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Original Article
Multiple extrarenal manifestations in hemolytic uremic syndrome: A case report
Eugene Kim, So-Young Kim
Clin Exp Pediatr. 2007;50(12):1261-1265.   Published online December 15, 2007
Extrarenal manifestations of hemolytic uremic syndrome (HUS) have increasingly been recognized and may be major determinants of mortality and morbidity. Although microthrombi are often found in the pulmonary and coronary circulation, apparent lung and cardiac involvement are clinically infrequent. We describe here a 10-month-old boy with HUS who developed pulmonary hemorrhage, acute respiratory distress syndrome and dilated cardiomyopathy. Complete renal...
A case of neonatal amoebiasis with after-birth vomiting and bloody stool
Jimin Kahng, So-Young Kim
Clin Exp Pediatr. 2007;50(12):1257-1260.   Published online December 15, 2007
E. histolytica has a simple life cycle with two stages: an infective cyst and an invasive trophozoite. It lives on humans as its host. Its infection occurs through the ingestion of the cyst form, and the disease begins when the trophozoite, converted at the small intestine, adheres to colonic epithelial cells with a latent period of two days to four...
A case of cystic fibrosis presented with meconium ileus in a female neonate
In-Ok Hwang, Eun-Sil Lee
Clin Exp Pediatr. 2007;50(12):1252-1256.   Published online December 15, 2007
Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF) in infants. It arises from the intraluminal accumulation of highly viscid, protein-rich meconium, typically present in the terminal ileum as a neonatal intestinal obstruction. Therefore, the clinical symptoms include abdominal distension, bilious vomiting and delayed passage of meconium. CF is caused by mutations in the transmembrane conductance regulator...
Change of hemostatic markers according to the clinical state in Kawasaki disease
Yong Beom Kim, You Sook Yoon, Sang Yun Lee, Hong Ryang Kil
Clin Exp Pediatr. 2007;50(12):1247-1251.   Published online December 15, 2007
Purpose : Pathologically, Kawasaki disease (KD) is associated with widespread vascular endothelial damage in the acute phase. The vasculitis induced endothelial injury leads to coagulation abnormalities. Abnormalities of endothelial function, platelet activation, and fibrinolysis are present during acute phase and long after the onset of KD. The aim of study is to evaluate the change of hemostatic markers in the...
The effects of shortened dexamethasone administration on remission rate and potential complications during remission induction treatment for pediatric acute lymphoblastic leukemia
Jae Wook Lee, Kwang Hee Lee, Young Joo Kwon, Dae Hyoung Lee, Nak Gyun Chung, Dae Chul Jeong, Bin Cho, Hack Ki Kim
Clin Exp Pediatr. 2007;50(12):1217-1224.   Published online December 15, 2007
Purpose : Due to its high potency against leukemic blasts, our institution has opted for the use of dexamethasone during acute lymphoblastic leukemia (ALL) remission induction, but in our most recent treatment protocol, CMCPL-2005, we shortened the length of steroid treatment from 4 to 3 weeks. We compared both the rates of remission induction and significant complications observed during induction...
Risk Factors of Unprovoked Seizures after Acute Symptomatic Seizures in Children
Eun Ju Lee, Won Seop Kim
Clin Exp Pediatr. 2007;50(11):1097-1103.   Published online November 15, 2007
Purpose : Acute symptomatic seizure is defined as a temporary seizure together with acute systemic, metabolic, or toxic insult in association with an acute central nervous system insult. And unprovoked seizure is defined as seizure without provocating factors. We studied the risk factors of unprovoked seizures after acute symptomatic seizure in children. Methods : We retrospectively reviewed the records of...
Late aortic root dilatation and aortic regurgitation in repaired tetralogy of fallot
Jeong Eun Kim, Kyong Hur, Hae Sik Kwon, Byung Won Yoo, Jae Young Choi, Jun Hee Sul
Clin Exp Pediatr. 2007;50(10):976-981.   Published online October 15, 2007
Purpose : Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its hemodynamic nature in patients with repaired TOF. Methods : We investigated 130 repaired TOF patients more than 15 years of age who followed-up by...
Review Article
Hemolytic uremic syndrome
Hye Won Park
Clin Exp Pediatr. 2007;50(10):931-937.   Published online October 15, 2007
The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however,...
Original Article
Serum ghrelin and leptin concentrations in children with cancer : comparisons with normal children
So Hyun Park, Min Ho Jung, Nac Gyun Chung, Byung-Kyu Suh, Byung Churl Lee
Clin Exp Pediatr. 2007;50(9):905-911.   Published online September 15, 2007
Purpose : Ghrelin, being secreted from the stomach, stimulates growth hormone secretion and controls energy homeostasis by increasing appetite. Leptin, being secreted from the adipocytes, controls weight and energy homeostasis by decreasing appetite. Leptin concentration is reported to increase after childhood cancer therapy. This study was aimed to compare ghrelin and leptin concentrations in normal children and children who received...
Chromosomal analyses of 4,500 cases of the peripheral blood : An experience in a single hospital for 25 years
Hye-Eun Seo, Ji Hye Lee, Ji Yoon Kim, Dong Ha Lee, Heung Kyo Lee, Kun Soo Lee
Clin Exp Pediatr. 2007;50(9):875-881.   Published online September 15, 2007
Purpose : Chromosome analysis is important in genetic study and genetic counseling. This study was performed to evaluate the type and incidence of chromosome abnormalities in a single hospital for 25 years. Methods : Chromosome analyses were performed on peripheral blood lymphocytes, obtained from 4,856 patients with suspected chromosomal aberrations, referred to cytogenetic laboratory in Department of Pediatrics, Kyungpook National...
Review Article
The etiologies of neonatal cholestasis
Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2007;50(9):835-840.   Published online September 15, 2007
Any infant noted to be jaundiced at 2 weeks of age should be evaluated for cholestasis with measurement of total and direct serum bilirubin. With the insight into the clinical phenotype and the genotypephenotype correlations, it is now possible to evaluate more precisely the neonate who presents with conjugated hyperbilirubinemia. Testing should be performed for the specific treatable causes of...
Original Article
Plasma B-type natriuretic peptide (BNP): a useful marker for anthracycline-induced cardiotoxicity in Korean children with cancer
Hyun Dong Lee, Jae Min Lee, Yong Jik Lee, Young Hwan Lee, Jeong Ok Hah
Clin Exp Pediatr. 2007;50(8):774-780.   Published online August 15, 2007
Purpose : The anthracyclines (AC) are widely used chemotherapeutic agents for pediatric cancers. However, the therapeutic use of these agents is limited by their cardiotoxicity. The aim of the present study was to investigate the usefulness of plasma B-type natriuretic peptide (BNP) levels as a marker for AC-induced cardiotoxicity compared to echocardiography in Korean children with cancer. Methods : Fifty-five pediatric...
A study for post-diagnostic lumbar puncture headache in children
Gook Chan Jang, Eun Seok Yang, Kyung Rye Moon, Young Bong Park, Young Il Rho
Clin Exp Pediatr. 2007;50(8):761-766.   Published online August 15, 2007
Purpose : Post-lumbar puncture headache is common complaint. A study of post-diagnostic lumbar puncture headache in children is rare. Various factors that might influence the occurrence of post- diagnostic lumbar puncture headache in children exist. The purpose of this prospective study was to assess the frequency and risk factors for post-diagnostic lumbar puncture headache in children. Methods : From March 2005...
Case Report
Two cases of Chryseobacterium meningosepticum infection in a neonatal intensive care unit
Hye Sun Yoon
Clin Exp Pediatr. 2007;50(7):698-701.   Published online July 15, 2007
We report on two premature infants who developed nosocomial infection caused by Chryseobacterium meningosepticum in a neonatal intensive care unit (NICU). One premature infant developed sepsis, meningitis, and hydrocephalus, and was treated successfully with ciprofloxacin plus trimethoprim-sulfamethoxazole combination therapy for 4 weeks and with a ventriculoperitoneal shunt. The other premature infant, who was in a chronically debilitated state, had infection...
Review Article
Hematopoietic stem cell transplantation : overview for general pediatrician
Tai Ju Hwang
Clin Exp Pediatr. 2007;50(7):613-621.   Published online July 15, 2007
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...
Acute lymphoblastic leukemia in children: past, present and future
Hyoung Jin Kang, Hee Young Shin, Hyo Seop Ahn
Clin Exp Pediatr. 2007;50(7):601-605.   Published online July 15, 2007
The cure rate of acute lymphoblastic leukemia (ALL) in children dramatically improved over past 5 decades from zero to about 80%. The main cause of improvement is owing to the development of chemotherapy by multicenter clinical trial of large study groups with the understanding of leukemia biology. Recently, pediatric ALL protocols were applied to the treatment of adolescent and even...
Case Report
A case of childhood relapsing/remitting multiple sclerosis and interferon β-1b treatment in a Korean patient
Hyun Seok Kim, Won Deok Lee, Jun Hwa Lee, Kyung Lae Cho
Clin Exp Pediatr. 2007;50(6):580-584.   Published online June 15, 2007
Multiple sclerosis (MS) is a demyelinating disorder that affects discrete areas of the CNS, including the optic nerves, in a quite variable relapsing-remitting fashion over a prolonged period of time. Although MS is usually considered to be a disease that affects peoples in early to middle adulthood, children do develop multiple sclerosis. The frequency of MS onset before the age...
Original Article
Polymorphism in the promoter region of the plasminogen activator inhibitor-1 (PAI-1) gene in Kawasaki disease
Mi Young Han
Clin Exp Pediatr. 2007;50(6):570-575.   Published online June 15, 2007
Purpose : To demonstrate genetic background of pathogenesis of Kawasaki disease (KD), I examined the genetic polymorphism of plasminogen activator inhibitor-1 (PAI-1) in KD patients. Methods : PCR-RFLP of PAI- 1 promotor gene was analyzed in 56 KD patients admitted to Kyunghee University Hospital, Gachon Medical School Gil Hospital, and Eulji Hospital from March to August 2000 and 206 normal...
Review Article
Recent advances in histiocytic disorders
Jong Jin Seo
Clin Exp Pediatr. 2007;50(6):524-530.   Published online June 15, 2007
The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades,...
Aplastic anemia
Hack Ki Kim
Clin Exp Pediatr. 2007;50(6):519-523.   Published online June 15, 2007
Aplastic anemia is a rare disease, which is characterized by pancytopenia and hypocellular bone marrow without infiltration of abnormal cells or fibrosis. The incidence in Asia is higher than in the West and new cases are diagnosed at a rate of 5.1 per million pediatric populations per year in Korea. The pathophysiology is understood roughly by defective hematopoiesis, impaired bone...
Hemolytic anemia in pediatrics
Jeong Ok Hah
Clin Exp Pediatr. 2007;50(6):511-518.   Published online June 15, 2007
To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types : RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea,...
Case Report
A case of dapsone syndrome
Yoo Jong Won, Ok Lan Kim, Seung Taek Yu, Young Wook Yoon, Du Young Choi
Clin Exp Pediatr. 2007;50(5):493-496.   Published online May 15, 2007
Diamino-diphenyl-sulfone (Dapsone) is widely used in the treatment of leprosy and a variety of blistering skin diseases. It sometimes has adverse side effects with common usual doses, such as skin, nervous system, gastrointestinal tract, liver, kidney and hematologic toxicity. One of these side effects is a rare but serious hypersensitivity reaction called dapsone syndrome, which occurs several weeks after the...
Original Article
Antithrombin-III as an early prognostic factor in children with acute lung injury
Young Seung Lee, Seonguk Kim, Eun Kyeong Kang, June Dong Park
Clin Exp Pediatr. 2007;50(5):443-448.   Published online May 15, 2007
Purpose : To evaluate the potential prognostic value of the antithrombin-III (AT-III) level in the children with acute lung injury (ALI), we analyzed several early predictive factors of death including AT-III level at the onset of ALI and compared the relative risk of them for mortality. Methods : Over a 18-month period, a total of 198 children were admitted to our...
Review Article
Pathophysiology and management of disorders in water metabolism
Dong Un Kim
Clin Exp Pediatr. 2007;50(5):430-435.   Published online May 15, 2007
Even though we drink and excrete water without recognition, the amount and the composition of body fluid remain constant everyday. Maintenance of a normal osmolality is under the control of water balance which is regulated by vasopressin despite sodium concentration is the dominant determinant of plasma osmolality. The increased plasma osmolality (hypernatremia) can be normalized by the concentration of urine,...
Case Report
A case of adrenocortical adenoma following long-term treatment in a patient with congenital adrenal hyperplasia
Seung Rim Lho, So Hyun Park, Min Ho Jung, Byung Churl Lee
Clin Exp Pediatr. 2007;50(3):302-305.   Published online March 15, 2007
As a result of the widespread use and enhanced quality of high-resolution radiological techniques, a recent report has revealed a relatively high prevalence of small adrenal tumors in patients with untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency. However, there are scarcely any pediatric cases of adrenocortical tumor following long-term treatment in patients suffering with congenital adrenal hyperplasia. We report...
Original Article
Clinical features of congenital muscular torticollis
Ji Eun Jun, Hye Kyeong Ryu, Jae Won Shim, Jung Yeon Shim, Hye Lim Jung, Moon Soo Park, Deok-Soo Kim
Clin Exp Pediatr. 2007;50(3):241-247.   Published online March 15, 2007
Purpose : Congenital muscular torticollis (CMT) is a common and benign congenital disorder of the musculoskeletal system in neonates and infants. The pathophysiology is that the sternocleidomastoid muscle (SCM) is shortened on the involved side by fibrosis, leading to ipsilateral tilt and contralateral rotation of the face and chin. In this study, we investigated the clinical features of CMT, the...
Comparison of growth and neuropsychological function after treatment for hematologic and oncologic diseases in monozygotic twins
Gi Hwan Kim, Hoon Kook, Hee Jo Baek, Dong Kyun Han, Eun Song Song, Young Kook Jo, Ic Sun Choi, Young Ok Kim, Chan Jong Kim, Young Jong Woo, Soo Jin Yang
Clin Exp Pediatr. 2007;50(2):182-189.   Published online February 15, 2007
Purpose : The primary purpose of this study was to evaluate the growth and neuropsychologic function following treatments for pediatric hematologic and oncologic diseases. Healthy monozygotic twins served as ideal controls for comparison to exclude possible confounding factors. Methods : Seven children treated with various hematologic and oncologic diseases were included in the study: acute lymphoblastic leukemia (ALL; n=2), Diamond-Blackfan anemia...
Review Article
Regionalization of neonatal care and neonatal transport system
Jong Beom Sin
Clin Exp Pediatr. 2007;50(1):1-6.   Published online January 15, 2007
In the United States, The concept of designation for hospital facilities that care for newborn infants according to the level of complexity of care provided was first proposed in 1976. The extent of perinatal health care regionalization varies widely from one area to the other. facilities that provide hospital care for newborn are classified into three categories on the basis...
Case Report
Acute hemorrhagic edema in an infant mimicking Henoch-Sch nlein purpura: a case study
Hyang Mo Lee, Eun Young Kang, Han Uk Kim, Pyoung Han Hwang
Clin Exp Pediatr. 2006;49(12):1354-1357.   Published online December 15, 2006
Acute hemorrhagic edema of infancy (AHEI) is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in infants and children younger than 2 years. AHEI is characterized clinically by marked peripheral edema and fever as well as large palpable purpuric and ecchymotic skin lesions in a target-like pattern, mainly on the face, ears and extremities, similar to the skin findings...
Original Article
Second allogeneic hematopoietic stem cell transplantation in children to overcome graft failure or relapse after initial transplant
Dong-Yeon Kim, Do Kyun Kim, Soo Young Kim, Seok Joo Kim, Dong Gyun Han, Hee Jo Baek, Hoon Kook, Tai-Ju Hwang
Clin Exp Pediatr. 2006;49(12):1329-1339.   Published online December 15, 2006
Purpose : Failure of hematopoietic stem cell transplantation(HSCT) may be encountered in practice because of either relapse of the malignancy or dysfunction of the graft. Second HSCT may be the only option for some patients whose initial HSCT failed. Methods : From May, 1991 to December, 2004, 115 HSCTs were performed at the Pediatric Blood & Marrow Transplantation Center, Chonnam National...
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