Lowe et al. have described a disease characterized by organic aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation, this disease is now called as Lowe's syndrome or oculo-cerebro-renal syndrome which manifests growth plates, metabolic acidosis, and characteristic eye changes, including bilateral cataract, glaucoma and nystagmus. It is a congenital hereditary affectation. Most of the patients with this syndrome are male sex. We report a new case of Lowe's syndrome in a 5-year old girl.
Acidosis ; Ammonia ; Cataract ; Child ; Female ; Glaucoma ; Growth Plate ; Humans ; Hydrophthalmos ; Intellectual Disability ; Male

Arthroscopy became a popular techique for the diagnosis and treatment of wrist disorder. But the specific indications for the wrist arthroscopy are not well defined. So we reviewed our experience of 16 wrist arthroscopies of radiocarpal and midcarpal joints. With this review, arthroscopy was of benifit in the diagnosis and treatment of the lesion of the triangular fibrocartilage complex and also in the synovectomy.
Arthroscopy ; Diagnosis ; Humans ; Joints ; Triangular Fibrocartilage ; Wrist

This case was an acquired aplastic anemia patient who required a major operation for excision of a mediastinal mass. The authors previewed that the coagulation abnormalities would be developed due to major operation. Thus we decided to monitoring the coagulation function using the thromboelastography during the perioperative period and checked the complete blood count, concommitantly. The total blood volume lost during operation was 1800 ml, so we gave him a transfusion of 10 U's of platelet concentrate, 10 U's of pheretic platelet rich plasma and 5 U's of whole blood. The thromboelastography was a good guide that helped us to avoid excessive treatment of the coagulation abnormalities. We concluded that the thromboelastograhy was a reliable and effective monitoring system at the intraoperative coagulation management.
Anemia, Aplastic* ; Anesthesia, General ; Blood Cell Count ; Blood Platelets ; Blood Volume ; Humans ; Perioperative Period ; Platelet-Rich Plasma ; Thrombelastography

Gougerost-Blum syndrome is a rare disease and characterized by lichenoid papules in association with purpuric lesions. So far as we know there has been no report of, in literatures, Gougerot-Blum syndrome with leukemia. We experienced a case of typical Gougerrot_Blum syndrome with acute myeloblastic leukemia in a 30 year-old female patient. This patient complained of rashes on whole body for 2 months, and frequent epistaxis and gum bleeding for 20 days. The skin revealed miliary to rice sized rusty colored lichenoid papules on whole body especially on legs and arms. Histopathologically the skin showed the picture of Gougerot-Blum syndrome. On peripheral blood examination and bone marrow aspiration myeloblastic leukemia was confirmed. The patient was treated with 6-mercaptopurine, cytoxan and prednisolone with slight improvement of skin lesions, but no improvement of leukumia itselt.
6-Mercaptopurine ; Adult ; Arm ; Bone Marrow ; Cyclophosphamide ; Epistaxis ; Exanthema ; Female ; Gingiva ; Granulocyte Precursor Cells ; Hemorrhage ; Humans ; Leg ; Leukemia* ; Leukemia, Myeloid, Acute ; Prednisolone ; Rare Diseases ; Skin

No abstract available.
Arthroscopy* ; Elbow*

In the treatment of flexor tendon injury of the hand, re-rupture after primary tendon repair is one of complications which occurs occasionally and so impose burden on both the patient and the surgeon. Authors experienced twelve cases of re-rupture after primary flexor tendon repair of 274 patients from Mar. 1989 to Mar.1996. The incidence of re-rupture after primary flexor tendon repair was 4.4% in author's series. One case happened with slip down injury, and in six cases re-rupture occurred during physical therapy with snapping click sound. However in five cases, the patients conldnt recognize any related causes. In majority of cases, re-ruptures were identified between three and five weeks after primary repair by the surgeon and the patient, so it could be suggested that the attention should be paid for the high possiblity of re-rupture during this period. Operative findings were the resorption and friability of repaired end with insecure suture fixation in two cases, rupture of suture material in four cases and loosening of the knot in six cases. From this study, the authors suggest the importance of knot, and recommend to make more than four knots on suture tie with attention to the tie direction, and advise careful physical therapy according to each patients' different situation. In the treatment of re-rupture, end-to-end re-anastomosis was available in seven cases (59%). In five cases (41%), tendon graft was needed. The clinical result of the re-rupture cases was evaluated by the Stickland evaluation method, and it was satisfactory in 67% of the patients who had the complication of re-rupture.
Hand* ; Humans ; Incidence ; Rupture ; Sutures ; Tendon Injuries ; Tendons* ; Transplants

Transient osteoporosis of the hip is a self-limited condition of uncertain etiology and pathogenesis that affects young and middle aged adults. Even its clinical features are similar to the features of osteonecrosis the prognosis and clinical end result are quite different. So differential diagnosis between the two is very important. We present a case of transient osteoporosis of the hip in middle aged man, which was completely reeovered after conservative treatment.
Adult ; Diagnosis, Differential ; Hip ; Humans ; Middle Aged ; Osteonecrosis ; Osteoporosis ; Prognosis

PURPOSE: Nowadays pharmacologic provocation tests and physiologic tests are usually used to determine growth hormone(GH) deficiency in short stature. But this method has many problems. We know GH stimulates the release and synthesis of insulin-like growth factor-I(IGF-I) and measuring the level of IGF-I is relatively simple. So we measured plasma IGF-I to watch the correlation with the GH levels and to determine it may replace the complicated stimulation tests. METHODS: At the department of Pediatrics in Dong San Hospital from Dec. 1996 to Aug. 1998, childrens who visited for evaluation of short stature and measured GH and IGF-I simultaneously were reviewed. After clonidine and insulin administration, exercise and sleep, we measured their peak GH level and IGF-I level by the immunoradiometric assay(IRMA) kit. RESULTS: The ratio of boys and girls were 22 to 31 and the cases below 3 rd percentile were 26 which was the most. With phamacologic provocation test, there were 43 cases whose level of peak GH below 7 ng/mL(group I) but with combined phamacologic provocation and physiologic tests there were 27 cases of group I. The mean IGF-I level showed correlation with aging in both male and female(r=0.53, P<0.05). The relationship between peak GH and IGF-I level are found when we tested both phamacologic and physiologic combined tests. The mean IGF-I level did not correlate with height percentile. CONCLUSION: Measuring the IGF-I value was useful to detect GH deficient children but combined tests were more helpful.
Aging ; Child* ; Clonidine ; Female ; Growth Hormone ; Humans ; Insulin ; Insulin-Like Growth Factor I* ; Male ; Pediatrics ; Plasma

Phototherapy with UVB has become a cornerstone in treating uremic pruritus. To investigate the additional benefit of emollient, we performed emollien-phototherapy in six uremic pruritus patients on hemodialysis, one of which was not responding with UVB only. After applying mineral oil over the whole body, UVB phototh.rapy was performed two to three times weekly. Improvement was noted within one to three treatments and after three to ten treatments, pruritus markedly or totally disappeared in al six patients. This result suggests that emollient-phototherapy is as effective as, or in some patients, more effective than phototherapy with UVB only in managing uemic pruritus.
Humans ; Mineral Oil ; Phototherapy ; Pruritus* ; Renal Dialysis

We evaluated median value and lower confidence limit of total serum IgE level in each age group of 1,493 pure atopic dermatitis patient without respiratory atopic diseases. The results were as follows . 1. The median value of total serum IgE level of each age group in patients with atopic dermatitis distributed from 56.5 IU/@Ll to 660It.J/C. The distributions of the median value of total serum IgE levels of total patients with atopic dermatitis, total male patients with atopic dermatitis, and total female patients with atopic dermatitis were 185 IU/C, 220 IUj'@C, and 165 IUj'C. There was no statistical significance of median value of total serum IgE level between mole patients and female patients. 2. The 95% and 99% lower confidence limit(one-sided) of total serum IgE level of ea.ch age group in patients with atopic dermatitis distributed from 32.6 IU /n4 to 287IU/v4 and from 31 IU/m0 to 202 IU4. The 95% lower confidence limit (one-sided) of total serum IgE level of total patients with atopic dermatitis, tatal male patients with atopic dermatitis, and. total female patients with atopic dermatitis were 170 II.J/m0, 185 IU,m/l, and 133 IUm/l. The 99% lower confidence limit.(one-sided) of total serum IgE level of total patient with atopic dermatitis, total male patient with atopic dermatitis, and total female patients with atopic dermatitis were 165 IU/ml, 180 IU/ml, and 125 IU/ml.
Dermatitis, Atopic* ; Female ; Humans ; Immunoglobulin E* ; Male