Autoimmune Polyendocrine Syndrome Complicated by Pulmonary Hypertension

2020 ◽  
Vol 20 ◽  
Author(s):  
Olga Eugenia Disoteo ◽  
Benedetta Zampetti ◽  
Andrea Garascia ◽  
Roberto Attanasio ◽  
Renato Cozzi
Keyword(s):  
Clinical Course ◽  
Diabetes Mellitus Type 1 ◽  
Primary Hypothyroidism ◽  
Autoimmune Polyendocrine Syndrome ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Work Up ◽  
Mean Time

: A 24-yo female was admitted for acute renal failure, melanoderma, hyponatremia and hyperkalemia. The clinical suspicion of Addison's disease was confirmed by laboratory test and the appropriate replacement therapy with corticosteroids and fludrocortisone was started. In the mean-time primary hypothyroidism and diabetes mellitus type 1 were disclosed and treated, thus fulfilling a diagnosis of autoimmune polyendocrine syndrome type 2. Eighteen months later she was admitted for right sided heart failure. The work-up allowed to diagnose pulmonary arterial hypertension. Here we report the clinical course and discuss the putative link between these two rare diseases.

scholarly journals Surgical results for hypertensive intracerebral hemorrhagesdepending on intervention timing

2021 ◽  
Vol 15 (4) ◽  
pp. 36-43
Author(s):  
Vladimir G. Dashyan ◽  
Ivan M. Godkov ◽  
Leonid V. Prokop’ev ◽  
Andrey A. Grin ◽  
Vladimir V. Krylov
Keyword(s):  
Disease Onset ◽  
Haemorrhagic Stroke ◽  
Total Sample ◽  
Intracerebral Haematoma ◽  
Intracranial Hemorrhages ◽  
The Mean ◽  
Type 3 ◽  
Mean Time

Study aim. To analyse the surgical outcomes in patients with haemorrhagic stroke depending on the timing of surgery. Materials and methods. We performed a retrospective analysis of 500 patients (335 (67%) men and 165 (33%) women), who underwent surgical treatment of hypertensive intracranial hemorrhages at the N.V. Sklifosovsky Research Institute for Emergency Medicine between 1997 and 2020. The mean patient age was 53.1 12.2 years. The mean time until surgery was 3.3 2.6 days. Outcomes were assessed on day 30 from disease onset using the modified Rankin Scale (mRS). Results. In the total sample, outcomes as measured by the mRS were as follows: type 0 84 (16.8%) patients, type 1 37 (7.4%), type 2 46 (9.2%), type 3 38 (7.6%), type 4 43 (8.6%), type 5 142 (28.4%) and type 6 110 (22.0%). Treatment results were better when surgery was delayed (2 = 64.4; p 0.00001). Mortality was 36.4% after surgery conducted in the first day after haemorrhage, while mRS scores of 02 made up 18.6%. Mortality was 20.4% after surgery conducted on the second or third day, and mRS scores of 02 made up 29.6%. Mortality was 17.4% after surgery conducted on day 47, and mRS scores of 02 outcomes were present in 49.0% of subjects. Mortality was 8.8% when surgery was performed on day 8 or later, and favourable outcomes were present in 48.5% of patients. Conclusion. Intracerebral haematoma excision on day 23 leads to better outcomes in patients with reduced levels of alertness up to sopor, while surgery after day 3 leads to better results in alert patients and those with obtundation.

scholarly journals EXPRESS: Pulmonary Hypertension Associated With Neurofibromatosis Type 2

2021 ◽  
pp. 204589402110295
Author(s):  
Hirohisa Taniguchi ◽  
Tomoya Takashima ◽  
Ly Tu ◽  
Raphaël Thuillet ◽  
Asuka Furukawa ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Neurofibromatosis Type ◽  
Pulmonary Vascular Remodeling ◽  
Life Threatening ◽  
History Of ◽  
Pulmonary Arterial ◽  
First Time

Although precapillary pulmonary hypertension (PH) is a rare but severe complication of patients with neurofibromatosis type 1 (NF1), its association with NF2 remains unknown. Herein, we report a case of a 44-year-old woman who was initially diagnosed with idiopathic pulmonary arterial hypertension (IPAH) and treated with PAH-specific combination therapy. However, a careful assessment for a relevant family history of the disease and genetic testing reveal that this patient had a mutation in the NF2 gene. Using immunofluorescence and Western blotting, we demonstrated a decrease in endothelial NF2 protein in lungs from IPAH patients compared to control lungs, suggesting a potential role of NF2 in PAH development. To our knowledge, this is the first time that precapillary PH has been described in a patient with NF2. The altered endothelial NF2 expression pattern in PAH lungs should stimulate work to better understand how NF2 is contributing to the pulmonary vascular remodeling associated to these severe life-threatening conditions.

321P Clinical course comparison between patients with type 1 and type 2 uterine endometrial cancers

2016 ◽  
Vol 27 (suppl_9) ◽  
Author(s):  
Y. Takehara ◽  
M. Yunokawa ◽  
S. Sasada ◽  
E. Noguchi ◽  
A. Shimomura ◽  
...  
Keyword(s):  
Clinical Course ◽  
Endometrial Cancers

scholarly journals Modified approach to the characterization of adrenal nodules using a standard abdominal magnetic resonance imaging protocol

2017 ◽  
Vol 50 (1) ◽  
pp. 19-25 ◽  
Author(s):  
António P. Matos ◽  
Richard C. Semelka ◽  
Vasco Herédia ◽  
Mamdoh AlObaidiy ◽  
Filipe Veloso Gomes ◽  
...  
Keyword(s):  
Sensitivity And Specificity ◽  
Imaging Protocol ◽  
Intensity Index ◽  
Abdominal Magnetic Resonance Imaging ◽  
Phase Type ◽  
The Mean ◽  
Magnetic Resonance Imaging Protocol ◽  
Type 3

Abstract Objective: To describe a modified approach to the evaluation of adrenal nodules using a standard abdominal magnetic resonance imaging protocol. Materials and Methods: Our sample comprised 149 subjects (collectively presenting with 132 adenomas and 40 nonadenomas). The adrenal signal intensity index was calculated. Lesions were grouped by pattern of enhancement (PE), according to the phase during which the wash-in peaked: arterial phase (type 1 PE); portal venous phase (type 2 PE); and interstitial phase (type 3 PE). The relative and absolute wash-out values were calculated. To test for mean differences between adenomas and nonadenomas, Student's t-tests were used. Receiver operating characteristic curve analysis was also performed. Results: The mean adrenal signal intensity index was significantly higher for the adenomas than for the nonadenomas (p < 0.0001). Chemical shift imaging showed a sensitivity and specificity of 94.4% and 100%, respectively, for differentiating adenomas from nonadenomas. Of the adenomas, 47.6%, 48.5%, and 3.9%, respectively, exhibited type 1, 2, and 3 PEs. For the mean wash-in proportions, significant differences were found among the enhancement patterns. The wash-out calculations revealed a trend toward better lesion differentiation for lesions exhibiting a type 1 PE, showing a sensitivity and specificity of 71.4% and 80.0%, respectively, when the absolute values were referenced, as well as for lesions exhibiting a type 2 PE, showing a sensitivity and specificity of 68.0% and 100%, respectively, when the relative values were referenced. The calculated probability of a lipid-poor lesion that exhibited a type 3 PE being a nonadenoma was > 99%. Conclusion: Subgrouping dynamic enhancement patterns yields high diagnostic accuracy in differentiating adenomas from nonadenomas.

scholarly journals There is a Correlation between Fragility Fractures of the Pelvis and a Circle-Type Morphology of the True Pelvis, but Not with Pelvic Incidence

2021 ◽  
pp. 1-6
Author(s):  
Matthias Spalteholz ◽  
Matthias Spalteholz ◽  
Gulow Jens ◽  
Pap Geza
Keyword(s):  
Pelvic Incidence ◽  
Fragility Fractures ◽  
Statistical Correlation ◽  
X Ray ◽  
Lack Of Information ◽  
The Mean ◽  
Fracture Types ◽  
Type 3

Purpose: Osteoporosis is a major risk factor for the development of fragility fractures of the pelvis (FFP). There is a lack of information about the influence of anatomical conditions such as Pelvic Incidence and Pelvic Ratio (DT/DS ratio) on this kind of fractures. Methods: This is a monocentric retrospective analysis. X-ray images of the lumbar spine and pelvis and 3D-MPR CT reconstructions of the pelvis were analysed to determine Pelvic Incidence (PI) and Pelvic Ratio (PR) in 141 fragility fractures of the pelvis. Statistical analyses were performed to examine the correlation between these spinopelvic parameters and fragility fractures of the pelvis. Results: A total of 141 fragility fractures of the pelvis (14 men = 9.93%, 127 women = 90.07%) were analysed. According to the FFP-classification we recognized FFP type 1 fractures in 19.15%, FFP type 2 in 41.13%, FFP type 3 in 8.51% and FFP type 4 fractures in 32.21%. The mean PI was 58.83º. There was no statistical correlation between PI and fracture types (p=0.81). The mean PR was 1.099. 57 patients (40.43%) demonstrated a DT/DS ratio ≤ 1.06, corresponding to a circle-type morphology. 24 patients (17.02%) demonstrated a DT/DS ratio ≥ 1.18, corresponding to an ellipse-type pelvis. A circle-type pelvis is significantly more often associated with fragility fractures of the pelvis than an ellipse-type morphology (p<0.001). Conclusion: The results of our work demonstrate a strong statistical correlation between the circle-type morphology of the pelvis (PR ≤ 1.06) and fragility fractures of the pelvis. There is no statistical correlation between fragility fractures of the pelvis and Pelvic Incidence.

Surgery in autoimmune pancreatitis

2021 ◽  
Author(s):  
Sara Nikolic ◽  
Poya Ghorbani ◽  
Raffaella Pozzi Mucelli ◽  
Sam Ghazi ◽  
Francisco Baldaque- Silva ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
University Hospital ◽  
Premalignant Lesions ◽  
Single Centre ◽  
Age At Surgery ◽  
Diagnostic Work ◽  
Work Up

Introduction: Autoimmune pancreatitis (AIP) is a disease that may mimic malignant pancreatic lesions both in terms of symptomatology and imaging appearance. The aim of the present study is to analyse experiences of surgery in patients with AIP in one of the largest European cohorts. Methods: We performed a single-centre retrospective study of patients diagnosed with AIP at the Department of Abdominal Diseases at Karolinska University Hospital in Stockholm, Sweden, between January 2001 and October 2020. Results: There were 159 patients diagnosed with AIP, and among them 35 (22.0%) patients had surgery: 20 (57.1%) males and 15 (42.9%) females; average age at surgery was 59 years (range 37-81). Follow-up period after surgery was 67 months (range 1-235). AIP type 1 was diagnosed in 28 (80%) patients and AIP type 2 in 7 (20%) patients. Malignant and premalignant lesions were diagnosed in 8 (22.9%) patients for whom AIP was not the primary differential diagnosis but, in all cases, it was described as a simultaneous finding and recorded in retrospective analysis in histological reports of surgical specimens. Conclusions: Diagnosis of AIP is not always straightforward, and, in some cases, it is not easy to differentiate it from the malignancy. Surgery is generally not indicated for AIP but might be considered in patients when suspicion of malignant/premalignant lesions cannot be excluded after complete diagnostic work-up.

NASAL GLUCAGON DELIVERY IS MORE SUCCESSFUL THAN INJECTABLE DELIVERY: A SIMULATED SEVERE HYPOGLYCEMIA RESCUE

2020 ◽  
Vol 26 (4) ◽  
pp. 407-415
Author(s):  
Julie A. Settles ◽  
Gregg Francis Gerety ◽  
Erik Spaepen ◽  
Jeffrey Gideon Suico ◽  
Christopher J. Child
Keyword(s):  
Success Rate ◽  
High Stress ◽  
Real Life ◽  
Severe Hypoglycemia ◽  
High Fidelity ◽  
Success Rates ◽  
Dose Delivery ◽  
Mean Time

Objective: A severe hypoglycemia (SH) episode is an acute, high-stress moment for the caregivers of persons with diabetes (PWD). We compared the success rates of nasal glucagon (NG) and injectable glucagon (IG) administration for PWD-trained and untrained users in treating simulated SH episodes. Methods: Thirty-two PWD-trained users and 33 untrained users administered NG and IG to high-fidelity manikins simulating treatment of an SH emergency. Simulation rooms resembled common locations with typical diabetic supplies and stressor elements mimicking real-life SH environments. Success rate and time to administer glucagon were measured. Results: Of all the PWD-trained and untrained users, 58/64 (90.6%) could successfully deliver NG, while 5/63 (7.9%) could successfully deliver IG. For NG simulations, 28/31 (90.3%) PWD-trained users and 30/33 (90.9%) untrained users could successfully administer the dose (mean time 47.3 seconds and 44.5 seconds, respectively). For IG simulations, 5/32 (15.6%) PWD-trained users successfully injected IG (mean time 81.8 seconds), whereas none (0/31 [0%]) of the untrained users were successful. Reasons for unsuccessful administration of NG included oral administration and incomplete pushing of the device plunger. For IG, inability to perform reconstitution steps, partial dose delivery, and injection at an inappropriate site were the causes for failure. Conclusion: With or without training, the success rate for administering NG was 90.6%, whereas it was only 7.9% for IG. NG was easily and quickly administered even by untrained users, whereas training was necessary for successful administration of IG. NG may expand the community of caregivers who can help PWD during an SH episode. Abbreviations: IG = injectable glucagon; NG = nasal glucagon; PWD = person with diabetes; SH = severe hypoglycemia; T1D = type 1 diabetes; T2D = type 2 diabetes

scholarly journals Expression of cyclooxygenase-2 in orbital fibroadipose connective tissues of Graves’ ophthalmopathy patients

2006 ◽  
Vol 155 (5) ◽  
pp. 681-685 ◽  
Author(s):  
E Berrin Yuksel Konuk ◽  
Onur Konuk ◽  
Muge Misirlioglu ◽  
Adnan Menevse ◽  
Mehmet Unal
Keyword(s):  
Disease Duration ◽  
Clinical Activity ◽  
Connective Tissues ◽  
Positive Correlation ◽  
Graves Ophthalmopathy ◽  
Compressive Neuropathy ◽  
The Mean ◽  
Cox 2

Objective: The aim of this study is to evaluate the expression of cycloocygenase-2 (COX-2) in orbital fibroadipose connective tissue in Graves’ ophthalmopathy (GO) patients, and investigate the associations between COX-2 expression and GO characteristics. Methods: The orbital fibroadipose connective tissues of 23 cases demonstrating moderate or severe GO, and eight control subjects without any history of thyroid or autoimmune disease were analyzed for COX-2 mRNA expression. Real-time relative quantitative PCR was performed to assess transcripts of COX-2 using the LightCycler. The disease activity was evaluated by the clinical activity score (CAS). The clinical features of GO were evaluated by total eye score (TES) and the cases were divided into two groups; type 1 cases included higher degrees of proptosis with orbital fat volume increase, and type 2 cases included cases with compressive neuropathy and limited extraocular muscle functions. Results: The mean ± s.d. disease duration was 5.7 ± 7.1 years. The mean ± s.d. CAS and TES of cases were 1.60 ± 1.04 and 7.5 ± 1.8 respectively. The mean ± s.d. expression of COX-2 was 0.023 ± 0.013 and 0.010 ± 0.002 in GO cases and controls (P = 0.008), and 0.015 ± 0.073 and 0.029 ± 0.135 in type 1 and type 2 cases respectively (P = 0.007). COX-2 expression showed a statistically significant positive correlation with TES (r = 0.634, P = 0.001), and a negative correlation with the disease duration (r = −0.621, P = 0.002). Conclusions: COX-2 is expressed at higher levels in orbital fibroadipose tissues of GO cases. This showed a positive correlation with increasing severity of orbital disease suggesting possible relation with COX-2 expression and orbital inflammation in GO.

Genetic Polymorphisms of Antioxidant and Antiglycation Enzymes and Diabetic Complications. How Much Can we Learn from the Genes?

2017 ◽  
Vol 126 (01) ◽  
pp. 7-13
Author(s):  
Dimitrios Oikonomou ◽  
Jan Benedikt Groener ◽  
Ruan Cheko ◽  
Zoltan Kender ◽  
Lars Kihm ◽  
...  
Keyword(s):  
Diabetic Complications ◽  
Diabetes Mellitus Type 1 ◽  
Diabetic Complication ◽  
Clinical Use ◽  
Oxygen Species ◽  
Patients With Diabetes ◽  
Genetically Determined ◽  
Novel Therapeutic

AbstractThere is growing evidence that reactive metabolites, such as reactive oxygen species and dicarbonyls contribute to diabetic complications. Formation, accumulation, and detoxification of these metabolites are controlled by several enzymes, some of which have genetically determined levels of expression or function. This review not only gives an overview of the different SNPs studied in patients with diabetes mellitus type 1 and type 2, but in addition attempts to bridge the gap between a genetic study and clinical use. Therefore, not only the results of the studies are reviewed, but also their use in identification of subgroups where an increased or decreased risk for a diabetic complication is described, as well as their use in developing novel therapeutic options based on understanding the contribution of an enzyme to a given complication.

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