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Original Article
A Case of Potter Syndrome Syndrome Accompanied with Partial Agenesis of Corpus Callosum.
Soon Sup Jang, Byung Chun Suh, Kyoo Hwan Rhee, Soo Jee Moon, Keun Soo Lee, Byung Tae Park, Eun Kyung Hong
Clin Exp Pediatr. 1987;30(11):1287-1293.   Published online November 30, 1987
We have experienced a case of Potter syndrome recently. He was a newborn infant who suffered from severe respiratory distress soon after birth. His face was characterized by facies renalies and chest X-ray film revealed bilateral pneumothorax. Anuria since birth and findings of abdominal ultrasonography strongly suggested bilateral renal agenesis. Severe respiratory acidosis was progressed in spite of proper management and died at 11...
A Case of Potter Syndrome with Bilateral Polycystic Kidneys.
Chun Hwa Lee, Jung Hwan Choi, Yong Choi, Chong Ku Yun, Yeon Lim Suh, Je G Chi, Son Moon Shin
Clin Exp Pediatr. 1987;30(11):1282-1286.   Published online November 30, 1987
In 1946, Potter described a series of 20 cases of infants in whom bilateral absence of the kidneys was associated with hypoplasia of the lungs and characteristic faces. Afterwards it has been known that any condition that causes significant reduction in urine formation or excretion will result in oligohydramnios accounts for many of the nonrenal features of Potter syndorme. We report a case...
A Case of Mucocutaneous Lymph Node Syndrome Complicated by Aseptic Meningitis, Hydrops of the Gallbladeder and Dilatation of the Coronary Arteries.
Ho Jin Lee, Sung Sook Cho, Hyung Sik Moon, Hong Kue Lee, Soon Il Lee, Young Seok Lee
Clin Exp Pediatr. 1987;30(11):1259-1264.   Published online November 30, 1987
We experienced 1 case of mucocutaneous lymph node syndrome complicated by aseptic meningitis, hydrops of the gallbladder and dilatation of the coronary arteries in 2 year 8 month old female infant. Her clinical synptoms, laboratory data and clinical courses were presented with brief review of the literatures. This baby admitted because of high fever and vomiting for 6 days. She had injection of...
Factors Influencing the Prognosis of Reye Syndrome.
Chang Ho Hong, Heung Dong Kim, Pyung Kil Kim
Clin Exp Pediatr. 1987;30(11):1252-1258.   Published online November 30, 1987
While the etiology of Reye syndrome is still unknown, wide variations in both the clinical spectrum and the therapeutic outcome of patients have been noted. We reviewed the cases of 42 Reye syndrome patients who were admitted to Young Dong Severance Hospital during the period from April, 1983 to July, 1986 and analyzed factors influencing the prognosis of Reye syndrome. The patients were divided...
A Clinical Studey on Reye Syndrome.
Myung Ho Oh, Hung Sub Choi, Chang Hwi Kim, Sang Jhoo Lee
Clin Exp Pediatr. 1987;30(11):1243-1251.   Published online November 30, 1987
We took clinical study for 47 cases of Reye syndrome at S.C.H. University Hospital during last 8 years from Jan:, 1979 to Dec., 1986. The results obtained were as follows; 1) There was no significant difference in annual incidence and the seasonal incidence was highest in sumer and autumn. 2) The sex ratio of male to female was...
Plasma Renin Acivity in Children with Acute Glomerulonephritis and Nephrotic Syndrome.
Oh Kyun Kwon, Young Hun Chung
Clin Exp Pediatr. 1987;30(11):1213-1220.   Published online November 30, 1987
This is a study on plasma renin acitivity (PRA) which was obtanined from the patients with acute glomerulonephritis or nephrotic syndrome and normotensive control group admitted in Chungnam National University Hospital from Oct. 1985 to Aug. 1986 to confirm any differences of PRA between groups and to establish their influencing factors. The results were as follows; 1) PRA of noimal children in preschool age (3.49 ±0.98...
Case Report
A Case of Beckwith-Wiedemann Syndrome.
Hyo Sook Hong, Hong Chul Lee, Oh Kyung Lee, Myung Ho Lee
Clin Exp Pediatr. 1987;30(10):1166-1169.   Published online October 31, 1987
Beckwith-Wiedemann syndrome constitutes a clinicopathologic entity characterized macroglossia, omphalocele or umblical hernia, visceromegaly, postnatal gigantism, microcephaly, Nevus flammeus, ear lobe grooves, hemihypertrophy and other abnormalities. The anomalies of the syndrome are facultative and not obligatory. We experience one case of Beck with-Wiedemann syndrome. This 1-day-aged female neonate showed macroglossia, gigantism, omphalocele and hypoglycemia. Review of literature was done briefly.
Case of Peripheral Neuroepithelioma with Ectopic ACTH Syndrome.
O Y Lee, K S Lee, H I Chung, H J Park, M J Shin, K J Cho, J G Chi
Clin Exp Pediatr. 1987;30(10):1152-1160.   Published online October 31, 1987
Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. The authors observed the course of a 1-year-old Korean female infant who presented with an enlarging mass in the right hand and manifested hepatic and pulmonary metastases at the time of diagnosis. She also presented with characteristic Cushingoid features, such as moon face, buffalo hump, and truncal obesity. Laboratory...
Original Article
A Clinical Studies in Patients with Turner's Syndrome.
Chang Hyun Yang, Duk Hi Kim, Kir Young KIm, Young Ho Yang
Clin Exp Pediatr. 1987;30(10):1143-1151.   Published online October 31, 1987
This study is systematical cytogenetic and clinico-hormonal analysis of 42 cases who were diagnosed as Turner’s syndrome at the Yonsei Medical Center from Jan. 1971 to Dec. 1985. The careful history taking, physical examination, cytogenetic studies, radiologic and hormonal evalua- tion, and GnRH stimulation test were performed. The results were as follows. 1) The patients ranged in age from 14 months to 30 years at the...
Case Report
A Case of Alport's Syndrome.
Jung Bae Lee, Jong Kyun Lee, Pyung Kil Kim, Hyeon Joo Jeong, In Jun Choi
Clin Exp Pediatr. 1987;30(9):1040-1048.   Published online September 30, 1987
We have experienced a case of Alporfs syndrome. The patient was suspicious to make the dignosis of Alporfs syndrome at first examination when he was five years and seven months old. At that time diagnostic work-up were included audiogram and kidney biopsy. His family history was more impressive. His elder brother has died with undetermined cause of nephrotic syndrome one month ago and his...
Original Article
Syndrome of Inappropriate Secretion of Antidiuretic Hormone(SIADH) in Acute Respiratory Diseases.
Young Jee KIm, Young Yull Koh, Jeong Kee Seo, Hung Ko Moon
Clin Exp Pediatr. 1987;30(9):1021-1028.   Published online September 30, 1987
The SIADH incidence and relevant factors in acute respiratory diseases were evaluated in 461 children with over 28 days old, hospitalized for bronchiolitis, viral pneumonia, bacterial pneumonia, mycoplasma pneumonia and asthma, without other causes of electrolyte imbalance. The overall incidence of SIADH in acute respiratory diseases was 11.93%. The incidence in mycoplasma pneumonia (19.05%) was highest and incidence in bacterial pneumonia (17.42%), viral pneumonia (9.17%),...
A Case of Bartter's Syndrome.
Jeong A Hwang, Jung Hye Choi, Jae Seung Lee
Clin Exp Pediatr. 1987;30(8):922-927.   Published online August 31, 1987
Bartter’s syndrome is rare disease which is characterized by hypokalemia, hypochloremic meta- bolic alkalosis, hyperreninemic hyperaldosteronism, hyporesponsiveness to pressors, and juxtag- lomeurlar apparatus hyperplasia. We had experienced a case of Bartter’s syndrome in 4 months old male infant. The diagnosis was made by clinical, biochemical and pathological studies. Literatures were reviewed briefly.
Two Cases of Congenital Asplenia.
Man Chul Ha, Young Tak Lim, Hi Joo Chun, Hi Ju Park, Chan Yung Kim
Clin Exp Pediatr. 1987;30(8):916-921.   Published online August 31, 1987
Congenital absence of spleen is often associated with malpositions and malformations of other organ system such as congenital heart disease partial situs inversus, symmetric lobulation of the liver and the lungs. This is a rare syndrome but has been reported in increasing frequency in recent years. The pure congenital asplenia is a very rare disease. We experienced two cases of congenital asplenia. One is...
Two Cases of Peutz-Jeghers Syndrome.
Joo Saeng Moon, Sung Ki Jin, Doo Sung Moon, Kyung Sook Cho, Jong Dase Cho
Clin Exp Pediatr. 1987;30(8):907-915.   Published online August 31, 1987
Peutz-Jeghers syndrome is characterized by hamartomtous polyps of the gastrointestinal tract (stomach, small bowel, and colon) and mucocutaneous pigmentation (lips, oral mucosa, fingers, forearms, toes and umbilical area). About 50% of the reported cases have a positive family history of autosomal dominant mode of inheritacnce, whereas the remaining 50% are sporadic. We have experienced two case of Peutz-Jeghers syndrome with no definite family history....
Case Report
A Case of Potter Syndrome Associated with Renal Dysplasia.
Young Woo Park, Joon Ho Kim, Eun Ryoung Kim, Soon Jae Lee, Young Chul Moon, Young Jin Kim
Clin Exp Pediatr. 1987;30(7):812-817.   Published online July 31, 1987
Potter syndrome is relatively rare congenital defect in with agenesis or dysplasia of the kidney is associated with oligohydramnios, pulmonary hypoplasia, characteristic facial features and other abnormalities. The etiology is unknown. But various theories are reviewed and the suggestion is made that the cause of Potter syndrome in newborns may well be multifactorial. Among the various factors, we agree to the opinion that oligohydramnios...
A Case of Hallermann-Streiff Syndrome.
Jin Sang Son, Ho Youn Hwang, Han Ku Moon, Jeong Ok Hah
Clin Exp Pediatr. 1987;30(6):691-694.   Published online June 30, 1987
We experienced a case of Hallermann-Streiff syndrome in a 3 month old male with hepatitis B characterized by dyscephaly with bird face, proportionate nanism, hypotrichosis, atrophy of skin, bilateral microphthalmia and congenital cataract. A brief review of literature was made.
A Case of Williams Elfin Facies Syndrome.
Kyung Jin Shin, Heon Seob Song, Byung Sook Park, In Soo Choi, Chan Uhng Joo, Jung Ku Jo
Clin Exp Pediatr. 1987;30(6):684-690.   Published online June 30, 1987
Williams elfin facies syndrome is a disorder which has previously designated as the pecular facies, mental retardation and supravalvular aortic stenosis. Supravalvular aortic stenosis is the least form of left ventricular outflow obstruction, originating at superior margin of sinuses of Valsalva just above the level of the coronary arteries. We had experienced a case of Williams elfin facies syndrome who had characteristic face, mental retardation...
A Case of Kasabach-Merritt Syndrome.
Byong Lae Kim, Jeong Seo Koh, Woan Chul Suh, Jae Kon Ko
Clin Exp Pediatr. 1987;30(5):577-582.   Published online May 31, 1987
Kasabach-Merritt Syndrome is association of thrombocytopenia with giant hemangioma. A 20 days, male patient had thrombocytopenia, the hemangioma involved the skin of the right distal femur. This patient was placed on combined therapy with radiation and steroid. Following the conservative therapy, the hemangioma regressed and the platelet returned to normal range. We presented the case with brief review of the literatures.
Original Article
A Case of Cloverleaf Skull; Kleeblattschael Syndrome.
Chang Hyon Yang, Kyu Earn Kim, Chang Ho Hong, Young Mo Sohn, Don Gyu Yang, Kee Keun Oh
Clin Exp Pediatr. 1987;30(4):456-460.   Published online April 30, 1987
A case of Cloverleaf skull; Kleeblattschadel syndrome in a 70-day old male infant with facial deformity since birth was seen. The diagnosis was made on the basis of typical morphologic clinical features an radiologic findings. A brief review of the related literature was given.
A Case of Congestive Splenomegaly(Banti Syndrome) with Hypersplenism.
Hyun Mo Cheong, Jae Kwang Hong, Joon Taek Park, Jung Sik Min, Chang Hee Choi, Seung Hye Ahn, Sang Chull Kim
Clin Exp Pediatr. 1987;30(4):416-421.   Published online April 30, 1987
The authors experienced a case of hypersplenism in a 12 year-old male patient with the chief complaints of nasal bleeding and palpable mass on LUQ of abdomen. The patient had splenomegaly, leukopenia, and thrombocytopenia. He had high portal venous pressure of about 340 mmH20 and the splenoportography showed non-visualized portal vein and tortuous vessels suggesting portal vein obstruction. Splenectomy was done and his symptoms...
Chlorambucil Treatment in Chidren with Frequently Relapsing Minimal Lesion Nephrotic Syndrome.
Myung Hee Chung, Hae Woon Chang, Haeng Mi Kim, Ja Hoo Koo
Clin Exp Pediatr. 1987;30(4):370-377.   Published online April 30, 1987
A clinical study was conducted on children with frequently relapsing nephrotic syndrome to see the therapeutic effectiveness of chlorambucil therapy. Study population consisted of 21 children with biopsy-proven minimal lesion nephrotic syndrome, in whom chlorambucil therapy was given to control their frequent relapses and/or steroid dependency. Eleven patients were frequent relapser and 10 were steroid dependant. Study period extended from December 1980 to August...
Case Report
A Case of "Cri-du-Chat" Syndrome.
Hye Lyung Baik, Gui Sook Choi, Joon Cho, Moon Soo Park, Jin Keum Chang, Sung Woo Shin, Shin Yong Moon
Clin Exp Pediatr. 1987;30(3):309-313.   Published online March 31, 1987
A case of Cri-du-Chat syndrome was presented which was characterized by typical cat cry, staring gaze and other common deletion syndrome. The karyotype of it was proved to be 46, XY, (5p_) and a brief review was added for this case.
Original Article
Clinical Study on the Hypoplastic Right Heart Syndrome.
Sejung Sohn, Soon Ung Kang, Chung Il Noh, Jung Yun Choi, Yong Soo Yun, Chang Yee Hong, Dug Ha Kim, Jong Lin Rhi
Clin Exp Pediatr. 1987;30(3):281-290.   Published online March 31, 1987
A clinical observation was made on 12 cases of the hypoplastic right heart syndrome who were admitted to the department of Pediatrics, Seoul National University Hospital from January 1978 to May 1986. The -results were as follows; 1) The ratio of female to male was 1:1 and, according to the anatomic classification by Khoury, 6 cases were in type 1, 4 cases in type II...
A Cases of Dandy-Walker Syndrome.
Jong Lin Rhi, Yoon Dok Kim, Hyung Ro Moon
Clin Exp Pediatr. 1987;30(2):234-239.   Published online February 28, 1987
The Dandy-Walker syndrome is a developmental disorders of the brain characterized by cystic deformity of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. Other systemic anomalies are associated with the syndrome. We experienced a case in a 15 day old female infant who presented initially with frequent aspirations and respiratory difficulty. On physical examination an encephalocele on the occipital area, a...
A Case of Congenital Asplenia Syndrome.
Hyung Sook Kim, Jin Soo Cho, Sung Kee Jim, Doo Sung Moon, kyung Sook Cho, Jong Dae Cho
Clin Exp Pediatr. 1987;30(2):218-222.   Published online February 28, 1987
Department of Pediatrics, Maliknol Hospital The congenital asplenia syndrome is very rarely seen and is characterized by congenital absence of the spleen, usually associated with other groups of cardio-vascular, bronchopulmonary and gastrointestinal tract. The authors recently experienced a case of congenital asplenia syndrome in a 6 month-old, male infant. He has no splenic image by using 99m Technetium-sulfur scanning. He also had common atrioventricular...
A Retrospective Study on the Effect of Cyclophosphamide in Children with Minmal Change Nephrotic Syndrome.
Il Soo Ha, Kwang Wook Ko, Yong Il Kim
Clin Exp Pediatr. 1987;30(2):158-174.   Published online February 28, 1987
Thirty one children associated with biopsy-proven minimal change nephrotic syndrome with frequent relapse, were placed on cyclophosphamide, and its effect was analyzed. Cyclophosphamide was given with the dosage of 2.46±0.42 mg/kg/day for 58±7 days along with the alternate day corticosteroid. Sixty three percent of the patients showed complete remission and twenty seven percent of the patients resulted in partial remission after the treatment. Those...
Case Report
A Case of Poland Syndrome.
Jong Deok Kim, Hyung Kook Ki, In Kyung Sung, Byung Churl Lee
Clin Exp Pediatr. 1987;30(1):99-101.   Published online January 31, 1987
The Poland syndrome consists of congenital unilateral absence of the pectoralis major muscle and ipsilateral anomalies of the hand, typically syndactyly. It may be combined with many other anomalies. Recently, we experienced an 8 days old boy, who had the total absence of the metacarpals and phalanges of left hand and defect of the pectoralis muscle on the same side. A brief review of the...
A Case of Turner's Syndrome with Hydronephrosis.
Hye Ryun Kang, Hee Bong Park, Myung Jin Kim, Mee Na Lee
Clin Exp Pediatr. 1987;30(1):94-98.   Published online January 31, 1987
We Have experienced a case of Turner’s syndrome with horseshoe kidney, hydronephrosis and gastrointestinal bleeding in a 5 months old female. The patient presented with huge palpable abdominal masses, high arched palate, micrognathia and hypoplasia of finger and toenails. On operation, horseshoe kidney, hydronephrosis and obstruction of left ureteropelvic junction and atresia of left ureter were found. Left nephrectomy was performed. The child was diagnosed...
A Case of Hunter Syndrome.
S K Kim, W P Chung, H S Kim, S H Kim, C M Kang
Clin Exp Pediatr. 1986;29(12):1393-1398.   Published online December 31, 1986
Authors experienced a case of Hunter syndrome in a 5 year old girl who was admitted to Pediatric Department of Dong-San Hospital, Keimyung University for the evaluation of short stature and mental retardation. Patient had characteristic peculiar features of mucopolysaccharidoses and changes in skull, rib, vertebra and long bone radiography. Corneal clouding was absent. Screenings test using methylene blue for mucopolysaccharidoses was positive. The review of...
A Case of Blue Rubber Bleb Nevus Syndrome.
Kwang Soo Oh, Soon Kook Choi, San Ho Kim, Bock Keun Kee, Hyung Sun Sohn, Seok Don Park
Clin Exp Pediatr. 1986;29(12):1372-1376.   Published online December 31, 1986
The Blue Rubber Bleb Nevus Syndrome is defined as rarely occuring hemangioma of the skin and gastrointestinal tract, with intestinal hemorrhage. Bean in 1958 gave the name Blue Rubber Bleb Nevus (BRBN) to a variant of bluish hemangiomas of the gastrointestinal tract which causes bleeding, which had been described for the first time by Gascoyen. We experienced a case of Blue Rubber Bleb Nevus...
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