Glaucoma Progression in Treatment-Naïve Normal-Tension Glaucoma Patients with Myopia - Role of Intraocular Pressure.

Precis: While myopia has been recognized as a positive prognostic factor for NTG progression in the adult population, some myopic NTG eyes exhibited significant progression within two years when left untreated, even under low IOP.

Purpose: To determine the natural history and risk factors associated with progressive retinal nerve fiber layer (RNFL) thinning in previously stable, treatment-naïve, normal-tension glaucoma (NTG) patients with myopia.

Methods: This study included 111 myopic NTG eyes without intraocular pressure (IOP)-lowering treatment for at least 1 year and without disease progression during the treatment-free period.

Alveolar rhabdomyosarcoma with massive disseminated intravascular coagulopathy treated with systemic chemotherapy.

It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis.

Radiodensity on serial chest X-rays for the diagnosis of foreign body aspiration in children.

Objective: To evaluate the utility of measuring lung radiodensity from chest X-ray for the diagnosis of foreign body aspiration.

Methods: Records of 59 children with foreign body aspiration were retrospectively reviewed. Lung radiodensity and radiodensity ratio (right/left lung radio density) before and after foreign body removal were measured.

Graves disease following rabbit antithymocyte globulin treatment of severe aplastic anemia in a Korean child.

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA.

Thyroid autoimmunity in children and adolescents with newly diagnosed type 1 diabetes mellitus.

Purpose: This study aim to investigate the occurrence of autoimmune thyroid disease in children and adolescents at onset of type 1 diabetes mellitus (T1DM) and to assess whether the presence of diabetes-specific autoantibodies can predict the autoimmune thyroid disorder.

Methods: Seventy-three children with T1DM were recruited. Glutamic acid decarboxylase antibodies (GADA), islet cell antibodies (ICA), insulin autoantibodies (IAA), and thyroid antibodies were determined in all patients at the time of diagnosis.

Long-term follow-up of Fanconi anemia: clinical manifestation and treatment outcome.

Purpose: The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease.

Methods: The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed.

Results: The median age at diagnosis was 6.

Hereditary hemolytic anemia in Korea from 2007 to 2011: A study by the Korean Hereditary Hemolytic Anemia Working Party of the Korean Society of Hematology.

Background: The number of patients diagnosed with hereditary hemolytic anemia (HHA) has increased since the advent of novel diagnostic techniques that accurately identify this disorder. Here, we report data from a survey on the prevalence and characteristics of patients diagnosed with HHA in Korea from 2007 to 2011.

Methods: Information on patients diagnosed with HHA in Korea and their clinical and laboratory results were collected using a survey questionnaire.

Solitary Langerhans cell histocytosis of skull and spine in pediatric and adult patients.

Purpose: Langerhans cell histiocytosis (LCH) is a rare neoplasm and has heterogeneous clinical presentation and behavior. We analyzed solitary lytic lesions of the skull and spine in pediatric and adult patients.

Methods: Between 2001 and 2011, 42 patients underwent surgery for LCH.

Quantitative comparison of CDKN2B methylation in pediatric and adult myelodysplastic syndromes.

Background/aims: Transcriptional repression of tumor suppressor genes is determined by the quantity of promoter hypermethylation. We analyzed the methylation quantity of CDKN2B in pediatric myelodysplastic syndromes (MDS).

Methods: Quantitative measurement of CDKN2B methylation was performed in 25 pediatric MDS patients and 12 controls using pyrosequencing, and the result was compared with those from 74 adult MDS cases and 31 adult controls.

Pre-engraftment syndrome after unrelated cord blood transplantation: a predictor of engraftment and acute graft-versus-host disease.

Pre-engraftment syndrome (PES) is poorly characterized, and its clinical significance and the prognostic impact after unrelated cord blood transplantation (CBT) are unclear. To address these issues, we retrospectively analyzed the incidence, risk factors, and clinical outcomes of PES in unrelated CBT recipients. Data of 381 patients who received unrelated CBT from 18 medical centers in Korea were reviewed.

Implication of early lymphocyte recovery after allogeneic hematopoietic stem cell transplantation in children with leukemia.

Purpose: The repopulating lymphocytes after allogeneic hematopoietic stem cell transplantation have an important role not only on the prevention of serious infections in the early transplantation period, but also on the killing of residual leukemic cells by graft-versus-leukemia effect. The aim of this study was to analyze the impact of lymphocyte recovery after allogeneic stem cell transplantation in children with hematologic malignancies.

Materials And Methods: We evaluated 69 children transplanted for acute lymphoblastic leukemia (ALL) (n=34), acute myeloid leukemia (AML) (n=26), chronic leukemia (n=7) and juvenile myelomonocytic leukemia (n=2) between 1996 and 2008 at the Chonnam National University Hospital, Korea.

Founder effects in two predominant intronic mutations of UNC13D, c.118-308C>T and c.754-1G>C underlie the unusual predominance of type 3 familial hemophagocytic lymphohistiocytosis (FHL3) in Korea.

Familial hemophagocytic lymphohistiocytosis (familial HLH or FHL) is a potentially fatal autosomal recessive disorder. Our previous study demonstrated that UNC13D mutations (FHL3) account for ~90 % of FHL in Korea with recurrent splicing mutation c.754-1G>C (IVS9-1G>C).

Recurrent plastic bronchitis in a child with 2009 influenza A (H1N1) and influenza B virus infection.

Plastic bronchitis is an uncommon disorder characterized by the formation of bronchial casts. It is associated with congenital heart disease or pulmonary disease. In children with underlying conditions such as allergy or asthma, influenza can cause severe plastic bronchitis resulting in respiratory failure.

Scrub typhus-associated severe hemophagocytic lymphohistiocytosis with encephalomyelitis leading to permanent sequelae: a case report and review of the literature.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disorder. There have been a few reports on HLH secondary to scrub typhus in adults. Here, we describe the case of a 9-year-old Korean girl who presented with the typical findings of HLH.

Unicentric Castleman disease relapsed after rituximab-CHOP chemotherapy or radiation therapy in an adolescent.

Castleman disease (CD), an atypical lymphoproliferative disorder of unknown etiology, is rare. Unicentric CD can be cured after resection of the involved lymph nodes. However, rarely, patients with the unicentric-plasma cell variant may require additional therapy after resection for persistent systemic symptoms.

Long-term graft-versus-tumor effect following reduced intensity hematopoietic stem cell transplantation in a child with metastatic renal cell carcinoma.

Pediatric renal cell carcinoma (RCC) is rare and different from adult RCC. Although target agents have recently been introduced, allogeneic hematopoietic stem cell transplantation exploiting graft-versus-tumor effect still remains an important treatment option for metastatic RCC. A 2-year-old male with RCC developed hepatic metastases 6 months following radical nephrectomy and subsequent cytokine therapy.

Hematopoietic stem cell transplantation in children with leukemia: a single institution experience with respect to donors.

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.

Mitochondrial DNA copy number and hnRNP A2/B1 protein: biomarkers for direct exposure of benzene.

The present study was performed to identify biomarkers for exposure of benzene in blood cells and hematopoietic tissues. Peripheral mononuclear cells, hematopoietic stem cells, and leukemia cell lines were cultured in RPMI 1640 media with the addition of 0, 1, and 10 mM of benzene. Hydrogen peroxide was measured using an enzyme immunoassay.

Selective embolization of the internal iliac arteries for the treatment of intractable hemorrhage in children with malignancies.

Purpose: Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies.

Perivascular epithelioid cell tumor (PEComa) of the ascending colon: the implication of IFN-α2b treatment.

A 7-year-old boy presented with hematochezia and abdominal pain. A 3.7-cm-sized mass was identified in the ascending colon by abdominal computed tomography and colonoscopy.

Efficacy and safety of deferiprone (Ferriprox), an oral iron-chelating agent, in pediatric patients.

Background: Iron overload is a predictable and life-threatening complication in patients dependent on the regular transfusion of RBCs. The aims of this study were to investigate the efficacy and safety of deferiprone in a variety of pediatric hematologic and/or oncologic patients with a high iron overload.

Methods: SEVENTEEN PATIENTS (AGE: 1.