• Neonatal Medicine
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• v.17 no.1
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• pp.75-83
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• 2010

Purpose : This study was performed to determine the predictors of failed closure of a patent ductus arteriosus (PDA) following the first course of indomethacin in symptomatic preterm infants. Methods : Forty three of 43 preterm infants, admitted to the neonatal intensive care unit diagnosed with PDA and treated with indomethacin at the Korea University Medical Center between January 1990 and October 2007, ware studied. The perinatal risk factors affecting the failed closure of PDA were retrospectively assessed. Results : The failed PDA closure group included 16 (37%) out of 43 infants three of whom underwent surgery. The closure group included 27 (63%) out of 43 infants. In the failed closure group, the Apgar scores (1 min, 5 min) were significantly higher (P<0.05) and antenatal steroid administration was significantly lower (P<0.05). In addition, dopamine administration was significantly lower (P<0.05) and the mean postnatal age at diagnosis was significantly lower (P<0.05). Multiple logistic regression for the prediction of failed PDA closure found only antenatal steroid administration (OR 0.092, CI 0,010-0.826, P=0.0331) as an associated factor. Conclusion : In patients with antenatal steroid administration the failed PDA closure rate was significantly lower. Therefore, antenatal steroid administration can be considered as an important factor for the closure of PDA in preterm pregnancies.

• Journal of Yeungnam Medical Science
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• v.16 no.1
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• pp.60-68
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• 1999

A nationwide survey was conducted to investigate the annual occurrence rate of neonatal sepsis, maternal risk factors in neonatal sepsis, localized infection in neonates, causative organisms in nosocomial infection and the most common causative organism for neonatal sepsis in Korea. Clinical and bacteriological data wele collected from 37 neonatal units to perform retrospective review of the medical records of the newborn infants who were confirmed as having neonatal sepsis and whose blood culture was collected to isolate organisms for one year study period from January to December in 1997. 78,463 neonates were born at 37 hospital in 1997, and 20,869 neonates were admitted to the neonatal units, During this period, 772 episodes of neonatal sepsis were recorded in 517 neonates. The occurrence rate of neonatal sepsis was 0.73%(0~2.95%). Male to female ratio was 1.15:1, and 303 cases(42.1%) were born prematurely. The main pathogens of early onset of sepsis were S. aureus(20%), S. epidermidis(14.4%) and coagulase negative staphylococcus(14. 4%). Gram negative bacilli including Enterobacter spp (7.2%), E. coli(5.1%), Klepstella(4.5%), Pseudomonas(3.7%) and Enterobacter faectum(3.6%) accounted for 24.1% of sepsis. Group B beta-hemolytic streptococcus were isolated only in two cases. Common obstetric factors were PROM(21.1%), difficulty delivery(18.7%), fetal tachycardia(5.3%), chorioamnionitis(4.9%), and maternal fever(4.7%). The main pathogens of late-onset sepsis were S. aureus(22.3%), S. epidermidis(20.4%) and CONS(9.9%). There were 6 cases(1.0%) of Candida sepsis, Frequent focal infections accompanying sepsis were pneumonia(26.1%), urinary tract infection(10.5%), meningitis(8.2%), and arthritis(3.6%), S. epidermidis(22.0%) and s. aureus(21.7%) were also the most common pathogens in 373 nosocomial infection.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.77-82
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• 2001

Purpose: The fecal acid steatocrit is an improved steatocrit method for the evaluation of fecal fat. The present study was set up in order to define the normal range of acid steatocrit values during the first 3 months of life. Methods: Fecal acid steatocrit values were determined in 78 healthy full term and in 21 healthy prematurely born infants between May 1998 and April 2000. The acid steatocrit method was performed in these babies during the first 3 months of life. Results: Steatorrhea occurs during the first month and then decreases, as shown by the fall in the acid steatocrit curve from 1st to 3rd month in our subject. Very high acid steatocrit results (above 90%) were found in all full term and premature infants during the first month of age. Acid steatocrit results of human milk-fed infants were significantly lower than those of formula-fed infants (p=0.0018). Conclusion: We conclude that high acid steatocrit results during the first 1 month of age can be due to physiologic steatorrhea. The acid steatocrit micromethod can be used for the evaluation of milk fat absorption in infants and monitoring steatorrhea instead of other more cumbersome methods.

• Journal of Korean Physical Therapy Science
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• v.3 no.1
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• pp.863-870
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• 1996

The 46 patients were investigated with the congenital infant muscular torticollis referred Dept. of physical therapy in Dong San Medical Center from January in 1995 to January in 1996. 1. The ratio between males and females was scaled to 1.5 : 1.23 cases(50.0 %) were involved with left side and 22 cases(47.8 %) involved with right side, and one case was of bilaterality. 2. Sequence of birth, the first bone babys were the most distributed with 34cases(73.9 %), the second bone babys were followed with 11cases(23.9 %). 3. There were normal deliveries 60.8 %, Cearean section deliveries 23.9 %, difficulty deliveries .0 % and breech presentation was 2.2 %. 4. The case of cephalic asymmetry consisted 60.0 % of all congenital torticollis and most frequently found at the age of all $5\sim8$ weeks(45.7 %). In 26.1 % of all congenital torticollis cases, facial asymmetry was found and these cases were most frequently in the age of $5\sim8$ weeks(26.1 %), also in the age group of $9\sim12$ weeks and $17\sim20$ weeks consecutively(each 3 %).(P<0.05) 5. The duration of therapy required differently according to the severity of the torticollis, in mild cases, it took $1\sim2$ weeks cases(30.4 %) revealed high therapeutic effect in 54.3 % of the cases. In moderately involved cases(30.4 %), therapy required $3\sim4$ weeks in 13 % of the cases showed improvements. In the most severely involved cases, (7 %) it took more than $9\sim10$ weeks of therapy and showed improvement in 6.5 % only.(P<0.005) 6. The result of this study showed the best therapeutic effects were noticed in the mild cases of congenital torticollis(24 cases 45.6 %) and excellent improvement in moderately involved cases (28.1 %) and 13.1 % of the most severely involved cases(P<0.05).

• Clinical and Experimental Reproductive Medicine
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• v.34 no.2
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• pp.87-94
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• 2007

Objective: To examine determinants of successful pregnancy and evaluate COH-IVF outcomes of infertile patients after conservative surgical treatment of borderline ovarian tumor (BOT). Methods: In women of BOT (n=93), from January 1995 to December 1999, 44 of 93 women underwent conservative surgical treatment. From theses 44 women, patients characteristics, surgical and histological parameters were compared between 14 women who conceived and 30 women who failed to conceive. For 5 infertile women of 30 women who failed to conceive, 10 attempt IVF cycles were analysed; clinical pregnancy rate (CPR), implantation rate (IR) and live birth rate (LBR). Results: Women who conceived tend to be younger (25.9 vs 27.0 years) and lower serum CA-125 level (59.7 vs 72.9) compared to women who failed to conceive without significant difference. For 8 cycles out of 10 attempt IVF cycles, except for 2 cancellation cycles, the mean number of oocytes retrieved was 5.6 (range 2$\sim$16) with a mean fertilization rate of 74.4%. The CPR, IR and LBR per embryo transfer were 50.0% (4/8 cycles), 31.6% (6/19) and 50.0% (4/8 cycles) respectively. During the mean follow-up period after COH-IVF initiation, 29.6 (range 14$\sim$61) months, no recurrence was found. Conclusion: No determinant of successful pregnancy was found after conservative treatment for BOT. COH-IVF may be considered for infertile patients after conservative treatment of BOT. However, larger clinical studies with longer follow-up are necessary to evaluate the safety and efficacy of COH-IVF. All patients should be informed of the potential risks associated with ovarian hyperstimulation and close follow-up is necessary after COH-IVF.

• Clinical and Experimental Reproductive Medicine
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• v.34 no.2
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• pp.125-131
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• 2007

Objective: The aim of this study was to compare GnRH antagonist and agonist flare-up treatment in the management of poor responder patients. Methods: One hundred forty-four patients from Jan. 1, 2002 to Aug. 31, 2005 undergoing IVF/ICSI treatment who responded poorly to the previous cycle (No. of oocyte retrieved$\leq$5) and had high early follicular phase follicle stimulating hormone (FSH>12 mIU/ml were selected. Seventy-five patients received agonist flare-up protocol and 71 patients received antagonist protocol. We analyzed the number of oocytes retrieved, number of good embryos (GI, GI-1), total dose of hMG administered, implantation rate, cycle cancellation rate, pregnancy rate, live birth rate. Results: The cancellation rate was high in antagonist protocol (53.5% vs. 30.1%). The number of oocyte retrieved, the number of good embyos were high in agonist flare-up group. There was no statistical difference between GnRH agonist flare up protocol and GnRH antagonist protocol in implantation rate (14.5%, 10.1%), clinical pregnancy rate per transfer (29.4%, 21.2%) and live birth rate per transfer (21.6%, 18.2%). Although the result was not statistically significant, GnRH agonist flare up group showed a nearly doubled pregnancy rate and live birth rate per initial cycle than GnRH antagonist group. Conclusions: The agonist flare-up protocol appears to be slightly more effective than the GnRH antagonist protocol in implantation rate, pregnancy rate, live birth rate but shows statistically no significance. Agonist flare-up protocol improved the ovarian response in poor responders. However, based of the result of the study, we can expect improved ovarian response in poor responders by GnRH agonist flare up protocol.

• Pediatric Infection and Vaccine
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• v.16 no.1
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• pp.80-86
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• 2009

Purpose : This study was performed to determine the clinical course of BCG lymphadenitis. Methods : Between May 2005 and April 2009, the medical records of 33 patients with BCG lymphadenitis were retrospectively reviewed. If needed, needle aspiration was recommended without surgical resection or antituberculous medication. Results : Of the 33 patients who were identified, 21 were males and 12 were females. Among the 33 patients, 32 were full-term babies. The mean age was 6 months (range, 2-35 months) and the most prevalent site of the lesion was the left axilla. BCG lymphadenitis was observed 1-34 months after BCG vaccination, mostly 1-6 months after vaccination. The size of the enlargement was generally 1-3 cm. The strains were identified as French (n=14), Danish (n=7), and Tokyo (n=12). BCG lymphadenitis regressed spontaneously in 19 patients. After 1-5 needle aspirations, 14 patients recovered completely. Complete regression of lymphadenitis was recorded over an average period of 4 months. Conclusion : Clinicians need to be aware of the clinical features of BCG lymphadenitis. For management of BCG lymphadenitis, regular follow-up with observation should be the mainstay. Needle aspiration is a safe and easy treatment for suppurative BCG lymphadenitis.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.42-50
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• 2010

Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.35-42
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• 2018

Purpose: To follow up Korean patients with metabolic and endocrine disorders ascertained by Korea Genetics Research Center, and assess the long-term effectiveness of extended newborn screening program in Korea. Methods: From January 2000 to December 2017, tandem mass spectrometry and fluoroimmunoassay were employed in extended newborn screening (NBS). The NBS program obtained dried blood spots from 283,626 babies, 48 hours after birth, and screened for galactosemia, congenital hypothyroidism (CH), congenital adrenal hyperplasia (CAH), and 50 preventable inborn errors of amino acid, fatty acid, and organic acid metabolism. Results: 28 cases of amino acid disorders, 75 cases of organic acid disorders, 27 cases of fatty acid disorders, 51 cases of urea cycle disorders, 127 cases of CH, 14 cases of CAH, and 15 cases of galactosemia were ascertained through NBS and subsequent confirmatory laboratory tests. Patients with amino acid metabolic disorders, galactosemia, CH, or CAH were more likely to have a better long-term outcome if detected early. Early management of MSUD led to much better outcome in over 90%. Despite early intervention, 32% of other organic acidemia cases still resulted in developmental delay and neurological problems. Fatty acid disorders showed varied results; those with EMA and MCAD had a good outcome, but those with VLCAD had serious neurological problems and considerably higher mortality. 75% with UCD experienced serious neurological complications and higher mortality. Conclusion: The nation-wide NBS program must be accompanied by comprehensive long-term management and physician and family education of inborn errors of metabolism for a better outcome.

• 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)