Lee, Ye Seung;Kim, Hui Kwon;Kim, Hye Rim;Lee, Jong Yoon;Choi, Joong Wan;Bae, Eun Ju;Oh, Phil Soo;Park, Won Il;Ki, Chang Seok;Lee, Hong Jin
Clinical and Experimental Pediatrics
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v.57
no.5
/
pp.240-244
/
2014
Pseudohypoparathyroidism type Ia (PHP Ia) is a disorder characterized by multiform hormonal resistance including parathyroid hormone (PTH) resistance and Albright hereditary osteodystrophy (AHO). It is caused by heterozygous inactivating mutations within the Gs alpha-encoding GNAS exons. A 9-year-old boy presented with clinical and laboratory abnormalities including hypocalcemia, hyperphosphatemia, PTH resistance, multihormone resistance and AHO (round face, short stature, obesity, brachydactyly and osteoma cutis) which were typical of PHP Ia. He had a history of repeated convulsive episodes that started from the age of 2 months. A cranial computed tomography scan showed bilateral calcifications in the basal ganglia and his intelligence quotient testing indicated mild mental retardation. Family history revealed that the patient's maternal relatives, including his grandmother and 2 of his mother's siblings, had features suggestive of AHO. Sequencing of the GNAS gene of the patient identified a heterozygous nonsense mutation within exon 11 (c.637 C>T). The C>T transversion results in an amino acid substitution from Gln to stop codon at codon 213 ($p.Gln213^*$). To our knowledge, this is a novel mutation in GNAS.
Background: Attention deficit hyperactivity disorder (ADHD) symptoms have a major impact on individuals, families, and society. Therefore identification risk factors of ADHD are a public health priority. Purpose: This is meta-analysis evaluated the association between maternal prepregnancy body mass index and the risk of ADHD among the resulting offspring. Methods: The search identified studies published through December 2018 in the PubMed, Web of Science, and Scopus databases. The odds ratios (ORs) or hazard ratios (HRs) with 95% confidence intervals (CI) extracted from eligible studies were used as the common measure of association among studies. Results: A significant association was found between overweight women and the risk of ADHD among children with the pooled HR and OR estimates (HR, 1.27 and 95% CI, 1.17-1.37; OR, 1.28 and 95% CI, 1.15-1.40, respectively). This association was significant between obese women and the risk of ADHD among children and adolescents with the pooled estimates of HR and OR (HR, 1.65 and 95% CI, 1.55-1.76; OR, 1.42 and 95% CI, 1.23-1.61). Conclusion: The current epidemiological studies present sufficient evidence that prepregnancy overweight and obesity are significantly associated with an increased risk of ADHD among children and adolescents. These findings provide a new approach to preventing ADHD by controlling weight gain in the prenatal period, which should be considered by policymakers.
Yoon, Jong Wan;Park, Hyun A;Lee, Jieun;Kim, Jae Hyun
Clinical and Experimental Pediatrics
/
v.60
no.12
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pp.395-402
/
2017
Purpose: The potential effect of gonadotropin-releasing hormone agonist (GnRHa) treatment on the weight of girls with central precocious puberty (CPP) remains a controversy. We investigated anthropometric changes during and after GnRHa treatment among girls with CPP. Methods: This retrospective study evaluated data from 127 girls with CPP who received GnRHa treatment for ${\geq}2years$. Height, weight, and body mass index (BMI) values were compared at the baseline (visit 1), after 1 year of GnRHa treatment (visit 2), the end of GnRHa treatment (visit 3), and 6-12 months after GnRHa discontinuation (visit 4). Results: The height z score for chronological age (CA) increased continuously between visit 1 and visit 4. No significant differences were observed in BMI z score for CA between visits 1 and 4. However, an increasing trend in the BMI z score for bone age (BA) was observed between visits 1 and 4. The numbers of participants who were of normal weight, overweight, and obese were 97, 22, and 8, respectively, at visit 1, compared to 100, 16, and 11, respectively, at visit 4 (P=0.48). Conclusion: Among girls with CPP, the overall BMI z score for CA did not change significantly during or after GnRHa treatment discontinuation, regardless of their BMI status at visit 1. However, the BMI z score for BA showed an increasing trend during GnRHa treatment and a decreasing trend after discontinuation. Therefore, long-term follow-up of BMI changes among girls with CPP is required until they attain adult height.
Gonadotropin-releasing hormone analogs (GnRHa) are widely used to treat central precocious puberty (CPP). The efficacy and safety of GnRHa treatment are known, but concerns regarding long-term complications are increasing. Follow-up observation results after GnRHa treatment cessation in female CPP patients up to adulthood showed that treatment (especially <6 years) was beneficial for final adult height relative to that of pretreated or untreated patients. Puberty was recovered within 1 year after GnRHa treatment discontinuation, and there were no abnormalities in reproductive function. CPP patients had a relatively high body mass index (BMI) at the time of CPP diagnosis, but BMI standard deviation score maintenance during GnRHa treatment seemed to prevent the aggravation of obesity in many cases. Bone mineral density decreases during GnRHa treatment but recovers to normal afterwards, and peak bone mass formation through bone mineral accretion during puberty is not affected. Recent studies reported a high prevalence of polycystic ovarian syndrome in CPP patients after GnRHa treatment, but it remains unclear whether the cause is the reproductive mechanism of CPP or GnRHa treatment itself. Studies of the psychosocial effects on CPP patients after GnRHa treatment are very limited. Some studies have reported decreases in psychosocial problems after GnRHa treatment. Overall, GnRHa seems effective and safe for CPP patients, based on long-term follow-up studies. There have been only a few long-term studies on GnRHa treatment in CPP patients in Korea; therefore, additional long-term follow-up investigations are needed to establish the efficacy and safety of GnRHa in the Korean population.
Lim, Chang Hoon;Kim, Eun Ji;Kim, Jong Hyun;Lee, Jue Seong;Lee, Yoon;Park, Sang Hee
Clinical and Experimental Pediatrics
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v.60
no.1
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pp.17-23
/
2017
Purpose: To examine the correlation of depression with Internet use and body image perception, and to analyze the risk factors of depression in a total of 920 students in Seoul, Korea. Methods: Students were recruited by contacting school principals and teachers and were encouraged to fill out a self-report questionnaire designed specifically for this study in July of 2008. Results: Female participants had an increased risk for depression than did male participants (adjusted odds ratio [aOR], 1.790; 95% confidence interval [CI], 1.330-2.410, P<0.001). Older students were more susceptible to depression (aOR, 1.246; 95% CI, 1.115-1.392, P<0.001). Longer daily Internet use and more frequent Internet use were analyzed as risk factors for depression. No physical activity was a risk factor for depression (aOR, 0.392; 95% CI, 1.264-4.526, P=0.014). Dissatisfaction with one's body image increased the risk for depression (aOR, 1.373; 95% CI, 1.169-1.613; P<0.001). Obesity and perception of body image showed no significant relationship with increased risk for depression. Conclusion: Prevalence of depression was 13.8% in adolescents in Seoul, Korea, in July 2008. Female sex, age, daily Internet use duration, weekly Internet use frequency, physical activity, and dissatisfaction with one's body image independently increased risk of depression.
McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children's Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She underwent abdominal sonography, magnetic resonance imaging, genitography and cystoscopy which confirmed HMC with a transverse vaginal septum. X-rays of the hand and foot showed bony fusion of the left third and fourth metacarpal bones, right fourth dysplastic metacarpal bone and phalanx, right PAP and hypoplastic left foot with left fourth and fifth dysplastic metatarsal bones. In addition, she had soft palate cleft, mild hydronephroses of both kidneys, hypoplastic right kidney with ectopic location and mild rotation, uterine didelphys with transverse vaginal septum and low-type imperforated anus. She was temporarily treated with ultrasound-guided transurethral aspiration of the HMC. Our patient with HMC and PAP was diagnosed with MKS because she has two typical abnormality of MKS and she has no definite complications of retinal disease, learning disability, obesity and renal failure that develop in Bardet-Biedl syndrome, but not in MKS until 33 months of age. Here, we describe a case of a Korean patient with MKS.
Background: The seropositivity rate of hepatitis B surface antigen (anti-HBs) antibodies is known to be ≥95% after hepatitis B virus vaccination during infancy. However, a low level or absence of anti-HBs in healthy children is discovered in many cases. Recent studies in adults reported that a reduced anti-HBs production rate is related to obesity. Purpose: To investigate whether body mass index (BMI) affects anti-HBs levels in healthy children following 3 serial dose vaccinations in infancy. Methods: We recruited 1,200 healthy volunteers aged 3, 5, 7, or 10 years from 4-day care centers and 4 elementary schools. All subjects completed a questionnaire including body weight, height, and vaccine type received. Levels of serum hepatitis B surface antigen (HBsAg) and anti-HBs in all subjects were analyzed using electrochemiluminescence immunoassay. The standardized scores (z score) for each sex and age were obtained using the lambda-mu-sigma method in the 2017 Korean National Growth Charts for children and adolescents. Results: Our subjects (n=1,200) comprised 750 males (62.5%) and 450 females (37.5%). The overall anti-HBs seropositivity rate was 57.9% (695 of 1,200). We identified significant differences in mean BMI values between seronegative and seropositive groups (17.45 vs. 16.62, respectively; P<0.001). The anti-HBs titer was significantly decreased as the BMI z score increased adjusting for age and sex (B=-15.725; standard error=5.494; P=0.004). The probability of anti-HBs seropositivity based on BMI z score was decreased to an OR of 0.820 after the control for confounding variables (95% confidence interval, 0.728-0.923; P=0.001). Conclusion: There was a significant association between anti-HBs titer and BMI z score after adjustment for age and sex. Our results indicate that BMI is a potential factor affecting anti-HBs titer in healthy children.
The purpose of this study was to develop cardiovascular health related percent body-fat standards that may be applied to epidemiologic investigations of the prevalence and incidence of obesity in adolescents, pediatric health screenings, and youth fitness tests. The subjects included 102 males and 80 females aged 19~22years. All subject were Honam University Students Total body fat was derived from body density which was estimated from age and the triceps and subscapular skinfold thickness measured with Lang calipers to the nearst l.0mm. Serum total cholesterol and lipoprotein cholesterol fraction(HDL-CLDL-C) were measured from blood obtained from fore arm vein after blood pressure measurement. In analyses to determine critical fat levels associated with elevated CDD(Chronic Degenerative Disease) risk factors;male and female were grouped by level of percent fat as follows: male, 〈 10%, 10-14.9%, 15-19.9%, 20-24.9%, and $\geq$ 25%;female, 〈 20%, 20-24.9%, 25-29.9%, 30-34.9%, and $\geq$ 35%. As the results of the data, the conclusions were as follows: 1. A dose respones effect was observed between blood pressure and percent body fat in males and females; in contrast, total cholesterol and lipoprotein ratios were relatively independant of percent fat among the lower four fatness group in males and the lower three fatness groups in females. 2. The percentage of subjects in the uppermost quintile for S-Bp, D-Bp, TC, LDL/H was significantly(P〈.05) greater than expected by change alone(20%) in males with $\geq$ 25% fat and in females with $\geq$ 30% fat females with $\geq$35% had even greater representation in the uppermost quintile of all CDD risk factors compared to females with 30-34.9% fat.
Renal cortical necrosis (RCN) is patchy or diffuse ischemic destruction of the renal cortex caused by significantly reduced renal arterial perfusion. It is a rare cause of acute kidney injury (AKI) and is associated with high mortality. Here, we review the case of RCN in a 15-year-old boy who developed AKI. A 15-year-old boy was referred to our hospital from a local hospital due to a sharp decrease in his renal function. He presented with acute flank pain, nausea with vomiting, and oliguria for the past two days. He had taken a single dose of antihistamine for nasal congestion. At our hospital, his peak blood pressure was 148/83 mmHg and he had a high body mass index of $32.9kg/m^2$. The laboratory data showed a blood urea nitrogen (BUN) of 28.4 mg/dL, a creatinine of 4.26 mg/dL, and a glomerular filtration rate estimated from the serum cystatin C of $20.2mL/min/1.73m^2$. Proteinuria (spot urine protein to creatinine ratio 1.66) with pyuria was observed. Kidney sonography showed parenchymal swelling and increased renal echogenicity. Due to rapidly progressing nephritis, steroid pulse therapy (750 mg/IV) was done on the second day of his admission and the patient showed complete recovery with normal renal function. However, the kidney biopsy findings revealed renal cortical hemorrhagic necrosis. Multifocal, relatively well-circumscribed, hemorrhagic necrotic areas (about 25%) were detected in the tubulointerstitium. Although RCN is an unusual cause of AKI, especially in children, pediatricians should consider the possibility of RCN when evaluating patients with rapidly decreasing renal function.
Kim, Hyun-Jun;Kim, Tae-Un;Lee, Sangyeoup;Shin, Goon-Soo;Kim, Young-Joo;Kim, Su-Yung
Clinical and Experimental Pediatrics
/
v.49
no.9
/
pp.946-951
/
2006
Purpose : The purpose of this study was to demonstrate the effectiveness of combined exercise for 12 weeks on the adiponectin and obesity related variables in overweight and obese children. Methods : Eighteen children in 5th grade in a certain elementary school in Busan were recruited. They were all overweight or obese children(more than 85 percentile in body mass index). Nine children in the experimental group were given exercises consisting of walking and band resistant training for 12 weeks. Auxological data(including height, weight and body fat mass) and laboratory data (fasting blood sugar, insulin, adiponectin) were checked at baseline and at the 1 week, and at the 4 weeks and 12 weeks stages of their exercise program. Insulin resistance and sensitivity were evaluated indirectly using HOMA index and QUICKI index. Results : Adiponectin gradually decreased until the 4 weeks point and gradually increased thereafter to the starting level at the 12 weeks stage. Body weight, body mass index(BMI) and HOMA index significantly decreased more at the 1 week, 4 weeks, and 12 weeks stages in the experimental group than in the control group. Body fat mass significantly decreased at 12 weeks. The change of insulin was significantly correlated with changes of body weight and BMI. But there was no correlation between changes of adiponectin and changes of insulin. Conclusion : Exercise seems to effect the adiponectin concentration. And it might be assumed that exercise increases the adiponectin concentration if it is continued for long time(may be more than 12 weeks). More studies may be necessary to draw that conclusion.
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