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Clinical Neurology and Neurosurgery Oct 2023Rathke's cleft cyst is rare, with variable characteristics and no unified categorization system. This study aimed to evaluate long-term outcomes, based on different...
BACKGROUND
Rathke's cleft cyst is rare, with variable characteristics and no unified categorization system. This study aimed to evaluate long-term outcomes, based on different categorizations, of symptomatic Rathke's cleft cysts treated with endoscopic endonasal approach.
METHODS
This retrospective study of 38 patients with symptomatic Rathke's cleft cyst treated with endoscopic endonasal approach from 2006/06-2021/08 recorded pre- and post-operative clinical presentation, endocrine and visual tests, radiological findings, and resection status. Rathke's cleft cysts were categorized by both cyst consistency and radiological features and clinical characteristics were analyzed.
RESULTS
The most common pre-operative symptoms were visual field deficit (65.8%) and hypopituitarism (39.5 %). Visual field deficit improved in 84% of affected patients, and hyperprolactinemia improved in 80% of affected patients. Pre-operative hypothyroidism and hypogonadism were associated with radiological type 3 cysts, while headache was more common in type B and C. Type 3 cysts were also associated post-operative hypogonadism and hypothyroidism. Permanent Diabetes insipidus was found in 3 patients (7.9%). Cyst height was a significant factor related to pre-operative visual field deficit and post-operative Diabetes insipidus. Residual cysts were found in 11 cases (30.6%) and 9 patients experienced regrowth/recurrence. Residual cysts were a significant factor in regrowth/recurrence. Recurrence rate and post-operative complications were not correlated with different subtypes.
CONCLUSIONS
Endoscopic endonasal approach for removal of Rathke's cleft cyst is a safe and effective intervention. It leads to significant improvement in visual field deficit and recovery of hyperprolactinemia. Although the incidence of post-operative Diabetes insipidus is high, it is usually temporary. Although different categorizations are not correlated to recurrence rate, they could help predict the status of hormone deficit.
PubMed: 37517273
DOI: 10.1016/j.clineuro.2023.107882 -
Annals of Medicine and Surgery (2012) Aug 2023Osmotic demyelination syndrome (ODS) is a neurological disorder usually after rapid correction of hyponatremia. Only few cases of ODS with hypernatremia and diabetes...
UNLABELLED
Osmotic demyelination syndrome (ODS) is a neurological disorder usually after rapid correction of hyponatremia. Only few cases of ODS with hypernatremia and diabetes insipidus (DI) in postpartum state is reported. Postpartum hypernatremia is described as severe hypernatremia in postpartum period and presents as an encephalopathy with rhabdomyolysis with diffuse white matter hyperintensities suggestive of osmotic demyelination.
CASE PRESENTATION
The authors present a case of 29-year-old female who presented with chief complaint of altered sensorium and quadriparesis. Two days prior to onset of symptoms, she underwent caesarean section, was kept on nil per oral and free fluid restriction, after which she had confusion, altered sensorium, and weakness in all four limbs. Sodium level was 170 mEq/l. Urine osmolality and plasma osmolality was 150 and 410 mOsm/kg of water, respectively. MRI showed high signal intensity lesion in pons suggestive of demyelination. She was diagnosed ODS with transient DI and quadriparesis, in postpartum period due to further rise in sodium after free fluid restriction and nil per oral. She was treated with desmopressin, 5% dextrose and 0.9% normal saline, her quadriparesis recovered and desmopressin was tapered and stopped over 45 days and discharged at stable state.
CLINICAL DISCUSSION
ODS can rarely be associated with hypernatremia in postpartum female presenting as quadriparesis and altered sensorium.
CONCLUSION
Clinicians should be familiar of ODS with hypernatremia with transient DI in postpartum period, which is reversible and can be managed by desmopressin and fluid replacement.
PubMed: 37554876
DOI: 10.1097/MS9.0000000000000987 -
Proceedings of the National Academy of... Jul 2023To accomplish concerted physiological reactions, nature has diversified functions of a single hormone at at least two primary levels: 1) Different receptors recognize...
To accomplish concerted physiological reactions, nature has diversified functions of a single hormone at at least two primary levels: 1) Different receptors recognize the same hormone, and 2) different cellular effectors couple to the same hormone-receptor pair [R.P. Xiao, , re15 (2001); L. Hein, J. D. Altman, B.K. Kobilka, , 181-184 (1999); Y. Daaka, L. M. Luttrell, R. J. Lefkowitz, , 88-91 (1997)]. Not only these questions lie in the heart of hormone actions and receptor signaling but also dissecting mechanisms underlying these questions could offer therapeutic routes for refractory diseases, such as kidney injury (KI) or X-linked nephrogenic diabetes insipidus (NDI). Here, we identified that G-biased signaling, but not G activation downstream of EP4, showed beneficial effects for both KI and NDI treatments. Notably, by solving Cryo-electron microscope (cryo-EM) structures of EP3-G, EP4-G, and EP4-G in complex with endogenous prostaglandin E (PGE)or two synthetic agonists and comparing with PGE-EP2-G structures, we found that unique primary sequences of prostaglandin E2 receptor (EP) receptors and distinct conformational states of the EP4 ligand pocket govern the G/G transducer coupling selectivity through different structural propagation paths, especially via TM6 and TM7, to generate selective cytoplasmic structural features. In particular, the orientation of the PGE ω-chain and two distinct pockets encompassing agonist L902688 of EP4 were differentiated by their G/G coupling ability. Further, we identified common and distinct features of cytoplasmic side of EP receptors for G/G coupling and provide a structural basis for selective and biased agonist design of EP4 with therapeutic potential.
Topics: Dinoprostone; Signal Transduction; Receptors, Prostaglandin; GTP-Binding Protein alpha Subunits, Gs; Hormones; Receptors, Prostaglandin E, EP4 Subtype; Receptors, Prostaglandin E, EP2 Subtype; Receptors, Prostaglandin E, EP3 Subtype
PubMed: 37478163
DOI: 10.1073/pnas.2216329120 -
The Journal of Physiology Jul 2024Aquaporin-2 (AQP2) is a member of the aquaporin water channel family. In the kidney, AQP2 is expressed in collecting duct principal cells where it facilitates water... (Review)
Review
Aquaporin-2 (AQP2) is a member of the aquaporin water channel family. In the kidney, AQP2 is expressed in collecting duct principal cells where it facilitates water reabsorption in response to antidiuretic hormone (arginine vasopressin, AVP). AVP induces the redistribution of AQP2 from intracellular vesicles and its incorporation into the plasma membrane. The plasma membrane insertion of AQP2 represents the crucial step in AVP-mediated water reabsorption. Dysregulation of the system preventing the AQP2 plasma membrane insertion causes diabetes insipidus (DI), a disease characterised by an impaired urine concentrating ability and polydipsia. There is no satisfactory treatment of DI available. This review discusses kinases that control the localisation of AQP2 and points out potential kinase-directed targets for the treatment of DI.
Topics: Aquaporin 2; Animals; Humans; Protein Kinases; Diabetes Insipidus; Cell Membrane
PubMed: 37440212
DOI: 10.1113/JP284100 -
Acta Neurologica Belgica Apr 2024Craniopharyngioma (CP), a rare benign intracranial tumor, is still a major clinical challenge. There are two major histologic phenotypes: papillary CP (PCP) and...
Craniopharyngioma (CP), a rare benign intracranial tumor, is still a major clinical challenge. There are two major histologic phenotypes: papillary CP (PCP) and adamantinomatous CP (ACP). This research aimed to assess the occurrence of central diabetes insipidus (antidiuretic hormone deficiency), the level of prolactin, and the stalk effect between PCP and ACP subtypes prior to and after surgery. Clinical data of CP patients before and after surgical resection of the tumor were analyzed retrospectively. These patients were divided into PCP and ACP groups, in accordance with the pathologic classification. The data of prolactin level, 24-h urinary volume, urine specific gravity and electrolyte status before and after surgery were evaluated in these two CP subtypes. A total of 86 CP patients were included, among which 28 patients were PCP and 58 were ACP. Compared to those prior to surgery, 24-h urine volume, serum sodium and serum chlorine concentrations were obviously increased, while prolactin and urine specific gravity were remarkably decreased in all the CP patients after surgery. Compared to those before operation, prolactin level and urine specific gravity were decreased, and 24-h urine volume, serum sodium and serum chlorine were elevated after operation in ACP patients. Moreover, after surgery, 24-h urine volume in PCP patients was higher than that in ACP group. The central diabetes insipidus in patients with CP was aggravated after surgical resection, especially in ACP patients. Moreover, the central diabetes insipidus of PCP subtype was more serious than that of ACP subtype.
PubMed: 38669000
DOI: 10.1007/s13760-024-02558-1 -
Annales D'endocrinologie Aug 2023Endocrine disorders are the most frequent postoperative complications in patients undergoing pituitary surgery. Given the absence of recent guidelines on the... (Review)
Review
PURPOSE
Endocrine disorders are the most frequent postoperative complications in patients undergoing pituitary surgery. Given the absence of recent guidelines on the postoperative care following pituitary surgery, this article summarizes the available evidence on the topic.
METHOD
We conducted a systematic search of PubMed up to 2021 and updated the search in December 2022. We retrieved 119 articles and included 53 full-text papers.
RESULTS
The early postoperative care consists of the assessment for cortisol deficiency and diabetes insipidus (DI). Experts suggest that all patients should receive a glucocorticoid (GC) stress dose followed by a rapid taper. The decision for GC replacement after discharge depends on the morning plasma cortisol level on day 3 after surgery. Experts suggest that patients with a morning plasma cortisol<10 mcg/dL should receive GC replacement at discharge, and those with 10-18 mcg/dL a morning dose only, with formal assessment of the hypothalamic-pituitary-adrenal axis at week 6 postoperatively. When the cortisol level is>18 mcg/dL, the patient can be discharged safely without GC, as suggested by observational studies. Postoperative care also includes a close monitoring of water balance. If DI develops, desmopressin is used only in case of uncomfortable polyuria or hypernatremia. The assessment of other hormones is indicated at 3 months postoperatively and beyond.
CONCLUSION
The evaluation and treatment of patients following pituitary surgery are based on expert opinion and a few observational studies. Further research is needed to provide additional evidence on the most appropriate approach.
Topics: Humans; Diabetes Insipidus; Glucocorticoids; Hydrocortisone; Hypothalamo-Hypophyseal System; Pituitary Diseases; Pituitary Neoplasms; Pituitary-Adrenal System; Clinical Protocols; Postoperative Complications
PubMed: 37019429
DOI: 10.1016/j.ando.2023.03.026 -
Pituitary Aug 2023Large Rathke's cleft cysts (LRCCs) and cystic craniopharyngiomas (CCPs) arise from the same embryological origin and may have similar MR presentations. However, the two...
PURPOSE
Large Rathke's cleft cysts (LRCCs) and cystic craniopharyngiomas (CCPs) arise from the same embryological origin and may have similar MR presentations. However, the two tumors have different management strategies and outcomes. This study was designed to evaluate the clinical and imaging findings of LRCCs and CCPs, aiming to evaluate their pretreatment diagnosis and outcomes.
METHODS
We retrospectively enrolled 20 patients with LRCCs and 25 patients with CCPs. Both tumors had a maximal diameter of more than 20 mm. We evaluated the patients' clinical and MR imaging findings, including symptoms, management strategies, outcomes, anatomic growth patterns and signal changes.
RESULTS
The age of onset for LRCCs versus CCPs was 49.0 ± 16.8 versus 34.2 ± 22.2 years (p = .022); the following outcomes were observed for LRCCs versus CCPs: (1) postoperative diabetes insipidus: 6/20 (30%) versus 17/25 (68%) (p = .006); and (2) posttreatment recurrence: 2/20 (10%) versus 10/25 (40%) (p = .025). The following MR findings were observed for LRCCs versus CCPs: (1) solid component: 7/20 (35%) versus 21/25 (84%) (p = .001); (2) thick cyst wall: 2/20 (10%) versus 12/25 (48%) (p = .009); (3) intracystic septation: 1/20 (5%) versus 8/25 (32%) (p = .030); (4) snowman shape: 18/20 (90%) versus 1/25 (4%) (p < .001); (5) off-midline extension: 0/0 (0%) versus 10/25 (40%) (p = .001); and (6) oblique angle of the sagittal long axis of the tumor: 89.9° versus 107.1° (p = .001).
CONCLUSIONS
LRCCs can be differentiated from CCPs based on their clinical and imaging findings, especially their specific anatomical growth patterns. We suggest using the pretreatment diagnosis to select the appropriate surgical approach and thus improve the clinical outcome.
Topics: Humans; Child; Adolescent; Young Adult; Adult; Middle Aged; Craniopharyngioma; Pituitary Neoplasms; Retrospective Studies; Central Nervous System Cysts; Magnetic Resonance Imaging
PubMed: 37227614
DOI: 10.1007/s11102-023-01326-3 -
Journal of Neurological Surgery Reports Apr 2024This article aims to identify patient- and surgery-related factors that could predict the development of postoperative central diabetes insipidus (DI). This is a...
This article aims to identify patient- and surgery-related factors that could predict the development of postoperative central diabetes insipidus (DI). This is a retrospective case-control study conducted at a single-institution tertiary referral center. Patients undergoing endoscopic endonasal skull base surgery for pituitary adenoma between November 2018 and April 2023 were recruited. The main outcome measures collected include age, gender, comorbidities, tumor type, postoperative DI, intraoperative and postoperative cerebrospinal fluid (CSF) leak, flow of CSF leak, number of layers required for repair, the use of nasal packing, and hospital length of stay. A total of 20 patients developed DI postoperatively. Patients' demographic and comorbidity profile did not correlate with DI development. The encounter of an intraoperative CSF leak was correlated with postop DI (chi-square (1) = 18.35, < 0.001) with a relative risk (RR) of 2.7 (confidence interval [CI] = 1.37-5.28). The use of nasal packs was also correlated with postop DI (chi-square (1) = 10.17, = 0.001) with a RR of 1.8 (CI = 1.15-2.87). Defects requiring a two or more layers for reconstruction also correlated with postop DI compared to single layer repairs (chi-square (1) = 12.15, < 0.001) irrelevant of the materials used. Development of DI postop correlated with an increased hospital length of stay ( (64) = -3.35, = 0.001). The physician should be careful when evaluating patients with pituitary adenomas in the postoperative period, particularly those with intraoperative CSF leak, nasal packing, and those who underwent multilayer reconstruction of the surgical defect.
PubMed: 38835396
DOI: 10.1055/a-2324-1303 -
JCEM Case Reports Jun 2024In this report we present a case of a 14-year-old girl with type 1 diabetes mellitus (T1DM) who experienced glycemic instability and multiple hormonal deficits,...
In this report we present a case of a 14-year-old girl with type 1 diabetes mellitus (T1DM) who experienced glycemic instability and multiple hormonal deficits, including diabetes insipidus, central hypothyroidism, and central adrenal insufficiency. Brain and sellar magnetic resonance imaging revealed a mass in the suprasellar region, which was confirmed to be a pituitary abscess through transsphenoidal biopsy. T1DM is a chronic systemic disease that can lead to suboptimal glycemic control and increased susceptibility to infections. Pituitary abscess is a rare and serious infection that can manifest with nonspecific signs and symptoms, as well as pituitary hormonal deficiencies. Currently, after a 6-year follow-up the pituitary hormone deficiencies have resolved apart from persistent partial diabetes insipidus. Through a review of the current literature, we discuss the clinical characteristics of pituitary abscess, the challenges in diagnosing it, and speculate on the potential clinical and pathophysiological relationship between this uncommon infection and T1DM in our patient.
PubMed: 38832005
DOI: 10.1210/jcemcr/luae057 -
American Journal of Kidney Diseases :... Jul 2024
Topics: Humans; Kidney Transplantation; Tissue Donors; Male; Female; Middle Aged; Diabetes Insipidus; Adult; Treatment Outcome; Transplant Recipients; Retrospective Studies; Kidney Failure, Chronic
PubMed: 38160701
DOI: 10.1053/j.ajkd.2023.10.015