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JAAD Case Reports Sep 2023
PubMed: 37680570
DOI: 10.1016/j.jdcr.2023.07.003 -
Annals of Medicine and Surgery (2012) Aug 2023Munchausen syndrome is known as a factitious disorder imposed on the self. Factitious Cushing's syndrome (CS) is a very rare form of Munchausen syndrome, presenting with...
UNLABELLED
Munchausen syndrome is known as a factitious disorder imposed on the self. Factitious Cushing's syndrome (CS) is a very rare form of Munchausen syndrome, presenting with varied clinical and biochemical features, making diagnosis challenging.
CASE PRESENTATION
A 40-year-old female patient who worked as a registered nurse presented with clinical features of CS but denied any exogenous corticosteroid use. The endocrine workup revealed that the patient had a high 24 h urinary-free cortisol collection before admission. Subsequent evaluations showed low levels of morning cortisol and plasma adrenocorticotropic hormone along with a suppressed overnight low-dose dexamethasone suppression test, leading to an investigation of hypercortisolism. Unexpectedly, subsequent testing showed a normal 24 h urinary-free cortisol level. Additionally, the patient was diagnosed with panhypopituitarism, the radiological investigations showed normal pituitary and adrenal glands. Despite consistently denying the use of corticosteroids, it was finally discovered that the patient had been surreptitiously taking prednisone and receiving multiple dexamethasone injections over the past few months. The patient received treatment through a gradual prednisone tapering regimen, accompanied by comprehensive psychiatric evaluation and management.
CONCLUSION
This case underscores the exceptional rarity of factitious CS and emphasizes the importance of considering it as a potential differential diagnosis in hypercortisolism cases, particularly when the patient's medical history contradicts investigative findings. Furthermore, it highlights the criticality of adopting a multidisciplinary approach to investigate patients whose clinical presentation aligns with factitious CS.
PubMed: 37554867
DOI: 10.1097/MS9.0000000000001050 -
Brain Tumor Research and Treatment Jul 2023The World Health Organization (WHO) updated the classification of pituitary tumors in 2022. The new classification presents detailed histological subtyping of a... (Review)
Review
The World Health Organization (WHO) updated the classification of pituitary tumors in 2022. The new classification presents detailed histological subtyping of a pituitary neuroendocrine tumor (PitNET) based on the tumor cell lineage, cell type, and related characteristics. The immunohistochemistry for pituitary transcription factors (PIT1, TPIT, SF1, GATA3, and ERα) is routinely needed in this classification. The controversy regarding the change of behavior code of all PitNET/pituitary adenoma from "0" for benign tumors to "3" for primary malignant tumors is a topic of debate among experts, nowadays. Some authors represent that pituitary adenoma has a tendency for hemorrhage and necrosis and frequent invasion of the cavernous sinus. However, most small PitNET/pituitary adenoma do not need any treatment because of benign biologic behavior or less than 5% recurrence after gross total removal. Pituitary apoplexy is also benign nature but has a tendency of cranial nerve compression or panhypopituitarism. Most of cavernous invasion is compression of the cavernous sinus. Aggressive PitNET/pituitary adenoma with malignant biological behavior is less than 1%.
PubMed: 37550816
DOI: 10.14791/btrt.2023.0015 -
Radiology Case Reports Oct 2023A 38-year-old man who was delivered in a breech position presented with delayed development of secondary sexual characteristics and malaise. He was diagnosed with...
A 38-year-old man who was delivered in a breech position presented with delayed development of secondary sexual characteristics and malaise. He was diagnosed with panhypopituitarism caused by interruption of the pituitary stalk due to perinatal complications. Brain magnetic resonance imaging findings for pituitary stalk interruption syndrome are well-documented; however, reports of the imaging findings of the bones and several organs related to the effects of panhypopituitarism are limited. In this patient with anterior pituitary dysfunction, imaging revealed diverse sequelae, including delayed skeletal maturation, osteopenia, genital atrophy, fatty liver, and adrenal atrophy. Radiologists may find it difficult to discern complex imaging findings unless they are informed of the clinical course of the patient. Therefore, radiologists should coordinate with clinicians to arrive at a diagnosis.
PubMed: 37547790
DOI: 10.1016/j.radcr.2023.07.039 -
SAGE Open Medical Case Reports 2023Hypogonadotropic hypogonadism is a common finding in patients who are diagnosed with a prolactinoma. It can be accompanied by the presence of other pituitary hormone...
Hypogonadotropic hypogonadism is a common finding in patients who are diagnosed with a prolactinoma. It can be accompanied by the presence of other pituitary hormone deficits, including secondary adrenal insufficiency and central hypothyroidism. While the proportion of improvement in endocrine deficits over the short term is well characterized, there is not enough literature about the recovery of pituitary function over the longer term. We present the case of a 23-year-old man with a giant prolactinoma who initially presented with pituitary hemorrhage and panhypopituitarism. He underwent decompression of the pituitary tumor followed by treatment with cabergoline. Over a 9-year follow-up period, we noted that the hypogonadotropic hypogonadism resolved after 4 years and the secondary adrenal insufficiency resolved after 8 years. This case suggests that partial or complete recovery of the pituitary function is possible over the long-term even in patients with a giant prolactinoma.
PubMed: 37533485
DOI: 10.1177/2050313X231190672 -
Endocrine Connections Oct 2023Hypothalamic dysfunction is a rare condition and can be encountered in patients who have been diagnosed or treated for a suprasellar brain tumor. Due to its rarity, the...
OBJECTIVE
Hypothalamic dysfunction is a rare condition and can be encountered in patients who have been diagnosed or treated for a suprasellar brain tumor. Due to its rarity, the signs and symptoms of hypothalamic dysfunction may be difficult to recognize, leading to delayed diagnosis of the suprasellar brain tumor or to difficulties in finding the health-care expertise for hypothalamic dysfunction after tumor treatment. To improve the care and outcome of patients with acquired hypothalamic dysfunction, professionals are required to understand the patient's needs.
DESIGN
A worldwide online survey was distributed from April 2022 to October 2022 to patients with childhood-onset hypothalamic dysfunction (as reported by the patient) following a brain tumor.
METHODS
Patients were notified about the survey through patient advocacy groups, the SIOPe craniopharyngioma working group and the Endo-ERN platform.
RESULTS
In total, 353 patients with hypothalamic dysfunction following craniopharyngioma (82.2%), low-grade glioma (3.1%) or a pituitary tumor (8.2%) or caregivers responded to the survey. Sixty-two percent had panhypopituitarism. Obesity (50.7%) and fatigue (48.2%) were considered the most important health problems. Unmet needs were reported for help with diet, exercise and psychosocial issues. Patients' suggestions for future research include new treatments for hypothalamic obesity and alternative ways for hormone administration.
CONCLUSIONS
According to the patient's perspective, care for acquired hypothalamic dysfunction can be improved if delivered by experts with a holistic view of the patient in a multidisciplinary setting with a focus on quality of life. Future care and research on hypothalamic dysfunction must integrate the patients' unmet needs.
SIGNIFICANCE STATEMENT
Patients with hypothalamic dysfunction may experience a variety of symptoms, which are not always adequately recognized or addressed. In previous papers, the perspective of caregivers of children with craniopharyngioma has been reported (Klages et al. 2022, Craven et al. 2022). Now we address the patients' perspective on acquired hypothalamic dysfunction using an Endo-ERN global survey. According to the patients' perspective, care can be improved, with needs for improvement in the domains of obesity, fatigue and lifestyle. Research may focus on ways to improve hypothalamic obesity and alternative ways for hormone administration. Ideally, care should be delivered by doctors who have a holistic view of the patient in a multidisciplinary expert team. The results of this study can be used to formulate best practices for clinical care and to design future research proposals.
PubMed: 37531603
DOI: 10.1530/EC-23-0147 -
Journal of Medical Cases Jun 2023Hyperbilirubinemia and transaminitis are rarely associated with a disorder of endocrine function. It mostly manifests as a cholestatic pattern of liver injury. Herein, a...
Hyperbilirubinemia and transaminitis are rarely associated with a disorder of endocrine function. It mostly manifests as a cholestatic pattern of liver injury. Herein, a 25-year-old female patient with a past medical history of congenital hypopituitarism due to pituitary ectopia presented with serum direct bilirubin level of 9.9 mg/dL and aspartate transaminase (AST)/alanine transaminase (ALT) of 60/47 U/L. All tests for chronic liver disease imaging and liver biopsy were normal. She was found to have central hypothyroidism and low cortisol level. She was started on intravenous (IV) levothyroxine 75 µg daily and IV hydrocortisone 10-5 mg AM/PM. She was discharged on oral levothyroxine 88 µg daily and hydrocortisone orally 10 mg twice daily. Follow-up labs 1 month later showed completely normal liver function test. In conclusion, hyperbilirubinemia due to congenital hypopituitarism can occur in adults. Delayed recognition of underlying endocrine disorder as a cause of hyperbilirubinemia and hepatocellular inflammation can result in end-stage liver damage due to prolonged cholestasis.
PubMed: 37435107
DOI: 10.14740/jmc4102 -
Journal of Vector Borne Diseases 2023Sheehan's syndrome is a pituitary disease resulting from severe postpartum hemorrhage and can present with varying degrees of pituitary insufficiency. Although its... (Review)
Review
Sheehan's syndrome is a pituitary disease resulting from severe postpartum hemorrhage and can present with varying degrees of pituitary insufficiency. Although its incidence is decreasing in developed countries, it continues to be one of the most common causes of hypopituitarism in underdeveloped and developing countries. Here, we report a case of Sheehan's syndrome which was diagnosed following an episode of severe dengue infection, in a 38-year-old female.
Topics: Pregnancy; Female; Humans; Adult; Hypopituitarism; Postpartum Hemorrhage; Dengue
PubMed: 37417174
DOI: 10.4103/0972-9062.364757 -
Clinical Case Reports Jul 2023We reported a 23-year-old male patient with panhypopituitarism who underwent two resections for craniopharyngioma and received postoperative hormone replacement therapy....
We reported a 23-year-old male patient with panhypopituitarism who underwent two resections for craniopharyngioma and received postoperative hormone replacement therapy. The Tc-MDP bone scan revealed focal high uptake of radioactive nuclide in multiple large joints. The SPECT/CT demonstrated the focal high uptake in their metaphysis. Thus, delayed epiphyseal closure was considered.
PubMed: 37415579
DOI: 10.1002/ccr3.7644 -
Journal of Neurological Surgery. Part... Aug 2023Wide variations exist in the management of craniopharyngiomas, including pituitary stalk preservation/sacrifice. This study examines the practice patterns over 16...
Wide variations exist in the management of craniopharyngiomas, including pituitary stalk preservation/sacrifice. This study examines the practice patterns over 16 years using the endoscopic endonasal approach for the resection of craniopharyngiomas and it examines the effects of stalk preservation. Retrospective analysis was conducted for 66 patients who underwent endoscopic transsphenoidal surgery for resection of craniopharyngiomas. Patients were stratified into three epochs: 2005 to 2009 ( = 20), 2010 to 2015 ( = 23), and 2016 to 2020 ( = 20), to examine the evolution of surgical outcomes. Subgroup analysis between stalk preservation/stalk sacrifice was conducted for rate of gross total resection, anterior pituitary function preservation, and development of new permanent diabetes insipidus. Gross total resection rates across the first, second, and third epochs were 20, 65, and 52%, respectively ( = 0.042). Stalk preservation across epochs were 100, 5.9, and 52.6% ( = 0.0001). New permanent diabetes insipidus did not significantly change across epochs (37.5, 68.4, 71.4%; = 0.078). Preservation of normal endocrine function across epochs was 25, 0, and 23.8%; ( = 0.001). Postoperative cerebrospinal fluid (CSF) leaks significantly decreased over time (40, 4.5, and 0%; [ = 0.0001]). Stalk preservation group retained higher normal endocrine function (40.9 vs. 0%; = 0.001) and less normal-preoperative to postoperative panhypopituitarism (18.4 vs. 56%; = 0.001). Stalk sacrifice group achieved higher GTR (70.8 vs. 28%, = 0.005). At last follow-up, there was no difference in recurrence/progression rates between the two groups. There is a continuous evolution in the management of craniopharyngiomas. Gross total resection, higher rates of pituitary stalk and hormonal preservation, and low rates of postoperative CSF leak can be achieved with increased surgical experience.
PubMed: 37405242
DOI: 10.1055/s-0042-1751291