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Sample records for hormone deficient children

  1. Growth Hormone Deficiency in Children

    MedlinePlus

    ... Slowed growth in height in infants, children, or adolescents (teenagers) • A young-looking face compared with other ... your child if you see signs of poor self-esteem or sadness that could be related to being ...

  2. Genetics Home Reference: isolated growth hormone deficiency

    MedlinePlus

    ... Isolated growth hormone deficiency Educational Resources (10 links) Boston Children's Hospital CLIMB: Growth Hormone Deficiency Information Sheet (PDF) Disease InfoSearch: Isolated growth hormone deficiency ...

  3. Growth hormone deficiency in children and young adults.

    PubMed

    Oświęcimska, Joanna; Roczniak, Wojciech; Mikołajczak, Agata; Szymlak, Agnieszka

    2016-09-13

    Growth hormone (GH) is a naturally occurring polypeptide hormone produced by somatotropic cells in the anterior pituitary. The main function of somatotropin is stimulation of linear growth, but it also affects carbohydrate metabolism, increases bone mass and has potent lipolytic, antinatriuretic and antidiuretic effects. Growth hormone deficiency (GHD) may occur both in children and in adults. At the moment there is no gold standard for the diagnosis of GHD, and the diagnosis should take into account clinical, auxological, biochemical and radiological changes and, if necessary, genetic testing. Recent studies have highlighted that the biochemical diagnosis of GH deficiency is still imperfect. Stimuli used in the tests are non-physiological, and various substances are characterized by a different mechanism of action and potency. A few years ago it was thought that GHD treatment in children must be completed at the end of linear growth. Studies performed in the last two decades have shown that GHD deficiency in adults may result in complex clinical problems, and if untreated shortens the life expectancy and worsens its comfort. Discontinuation of GH therapy after the final height has been reached in fact negatively impacts the physiological processes associated with the transition phase, which is the period of human life between achieving the final height and 25-30 years of age. Given the adverse metabolic effects of GH treatment interruption after linear growth has been completed, the latest recommendations propose reassessment of GH secretion in the period at least one month after cessation of treatment and continuation of the therapy in case of persistent deficit.

  4. Growth hormone treatment in non-growth hormone-deficient children

    PubMed Central

    Carta, Luisanna; Ibba, Anastasia; Guzzetti, Chiara

    2014-01-01

    Until 1985 growth hormone (GH) was obtained from pituitary extracts, and was available in limited amounts only to treat severe growth hormone deficiency (GHD). With the availability of unlimited quantities of GH obtained from recombinant DNA technology, researchers started to explore new modalities to treat GHD children, as well as to treat a number of other non-GHD conditions. Although with some differences between different countries, GH treatment is indicated in children with Turner syndrome, chronic renal insufficiency, Prader-Willi syndrome, deletions/mutations of the SHOX gene, as well as in short children born small for gestational age and with idiopathic short stature. Available data from controlled trials indicate that GH treatment increases adult height in patients with Turner syndrome, in patients with chronic renal insufficiency, and in short children born small for gestational age. Patients with SHOX deficiency seem to respond to treatment similarly to Turner syndrome. GH treatment in children with idiopathic short stature produces a modest mean increase in adult height but the response in the individual patient is unpredictable. Uncontrolled studies indicate that GH treatment may be beneficial also in children with Noonan syndrome. In patients with Prader-Willi syndrome GH treatment normalizes growth and improves body composition and cognitive function. In any indication the response to GH seems correlated to the dose and the duration of treatment. GH treatment is generally safe with no major adverse effects being recorded in any condition. PMID:24926456

  5. Effect of growth hormone-releasing factor on growth hormone release in children with radiation-induced growth hormone deficiency

    SciTech Connect

    Lustig, R.H.; Schriock, E.A.; Kaplan, S.L.; Grumbach, M.M.

    1985-08-01

    Five male children who received cranial irradiation for extrahypothalamic intracranial neoplasms or leukemia and subsequently developed severe growth hormone (GH) deficiency were challenged with synthetic growth hormone-releasing factor (GRF-44), in an attempt to distinguish hypothalamic from pituitary dysfunction as a cause of their GH deficiency, and to assess the readily releasable GH reserve in the pituitary. In response to a pulse of GRF-44 (5 micrograms/kg intravenously), mean peak GH levels rose to values higher than those evoked by the pharmacologic agents L-dopa or arginine (6.4 +/- 1.3 ng/mL v 1.5 +/- 0.4 ng/mL, P less than .05). The peak GH value occurred at a mean of 26.0 minutes after administration of GRF-44. These responses were similar to those obtained in children with severe GH deficiency due to other etiologies (peak GH 6.3 +/- 1.7 ng/mL, mean 28.0 minutes). In addition, there was a trend toward an inverse relationship between peak GH response to GRF-44 and the postirradiation interval. Prolactin and somatomedin-C levels did not change significantly after the administration of a single dose of GRF-44. The results of this study support the hypothesis that cranial irradiation in children can lead to hypothalamic GRF deficiency secondary to radiation injury of hypothalamic GRF-secreting neurons. This study also lends support to the potential therapeutic usefulness of GRF-44 or an analog for GH deficiency secondary to cranial irradiation.

  6. Effect of growth hormone (GH)-releasing hormone (GRH) on plasma GH in relation to magnitude and duration of GH deficiency in 26 children and adults with isolated GH deficiency or multiple pituitary hormone deficiencies: evidence for hypothalamic GRH deficiency.

    PubMed

    Schriock, E A; Lustig, R H; Rosenthal, S M; Kaplan, S L; Grumbach, M M

    1984-06-01

    Synthetic, amidated, 44 amino acid GH-releasing hormone ( GRH -44) was administered iv at a dose of 5 micrograms/kg to 20 patients with severe GH deficiency (GHD), 6 children and adolescents with partial GHD, and 6 non-GH deficient ( NGHD ) children and adolescents. The 17 patients with severe GHD that responded to GRH -44 had lower peak concentrations of plasma GH than the NGHD individuals (5.0 +/- 1.2 (SEM) vs. 27.2 +/- 3.5 ng/ml; P less than 0.0001). The children and adolescents with severe GHD tended to have higher peak GH responses to GRH -44 than the GHD adults (6.9 +/- 1.7 vs. 2.4 +/- 0.3 ng/ml) although the difference was not significant. The peak GH concentration was attained earlier in the GHD children and adolescents than in the GHD adults (28 +/- 4.7 vs. 69.3 +/- 13 min, P less than 0.004). There was a negative correlation between chronological age and peak plasma GH response to GRH in the children and adolescents with severe GHD (r = -0.758, P less than 0.02). Children and adolescents with partial GHD had a higher mean peak concentration of plasma GH (13. 1 +/- 1.8 ng/ml) than the children, adolescents, and adults with severe GHD (P less than 0.04), but one lower than the NGHD children and adolescents (P less than 0.05). In both severe and partial GHD the GH response to GRH was greater than that elicited by standard pharmacological tests. Serum somatomedin-C did not increase after a single pulse of GRH -44 in the 12 GHD patients studied. PRL increased minimally 30 min after 5 micrograms/kg iv GRH -44 in patients with multiple hypothalamic-pituitary hormone deficiencies but not in patients with isolated GHD or in NGHD individuals. The GH responses to GRH suggest that the majority of patients with isolated GHD as well as those with multiple hypothalamic-pituitary hormone deficiencies have deficiency of hypothalamic GRH . Lack of a GH response to a single pulse of GRH does not exclude GRH deficiency as priming of the somatotrope with multiple pulses of

  7. Educating children and families about growth hormone deficiency and its management: part 1.

    PubMed

    Collin, Jacqueline; Whitehead, Amanda; Walker, Jenny

    2016-02-01

    The management of growth hormone deficiency is long term. Children may be diagnosed at pre-school age meaning relationships with the paediatric endocrine team may last more than 15 years. The education role of the paediatric endocrine nurse specialist is essential in working in partnership with families over a long period of time. Children and young people have changing needs for information to help them understand their condition and growth hormone deficiency treatment as they grow up. Developing positive working relationships with parents, children and young people enables their developmental needs and the context in which they live their lives to be central to any educational planning for them. Addressing developmental needs when providing information on growth hormone deficiency to children and young people reinforces the need for education to be an ongoing process and not a one-off event. This is part one of a two-part article. The second part will be published in the March issue of Nursing Children and Young People and it focuses on educating children, young people and their parents about the condition, and includes case studies.

  8. Growth hormone deficiency

    MedlinePlus

    ... dosage of the medicine. Serious side effects of growth hormone treatment are rare. Common side effects include: Headache Fluid ... years. The rate of growth then slowly decreases. Growth hormone therapy does not work for all children. Left untreated, ...

  9. Educating children and families about growth hormone deficiency and its management: part 2.

    PubMed

    Collin, Jacqueline; Whitehead, Amanda; Walker, Jenny

    2016-03-01

    Growth hormone deficiency (GHD) is a long-term condition, therefore creating ongoing partnerships with families is a fundamental part of the role of a paediatric endocrine nurse specialist (PENS). Teaching children, young people and their families about GHD and exploring what it means to them and how they can manage their ongoing treatment is central to building positive relationships. Educating children about the management of their growth hormone treatment (GHT) is an ongoing process and professionals must respond to the changing needs for that information children may have as they grow and develop. Long-term relationships with families are strengthened by recognising and respecting the developing expertise of families as they gain confidence and competence to manage GHT. This article is the second of two parts. Part one was published in the February issue of Nursing Children and Young People and covered an overview of growth hormone, causes and clinical presentation of GHD, development and availability of GHT and the role of the PENS in building partnerships with parents. The focus of this article is the education role of the PENS and the importance of providing information that is appropriate to the child or young person's developmental age.

  10. Association between Decreased Klotho Blood Levels and Organic Growth Hormone Deficiency in Children with Growth Impairment

    PubMed Central

    Ben Ami, Michal; Levy-Shraga, Yael; Mazor-Aronovitch, Kineret; Pinhas-Hamiel, Orit; Yeshayahu, Yonatan; Hemi, Rina; Kanety, Hannah; Rubinek, Tami; Modan-Moses, Dalit

    2014-01-01

    Objective Klotho is an aging-modulating protein expressed mainly in the kidneys and choroid plexus, which can also be shed, released into the circulation and act as a hormone. Klotho deficient mice are smaller compared to their wild-type counterparts and their somatotropes show marked atrophy and reduced number of secretory granules. Recent data also indicated an association between klotho levels and growth hormone (GH) levels in acromegaly. We aimed to study the association between klotho levels and GH deficiency (GHD) in children with growth impairment. Design Prospective study comprising 99 children and adolescents (aged 9.0±3.7 years, 49 male) undergoing GH stimulation tests for short stature (height-SDS = −2.1±0.6). Klotho serum levels were measured using an α-klotho ELISA kit. Results Klotho levels were significantly lower (p<0.001) among children with organic GHD (n = 11, 727±273 pg/ml) compared to both GH sufficient participants (n = 59, 1497±754 pg/ml) and those with idiopathic GHD (n = 29, 1645±778 pg/ml). The difference between GHS children and children with idiopathic GHD was not significant. Klotho levels positively correlated with IGF-1- standard deviation scores (SDS) (R = 0.45, p<0.001), but were not associated with gender, pubertal status, age or anthropometric measurements. Conclusions We have shown, for the first time, an association between low serum klotho levels and organic GHD. If validated by additional studies, serum klotho may serve as novel biomarker of organic GHD. PMID:25198618

  11. Treatment of short stature and growth hormone deficiency in children with somatotropin (rDNA origin).

    PubMed

    Hardin, Dana S

    2008-12-01

    Somatotropin (growth hormone, GH) of recombinant DNA origin has provided a readily available and safe drug that has greatly improved management of children and adolescents with GH deficiency (GHD) and other disorders of growth. In the US and Europe, regulatory agencies have given approval for the use of GH in children and adults who meet specific criteria. However, clinical and ethical controversies remain regarding the diagnosis of GHD, dosing of GH, duration of therapy and expected outcomes. Areas which also require consensus include management of pubertal patients, transitioning pediatric patients to adulthood, management of children with idiopathic short stature and the role of recombinant IGF-1 in treatment. Additionally, studies have demonstrated anabolic benefits of GH in children who have inflammatory-based underlying disease and efficacy of GH in overcoming growth delays in people treated chronically with corticosteroids. These areas are open for possible new uses of this drug. This review summarizes current indications for GH use in children and discusses areas of clinical debate and potential anabolic uses in chronic illness.

  12. Relationship between initial treatment effect of recombinant human growth hormone and exon 3 polymorphism of growth hormone receptor in Chinese children with growth hormone deficiency

    PubMed Central

    Zheng, Zhangqian; Cao, Lingfeng; Pei, Zhou; Zhi, Dijing; Zhao, Zhuhui; Xi, Li; Cheng, Ruoqian; Luo, Feihong

    2015-01-01

    The aim of this study is to investigate the frequency distribution of exon 3 deleted (d3-GHR) genetic polymorphism of growth hormone receptor (GHR) in growth hormone deficient (GHD) Chinese children and to explore the correlation between the growth promoting effects of recombinant human growth hormone (rhGH) and exon 3 genetic polymorphism of GHR in GHD children. In this study, 111 GHD (excluded small for gestational age) children were treated with rhGH (0.20 mg/kg/week) for six months. The body height (Ht), body weight, bone age (BA) and growth velocity (GV) were measured before and after six months of treatment. The d3-GHR and full length GHR (fl-GHR) were analyzed to detect the frequency distribution of two isoforms and their influence on growth promoting effect of rhGH. The results indicated that the frequencies of fl/fl, fl/d3 and d3/d3 GHR genotypes were 67.6%, 18.9% and 13.5%. After six months of GH therapy, there were significant differences of ΔGV (ΔGV: 10.77±3.40 cm/year vs 12.18±3.08 cm/year) (P<0.05) and ΔHt (ΔHt: 5.38±1.70 cm vs 6.09±1.54 cm) (P<0.05) were found among GHD children with different genotypes (fl/fl vs fl/d3 and d3/d3). In conclusion, the frequency distribution of three GHR genotypes in 111 Chinese GHD children was different from that reported in Caucasian, indicating the existence of ethnic difference of exon 3 GHR polymorphism. There was a closely relationship between GHR genotypes and growth-promoting effect of rhGH in Chinese GHD children. PMID:26221355

  13. Vitamin D deficiency in Korean children: prevalence, risk factors, and the relationship with parathyroid hormone levels

    PubMed Central

    Chung, In Hyuk; Kim, Hae Jung; Chung, Sochung

    2014-01-01

    Purpose This study was performed to investigate the relationship between serum vitamin D and parathyroid hormone (PTH) levels as well as to describe the prevalence and the risk factors of vitamin D deficiency (VDD) in Korean children. Methods Participants were 1,212 children aged 4 to 15 years, who visited Bundang CHA Medical Center (located at 37°N) between March 2012 and February 2013. Overweight was defined as body mass index≥85th percentile. Participants were divided into 4 age groups and 2 seasonal groups. VDD was defined by serum 25-hydroxyvitamin D (25OHD) <20 ng/mL. Results The level of 25OHD was significantly lower in overweight group than in normal weight group (17.1±5.1 ng/mL vs. 19.1±6.1 ng/mL, P<0.001). Winter-spring season (odds ratio [OR], 4.46; 95% confidence interval [CI], 3.45-5.77), older age group (OR, 1.60; 95% CI, 1.36-1.88), and overweight (OR, 2.21; 95% CI, 1.62-3.01) were independently related with VDD. The PTH levels were significantly higher in VDD group compared to vitamin D insufficiency and sufficiency group (P<0.001). In normal weight children, 25OHD (β=-0.007, P<0.001) and ionized calcium (β=-0.594, P=0.007) were independently related with PTH, however, these associations were not significant in overweight children. Conclusion VDD is very common in Korean children and its prevalence increases in winter-spring season, in overweight children and in older age groups. Further investigation on the vitamin D and PTH metabolism according to adiposity is required. PMID:25077091

  14. Baseline Body Composition in Prepubertal Short Stature Children with Severe and Moderate Growth Hormone Deficiency

    PubMed Central

    Klesiewicz, Marta

    2016-01-01

    Objective. To compare body composition parameters in short children with severe versus moderate and no growth hormone deficiency (GHD). Design and Method. 61 children (40 boys) were studied. Height SDS, BMI Z-score, waist/height ratio (W/HtR), and body composition parameters (BIA) as fat tissue (FAT%), fat-free mass (FFM%), predicted muscle mass (PMM%), and total body water (TBW%) were evaluated. GH secretion in the overnight profile and two stimulation tests and insulin-like growth factor 1 (IGF-1) level were measured. Results. Overall, in 16 (26%) moderate (7.0 > peak GH < 10 ng/mL) and in 11 (18%) severe (GH ≤ 7.0 ng/mL) GHD was diagnosed. In children with sGHD BMI Z-score, W/HtR and FAT% were significantly higher, while FFM%, PMM%, and TBW% were significantly lower versus mGHD and versus noGHD subgroups. No significant differences between mGHD and noGHD were found. There were no differences in height SDS and IGF-1 SDS between evaluated subgroups. Night GH peak level correlated significantly with FAT%, FFM%, PMM%, and TBW%, (p < 0.05) in the entire group. Conclusions. Only sGHD is associated with significant impairment of body composition. Body composition analysis may be a useful tool in distinguishing between its severe and moderate form of GHD. PMID:27656208

  15. 47,+(9q-) in unrelated three children with plasma growth hormone deficiency.

    PubMed

    Fujita, H; Shimazaki, M; Takeuchi, T; Hayakawa, Y; Oura, T

    1976-03-12

    Marker chromosomes carried by unrelated 3 cases were identified as a part of No. 9 chromosome through the analysis of the chromatid fine structure after trypsin-giemsa treatment. They showed characteristic features of that 9p trisomic syndrome which were described by Rethoré et al. (1973). In addition to those features, some clinical and laboratory findings on growth hormon deficiency were disclosed in this report.

  16. Growth hormone deficiency following radiation therapy of primary brain tumors in children.

    PubMed

    Kanev, P M; Lefebvre, J F; Mauseth, R S; Berger, M S

    1991-05-01

    The medical records of 123 patients treated for brain tumors at Children's Hospital and Medical Center, Seattle, Washington, between 1985 and 1987 were reviewed. The endocrinological complications of radiation therapy and the effectiveness of growth hormone (GH) replacement therapy were assessed. These were the first 2 years after synthetic GH became available. The disease pathology was confirmed at craniotomy or biopsy in 108 patients. Ninety-five children completed radiation therapy and 65 of these were alive at the time of review; these 65 children represent the study population. The most common tumor types were medulloblastoma, craniopharyngioma, and ependymoma. Endocrine evaluation was initiated with changes in the patients' growth velocity. Patient workup included skeletal x-ray films for determination of bone and analysis of thyroxin, thyroid-stimulating hormone, and somatomedin-C levels. Following 1-dopa and clonidine stimulation, provocative studies of GH levels were performed. Growth hormone failure and short stature were observed in 26 children, most commonly in the 2nd year after tumor treatment. Eight patients with GH failure were also hypothyroid. Hormone replacement therapy was initiated with recombinant GH, 0.05 mg/kg/day, and all children so treated showed an increase in height, with eight patients experiencing catch-up growth. There were no complications of therapy or tumor recurrence. Studies of baseline bone age and somatomedin-C levels on completion of radiation therapy are recommended. Comprehensive endocrine studies should follow changes in the patients' growth velocity. With early GH replacement, catch-up growth is possible and normal adult heights may be achieved.

  17. Imbalance in the blood antioxidant system in growth hormone-deficient children before and after 1 year of recombinant growth hormone therapy.

    PubMed

    Pankratova, Maria S; Baizhumanov, Adil A; Yusipovich, Alexander I; Faassen, Maria; Shiryaeva, Tatyana Yu; Peterkova, Valentina A; Kovalenko, Svetlana S; Kazakova, Tatiana A; Maksimov, Georgy V

    2015-01-01

    The aim of our study was to examine the effects of 12-month therapy with recombinant growth hormone (rGH) on the blood antioxidant system in children with growth hormone deficiency (GHD). Total antioxidant capacity (TAC) of plasma was measured by FRAP (ferric reducing antioxidant power or ferric reducing ability of plasma); activities of superoxide dismutase (SOD) and catalase (CAT) in erythrocytes were assessed; non-protein thiols (NT) and ceruloplasmin (CP) levels were also measured. These parameters were determined before and after 12 month of rGH treatment. Eleven treatment-naive prepubertal children with growth hormone deficiency were included in the study. Another 11 prepubertal children comprised a control group. Before rGH treatment, TAC of plasma and NT level in the control group were significantly lower (726 ± 196 vs. 525 ± 166 µmol/L, P = 0.0182 and 0.92 ± 0.18 vs. 0.70 ± 0.22 µmol/ml, P = 0.0319, before and after the therapy, respectively). The only parameter that significantly (19.6 ± 4.7 vs. 14.5 ± 3.4 Units/g Hb, P = 0.0396) exceeded the same in the control group after rGH therapy was SOD activity. However, none of the measured parameters of antioxidant system in GHD children, except for TAC (525 ± 166 vs. 658 ± 115 µmol/L, P = 0.0205), exhibited significant improvement toward the end of the 12-month treatment period, although non-significant changes in CAT activity and CP level were also observed. This work has demonstrated that some parameters of the blood antioxidant system are out of balance and even impaired in GHD children. A 12-month treatment with rGH resulted in a partial improvement of the antioxidant system.

  18. Effect of recombinant human growth hormone (rhGH) on hemoglobin concentration in children with idiopathic growth hormone deficiency-related anemia.

    PubMed

    Miniero, Roberto; Altomare, Federica; Rubino, Mario; Matarazzo, Patrizia; Montanari, Claudio; Petri, Antonella; Raiola, Giuseppe; Bona, Gianni

    2012-08-01

    Normocytic-normochromic anemia (NC/NC) has been attributed to impaired bone marrow erythropoiesis in growth hormone (GH)-deficient patients. Moreover, the GH/insulin-like growth factor-1 (IGF-1) axis has been implicated in erythropoiesis regulation. In this retrospective multicenter study, we evaluated the incidence of NC/NC anemia in 279 children (196 boys), median age 10.52 years, with isolated idiopathic GH deficiency, and the effect of recombinant human growth hormone (rhGH) therapy on hemoglobin levels. At 6-month intervals, we recorded the Hb standard deviation score (Hb-SDS), the IGF-1-SDS, weight, height, and pubertal stage. Forty-one boys and 7 girls had NC/NC anemia before starting substitutive therapy (-2.59 SD). The Hb-SDS was significantly increased (P<0.05) after 12 months of rhGH therapy. The effect of rhGH continued up to 48 months (-0.39 SD), at which point all children had normal hemoglobin values. In conclusion, rhGH therapy resulted in normal hemoglobin values in all children enrolled in the study. These data support the concept that the GH/IGF-1 axis promotes erythropoiesis in vivo.

  19. Prospective study of the development of growth hormone deficiency in children given cranial irradiation, and its relation to statural growth

    SciTech Connect

    Brauner, R.; Rappaport, R.; Prevot, C.; Czernichow, P.; Zucker, J.M.; Bataini, P.; Lemerle, J.; Sarrazin, D.; Guyda, H.J.

    1989-02-01

    Although GH deficiency (GHD) is the most frequent hormonal abnormality that occurs after cranial radiation, the natural course of this complication and its relationship to growth in children are not known. Therefore, we undertook a 2-yr prospective study of 16 children, aged 1.7-15 yr at the time of treatment, who received cranial (31-42 Gy (1 Gy = 100 rads)) and spinal radiation for medulloblastoma or ependymoma (group I). Their growth was compared to that of 11 children given similar doses of cranial radiation only (group II). The mean plasma GH response to arginine-insulin test (AITT) was 9.1 +/- 1.5 (+/- SE) micrograms/L in group I and 8.5 +/- 1.8 micrograms/L in group II (P = NS). After 2 yr, 16 of the 27 children had a peak plasma GH value below 8 micrograms/L after AITT, and 10 children had a peak response less than 5 micrograms/L. In addition, in group I, AITT and sleep-related GH secretion were compared; at the 2 yr follow-up only 3 of 13 children had discrepant results. At the 2 yr follow-up children treated by cranial and spinal radiation had a mean height of -1.46 +/- 0.40 SD below the normal mean. In contrast, the children given only cranial radiation had a mean height of -0.15 +/- 0.18 SD; P less than 0.02. Therefore, most of the growth retardation appeared to be due to lack of spinal growth. GHD is thus an early complication of cranial radiation in these children, and no significant growth retardation can be attributed to GHD during the first 2 yr. These data contribute to the organization of follow-up in irradiated children in order to decide when human GH treatment is necessary.

  20. Validating genetic markers of response to recombinant human growth hormone in children with growth hormone deficiency and Turner syndrome: the PREDICT validation study

    PubMed Central

    Stevens, Adam; Murray, Philip; Wojcik, Jerome; Raelson, John; Koledova, Ekaterina; Chatelain, Pierre

    2016-01-01

    Objective Single-nucleotide polymorphisms (SNPs) associated with the response to recombinant human growth hormone (r-hGH) have previously been identified in growth hormone deficiency (GHD) and Turner syndrome (TS) children in the PREDICT long-term follow-up (LTFU) study (Nbib699855). Here, we describe the PREDICT validation (VAL) study (Nbib1419249), which aimed to confirm these genetic associations. Design and methods Children with GHD (n = 293) or TS (n = 132) were recruited retrospectively from 29 sites in nine countries. All children had completed 1 year of r-hGH therapy. 48 SNPs previously identified as associated with first year growth response to r-hGH were genotyped. Regression analysis was used to assess the association between genotype and growth response using clinical/auxological variables as covariates. Further analysis was undertaken using random forest classification. Results The children were younger, and the growth response was higher in VAL study. Direct genotype analysis did not replicate what was found in the LTFU study. However, using exploratory regression models with covariates, a consistent relationship with growth response in both VAL and LTFU was shown for four genes – SOS1 and INPPL1 in GHD and ESR1 and PTPN1 in TS. The random forest analysis demonstrated that only clinical covariates were important in the prediction of growth response in mild GHD (>4 to <10 μg/L on GH stimulation test), however, in severe GHD (≤4 μg/L) several SNPs contributed (in IGF2, GRB10, FOS, IGFBP3 and GHRHR). Conclusions The PREDICT validation study supports, in an independent cohort, the association of four of 48 genetic markers with growth response to r-hGH treatment in both pre-pubertal GHD and TS children after controlling for clinical/auxological covariates. However, the contribution of these SNPs in a prediction model of first-year response is not sufficient for routine clinical use. PMID:27651465

  1. Effect of Growth Hormone Deficiency on Brain Structure, Motor Function and Cognition

    ERIC Educational Resources Information Center

    Webb, Emma A.; O'Reilly, Michelle A.; Clayden, Jonathan D.; Seunarine, Kiran K.; Chong, Wui K.; Dale, Naomi; Salt, Alison; Clark, Chris A.; Dattani, Mehul T.

    2012-01-01

    The growth hormone-insulin-like growth factor-1 axis plays a role in normal brain growth but little is known of the effect of growth hormone deficiency on brain structure. Children with isolated growth hormone deficiency (peak growth hormone less than 6.7 [micro]g/l) and idiopathic short stature (peak growth hormone greater than 10 [micro]g/l)…

  2. The Impact of Growth Hormone Therapy on the Apoptosis Assessment in CD34+ Hematopoietic Cells from Children with Growth Hormone Deficiency.

    PubMed

    Kawa, Miłosz Piotr; Stecewicz, Iwona; Piecyk, Katarzyna; Paczkowska, Edyta; Rogińska, Dorota; Sobuś, Anna; Łuczkowska, Karolina; Pius-Sadowska, Ewa; Gawrych, Elżbieta; Petriczko, Elżbieta; Walczak, Mieczysław; Machaliński, Bogusław

    2017-01-07

    Growth hormone (GH) modulates hematopoietic cell homeostasis and is associated with apoptosis control, but with limited mechanistic insights. Aim of the study was to determine whether GH therapeutic supplementation (GH-TS) could affect apoptosis of CD34+ cells enriched in hematopoietic progenitor cells of GH deficient (GHD) children. CD34+ cells from peripheral blood of 40 GHD children were collected before and in 3rd and 6th month of GH-TS and compared to 60 controls adjusted for bone age, sex, and pubertal development. Next, apoptosis assessment via different molecular techniques was performed. Finally, to comprehensively characterize apoptosis process, global gene expression profile was determined using genome-wide RNA microarray technology. Results showed that GH-TS significantly reduced spontaneous apoptosis in CD34+ cells (p < 0.01) and results obtained using different methods to detect early and late apoptosis in analyzed cells population were consistent. GH-TS was also associated with significant downregulation of several members of TNF-alpha superfamily and other genes associated with apoptosis and stress response. Moreover, the significant overexpression of cyto-protective and cell cycle-associated genes was detected. These findings suggest that recombinant human GH has a direct anti-apoptotic activity in hematopoietic CD34+ cells derived from GHD subjects in course of GH-TS.

  3. The Impact of Growth Hormone Therapy on the Apoptosis Assessment in CD34+ Hematopoietic Cells from Children with Growth Hormone Deficiency

    PubMed Central

    Kawa, Miłosz Piotr; Stecewicz, Iwona; Piecyk, Katarzyna; Paczkowska, Edyta; Rogińska, Dorota; Sobuś, Anna; Łuczkowska, Karolina; Pius-Sadowska, Ewa; Gawrych, Elżbieta; Petriczko, Elżbieta; Walczak, Mieczysław; Machaliński, Bogusław

    2017-01-01

    Growth hormone (GH) modulates hematopoietic cell homeostasis and is associated with apoptosis control, but with limited mechanistic insights. Aim of the study was to determine whether GH therapeutic supplementation (GH-TS) could affect apoptosis of CD34+ cells enriched in hematopoietic progenitor cells of GH deficient (GHD) children. CD34+ cells from peripheral blood of 40 GHD children were collected before and in 3rd and 6th month of GH-TS and compared to 60 controls adjusted for bone age, sex, and pubertal development. Next, apoptosis assessment via different molecular techniques was performed. Finally, to comprehensively characterize apoptosis process, global gene expression profile was determined using genome-wide RNA microarray technology. Results showed that GH-TS significantly reduced spontaneous apoptosis in CD34+ cells (p < 0.01) and results obtained using different methods to detect early and late apoptosis in analyzed cells population were consistent. GH-TS was also associated with significant downregulation of several members of TNF-alpha superfamily and other genes associated with apoptosis and stress response. Moreover, the significant overexpression of cyto-protective and cell cycle-associated genes was detected. These findings suggest that recombinant human GH has a direct anti-apoptotic activity in hematopoietic CD34+ cells derived from GHD subjects in course of GH-TS. PMID:28067847

  4. [Influence of replacement growth hormone therapy (hGH) on pituitary-thyroid and pituitary-adrenal systems in prepubertal children with GH deficiency].

    PubMed

    Vyshnevs'ka, O A; Bol'shova, O V

    2013-06-01

    Today, the most pathogenic therapy of GH deficiency is hGH replacement therapy. Replacement hGH therapy a highly effective method of growth correction in children with GH deficiency, but further investigations are necessary for timely detection of disturbances of other organs and systems. The authors reported that hGH therapy supressed thyroid and adrenal functions. Besides, most patients with GH deficiency have multiple defficiency of pituitary hormones (both TSH and ACTH), so hGH therapy can enhances hypothyroidism and hypoadrenalism. In the Department of Pediatric Endocrinology of the Institute of Endocrinology and Metabolism a great experience was accumulated in the treatment of GH deficiency children and in the study of the efficacy and safety of this treatment.

  5. Hypoglycemia associated with clonidine testing for growth hormone deficiency.

    PubMed

    Huang, C; Banerjee, K; Sochett, E; Perlman, K; Wherrett, D; Daneman, D

    2001-08-01

    We have observed 4 cases of hypoglycemia associated with clonidine stimulation of growth hormone secretion; only one patient had growth hormone deficiency. Significant drowsiness after administration of clonidine may prolong the period of fasting in these children and mask early signs and symptoms, leading to severe hypoglycemia.

  6. Validation of Prediction Models for Near Adult Height in Children with Idiopathic Growth Hormone Deficiency Treated with Growth Hormone: A Belgian Registry Study

    PubMed Central

    Straetemans, Saartje; De Schepper, Jean; Thomas, Muriel; Verlinde, Franciska; Rooman, Raoul

    2016-01-01

    Background/Aim To validate prediction models for near final adult height (nFAH) by Ranke et al. [Horm Res Paediatr 2013;79:51-67]. Methods Height data of 127 (82 male) idiopathic growth hormone (GH)-deficient children, treated with GH until nFAH, were retrieved from the database of the Belgian Society for Pediatric Endocrinology and Diabetology (BESPEED). nFAH was predicted after first-year GH treatment, applying prediction models by Ranke et al. Bland-Altman plots and Clarke error grid analyses were performed to assess clinical significance of the differences between observed and predicted nFAH. Results In males, the predicted nFAH was higher than the observed nFAH (difference: 0.2 ± 0.7 SD; p < 0.01). In females, there was no significant difference. Bland-Altman analyses showed that the means of the differences between observed and predicted nFAH were close but not equal to zero, with overprediction for smaller heights and underprediction for taller heights. Clarke error grid analysis: in males, 59-61% of the predicted nFAH were within 0.5 SDS and 88% within 1.0 SDS from the observed nFAH; in females, 40-44% of the predicted nFAH were within 0.5 SDS and 76-78% within 1.0 SDS from the observed nFAH. Conclusion Ranke's models accurately predicted nFAH in females and overpredicted nFAH in males by about 1.5 cm. In most individuals, the predicted nFAH was within 1 SDS of observed nFAH. These models can be of help in giving realistic expectations of adult height. PMID:27598343

  7. Growth Hormone Deficiency in Adults

    MedlinePlus

    ... y Cuidadores Hormones and Health Journey Through the Endocrine System Endocrine Disrupting Chemicals (EDCs) Endocrine Glands and Types ... Clinical Trials Hormones and Health Journey Through the Endocrine System Endocrine Disrupting Chemicals (EDCs) Endocrine Glands and Types ...

  8. Genetics Home Reference: combined pituitary hormone deficiency

    MedlinePlus

    ... People with combined pituitary hormone deficiency may have hypothyroidism, which is underactivity of the butterfly-shaped thyroid gland in the lower neck. Hypothyroidism can cause many symptoms, including weight gain and ...

  9. Growth Hormone Deficiency, Brain Development, and Intelligence

    ERIC Educational Resources Information Center

    Meyer-Bahlburg, Heino F. L.; And Others

    1978-01-01

    Available from: American Medical Association, 535 N. Dearborn Street, Chicago, Illinois 60610. In order to determine what effect, if any, growth hormone (GH) has on human brain development, 29 patients (mean age 11.7 years) with GH deficiency were selected according to the following criteria: no evidence of reversible GH deficiency, onset of…

  10. Hypopituitarism: growth hormone and corticotropin deficiency.

    PubMed

    Capatina, Cristina; Wass, John A H

    2015-03-01

    This article presents an overview of adult growth hormone deficiency (AGHD) and corticotropin deficiency (central adrenal failure, CAI). Both conditions can result from various ailments affecting the hypothalamus or pituitary gland (most frequently a tumor in the area or its treatment). Clinical manifestations are subtle in AGHD but potentially life-threatening in CAI. The diagnosis needs dynamic testing in most cases. Treatment of AGHD is recommended in patients with documented severe deficiency, and treatment of CAI is mandatory in all cases. Despite significant progress in replacement hormonal therapy, more physiologic treatments and more reliable indicators of treatment adequacy are still needed.

  11. Novel mutations associated with combined pituitary hormone deficiency.

    PubMed

    Romero, Christopher J; Pine-Twaddell, Elyse; Radovick, Sally

    2011-06-01

    The pituitary gland produces hormones that play important roles in both the development and the homeostasis of the body. A deficiency of two or several of these pituitary hormones, known as combined pituitary hormone deficiency, may present in infants or children due to an unknown etiology and is considered congenital or idiopathic. Advancements in our understanding of pituitary development have provided a genetic basis to explain the pathophysiological basis of pituitary hormone disease. Nevertheless, there are several challenges to the precise characterization of abnormal genotypes; these exist secondary to the complexities of several of the hypothalamic/pituitary developmental factors and signals, which ultimately integrate in a temporal and spatial dependent manner to produce a mature gland. Furthermore, the clinical presentation of pituitary hormone disease may be dynamic as subsequent hormone deficiencies may develop over time. The characterization of patients with mutations in genes responsible for pituitary development provides an opportunity to discover potential novel mechanisms responsible for pituitary pathophysiology. The focus of this review is to report the most recent mutations in genes responsible for pituitary development in patients with hypopituitarism and emphasize the importance to physicians and researchers for characterizing these patients. Continuing efforts toward understanding the molecular basis of pituitary development as well as genetic screening of patients with pituitary disease will offer new insights into both diagnostic and potential therapeutic options that will decrease the morbidity and mortality in patients with hypopituitarism.

  12. Language deficiency in children.

    PubMed

    Morehead, D M; Morehead, K E; Morehead, W A

    1980-01-01

    Research in cognition and language has provided useful constructs which suggests that specific deficits underlie language deficiencies in children. In addition, this research has provided procedures that the determine what a child knows about language at a particular level of development and has established a sequence of linguistic development that maps the specific content and structure of training programs. Two new areas of research offer additional approaches to assessment and remediation. One approach focuses on the actual principles and strategies that normal children use to learn language, making it possible to determine which methods are most efficient. The second research approach looks at the contextual conditions adults and children provide the first language learner. Preliminary work suggests that the natural conditions found universally in first language learning may be the best indicators of how to proceed with language-deficient children.

  13. Anthropometric measurements in patients with growth hormone deficiency before treatment with human growth hormone.

    PubMed

    Zachmann, M; Fernandez, F; Tassinari, D; Thakker, R; Prader, A

    1980-05-01

    In 74 children (52 males, 22 females) with growth hormone (GH) deficiency (30 cases with isolated GH-deficiency, two of them familial; 4 familial and one isolated case with tendency for formation of antibodies against hGH; 29 with other pituitary hormone defects; 10 craniopharyngiomas), various anthropometric measurements were analyzed before treatment with hGH. In all groups, standing height, sitting height, and subischial leg height were equally retarded, and bihumeral width was more retarded than biiliac width; the head was relatively large; fat tissue was increased with subscapular skinfolds being greater than triceps skinfolds, indicating relative obestiy of the trunk; muscle and/or bone mass was reduced. In isolated GH-deficiency, head shape was slightly scaphoid; in combined defects, it was round, and in craniopharyngioma cases, it was brachycephalic. It is concluded that antrhopometric measurements may help in differentiating the type of GH-deficiency.

  14. Effects of recombinant growth hormone (GH) replacement and psychomotor and cognitive stimulation in the neurodevelopment of GH-deficient (GHD) children with cerebral palsy: a pilot study.

    PubMed

    Devesa, Jesús; Alonso, Begoña; Casteleiro, Nerea; Couto, Paula; Castañón, Beatriz; Zas, Eva; Reimunde, Pedro

    2011-01-01

    Cerebral palsy (CP) is the main cause of physical disability in childhood and is an important health issue that has a strong socioeconomic impact. There is no effective treatment for CP and therapeutic approaches report only partial benefits for affected people. In this study we assessed the effects of growth hormone (GH) treatment combined with psychomotor and cognitive stimulation in the neurodevelopment of children with CP and GH deficiency (GHD). The study was carried out in 11 patients (7 boys and 4 girls; 4.12 ± 1.31 years) with GHD and CP who were treated with recombinant GH (rGH) and psychomotor and cognitive stimulation during 2 months. Battelle Developmental Inventory Screening Test (BDIST) was performed 2 months before commencing GH treatment, just before commencing GH administration, and after 2 months of combined treatment involving GH and cognitive stimulation. Psychomotor and cognitive status did not change during the period in which only cognitive stimulation was performed; however, significant improvements in personal and social skills, adaptive behavior, gross motor skills and total psychomotor abilities, receptive and total communication, cognitive skills and in the total score of the test (P < 0.01), and in fine motor skills and expressive communication (P < 0.02) were observed after the combined treatment period. Therefore, GH replacement together with psychomotor and cognitive stimulation seem to be useful for the appropriate neurodevelopment of children with GHD and CP.

  15. Usability and Tolerability of the Norditropin NordiFlex® Injection Device in Children Never Previously Treated With Growth Hormone

    ClinicalTrials.gov

    2014-06-23

    Growth Hormone Disorder; Growth Hormone Deficiency in Children; Genetic Disorder; Turner Syndrome; Foetal Growth Problem; Small for Gestational Age; Chronic Kidney Disease; Chronic Renal Insufficiency; Delivery Systems

  16. Climacteric in untreated isolated growth hormone deficiency

    PubMed Central

    Menezes, Menilson; Salvatori, Roberto; Oliveira, Carla R.P.; Pereira, Rossana M.C.; Souza, Anita H.O.; Nobrega, Luciana M.A.; Cruz, Edla do A.C.; Menezes, Marcos; Alves, Érica O.; Aguiar-Oliveira, Manuel H.

    2008-01-01

    Objective To study the time, intensity of symptoms, hormonal profile, and related morbidity of climacteric in women with untreated isolated growth hormone (GH) deficiency (IGHD). Design Women belonging to a large Brazilian kindred with IGHD due to a homozygous mutation in the GH-releasing hormone receptor gene were studied. None of them had ever received GH replacement therapy. A two-step protocol was performed. In the first case-control experiment, aimed to determine the age at climacteric, we compared eight women with IGHD and 32 normal women between 37 and 55 years of age. In the second cross-sectional experiment, aimed to determine the severity of climacteric symptoms, seven women with IGHD (aged 47-65 y) were compared with 13 controls (aged 44-65 y). The Kupperman Index scores, serum follicle-stimulating hormone, luteinizing hormone, prolactin, and estradiol levels were determined, and pelvic and mammary ultrasonography, mammography, and colpocytology were performed. Results The number of women with follicle-stimulating hormone above 20 mIU/mL was higher in women with IGHD than controls. Kupperman’s Index was not different between the two groups. Menarche had been delayed and parity was lower in women with IGHD. Hormonal profile was similar, but prolactin was lower in women with IGHD. Uterine volume was smaller in women with IGHD, and endometrial thickness and ovarian volume were similar in the two groups. No difference in breast images or in colpocytology was observed between the two groups. Conclusions Menarche was delayed and the beginning of climacteric is anticipated in untreated lifetime IGHD, but menopausal symptoms and hormonal profile resemble the normal climacteric. PMID:18223507

  17. Effect of growth hormone deficiency on brain structure, motor function and cognition.

    PubMed

    Webb, Emma A; O'Reilly, Michelle A; Clayden, Jonathan D; Seunarine, Kiran K; Chong, Wui K; Dale, Naomi; Salt, Alison; Clark, Chris A; Dattani, Mehul T

    2012-01-01

    The growth hormone-insulin-like growth factor-1 axis plays a role in normal brain growth but little is known of the effect of growth hormone deficiency on brain structure. Children with isolated growth hormone deficiency (peak growth hormone <6.7 µg/l) and idiopathic short stature (peak growth hormone >10 µg/l) underwent cognitive assessment, diffusion tensor imaging and volumetric magnetic resonance imaging prior to commencing growth hormone treatment. Total brain, corpus callosal, hippocampal, thalamic and basal ganglia volumes were determined using Freesurfer. Fractional anisotropy (a marker of white matter structural integrity) images were aligned and tract-based spatial statistics performed. Fifteen children (mean 8.8 years of age) with isolated growth hormone deficiency [peak growth hormone <6.7 µg/l (mean 3.5 µg/l)] and 14 controls (mean 8.4 years of age) with idiopathic short stature [peak growth hormone >10 µg/l (mean 15 µg/l) and normal growth rate] were recruited. Compared with controls, children with isolated growth hormone deficiency had lower Full-Scale IQ (P < 0.01), Verbal Comprehension Index (P < 0.01), Processing Speed Index (P < 0.05) and Movement-Assessment Battery for Children (P < 0.008) scores. Verbal Comprehension Index scores correlated significantly with insulin-like growth factor-1 (P < 0.03) and insulin-like growth factor binding protein-3 (P < 0.02) standard deviation scores in isolated growth hormone deficiency. The splenium of the corpus callosum, left globus pallidum, thalamus and hippocampus (P < 0.01) were significantly smaller; and corticospinal tract (bilaterally; P < 0.045, P < 0.05) and corpus callosum (P < 0.05) fractional anisotropy were significantly lower in the isolated growth hormone deficiency group. Basal ganglia volumes and bilateral corticospinal tract fractional anisotropy correlated significantly with Movement-Assessment Battery for Children scores, and

  18. Growth hormone deficiency in treated acromegaly.

    PubMed

    Mazziotti, Gherardo; Marzullo, Paolo; Doga, Mauro; Aimaretti, Gianluca; Giustina, Andrea

    2015-01-01

    Growth hormone deficiency (GHD) of the adult is characterized by reduced quality of life (QoL) and physical fitness, skeletal fragility, and increased weight and cardiovascular risk. Hypopituitarism may develop in patients after definitive treatment of acromegaly, but an exact prevalence of GHD in this population is still uncertain owing to limited awareness and the scarce and conflicting data available on this topic. Because acromegaly and GHD may yield adverse consequences on similar target systems, the final outcomes of some complications of acromegaly may be further affected by the occurrence of GHD. However, it is still largely unknown whether patients with post-acromegaly GHD may benefit from GH replacement. We review the diagnostic, clinical, and therapeutic aspects of GHD in adult patients treated for acromegaly.

  19. Psychomotor retardation in a girl with complete growth hormone deficiency.

    PubMed

    Dayal, Devi; Malhi, Prabhjot; Kumar Bhalla, Anil; Sachdeva, Naresh; Kumar, Rakesh

    2013-01-01

    Infants with complete growth hormone deficiency may suffer from psychomotor retardation in addition to severe growth failure. Without replacement therapy, they may have a compromised intellectual potential manifesting as learning disabilities and attention-deficit disorders in later life. In this communication, we discuss an infant who showed improvement in physical growth after growth hormone therapy but her psychomotor skills did not improve probably due to late start of treatment. There is a need to start growth hormone therapy as early as possible in infants with complete growth hormone deficiency to avoid adverse effects on psychomotor and brain development.

  20. Growth hormone deficiency and pituitary malformation in a recurrent Cat-Eye syndrome: a family report.

    PubMed

    Jedraszak, Guillaume; Braun, Karine; Receveur, Aline; Decamp, Matthieu; Andrieux, Joris; Rabbind Singh, Amrathlal; Copin, Henri; Bremond-Gignac, Dominique; Mathieu, Michèle; Rochette, Jacques; Morin, Gilles

    2015-10-01

    Growth hormone deficiency affects roughly between one in 3000 and one in 4000 children with most instances of growth hormone deficiency being idiopathic. Growth hormone deficiency can also be associated with genetic diseases or chromosome abnormalities. Association of growth hormone deficiency together with hypothalamic-pituitary axis malformation and Cat-Eye syndrome is a very rare condition. We report a family with two brothers presenting with growth delay due to a growth hormone deficiency associated with a polymalformation syndrome. They both displayed pre-auricular pits and tags, imperforate anus and Duane retraction syndrome. Both parents and a third unaffected son displayed normal growth pattern. Cerebral MRI showed a hypothalamic-pituitary axis malformation in the two affected brothers. Cytogenetic studies revealed a type I small supernumerary marker chromosome derived from chromosome 22 resulting in a tetrasomy 22pter-22q11.21 characteristic of the Cat-Eye syndrome. The small supernumerary marker chromosome was present in the two affected sons and the mother in a mosaic state. Patients with short stature due to growth hormone deficiency should be evaluated for chromosomal abnormality. Family study should not be underestimated.

  1. Growth hormone deficiency and diabetes insipidus as a complication of endoscopic third ventriculostomy.

    PubMed

    Tafuri, Kimberly S; Wilson, Thomas A

    2012-12-01

    Endoscopic third ventriculostomy (ETV) has become the procedure of choice for the treatment of obstructive hydrocephalus in children and adults. Endocrinological complications of ETV in children are rare. Diabetes insipidus (DI) is the most common and accounts for only 0.5% of complications from ETV. The majority of documented cases are transient. To date, there are no documented cases of multiple pituitary hormone deficiencies. We present here a 6-year-old girl with growth hormone deficiency and permanent DI which developed as a complication of ETV. This patient is unique in both demonstrating multiple pituitary hormone deficiencies and the classical triphasic response of DI after ETV. We postulate that these complications were caused by compression of the pituitary stalk and hypothalamic injury during the procedure. We compare our case presentation to experimental studies conducted in rats.

  2. Exceptional Association Between Klinefelter Syndrome and Growth Hormone Deficiency.

    PubMed

    Doubi, Sana; Amrani, Zoubida; Ouahabi, Hanan El; Boujraf, Saïd; Ajdi, Farida

    2015-01-01

    Klinefelter syndrome (KS) is characterized in adults by the combination of a tall stature, small testes, gynecomastia, and azoospermia. This case is described in a North African population of the Mediterranean region of North Africa. We report the case of a male 16 years old, of Arab ethnic origin, and diagnosed with this syndrome, who had a small height in relation to a growth hormone (GH) deficiency and a history of absence seizures (generalized myoclonic epilepsy). The patient's size was <-2.8 standard deviation (SD) with weight <-3 SD. GH deficiency was isolated and confirmed by two dynamic tests (insulin - hypoglycemia tolerance test and clonidine) with normal hypothalamic magnetic resonance imaging (MRI). GH supplementation using recombinant GH was advocated, while gonadotropin treatment was deferred. Small size in children or adolescents should not eliminate the diagnosis of Klinefelter syndrome - on the contrary, the presence of any associated sign (brain maturation, delay in puberty, aggressiveness) should encourage one to request a karyotype for the diagnosis and appropriate care of any case of KS that can be associated with GH deficiency, or which is in a variant form (isochromosome Xq, 49,XXXXY).

  3. Cortistatin vaccination--a solution to growth hormone deficiency.

    PubMed

    Moaeen-ud-Din, M; Malik, Nosheen; Guo, Yang Li; Ali, Ahmad; Babar, Masroor Ellahi

    2009-12-01

    Cortistatin and somatostatin are neuropeptides which have inhibitory effects on growth hormone through common five receptors. Although, both have inhibitory effects but, only cortistatin has direct inhibitory effects on growth hormone secretagogue and is more potent inhibitor of growth hormone than somatostatin. This control of growth hormone can be manipulated through immunoneutralization of cortistatin through cortistatin DNA vaccine rather than antibodies application. A DNA vaccine of cortistatin can be produced using recombinant DNA technology in a eukaryotic expression system and will serve as a tool not to only alleviate the growth hormone deficiency problems in human but, can also be used to improve growth rate in farm animals.

  4. IODIDE DEFICIENCY, THYROID HORMONES, AND NEURODEVELOPMENT

    EPA Science Inventory

    ABSTRACT BODY: Iodide is an essential nutrient for thyroid hormone synthesis. Severe iodide insufficiency during early development is associated with cognitive deficits. Environmental contaminants can perturb the thyroid axis and this perturbation may be more acute under conditio...

  5. A Critical Appraisal of Growth Hormone Therapy in Growth Hormone Deficiency and Turner Syndrome Patients in Turkey

    PubMed Central

    Yavaş Abalı, Zehra; Darendeliler, Feyza; Neyzi, Olcay

    2016-01-01

    Early detection of abnormal growth, identification of the underlying cause, and appropriate treatment of the medical condition is an important issue for children with short stature. Growth hormone (GH) therapy is widely used in GH-deficient children and also in non-GH-deficient short stature cases who have findings conforming to certain indications. Efficacy of GH therapy has been shown in a multitude of short- and long-term studies. Age at onset of GH therapy is the most important factor for a successful treatment outcome. Optimal dosing is also essential. The aim of this review was to focus on challenges in the early diagnosis and appropriate management of short stature due to GH deficiency (GHD) and Turner syndrome. These are the most frequent two indications for GH therapy in Turkey approved by the Ministry of Health for coverage by the national insurance system. PMID:27354120

  6. Long-term effects of growth hormone (GH) replacement therapy on hematopoiesis in a large cohort of children with GH deficiency.

    PubMed

    Esposito, Andrea; Capalbo, Donatella; De Martino, Lucia; Rezzuto, Martina; Di Mase, Raffaella; Pignata, Claudio; Salerno, Mariacarolina

    2016-07-01

    The aim of our prospective case-control study was to evaluate long-term effects of GH replacement therapy on erythrocytes parameters, leukocytes, and platelets numbers in a large cohort of children with isolated GH deficiency (GHD). Hemoglobin (Hb) concentration, hematocrit (Hct), mean corpuscular volume, mean corpuscular hemoglobin, red cell distribution width, number of erythrocytes, leukocytes, neutrophils, lymphocytes, monocytes and platelets, ferritin, and C-reactive protein were evaluated in 85 children with isolated GHD (10.20 ± 3.50 years) before and annually during the first 5 years of GH replacement therapy and in 85 healthy children age and sex comparable to patients during 5 years of follow-up. Compared with controls, GHD children at study entry showed lower Hb (-1.18 ± 0.87 vs. -0.40 ± 0.90 SDS, p < 0.0001), red cells number (-0.24 ± 0.81 vs. 0.25 ± 1.14 SDS, p < 0.0001), and Hct (-1.18 ± 0.86 vs. -0.68 ± 0.99 SDS, p < 0.0001). Twelve GHD patients (14 %) showed a normocytic anemia. GH therapy was associated with a significant increase in Hb, Hct, and red cells number which became all comparable to controls within the first 2 years of treatment. Moreover, hemoglobin levels normalized in all anemic GHD patients after 5 years of therapy. No difference between patients and controls was found in leukocytes and platelets numbers neither at baseline nor during the study. GHD in childhood is associated with an impairment of erythropoiesis which causes a normocytic anemia in a considerable percentage of patients. GH replacement therapy exerts a beneficial effect leading to a significant increase of erythrocytes parameters and recovery from anemia. Neither GHD nor GH replacement treatment exerts effects on leukocytes or platelets numbers.

  7. Growth hormone deficiency: new approaches to the diagnosis.

    PubMed

    Binder, Gerhard

    2011-09-01

    International consensus statements based on expert experience recommended guide lines how to diagnose GHD. Most recommendations reached only a low level of evidence. Cut-offs for GH were central part of these recommendations, their definition however was arbitrary. Evidence based cut-offs are needed. Using newborn screening cards from healthy and affected newborns, the GH cut-off to detect severe congenital GHD was reassessed and redefined. A GH cut-off level of 7 microg/L confirmed the diagnosis of severe GHD with 100% sensitivity and 98% specificity on the basis of our assay method, if clinical evidence was present. The previous cut-off of 20 microg/L cited in the international consensus statements was based on old GH assays methods not used anymore. For the calculation of an non-arbitrary GH cut-off for biochemical testing in children, we defined an auxological gold standard for GH deficiency: non-familial short stature due to catch-down growth during the childhood phase of growth in combination with an effective catch-up growth in response to low-dose GH therapy, after exclusion of alternative growth disorders and other potential confounders of growth velocity (true positives). Reference cohorts were normally growing children with Turner syndrome or SGA short stature having the same age (true negatives). Using our in-house GH RIA, highest diagnostic accuracy was provided at a peak GH cutoff during spontaneous secretion at night of 7.3 microg/L (sensitivity, 96.8%; specificity, 82.4%; AUC = 0.93). For arginine, cut-off with the highest number of true test results was 6.6 microg/L (sensitivity, 84.3%; specificity, 75.5%; AUC = 0.83). Importantly, children diagnosed GHD in the past with GH test values above the new cut-offs showed a lower response to GH. In conclusion, by use of retrospective and prospective cohort studies evidence-based cut-offs for GH levels measured in newborns and children can be calculated. By use of these cut-offs, tests can be improved

  8. Leptin deficiency in maltreated children.

    PubMed

    Danese, A; Dove, R; Belsky, D W; Henchy, J; Williams, B; Ambler, A; Arseneault, L

    2014-09-23

    Consistent with findings from experimental research in nonhuman primates exposed to early-life stress, children exposed to maltreatment are at high risk of detrimental physical health conditions, such as obesity and systemic inflammation. Because leptin is a key molecule involved in the regulation of both energy balance and immunity, we investigated abnormalities in leptin physiology among maltreated children. We measured leptin, body mass index and C-reactive protein in 170 12-year-old children members of the Environmental-Risk Longitudinal Twin Study, for whom we had prospectively-collected information on maltreatment exposure. We found that maltreated children exhibited blunted elevation in leptin levels in relation to increasing levels of physiological stimuli, adiposity and inflammation, compared with a group of non-maltreated children matched for gender, zygosity and socioeconomic status. These findings were also independent of key potential artifacts and confounders, such as time of day at sample collection, history of food insecurity, pubertal maturation and depressive symptoms. Furthermore, using birth weight as a proxy measure for leptin, we found that physiological abnormalities were presumably not present at birth in children who went on to be maltreated but only emerged over the course of childhood, after maltreatment exposure. Leptin deficiency may contribute to onset, persistence and progression of physical health problems in maltreated children.

  9. Combination growth hormone and gonadotropin releasing hormone analog therapy in 11beta-hydroxylase deficiency.

    PubMed

    Bajpai, Anurag; Kabra, Madhulika; Menon, P S N

    2006-06-01

    Diagnosis of 11beta-hydroxylase deficiency was made in a boy at the age of 2 1/2 years on the basis of peripheral precocious puberty, growth acceleration (height standard deviation score +4.4) with advanced skeletal maturation (bone age 8.4 years) and elevated deoxycortisol levels. Glucocorticoid supplementation led to normalization of blood pressure but was associated with progression to central precocious puberty and increase in bone age resulting in decrease in predicted adult height to 133.7 cm (target height 163 cm). The child was started on GnRH analog (triptorelin 3.75 mg every 28 days), which led to improvement in predicted adult height by 3.1 cm over 15 months. Addition of growth hormone (0.1 IU/kg/day) resulted in improvement in predicted adult height (151 cm) and height deficit (12 cm) over the next 3.6 years. Final height (151 cm) exceeded predicted height at the initiation of GnRH analog treatment by 17.3 cm. This report suggests that combination GH and GnRH analog treatment may be useful in improving height outcome in children with 11beta-hydroxylase deficiency and compromised final height.

  10. Mexico City normal weight children exposed to high concentrations of ambient PM2.5 show high blood leptin and endothelin-1, vitamin D deficiency, and food reward hormone dysregulation versus low pollution controls. Relevance for obesity and Alzheimer disease.

    PubMed

    Calderón-Garcidueñas, Lilian; Franco-Lira, Maricela; D'Angiulli, Amedeo; Rodríguez-Díaz, Joel; Blaurock-Busch, Eleonore; Busch, Yvette; Chao, Chih-kai; Thompson, Charles; Mukherjee, Partha S; Torres-Jardón, Ricardo; Perry, George

    2015-07-01

    Millions of Mexico, US and across the world children are overweight and obese. Exposure to fossil-fuel combustion sources increases the risk for obesity and diabetes, while long-term exposure to fine particulate matter (PM2.5) and ozone (O3) above US EPA standards is associated with increased risk of Alzheimer's disease (AD). Mexico City Metropolitan Area children are chronically exposed to PM2.5 and O3 concentrations above the standards and exhibit systemic, brain and intrathecal inflammation, cognitive deficits, and Alzheimer disease neuropathology. We investigated adipokines, food reward hormones, endothelial dysfunction, vitamin D and apolipoprotein E (APOE) relationships in 80 healthy, normal weight 11.1±3.2 year olds matched by age, gender, BMI and SES, low (n: 26) versus high (n:54) PM2.5 exposures. Mexico City children had higher leptin and endothelin-1 (p<0.01 and p<0.000), and decreases in glucagon-like peptide-1 (GLP 1), ghrelin, and glucagon (<0.02) versus controls. BMI and leptin relationships were significantly different in low versus high PM2.5 exposed children. Mexico City APOE 4 versus 3 children had higher glucose (p=0.009). Serum 25-hydroxyvitamin D<30 ng/mL was documented in 87% of Mexico City children. Leptin is strongly positively associated to PM 2.5 cumulative exposures. Residing in a high PM2.5 and O3 environment is associated with 12h fasting hyperleptinemia, altered appetite-regulating peptides, vitamin D deficiency, and increases in ET-1 in clinically healthy children. These changes could signal the future trajectory of urban children towards the development of insulin resistance, obesity, type II diabetes, premature cardiovascular disease, addiction-like behavior, cognitive impairment and Alzheimer's disease. Increased efforts should be made to decrease pediatric PM2.5 exposures, to deliver health interventions prior to the development of obesity and to identify and mitigate environmental factors influencing obesity and Alzheimer

  11. Concomitant occurrence of Turner syndrome and growth hormone deficiency.

    PubMed

    Yu, Jung; Shin, Ha Young; Lee, Chong Guk; Kim, Jae Hyun

    2016-11-01

    Turner syndrome (TS) is a genetic disorder in phenotypic females that has characteristic physical features and presents as partial or complete absence of the second sex chromosome. Growth hormone deficiency (GHD) is a condition caused by insufficient release of growth hormone from the pituitary gland. The concomitant occurrence of TS and GHD is rare and has not yet been reported in Korea. Here we report 2 cases of TS and GHD. In case 1, GHD was initially diagnosed. Karyotyping was performed because of the presence of the typical phenotype and poor response to growth hormone therapy, which revealed 45,X/45,X+mar. The patient showed increased growth velocity after the growth hormone dose was increased. In case 2, a growth hormone provocation test and chromosomal analysis were performed simultaneously because of decreased growth velocity and the typical TS phenotype, which showed GHD and a mosaic karyotype of 45,X/46,XX. The patient showed spontaneous pubertal development. In female patients with short stature, it is important to perform a throughout physical examination and test for hormonal and chromosomal abnormalities because diagnostic accuracy is important for treatment and prognosis.

  12. Concomitant occurrence of Turner syndrome and growth hormone deficiency

    PubMed Central

    Yu, Jung; Shin, Ha Young; Lee, Chong Guk

    2016-01-01

    Turner syndrome (TS) is a genetic disorder in phenotypic females that has characteristic physical features and presents as partial or complete absence of the second sex chromosome. Growth hormone deficiency (GHD) is a condition caused by insufficient release of growth hormone from the pituitary gland. The concomitant occurrence of TS and GHD is rare and has not yet been reported in Korea. Here we report 2 cases of TS and GHD. In case 1, GHD was initially diagnosed. Karyotyping was performed because of the presence of the typical phenotype and poor response to growth hormone therapy, which revealed 45,X/45,X+mar. The patient showed increased growth velocity after the growth hormone dose was increased. In case 2, a growth hormone provocation test and chromosomal analysis were performed simultaneously because of decreased growth velocity and the typical TS phenotype, which showed GHD and a mosaic karyotype of 45,X/46,XX. The patient showed spontaneous pubertal development. In female patients with short stature, it is important to perform a throughout physical examination and test for hormonal and chromosomal abnormalities because diagnostic accuracy is important for treatment and prognosis. PMID:28018463

  13. Isolated growth hormone deficiency type 2: from gene to therapy.

    PubMed

    Miletta, Maria Consolata; Lochmatter, Didier; Pektovic, Vibor; Mullis, Primus-E

    2012-01-01

    Isolated growth hormone deficiency type-2 (IGHD-2), the autosomal-dominant form of GH deficiency, is mainly caused by specific splicing mutations in the human growth hormone (hGH) gene (GH-1). These mutations, occurring in and around exon 3, cause complete exon 3 skipping and produce a dominant-negative 17.5 kD GH isoform that reduces the accumulation and secretion of wild type-GH (wt-GH). At present, patients suffering from IGHD-2 are treated with daily injections of recombinant human GH (rhGH) in order to reach normal height. However, this type of replacement therapy, although effective in terms of growth, does not prevent toxic effects of the 17.5-kD mutant on the pituitary gland, which can eventually lead to other hormonal deficiencies. Considering a well-known correlation between the clinical severity observed in IGHD-2 patients and the increased expression of the 17.5-kD isoform, therapies that specifically target this isoform may be useful in patients with GH-1 splicing defects. This chapter focuses on molecular strategies that could represent future directions for IGHD-2 treatment.

  14. Peptic ulceration may be a hormonal deficiency disease.

    PubMed

    Love, Jack W

    2008-01-01

    Evidence is reviewed that Helicobacter pylori infection may cause a deficiency of the hormone secretin that allows peptic ulcer disease to develop by impairing the body's defenses to gastric acid. Secretin is released into the circulation from the S-cells of the duodenal crypts in response to gastric acid entering the duodenum. Once in the circulation, secretin has five well-documented effects that protect the upper intestine from gastric acid: it stimulates secretion of bicarbonate rich exocrine pancreatic juice; it stimulates secretion of alkaline bile; it stimulates secretion of alkaline mucus from the duodenal submucosal glands of Brunner; it inhibits the humoral phase of gastric secretion; and it inhibits gastric motility, thereby delaying gastric emptying. Impaired secretin release and reduced duodenal S-cells have been documented in peptic ulcer patients compared with control patients. Clinical evidence that patients with H. pylori infection and peptic ulceration have increased gastric secretion and motility and decreased duodenal bicarbonate response to gastric acid, all of which normalize after eradication of the infection, could be explained by reversible impairment of the secretin mechanism. Gastric metaplasia in the duodenum with H. pylori infection is known to reduce the S-cell population. The fact that not all patients with H. pylori infection develop peptic ulceration suggests that degree of secretin deficiency determined by extent of the infection must reach a critical level for peptic ulceration to occur. Peptic ulceration may be a hormonal deficiency disease, a result of secretin deficiency caused by H. pylori infection. It may be the first example of a specific hormonal deficiency disease caused by a specific bacterial infection.

  15. Concomitant therapies (glucocorticoids and sex hormones) in adult patients with growth hormone deficiency.

    PubMed

    Scaroni, C; Ceccato, F; Rizzati, S; Mantero, F

    2008-09-01

    Adult-onset GH deficiency (GHD), mostly due to organic lesions of the pituitary-hypothalamic region, is frequently associated with multiple anterior pituitary deficiencies that need long-term substitutive treatment. The GH-IGF-I axis may play an important role in modulating peripheral metabolism of hormones (adrenal, thyroid, and sex hormones) and these interactions may have clinically significant implications on the phenotypes of adult GHD patients and on the effects of the combined replacement hormonal treatment of this condition. By accelerating the peripheral metabolism of cortisol, GH therapy may precipitate adrenal insufficiency in susceptible hypopituitary patients; estrogen replacement blunts the response to GH in women whereas in men with androgen substitution the responsivity increases over time. Endocrinologists should be mindful of these phenomena when starting patients with hypopituitarism on GH replacement therapy.

  16. Baraitser and Winter syndrome with growth hormone deficiency

    PubMed Central

    Chentli, Farida; Zellagui, Hadjer

    2014-01-01

    Baraitser–Winter syndrome (BWS), first reported in 1988, is apparently due to genetic abnormalities that are still not well-defined, although many gene abnormalities are already discovered and de novo missense changes in the cytoplasmic actin-encoding genes (called ACTB and ACTG1) have been recently discovered. The syndrome combines facial and cerebral malformations. Facial malformations totally or partially present in the same patient are: Iris coloboma, bilateral ptosis, hypertelorism, broad nasal bridge, and prominent epicanthic folds. The various brain malformations are probably responsible for growth and mental retardation. To the best of our knowledge, the syndrome is very rare as few cases have been reported so far. Our aim was to describe a child with a phenotype that looks like BWS with proved partial growth hormone (GH) deficiency which was not reported before. A girl aged 7-year-old of consanguineous parents was referred for short stature and mental retardation. Clinical examination showed dwarfism and a delay in her mental development. Other clinical features included: Strabismus, epicanthic folds, broad nasal bridge, and brain anomalies such as lissencephaly, bilateral hygroma, and cerebral atrophy. Hormonal assessment showed partial GH deficiency without other endocrine disorders. Our case looks exactly like BWS. However, apart from facial and cerebral abnormalities, there is a partial GH deficiency which can explain the harmonious short stature. This case seems worth to be reported as it adds GH deficiency to the very rare syndrome. PMID:25624931

  17. Maternal iron deficiency alters circulating thyroid hormone levels in developing neonatal rats

    EPA Science Inventory

    Thyroid hormone insufficiency and iron deficiency (FeD) during fetal and neonatal life are both similarly deleterious to mammalian development suggesting a possible linkage between iron and thyroid hormone insufficiencies. Recent published data from our laboratory demonstrate a r...

  18. Color Vision Deficiencies in Children. United States.

    ERIC Educational Resources Information Center

    National Center for Health Statistics (DHEW/PHS), Hyattsville, MD.

    Presented are prevalence data on color vision deficiencies (color blindness) in noninstitutionalized children, aged 6-11, in the United States, as estimated from the Health Examination Survey findings on a representative sample of over 7,400 children. Described are the two color vision tests used in the survey, the Ishihara Test for Color…

  19. Evaluation of growth hormone release and human growth hormone treatment in children with cranial irradiation-associated short stature

    SciTech Connect

    Romshe, C.A.; Zipf, W.B.; Miser, A.; Miser, J.; Sotos, J.F.; Newton, W.A.

    1984-02-01

    We studied nine children who had received cranial irradiation for various malignancies and subsequently experienced decreased growth velocity. Their response to standard growth hormone stimulation and release tests were compared with that in seven children with classic GH deficiency and in 24 short normal control subjects. With arginine and L-dopa stimulation, six of nine patients who received radiation had a normal GH response (greater than 7 ng/ml), whereas by design none of the GH deficient and all of the normal children had a positive response. Only two of nine patients had a normal response to insulin hypoglycemia, with no significant differences in the mean maximal response of the radiation and the GH-deficient groups. Pulsatile secretion was not significantly different in the radiation and GH-deficient groups, but was different in the radiation and normal groups. All subjects in the GH-deficient and radiation groups were given human growth hormone for 1 year. Growth velocity increased in all, with no significant difference in the response of the two groups when comparing the z scores for growth velocity of each subject's bone age. We recommend a 6-month trial of hGH in children who have had cranial radiation and are in prolonged remission with a decreased growth velocity, as there is no completely reliable combination of GH stimulation or release tests to determine their response.

  20. Rasch Measurement in the Assessment of Growth Hormone Deficiency in Adult Patients.

    ERIC Educational Resources Information Center

    Prieto, Luis; Roset, Montse; Badia, Xavier

    2001-01-01

    Tested the metric properties of a Spanish version of the Assessment of Growth Hormone Deficiency in Adults (AGHDA) questionnaire through Rasch analysis with a sample of 356 adult patients in Spain. Results suggest that the Spanish AGHDA could be a useful complement of the clinical evaluation of growth hormone deficiency patients at group and…

  1. Adult Growth Hormone Deficiency – Benefits, Side Effects, and Risks of Growth Hormone Replacement

    PubMed Central

    Reed, Mary L.; Merriam, George R.; Kargi, Atil Y.

    2013-01-01

    Deficiency of growth hormone (GH) in adults results in a syndrome characterized by decreased muscle mass and exercise capacity, increased visceral fat, impaired quality of life, unfavorable alterations in lipid profile and markers of cardiovascular risk, decrease in bone mass and integrity, and increased mortality. When dosed appropriately, GH replacement therapy (GHRT) is well tolerated, with a low incidence of side effects, and improves most of the alterations observed in GH deficiency (GHD); beneficial effects on mortality, cardiovascular events, and fracture rates, however, remain to be conclusively demonstrated. The potential of GH to act as a mitogen has resulted in concern over the possibility of increased de novo tumors or recurrence of pre-existing malignancies in individuals treated with GH. Though studies of adults who received GHRT in childhood have produced conflicting reports in this regard, long-term surveillance of adult GHRT has not demonstrated increased cancer risk or mortality. PMID:23761782

  2. Prader-Willi syndrome and growth hormone deficiency.

    PubMed

    Aycan, Zehra; Baş, Veysel Nijat

    2014-01-01

    Prader-Willi syndrome (PWS) is a rare multisystem genetic disorder demonstrating great variability with changing clinical features during patient's life. It is characterized by severe hypotonia with poor sucking and feeding difficulties in early infancy, followed by excessive eating and gradual development of morbid obesity in later infancy or early childhood. The phenotype is most probably due to hypothalamic dysfunction which is also responsible for growth hormone (GH) and thyroid-stimulating hormone (TSH) deficiencies, central adrenal insufficiency and hypogonadism. The multidimensional problems of patients with PWS can be managed with multidisciplinary approach. Reduced GH secretion, low peak GH response to stimulation, decreased spontaneous GH secretion and low serum IGF-1 levels in PWS patients have been documented in many studies. GH therapy has multiple beneficial effects on growth and body composition, motor and mental development in PWS patients. The recommended dosage for GH is 0.5-1 mg/m2/day. GH therapy should not be started in the presence of obstructive sleep apnea syndrome, adenotonsillar hypertrophy, severe obesity and diabetes mellitus. GH treatment should be considered for patients with genetically confirmed PWS in conjunction with dietary, environmental and life-style measures.

  3. Severe hyponatremia due to ACTH insufficiency in a 14 year-old girl with growth hormone deficiency.

    PubMed

    Tseng, Lo-Lin; Lue, Hung-Chi; Huang, Cheng-Hung; Niu, Dau-Ming

    2010-01-01

    SIADH-like hyponatremia as the presenting manifestation of ACTH deficiency is rare in childhood. Here we report a 14 year-old girl who, after 8 years of GH replacement and subsequent treatment for subclinical secondary hypothyroidism, presented with confusion and disorientation due to severe hyponatremia. When her pituitary axis was re-assessed, she was diagnosed as having ACTH deficiency associated with multiple pituitary hormone deficiencies (MPHD) (including GH, FSH, LH, and subclinical TSH deficiencies). She responded poorly to treatment with only hypertonic fluid, but improved after addition of hydrocortisone replacement. The purpose of this paper is to emphasize the importance of suspecting ACTH insufficiency in children with GH deficiency if hyponatremia develops.

  4. Improved response of growth hormone to growth hormone-releasing hormone and reversible chronic thyroiditis after hydrocortisone replacement in isolated adrenocorticotropic hormone deficiency.

    PubMed

    Inagaki, Miho; Sato, Haruhiro; Miyamoto, Yoshiyasu; Hirukawa, Takashi; Sawaya, Asako; Miyakogawa, Takayo; Tatsumi, Ryoko; Kakuta, Takatoshi

    2009-07-20

    We report a 44-year-old Japanese man who showed a reversible blunted response of growth hormone (GH) to GH-releasing hormone (GRH) stimulation test and reversible chronic thyroiditis accompanied by isolated ACTH deficiency. He was admitted to our hospital because of severe general malaise, hypotension, and hypoglycemia. He showed repeated attacks of hypoglycemia, and his serum sodium level gradually decreased. Finally, he was referred to the endocrinology division, where his adrenocorticotropic hormone (ACTH) and cortisol values were found to be low, and his GH level was slightly elevated. An increased value of thyroid stimulating hormone (TSH) and decreased values of free triidothyronine and free thyroxine were observed along with anti-thyroglobulin antibody, suggesting chronic thyroiditis. Pituitary stimulation tests revealed a blunted response of ACTH and cortisol to corticotropin-releasing hormone, and a blunted response of GH to GRH. Hydrocortisone replacement was then started, and this improved the patient's general condition. His hypothyroid state gradually ameliorated and his titer of anti-thyroglobulin antibody decreased to the normal range. Pituitary function was re-evaluated with GRH stimulation test under a maintenance dose of 20 mg/day hydrocortisone and showed a normal response of GH to GRH. It is suggested that re-evaluation of pituitary and thyroid function is useful for diagnosing isolated ACTH deficiency after starting a maintenance dose of hydrocortisone in order to avoid unnecessary replacement of thyroid hormone.

  5. Zip1, Zip2, and Zip8 mRNA expressions were associated with growth hormone level during the growth hormone provocation test in children with short stature.

    PubMed

    Sun, Ping; Wang, Shifu; Jiang, Yali; Tao, Yanting; Tian, Yuanyuan; Zhu, Kai; Wan, Haiyan; Zhang, Lehai; Zhang, Lianying

    2013-10-01

    Short stature of children is affected by multiple factors. One of them is growth hormone (GH) deficiency. Growth hormone therapy can increase the final height of children with growth hormone deficiency. Zinc is found to induce dimerization and to enhance the bioactivity of human GH. Two gene families have been identified involved in zinc homeostasis. Previous studies in our laboratory have shown that Zip1, Zip2, Zip6, and ZnT1 mRNA were associated with zinc level in established human breast cancer in nude mice model; Zip8 was significantly lower in zinc-deficient Wistar rats in kidney. In this study, five zinc transporters: Zip1, Zip2, Zip6, Zip8, and ZnT1 were chosen. We aimed to investigate the mRNA expression of zinc transporters and to explore the relationship between zinc transporters and growth hormone in short stature children. Growth hormone provocation test is used to confirm the diagnosis of growth hormone deficiency. Six short children for the test were enrolled. At the same time, 15 sex- and age-matched normal children were enrolled as control. The expression levels of zinc transporters in peripheral blood mononuclear cells were determined by quantitative real-time PCR. Zip1 and Zip2 mRNA expression positively correlated with growth hormone level (r = 0.5133, P = 0.0371; r = 0.6719, P = 0.0032); Zip8 mRNA expression negatively correlated with growth hormone level (r = -0.5264, P = 0.0285) during the test in short stature children. The average expression level of Zip2 was significantly higher and Zip6, Zip8 mRNA levels were significantly lower in short stature children than in health controls at 0 min (P < 0.05, P < 0.05).

  6. Challenges in the Diagnosis and Management of Growth Hormone Deficiency in India

    PubMed Central

    John, Mathew; Koledova, Ekaterina; Kumar, Kanakatte Mylariah Prasanna

    2016-01-01

    In clinical practice, every year approximately 150,000 children are referred with short stature (SS) based on a cut-off of fifth percentile. The most important endocrine and treatable cause of SS is growth hormone deficiency (GHD). The lack of reliable data on the prevalence of GHD in India limits estimation of the magnitude of this problem. The diagnosis and treatment of GHD are hurdled with various challenges, restricting the availability of growth hormone (GH) therapy to only a very limited segment of the children in India. This review will firstly summarize the gaps and challenges in diagnosis and treatment of GHD based on literature analysis. Subsequently, it presents suggestions from the members at advisory board meetings to overcome these challenges. The advisory board suggested that early initiation of the therapy could better the chances of achieving final adult height within the normal range for the population. Education and awareness about growth disorders among parents, regular training for physicians, and more emphasis on using the Indian growth charts for growth monitoring would help improve the diagnosis and treatment of children with GHD. Availability of an easy-to-use therapy delivery system could also be beneficial in improving adherence and achieving satisfactory outcomes. PMID:27867396

  7. Laboratory diagnosis of multiple pituitary hormone deficiencies: issues with testing of the growth and thyroid axes.

    PubMed

    Nakamoto, Jon

    2009-01-01

    Clinical manifestations of hypopituitarism are variable and depend on the severity of hormone deficiency, creating a diagnostic challenge for diagnosis of the non-classical patient who may have a less severe growth hormone (GH) deficiency and only a suggestion of possible hypothyroidism. Laboratory tests contribute to the diagnostic process, but the tests for growth and thyroid dysfunction, two of the most common manifestations of multiple pituitary hormone deficiency, are some of the most problematic from a methodological perspective. Patients in the "grey zone" of diagnosis, for whom there is no distinct dividing line or gold standard diagnostic test, are the focus of this article. Issues relating to the use of laboratory tests involving GH, insulin-like growth factor-I, and free thyroxine in the diagnosis of GH and thyroid deficiency are reviewed. Assay harmonization initiatives are required before clinical research studies are performed to establish diagnostic thresholds for GH and thyroid hormone deficiencies.

  8. Effects of growth hormone and insulin-like growth factor 1 deficiency on ageing and longevity.

    PubMed

    Laron, Zvi

    2002-01-01

    Present knowledge on the effects of growth hormone (GH)/insulin-like growth hormone (IGF)1 deficiency on ageing and lifespan are reviewed. Evidence is presented that isolated GH deficiency (IGHD), multiple pituitary hormone deficiencies (MPHD) including GH, as well as primary IGE1 deficiency (GH resistance, Laron syndrome) present signs of early ageing such as thin and wrinkled skin, obesity, hyperglycemia and osteoporosis. These changes do not seem to affect the lifespan, as patients reach old age. Animal models of genetic MPHD (Ames and Snell mice) and GH receptor knockout mice (primary IGF1 deficiency) also have a statistically significant higher longevity compared to normal controls. On the contrary, mice transgenic for GH and acromegalic patients secreting large amounts of GH have premature death. In conclusion longstanding GH/IGF1 deficiency affects several parameters of the ageing process without impairing lifespan, and as shown in animal models prolongs longevity. In contrast high GH/IGF1 levels accelerate death.

  9. Laryngeal and vocal evaluation in untreated growth hormone deficient adults

    PubMed Central

    Barreto, Valéria M.P.; D'Ávila, Jeferson S.; Sales, Neuza J.; Gonçalves, Maria Inês R.; Seabra, Juliane Dantas; Salvatori, Roberto; Aguiar-Oliveira, Manuel H.

    2009-01-01

    OBJECTIVE To evaluate the consequences of lifetime, severe and untreated isolated growth hormone deficiency (IGHD) on vocal and laryngeal function. STUDY DESIGN Cross-sectional. SUBJECTS AND METHODS A total of 23 IGHD adult subjects and 22 controls were administered a questionnaire about vocal complaints and harmful voice habits, and underwent video-laryngostroboscopic examination, voice evaluation by perceptual-auditory analysis with GRBAS scale including grade of dysphonia, roughness, breathiness, asthenia and strain items, objective voice evaluation by maximum phonation time (MPT), and acoustic analysis. RESULTS There was no difference in vocal complaints between IGHD subjects and controls. Vocal abuse and smoking were more frequent in IGHD subjects. IGHD subjects presented higher values for roughness, breathiness, and strain. Laryngopharyngeal reflux (LPR) signs and laryngeal constriction were more frequent in IGHD individuals. MPT was similar in the two groups. Fundamental frequency was higher in IGHD females and males. Harmonic to noise ratio was higher in IGHD in both genders and shimmer was lower in IGHD females. CONCLUSIONS IGHD subjects have higher prevalence of signs of LPR and laryngeal constriction, with high pitch in both genders, which suggests a prominent role of IGHD on these parameters. PMID:19130959

  10. [Benefits and risks of growth hormone in adults with growth hormone deficiency].

    PubMed

    Díez, Juan J; Cordido, Fernando

    2014-10-21

    Adult growth hormone (GH) deficiency is a well-recognized clinical syndrome with adverse health consequences. Many of these may improve after replacement therapy with recombinant GH. This treatment induces an increase in lean body mass and a decrease in fat mass. In long-term studies, bone mineral density increases and muscle strength improves. Health-related quality of life tends to increase after treatment with GH. Lipid profile and markers of cardiovascular risk also improve with therapy. Nevertheless, GH replacement therapy is not without risk. According to some studies, GH increases blood glucose, body mass index and waist circumference and may promote long-term development of diabetes and metabolic syndrome. Risk of neoplasia does not appear to be increased in adults treated with GH, but there are some high-risk subgroups. Methodological shortcomings and difficulties inherent to long-term studies prevent definitive conclusions about the relationship between GH and survival. Therefore, research in this field should remain active.

  11. Efficacy and Safety of Sustained-Release Recombinant Human Growth Hormone in Korean Adults with Growth Hormone Deficiency

    PubMed Central

    Kim, Youngsook; Hong, Jae Won; Chung, Yoon-Sok; Kim, Sung-Woon; Cho, Yong-Wook; Kim, Jin Hwa; Kim, Byung-Joon

    2014-01-01

    Purpose The administration of recombinant human growth hormone in adults with growth hormone deficiency has been known to improve metabolic impairment and quality of life. Patients, however, have to tolerate daily injections of growth hormone. The efficacy, safety, and compliance of weekly administered sustained-release recombinant human growth hormone (SR-rhGH, Declage™) supplement in patients with growth hormone deficiency were evaluated. Materials and Methods This trial is 12-week prospective, single-arm, open-label trial. Men and women aged ≥20 years with diagnosed growth hormone deficiency (caused by pituitary tumor, trauma and other pituitary diseases) were eligible for this study. Each subject was given 2 mg (6 IU) of SR-rhGH once a week, subcutaneously for 12 weeks. Efficacy and safety at baseline and within 30 days after the 12th injection were assessed and compared. Score of Assessment of Growth Hormone Deficiency in Adults (AGHDA score) for quality of life and serum IGF-1 level. Results The IGF-1 level of 108.67±74.03 ng/mL was increased to 129.01±68.37 ng/mL (p=0.0111) and the AGHDA QoL score was decreased from 9.80±6.51 to 7.55±5.76 (p<0.0001) at week 12 compared with those at baseline. Adverse events included pain, swelling, erythema, and warmth sensation at the administration site, but many adverse events gradually disappeared during the investigation. Conclusion Weekly administered SR-rhGH for 12 weeks effectively increased IGF-1 level and improved the quality of life in patients with GH deficiency without serious adverse events. PMID:24954335

  12. Growth hormone treatment in growth hormone-deficient adults. II. Effects on exercise performance.

    PubMed

    Cuneo, R C; Salomon, F; Wiles, C M; Hesp, R; Sönksen, P H

    1991-02-01

    Growth hormone (GH) treatment in adults with GH deficiency increases lean body mass and thigh muscle cross-sectional area. The functional significance of this was examined by incremental cycle ergometry in 24 GH-deficient adults treated in a double-blind placebo-controlled trial with recombinant DNA human GH (rhGH) for 6 mo (0.07 U/kg body wt daily). Compared with placebo, the rhGH group increased mean maximal O2 uptake (VO2max) (+406 +/- 71 vs. +133 +/- 84 ml/min; P = 0.016) and maximal power output (+24.6 +/- 4.3 vs. +9.7 +/- 4.8 W; P = 0.047), without differences in maximal heart rate or ventilation. Forced expiratory volume in 1 s, vital capacity, and corrected CO gas transfer were within normal limits and did not change with treatment. Mean predicted VO2max, based on height and age, increased from 78.9 to 96.0% in the rhGH group (compared with 78.5 and 85.0% for placebo; P = 0.036). The anaerobic ventilatory threshold increased in the rhGH group (+159 +/- 39 vs. +1 +/- 51 ml/min; P = 0.02). The improvement in VO2max was noted when expressed per kilogram body weight but not lean body mass or thigh muscle area. We conclude that rhGH treatment in adults with GH deficiency improves and normalizes maximal exercise performance and improves submaximal exercise performance and that these changes are related to increases in lean body mass and muscle mass. Improved cardiac output may also contribute to the effect of rhGH on exercise performance.

  13. The cardiovascular system in growth hormone excess and growth hormone deficiency.

    PubMed

    Lombardi, G; Di Somma, C; Grasso, L F S; Savanelli, M C; Colao, A; Pivonello, R

    2012-12-01

    The clinical conditions associated with GH excess and GH deficiency (GHD) are known to be associated with an increased risk for the cardiovascular morbidity and mortality, suggesting that either an excess or a deficiency in GH and/or IGF-I is deleterious for cardiovascular system. In patients with acromegaly, chronic GH and IGF-I excess commonly causes a specific cardiomyopathy characterized by a concentric cardiac hypertrophy associated with diastolic dysfunction and, in later stages, with systolic dysfunction ending in heart failure if GH/IGF-I excess is not controlled. Abnormalities of cardiac rhythm and anomalies of cardiac valves can also occur. Moreover, the increased prevalence of cardiovascular risk factors, such as hypertension, diabetes mellitus, and insulin resistance, as well as dyslipidemia, confer an increased risk for vascular atherosclerosis. Successful control of the disease is accompanied by a decrease of the cardiac mass and improvement of cardiac function and an improvement in cardiovascular risk factors. In patients with hypopituitarism, GHD has been considered the under- lying factor of the increased mortality when appropriate standard replacement of the pituitary hormones deficiencies is given. Either childhood-onset or adulthood-onset GHD are characterized by a cluster of abnormalities associated with an increased cardiovascular risk, including altered body composition, unfavorable lipid profile, insulin resistance, endothelial dysfunction and vascular atherosclerosis, a decrease in cardiac mass together with an impairment of systolic function mainly after exercise. Treatment with recombinant GH in patients with GHD is followed by an improvement of the cardiovascular risk factors and an increase in cardiac mass together with an improvement in cardiac performance. In conclusion, acromegaly and GHD are associated with an increased risk for cardiovascular morbidity and mortality, but the control of GH/IGF-I secretion reverses cardiovascular

  14. Isolated adrenocorticotropic hormone deficiency development during chemotherapy for gastric cancer: a case report

    PubMed Central

    2014-01-01

    Introduction Isolated adrenocorticotropic hormone deficiency is an endocrinological disorder characterized by loss of adrenocorticotropic hormone and resultant adrenal insufficiency. Affected patients often present with fatigue, anorexia, and hyponatremia. Although the number of reported cases has been recently increasing, isolated adrenocorticotropic hormone deficiency combined with malignant neoplasia is very rare. Here we describe a patient with gastric cancer who developed unexpected isolated adrenocorticotropic hormone deficiency during chemotherapy. Case presentation A 72-year-old Japanese man was admitted to our hospital because of febrile neutropenia due to chemotherapy for gastric cancer recurrence. Although the neutropenia and fever immediately improved, he became unable to take any oral medications and was bedridden 1 week after admission. His serum sodium level abruptly decreased to 122mEq/L on the fifth day of hospitalization. We performed endocrinological studies to investigate the cause of his hyponatremia and plasma hyposmolality. His plasma adrenocorticotropic hormone and cortisol levels were very low. However, his serum levels of all other anterior pituitary hormones were slightly elevated. We then performed a corticotropin-releasing hormone test, which showed that neither his plasma adrenocorticotropic hormone nor cortisol level responded to corticotropin-releasing hormone stimulation. We definitively diagnosed isolated adrenocorticotropic hormone deficiency based on these findings. Hydrocortisone replacement therapy was begun at 20mg/day, resulting in a marked improvement in his anorexia and general fatigue within a few days. His serum sodium level was also normalized immediately after the administration of hydrocortisone. He was discharged from our hospital on the 50th day of hospitalization. Conclusions The present case is the second report of a patient with concurrent isolated adrenocorticotropic hormone deficiency and gastric cancer and the

  15. Growth Hormone Response after Administration of L-dopa, Clonidine, and Growth Hormone Releasing Hormone in Children with Down Syndrome.

    ERIC Educational Resources Information Center

    Pueschel, Seigfried M.

    1993-01-01

    This study of eight growth-retarded children with Down's syndrome (aged 1 to 6.5 years) found that administration of growth hormone was more effective than either L-dopa or clonidine. Results suggest that children with Down's syndrome have both anatomical and biochemical hypothalamic derangements resulting in decreased growth hormone secretion and…

  16. Growth hormone replacement therapy reduces risk of cancer in adult with growth hormone deficiency: A meta-analysis

    PubMed Central

    Li, Zhanzhan; Zhou, Qin; Li, Yanyan; Fu, Jun; Huang, Xinqiong; Shen, Liangfang

    2016-01-01

    The risk of growth hormone on cancer in adult with growth hormone deficiency remains unclear. We carried out a meta-analysis to evaluate the risk of cancer in adult with and without growth hormone replacement therapy. We searched PubMed, Web of Science, China National Knowledge Infrastructure, and WanFang databases up to 31 July 2016 for eligible studies. Pooled risk ratio (RR) with 95% confidence interval (CI) was calculated using fixed-or random-effects models if appropriate. The Newcastle-Ottawa Scale was used to assess the study quality. Two retrospective and seven prospective studies with a total of 11191 participants were included in the final analysis. The results from fixed-effects model showed this therapy was associated with the deceased risk of cancer in adult with growth hormone deficiency (RR=0.69, 95%CI: 0.59-0.82), with low heterogeneity within studies (I2=39.0%, P=0.108). We performed sensitivity analyses by sequentially omitting one study each time, and the pooled RRs did not materially change, indicating that our results were statistically stable. Begger's and Egger's tests suggested that there was no publication bias (Z=-0.63, P=0.520; t=0.16, P=0.874). Our study suggests that growth hormone replacement therapy could reduce risk of cancer in adult with growth hormone deficiency. PMID:27835910

  17. Growth hormone stimulation test - series (image)

    MedlinePlus

    The growth hormone (GH) is a protein hormone released from the anterior pituitary gland under the control of the hypothalamus. ... performed on infants and children to identify human growth hormone (hGH) deficiency as a cause of growth retardation. ...

  18. Type 2 diabetes mellitus accompanied by isolated adrenocorticotropic hormone deficiency and gastric cancer.

    PubMed

    Kamiya, Yuji; Murakami, Masami

    2009-01-01

    A 69-year-old man with type 2 diabetes mellitus was admitted to our hospital because of appetite loss, nausea and vomiting. Gastroscopy revealed gastric cancer. Levels of plasma cortisol were decreased. Neither adrenocorticotropic hormone (ACTH) nor cortisol levels were adequately increased in response to a mixed intravenous administration of corticotropin-releasing hormone, growth hormone-releasing hormone, thyrotropin-releasing hormone and lutenizing hormone-releasing hormone, although other pituitary hormones were increased adequately. He was diagnosed as having isolated ACTH deficiency (IAD). Anti-pituitary antibody and anti-parietal cell antibody were positive. At least in part, these antibodies may play pathogenic roles of development of IAD and gastric cancer.

  19. [Hormonal deficiencies in the elderly: is there a role for replacement therapy?].

    PubMed

    Racaru-Honciuc, Valentina; Betea, Daniela; Scheen, André J

    2014-08-27

    Biological aging is characterized by a progressive loss of the secretion of various hormones, a phenomenon that leads some physicians to propose an anti-aging hormonal therapy. It is mandatory to differentiate: 1) the physiological functional loss, which is a natural phenomenon without clear deleterious consequences on health and should not be compensated by the administration of hormones only to restore plasma levels similar to those measured in young people and 2) a pathological defect that deserves a replacement therapy to correct the endocrine deficiency and improve the health status of older individuals. This article considers the deficiencies in insulin, thyroid hormones, growth hormone, dehydroepiandrosterone (DHEA) and testosterone. For each hormone, a benefit/risk ratio of a so-called replacement therapy will be analyzed.

  20. Lead Toxicity and Iron Deficiency in Utah Migrant Children.

    ERIC Educational Resources Information Center

    Ratcliffe, Stephen D.; And Others

    1989-01-01

    Determines the frequency of presumptive iron deficiency and lead toxicity in 198 Utah migrant children, aged 9-72 months. There were no confirmed cases of lead toxicity. Thirteen percent of all children tested, and 30 percent of those aged 9-23 months, were iron deficient. Hematocrit determination is an insensitive screen for iron deficiency.…

  1. Children with limb deficiencies: demographic characteristics.

    PubMed

    Al-Worikat, Abdel Fattah; Dameh, Walid

    2008-03-01

    The aim of this study is to determine the demographic data for amputations in children in relation of age, sex, level and cause of amputation. Data were collected from the records of amputees who attended the prosthetic clinic at the Royal Rehabilitation Center, King Hussein Medical Centre, Jordan, between 1 January 1995 and 31 December 2005. Demographic data (age, sex, level and cause of amputation) were analyzed. Some 120 children with different levels of amputation were included with mean age of 6.2 years. There were 64 (53.3%) males and 56 (46.7%) females. Male to female ratio was 1.15:1. The dominant level of amputation was trans-radial in 10 patients (15.62%) in the upper limb and trans-tibial in 18 patients (28.12%) in the lower limb. The dominant cause of amputation was congenital deficiency in 56 patients (46.67%) followed by trauma in 48 (40%). The results of this study presented greater similarities to others in the literature, congenital limb deficiency being the dominant cause of amputation in children. This study helps in planning the needs for materials and budgets for the treatment of amputee children in Jordan.

  2. [Growth hormone treatment update].

    PubMed

    2014-02-01

    Short stature in children is a common cause for referral to pediatric endocrinologists, corresponding most times to normal variants of growth. Initially growth hormone therapy was circumscribed to children presenting growth hormone deficiency. Since the production of recombinant human hormone its use had spread to other pathologies.

  3. Effects of recombinant growth hormone replacement and physical rehabilitation in recovery of gross motor function in children with cerebral palsy

    PubMed Central

    Reimunde, Pedro; Rodicio, Cristina; López, Natalia; Alonso, Alba; Devesa, Pablo; Devesa, Jesús

    2010-01-01

    Cerebral palsy is an important health issue that has a strong socioeconomic impact. There is no cure for cerebral palsy, and therapeutic approaches only report small benefits for affected people. In this study we assessed the effects of growth hormone treatment (0.3 μg/kg/day) combined with physical rehabilitation in the recovery of gross motor function in children with growth hormone deficiency and cerebral palsy (four males and six females, mean age 5.63 ± 2.32 years) as compared with that observed in a similar population of cerebral palsy children (five males, five females, mean age 5.9 ± 2.18 years) without growth hormone deficiency treated only with physical rehabilitation for two months. The Gross Motor Function Measure (GMFM-88) and Modified Ashworth Scale were performed before commencing the treatment and after completion thereof. In children with cerebral palsy and growth hormone deficiency, Dimension A (P < 0.02), dimension B (P < 0.02), and dimension C (P < 0.02) of the GMFM-88, and the total score of the test (P < 0.01) significantly improved after the treatment; dimension D and dimension E did not increase, and four of five spastic patients showed a reduction in spasticity. However, in children with cerebral palsy and without growth hormone deficiency, only the total score of the test improved significantly after the treatment period. This indicates that growth hormone replacement therapy was responsible for the large differences observed between both groups in response to physical rehabilitation. We propose that the combined therapy involving growth hormone administration and physical rehabilitation may be a useful therapeutic approach in the recovery of gross motor function in children with growth hormone deficiency and cerebral palsy. PMID:21151628

  4. Effects of recombinant growth hormone replacement and physical rehabilitation in recovery of gross motor function in children with cerebral palsy.

    PubMed

    Reimunde, Pedro; Rodicio, Cristina; López, Natalia; Alonso, Alba; Devesa, Pablo; Devesa, Jesús

    2010-11-30

    Cerebral palsy is an important health issue that has a strong socioeconomic impact. There is no cure for cerebral palsy, and therapeutic approaches only report small benefits for affected people. In this study we assessed the effects of growth hormone treatment (0.3 μg/kg/day) combined with physical rehabilitation in the recovery of gross motor function in children with growth hormone deficiency and cerebral palsy (four males and six females, mean age 5.63 ± 2.32 years) as compared with that observed in a similar population of cerebral palsy children (five males, five females, mean age 5.9 ± 2.18 years) without growth hormone deficiency treated only with physical rehabilitation for two months. The Gross Motor Function Measure (GMFM-88) and Modified Ashworth Scale were performed before commencing the treatment and after completion thereof. In children with cerebral palsy and growth hormone deficiency, Dimension A (P < 0.02), dimension B (P < 0.02), and dimension C (P < 0.02) of the GMFM-88, and the total score of the test (P < 0.01) significantly improved after the treatment; dimension D and dimension E did not increase, and four of five spastic patients showed a reduction in spasticity. However, in children with cerebral palsy and without growth hormone deficiency, only the total score of the test improved significantly after the treatment period. This indicates that growth hormone replacement therapy was responsible for the large differences observed between both groups in response to physical rehabilitation. We propose that the combined therapy involving growth hormone administration and physical rehabilitation may be a useful therapeutic approach in the recovery of gross motor function in children with growth hormone deficiency and cerebral palsy.

  5. Growth hormone deficiency in a dopa-responsive dystonia patient with a novel mutation of guanosine triphosphate cyclohydrolase 1 gene.

    PubMed

    Lin, Yu; Wang, Dan-Ni; Chen, Wan-Jin; Lin, Xiang; Lin, Min-Ting; Wang, Ning

    2015-05-01

    Dopa-responsive dystonia is a rare hereditary movement disorder caused by mutations in the guanosine triphosphate cyclohydrolase 1 (GCH1) gene. This disease typically manifests in dystonia, with marked diurnal fluctuation and a dramatic response to levodopa. However, growth retardation in dopa-responsive dystonia has rarely been reported, and the etiology of short stature is not clarified. Here, we report a 14-year-old patient with extremities dystonia and short stature. Treatment with levodopa relieved his symptoms and resulted in a height increase. We also investigated the mutation in GCH1 and the etiology of short stature in this case. Sequence analysis of GCH1 revealed a novel mutation (c.695G>T). Laboratory examinations and imaging confirmed the diagnosis of growth hormone deficiency. We conclude that our case reveals a rare feature for dopa-responsive dystonia and suggests a possible pathogenic link between growth hormone deficiency and dopa-responsive dystonia. We recommend levodopa as the first choice for treating dopa-responsive dystonia in children with growth hormone deficiency.

  6. Cognitive deficiencies in emotionally disturbed children.

    PubMed

    Curley, J F; Pabis, R

    1978-01-01

    This research investigated development of cognitive abilities in a normal vs. emotionally disturbed school age population (N = 240) ages 6-12. The Ss had to display skills on the Southern Illinois University Test necessary to show understanding of Piagetian concepts of class inclusion, class exclusion, and complement of set. A three way analysis of variance indicated significant main effects for age, sex, and emotionality factors. There were, however, no significant interactions among these factors. Emotionally disturbed children were not only deficient in the measured cognitive skills, but even the rate of development of these cognitive skills was inferior to that of the normal population.

  7. Lead toxicity and iron deficiency in Utah migrant children

    SciTech Connect

    Ratcliffe, S.D.; Lee, J.; Lutz, L.J.; Woolley, F.R.; Baxter, S. ); Civish, F. ); Johnson, M. )

    1989-05-01

    The authors determined the frequency of presumptive iron deficiency and lead toxicity in 198 Utah migrant children, ages 9-72 months, during the summer of 1985. There were no confirmed cases of lead toxicity, 13% of those tested and 30% of the children ages 9-23 months were iron deficient. Hematocrit determinations accurately predicted iron deficiency in only 35% of the children confirmed to have this disorder via erythrocyte protoporphyrin screening.

  8. Lead toxicity and iron deficiency in Utah migrant children.

    PubMed Central

    Ratcliffe, S D; Lee, J; Lutz, L J; Woolley, F R; Baxter, S; Civish, F; Johnson, M

    1989-01-01

    We determined the frequency of presumptive iron deficiency and lead toxicity in 198 Utah migrant children, ages 9-72 months, during the summer of 1985. There were no confirmed cases of lead toxicity. Thirteen per cent of those tested and 30 per cent of the children ages 9-23 months were iron deficient. Hematocrit determinations accurately predicted iron deficiency in only 35 per cent of the children confirmed to have this disorder via erythrocyte protoporphyrin screening. PMID:2650572

  9. Absence of serum growth hormone binding protein in patients with growth hormone receptor deficiency (Laron dwarfism)

    SciTech Connect

    Daughaday, W.H.; Trivedi, B.

    1987-07-01

    It has recently been recognized that human serum contains a protein that specifically binds human growth hormone (hGH). This protein has the same restricted specificity for hGH as the membrane-bound GH receptor. To determine whether the GH-binding protein is a derivative of, or otherwise related to, the GH receptor, the authors have examined the serum of three patients with Laron-type dwarfism, a condition in which GH refractoriness has been attributed to a defect in the GH receptor. The binding of /sup 125/I-labeled hGH incubated with serum has been measured after gel filtration of the serum through an Ultrogel AcA 44 minicolumn. Results are expressed as percent of specifically bound /sup 125/I-hGH and as specific binding relative to that of a reference serum after correction is made for endogenous GH. The mean +/- SEM of specific binding of sera from eight normal adults (26-46 years of age) was 21.6 +/- 0.45%, and the relative specific binding was 101.1 +/- 8.6%. Sera from 11 normal children had lower specific binding of 12.5 +/- 1.95% and relative specific binding of 56.6 +/- 9.1%. Sera from three children with Laron-type dwarfism lacked any demonstrable GH binding, whereas sera from 10 other children with other types of nonpituitary short stature had normal relative specific binding. They suggest that the serum GH-binding protein is a soluble derivative of the GH receptor. Measurement of the serum GH-binding protein may permit recognition of other abnormalities of the GH receptor.

  10. Chromatic VEP in children with congenital colour vision deficiency.

    PubMed

    Tekavčič Pompe, Manca; Stirn Kranjc, Branka; Brecelj, Jelka

    2010-09-01

    Visual evoked potentials to chromatic stimulus (cVEP) are believed to selectively test the parvocellular visual pathway which is responsible for processing information about colour. The aim was to evaluate cVEP in children with red-green congenital colour vision deficiency. VEP responses of 15 colour deficient children were compared to 31 children with normal colour vision. An isoluminant red-green stimulus composed of horizontal gratings was presented in an onset-offset manner. The shape of the waveform was studied, as well as the latency and amplitude of positive (P) and negative (N) waves. cVEP response did not change much with increased age in colour deficient children, whereas normative data showed changes from a predominantly positive to a negative response with increased age. A P wave was present in 87% of colour deficient children (and in 100% of children with normal colour vision), whereas the N wave was absent in a great majority of colour deficient children and was present in 80% of children with normal colour vision. Therefore, the amplitude of the whole response (N-P) decreased linearly with age in colour deficient children, whereas in children with normal colour vision it increased linearly. P wave latency shortened with increased age in both groups. cVEP responses differ in children with congenital colour vision deficiency compared to children with normal colour vision.

  11. Growth hormone releasing hexapeptide-6 (GHRP-6) test in the diagnosis of GH-deficiency.

    PubMed

    Pombo, M; Leal-Cerro, A; Barreiro, J; Peñalva, A; Peino, R; Mallo, F; Dieguez, C; Casanueva, F F

    1996-06-01

    Pituitary GH reserve can be assessed by substances that act directly at the somatotroph, such as GHRH, or by a variety of metabolic and neuropharmacological tests acting at the hypothalamic level, such as hypoglycemia, clonidine or L-Dopa. In order to evaluate GHRP-6 as a test of pituitary GH reserve, we studied GH responses of i.v. administered GHRP-6 in a group of short-statured children, as well as in a group of adults diagnosed with growth hormone deficiency (GHD) by conventional GH testing. Although we found that the GH response to GHRP-6 was lower in patients with GHD than in normal children, on an individual basis a considerable degree of overlap was observed between the two groups. In contrast, we found an almost complete blockade of GH response to either GHRP-6 or GHRH plus GHRP-6 in patients with pituitary stalk transection, suggesting that this could be a cost-effective test for the diagnosis of this condition. A similar finding was also obtained in GH response to the combined administration of GHRH plus GHRP-6 in patients with GHD of adult onset; this test may well prove valuable in the diagnosis of this clinical entity.

  12. Defective minor spliceosome mRNA processing results in isolated familial growth hormone deficiency

    PubMed Central

    Argente, Jesús; Flores, Raquel; Gutiérrez-Arumí, Armand; Verma, Bhupendra; Martos-Moreno, Gabriel Á; Cuscó, Ivon; Oghabian, Ali; Chowen, Julie A; Frilander, Mikko J; Pérez-Jurado, Luis A

    2014-01-01

    The molecular basis of a significant number of cases of isolated growth hormone deficiency remains unknown. We describe three sisters affected with severe isolated growth hormone deficiency and pituitary hypoplasia caused by biallelic mutations in the RNPC3 gene, which codes for a minor spliceosome protein required for U11/U12 small nuclear ribonucleoprotein (snRNP) formation and splicing of U12-type introns. We found anomalies in U11/U12 di-snRNP formation and in splicing of multiple U12-type introns in patient cells. Defective transcripts include preprohormone convertases SPCS2 and SPCS3 and actin-related ARPC5L genes, which are candidates for the somatotroph-restricted dysfunction. The reported novel mechanism for familial growth hormone deficiency demonstrates that general mRNA processing defects of the minor spliceosome can lead to very narrow tissue-specific consequences. Subject Categories Genetics, Gene Therapy ' Genetic Disease; Metabolism PMID:24480542

  13. Defective minor spliceosome mRNA processing results in isolated familial growth hormone deficiency.

    PubMed

    Argente, Jesús; Flores, Raquel; Gutiérrez-Arumí, Armand; Verma, Bhupendra; Martos-Moreno, Gabriel Á; Cuscó, Ivon; Oghabian, Ali; Chowen, Julie A; Frilander, Mikko J; Pérez-Jurado, Luis A

    2014-03-01

    The molecular basis of a significant number of cases of isolated growth hormone deficiency remains unknown. We describe three sisters affected with severe isolated growth hormone deficiency and pituitary hypoplasia caused by biallelic mutations in the RNPC3 gene, which codes for a minor spliceosome protein required for U11/U12 small nuclear ribonucleoprotein (snRNP) formation and splicing of U12-type introns. We found anomalies in U11/U12 di-snRNP formation and in splicing of multiple U12-type introns in patient cells. Defective transcripts include preprohormone convertases SPCS2 and SPCS3 and actin-related ARPC5L genes, which are candidates for the somatotroph-restricted dysfunction. The reported novel mechanism for familial growth hormone deficiency demonstrates that general mRNA processing defects of the minor spliceosome can lead to very narrow tissue-specific consequences.

  14. [Vitamin deficiencies in breastfed children due to maternal dietary deficiency].

    PubMed

    Kollée, L A A

    2006-03-04

    Dietary deficiencies of vitamin B12 and vitamin D during pregnancy and lactation may result in health problems in exclusively breastfed infants. Vitamin-B12 deficiency in these infants results in irritability, anorexia and failure to thrive during the first 4-8 months of life. Severe and permanent neurodevelopmental disturbances may occur. The most at risk for vitamin-B12 deficiency are breast-fed infants ofveganist and vegetarian mothers. Mothers who cover their skin prevent exposure to the sun and may consequently be at risk for vitamin-D deficiency, as well as putting their offspring at risk. In prenatal and perinatal care, it is important to take the maternal dietary history in order to be able to prevent or treat these disorders. Guidelines for obstetrical and neonatal care should include the topic of vitamin deficiency.

  15. Predicting the Probability of Abnormal Stimulated Growth Hormone Response in Children After Radiotherapy for Brain Tumors

    SciTech Connect

    Hua Chiaho; Wu Shengjie; Chemaitilly, Wassim; Lukose, Renin C.; Merchant, Thomas E.

    2012-11-15

    Purpose: To develop a mathematical model utilizing more readily available measures than stimulation tests that identifies brain tumor survivors with high likelihood of abnormal growth hormone secretion after radiotherapy (RT), to avoid late recognition and a consequent delay in growth hormone replacement therapy. Methods and Materials: We analyzed 191 prospectively collected post-RT evaluations of peak growth hormone level (arginine tolerance/levodopa stimulation test), serum insulin-like growth factor 1 (IGF-1), IGF-binding protein 3, height, weight, growth velocity, and body mass index in 106 children and adolescents treated for ependymoma (n = 72), low-grade glioma (n = 28) or craniopharyngioma (n = 6), who had normal growth hormone levels before RT. Normal level in this study was defined as the peak growth hormone response to the stimulation test {>=}7 ng/mL. Results: Independent predictor variables identified by multivariate logistic regression with high statistical significance (p < 0.0001) included IGF-1 z score, weight z score, and hypothalamic dose. The developed predictive model demonstrated a strong discriminatory power with an area under the receiver operating characteristic curve of 0.883. At a potential cutoff point of probability of 0.3 the sensitivity was 80% and specificity 78%. Conclusions: Without unpleasant and expensive frequent stimulation tests, our model provides a quantitative approach to closely follow the growth hormone secretory capacity of brain tumor survivors. It allows identification of high-risk children for subsequent confirmatory tests and in-depth workup for diagnosis of growth hormone deficiency.

  16. Circulating thyroid hormone levels in children

    PubMed Central

    Corcoran, J. M.; Eastman, C. J.; Carter, J. N.; Lazarus, L.

    1977-01-01

    Extensive use of radioimmunoassay for routine measurement of serum thyroid hormones in paediatric thyroid disorders showed inconsistencies between laboratory results based upon adult criteria and clinical observation. To resolve this disparity, serum triiodothyronine (T3) and thyroxine (T4) levels were measured by radioimmunoassay in 354 healthy children aged between 3 weeks and 17 years. The mean serum T3 concentration in children up to 10 years of age was 1·94±0·35 ng/ml (SD) which was higher than the mean serum T3 of 1·37±0·25 ng/ml in healthy adults. Similarly, the mean serum T4 of 10±2·5 μg/100 ml was higher than the adult mean serum T4 of 8·5±1·5 μg/100 ml. Neither concentration changed significantly from 3 weeks to 10 years of age, nor was there any sex difference. In girls serum T3 and T4 concentrations declined gradually from age 10 to maturity. A perimenarcheal nadir observed in the T4 data was thought to reflect the joint effects of the age-dependent fall in circulating T4 and the concomitant oestrogen-dependent rise in thyroxine-binding globulin. In boys the decline in serum T3 occurred approximately 2 years later than in the girls. These observations show that the normal ranges for serum T3 and T4 in children are higher than those in adults and that reference to normal adult ranges may lead to misclassification in diagnosis and monitoring of paediatric thyroid disorders. PMID:921322

  17. Model of pediatric pituitary hormone deficiency separates the endocrine and neural functions of the LHX3 transcription factor in vivo

    PubMed Central

    Colvin, Stephanie C.; Malik, Raleigh E.; Showalter, Aaron D.; Sloop, Kyle W.; Rhodes, Simon J.

    2011-01-01

    The etiology of most pediatric hormone deficiency diseases is poorly understood. Children with combined pituitary hormone deficiency (CPHD) have insufficient levels of multiple anterior pituitary hormones causing short stature, metabolic disease, pubertal failure, and often have associated nervous system symptoms. Mutations in developmental regulatory genes required for the specification of the hormone-secreting cell types of the pituitary gland underlie severe forms of CPHD. To better understand these diseases, we have created a unique mouse model of CPHD with a targeted knockin mutation (Lhx3 W227ter), which is a model for the human LHX3 W224ter disease. The LHX3 gene encodes a LIM-homeodomain transcription factor, which has essential roles in pituitary and nervous system development in mammals. The introduced premature termination codon results in deletion of the carboxyl terminal region of the LHX3 protein, which is critical for pituitary gene activation. Mice that lack all LHX3 function do not survive beyond birth. By contrast, the homozygous Lhx3 W227ter mice survive, but display marked dwarfism, thyroid disease, and female infertility. Importantly, the Lhx3 W227ter mice have no apparent nervous system deficits. The Lhx3 W227ter mouse model provides a unique array of hormone deficits and facilitates experimental approaches that are not feasible with human patients. These experiments demonstrate that the carboxyl terminus of the LHX3 transcription factor is not required for viability. More broadly, this study reveals that the in vivo actions of a transcription factor in different tissues are molecularly separable. PMID:21149718

  18. Glucocorticoid-Induced Osteoporosis in Children with 21-Hydroxylase Deficiency

    PubMed Central

    Ventura, Annamaria; Brunetti, Giacomina; Colucci, Silvia; Oranger, Angela; Ladisa, Filomena; Cavallo, Luciano; Grano, Maria; Faienza, Maria Felicia

    2013-01-01

    21-Hydroxylase deficiency (21-OHD) is the most common cause of congenital adrenal hyperplasia (CAH), resulting from deletions or mutations of the P450 21-hydroxylase gene (CYP21A2). Children with 21-OHD need chronic glucocorticoid (cGC) therapy, both to replace congenital deficit in cortisol synthesis and to reduce androgen secretion by adrenal cortex. GC-induced osteoporosis (GIO) is the most common form of secondary osteoporosis that results in an early, transient increase in bone resorption accompanied by a decrease in bone formation, maintained for the duration of GC therapy. Despite the conflicting results in the literature about the bone status on GC-treated patients with 21-OHD, many reports consider these subjects to be at risk for osteoporosis and fractures. In bone cells, at the molecular level, GCs regulate various functions including osteoblastogenesis, osteoclastogenesis, and the apoptosis of osteoblasts and osteocytes. In this paper, we focus on the physiology and biosynthesis of endogenous steroid hormones as well as on the effects of GCs on bone cells, highlighting the pathogenetic mechanism of GIO in children with 21-OHD. PMID:23484098

  19. Glucocorticoid-induced osteoporosis in children with 21-hydroxylase deficiency.

    PubMed

    Ventura, Annamaria; Brunetti, Giacomina; Colucci, Silvia; Oranger, Angela; Ladisa, Filomena; Cavallo, Luciano; Grano, Maria; Faienza, Maria Felicia

    2013-01-01

    21-Hydroxylase deficiency (21-OHD) is the most common cause of congenital adrenal hyperplasia (CAH), resulting from deletions or mutations of the P450 21-hydroxylase gene (CYP21A2). Children with 21-OHD need chronic glucocorticoid (cGC) therapy, both to replace congenital deficit in cortisol synthesis and to reduce androgen secretion by adrenal cortex. GC-induced osteoporosis (GIO) is the most common form of secondary osteoporosis that results in an early, transient increase in bone resorption accompanied by a decrease in bone formation, maintained for the duration of GC therapy. Despite the conflicting results in the literature about the bone status on GC-treated patients with 21-OHD, many reports consider these subjects to be at risk for osteoporosis and fractures. In bone cells, at the molecular level, GCs regulate various functions including osteoblastogenesis, osteoclastogenesis, and the apoptosis of osteoblasts and osteocytes. In this paper, we focus on the physiology and biosynthesis of endogenous steroid hormones as well as on the effects of GCs on bone cells, highlighting the pathogenetic mechanism of GIO in children with 21-OHD.

  20. [Trace elements deficiency in children receiving nutritional management].

    PubMed

    Masumoto, Kouji

    2016-07-01

    The trace elements is very important in growth of children, especially receiving nutritional management, including parenteral or enteral nutrition. Therefore, clinicians treating children should recognize regarding both the function and deficiency of trace elements. In this article, in nutritional management of children, the basic and recent knowledge was described regarding the function of some important trace elements, including zinc, copper, selenium, and iodine. In addition, the symptoms, diagnosis, and treatments in each trace element deficiency were also described.

  1. Muscle force and endurance in untreated and human growth hormone or insulin-like growth factor-I-treated patients with growth hormone deficiency or Laron syndrome.

    PubMed

    Brat, O; Ziv, I; Klinger, B; Avraham, M; Laron, Z

    1997-01-01

    Muscle force and endurance of four muscle groups (biceps, triceps, hamstrings and quadriceps) were measured by a computerized device in three groups: (A) 4 boys with isolated growth hormone deficiencies (IGHD) examined before at 10 and 24 months of hGH treatment; (B) 5 children (2 F, 3 M) with Laron syndrome were examined 3.5-4 years after initiation of insulin-like growth factor-I (IGF-I) treatment, and (C) comprised 8 untreated adults (5 F, 3 M) with Laron syndrome. For each patient, 2 matched controls, by age, sex, physical activity and height below the 50th percentile, were examined. GH- or IGF-I-deficient patients before treatment revealed reduced muscle force and endurance. GH treatment (0.6 U/kg/week) restored muscle force and endurance, progressively, mainly in the boys with puberty. Three to 4 years of IGF-I treatment (150 micrograms/kg/day) in patients with Laron syndrome proved to have a weaker effect than GH in restoring muscle force. The difference in effectiveness between hGH and IGF-I in restoring muscle force may be due to either the more marked muscle underdevelopment in Laron syndrome patients than in patients with IGHD or a difference in action potential between the two hormones.

  2. Consequences of monocarboxylate transporter 8 deficiency for renal transport and metabolism of thyroid hormones in mice.

    PubMed

    Trajkovic-Arsic, Marija; Visser, Theo J; Darras, Veerle M; Friesema, Edith C H; Schlott, Bernhard; Mittag, Jens; Bauer, Karl; Heuer, Heike

    2010-02-01

    Patients carrying inactivating mutations in the gene encoding the thyroid hormone transporting monocarboxylate transporter (MCT)-8 suffer from a severe form of psychomotor retardation and exhibit abnormal serum thyroid hormone levels. The thyroidal phenotype characterized by high-serum T(3) and low-serum T(4) levels is also found in mice mutants deficient in MCT8 although the cause of these abnormalities is still unknown. Here we describe the consequences of MCT8 deficiency for renal thyroid hormone transport, metabolism, and function by studying MCT8 null mice and wild-type littermates. Whereas serum and urinary parameters do not indicate a strongly altered renal function, a pronounced induction of iodothyronine deiodinase type 1 expression together with increased renal T(3) and T(4) content point to a general hyperthyroid state of the kidneys in the absence of MCT8. Surprisingly, accumulation of peripherally injected T(4) and T(3) into the kidneys was found to be enhanced in the absence of MCT8, indicating that MCT8 deficiency either directly interferes with the renal efflux of thyroid hormones or activates indirectly other renal thyroid hormone transporters that preferentially mediate the renal uptake of thyroid hormones. Our findings indicate that the enhanced uptake and accumulation of T(4) in the kidneys of MCT8 null mice together with the increased renal conversion of T(4) into T(3) by increased renal deiodinase type 1 activities contributes to the generation of the low-serum T(4) and the increase in circulating T(3) levels, a hallmark of MCT8 deficiency.

  3. Three Novel Missense Mutations within the LHX4 Gene Are Associated with Variable Pituitary Hormone Deficiencies

    PubMed Central

    Pfaeffle, Roland W.; Hunter, Chad S.; Savage, Jesse J.; Duran-Prado, Mario; Mullen, Rachel D.; Neeb, Zachary P.; Eiholzer, Urs; Hesse, Volker; Haddad, Nadine G.; Stobbe, Heike M.; Blum, Werner F.; Weigel, Johannes F. W.; Rhodes, Simon J.

    2008-01-01

    Context: The LHX4 LIM-homeodomain transcription factor has essential roles in pituitary gland and nervous system development. Heterozygous mutations in LHX4 are associated with combined pituitary hormone deficiency. Objectives: Our objectives were to determine the nature and frequency of LHX4 mutations in patients with pituitary hormone deficiency and to examine the functional outcomes of observed mutations. Design: The LHX4 gene sequence was determined from patient DNA. The biochemical and gene regulatory properties of aberrant LHX4 proteins were characterized using structural predictions, pituitary gene transcription assays, and DNA binding experiments. Patients: A total of 253 patients from 245 pedigrees with GH deficiency and deficiency of at least one additional pituitary hormone was included in the study. Results: In five patients, three types of heterozygous missense mutations in LHX4 that result in substitution of conserved amino acids were identified. One substitution is between the LIM domains (R84C); the others are in the homeodomain (L190R; A210P). The patients have GH deficiency; some also display reductions in TSH, LH, FSH, or ACTH, and aberrant pituitary morphology. Structural models predict that the aberrant L190R and A210P LHX4 proteins would have impaired DNA binding and gene activation properties. Consistent with these models, EMSAs and transfection experiments using pituitary gene promoters demonstrate that whereas the R84C form has reduced activity, the L190R and A210P proteins are inactive. Conclusions: LHX4 mutations are a relatively rare cause of combined pituitary hormone deficiency. This report extends the range of phenotypes associated with LHX4 gene mutations and describes three novel exonic mutations in the gene. PMID:18073311

  4. Iron Deficiency in Preschool Children with Autistic Spectrum Disorders

    ERIC Educational Resources Information Center

    Bilgic, Ayhan; Gurkan, Kagan; Turkoglu, Serhat; Akca, Omer Faruk; Kilic, Birim Gunay; Uslu, Runa

    2010-01-01

    Iron deficiency (ID) causes negative outcomes on psychomotor and behavioral development of infants and young children. Children with autistic spectrum disorders (ASD) are under risk for ID and this condition may increase the severity of psychomotor and behavioral problems, some of which already inherently exist in these children. In the present…

  5. Effects of growth hormone on growth performance, haematology, metabolites and hormones in iron-deficient veal calves.

    PubMed

    Ceppi, A; Blum, J W

    1994-08-01

    Effects of subcutaneous (s.c.) administration of 50 micrograms/kg body weight of recombinant bovine growth hormone (rbGH) or saline were studied for 11 weeks in 40 intact male veal calves supplied 50 mg or 10 mg of iron (Fe)/kg of milk replacer (MR). Feed intake, average daily gain and growth: feed ratio were reduced in Fe-deficient calves, but not significantly influenced by rbGH. Plasma Fe and haemoglobin concentration, red-cell number and packed cell volume were decreased in Fe-deficient calves (P < 0.05) and rbGH further reduced red-cell number in Fe-deficient calves (P < 0.05). The age-dependent increase of total Fe binding capacity was greater in Fe-deficient calves and enhanced by rbGH (P < 0.05). Plasma urea concentrations increased, whereas glucose (G) and triiodothyronine (T3) levels decreased in Fe-deficient calves. rbGH significantly increased G in calves fed MR containing 50 mg/kg (P < 0.05) and influenced urea concentrations (P < 0.05). Plasma insulin (I) and IGF-I concentrations were lower in Fe-deficient calves (P < 0.05). Plasma GH in the first hours after rbGH injections increased (P < 0.05) to higher levels in calves fed 10 than in those fed 50 mg Fe/kg MR, but incremental changes were comparable. In conclusion, low Fe intake caused haematologic, metabolic and endocrine changes. Plasma IGF-I, I and T3 concentrations after rbGH administration and effects of rbGH on IGF-I in Fe-deficient calves were reduced, even though plasma GH levels were increased.

  6. Glucose-6-phosphate dehydrogenase deficiency in Nigerian children.

    PubMed

    Williams, Olatundun; Gbadero, Daniel; Edowhorhu, Grace; Brearley, Ann; Slusher, Tina; Lund, Troy C

    2013-01-01

    Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzymopathy and in Sub-Saharan Africa, is a significant cause of infection- and drug-induced hemolysis and neonatal jaundice. Our goals were to determine the prevalence of G6PD deficiency among Nigerian children of different ethnic backgrounds and to identify predictors of G6PD deficiency by analyzing vital signs and hematocrit and by asking screening questions about symptoms of hemolysis. We studied 1,122 children (561 males and 561 females) aged 1 month to 15 years. The mean age was 7.4 ± 3.2 years. Children of Yoruba ethnicity made up the largest group (77.5%) followed by those Igbo descent (10.6%) and those of Igede (10.2%) and Tiv (1.8%) ethnicity. G6PD status was determined using the fluorescent spot method. We found that the overall prevalence of G6PD deficiency was 15.3% (24.1% in males, 6.6% in females). Yoruba children had a higher prevalence (16.9%) than Igede (10.5%), Igbo (10.1%) and Tiv (5.0%) children. The odds of G6PD deficiency were 0.38 times as high in Igbo children compared to Yoruba children (p=0.0500). The odds for Igede and Tiv children were not significantly different from Yoruba children (p=0.7528 and 0.9789 respectively). Mean oxygen saturation, heart rate and hematocrit were not significantly different in G6PD deficient and G6PD sufficient children. The odds of being G6PD deficient were 2.1 times higher in children with scleral icterus than those without (p=0.0351). In conclusion, we determined the prevalence of G6PD deficiency in Nigerian sub-populations. The odds of G6PD deficiency were decreased in Igbo children compared to Yoruba children. There was no association between vital parameters or hematocrit and G6PD deficiency. We found that a history of scleral icterus may increase the odds of G6PD deficiency, but we did not exclude other common causes of icterus such as sickle cell disease or malarial infection.

  7. Vitamin D deficiency in refugee children from conflict zones.

    PubMed

    Sheikh, Mohamud; Wang, Shu; Pal, Abhijit; MacIntyre, C Raina; Wood, Nicholas; Gunesekera, Hasantha

    2011-02-01

    Vitamin D deficiency is common in newly resettled refugee children and is associated with significant morbidity including rickets. To determine risk factors and burden of vitamin D deficiency in newly resettled refugee children in Australia. A descriptive epidemiological study and survey on refugee children attending an outpatient general health clinic at the Children's Hospital Westmead, Sydney. 215 patients were examined (age range 0-17 years), (76%) majority were from Africa. Mean serum 25OHD level was 46 nmol/L (SD = 24) (sufficiency range 50-150 nmol/L). 40% had mild deficiency (26-50 nmol/L), 19% moderate deficiency (13-25 nmol/L) and 2% were severely deficient (<13 nmol/L). Deficiency was most common in East African (72%) and Middle Eastern (66%) refugees, children in early puberty (89%) and those living in Australia >6 months (71%). Deficient children were more likely to have had movement restrictions and longer time in hiding in their country of refuge (OR 3:1[CI 0.9-9.7], P = .062).

  8. The influence of growth hormone (GH) deficiency and GH replacement on quality of life in GH-deficient patients.

    PubMed

    Deijen, J B; van der Veen, E A

    1999-01-01

    The total absence of hormones such as cortisol or thyroxine causes death within weeks. Lack of estrogen or testosterone is followed by infertility and impaired sexual functioning. Relative deficiencies of almost all classical hormones have a substantial impact on quality of life (QOL). However, in contrast to virtually all aspects of metabolism, QOL is difficult to measure. Only recently have tests been developed to assess general QOL, whereas specific tests address those aspects of QOL affected only in specific situations or disease states. For example, in rheumatoid arthritis and other chronic disabling diseases, the use of measures of QOL to assess treatment modalities is almost routine. In diseases with overt metabolic disturbances attention is generally focused on changes in metabolic parameters and the issue of QOL is neglected. Although very few practising endocrinologists will not support the idea that they specialize in improving QOL, its assessment in patients with endocrinological disorders began only recently--in patients with growth hormone (GH) deficiency only 10 years ago. It became apparent that GH deficiency in adult life is unmistakably followed by changes in parameters that determine QOL. In adults with childhood-onset GH deficiency, the unemployment rate is higher and the marriage rate lower than in the general population. Another symbol of success in life, the possession of a driver's licence, is less frequently attained by these patients. Most patients with adult-onset GH deficiency score unfavourably in questionnaires such as the Nottingham Health Profile. GH substitution is now available on a scale large enough to enable studies to be made of the effects on QOL in adults. The first studies were reported in 1989. However, only in the last few years have studies appeared in which sufficient number of patients and sufficient length of treatment were reported to allow a more objective judgement of the effectiveness of GH substitution. Although

  9. Isolated Adrenocorticotropic Hormone or Thyrotropin Deficiency Following Mild Traumatic Brain Injury: Three Cases with Long-Term Follow-Up

    PubMed Central

    Baek, Cho-Ok; Kim, Yu Ji; Kim, Ji Hye

    2015-01-01

    Few studies have examined the clinical features and long-term outcomes of isolated pituitary hormone deficiencies after traumatic brain injury (TBI). Such deficiencies typically present at time intervals after TBI, especially after mild injuries such as concussions, which makes their diagnosis difficult without careful history taking. It is necessary to improve diagnosis and prevent life threatening or morbid conditions such as those that may occur in deficiencies of adrenocorticotropic hormone (ACTH) or thyroid-stimulating hormone (as known as thyrotropin, TSH), the two most important pituitary hormones in hypopituitarism treatment. Here, we report two cases of isolated ACTH deficiency and one case of isolated TSH deficiency. These patients presented at different time points after concussion and underwent long-term follow-ups. PMID:27169080

  10. Isolated Adrenocorticotropic Hormone or Thyrotropin Deficiency Following Mild Traumatic Brain Injury: Three Cases with Long-Term Follow-Up.

    PubMed

    Baek, Cho-Ok; Kim, Yu Ji; Kim, Ji Hye; Park, Ji Hyun

    2015-10-01

    Few studies have examined the clinical features and long-term outcomes of isolated pituitary hormone deficiencies after traumatic brain injury (TBI). Such deficiencies typically present at time intervals after TBI, especially after mild injuries such as concussions, which makes their diagnosis difficult without careful history taking. It is necessary to improve diagnosis and prevent life threatening or morbid conditions such as those that may occur in deficiencies of adrenocorticotropic hormone (ACTH) or thyroid-stimulating hormone (as known as thyrotropin, TSH), the two most important pituitary hormones in hypopituitarism treatment. Here, we report two cases of isolated ACTH deficiency and one case of isolated TSH deficiency. These patients presented at different time points after concussion and underwent long-term follow-ups.

  11. An unusual combination of Klinefelter syndrome and growth hormone deficiency in a prepubertal child.

    PubMed

    Ramesh, Jayanthy; Nagasatyavani, Mudiganti; Venkateswarlu, Javvadii; Nagender, Jakka

    2014-09-01

    Klinefelter syndrome (KS) is the most common chromosomal aneuploidy in males. It is very difficult to diagnose this disorder in childhood due to absence of significant manifestations before puberty. These patients usually present with tall stature. We report a case of KS with short stature due to growth hormone deficiency. The boy's height was below the 3rd centile with significant delay in bone age. He responded well to growth hormone injections. In view of mental subnormality karyotyping was done, which revealed KS (47XXY).

  12. Iron deficiency anemia from diagnosis to treatment in children

    PubMed Central

    Özdemir, Nihal

    2015-01-01

    Iron deficiency is the most common nutritional deficiency worldwide and an important public health problem especially in developing countries. Since the most important indicator of iron deficieny is anemia, the terms “iron deficiency” and “iron deficiency anemia” are often used interchangeably. However, iron deficiency may develop in the absence of anemia and the tissues may be affected from this condition. The most common causes of iron deficiency in children include insufficient intake together with rapid growth, low birth weight and gastrointestinal losses related to excessive intake of cow’s milk. If insufficient intake can be excluded and there is insufficient response to oral iron treatment in patients with iron deficiency especially in older children, blood loss should be considered as the underlying cause. The main principles in management of iron deficiency anemia include investigation and elimination of the cause leading to iron deficiency, replacement of deficiency, improvement of nutrition and education of the patient and family. In this article, the practical approaches in the diagnosis and treatment of iron deficiency and the experience of our center have been reviewed. PMID:26078692

  13. Effect of copper deficiency on the content and secretion of pancreatic islet hormones

    SciTech Connect

    Bhathena, S.J.; Voyles, N.R.; Timmers, K.I.; Fields, M.; Kennedy, B.W.; Recant, L.

    1986-03-01

    Experimental copper (Cu) deficiency in rats is characterized by glucose intolerance and hyperlipemia. Its severity is increased by dietary fructose (F) as compared to starch (S). Since islet hormones are intimately involved in carbohydrate metabolism the authors studied the effects of Cu deficiency on their content and secretion. Rats were fed Cu deficient (CuD) (0.6 ..mu..g Cu/g) or Cu supplemented (6.0 ..mu..g Cu/g) diets with either 62% F or S for 7 weeks after weaning. Feeding CuD diets decreased plasma insulin (I) (P < 0.001) but not plasma glucagon (G). F feeding compared to S magnified the effects of Cu deficiency. Total pancreatic content of I in CuD rats was increased threefold (P < 0.001). Total somatostatin content increased significantly only in the pancreas of CuD rats fed F. Although total G content was not altered in CuD rats, when G was expressed per g protein or g wet weight, significant increases were found in CuD rats fed F. Thus, of the islet hormones, the major effect of Cu deficiency was on I. When pancreata were perfused in vitro with high glucose, pancreas from CuD rats had reduced insulin response. Thus, cellular functions dependent on Cu are involved in maintaining the ability of the islets of Langerhans to secrete I in a normal fashion.

  14. Cat eye syndrome and growth hormone deficiency with pituitary anomalies: a case report and review of the literature.

    PubMed

    Melo, Cláudia; Gama-de-Sousa, Susana; Almeida, Filipa; Rendeiro, Paula; Tavares, Purificação; Cardoso, Helena; Carvalho, Sónia

    2013-10-15

    Cat eye syndrome is a rare congenital disease characterized by the existence of a supernumerary chromosome derived from chromosome 22, with a variable phenotype comprising anal atresia, coloboma of the iris and preauricular tags or pits. We report a girl with cat eye syndrome, presenting short stature, with growth hormone deficiency due to posterior pituitary ectopia. Short stature is a common feature of this syndrome, and the association with a structural pituitary anomaly has been described, however growth hormone deficiency and the underlying mechanisms are rarely reported. A review on short stature and growth hormone deficiency in cat eye syndrome is conducted.

  15. Iron deficiency in young Bradford children from different ethnic groups.

    PubMed Central

    Ehrhardt, P

    1986-01-01

    Haematological parameters and iron state were studied in children admitted to hospital consecutively during a six month period. A total of 147 of 598 children (24.6%) were anaemic, with haemoglobin values below the third centile of the reference range, and 131 of 400 children (32.8%) were iron deficient, with serum ferritin concentrations less than 10 micrograms/l. Both findings were more common in children from the Asian ethnic minority. The "routine" full blood count is a useful tool for the presumptive identification of iron deficiency in childhood. Iron deficiency is deleterious to the health of young children. In view of its extent and degree--not exclusively among the Asian ethnic minority--a community based preventive programme on the lines of the Stop Rickets Campaign is recommended. PMID:3080103

  16. Linkage of congenital isolated adrenocorticotropic hormone deficiency to the corticotropin releasing hormone locus using simple sequence repeat polymorphisms

    SciTech Connect

    Kyllo, J.H.; Collins, M.M.; Vetter, K.L.

    1996-03-29

    Genetic screening techniques using simple sequence repeat polymorphisms were applied to investigate the molecular nature of congenital isolated adrenocorticotropic hormone (ACTH) deficiency. We hypothesize that this rare cause of hypocortisolism shared by a brother and sister with two unaffected sibs and unaffected parents is inherited as an autosomal recessive single gene mutation. Genes involved in the hypothalamic-pituitary axis controlling cortisol sufficiency were investigated for a causal role in this disorder. Southern blotting showed no detectable mutations of the gene encoding pro-opiomelanocortin (POMC), the ACTH precursor. Other candidate genes subsequently considered were those encoding neuroendocrine convertase-1, and neuroendocrine convertase-2 (NEC-1, NEC-2), and corticotropin releasing hormone (CRH). Tests for linkage were performed using polymorphic di- and tetranucleotide simple sequence repeat markers flanking the reported map locations for POMC, NEC-1, NEC-2, and CRH. The chromosomal haplotypes determined by the markers flanking the loci for POMC, NEC-1, and NEC-2 were not compatible with linkage. However, 22 individual markers defining the chromosomal haplotypes flanking CRH were compatible with linkage of the disorder to the immediate area of this gene of chromosome 8. Based on these data, we hypothesize that the ACTH deficiency in this family is due to an abnormality of CRH gene structure or expression. These results illustrate the useful application of high density genetic maps constructed with simple sequence repeat markers for inclusion/exclusion studies of candidate genes in even very small nuclear families segregating for unusual phenotypes. 25 refs., 5 figs., 2 tabs.

  17. Stories of experiences of care for growth hormone deficiency: the CRESCERE project

    PubMed Central

    Marini, Maria G; Chesi, Paola; Mazzanti, Laura; Guazzarotti, Laura; Toni, Teresa D; Salerno, Maria C; Officioso, Annunziata; Parpagnoli, Maria; Angeletti, Cristina; Faienza, Maria F; Iezzi, Maria L; Aversa, Tommaso; Sacchetti, Cinzia

    2016-01-01

    Aims: Growth hormone deficiency therapy is demanding for patients and caregivers. Teams engaged in the clinical management of growth hormone deficiency therapy need to know how families live with this condition, to provide an adequate support and prevent the risk of withdrawal from therapy. Methods: Using Narrative Medicine, testimonies from patients, their parents and providers of care were collected from 11 Italian centers. Narrations were analyzed throughout an elaboration of recurring words and expressions. Results: Although care management and outcomes were considered satisfying in the 182 collected narratives, recurring signals of intolerance among adolescents and the worry of not being well informed about side effects among parents are open issues. Conclusion: Narratives found that communication issues could decrease adherence and influence the physicians’ clinical practice. PMID:28031934

  18. Isolated adrenocorticotropic hormone deficiency due to probable lymphocytic hypophysitis in a woman

    PubMed Central

    Kacem, Faten Hadj; Charfi, Nadia; Mnif, Mouna Feki; Kamoun, Mahdi; Akid, Faouzi; Mnif, Fatma; Naceur, Basma Ben; Rekik, Nabila; Mnif, Zainab; Abid, Mohamed

    2013-01-01

    We report a 22-year-old woman who presented with asthenia, weight loss and hypotension in which extensive pituitary and adrenal investigations were diagnostic of isolated adrenocorticotropic hormone deficiency (IAD) of pituitary origin. Magnetic resonance imaging of the hypothalamus and pituitary showed a normal-sized pituitary, with no mass lesion. The diagnosis of IAD probably secondary to lymphocytic hypophysitis (LYH) was made. IAD is able to be the way of presentation of LYH, although the disease could or could not turn into a panhypopituitarism. Prompt recognition of this potentially fatal condition is important because of the availability of effective treatment. Indeed, regular endocrine and imaging follow up is important for patients with IAD and normal initial pituitary imaging results to detect early new-onset pituitary hormones deficiencies or imaging abnormalities. PMID:24251125

  19. Placenta passage of the thyroid hormone analog DITPA to male wild-type and Mct8-deficient mice.

    PubMed

    Ferrara, Alfonso Massimiliano; Liao, Xiao-Hui; Gil-Ibáñez, Pilar; Bernal, Juan; Weiss, Roy E; Dumitrescu, Alexandra M; Refetoff, Samuel

    2014-10-01

    Monocarboxylate transporter 8 (MCT8) deficiency causes severe X-linked intellectual and neuropsychological impairment associated with abnormal thyroid function tests (TFTs) producing thyroid hormone (TH) deprivation in brain and excess in peripheral tissues. The TH analog diiodothyropropionic acid (DITPA) corrected the TFTs abnormalities and hypermetabolism of MCT8-deficient children but did not improve the neurological phenotype. The latter result was attributed to the late initiation of treatment. Therefore, we gave DITPA to pregnant mice carrying Mct8-deficient embryos to determine whether DITPA, when given prenatally, crosses the placenta and affects the serum TFTs and cerebral cortex of embryos. After depletion of the endogenous TH, Mct8-heterozygous pregnant dams carrying both wild-type (Wt) and Mct8-deficient (Mct8KO) male embryos were given DITPA. Effects were compared with those treated with levothyroxine (L-T4). With DITPA treatment, serum DITPA concentration was not different in the two genotypes, which produced equal effect on serum TSH levels in both groups of pups. In contrast, with L-T4 treatment, TSH did not normalize in Mct8KO pups whereas it did in the Wt littermates and dams despite higher concentration of serum T4. Finally, both treatments similarly modulated the expression of the TH-dependent genes Shh, Klf9, and Aldh1a3 in brain. Thus, the ability of DITPA to cross the placenta, its thyromimetic action on the expression of TH-dependent genes in brain, and its better accessibility to the pituitary than L-T4, as assessed by serum TSH, make DITPA a candidate for the prenatal treatment of MCT8 deficiency.

  20. Placenta Passage of the Thyroid Hormone Analog DITPA to Male Wild-Type and Mct8-Deficient Mice

    PubMed Central

    Ferrara, Alfonso Massimiliano; Liao, Xiao-Hui; Gil-Ibáñez, Pilar; Bernal, Juan; Weiss, Roy E.; Dumitrescu, Alexandra M.

    2014-01-01

    Monocarboxylate transporter 8 (MCT8) deficiency causes severe X-linked intellectual and neuropsychological impairment associated with abnormal thyroid function tests (TFTs) producing thyroid hormone (TH) deprivation in brain and excess in peripheral tissues. The TH analog diiodothyropropionic acid (DITPA) corrected the TFTs abnormalities and hypermetabolism of MCT8-deficient children but did not improve the neurological phenotype. The latter result was attributed to the late initiation of treatment. Therefore, we gave DITPA to pregnant mice carrying Mct8-deficient embryos to determine whether DITPA, when given prenatally, crosses the placenta and affects the serum TFTs and cerebral cortex of embryos. After depletion of the endogenous TH, Mct8-heterozygous pregnant dams carrying both wild-type (Wt) and Mct8-deficient (Mct8KO) male embryos were given DITPA. Effects were compared with those treated with levothyroxine (L-T4). With DITPA treatment, serum DITPA concentration was not different in the two genotypes, which produced equal effect on serum TSH levels in both groups of pups. In contrast, with L-T4 treatment, TSH did not normalize in Mct8KO pups whereas it did in the Wt littermates and dams despite higher concentration of serum T4. Finally, both treatments similarly modulated the expression of the TH-dependent genes Shh, Klf9, and Aldh1a3 in brain. Thus, the ability of DITPA to cross the placenta, its thyromimetic action on the expression of TH-dependent genes in brain, and its better accessibility to the pituitary than L-T4, as assessed by serum TSH, make DITPA a candidate for the prenatal treatment of MCT8 deficiency. PMID:25051435

  1. Dose-Response Analysis of Developmental Iodide Deficiency: Reductions in Thyroid Hormones and Impaired Hippocampal Synaptic Transmission

    EPA Science Inventory

    Iodide is an essential nutrient for thyroid hormone synthesis and severe iodide deficiency (ID) during early development is associated with neurological impairments. Several environmental contaminants can perturb the thyroid axis and this perturbation may be more acute under cond...

  2. Fungal exposure endocrinopathy in sinusitis with growth hormone deficiency: Dennis-Robertson syndrome.

    PubMed

    Dennis, Donald; Robertson, David; Curtis, Luke; Black, Judson

    2009-01-01

    A retrospective study was carried out on 79 patients with a history of mold exposure, fatigue, and chronic rhinosinusitis (CRS) to determine whether there is a causal relationship between fungal exposure and chronic sinusitis, fatigue, and anterior hypopituitarism, especially growth hormone deficiency (GHD). Of the patients, 94% had a history of CRS, endoscopically and/or computed tomography (CT) confirmed; 100% had chronic fatigue and 100% had either significant history of indoor mold exposure and/or positive mold plate testing as measured by settle plates, with an average colony count of 21 (0-4 normal). A total of 62 had positive mold plate testing and 17 had positive history of mold exposure. Of 75, 73 (97.3%) had positive serum immunoglobulin G (IgG)-specific antibodies to fungal antigens. Out of 8, 7 were positive for urinary trichothecenes. Resting levels of insulin-like growth factor 1 (IGF-1) averaged 123 ng/mL (range 43-285, normal 88-249 ng/mL). Despite normal resting levels of IGF-1, significant deficiency of serum human growth hormone (GH) was confirmed by insulin tolerance test (ITT) in 40 of 50 tested. In all, 51% (40/79) were GH deficient. Primary or secondary hypothyroidism in T3 and/or T4 was seen in 81% (64/79) patients; 75% (59/79) had adrenocorticotrophic hormone (ACTH) deficiency. Fungal exposure endocrinopathy likely represents the major cause of GHD, affecting approximately 4.8 million people compared to approximately known 60,000 cases from all other causes. A literature review indicates a possible mechanism of GHD in fungal exposure is that the fungal glucan receptors in the lenticulostellate cells of the anterior pituitary bind to fungal cells wall glucans and activate the innate immune system, which activates macrophages that destroy the fungus and lenticulostellate tissue. Treatment of patients included normal saline nasal irrigations, antifungal and antibiotic nasal sprays, appropriate use of oral antibiotics and antifungals, facial

  3. Vitamin D deficiency among children with epilepsy in South Queensland.

    PubMed

    Fong, Choong Yi; Riney, Catherine J

    2014-03-01

    This study evaluated prevalence and risk factors for vitamin D deficiency among children with epilepsy on long-term antiepileptic drugs treated in South Queensland, Australia. Children with epilepsy seen in a tertiary neurology clinic were contacted requesting bone health blood tests during winter of 2011. Vitamin D deficiency was defined as 25-hydroxy vitamin D levels <20 ng/mL, and insufficiency between 21 and 29 ng/mL. One hundred thirty letters were sent, with 111 (85%) subsequently having blood tests performed. Vitamin D deficiency was identified in 24 (22%) of 111 and an additional 45 (41%) of 111 had vitamin D insufficiency. Multiple logistic regression analysis identified children on >2 antiepileptic drugs or with underlying genetic etiologies were more likely to have vitamin D deficiency. High proportion of children on long-term antiepileptic drugs in Queensland risk vitamin D deficiency and insufficiency despite living in the subtropics. Vitamin D monitoring and supplementation is important in the management of children on long-term antiepileptic drugs requiring tertiary care in Queensland.

  4. Interleukin 2 production in iron-deficient children.

    PubMed

    Galan, P; Thibault, H; Preziosi, P; Hercberg, S

    1992-01-01

    The relationship between iron status and capacity for IL-2 production by lymphocytes was assessed in 81 children from 6 mo to 3 yr of age selected at random from a population with low socioeconomic status, undergoing free systematic examination in four children's health centers in the Paris area. Iron deficiency was defined by the existence of at least two abnormal values among the three indicators of iron status: serum ferritin level less than or equal to 12 micrograms/L, transferrin saturation less than 12%, and erythrocyte protoporphyrin concentration greater than 3 micrograms/g hemoglobin. According to this definition, 53 children were classified as iron deficient and 28 as iron sufficient. No differences were observed between the iron-deficient and iron-sufficient groups in terms of the IL-2 concentration without stimulation by PHA. IL-2 production by lymphocytes stimulated with PHA, as well as the stimulation index (ratio of IL-2 concentration following stimulation by PHA to that of IL-2 concentration without stimulation by PHA) were significantly lower in iron-deficient children. The reduction in IL-2 production by activated lymphocytes observed in our study of iron-deficient children may be responsible for impairments in immunity found by other authors, particularly in cell-mediated immunity.

  5. Thyroid function and stress hormones in children with stress hyperglycemia.

    PubMed

    Bordbar, Mohammad Reza; Taj-Aldini, Reza; Karamizadeh, Zohre; Haghpanah, Sezaneh; Karimi, Mehran; Omrani, Gholam Hossein

    2012-12-01

    The purpose of the study is to determine the prevalence of stress hyperglycemia and to investigate how thyroid and stress hormones alter during stress hyperglycemia in children admitted to pediatric emergency wards. A prospective cross-sectional study was conducted in children, less than 19 years old, who were admitted to pediatric emergency wards of Nemazee and Dastgheib Hospitals, Shiraz, Southern Iran. Those patients taking steroids, beta-agonists or intravenously administered glucose before venipuncture, and patients with diabetes mellitus (DM) or thyroid diseases were excluded. Children with blood glucose ≥ 150 mg/dL during admission were regarded as cases. The controls were age- and- sex- matched, euglycemic children. Stress hormones including cortisol, insulin, growth hormone, and prolactin were measured, and thyroid function was tested with a radioimmunoassay (RIA) method in all cases and controls. The results showed that among 1,054 screened children, 39 cases (3.7 %) had stress hyperglycemia and 89 controls were included in the study. The occurrence of hyperglycemia was independent of sex, but it occurred mostly in children under 6 years old. Hyperglycemia occurred more frequently in patients with a positive family history of DM (odds ratio = 3.2, 95 % CI = 1.3-7.9, and P = 0.009). There were no significant differences between cases and controls regarding any hormones except higher cortisol, and lower total T3 and T4 in cases compared with controls. Neither of cases developed diabetes in the 24-month follow-up period. These findings led us to the conclusion that stress hyperglycemia is occasionally seen in critically ill patients. Among the stress hormones measured, only cortisol increased during hyperglycemia. It seems that hyperglycemia is not an important risk factor for future diabetes.

  6. Children with classic congenital adrenal hyperplasia have decreased amygdala volume: potential prenatal and postnatal hormonal effects.

    PubMed

    Merke, Deborah P; Fields, Jeremy D; Keil, Margaret F; Vaituzis, A Catherine; Chrousos, George P; Giedd, Jay N

    2003-04-01

    Children with classic congenital adrenal hyperplasia (CAH) have multiple endocrine imbalances, including prenatal glucocorticoid and adrenomedullary deficiency and androgen excess, with possible postnatal iatrogenic glucocorticoid excess, hyperandrogenism, and adrenomedullary hypofunction. Prenatal masculinization of the brain has been suggested in girls with classic CAH. Hormones of the hypothalamic-pituitary-adrenal axis and sex hormones interact with extrahypothalamic regulatory centers of the brain, including the amygdala and hippocampus. The amygdala is important in the processing of emotion and generation of fear, whereas the hippocampus plays an important role in memory. Chronic hypercortisolemia has been shown to be associated with hippocampal damage, while glucocorticoids and corticotropin-releasing factor play a major role in the regulation of amygdala function. We performed magnetic resonance imaging of the brain on 27 children with classic CAH and 47 sex- and age-matched controls. Volumes of the cerebrum, ventricles, temporal lobe, amygdala, and hippocampus were quantified. Females with CAH did not have brains with male-specific characteristics. In contrast, a significant decrease in amygdala volume was observed in both males and females with CAH (males, P = 0.01; females, P = 0.002). Iatrogenic effects on the hippocampus due to glucocorticoid therapy were not observed in children with CAH. These results suggest that prenatal glucocorticoid deficiency with resulting alterations in hypothalamic-pituitary-adrenal axis regulation, sex steroid excess, or some combination of these preferentially affect the growth and development of the amygdala, a structure with major functional implications that warrant further exploration.

  7. Iron deficiency occurs frequently in children with cystic fibrosis.

    PubMed

    Uijterschout, Lieke; Nuijsink, Marianne; Hendriks, Daniëlle; Vos, Rimke; Brus, Frank

    2014-05-01

    In adult CF patients iron deficiency (ID) is common and primarily functional due to chronic inflammation. No recent data are available on the cause of ID and iron deficiency anemia (IDA) in children with CF. Over the last decades onset of inflammation and pulmonary disease in children with CF is delayed by improved nutritional status. We questioned whether ID occurs in the same extent among children with CF as in adult CF patients. We therefore conducted a study to investigate the iron status of children with CF and to determine whether ID and IDA are associated with dietary iron intake, lung disease severity and Pseudomonas aeruginosa (PA) infection. Clinical charts of 53 children with CF aged 0-16 were reviewed. Follow-up varied from 1 to 14 years with 343 annual observations in total. Thirty-two children (60.4%) were iron deficient in at least 1 year and ID was present in 84 of 343 observations (24.5%). In 2011 ID was present in 9 children (17.0%). Ten children (18.9%) were anemic in at least 1 year and anemia was present in 13 of 328 observations (4.0%). IDA was present in at least 1 year in 6 children (11.3%). Ferritin (Fer) was positively associated with age. Higher Fer values found in older children represent an increased state of inflammation, rather than an improved iron status, and might increase the relative contribution of functional ID. This study shows that ID is common in relatively healthy, well-nourished children with CF. The mechanism of ID in children with CF is currently unknown. A prospective study using both soluble transferrin receptor and Fer as indicators for ID will provide more insight in the incidence and causes of ID in children with CF.

  8. High Prevalence of Vitamin B12 Deficiency and No Folate Deficiency in Young Children in Nepal

    PubMed Central

    Ng’eno, Bernadette N.; Perrine, Cria G.; Whitehead, Ralph D.; Subedi, Giri Raj; Mebrahtu, Saba; Dahal, Pradiumna; Jefferds, Maria Elena D.

    2017-01-01

    Many children in low- and middle-income countries may have inadequate intake of vitamin B12 and folate; data confirming these inadequacies are limited. We used biochemical, demographic, behavioral and anthropometric data to describe the folate and vitamin B12 concentrations among six- to 23-month-old Nepalese children. Vitamin B12 (serum B12 < 150 pmol/L) and folate deficiencies (red blood cell (RBC) folate < 226.5 nmol/L) were assessed. We used logistic regression to identify predictors of vitamin B12 deficiency. The vitamin B12 geometric mean was 186 pmol/L; 30.2% of children were deficient. The mean RBC folate concentration was 13,612 nmol/L; there was no deficiency. Factors associated with vitamin B12 deficiency included: (a) age six to 11 months (adjusted odds ratio (aOR) 1.51; 95% confidence interval (CI): 1.18, 1.92) or 12–17 months (aOR 1.38; 95% CI: 1.10, 1.72) compared to 18–23 months; (b) being stunted (aOR 1.24; 95% CI: 1.03, 1.50) compared to not being stunted; (c) and not eating animal-source foods (aOR 1.85; 95% CI: 1.42, 2.41) compared to eating animal-source foods the previous day. There was a high prevalence of vitamin B12 deficiency, but no folate deficiency. Improving early feeding practices, including the consumption of rich sources of vitamin B12, such as animal-source foods and fortified foods, may help decrease deficiency. PMID:28106733

  9. High Prevalence of Vitamin B12 Deficiency and No Folate Deficiency in Young Children in Nepal.

    PubMed

    Ng'eno, Bernadette N; Perrine, Cria G; Whitehead, Ralph D; Subedi, Giri Raj; Mebrahtu, Saba; Dahal, Pradiumna; Jefferds, Maria Elena D

    2017-01-17

    Many children in low- and middle-income countries may have inadequate intake of vitamin B12 and folate; data confirming these inadequacies are limited. We used biochemical, demographic, behavioral and anthropometric data to describe the folate and vitamin B12 concentrations among six- to 23-month-old Nepalese children. Vitamin B12 (serum B12 < 150 pmol/L) and folate deficiencies (red blood cell (RBC) folate < 226.5 nmol/L) were assessed. We used logistic regression to identify predictors of vitamin B12 deficiency. The vitamin B12 geometric mean was 186 pmol/L; 30.2% of children were deficient. The mean RBC folate concentration was 13,612 nmol/L; there was no deficiency. Factors associated with vitamin B12 deficiency included: (a) age six to 11 months (adjusted odds ratio (aOR) 1.51; 95% confidence interval (CI): 1.18, 1.92) or 12-17 months (aOR 1.38; 95% CI: 1.10, 1.72) compared to 18-23 months; (b) being stunted (aOR 1.24; 95% CI: 1.03, 1.50) compared to not being stunted; (c) and not eating animal-source foods (aOR 1.85; 95% CI: 1.42, 2.41) compared to eating animal-source foods the previous day. There was a high prevalence of vitamin B12 deficiency, but no folate deficiency. Improving early feeding practices, including the consumption of rich sources of vitamin B12, such as animal-source foods and fortified foods, may help decrease deficiency.

  10. Effects of aerobic exercise on ectopic lipids in patients with growth hormone deficiency before and after growth hormone replacement therapy

    PubMed Central

    Christ, Emanuel R.; Egger, Andrea; Allemann, Sabin; Buehler, Tania; Kreis, Roland; Boesch, Chris

    2016-01-01

    Growth hormone replacement therapy (GHRT) increases exercise capacity and insulin resistance while it decreases fat mass in growth hormone-deficient patients (GHD). Ectopic lipids (intramyocellular (IMCL) and intrahepatocellular lipids (IHCL) are related to insulin resistance. The effect of GHRT on ectopic lipids is unknown. It is hypothesized that exercise-induced utilization of ectopic lipids is significantly decreased in GHD patients and normalized by GHRT. GHD (4 females, 6 males) and age/gender/waist-matched control subjects (CS) were studied. VO2max was assessed on a treadmill and insulin sensitivity determined by a two-step hyperinsulinaemic-euglycaemic clamp. Visceral (VAT) and subcutaneous (SAT) fat were quantified by MR-imaging. IHCL and IMCL were measured before and after a 2 h exercise at 50–60% of VO2max using MR-spectroscopy (∆IMCL, ∆IHCL). Identical investigations were performed after 6 months of GHRT. VO2max was similar in GHD and CS and significantly increased after GHRT; GHRT significantly decreased SAT and VAT. 2 h-exercise resulted in a decrease in IMCL (significant in CS and GHRT) and a significant increase in IHCL in CS and GHD pre and post GHRT. GHRT didn’t significantly impact on ∆IMCL and ∆IHCL. We conclude that aerobic exercise affects ectopic lipids in patients and controls. GHRT increases exercise capacity without influencing ectopic lipids. PMID:26792091

  11. Efficacy and safety of growth hormone treatment in adults with growth hormone deficiency: a systematic review of studies on morbidity.

    PubMed

    van Bunderen, Christa C; van Varsseveld, Nadège C; Erfurth, Eva Marie; Ket, Johannes C F; Drent, Madeleine L

    2014-07-01

    Due to the positive effects demonstrated in randomized clinical trials on cardiovascular surrogate markers and bone metabolism, a positive effect of growth hormone (GH) treatment on clinically relevant end-points seems feasible. In this review, we discuss the long-term efficacy and safety of GH treatment in adult patients with growth hormone deficiency (GHD) with emphasis on morbidity: fatal and nonfatal cardiovascular disease (CVD) and stroke, fractures, fatal and nonfatal malignancies and recurrences, and diabetes mellitus. A positive effect of GH treatment on CVD and fracture risk could be concluded, but study design limitations have to be considered. Stroke and secondary brain tumours remained more prevalent. However, other contributing factors have to be taken into account. Regrowth and recurrences of (peri)pituitary tumours were not increased in patients with GH treatment compared to similar patients without GH treatment. All fatal and nonfatal malignancies were not more prevalent in GH-treated adults compared to the general population. However, follow-up time is still relatively short. The studies on diabetes are difficult to interpret, and more evidence is awaited. In clinical practice, a more individualized assessment seems appropriate, taking into consideration the underlying diagnosis of GHD, other treatment regimens, metabolic profile and the additional beneficial effects of GH set against the possible risks. Large and thoroughly conducted observational studies are needed and seem the only feasible way to inform the ongoing debate on health care costs, drug safety and clinical outcomes.

  12. Developing a service for children with iron deficiency anaemia.

    PubMed

    Bartle, Catherine

    The IDEAS (Iron Deficiency Early Anaemia Services) project is a programme aimed at detecting and offering a system of care for children with iron deficiency anaemia. It is designed and run by health visitors and nurses in an inner-city area of Bradford. Children with low haemoglobin are referred by the health visitor to the specialist nurse-led clinic. Dietary advice consistent with tradition and culture is given and appropriate referrals made to the GP for iron supplementation, and to the consultant paediatrician or haemoglobinopathy adviser when appropriate. An important development has been the identification of a previously undiagnosed abnormal haemoglobin trait.

  13. Deficiency of antidiuretic hormone: a rare cause of massive polyuria after kidney transplantation.

    PubMed

    Jang, Kyung Mi; Sohn, Young Soo; Hwang, Young Ju; Choi, Bong Seok; Cho, Min Hyun

    2016-04-01

    A 15-year-old boy, who was diagnosed with Alport syndrome and end-stage renal disease, received a renal transplant from a living-related donor. On postoperative day 1, his daily urine output was 10,000 mL despite normal graft function. His laboratory findings including urine, serum osmolality, and antidiuretic hormone levels showed signs similar to central diabetes insipidus, so he was administered desmopressin acetate nasal spray. After administering the desmopressin, urine specific gravity and osmolality increased abruptly, and daily urine output declined to the normal range. The desmopressin acetate was tapered gradually and discontinued 3 months later. Graft function was good, and urine output was maintained within the normal range without desmopressin 20 months after the transplantation. We present a case of a massive polyuria due to transient deficiency of antidiuretic hormone with the necessity of desmopressin therapy immediately after kidney transplantation in a pediatric patient.

  14. Is immune system-related hypertension associated with ovarian hormone deficiency?

    PubMed Central

    Sandberg, Kathryn; Ji, Hong; Einstein, Gillian; Au, April; Hay, Meredith

    2017-01-01

    The immune system is known to contribute to the development of high blood pressure in males. However, the role of the immune system in the development of high blood pressure in females and the role of ovarian hormones has only recently begun to be studied. In animal studies, both the sex of the host and the T cell are critical biological determinants of susceptibility and resistance to hypertension induced by angiotensin II. In women, natural menopause is known to result in significant changes in the expression of genes regulating the immune system. Likewise, in animal models, ovariectomy results in hypertension and an upregulation in T-cell tumour necrosis factor-α-related genes. Oestrogen replacement results in decreases in inflammatory genes in the brain regions involved in blood pressure regulation. Together, these studies suggest that the response of the adaptive immune system to ovarian hormone deficiency is a significant contributor to hypertension in women. PMID:26419911

  15. Growth Hormone Deficiency in a Child with Neurofibromatosis-Noonan Syndrome.

    PubMed

    Vurallı, Doğuş; Gönç, Nazlı; Vidaud, Dominique; Özön, Alev; Alikaşifoğlu, Ayfer; Kandemir, Nurgün

    2016-03-05

    Neurofibromatosis-Noonan syndrome (NFNS) is a distinct entity which shows the features of both NF1 (neurofibromatosis 1) and Noonan syndrome (NS). While growth hormone deficiency (GHD) has been relatively frequently identified in NF1 and NS patients, there is limited experience in NFNS cases. The literature includes only one case report of a NFNS patient having GHD and that report primarily focuses on the dermatological lesions that accompany the syndrome and not on growth hormone (GH) treatment. Here, we present a 13-year-old girl who had clinical features of NFNS with a mutation in the NF1 gene. The case is the first NFNS patient reported in the literature who was diagnosed to have GHD and who received GH treatment until reaching final height. The findings in this patient show that short stature is a feature of NFNS and can be caused by GHD. Patients with NFNS who show poor growth should be evaluated for GHD.

  16. Growth Hormone Deficiency in a Child with Neurofibromatosis-Noonan Syndrome

    PubMed Central

    Vurallı, Doğuş; Gönç, Nazlı; Vidaud, Dominique; Özön, Alev; Alikaşifoğlu, Ayfer; Kandemir, Nurgün

    2016-01-01

    Neurofibromatosis-Noonan syndrome (NFNS) is a distinct entity which shows the features of both NF1 (neurofibromatosis 1) and Noonan syndrome (NS). While growth hormone deficiency (GHD) has been relatively frequently identified in NF1 and NS patients, there is limited experience in NFNS cases. The literature includes only one case report of a NFNS patient having GHD and that report primarily focuses on the dermatological lesions that accompany the syndrome and not on growth hormone (GH) treatment. Here, we present a 13-year-old girl who had clinical features of NFNS with a mutation in the NF1 gene. The case is the first NFNS patient reported in the literature who was diagnosed to have GHD and who received GH treatment until reaching final height. The findings in this patient show that short stature is a feature of NFNS and can be caused by GHD. Patients with NFNS who show poor growth should be evaluated for GHD. PMID:26758488

  17. Targeting GH-1 splicing as a novel pharmacological strategy for growth hormone deficiency type II.

    PubMed

    Miletta, Maria Consolata; Flück, Christa E; Mullis, Primus-E

    2017-01-15

    Isolated growth hormone deficiency type II (IGHD II) is a rare genetic splicing disorder characterized by reduced growth hormone (GH) secretion and short stature. It is mainly caused by autosomal dominant-negative mutations within the growth hormone gene (GH-1) which results in missplicing at the mRNA level and the subsequent loss of exon 3, producing the 17.5-kDa GH isoform: a mutant and inactive GH protein that reduces the stability and the secretion of the 22-kDa GH isoform, the main biologically active GH form. At present, patients suffering from IGHD II are treated with daily injections of recombinant human GH (rhGH) in order to reach normal height. However, this type of replacement therapy, although effective in terms of growth, does not prevent the toxic effects of the 17.5-kDa mutant on the pituitary gland, which may eventually lead to other hormonal deficiencies. As the severity of the disease inversely correlates with the 17.5-kDa/22-kDa ratio, increasing the inclusion of exon 3 is expected to ameliorate disease symptoms. This review focuses on the recent advances in experimental and therapeutic strategies applicable to treat IGHD II in clinical and preclinical contexts. Several avenues for alternative IGHD II therapy will be discussed including the use of small interfering RNA (siRNA) and short hairpin RNA (shRNA) constructs that specifically target the exon 3-deleted transcripts as well as the application of histone deacetylase inhibitors (HDACi) and antisense oligonucleotides (AONs) to enhance full-length GH-1 transcription, correct GH-1 exon 3 splicing and manipulate GH pathway.

  18. Lactose intolerance and lactase deficiency in children.

    PubMed

    Rings, E H; Grand, R J; Büller, H A

    1994-10-01

    The term lactase deficiency is widely used to indicate a low or absent level of lactase enzyme in the small intestine, leading to lactose intolerance. This term is correctly used when the intestinal mucosa is damaged and results in secondary lactase deficiency. In the case of the genetically determined decrease of lactase activity during childhood, however, low lactase levels suggest that the majority of the world's population is "abnormal," whereas individuals from caucasian extraction with high levels of lactase enzyme throughout life are then considered "normal." It would be better to ascribe racial and ethnic lactose malabsorption as the result of genetically determined reduction of lactase activity, rather then implying an "abnormality" by the term, "deficiency." Recent studies reveal that this genetic control is at the transcriptional level. The symptomatology of lactose intolerance varies widely, and the diagnostic method of choice is the lactose breath hydrogen test in combination with clinical findings, although small intestinal biopsies should be performed when mucosal diseases are suspected. Treatment of lactose intolerance depends on the age of the child. In young infants complete restriction of lactose containing foods is rarely necessary.

  19. NFKB2 mutation in common variable immunodeficiency and isolated adrenocorticotropic hormone deficiency

    PubMed Central

    Shi, Chuan; Wang, Fen; Tong, Anli; Zhang, Xiao-Qian; Song, Hong-Mei; Liu, Zheng-Yin; Lyu, Wei; Liu, Yue-Hua; Xia, Wei-Bo

    2016-01-01

    Abstract Background Common variable immunodeficiency (CVID) with central adrenal insufficiency is a recently defined clinical syndrome caused by mutations in the nuclear factor kappa-B subunit 2 (NFKB2) gene. We present the first case of NFKB2 mutation in Asian population. Methods and Results An 18-year-old Chinese female with adrenocorticotropic hormone (ACTH) deficiency was admitted due to adrenal crisis and pneumonia. She had a history of recurrent respiratory infections since childhood and ectodermal abnormalities were noted during physical examination. Immunologic tests revealed panhypogammaglobulinemia and deficient natural killer (NK)-cell function. DNA sequencing of NFKB2 identified a heterozygous nonsense mutation (c.2563 A>T, p.855: Lys>∗) in the patient but not her parents. Conclusion Clinicians should be alert to comorbidities of adrenal insufficiency and ectodermal dysplasia in CVID patients as these might suggest a rare hereditary syndrome caused by NFKB2 mutation. PMID:27749582

  20. The serum level of 25-hydroxyvitamin D for maximal suppression of parathyroid hormone in children: the relationship between 25-hydroxyvitamin D and parathyroid hormone

    PubMed Central

    Kang, Jung In; Lee, Yoon Suk; Han, Ye Jin; Kong, Kyoung Ae

    2017-01-01

    Purpose Serum level of 25-hydroxyvitamin D (25-OHD) is considered as the most appropriate marker of vitamin D status. However, only a few studies have investigated the relationship between 25-OHD and parathyroid hormone (PTH) in children. To this end, this study was aimed at evaluating the lowest 25-OHD level that suppresses the production of parathyroid hormone in children. Methods A retrospective record review was performed for children aged 0.2 to 18 years (n=193; 106 boys and 87 girls) who underwent simultaneous measurements of serum 25-OHD and PTH levels between January 2010 and June 2014. Results The inflection point of serum 25-OHD level for maximal suppression of PTH was at 18.0 ng/mL (95% confidence interval, 14.3–21.7 ng/mL). The median PTH level of the children with 25-OHD levels of <18.0 ng/mL was higher than that of children with 25-OHD levels ≥ 18.0 ng/mL (P<0.0001). The median calcium level of children with 25-OHD levels<18.0 ng/mL was lower than that of children with 25-OHD levels≥18.0 ng/mL (P=0.0001). The frequency of hyperparathyroidism was higher in the children with 25-OHD levels<18.0 ng/mL than in the children with 25-OHD levels≥18.0 ng/mL (P<0.0001). Hypocalcemia was more prevalent in the children with 25-OHD levels<18.0 ng/mL than in the children with 25-OHD levels≥18.0 ng/mL (P<0.0001). Conclusion These data suggest that a vitamin D level of 18.0 ng/mL could be the criterion for 25-OHD deficiency in children at the inflection point of the maximal suppression of PTH. PMID:28289433

  1. A phase 2 trial of long-acting TransCon growth hormone in adult GH deficiency.

    PubMed

    Höybye, Charlotte; Pfeiffer, Andreas F H; Ferone, Diego; Christiansen, Jens Sandahl; Gilfoyle, David; Christoffersen, Eva Dam; Mortensen, Eva; Leff, Jonathan A; Beckert, Michael

    2017-04-01

    TransCon growth hormone is a sustained-release human growth hormone prodrug under development in which unmodified growth hormone is transiently linked to a carrier molecule. It is intended as an alternative to daily growth hormone in the treatment of growth hormone deficiency. This was a multi-center, randomized, open-label, active-controlled trial designed to compare the safety (including tolerability and immunogenicity), pharmacokinetics and pharmacodynamics of three doses of weekly TransCon GH to daily growth hormone (Omnitrope). Thirty-seven adult males and females diagnosed with adult growth hormone deficiency and stable on growth hormone replacement therapy for at least 3 months were, following a wash-out period, randomized (regardless of their pre-study dose) to one of three TransCon GH doses (0.02, 0.04 and 0.08 mg GH/kg/week) or Omnitrope 0.04 mg GH/kg/week (divided into 7 equal daily doses) for 4 weeks. Main outcomes evaluated were adverse events, immunogenicity and growth hormone and insulin-like growth factor 1 levels. TransCon GH was well tolerated; fatigue and headache were the most frequent drug-related adverse events and reported in all groups. No lipoatrophy or nodule formation was reported. No anti-growth hormone-binding antibodies were detected. TransCon GH demonstrated a linear, dose-dependent increase in growth hormone exposure without accumulation. Growth hormone maximum serum concentration and insulin-like growth factor 1 exposure were similar after TransCon GH or Omnitrope administered at comparable doses. The results suggest that long-acting TransCon GH has a profile similar to daily growth hormone but with a more convenient dosing regimen. These findings support further TransCon GH development.

  2. Psychological and electroencephalographic study in school children with iron deficiency.

    PubMed

    Otero, G A; Aguirre, D M; Porcayo, R; Fernández, T

    1999-08-01

    Two groups were chosen from a randomly selected group of one hundred 6-12 years old primary school children. One group was formed by iron deficient (ID), not anemic children, and a control group (C) by iron replete children. Both groups, matched by age, sex, and sociocultural level, were studied using WISC-R, a computerized test of learning (DEL) and a qEEG. The WISC-R test showed that ID children had significantly lower values in WISC items of information, comprehension and verbal, performance and full scale IQ than C children. On the other hand, the EEG power spectrum showed more theta energy in all leads using Laplacian montage and more delta energy in frontal areas using referential montage in ID than in C children. It was found that beside the well known effect of iron deficiency upon intellectual performance during childhood, the EEG power spectrum of ID children had a slower activity than in iron replete children suggesting a developmental lag and/or a CNS dysfunction.

  3. Growth hormone secretagogue receptor deficiency in mice protects against obesity‐induced hypertension

    PubMed Central

    Harris, Louise E.; Morgan, David G.; Balthasar, Nina

    2014-01-01

    Abstract Growth hormone secretagogue receptor (GHS‐R) signaling has been associated with growth hormone release, increases in food intake and pleiotropic cardiovascular effects. Recent data demonstrated that acute GHS‐R antagonism leads to increases in mean arterial pressure mediated by the sympathetic nervous system in rats; a highly undesirable effect if GHS‐R antagonism was to be used as a therapeutic approach to reducing food intake in an already obese, hypertensive patient population. However, our data in conscious, freely moving GHS‐R deficient mice demonstrate that chronic absence of GHS‐R signaling is protective against obesity‐induced hypertension. GHS‐R deficiency leads to reduced systolic blood pressure variability (SBPV); in response to acute high‐fat diet (HFD)‐feeding, increases in the sympathetic control of SBPV are suppressed in GHS‐R KO mice. Our data further suggest that GHS‐R signaling dampens the immediate HFD‐mediated increase in spontaneous baroreflex sensitivity. In diet‐induced obesity, absence of GHS‐R signaling leads to reductions in obesity‐mediated hypertension and tachycardia. Collectively, our findings thus suggest that chronic blockade of GHS‐R signaling may not result in adverse cardiovascular effects in obesity. PMID:24760503

  4. Impact of growth hormone replacement therapy on sleep in adult patients with growth hormone deficiency of pituitary origin

    PubMed Central

    Morselli, Lisa L.; Nedeltcheva, Arlet; Leproult, Rachel; Spiegel, Karine; Martino, Enio; Legros, Jean-Jacques; Weiss, Roy E.; Mockel, Jean; Van Cauter, Eve; Copinschi, Georges

    2013-01-01

    Objectives We previously reported that adult patients with GH deficiency (GHD) due to a confirmed or likely pituitary defect, as compared to healthy controls individually matched for age, gender and BMI, have more slow-wave sleep (SWS) and higher delta activity (a marker of SWS intensity). Here we examined the impact of recombinant human GH (rhGH) therapy, compared to placebo, on objective sleep quality in a subset of patients from the same cohort. Design Single-blind randomized cross-over design study. Methods Fourteen patients with untreated GHD of confirmed or likely pituitary origin, aged 22–74 yr, participated in the study. Patients with associated hormonal deficiencies were on appropriate replacement therapy. Polygraphic sleep recordings, with bedtimes individually tailored to habitual sleep times, were performed after 4 months on rhGH or placebo. Results Valid data were obtained in 13 patients. At the end of rhGH treatment period, patients had a shorter sleep period time than at the end of the placebo period (479±11 vs 431±19 min respectively; p=0.005), primarily due to an earlier wake up time, and a decrease in the intensity of SWS (delta activity) (559±125 vs 794±219 μV2, respectively; p=0.048). Conclusions Four months of rhGH replacement therapy partly reversed sleep disturbances previously observed in untreated patients. The decrease in delta activity associated with rhGH treatment adds further evidence to the hypothesis that the excess of high intensity SWS observed in untreated pituitary GHD patients is likely to result from overactivity of the hypothalamic GHRH system due to the lack of negative feedback inhibition by GH. PMID:23447518

  5. The Thyroid Hormone Analog DITPA Ameliorates Metabolic Parameters of Male Mice With Mct8 Deficiency.

    PubMed

    Ferrara, Alfonso Massimiliano; Liao, Xiao-Hui; Ye, Honggang; Weiss, Roy E; Dumitrescu, Alexandra M; Refetoff, Samuel

    2015-11-01

    Mutations in the gene encoding the thyroid hormone (TH) transporter, monocarboxylate transporter 8 (MCT8), cause mental retardation in humans associated with a specific thyroid hormone phenotype manifesting high serum T3 and low T4 and rT3 levels. Moreover, these patients have failure to thrive, and physiological changes compatible with thyrotoxicosis. Recent studies in Mct8-deficient (Mct8KO) mice revealed that the high serum T3 causes increased energy expenditure. The TH analog, diiodothyropropionic acid (DITPA), enters cells independently of Mct8 transport and shows thyromimetic action but with a lower metabolic activity than TH. In this study DITPA was given daily ip to adult Mct8KO mice to determine its effect on thyroid tests in serum and metabolism (total energy expenditure, respiratory exchange rate, and food and water intake). In addition, we measured the expression of TH-responsive genes in the brain, liver, and muscles to assess the thyromimetic effects of DITPA. Administration of 0.3 mg DITPA per 100 g body weight to Mct8KO mice brought serum T3 levels and the metabolic parameters studied to levels observed in untreated Wt animals. Analysis of TH target genes revealed amelioration of the thyrotoxic state in liver, somewhat in the soleus, but there was no amelioration of the brain hypothyroidism. In conclusion, at the dose used, DITPA mainly ameliorated the hypermetabolism of Mct8KO mice. This thyroid hormone analog is suitable for the treatment of the hypermetabolism in patients with MCT8 deficiency, as suggested in limited preliminary human trials.

  6. The Thyroid Hormone Analog DITPA Ameliorates Metabolic Parameters of Male Mice With Mct8 Deficiency

    PubMed Central

    Ferrara, Alfonso Massimiliano; Liao, Xiao-Hui; Ye, Honggang; Weiss, Roy E.; Dumitrescu, Alexandra M.

    2015-01-01

    Mutations in the gene encoding the thyroid hormone (TH) transporter, monocarboxylate transporter 8 (MCT8), cause mental retardation in humans associated with a specific thyroid hormone phenotype manifesting high serum T3 and low T4 and rT3 levels. Moreover, these patients have failure to thrive, and physiological changes compatible with thyrotoxicosis. Recent studies in Mct8-deficient (Mct8KO) mice revealed that the high serum T3 causes increased energy expenditure. The TH analog, diiodothyropropionic acid (DITPA), enters cells independently of Mct8 transport and shows thyromimetic action but with a lower metabolic activity than TH. In this study DITPA was given daily ip to adult Mct8KO mice to determine its effect on thyroid tests in serum and metabolism (total energy expenditure, respiratory exchange rate, and food and water intake). In addition, we measured the expression of TH-responsive genes in the brain, liver, and muscles to assess the thyromimetic effects of DITPA. Administration of 0.3 mg DITPA per 100 g body weight to Mct8KO mice brought serum T3 levels and the metabolic parameters studied to levels observed in untreated Wt animals. Analysis of TH target genes revealed amelioration of the thyrotoxic state in liver, somewhat in the soleus, but there was no amelioration of the brain hypothyroidism. In conclusion, at the dose used, DITPA mainly ameliorated the hypermetabolism of Mct8KO mice. This thyroid hormone analog is suitable for the treatment of the hypermetabolism in patients with MCT8 deficiency, as suggested in limited preliminary human trials. PMID:26322373

  7. Athletic induced iron deficiency: new insights into the role of inflammation, cytokines and hormones.

    PubMed

    Peeling, Peter; Dawson, Brian; Goodman, Carmel; Landers, Grant; Trinder, Debbie

    2008-07-01

    Iron is utilised by the body for oxygen transport and energy production, and is therefore essential to athletic performance. Commonly, athletes are diagnosed as iron deficient, however, contrasting evidence exists as to the severity of deficiency and the effect on performance. Iron losses can result from a host of mechanisms during exercise such as hemolysis, hematuria, sweating and gastrointestinal bleeding. Additionally, recent research investigating the anemia of inflammation during states of chronic disease has allowed us to draw some comparisons between unhealthy populations and athletes. The acute-phase response is a well-recognised reaction to both exercise and disease. Elevated cytokine levels from such a response have been shown to increase the liver production of the hormone Hepcidin. Hepcidin up-regulation has a negative impact on the iron transport and absorption channels within the body, and may explain a potential new mechanism behind iron deficiency in athletes. This review will attempt to explore the current literature that exits in this new area of iron metabolism and exercise.

  8. Mice deficient in MCT8 reveal a mechanism regulating thyroid hormone secretion.

    PubMed

    Di Cosmo, Caterina; Liao, Xiao-Hui; Dumitrescu, Alexandra M; Philp, Nancy J; Weiss, Roy E; Refetoff, Samuel

    2010-09-01

    The mechanism of thyroid hormone (TH) secretion from the thyroid gland into blood is unknown. Humans and mice deficient in monocarboxylate transporter 8 (MCT8) have low serum thyroxine (T4) levels that cannot be fully explained by increased deiodination. Here, we have shown that Mct8 is localized at the basolateral membrane of thyrocytes and that the serum TH concentration is reduced in Mct8-KO mice early after being taken off a treatment that almost completely depleted the thyroid gland of TH. Thyroid glands in Mct8-KO mice contained more non-thyroglobulin-associated T4 and triiodothyronine than did those in wild-type mice, independent of deiodination. In addition, depletion of thyroidal TH content was slower during iodine deficiency. After administration of 125I, the rate of both its secretion from the thyroid gland and its appearance in the serum as trichloroacetic acid-precipitable radioactivity was greatly reduced in Mct8-KO mice. Similarly, the secretion of T4 induced by injection of thyrotropin was reduced in Mct8-KO in which endogenous TSH and T4 were suppressed by administration of triiodothyronine. To our knowledge, this study is the first to demonstrate that Mct8 is involved in the secretion of TH from the thyroid gland and contributes, in part, to the low serum T4 level observed in MCT8-deficient patients.

  9. Carnitine deficiency in children receiving continuous renal replacement therapy.

    PubMed

    Sgambat, Kristen; Moudgil, Asha

    2016-01-01

    Carnitine deficiency is known to occur in chronic hemodialysis; however, the effect of continuous renal replacement therapy (CRRT) on carnitine homeostasis has not been studied. We hypothesized that children receiving CRRT are at risk for deficiency because of continuous removal, absent intake, decreased production, and comorbidities related to critical illness. Records of patients with acute kidney injury receiving CRRT at Children's National Health System between 2011 and 2014 were reviewed for total carnitine (TC), free carnitine (FC), feeding modality, severity of illness, and survival outcome. The proportion of carnitine-deficient patients at baseline, 1, 2, and ≥3 weeks on CRRT were compared by chi-square, and relationships with other variables assessed by Pearson's correlation and logistic regression. The study group included 42 CRRT patients, age 7.9 + 1.1 years. At baseline, 30.7% and 35.7% of patients were TC and FC deficient. Within 1 week, 64.5% (P = 0.03) and 70% (P = 0.03) were TC and FC deficient, and prevalence of deficiency increased to 80% (P = 0.01) and 90% (P = 0.008) by 2 weeks; 100% of patients were TC and FC deficient after being on CRRT for ≥3 weeks (P = 0.005 and P = 0.01, respectively, vs. baseline). TC and FC levels negatively correlated with days on CRRT (r = -0.39, P = 0.001 and r = -0.35, P = 0.005). Patients with TC and FC deficiency had 5.9 and 4.9 greater odds of death than those with normal levels (P = 0.02 and P = 0.03). Carnitine is significantly and rapidly depleted with longer time on CRRT, and carnitine deficiency is associated with increased mortality. Consequences of deficiency and benefits of supplementation in the pediatric CRRT population should be investigated.

  10. Obesity, diabetes and cancer: insight into the relationship from a cohort with growth hormone receptor deficiency.

    PubMed

    Guevara-Aguirre, Jaime; Rosenbloom, Arlan L

    2015-01-01

    Obesity with insulin-resistant diabetes and increased cancer risk is a global problem. We consider the alterations of metabolism attendant on the underlying pathogenic overnutrition and the role of the growth hormone (GH)-IGF-1 axis in this interaction. Obesity-induced insulin resistance is a determinant of diabetes. Excess glucose, and an elevated concentration of insulin acting through its own receptors along with complex interactions with the IGF-1 system, will add extra fuel and fuel signalling for malignant growth and induce anti-apoptotic activities, permitting proliferation of forbidden clones. In Ecuador there are ~100 living adults with lifelong IGF-1 deficiency caused by a GH receptor (GHR) mutation who, despite a high percentage of body fat, have markedly increased insulin sensitivity compared with age- and BMI-matched control relatives, and no instances of diabetes, which is present in 6% of unaffected relatives. Only 1 of 20 deceased individuals with GHR deficiency died of cancer vs 20% of ~1,500 relatives. Fewer DNA breaks and increased apoptosis occurred in cell cultures exposed to oxidant agents following addition of serum from GHR-deficient individuals vs serum from control relatives. These changes were reversible by adding IGF-1 to the serum from the GHR-deficient individuals. The reduction in central regulators of pro-ageing signalling thus appears to be the result of an absence of GHR function. The complex inter-relationship of obesity, diabetes and cancer risk is related to excess insulin and fuel supply, in the presence of heightened anti-apoptosis and uninhibited DNA damage when GHR function is normal.

  11. Vitamin D across growth hormone (GH) disorders: From GH deficiency to GH excess.

    PubMed

    Ciresi, A; Giordano, C

    2017-04-01

    The interplay between vitamin D and the growth hormone (GH)/insulin-like growth factor (IGF)-I system is very complex and to date it is not fully understood. GH directly regulates renal 1 alpha-hydroxylase activity, although the action of GH in modulating vitamin D metabolism may also be IGF-I mediated. On the other hand, vitamin D increases circulating IGF-I and the vitamin D deficiency should be normalized before measurement of IGF-I concentrations to obtain reliable and unbiased IGF-I values. Indeed, linear growth after treatment of nutritional vitamin D deficiency seems to be mediated through activation of the GH/IGF-I axis and it suggests an important role of vitamin D as a link between the proliferating cartilage cells of the growth plate and GH/IGF-I secretion. Vitamin D levels are commonly lower in patients with GH deficiency (GHD) than in controls, with a variable prevalence of insufficiency or deficiency, and this condition may worsen the already known cardiovascular and metabolic risk of GHD, although this finding is not common to all studies. In addition, data on the impact of GH treatment on vitamin D levels in GHD patients are quite conflicting. Conversely, in active acromegaly, a condition characterized by a chronic GH excess, both increased and decreased vitamin D levels have been highlighted, and the interplay between vitamin D and the GH/IGF-I axis becomes even more complicated when we consider the acromegaly treatment, both medical and surgical. The current review summarizes the available data on vitamin D in the main disorders of the GH/IGF-I axis, providing an overview of the current state of the art.

  12. Reappraisal of bovril as a source of arginine in the arginine stimulation test for growth hormone deficiency.

    PubMed

    Loh, H H; Norlela, S; Nor Azmi, K

    2015-06-01

    The purpose of this case study is to report the use of oral Bovril (a food supplement which contains arginine) as an alternative test for growth hormone stimulation test. We performed oral Bovril test in 3 patients -- one with suspected growth hormone deficiency in whom insulin tolerance test could not be performed (subject A), one sex-matched control (subject B), and one with confirmed growth hormone deficiency (subject C). 14g/m(2) of oral Bovril was mixed with 150ml of warm water and was given to all three subjects. Blood for growth hormone was taken at baseline, and every 30 minutes till 150 minutes after ingestion of oral Bovril. The ingestion of oral Bovril showed a positive response in subjects A and B, with highest growth hormone levels of 28.4mIU/L and 42.0mIU/L respectively at 150 minutes. Subject C had suppressed growth hormone throughout the test. Oral Bovril is readily available and is a safe alternative for standard growth hormone stimulation test.

  13. Nutrition, evolution and thyroid hormone levels - a link to iodine deficiency disorders?

    PubMed

    Kopp, Wolfgang

    2004-01-01

    An increased iodine requirement as a result of significant changes in human nutrition rather than a decreased environmental iodine supply is suggested to represent the main cause of the iodine deficiency disorders (IDD). The pathomechanism proposed is based on the fact that serum concentrations of thyroid hormones, especially of trijodothyronine (T3), are dependent on the amount of dietary carbohydrate. High-carbohydrate diets are associated with significantly higher serum T3 concentrations, compared with very low-carbohydrate diets. While our Paleolithic ancestors subsisted on a very low carbohydrate/high protein diet, the agricultural revolution about 10,000 years ago brought about a significant increase in dietary carbohydrate. These nutritional changes have increased T3 levels significantly. Higher T3 levels are associated with an enhanced T3 production and an increased iodine requirement. The higher iodine requirement exceeds the availability of iodine from environmental sources in many regions of the world, resulting in the development of IDD.

  14. Hormonal treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

    PubMed

    Bachelot, A; Chakhtoura, Z; Rouxel, A; Dulon, J; Touraine, P

    2007-09-01

    During childhood, the main aims of the medical treatment of congenital adrenal hyperplasia (CAH) secondary to 21-hydroxylase deficiency, are to prevent salt loss and virilization and to achieve normal stature and normal puberty. As such, there is a narrow therapeutic window through which the intended results can be achieved. In adulthood, the clinical management has received little attention, but recent studies have shown the relevance of long-term follow-up of these patients. Indeed, long-term evaluation of adult CAH patients enables the identification of multiple clinical, hormonal and metabolic abnormalities as bone mineral density alteration, overweight and disturbed reproductive functions. In women with classic CAH, low fertility rate is reported, and is probably the consequence of multiple factors, including neuroendocrine and hormonal factors, feminizing surgery, and psychological factors. Men with CAH may present hypogonadism either through the effect of adrenal rests or from suppression of gonadotropins resulting in infertility. These patients should therefore be carefully followed-up, from childhood through to adulthood, to avoid these complications and to ensure treatment compliance and tight control of the adrenal androgens, by multidisciplinary teams who have knowledge of CAH.

  15. Regulation of NR4A by nutritional status, gender, postnatal development and hormonal deficiency.

    PubMed

    Pérez-Sieira, S; López, M; Nogueiras, R; Tovar, S

    2014-03-03

    The NR4A is a subfamily of the orphan nuclear receptors (NR) superfamily constituted by three well characterized members: Nur77 (NR4A1), Nurr1 (NR4A2) and Nor 1 (NR4A3). They are implicated in numerous biological processes as DNA repair, arteriosclerosis, cell apoptosis, carcinogenesis and metabolism. Several studies have demonstrated the role of this subfamily on glucose metabolism, insulin sensitivity and energy balance. These studies have focused mainly in liver and skeletal muscle. However, its potential role in white adipose tissue (WAT), one of the most important tissues involved in the regulation of energy homeostasis, is not well-studied. The aim of this work was to elucidate the regulation of NR4A in WAT under different physiological and pathophysiological settings involved in energy balance such as fasting, postnatal development, gender, hormonal deficiency and pregnancy. We compared NR4A mRNA expression of Nur77, Nurr1 and Nor 1 and found a clear regulation by nutritional status, since the expression of the 3 isoforms is increased after fasting in a leptin-independent manner and sex steroid hormones also modulate NR4A expression in males and females. Our findings indicate that NR4A are regulated by different physiological and pathophysiological settings known to be associated with marked alterations in glucose metabolism and energy status.

  16. Precocious metamorphosis in the juvenile hormone-deficient mutant of the silkworm, Bombyx mori.

    PubMed

    Daimon, Takaaki; Kozaki, Toshinori; Niwa, Ryusuke; Kobayashi, Isao; Furuta, Kenjiro; Namiki, Toshiki; Uchino, Keiro; Banno, Yutaka; Katsuma, Susumu; Tamura, Toshiki; Mita, Kazuei; Sezutsu, Hideki; Nakayama, Masayoshi; Itoyama, Kyo; Shimada, Toru; Shinoda, Tetsuro

    2012-01-01

    Insect molting and metamorphosis are intricately governed by two hormones, ecdysteroids and juvenile hormones (JHs). JHs prevent precocious metamorphosis and allow the larva to undergo multiple rounds of molting until it attains the proper size for metamorphosis. In the silkworm, Bombyx mori, several "moltinism" mutations have been identified that exhibit variations in the number of larval molts; however, none of them have been characterized molecularly. Here we report the identification and characterization of the gene responsible for the dimolting (mod) mutant that undergoes precocious metamorphosis with fewer larval-larval molts. We show that the mod mutation results in complete loss of JHs in the larval hemolymph and that the mutant phenotype can be rescued by topical application of a JH analog. We performed positional cloning of mod and found a null mutation in the cytochrome P450 gene CYP15C1 in the mod allele. We also demonstrated that CYP15C1 is specifically expressed in the corpus allatum, an endocrine organ that synthesizes and secretes JHs. Furthermore, a biochemical experiment showed that CYP15C1 epoxidizes farnesoic acid to JH acid in a highly stereospecific manner. Precocious metamorphosis of mod larvae was rescued when the wild-type allele of CYP15C1 was expressed in transgenic mod larvae using the GAL4/UAS system. Our data therefore reveal that CYP15C1 is the gene responsible for the mod mutation and is essential for JH biosynthesis. Remarkably, precocious larval-pupal transition in mod larvae does not occur in the first or second instar, suggesting that authentic epoxidized JHs are not essential in very young larvae of B. mori. Our identification of a JH-deficient mutant in this model insect will lead to a greater understanding of the molecular basis of the hormonal control of development and metamorphosis.

  17. Commercial assays available for insulin-like growth factor I and their use in diagnosing growth hormone deficiency.

    PubMed

    Clemmons, D R

    2001-01-01

    Radioimmunoassays of insulin-like growth factor I (IGF-I) are commonly used for screening adults and children for growth hormone (GH) deficiency or excess. There are, however, many problems with such assays. Attempts to resolve these problems have focused on methods of separating IGF-I from its binding proteins, and on reducing inter- and intra-assay variability. In particular, the collection of sufficient high-quality normative data is a major difficulty in many laboratories. Clinical evaluation of assays is also problematic. IGF-I levels vary with age after puberty, and this is complicated by the maintenance of IGF-binding protein 3 levels by IGF-II. Generally, studies have shown that IGF-I is sensitive and specific for the diagnosis of acromegaly, but screening for GH deficiency (GHD) is less precise. The most commonly used commercial assays are immunoradiometric (IRMA) sandwich assays, using antibodies specific to IGF-I. IRMA assays are quick and accurate, and the two-site antibody reactivity produces a high degree of specificity. Additional techniques such as acid-ethanol extraction or saturation with IGF-II can improve reliability. More recently, the introduction of chemiluminescence has provided enhanced speed and sensitivity. The clinical use of these assays has provided a wealth of information regarding the diagnosis of GHD, and it may be possible to reduce the number of patients who require provocative GH testing. IGF-I assays are also of great use in monitoring GH replacement therapy. Despite the problems, IGF-I measurement is currently the best indirect method available for screening and monitoring patients with GHD.

  18. Iron Deficiency Is Common During Remission in Children With Inflammatory Bowel Disease

    PubMed Central

    Wikholm, Emma; Malmborg, Petter; Forssberg, Maria; Hederos, Carl-Axel; Wikström, Sverre

    2016-01-01

    The aim was to study prevalence of iron deficiency in children with inflammatory bowel disease (IBD) during remission. In addition, there was an observational evaluation of hematological response to oral iron. A population-based retrospective study including 90 Swedish children (median 13 years) with IBD was performed. Patient records covered in median 25 months. Iron deficiency was present in 70/77 children (91%) in which iron status could be assessed. In clinical and biochemical remission, iron deficiency was found in 57/67 (85%) of children, and 23 (34%) of them had iron deficiency anemia. Thirty-six iron-deficient children were prescribed oral iron supplementation and 32 (89%) improved hemoglobin levels over 6 months. In conclusion, iron deficiency is common during clinical remission in children with IBD, even in cohorts with low prevalence of anemia. Therefore, regular biochemical screening for iron deficiency is warranted during all stages of disease, irrespective of symptoms and inflammatory blood markers. PMID:27336004

  19. Tissue-specific alterations in thyroid hormone homeostasis in combined Mct10 and Mct8 deficiency.

    PubMed

    Müller, Julia; Mayerl, Steffen; Visser, Theo J; Darras, Veerle M; Boelen, Anita; Frappart, Lucien; Mariotta, Luca; Verrey, Francois; Heuer, Heike

    2014-01-01

    The monocarboxylate transporter Mct10 (Slc16a10; T-type amino acid transporter) facilitates the cellular transport of thyroid hormone (TH) and shows an overlapping expression with the well-established TH transporter Mct8. Because Mct8 deficiency is associated with distinct tissue-specific alterations in TH transport and metabolism, we speculated that Mct10 inactivation may compromise the tissue-specific TH homeostasis as well. However, analysis of Mct10 knockout (ko) mice revealed normal serum TH levels and tissue TH content in contrast to Mct8 ko mice that are characterized by high serum T3, low serum T4, decreased brain TH content, and increased tissue TH concentrations in the liver, kidneys, and thyroid gland. Surprisingly, mice deficient in both TH transporters (Mct10/Mct8 double knockout [dko] mice) showed normal serum T4 levels in the presence of elevated serum T3, indicating that the additional inactivation of Mct10 partially rescues the phenotype of Mct8 ko mice. As a consequence of the normal serum T4, brain T4 content and hypothalamic TRH expression were found to be normalized in the Mct10/Mct8 dko mice. In contrast, the hyperthyroid situation in liver, kidneys, and thyroid gland of Mct8 ko mice was even more severe in Mct10/Mct8 dko animals, suggesting that in these organs, both transporters contribute to the TH efflux. In summary, our data indicate that Mct10 indeed participates in tissue-specific TH transport and also contributes to the generation of the unusual serum TH profile characteristic for Mct8 deficiency.

  20. Tissue-specific thyroid hormone deprivation and excess in monocarboxylate transporter (mct) 8-deficient mice.

    PubMed

    Dumitrescu, Alexandra M; Liao, Xiao-Hui; Weiss, Roy E; Millen, Kathleen; Refetoff, Samuel

    2006-09-01

    Mutations of the X-linked thyroid hormone (TH) transporter (monocarboxylate transporter, MCT8) produce in humans unusual abnormalities of thyroid function characterized by high serum T3 and low T4 and rT3. The mechanism of these changes remains obscure and raises questions regarding the regulation of intracellular availability and metabolism of TH. To study the pathophysiology of MCT8 deficiency, we generated Mct8 knockout mice. Male mice deficient in Mct8 (Mct8(-/y)) replicate the thyroid abnormalities observed in affected men. TH deprivation and replacement with L-T3 showed that suppression of TSH required higher serum levels T3 in Mct8(-/y) than wild-type (WT) littermates, indicating hypothalamus and/or thyrotroph resistance to T3. Furthermore, T4 is required to maintain the high serum T3 level because the latter was not different between the two genotypes during administration of T3. Mct8(-/y) mice have 2.3-fold higher T3 content in liver associated with 6.1- and 3.1-fold increase in deiodinase 1 mRNA and enzymatic activity, respectively. The relative T3 excess in liver of Mct8(-/y) mice produced a decrease in serum cholesterol (79 +/- 18 vs. 137 +/- 38 mg/dl in WT) and an increase in alkaline phosphatase (107 +/- 23 vs. 58 +/- 3 U/liter in WT) levels. In contrast, T3 content in cerebrum was 1.8-fold lower in Mct8(-/y) mice, associated with a 1.6- and 10.6-fold increase in D2 mRNA and enzymatic activity, respectively, as previously observed in TH-deprived WT mice. We conclude that cell-specific differences in intracellular TH content due to differences in contribution of the various TH transporters are responsible for the unusual clinical presentation of this defect, in contrast to TH deficiency.

  1. Sex Hormone-Binding Globulin in Children and Adolescents.

    PubMed

    Aydın, Banu; Winters, Stephen J

    2016-03-05

    Sex hormone-binding globulin (SHBG) is a circulating glycoprotein that transports testosterone and other steroids in the blood. Interest in SHBG has escalated in recent years because of its inverse association with obesity and insulin resistance, and because many studies have linked lower circulating levels of SHBG to metabolic syndrome, type 2 diabetes, nonalcoholic fatty liver disease, polycystic ovary syndrome, and early puberty. The purpose of this review is to summarize molecular, clinical, endocrine, and epidemiological findings to illustrate how measurement of plasma SHBG may be useful in clinical medicine in children.

  2. Sex Hormone-Binding Globulin in Children and Adolescents

    PubMed Central

    Aydın, Banu; Winters, Stephen J.

    2016-01-01

    Sex hormone-binding globulin (SHBG) is a circulating glycoprotein that transports testosterone and other steroids in the blood. Interest in SHBG has escalated in recent years because of its inverse association with obesity and insulin resistance, and because many studies have linked lower circulating levels of SHBG to metabolic syndrome, type 2 diabetes, nonalcoholic fatty liver disease, polycystic ovary syndrome, and early puberty. The purpose of this review is to summarize molecular, clinical, endocrine, and epidemiological findings to illustrate how measurement of plasma SHBG may be useful in clinical medicine in children. PMID:26761949

  3. Initiating growth hormone therapy for children and adolescents.

    PubMed

    Acerini, Carlo; Albanese, Assunta; Casey, Angela; Denvir, Louise; Jones, Julie; Mathew, Verghese; Musson, Pauline; Sparrow, Susan

    It is common for children and adolescents on growth hormone (GH) treatment to miss one or more injections per week, thereby compromising their linear growth outcome. Among factors likely to affect treatment concordance are patient education and support in the selection of the most appropriate GH injection device. The authors discovered inconsistencies in the process of starting patients on GH therapy throughout the UK, and found that there were no clinical recommendations to support health professionals starting patients on treatment. This article describes the issues involved and the development of practical recommendations for use when starting paediatric patients on long-term GH therapy.

  4. Serum hepcidin levels in Helicobacter pylori-infected children with iron-deficiency anemia: a case-control study.

    PubMed

    Azab, Seham F A; Esh, Asmaa M H

    2013-11-01

    Recently, hepcidin, an antimicrobial-like peptide hormone, has evolved as the master regulator of systemic iron homeostasis. Hepcidin integrates signals from diverse physiological inputs, forming a key connection between iron trafficking and response to infection. In this study, we aimed to investigate whether Helicobacter pylori infection modulates serum hepcidin level and response to oral iron therapy in children with iron-deficiency anemia. This was a case-control study including 60 children with iron-deficiency anemia (IDA; 30 H. pylori infected and 30 H. pylori noninfected) and 30 healthy children with comparable age and gender as the control group. Iron parameters including serum iron, ferritin, transferrin, total iron binding capacity, and transferrin saturation and serum hepcidin levels were assessed initially and after 3 months of oral iron therapy for IDA. Compared to the control group, serum hepcidin was significantly lower in H. pylori-noninfected children with IDA (P < 0.01) and significantly higher in H. pylori-infected children with IDA (P < 0.01). Hepcidin increased significantly in noninfected children with IDA after 3 months of oral iron therapy (P < 0.01). On the other hand, H. pylori-infected children showed nonsignificant change in hepcidin level after oral iron therapy (P > 0.05). Although hepcidin showed significant positive correlations with serum ferritin, hemoglobin (Hb), iron, and transferrin saturation in noninfected children with IDA (P < 0.01), it showed significant negative correlations with serum ferritin, Hb, iron, and transferrin saturation in H. pylori-infected children with IDA (P < 0.05). H. pylori infection upregulates serum hepcidin levels and was associated with diminished response to oral iron therapy in children with iron-deficiency anemia.

  5. Prevention of Iron-Deficiency Anemia in Infants and Children of Preschool Age.

    ERIC Educational Resources Information Center

    Fomon, Samuel J.

    Iron-deficiency anemia is almost certainly the most prevalent nutritional disorder among infants and young children in the United States. Anemia is frequently seen among children of low socioeconomic status but is probably also the most frequent nutritional deficiency disease seen among children cared for by private doctors. Possible reasons for…

  6. Iron deficiency anaemia and blood lead concentrations in Brazilian children.

    PubMed

    Rondó, Patricia Helen Carvalho; Conde, Andréia; Souza, Miriam Coelho; Sakuma, Alice

    2011-09-01

    This study investigated the relationship between iron deficiency/iron deficiency anaemia, assessed by several parameters, and blood lead concentration in children. This cross-sectional study involved 384 Brazilian children, aged 2-11 years, who lived near a lead-manipulating industry. Complete blood counts were obtained by an automated cell counter. Serum iron, total iron binding capacity (TIBC) and ferritin were determined respectively, by colorimetric, turbidimetric methods and chemiluminescence. Blood lead was measured by atomic absorption spectrophotometry. The impact of several parameters for assessment of iron status (haemoglobin, serum iron, TIBC, transferrin saturation, ferritin, red cell indices and red cell distribution width) and variables (gender, age, mother's education, income, body mass index, iron intake, and distance from home to lead-manipulating industry) on blood lead concentration was determined by multiple linear regression. There were significant negative associations between blood lead and the distance from home to the lead-manipulating industry (P<0.001), Hb (P=0.019), and ferritin (P=0.023) (R(2)=0.14). Based on these results, further epidemiological studies are necessary to investigate the impact of interventions like iron supplementation or fortification, as an attempt to decrease blood lead in children.

  7. Cognitive and Adaptive Advantages of Growth Hormone Treatment in Children with Prader-Willi Syndrome

    ERIC Educational Resources Information Center

    Dykens, Elisabeth M.; Roof, Elizabeth; Hunt-Hawkins, Hailee

    2017-01-01

    Background: People with Prader-Willi syndrome (PWS) typically have mild to moderate intellectual deficits, compulsivity, hyperphagia, obesity, and growth hormone deficiencies. Growth hormone treatment (GHT) in PWS has well-established salutatory effects on linear growth and body composition, yet cognitive benefits of GHT, seen in other patient…

  8. Iron deficiency is unacceptably high in refugee children from Burma.

    PubMed

    Kemmer, Teresa M; Bovill, Maria E; Kongsomboon, Wantanee; Hansch, Steven J; Geisler, Karen L; Cheney, Carrie; Shell-Duncan, Bettina K; Drewnowski, Adam

    2003-12-01

    Iron-deficiency anemia (IDA) in refugees is reported to be among the major medical problems worldwide. Because food rations are typically inadequate in iron, long-term reliance is a key predictor of anemia among displaced people. Comprehensive nutritional assessments of refugee children from Burma have not previously been completed. Refugee children aged 6-59 mo were studied to determine 1) the prevalences of anemia, iron deficiency (ID) and IDA and 2) the factors associated with anemia and ID. Cluster sampling in three camps and convenience sampling in two additional camps were used. Hemoglobin (Hb) levels were measured and micro mol zinc protoporphyrin/mol heme were determined in 975 children. Logistic regression analyses (95% CI) determined predictors of anemia and ID. The prevalences of IDA, anemia and ID in these refugee children were 64.9, 72.0 and 85.4%, respectively. Predictors of anemia included young age (P < 0.001), food ration lasting <1 mo (P = 0.001), daily consumption of dietary iron inhibitors (P < 0.05), weight-for-height Z-score of <-2 (P < 0.05), male gender (P < 0.05) and uneducated father (P < 0.001). Predictors of ID were young age (P < 0.001) and recently reported illness (P < 0.05). Laboratory tests confirmed that anemia and ID are major health problems among these refugee children and that ID is the leading cause of anemia. A comprehensive nutrition and public health-focused approach to combating anemia and ID is essential. Following the presentation of results to policy makers, the improvement of the micronutrient content of rations has been initiated.

  9. Deficient motor timing in children with neurofibromatosis type 1.

    PubMed

    Debrabant, Julie; Plasschaert, Ellen; Caeyenberghs, Karen; Vingerhoets, Guy; Legius, Eric; Janssens, Sandra; Van Waelvelde, Hilde

    2014-11-01

    Neurofibromatosis type 1 (NF1) is one of the most common single-gene disorders affecting fine and visual-motor skills. This case-control study investigated motor timing as a possible related performance deficit in children with NF1. A visual-motor reaction time (VRT) test was administered in 20 NF1 children (mean age 9 years 7 months) and 20 age- and gender-matched typically developing (TD) children. Copying and tracing performance were evaluated using the Beery-Buktenica Developmental Test of Visual-Motor Integration (Beery VMI). Children with NF1 responded with an increased reaction time (RT) to temporally predictive stimuli compared to TD children, whereas RT at unpredictive stimuli did not differ between groups. Motor timing indexed by the RT decrease at predictive stimuli significantly associated with the Beery VMI copy and tracing outcomes. Deficient motor timing as an actual symptom may add to further research on the pathogenesis of NF1-associated motor impairment and the development of more effective treatment.

  10. Ovarian hormone deficiency reduces intrinsic excitability and abolishes acute estrogen sensitivity in hippocampal CA1 pyramidal neurons

    PubMed Central

    Wu, Wendy W.; Adelman, John P.; Maylie, James

    2011-01-01

    Premature and uncompensated loss of ovarian hormones following ovariectomy (OVX) elevates the risks of cognitive impairment and dementia. These risks are prevented with estrogen (E2)-containing hormone replacement therapy initiated shortly following OVX but not after substantial delay. Currently the cellular bases underlying these clinical findings are unknown. At the cellular level, intrinsic membrane properties regulate the efficiency of synaptic inputs to initiate output action potentials (APs), thereby affecting neuronal communication hence cognitive processing. This study tested the hypothesis that in CA1 pyramidal neurons, intrinsic membrane properties and their acute regulation by E2 require ovarian hormones for maintenance. Whole-cell current clamp recordings were performed on neurons from ~7 months old OVX rats that experienced either short-term (10 days, control OVX) or long-term (5 months, OVXLT) ovarian hormone deficiency. The results reveal that long-term hormone deficiency reduced intrinsic membrane excitability (IE) as measured by the number of evoked action potentials (APs) and firing duration for a given current injection. This was accompanied by AP broadening, an increased slow afterhyperpolarization (sAHP), and faster accumulation of NaV channel inactivation during repetitive firing. In the control OVX neurons, E2 acutely increased IE and reduced the sAHP. In contrast, acute regulation of IE by E2 was absent in the OVXLT neurons. Since the degree of IE of hippocampal pyramidal neurons is positively related with hippocampus-dependent learning ability, and modulation of IE is observed following successful learning, these findings provide a framework for understanding hormone deficiency-related cognitive impairment and the critical window for therapy initiation. PMID:21325532

  11. Do deficiencies in growth hormone and insulin-like growth factor-1 (IGF-1) shorten or prolong longevity?

    PubMed

    Laron, Zvi

    2005-02-01

    Present knowledge on the effects of growth hormone (GH) and insulin-like growth factor-I (IGF-I) deficiency on aging and lifespan are controversial. Studying untreated patients with either isolated GH deficiency due to GH gene deletion, patients with multiple pituitary hormone deficiency due to PROP-1 gene mutation and patients with isolated IGF-I deficiency due to deletions or mutations of the GH receptor gene (Laron syndrome); it was found, that these patients despite signs of early aging (wrinkled skin, obesity, insulin resistance and osteopenia) have a long life span reaching ages of 80-90 years. Animal models of genetic GH deficiencies such as Snell mice (Pit-1 gene mutations) the Ames mice (PROP-1 gene mutation) and the Laron mice (GH receptor gene knock-out) have a statistically significant higher longevity compared to normal controls. On the contrary, mice transgenic for GH and acromegalic patients secreting high amounts of GH have premature death. Those data raise the question whether pharmacological GH administration to adults is deleterious, in contrast to policies advocating such therapies.

  12. Dose dependency of time of onset of radiation-induced growth hormone deficiency

    SciTech Connect

    Clayton, P.E.; Shalet, S.M. )

    1991-02-01

    Growth hormone (GH) secretion during insulin-induced hypoglycemia was assessed on 133 occasions in 82 survivors of childhood malignant disease. All had received cranial irradiation with a dose range to the hypothalamic-pituitary axis of 27 to 47.5 Gy (estimated by a schedule of 16 fractions over 3 weeks) and had been tested on one or more occasions between 0.2 and 18.9 years after treatment. Results of one third of the GH tests were defined as normal (GH peak response, greater than 15 mU/L) within the first 5 years, in comparison with 16% after 5 years. Stepwise multiple linear regression analysis showed that dose (p = 0.007) and time from irradiation (p = 0.03), but not age at therapy, had a significant influence on peak GH responses. The late incidence of GH deficiency was similar over the whole dose range (4 of 26 GH test results normal for less than 30 Gy and 4 of 25 normal for greater than or equal to 30 Gy after 5 years), but the speed of onset over the first years was dependent on dose. We conclude that the requirement for GH replacement therapy and the timing of its introduction will be influenced by the dose of irradiation received by the hypothalamic-pituitary axis.

  13. Epigenetic Upregulation of Corticotrophin-Releasing Hormone Mediates Postnatal Maternal Separation-Induced Memory Deficiency

    PubMed Central

    Wang, Aiyun; Nie, Wenying; Li, Haixia; Hou, Yuhua; Yu, Zhen; Fan, Qing; Sun, Ruopeng

    2014-01-01

    Accumulating evidences demonstrated that early postnatal maternal separation induced remarkable social and memory defects in the adult rodents. Early-life stress induced long-lasting functional adaptation of neuroendocrine hypothalamic-pituitary-adrenal axis, including neuropeptide corticotrophin-releasing hormone (CRH) in the brain. In the present study, a significantly increased hippocampal CRH was observed in the adult rats with postnatal maternal separation, and blockade of CRHR1 signaling significantly attenuated the hippocampal synaptic dysfunction and memory defects in the modeled rats. Postnatal maternal separation enduringly increased histone H3 acetylation and decreased cytosine methylation in Crh promoter region, resulting from the functional adaptation of several transcriptional factors, in the hippocampal CA1 of the modeled rats. Enriched environment reversed the epigenetic upregulation of CRH, and ameliorated the hippocampal synaptic dysfunction and memory defects in the adult rats with postnatal maternal separation. This study provided novel insights into the epigenetic mechanism underlying postnatal maternal separation-induced memory deficiency, and suggested environment enrichment as a potential approach for the treatment of this disorder. PMID:24718660

  14. Short-term growth hormone treatment in children with Hurler syndrome after hematopoietic cell transplantation

    PubMed Central

    Polgreen, Lynda E.; Plog, Melissa; Schwender, James D.; Tolar, Jakub; Thomas, William; Orchard, Paul J.; Miller, Bradley S.; Petryk, Anna

    2011-01-01

    Summary Children with Hurler syndrome experience progressive growth failure after hematopoietic cell transplantation (HCT). The goal of this study was to review the safety and efficacy of growth hormone (GH) in eight children with Hurler syndrome who were treated at our institution with GH for short stature or GH deficiency between 2005 and 2008. The age at initiation of treatment with GH was 9.6 ± 2.3 years and time since HCT was 7.5 ± 1.5 years. Mean GH dose was 0.32 mg/kg/week. Baseline growth velocity was 3.5 ± 1.5 cm/yr (−2.6 ± 1.9 SDS) and increased to 5.2 ± 3.0 cm/yr (−0.1 ± 3.6 SDS) after 1 year of treatment. Of 6 patients with radiographic data there was 1 progression of scoliosis, 1 progression of kyphosis, and 1 progression of genu valgum. No patient discontinued treatment due to progression of skeletal disease. One patient discontinued GH due to slipped capital femoral epiphysis (SCFE). Preliminary data suggest that one year GH treatment may modestly improve growth velocity in children with Hurler syndrome. PMID:19252529

  15. Isolated growth hormone deficiency (GHD) in childhood and adolescence: recent advances.

    PubMed

    Alatzoglou, Kyriaki S; Webb, Emma Alice; Le Tissier, Paul; Dattani, Mehul T

    2014-06-01

    The diagnosis of GH deficiency (GHD) in childhood is a multistep process involving clinical history, examination with detailed auxology, biochemical testing, and pituitary imaging, with an increasing contribution from genetics in patients with congenital GHD. Our increasing understanding of the factors involved in the development of somatotropes and the dynamic function of the somatotrope network may explain, at least in part, the development and progression of childhood GHD in different age groups. With respect to the genetic etiology of isolated GHD (IGHD), mutations in known genes such as those encoding GH (GH1), GHRH receptor (GHRHR), or transcription factors involved in pituitary development, are identified in a relatively small percentage of patients suggesting the involvement of other, yet unidentified, factors. Genome-wide association studies point toward an increasing number of genes involved in the control of growth, but their role in the etiology of IGHD remains unknown. Despite the many years of research in the area of GHD, there are still controversies on the etiology, diagnosis, and management of IGHD in children. Recent data suggest that childhood IGHD may have a wider impact on the health and neurodevelopment of children, but it is yet unknown to what extent treatment with recombinant human GH can reverse this effect. Finally, the safety of recombinant human GH is currently the subject of much debate and research, and it is clear that long-term controlled studies are needed to clarify the consequences of childhood IGHD and the long-term safety of its treatment.

  16. Sulphur limitation and early sulphur deficiency responses in poplar: significance of gene expression, metabolites, and plant hormones.

    PubMed

    Honsel, Anne; Kojima, Mikiko; Haas, Richard; Frank, Wolfgang; Sakakibara, Hitoshi; Herschbach, Cornelia; Rennenberg, Heinz

    2012-03-01

    The influence of sulphur (S) depletion on the expression of genes related to S metabolism, and on metabolite and plant hormone contents was analysed in young and mature leaves, fine roots, xylem sap, and phloem exudates of poplar (Populus tremula×Populus alba) with special focus on early consequences. S depletion was applied by a gradual decrease of sulphate availability. The observed changes were correlated with sulphate contents. Based on the decrease in sulphate contents, two phases of S depletion could be distinguished that were denominated as 'S limitation' and 'early S deficiency'. S limitation was characterized by improved sulphate uptake (enhanced root-specific sulphate transporter PtaSULTR1;2 expression) and reduction capacities (enhanced adenosine 5'-phosphosulphate (APS) reductase expression) and by enhanced remobilization of sulphate from the vacuole (enhanced putative vacuolar sulphate transporter PtaSULTR4;2 expression). During early S deficiency, whole plant distribution of S was impacted, as indicated by increasing expression of the phloem-localized sulphate transporter PtaSULTR1;1 and by decreasing glutathione contents in fine roots, young leaves, mature leaves, and phloem exudates. Furthermore, at 'early S deficiency', expression of microRNA395 (miR395), which targets transcripts of PtaATPS3/4 (ATP sulphurylase) for cleavage, increased. Changes in plant hormone contents were observed at 'early S deficiency' only. Thus, S depletion affects S and plant hormone metabolism of poplar during 'S limitation' and 'early S deficiency' in a time series of events. Despite these consequences, the impact of S depletion on growth of poplar plants appears to be less severe than in Brassicaceae such as Arabidopsis thaliana or Brassica sp.

  17. Short-term therapy with recombinant growth hormone in polytransfused thalassaemia major patients with growth deficiency.

    PubMed

    Cavallo, L; Gurrado, R; Zecchino, C; Manolo, F; De Sanctis, V; Cisternino, M; Caruso-Nicoletti, M; Galati, M

    1998-01-01

    Growth failure is commonly described in polytransfused thalassaemia major patients (Th) with or without growth hormone (GH) releasing hormone-GH axis impairment. We have investigated the efficacy of short-term recombinant GH (rhGH) therapy (Saizen [Serono] 0.1 IU/kg/day 6 evenings/week administered s.c. for 12 months) on growth and predicted final height in 28 (19M, 9F) regularly transfused Th with growth deficiency (aged 14.8 +/- 2.0 yr) on long term desferrioxamine s.c. therapy. All Th had no evidence of congestive heart failure, hypothyroidism or impaired glucose tolerance; in all patients the GH peak (evaluated during both insulin and clonidine test) was < or = 20 mIU/l; hypergonadotropic hypogonadism was excluded in Th with delayed puberty. At the start of therapy height age (HA)/bone age (BA) ratio was 0.92 +/- 0.12. Bone age delay was positively correlated to chronological age (CA), serum ferritin levels (mean of the last three years), the age at the start of chelation therapy, growth velocity calculated for CA during the last year; a positive correlation was also found between circulating IGF-I levels and age at the start of chelation therapy. After 1 year on rhGH therapy there was a significant increase of height calculated for CA (not for BA), of growth velocity calculated for both CA and BA and of circulating IGF-I levels; the HA variation/BA variation ratio was 1.85 +/- 1.71, without any significant difference between predicted final height at the start (-1.08 +/- 1.28 SDS) and at the end of rhGH therapy (-0.88 +/- 1.13). The variation of height calculated for CA was positively correlated to both CA and growth velocity during the last year before rhGH therapy (calculated for CA) and negatively to the height at the start (calculated for CA). There were no side effects and haematological parameters did not show significant changes. In conclusion, our data, obtained in a relatively large group of Th, confirm the emerging results of short-term (12 months

  18. Clonidine-stimulated growth hormone concentrations (cut-off values) measured by immunochemiluminescent assay (ICMA) in children and adolescents with short stature

    PubMed Central

    de Fátima Borges, Maria; Teixeira, Flávia Carolina Cândida; Feltrin, Aline Karin; Ribeiro, Karina Alvarenga; Nascentes, Gabriel Antonio Nogueira; Resende, Elisabete Aparecida Mantovani Rodrigues; Ferreira, Beatriz Pires; Silva, Adriana Paula; Palhares, Heloísa Marcelina Cunha

    2016-01-01

    OBJECTIVES: To establish cut-off values for growth hormone concentrations using clonidine as a secretagogue and an immunochemiluminescent assay as the method of measurement and to analyze the response time as well as the influence of gender, nutritional status and pubertal stage. METHODS: A total of 225 tests were performed in 3 patient groups, categorized as group 1 (normal), group 2 (idiopathic short stature) and group 3 (growth hormone deficiency). Among the 199 disease-free individuals, 138 were prepubertal, and 61 were pubertal. Clonidine (0.1 mg/m2) was orally administered, and the growth hormone level was measured by immunochemiluminescent assay. The growth hormone peak and the difference between the growth hormone peak and the baseline level were then analyzed. Statistical analyses were performed using Student's t-test or the Mann-Whitney test and Kruskal-Wallis test followed by Dunn's post hoc test. Cut-off values were determined using a receiver operating characteristic curve. RESULTS: Group 1 and group 2 had no difference in growth hormone peak, gender, body mass index standard deviation score, or pubertal stage. Group 3 exhibited a significantly lower growth hormone peak than the other groups did. The receiver operating characteristic curve demonstrated that growth hormone concentrations ≥ 3.0 ng/mL defined responsiveness to clonidine. In total, 3.02% of individuals in group 1 and group 2 were considered false positive, i.e., these children lacked growth hormone deficiency and had a peak below 3.0 ng/mL. CONCLUSION: Clonidine-stimulated growth hormone concentrations ≥3 ng/mL, as measured by immunochemiluminescent assay, suggest responsiveness to the stimulus regardless of gender, body mass index standard deviation score or pubertal stage. PMID:27166774

  19. Beyond Stimulus Deprivation: Iron Deficiency and Cognitive Deficits in Postinstitutionalized Children

    ERIC Educational Resources Information Center

    Doom, Jenalee R.; Gunnar, Megan R.; Georgieff, Michael K.; Kroupina, Maria G.; Frenn, Kristin; Fuglestad, Anita J.; Carlson, Stephanie M.

    2014-01-01

    Children adopted from institutions have been studied as models of the impact of stimulus deprivation on cognitive development (Nelson, Bos, Gunnar, & Sonuga-Barke, 2011), but these children may also suffer from micronutrient deficiencies (Fuglestad et al., 2008). The contributions of iron deficiency (ID) and duration of deprivation on…

  20. Growth hormone deficiency in monozygotic twins with autosomal dominant pseudohypoparathyroidism type Ib.

    PubMed

    Sano, Shinichiro; Iwata, Hiromi; Matsubara, Keiko; Fukami, Maki; Kagami, Masayo; Ogata, Tsutomu

    2015-01-01

    Pseudohypoparathyroidism (PHP) is associated with compromised signal transductions via PTH receptor (PTH-R) and other G-protein-coupled receptors including GHRH-R. To date, while GH deficiency (GHD) has been reported in multiple patients with PHP-Ia caused by mutations on the maternally expressed GNAS coding regions and in two patients with sporadic form of PHP-Ib accompanied by broad methylation defects of maternally derived GNAS differentially methylated regions (DMRs), it has not been identified in a patient with an autosomal dominant form of PHP-Ib (AD-PHP-Ib) accompanied by an STX16 microdeletion and an isolated loss of methylation (LOM) at exon A/B-DMR. We studied 5 4/12-year-old monozygotic twins with short stature (both -3.4 SD) and GHD (peak GH values, <6.0 μg/L after arginine and clonidine stimulations). Molecular studies revealed maternally derived STX16 microdeletions and isolated LOMs at exon A/B-DMR in the twins, confirming the diagnosis of AD-PHP-Ib. GNAS mutation was not identified, and neither mutation nor copy number variation was detected in GH1, POU1F1, PROP1, GHRHR, LHX3, LHX4, and HESX1 in the twins. The results, in conjunction with the previous finding that GNAS shows maternal expression in the pituitary, suggest that GHD of the twins is primarily ascribed to compromised GHRH-R signaling caused by AD-PTH-Ib. Thus, resistance to multiple hormones including GHRH should be considered in AD-PHP-Ib.

  1. Crohn's disease and growth deficiency in children and adolescents.

    PubMed

    Gasparetto, Marco; Guariso, Graziella

    2014-10-07

    Nutritional concerns, linear growth deficiency, and delayed puberty are currently detected in up to 85% of patients with Crohn's disease (CD) diagnosed at childhood. To provide advice on how to assess and manage nutritional concerns in these patients, a Medline search was conducted using "pediatric inflammatory bowel disease", "pediatric Crohn's disease", "linear growth", "pubertal growth", "bone health", and "vitamin D" as key words. Clinical trials, systematic reviews, and meta-analyses published between 2008 and 2013 were selected to produce this narrative review. Studies referring to earlier periods were also considered if the data was relevant to our review. Although current treatment strategies for CD that include anti-tumor necrosis factor-α therapy have been shown to improve patients' growth rate, linear growth deficiencies are still common. In pediatric CD patients, prolonged diagnostic delay, high initial activity index, and stricturing/penetrating type of behavior may cause growth deficiencies (in weight and height) and delayed puberty, with several studies reporting that these patients may not reach an optimal bone mass. Glucocorticoids and inflammation inhibit bone formation, though their impact on skeletal modeling remains unclear. Long-term control of active inflammation and an adequate intake of nutrients are both fundamental in promoting normal puberty. Recent evidence suggests that recombinant growth factor therapy is effective in improving short-term linear growth in selected patients, but is of limited benefit for ameliorating mucosal disease and reducing clinical disease activity. The authors conclude that an intense initial treatment (taking a "top-down" approach, with the early introduction of immunomodulatory treatment) may be justified to induce and maintain remission so that the growth of children with CD can catch up, ideally before puberty. Exclusive enteral nutrition has a key role in inducing remission and improving patients

  2. Absence of the dawn phenomenon and abnormal lipolysis in type 1 (insulin-dependent) diabetic patients with chronic growth hormone deficiency.

    PubMed

    Boyle, P J; Avogaro, A; Smith, L; Shah, S D; Cryer, P E; Santiago, J V

    1992-04-01

    To determine the role of growth hormone in overnight insulin requirements and lipolysis, five patients with chronic growth hormone deficiency and Type 1 (insulin-dependent) diabetes mellitus and six control patients with diabetes were each studied on two separate nights. Insulin was infused at a variable rate throughout one night to maintain euglycaemia and fixed at 04.00 hours on another. During the variable infusion, euglycaemia was maintained in control patients by a 36% increase in insulin infusion rate between 03.00 and 08.00 hours while a 46% decrease in the rate was required in growth hormone deficient patients (p less than 0.02). Despite this difference, mean free insulin values were equivalent. This finding is suggestive of increased insulin clearance in growth hormone sufficient patients. Glucose levels rose in control and fell in growth hormone deficient patients when insulin infusion rates were fixed at 04.00 hours. Glycerol production and non-esterified fatty acid concentrations were significantly lower in the growth hormone deficient diabetic patients, p less than 0.001, and when normalized with a heparin infusion, had no effect on insulin requirements. We conclude that: (1) growth hormone contributes to the development of the "dawn phenomenon," possibly by increasing insulin clearance (2) growth hormone helps sustain nocturnal lipolysis in Type 1 diabetes and (3) non-esterified fatty acids are not involved in the dawn phenomenon.

  3. Evaluation of impact of steroid replacement treatment on bone health in children with 21-hydroxylase deficiency.

    PubMed

    Delvecchio, M; Soldano, L; Lonero, A; Ventura, A; Giordano, P; Cavallo, L; Grano, M; Brunetti, G; Faienza, M F

    2015-04-01

    There are conflicting data regarding the potential impact of chronic glucocorticoid (GC) therapy on the bone mineral density of patients with congenital adrenal hyperplasia (CAH). Previous studies performed by dual-energy X-ray absorptiometry reported conflicting results. The purpose of this study was to assess the impact of chronic GC replacement treatment in children with classical and non classical CAH due to 21-hydroxylase deficiency (21-OHD) by quantitative ultrasonometry (QUS), an easy, cheap, and radiation-free technique. The study population consisted of nineteen 21-OHD patients (nine males) on lifelong GC treatment. Anthropometric, hormonal, and treatment data were recorded for each patient, and bone quality was assessed by QUS measurements. QUS findings (amplitude-dependent speed of sound and bone transmission time) were normal in 21-OHD patients and did not correlate with duration of treatment, daily, total, and yearly hydrocortisone dose. Furthermore, no significant correlation was found between QUS findings and 17α-hydroxy progesterone, Δ4-androstenedione, and testosterone levels. In conclusion, our results provide reassurance that currently used replacement doses of GC do not have a major impact on bone in patients with CAH. QUS seems to be a reliable tool for screening of bone health in children with 21-OHD.

  4. Directed forgetting in young children: evidence for a production deficiency.

    PubMed

    Aslan, Alp; Staudigl, Tobias; Samenieh, Anuscheh; Bäuml, Karl-Heinz T

    2010-12-01

    When people are cued to forget a previously studied list of items and to learn a new list instead, such cuing typically leads to forgetting of the first list and to memory enhancement of the second. In two experiments, we examined such listwise directed forgetting in children (and adults), using a forget cue that placed either high emphasis or low emphasis on the need to forget. In the low-emphasis condition, (adult-like) List 1 forgetting was present in fourth graders, but not in first graders (and kindergartners); in contrast, in the high-emphasis condition, (adult-like) List 1 forgetting was present from first grade on. Only fourth graders showed (adult-like) List 2 enhancement, regardless of task instruction. The finding that first graders showed List 1 forgetting only in the high-emphasis condition points to a production deficiency in first graders' directed forgetting, suggesting that the children are capable of intentional forgetting but fail to do so spontaneously. The finding that first graders showed List 1 forgetting without List 2 enhancement suggests that the two directed-forgetting effects are mediated by different processes with different developmental trajectories.

  5. Food fortification for addressing iron deficiency in Filipino children: benefits and cost-effectiveness.

    PubMed

    Detzel, Patrick; Wieser, Simon

    2015-01-01

    Iron deficiency is one of the most widespread nutritional disorders in both developing and industrialized countries, making it a global public health concern. Anemia, mainly due to iron deficiency, affects one third of the world's population and is concentrated in women and children below 5 years of age. Iron deficiency anemia has a profound impact on human health and productivity, and the effects of iron deficiency are especially pronounced in the first 1,000 days of life. This critical window of time sets the stage for an individual's future physiological and cognitive health, underscoring the importance of addressing iron deficiency in infants and young children. This review focuses on the use of fortified foods as a cost-effective tool for addressing iron deficiency in infants and young children in the Philippines.

  6. 'A disease that makes criminals': encephalitis lethargica (EL) in children, mental deficiency, and the 1927 Mental Deficiency Act.

    PubMed

    Ruiz, Violeta

    2015-03-01

    Encephalitis lethargica (EL) was an epidemic that spread throughout Europe and North America during the 1920s. Although it could affect both children and adults alike, there were a strange series of chronic symptoms that exclusively affected its younger victims: behavioural disorders which could include criminal propensities. In Britain, which had passed the Mental Deficiency Act in 1913, the concept of mental deficiency was well understood when EL appeared. However, EL defied some of the basic precepts of mental deficiency to such an extent that amendments were made to the Mental Deficiency Act in 1927. I examine how clinicians approached the sequelae of EL in children during the 1920s, and how their work and the social problem that these children posed eventually led to changes in the legal definition of mental deficiency. EL serves as an example of how diseases are not only framed by the society they emerge in, but can also help to frame and change existing concepts within that same society.

  7. Prevalence of Iron Deficiency and Anemia among Young Children with Acute Diarrhea in Bhaktapur, Nepal

    PubMed Central

    Chandyo, Ram K.; Ulak, Manjeswori; Adhikari, Ramesh K.; Sommerfelt, Halvor; Strand, Tor A.

    2015-01-01

    Iron deficiency anemia is still common in children under five years of age and may impair their growth and cognitive development. Diarrhea is the second most common reason for seeking medical care for young children in Nepal. However, neither screening programs nor effective preventive measures for anemia and iron deficiencies are in place among children with diarrhea in many developing countries. The aims of this study were to determine the prevalence of anemia and iron deficiency and explore their associations with clinical, socioeconomic, and anthropometric parameters in Nepalese children. This was a cross-sectional study based on 1232 children, six to 35 months old, with acute diarrhea participating in a zinc supplementation trial. The mean (SD) hemoglobin was 11.2 g/dL (1.2). Anemia was found in 493 children (40%); this estimate increased to 641 (52%) when we adjusted for the altitude of the study area (hemoglobin <11.3 g/dL). One in every three children had depleted iron stores and 198 (16%) of the children had both depleted iron stores and anemia, indicating iron deficiency anemia. The prevalence of anemia among children presenting with acute diarrhea was high but the degree of severity was mainly mild or moderate. Iron deficiency explained less than half of the total anemia, indicating other nutritional deficiencies inducing anemia might be common in this population. PMID:27417782

  8. The use of zinc protoporphyrin in screening young children for iron deficiency.

    PubMed

    Siegel, R M; LaGrone, D H

    1994-08-01

    The common practice of screening children for iron deficiency with hematocrit (HCT) or hemoglobin detects only those children with iron deficiency severe enough to cause anemia. At 40 cents per test, zinc protoporphyrin (ZPP) can be measured in the physician's office and identify iron deficiency before anemia develops. The purpose of our study was to evaluate ZPP screening in young children by hematofluorimetry. All children between 9 and 36 months old were enrolled over a 1-year period. All children with HCT < 33% or iron deficiency (ZPP > or = 50 mumoles per mole-heme) were treated with ferrous sulfate at 3 mg-Fe/kg/day for 3 months. Four hundred and fifty-eight children were screened with a blood ZPP and spun HCT at entry. Two hundred and forty-three children (53%) had both a normal ZPP and HCT, 155 (34%) had a ZPP > or = 50 and normal HCT, 26 (6%) had a ZPP > or = 50 with HCT < 33%, and 34 (7%) had a normal ZPP and HCT < 33%. Of those with an elevated ZPP and normal HCT, 76% had a therapeutic response to iron therapy, with a 10% decrease in ZPP (P < 0.005). ZPP had a greater sensitivity (81%) than HCT (16%) in identifying children with iron deficiency. ZPP proved to be an effective and inexpensive addition to HCT in identifying children with iron deficiency.

  9. Familial idiopathic gonadotropin deficiency not linked to gene for gonadotropin-releasing hormone (GnRH) in Brazilian kindred

    SciTech Connect

    Faraco, J.; Francke, U.; Toledo, S.

    1994-09-01

    Familial idiopathic gonadotropin deficiency (FIGD) is an autosomal recessive disorder which results in failure to develop secondary sexual characteristics. The origin is a hypothalamic defect resulting in insufficient secretion of gonadotropin-releasing hormone GnRH (also called LHRH, luteinizing hormone releasing hormone) and follicle-stimuating hormone (FSH). FIGD has been determined to be a separate entity from Kallmann syndrome which presents with hypogonadism as well as anosmia. The FIGD phenotype appears to be analogous to the phenotype of the hpg (hypogonadal) mouse. Because the hpg phenotype is the result of a structurally abnormal GnRH gene, we have studied the GnRH gene in individuals from a previously reported Brazilian FIGD family. An informative dimorphic marker in the signal peptide sequence of the GnRH gene allowed assessment of linkage between the disease gene and the GnRH locus in this pedigree. We have concluded that the GnRH locus is not linked to the disease-causing mutation in these hypogonadal individuals. Recent evidence suggests that neuropeptide Y (NPY) may play a role in the initiation of puberty. We hypothesize that mutations in NPY may result in failure to secrete GnRH. We have characterized three diallelic frequent-cutter restriction fragment length polymorphisms within the human NPY locus, and are currently using these markers to determine if the NPY gene is linked to, and possibly the site of the disease mutation in this kindred.

  10. Management of Adult Growth Hormone Deficiency at Peking Union Medical College Hospital: A Survey among Physicians(△).

    PubMed

    Yang, Hong-Bo; Zhang, Meng-Qi; Pan, Hui; Zhu, Hui-Juan

    2016-09-20

    Objective To evaluate physicians' attitude and knowledge about the management of adult growth hormone deficiency (AGHD) at Peking Union Medical College Hospital and impact factors associated with better decision-making.Methods A 21-question anonymous survey was distributed and collected at Peking Union Medical College Hospital, a major teaching hospital in Chinese Academy of Medical Sciences. Data of physicians' educational background, clinical training, patient workload per year and continuing medical education in AGHD were collected. Factors associated with appropriate answers were further analyzed by multivariate regression models.Results One hundred and eighteen internal medicine residents, endocrine fellows, attending physicians and visiting physicians responded to the survey. Among them, 44.9% thought that AGHD patients should accept recombinant human growth hormone replacement therapy. Moreover, 56.8% selected insulin tolerance test and growth hormone-releasing hormone-arginine test for the diagnosis of AGHD. Logistic regression analysis of physician demographic data, educational background, and work experience found no consistent independent factors associated with better decision-making, other than continued medical education, that were associated with treatment choice.Conclusions The physicians' reported management of AGHD in this major academic healthcare center in Beijing was inconsistent with current evidence. High quality continued medical education is required to improve Chinese physician management of AGHD.

  11. Thyroid hormone synthesis: a potential target of a Chinese herbal formula Haizao Yuhu Decoction acting on iodine-deficient goiter

    PubMed Central

    Mao, Xia; Yan, Chen; Guo, Xiaodong; Guo, Qiuyan; Liu, Zhenli; Song, Zhiqian; Lin, Na

    2016-01-01

    Haizao Yuhu Decoction (HYD), a famous multi-component herbal formula, has been widely used to treat various thyroid-related diseases, including iodine-deficient goiter. Herb pair Thallus Sargassi Pallidi (HZ) and Radix Glycyrrhizae (GC), one of the so-called “eighteen antagonistic medicaments”, contains in HYD. To explore pharmacological mechanisms of HYD acting on iodine-deficient goiter and to provide evidence for potential roles of herb pair HZ and GC in HYD, our genome-wide microarray detection and network analysis identified a list of goiter-related genes, mainly involved into the alterations in hypothalamus-pituitary-thyroid/gonad/growth axes. Then, the disease genes-drug genes interaction network illustrated the links between HYD regulating genes and goiter-related genes, and identified the candidate targets of HYD acting on goiter. Functionally, these candidate targets were closely correlated with thyroid hormone synthesis. Moreover, the potential regulating genes of herb pair HZ and GC were revealed to be crucial components in the pathway of thyroid hormone synthesis. The prediction results were all verified by following experiments based on goiter rats. Collectively, this integrative study combining microarray gene expression profiling, network analysis and experimental validations offers the convincing evidence that HYD may alleviate iodine-deficient goiter via regulating thyroid hormone synthesis, and explains the necessity of herb pair HZ and GC in HYD. Our work provides a novel and powerful means to clarify the mechanisms of action for multi-component drugs such as herbal formulae in a holistic way, which may improve drug development and applications. PMID:27384475

  12. Treatable Bedridden Elderly―Recovery from Flexion Contracture after Cortisol Replacement in a Patient with Isolated Adrenocorticotropic Hormone Deficiency

    PubMed Central

    Tanaka, Takamasa; Terada, Norihiko; Fujikawa, Yoshiki; Fujimoto, Takushi

    2016-01-01

    Isolated adrenocorticotropic hormone deficiency (IAD) is a rare disorder with diverse clinical presentations. A 79-year-old man was bedridden for six months due to flexion contractures of the bilateral hips and knees, along with hyponatremia. He was diagnosed with IAD based on the results of endocrine tests. After one month of corticosteroid replacement, he recovered and was able to stand up by himself. Although flexion contracture is a rare symptom of IAD, steroid replacement therapy may be effective, even for seemingly irreversibly bedridden elderly patients. In bedridden elderly patients with flexion contractures, we should consider and look for any signs of adrenal insufficiency. PMID:27746435

  13. Treatable Bedridden Elderly -Recovery from Flexion Contracture after Cortisol Replacement in a Patient with Isolated Adrenocorticotropic Hormone Deficiency.

    PubMed

    Tanaka, Takamasa; Terada, Norihiko; Fujikawa, Yoshiki; Fujimoto, Takushi

    Isolated adrenocorticotropic hormone deficiency (IAD) is a rare disorder with diverse clinical presentations. A 79-year-old man was bedridden for six months due to flexion contractures of the bilateral hips and knees, along with hyponatremia. He was diagnosed with IAD based on the results of endocrine tests. After one month of corticosteroid replacement, he recovered and was able to stand up by himself. Although flexion contracture is a rare symptom of IAD, steroid replacement therapy may be effective, even for seemingly irreversibly bedridden elderly patients. In bedridden elderly patients with flexion contractures, we should consider and look for any signs of adrenal insufficiency.

  14. Hormones

    MedlinePlus

    ... affect many different processes, including Growth and development Metabolism - how your body gets energy from the foods you eat Sexual function Reproduction Mood Endocrine glands, which are special groups of cells, make hormones. The major endocrine glands are the ...

  15. Spino-Cerebellar Degeneration, Hormonal Disorder, Hypogonadism, Deaf Mutism and Mental Deficiency

    ERIC Educational Resources Information Center

    Sylvester, P. E.

    1972-01-01

    Post mortem examinations were done on two adult siblings (one female and one male) who had been clinically described as suffering from mental handicap, deaf mutism, ataxia, hypogonadism, and hormonal disorders. (DB)

  16. Reproductive hormone-dependent and -independent contributions to developmental changes in kisspeptin in GnRH-deficient hypogonadal mice.

    PubMed

    Gill, John C; Wang, Oulu; Kakar, Shelley; Martinelli, Enzo; Carroll, Rona S; Kaiser, Ursula B

    2010-07-30

    Kisspeptin is a potent activator of GnRH-induced gonadotropin secretion and is a proposed central regulator of pubertal onset. In mice, there is a neuroanatomical separation of two discrete kisspeptin neuronal populations, which are sexually dimorphic and are believed to make distinct contributions to reproductive physiology. Within these kisspeptin neuron populations, Kiss1 expression is directly regulated by sex hormones, thereby confounding the roles of sex differences and early activational events that drive the establishment of kisspeptin neurons. In order to better understand sex steroid hormone-dependent and -independent effects on the maturation of kisspeptin neurons, hypogonadal (hpg) mice deficient in GnRH and its downstream effectors were used to determine changes in the developmental kisspeptin expression. In hpg mice, sex differences in Kiss1 mRNA levels and kisspeptin immunoreactivity, typically present at 30 days of age, were absent in the anteroventral periventricular nucleus (AVPV). Although immunoreactive kisspeptin increased from 10 to 30 days of age to levels intermediate between wild type (WT) females and males, corresponding increases in Kiss1 mRNA were not detected. In contrast, the hpg arcuate nucleus (ARC) demonstrated a 10-fold increase in Kiss1 mRNA between 10 and 30 days in both females and males, suggesting that the ARC is a significant center for sex steroid-independent pubertal kisspeptin expression. Interestingly, the normal positive feedback response of AVPV kisspeptin neurons to estrogen observed in WT mice was lost in hpg females, suggesting that exposure to reproductive hormones during development may contribute to the establishment of the ovulatory gonadotropin surge mechanism. Overall, these studies suggest that the onset of pubertal kisspeptin expression is not dependent on reproductive hormones, but that gonadal sex steroids critically shape the hypothalamic kisspeptin neuronal subpopulations to make distinct contributions to

  17. Neurocognitive outcomes of children secondary to mild iodine deficiency in pregnant women.

    PubMed

    Caron, Philippe

    2015-07-01

    Iodine deficiency is the most important preventable cause of brain damage worldwide. During pregnancy, severe iodine deficiency causes endemic cretinism, whereas mild-to-moderate iodine deficiency impairs neurocognitive function of the offspring. Numerous reports demonstrate the impact of iodine supplementation on prevention of cretinism, and recent studies evaluate the effects of iodine prophylaxis on neurocognitive development in children of women with mild-to-moderate iodine deficiency. Iodine prophylaxis is generally well tolerated without side effects for the pregnant women and the offspring. In France, the iodine status was recently considered as satisfactory in children and adult population, but regional studies conducted during the last two decades have shown that healthy women are mild-to-moderately iodine deficient during pregnancy. According to recent World Heath Organization guidelines, systematic iodine prophylaxis is recommended in women planning a pregnancy, during gestation and lactation in order to prevent maternal, neonatal and infantile consequences of mild-to-moderate iodine deficiency.

  18. Clinical, biochemical, and genetic analysis of two korean patients with trichorhinophalangeal syndrome type I and growth hormone deficiency.

    PubMed

    Sohn, Young Bae; Ki, Chang-Seok; Park, Sung Won; Cho, Sung-Yoon; Ko, Ah-Ra; Kwon, Min-Jung; Kim, Ji-Youn; Park, Hyung-Doo; Kim, Ok-Hwa; Jin, Dong-Kyu

    2012-01-01

    Tricho-rhino-phalangeal syndrome type I (TRPSI) is a rare autosomal dominant hereditary disorder characterized by sparse hair, bulbous nose, long philtrum, thin upper lip, and skeletal abnormalities including cone-shaped epiphyses, shortening of the phalanges, and short stature. TRPSI is caused by mutations in the TRPS1 gene. Herein, we report two Korean cases of TRPSI. Although both patients (a 17-year-old-female and a 14-year-old male) had typical clinical findings, Patient 1 had an additional growth hormone (GH) deficiency. Treatment with recombinant human growth hormone (rhGH) 0.7 IU/kg/week led to an increase in growth velocity. Over 10 years of GH therapy, the mean growth velocity was 5.7 ± 0.9 cm/year. However, the patient 2 did not show apparent GH deficiency by GH stimulation test, had a poor response with rhGH therapy and GH therapy was discontinued after 6 months. Upon genetic analysis of the TRPS1 gene, two mutations were found. Patient 1 had a heterozygous mutation c.2520dupT (p.Arg841LysfsX3) which had not been previously reported. Patient 2 had a known nonsense mutation c.1630C>T (p.Arg544X). In summary, we were the first to report Korean patients with mutation of TRPS1.

  19. Maternal thyroid hormone deficiency affects the fetal neocorticogenesis by reducing the proliferating pool, rate of neurogenesis and indirect neurogenesis.

    PubMed

    Mohan, Vishwa; Sinha, Rohit A; Pathak, Amrita; Rastogi, Leena; Kumar, Praveen; Pal, Amit; Godbole, Madan M

    2012-10-01

    Neuronal progenitor cell proliferation and their optimum number are indispensable for neurogenesis, which is determined by cell cycle length and cell cycle quitting rate of the dividing progenitors. These processes are tightly orchestrated by transcription factors like Tbr2, Pax6, and E2f-1. Radial glia and intermediate progenitor cells (IPC) through direct and indirect neurogenesis maintain surface area and neocortical thickness during development. Here we show that fetal neurogenesis is maternal thyroid hormone (MTH) dependent with differential effect on direct and indirect neurogenesis. MTH deficiency (MTHD) impairs direct neurogenesis through initial down-regulation of Pax6 and diminished progenitor pool with recovery even before the onset of fetal thyroid function (FTF). However, persistent decrease in Tbr2 positive IPCs, diminished NeuN positivity in layers I-III of neocortex, and reduced cortical thickness indicate a non-compensatory impairment in indirect neurogenesis. TH deficiency causes disrupted cell cycle kinetics and deranged neurogenesis. It specifically affects indirect neurogenesis governed by intermediate progenitor cells (IPCs). TH replacement in hypothyroid dams partially restored the rate of neurogenesis in the fetal neocortex. Taken together we describe a novel role of maternal TH in promoting IPCs derived neuronal differentiation in developing neo-cortex. We have also shown for the first time that ventricular zone progenitors are TH responsive as they express its receptor, TR alpha-1, transporters (MCT8) and deiodinases. This study highlights the importance of maternal thyroid hormone (TH) even before the start of the fetal thyroid function.

  20. Growth Hormone Deficiency after Treatment of Acromegaly: A Randomized, Placebo-Controlled Study of Growth Hormone Replacement

    PubMed Central

    Miller, Karen K.; Wexler, Tamara; Fazeli, Pouneh; Gunnell, Lindsay; Graham, Gwenda J.; Beauregard, Catherine; Hemphill, Linda; Nachtigall, Lisa; Loeffler, Jay; Swearingen, Brooke; Biller, Beverly M. K.; Klibanski, Anne

    2010-01-01

    Context: The effects of GH replacement therapy in patients who develop GH deficiency (GHD) after cure of acromegaly have not been established in a placebo-controlled study. Objective: The objective of the study was to determine whether GH replacement improves body composition, cardiovascular risk markers and quality of life in patients with GHD and prior acromegaly. Design: This was a 6-month, randomized, placebo-controlled study. Setting: The study was conducted at a clinical translational science center. Study Participants: Participants included 30 subjects with prior acromegaly and current GHD. Intervention: Interventions included GH or placebo. Main Outcome Measures: Body composition (dual-energy x-ray absorptiometry and cross-sectional computed tomography at L4), cardiovascular risk markers (high-sensitivity C-reactive protein (hsCRP), total, high-density lipoprotein and low-density lipoprotein cholesterol, fibrinogen, and carotid intimal-medial thickness), and quality of life were measured. Results: The mean GH dose at 6 months was 0.58 ± 0.26 mg/d. Total fat mass, visceral adipose tissue (−15.3 ± 18.6 vs. 1.3 ± 12.5%, P = 0.01), and total abdominal fat decreased, and fat-free mass increased, in the GH vs. placebo group. Mean hsCRP levels decreased, but there was no GH effect on other cardiovascular risk markers. There was no change in glycosylated hemoglobin or homeostasis model assessment insulin resistance index. Quality of life improved with GH. Side effects were minimal. Conclusions: This is the first randomized, placebo-controlled study of the effects of GH replacement therapy on body composition and cardiovascular end points in patients who have developed GH deficiency after treatment for acromegaly, a disease complicated by metabolic and body composition alterations and increased cardiovascular risk. GH replacement decreased visceral adipose tissue, increased fat-free mass, decreased hsCRP, and improved quality of life in patients with GHD after

  1. The ketogenic diet in children with Glut1 deficiency syndrome and epilepsy.

    PubMed

    Rauchenzauner, Markus; Klepper, Jörg; Leiendecker, Bärbel; Luef, Gerhard; Rostasy, Kevin; Ebenbichler, Christoph

    2008-11-01

    The effects of a long-term ketogenic diet in children with Glut1 deficiency syndrome on metabolism are unknown. Our results indicate a characteristic effect of a long-term ketogenic diet on glucose and lipid homeostasis in Glut1 deficiency syndrome. Although serum lipids and apolipoproteins reflect a proatherogenic lipoprotein profile, adipocytokine constellation is not indicative of enhanced cardiovascular risk.

  2. The Cognitive and Social-Emotional Correlates of Color Deficiency in Children.

    ERIC Educational Resources Information Center

    Wilkinson, William K.

    Although color vision deficiency is a prevalent problem in childhood, little is known about the cognitive and social-emotional variables linked to this difficulty. A review of the literature revealed seven empirical articles exploring the psychological functioning of color-deficient children, with the results yielding contradictory findings. The…

  3. Postnatal growth hormone deficiency in growing rats causes marked decline in the activity of spinal cord acetylcholinesterase but not butyrylcholinesterase.

    PubMed

    Koohestani, Faezeh; Brown, Chester M; Meisami, Esmail

    2012-11-01

    The effects of growth hormone (GH) deficiency on the developmental changes in the abundance and activity of cholinesterase enzymes were studied in the developing spinal cord (SC) of postnatal rats by measuring the specific activity of acetylcholinesterase (AChE), a marker for cholinergic neurons and their synaptic compartments, and butyrylcholinesterase (BuChE), a marker for glial cells and neurovascular cells. Specific activities of these two enzymes were measured in SC tissue of 21- and 90 day-old (P21, weaning age; P90, young adulthood) GH deficient spontaneous dwarf (SpDwf) mutant rats which lack anterior pituitary and circulating plasma GH, and were compared with SC tissue of normal age-matched control animals. Assays were carried out for AChE and BuChE activity in the presence of their specific chemical inhibitors, BW284C51 and iso-OMPA, respectively. Results revealed that mean AChE activity was markedly and significantly reduced [28% at P21, 49% at P90, (p<0.01)] in the SC of GH deficient rats compared to age-matched controls. GH deficiency had a higher and more significant effect on AChE activity of the older (P90) rats than the younger ones (P21) ones. In contrast, BuChE activity in SC showed no significant changes in GH deficient rats at either of the two ages studied. Results imply that, in the absence of pituitary GH, the postnatal proliferation of cholinergic synapses in the rat SC, a CNS structure, where AChE activity is abundant, is markedly reduced during both the pre- and postweaning periods; more so in the postweaning than preweaning ages. In contrast, the absence of any effects on BuChE activity implies that GH does not affect the development of non-neuronal elements, e.g., glia, as much as the neuronal and synaptic compartments of the developing rat SC.

  4. Combinatorial effects of malaria season, iron deficiency, and inflammation determine plasma hepcidin concentration in African children

    PubMed Central

    Armitage, Andrew E.; Khandwala, Shivani; Mwangi, Tabitha W.; Uyoga, Sophie; Bejon, Philip A.; Williams, Thomas N.; Prentice, Andrew M.; Drakesmith, Hal

    2014-01-01

    Hepcidin is the master regulatory hormone that governs iron homeostasis and has a role in innate immunity. Although hepcidin has been studied extensively in model systems, there is less information on hepcidin regulation in global health contexts where iron deficiency (ID), anemia, and high infectious burdens (including malaria) all coexist but fluctuate over time. We evaluated iron status, hepcidin levels, and determinants of hepcidin in 2 populations of rural children aged ≤8 years, in the Gambia and Kenya (total n = 848), at the start and end of a malaria season. Regression analyses and structural equation modeling demonstrated, for both populations, similar combinatorial effects of upregulating stimuli (iron stores and to a lesser extent inflammation) and downregulating stimuli (erythropoietic drive) on hepcidin levels. However, malaria season was also a significant factor and was associated with an altered balance of these opposing factors. Consistent with these changes, hepcidin levels were reduced whereas the prevalence of ID was increased at the end of the malaria season. More prevalent ID and lower hepcidin likely reflect an enhanced requirement for iron and an ability to efficiently absorb it at the end of the malaria season. These results, therefore, have implications for ID and malaria control programs. PMID:24596418

  5. Combinatorial effects of malaria season, iron deficiency, and inflammation determine plasma hepcidin concentration in African children.

    PubMed

    Atkinson, Sarah H; Armitage, Andrew E; Khandwala, Shivani; Mwangi, Tabitha W; Uyoga, Sophie; Bejon, Philip A; Williams, Thomas N; Prentice, Andrew M; Drakesmith, Hal

    2014-05-22

    Hepcidin is the master regulatory hormone that governs iron homeostasis and has a role in innate immunity. Although hepcidin has been studied extensively in model systems, there is less information on hepcidin regulation in global health contexts where iron deficiency (ID), anemia, and high infectious burdens (including malaria) all coexist but fluctuate over time. We evaluated iron status, hepcidin levels, and determinants of hepcidin in 2 populations of rural children aged ≤8 years, in the Gambia and Kenya (total n = 848), at the start and end of a malaria season. Regression analyses and structural equation modeling demonstrated, for both populations, similar combinatorial effects of upregulating stimuli (iron stores and to a lesser extent inflammation) and downregulating stimuli (erythropoietic drive) on hepcidin levels. However, malaria season was also a significant factor and was associated with an altered balance of these opposing factors. Consistent with these changes, hepcidin levels were reduced whereas the prevalence of ID was increased at the end of the malaria season. More prevalent ID and lower hepcidin likely reflect an enhanced requirement for iron and an ability to efficiently absorb it at the end of the malaria season. These results, therefore, have implications for ID and malaria control programs.

  6. The effect of vitamin D therapy on hematological indices in children with vitamin D deficiency.

    PubMed

    Soliman, Ashraf T; Eldabbagh, Muhamed; Elawwa, Ahmed; Ashour, Rasha; Saleem, Wael

    2012-12-01

    We investigated the effect of vitamin D on hematological indices, blood pressure (BP) and heart rate (HR) in children with vitamin D deficiency before and after treatment. Vitamin D deficiency does not have a significant effect on red blood cell count and indices, total and differential white blood cell count, or on BP and HR. A mega-dose vitamin D therapy did not have a significant effect on all these parameters in children.

  7. Tetrac can replace thyroid hormone during brain development in mouse mutants deficient in the thyroid hormone transporter mct8.

    PubMed

    Horn, Sigrun; Kersseboom, Simone; Mayerl, Steffen; Müller, Julia; Groba, Claudia; Trajkovic-Arsic, Marija; Ackermann, Tobias; Visser, Theo J; Heuer, Heike

    2013-02-01

    The monocarboxylate transporter 8 (MCT8) plays a critical role in mediating the uptake of thyroid hormones (THs) into the brain. In patients, inactivating mutations in the MCT8 gene are associated with a severe form of psychomotor retardation and abnormal serum TH levels. Here, we evaluate the therapeutic potential of the TH analog 3,5,3',5'-tetraiodothyroacetic acid (tetrac) as a replacement for T(4) in brain development. Using COS1 cells transfected with TH transporter and deiodinase constructs, we could show that tetrac, albeit not being transported by MCT8, can be metabolized to the TH receptor active compound 3,3',5-triiodothyroacetic acid (triac) by type 2 deiodinase and inactivated by type 3 deiodinase. Triac in turn is capable of replacing T(3) in primary murine cerebellar cultures where it potently stimulates Purkinje cell development. In vivo effects of tetrac were assessed in congenital hypothyroid Pax8-knockout (KO) and Mct8/Pax8 double-KO mice as well as in Mct8-KO and wild-type animals after daily injection of tetrac (400 ng/g body weight) during the first postnatal weeks. This treatment was sufficient to promote TH-dependent neuronal differentiation in the cerebellum, cerebral cortex, and striatum but was ineffective in suppressing hypothalamic TRH expression. In contrast, TSH transcript levels in the pituitary were strongly down-regulated in response to tetrac. Based on our findings we propose that tetrac administration offers the opportunity to provide neurons during the postnatal stage with a potent TH receptor agonist, thereby eventually reducing the neurological damage in patients with MCT8 mutations without deteriorating the thyrotoxic situation in peripheral tissues.

  8. The sweating apparatus in growth hormone deficiency, following treatment with r-hGH and in acromegaly.

    PubMed

    Hasan, W; Cowen, T; Barnett, P S; Elliot, E; Coskeran, P; Bouloux, P M

    2001-06-20

    Adult growth hormone deficient patients are known to exhibit reduced sweating and their ability to thermoregulate is diminished. Treatment of these patients with recombinant human growth hormone (r-hGH) is claimed to reverse these abnormalities. We have investigated this claim, as well as the mechanism underlying these altered sweating responses in GH-deficient patients as part of a placebo-controlled study on the effects of 6-12 months r-hGH therapy. Skin biopsies were obtained from these subjects and changes in morphology and innervation parameters for the eccrine sweat glands were examined. These included histochemistry for acetylcholinesterase (AChE) and immunohistochemistry for the neuropeptide vasoactive intestinal polypeptide (VIP) and for PGP9.5, a general neuronal marker. Sweat gland acinar size and periacinar innervation were measured by computerised image analysis. The patients underwent pilocarpine iontophoresis sweat rate tests and their serum insulin-like growth factor 1 (IGF-1) levels were assessed. Since active acromegaly involves excess GH secretion and hyperhidrosis, skin biopsies and sweat tests were also carried out on a group of these patients, as well as on control subjects. We have demonstrated a sweating defect in adult GH-deficiency which is accompanied by a reduction in AChE and VIP levels in the nerve supply to sweat glands. Following r-hGH therapy, an increase in AChE and VIP staining is seen in the sudomotor nerves accompanied by restoration of sweat rates and serum IGF-1 levels. Hence, normalization of sweat gland function includes recovery of sudomotor synapse constituents. A trophic effect of GH on sweat gland epithelium and/or on the associated nerves is proposed, supported by the observation that in acromegaly the size of sweat gland acini and the density of innervation to the sweat glands was greater than in controls.

  9. Participation and quality of life in children and adolescents with congenital limb deficiencies: A narrative review.

    PubMed

    Michielsen, Anka; Van Wijk, Iris; Ketelaar, Marjolijn

    2010-12-01

    Children and adolescents with congenital limb deficiencies are visibly and physically different from their peers. They present limitations in activities, depending on the severity of deficiency. Therefore they are at risk for lower participation in social and leisure activities. This might negatively influence the perception on their quality of life. The aim of this narrative review is to describe participation and quality of life in children with congenital limb deficiencies. Participation and quality of life are relatively new concepts. Psychosocial functioning, being closely related to the concept of quality of life, is described as well. A comprehensive review of the literature was conducted on participation, quality of life and psychosocial functioning in children and adolescents with congenital limb deficiencies. The review involved a systematic search using multiple data sources. Fifteen cross-sectional studies were included in this review. The literature to date provides limited knowledge on how children and adolescents with congenital limb deficiencies participate and how they perceive their quality of life. The psychosocial functioning, although described as at risk, appears to be comparable to healthy peers. In conclusion, more research is needed on how children and adolescents with congenital limb deficiencies participate and how they perceive their quality of life. A broader perspective will not only help parents in making the right choices for their children, but can also have implications for health care providers, teachers and agencies funding rehabilitation services.

  10. Hormone-sensitive lipase deficiency suppresses insulin secretion from pancreatic islets of Lep{sup ob/ob} mice

    SciTech Connect

    Sekiya, Motohiro; Yahagi, Naoya; Tamura, Yoshiaki; Okazaki, Hiroaki; Igarashi, Masaki; Ohta, Keisuke; Takanashi, Mikio; Kumagai, Masayoshi; Takase, Satoru; Nishi, Makiko; Takeuchi, Yoshinori; Izumida, Yoshihiko; Kubota, Midori; Ohashi, Ken; Iizuka, Yoko; Yagyu, Hiroaki; Gotoda, Takanari; Nagai, Ryozo; Shimano, Hitoshi; Yamada, Nobuhiro; and others

    2009-09-25

    It has long been a matter of debate whether the hormone-sensitive lipase (HSL)-mediated lipolysis in pancreatic {beta}-cells can affect insulin secretion through the alteration of lipotoxicity. We generated mice lacking both leptin and HSL (Lep{sup ob/ob}/HSL{sup -/-}) and explored the role of HSL in pancreatic {beta}-cells in the setting of obesity. Lep{sup ob/ob}/HSL{sup -/-} developed elevated blood glucose levels and reduced plasma insulin levels compared with Lep{sup ob/ob}/HSL{sup +/+} in a fed state, while the deficiency of HSL did not affect glucose homeostasis in Lep{sup +/+} background. The deficiency of HSL exacerbated the accumulation of triglycerides in Lep{sup ob/ob} islets, leading to reduced glucose-stimulated insulin secretion. The deficiency of HSL also diminished the islet mass in Lep{sup ob/ob} mice due to decreased cell proliferation. In conclusion, HSL affects insulin secretary capacity especially in the setting of obesity.

  11. Iron-deficiency Anemia in Children with Febrile Seizure: A Case-Control Study

    PubMed Central

    GHASEMI, Fateme; VALIZADEH, Fateme; TAEE, Nadere

    2014-01-01

    Objective Considering the recurrence of febrile seizure and costs for families, many studies have attempted to identify its risk factors. Some recent studies have reported that anemia is more common in children with febrile convulsion, whereas others have reported that iron deficiency raises the seizure threshold. This study was done to compare iron-deficiency anemia in children with first FS with children having febrile illness alone and with healthy children. Materials & Methods This case-control study evaluated 300 children in three groups (first FS, febrile without convulsion, and healthy) in Khoramabad Madani Hospital from September 2009 to September 2010. Body temperature on admission was measured using the tympanic method. CBC diff, MCV, MCH, MCHC, serum iron, plasma ferritin and TIBC tests were performed for all participants. Data were analyzed by frequency, mean, standard deviation, ANOVA, and chi-square statistical tests. Odds ratios were estimated by logistic regression at a confidence level of 95%. Results Forty percent of the cases with FS had iron-deficiency anemia, compared to 26% of children with febrile illness without seizure and 12% of healthy children. The Odds ratio for iron-deficiency anemia in the patients with FS was 1.89 (95% CI, 1.04-5.17) compared to the febrile children without convulsion and 2.21 (95% CI, 1.54-3.46) compared to the healthy group. Conclusion Children with FS are more likely to be iron-deficient than those with febrile illness alone and healthy children. Thus, iron-deficiency anemia could be a risk factor for FS. PMID:24949050

  12. Induction of chronic growth hormone deficiency by anti-GH serum

    NASA Technical Reports Server (NTRS)

    Grindeland, R. E.; Smith, A. T.; Ellis, S.; Evans, E. S.

    1974-01-01

    The observations reported indicate that the growth rate of neonatal rats can be specifically inhibited for at least 78 days following the administration of antisera against growth hormone (GH) for only four days after birth. The inhibition can be correlated with a marked deficit of tibial growth promoting activity in the pituitary but not with the plasma concentrations of immuno-reactive GH.

  13. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency.

    PubMed

    Knisely, A S; Strautnieks, Sandra S; Meier, Yvonne; Stieger, Bruno; Byrne, Jane A; Portmann, Bernard C; Bull, Laura N; Pawlikowska, Ludmila; Bilezikçi, Banu; Ozçay, Figen; László, Aranka; Tiszlavicz, László; Moore, Lynette; Raftos, Jeremy; Arnell, Henrik; Fischler, Björn; Németh, Antal; Papadogiannakis, Nikos; Cielecka-Kuszyk, Joanna; Jankowska, Irena; Pawłowska, Joanna; Melín-Aldana, Hector; Emerick, Karan M; Whitington, Peter F; Mieli-Vergani, Giorgina; Thompson, Richard J

    2006-08-01

    Hepatocellular carcinoma (HCC) is rare in young children. We attempted to see if immunohistochemical and mutational-analysis studies could demonstrate that deficiency of the canalicular bile acid transporter bile salt export pump (BSEP) and mutation in ABCB11, encoding BSEP, underlay progressive familial intrahepatic cholestasis (PFIC)--or "neonatal hepatitis" suggesting PFIC--that was associated with HCC in young children. We studied 11 cases of pediatric HCC in the setting of PFIC or "neonatal hepatitis" suggesting PFIC. Archival liver were retrieved and immunostained for BSEP. Mutational analysis of ABCB11 was performed in leukocyte DNA from available patients and parents. Among the 11 nonrelated children studied aged 13-52 months at diagnosis of HCC, 9 (and a full sibling, with neonatal hepatitis suggesting PFIC, of a tenth from whom liver was not available) had immunohistochemical evidence of BSEP deficiency; the eleventh child did not. Mutations in ABCB11 were demonstrated in all patients with BSEP deficiency in whom leukocyte DNA could be studied (n = 7). These mutations were confirmed in the parents (n = 14). With respect to the other 3 children with BSEP deficiency, mutations in ABCB11 were demonstrated in all 5 parents in whom leukocyte DNA could be studied. Thirteen different mutations were found. In conclusion, PFIC associated with BSEP deficiency represents a previously unrecognized risk for HCC in young children. Immunohistochemical evidence of BSEP deficiency correlates well with demonstrable mutation in ABCB11.

  14. Non-invasive screening of cytochrome c oxidase deficiency in children using a dipstick immunocapture assay.

    PubMed

    Rodinová, M; Trefilová, E; Honzík, T; Tesařová, M; Zeman, J; Hansíková, H

    2014-01-01

    Cytochrome c oxidase (CIV) deficiency is among the most common childhood mitochondrial disorders. The diagnosis of this deficiency is complex, and muscle biopsy is used as the gold standard of diagnosis. Our aim was to minimize the patient burden and to test the use of a dipstick immunocapture assay (DIA) to determine the amount of CIV in non-invasively obtained buccal epithelial cells. Buccal smears were obtained from five children with Leigh syndrome including three children exhibiting a previously confirmed CIV deficiency in muscle and fibroblasts and two children who were clinical suspects for CIV deficiency; the smear samples were analysed using CI and CIV human protein quantity dipstick assay kits. Samples from five children of similar age and five adults were used as controls. Analysis of the controls demonstrated that only samples of buccal cells that were frozen for a maximum of 4 h after collection provide accurate results. All three patients with confirmed CIV deficiency due to mutations in the SURF1 gene exhibited significantly lower amounts of CIV than the similarly aged controls; significantly lower amounts were also observed in two new patients, for whom later molecular analysis also confirmed pathologic mutations in the SURF1 gene. We conclude that DIA is a simple, fast and sensitive method for the determination of CIV in buccal cells and is suitable for the screening of CIV deficiency in non-invasively obtained material from children who are suspected of having mitochondrial disease.

  15. Can Maori Children Really Be Positioned as "Deficient" Learners for Reading English?

    ERIC Educational Resources Information Center

    Harris, Fleur

    2009-01-01

    Maori are the indigenous people of New Zealand. Since British colonial settlement in the early 1800s, Maori children have been predominantly educated in an English-speaking system dominated by colonial governance. In this institution, Maori children have been constructed as deficient learners, primarily in relation to a colonial curriculum taught…

  16. Educational Interventions for Visual-Motor Deficiencies That Affect Handwriting in School-Aged Children.

    ERIC Educational Resources Information Center

    Dikowski, Timothy J.

    This practicum was designed to remediate handwriting skills in school-aged children who displayed visual-motor deficiencies that affect mechanical skills. Practicum goals were to: (1) identify and diagnose children with handwriting delays; (2) involve school and parent interaction by involving them with pre- and post-program assessment; (3)…

  17. Mathematics Deficiencies in Children with Very Low Birth Weight or Very Preterm Birth

    ERIC Educational Resources Information Center

    Taylor, H. Gerry; Espy, Kimberly Andrews; Anderson, Peter J.

    2009-01-01

    Children with very low birth weight (VLBW, less than 1500 g) or very preterm birth (VPTB, less than 32 weeks gestational age or GA) have more mathematics disabilities or deficiencies (MD) and higher rates of mathematics learning disabilities (MLD) than normal birth weight term-born children (NBW, greater than 2500 g and greater than 36 weeks GA).…

  18. Plasma Amino Acids Profiles in Children with Autism: Potential Risk of Nutritional Deficiencies.

    ERIC Educational Resources Information Center

    Arnold, Georgianne L.; Hyman, Susan L.; Mooney, Robert A.; Kirby, Russell S.

    2003-01-01

    The plasma amino acid profiles of 10 children with autism on gluten and casein restricted diets and 26 on unrestricted diets were reviewed. There was a trend for the children on restricted diets to have an increased prevalence of essential amino acid deficiencies and lower plasma levels of essential acids. (Contains references.) (Author/CR)

  19. MOD-4023, a long-acting carboxy-terminal peptide-modified human growth hormone: results of a Phase 2 study in growth hormone-deficient adults

    PubMed Central

    Strasburger, Christian J; Vanuga, Peter; Payer, Juraj; Pfeifer, Marija; Popovic, Vera; Bajnok, László; Góth, Miklós; Olšovská, Veˇra; Trejbalová, L‘udmila; Vadasz, Janos; Fima, Eyal; Koren, Ronit; Amitzi, Leanne; Bidlingmaier, Martin; Hershkovitz, Oren; Biller, Beverly M K

    2016-01-01

    Objective Growth hormone (GH) replacement therapy currently requires daily injections, which may cause distress and low compliance. C-terminal peptide (CTP)-modified growth hormone (MOD-4023) is being developed as a once-weekly dosing regimen in patients with GH deficiency (GHD). This study’s objective is to evaluate the safety, pharmacokinetics (PK), pharmacodynamics (PD) and efficacy of MOD-4023 administered once-weekly in GHD adults. Design 54 adults with GHD currently treated with daily GH were normalized and randomized into 4 weekly dosing cohorts of MOD-4023 at 18.5%, 37%, 55.5% or 123.4% of individual cumulative weekly molar hGH dose. The study included 2 stages: Stage A assessed the effectiveness and PK/PD profiles of the 4 dosing regimens of MOD-4023. Stage B was an extension period of once-weekly MOD-4023 administration (61.7% molar hGH content) to collect further safety data and confirm the results from Stage A. Results Dose-dependent response was observed for both PK and PD data of weekly MOD-4023 treatment. Insulin-like growth factor I (IGF-I) SDS levels were maintained within normal range. The 18.5% cohort was discontinued due to low efficacy. MOD-4023 was well tolerated and exhibited favorable safety profile in all dose cohorts. The reported adverse events were consistent with known GH-related side effects. Conclusions Once-weekly MOD-4023 administration in GHD adults was found to be clinically effective while maintaining a favorable safety profile and may obviate the need for daily injections. Weekly GH injections may improve compliance and overall outcome. The promising results achieved in this Phase 2 study led to a pivotal Phase 3 trial, which is currently ongoing. PMID:27932411

  20. Insulin-like growth factor-binding protein-2 levels in pediatric patients with growth hormone deficiency, eating disorders and acute lymphoblastic leukemia.

    PubMed

    Barrios, V; Buño, M; Pozo, J; Muñoz, M T; Argente, J

    2000-01-01

    Insulin-like growth factor (IGF)-binding protein-2 (IGFBP-2) is altered in different diseases and might be used as an indication of its severity. The aims of our study were to investigate: (1) the developmental pattern of the serum IGFBP-2 concentration at birth and during childhood and adolescence; (2) whether the serum IGFBP-2 level could be a marker for the diagnosis and evolution of diseases where the growth hormone (GH)-IGF axis is altered, and (3) whether this binding protein shows a relationship with IGF-I, its free fraction, IGFBP-1 and -3. We report reference values for 55 normal full-term newborns and 221 normal children who were divided into 5 groups according to their Tanner stage. Serum levels were higher in newborns when compared with Tanner stages I-V (p < 0.001, ANOVA), with no further changes throughout development. Furthermore, we studied IGFBP-2 levels in 24 children with congenital GH deficiency (GHD), 26 with acute lymphoblastic leukemia (ALL), 75 obese children, and 60 girls with anorexia nervosa (AN) at diagnosis and during a follow-up period. IGFBP-2 at diagnosis was increased in GHD, ALL and AN, and decreased in obesity (p < 0.05, ANOVA). During the follow-up, IGFBP-2 concentrations tended to normalize. IGFBP-2 correlated positively with IGFBP-1 and negatively with IGF-I and IGFBP-3 in normal subjects and at diagnosis of the pathologies studied. Although IGFBP-2 functions are not well understood, these results suggest a possible role for this protein in diseases where the GH-IGF axis is altered.

  1. Growth hormone deficiency and central precocious puberty in Klinefelter syndrome: report of a case and review of KIGS database.

    PubMed

    Gonzales-Ellis, Bryn A; Pingul, Mia M; Reddy, Sujana; Boney, Charlotte M; Wajnrajch, Michael P; Quintos, Jose Bernarto

    2013-01-01

    Growth hormone deficiency (GHD) and central precocious puberty (CPP) have each, individually, been described in patients with Klinefelter syndrome. However, the combination of GHD, CPP, and Klinefelter syndrome has never been reported. We described a Klinefelter syndrome patient who developed GHD at age 2 10/12 years and CPP at 8 6/12 years. Despite CPP, GnRH agonist therapy was not initiated because of his excellent predicted adult height. At 11 8/12 years, his height was 164.6 cm, close to his mid-parental target height of 165 cm. We report an additional nine patients with Klinefelter syndrome and GHD from the Pfizer International Growth Study (KIGS) database, none of whom had CPP. We conclude that the combination of GHD and CPP is very rare in Klinefelter syndrome and that CPP is unlikely to compromise final adult height.

  2. Response to long-term growth hormone therapy in patients affected by RASopathies and growth hormone deficiency: Patterns of growth, puberty and final height data.

    PubMed

    Tamburrino, Federica; Gibertoni, Dino; Rossi, Cesare; Scarano, Emanuela; Perri, Annamaria; Montanari, Francesca; Fantini, Maria Pia; Pession, Andrea; Tartaglia, Marco; Mazzanti, Laura

    2015-11-01

    RASopathies are developmental disorders caused by heterozygous germline mutations in genes encoding proteins in the RAS-MAPK signaling pathway. Reduced growth is a common feature. Several studies generated data on growth, final height (FH), and height velocity (HV) after growth hormone (GH) treatment in patients with these disorders, particularly in Noonan syndrome, the most common RASopathy. These studies, however, refer to heterogeneous cohorts in terms of molecular information, GH status, age at start and length of therapy, and GH dosage. This work reports growth data in 88 patients affected by RASopathies with molecularly confirmed diagnosis, together with statistics on body proportions, pubertal pattern, and FH in 33, including 16 treated with GH therapy for proven GH deficiency. Thirty-three patients showed GH deficiency after pharmacological tests, and were GH-treated for an average period of 6.8 ± 4.8 years. Before starting therapy, HV was -2.6 ± 1.3 SDS, and mean basal IGF1 levels were -2.0 ± 1.1 SDS. Long-term GH therapy, starting early during childhood, resulted in a positive height response compared with untreated patients (1.3 SDS in terms of height-gain), normalizing FH for Ranke standards but not for general population and Target Height. Pubertal timing negatively affected pubertal growth spurt and FH, with IGF1 standardized score increased from -2.43 to -0.27 SDS. During GH treatment, no significant change in bone age velocity, body proportions, or cardiovascular function was observed.

  3. Improved adipose tissue metabolism after 5-year growth hormone replacement therapy in growth hormone deficient adults: The role of zinc-α2-glycoprotein

    PubMed Central

    Balaž, Miroslav; Ukropcova, Barbara; Kurdiova, Timea; Vlcek, Miroslav; Surova, Martina; Krumpolec, Patrik; Vanuga, Peter; Gašperíková, Daniela; Klimeš, Iwar; Payer, Juraj; Wolfrum, Christian; Ukropec, Jozef

    2014-01-01

    Growth hormone (GH) supplementation therapy to adults with GH deficiency has beneficial effects on adipose tissue lipid metabolism, improving thus adipocyte functional morphology and insulin sensitivity. However, molecular nature of these effects remains unclear. We therefore tested the hypothesis that lipid-mobilizing adipokine zinc-α2-glycoprotein is causally linked to GH effects on adipose tissue lipid metabolism. Seventeen patients with severe GH deficiency examined before and after the 5-year GH replacement therapy were compared with age-, gender- and BMI-matched healthy controls. Euglycemic hyperinsulinemic clamp was used to assess whole-body and adipose tissue-specific insulin sensitivity. Glucose tolerance was determined by oGTT, visceral and subcutaneous abdominal adiposity by MRI, adipocyte size morphometrically after collagenase digestion, lipid accumulation and release was studied in differentiated human primary adipocytes in association with GH treatment and zinc-α2-glycoprotein gene silencing. Five-year GH replacement therapy improved glucose tolerance, adipose tissue insulin sensitivity and reduced adipocyte size without affecting adiposity and whole-body insulin sensitivity. Adipose tissue zinc-α2-glycoprotein expression was positively associated with whole-body and adipose tissue insulin sensitivity and negatively with adipocyte size. GH treatment to adipocytes in vitro increased zinc-α2-glycoprotein expression (>50%) and was paralleled by enhanced lipolysis and decreased triglyceride accumulation (>35%). Moreover, GH treatment improved antilipolytic action of insulin in cultured adipocytes. Most importantly, silencing zinc-α2-glycoprotein eliminated all of the GH effects on adipocyte lipid metabolism. Effects of 5-year GH supplementation therapy on adipose tissue lipid metabolism and insulin sensitivity are associated with zinc-α2-glycoprotein. Presence of this adipokine is required for the GH action on adipocyte lipid metabolism in vitro

  4. Effects of iron deficiency on attention and learning processes in preschool children: Bandung, Indonesia.

    PubMed

    Soewondo, S; Husaini, M; Pollitt, E

    1989-09-01

    A double-blind clinical trial was conducted in Indonesia to assess effects of iron supplementation on performance of iron-depleted and iron-deficient anemic children in discrimination and oddity learning tasks. Half these children received elemental Fe for 8 wk; the others received a placebo. There were significant changes from pre- to postintervention evaluations in ferritin, transferrin saturation, free erythrocyte protoporphyrin, and hemoglobin among the anemic and iron-depleted children; no changes were observed among the placebos or any of the iron-replete children. The magnitude of hematological changes in anemic children treated with iron was small; yet, after treatment the children's mean ferritin, transferrin saturation, and hemoglobin values were above the cutoff points used for the definition of iron-deficiency anemia (IDA). Pre- and posttreatment psychological test data show that IDA produces alterations in cognitive processes related to visual attention and concept acquisition, alterations reversed with iron treatment.

  5. Iron deficiency associated with higher blood lead in children living in contaminated environments.

    PubMed Central

    Bradman, A; Eskenazi, B; Sutton, P; Athanasoulis, M; Goldman, L R

    2001-01-01

    The evidence that iron deficiency increases lead child exposure is based primarily on animal data and limited human studies, and some of this evidence is contradictory. No studies of iron status and blood lead levels in children have accounted for environmental lead contamination and, therefore, the source of their exposure. Thus, no studies have directly determined whether iron deficiency modifies the relationship of environmental lead and blood lead. In this study, we compared blood lead levels of iron-deficient and iron-replete children living in low, medium, or highly contaminated environments. Measurements of lead in paint, soil, dust, and blood, age of housing, and iron status were collected from 319 children ages 1-5. We developed two lead exposure factors to summarize the correlated exposure variables: Factor 1 summarized all environmental measures, and Factor 2 was weighted for lead loading of house dust. The geometric mean blood lead level was 4.9 microg/dL; 14% exceeded 10 microg/dL. Many of the children were iron deficient (24% with ferritin < 12 ng/dL). Seventeen percent of soil leads exceeded 500 microg/g, and 23% and 63% of interior and exterior paint samples exceeded 5,000 microg/g. The unadjusted geometric mean blood lead level for iron-deficient children was higher by 1 microg/dL; this difference was greater (1.8 microg/dL) after excluding Asians. Blood lead levels were higher for iron-deficient children for each tertile of exposure as estimated by Factors 1 and 2 for non-Asian children. Elevated blood lead among iron-deficient children persisted after adjusting for potential confounders by multivariate regression; the largest difference in blood lead levels between iron-deficient and -replete children, approximately 3 microg/dL, was among those living in the most contaminated environments. Asian children had a paradoxical association of sufficient iron status and higher blood lead level, which warrants further investigation. Improving iron status

  6. Cloning and characterization of mouse growth hormone-releasing hormone (GRH) complementary DNA: increased GRH messenger RNA levels in the growth hormone-deficient lit/lit mouse.

    PubMed

    Frohman, M A; Downs, T R; Chomczynski, P; Frohman, L A

    1989-10-01

    We have isolated and cloned the full length cDNA for mouse GH-releasing hormone (mGRH) from mouse hypothalamus using a recently described strategy involving the polymerase chain reaction technique (PCR). Degenerate oligonucleotide primers were selected based on short (six amino acids) conserved regions in the human and rat GRH peptides that would recognize DNA sequences encoding similar amino acids regardless of codon usage. Primer-extended cDNA was amplified by PCR on cDNA templates prepared by reverse transcribing total mouse hypothalamic RNA. After cloning and sequencing the initial product, the 3' and 5' ends of mGRH were generated using a separate PCR strategy (RACE protocol). The mGRH cDNA encodes a 103-amino acid reading frame, structurally similar to the human and rat GRH genes, containing a signal sequence, a 42-residue GRH peptide, and a 31-residue C-terminal region. Although the structures of mouse and rat GRH are highly conserved in the signal peptide and C-terminal region, there is considerable diversity in the GRH region, which exhibits nearly comparable homology with the rat (68%) and human (62%) structures. Differences between mouse and rat GRH were also found in the amino acid cleavage sites at the 5' and 3' ends of the mature peptide and at the polyadenylation signal.(ABSTRACT TRUNCATED AT 250 WORDS)

  7. Iron, Anemia, and Iron Deficiency Anemia among Young Children in the United States

    PubMed Central

    Gupta, Priya M.; Perrine, Cria G.; Mei, Zuguo; Scanlon, Kelley S.

    2016-01-01

    Iron deficiency and anemia are associated with impaired neurocognitive development and immune function in young children. Total body iron, calculated from serum ferritin and soluble transferrin receptor concentrations, and hemoglobin allow for monitoring of the iron and anemia status of children in the United States. The purpose of this analysis is to describe the prevalence of iron deficiency (ID), anemia, and iron deficiency anemia (IDA) among children 1–5 years using data from the 2007–2010 National Health and Nutrition Examination Survey (NHANES). Prevalence of ID, anemia, and IDA among children 1–5 years was 7.1% (5.5, 8.7), 3.2% (2.0, 4.3), and 1.1% (0.6, 1.7), respectively. The prevalence of both ID and anemia were higher among children 1–2 years (p < 0.05). In addition, 50% of anemic children 1–2 years were iron deficient. This analysis provides an update on the prevalence of ID, anemia, and IDA for a representative sample of US children. Our results suggest little change in these indicators over the past decade. Monitoring of ID and anemia is critical and prevention of ID in early childhood should remain a public health priority. PMID:27249004

  8. Reduced autophagy in livers of fasted, fat-depleted, ghrelin-deficient mice: Reversal by growth hormone

    PubMed Central

    Zhang, Yuanyuan; Fang, Fei; Goldstein, Joseph L.; Brown, Michael S.; Zhao, Tong-Jin

    2015-01-01

    Plasma growth hormone (GH) and hepatic autophagy each have been reported to protect against hypoglycemia in the fasted state, but previous data have not linked the two. Here we demonstrate a connection using a mouse model of fasting in a fat-depleted state. Mice were subjected to 1 wk of 60% calorie restriction, causing them to lose nearly all body fat. They were then fasted for 23 h. During fasting, WT mice developed massive increases in plasma GH and a concomitant increase in hepatic autophagy, allowing them to maintain viable levels of blood glucose. In contrast, lethal hypoglycemia occurred in mice deficient in the GH secretagogue ghrelin as a result of knockout of the gene encoding ghrelin O-acyltransferase (GOAT), which catalyzes a required acylation of the peptide. Fasting fat-depleted Goat−/− mice showed a blunted increase in GH and a marked decrease in hepatic autophagy. Restoration of GH by infusion during the week of calorie restriction maintained autophagy in the Goat−/− mice and prevented lethal hypoglycemia. Acute injections of GH after 7 d of calorie restriction also restored hepatic autophagy, but failed to increase blood glucose, perhaps owing to ATP deficiency in the liver. These data indicate that GH stimulation of autophagy is necessary over the long term, but not sufficient over the short term to maintain blood glucose levels in fasted, fat-depleted mice. PMID:25583513

  9. Short stature and growth hormone deficiency in a girl with encephalocraniocutaneous lipomatosis and Jaffe-Campanacci syndrome: a case report

    PubMed Central

    Choi, Eun mi; Jung, Nani; Shim, Ye Jee; Choi, Hee Joung; Kim, Joon Sik; Song, Kwang Soon; Lee, Hee Jung; Kim, Sang Pyo

    2016-01-01

    A 9-year-old Tajikistani girl presented to Keimyung University Dongsan Medical Center for evaluation of a skin lesion on her left eyelid, focal alopecia, unilateral ventricular dilatation, and aortic coarctation. She was diagnosed with encephalocraniocutaneous lipomatosis (ECCL) according to Moog's diagnostic criteria. Café-au-lait spots were found on the left side of her trunk. Multiple nonossifying fibromas were found on her left proximal humerus, left distal femur, both proximal tibias, and left proximal fibula, suggesting Jaffe-Campanacci syndrome (JCS), following imaging of the extremities. Many JCS cases with multiple Café-au-lait macules, multiple nonossifying fibromas may actually have Neurofibromatosis type-1 (NF1). Thus, comprehensive molecular analysis to exclude NF1 mutation was performed using her blood sample. The NF1 mutation was not found. Her height was under the 3rd percentile and her bone age was delayed as compared with her chronological age. Baseline growth hormone (GH) level was below the normal range. Using the insulin stimulation and levo-dihydroxyphenylalanine tests, GH deficiency was confirmed. We present a case of GH deficiency with typical features of ECCL and JCS. PMID:28164079

  10. Development of antibodies against growth hormone (GH) during rhGH therapy in a girl with idiopathic GH deficiency: a case report.

    PubMed

    Meazza, Cristina; Schaab, Michael; Pagani, Sara; Calcaterra, Valeria; Bozzola, Elena; Kratzsch, Juergen; Bozzola, Mauro

    2013-01-01

    A 12.5-year-old Italian girl was referred to our institute for progressive growth failure from the age of 6 years, with a height of 128.2 cm (-3.37 SDS) and a bone age of 9 years. Endocrinological evaluation revealed a partial growth hormone deficiency (GHD) and GH therapy was started at a dosage of 0.25 mg/kg/week. During the first 3 years, she showed an increase in growth rate and experienced pubertal development onset. Then a poor growth rate (2 cm/year=0.43 SDS) was observed, notwithstanding an increase in GH dosage (0.35 mg/kg/week) and good compliance. We found a positive anti-GH antibody titre (1:1850, cutoff 1/100), confirmed 6 months later (1:2035); the antibodies had low binding capacity (0.63 μg/mL) and were only partially capable of inhibiting the GH effect. However, GH treatment was discontinued, and after 3 months the antibody titre decreased (1:950). In conclusion, we suggest evaluation of anti-GH antibodies in GH-treated idiopathic GHD children in whom growth response decreases after some years of therapy.

  11. Association of thyroid hormones with obesity and metabolic syndrome in Japanese children.

    PubMed

    Minami, Yukako; Takaya, Ryuzo; Takitani, Kimitaka; Ishiro, Manabu; Okasora, Keisuke; Niegawa, Tomomi; Tamai, Hiroshi

    2015-09-01

    Obesity is associated with health consequences, and thyroid dysfunction may be an adaption to the increased energy expenditure in obesity. With the rising prevalence of obesity in childhood, the prevalence of metabolic syndrome may also increase. In the current study, we have shown gender differences in the association of thyroid hormones with obesity, and attempted to elucidate the relationship between thyroid hormones and anthropometric parameters and biochemical data in obese Japanese children. We analyzed anthropometric measurements, blood pressure, body composition, thyroid hormones, and lipid profiles in 283 obese children. The association between thyroid hormones and several parameters differed by gender. The free T3 to free T4 ratio (fT3/fT4) in boys was negatively associated with the quantitative insulin sensitivity check index, whereas in girls, thyroid-stimulating hormone levels were positively correlated with levels of glucose, diastolic blood pressure, and non-high density lipoprotein-cholesterol, and fT3/fT4 was positively correlated with uric acid levels. FT3/fT4 in boys with metabolic syndrome was relatively higher than in those without metabolic syndrome. The cause of gender differences is unknown. Therefore, further studies with larger sample sizes and a long-term follow-up period are needed to address the influence of thyroid hormones on various parameters.

  12. Association of thyroid hormones with obesity and metabolic syndrome in Japanese children

    PubMed Central

    Minami, Yukako; Takaya, Ryuzo; Takitani, Kimitaka; Ishiro, Manabu; Okasora, Keisuke; Niegawa, Tomomi; Tamai, Hiroshi

    2015-01-01

    Obesity is associated with health consequences, and thyroid dysfunction may be an adaption to the increased energy expenditure in obesity. With the rising prevalence of obesity in childhood, the prevalence of metabolic syndrome may also increase. In the current study, we have shown gender differences in the association of thyroid hormones with obesity, and attempted to elucidate the relationship between thyroid hormones and anthropometric parameters and biochemical data in obese Japanese children. We analyzed anthropometric measurements, blood pressure, body composition, thyroid hormones, and lipid profiles in 283 obese children. The association between thyroid hormones and several parameters differed by gender. The free T3 to free T4 ratio (fT3/fT4) in boys was negatively associated with the quantitative insulin sensitivity check index, whereas in girls, thyroid-stimulating hormone levels were positively correlated with levels of glucose, diastolic blood pressure, and non-high density lipoprotein-cholesterol, and fT3/fT4 was positively correlated with uric acid levels. FT3/fT4 in boys with metabolic syndrome was relatively higher than in those without metabolic syndrome. The cause of gender differences is unknown. Therefore, further studies with larger sample sizes and a long-term follow-up period are needed to address the influence of thyroid hormones on various parameters. PMID:26388669

  13. High prevalence of vitamin D insufficiency/deficiency in Dutch multi-ethnic obese children.

    PubMed

    Radhakishun, Nalini; van Vliet, Mariska; von Rosenstiel, Ines; Weijer, Olivier; Diamant, Michaela; Beijnen, Jos; Brandjes, Dees

    2015-02-01

    Vitamin D insufficiency/deficiency is common among non-white children; however, little is known about the prevalence of vitamin D insufficiency/deficiency in non-white obese children living in the Netherlands. Therefore, a retrospective analysis was performed on data from multi-ethnic Dutch children and adolescents 6–18 years who visited the obesity outpatient clinic in 2012–2013. We performed anthropometric measurements, oral glucose tolerance test, and measured 25(OH)D and lipid levels. Vitamin D insufficiency was defined as 25(OH)D levels 37.5- <50 nmol/L and vitamin D deficiency as 25(OH)D <37.5 nmol/L. In total, data from 387 children were obtained (mean age 11.6 years, 41.1 % boys, 10.3 % Dutch native, 25.6 % Turkish, 24.5 % Moroccan, 7.5 % African Surinamese, and 7.0 % West African). The median 25(OH)D level was 34 (range 12–105) nmol/L. In total, 17.8 % were vitamin D sufficient, 24.5 % with vitamin D insufficiency, and 57.6 % with vitamin D deficiency. Obese ethnic children showed the highest (87.5 %) and normal weight white children showed the lowest (20.0 %) prevalence of vitamin D insufficiency/deficiency . Conclusion: Vitamin D insufficiency and deficiency is extremely prevalent in treatment-seeking obese ethnic children. However, there was no evidence of an effect of vitamin D status on various components of the metabolic syndrome in our cohort.

  14. Screening for hormonal, monogenic, and syndromic disorders in obese infants and children.

    PubMed

    Mason, Kelly; Page, Laura; Balikcioglu, Pinar Gumus

    2014-09-01

    The prevalence of pediatric obesity in the United States is nearly 17%. Most cases are "exogenous", resulting from excess energy intake relative to energy expenditure over a prolonged period of time. However, some cases of obesity are "endogenous", associated with hormonal, genetic, or syndromic disorders such as hypothyroidism, Cushing's syndrome, growth hormone deficiency, defective leptin signaling, mutations in the melanocortin 4 receptor, and Prader-Willi and Bardet-Biedl syndromes. This article reviews the hormonal, monogenic, and syndromic causes of childhood obesity and identifies critical features that distinguish "endogenous" obesity disorders from the more common exogenous obesity. Findings that raise suspicion for endogenous obesity include onset in infancy, lack of satiety, poor linear growth, dysmorphic features, and cognitive dysfunction. Selection and interpretation of appropriate laboratory tests and indications for subspecialist referral are also discussed.

  15. Screening for Hormonal, Monogenic, and Syndromic Disorders in Obese Infants and Children

    PubMed Central

    Mason, Kelly; Page, Laura; Balikcioglu, Pinar Gumus

    2015-01-01

    The prevalence of pediatric obesity in the United States is nearly 17%. Most cases are “exogenous”, resulting from excess energy intake relative to energy expenditure over a prolonged period of time. However, some cases of obesity are “endogenous”, associated with hormonal, genetic, or syndromic disorders such as hypothyroidism, Cushing’s syndrome, growth hormone deficiency, defective leptin signaling, mutations in the melanocortin 4 receptor, and Prader-Willi and Bardet-Biedl syndromes. This article reviews the hormonal, monogenic, and syndromic causes of childhood obesity and identifies critical features that distinguish “endogenous” obesity disorders from the more common exogenous obesity. Findings that raise suspicion for endogenous obesity include onset in infancy, lack of satiety, poor linear growth, dysmorphic features, and cognitive dysfunction. Selection and interpretation of appropriate laboratory tests and indications for subspecialist referral are also discussed. PMID:25198446

  16. Hepatitis in children with acquired immune deficiency syndrome. Histopathologic and immunocytologic features.

    PubMed

    Duffy, L F; Daum, F; Kahn, E; Teichberg, S; Pahwa, R; Fagin, J; Kenigsberg, K; Kaplan, M; Fisher, S E; Pahwa, S

    1986-01-01

    Hepatic morphology and immunocytology were evaluated in 4 children with clinical and immunologic characteristics of the acquired immune deficiency syndrome or acquired immune deficiency syndrome related complex. All 4 children had hepatomegaly and increased serum alanine and aspartate aminotransferase activity. Both lobular and portal changes were noted. Lymphocytic infiltration, piecemeal necrosis, hepatocellular and bile duct damage, sinusoidal cell hyperplasia, and endothelialitis were prominent. Vesicular rosettes in sinusoidal lymphocytes and tubuloreticular structures in sinusoidal endothelial cells were demonstrated by electron microscopy. The lymphocytic infiltrate in both the lobular and portal spaces was characterized by a relative increase of cytotoxic/suppressor (T8) cells. Hepatitis may be a common feature of pediatric acquired immune deficiency syndrome and acquired immune deficiency syndrome-related complex. Although the histopathologic changes are consistent with chronic active hepatitis, the specific pathogenesis remains to be determined.

  17. Effects of pituitary hormone deficiency on growth and glucose metabolism of the sheep fetus.

    PubMed

    Fowden, A L; Forhead, A J

    2007-10-01

    Pituitary hormones are essential for normal growth and metabolic responsiveness after birth, but their role before birth remains unclear. This study examined the effects of hypophysectomizing fetal sheep on their growth and glucose metabolism during the late normal and extended periods of gestation, and on their metabolic response to maternal fasting for 48 h near term. Fetal hypophysectomy reduced crown rump length (CRL), limb lengths, and body weight but increased ponderal index relative to controls near normal term. It also lowered the daily rate of crown rump length increment uniformly from 35 d before, to 20 d after normal term. Hypophysectomized (HX) fetuses had normal weight-specific rates of umbilical uptake, utilization, and oxidation of glucose but lower rates of umbilical oxygen uptake than controls near term. All these metabolic rates were significantly less in HX fetuses during the extended period of gestation than in HX and intact fetuses near normal term. In contrast to controls, glucogenesis was negligible in HX fetuses during maternal fasting. Consequently, the rate of glucose utilization decreased significantly in fasted HX but not intact fetuses. Conversely, the rate of CO(2) production from glucose carbon decreased in fasted intact but not HX fetuses. Fetal hypophysectomy also prevented the fasting-induced increases in plasma cortisol and norepinephrine concentrations seen in controls. These findings demonstrate that the pituitary hormones are important in regulating the growth rate and adaptive responses of glucose metabolism to undernutrition in fetal sheep. They also suggest that fetal metabolism is altered when gestational length is extended.

  18. Blood and hair lead in children with different extents of iron deficiency in Karachi.

    PubMed

    Rahman, Muhammad Ataur; Rahman, Bushra; Ahmad, Muhammad Saeed; Blann, Andrew; Ahmed, Nessar

    2012-10-01

    Childhood iron deficiency has a high incidence in Pakistan. Some but not all studies have shown that dietary iron deficiency may cause increased absorption of lead as both compete for the same transporters in the small intestine. Therefore, children in Pakistan, residing in heavily polluted cities like Karachi may be prone to lead poisoning. This hypothesis was tested by investigating blood and hair lead concentrations in children from Karachi who were divided into four groups of iron status; normal, borderline iron deficiency, iron deficiency and iron deficiency anaemia. A prospective observational study was conducted where 269 children were categorized into four groups of iron status using the World Health Organization criteria and one based on soluble transferrin receptor measurements. Blood iron status was determined using a full blood count, serum iron, ferritin, transferrin saturation and soluble transferrin receptor measurements. Blood lead was determined by graphite atomic absorption spectroscopy, whereas hair lead was assessed using an inductively coupled plasma atomic emission spectroscopy technique. Blood lead concentrations were significantly higher in children with iron deficiency anaemia (mean [95% confidence intervals] were 24.9 [22.6-27.2] μg/dL) compared to those with normal iron status (19.1 [16.8-21.4] μg/dL) using WHO criteria. In contrast, hair lead content was not significantly different in children of different iron status. Our findings reinforce the importance of not only reducing environmental lead pollution but also the development of national health strategies to reduce childhood iron deficiency in Pakistan.

  19. Blood and hair lead in children with different extents of iron deficiency in Karachi

    SciTech Connect

    Ataur Rahman, Muhammad; Rahman, Bushra; Saeed Ahmad, Muhammad; Blann, Andrew; Ahmed, Nessar

    2012-10-15

    Childhood iron deficiency has a high incidence in Pakistan. Some but not all studies have shown that dietary iron deficiency may cause increased absorption of lead as both compete for the same transporters in the small intestine. Therefore, children in Pakistan, residing in heavily polluted cities like Karachi may be prone to lead poisoning. This hypothesis was tested by investigating blood and hair lead concentrations in children from Karachi who were divided into four groups of iron status; normal, borderline iron deficiency, iron deficiency and iron deficiency anaemia. A prospective observational study was conducted where 269 children were categorized into four groups of iron status using the World Health Organization criteria and one based on soluble transferrin receptor measurements. Blood iron status was determined using a full blood count, serum iron, ferritin, transferrin saturation and soluble transferrin receptor measurements. Blood lead was determined by graphite atomic absorption spectroscopy, whereas hair lead was assessed using an inductively coupled plasma atomic emission spectroscopy technique. Blood lead concentrations were significantly higher in children with iron deficiency anaemia (mean [95% confidence intervals] were 24.9 [22.6-27.2] {mu}g/dL) compared to those with normal iron status (19.1 [16.8-21.4] {mu}g/dL) using WHO criteria. In contrast, hair lead content was not significantly different in children of different iron status. Our findings reinforce the importance of not only reducing environmental lead pollution but also the development of national health strategies to reduce childhood iron deficiency in Pakistan.

  20. [Iron-deficiency anemia in children. A old problem not yet resolved].

    PubMed

    Ramírez-Mayans, Jaime A; Ortiz-López, Carolina; García-Campos, Margarita; Cervantes-Bustamante, Roberto; Mata-Rivera, Norberto; Zárate-Mondragón, Flora; Mason-Cordero, Thomas

    2003-01-01

    Iron-deficiency anemia is still a health problem worldwide. Iron supplementation of some foods such as milk formulas and cereals apparently has not been the solution due to bioavailability of iron. In Mexico, there is high prevalence of anemia in children to date, mainly those under 2 years of age and predominantly in the Southern part of the country. Probably the main causes are iron-deficiency anemia in pregnant women, recurrent infections, such as gastroenteritis and parasites, and the most important one undoubtedly, deficient iron intake.

  1. Micronutrient Deficiencies and Plasmodium vivax Malaria among Children in the Brazilian Amazon

    PubMed Central

    Benzecry, Silvana Gomes; Alexandre, Márcia Almeida; Vítor-Silva, Sheila; Salinas, Jorge Luis; de Melo, Gisely Cardoso; Marinho, Helyde Albuquerque; Paes, Ângela Tavares; de Siqueira, André Machado; Monteiro, Wuelton Marcelo; Lacerda, Marcus Vinícius Guimarães; Leite, Heitor Pons

    2016-01-01

    Background There is a growing body of evidence linking micronutrient deficiencies and malaria incidence arising mostly from P. falciparum endemic areas. We assessed the impact of micronutrient deficiencies on malaria incidence and vice versa in the Brazilian state of Amazonas. Methodology/Principal Findings We evaluated children <10 years old living in rural communities in the state of Amazonas, Brazil, from May 2010 to May 2011. All children were assessed for sociodemographic, anthropometric and laboratory parameters, including vitamin A, beta-carotene, zinc and iron serum levels at the beginning of the study (May 2010) and one year later (May 2011). Children were followed in between using passive surveillance for detection of symptomatic malaria. Those living in the study area at the completion of the observation period were reassessed for micronutrient levels. Univariate Cox-proportional Hazards models were used to assess whether micronutrient deficiencies had an impact on time to first P. vivax malaria episode. We included 95 children median age 4.8 years (interquartile range [IQR]: 2.3–6.6), mostly males (60.0%) and with high maternal illiteracy (72.6%). Vitamin A deficiencies were found in 36% of children, beta-carotene deficiency in 63%, zinc deficiency in 61% and iron deficiency in 51%. Most children (80%) had at least one intestinal parasite. During follow-up, 16 cases of vivax malaria were diagnosed amongst 13 individuals. Micronutrient deficiencies were not associated with increased malaria incidence: vitamin A deficiency [Hazard ratio (HR): 1.51; P-value: 0.45]; beta-carotene [HR: 0.47; P-value: 0.19]; zinc [HR: 1.41; P-value: 0.57] and iron [HR: 2.31; P-value: 0.16]). Upon reevaluation, children with al least one episode of malaria did not present significant changes in micronutrient levels. Conclusion Micronutrient serum levels were not associated with a higher malaria incidence nor the malaria episode influenced micronutrient levels. Future studies

  2. Differential sensitivity of growth hormone-releasing hormone and somatostatin release from perifused mouse hypothalamic fragments in response to glucose deficiency.

    PubMed

    Sato, M; Frohman, L A

    1993-06-01

    The effects of glucose deficiency on growth hormone (GH)-releasing hormone (GRH) and somatostatin (SRIH) release from mouse hypothalamic fragments were investigated using an in vitro perifusion system. Fragments were perifused with Krebs-Ringer bicarbonate solution (KRB) containing 5.6 mM glucose for 3 h followed by reduced glucose concentrations in KRB for the next 2 h. GRH release was simulated by 0.7-2.8 mM glucose in an inverse concentration-dependent manner. In contrast, SRIH release was not stimulated by glucose at concentrations of 2.8 and 1.4 mM; only at 0.7 mM was there a modest stimulation of SRIH release that was comparable to the effect of 2.8 mM glucose on GRH release. The maximal stimulation of GRH and SRIH release by 0.7 mM glucose was 221 and 150%, respectively, of controls. Glucose concentrations of 11.2 and 22.4 mM inhibited GRH release but did not alter SRIH release. The glucose analog 2-deoxy-D-glucose (2-DG; 5.6-39.2 mM) also stimulated GRH release in a dose-dependent manner, and SRIH release was less sensitive to 2-DG than was GRH. The maximal stimulation of GRH and SRIH release by 39.2 mM 2-DG was 190 and 147%, respectively, of controls. Increases in GRH and SRIH release stimulated by 30 mM KCl 1 h after exposure to low glucose or 2-DG were not significantly different from those after exposure to 5.6 mM glucose. However, the SRIH response to K(+)-induced depolarization was much greater than that of GRH. The glucose intermediate pyruvate (4.9 and 9.8 mM) partially inhibited both GRH and SRIH release induced by 0.7 mM glucose.(ABSTRACT TRUNCATED AT 250 WORDS)

  3. [The effect of deficiency of selected bioelements on hyperactivity in children with certain specified mental disorders].

    PubMed

    Starobrat-Hermelin, B

    1998-01-01

    The aim of my work was the answer to the following questions: how often does the deficiency of magnesium, copper, zinc, calcium, iron occur among hyperactive children in comparison with healthy children, deficiency of which of the considered bioelements is the most frequent, what is the effect of supplementation of deficit element on hyperactivity and does it depend on other certain disorders that coexist with hyperactivity? In a process of establishing the subject diagnosis I have followed the DSM IV criteria recognizing ADHD among examined ones. I have determined the deficiency of magnesium, copper, zinc, calcium, iron in the group of 116 children with diagnosed ADHD. Consequently, as a result, I have found out that shortage of above-mentioned bioelements occurs more often among hyperactive children than among those being healthy, and deficiency of magnesium is the most frequent in this respect. Further, I have divided the group of 110 children with magnesium deficiency into two groups according to the other mental disorders that coexist with ADHD: 1) the group where hyperactivity coexists with disorders typical for developmental age such as enuresis, tics, separation anxiety, stuttering, selective mutism (63 children); 2) the group where hyperactivity coexists with disruptive behaviour disorders: conduct disorder and oppositional defiant disorder (47 children). The content of magnesium, copper, zinc, calcium, iron has been determined respectively in blood (serum and red cells) and in hair by atomic absorption spectroscopy method in both groups accordingly. At the same time, the hyperactivity tests were carried out using Conner's Rating Scales for Parents and Teachers, Wender's Scale as well as Quotient of Development to Freedom from Distractibility. During the statistical analysis the inparametric tests have been used taking as a significance level p < 0.05. On the ground of obtained findings I have not stated any significant differences in bioelements content

  4. Vitamin-D deficiency predicts infections in young north Indian children: A secondary data analysis

    PubMed Central

    Taneja, Sunita; Bhandari, Nita; Sinha, Bireshwar; Upadhyay, Ravi Prakash; Bhan, Maharaj Kishan

    2017-01-01

    Background Recent studies have demonstrated a relationship between poor vitamin D status and respiratory infections and diarrhea among young children. Acute lower respiratory infections (ALRI) and diarrhea are among the two most important causes of death in under-5 children. In this paper, we examined the extent to which vitamin-D deficiency (<10 ng/ml) predicts ALRI, clinical pneumonia and diarrhea among 6 to 30 months old children. Methods We used data from a randomized controlled trial (RCT) of daily folic acid and/or vitamin B12 supplementation for six months in 6 to 30 months old children conducted in Delhi, India. Generalized estimating equations (GEE) were used to examine the associations between vitamin-D deficiency and episodes of ALRI, clinical pneumonia and diarrhea. Results Of the 960 subjects who had vitamin-D concentrations measured, 331(34.5%) were vitamin-D deficient. We found, after controlling for relevant potential confounders (age, sex, breastfeeding status, wasting, stunting, underweight, anemia status and season), that the risk of ALRI was significantly higher among vitamin-D deficient (OR 1.26; 95% CI: 1.03 to 1.55) compared to vitamin-D-replete children in the six months follow-up period. Vitamin-D status was not associated with episodes of diarrhea or clinical pneumonia. Conclusion Vitamin-D deficiency is common in young children in New Delhi and is associated with a higher risk of ALRI. The role of vitamin D in Indian children needs to be elucidated in further studies. PMID:28273084

  5. Glucose-6-phosphate dehydrogenase deficiency among children attending the Emergency Paediatric Unit of Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria.

    PubMed

    Isaac, Iz; Mainasara, As; Erhabor, Osaro; Omojuyigbe, St; Dallatu, Mk; Bilbis, Ls; Adias, Tc

    2013-01-01

    Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most common human enzyme deficiencies in the world. It is particularly common in populations living in malaria-endemic areas, affecting more than 400 million people worldwide. This present study was conducted with the aim of determining the prevalence of G6PD deficiency among children visiting the Emergency Paediatric Unit of Usmanu Danfodiyo University Teaching Hospital for pediatric-related care. The study included 118 children, made up of 77 (65.3%) males and 41 (34.7%) females aged ≤5 years with mean age of 3.26 ± 1.90 years. Randox G6PD quantitative in vitro test screening was used for the diagnosis of G6PD deficiency. Of the 118 children tested, 17 (14.4%) were G6PD-deficient. Prevalence of G6PD deficiency was concentrated predominantly among male children (22.1%). Male sex was significantly correlated with G6PD deficiency among the children studied (r = 7.85, P = 0.01). The highest prevalence occurred among children in the 2- to 5-year age-group. Of the 17 G6PD-deficient children, twelve (70.2%) were moderately deficient, while five (29.4%) were severely deficient. Blood film from G6PD-deficient children indicated the following morphological changes; Heinz bodies, schistocytes, target cells, nucleated red cells, spherocytes, and polychromasia. This present study has shown a high prevalence of G6PD deficiency among children residing in Sokoto in the northwestern geopolitical zone of Nigeria. The study indicated a male sex bias in the prevalence of G6PD deficiency among the children studied. There is a need for the routine screening of children for G6PD deficiency in our environment, to allow for evidence-based management of these children and to ensure the avoidance of food, drugs, and infective agents that can potentially predispose these children to oxidative stress as well as diseases that deplete micronutrients that protect against oxidative stress. There is need to build capacity in our

  6. [Study on behavior of thyroid gland hormones in Bydgoszcz province children and teenagers with simplex struma].

    PubMed

    Wawrzeńczyk, M; Balcar-Boroń, A; Kretowicz, W; Kurylak, A

    1998-01-01

    Struma caused by iodine deficit is an endemic disorder in specified regions. The purpose of the study was a biochemical characteristic of struma simplex in children from Bydgoszcz province and evalued correlation between laboratory exponents of thyroid gland function, size of the gland and kind of the struma. It was confirmed that neutral struma of children generally takes a course with euthyroidism. In children with struma were confirmed significant lower values of T4 and no significant higher values of T3. Value of TSH was no different in comparison with the control group. Proved was that hormonal thyroid activity is lower when struma is getting bigger.

  7. Serum galactose-deficient IgA1 levels in children with IgA nephropathy.

    PubMed

    Jiang, Mengjie; Jiang, Xiaoyun; Rong, Liping; Xu, Yuanyuan; Chen, Lizhi; Qiu, Zeting; Mo, Ying

    2015-01-01

    Immunoglobulin A nephropathy (IgAN) is an immunopathologic diagnosis based on a renal biopsy, it is characterized by deposits of IgA-containing immune complexes in the mesangium. Adults with IgAN have a galactose-deficient IgA1 in the circulation and glomerular deposition. There are few studies on the glycosylation of serum IgA1 in children with IgAN. To measure the serum levels of galactose-deficient IgA1 in pediatric patients with IgAN, 72 biopsy-proven IgAN children were divided into 3 groups based on the clinical features: isolated hematuria group (24 patients), hematuria and proteinuria group (22 patients), and nephritic syndrome group (26 patients). They were also divided into 3 groups according to pathologic grading: grade I + II group (25 patients), grade III group (33 patients) and grade IV + V group (14 patients). 30 healthy children were recruited as a control group. We used vicia villosa lectin binding enzyme-linked immunosorbent assay to measure the serum levels of galactose-deficient IgA1 in all groups and controls. Serum levels of galactose-deficient IgA1 in children with IgAN were higher than controls (P < 0.01). There were no significant differences in serum levels of galactose-deficient IgA1 among the different clinical and pathologic grading groups. The values of the area under the curve for galactose-deficient IgA1 levels were 0.976 (95% CI, 0.953-1.000). The cutoff point for galactose-deficient IgA1 levels was 0.125, with a sensitivity of 87.5% and a specificity of 83.3%, with a positive predictive value of 92.6% and a negative predictive value of 73.5% (P < 0.01). Children with IgAN presented serum galactose-deficient IgA1, which has shown no relationship with the clinical manifestations and pathologic grading of the disease. Detection of serum galactose-deficient IgA1 levels by vicia villosa lectin binding enzyme-linked immunosorbent assay has a certain clinical value in diagnosis of children with IgAN.

  8. Efficacy and safety of growth hormone replacement therapy in Japanese adults with growth hormone deficiency: a post-marketing observational study.

    PubMed

    Shimatsu, Akira; Tai, Shigeru; Imori, Makoto; Ihara, Katsuichiro; Taketsuna, Masanori; Funai, Jumpei; Tanaka, Toshiaki; Teramoto, Akira; Irie, Minoru; Chihara, Kazuo

    2013-01-01

    This large-scale observational study examined the long-term effectiveness and safety of growth hormone (GH) replacement therapy for adult GH deficiency (GHD) in Japanese clinical practice using the Hypopituitary Control and Complications Study database. The study included 402 GHD patients for safety analyses and a subset of 209 patients (149 adult-onset and 60 childhood-onset GHD patients) who had not previously received GH replacement therapy for the efficacy analyses. Data on clinical, metabolic, quality of life (QoL) characteristics, and all adverse events (AEs) were collected at baseline (start of GH treatment), 6 months, 1 year and 2 years. Over the observation period, there were improvements from baseline in insulin-like growth factor-I standard deviation scores (P<0.001), although the changes in metabolic parameters were minimal. QoL (Short Form-36) Z-scores significantly increased from baseline in both onset-type groups for several subscale domains (P<0.05). A total of 145 (36.1%) patients experienced ≥1 AE. Common AEs were hyperlipidaemia (2.7%) and hyperinsulinaemia (2.2%). Some patients experienced recurrent hypothalamic/pituitary tumour (events per 1000 patient-years: 2.78), new benign (0.93), malignant tumour (10.28) or other new tumour (0.93), new diabetes mellitus (7.45), and new stroke (3.71). Seven patients died during the observation period. Our safety findings are inconclusive about the associations between GH replacement and AEs, although the incidence of diabetes mellitus and cardiovascular events are similar to those reported in the Japanese general population. In conclusion, the key beneficial effects of GH replacement therapy for GHD are observed in routine clinical practice in Japan.

  9. Effect of growth hormone treatment on diastolic function in patients who have developed growth hormone deficiency after definitive treatment of acromegaly

    PubMed Central

    Fazeli, Pouneh K.; Teoh, Jonathan G.; Lam, Eleanor L.; Gerweck, Anu V.; Wexler, Tamara L.; Teo, Eliza P.; Russell, Brian M.; Durst, Ronen; McCarty, David; Weiner, Rory B.; Picard, Michael H.; Klibanski, Anne; Miller, Karen K.

    2015-01-01

    Objective Although growth hormone (GH) replacement is prescribed for patients with hypopituitarism due to many etiologies, it is not routinely prescribed for patients with GH deficiency (GHD) after cure of acromegaly (acroGHD). This study was designed to investigate the effect of GH replacement on cardiac parameters in acroGHD. Design We prospectively evaluated for 12 months 23 patients with acroGHD: 15 subjects on GH replacement and eight subjects not on GH replacement. Main outcome measures included LV mass corrected for body surface area (LVM/BSA) and measures of diastolic dysfunction (E/A ratio and deceleration time), as assessed by echocardiography. Results After 12 months of follow-up, there were no differences between the GH-treated group and the untreated group in LVM/BSA (GH: 74.4 ± 22.5g/m2 vs untreated: 72.9 ± 21.3g/m2, p=0.89), E/A ratio (GH: 1.21 ± 0.39 vs untreated: 1.08 ± 0.39, p=0.50) or deceleration time (GH: 224.5 ± 60.1ms vs untreated: 260 ± 79.8ms, p=0.32). The overall degree of diastolic function was similar between the groups with 42.9% of untreated subjects and 50% of GH-treated subjects (p=0.76) classified as having normal diastolic function at follow-up. Conclusions There were no significant differences in LVM/BSA or parameters of diastolic function in patients with a history of acromegaly treated for GHD as compared to those who were untreated. These data are reassuring with respect to cardiovascular safety with GH use after treatment for acromegaly, although further longer term study is necessary to evaluate the safety and efficacy of GH treatment in this population. PMID:26774401

  10. Plasma thyrotropin-releasing hormone concentrations in the rat. Effect of thyroid excess and deficiency and cold exposure.

    PubMed Central

    Emerson, C H; Utiger, R D

    1975-01-01

    To investigate the physiology of thyrotropin-releasing hormone (TRH) secretion from hypothalamus and brain, a method for measurement of peripheral plasma TRH concentrations in rats was developed. Blood was collected in heparin and dimercaptopropanol containing [3H]TRH to determine recovery. The plasma was extracted with methanol and the redissolved dried methanol extracts applied to anti-TRH Sepharose columns. These columns bound greater than 80% of 125I-TRH applied and had a capacity in excess of 20 ng TRH. TRH was eluted from the anti-TRH Sepharose with acetic acid and quantitated by radioimmunoassay of the lyophilized acetic acid eluate. Mean recovery of unlabeled TRH was 44.7+/-6.1% (SD) and mean recovery of [3H]TRH was 44.0+/-4.0%. Mean plasma TRH concentrations, corrected for recovery, in plasma pools from eight groups of normal male rats (four to seven pools/experiment, five to seven rats/pool) ranged from 7 to 30 pg/ml (mean, 16). In experiments in which rats were given 5, 10, 15, 0r 50 mug thyroxine daily for 1 wk or in thyroidectomized rats, mean plasma TRH concentrations did not differ significantly from those of control animals sacrificed at the same time. In each experiment, four to seven plasma pools, each from five to seven rats, were processed from both control and experimental groups. No changes in plasma TRH concentrations were found in rats exposed to cold (4degreeC) for 30, 60, and 90-180 min. Signigicant increases in plasma thyrotropin (TSH) concentrations were found in all cold-exposed animals. These results provide no evidence that thyroid hormone excess of deficiency affects TRH secretion. If TRH secretion is responsible for cold-induced increases in plasma TSH concentrations, the increase in TRH secretion is of insufficient magnitude to alter periperal plasma TRH concentrations. PMID:811690

  11. Thyroid hormones changes in infants and children with metabolic acidosis.

    PubMed

    Tahirović, H F

    1991-10-01

    The influence of the acidotic state on the thyroxine (T4) peripheral metabolism was studied in two different forms of metabolic acidosis, ie infantile diarrhea and diabetic ketoacidosis. The serum concentrations of T4, free T4 (FT4), triiodothyronine (T3), reverse T3 (rT3), thyrotropin (TSH) and thyroxine-binding globulin (TBG) were measured and compared to healthy control groups. Lower T4 and T3 and higher rT3 serum concentrations were found in both tested groups of patients in relation to the control groups. In infants with severe metabolic acidosis FT4 values were lower than those observed in the control group. In addition, serum TBG levels were lower in diabetic patients as compared to control subjects. Despite the reduced serum T3 and T4 concentrations in both groups of patients, TSH concentrations, were within the normal range. Therefore, we concluded that acidosis caused either by diarrhea (not so far described) or by diabetes mellitus (well documented up to now) affects the thyroid hormones metabolism in a similar way, at least as far as the thyroid hormones blood levels are concerned.

  12. Deficient auditory processing in children with Asperger Syndrome, as indexed by event-related potentials.

    PubMed

    Jansson-Verkasalo, Eira; Ceponiene, Rita; Kielinen, Marko; Suominen, Kalervo; Jäntti, Ville; Linna, Sirkka Liisa; Moilanen, Irma; Näätänen, Risto

    2003-03-06

    Asperger Syndrome (AS) is characterized by normal language development but deficient understanding and use of the intonation and prosody of speech. While individuals with AS report difficulties in auditory perception, there are no studies addressing auditory processing at the sensory level. In this study, event-related potentials (ERP) were recorded for syllables and tones in children with AS and in their control counterparts. Children with AS displayed abnormalities in transient sound-feature encoding, as indexed by the obligatory ERPs, and in sound discrimination, as indexed by the mismatch negativity. These deficits were more severe for the tone stimuli than for the syllables. These results indicate that auditory sensory processing is deficient in children with AS, and that these deficits might be implicated in the perceptual problems encountered by children with AS.

  13. Exposure to polychlorinated biphenyls and levels of thyroid hormones in children.

    PubMed Central

    Osius, N; Karmaus, W; Kruse, H; Witten, J

    1999-01-01

    As part of an epidemiologic study on exposure to a toxic waste incineration plant we investigated whether blood concentrations of polychlorinated biphenyls (PCBs), lead, and cadmium, as well as concentration of mercury in 24-hr urine samples were associated with thyroid hormone status. As an indication of status, we determined levels of thyroid-stimulating hormone (TSH), free thyroxine (FT(4)), and free triiodothyronine (FT(3)) in children living in households where [less than/equal to] 10 cigarettes were smoked per day. Eight PCB congeners (PCBs 101, 118, 138, 153, 170, 180, 183, and 187) were measured in whole blood samples. Of these, seven congeners (PCB 101 was not detected in any sample) and the sum of all PCB congeners were analyzed as predictors for thyroid hormone status in separate linear regression models adjusted for potential confounders. In addition, the possible effects of cadmium, lead, and mercury on levels of thyroid hormones were examined. Blood concentrations and information on questionnaire data were available for 320 children 7-10 years of age. We found a statistically significant positive association between the mono-ortho congener PCB 118 and TSH as well as statistically significant negative relationships of PCBs 138, 153, 180, 183, and 187 to FT(3). There was no association for the PCB congeners and FT(4). Blood cadmium concentration was associated with increasing TSH and diminishing FT(4). Blood lead and urine concentration of mercury were of no importance to thyroid hormone levels. The results stress the need for future studies on the possible influences of PCB and cadmium exposure on thyroid hormones, particularly in children. These studies should also take neurologic development into account. PMID:10504153

  14. Testosterone Replacement Therapy Prevents Alterations of Coronary Vascular Reactivity Caused by Hormone Deficiency Induced by Castration.

    PubMed

    Rouver, Wender Nascimento; Delgado, Nathalie Tristão Banhos; Menezes, Jussara Bezerra; Santos, Roger Lyrio; Moyses, Margareth Ribeiro

    2015-01-01

    The present study aimed to determine the effects of chronic treatment with different doses of testosterone on endothelium-dependent coronary vascular reactivity in male rats. Adult male rats were divided into four experimental groups: control (SHAM), castrated (CAST), castrated and immediately treated subcutaneously with a physiological dose (0.5 mg/kg/day, PHYSIO group) or supraphysiological dose (2.5 mg/kg/day, SUPRA group) of testosterone for 15 days. Systolic blood pressure (SBP) was assessed at the end of treatment through tail plethysmography. After euthanasia, the heart was removed and coronary vascular reactivity was assessed using the Langendorff retrograde perfusion technique. A dose-response curve for bradykinin (BK) was constructed, followed by inhibition with 100 μM L-NAME, 2.8 μM indomethacin (INDO), L-NAME + INDO, or L-NAME + INDO + 0.75 μM clotrimazole (CLOT). We observed significant endothelium-dependent, BK-induced coronary vasodilation, which was abolished in the castrated group and restored in the PHYSIO and SUPRA groups. Furthermore, castration modulated the lipid and hormonal profiles and decreased body weight, and testosterone therapy restored all of these parameters. Our results revealed an increase in SBP in the SUPRA group. In addition, our data led us to conclude that physiological concentrations of testosterone may play a beneficial role in the cardiovascular system by maintaining an environment that is favourable for the activity of an endothelium-dependent vasodilator without increasing SBP.

  15. Hormone-sensitive lipase deficiency alters gene expression and cholesterol content of mouse testis

    PubMed Central

    Wang, Feng; Chen, Zheng; Ren, Xiaofang; Tian, Ye; Wang, Fucheng; Liu, Chao; Jin, Pengcheng; Li, Zongyue; Zhang, Feixiong

    2016-01-01

    Hormone-sensitive lipase-knockout (HSL−/−) mice exhibit azoospermia for unclear reasons. To explore the basis of sterility, we performed the following three experiments. First, HSL protein distribution in the testis was determined. Next, transcriptome analyses were performed on the testes of three experimental groups. Finally, the fatty acid and cholesterol levels in the testes with three different genotypes studied were determined. We found that the HSL protein was present from spermatocyte cells to mature sperm acrosomes in wild-type (HSL+/+) testes. Spermiogenesis ceased at the elongation phase of HSL−/− testes. Transcriptome analysis indicated that genes involved in lipid metabolism, cell membrane, reproduction and inflammation-related processes were disordered in HSL−/− testes. The cholesterol content was significantly higher in HSL−/− than that in HSL+/+ testis. Therefore, gene expression and cholesterol ester content differed in HSL−/− testes compared to other testes, which may explain the sterility of male HSL−/− mice. PMID:27920259

  16. Fibroblast Growth Factor Receptor 3 Deficiency Does Not Impair the Osteoanabolic Action of Parathyroid Hormone on Mice

    PubMed Central

    Xie, Yangli; Yi, Lingxian; Weng, Tujun; Huang, Junlan; Luo, Fengtao; Jiang, Wanling; Xian, Cory J; Du, Xiaolan; Chen, Lin

    2016-01-01

    Summary: PTH stimulates bone formation in Fgfr3 knockout mice through promotion of proliferation and differentiation in osteoblasts. Introduction: Previous studies showed that endogenous fibroblast growth factor 2 (FGF-2) is required for parathyroid hormone (PTH)-stimulated bone anabolic effects, however, the exact mechanisms by which PTH stimulate bone formation and the function of FGF receptors in mediating these actions are not fully defined. FGF receptor 3 (FGFR3) has been characterized as an important regulator of bone metabolism and is confirmed to cross-talk with PTH/PTHrP signal in cartilage and bone development. Methods: Fgfr3 knockout and wild-type mice at 2-month-old and 4-month-old were intraperitoneally injected with PTH intermittently for 4 weeks and then the skeletal responses to PTH were assessed by dual energy X-ray absorptiometry (DEXA), micro-computed tomography (μCT) and bone histomorphometry. Results: Intermittent PTH treatment improved bone mineral density (BMD) and femoral mechanical properties in both Fgfr3-/- and wild-type mice. Histomorphometric analysis showed that bone formation and bone resorption were increased in both genotypes following PTH treatment. PTH treatment increased trabecular bone volume (BV/TV) in WT and Fgfr3-deficient mice. The anabolic response in Fgfr3-deficient and wild-type bone is characterized by an increase of both bone formation and resorption-related genes following PTH treatment. In addition, we found that Fgfr3 null osteoblasts (compared to wild-type controls) maintained normal abilities to response to PTH-stimulated increase of proliferation, differentiation, expression of osteoblastic marker genes (Cbfa1, Osteopontin and Osteocalcin), and phosphorylation of Erk1/2. Conclusions: Bone anabolic effects of PTH were not impaired by the absence of FGFR3, suggesting that the FGFR3 signaling may not be required for osteoanabolic effects of PTH activities. PMID:27489502

  17. Impact of Oatp1c1 deficiency on thyroid hormone metabolism and action in the mouse brain.

    PubMed

    Mayerl, Steffen; Visser, Theo J; Darras, Veerle M; Horn, Sigrun; Heuer, Heike

    2012-03-01

    Organic anion-transporting polypeptide 1c1 (Oatp1c1) (also known as Slco1c1 and Oatp14) belongs to the family of Oatp and has been shown to facilitate the transport of T(4). In the rodent brain, Oatp1c1 is highly enriched in capillary endothelial cells and choroid plexus structures where it may mediate the entry of T(4) into the central nervous system. Here, we describe the generation and first analysis of Oatp1c1-deficient mice. Oatp1c1 knockout (KO) mice were born with the expected frequency, were not growth retarded, and developed without any overt neurological abnormalities. Serum T(3) and T(4) concentrations as well as renal and hepatic deiodinase type 1 expression levels were indistinguishable between Oatp1c1 KO mice and control animals. Hypothalamic TRH and pituitary TSH mRNA levels were not affected, but brain T(4) and T(3) content was decreased in Oatp1c1-deficient animals. Moreover, increased type 2 and decreased type 3 deiodinase activities indicate a mild hypothyroid situation in the brain of Oatp1c1 KO mice. Consequently, mRNA expression levels of gene products positively regulated by T(3) in the brain were down-regulated. This central nervous system-specific hypothyroidism is presumably caused by an impaired passage of T(4) across the blood-brain barrier and indicates a unique function of Oatp1c1 in facilitating T(4) transport despite the presence of other thyroid hormone transporters such as Mct8.

  18. Prevalence of anemia, iron deficiency, thalassemia and glucose-6-phosphate dehydrogenase deficiency among hill-tribe school children in Omkoi District, Chiang Mai Province, Thailand.

    PubMed

    Yanola, Jintana; Kongpan, Chatpat; Pornprasert, Sakorn

    2014-07-01

    The prevalaence of anemia, iron deficiency, thalassemia and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency were examined among 265 hill-tribe school children, 8-14 years of age, from Omkoi District, Chiang Mai Province, Thailand. Anemia was observed in 20 school children, of whom 3 had iron deficiency anemia. The prevalence of G-6-PD deficiency and β-thalassemia trait [codon 17 (A>T), IVSI-nt1 (G>T) and codons 71/72 (+A) mutations] was 4% and 8%, respectively. There was one Hb E trait, and no α-thalassemia-1 SEA or Thai type deletion. Furthermore, anemia was found to be associated with β-thalassemia trait in 11 children. These data can be useful for providing appropriate prevention and control of anemia in this region of Thailand.

  19. Prevalence of Iron deficiency anemia in children with liver cirrhosis: A cross-sectional study

    PubMed Central

    Zareifar, Soheila; Dehghani, Seyed Mohsen; Rahanjam, Najmeh; Farahmand Far, Mohammad Reza

    2015-01-01

    Background: Among the many complications reported for cirrhosis, iron deficiency anemia (IDA) has attracted much attention. This type of anemia, in contrast to other types of anemia, is easy to treat prophylactically, but if left untreated can lead to a poor quality of life. The aim of this study was to estimate the hemoglobin and serum iron levels among patients with liver cirrhosis for the early diagnosis of IDA and to avoid unnecessary testing and iron supplementation. Subjects and Methods: In this cross-sectional study, 88 children diagnosed with cirrhosis were included, and the values of hemoglobin, serum iron levels and relationship between serum iron (SI), total iron-binding capacity (TIBC), prothrombine time (PT), international normalization ratio (INR), total and direct bilirubin and hepatic enzymes were estimated using paired t test, Mann-Whitney, Chi-square and Kruskal-Wallis tests. Results: Forty-six (52.3%) of 88 children were girls and 42 (47.7%) were boys. Forty-eight (54.5%) patients had anemia and 8 (9%) had iron deficiency anemia (5 boys, 5.6%, and 3 girls, 3.4%). No relationships were observed between iron deficiency anemia and the patient’s age or gender, whereas there was a relationship between iron deficiency and severity and duration of the disease, although the correlation was not statistically significant. Conclusion: The high frequency of iron deficiency anemia in children with cirrhosis (9%) suggests that timely screening should be used for early diagnosis and treatment. PMID:26261697

  20. Malnutrition and micronutrient deficiencies among Bhutanese refugee children--Nepal, 2007.

    PubMed

    2008-04-11

    Acute and chronic malnutrition and micronutrient deficiencies have been found in refugee camp populations. In southeastern Nepal, despite consistent access by refugees to general rations, certain micronutrient deficiencies have posed a substantial health burden to the approximately 100,000 Bhutanese residing in seven refugee camps. Limited food diversity, frequent illness, and poor feeding practices have been cited as underlying causes of poor nutritional status in this population. Annual surveys to assess levels of acute malnutrition (i.e., wasting) and chronic malnutrition (i.e., stunting) have been conducted in these camps by the Association of Medical Doctors of Asia (AMDA) and United Nations High Commissioner for Refugees (UNHCR); however, the capacity to reliably evaluate micronutrient deficiencies has not existed locally in the camps. In January 2007, AMDA and CDC, at the request of UNHCR and the World Food Programme (WFP), conducted a nutritional survey of children aged 6-59 months, assessing 1) the prevalence of acute malnutrition, chronic malnutrition, underweight, anemia, and angular stomatitis (i.e., riboflavin deficiency); 2) the cumulative incidence of diarrhea and acute respiratory illness (ARI); and 3) the feeding practices of the children's mothers. This report describes the results of that survey, which indicated that, although acute malnutrition was found in only 4.2% of the children, chronic malnutrition was found in 26.9% and anemia in 43.3%. These findings underscore the importance of monitoring both malnutrition and micronutrient deficiencies and addressing the underlying causes of nutritional deficits.

  1. Prevalence of vitamin A deficiency in children aged 6-9 years in Wukro, northern Ethiopia.

    PubMed Central

    Kassaye, T.; Receveur, O.; Johns, T.; Becklake, M. R.

    2001-01-01

    OBJECTIVE: To determine the prevalence of vitamin A deficiency in children aged 6-9 years in northern Ethiopia. METHODS: A cross-sectional study was carried out and the data were analysed for 824 (61.5%) of 1339 eligible children for whom there was complete information on biochemical vitamin A status, dietary vitamin A intake, ocular examination for xerophthalmia, and anthropometry. FINDINGS: The prevalence of xerophthalmia was 5.8%; serum retinol levels were below 0.35 mumol/l and between 0.35 and 0.70 mumol/l in 8.4% and 51.1% of the children respectively. The liver vitamin A reserve (modified relative dose response ratio > or = 0.06) was low in 41.0% of the children. CONCLUSION: The high prevalence of severe vitamin A deficiency in children aged 6-9 years indicates the need to reevaluate the practice of targeting vitamin A supplementation programmes on children under 6 years of age in areas where vitamin A deficiency is endemic. PMID:11417037

  2. Intravenous Iron Sucrose for Children with Iron Deficiency Failing to Respond to Oral Iron Therapy

    PubMed Central

    Crary, Shelley E.; Hall, Katherine; Buchanan, George R.

    2010-01-01

    Background For decades parenteral iron has been used in patients with iron deficiency unresponsive to oral iron therapy and in hemodialysis-dependent patients receiving erythropoietin. Newer intravenous (IV) iron formulations such as iron sucrose have replaced high molecular weight iron dextran in dialysis patients; however, the use of parenteral iron in children without renal disease has not been well defined. Procedure Pharmacy records were reviewed on children (≤ 18 yrs of age) who received IV iron sucrose at Children's Medical Center Dallas between January 1, 2004 and June 30, 2009. Patients who received iron sucrose for chronic renal disease were excluded from analysis. Results Thirty-eight children received iron sucrose for non-renal indications, 13 with iron deficiency refractory to oral iron therapy, 13 with iron malabsorption or dependence on parenteral nutrition, 7 for chronic gastrointestinal blood loss, and 5 for miscellaneous indications. Among these 38 children, who received a total of 510 doses of IV iron sucrose, there were only 6 adverse reactions. Patients in all categories had a good response to the iron sucrose, with a median hemoglobin rise of 1.9 – 3.1 g/dl depending on the indication. Conclusions Parenteral iron is a safe and effective means to treat iron deficiency in children who cannot receive or do not respond to oral iron due to intolerance, poor adherence or iron malabsorption. PMID:21298748

  3. Specific antibody deficiency in children with recurrent respiratory infections: a controlled study with follow-up

    PubMed Central

    Ruuskanen, O; Nurkka, A; Helminen, M; Viljanen, M K; Käyhty, H; Kainulainen, L

    2013-01-01

    Specific antibody deficiency (SAD) to unconjugated pneumococcal vaccine (PPV) is an established primary B cell immunodeficiency. The occurrence and natural history of SAD in children is unclear. We conducted an observational study to identify SAD in children with recurrent respiratory infections. Ninety-nine children, mean age 5·9 (range 2–16) years, with recurrent or severe infections were vaccinated with PPV; serum antibody concentrations for serotypes 4, 6B, 9V, 14, 18C, 19F and 23F were measured before and 2 weeks after vaccination with enzyme immunoassay. The retrospective control group consisted of 89 healthy children matched for age and gender. No children had received previous conjugated pneumococcal vaccine (PCV) or PPV. The structured history of infectious diseases of all participants was collected. Ten of 91 (11%) children (eight excluded due to immunoglobulin G subclass deficiency) with recurrent respiratory infections had SAD. In the control group, three children (3%) responded inadequately to PPV (P = 0·05). Most children with SAD also had many other minor immune defects. After 0·5–5 years (medium 3·8), eight children with SAD were revaccinated with PPV; five responded adequately and three inadequately. Two SAD children were revaccinated with PCV, one developed an adequate and one an inadequate response. Two children with SAD received treatment with intravenous immunoglobulin; the remaining eight children recovered without replacement therapy during the follow-up. SAD is common in young children with recurrent respiratory infections, but it is often transient and resolves itself within a few years without specific treatment. PMID:23574320

  4. Evaluation of dental and bone age in iron-deficient anemic children of South India

    PubMed Central

    Kumar, Vinod; Haridas, Harish; Hunsigi, Prahlad; Farooq, Umar; Erugula, Sridhar R.; Ealla, Kranti K. R.

    2016-01-01

    Aims and Objectives: Dental and bone age is very essential for the dental practitioner in planning treatments and is an extra source of information for the pediatrician, orthopedician, and endocrinologist. There are few published data regarding collation between dental age, bone age, and chronological age in iron-deficiency anemic children. This study has been undertaken to evaluate and compare dental age, bone age, and chronological age in children with iron-deficiency anemia. Materials and Methods: One hundred iron-deficiency anemic children were selected in the age group of 8–14 years. Chronological age of the child was recorded by asking birth date from parents or checking school records. Dental age was calculated by Demirjians method and bone age was evaluated using Bjork, Grave, and Brown's method. Unpaired student's t-test and Pearson's correlation coefficient were the two statistical tests applied to compare dental, bone, and chronological age. Results: Dental and bone age was significantly lower (P < 0.001) compared to chronological age. The correlation between the three ages was positive in both sexes. Conclusion: Dental and bone age retardation was a significant feature in our sample of 100 iron-deficient anemic children. Bone age and dental age are valuable parameters in assessing the overall growth of the child. Further studies are required to corroborate our findings. PMID:27891309

  5. The Modern Approach to the Preschool Upbringing of Children with Developmental Deficiencies.

    ERIC Educational Resources Information Center

    Strebeleva, E. A.

    1997-01-01

    Presents the findings from a Russian survey that investigated directions for corrective-upbringing work with preschool age children who have deficiencies in mental development. Explains that the degree and amount of corrective work depends on the severity of the impairment. Focuses on different components of corrective-upbringing, such as the…

  6. Suitability of School Textbooks for 5 to 7 Year Old Children with Colour Vision Deficiencies

    ERIC Educational Resources Information Center

    Torrents, Aurora; Bofill, Francesc; Cardona, Genis

    2011-01-01

    Purpose: The aim of the present study was to determine, through colorimetric analysis, whether school textbooks for children aged 5 to 7 years contained tasks requiring normal colour vision discrimination for their resolution. In addition, the performance of a group of observers with diverse colour vision deficiencies was evaluated while…

  7. Impact of iron deficiency anemia on the function of the immune system in children

    PubMed Central

    Hassan, Tamer Hasan; Badr, Mohamed Ahmed; Karam, Nehad Ahmed; Zkaria, Marwa; El Saadany, Hosam Fathy; Abdel Rahman, Doaa Mohamed; Shahbah, Doaa Abdallah; Al Morshedy, Salah Mohamed; Fathy, Manar; Esh, Asmaa Mohamed Hosni; Selim, Amal Mohamed

    2016-01-01

    Abstract The importance of iron deficiency as a public health problem is based ultimately on the seriousness of its consequences on health. The most extensively investigated consequences of iron deficiency involve work performance and immune function. The significance of the effects on work performance is generally accepted. In contrast, data on the influence of iron deficiency on immune function are often perceived as being confusing and contradictory. We aimed to evaluate the effect of iron deficiency anemia on humoral, cellular, nonspecific immunity, and also the effect on the cytokines that are the key factors of many immunologic steps. Forty children with iron deficiency anemia and 20 age and sex-matched healthy children were included. All children were subjected to full medical history, thorough clinical examination, complete blood count, iron indices (serum iron, serum total iron-binding capacity, serum ferritin, and transferrin saturation), immunoglobulin assay (IgA, IgG, and IgM), interleukin (IL)-6 serum level, study of T-lymphocyte subsets, and evaluation of phagocytic function of macrophages and oxidative burst activity of neutrophils. Patients had significantly lower IgG levels, IL-6, phagocytic activity, and oxidative burst of neutrophils than controls, although there was no significant difference between patients and controls with regard to other immunoglobulins and CD4/CD8 ratio. There was significantly positive correlation between serum iron and IL-6 serum level. We concluded that humoral, nonspecific immunity (phagocytic activity and oxidative burst), and the IL-6 are influenced in patients with iron deficiency anemia. Study of these abnormalities after correction of iron deficiency is strongly needed. PMID:27893677

  8. Effectiveness of zinc protoporphyrin/heme ratio for screening iron deficiency in preschool-aged children.

    PubMed

    Yu, Kyeong Hee

    2011-02-01

    Hemoglobin and zinc protoporphyrin (ZPP) tests are commonly used to screen for iron deficiency, but little research has been done to systematically evaluate the sensitivity and specificity of these two tests. The goal of this study was to evaluate the effectiveness of zinc protoporphyrin/heme (ZPP/H) ratio as a point-of-service screening test for iron deficiency among preschool-aged children by comparing the sensitivity and specificity of hemoglobin, ZPP/H ratio, and serum ferritin (SF). Also completed were assessments for the prevalence of anemia, iron deficiency (ID), and iron deficiency anemia (IDA) with indicators of ferritin models. This study was carried out with 95 children ages 3 to 6 y. Anthropometric measurements were assessed, and blood samples were analyzed for hemoglobin, SF, transferrin saturation (TS), and ZPP. Anemia was common and the prevalences of anemia, ID, and IDA were 14.7%, 12.6%, and 5.2%, respectively. The ZPP/H ratio was strongly and significantly correlated with hemoglobin. And ZPP/H ratio was a more sensitive test for ID than hemoglobin or SF measurement, correctly identifying more than twice as many iron-deficient children (sensitivity of 91.7%, compared to 41.7% for hemoglobin and SF). However, ZPP/H ratio had lower specificity (60.2%, compared to 89.1% for hemoglobin or 96.4% for SF) and resulted in the false identification of more subjects who actually were not iron deficient than did hemoglobin or SF. Low hemoglobin concentration is a late-stage indicator of ID, but ZPP/H ratio can detect ID at early stages and can be performed easily at a relatively low cost. Therefore, ZPP/H ratio can serve as a potential screening test for pre-anemic iron deficiency in community pediatric practices.

  9. Long-term Treatment with Oriental Medicinal Herb Artemisia princeps Alters Neuroplasticity in a Rat Model of Ovarian Hormone Deficiency

    PubMed Central

    Kim, Hyun-Bum; Kwon, Byeong-Jae; Cho, Hyun-Ji; Kim, Ji-Won; Chon, Jeong-Woo; Do, Moon-Ho; Park, Sang-Yong; Kim, Sun-Yeou; Maeng, Sung-Ho; Park, Yoo-Kyoung

    2015-01-01

    Artemisia princeps (AP) is a flowering perennial used as a traditional medicine and dietary supplement across East Asia. No study has yet assessed its effects on synaptic plasticity in hippocampus and much less in a model of ovarian hormone deficiency. We examined the influence of chronic oral AP ethanol extract treatment in ovariectomized rats on the induction of long-term depression in a representative synapse (CA3-CA1) of the hippocampus. Ovariectomized rats demonstrated lower trabecular mean bone mineral densities than sham, validating the establishment of pathology. Against this background of pathology, AP-treated ovariectomized rats exhibited attenuated long-term depression (LTD) in CA1 relative to water-treated controls as measured by increased field excitatory post-synaptic potentials (fEPSP) activation averages over the post-stimulation period. While pathological significance of long-term depression (LTD) in ovariectomized rats is conflicting, that AP treatment significantly affected its induction offers justification for further study of its influences on plasticity and its related disorders. PMID:25792871

  10. Puberty, statural growth, and growth hormone release in children with cerebral palsy

    PubMed Central

    Kuperminc, Michelle N.; Gurka, Matthew J.; Houlihan, Christine M.; Henderson, Richard C.; Roemmich, James N.; Rogol, Alan D.

    2010-01-01

    Objective Children with cerebral palsy (CP) are smaller than normally growing children.. The association between the growth hormone (GH) axis and growth in children with CP during puberty is unknown. We compared growth and markers of the GH axis in pre-pubertal and pubertal children with moderate to severe CP and without CP over a three-year period. Study design Twenty children with CP, ages 6–18, Gross Motor Function Classification System levels III–V, were compared to a group of sixty-three normally growing children of similar age. Anthropometry, Tanner stage, bone age, and laboratory analyses were performed every six months for three years. Laboratory values included spontaneous overnight GH release, fasting IGF-1 and IGFBP-3. Repeated measures models were used to evaluate interactions among Tanner stage and group (children with CP vs. reference children), taking into account gender, age, and nutritional status. Results Children with CP grew more slowly than those without CP at all Tanner stages (p<0.01). Patterns of IGF-1 and GH secretion in children with CP were similar to those of the reference group; however, the concentrations of IGF-1 (p<0.01) and GH (p<0.01) were lower in girls with CP, with a similar trend for boys (p=0.10 and 0.14, respectively). Conclusions Diminished circulating IGF-1 and GH concentrations may explain the differences in growth between the two groups. PMID:20216931

  11. Multiple sulfatase deficiency: A case series of four children.

    PubMed

    Incecik, Faruk; Ozbek, Mehmet N; Gungor, Serdal; Pepe, Stefano; Herguner, Ozlem M; Mungan, Neslihan Onenli; Gungor, Sabiha; Altunbasak, Sakir

    2013-10-01

    Multiple sulfatase deficiency is biochemically characterized by the accumulation of sulfated lipids and acid mucopolysaccharides. The gene sulfatase-modifying factor 1 (SUMF1), recently identified, encodes the enzyme responsible for post-translational modification of a cysteine residue, which is essential for the activity of sulfatases. We describe clinical findings and mutation analysis of four patients. The patients presented with hypotonia, developmental delay, coarse face, ichthyosis, and hepatosplenomegaly. The diagnosis was made through clinical findings, enzymatic assays, and mutation analysis. We were detected to be homozygous for a novel missense mutation c. 739G > C causing a p.G247R amino acid substitution in the SUMF1 protein.

  12. Folate Deficiency, Atopy, and Severe Asthma Exacerbations in Puerto Rican Children

    PubMed Central

    Blatter, Joshua; Brehm, John M.; Sordillo, Joanne; Forno, Erick; Boutaoui, Nadia; Acosta-Pérez, Edna; Alvarez, María; Colón-Semidey, Angel; Weiss, Scott T.; Litonjua, Augusto A.; Canino, Glorisa

    2016-01-01

    Background: Little is known about folate and atopy or severe asthma exacerbations. We examined whether folate deficiency is associated with number of positive skin tests to allergens or severe asthma exacerbations in a high-risk population and further assessed whether such association is explained or modified by vitamin D status. Methods: Cross-sectional study of 582 children aged 6 to 14 years with (n = 304) and without (n = 278) asthma in San Juan, Puerto Rico. Folate deficiency was defined as plasma folate less than or equal to 20 ng/ml. Our outcomes were the number of positive skin tests to allergens (range, 0–15) in all children and (in children with asthma) one or more severe exacerbations in the previous year. Logistic and negative binomial regression models were used for the multivariate analysis. All multivariate models were adjusted for age, sex, household income, residential proximity to a major road, and (for atopy) case/control status; those for severe exacerbations were also adjusted for use of inhaled corticosteroids and vitamin D insufficiency (a plasma 25[OH]D < 30 ng/ml). Measurements and Main Results: In a multivariate analysis, folate deficiency was significantly associated with an increased degree of atopy and 2.2 times increased odds of at least one severe asthma exacerbation (95% confidence interval for odds ratio, 1.1–4.6). Compared with children who had normal levels of both folate and vitamin D, those with both folate deficiency and vitamin D insufficiency had nearly eightfold increased odds of one or more severe asthma exacerbation (95% confidence interval for adjusted odds ratio, 2.7–21.6). Conclusions: Folate deficiency is associated with increased degree of atopy and severe asthma exacerbations in school-aged Puerto Ricans. Vitamin D insufficiency may further increase detrimental effects of folate deficiency on severe asthma exacerbations. PMID:26561879

  13. Relationships among blood lead levels, iron deficiency, and cognitive development in two-year-old children.

    PubMed Central

    Ruff, H A; Markowitz, M E; Bijur, P E; Rosen, J F

    1996-01-01

    The goals of this study were to explore the relationship of declining blood lead levels and cognitive development in 42 moderately lead poisoned children around 2 years of age and to investigate the potential interaction between iron and lead levels in the course of development. The cognitive functioning of children was assessed upon enrollment into a comprehensive intervention and 6 months later. The intervention consisted of chelation treatment, if appropriate, iron supplementation, if needed, and steps to eliminate the source of lead in the home environment. The children were referred because of blood lead levels between 25 and 55 mu g/dl; they were also selected on the basis of age between 18 and 30 months. The outcome measures were the global score on a standardized test of cognitive development and subscale scores for perceptual-motor and language functioning. Cognitive change over 6 months was related to an interaction between change in blood lead and initial iron status. Specifically, the change in standardized score (particularly change in perceptual-motor performance) was strongly related to change in blood lead in children who were iron sufficient at the outset: there was an increase of 1.2 points for every 1 mu g/dl decrease in blood lead. There was no such relationship in iron-deficient children. Secondary analyses suggested that 1) the change in cognitive functioning of iron-deficient children was related to change in hemoglobin, and 2) the decline in blood lead was less in iron-deficient than in iron-sufficient children. Thus, when iron is sufficient, changes in blood lead and changes in cognition are inversely related. When iron is deficient, other processes affect the outcome. PMID:8820586

  14. Effectiveness of prosthetic rehabilitation of children with limb deficiencies present at birth.

    PubMed

    Sener, G; Yiğiter, K; Bayar, K; Erbahçeci, F

    1999-08-01

    This study was performed to investigate the efficacy of prosthetic rehabilitation in children with congenital malformations, also to determine whether there were any factors related to family history and pregnancy which could have affected the formation of such deficiencies. Twenty-five (25) limb deficient children were provided with prostheses and received rehabilitation. A detailed family and pregnancy history was researched through the records. The ability of performing activities of daily living was scored on a four point scale. Thirteen (13) lower and 12 upper limb deficient children with a mean age of 4.88+/-2.52 years were included in the study. Ten (10) children had longitudinal and 15 transverse deficiencies. Traditional marriages amongst close cousins were observed in 31%. Five (5) mothers used medications, 2 received radiodiagnostic tests and 2 had bleedings during pregnancy. Some 84.7% of the lower limb group became independent walkers without requiring walking aids while 15.3% of them required Canadian crutches for independent ambulation. A total of 61.5% of these children actively participated in recreation with peers. In the upper limb group 41.6% were completely independent in self-care, feeding and hand skills; 33.3% showed independence with self-help devices while 25% tended to use the prostheses for assistive purposes. Participation in recreational activities was 58.2%. The author's results have shown that the children gained a functional activity level although the prostheses were provided in a late period. It can be concluded that success in rehabilitating these children is a true challenge.

  15. Vitamin D deficiency in children aged 6 to 12 years: single center's experience in Busan

    PubMed Central

    Roh, Young Eun; Kim, Bo Ryung; Choi, Won Bok; Kim, Young Mi; Cho, Min-Jung; Kim, Hye-Young; Park, Kyung Hee; Kim, Kwang Hoon; Chun, Peter; Kim, Su Young

    2016-01-01

    Purpose This study investigated the prevalence and risk factors associated with vitamin D deficiency in children. Methods We analyzed the medical records of 330 patients from the age of 6 to 12, who visited the endocrinology clinic of the Department of Pediatrics at Pusan National University Hospital, from September, 2013 to May, 2014. According to their serum 25-hydroxyvitamin D (25(OH)D) levels, the patients were grouped into either the deficiency group (25(OH)D<20 ng/mL), or the sufficiency group (25(OH)D≥20 ng/mL). The differences between the 2 groups were compared. Results There were 195 patients (59.1%) who had vitamin D deficiency. Their mean serum 25(OH)D level was 14.86±3.20 ng/mL. The differences in sex, age, and pubertal status between the 2 groups were not statistically significant. Weight standard deviation score (SDS), and body mass index SDS, were significantly higher in the vitamin D deficiency group (P=0.002 for each), compared to the sufficiency group. Compared with Autumn, both Spring (odds ratio [OR], 9.7; 95% confidence interval [CI], 4.3–22.0), and Winter (OR, 5.9; 95% CI, 3.5–10.0), were risk factors for vitamin D deficiency. In multiple logistic regression analysis, only seasonal differences have been confirmed to have an effect on vitamin D deficiency. Conclusion Vitamin D deficiency in children aged 6 to 12 years is very common. Spring and Winter are the most important risk factors for vitamin D deficiency. We suggest that it is necessary to supplement the guideline for the vitamin D intake according to our situation. PMID:27777907

  16. Growth hormone (GH) treatment reverses early atherosclerotic changes in GH-deficient adults.

    PubMed

    Pfeifer, M; Verhovec, R; Zizek, B; Prezelj, J; Poredos, P; Clayton, R N

    1999-02-01

    Hypopituitary patients have increased mortality from vascular disease, and in these patients, early markers of atherosclerosis [increased carotid artery intima-media thickness (IMT) and reduced distensibility] are more prevalent. As GH replacement can reverse some risk factors of atherosclerosis, the present study examined the effect of GH treatment on morphological and functional changes in the carotid and brachial arteries of GH-deficient (GHD) adults. Eleven GHD hypopituitary men (24-49 yr old) were treated with recombinant human GH (0.018 U/kg BW x day) for 18 months. IMT of the common carotid artery (CCA) and the carotid bifurcation (CB), and flow-mediated endothelium-dependent dilation (EDD) of the brachial artery were measured by B mode ultrasound before and at 3, 6, 12, and 18 months of treatment, and values were compared with those in 12 age-matched control men. Serum concentrations of lipids, lipoprotein(a), insulin-like growth factor I (IGF-I), and IGF-binding protein-3 (IGFBP-3) were also measured. In GHD men before treatment the IMTs of the CCA [mean(SD), 0.67(0.05) mm] and CB [0.75(0.04) mm] were significantly greater (P < 0.001) than those in control men [0.52(0.07) and 0.65(0.07) mm, respectively]. GH treatment normalized the IMT of the CCA by 6 months [0.53(0.04) mm] and that of the CB by 3 months [0.68(0.05) mm]. The IMT of the carotid artery (CCA and CB) was negatively correlated with serum IGF-I (r = -0.53; P < 0.0001). There was a significant improvement in flow-mediated EDD of the brachial artery at 3 months, which was sustained at 6 and 18 months of GH treatment (P < 0.05). GH treatment increased high density lipoprotein cholesterol at 3 and 6 months, but did not reduce total or low density lipoprotein cholesterol and was without effect on lipoprotein(a). There was no correlation between plasma lipids and changes in IMT or EDD of the arteries examined. In conclusion, GH treatment of hypopituitary GHD men reverses early morphological and

  17. Behavioral Effects of Org 2766, a Synthetic Analog of the Adrenocorticotrophic Hormone (4-9), in 14 Outpatient Autistic Children.

    ERIC Educational Resources Information Center

    Buitelaar, Jan K.; And Others

    1990-01-01

    Fourteen autistic children (ages 5-13) were administered Org 2766 (a synthetic analog of the adrenocorticotrophic hormone 4-9) or a placebo for 4 weeks. The hormone appeared to decrease stereotypic behavior and increase such behaviors as "change toys,""locomote," and "talk," though Aberrant Behavior Checklist ratings…

  18. Consequences of not treating children with laron syndrome (primary growth hormone insensitivity).

    PubMed

    Laron, Z

    2001-01-01

    The follow-up of a large cohort of patients with Laron syndrome (LS) from infancy to adult age has enabled us to determine the effects of long-term insulin-like growth factor-I (IGF-I) deficiency on auxological, biochemical, physiological and psychological parameters. We found that early and continuous IGF-I deficiency (the anabolic effector of growth hormone) causes dwarfism, acromicria, organomicria, marked obesity, insulin resistance, retardation of skeletal maturation and osteoporosis, as well as muscular and central nervous tissue underdevelopment, and a series of biochemical changes including hypercholesterolemia. These multiple pathologies impair the quality of life of these patients. It is concluded that patients with LS need IGF-I replacement treatment throughout life.

  19. Nutritional deficiencies and overweight prevalence among children with autism spectrum disorder.

    PubMed

    Shmaya, Yael; Eilat-Adar, Sigal; Leitner, Yael; Reif, Shimon; Gabis, Lidia

    2015-03-01

    Children with autism spectrum disorder (ASD) are at risk of developing nutritional deviations. Three to six year old children with ASD were compared to their typically developing siblings and to a typically developing age and gender matched control group, in order to evaluate their intake and body mass index. Nutrient intake was compared to the Dietary Reference Intake using three-day diet diaries completed by the parents. The sum percentage of nutritional deficiencies in the ASD group compared to the typical development group was 342.5% (±122.9%) vs. 275.9% (±106.8%), respectively (P=0.026). A trend toward higher deficiency in the ASD group was observed as compared to the sibling group 363% (±122.9%) vs. 283.2% (±94.7%) (P=0.071). A higher body mass index was found in the ASD group compared to their counterparts, despite their nutritional deficiencies. In conclusion, children with ASD are more likely to suffer from nutritional deficiencies despite higher body mass index.

  20. Iron Deficiency and Other Types of Anemia in Infants and Children.

    PubMed

    Wang, Mary

    2016-02-15

    Anemia, defined as a hemoglobin level two standard deviations below the mean for age, is prevalent in infants and children worldwide. The evaluation of a child with anemia should begin with a thorough history and risk assessment. Characterizing the anemia as microcytic, normocytic, or macrocytic based on the mean corpuscular volume will aid in the workup and management. Microcytic anemia due to iron deficiency is the most common type of anemia in children. The American Academy of Pediatrics and the World Health Organization recommend routine screening for anemia at 12 months of age; the U.S. Preventive Services Task Force found insufficient evidence to assess the benefits vs. harms of screening. Iron deficiency anemia, which can be associated with cognitive issues, is prevented and treated with iron supplements or increased intake of dietary iron. The U.S. Preventive Services Task Force found insufficient evidence to recommend screening or treating pregnant women for iron deficiency anemia to improve maternal or neonatal outcomes. Delayed cord clamping can improve iron status in infancy, especially for at-risk populations, such as those who are preterm or small for gestational age. Normocytic anemia may be caused by congenital membranopathies, hemoglobinopathies, enzymopathies, metabolic defects, and immune-mediated destruction. An initial reticulocyte count is needed to determine bone marrow function. Macrocytic anemia, which is uncommon in children, warrants subsequent evaluation for vitamin B12 and folate deficiencies, hypothyroidism, hepatic disease, and bone marrow disorders.

  1. Lack of association between plasma leptin levels and appetite in children with iron deficiency.

    PubMed

    Topaloglu, A K; Hallioglu, O; Canim, A; Duzovali, O; Yilgor, E

    2001-01-01

    A negative correlation between leptin and appetite or food intake has been shown in healthy individuals. However, the role of leptin in clinical conditions characterized by anorexia has not been established. One of the well-known clinical features of iron-deficiency anemia is poor appetite. We examined the changes in plasma leptin levels in relation to expected improvement in appetite with iron treatment in children with iron deficiency. In 24 infants and small children (mean age +/- standard deviation = 19.6 +/- 7.7 months) with iron deficiency, we studied plasma leptin levels before and after iron therapy. After 15.0 +/- 2.4 wk of iron treatment, serum ferritin levels improved significantly, with accompanying increases in their subjective appetite scores and food intakes. However, as their mean age and plasma leptin levels adjusted their body mass indexes were unchanged. Serum ferritin correlated significantly with appetite score (r = 0.680, P < 0.001) and food intake (r = 0.480, P < 0.01). Leptin correlated only with body mass index (r = 0.405, P < 0.01). Lack of association between plasma leptin levels and degree of appetite in iron-deficient children treated with iron suggests a leptin-independent mechanism for the observed increase in appetite.

  2. Growth of short children born small for gestational age and their response to growth hormone therapy.

    PubMed

    Prasad, Hemchand Krishna; Khadilkar, Vaman V; Chiplonkar, Shashi A; Khadilkar, Anuradha V

    2013-05-08

    Growth hormone [GH] is licensed for use in children born small for gestational age (SGA) who fail to catch-up. We retrospectively compared the response of twenty children born SGA (who satisfied the auxological criteria) to growth hormone (Group I) versus randomly selected age and sex matched controls from a group of SGA children with growth related complaints, not treated with GH (Group II). After 2 years of GH therapy the HAZ increased from -2.8 to -1.6 in Group I, compared 2.2 to -1.7 in group II (P-value < 0.05). The percentage of pubertal children rose from 55% to 65% in cases versus 60% to 75% in the controls (P>0.05). GH resulted in increase in growth velocity Z-score during the first year and (4.3±0.5 in Group-I versus - 0.5±0.6 in Group-II, P<0.05) second year of treatment (1.7±0.4 in cases versus -0.6±0.7 in controls, P<0.05).Thus, GH improves height of short SGA children without accelerating pubertal progression.

  3. Study of the correlation between growth hormone deficiency and serum leptin, adiponectin, and visfatin levels in adults.

    PubMed

    Li, Z-P; Zhang, M; Gao, J; Zhou, G-Y; Li, S-Q; An, Z-M

    2014-02-14

    We aimed to determine the significance and changes in leptin, adiponectin (ADP), and visfatin levels in adults with growth hormone deficiency (GHD). Forty adults (19 men, 21 women) who had been diagnosed with GHD comprised the observation group, while 36 healthy adults (18 men, 18 women) were used as the control group. Fasting venous blood was collected to detect leptin, ADP, and visfatin levels. There was no statistically significant difference (P > 0.05) between the GHD group and the control group in terms of gender ratio, age, and body mass index. The waist-to-hip ratio (0.894 ± 0.061 vs 0.830 ± 0.481), cholesterol (4.99 ± 1.046 vs 4.18 ± 0.683), triglyceride (1.97 ± 1.428 vs 1.08 ± 0.403), LDL (2.91 ± 0.980 vs 2.29 ± 0.540), leptin (3.00 ± 1.233 vs 1.89 ± 1.554), ADP (15.26 ± 6.449 vs 10.24 ± 7.608), and visfatin levels (10.42 ± 3.715 vs 5.87 ± 3.90) in the GHD group were significantly higher than those in the control group (all P < 0.05). The levels of growth hormone (1.68 ± 1.67 vs 15.53 ± 6.23), insulin-like growth factor-1 (IGF-1, 22.64 ± 16.41 vs 61.85 ± 28.48), IGF-binding protein-3 (4889 ± 2962 vs 6866 ± 3823), and dehydroepiandrosterone sulfate (1.466 ± 1.804 vs 6.000 ± 2.767) in the GHD group were significantly lower than those in the control group (all P < 0.05). Correlation analysis demonstrated that leptin level was positively correlated to ADP and visfatin in both the GHD and control groups and negatively correlated to IGF-1 (r = 0.332, P < 0.05). Logistic regression analysis demonstrated that leptin, ADP, and visfatin were independent risk factors for adults with GHD.

  4. Reinforcement and Stimulant Medication Ameliorate Deficient Response Inhibition in Children with Attention-Deficit/Hyperactivity Disorder.

    PubMed

    Rosch, Keri S; Fosco, Whitney D; Pelham, William E; Waxmonsky, James G; Bubnik, Michelle G; Hawk, Larry W

    2016-02-01

    This study examined the degree to which reinforcement, stimulant medication, and their combination impact response inhibition in children with Attention-Deficit/Hyperactivity Disorder (ADHD). Across three studies, participants with ADHD (n = 111, 25 girls) and typically-developing (TD) controls (n = 33, 6 girls) completed a standard version of the stop signal task (SST) and/or a reinforcement-manipulation SST with performance-contingent points. In two of these studies, these tasks were performed under placebo or 0.3 and 0.6 mg/kg methylphenidate (MPH) conditions. Cross-study comparisons were conducted to test hypotheses regarding the separate and combined effects of reinforcement and methylphenidate on response inhibition among children with ADHD relative to TD controls. Baseline response inhibition was worse among children with ADHD compared to controls. MPH produced dose-related improvements in response inhibition in children with ADHD; compared to non-medicated TD controls, 0.3 mg/kg MPH normalized deficient response inhibition, and 0.6 mg/kg MPH resulted in better inhibition in children with ADHD. Reinforcement improved response inhibition to a greater extent for children with ADHD than for TD children, normalizing response inhibition. The combination of MPH and reinforcement improved response inhibition among children with ADHD compared to reinforcement alone and MPH alone, also resulting in normalization of response inhibition despite repeated task exposure. Deficient response inhibition commonly observed in children with ADHD is significantly improved with MPH and/or reinforcement, normalizing inhibition relative to TD children tested under standard conditions.

  5. [Prevalence of sub-clinical vitamin A deficiency and malnutrition in slum children in Maraicabo - Venezuela].

    PubMed

    Castejón, H V; Ortega, P; Díaz, M E; Amaya, D; Gómez, G; Ramos, M; Alvarado, M V; Urrieta, J R

    2001-03-01

    The present cross sectional study was carried out to estimate the prevalence of vitamin A deficiency among children by means of clinics and conjunctival impression cytology (CIC), and nutritional status by anthropometric indicators H//A, W//A, W//H. The study population included 157 children 2-6 y old, from urban and rural slums of Maracaibo, Venezuela, Conjunctival impression cytology was performed by ICEPO standard procedure. Z-score was applied to anthropometric data with reference values of NCHS-WHO. No evidence of clinical or ophthalmologic signs of vitamin A deficiency were detected. The prevalence of subclinical vitamin A deficiency, as detected by abnormal CIC, was 35.4%, being higher in rural children (48.3%). These prevalence values are higher than the criteria laid down by WHO/UNICEF to indicate a public health problem (> 20%). Mild or moderate protein-energy global malnutrition and stunting were detected in 36.1% and 44.6% of children, respectively. Abnormal CIC was indistinctly observed (approximately equal to 35%) as much in children with adequate nutrition as in malnourished ones. There was no significant difference in the distribution of the CIC results in relation to nutritional status. The findings indicate that CIC and Z-score of nutritional anthropometric data are useful to characterize the risk of vitamin A deficiency and of malnutrition in communities. Beside the implementation of an integral nutritional program which includes supplementation, food fortification and dietary diversification, improvement of socio-economic and sanitation conditions and also the educational level, with emphasis on nutritional and health education, are highly recommended.

  6. Effects of maternal education on diet, anemia, and iron deficiency in Korean school-aged children

    PubMed Central

    2011-01-01

    Background We investigated the relationship among socioeconomic status factors, the risk of anemia, and iron deficiency among school-aged children in Korea. Methods The sample consisted of fourth-grade students aged 10 y recruited from nine elementary schools in Korean urban areas in 2008 (n = 717). Anthropometric and blood biochemistry data were obtained for this cross-sectional observational study. Anemia was defined as hemoglobin levels lower than 11.5 g/dl. Iron deficiency was defined as serum iron levels lower than 40 ug/dl. We also obtained data on parental education from questionnaires and on children's diets from 3-day food diaries. Parental education was categorized as low or high, with the latter representing an educational level beyond high school. Results Children with more educated mothers were less likely to develop anemia (P = 0.0324) and iron deficiency (P = 0.0577) than were those with less educated mothers. This group consumed more protein (P = 0.0004) and iron (P = 0.0012) from animal sources than did the children of less educated mothers, as reflected by their greater consumption of meat, poultry, and derivatives (P < 0.0001). Logistic regression analysis revealed a significant inverse relationship between maternal education and the prevalence of anemia (odds ratio: 0.52; 95% confidence interval: 0.32, 0.85). Conclusions As a contributor to socioeconomic status, maternal education is important in reducing the risk of anemia and iron deficiency and in increasing children's consumption of animal food sources. PMID:22087564

  7. Characterization of soybean β-expansin genes and their expression responses to symbiosis, nutrient deficiency, and hormone treatment.

    PubMed

    Li, Xinxin; Zhao, Jing; Walk, Thomas C; Liao, Hong

    2014-03-01

    Expansins are plant cell wall-loosening proteins encoded by a superfamily of genes including α-expansin, β-expansin, expansin-like A, and expansin-like B proteins. They play a variety of biological roles during plant growth and development. Expansin genes have been reported in many plant species, and results primarily from graminaceous members indicate that β-expansins are more abundant in monocots than in dicots. Soybean [Glycine max (L.) Merr] is an important legume crop. This work identified nine β-expansin gene family members in soybean (GmEXPBs) that were divided into two distinct classes based on phylogeny and gene structure, with divergence between the two groups occurring more in introns than in exons. A total of 887 hormone-responsive and environmental stress-related putative cis-elements from 188 families were found in the 2-kb upstream region of GmEXPBs. Variations in number and type of cis-elements associated with each gene indicate that the function of these genes is differentially regulated by these signals. Expression analysis confirmed that the family members were ubiquitously, yet differentially expressed in soybean. Responsiveness to nutrient deficiency stresses and regulation by auxin (indole-3-acetic acid) and cytokinin (6-benzylaminopurine) varied among GmEXPBs. In addition, most β-expansin genes were associated with symbiosis of soybean inoculated with Rhizobium or abuscular mycorrhizal fungi (AMF). Taken together, these results systematically investigate the characteristics of the entire GmEXPB family in soybean and comprise the first report analyzing the relationship of GmEXPBs with rhizobial or AMF symbiosis. This information is a valuable step in the process of understanding the expansin protein functions in soybean and opens avenues for continued researches.

  8. Vitamin D Deficiency among Female Nurses of Children's Medical Center Hospital and Its Related Factors.

    PubMed

    Rajebi, Hamid; Khodadad, Ahmad; Fahimi, Golshan; Abolhassani, Hassan

    2016-02-01

    Vitamin D deficiency is one of the most preventable challenges worldwide. The aim of this study was to determine the prevalence of vitamin D deficiency among female nurses working at Children's Medical Center Hospital in Tehran, Iran, due to the risk factor of being a notably long period indoors and the fact that their health status may have consequences on the process of patients' treatment. A total of 114 female nurses who were at least 20 years old entered the study voluntarily, and a questionnaire was applied to collect information on lifestyle and other factors associated with vitamin D deficiency. A sample of blood was taken to measure 25-hydroxyvitamin D (25-OHD) and cut off value to indicate deficiency was considered below 10 ng/ml, and the amounts of 10-29 ng/ml were declared insufficient. The mean of 25-OHD was 11.7±9.3 ng/ml. A total of 79 subjects (69.3%) had a deficient level of vitamin D, 28 subjects (24.6%) had an insufficient level and only 7 subjects (6.1%) had sufficient level of vitamin D. The deficiency was more noticeable in the age group of 26-35 years old. Prevalence of vitamin D deficiency had a significant correlation with younger subjects (P<0.001). There was no significant association among other factors such as body mass index (BMI), health status complications, regular exercise, and duration of sun exposure. High prevalence of vitamin D deficiency in the study population leads to emphasise the need to screen health care workers for vitamin D levels.

  9. Low and Normal IGF-1 Levels in Patients with Chronic Medical Disorders (CMD) is Independent of Anterior Pituitary Hormone Deficiencies: Implications for Treating IGF-1 Abnormal Deficiencies with CMD.

    PubMed

    Braverman, E; Oscar-Berman, M; Lohmann, R; Kennedy, R; Kerner, M; Dushaj, K; Blum, K

    2013-02-09

    Over time, based on evidence-based medicine, a number of hormonal test levels including IGF-1 had been raised or lowered to meet new criteria standards. In particular, IGF-1 plasma levels have been shown in several studies to be an independent diagnostic tool in Adult Growth Hormone Deficiency (AGHD). Many endocrinology studies link low IGF-1 plasma levels with low levels of other anterior pituitary hormones (i.e., LH, FSH, and TSH). Low IGF-1 is considered by most to be between 84-100 µ/l and numerous studies recommend that raising IGF-1 to high normal range reverses Chronic Medical Diseases (CMD), improves bone mineral density (BMD), and fibromyalgia. Moreover, some studies suggest that low levels of IGF-1 by itself independent of anterior pituitary deficiencies is sufficient to determine AGHD in humans. In order to determine the relationship of low IGF-1 with that of LH, FSH, and TSH levels in subjects with CMD, we evaluated these levels (± SD) in 944 patients. Patients with IGF-1 below 84 µ/l, 100 µ/l, and 150 µ/l were accessed. 9.22% had less than 84 µ/l (SD ± 12.52); 19.9% had less than 100 µ/l (SD ± 9.54); and 51.6 had less than 150 µ/l (SD ± 26.0). Specifically, the percentages found for low LH, FSH, and TSH were only 4.2%, 4.8%, and 6.5%. We conclude that IGF-1 deficiencies occur independent of comorbid deficiencies of LH, FSH, and TSH. Finally, we propose that based on the present investigation, IGF-1 low levels between the range of 84-100 µ/l may be too low to be considered as an independent diagnostic marker to treat AGHD with CMD.

  10. Low and Normal IGF-1 Levels in Patients with Chronic Medical Disorders (CMD) is Independent of Anterior Pituitary Hormone Deficiencies: Implications for Treating IGF-1 Abnormal Deficiencies with CMD

    PubMed Central

    Braverman, E; Oscar-Berman, M; Lohmann, R; Kennedy, R; Kerner, M; Dushaj, K; Blum, K

    2013-01-01

    Over time, based on evidence–based medicine, a number of hormonal test levels including IGF-1 had been raised or lowered to meet new criteria standards. In particular, IGF-1 plasma levels have been shown in several studies to be an independent diagnostic tool in Adult Growth Hormone Deficiency (AGHD). Many endocrinology studies link low IGF-1 plasma levels with low levels of other anterior pituitary hormones (i.e., LH, FSH, and TSH). Low IGF-1 is considered by most to be between 84–100 µ/l and numerous studies recommend that raising IGF-1 to high normal range reverses Chronic Medical Diseases (CMD), improves bone mineral density (BMD), and fibromyalgia. Moreover, some studies suggest that low levels of IGF-1 by itself independent of anterior pituitary deficiencies is sufficient to determine AGHD in humans. In order to determine the relationship of low IGF-1 with that of LH, FSH, and TSH levels in subjects with CMD, we evaluated these levels (± SD) in 944 patients. Patients with IGF-1 below 84 µ/l, 100 µ/l, and 150 µ/l were accessed. 9.22% had less than 84 µ/l (SD ± 12.52); 19.9% had less than 100 µ/l (SD ± 9.54); and 51.6 had less than 150 µ/l (SD ± 26.0). Specifically, the percentages found for low LH, FSH, and TSH were only 4.2%, 4.8%, and 6.5%. We conclude that IGF-1 deficiencies occur independent of comorbid deficiencies of LH, FSH, and TSH. Finally, we propose that based on the present investigation, IGF-1 low levels between the range of 84–100 µ/l may be too low to be considered as an independent diagnostic marker to treat AGHD with CMD. PMID:23616929

  11. Muscle and skeletal health in children and adolescents with GH deficiency.

    PubMed

    Improda, Nicola; Capalbo, Donatella; Esposito, Andrea; Salerno, Mariacarolina

    2016-12-01

    In addition to promoting linear growth, GH plays a key role in the regulation of bone and muscle development and metabolism. Although GH deficiency is frequently listed among the causes of secondary osteoporosis in children, its impact on bone and muscle health and on fracture risk is still not completely established. Current data suggest that childhood-onset GH deficiency can affect bone and muscle mass and strength, with GH replacement therapy exerting beneficial effects. Moreover, GH withdrawal at final height can result in reduced peak bone and muscle mass, potentially leading to increased fracture risk in adulthood. Thus, the muscle-bone unit in GH deficient subjects should be monitored during childhood and adolescence in order to prevent osteoporosis and increased fracture risk and GH replacement should be tailored to ensure an optimal bone and muscle health.

  12. Constitutional Delay Influences the Auxological Response to Growth Hormone Treatment in Children with Short Stature and Growth Hormone Sufficiency

    PubMed Central

    Gunn, Katherine C.; Cutfield, Wayne S.; Hofman, Paul L.; Jefferies, Craig A.; Albert, Benjamin B.; Gunn, Alistair J.

    2014-01-01

    In a retrospective, population based cohort study, we examined whether constitutional delay was associated with the growth response to growth hormone (GH) in children with short stature and normal GH responses. 70 patients were treated with 21 GH iu/m2/week from 1975 to 2013 throughout New Zealand. Demographic and auxological data were prospectively collected and standard deviation scores (SDS) were calculated for height (HtSDS), yearly growth velocity (GV-SDS), body mass index (BMI-SDS) and predicted adult height (PAH-SDS) at time of the last available bone age. In the first year, GH was associated with marked increase in HtSDS (+0.46 (0.19, 0.76), p < 0.001) and GV-SDS (from −1.9 (−3.6, −0.7) to +2.7 (0.45, 4.2), p < 0.001). The increase in HtSDS but not in GV-SDS was greatest with younger patients and greater bone age delay, with no effect of sex, BMI-SDS or baseline HtSDS. PAH-SDS increased with treatment (+0.94 (0.18, 1.5)); increased PAH-SDS was associated with less bone age delay and greater initial increase in HtSDS. This study shows that greater bone age delay was associated with greater initial improvement in height but less improvement in predicted adult heights, suggesting that children with very delayed bone ages may show accelerated maturation during GH treatment. PMID:25317732

  13. Pituitary response to thyrotropin releasing hormone in children with overweight and obesity

    PubMed Central

    Rijks, Jesse; Penders, Bas; Dorenbos, Elke; Straetemans, Saartje; Gerver, Willem-Jan; Vreugdenhil, Anita

    2016-01-01

    Thyroid stimulating hormone (TSH) concentrations in the high normal range are common in children with overweight and obesity, and associated with increased cardiovascular disease risk. Prior studies aiming at unravelling the mechanisms underlying these high TSH concentrations mainly focused on factors promoting thyrotropin releasing hormone (TRH) production as a cause for high TSH concentrations. However, it is unknown whether TSH release of the pituitary in response to TRH is affected in children with overweight and obesity. Here we describe TSH release of the pituitary in response to exogenous TRH in 73 euthyroid children (39% males) with overweight or (morbid) obesity. Baseline TSH concentrations (0.9–5.5 mU/L) were not associated with BMI z score, whereas these concentrations were positively associated with TSH concentrations 20 minutes after TRH administration (r2 = 0.484, p < 0.001) and the TSH incremental area under the curve during the TRH stimulation test (r2 = 0.307, p < 0.001). These results suggest that pituitary TSH release in response to TRH stimulation might be an important factor contributing to high normal serum TSH concentrations, which is a regular finding in children with overweight and obesity. The clinical significance and the intermediate factors contributing to pituitary TSH release need to be elucidated in future studies. PMID:27485208

  14. Predicting longitudinal growth curves of height and weight using ecological factors for children with and without early growth deficiency.

    PubMed

    Black, M M; Krishnakumar, A

    1999-02-01

    Growth curve models were used to examine the effect of genetic and ecological factors on changes in height and weight of 225 children from low income, urban families who were assessed up to eight times in the first 6 y of life. Children with early growth deficiency [failure to thrive (FTT)] (n = 127) and a community sample of children without growth deficiency (n = 98) were examined to evaluate how genetic, child and family characteristics influenced growth. Children of taller and heavier parents, who were recruited at younger ages and did not have a history of growth deficiency, had accelerated growth from recruitment through age 6 y. In addition, increases in height were associated with better health, less difficult temperament, nurturant mothers and female gender; increases in weight were associated with better health. Children with a history of growth deficiency demonstrated slower rates of growth than children in the community group without a history of growth deficiency. In the community group, changes in children's height and weight were related to maternal perceptions of health and temperament and maternal nurturance during feeding, whereas in the FTT group, maternal perceptions and behavior were not in synchrony with children's growth. These findings suggest that, in addition to genetic factors, growth is dependent on a nurturant and sensitive caregiving system. Interventions to promote growth should consider child and family characteristics, including maternal perceptions of children's health and temperament and maternal mealtime behavior.

  15. Ferrous sulfate versus iron polymaltose complex for treatment of iron deficiency anemia in children.

    PubMed

    Bopche, Ankur Vikas; Dwivedi, Rashmi; Mishra, Rakesh; Patel, G S

    2009-10-01

    We assessed the clinical response and side effects of Ferrous sulfate (FS) and Iron polymaltose complex (IPC) in 118 children with Iron deficiency anemia (IDA). Subjects were randomized to receive therapy with either oral IPC (Group A, n=59) or oral FS (Group B, n=59); all were given elemental iron in three divided doses of 6 mg/kg/day. One hundred and six children could be followed up; 53 in each group. Children who received ferrous sulfate were having higher hemoglobin level, and less residual complaints as compared to those who had received iron polymaltose complex. Our study suggests ferrous sulfate has a better clinical response and less significant adverse effects during treatment of IDA in children.

  16. Urinary levels of early kidney injury molecules in children with vitamin B12 deficiency.

    PubMed

    Güneş, Ali; Aktar, Fesih; Tan, İlhan; Söker, Murat; Uluca, Ünal; Balık, Hasan; Mete, Nuriye

    2016-10-01

    The aim of this study was to investigate urine early kidney injury molecules, including human kidney injury molecule-1 (KIM-1), liver-type fatty-acid binding protein (L-FABP), N-acetyl-b-D-glucosaminidase A (NAG), and neutrophil gelatinase-associated lipocalin (NGAL) in children with vitamin B12 (cobalamin) deficiency (CD). Twelve children with vitamin B12 deficiency and 20 healthy matched controls were included. Hematologic parameters, serum urea, creatinine (Cr), electrolytes, B12 and folate levels were recorded. Estimated glomerular filtration rate (eGFR) was calculated. Urine protein, electrolytes, andurinary early markers were measured. Patients with CD had significantly higher urine electrolyte/Cr ratios (p <0.05). Significantly higher urinary KIM-1/Cr, L-FABP/Cr, NAG/Cr and NGAL/Cr were found in CD group (p <0.05). Significant negative correlations were found between levels of serum B12 and urinary markers in the patients (p <0.05). Increased urinary kidney injury molecules and electrolytes in children with B12 deficiency suggest a possible subclinical renal dysfunction, which cannot be determined by conventional kidney function tests.

  17. MANAGEMENT OF ENDOCRINE DISEASE: Growth and growth hormone therapy in short children born preterm.

    PubMed

    Boguszewski, Margaret Cristina da Silva; Cardoso-Demartini, Adriane de Andre

    2017-03-01

    Approximately 15 million babies are born preterm across the world every year, with less than 37 completed weeks of gestation. Survival rates increased during the last decades with the improvement of neonatal care. With premature birth, babies are deprived of the intense intrauterine growth phase, and postnatal growth failure might occur. Some children born prematurely will remain short at later ages and adult life. The risk of short stature increases if the child is also born small for gestational age. In this review, the effects of being born preterm on childhood growth and adult height and the hormonal abnormalities possibly associated with growth restriction are discussed, followed by a review of current information on growth hormone treatment for those who remain with short stature during infancy and childhood.

  18. Vitamin A deficiency and inflammatory markers among preschool children in the Republic of the Marshall Islands

    PubMed Central

    Maqsood, Maria; Dancheck, Barbara; Gamble, Mary V; Palafox, Neal A; Ricks, Michelle O; Briand, Kennar; Semba, Richard D

    2004-01-01

    Background The exclusion of individuals with elevated acute phase proteins has been advocated in order to improve prevalence estimates of vitamin A deficiency in surveys, but it is unclear whether this will lead to sampling bias. The purpose of the study was to determine whether the exclusion of individuals with elevated acute phase proteins is associated with sampling bias and to characterize inflammation in children with night blindness. Methods In a survey in the Republic of the Marshall Islands involving 281 children, aged 1–5 years, serum retinol, C-reactive protein (CRP), and α1-acid glycoprotein (AGP) were measured. Results Of 281 children, 24 (8.5%) had night blindness and 165 (58.7%) had serum retinol <0.70 μmol/L. Of 248 children with AGP and CRP measurements, 123 (49.6%) had elevated acute phase proteins (CRP >5 mg/L and/or AGP >1000 mg/L). Among children with and without night blindness, the proportion with serum retinol <0.70 μmol/L was 79.2% and 56.8% (P = 0.03) and with anemia was 58.3% and 35.7% (P = 0.029), respectively. The proportion of children with serum retinol <0.70 μmol/L was 52.0% after excluding children with elevated acute phase proteins. Among children with and without elevated acute phase proteins, mean age was 2.8 vs 3.2 years (P = 0.016), the proportion of boys was 43.1% vs. 54.3% (P = 0.075), with no hospitalizations in the last year was 11.0% vs 23.6% (P = 0.024), and with anemia was 43.8% vs 31.7% (P = 0.05), respectively. Conclusions Exclusion of children with inflammation in this survey of vitamin A deficiency does not improve prevalence estimates for vitamin A deficiency and instead leads to sampling bias for variables such as age, gender, anemia, and hospitalization history. PMID:15588289

  19. Reduced pulsatile growth hormone secretion in children after therapy for acute lymphoblastic leukemia

    SciTech Connect

    Blatt, J.; Bercu, B.B.; Gillin, J.C.; Mendelson, W.B.; Poplack, D.G.

    1984-02-01

    Basal growth hormone levels were measured every 20 minutes over 24 hours in eight long-term survivors of acute lymphoblastic leukemia and in 13 age- and pubertal stage-matched normal children. Among the patients, the median total basal growth hormone output (AUC) was 43 units, compared with 341 units in the normal control group (P less than 0.001). In the patients, mean pulse amplitude (6.9 ng/ml) and frequency (4.6) over 24 hours also were reduced, compared with the control values (32 ng/ml and 8.5, P less than 0.001 and P less than 0.05, respectively). In addition, normal children secreted more GH at night (median AUC 280) than during the day (113, P less than 0.001). However, this diurnal pattern was absent in three of the patients studied. These data suggest that perturbations of spontaneous pulsatile GH secretion are common after standard therapy for ALL and may be a sensitive means of detecting therapy-related neuroendocrine damage. Blunting of spontaneous pulsatile GH secretion may contribute to the abnormalities in growth seen in children with ALL.

  20. Elevated Serum Polybrominated Diphenyl Ethers and Alteration of Thyroid Hormones in Children from Guiyu, China

    PubMed Central

    Xu, Xijin; Liu, Junxiao; Zeng, Xiang; Lu, Fangfang; Chen, Aimin; Huo, Xia

    2014-01-01

    Informal electronic waste (e-waste) recycling results in serious environmental pollution of polybrominated diphenyl ethers (PBDEs) and heavy metals. This study explored whether there is an association between PBDEs, heavy metal and key growth- and development-related hormones in children from Guiyu, an e-waste area in southern China. We quantified eight PBDE congeners using gas chromatographic mass spectrometry, lead and cadmium utilizing graphite furnace atomic absorption spectrometry, three thyroids with radioimmunoassay and two types of growth hormones by an enzyme-linked immune-sorbent assay (ELISA) in 162 children, 4 to 6 years old, from Guiyu. In blood, median total PBDE was 189.99 ng/g lipid. Lead and cadmium concentrations in blood averaged 14.53±4.85 µg dL−1 and 0.77±0.35 µg L−1, respectively. Spearman partial correlation analysis illustrated that lead was positively correlated with BDE153 and BDE183. Thyroid-stimulating hormone (TSH) was positively correlated with almost all PBDE congeners and negatively correlated with insulin-like growth factor binding protein-3 (IGFBP-3), whereas free triiodothyronine (FT3) and free thyroxine (FT4) were negatively correlated with BDE154. However, no correlation between the hormones and blood lead or cadmium levels was found in this study. Adjusted multiple linear regression analysis showed that total PBDEs was negatively associated with FT3 and positively associated with TSH. Notably, FT4 was positively correlated with FT3, house functions as a workshop, and father's work involved in e-waste recycling and negatively correlated with vitamin consumptions. TSH was negatively related with FT4, paternal residence time in Guiyu, working hours of mother, and child bean products intake. IGFBP-3 was positively correlated with IGF-1 and house close to an e-waste dump. These results suggest that elevated PBDEs and heavy metals related to e-waste in Guiyu may be important risk factors for hormone alterations in children

  1. Mercury exposure, nutritional deficiencies and metabolic disruptions may affect learning in children.

    PubMed

    Dufault, Renee; Schnoll, Roseanne; Lukiw, Walter J; Leblanc, Blaise; Cornett, Charles; Patrick, Lyn; Wallinga, David; Gilbert, Steven G; Crider, Raquel

    2009-10-27

    Among dietary factors, learning and behavior are influenced not only by nutrients, but also by exposure to toxic food contaminants such as mercury that can disrupt metabolic processes and alter neuronal plasticity. Neurons lacking in plasticity are a factor in neurodevelopmental disorders such as autism and mental retardation. Essential nutrients help maintain normal neuronal plasticity. Nutritional deficiencies, including deficiencies in the long chain polyunsaturated fatty acids eicosapentaenoic acid and docosahexaenoic acid, the amino acid methionine, and the trace minerals zinc and selenium, have been shown to influence neuronal function and produce defects in neuronal plasticity, as well as impact behavior in children with attention deficit hyperactivity disorder. Nutritional deficiencies and mercury exposure have been shown to alter neuronal function and increase oxidative stress among children with autism. These dietary factors may be directly related to the development of behavior disorders and learning disabilities. Mercury, either individually or in concert with other factors, may be harmful if ingested in above average amounts or by sensitive individuals. High fructose corn syrup has been shown to contain trace amounts of mercury as a result of some manufacturing processes, and its consumption can also lead to zinc loss. Consumption of certain artificial food color additives has also been shown to lead to zinc deficiency. Dietary zinc is essential for maintaining the metabolic processes required for mercury elimination. Since high fructose corn syrup and artificial food color additives are common ingredients in many foodstuffs, their consumption should be considered in those individuals with nutritional deficits such as zinc deficiency or who are allergic or sensitive to the effects of mercury or unable to effectively metabolize and eliminate it from the body.

  2. Mercury exposure, nutritional deficiencies and metabolic disruptions may affect learning in children

    PubMed Central

    Dufault, Renee; Schnoll, Roseanne; Lukiw, Walter J; LeBlanc, Blaise; Cornett, Charles; Patrick, Lyn; Wallinga, David; Gilbert, Steven G; Crider, Raquel

    2009-01-01

    Among dietary factors, learning and behavior are influenced not only by nutrients, but also by exposure to toxic food contaminants such as mercury that can disrupt metabolic processes and alter neuronal plasticity. Neurons lacking in plasticity are a factor in neurodevelopmental disorders such as autism and mental retardation. Essential nutrients help maintain normal neuronal plasticity. Nutritional deficiencies, including deficiencies in the long chain polyunsaturated fatty acids eicosapentaenoic acid and docosahexaenoic acid, the amino acid methionine, and the trace minerals zinc and selenium, have been shown to influence neuronal function and produce defects in neuronal plasticity, as well as impact behavior in children with attention deficit hyperactivity disorder. Nutritional deficiencies and mercury exposure have been shown to alter neuronal function and increase oxidative stress among children with autism. These dietary factors may be directly related to the development of behavior disorders and learning disabilities. Mercury, either individually or in concert with other factors, may be harmful if ingested in above average amounts or by sensitive individuals. High fructose corn syrup has been shown to contain trace amounts of mercury as a result of some manufacturing processes, and its consumption can also lead to zinc loss. Consumption of certain artificial food color additives has also been shown to lead to zinc deficiency. Dietary zinc is essential for maintaining the metabolic processes required for mercury elimination. Since high fructose corn syrup and artificial food color additives are common ingredients in many foodstuffs, their consumption should be considered in those individuals with nutritional deficits such as zinc deficiency or who are allergic or sensitive to the effects of mercury or unable to effectively metabolize and eliminate it from the body. PMID:19860886

  3. Prevalence of Iron Deficiency Anaemia Among School Children in Kenitra, Northwest of Morocco.

    PubMed

    Achouri, I; Aboussaleh, Y; Sbaibi, R; Ahami, A; El Hioui, M

    2015-04-01

    Iron deficiency anaemia is an important health problem in Morocco. This study was conducted to estimate the prevalence of anaemia among school children in Kenitra. The sample represents school children of all educational levels and age ranged between 6-15 years. The level of hemoglobin, haematocrit, mean corpuscular volume, mean corpuscular hemoglobin and mean corpuscular hemoglobin concentration was measured in a group of 271 school children. The seric iron was assessed and anaemia was defined when hemoglobin < 11.5 g dL(-1). A questionnaire was developed to obtain information about the daily food consumption and socio-economic conditions. The prevalence of anaemia was 16.2%. The mean hemoglobin concentration was 12.53 g dL(-1) in boys and 12.52 g dL(-1) in girls. The results suggest that iron deficiency is an important determinant of anaemia in this population. There was a significant relationship between education of the mother and anaemia in children (p = 0.004) but not with the family income. It is concluded that improving the economic status of the family, women education and health education about balanced animal and plant food consumption are recommended strategies to reduce the burden of anaemia.

  4. Knowledge, awareness, attitudes and sources of vitamin D deficiency and sufficiency in Saudi children

    PubMed Central

    Alshamsan, Fatimah M.; Bin-Abbas, Bassam S.

    2016-01-01

    Objectives: To examine the awareness, knowledge, and attitudes regarding vitamin D and its sources among Saudi Arabian children. Methods: One hundred patients (vitamin D-deficient children) and 100 healthy children participated in the study. Participants were interviewed separately on topics including their awareness, knowledge, and attitudes regarding vitamin D using multiple-choice, and short-answer questions. This prospective, cross-sectional study was performed on children and adolescents between January 2011 and December 2011 at the Security Forces Hospital, Riyadh, Saudi Arabia. Results: Only 28% were aware of the sources of vitamin D, while 64% of healthy subjects were aware. Similarly, only 50% of patients and their parents knew of the health benefits of vitamin D, while 70% of healthy subjects had knowledge. Hence, our findings agree with previous findings that low levels of awareness and knowledge regarding vitamin D and its sources persist. Conclusion: Most vitamin D deficient cases were unaware of vitamin D sources. However, half of the patient/parents were aware of benefits of vitamin D. Hence, we recommend that an improved campaign for awareness, knowledge, and attitudes regarding vitamin D and its sources are needed. PMID:27146624

  5. Thyroid hormone-regulated mouse cerebral cortex genes are differentially dependent on the source of the hormone: a study in monocarboxylate transporter-8- and deiodinase-2-deficient mice.

    PubMed

    Morte, Beatriz; Ceballos, Ainhoa; Diez, Diego; Grijota-Martínez, Carmen; Dumitrescu, Alexandra M; Di Cosmo, Caterina; Galton, Valerie Anne; Refetoff, Samuel; Bernal, Juan

    2010-05-01

    Thyroid hormones influence brain development through the control of gene expression. The concentration of the active hormone T(3) in the brain depends on T(3) transport through the blood-brain barrier, mediated in part by the monocarboxylate transporter 8 (Mct8/MCT8) and the activity of type 2 deiodinase (D2) generating T(3) from T(4). The relative roles of each of these pathways in the regulation of brain gene expression is not known. To shed light on this question, we analyzed thyroid hormone-dependent gene expression in the cerebral cortex of mice with inactivated Mct8 (Slc16a2) and Dio2 genes, alone or in combination. We used 34 target genes identified to be controlled by thyroid hormone in microarray comparisons of cerebral cortex from wild-type control and hypothyroid mice on postnatal d 21. Inactivation of the Mct8 gene (Mct8KO) was without effect on the expression of 31 of these genes. Normal gene expression in the absence of the transporter was mostly due to D2 activity because the combined disruption of Mct8 and Dio2 led to similar effects as hypothyroidism on the expression of 24 genes. Dio2 disruption alone did not affect the expression of positively regulated genes, but, as in hypothyroidism, it increased that of negatively regulated genes. We conclude that gene expression in the Mct8KO cerebral cortex is compensated in part by D2-dependent mechanisms. Intriguingly, positive or negative regulation of genes by thyroid hormone is sensitive to the source of T(3) because Dio2 inactivation selectively affects the expression of negatively regulated genes.

  6. Zinc deficiency in infants and children: a review of its complex and synergistic interactions.

    PubMed

    Krebs, Nancy F; Miller, Leland V; Hambidge, K Michael

    2014-11-01

    Zinc deficiency is estimated to contribute to over half a million deaths per year in infants and children under 5 years of age. This paper reviews the features of mild-to-moderate zinc deficiency, which include growth faltering, deficits in immune function and altered integrity and function of the gastro-intestinal tract. Sub-clinical features include oxidative stress and a pro-inflammatory state. The homeostatic response to low dietary zinc intake by increasing absorption is limited, especially if the source of zinc is of poor bioavailability, and conservation of endogenous intestinal losses is a critical component of adaptation. Owing to low zinc intakes, older breastfed infants, especially those of low birthweight, are predictably at risk of zinc deficiency if complementary food choices are unfortified and/or low in zinc. Host factors such as young age, poor intra-uterine zinc accretion owing to poor maternal status and/or prematurity, and gastro-intestinal dysfunction also potently predispose to zinc deficiency. Environmental enteropathy, which is prevalent in low-resource settings, may substantially impair zinc absorption and/or increase endogenous losses, and thus lead to relatively high zinc requirements. Emerging evidence highlights common features between chronic inflammation and zinc deficiency, and each may exacerbate the other. More investigations of zinc homeostasis in populations in low-resource settings are needed to better quantify absorption capacity and losses. Effective preventive strategies must address potentially higher zinc requirements as well as the underlying context that perpetuates a vicious cycle of zinc deficiency and multiple adverse outcomes.

  7. The effect of subclinical infantile thiamine deficiency on motor function in preschool children.

    PubMed

    Harel, Yael; Zuk, Luba; Guindy, Michal; Nakar, Orly; Lotan, Dafna; Fattal-Valevski, Aviva

    2017-01-29

    We investigated the long-term implications of infantile thiamine (vitamin B1) deficiency on motor function in preschoolers who had been fed during the first 2 years of life with a faulty milk substitute. In this retrospective cohort study, 39 children aged 5-6 years who had been exposed to a thiamine-deficient formula during infancy were compared with 30 age-matched healthy children with unremarkable infant nutritional history. The motor function of the participants was evaluated with The Movement Assessment Battery for Children (M-ABC) and the Zuk Assessment. Both evaluation tools revealed statistically significant differences between the exposed and unexposed groups for gross and fine motor development (p < .001, ball skills p = .01) and grapho-motor development (p = .004). The differences were especially noteworthy on M-ABC testing for balance control functioning (p < .001, OR 5.4; 95% CI 3.4-7.4) and fine motor skills (p < .001, OR 3.2; 95% CI 1.8-4.6). In the exposed group, both assessments concurred on the high rate of children exhibiting motor function difficulties in comparison to unexposed group (M-ABC: 56% vs. 10%, Zuk Assessment: 59% vs. 3%, p < .001). Thiamine deficiency in infancy has long-term implications on gross and fine motor function and balance skills in childhood, thiamine having a crucial role in normal motor development. The study emphasizes the importance of proper infant feeding and regulatory control of breast milk substitutes.

  8. Calcium-regulating hormones, bone mineral content, breaking load and trabecular remodeling are altered in growing pigs fed calcium-deficient diets.

    PubMed

    Eklou-Kalonji, E; Zerath, E; Colin, C; Lacroix, C; Holy, X; Denis, I; Pointillart, A

    1999-01-01

    Studies on calcium nutrition in appropriate large animal models can be directly relevant to humans. We have examined the effect of dietary Ca deficiency on various bone and bone-related variables, including plasma markers, histomorphometry, mineral content and breaking strength in pigs. Three groups of eight 38-d-old female pigs were fed adequate (0.9%; control), low (0.4%; LCa) or very low (0.1%; VLCa) Ca diets for 32 d. Plasma Ca significantly decreased over time only in the VLCa-deficient pigs. The concentrations of the parathyroid hormones (PTH) and calcitriol increased as Ca deficiency developed, and the plasma PTH and calcitriol levels varied inversely with dietary Ca. The total bone ash contents, bending moments, trabecular bone volume and the mineral apposition rate all decreased as the calcium intake decreased. The osteoclast surface areas were greater than those of controls in both Ca-deficient groups, whereas the osteoblast surface areas were greater only in the VLCa group. The plasma osteoblast-related markers (alkaline phosphatase, carboxy-terminal propeptide of type I procollagen and osteocalcin) were either greater or unaffected in the Ca-deficient pigs. The results indicate that deficient bone mineralization combined with an increased bone resorption led to bone loss and fragility. The differences in the changes in bone cells (number and activity) between LCa and VLCa groups might be due to differences (time and extent) of circulating PTH and calcitriol. The defective mineralization in both Ca-depleted groups resulted mainly from the lack of Ca because their osteoblast activity was either maintained or stimulated. The results also underline the progressive sensitivity of pigs to Ca supply and the usefulness of this model.

  9. Adult-onset deficiency in growth hormone and insulin-like growth factor-I alters oligodendrocyte turnover in the corpus callosum.

    PubMed

    Hua, Kun; Forbes, M Elizabeth; Lichtenwalner, Robin J; Sonntag, William E; Riddle, David R

    2009-08-01

    Growth hormone (GH) and insulin-like growth factor-I (IGF-I) provide trophic support during development and also appear to influence cell structure, function and replacement in the adult brain. Recent studies demonstrated effects of the GH/IGF-I axis on adult neurogenesis, but it is unclear whether the GH/IGF-I axis influences glial turnover in the normal adult brain. In the current study, we used a selective model of adult-onset GH and IGF-I deficiency to evaluate the role of GH and IGF-I in regulating glial proliferation and survival in the adult corpus callosum. GH/IGF-I-deficient dwarf rats of the Lewis strain were made GH/IGF-I replete via twice daily injections of GH starting at postnatal day 28 (P28), approximately the age at which GH pulse amplitude increases in developing rodents. GH/IGF-I deficiency was initiated in adulthood by removing animals from GH treatment. Quantitative analyses revealed that adult-onset GH/IGF-I deficiency decreased cell proliferation in the white matter and decreased the survival of newborn oligodendrocytes. These findings are consistent with the hypothesis that aging-related changes in the GH/IGF-I axis produce deficits in ongoing turnover of oligodendrocytes, which may contribute to aging-related cognitive changes and deficits in remyelination after injury.

  10. Prevalence of thalassaemia, iron-deficiency anaemia and glucose-6-phosphate dehydrogenase deficiency among Arab migrating nomad children, southern Islamic Republic of Iran.

    PubMed

    Pasalar, M; Mehrabani, D; Afrasiabi, A; Mehravar, Z; Reyhani, I; Hamidi, R; Karimi, M

    2014-12-17

    This study investigated the prevalence of iron-deficiency anaemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency and β-thalassaemia trait among Arab migrating nomad children in southern Islamic Republic of Iran. Blood samples were analysed from 134 schoolchildren aged < 18 years (51 males, 83 females). Low serum ferritin (< 12 ng/dL) was present in 17.9% of children (21.7% in females and 11.8% in males). Low haemoglobin (Hb) correlated significantly with a low serum ferritin. Only 1 child had G6PD deficiency. A total of 9.7% of children had HbA2 ≥ 3.5 g/dL, indicating β-thalassaemia trait (10.8% in females and 7.8% in males). Mean serum iron, serum ferritin and total iron binding capacity were similar in males and females. Serum ferritin index was as accurate as Hb index in the diagnosis of iron-deficiency anaemia. A high prevalence of β-thalassaemia trait was the major potential risk factor in this population.

  11. Endocrinological issues and hormonal manipulation in children and men with Klinefelter syndrome.

    PubMed

    Wosnitzer, Matthew S; Paduch, Darius A

    2013-02-15

    47, XXY or Klinefelter syndrome (KS), the most common chromosomal aberration in males, is characterized by either absolute or relative hypogonadism with frequent decline in serum testosterone (T) following the onset of puberty. Decreased T levels are the result of testicular dysfunction with decrease in size of Leydig cells, and loss of germs and Sertoli cells leading to tubular hyalinization. Increase in estradiol results from over-expression of aromatase CYP19. Deficient androgen production and observed varied response of end-organs to T leads to delayed progression of puberty with decreased facial/body hair, poor muscle development, osteoporosis, and gynecomastia. It is possible that hypogonadism and excessive estradiol production contribute to emotional and social immaturity, and specific learning disabilities in KS. Based on the authors' experience and literature review, early fertility preservation and hormonal supplementation may normalize pubertal development, prevent metabolic sequelae of hypogonadism, and have a positive effect on academic and social development. No randomized clinical trials are available studying the effects of T supplementation on reproductive or cognitive issues in KS. Aggressive T supplementation (topical gel) and selective use of aromatase inhibitors may be considered at the onset of puberty with careful follow-up and titration to reach age-specific high-normal physiologic serum values. The decision to institute hormonal therapy should be part of a multidisciplinary approach including physical, speech, behavioral, and occupational therapy. © 2013 Wiley Periodicals, Inc.

  12. Effects of levetiracetam and valproic acid monotherapy on sex-steroid hormones in prepubertal children--results from a pilot study.

    PubMed

    Rauchenzauner, Markus; Bitsche, Gabriele; Svalheim, Sigrid; Tauboll, Erik; Haberlandt, Edda; Wildt, Ludwig; Rostasy, Kevin; Luef, Gerhard

    2010-02-01

    The influence of levetiracetam (LEV) and valproic acid (VPA) monotherapy on sex-steroid hormone profile was investigated in thirty prepubertal children. VPA-treated children showed greatest androstendione concentrations when compared to LEV treated children (p=0.016) and to controls (p=0.011). All other reproductive endocrine hormones were similar among groups. In conclusion, LEV does not seem to induce changes in reproductive endocrine functions as well as clinically relevant endocrine side effects in prepubertal children.

  13. Vitamin D Deficiency in Obese Children and Its Relationship to Glucose Homeostasis

    PubMed Central

    Olson, Micah L.; Maalouf, Naim M.; Oden, Jon D.; White, Perrin C.

    2012-01-01

    Objectives: The aim of the study was to compare the prevalence of vitamin D deficiency in obese and non-overweight children in North Texas, to examine relationships between dietary habits and 25-hydroxyvitamin D [25(OH)D] level in obese children, and to examine the relationship between 25(OH)D level and markers of abnormal glucose metabolism and blood pressure. Patients and Methods: Using a cross-sectional design, systolic and diastolic blood pressure, dietary information, serum 25(OH)D, fasting glucose and insulin, 2-h glucose from oral glucose tolerance test, hemoglobin A1c, and homeostasis model assessment of insulin resistance were recorded for 411 obese subjects (6–16 yr old) at an obesity referral clinic. 25(OH)D was also obtained from 87 control non-overweight subjects (6–16 yr old). Results: Ninety-two percent of obese subjects had a 25(OH)D level below 75 nmol/liter, and 50% were below 50 nmol/liter. Among non-overweight subjects, these frequencies were 68 and 22%, respectively (both P < 0.01 compared with obese subjects). 25(OH)D was negatively associated with soda intake (P < 0.001), juice intake (P = 0.009), and skipping breakfast (P < 0.001). 25(OH)D was negatively correlated with homeostasis model assessment of insulin resistance (r = −0.19; P = 0.001) and 2-h glucose (r = −0.12; P = 0.04) after adjustment for body mass index and age but was not correlated with hemoglobin A1c, systolic blood pressure Z score, or diastolic blood pressure Z score. Conclusions: Vitamin D deficiency is common in children in this southern United States location and is significantly more prevalent in obese children. Lower 25(OH)D level is associated with risk factors for type 2 diabetes in obese children. PMID:22072738

  14. Iron Deficiency and the Cognitive and Psychomotor Development of Children: A Pilot Study with Institutionalized Children.

    ERIC Educational Resources Information Center

    Driva, A.; And Others

    1985-01-01

    Describes a pilot study, involving 48 institutionalized infants and toddlers, which aimed to treat iron deficiency anemia and to discover other factors contributing to the problem. Results indicate improvement in cognitive development after the administration of iron among three groups, while no significant differences were observed in psychomotor…

  15. Improved growth velocity of a patient with Noonan-like syndrome with loose anagen hair (NS/LAH) without growth hormone deficiency by low-dose growth hormone therapy.

    PubMed

    Takasawa, Kei; Takishima, Shigeru; Morioka, Chikako; Nishioka, Masato; Ohashi, Hirofumi; Aoki, Yoko; Shimohira, Masayuki; Kashimada, Kenichi; Morio, Tomohiro

    2015-10-01

    Noonan-like syndrome with loose anagen hair (NS/LAH; OMIM 607721) is caused by a heterozygous c.4A>G mutation in SHOC2. Most cases exhibit both growth hormone deficiency (GHD) and growth hormone insensitivity (GHI) and thus require a high dose of growth hormone (GH) therapy (e.g., 35-40 µg/kg/day). We report on a genetically diagnosed NS/LAH patient manifesting severe short stature (-3.85 SDs) with low serum level of IGF1, 30 ng/ml. The peak levels of GH stimulation tests were within the normal range, and GHI was not observed in the IGF1 generation test. However, with low-dose GH therapy (25 µg/kg/day) for two years, IGF1 level and height were remarkably improved (IGF1: 117 ng/ml, height SDs: -2.20 SDs). Further, catch-up of motor development and improvement of the proportion of extending limbs to trunk were observed (the Developmental Quotient score increased from 68 to 98 points, and the relative sitting height ratio decreased from 0.62 to 0.57). Our results suggest that endocrinological causes for short stature are variable in NS/LAH and that GH therapy should be considered as a possible treatment for delayed development in NS/LAH.

  16. Iron Deficiency and Iron Deficiency Anemia in Children With First Attack of Seizure and on Healthy Control Group: A Comparative Study

    PubMed Central

    FALLAH, Razieh; TIRANDAZI, Behnaz; FERDOSIAN, Farzad; FADAVI, Nafiseh

    2014-01-01

    Objective Seizures are the most common pediatric neurologic problem. Research of the association between iron deficiency and seizures has shown conflicting results. This study evaluates iron status of children with a first seizure attack (febrile seizure (FS) or first unprovoked afebrile seizure (FUS) and healthy control group. Materials & Methods In a cross sectional case control study, iron status of 6–60 month year old admitted children with first seizure to Shahid Sadoughi Hospital from August 2011–December 2012 were evaluated and compared with healthy control children that were referred to primary health care center of Azadshar, Yazd, Iran. Results 150 children were compared in three equal (FS, afebrile seizure, and control) groups. Hemoglobin levels in FUS (11.39 ± 1.07 g/dl) and FS (11.46 ± 1.18 g/dl) were lower than the control group (11.9 ± 0.89 g/dl) group. Serum iron levels in FS (38.52 ± 11.38 μg/dL) and FUS (42.68 ± 14.76 μg/dL) were lower than the control group (54.32 ± 13.46 μg/dL). Serum ferritin level in FUS (46.21 ± 27.63 ng/mL) and FS (48.91 ± 22.96 ng/ mL) was lower than the control group (75.13 ± 35.57 ng/mL). Iron deficiency (48% in FS, 44% in FUS and 28% in control group) and iron deficiency anemia (26% in FUS, 22% in FS, and 10% in healthy children) was more frequent in children with seizures. Conclusion Iron status should be evaluated in children with a first attack of febrile or afebrile seizures. PMID:25143769

  17. Psychological adaptation in children with idiopathic short stature treated with growth hormone or placebo.

    PubMed

    Ross, Judith L; Sandberg, David E; Rose, Susan R; Leschek, Ellen Werber; Baron, Jeffrey; Chipman, John J; Cassorla, Fernando G; Quigley, Charmian A; Crowe, Brenda J; Roberts, Kristen; Cutler, Gordon B

    2004-10-01

    The influence of short stature on psychological adaptation in childhood and adolescence is controversial. GH is currently used to treat children with idiopathic short stature (ISS, also known as non-GH-deficient short stature). This study represents the first double-blind, placebo-controlled trial of the effects of GH on the psychological adaptation of children and adolescents with ISS, treated with GH until adult height was attained.Sixty-eight children (53 males, 15 females), 9-16 yr old, with marked ISS (measured height or predicted adult height -2.5 sd or less) received either GH 0.074 mg/kg or placebo sc three times per week until height velocity decreased to less than 1.5 cm/yr. Parents completed the Child Behavior Checklist (CBCL) and children the Self-Perception Profile (SPP) and Silhouette Apperception Technique at baseline and annually thereafter. Baseline behavioral/emotional adjustment (CBCL) and self-concept (SPP) scores for children with ISS were within the normative range. The two study groups exhibited similar behavioral and self-concept profiles (CBCL) during the first 2 yr of the study. However, CBCL behavior problems (internalizing, externalizing, and total problems) appeared to decline, in yr 3 and 4, in the GH-treated group relative to the placebo-treated group. Group differences in CBCL competency domains and the SPP were not observed at any point during the study. Short stature among children with ISS enrolled in this long-term, placebo-controlled study was not associated with problems in psychological adaptation or self-concept with the psychological instruments employed. GH treatment was associated with a trend toward improvement in problem behaviors, as measured by questionnaires (CBCL) completed by study participants' parents. It remains to be determined whether GH treatment significantly impacts adaptation, psychosocial function, or quality of life in children with ISS.

  18. Severe Iron Deficiency Anemia in Infants and Young Children, Requiring Hospital Admission

    PubMed Central

    Lundblad, Kristin; Rosenberg, Jonathan; Mangurten, Henry; Angst, Denise B.

    2016-01-01

    Objective. This study evaluated patient characteristics, milk intake, and associated lab findings of children 6 months to 5 years old, admitted to a children’s hospital with severe iron-deficiency anemia (IDA). Methods. A chart review was conducted on patients admitted with microcytic anemia (hemoglobin concentration less than 7 g/dL), accompanied by a low serum ferritin and/or low serum iron level between January 2000 and December 2006. Results. A total of 18 children with severe IDA were evaluated. Many had parents with private insurance and jobs. Almost all children drank >24 ounces of milk daily. Hemoglobin on admission was 3.8 g/dL, and the mean of the patients’ mean corpuscular volume was 52.8 fL. Median iron levels were 4 µg/dL. Conclusions. Severe IDA is still prevalent in children, yet physicians may not perform necessary testing. The devastating long-term effects of severe IDA should prompt clinicians to screen for severe IDA in children regardless of absent risk factors. PMID:27335995

  19. Thyroid Hormone Levels in Obese Children and Adolescents with Non-Alcoholic Fatty Liver Disease

    PubMed Central

    Torun, Emel; Özgen, İlker Tolga; Gökçe, Selim; Aydın, Sinem; Cesur, Yaşar

    2014-01-01

    Ob­jec­ti­ve: We aimed to determine the association of thyroid functions with the components of metabolic syndrome (MS) and non-alcoholic fatty liver disease (NAFLD) in pediatric obese patients. Methods: The study included 109 obese children (aged 9-15 years) and a control group of 44 healthy age and gender-matched children of normal weight. NAFLD was diagnosed by conventional ultrasound examination. We assessed the anthropometric data and serum biochemical parameters including lipid profile, alanine aminotransferase (ALT), fasting glucose and insulin levels and thyroid stimulating hormone (TSH), free thyroxine (fT4) and free triiodothyronine (fT3) levels. The homeostasis model assessment of insulin resistance (HOMA-IR) was calculated as a measure of IR. Results: The mean age and gender distributions in the groups were similar (p=0.23). The mean body mass index (BMI) z-scores of obese children with grade 2-3 NAFLD were significantly higher than those of the obese children without hepatic steatosis (p<0.001). Mean ALT, triglyceride (TG) and LDL cholesterol increased and HDL-cholesterol significantly decreased as the hepatic steatosis increased (p<0.05). HOMA-IR levels in obese subjects with grade 2-3 NAFLD were significantly higher than those in both obese children without NAFLD and grade 1 NADFL (p=0.05 and 0.001, respectively). In the obese subjects, TSH levels were increased significantly as the degree of steatosis increased (p=0.04) but fT3 and fT4 levels were not different. In correlation analysis, TSH was significantly correlated with ALT, BMI SDS and the degree of steatosis. Conclusions: Obese children demonstrate an increase in TSH levels as the degree of steatosis increased. PMID:24637308

  20. Bone mineral density in prepubertal children with beta-thalassemia: correlation with growth and hormonal data.

    PubMed

    Soliman, A T; El Banna, N; Abdel Fattah, M; ElZalabani, M M; Ansari, B M

    1998-05-01

    Patients with beta-thalassemia major (beta-thalassemia) frequently have bone disorders of multifactorial etiology. We attempted to analyze the relationship between the bone mineral density ([BMD] measured by dual-photon absorptiometry) and auxanologic parameters, degree of siderosis, function of the growth hormone (GH)/insulin-like growth factor-I (IGF-I)/IGF-binding protein-3 (IGFBP3) axis, calcium-phosphate balance, parathyroid hormone (PTH), and cytokines (interleukin-1beta [IL-1] and tumor necrosis factor-alpha [TNF-alpha]) in 30 prepubertal children with beta-thalassemia major and 15 age-matched children with constitutional short stature (CSS), who have normal glucose tolerance and thyroid function. Children with beta-thalassemia had a significantly decreased BMD and mean BMD% for age and sex (0.75+/-0.24 g/cm2 and 71%+/-10%, respectively) versus children with CSS (1.06+/-0.3 g/cm2 and 92%+/-7%, respectively). Thalassemic patients had significantly lower circulating concentrations of IGF-I and IGFBP3 (49+/-21 ng/mL and 1.2+/-0.25 mg/L, respectively) compared with control children (153+/-42 ng/mL and 2.1+/-0.37 mg/L, respectively). The GH response to provocation by clonidine and glucagon was defective (peak GH < 7 microg/L) in 12 of the 30 thalassemic children. Serum concentrations of IL-1beta and TNF-alpha did not differ among the two study groups. Hypocalcemia was detected in five of the 30 thalassemic patients: hypoparathyroidism was diagnosed in two of the five and rickets in the other three. BMD was highly correlated with the circulating concentrations of IGF-I and IGFBP3, as well as with the auxanologic parameters (age, weight, height, height standard deviation score [HSDS], and body mass index [BMI]). It is suggested that increasing the circulating IGF-I concentration through aggressive nutritional therapy and/or GH/IGF-I therapy with supplementation with vitamin D and/or calcium might improve bone growth and mineralization and prevent the development of

  1. Growth hormone concentration and disappearance rate, insulin-like growth factors I and II and insulin levels in iron-deficient veal calves.

    PubMed

    Ceppi, A; Mullis, P E; Eggenberger, E; Blum, J W

    1994-01-01

    In calves with severe iron (Fe) deficiency, insulin-like growth factor (IGF)-I levels and IGF-I responses to exogenous growth hormone (GH) are reduced, while insulin-dependent glucose utilization is enhanced. Blood plasma concentrations of immunoreactive insulin (IRI), IGF-I, IGF-II and GH, and the half-life of blood plasma GH [after an i.v. injection of recombinant bovine GH (rbGH; 100 micrograms rbGH/kg body weight)] were measured in 20 calves at body weights between 160 and 190 kg. Calves were fed milk replacers containing 50 or 10 mg Fe/kg (groups Fe50 and Fe10, respectively). Daily weight gain and feed utilization were similar in both groups. Group Fe10 developed mild Fe deficiency anemia and blood plasma urea-nitrogen concentrations were higher (p < 0.05) than in group Fe50. IGF-I and IGF-II concentrations did not vary consistently over a 10-hour period and were not significantly influenced by different Fe intakes. The IRI concentration increased transiently (p < 0.05) after feed intake, but the total response was (not significantly) smaller in Fe-deficient calves. Plasma GH concentration changed episodically and was similar in both groups. Loss of GH from the circulation after i.v. rbGH injection, estimated by biexponential analysis, during the distribution or alpha phase (first 16 min) was similar in both groups, but during the beta phase was shorter (p < 0.05) in group Fe10 than in group Fe50 (29.9 and 34.2 min, respectively). The increased disappearance rate of GH, seen even in mild Fe deficiency, may contribute to reduced GH levels and IGF-I responses to GH in severe Fe deficiency.

  2. Iron deficiency anemia among children: Addressing a global public health problem within a Canadian context.

    PubMed

    Christofides, Anna; Schauer, Claudia; Zlotkin, Stanley H

    2005-12-01

    Despite current Canadian pre- and perinatal nutrition programs, the prevalence of both iron deficiency and iron deficiency anemia (IDA) is very high among young Aboriginal children from Canada's remote north. The major risk factors for IDA include prolonged consumption of evaporated cow's milk, chronic infection and prolonged exclusive breastfeeding. In the present article, the authors discuss IDA as a significant public health problem in Canadian Aboriginal communities. Whereas the prevalence of IDA in Canadian children is between 3.5% and 10.5% in the general population, in two Northern Ontario First Nations communities and one Inuit community, the anemia rate was 36%, with 56% having depleted iron stores. Traditional methods of preventing IDA, including targeted fortification, dietary diversification and supplementation, have not solved the problem. The authors' research group at The Hospital for Sick Children in Toronto, Ontario, conceived of the strategy of 'home fortification' with 'Sprinkles' - single-dose sachets containing micronutrients in a powder form, which are easily sprinkled onto any foods prepared in the household. In Sprinkles, the iron (ferrous fumarate) is encapsulated within a thin lipid layer to prevent the iron from interacting with food. Sprinkles have been shown to be efficacious in the treatment of anemia in many developing countries. Their use in Aboriginal communities to treat and prevent anemia is described in the present paper. The authors believe that children in Aboriginal communities across Canada would potentially benefit if Sprinkles were incorporated into Health Canada's current distribution system, in combination with a social marketing strategy to encourage their use.

  3. Prevalence of Vitamin D Deficiency and Calcium Homeostasis in Saudi Children

    PubMed Central

    Al Shaikh, Adnan M.; Abaalkhail, Bahaa; Soliman, Ashraf; Kaddam, Ibrahim; Aseri, Khalid; Al Saleh, Yousef; Al Qarni, Ali; Al Shuaibi, Ahmed; Al Tamimi, Waleed; Mukhtar, Abdel Moniem

    2016-01-01

    Objective: Vitamin D deficiency (VDD) and vitamin D insufficiency (VDI) are significant health problems all over the world. The aim of this study was to determine the prevalence of VDD and VDI in children and adolescents residing in 8 provinces in the Kingdom of Saudi Arabia and to also investigate calcium homeostasis in these subjects. Methods: A cross-sectional study was conducted in 2110 participants aged between 6 and 15 years. Information on socio-demographic status, anthropometric measurements, knowledge about vitamin D, color of the skin, dietary intake, sun exposure experience, smoking, and physical activity were collected through a questionnaire given to the parents of all subjects. The subjects were divided into three groups as vitamin D deficient, vitamin D insufficient, and vitamin sufficient according to their blood level of vitamin D [VDD ≤25 nmol/L (25 hydroxy vitamin D), VDI >25-50 nmol/L, and VDS >50 nmol/L]. Results: VDD was highly prevalent in this group of children. 95.3 of the subjects had either VDD (45.5%) or VDI (49.9%). The prevalence rate of VDD combined with VDI was higher in females (97.8%) compared to males (92.8%) (p<0.001). Only 1.6% had significant hypocalcaemia. Children with dark skin had lower concentrations of vitamin D and higher concentrations of parathormone. A positive correlation was observed between 25 hydroxy vitamin D level and serum calcium, inorganic phosphate, and alkaline phosphatase concentrations. Conclusion: The results showed a high prevalence of VDD and VDI in Saudi children with significantly higher prevalence in girls. These findings necessitate the set-up of a national program for vitamin D supplementation and health education for this vulnerable group. PMID:27476528

  4. Effects of inhaled corticosteroids and inhaled cromolyn sodium on urinary growth hormone excretion in asthmatic children.

    PubMed

    Soferman, R; Sapir, N; Spirer, Z; Golander, A

    1998-11-01

    Over the past few years there has been an increasing awareness that asthma is a chronic inflammatory airways disease. The current therapeutic strategies for treating asthma focus on suppressing the inflammatory process by using cromones or inhaled corticosteroids (ICS). The beneficial effects of ICS in asthma are now well known, but its detrimental effect on linear growth remains a controversial issue. The aim of this open label, nonrandomized, cross-sectional, one-time study was to determine the influence of these drugs on urinary growth hormone (U-GH) levels in prepubertal asthmatic children. U-GH levels were measured in 47 prepubertal asthmatic children who had been treated for at least 6 months with either ICS (beclomethasone or budesonide at a mean daily dose of 360 microg) or with 80 mg daily dose of cromolyn sodium (CrS). There were also nine healthy children who served as a control. These three groups of children were matched for age and gender ratio. The mean level of U-GH in the CrS-treated group was 2.94 +/- 0.96 ng/night; this was significantly higher compared to the mean level of the ICS-treated group (1.99 +/- 0.83 ng/night; P < 0.001) and to the mean level of the control group (1.98 +/- 0.39 ng/night; P < 0.006). There was no significant difference between the mean level of U-GH in the group treated by ICS and the controls (P < 0.9). These results show that the mean levels of U-GH secretion of the children who were treated by CrS for 6 months was significantly increased, compared to the mean U-GH level of the ICS-treated group and the controls. The mean U-GH levels in the last two groups showed no statistically significant difference.

  5. Risk factors for vitamin A deficiency among preschool aged children in Pohnpei, Federated States of Micronesia.

    PubMed

    Yamamura, Carrie M; Sullivan, Kevin M; van der Haar, Frits; Auerbach, Steven B; Iohp, Kidsen K

    2004-02-01

    In 1993, the Department of Health of the Federated States of Micronesia (FSM) conducted a population-based stratified random survey among 355 children aged 24-48 months in Pohnpei, one of the four FSM States. The objective was to determine the prevalence, and explore risk factors for vitamin A deficiency (VAD). Trained field workers collected data from a range of demographic, dietary and socioeconomic variables related to the children. The serum retinol concentration was 19.4 +/- 7.5 microg/dl (mean +/- SD), and the VAD prevalence (serum retinol <20 microg/dl) 53.1 per cent. The significant independent risk factors, determined by logistic regression, were: mother's work at home, sibling <2 years older, rural household located on the main island, early weaning, and child anemia, controlling for pipe water and electricity in the household. If compared with a reference of apparently healthy children of similar age in the USA, the distribution of serum retinol among young Pohnpei children was shifted entirely to low levels. We conclude that eliminating the pervasive VAD problem in Pohnpei would require a multi-pronged tactical approach that combines dietary improvement strategies with the ongoing supplementation effort.

  6. Sex Hormones in Allergic Conjunctivitis: Altered Levels of Circulating Androgens and Estrogens in Children and Adolescents with Vernal Keratoconjunctivitis

    PubMed Central

    Sacchetti, Marta; Lambiase, Alessandro; Moretti, Costanzo; Bonini, Stefano

    2015-01-01

    Purpose. Vernal keratoconjunctivitis (VKC) is a chronic allergic disease mainly affecting boys in prepubertal age and usually recovering after puberty. To evaluate a possible role of sex hormones in VKC, serum levels of sex hormones in children and adolescents with VKC were assessed. Methods. 12 prepubertal and 7 early pubertal boys with active VKC and 6 male patients with VKC in remission phase at late pubertal age and 48 healthy age and sex-matched subjects were included. Serum concentration of estrone, 17 beta-estradiol, dehydroepiandrosterone-sulfate, total testosterone and free testosterone, dihydrotestosterone (DHT), cortisol, delta-4-androstenedione, follicle-stimulating hormone, luteinizing hormone, and sex-hormones binding globuline (SHBG) were evaluated. Results. Serum levels of Estrone were significantly increased in all groups of patients with VKC when compared to healthy controls (P < 0.001). Prepubertal and early pubertal VKC showed a significant decrease in DHT (P = 0.007 and P = 0.028, resp.) and SHBG (P = 0.01 and P = 0.002, resp.) when compared to controls and serum levels of SHBG were increased in late pubertal VKC in remission phase (P = 0.007). Conclusions and Relevance. VKC patients have different circulating sex hormone levels in different phases of the disease and when compared to nonallergic subjects. These findings suggest a role played by sex hormones in the pathogenesis and/or activity of VKC. PMID:25756057

  7. Circadian rhythm of hormones is extinguished during prolonged physical stress, sleep and energy deficiency in young men.

    PubMed

    Opstad, K

    1994-07-01

    The circadian rhythm of hormones (N = 10) and mental performance (N = 18) was investigated in male cadets during a 5-day military training course with continuous heavy physical activities corresponding to 35% of the maximal oxygen uptake, with almost total lack of food and sleep. The 24-h means for androstenedione, dihydroepiandrosterone (DHEA), 17 alpha-hydroxyprogesterone, testosterone and thyroid-stimulating hormone decreased strongly during the course, and the circadian rhythm was extinguished below the minimum levels measured during the control experiment. The 24-h means for cortisol, dihydroepiandrosterone sulfate (DHEA-S) and progesterone increased during the course, and the circadian rhythm was abolished above the maximum levels of the control experiment. A gradual increase was found in thyroxine, free thyroxine and triiodothyronine during the first 12 h of activities, followed by a constant decrease for the rest of the course. Mental performance decreased during the course and the amplitude of its circadian rhythm increased from +/- 10% to +/- 30% of the 24-h mean. The circadian rhythms investigated were almost normalized after 4-5 days of rest. However, the nocturnal rise for cortisol, androstenedione and DHEA appeared earlier, and the plasma levels of thyroid hormones, estradiol and DHEA-S were lower during the recovery experiment than in the control experiment. The responses to stress of the circadian rhythm for mental performance and steroid hormones during the course indicate a differential regulation.

  8. Predominance of Giardia lamblia assemblage A among iron deficiency anaemic pre-school Egyptian children.

    PubMed

    Hussein, Eman M; Zaki, Wafaa M; Ahmed, Shahira A; Almatary, Amal M; Nemr, Nader I; Hussein, Abdalla M

    2016-04-01

    Intestinal parasites and nutritional deficiency can coexist and influence each other. This study aimed to clarify the association between Giardia genotypes and presence of iron deficiency anaemia (IDA) among pre-school Egyptian children. Two groups (IDA and non-anaemic) of giardiasis children (44/group) were selected according to their recovery response after treatment of giardiasis. Each group included 24 and 20 gastrointestinal symptomatic and asymptomatic, respectively. Giardia human genotypes were performed by intergenic spacer (IGS) gene based polymerase chain reaction (PCR) with high-resolution melting curve (HRM). PCR/HRM proved that Tms of assemblage A and B ranged from 79.31 ± 0.29 to 84.77 ± 0.31. In IDA patients, assemblages A and B were found among 40/44 (90.9 %) and 4/44 (9.1 %), respectively, while in non-anaemic patients, assemblages A and B were found in 10/44 (22.7 %) and 32/44 (72.7 %), respectively, beside two (4.6 %) cases had mixed infection. The difference was statistically significant. No significant relation was found between symptomatic or asymptomatic assemblages and IDA as assemblage A was found in 21/24 (87.5 %) and 19/20 (95 %) of symptomatic and asymptomatic, respectively, while 3/24 (12.5 %) and 1/20 (5 %) of assemblage B were symptomatic was asymptomatic, respectively. A significant relation was found between assemblage A subtypes distribution among IDA patients as AI and AII were detected on 23 (52.3 %) and 16 (36.4 %) of patients, respectively, while one case (2.3 %) had mixed infection. In conclusion, assemblage A is predominant among IDA giardiasis children suggesting its role in enhancing the occurrence of IDA while B has a protective role.

  9. Social Costs of Iron Deficiency Anemia in 6–59-Month-Old Children in India

    PubMed Central

    Plessow, Rafael; Arora, Narendra Kumar; Brunner, Beatrice; Tzogiou, Christina; Eichler, Klaus; Brügger, Urs; Wieser, Simon

    2015-01-01

    Introduction Inadequate nutrition has a severe impact on health in India. According to the WHO, iron deficiency is the single most important nutritional risk factor in India, accounting for more than 3% of all disability-adjusted life years (DALYs) lost. We estimate the social costs of iron deficiency anemia (IDA) in 6–59-month-old children in India in terms of intangible costs and production losses. Materials and Methods We build a health economic model estimating the life-time costs of a birth cohort suffering from IDA between the ages of 6 and 59 months. The model is stratified by 2 age groups (6–23 and 24–59-months), 2 geographical areas (urban and rural), 10 socio-economic strata and 3 degrees of severity of IDA (mild, moderate and severe). Prevalence of anemia is calculated with the last available National Family Health Survey. Information on the health consequences of IDA is extracted from the literature. Results IDA prevalence is 49.5% in 6–23-month-old and 39.9% in 24–58-month-old children. Children living in poor households in rural areas are particularly affected but prevalence is high even in wealthy urban households. The estimated yearly costs of IDA in 6–59-month-old children amount to intangible costs of 8.3 m DALYs and production losses of 24,001 m USD, equal to 1.3% of gross domestic product. Previous calculations have considerably underestimated the intangible costs of IDA as the improved WHO methodology leads to a threefold increase of DALYs due to IDA. Conclusion Despite years of iron supplementation programs and substantial economic growth, IDA remains a crucial public health issue in India and an obstacle to the economic advancement of the poor. Young children are especially vulnerable due to the irreversible effects of IDA on cognitive development. Our research may contribute to the design of new effective interventions aiming to reduce IDA in early childhood. PMID:26313356

  10. Growth hormone treatment in young children with Down's syndrome: effects on growth and psychomotor development

    PubMed Central

    Anneren, G; Tuvemo, T; Carlsson-Skwirut, C; Lonnerholm, T; Bang, P; Sara, V; Gustafsson, J

    1999-01-01

    BACKGROUND—Learning disability and short stature are cardinal signs of Down's syndrome. Insulin-like growth factor I (IGF-I), regulated by growth hormone (GH) from about 6 months of age, may be involved in brain development.
AIMS—To study long term effects of GH on linear growth and psychomotor development in young children with Down's syndrome. 
Study design—Fifteen children with Down's syndrome were treated with GH for three years from the age of 6 to 9 months (mean, 7.4). Linear growth, psychomotor development, skeletal maturation, serum concentrations of IGF-I and its binding proteins (BPs), and cerebrospinal fluid (CSF) concentrations of IGF-II were studied.
RESULTS—The mean height of the study group increased from −1.8 to −0.8 SDS (Swedish standard) during treatment, whereas that of a Down's syndrome control group fell from −1.7 to −2.2 SDS. Growth velocity declined after treatment stopped. Head growth did not accelerate during treatment. No significant difference in mental or gross motor development was found. The low concentrations of serum IGF-I and IGFBP-3 became normal during GH treatment.
CONCLUSIONS—GH treatment results in normal growth velocity in Down's syndrome but does not affect head circumference or mental or gross motor development. Growth velocity declines after treatment stops.

 PMID:10086938

  11. [Changes in the expression of receptors of steroid hormones in the development of partial androgen deficiency of aging men (PADAM)].

    PubMed

    Pecherskiĭ, A V; Semiglazov, V F; Komiakov, B K; Guliev, B G; Gorelov, A I; Novikov, A I; Pecherskiĭ, V I; Simonov, N N; Guliaev, A V; Samusenko, I A; Vonskiĭ, M S; Muttenberg, A G; Loran, O B

    2005-01-01

    This work is devoted to the vital topic of the influence of partial androgen deficiency of aging men (PADAM) on the development of cells with androgen receptors. The results obtained in this study suggest a conclusion that the production of testosterone by some tumors and tissues of the peritumorous zone, which is accompanied by increased proliferative activity and disturbance of the regulation of the cell cycle, is caused by PADAM. The given changes are directed at compensating for testicular deficiency (in particular at overcoming the androgen-dependent stage of development of androgen-sensitive cells). These changes are a partial manifestation of metabolic syndrome (X-syndrome). The atypical cells, which unavoidably develop during metabolic syndrome, are dealt with by means of the immune system, whose capabilities become less and less adequate in the given circumstances.

  12. Serum leptin levels and leptin expression in growth hormone (GH)-deficient and healthy adults: influence of GH treatment, gender, and fasting.

    PubMed

    Kristensen, K; Pedersen, S B; Fisker, S; Nørrelund, H; Rosenfalck, A M; Jørgensen, J O; Richelsen, B

    1998-12-01

    Growth hormone (GH) treatment is associated with a reduction in fat mass in healthy and GH-deficient (GHD) subjects. This is mainly mediated via a direct GH action on adipose cells and stimulation of lipolysis. Leptin is secreted from adipose tissue and may be involved in signaling information about adipose tissue stores to the brain. Hormonal regulation of leptin is still not fully elucidated, and in the present study, we investigated both the long-term (4-month) and short-term (28-hour) GH effects on serum leptin and leptin gene expression in subcutaneous adipose tissue. In GHD adults (n = 24), leptin correlated with most estimates of adiposity (r = .62 to .86), as previously found in healthy subjects. However, no correlation was observed with intraabdominal fat determined by computed tomographic (CT) scan (INTRA-CT). GH treatment for 4 months had no independent effect on either serum leptin or leptin gene expression. In a short-term study, we found that fasting gradually reduced leptin levels in both healthy men and GHD adults, with a maximum reduction of 58% to 60% (P < .01) after 31 hours. No independent effect of GH suppression or GH substitution on serum leptin was found during fasting. Adipose tissue leptin mRNA correlated with serum leptin (r = .51, P < .01) and the body mass index ([BMI] r = .55, P < .05). Serum leptin levels and gene expression were significantly higher in women compared with men (26.6 +/- 5.8 v 10.0 +/- 1.30 ng/mL, P < .05). However, in regression analysis accounting for the gender differences in subcutaneous femoral adipose tissue (FEM-CT), the difference in serum leptin disappeared, indicating that subcutaneous femoral fat or factors closely related to femoral fat (eg, sex hormones) may be causal factors for the gender difference in leptin.

  13. Gender identity of children and young adults with 5alpha-reductase deficiency.

    PubMed

    Praveen, E P; Desai, Ankush K; Khurana, M L; Philip, Jim; Eunice, Marumudi; Khadgawat, Rajesh; Kulshreshtha, Bindu; Kucheria, Kiran; Gupta, Devendra K; Seith, Ashu; Ammini, Ariachery C

    2008-02-01

    Male pseudohermaphroditism (46,XY DSD) due to 5alpha-reductase deficiency has been recognized for the last few decades. There is scant literature on this entity in India. We compiled data on five patients with this disorder. Four of our five patients were reared as females. Our assessment of these children reveals that they had male gender identity from childhood. Three of the four reared as females chose to change gender role at adolescence, while the fourth is still prepubertal. We conclude that all these patients had male gender identity from early childhood. The parents took note of this only after the appearance of male secondary sexual characteristics at puberty, thereby giving an impression of change in gender identity and gender role.

  14. Cervical shedding of herpes simplex virus in human immunodeficiency virus-infected women: effects of hormonal contraception, pregnancy, and vitamin A deficiency.

    PubMed

    Mostad, S B; Kreiss, J K; Ryncarz, A J; Mandaliya, K; Chohan, B; Ndinya-Achola, J; Bwayo, J J; Corey, L

    2000-01-01

    Genital shedding of herpes simplex virus (HSV) results in frequent transmission of infection to sexual partners and neonates. In a cross-sectional study, cervical shedding of HSV DNA was detected in 43 (17%) cervical swab samples from 273 women seropositive for HSV-1, HSV-2, and human immunodeficiency virus type 1 (HIV-1). Cervical shedding of HSV was significantly associated with oral contraception (adjusted odds ratio [aOR], 4.5; 95% confidence interval [CI], 1.7-12.2), use of depo-medroxyprogesterone acetate (aOR, 3.2; 95% CI, 1.3-7.7), and pregnancy (aOR, 7.9; 95% CI, 2.0-31.7). In the subgroup of women who were not pregnant and not using hormonal contraception (n=178), serum vitamin A was highly predictive of cervical HSV shedding: concentrations indicating severe deficiency, moderate deficiency, low-normal, and high-normal status were associated with 29%, 18%, 8%, and 2% prevalences of cervical HSV shedding, respectively (linear trend, P=.0002). Several factors appear to influence HSV reactivation in HIV-1 seropositive women.

  15. Mandibular coronoid process in parathyroid hormone-related protein-deficient mice shows ectopic cartilage formation accompanied by abnormal bone modeling.

    PubMed

    Shibata, Shunichi; Suda, Naoto; Fukada, Kenji; Ohyama, Kimie; Yamashita, Yasuo; Hammond, Vicki E

    2003-07-01

    Parathyroid hormone-related protein (PTHrP) null mutant mice were analyzed to investigate an additional role for PTHrP in cell differentiation. We found ectopic cartilage formation in the mandibular coronoid process in newborn mice. While many previous studies involving PTHrP gene knockout mouse have shown that the cartilage in various regions becomes smaller, this is the first report showing an "increase" of cartilage volume. Investigations of mandibular growth using normal mice indicated that coronoid secondary cartilage never formed from E 15 to d 4, but small amount of cartilage temporally formed at d 7, and this also applies to PTHrP-wild type mice. Therefore, PTHrP deficiency consequently advanced the secondary cartilage formation, which is a novel role of PTHrP in chondrocyte differentiation. In situ hybridization of matrix proteins showed that this coronoid cartilage had characteristics of the lower hypertrophic cell zone usually present at the site of endochondral bone formation and/or "chondroid bone" occasionally found in distraction osteogenesis. In addition, the coronoid process in the PTHrP-deficient mouse also showed abnormal expansion of bone marrow and an increase in the number of multinucleated osteoclasts, an indication of abnormal bone modeling. These results indicate that PTHrP is involved in bone modeling as well as in chondrocyte differentiation. In situ hybridization of matrix protein mRNAs in the abnormal mandibular condylar cartilage revealed that this cartilage was proportionally smaller, supporting previous immunohistochemical results.

  16. Flow cytometry analysis of OKT4 epitope deficiency in South African black children.

    PubMed Central

    Hughes, E J; Goddard, E A; Bouic, P; Beatty, D W

    1994-01-01

    A case of OKT4 epitope deficiency referred for investigation with suspected immunodeficiency is described. Flow cytometry analysis of OKT4 epitope deficiency in a study group of healthy black children showed different manifestations of the lack of OKT4 epitope; a complete lack of OKT4+CD4+ peripheral blood lymphocytes (PBL) with normal numbers of OKT4A+ and Leu3a-CD4+ PBL, decreased percentage OKT4+CD4+ compared with OKT4A+ and Leu-3a+CD4+ PBL, decreased fluorescent staining intensity with OKT4 and a biphasic OKT4 staining pattern associated with a reduced OKT4/Leu-3a ratio. The percentage and fluorescent intensity of OKT4+CD4+ PBL in the study group were significantly lower (P < 0.0001) than Leu-3a+CD4+ and OKT4A+CD4+ PBL. There is thus considerable risk of under-estimating the number of CD4+ cells in black South Africans if the OKT4 MoAb is used. PMID:7527746

  17. Anemia, micronutrient deficiencies, and malaria in children and women in Sierra Leone prior to the Ebola outbreak

    Technology Transfer Automated Retrieval System (TEKTRAN)

    To identify the factors associated with anemia and to document the severity of micronutrient deficiencies, malaria and inflammation, a nationally representative cross-sectional survey was conducted. A three-stage sampling procedure was used to randomly select children <5 years of age and adult women...

  18. A survey of children affected by ectomermal dysplasia syndromes shows an increased prevalence of atopic disorders and immune deficiency

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Ectodermal dysplasia (ED) syndromes are rare genetic disorders that affect the development of tissues derived from the embryonic ectoderm. Studies and anecdotal experience have indicated that atopic disorders (AD) and immune deficiencies (ID) may be associated with ED in children. Some ED genotypes ...

  19. Functional characterization of a human POU1F1 mutation associated with isolated growth hormone deficiency: a novel etiology for IGHD

    PubMed Central

    Sobrier, Marie-Laure; Tsai, Yu-Cheng; Pérez, Christelle; Leheup, Bruno; Bouceba, Tahar; Duquesnoy, Philippe; Copin, Bruno; Sizova, Daria; Penzo, Alfredo; Stanger, Ben Z.; Cooke, Nancy E.; Liebhaber, Stephen A.; Amselem, Serge

    2016-01-01

    POU1F1, a pituitary-specific POU-homeo domain transcription factor, plays an essential role in the specification of the somatotroph, lactotroph and thyrotroph lineages and in the activation of GH1, PRL and TSHβ transcription. Individuals with mutations in POU1F1 present with combined deficiency of GH, PRL and TSH. Here, we identified a heterozygous missense mutation with evidence of pathogenicity, at the POU1F1 locus, in a large family in which an isolated growth hormone deficiency segregates as an autosomal dominant trait. The corresponding p.Pro76Leu mutation maps to a conserved site within the POU1F1 transactivation domain. Bandshift assays revealed that the mutation alters wild-type POU1F1 binding to cognate sites within the hGH-LCR and hGH1 promoter, but not to sites within the PRL promoter, and it selectively increases binding affinity to sites within the hGH-LCR. Co-immunoprecipitation studies reveal that this substitution enhances interactions of POU1F1 with three of its cofactors, PITX1, LHX3a and ELK1, and that residue 76 plays a critical role in these interactions. The insertion of the mutation at the mouse Pou1f1 locus results in a dramatic loss of protein expression despite normal mRNA concentrations. Mice heterozygous for the p.Pro76Leu mutation were phenotypically normal while homozygotes demonstrated a dwarf phenotype. Overall, this study unveils the involvement of POU1F1 in dominantly inherited isolated GH deficiency and demonstrates a significant impact of the Pro76Leu mutation on DNA-binding activities, alterations in transactivating functions and interactions with cofactors. Our data further highlight difficulties in modeling human genetic disorders in the mouse despite apparent conservation of gene expression pathways and physiologic functions. PMID:26612202

  20. The association of concurrent vitamin D and sex hormone deficiency with bone loss and fracture risk in older men: the osteoporotic fractures in men (MrOS) study.

    PubMed

    Barrett-Connor, Elizabeth; Laughlin, Gail A; Li, Hong; Nielson, Carrie M; Wang, P Ying; Dam, Tien T; Cauley, Jane A; Ensrud, Kristine E; Stefanick, Marcia L; Lau, Edith; Hoffman, Andrew R; Orwoll, Eric S

    2012-11-01

    Low 25-hydroxyvitamin D (VitD), low sex hormones (SH), and high sex hormone binding globulin (SHBG) levels are common in older men. We tested the hypothesis that combinations of low VitD, low SH, and high SHBG would have a synergistic effect on bone mineral density (BMD), bone loss, and fracture risk in older men. Participants were a random subsample of 1468 men (mean age 74 years) from the Osteoporotic Fractures in Men Study (MrOS) plus 278 MrOS men with incident nonspine fractures studied in a case-cohort design. "Abnormal" was defined as lowest quartile for VitD (<20 ng/mL), bioavailable testosterone (BioT, <163 ng/dL), and bioavailable estradiol (BioE, <11 pg/mL); and highest quartile for SHBG (>59 nM). Overall, 10% had isolated VitD deficiency; 40% had only low SH or high SHBG; 15% had both SH/SHBG and VitD abnormality; and 35% had no abnormality. Compared to men with all normal levels, those with both SH/SHBG and VitD abnormality tended to be older, more obese, and to report less physical activity. Isolated VitD deficiency, and low BioT with or without low VitD, was not significantly related to skeletal measures. The combination of VitD deficiency with low BioE and/or high SHBG was associated with significantly lower baseline BMD and higher annualized rates of hip bone loss than SH abnormalities alone or no abnormality. Compared to men with all normal levels, the multivariate-adjusted hazard ratio (95% confidence interval [CI]) for incident nonspine fracture during 4.6-year median follow-up was 1.2 (0.8-1.8) for low VitD alone; 1.3 (0.9-1.9) for low BioE and/or high SHBG alone; and 1.6 (1.1-2.5) for low BioE/high SHBG plus low VitD. In summary, adverse skeletal effects of low sex steroid levels were more pronounced in older men with low VitD levels. The presence of low VitD in the presence of low BioE/high SHBG may contribute substantially to poor skeletal health.

  1. Growth Hormone Deficiency Is Associated with Worse Cardiac Function, Physical Performance, and Outcome in Chronic Heart Failure: Insights from the T.O.S.CA. GHD Study

    PubMed Central

    Giallauria, Francesco; Bossone, Eduardo; Isgaard, Jörgen; Marra, Alberto M.; Bobbio, Emanuele; Vriz, Olga; Åberg, David N.; Masarone, Daniele; De Paulis, Amato; Saldamarco, Lavinia; Vigorito, Carlo; Formisano, Pietro; Niola, Massimo; Perticone, Francesco; Bonaduce, Domenico; Saccà, Luigi; Colao, Annamaria; Cittadini, Antonio

    2017-01-01

    Background Although mounting evidence supports the concept that growth hormone (GH) deficiency (GHD) affects cardiovascular function, no study has systematically investigated its prevalence and role in a large cohort of chronic heart failure (CHF) patients. Aim of this study is to assess the prevalence of GHD in mild-to-moderate CHF and to explore clinical and functional correlates of GHD. Methods One-hundred thirty CHF patients underwent GH provocative test with GHRH+arginine and accordingly categorized into GH-deficiency (GHD, n = 88, age = 61.6±1.1 years, 68% men) and GH-sufficiency (GHS, n = 42, age = 63.6±1.5 years, 81% men) cohorts. Both groups received comprehensive cardiovascular examination and underwent Doppler echocardiography, cardiopulmonary exercise testing, and biochemical and hormonal assay. Results GHD was detected in roughly 30% of CHF patients. Compared to GHD, GHS patients showed smaller end-diastolic and end-systolic LV volumes (-28%, p = .008 and -24%, p = .015, respectively), lower LV end-systolic wall stress (-21%, p = .03), higher RV performance (+18% in RV area change, p = .03), lower estimated systolic pulmonary artery pressure (-11%, p = .04), higher peak VO2 (+20%, p = .001) and increased ventilatory efficiency (-12% in VE/VCO2 slope, p = .002). After adjusting for clinical covariates (age, gender, and tertiles of LV ejection fraction, IGF-1, peak VO2, VE/VCO2 slope, and NT-proBNP), logistic multivariate analysis showed that peak VO2 (β = -1.92, SE = 1.67, p = .03), VE/VCO2 slope (β = 2.23, SE = 1.20, p = .02) and NT-proBNP (β = 2.48, SE = 1.02, p = .016), were significantly associated with GHD status. Finally, compared to GHS, GHD cohort showed higher all-cause mortality at median follow-up of 3.5 years (40% vs. 25%, p < .001, respectively), independent of age, sex, NT-proBNP, peak VO2 and LVEF. Conclusions GH deficiency identifies a subgroup of CHF patients characterized by impaired functional capacity, LV remodeling and elevated

  2. The Effect of Recombinant Growth Hormone Treatment in Children with Idiopathic Short Stature and Low Insulin-Like Growth Factor-1 Levels

    PubMed Central

    Şıklar, Zeynep; Kocaay, Pınar; Çamtosun, Emine; İsakoca, Mehmet; Hacıhamdioğlu, Bülent; Savaş Erdeve, Şenay; Berberoğlu, Merih

    2015-01-01

    Objective: Idiopathic short stature (ISS) constitutes a heterogeneous group of short stature which is not associated with an endocrine or other identifiable cause. Some ISS patients may have varying degrees of insulin-like growth factor-1 (IGF-1) deficiency. Recombinant growth hormone (rGH) treatment has been used by some authors with variable results. Reports on long-term rGH treatment are limited. Methods: In this study, 21 slowly growing, non-GH-deficient ISS children who received rGH treatment for 3.62±0.92 years were evaluated at the end of a 5.42±1.67-year follow-up period. The study group included patients with low IGF-1 levels who also responded well to an IGF generation test. The patients were divided into two groups as good responders [height increment >1 standard deviation (SD)] and poor responders (height increment <1 SD) at the end of the follow-up period. Results: The height of the patients improved from -3.16±0.46 SD score (SDS) to -1.9±0.66 SDS. At the end of the follow-up period, mean height SDS was -1.72. Eleven of the patients showed a good response to treatment. Clinical parameters were essentially similar in the good responders and the poor responders groups. A female preponderance was noted in the good responders group. Conclusion: rGH treatment can safely be used in ISS children. Long-term GH treatment will ameliorate the height deficit and almost 40% of patients may reach their target height. PMID:26777041

  3. IGF-1 receptor deficiency in thyrocytes impairs thyroid hormone secretion and completely inhibits TSH-stimulated goiter.

    PubMed

    Ock, Sangmi; Ahn, Jihyun; Lee, Seok Hong; Kang, Hyun; Offermanns, Stefan; Ahn, Hwa Young; Jo, Young Suk; Shong, Minho; Cho, Bo Youn; Jo, Daewoong; Abel, E Dale; Lee, Tae Jin; Park, Woo Jin; Lee, In-Kyu; Kim, Jaetaek

    2013-12-01

    Although thyroid-stimulating hormone (TSH) is known to be a major regulator of thyroid hormone biosynthesis and thyroid growth, insulin-like growth factor 1 (IGF-1) is required for mediating thyrocyte growth in concert with TSH in vitro. We generated mice with thyrocyte-selective ablation of IGF-1 receptor (TIGF1RKO) to explore the role of IGF-1 receptor signaling on thyroid function and growth. In 5-wk-old TIGF1RKO mice, serum thyroxine (T4) concentrations were decreased by 30% in concert with a 43% down-regulation of the monocarboxylate transporter 8 (MCT8), which is involved in T4 secretion. Despite a 3.5-fold increase in circulating concentrations of TSH, thyroid architecture and size were normal. Furthermore, thyrocyte area was increased by 40% in WT thyroids after 10 d TSH injection, but this effect was absent in TSH-injected TIGF1RKO mice. WT mice treated with methimazole and sodium perchlorate for 2 or 6 wk exhibited pronounced goiter development (2.0 and 5.4-fold, respectively), but in TIGF1RKO mice, goiter development was completely abrogated. These data reveal an essential role for IGF-1 receptor signaling in the regulation of thyroid function and TSH-stimulated goitrogenesis.

  4. Growth Hormone Response to L-Dopa and Clonidine in Autistic Children.

    ERIC Educational Resources Information Center

    Realmuto, George M.; And Others

    1990-01-01

    Seven medication-free autistic subjects (ages 6-19) were administered clonidine and L-Dopa to investigate neuroendocrine responses through changes in growth hormone levels. Findings showed that, compared to normal controls, the L-Dopa-stimulated growth hormone peak was delayed and the clonidine growth hormone peak was premature. (Author/JDD)

  5. Iron and iodine deficiencies among under-2 children, adolescent girls, and pregnant women of Bangladesh: association with common diseases.

    PubMed

    Harun-Or-Rashid, M; Khatun, Uh Farida; Yoshida, Yoshitoku; Morita, Satoshi; Chowdhury, Nuruddin; Sakamoto, Junichi

    2009-02-01

    We examined the frequency of iron and iodine deficiencies and associations of iron and iodine deficiencies with common diseases among under-2 children, adolescent girls, and pregnant women of Bangladesh. We assayed the blood hemoglobin concentration in 395 under-2 children, 355 adolescent girls, and 263 pregnant women, the urinary iodine concentration of those adolescent girls and pregnant women, and the iodine level of all household salt specimens. The history of common diseases within their previous 2 weeks were also obtained from recall to explore the associations of iron and iodine deficiencies with common diseases. Anemia was found in 49.1% of children, 24.8% of adolescent girls, and 44.4% of pregnant women using defined cut-off values (Hb < 11.0 g/dL for under-2 children and pregnant women; <12.0 g/dL for adolescent girls). Prevalence of iodine deficiencies (urinary iodine <100 microg/L) was 38.4% in adolescent girls and 39.4% in pregnant women, and 39.4% of salt specimens had inadequate iodine (<15 ppm). The relative risk (RR) and 95% confidence intervals (CI) were estimated and adjusted for age, sex, and gestational age to explore the associations of iron and iodine deficiencies with common diseases. The RR of anemia was increased for fever (RR = 1.7, 95% CI = 1.3-2.3), ear infection (RR = 3.4, 95% CI = 1.3-8.5), skin disease (RR = 1.4, 95% CI = 0.9-2.2), and pneumonia (RR = 3.7, 95% CI = 0.7-19.5). The RR of iodine deficiency was elevated for diarrhea/dysentery (RR = 2.2, 95% CI = 1.1l-4.4) and eye infection (RR = 2.1, 95% CI = 0.5-9.4). We concluded that iron and iodine deficiencies are quite high among the Bangladeshi population. Observed associations of iron and iodine deficiencies with common diseases indicated the necessity of eliminating iron and iodine deficiencies from this vulnerable population through strengthening of iron and iodine supplementation, in order to prevent diseases and promote health conditions.

  6. Urinary Kidney Injury Molecules in Children with Iron-Deficiency Anemia

    PubMed Central

    Güneş, Ali; Ece, Aydın; Aktar, Fesih; Tan, İlhan; Söker, Murat; Karabel, Duran; Balık, Hasan; Uluca, Ünal; Şen, Velat; Yolbaş, İlyas

    2015-01-01

    Background The aim of this study was to investigate the urine levels of human kidney injury molecule-1 (KIM-1), neutrophil gelatinase-associated lipocalin (NGAL), N-acetyl-β-D-glucosaminidase (NAG), and liver-type fatty acid-binding protein (L-FABP) in children with iron-deficiency anemia (IDA). Material/Methods Thirty-five children with IDA and 32 matched healthy controls were recruited. We assessed complete blood count, serum iron, iron-binding capacity, ferritin, serum levels of urea, creatinine (Cr), sodium (Na), potassium (K), calcium (Ca), and glucose levels. Estimated glomerular filtration rate (eGFR) was calculated. Urinary NAG, NGAL, KIM-1, and L-FABP were measured and divided by urine creatinine for comparisons. Results There were no significant differences in serum urea, Cr, or eGFR between the IDA group and the control group (p>0.05, for all). IDA patients had significantly higher urine NGAL/Cr, L-FABP/Cr, KIM-1/Cr, and NAG/Cr compared with the control group (p<0.05). There were significant negative correlations between hemoglobin, hematocrit, red blood cell count, and urine NGAL/Cr, NAG/Cr, L-FABP/Cr, KIM-1/Cr levels (p<0.05). Conclusions Higher urinary kidney injury molecule levels in IDA patients suggest a possible subclinical renal injury in pediatric IDA patients whose renal functions and serum electrolytes were normal. PMID:26697893

  7. The effects on malaria of treatment of iron-deficiency anaemia with oral iron in Gambian children.

    PubMed

    Smith, A W; Hendrickse, R G; Harrison, C; Hayes, R J; Greenwood, B M

    1989-03-01

    In order to determine whether giving iron to iron-deficient children increases their susceptibility to malaria, 213 Gambian children aged between 6 months and 5 years with iron-deficiency anaemia were randomized to receive either oral iron or placebo during the rainy season when malaria transmission is maximal. Haematological and iron measurements improved significantly in the group given iron. Regular morbidity surveys showed that fever associated with parasitaemia occurred more frequently in the iron-treated group than in the placebo group. This difference was not significant for all parasitaemias grouped together, but became significant and progressively larger for parasitaemias of ten or more positive fields per 100 high power fields (P less than 0.025), and for parasitaemias of 50 or more positive fields per 100 high power fields (P less than 0.01). Three children in the iron-treated group but none in the placebo group had more than one episode of fever and parasitaemia. Splenomegaly rates rose appreciably during the study in both groups, but in children at age 2 years the splenomegaly rate at the end of the study was significantly greater in the iron-treated group. We concluded that there is a significantly increased risk of fever associated with severe malarial parasitaemia for children with iron-deficiency anaemia given iron during the season of maximal malaria transmission in this part of The Gambia.

  8. Hormone influence on the spatial regulation of IRT1 expression in iron-deficient Arabidopsis thaliana roots.

    PubMed

    Blum, Ailisa; Brumbarova, Tzvetina; Bauer, Petra; Ivanov, Rumen

    2014-04-10

    The IRON-REGULATED TRANSPORTER1 (IRT1) is the principal importer of soil iron in Arabidopsis thaliana. It has a complex intracellular trafficking behavior, including continuous cycling between plasma membrane and endosomes. SORTING NEXIN1 is required for the recycling of endosome-localized IRT1. In its absence, IRT1 is mistargeted for degradation, resulting in reduced plant iron-uptake efficiency. Consequently, IRT1 promoter activity gets limited to a specific portion of the root. We tested the influence of two hormones known to positively affect iron uptake on IRT1 spatial regulation. We found that ethylene treatment in wild-type background mimics the effects of the SNX-loss-of-function situation. Conversely, auxin splits the IRT1 expression zone and forces it toward the two extremities of the root. This shows that IRT1 expression along the root is modulated by ethylene-auxin interplay.

  9. Vitamin A deficiency among children--Federated States of Micronesia, 2000.

    PubMed

    2001-06-22

    Vitamin A, a fat-soluble, heat-stable nutrient (retinol) derived from animal sources and certain fruits and vegetables, forms the basic component of retinal pigments and plays a vital role in optimal health, growth, and development. Vitamin A deficiency (VAD) (serum retinol < or = 20 microg/dL [< or = 0.7 micromol/L] for subclinical VAD) can substantially increase the risk for childhood mortality from infectious and noninfectious causes. VAD impairs the mobilization and transport of iron and is usually associated with anemia and reduced growth. VAD is a major public health problem in parts of Africa, Asia, Latin America, and the Western Pacific. In Chuuk and Pohnpei, two of the four Federated States of Micronesia (FSM) (2000 population: 107,008), nutrition surveys during the early 1990s documented VAD prevalences among the highest in the world (CDC, unpublished data, 1991; U.S. Public Health Service, unpublished data, 1994). In response to these findings, FSM health authorities, with support of the United Nations Children's Fund (UNICEF), began distributing vitamin A supplements in 1993 and 1998 in Chuuk and Pohnpei, respectively. In November 1999, FSM requested assistance from CDC in VAD assessment surveys of children in Kosrae and Yap, the other two FSM states. This report summarizes levels of serum retinol and prevalence of VAD and other indicators of nutritional status among children aged 24-59 months in Kosrae and Yap. The findings indicated low serum retinol levels and high VAD prevalences but no substantial stunting or wasting. A comprehensive, long-term national strategy is needed in FSM to promote sustained improvement in vitamin A status.

  10. Red Blood Cell Distribution Width and the Platelet Count in Iron-deficient Children Aged 0.5-3 Years.

    PubMed

    Akkermans, M D; Uijterschout, L; Vloemans, J; Teunisse, P P; Hudig, F; Bubbers, S; Verbruggen, S; Veldhorst, M; de Leeuw, T G; van Goudoever, J B; Brus, F

    2015-01-01

    Early detection of iron deficiency (ID) and iron deficiency anemia (IDA) in young children is important to prevent impaired neurodevelopment. Unfortunately, many biomarkers of ID are influenced by infection, thus limiting their usefulness. The aim of this study was to investigate the value of red blood cell distribution width (RDW) and the platelet count for detecting ID(A) among otherwise healthy children. A multicenter prospective observational study was conducted in the Netherlands to investigate the prevalence of ID(A) in 400 healthy children aged 0.5-3 years. ID was defined as serum ferritin (SF) <12 μg/L in the absence of infection (C-reactive protein [CRP] <5 mg/L) and IDA as hemoglobin <110 g/L combined with ID. RDW (%) and the platelet count were determined in the complete blood cell count. RDW was inversely correlated with SF and not associated with CRP. Calculated cutoff values for RDW to detect ID and IDA gave a relatively low sensitivity (53.1% and 57.1%, respectively) and specificity (64.7% and 69.9%, respectively). Anemic children with a RDW >14.3% had a 2.7 higher odds (95% confidence interval [CI]: 1.2-6.3) to be iron deficient, compared with anemic children with a RDW <14.3%. The platelet count showed a large range in both ID and non-ID children. In conclusion, RDW can be helpful for identifying ID as the cause of anemia in 0.5- to 3-year-old children, but not as primary biomarker of ID(A). RDW values are not influenced by the presence of infection. There appears to be no role for the platelet count in diagnosing ID(A) in this group of children.

  11. Epidemiology of iodine deficiency.

    PubMed

    Vanderpump, Mark P

    2017-04-01

    Iodine is an essential component of the thyroid hormones thyroxine (T4) and triiodothyronine (T3) produced by the thyroid gland. Iodine deficiency impairs thyroid hormone production and has adverse effects throughout life, particularly early in life as it impairs cognition and growth. Iodine deficiency remains a significant problem despite major national and international efforts to increase iodine intake, primarily through the voluntary or mandatory iodization of salt. Recent epidemiological data suggest that iodine deficiency is an emerging issue in industrialized countries, previously thought of as iodine-sufficient. International efforts to control iodine deficiency are slowing, and reaching the third of the worldwide population that remains deficient poses major challenges.

  12. Vitamin A deficiency is associated with gastrointestinal and respiratory morbidity in school-age children.

    PubMed

    Thornton, Kathryn A; Mora-Plazas, Mercedes; Marín, Constanza; Villamor, Eduardo

    2014-04-01

    Infection is an important cause of morbidity throughout childhood. Poor micronutrient status is a risk factor for infection-related morbidity in young children, but it is not clear whether these associations persist during school-age years. We examined the relation between blood concentrations of micronutrient status biomarkers and risk of gastrointestinal and respiratory morbidity in a prospective study of 2774 children aged 5-12 y from public schools in Bogotá, Colombia. Retinol, zinc, ferritin, mean corpuscular volume, hemoglobin, erythrocyte folate, and vitamin B-12 concentrations were measured in blood at enrollment into the cohort. Children were followed for 1 academic year for incidence of morbidity, including diarrhea with vomiting, cough with fever, earache or ear discharge with fever, and doctor visits. Compared with adequate vitamin A status (≥30.0 μg/dL), vitamin A deficiency (<10.0 μg/dL) was associated with increased risk of diarrhea with vomiting [unadjusted incidence rate ratio (IRR): 2.17; 95% CI: 0.95, 4.96; P-trend = 0.03] and cough with fever (unadjusted IRR: 2.36; 95% CI: 1.30, 4.31; P-trend = 0.05). After adjustment for several sociodemographic characteristics and hemoglobin concentrations, every 10 μg/dL plasma retinol was associated with 18% fewer days of diarrhea with vomiting (P < 0.001), 10% fewer days of cough with fever (P < 0.001), and 6% fewer doctor visits (P = 0.01). Every 1 g/dL of hemoglobin was related to 17% fewer days with ear infection symptoms (P < 0.001) and 5% fewer doctor visits (P = 0.009) after controlling for sociodemographic factors and retinol concentrations. Zinc, ferritin, mean corpuscular volume, erythrocyte folate, and vitamin B-12 status were not associated with morbidity or doctor visits. Vitamin A and hemoglobin concentrations were inversely related to rates of morbidity in school-age children. Whether vitamin A supplementation reduces the risk or severity of infection in children over 5 y of age needs to

  13. Biological Systems of Vitamin K: A Plasma Nutriproteomics Study of Subclinical Vitamin K Deficiency in 500 Nepalese Children

    PubMed Central

    Schulze, Kerry J.; Cole, Robert N.; Wu, Lee S. F.; Yager, James D.; Groopman, John; Christian, Parul; West, Keith P.

    2016-01-01

    Abstract Vitamin K (VK) is a fat-soluble vitamin whose deficiency disrupts coagulation and may disturb bone and cardiovascular health. However, the scale and systems affected by VK deficiency in pediatric populations remains unclear. We conducted a study of the plasma proteome of 500 Nepalese children 6–8 years of age (male/female ratio = 0.99) to identify proteins associated with VK status. We measured the concentrations of plasma lipids and protein induced by VK absence-II (PIVKA-II) and correlated relative abundance of proteins quantified by mass spectrometry with PIVKA-II. VK deficiency (PIVKA-II >2 μg/L) was associated with a higher abundance of low-density lipoproteins, total cholesterol, and triglyceride concentrations (p < 0.01). Among 978 proteins observed in >10% of the children, five proteins were associated with PIVKA-II and seven proteins were differentially abundant between VK deficient versus sufficient children, including coagulation factor-II, hemoglobin, and vascular endothelial cadherin, passing a false discovery rate (FDR) threshold of 10% (q < 0.10). Among 27 proteins associated with PIVKA-II or VK deficiency at a less stringent FDR (q < 0.20), a network comprised of hemoglobin subunits and erythrocyte anti-oxidative enzymes were highly and positively correlated each other (all r > 0.7). Untargeted proteomics offers a novel systems approach to elucidating biological processes of coagulation, vascularization, and erythrocyte oxidative stress related to VK status. The results may help elucidate subclinical metabolic disturbances related to VK deficiency in populations. PMID:26913649

  14. Black bear parathyroid hormone has greater anabolic effects on trabecular bone in dystrophin-deficient mice than in wild type mice.

    PubMed

    Gray, Sarah K; McGee-Lawrence, Meghan E; Sanders, Jennifer L; Condon, Keith W; Tsai, Chung-Jui; Donahue, Seth W

    2012-09-01

    Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease that has deleterious consequences in muscle and bone, leading to decreased mobility, progressive osteoporosis, and premature death. Patients with DMD experience a higher-than-average fracture rate, particularly in the proximal and distal femur and proximal tibia. The dystrophin-deficient mdx mouse is a model of DMD that demonstrates muscle degeneration and fibrosis and osteoporosis. Parathyroid hormone, an effective anabolic agent for post-menopausal and glucocorticoid-induced osteoporosis, has not been explored for DMD. Black bear parathyroid hormone (bbPTH) has been implicated in the maintenance of bone properties during extended periods of disuse (hibernation). We cloned bbPTH and found 9 amino acid residue differences from human PTH. Apoptosis was mitigated and cAMP was activated by bbPTH in osteoblast cultures. We administered 28nmol/kg of bbPTH 1-84 to 4-week old male mdx and wild type mice via daily (5×/week) subcutaneous injection for 6 weeks. Vehicle-treated mdx mice had 44% lower trabecular bone volume fraction than wild type mice. No changes were found in femoral cortical bone geometry or mechanical properties with bbPTH treatment in wild type mice, and only medio-lateral moment of inertia changed with bbPTH treatment in mdx femurs. However, μCT analyses of the trabecular regions of the distal femur and proximal tibia showed marked increases in bone volume fraction with bbPTH treatment, with a greater anabolic response (7-fold increase) in mdx mice than wild type mice (2-fold increase). Trabecular number increased in mdx long bone, but not wild type bone. Additionally, greater osteoblast area and decreased osteoclast area were observed with bbPTH treatment in mdx mice. The heightened response to PTH in mdx bone compared to wild type suggests a link between dystrophin deficiency, altered calcium signaling, and bone. These findings support further investigation of PTH as an anabolic

  15. Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast.

    PubMed

    Haglind, C Bieneck; Nordenström, A; Ask, S; von Döbeln, U; Gustafsson, J; Stenlid, M Halldin

    2015-03-01

    Children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) have a defect in the degradation of long-chain fatty acids and are at risk of hypoketotic hypoglycemia and insufficient energy production as well as accumulation of toxic fatty acid intermediates. Knowledge on substrate metabolism in children with LCHAD deficiency during fasting is limited. Treatment guidelines differ between centers, both as far as length of fasting periods and need for night feeds are concerned. To increase the understanding of fasting intolerance and improve treatment recommendations, children with LCHAD deficiency were investigated with stable isotope technique, microdialysis, and indirect calometry, in order to assess lipolysis and glucose production during 6 h of fasting. We found an early and increased lipolysis and accumulation of long chain acylcarnitines after 4 h of fasting, albeit no patients developed hypoglycemia. The rate of glycerol production, reflecting lipolysis, averaged 7.7 ± 1.6 µmol/kg/min, which is higher compared to that of peers. The rate of glucose production was normal for age; 19.6 ± 3.4 µmol/kg/min (3.5 ± 0.6 mg/kg/min). Resting energy expenditure was also normal, even though the respiratory quotient was increased indicating mainly glucose oxidation. The results show that lipolysis and accumulation of long chain acylcarnitines occurs before hypoglycemia in fasting children with LCHAD, which may indicate more limited fasting tolerance than previously suggested.

  16. Evaluation Efficacy of Ferrous Sulfate Therapy on Headaches of 5-15 Years Old Iron Deficient Children with Migraine

    PubMed Central

    Fallah, R; Zare Bidoki, S; Ordooei, M

    2016-01-01

    Background Some researches have shown the association between iron deficiency and migraine headache in adults. The aim of present study was to evaluate efficacy of ferrous sulfate treatment on migraine headaches of 5-15 years old migraineur children with iron deficiency. Materials and Methods In a quasi- experimental study, monthly frequency, severity, duration and disability of headaches of 5-15 years old migraineur children that prophylactic therapy was indicated in them and had iron deficiency who were referred to Pediatric Neurology Clinic of Shahid Sadoughi University of Medical Sciences, Yazd, Iran between 2013 and 2015 and were treated with 2mg/kg/day topiramate plus 4mg/kg/day of ferrous sulfate for three consecutive months, were evaluated and headache characteristics before and after treatment were compared. Results In this study, 98 children with mean age of 9.72±3.19 were evaluated that 31children (31.6%) had iron deficiency. Monthly frequency (22.89±7.18 vs.14.5±4.56, P= 0.02), severity score (8.12± 1.76 vs. 5.03±1.15, P= 0.02) and disability score of headache (38.23±10.7vs. 30.12±7.46, P= 0.03) were more in children with iron deficiency. Iron therapy was effective in decreasing of monthlyfrequency 22.89± 7.18 vs. 10.13±4.51, P = 0.001), severity score (8.12±1.76 vs. 5.11±1.62, P =0.001), duration (2.14±1.23 vs.1.14±1.01, P= 0.001) and disability score of headache (38.23±10.7 vs. 22.87±8.65, P= 0.01). Conclusion In children, iron deficiency increased monthly frequency, severity and disability of migraine headache and ferrous sulfate can be used as a safe and effective drug in migraine prophylaxis. PMID:27222700

  17. [Fortification of fluid milk for the prevention and treatment of iron deficiency anemia in children under 4 years of age].

    PubMed

    Torres, M A; Lobo, N F; Sato, K; Queiroz, S de S

    1996-08-01

    The effectiveness of the use of chelate amino acid iron fortified fluid milk in the treatment of iron deficiency in children under four years of age was studied. The 269 children included in this trial received 1 liter/day of fluid milk fortified with 3 mg of chelate amino acid iron and were evaluated at six monthly intervals. At the beginning of the study 62.3% of the children presented anemia. After 6 months, this percentage had decreased to 41.8% and at the end of one year to 26.4%. The greatest decreases occurred in the groups comprising the subjects who were of 12 to 23 months of age and those under one year of age. Among the children who presented initial hemoglobin levels under 9.5 g/dl, 59.3% were free of anemia after one year of follow-up. Of those presenting initial hemoglobin levels between 9.5 and 10.9 g/dl, 66.7% recovered from their anemia. There was also greater hematological improvement in the children that ingested over 750 ml/day of fortified milk in those families that did not share the supply of supplement among their other members and in those families that had only one child under five years of age. These findings led to the conclusion that the fortification of fluid milk is a viable and effective method for the treatment of iron deficiency in pre-school children.

  18. Reversible Albumin-Binding GH Possesses a Potential Once-Weekly Treatment Profile in Adult Growth Hormone Deficiency

    PubMed Central

    Janukonyté, Jurgita; Klose, Marianne; Marina, Djordje; Tanvig, Mette; Nielsen, Lene F.; Höybye, Charlotte; Andersen, Marianne; Feldt-Rasmussen, Ulla; Christiansen, Jens Sandahl

    2016-01-01

    Context: NNC0195-0092 is a reversible, albumin-binding GH derivative, developed for once-weekly administration. Objectives: The objective of the study was to evaluate safety, local tolerability, pharmacodynamics, and pharmacokinetics of multiple, once-weekly doses of NNC0195-0092, compared with daily GH. Design and Setting: This was a phase 1, randomized, open-label, active-controlled, multiple-dose, dose-escalation trial. Patients: Thirty-four GH-treated adult subjects (male, n = 25) with GH deficiency participated in the study. Interventions and Main Outcome Measures: Subjects were sequentially assigned into four cohorts of eight subjects, randomized within each cohort (3:1) to once-weekly NNC0195-0092 (n = 6) for 4 weeks (0.02, 0.04, 0.08, and 0.12 mg/kg) or daily injections of Norditropin NordiFlex (n = 2) for 4 weeks with a dose replicating the pretrial dose of somatropin. A safety assessment was performed prior to initiating treatment at the next dose level of NNC0195-0092. Daily GH treatment was discontinued 14 days before the trial start. Blood samples were drawn for assessment of safety, pharmacokinetics, pharmacodynamics (IGF-1 and IGF-binding protein-3) profiles, and immunogenicity studies. Results: Numbers of adverse events were similar at the dose levels of 0.02, 0.04, and 0.08 mg/kg NNC0195-0092 vs daily injections of Norditropin NordiFlex, whereas the number of adverse events was greater at the highest dose level of NNC0195-0092 (0.12 mg/kg). NNC0195-0092 (area under the curve[0–168h]) and peak plasma concentration) increased in a dose-dependent manner, and a dose-dependent increase in IGF-1 levels was observed. IGF-1 profiles were elevated for at least 1 week, and for the 0.02-mg/kg and 0.04-mg/kg NNC0195-0092 doses, the observed IGF-1 levels were similar to the levels for the active control group. Conclusion: Four once-weekly doses of NNC0195-0092 (dose range 0.02–0.12 mg/kg) administered to adult patients with GH deficiency were well tolerated

  19. Evaluation of thyroid hormones in children receiving carbamazepine or valproate: a prospective study.

    PubMed

    Kafadar, İhsan; Kılıç, Betül Aydın; Arapoglu, Mujde; Yalçın, Koray; Dalgıç, Nazan

    2015-01-01

    The aim of this study was to determine the alterations in thyroid function during carbamazepine or valproate monotherapy in a prospective study. Forty patients treated with valproate, 33 patients treated with carbamazepine, and 36 control patients, all aged between 2 and 18 years, were enrolled in our study. Serum levels of thyroid hormones were measured before the beginning of the antiepileptic therapy and at 6 and 12 months of treatment. Carbamazepine-treated patients showed mean serum thyroid hormone levels significantly lower than baseline evaluation and the control group. Thyroid-stimulating hormone levels at 6 and 12 months were not significantly different in carbamazepine treated patients. Serum hormone levels did not change during valproate treatment. Thyroid-stimulating hormone levels were significantly higher at the 12th month of valproate treatment. Our data suggest that although carbamazepine causes significant alterations in thyroid hormone levels, these changes do not lead to clinical symptoms at the follow-up period of 12 months.

  20. Relationship between hyperglycemia, hormone disturbances, and clinical evolution in severely hyperglycemic post surgery critically ill children: an observational study

    PubMed Central

    2014-01-01

    Background To study hormonal changes associated with severe hyperglycemia in critically ill children and the relationship with prognosis and length of stay in intensive care. Methods Observational study in twenty-nine critically ill children with severe hyperglycemia defined as 2 blood glucose measurements greater than 180 mg/dL. Severity of illness was assessed using pediatric index of mortality (PIM2), pediatric risk of mortality (PRISM) score, and pediatric logistic organ dysfunction (PELOD) scales. Blood glucose, glycosuria, insulin, C-peptide, cortisol, corticotropin, insulinlike growth factor-1, growth hormone, thyrotropin, thyroxine, and treatment with insulin were recorded. β-cell function and insulin sensitivity and resistance were determined on the basis of the homeostatic model assessment (HOMA), using blood glucose and C-peptide levels. Results The initial blood glucose level was 249 mg/dL and fell gradually to 125 mg/dL at 72 hours. Initial β-cell function (49.2%) and insulin sensitivity (13.2%) were low. At the time of diagnosis of hyperglycemia, 50% of the patients presented insulin resistance and β-cell dysfunction, 46% presented isolated insulin resistance, and 4% isolated β-cell dysfunction. β-cell function improved rapidly but insulin resistance persisted. Initial glycemia did not correlate with any other factor, and there was no relationship between glycemia and mortality. Patients who died had higher cortisol and growth hormone levels at diagnosis. Length of stay was correlated by univariate analysis, but not by multivariate analysis, with C-peptide and glycemic control at 24 hours, insulin resistance, and severity of illness scores. Conclusions Critically ill children with severe hyperglycemia initially present decreased β-cell function and insulin sensitivity. Nonsurvivors had higher cortisol and growth hormone levels and developed hyperglycemia later than survivors. PMID:24628829

  1. Short dialyzed children respond less to growth hormone than patients prior to dialysis. German Study Group for Growth Hormone Treatment in Chronic Renal Failure.

    PubMed

    Wühl, E; Haffner, D; Nissel, R; Schaefer, F; Mehls, O

    1996-06-01

    Recombinant human growth hormone (rhGH) is a new treatment modality for short children with chronic renal failure (CRF) prior to and during dialysis. It is difficult to analyze whether dialysis patients respond less to rhGH than children with CRF on conservative treatment because they are older and often in a pubertal age range. One hundred and eight patients were treated with 28-30 IU rhGH/kg per week for at least 1 year. We analyzed the growth response to rhGH in 56 prepubertal patients aged less than 10 years at the start of rhGH treatment; 38 children with a mean age of 6.5 +/- 2.4 years were on conservative treatment (CT) and 18 patients with a mean age of 6.5 +/- 2 years on dialysis treatment (D). Mean height velocity was 4.9 +/- 2.3 cm/year in children on CT and 4.6 +/- 1.8 cm/year in children on D. During the 1st treatment year, height velocity was 9.5 +/- 3.8 cm/year in CT patients and 7.3 +/- 1.3 cm/year in D patients (P < 0.05). The change in height was +1.1 +/- 0.8 standard deviation (SD) in CT patients and +0.5 +/- 0.4 SD in D patients (P < 0.005). During the 2nd treatment year, the change in height was again greater in CT patients (0.5 +/- 0.4 SD vs. 0.2 +/- 0.4 SD; P < 0.05). The difference in height velocity and change in height standard deviation score was also significant when a subgroup of patients was matched for sex, age, height. Height velocity and the change in height velocity during rhGH treatment were not correlated with residual renal function, the degree of anemia, or metabolic acidosis. We conclude that short children on D respond less to rhGH than short children on CT, indicating a greater insensitivity to rhGH during D treatment.

  2. Growth and growth hormone: An overview.

    PubMed

    Teran, Enrique; Chesner, Jaclyn; Rapaport, Robert

    2016-06-01

    Growth is a good indicator of a child's health. Growth disturbances, including short stature or growth failure, could be indications of illnesses such as chronic disease, nutritional deficits, celiac disease or hormonal abnormalities. Therefore, a careful assessment of the various requirements for normal growth needs to be done by history, physical examination, and screening laboratory tests. More details will be reviewed about the GH-IGF axis, its abnormalities with special emphasis on GH deficiency, its diagnosis and treatment. GH treatment indications in the US will be reviewed and a few only will be highlighted. They will include GH deficiency, as well as the treatment of children born SGA, including the results of a US study using FDA approved dose of 0.48mg/kg/week. GH deficiency in adults will also be briefly reviewed. Treatment of patients with SHOX deficiency will also be discussed. Possible side effects of GH treatment and the importance of monitoring safety will be highlighted.

  3. Effect of recombinant human growth hormone on changes in height, bone mineral density, and body composition over 1-2 years in children with Hurler or Hunter syndrome.

    PubMed

    Polgreen, Lynda E; Thomas, William; Orchard, Paul J; Whitley, Chester B; Miller, Bradley S

    2014-02-01

    Patients with Hurler or Hunter syndrome typically have moderate to severe growth deficiencies despite therapy with allogeneic hematopoietic stem cell transplantation and/or enzyme replacement therapy. It is unknown whether treatment with recombinant human growth hormone (hGH) can improve growth in these children. The objectives of this study were to determine the effects of hGH on growth, bone mineral density (BMD), and body composition in children with Hurler or Hunter syndrome enrolled in a longitudinal observational study. The difference in annual change in outcomes between hGH treated and untreated subjects was estimated by longitudinal regression models that adjusted for age, Tanner stage, and sex where appropriate. We report on 23 participants who completed at least 2 annual study visits (10 [43%] treated with hGH): Hurler syndrome (n=13) average age of 9.8 ± 3.1 years (range 5.3-13.6 years; 54% female) and Hunter syndrome (n=10) average age of 12.0 ± 2.7 years (range 7.0-17.0 years; 0% female). As a group, children with Hurler or Hunter syndrome treated with hGH had no difference in annual change in height (growth velocity) compared to those untreated with hGH. Growth velocity in hGH treated individuals ranged from -0.4 to 8.1cm/year and from 0.3 to 6.6 cm/year in the untreated individuals. Among children with Hunter syndrome, 100% (N=4) of those treated but only 50% of those untreated with hGH had an annual increase in height standard deviation score (SDS). Of the individuals treated with hGH, those with GHD had a trend towards higher annualized growth velocity compared to those without GHD (6.5 ± 1.9 cm/year vs. 3.5 ± 2.1cm/year; p=.050). Children treated with hGH had greater annual gains in BMD and lean body mass. In conclusion, although as a group we found no significant difference in growth between individuals treated versus not treated with hGH, individual response was highly variable and we are unable to predict who will respond to treatment. Thus

  4. Treatment with N- and C-Terminal Peptides of Parathyroid Hormone-Related Protein Partly Compensate the Skeletal Abnormalities in IGF-I Deficient Mice

    PubMed Central

    Portal-Núñez, Sergio; Murillo-Cuesta, Silvia; Lozano, Daniel; Cediel, Rafael; Esbrit, Pedro

    2014-01-01

    Insulin-like growth factor-I (IGF-I) deficiency causes growth delay, and IGF-I has been shown to partially mediate bone anabolism by parathyroid hormone (PTH). PTH-related protein (PTHrP) is abundant in bone, and has osteogenic features by poorly defined mechanisms. We here examined the capacity of PTHrP (1–36) and PTHrP (107–111) (osteostatin) to reverse the skeletal alterations associated with IGF-I deficiency. Igf1-null mice and their wild type littermates were treated with each PTHrP peptide (80 µg/Kg/every other day/2 weeks; 2 males and 4 females for each genotype) or saline vehicle (3 males and 3 females for each genotype). We found that treatment with either PTHrP peptide ameliorated trabecular structure in the femur in both genotypes. However, these peptides were ineffective in normalizing the altered cortical structure at this bone site in Igf1-null mice. An aberrant gene expression of factors associated with osteoblast differentiation and function, namely runx2, osteoprotegerin/receptor activator of NF-κB ligand ratio, Wnt3a, cyclin D1, connexin 43, catalase and Gadd45, as well as in osteocyte sclerostin, was found in the long bones of Igf1-null mice. These mice also displayed a lower amount of trabecular osteoblasts and osteoclasts in the tibial metaphysis than those in wild type mice. These alterations in Igf1-null mice were only partially corrected by each PTHrP peptide treatment. The skeletal expression of Igf2, Igf1 receptor and Irs2 was increased in Igf1-null mice, and this compensatory profile was further improved by treatment with each PTHrP peptide related to ERK1/2 and FoxM1 activation. In vitro, PTHrP (1–36) and osteostatin were effective in promoting bone marrow stromal cell mineralization in normal mice but not in IGF-I-deficient mice. Collectively, these findings indicate that PTHrP (1–36) and osteostatin can exert several osteogenic actions even in the absence of IGF-I in the mouse bone. PMID:24503961

  5. Alterations in vitamin D metabolite, parathyroid hormone and fibroblast growth factor-23 concentrations in sclerostin-deficient mice permit the maintenance of a high bone mass.

    PubMed

    Ryan, Zachary C; Craig, Theodore A; McGee-Lawrence, Meghan; Westendorf, Jennifer J; Kumar, Rajiv

    2015-04-01

    Humans with mutations of the sclerostin (SOST) gene, and knockout animals in which the Sost gene has been experimentally deleted, exhibit an increase in bone mass. We review the mechanisms by which Sost knockout mice are able to accrete increased amounts of calcium and phosphorus required for the maintenance of a high bone mass. Recently published information from our laboratory, shows that bone mass is increased in Sost-deficient mice through an increase in osteoblast and a decrease in osteoclast activity, which is mediated by activation of β-catenin and an increase in prostacyclin synthesis in osteocytes and osteoblasts. The increases in calcium and phosphorus retention required for enhanced bone mineral accretion are brought about by changes in the vitamin D endocrine system, parathyroid hormone (PTH) and fibroblast growth factor-23 (FGF-23). Thus, in Sost knockout mice, concentrations of serum 1,25-dihydroxyvitamin D (1,25(OH)2D) are increased and concentrations of FGF-23 are decreased thereby allowing a positive calcium and phosphorus balance. Additionally, in the absence of Sost expression, urinary calcium is decreased, either through a direct effect of sclerostin on renal calcium handling, or through its effect on the synthesis of 1,25(OH)2D. Adaptations in vitamin D, PTH and FGF-23 physiology occur in the absence of sclerostin expression and mediate increased calcium and phosphorus retention required for the increase in bone mineralization. This article is part of a Special Issue entitled '17th Vitamin D Workshop'.

  6. [Index finger pollicization for congenitally deficient first finger of the hand in children].

    PubMed

    Vázquez Rueda, F; Ayala Montoro, J; Blanco López, F; Ocaña Losa, J M

    2001-10-01

    Pollicization is a single-stage neurovascular pedicle transfer of the index digit to function as a thumb. The objective of this study is to investigate the results of index finger pollicization for correction of congenital deficiency of the first ray in pediatric hand. We have done 6 pollicizations of index fingers in 6 hands (there were 2 right hands, 2 left hands, and 1 bilaterally) in 5 patients (4 boys and 1 girl) who had absent or nonfunctioning thumbs (type III-V of Blauth's classification). Associated anomalies where numerous and included radial club hand, mirror hand and cardiovascular and urologic anomalies. The average time of Kirschner wire extraction was 32 days (30 to 36 days) and to beginning the hand rehabilitation at 5 degrees to 10 degrees day. The average age at pollicization was 5.5 years (range 2 to 8 years), and follow-up averaged 8 years (5 to 11 years). The cosmetic and functional results were excellent, with manual dexterity of prehension and opposition. Pollicization in children can be performed at least 2 years of age, to due of minor risk of neurovascular lesion but without delayed the cortical representation of the pollicized finger.

  7. [Serum levels of Zn in children with different degress of nutritional deficiency].

    PubMed

    Amesty-Valbuena, Alis; Pereira-Medero, Nayda; Núñez-González, José Rafael de Jesús; García, Doris; de Villaroel, Monserrat Vicente; Granadillo, Víctor; Manzanilla, José; Fernández, Denny

    2006-12-01

    The importance of Zinc (Zn) as a necessary oligoclement for human nutrition begins in the first three decades of life. At the moment, the role that Zn plays in the infantile nutrition is very well-known, acquiring a special connotation in children with proteic-energetics malnutrition (PEM). In this study the daily ingestion and the serum measuremets of Zn were determined in 64 undernourished children (light, mild and severe) and in 25 eutrophic children with ages between 1 to 5 years, belonging to families of the strata IV and V according to the Graffar scale corrected by age. The results of the serum values of Zn were for the light undernourished of 39.73 +/- 14.97 microg/dL (30.38 microg/dL-44.56 microg/dL), for the mild undernourished of 35.07 +/- 28.13 microg/dL (27.76 microg/dL-65.80 microg/dL) and for the severe undernourished of 15.48 +/- 10.44 microg/dL (5.57 microg/dL-28.56 microg/dL), which were diminished in relation with the control group, 76.71 +/- 33.29 microg/dL (45.75 microg/dL - 78.27 microg/dL) with p < 0.0001. Equally, there were significant differences (p < 0.001) among the group of severe undernourished with the light undernourished and normal subjects. In relation with the daily ingestion of Zn, a statistically significant difference was observed only (p < 0.001) in the severe undernourished, 1.87 +/- 0.54 mg/día (1.20 mg/día-2.87 mg/día) when comparing them with the light undernourished, 5.48 +/- 0.98 mg/día (3.50 mg/día-7.87 mg/día), the mild undernourished, 4.99 +/- 1.24 mg/día (4.10 mg/día-11.42 mg/día) ) and the normal subjects, 6.22 +/- 0.98 mg/día (4.8 mg/día-8.02 mg/día). There was a positive correlation between ingestion and seric values of Zn when the 3 undernourished groups were studied. These results allow to conclude that both the seric values of Zn and its ingestion show modifications in relation with the degree of nutritional deficiency.

  8. Vitamin D Deficiency and Glycemic Status in Children and Adolescents with Type 1 Diabetes Mellitus

    PubMed Central

    Savastio, Silvia; Cadario, Francesco; Genoni, Giulia; Bellomo, Giorgio; Bagnati, Marco; Secco, Gioel; Picchi, Raffaella; Giglione, Enza; Bona, Gianni

    2016-01-01

    Background Vitamin D (25OHD) effects on glycemic control are unclear in children and adolescents with type 1 diabetes. Aims of this study were to investigate 25OHD status among children with T1DM and its relationship with insulin sensitivity and glycemic status. Subjects and Methods A cross sectional study was carried out between 2008–2014. A total of 141 patients had a T1DM >12 months diagnosis and were enrolled in the present study. Of these 35 (24.8%) were migrants and 106 (75.2%) Italians (T2). We retrospectively analyzed data at the onset of the disease (T0)(64 subjects) and 12–24 months before the last visit (T1,124 subjects). Fasting glucose, glycated hemoglobin (HbA1c), 25OHD levels and daily insulin requirement were evaluated and Cholecalciferol 1000 IU/day supplementation for the management of vitamin D insufficiency (<75 nmol/L) was systematically added. Results A generalized 25OHD insufficiency was found at each study time, particularly in migrants. At T0, the 25OHD levels were inversely related to diabetic keto-acidosis (DKA) severity (p<0.05). At T1 and T2, subjects with 25OHD ≤25nmol/L (10 ng/mL) showed higher daily insulin requirement (p<0.05) and HbA1c values (p<0.01) than others vitamin D status. The 25OHD levels were negatively related with HbA1c (p<0.001) and daily insulin dose (p<0.05) during follow up. There was a significant difference in 25OHD (p<0.01) between subjects with different metabolic control (HbA1c <7.5%,7.5–8%,>8%), both at T1 and T2. In supplemented subjects, we found a significant increase in 25OHD levels (p<0.0001) and decrease of HbA1c (p<0.001) between T1 and T2, but this was not significant in the migrants subgroup. Multivariate regression analysis showed a link between HbA1c and 25OHD levels (p<0.001). Conclusions Children with T1DM show a generalized 25OHD deficiency that impact on metabolic status and glycemic homeostasis. Vitamin D supplementation improves glycemic control and should be considered as an

  9. Depleted iron stores and iron deficiency anemia associated with reduced ferritin and hepcidin and elevated soluble transferrin receptors in a multiethnic group of preschool-age children.

    PubMed

    Weiler, Hope A; Jean-Philippe, Sonia; Cohen, Tamara R; Vanstone, Catherine A; Agellon, Sherry

    2015-09-01

    Iron deficiency anemia is prevalent in subgroups of the Canadian population. The objective of this study was to examine iron status and anemia in preschool-age children. Healthy children (n = 430, 2-5 years old, Montreal, Quebec, Canada) were sampled from randomly selected daycares. Anthropometry, demographics, and diet were assessed. Biochemistry included hemoglobin, ferritin, soluble transferrin receptors (sTfR), ferritin index, markers of inflammation (C-reactive protein, interleukin 6 (IL-6), and tumour necrosis factor alpha (TNFα)), and hepcidin. Iron deficiency and anemia cutoffs conformed to the World Health Organization criteria. Differences among categories were tested using mixed-model ANOVA or χ(2) tests. Children were 3.8 ± 1.0 years of age, with a body mass index z score of 0.48 ± 0.97, and 51% were white. Adjusted intakes of iron indicated <1% were at risk for deficiency. Hemoglobin was higher in white children, whereas ferritin was higher with greater age and female sex. Inflammatory markers and hepcidin did not vary with any demographic variable. The prevalence of iron deficiency was 16.5% (95% confidence interval (CI), 13.0-20.0). Three percent (95% CI, 1.4-4.6) of children had iron deficiency anemia and 12.8% (95% CI, 9.6-16.0) had unexplained anemia. Children with iron deficiency, with and without anemia, had lower plasma ferritin and hepcidin but higher sTfR, ferritin index, and IL-6, whereas those with unexplained anemia had elevated TNFα. We conclude that iron deficiency anemia is not very common in young children in Montreal. While iron deficiency without anemia is more common than iron deficiency with anemia, the correspondingly reduced circulating hepcidin would have enabled heightened absorption of dietary iron in support of erythropoiesis.

  10. Population-Based Screening for Selective Immunoglobulin A (IgA) Deficiency in Lithuanian Children Using a Rapid Antibody-Based Fingertip Test

    PubMed Central

    Urbonas, Vaidotas; Sadauskaite, Jolita; Cerkauskiene, Rimante; Kaminskas, Arvydas; Mäki, Markku; Kurppa, Kalle

    2016-01-01

    Background Selective immunoglobulin A (IgA) deficiency is the most common inherited immunodeficiency disorder world-wide. An early diagnosis is advocated because of the increased risk of infections, autoimmune diseases, and allergic reactions. We investigated the usefulness of a rapid point-of-care test in detecting for IgA deficiency in a population with a previously unknown prevalence. Material/Methods Altogether, 1000 children aged 11–13 years from randomly selected Lithuanian schools were enrolled. A point-of-care test with a fingertip sample was used to screen for the presence of IgA deficiency in children whose parents gave consent. Those with suspected IgA deficiency were referred to hospital for further clinical examination and confirmation of the diagnosis. In addition, their medical histories were compared with those of 30 age- and sex-matched healthy controls. Results IgA deficiency was suspected in one girl and in three boys on the basis of the rapid test, and the diagnosis was confirmed for all four cases (prevalence 0.4%, 95% confidence interval 0.16–1.02%). There was no difference in disease history or complications between IgA-deficient children and healthy controls. Conclusions The rapid antibody test is a practical and accurate method to diagnose selective IgA deficiency in children. The prevalence of IgA deficiency among Lithuanian schoolchildren is 1:250. PMID:27920422

  11. Iron Deficiency Parameters in Children and Adolescents with Attention-Deficit/Hyperactivity Disorder.

    PubMed

    Percinel, Ipek; Yazici, Kemal Utku; Ustundag, Bilal

    2016-04-01

    The aim of this study is to compare iron deficiency parameters in patients with stimulant-naive attention-deficit/hyperactivity disorder (ADHD) and healthy controls, to investigate whether there are differences among the ADHD presentations, and to evaluate the relationship between ADHD symptom severity and serum ferritin levels. In addition, ADHD-Predominantly Inattentive (ADHD-PI) patients with restrictive hyperactivity/impulsivity symptoms were evaluated as a separate group with "restrictive inattention presentation" (ADHD-Rest) and were compared with other groups. Patients with ADHD-Rest are typically defined as having six or more symptoms of inattention and fewer than three symptoms of hyperactivity/impulsivity. A total of 200 ADHD cases consisting of 100 ADHD-Combine (ADHD-C) and 100 ADHD-PI and a total of 100 healthy control cases were included in the study. The Schedule for Affective Disorders and Schizophrenia for School Age Children-Present and Lifetime Version was performed in a semi-structured interview during the diagnosis. The Turgay DSM-IV-Based Child and Adolescent Behavior Disorders Screening and Rating Scale, the Conners' Rating Scale-Revised: Long Form (Parent-Teacher) (CPRSR:L, CTRS-R:L) were used for clinical evaluation. Hemogram, serum iron, iron binding capacity and serum ferritin levels were assessed. There were no significant differences between the ADHD patients and the healthy control cases in terms of iron deficiency parameters. Further, there were no significant differences among the ADHD presentations in terms of the same parameters, nor were there any significant differences when the groups were examined after the identification of the ADHD-Rest. The CPRS-R:L Hyperactivity and the CTRS-R:L Hyperactivity scores were negatively correlated with serum ferritin level in the ADHD group. To our knowledge, our current study is the first to compare serum ferritin levels in ADHD-Rest with other presentations of ADHD, and included the largest

  12. Pharmacogenomics of insulin-like growth factor-I generation during GH treatment in children with GH deficiency or Turner syndrome

    PubMed Central

    Stevens, A; Clayton, P; Tatò, L; Yoo, H W; Rodriguez-Arnao, M D; Skorodok, J; Ambler, G R; Zignani, M; Zieschang, J; Della Corte, G; Destenaves, B; Champigneulle, A; Raelson, J; Chatelain, P

    2014-01-01

    Individual responses to growth hormone (GH) treatment are variable. Short-term generation of insulin-like growth factor-I (IGF-I) is recognized as a potential marker of sensitivity to GH treatment. This prospective, phase IV study used an integrated genomic analysis to identify markers associated with 1-month change in IGF-I (ΔIGF-I) following initiation of recombinant human (r-h)GH therapy in treatment-naïve children with GH deficiency (GHD) (n=166) or Turner syndrome (TS) (n=147). In both GHD and TS, polymorphisms in the cell-cycle regulator CDK4 were associated with 1-month ΔIGF-I (P<0.05). Baseline gene expression was also correlated with 1-month ΔIGF-I in both GHD and TS (r=0.3; P<0.01). In patients with low IGF-I responses, carriage of specific CDK4 alleles was associated with MAPK and glucocorticoid receptor signaling in GHD, and with p53 and Wnt signaling pathways in TS. Understanding the relationship between genomic markers and early changes in IGF-I may allow development of strategies to rapidly individualize r-hGH dose. PMID:23567489

  13. Iron fortification of whole wheat flour reduces iron deficiency and iron deficiency anemia and increases body iron stores in Indian school-aged children.

    PubMed

    Muthayya, Sumithra; Thankachan, Prashanth; Hirve, Siddhivinayak; Amalrajan, Vani; Thomas, Tinku; Lubree, Himangi; Agarwal, Dhiraj; Srinivasan, Krishnamachari; Hurrell, Richard F; Yajnik, Chittaranjan S; Kurpad, Anura V

    2012-11-01

    Wheat is the primary staple food for nearly one-third of the world's population. NaFeEDTA is the only iron (Fe) compound suitable for fortifying high extraction flours. We tested the hypothesis that NaFeEDTA-fortified, whole wheat flour reduces Fe deficiency (ID) and improves body Fe stores (BIS) and cognitive performance in Indian children. In a randomized, double-blind, controlled, school feeding trial, 6- to 15-y-old, Fe-depleted children (n = 401) were randomly assigned to either a daily wheat-based lunch meal fortified with 6 mg of Fe as NaFeEDTA or an otherwise identical unfortified control meal. Hemoglobin (Hb) and Fe status were measured at baseline, 3.5 mo, and 7 mo. Cognitive performance was evaluated at baseline and 7 mo in children (n = 170) at one of the study sites. After 7 mo, the prevalence of ID and ID anemia in the treatment group significantly decreased from 62 to 21% and 18 to 9%, respectively. There was a time x treatment interaction for Hb, serum ferritin, transferrin receptor, zinc protoporphyrin, and BIS (all P < 0.0001). Changes in BIS differed between the groups; it increased in the treatment group (0.04 ± 0.04 mmol/kg body weight) and decreased in the control group (-0.02 ± 0.04 mmol/kg body weight) (P < 0.0001). In sensory tests, NaFeEDTA-fortified flour could not be differentiated from unfortified flour. There were no significant differences in cognitive performance tests between the groups. NaFeEDTA-fortified wheat flour markedly improved BIS and reduced ID in Fe-depleted children. It may be recommended for wider use in national school feeding programs.

  14. Disruption of thyroid hormone homeostasis in Ugt1a-deficient Gunn rats by microsomal enzyme inducers is not due to enhanced thyroxine glucuronidation

    SciTech Connect

    Richardson, Terrilyn A.; Klaassen, Curtis D.

    2010-10-01

    Microsomal enzyme inducers (MEI) that increase UDP-glucuronosyltransferases (UGTs) are thought to increase glucuronidation of thyroxine (T{sub 4}), thus reducing serum T{sub 4}, and subsequently increasing thyroid stimulating hormone (TSH). Ugt1a1 and Ugt1a6 mediate T{sub 4} glucuronidation. Therefore, this experiment determined the involvement of Ugt1a enzymes in increased T{sub 4} glucuronidation, decreased serum T{sub 4}, and increased TSH after MEI treatment. Male Wistar and Ugt1a-deficient Wistar (Gunn) rats were fed a control diet or diet containing pregnenolone-16{alpha}-carbonitrile (PCN; 800 ppm), 3-methylcholanthrene (3-MC; 200 ppm), or Aroclor 1254 (PCB; 100 ppm) for 7 days. Serum T{sub 4}, triiodothyronine (T{sub 3}), and TSH concentrations, hepatic T{sub 4}/T{sub 3} glucuronidation, and thyroid histology and follicular cell proliferation were investigated. PCN, 3-MC, and PCB treatments decreased serum T{sub 4}, whereas serum T{sub 3} was maintained in both Gunn and Wistar rats (except for PCB treatment). TSH was increased in Wistar and Gunn rats after PCN (130 and 277%) or PCB treatment (72 and 60%). T{sub 4} glucuronidation in Wistar rats was increased after PCN (298%), 3-MC (85%), and PCB (450%), but was extremely low in Gunn rats, and unchanged after MEI. T{sub 3} glucuronidation was increased after PCN (121%) or PCB (58%) in Wistar rats, but only PCN increased T{sub 3} glucuronidation in Gunn rats (43%). PCN treatment induced thyroid morphological changes and increased follicular cell proliferation in both strains. These data demonstrate that T{sub 4} glucuronidation cannot be increased in Ugt1a-deficient Gunn rats. Thus, the decrease in serum T{sub 4}, increase in TSH, and increase in thyroid cell proliferation after MEI are not dependent on increased T{sub 4} glucuronidation, and cannot be attributed to Ugt1a enzymes.

  15. GH deficiency status combined with GH receptor polymorphism affects response to GH in children.

    PubMed

    Valsesia, Armand; Chatelain, Pierre; Stevens, Adam; Peterkova, Valentina A; Belgorosky, Alicia; Maghnie, Mohamad; Antoniazzi, Franco; Koledova, Ekaterina; Wojcik, Jerome; Farmer, Pierre; Destenaves, Benoit; Clayton, Peter

    2015-12-01

    Meta-analysis has shown a modest improvement in first-year growth response to recombinant human GH (r-hGH) for carriers of the exon 3-deleted GH receptor (GHRd3) polymorphism but with significant interstudy variability. The associations between GHRd3 and growth response to r-hGH over 3 years in relation to severity of GH deficiency (GHD) were investigated in patients from 14 countries. Treatment-naïve pre-pubertal children with GHD were enrolled from the PREDICT studies (NCT00256126 and NCT00699855), categorized by peak GH level (peak GH) during provocation test: ≤4 μg/l (severe GHD; n=45) and >4 to <10 μg/l mild GHD; n=49) and genotyped for the GHRd3 polymorphism (full length (fl/fl, fl/d3, d3/d3). Gene expression (GE) profiles were characterized at baseline. Changes in growth (height (cm) and SDS) over 3 years were measured. There was a dichotomous influence of GHRd3 polymorphism on response to r-hGH, dependent on peak GH level. GH peak level (higher vs lower) and GHRd3 (fl/fl vs d3 carriers) combined status was associated with height change over 3 years (P<0.05). GHRd3 carriers with lower peak GH had lower growth than subjects with fl/fl (median difference after 3 years -3.3 cm; -0.3 SDS). Conversely, GHRd3 carriers with higher peak GH had better growth (+2.7 cm; +0.2 SDS). Similar patterns were observed for GH-dependent biomarkers. GE profiles were significantly different between the groups, indicating that the interaction between GH status and GHRd3 carriage can be identified at a transcriptomic level. This study demonstrates that responses to r-hGH depend on the interaction between GHD severity and GHRd3 carriage.

  16. Use of combined measures from capillary blood to assess iron deficiency in rural Kenyan children.

    PubMed

    Shell-Duncan, Bettina; McDade, Thomas

    2004-02-01

    Community-based surveys of iron deficiency (ID) require simple, accurate methods that can be used in remote areas. The objective of this study was to assess iron status in rural Kenya using "field-friendly" methods for capillary blood, including an improved dried blood spot assay for transferrin receptor (TfR). A single finger stick was used to obtain capillary blood from 275 school-age children. Whole blood was applied directly to filter paper, dried, and later analyzed for TfR, as well as C-reactive protein (CRP), an acute-phase protein that serves as a general marker of inflammation. Capillary blood was also used to measure hemoglobin (Hb) concentration and the ratio of zinc protoporphyrin to heme (ZPP:H). The Hb concentration alone provides the lowest estimate of the prevalence of ID (8.0%). Because ZPP:H is reported to be elevated in the presence of inflammation, we constructed a preliminary diagnostic model based on elevated ZPP:H and normal CRP level, estimating the prevalence of ID at 25.9%. When TfR is added to a multiple criteria model (elevated ZPP:H in the absence of elevated CRP and/or elevated TfR level) the prevalence of ID is estimated to be 31.2%. This study demonstrates the diagnostic utility of combining TfR with other indexes of iron status, enabling the detection of ID in both the presence and absence of infection. Furthermore, this study is the first field application of TfR blood-spot methods, and it demonstrates their feasibility in remote field settings.

  17. Vitamin D deficiency and its predictors in a country with thirteen months of sunshine: the case of school children in central Ethiopia.

    PubMed

    Wakayo, Tolassa; Belachew, Tefera; Vatanparast, Hassan; Whiting, Susan J

    2015-01-01

    Studies examining vitamin D status among children living in sunny climates indicated that children did not receive adequate vitamin D, however, this has not been looked at among children living in Ethiopia. In this study, we determined vitamin D deficiency and its predictors among school children aged 11-18 years, examining circulating 25-hydroxy vitamin D [25(OH)D]. The school-based cross-sectional study was conducted in schools in Adama Town (n = 89) and in rural Adama (n = 85) for a total sample of 174. Students were randomly selected using multi-stage stratified sampling method from both settings. Socioeconomic status of parents and demographic, anthropometric, sun exposure status and blood 25(OH)D levels were obtained. Vitamin D deficiency, defined as circulating levels of 25(OH)D <50 nmol/L, was found in 42% of the entire study participants. Prevalence of deficiency was significantly higher among students in urban setting compared to rural (61.8% vs 21.2%, respectively, p<0.001). After controlling for potential confounders using multivariable logistic regression model, duration of exposure to sunlight, amount of body part exposed to sunlight, place of residence, maternal education, body fatness, having TV/computer at home and socioeconomic status were significant predictors of vitamin D deficiency. The findings suggest that Vitamin D deficiency was prevalent in healthy school children living both in urban and rural areas of a country with abundant year round sunshine providing UVB, with the prevalence of deficiency being significantly higher among urban school children who were less exposed to sunlight. Behaviour change communication to enhance exposure to ultraviolet light is critical to prevent vitamin D deficiency in tropical country like Ethiopia. Further study is required to assess the deleterious effect of its deficiency on bone mineral homeostasis of growing children in Ethiopia during their most critical period of bone development.

  18. Vitamin D Deficiency and Its Predictors in a Country with Thirteen Months of Sunshine: The Case of School Children in Central Ethiopia

    PubMed Central

    Wakayo, Tolassa; Belachew, Tefera; Vatanparast, Hassan; Whiting, Susan J.

    2015-01-01

    Studies examining vitamin D status among children living in sunny climates indicated that children did not receive adequate vitamin D, however, this has not been looked at among children living in Ethiopia. In this study, we determined vitamin D deficiency and its predictors among school children aged 11–18 years, examining circulating 25-hydroxy vitamin D [25(OH)D]. The school-based cross-sectional study was conducted in schools in Adama Town (n = 89) and in rural Adama (n = 85) for a total sample of 174. Students were randomly selected using multi-stage stratified sampling method from both settings. Socioeconomic status of parents and demographic, anthropometric, sun exposure status and blood 25(OH)D levels were obtained. Vitamin D deficiency, defined as circulating levels of 25(OH)D <50 nmol/L, was found in 42% of the entire study participants. Prevalence of deficiency was significantly higher among students in urban setting compared to rural (61.8% vs 21.2%, respectively, p<0.001). After controlling for potential confounders using multivariable logistic regression model, duration of exposure to sunlight, amount of body part exposed to sunlight, place of residence, maternal education, body fatness, having TV/computer at home and socioeconomic status were significant predictors of vitamin D deficiency. The findings suggest that Vitamin D deficiency was prevalent in healthy school children living both in urban and rural areas of a country with abundant year round sunshine providing UVB, with the prevalence of deficiency being significantly higher among urban school children who were less exposed to sunlight. Behaviour change communication to enhance exposure to ultraviolet light is critical to prevent vitamin D deficiency in tropical country like Ethiopia. Further study is required to assess the deleterious effect of its deficiency on bone mineral homeostasis of growing children in Ethiopia during their most critical period of bone development. PMID:25822900

  19. Exogenous hormones and other drug exposures of children with congenital heart disease.

    PubMed

    Rothman, K J; Fyler, D C; Goldblatt, A; Kreidberg, M B

    1979-04-01

    A history of oral contraceptive use, hormonal pregnancy tests, prescribed hormones and other drugs was obtained from 390 mothers of infants with congenital heart disease and 1254 mothers of normal infants in Massachusetts. The data show a small positive association between estrogen/progesterone exposure and cardiac malformation, the prevalence ratio estimate of exposed to non-exposed being 1.5 (90 per cent confidence limits are 1.0, 2.1). No association was evident, however, between hormones and trunco-conal or any other class of defect among the cases, an observation which casts doubt on a causal relationship betweem hormones and cardiovascular malformations. Several other drugs were reported more frequently by cases' mothers. These include: ampicillin; aspirin; a combined anti-nausea agent (doxylamine succinate, dicyclomine hydrochloride and pyridoxine hydrochloride); chlordiazopoxide, codeine, diazepam, diphenylhydantoin; insulin; phenobarbital; phenothiazine; phenylephrine; and tetracycline.

  20. A two year observation of the process of applying recombinant IGF-1 to treat short stature in children with primary IGF-1 deficiency -- case reports of 3 patients.

    PubMed

    Petriczko, Elżbieta; Wikiera, Beata; Horodnicka-Józwa, Anita; Marcinkiewicz, Katarzyna; Szmit-Domagalska, Justyna; Kędzia, Andrzej; Durzyńska, Julia; Broniarczyk, Justyna; Gabryelczyk, Bartosz; Noczyńska, Anna; Walczak, Mieczysław

    2011-01-01

    Growth deficiency is one of the most frequent causes of referral to Endocrinology Outpatient Clinic. IGF-1 (insulin-like growth factor 1) deficiency is one of the rarest causes of short stature. In 2009 in Poland a therapeutic programme was set up for children with severe primary IGF-1 deficiency. The authors present the data of three first polish patients qualified for the rhIGF-1 (recombinant human insulin-like growth factor 1) - mecasermin. The authors conclude that the treatment with rhIGF-1 significantly improves growth velocity in patients with IGF-1 deficiency. During two years of mecasermin treatment no serious side effects were noted.

  1. Outgrowth of BT-474 human breast cancer cells in immune-deficient mice: a new in vivo model for hormone-dependent breast cancer.

    PubMed Central

    van Slooten, H. J.; Bonsing, B. A.; Hiller, A. J.; Colbern, G. T.; van Dierendonck, J. H.; Cornelisse, C. J.; Smith, H. S.

    1995-01-01

    The effect of co-inoculation of basement membrane matrix, Matrigel and two human breast cancer cell lines, BT-474 and SK-BR-3, was tested in immune-deficient mice. Both cell lines strongly overexpress c-ErbB-2 protein, whereas only BT-474 is reported to be oestrogen receptor positive. Co-inoculation of Matrigel and BT-474 cells but not of Matrigel and SK-BR-3 cells resulted in tumour formation in bg-nu-xid mice. Oestrogen supplementation greatly enhanced tumorigenicity, but did not seem to be an absolute requirement. In vivo, BT-474 cells grow as a poorly differentiated adenocarcinoma with a doubling time of 9.4 +/- 1.1 days after inoculation into the neck region. A high proliferative activity appears to be compensated by a relatively high rate of cell loss, as BT-474 tumours contain many cells with the typical morphology of apoptotic cell death. Wild-type p53, known to participate in the induction of apoptosis, is absent from the tumours, whereas Bcl-2, known to inhibit apoptosis, is expressed at intermediate levels. BT-474 tumours tend to metastasise to the regional lymph nodes and are capable of forming micrometastatic lesions in the lung. Flow cytometrical analysis of DNA ploidy demonstrated no change in tumours compared with the cell line. Immunohistochemical and flow cytometrical detection of a number of hormone and growth factor receptors, transcription factors, cell adhesion molecules and proteins involved in proliferation and cell death demonstrated no major changes in ploidy and phenotype of tumours compared with the cell line. High expression of the cell-surface molecules c-ErbB-2 and episialin make it a potentially useful model for research in immune therapy. Images Figure 2 PMID:7599056

  2. Insulin-like growth factor-I treatment of children with Laron syndrome (primary growth hormone insensitivity).

    PubMed

    Laron, Zvi

    2008-03-01

    Laron syndrome (LS, congenital primary GH insensitivity) is caused by deletions or mutations in the GH receptor gene, resulting in an inability to generate insulin-like growth factor-I (IGF-I). If untreated, the deficiency of IGF-I results in severe dwarfism, as well as skeletal and muscular underdevelopment. The only treatment is the daily administration of recombinant IGF-I. This review summarizes the present experience by several groups worldwide. The main conclusions are: A. The one or two injections regimen result in the same growth velocity; B. The growth velocity obtained with IGF-I administration is smaller than that observed with hGH in children with congenital isolated GH deficiency; C. Overdosage of IGF-I causes a series of adverse effects which can be avoided by carefully monitoring the serum IGF-I and GH levels.

  3. Iron, Magnesium, Vitamin D, and Zinc Deficiencies in Children Presenting with Symptoms of Attention-Deficit/Hyperactivity Disorder

    PubMed Central

    Villagomez, Amelia; Ramtekkar, Ujjwal

    2014-01-01

    Attention-Deficit/Hyperactivity Disorder (ADHD) is a neurodevelopmental disorder increasing in prevalence. Although there is limited evidence to support treating ADHD with mineral/vitamin supplements, research does exist showing that patients with ADHD may have reduced levels of vitamin D, zinc, ferritin, and magnesium. These nutrients have important roles in neurologic function, including involvement in neurotransmitter synthesis. The aim of this paper is to discuss the role of each of these nutrients in the brain, the possible altered levels of these nutrients in patients with ADHD, possible reasons for a differential level in children with ADHD, and safety and effect of supplementation. With this knowledge, clinicians may choose in certain patients at high risk of deficiency, to screen for possible deficiencies of magnesium, vitamin D, zinc, and iron by checking RBC-magnesium, 25-OH vitamin D, serum/plasma zinc, and ferritin. Although children with ADHD may be more likely to have lower levels of vitamin D, zinc, magnesium, and iron, it cannot be stated that these lower levels caused ADHD. However, supplementing areas of deficiency may be a safe and justified intervention. PMID:27417479

  4. Genetics Home Reference: biotinidase deficiency

    MedlinePlus

    ... links) Children Living With Inherited Metabolic Diseases (CLIMB) (UK): Biotinidase Deficiency (PDF) Disease InfoSearch: Biotinidase Deficiency Illinois ... Group Children Living with Inherited Metabolic Diseases (CLIMB) (UK) National Organization for Rare Disorders (NORD) GeneReviews (1 ...

  5. The relationship of PTH Bst BI polymorphism, calciotropic hormone levels, and dental fluorosis of children in China.

    PubMed

    Wen, Shibao; Li, Anqi; Cui, Liuxin; Huang, Qi; Chen, Hongyang; Guo, Xiaoyi; Luo, Yixin; Hao, Qianyun; Hou, Jiaxiang; Ba, Yue

    2012-06-01

    The aim of this study was to explore the association of parathyroid hormone (PTH) gene Bst BI polymorphism, calciotropic hormone levels, and dental fluorosis of children. A case-control study was conducted in two counties (Kaifeng and Tongxu) in Henan Province, China in 2005-2006. Two hundred and twenty-five children were recruited and divided into three groups including dental fluorosis group (DFG), non-dental fluorosis group (NDFG) from high fluoride areas, and control group (CG). Urine fluoride content was determined using fluoride ion selective electrode; PTH Bst BI were genotyped using PCR-RFLP; osteocalcin (OC) and calcitonin (CT) levels in serum were detected using radioimmunoassay. Genotype distributions were BB 85.3% (58/68), Bb 14.7% (10/68) for DFG; BB 77.6% (52/67), Bb 22.4% (15/67) for NDFG; and BB 73.3% (66/90), Bb 27.7% (24/90) for CG. No significant difference of Bst BI genotypes was observed among three groups (P > 0.05). Serum OC and urine fluoride of children were both significantly higher in DFG and NDFG than in CG (P < 0.05, respectively), while a similar situation was not observed between DFG and NDFG in high fluoride areas (P > 0.05). Serum OC level of children with BB genotype was significantly higher compared to those with Bb genotype in high fluoride areas (P < 0.05). However, no significant difference of serum CT or calcium (Ca) was observed. In conclusion, there is no correlation between dental fluorosis and PTH Bst BI polymorphism. Serum OC might be a more sensitive biomarker for detecting early stages of dental fluorosis, and further studies are needed.

  6. What Causes Alpha-1 Antitrypsin Deficiency?

    MedlinePlus

    ... Causes Alpha-1 Antitrypsin Deficiency? Alpha-1 antitrypsin (AAT) deficiency is an inherited disease. "Inherited" means it's ... parents to children through genes. Children who have AAT deficiency inherit two faulty AAT genes, one from ...

  7. A genome-wide association study of thyroid stimulating hormone and free thyroxine in Danish children and adolescents

    PubMed Central

    Svendstrup, Mathilde; Ohrt, Johanne Dam; Dahl, Maria; Fonvig, Cilius Esmann; Hollensted, Mette; Have, Christian Theil; Kadarmideen, Haja N.; Pedersen, Oluf; Hansen, Torben; Holm, Jens-Christian; Grarup, Niels

    2017-01-01

    Background Hypothyroidism is associated with obesity, and thyroid hormones are involved in the regulation of body composition, including fat mass. Genome-wide association studies (GWAS) in adults have identified 19 and 6 loci associated with plasma concentrations of thyroid stimulating hormone (TSH) and free thyroxine (fT4), respectively. Objective This study aimed to identify and characterize genetic variants associated with circulating TSH and fT4 in Danish children and adolescents and to examine whether these variants associate with obesity. Methods Genome-wide association analyses of imputed genotype data with fasting plasma concentrations of TSH and fT4 from a population-based sample of Danish children, adolescents, and young adults, and a group of children, adolescents, and young adults with overweight and obesity were performed (N = 1,764, mean age = 12.0 years [range 2.5−24.7]). Replication was performed in additional comparable samples (N = 2,097, mean age = 11.8 years [1.2−22.8]). Meta-analyses, using linear additive fixed-effect models, were performed on the results of the discovery and replication analyses. Results No novel loci associated with TSH or fT4 were identified. Four loci previously associated with TSH in adults were confirmed in this study population (PDE10A (rs2983511: β = 0.112SD, p = 4.8 ∙ 10−16), FOXE1 (rs7847663: β = 0.223SD, p = 1.5 ∙ 10−20), NR3C2 (rs9968300: β = 0.194SD), p = 2.4 ∙ 10−11), VEGFA (rs2396083: β = 0.088SD, p = 2.2 ∙ 10−10)). Effect sizes of variants known to associate with TSH or fT4 in adults showed a similar direction of effect in our cohort of children and adolescents, 11 of which were associated with TSH or fT4 in our study (p<0.0002). None of the TSH or fT4 associated SNPs were associated with obesity in our cohort, indicating no pleiotropic effects of these variants on obesity. Conclusion In a group of Danish children and adolescents, four loci previously associated with plasma TSH

  8. Growth hormone and the transition from puberty into adulthood.

    PubMed

    Attanasio, Andrea F; Shalet, Stephen M

    2007-03-01

    With modern growth hormone (GH) replacement algorithms, children with a diagnosis of growth hormone deficiency achieve at the end of pediatric GH treatment an adult height that is on the average in the normal range. Recent experience with GH replacement in young adults with childhood-onset growth hormone deficiency, however, has shown that these patients present with variable degrees of somatic immaturity. As childhood GH treatment is discontinued when final height is attained, attention moves to the phase of somatic development that follows the end of longitudinal growth, called ''transition'', which had been excluded previously from consideration for either pediatric or adult GH replacement. This article reviews the changes taking place during this phase of development and their relevance for the attainment of adult body maturation. The critical role of GH in this process is described.

  9. Iron-Deficiency Anemia (For Parents)

    MedlinePlus

    ... Your 1- to 2-Year-Old Iron-Deficiency Anemia KidsHealth > For Parents > Iron-Deficiency Anemia Print A ... common nutritional deficiency in children. About Iron-Deficiency Anemia Every red blood cell in the body contains ...

  10. Iron deficiency anaemia in childhood and thyroid function.

    PubMed

    Tienboon, Prasong; Unachak, Kewalee

    2003-01-01

    Studies in animals and adults have indicated iron deficiency anaemia to be associated with altered thyroid hormone metabolism. The aim of the present study was to determine the effect of iron deficiency anaemia on the thyroid function of young children. Concentrations of thyroxine (T4) and triiodothyronine (T3), free thyroid hormones (fT4 and fT3), thyroxine binding globulin (TBG), and thyroid stimulating hormone (TSH) were measured in the basal state and in response to an intravenous bolus of thyrotropin releasing hormone (TRH) in nine children one to three years of age with iron deficiency anaemia (IDA) before and after treatment with oral iron. The results of the anaemic children were also compared to basal and stimulated concentrations of thyroid hormones, TBG, and TSH of eight iron sufficient, age-matched children. Seven of the IDA and 6 of the control children were male. The mean haemoglobin (Hb) and serum ferritin (SF) in the IDA children at baseline were 93g/L (range 81-102) and 6g/L (range 1-12) which increased to 121g/L (range 114-129) and 54g/L (range 19-175), respectively, after a mean of 2.3 months (SD 0.5) of iron therapy. In the control group, mean Hb and SF were 125g/L (range 114-130) and 51 g/L (range 24-144), respectively. The basal values of TBG and thyroid hormones of the IDA children before and after iron treatment were not different from the control children. Similarly, there was no statistical difference in the thyroid hormones in the IDA children before compared to after resolution of the anaemia. Compared to the control children, the TSH response over time to TRH, TSH area under the curve (TSHAUC), and the peak TSH value after stimulation were all lower in the IDA children both before and after resolution of anaemia, but the differences were not significant. Iron therapy and resolution of anaemia had no effect among the IDA children. The time to reach the peak TSH concentration was longer in the IDA children (P=0.08) than the control

  11. Hormonal Control of Fetal Growth.

    ERIC Educational Resources Information Center

    Cooke, Paul S.; Nicoll, Charles S.

    1983-01-01

    Summarizes recent research on hormonal control of fetal growth, presenting data obtained using a new method for studying the area. Effects of endocrine ablations and congenital deficiencies, studies of hormone/receptor levels, in-vitro techniques, hormones implicated in promoting fetal growth, problems with existing methodologies, and growth of…

  12. Differential effects of central and peripheral administration of growth hormone (GH) and insulin-like growth factor on hypothalamic GH-releasing hormone and somatostatin gene expression in GH-deficient dwarf rats.

    PubMed

    Sato, M; Frohman, L A

    1993-08-01

    The roles of GH and insulin-like growth factor-I (IGF-I) in the regulation of hypothalamic GH-releasing hormone (GRH) and somatostatin (SRIH) gene expression were investigated in the GH-deficient dwarf (dw) rat, in which endogenous feedback signals are lacking. Adult male and female dw rats were treated with GH or IGF-I by systemic (sc) administration or intracerebroventricular (icv) infusion, and hypothalamic GRH and SRIH mRNA were determined by Northern blotting and densitometric analysis. Systemic sc injection of rGH (75 micrograms every 12 h for 3 days) decreased GRH mRNA levels in both sexes. However, systemic sc injection of human IGF-I (150 micrograms every 12 h for 3 days) did not affect GRH mRNA levels in either sex despite significant stimulation of body weight gain. The use of a continuous sc infusion, which normalized serum IGF-I levels, and prolongation of the treatment period to 7 days also failed to change GRH mRNA levels. SRIH mRNA was unaffected by systemic administration of either GH or IGF-I. Continuous icv infusion of GH (1 microgram/h for 7 days) decreased GRH mRNA levels in both sexes, but did not alter SRIH mRNA levels. Continuous icv infusion of IGF-I (100 ng/h for 7 days) decreased GRH mRNA in both sexes. In contrast, SRIH mRNA levels were increased in both sexes. IGF-I decreased GRH mRNA levels at icv infusion rates of 100 and 300 ng/h and stimulated SRIH mRNA levels at infusion rates of 30 and 100 ng/h. Food intake was unaffected at these infusion rates. Changes in GRH and SRIH mRNA levels in response to systemic or central GH and IGF-I administration were similar in both sexes, except that the decrease in GRH mRNA levels produced by the icv infusion of IGF-I was greater in female than in male rats. The results provide evidence for a direct inhibitory feedback effect of GH in the central nervous system on the regulation of hypothalamic GRH gene expression that is independent of peripheral IGF-I. IGF-I feedback, in contrast, appears to

  13. Anaemia, iron deficiency and a common polymorphism of iron-regulation, TMPRSS6 rs855791, in Rwandan children.

    PubMed

    Danquah, Ina; Gahutu, Jean-Bosco; Zeile, Irene; Musemakweri, Andre; Mockenhaupt, Frank P

    2014-01-01

    Anaemia in children living in sub-Saharan Africa is common, but its causes are diverse. In 545 children below 5 years of age from rural southern Rwanda, we assessed the role of iron deficiency (ID) and of the TMPRSS6 736(V) (rs855791) allele, known to reduce iron status and haemoglobin (Hb) levels, in anaemia and Hb concentrations. Anaemia (Hb <11 g/dl) was present in 34.4% of the children and ID (ferritin <12 ng/ml) in 17.6%. The TMPRSS6 736(V) allele was uncommon (allele frequency, 0.096) and not associated with ID. In multivariate analysis, ID was positively associated with anaemia (adjusted odds ratio, 1.67) to an extent comparable with α(+) -thalassaemia, breastfeeding, inflammation and low household income, but the odds were substantially higher in Plasmodium falciparum infection (adjusted odds ratio, 10.3). These findings were verified in a multivariate analysis of Hb concentrations. The TMPRSS6 736(V) allele only tended to be associated with low Hb levels. TMPRSS6 736(V) is comparatively rare among Rwandan children and may only slightly contribute to low Hb concentrations. Preventable causes of anaemia, notably ID and P. falciparum infection, largely outweigh its impact and need to be addressed to improve the haematological status of children in the study area.

  14. Serum lipoprotein pattern as modified in G6PD-deficient children during haemolytic anaemia induced by fava bean ingestion.

    PubMed

    Dessì, S; Batetta, B; Spano, O; Pulisci, D; Mulas, M F; Muntoni, S; Armeni, M; Sanna, C; Antonucci, R; Pani, P

    1992-04-01

    In the present study, plasma lipid concentrations were determined at different times after admission in sera from G6PD-deficient children during haemolytic crisis induced by fava bean ingestion. Reductions in total, LDL and HDL cholesterol were found in association with the maximum of bone marrow hyperplasia. A return towards normal values occurred with regression of the disease. No changes in other lipid parameters were observed. These data suggest that alterations of lipoprotein pattern, other than in experimental animals, are also present in humans with non-malignant proliferative processes. These changes appear to be a consequence of the disease, probably due to an increased utilization of cholesterol by proliferating cells.

  15. Adult-onset deficiency in growth hormone and insulin-like growth factor-I decreases survival of dentate granule neurons: insights into the regulation of adult hippocampal neurogenesis.

    PubMed

    Lichtenwalner, Robin J; Forbes, M Elizabeth; Sonntag, William E; Riddle, David R

    2006-02-01

    Insulin-like growth factor-I (IGF-I), long thought to provide critical trophic support during development, also has emerged as a candidate for regulating ongoing neuronal production in adulthood. Whether and how IGF-I influences each phase of neurogenesis, however, remains unclear. In the current study, we used a selective model of growth hormone (GH) and plasma IGF-I deficiency to evaluate the role of GH and IGF-I in regulating cell proliferation, survival, and neuronal differentiation in the adult dentate gyrus. GH/IGF-I-deficient dwarf rats of the Lewis strain were made GH/IGF-I replete throughout development via twice daily injections of GH, and then GH/IGF-I deficiency was initiated in adulthood by removing animals from GH treatment. Bromodeoxyuridine (BrdU) labeling revealed no effect of GH/IGF-I deficiency on cell proliferation, but adult-onset depletion of GH and plasma IGF-I significantly reduced the survival of newly generated cells in the dentate gyrus. Colabeling for BrdU and markers of immature and mature neurons revealed a selective effect of GH/IGF-I deficiency on the survival of more mature new neurons. The number of BrdU-labeled cells expressing the immature neuronal marker TUC-4 did not differ between GH/IGF-I-deficient and -replete animals, but the number expressing only the marker of maturity NeuN was lower in depleted animals. Taken together, results from the present study suggest that, under conditions of short-term GH/IGF-I deficiency during adulthood, dentate granule cells continue to be produced, to commit to a neuronal fate, and to begin the process of neuronal maturation, whereas survival of the new neurons is impaired.

  16. Influence of alimentary zinc deficiency on the concentration of growth hormone (GH), insulin-like growth factor I (IGF-I) and insulin in the serum of force-fed rats.

    PubMed

    Roth, H P; Kirchgessner, M

    1994-09-01

    The study described here investigates the influence of a specific alimentary Zn deficiency on the concentration of growth hormone (GH), insulin-like growth factor I (IGF-I) and insulin in the serum of force-fed rats. For this purpose 24 male Sprague-Dawley rats with an average bodyweight of 108 g were divided into 2 groups of 12 animals each. The Zn-deficient group and the control group received for 12 days a semi-synthetic casein diet with a Zn content of 1.3 and 25 ppm respectively. In order to prevent the reduced feed intake which occurs in Zn deficiency and the associated energy and protein shortage from interfering with the experimental parameters, all animals were fed 4 times daily by gastric tube. This made it possible to supply all animals with adequate-nutrients and to synchronise the feed intake exactly. After 12 days the depleted rats were in a severe state of Zn deficiency, as demonstrated by the reduction of Zn in the serum and the femur by 62% and 44% respectively and the 70% lower serum activity of alkaline phosphatase. In the Zn-deficient rats the concentration of GH in the serum was significantly increased by 78%, while IGF-1 and insulin were significantly reduced by 28% and 25% respectively. It is thought that the growth depression observed in the Zn-deficient rats in this study despite their identical feed intake is probably due to a reduced concentration of IGF-I and insulin and that the biological activity or the binding of GH to receptors is impaired in specific alimentary Zn deficiency.

  17. Hormonal Correlates of Clozapine-Induced Weight Gain in Psychotic Children: An Exploratory Study

    ERIC Educational Resources Information Center

    Sporn, Alexandra L.; Bobb, Aaron J.; Gogtay, Nitin; Stevens, Hanna; Greenstein, Deanna K.; Clasen, Liv S.; Tossell, Julia W.; Nugent, Thomas; Gochman, Peter A.; Sharp, Wendy S.; Mattai, Anand; Lenane, Marge C.; Yanovski, Jack A.; Rapoport, Judith L.

    2005-01-01

    Objective: Weight gain is a serious side effect of atypical antipsychotics, especially in childhood. In this study, the authors examined six weight gain-related hormones in patients with childhood-onset schizophrenia (COS) after 6 weeks of clozapine treatment. Method: Fasting serum samples for 24 patients with COS and 21 matched healthy controls…

  18. Iodine, copper, zinc, selenium and molybdenum levels in children aged between 6 and 12 years in the rural area with iodine deficiency and in the city center without iodine deficiency in Hatay

    PubMed Central

    Çelik, Tanju; Savaş, Nazan; Kurtoğlu, Selim; Sangün, Özlem; Aydın, Zeki; Mustafa, Didin; Öztürk, Oktay Hasan; Mısırlıoğlu, Seher; Öktem, Murat

    2014-01-01

    Aim: Thyroid diseases related with iodine deficiency are observed commonly in our country and in the world. In this study, we aimed to investigate iodine deficiency in urine and selenium, zinc, copper or molybdenum deficiency which may accompany this in children aged between 6 and 12 years in two schools in the province of Hatay (endemic goitre region). Material and Methods: This study is a case-control field-study in which students aged between 6 and 12 years were included. One hundred fourteen subjects from the village of Tanışma related to the center of our province and 100 subjects from the city center of Hatay (Antakya) were included in the study. Iodine, selenium, zinc, copper and molybdenum levels were measured in the urine samples of the students included in the study. Results: Iodine deficiency was found with a severe (5%), moderate (18.4%) and mild degree (43%), respectively in the village of Tanışma. Mild iodine deficiency (7%) was found in the center of Hatay. No moderate and severe iodine deficiency was found in the control group. A significant difference was found between the groups in terms of urine iodine excretion (p<0.001). A significant correlation was found between the levels of iodine, selenium, zinc and molybdenum (p<0.05). A moderately positive correlation was found between iodine and selenium (p<0.001). A moderately positive correlation was found between iodine and zinc levels (p<0.001) and a weak correlation was found between iodine and molybdenum (p<0.01). No significant correlation was found between iodine level and copper level (p>0.05). Conclusions: Selenium and zinc deficiency may accompany iodine deficiency. Selenium and zinc deficiency should be considered in individuals who are found to have iodine deficiency especially in endemic goitre regions. PMID:26078645

  19. Pre- and within-meal effects of fluid dairy products on appetite, food intake, glycemia, and regulatory hormones in children.

    PubMed

    Vien, Shirley; Luhovyy, Bohdan L; Patel, Barkha P; Panahi, Shirin; El Khoury, Dalia; Mollard, Rebecca C; Hamilton, Jill K; Anderson, G Harvey

    2017-03-01

    The effect of beverages commonly consumed by children in-between or with meals on short-term food intake (FI) and glycemic control has received little attention. Therefore, 2 experiments were conducted in 9- to 14-year-old children following a randomized repeated-measures design. Experiment 1 (n = 32) compared the effects of water (control) and isocaloric (130 kcal) amounts of 2% milk, chocolate milk, yogurt drink, and fruit punch on subjective appetite and FI. Experiment 2 (n = 20) compared the effects of isocaloric (130 kcal) amounts of 2% milk and fruit punch on subjective appetite, FI, and glycemic and appetite hormone responses. One serving of the beverages was given as a pre-meal drink at baseline (0 min) and a second serving 60 min later with an ad libitum pizza meal. Meal FI in experiment 1 was lower by 14% and 10%, respectively, after chocolate milk and yogurt drink (p < 0.001), but not milk, compared with water. Cumulative energy intake (beverages plus meal) was higher after caloric beverages than water. In experiment 2, no differences occurred in pre-meal but post-meal glucose was 83% higher in overweight/obese than normal-weight children (p = 0.02). Milk led to higher pre-meal glucagon-like peptide-1 and post-meal peptide tyrosine tyrosine (PYY) than fruit punch (p < 0.01) but insulin did not differ between treatments. In conclusion, dairy products consumed before and with a meal have more favourable effects on FI, appetite, and satiety hormones than a sugar-sweetened beverage, but all caloric beverages result in more cumulative calories than if water is the beverage.

  20. Platelet function alterations and their relation to P-selectin (CD62P) expression in children with iron deficiency anemia.

    PubMed

    Yıldırım, Zuhal K; Orhan, Mehmet F; Büyükavcı, Mustafa

    2011-03-01

    Iron deficiency anemia (IDA) may cause platelet aggregation dysfunction and this can be reversed by iron therapy. On the other hand, it has been reported that the platelet fractions carrying the platelet activation markers, CD62P and CD63, are increased in thalassemic patients and there is a significant correlation between the increased levels of soluble P-selectin and free iron in sickle cell disease. This study was performed to investigate the alterations of platelet functions and whether iron deficiency results in diminished expression of activation marker (P-selectin; CD62P) leading to platelet aggregation dysfunction in children with IDA. Hemoglobin, erythrocyte indices (mean erythrocyte volume and red blood cell distribution width), serum levels of iron, transferrin and ferritin, platelet aggregation tests (with ADP, collagen, and ristocetin), PFA-100 closure time, and CD62P expression were evaluated in fasting blood samples of 22 children with IDA and 20 children without anemia. CD62P expression was detected by flow cytometry in normal and 5 μmol/l ADP-activated platelets. Mean closure times were longer in the patient group than control. In platelet aggregation tests, mean values of maximum aggregation times by ristocetin, ADP, and collagen were also more prolonged in patient group. Ristocetin-induced maximum aggregation rates (amplitude) were significantly higher in patients. However, ADP and collagen induction did not produce the same effect. CD62P expressions were significantly higher on activated platelets of the patient group, although they were similar in both groups before activation by ADP. These findings suggest that platelet aggregation and adhesion have been delayed in children with IDA; however, platelet function abnormalities are not associated with CD62P expression on platelet surface.

  1. Phonological abilities in literacy-impaired children: Brain potentials reveal deficient phoneme discrimination, but intact prosodic processing.

    PubMed

    Männel, Claudia; Schaadt, Gesa; Illner, Franziska K; van der Meer, Elke; Friederici, Angela D

    2017-02-01

    Intact phonological processing is crucial for successful literacy acquisition. While individuals with difficulties in reading and spelling (i.e., developmental dyslexia) are known to experience deficient phoneme discrimination (i.e., segmental phonology), findings concerning their prosodic processing (i.e., suprasegmental phonology) are controversial. Because there are no behavior-independent studies on the underlying neural correlates of prosodic processing in dyslexia, these controversial findings might be explained by different task demands. To provide an objective behavior-independent picture of segmental and suprasegmental phonological processing in impaired literacy acquisition, we investigated event-related brain potentials during passive listening in typically and poor-spelling German school children. For segmental phonology, we analyzed the Mismatch Negativity (MMN) during vowel length discrimination, capturing automatic auditory deviancy detection in repetitive contexts. For suprasegmental phonology, we analyzed the Closure Positive Shift (CPS) that automatically occurs in response to prosodic boundaries. Our results revealed spelling group differences for the MMN, but not for the CPS, indicating deficient segmental, but intact suprasegmental phonological processing in poor spellers. The present findings point towards a differential role of segmental and suprasegmental phonology in literacy disorders and call for interventions that invigorate impaired literacy by utilizing intact prosody in addition to training deficient phonemic awareness.

  2. [Prevalence of anemia and iron deficiency in 12 year old school children from Jujuy].

    PubMed

    Buys, María C; Guerra, Lidia N; Martín, Beatriz; Miranda, Carmen E; Torrejón, Irma; Garrot, Teresa

    2005-01-01

    Iron deficiency is highly frequent among adolescents. Its early detection can prevent the development of a ferropenic anemia, a serious condition. The problem has not been well studied in our country. The purpose of this work was to determine the frequency of iron deficiency and anemia in adolescents. The criteria considered were: hematocrit below 38%, b) saturation transferrin below 16%, c) ferritin below 15 ng/ml. The study was carried out in 2265 schoolchildren, 12 years old, of both sexes, in urban and periurban areas in the city of San Salvador de Jujuy (1250 a.s.l.). The following parameters were measured: hematocrit as well as serum iron and total iron binding capacity, both by colorimetric method. Ferritin was measured by ELISA. Anemia was not found. Iron deficiency as estimated by the iron functional component, was found in 25% of girls and 21% of boys and, through iron stores, in 28% of girls and 18% of boys. Iron deficiency stores in both sexes is the more relevant alteration, indicating that the population sample here studied constitutes a highly vulnerable group. The early detection of iron deficiency will help physical and intellectual development so that adequate sanitary policies are necessary for its prevention.

  3. The corticotropin-releasing hormone test in normal short children: comparison of plasma adrenocorticotropin and cortisol responses to human corticotropin-releasing hormone and insulin-induced hypoglycemia.

    PubMed

    Goji, K

    1989-03-01

    The human corticotropin-releasing hormone (hCRH) tests were performed in twelve normal short children, and the responses of plasma ACTH and cortisol to iv administration of 1 micrograms/kg hCRH were compared with those to insulin-induced hypoglycemia. After administration of hCRH, the mean plasma ACTH level rose from a basal value of 3.3 +/- 0.4 pmol/l (mean +/- SEM) to a peak value of 9.2 +/- 0.8 pmol/l at 30 min, and the mean plasma cortisol level rose from a basal value of 231 +/- 25 nmol/l to a peak value of 546 +/- 30 nmol/l at 30 min. The ACTH response after insulin-induced hypoglycemia was greater than that after hCRH administration; the mean peak level (P less than 0.01), the percent maximum increment (P less than 0.01), and the area under the ACTH response curve (P less than 0.01) were all significantly greater after insulin-induced hypoglycemia than those after hCRH administration. Although the mean peak cortisol level after insulin-induced hypoglycemia was about 1.3-fold higher than that after hCRH administration (P less than 0.01), neither the percent maximum increment in plasma cortisol nor the area under the cortisol response curve after insulin-induced hypoglycemia was significantly different from that after hCRH administration. Consequently, the acute increases in plasma ACTH after the administration of 1 microgram/kg hCRH stimulated the adrenal gland to almost the same cortisol response as that obtained with a much greater increase in plasma ACTH after insulin-induced hypoglycemia. These results suggest that a plasma ACTH peak of 9-11 pmol/l produces near maximum acute stimulation of adrenal steroidogenesis.

  4. [Observation on clinical efficacy of Baoerkang san on spleen-deficiency and dampness-obstructing diarrhea in children].

    PubMed

    Zhang, Li-xue

    2015-04-01

    The study aims to observe the clinical efficacy of Baoerkang san on spleen-deficiency and dampness-obstructing diarrhea in children. Children diarrhea divided into acute diarrhea (group I) and chronic persistent diarrhea (group II). A randomized, double-blind, positive control test was conducted for dose finding. The 340 cases with acute diarrhea in group I were divided into three groups: the high dose group (112 patients), the low dose group (113 patients) and the positive control group (115 patients), which were treated for 3 days. Their clinical efficacies were compared to evaluate the clinical safety of Baoerkang san. The 167 patients with chronic persistent diarrhea in group If were divided into the high dose group (56 patients), the low dose group (55 patients) and the control group (56 patients), which were treated for 5 days. Their clinical efficacy were compared to evaluate the clinical safety of Baoerkang san. According to the results, the cure rate and the effective rate of acute diarrhea and chronic persistent diarrhea in the high dose group and the low dose group were significantly higher than that of the positive control group (P <0. 05, P < 0.01). In the treatment of spleen-deficiency and dampness-obstructing diarrhea with traditional Chinese medicines, the cure rate and the effective rate of acute diarrhea and chronic persistent diarrhea in the high dose group and the low dose group were significantly higher than that of the positive control group (P < 0.05, P < 0.01). During the test, all of the three groups did not suffer any adverse event, with no any abnormality in general physical indexes. In conclusion, Baoerkang san shows a significant efficacy in treating acute diarrhea and chronic persistent diarrhea (spleen-deficiency and dampness-obstructing) and safe clinical application.

  5. Synergistic effect of obesity and lipid ingestion in suppressing the growth hormone response to exercise in children

    PubMed Central

    Oliver, Stacy R.; Hingorani, Sunita R.; Rosa, Jaime S.; Zaldivar, Frank P.

    2012-01-01

    Diet plays an important role in modulating exercise responses, including activation of the growth hormone (GH)/insulin-like growth factor-I (IGF-1) axis. Obesity and fat ingestion were separately shown to reduce exercise GH responses, but their combined effect, especially important in children, has not been studied. We therefore measured the GH response to exercise [30-min intermittent cycling, ten 2-min bouts at ∼80% maximal aerobic capacity (V̇o2max), separated by 1-min rest], started 45 min after ingestion of a high-fat meal (HFM) in 16 healthy [controls; body mass index percentile (BMI%ile) 51 ± 7], and 19 obese (Ob, BMI%ile 97 ± 0.4) children. Samples were drawn at baseline (premeal), and at start, peak, and 30 min postexercise. In the Ob group, a marked ∼75% suppression of the GH response (ng/ml) to exercise was observed (2.4 ± 0.6 vs. 10.6 ± 2.1, P < 0.001). This level of suppression was also significantly greater compared with age-, fitness-, and BMI-matched historical controls that had performed identical exercise in fasting conditions. Our data indicate that the reduction in the GH response to exercise, already present in obese children vs. healthy controls, is considerably amplified by ingestion of fat nutrients shortly before exercise, implying a potentially downstream negative impact on growth factor homeostasis and long-term modulation of physiological growth. PMID:22518832

  6. Heritability of hyperphagic eating behavior and appetite-related hormones among Hispanic children

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Eating in the absence of hunger (EAH) may be a genetically influenced phenotype of overweight children, but evidence is limited. This research evaluated the heritability (h2) of EAH and its association with overweight among Hispanic children 5 to 18 years old. Genetic and environmental associations ...

  7. Hormone levels

    MedlinePlus

    Blood or urine tests can determine the levels of various hormones in the body. This includes reproductive hormones, thyroid hormones, adrenal hormones, pituitary hormones, and many others. For more information, see: ...

  8. Anemia, Micronutrient Deficiencies, and Malaria in Children and Women in Sierra Leone Prior to the Ebola Outbreak - Findings of a Cross-Sectional Study

    PubMed Central

    Wirth, James P; Rohner, Fabian; Woodruff, Bradley A; Chiwile, Faraja; Yankson, Hannah; Koroma, Aminata S; Russel, Feimata; Sesay, Fatmata; Dominguez, Elisa; Petry, Nicolai; Shahab-Ferdows, Setareh; de Onis, Mercedes; Hodges, Mary H

    2016-01-01

    To identify the factors associated with anemia and to document the severity of micronutrient deficiencies, malaria and inflammation, a nationally representative cross-sectional survey was conducted. A three-stage sampling procedure was used to randomly select children <5 years of age and adult women from households in two strata (urban and rural). Household and individual data were collected, and blood samples from children and women were used to measure the prevalence of malaria, inflammation, and deficiencies of iron, vitamin A, folate, and vitamin B12. 839 children and 945 non-pregnant women were included in the survey. In children, the prevalence rates of anemia (76.3%; 95% CI: 71.8, 80.4), malaria (52.6%; 95% CI: 46.0, 59.0), and acute and chronic inflammation (72.6%; 95% CI: 67.5, 77.1) were high. However, the prevalence of vitamin A deficiency (17.4%; 95% CI: 13.9, 21.6) was moderate, and the prevalence of iron deficiency (5.2%; 95% CI: 3.3, 8.1) and iron-deficiency anemia (3.8%; 95% CI: 2.5, 5.8) were low. Malaria and inflammation were associated with anemia, yet they explained only 25% of the population-attributable risk. In women, 44.8% (95% CI: 40.1, 49.5), 35.1% (95% CI: 30.1, 40.4), and 23.6% (95% CI: 20.4, 27.3) were affected by anemia, malaria, or inflammation, respectively. The prevalence rates of iron deficiency (8.3%; 95% CI: 6.2, 11.1), iron-deficiency anemia (6.1%; 95% CI: 4.4, 8.6), vitamin A deficiency (2.1%; 95% CI: 1.1, 3.1) and vitamin B12 deficiency (0.5%; 95% CI: 0.2, 1.4) were low, while folate deficiency was high (79.2%; 95% CI: 74.1, 83.5). Iron deficiency, malaria, and inflammation were significantly associated with anemia, but explained only 25% of cases of anemia. Anemia in children and women is a severe public health problem in Sierra Leone. Since malaria and inflammation only contributed to 25% of anemia, other causes of anemia, such as hemoglobinopathies, should also be explored. PMID:27163254

  9. An inflection point of serum 25-hydroxyvitamin D for maximal suppression of parathyroid hormone is not evident from multi-site pooled data in children and adolescents

    Technology Transfer Automated Retrieval System (TEKTRAN)

    In adults, maximal suppression of serum parathyroid hormone (PTH) has commonly been used to determine the sufficiency of serum 25-hydroxyvitamin D [25(OH) D]. In children and adolescents, the relationship between serum 25(OH) D and PTH is less clear, and most studies reporting a relationship are der...

  10. Disturbances in the growth hormone-insulin-like growth factor axis in children and adolescents with different eating disorders.

    PubMed

    Argente, J; Caballo, N; Barrios, V; Muñoz, M T; Pozo, J; Chowen, J A; Hernández, M

    1997-01-01

    Numerous endocrine abnormalities of the growth hormone (GH)-insulin-like growth factor axis have been described in patients with both anorexia nervosa and obesity during childhood and adolescence. These alterations include changes in the levels of 24-hour spontaneous GH secretion, high-affinity, low-capacity GH binding protein (GHBP), IGF-I, IGF-II and the IGF binding proteins (IGFBPs). However, the existing information is sometimes confusing and contradictory. Furthermore, little or no data in these pathologies are available concerning IGFBP-2 or free IGF-I. We have analysed the GH-IGF axis in large populations of adolescents with anorexia nervosa and prepubertal children with exogenous obesity. These patients were studied at the time of diagnosis and at two timepoints during nutritional therapy and normal weight recovery. The results of these studies using age- and sex-matched controls are described here.

  11. Pituitary function after treatment of intracranial tumours in children.

    PubMed

    Shalet, S M; Beardwell, C G; Morris-Jones, P H; Pearson, D

    1975-07-19

    Pituitary-function tests have been done in twenty-seven patients at various times after treatment in childhood for intracranial tumours not directly involving the hypothalamic-pituitary region. Impaired growth hormone (G.H.) responses to hypoglycaemia and 'Bovril' were found in ten children. There seeems to be progressive impairment in G.H. production with time after treatment. During the first 3 months after neurosurgery no child was found to be G.H. deficient, but the peak G.H. response of this group seemed to be blunted when compared with a control group of children who had been treated for abdominal tumours. The rest of anterior-pituitary function in G.H.-deficient children seems quite normal except for a significantly greater basal thyroid-stimulating hormone (T.S.H.) level and T;S.H. response after thyrotrophin-releasing hormone. Two girls have developed secondary amenorrhoea, and one is G.H. deficient.

  12. Zinc deficiency in children with environmental enteropathy - development of new strategies: Report from an expert workshop

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Zinc deficiency is a major cause of childhood morbidity and mortality. The WHO/UNICEF strategy for zinc supplementation as adjunctive therapy for diarrhea is poorly implemented. A conference of experts in zinc nutrition and gastrointestinal disorders was convened to consider approaches that might co...

  13. Deficient Orthographic and Phonological Representations in Children with Dyslexia Revealed by Brain Activation Patterns

    ERIC Educational Resources Information Center

    Cao, Fan; Bitan, Tali; Chou, Tai-Li; Burman, Douglas D.; Booth, James R.

    2006-01-01

    Background: The current study examined the neuro-cognitive network of visual word rhyming judgment in 14 children with dyslexia and 14 age-matched control children (8- to 14-year-olds) using functional magnetic resonance imaging (fMRI). Methods: In order to manipulate the difficulty of mapping orthography to phonology, we used conflicting and…

  14. Production Deficiencies in Elicited Language but Not in the Spontaneous Verbalizations of Hyperactive Children.

    ERIC Educational Resources Information Center

    Zentall, Sydney S.

    1988-01-01

    Twenty-two hyperactive children were more spontaneously talkative than controls during transitions and nonverbal tasks (nonelicited conditions), but less talkative when asked to tell stories (elicited conditions). Findings suggest that minimal stimulus input precipitate excessive verbal activity from hyperactive children, while stories requiring…

  15. Vitamin D deficiency in critically ill children: A roadmap to interventional research

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Two studies published this month in Pediatrics provide new and unique information regarding the relationship between vitamin D status and critical illnesses in children admitted to PICUs in the United States and Canada. These two studies, from Boston Children's Hospital and six PICUs in Canada, demo...

  16. [Vitamin A deficiency and the anthropometric nutritional status of urban and rural marginalized children in the state of Zulia, Venezuela].

    PubMed

    Amaya-Castellanos, Daisy; Viloria-Castejón, Haydée; Ortega, Pablo; Gómez, Gisela; Urrieta, Jesús R; Lobo, Pablo; Estévez, Jesús

    2002-06-01

    The present transversal study was carried out to estimate the prevalence of both vitamin A deficiency (VAD) and protein-energy malnutrition among children (24 to 85 months) from three urban slums (n = 173) in Maracaibo city, Zulia State, Venezuela and a rural slum area (n = 34), vieinal to Maracaibo, by measuring serum retinol and z score of anthropometric indices Height//Age (H//AZ); Weight//Age (W//AZ) and Weight//Height (W//HZ), compared to NCHS-WHO reference values. The Graffar's methodology adapted to Venezuela by Méndez Castellano (1986) confirmed the underprivileged socio-economic condition of the children population. For serum retinol analysis, peripheral venous blood was drawn and serum was treated according to the Bieri et al. (1979) technique and HPLC procedure. Values were recorded in microgram/dL. Statistical analysis was done by using Epi Info 2000, release 1.0 and SAS release 6.0 (1996) computer programs. The prevalence of VAD (serum retinol < 20 micrograms/dL) in the total children population (n = 207) was 22.2%, being higher in urban children than in rural children (22.5% vs 20.5%). No clinical signs of VAD were detected in the children. The nutritional status analysed by Z score of anthropometric indices revealed that 27.4% of children suffered from undernutrition (Z score = -2 to > -3 SD), being 15.4% stunted, 9.6% wasted and 2.6% with acute protein-energy malnutrition. Neither severe malnutrition nor overweight were detected. According with the H//AZ index, 54.6% of children had adequate nutrition. However 23% of them suffered from VAD. In children at risk of developing undernutrition (Z score = -1 to > -2 SD), 20.9% had VAD and of the stunted children, 21.9% presented VAD. With W//AZ, 60.3%, 29.9% and 9.1% of children were with adequate nutrition, at risk, or wasted respectively; of each group, 25.6%, 17.7% and 15%, respectively had serum retinol values below 20 micrograms/dL. The Z score of W//HZ indicator revealed that 87.4%, 10.4% and 2

  17. Iodine deficiency in pregnancy: the effect on neurodevelopment in the child.

    PubMed

    Skeaff, Sheila A

    2011-02-01

    Iodine is an integral part of the thyroid hormones, thyroxine (T(4)) and tri-iodothyronine (T(3)), necessary for normal growth and development. An adequate supply of cerebral T(3), generated in the fetal brain from maternal free T(4) (fT(4)), is needed by the fetus for thyroid hormone dependent neurodevelopment, which begins in the second half of the first trimester of pregnancy. Around the beginning of the second trimester the fetal thyroid also begins to produce hormones but the reserves of the fetal gland are low, thus maternal thyroid hormones contribute to total fetal thyroid hormone concentrations until birth. In order for pregnant women to produce enough thyroid hormones to meet both her own and her baby's requirements, a 50% increase in iodine intake is recommended. A lack of iodine in the diet may result in the mother becoming iodine deficient, and subsequently the fetus. In iodine deficiency, hypothyroxinemia (i.e., low maternal fT(4)) results in damage to the developing brain, which is further aggravated by hypothyroidism in the fetus. The most serious consequence of iodine deficiency is cretinism, characterised by profound mental retardation. There is unequivocal evidence that severe iodine deficiency in pregnancy impairs brain development in the child. However, only two intervention trials have assessed neurodevelopment in children of moderately iodine deficient mothers finding improved neurodevelopment in children of mothers supplemented earlier rather than later in pregnancy; both studies were not randomised and were uncontrolled. Thus, there is a need for well-designed trials to determine the effect of iodine supplementation in moderate to mildly iodine deficient pregnant women on neurodevelopment in the child.

  18. Micronutrient Deficiencies and Related Factors in School-Aged Children in Ethiopia: A Cross-Sectional Study in Libo Kemkem and Fogera Districts, Amhara Regional State

    PubMed Central

    Herrador, Zaida; Sordo, Luis; Gadisa, Endalamaw; Buño, Antonio; Gómez-Rioja, Rubén; Iturzaeta, Jose Manuel; de Armas, Lisset Fernandez; Benito, Agustín; Aseffa, Abraham; Moreno, Javier; Cañavate, Carmen; Custodio, Estefania

    2014-01-01

    Introduction The present study describes the distribution of selected micronutrients and anaemia among school-aged children living in Libo Kemkem and Fogera (Amhara State, Ethiopia), assessing differences by socio-demographic characteristics, health status and dietary habits. Methods A cross-sectional survey was carried out during May–December 2009. Socio-demographic characteristics, health status and dietary habits were collected. Biomarkers were determined for 764 children. Bivariate and multivariable statistical methods were employed to assess micronutrient deficiencies (MD), anaemia, and their association with different factors. Results More than two thirds of the school-aged children (79.5%) had at least one MD and 40.5% had two or more coexisting micronutrient deficiencies. The most prevalent deficiencies were of zinc (12.5%), folate (13.9%), vit A (29.3%) and vit D (49%). Anaemia occurred in 30.9% of the children. Children living in rural areas were more likely to have vit D insufficiency [OR: 5.9 (3.7–9.5)] but less likely to have folate deficiency [OR: 0.2 (0.1–0.4)] and anaemia [OR: 0.58 (0.35–0.97)]. Splenomegaly was positively associated with folate deficiency and anaemia [OR: 2.77 (1.19–6.48) and 4.91 (2.47–9.75)]. Meat and fish consumption were inversely correlated with zinc and ferritin deficiencies [OR: 0.2 (0.1–0.8) and 0.2 (0.1–0.9)], while oil consumption showed a negative association with anaemia and deficiencies of folate and vitamin A [0.58 (0.3–0.9), OR: 0.5 (0.3–0.9) and 0.6 (0.4–0.9)]. Serum ferritin levels were inversely correlated to the presence of anaemia (p<0.005). Conclusion There is a high prevalence of vitamin A deficiency and vitamin D insufficiency and a moderate prevalence of zinc and folate deficiencies in school-aged children in this area. The inverse association of anaemia and serum ferritin levels may be due to the presence of infectious diseases in the area. To effectively tackle malnutrition

  19. Use of iron supplements in children aged 1-2 years with iron deficiency anemia: A cross-sectional study

    PubMed Central

    Sezik, Handan Atsiz; Can, Huseyin; Kurnaz, Mehmet Ali; Tuna, Mine; Ay, Zeynep

    2015-01-01

    Objectives: Iron deficiency (ID) is the most common nutritional problem in the world and is the most common cause of childhood anemia. In this study, our aim was to find out about the state of usage of iron preparation, which is distributed free of charge by the Ministry of Health, for the infants between 4-12 months in our country, as well as detecting the awareness degree of families those who are informed about iron-deficiency anemia (IDA), prophylaxis of the drug and to determine the drug’s effectiveness. Methods: It was a cross-sectional survey. The laboratory values from the files of the children aged 1-2 those who visited our hospital’s department of pediatrics, between January 2010 to August 2013, were collected. The survey included families who have children diagnosed with IDA. Questions included about families’ sociodemographic characteristics, the state of the usage of the iron drug, how much information received in terms of the side effects- consumption period and dosage. Results: A total of 139 children were enrolled in our study. While 77.7% of the families who participated stated that (n = 108) iron medicine was prescribed other 43.2% of families stated (n = 60) was prescribed and they were informed about iron pills and IDA. 25.9% of families had received information about drug’s side effects, 74.8% of them had information about period of consumption and 77.7% said they were given information about the drug dose. The average duration of use of iron medicine was 6.98±4.52 (min: 1, max: 24) months. It has been noted that; parent’s education level, mother’s occupation, child’s gender, how the child was born and receiving information about how to use the medicine had no effects on usage of the drug in children. Nevertheless, it has been noticed that, when the families were given proper information the drug use increased and the patients compliance with medications also increased. Conclusion: We believe that, due to frequent diagnosis of

  20. A comparative study of fluoride ingestion levels, serum thyroid hormone & TSH level derangements, dental fluorosis status among school children from endemic and non-endemic fluorosis areas.

    PubMed

    Singh, Navneet; Verma, Kanika Gupta; Verma, Pradhuman; Sidhu, Gagandeep Kaur; Sachdeva, Suresh

    2014-01-03

    The study was undertaken to determine serum/urinary fluoride status and comparison of free T4, free T3 and thyroid stimulating hormone levels of 8 to 15 years old children with and without dental fluorosis living in an endemic and non-endemic fluorosis area. A sample group of 60 male and female school children, with or without dental fluorosis, consuming fluoride-contaminated water in endemic fluoride area of Udaipur district, Rajasthan were selected through a school dental fluorosis survey. The sample of 10 children of same age and socio-economic status residing in non endemic areas who did not have dental fluorosis form controls. Fluoride determination in drinking water, urine and blood was done with Ion 85 Ion Analyzer Radiometer with Hall et al. method. The thyroid gland functional test was done by Immonu Chemiluminiscence Micropartical Assay with Bayer Centaur Autoanalyzer. The significantly altered FT3, FT4 and TSH hormones level in both group1A and 1B school children were noted. The serum and urine fluoride levels were found to be increased in both the groups. A significant relationship of water fluoride to urine and serum fluoride concentration was seen. The serum fluoride concentration also had significant relationship with thyroid hormone (FT3/FT4) and TSH concentrations. The testing of drinking water and body fluids for fluoride content, along with FT3, FT4, and TSH in children with dental fluorosis is desirable for recognizing underlying thyroid derangements and its impact on fluorosis.

  1. Carnitine-acylcarnitine translocase deficiency. Clinical course of three Saudi children with a severe phenotype.

    PubMed

    Al-Sannaa, Nouriya A; Cheriyan, George M

    2010-08-01

    Carnitine-acylcarnitine translocase (CACT) deficiency (McKusick 212138) is a rare life threatening disorder characterized by hypoketotic hypoglycemia, hyperammonemia, encephalopathy, cardiomyopathy hepatopathy, and myopathy. Here, we present a detailed clinical course of 3 Saudi siblings with a severe phenotype. The third patient was described in more detail. Early medical intervention in the form of 25% dextrose intravenous infusion and carnitine supplement followed by a gradual introduction of a high carbohydrate low fat special formula resulted in a good clinical and biochemical response to the treatment in our patient. However, early nephrocalcinosis, severe hypotonia, and subsequently intravascular cerebral accident could not be prevented. He died at 18 months of age as a result of metabolic decompensation. This suggests that CACT deficiency is still a lethal disorder even with an early and aggressive medical intervention.

  2. Growth hormone significantly increases the adult height of children with idiopathic short stature: comparison of subgroups and benefit

    PubMed Central

    2014-01-01

    Background Children with Idiopathic Short Stature do not attain a normal adult height. The improvement of adult height with treatment with recombinant human growth hormone (rhGH), at doses of 0.16 to 0.28 mg/kg/week is modest, usually less that 4 cm, and they remain short as adults. The benefit obtained seems dose dependent and benefits of 7.0 to 8.0 cm have been reported with higher doses of 0.32 to 0.4 mg/kg/week, but the number of studies is limited. The topic has remained controversial. Objective The objective was to conduct a retrospective analysis of our experience with 123 children with ISS treated with 0.32 ± 0.03 mg/kg/week of rhGH, with the aim of comparing the different subgroups of non-familial short stature, familial short stature, normal puberty, and delayed puberty and to assess the benefit by comparison with 305 untreated historical controls, from nine different randomized and nonrandomized controlled studies. Results Eighty eight of our children (68 males and 20 females) attained an adult height or near adult height of -0.71 SDS (0.74 SD) (95% CI, -0.87 to -0.55) with a benefit over untreated controls of 9.5 cm (7.4 to 11.6 cm) for males and 8.6 cm (6.7 to 10.5 cm) for females. In the analysis of the subgroups, the adult height and adult height gain of children with non-familial short stature were significantly higher than of familial short stature. No difference was found in the cohorts with normal or delayed puberty in any of the subgroups, except between the non-familial short stature and familial short stature puberty cohorts. This has implications for the interpretation of the benefit of treatment in studies where the number of children with familial short stature in the controls or treated subjects is not known. The treatment was safe. There were no significant adverse events. The IGF-1 values were essentially within the levels expected for the stages of puberty. Conclusion Our experience was quite positive with normalization of

  3. Anti-Müllerian hormone levels in serum from human foetuses and children: pattern and clinical interest.

    PubMed

    Guibourdenche, J; Lucidarme, N; Chevenne, D; Rigal, O; Nicolas, M; Luton, D; Léger, J; Porquet, D; Noël, M

    2003-12-15

    Serum anti-Müllerian hormone (AMH) determination has been used to investigate gonadal development and abnormal sexual differentiation, but until recently, it was based on assays developed by specialized laboratories. A short time ago, a sensitive assay kit was developed commercially (Immunotech-Beckman Coulter) for clinical use. With this method, we established usual levels of serum AMH in fetuses, newborns, and pre-pubertal children, and evaluated the clinical value of this assay. AMH measurement required only 25 microl of sample and could be performed within 3 h. In females, AMH emerged after birth at low levels (median: 4 ng/ml). In males, AMH levels remained stable during fetal life (median: 44.4 ng/ml), peaked in the first months of life to reach a median of 124.7 ng/ml, then fell with wide individual variations. Cord blood AMH levels at birth may be useful to investigate ambiguous genitalia suspected prenatally. In children with isolated microphallus or hypospadias, decreased AMH values are in favor of testis dysfunction. When testes cannot be palpated, a single determination of serum AMH levels can distinguish between anorchia and cryptorchidism.

  4. Post-natal anaemia and iron deficiency in HIV-infected women and the health and survival of their children.

    PubMed

    Isanaka, Sheila; Spiegelman, Donna; Aboud, Said; Manji, Karim P; Msamanga, Gernard I; Willet, Walter C; Duggan, Christopher; Fawzi, Wafaie W

    2012-07-01

    Prenatal iron supplementation may improve pregnancy outcomes and decrease the risk of child mortality. However, little is known about the importance of post-natal maternal iron status for child health and survival, particularly in the context of HIV infection. We examined the association of maternal anaemia and hypochromic microcytosis, an erythrocyte morphology consistent with iron deficiency, with child health and survival in the first two to five years of life. Repeated measures of maternal anaemia and hypochromic microcytosis from 840 HIV-positive women enrolled in a clinical trial of vitamin supplementation were prospectively related to child mortality, HIV infection and CD4 T-cell count. Median duration of follow-up for the endpoints of child mortality, HIV infection and CD4 cell count was 58, 17 and 23 months, respectively. Maternal anaemia and hypochromic microcytosis were associated with greater risk of child mortality [hazard ratio (HR) for severe anaemia = 2.58, 95% confidence interval (CI): 1.66-4.01, P trend < 0.0001; HR for severe hypochromic microcytosis = 2.36, 95% CI: 1.27-4.38, P trend = 0.001]. Maternal anaemia was not significantly associated with greater risk of child HIV infection (HR for severe anaemia = 1.46, 95% CI: 0.91, 2.33, P trend = 0.08) but predicted lower CD4 T-cell counts among HIV-uninfected children (difference in CD4 T-cell count/µL for severe anaemia: -93, 95% CI: -204-17, P trend = 0.02). The potential child health risks associated with maternal anaemia and iron deficiency may not be limited to the prenatal period. Efforts to reduce maternal anaemia and iron deficiency during pregnancy may need to be expanded to include the post-partum period.

  5. Trace Element Status (Iron, Zinc, Copper, Chromium, Cobalt, and Nickel) in Iron-Deficiency Anaemia of Children under 3 Years

    PubMed Central

    Angelova, Maria Georgieva; Petkova-Marinova, Tsvetelina Valentinova; Pogorielov, Maksym Vladimirovich; Loboda, Andrii Nikolaevich; Nedkova-Kolarova, Vania Nedkova; Bozhinova, Atanaska Naumova

    2014-01-01

    Aim. To determine trace element status and aetiologic factors for development of trace elements deficiencies in children with iron-deficiency anaemia (IDA) aged 0 to 3 years. Contingent and Methods. 30 patients of the University Hospital, Pleven, Bulgaria—I group; 48 patients of the Sumy Regional Child's Clinical Hospital, Sumy, Ukraine—II group; 25 healthy controls were investigated. Serum concentrations of iron, zinc, copper, chromium, cobalt, and nickel were determined spectrophotometrically and by atomic absorption spectrophotometry. Results. Because the obtained serum levels of zinc, copper, and chromium were near the lower reference limits, I group was divided into IA and IB. In IA group, serum concentrations were lower than the reference values for 47%, 57%, and 73% of patients, respectively. In IB group, these were within the reference values. In II group, results for zinc, cobalt, and nickel were significantly lower (P < 0.05), and results for copper were significantly higher in comparison to controls. Conclusion. Low serum concentrations of zinc, copper, cobalt, and nickel were mainly due to inadequate dietary intake, malabsorption, and micronutrient interactions in both studied groups. Increased serum copper in II group was probably due to metabolic changes resulting from adaptations in IDA. Data can be used for developing a diagnostic algorithm for IDA. PMID:24839556

  6. Glucose-6-phosphate dehydrogenase deficiency, chlorproguanil-dapsone with artesunate and post-treatment haemolysis in African children treated for uncomplicated malaria

    PubMed Central

    2012-01-01

    Background Malaria is a leading cause of mortality, particularly in sub-Saharan African children. Prompt and efficacious treatment is important as patients may progress within a few hours to severe and possibly fatal disease. Chlorproguanil-dapsone-artesunate (CDA) was a promising artemisinin-based combination therapy (ACT), but its development was prematurely stopped because of safety concerns secondary to its associated risk of haemolytic anaemia in glucose-6-phosphate dehydrogenase (G6PD)-deficient individuals. The objective of the study was to assess whether CDA treatment and G6PD deficiency are risk factors for a post-treatment haemoglobin drop in African children <5 years of age with uncomplicated malaria. Methods This case–control study was performed in the context of a larger multicentre randomized clinical trial comparing safety and efficacy of four different ACT in children with uncomplicated malaria. Children, who after treatment experienced a haemoglobin drop ≥2 g/dl (cases) within the first four days (days 0, 1, 2, and 3), were compared with those without an Hb drop (controls). Cases and controls were matched for study site, sex, age and baseline haemoglobin measurements. Data were analysed using a conditional logistic regression model. Results G6PD deficiency prevalence, homo- or hemizygous, was 8.5% (10/117) in cases and 6.8% (16/234) in controls (p = 0.56). The risk of a Hb drop ≥2 g/dl was not associated with either G6PD deficiency (adjusted odds ratio (AOR): 0.81; p = 0.76) or CDA treatment (AOR: 1.28; p = 0.37) alone. However, patients having both risk factors tended to have higher odds (AOR: 11.13; p = 0.25) of experiencing a Hb drop ≥2 g/dl within the first four days after treatment, however this finding was not statistically significant, mainly because G6PD deficient patients treated with CDA were very few. In non-G6PD deficient individuals, the proportion of cases was similar between treatment groups while in G

  7. Association of E-selectin with hematological, hormonal levels and plasma proteins in children with end stage renal disease

    PubMed Central

    Meamar, Rokhsareh; Shafiei, Mohammad; Abedini, Amin; Ghazvini, Mohammad Reza Aghaye; Roomizadeh, Peyman; Taheri, Shahram; Gheissari, Alaleh

    2016-01-01

    Background: Hypercoagulable state is a common serious problem in patients with end-stage renal disease (ESRD). ESRD patients are in a condition of chronic inflammation. An increased level of E-selectin, “a key adhesion molecule that regulates leukocyte bindings to endothelium at damaged sites,” accompanies the higher risk of inflammation in ESRD patients. We aimed to investigate the possible correlation among E-selectin as an adhesion molecule, coagulation factors, and inflammatory factors in children with ESRD. Materials and Methods: Thirty-five child patients with ESRD who had been on regular dialysis treatment were registered in our study. Nighteen sex- and age-matched healthy volunteers were used as the control group. Laboratory tests were requested for the evaluation of hematological and biochemical parameters, and parathyroid hormone (PTH), and for coagulation state; fibrinogen, protein C, and protein S were measured. The enzyme-linked immunosorbent assay (ELISA) (Biomerica, CA, and IDS, UK). for serum E-selectin assay was provided by R and D Systems (Abingdon, UK). Results: Hemoglubolin (Hb), blood urea nitrogen (BUN), creatinine, calcium, PTH, triglyceride (TG) concentrations in serum as well as E-selectin showed significant difference between the two study groups, as indeed was expected. Serum E-selectin was significantly higher (P value = 0.033) in dialysis patients than in healthy subjects. E-selectin was positively correlated only with phosphorus in ESRD children (r = 0.398, P = 0.018). No association was found for other parameters. Conclusion: Although in our study circulating E-selectin concentration “as an inflammatory maker” is independently positively associated with limited blood markers, for better evaluation, well-designed cohort studies should be examined in ESRD children. PMID:27563628

  8. Obesity-Related Hormones in Low-Income Preschool-Age Children: Implications for School Readiness

    ERIC Educational Resources Information Center

    Miller, Alison L.; Lumeng, Carey N.; Delproposto, Jennifer; Florek, Brian; Wendorf, Kristin; Lumeng, Julie C.

    2013-01-01

    Mechanisms underlying socioeconomic disparities in school readiness and health outcomes, particularly obesity, among preschool-aged children are complex and poorly understood. Obesity can induce changes in proteins in the circulation that contribute to the negative impact of obesity on health; such changes may relate to cognitive and emotion…

  9. A Program of Visual-Motor-Perceptual Training to Determine Its Effects upon Primary Level Children with Reading and Learning Deficiencies. Final Report.

    ERIC Educational Resources Information Center

    Litchfield, Ticknor B.

    The purpose of the project was to determine to what extent visual, motor, and perceptual training would improve the reading and general achievement of children with visual, motor, and perceptual deficiencies. Eighty first, second, and third graders, identified as having such handicaps by gross and fine screening instruments, were randomly divided…

  10. Iron Deficiency in Autism and Asperger Syndrome.

    ERIC Educational Resources Information Center

    Latif, A.; Heinz, P.; Cook, R.

    2002-01-01

    Retrospective analysis of the full blood count and, when available, serum ferritin measurements of 96 children (52 with autism and 44 with Asperger syndrome) found six autistic children had iron deficiency and 12 of the 23 autistic children with serum ferritin measures were iron deficient. Far fewer Asperger children were iron deficient. Results…

  11. Nitric oxide (NO)-mediated mitochondrial damage plays a critical role in T-2 toxin-induced apoptosis and growth hormone deficiency in rat anterior pituitary GH3 cells.

    PubMed

    Liu, Xianglian; Guo, Pu; Liu, Aimei; Wu, Qinghua; Xue, Xijuan; Dai, Menghong; Hao, Haihong; Qu, Wei; Xie, Shuyu; Wang, Xu; Yuan, Zonghui

    2017-04-01

    T-2 toxin, a major compound of trichothecenes, induces cell apoptosis and growth hormone (GH) deficiency and causes considerable growth retardation in animals and human cells. However, the mechanism underlying its growth suppression still remains unclear. Recent studies have suggested that ROS induced cell apoptosis and animal feed intake reduction, but there are limited reports on the role of RNS in T-2 toxin-mediated mitochondrial damage, cell apoptosis and growth retardation. Herein, T-2 toxin-induced GH3 cell damage and apoptosis were tested by MTT assay, LDH leakage and flow cytometry, respectively. Intracellular NO and antioxidant enzyme activity, ΔΨm, morphometric changes of mitochondria, the caspase pathway, and inflammatory factors were investigated. Free radical scavengers NAC, SOD and NO scavenger haemoglobin were used to explore the role of oxidative stress and the relationship between NO production and caspase pathway. The results clearly revealed that T-2 toxin caused significant increases in NO generation, cell apoptosis, GH deficiency, increased iNOS activity, upregulation of inflammatory factors and caspase pathway, decreases in ΔΨm and morphosis damage. These data suggest that mitochondria are a primary target of T-2 toxin-induced NO, and NO is a key mediator of T-2 toxin-induced cell apoptosis and GH deficiency via the mitochondria-dependent pathway in cells.

  12. Comparisons of thyroid hormone, intelligence, attention, and quality of life in children with obstructive sleep apnea hypopnea syndrome before and after endoscopic adenoidectomy.

    PubMed

    Feng, Hui-Wei; Jiang, Tao; Zhang, Hong-Ping; Wang, Zhe; Zhang, Hai-Ling; Zhang, Hui; Chen, Xue-Mei; Fan, Xian-Liang; Tian, Yu-Dong; Jia, Tao

    2015-01-01

    Objective. The aim of this study was to compare the differences in thyroid hormone, intelligence, attention, and quality of life (QoL) of children with obstructive sleep apnea hypopnea syndrome (OSAHS) before and after endoscopic adenoidectomy. Method. A total of 35 OSAHS children (21 males and 14 females with a mean age of 6.81 ± 1.08 years) were included in this study for analyzing the levels of thyroid hormone, intelligence, attention, and QoL. There were 22 children underwent endoscopic adenoidectomy with bilateral tonsillectomy (BT), while the other 13 children who underwent endoscopic adenoidectomy without bilateral tonsillectomy without BT. Results. Our results revealed no significant difference in serum free triiodothyronine (FT3), free thyroxine (FT4), and thyroid stimulating hormone (TSH) levels in OSAHS children before and after endoscopic adenoidectomy (all P > 0.05). However, there were significant differences in full-scale intelligence quotient (FIQ) (92.45 ± 5.88 versus 106.23 ± 7.39, P < 0.001), verbal intelligence quotient (VIQ) (94.17 ± 15.01 versus 103.91 ± 9.74, P = 0.006), and performance intelligence quotient (PIQ) (94.12 ± 11.04 versus 104.31 ± 10.05, P = 0.001), attention (98.48 ± 8.74 versus 106.87 ± 8.58, P < 0.001), and total OSA-18 scores (87.62 ± 17.15 versus 46.61 ± 10.15, P < 0.001) between before and after endoscopic adenoidectomy in OSAHS children. Conclusion. Our findings provided evidence that the intelligence, attention, and QoL of OSAHS children may be significantly improved after endoscopic adenoidectomy.

  13. N300 Indexes Deficient Integration of Orthographic and Phonological Representations in Children with Dyslexia

    ERIC Educational Resources Information Center

    Hasko, Sandra; Bruder, Jennifer; Bartling, Jurgen; Schulte-Korne, Gerd

    2012-01-01

    In transparent orthographies, like German, children with developmental dyslexia (DD) are mainly characterized by a reading fluency deficit. The reading fluency deficit might be traced back to a scarce integration of orthographic and phonological representations. In order to address this question, the present study used EEG to investigate the N300,…

  14. Association of PCB, PBDE and PCDD/F body burdens with hormone levels for children in an e-waste dismantling area of Zhejiang Province, China.

    PubMed

    Xu, Peiwei; Lou, Xiaoming; Ding, Gangqiang; Shen, Haitao; Wu, Lizhi; Chen, Zhijian; Han, Jianlong; Han, Guangen; Wang, Xiaofeng

    2014-11-15

    Increased electronic waste (e-waste) has raised public concerns regarding exposure to numerous toxic contaminants, particularly polychlorinated biphenyls (PCBs), polybrominated diphenyl ethers (PBDEs) and polychlorinated dibenzo-p-dioxins and dibenzofurans (PCDD/Fs). In China, the body burdens of PCBs, PBDEs and PCDD/Fs are associated with thyroid hormones in populations from e-waste dismantling sites; however, it is unclear whether this association occurs in children. In this study, we determined the serum levels of PCBs, PBDEs and PCDD/Fs and the endocrine hormones including free triiodothyronine (FT3), total triiodothyronine (TT3), free thyroxine (FT4), total thyroxine (TT4), thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), cortisol and growth hormone (GH) in 21 children from an e-waste dismantling area and 24 children from a control area. The results showed that the mean levels of ∑PCBs and ∑PBDEs in the exposure group were significantly higher than in the control group (40.56 and 32.09 ng g(-1) lipid vs. 20.69 and 8.43 ng g(-1) lipid, respectively, p<0.01 for each), and the mean level of ∑PCDD/Fs in the exposure group was higher than in the control group, but the difference was not significant (206.17 vs. 160.27 pg g(-1) lipid, p>0.05). For the endocrine hormones, we did not find significant differences between the exposed and control groups, although the mean levels of FT3, TT3, TT4, ACTH, cortisol and GH were higher, whereas the mean levels of FT4 and TSH were lower in the exposed group. The mean level of ∑PBDEs was positively correlated with the mean levels of ∑PCBs (r=0.60, p<0.05) and ∑PCDD/Fs (r=0.61, p<0.05). Furthermore, the mean level of ∑PBDEs was positively correlated with ACTH (r=0.61, p<0.05). In conclusion, our data suggested that exposure to e-waste dismantling environment increased the body burdens of PCBs and PBDEs in local children and that these contaminants released from the e-waste might contribute to

  15. Justified and unjustified use of growth hormone

    PubMed Central

    van der Lely, A J

    2004-01-01

    Growth hormone (GH) replacement therapy for children and adults with proven GH deficiency due to a pituitary disorder has become an accepted therapy with proven efficacy. GH is increasingly suggested, however, as a potential treatment for frailty, osteoporosis, morbid obesity, cardiac failure, and various catabolic conditions. However, the available placebo controlled studies have not reported many significant beneficial effects, and it might even be dangerous to use excessive GH dosages in conditions in which the body has just decided to decrease GH actions. GH can indeed induce changes in body composition that are considered to be advantageous to GH deficient and non-GH deficient subjects. In contrast to GH replacement therapy in GH deficient subjects, however, excessive GH action due to GH misuse seems to be ineffective in improving muscle power. Moreover, there are no available study data to indicate that the use of GH for non-GH deficient subjects should be advocated, especially as animal data suggest that lower GH levels are positively correlated with longevity. PMID:15466991

  16. Effect of recombinant human growth hormone on intestinal absorption and body composition in children with short bowel syndrome.

    PubMed

    Goulet, Olivier; Dabbas-Tyan, Myriam; Talbotec, Cécile; Kapel, Nathalie; Rosilio, Myriam; Souberbielle, Jean-Claude; Corriol, Odile; Ricour, Claude; Colomb, Virginie

    2010-01-01

    This prospective study aimed to establish the effect of recombinant human growth hormone (rhGH) on intestinal function in children with short bowel syndrome (SBS). Eight children with neonatal SBS were included. All were dependent on parenteral nutrition (PN) for >3 years (range, 3.8-11.6 years), with PN providing >50% of recommended dietary allowance for age (range, 50%-65%). The subjects received rhGH (Humatrope) 0.13 mg/kg/d subcutaneously over a 12-week period. The follow-up was continued over a 12-month period after rhGH discontinuation. Clinical and biological assessments were performed at baseline, at the end of the treatment period, and 12 months after the end of treatment. No side effects related to rhGH were observed. PN requirements were decreased in all children during the course of rhGH treatment. Between baseline and the end of treatment, significant increases were observed in concentrations (mean ± standard deviation) of serum insulin-like growth factor 1 (103.1 ± 49.9 µg/L vs 153.5 ± 82.2 µg/L; P < .01), serum insulin-like growth factor-binding protein 3 (1.7 ± 0.6 mg/L vs 2.5 ± 0.9 mg/L; P < .001), and plasma citrulline (16.5 ± 14.8 µmol/L vs 25.2 ± 18.3 µmol/L; P < .05). A median 54% increase in enteral intake (range, 10%-244%) was observed (P < .001) and net energy balance improved significantly (P < .002). It was necessary for 6 children to be maintained on PN or restarted after discontinuation of rhGH treatment, and they remained on PN until the end of the follow-up period. A 12-week high-dose rhGH treatment allowed patients to decrease PN, but only 2 patients could be definitively weaned from PN. Indications and cost-effectiveness of rhGH treatment for SBS pediatric patients need further evaluation.

  17. Prevalence of Vitamin D deficiency and associated risk factors among children residing at high altitude in Shimla district, Himachal Pradesh, India

    PubMed Central

    Kapil, Umesh; Pandey, Ravindra Mohan; Goswami, Ravinder; Sharma, Brij; Sharma, Neetu; Ramakrishnan, Lakshmy; Singh, Gajendra; Sareen, Neha; Sati, Hem Chandra; Gupta, Aakriti; Sofi, Nighat Yaseen

    2017-01-01

    Introduction: Vitamin D is important for bone development in children. A high prevalence of Vitamin D deficiency (VDD) has been documented from different parts of India. However, limited data are available on VDD among children residing at high altitude region of country. Objectives: To assess the prevalence of VDD and associated risk factors among children in the age group of 6–18 years in Shimla, Himachal Pradesh. Methods: A community-based cross-sectional study was conducted in the year 2014–2015. A total of 626 children in the age group of 6–18 years were enrolled from 30 clusters which were identified using population proportionate to size sampling method. A minimum of 20 children in the age group of 6–18 years per cluster were selected using random number tables. The data on socioeconomic status, physical activity, sunlight exposure, and biochemical parameters of bone and mineral metabolism were assessed. Results: Ninety-three percent of school-age children were found Vitamin D deficient as per serum 25(OH) D levels of <20 ng/ml. The prevalence was significantly higher among females. Conclusion: A high prevalence of VDD was found in children residing in high altitude region. PMID:28217519

  18. The usefulness of soluble transferrin receptor in the diagnosis and treatment of iron deficiency anemia in children

    PubMed Central

    Yoon, Se Hoon; Kim, Dong Sup; Yu, Seung Taek; Shin, Sae Ron

    2015-01-01

    Purpose Soluble transferrin receptor (sTfR) is a truncated extracellular form of the membrane transferrin receptor produced by proteolysis. Concentrations of serum sTfR are related to iron status and erythropoiesis in the body. We investigated whether serum sTfR levels can aid in diagnosis and treatment of iron deficiency anemia (IDA) in children. Methods Ninety-eight patients with IDA were enrolled and were classified according to age at diagnosis. Group 1 comprised 78 children, aged 6-59 months, and group 2 comprised 20 adolescents, aged 12-16 years. Results In group 1, patients' serum sTfR levels correlated negatively with mean corpuscular volume; hemoglobin (Hb), ferritin, and serum iron levels; and transferrin saturation and positively with total iron binding capacity (TIBC) and red cell distribution width. In group 2, patients' serum sTfR levels did not correlate with ferritin levels and TIBC, but had a significant relationship with other iron indices. Hb and serum sTfR levels had a significant inverse relationship in both groups; however, in group 1, there was no correlation between Hb and serum ferritin levels. In 30 patients of group 1, serum sTfR levels were significantly decreased with an increase in Hb levels after iron supplementation for 1 month. Conclusion Serum sTfR levels significantly correlated with other diagnostic iron parameters of IDA and inversely correlated with an increase in Hb levels following iron supplementation. Therefore, serum sTfR levels can be a useful marker for the diagnosis and treatment of IDA in children. PMID:25729394

  19. Are Children with Asthma in South Korea Also Associated with Vitamin D Deficiency?

    PubMed

    Kim, Yu-Ri; Seo, Sung-Chul; Yoo, Young; Choung, Ji Tae

    2017-02-14

    Recently, epidemiologic studies have shown that the lack of vitamin D levels may be associated with high asthma prevalence, but its effect is still controversial, depending on season, area, and food consumption. We aimed to examine the association of serum vitamin D levels with the prevalence of pediatric asthma in Korea. A total of 80 children (50 asthmatic children and 30 healthy controls) aged 6-14 years were participated in this study. Serum 25-hydroxy vitamin D (vitamin D) levels were measured and compared between the two groups. Moreover, the relationship of serum vitamin D levels with results of pulmonary function test and environmental factors (lifestyle habits and residential factors) collected by a questionnaire survey were examined in asthmatic patients. Serum vitamin D levels in asthmatic children (16.63 ± 4.20 ng/mL) were significantly lower than that in healthy controls (24.24 ± 6.76 ng/mL) (P<0.05). Also, we found that the prevalence of asthma increase to 0.79-fold (OR, 0.788; 95% C.I., 0.707-0.879; P<0.001) as serum vitamin D level is 1 ng/mL decreases. The increased time spent in outdoor could affect the increases of vitamin D levels significantly. However, no associations of vitamin D level with pulmonary function and residential environmental factors (i.e., housing type, living floor, and indoor activity time) were observed. Our findings suggest that serum vitamin D levels were also associated with pediatric asthma in Korea. Moreover, management of vitamin D level in asthmatic children would be a promising approach for preventing exaggeration of their severity.

  20. Sugar - hormone crosstalk in seed development: Two redundant pathways of IAA biosynthesis are regulated differentially in the invertase-deficient miniature1 (mn1) seed mutant in maize

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The miniature1 (mn1) seed phenotype is a loss-of-function mutation at the Mn1 locus that encodes a cell wall invertase; its deficiency leads to pleiotropic changes including altered sugar levels and decreased levels of IAA throughout seed development. To understand the molecular details of such suga...

  1. Intake of Phthalate-tainted Foods and Serum Thyroid Hormones in Taiwanese Children and Adolescents.

    PubMed

    Tsai, Hui-Ju; Wu, Chia-Fang; Tsai, Yi-Chun; Huang, Po-Chin; Chen, Mei-Lien; Wang, Shu-Li; Chen, Bai-Hsiun; Chen, Chu-Chih; Wu, Wen-Chiu; Hsu, Pi-Shan; Hsiung, Chao A; Wu, Ming-Tsang

    2016-07-29

    On April-May, 2011, phthalates, mainly Di-(2-ethylhexyl) phthalate (DEHP), were deliberately added to a variety of foodstuff as a substitute emulsifier in Taiwan. This study investigated the relationship between DEHP-tainted foodstuffs exposure and thyroid function in possibly affected children and adolescents. Two hundred fifty participants <18 years possibly exposed to DEHP were enrolled in this study between August 2012 and January 2013. Questionnaires were used to collect details on their past exposure to DEHP-tainted food items. Blood and urine samples were collected for biochemical workups to measure current exposure derived from three urinary DEHP metabolites using a creatinine excretion-based model. More than half of 250 participants were estimated to be exposed to DEHP-tainted foods found to exceed the recommend tolerable daily intake of DEHP established by the European Food Safety Authority (<50 μg/kg/day). The median daily DEHP intake (DDI) among those 250 participants was 46.52 μg/kg/day after multiple imputation. This value was ~10-fold higher than the current median DEHP intake (4.46 μg/kg/day, n = 240). Neither past nor current DEHP exposure intensity was significantly associated with serum thyroid profiles. Future studies may want to follow the long-term health effects of this food scandal in affected children and adolescents.

  2. Intake of Phthalate-tainted Foods and Serum Thyroid Hormones in Taiwanese Children and Adolescents

    NASA Astrophysics Data System (ADS)

    Tsai, Hui-Ju; Wu, Chia-Fang; Tsai, Yi-Chun; Huang, Po-Chin; Chen, Mei-Lien; Wang, Shu-Li; Chen, Bai-Hsiun; Chen, Chu-Chih; Wu, Wen-Chiu; Hsu, Pi-Shan; Hsiung, Chao A.; Wu, Ming-Tsang

    2016-07-01

    On April-May, 2011, phthalates, mainly Di-(2-ethylhexyl) phthalate (DEHP), were deliberately added to a variety of foodstuff as a substitute emulsifier in Taiwan. This study investigated the relationship between DEHP-tainted foodstuffs exposure and thyroid function in possibly affected children and adolescents. Two hundred fifty participants <18 years possibly exposed to DEHP were enrolled in this study between August 2012 and January 2013. Questionnaires were used to collect details on their past exposure to DEHP-tainted food items. Blood and urine samples were collected for biochemical workups to measure current exposure derived from three urinary DEHP metabolites using a creatinine excretion-based model. More than half of 250 participants were estimated to be exposed to DEHP-tainted foods found to exceed the recommend tolerable daily intake of DEHP established by the European Food Safety Authority (<50 μg/kg/day). The median daily DEHP intake (DDI) among those 250 participants was 46.52 μg/kg/day after multiple imputation. This value was ~10-fold higher than the current median DEHP intake (4.46 μg/kg/day, n = 240). Neither past nor current DEHP exposure intensity was significantly associated with serum thyroid profiles. Future studies may want to follow the long-term health effects of this food scandal in affected children and adolescents.

  3. Effects of Gender on the Association of Urinary Phthalate Metabolites with Thyroid Hormones in Children: A Prospective Cohort Study in Taiwan

    PubMed Central

    Weng, Te-I; Chen, Mei-Huei; Lien, Guang-Wen; Chen, Pai-Shan; Lin, Jasper Chia-Cheng; Fang, Cheng-Chung; Chen, Pau-Chung

    2017-01-01

    Phthalates are considered endocrine disruptors. Our study assessed the gender-specific effects of phthalate exposure on thyroid function in children. In total, 189 Taiwanese children were enrolled in the study. One-spot urine and blood samples were collected for analyzing 12 phthalate metabolites in urine and thyroid hormones. The association between urinary phthalate metabolites and serum thyroid hormones was determined using a generalized linear model with a log link function; the children were categorized into groups for analysis according to the 33rd and 66th percentiles. The data were stratified according to gender and adjusted for a priori defined covariates. In girls, a positive association existed between urinary di-2-ethylhexyl phthalate (DEHP) metabolites (mono-(2-ethylhexyl) phthalate, mono-(2-ethyl-5-oxohexyl) phthalate, and mono-(2-ethyl-5-hydroxyhexyl) phthalate) and free thyroxine (T4). In boys, urinary dibutyl phthalate (DBP) metabolites (mono-i-butyl phthalate and mono-n-butyl phthalate) were positively associated with free triiodothyronine (T3). After categorizing each exposure into three groups, urinary DEHP metabolites were positively associated with free T3 levels in boys. Our results suggested that DEHP is associated with free T4 in girls and that DBP is associated with free T3 in boys. Higher DEHP metabolite concentrations exerted larger effects on free T3 in boys. These results reveal the gender-specific relationships between phthalate metabolites and thyroid hormones. PMID:28146055

  4. [Hormone replacement therapy--growth hormone, melatonin, DHEA and sex hormones].

    PubMed

    Fukai, Shiho; Akishita, Masahiro

    2009-07-01

    The ability to maintain active and independent living as long as possible is crucial for the healthy longevity. Hormones responsible for some of the manifestations associated with aging are growth hormone, insulin-like growth factor-1 (IGF-1), melatonin, dehydroepiandrosterone (DHEA), sex hormones and thyroid hormones. These hormonal changes are associated with changes in body composition, visceral obesity, muscle weakness, osteoporosis, urinary incontinence, loss of cognitive functioning, reduction in well being, depression, as well as sexual dysfunction. With the prolongation of life expectancy, both men and women today live the latter third life with endocrine deficiencies. Hormone replacement therapy may alleviate the debilitating conditions of secondary partial endocrine deficiencies by preventing or delaying some aspects of aging.

  5. Effects of partial and total colectomy on mineral and acid-base homoeostasis in the rat: magnesium deficiency, hyperphosphaturia and osteopathy, in the presence of high serum 1,25-dihydroxyvitamin D but normal parathyroid hormone.

    PubMed

    Croner, R; Schwille, P O; Erben, R G; Gepp, H; Stahr, K; Rümenapf, G; Parth, R; Scheuerlein, H

    2000-06-01

    The effects of colectomy on acid-base status, extra-osseous and bone minerals, calciotropic hormones and bone morphology have not yet been studied. To rectify this, groups of normally fed male rats were subjected to distal (n=11), proximal (n=12) or total (n=12) colectomy. Sham-operated rats (n=12) served as controls. At 112 (+/-2) days after colectomy the following changes were noted: (1) weight gain was delayed; (2) faecal excretion of calcium and phosphorus was normal, whereas that of magnesium was increased; (3) intestinal calcium secretion and absorption of calcium and phosphorus were normal, but magnesium absorption was decreased; (4) urinary excretion of magnesium was also decreased, that of phosphorus was increased, and that of pyridinium and deoxypyridinium tended to be high; (5) the serum levels of ionized magnesium, total calcium, 25-hydroxyvitamin D and parathyroid hormone were normal, while that of 1,25-dihydroxyvitamin D was markedly elevated; and (6) bone magnesium and phosphorus content were decreased, but bone calcium was normal, and thus the bone calcium/phosphorus ratio was high. These abnormalities were associated with moderate metabolic acidosis, as reflected by high urinary ammonium, low citrate and low total CO(2), but normal blood gases. Significant structural abnormalities of bone were not detectable, but trabecular bone tended to show rarefication. Distal colectomy had the least effect, whereas proximal and total colectomies had a distinct effect, on these parameters. It is concluded that colectomy in the rat causes: (1) a syndrome of magnesium deficiency of intestinal origin, compensated metabolic acidosis, urinary phosphorus loss, and high circulating 1,25-dihydroxyvitamin D levels, with the degree depending on the extent of surgical resection; and (2) brittle bones, a feature characteristic of low bone magnesium and more generalized magnesium deficiency. The mechanisms leading to this syndrome are unknown, but altered tissue levels of

  6. Evaluation of appetite-stimulating hormones in prepubertal children with epilepsy during topiramate treatment.

    PubMed

    Okuyaz, Cetin; Kursel, Onur; Komur, Mustafa; Tamer, Lulufer

    2012-12-01

    We investigated the mechanism of topiramate-related appetite loss and exposed its relationship to body weight, body mass index, body fat index, and serum insulin, lipid, leptin, neuropeptide-Y, cortisol, ghrelin, and adiponectin levels. Twenty children with epilepsy were evaluated at baseline and months 3 and 6 of treatment. Their body fat index, leptin, and neuropeptide-Y levels significantly decreased at month 3, whereas significant decreases occurred in body weight, body mass index, body fat index, neuropeptide-Y, cholesterol, and cortisol levels of patients at month 6 compared with baseline. Weight loss during topiramate treatment was attributed to loss of appetite and reduced food intake caused by reductions in neuropeptide-Y. To the best of our knowledge, this study is the first to describe reductions in neuropeptide-Y with topiramate use in humans.

  7. Vitamin D deficiency and sufficiency among Canadian children residing at high latitude following the revision of the RDA of vitamin D intake in 2010.

    PubMed

    Munasinghe, Lalani L; Yuan, Yan; Willows, Noreen D; Faught, Erin L; Ekwaru, John P; Veugelers, Paul J

    2017-03-01

    Recently, countries at high latitudes have updated their vitamin D recommendations to ensure adequate intake for the musculoskeletal health of their respective populations. In 2010, the dietary guidelines for vitamin D for Canadians and Americans aged 1-70 years increased from 5 μg/d to 15 μg/d, whereas in 2016 for citizens of the UK aged ≥4 years 10 μg/d is recommended. The vitamin D status of Canadian children following the revised dietary guidelines is unknown. Therefore, this study aimed to assess the prevalence and determinants of vitamin D deficiency and sufficiency among Canadian children. For this study, we assumed serum 25-hydroxy vitamin D (25(OH)D) concentrations <30 nmol/l as 'deficient' and ≥50nmol/l as 'sufficient'. Data from children aged 3-18 years (n 2270) who participated in the 2012/2013 Canadian Health Measures Survey were analysed. Of all children, 5·6 % were vitamin D deficient and 71 % were vitamin D sufficient. Children who consumed vitamin D-fortified milk daily (77 %) were more likely to be sufficient than those who consumed it less frequently (OR 2·7; 95 % CI 1·4, 5·0). The 9 % of children who reported taking vitamin D-containing supplements in the previous month had higher 25(OH)D concentrations (OR 6·9 nmol/l; 95 % CI 1·1, 12·7 nmol/l) relative to those who did not. Children who were older, obese, of non-white ethnicity and from low-income households were less likely to be vitamin D sufficient. To improve vitamin D status, consumption of vitamin D-rich foods should be promoted, and fortification of more food items or formal recommendations for vitamin D supplementation should be considered.

  8. Megestrol acetate to correct the nutritional status in an adolescent with growth hormone deficiency: Increase of appetite and body weight but only by increase of body water and fat mass followed by profound cortisol and testosterone depletion.

    PubMed

    Schmid, I; Stachel, D K; Freudenberg, S; Schmitt, M; Schuster, F; Haas, R J

    2002-01-01

    Megestrol acetate (MA) is a synthetic, orally active derivative of the naturally occurring hormone progesterone. MA is increasingly used to correct loss of appetite and improve the nutritional status. We used MA in an adolescent with growth hormone (GH) deficiency due to former irradiation therapy in order to evaluate if MA can improve the nutritional status. In fact, MA increased appetite and weight dose-dependent. The energy expenditure measured by indirect calorimetry changed from hypo- to normometabolism. However, weight gain was first primarily due to an increase in body water and then in fat mass. The gain of fat mass was much more prominent than the gain of fat free mass. As important side-effect, MA lead to rapid and profound cortisol and testosterone depletion after only 10 days with a long-lasting effect on testosterone depletion. Therefore, MA as a single therapy cannot be recommended to improve the nutritional status. If MA is given, cortisol and testosterone levels have to be monitored and supplemented as needed.

  9. Iodine deficiency: Clinical implications.

    PubMed

    Niwattisaiwong, Soamsiri; Burman, Kenneth D; Li-Ng, Melissa

    2017-03-01

    Iodine is crucial for thyroid hormone synthesis and fetal neurodevelopment. Major dietary sources of iodine in the United States are dairy products and iodized salt. Potential consequences of iodine deficiency are goiter, hypothyroidism, cretinism, and impaired cognitive development. Although iodine status in the United States is considered sufficient at the population level, intake varies widely across the population, and the percentage of women of childbearing age with iodine deficiency is increasing. Physicians should be aware of the risks of iodine deficiency and the indications for iodine supplementation, especially in women who are pregnant or lactating.

  10. Potential Contribution of Iron Deficiency and Multiple Factors to Anemia Among 6- to 72-Month-Old Children in the Kokang Area of Myanmar.

    PubMed

    Zhao, Ai; Gao, Hongchong; Li, Bo; Yu, Kai; Win, Naing Naing; Zhang, Yumei; Wang, Peiyu

    2015-10-01

    The prevalence of anemia among children in Myanmar has been reported to be among the highest in the world. This study was conducted to determine 1) the prevalence of anemia in preschool children and 2) risk factors associated with anemia. A total of 138 children aged from 6 to 72 months were recruited through cluster sampling from six villages in Kokang. Hemoglobin (Hb) concentration, blood trace elements, and anthropometric indicators were measured. Feces samples were collected to examine for the presence of ascarid eggs. The overall prevalence of anemia in children was 61.6%, including 10.9% with severe anemia. Meanwhile, high prevalence of stunting (40.0%), underweight (22.4%), wasting (6.3%), and small head circumference (6.7%) was found. Children with anemia were more prone to stunting. Children with severe anemia and moderate anemia had significantly lower blood iron and zinc levels than children without anemia (P < 0.001 and P = 0.007). The prevalence of ascarid infection was 64.9%; however, it was not associated with anemia. Drinking spring water was positively associated with anemia (odds ratio [OR] = 6.368). This study demonstrated that anemia is an important public health problem among children from the Kokang area. Iron deficiency and drinking spring water may be the important causes of anemia among children.

  11. Potential Contribution of Iron Deficiency and Multiple Factors to Anemia among 6- to 72-Month-Old Children in the Kokang Area of Myanmar

    PubMed Central

    Zhao, Ai; Gao, Hongchong; Li, Bo; Yu, Kai; Win, Naing Naing; Zhang, Yumei; Wang, Peiyu

    2015-01-01

    The prevalence of anemia among children in Myanmar has been reported to be among the highest in the world. This study was conducted to determine 1) the prevalence of anemia in preschool children and 2) risk factors associated with anemia. A total of 138 children aged from 6 to 72 months were recruited through cluster sampling from six villages in Kokang. Hemoglobin (Hb) concentration, blood trace elements, and anthropometric indicators were measured. Feces samples were collected to examine for the presence of ascarid eggs. The overall prevalence of anemia in children was 61.6%, including 10.9% with severe anemia. Meanwhile, high prevalence of stunting (40.0%), underweight (22.4%), wasting (6.3%), and small head circumference (6.7%) was found. Children with anemia were more prone to stunting. Children with severe anemia and moderate anemia had significantly lower blood iron and zinc levels than children without anemia (P < 0.001 and P = 0.007). The prevalence of ascarid infection was 64.9%; however, it was not associated with anemia. Drinking spring water was positively associated with anemia (odds ratio [OR] = 6.368). This study demonstrated that anemia is an important public health problem among children from the Kokang area. Iron deficiency and drinking spring water may be the important causes of anemia among children. PMID:26195457

  12. Neuroplastic effects of music lessons on hippocampal volume in children with congenital hypothyroidism.

    PubMed

    Zendel, Benjamin Rich; Willoughby, Karen A; Rovet, Joanne F

    2013-12-04

    Children with congenital hypothyroidism (CH) who experience a neonatal thyroid hormone deficiency have reduced hippocampal volumes compared with healthy controls. Interestingly, evidence suggests that musical training can contribute to structural plasticity in a number of brain areas, including the hippocampus. Therefore, we investigated whether taking music lessons could ameliorate the volumetric reductions of the hippocampus in children with CH. Left and right hippocampal volumes were measured in four groups of children: children with CH with and without music lessons, and healthy controls with and without music lessons. We found that the volume of the right hippocampus was comparable between children with CH who had taken music lessons and the healthy controls. Children with CH who had not taken music lessons had reduced hippocampal volumes compared with the other three groups. These results suggest that music lessons may induce structural neuroplasticity in children with atypical hippocampal development because of early thyroid hormone deficiencies.

  13. Young Zanzibari Children with Iron Deficiency, Iron Deficiency Anemia, Stunting, or Malaria Have Lower Motor Activity Scores and Spend Less Time in Locomotion

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Motor activity improves cognitive and social-emotional development through a child’s exploration of his or her physical and social environment. This study assessed anemia, iron deficiency, hemoglobin (Hb), length-for-age Z-score (LAZ), and malaria infection as predictors of motor activity in 771 chi...

  14. Growth hormone (GH)-transgenic insulin-like growth factor 1 (IGF1)-deficient mice allow dissociation of excess GH and IGF1 effects on glomerular and tubular growth.

    PubMed

    Blutke, Andreas; Schneider, Marlon R; Wolf, Eckhard; Wanke, Rüdiger

    2016-03-01

    Growth hormone (GH)-transgenic mice with permanently elevated systemic levels of GH and insulin-like growth factor 1 (IGF1) reproducibly develop renal and glomerular hypertrophy and subsequent progressive glomerulosclerosis, finally leading to terminal renal failure. To dissociate IGF1-dependent and -independent effects of GH excess on renal growth and lesion development in vivo, the kidneys of 75 days old IGF1-deficient (I(-/-)) and of IGF1-deficient GH-transgenic mice (I(-/-)/G), as well as of GH-transgenic (G) and nontransgenic wild-type control mice (I(+/+)) were examined by quantitative stereological and functional analyses. Both G and I(-/-)/G mice developed glomerular hypertrophy, hyperplasia of glomerular mesangial and endothelial cells, podocyte hypertrophy and foot process effacement, albuminuria, and glomerulosclerosis. However, I(-/-)/G mice exhibited less severe glomerular alterations, as compared to G mice. Compared to I(+/+) mice, G mice exhibited renal hypertrophy with a significant increase in the number without a change in the size of proximal tubular epithelial (PTE) cells. In contrast, I(-/-)/G mice did not display significant PTE cell hyperplasia, as compared to I(-/-) mice. These findings indicate that GH excess stimulates glomerular growth and induces lesions progressing to glomerulosclerosis in the absence of IGF1. In contrast, IGF1 represents an important mediator of GH-dependent proximal tubular growth in GH-transgenic mice.

  15. Recognition of medium-chain acyl-CoA dehydrogenase deficiency in asymptomatic siblings of children dying of sudden infant death or Reye-like syndromes.

    PubMed

    Roe, C R; Millington, D S; Maltby, D A; Kinnebrew, P

    1986-01-01

    The medium-chain acyl-CoA dehydrogenase (MCAD) deficiency of mitochondrial beta oxidation has been identified in two asymptomatic siblings in a family in which two previous deaths had been recorded, one attributed to sudden infant death syndrome and the other to Reye syndrome. Recognition of this disorder in one of the deceased and in the surviving siblings was accomplished by detection of a diagnostic metabolite, octanoylcarnitine, using a new mass spectrometric technique. This resulted in early treatment with L-carnitine supplement in the survivors, which should prevent metabolic deterioration. Further studies suggest that breast-feeding may be protective for infants with MCAD deficiency. Families with children who have had Reye syndrome or in which sudden infant death has occurred are at risk for MCAD deficiency. We suggest that survivors and asymptomatic siblings should be tested for this treatable disorder.

  16. Growth, chronic kidney disease and pediatric kidney transplantation: is it useful to use recombinant growth hormone in Colombian children with renal transplant?

    PubMed

    Castañeda, D A; López, L F; Ovalle, D F; Buitrago, J; Rodríguez, D; Lozano, E

    2011-11-01

    Kidney transplantation has become the best treatment for children with chronic kidney disease (CKD). In recent times, knowledge concerning the effect of CKD and kidney transplantation over the normal growth rate has increased; now it is known that 40% of children with CKD do not reach the expected height for age. Growth retardation has been associated with the type of nephropathy, metabolic and endocrine disorders that are secondary to kidney disease, immunosuppressive therapy with glucocorticoids, and suboptimal function of renal allograft. Nowadays, we know better the role of the growth hormone/insulin-like growth factor 1 axis in growth retardation we can see it in children with CKD or recipients of renal allograft. Several studies have shown that administration of recombinant growth hormone (rhGH) has a positive effect on the longitudinal growth of children and teenagers who have received a kidney transplant. On the other hand, there have been reported side effects associated with using rhGH; however, these are not statistically significant. In this article, we show a small review about growth in children with CKD and/or recipients of renal allografts the growth pattern of three children who were known by the Transplant Group of National University of Colombia, and the results obtained with the use of rhGH in one of these cases. We want to show the possibility of achieving a secure use of rhGH in children with CKD and its use as a therapeutic option for treating the growth retardation in children with kidney transplantation, and set out the need of typifying the growth pattern of Colombian children with CKD and/or who are recipients of renal allografts through multicenter studies to propose and analyze the inclusion of rhGH in the therapeutic scheme of Colombian children with these two medical conditions. rhGH could be a useful tool for treating children with CKD or kidney transplantation who have not reached the expected longitudinal growth for age. However

  17. High dickkopf-1 levels in sera and leukocytes from children with 21-hydroxylase deficiency on chronic glucocorticoid treatment.

    PubMed

    Brunetti, Giacomina; Faienza, Maria Felicia; Piacente, Laura; Ventura, Annamaria; Oranger, Angela; Carbone, Claudia; Benedetto, Adriana Di; Colaianni, Graziana; Gigante, Margherita; Mori, Giorgio; Gesualdo, Loreto; Colucci, Silvia; Cavallo, Luciano; Grano, Maria

    2013-03-01

    Children with 21-hydroxylase deficiency (21-OHD) need chronic glucocorticoid (cGC) therapy to replace congenital deficit of cortisol synthesis, and this therapy is the most frequent and severe form of drug-induced osteoporosis. In this study, we enrolled 18 patients (9 females) and 18 sex- and age-matched controls. We found in 21-OHD patients high serum and leukocyte levels of dickkopf-1 (DKK1), a secreted antagonist of the Wnt/β-catenin signaling pathway known to be a key regulator of bone mass. In particular, we demonstrated by flow cytometry, confocal microscopy, and real-time PCR that monocytes, T lymphocytes, and neutrophils from patients expressed high levels of DKK1, which may be related to the cGC therapy. In fact, we showed that dexamethasone treatment markedly induced the expression of DKK1 in a dose- and time-dependent manner in leukocytes. The serum from patients containing elevated levels of DKK1 can directly inhibit in vitro osteoblast differentiation and receptor activator of NF-κB ligand (RANKL) expression. We also found a correlation between both DKK1 and RANKL or COOH-terminal telopeptides of type I collagen (CTX) serum levels in 21-OHD patients on cGC treatment. Our data indicated that DKK1, produced by leukocytes, may contribute to the alteration of bone remodeling in 21-OHD patients on cGC treatment.

  18. Classical and non-classical causes of GH deficiency in the paediatric age.

    PubMed

    Di Iorgi, Natascia; Morana, Giovanni; Allegri, Anna Elsa Maria; Napoli, Flavia; Gastaldi, Roberto; Calcagno, Annalisa; Patti, Giuseppa; Loche, Sandro; Maghnie, Mohamad

    2016-12-01

    Growth hormone deficiency (GHD) may result from a failure of hypothalamic GHRH production or release, from congenital disorders of pituitary development, or from central nervous system insults including tumors, surgery, trauma, radiation or infiltration from inflammatory diseases. Idiopathic, isolated GHD is the most common sporadic form of hypopituitarism. GHD may also occur in combination with other pituitary hormone deficiencies, and is often referred to as hypopituitarism, combined pituitary hormone deficiency (CPHD), multiple pituitary hormone deficiency (MPHD) or panhypopituitarism. Children