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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...

Case Report
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...

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...
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...
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....
Two Siblings of Non Salt Losing Congenital Adrenal Hyperplasia due to 21-hydroxylase Deficiency
Seung Cheol Lee, Byung Kyu Suh, Byung Churl Lee
Clin Exp Pediatr. 1995;38(4):574-581.   Published online April 15, 1995
Congenital adrenal hyperplasia in caused by a defect in the biosynthesis of cortisol as a result of deviciency in one of the essential enzymes, most commonly 21-hydroxylase and is an autosomal recessive disease in close genetic linkage with HLA. The common clinical manifestations of congenital adrenal hyperplasia is progressive virilization in both sexes, abnormal external genitalia in the...
Congenital Adrenal Hyperplasia with 21-hydroxylase Deficiencies in Twins
Young Don Kim, Jeong Hwa Choi, Jae Hong Park, Hee Ju Park, Seong Suk Jeon
Clin Exp Pediatr. 1994;37(10):1469-1473.   Published online October 15, 1994
Congenital adrenal hperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic and non-classic 21-hydroxylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous...
Original Article
Growth Pattern and Final Height in Congeital Adrenal Hyperplasia
Ho Seong Kim, Duk Hi Kim
Clin Exp Pediatr. 1993;36(10):1359-1365.   Published online October 15, 1993
Clinical characteristics and growth pattern were evaluated in 42 patients with classical congenital adrenal hyperplasia treated since diagnosis. And final height was evaluated in 16 patients who had reached final adult height with regard to clinical form, degree of hormonal control, and age of initial treatment. The results were as follows; 1) Among 42 patients with classical congenital adrenal hyperplasia, the male...
Study on the Clitoral Size of the Korean Female Newborn.
Jong Lin Rhi, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1988;31(8):1007-1013.   Published online August 31, 1988
The disturbance of sex hormone secretions during the fetal life results in abnormal development of the fetal sex organs including the external genitalia. In the congenital adrenal hyperplasia, especially, the external genitalia is often virilized at birth and the clitomegaly in this disorder can be an important clue for the early diagnosis. For the normal range of the clitoral size of the Korean female...
Congenital adrenal Hyperplasia.
Byoung Hai Ahn, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(6):604-612.   Published online June 30, 1986
Clinical data of twenty-eight children with congenital adrenal hyperplasia who were admitted to the Seoul National University Hospital from June 1979 to July 1984 were analyzed retrospectively. Male to female ratio was 1 : 1.3. The average age at diagnosis was 3.2 years, 1.3 months for eleven salt-losers and all were 3 months or younger, 4.6 years for seventeen nonsaltlosers....
Case Report
Two Cases of The Salt-losing Congenital Adrenal Hyperplasia.
Sung Hun Eun, Cheong Min Lee, Young Chang Tockgo, Hyun Keum Lee
Clin Exp Pediatr. 1985;28(5):499-504.   Published online May 31, 1985
The authers experienced two cases of salt losing congenital adrenal hyperplasia. Case 1. was a 22 day old male infant, who had vomiting and lethargy. Increased urinary 17-KS and decreased 17-OHCS were observed. He was been treated with oral hydrocortisone and florinef, successfully. Case 2. was a 30 day old male infant, who had anorexia, convulsion, and diffusely pigmented skin....
2 Case of Salt-losing Form of Congenital Adrenal Hyperplasia.
S B Jo, J K Yoon, S K Lee, I J Kang
Clin Exp Pediatr. 1984;27(11):1123-1127.   Published online November 30, 1984
We experienced 2 cases of salt losing form of congenital adrenal hyperplasia. Case I was 3days old female neonate who had dehydration and large clitoris. Case H was 22 days old male neonate who had large pigmented penis. Both cases had hyponatremia and hyperkalemia. Their 24 hours urinary 17-ketosterod was also increased. Fluid therapy, hydrocortisone and florinef were tried with improvement. A brief review related literatures...
Adrenogenital Syndrome with Congenital Adrenal Hyperplasia.
Myoung Sung Moon, Kwang Nam Kim, Woo Gill Lee
Clin Exp Pediatr. 1984;27(5):511-515.   Published online May 31, 1984
When the adrenogenital syndrome is associated with congenital adrenal hyperplasia, it is caused by an inborn defect in the biosynthesis of adrenal corticoids. Especially, infant with congenital adrenal hyperplasia caused by a 21-hydroxylation. defect in steroidogenesis often present with salt-losing crisis, hyponatremia and dehydration. Vomiting is prominent and disturbances in cardiac rate and rhythm may occur. Without treatment, collapse and death...
Three Cases of female Pseudohermaphroditism with Congenital Adrenal Hyperplasia.
Yaung Sook Choi, Shin Chul Jun, Hie Ju Park, Chan Yung Kim
Clin Exp Pediatr. 1984;27(3):297-302.   Published online March 31, 1984
We have experienced three cases of female pseudohermaphroditism with congenital adrenal hyperplasia. First case was salt-losing type and the other two cases were non salt-losing type. First patient, 2 months old female infant, was admitted to hospital because of vomiting, diarrhea, dehydration and ambiguous sex. In electrolyte study, she had hyponatremia and hyperkalemia. The other two cases, 2 Years 10 Months...
A Case of Salt-losing Form of Congenital Adrenal Hyperplasia.
J H Kwon, Y A Kim, J J Kim, D H Shin
Clin Exp Pediatr. 1983;26(12):1231-1235.   Published online December 31, 1983
A" 9-month old female was admitted via emergency room in convulsive state with the chief complaints of diarrhea and vomiting. The clitoromegaly and accessory nipples on both anter- ior axillary region were noticed in her early age. Serum electrolytes revealed hyponatremia and hyperkalemia. Urinary 17-ketosteroid level and plasma renin activity were increased. After the immediate treatment of salt-losing crisis, her general condition was recovered and serum...
A Case of Female Pseudohermaphroditism With Congenital Adrenal Hyperplasia.
Hyo Jung Kim, Hye Kyung Lee, Hyang Sook Kim, Soon Jai Lee, Sung Woo Shin
Clin Exp Pediatr. 1983;26(6):589-592.   Published online June 30, 1983
We experienced a case of female pseudohermaphroditism with congenital adrenal hyperplasia. The patient, a 10 day old female infant, was presented characteristic findings such as ambiguous sex and dehydration. The vaginogram revealed normal internal genital organs. The chromosome analysis was 46, XX. The electrolyte study showed hyponatremia and hyperkalemia. The 24 hours urinary 17-ketosteroid and 17-OHCS were 2.5 mg and 0.9 mg...
Two Cases of the Salts Losing Congenital Adrenal Hyperplasia.
Hee Ju Kim, Hyung Ro Moon
Clin Exp Pediatr. 1981;24(11):1092-1099.   Published online November 15, 1981
The authers experienced two cases of salt losing congenital adrenal hyperplasia. Case I was a 100 day old male infant, who had dehydration, failure to thrive, and diffusely pigmented skin. Very low plasma aldosterone and markedly increased urinary 17KS were observed. He had been, treated with daily DOCA injection and oral hydrocortisone, successfully. Case H was a 5 year and...
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