PURPOSE: The aim of this study was to compare the results of the lateral entry pin technique and the crossed pin technique in treatment of Gartland type III humerus supracondylar fracture. MATERIALS AND METHODS: Seventeen patients (group I) underwent surgery using the lateral entry pin technique, and 33 patients (group II) underwent surgery using the crossed pin technique for Gartland type III humerus supracondylar fracture in Hanyang University Seoul Hospital between January 2011 and January 2014. Maintenance of reduction was compared between the 2 surgical techniques by measuring changes in Baumann angle and lateral humerocapitellar angle after surgery and after pin removal in groups I and II. In addition, the final carrying angle and level of loss of functional movement were measured for comparison of clinical results between the 2 groups. Occurrence of ulnar nerve palsy in the 2 groups was also examined. RESULTS: The mean Baumann angle and lateral humerocapitellar angle changes were 3.3degrees and 3.7 in group I and 3.1degrees and 3.4degrees in group II, respectively. No statistically significant differences were found between the 2 groups. Clinical results showed that the changes in the final carrying angle and range of motion were 2.9degrees and 2.6degrees in group I and 2.6degrees and 3.0degrees in group II, respectively, indicating no significant differences between the 2 groups. In terms of nerve damage, 1 patient in group II had temporary iatrogenic ulnar nerve palsy. CONCLUSION: The lateral entry pin technique may be regarded as an appropriate treatment that reduces the risk of iatrogenic ulnar nerve palsy and provides satisfactory results in Gartland type III humerus supracondylar fracture patients.
Humans ; Humeral Fractures* ; Humerus ; Range of Motion, Articular ; Seoul ; Ulnar Neuropathies

Ganglion cysts that occurred within sheaths of peripheral nerves have been documented in literatures, but it is relatively rare condition. The peroneal nerve is the most common site of involvement. Other reported sites of involvement are the radial, ulnar, median, sciatic, tibial and posterior interosseous nerves. We report a case of the intraneual ganglion cyst within peroneal nerve and another case of the intraneural ganglion cyst within a medial plantar nerve that were successfully excised without neurologic complication.
Ganglion Cysts* ; Peripheral Nerves* ; Peroneal Nerve ; Tibial Nerve

An experiment was planned to observe the histopathological alteration with administration of the Rheomacrodex and blood pressure changes in induced cerebral infarct after occlusion. Eighty well developed cats, weighing 2.3 to 3.5kg, were used in this experiment. The right MCA was exposed through temporal approach and the proximal part of the MCA was occluded with a silver clip. The animals were divided into 4 groups: The control group was comprised of 20 cats with occlusion of the right MCA alone, Rheomacrodex-treated group was comprised of 20 cats after occlusion of right MCA, induced hypotension and hypertension groups consisted in each 20 cats following occlusion of the MCA. The animals were sacrificed at intervals of 3 hours, 6 hours, 24 hours, 1 week and 2 weeks respectively after occlusion of the MCA. The animals were studied for clinical deficits and histopathological changes of the cerebral infarct according to the time courses. The results obtained were as follows: 1) In the control group, severe contralateral hemiplegia was developed in the early stage following the MCA occlusion, however the neurological deficits were progressively improved to the state of abnormal walking in 24 hours to 2 weeks. The hemorrhagic infarct was involving the basal ganglia, internal capsule and extending to the cortex with mild brain edema in the early stage and the area of the infarct was gradually enlarged from 6 hours to 24 hours following the MCA occlusion. Although the brain edema of surrounding area of the lesion was remained unchanged, the size and distribution of the infarct were decreased in one week to 2 weeks. Extensive ischemic neuronal damage was observed in the control group. 2) In the Rheomacrodex-treated group, mild to moderate neurological deficit was developed in the early stage after MCA occlusion and the deficit was less severe than control group. The clinical deficit was improving in the time course and one case had shown completely normal activity in 2 weeks. The distribution of the infarct was well defined and it was smaller than control group. The infarct mainly involved the basal ganglia and internal capsule. The area of the infarct was gradually enlarged from 6 hours to one week after MCA occlusion, then the extent of the infarct was decreased in 2 weeks. The ischemic neuronal change in this group was less severe than control group. 3) In the induced hypotension group, the early neurological deficit was worse than that of the control group and severe hemiplegia was developed in one week. There was minimal improvement of the neurological deficit in 2 weeks. The area of the infarct was ill-defined and hemorrhagic extending a large portion of the brain with severe brain edema. The infart was involving the basal ganglia, internal capsule, claustrum and the cortex from 3 hours to 24 hours after the occlusion and the area of the infarct was not changed during the observation. Severe ischemic nerve cell change or resolution of the cells was oserved in this group. 4) In the induced hypertension group, the neurological deficit was mild and it was better than that of the control group. The distribution of the infarct was well localized and minimum in extent. The extent of the infarct was not changed during the observation. There was no observable gross brain edema and the ischemic nerve cell changes were not severe.
Animals ; Basal Ganglia ; Blood Pressure* ; Brain ; Brain Edema ; Cats ; Dextrans* ; Hemiplegia ; Hypertension ; Hypotension ; Internal Capsule ; Middle Cerebral Artery* ; Neurons ; Silver ; Walking

Aim of this study was to compare the outcomes of transplantation by donor source and to help select the best alternative donor in children with leukemia. Donor sources included matched related donor (MRD, n = 35), allele-matched unrelated donor (M-UD, n = 10) or -mismatched (MM)-UD (n = 13) or unrelated umbilical cord blood (UCB, n = 11). UCB group had a significantly higher incidence of grade II-IV acute graft versus host disease (MRD, 11.8%; M-UD, 30.0%; MM-UD, 15.4%, UCB, 54.4%, P = 0.004) but there was no difference in incidence of chronic graft versus host disease between 4 groups. The 5-yr leukemia-free survival (LFS) was 76.7%, 60.0%, 69.2%, and 45.5%, respectively (P = 0.128). MRD group showed higher LFS rate than UCB group (P = 0.022). However, LFS of M-UD and MM-UD together (65.2%) was not different from that of MRD group (76.7%, P = 0.325), or from that of UCB (45.5%, P = 0.190). The relapse incidence at 5 yr was 17.1%, 20.0%, 15.4%, and 0%, respectively (P = 0.460). The 100-day treatment-related mortality was 2.9%, 20.0%, 7.7%, and 36.4%, respectively (P = 0.011). Despite the limitations of small number of patients, unrelated donor transplants including even allele-mismatched ones, seem to be as effective in children with leukemia lacking suitable relative donors. Also, UCB transplant may serve as another possible option in urgent transplants.
Adolescent ; Child ; Child, Preschool ; *Cord Blood Stem Cell Transplantation/adverse effects/methods ; Disease-Free Survival ; Female ; Fetal Blood/transplantation ; Graft vs Leukemia Effect ; *Hematopoietic Stem Cell Transplantation/adverse effects/methods/mortality ; Histocompatibility Testing ; Humans ; Infant ; Leukemia/mortality/*therapy ; Male ; Transplantation, Homologous ; Treatment Outcome ; Unrelated Donors

The term MYH9-related disorders indicates a group of autosomal dominant illnesses, formerly known as May-Hegglin anomaly, Sebastian syndrome, Fechtner syndrome and Epstein syndrome, caused by mutations of MYH9, the gene encoding for the heavy chain of non-muscle myosin IIA (NMMHC-IIA). We experienced a family with macrothrombocytopenia without leukocyte inclusion. A 5-year-old girl was found to have macrothrombocytopenia incidentally. Her father also had macrothromtocytopenia, but had been suffering from hearing loss and chronic renal failure. Meticulous search by light and electron microscopy failed to detect leukocyte inclusions. To our knowledge, these cases seem to be the first description of autosomal dominant Epstein giant platelet syndrome in Korea.
Bernard-Soulier Syndrome* ; Child, Preschool ; Fathers ; Female ; Hearing Loss ; Humans ; Kidney Failure, Chronic ; Korea ; Leukocytes ; Microscopy, Electron ; Nonmuscle Myosin Type IIA

BACKGROUND: The prognosis of pediatric acute myeloid leukemia (AML) has recently improved. This study aimed to describe the epidemiology, changes in treatment strategies, and improvement of outcomes in Gwangju-Chonnam children with AML over 2 decades. METHODS: Medical records of 116 children with newly diagnosed AML were retrospectively reviewed for demographic characteristics, prognostic groups including cytogenetic risks, treatment protocols, and survival rates over the periods between 1996 and 2005 (Period I, N=53), and 2006 and 2015 (Period II, N=38). RESULTS: The annual incidence of AML has decreased with reduced pediatric population. The 5-year Kaplan-Meier (K-M) estimated overall survival (OS) and event-free survival (EFS) rates in 110 AML patients were 53.2±5.1% and 43.8±5.1%, respectively. The 5-year OS rate significantly improved during period II (70.3±7.0%) as compared to that during period I (40.0±6.8%) (P =0.001). The 5-year OS was not significantly different among cytogenetic risk groups (P =0.11). Fifty-eight patients underwent hematopoietic stem cell transplantation (HSCT). The K-M 5-year estimated survival for transplanted patients was 53.7±7.0%, while that for chemotherapy-only patients was 30.1±9.1% (P =0.014). Among the prognostic factors, treatment modality was the only independent factor. The chemotherapy-only group had a relative risk of 2.06 for death compared with the transplantation group (P=0.015). CONCLUSION: The survival of Korean children with AML has improved to a level comparable with that of developed countries over 2 decades, owing to a change in induction strategy, better supportive care with economic growth, refinement of HSCT techniques including a better selection of patients based on prognostic groups, and stem cell donor selection.
Child* ; Clinical Protocols ; Cytogenetics ; Developed Countries ; Disease-Free Survival ; Donor Selection ; Economic Development ; Epidemiology ; Hematopoietic Stem Cell Transplantation ; Humans ; Incidence ; Leukemia, Myeloid, Acute* ; Medical Records ; Prognosis ; Retrospective Studies ; Stem Cells ; Survival Rate ; Treatment Outcome*

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and might be an effective option in patients with EBV-HLH with progressive CNS disease. Moreover, the addition of rituximab to RIC appears to be safe and effective in suppressing EBV in the patients with EBV-HLH.
Antibodies, Monoclonal, Murine-Derived ; Central Nervous System ; Central Nervous System Diseases ; Herpesvirus 4, Human ; Humans ; Lymphohistiocytosis, Hemophagocytic ; Stem Cell Transplantation ; Stem Cells ; Transplants ; Rituximab

X-linked hyper-IgM syndrome (XHIM) is a rare primary immunodeficiency disorder, caused by mutations of the gene encoding CD40 ligand (CD40L; CD154). We report the clinical manifestations and mutational analysis of the CD40L gene observed in a male patient from a XHIM family. Having hypogammaglobulinemia and elevated IgM, the 3-yr-old boy exhibited the characteristic clinical features of XHIM. The patient suffered from frequent respiratory infections, and chronic enteritis caused by Cryptosporidium parvum. In addition, a lymph node biopsy and a culture from this sample revealed C. neoformans infection. Activated lymphocytes from the patient failed to express CD40L on their surface as assessed by flow cytometry and a missence mutation (W140R) was found at the XHIM hotspot in his CD40L cDNA to confirm the diagnosis. Genetic analysis of the mother and sister showed a heterozygote pattern, indicating carrier status. To our knowledge, this is the first report on the molecular diagnosis of an XHIM patient in Korea.
Animals ; CD40 Ligand/*genetics ; Child, Preschool ; Cryptococcosis/*complications/genetics/immunology ; Cryptococcus neoformans ; Cryptosporidiosis/*complications/genetics/immunology ; *Cryptosporidium parvum ; Female ; *Heterozygote ; Humans ; Hypergammaglobulinemia/complications/*diagnosis/genetics/immunology ; Immunoglobulin M/*blood ; Korea ; Male ; Pedigree ; *X Chromosome

Selective IgA deficiency is one of the most common primary immunodeficiency. Some patients with IgA deficiency also have deficits in one or more immunoglobulin G subclasses. It has been estimated that up to 25% of patients with certain primary immunodeficiencies will develop tumors, primarily B-cell lymphomas during their lifetime. We hereby present 2 cases of malignant lymphomas, one diffuse large cell lymphoma and another mixed cellularity Hodgkin's disease, respectively, which developed in patients with selective IgA and IgG subclass deficiency.
Child* ; Hodgkin Disease ; Humans ; IgA Deficiency ; Immunoglobulin A* ; Immunoglobulin G* ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Large B-Cell, Diffuse ; Lymphoma, Non-Hodgkin

PURPOSE: Hepatic veno-occlusive disease (VOD) is a life-threatening complication occurring early after stem cell transplantation (SCT). Early diagnosis and effective treatment has not been established in severe VOD. Because there are few reports on VOD in Korean children, we evaluated the clinical characteristics of VOD following SCT in children. METHODS: We retrospectively reviewed the chart of all patients (n=116) receiving SCTs in CNUH Pediatric BMT center between May, 1991 and June, 2004. RESULTS: VOD developed in 11 patients (9.5%) (median age, 9.8 years; range, 2 to 13.9). Underlying diagnoses were ALL (n=3), severe aplastic anemia (n=3), AML (n=2), acute biphenotypic leukemia (n=1), neuroblastoma (n=1), and myelodysplastic syndrome (n=1). The median day of onset of VOD was D+9 (range, D-3 to D+19). VOD was classified as moderate in 5 and severe in 6 cases. Maximum level of serum total bilirubin was 2.9 mg/dL (range, 2.1 to 9.2) in moderate VOD and 7.3 mg/dL in severe VOD (range, 2.0 to 24.2) at D+18 (range, D-5 to D+59). We successfully treated VOD with various combinations including tPA and heparin (2/5, 40%), ursodeoxycholic acid (2/5, 40%), N-acetylcysteine (3/5, 60%), and defibrotide (1/2, 50%). All of 5 patients with moderate VOD survived at D+100 (range, 5.5+ to 66.6+ months). Five of 6 (83%) patients with severe VOD died within first 19 day from complications of VOD. CONCLUSION: This retrospective study showed that the incidence of VOD was 9.5%, and the mortality of severe VOD was still high which would necessitate early diagnosis, effective prevention and treatment.
Acetylcysteine ; Anemia, Aplastic ; Bilirubin ; Child ; Diagnosis ; Early Diagnosis ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Heparin ; Hepatic Veno-Occlusive Disease* ; Humans ; Incidence ; Leukemia, Biphenotypic, Acute ; Mortality ; Myelodysplastic Syndromes ; Neuroblastoma ; Retrospective Studies* ; Stem Cell Transplantation ; Ursodeoxycholic Acid