Acromegaly
Nadia Barghouthi, Jessica Perini in Endocrine Diseases in Pregnancy and the Postpartum Period, 2021
Acromegaly is a rare disease that is caused by excessive growth hormone production. Diagnosis of acromegaly during pregnancy is often confounded by pregnancy-associated hormonal changes. Acromegaly is caused by excess growth hormone secretion from pituitary somatotroph cells and occurs most commonly in the context of a benign pituitary adenoma. Medical therapy or surgery can be considered in pregnant patients with acromegaly if they have significant symptoms, including persistent headache or new visual impairment suggesting tumor growth. Dopamine agonists are incompatible with breastfeeding, and there is minimal data to support the use of octreotide or pegvisomant during lactation. The classic physical features of acromegaly include enlarged hands and feet with broad fingers and toes. Facial deformities are often present, including a rectangular face, widened nose, prominent forehead, and enlarged lower jaw with widely spaced teeth. The potential safety of medical therapy is based on animal studies and case reports.
Control of endocrine hormone release
Ben Greenstein in Rapid Revision in Endocrinology, 2017
This chapter aims to understand the basic principles of feedback mechanisms, and presents important examples of the relevance of feedback mechanisms in basic and clinical endocrinology and how these may be used as diagnostic tests. It provides general principles of treatment using knowledge of endocrine feedback systems. Feedback systems maintain a set point for example central heating temperature regulation. Biological feedback mechanisms also control: fertility, metabolism, poorly understood cycles of sleep and wakefulness and circadian and other rhythms. Endocrine feedback systems include control of: brain and pituitary hormones, adrenal hormones, gastrointestinal tract hormones, growth hormones, sex hormones, thyroid hormones, feeding and satiety hormones, and salt and water balance. Many endocrine diseases result from disturbances of feedback control systems, and tests of feedback systems are of diagnostic value in, for example: acromegaly, Addison's disease, Cushing's disease, diabetes mellitus, growth disorders, hypo- or hyperthyroidism, infertility, and obesity.
Endocrine and reproductive disorders
Angus Clarke, Alex Murray, Julian Sampson in Harper's Practical Genetic Counselling, 2019
The genetics of diabetes mellitus provides a paradigm for the role of genetic factors in common disorders of complex determination. Type II diabetes is an exceptionally common disorder that shows extreme geographical variation but is continuing to increase in prevalence with the trend to higher body mass index levels in many populations. Acromegaly and related disorders of pituitary hypersecretion are mostly sporadic but may form part of the autosomal dominant type 1 multiple endocrine neoplasia. A heterogeneous group of proportionate growth failure, most often from intrauterine life, remains after endocrine causes have been excluded. Most chromosomal disorders impair growth, but specific causes to be considered include Russell-Silver syndrome and Seckel syndrome. Gorlin syndrome, Simpson-Golabi-Behmel syndrome, the PTEN-related disorder Cowden syndrome and the fragile X syndrome Fragile X syndrome type A may be associated with macrocephaly and overgrowth. Genetic counselling in this area must rest on a well-defined diagnostic assessment that integrates clinical assessment with endocrinological and genetic investigations as appropriate.
Sleep apnea in acromegaly: a review on prevalence, pathogenetic aspects and treatment
Published in Expert Review of Endocrinology & Metabolism, 2012
Maria Vittoria Davì, Andrea Giustina
Sleep apnea syndrome is a common complication of acromegaly with a negative impact on quality of life and survival. Obstructive sleep apnea is the prevailing form and is characterized by recurrent episodes of apnea and hypopnea owing to the total or partial collapse of the upper airways during sleep. The craniofacial deformations and the hypertrophy of upper airway soft tissue are responsible for its occurrence. Successful treatment of acromegaly can improve the severity of this complication, but can only seldom reverse it, particularly after a long time of active acromegaly. Thus, it is advisable to evaluate patients for sleep apnea syndrome at diagnosis and during treatment, and also when acromegaly is biochemically controlled. In selected cases, continuous positive airway pressure should be implemented to improve patient outcome.
Octreotide for acromegaly treatment: a reappraisal
Published in Expert Opinion on Pharmacotherapy, 2013
Andrea Giustina, Ioannis Karamouzis, Ilaria Patelli, Gherardo Mazziotti
Introduction: Acromegaly is a rare disorder characterized by excess secretion of growth hormone (GH) generally caused by a pituitary macroadenoma and associated with reduced life expectancy if the disease is untreated. This article covers the recent available evidences published on octreotide, the first somatostatin analog introduced into clinical practice for the medical treatment of acromegaly. Areas covered: This article discusses i) pharmacology of somatostatin and octreotide; ii) biochemical effects of regular octreotide and long-acting repeatable formulation; iii) tumor shrinkage effects of octreotide in acromegaly; iv) impact of octreotide on acromegalic clinical manifestations and chronic complications; v) safety of octreotide and vi) place of octreotide in the guidelines for acromegaly treatment. Full-text articles in the English language were selected from a PubMed search spanning 1984 – 2013, for keywords including ‘octreotide,' ‘acromegaly,' ‘GH,' ‘IGF-I,' and ‘tumor shrinkage.' Reference lists in selected papers were also used to broaden the search. Expert opinion: Octreotide is a mature drug with a consolidated favorable benefit versus risks profile in the treatment of acromegaly.
Tear Osmolarity and Tear Function Changes in Patients with Acromegaly
Published in Current Eye Research, 2015
Ceyhun Arici, Esra Hatipoglu, Guzin Iskeleli, Pinar Sultan, Cansu Yuksel, Sadi Gundogdu, Pinar Kadioglu
Purpose: Since acromegaly is a disease with various systemic complications, it may also have ophthalmologic consequences. The aim of the current study was to compare the tear osmolarity and tear function changes in patients with acromegaly with those in healthy controls. Design: Prospective, cross-sectional study. Materials and methods: Fifty-nine consecutive patients with acromegaly and 62 age and gender matched healthy volunteers were enrolled in the study. Tear osmolarity measurement with TearLab Osmolarity System (Tearlab, San Diego, CA), tear film break-up time (TBUT) assessment, and the Schirmer test without anesthesia were performed in the same order in each group. Growth hormone (GH) and insulin like growth factor-1 (IGF1) levels were also determined in the study group. Results: The mean TBUT was lower in acromegalic patients (9.1 ± 3.6 seconds) than in healthy controls (10.7 ± 2.9 s) (p = 0.009). The difference between the two groups in tear osmolarity and Schirmer test results (p = 0.08 and p = 0.9, respectively) was not statistically different. Conclusions: Acromegaly may a cause a decrease in TBUT in the affected patient. Preservation of normal tear osmolarity and normal Schirmer test results suggests that this might be due to effects on the meibomian glands.