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Journal of Pediatric Endocrinology & Metabolism: JPEM

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https://www.readbyqxmd.com/read/29750655/metabolic-risk-factors-in-adolescent-girls-with-type-1-diabetes
#1
Cíntia Castro-Correia, Rita Santos-Silva, Marta Pinheiro, Carla Costa, Manuel Fontoura
BACKGROUND: The incidence of pediatric metabolic syndrome (MS) has progressively increased. The incidence of type 1 diabetes mellitus (T1DM) has also increased. Thus, some children and adolescents with T1DM exhibit MS parameters. The aim of the study was to evaluate the presence of MS parameters in female adolescents with T1DM based on their nutritional status. METHODS: We evaluated 44 adolescents with T1DM (consecutive non-randomized sample) aged between 14 and 18 years, who were on intensive therapy with insulin...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750654/initial-presentations-and-associated-clinical-findings-in-patients-with-classical-congenital-adrenal-hyperplasia
#2
Asmaa Adel Milyani, Abdulmoein Eid Al-Agha, Mashael Al-Zanbagi
BACKGROUND: The objective of this study was to investigate the initial presenting features of children with classical congenital adrenal hyperplasia (CAH). METHODS: This is a descriptive retrospective study over a period of 5 years. Data analysis was performed using Statistical Package for Social Science. RESULTS: Ambiguous genitalia (AG) was the commonest initial presentation marking 50.7% of the patients. On the other hand, 33.3% presented with a salt-losing crisis, 4...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750653/risk-of-hypoglycemia-in-youth-with-type-2-diabetes-on-insulin
#3
Madhia Shahid, Gabriel Q Shaibi, Hayley Baines, Pamela Garcia-Filion, Zoe Gonzalez-Garcia, Micah Olson
BACKGROUND: The objective of this study was to ascertain the risk of hypoglycemia among youth with type 2 diabetes (T2D) on insulin therapy. METHODS: Twenty-two youth with T2D on insulin therapy (M=12, F=10, age=14.4±4.0 years) were enrolled from a single pediatric endocrine practice. They were followed-up for 3 months with weekly phone calls and monthly in-person visits to review blood glucose logs and document any signs or symptoms of hypoglycemia (defined as finger stick glucose of ≤70 mg/dL)...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750652/definition-of-reference-ranges-for-%C3%AE-isomerized-carboxy-terminal-telopeptide-collagen-type-i-for-children-and-adolescents
#4
Veronica C P de Melo, Paulo R S Ferreira, Lucelia O Ricardi, Marcelo C Batista, Carolina N França, Carlos E Dos S Ferreira
BACKGROUND: Bone metabolism involves many complex pathways that are disturbed by several bone diseases. The literature shows some limitations concerning pediatric reference intervals to bone markers, mainly because of the low number of patients included in the studies, the heterogeneity of methods, beyond the fact that it is time-consuming and expensive. The aim of this study was to determine reference values for β-isomerized carboxy-terminal telopeptides collagen type I (β-CTX), a marker of bone resorption, for children and adolescents...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750651/predictors-of-bone-maturation-growth-rate-and-adult-height-in-children-with-central-precocious-puberty-treated-with-depot-leuprolide-acetate
#5
Karen O Klein, Sanja Dragnic, Ahmed M Soliman, Peter Bacher
BACKGROUND: Children with central precocious puberty (CPP) are treated with gonadotropin-releasing hormone agonists (GnRHa) to suppress puberty. Optimizing treatment outcomes continues to be studied. The relationships between growth, rate of bone maturation (bone age/chronological age [ΔBA/ΔCA]), luteinizing hormone (LH), predicted adult stature (PAS), as well as variables influencing these outcomes, were studied in children treated with depot leuprolide (LA Depot) Methods: Subjects (64 girls, seven boys) with CPP received LA Depot every 3 months for up to 42 months...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750650/exertional-rhabdomyolysis-in-carbonic-anhydrase-12-deficiency
#6
Dekel Avital, Eli Hershkovitz, Neta Loewenthal
BACKGROUND: Carbonic anhydrase 12 (CA12) deficiency, a newly recognized rare disorder, has been described among Israeli Bedouin kindred as an autosomal recessive form of isolated salt wasting in sweat, which leads to severe infantile hyponatremic dehydration, visible salt precipitation after sweating, poor feeding and slow weight gain in infancy. CASE PRESENTATION: We present two adolescents diagnosed with CA12 deficiency who developed severe rhabdomyolysis as a result of physical activity in a hot climate...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750649/cardiac-examination-in-children-with-laron-syndrome-undergoing-mecasermin-therapy
#7
Nurdan Erol, Metin Yıldız, Ayla Güven, Ayse Yıldırım
BACKGROUND: Laron syndrome (LS), which can be defined as primary growth hormone resistance or insensitivity, is a rare genetic disease inherited by an autosomal recessive trait. Although it is undistinguishable from growth hormone deficiency, LS has high levels of growth hormone, but insulin-like growth factor (IGF-1) cannot be synthesized. Mecasermin treatment is the only option for the patients who suffer from LS. This study aims to research cardiac findings of children with LS, who receive treatment with mecasermin...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750648/current-status-of-the-congenital-hypothyroidism-neonatal-screening-program-in-adana-province-turkey
#8
Yılmaz Kor, Deniz Kor
BACKGROUND: Congenital hypothyroidism (CH) is a common cause of mental retardation; it has a worldwide incidence ranging from 1:3000 to 1:4500 live births. Predictably, an increase in the reported incidence of primary CH occurs when the cut-off levels of thyroid-stimulating hormone are lowered. We aimed to evaluate the results of a congenital hypothyroidism screening program and current status in this study. METHODS: Analysis results of 1300 infants who were referred to the endocrinology polyclinic because of suspected CH within the scope of the Ministry of Health National Neonatal Screening Program were retrospectively evaluated...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750647/the-etiologies-and-incidences-of-congenital-hypothyroidism-before-and-after-neonatal-tsh-screening-program-implementation-a-study-in-southern-thailand
#9
Somchit Jaruratanasirikul, Jutarat Piriyaphan, Tansit Saengkaew, Waricha Janjindamai, Hutcha Sriplung
BACKGROUND: Congenital hypothyroidism (CH) is one of the common causes of intellectual disability which can be prevented by early detection of an elevated thyroid stimulating hormone (TSH) level in the newborn and by treatment with thyroxine. In Thailand, neonatal TSH screening was implemented nationwide in 2005. The objective of the study was to determine the etiologies and the estimated incidences of CH in southern Thailand before and after the implementation of a neonatal TSH screening program in 2005...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29729148/brown-adipose-tissue-in-young-adults-who-were-born-preterm-or-small-for-gestational-age
#10
Anna Kistner, Henric Rydén, Björn Anderstam, Ann Hellström, Mikael Skorpil
BACKGROUND: Brown adipose tissue (BAT) is present and functions to dissipate energy as heat in young adults and can be assessed using magnetic resonance imaging (MRI) to estimate the voxel fat fraction, i.e. proton density fat fraction (PDFF). It is hypothesized that subjects born preterm or small for gestational age (SGA) may exhibit disrupted BAT formation coupled to metabolic factors. Our purpose was to assess the presence of BAT in young adults born extremely preterm or SGA in comparison with controls...
May 5, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29723156/circulating-soluble-rage-and-cell-surface-rage-on-peripheral-blood-mononuclear-cells-in-healthy-children
#11
Alberto García-Salido, Gustavo Melen, Vanesa Gómez-Piña, Gonzalo Oñoro-Otero, Ana Serrano-González, Juan Casado-Flores, Manuel Ramírez
BACKGROUND: The receptor for advanced glycation end products (RAGE) has a critical role in the pathogenesis of inflammation. In healthy children, its basal expression on the peripheral blood mononuclear cell (PBMC) and the basal circulating soluble RAGE (sRAGE) levels are unknown. The aim of this study was to describe both. METHODS: This is a monocentric, observational and descriptive study of samples obtained from healthy children. The RAGE expression on PBMC was analyzed using flow cytometry...
May 3, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29715194/trends-in-the-use-of-puberty-blockers-among-transgender-children-in-the-united-states
#12
Carla Marisa Lopez, Daniel Solomon, Susan D Boulware, Emily R Christison-Lagay
BACKGROUND: The objective of the study was to identify national trends in the utilization of histrelin acetate implants among transgender children in the United States. METHODS: We analyzed demographic, diagnostic and treatment data from 2004 to 2016 on the use of histrelin acetate reported to the Pediatric Health Information System (PHIS) to determine the temporal trends in its use for transgender-related billing diagnoses, e.g. "gender identity disorder"...
May 1, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29715193/severe-lactic-acidosis-in-an-extremely-low-birth-weight-infant-due-to-thiamine-deficiency
#13
Hulya Ozdemir, Hulya Bilgen, Zeynep Alp Unkar, Ertugrul Kiykim, Asli Memisoglu, Eren Ozek
BACKGROUND: In this case report, we present a preterm newborn with persistent lactic acidosis who received total parenteral nutrition (TPN) that lacked thiamine. CASE PRESENTATION: A 28-week-old, 750 g female infant was born with an Apgar score of 8 at the 5th minute. Umbilical cord blood gas levels, including lactate level, were normal, and she was admitted to our neonatal intensive care unit (NICU). Achieving full enteral feeding was not possible due to gastric residues and abdominal distention, making the patient dependent on TPN during the first 2 weeks of life...
May 1, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29715192/does-initial-dosing-of-levothyroxine-in-infants-with-congenital-hypothyroidism-lead-to-frequent-dose-adjustments-secondary-to-iatrogenic-hyperthyroidism-on-follow-up
#14
Meghan Craven, Graeme R Frank
BACKGROUND: Congenital hypothyroidism (CH) is the most common preventable cause of intellectual disability. The recommended starting dose of levothyroxine (LT4) is between 10 and 15 μg/kg, an extremely wide range. We hypothesized that a sizable proportion of newborns treated for CH at the higher end of the dosage range become biochemically hyperthyroid at a follow-up visit. METHODS: This study is a retrospective chart review of infants with CH between 2002 and 2012...
May 1, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29715191/five-novel-alms1-gene-mutations-in-six-patients-with-alstr%C3%A3-m-syndrome
#15
Suna Kılınç, Didem Yücel-Yılmaz, Aylin Ardagil, Süheyla Apaydın, Diana Valverde, Rıza Köksal Özgül, Ayla Güven
BACKGROUND: Alström syndrome is a rare autosomal recessive inherited disorder caused by mutations in the ALMS1 gene. METHODS: We describe the clinical and five novel mutational screening findings in six patients with Alström syndrome from five families in a single center with distinct clinical presentations of this condition. RESULTS: Five novel mutations in ALMS1 in exon 8 and intron 17 were identified, one of them was a compound heterozygous: c...
May 1, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29715190/incidence-of-congenital-hypothyroidism-in-china-data-from-the-national-newborn-screening-program-2013-2015
#16
Kui Deng, Chunhua He, Jun Zhu, Juan Liang, Xiaohong Li, Xiaoyan Xie, Ping Yu, Nana Li, Qi Li, Yanping Wang
BACKGROUND: Congenital hypothyroidism (CH) is one of the most frequent, preventable causes of mental retardation. Little has been reported on the epidemiological characteristics of CH in China. We aimed to estimate the incidence of CH in China and investigate its geographical variation. METHODS: We analyzed data from the nationwide newborn screening program for CH between 2013 and 2015. Poisson regression was used to generate the odds ratios (ORs) and 95% confidence intervals (CIs) between the rates of CH and selected demographic characteristics and assess the potential association between CH incidence and geographical locations...
May 1, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29688888/body-composition-and-metabolic-health-of-young-male-adults-with-childhood-onset-multiple-pituitary-hormone-deficiency-after-cessation-of-growth-hormone-treatment
#17
Hongbo Yang, Linjie Wang, Xiaonan Qiu, Kemin Yan, Fengying Gong, Huijuan Zhu, Hui Pan
BACKGROUND: Recombinant human growth hormone (rhGH) replacement therapy is usually stopped after linear growth completion in patients with growth hormone deficiency. In patients with multiple pituitary hormone deficiency (MPHD), the long-term effects of discontinuation of rhGH replacement are unknown. METHODS: In this study, the anthropometric and metabolic parameters of 24 male patients with adult growth hormone deficiency (AGHD) due to MPHD in childhood after cessation of rhGH therapy for a mean of 7...
April 24, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29688887/hematuria-as-an-adverse-outcome-following-provocative-growth-hormone-stimulation-testing-in-children
#18
Rajeev Thirunagari, Alexandra Marrone, Hannah Elsinghorst, Lucy D Mastrandrea
BACKGROUND: Provocative growth hormone (GH) stimulation testing is used to evaluate short stature and growth failure in children. Agents commonly used for testing include clonidine, arginine and glucagon. While stimulation testing is generally considered safe, gross hematuria has been described as a rare idiopathic complication of GH stimulation testing. This study was designed to estimate the incidence of both microscopic and macroscopic hematuria following GH testing with different provocative agents...
April 24, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29672274/cutaneous-mucormycosis-as-a-presenting-feature-of-type-1-diabetes-in-a-boy-case-report-and-review-of-the-literature
#19
Ganesh Jevalikar, Siddhnath Sudhanshu, Sanjay Mahendru, Smita Sarma, Khalid J Farooqui, Ambrish Mithal
BACKGROUND: Mucormycosis is a potentially fatal complication of diabetes. The rhino-orbito-cerebral form is the most common presentation, however, rarely other types can also be seen. CASE PRESENTATION: We describe the case of a 4½ -year-old boy not previously known to be a diabetic who presented to the plastic surgery department for gangrene of the left middle finger with surrounding erythema and induration. After the diagnosis of diabetes and initial treatment, pus from the wound showed broad aseptate hyphae suggestive of mucormycosis which was further confirmed on culture...
April 19, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29672273/bone-mineral-density-comparison-of-adolescents-with-constitutional-thinness-and-anorexia-nervosa
#20
Melis Pehlivantürk Kızılkan, Sinem Akgül, Orhan Derman, Nuray Kanbur
BACKGROUND: The negative impact of anorexia nervosa (AN) on bone health is well defined. However, there are very few studies evaluating the effect of constitutional thinness on bone health, especially in the adolescent period and in the male gender. The aim of this study is to compare the bone mineral density (BMD) measurements of adolescents with AN and with constitutional thinness. METHODS: Between April 2013 and March 2014, 40 adolescents with AN and 36 adolescents with constitutional thinness participated in the study...
April 19, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
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