somatomedin-C Levels Research Articles

Evaluation of Bone Mineral Density in Children with Celiac Disease

Background: Osteoporosis is a known entity in individuals with celiac disease (CD). Objectives: The aim of this study was to evaluate bone mineral density (BMD) measurements in children with CD. Methods: This was a retrospective design study in a tertiary hospital in Turkey. Results: Totally 106 patients were included, and the mean age was 10.2 ± 3.9 years. The mean L1-L4 Z score was 0.730 ± 0.197 (based on sex and height in Turkish children). About 20.7% (n = 22) had a history of at least one fracture and/or bone pain. Bone mineral density values obtained from lumbar (L1-L4) vertebrae were significantly lower in patients with a prior fracture and/or bone pain compared to the patient group without a fracture (0.822 ± 0.242 g/cm2 vs 0.706 ± 0.178 g/cm2, respectively; P = 0.047). L1-L4 Z score (based on sex and height in Turkish children) was lower in patients with a body mass index (BMI) SDS value below -2 (moderately or severely malnourished) than those with a BMI SDS above -2 (0.02 ± 1.19 vs 1.4 ± 1.82, respectively; P = 0.014). A moderate positive correlation (r = 0.547; P < 0.01) was found between somatomedin-C (IGF-1) level and L1-L4 BMD (g/cm2) measurement. It was remarkable that low BMD was not identified in any patients using L1-L4 Z-score by height and L1-L4 Z-score by age of Turkish children. Nine (8.5%) patients were found to have low BMD using the United States (US) age- and sex-specific L1-L4 Z-score from the GE Healthcare Lunar iDXA system. Conclusions: Bone mineral density should be screened considering risk factors for suboptimal bone health in children with celiac disease. False "normal" BMD interpretations can be avoided by taking into account the model of the BMD measuring device and by using the device's normal ranges in case of inconsistency with the Turkish reference values.

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Oral supplement enriched in HMB combined with pulmonary rehabilitation improves body composition and health related quality of life in patients with bronchiectasis (Prospective, Randomised Study)

Pulmonary Rehabilitation (PR) is recommended for bronchiectasis but there is no data about its effect on body composition. The aim of this study is to assess the effect of Pulmonary Rehabilitation (PR) for 12 weeks in normally-nourished non-cystic-fibrosis bronchiectasis patients compared with the effect of PR plus a hyperproteic oral nutritional supplement enriched with beta-hydroxy-beta-methylbutyrate (HMB) on body composition, muscle strength, quality of life and serum biomarkers. single center randomized controlled trial, parallel treatment design: Participants were randomly assigned to receive PR for 12 weeks or PR plus ONS (PRONS) (one can per day). Outcome assessments were performed at baseline, 12 weeks and 24 weeks: body composition (Dual-energy X-Ray Absorptiometry (DEXA), mid-arm muscle circumference (MAMC), phase angle by Bio-impedance), health related quality of life (Spanish QOL-B-V3.0, Physical Functioning Scale), handgrip strength, diet questionnaire, and plasma levels of prealbumin, myostatin and somatomedin-c. Thirty patients were randomized (15 per group) without differences in clinical and respiratory variables. In the PRONS group bone mineral density (BMD), mean and maximum handgrip dynamometry, MAMC, QOLB and prealbumin were significantly increased from baseline at 12 and 24 weeks and Fat free Mass (FFM) and FFM index, at 12 weeks. In the PR group only mean handgrip dynamometry and prealbumin were significantly increased at 12 and 24 weeks. In both groups plasma myostatin was reduced at 12 weeks (without significant differences). The addition of a hyperproteic ONS enriched with HMB to Pulmonary Rehabilitation could improve body composition, BMD, muscle strength and health related quality of life in bronchiectasis patients. Clinical Trials Number NCT02048397.

‘What kind of diabetes did you say I have?’

A 15-year-old male with septo-optic dysplasia, panhypopituitarism, developmental delay, diabetes insipidus, and autism was admitted to the hospital for hypoglycaemia. The patient started having sudden seizure activity that prompted fuller evaluation. His glucose level, upon admission to the hospital, was 28mg/dl (1.6mmol/L). The patient was resuscitated with intravenous glucose and his seizure activity stopped. The past medical history included the diagnosis of septo-optic dysplasia at birth, growth hormone deficiency, secondary hypothyroidism, secondary adrenal insufficiency, diabetes insipi-dus, blindness, aggressive behaviour and autism. The patient had been taking growth hormone, levothyroxine, hydrocortisone and desmopressin acetate. There was no history of missed doses, fever, missed meals, vomiting, nor any other illnesses. He attends a school for blind children with special needs. His condition was monitored by a school nurse and supervised by a physician responsible for the school district system. His physical examination revealed no rashes, hyperpigmentation, nor any focal neurological finding. Laboratory findings revealed initial hypoglycaemia as mentioned previously, normal T4, and somatomedin-C levels, white blood cell count, and electrolytes. During hospital stay, the patient recovered from hypoglycaemia and went back to his previous functioning levels. Since there was no clear aetiology for the hypoglycaemia episode, the endocrinology team asked for documented dietary intake and scheduled medications given prior to hospital admission. This revealed no missed medication doses, but showed two daily doses of 20 units of insulin glargine (rDNA origin) injection that were given to the patient in the past two days prior to hospital admission. Further investigation showed that there was a new physician who took over the responsibility of the school system, and he ordered the insulin doses for a presumed diagnosis of diabetes mellitus rather than diabetes insipidus. The nurse never questioned the sudden start of insulin therapy without the documentation of hyperglycaemia. The mother was notified about the cause of hypoglycaemia; she sought legal action against this physician at the state school. The school system decided to settle the case out of court. The physician was penalised by the local State Medical Board and was required to take educational training courses to be able to differentiate types of diabetes. ‘Diabetes’ means to ‘flow through’ or to ‘siphon’ in Greek language since affected patients urinate frequently. Ancient Greek physicians used to taste urine to differentiate the two types of diabetes (mellitus vs insipidus) to distinguish the honeyed sweet diabetes. While the two conditions share the word ‘diabetes’, their aetiologies and medical management are completely different. Of note, Wolfram syndrome, or DIDMOAD syndrome, combines diabetes insipidus (DI), diabetes mellitus (DM), optic nerve atrophy (OA) and deafness (D).

Decreased Peripheral Insulin Sensitivity in Acromegalic Patients

In 13 nondiabetic acromegalic patients glucose homeostasis was studied by use of the hyperglycaemic clamp technique and compared to a group of sex and age matched and a group of sex, age and weight matched controls. When compared to a control group of normal weight glucose stimulated insulin release (I) was significantly increased and tissue sensitivity to insulin (M/I) significantly decreased. However, no significant differences were observed when the parameters were compared with a weight matched group. Glucose stimulated insulin release correlated positively with growth hormone (GH) and somatomedin-C levels, whereas no such a correlation could be obtained for M/I. Thus, chronic growth hormone excess seems to induce hyperinsulinaemia which in turn leads to obesity and metabolic changes comparable to those of obesity.

Endocrine Effects of Erythropoietin in Cancer Patients

The recent advances in the knowledge of the psychoneuroimmunological pathogenesis of human neoplasms have demonstrated the existence of feed-back mechanisms operating between interleukins and endocrine secretions, which play an important role in the regulation of the immune responses, including the anticancer immunity. In contrast, few studies only have been performed to investigate the possible relation between endocrine activities and hematopoietic growth factors. The present study was performed to analyze the acute endocrine effects of erythropoietin-alpha (EPO) on the main endocrine secretions. The study was carried out in 10 advanced solid tumor patients. EPO was injected subcutaneously at a dose of 10,000 U, and venous blood samples were collected before and 2, 4 and 6 h after EPO administration. No significant changes in mean serum levels of FSH, LH and TSH were seen in response to EPO. Cortisol and DHEAS concentrations increased after EPO injection, whereas those of PRL decreased, but none of these differences was statistically significant. Finally, mean serum levels of both growth hormone (GH) and somatomedin-C (IGF-1) significantly decreased after EPO administration. This preliminary study shows that EPO may inhibit GH secretion from the pituitary gland and IGF-1 production. Since GH would stimulate EPO release, the results of this study may suggest the existence of feedback mechanism operating between GH secretion and EPO production, with inhibitory effect of EPO on GH secretion, and stimulatory action of GH on EPO production. Therefore, this study would describe the first example of hemato-endocrine feedback mechanisms. Moreover, this study, by showing an inhibitory effect of EPO on IGF-1 secretion, would suggest a possible use of EPO in the medical oncology not only for the treatment of cancer related anemia, but also to counteract tumor growth by blocking IGF-1 production, which has been proven to be a growth factor for several tumor histotypes. Obviously, IGF-1 is not the only tumor growth factor, but it could play a fundamental role in the regulation of production and activity of several other tumor growth factors. In any case, this study describes the only acute endocrine effects of EPO. Therefore, further studies, by evaluating the endocrine effects of a chronic treatment with EPO, will be required to establish which may be its effect on IGF-1 endogenous production, and its consequence on survival time.

Growth hormone secreting adenoma with unusual extension: coexisting pituitary cyst and its clinical significance

A 58 year old man showed acromegalic features. The serum growth hormone (GH) level was 7.3 ng/ml and SMC (somatomedin-C) 637 U/ml. Triple stimulation test showed abnormal response compatible with a GH secreting tumour. The conventional enhanced MRI revealed a less enhanced hemisphere-shaped lesion at the right corner of the sella turcica. In addition, dynamic MRI demonstrated an elongated lesion extending to the left beyond the midline. The patient underwent transsphenoidal surgery. Besides the soft and suckable tumour at the right corner, we entered into a small cavity loosely filled with the tumour, which was subsequently also removed. The operative finding corresponded to the lesion shown in dynamic MRI. Postoperative GH and SMC levels became 2.3 ng/ml and 326 U/ml respectively.Incidental pituitary cystic lesions in autopsied cases have been reported to be 6–33%. This case had a GH secreting adenoma with coexisting pituitary cyst. The coexisting pituitary cyst supposedly influenced the unusual shape and extension of the pituitary adenoma. Coexistence of such lesion should be kept in mind for microadenoma on neuroradiological evaluation and on intraoperative inspection surrounding the tumour. Copyright 2000 Harcourt Publishers LtdKeywords: GH secreting tumour, MRI, dynamic MRI, pituitary cyst

High Serum Levels of Somatomedin-C and Diabetes Mellitus Caused by Obstructive Hydrocephalus: Case Report

High Serum Levels of Somatomedin-C and Diabetes Mellitus Caused by Obstructive Hydrocephalus: Case Report

High Serum Levels of Somatomedin-C and Diabetes Mellitus Caused by Obstructive Hydrocephalus: Case Report

High Serum Levels of Somatomedin-C and Diabetes Mellitus Caused by Obstructive Hydrocephalus: Case Report

Factors predicting pituitary adenoma invasiveness in acromegalic patients.

Forty-four adult acromegalic patients carrying growth hormone-producing pituitary macroadenomas were investigated with neuroradiological and endocrinological techniques. Plasma growth hormone and somatomedin-C levels were repeatedly measured before surgical removal of tumors and during the follow-up period. Twenty-five patients presented preoperatively with an invasive adenoma that involved the cavernous sinus (CS). Diagnosis of tumor invasivity was made according to distinct neuroradiological criteria and was confirmed or rejected during surgery Significantly higher basal growth hormone levels were found in patients with CS invasion than in cases without tumor growth in the CS. Evidence is presented that plasma growth hormone level in acromegalics is a more sensitive indicator for predicting tumor invasiveness than somatomedin-C. Growth hormone basal values before surgery and the extent of their decrease after removal of tumor correlate with adenoma growth in the parasellar compartments and should be used as a prognostic factor to aid in planing adjuvant tumor treatment.

Attenuating effect of castration on glomerular injury is age-dependent in unilaterally nephrectomized male Sprague-Dawley rats.

To clarify a role of sex hormones in greater susceptibility of young rats than adults to the development of focal and segmental glomerulosclerosis (FSGS), we castrated animals at different ages and investigated whether the attenuating effect of castration on FSGS is age-dependent in unilaterally nephrectomized male Sprague-Dawley (SD) rats. At 6 weeks of age, all groups received unilateral right nephrectomy (Nx) and group 2 was simultaneously castrated, while group 1 received a sham operation. Group 3 was castrated at 3 months of age, and group 4 at 6 months of age. Body weight, blood pressure, urinary protein and serum constituents were investigated every 2 months from 4 to 14 months of age. At 6 and 14 months of age, rats were studied morphologically. Castration at 6 weeks of age or at 3 months of age significantly inhibited the compensatory glomerular hypertrophy and hyperfunction with regard to the creatinine clearance as seen in Nx rats at 6 months of age and significantly reduced glomerular injury at the end of the experiment, while castration at 6 months produced neither an inhibitory effect on glomerular hypertrophy nor an attenuating effect on glomerular injury. Serum levels of growth hormone (GH) and somatomedin-C (SmC) were decreased by castration to a greater extent when castrated at younger age. These findings indicated that GH and SmC influenced by male sex hormone seem to play a more important role at younger age than in adults in exerting its effect on glomerular growth, leading somehow to glomerular injury in aging, unilaterally nephrectomized male SD rats.

Growth in children undergoing bone marrow transplantation after busulfan and cyclophosphamide conditioning.

The purpose of this study was to evaluate the effect of busulfan and cyclophosphamide (BUCY) as conditioning regimen for bone marrow transplantation (BMT) on growth of children following BMT. Growth assessment was prospectively done in all 25 children who underwent BMT following BUCY conditioning from 1989 to 1994 at Children's Hospital of Philadelphia. The height and growth rates were expressed as standard deviation scores (SDS). The height SDS prior to BMT were compared with 1, 2, and 3 years post-BMT. The growth rate SDS 1 year and 2 years post-BMT were also compared. Pubertal children were excluded from the analysis of growth rate. Median age of patients was 7 years (range, 1.3-15 years). A total of 22/25 patients were transplanted for AML, and three patients had myelodysplastic syndrome. Equal numbers of patients had autologous and allogeneic transplants. Seven patients received corticosteroids for varying lengths of time. The pre-BMT height SDS (mean +/- SD) for the group was -0.4 +/- 1.3. The mean height SDS for 19 children at 1 year post-BMT was +/- 0.1 +/- 1.2 and for 10 children, 2 years post-BMT was -0.3 +/- 1.6. Seven children who were 3 years post-BMT had the mean height SDS of -0.2 +/- 1.5. There was no statistically significant difference between pre-BMT height SDS and 1 year post-BMT (p = 0.49) and 2 years post-BMT (p = 0.42). The mean growth rate at 1 year post-BMT was -0.1 +/- 2.7 and at 2 years post-BMT was -0.9 +/- 2.3. The difference was not statistically different (p = 0.15). Somatomedin-C (insulin growth factor 1, IGF-1) levels were normal in all 13 children tested at 1 year post-BMT. IGF binding protein (BP)-3 levels were done in 10 children at 1 year and were found to be normal in all. Thyroid function studies were done in all patients pre-BMT and 1 year post-BMT and were normal for all. Bone age assessment was appropriate for age in all 14 patients tested at 1 year post-BMT. There was no evidence of growth failure following BMT with BUCY conditioning in this group of patients.

An Association between Acromegaly and Thyroid Carcinoma

Acromegaly has been associated with goiter as well as with benign and malignant tumors. Three cases of thyroid carcinoma in patients with acromegaly had been reported in the literature. We previously reported two additional cases of thyroid carcinoma and now present a third from 100 patients with acromegaly, exceeding the expected prevalence. All three thyroid carcinomas were multifocal, suggesting a generalized promotion toward malignancy. These patients had elevated levels of growth hormone and somatomedin-C [insulin-like growth factor (IGF-1)] at the time of thyroid carcinoma diagnosis. We discuss the role of IGF-1 in both benign and malignant thyroid growth, including the presence of IGF-1 receptors on thyroid cells, stimulation of thyroid cell replication by IGF-1, and paracrine secretion of IGF-1 and its binding proteins by thyroid cells. We propose possible mechanisms for the development of thyroid carcinoma in patients with acromegaly and discuss implications for the physician treating patients with acromegaly.

Growth hormone response to growth hormone releasing hormone and to clonidine stimulation in peripubertal patients with Major Depressive Disorder

The responses of growth hormone (GH) to administration of growth hormone-releasing hormone (GHRH-1 μg/kg b.w.) and of clonidine (clon-2.5 μg/kg b.w.) and basal levels of somatomedin C (SmC) were measured in nine peripubertal patients with Major Depressive Disorder (MDD) and in 9 age- and gender-matched controls. Basal GH and SmC levels, and GH response to GHRH did not differ in patients and controls, whereas responses to clonidine were significantly higher in some and lower in other patients than in controls.

Triplet pregnancy after pulsatile gonadotrophin-releasing hormone treatment in an acromegalic woman

A triplet pregnancy is reported in an acromegalic woman with hypothalamic amenorrhoea treated with pulsatile gonadotrophin-releasing hormone (GnRH). The patient was on bromocriptine medication and had slightly elevated growth hormone (GH) and somatomedin-C (Sm-C) levels. This probably accounted for the conception of triplets in the first stimulation cycle.

Effect of Parenteral Hyperalimentation on Growth in Glycogen Storage Disease Type IA

A boy who was diagnosed at 8 months of age as suffering from glycogen storage disease (GSD) type Ia, was treated with parenteral hyperalimentation (PHA). He had previously been treated with frequent meals and uncooked cornstarch, but compliance was poor and physical development was delayed. Growth hormone secretion was normal, but somatomedin-C (Sm-C) levels were low. Multiple focal fatty changes were found in the liver at age 12 years, when home PHA (960kcal/day) was started. Soon thereafter growth velocity increased markedly, and Sm-C levels became normal. The liver lesions also disappeared. Thus PHA was effective in the treatment of this patient with GSD Ia.

Systemic growth hormone does not affect human sleep.

Subsequent to sleep onset, GH concentrations increase markedly, suggesting a stimulatory influence of sleep on GH secretion. However, results have been inconsistent as to whether GH conversely exerts a significant influence on sleep. Hence, the effects of exogenous administration of GH and of GH secretion stimulated by GH-releasing hormone (GHRH) on sleep were reexamined in 3 experiments in healthy male volunteers. In Exp I, 12 men participated on 3 experimental nights, receiving a constant iv infusion of 5 IU GH (between 2100-0700 h), an im bolus injection of 5 IU GH at 2100 h, and placebo. In Exp II, the effects of a short iv infusion of a high dose of 48 IU GH (between 2345-2315 h) on sleep were evaluated in 3 men. In Exp III, the effects of continuous infusion of 30 micrograms/h GHRH (between 2200-0700 h) on sleep were compared to the placebo condition in 10 men. Experiments were double blind, within-subject, cross-over comparisons and included an adaptation night before experimental nights. On all nights, the subjects went to bed at 2300 h and were awakened at 0700 h. Administration of GH elevated plasma GH and somatomedin-C levels throughout the night (P < 0.005). Neither im administration of 5 IU GH nor iv administration of 5 and 48 IU GH had any effect on the total sleep time or the time spent in different sleep stages during the whole night or in the first and second halves of sleep time. Infusion of GHRH increased nocturnal GH secretion (P < 0.005), but the episodic pattern of GH secretion was maintained. However, sleep remained unchanged during GHRH infusion. From these results we conclude that in healthy man, systemic GH has no physiological role for sleep regulation.

Klinefelter Syndrome with Short Stature

A case of Klinefelter syndrome (KS) is presented where the height SD score was -2.0 and the bone age was 7.0yr when the chronological age was 11.0yr. The patienthad a normal response to a GH stimulation test, but his mean GH secretion during sleep, the first morning-voided urinary GH and the plasma somatomedin-C (Sm-C) levels were abnormally low. It is likely that the patient's short stature resulted from GH neurosecretory dysfunction (GHND).

Growth and Development in White Patients with Sickle Cell Diseases

We have evaluated height, weight, bone age, somatomedin-C levels, and pubertal development in 114 Sicilian patients affected by sickle cell diseases (SCDs). Thirty-one had homozygous sickle hemoglobin (SS), 55 S-beta 0 thalassemia, and 28 S-beta + thalassemia. In both children and adults, the mean height and weight were approximately 1 SD below the normal mean for age. The height was below the normal range only in a few subjects (8 children and 4 adult women). Somatomedin-C levels were within the normal range in most of the patients (37/44 children and 17/22 adults). Bone age revealed a slight delay in skeletal maturation (mean chronological age and bone age were 7.7 +/- 3 and 7.11 +/- 2.9 respectively; p < 0.05). Mean age at menarche was increased compared to normal subjects. Our findings show that Sicilian patients with SCD exhibit a moderate delay of growth and adolescence but attain a final height within the normal range.

Growth and Development in White Patients with Sickle Cell Diseases

We have evaluated height, weight, bone age, somatomedin-C levels, and pubertal development in 114 Sicilian patients affected by sickle cell diseases (SCDs). Thirty-one had homozygous sickle hemoglobin (SS), 55 S-beta 0 thalassemia, and 28 S-beta + thalassemia. In both children and adults, the mean height and weight were approximately 1 SD below the normal mean for age. The height was below the normal range only in a few subjects (8 children and 4 adult women). Somatomedin-C levels were within the normal range in most of the patients (37/44 children and 17/22 adults). Bone age revealed a slight delay in skeletal maturation (mean chronological age and bone age were 7.7 +/- 3 and 7.11 +/- 2.9 respectively; p < 0.05). Mean age at menarche was increased compared to normal subjects. Our findings show that Sicilian patients with SCD exhibit a moderate delay of growth and adolescence but attain a final height within the normal range.