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Original Article
Manometric Response to Heller's Myotomy in Achalasia.
Chun Wha Lee, Kyung Mo Kim, Jeong Kee Seo, Hyung Ro Moon, Joo Hyun Kim
Clin Exp Pediatr. 1989;32(1):101-108.   Published online January 31, 1989
Achalasia is an espohageal motility disorder. It is characterized by the loss LES relaxation on swallowing and of esophageal peristalsis and results in dysphagia, regurgitation and substernal pain. Manometry is one of several diagnostic tests of achalasia and reveals 1) Complete absence of esophageal peristalsis, 2) hypertension of LES. 3) impaired sphincter relaxation on swallowing and 4) positive esophageal body pressure compared to intragastric pressure. We...
Two Cases of Tsutsugamushi Disease in Children.
Eun Saing Jee, Hae Lim Chung, Hoan Jong Lee, Hyung Ro Moon
Clin Exp Pediatr. 1988;31(11):1509-1515.   Published online November 30, 1988
Tsutsugamushi disease is an acute infectious and febrile disease that is transmitted to human by larvae of several species of mites, Leptotrimbidium pallidum, Leptotrimbidium akamushi and Leptotrimbidium scutellare. Recently in Korea, this disease is increasingly reported in adult age but few cases are reported in pediatric age. We experienced two cases of tsutsugamushi disease in children, one of whom had no rash. This disease should be...
Study on the Clitoral Size of the Korean Female Newborn.
Jong Lin Rhi, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1988;31(8):1007-1013.   Published online August 31, 1988
The disturbance of sex hormone secretions during the fetal life results in abnormal development of the fetal sex organs including the external genitalia. In the congenital adrenal hyperplasia, especially, the external genitalia is often virilized at birth and the clitomegaly in this disorder can be an important clue for the early diagnosis. For the normal range of the clitoral size of the Korean female...
Comparison of the Effects of Portacaval Shunt and Dietary therapy in Type I Gyocogen Storagy Diseases(GSD).
Dong Hyun Ju, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1988;31(6):762-771.   Published online June 30, 1988
Type I GSD is a metabolic disease resulted from defective glucose-6-phosphatase activities in liver, kindey and intestine. They are characterized by growth retardation, enlargement of liver and kidney, hypoglycemia, hyperuricemia, and hyperlipidemia. Various methods were suggested for the management of GSD. They are night-time nasogastric feeding of glucose solution with frequent day-time feeding, intravenous hyperalimentation, cornstarch therapy end-to-side portacaval shunt, etc. In this study, the effects...
Normal TSH Levels in Neonates by TSH Screening test.
Jae Won Song, Jong Lin Rhi, Sei Won Yang, Jung Hwan Choi, Chong Ku Yun, Hyung Ro Moon, Bo Youn Cho, Chang Soon Koh
Clin Exp Pediatr. 1988;31(6):754-761.   Published online June 30, 1988
In 110 term neonates without perinatal problems who were bom in SNUH, blood TSH levels were measured by TSH screening test for congenital hypothyroidism. The results are as follows 1) Normal TSH level was 14.8±7.8 µU/ml, 8.8±4.7 µU/ml, and 6.5±2.7 µU/ml in neonates of age 0 〜24 hours, 24—48 hours, and 48〜72 hours respectively, and all TSH values in neonates of age 48〜72 hours were...
A Case of Familial Dysautonomia.
Hong Jin Lee, Dong Kyu Jin, Sei Won Yang, Jeong Kee Seo, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1988;31(5):648-654.   Published online May 31, 1988
Few documented cases of Familial dysautonomia fulfilling current diagnostic criteria have been recognized in non-Jews especially in orientals. In our case diagnosis was established in 8 year old Korean girl. She fulfilled 8 out of 9 essential diagnostic criteria of Riley. It represents a report of this syndrome with achalasia and improved with modified Heller’s myotomy.
The Prevalence of Antithyroid Autoantibodies in Korean Children.
Bung Hai Ahn, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1988;31(3):329-338.   Published online March 31, 1988
The prevalence of antithyroid autoantibodies and the relationship between the presence of autoantibodies and thyroid functions were studied in 752 Korean children of 10 to 15 years of age with normal thyroids. Antimicrosomal antibodies (AMA) were measured by the indirect agglutination technique using artificial gelatin particle carriers sensitized with thyroid microsomal antigen and antithyroglobulin antibodies (ATA) by the tanned red cell hemagglutination technique. Circulating AMA...
A Case of Type A Niemann Pick Disease.
Hyo Nam Cho, Hong Jin Lee, Jae Won Song, Jung Hwan Choi, Hyung Ro Moon, Je Geun Ji, Myeong Hee Park
Clin Exp Pediatr. 1987;30(12):1461-1467.   Published online December 31, 1987
We experienced a case of type A Niemann Pick disease in 13 month female baby. She showed marked hepatosplenomegaly, developmental regression and apathy. Fundoscopic examination revealed a cherry red spot in the macula. Numerous foamy histiocyte were seen in bone marrow. Autopsy finding revealed that liver, spleen, thymus, lymph node, lung and brain were involved. Liver showed fatty change. Generalized muscular atrophy and patch...
Study on the Korean Infant Development Screening Test.
Jong Lin Rhi, Jung Hwan Choi, Hyung Ro Moon, Sechin Cho
Clin Exp Pediatr. 1987;30(8):852-867.   Published online August 31, 1987
Developmental problems are relatively prevalent and often treatable if early interventions are to be given. Much better prognosis will also be given if they are treated early & properly by early detection through the use of the developmental screening test. Developmental screening is the application of relatively quick and simple procedures to an asymptomatic population to select out those individuals who are highly likely...
Case Report
A Case of Methylmalonic Acidemia.
Hyung Ro Moon, Hae Rim Jung, Mi Ryung Um
Clin Exp Pediatr. 1987;30(7):791-796.   Published online July 31, 1987
Methylmalonic acidemia is an inborn error of metabolsim, transmitted as an autosomal recessive traits. Symptoms begin early in life and they are pernicious vomiting, lethargy, hepatomegaly, and failure to thrive. Laboratory finidngs are ketosis, intermittent hyperglycinemia, and methymalonic aciduria without vitamin B12 deficiency. Six distinct subtypes of this disorders have been characterized. Some of them respond well to exogenous vitamin B12 therapy. We have experienced a...
A Case of Gaucher's Disease.
Hyo Nam Cho, Myung Cheol Cho, Hyung Ro Moon, Je Geun Chi, Hyo Min Kim
Clin Exp Pediatr. 1987;30(7):784-790.   Published online July 31, 1987
We experienced a case of Gaucher’s disease of acute infantile type in a 12 month old male boy. The patient showed hepatosplenomegaly with anemia and thrombocytopenia, developmental delay and frequent infection. There were characteristic Gaucher’s cells in bone marrow aspiration and biopsy of liver, spleen, & lymph node. Splenectomy was done at 25 month old. He expired 3 days after splenectomy. Autopsy was done....
Original Article
Clinical Approach to Dysmorphic Syndromes.
Hyung Ro Moon
Clin Exp Pediatr. 1987;30(7):707-711.   Published online July 31, 1987
A Clinical Observation on Fulminant Hepatitis in Children.
Hoan Jong Lee, Jeong Kee Seo, Hyung Ro Moon
Clin Exp Pediatr. 1987;30(4):406-415.   Published online April 30, 1987
Twenty-seven cases of fulminant hepatitis in children were retrospectively analyzed. The overall survival rate was 11%. The interval between onset of encephalopathy and death, and that between jaundice and death were 2-26 days (median 4 days), 4-61 days (median 14 days), respectively. HBs antigenemia was detected in 24%, HAV IgM was negative in 5 patients tested, and 4 cases were suspected as non-A, non-B...
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...
Pheochromocytoma in Two 10-year Old Children.
Young Soo Kim, Young Hoon Kim, Se Won Yang, Yong Choi, Hyung Ro Moon
Clin Exp Pediatr. 1987;30(2):207-211.   Published online February 28, 1987
We experienced two cases of pheochromocytoma in 10 year-old boy and girl. Boy had bilateral and girl had unilateral pheochromocytoma. Both patients who were severely retarded in height growth had hypertension and one patient had cardiomyopathy. In both patients, vanillylmandelic acid in urine was increased, and phentolamine test was positive. Adrenal mass was visualized by C.T. in each ease. Successful surgical removal was done,...
Russell-Silver Syndrome.
Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(7):715-722.   Published online July 31, 1986
Seventeen cases(12 boys and 4 girls) with Russell-Silver syndrome were evaluated for weight, height and bone age at initial presentation and during follow up. 1) Distribution of patient` s age at initial presentation was between 1 day and 75/12 year of age. 2) In 14 cases, the birth weight was below 3 percentile except for one case(3 percentile). In 15 cases, the...
Congenital adrenal Hyperplasia.
Byoung Hai Ahn, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(6):604-612.   Published online June 30, 1986
Clinical data of twenty-eight children with congenital adrenal hyperplasia who were admitted to the Seoul National University Hospital from June 1979 to July 1984 were analyzed retrospectively. Male to female ratio was 1 : 1.3. The average age at diagnosis was 3.2 years, 1.3 months for eleven salt-losers and all were 3 months or younger, 4.6 years for seventeen nonsaltlosers....
Case Report
Three Cases of Familial Glucocorticoid Deficiency.
Soo Heum Lim, Young Wun Kim, Byoung Hai Ahn, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(5):549-552.   Published online May 31, 1986
Familial glucocorticoid defiency is a form of chronic adrenal insufficiency which is characterized by isolated deficiency of glucocorticoid and elevated levels of corticotropin in association with normal aldosterone production. It is thought to be degenerative process of adrenal cortex, with autosomal recessive or X-linked recessive pattern of inheritance. Skin hyperpigmentation and hypoglycemic symptom are characteristic and salt-losing symptom doesn’t develop....
A Case of the Dubowitz Syndrome.
Kyung Ae Lee, Chi Ho Yun, Jae Sun Jung, Hee Ju Kim, Sung Ill Ahn, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(4):436-440.   Published online April 30, 1986
The Dubowitz syndrome is an autosomal recessive condition characterized by intrauterine growth retardation, postnanal growth retardation, peculiar facial appearance, short stature, low birth weight, microcephaly and borderline intelligence or mild mental retardation. We reported a typical case of the Dubowitz syndrome in a full term male infant together with a brief review of pertinent literatures since the incidence of the...
Hepatoblastoma with Rupture and Hemorrhage.
Kwang Wook Ko, Hyung Ro Moon, Je Geun Chi, Kwi Won Park
Clin Exp Pediatr. 1985;28(12):1261-1261.   Published online December 31, 1985
A Case of Niemann-Pick Disease with Sea-Blue histiocytes in the Bone Marrow.
Young Sun Kim, Soo Heum Lim, Jeong Kee Seo, Hyo Seop Ahn, Hyung Ro Moon
Clin Exp Pediatr. 1985;28(12):1238-1244.   Published online December 31, 1985
A case of Niemann-Pick disease in a 12 year and 6 month old Korean male was reported. The patient showed marked hepatosplenomegaly without neurologic symptoms. Fundoscopic examination revealed a cherry-red spot with white ring in the maculae. Numerous foamy histiocytes and sea-blue histiocytes were seen in the bone marrow. The liver biopsy specimen showed the diffuse ballooning of hepatocytes obliterating...
Two Case of Watson-Alagille Syndrome.
Heon Seok Han, Soo Heum Lim, Young Sun Kim, Jeong Kee Seo, Hyung Ro Moon
Clin Exp Pediatr. 1985;28(10):1047-1051.   Published online October 31, 1985
Watson-Alagille syndrome (Arteriohepatic dysplasia, Syndromatic paucity of interlobular bile duct), a disease characterized by chronic liver disease, characteristic facies, cardiova scular abnormalities, and vertebral arch abnormalities has paucity of interlobular bile ducts on histologic examination of liver. We experienced two cases of Watson-Alagille syndrome in a 7-month-old male infant and a 12-month-old male infant who had the chief complaint of...
Two Cases of the Rud Syndrome in Siblings.
Mi Jeong Lee, Myung Geul Yum, Jung Hwan Choi, Chong Ku Yoon, Hyung Ro Moon
Clin Exp Pediatr. 1985;28(6):610-615.   Published online June 30, 1985
The Rud syndrome, which is characterized by ichthyosis, mental retardation, seizure, dwarfism, sexual infantilism, etc., was described by Rud in 1927 at first. It is hereditary disorder whose mode of inheritance is autosomal recessive. Because the incidence of this syndrome is very rare, the Rud syndrome has not been reported till now in Korea. We experienced two cases of the...
Hypothyroid Myopathy.
Kawng Wook Ko, Je Geun Chi, Hyung Ro Moon
Clin Exp Pediatr. 1984;27(11):1147-1150.   Published online November 30, 1984
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 Glycogen Storage Disease Type IIa.
Dong Hyung Choo, Dong Kyoon Kim, Hyung Ro Moon, Choong Kon Kim
Clin Exp Pediatr. 1984;27(6):621-627.   Published online June 30, 1984
Glycogen storage disease type Ha(GSD la), also known as generalized glycogenosis, cardiomegalic glycogenosis, and Pompe disease, is a very rare form of glycogenosis. The authors reported a case of GSD J a in an eight months old girl who presented with respiratory problems, marked hypotonia, cardiomegaly, and macroglossia. The diagnosis was made on clinical grounds including pathologic findings of muscle...
Adrenal Cortical Carcinoma in a Nineteen Month Old Girl.
Sei Weon Yang, Jong Jin Seo, Hyung Ro Moon
Clin Exp Pediatr. 1984;27(4):413-418.   Published online April 30, 1984
Adrenal carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as Cushing syndrome, virilization, feminization or precocious puberty. The authors report a nineteen month old girl who exhibited features of Cushing syndrome and virilization caused by adrenal cortical carcinoma measuring 5.5X5.0X2.8 cm. She was successfully...
Hereditary Hemorrhagic Telangiectasia with Pulmonary Arteriovenous Fistula.
Heui Jeen Kim, Young Soo Yoon, Jeong Kee Seo, Hyung Ro Moon, Kyung Mo Yeon
Clin Exp Pediatr. 1984;27(4):390-394.   Published online April 30, 1984
Four Cases of Papillary Thyroid Cancer in Childhood.
Sei Weon Yang, Sei Won Park, In Sil Lee, Hyo Seup Ahn, Hyung Ro Moon, Chang Yee Hong, Kwi Won Park, Je Geun Chi
Clin Exp Pediatr. 1984;27(3):282-287.   Published online March 31, 1984
Papaillary thyroid cancer is a rare lesion in children. It progresses slowly and its prognosis is relatively good. A painless nodule in the thyroid or in the neck is the usual first evidence of disease. Cervical lymph node involvement is usually present at the time of the initial diagnosis and is often bilateral. The lungs are the most common site...
A Case of Treacher Collins Syndrome.
Hee Shang Youn, Koo Soo Kim, Hyung Ro Moon
Clin Exp Pediatr. 1984;27(2):207-212.   Published online February 28, 1984
The authors report a 5-year old boy who showed the abnormalities comprising relatively complete from of Treacher Colilns syndrome. These include hypoplasia of malar and mandibular bones, antimongoloid slant of palpebral fissures, colobomata of lower eyelids with defective eyelashes on medial sides, projection of scalp hair onto lateral cheeks, malformed auricles, complete atresia of both external auditory canals with defective...