A Chinese case of X-linked acrogigantism and systemic review

2020 ◽  
Author(s):  
Hanting Liang ◽  
Fengying Gong ◽  
Zhihui Liu ◽  
Yingying Yang ◽  
Yong Yao ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Web Of Science ◽  
Standard Deviation Score ◽  
Systemic Review ◽  
Maximum Diameter ◽  
Comparative Genomic ◽  
Facial Features ◽  
Chinese Case ◽  
Pituitary Enlargement ◽  
Magnetic Resonance Imaging Mri

Introduction: To describe a Chinese case of X-linked acrogigantism (X-LAG) and summarize the characteristics and treatment of all reported cases. Methods: Clinical materials and biological samples from a 5-year and 2-month-old female due to “growth acceleration for 4 years” were collected. Array comparative genomic hybrid (aCGH) and further verification were performed. All X-LAG cases from the PubMed and Web of Science databases were collected and summarized using available data. Results: The patient presented accelerating growth since 1 year of age, and her height reached 134.6 cm (+5.24 standard deviation score, SDS) when she was 5 years and 2 months old. She also had coarsening facial features, snoring, and acral enlargement. Growth hormone (GH) was not suppressed by the glucose-GH inhibition test, and insulin-like growth factor 1 (IGF-1) and prolactin levels were elevated. Pituitary magnetic resonance imaging (MRI) revealed a pituitary enlargement with a maximum diameter of 22.3 mm. Octreotide imaging indicated the presence of a pituitary adenoma. The tumor shrank slightly after three courses of somatostatin analog but without clinical or biochemical remissions, of which the GH nadir value was 9.4 ng/ml, and IGF-1 was elevated to 749 ng/ml. Therefore, she underwent transsphenoidal surgery. Immunohistochemistry showed GH-positive and PRL-positive cells in the pituitary adenoma. Xq26.3 microduplication of the patient’s germline DNA was identified by aCGH. Of all 35 reported cases, females accounted for 71.43%. There were 93.10% and 53.83% patients with hyperprolactinemia and hyperinsulinemia, respectively. Pathology showed that 75.00% of cases were adenomas. Ninety percent of cases had germline variants. The clinical and biochemical remission rates were 78.26% and 82.61%, respectively. However, the rate of complication occurrence during therapy reached 80%. Conclusion: It is important to recognize the possibility of X-LAG when a child under 2 years old presents overgrowth. Early diagnosis and treatment are of great importance for better treatment efficacy and clinical outcome.

Neuroimaging Outcomes in Studies of Cognitive Training in Mild Cognitive Impairment and Early Alzheimer’s Disease: A Systematic Review

2020 ◽  
Vol 17 (5) ◽  
pp. 472-486
Author(s):  
Lucy Beishon ◽  
Kannakorn Intharakham ◽  
David Swienton ◽  
Ronney B. Panerai ◽  
Thompson G. Robinson ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cognitive Impairment ◽  
Search Strategy ◽  
Web Of Science ◽  
Cochrane Library ◽  
Functional Magnetic Resonance ◽  
Resonance Imaging ◽  
Early Alzheimer’S Disease ◽  
Magnetic Resonance Imaging Mri ◽  
The Cochrane Library

Background: Cognitive Training (CT) has demonstrated some benefits to cognitive and psychosocial function in Mild Cognitive Impairment (MCI) and early dementia, but the certainty related to those findings remains unclear. Therefore, understanding the mechanisms by which CT improves cognitive functioning may help to understand the relationships between CT and cognitive function. The purpose of this review was to identify the evidence for neuroimaging outcomes in studies of CT in MCI and early Alzheimer’s Disease (AD). Methods: Medline, Embase, Web of Science, PsycINFO, CINAHL, and The Cochrane Library were searched with a predefined search strategy, which yielded 1778 articles. Studies were suitable for inclusion where a CT program was used in patients with MCI or AD, with a structural or functional Magnetic Resonance Imaging (MRI) outcome. Studies were assessed for quality using the Downs and Black criteria. Results: Medline, Embase, Web of Science, PsycINFO, CINAHL, and The Cochrane Library were searched with a predefined search strategy, which yielded 1778 articles. Studies were suitable for inclusion where a CT program was used in patients with MCI or AD, with a structural or functional Magnetic Resonance Imaging (MRI) outcome. Studies were assessed for quality using the Downs and Black criteria. Conclusions: CT resulted in variable functional and structural changes in dementia, and conclusions are limited by heterogeneity and study quality. Larger, more robust studies are required to correlate these findings with clinical benefits from CT.

scholarly journals Association between CYP3A5 Polymorphism and Statin-Induced Adverse Events: A Systemic Review and Meta-Analysis

2021 ◽  
Vol 11 (7) ◽  
pp. 677
Author(s):  
Jeong Yee ◽  
Hamin Kim ◽  
Yunhee Heo ◽  
Ha-Young Yoon ◽  
Gonjin Song ◽  
...  
Keyword(s):  
Adverse Events ◽  
Web Of Science ◽  
Meta Analysis ◽  
Statistical Significance ◽  
Systemic Review ◽  
Event Risk ◽  
Total Data ◽  
Lipophilic Statin ◽  
The Relationship ◽  
Review Manager

Purpose: Cytochrome P450 (CYP) is involved in the metabolism of statins; CYP3A5 is the main enzyme responsible for lipophilic statin metabolism. However, the evidence of the association between CYP3A5*3 polymorphism and the risk of statin-induced adverse events remains unclear. Therefore, this study aimed to perform a systematic review and meta-analysis to investigate the relationship between the CYP3A5*3 polymorphism and the risk of statin-induced adverse events. Methods: The PubMed, Web of Science, and EMBASE databases were searched for qualified studies published until August 2020. Observational studies that included the association between statin-induced adverse events and the CYP3A5*3 polymorphism were reviewed. The odds ratios (ORs) and 95% confidence intervals (CIs) were evaluated to assess the strength of the relationship. The Mantel–Haenszel method was used to provide the pooled ORs. Heterogeneity was estimated with I2 statistics and publication bias was determined by Begg’s and Egger’s test of the funnel plot. Data analysis was performed using Review Manager (version 5.4) and R Studio (version 3.6). Results: In total, data from 8 studies involving 1614 patients were included in this meta-analysis. The CYP3A5*3 polymorphism was found to be associated with the risk of statin-induced adverse events (*3/*3 vs. *1/*1 + *1/*3: OR = 1.40, 95% CI = 1.08–1.82). For myopathy, the pooled OR was 1.30 (95% CI: 0.96–1.75). The subgroup analysis of statin-induced myopathy revealed a trend, which did not achieve statistical significance. Conclusions: This meta-analysis demonstrated that the CYP3A5*3 polymorphism affected statin-induced adverse event risk. Therefore, CYP3A5 genotyping may be useful to predict statin toxicity.

NCOG-31. GIANT PITUITARY ADENOMA (GPA) SCORE: A NOVEL SCORING SYSTEM TO PREDICT POSTOPERATIVE OUTCOMES OF GIANT PITUITARY ADENOMAS

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi158-vi158
Author(s):  
Syed Ather Enam ◽  
Fauzan Alam Hashmi ◽  
Sanam Mir Ghazi ◽  
Ahsan Ali Khan ◽  
Muhammad Bilal Tariq ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Linear Regression ◽  
Pituitary Adenomas ◽  
Scoring System ◽  
Tumor Resection ◽  
Maximum Diameter ◽  
Giant Pituitary Adenoma ◽  
Giant Pituitary Adenomas ◽  
The Mean ◽  
Suprasellar Extension

Abstract BACKGROUND Giant pituitary adenomas (GPA) are uncommon and highly variable in morphology and extension. There is no scoring system that considers all the dimensions of adenoma invasion. We developed a new Giant Pituitary Adenoma score and report our surgical experience and evaluate outcomes after resection of these tumors in accordance with the preoperative score. METHODS We developed a novel scoring system for classifying giant pituitary adenomas, and 11-year data of GPA surgery at our center was collected retrospectively, based on this scoring system. GPA Score considered tumor’s parasellar extension, encasement of cavernous internal carotid artery (ICA), suprasellar extension > 2 cm, suprasellar extension > 4cm and retrosellar extension. Maximum possible score was 9. The scoring system was applied to 53 patients of GPA who underwent surgical resection between January 1, 2006, and December 2017. The Lundin-Pederson (ABC/2) method was used to calculate the tumor volume both pre- and post-resection and linear regression was used to assess the relationship between extent of tumor resection and GPA score. RESULTS The median age of the study population was 42.08 ± 16.49 years. The mean maximum diameter of the pituitary adenomas was 5.0 cm (range 4.0 cm-8.5cm) while the mean volume of the adenomas was 27.3 cm3 (range 10 cm3-149 cm3). There were 3 cases of score 2, 5 cases of score 3, 13 cases of score 4, 20 cases of score 5, 9 cases of score 6 and 3 cases of score 7. The range of tumor volumes of tumors for scores from 2-7 was 17.3 cm3 to 65.8 cm3 and GPA score was correlated with the percent residual tumor using linear regression that was statistically significant (p= 0.001). CONCLUSION GPA Score is a reliable scoring system to predict the extent and subsequent difficulty in tumor resection in GPA.

Giant plurihormonal pituitary adenoma in a child – case study

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Elżbieta Moszczyńska ◽  
Wiesława Grajkowska ◽  
Maria Maksymowicz ◽  
Joanna Malicka ◽  
Mieczysław Szalecki ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Middle Cranial Fossa ◽  
Thyroid Stimulating Hormone ◽  
Somatostatin Analogue ◽  
Tall Stature ◽  
Pituitary Macroadenoma ◽  
Hand Tremor ◽  
Cranial Fossa ◽  
Magnetic Resonance Imaging Mri ◽  
Plurihormonal Pituitary Adenoma

Abstract Objectives To describe the case of a 12-year-old girl with a rare plurihormonal pituitary macroadenoma secreting prolactin (PRL), growth hormone (GH), thyroid-stimulating hormone (TSH), and alpha subunit (α-SU). Case presentation The patient experienced recurrent headaches and progressing loss of vision in one eye. During the examination, abnormalities such as tall stature, coarse facial features, enlarged feet and hands, tachycardia, hand tremor, hyperhidrosis, galactorrhea, and goiter were observed. Head magnetic resonance imaging (MRI) revealed a solid tumor in the anterior and middle cranial fossa, measuring 80 × 50 × 55 mm. A stereotactic biopsy revealed plurihormonal Pit-1 positive pituitary adenoma secreting PRL, GH, and TSH. A pituitary hyperfunction with PRL, GH, TSH, and α-SU excess was diagnosed. The patient was successfully treated pharmacologically with dopamine agonists and somatostatin analogue, and a decrease of tumor volume (30%) was achieved. Conclusions When neurosurgery is not possible, long-term pharmacological treatment of plurihormonal pituitary macroadenoma can be a safe and relatively effective alternative.

Ostmann’s Fat Pad—Does it Really Matter?

2020 ◽  
pp. 000348942094321
Author(s):  
Ameen Biadsee ◽  
Feda Fanadka ◽  
Or Dagan ◽  
Kassem Firas ◽  
Benny Nageris
Keyword(s):  
Otitis Media ◽  
Chronic Otitis Media ◽  
Statistical Correlation ◽  
Maximum Diameter ◽  
Control Group ◽  
Fat Pad ◽  
Medial Border ◽  
Medial Pterygoid ◽  
Tensor Veli Palatini Muscle ◽  
Magnetic Resonance Imaging Mri

Objective: To compare the size of Ostmann’s fat pad (OFP) between healthy ears and ears with chronic otitis media with cholestatoma (COMwC) using magnetic resonance imaging (MRI). Methods: Twenty-six patients with unilateral COMwC underwent mastoidectomy. Pre-operative MRI records were reviewed retrospectively. The healthy ears served as the control group. OFP is represented by the maximum diameter of the high intensity area medial to the tensor veli palatini muscle (TVP); M1. A reference diameter was defined from the medial border of OFP reaching the medial border of the medial pterygoid muscle; M2. Values of M1, M2 and the ratio of M1:M2 was compared between the healthy and pathological ear in each patient. Results: All 26 patients (16 females,10 males) had unilateral cholestatoma. Mean age was 37.6 years (range 19-83). In the healthy (H) ears group, mean M1H was 2.04 ± 0.53 mm, mean M2H was 9.57 ± 2.57 mm. In the pathological (P) ears group; mean M1P was 2.03 ± 0.55 mm, mean M2P was 9.86 ± 2.37 mm. A comparison of M1 and M2 values between the healthy and pathological ear groups was not statistically significant ( P = .853 and P = .509, respectively). Mean M1H:M2H ratio in the healthy ears group was 0.22 ± 0.05, mean M1P:M2P ratio in the pathological ear group was 0.21 ± 0.06. A comparison between these ratios found no significant statistical correlation ( P = .607). Conclusion: The size of Ostmann’s fat pad does not affect the development of chronic otitis media with cholestatoma in adults.

scholarly journals GH-Producing Pituitary Adenoma and Concomitant Rathke’s Cleft Cyst: A Case Report and Short Review

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Ryota Tamura ◽  
Satoshi Takahashi ◽  
Katsura Emoto ◽  
Hideaki Nagashima ◽  
Masahiro Toda ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Sella Turcica ◽  
Short Review ◽  
Pituitary Hormones ◽  
Rathke’S Cleft Cyst ◽  
Resonance Imaging ◽  
Mr Images ◽  
Rathke's Cleft Cyst ◽  
Magnetic Resonance Imaging Mri ◽  
Rathke's Cleft

Concomitant pituitary adenoma (PA) and Rathke’s cleft cyst (RCC) are rare. In some cases, such PA is known to produce pituitary hormones. A 53-year-old man was admitted to our hospital with a diagnosis of lacunar infarction in the left basal ganglia. Magnetic resonance imaging (MRI) incidentally showed a suprasellar mass with radiographic features of RCC. When he consulted with a neurosurgical outpatient clinic, acromegaly was suspected based on his appearance. A diagnosis of growth hormone- (GH-) producing PA was confirmed from hormonal examinations and additional MRI. Retrospectively, initial MR images also showed intrasellar mass that is compatible with the diagnosis of PA other than suprasellar RCC. The patient underwent endonasal-endoscopic removal of the PA. Since we judged that the RCC of the patient was asymptomatic, only the PA was completely removed. The postoperative course of the patient was uneventful and GH levels gradually normalized. Only 40 cases of PA with concomitant RCC have been reported to date, including 13 cases of GH-producing PA. In those 13 cases, RCC tended to be located in the sella turcica, and suprasellar RCC like this case appears rare. In a few cases, concomitant RCCs were fenestrated, but GH levels normalized postoperatively as in the cases without RCC fenestration. If radiographic imaging shows typical RCC, and PA is not obvious at first glance, the possibility of concomitant PA still needs to be considered. In terms of treatment, removal of the RCC is not needed to achieve hormone normalization.

scholarly journals Quantitative Volumetric Analysis Post Transsphenoidal Pituitary Adenoma Surgery

2012 ◽  
Vol 39 (5) ◽  
pp. 600-604 ◽  
Author(s):  
Alireza Mansouri ◽  
Sean Symons ◽  
Michael Schwartz ◽  
Joseph Chen ◽  
Farhad Pirouzmand
Keyword(s):  
Pituitary Adenoma ◽  
Volumetric Analysis ◽  
Point Of View ◽  
Size Difference ◽  
Resonance Imaging ◽  
Residual Mass ◽  
Postoperative Assessment ◽  
The Difference ◽  
Magnetic Resonance Imaging Mri

Background:Computed tomogram (CT) imaging is often used for immediate postoperative assessment of transsphenoidal pituitary adenoma resection while magnetic resonance imaging (MRI) is used for follow-up. The residual mass is known to decrease in size over time but the difference between the two imaging modalities has not been quantified. Our objective was to quantify the size difference of the residual mass on immediate postoperative CT compared with delayed MRI.Methods:Retrospective analysis of 69 patients who had undergone pituitary adenoma resection at our institution between 2004-2010. Sellar and suprasellar diameter, along with the overall volume of the residual mass were measured on both the immediate postoperative CT and delayed MRI.Results:Average preoperative sellar and suprasellar diameter was 22.2 ± 4.6mm and 20.9 ± 5.9mm, respectively. Average sellar residual diameter on immediate postoperative CT (16.5 ± 5.4 mm, 25% reduction) was significantly larger than delayed MRI (10.6 ± 6.2mm, 52% reduction). The average suprasellar component on CT (15.5±6.5mm, 26% reduction) was also significantly larger than that on MRI (3.3 ± 5.4 mm, 84% reduction). The postoperative CT showed a 46% reduction in volume while a 71% reduction was noted on the delayed MRI.Conclusion:A significant reduction in residual mass is noted on delayed MR imaging compared with immediate postoperative CT. Therefore, from a resource management and prognostication point of view, CT should be used for immediate postoperative assessment while delayed MRI should be used to assess operative success and for communication with patients.

scholarly journals Molecular epidemiology of hypervirulent Klebsiella pneumoniae: a systematic review and meta-analysis

2021 ◽  
Author(s):  
Rahimeh Sanikhani ◽  
Mohammad Moeinirad ◽  
Fereshteh Shahcheraghi ◽  
Arezou Lari ◽  
Sepideh Fereshteh ◽  
...  
Keyword(s):  
Systematic Review ◽  
Klebsiella Pneumoniae ◽  
Molecular Epidemiology ◽  
Liver Abscess ◽  
Molecular Typing ◽  
Web Of Science ◽  
Meta Analysis ◽  
Systemic Review ◽  
Clonal Group ◽  
Sequence Types

Classical (CKp) and hypervirulent (hvKp) Klebsiella pneumoniae are two different circulating pathotypes. The aim of this study was to assess the prevalence, epidemiology and molecular relatedness of hvKps using a systemic review and meta-analysis. The data extracted from Medline, Embase, and Web of Science and finally 14 studies met the eligible criteria. To combine prevalence proportions of all studies, we performed the metaprop command embedded in the Meta package software. Totally, of 1814 K. pneumoniae isolates, 21.7% (394/1814) were hvKp. The molecular typing showed that all hvKp isolates were grouped into 50 different sequence types (STs) of them ST23, ST11, ST65 and ST86 were common. K1, K2 and K64 were dominant capsule serotypes that strongly related to ST23, ST65 and ST11, respectively. It seems that clonal group 23 (CG23) is associated with liver abscess and CG11 related to various clinical sources.

Potential benefit of GnRH-agonist treatment before uterine artery embolization for large fibroids: MRI prediction of fibroid volume reduction

2021 ◽  
pp. 028418512110388
Author(s):  
Woo Jin Lee ◽  
Man-Deuk Kim ◽  
Kyunghwa Han ◽  
Ye Ryung Won ◽  
Abdulrahman Alqahtani ◽  
...  
Keyword(s):  
Gnrh Agonist ◽  
Uterine Artery Embolization ◽  
Uterine Artery ◽  
Reduction Rate ◽  
Volume Reduction ◽  
Maximum Diameter ◽  
Artery Embolization ◽  
Before And After ◽  
Submucosal Fibroids ◽  
Magnetic Resonance Imaging Mri

Background Signal intensity (SI) of predominant fibroid (F1) on T2-weighted (T2W) images is useful for predicting the volume reduction response after gonadotropin-releasing hormone (GnRH)-agonist treatment. Few studies have been published regarding when and how to use GnRH agonist before UAE. Purpose To investigate magnetic resonance imaging (MRI) prediction of volume reduction rate (VRR) of large fibroids after GnRH-agonist treatment before uterine artery embolization (UAE) as well as the efficacy of UAE based on MRI. Material and Methods Data from 30 patients with a large fibroid and MRI results both before and after GnRH-agonist treatment were retrospectively analyzed. Indications for GnRH-agonist treatment are fibroids with a maximum diameter ≥10 cm or pedunculated submucosal fibroids ≥8 cm. GnRH agonist (3.75 mg leuprolide acetate) was administered subcutaneously once per month 2–6 times. SI of F1 on T2W imaging was measured: the SI was referenced to the SI of the rectus abdominis muscle (F/R). Results Mean maximum fibroid diameter was 11.1 ± 1.9 cm (range = 8.0–15.5 cm). Mean number of GnRH-agonist injections before UAE was 2.8 (range = 2–6). For predicting VRR ≥50% and <30%, the optimal cut-off values of F/R were 2.58 (sensitivity 80%, specificity 80%) and 1.69 (sensitivity 100%, specificity 70%), respectively. Of the 30 patients, fibroid infarction was complete in 29 (96.7%). Conclusion SI of F1 on T2W imaging is useful for predicting the volume reduction response after GnRH-agonist treatment. After GnRH-agonist treatment for large fibroids, UAE is effective to achieve complete infarction of fibroids.

scholarly journals Comparison of Constructive Interference in Steady-State and T1-Weighted MRI Sequence at Detecting Pituitary Adenomas in Cushing's Disease Patients

2018 ◽  
Vol 79 (06) ◽  
pp. 593-598 ◽  
Author(s):  
Min Lang ◽  
Ghaith Habboub ◽  
Doksu Moon ◽  
Abin Bandyopadhyay ◽  
Danilo Silva ◽  
...  
Keyword(s):  
Steady State ◽  
Pituitary Adenoma ◽  
Cushing’S Disease ◽  
Pituitary Adenomas ◽  
Cushing's Disease ◽  
Breath Hold ◽  
Mri Imaging ◽  
Constructive Interference ◽  
Ciss Sequence ◽  
Magnetic Resonance Imaging Mri

Background T1-weighted magnetic resonance imaging (MRI) remains the standard pituitary imaging sequence. However, up to 50% of pituitary adenomas are missed with standard imaging in Cushing's disease (CD) patients. Utility of other imaging sequences needs be explored. Objective To compare a T2-gradient echo sequence, constructive interference in steady state (CISS), with a contrast-enhanced (CE) T1-weighted sequence, volume-interpolated breath-hold examination (VIBE), in the detection of pituitary adenomas in CD patients. Design Retrospective analysis of CD patients who underwent endoscopic transsphenoidal pituitary adenomectomy at our institution. Patients and Measurement Twelve patients were included in the study. Two neuroradiologists, who were blinded to the patients' clinical and surgical findings, independently reviewed the CE-VIBE and CISS images. Localization of pituitary adenoma from imaging was compared with intraoperative localization. Results The averaged sensitivity of detecting pituitary adenoma in CD patients was not significantly different between CE-VIBE sequence (63%) and CISS sequence (54%). The positive predictive value was 75% (Observer A) and 100% (Observer B) with CE-VIBE sequence, and 64% (Observer A) and 100% (Observer B) with CISS sequence. In two patients, pituitary adenoma was easily localized with CISS sequence but hard to detect with CE-VIBE sequence. In two other patients, the adenoma was much better delineated on CE-VIBE sequence. Conclusion In our series, the addition of CISS sequence to T1-weighted MRI imaging protocols improved the detection of pituitary adenomas in CD patients. CISS sequence may be a useful adjunct to T1-weighted pituitary MRI protocols and an appropriate alternative for patients with gadolinium contraindications.

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