• Childhood Kidney Diseases
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• 제4권2호
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• pp.161-165
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• 2000

저자들은 남매에서 3주 정도의 차이를 둔 비슷한 시기에 피부의 자반성 구진, 관절의 부종 및 관절통을 주소로 내원하여 HS 자반증으로 진단받은 두명의 환아를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• 대한두개안면성형외과학회지
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• 제24권4호
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• pp.185-188
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• 2023

Rhabdomyosarcoma is the most common soft tissue sarcoma in children, accounting for 4.5% of all cases of cancer in childhood. Although the head and neck are the most common sites of rhabdomyosarcoma, oral lesions are relatively rare and account for only 10% to 12% of head and neck rhabdomyosarcoma cases. This is a case report of a girl aged 2 years and 1 month who initially presented with an upper lip mass that invaded the oral mucosa, oral skin, and nostril skin, causing narrowing of the airway. Through our case, we show that rapidly growing small round cell malignancies, especially rhabdomyosarcoma, can be effectively diagnosed and treated at the same time using primary resection with intraoperative frozen section biopsy and that the time spent waiting for the results of preoperative biopsy can be saved in this way, particularly when the patient's symptoms are intensifying rapidly and require immediate operation.

• Childhood Kidney Diseases
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• 제26권2호
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• pp.86-90
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• 2022

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis requires prompt diagnosis and treatment, since renal function at the time of diagnosis is significantly associated with renal outcomes. Here, we report two pediatric patients with ANCA-positive glomerulonephritis initially presenting with hematuria, mild proteinuria, and normal renal function. The first patient with a high myeloperoxidase-ANCA titer (>134 IU/mL) was diagnosed with rapidly progressive glomerulonephritis based on renal biopsy and treated with immunosuppressive therapy after 10 months of follow-up. The second patient with a low myeloperoxidase-ANCA titer (11 IU/mL) maintained normal kidney function without medication. Two cases showed different clinical course according to ANCA titer.

• Clinical and Experimental Pediatrics
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• 제50권6호
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• pp.580-584
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• 2007

다발성 경화증은 중추신경계 백질의 여러 부위를 시간 간격을 두고 침범하는 만성재발성 질환이다. 주로 20세에서 40세 사이에 발병하며 15세 이하 소아에서도 3-5% 정도 발병하는 소아기에는 매우 드문 질환이다. 원인은 아직 정확히 밝혀지지 않았으나 유전적, 환경적 및 감염과 연관된 자가면역반응 등 여러요인이 복합적으로 작용하는 것으로 생각하고 있다. 임상증상은 침범된 백질 부위에 따라 다양한데 사지근력 약화나 저림, 시력장애, 감각장애, 운동실조 등 다양한 증세로 호전과 재발을 반복한다. 본 저자들은 경련, 왼쪽 편마비 등의 증상으로 6세에 첫 발병 후 메틸프레드니솔론(methylprednisolon) 치료 후 증상 완전 회복 있었으나 6개월 후 경련, 두통, 왼쪽 안와주위 통증 등으로 다시 입원하는 등 4년간 추적 관찰 중 4차례 메틸프레드니솔론 치료 실시하였으나 다른 양상의 신경학적 증상으로 재발하여 Interferon-${\beta}$-1b($Betaferon^{(R)}$, SheringAG, Germany) 예방 치료를 실시한 다발성 경화증 환자 1례를 경험 하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
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• 제22권1호
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• pp.113-118
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• 2005

Diverticulosis of the small intestine is a rare entity, compared with that of duodenum or colon, and is found in only 1% of autopsied patients. The main complications are diverticulitis with or without a perforation, obstruction and hemorrhage, which are associated with a high mortality. Intussusception is primarily a disease of childhood; with only 5 to 10% of cases occurring in adults. In contrast to childhood intussusception, 90% of adult intussusception cases are had an associated pathologic processes. An inflammatory fibroid polyp is an uncommonly localized non-neoplastic lesion of the gastrointestinal tract. It occurs most often in the stomach and secondly in the ileum. It rarely occurs in other organs such as the colon, jejunum, duodenum and esophagus. We report a case of jejunal diverticulitis with a perforation combined with intussusception caused by an inflammatory fibroid polyp. A 78-year-old female presented with abdominal pain, fever and chill. Contrast CT scan showed intussusception of the ileum. The patient was treated with a small bowel segmental resection. After surgery, the specimen showed jejunal diverticulitis with perforation.

• Childhood Kidney Diseases
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• 제13권2호
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• pp.242-247
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• 2009

소아청소년기의 당뇨병은 대부분 제1형 당뇨병으로, 이 때 발생하는 혈관합병증으로서 당뇨병성 신병증은 소아에서 흔하지 않지만 신부전까지 초래할 수 있는 심각한 합병증이다. 혈당조절이 불량하고 사춘기나 그 이후에 당뇨병이 발생하는 경우에 혈관합병증의 발생이 증가하므로 소아청소년기의 당뇨병이 청소년기에 당뇨병성 신병증으로 발현하는 경우는 드물고, 더욱이 말기 신질환으로 진행하는 경우는 매우 드물다. 저자들은 혈당 조절이 불량했던 제 1형 당뇨병 소아 환자에서 혈뇨와 단백뇨가 관찰되어 조직 검사를 통해 사춘기 전에 발생한 당뇨병성 신병증을 확인하고 사춘기에 말기 신질환으로 진행한 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Advances in pediatric surgery
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• 제10권2호
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• pp.117-122
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• 2004

Diagnosis of acute appendicitis in children is sometimes difficult. The aim of this study is to validate a clinical scoring system and ultrasonography for the early diagnosis and treatment of appendicitis in childhood. This is a prospective study on 59 children admitted with abdominal pain at St. Mary's Hospital, the Catholic University of Korea from July 2002 to August 2003. We applied Madan Samuel's Pediatric Appendicitis Score (PAS) based on preoperative history, physical examination, laboratory finding and ultrasonography. This study was designed as follows: patients with score 5 or less were observed regardless of the positive ultrasonographic finding, patients with score 6 and 7 were decided according to the ultrasonogram and patients above score 8 were operated in spite of negative ultrasonographic finding. The patients were divided into two groups, appendicitis (group A) and non-appendicitis groups (group B). Group A consisted of 36 cases and Group B, 23 cases. Mean score of group A was 8.75 and group B was 6.13 (p<0.001). Comparing the diagnostic methods in acute appendicitis by surveying sensitivity, specificity, positive predictive value, negative predictive value, and accuracy, PAS gave 1.0000, 0.3043, 0.6923, 1.0000, and 0.7288, and ultrasonography gave 0.7778, 0.9130, 0.9333, 0.7241, and 0.8300 while the combined test gave 1.0000, 0.8696, 0.9231, 1.0000, and 0.9490, respectively. Negative laparotomy rate was 3 %. In conclusion, the combination of PAS and ultrasonography is a more accurate diagnostic tool than either PAS or ultrasonography.

• Childhood Kidney Diseases
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• 제24권1호
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• pp.36-41
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• 2020

Purpose: Hepatitis B virus (HBV) infection is among etiologies of secondary membranous nephropathy (MN) in pediatric patients. We evaluated expression of phospholipase A2 receptor (PLA2R), a specific target antigen of primary MN, in pediatric HBV-related MN. Methods: We retrospectively reviewed patients with biopsy-proven HBV-related MN from the renal biopsy registry and electronic medical records of Severance Hospital, Seoul, Korea, from 1993 to 2004. Paraffin-embedded human kidney tissues were retrieved and immunohistochemically stained for PLA2R. Results: Ten pediatric patients with 13 biopsied specimens were reviewed. The predominant pathological stage was stage II-III, and second was stage II. The intensity of staining for IgG was greatest, with less intense staining for IgM, IgA, C3, C4, and C1q. All the patients had angiotensin-converting enzyme inhibitor combined with glucocorticoid, and four patients converted to cyclosporine treatment from glucocorticoid monotherapy. Urinalysis of all the patients normalized after variable period. PLA2R staining was demonstrated in the outer glomerulus in 3 out of 13 biopsies, 2 of which were obtained from the same patient over a 5-year interval. Conclusions: PLA2R was expressed in a small number of cases diagnosed as pediatric HBV-related MN, indicating that some HBV-related MN cases may be primary MN concurrent with HBV infection.

• 대한유전성대사질환학회지
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• 제13권1호
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• pp.48-53
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• 2013

Tyrosinemia I (fumarylacetoacetate hydrolase deficiency) is an autosomal recessive inborn error of tyrosine metabolism that produces liver failure in infancy or a more chronic course of liver disease with cirrhosis, often complicated by hepatocellular carcinoma in childhood or early adolescence. We studied a 37-year-old woman with tyrosinemia I whose severe liver disease in infancy and rickets during childhood were resolved with dietary therapy. From 14 years of age, she resumed unrestricted diet with the continued presence of the biochemical features of tyrosinemia, yet maintained normal liver function. In adult years, she accumulated only a small amount of succinylacetone. Despite this evolution to a mild biochemical and clinical phenotype, she eventually developed hepatocellular carcinoma. Her fumarylacetoacetate hydrolase genotype consists of a splice mutation, IVS6-1G>T, and a novel missense mutation, p.Q279R. Studies of resected liver revealed the absence of hydrolytic activity and immunological expression of fumarylacetoacetate hydrolase in tumour. In the non-tumoral areas, however, 53% of normal hydrolytic activity and immunologically present fumarylacetoacetate hydrolase were found. This case demonstrates the high risk of liver cancer in tyrosinemia I even in a seemingly favorable biological environment. In this study of tyrosinemia I, Case 2 with negative succinylacetone accumulation and the recovery of acute liver failure was compared with Case 1. Diet restriction and NTBC treatment are crucial to prevent hepatocellular carcinoma until liver transplant can take place and cure the condition. Further studies are needed to examine cases where liver cancer did not result despite clinical symptoms/signs of tyrosinemia type I.

• 한국콘텐츠학회논문지
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• 제17권12호
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• pp.148-157
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• 2017

본 연구는 예비유아교사 대상 부모교육 수업을 액션러닝으로 운영한 사례를 분석하여 부모교육 수업에서 액션러닝이 어떻게 활용 될 수 있는지 그 효과를 제시하였다. 대전에 위치한 P대학교 유아교육학과에 재학중인 예비교사 32명(유아교육학과 30명, 복수전공자 2명)을 대상으로 12주간 동안 이론과 실제 액션러닝 프로그램을 실시하였다. 자료 수집은 학생들이 매주 작성한 성찰일지, 조별과제, 심층면담자료, 동료 및 자기 평가를 통해 이루어졌으며 질적 사례연구 접근으로 분석하였다. 질적 분석 결과는 다음과 같다. 액션러닝 교수법은 학생 주도적, 그룹수업 경험을 통해 다양한 사고를 접하고 사고의 폭을 확장할 수 있었다. 예비 부모로서, 예비 유아교사로서 부모로서의 역량과 부모접하기 역량을 향상시킬 수 있는 경험을 하였다. 실제 갈등 상황을 해결해 보는 경험을 통한 실천적 지식의 획득은 어려운 부분도 있었지만, 부모역할과 유아교사 역할이 힘들 수 있음을 인식하는 경험과 앞으로 발생할 수 있는 실제 상황 해결에 자신감을 가질 수 있는 계기가 되었다.