A case of recurrent rhinocerebral mucormycosis that has occurred during an induction chemotherapy for acute megakaryocytic leukemia in a 10 year-old boy is reported. He had suffered from high fever, proptosis, right eye ball pain and necrotic inflammation of hard palate during the chemotherapy of leukemia. CT scan of the paranasal sinus showed inflammatory change of right ethmoid and maxillary sinuses, and right orbital cystic mass which displaced medial rectus muscle. Pathologic examination of the inflammatory mass revealed mucormycosis with characteristic hyphae invading vessel walls. He was managed with 2 times of extensive debridement of necrotic tissue and currettage of cystic mass, and intravenous amphotericin-B for 80 days with apparent improvement. Seven months after discharge from the hospital, necrosis of posterior nasal septum and hard palate was noted for second time. It was managed again with 2 times of extensive debridement. Since this last operation he is on follow-up for 16 months uneventfully and is on therapy with low dose Ara-C in continuous remission.
Child ; Cytarabine ; Debridement ; Drug Therapy ; Exophthalmos ; Fever ; Follow-Up Studies ; Humans ; Hyphae ; Induction Chemotherapy ; Inflammation ; Leukemia ; Leukemia, Megakaryoblastic, Acute ; Male ; Maxillary Sinus ; Mucormycosis* ; Nasal Septum ; Necrosis ; Orbit ; Palate, Hard ; Tomography, X-Ray Computed

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*