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Original Article
Neonatology (Perinatology)
Diagnostic and prognostic value of proadrenomedullin in neonatal sepsis
Sameh Samir Fahmey, Heba Mostafa, Noha Abd Elhafeez, Heba Hussain
Clin Exp Pediatr. 2018;61(5):156-159.   Published online May 28, 2018
Purpose

Sepsis is a major cause of neonatal morbidity and mortality. Early diagnosis is a major problem because of the lack of specific clinical signs. Therefore, a reliable diagnostic marker is needed to guide the use of antimicrobial agents. The objective of our study was to assess the value of proadrenomedullin (pro-ADM) in establishing the diagnosis and evaluating the prognosis of...

Endocrinology
The effect of low-dose intravenous bisphosphonate treatment on osteoporosis in children with quadriplegic cerebral palsy
Soon Jeong Moon, Young Min An, Soon Ki Kim, Young Se Kwon, Ji Eun Lee
Clin Exp Pediatr. 2017;60(12):403-407.   Published online December 22, 2017
Purpose

Quadriplegic children with cerebral palsy are more susceptible to osteoporosis because of various risk factors that interfere with bone metabolism. Pamidronate is effective for pediatric osteoporosis, but there are no guidelines for optimal dosage or duration of treatment in quadriplegic children with osteoporosis. We aimed to evaluate the efficacy of low-dose pamidronate treatment in these patients.

Methods

Ten quadriplegic patients on antiepileptic...

Review Article
Endocrinology
Management issues of congenital adrenal hyperplasia during the transition from pediatric to adult care
Jin-Ho Choi, Han-Wook Yoo
Clin Exp Pediatr. 2017;60(2):31-37.   Published online February 27, 2017

Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from...

Adrenal and thyroid function in the fetus and preterm infant
Hye Rim Chung
Clin Exp Pediatr. 2014;57(10):425-433.   Published online October 31, 2014

Adrenal and thyroid hormones are essential for the regulation of intrauterine homeostasis, and for the timely differentiation and maturation of fetal organs. These hormones play complex roles during fetal life, and are believed to underlie the cellular communication that coordinates maternal-fetal interactions. They serve to modulate the functional adaptation for extrauterine life during the perinatal period. The pathophysiology of systemic...

Case Report
A Korean boy with atypical X-linked adrenoleukodystrophy confirmed by an unpublished mutation of ABCD1
Hye Jeong Jwa, Keon Su Lee, Gu Hwan Kim, Han Wook Yoo, Han Hyuk Lim
Clin Exp Pediatr. 2014;57(9):416-419.   Published online September 30, 2014

X-linked adrenoleukodystrophy (X-ALD) is a rare peroxisomal disorder, that is rapidly progressive, neurodegenerative, and recessive, and characteristically primary affects the central nervous system white matter and the adrenal cortex. X-ALD is diagnosed basaed on clinical, radiological, and serological parameters, including elevated plasma levels of very long chain fatty acids (VLCFA), such as C24:0 and C26:0, and high C24:0/C22:0 and C26:0/C22:0...

Testicular adrenal rest tumors in a patient with untreated congenital adrenal hyperplasia
Hye Young Jin, Jin Ho Choi, Gu Hwan Kim, Chung Sik Lee, Han Wook Yoo
Clin Exp Pediatr. 2011;54(3):137-140.   Published online March 31, 2011

Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of...

Review Article
Growth and sex differentiation of children born small for gestational age
Se Young Kim
Clin Exp Pediatr. 2009;52(2):142-151.   Published online February 15, 2009
The incidence of small for gestational age (SGA) births is frequent, accounting for 2.3% to 8% of all live births. Several childhood and adult diseases are related to early postnatal growth and birth size, and 10% of children born SGA may have a short stature throughout postnatal life. Additionally, they may have abnormal growth hormone (GH)-insulin like growth factor axis,...
Case Report
A case of testicular adrenal rest tumor in a male child with congenital adrenal hyperplasia
Joo Hwa Kim, Kyong Ah Yun, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2008;51(9):1018-1022.   Published online September 15, 2008
Testicular adrenal rest tumors are a well-known complication in male patients with congenital adrenal hyperplasia. Corticosteroid suppressive therapy usually results in the regression of these tumors. We describe a patient with 21-hydroxylase deficiency who developed bilateral testicular masses. Despite steroid suppressive therapy, the tumors did not regress and hormonal control was poor. Consequently, bilateral partial orchiectomies were performed.
Original Article
Interpretation of screening for congenital adrenal hyperplasia in preterm infants
Hye Rim Chung, Choong Ho Shin, Sei Won Yang, Kyong Ah Yun, Young Ah Lee, So Eun Park, Chang Won Choi, Byung Il Kim, Jung Hwan Choi, Junghan Song
Clin Exp Pediatr. 2008;51(6):616-621.   Published online June 15, 2008
Purpose : This study was undertaken to identify factors that influence 17-OHP levels in preterm infants and to suggest a reasonable follow-up schedule of screening for congenital adrenal hyperplasia (CAH) in preterm infants. Methods : The 17-OHP concentrations in filter paper blood spots of 427 preterm infants were obtained. The effects of gestational age (GA), systemic diseases, and antenatal dexamethasone...
Analysis of the association between bronchial hyperresponsiveness and genetic polymorphism of β2-adrenoceptor in adolescents with long-term asthma remission
Hee Kang, Young Yull Koh
Clin Exp Pediatr. 2007;50(6):556-564.   Published online June 15, 2007
Purpose : We hypothesized that the persisting bronchial hyperresponsiveness (BHR) of adolescents with asthma remission may be controlled mainly by genetic factors, and the BHR of symptomatic asthma by airway inflammation. β2-adrenoceptor gene is considered to be a candidate gene in the development of BHR. Thus, β2-adrenoceptor gene polymorphism may be associated with the BHR of adolescents with asthma remission,...
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...
Review Article
Evaluation of function and disorders of the adrenal gland in neonates
Il Tae Hwang
Clin Exp Pediatr. 2007;50(3):236-240.   Published online March 15, 2007
Majority of sick full term newborns have adequate adrenal cortical function in response to stress. Acutely ill neonates with a basal cortisol level less than 15 g/dL (414 nmol/L) suggest adrenal insufficiency and require function testing of adrenal function. In premature infant, immaturity of hypothalamic-pituitary adrenal axis (HPA axis), may limit the ability to increase cortisol production in response...
Original Article
The Preventive Effect of Dexrazoxane and Pentoxifylline on Adriamycin Induced Cardiomyopathy
Ling Zhu, Eun-Jung Bae, Il-Soo Ha, Jung-Wook Seo, Chung-Il Noh, Jung-Yun Choi, Yong-Soo Yun
Clin Exp Pediatr. 2005;48(12):1378-1384.   Published online December 15, 2005
Purpose : We hypothesized dexrazoxane(DXR) and pentoxifylline(PTX) may prevent myocardial damage in adriamycin(ADR)-induced cardiomyopathic rat model. We also investigated their effects on the myocardial apoptosis and fibrosis in ADR induced cardiomyopathy. Methods : The six-week old female Spregue-Dawley rats were divided into control group(CNT, n= 4), ADR group(n=6), ADR+DXR group(DXR, n=5), ADR+PTX group(PTX, n=6), ADR+DXR+PTX group(DXPT, n=5). ADR(5 mg/week, twice) was...
Lorenzo's Oil Treatment in X-linked Adrenoleukodystrophy
Hyo Jung Kim, Eun Young Kim, Young Jong Woo, Hoon Kook
Clin Exp Pediatr. 2005;48(11):1232-1238.   Published online November 15, 2005

Purpose: Though more than 15 years have passed after introduction of Lorenzo's oil therapy in X- linked adrenoleukodystrophy(ALD), its efficacy is still fully not known. In patients who already have neurologic symptoms, most reports indicate that the neurologic disability continues to increase. We evaluated its efficacy in X-linked ALD patients. Methods: Four patients who were treated with Lorenzo's oil for at...
Gene Expression of Metalloproteinases, Tissue Inhibitors of Metalloproteinases and Cytokines in Adriamycin-induced Cardiomyopathy
Young Mi Hong
Clin Exp Pediatr. 2005;48(2):197-203.   Published online February 15, 2005
ity has been reported to occur in both clinical and experimental forms of dilated cardiomyopathy. There was no report about MMP in adriamycin(ADR)-induced cardiomyopathy. The purpose of this study was to investigate gene expression of MMP and tissue inhibitor of metalloproteinases(TIMP) in ADR-induced cardiomyopathy and clarify the relationship between MMP and cytokines. Methods : Male Sprague-Dawley rats were divided into two...
A Clinical Study and Sonographic Findings of Neonatal Adrenal Hemorrhage
Ok Yeon Cho, Kyoung Eun Lee, Eun Jung Shim, Do Jun Cho, Dug Ha Kim, Ki Sik Min, Ki Yang Yoo, Kwan Sub Lee
Clin Exp Pediatr. 2004;47(5):535-542.   Published online May 15, 2004
Purpose : This study was performed to present out experience of neonatal adrenal hemorrhage (NAH) and to help diagnosis and management of NAH. Methods : We conducted a retrospective study on 14 neonates diagnosed as NAH from January 1993 to August 2002 at Hallym Medical Center. Their clinical manifestations, risk factors, sonographic findings and progression of NAH were evaluated using...
Case Report
A Case of True Precocious Puberty Complicating Congenital Adrenal Hyperplasia
Su Jin Kim, Ju Suk Lee, Su Yung Kim
Clin Exp Pediatr. 2003;46(4):400-403.   Published online April 15, 2003
Congenital adrenal hyperplasia(CAH) is a recognized cause of precocious pseudopuberty. Some children with CAH also develop true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis. We review a case of CAH who eventually developed central precocious puberty nine months after initial treatment with corticosteroid. A 3-year-old boy visited complaining of rapid growth, a large penis and frequent penile erections....
Original Article
Assessment of Left Ventricular Function with Echocardiography in Patients Treated with Adriamycin : A Load-Independent Index of Myocardial Contractility and Comparisons between Rest and Exercise
Pyoung Soo Park, Hye Young Park, Hae Yong Lee
Clin Exp Pediatr. 2002;45(2):214-222.   Published online February 15, 2002
Purpose : The aim of this study was to evaluate myocardial injury in children treated with adriamycin by echocardiography, which is non-invasive and safe measurement for children. Methods : Left ventricular dimensions, wall stress, and contractile function were determined by echocardiographic methods in 17 patient recepients with adriamycin chemotherapy at rest(group 1) and during stress(group 2). Twenty age-matched normal subjects were...
Case Report
A Case of Primitive Neuroectodermal Tumor of the Adrenal Gland
Sun Pil Jung, Chong Gwon Oh, In Seok Lim, Dong Keun Lee, Byoung Hoon Yoo
Clin Exp Pediatr. 2001;44(12):1459-1462.   Published online December 15, 2001
Primitive neuroectodermal tumors(PNETs) are a group of small round cell tumors that may arise in the central or peripheral nervous system. In the extracranial location, these neoplasms may occur anywhere and at any age group, but most likely in the bone and soft tissue of children and young adults. The incidence of adrenal gland involvement is very rare. This tumor...
A Case of Encephalopathy Presented with Motor Aphasia and Quadriplegia Following Intrathecal Methotrexate
Seon Woo Lee, Eun Hye Park, Hyun-Sang Cho, Jae Kook Cha, Hae Ran Lee
Clin Exp Pediatr. 2001;44(9):1066-1069.   Published online September 15, 2001
Intrathecal administration of methotrexate(IT-MTX) has constituted the standard approach to prophylaxis and treatment of central nevous system(CNS) leukemia. We experienced a quadriplegia and motor aphasia in a 14-year-old boy following repeated IT-MTX for the prophylaxis of meningeal leukemia. He was diagnosed as ALL without CNS involvement and treated by CCG- 1882 protocol. IT-MTX was administered for CNS prophylaxis. The patient began complaining of urinary incontinence,...
Original Article
A Case of Xp21 Contiguous Gene Deletion Syndrome with Hyperglycerolemia, Congenital Adrenal Hypoplasia and Duchenne Muscular Dystrophy
Dae-Won Shin, Jun Huh, Hong-Jin Lee, Won-Ill Park, Kyung-Ja Lee, Yoon-Sook Shin, D.R. Sjarif, B.T. Poll-The
Clin Exp Pediatr. 2001;44(1):83-88.   Published online January 15, 2001
On Xp21 region several genes such as adrenal hypoplasia congenita(AHC) gene, glycerol kinase (GK) gene and Duchenne muscular dystrophy(DMD) gene are located contiguously. If there is a long deletion in that region, various combination of genetic defect can be occurred from one kind of genetic defect to all three kinds of genetic defect simultaneously. In case of more than two...
Serum Lipid Levels and Fatty Acid Metabolism in the Rat with Adriamycin Induced Cardiomyopathy
Young Mi Hong, Hye Ran Yoon
Clin Exp Pediatr. 2000;43(11):1480-1487.   Published online November 15, 2000
Purpose : Adriamycin induced cardiomyopathy is irreversible and may procede to clinical congestive heart failure. Recently, it has been suggested that adriamycin may exert cardiomyopathy due to inhibition of transport across mitochondrial membranes mediated by the carnitine palmitoyltransferase system(CPT I). The effect of adriamycin on fatty acid metabolism according to cumulative dose was not clarified. The purpose of this study...
Protective Effect of Probucol against Adriamycin-Induced Apoptosis in Cultured Rat Cardiac Myocytes
Sun-Ju Lee, Dong-Seok Lee, Yong-Wook Jung
Clin Exp Pediatr. 2000;43(6):746-754.   Published online June 15, 2000
Purpose : In adriamycin(ADR)-induced cardiomyopathy, several different mechanisms are suggested. However, little information is available regarding the role of apoptosis. In the present study, we examined the induction of apoptosis on ADR treatment and anti-apoptotic effects of probucol, a lipid-lowering drug, and we also studied the changes of bcl-2 expression in order to see the molecular mechanisms underlying the effect...
The Use of Quadruple Blood Bags Transfusion of Low Birth Weight Neonates
Sang Hoon Lee, Jean Yoon, Yang Park, Sung Kyu Lee, Hee Chung Kim, Woo Sup Shim
Clin Exp Pediatr. 1999;42(8):1122-1129.   Published online August 15, 1999
Purpose : Low-birth-weight(LBW) neonates often need multiple transfusions during their neonatal period. The routine use of the 500ml mono-bag system in Korea, even in neonatal transfusions, results in a great waste of blood and exposes the neonate to multiple blood donors. To reduce the waste of blood and the number of exposures, we used quadruple blood bags in the transfusion...
The Role of Apoptosis in Adriamycin Induced Cardiotoxicity and Preventive Effect of L-carnitine in Rat
Young Mi Hong, Seung Sook Kim
Clin Exp Pediatr. 1998;41(6):760-768.   Published online June 15, 1998
Purpose : Adriamycin is very important in cancer chemotherapy. Unfortunately, the drug shows cumulative dose dependent cardiotoxicity. The precise mechanism of this remains unclear. It has been shown that adriamycin induces programmed cell death or apoptosis in many tissues. But the role of apoptosis in adriamycin induced cardiotoxicity is uncertain. Methods : Sprague-Dawley rats were divided into four groups of ten animals each. All...
Case Report
Two Cases of Wolman's in Siblings
Joon Sung Kim, Tai Huyng Cho, Hoon Kook, Seung Hee Choi, Young Youn Choi, Chang Soo Park, Heoung Keun Kang, Dong Kyu Jin, Tai Ju Hwang
Clin Exp Pediatr. 1998;41(4):529-537.   Published online April 15, 1998
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...
Original Article
Clinical Features of Adrenocortical Neoplasms
Jung-Eun Lee, So-Chung Chung, Duk-Hi Kim, Ho-Sung Kim
Clin Exp Pediatr. 1997;40(5):680-689.   Published online May 15, 1997
Purpose : Adrenocortical tumors are uncommon in children and comprise only a small proportion of primary adrenal neoplasms. The biologic behavior of these tumors may be very difficult to predict, and their rarity has hindered identification of clinical characteristics. Patients with functioning tumors have excessive steroid hormone production, and the clinical manifestation depends on the predominant hormone produced. The detection of...
Case Report
A CaseofAdrenal Aplasi
Yoon-Ok Kim, Myoung-Bum Choi, Yun-Kyeong Cho, Jae-Yong Lim, Yang-Suk Jung, Suong-Hwan Kim, Hyang-Ok Woo, Hee-Shang Youn
Clin Exp Pediatr. 1996;39(11):1627-1630.   Published online November 15, 1996
Major known causes of neonatal adrenal insufficiency are prolonged maternalsteroid use, adrenal hemorrhage from the perinatal stress and adrenogenital syndrome. Theoretically adrenal aplasia might be a cause of neonatal adrenal insufficiency but it has not been reported yet. We had experienced a case of adrenal aplasia in a 5 day-old male neonate whose chief complaint was hyperpigmentation. His laboratory findings were compatible...
A Case of Congenital Lipoid Adrenal Hyperplasia
Seo-Jeong Kim, Joo-Sik Choi, Kyu-Hyung Lee, Dong-Kyu Jin
Clin Exp Pediatr. 1996;39(4):567-571.   Published online April 15, 1996
Congenital lipoid adrenal hyperplasia is the most severe form of CAH, leading to impaired production of all steroid hormones including glucocorticoids, mineralocorticoid, and sex steroids. The affected individuals are all phenotypically female with a severe salt-losing syndrome that is fatal if steroid replacement is not begun immediately after birth. The lesion of this disorder has been suggested to be in...
Two Cases of Neonatal Adrenal Hemorrhage
Kyeong Hye Jeong, Sung Suk Jeon, Ok Sung Jeong, Son Sang Seo, Jung Mee Kweon
Clin Exp Pediatr. 1995;38(10):1411-1416.   Published online October 15, 1995
Neonatal adrenal hemorrhage is not uncommon due to its relative large size and vascularity, thus making it vulnerable to traumatic and asphyxial injuries. It뭩 etiology is unknown, but postulated etiologies include maternal diabetes, obstetric trauma, asphyxia, thrombocytopenia and coagulation defect. Clinical manifestations ate those of a paplable mass in the affected flank, anemia and jaundice. Hypoglycemia, hypotension and hypovolemia may...
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