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Case Report
A Case of Weismann-Netter Stuhl Syndrome.
Kwang Ho Kim, Hong Ku Lee, Dong Hwan Cha, Jung Sim Choi, Chie Ja Cho, Jung Sook Lee
Clin Exp Pediatr. 1983;26(6):622-625.   Published online June 30, 1983
Weismann-Netter-Stuhl syndrome is congenital anterior bowing of the diaphyses of the tibia and fibula usually bilateral and symmetical, which is clinically resembles the sabre shin of syphilis. A case of Weismann-Netter-Stuhl syndrome in a 5 years oldboy was presented with a brief review of literature. This boy showed stunted growth, anterior bowing of legs and moderate degree of dyphosis, clear intelligence. X-ray showed...
A Case of Cornelia De Large Syndrome.
Do Seung Lee, Dae Young Hwang, Jeong Sick Min, Jae Sun Park
Clin Exp Pediatr. 1983;26(6):616-621.   Published online June 30, 1983
We have experienced a case of1 Cornelia de Lange Syndrome in a male infant. At first visit, the patient showed typical appearance of microcephaly, cleft palate, generalized hirsutism and growth retardation due to poor sucking since birth. The face of the patient was characterized by micrognathia, antirrongolism slant of eyes, synorphys of eye brows, bushy forehead and bushy lateral aspect of both cheeks, all...
A Case of Bartter'S Syndrome.
Yung Suk Song, Moon Ho Chung, Ha Baik Lee, Chong Moo Park
Clin Exp Pediatr. 1983;26(6):611-615.   Published online June 30, 1983
Bartter’s syndrome is rare disease which is characterised by hypokalemic, hypochloremic metabolic alkalosis, hyperreninemia, secondary aldosteronism, hyperplasia of juxtaglomerular apparatus without hypertension. We had experienced a case of Barker's syndrome in 4 months old girl. The diagnosis was made by clinical, hematologic, and biochemical studies. Literatures are reviewed briefly
Original Article
A Clinical Observational on Stenens-Johnson Syndrome.
T W Paik, H Y Chung, T C Kwon, Y D Kwon, C M Kang
Clin Exp Pediatr. 1983;26(5):482-488.   Published online May 31, 1983
A clinical study of 17 cases of Stevens-Johnson Syndrome in infancy and childhood, which were admitted to the Pediatric Department of Keimyung University, School of Medicine during the period of 6 and half years from January 1975 through July 1981 was made. The results are summarized as follows: 1) Stevens-Johnson syndrome has tendency of increasing incidence in recent years. 64.4% of the cases were seen during...
Study of Serum Lipids and Lipoprotein in Patients with Nephrotic Syndrome.
Eui Soo Park, Jeh Hoon Shin, Woo Gill Lee, Chong Moo Park
Clin Exp Pediatr. 1983;26(4):349-357.   Published online April 30, 1983
The nephrotic syndrome is characterized by generalized edema, hypoproteinemia (serum albumin; below 2.5 gm/dl), hyperlipidemia (serum cholesterol; above 220 mg/dl), massive proteinuria (2.0 gm/M2/24 hrs or more). For causes of hyperlipidemia, decreased plasma lipoprotein lipase activity and decreased serum albumin have been postulated as contributing factors. So the serum lipids and lipoproteins were studied in 24 patients with nephrotic syndrome...
Case Report
Two Cases of Di Guglielmo Syndrome.
Seok Ku Kim, Seong Ja Jeon, Chul Lee, Myung Ho Lee, Sook Ja Park
Clin Exp Pediatr. 1983;26(3):289-284.   Published online March 31, 1983
Di Guglielmo Syndrome is a relatively rare disease in childhood and refers to an acute or chronic myeloproliferative disorder in which erythrocytes and their precursors are the cells predominantly involved in the leukemic process. We experienced two cases of Di Guglielmo syndrome, an llyear-old girl and a 14 year-old boy, who had the chief complaints of severe anemia, fever, dizziness and general...
A Case Report Kostmann Syndrome.
Han Wook Yoo, Son Moon Shin, Hyo Seop Ahn, Yong Choi, Chang Yee Hong
Clin Exp Pediatr. 1983;26(3):284-288.   Published online March 31, 1983
Kostmann syndrome (Infantile genetic agranulocytosis), a disease characterized by recurrent infections leading to death in infancy, shows an agranulocytosis with variable monocytosis, eosinophilia in the peripheral blood, accompanied by the maturation arrest of the myelocytic series at the promyelocyte-myelocyte level. A 2 years 3 month old female patient with Kostmann syndrome is reported, who has suffered from recurrent infections since 1 month of age. During...
Original Article
Lipoprotein Profiles in Minimal Change Nephrotic Syndrome.
Yong Choi, Heui Jeen Kim, Hae Il Cheong, Jeong Kee Seo, Kwang Wook Ko
Clin Exp Pediatr. 1983;26(3):237-246.   Published online March 31, 1983
Data obtained from 59 hospitalized patients with minimal change nephrotic syndrome which was confirmed by kidney biopsy, were analyzed for serum albumin, serum lipids, 24hr urine protein, creatinine clearanee, HDL-eholesterol. In 38 of the patients, lipoproteins were analyzed. The following results were obained 1. Serum phospolipid (PL) was increased whenever serum total cholesterol (TC) was increased but to a lesser degree. The ratio of TC/PL were...
Case Report
9 Case of Klinefelter Syndrome.
Hack Joo Cha, Jong Sub Lee, Ki Bok Kim
Clin Exp Pediatr. 1983;26(2):203-208.   Published online February 28, 1983
A cytogenetic study was carried out on 9 cases of Klinefelter Syndrome referred to the Department during a-3-year period from February, 1979 through February, 1982. The following results were obtained. 1) The distribution of age at the time of definite diagnosis was between the age of 17 and 31, average being 23.8 years. 2) Mean height was 173.2 cm. 3) Chief complaints were small genitalia...
A Case of Hypereosinophilic Syndrome.
Chang Moo Lee, Chull Kwon Chung, Soon Ok Byun, Ji Sub Oh
Clin Exp Pediatr. 1983;26(2):193-197.   Published online February 28, 1983
We experienced a case of Hypereosinophilic syndrome with congestive heart failure in 15 month old girl. The clinical, hematologic and E.C.G. findings were compatible with hype- reosinophilic syndrome. She was treated with oxygen, digoxine, antibiotics and mebendazole. But on 8th day of admission she suddenly died. A brief review of the related literature was made on this subject.
Original Article
A Clinical Study on Reye Syndrome.
N C Kim, C H Kim
Clin Exp Pediatr. 1983;26(2):158-164.   Published online February 28, 1983
We analyzed 15 infants and children who were admitted and diagnosed as Reye syndrome. at Gu Mi Soon Chun Hyang Hospital during the period of 9 months from Feb. 1981 to Oct. 1981 The results were as follows. 1) The cases of Reye syndrome were 15 patients (1. 6%) among 579 hospitaHzed patients and 4 patients (26.7%) died of Reye syndrome. 2) It affected male...
Respiratory Distress Syndrome of the New born Infants and Complications of Pregnancy.
Yoon Joo Choi, Eun Ai Lee, Seung Joo Lee, Keun Lee
Clin Exp Pediatr. 1983;26(2):118-123.   Published online February 28, 1983
A group of 514 newborn infants with gestational ages between 28 weeks and 36 weeks were studied for the influence of pronolonged rupture of amniotic membranes and maternal hype- rtension. during pregnancy on. the incidence of Idiopathic Respiratory Distress Syndrome (IRDS). The incidence of IRDS in. complicated pregnancy is that significantly lower than in the control groups. (8.9% vs 18.8%, p<0.05) The occurence of...
Case Report
Eleven Cases of Turner Syndrome.
Jong Sub Lee, Hack Joo Cha, Ki Bok Kim
Clin Exp Pediatr. 1983;26(1):91-96.   Published online January 31, 1983
Among the patients who have been referred to our Cytogenetic Laboratory with the suspicion of Turner Syndrome during the past 8 years, eleven were confirmed by cytogenetic analyses. Clinical features and the results of cytogenetic analyses of those cases are summarized as follows: 1) The patients, all females, ranged in age from 16 to 25 years at the time of first consultation, and their...
Two Case of Ginaotti-Crosti Syndrome.
Jae Ho Lee, Soon Ung Kang, Jeong Kee Seo, Hyo Seop Ahn, Kwng Wook Ko, Won Suk Kim
Clin Exp Pediatr. 1982;25(12):1289-1294.   Published online December 31, 1982
The Gianotti-Crosti syndrome is an. infrequently recognized disorder with distinctive clinical features. We have experienced two cases of Gianotti-Crosti syndrome diagnosed by clinical manifestation, laboratory data and histopathologic finding of skin. Case one; An 18-month-old girl was admitted to the pediatric ward with the chief complaint of erythematous rash. Its onset was preceded by an. upper respiratory tract infection. She had a wide spread erythematous...
A Case of Acardi's Syndrome.
Bok Lyun Park, Hee Jung Chung, Chang Joon Coe, Young Sook Seo
Clin Exp Pediatr. 1982;25(12):1285-1288.   Published online December 31, 1982
A case of Aicardi’s syndrome is experienced at the Department of Pediatrics, College of Medicine, Yonsei University recently. This patient was 50 days of female infant, who had infantile spasm, and her development was deteriorated recently. Her CT scan showed partial absence of corpus callosum and whose EEG was a pattern of modified hypsarrhythmia limited only on left hmisphere. She also had hemivertebra at...
A Case of Potter Syndrome.
Young Mi Hong, Hae Seung Kim, Jung Gon Kim, Keun Lee
Clin Exp Pediatr. 1982;25(11):1181-1184.   Published online November 30, 1982
In 1946 Potter described a series of 20 cases in infants in whom bilateral absence of the kidneys was associated with, hypoplasia of the lung and characteristic face. The main facial features she described were increased space between the eyes, a prominent fold which arises at inner canthus and sweeps downward and laterally below the eyes, unusual flattening of the nose, excessive recession of...
Original Article
Physical Features of Korean Children of Down Syndrome.
Hack Joo Cha, You Nam Kim, Ki Bok Kim
Clin Exp Pediatr. 1982;25(10):994-1006.   Published online October 31, 1982
Ths physical features characteristic of Down Syndrome in Korean children were analyzed in 81 patients confirmed by cytogenetic examination in the Department of Pediatrics, Kwangju Christian Hospital, during the period from March, 1974 through July, 1981, and results are- summarized as follows: 1) The sex ratio was 2 males to one female. 2) The frequency of most stigmata found among Down cases were signifi cantly higher than, among...
A case of Chedia-Higashi Syndrome.
D K Moon, S H Shin, T C Kwon, C M Kang
Clin Exp Pediatr. 1982;25(8):843-847.   Published online August 31, 1982
A case of Chedia-Higashi syndrome in a seven months old male was presented. The diagnosis was suspected by albinism, nystagmus, photophobia, cervical lymphadenopathy, hepatosplenomegaly, past history of frequent infection and was confirmed by the peripheral and bone marrow findings of large purplish inclusion bodies in the cytoplasms of granulocytes and lymphocytes. A brief review of related literature was attempted.
A Case of Infantile Cortical Hyperostosis.
Won Bae Lee, Hyun Koo Oh, Jae Chung Lee, Sung Hoon Cho
Clin Exp Pediatr. 1982;25(8):839-842.   Published online August 31, 1982
Infantile cortical hyperostosis occurs in the first few months of life, and is characterized by irritability, fever, soft tissue swelling and radiological evidence of a periosteal reaction, particularly of the mandible. It's cause is unknown. The disease has been reported over 130 cases worldwidely, with only 2 cases in Korea sofar. Recently we experienced a case of infantile cortical hyperostosis....
Changes in Serum Complement and Immunglonulins following Cardiopulmonary Bypass.
Hong Jin Kim, Jun Hee Sul, Sung Kyn Lee, Dong Shik Chin, Kwang Ho Kim, Pill Whoon Hong
Clin Exp Pediatr. 1982;25(8):781-786.   Published online August 31, 1982
Clinical problem occuring in the early post operative period after cardiopulmonary bypass may ne related to the altered function of capillary bed which was affected by activation of complement system. This is so called post perfusion syndrome. We selected 20 patients with confirmed congenital heart disease by cariac catheterization. We studied the changes in the C3 , immunolglobulins, total protein,...
Case Report
One Case of 13q-Syndrome.
C O Lee, T H Lee, D W Lee, S J Lee, D.W Lee, D.Y Kang, K.Y Choi, Je G Ghi
Clin Exp Pediatr. 1982;25(7):743-747.   Published online July 31, 1982
13 q-syndrome based on loss of specific band was first described by Bain and Gauld in 1963 and may be transmitted if one parents is translocation carrier. We experienced one case of 13p-syndrome with ercephalocele in newborn diagnosed by clinical feature, chromosomal study and autopsy findings. He had multiple anomalies such as microcephaly, hypertelorism, microphthalmia, absent thumbs, clinodactyly, equinovarus, bifid...
Sonography in the Diagnosis and Management of Hydrops of the Gallbladder in Childern with Mucocutaneous Lymph Node Syndrome.
Byung Lyul Lee, Byung Hwan Lee, Kwang Chan Doh, Sung Il Ahn
Clin Exp Pediatr. 1982;25(6):633-637.   Published online June 30, 1982
Acute hydrops of the gallbladder is described in one patient who had mucocutaneous lymph node syndrome. During the course of her illness, acute addominal pain, vomiting and right upper guadrant tenderness were developed. Diagnosis was readily made with ultrasonography, and the patient was followed with this te-chnique. We managed her conventionally without surgical intervention. The galbladder returned to normal size...
Hyperviscosity Syndrome in Newborn.
Yong Soon Kim, Sook Hee Lee, Ja Hoon Koo, Doo Hong Ahn
Clin Exp Pediatr. 1982;25(6):622-627.   Published online June 30, 1982
We experienced Hyperviscosity syndrome in 5 newborn infants during 6 months period from August 1980 to January 1981. Diagnosis was made on the basis of characteristic clinical symp-toms along with polythemia. The following results were obtained. Sex in 5 affected infants showed male in 2 and female in 3. And gestational age showed preterm in 3 and full term in...
Two Cases of Postvaricella Reye Syndrome.
Won A Park, Sang Keun Chi, In Ho Kim, Churl Young Chung
Clin Exp Pediatr. 1982;25(5):528-532.   Published online May 31, 1982
Authors observed two cases of Reye syndrome in 7 and 8 year old male patients who had begun to vomit after chickenpox. The interval between the onset of varicella rash and encephalopathy of Reye syndrome was 4 days in both these cases. The initial laboratory findings revealed increased serum transaminase and blood ammonia levels which returned to normal on 18...
A case of Pierre-Robin Syndrome.
Soon Ja Han, yeongok bang, Dong Hwan Lee, Sang Jhoo Lee
Clin Exp Pediatr. 1982;25(4):419-422.   Published online April 30, 1982
Micrognathia, cleft palate and glossoptosis, a combination of anomalies known as Pierre Robin syndrome, causes severe respiratory and alimentary difficulties in affected infants. With appropriate care and treatment, mental development can be made normal. But about 10~25% of children become retarded. We have experienced above case in a female infant, aged 6 months. She was acmitted on account of respiratory...
A Case of Hypereosinophilic Syndrome.
Chul Young Lee, Ki Sup Chung, Byung Soo Kim, Chang Jin Kim, Chan Il Park
Clin Exp Pediatr. 1982;25(4):388-392.   Published online April 30, 1982
Marked eosinophilia is a common finding in many disease, occurring most frequently in allergic and parasitic conditions. A Separate syndrome characterized by peripheral eosinophilia and multisystem eosinophilic infiltration has been reported by many authors with a variety of diagnoses. In 1968, Hardy and Anderson proposed the term hypereosinophilic syndrome to encompass the entire group and in 1975, Chusid reported 14...
Original Article
Clinical Diagnostic Application of Serum Cholinesterase.
Kyu Cha Kim
Clin Exp Pediatr. 1982;25(3):258-268.   Published online March 31, 1982
Serum cholinesterase and other commonly employed function tests were determined in 12 patients with nephrotic syndrome, 17 patients with infectious hepatitis and 7 patients with acute glomerulonephritis who were available for se5rial studies the evaluation of which was helpful in diagnosis, management and prognosis of various disease. The results were as follows. 1) Serum cholinesterase activity in patients with nephrotic...
Case Report
A case of Finnish Type of Congenital Nephrotic Syndrome.
Jeong Sik Min, Yang Kook Shon, Soo Woong Lee, Suk Chul Kang, Yong Koo Park, Moon Ho Yang
Clin Exp Pediatr. 1982;25(2):175-182.   Published online February 28, 1982
The Finnish type of Congenital Nephrotic Syndrome is characterized by large placenta, early manifestation, growth and developmental delay and resistance to treatment. Authors experienced a case of characteristic Finnish type of Congenital Nephrotic Syndrome in a girl, who was admitted to the Pediatric Department of KHUH at 2 1/1 months of age because of generalized edema and abdominal distension and...
A Case of Trisomy Syndrome.
Keun Chul Choi, Hae Jung Cho, Que Sang Lee, Myung Ho Lee
Clin Exp Pediatr. 1982;25(1):102-106.   Published online January 31, 1982
We have seen a case of Edward syndrome in a new born neonate who was born at local obstetrics clinics to a healthy 27 years old mother. Just after birth, she was transfered to us and brought to emergency room, because of dyspnea and abnormal apperance, characterized by prominent occiput low set malformed ear, micrognathia, webbed neck, short sternum, small...
The Kocher-Debre-Semelaigne Syndrome.
M Y Chung, S I Kwon, S J Lee, D H Kum
Clin Exp Pediatr. 1982;25(1):92-96.   Published online January 31, 1982
A 4-year old girl with the Kocher-Debre-Semelaigne syndrome, hypothyroidism with generalized muscular hypertrophy is described. Histologic examination of a calf muscle showed musclular hypertrophy with focal eosinophilic degeneration. The stigmas of hypothyroidism and the muscular hypertrophy disappeared promptly and concomitantly following therapy with desiccated thyroid. Similar cases reported in literature are briefly reviewed. The clinical and histologic changes of the...
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