No abstract available.
Family Practice* ; Humans

No abstract available.
Craniopharyngioma* ; Magnetic Resonance Imaging*

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

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

PURPOSE:In idiopathic central precocious puberty(CPP), characterized by premature but otherwise normal puberty, the early onset of the pubertal growth spurt with increased height velocity results in premature epiphyseal closure with reduced final height. We examined the growth and growth-determining factors in female patients with CPP treated with a long-acting luteinizing hormone-releasing hormone analogue(Tryptorelin). METHODS:Ten female patients who were diagnosed as idiopathic precocious puberty were treated with Tryptorelin(0.06mg/kg, IM every 4 weeks) for 2 years. We evaluated the patients into two groups(Group A, 4 cases, predicted adult height before treatment were less than 150cm; Group B, 6 cases, predicted adult height before treatment were 150cm or greater), and analysed the growth and its determing factors. RESULTS:In total patients, the growth velocity during the second year were decreased to 4.1+/-1.9 from 5.7+/-2.2/yr during the first year(p>0.05) and no significant difference was found in predicted adult height(PAH) before and after 2 years of treatment(152.3+/-6.7 vs. 1453.9+/-6.8cm). The difference between the PAH before and after 2 years of treatment was not correlated with age, bone age, PAH, height standard deviation score(Ht SDS) before treatment, but correlated with difference between the PAH before and after 1 year of treatment(r=0.89310, P=0.0005). The mean Ht SDS for bone age in group A were significantly lower than those in group B(P<0.05). In group A, the mean PAH increased from 147.0+/-1.9 to 153.7+/-3.7cm during two years of treatment, but no difference in PAH was found in group B. So PAH were similar in two groups(154.1+/-8.7 vs. 153.7+/-3.7cm) after second year. The mean difference between bone age and chronological age decreased from 4.5+/-1.3 to 3.0+/-1.3 years in group A(P<0.05). CONCLUSION: Long-term Tryptorelin treatment is more effective in girls with idiopathic central precocious puberty, whose PAH before treatment were below 150cm. If PAH before treatment is 150 cm or greater or increase in PAH during the first year of treatment is poor, this treatment modality might be useless in terms of improved growth.
Adolescent ; Adult ; Female* ; Gonadotropin-Releasing Hormone* ; Humans ; Lutein* ; Puberty ; Puberty, Precocious*

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

A patient, 59 years old male, with paroxysmal AV reentrant tachycardia utilizing a concealed left lateral accessory pathway with long VA conduction time is presented. During tachycardia P waves were negative in leads I, aVL, and V6 and positive in leads aVR and V1. The R-P/P-R ratio was 1.6. Radiofrequency catheter ablation was successful to interrupt the VA conduction through the accessory pathway.
Catheter Ablation ; Humans ; Male ; Middle Aged ; Tachycardia*

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