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Original Article
Re-evaluation of the indication for renal biopsy in childhood nephrotic syndrome.
Eun Kyoung Sohn, Byung Soo Cho, Chang Il Ahn
Clin Exp Pediatr. 1991;34(6):805-811.   Published online June 30, 1991
Renal biopsy is indicated in nephrotic syndrome when histopathologic types other than minimal change pattern or the secondary nephrotic syndrome are suspected. But renal biopsy in children has some limitation because of the technical problem. In order to assess the clinicopathologic correlation and to re-evaluate the biopsy indication, the clinical manifestations and histopathologic types were analyzed statisically on the 86 cases of childhood nephrotic syndrome...
A case of the lupus syndrome induced by hydralazine.
Beom Soo Park, Joong Gon Kim, Yong Soo Yun
Clin Exp Pediatr. 1991;34(5):736-739.   Published online May 31, 1991
A reversible syndrome resembling systemic lupus erythematosus is induced by hydralazine which has been used in treatment of hypertension or congestive heart failure. The incidence of the lupus syndrome induced by hydralazine was estimated at 6.8% in adults (Cameron, 1984) but there was few reports in children. A 9 year old girl had arthralgia, fever, splenomegaly, pleurisy and oral ulcer after 62 months of hydralazine...
Two cases of Goldenhar syndrome.
Moo Young Song, Min Sik Kim, Nam Soo Park, Un Jun Hyoung, Jin Oh Lee, Eun Ryoung Kim
Clin Exp Pediatr. 1991;34(5):730-735.   Published online May 31, 1991
Goldenhar syndrome is a pattern of malformation with wide variability of expression characterized by epibulbar dermoids, preauricular appendages and pretragal blind-ended fistulas, and vertebral anomalies. We have experienced two cases of Goldenhar syndrome. In one case multiple hemivertebrae, anomalous ribs, hydrocephalus with abscence of the septum pellucidum and mild widening of the subarachnoid space were noted. Diagnosis was made by physical examination and radiological findings. A brief...
A case of 4p- syndrome with oligomeganephronia.
Ri Sa Lee, Bong Sik Kong, Beyong Il Kim, Sang Kyu Park, Ho Jin Park, Soong Deok Lee, Je Geun Chi
Clin Exp Pediatr. 1991;34(4):558-565.   Published online April 30, 1991
The 4p_ syndrome results from structural deficiency of chromosome 4. We experienced a patient of 4p~ syndrome who all features of already described Wolf syndrome, i.e., prominent glabella, hypertelorism, broad beak nose, cleft lip and palate, kyphoscoliosis, hypospadia, etc.. Postmortem examination revealed multiple visceral anomalies, including large atrial septal defect, diaphragmatic eventration, intestinal malrotation, ankyloglossia, and hemivertebrae. The kidneys showed a marked simple hypoplasia weighing...
A case of Hunter syndrome.
Suk Hyun Ha, Young Sun Ko, Mi Soo Ahn, Ji Sub Oh
Clin Exp Pediatr. 1991;34(3):398-403.   Published online March 31, 1991
Authors experienced a case of Hunter syndrome in 4 old male patient, whose diagnosis was established by typical findings in physical examination, family history, screening test using methylene blue for mucopolysaccaharides, and radiologic studies. We report this case with a brief review of literatures.
Clinical study of Kasabach-Merritt syndrome.
Eun Jung Bae, Young Ah Lee, Hee Young Shin, Hyo Seop Ahn
Clin Exp Pediatr. 1991;34(3):371-379.   Published online March 31, 1991
A retrospective analysis of clinical findings in 20 patients with Kasabach-Merritt syndrome who visited Department of Pediatrics, Seoul National University Hospital from January, 1979 to June, 1990 was performed. The average age of occurrence was 6.4 month and male to female ratio was 1: 1.2. The mean size of hemangioma was 11.0x8.5 cm and the locations were cutanous in 18 (extrem- ities 5, trunk...
A case of hyperimmunoglobulin E syndrome.
Myeong Cheol Shinn, Sung Dong Choi, Byung Gyu Suh, Jin Han Kang, Joon Sung Lee
Clin Exp Pediatr. 1991;34(2):292-298.   Published online February 28, 1991
The hyperimmunoglobulin E syndrome is a primary immunodeficiency disorder characterized by serious recurrent bacterial infections of the skin and respiratory system in early life, and markedly elevated serum IgE level. These recurrent infections are generally subcutaneous abscesses caused by Staphylococcus aureus. Also, additional characteristrics of this syndrome include coarse facial appearance, chronic pruritic dermatitis and eosinophilia. The baisic immunologic defect mechanism in hyperimmunoglobulin E syndrome is...
Two cases of Edward syndrome.
Jin Bok Hwang, Woo Hyun Kwon, Soon Young Song, Chang Ho Han, Hye Li Chung, Young Dae Kwon
Clin Exp Pediatr. 1991;34(2):250-255.   Published online February 28, 1991
We presented two cases of Edward syndrome in this report. Both cases were bom with multiple congenital malformation which were characterized by low set malformed ears, micrognathia, webbed neck, rocker bottom feet, small pelvic and abducted, index and third fingers flexed upon with simian crease and small sized great toes with mild dorsifiexion. A large VSD was found on autopsy in one case who...
The clinical study on MCLS.
Young Sun Ko, Ji Sun Cho, Hong Bae Kim, Ji Sub Oh
Clin Exp Pediatr. 1991;34(2):240-249.   Published online February 28, 1991
Forty six children with mucocutaneous lymph node syndrome were diagnosed and treated through admission at the Department of Pediatrics, Wallace Memorial Baptist Hospital from January 1987 to January 1990. Ther were investigated for clinical pattern and laboratory correlations. The results were as follows: 1) Most patients (95.7%) were under four years of the age. 2) The ratio of male to female was 1.6:1. 3) Seasonal incidence was high in...
Adrenocortical carcinoma in a twelve month old male infant.
Woo Ryoung Lee, Kyoung Whan Oh, Chang Hwi Kim, Sang Jhoo Lee
Clin Exp Pediatr. 1991;34(1):137-143.   Published online January 31, 1991
Adrenocortical carcinoma is a rare disease in childhood. Most of these carcinomas are functional, produc- ing endocrine abnormalities such as Cushing syndrome, virilization, precocious puberty, hyperaldosteronism or feminization. We experienced a case of adrenocortical carcinoma in a 12 month old male infant who exhibited features of Cushing syndrome, Virilization, precocious puberty and hyperaldosteronism, which were due to functional adrenocortical carcinoma. Microscopic examination revealed cellular pleomorphism...
A Case of Beckwith-Wiedemann Syndrome.
E.W. Park, D.U. Kim, W.H. Choi, K.W. Kim
Clin Exp Pediatr. 1991;34(1):126-129.   Published online January 31, 1991
We experienced a case of Beck with-Wiedemann Syndrome. This 1-day-old female neonate manifested gigantism, macroglossia, anomaly of the umbilical cord, hemihypertrophy, mild he- patomegaly, congenital heart disease and severe hypoglycemia. A brief review of related literature was made.
A case of report fiber syndrome.
So Young Lee, Sun Yang Hong, Jae Hoon Shin, In Joon Seol
Clin Exp Pediatr. 1991;34(1):115-119.   Published online January 31, 1991
This is a report of 10 years old boy whose complete total ophthalmoplegia, ataxia and areflexia (Fisher syndrome) were the outstanding clinical features.
Clinical considerations of acute leukemia or transient myeloprolifo- rative disorder in Down syndrome.
Eun Sil Dong, Sung Hee Jang, Hong Hoe Koo, Hye Lim Jung, Hee Young Shin, Hyo Seop Ahn
Clin Exp Pediatr. 1991;34(1):74-82.   Published online January 31, 1991
Children with Down syndrome have an increased incidence of acute leukemia. Infants with Down syndrome are also at risk of developing a transient myeloproliferative disorder indistinguishable from acute nonlymphocytic leukemia (ANLL) except by its eventual clinical recovery. We observed 11 patients with acute leukemia or transient myeloproliferative disorder in Down syndrome who had admitted to the Departmetn of Pediatrics, Seoul National University Children’s Hospital,...
Therapeutic effects of plasmapheresis in Guillian-Barre syndrome.
Sung Jin Chang, Sun Hee Jung, Eun Sook Suh, Chin Moo Kang
Clin Exp Pediatr. 1991;34(1):66-73.   Published online January 31, 1991
Although the exact mechanism of the immunologic injury is unknown, the pathogenesis of the Guillain-Barr受 syndrome is explained as an autoimmune attack directed against to peripheral nervous myelin. So plasmapheresis is tried for treatment of Guillain-Barr즌 syndrome. Authors analysed 12 cases af Guillain-Barr受 syndrome who were treated with plasmapheresis and compared the results with that of the 13 cases, treated with conventional therapy. Duration between...
A clinical study on meconium-stained babies.
Cheol Ki Min, Sung Suk Bang, Chong Young Park, Hae Ran Lee, Choon Myung Ro
Clin Exp Pediatr. 1991;34(1):24-30.   Published online January 31, 1991
Among a total of 667 newborns delivered at Han Gang Sacred Heart Hospital from January 1988 to July 1989, a clinical study was done regarding meconium-stained babies. The results were summarized as follows: 1) The incidence of meconium staining was 25.9% (173 babies) and there was no difference of frequency in sex. 2) The higher the birth weigt and the gestational age, the higher the incidence of...
A clinical study on the idiopathic respiratory distress syndrome of the newborn: comparison of radiographic and clinical findings.
Byoung Yul Lim, Chong Woo Bae, Yong Mook Choi, Chang Il Ahn, Sun Wha Lee
Clin Exp Pediatr. 1991;34(1):16-23.   Published online January 31, 1991
Idiopathic respiratory distress syndrome (IRDS) of the newborn remains an important and signifi- cant cause of morbidity and mortality despite of improvement in neonatal care. The clinical and radiologic features observed are the results of surfactant deficiency combined with a highly compliant chest wall. We have been applied the clinical and radiologic features in the diagnosis of IRDS. In 1974, Bomsel et al reported the...
A Case of MELAS Syndrome.
Ki Joong Kim, Yong Seung Hwang, Young In Choi, Sung Hye Park, Je Geun Chi
Clin Exp Pediatr. 1990;33(11):1586-1592.   Published online November 30, 1990
MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode) syndrome is a rare but clinicopathologically distinctive mitochondrial disorder. We experienced a case of MELAS syndrnmp in A 11 year-old girl who showed repeated attacks of stroke-like episodes, vomitng, cortical blindness, hearing deficit and alternating hemiplegia. Serum lactic acid level was elevated (6.4 mmol/Z). The diagnosis was confirmed by muscle biopsy which revealed dispersed ragged-red fibers.
A Case of Acute Megakaryoblastic Leukemia in infantwith down Syndrome.
Jang Sik Moon, Hae Young Hwang, Sejung Sohn, Hak Soo Lee, Heum Rye Park
Clin Exp Pediatr. 1990;33(10):1441-1446.   Published online October 31, 1990
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 more than relationship to it. The authors report a case of acute megakaryoblastic leukemia in a 17 day-old male patient who was admitted for evaluation of high fever and dyspnea. The infiltration of atypical megakaryocytes...
A Case of Lissencephaly with West Syndrome.
Byoung Yul Lim, Ji Ho Song, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1990;33(10):1413-1417.   Published online October 31, 1990
Neuronal migrational disorders of the brain represent abnormalities in the formation of the neocortex caused by faulty migration of the subependimal neuroblasts. Lissencephaly is a rare congenital anomaly representing an arrest of brain development before the third or fourth month of gestaional age. The most common clinical findings were seizure, delayed development, failure to thrive and hydrocephalus. The authors experienced a case of lissencephaly with...
Thyroid function Study in Respiratory distress Syndrome.
Sung Soo Kong, Mi Young Chung, Dong Hyunk Kum
Clin Exp Pediatr. 1990;33(10):1394-1398.   Published online October 31, 1990
It has been known that intrauterine thyroid hormone deficiency may be one of the factors predis- posing to RDS in premature infants. L-thyroxine has been shown to increase the production opf lung surfactant, widely accepted as deficient in RDS infants, as association between lung immaturity and fetal thyroid function is postulated. The authors investigated thyroid hormone concentration at birth and 5, 15 days of...
A Case of 9p-Syndrome.
Bok Hoi Kim, Shin Heh Kang, Kir Young Kim, Kyung Ja Cho, Kyung Hee Ha, Bum Suk Lee
Clin Exp Pediatr. 1990;33(9):1262-1265.   Published online September 30, 1990
We experienced a case of 9p- syndrome in a 2 year and 5 month old male child who manifested psychomotor retardation and multiple congenital anomalies. His karyotype was 46, XY, del (9) (p22). A brief review of literature was also presented.
A Case of Adrenal Cortical Carcinoma.
Ho Won Kang, Hong Ja Kang, Hong Bae Kim, Ji Sub Oh
Clin Exp Pediatr. 1990;33(8):1159-1165.   Published online August 31, 1990
This is a case report of adrenal cortical carcinoma in a 2 year 7 month old male patient who was diagnosed by clinical features, endocrinologic studies, radiologic studies and pathologic examina- tions. Cushing syndrome and virilization caused by adrenal cortical carcinoma were found. Left total adrenalectomy was performed. No evidence of metastasis was found at the time of operation. 3 months later, lung metastases...
A Case of Hemolytic Uremic Syndrome.
Seung Cheol Lee, Chong Guk Lee, Seong Hwan Kim, Sang Woo Kim, Hyun Soon Lee
Clin Exp Pediatr. 1990;33(8):1140-1147.   Published online August 31, 1990
A female child aged 7 months was admitted because of high fever, vomiting and stuporous conciousness and revealed to have hemolytic uremic syndrome. Anuria persisted 13 days and required peritoneal dialysis 4 times a day during the anuric period. Her kidney function recovered somewhat after dialysis but neurologic complications of marked hydrocephalus and brain atrophy remained.
A Clinical Study on the Posterior Subcapsular Cataracts in Childhood Nephrotic Syndrome.
Dong Kyu Jin, Ja Wook Koo, Hae Il Cheong, Yong Choi, Kwang Wook Ko, Jong Hoon Lee, Young Suk Yu
Clin Exp Pediatr. 1990;33(8):1097-1103.   Published online August 31, 1990
A clinical study was carried out in 73 children with nephrotic syndrome who had had regular ophthalmologic examination annually at Seoul National University Children’s Hospital from March 1976 to February 1990 with the aim of finding the relationship between the posterior subcapsular cataract (PSC) and the effect of corticosteroids. The results were summarized as follows: 1) The number of patients with PSC were 23 cases...
A Case of (21q 21q) Translocation Down Syndrome Inherited from a t(21q 21q) Balanced Carrier Mother.
Byeong Gie Yeo, Chong Woo Bae, Yong Mook Choi, Chang Il Ahn, Bo Hoon Oh
Clin Exp Pediatr. 1990;33(7):1004-1008.   Published online July 31, 1990
Vast majority of t(21q 21q) Down syndrome occur de novo and familial cases are extremely rare. In familial translocation Down syndrome, One of the parent show 45 chromosomes. In general, the carrier parent carrying (21q 21q) translocation is phenotypically normal because significant amount of genetic material has not been lost in the translocation process. Homologous Robertsonian translocation can be produced either by abnormal gametogenesis...
A Case of Edward Syndrome.
Bong Hyune Chung, Hee Jung Seo, Young Hee You, Hyun Sook Lee
Clin Exp Pediatr. 1990;33(7):999-1003.   Published online July 31, 1990
A case of 18 trisomy syndrome in a female baby who bom at our K.V.H. was presented. The baby had multiple anomalies those were characterized by intrauterine growth retardation, narrow microce- phalic skull with prominent occiput, low-set malformed ears, micrognathia, rocker-bottom feet with short & big toe, shield-like chest and webbed neck. Chromosomal study showed trisomy 18. On autopsy, Horseshoe-kidney had been found.
Clinical Analysis of Children with Nephrotic Syndrome.
Ki Young Cheong, Keon Su Rhee
Clin Exp Pediatr. 1990;33(7):959-969.   Published online July 31, 1990
A clinical analysis was made on 51 cases with nephrotic syndrome who were admitted to Pediatric Department of Chung Nam National University Hospital from January 1st, 1984 to December 31st, 1988. Clinical manifestation, response to the inital continuous steroid therapy, relapsing patterns, factors affecting response to steroid therapy, pathologic findings and difference in laboratory findings between relapser and nonrelapser were evaluated. Results were as follows; 1) Among...
A Case of Poland's Syndrome.
Seok Gyoung Kang, Joo Tae Choi, Wha Mo Lee, Young Seok Jeon
Clin Exp Pediatr. 1990;33(6):860-863.   Published online June 30, 1990
Poland’s syndrome is a congenital disorder associated with absence of the pectoralis muscle and ipsilateral defect of the upper extremity, usually syndactyly. The syndrome is not hereditary and is of unknown origin. It affects males more frequently than females. Recently, we experienced a 1 year old male, who had the absence of right pectoralis muscle and ipsilateral syndactyly with hypoplasia of fingers.
A Case of Dandy-Walker Syndrome Associated with Multiple Congenital Anomalies.
Gye Weon Shin, Chul Ho Lee, Bo Kyung Cho, Chung Sik Chun, Sung Hun Cho
Clin Exp Pediatr. 1990;33(6):848-853.   Published online June 30, 1990
The Dandy-Walker Syndrome is a developmental disorders of the brain characterized by cystic deformity of fourth ventricle, agenesis or hypoplasia of the cerebellar vermis, and hydrocephalus. This syndrome is frequently associated with central nervous system malformation and systemic anomalies. We experienced a case in a 2-day-old female who has multiple congenital anomalies. On physical examinations, macrocephaly with prominent occiput, encephalocele in the occipital area, microphth- almia,...
An Experience of High Dose Intravenous Immunoglobulin Therapy in Refractory Evans Syndrome.
Hee Sup Kim, Won Sup Shin, Sang Il Lee, Sang Woo Kim
Clin Exp Pediatr. 1990;33(5):701-707.   Published online May 31, 1990
Evans syndrome, a combination of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura, relapses freguently and is refractory to conventional treatment such as corticosteroid, immunosuppressive agents, splenectomy and plasma exchange. Recently Oda et al applied high dose immunoglobulin (400/kg/day for 4 days repeated 2 weeks later) to 5 month-old infant with refractory Evans syndrome, and was successful in maintaining remission for five years and nine...