Burosumab treatment for fibroblast growth factor-23-associated hypophosphatemia in an adult patient with severe fibrous dysplasia in McCune-Albright syndrome: case report and review of the literature.

McCune-Albright syndrome (MAS) is a rare mosaic genetic disorder caused by a mutation in the gene and typically presents with a triad of symptoms: fibrous dysplasia of bones, café-au-lait macules, and precocious puberty. The mutation leads to overproduction of fibroblast growth factor-23 (FGF23), which may result in hypophosphatemia. Burosumab, a monoclonal antibody against FGF23, is approved for the treatment X-linked hypophosphatemia and tumor-induced osteomalacia.

Collaboration Between Endocrinologists and Dentists in the Care of Patients with Acromegaly-A Narrative Review.

Acromegaly is caused by an excessive secretion of growth hormone and the secondary elevation of IGF-1 levels, leading to progressive changes in multiple body systems, including the craniofacial region and oral cavity. Dental manifestations such as mandibular overgrowth, macroglossia, malocclusion, periodontal disease, and prosthetic difficulties represent not only a clinical component of the disease but also a significant therapeutic and diagnostic challenge. The aim of this review is to present the current state of knowledge on the relationship between acromegaly and oral health and to analyze the role of interdisciplinary collaboration between endocrinologists and dentists in patient care.

Endocrine alterations in patients with pachydermoperiostosis.

Context: Pachydermoperiostosis (primary hypertrophic osteoarthropathy, PHO) usually due to biallelic loss-of-function variants in HPGD and SLCO2A1, has some features overlapping with acromegaly and often referred to endocrinologists. A detailed endocrine assessment is not available for these patients.

Objective: To assess the genetic and endocrine characteristics of PHO patients referred to endocrine centres with a possible diagnosis of acromegaly.

Uterine fibroids in women diagnosed with acromegaly: a systematic review.

The review discusses the relationship between acromegaly and uterine fibroids. It highlights variations in research methodologies and inconsistent findings, emphasizing the complex nature of fibroid development and the role of the somatotropic axis. Additionally, it addresses demographic factors and examines the potential impact of therapies on the risk and prevalence of uterine fibroids in individuals with acromegaly.

Pathologic Characteristics of Somatotroph Pituitary Tumors-An Observational Single-Center Study.

The pathologic evaluation of a tumor tissue is an essential part of an acromegaly patient's assessment. This study aimed to analyze the pathologic characteristics of pituitary tumors in patients with acromegaly. The demographic data, in addition to the hormonal, imaging, and pathologic results of 120 patients with acromegaly after pituitary surgery, were extracted from the Polish Acromegaly Registry.

Skeletal complications in acromegaly.

Hypersecretion of growth hormone (GH) is rare and typically results from a pituitary functional tumor - somatotropinoma. It leads to excessive linear bone growth and manifests as gigantism if occurring in childhood and adolescence, before the closure of epiphyses or as a acromegaly in adulthood. The excess of GH impacts bone metabolism directly as well as indirectly through increased insulin-like growth factor 1 (IGF-1).

Effects of acromegaly treatment on left ventricular systolic function assessed by speckle tracking echocardiography in relation to sex differences: results from a prospective single center study.

Background: Despite the preserved LVEF, patients with acromegaly are characterized by subclinical systolic dysfunction i.e., abnormal global longitudinal strain (GLS) assessed by speckle tracking echocardiography (STE).

Macro-GH - A clinical entity causing a diagnostic challenge - A case report.

Aim: Presentation of a new case of a patient with macro-GH, that may interfere with different GH assays leading to false-positive results in serum samples.

Case Presentation: A 61-year-old female was referred with a pituitary macroadenoma and elevated growth hormone levels. The laboratory tests showed increased fasting GH level, measured by a sandwich chemiluminescence immunoassay (LIAISON® XL) without suppression on oral glucose tolerance test and normal IGF-1.

Clinical, hormonal and pathomorphological markers of somatotroph pituitary neuroendocrine tumors predicting the treatment outcome in acromegaly.

Background: Transsphenoidal adenomectomy (TSS) of somatotroph pituitary neuroendocrine tumor (PitNET) is the first-line treatment of acromegaly. Pharmacological treatment is recommended if surgery is contraindicated or did not lead to disease remission. The choice of treatment best fitting each patient should be based on thorough investigation of patients' characteristics.

Pituitary MRI Features in Acromegaly Resulting From Ectopic GHRH Secretion From a Neuroendocrine Tumor: Analysis of 30 Cases.

Context: Ectopic acromegaly is a consequence of rare neuroendocrine tumors (NETs) that secrete GHRH. This abnormal GHRH secretion drives GH and IGF-1 excess, with a clinical presentation similar to classical pituitary acromegaly. Identifying the underlying cause for the GH hypersecretion in the setting of ectopic GHRH excess is, however, essential for proper management both of acromegaly and the NET.

Real-world experience with pasireotide-LAR in resistant acromegaly: a single center 1-year observation.

Context: Pasireotide-LAR, a second-generation somatostatin receptor ligand (SRL), is recommended for patients with acromegaly as second-line treatment. Its efficacy and safety were assessed in clinical trials; however, the real-world evidence is still scarce.

Objective: The aim of this study was to evaluate the impact of 1-year treatment with pasireotide-LAR on disease control and glucose metabolism in acromegaly patients resistant to first-generation SRLs.

Management of endocrine immune-related adverse events of immune checkpoint inhibitors: an updated review.

Immune checkpoint inhibitors (ICIs) belong to a new group of anticancer drugs targeting T-cell proteins involved in the activation of immune response toward malignancies. Their introduction into clinical practice was a milestone in modern cancer treatment. However, the significant advantage of ICIs over conventional chemotherapy in terms of therapeutic efficacy is accompanied by new challenges related to specific side effects.

Immunotherapy of solid tumors: safety of treatment.

Immunotherapy with immune checkpoint inhibitors (ICIs) was shown to improve survival of patients with solid tumors such as: melanoma, renal carcinoma, non-small cell lung cancer, cutaneous carcinomas, or head and neck carcinoma. However, a special type of ICIs toxicity is observed, namely noninfectious inflammation of different organs associated with autoimmunity known as immune‑related adverse events (irAEs). This noninfectious inflammation may affect the endocrine system, gastrointestinal tract, heart, skin, and nervous system.

Corticotropinoma as the underlying cause of intermittent Cushing's syndrome in a patient previously diagnosed with primary pigmented nodular adrenocortical disease.

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Subclinical left ventricular systolic dysfunction in patients with naive acromegaly - assessment with two-dimensional speckle-tracking echocardiography: retrospective study.

Introduction: The aim of the study was to evaluate global longitudinal strain (GLS) in patients with naive acromegaly with normal left ventricular (LV) ejection fraction (EF).

Material And Methods: Forty-three consecutive patients with naive acromegaly with normal LV systolic function as measured by EF, examined from 2008 to 2016, and 52 patients of a control group matched for age and sex underwent two-dimensional speckle-tracking echocardiography to assess GLS.

Results: The median GLS was significantly lower in the acromegaly group than in the control group (in %, -16.

Heart in Acromegaly: The Echocardiographic Characteristics of Patients Diagnosed with Acromegaly in Various Stages of the Disease.

To determine whether the echocardiographic presentation allows for diagnosis of acromegalic cardiomyopathy. 140 patients with acromegaly underwent echocardiography as part of routine diagnostics. The results were compared with the control group comprising of 52 age- and sex-matched healthy volunteers.

Fatal Carney Complex in Siblings Due to De Novo Large Gene Deletion.

Context: Carney complex (CNC) is a rare multiple neoplasia syndrome involving cardiac, endocrine, neural, and cutaneous tumors and a variety of pigmented skin lesions. CNC can be inherited as an autosomal dominant trait, but in about one-third of patients, the disease is caused by de novo mutation in the PRKAR1A gene localized on chromosome 17q22-24. Most of the mutations include single base substitutions and small deletions/insertions not exceeding 15 base pairs.

The impact of transsphenoidal surgery on glucose homeostasis and insulin resistance in acromegaly.

Background And Purpose: Impaired glucose tolerance and overt diabetes mellitus are frequently associated with acro-megaly. The aim of this study was to find out whether these alterations could be reversed after transsphenoidal surgery.

Material And Methods: Two hundred and thirty-nine acromegalic patients were studied before and 6-12 months after transsphenoidal surgery.

Abnormalities in glucose homeostasis in acromegaly. Does the prevalence of glucose intolerance depend on the level of activity of the disease and the duration of the symptoms?

Introduction: Acromegaly is characterized not only by disabling symptoms, but also by relevant co-morbidities. Insulin resistance, leading to glucose intolerance is one of the most important contributory factors to the cardiovascular mortality in acromegaly.

Material And Methods: We analysed the records of 220 naïve patients with acromegaly diagnosed at our Department in the years 1995-2007.