No abstract available.
Axilla ; Poroma*

Drash syndrome, which was first reported by Denys et al. in 1967 is a complex disorder which associates a nephropathy, Wilms' tumor, and male pseudohermaphroditism. The common denominator is a nephropathy. The nephropathy may be associated with either genital abnormalities or Wilms' tumor, and these associations are called incomplete form of Drash syndrome. This syndrome appears early in life and the first sign usually is genital ambiguity. The nephropathy presents with proteinuria, hematuria and hypertension, and eventually progresses to end stage renal failure. Renal biopsy may reveal a variety of glomerular and interstitial changes. Wilms' tumor may appear as s mass on ultrasound or it may not be recognized until nephrectomy or even autopsy. We report on a boy with nephropathy and genital abnormalities. A nephrotic syndrome with hypertension was present when first seen at 15 days of age. The karyotype was 46, XY and external genitalia was ambiguous. The nephrotic syndrome and signs of renal insufficiency persisted and he died at the age of 40 days. Histopathologic findings of kidney at autopsy revealed those of diffuse mesangial sclerosis. The case was presented with brief review of literatures.
46, XY Disorders of Sex Development ; Autopsy ; Biopsy ; Denys-Drash Syndrome* ; Disorders of Sex Development ; Genitalia ; Hematuria ; Humans ; Hypertension ; Karyotype ; Kidney ; Male ; Nephrectomy ; Nephrotic Syndrome ; Proteinuria ; Renal Insufficiency ; Sclerosis ; Ultrasonography ; Wilms Tumor

OBJECTIVE: The objective of this study is to compare the effectiveness and safety in the labor induction between the high dose oxytocin method and the new low dose oxytocin method. STUDY DESIGN: Firstly, we selected 125 pregnant women hospitalized, having the indication of labor induction from March, 1995 to August, 1996. Of them, we selected 61 pregnant women tothem the high dose oxytocin method was used, as the control group, and in- creased the quantity of 2.5 mU/min every 20 minutes with the start dose of 2.5 mU/min to them. On the other hand, with the start dose of 1.25 mU/min, we increased the quantity of 1.25 mU/min every 20 minutes to the study group of low dose oxytocin method, 64 pregnant women. RESULTS: No statistical significance was found in the time from the effective uterine contraction to the delivery in the study group, in contrast to that of the control group to them the labor induction was conducted by using the high dose oxytocin. Maximum amount used to the high dose oxytocin was significantly more than that of the low dose oxytacin, but in the total given dose, there was no significant difference between two groups. Maxi- mum uterine contraction of the control group did not show any significant. difference from that of the study group, and there was also no significant difference in the frequency of generating the complications such as fetal distress. CONCLUSION: There was no difference in the labor.induction -to delivery time, and the complications of fetus, between the existing high dose oxytocin method and the new low dose oxytocin method. Therefore it is thought the low dose oxytocin method may reduce the possibility of a complieation compared with the high dose oxytocin method. However, it is considered this matter must be investigated further in the futrre.
Female ; Fetal Distress ; Fetus ; Hand ; Humans ; Oxytocin* ; Pregnant Women ; Uterine Contraction

Phenylketonuria is metabolic disorder that results from a deficiency of the hepatic phenylalanine hydroxylase. But among patients with hyperphenylalaninemia, the defect resides in one of the enzymes necessary for production or recycling of tetrahydrobiopterin (BH4). The reduction of BH4 affects not only phenylalanine metabolism but also formation of the tyrosine related neurotransmitter, dopamin and tryptophan related neurotransmitter, serotonin. Administration of L-dopa and 5-hydroxytriptophan seems to be the most effective treatment and may prevent irreversible neurologic damage if started early in life in hyperphenylalaninemia due to deficiency of cofactor BH4. Therefore, all patients with PKU and hyperphenylalaninemia should be tested for BH4 deficiency as early as passible. So we measured reduced forms of biopterin in urines of 19 phenylketonuria patients by Funkushima and Nixon method and 13 of PKU patients measured dihydropterin reductase (DHPR) in white blood cells by modified Narisawa method. We could not find abnormal pterin patterns of cofactor BH4 and normal value of DHPR. All Korean 19 PKU children were classic PKU. A missense mutation has been identified in the phenylalanine hydroxylase (PAH) gene of 16 Koran PKU patients. 5 mutations (IVS4, Y204 C, R243Q, Y356 X, R413 P) have been identified. The frequency of these mutations was found to be 50% of PKU alleles. The IVS4 mutation had a high frequency in Korea and southern China, due to the result of the founder effect and genetic drift. the R413 P mutation, which may have originated in the regions surrounding the Baikal, expanded to northen China and Japan. We were not able to find Caucasian mutations in Korean ptiets. PKU mutations occured after racial divergence between Caucasian and Mongoloids. We observed that PKU patients with Y 204 C and R413 P mutations showed mild mild clinical phenotype but IVS4 mutation had severe mental retardation. the establishment of genotype will therefore aid in the prediction of clinical phenotypes in patients with this disease. So, pterin and DHPR measurement and DNA analsis will be useful for prognosis and proper treatment of PKU patients.
Alleles ; Biopterin ; Child ; China ; DNA* ; Founder Effect ; Genetic Drift ; Genotype ; Humans ; Intellectual Disability ; Japan ; Korea ; Leukocytes ; Levodopa ; Metabolism ; Mutation, Missense ; Neurotransmitter Agents ; Oxidoreductases ; Phenotype ; Phenylalanine ; Phenylalanine Hydroxylase ; Phenylketonurias ; Prognosis ; Recycling ; Reference Values ; Serotonin ; Tryptophan ; Tyrosine

No abstract available.
Phenylketonurias*

The objective of this study was to explore the psychosocial factors such as life events, coping styles and family relationships in middle-aged depressed women. This study was designed to compare how different variables relate to depression in two different test groups: a depressed patient group and a normal group. The Beck Depression Inventory(BDI), The Ways of Coping Checklist, Interpersonal Support Evaluation List(ISEL), Family Environment Scale were administered to 116 depressed patients and 113 normal persons between the ages of 35 and 64. The results were as follows 1) The highest stress was marital stress(n=42, 36.2%) in patient group and family stress(n=44, 38.9%) in normal group, respectively. 2) There were significant differences between patient group and normal group in BDI scores (t=15.94, p<.0001), lift events(t=4.73, p<.0001), active coping(t=6.29, p<.0001), social support(t=7.20, p<.0001), and family relationship(t=5.75, p<.0001) except for passive coping(t=0.93, p=.35). 3) In depressed patient group, BDI scores had a significantly positive correlation with the scores of the life event(r=.24, p<.01) and negative correlation with active coping(r= -.22, p<01), social support(r=-.35, p<.001) and family relationships(r=-.30, p<.001). 4) In depressed patient group, multiple regression analysis showed that social support(12.3%, beta=-.281, T=-3.162, P=.002), lift events(5.1%, beta=.279, T=3.195, F=.002), and active coping(3.5%, beta=-.204, T=-2.225, F=.028) had predictability on the BDI scores and the total predictability was 20.9%. 5) Stress experienced in family relationships were highest in life events and there was a lack of consistency(in the BDI scores ol the coping styles). Social support was the most important factor and there were no significant differences between the two groups in family relationships.
Checklist ; Depression ; Family Relations* ; Female ; Humans ; Psychology

PURPOSE: This study was conducted to investigate and analyze the association between stress from shift and non-shift work as well as the effects living habits have on eating habits in order to identify why and how workers can improve their health and form proper eating habits for higher working efficiency. METHODS: The subjects of this study were 361 workers from K manufacturing company from April 7 to 11, 2014 and they were surveyed using a questionnaire. The subjects were divided into two groups according to working pattern: shift workers (n = 216) and non-shift workers (n = 110). RESULTS: In the general characteristics, there were significant differences in age, work career, work time, marriage, monthly income, and education levels between the two groups. For healthy behaviors, significant differences in subjective health status, moderate physical activity, drinking, smoking, and sleep time were observed between shift workers and non-shift workers. For eating habits, scores of non-shift workers having a regular mealtime, balanced meal composition, and vegetable and seaweed intakes were significantly higher than those of shift workers. The sum score of dietary habits in non-shift workers was also significantly lower than that in shift workers (p < 0.05). Total job stress score did not significantly differ between the two groups. CONCLUSION: The sum of eating habit scores according to work types was 16.1 ± 0.6 in non-shift workers and 14.0 ± 0.3 in shift workers. These results suggest that it is necessary to provide food suitable to characteristics of different workers according to work type which should be provided along with daily nutrition counseling to help subjects recognize their status.
Counseling ; Diagnostic Self Evaluation ; Drinking ; Eating* ; Education ; Food Habits ; Gwangju* ; Marriage ; Meals ; Motor Activity ; Seaweed ; Smoke ; Smoking ; Vegetables

Bone Density ; Bone Regeneration ; Follow-Up Studies ; Humans ; Jaw ; Jaw Cysts ; Mandible ; Maxilla ; Transplants

OBJECTIVES: Patients with smell loss after craniocerebral trauma are known to have some brain abnormalities, but there was no study to analyze the findings according to the time interval between injury and evaluation. We aimed to identify whether the time interval may influence on the findings in the brain. METHODS: Medical records of 19 patients with posttraumatic olfactory dysfunction were reviewed. All of them underwent a magnetic resonance imaging and olfactory function tests. The patients were divided into early (n=10) and delayed (n=9) groups according to the time interval. RESULTS: Magnetic resonance imaging was taken at a mean time of 2.2 and 59.6 months after trauma in the early and delayed groups, respectively. Abnormal findings in the brain were found in 6 and 8 patients in the early and delayed groups, respectively. The olfactory bulb and orbitofrontal cortex were commonly affected olfactory pathways in both groups. In the early group, the abnormalities were brain tissue defect, hemorrhage, and focal edema whereas tissue defect was the only finding in the delayed group. In the early group, 5 of 6 patients with severe olfactory dysfunction showed brain abnormality while 1 of 4 patients with mild dysfunction had abnormality. In the delayed group, all the patients had severe dysfunction and 8 of 9 patients showed brain abnormality. CONCLUSION: Most patients with traumatic olfactory dysfunction had abnormality in the brain, and brain abnormality might be different according to the timing of evaluation. Furthermore, there might be an association between the severity of olfactory dysfunction and radiological abnormalities.
Brain ; Craniocerebral Trauma ; Edema ; Hemorrhage ; Humans ; Magnetic Resonance Imaging* ; Medical Records ; Olfactory Bulb ; Olfactory Pathways ; Smell

The 12th rib syndrome is a disease that causes pain between the upper abdomen and the lower chest. It is assumed that the impinging on the nerves between the ribs causes pain in the lower chest, upper abdomen, and flank. A 74-year-old female patient visited a pain clinic complaining of pain in her back, and left chest wall at a 7 on the 0-10 Numeric Rating scale (NRS). She had a lateral fixation at T12-L2, 6 years earlier. After the operation, she had multiple osteoporotic compression fractures. When the spine was bent, the patient complained about a sharp pain in the left mid-axillary line and radiating pain toward the abdomen. On physical examination, the 10th rib was not felt, and an image of the rib-cage confirmed that the left 10th rib was severed. When applying pressure from the legs to the 9th rib of the patient, pain was reproduced. Therefore, the patient was diagnosed with 9th rib syndrome, and ultrasound-guided 9th and 10th intercostal nerve blocks were performed around the tips of the severed 10th rib. In addition, local anesthetics with triamcinolone were administered into the muscles beneath the 9th rib at the point of the greatest tenderness. The patient's pain was reduced to NRS 2 point. In this case, it is suspected that the patient had a partial resection of the left 10th rib in the past, and subsequent compression fractures at T8 and T9 led to the deformation of the rib cage, causing the tip of the remaining 10th rib to impinge on the 9th intercostal nerves, causing pain.
Abdomen ; Abdominal Pain ; Aged ; Anesthetics, Local ; Female ; Fractures, Compression ; Humans ; Intercostal Nerves ; Leg ; Muscles ; Neuralgia ; Pain Clinics ; Physical Examination ; Ribs* ; Spine ; Thoracic Wall ; Thorax ; Triamcinolone