Journal of Pediatric Endocrinology & Metabolism最新文献

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Long-term efficacy and safety of PEGylated recombinant human growth hormone in treating Chinese children with growth hormone deficiency: a 5-year retrospective study. 聚乙二醇化重组人生长激素治疗中国生长激素缺乏症儿童的长期疗效和安全性:一项为期 5 年的回顾性研究。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-08-23 Print Date: 2024-10-28 DOI: 10.1515/jpem-2024-0189
Lele Hou, Shaofen Lin, Zulin Liu, Lina Zhang, Hui Ou, Siqi Huang, Huilian Dai, Zhe Meng, Liyang Liang
{"title":"Long-term efficacy and safety of PEGylated recombinant human growth hormone in treating Chinese children with growth hormone deficiency: a 5-year retrospective study.","authors":"Lele Hou, Shaofen Lin, Zulin Liu, Lina Zhang, Hui Ou, Siqi Huang, Huilian Dai, Zhe Meng, Liyang Liang","doi":"10.1515/jpem-2024-0189","DOIUrl":"10.1515/jpem-2024-0189","url":null,"abstract":"<p><strong>Objectives: </strong>The study endeavored to evaluate the prolonged efficacy and safety of PEGylated rhGH (PEG-rhGH) administration in Chinese children diagnosed with growth hormone deficiency (GHD) over a 5-year period.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on children with GHD, who received a 0.2 mg/kg/week dose of PEG-rhGH between 2016 and 2023 in our department.</p><p><strong>Results: </strong>The height standard deviation score (Ht SDS) exhibited a marked elevation post-PEG-rhGH administration (p<0.001), sustaining this enhancement beyond year 3, with increments recorded at 0.94±0.37, 1.49±0.48, 1.77±0.51, 2.12±0.65, and 2.15±0.58 across 5 years. Similarly, the height velocity (HV), insulin-like growth factor-1 standard deviation score (IGF-1 SDS), and bone age to chronological age ratio (BA/CA ratio) underwent significant augmentations (p<0.01). Remarkably, no signs of rapid bone maturation were detected during the 5-year observation. Among the participants, 31 patients (59.62 %) experienced adverse events, of which eight instances (15.38 %) were classified as treatment-related adverse events, but none were severe or unexpected. Additionally, high-density lipoprotein (HDL) levels rose while low-density lipoprotein (LDL) levels fell, both remaining within the standard range throughout the treatment phase.</p><p><strong>Conclusions: </strong>Administering PEG-rhGH at a dosage of 0.2 mg/kg/week proved both effective and well-tolerated in treating prepubertal children with GHD. This regimen also demonstrated positive impacts on lipid metabolism over an extended treatment period.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"892-899"},"PeriodicalIF":1.3,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142074410","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Urinary biomarkers NG AL and beta-2 microglobulin in children with type 1 diabetes mellitus. 1 型糖尿病患儿尿液生物标记物 NG AL 和 beta-2 微球蛋白。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-08-20 Print Date: 2024-09-25 DOI: 10.1515/jpem-2024-0172
Nimisha Sachan, Aashima Dabas, Mukta Mantan, Pradeep K Dabla
{"title":"Urinary biomarkers NG AL and beta-2 microglobulin in children with type 1 diabetes mellitus.","authors":"Nimisha Sachan, Aashima Dabas, Mukta Mantan, Pradeep K Dabla","doi":"10.1515/jpem-2024-0172","DOIUrl":"10.1515/jpem-2024-0172","url":null,"abstract":"<p><strong>Objectives: </strong>To study the urinary neutrophil gelatinase-associated lipocalin (NGAL) and beta-2-microglobulin (β2M) levels as markers of tubular damage in children with type 1 diabetes (T1DM).</p><p><strong>Methods: </strong>Forty T1DM children and 40 age-matched controls were enrolled. Subjects with coexisting kidney disorder, intake of oral glucose lowering drugs and syndromic diabetes mellitus were excluded. Fasting plasma glucose, glycated hemoglobin (HbA<sub>1c</sub>), kidney function, urinary albumin-creatinine ratio (UACR), NGAL and β2M were measured and compared in cases and controls.</p><p><strong>Results: </strong>The median (IQR) age of cases and controls was 10.6 (8, 14.2) and 10.7 (8.4, 13.7) years, respectively. Cases had disease duration of 4 (3, 6.8) years and HbA<sub>1c</sub> 10.9 (9, 13.1) %. Microalbuminuria was seen in 14 (35 %). Median (IQR) levels of UACR were higher in cases than controls [19.38 (10.27, 35.26) and 6.49 (3.10, 11.65) µg/mg; p<0.001], similarly NGAL/creatinine [352.21 (191.49, 572.45) and 190.54 (125.91, 322.83) ng/mg; p=0.006], unlike β2M/creatinine [1.7 (0.43, 6.02) and 2.12 (1.05, 4.47) µg/mg; p=0.637]. Children with higher HbA<sub>1c</sub> (≥10 %) had higher urinary ACR and tubular biomarkers than HbA<sub>1c</sub><10 % (p>0.05). Urinary ACR showed positive correlation with NGAL/creatinine (r=0.38, p=0.019) and β2M/creatinine (r=0.42, p=0.009).</p><p><strong>Conclusions: </strong>Urinary biomarkers NGAL and β2M were elevated in the presence of normal urinary microalbumin levels suggestive of early tubular damage in T1DM.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"764-772"},"PeriodicalIF":1.3,"publicationDate":"2024-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142019387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Applicability of the External Genitalia Score (EGS) in Indian neonates and children up to 2 years of age. 外生殖器评分(EGS)在印度新生儿和 2 岁以下儿童中的适用性。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-08-06 Print Date: 2024-09-25 DOI: 10.1515/jpem-2024-0130
Pamali Mahasweta Nanda, Jaivinder Yadav, Devi Dayal, Rakesh Kumar, Praveen Kumar, Jogender Kumar, Harvinder Kaur, Pooja Sikka
{"title":"Applicability of the External Genitalia Score (EGS) in Indian neonates and children up to 2 years of age.","authors":"Pamali Mahasweta Nanda, Jaivinder Yadav, Devi Dayal, Rakesh Kumar, Praveen Kumar, Jogender Kumar, Harvinder Kaur, Pooja Sikka","doi":"10.1515/jpem-2024-0130","DOIUrl":"10.1515/jpem-2024-0130","url":null,"abstract":"<p><strong>Objectives: </strong>To generate normative data and validate the recently developed, gender-neutral, External Genitalia Score (EGS) in Indian preterm and term neonates and children up to 2 years of age with normal and atypical genitalia.</p><p><strong>Methods: </strong>This observational study included 1,040 neonates born between 28 and 42 weeks of gestation and 152 children between 1 and 24 months of age. In addition, 50 children with disorders of sex development (DSD) were also enrolled in the study. The Prader stage/external masculinization score (EMS) (as applicable), anogenital ratio (AGR) and EGS were assessed for all neonates and children with typical and atypical genitalia.</p><p><strong>Results: </strong>Median EGS values in newborn males with typical genitalia were 9.5 at 28-31 weeks, 10.5 at 32-33 weeks, 11 at 34 weeks and 11.5 in males at 35-42 weeks of gestation. For all females with typical genitalia, the EGS was 0. EMS and EGS showed a positive correlation in males with typical genitalia (r=0.421, p=0.000**) and all children with DSD (r=0.857, p=0.000**). Mean AGR in males and females with typical genitalia and those with DSD were 0.52±0.07, 0.31±0.05 and 0.47±0.13, respectively. EGS correlated with AGR in all males with typical genitalia (r=0.107, p=0.008**), and in all children with DSD (r=0.473, p=0.001**).</p><p><strong>Conclusions: </strong>The EGS enables accurate, gender-neutral and comprehensive assessment of external genitalia in Indian neonates and children with typical and atypical genitalia/DSD. Evaluation for DSD is recommended in any child with EGS greater than 0 and ≤10th percentile for gestation or age (10.5 in a term neonate).</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"811-819"},"PeriodicalIF":1.3,"publicationDate":"2024-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141890743","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A novel variant of the STAR gene: nonclassical presentation from Turkey. STAR基因的一种新型变体:来自土耳其的非典型表现。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-07-24 Print Date: 2024-09-25 DOI: 10.1515/jpem-2024-0156
Emel Hatun Aytaç Kaplan, Alper Gezdirici, Zümrüt Kocabey Sütçü, Ezgi Gökpinar İli
{"title":"A novel variant of the <i>STAR</i> gene: nonclassical presentation from Turkey.","authors":"Emel Hatun Aytaç Kaplan, Alper Gezdirici, Zümrüt Kocabey Sütçü, Ezgi Gökpinar İli","doi":"10.1515/jpem-2024-0156","DOIUrl":"10.1515/jpem-2024-0156","url":null,"abstract":"<p><strong>Objectives: </strong>Lipoid congenital adrenal hyperplasia (LCAH) is a rare autosomal recessive disease caused by mutations in the steroidogenic acute regulatory protein (<i>STAR</i>) gene, expressed in the adrenal and gonadal tissues. In classical LCAH, individuals with 46, XY chromosomes present with a female appearance of the external genitalia due to insufficient androgen production. In the non-classical form, a milder phenotype is observed with male external genitalia. Here, we present a non-classical LCAH diagnosis with a newly identified c.266T>A (p.Ile89Asn) likely pathogenic homozygous variant in a 46, XY infant.</p><p><strong>Case presentation: </strong>A three-month-and-thirteen-day-old male proband presented with clinical features of cortisol and mineralocorticoid deficiencies. The manifestation of salt-wasting syndrome occurred relatively late, and although the external genitalia appeared male, there was a mild virilization defect. The combination of mild impairment in androgen production and severe salt-wasting syndrome is an intriguing finding in our patient. Peripheral blood samples were obtained from the patient and his family. The newly identified variant, determined by next-generation sequencing analysis, was confirmed by segregation analysis showing carrier status in both parents.</p><p><strong>Conclusions: </strong>We aim to contribute to the literature by elucidating molecular mechanisms by presenting an atypical presentation and a newly identified variant.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"835-839"},"PeriodicalIF":1.3,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141753252","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Children and adolescents with differentiated thyroid cancer from 1998 to 2018: a retrospective analysis. 1998年至2018年分化型甲状腺癌的儿童和青少年:回顾性分析。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-07-22 Print Date: 2024-09-25 DOI: 10.1515/jpem-2024-0191
Wei Li, Shanling Zhang, Zilu Gao, Yingjie Tao, Xudong Wang, Junping Cheng
{"title":"Children and adolescents with differentiated thyroid cancer from 1998 to 2018: a retrospective analysis.","authors":"Wei Li, Shanling Zhang, Zilu Gao, Yingjie Tao, Xudong Wang, Junping Cheng","doi":"10.1515/jpem-2024-0191","DOIUrl":"10.1515/jpem-2024-0191","url":null,"abstract":"<p><strong>Objectives: </strong>This study aims to investigate the clinical features of differentiated thyroid carcinoma (DTC) in children and adolescents under 18 years and assess the impact of surgery combined with thyroid hormone and radioactive iodine (RAI) on their prognosis.</p><p><strong>Methods: </strong>A retrospective observational study was conducted, involving children/adolescents with DTC who underwent surgery at the Head and Neck Department of Tianjin Medical University Cancer Institute and Hospital from January 1998 to December 2018.</p><p><strong>Results: </strong>Among 198 patients, 130 (65.7 %) were female. According to the American Thyroid Association guidelines, cases were categorized as low (106, 53.5 %), intermediate (54, 27.3 %), and high (38, 19.2 %) risk. The follow-up duration ranged from 3 to 23 years. Local recurrence and distant metastasis were identified in 21 (10.6 %) and 14 (7.1 %) cases, respectively. All patients received levothyroxine, while RAI therapy was administered to intermediate- and high-risk patients. The local recurrence and distant metastasis rates in these two groups were 33.3 and 39.5 %, respectively, with no recurrence or metastasis in the low-risk group. Persistent without structural evidence of disease were 0.9, 3.7, and 26.3 % at end of follow-up for the low-, intermediate-, and high-risk groups, respectively. The overall survival rates for all three groups were 100 %, while disease-free survival rates were 99.1, 63.0, and 34.2 % for the low-, intermediate-, and high-risk groups, respectively.</p><p><strong>Conclusions: </strong>Children/adolescents with low-risk DTC exhibited a favorable prognosis even without RAI. However, intermediate- and high-risk DTC patients, despite RAI and levothyroxine treatment, showed elevated rates of persistent disease, local recurrence, and distant metastasis.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"796-803"},"PeriodicalIF":1.3,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141724932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
STX16 exon 5-7 deletion in a patient with pseudohypoparathyroidism type 1B. 一名假性甲状旁腺功能亢进症 1B 型患者的 STX16 第 5-7 号外显子缺失。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-07-22 Print Date: 2024-08-27 DOI: 10.1515/jpem-2023-0562
Li Chen, Chuanbin Yang, Xiaoxiao Zhang, Beibei Chen, Peibing Zheng, Tingting Li, Wenjing Song, Hua Gao, Xiaofang Yue, Jiajun Yang
{"title":"<i>STX16</i> exon 5-7 deletion in a patient with pseudohypoparathyroidism type 1B.","authors":"Li Chen, Chuanbin Yang, Xiaoxiao Zhang, Beibei Chen, Peibing Zheng, Tingting Li, Wenjing Song, Hua Gao, Xiaofang Yue, Jiajun Yang","doi":"10.1515/jpem-2023-0562","DOIUrl":"10.1515/jpem-2023-0562","url":null,"abstract":"<p><strong>Objectives: </strong>Pseudohypoparathyroidism (PHP) comprises a cluster of heterogeneous diseases characterized by hypocalcemia and hyperphosphatemia due to parathyroid hormone (PTH) resistance. PHP type 1B (PHP1B) is caused by heterozygous maternal deletions within GNAS or STX16. STX16 exon 2-6 deletion is commonly observed in autosomal dominant (AD)-PHP1B, while sporadic PHP1B commonly results from methylation abnormalities of maternal differentially methylated regions and remains unclear at the molecular level.</p><p><strong>Case presentation: </strong>A 39-year-old male patient with PHP1B, who had his first seizure at 15 years of age, presented to our hospital. The methylation-specific multiplex ligation-dependent probe amplification results showed a half-reduced copy number of STX16 exon 5-7 and loss of methylation at GNAS exon A/B. His mother also had a half-reduced copy number of STX16 exon 5-7 but with normal methylation of GNAS. His father has a normal copy number of STX16 and normal methylation of GNAS.</p><p><strong>Conclusions: </strong>For the recognition and early diagnosis of this kind of disease, here we report the clinical symptoms, auxiliary examinations, genetic testing characteristics, and treatment of the patient.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"734-740"},"PeriodicalIF":1.3,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141724872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Novel homozygous mutation in SCNN1A gene in an Iranian boy with PHA1B. 一名患有 PHA1B 的伊朗男孩的 SCNN1A 基因发生了新的同源突变。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-07-05 Print Date: 2024-08-27 DOI: 10.1515/jpem-2023-0505
Fatemeh Saffari, Ensiyeh Bahadoran, Ali Homaei, Sahar Moghbelinejad
{"title":"Novel homozygous mutation in SCNN1A gene in an Iranian boy with PHA1B.","authors":"Fatemeh Saffari, Ensiyeh Bahadoran, Ali Homaei, Sahar Moghbelinejad","doi":"10.1515/jpem-2023-0505","DOIUrl":"10.1515/jpem-2023-0505","url":null,"abstract":"<p><strong>Objectives: </strong>Pseudohypoaldosteronism type 1 (PHA1) has two genetically distinct variants, including renal and systemic forms. Systemic PHA type I (PHA1B) has varying degrees of clinical presentation and results from mutations in genes encoding subunits of the epithelial sodium channel (ENaC) including the alpha, beta, and gamma subunits. To date, about 45 variants of PHA1B have been identified.</p><p><strong>Case presentation: </strong>We report a boy with PHA1B, who presented with vomiting, lethargy, and poor feeding due to salt wasting six days after birth. The patient had electrolyte imbalances. A novel SCNN1A (sodium channel epithelial subunit alpha) gene mutation, NM_001038.6:c.1497G>C, with an autosomal recessive pattern, was identified by whole exosome sequencing. This variant was inherited as a homozygote from both heterozygous parents.</p><p><strong>Conclusions: </strong>PHA should be considered in neonates with hyponatremia and hyperkalemia. This case report presents a patient with a novel mutation in SCNN1A that has not been previously reported. Long-term follow-up of identified patients to understand the underlying phenotype--genotype link is necessary.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"745-749"},"PeriodicalIF":1.3,"publicationDate":"2024-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Dihydropyrimidinase deficiency with atrioventricular septal defect: a case report. 伴有房室间隔缺损的二氢嘧啶酶缺乏症:病例报告。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-07-04 Print Date: 2024-08-27 DOI: 10.1515/jpem-2023-0518
İzzet Erdal, Yılmaz Yıldız, Oya Kuseyri Hübschmann, Dorothea Haas, Ceren Günbey, İlker Ertuğrul, Dilek Yalnızoğlu
{"title":"Dihydropyrimidinase deficiency with atrioventricular septal defect: a case report.","authors":"İzzet Erdal, Yılmaz Yıldız, Oya Kuseyri Hübschmann, Dorothea Haas, Ceren Günbey, İlker Ertuğrul, Dilek Yalnızoğlu","doi":"10.1515/jpem-2023-0518","DOIUrl":"10.1515/jpem-2023-0518","url":null,"abstract":"<p><strong>Objectives: </strong>Dihydropyrimidinase deficiency is a rare autosomal recessive disorder of the pyrimidine degradation pathway, with fewer than 40 patients published. Clinical findings are variable and some patients may remain asymptomatic. Global developmental delay and increased susceptibility to 5-fluorouracil are commonly reported. Here we present atrioventricular septal defect as a novel feature in dihydropyrimidinase deficiency.</p><p><strong>Case presentation: </strong>A four-year-old male with global developmental delay, dysmorphic facies, autistic features and a history of seizures was diagnosed with dihydropyrimidinase deficiency based on strikingly elevated urinary dihydrouracil and dihydrothymine and a homozygous pathogenic nonsense variant in DPYS gene. He had a history of complete atrioventricular septal defect corrected surgically in infancy.</p><p><strong>Conclusions: </strong>This is the second report of congenital heart disease in dihydropyrimidinase deficiency, following a single patient with a ventricular septal defect. The rarity of the disease and the variability of the reported findings make it difficult to describe a disease-specific clinical phenotype. The mechanism of neurological and other systemic findings is unclear. Dihydropyrimidinase deficiency should be considered in patients with microcephaly, developmental delay, epilepsy and autistic traits. We suggest that congenital heart disease may also be a rare phenotypic feature.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"741-744"},"PeriodicalIF":1.3,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Robust growth hormone responses to GH-releasing peptide 2 in adolescents. 青少年对促生长激素释放肽 2 的强大生长激素反应。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-07-04 Print Date: 2024-08-27 DOI: 10.1515/jpem-2024-0115
Takanori Onuki, Tadokoro Hiroaki, Kentaro Sawano, Nao Shibata, Hiromi Nyuzuki, Yohei Ogawa, Masayasu Okada, Hirohito Sone, Keisuke Nagasaki
{"title":"Robust growth hormone responses to GH-releasing peptide 2 in adolescents.","authors":"Takanori Onuki, Tadokoro Hiroaki, Kentaro Sawano, Nao Shibata, Hiromi Nyuzuki, Yohei Ogawa, Masayasu Okada, Hirohito Sone, Keisuke Nagasaki","doi":"10.1515/jpem-2024-0115","DOIUrl":"10.1515/jpem-2024-0115","url":null,"abstract":"<p><strong>Objectives: </strong>GH-releasing peptide-2 (GHRP2) can be used for provocative growth hormone testing (GHT). Since it acts as a powerful stimulus for GH secretion, cut-off peak GH level in GHRP2 loading test (GHRP2T) is higher than in other GHT. Nevertheless, data on response at adolescents are limited. This report aimed to investigate peak GH levels in GHRP2T in adolescents.</p><p><strong>Methods: </strong>Clinical data of adolescents after onset of puberty who underwent GHRP2T at our institution from May 2010 to March 2023 were collected retrospectively. Subjects were classified into three groups according to underlying diseases.</p><p><strong>Results: </strong>A total of 23 patients were included: 12 in organic or genetic GHD (o/gGHD) group, three in idiopathic GHD (iGHD) group, and eight in short stature (SS) group. The median GH peak levels were 3.4 ng/mL in o/gGHD group, 88.9 ng/mL in iGHD group, and 90.1 ng/mL in SS group, indicating a robust response of GH peak levels in iGHD and SS groups. Two patients exceeded the cut-off for GHRP2T but below for other GHT, indicating the current cut-off for GHRP2T may miss some GHD patients.</p><p><strong>Conclusions: </strong>The GH response to GHRP2T in adolescents except the o/gGHD group may be robustly responsive. For the correct diagnosis of GHD, the cut-off peak GH levels in GHRP2T in adolescents may require revisiting.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"730-733"},"PeriodicalIF":1.3,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494092","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Glucose and lipid-related indicators in relation to elevated alanine aminotransferase in a pediatric population. 儿科人群中与丙氨酸氨基转移酶升高有关的血糖和血脂相关指标。
IF 1.3 4区 医学
Journal of Pediatric Endocrinology & Metabolism Pub Date : 2024-06-28 Print Date: 2024-07-26 DOI: 10.1515/jpem-2024-0092
Azin Momeni, Maryam Yazdi, Mohammad Esmail Motlagh, Mostafa Qorbani, Roya Kelishadi
{"title":"Glucose and lipid-related indicators in relation to elevated alanine aminotransferase in a pediatric population.","authors":"Azin Momeni, Maryam Yazdi, Mohammad Esmail Motlagh, Mostafa Qorbani, Roya Kelishadi","doi":"10.1515/jpem-2024-0092","DOIUrl":"10.1515/jpem-2024-0092","url":null,"abstract":"<p><strong>Objectives: </strong>To determine the associations between various glucose and lipid-related indicators with elevated alanine aminotransferase (ALT) in pediatric population.</p><p><strong>Methods: </strong>We analyzed the cross-sectional data of 3,771 Iranian children and adolescents aged 7-18 years using the fifth survey of a national school-based surveillance program. The predictive power of 11 different glucose and lipid-related indicators for predicting elevated ALT was examined using receiver operating characteristic (ROC) curve.</p><p><strong>Results: </strong>In the total sample non-HDL-C, non-HDL-C/HDL-C, and TC/HDL-C showed the largest area under the curve (AUC) for elevated ALT detection, with 0.731 (cut-off, 129.5 mg/dL), 0.706, and 0.706, respectively. In girls, non-HDL-C had the highest predictive value (AUC, 0.741, cut-off, 129.5 mg/dL). Among boys, non-HDL-C/HDL-C and TC/HDL-C showed the largest AUC of 0.753 with optimum cut-off values of 2.63 and 3.63, respectively.</p><p><strong>Conclusions: </strong>The findings of this study suggest that non-HDL-C, non-HDL-C/HDL-C, and TC/HDL-C can be predictors of elevated ALT in the pediatric population. These indices can be useful in large population-based studies for predicting children and adolescents at risk of fatty liver.</p>","PeriodicalId":50096,"journal":{"name":"Journal of Pediatric Endocrinology & Metabolism","volume":" ","pages":"597-604"},"PeriodicalIF":1.3,"publicationDate":"2024-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141460456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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