PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver

No abstract available.
Female ; HELLP Syndrome* ; Pregnancy

OBJECTIVES: Although previous studies have suggested that Type 1 or Type C personality may be associated with the development of cancer, the results have not been consistent. There have been some evidences that repression, denial, or non-expression of anger are related with the incidence of colorectal cancer. However, according to the results of recent researches, aggressive hostility was related to the development of colorectal cancer. This study attempted to delineate psychological characteristics or personality patterns of the patients with colorectal cancer based on multidemensions of anger and Type 1 personality for cancer development. METHOD: The subjects were composed of 35 patients with colorectal cancer and 37 normal controls. Grossarth-Maticek personality questionnaire and the Spielberger state-trait anger expression inventory were administered. RESULTS: In comparison with the normal control group, the colorectal cancer patient group showed significantly higher scores on anger experience, angry temperament and state anger but no significant differences on Type 1 personality scale and other subscales. In the discriminant analysis patients and control groups were classified by means of state anger, angry temperament, angry reaction, anger-in, anger-out and anger control variables. When angry temperament variable was selected as a discriminant variable, 65.8% of cases had been correctly classified. CONCLUSIONS: These results suggested the relationship between the disposition to experience and express anger without particular provocation and the development of cancer.
Anger* ; Colorectal Neoplasms* ; Denial (Psychology) ; Hostility ; Humans ; Incidence ; Surveys and Questionnaires ; Repression, Psychology ; Temperament

No abstract available.
Exotropia*

Nasoalveolar molding (NAM) device is an effective treatment for protruding maxilla in infants with cleft palate. However, only a few studies have investigated the effect of NAM devices on the treatment of protruding maxilla in infants with cleft lip only. We have designed a combination treatment using NAM devices prior to cheiloplasy for cleft lip-only patients with severe anterior protrusion of the premaxilla. Three cleft lip-only infants with 1-cm or more of premaxilla protrusion were included. Definitive cheiloplasty was performed at 6 months of age without any preoperative correction in infant 1. Cheiloplasty was performed in conjunction with the use of NAM device and lip adhesion in infants 2 and 3. Postoperative columella length and anterior-posterior dimension of the protruding premaxilla were compared amongst the infants. We were able to obtain satisfactory postoperative columella length and general nasal appearance.
Cleft Lip* ; Cleft Palate ; Fungi* ; Humans ; Infant ; Lip* ; Maxilla ; Orthodontic Appliances

PURPOSE: The purpose of this study is to evaluate the clinical and radiologic results of using Acutrak screws for treating ankle medial malleolar fracture. MATERIALS AND METHODS: We reviewed 38 cases of ankle medial malleolar fracture treated with Acutrak screws from February 2005 to May 2008. RESULTS: In clinical result, there were 30 exellent cases, 7 good cases, 1 fair case. In radiologic result, there is no case with reduction loss. Average union time is 10.5 weeks. CONCLUSION: We conclude that Acutrak screw fixation is a useful method for ankle medial malleolar fracture, there are many advantages in accurate anatomical reduction, small incision, short operative time.
Animals ; Ankle ; Operative Time

The term "disorders of sex development" (DSD) includes congenital conditions in which development of chromosomal, gonadal or anatomical sex is atypical. Steroid 5-alpha reductase type 2 deficiency (5alpha-RD2) is an uncommon autosomal recessive disorder of sexual differentiation. It results from impaired conversion of testosterone (T) to dihydrotestosterone (DHT) due to mutations in the steroid 5-alpha reductase type 2 (SRD5A2) gene. It is characterized by a lack of masculinization in XY individuals due to failure to convert testosterone to dihydrotestosterone. More than 40 mutations have been reported in all five exons of the SRD5A2 gene. Here, we report on a 17-day-old Korean newborn who was confirmed to have 5alpha-RD2 by SRD5A2 gene analysis. He manifested micropenis, hypospadia and bilateral cryptorchidism without skin hyperpigmentation. T/DHT ratio after human chorionic gonadotropin (hCG) stimulation was slightly increased and genetic analysis of SRD5A2 revealed compound heterozygous mutations, c.657C > G (p.Phe219Leu) and c.656del (p.Phe219SerfsX60), the former of which is a novel mutation. We report a novel SRD5A2 gene mutation in a Korean newborn with 5alpha-RD2.
Infant, Newborn ; Humans

In this report, we present a scalp defect reconstruction with lateral arm free flap. We highlight the difficulty in obtaining a recipient vein and the venous drainage managed through an open end of the donor vein. A 52-year-old woman presented with a pressure sore on the left scalp. A lateral arm free flap was transferred to cover this 8x6 cm defect. The arterial anastomosis was successful, but no recipient vein could be identified within the wound bed. Instead, we used a donor venous end for the direct open venous drainage. In order to keep this exposed venous end patent, we applied heparin-soaked gauze dressing to the wound. Also, the vein end was mechanically dilated and irrigated with heparin solution at two hour intervals. Along with fluid management and blood transfusion, this management was continued for the five days after the operation. The flap survived well without any complication. Through this case, we were able to demonstrate that venous congestion can be avoided by drainage of the venous blood through an open vessel without the use of leeches.
Arm ; Bandages ; Blood Transfusion ; Drainage* ; Estrogens, Conjugated (USP)* ; Female ; Free Tissue Flaps ; Heparin ; Humans ; Hyperemia ; Leeches ; Middle Aged ; Pressure Ulcer ; Salvage Therapy ; Scalp ; Tissue Donors ; Veins ; Wounds and Injuries

Biliary-enteric fistula is a rare disease, and the common causes of biliary-enteric fistula are gallstone, peptic ulcer, malignancy and trauma. It is known that the most common type of biliary-enteric fistula is the cholecysto-duodenal fistula, yet the combination of choledocho-duodeno-colonic fistula is a rare finding. A 78-year-old woman was admitted because she had suffered with right upper quadrant pain, a febrile sense and chills for 2 days. We confirmed the choledocho-duodeno-colonic fistula by performing gastroduodenoscopy, abdominal CT and an upper GI series. So, we report here on an usual case of choledocho-duodeno-colonic fistula, along with a review of the relevant literatures.
Aged ; Biliary Fistula ; Chills ; Female ; Fistula* ; Gallstones ; Humans ; Peptic Ulcer ; Rare Diseases ; Tomography, X-Ray Computed

Short-chain acyl-CoA dehydrogenase deficiency (SCADD; OMIM # 201470) is an autosomal recessive inborn error of mitochondrial fatty acid beta-oxidation, presenting with a variety of clinical signs and symptoms. Developmental delay, hypertonia or hypotonia, ketotic hypoglycemia, and epilepsy are most frequently reported. In general, patients diagnosed through newborn screening have shown normal growth and development in contrast to those diagnosed as a result of clinically initiated evaluations. Here, the case of an asymptomatic Korean newborn with SCADD identified by tandem mass spectrometry is reported. The patient showed an elevated concentration of butyrylcarnitine detected on newborn screening. Urinary excretion of ethylmalonic acid was elevated by urine organic acid analysis. To confirm the diagnosis of SCADD, a direct sequencing analysis of 10 coding exons and the exon-intron boundaries of the ACADS gene were performed. Genetic analysis of ACADS showed the following novel compound heterozygous missense mutations: c.277C>A (p.Leu93Ile) on exon3 and c.682G>A (p.Glu288Lys) on exon6. These results will provide further evidence of mutational heterogeneity for SCADD.
Acyl-CoA Dehydrogenase ; Butyryl-CoA Dehydrogenase ; Carnitine ; Clinical Coding ; Databases, Genetic ; Epilepsy ; Exons ; Growth and Development ; Humans ; Hypoglycemia ; Infant, Newborn ; Malonates ; Mass Screening ; Muscle Hypotonia ; Population Characteristics ; Tandem Mass Spectrometry