PURPOSE: Childhood obesity is associated with nonalcoholic fatty liver disease (NAFLD), and it has become one of the most common causes of childhood chronic liver diseases which significant as a cause of liver related mortality and morbidity in children in the United States. The development of simpler and easier clinical indices for medical practice is needed to identify advanced hepatic fibrosis in childhood NAFLD instead of invasive method like liver biopsy. FibroScan and aspartate aminotransferase (AST)-to-platelet ratio index (APRI) have been proposed as a simple and noninvasive predictor to evaluate hepatic fibrosis in several liver diseases. APRI could be a good alternative to detect pathologic change in childhood NAFLD. The purpose of this study is to validate the efficacy of APRI for assessing hepatic fibrosis in childhood NAFLD based on FibroScan. METHODS: This study included 23 children with NAFLD who underwent FibroScan. Clinical, laboratory and radiological evaluation including APRI was performed. To confirm the result of this study, 6 patients received liver biopsy. RESULTS: Factors associated with hepatic fibrosis (stiffness measurement >5.9 kPa Fibroscan) were triglyceride, AST, alanine aminotransferase, platelet count, APRI and collagen IV. In multivariate analysis, APRI were correlated with hepatic fibrosis (>5.9 kPa). In receiver operating characteristics curve, APRI of meaningful fibrosis (cutoff value, 0.4669; area under the receiver operating characteristics, 0.875) presented sensitivity of 94%, specificity of 66%, positive predictive value of 94%, and negative predictive value of 64%. CONCLUSION: APRI might be a noninvasive, simple, and readily available method for medical practice to predict hepatic fibrosis of childhood NAFLD.
Alanine Transaminase ; Aspartate Aminotransferases ; Aspartic Acid ; Biopsy ; Child ; Collagen ; Fatty Liver ; Fibrosis ; Humans ; Liver ; Liver Diseases ; Multivariate Analysis ; Obesity ; Platelet Count ; ROC Curve ; Sensitivity and Specificity ; United States

PURPOSE: The selection of the recipient vessels in breast reconstruction has a great influence on the surgical result and the shape of the reconstructed breast. We would like to introduce the criteria for the selection of recipient vessels in delayed reconstruction of the breast. METHODS: We studied 56 patients with delayed breast reconstruction using free TRAM flaps from April 1994 to December 2006. The thoracodorsal and the ipsilateral internal mammary vessels were used as recipients in 25 patients each, the opposite internal mammary vessels in 3 patients, the thoracoacromial vessels in 2 patients, and the transverse cervical artery with the cephalic vein in 1 patient. The survival rate of the flaps, the vessel diameter, the length of the pedicles, and the convenience of vessel dissection were studied. RESULTS: The diameter of the recipient vessel did not influence the anastomosis. The operation time, the survival rate of flap, the postoperative complications showed no significant difference according to the recipient vessel. Dissection of the thoracodorsal vessels was tedious due to scar formation from the prior operation. Dissection of the internal mammary vessels proved to be relatively easy, and the required length of the pedicle was shorter than any other site, but the need for removal of rib cartilage makes this procedure inconvenient. CONCLUSION: The first choice of the recipient vessel in immediate breast reconstruction is the thoracodorsal vessels, but in cases of delayed reconstruction the internal mammary vessels are favored as the first choice, because the thoracodorsal vessels have a high unusability rate. If the ipsilateral internal mammary vessels prove to be useless, the contralateral vessels can be used. The thoracoacromial vessels are useful, when the mastectomy scar is located in the upper portion. The transverse cervical artery and the cephalic vein can serve as the last resort, if all other vessels are unreliable.
Arteries ; Breast* ; Cartilage ; Cicatrix ; Female ; Health Resorts ; Humans ; Mammaplasty* ; Mastectomy ; Postoperative Complications ; Ribs ; Survival Rate ; Veins

Hallermann-Streiff syndrome is a very rare congenital disorder, which is primarily characterized by the head and face abnormalities. Approximately 180 cases have been reported worldwide, including 8 cases in Korea since it was first described by Hallermann in 1893. Patients exhibit a bird-like face, hypotrichosis, atrophy of skin, dental abnormalities, proportionate nanism, and various ophthalmic disorders, including congenital cataracts and bilateral micropthalmia. As a result of many life-threatening complications, such as respiratory and cardiac difficulties, many patients die during infancy. We report here two cases of HSS diagnosed immediately after birth with literature reviews. They showed two additional characteristics, including chubby cheeks and antenatal ultrasonographic findings, which have not been mentioned in previous reports.
Atrophy ; Cataract ; Cheek ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Dwarfism ; Hallermann's Syndrome ; Head ; Humans ; Hypotrichosis ; Korea ; Microphthalmos ; Parturition ; Skin

In Crohn's disease, mild gastrointestinal bleeding often occurs; however massive gastrointestinal hemorrhage, which can have a dramatic effect on a patient's vital sign, is rare. This could result in potentially life-threatening complications, which can lead to death. Massive hemorrhagic Crohn's disease is not well known and for this reason, they are a diagnostic and therapeutic challenge. Various diagnostic and therapeutic methods are currently being developed and used. The surgical method is often used only as a last measure since this approach has the risk of serious complications that may endanger patients. However, if massive bleeding continues even after all therapeutic methods are used, the surgical method must be implemented. In this case, all therapeutic methods were found to be ineffective; therefore, surgery was used as a last option. Ultimately, the surgical method was found to be successfully used to treat life-threatening hemorrhagic Crohn's disease.
Crohn Disease ; Gastrointestinal Hemorrhage ; Hemorrhage ; Humans ; Vital Signs

Noonan syndrome is an autosomal dominant, multisystem disorder. Autoimmune thyroiditis with hypothyroidism is an infrequent feature in patients with Noonan syndrome. A 16-year-old boy was admitted because of chest discomfort and dyspnea; an echocardiogram revealed pericardial effusion. Additional investigations led to a diagnosis of severe hypothyroidism due to Hashimoto thyroiditis. The patient was treated with L-thyroxine at 0.15 mg daily. However, during admission, he developed symptoms of cardiac tamponade. Closed pericardiostomy was performed, after which the patient's chest discomfort improved, and his vital signs stabilized. Herein, we report a case of an adolescent with Noonan syndrome, who was diagnosed with Hashimoto thyroiditis with an unusual presentation of cardiac tamponade.
Adolescent ; Cardiac Tamponade* ; Diagnosis ; Dyspnea ; Hashimoto Disease* ; Humans ; Hypothyroidism ; Male ; Noonan Syndrome* ; Pericardial Effusion ; Pericardial Window Techniques ; Thorax ; Thyroiditis, Autoimmune ; Thyroxine ; Vital Signs

Chronic pancreatitis is a progressive inflammatory disease resulting from repeated episodes of acute pancreatitis that impair exocrine function and eventually produce endocrine insufficiency. Some causes of chronic pancreatitis appear to be associated with alterations in the serine-protease inhibitor, Kazal type 1 (SPINK1), cationic trypsinogen (PRSS1), and cystic fibrosis-transmembrane conductance regulator (CFTR) genes, or with structural disorders in the pancreaticobiliary ductal system, such as pancreatic divisum or anomalous pancreaticobiliary ductal union (APBDU). However, it is unusual to observe both genetic alteration and structural anomaly. Here, we report 2 cases with both APBDU and a mutation in the SPINK1 genes, and we discuss the implications of these findings in clinical practice.
Pancreatitis ; Pancreatitis, Chronic ; Trypsinogen

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.
Early Diagnosis ; Heart ; Heart Murmurs ; Humans ; Infant, Newborn ; Korea ; Recurrence ; Scimitar Syndrome* ; Siblings*

PURPOSE: Benign childhood epilepsy with centrotemporal spikes (BECTS) is known to have a good prognosis, and is easily controlled with antiepileptic drugs. In this study, we evaluate the efficacy of low-dose topiramate (TPM), once at night time in children with BECTS, and compare with that of oxcarbazepine (OXC). METHODS: OXC was used as a first drug in 58 patients with BECTS at the department of pediatrics, Chonnam National University hospital from Jan. 2003 to Nov. 2010. Their medical records were reviewed, focusing on the onset age of seizure, the dosage of drugs, response to treatment and other clinical features. Since Aug. 2008 through Apr. 2011, some newly diagnosed patients with BECTS were given a low-dose (25mg) of TPM, once at nighttime. The efficacy was reviewed in 14 children, who had been followed up in the outpatient clinic for longer than twelve months. RESULTS: Forty five out of 58 (77.6%) patients were well controlled with OXC. Thirteen out of 58 (22.4%), seizures recurred, on whom TPM (n=9) or lamotrigine (n=4) was added. Nine (64.3%) of the 14 patients who were started with low dose TPM became seizure-free for at least 12 months. However, Two out of 14, the dose of TPM had to be increased up to 100 mg/day and 75 mg/day, respectively. In three, seizure was controlled with OXC. CONCLUSION: A single low-dose TPM would be as effective as the conventional OXC without any adverse effects in children with BECTS.
Age of Onset ; Ambulatory Care Facilities ; Anticonvulsants ; Carbamazepine ; Child ; Epilepsy ; Epilepsy, Rolandic ; Fructose ; Humans ; Medical Records ; Pediatrics ; Prognosis ; Seizures ; Triazines

PURPOSE: Indomethacin has been reported as the prophylaxis and initial treatment of preterm infants with patent ductus arteriosus (PDA). However, there was controversy over indomethacin treatment in full-term infants with symptomatic PDA. Therefore, we evaluate the effect of indomethacin as a treatment of full-term infants with symptomatic PDA. METHODS: A retrospective study was performed to evaluate the effectiveness of indomethacin in full-term infants who had birth weight > or =2,500 g and a gestational age > or =37 weeks with symptomatic PDA at Chonnam National University Hospital between January 2007 and December 2009. According to responsiveness of indomethacin, we classified them into three groups: 1) complete responder which were completely closed after indomethacin treatment, 2) partial responder which were incompletely closed but symptoms were improved, 3) non responder which were conducted surgical ligation because did not respond. RESULTS: Among the total 29 full-term infants treated with indomethacin, 13 (44.8%) were complete responder, 8 (27.6%) were partial responder, and 8 (27.6%) were non responder. There were no significant differences in birth weight, narrow diameter of PDA, and dose of indomethacin between three groups. However, the age at initiation of treatment using indomethacin of complete (4.8+/-4.5 days, P=0.03) and partial responder (6.3+/-2.0 days, P=0.04) were earlier than those of non responder (13.8+/-8.1 days). CONCLUSION: Indomethacin can expect an effective treatment of PDA in full-term infants prior to surgical ligation.
Birth Weight ; Ductus Arteriosus, Patent* ; Gestational Age ; Humans ; Indomethacin* ; Infant* ; Infant, Newborn ; Infant, Premature ; Jeollanam-do ; Ligation ; Retrospective Studies

BACKGROUND: Hypercalciuria is one of the most common causes of unexplained isolated hematuria. The diagnostic methods for hypercalciuria have not yet been standardized. The aim of this study was to assess whether random urinary calcium/creatinine ratio could be used as a screening tool for hypercalciuria in children with hematuria. METHODS: This prospective study included 264 children with primary hematuria for whom both random and 24 hr urinary evaluations were performed. Pearson correlation and ROC curve were used to assess the correlations. A multiple linear regression model was used to analyze effects of age, weight, height, body mass index, and body surface area on random urinary calcium/creatinine ratio. RESULTS: There was a moderately strong correlation between random urinary calcium/creatinine ratio and 24 hr urinary calcium excretion (r=0.584, P<0.001). The most appropriate cutoff value of random urinary calcium/creatinine ratio for the estimation of hypercalciuria was 0.075 mg/mg (sensitivity, 77.8%; specificity, 64.3%; area under the curve, 0.778). Body mass index and 24 hr urinary calcium excretion significantly affected random urinary calcium/creatinine ratio with a low coefficient of determination (r2=0.380, P<0.001). CONCLUSIONS: Random urinary calcium/creatinine ratio is not suitable for screening hypercalciuria in children with hematuria. Twenty-four hour urinary analysis should be performed to diagnose hypercalciuria in children with hematuria.
Adolescent ; Area Under Curve ; Body Mass Index ; Calcium/*urine ; Child ; Child, Preschool ; Creatinine/*urine ; Female ; Hematuria/*complications ; Humans ; Hypercalciuria/*complications/*diagnosis ; Linear Models ; Male ; Prospective Studies ; ROC Curve ; Sensitivity and Specificity