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Original Article
A Case of Potter Syndrome Type Ⅰ.
Jin Hong Park, Mi Jeong Koh, Yeon Kyun Oh, Chan Choi
Clin Exp Pediatr. 1989;32(8):1150-1154.   Published online August 31, 1989
We experienced a case of Potter syndrome type I characterized by polycystic kidney, pulmomnary hypoplasia and abnormal face in a 1 day-old male neonate with respiratory distress. Diagnosis was made with autopsy findings. A brief review of literature was made.
A Case of Peutz-Jeghers Syndrome.
Ick berm Park, Sung guen Oh, Jung suk Ko, Sang hyun Byun, Kyu chul Choeh
Clin Exp Pediatr. 1989;32(7):990-994.   Published online July 31, 1989
This is a report of our experience of Peutz-Jeghers syndrome in a 14 year old girl having family history. She was admitted with complaints of crampy abdominal pain and vomiting, and showed melanotic pigmentation on the lips, face around nose, oral mucosa around molar areas, fingers and toes. Colon study revealed multiple polyposis in colon and small bowel. In family, her father has melanotic...
A Case of Laurence-Moon-Biedl Syndrome Including Diabetic Mellitus.
Byoung Hoon Lee, Byung Rai Cho, Myoung Ik Lee, Keun Chan Sohn, Hyung Joon Yoo
Clin Exp Pediatr. 1989;32(6):857-861.   Published online June 30, 1989
Laurence-Moon-Biedl syndrome has a varialbe phenotype and includes obesity, mental deficiency, retinitis pigmentosa, polydactyly and hypogonadotrophic hypogonadism. This report concerns a rare case of a 13-year-old girl having diabetic mellitus with Laurence-Moon-Biedl syndrome. The relevant literature was reviewed.
A Case of Prune Belly Syndrome.
Myoung Suk Nam, Soon Soen Lim, Young Ha Kim, Moon Ki Joe
Clin Exp Pediatr. 1989;32(6):852-856.   Published online June 30, 1989
We have recently experienced a case of prune.belly syndrome in 1.day.old male with congenital defect of abdominal muscles, both cryptorchidism, ureter and bladder dilatation, left renal cyst. Imperforate anus and left club foot were associated. The diagnosis was confirmed by autopsy. A brief review of Iiterature was made.
3 Cases of Prune Belly syndrome.
Chong Sung Chung, Chong Woo Bae, Byung Soo Cho, Chang Il Ahn, Ju Hie Lee, Moon Ho Yang
Clin Exp Pediatr. 1989;32(6):845-851.   Published online June 30, 1989
The prune belly syndrome is a rare congenital anomaly characterized by laxed, wrinkled abdominal wall, cryptorchidism and urinary tract anomalies. But it has wide spectrum of clinical severity, clinical presentation and other anomalies such as pulmonary and skeletal anomalies. We suggested that we should examine the abdominal wall carefully in order to detect mild form (class III) of prune belly syndrome when we...
A case of Evans Syndrome Associated with A-V Malformation.
Keun Haeng Cho, Min Young Lee, Kwang Chul Lee, Young Sook Hong, Soon Kyum Kim
Clin Exp Pediatr. 1989;32(6):839-844.   Published online June 30, 1989
In 1951, Evans and associates first described a group of patients with primary thrombocytopenic purpura for a diagnosis of Evans syndrome, The criteria for a diagnosis of Evans syndrome were(l) hemolytic anemia with a positive direct coombs’ test and thrombocytopenia occuring either simultaneously or in succession and(2) the absence of any known underlying etiology. We experienced a case of Evans syndrome associated with...
Bone Age Determination and Hand Radiographic Findings in Children With Russell-Silver Syndrome.
Hun Kyu Lim, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1989;32(6):823-833.   Published online June 30, 1989
Russell.Silver syndrome is a disease of unknown etiology characterized by prenatal onset dwarfism irrespective of full term gestation, growth retardation, body asymmetry, craniofacial dysproportion with a triangular face, short and incurved little finger, etc. Forty.two children were diagnosed as Russell.Silver syndrome by Silver’s criteria at SNU Children' s Hospital from Apr. 1981 till Aug. 1988, and we reviewed clinical data and hand radiographs of...
Serum IgE Level in Patients with Minimal Change Nephrotic Syndrome.
Sung Oh Kim, Hee Jung Ahn, Seong Ho Kim, Woo Gill Lee
Clin Exp Pediatr. 1989;32(6):789-796.   Published online June 30, 1989
It has been reported that many patients with minimal change nephrotic syndrome(MCNS) have high serum IgE level, but its significance has not been disclosed yet. In this study, serum levels of IgE were evaluated in different stages of the disease with or without treatment in 26 MCNS(proven by renal biopsy) patients admitted to the Department of Pediatrics Hanyang University Hospital from January, 1980 to...
Two Cases of Poland Syndrome.
Kul Ha Yoo, Ki Soo Pai, Byung Ju Chung, Chul Lee, Dong Gwan Han, Jong Doo Lee
Clin Exp Pediatr. 1989;32(5):713-717.   Published online May 31, 1989
Poland syndrome is characterized by an absence of the pectoralis major muscle, with ipsilateral defect of the upper extremity, usually syndactyly. The incidence of this syndrome has been estimated at 1 per 25,000 to 77,000 persons and more than 100 patients have been described worldwide. Recently, we experienced two patients, the one was 14 year old male, who showed absence of left pectralis maior...
A Case of Virus Associated Hemophagocytic Syndrome.
Yang Soo Kang, Ue Chong Yang, Hae Il Cheoung, Ho Jin Park, Mi Ja Shin, Je Geun Chi
Clin Exp Pediatr. 1989;32(4):567-575.   Published online April 30, 1989
Virus associated hemophagocytic syndrome is a rare hemophagocytic disorder, apparently associated with active viral infection, apparently associated with active viral infection, in which reactive histiocytes proliferate in the reticuloendothelial system, mainly bone marrow and medullary portions of lymph nodes, as a non-malignant, reversible but potentially constitutional symptoms with high fever and hepatosplenomegaly and by laboratory findings of pancytopenia, hemophagocytosis, abnormal liver function and coagulopathy. Inappropriate immunosuppressive...
A Case of Hypereosinophilic Syndrome.
Byoung Hoon Lee, Jae Wook Ko, Soon Wha Kim, Don Hee Ahn
Clin Exp Pediatr. 1989;32(4):561-566.   Published online April 30, 1989
Hypereosinophilic syndrome is a state of severe eosinophilia of unknown cause accomanied by multiple organ-system involvement. The criteria of hypereosinophilic syndrome includes. (1) A persistent eosinophil count of more than 1.5 X 10 9 /L for associated with the signs and symptoms of hypereosinohilic disease; (2) a lack of evidence for parasite, allergic, or other known causes of eosinophila; (3) presumptive signs and...
A Case of Edward Syndrom.
Youn Hong Choi, Hyun Joo Choi, Eun Hwa Shin, Ju Hong Cha, Kwang Jeon Kim
Clin Exp Pediatr. 1989;32(3):396-401.   Published online March 31, 1989
Edward syndrome is known as 18-trisomy with multiple congenital anomalies. Recently, we experienced a case of 18-trisomy syndrome in a new-born female baby who was born at Chung Gu Sung Sim Hospital. Grossly this baby had multiple anomalies which were characterized by prominent occiput, hypertelorism, small palpebral fissure, small oral opening, high arched palate, micrognathia, low-set malformed ear, rocker bottom feet, narrow pelvis, limited...
Clinical Application of Shake test od Gastric Aspiretes for the Prediction od Respiratory Distress Syndrome in the Newborn infants.
In Sang Jeon, Hann Tchah, Myoung Jae Choi, Beyng Il Kim, Jung Hwan Choi, Chong Ku Yun
Clin Exp Pediatr. 1989;32(3):349-358.   Published online March 31, 1989
To study the predictive value of gastric aspirates shake test on respiratory distress syndrome (RDS) in the newborn infants, we carried out the shake test on gastric aspirates of all newborn who admitted to NICU of Seoul National University Hospital from July 1986 to December 1987. We successfully carried out gastric aspirates shake test on 96 newborn infants and analyzed the relation between gastric...
A Case of Congenital Megakaryoblastic Leukemia Accompanied by Down Syndrome Which was Diagnosed by Autopsy Findings.
Il Kyung Kim, Dong Beom lee, Jung Sik Min, Chang Hee Choi, Je Geun Chi
Clin Exp Pediatr. 1989;32(2):262-269.   Published online February 28, 1989
Many cases of acute megakaryonlastic leukemia have been reported in childhood or adults, but congenital form or the cases occuring in neonatal period is very rare throughout the world. In Korea only one case was reported previously by Chung et al in 1987. The authors experienced a case of congenital megakaryoblastic leukemia accompanied by Down' s syndrome, which was confirmed by autopsy and positive...
11 q Deletion Syndrome.
Y B Im, S B Park, B Y Pyun, J O Park, S J Lee, S Y Moon
Clin Exp Pediatr. 1989;32(2):239-243.   Published online February 28, 1989
llq deletion syndrome is a rare chromosomal anomaly. The authors experienced a case of llq deletion syndrome with congenital glaucoma and infantile spasm in a female child. She showed delayed psychomotor development, flat occiput, hypertelorism, low set malformed ears, flat nasal bridge, short neck, simian line on right palm and throm. bocytopenia. Chromosomal study showed the deletion of the long arm of chromosme 11, karyotypically...
A Case of Neonatal Short Bowel Syndrome.
Gill Hyoun Kim, In Chul Yoo, Dong Hwan Lee, Sang Jhoo Lee
Clin Exp Pediatr. 1989;32(1):81-86.   Published online January 31, 1989
We experienced a case of neonatal short bowel syndrome due to multiple jejuno-ileal atresia with microcolon. On the 1st day of her life, there were symptoms of vomiting and abdominal distension. The radiological study show multiple dilated intestinalloops and malrotation. On the 4th day of her life, the extensive enterectomy was done. The remaining small bowel consisted of 30 cm of proximal jejunum and distal...
Clinical Observation on Guillain-Barre Syndrome in Children.
Byung Ran Yun, Hye Kyung Han, Yong Seung Hwang
Clin Exp Pediatr. 1989;32(1):52-61.   Published online January 31, 1989
Clinical observation was performed on 65 cases of Guillain -Barré syndrome which were en at the Department of Pediatrics, Seoul National University Hospital, during the past 10 years from January 1977 to December 1986. The results were as follows: 1) The ratio of male to female was 2:1 and 56.9% of the total cases were seen between the ages of 1-5 years. 2) The highest seasonal...
A Case of Beckwith-Wiedemann Syndrome.
Jeong Wi Wook, Eun Young Choi, Min Shik Kim, Hey Sun Lee, Young Min Ahn
Clin Exp Pediatr. 1988;31(12):1685-1688.   Published online December 31, 1988
We experienced one case of Beckwith-Wiedemann syndrome. This 5 day aged female neonate manifested gigantism, facial nevus flammeus, macroglossia, Promi- nent occiput, unusual linear fissures in lobule of external ear, omphalocele, gall bladder hydrops, and mild hypoglycemia. A brief review of related literature was done.
A case of Marfan Syndrome.
T U Kim, S H Bae, H S Yoon, S H Kim
Clin Exp Pediatr. 1988;31(12):1680-1684.   Published online December 31, 1988
We experienced a case of Marfan syndrome in 10 year-old girl with dolichomorphism, myopia, dilatation of ascending aorta, PDA, mitral valve prolapse. Diagnosis was established by clinical characteristics with echocardiography and cardiac catheter- ization. And also, Pertinent literatures on Marfan svndrome were reviewed briefly.
A Case of Kartagener's Syndrome.
Young Jin Min, Chang Il Ahn, Kyung Sook Cho, Jong Dae Cho
Clin Exp Pediatr. 1988;31(11):1522-1526.   Published online November 30, 1988
We experienced a case of Kartagener’s syndrome in a 15 year-old male patient who showed bronchiectasis, sinusitis, and situs inversus by X-ray and physical examination. We report a case of Kartagener's syndrome and a brief review of literliture was done.
A Study of Meconium Aspiration Syndrome.
Chae Sup Yoo, Mee Kyung Kim, Keun Lee, Jae Sun Jung
Clin Exp Pediatr. 1988;31(11):1425-1431.   Published online November 30, 1988
The authors observed 51 cases of meconium aspiration syndrome in newborns who were bom at Ewha Womans University Hospital during the period of January 1986 through May, 1987. The results are summarized as follows: 1) The incidence of meconium aspiration syndrome was 1.6% (51 cases). 2) There was a significantly greater incidence of developing meconium aspiration syndrome in meconium stained babies who had toxemia mothers and low...
A Case of Dapsone Syndrome.
Jeong Kyu Seoh, Hye Kyong Bae, Jae Seung Yang, Eung Deok Choi, Baek Keun Lim, Jong Soo Kim
Clin Exp Pediatr. 1988;31(10):1376-1380.   Published online October 31, 1988
Dapsone syndrome is a rare hypersensitivity reaction to Dapsone. The reaction included fever, malaise, dermatitis jaundice with hepatic dysfunction, lymphadenopathy, and hemolytic anemia. We have experienced a case of Dapsone syndrome in a 10 years old male. This patients had taken D.D.S. with 50 mg per day for 1 month to treat an unknown skin disease. After then, above mentioned symptoms and signs were...
A Case of Treacher Collins Syndrome.
B I Choo, Y M Jung, S S Kee, U S Shim, H S Lee
Clin Exp Pediatr. 1988;31(10):1371-1375.   Published online October 31, 1988
The authors report a 3 days old newborn who showed the abnormalities comprising relatively complete form of Treacher Collins syndrome. These include following findings; 1) Antimongoloid slant of palpebral fissures. 2) Coloboma in outer third of lower lid with absence of cilia medial to coloboma. 3) Large nose with narrow nares and flattened frontonasal angle. 4) Macrostomia with cleft palate. 5) Deformed pinna, extra ear tag, blind fistura, absent external auditory canals,...
A Case of 4P-Syndrome.
Hong Kyu Lee, Sung Sik Lee, Soon Il Lee, Young Seok Lee, Kweon Ha Son
Clin Exp Pediatr. 1988;31(10):1366-1370.   Published online October 31, 1988
The first short arm deletion syndrome in human was described by Lejeune et al. in 1963. This has been labelled the 5p- syndrome. It was given the name ‘cri du chat` because of the characteristic kitten like cry of these patients. Two years later, Wolf et al. demonstrated a second short arm deletion syndrome which had no kitten like cry, but had characteristic...
Diagnosis of Meconium Aspiration by Spectrophotometric Analysis of Urine.
Chang Yul Kim, Hye Kyung Bae, Mee Kyung Namgoong, Baek Keun Lim, Joong Soo Kim
Clin Exp Pediatr. 1988;31(10):1275-1280.   Published online October 31, 1988
We measured optical density of aqueous extracts of meconium and meconium stained amniotic fluid at 390 nm, 405 nm, 420 nm, 435 nm, 450 nm by spectrophotometry. We also applied the spectrophotometric method to the urine from 25 infants with clinical sign of meconium aspiration and from normal infants. The absorption band at 450 nm observed on the spectra of urine was estimated as...
Lipoproteinelectropheresis pattern in Nephrotic Syndrome.
Soon Don Hong, Byung Soo Cho, Chang Il Ahn
Clin Exp Pediatr. 1988;31(9):1163-1170.   Published online September 30, 1988
This study was undertaken to eluidate lipoprotein patterns in patients with childhood nephrotic syndrome by response to initial steroid therapy, serum albumin level in MCNS and other histologic classifications. The result were summerized as follows. 1) There were 19 cases (52.8%) of abnormal lipoprotein patterns by Fredrickson classifications, type Ila were 6 cases (16.7%), type lib were 1 cases (2.8%) and type IV were 12 cases...
A Study on Prognostic Factors of Meconium Aspiraion Syndrome.
Oh Kyung Lee, Hong Chul Lee, Wan Seoub Kim, Myung Ho Lee
Clin Exp Pediatr. 1988;31(9):1114-1119.   Published online September 30, 1988
We observed 42 cases of neonates, who were diagnosed as having Meconium aspiration syndrome in neonatal intensive care unit of Presbyterian Medical Center from July 1984 to May 1987. The followings were obtained: 1) The incidence of Meconium aspiration syndrome was 0.6% of all deliveries and 7.4% of meconium stained babies. 2) The geatational age was over 37 weeks in all cases and the body weight was...
A Case of Ectopic ACTH Syndrome Caused by Wilms Tumor.
Dong Hun Chi, Sang Bong Han, Young Jong Woo, Tai Ju Hwang
Clin Exp Pediatr. 1988;31(8):1071-1078.   Published online August 31, 1988
We experienced a extremely rare case of ectopic ACTH syndrome caused by Wilms tumor in a 4 year 8 month-old female patient. We performed specific radiologic and hormonal studies. Diagnosis was confirmed by histologic and biochemical findings of tumor mass after right nephrectomy. A brief review of related literature was made.
A Case of Acute Megakaryoblastic Leukemia in Infant with Down's Syndrome.
Jung Soon Whang, Soon hee Kim, Moon Ja Kim, Keun Lee, Je Geun Chi
Clin Exp Pediatr. 1988;31(8):1064-1070.   Published online August 31, 1988
Acute megakaryoblastic leukemia is an uncommonly recognized disorder that is characterized by rapidly progressive proliferation of atypical megakaryocytes and their precursor cells, and fatal course. Abnormalities in chromosome 21 may have a more than random relationship to acute megakaryoblastic leukemia. The authors reports a case of acute megakaryoblastic leukemia in a 2 month old female patient who was admitted for evaluation of hepatosplenomgaly. Multiple organ infiltration...
Thyriod Function Studies in Children with Nephrotic Syndrome.
In Hoon Lee, Chan Yung Kim
Clin Exp Pediatr. 1988;31(8):1037-1047.   Published online August 31, 1988
To evaluate the influence of nephrotic syndrome on the thyroid function test in children, serum T3, T4, free T4, TSH, TBG and rT3 were measured using radioimmunoassay in 14 clinically euthyroid children with nephrotic syndrome who were admitted to Department of Pediatrics, Pusan National University Hospital from July, 1986 to August, 1987. Control group was 15 children who visit our outpatient department for minor...
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