• Pediatric Infection and Vaccine
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• v.3 no.2
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• pp.154-162
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• 1996

Purpose : Adenoviruses(Ad) have been shown to play an important role in the etiology of severely acute respiratory diseases, particulary in infants and young children, and the occurrence of fatal outcome and chronic pulmonary sequelae in association with adenoviral infection has been a cause of great interest and concern. This report presents the resul of a retrospective analysis on 30 cases of lower respiratory infection from which adenovirus was isolated. Patients & Methods : The 30 patients in this study represent all detected cases of adenovial infection out of 240 children who were admitted to Sang Sung Medical Center between February to June 1996 showing signs and symptoms of lower respiratory tract infection. The diagnosis of adenovirus infection was based on microscopic visualization of typical cytopathic effect in HEp-2 tissue culture and used monoclonal Ab with nasopharyngeal aspiration. Results : The male/female ratio was 2:1 and the majority of age range was below 36months. Clinical diagnoses in all 30 patients were pneumonia(n=21), bronchitis and Bronchiolitis(n=5) and ARDS(n=4). We recieved the most of patients in the month of May. The chief complaints were fever(93.3%) and cough(80%) and extrapulmonary symptoms were diarrhea(n=5), seizure(n=4), abdominal pain(n=1). The mean duration of fever was $11.95{\pm}6.54$days. Physical examination on admission were crackles(73.3%), coarse breathing sounds(60%), hepatosplenomegaly(33.3%), decreased brething sounds(30%). In WBC counts, 8cases were below $4000/mm^3$ and 14 cases were above $10,000/mm^3$. In platelets counts, 4cases were below $150,000/mm^3$ and 10 cases were above $450,000/mm^3$. 21 cases were above 1 in CRP. GOT and GPT were abnormal in some cases. Chest X-ray revealed diffuse pulmonary infiltration(n=15), pleural effusion(n=6), consolidation(n=4) and hyperaeration(n=3). Seven patients were treated at the peditric intensive care unit with respiratory support and high dose of gammaglobulin. However, one patients died even through he was treated with NO ventilation and high frequency ventilation. Conclusion : Those with adenoviral pneumonia and respiratory infection having long fever duration and symptoms like bacterial pneumonia must be carefully differentiated in order to provide proper treatement and preventive measures due to possible fatal outcome.

• Journal of Nutrition and Health
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• v.5 no.2
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• pp.91-103
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• 1972

Zinc is one of the essential trace elements in the living organism for growth and health. The first identified metalloenzyme, carbonic anhydrase, is a zinc compound and several others have been described since. Among zinc deficiency syndromes in animals porcine parakeratosis has been successfully treated with zinc supplements, and in man a syndrome of anemia, hypogonadism, hepatosplenomegaly, and dwarfism, prevalent in parts of Iran and Egypt, has been ascribed to lack of zinc in the diet. Dietary zinc excess in the rat is manifested by a hypochromic, microcytic anemia, poor growth, reduction in liver catalase and cytochrome oxidase. The present study is an attempt to delineate the changes of tissue contents of trace elements, especially of iron, copper and zinc in liver and kidneys of the rats. Weanling albino rats, weighing 60 to 80gm. were used in this experiments. The rats were housed in cages with aluminum floors and received feed and distilled water ad libitum. Animals were divided into three groups, control, low zinc diet and high zinc diet groups. The high zinc diet group was subdivided into 0.5% Zn and 0.7%Zn groups. The supplementary copper or iron was added to the high dietary zinc groups. The animals were sacrificed and the tissues were washed several times with deionized water. The wet digested samples were analyzed by Hitachi Model 207 atomic absorption spectro-photometer for the determination of iron, copper and zinc in the liver and kidney. Hemoglobin level in the blood was measured by cyanmethemoglobin method. The results of this study are as follows: 1) All rats fed high zinc diets and low zinc diets gained less weight than control. Weight gain was not improved by the supplementary copper or iron and both. 2) Hemoglobin concentration was decreased significantly in the rats fed high zinc diets and less in the low zinc diet. Supplementary copper and iron to the higher zinc diet appeared to give some improvement of anemia. 3) The iron contents of the liver and kidneys were significantly decreased in the high zinc groups and the reduction was more significantly in the rats receiving higher zinc diet (0.7%). The supplementary copper caused a further depression of liver iron. On the other hand, the iron, added to the high zinc diet lessoned the severity of the decrease in liver iron and caused kidney iron to be maintained almost at the level found in the rats fed by zinc and supplementary copper diet. 4) High zinc diets did not change copper content of the liver and kidney. Supplementary copper elevated the concentration in the liver and kidney and added iron had no effect on the accumulation of copper in the liver and kidneys. 5) The high zinc diets caused marked increases of zinc content in the liver and kidney. Supplementary iron to the high zinc diet caused increases of zinc contents of liver and kidneys.

• Kosin Medical Journal
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• v.33 no.3
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• pp.347-357
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• 2018

Objectives: Few studies of pediatric Epstein-Barr virus (EBV)-associated infectious mononucleosis (IM) have been conducted in Korea. We evaluated the clinical features of children with IM to define differences according to age. Methods: We conducted retrospective chart reviews of 68 children aged 0 to 15 years who were diagnosed by EBV-associated IM with EBV-Viral Capsid Antigen(VCA) IgM at laboratory test and were admitted between 2010 and 2014. The children were classified into four age groups: aged 0-3, 4-6, 7-9, and 10-15 years. Results: The age distribution of patients was as follows: 19 (27.9%) 0-3, 25 (36.8%) 4-6, 13 (19.1%) 7-9, and 11 (16.2%) 10-15. Fever was the most common presentation regardless of age. It was more common in the 0-3 group than the 4-6 group (P = 0.018). Pharyngitis was more common in the 7-9 group than the 0-3 group (P = 0.048), and myalgia was more common in the 10-15 group than the 0-3 group (P = 0.007). Pharyngitis was accompanied by lymphadenopathy, protracted fever, and rash. In the 0-3 age group, the prevalence of rash was higher while the percentage of atypical lymphocytes was lower, but there was no statistical support for this tendency. There were no differences in the frequency of hepatosplenomegaly or laboratory findings between age groups. Conclusions: IM is not uncommon in young children and its clinical presentation varies with age. Therefore, IM should be suspected in young febrile children with pharyngitis and rash despite low percentages of atypical lymphocytes.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.213-221
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• 2005

Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.

• Pediatric Infection and Vaccine
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• v.5 no.1
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• pp.69-78
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• 1998

Purpose : The risk of severe tuberculous disease such as meningitis or miliary tuberculosis increases as younger is the child at the time of infection. Therefore, the early diagnosis and prompt treatment is mandatory for infants with tuberculosis. This study was undertaken to describe the epidemiology, clinical and radiographic manifestations, and response to therapy in infants with tuberculous disease. Methods : Medical records of 29 infants with tuberculosis diagnosed at the Seoul National University Children's Hospital from July, 1985, to April, 1997, were reviewed, retrospectively. A case of tuberculosis was confirmed if M. tuberculosis was isolated from any body site or if there was histologic proof of tuberculosis. Otherwise, the diagnoses were individualized considering history of contact with contagious adult case, clinical manifestations, chest X-ray findings, result of a Mantoux test reaction with 5 tuberculin unit of PPD, and the response to therapy. Results : The mean age at diagnosis was $7.00{\pm}2.65$ months (range, 3 to 12 months). Twelve cases had isolated pulmonary diseases, and the rest had pulmonary disease and meningitis, 5 cases; pulmonary disease and cervical lymphadenitis, 3; isolated meningitis, 3; and miliary tuberculosis, 6. Source case was identified in 19 cases, 7 of which were detected with retrograde manner. Twenty seven of 29 were symptomatic at their initial visit. The presenting symptoms were mainly respiratory or neurologic, and respiratory difficulty was accompanied in 7 cases. Physical examination revealed wheezing in 7 cases and decreased breath sounds in 9. Hepatomegaly or hepatosplenomegaly were frequent. Chest radiographs showed lung parenchymal disease with hilar lymphadenopathy in 18 cases, and focal or generalized emphysematous change in 7 cases. Conclusion : Most of the infants with tuberculosis are symptomatic at diagnosis, and many of infants with intrathoracic tuberculosis presented with symptoms of bronchial obstruction. When tuberculosis is suspected in an infant, the adult source case should be vigorously investigated to aid in diagnosis and for the prevention of further transmission of tuberculous disease. Almost half of infant tuberculosis are preventable if prophylaxis were given when adult cases were diagnosed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.28-35
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• 2007

Purpose: The aim of this study was to evaluate the clinical usefulness of ultrasound examination of children performed by a pediatrician. Methods: One thousand children who presented with symptoms of a gastrointestinal disorder and underwent abdominal ultrasound evaluation in the Department of Pediatrics, between January 2003 and June 2006, were included in this study. We analyzed the patient's medical records and ultrasound results retrospectively. Results: Among the 1,000 patients, 58.4% were male and 41.6% were female. The mean age of the patients was $4.7{\pm}4.0$ years. The main reasons for ultrasound were abdominal pain (43.9%), vomiting (17.3%), elevated liver enzymes (11.8%), and jaundice (9.8%). Abnormal ultrasound findings were present in 57.9% of cases. The major abnormal findings were mesenteric lymphadenitis (29.2%), fatty liver (12.1%), hepatitis (6.4%), hepatosplenomegaly (6.2%), and acute appendicitis (4.8%). The time interval between the initial medical evaluation and the ultrasound evaluation was within 24 hours in most cases (78.5%). The main findings in children with abdominal pain were mesenteric lymphadenitis (32.6%), fatty liver (5.9%), intussusception (2.7%), and acute appendicitis (2.7%). The main findings in children with vomiting were mesenteric lymphadenitis (12.7%), hypertrophic pyloric stenosis (10.4%), and acute appendicitis (3.5%). The major ultrasound findings in children with urinary tract diseases were hydronephrosis (45.4%), urolithiasis (21.5%) and cystic renal disease (18.1%). Conclusion: Ultrasound examination played an important role as a non-invasive and prompt screening examination for detection of abdominal diseases. Ultrasound was an important tool for pediatricians to determine timely information for patient management.

• Journal of Yeungnam Medical Science
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• v.18 no.2
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• pp.239-245
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• 2001

Background: Acute idiopathic thrombocytopenic purpura(ITF) is one of the common hematologic disorders in children. Bone marrow aspiration (BMA) is often performed in children with acute ITP to rule out leukemia, aplastic anemia or other hematologic diseases. However, whether BMA is needed in children with typical clinical and hematological features of acute ITP have been questioned. This study was performed to examine the proper indication of BMA in acute childhood ITF. Materials and Methods: The medical records and BMA reports of children with the provisional diagnosis of acute ITP were reviewed from January 1984 to December 2000. Patients were divided into two groups, one with typical and another with atypical clinical and hematological features of acute ITP. Typical acute ITP group was characterized by the history of previous viral infection, well being appearance, no hepatosplenomegaly, no lymphadenopathy, normal Hb, WEC, neutrophil count and peripheral blood smear except thrombocytopenia. A platelet count of $50{\times}l0^9/L$ or lower was the cutoff level. Results: Total 120 children with the provisional diagnosis of acute ITP were included. One hundred eighteen of them were confirmed to have acute ITP by BMAs. Of these, 66 had typical and 54 had atypical features. All of typical features and 52 of 54 with atypical features of acute ITP were confirmed to have acute ITP by BMAs. Two patients with atypical features of acute ITP were diagnosed as aplastic anemia and myelodyspalstic syndrome, respectively, by BMAs. Conclusion: This study concludes that BMA is not needed for the children with typical features of acute ITP but it is needed for the children with atypical features of acute ITP to rule out other hematologic disorders.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.2
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• pp.55-62
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• 2020

A 22-month-old girl who had taken iron supplements due to iron deficiency anemia, presented bloody mucoid stool for one month. She had a bruise at the right periorbital area due to minor trauma and hepatosplenomegaly. Laboratory studies showed anemia, thrombocytopenia, elevated alkaline phosphatase (ALP), hypophosphatemia, decreased haptoglobin, hypocomplementemia, negative direct/indirect Coomb's test, normal vitamin D3 level and high PTHi. Wrist x-ray showed no signs of rickets. The abdominal ultrasound showed only accessory spleen. Tandem mass spectrometry was normal. During follow up, bloody stool regressed after seven days of withdrawal of iron supplement and cow milk, and the total CO2 level had been within 15-20 mEq/L with normal anion gap. NGS (next generation sequencing) panel test for evaluation of renal tubular acidosis showed negative results. After low dose steroid and vitamin D supplements under the impression of hypocomplementemic vasculitis, thrombocytopenia, C3/C4, decreased haptoglobin, and elevated ALP level became normal. At 57 months of age, laboratory findings showed elevated liver enzyme, ALP and gamma-glutamyl transferase again. And liver cirrhosis with splenomegaly and diffuse renal disease were reported with abdomen CT scan. Liver biopsy reported macro- and micronodular cirrhosis. Urine organic acid profile showed elevated succinylacetone level. Whole exome sequencing revealed novel compound heterozygous mutations (NM_00137.2:c.107T>C, NM_00137, 2:c.614T>C) in FAH gene and confirmed by Sanger sequencing. Consequently, the patient was diagnosed as chronic hereditary tyrosinemia type I. She started low phenylalanine/tyrosine diet and nitisinone treatment. Our case had presented symptoms very slowly, which is the first case of chronic tyrosinemia type I in South Korea.