• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Tuberculosis and Respiratory Diseases
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• v.45 no.5
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• pp.1058-1066
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• 1998

Background: Actinomycotic infection is uncommon and primary actinomycosis of the lung and chest wall has been less frequently reported. This disease may present as chronic debilitating illness with radiologic manifestation simulating lung tumor, pulmonary infiltrating lesion, or chronic suppuration. Diagnosis of choice was not definded yet and role of bronchoscopy on diagnosis was not described yet. Methods: From 1989 to 1998, we experienced 17 cases of thoracic actinomycosis. We have reviewed the case notes of 17 patients with thoracic actinomycosis. The mean age at presentation was $53{\pm}13$ years, 11 were male. Results: Cough, hemoptysis, sputum production, chest pain and weight loss were the commonest symptoms. The mean delay between presentation and diagnosis was $6.6{\pm}7.8$ months. There were six patients who presented with a clinical picture of a suppurative lesion and eleven patients were suspected of having primary lung tumor initially. In no cases was made an accurate diagnosis at the time of hospital admission. Associated diseases were emphysema (1 case), bronchiectasis (2 cases) and tuberculosis (2 cases). Bronchoscopic findings were mucosal swelling and stenosis(n=4), mucosal swelling, stenosis and necrotic covering (n=2), mass (n=3), mass and necrotic covering (n=1) and normal(n=6). Radiologic findings were mass lesion(n=8), pneumonitis(n=3), atelectasis(n=3), pleural effusion(n=2), and normal(n=3). Final diagnosis was based on percutaneous needle aspiration and biopsy (n=3), bronchoscopic biopsy specimens (n=9), mediastinoscopic biopsy (n=1) and histologic examination of resected tissue in the remaining patients(n=4) who received surgical excision. Among 17 patients, 13 were treated medically and the other 4 received surgical intervention followed by antibiotic treatment. Regarding the surgically treated patients, suspected malignancy is the most common indication for operation. However. both medically and surgically treated patients achieved good clinical results. Conclusion: Thoracic actinomycosis is rare. but should still be considered in the differential diagnosis of a chrinic, localized pulmonary lesion. Thoracic actinomycosis may co-exist with pulmonary tuberculosis or lung cancer. If the lesion is located in the central of the lung. the bronchoscopy is recommanded for the diagnosis.

• Pediatric Infection and Vaccine
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• v.14 no.2
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• pp.145-154
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• 2007

Purpose : Rotavirus is one of the most important etiologic agents of nosocomial infections among the neonates. This study was designed to investigate nosocomial rotavirus infection in neonates who were admitted to a postpartum-care center after birth. Methods : From March 2005 to September 2006, 957 healthy neonates were examined for rotavirus antigen in stool by immunochromatographic method and 216 neonates were rotavirus antigen positive within 24 hours after admitted to a postpartum-care center. We reviewed the nursing charts retrospectively such as characteristics, monthly distribution, birth hospitals, delivery methods, feeding types and clinical manifestations. Results : Among 957 neonates, 216 neonates (22.6%) were rotavirus antigen positive and there were no differences in sex, birth weight, gestational age. Monthly positive rate of rotavirus antigen showed diversity from 10% to 36%. According to birth hospitals, positive rate showed diversity from 3.5% to 53.6%. Out of 957 neonates, 655 cases (68.4%) were born of vaginal delivery and mean hospitalized duration was 2.4 days, 302 cases (31.6%) were born of cesarean section and mean hospitalized duration was 5.7 days. 17.6% of vaginal delivery and 33.4% of cesarean section were rotavirus antigen positive. The positive rate was higher in neonates by cesarean section than vaginal delivery (P<0.001). According to feeding types, positive rate of rotavirus antigen was lower in breast-fed group than formula-fed group (P<0.001). Proportion of symptomatic case among rotavirus antigen positive was 34.7%. Most common clinical manifestation was diarrhea (61.3%), following poor feeding (45.3%), fever (40.0%), vomiting (25.3%), delayed weight gain (12.0%), and decreased urine amount (5.3%). Conclusion : Some neonates were already infected before admission to a postpartum-care center. Without meticulous management, nosocomial rotavirus infection would transmit rapidly in a postpartum-care center spreading to the community. Recommendation of breast-feeding, routine rotavirus screeing test with or without symptom, and isolation of all rotavirus antigen positive neonates in a postpartum-care center seem to be necessary. Also attentive hygiene education and further investigations of rotavirus infection in a postpartum-care center would be needed.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.832-838
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• 2005

Purpose : Mycoplasma pneumoniae infection is relatively common in childhood. Its extrapulmonary manifestations have been reported so much, but hepatitis associated with it has been reported rarely in Korea. Methods : A clinical study was performed on 556 patients of M. pneumoniae pneumonia diagnosed serologically at Gil hospital from January 2001 to December 2004. We reviewed 65 cases among these patients, who had elevated level of serum AST and ALT greater than 50 IU/L respectively without evidence of hepatitis A, B, C, Cytomegalovirus and Ebstein-Barr virus infections. Results : Hepatitis occurred in 11.7% of Mycoplasma pneumoniae pneumonia, especially in fall and winter times. Male to female ratio was 1.2 : 1 and the mean age was 4 years and 3 months. Besides hepatitis, cough(95.4%), sputum(52.3%) and dyspnea(12.3%) were common as pulmonary manifestations. And among gastrointestinal manifestations, nausea/vomiting(26.2%) was the most common symptom, followed by poor oral intake(12.3%), diarrhea(12.3%) and abdominal pain(6.2%). In addition to hepatomegaly(4.6%) and splenomegaly(4.6%), coarse breathing sound was the most common physical manifestation, followed by rale(63.1%), pharyngeal injection(26.2%), and rash(10.8%). Anemia was noted in 20.0%, neutrophilia in 10.8%, eosinphilia in 38.5% and thrombocytosis in 6.2%, respectively. Mean level of ESR and CRP was 32.02 mm/hr and 6.69 mg/dL, respectively. Mean level of AST and ALT was 293.80 IU/L and 181.48 IU/L, respectively. Hyperbilirubinemia was noted in 7.7% and hypoalbuminemia was noted in 58.5%. Lobar or lobular pneumonia(78.5%) was the most common finding in chest X-ray and left lower lobe(39.2%) was most commonly affected. Pleural effusion was noted in 26.2%. Mean duration of hospitalization was 9.91 days. Serum AST/ALT level was normalized within 9.94 days and pulmonary consolidation resolved within 14.29 days. Conclusion : The prognosis of M. pneumoniae hepatitis is good. However, liver function should be considerately checked in M. pneumoniae infection because its incidence is not so low.

• Tuberculosis and Respiratory Diseases
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• v.44 no.1
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• pp.146-153
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• 1997

Background : Chronic cough is commomly defined as a persistent or recurrent cough exceeding 3 week's duration. The prevalence of chroinc cough is reported to range from 14% to 23 % for nonsmoking adults. The post nasal drip syndrome has been determined to be the most common cause of chronic cough, followed by asthma, chronic bronchitis, gastroesophageal reflux and bronchiectasis. Cough can be the only manifestation of asthma. Bronchial provocation tests are useful in diagnosing cough variant asthma. We investigated the clinical or laboratory findings and the incidence of airway hyperresponsiveness and evaluated the etiology in patients with chronic cough. Method : We evaluated 46 patients with chronic cough. Methacholine challenge test were done. Results : The results were as follows : 1) Thirty - five percent(16/46) of the chronic cough patients and 44% of the post nasal drip syndrom(7/16) showed the positive responses to methacholine challenge test 2) The underlying causes of chronic cough were post nasal drip syndrome in 35%, bronchitis in 21.7%, cough-variant asthma in 17.4%, and unknown condition in 25.9%. 3) Airway hyperresponsiveness in chronic cough was not related to respiratory symptom, nasal symptom, post nasal drip, smoking, derangement of ventilatory function, atopy, or sinusitis. Conclusion : Airway hyperresponsivenss in patients with chronic cough increased in frequency when compaired with normal control, allergic rhinitis. Cough-variant asthma account for 17.4% of patients with chronic cough.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.2
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• pp.181-190
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• 2001

Purpose: The aim of this study is to investigate the usefulness of intestinal ultrasonography (US) and upper gastrointestinal endoscopy in the early diagnosis of Henoch-Sch$\"{o}$nlein purpura (HSP) with the gastrointestinal (GI) symptoms preceding the emergence of the skin lesion. Methods: The clinical, intestinal US and upper gastrointestinal endoscopic records of 85 patients (88 cases) with GI symptoms relating to HSP presenting between January 1999 and April 2001 were reviewed. Results: 1) GI symptoms were observed in 52 cases (59%) and skin, joint, renal and scrotal manifestations were observed in 88 (100%), 64 (73%), 15 (17%), 3 cases (3%) respectively. 2) Out of 52 cases with GI symptoms, abdominal pain was observed in all cases (100%). Positive stool occult blood, nausea and vomiting, abdominal tenderness, melena or tarry stool, diarrhea, hematemesis, rebound tenderness and rigidity were observed in 28 (50%), 17 (33%), 17 (33%), 12 (23%), 6 (12%), 4 (8%), 1 (2%) and 1 case (2%) respectively in order of frequency. 3) Intestinal US examination was performed in 27 cases with HSP and GI symptoms (52 cases). Out of 27 sonographic examinations 22 showed abnormal findings. Thickening of the duodeno-jejunal wall was observed in 16 cases (73%). Free peritoneal fluid, enlarged mesenteric lymph node, ileus and abnormal gall bladder were seen in 8 (36%), 8 (36%), 4 (18%) and 1 case (5%) respectively. In three cases of HSP without GI symptoms, those changes were absent. 4) In all of five cases with HSP and GI symptoms, endoscopic study showed mucosal edema and multiple hemorrhagic erosions especially at the second portion of the duodenum. Biopsy specimens from the duodenum of 2 cases out of 5 endoscopic examinations showed acute inflammatory infiltrates in the mucosa with hemorrhage. 5) Both intestinal US and endoscopic studies were performed in 4 cases with HSP and GI symptoms simultaneously. Out of 4 those cases, 3 cases showed the thickened duodeno-jejunal wall on the intestinal US, which suggested erosive hemorrhagic duodenitis by endoscopic findings. Conclusion: The typical but nonpathognomonic intestinal US findings including the thickening of the duodeno-jejunal wall and upper gastrointestinal endoscopic findings including hemorrhagicerosive duodenitis, in children with GI symptoms, should be considered a manifestation of HSP, even in the absence of skin lesion.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Advances in pediatric surgery
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• v.1 no.2
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• pp.149-161
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• 1995

The first national survey on esophageal atresia and tracheoesophageal fistula was conducted to access the current status of its incidence, clinical manifestation, preoperative diagnosis and management, type of its anomaly, associated anomalies, and surgical results and course. The 43 members of the Korea Association of Pediatric Surgeons received questionnaires and registration forms to be filled out on each patient who were born during the three years from January 1, 1992 through December 31, 1994. Questionnaires composed of six broad areas which include 1) preoperative diagnosis and management, 2) surgical technic, 3) long gap, 4) postoperative management, and 5) complications and courses. A total of 148 cases was returned by 28 members working at 23 institutions and 27 members returned questionnaires. We obtained the following results by analysis of the 148 cases of tracheoesophageal anomalies. The incidence of tracheoesophageal anomaly was about 1/10,000-11,000 in 1994, which is one third of that of anorectal malformations in Korea and the distribution of the patients was almost proportionate to the size of each province. Both sexes are about equally affected. Majority of the members make diagnosis of tracheoesophageal anomaly by taking a simple infantogram with a radiopaque tube in upper pouch and a little under half(46%) prefers to perform echocardiography as a part of preoperative management to identify congenital heart disease and lateralize the aorta. Esophageal atresia with distal TEF(87.5%) was by far the most common and there were pure esophageal atresia(5.6%), H-type TEF(2.1%), and so on. About half(49%) of the patients had one or more associated anomalies in addition to tracheoesophageal anomalies. Congenital heart disease was associated in 46 cases(31%), anorectral malfomations in 19 cases(13%), musculoskeletal anomalies in 15 cases(10%), genitourinary anomalies in 10 cases(7%) and gastrointestinal anomalies in 7 cases(5%). Postoperatively, parenteral nutrition and assisted ventilation were given in 66% and 52% of patients respectively. Ninety three(74%) of 126 cases who underwent sugical procedure, experienced one or more complications such as respiratory complication(65%), leak(22%), stricture(21%) and so on. The survival rates related to the Waterston risk categories were 90.2% in group A, 71.4-75% in $B_1$, $B_2$, and $C_1$, groups, and 28% in group $C_2$, and the overall survival rate was 71.4%. Thirty six(28.6%) of 126 cases died of pneumonia/sepsis(12 cases), respiratory failure(12 cases), and congenital heart disease(4 cases). With short term follow-up, 69% of patients have been excellent whereas remainders of the cases have suffered from some sort of morbidity related to gastroesophageal reflux, recurrent respiratory infection, and esophageal stricture.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.28 no.6
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• pp.511-519
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• 2006

Autogenous bone grafts have been considered the gold standard for maxillofacial bony defects. However, this procedure could entail a complicated surgical procedure as well as potential donor site morbidity. Possibly the best solution for bone-defect regeneration is a tissue engineering approach, i.e. the use of a combination of a suitable scaffold with osteogenic cells. A major source of osteogenic cells is the bone marrow. Bone marrow-derived mesenchymal stem cells are multipotent and have the ability to differentiate into osteoblastic, chondrocytic, and adipocytic lineage cells. However, the isolation of cells from bone marrow has someproblems when used in clinical setting. Bone marrow aspiration is sometimes potentially more invasive and painful procedure and carries of a risk of morbidity and infection. A minimally invasive, easily accessible alternative would be cells derived from periosteum. The periosteum also contains multipotent cells that have the potential to differentiate into osteoblasts and chondrocytes. In the present study, we evaluated the osteogenic activity and mineralization of cultured human periosteal-derived cells. Periosteal explants were harvested from mandibule during surgical extraction of lower impacted third molar. The periosteal cells were cultured in the osteogenic inductive medium consisting of DMEM supplemented with 10% fetal calf serum, 50g/ml L-ascorbic acid 2-phosphate, 10 nmol dexamethasone and 10 mM -glycerophosphate for 42 days. Periosteal-derived cells showed positive alkaline phosphatase (ALP) staining during 42 days of culture period. The formation of ALP stain showed its maximal manifestation at day 14 of culture period, then decreased in intensity during the culture period. ALP mRNA expression increased up to day 14 with a decrease thereafter. Osteocalcin mRNA expression appeared at day 7 in culture, after that its expression continuously increased in a time-dependent manner up to the entire duration of culture. Von Kossa-positive mineralization nodules were first present at day 14 in culture followed by an increased number of positive nodules during the entire duration of the culture period. In conclusion, our study showed that cultured human periosteal-derived cells differentiated into active osteoblastic cells that were involved in synthesis of bone matrix and the subsequent mineralization of the matrix. As the periosteal-derived cells, easily harvested from intraoral procedure such as surgical extraction of impacted third molar, has the excellent potential of osteogenic capacity, tissue-engineered bone using periosteal-derived cells could be the best choice in reconstruction of maxillofacial bony defects.

• Journal of Nutrition and Health
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• v.4 no.1
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• pp.1-19
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• 1971

Nutritional anemia is an important nutritional problem affecting large population groups in most developing countries. Nutritional anemia is caused by the absence of any dietary essential involed in hemoglobin formation or by poor absorption of these dietary components. The most likely causes are lack of dietary iron, and folate, vitamin $B_{12}$ and high qualify protein. Anemia is considered to be a late mainfeastation of nutritional deficiencies, and even mild anemia is not the earilest sign of such a deficiency. Therefore, the object of therapy is to correct underlying deficiency rather than merely its manifestation. Iron deficiency anemia is generally much the most common form of anemia. And it is very prevalent particularly in pregnant women and young children, especially under five year of life. According to the rapid growth rate of infants, dietary iron should he provided for infants over three months of age in adequate amounts for the synthesis of hemoglobin required by the increasing blood volume and for the demands of newly formed cells. The principal causes of iron deficiency anemia are an inadequate dietary iron content, interference with absorption of iron from the intestine, excessive losses of iron from the body, disturbance of iron metabolism by infection, and social and cultural environments. The present study is planned to obtain informations concerning nutritional anemia through anthropometric and biochemical determinations for the assessment of nutriture in pre-school children. Determination was taken in 226 pre-school children in ruraI arae in 1968, 122 pre-school children in 1970, and 1526 hospitalized pre-school children in 1970. The results of this study are as follows; (1) According to Iowa Malnutrition Borderline (85 percentile) for weight, the proportions of underweighed pre-school boys and girls in rural area were 47.2% and 46.2% in1968, and were 36.1% and 51.8% in 1970. According to Iowa Malnutrition Borderline for height, the proportions of underheight boys and girls in rural area were 30.5% and 33.7%, and were 26.2% and 21.8% in 1970. Malnutrition scores of underweight for height values of boys and girls in rural area were 19.3 and 17.3 in 1968, and the scores of boys and girls were 15.6 and 15.5 in 1970. (2) The mean hemoglobin values of boys and girls in rural area were $11.2{\pm}1.8g/100ml\;and\;11.4{\pm}1.6g/100ml$ in 1968. In 1970, the mean values of boys and girls in rural area were $11.3{\pm}1.3g/100ml\;and\;11.7{\pm}2.4g/100ml$. The mean hemoglobin values of hospitalized boys and girls were $11.9{\pm}2.2g/100ml\;and\;11.7{\pm}2.4g/100ml$ in 1970. It is found that 92 of 215 children (42.7%) in rural area had concentrations of hemoglobin less than 11.0g/100ml in 1968. In 1970, 55 of 121 children (45.4%) in rural area and 559 of 1526 hospitalized children (36.6%) had concentrations of hemoglobin less than 11.0g/100ml. (3) The mean hematocrit levels of hospitalized boys and girls were $35{\pm}26.8%\;and\;35.4{\pm}6.4%$ in 1970. And 443 of 1334 hospitalized children (33.2%) had hematocrit values below 33%. (4) The average mean corpuscular hemoglobin concentration levels of hospitalized boys and girls were $32.4{\pm}2.2\;and\;32.3{\pm}2.2$ in 1970. And 1016 of 1352 hospitalized children (75.1%) had the mean corpuscular hemoglobin values below 34. (5) The mean iron values of young children in rural area and hospitalized children were $62.0{\pm}6.3{\mu}g/100ml\;and\;60.7{\pm}22.8{\mu}g/100ml$. The proportions of anemia cases below $50{\mu}g/100ml$ in rural area was 37.9%, and 34.3% in hospitalized children. (6) The mean total iron binding capacity of young children in rural area was $376{\pm}57.88{\mu}g/100ml,\;and\;342.2{\pm}6.15{\mu}g/100ml$ in hospitalized children. (7) The average transferrin saturation percentage of young children in rural area was $16.9{\pm}4.7%,\;and\;18.0{\pm}8.4%$ in hospitalized children. The proportions of anemia cases below 15% of young chi1dren in rural area and hospitalized children were 48.3% and 41.2%. Therefore, authors wish to recommend that the following further studies should be undertaken: (1) Standardization of simplied laboratory examination of nutritional anemia. (2) The prevalence of nutritional anemia and the requirements of iron, folate, and vitamin $B_{12}$ of pre-school children. (3) The content and absorption of iron in Korean food. (4) The pathogenesis of nutritional anemia and prevention of parasitic disease. (5) Maternal health and nutrition education.