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Case Report
A case of Bartter syndrome type I with atypical presentations
Eun Hye Lee, Ju Sun Heo, Hyun Kyung Lee, Kyung Hee Han, Hee Gyung Kang, Il Soo Ha, Yong Choi, Hae Il Cheong
Clin Exp Pediatr. 2010;53(8):809-813.   Published online August 31, 2010

Bartter syndrome (BS) is an autosomal recessively inherited rare renal tubular disorder characterized by hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal to low blood pressure due to a renal loss of sodium. Genetically, BS is classified into 5 subtypes according to the underlying genetic defects, and BS is clinically categorized into antenatal BS and classical BS according to onset...

Original Article
Clinical, endocrinological and radiological courses in patients who was initially diagnosed as idiopathic central diabetes insipidus
Seung Joon Chung, Seong Yong Lee, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2007;50(11):1110-1115.   Published online November 15, 2007
Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and...
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 Congenital Nephrogenic Diabetes Insipidus Confirmed by Gene Analysis
Eun Young Cho, Jin Hee Oh, Dae Kyun Koh
Clin Exp Pediatr. 2005;48(6):669-674.   Published online June 15, 2005
Nephrogenic diabetes insipidus (NDI) is a disorder in which the secretion of antidiuretic hormone is normal, but the response of the renal collecting tubules to vasopressin is impaired. Compared with acquired NDI (a-NDI), which is secondary to chronic bilateral incomplete urinary tract obstruction with hydronephrosis, congenital NDI (c-NDI) is a very rare heritable disorder that usually follows the X- linked...
A Case of Teratocarcinoma with Central Diabetes Insipidus
Jong Hoon Kim, In Seok Lim, Eung Sang Choi, Byoung Hoon Yoo
Clin Exp Pediatr. 2004;47(4):453-457.   Published online April 15, 2004
Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in...
A Case of Congenital Partial Nephrogenic Diabetes Insipidus
Eun Ha Mo, In Hye Nam, Min Ja Chung, Jae Hong Yu
Clin Exp Pediatr. 2002;45(7):902-905.   Published online July 15, 2002
The most common form of genetic nephrogenic diabetes insipidus(NDI), a rare inherited disorder, is congenital and is transmitted in an X-linked recessive mode. It is refractory to the antidiuretic effect of normal to moderately increased levels of plasma arginine vasopressin(AVP) but, in some cases, may respond to high levels of the hormone or its analogue, deamino-D-arginine vasopressin(DDAVP). X-linked congenital NDI...
Original Article
Central Diabetes Insipidus in Children Related to Craniotomy for a Brain Tumor
Seung Mi Song, Eun Jung Park, Jung Sim Kim, Hong Hoe Koo, Mun Hyang Lee, Hyung Jin Shin, Dong Kyu Jin
Clin Exp Pediatr. 1999;42(12):1702-1710.   Published online December 15, 1999
Purpose : Diabetes insipidus(DI) has been known to be a relatively common complication after craniotomy. We have investigated the incidence and clinical course of DI in children related to craniotomy for a brain tumor and determined the risk factors of postoperative DI. Methods : Sixty-two pediatric patients, who have undergone craniotomy for a brain tumor(including stereotactic biopsy) from February 1995 through...
Case Report
A case of Central Diabetes Insipidus Associated with Brachycephaly
Woo Sik Kang, Mee Kyung Namgoong, Jae Seung Yang, Baek Keun Lim, Jong Soo Kim
Clin Exp Pediatr. 1994;37(2):282-287.   Published online February 15, 1994
Brachyecphaly is a kind of craniosynostosis. Because of premature closure of the coronal suture, the skull is shorter in the anteroposterior diameter but is widened with a high vault and the occiput and borehead are flattened. Diabetes insipidus had been reported in oxycephaly. We have experienced a case of central diabetes insipidus associated with brachcephaly. A brief review of related literatures...
A Case of Holoprosencephaly, Alobar Type
Kyu Sun CHoi, Kang Seo Park, Young Tack Jang, Wan Seob Kim
Clin Exp Pediatr. 1992;35(3):390-394.   Published online March 15, 1992
A case of Holoprosencephaly, alobar type , was presented here and 13-month follow-up has been made. He had dyspnea, polikilothermia, frequent atypical seizures, hypernatremia & pitressinresponsive Diabetes insipidus as clinical manifeststions. Chromosome studies showed normal male karyotype (46XY). The diagnosis was confirmed by brain CT scan. A brief review of the related literature was made.
Original Article
A case of nephrogenic diabetes insipidus due to vesicoureteral reflux.
Yong Bae Suh, Jong Yul Jeon, Sung Ho Cha, Byoung Soo Cho, Chang Il Ahn
Clin Exp Pediatr. 1991;34(9):1299-1304.   Published online September 30, 1991
Nephrogenic diabetes insipidus is a disorder characterized by polydipsia and polyuria resulting from failure of the renal tubules and collecting ducts to respond to antidiuretic hormone despite elevated blood levels of antidiuretic hormone. We experienced a case of nephrogenic diabetes insipidus in 13 months-old girl probably secondary to vesicoureteral reflux grade IV. The clinical manifestations were extreme thirst, polydipsia and polyuria. Family history was nonspecific and...
A Case of Diabetes Insipidus in a Child with Eosinophilic Granuloma of Pituitary Stalk.
Dong Hyung Kim, Eun Kyung Won, Jung Sik Min, Chang Hee Choi
Clin Exp Pediatr. 1990;33(10):1454-1460.   Published online October 31, 1990
A 12 year old girl suffering visual disturbance for 4 months, and polydipsia and polyuria for last 20 days revealed an ovoid homogeneous mass on enhanced sella turcica coronal CT scan and result of water deprivation test was compatible with that of diabetes insipidus. Subtotoal rescetion of the brownish soft mass (0.5 x 0.7 cm) of pituitary stalk was performed neurosurgically, and histologically the...
Clinical Study of Diabetes Insipidus.
Jung Bae Lee, Duk Hi Kim
Clin Exp Pediatr. 1987;30(8):882-890.   Published online August 31, 1987
From Aug. 1979 to June 1986 we reviewed 27 patients with diabetes insipidus to observe the clinical features, laboratory findings and treatment of diabetes insipidus. The results were as follows: 1) Central diabetes insipidus were 22 cases (81.5%) and nephrogenic diabetes insipidus were 5 cases (18.5%). Etiologic factors in the central diabetes insipidus included idiopathic (54.6%), craniopharyn- gioma (18.2%), germinoma (13.6%), post-encephalitis (9.1%) and Histiocytosis X...
Diagnosis and Treatment of Diabetes Insipidus in Childhood.
Yong Hoom Park, Jin Sang Son, Jeong Ok Hah
Clin Exp Pediatr. 1987;30(7):749-758.   Published online July 31, 1987
In eight cases of diabetes insipidus who were diagnosed at the pediatric department of Yeungnam University Hospital from July 1985 till May 1986, the levels of plaana AVP were measured by radioimmunoassay and the effects of various treatment were studied. In central diabetes insipidus patients basal plasma AVP level was 2.92 ±2.55 pg/ml which was significantly lower than the level of control group, 8.25...
Case Report
Three Cases of Diebetes Insipidus.
Eue Bon Koo, Hak Jun Go, Tai Gyu Whang, Soon Yong Lee
Clin Exp Pediatr. 1986;29(1):88-94.   Published online January 31, 1986
We reported three cases of Diabetes Insipidus which were characterized by polyuria and polydipsia. One case was idiopathic and two cases were associated with tuberculous meningitis and pinealoma respectively. Water restriction test and hypertonic saline infusion test revealed little change in urine volume and specific gravity but good response to intramuscular injection of pitressin in all of the three cases....
Three Cases of Nephrogenic Diabetes Insipitus.
Hey Sun Lee, Byoung Hai Ahn, Jong Jin Seo, Hyung Ro Moon
Clin Exp Pediatr. 1984;27(9):912-917.   Published online September 30, 1984
From July 1981 to Feb. 1983, we experienced three cases of nephrogenic diabetes insipidus in which the initial diagnostic clues were repeated episodes of unexplained fever in infancy and which were confirmed by water deprivation and pitressin tests and clinical responses to administration of hydrochlorthiazide. So we report this with some review of literatures.
A Case of Histiocytosis X(Hand-Schuller-Christian Syndrome).
Yeong Bong Park, Jin Heon Kim, Jong Myeon Hong, Chang Soo Ra
Clin Exp Pediatr. 1983;26(12):1209-1214.   Published online December 31, 1983
A case of histiocytosis X (Hand-Schuller-Christian syndrome) in a 3 year old girl is presented. The patients has the triad of exophthalmos, diabetes insipidus and skeletal lesions, plus hepatomegaly, wide spread pulmonary infiltration and growth retardation. The patient was treated with chlorambucil, prednisone, radiation therapy and chlorpropamide therapy for diabetes insipidus with good response. Similar cases reported in literature are briefly...
Nephrogenic Diabetes Insipidus occurred in 2 Brother.
Jin Heon Kim, Cheol Won Park, Bong Soo Lee, Chang Soo Ra
Clin Exp Pediatr. 1983;26(9):916-921.   Published online September 30, 1983
Nephrogenic diabetes insipidus is a congenital hereditary disorders in which the kidney do not respond to vasopressin, and the disease occurs principally in males and is probably inherited by Xlinked recessive mode. We experienced two cases of nephrogenic diabetes insipidus occurred in brothers. The clinical manifestations were extreme thirst and frequent urination of large volume of dilute urine, approximately 5,500ml per day with specific gravity...
A Case of Congenital Nephrogenic Diabetes Insipidus.
Jae Sook Kim, Sook Hee Kong, Choong Hee Kim
Clin Exp Pediatr. 1983;26(9):909-915.   Published online September 30, 1983
This 18/12-year old female baby was diagnosed as primary nephrogenic diabetes insipidus， whose main complaint was polydypsia and polyuria from birth, subsequently she developed failure to thrive. Family history was nonspecific. Laboratory findings revealed failure of concentrates urine with water restriction test and pitressin test. And so renal hypoplasia on the right side was revealed on retrograde pyelography and renal scan. She was...
A Case of Hand Schuller Christian Disease.
Yeon Saeng Lee, Soo Young Kim, So Kyung Park, Dong Hak Shin
Clin Exp Pediatr. 1983;26(4):392-396.   Published online April 30, 1983
Hand-Schuller-Christian disease is reactive granulomatous lesion with histiocytic proliferation, the cause of which is unknown. Recently, we experienced a case in a 3 years old female with polydipsia and polyuria. We established the diagnosis with clinical features, radiologic examination and bone biopsy- Histiologic examination of frontal bone showed mild to moderate infiltration of eosinophils， lymphocytes, plasma cells and large histioytes and fibrosis. Similiar cases reported in...
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