Inappropriate Leptin Secretion in Thalassemia: A Potential Cofactor of Pubertal Timing Derangement

Perrone, Laura; Perrotta, Silverio; Raimondo, Paolo; Mucerino, J.; Rosa, Carla de Oliveira Barbosa; Siciliani, Maria Carmen; Santoro, Nicola; Giudice, Emanuele Miraglia del

doi:10.1515/jpem.2003.16.6.877

Cited by 15 publications

(15 citation statements)

References 2 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Three other patients with volume loss and hypogonadism had only modest pituitary iron overload ( Z < 4). We cannot exclude the possibility that chronic anemia, increased metabolic demand, or other physiologic stressors may contribute to decreased pituitary volume in TM patients; these factors could influence hypothalamic function, for example, by suppressing leptin levels [19–22]. Alternatively, these patients may have had severe pituitary iron overload in the past, suffered irreversible damage, and were subsequently chelated to a normal or less severe pituitary iron levels.…”

Section: Discussionmentioning

confidence: 99%

Pituitary iron and volume predict hypogonadism in transfusional iron overload

et al. 2011

View full text Add to dashboard Cite

Hypogonadism is the most common morbidity in patients with transfusion-dependent anemias such as thalassemia major. We used magnetic resonance imaging (MRI) to measure pituitary R2 (iron) and volume to determine at what age these patients develop pituitary iron overload and volume loss. We recruited 56 patients (47 with thalassemia major, five with chronically transfused thalassemia intermedia and four with Blackfan-Diamond syndrome) to have pituitary MRIs to measure pituitary R2 and volume. Hypogonadism was defined clinically based on the timing of secondary sexual characteristics or the need for sex hormone replacement therapy. Patients with transfusional iron overload begin to develop pituitary iron overload in the first decade of life; however, clinically significant volume loss was not observed until the second decade of life. Severe pituitary iron deposition (Z > 5) and volume loss (Z < -2.5) were independently predictive of hypogonadism. Pituitary R2 correlated significantly with serum ferritin as well as liver, pancreatic, and cardiac iron deposition by MRI. Log pancreas R2* was the best single predictor for pituitary iron, with an area under the receiving operator characteristic curve of 0.88, but log cardiac R2* and ferritin were retained on multivariate regression with a combined r(2) of 0.71. Pituitary iron overload and volume loss were independently predictive of hypogonadism. Many patients with moderate-to-severe pituitary iron overload retained normal gland volume and function, representing a potential therapeutic window. The subset of hypogonadal patients having preserved gland volumes may also explain improvements in pituitary function observed following intensive chelation therapy.

show abstract

Section: Discussionmentioning

confidence: 99%

Pituitary iron and volume predict hypogonadism in transfusional iron overload

et al. 2011

View full text Add to dashboard Cite

show abstract

“…Only leptin has been studied in thalassaemic patients. In recent studies, leptin levels have been evaluated in relation to puberty and reproductive function [18,19,20,21] as well as haematopoietic activity. In all studies, leptin was significantly lower in thalassaemic patients, and the positive correlation of BMI with leptin levels was statistically significant only in healthy controls, as observed in our study group.…”

Section: Discussionmentioning

confidence: 99%

Serum Adipocytokine and Vascular Inflammation Marker Levels in Beta-Thalassaemia Major Patients

Chaliasos

Challa²,

Hatzimichael³

et al. 2010

Acta Haematol

View full text Add to dashboard Cite

Background/Aim: The adipocytokines leptin and adiponectin represent a critical link between metabolism, immunity and chronic inflammation. A chronic vascular inflammatory state plays an important role in the pathophysiology of thalassaemia. We aimed to analyze the levels of these adipocytokines and determine any possible correlations with disease severity or vascular inflammation markers in beta-thalassaemia. Methods: Serum leptin, adiponectin, high-sensitivity C-reactive protein, endothelins, vascular adhesion molecule-1, intracellular adhesion molecule-1 and L- and E-selectin were measured in 28 beta-thalassaemia patients and compared with levels in healthy controls. Results: Leptin was significantly lower in patients compared to controls (2.23 ± 1.8 vs. 10.24 ± 5.78 µg/l; p = 0.0018), whereas adiponectin was elevated (11.75 ± 5.67 vs. 6.83 ± 2.75 µg/l; p = 0.009). For both adipocytokines, no correlations were found with characteristics such as age, gender, type of chelation, body mass index z score or haemoglobin. Leptin, but not adiponectin, was negatively correlated with ferritin (p = 0.032, r = –0.61). No correlations were found between leptin and the inflammation markers. However, adiponectin was positively correlated with endothelin-1 (p = 0.022, r = 0.63). Conclusions: Serum leptin is low in beta-thalassaemia, perhaps due to the toxic effect of iron overload on adipose tissue. Paradoxically, adiponectin levels are high and positively correlated with endothelin-1, raising questions about the pro- or anti-inflammatory role of this adipocytokine in beta-thalassaemia.

show abstract

“…12 This hormone primarily acts on the hypothalamus and its deficiency in the ob/ob mouse results in persistent immaturity as a result of hypothalamic-pituitary malfunction. 13 To our knowledge, several studies have been published on leptin levels in different age groups of thalassemic patients [14][15][16] and in all of them low leptin levels were observed, with variable conclusions being reached depending on the study design and the patient cohort.…”

Section: Introductionmentioning

confidence: 99%

Ghrelin and leptin levels in relation to puberty and reproductive function in patients with beta-thalassemia

Moshtaghi-Kashanian¹,

Razavi²

2009

View full text Add to dashboard Cite

OBJECTIVE:Recently published animal studies indicate that leptin and ghrelin play a role in puberty initiation and progress as well as in reproduction. The aim of our study was to evaluate the relation of these two hormones to the pubertal maturity and fertility status in patients with β-thalassemia. DESIGN: Blood levels of leptin, acylated ghrelin and sex hormones were determined in 97 (59 males and 38 females) beta-thalassemic patients, aged 18-23 years and in 50 healthy subjects (27 males and 23 females) matched for age. Body Mass Index (BMI) was also assessed. RESULTS: Besides lower BMI, all the hormones evaluated were significantly lower in β-thalassemic patients compared to controls (p<0.001). Furthermore, the leptin/ghrelin ratio in female patients was lower than the values obtained in the controls (p<0.001). Finally, significant negative correlations (p=0.050) were detected between circulating levels of acylatedghrelin and LH, FSH and sex hormones in female and male patients. CONCLUSIONS: The lower values of leptin and ghrelin in patients with β-thalassemia possibly constitute another hormonal imbalance which may contribute to the phenotype of impaired growth and sexual maturation encountered in these patients. The findings on the ghrelin levels constitute a novel observation.

show abstract

Inappropriate Leptin Secretion in Thalassemia: A Potential Cofactor of Pubertal Timing Derangement

Cited by 15 publications

References 2 publications

Pituitary iron and volume predict hypogonadism in transfusional iron overload

Pituitary iron and volume predict hypogonadism in transfusional iron overload

Serum Adipocytokine and Vascular Inflammation Marker Levels in Beta-Thalassaemia Major Patients

Ghrelin and leptin levels in relation to puberty and reproductive function in patients with beta-thalassemia

Contact Info

Product

Resources

About