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1.
Mol Cell Endocrinol ; 499: 110613, 2020 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-31605742

RESUMO

Most peptide hormones originate from secretory protein precursors synthesized within the endoplasmic reticulum (ER). In this specialized organelle, the newly-made prohormones must fold to their native state. Completion of prohormone folding usually occurs prior to migration through the secretory pathway, as unfolded/misfolded prohormones are retained by mechanisms collectively known as ER quality control. Not only do most monomeric prohormones need to fold properly, but many also dimerize or oligomerize within the ER. If oligomerization occurs before completion of monomer folding then when a poorly folded peptide prohormone is retained by quality control mechanisms, it may confer ER retention upon its oligomerization partners. Conversely, oligomerization between well-folded and improperly folded partners might help to override ER quality control, resulting in rescue of misfolded forms. Both scenarios appear to be possible in different animal models of endocrine disorders caused by genetic defects of protein folding in the secretory pathway. In this paper, we briefly review three such conditions, including familial neurohypophyseal diabetes insipidus, insulin-deficient diabetes mellitus, and hypothyroidism with defective thyroglobulin.

2.
Endocr Relat Cancer ; 27(1): R1-R20, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31645015

RESUMO

Endocrine organs are metastatic targets for several primary cancers, either through direct extension from nearby tumour cells or dissemination via the venous, arterial and lymphatic routes. Although any endocrine tissue can be affected, most clinically relevant metastases involve the pituitary and adrenal glands with the commonest manifestations being diabetes insipidus and adrenal insufficiency respectively. The most common primary tumours metastasing to the adrenals include melanomas, breast and lung carcinomas, which may lead to adrenal insufficiency in the presence of bilateral adrenal involvement. Breast and lung cancers are the most common primaries metastasing to the pituitary, leading to pituitary dysfunction in approximately 30% of cases. The thyroid gland can be affected by renal, colorectal, lung and breast carcinomas, and melanomas, but has rarely been associated with thyroid dysfunction. Pancreatic metastasis can lead to exo-/endocrine insufficiency with renal carcinoma being the most common primary. Most parathyroid metastases originate from breast and lung carcinomas and melanoma. Breast and colorectal cancers are the most frequent ovarian metastases; prostate cancer commonly affects the testes. In the presence of endocrine deficiencies, glucocorticoid replacement for adrenal and pituitary involvement can be life saving. As most metastases to endocrine organs develop in the context of disseminated disease, surgical resection or other local therapies should only be considered to ameliorate symptoms and reduce tumour volume. Although few consensus statements can be made regarding the management of metastases to endocrine tissues because of the heterogeneity of the variable therapies, it is important that clinicians are aware of their presence in diagnosis.

4.
Neuro Endocrinol Lett ; 40(2): 99-104, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31785217

RESUMO

OBJECTIVE: Pituitary abscess is a rare condition, with approximately 300 cases reported in the literature. Primary pituitary abscess, the most common type, occurs in previously healthy normal glands. Secondary pituitary abscess is secondary to pre-existing lesions in the pituitary region, such as pituitary adenoma, Rathke's cleft cysts, or craniopharyngioma and infections. MATERIAL AND METHODS: A total of 2281 patients underwent pituitary surgery via endoscopic transsphenoidal approach in Kocaeli University Pituitary Gland Research Center between 1997 and 2018. Among this cohort 9 patients (4 female and 5 male) were diagnosed with primary pituitary abscess based on both intraoperative findings and postoperative histopathological evidence. RESULTS: Primary pituitary abscess incidence was obtained 0.39% in our center. Mean age of the patients was 50 years old. There was no history of pituitary surgery, radiotherapy and infection diseases in our patients. Visual symptoms were prominent in two patients, hypopituitarism was found in 5 out of the 9 patients. All patients have typical pituitary lesion on pituitary magnetic resonance imaging. Staphylococcus species were the most commonly isolated organisms in the culture. A few weeks of antibiotic therapy were administered after surgery. CONCLUSION: Presentation of fever, headache, diabetes insipidus, hypopituitarism and a sellar cystic mass with an enhanced rim after gadolinium contrast on pituitary magnetic resonance imaging may be suggestive of a pituitary abscess. Transsphenoidal endoscopic surgery, proper antibiotics and appropriate hormone replacement therapy when necessary are the keys of pituitary abscess treatment.

5.
Mol Cell Endocrinol ; : 110653, 2019 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-31785344

RESUMO

This review focuses on the cellular and molecular aspects underlying familial neurohypophyseal diabetes insipidus (DI), a rare disorder that is usually transmitted in an autosomal-dominant fashion. The disease, manifesting in infancy or early childhood and gradually progressing in severity, is caused by fully penetrant heterozygous mutations in the gene encoding prepro-vasopressin-neurophysin II, the precursor of the antidiuretic hormone arginine vasopressin (AVP). Post mortem studies in affected adults have shown cell degeneration in vasopressinergic hypothalamic nuclei. Studies in cells expressing pathogenic mutants and knock-in rodent models have shown that the mutant precursors are folding incompetent and fail to exit the endoplasmic reticulum (ER), as occurs normally with proteins that have entered the regulated secretory pathway. A portion of these mutants is eliminated via ER-associated degradation (ERAD) by proteasomes after retrotranslocation to the cytosol. Another portion forms large disulfide-linked fibrillar aggregates within the ER, in which wild-type precursor is trapped. Aggregation capacity is independently conferred by two domains of the prohormone, namely the AVP moiety and the C-terminal glycopeptide (copeptin). The same domains are also required for packaging into dense-core secretory granules and regulated secretion, suggesting a disturbed balance between the physiological self-aggregation at the trans-Golgi network and avoiding premature aggregate formation at the ER in the disease. The critical role of ERAD in maintaining physiological water balance has been underscored by experiments in mice expressing wild-type AVP but lacking critical components of the ERAD machinery. These animals also develop DI and show amyloid-like aggregates in the ER lumen. Thus, the capacity of the ERAD is exceeded in autosomal dominant DI, which can be viewed as a neurodegenerative disorder associated with the formation of amyloid ER aggregates. While DI symptoms develop prior to detectable cell death in transgenic DI mice, the eventual loss of vasopressinergic neurons is accompanied by autophagy, but the mechanism leading to cell degeneration in autosomal dominant neurohypophyseal DI still remains unknown.

7.
Oncologist ; 2019 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-31784491

RESUMO

BACKGROUND: Patients with pituitary metastasis (PM) have a relatively poor prognosis. We describe the presentation, management, and outcomes of patients with PM. SUBJECTS, MATERIALS, AND METHODS: We performed a retrospective review of patients diagnosed with PM at a single institution from 1996 to 2015. Eighty-five patients diagnosed with metastasis to the pituitary or sella turcica by pathology or based on a combination of neuroimaging and clinical findings were included. Univariate and multivariable Cox regressions evaluated associations between clinical factors and overall survival. RESULTS: The most frequent sites of primary malignancies resulting in PM were lung (26%) and breast (26%). Median age at diagnosis was 60 years (range, 18-95). The most common complaints at diagnosis included visual deficits (62%), headache (47%), and cranial nerve palsy (31%). Seventy percent of patients had pituitary insufficiency-adrenal insufficiency (59%), hypothyroidism (59%), or diabetes insipidus (28%). Management of PM included radiation therapy (76%), chemotherapy (68%), surgical resection (21%), or combination therapy (71%). Fifty percent and 52% of patients who received surgical treatment and irradiation, respectively, reported symptomatic improvement. Median overall survival (OS) was 16.5 months (95% confidence interval: 10.7-25.4). On multivariable analysis, a primary cancer site other than lung or breast (p = .020), age <60 years (p = .030), and surgical resection (p = .016) were associated with longer OS. CONCLUSION: Patients <60 years of age, those with primary tumor sites other than lung or breast, and those who undergo surgical resection of the pituitary lesion may have prolonged survival. Surgical resection and radiation treatment resulted in symptomatic improvement in ~50% of patients. IMPLICATIONS FOR PRACTICE: This study is the largest original series of patients with metastatic disease to the sella. In patients with pituitary metastasis, younger age, primary site other than lung or breast, and metastatic resection may prolong survival. Resection and radiation led to symptomatic improvement in ∼50% of patients. Seventy percent of patients had hypopituitarism. These hormonal deficiencies can be life threatening and can result in substantial morbidity if left untreated. Patients should be treated using a multimodality approach-including a potential role for surgery, radiation, chemotherapy, and hormone replacement-with the goal of improving survival and quality of life.

8.
Eur J Pharmacol ; : 172803, 2019 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-31738937

RESUMO

Nephrogenic Diabetes Insipidus is a rare disorder which is characterized by severe water imbalance in the body. The disease can be acquired or inherited. AVPR2 (arginine vasopressin type 2 receptor) mutations are responsible for genetical type of the disorder. Mutations in the AVPR2 gene may cause loss-of-function due to conformational defects. According to the mutation type, the three-dimensional structure of AVPR2 may be affected even if it is functional and therefore it may not reach the plasma membrane where it is functional. Consequently, it is generally trapped in the Endoplasmic reticulum or Golgi apparatus, which are the quality control systems of the cell. Pharmacological chaperones have been used to retrieve these mutant AVPR2s from these quality control systems of the cell and take them to the plasma membrane for therapeutic purposes. In this study, in order to analyze the effects of two pharmacological chaperones, SR121463B and SR49059, we performed total ELISA and surface ELISA studies and cAMP accumulation assays on mutant receptors (G12E, R68W, V88M, ΔR67_G69/G107W, R106C, V162A and T273M). We observed that pharmacological chaperones may act differently on mutated AVPR2s. Cell surface expression of the mutant receptors and cAMP accumulation response, after stimulation with AVP, were mostly improved by these pharmacological chaperones. We believe that, this study presents important results with respect to the process of the variable type of mutated proteins in the cell and may help in developing a process of new types of chaperones.

9.
Diagn Interv Radiol ; 2019 Oct 31.
Artigo em Inglês | MEDLINE | ID: mdl-31670263

RESUMO

PURPOSE: We aimed to evaluate the frequency and neuroimaging features of Rathke's cleft cysts (RCCs) in children examined for endocrine-related diseases and to determine changes in the neuroimaging features of RCCs during the follow-up of children. We hypothesize that RCCs are being more commonly diagnosed in children with endocrine-related diseases and most of the RCCs show neither fluid intensity nor intensity due to high protein content on magnetic resonance imaging (MRI). METHODS: After approval by the local ethics committee, the medical records and contrast-enhanced pituitary MRI of 833 children (boys/girls, 338/495; mean age±SD, 9.4±3.7 years) were retrospectively reviewed between January 2016 and January 2019. The size, location, signal intensities, and postcontrast enhancement pattern of RCCs were assessed by a pediatric radiologist. Same imaging features were also independently reviewed by another radiologist to determine the interobserver agreement by using the kappa statistics (κ) and intraclass correlation coefficient (ICC). RESULTS: RCC was evident on MRI in 13.5% of the patients (boys/girls, 39/74; mean age±SD, 9.8±3.9 years). The mean size of RCCs was 5.5 mm (range, 3.1-8.5 mm). An RCC frequency higher than expected was found in patients with central precocious puberty, diabetes insipidus, and hypersecretion of prolactin (P = 0.007). The mean size of RCCs did not show significant differences among the clinical indications for MRI (P ≥ 0.461). All RCCs showed abnormal signal on T2-weighted image and most (89%) showed neither fluid intensity nor intensity due to high protein content (i.e., isointense on T1-weighted imaging and hypointense on T2-weighted imaging compared with the normal anterior pituitary gland). Eighty-four patients with RCCs (74%) had follow-up MRI and the mean follow-up was 1.5 years. In follow-ups, five RCCs disappeared; the mean size of 10 RCCs increased and that of 6 RCCs decreased. These size changes were not statistically significant (P = 0.376). No signal intensity changes of RCCs were seen during the follow-up, except for 4 RCCs, whose protein content increased over time and T1 signals increased on imaging. Interobserver agreements were almost perfect for the MRI findings of RCCs (κ and ICC range, 0.81-1, P < 0.001). CONCLUSION: RCCs were not uncommon in patients examined for endocrine-related diseases, and nearly 1 in 10 patients had an RCC. The size and signal intensities of RCCs may change over time and the evolution of RCCs is unpredictable. Most RCCs showed neither fluid intensity nor intensity due to high protein content on MRI, and all RCCs had an abnormal signal on T2-weighted imaging, thus eliminating the need to administer a contrast agent at follow-up imaging of the patients.

10.
Prog Urol ; 2019 Nov 21.
Artigo em Francês | MEDLINE | ID: mdl-31761518

RESUMO

Wolfram syndrome is a neurodegenerative disorder characterized by childhood onset diabetes mellitus, optic nerve atrophy, diabetes insipidus, hearing impairment, brainstem alteration and commonly bladder and bowel dysfunction. OBJECTIVE: We present here, 6 new cases of urinary dysfunction in this rare disease. RESULTS: All patients had urinary retention with overactive bladder. The urodynamic assessment found overactive detrusor in 3 cases. Five out of six patients performed self-catheterization and were treated with anticholinergics or intradetrusor injection of botulinum toxin. The follow-up at 5 years found an alteration of the upper urinary tracts and a renal failure 3/6. CONCLUSION: Urinary dysfunction is common in Wolfram syndrome, mainly characterized by overactive bladder and urinary retention. The urological risk is major requiring a systelmatic follow-up of these patients. LEVEL OF EVIDENCE: 4.

12.
J Neurooncol ; 145(3): 403-413, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31677031

RESUMO

INTRODUCTION: Cushing's disease (CD) is rare disorder that should be adequately managed to optimize long-term prognosis. Treatment of CD is multidisciplinary and often includes surgical resection, adjuvant stereotactic radiosurgery (SRS), and medical treatment. Here we review surgical and radiosurgical treatment strategies for ACTH producing pituitary adenomas. METHODS: A comprehensive literature review was carried out to review remission and recurrence rates, and complications of surgical and SRS treatments of ACTH producing pituitary adenomas. RESULTS: Surgical resection plays a central role in the management of ACTH secreting pituitary adenomas and usually allows rapid endocrine remission that ranges from 69 to 90%. The most common complications after resection include some degree of new hypopituitarism, diabetes insipidus and CSF leak. Devastating complications, such as injury of vascular and neural structures, are very rare. Surgeon experience and adequate pre-operative imaging are important for safe and successful surgery. Endocrine recurrence rates after resection range from 9 to 30%. SRS is often employed for incompletely resected adenomas. Endocrine remission after SRS ranges from 35 to 72%. The most common complication of SRS is new anterior pituitary gland deficiency. Recurrence rates after GKRS range from 18 to 24%. CONCLUSIONS: Transsphenoidal resection of ACTH producing pituitary adenoma is a safe and highly effective procedure for CD in experienced hands. Radiosurgery is more frequently used as treatment of residual and recurrent adenoma and persistent CD. Long-term endocrine and imaging follow-up is important as delayed recurrences and hypopituitarism are not infrequent.

14.
J Neurosurg ; : 1-7, 2019 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-31731279

RESUMO

OBJECTIVE: Recovery from preexisting hypopituitarism after transsphenoidal surgery for pituitary adenoma is an important outcome to investigate. Furthermore, pituitary function has not been thoroughly evaluated after fully endoscopic surgery, and benchmark outcomes have not been clearly established. Here, the authors characterize pituitary gland outcomes with a focus on gland recovery following endoscopic transsphenoidal removal of clinically nonfunctioning adenomas. METHODS: This multicenter prospective study was conducted at 6 US pituitary centers among adult patients with nonfunctioning pituitary macroadenomas who had undergone endoscopic endonasal pituitary surgery. Pituitary gland function was evaluated 6 months after surgery. RESULTS: The 177 enrolled patients underwent fully endoscopic transsphenoidal surgery; 169 (95.5%) of them were available for follow-up. Ninety-five (56.2%) of the 169 patients had had a preoperative deficiency in at least one hormone axis, and 20/95 (21.1%) experienced recovery in at least one axis at the 6-month follow-up. Patients with adrenal insufficiency were more likely to recover (10/34 [29.4%]) than were those with hypothyroidism (8/72 [11.1%]) or male hypogonadism (5/50 [10.0%]). At the 6-month follow-up, 14/145 (9.7%) patients had developed at least one new deficiency. The study did not identify any predictors of gland recovery (p ≥ 0.20). Permanent diabetes insipidus was observed in 4/166 (2.4%) patients. Predictors of new gland dysfunction included a larger tumor size (p = 0.009) and Knosp grade 3 and 4 (p = 0.051). CONCLUSIONS: Fully endoscopic pituitary surgery resulted in improvement of pituitary gland function in a substantial minority of patients. The deficiency from which patients were most likely to recover was adrenal insufficiency. Overall rates of postoperative permanent diabetes insipidus were low. This study provides multicenter benchmark neuroendocrine clinical outcome data for the endoscopic technique.

15.
Pan Afr Med J ; 33: 293, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31692902

RESUMO

Erdheim-Chester disease (ECD) is a very rare and aggressive form of non-Langerhans histiocytosis with unclear pathogenesis. Because of the heterogeneity of clinical presentation, diagnosis is often challenging and delayed. Currently, Interferon alpha is the first line treatment that is associated with a better survival. The prognosis is relatively poor, especially in case of neurological and cardiovascular involvement. Herein, we report the case of a 64-year-old Tunisian female patient presenting an aggressive form of ECD revealed by diabetes insipidus and cerebellar ataxia with a diagnosis delay of 4 years. The assessment of disease extent had also shown associated asymptomatic cardiac and bone involvement. Pegylated Interferon alpha was started at high dose allowing disease stabilization. This case illustrates that physicians should be aware of the heterogeneous manifestations of ECD in order to insure an early diagnosis and treatment. Long-term and regular follow-up is crucial because of the risk of disease progression.


Assuntos
Ataxia Cerebelar/etiologia , Diabetes Insípido/etiologia , Doença de Erdheim-Chester/diagnóstico , Diagnóstico Tardio , Doença de Erdheim-Chester/complicações , Doença de Erdheim-Chester/tratamento farmacológico , Feminino , Humanos , Interferon-alfa/administração & dosagem , Pessoa de Meia-Idade , Polietilenoglicóis/química , Prognóstico , Tunísia
16.
Pituitary ; 2019 Nov 14.
Artigo em Inglês | MEDLINE | ID: mdl-31728907

RESUMO

PURPOSE: Several institutions recently published their experiences with unplanned readmissions rates after transsphenoidal surgery for pituitary lesions. Readmission rates on a national level, however, have not been explored in depth. We investigated nationwide trends in this procedure and associated independent predictors, costs, and causes of 30-day readmission. METHODS: The Nationwide Readmissions Database was queried to identify patients 18 and older who underwent transsphenoidal surgery for pituitary lesion resection (2010-2015). National trends and statistical variances were calculated based on weighted, clustered, and stratified sample means. RESULTS: Of the weighted total of 44,759 patients treated over the 6-year period, 4658 (10.4%) were readmitted within 30 days. Readmission rates did not change across the survey period (P = 0.71). Patients readmitted had a higher prevalence of comorbidities than those not readmitted (82.5% vs. 78.4%, respectively, P < 0.001), experienced more postoperative complications (47.2% vs. 31.8%, P < 0.001), and had a longer length of stay (6.59 vs. 4.23 days, P < 0.001) during index admission. The most common causes for readmission were SIADH (17.5%) and other hyponatremia (16.4%). Average total readmission cost was $12,080 with no significant trend across the study period (P = 0.25). Predictors for readmission identified included diabetes mellitus, psychological disorders, renal failure, and experiencing diabetes insipidus during the index admission. CONCLUSION: Unplanned readmission is an important quality metric. While transsphenoidal pituitary surgery is a relatively safe procedure, 30-day readmission rates and costs have not declined. Future studies on institutional protocols targeting these identified predictors to prevent readmission are necessary to decrease readmission rates on a national scale.

17.
J Affect Disord ; 262: 149-154, 2019 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-31733459

RESUMO

OBJECTIVES: Statins have recently been linked to having effects on cognition and mood in mood disorders, though results are mixed. In this paper, we use data from a recent randomized controlled trial (RCT) to examine the effect of statins on cognition and mood in patients with Bipolar Disorder (BD) and Major Depressive Disorder (MDD). METHODS: This is a secondary analysis of a randomized, double-blind, placebo-controlled clinical trial (n = 60) originally designed to examine the effect of atorvastatin (n = 27) versus placebo (n = 33) for lithium-induced diabetes insipidus in BD and MDD patients who were using lithium. For this analysis, the primary outcome was global cognition Z-score at 12-weeks adjusted for baseline. The secondary cognition outcomes were (1) Screen for Cognitive Impairment in Psychiatry (SCIP), and (2) executive function Z-score. The primary mood outcome (secondary outcome of this analysis) was depression relapse during 12-week follow-up (Mongomery Asberg Depression Rating Scale (MADRS) ≥10). The secondary mood outcomes were (1) relapse rate into a manic episode, and (2) relapse rate into any mood episode. RESULTS: After 12 weeks follow-up, atorvastatin and placebo groups did not differ in terms of global cognition Z-score (ß = -0.009287 (-0.1698,0.1512), p-value = 0.91). Similarly, composite Z-scores for SCIP and executive functions did not differ significantly. Depression relapse during 12-week follow-up was not significantly different between the groups (χ2 (1) = 0.148, p-value = 0.70). Similarly, there was no difference between groups regarding relapse into mania. CONCLUSION: In BD and MDD patients with lithium-induced nephrogenic diabetes insipidus randomized to atorvastatin or placebo, we found no significant differences in cognition and mood outcomes at 12-week follow-up.

18.
Endocr J ; 2019 Nov 19.
Artigo em Inglês | MEDLINE | ID: mdl-31748430

RESUMO

Central diabetes insipidus (CDI) is characterized by polyuria and polydipsia caused by impairment of arginine vasopressin (AVP) secretion. In this study, we evaluated plasma AVP concentrations during a hypertonic saline infusion test using a new AVP radioimmunoassay (RIA) which is now available in Japan. Thirteen control subjects, mostly with hypothalamo-pituitary disease but without CDI, and 13 patients with CDI were enrolled in the study. Whether or not subjects had CDI was determined based on the totality of clinical data, which included urine volumes and osmolality. Regression analysis of plasma AVP and serum Na concentrations revealed that the gradient was significantly lower in the CDI group than in the control group. The area under the receiver-operating-characteristic (ROC) curve was 0.99, and the <0.1 gradient cut-off values for the simple regression line to distinguish CDI from control had a 100% sensitivity and a 77% specificity. The ROC analysis with estimated plasma AVP concentrations at a serum Na concentration of 149 mEq/L showed that the area under the ROC curve was 1.0 and the <1.0 pg/mL cut-off values of plasma AVP had a 99% sensitivity and a 95% specificity. We conclude that measurement of AVP by RIA during a hypertonic saline infusion test can differentiate patients with CDI from those without CDI with a high degree of accuracy. Further investigation is required to confirm whether the cut-off values shown in this study are also applicable to a diagnosis of partial CDI or a differential diagnosis between CDI and primary polydipsia.

19.
J Neurosurg ; : 1-11, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31675725

RESUMO

OBJECTIVE: Rathke's cleft cyst (RCC) is a benign cystic lesion with a relatively high incidence of local recurrence that occasionally requires repeat surgery. To prevent recurrence, simple cyst fenestration and drainage of the cyst contents to the sphenoid sinus is recommended, but it occasionally recurs. The authors postulated that obstruction of fenestration is a main cause of recurrence, and they developed a method, named the "mucosa coupling method (MC method)," that maintains persistent drainage. In this method, the RCC epithelium and the mucosa of the sphenoid sinus are connected, which promotes re-epithelialization between the two epithelia, maintaining persistent drainage. The outcome of this method was compared with that of conventional cyst fenestration. METHODS: In a consecutive series of 40 patients with RCC, the surgical strategy was changed during the study period: from December 2009 to September 2014 (the conventional period), 24 patients were scheduled to be treated using the conventional fenestration method, whereas from September 2014 to September 2017 (the MC period), 16 patients were scheduled to be treated using the MC method. However, because of an intraoperative CSF leak, the fenestration was closed during surgery in 3 patients in the conventional period and 2 in the MC period; therefore, these 5 patients were excluded from the analysis. Twenty-one patients treated with the conventional fenestration method (conventional group) and 14 patients treated with the MC method (MC group) were analyzed. All patients regularly underwent MRI after surgery to detect reaccumulation of cyst contents. The rate of reaccumulation with and without reoperation, visual outcomes, endocrinological outcomes, and postoperative complications were compared between these two groups. RESULTS: The median follow-up period in all 35 patients was 48.0 months (range 1-96 months), 54.0 months (range 1-96 months) in the conventional group and 35.5 months (range 12-51 months) in the MC group. No reaccumulation was detected on MRI in the 14 patients in the MC group, whereas it was noted in 9 (42.9%) of 21 patients in the conventional group, and 2 of these 9 patients required repeat surgery. There were no significant differences in visual and endocrinological outcomes and complications between these two groups. CONCLUSIONS: The MC method for RCC is effective for preventing obstruction of cyst fenestration, which contributes to preventing cyst reaccumulation. Furthermore, this method is equivalent to the conventional fenestration method in terms of visual and endocrinological outcomes and the complication rate.

20.
Endokrynol Pol ; 70(5): 430-437, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31681969

RESUMO

INTRODUCTION: In the clinical setting, the diagnosis of neurosarcoidosis in patients with central diabetes insipidus (CDI) is typically based both on symptoms (i.e. polydipsia or polyuria) and brain magnetic resonance imaging (MRI) findings (e.g. pituitary abnormality). However, inconsistent changes in the patient's symptoms and brain MRI findings may occur during the clinical course of the disease. This review was performed to summarise the relationship between symptoms and brain MRI findings in previously reported cases of neurosarcoidosis with CDI. MATERIAL AND METHODS: Case studies of patients diagnosed with neurosarcoidosis with CDI were collected via a PubMed search of studies published through 30 June 2018. RESULTS: Thirteen eligible studies were reviewed (20 patients; 12 men, 8 women; mean age 33 years). Polydipsia or polyuria was the first symptom in 13 patients. The mean duration from disease onset to diagnosis was 3.4 months. Brain MRIs showed abnormal findings in the hypothalamus and pituitary for 17 patients. Immunosuppressive drugs were used in 17 patients. For 14 patients, MRI findings improved, while symptoms did not. CONCLUSION: Patients with both neurosarcoidosis and CDI symptoms often do not improve, despite the fact that brain MRI findings often improve following treatment. More studies involving detailed pathological analyses and longer follow-up periods are necessary.

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