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

No abstract available.
Craniopharyngioma* ; Magnetic Resonance Imaging*

No abstract available.
Family Practice* ; Humans

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

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

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

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

Interhemispheric approach has been accepted as a standard method approaching distal anterioir cerebral artery(DACA) aneurysm. In the approach, most authors use the surgical method retracting the superior sagittal sinus(SSS) after dural reflection. However, it results in limited surgical space and makes the surgical procedure difficult technically. To overcome the limitation, we tried to ligate and cut SSS in 13 patients out of 23 patients of DACA aneurysm from Mar. 1983 to Feb. 1993. Out of total 583 intracranial aneurysms operated on in that period, the proportion of DACA aneurysm was 3.95%. Mean age was 52.7 years ranging from 32 to 66 years. The sex ration was 7:16, showing female predominancy. As presenting symptoms, twenty two patients had subrachnoid hemorrhage and one patient had a third nerve palsy due to an unruptured posterior communicating artery aneurysm. On admission, 4 patients(17.4%) were in grade I, 10(43.5%) in grade II, 6(26.1%) in grade III, 3(13%) in grade IV according to the Hunt and Hess grading system. In 18 patients(78.3%), aneurysms were located at bifurcation of pericallosal and callosomarginal artery and in 5(21.7%) at bifurcation of frontopolar and pericallosal artery. Associated vascular anomalies were A1 hypoplasia(2 cases), multiple aneurysms(5 cases), fenestration of anterior cerebral(1 case) and arteriovenous malformation(1 case). Thirteen cases were operated on with ligation of the SSS and 10 without ligation. Overall surgical outcomes scored by Glasgow outcome scale were : 11 patients(47.8%) in Grade I, 6(26.1%) in Grade II, 2(8.7%) in Grade III, 4(17.4%) in Grade V. The outcomes in ligation group were : 8 patients(61.5%) in Grade I, 4(30.8%) in Grade II, 1(7.7%) in Grade III, and no death. The outcomes in non-ligation group were : 4 patients(40%) in Grade I, 1(10%) in Grade II, 1(10%) in Grade III, and 4(40%) in Grade V. These results showed that the patients operated on with ligation of SSS had more favorable outcomes than the patients without ligation. It seems that the SSS in the interhemispheric approach might provide several advantages and better operative outcome over the conventional method.
Aneurysm ; Anterior Cerebral Artery* ; Arteries ; Female ; Glasgow Outcome Scale ; Hemorrhage ; Humans ; Intracranial Aneurysm* ; Ligation* ; Oculomotor Nerve Diseases ; Superior Sagittal Sinus