We experienced a case of acute megakaryoblastic leukemia (M7) with Down syndrome. The patient was admitted due to premature SGA, which revealed characteristic facial figure of Down syndrome. M7 was diagnosed with PB smear which showed abundant megakaryoblast and confirmed by using the monoclonal antiplatelet glycoprotein IIb/IIIa antibody (J 15). On 25th hospital day, the patient gained weight but discharged without further treatment of M7. Authors... |
In a prospective investigation of 21 chi Idem 3 years of age or more with Mycoplasma pneumoniae pneumonia, we analyzed the diagnostic efficacy of High Density Composite Particles Agglutinin(HDDA) test comparing with cold agglutinin tests and clinical findings. The results obtained were as followed. 1) Among 24 total cases, the number of cases showing positive reaction to HDPA and to cold agglutination test were 17 and... |
The purpose of this study was to determine the relationship between improvement of pulmonary, function and diuresis in respiratory distress syndrome. This study was based on 24 cases of RDS infants admitted in NICU of Ewha Womans University from January 1988 to December 1990. The results were as follows. 1) Mean birth weight of study cases was 2.11 kg with gestational age between 29〜39 weeks. 2) Maximum... |
The diagnosis of renal osteodystrophy and osteoporosis is established by clinical manifestations, biochemical laboratory tests, X-ray and bone scan but early diagnosis, determination of treatment of various metabolic bone diseases are not satisfied with any above methods. Dual energy X-ray absorptiometry (DEXA) has been shown to be a reliable, reproducible, and noninvasive way to assess bone mineral status. We evaluated the bone mineral density... |
Acrdermititis enteropathica is a rare autosomal recessive disorder of zinc absorption and its cardinal symptoms appear at the time of weaning from breast milk which has been referred to as most appropriate formula to prevent and treat this disease. However, recent reports presented Transient Symptomatic Zinc deficiency (TSZD) in infants fed breast milk whom they treated with transitory zinc supplement with no recurrence. We experienced... |
35 childhood insulin-dependent (Typel) diabetes mellitus were typed for HLA and examined for presence of islet cell antibody and level of C-peptide. The following results were obtained: 1) HLA types were present as follows; HLA A24 (9) in 58%, DR3 in 38% and DR4 in 62%. This was slightly higher than the Korean population of 38%, 7% and 48% but there was no significant difference. 2) When... |
Neonatal asphyxia is insult to the newborn due to lack of oxygen or perfusion to various organs. It is associated with tissue hypoxia, especially brain. So hypoxic-ischemic brain injury is the most important complication known as hypoxic-ischemic encephalopathy. The EEG as well as various evoked potentials (auditory, visual and somatosensory) may assist in evaluating the severity of the damage. This study was undertaken to... |
The syndrome of Mixed Gonadal Dysgenesis is characterized by a unilateral testis, usually intra- abdominal a streak gonad on contralateral side, persistent Mullerian structures and ambiguous genitalia. These patients are chromatin negative and exhibit XO/XY mosaicism, probably resulting from a cytogenetic error very early in embryogenesis. The testis and the streak gonad should be removed because of the potential devlopement of a gonadoblastoma and the virilization... |
We experienced a case of Adrenoleukodystrophy (ALD), which is a genetically determined metabolic disorder associated with progressive demyelination of brain white matter and adrenal insuficiency. An 11 year old male revealed rapidly evolving neuologic progression, decreased adrenal reserve, increased plasma content of very long chain fatty acids (VLCFAs) and typical radiological findings. We report here the clinical findings of this patient with brief review of... |
Among those who were admitted to the Pediatric Intensive Care Unit (PICU), Seoul National University Children’s Hospital and developed acute renal failure (ARF) during the disease course, 11 children received continuous arteriovenous hemofiltration (CAVH) by pediatricians during a 3-year- period from Nov. 85 to Oct. 88. Their clinical findings were analyzed retrospectively, and the results were as follows; 1) They were 5 boys and 6... |
Visual.Motor Integration (VMI) test, designed by Beery, is one of the battery, which is commomly used in investigating the developmental status of child. As the test was designed for American children, the test may be not feasible for Korean children because of difference of cultural back. ground, race as well as econornic status. 1674 normal Korean children aged from 5 to 12 were tested... |
The 267 cases of meningitis were observed at National Medical Center between Jan. 1st 1979 and May 31, 1986. The results were as follow; 1) In BM and TM sexual difference was not notice, but male was more in AM. 2) In BM infancy was the most common, but school age was common was AM. 3) Most cases developed during spring in BM and TM... |
Desquamative interstitial pneumonia (DIP) is a variant of idiopathic interstitial pneumonias, but characteristicalIy has massive desquamation and proliferation of alveolar cells into the distal air space with minimal fibrosis of the alveolar wall. DIP seems to have better response to corticosteroid and benign prognosis than idiopathic interstitial pneumonia. We experienced one case of 6-year old girl who showed typical picture of DIF on lung... |
BRE, probably the commonest varierity of partial epilepsy in childhood has 6 characteristics; it occurs in children, without neuropsychiatric antecidents, never related to progressive cerebral lesion, interictal EEG shows Rolandic spikes, the seizure are varied and there is always recovery in puberty at the latest. We reviewed 70 patients with BRE to observe the age and sex distribution, EEG findings, clinical sign and symptoms, treatment... |
The clinical study on 28 cases of ABO HDN who were diagnosed at Pediatric Department of Severance hospital and Yong Dong Severance hospital, Yonsei University for last 5 months was. And in these cases, heat elution test in combined with direct Coombs test using standard techniques were performed to evaluate the clinical significance of serological diagnosis. The following results were obtained. 1) Of 28 ABO HDN... |
Two asthmatic children associated with allergic rhinitis were performed nasal provocation test with various allergens which were detected by allergic skin test. The degree of nasal obstruction induced by allergens were measured with Youlten’s Peak Nasal Inspiratory Flow (PNIF) meter and Red top,Trichophyton and Sagebrush were known as causative allergens in case 1, and Chrysanthemum was detected as causative allergen in case 2. The... |
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... |
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... |
We have experienced a case of renal candidiasis in type I DM (IDDM). The patient was a 15-year old girl who suffered from type I DM since 3 years earlier with development of retinopathy, cataract & renal failure. Ultrasonic examination revealed multiple fungus balls in renal pelvis & urinary bladder. Candida tropicalis was cultured from fungus balls passed during urination. A brief... |
This study was undertaken to evaluate the significance of brain CT in children with meningitis. The findings of brain CT in 71 children with meningitis were evaluated in relation to the clinical outcome. We divided 71 cases into 5 groups according to clinical outcome on discharge, that is, Group I: Recovery. Group II: Discharge with improvement. Group III: Discharge with residual sequalae. Group IV:... |
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... |
Partial trisomy 10(q22-q26) is a extremely rare chromosomal anomaly. The authors experienced a case of this syndrome in a female newborn infant. She showed prominent occiput, flat face, blepharophimosis, hypertelorism, lowset ears, micrognathia, high-arched palate, simian crease on Lt. palm & overlapping of the 5th finger over the 4th finger, relatively great big toe, and single umbilical artery.Chromosomal study showed the abnormal chromosome... |
Unilateral Absence of Pulmonary Artery(UAPA) is a rare disease. It is characterized by no specfiic cardiopulmonary symptoms in general. We experienced a case of UAPA in a 9 year-old female patient with the chief complaint of abnormal chest Xray finding. Lung perfusion scan, cardiac catheterization with pulmonary and aortic angiography confirmed the diagnosis of right side UAPA. |
143 cases(106 males, 37 females) with asthmatic children (aged from 54/i2 to 144/i2 year old) were performed pulmonary function test before and after exercise to detect exerciseinduced asthma (El A). If peak expiratory flow rate(PEFR), checked with Wright’s peak flowmeter, decreased 15% cr more after exercise loading test (up and down stair method for 6 minutes), the result was considered to... |
Osteogenesis imperfecta is an inherited disorders of connective tissue that affects the skeleton, ligament, sclera, and dentin. Characterized clinical manifestation are multiplefracture, blue sclera, deafness, joint laxity, thin skin, and odontogenesis imperfecta. We experienced acase of osteogenesis imperfecta in a one day old male newborn who chiefly complain respiratory distress, triangular shaped head and fracture and deformity of lower extremity.... |
We have experienced a case of primary lung cancer (squamous cell ca.) in a 15 years-old boy who presented with productive coughing and mild fever. The diagnosis was made through thoracic roentgenogram, thoracotomy, and biopsy. A brief review of the related literature is given. |
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... |
We experienced a case of Crouzon* s disease in a 11 year 8 month-old boy with chief complaints of facial deformities and progressive impairment of visual acuity. Physical examination showed craniosynostosis, bilateral exophthalmos, external strabismus, birdlike beaked nose, high-arched palate, hypoplasia of maxilla, prognathism of mandible and dental malocclusion. The review of the literature was made briefly. |
Bone marrow cell cultures (GM-CFU) were performed in the 15 cases of children with aplastic anemia and the correlation with the clinical course were investigated. They have been treated with androgen with or without low dose prednisone and supportive care. Bone marrow cell cultures from 8 children without any evidence of aplastic anemia were performed as a control. 1)In control... |
We observed a case of congenital nonspherocytic hemolytic anemia who was a 24 month old boy with the signs of jaundice 技 pallor. Evidences of the same disease was also found in the patient' s elder brother & father. We diagnosed these cases by history & physical examination, peripheral & B.M. blood smears, osmotic fragility test, autohemolysis test and etc. A... |