KCNJ11 gene mutation was searched in 3 families with neonatal diabetes. A KCNJ11 175 G>A (V59M) mutation was found in one child, while no KCNJ11 gene mutation was found in his parents. No mutation was found in the other two families. The result indicated that KCNJ11 gene mutation might lead to the onset of neonatal diabetes mellitus in Chinese.

Deep skin and soft tissue defects with exposed bone and tendon is difficult to treat, because skin graft rarely survives and flap surgery is sacrifice of donor site. Since "Stage I" membrane was developed by Yannas and Bruke in 1980, numerous kinds of artificial skin have been developed. The adaptability of "Terudermis", developed by the Terumo Co., as an artificial skin composed of sponge made of a fibrillar atelocollagen and a heat-denatured atelocollagen, was clinically evaluated on application to 13 cases presenting deep skin and soft tissue defect with exposed bones and tendons from October 1997 to march 1998. Terudermis has the advantage of allowing early incorporation of fibroblasts and capillaries into its collagen sponge due to very weak dehydrothermal cross-linking. Before Terudermis graft, several days of wet dressing and debridement were required to prepare healthy well-vascularized bed because Terudermis was weak on unsanitary wounds. After bed preparation, Terudermis was grafted like usual skin graft. Tie-over bolster dressing or compressive dressing was used case by case. The dressing was opened 2~3 days after Terudermis grafting. Wet dressing was done daily until the skin graft was done. Autologous skin graft was done 2-3 weeks after Terudermis graft. Our clinical results indicated that Terudermis was beneficial in treating 77% of our patients. Through the use of this new method, treatment of severe skin and soft tissue defects that are usually treated by musculocutaneous or other conventional skin flaps can be replaced by Terudermis as an new artificial dermis.
Bandages ; Capillaries ; Collagen ; Debridement ; Dermis ; Fibroblasts ; Humans ; Membranes ; Porifera ; Skin ; Skin, Artificial ; Tendons ; Tissue Donors ; Transplants ; Wounds and Injuries*

As being obesity is associated with numerous health problems, effective fat loss strategies are required. Although dieting has been the major fat loss method, aerobic exercise programs have been shown to increase cardiorespiratory fitness and preserve fat-free mass. Most aerobic exercise interventions have consisted of moderate-intensity steady-state exercise. Disappointingly, these kinds of exercise programs have resulted in minimal fat loss. In contrast, high-intensity intermittent exercise (HIIE) has been shown to result in greater fat loss. Accumulating evidence suggests that high intensity intermittent exercise (HIIE) has the potential to be an economical and effective exercise protocol for reducing fat of overweight individuals, especially Tabata workout and Crossfit.
Diet ; Exercise ; Methods ; Obesity* ; Overweight

OBJECTIVE: To evaluate the efficacy of low dose gonadotropin releasing hormone agonist(GnRH-a) therapy combined with high dose human menopausal gonadotrpin(hMG) following estrogen & progesteron therapy for poor responders. METHODS: From May 1997 to Feb 1999, 36 patients who were defined as poor responders on previous consecutive two and more superovulation cycles were randomly allocated to lowdose GnRH-a short protocol with high dose hMG protocol pretreated with estrogen & progesterone(E/P therapy)(n=16)(study group) and the clomiphene citrate with hMG(n=20)(control group). All patients were planned to undergone in-vitro- fertilization(IVF) and embryo transfer(ET) after controlled ovarian hyperstimulation(COH). RESULTS: Two groups were similar with respect to clinical features and basal FSH and E2 levels. The mean level of E2 on day 5, 304.3+/-148.ng/ml in study group was significantly higher than that in control group, 182+/-34.9ng/ml. The mean levels of E2 on hCG day was also significantly higher in study group than control group(1324+/-320ng/ml, vs 414+/-168ng/ml). The mean day of hCG day in study group, 12.3+/-0.3 was shorter than that in control group, 13.8+/-0.4. The concellation rates of cycles were significantly lower in study group than control group(13.2% vs 84.2%). But clinical pregnancy rates did not showed the significant difference between two groups. CONCLUSION: The study suggested that a lowdose GnRH-a short protocol with high dose hMG pretreated with estrogen & progesterone can improve the ovarian response in poor responder group.
Clomiphene ; Embryonic Structures ; Estrogens ; Gonadotropin-Releasing Hormone* ; Humans ; Pregnancy Rate ; Progesterone ; Superovulation

The thermostability of the inulinase was studied in this resea rc h. Some alcoholic materials and thickening agent could enhance the thermostabli lity of the inulinase. Using glycerol、xanthic pastern and though orthogonal ex periments of three elements and three levels, a satisfying protective agent, whi ch included glycerin(6%), xanthan gum(0.6%) and CaCl_2 (100mmol/mL) and ha d a significant effect on the enhancement of the inulinase thermostability, was acquired.

Ameloblastic fibro-odontoma(AFO) is a rare mixed odontogenic tumor. It is composed of connective tissue characteristic of an ameloblastic fibroma and calcified tissue as a complex or compound odontoma. AFO usually presents itself as an asymptomatic swelling of jaw or failure of tooth eruption. The lesion usually occurs in individual less than 30 years old. The differential diagnosis of this tumor includes odontoma, ameloblastoma, and ameloblastic fibroma. This report describes an ameloblastic fibro-odontoma occurring in maxilla of sixteen-year-old female. The lesion was treated by surgical enucleation and curettage without extraction of the involved canine(#23). This patient has shown no sign of recurrence during postoperative 34 months. So we report our case with review of literatures.
Adult ; Ameloblastoma ; Ameloblasts* ; Connective Tissue ; Curettage ; Diagnosis, Differential ; Female ; Fibroma ; Humans ; Jaw ; Maxilla* ; Odontogenic Tumors ; Odontoma ; Recurrence ; Tooth Eruption

A 71-year-old woman presented with an enlarging mass in the right buttock, with pain and tingling sensation in sitting position. Five years ago, she was diagnosed with acute limb ischemia due to acute thrombosis of right persistent sciatic artery (PSA), and she underwent successful thromboembolectomy and femoro-tibioperoneal trunk bypass. Computed tomography angiography revealed a huge PSA aneurysm (PSAA). During the previous bypass, the distal popliteal artery was ligated just above the distal anastomosis to exclude the PSAA, whose proximal end was already thrombosed. However, PSAA has grown to cause compression symptoms, and the mechanism of aneurysm growth can be ascribed to type 1a or type 2 endoleak. In order to relieve the compression symptoms, aneurysm excision was performed without any injury to the sciatic nerve. A postoperative tingling sensation due to sciatic-nerve stimulation in the supine position resolved spontaneously one month after surgery.
Aged ; Aneurysm* ; Angiography ; Arteries* ; Buttocks ; Congenital Abnormalities ; Endoleak ; Extremities ; Female ; Humans ; Ischemia ; Popliteal Artery ; Sciatic Nerve ; Sciatica ; Sensation ; Supine Position ; Thrombosis

Wilson's disease is an autosomal recessive disorder resulting from an excessive accumulation of copper in the liver, cornea, kidneys, and in the basal ganglia of the brain. The prominent speech disturbances of Wilson's disease include monopitch, monoloudness, slow rate, low pitch, delayed in initiating speech and rarely palilalia. A19-year-old woman developed palilalia which was characterized by compulsive repetition of a phrase with increasing rapidity and with a decrescendo of voice volume. Although she had been suffering from liver cirrhosis for the past 8 months, the palilalia was the only neurological sign at initial examination. She showed a low serum ceruloplasmin, low serum copper, increased urinary copper excretion, and Kayser-Fleischer rings. Brain magnetic resonance images showed high signals in the bilateral basal ganglia in T2-weighted images, and slight cortical atrophy.
Atrophy ; Basal Ganglia ; Brain ; Ceruloplasmin ; Copper ; Cornea ; Female ; Hepatolenticular Degeneration* ; Humans ; Kidney ; Liver ; Liver Cirrhosis ; Voice

STUDY DESIGN: This is a retrospective study evaluating the efficacy of new surgical technique for treatment of fixed lumbosacral kyphosis. OBJECTIVES: To report a new method of vertebral column resection and to determine its efficacy in the treatment of fixed lumbosacral kyphosis. SUMMARY OF BACKGROUND DATA: The treatment of fixed severe spinal deformity is very difficult and only a few surgical methods are reported. The vertebral column resection from anterior and posterior is a radical method to treat the severe deformity but it has many problems with anterior and posterior approachs. MATERIALS AND METHODS: A new surgical method of vertebral column resection from posterior approach only(PVCR) was carried out in 5 patients of fixed lumbosacral Tb kyphosis from October 1997 to March 1998. The mean age was 43.4 years(range 35-61 years), and four were female and one male. The average postoperative follow-up period was 5.5 months(ranged from 2 months to 12 months). An average of 2.6 vertebrae was resected. The degree of sagittal curves was measured using the Cobb technique preoperatively and postoperatively. the rejional lumbosacral kyphotic angle and compensatory thoracic curvature from 74 to 712. The sagittal imbalance was measured by distance from C7 plumb line to postero-superior corner of 51 using 14 x 36 inch standing lateral radiograph. RESULTS: The sagittal deformities were corrected from an average of kyphosis 37.4 to lodosis 8.4 at lumbosacral level and from an average of lodosis 31.6 to lodosis 8.2 at thoracic level. The sagittal imbalance was improved from an average of +11.7cm to +2.2cm, for a posterior immigration of 9.5cm. The mean operating time was 334 minutes and with an average blood loss of 4338m1. Complication comprised of motor weakness with deep wound infection in one case and superficial wound infection in another case. The transient hip flexor weakness was seen in all the patients. The hip flexor power recovered in 3-4 weeks. The patient of motor weakness and deep infection showed partial motor improvement and complete wound healing at last 2 month follow-up. CONCLUSION: one-stage posterior vertebral column resection is a promising technique for treatment of the severe fixed lumbosacral kyphosis.
Congenital Abnormalities ; Emigration and Immigration ; Female ; Follow-Up Studies ; Hip ; Humans ; Kyphosis* ; Male ; Retrospective Studies ; Spine* ; Wound Healing ; Wound Infection

BACKGROUND & OBJECTIVES: Hypertensive encephalopathy is an acute neurologic syndrome characterized by abrupt and marked elevation of blood pressure, headache, vomiting, seizure, visual disturbance, and altered mental status. This syndrome may occur as a complication of toxemia, renal artery stenosis and acute glomerulonephritis. We report 4 young patients with occipital lobe seizure, as a presenting sign of hypertensive encephatopathy, whose brain MRI and perfusion scans showed lesions on bilateral occipital lobes. Case : Four young patients experienced moderate to severe headache, visual illusion, generalized seizure, and loss of sight for a few days. Their systolic blood pressure was 150-170mmHg and diastolic blood pressure 100-120mmHg. Three patients had proteinuria and hematuria as the results of acute glomerulonephritis. CSF findings were within normal limits. EEG showed intermittent generali,ed or bilateral temporoparietooccipital slowings in all patients and continuous parietooccipital slowings in one patient. Brain MRI man showed hyperintensity signal in T2WI and hypointensity signal in TlWl on bilateral occipital lobes and SPECT scan showed normal perfusion three patients and increased perfusion on occipital areas in one patient. They were treated with antihypertemsive agents and anticonvulsants (phenobarbital or valporic acid) for 2-10 months. Seizure was well controlled and didn't recur though discontinuing anticonvulsant. CONCLUSION: In young age, acute hypertension may cause clinically occipital lobe seizure and radiologically bilateral occipital lobe lesions.
Adolescent* ; Anticonvulsants ; Blood Pressure ; Brain ; Electroencephalography ; Glomerulonephritis ; Headache ; Hematuria ; Humans ; Hypertension ; Hypertensive Encephalopathy* ; Illusions ; Magnetic Resonance Imaging ; Occipital Lobe* ; Perfusion ; Proteinuria ; Renal Artery Obstruction ; Seizures* ; Tomography, Emission-Computed, Single-Photon ; Toxemia ; Vomiting