No abstract available.
Family Practice* ; Humans

No abstract available.
Bile Duct Neoplasms* ; Bile Ducts* ; Bile*

No abstract available.
Craniopharyngioma* ; Magnetic Resonance Imaging*

Berardinelli lipodystrophy syndrome is a rare autosomal recessive disorder, characterized by loss of body fat, muscular hypertrophy, acanthosis nigricans, hepatomegaly, hyperlipidemia, insulin resistant diabetes, and elevated metabolic rate. The mechanism(s) responsible for these abnormalities is not known. We report a forteen-month old girl with Berardinelli Lipodystriphy Syndrome, who had signs above mentioned, with a brief review and its related literatures.
Acanthosis Nigricans ; Adipose Tissue ; Female ; Hepatomegaly ; Humans ; Hyperlipidemias ; Hypertrophy ; Insulin ; Lipodystrophy*

BACKGROUND: There have recently been some reports suggesting that once-daily intravenous busulfan (IV Bu) as a conditioning regime for hematopoietic stem cell transplantation (HSCT) possibly reduces the toxicities without influencing the clinical outcome as compared with the traditional 4 times daily dosage schedule. We report here on the clinical outcome of once-daily IV Bu as a conditioning regime for HSCT in children with AML at a single treatment center. METHODS: We retrospectively analyzed nine AML children who received HSCT with using the once-daily IV Bu (110~130 mg/m2 on 4 consecutive days) conditioning regimen at the Department of Pediatrics, Pusan National University Hospital from 2003 to 2007. RESULTS: The median age at HSCT was 8.25 years. As for the conditioning regimens, the HLA-matched sibling peripheral HSCT (N=4) was Flu/Bu, the CBT and unrelated BMT (N=4) was Flu/Bu/ATG and the autologous HSCT (N=1) was Bu/Cy. There was only one case of primary graft failure in an unrelated donor CBT recipient. The median time to neutrophil engraftment was 14 days and the median time to platelet engraftment was 19 days. The transplant-related toxicities were acceptable; there were no case with CNS toxicity and VOD was observed in two cases (1 mild case of VOD and 1 moderate case of VOD). Acute GVHD was noted in two cases (1 case of grade I and 1 case of IV). With a median follow up of 33 months, there were two cases of relapse and two cases of death. CONCLUSION: Once-daily IV Bu as a conditioning regimen for HSCT in children with AML was well tolerated and convenient with relatively moderate toxicities, but additional studies are needed to determine the therapeutic efficacy and pharmacokinetics of once-daily IV Bu in children who are undergoing HSCT.
Appointments and Schedules ; Blood Platelets ; Busulfan ; Child ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Leukemia, Myeloid, Acute ; Neutrophils ; Pediatrics ; Recurrence ; Retrospective Studies ; Siblings ; Transplants ; Unrelated Donors

PURPOSE: To construct new Korean reference curves for birth weight by sex and gestational age using contemporary Korean birth weight data and to compare them with the Lubchenco and the 2010 United States (US) intrauterine growth curves. METHODS: Data of 2,336,727 newborns by the Korean Statistical Information Service (2008-2012) were used. Smoothed percentile curves were created by the Lambda Mu Sigma method using subsample of singleton. The new Korean reference curves were compared with the Lubchenco and the 2010 US intrauterine growth curves. RESULTS: Reference of the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th percentiles birth weight by gestational age were made using 2,249,804 (male, 1,159,070) singleton newborns with gestational age 23-43 weeks. Separate birth weight curves were constructed for male and female. The Korean reference curves are similar to the 2010 US intrauterine growth curves. However, the cutoff values for small for gestational age (<10th percentile) of the new Korean curves differed from those of the Lubchenco curves for each gestational age. The Lubchenco curves underestimated the percentage of infants who were born small for gestational age. CONCLUSION: The new Korean reference curves for birth weight show a different pattern from the Lubchenco curves, which were made from white neonates more than 60 years ago. Further research on short-term and long-term health outcomes of small for gestational age babies based on the new Korean reference data is needed.
Asian Continental Ancestry Group ; Birth Weight* ; Female ; Gestational Age* ; Growth Charts ; Humans ; Infant ; Infant, Newborn ; Information Services* ; Male ; United States

OBJECTIVE: The purpose of this study is to determine the efficacy of anterior lumbar interbody fusion(ALIF) for the patients with focal type of degenerative flat back. METHODS: The authors reviewed clinical and radiologic data of 16 patients with focal type of degenerative flat back who underwent ALIF during the period between December 1999 and November 2000. The operation procedure was ALIF using titanium or carbon fiber cages of lordotic angle, which were filled with allograft or autograft mixed with allograft through minilaparotomy. RESULTS: The majority of the patients were female(15 female: 1 male) and the mean age was 60.1 years. Six patients were operated on one level, ten on two levels. The mean follow up period was 10.7 months. The mean operation time was 131minutes and the average estimated blood loss was 133ml. Transfusion was not needed in any case. The mean preoperative lumbar lordotic angle was 4.1 degree(-7.4-41.6) and improved to 17.5 degree(-4.5-41.9) postoperatively. Radiological fusion was achieved in 13 patients(81%). The Oswestry disability index score improved from 62.5% to 23.9% and the patient's subjective improvement rate was 80%. Complications were infection in one patient, incisional hernia in one, symptomatic pseudoarthrosis in one and transient sympathetic disturbance in left leg in three. CONCLUSION: In a subset of the patients with degenerative flat back who showed disc degeneration in one or two levels, ALIF using cages improved patient's symptoms effectively. It seems that ALIF through minilaparotomy can be one of the surgical options in the treatment of the focal type of degenerative flat back.
Allografts ; Autografts ; Carbon ; Female ; Follow-Up Studies ; Hernia ; Humans ; Intervertebral Disc Degeneration ; Laparotomy ; Leg ; Pseudarthrosis ; Titanium

Authors reviewed 37 cases of 25 patients with carpal tunnel syndrome treated surgically at Department of Neurosurgery of Kyung Hee University Hospital from January 1981 to February 1992. The sex ratio was 1:24 showing female predominancy. Distribution of age ranged from 26 years to 65 years. Duration of symptoms ranged from 15 days to 20 years. Tinel's sign was positive in 24 patients ans 10 patients had muscle atrophy. Twelve patients were operated on both hands, 7 patients on the right and 6 patients on the left. Operations were performed under the general anesthesia in 4 patients, under the axillary block in 3 patients and under the local lidocaine infiltration in 18 patients. In postoperative periods, 5 patients experienced transient aggravation of numbness of fingers, 1 patient had wound problem, 1 patient hypalgesia and 1 patient transient motor weakness which was thought to be due to residual effect of axillary block. Surgical outcomes were good in 35 cases(94.6%), fair in 1 case(2.7%) and poor in 1 case(2.7%).
Anesthesia, General ; Carpal Tunnel Syndrome* ; Electromyography ; Female ; Fingers ; Hand ; Humans ; Hypesthesia ; Lidocaine ; Median Nerve ; Muscular Atrophy ; Neurosurgery ; Postoperative Period ; Sex Ratio ; Wounds and Injuries

The effect of Pheniramine(Avil), a histaminergic-1 receptor blocking agent presently employed in treating various allergic diseases on pressor actions of norepinephring(NE) and tyramine (TR) was studied in the rabbit. Pheniramine, when given into a femoral vein with a dose(3mg/kg) enough to block H1-receptor, potentiated markedly the pressor responses of NE and TR. The pressor action of NE augmented by pheniramine was not affected by additional adminstration of debrisoquin (Drenergic neuron blocker) or phenelzine(monoamine oxidase inhibitor) or desipramine(U1-uptake blocker), or while potentiated by additional treatment with chlorisondamine(ganglionic blocker)or reserpine(catecholamine depleter). The hypertensive response of NE to phenelzine or desipramine was reinforced significantly by addition of pheniramine, but the response of NE in rabbits treated with reserpine or chlorisondamine or debrisoquin was not influenced by pheniramine-addition. Elevation of blood pressure to TR potentiated by pheniramine was attenuated significantly by reserpine treatment with chlorisondamine made the significant augmentation of pressor action to TR after pheniramine. Tyramine-induced response of blood pressure after pheniramine, but the response of blood pressure to TR caused by phenelzine or desipramine was enhanced markedly by pheniramine-treatment. From the above experimental results, it is thought that the pressor effect of NE and TR potentiated by pheniramine is similar to that of debrisoquin, i.e. the sensitization of effector cell, and that central action of pheniramine can not ruled out.
Blood Pressure ; Chlorisondamine ; Debrisoquin ; Desipramine ; Femoral Vein ; Neurons ; Norepinephrine* ; Oxidoreductases ; Phenelzine ; Pheniramine ; Rabbits ; Reserpine ; Tyramine*

Autoimmune diseases are occasionally associated with other autoimmune diseases in the same patients. Graves disease has been associated with systemic rheumatic diseases including systemic lupus erythematosus (SLE). The diagnosis of Graves disease in patients with SLE is well known, especially in adults, but few case reports have involved children and adolescents. We report a 14-year-old female who presented with Graves disease, proteinuria, arthralgia, decreased complement, and positive antinuclear antibody and who was diagnosed with SLE. Renal biopsy revealed class IV lupus glomerulonephritis. These clinical, laboratory, and histological findings indicate that class IV lupus nephritis may be associated with Graves disease. This possibility should be borne in mind when following patients with Graves disease in order to avoid any delay in the diagnosis and treatment of serious systemic autoimmune diseases such as SLE.
Adolescent ; Adult ; Antibodies, Antinuclear ; Arthralgia ; Autoimmune Diseases ; Biopsy ; Child ; Complement System Proteins ; Female ; Graves Disease ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Proteinuria ; Rheumatic Diseases