Schinzel-Giedion syndrome is a rare autosomal recessive condition characterized by a typical face including midface retraction, urogenital anomalies, and skeletal manifestations. So far 30 patients have been reported. This is the first report of this syndrome demonstrated in Korea. We describe a male infant with the typical findings of Schinzel-Giedion syndrome, also showing sacrococcygeal teratoma.
Humans ; Infant ; Korea ; Male ; Teratoma

This paper reflects on the standardization activities of nursing documentation. Even though nurses are the most important manpower in terms of collecting patients' data, nursing documentation have been overlooked in the process of developing electronic patients records. It is impossible to complete a computerized patient record system without including nursing documentation. Standardization of nursing documentation is the first step toward a computerized documentation system. In this study nursing documentation forms were gathered from 11 tertiary hospital with more than 500 beds in Seoul. Out of various nursing documentation, 9 essential forms were chosen to standardize. They are admission assessment, form, nursing treatment record, nursing care plan, discharge planning record, patient transfer record, clinical observation record, nursing treatment record, nursing progress notes, critical care flow sheet, and preoperative checklist Forms and data elements were reviewed and analyzed. It was learned that there is no one perfect from that could be used in any agency. Data elements were analyzed and standardized. Data elements to be included in each form were selected. Standardized forms were developed with the selected data element. Guideline outlining how to use each nursing form were developed. Now it is in the process of validating the forms and the guidelines at 240 nursing units at 8 tertiary hospitals. The results of the validation study will be incorporated in the final version of nursing forms and they will be introduced to general nursing population at an open forum to be held by Korean Nurses Association at the end of this year. This standardization activities will have a great impact on nursing practice, education, administration and research.
Checklist ; Critical Care ; Education ; Health Records, Personal ; Humans ; Nursing Informatics* ; Nursing Records* ; Nursing* ; Patient Discharge ; Patient Transfer ; Seoul ; Tertiary Care Centers

Ewing sarcoma is an uncommon bone malignancy of childhood. Although Ewing sarcoma is mostly a tumor of bone, it may also arise from soft tissues (extraskeletal Ewing sarcoma). We report a case of extraskeletal Ewing sarcoma that arose in the retroperitoneum of a 18-month-old girl and presented with right leg pain and gait disturbance. A brief review of related literatures was also made.
Female ; Gait ; Humans ; Infant ; Leg ; Sarcoma, Ewing*

BACKGROUND: The arterial baroreflex is a key mechanism for maintaining blood pressure homeostasis. Low-dose atropine (LDA) causes bradycardia, either by acting on the sinoatrial node or due to its effect on central muscarinic receptors, which increases vagal activity. We evaluated the effect of LDA on baroreflex sensitivity (BRS) in healthy awake subjects. METHODS: We assessed changes in RR interval (RRI) and systolic blood pressure (SBP), power spectral densities of heart rate variability (HRV) and systolic blood pressure variability (SBPV), and spontaneous BRS by using transfer function analysis before and after LDA (2microgram/kg) in 17 healthy volunteers. RESULTS: LDA induced not only bradycardia but also increased of the high-frequency (HF) component of HRV, RMSSD (root mean squared successive difference interval), and pNN50 (percentage of sinus cycles differing from the preceding cycle by > 50 ms). The HF and LF components of SBPV remained unchanged. Spontaneous BRS determined by transfer function analysis increased significantly (P < 0.05), and changes in BRS were significantly associated with changes in the HF component of HRV (P < 0.05). CONCLUSIONS: LDA increased vagal cardiac function and arterial baroreflex in awake subjects. This result suggests that increased vagal cardiac function by LDA application is related to baroreflex increase.
Atropine* ; Baroreflex* ; Blood Pressure ; Bradycardia ; Healthy Volunteers ; Heart Rate ; Homeostasis ; Receptors, Muscarinic ; Sinoatrial Node

Although dietary protein restriction may protect against progression of renal failure, it is important to consider whether protein restriction can be attained without inducing malnutrition. We assessed the calculated dietary protein intake(cDPI) by 24 hour urinary collection and food intake, biochemical nutritional indices and the results of anthropometric measurement in 83 predialysis patients with different stages of chronic renal failure(CRF) and 84 controls. Dietary interventions were minimal. We categorized patients into three groups according to whether their creatinine clearance(Ccr) was greater than 25(group A), 10 to 25(group B), or less than 10ml/min(group C). 1) The mean(+/-SD) cDPI was significantly lower in group C(0.77+/-0.17g/kg/day) and group B(0.84+/- 0.16g/kg/day) than in group A(1.04+/-0.21g/kg/day) and controls(1.14+/-0.22g/kg/day)(P<0.05). The mean (+/-SD) high biologic value protein intake was significantly lower in group C(0.29+/-0.25g/kg/day) and group B(0.39+/-0.27g/kg/day) than in group A (0.48+/-0.35g/kg/day)(P<0.05). The cDPI(r=0.50, P< 0.05), high biologic value protein intake(r=0.39, P< 0.05) were positively correlated with the Ccr. 2) The mean (SD) total lymphocyte count (TLC) was significantly lower in group C(1,554+/-368/mm3) and group B(1,972+/-470/mm3) than in group A(2,111+/-540/mm3) and controls(2,177+/-589/mm3)(P<0.05). The TLC was positively correlated with the Ccr(r= 0.28, P<0.05). The levels of albumin and transferrin were lower in patients with CRF than in controls (P<0.05). There was no difference in the levels of albumin, transferrin, prealbumin, insulin-like growth factor-1, cholesterol and anthropometric measurements among the different stages of CRF. CONCLUSION: In predialysis patients with CRF, the dietary protein and high biologic value protein intake spontaneously decreases as renal function declines. Several nutritional indices, such as TLC, albumin and transferrin were lower in predialysis patient with CRF than controls. Therefore objective measurement of DPI should be considered to educate a low protein diet in predialysis patients with CRF.
Cholesterol ; Creatinine ; Diet, Protein-Restricted ; Dietary Proteins* ; Eating ; Humans ; Kidney Failure, Chronic ; Lymphocyte Count ; Malnutrition ; Nutrition Assessment* ; Prealbumin ; Renal Insufficiency ; Renal Insufficiency, Chronic* ; Transferrin

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

PURPOSE: The purposes of this study were to determine short- and long-term fractal correlation behavior of heart rates during daily activity in patients with neurocardiogenic syncope. METHODS: Twenty five patients with histories of neurocardiogenic syncope episodes were included. Their analogue 24h ambulatory Holter electrocardiograms were analyzed. The tape was digitized and the digitized electrocardiograms were partioned into sections of one hour. Then their RR intervals were measured and 20,000 points of RRI were used. To quantify the fractal correlation behavior, we employed the detrended fluctuation analysis, and short-term(n16, alpha2) fractal scaling exponents were calculated. RESULTS: When compared to control, 24-hour average values of alpha1 and all alpha1 values at quarters of each day were significantly higher in patients with syncope. On the contrary, their 24-hour average value of alpha2 and all alpha2 values at quarters of each day were lower in patients with syncope. However, statistical significances were found in 24-hour average value of alpha2 and in alpha2 value at MN-6AM. CONCLUSION: In the syncope patients with neurocardiogenic syncope, short-term fractal scaling exponents of RR interval was significantly high throughout the day. Therefore, their RR intervals were smoother in the short term scale and had a tendency to continue in the same direction of increase or decrease, which may contribute to persistent decrease in heart rate during a syncopal attack.
Child* ; Electrocardiography ; Fractals* ; Heart Rate* ; Heart* ; Humans ; Syncope ; Syncope, Vasovagal*

Transcervical resection of endometrium is under-utilized in the treatment of dysfunctional uterine bleeding, uterine myoma and menorrhagia. The procedure is similar to transurethral resection of prostate in men with a possibility of substantial absorption of irrigation fluid. Absorption of a large volume of fluid can cause excessive intravascular volume, hyponatremia, cerebral edema and death. Severe hyponatremia leading to extrapontine myelinolysis is an extremely rare complication of this procedure. So, We report a case of developed extrapontine myelinolysis after hysteroscopic myomectomy which, however, showed complete recovery.
Absorption ; Brain Edema ; Endometrium ; Female ; Humans ; Hyponatremia ; Leiomyoma ; Male ; Menorrhagia ; Metrorrhagia ; Myelinolysis, Central Pontine ; Transurethral Resection of Prostate

Central venous cannulation via an internal jugular vein has become a common procedure in the management and monitoring of severely ill patients. Although complications such as carotid artery puncture or pneumothorax have been reported, hemothorax is relatively uncommon. We describe a case of hydrohemothorax and subclavian artery laceration which occurred during right internal jugular vein cannulation. A 44-year-old female patient was admitted for laparoscopic adrenalectomy under general anesthesia. For central venous pressure monitoring, central venous cannulation performed. However, dilator overinsertion injured the right subclavian artery. This led to a massive ipsilateral hydrohemothorax requiring thoracotomy for subclavian artery repair. This case suggests that central venous cannulation should be done carefully and improves awareness of the potential for dilator induced injury.
Adrenalectomy ; Adult ; Anesthesia, General ; Carotid Arteries ; Catheterization* ; Central Venous Pressure ; Female ; Hemothorax ; Humans ; Jugular Veins* ; Lacerations* ; Pneumothorax ; Punctures ; Subclavian Artery* ; Thoracotomy

Female phenotype of a 46,XY male may originates from male pseudohermaphroditism due to 17alpha-hydroxylase deficiency. Lack of cortisol increases adrenocorticotropic hormone (ACTH) and mineralocorticoid production, leading to low renin hypertention and hypokalemia. A 41-year-old phenotypic female presented primary amenorrhea and hypertension. In the hormonal profile, the levels of serum estradiol, testosterone, rennin, and cortisol were decreased and ACTH and deoxycorticosterone were increased. Laparoscopic bilateral gonadectomy was performed, and corticosteroid, antihypertensive drugs, and estrogen were administered. We report this case with a brief review of the literatures.
46, XY Disorders of Sex Development* ; Adrenal Hyperplasia, Congenital ; Adrenocorticotropic Hormone ; Adult ; Amenorrhea ; Antihypertensive Agents ; Chymosin ; Desoxycorticosterone ; Estradiol ; Estrogens ; Female ; Humans ; Hydrocortisone ; Hypertension ; Hypokalemia ; Male* ; Phenotype ; Renin ; Testosterone