• 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.

• 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.

• Childhood Kidney Diseases
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• 제19권1호
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• pp.14-22
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• 2015

Renal scintigraphic methods, which are physiologic and minimally invasive, have played important role in the management of various renal diseases in children, providing sensitive tool for early detection of disease even before structural changes become prominent and giving valuable functional and anatomical information to aid medical decision makings in the treatment and follow-up of patients. This review article focuses on the concept and advantages of renal scintigraphy in pediatric patients with various urologic diseases.

• 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.

• 대한소아치과학회지
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• 제24권4호
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• pp.743-750
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• 1997

Gingivitis is the most prevalent type of periodontal disease and the dental plaque is considered as a major contributory factor. As the poor oral hygiene is firmly related to the occurrence of periodontal disease, pediatric dentist should make every effort to promote the oral health and control the plaque effectively for the high risk patients, especially for those who are under orthodontic treatment. P.M.T.C.(Professional Mechanical Tooth Cleaning), introduced by Dr. P. Axelsson in 1969, is a very effective method of plaque removal and can be performed by specially trained personnel. Two pediatric orthodontic patients were treated by P.M.T.C. for the elimination of gingivitis and gingival swelling. Signi ficant improvements of gingival condition were achieved in both cases but additional preventive programs and home care along with professional office care seem to be necessary for the best result.

• Clinical and Experimental Pediatrics
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• 제60권5호
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• pp.129-137
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• 2017

The event-free survival (EFS) for pediatric acute lymphoblastic leukemia (ALL) has shown remarkable improvement in the past several decades. In Korea also, a recent study showed 10-year EFS of 78.5%. Much of the improved outcome for pediatric ALL stems from the accurate identification of prognostic factors, the designation of risk group based on these factors, and treatment of appropriate duration and intensity according to risk group, done within the setting of cooperative clinical trials. The schema of first-line therapy for ALL remains mostly unchanged, although many groups have now reported on the elimination of cranial irradiation in all patients with low rates of central nervous system relapse. Specific high risk subgroups, such as Philadelphia chromosome-positive (Ph+) ALL and infant ALL continue to have significantly lower survival than other ALL patients. The introduction of tyrosine kinase inhibitors into therapy has led to enhanced outcome for Ph+ ALL patients. Infant ALL patients, particularly those with MLL rearrangements, continue to have poor outcome, despite treatment intensification including allogeneic hematopoietic cell transplantation. Relapsed ALL is a leading cause of mortality in pediatric cancer. Recent advances in immunotherapy targeting the CD19 of the ALL blast have shown remarkable efficacy in some of these relapsed and refractory patients. With improved survival, much of the current focus is on decreasing the long-term toxicities of treatment.

• 대한장애인치과학회지
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• 제3권1호
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• pp.6-10
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• 2007

Many disabled patients need extensive dental treatment because they have much difficulty in maintaining their oral hygiene. However, because they are not cooperative and not manageable, they require physical restraints, drug induced sedation or general anesthesia. General anesthesia is useful in control of the patients who cannot be treated in other ways. Additionally, general anesthesia provides more safe environment for medically compromised patients. The aim of the present study was to investigate a kind of disorder, sources of referral, preoperative oral condition, experience of dental visit, and performing general anesthesia or not of 930 disabled patients in Kyung-Hee University dental hospital for 30 years.

• Advances in pediatric surgery
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• 제16권1호
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• pp.11-17
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• 2010

Pancreatic tumors in children are relatively rare, and their prognosis differs from that in adults. The purpose of this study is to examine the clinical characteristics, treatment, and prognosis for children with pancreatic tumors. We retrospectively reviewed the medical records of children under 15 years of age with pancreatic tumors who were treated surgically at Asan Medical Center between January 1992 and November 2009. There were 16 patients, fourteen of whom were pathologically diagnosed with solid pseudopapillary tumor. The other two patients were diagnosed with pancreatoblastoma and acinar cell carcinoma, respectively. Six patients of the 16 patients (38 %) were male, and there was a male-to-female ratio of 1:1.6. The initial presentations were upper abdominal pain in eight patients (50 %), palpable abdominal mass in three, and vomiting in one. Four patients were diagnosed incidentally. Six patients' tumors were located in the pancreatic head, six in the pancreatic body, and four in the pancreatic tail, respectively. The surgical procedures performed included distal pancreatectomy (n=7, 44 %), median segmentectomy (n=3), enucleation (n=3), pancreaticoduodenectomy (n=2), and pylorus-preserving pancreaticoduodenectomy (n=1). Three patients underwent laparoscopic surgery. The median tumor size was 6.5 cm (1.8~20 cm). Early surgical complications included pancreatic fistula (n=4), bile leakage (n=1), and delayed gastric emptying (n=1). A late complication in one patient was diabetes. The median follow-up period was five years and four months, and all patients survived without recurrence. While pancreatic tumors in adults have a poor prognosis, pancreatic tumors of childhood are usually curative with complete resection and thus have a favorable prognosis.