Peter Gunczler - Academia.edu (original) (raw)

Papers by Peter Gunczler

Research paper thumbnail of Sindrome de Kocher-Debre-Semelaigne: hipotiroidismo con pseudohipertrofia muscular; reporte de dos casos en hermanas gemelas; Koscher-Debre-Semelaigne …

Arch. venez. pueric. …, 1987

Base de dados : LILACS. Pesquisa : 56030 [Identificador único]. Referências encontradas : 1 [refi... more Base de dados : LILACS. Pesquisa : 56030 [Identificador único]. Referências encontradas : 1 [refinar]. Mostrando: 1 .. 1 no formato [Detalhado]. página 1 de 1, 1 / 1, LILACS, seleciona. para imprimir. Fotocópia. experimental, Documentos relacionados. Id: 56030. ...

Research paper thumbnail of Distribución de las frecuencias alélicas y genotípicas del polimorfismo ghrd3 en pacientes venezolanos con talla baja

Revista Venezolana de …

Resumen: Objetivos. La deleción (GHRd3) o inserción (GHRfl) del exón 3 es un polimorfismo común e... more Resumen: Objetivos. La deleción (GHRd3) o inserción (GHRfl) del exón 3 es un polimorfismo común en el gen del receptor de la hormona de crecimiento (GHR) en los seres humanos. La presencia del alelo GHRd3 se ha asociado con el grado de respuesta ...

Research paper thumbnail of Endothelial Function, Carotid Artery Intima-Media Thickness, Epicardial Adipose Tissue, and Left Ventricular Mass and Function in Growth Hormone-Deficient Adolescents: Apparent Effects of Growth Hormone Treatment on These Parameters

The Journal of Clinical Endocrinology Metabolism, Jul 2, 2013

The purpose of this study was to determine whether GH-deficient (GHD) adolescents have abnormalit... more The purpose of this study was to determine whether GH-deficient (GHD) adolescents have abnormalities of cardiac and vascular function detectable during the teenage years. Ten GHD children on GH treatment with a chronological age (CA) of 14.6 +/- 1.7 yr and 12 untreated GHD adolescents with a CA of 15.0 +/- 3.0 yr were studied. Cardiac mass and function, carotid artery intima-media thickness, flow-mediated endothelium-dependent vasodilation (percent change from baseline diameter during hyperemia), and hyperemia-induced blood flow increase of the brachial artery (percent change from baseline) and epicardial adipose tissue were evaluated by echocardiography. Fourteen healthy adolescents served as controls. Untreated GHD adolescents present with a reduced left ventricular mass when compared with controls (P < 0.05) and a lower flow-mediated endothelium-dependent increase in the diameter of the brachial artery during hyperemia than both controls and treated GHD subjects (P < 0.02), whereas their epicardial adipose tissue is significantly higher than that of healthy controls (P < 0.02). Interventricular septum thickness, posterior wall thickness, left ventricular ejection fraction, and carotid artery intima-media thickness were similar in all three groups. Hyperemia-induced blood flow increase was greater in treated GHD adolescents than both untreated subjects and controls (P < 0.001). Body mass index correlated positively with epicardial adipose tissue in all three groups and with carotid intima-media thickness in treated and untreated GHD adolescents. GHD adolescents have a reduced left ventricular mass and vascular abnormalities manifested by lower flow-mediated endothelium-dependent vasodilation. These findings together with an increase in epicardial adipose tissue, a good indicator of abdominal/visceral fat, may contribute to an increased cardiovascular risk in the long term. An improvement in endothelial function and a reduction in arterial stiffness appear to occur after GH replacement.

Research paper thumbnail of Tratamiento de la pubertad precoz verdadera con analógos de GnRH: efectos sobre variables antropométricas y niveles hormonales

Arch Venez Pueric Pediatr, Dec 1, 1994

Research paper thumbnail of Higher prevalence of obesity and overweight without an adverse metabolic profile in girls with central precocious puberty compared to girls with early puberty, regardless of GnRH analogue treatment

International journal of pediatric endocrinology, 2014

1. To determine BMI, obesity/overweight rates, glucose and lipids at baseline, during GnRHa treat... more 1. To determine BMI, obesity/overweight rates, glucose and lipids at baseline, during GnRHa treatment and shortly after therapy discontinuation in female children with CPP and EP. 2. To compare this response to that seen in a similar group of untreated patients. A retrospective analysis of 71 children with either CPP (n = 37) or EP (n = 34) was undertaken. Forty three were treated with a GnRHa for at least 2 years, while 28 were followed without treatment. At the time of diagnosis, a higher BMI (z-score of 1.1 ± 0.8 vs. 0.6 ± 0.7, p = 0.004) and a higher prevalence of obesity/overweight (72.9 vs. 35.3%, p = 0.001) was observed in subjects with CPP when compared to those with EP. Children with EP had higher fasting glucose and total cholesterol than those with CPP. BMI z-score, obesity/overweight rates, fasting glucose and lipids did not change significantly in girls with CPP or EP during 3 yrs of follow up, regardless of treatment. Weight z-scores were higher at 3 years in treated t...

Research paper thumbnail of Epidemiology and inmunogenetics in recently diagnosed Venezuelan children with insulin-dependent diabetes mellitus

The Journal of pediatric endocrinology

Genetic and immunological markers in children with Type I diabetes have not been studied previous... more Genetic and immunological markers in children with Type I diabetes have not been studied previously in Venezuela. We evaluated 91 newly diagnosed IDDM children mean age 7.8 +/- 4.5 (range 0.8-20.8 years), 51 females and 40 males. Eleven percent of first degree relatives had a family history of Type I IDDM; 56.7% had had upper respiratory infection prior to diagnosis and 12.7% had had either mumps or varicella. Peak incidence of disease was found in February and March and August to October. Eighty seven percent had HLA-DR3 and/or DR4 vs 36% of the Venezuelan general population; 81.6% were HLA-DQW2 and/or HLA-DQW8. We found 55.9% to have positive islet cell antibodies (ICA) with 4 of these having a positive complement fixation test. Three patients (7.9%) were found to have positive insulin autoantibodies. Only 3 out of 11 HLA-identical siblings had positive ICAs, while none had positive insulin autoantibodies. One of them also had a positive complement fixation test; this subject deve...

Research paper thumbnail of Decreased Trabecular Bone Mineral Density in Children with Idiopathic Short Stature. Normalization of Bone Density and Increased Bone Turnover after One Year of Growth Hormone Treatment

Pediatric Research, 1999

Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment wit... more Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment with GH leads to an improvement in their bone mineral density (BMD). The effect of GH on the BMD of children with idiopathic short stature is unknown. We studied 14 short, slowly growing, otherwise healthy, prepubertal children without GH deficiency (7 girls and 7 boys) with a chronological age of 10.9 +/- 1.4 years, bone age of 8.8 +/- 1.5 years, and height of 127.8 +/- 8.5 cm (height SD score of -2.2 +/- 0.5). Growth velocity increased from 3.9 +/- 1.1 cm/y to 8.0 +/- 1.9 cm/y, and height SD score improved from -2.2 +/- 0.5 to -1.8 +/- 0.5 after 12 months of GH treatment (P <.007 and P <.001, respectively). Baseline lumbar spine BMD was decreased when compared with that of a control group of children matched for bone age and height (0.645 +/- 0.09 g/cm(2) vs 0.730 +/- 0.08 g/cm(2); P <.003). Lumbar spine BMD increased in subjects with ISS after 1 year of GH treatment from 0.645 +/- 0.09 g/cm(2) to 0.808 +/- 0.04 g/cm(2) (P <.05), reaching levels similar to those of control subjects, followed up without therapy (0.808 +/- 0.04 g/cm(2) vs 0.760 +/- 0.08 g/cm(2)); lumbar spine BMD increased 25.3% in the subjects with ISS and 4.1% in the control subjects. Femoral neck BMD did not change during treatment. Serum concentrations of the carboxy-terminal propeptide of type 1 collagen increased from 231.6 +/- 65.5 microg/L to 351.6 +/- 87.2 microg/L, and levels of the carboxy-terminal cross-linked telopeptide of type 1 collagen increased from 9.9 +/- 5.9 microg/L to 13.9 +/- 2.4 microg/L. Children with ISS have decreased lumbar spine BMD, which increases with GH therapy, reaching levels similar to those of control subjects. Bone turnover increased as indicated by a rise in bone formation and bone resorption markers.

Research paper thumbnail of Decreased trabecular bone mineral density (BMD) in children and adolescents with insulin dependent diabetes mellitus (IDDM)

Osteoporosis International, 1996

ABSTRACT Without Abstract

Research paper thumbnail of Marcadores genéticos y estudios epidemiológicos en la diabetes insulino dependiente en niños venezolanos

SUMMARY New developments in the epidemiology and genetics of type I diabetes mellitus of autoimmu... more SUMMARY New developments in the epidemiology and genetics of type I diabetes mellitus of autoimmune etiology and the discovery of immunogenetic markers, are helping the physiopathology of the slow process of pancreatic beta cell destruction in the pre-diabetic stage that finally ends in the classical form of insulin-dependent diabetes mellitus. It has been only in these past years that this

Research paper thumbnail of EPIDEMIOLOGY AND IMMUNOGENETICS OF INSULIN-DEPENDENT DIABETES MELLITUS IN VENEZUELAN CHILDREN

Research paper thumbnail of Decreased Bone Mineral Densities (BMD) and Bone Formation Markers During the First Year After Diagnosis of Insulin Dependent Diabetes Mellitus (IDDM) in Children. 431

Research paper thumbnail of 6??OH Cortisol (6OHF)as Indicator of Altered Cortisol (F) Metabolism

Pediatric Research, 1978

normal, 8 hypothyroid and 1 hyperthyroid child. On hand of the results obtained in the hypothyroi... more normal, 8 hypothyroid and 1 hyperthyroid child. On hand of the results obtained in the hypothyroid children the wthors feel that the unresponsiveness of TSH to TRH does not necessarily indicate a pituitary defect. The authors have also analyzed the values of LH and FSH during stimulation with LH-RH in 32 patients with delayed puberty, in 4 children with primary and in 8 children with seconda-

Research paper thumbnail of Deficiency of Cortisol 11??-Ketoreductase ??? A New Metabolic Defect

Pediatric Research, 1978

e v e l s of 17-OH progesterone ( I 7-OHP) , testosterone(T) and c o r t i s o l (F) i n children... more e v e l s of 17-OH progesterone ( I 7-OHP) , testosterone(T) and c o r t i s o l (F) i n children with cong e n i t a l adrenal hyperplasia (CAH) w i t h and without treatment.

Research paper thumbnail of CHANGES IN BONE MINERAL DENSITY, GROWTH VELOCITY AND RENAL FUNCTION IN UREMIC PREPUBERTAL CHILDREN TREATED WITH GROWTH HORMONE

Pediatric Research, 1995

Chronic emsure to sex steroids dmmg childhccd accelerates skeletal mrituration and mv oom-crromis... more Chronic emsure to sex steroids dmmg childhccd accelerates skeletal mrituration and mv oom-crromise f &a1 heicht. The arowth of 26 chlldren (18 glrls, 8 Gys) hisease-free of A~C for more rt.an 1 yr were renewed. The xltlal clu-lcal slappeared at 2.9 t 2.8 yr and the dlap.osls wa.; made at 3.6 f 2.8 yr. Tlme of follow-up after suroerv was 6 2 f 3.5 vr (ranoe 1.5 -13 0). 50% had adrenogenltai sp-~drome (AS ) , 38% mlxed s$drome (MS: Cushmg ' s plus hS), 8% no endocrine syrptoms and 4% C~shlng syndrone At the t~re of dlaonosis the mean H-SDS was hlober than taroet helait-SDS (TH-SDS; p-< 0.0001). H-SDS was not dizferent betwe& AS &d MS groups (g > 0.1). Bone age lB.4; 6.2 53.6) was grearer than height age (HA; 4.2 : 2.7) and chronological age (CA; 3.8t 2.91 (P < 0.05), whereas :iA a~d CA were not different . -BA adv~nced mre than CA m the 1st

Research paper thumbnail of 318 DEFICIENCY OF CORTISOL 11-β-KETOREDUCTASE — METABOLIC DEFECT

Pediatric Research, 1978

Sponsored by Lewis A. Barness. The importance of good glycemic control is the presumed prevention... more Sponsored by Lewis A. Barness. The importance of good glycemic control is the presumed prevention of the long term sequela of dlabetes.

Research paper thumbnail of Decreased trabecular bone mineral density in children with idiopathic short stature: Normalization of bone density and increased bone turnover after 1 year of growth hormone treatment

The Journal of Pediatrics, 1999

Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment wit... more Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment with GH leads to an improvement in their bone mineral density (BMD). The effect of GH on the BMD of children with idiopathic short stature is unknown. We studied 14 short, slowly growing, otherwise healthy, prepubertal children without GH deficiency (7 girls and 7 boys) with a chronological age of 10.9 +/- 1.4 years, bone age of 8.8 +/- 1.5 years, and height of 127.8 +/- 8.5 cm (height SD score of -2.2 +/- 0.5). Growth velocity increased from 3.9 +/- 1.1 cm/y to 8.0 +/- 1.9 cm/y, and height SD score improved from -2.2 +/- 0.5 to -1.8 +/- 0.5 after 12 months of GH treatment (P &lt;.007 and P &lt;.001, respectively). Baseline lumbar spine BMD was decreased when compared with that of a control group of children matched for bone age and height (0.645 +/- 0.09 g/cm(2) vs 0.730 +/- 0.08 g/cm(2); P &lt;.003). Lumbar spine BMD increased in subjects with ISS after 1 year of GH treatment from 0.645 +/- 0.09 g/cm(2) to 0.808 +/- 0.04 g/cm(2) (P &lt;.05), reaching levels similar to those of control subjects, followed up without therapy (0.808 +/- 0.04 g/cm(2) vs 0.760 +/- 0.08 g/cm(2)); lumbar spine BMD increased 25.3% in the subjects with ISS and 4.1% in the control subjects. Femoral neck BMD did not change during treatment. Serum concentrations of the carboxy-terminal propeptide of type 1 collagen increased from 231.6 +/- 65.5 microg/L to 351.6 +/- 87.2 microg/L, and levels of the carboxy-terminal cross-linked telopeptide of type 1 collagen increased from 9.9 +/- 5.9 microg/L to 13.9 +/- 2.4 microg/L. Children with ISS have decreased lumbar spine BMD, which increases with GH therapy, reaching levels similar to those of control subjects. Bone turnover increased as indicated by a rise in bone formation and bone resorption markers.

Research paper thumbnail of Growth hormone deficiency, low levels of adiponectin, and unfavorable plasma lipid and lipoproteins

The Journal of Pediatrics, 2006

Objective To examine the impact of adolescent growth hormone deficiency (GHD) on circulating adip... more Objective To examine the impact of adolescent growth hormone deficiency (GHD) on circulating adiponectin levels and the relation between adiponectin, fasting insulin, plasma lipid, and lipoprotein levels.

Research paper thumbnail of Coronary artery calcification, serum lipids, lipoproteins, and peripheral inflammatory markers in adolescents and young adults with type 1 diabetes

The Journal of Pediatrics, 2006

To determine whether coronary artery calcification (CAC), elevated fasting lipids, and lipoprotei... more To determine whether coronary artery calcification (CAC), elevated fasting lipids, and lipoproteins and peripheral inflammatory markers are present in insulin-dependent diabetic adolescents and young adults several years after diagnosis. Hispanic insulin-dependent diabetics (n = 32) diagnosed a mean of 7.8 +/- 4.5 years ago (range, 3 to 16 years), with a mean glycosylated hemoglobin concentration at the time of the study of 8.8% +/- 2.3% and a mean chronological age of 16.1 +/- 4.4 years, were evaluated. Healthy patients (n = 15) with a chronological age (CA) of 15.2 +/- 2.2 years served as control subjects. CAC was assessed by multiple slice computed tomography, and total CAC score in Agatston units was calculated. Fasting lipids, C-reactive protein, apolipoprotein (Apo) A, Apo B, and metalloproteinase-9 (MMP-9) concentrations were measured in all subjects. Neither adolescents with type 1 diabetes nor healthy control subjects presented with evidence of CAC. Fasting lipids, Apo A, Apo B, CRP, and MMP-9 concentrations were similar between diabetic subjects and control subjects. However, 34.4% and 25.0% of our type 1 diabetic subjects had elevated total and LDL cholesterol levels (&amp;amp;amp;amp;gt;200 and &amp;amp;amp;amp;gt;130 mg/dL, respectively), whereas 15.6% and 28.1% had elevated triglyceride and Apo B concentrations (&amp;amp;amp;amp;gt;150 mg/dL and &amp;amp;amp;amp;gt;100 mg/dL, respectively). In addition, 28.1% and 34.4% presented with elevated CRP and MMP-9 levels (&amp;amp;amp;amp;gt;2 mg/L and &amp;amp;amp;amp;gt;80 ng/mL, respectively). Total, LDL and HDL cholesterol, triglycerides, Apo B, CRP, and MMP-9 concentrations correlated positively with duration of the disease and with glycosylated hemoglobin levels. Although the study adolescents with type 1 diabetes did not present any radiologic evidence of CAC at this stage of the disease, they remain a high-risk group for the development of microvascular and macrovascular artery disease, as risk factors such as elevated lipoproteins and proinflammatory markers are already present in a significant percentage of patients studied.

Research paper thumbnail of Rathke Cleft Cyst as Cause of Growth Hormone Deficiency in a 9-Year-Old Girl

The Journal of Pediatrics, 2012

Research paper thumbnail of Decreased Bone Mineral Density and Bone Formation Markers Shortly After Diagnosis of Clinical Type 1 Diabetes Mellitus

Journal of Pediatric Endocrinology and Metabolism, 2000

We recently demonstrated that children with type 1 diabetes mellitus (DM) have decreased lumbar s... more We recently demonstrated that children with type 1 diabetes mellitus (DM) have decreased lumbar spine bone mineral density (BMD) as early as four years after clinical diagnosis of the disease. In order to determine whether osteopenia is already present in patients very early on after diagnosis of clinical DM, we evaluated the bone mineral status of a group of newly diagnosed children (5.8 ± 1.5 mo after diagnosis). We studied 23 prepubertal children (7 M, 16 F) with a mean chronological age of 9.5 ± 2.2 yr and a mean glycosylated hemoglobin of 8.9 ± 2.4%. Lumbar spine and femoral neck BMD were measured by dual X-ray absorptiometry, while bone turnover was assessed by the determination of the serum concentration of the carboxyterminal propeptide of type I collagen (PICP) and the carboxy-terminal cross-linked telopeptide of type I collagen (N-telopeptide). Results were compared to those of age, height, and pubertal status matched controls. Lumbar spine BMD Z-scores were decreased in patients compared to controls (Z-scores of -0.89 ± 1.2, with 10 of 22 patients showing values >1 SD below the mean). When lumbar spine Z-scores were analyzed in those patients with <3 months or z3 months since diagnosis of DM a significant difference was noticed between groups (-0.648 ± 1.12 vs -1.267 ± 1.17; P <0.02). No significant differences were noted in femoral neck BMD and total BMD between groups. Serum PICP levels were decreased when compared to controls (233.6 ± 39.3 vs 375.9 ± 50.7 ~gll; P <0.002), while serum N-telopeptide concentrations, although increased, were not significantly different (9.3 ± 1.3 vs 5.7 ± 1.5 ug/l), In summary, early on after the diagnosis of type 1 DM, children present with decreased lumbar spine BMD and decreased bone formation markers. KEYWORDS type I diabetes mellitus, bone mineral density, bone formation markers, bone resorption markers

Research paper thumbnail of Sindrome de Kocher-Debre-Semelaigne: hipotiroidismo con pseudohipertrofia muscular; reporte de dos casos en hermanas gemelas; Koscher-Debre-Semelaigne …

Arch. venez. pueric. …, 1987

Base de dados : LILACS. Pesquisa : 56030 [Identificador único]. Referências encontradas : 1 [refi... more Base de dados : LILACS. Pesquisa : 56030 [Identificador único]. Referências encontradas : 1 [refinar]. Mostrando: 1 .. 1 no formato [Detalhado]. página 1 de 1, 1 / 1, LILACS, seleciona. para imprimir. Fotocópia. experimental, Documentos relacionados. Id: 56030. ...

Research paper thumbnail of Distribución de las frecuencias alélicas y genotípicas del polimorfismo ghrd3 en pacientes venezolanos con talla baja

Revista Venezolana de …

Resumen: Objetivos. La deleción (GHRd3) o inserción (GHRfl) del exón 3 es un polimorfismo común e... more Resumen: Objetivos. La deleción (GHRd3) o inserción (GHRfl) del exón 3 es un polimorfismo común en el gen del receptor de la hormona de crecimiento (GHR) en los seres humanos. La presencia del alelo GHRd3 se ha asociado con el grado de respuesta ...

Research paper thumbnail of Endothelial Function, Carotid Artery Intima-Media Thickness, Epicardial Adipose Tissue, and Left Ventricular Mass and Function in Growth Hormone-Deficient Adolescents: Apparent Effects of Growth Hormone Treatment on These Parameters

The Journal of Clinical Endocrinology Metabolism, Jul 2, 2013

The purpose of this study was to determine whether GH-deficient (GHD) adolescents have abnormalit... more The purpose of this study was to determine whether GH-deficient (GHD) adolescents have abnormalities of cardiac and vascular function detectable during the teenage years. Ten GHD children on GH treatment with a chronological age (CA) of 14.6 +/- 1.7 yr and 12 untreated GHD adolescents with a CA of 15.0 +/- 3.0 yr were studied. Cardiac mass and function, carotid artery intima-media thickness, flow-mediated endothelium-dependent vasodilation (percent change from baseline diameter during hyperemia), and hyperemia-induced blood flow increase of the brachial artery (percent change from baseline) and epicardial adipose tissue were evaluated by echocardiography. Fourteen healthy adolescents served as controls. Untreated GHD adolescents present with a reduced left ventricular mass when compared with controls (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05) and a lower flow-mediated endothelium-dependent increase in the diameter of the brachial artery during hyperemia than both controls and treated GHD subjects (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.02), whereas their epicardial adipose tissue is significantly higher than that of healthy controls (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.02). Interventricular septum thickness, posterior wall thickness, left ventricular ejection fraction, and carotid artery intima-media thickness were similar in all three groups. Hyperemia-induced blood flow increase was greater in treated GHD adolescents than both untreated subjects and controls (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). Body mass index correlated positively with epicardial adipose tissue in all three groups and with carotid intima-media thickness in treated and untreated GHD adolescents. GHD adolescents have a reduced left ventricular mass and vascular abnormalities manifested by lower flow-mediated endothelium-dependent vasodilation. These findings together with an increase in epicardial adipose tissue, a good indicator of abdominal/visceral fat, may contribute to an increased cardiovascular risk in the long term. An improvement in endothelial function and a reduction in arterial stiffness appear to occur after GH replacement.

Research paper thumbnail of Tratamiento de la pubertad precoz verdadera con analógos de GnRH: efectos sobre variables antropométricas y niveles hormonales

Arch Venez Pueric Pediatr, Dec 1, 1994

Research paper thumbnail of Higher prevalence of obesity and overweight without an adverse metabolic profile in girls with central precocious puberty compared to girls with early puberty, regardless of GnRH analogue treatment

International journal of pediatric endocrinology, 2014

1. To determine BMI, obesity/overweight rates, glucose and lipids at baseline, during GnRHa treat... more 1. To determine BMI, obesity/overweight rates, glucose and lipids at baseline, during GnRHa treatment and shortly after therapy discontinuation in female children with CPP and EP. 2. To compare this response to that seen in a similar group of untreated patients. A retrospective analysis of 71 children with either CPP (n = 37) or EP (n = 34) was undertaken. Forty three were treated with a GnRHa for at least 2 years, while 28 were followed without treatment. At the time of diagnosis, a higher BMI (z-score of 1.1 ± 0.8 vs. 0.6 ± 0.7, p = 0.004) and a higher prevalence of obesity/overweight (72.9 vs. 35.3%, p = 0.001) was observed in subjects with CPP when compared to those with EP. Children with EP had higher fasting glucose and total cholesterol than those with CPP. BMI z-score, obesity/overweight rates, fasting glucose and lipids did not change significantly in girls with CPP or EP during 3 yrs of follow up, regardless of treatment. Weight z-scores were higher at 3 years in treated t...

Research paper thumbnail of Epidemiology and inmunogenetics in recently diagnosed Venezuelan children with insulin-dependent diabetes mellitus

The Journal of pediatric endocrinology

Genetic and immunological markers in children with Type I diabetes have not been studied previous... more Genetic and immunological markers in children with Type I diabetes have not been studied previously in Venezuela. We evaluated 91 newly diagnosed IDDM children mean age 7.8 +/- 4.5 (range 0.8-20.8 years), 51 females and 40 males. Eleven percent of first degree relatives had a family history of Type I IDDM; 56.7% had had upper respiratory infection prior to diagnosis and 12.7% had had either mumps or varicella. Peak incidence of disease was found in February and March and August to October. Eighty seven percent had HLA-DR3 and/or DR4 vs 36% of the Venezuelan general population; 81.6% were HLA-DQW2 and/or HLA-DQW8. We found 55.9% to have positive islet cell antibodies (ICA) with 4 of these having a positive complement fixation test. Three patients (7.9%) were found to have positive insulin autoantibodies. Only 3 out of 11 HLA-identical siblings had positive ICAs, while none had positive insulin autoantibodies. One of them also had a positive complement fixation test; this subject deve...

Research paper thumbnail of Decreased Trabecular Bone Mineral Density in Children with Idiopathic Short Stature. Normalization of Bone Density and Increased Bone Turnover after One Year of Growth Hormone Treatment

Pediatric Research, 1999

Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment wit... more Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment with GH leads to an improvement in their bone mineral density (BMD). The effect of GH on the BMD of children with idiopathic short stature is unknown. We studied 14 short, slowly growing, otherwise healthy, prepubertal children without GH deficiency (7 girls and 7 boys) with a chronological age of 10.9 +/- 1.4 years, bone age of 8.8 +/- 1.5 years, and height of 127.8 +/- 8.5 cm (height SD score of -2.2 +/- 0.5). Growth velocity increased from 3.9 +/- 1.1 cm/y to 8.0 +/- 1.9 cm/y, and height SD score improved from -2.2 +/- 0.5 to -1.8 +/- 0.5 after 12 months of GH treatment (P &lt;.007 and P &lt;.001, respectively). Baseline lumbar spine BMD was decreased when compared with that of a control group of children matched for bone age and height (0.645 +/- 0.09 g/cm(2) vs 0.730 +/- 0.08 g/cm(2); P &lt;.003). Lumbar spine BMD increased in subjects with ISS after 1 year of GH treatment from 0.645 +/- 0.09 g/cm(2) to 0.808 +/- 0.04 g/cm(2) (P &lt;.05), reaching levels similar to those of control subjects, followed up without therapy (0.808 +/- 0.04 g/cm(2) vs 0.760 +/- 0.08 g/cm(2)); lumbar spine BMD increased 25.3% in the subjects with ISS and 4.1% in the control subjects. Femoral neck BMD did not change during treatment. Serum concentrations of the carboxy-terminal propeptide of type 1 collagen increased from 231.6 +/- 65.5 microg/L to 351.6 +/- 87.2 microg/L, and levels of the carboxy-terminal cross-linked telopeptide of type 1 collagen increased from 9.9 +/- 5.9 microg/L to 13.9 +/- 2.4 microg/L. Children with ISS have decreased lumbar spine BMD, which increases with GH therapy, reaching levels similar to those of control subjects. Bone turnover increased as indicated by a rise in bone formation and bone resorption markers.

Research paper thumbnail of Decreased trabecular bone mineral density (BMD) in children and adolescents with insulin dependent diabetes mellitus (IDDM)

Osteoporosis International, 1996

ABSTRACT Without Abstract

Research paper thumbnail of Marcadores genéticos y estudios epidemiológicos en la diabetes insulino dependiente en niños venezolanos

SUMMARY New developments in the epidemiology and genetics of type I diabetes mellitus of autoimmu... more SUMMARY New developments in the epidemiology and genetics of type I diabetes mellitus of autoimmune etiology and the discovery of immunogenetic markers, are helping the physiopathology of the slow process of pancreatic beta cell destruction in the pre-diabetic stage that finally ends in the classical form of insulin-dependent diabetes mellitus. It has been only in these past years that this

Research paper thumbnail of EPIDEMIOLOGY AND IMMUNOGENETICS OF INSULIN-DEPENDENT DIABETES MELLITUS IN VENEZUELAN CHILDREN

Research paper thumbnail of Decreased Bone Mineral Densities (BMD) and Bone Formation Markers During the First Year After Diagnosis of Insulin Dependent Diabetes Mellitus (IDDM) in Children. 431

Research paper thumbnail of 6??OH Cortisol (6OHF)as Indicator of Altered Cortisol (F) Metabolism

Pediatric Research, 1978

normal, 8 hypothyroid and 1 hyperthyroid child. On hand of the results obtained in the hypothyroi... more normal, 8 hypothyroid and 1 hyperthyroid child. On hand of the results obtained in the hypothyroid children the wthors feel that the unresponsiveness of TSH to TRH does not necessarily indicate a pituitary defect. The authors have also analyzed the values of LH and FSH during stimulation with LH-RH in 32 patients with delayed puberty, in 4 children with primary and in 8 children with seconda-

Research paper thumbnail of Deficiency of Cortisol 11??-Ketoreductase ??? A New Metabolic Defect

Pediatric Research, 1978

e v e l s of 17-OH progesterone ( I 7-OHP) , testosterone(T) and c o r t i s o l (F) i n children... more e v e l s of 17-OH progesterone ( I 7-OHP) , testosterone(T) and c o r t i s o l (F) i n children with cong e n i t a l adrenal hyperplasia (CAH) w i t h and without treatment.

Research paper thumbnail of CHANGES IN BONE MINERAL DENSITY, GROWTH VELOCITY AND RENAL FUNCTION IN UREMIC PREPUBERTAL CHILDREN TREATED WITH GROWTH HORMONE

Pediatric Research, 1995

Chronic emsure to sex steroids dmmg childhccd accelerates skeletal mrituration and mv oom-crromis... more Chronic emsure to sex steroids dmmg childhccd accelerates skeletal mrituration and mv oom-crromise f &a1 heicht. The arowth of 26 chlldren (18 glrls, 8 Gys) hisease-free of A~C for more rt.an 1 yr were renewed. The xltlal clu-lcal slappeared at 2.9 t 2.8 yr and the dlap.osls wa.; made at 3.6 f 2.8 yr. Tlme of follow-up after suroerv was 6 2 f 3.5 vr (ranoe 1.5 -13 0). 50% had adrenogenltai sp-~drome (AS ) , 38% mlxed s$drome (MS: Cushmg ' s plus hS), 8% no endocrine syrptoms and 4% C~shlng syndrone At the t~re of dlaonosis the mean H-SDS was hlober than taroet helait-SDS (TH-SDS; p-< 0.0001). H-SDS was not dizferent betwe& AS &d MS groups (g > 0.1). Bone age lB.4; 6.2 53.6) was grearer than height age (HA; 4.2 : 2.7) and chronological age (CA; 3.8t 2.91 (P < 0.05), whereas :iA a~d CA were not different . -BA adv~nced mre than CA m the 1st

Research paper thumbnail of 318 DEFICIENCY OF CORTISOL 11-β-KETOREDUCTASE — METABOLIC DEFECT

Pediatric Research, 1978

Sponsored by Lewis A. Barness. The importance of good glycemic control is the presumed prevention... more Sponsored by Lewis A. Barness. The importance of good glycemic control is the presumed prevention of the long term sequela of dlabetes.

Research paper thumbnail of Decreased trabecular bone mineral density in children with idiopathic short stature: Normalization of bone density and increased bone turnover after 1 year of growth hormone treatment

The Journal of Pediatrics, 1999

Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment wit... more Patients with growth hormone (GH) deficiency have impaired bone mineral metabolism; treatment with GH leads to an improvement in their bone mineral density (BMD). The effect of GH on the BMD of children with idiopathic short stature is unknown. We studied 14 short, slowly growing, otherwise healthy, prepubertal children without GH deficiency (7 girls and 7 boys) with a chronological age of 10.9 +/- 1.4 years, bone age of 8.8 +/- 1.5 years, and height of 127.8 +/- 8.5 cm (height SD score of -2.2 +/- 0.5). Growth velocity increased from 3.9 +/- 1.1 cm/y to 8.0 +/- 1.9 cm/y, and height SD score improved from -2.2 +/- 0.5 to -1.8 +/- 0.5 after 12 months of GH treatment (P &lt;.007 and P &lt;.001, respectively). Baseline lumbar spine BMD was decreased when compared with that of a control group of children matched for bone age and height (0.645 +/- 0.09 g/cm(2) vs 0.730 +/- 0.08 g/cm(2); P &lt;.003). Lumbar spine BMD increased in subjects with ISS after 1 year of GH treatment from 0.645 +/- 0.09 g/cm(2) to 0.808 +/- 0.04 g/cm(2) (P &lt;.05), reaching levels similar to those of control subjects, followed up without therapy (0.808 +/- 0.04 g/cm(2) vs 0.760 +/- 0.08 g/cm(2)); lumbar spine BMD increased 25.3% in the subjects with ISS and 4.1% in the control subjects. Femoral neck BMD did not change during treatment. Serum concentrations of the carboxy-terminal propeptide of type 1 collagen increased from 231.6 +/- 65.5 microg/L to 351.6 +/- 87.2 microg/L, and levels of the carboxy-terminal cross-linked telopeptide of type 1 collagen increased from 9.9 +/- 5.9 microg/L to 13.9 +/- 2.4 microg/L. Children with ISS have decreased lumbar spine BMD, which increases with GH therapy, reaching levels similar to those of control subjects. Bone turnover increased as indicated by a rise in bone formation and bone resorption markers.

Research paper thumbnail of Growth hormone deficiency, low levels of adiponectin, and unfavorable plasma lipid and lipoproteins

The Journal of Pediatrics, 2006

Objective To examine the impact of adolescent growth hormone deficiency (GHD) on circulating adip... more Objective To examine the impact of adolescent growth hormone deficiency (GHD) on circulating adiponectin levels and the relation between adiponectin, fasting insulin, plasma lipid, and lipoprotein levels.

Research paper thumbnail of Coronary artery calcification, serum lipids, lipoproteins, and peripheral inflammatory markers in adolescents and young adults with type 1 diabetes

The Journal of Pediatrics, 2006

To determine whether coronary artery calcification (CAC), elevated fasting lipids, and lipoprotei... more To determine whether coronary artery calcification (CAC), elevated fasting lipids, and lipoproteins and peripheral inflammatory markers are present in insulin-dependent diabetic adolescents and young adults several years after diagnosis. Hispanic insulin-dependent diabetics (n = 32) diagnosed a mean of 7.8 +/- 4.5 years ago (range, 3 to 16 years), with a mean glycosylated hemoglobin concentration at the time of the study of 8.8% +/- 2.3% and a mean chronological age of 16.1 +/- 4.4 years, were evaluated. Healthy patients (n = 15) with a chronological age (CA) of 15.2 +/- 2.2 years served as control subjects. CAC was assessed by multiple slice computed tomography, and total CAC score in Agatston units was calculated. Fasting lipids, C-reactive protein, apolipoprotein (Apo) A, Apo B, and metalloproteinase-9 (MMP-9) concentrations were measured in all subjects. Neither adolescents with type 1 diabetes nor healthy control subjects presented with evidence of CAC. Fasting lipids, Apo A, Apo B, CRP, and MMP-9 concentrations were similar between diabetic subjects and control subjects. However, 34.4% and 25.0% of our type 1 diabetic subjects had elevated total and LDL cholesterol levels (&amp;amp;amp;amp;gt;200 and &amp;amp;amp;amp;gt;130 mg/dL, respectively), whereas 15.6% and 28.1% had elevated triglyceride and Apo B concentrations (&amp;amp;amp;amp;gt;150 mg/dL and &amp;amp;amp;amp;gt;100 mg/dL, respectively). In addition, 28.1% and 34.4% presented with elevated CRP and MMP-9 levels (&amp;amp;amp;amp;gt;2 mg/L and &amp;amp;amp;amp;gt;80 ng/mL, respectively). Total, LDL and HDL cholesterol, triglycerides, Apo B, CRP, and MMP-9 concentrations correlated positively with duration of the disease and with glycosylated hemoglobin levels. Although the study adolescents with type 1 diabetes did not present any radiologic evidence of CAC at this stage of the disease, they remain a high-risk group for the development of microvascular and macrovascular artery disease, as risk factors such as elevated lipoproteins and proinflammatory markers are already present in a significant percentage of patients studied.

Research paper thumbnail of Rathke Cleft Cyst as Cause of Growth Hormone Deficiency in a 9-Year-Old Girl

The Journal of Pediatrics, 2012

Research paper thumbnail of Decreased Bone Mineral Density and Bone Formation Markers Shortly After Diagnosis of Clinical Type 1 Diabetes Mellitus

Journal of Pediatric Endocrinology and Metabolism, 2000

We recently demonstrated that children with type 1 diabetes mellitus (DM) have decreased lumbar s... more We recently demonstrated that children with type 1 diabetes mellitus (DM) have decreased lumbar spine bone mineral density (BMD) as early as four years after clinical diagnosis of the disease. In order to determine whether osteopenia is already present in patients very early on after diagnosis of clinical DM, we evaluated the bone mineral status of a group of newly diagnosed children (5.8 ± 1.5 mo after diagnosis). We studied 23 prepubertal children (7 M, 16 F) with a mean chronological age of 9.5 ± 2.2 yr and a mean glycosylated hemoglobin of 8.9 ± 2.4%. Lumbar spine and femoral neck BMD were measured by dual X-ray absorptiometry, while bone turnover was assessed by the determination of the serum concentration of the carboxyterminal propeptide of type I collagen (PICP) and the carboxy-terminal cross-linked telopeptide of type I collagen (N-telopeptide). Results were compared to those of age, height, and pubertal status matched controls. Lumbar spine BMD Z-scores were decreased in patients compared to controls (Z-scores of -0.89 ± 1.2, with 10 of 22 patients showing values >1 SD below the mean). When lumbar spine Z-scores were analyzed in those patients with <3 months or z3 months since diagnosis of DM a significant difference was noticed between groups (-0.648 ± 1.12 vs -1.267 ± 1.17; P <0.02). No significant differences were noted in femoral neck BMD and total BMD between groups. Serum PICP levels were decreased when compared to controls (233.6 ± 39.3 vs 375.9 ± 50.7 ~gll; P <0.002), while serum N-telopeptide concentrations, although increased, were not significantly different (9.3 ± 1.3 vs 5.7 ± 1.5 ug/l), In summary, early on after the diagnosis of type 1 DM, children present with decreased lumbar spine BMD and decreased bone formation markers. KEYWORDS type I diabetes mellitus, bone mineral density, bone formation markers, bone resorption markers