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Journal of Mid-life Health 2022Lymphocytic hypophysitis (LYH) is a rare inflammatory disease in which lymphoplasmacytic infiltration of the pituitary gland can potentially lead to panhypopituitarism....
Lymphocytic hypophysitis (LYH) is a rare inflammatory disease in which lymphoplasmacytic infiltration of the pituitary gland can potentially lead to panhypopituitarism. This entity was first recognized in 1962 by Goudie and Pinkerton. We report the case of a 36-year-old nonpregnant female operated for a presumed nonsecreting pituitary adenoma. On intraoperative squash cytology and histology of excision biopsy confirmed the diagnosis of LYH. Review of the literature shows that most reported cases have occurred in women during pregnancy or during postpartum period. Many were accurately diagnosed only after biopsy or at necropsy. It is suspected to be an autoimmune endocrinopathy as concomitant lymphocytic infiltration of the thyroid and pituitary glands has been noted and antipituitary antibodies have been found in some instances. Clinically and radiologically, LYH may mimic a nonsecretory pituitary adenoma. Intraoperative diagnosis may prevent unnecessary excision of potentially functioning pituitary.
PubMed: 36950203
DOI: 10.4103/jmh.jmh_32_21 -
F1000Research 2017The understanding of hypopituitarism has increased over the last three years. This review provides an overview of the most important recent findings. Most of the recent... (Review)
Review
The understanding of hypopituitarism has increased over the last three years. This review provides an overview of the most important recent findings. Most of the recent research in hypopituitarism has focused on genetics. New diagnostic techniques like next-generation sequencing have led to the description of different genetic mutations causative for congenital dysfunction of the pituitary gland while new molecular mechanisms underlying pituitary ontogenesis have also been described. Furthermore, hypopituitarism may occur because of an impairment of the distinctive vascularization of the pituitary gland, especially by disruption of the long vessel connection between the hypothalamus and the pituitary. Controversial findings have been published on post-traumatic hypopituitarism. Moreover, autoimmunity has been discussed in recent years as a possible reason for hypopituitarism. With the use of new drugs such as ipilimumab, hypopituitarism as a side effect of pharmaceuticals has come into focus. Besides new findings on the pathomechanism of hypopituitarism, there are new diagnostic tools in development, such as new growth hormone stimulants that are currently being tested in clinical trials. Moreover, cortisol measurement in scalp hair is a promising tool for monitoring cortisol levels over time.
PubMed: 28299199
DOI: 10.12688/f1000research.9436.1 -
Pituitary Jun 2024We aimed to investigate the prevalence and the diagnostic criteria of hypoprolactinemia in patients with panhypopituitarism and the effects of hypoprolactinemia on...
PURPOSE
We aimed to investigate the prevalence and the diagnostic criteria of hypoprolactinemia in patients with panhypopituitarism and the effects of hypoprolactinemia on depression and sexual functions.
MATERIALS AND METHODS
Forty-eight patients with panhypopituitarism and 20 healthy volunteers were included. Basal hormone levels were measured and a TRH stimulation test was performed. For the evaluation of sexual functions, questionnaries of Female Sexual Functional Index (FSFI) for females and International Erectile Functional Index for males were performed to the subjects. Depressive symptoms were evaluated by Beck Depression Envontory score (BDI-II).
RESULTS
The peak PRL response to TRH stimulation test at 5th percentile in the control group was 18.6 ng/ml in males and 41.6 ng/ml in females and accepted as the cut-offs for sufficient response of PRL. Prolactin was insufficient in 42(87.5%) patients. A basal PRL level of ≤ 5.7 ng/ml in males and 7.11 ng/ml in females was 100% specific in predicting an inadequate response to TRH stimulation test with 80% and 70% sensitivity respectively. A basal PRL level of ≥ 8.5 ng/dl in males was 100% specific and 76% sensitive, and in females a level of ≥ 15.2 ng/dl was 96% specific and 66% sensitive in predicting an adequate response to TRH. PRL deficient patients with panhypopituitarism had higher depression scores compared to the controls, lower sexual function scores in males.
CONCLUSION
PRL deficiency is prevalent among individuals with panhypopituitarism, with the potential to result in elevated depression scores in both sexes and impaired sexual functions in males. A basal PRL level seems to be sufficient for the diagnosis of hypoprolactinemia in routine clinical practice.
Topics: Humans; Male; Hypopituitarism; Female; Prolactin; Adult; Depression; Prevalence; Middle Aged; Thyrotropin-Releasing Hormone; Case-Control Studies; Young Adult
PubMed: 38700812
DOI: 10.1007/s11102-024-01393-0 -
The Medical Journal of Malaysia Jul 2021Madam LPS, a 69 years old lady complained of left eye blurring of vision since January 2017. It was associated with left orbital swelling with chemosis, eye redness,...
Madam LPS, a 69 years old lady complained of left eye blurring of vision since January 2017. It was associated with left orbital swelling with chemosis, eye redness, epiphora, several episodes of self-limiting epistaxis and bilateral ophthalmoplegia. Other neurological examinations and cerebellar systems were intact. Magnetic Resonance Imaging (MRI) Brain and Orbit were performed, depicting a sellar mass with suprasellar extension with blood investigations results showed panhypopituitarism. She underwent bilateral orbital decompression. Trans-nasal endoscopic biopsy showed suppurative granulomatous lesion, which cultured Candida Albicans and Candida Galbrata. She was started on antifungal and hormonal replacement therapy for panhypopituitarism. Unfortunately, she did not respond well to treatment as repeated MRI Brain on December 2018 showed increase in size of sellar mass causing obstructive hydrocephalus and increasing size of left orbital lesion. She was counselled for another debulking surgery with a ventriculoperitoneal (VP) shunt. HPE taken were reported as chronic inflammatory process in favour to fungal infection. Pituitary infections may mimic pituitary mass. Some may exhibit symptoms of panhypopituitarism as well. Thus, physical examination, MRI brain imaging as well as HPE of biopsy are important aids to achieve diagnosis. Optimal treatment of fungal pituitary abscess includes transsphenoidal surgery combined with antifungal therapy.
Topics: Aged; Female; Humans; Hypopituitarism; Magnetic Resonance Imaging; Mycoses; Pituitary Diseases; Pituitary Gland
PubMed: 34305132
DOI: No ID Found -
Internal Medicine (Tokyo, Japan) Mar 2024A 59-year-old man was admitted to our hospital with hyponatremia. An endocrine examination indicated panhypopituitarism, and magnetic resonance imaging revealed a...
A 59-year-old man was admitted to our hospital with hyponatremia. An endocrine examination indicated panhypopituitarism, and magnetic resonance imaging revealed a mass-like lesion in the pituitary gland. Sinus endoscopy revealed a fungal mass in the sphenoid sinus, and the patient was diagnosed with hypopituitarism due to aspergillosis of the central nervous system (CNS). The patient's hyponatremia resolved with hydrocortisone replacement. Although the right internal carotid artery was eventually occluded, antifungal medications were administered for the aspergillosis, and the patient's general condition improved. The patient's CNS lesions have remained under control since discharge. This is the first case to suggest that ACTH secretion may be relatively preserved in Aspergillus-induced hypopituitarism.
PubMed: 38462513
DOI: 10.2169/internalmedicine.3390-23 -
Hormones and the bone marrow: panhypopituitarism and pancytopenia in a man with a pituitary adenoma.Journal of General Internal Medicine May 2015In rare cases, pancytopenia results from hormonal deficiencies that arise in the setting of panhypopituitarism. Here we describe the unusual case of a 60-year-old man... (Review)
Review
In rare cases, pancytopenia results from hormonal deficiencies that arise in the setting of panhypopituitarism. Here we describe the unusual case of a 60-year-old man who presented with progressive fatigue and polyuria, and whose laboratory workup revealed a deficiency of the five hormones associated with the action of the anterior pituitary (thyroid hormone, testosterone, cortisol, prolactin, and insulin-like growth factor-1). Imaging of the pituitary demonstrated a cystic mass consistent with a pituitary adenoma replacing much of the normal pituitary tissue. His symptoms and hematologic abnormalities rapidly resolved with prednisone and levothyroxine supplementation. While the majority of reported cases of panhypopituitarism with bone marrow suppression are the result of peripartum sepsis or hemorrhage leading to pituitary gland necrosis (Sheehan's syndrome), it is also important to consider the diagnosis of hypopituitarism in patients with hypothyroidism, low cortisol levels, and pancytopenia. The causal relationship between pancytopenia and panhypopituitarism is not well understood, though it does reinforce the important influence of these endocrine hormones on the health of the bone marrow.
Topics: Adenoma; Bone Marrow; Drug Therapy, Combination; Hematologic Tests; Humans; Hypopituitarism; Kidney Function Tests; Magnetic Resonance Imaging; Male; Middle Aged; Pancytopenia; Pituitary Neoplasms; Prednisone; Rare Diseases; Thyroxine; Treatment Outcome
PubMed: 25583570
DOI: 10.1007/s11606-014-3161-x -
Proceedings of the Royal Society of... Apr 1949
Topics: Disease; Humans; Hyperthyroidism; Hypopituitarism; Pituitary Diseases; Pituitary Gland; Thyroid Gland
PubMed: 18127917
DOI: No ID Found -
JAMA Network Open Oct 2020Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis affecting multiple organs and commonly caused by somatic pathogenic variants in BRAF V600E and...
IMPORTANCE
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis affecting multiple organs and commonly caused by somatic pathogenic variants in BRAF V600E and mitogen-activated protein kinase genes. Clinical features of ECD result from histiocytic involvement of various tissues; while endocrine involvement in ECD occurs frequently, the prevalence of central or primary hypothyroidism has not been thoroughly investigated.
OBJECTIVE
To assess hypothalamus-pituitary-thyroid (HPT) dysfunction in patients with ECD.
DESIGN, SETTING, AND PARTICIPANTS
This cross-sectional study included 61 patients with ECD who were enrolled in a natural history study at a tertiary care center between January 2011 and December 2018. ECD was diagnosed on the basis of clinical, genetic, and histopathological features. Data were analyzed in March 2020.
EXPOSURE
Diagnosis of ECD.
MAIN OUTCOMES AND MEASURES
Main outcome was the prevalence of thyroid dysfunction in adults with ECD compared with community estimates. Patients underwent baseline evaluation with a thyroid function test, including thyrotropin, free thyroxine (fT4), and total thyroxine (T4), and sellar imaging with magnetic resonance imaging or computed tomography scan. The association of HPT dysfunction was assessed for differences in age, sex, body mass index, BRAF V600E status, high sensitivity C-reactive protein level, sellar imaging, and pituitary hormonal dysfunction.
RESULTS
A total of 61 patients with ECD (46 [75%] men; mean [SD] age, 54.3 [10.9] years) were evaluated. Seventeen patients (28%) had hypothyroidism requiring levothyroxine therapy. The prevalence of both central and primary hypothyroidism were higher than community estimates (central hypothyroidism: 9.8% vs 0.1%; odds ratio, 109.0; 95% CI, 37.4-260.6; P < .001; primary hypothyroidism: 18.0% vs 4.7%; OR, 4.4; 95% CI, 2.1-8.7; P < .001). Patients with hypothyroidism (both primary and central), compared with patients with euthyroidism, had higher body mass index (median [interquartile range] 31.4 [28.3-38.3] vs 26.7 [24.4-31.9]; P = .004) and a higher prevalence of panhypopituitarism (7 [47%] vs 3 [7%]; P < .001). Among patients with hypothyroidism, those with central hypothyroidism, compared with patients with primary hypothyroidism, had a lower mean (SD) body mass index (28.3 [2.6] vs 36.3 [5.9]; P = .007) and higher frequencies of abnormal sellar imaging (5 [83%] vs 3 [27%]; P = .050) and panhypopituitarism (5 [83%] vs 3 [27%]; P = .050).
CONCLUSIONS AND RELEVANCE
In this cohort study, a higher prevalence of central and primary hypothyroidism was identified in patients with ECD compared with the community. There should be a low threshold for testing for hypothyroidism in patients with ECD, and treatment should follow standard guidelines.
Topics: Adult; Causality; Cohort Studies; Cross-Sectional Studies; Disease Progression; Erdheim-Chester Disease; Female; Humans; Hypothyroidism; Male; Prevalence; Thyroid Function Tests
PubMed: 33119105
DOI: 10.1001/jamanetworkopen.2020.19169 -
Cureus Jul 2020Hypopituitarism is a rare disorder. Hypopituitarism can present as a deficiency of individual anterior pituitary hormones (e.g., adrenocorticotropic hormone,...
Hypopituitarism is a rare disorder. Hypopituitarism can present as a deficiency of individual anterior pituitary hormones (e.g., adrenocorticotropic hormone, thyroid-stimulating hormone, luteinizing hormone, follicle-stimulating hormone, prolactin, growth hormone) or posterior pituitary hormones (e.g., oxytocin, vasopressin) or as the deficiency of all these pituitary hormones, also known as panhypopituitarism. Here, we discuss a 59-year-old man who presented with two episodes of unwitnessed syncope after an episode of vomiting. On admission, the patient was hypotensive to 88/54 mmHg, afebrile, and with a leukocyte count of 21.43 K/µL (reference range: 3.80 to 10.50 K/µL). CT scan of the head revealed a hyperdensity in the left intracranial internal carotid artery just proximal to the bifurcation, suggesting an artifact or presence of an embolus. Additional findings included a sellar mass with calcifications and suprasellar extensions. The patient was admitted for further workup of syncope. Other differential diagnoses included sepsis, stroke, cardiac arrhythmias, and pulmonary embolism. Sepsis, stroke, and cardiac workup were negative for significant findings. The patient remained persistently hypotensive despite aggressive intravenous hydration, raising suspicion for an underlying endocrine disorder. MRI of the brain was negative for stroke but again was significant for a sellar mass. Additional workup showed a deficiency of all the anterior pituitary hormones likely secondary to mass effect. The patient was diagnosed with panhypopituitarism due to pituitary macroadenoma.
PubMed: 32789047
DOI: 10.7759/cureus.9102 -
Pediatric Endocrinology, Diabetes, and... 2022Hypopituitarism is a chronic disease characterized by partial or complete absence of secretion of one or more pituitary hormones. Delayed diagnosis can have serious...
INTRODUCTION
Hypopituitarism is a chronic disease characterized by partial or complete absence of secretion of one or more pituitary hormones. Delayed diagnosis can have serious consequences during the neonatal period and adulthood.
MATERIAL AND METHODS
A retrospective study was conducted on patients who had started treatment in the Children's Clinical University Hospital from 1 January 1995 to 31 December 2020. In total 243 patients were included; they were divided into 3 subgroups: isolated growth hormone deficiency, multiple pituitary hormone deficiency, and panhypopituitarism.
RESULTS
The prevalence of hypopituitarism in Latvia is about 45 cases per 100,000 live births. The average detection age of abnormal growth in Latvia is 8 years and 3 months. Most cases had isolated growth hormone deficiency, at 67.1% (n = 163), followed by cases of multiple pituitary hormone deficiency, at 26.3% (n = 64), and cases of panhypopituitarism, at 6.6% (n = 16). Abnormalities in MRI were found in 44.7% (n = 101) of patients. The most best therapeutic effect was achieved in the first year of treatment: with growth of an average of 9.3 cm (+0.1 SD) for isolated growth hormone deficiency, 9.0 cm (+0.6 SD) for multiple pituitary hormone deficiency, and 11.7 cm (+1SD) for patients with panhypopituitarism.
CONCLUSIONS
It is important to increase awareness and promote early diagnosis for hypopituitarism patients in Latvia and in Europe. More attention should be paid to education about growth restriction problems to parents, caregivers, and other specialists. The treatment should be adjusted individually with the focus not only on physical and mental health but also on safety and treatment costs.
Topics: Infant, Newborn; Child; Humans; Retrospective Studies; Dwarfism, Pituitary; Latvia; Hypopituitarism; Hormones
PubMed: 36047289
DOI: 10.5114/pedm.2022.118320