Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirrned by kidney biopsy due to her mother's refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome. She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. (J Korean Pediatr Soc 2000;43-837-841)
Acceleration ; Adolescent ; Biopsy ; Blood Glucose ; Cataract ; Diabetes Mellitus* ; Diet, Diabetic ; Diabetic Nephropathies ; Diabetic Retinopathy ; Disulfiram ; Education ; Female ; Glycogen ; Hepatocytes ; Hepatomegaly ; Humans ; Insulin ; Kidney ; Liver ; Obesity, Abdominal ; Physical Examination ; Sexual Maturation

PURPOSE: This study tries to examine the effects of hand moxibustion and press pellet by applying them to male manufacturing workers with low back pain, and use them to develop nursing interventions for workers. METHODS: The data were collected from August to October, 2018, and the subjects were 60 men, processed by using SPSS/WIN 21.0 to perform homogeneity test with χ²-, t-, and hypothesis tests with repeated measures, ANOVA and Scheffé test. RESULTS: Hypothesis 1 that “the experimental group provided with hand moxibustion and hand press pellet would give lower scores for low back pain than the control group” was supported (F=78.71, p<.001). Hypothesis 2 that “the experimental group provided with hand moxibustion and hand press pellet would have a wider range of motion than the control group” was also supported (F=17.44, p<.001). Hypothesis 3 that “the experimental group provided with hand moxibustion and hand press pellet would give lower scores for depression than the control group,” again, was supported (F=16.95, p<.001). CONCLUSION: Hand moxibustion and hand press pellet are effective in relieving low back pain for male workers, in increasing the range of motion, and in decreasing depression.
Depression ; Hand ; Humans ; Joints ; Low Back Pain ; Male ; Moxibustion ; Nursing ; Range of Motion, Articular

Apert syndrome is an uncommon congenital disorder characterized by malformation of the skull in association with symmetrical syndactyly of both hands and feet. This syndrome is autosornal dominant. The original description was presented by Apert in 1906. Since then more than 200 cases have been reported in the world. Recently, we experienced a case of newhorn male infant with congenital anomalies of the skull and extremities. Molecular biologically, he was found to have Ser252Try mutation in the FGFR2 exonIIIa. A brief review of literature was made.
Acrocephalosyndactylia ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Extremities ; Fibroblast Growth Factors* ; Fibroblasts* ; Foot ; Hand ; Humans ; Infant ; Male ; Receptor, Fibroblast Growth Factor, Type 2* ; Receptors, Fibroblast Growth Factor* ; Skull ; Syndactyly

BACKGROUND/AIMS: Although intravenous proton pump inhibitor (PPI) has been used for the prevention of post endoscopic submucosal dissection (ESD) bleeding, the route of administration has not been confirmed. The aim of the present study was to compare the efficacy of intravenous and oral PPI administration for the prevention of delayed post ESD bleeding. METHODS: Total 166 consecutive patients were randomly assigned to 30 mg lansoprazol twice a day (PO group) and 120 mg pantoprazole intravenous injection (IV group) for 48 hours. Finally, 65 patients in PO group and 87 patients in IV group were analyzed. After ESD, all patients underwent follow up endoscopy after 24 hours and were observed the symptoms of bleeding up to 60 days after ESD. RESULTS: Age, sex and use of anticoagulants were not different between groups. At follow up endoscopy after 24 hours, oozing and exposed vessel was noted in 4.6% of PO group and 8.0% of IV group and there was no significant difference. Delayed bleeding occurred in 4 of 65 patients (6.2%) in the PO group and 8 of 87 patients (9.2%) in the IV group (p>0.999). By multivariate analysis, oozing or exposed vessels at follow up endoscopy were risk factors for delayed bleeding (OR=17.5, p=0.022). CONCLUSIONS: There was no significant difference in the delayed bleeding, length of hospital stay according to the administration route. Bleeding stigmata at follow up endoscopy was risk factor of delayed bleeding. Oral PPI administration can cost-effectively replace IV PPI for prevention of post ESD bleeding.
2-Pyridinylmethylsulfinylbenzimidazoles/therapeutic use ; *Administration, Oral ; Aged ; Anticoagulants/therapeutic use ; Endoscopic Mucosal Resection/*adverse effects ; Female ; Gastroscopy ; Humans ; *Injections, Intravenous ; Lansoprazole/therapeutic use ; Male ; Middle Aged ; Odds Ratio ; Postoperative Hemorrhage/etiology/*prevention & control ; Prospective Studies ; Proton Pump Inhibitors/*therapeutic use ; Risk Factors ; Stomach Neoplasms/surgery

BACKGROUND/AIMS: Relapse in inflammatory bowel disease (IBD) is not predictable, although several factors have been suggested. The aims of the current study were to assess and compare the possible causes of clinical relapse as perceived by patients and parents. METHODS: Of 107 young (<35 years old) IBD patients, 26 patients who experienced recent (<3 month) relapse and their parents completed a questionnaire at the same time. Baseline characteristics and clinical manifestations were reviewed and the most common causes of relapse as perceived by patients and parents were compared. RESULTS: Median patient age was 22.5 years and the male to female ratio was 17:9. Crohn's disease was diagnosed in 23 patients and ulcerative colitis in the other three patients. Mean disease duration was 39.8+/-30.3 months. Eighteen (69.2%) patients experienced stress before relapse. Fifteen (57.7%) parents thought that their children experienced stress before relapse. Agreement between patients and parents for non-adherence to medication and stress was 100% and 73.1%, respectively. Stress was considered the most likely cause of relapse in both groups. Discordance rate between parents and patients with respect to main causes of relapse was 40.4%. CONCLUSIONS: Stress was perceived to be the most common condition noted before clinical relapse in young IBD patients and their parents. However, the discordance rate between patients and parents with respect to the main causes of relapse was 40.4%. This result suggests a considerable difference in terms of disease understanding between young IBD patients and parents.
Adolescent ; Adult ; Colitis, Ulcerative/diagnosis/psychology ; Crohn Disease/diagnosis/psychology ; Female ; Humans ; Inflammatory Bowel Diseases/*diagnosis/psychology ; Male ; Medication Adherence ; Parents/*psychology ; Recurrence ; Stress, Psychological ; Surveys and Questionnaires ; Young Adult

Langerhans cell histiocytosis can cause central diabetes insipidus. Here, a case of Langerhans cell histiocytosis invading the pituitary stalk was experienced. The patient was 15 years old boy, with complaint of polydipsia and polyuria. A water deprivation test was carried out, and the urine osmolarity was increased from 165 to 469 mosm/kg following an injection of AVP to confirm the diagnosis of central diabetes insipidus. A pituitary function stimulation test gave a normal response. A sellar MRI was performed, which showed a thickened pituitary stalk mass (about 5.7mm), with an increased size, 6.9 mm, on a second MRI 2 month later. A tissue biopsy was performed, which showed aggregates of histiocytes and inflammatory cells, with prominent eosinophils (H&E), and also revealed strong reactivity to anti-CD1a antibody on the immunohistochemistry. After confirmative tissue diagnosis, the patient received radiotherapy (900 cGy). The thickened mass of the pituitary stalk disappeared on the MRI following the radiotherapy. The patient was managed with DDAVP nasal spray, after which the polyuric symptoms were completely relieved.
Adolescent ; Biopsy ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic* ; Diagnosis ; Eosinophils ; Histiocytes ; Histiocytosis, Langerhans-Cell* ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Male ; Osmolar Concentration ; Pituitary Gland ; Polydipsia ; Polyuria ; Radiotherapy ; Water Deprivation

The purpose of this paper is to introduce standardization activities of nursing documentation for special nursing units following standardization efforts for general nursing units last year. Modified Delphi approach with expert panel was used to identify essential nursing documents and data set for each units. Expert panel was consisted of head nurses or charge nurses of each special nursing unit from 8 tertiary hospitals with more than 500 beds in Seoul. the secretary-general of Clinical Nurses Association and a faculty of College of Nursing. The exiting nursing forms of seven special nursing units, which include Emergency room, Intensive care unit, Operating room, Respiratory intensive care unit. Delivery floor. Nursery and Dialysis room, were analyzed and prototypes of the standard nursing forms and guidelines were developed. The clinical field test was done with the help of Clinical Nurses Association. At the field test 3.744 clinical staff nurses from 20 tertiary hospitals with more than 500 beds in Korea were involved and provided feedback. Finally public hearing was held and more than 600 nurses from 116 hospitals attended and provided feedback. Through these process consensus of nursing community was attained for standard documents and data items. The result is available at http://nursing.snu.ac.kr/standard/ through internet.
Consensus ; Dataset ; Dialysis ; Emergency Service, Hospital ; Hearing ; Intensive Care Units ; Internet ; Korea ; Nurseries ; Nursing* ; Nursing, Supervisory ; Operating Rooms ; Seoul ; Tertiary Care Centers

BACKGROUND/AIMS: The eradication rates of Helicobacter pylori infection have been reported to have decreased over the years due to antibiotics resistance. The aim of this study is to investigate the trend of eradication rates of first-line triple therapy for H. pylori over the past 13 year period, and to evaluate factors affecting H. pylori eradication in Daegu and Gyeongsangbuk-do, Korea. METHODS: A total of 2,982 patients with H. pylori infection who were treated with either 1 week or 2 weeks first-line therapy (proton pump inhibitor [PPI], amoxicillin, and clarithromycin) from January 1999 through December 2011 were included in this study. Data were collected by retrospectively reviewing the medical records. RESULTS: The overall H. pylori eradication rate was 87.2%. The eradication rates from 1999 to 2011 fluctuated between 78.0% and 95.7%, but no definite evidence of a decreasing tendency was seen over the 13 year period (p=0.113). Furthermore, there was no significant difference in the eradication rate according to the duration of therapy (p=0.592). However, there was a significant difference in the eradication rate among various PPIs (p<0.01). CONCLUSIONS: There was no decreasing trend in the H. pylori eradication rate over the past 13 years in Daegu and Gyeongsangbuk-do, Korea. There also was no difference in the eradication rates depending on duration of therapy. However, a significant difference was noted among various PPIs.
Adult ; Aged ; Amoxicillin/therapeutic use ; Anti-Bacterial Agents/*therapeutic use ; Clarithromycin/therapeutic use ; Disease Eradication/*trends ; Drug Administration Schedule ; Drug Therapy, Combination ; Endoscopy, Gastrointestinal ; Esomeprazole/therapeutic use ; Female ; Helicobacter Infections/*drug therapy/pathology ; *Helicobacter pylori ; Humans ; Logistic Models ; Male ; Middle Aged ; Omeprazole/therapeutic use ; Proton Pump Inhibitors/*therapeutic use ; Rabeprazole/therapeutic use ; Republic of Korea ; Retrospective Studies ; Treatment Outcome

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: (1) definition and diagnosis of overweight and obesity in children and adolescents; (2) principles of treatment of pediatric obesity; (3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; (4) pharmacotherapy; and (5) bariatric surgery.
Adolescent ; Bariatric Surgery ; Child ; Diagnosis* ; Diet ; Drug Therapy ; Gastroenterology* ; Humans ; Korea ; Life Style ; Mental Health ; Obesity ; Overweight ; Pediatric Obesity*

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: 1) definition and diagnosis of overweight and obesity in children and adolescents; 2) principles of treatment of pediatric obesity; 3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; 4) pharmacotherapy; and 5) bariatric surgery.
Adolescent ; Bariatric Surgery ; Child ; Diagnosis* ; Diet ; Drug Therapy ; Gastroenterology* ; Humans ; Korea ; Life Style ; Mental Health ; Obesity ; Overweight ; Pediatric Obesity*