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Child's Nervous System : ChNS :... Mar 2021Pituitary hyperplasia following primary hypothyroidism in pediatric age group population is considered rare with reports of unnecessary neurosurgical intervention for... (Review)
Review
INTRODUCTION
Pituitary hyperplasia following primary hypothyroidism in pediatric age group population is considered rare with reports of unnecessary neurosurgical intervention for this medically treatable condition. Given the paucity of information on this topic, it is timely to provide clinicians with a comprehensive summary of available research.
METHODS
A search of published studies in Pubmed, PsychInfo and Cochrane Database with the terms "pituitary hyperplasia" or "pituitary hypertrophy" and "hypothyroidism" was performed and the results filtered for English language, pediatric (0-18 years) population and CT or MRI confirmed findings. 55 studies met the inclusion criteria. Data for a total of 110 patients with pituitary hyperplasia following primary hypothyroidism were extracted. The study population included 29 males and 81 females (M: F= 0.35:1). Patient age varied from 3 weeks to 18 years with a mean age of 10.22 years.
RESULTS
The most common clinical presentations included growth retardation, constipation and features of myxedema which were present in 78, 36 and 18 percent of children included in our review. Neuroimaging showed the mean (SD) pituitary height being 13.48 mm (4.72 mm). All of the patients achieved resolution of their pituitary mass and clinical as well as biochemical abnormalities 1 to 26 months after initiation of thyroid hormone replacement therapy, with an average time interval of 7.22 months. Our review has tried to delve in the pathophysiology as well as clinical, biochemical and radiological aspects of pediatric pituitary hyperplasia secondary to primary hypothyroidism and provide recommendations for treatment and follow-up. This may help anyone concerned gain a substantial knowledge on this topic.
Topics: Child; Female; Hormone Replacement Therapy; Humans; Hyperplasia; Hypothyroidism; Male; Pituitary Diseases; Pituitary Gland
PubMed: 33404718
DOI: 10.1007/s00381-020-05014-6 -
Expert Review of Endocrinology &... Jul 2019Treatment of childhood brain tumors, including surgical resection and especially external beam radiation, often results in endocrine complications manifested by... (Review)
Review
INTRODUCTION
Treatment of childhood brain tumors, including surgical resection and especially external beam radiation, often results in endocrine complications manifested by hypopituitarism, which can involve growth hormone deficiency, hypothyroidism, adrenal insufficiency, disorders of puberty, diabetes insipidus, and hypothalamic obesity.
AREAS COVERED
A comprehensive literature search was conducted on Medline (publications from the 1990s to 01/2019) including systematic reviews, meta-analyses, longitudinal controlled studies, retrospective cohort studies, and case reports. Herein, we present an up-to-date review of the current literature regarding endocrine sequellae of childhood brain tumor survivors.
EXPERT OPINION
Late endocrine sequellae can arise many years after the initial treatment of tumor, so at least annual surveillance of growth, puberty, weight, development, and endocrine status is recommended for at least 10 years after tumor therapy. This follow up should encompass childhood and adulthood among survivors. If found early, outcomes of endocrinopathies are favorable when treated appropriately. Newer tumor therapy modalities, such as proton beam radiation, offer the potential for fewer endocrine complications, but such benefit has yet to be demonstrated, and more research into short- and long-term outcomes is needed.
Topics: Adolescent; Adrenal Insufficiency; Adult; Brain Neoplasms; Cancer Survivors; Child; Child, Preschool; Female; Humans; Hypogonadism; Hypothalamic Diseases; Hypothyroidism; Male; Pituitary Diseases; Puberty, Precocious
PubMed: 31131647
DOI: 10.1080/17446651.2019.1620599 -
Endocrinology Dec 2018Ca2+ influx through voltage-gated Ca2+ channels (VGCCs) plays a key role in GH secretion. In this review, we summarize the current state of knowledge regarding the... (Review)
Review
Ca2+ influx through voltage-gated Ca2+ channels (VGCCs) plays a key role in GH secretion. In this review, we summarize the current state of knowledge regarding the physiology and molecular machinery of VGCCs in pituitary somatotrophs. We next discuss the possible involvement of Ca2+ channelopathies in pituitary disease and the potential use of Ca2+ channel blockers to treat pituitary disease. Various types of VGCCs exist in pituitary cells. However, because L-type Ca2+ channels (LTCCs) contribute the major component to Ca2+ influx in somatotrophs, lactotrophs, and corticotrophs, we focused on these channels. An increasing number of studies in recent years have linked genetic missense mutations in LTCCs to diseases of the human cardiovascular, nervous, and endocrine systems. These disease-associated genetic mutations occur at homologous functional positions (activation gates) in LTCCs. Thus, it is plausible that similar homologous missense mutations in pituitary LTCCs can cause abnormal hormone secretion and underlying pituitary disorders. The existence of LTCCs in pituitary cells opens questions about their sensitivity to dihydropyridines, a group of selective LTCC blockers. The dihydropyridine sensitivity of pituitary cells, as with any other excitable cell, depends primarily on two parameters: the pattern of their electrical activity and the dihydropyridine sensitivity of their LTCC isoforms. These two parameters are discussed in detail in relation to somatotrophs. These discussions are also relevant to lactotrophs and corticotrophs. High dihydropyridine sensitivity may facilitate their use as drugs to treat pituitary oversecretion disorders such as acromegaly, hyperprolactinemia, and Cushing disease.
Topics: Acromegaly; Animals; Calcium; Calcium Channels; Channelopathies; Humans; Hyperprolactinemia; Molecular Targeted Therapy; Pituitary ACTH Hypersecretion; Pituitary Diseases; Pituitary Gland, Anterior; Somatotrophs
PubMed: 30395240
DOI: 10.1210/en.2018-00743 -
Endocrine Nov 2015Endocrine abnormalities are known to affect patients with Fabry disease (FD). Pituitary gland theoretically represents an ideal target for FD because of high...
Endocrine abnormalities are known to affect patients with Fabry disease (FD). Pituitary gland theoretically represents an ideal target for FD because of high vascularization and low proliferation rate. We explored pituitary morphology and function in a cohort of FD patients through a prospectic, monocentric study at an Academic Tertiary Center. The study population included 28 FD patients and 42 sex and age-matched normal subjects. The protocol included a contrast enhancement pituitary MRI, the assessment of pituitary hormones, anti-pituitary, and anti-hypothalamus antibodies. At pituitary MRI, an empty sella was found in 11 (39%) FD patients, and in 2 (5%) controls (p < 0.001). Pituitary volume was significantly smaller in FD than in controls (p < 0.001). Determinants of pituitary volume were age and alpha-galactosidase enzyme activity. Both parameters resulted independently correlated at multivariate analysis. Pituitary function was substantially preserved in FD patients. Empty sella is a common finding in patients with FD. The major prevalence in the elderly supports the hypothesis of a progressive pituitary shrinkage overtime. Pituitary function seems not to be impaired in FD. An endocrine workup with pituitary hormone assessment should be periodically performed in FD patients, who are already at risk of cardiovascular complications.
Topics: Adult; Empty Sella Syndrome; Fabry Disease; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Pituitary Diseases
PubMed: 25896551
DOI: 10.1007/s12020-015-0604-z -
Annales D'endocrinologie Oct 2018Hypophysitis is a frequent toxic endocrine side-effect of immunotherapy. Prevalence is higher with anti-CTLA-4 antibodies (4-20%) or in association with PD-1 inhibitors... (Review)
Review
Hypophysitis is a frequent toxic endocrine side-effect of immunotherapy. Prevalence is higher with anti-CTLA-4 antibodies (4-20%) or in association with PD-1 inhibitors (8%). Diagnosis is presumptive, based on poorly specific clinical symptoms (usually, headache and asthenia) and/or hyponatremia and/or at least one pituitary deficit and/or abnormal imaging. Visual disorder or polyuropolydipsic syndrome are exceptional. In decreasing order of frequency, deficits are thyrotropic (86-100%), gonadotropic (85-100%) or corticotropic (50-73%); somatotropin deficit or abnormal prolactin level are rarer. Pituitary MRI in acute phase shows variable moderate increase in pituitary volume, ruling out differential diagnoses, especially pituitary metastasis. Treatment of corticotropin deficiency requires systematic emergency replacement therapy, with the usual modalities, while treatment of other deficits depends on clinical status and progression. Thyrotropin and gonadotropin deficits usually recover, but corticotropin deficiency persists over the long term, requiring education and specialized endocrinologic follow-up. Onset of hypophysitis does not contraindicate continuation of immunotherapy and does not usually require high dose synthetic glucocorticoids.
Topics: Consensus; Humans; Immunotherapy; Monitoring, Physiologic; Neoplasms; Pituitary Diseases
PubMed: 30126625
DOI: 10.1016/j.ando.2018.07.008 -
European Journal of Endocrinology Nov 2019Pituitary stalk interruption syndrome (PSIS) is a congenital pituitary anatomical defect. This syndrome is an antenatal developmental defect belonging to the... (Review)
Review
Pituitary stalk interruption syndrome (PSIS) is a congenital pituitary anatomical defect. This syndrome is an antenatal developmental defect belonging to the holoprosencephaly phenotype spectrum. It is heterogeneous regarding clinical, biological and radiological presentation and is characterized by the following triad: thin (<1 mm) or interrupted pituitary stalk connecting the hypothalamus to the pituitary gland, no eutopic posterior lobe, and hypoplasia or aplasia of the anterior lobe. This review reports current knowledge about the composite pathogenesis, for which underlying mechanisms remain unclear. Current data suggest genetic origins involving early developmental gene mutations with complex inheritance patterns and environmental influence, placing PSIS at the crossroads between Mendelian and multifactorial diseases. The phenotype associated with PSIS is highly heterogeneous with a high incidence of various combinations of hormonal deficiencies, sometimes associated with extra-pituitary birth defects. The age at onset is variable, but typical presentation is evolutive combined anterior pituitary hormone deficiencies at pediatric age, which progress even during adulthood to panhypopituitarism. Therefore, patients' follow-up throughout life is essential for adequate management.
Topics: Humans; Hypopituitarism; Phenotype; Pituitary Diseases; Pituitary Gland; Syndrome
PubMed: 31480013
DOI: 10.1530/EJE-19-0168 -
Minerva Endocrinologica Dec 2018Pregnancy-related hypophysitis (PR-Hy) that typically occurs during late pregnancy and the early postpartum period is considered an autoimmune disorder. Knowledge and... (Review)
Review
Pregnancy-related hypophysitis (PR-Hy) that typically occurs during late pregnancy and the early postpartum period is considered an autoimmune disorder. Knowledge and awareness of characteristic clinical symptoms and specific endocrine and neuroradiological features mostly enables a correct diagnosis and guarantees best possible management. In the presence of mild clinical symptoms, a "wait-and-watch" approach is recommended. Transsphenoidal surgery or glucocorticoid therapy is indicated if progressive clinical signs or severe visual symptoms are observed. Reported cases indicate that transsphenoidal surgery performed during pregnancy can reverse visual symptoms and has a minimal risk to the health of the fetus and mother. Glucocorticoid therapy has a high initial response rate, but recurrence can occur after discontinuation. In late-pregnancy hypophysitis, the options of delaying therapy until the child is born or, if symptoms are severe, of inducing delivery prior to therapy have to be considered. Sheehan's syndrome (SS) is characterized by irreversible, partial or complete hypopituitarism due to pituitary infarction. The predominating causative event is massive peripartum hemorrhage (PPH). A considerable diagnostic delay is mostly observed. PPH and the typical postpartum findings of agalactia and amenorrhea should alert for the diagnosis of SS and prompt endocrine evaluation. Timely hormone replacement therapy is paramount to reduce morbidity and mortality and improve quality of life of afflicted women. Adrenal failure is a major endocrinological risk both of hypophysitis and of Sheehan syndrome. Early recognition and treatment is paramount to avoid life-threatening adrenal crisis.
Topics: Acute Disease; Adult; Female; Humans; Hypopituitarism; Pituitary Diseases; Pregnancy; Pregnancy Complications
PubMed: 29463076
DOI: 10.23736/S0391-1977.18.02814-6 -
Frontiers in Endocrinology 2020The pituitary gland has the primordial ability to dynamically adapt its cell composition to changing hormonal needs of the organism throughout life. During the first... (Review)
Review
The pituitary gland has the primordial ability to dynamically adapt its cell composition to changing hormonal needs of the organism throughout life. During the first weeks after birth, an impressive growth and maturation phase is occurring in the gland during which the distinct hormonal cell populations expand. During pubertal growth and development, growth hormone (GH) levels need to peak which requires an adaptive enterprise in the GH-producing somatotrope population. At aging, pituitary function wanes which is associated with organismal decay including the somatopause in which GH levels drop. In addition to these key time points of life, the pituitary's endocrine cell landscape plastically adapts during specific (patho-)physiological conditions such as lactation (need for PRL) and stress (engagement of ACTH). Particular resilience is witnessed after physical injury in the (murine) gland, culminating in regeneration of destroyed cell populations. In many other tissues, adaptive and regenerative processes involve the local stem cells. Over the last 15 years, evidence has accumulated that the pituitary gland houses a resident stem cell compartment. Recent studies propose their involvement in at least some of the cell remodeling processes that occur in the postnatal pituitary but support is still fragmentary and not unequivocal. Many questions remain unsolved such as whether the stem cells are key players in the vivid neonatal growth phase and whether the decline in pituitary function at old age is associated with decreased stem cell fitness. Furthermore, the underlying molecular mechanisms of pituitary plasticity, in particular the stem cell-linked ones, are still largely unknown. Pituitary research heavily relies on transgenic mouse models. While having proven their value, answers to pituitary stem cell-focused questions may more diligently come from a novel powerful research model, termed organoids, which grow from pituitary stem cells and recapitulate stem cell phenotype and activation status. In this review, we describe pituitary plasticity conditions and summarize what is known on the involvement and phenotype of pituitary stem cells during these pituitary remodeling events.
Topics: Animals; Humans; Pituitary Diseases; Pituitary Gland; Stem Cells
PubMed: 33584539
DOI: 10.3389/fendo.2020.604519 -
Clinical Endocrinology Oct 2016The spectrum of pituitary stalk (PS) pathology is vast, presenting a diagnostic challenge. Published large series of PS lesions demonstrate neoplastic conditions are... (Review)
Review
The spectrum of pituitary stalk (PS) pathology is vast, presenting a diagnostic challenge. Published large series of PS lesions demonstrate neoplastic conditions are most frequent, followed by inflammatory, infectious and congenital diseases. Inflammatory pathologies however, account for the majority of PS lesions in published small case series and case reports. Physicians must be familiar with the major differential diagnoses and necessary investigations. A comprehensive history and thorough clinical examination is critical. Although magnetic resonance imaging of the PS in disease is nonspecific, associated intracranial features may narrow the differential diagnosis. Initial investigations include basic pathology and computer tomography imaging of the neck, chest, abdomen and pelvis. Further investigations should be guided by the clinical context. PS biopsy should be considered when a diagnosis is regarded essential in centres where an experienced neurosurgeon is available. Treatment is dependent on the underlying disease process and may necessitate pituitary hormone replacement.
Topics: Diagnosis, Differential; Diagnostic Imaging; Humans; Pituitary Diseases; Pituitary Gland; Pituitary Neoplasms
PubMed: 26950774
DOI: 10.1111/cen.13058 -
Frontiers in Endocrinology 2022Pituitary stalk thickening (PST) is a rare abnormality in the pediatric population. Its etiology is heterogeneous. The aim of the study was to identify important... (Review)
Review
BACKGROUND
Pituitary stalk thickening (PST) is a rare abnormality in the pediatric population. Its etiology is heterogeneous. The aim of the study was to identify important clinical, radiological and endocrinological manifestations of patients with PST and follow the course of the disease.
MATERIALS AND METHODS
It is a study conducted in 23 patients (13 boys) with PST with/without central diabetes insipidus (CDI) diagnosed between 1990 and 2020 at Children's Memorial Health Institute (CMHI) in Warsaw, Poland. We analyzed demographic data, clinical signs and symptoms, radiological findings, tumor markers, hormonal results, treatment protocols and outcomes.
RESULTS
The median age at the diagnosis of PST was 9.68 years (IQR: 7.21-12.33). The median time from the onset of the symptoms to the diagnosis was 2.17 years (IQR: 1.12-3.54). The most common initially reported manifestations were polydipsia, polyuria and nocturia (82.6%); most of the patients (56.5%) also presented decreased growth velocity. Hormonal evaluation at the onset of PST revealed: CDI (91.3%), growth hormone deficiency (GHD) (56.5%), hyperprolactinemia (39%), central hypothyroidism (34.8%), adrenal insufficiency (9%), precocious puberty (8.7%). The majority of the patients were diagnosed with germinoma (seventeen patients - 73.9%, one of them with teratoma and germinoma). Langerhans cell histiocytosis (LCH) was identified in three patients (multisystem LCH in two patients, and unifocal LCH in one patient). A single case of atypical teratoid rhabdoid tumor, suspected low-grade glioma (LGG) and lymphocytic infundibuloneurohypophysitis (LINH). The overall survival rate during the observational period was 87.0%.
CONCLUSIONS
The pituitary infundibulum presents a diagnostic imaging challenge because of its small size and protean spectrum of disease processes. Germinoma should be suspected in all children with PST, especially with CDI, even when neurological and ophthalmological symptoms are absent.
Topics: Brain Neoplasms; Child; Diabetes Insipidus, Neurogenic; Germinoma; Histiocytosis, Langerhans-Cell; Humans; Magnetic Resonance Imaging; Male; Pituitary Diseases; Pituitary Gland
PubMed: 35669693
DOI: 10.3389/fendo.2022.868558