The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease. The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed. The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more... |
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. We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic... |
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. The patient underwent surgical resection. Pathological examination revealed a low-grade perivascular epithelioid cell tumor (PEComa). PEComa in the colon is very rare. Only a few cases have been reported so far. An effective treatment method for... |
Purpose : This study aims to compare the outcome of total body irradiation (TBI)- or non-TBI-containing conditioning regimens for leukemia in children. Methods : We retrospectively evaluated 77 children conditioned with TBI (n=40) or non-TBI (n=37) regimens, transplanted at Chonnam National University Hospital between January 1996 and December 2007. The type of transplantation, disease status at the time of transplant, conditioning regimen,... |
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other... |
Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children younger than 4 years and is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. HUS associated with diarrheal prodrome is usually caused by Shiga toxin-producing Escherichia coli O157:H7 or by Shigella dysenteriae, which generally has a better outcome. However, atypical cases show a... |
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... |
Facial palsy as the presenting symptom of leukemia is very rare, especially in acute myeloid leukemia. A review of the medical literature identified reports on 8 children with AML who had facial paralysis as the presenting sign. Whole brain irradiation (WBI) has been applied in most cases. We present the cases of 3 such children. Achieving a remission without WBI,... |
The clinical features of ring chromosome 13 include mental and growth retardation, CNS anomalies, facial dysmorphism, cardiac defects, genital malformations, limb anomalies, skeletal deformities and anal malformations. Although many cases of ring chromosome 13 have been reported worldwide, only 6 cases have been reported in Korea, and the latter cases were not mosaic but pure ring chromosome 13. Here we... |
Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome is caused by deletion of chromosome 11p13, including the Wilms` tumor (WT1) and aniridia gene (PAX6) loci. Here, we report the first case of WAGR syndrome in Korea; the patient was a 2-year-old girl with bilateral aniridia from birth who presented with abdominal distention and mental retardation. Cytogenetically, she had... |
A lung hernia, defined as the protrusion of pulmonary tissue and pleural membranes through a defect in the thoracic wall, is a rare event. It can be congenital or acquired, and cervical, thoracic, or diaphragmatic in location. We report the rare occurrence of a congenital atraumatic lung herniation through the azygoesophageal recess. An 8-month-old male infant, who was born... |
Paragonimiasis is a parasitic infection that occurs following the ingestion of infectious Paragonimus metacercariae from crabs or crayfish. Pulmonary paragonimiasis is the most common clinical manifestation of this infection, but several ectopic paragonimiasis cases have also been reported. Among them, cases of subcutaneous paragonimiasis are rare, especially in children. We report a case of subcutaneous paragonimiasis of the right abdominal... |
Hematopoietic stem cell transplantation (HSCT) has expanded and evolved substantially in the last decades to treat various malignant and nonmalignant diseases. However, the conditioning regimen can lead to transplantation related death by major organ dysfunction, severe infection and bleeding. In the allogeneic setting, graft versus host disease may also develop, making post-transplant management complex. To overcome these problems, new stem... |
Sideroblastic anemia is a rare, heterogeneous group of disorders characterized by hyperferremia, microcytic hypochromic anemia, and bone marrow erythroid hyperplasia with the presence of numerous ringed sideroblasts. We describe herewith the case of a rare coincidence of sideroblastic anemia and mitral valve prolapse with resultant regurgitation in a 2-year-old boy. In addition to the inherent propensity for the development of... |
Purpose : Popular use of fetal ultrasonography has increased to detect congenital hydronephrosis(CH) which is the most common anomaly prenatally detected. We'd like to determine the frequency and clinical characteristics of prenatally diagnosed CH and outcome of ureteropelvic junction stenosis(UPJS). Methods : The records of births between January 1994 and June 2003 in Chonnam National University Hospital(CNUH), and the records of... |
Purpose : The mortality rate of neonatal sepsis has been decreased, however, the incidence has not significantly decreased because of increased invasive procedures. This study was designed to make guidelines for choosing antibiotics by analyzing the causative microorganisms and their antibiotics sensitivity test according to the onset of neonatal sepsis. Methods : One hundred seven cases of culture proven sepsis... |
Purpose : Vancomycin-resistant enterococci(VRE) are now nosocomial pathogens in Korea. But little is known about the prevalence of stool colonization with VRE in neonates in Korea. So we studied the prevalence and risk factors of VRE colonization in the Neonatal Intensive Care Unit(NICU). Methods : From January 2000 to December 2004, the medical records of 294 neonates(127 cases of VRE group... |
Isolated relapse of myeloid leukemia as a granulocytic sarcoma(GS) following allogeneic bone marrow transplantation(BMT) is very rare manifestation, and usually associated with a poor prognosis. We report a case of isolated intracranial GS in an infant with myelodysplastic syndrome(MDS) following unrelated BMT. A 7 month-old girl was diagnosed with refractory anemia with excess blasts (RAEB). During observation for a couple... |
Purpose : Neurologic complications(NC) after hematopoietic stem cell transplantation(HSCT) are important because of high mortality and severe sequelae. We analyzed the incidence, manifestation and risk factors of NC in 61 children undergoing HSCT. Methods : We retrospectively analyzed NC in 61 cases(42 male and 19 female, aged one to 16 years) given HSCT between 1996 and 2003 due to hematologic malignacies,... |
Purpose : With the development of neonatal intensive care and the increased use of systemic antibiotics, candida sepsis has become one of the most important causes of neonatal morbidity and mortality. The purpose of this study was to investigate the prevalence rate and its associated risk factors. Methods : We retrospectively reviewed medical records of 28 cases with candida sepsis who... |
Purpose : We performed this study to evaluate the clinical efficacy and safety of dexamethasone in preventing laryngeal edema following exubation. Methods : We retrospectively reviewed the medical records of 100 neonates who underwent endotracheal intubation and mechanical ventilation. Subjects were divided into two groups; dexamethasone treated(60 cases, dexamethasone group) and non-treated(40 cases, control group). We examined the underlying diseases of... |
Wolman' disease is a rare autosomal recessive disorder in which a deficiency of lysosomal acid lipase leads to the accumulation of cholesteryl esters and trigycerides in most of the body tissues. Clinically, it is characterized by abdominal distention, hepatosplenomegaly, vomiting, intractable diarrgeam steatorrhea, malabsorption, inanitionm failure to thrive, and bilateral enlargement and calcification of the adrenal glands demonstrated by roentgenographic... |
Purpose : The universal vaccination against hepatitis B during early infancy is the only effective way to control hepatitis B infection in highly endemic areas in Korea. The purpose of this study was to assess the effectiveness of simultaneous hepatitis B vaccination with DPT and oral polio at 2, 4, 6 months of age in babies of HBs antigen negative mothers. Methods : One... |
Purpose : Fungal infections are an important cause of morbidity and mortality in patients with hematologic malignancies. The therapy of choice in documented or suspected invasive fungal infections has been intravenous Amphotericin B(AmB). Adverse effects such as fever, chills, thrombophlebitis, nausea or vomiting are common. A more serious adverse effect is potential renal impairment. As AmB administration mixed with Intralipid(AmB/Intralipid)... |
Purpose : Since umbilical cord blood (UCB), which used to be discarded, was found to be a source of enriched hematopoietic stem and progenitor cells, basic research to elucidate characteristics of UCB hematopoietic stem cells (HSCs) and its clinical application to bedside transplantation have been attempted. Moreover, stem cell transplantation (SCT) has expanded its role, not only in hematopoietic reconstitution, but in cancer therapy,... |
Purpose : We studied the incidence and clinical course of BCG lymphadenitis to investigate the BCG related problem. Subject and Methods: Three hundred and eighty-six infants received BCG vaccine (Pasteur-1173p2) in the Well Baby Clinic, Dept. of Pediatrics, Chonnam University Hospital from May 1993 and April 1994. Among them, twenty three infants developed regional lymphadenitis. We investigated the clinical course... |
Purpose : Central venous catheters are used to deliver antibiotics, chemotherapeutic agents, and parenteral nutrition for a extended period of time as well as to draw samples in immunocompromised patients to minimize the discomfort with sampling and the risk of introducing infections. Hickman/Broviac catheters or implantable devices have been used in pediatric patients. This study is aimed to evaluate the safety and clinical efficacy of... |
Steroid-induced lipodystrophy is preduced by long-acting injectable glucocortiocoids (triamcinolone acetonides) within three weeks after intramuscular injection. It can be caused by inappropriate use of injectable steroids. Although it is recovered spontaneously without medication or plastic surgery, it may lasts for several years. Recently we experienced four cases of steroid-induced lipodystrophy and present a brief review of related literatures. |
Thirty one infants of diabetic mothers(IDM) who had been admitted in Neonatal Intensive Care Unit at Chonnam University Hospital from January 1987 to July 1991 were studied for evaluation of their perinatal outcome and prognosis. The results were as follws; 1) The distribution of diabetic mothers according to modified White뭩 classification was GD & class A in 12 cases (38.7%), B in... |
Providing pediatric cancer patients with medical information is thought to facilitate realistic coping; however, systematic research into this concept has been lacking. We analyzed the attitudes of nationwide 220 cancer patients, their parents and doctors towards the care of children with cancer, concerning the patients' understanding of their diseases, from whom the patient heard about his(her) disease, whether the patient... |
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