cushing's disease
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2022 ◽  
pp. 152-161
Author(s):  
N. V. Kuritsyna ◽  
U. A. Tsoy ◽  
V. Yu. Cherebillo ◽  
A. A. Paltsev ◽  
A. I. Tsiberkin ◽  
...  

Introduction. Transsphenoidal adenomectomy (TSA) is the method of choice in the treatment of Cushing’s disease (CD), but remission of hypercorticism cannot be achieved in all patients. The search for predictors of CD remission after TSA remains to be an important challenge in the endocrinology today.Aim. To study the preoperative and postoperative data of patients with CD to identify the predictors of hypercorticism remission after TSA.Materials and methods. 101 patients with confirmed CD after TSA were included. One year after operation all patients were examined for the presence of hypercorticism remission and divided into two groups: with CD remission and its absence. In both groups’ preoperative pituitary magnetic resonance imaging (MRI) data, the results of preoperative high dose dexamethasone suppression test (HDDST) and the results of serum cortisol collected in the morning 2–3 days after surgery were compared.Results. One year after TSA, CD remission was confirmed in 63 patients (62.4%), whereas in 38 patients (37.6%) hypercortisolism persisted. Favorable predictors of CD remission were: the adenoma size > 3 mm without the invasive growth according to pituitary MRI (specificity 82.4%, sensitivity 82.8%), serum cortisol suppression ≥ 74% in preoperative HDDST (specificity 81.5%, sensitivity 86.3%), morning serum cortisol level in 2–3 days after surgery ≤ 388 nmol/l (specificity 79.3%, sensitivity 97.4%).Conclusions. Pituitary MRI data, the results of preoperative HDDST and morning serum cortisol in the 2–3 days after surgery can be used as predictors of CD remission.


Author(s):  
Ana J. PEREIRA ◽  
Natalia X. ANDRADE ◽  
Nina R. MUSOLINO ◽  
Valter A. CESCATO ◽  
Gilberto O. SILVA ◽  
...  

2021 ◽  
Vol 36 (1) ◽  
Author(s):  
Ignatius N. Esene ◽  
Tarek Elserry ◽  
Hesham Radwan ◽  
Ahmad Mohammed Elsabaa

Abstract Background The first line of treatment of Cushing’s disease (CD) is surgery. However, surgical resection is not amenable in all cases and the role of radiosurgical management of residual tumors or upfront treatment has been reported. Our study highlights the effectiveness and safety of Gamma Knife Radiosurgery for the treatment of Cushing’s disease. Methods This was an ambidirectional descriptive cohort study on 16 consecutive patients with a confirmed Cushing’s disease that underwent Gamma Knife Radiosurgery (GKR) before July 2014 and assessed for outcome during the study period between January 2014 and June 2016 (30 Months). We included patients with a minimum of two years follow up. The main outcomes were biochemical remission and tumor volume control. Secondary outcomes were visual field changes and morbidity. Results Sixteen cases with CD were included into the study. The Mean age ± SD was 34.81 ± 10.10 years. The male to female sex ratio was 1:3. Six cases (37.5%) were de novo. Normalization of hypersecretion at 2 years was achieved in 13 cases (81.3%).The median hormone normalization time was 23 months. Tumor volume control was achieved in all the cases, whereas tumor shrinkage was achieved in (10 cases) 62.5%. The median shrinkage time was 13 months. Of the 12 eyes with pre-Gamma Knife visual affection, 8 (75%) normalized, 4 (25%) improved, and none deteriorated. No patient developed new hypopituitarism after GK radiosurgery. One case developed diplopia at 24 months follow up from abducens palsy. No mortality occurred in our series. Conclusion Gamma Knife Stereotactic Radiosurgery is an effective and safe treatment option for Cushing’s disease. It can be used as a complementary therapeutic procedure to classic surgery or as a first line treatment in selected number of patients.


Nutrients ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 4329
Author(s):  
Alexandra Povaliaeva ◽  
Viktor Bogdanov ◽  
Ekaterina Pigarova ◽  
Artem Zhukov ◽  
Larisa Dzeranova ◽  
...  

In this study we aimed to assess vitamin D metabolism in patients with Cushing’s disease (CD) compared to healthy individuals in the setting of bolus cholecalciferol treatment. The study group included 30 adults with active CD and the control group included 30 apparently healthy adults with similar age, sex and BMI. All participants received a single dose (150,000 IU) of cholecalciferol aqueous solution orally. Laboratory assessments including serum vitamin D metabolites (25(OH)D3, 25(OH)D2, 1,25(OH)2D3, 3-epi-25(OH)D3 and 24,25(OH)2D3), free 25(OH)D, vitamin D-binding protein (DBP) and parathyroid hormone (PTH) as well as serum and urine biochemical parameters were performed before the intake and on Days 1, 3 and 7 after the administration. All data were analyzed with non-parametric statistics. Patients with CD had similar to healthy controls 25(OH)D3 levels (p > 0.05) and higher 25(OH)D3/24,25(OH)2D3 ratios (p < 0.05) throughout the study. They also had lower baseline free 25(OH)D levels (p < 0.05) despite similar DBP levels (p > 0.05) and lower albumin levels (p < 0.05); 24-h urinary free cortisol showed significant correlation with baseline 25(OH)D3/24,25(OH)2D3 ratio (r = 0.36, p < 0.05). The increase in 25(OH)D3 after cholecalciferol intake was similar in obese and non-obese states and lacked correlation with BMI (p > 0.05) among patients with CD, as opposed to the control group. Overall, patients with CD have a consistently lower 25(OH)D3/24,25(OH)2D3 ratio, which is indicative of a decrease in 24-hydroxylase activity. This altered activity of the principal vitamin D catabolism might influence the effectiveness of cholecalciferol treatment. The observed difference in baseline free 25(OH)D levels is not entirely clear and requires further study.


Author(s):  
Adriana G Ioachimescu ◽  
Neevedita Goswami ◽  
Talin Handa ◽  
Adlai Pappy ◽  
Emir Veledar ◽  
...  

Abstract Purpose The impact of race on presentation and postoperative outcomes in adults with acromegaly (ACM) and Cushing’s disease (CD) has not been evaluated. Methods This is a retrospective study of consecutive patients operated at a large-volume pituitary center. We evaluated: 1) racial distribution of patients residing in the metropolitan area (Metro, N=124) versus 2010 U.S. Census data, 2) presentation and postoperative outcomes in Black versus White for patients from the entire catchment area (N=241). Results For Metro area (32.4% Black population), Black patients represented 16.75% ACM (p=0.006) and 29.2% CD (p=0.56).Among 112 total ACM patients, presentations with headaches or incidentaloma were more common in Black patients (76.9% versus 31% White, p=0.01). Black patients had a higher prevalence of diabetes (54% versus 16% White, p=0.005), significantly lower IGF-1 deviation from normal (p=0.03) and borderline lower median GH levels (p=0.09). Mean tumor diameter and proportion of tumors with cavernous sinus invasion were similar. Three-month biochemical remission (46% Black, 55% White, p=0.76) and long-term IGF-1 control by multimodality therapy (92.3% Black, 80.5% White, p=0.45) were similar. Among 129 total CD patients, Black patients had more hypopituitarism (69% vs 45% White, p=0.04) and macroadenomas (33% vs 15% White, p=0.05). At 3 months, remission rate was borderline higher in White (92% vs 78% Black, p=0.08) which was attributed to macroadenomas by logistic regression. Conclusion We identified disparities regarding racial distribution, clinical and biochemical characteristics in ACM, suggesting late or missed diagnosis in Black patients. Large nationwide studies are necessary to confirm our findings.


2021 ◽  
Author(s):  
M.K. de Filette Jeroen ◽  
Sol Bastiaan ◽  
Awada Gil ◽  
E. Andreescu Corina ◽  
Unuane David ◽  
...  

2021 ◽  
Vol 12 ◽  
Author(s):  
Xueqing Zheng ◽  
He Wang ◽  
Wentai Zhang ◽  
Shanshan Feng ◽  
Yifan Liu ◽  
...  

PurposeCushing’s disease (CD) is a rare disease that contributes to 70–80% hypercortisolemia, which presents similarities and differences between pediatric and adult patients, and even between male and female patients. However, the comparative study of CD between different age groups and different genders is still insufficient. The aim of the study is to make a systematic comparison to reveal the gender differences in children and adult patients of CD, helping clinicians to provide optimal treatment for different groups of patients.MethodsWe conducted a retrospective research consisting of 30 pediatric and 392 adult CD patients in a single center in Peking Union Medical College Hospital. All 422 patients showed symptoms related to hypercortisolism and received adenoma excision surgery in the department of neurosurgery between 2014 and 2020.ResultsFor the accuracy of diagnosis, the sensitivity of BIPSS at baseline in pediatric patients was lower than in adults (75 vs. 91%, P = 0.054) but increased greatly after desmopressin stimulation (94 vs. 95%). However, the accuracy of lateralization for BIPSS was not preferred for prediction. As for clinical manifestations, growth retardation, weight gain, hirsutism, and acne were more prevalent for children, while for adults, hypertension, osteopenia, glucometabolic disorder, easy bruising, hair loss, and weight loss were more frequently seen. As previously reported, we observed a significant difference between the male prevalence of pediatric and adult patients (50 vs. 17%, P &lt; 0.001), which was possibly caused by the more severe and earlier onset of a series of symptoms. Gender-related comparison showed greater morbidity of nephrolithiasis, hypokalemia, hypertension, easy bruising, osteopenia, and striae for male patients, while irregular menses, hirsutism, and hair loss were more common for female patients. Further analysis showed that the secretory activity of the PA axis was higher for males, presenting as the more remarkable alteration of laboratory parameters and contributing to the more severe clinical manifestations. For patients treated with transsphenoidal pituitary surgery (TSS), the immediate prognosis could be predicted by operation history, invasiveness, Ki-67, and information provided by MRI, including tumor size and Knosp grading. However, we still lack methods to predict long-term prognosis.ConclusionsOur study is the first detailed and systematic comparison between pediatric and adult CD patients. Further exploration of the impact of CD on different genders reveals a more severe and probably an earlier-onset pattern of CD for male patients.


2021 ◽  
Vol 49 ◽  
Author(s):  
D. S. Mikhaylova ◽  
L. K. Dzeranova ◽  
O. Yu. Rebrova ◽  
E. A. Pigarova ◽  
L. Ya. Rozhinskaya ◽  
...  

Background: At present, surgical intervention is a  major treatment strategy for pituitary adenomas. It is commonly complicated with water and electrolyte imbalance. The prevalence of post-surgical central diabetes insipidus (CDI) may amount to 30%; however, its risk factors have not been established.Aim: To assess the rates and nosological distribution of CDI after transnasal adenomectomy and to identify its risk factors.Materials and methods: This retrospective study included 96 patients aged 20 to 65 years (median [Q1; Q3] 43  [34.5; 53.5]), who had transnasal adenomectomy in 2010–2011  due to Cushing's disease, acromegaly, prolactinoma, non-functioning pituitary adenoma. All patients underwent clinical examination and laboratory work-up before and after surgery. Their post-surgical course was assessed for 5  to 7  years. Depending on identified manifestations, the patients were categorized into the permanent CDI (n=15) and transient CDI (n=34); 47 patients had no CDI.Results: Cushing's disease (odds ratio (OR) 6.1, 95%  confidence interval (CI) 2.3–16.1), secondary adrenal insufficiency (OR 6.8, 95%  CI 2.6– 18.3) and adrenocorticotropic hormone levels of˂15.8 pg/mL (OR 5.0, 95% CI 1.9–13.5), microadenoma (OR 4.5, 95%  CI 1.7–11.5) promote transient postoperative CDI, whereas macroadenoma decrease this risk (OR 0.2, 95%  CI 0.1–0.5). The transient CDI was also more common in patients with secondary hypothyroidism, cortisol level of˂200  nmol/L, adenoma's volume at magnetic resonance imaging of<0.83 cm3  at trend level and more rare in patients with acromegaly and loss of tropic hormones before surgery at trend level as well. No significant risk factors were identified for permanent CDI, but it could be more often associated with secondary hypothyroidism, absence of any adenoma at magnetic resonance imaging and pituitary injury during the surgery and less frequent in patients with macroadenomas at trend level. Conclusion: The proportion of permanent postoperative CDI was 16% (95% CI 9–24), and that of the transient form 35%  (95%  CI 25–45). ˂0.83 cm3  at trend level and more rare in patients with acromegaly and loss of tropic hormones before surgery at trend level as well. No significant risk factors were identified for permanent CDI, but it could be more often associated with secondary hypothyroidism, absence of any adenoma at magnetic resonance imaging and pituitary injury during the surgery and less frequent in patients with macroadenomas at trend level.Conclusion: The proportion of permanent postoperative CDI was 16% (95% CI 9–24), and that of the transient form 35%  (95%  CI 25–45). Cushing's disease, microadenoma, development of secondary adrenal insufficiency and adrenocorticotropic hormone levels of˂15.8 pg/mL after surgery increase the probability of transient CDI, whereas macroadenoma does decrease this risk. No significant risk factors were identified for permanent CDI. 


2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Keijun Kakihara ◽  
Kengo Asamizu ◽  
Kei Moritsugu ◽  
Masahide Kubo ◽  
Tetsuya Kitaguchi ◽  
...  

AbstractUbiquitin-specific protease 8 (USP8) is a deubiquitinating enzyme involved in multiple membrane trafficking pathways. The enzyme activity is inhibited by binding to 14-3-3 proteins. Mutations in the 14-3-3-binding motif in USP8 are related to Cushing’s disease. However, the molecular basis of USP8 activity regulation remains unclear. This study identified amino acids 645–684 of USP8 as an autoinhibitory region, which might interact with the catalytic USP domain, as per the results of pull-down and single-molecule FRET assays performed in this study. In silico modelling indicated that the region forms a WW-like domain structure, plugs the catalytic cleft, and narrows the entrance to the ubiquitin-binding pocket. Furthermore, 14-3-3 inhibited USP8 activity partly by enhancing the interaction between the WW-like and USP domains. These findings provide the molecular basis of USP8 autoinhibition via the WW-like domain. Moreover, they suggest that the release of autoinhibition may underlie Cushing’s disease due to USP8 mutations.


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