• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.91-95
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• 2002

Eosinophilic gastroenteritis, which shows characteristic eosinophilic inflammation, involves any part of the intestine from esophagus to colon. The immunopathogenesis is expected to be associated with IgE-mediated or non-IgE-mediated reaction, but the precise mechanism is not revealed yet. The clinical manifestation is variably dependent on the extent of eosinophilic infiltration. Usually the symptoms, such as abdominal pain, diarrhea, vomiting, wax and wane for a few months result in failure to thrive, which implicates the importance of early detection. Although the diagnosis is made through clinical and histopathologic evidences, we should suspect the illness in a case of anemia, hypoalbuminemia, and peripheral eosinophilia. Recently, we experienced a case of non-IgE-mediated eosinophilic colitis in a 11-year-old male who complained of diarrhea, right lower abdominal pain, body weight loss, was diagnosed with peculiar histopathologic finding of sigmoid colon specimens obtained by sigmoidoscopy.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.29-34
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• 2019

Prader-Willi Syndrome (PWS) is a rare neurologic disorder with a prevalence 1/10,000-30,000. The cause of PWS is an abnormalities of chromosome 15q11.2-q13 which is an imprinting gene. Obesity and hyperphagia are characteristic features on a PWS adult. On the other hand, the birth weight, height and body mass index (BMI) of PWS infants are 15-20% lower than those of normal babies and there is a failure to thrive until 24-month-old. Most of PWS patients are treated under general anesthesia and conscious sedation. This case is a treatment of severe caries in a PWS 46 month-old child without general anesthesia and conscious sedation. He came to the Department of Pediatric Dentistry, Kyung Hee University Dental Hospital at Gangdong, Seoul, Korea with a trauma history. He had a medical history with heart surgery and needed to take antibiotics prophylaxis. The luxated left upper primary tooth were removed and caries treatment were done as an outpatient without general anesthesia, conscious sedation.

• Clinical and Experimental Pediatrics
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• v.49 no.1
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• pp.107-111
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• 2006

Crohn disease (CD) is rare, but the incidence of CD has been increasing over the past ten years. We found two cases of CD, associated with myelodysplastic syndrome (MDS), for the first time in children. In the first patient, MDS was diagnosed at three years of age and CD developed later at eight years of age. The patient presented with recurrent abdominal pain, diarrhea, bloody stools and failure to thrive. Colonoscopy revealed cobble stone like mucosa and mass like lesions with superficial ulceration and inflammatory exudates, observed from the cecum to ascending colon. Ileo-cecal biopsy samples showed ulcers with skipped areas and lymphoid infiltrations. The patient was started on treatment with mesalazine and deflazacort, and symptoms remitted. In the second patient, MDS was diagnosed at nine years of age and CD developed at 13 years of age. This patient has recurrent hematochezia, abdominal pain, vomiting and fever. Colonoscopy revealed a large, deep indurative ulceration on the cecal side of the ileo-cecal valve. Ileocecectomy was done, and histology revealed ulceration with transmural inflammation and lymphoid aggregates. Symptoms improved after ileocecectomy.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.242-246
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• 2009

The clinical features of ring chromosome 13 include mental and growth retardation, CNS anomalies, facial dysmorphism, cardiac defects, genital malformations, limb anomalies, skeletal deformities and anal malformations. Although many cases of ring chromosome 13 have been reported worldwide, only 6 cases have been reported in Korea, and the latter cases were not mosaic but pure ring chromosome 13. Here we report a case with mosaic ring chromosome 13. The baby boy was born at 37 weeks of gestation by induced vaginal delivery due to intrauterine growth restriction (IUGR). He was the second baby of a 28-year-old hepatitis B carrier mother and a 32-year-old father. There was no family history of chromosomal anomalies. The baby was a symmetric IUGR with a birth weight of 1,860 g, length of 44.8 cm, and head circumference of 29.4 cm. The physical examination revealed microcephaly, trigonocephaly, flat occiput, large ears, short neck and dysmorphic facial features, including microophthalmia, hypertelorism, antimongoloid slanting palpebral fissures, a flat nasal bridge, and micrognathia. The karyotype of this patient performed by peripheral blood lymphocytes was 46,XY,r(13)(p13q34)/45,XY,-13/46,XY,dic r(13;13)(p13q34;p13q34). The baby showed failure to thrive, hypotonia, and developmental delay. We report the first case of mosaic ring chromosome 13 in a male baby in Korea and compare this case with other Korean cases of non-mosaic ring chromosome 13.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.2
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• pp.181-187
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• 2000

Purpose: The purpose of our study is to provide useful information for the prevalence of obesity by the standard weight for height and the relationship of the body image to dieting methods for weight control in children. Methods: The survey was performed by an anthropometry and a special questionnaire on children in grades 5 to 6 at elementary schools from May to June 1997 in Kwangju city. Results: 1) Selected individuals were categorized into obese, normal, thin by the standard weight for height and body image. The prevalence of obesity by the standard weight for height was 19.5%, normal was 70.5%, thin was 10.1% in males and respectively 10.5%, 66.3%, 23.1% in females. The prevalence of obesity in regards to body image was 18.4%, normal was 58.4%, thin was 23.2% in males and respectively 24.5%, 58.6%, 16.9% in females. 2) The obese body image was 6.8% in normal and thin groups in males and 19.4% in females. The body image of obese children who do not perceive themselves as obese was 32.6% in males and 28.7% in females. 3) The standard body image was 88.8% as realized by themselves, 77.9% when they compared themselves to friends, 62.7% as told by parents, 56.8% as told by friends, and 29.9% when they compared themselves to celebrities. 4) The source of information for dieting treatment was 44% from radio and television, 30% from books, newspapers, magazines, 17% from family and 9% from friends and seniors. 5) The prevalence of dieting according to the standard weight for height was 18.0% in thin, 18.7% in normal and 36.7% in obese group in males and respectively 17.8%, 22.4%, 46.3% in females. There was a statistically significant correlation between males and females (p<0.0005). 6) The prevalence of dieting up to 2 weeks in the thin and normal groups as recognized by weight centile was 17.3% according to body image. The prevalence of dieting up to 2 weeks in the obese groups was 37.2% according to body image (p<0.001). Conclusion: We suggest that many students perceived their body size incorrectly and tried weight control. The incorrect perception of body size seems to cause the problem of failure to thrive, malnutrition etc. It is necessary for further study to find and prevent side effects by unnecessary weight control and to provide proper health education and management about obesity.

• Journal of Gifted/Talented Education
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• v.21 no.1
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• pp.57-81
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• 2011

The purpose of this study was to explore the quality of life of musically talented students as measured by their external experiences (e.g., activities, companions) and internal experiences (e.g., flow, emotion). The participants in this study were 33 musically talented students (10 males, 23 females) aged 13 to 19. Study data were collected for 7 consecutive days using the Experience Sampling Method (ESM), which employs a cellular-phone as a signaling device. The results were as follows: First, in response to the 1625 random signals, musically talented students reported that 40.9% of their time was spent on productive activities. An additional 33.4% of time was used for maintenance activities and the rest of their time was spent on leisure/social activities. Also, musically talented students reported that 48.5% of their time was spent alone. When they were alone, they spent a lot of time engaging in productive activities (44.3%). Second, in order to measure the flow of their life, two methods were used. One used a 4-channel flow model (i.e. apathy, boredom, flow, anxiety) and the other used 8 dimensions and conditions of the flow experience (i.e. concentration, self-consciousness disappears, action and awareness merge, distorted sense of time, freedom from worry about failure, clear goals, immediate feedback, balance between challenges and skills). According to the former, when engaged in music-related activities, musically talented students usually reported flow (54.0%), while they felt apathy (41.3%) for daily routines activities. According to the latter method, musically talented students experienced flow for most productive activities, while they experienced flow least for maintenance activities. Emotional variables of ESF are comprised of 10 semantic scales (i.e. happy-sad, strong-weak, active-passive, sociablelonely, proud-ashamed, involved-detached, excited-bored, clear-confused, relaxed-worried, cooperative-competitive). Musically talented students reported experiencing the most positive emotion for social activities and experiencing the most negative emotion for maintenance activities. Results of this study assert that musically talented students had to trade off immediate enjoyment for developing their special gifts. They could not afford as much time for socializing with friends, and they had to spend more time alone compared to their peers without such gifts. Consequently, they were found to deprive themselves of the spontaneous good times that teenagers usually thrive on. They were helped in this respect by their autotelic personality traits, especially their strong need for achievement and endurance. The downside, however, is that the moment-to-moment quality of their moods suffered. The argument concerning musically talented students applies for all adolescents. The choices that talented students must make between immediate gratification and long-term development, and between solitude and companionship, are the same choices every young person must make, regardless of her or his level of talent. All of us have gifts that are potentially useful and worthy of being appreciated. But to develop these latent talents we must cultivate them, and this takes time and the investment of mental energy. The lifestyle that musically talented students develop can show us some of the choices all of us must make in order to cultivate our gifts.

• 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)

• 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.