We report a case of Hunter' s syndrome with characteristic nodules on the upper back. The patient was a 7-year-old Korean boy who presented with ivory-colored papules and nodules on both sides of the scapula, pectoral regions and lateral aspects of the upper arms. These lesione are regarded as pathognomonic cutaneous markers for Hunter s syndrome. He also presented with truncal hypertrichosis, retarded growth, short neck, round face, claw like contractures of hands, multiple joint contractures, and a clear cornea. Severely elevated glycosaminoglycan levels were present in the patient s urine samples. The patient s 5-year-old brother had similar clinical features.
Animals ; Arm ; Child ; Child, Preschool ; Contracture ; Cornea ; Hand ; Hoof and Claw ; Humans ; Hypertrichosis ; Joints ; Male ; Mucopolysaccharidosis II* ; Neck ; Scapula ; Siblings

A 4-year-old girl developed acute motor, sensory impairment, lower abdominal pain and urinary dysfunction after URI. In acute stage of disease, MRI showed diffuse spinal cord swelling and high signal intensity on T2WI below T2 body level. The patient did not improved and one month later, follow-up MRI showed diffuse spinal cord atrophy and syrinx formation as sequelae of ATM on T1WI below T9 level. To our knowledge, this is the first case showing the sequelae of acute transverse myelitis seen in Korea.
Abdominal Pain ; Atrophy* ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Korea ; Magnetic Resonance Imaging ; Myelitis, Transverse* ; Spinal Cord*

No abstract available.
Angiofibroma* ; Carbon Dioxide* ; Carbon* ; Lasers, Gas* ; Sirolimus* ; Tuberous Sclerosis*

No abstract available.
Aorta, Thoracic* ; Rupture*

No abstract available.
Diabetes Mellitus* ; Hearing* ; Humans

PURPOSE: We performed this study to evaluate the changes of total leukocytes and leukocyte types in CSF and peripheral blood (PB) in the early course of aseptic meningitis. METHODS: One hundred and eighty-nine children with aseptic meningitis, who were admitted to the Pediatric Department of Dong-A University Hospital during the period from June 1996 to October 1997 were included. Patients were divided into 4 groups by 12-hour intervals according to the time between the onset of illness and initial diagnostic lumbar puncture. We analyzed clinical pictures, total leukocytes and leukocyte types in CSF and peripheral blood (PB) in each group. RESULTS: 1) There was no significant difference in average total leukocyte counts in CSF between each group, and all groups were considered to be acute stage of illness. 2) The PMNL proportion of CSF leukocytes was 57.0+/-31.6% in group I, 44.1+/-32.3% in group II, 39.4+/-33.1% in group III and 26.9+/-27.9% in group IV. The PMNL percentage was significantly higher in group I than group III and IV and the proportion of patients with a predominance of PMNL was higher in group I than group III and IV (p<0.05) 3) The proportion of neutrophils in PB was highest in group I (76.5+/-15.7%) and significantly higher in group I and II than group III and IV (p<0.05). 4) Significant correlations were found between the proportion of PMNL in CSF and PB of patients (r=0.62, p<0.001) CONCLUSION: The change from a predominance of PMNL to a predominance of mononucler leukocytes was occurred 12-24 hours after onset, and there was a strong correlation between the proportion of neutrophils in CSF and PB.
Cerebrospinal Fluid* ; Child ; Humans ; Leukocyte Count ; Leukocytes* ; Meningitis, Aseptic* ; Neutrophils ; Spinal Puncture

Combid is a compound of Compaxine, which is a phenothiazine derivative, and Darbid that is a long acting anticholinergic synthetic. This medicant was originally intended for use in physical and emotional peptic ulcer cases. Because of it's pharmacological action, writers decided to consider the medicant as a preanesthetic medication. From a record of 2800 patients in the hospital for the year 1975 was administered as a premedicant at random, even though some contraindication did occur and is discussed later in this article, the following results were observed. 1. Because of the characteristics of a long acting effect of Combid Spansule, it can be administered orally with sips of water, 2~3 hours prior to operation, eliminating needless preanesthetic shots, without affecting the N.P.O. rule. 2. Sedative and antisialogue effects of the drug have been also satisfactory, therefore it is recommended to use as a premedicant especially in Korea because ether is commonly used. 3. The drug has an additional antiemetic effect to prevent vomiting from ether anesthesia. 4. Some side effects were observed such as dry mouth, tachycardia, fever and extrapyramidal action that occur from overdosage. 5. The contraindications to be considered when using Combid Spansule as a premedicant include dehydration, fever, tachycardia and parkinsonism. 6. Please note the writers do not recommend this application for short procedure such as closed reduction of Colles fracture and simple spinal anesthesia due to the long lasting drying effect.
Anesthesia ; Anesthesia, Spinal ; Antiemetics ; Colles' Fracture ; Dehydration ; Ether ; Fever ; Humans ; Korea ; Mouth ; Parkinsonian Disorders ; Peptic Ulcer ; Preanesthetic Medication ; Tachycardia ; Trimeprazine* ; Vomiting ; Water

Many cases have been reported indicating that postspinal headache can be relieved immediately by an epiduralinjection of saline: also autologous blood has recently been used successfully instead of saline. C.S.F. changes in 20 cases were observed in the support of the concept; that a continuous leakage in association with hypovolemia and hypotension of C.S.F is the primary cause of postspinal headache. Subarachnoid pressure increased immediately with Xylocaine injection into lumbar epidural space. A spinal needle was inserted into the subarachnoid space at the level Ll-L2 and opening pressure of C.S.F. was read, epidural Tuohy needle was inserted at the 4~5. Twenty-five ml of Xylocaine was injected into epidural space and C.S.F. pressure changes were observed. This procedure was performed on 10 patients in lateral position and another 10 patients were tested in the sitting position. Sitting position: Condition Highest pressure change Lowest pressure change Average pressure change Average opening pressure Horizontal position: Highest pressure change Lowest pressure change Average pressure' change Average opening pressure .Pressure rise 350 mm H2O 60 mm HO 191 mm H2O 369 mm H2O .290 mm H2O 40 mm H2O 14Z mm H2O 165 mm H2O .These pressure changes responded almost simultaneously as xylocaine was injected. The immediate relief of postspinal headache by injecting fluid into epidural space is simultaneous with the increase of C.S.F. pressure. In summary, the direct cause of postspinal headache is probably hypotension of C.S.F. pressure resulting from continuous leakage from spinal tap.
Epidural Space* ; Headache ; Humans ; Hypotension ; Hypovolemia ; Intracranial Pressure ; Lidocaine* ; Needles ; Spinal Puncture ; Subarachnoid Space

No abstract available.
Neurofibromatoses* ; Neurofibromatosis 1* ; Neurofibromin 1 ; Vitiligo*

No abstract available.
Onycholysis*