• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.30-40
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• 2000

Purpose: During the first year of life, cow's milk protein is the major offender causing food allergy. Cow's milk allergy (CMA) affects 2~7% of infants, of which approximately one-half show predominantly gastrointestinal symptoms. We studied the clinical types of cow's milk allergy with predominantly gastrointestinal symptoms (CMA-GI) of childhood. Methods: The retrospective study was performed on 30 (male 22, female 8) patients who had diagnosed as CMA-GI during 2 years and 3 months from March 1995 to June 1997. Results: 1) Children with CMA-GI presented in the three types of clinical manifestation on the basis of time to reaction to milk ingestion: Quick (Q) onset (5 cases), Slow (S) onset (20 cases), Quick & Slow (Q&S) (5 cases). 2) Age on admission of the three groups was significantly different (p<0.05): (Q onset: $81.4{\pm}67.1$ days, S onset: $31.9{\pm}12.7$ days, Q&S: $366.0{\pm}65.0$ days). Although the body weight at birth was 10~95 percentile in all patients, body weight on admission was different: (Q onset: 10~50 percentile, S onset: below 10 percentile, Q&S: 10~25 percentile). S onset group was significantly different compared with other groups (p<0.05) and 90% of this one was failure to thrive below 3 percentile. 3) Peripheral leukocyte counts were as followings: (Q onset: $5,700{\sim}12,300/mm^3$, S onset: $10,000{\sim}33,400/mm^3$, Q&S: $5,200{\sim}14,900/mm^3$). Slow onset group was significantly different compared with other groups (p<0.05). Serum albumin levels on admission were as followings: (Q onset: $4.2{\pm}0.4\;g/dl$, S onset: $3.0{\pm}0.3\;g/dl$, Q&S: $4.0{\pm}0.3\;g/dl$). S onset group was significantly different compared with other groups (p<0.05) and 85% of this one was below 3.5 g/dl. 4) Although morphometrical analysis on small intestinal mucosa did not show enteropathy in Q onset and Q&S groups, all cases of S onset revealed enteropathy: 45% of this one showed subtotal villous atrophy, 55 % showed partial villous atrophy. 5) Allergic reaction test to other foods was not performed in S onset group because of ethical problem and high risk in general condition. In Q onset group, allergic reaction to one or two other foods: soy formula, weaning formula and eggs. Q&S goup revealed allergic reactions to several foods or to most of all foods except protein hydrolysate formula: eggs, potatos, some kinds of sea food, apples, carrots, beef and chicken. 6) Serum IgE level, peripheral eosinophil counts, milk RAST, soy RAST, skin test were not significantly different among groups. Conclusion: CMA-GI may present in three clinical ways on the basis of time to reaction to milk ingestion, typical clinical findings and morphologic changes in the small bowel mucosal biopsy specimens. This clinical subdivision might be helpful in diagnostic and therapeutic approaches in CMA-GI. Early suspicion is mandatory especially in S onset type because of high risks with malnutrition and enteropathy.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.77-83
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• 2000

Purpose: Pregnancy in transplanted mother is considered as a high-risk pregnancy, and significant incidences of prematurity and low-birthweight(LBW) infants have been reported. The objective of this study is to examine the outcome of pregnancy in transplanted mothers and to evaluate the current growth status in their children. Patients and Methods: We retrospectively reviewed 54 pregnancies in 40 kidney recipients until June 1999. Outcomes of pregnancy were reviewed and assessment of the current growth status in children was performed. Results: 54 pregnancies in 40 recipients were identified; 22 ended in termination of pregnancy because of unwanted pregnancy or therapeutic purposes. And of the other 32, 29 livebirths resulted in 28 recipients. The mean age of conception was $30.3{\pm}3.8$ years, with a mean interval from transplantation to conception of $35.9{\pm}23.2$ months. All patients were maintained on immunosuppressive regimens. Incidence of drug-treated hypertension(HTN) prior to pregancy was $52\%$, HTN during pregnancy, $48\%$; preeclampsia, $41\%$; urinary tract infection, $48\%$; oligohydramnios $4\%$; and no rejection during pregnancy and up to 3month post delivery. Of the 29 liveborn infants, prematurity(<37wk) occurred in $52\%$, LBW(<2500g) in $62\%$, VLBW(<1500g) in $7\%$ and $48\%$ born intrauterine growth retardation(IUGR). Mean gestational age was $36.3{\pm}3.0\;wk$; a mean birthweight, $2.23{\pm}0.6\;kg$; a mean birth-height, $45.1{\pm}3.6cm$. Current mean height standard deviation score (height SDS) was $0.29{\pm}0.91$ and mean weight SDS was $0.62{\pm}1.34$. Only one child($4\%$) under 1 year of age was below 10 percentile in height. Most of children had no medical problems except for 4 children; cleft palate(1), tuberous sclerosis(1), essential hematuria(1), and one child expired due to sepsis. Conclusion: This study showed similar incidence of premaure birth($57\%$) and low birth weight infants($62\%$), but lower incidence of spontaneous abortion($5.6\%$) was observed and compared to other studies. Postnatal growth in majority of children($96\%$) achieved catch-up growth before 1 year. Present study supports a more optimistic view of pregnancy in renal transplant mother and normal growth in their children.

• 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)

• 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.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.376-381
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• 2003

Purpose : This study aimed to demonstrate the possible pathogenesis of granulopoiesis in patients of Kawasaki disease(KD) using quantitative analysis of G-CSF, GM-CSF and their CSFr. Methods : The plasma levels of G-CSF, GM-CSF, G-CSFr and GM-CSFr were studied in 14 patients in the acute phase of KD; 13 children with normal peripheral white blood cell counts were used as the normal control group. The plasma concentration of G-CSF, GM-CSF were analyzed by ELISA. The G-CSFr and GM-CSFr on the peripheral granulocytes were analyzed by a quantitative flow cytometric assay and QuantiBRITE, and the quantitative changes of receptors which did not combine with G-CSF and GM-CSF were measured. Results : The total number of leukocytes in KD was similar to normal control group, but the leukocytes increased according to the number of neutrophils. The plasma concentration of G-CSF were decreased similar to normal control group(P=0.133), but that of GM-CSF decreased more than the normal control group(P=0.227). The quantity of G-CSFr, GM-CSFr were revealed to be no less than the normal control(P=0.721, P=0.912). After incubation with excessive G-CSF, the expressed G-CSFr on the neutrophils were decreased in both groups(P=0.554). The quantities of expressions of GM-CSFr on the neutrophil after incubation with the excessive GM-CSF were always increased in both groups(P=0.255). The amount of GM-CSFr of neutrophils are in proportion to total white blood cells (r=0.788, P=0.035), but it wasn't in the case of KD(P=0.644). Conclusion : The leukocytosis in KD that mediated by increasing neutrophil was not correlated with the plasma concentrations of G-CSF and GM-CSF, and the amount of expression of G-CSFr and GM-CSFr on granulocyte. It is possible that the reduction of concentration of GM-CSF results by increasing the active GM-CSFr.

• Journal of Chest Surgery
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• v.36 no.1
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• pp.1-6
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• 2003

Currently, atrial septal defect repair has been considered low risk operation duo to the development of open heart surgery Not only the operation itself, but also the cosmetic aspect is now focused. Though many methods exist as minimally invasive cardiac surgery in atrial septal defect repair, some surgeons advocate that right anterolateral thoracotomy is better than the others in the cosmetic aspect and we compared right anterolateral thoracotomy with median sternotomy. Material and Method: From January 1999 to August 2002, 43 patient underwent atrial septal defect repair by one operator, including 15 patients through right anterolateral thoracotomy(group A) and 15 patients through median sternotomy(group B) in Hanyang university Hospital. The data were randomized and operation outcomes were analyzed between these two groups. Result: The mean weight of group A was 38.77$\pm$15.57kg and 38.21$\pm$21.82kg in group B. In group A, mean operation (OP) time was 197.6$\pm$61.40min, mean cardiopulmonary bypass(CPB) time was 48.66$\pm$13.02min and mean fibrillation time or aortic cross clamp(ACC) time was 30$\pm$11.64min. In group B, mean OP time was 212.33$\pm$31.95min, mean CPB time was 55$\pm$12.10min, and mean fibrillation or ACC time was 29.33$\pm$9.04min. There was no significant differences in these two groups. In group A, mean mechanical ventilation time was 3.78$\pm$0.78 hours, mean postoperative ICU stay was 1.2$\pm$0.47 days and mean postoperative hospital stay was 10.20 41.08 days. In group B, mean mechanical ventilation time was 5.95$\pm$3.73 hours, mean post operative ICU stay was 1.41$\pm$0.61 days, and mean postoperative hospital stay was 12.20$\pm$3.55 days. There was no any significant difference in two groups. Group A had significantly lower mean thoracic and pleuropericardial drainage than group B (175.33$\pm$90.54cc vs 352.33$\pm$239.43cc, p<0.05). Complication was seen in one case in group B, transient 2nd degree A-V block. Conclusion: Right anterolateral thoracotomy was better than median sternotomy not only in cosmetic aspect but also in postoperative thoracic and pleuropericardial drainage, using the same instrument(p.0.05). But, right anterolateral thoracotomy was more technically difficult due to narrow operative field and we should be careful of aortic cannulation.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.464-470
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• 2009

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.395-400
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• 2005

Purpose :Rotavirus is one of the most important causes of nosocomial infections among children. The aim of this study is to determine the risk of nosocomial rotavirus infections and to evaluate the effectiveness of breast-feeding and probiotics in the prevention of nosocomial rotavirus infections. Methods : This study was carried out on admitted children without diarrhea between March 1, 2003 and February 29, 2004. Three hundred ninety patients aged 4 days to 13 years during this study were available. We examined the feces of all children for rotavirus by latex agglutination on admission, during hospital, and after discharge, to see whether they developed diarrhea or not. Results : Nosocomial rotavirus infections was significantly increased with children under 12 months of age(P=0.008). The monthly attack rate was great between December and March(P=0.046). Prolonged hospital stay was associated with an increased attack rate of nosocomial rotavirus infections (P=0.003). The risk of nosocomial rotavirus infections was not associated with the number of roommates and whether or not they were breast-fed or fed on probiotics. Conclusion : Nosocomial rotavirus infections are significantly more likely to occur in children under 12 months of age, admitted between December and March, and with prolonged hospital stays. Prompt identification and isolation of children with nosocomial rotavirus infections, even without diarrhea, may decrease rates of nosocomial rotavirus infections.

• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.369-375
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• 2005

Purpose : Many inborn errors of metabolism can be completely cured with early detection and early treatment. This is why neonatal screening on inborn errors of metabolism is implemented worldwide. In this study, a cost-benefit analysis was performed on the neonatal screening of phenylketonuria and congenital hypothyroidism in Korea. Methods : This study included 2,908,231 neonates who took the neonatal screening on phenylketonuria and congenital hypothyroidism in Korea from January 1991 to December 2003. From those neonates, the incidence rates of phenylketonuria and congenital hypothyroidism were measured. Furthermore, based on 495,000 babies born in 2002, were calculated and compared the total costs in case when neonatal screening on phenylketonuria and congenital hypothyroidism is implemented, and when not. Results : If the neonatal screening on phenylketonuria and congenital hypothyroidism is implemented, benefits far exceed costs at a ratio of 1.77 : 1 in phenylketonuria, and 11.11 : 1 in congenital hypothyroidism. In terms of wons, the present neonatal screening on phenylketonuria and congenital hypothyroidism will gain us more than 29 billion wons every year. Conclusion : This study only concerns the monetary aspects of the neonatal screening. Therefore, the benefits of the neonatal screening is underestimated by ignoring precious but not measurable values such as quality of life. However, the present neonatal screening on phenylketonuria and congenital hypothyroidism is found to be beneficial and should continue for the good of the nation as well as that of the patients.

• Clinical and Experimental Pediatrics
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• v.52 no.11
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• pp.1221-1227
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• 2009

Purpose:To identify the risk factors for poor neurodevelopmental outcomes in high-risk neonates. Methods:We studied 94 infants admitted to the neonatal intensive care unit at the Sanggye Paik Hospital between January 2002 and November 2005 and evaluated the follow-up data. The following events were considered as risk factors: ${\leq}32$ weeks of gestation, very low birth weight, Apgar scores of less than 5, neonatal resuscitation, neonatal seizure, congenital infection, and abnormalities in cranial ultrasound or magnetic resonance imaging (MRI). The infants who had any one of these risk factors were included. They were evaluated at their mean corrected age of 13.84${\pm}$8.03 months. Mental and Psychomotor Developmental Indices (MDI, PDI) were determined by a clinical psychologist using the Bayley Scales of Infant Development II. Results:The mean MDI and PDI of all the patients were 96.28${\pm}$26.70 and 94.00${\pm}$22.80, respectively. Abnormalities on cranial ultrasound or MRI were significant predictors of both low MDI (P<0.05) and low PDI (P<0.001). These abnormalities showed a stronger association with low PDI than with low MDI. The infants with periventricular leukomalacia had the lowest MDI and PDI ($70.10{\pm}28.68$ and $69.70{\pm}24.91$, respectively). Apgar score at 1 minute and very low birth weight were also significant predictors for low PDI (P<0.05, P<0.05, respectively). Conclusion:Image findings with cranial ultrasound or MRI were the strongest predictors of neurodevelopmental outcome. Periventricular leukomalacia was the best predictive factor for mental and psychomotor development.