Minji Im;Chiwoo Kim;Juyoung Sung;Insung Kim;Ji-Hoon Hwang;Min-Sun Kim;Sung Yoon Cho
Journal of Genetic Medicine
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v.20
no.2
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pp.60-69
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2023
Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients-four with MPS I and five with MPS II-underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from -4.09±0.83 to -3.68±0.43 after 1 year of hGH treatment, and to -3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion: This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.
Objectives : The aim of this study was to evaluate the effect to the height-growth after the administration of herbal medicine to the prepuberty children Methods : This clinical study has been carried out with 18 children, who visited to the Department of Pediatrics, Dong-eui oriental medical center from January 2005 to August 2008. They had been treated for height-growth with herbal medicine more than 6 months. This study has been carried out by chart-review to see the effectiveness. Height percentile, BMI and GV(Growth Velocity) after medication was compared with the ones before medication. Results : 1. This clinical study has been carried out with 18 children, 12 boys(66.7%) and six girls(23.3%). Their average age was 10.30 years old. 2. Generally total children's average height percentile was increased compared to after medication. Especially eight of them has significantly increasd height percentile(44.4%). 3. Total children's average GV/6months was also increased after medication. Especially ten of them has significantly increasd GV/6months (55.6%). 4. Total children's average BMI also increased (17.01 to 17.85) after medication. Conclusions : Herbal medicine affects to the growth of pre-puberty children.
This study were conducted to assess the physical growth and developmental status of infants in orphanage in order to provide an empirical data. The subjects for this study were 104 infants and toddlers who were reared in an orphanage in D Metropolitan city. The instrument used for this study were anthropometric assessment and DDST for normative data of development. Data has been collected from September 1st, 1998 to August 31st, 2000 and were analyzed using SPSS/PC(Version 10.0) with frequency, mean, standard deviation, ANOVA and Chi-square test. The results of this study were as follows; 1. 30.8% of infants in orphanage had abnormal weight, 26.9% had abnormal length, and 22.1% had abnormal head circumference and most of them were distributed below 50 percentile of growth chart. 2. 53.8% of infants in orphanage had normal, 27.9% had qustionable, and 18.3% had abnormal developmental screening test results, especially, 31.5% of infants in orphanage ages 3 to 5 years had abnormal developmental screening test results, according to the Denver Developmental Screening Test(DDST). There was a significant developmental delay noted in the language and fine motor-adaptive sector. 3. It is anticipated that developmental delays would increase in severity by older the mean age of orphanage infants and longer the time being raised in orphanage. It would be concluded that the physical growth and developmental status of orphaned infants were very vulnerable and serious and it is suggested that there needed an effective intervention strategies to promote growth and development of infants in orphanage.
Purpose: Currently lung cancer ranks second in cancer for incidence rate and is a disease that ranks first for a death rate by cancerous growth because it is already advanced at the time of diagnosis. The purpose of this paper was to analyze the factors that affect the effectiveness of and rash occurrence by Epidermal Growth Factor Receptor Tyrosine Kinase Inhibitor (EGFR TKI) in patients with non-small cell lung cancer. Methods: A retrospective chart review of 100 patients, who took EGFR TKI (erlotinib, gefitinib) among patients who were diagnosed with non-small cell lung cancer in a Hospital in Korea between May 2005 and February 2008, was conducted. The drug effectiveness was evaluated by Response Evaluation Criteria In Solid Tumor. Results: EGFR mutation was the only factor associated with drug response (complete response and partial response). When stable disease was added to drug response as the evaluation parameter, ECOG and rash as well as EGFR mutation were found to be important factors. Survival, however, was not affected by EGFR mutation. The factors influenced on survival were older age (${\geq}65$), low ECOG ($1{\sim}2$), adenocarcinoma and rash. In the case of rash, group with EGFR mutation or low ECOG showed significantly higher chance of occurrence. There was no significant difference in rash occurrence between gefitinib and erlotinib groups. Conclusions: Based on the results, EGFR mutation positive and low ECOG ($1{\sim}2$) were significantly important factors for both effectiveness of EGFR TKI and rash occurrence. Also, rash itself was found to be an independently significant factor for the disease control and survival. Therefore, while administering EGFR TKI, patients who have the factors associated with rash occurrence should be closely monitored for effective and safe drug therapy.
For the purpose of ascertaining the status of physical growth and development of the University students, an intensive survey was conducted by the authors towards a total of 2,197 (male: 1,694, female: 503) who passed the written enterance examination at the Korea University in 1972. The items included the measurements on physical growth, various physical indices, status of visual distourbance and pulmonary infiltration. The findings and results can be summarized as follows. 1. Physical growth: The average of body height by sex were 167.2 cm in male and 156.0cm in female, body weight 59.3kg and 51.9kg, chest-girth 86.7cm and 81.2cm, sitting height 91.7cm and 85.8cm, respectively. 2. Various indices about physical growth & development; Relative body weight by sex were 35.47 and 33.27, relative chest girth 51.85 and 52.05, relative sitting height 54.84 and 55.00, respectively. $R{\ddot{a}}here$ index was 1,269 in males and 1,367 in females, Kaup index 2,121 and 2,133, Verveck index 87.32 and 85.32, respectively. 3. Status of visual distaurbance: The vision of the students under 0.9 with Han's testing chart was 52.83% and 55.27% in left eye and 53.60% and 54.87% in right eye, respectively. 4. Pulmonary infiltration Among the total 58 students were diagncsed as pulmonary infiltration (tuberculosis), of whom 43 were minimal case, 12 were moderately advanced cases and 3 were far advanced cases.
Azouz, Vitali;Ng, Marilyn;Patel, Niyant;Murthy, Ananth S.
Maxillofacial Plastic and Reconstructive Surgery
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v.42
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pp.8.1-8.5
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2020
Background: The cause of maxillary growth restriction in patients with cleft lip and palate remains controversial. While studies have investigated the effects surgical technique and timing have on maxillary growth, few focus on patients with isolated cleft palate (ICP). The purpose of this study was to determine the impact palate repair and its associated complications may have on maxillary growth. Methods: A retrospective chart review of ICP patients who underwent palatoplasty from 1962 to 1999 at Akron Children's Hospital was performed. Patient demographics, Veau type, age at primary repair, closure technique, presence of fistula or velopharyngeal insufficiency (VPI), number of palatal operations, maxillary hypoplasia (MH) frequency, and follow-up were recorded. Exclusion criteria included patients with cleft lip, submucous cleft, or syndromes. Results: Twenty-nine non-syndromic ICP patients were identified; 62% (n = 18) had Veau type 1 and 38% (n = 11) had Veau type 2. All patients underwent 2-flap or Furlow palatoplasty with mobilization of mucoperiosteal flaps. Vomerine flaps were used in all Veau 2 cleft palate closures. Palatoplasty was performed at a mean age of 19.9 ± 8.2 months. Average follow-up was 209 ± 66.5 months. The rate of VPI was 59% (n = 17) and the rate of oronasal fistula was 14% (n = 4). Conclusions: There was a low incidence of MH despite complications after initial palate closure. Our results seem to suggest that age at palate closure, type of cleft palate, and type of surgical technique may not be associated with MH. Additionally, subsequent procedures and complications after primary palatoplasty such as VPI and palatal fistula may not restrict maxillary growth.
Purpose: This study examined the factors that may affect the growth status of preterm infants. Methods: This study included 91 preterm infants born at <37 weeks of gestation (22.9-36.9 weeks of gestation), including 48 (52.7%) males and 43 (47.3%) females. Diet-related data were collected through parental questionnaires, and growth-related data, such as height and weight, were collected through the hospital medical records. Results: No significant difference in weight and growth was observed between early and late preterm infants. On the other hand, smaller averages of all weight z-score (recent weight at 40 weeks of gestation) included lower birth weight, height, and head circumference. On the other hand, infants' birth weight, height, and head circumference in the weight z-score of <0 (<50% in the age-weight growth chart) was smaller than those in the weight z-score of ≥0. Furthermore, neonatal intensive care unit (NICU) hospitalization period and NICU discharge were shorter with growth cessation age in weight z-score of <0. The weight growth velocity was associated with gestational age, birth weight, and medical treatment in the NICU. Thus, parents of preterm infants with low growth rates prefer more community care services for their children. Conclusion: Birth weight, age of preterm infants, and medical treatment in the NICU were factors related to early birth weight growth. Following NICU discharge, poor intake and intake issues were associated with poor growth after 40 weeks of gestation. Therefore, monitoring the growth of preterm infants requires continuous active involvement and supports for growth-promoting factors after NICU discharge.
Purpose: To investigate the health status such as growth, nutrition, disease, vaccination in child day care centers. Methods: A cross-sectional descriptive study was conducted on 280 children at 6 day care centers in Seoul. Parent completed a structured questionnaire and illness-logs from day-care staff were gathered for a month, June 2006. Body weight, height were measured and BMI was calculated. Results: Failure of growth was found in 2.2% of the children. Also there was 5.4% of the children who were under 5% and 8.6% were above 95% on BMI chart. For 4.3% of the children diseases were asthma, chronic rhinitis, sinusitis and so on. About six percent of the children missed vaccination such as Japanese encephalitis, DTaP and polio. Incidence rates of respiratory infection was 1.45 and gastroenteritis was .28. As a result, 22.5 % of the day care children had these kinds of health rick factors stated above and results showed statistical difference between health-groups and health-risk groups in age, duration of day care center, family structure, marital status of parent. Conclusion: A considerable number of the children at child daycare centers had the health risk factors. These results shows there is a need to develop and make provisions for health service programs by health experts in child day care centers.
Purpose: Congenital hypothyroidism (CH) is the most common endocrine disorder in children. Thyroid hormone deprivation results not only in mental retardation but also growth retardation. This study investigates the final height (FH) in Korean patients with CH detected by newborn screening and examines factors that may affect the FH. Methods: The medical records of Korean CH patients (n=45) were reviewed. The FH was examined and target height (TH) was calculated based on mid-parental height. The FH z score (FHZ) and TH z score (THZ) were computed using the 2007 Korean National Growth Chart. The FHZ and THZ were compared with a Student t test. The impact of the etiology of CH (athyreosis, dyshormonogenesis, ectopic thyoid, hypoplastic thyroid), initial serum thyroid stimulating hormone (TSH) level, initial free thyroxine (T4) level, and time of therapy initiation based on FH was assessed. Results: The mean FHZ was $0.10{\pm}1.01$ for male patients and $-0.11{\pm}1.09$ for female patients. There were no significant differences between FHZ and THZ for both female (P=0.356) and male patients (P=0.237). No significant relationship was found between FH and the etiology of CH, initial TSH level, initial free T4 level, and the time of therapy initiation. Conclusion: Early intervention and satisfactory management do not appear to impede growth in Korean patients with CH. Thus, early detection and proper management of patients with CH detected by newborn screening program are necessary.
In order to elucidate growth characteristics, physiological responses and leaf color changes of leaf-variegated Arundinaria munsuensis and Carex ciliato-marginata for variegata, These experiments were performed under four different light ragimes control(full sun), 40%, 70% and 85%. Plant height and leaf area became promoted as shading level increases in leaf-variegated Arundinaria munsuensis. Photosynthetic effect was the highest in 85% shading of the full sun. Thus, this plant could be growing in the deep shade condition. Plant growth and the leaf color changes were most obviously shown in the 40% shading level. In the Carex ciliatomarginata for. variegata growth status was the best and green or strong greenish yellow leaf color turned out to be much clearer in the 40% shading treatment. And photosynthetic activity was enhanced as the light intensity decreases.
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