• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.190-197
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• 2007

Purpose : Reduced growth and microvascular complications have been recognized as consequences of type 1 diabetes mellitus (T1DM). We assessed the effect of T1DM on growth and factors associated with the development of microvascular complications. Methods : We conducted a retrospective longitudinal evaluation of 154 patients above 16 years of age. We analyzed factors which affect final height standard deviation scores (SDS) and development of microvascular complications. Results : Final height SDS was $-0.11{\pm}1.15$ ($-0.26{\pm}1.33$ in females, $0.04{\pm}0.91$ in males). Final height SDS was significantly lower than midparental height SDS and height SDS at diagnosis. There was no difference in final height SDS according to age at onset, existence or nonexistence of complications, or average $HbA_{1C}$. Height SDS at onset of puberty, midparental height SDS and pubertal growth gain affected final height SDS. The number of patients with complications was 37 (24 percent). Microvascular complications developed at a younger age and after longer duration of diabetes in patients with a prepubertal onset of T1DM compared to patients with pubertal onset. Patients with complications had a higher level of average $HbA_{1C}$ than patients without complications. Patients whose microalbuminuria regressed had lower levels of average $HbA_{1C}$, systolic BP, second 24h urine microalbumin than patients with persistant or progressed microalbuminuria. Conclusion : The results suggest that degrees of glycemic control don't affect final height, but various factors associated with T1DM can impair growth potential. Additionally, the degrees of glycemic control and puberty affect the development of microvascular complications.

• Annals of Pediatric Endocrinology and Metabolism
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• v.23 no.4
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• pp.176-181
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• 2018

Noonan syndrome (NS) is an autosomal dominant disorder that involves multiple organ systems, with short stature as the most common presentation (>70%). Possible mechanisms of short stature in NS include growth hormone (GH) deficiency, neurosecretory dysfunction, and GH resistance. Accordingly, GH therapy has been carried out for NS patients over the last three decades, and multiple studies have reported acceleration of growth velocity (GV) and increase of height standard deviation score (SDS) in both prepubertal and pubertal NS patients upon GH therapy. One year of GH therapy resulted in almost doubling of GV compared with baseline; afterwards, the increase in GV gradually decreased in the following years, showing that the effect of GH therapy wanes over time. After four years of GH therapy, ~70% of NS patients reached normal height considering their age and sex. Early initiation, long duration of GH therapy, and higher height SDS at the onset of puberty were associated with improved final height, whereas gender, dosage of GH, and the clinical severity did not show significant association with final height. Studies have reported no significant adverse events of GH therapy regarding progression of hypertrophic cardiomyopathy, alteration of metabolism, and tumor development. Therefore, GH therapy is effective for improving height and GV of NS patients; nevertheless, concerns on possible malignancy remains, which necessitates continuous monitoring of NS patients receiving GH therapy.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.191-196
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• 2005

Purpose : Short stature is one of the characteristic features of Turner syndrome. We investigated the factors affecting final adult height(FAH) in patients with Turner syndrome. Methods : The study group was comprised of 60 patients who were diagnosed with Turner syndrome by chromosomal study and clinical phenotypes and attained FAH. Data were obtained from retrospective review of the medical records. We analyzed the factors influencing FAH in growth hormone(GH) treated and GH untreated groups. Results : Sixty patients were enrolled; 48 patients received GH treatment, and 12 patients did not. Mean duration of GH treatment was 35.8 months(range 4 to 120 months), and mean dosage of GH was $0.8{\pm}0.2IU/kg/wk$ in GH treated group. Mean growth velocity was $5.6{\pm}2.0cm/yr$, which was significantly higher than that during pretreatment period. In the GH treated group, mean chronological age, bone age, mean height, and height standard deviation(SD) score at GH treatment were $12.2{\pm}2.7yr$ $10.3{\pm}2.5yr$ $127.5{\pm}10.1cm$ and $-3.1{\pm}1.1$, respectively. In the GH treated group, the mean FAH and SD score of FAH were $146.9{\pm}5.8cm$ and $-2.7{\pm}1.2$, respectively, which showed significant differences compared with those of the GH untreated group. Analyzing the factors affecting FAH in GH-treated patients, only the SD score of height at the time of treatment was significantly related to FAH. Conclusion : GH treatment leads to an increment in FAH in patients with Turner syndrome. Average FAH gain was as much as 5.8 cm. SD score of height at the time of GH treatment was the only factor influencing FAH.

• Clinical and Experimental Pediatrics
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• v.46 no.8
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• pp.803-810
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• 2003

Purpose : The purpose of this study was to evaluate the factors affecting the final adult height and total height gain in idiopathic and organic growth hormone deficient(GHD) children after growth hormone(GH) treatment. Methods : Thirteen patients with idiopathic GHD and 22 patients with organic GHD who had been treated with GH and attained adult final height were included in this study. Factors which could affect the final adult height(FAH) and total height gain, were evaluated. Results : Height SDS(standard deviation score) at initial GH treatment in idiopathic GHD was significantly shorter than that in organic GHD($-4.13{\pm}1.28$ vs $-1.66{\pm}1.06$, P<0.001). Growth velocity during the first year of GH treatment was $9.69{\pm}3.19cm$(idiopathic GHD) and $7.87{\pm}3.65cm$(organic GHD). Height(SDS) at puberty in organic GHD was significantly greater than in idiopathic GHD ($-0.55{\pm}1.25$ vs $-2.28{\pm}0.95$, P<0.001). Final adult height(SDS) was significantly greater in organic GHD than in idiopathic GHD($0.22{\pm}1.06$ vs $-1.44{\pm}0.84$, P<0.001). In idiopathic GHD, total height gain (SDS) was most significantly correlated with midparental height minus initial height(MPH-IH)(SDS) (r=0.886, P<0.001). Total height gain(SDS) was more significantly correlated with MPH-IH(SDS) and prepubertal height gain(SDS) in idiopathic GHD(r=0.640, P=0.01, r=0.801, P<0.001). Conclusion : Final adult height was greater in organic GHD than in idiopathic GHD patients. While total height gain(SDS) was more pronounced in children with lower initial height compared to MPH, absolute final adult height was influenced by height at puberty. To improve the final adult height in children with GHD, height at onset of puberty must be increased by early diagnosis and continuous treatment with optimal doses of GH. There results should be evaluated with more patients.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.219-227
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• 2006

Purpose : We aim to identify the clinical and demographic characteristics in children who underwent renal transplantation(RTx) and to evaluate the influence on growth of RTx in children. Methods : We reviewed 17 medical records of chronic renal failure patients who underwent RTx from April 1992 and June 2004 at Busan Paik Hospital. Age and sex distribution, cause of disease, donor analysis, patient and graft survival rate, and the status of growth after RTx were analysed by retrospective study. Results : Eighteen RTx were performed in 17 patients(8 boys, 9 girls). The mean age at the time of RTx was $15.8{\pm}3.5$ years and the mean duration of dialysis therapy before RTx was $22.4{\pm}18.0$ months. The 1 year and 5 year patient survival rate were each 100%, and the 1 year and 5 year graft survival rate were 88%, 36% respectively. The most common cause of graft failure was chronic rejection. The mean final height of male patients was $162.8{\pm}10.0$ cm(143.0-172.5 cm) and of female patients was $154.5{\pm}12.1$ cm(135.8-160.0 cm). The mean height standard deviation score(Ht SDS) increased after RTx from -1.95 to -1.53 but the increment rate was not statistically significant. Similar changes were noted in individual patient analysis. Also there was no significant difference between the living-related donors and cadaveric donors. Conclusion : Our data shows that even successful RTx rarely results in full growth rehabilitation. To overcome retarded growth in children with chronic renal failure, appropriate combined management of metabolic and nutritional problems, correction of anemia, proper use of recombinant growth hormone therapy, early renal transplantation and shortening of the duration of dialysis would be necessary.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.65-73
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• 2007

Purpose : Short stature is an important complication that impairs the quality of life in survivors of childhood brain tumors. We studied their final adult height (FAH) to evaluate risk factors for short stature. Methods : We reviewed the medical data of 95 survivors of childhood brain tumors (64 males and 31 females) who had been followed up from 1982 to 2006, reached FAH, and had a more than five year-disease-free survival. Results : Final adult height standard deviation score (FAHTSDS: $mean{\pm}SD$) of the patients was lower than those of general population ($-1.15{\pm}1.72$), HTSDS at diagnosis ($-0.13{\pm}1.57$), and target HTSDS ($-0.49{\pm}0.69$). FAHTSDS of craniopharyngioma patients did not decrease ($0.57{\pm}1.17$), but those of germ cell tumor and medulloblastoma patients were significantly reduced ($-1.20{\pm}1.45$, $-2.70{\pm}1.46$; P<0.05). The patients treated with craniospinal radiation or chemotherapy had lower FAHTSDS ($-1.93{\pm}1.58$, $-2.27{\pm}1.44$; P<0.01). In the spinal irradiation group, the younger the age at diagnosis was, the more the loss of FAH (r=0.442, P<0.01). Growth hormone replacement (GHR) didn't improve FAHTSDS, but starting GHR under 12 years was an independent factor for improving FAH once treatment methods were taken into account (P=0.01). Conclusion : The younger age at diagnosis, spinal radiation and chemotherapy were all important risk factors of height loss, and height gain was expected in patients who received GHR under the age of 12 years. Therefore, regular check-ups of growth and early intervention with growth hormones are needed for high risk groups to improve FAH.

• Clinical and Experimental Pediatrics
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• v.58 no.1
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• pp.1-7
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• 2015

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.