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Introduction: Obesity, dyslipidaemia and insulin resistance are associated with hypopituitarism. The association between these conditions and Sheehan's syndrome (SS) caused by post-partum pituitary gland necrosis is poorly understood. This study aimed to assess cardiovascular risk surrogate markers in SS patients, and we compared clinical, biochemical and radiological testing with healthy controls. Methods: In this cross-sectional study, we studied 45 patients with SS on standard replacement therapy and compared them with healthy controls. All subjects underwent anthropometric, inflammatory marker and hormonal measurement (adrenocorticotropic hormone (ACTH), stimulated cortisol, insulin-like growth factor-1 (IGF-1), thyroxine (T4), follicle-stimulating hormone (FSH), luteinising hormone (LH), oestradiol (E2), prolactin (Prl), insulin, interleukin-6 (IL-6) and high-sensitivity C-reactive protein (hs-CRP)). Carotid intima-media thickness (CIMT), flow-mediated dilation (FMD) and echocardiography were also performed. Results: The mean age and body mass index (BMI) of SS patients were 48.1 ± 10.0 years and 24.3 ± 4.3 kg/m2, respectively, while those of controls were 44.6 ± 12.0 years and 24.6 ± 3.2 kg/m2, respectively. Systolic blood pressure was significantly higher in SS (124.6 ± 20.8 vs. 117.0 ± 18.6 mm of Hg, P < 0.05). All SS patients were hypothyroid, and all except one were hypocortisolaemic. Triglyceride (TG) levels were significantly higher in SS patients (165.6 ± 83.3 vs. 117.2 ± 56.1, P < 0.01), but no difference in the prevalence of metabolic syndrome (MetS) was found. hs-CRP (9.1 (5.2-18.5) vs. 1.5 (0.6-2.8), P < 0.001) and IL-6 (4.9 (3.7-7.3) vs. 3.1 (2.0-4.2), P < 0.001) were significantly higher in SS patients. CIMT was significantly increased in SS patients, but no difference in FMD was found. Echocardiography revealed no significant difference in left ventricular (LV) dimensions, interventricular thickness, posterior wall thickness, ejection fraction, LV mass and diastolic function. Conclusion: SS patients show increased cardiovascular risk with hypertension, dyslipidaemia and increased atherosclerotic and inflammatory markers.
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OBJECTIVE: Optic nerve hypoplasia (ONH), the congenital underdevelopment of the optic nerve, is an increasing cause of visual impairment and is associated with pituitary dysfunction. Past studies have focused on the relationship between ONH, pituitary deficiencies, and brain imaging. However, recent studies have demonstrated the true risk for hypopituitarism lies with the presence or absence of ONH, irrespective of midline brain findings. This study reviewed the relationship between the health of the optic nerve (visual acuity) and pituitary gland (number and age of development of pituitary deficiencies) as a way to stratify risk, regardless of imaging findings. DESIGN, PATIENTS AND MEASUREMENTS: Retrospective chart review of 197 patients seen at a single center from 2013 to 2022. Visual assessment was defined by distance acuity, and the presence of nystagmus or afferent pupillary defect. Pituitary deficiencies were diagnosed per Endocrine Society guidelines. RESULTS: In children with bilateral ONH (bONH), profound visual impairment was associated with more pituitary deficiencies between 0 and 15 years of age. The odds of having any pituitary deficiency were 4.9 times higher (95% confidence interval [95% CI]: 2.4-10.1) for patients with bONH versus unilateral ONH (uONH). Central hypothyroidism was the most common first presenting pituitary deficiency followed by growth hormone across all patients. CONCLUSION: This study shows a significant association between severity of visual impairment and increased probability of pituitary deficiencies in children with bONH versus uONH. Children with ONH require urgent endocrine evaluation due to risk of pituitary deficiencies, but risk stratification may also be based on severity of visual impairment.
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Introduction: Ectopic posterior pituitary (EPP) is a rare congenital abnormality, sometimes associated with other midline defects, such as pituitary stalk interruption syndrome (PSIS), in which thin or absent pituitary stalk and anterior pituitary hypoplasia are combined to EPP. Most cases are sporadic, with few reports of familial cases, and many congenital hypopituitarism (CH) cases remain unsolved. Objective: To search for candidate genes associated with this condition, we performed trio-based whole-exome sequencing (WES) on patients with EPP, including two familial cases. Methods: This study included subjects with EPP and PSIS diagnosed by a simple MRI protocol (FAST1.2). We performed two distinct analyses in the trio-based WES. We looked for previously described genes associated with pituitary development. Next, we investigated the whole exome for variants inherited in a pattern consistent with a monogenic etiology. Results: Ten families were evaluated; eight were composed of a child with EPP and healthy parents, one has two affected siblings, and one family has a son and mother with EPP. When analyzing the previously described candidate variants associated with pituitary development, we found variants in GLI2 and FGFR1 in three families. We also found six other variants of interest in three patients: KMT2A, GALR3, RTN4R, SEMA3A, NIPBL, and DSCAML1. Conclusion: The analysis allowed us to find previously reported and not reported GLI2 variants, all inherited from healthy parents, which reinforces the incomplete penetrance pattern of GLI2 variants in the development of EPP and draws attention to possible future functional studies of those variants that have a recurrent expression in CH. We also found novel FGFR1 and SEMA3A variants that suggest an oligogenic mechanism in PSIS and EPP, as seen in patients with hypogonadotropic hypogonadism. We report the first case of a patient with Wiedemann-Steiner syndrome and PSIS, suggesting that the KMT2A gene may be related to pituitary development. Furthermore, the trios' analysis allowed us to find five other variants of interest. Future investigations may clarify the roles of these variants in the etiology of EPP and PSIS.
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OBJECTIVE: Anterior pituitary dysfunction is one of the major causes of disability and morbidity in patients suffering from traumatic brain injury (TBI). The present study was undertaken to evaluate the incidence of anterior pituitary dysfunction in cases of moderate and severe TBI, its value in long-term prognostication, and the factors that predispose to a higher incidence of anterior pituitary dysfunction in acute and chronic phases. METHODS: This was a prospective cohort study wherein 216 patients with moderate and severe TBI were evaluated within 72 hours of TBI (acute phase) and at 6 months (chronic phase). RESULTS: At 6 months, out of the 216 patients, 95 patients had expired and 35 patients were lost to follow-up. The remaining 86 patients were evaluated at 6 months. In the acute phase, hypopituitarism was seen in 82.4% patients, thyroid axis deficiency was seen in 57.4% patients, gonadal axis deficiency in 54.2% patients, and adrenal axis deficiency in 13.8% patients. In the chronic phase, hypopituitarism was seen in 59.3% patients, thyroid axis deficiency was seen in 24.4% patients, gonadal axis deficiency in 32.6% patients, and adrenal axis deficiency in 23.3% patients. Patients with thyroid axis deficiency at admission had significant association with a bad modified Rankin Scale score at 6 months. CONCLUSIONS: Thyroid and gonadotropin axes were most commonly affected and deficiency of at least 1 axis was found in 82.4% patients in the acute phase and 59.3% in the chronic phase. Thyroid axis deficiency had a negative impact on prognosis in post-TBI patients.
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Langerhans cell histiocytosis (LCH) is a rare disorder involving an abnormal clonal proliferation of precursor cells of the mononuclear phagocytic system. The hypothalamic-pituitary axis is commonly affected by central nervous system (CNS) involvement, with central diabetes insipidus being the most common endocrine abnormality observed. We report the case of a 55-year-old female presenting with vision changes and found to have a hypothalamic mass that was responsive to high-dose steroids. After an initial diagnostic dilemma, the surgical pathology eventually confirmed the diagnosis of LCH. She is being treated with hormone supplementation for panhypopituitarism and intensity-modulated radiation therapy (IMRT) for the LCH. Our case highlights that LCH can present as isolated hypothalamic-pituitary involvement. Early diagnosis is critical to prevent extensive progression of the disease, ultimately leading to permanent physical and endocrine abnormalities. More studies are required to develop specific guidelines and approaches for patients with isolated hypothalamic-pituitary involvement due to LCH.
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Neurosarcoidosis (NS) with hypothalamic-pituitary (HP) involvement (HP-NS) is a rare clinical condition, conferring variable hormonal deficits that are typically irreversible. Here, we present 2 cases of NS with panhypopituitarism. The first patient presented with cauda equina syndrome and arginine vasopressin deficiency, while the second developed recurrent optic neuritis and vision loss in the setting of a sellar mass. In the first case, neurological symptoms resolved after therapy with high-dose glucocorticoids, infliximab, and methotrexate; while in the second, visual restoration followed resection of the granulomatous tissue and immunosuppressive therapy. In both cases, pituitary dysfunction persisted despite neurological improvement. We contextualized the presentations and outcomes through a literature review of HP-NS case reports and case series. This revealed high rates of extraneurologic sarcoidosis in HP-NS patients with panhypopituitarism, while underscoring the need for hormonal replacement-as endocrinopathies rarely respond to sarcoidosis-directed immunosuppression.
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Adipsic diabetes insipidus (ADI) is characterized by central diabetes insipidus and an impaired thirst response to hyperosmolality, leading to hypernatremia. Hyponatremia observed in patients with ADI has been considered a complication of desmopressin therapy. Herein, we present a case of impaired thirst sensation and arginine vasopressin (AVP) secretion without desmopressin therapy, in which hyponatremia developed due to preserved non-osmotic AVP secretion. A 53-year-old woman with hypopituitarism, receiving hydrocortisone and levothyroxine, experienced hyponatremia three times over 5 months without desmopressin treatment. The first hyponatremic episode (120 mEq/L) was complicated by a urinary tract infection with a plasma AVP level of 33.8 pg/mL. Subsequent hyponatremia episodes occurred after administration of antipsychotic (124 mEq/L) and spontaneously (125 mEq/L) with unsuppressed plasma AVP levels (1.3 and 1.8 pg/mL, respectively). Hypertonic saline infusion did not affect AVP or copeptin levels. Regulating water intake using a sliding scale based on body weight prevented the recurrence of hyponatremia without the use of desmopressin. Except during infection, plasma AVP levels (1.3 ± 0.4 pg/mL) were not significantly correlated with serum sodium levels (rs = -0.04, p = 0.85). In conclusion, we present a unique case of impaired thirst sensation and AVP secretion in which hyponatremia developed without desmopressin therapy. Preserved non-osmotic AVP secretion, possibly stimulated by glucocorticoid deficiency, may contribute to the development of hyponatremia in patients with ADI.
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Both local and external cranial radiotherapy (RT) can induce neurotoxicity and vascular damage of the hypothalamic-pituitary area, which can promote neuroendocrine alterations. While anterior pituitary insufficiency after RT has been extensively characterized, data on the effect of RT on prolactin (PRL) secretion are limited and heterogeneous, with different patterns of PRL behavior described in the literature. A progressive decline in PRL levels, reflecting a time-dependent, slowly evolving radiation-induced damage to the pituitary lactotroph cells has been reported. To date, the association between hypopituitarism and hypoprolactinemia in patients undergoing RT has not yet been fully investigated. The few available data suggest that lower PRL levels can predict an extent damage of the pituitary tissue and a higher degree of hypothalamic dysfunction. However, most studies on the effect of RT on pituitary function do not properly assess PRL secretion, as PRL deficiency is usually detected as part of hypopituitarism and not systematically investigated as an isolated disorder, which may lead to an underestimation of hypoprolactinemia after RT. In addition, the often-inadequate follow-up over a long period of time may contribute to the non-recognition of PRL deficiency after RT. Considering that hypoprolactinemia is associated with various metabolic complications, there is a need to define appropriate diagnostic and management criteria. Therefore, hypoprolactinemia should enter in the clinical investigation of patients at risk for hypopituitarism, mainly in those patients who underwent RT.
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Secondary adrenal insufficiency (SAI) is an endocrine disorder due to impaired secretion of ACTH resulting from any disease affecting the pituitary gland. Glucocorticoid replacement therapy is mandatory to ensure patient survival, haemodynamic stability, and quality of life. In fact, a correct dose adjustement is mandatory due to the fact that inappropriately low doses expose patients to hypoadrenal crisis, while inappropriately high doses contribute to glucose metabolic and cardiovascular deterioration. This review analyses the current evidence from available publications on the epidemiology and aetiology of SAI and examines the association between glucocorticoid replacement therapy and glucometabolic and cardiovascular effects.
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OBJECTIVE: The aim of this study was to investigate the incidence and risk factors of new-onset hypopituitarism after gamma knife radiosurgery (GKRS) for pituitary adenomas in a single center. METHODS: In this retrospective study, 241 pituitary adenoma patients who underwent GKRS from 1993 to 2016 were enrolled. These patients had complete endocrine, imaging, and clinical data before and after GKRS. The median follow-up time was 56.0 (range, 12.7-297.6) months. RESULTS: Fifty patients (20.7%) developed new-onset hypopituitarism after GKRS, including hypogonadism (n = 22), hypothyroidism (n = 29), hypocortisolism (n = 20), and growth hormone deficiency (n = 4). The median time to new-onset hypopituitarism was 44.1 (range, 13.5-141.4) months. The rates of new-onset hypopituitarism were 7%, 16%, 20%, 39%, and 45% at 1, 3, 5, 10, and 15 years, respectively. For those patients treated with a single GKRS, sex (p = 0.012), suprasellar extension (p = 0.048), tumor volume (≥ 5 cm3) (p < 0.001), tumor progression (p = 0.001), pre-existing hypopituitarism (p = 0.011), and previous surgery (p = 0.009) were significantly associated with new-onset hypopituitarism in univariate analysis. In the multivariate analysis, tumor volume (≥ 5 cm3) and tumor progression were associated with new-onset hypopituitarism (hazard ratio [HR] = 3.401, 95% confidence interval [CI] = 1.708-6.773, p < 0.001 and HR = 3.594, 95% CI = 1.032-12.516, p = 0.045, respectively). For patients who received 2 or more times GKRS, no risk factors associated with new-onset hypopituitarism were found. CONCLUSION: New-onset hypopituitarism was not uncommon after GKRS for pituitary adenomas. In this study, large tumor volume (≥ 5 cm3) and tumor progression were associated with new-onset hypopituitarism after a single GKRS.
Subject(s)
Adenoma , Hypopituitarism , Pituitary Neoplasms , Radiosurgery , Humans , Hypopituitarism/etiology , Hypopituitarism/epidemiology , Radiosurgery/adverse effects , Male , Female , Middle Aged , Pituitary Neoplasms/surgery , Adenoma/surgery , Adenoma/pathology , Adult , Retrospective Studies , Aged , Risk Factors , Follow-Up Studies , Young Adult , Adolescent , Incidence , Aged, 80 and over , Postoperative Complications/etiology , Postoperative Complications/epidemiology , Treatment OutcomeABSTRACT
A middle-aged woman presented with history of fatigue, low mood, swelling of limbs, and facial puffiness. On detailed history taking, she also complained of salt craving, secondary amenorrhea, and loss of libido for almost a decade. Investigations revealed pan-hypopituitarism. She was started on appropriate hormonal therapy which saw a rapid resolution of symptoms within 2 weeks. Sheehan's syndrome may have an acute presentation or chronic. The symptoms may be subtle like fatigue or overt like hypotension and syncope. A high degree of suspicion of Sheehan's syndrome is essential for its timely management, and goes a long way in preserving the quality of life.
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Craniopharyngiomas are rare hypothalamic-pituitary tumors found in young children, adolescents and adults, and their multidisciplinary management required, calls for consistent practices for practicioners, patients and families. The French Endocrine Society and French Society for Pediatric Endocrinology & Diabetes enlisted and coordinated adult and paediatric endocrinologists, neurosurgeons, pathologists, radiotherapists as well as psychologists, dieticians and a patient association, to draft a reference document on this severe disease. The management of craniopharyngiomas remains complex due to their aggressive nature, invasive behavior, and propensity for recurrence, requiring a sequential and measured therapeutic approach and follow-up in expert centers. Although patient survival rates are high, the consequences of both the tumor and its treatment can lead to serious comorbidities and impaired quality of life, particularly in those patients with lesional hypothalamic syndrome. Recent advances have allowed the two described tumor types - papillary and adamantinomatous - to be associated with distinct molecular signatures, specific pathophysiological mechanisms and ipso facto, distinct therapeutic approaches, including innovative medications for hyperphagia, that will continue to evolve. This consensus statement covers all stages in the management of patients with craniopharyngioma, from diagnosis to therapeutic strategies including the long-term follow-up.
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INTRODUCTION: Hypophysitis is an inflammatory disorder of the pituitary gland. It can manifest variously, with endocrinological and neuro-ophthalmologic symptoms and signs, due to the compression of sellar and parasellar structures. CASE REPRESENTATION: Although hypophysitis is rare, this pituitary disease can occur during pregnancy or in the postpartum period. In this report, we describe the case of a woman with partial hypopituitarism secondary to autoimmune hypophysitis who, five years after the diagnosis and the immunosuppressive treatment, had an uneventful pregnancy and successfully delivered a healthy infant at term. CONCLUSION: We reported the clinical history of the patient and the evolution of the disease and also reviewed the management and treatment of autoimmune hypophysitis during pregnancy.
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Prolactin is a polypeptide hormone composed of 199 amino acids, synthesized by lactotroph cells. Its primary effects are on the mammary gland and gonadal axes, but it also influences different organs and systems, particularly metabolic functions. Current literature has mainly focused on the diagnosis, monitoring, and treatment of hyperprolactinemia. Due to the lack of a well-established effective treatment for hypoprolactinemia, it is not clinically emphasized. Therefore, data on its diagnosis is limited. Hypoprolactinemia has been associated with metabolic dysfunctions such as type 2 diabetes mellitus, fatty liver, dyslipidemia, fertility problems, sexual dysfunction, and increased cardiovascular disease. While often seen as a part of combined hormone deficiencies due to pituitary damage, isolated prolactin deficiency is rare. Hypoprolactinemia can serve as a marker for extensive pituitary gland damage and dysfunction.Low or undetectable serum prolactin levels and the absence of a sufficient prolactin peak in the thyrotropin-releasing hormone (TRH) stimulation test are considered diagnostic for hypoprolactinemia. Gender appears to influence both basal prolactin levels and TRH stimulation test responses. Basal prolactin levels of, at least, 5 ng/mL for males and 7 ng/mL for females can be used as cut-off levels for normal prolactin reserve. Minimum peak prolactin responses of 18 ng/mL for males and 41 ng/mL for females to TRH stimulation can exclude hypoprolactinemia. However, larger population studies across different age groups and sexes are needed to better define normal basal prolactin levels and prolactin responses to the TRH stimulation test.
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Immune checkpoint inhibitors have revolutionized cancer therapy but are associated with a risk of endocrine immune-related adverse events, including pituitary complications. Autoimmune hypophysitis, traditionally a rare diagnosis, has become a more frequently encountered clinical entity with the emergence of antitumor immunotherapy. This mini-review aims to consolidate current knowledge, encompassing the epidemiology, pathophysiology, clinical presentation, diagnosis, and management of pituitary complications of immune checkpoint inhibitor use.
Subject(s)
Immune Checkpoint Inhibitors , Humans , Immune Checkpoint Inhibitors/adverse effects , Autoimmune Hypophysitis/chemically induced , Pituitary Diseases/chemically induced , Immunotherapy/adverse effects , Neoplasms/drug therapyABSTRACT
â¢Oxytocin is a multifaceted hypothalamic-pituitary hormone involved in energy homeostasis, mental health, and bone metabolism.â¢Oxytocin deficiency in energy deficit states and in hypopituitarism is associated with worse mental health and bone health.â¢Oxytocin modulates appetitive neurocircuitry, improves impulse control, and reduces food intake in humans.â¢Defining the oxytocin system in human physiology and pathophysiology could lead to novel therapeutic strategies.
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INTRODUCTION: Excess growth hormone (GH) secretion in acromegaly has a major impact on mineral balance and serum phosphate levels. However, the clinical utilization of serum phosphate levels as a marker for long-term disease outcomes in acromegaly has not been evaluated. METHODS: This is a retrospective study of patients with acromegaly who were followed in a tertiary center. Data were retrieved on patient characteristics, endocrine and biochemical evaluation, and tumor parameters. Comparisons were performed by measuring baseline phosphate levels and conducting correlation analysis and multivariable logistic regression. RESULTS: Sixty-one patients were followed for 4.5 years (range 1-21). Patients with hyperphosphatemia (> 4.5 mg/dl) at baseline had larger adenomas (15.0 mm [8.0, 47.0] vs. 10.0 mm [3.0, 24.0], p = 0.001), a rate chance of invasive adenoma (16 [80.0%] vs. 14 [46.7%], p = 0.02), and lower serum cortisol levels (226.0 nmol/l [27.6, 516.0] vs. 294.0 nmol/l [32.0, 610.0], p = 0.02). Baseline serum phosphate levels positively correlated with IGF-1 levels (r = 0.43, p = 0.003) and negatively correlated with morning plasma cortisol levels (r = -0.46, p = 0.002). Regarding long-term impact, baseline phosphate levels correlated with the number of pituitary axes involved 6 months after diagnosis (r-0.34, p = 0.01). In multivariable analysis, baseline plasma phosphate levels were independently associated with risk for disease progression/recurrence (odds ratio [OR] 9.66, 95% confidence interval [CI] 1.5, 105.9, p = 0.03) and for invasive adenoma (OR 6.21, 95% CI 1.6, 28.7, p = 0.01). CONCLUSION: Elevated pretreatment serum phosphate levels are associated with a greater risk of disease persistence and recurrence and with altered pituitary function in patients with acromegaly.
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PURPOSE: Successful treatment outcomes of adults with hypothalamic-pituitary disorders necessitate the adoption of intricate self-management behaviors, yet current scales for evaluating treatment adherence and satisfaction are inadequate for this patient group. This research introduces a novel treatment adherence, satisfaction and knowledge questionnaire (TASK-Q) developed specifically to identify patients' unmet needs in better assessing and managing these disorders. METHODS: The study was conducted in three phases: (1) generating items and testing content validity, (2) refining these items through a pilot study, and (3) a main study evaluating the psychometric properties of the TASK-Q scale among 262 adults in a Pituitary Nurse-led Clinic, with 152 (58%) patients completing the questionnaire. RESULTS: Exploratory factor analysis was used to test the factor structure and construct validity of the TASK-Q, revealing a 22-item scale divided into Satisfaction and Knowledge (17 items) and Adherence (5 items) subscales, and exhibiting high internal consistency (Cronbach's α = 0.90). Significant correlations were identified between satisfaction and knowledge (r = 0.67, p < 0.001), satisfaction and adherence (r = 0.23, p = 0.005), and knowledge and adherence (r = 0.43, p < 0.001). Complex treatment regimens, like daily growth hormone injections and adjusting glucocorticoids during illness, negatively affected adherence (p < 0.001). CONCLUSION: The TASK-Q is a novel validated scale that can effectively evaluate patients' perspectives on adherence, knowledge and satisfaction. Our findings highlight the significant impact of Advanced Nurse Practitioners in improving patient self-management behaviors, which likely leads to better treatment outcomes for people with hypothalamic-pituitary disorders.
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BACKGROUND: Pituitary apoplexy (PA) is characterized by acute hemorrhage or infarction of the pituitary gland. Management can be either conservative or surgical. Evidence favoring either is still limited to observational studies. This meta-analysis evaluates the effectiveness of both approaches on patient outcomes. METHODS: A systematic search was performed until February 2024. We included cohort studies of patients with PA. Patients were divided into 2 groups: a conservative management group and a surgery group, including early and late surgery. Outcomes of interest were assessed categorically using risk ratio (RR) and Mantel-Haenszel's random effects model. RESULTS: Of the 273 published articles, 15 cohort studies comprising 908 patients were included. There was no statistically significant difference between groups in recovery of ophthalmoplegia (RR=1.09, confidence interval [CI]=1.00-1.18, P=0.05), visual field (RR=1.09, CI=0.91-1.3, P=0.35), visual acuity (RR=1.05, CI=0.87-1.26, P=0.61), hypopituitarism (RR=1.37, CI=0.81-2.32, P=0.25), and tumor recurrence (RR=0.74, CI=0.34-1.61, P=0.45). This was similar for conservative management versus early surgery in recovery of visual field (RR=0.92, CI=0.62-1.37, P=0.68), visual acuity (RR=1.01, CI=0.81-1.26, P=0.93), and ophthalmoplegia (RR=0.92, CI=0.53-1.61, P=0.77). CONCLUSIONS: Both interventions provide comparable outcomes. These findings, though, are drawn from observational studies, and more severe cases typically undergo surgery. Larger studies are necessary to provide conclusive evidence.
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Sheehan's syndrome (SS) is a condition characterized by panhypopituitarism that generally occurs after an episode of postpartum bleeding. There are certain hypotheses regarding the development of SS in the postpartum period. Coagulation factor abnormalities have been reported to be associated with SS. Associated hypothyroidism and hypocortisolism have been found to cause coagulation abnormalities. After the correction of the hypothyroidism and hypocortisolism, there is a gradual correction of the coagulation abnormality. In our case, a middle-aged woman presented with recurrent episodes of hospital admission because of generalized weakness and fever. She was found to have a biochemistry profile suggestive of hypopituitarism with preserved gonadal function. Her hemogram was normal, but the coagulogram showed a prolongation of activated partial thromboplastin time with a near-normal prothrombin time. She was evaluated and found to have factor XI deficiency. In the background of excessive vaginal bleeding and hypopituitarism, a diagnosis of SS was made. The presence of factor XI deficiency may have led to excessive bleeding and the development of SS. To the best of our knowledge, there is no reported association of factor XI deficiency with SS in the literature, and this is the first reported case.