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Soluble α-klotho (sαKl) is an isoform of the α-klotho protein that regulates calcium metabolism but also has anti- aging, anti-apoptotic, and tumor suppressor effects. The assumption that sαKl could inhibit the insulin-like growth factor 1 (IGF-1) signaling pathway has increased the interest of its research in acromegaly - a pathology characterized by an excess of growth hormone (GH) and IGF-1. This review aimed to identify the role of sαKl in acromegaly. Most studies have identified higher concentrations of sαKl in acromegaly versus non-GH pituitary adenomas (PAs) or healthy subjects. Also, the sαKl level was higher in active acromegaly versus controlled or cured disease. Moreover, sαKl positively correlated with GH, IGF-1, and GH-secreting PA size. The increased level of sαKl seems to be due to the autonomous secretion of GH, considering their drastic decrease after transsphenoidal surgery. The data regarding the relationship between sαKl, age, and gender in acromegaly are inconsistent. The latest research noted a correlation between sαKl and various parameters for monitoring the quality of life in acromegaly. In conclusion, sαKl could reflect the disease activity. Furthermore, sαKl would represent an alternative for monitoring acromegalic patients with discrepancies between IGF-1 and GH. [ABSTRACT FROM AUTHOR]

• Les patients atteints d'acromégalie ont une faible prévalence de fractures vertébrales. • Étude des anomalies qualitatives de la colonne vertébrale chez les patients acromégaux. • Le patient acromégalie avait une forte prévalence de déformations de la colonne vertébrale. La plupart des études retrouvent une majoration du risque de fractures vertébrales chez les patients acromégales, malgré une densité minérale osseuse normale. Nous avons étudié la prévalence des fractures vertébrales chez les patients acromégales de moins de 80 ans suivis au CHU de Nantes. Étude transversale monocentrique réalisée au CHU de Nantes de 1988 à 2018. Cinquante patients (18 femmes, 32 hommes) d'un âge médian de 52,3 ans (27 -78 ans) ont été inclus. Les fractures vertébrales étaient évaluées sur des radiographies conventionnelles des rachis lombaire et thoracique de face et de profil, selon la méthode semi-quantitative de Genant. Nous avons également étudié les déformations vertébrales autres que les fractures, en utilisant 3 critères : présence d'ostéophytes, présence de pincement discal et aspect cunéiforme des vertèbres. Nous avons analysé la densité minérale osseuse par absorptiométrie bi-photonique et le statut hormonal. Trois patients (6 %) présentaient une fracture vertébrale : une fracture de grade 1 et 2 fractures de grade 2 selon la classification de Genant, chez deux patients ostéoporotiques et un patient ostéopénique. Leurs déficits anté-hypophysaires étaient substitués. En considérant les déformations franches (ostéophyte ou pincement discal ≥ grade 2 ou aspect cunéiforme), le rachis dorsal était déformé chez 22 patients (44 %) et le rachis lombaire chez 21 patients (42 %). Les patients acromégales présentaient une faible prévalence de fractures vertébrales, mais un nombre important de déformations vertébrales. Nous supposons que cette forte prévalence de déformations franches pourrait expliquer la forte prévalence de fractures vertébrales rapportée précédemment. [ABSTRACT FROM AUTHOR]

Résumé: L'acromégalie est une maladie rare, habituellement en rapport avec une hypersécrétion d'hormone de croissance (GH) et du facteur analogue à l'insuline (IGF-1), le plus souvent par un adénome hypophysaire. Les manifestations digestives de l'acromégalie sont principalement représentées par les polypes coliques, qui peuvent dégénérer par la suite. Le but de notre travail est de décrire l'apport de la coloscopie de dépistage chez les patients suivis pour acromégalie. Il s'agit d'une série rétrospective incluant 35 patients suivis pour acromégalie et ayant bénéficié d'une coloscopie de dépistage sur une période de neuf ans (de janvier 2009 à novembre 2019). Nous avons analysé le sexe des patients, leur âge au moment de la réalisation de la coloscopie, les comorbidités associées et le résultat de la coloscopie (localisation de polypes et leur type histologique). [ABSTRACT FROM AUTHOR]

Pituitary adenomas are frequent benign tumors that cause a high morbidity due to their complications. Most cases are sporadic but 5% arise in a familial setting, associated with other tumors (as is for MEN1 and MEN4, Carney Complex, McCune-Albright Syndrome) or without other associated disease as is for FIPA (Familial Isolated Pituitary Adenomas) families. AIP (Aryl-hydrocarbon Receptor Interacting Protein), a tumor suppressor gene, is mutated in approximately 30% of FIPA patients and in a much lesser percent in sporadic pituitary adenomas. Patients harboring an AIP mutation have certain characteristics: are younger and with larger pituitary adenomas, usually secreting growth hormone or prolactin and more resistant to current therapies as compared to AIP negative patients. Recently a new genotype was identified in FIPA patients with early onset gigantism - microduplications on chromosome Xq26.3 and the syndrome was called X-LAG (X-linked acrogigantism). In more than a half of FIPA patients the genetic background of the disease is still unknown and more work is needed in this direction. [ABSTRACT FROM AUTHOR]

Pituitary adenomas are frequent benign tumors that cause a high morbidity due to their complications. Most cases are sporadic but 5% arise in a familial setting, associated with other tumors (as is for MEN1 and MEN4, Carney Complex, McCune-Albright Syndrome) or without other associated disease as is for FIPA (Familial Isolated Pituitary Adenomas) families. AIP (Aryl-hydrocarbon Receptor Interacting Protein), a tumor suppressor gene, is mutated in approximately 30% of FIPA patients and in a much lesser percent in sporadic pituitary adenomas. Patients harboring an AIP mutation have certain characteristics: are younger and with larger pituitary adenomas, usually secreting growth hormone or prolactin and more resistant to current therapies as compared to AIP negative patients. Recently a new genotype was identified in FIPA patients with early onset gigantism - microduplications on chromosome Xq26.3 and the syndrome was called X-LAG (X-linked acrogigantism). In more than a half of FIPA patients the genetic background of the disease is still unknown and more work is needed in this direction. [ABSTRACT FROM AUTHOR]

Arterial hypertensions (HTA) of endocrine origin are a rare cause of hypertension; HTA overall prevalence don't exceed 4% of hypertensive patients. Research interest in endocrine HTA is due to the severity of some life-threatening, potentially curable and reversible forms of HTA. The aim of our study was to determine the clinical, paraclinical, etiological and therapeutic profile of secondary HTA of endocrine origin in patients treated in endocrinology department at the University Hospital Mohamed VI in Marrakech. We conducted a prospective, descriptive study spanned 4 years, enrolling 45 patients with endocrine HTA. The average age was 44.89 years, with a clear predominance of women (sex ratio 0.49). Etiology of endocrine HTA was dominated by pheochromocytoma (17 cases), hypercorticism (11 cases) and acromegaly (8 cases). HTA were paroxysmal in 24.4%. HTA were immediately classified as grade 3 severe in 40% of cases. HTA were complicated by heart disease in 24% of cases and by renal disease in 20% of cases. Curative treatment cleared up HTA in 60% of cases (27 cases). The diagnosis of secondary endocrine HTA is sometimes difficult because of the lack of clinical specificity. It is not unusual for HTA to be the only manifestation of the disease. In our study we noted the paroxysmal and severe nature of HTA. The potentially curable nature of HTA in more than two thirds of cases, demostrates the importance of early diagnosis of each severe HTA resistant to treatment or in the presence of suggestive clinical, biological or radiological signs. [ABSTRACT FROM AUTHOR]

Diabetes mellitus may occur as a complication of endocrine disorders and it's name is, according to current classification (American Diabetes Association): „other specific types of diabetes“. This particular endocrine diseases associate sustained and excessive hormonal secretion, which interferes insulin secretion or action. Over time, glucose intolerance and then diabetes occur. Generally, there are middle forms with no ketosis because of the persistence of endogenous insulin secretion. More then that, hyperglycemia is usually reversible with the treatment of the underlying endocrinopathy. Main endocrine diseases that cause diabetes are reviewed. [ABSTRACT FROM AUTHOR]

The need to evaluate patients for secondary hypertension is common in clinical practice. Suspicion of endocrine hypertension occurs when the disease starts suddenly at young age, in cases with loss of blood pressure control in a patient with previously well-controlled blood pressure or labile blood pressure cases. Careful medical history and physical examination are very important to rule out other factors responsible for these patterns of hypertension (certain medications, alcohol, lack of compliance to treatment or dietary salt restriction, panic attacks etc). Pheochromocytoma and Cushing syndrome - the major endocrine diseases associated with secondary hypertension - are rare in clinical practice. Instead, primary hyperaldosteronism is becoming more frequently identified. It can go unnoticed because of nonspecific clinical presentation and of the fact that hypokalaemia, classically described, is actually rare in practice. [ABSTRACT FROM AUTHOR]

L'acromégalie est une maladie endocrinienne rare, en rapport avec une hypersécrétion d'hormone de croissance. Elle a des conséquences rhumatologiques: l'arthropathie périphérique, l'atteinte rachidienne et les syndromes canalaires. L'atteinte articulaire accompagne une acromégalie active, sa survenue après un traitement radical et une rémission complète est rare. Nous présentons le cas d'une patiente de 70 ans ayant un antécédent d'acromégalie sur adénome hypophysaire il y a 25 ans, traitée chirurgicalement et déclarée en rémission complète, a développé une arthropathie destructrice des deux épaules. Le but de notre observation est de mettre le point sur la possibilité d'une atteinte articulaire au cours de l'acromégalie et de son retentissement fonctionnelle. [ABSTRACT FROM AUTHOR]