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See detailHypogonadisme Hypogonadotrope: sémiologie, physiopathologie, avancées génétiques et prises en charge
VALDES SOCIN, Hernan Gonzalo ULg

Scientific conference (2015, November 27)

Le contrôle neuroendocrinien de la reproduction chez les mammifères est régi par un réseau de neurones hypothalamiques d'environ 1500 neurones à GnRH, qui modulent l’activité de l'axe de la reproduction ... [more ▼]

Le contrôle neuroendocrinien de la reproduction chez les mammifères est régi par un réseau de neurones hypothalamiques d'environ 1500 neurones à GnRH, qui modulent l’activité de l'axe de la reproduction au cours de la vie. Plus récemment on a décrit une modulation du générateur à GnrH par des neurones à GnIH, kisspeptines et neurokinin B. L'hypogonadisme hypogonadotrope se décline en acquis (post traumatisme cranien ou TBI, post radiothérapie, associé à des maladies métaboliques ou inflammatoires, etc) et hypogonadisme hypogonadotrope congénital (HHC). Le HHC est un syndrome clinique qui est caractérisé par une insuffisance pubertaire partielle ou complète. Le HH congénital peut résulter d'une insuffisance hypothalamique de la sécrétion/action du GnRH ou d'une insuffisance de sécrétion/effets des gonadotrophines hypophysaires LH et FSH. Chez l'homme, plusieurs gènes qui participent à l'olfaction et à la migration neuronale de GnRH interagissent pendant l’embryogénèse et le développement fœtal. Un nombre grandissant de mutations de ces gènes est responsable de l’ HH congénital. Basé sur la présence ou l'absence de troubles de l'olfaction, le HH est divisé en deux syndromes : HH avec anosmie/hyposmie (le syndrome de Kallmann) et l’ hypogonadisme hypogonadotrophique normosmique isolé (HHnI). Le syndrome de Kallmann (KS) est une maladie hétérogène qui affecte 1 personne sur 5000, avec 3 à 5 fois plus d’hommes que de femmes. Le KS est associé à des mutations de gènes qui sont principalement liées à des défauts de la migration neuronale. Ces défauts reproductifs et olfactifs comprennent un phénotype variable, y compris une surdité neurosensorielle, un colobome, des syncinéties controlatérales bimanuelles, des malformations crâniofaciales et une agénésie rénale. Fait intéressant, les mutations invalidantes de certains gènes responsables du KS : PROKR2, FGFR1, FGF8, CHD7, DUSP6 et WDR11, sont également associées à un hypogonadisme IHH normosmique, tandis que des mutations KISS1/KISSR, TAC3/TACR3, GNRH1/GNRHR, LEP/LEPR, HESX1, βFSH et βLH (syndrome de Pasqualini) ne sont présentes que chez les patients atteints de IHH normosmique. Dans ce cours interuniversitaire Ulg-UlB, nous exposons les aspects cliniques , physiopathologiques, les avancées génétiques et la prise en charge de l'hypogonadisme hypogonadotrope en pathologie humaine. [less ▲]

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See detailHypogonadisme par déficience de LH.
Beckers, Albert ULg

in Bulletin et Mémoires de l'Académie Royale de Médecine de Belgique (2007), 162(5-6), 291-7298

A 30-year-old man was investigated for delayed puberty and infertility. These investigations showed a complete absence of circulating luteinizing hormone (LH). Genetic studies revealed a missense mutation ... [more ▼]

A 30-year-old man was investigated for delayed puberty and infertility. These investigations showed a complete absence of circulating luteinizing hormone (LH). Genetic studies revealed a missense mutation in the LHbeta gene (G36D). This mutation disrupts a vital cystine knot motif and abrogates the heterodimerization and secretion of LH. Treatment with hCG was instituted, which led to arise in testosterone and improvement in spermatogenesis. After in vitro fertilization the patient had a son who was heterozygous for the G36D mutation. A second patient with similar clinical and biological presentation has been explored. A non-frame shift deletion of 3 base-pairs was discovered at position 20 which led to the deletion of a lysine residue. The proband and his prepubescent brother were homozygotic for this mutation. These cases illustrate the important physiological role of LH in male sexual maturation and fertility. [less ▲]

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See detailHypogonadismes hypogonadotropes par déficience en LH
Beckers, Albert ULg; Burlacu, M. C.

Scientific conference (2008, May)

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See detailHypomelanosis of Ito: pigmentary mosaicism with immature melanosome in keratinocytes.
Devillers, Céline; QUATRESOOZ, Pascale ULg; Hermanns-Lê, Trinh ULg et al

in International Journal of Dermatology (2011), 50(10), 1234-9

BACKGROUND: Hypomelanosis of Ito is a rare genetic disorder characterized by whorled areas of hypomelanosis. The purpose of the present study was to revisit some aspects of Ito's hypomelanosis. METHODS ... [more ▼]

BACKGROUND: Hypomelanosis of Ito is a rare genetic disorder characterized by whorled areas of hypomelanosis. The purpose of the present study was to revisit some aspects of Ito's hypomelanosis. METHODS: Clinical observations included ultraviolet-light-enhanced visualization (ULEV) method. Histochemistry, immunohistochemistry, and electron microscopy were performed on biopsy samples from the hypopigmented areas and the surrounding skin. RESULTS: Both the ULEV and microscopic examinations revealed the heterogeneity of the pigmentation. Hypomelanosis was characterized by a reduction in melanosomes, both in melanocytes and keratinocytes. These organelles were immature and atypical, showing a weak tyrosinase immunoreactivity. Melanosome macroautophagy was prominent in keratinocytes. Some clusters of the same cells exhibited strong immunoreactivity for the Mac 387 antibody (Ca(2+) -dependent calprotectin). Ulex europaeus agglutinin-1 (UEA-1) decorated the superficial layers of the epidermis. Such features are typically found in functionally altered keratinocytes. A number of dermal cells exhibited intense phagocytic activity linked to lysozyme immunoreactivity. CONCLUSIONS: Both the melanosome depletion and macroautophagy of immature melanosomes in keratinocytes appeared to represent prominent aspects of hypomelanosis of Ito. In sum, Ito's hypomelanosis combines structural and functional changes affecting both the melanocytes and keratinocytes in the skin. [less ▲]

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See detailHyponatrémie induite par la carbamazépine et affection rénale : à propos d'un cas
PITCHOT, William ULg; ANSSEAU, Marc ULg; PAPART, Patrick ULg et al

in Psychiatrie et psychobiologie (1989)

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See detailHypophosphatémie et ostéomalacie oncogénique
Van der Rest, Catherine; CAVALIER, Etienne ULg; COLSON, Laurent ULg et al

in Revue Médicale Suisse (2011), 7

In this article, we will discuss about hypophosphatemia due to tumor-induced osteomalacia. This disease is characterized by severe muscular and articular tenderness inducing profound walking limitation ... [more ▼]

In this article, we will discuss about hypophosphatemia due to tumor-induced osteomalacia. This disease is characterized by severe muscular and articular tenderness inducing profound walking limitation. Clinical chemistry results show severe hypophosphatemia due to hyperphosphaturia. Fibroblast growth factor 23 (FGG-23) is abnormally high. Physiological role of FGF-23 is examined. We also consider the pathophysiology of tumor induced osteomalacia, the use of different investigations to localize the tumor and therapies available to treat this rare disease. [less ▲]

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See detailHypophyseal failure and amaurosis revealing an extensive inflammatory pseudotumor
Hansen, I.; Ronci, N.; Stevenaert, Achille ULg et al

in The 6th International Pituitary Congress - Abstract book (1999)

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See detailHypophysite du Post-Partum
Beckers, Albert ULg

Scientific conference (2004, December 03)

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See detailHypopituitarism in pituitary gigantism (results of an international study)
Rostomyan, Liliya ULg; Daly, Adrian ULg; Shah, NS et al

in The 3rd ENEA Workshop : Hypopituitarism - Abstract book (2013, December)

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See detailHypopituitarism in pituitary gigantism (results of an international study)
Rostomyan, L; Daly, Adrian ULg; Shah, N et al

in Abstract book - Symposium "Perspectives in Endocrinology" Congresses Highlights 2013:ECE Copenhagen, ENDO SF, SFE Paris (2014, February)

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See detailHypopituitarisme consécutif aux atteintes cérébrales: le traumatisme cranien et l'hémorragie sous-arachnoidienne mis en cause.
Valdes Socin, Hernan Gonzalo ULg; Vroonen, Laurent ULg; Robe, Pierre ULg et al

in Revue Médicale de Liège (2009), 64(9), 457-463

Brain injuries namely traumatic brain injuries (TBI) and subarachnoid haemorrhage (SAH) are relevant causes of acquired adult hypopituitarism, perhaps more prevalent than ever believed. TBI represent a ... [more ▼]

Brain injuries namely traumatic brain injuries (TBI) and subarachnoid haemorrhage (SAH) are relevant causes of acquired adult hypopituitarism, perhaps more prevalent than ever believed. TBI represent a major health problem with an annual incidence of 300 cases per 100.000. SAH affects six new cases per 1.000.000 habitants in USA. In Belgium we estimate nearly 30.000 new TBI cases and 600 SAH cases per year. In the English literature, TBI secondary hypopituitarism has been well documented in 14 retrospective and prospective series accounting for 1.077 cases. In all these series the main pituitary deficits were: GH (14%), ACTH (14%), gonadotrope (18%), TSH (7%) and diabetes insipidus (4%). SAH was documented as a cause of hypopituitarism in three retrospective series accounting for 110 cases and in one prospective series. In all these series main pituitary deficits were GH (25%), ACTH (15%), gonadotrope (8.5%), TSH (6%) and diabetes insipidus (4%). In this review, we analyze recent data and discuss diagnostic and treatment features of secondary hypopituitarism due TBI and SAH. [less ▲]

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See detailHyporeactivity of Alveolar Macrophages and Higher Respiratory Cell Permissivity Characterize DBA/2J Mice Infected by Influenza A Virus Hyporeactivity of Alveolar Macrophages and Higher Respiratory Cell Permissivity Characterize DBA/2J Mice Infected by Influenza A Virus.
Casanova Bustos, Tomas Ronaldo ULg; Van de Paar, Els; Desmecht, Daniel ULg et al

in JOURNAL OF INTERFERON & CYTOKINE RESEARCH (2015), 35(10), 808-820

Influenza A virus remains a major public health problem. Mouse models have been widely used to study influenza infection in mammals. DBA/2J and C57BL/6J represent extremes in terms of susceptibility to ... [more ▼]

Influenza A virus remains a major public health problem. Mouse models have been widely used to study influenza infection in mammals. DBA/2J and C57BL/6J represent extremes in terms of susceptibility to influenza A infection among inbred laboratory mouse strains. Several studies focused specifically on the factors responsible for the susceptibility of DBA/2J or the resistance of C57BL/6J and resulted in impressive lists of candidate genes or factors over- or underexpressed in one of the strains. We adopted a different phenotypical approach to identify the critical steps of the infection process accounting for the differences between DBA/2J and C57BL/6J strains. We concluded that both a dysfunction of alveolar macrophages and an increased permissivity of respiratory cells rendered DBA/2J more susceptible to influenza infection. [less ▲]

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See detailHypotension du grand prematuré
Rigo, Vincent ULg; Beauduin, Philippe; Rigo, Jacques ULg

in Revue Médicale de Liège (2007), 62(2), 86-93

One out of four very preterm infants will present with circulatory maladaptation during the first two days of life, with an increased risk of early complications and long term sequelae. Appreciation of ... [more ▼]

One out of four very preterm infants will present with circulatory maladaptation during the first two days of life, with an increased risk of early complications and long term sequelae. Appreciation of those transitional difficulties cannot be limited to blood pressure. Assesment of blood pressure itself must be done in relation with gestational age and birth weight adapted norms. The effects of therapies for low systemic blood flow on blood pressure, organs and cerebral circulations are better understood, but none of them has assessed for mortality or neurodevelopmental outcomes. [less ▲]

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See detailL'Hypotension orthostatique: 1ere partie: definition, symptomatologie, evaluation et physiopathologie.
Tyberghein, Maelle; PHILIPS, Jean-Christophe ULg; Krzesinski, Jean-Marie ULg et al

in Revue Médicale de Liège (2013), 68(2), 65-73

Orthostatic hypotension (OH) is defined by a drop in arterial blood pressure (BP) of at least 20 mmHg for systolic BP and 10 mmHg for diastolic BP after standing. Symptoms are generally quite typical, but ... [more ▼]

Orthostatic hypotension (OH) is defined by a drop in arterial blood pressure (BP) of at least 20 mmHg for systolic BP and 10 mmHg for diastolic BP after standing. Symptoms are generally quite typical, but may also be rather vague. Diagnosis may be easily made by the physician in his/ her office, and confirmed, if necessary, by more sophisticated measurements. Pathophysiology is generally rather complex, but mostly involves a defect in the autonomic nervous system, in its sympathetic component. Failure of peripheral vasoconstriction seems to play a more important role than the defect in reflex tachycardia. Causes of OH are multiples. OH may occur in healthy subjects, when exposed to exceptional circumstances, but is more generally associated with various diseases, either neurological disorders or pathologies characterized by hypovolemia. Medications can also aggravate the risk of OH, among which some antihypertensive or psychotropic agents. Elderly people, especially frailty subjects, are exposed to a high risk of OH, whose origin is often multifactorial, and this complication may have serious medical consequences. [less ▲]

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See detailL'hypotension orthostatique: 2eme partie. Epidemiologie, complications et traitements.
Tyberghein, M.; Philips, J.-C.; Krzesinski, Jean-Marie ULg et al

in Revue Médicale de Liège (2013), 68(4), 163-70

Orthostatic hypotension (OH) is a rather common phenomenon in clinical practice. It may occur in 5-10 % of normal individuals, but its prevalence increases with age and various pathologies, so that it may ... [more ▼]

Orthostatic hypotension (OH) is a rather common phenomenon in clinical practice. It may occur in 5-10 % of normal individuals, but its prevalence increases with age and various pathologies, so that it may rise above 35 % in certain subgroups of patients. OH is associated with various comorbidities, in particular cardio-cerebro-vascular accidents and falls (especially in the elderly), and may even increase mortality. It is, however, difficult to determine whether OH is simply a marker of frailty or whether it is really a risk factor. OH treatment involves physical manoeuvres or medications, which aim at inducing a peripheral vasoconstriction (midodrine, etilefrine) or an increase of circulating blood volume (9-alpha-fluohydrocortisone). However, their use should be cautious, because of a risk of arterial hypertension in supine position. [less ▲]

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See detailHypothalamic AMPKalpha1 and neuropeptides are involved in neonatal performance of broiler chicks hatched at different times
Wang, Y.; Koppenol, A.; Lesuisse, J. et al

Conference (2014)

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See detailHypothalamic and Thyroidal Regulation of Growth Hormone in Tilapia
Melamed, P. M.; Eliahu, N. A.; Levavi-Sivan, Berta et al

in General and Comparative Endocrinology (1995), 97

A radioimmunoassay (RIA) for recombinant tilapia growth hormone (GH) was established and validated. The ability of various hypothalamic factors to regulate GH secretion in the tilapia hybrid (Oreochromis ... [more ▼]

A radioimmunoassay (RIA) for recombinant tilapia growth hormone (GH) was established and validated. The ability of various hypothalamic factors to regulate GH secretion in the tilapia hybrid (Oreochromis niloticus x Oreochromis aureus) was studied. Somatostatin1-14 (SRIF1-14; 10-100 micrograms/kg) was found to reduce circulating GH levels in a dose-dependent manner. SRIF1-14 (0.1-1000 nM) inhibited GH release from perifused pituitary fragments (ED50 0.83 nM). Human growth hormone-releasing hormone fragment 1-29 (hGHRH1-29; 100 micrograms/kg) doubled circulating GH levels and modestly stimulated GH secretion in vitro. Carp growth hormone-releasing hormone (cGHRH) stimulated GH secretion in vitro to a similar degree at the same dose (1 microM). Injection of salmon gonadotropin-releasing hormone (sGnRH) superactive analog (10-100 micrograms/kg) increased plasma GH levels sixfold. sGnRH also stimulated GH release in vitro (ED50 142.56 nM). Dopamine (0.1-10 microM) and the D1 DA receptor agonist SKF 38393 increased GH secretion from perifused pituitary fragments dose-relatedly. Thyrotropin-releasing hormone (TRH) had no effect on GH secretion from perifused pituitary fragments, but increased plasma GH levels, as did bovine thyroid stimulating hormone (bTSH). The increased plasma GH in the bTSH-treated fish coincided with a dramatic increase in T4; however, TRH increased GH without changing T4 levels. T3 increased the synthesis of GH by isolated pituitaries (incorporation of [3H]leucine). SRIF1-14 seems to be a most potent hypothalamic regulator of GH secretion in tilapia; sGnRH and DA both increased GH secretion, although sGnRH elicited considerably greater responses at lower doses. Two forms of GHRH increased GH levels, although the unavailability of the homologous peptide prevented an accurate evaluation of its importance in regulating GH secretion. The thyroid axis (TRH, TSH, and T3) stimulates both synthesis and release of GH, although TRH did not appear to have a direct effect on the level of the pituitary. [less ▲]

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See detailHypothalamic cushing's disease.
Beckers, Albert ULg; Stevenaert, Achille ULg; Hennen, Georges ULg

Conference (1987)

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See detailHypothalamic Expression Of Oestrogen Receptor Alpha And Androgen Receptor Is Sex, Age And Region Dependent In Mice.
Brock, Olivier; Mees, Christelle De; Bakker, Julie ULg

in Journal of neuroendocrinology (2015)

Sex steroid hormones act on developing neural circuits regulating the hypothalamic-pituitary-gonadal axis and are involved in hormone-sensitive behaviours. These hormones act mainly via nuclear receptors ... [more ▼]

Sex steroid hormones act on developing neural circuits regulating the hypothalamic-pituitary-gonadal axis and are involved in hormone-sensitive behaviours. These hormones act mainly via nuclear receptors, i.e. oestrogen receptor-alpha (ERalpha) and androgen receptor (AR). By using immunohistochemistry, we analysed the expression level of ERalpha and AR throughout perinatal life [at embryonic (E) day 19 and postnatal (P) days 5-15-25] and in adulthood in several hypothalamic nuclei controlling reproduction in both wild-type (WT) and aromatase knockout (ArKO) (which cannot convert testosterone into oestradiol) mice to determine whether there are sex differences in hypothalamic ERalpha and AR expression and if so, whether these are established by oestradiol action. As early as E19, ERalpha immunoreactivity (-ir) was observed at same expression levels in both sexes in the anteroventral periventricular nucleus (AVPv), the medial preoptic area (MPOA), the bed nucleus of the stria terminalis (BnST), the ventrolateral part of the ventromedial hypothalamic nucleus (VMHvl) and the arcuate nucleus (ARC). Sex differences (female > male) in ERalpha-ir were only observed during the prepubertal period in the BnST (P5 to P25) and the MPOA (P15), but also in adulthood in these two brain regions. Sex differences in AR-ir (male > female) were observed at P5 in the AVPv and ARC, and at P25 in the MPOA and ARC as well as in adulthood in all hypothalamic regions analysed. In adulthood, gonadectomy and hormonal treatment (oestradiol or dihydrotestosterone) also strongly modulated ERalpha-ir and AR, respectively. Taken together, sex differences in ERalpha-ir and AR-ir were observed in all hypothalamic regions analysed, but most likely do not reflect oestradiol actions since ArKO mice of both sexes showed very similar expression levels as WT mice throughout perinatal development. This article is protected by copyright. All rights reserved. [less ▲]

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