• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권4호
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• pp.306-314
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• 2018

Purpose: The objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis. Methods: A cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations. Results: Of the 77 patients, 51.9% were male, with a median age of 147 months (7.0-297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02). Conclusion: The pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.

• Advances in pediatric surgery
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• 제17권1호
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• pp.45-50
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• 2011

Meckel's diverticulum (MD) has various clinical presentations and due to the limitation of imaging studies, pre-operative diagnosis is a challenge in pediatric patients. Recently, laparoscopic exploration has been suggested as a favorable method for the diagnosis and treatment of complicated MD. We investigated the results of laparoscopic-assisted surgery compared with open technique. We retrospectively studied patients who underwent resection of complicated MD at our institute from 1997 to 2010 and compared 11 treated by laparoscopic-assisted diverticulectomy (LD) with 11 treated by open diverticulectomy (OD) for complicated MD. Operation time was not significantly different in the two groups. Hospital stay and time to diet were not significantly different. Two patients were re-admitted due to mechanical ileus in the LD group. None of patients in either group needed re-operation. Considering the possibility of false-positive results with imaging studies and the cosmetic benefit, laparoscopic-assisted surgery is a safe and effective treatment modality to diagnose and treat complicated Meckel's diverticulum.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권1호
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• pp.31-36
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• 2014

Purpose: Infliximab (IFX) is considered safe and effective for the treatment of ulcerative colitis (UC) in both adults and children. The aim of this study was to evaluate the short- and long-term clinical course of IFX in Korean children with UC. Methods: Pediatric patients with UC who had received IFX infusions between November 2007 and May 2013 at Samsung Medical Center were retrospectively investigated. The clinical efficacy of IFX treatment was evaluated at 8 weeks (short term) and 54 weeks (long term) after the initiation of IFX treatment using the Pediatric Ulcerative Colitis Activity Index (PUCAI). The degree of response to IFX treatment was defined as complete response (PUCAI score=0), partial response (decrement of PUCAI score${\geq}20$ points), and non-response (decrement of PUCAI score <20 points). Adverse events associated with IFX treatment were also investigated. Results: Eleven pediatric patients with moderate to severe UC had received IFX. The remission rate after IFX treatment was 46% (5/11) and 82% (9/11) at 8 weeks and 54 weeks after IFX treatment, respectively. All patients who were steroid-dependent before treatment with IFX achieved remission at 54 weeks and were able to stop treatment with corticosteroids, while all steroid-refractory patients failed to achieve remission at 54 weeks after treatment with IFX. Conclusion: Response to IFX treatment after 8 weeks may predict a favorable long-term response to IFX treatment in Korean pediatric UC patients.

• Childhood Kidney Diseases
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• 제21권2호
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• pp.69-74
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• 2017

Purpose: Kidney transplantation (KT) is an ideal treatment for pediatric patients with end-stage renal disease (ESRD). We report the clinical outcomes of pediatric ESRD patients who underwent KT in a single regional center. Methods: We retrospectively investigated the medical records of 60 pediatric patients who were diagnosed with ESRD and underwent KT in our hospital between January 1985 and June 2016. Results: A total of 60 children and adolescents (40 male, 20 female; mean age, $13.86{\pm}4.26$ years) were included in this study. Six patients (10.0%) underwent KT immediately after receiving the diagnosis of ESRD, while the others underwent KT after dialysis treatment (mean period of dialysis, $368.7{\pm}4,41.8$ days). The mean donor age (50 living-related [83.3%], 10 deceased [16.7%]) was $40.0{\pm}12.85$ years and the male:female ratio was 1.07:1. The most common cause of ESRD was chronic glomerulonephritis. The overall survival rates at 1, 3, and 5 years after KT were 98%, 98%, and 96%, respectively, while the graft survival rates at 1, 3, and 5 years were 93%, 86%, and 68%, respectively. Children who underwent KT before 10 years of age had better monthly growth rates than those who underwent KT later than 10 years of age. Conclusions: KT is performed less frequently in children than in adults, but causes of ESRD vary and clinical outcomes after KT greatly affect the growth and development of pediatric patients. Therefore, further analysis and monitoring of clinical progression after KT in pediatric ESRD patients are necessary.

• Advances in pediatric surgery
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• 제7권2호
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• pp.142-146
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• 2001

Lymphangioma is a congenital malformation of the lymphatic system, commonly seen in the neck. Operation was the treatment of choice but it is difficult to resect the lymphangiomas completely. The aim of this study is to evaluate the result of intralesional injection of OK-432 as a treatment strategy of lymphangioma in children. Medical records of 51 cases of lymphangioma from March 1996 to February 2001 were reviewed retrospectively. Intralesional injection of 0.1mg OK-432 in 10ml normal saline was performed after the aspiration of as much fluid as possible. The location of the lesion was the face and neck in 26 patients, the chest wall in 14, the extremities in 9, and the abdominal wall in 2. The cystic type was present in 45 patients and the cavernous type in 6. Four postoperative recurrent cases were included. Fluid aspiration from the lesion was impossible in 5 patients. Development of fever after injection was observed in 27 patients and local inflammatory reaction was in 5 patients. There was no scar formation at injection sites. Complete shrinkage was observed in 20 patients, remarkable shrinkage in 23, slight shrinkage in 3, and no response in 5. Cystic type or aspiration-possible cases showed better outcome than cavernous type or aspiration-impossible cases. All of four recurrent cases after surgical excision showed at least remarkable shrinkage. These results indicate that intralesional injection of OK-432 is a safe and satisfactory treatment modality of lymphangiomas in children and might be considered as a treatment of choice, even in recurrent cases.

• Journal of Yeungnam Medical Science
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• 제21권1호
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• pp.67-73
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• 2004

소아에 비해 성인에서 발생한 신우요관이행 부폐색의 두드러진 임상적 특징은 소아에 비해 양측성인 경우가 현저히 적었으며, 진단이 지연되었음에도 불구하고 수술 성공률은 소아에서와 비슷하였다. 향후 소아 및 성인의 신우요관이행부폐색 환자에 대한 광범위한 임상 통계가 이루어진다면 이 질환의 정확한 자연사를 알 수 있으며, 또한 성인에서 발생한 이 질환이 태아나 소아 때부터의 질환인지, 아니면 성인이 되어 발생한 질환인지 확인할 수 있을 것이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권1호
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• pp.41-46
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• 2017

Purpose: Vitamin D deficiency is common in Crohn disease (CD). The aim of the study was to examine the prevalence of vitamin D deficiency and evaluate the association between vitamin D status and growth outcome in Korean pediatric CD patients. Methods: In this retrospective study, 17 children younger than 18 years old diagnosed with CD were enrolled and their serum 25-hydroxy vitamin D (25[OH]D) was checked between 2011 and 2015. We categorized the patients into two groups, Group 1 and Group 2. Group 1 included patients with serum 25(OH)D levels below 10 ng/mL, and Group 2 was for patients with a 25(OH)D serum levels between 10 ng/mL and 30 ng/mL. The z-scores for height (Htz), weight (Wtz), and body mass index (BMIz) were measured at baseline, 6 months, and 12 months. Results: The mean serum 25(OH)D levels of the total 65 CD patients and 17 enrolled patients were $15.64{\pm}6.9ng/mL$ and $13.1{\pm}5.1ng/mL$, respectively. There was no correlation at the beginning of the study between vitamin D level and growth parameters (Htz, Wtz, BMIz) or other variables including laboratory data and Pediatric Crohn Disease Activity Index. The Htz, Wtz, and BMIz in Group 1 showed no significant improvement at 6 months and 12 months follow-up. In Group 2, Wtz and BMIz showed significant improvements sustained until 12 months of follow-up. Htz showed no significant improvement at 6 months but there was significant improvement at 12 months. Conclusion: It seems that baseline vitamin D status affects growth outcome in pediatric CD.

• Childhood Kidney Diseases
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• 제26권1호
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• pp.40-45
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• 2022

Purpose: Chronic kidney disease (CKD) has various underlying causes in children. Identification of the underlying causes of CKD is important. Genetic causes comprise a significant proportion of pediatric CKD cases. Methods: In this study, we performed whole-exome sequencing (WES) to identify genetic causes of pediatric CKD. From January to June 2021, WES was performed using samples from pediatric patients with CKD of unclear etiology. Results: Genetic causes were investigated using WES in 37 patients (17 males) with pediatric CKD stages 1 (n=5), 2 (n=7), 3 (n=2), 4 (n=2), and 5 (n=21). The underlying diseases were focal segmental glomerulosclerosis (n=9), congenital anomalies of the kidney and urinary tract including reflux nephropathy (n=8), other glomerulopathies (n=7), unknown etiology (n=6), and others (n=7). WES identified genetic causes of CKD in 12 of the 37 patients (32.4%). Genetic defects were discovered in the COL4A4 (n=2), WT1 (n=2), ACTN4, CEP290, COL4A3, CUBN, GATA3, LAMA5, NUP107, and PAX2 genes. WT1 defects were found in patients whose pathologic diagnosis was membranoproliferative glomerulonephritis, and identification of CUBN defects led to discontinuation of immunosuppressive agents. Genetic diagnosis confirmed the clinical diagnosis of hypoparathyroidism, sensorineural deafness, and renal disease; Alport syndrome; and Joubert syndrome in three of the patients with CKD of unknown etiology (COL4A4 [n=2], CUBN [n=1]). Extrarenal symptoms were considered phenotypic presentations of WT1, PAX2, and CEP290 defects. Conclusions: WES provided a genetic diagnosis that confirmed the clinical diagnosis in a significant proportion (32.4%) of patients with pediatric CKD.

• Advances in pediatric surgery
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• 제15권2호
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• pp.149-156
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• 2009

Contralateral groin exploration (CGE) in children with unilateral inguinal hernia remains controversial. Between January 2002 and December 2007, 1967 pediatric patients with inguinal hernia were treated by two surgeons with different criteria of CGE (group A; boys younger than 2 years, older boys prematurely delivered, and all girls, B; birth weight lower than 2 kg with inguinal hernia presentation within 6 months after birth, and suspicious physical findings) at Samsung medical center. Patient's age, sex, body weight, diagnosis, and metachronous contralateral inguinal hernia (MIH) incidence were analyzed retrospectively. Among 895 patients in group A, CGE was performed in 460 patients (66.4 %) and MIH incidence was 1.7 %. In group B, 31 patients (3.5 %) had CGE among 1072 patients, and MIH incidence was 4.2 %. The average hospital costs of group A and B were 763,956 won and 500,708 won, respectively. The CGE criteria of group B had advantage in total hospital cost. The primary site and the age at presentation had a signiticant effect on the incidence of MIH. But MIH incidence was low and the more contralateral explorations lead to increase of total costs. Therefore, routine contralateral groin exploration and surgery for a patent processus vaginalis could not be justified.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권3호
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• pp.259-265
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• 2020

Purpose: Perforation of choledochal cyst (CC) is a relatively rare clinical presentation in pediatric populations and difficult to predict preoperatively. We assess the clinical implications by comparing clinical parameters based on a single-center experience between perforated and nonperforated CC to facilitate the appropriate management for future interventions. Methods: A total of 92 cases of CC in pediatric patients (aged <18 years) who received surgical management between January 2003 and December 2018 at a Pusan National University Children's Hospital were reviewed. After screening the clinical features of perforated cases, we compared the demographic findings, clinical characteristics, and some laboratory results between the perforated and nonperforated groups. Results: Perforated CC was identified in 8 patients (8.7%), and nonperforated CC in 84 patients (91.3%). Perforation can be classified into three categories: free perforation of cyst (3 cases), pinpoint perforation of cyst (2 cases), and necrotic change of cyst (3 cases). CC perforation occurred significantly more commonly in patients aged <24 months. Clinically, the perforated group showed significantly higher frequency of fever and higher C-reactive protein (CRP) level during the initial visit. Conclusion: Perforation is more likely to be suspected in patients aged <24 months presenting together with fever and high CRP level in the initial visit. It is also necessary to keep in mind that it indicates not only a possibility of complicated disease status regardless of its association with stones but also a difficulty of applying a minimal invasive procedure and relatively increased length of hospital stay.