• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.80-84
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• 2010

Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.163-171
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• 2011

Purpose : To evaluate the prevalence of vesicoureteral reflux (VUR) according to the timing of voiding cystourethrography (VCUG) in infantile urinary tract infection (UTI). Methods : The data of 134 infants (1-12 months) with renal cortical defect in $^{99m}Tc$-2, 3-dimercaptosuccinic acid ($^{99m}Tc$-DMSA) scan with a diagnosis of UTI in two hospitals from 2000 to 2010 were retrospectively analyzed. The VCUG was performed after 2 weeks from the diagnosis of UTI in Group I (n=68), and the VCUG was performed within 2 weeks from the diagnosis of UTI in Group II (n=66). Results : There were no significant differences between the two groups in the duration of fever, white blood cell count, C-reactive protein levels, and abnormalities in ultrasonography (P>0.05). There was no significant difference between the two groups in the prevelence of VUR, bilateral VUR, and severe VUR. VCUG-induced UTI was detected 16 (23.5%) of patients in whom the procedure was performed 2 weeks after the diagnosis, and none of VCUG-induced UTI occurred in those in whom the procedure was performed 2 weeks within the diagnosis. Conclusion : We conclude that the prevalence of VUR according to the timing of VCUG did not differ between the two groups in infantile UTI with renal cortical defect in DMSA scan. We also found that performing VCUG with antibiotics can decrease risk of VCUG-induced UTI.

• Journal of dental hygiene science
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• v.8 no.4
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• pp.283-289
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• 2008

This study was performed to obtain data about dental health to develop childrens' dental health education program. This research surved from from 22 July 2008 to 11 August 2008 and questionaire given to 140 teacher from Gwangsan-gu and Buk-gu and Dong-gu in Gwangju city. Collected data were analyzed with SPSS 12.0 program. The results of this study were as follows: 1. The dental health condition which teacher is recognizing is most with the facility 52.9% which is healthy, one day toothbrush qualitative number of times 3 times 67.1% and the dental floss qualitative number of times is 49.3%. Uses most the oral hygiene device which plentifully is 51.4% with the dental gargle solution, the dental problem which is knowing dental caries with 45.7% to be highest, perception dental sensitivity was 30.7%. 2. Periodical dental prosecuting says that 93.6% is executing, also the case which a dental inspection result in the parents notifies was 78.6%, after notifying treat, the case which confirms was 60.7%. The case which is the toothbrush qualitative time which is systematic was 95.0%, about dental healthily and food the case which executes an education was 93.6%. 3. About educational background by infantile dental health care of teacher with recognition currently the effectiveness of dental health care is higher from below junior college graduating (p < 0.05). dental hygienic relation experience event participation intention (p < 0.01) comes from the above of university graduation from infantile dental health care from important degree of teacher role was higher the junior college graduation group (p < 0.05). 4. Recognition there was not a difference which is beneficial with an educational background by about career by infantile dental health care of teacher. For the buccal cavity hygiene management of the infants thinks with the fact that also the development of the curricular material and the educational program and teacher buccal cavity hygiene education will be necessary.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.31-38
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• 2006

Purpose: Infantile constipation is one of the most common problems in pediatric gastrointestinal outpatient clinic. We planed to show the clinical feature of infantile constipation and explore the possible relationship between diets and symptoms of constipation. Methods: We analyzed the medical records and telephone questionnaire about infants under 2 years of age with constipation, who visited outpatient clinic of Department of Pediatrics, Ilsan Paik Hospital during the time from March 2002 to February 2005. Data including the symptoms and signs of constipation, diet history, and past-medical history were analyzed. Results: Total 96 infants, 40 male and 56 female, were enrolled in this study. The mean age was 9 months. Twenty-three infants were exclusively breast milk fed (BMF), 20 infants were cow's milk fed (CMF) and 53 infants had a history of mixed feeding with cow's milk and breast milk (MMF). Patients showed painful defecation (95.8%), abdominal distension (53.1%), palpable rectal stool (35.1%), hard stool (30.2%), blood-tinged stool (29.2%) and anal fissure (16.7%). Patients with exclusive cow milk feeding had prominent clinical features, such as anal fissure (p=0.03), hematochezia (p=0.04) and palpable rectal stool (p=0.025). Patients who had a history of larger intake of liquid food had a tendency to get anal skin tag (p<0.05). Conclusion: Exclusive breast milk feeding seemed important to avoid constipation with clinical significance. To educate caregivers in appropriate way of the weaning food may help the infants with constipation.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.62-67
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• 2018

Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.4
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• pp.366-376
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• 2020

Purpose: To assess the association between birth weight and the development of functional gastrointestinal disorders (FGIDs) in the first year of life. Methods: This is a secondary analysis of a prospective cohort multicenter study including neonates, consecutively enrolled at birth, and followed up for one year. At birth all infants were classified by birth weight as extremely low (ELBW), very low, or low when <1,000, <1,500, and <2,500 g, respectively, and by birth weight for gestational age as appropriate (AGA, weight in the 10-90th percentile), small (SGA, weight <10th percentile), and large (LGA, weight >90th percentile) for gestational age. FGIDs were classified according to the Rome III criteria and assessed at 1, 3, 6, and 12 months of life. Results: Among 1,152 newborns enrolled, 934 (81.1%) completed the study: 302 (32.3%) were preterm, 35 (3.7%) were ELBW, 104 (11.1%) were SGA, 782 (83.7%) were AGA, and 48 (5.1%) were LGA infants. Overall, throughout the first year of life, 718 (76.9%) reported at least one FGID. The proportion of infants presenting with at least one FGID was significantly higher in ELBW (97%) compared to LBW (74%) (p=0.01) and in LGA (85.4%) and SGA (85.6%) compared to AGA (75.2%) (p=0.0001). On multivariate analysis, SGA was significantly associated with infantile colic. Conclusion: We observed an increased risk of FGIDs in ELBW, SGA, and LGA neonates. Our results suggest that prenatal factors determining birth weight may influence the development of FGIDs in infants. Understanding the role of all potential risk factors may provide new insights and targeted approaches for FGIDs.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.3 no.1
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• pp.147-157
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• 1992

The fragile X syndrome, which is considered to be synonymous with the Martin-Bell syndrome, is a relatively common form of X-linked mental retardation. The syndrome seems to occure in many different ethnic groups and its prevalence among mentally retarded males has been estimated to be in the order of 2 to 6%. The karyotypic hallmark of the syndrome is made up with a pronounced constriction near each tip of the long arm of the X chromosome(fragile site), shown in vitro only under conditions in which thymidylate production is blocked(lowered folate levels). Special culture media are needed to demonstrate this constriction site. Major clinical features associated with the syndrome include macroorchidism, large or prominent ears, significant emotional and behavioral dysfunctions such as hyperactivity, self-injury, lack of eye contact and social interaction, schizophrenia, autism, etc., and speech and language dysfunctions ranging from nonverbal to verbal speech with moderate to severe expressive language delays. Some have minor clinical features in common such as an increase in birth weight high forehead, prognathism, increased head circumference in infancy and childhood which did not persist into adult life. The recent research findings have shown that the fragile X syndrome is associated with infantile autism. Many patients with the fragile X syndrome fulfill the diagnostic criteria for infantile autism. Therefore it is recommendable that any patient with developmental delays and autism or autistic manifestations should have a chromosomal analysis, including fragile X examination. In the present review, historical aspects, incidence, and clinical features are presented. Recent anecdotal reports of the association with autism and the clinical improvement following high dose folic acid treatment will be discussed.

• Journal of Korean Physical Therapy Science
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• v.3 no.2
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• pp.1055-1064
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• 1996

To this is the concept and changes of physical treatment that intervenes the understanding and restoring cerebral infantile paralysis in the medical welfare of the social well-being areas: Cerebral palsy, originated by Littel in 1862 and reported as "The cerebral palsies" by William and Osler, come to have its name and meaning. The early stage of intervention generally accepted by the circle of physical treatment in the medical profession was introduced by Sax in 1964. In Sax' theory, cerebral palsy is defined as "the stumbling block in the bodily exercises and postures, causing by the intercurrent disease or physical, mental defect." As we have seen it is desirable that the abovementioned limited steps be removed and allow physical therapists to examine and to treat cerebral palsies. For this, first educational system should make its way to a substantial improvement and congenital abnormal Test should be rapidly expanding to great proportions the whole nation, not part of the low-income group. Physical therapists, cerebral palsies and their parents must combine to make cerebral palsies' welfare the crowning well being state. Physical therapists, cerebral palsies and their parents, forming of trinity, should combine to make cerebral palsies' welfare the Super-Welfare State existence.

• The Journal of Pediatrics of Korean Medicine
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• v.29 no.4
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• pp.90-96
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• 2015

Objectives The purpose of this study was to report 35 children who had sleep disorder like night crying or night terror after traffic accidents. Methods This clinical study had been carried out with 35 infants and children, who visited to the department of Pediatrics, ${\bigcirc}{\bigcirc}$ Oriental Medical Hospital from January 2012 to June 2015. Results The study group had similar sex the ratio between boys and girls and the group had more of 1-3 year-old children. The number of patients who were diagnosed as night crying were 24, night terror were 7 and palpitation due to alarm were 4. All patients took herb medicine and 15 patients were treated by acupuncture. During the treatment, symptom of night crying and terror were disappeared. Conclusions This result showed that the oriental medical treatment was effective in children who suffered from night crying or night terror after traffic accidents.

• Childhood Kidney Diseases
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• v.16 no.1
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• pp.58-62
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• 2012

Urinary tract infection (UTI) is most commonly diagnosed bacterial infection in febrile infants. Renal abscess is a very rare complication of UTI in children. Early diagnosis and treatment with appropriate antibiotics are important because renal scar correlates positively with the time of treatment. Renal ultrasonography and abdominal computerized tomography facilitates an earlier diagnosis and is also useful in establishing percutaneous drainage. Extended broad spectrum antibiotics therapy alone can be effective in most types of renal abscesses in infant, but some antibiotics-resistant cases need surgical drainage or nephrectomy. We report a case of a infant UTI, that progressed to renal abscess despite early antibiotic treatment and was treated with US guided percutaneous needle aspiration.