scholarly journals Mifepristone Increases Thyroid Hormone Requirements in Patients With Central Hypothyroidism: A Multicenter Study

2019 ◽  
Vol 3 (9) ◽  
pp. 1707-1714 ◽  
Author(s):  
Francisco J Guarda ◽  
James Findling ◽  
Kevin C J Yuen ◽  
Maria Fleseriu ◽  
Lisa B Nachtigall
Keyword(s):  
Thyroid Hormone ◽  
Hormone Level ◽  
Cushing Syndrome ◽  
Case Series ◽  
The United States ◽  
Thyroid Status ◽  
Central Hypothyroidism ◽  
Cushing Disease ◽  
Retrospective Database

Abstract Purpose Mifepristone is a glucocorticoid and progesterone receptor blocker that can be used for patients with hyperglycemia and Cushing syndrome in whom surgery failed to achieve remission or who were ineligible for surgery. We report a case series of patients with Cushing disease (CD) and central hypothyroidism that presented with increased levothyroxine requirements during mifepristone therapy. Methods Retrospective longitudinal case series of patients with CD and central hypothyroidism treated with mifepristone in a retrospective database at four pituitary centers in the United States. Results Five patients with CD were found, all women, median age 50 (interquartile range 47 to 64.5). They received mifepristone because no adequate response or intolerance to other drugs was observed. Mifepristone initiation was associated with a decrease in free thyroxine levels, mandating a dose increase of a median 1.83 (1.71 to 3.5) times the initial dose of levothyroxine to achieve normal levels. Weight loss was seen in four of five patients, ranging from 3.2 to 42.6 kg in up to 54 months of follow-up. Conclusions Although the mechanism behind the decrease in thyroid hormone level is unknown, intestinal malabsorption, decreased residual thyroid function and increased inactivation of T4 via deiodinases are all potential causes. Whereas therapies for hypercortisolism aim to decrease features of hypercortisolemia such as weight gain and depression, hypothyroidism can hamper these goals. This case series raises awareness on the importance of assessment of thyroid status in patients receiving mifepristone to optimize clinical outcomes.

scholarly journals Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma

2019 ◽  
Vol 3 (8) ◽  
pp. 1518-1530 ◽  
Author(s):  
Henrik Falhammar ◽  
Adam Stenman ◽  
Jan Calissendorff ◽  
Carl Christofer Juhlin
Keyword(s):  
Blood Pressure ◽  
Surgical Management ◽  
Cushing Syndrome ◽  
Adrenocortical Adenoma ◽  
Cushing Disease ◽  
Genetic Syndrome ◽  
Alpha Blockers ◽  
Adrenal Medullary Hyperplasia ◽  
Symptoms And Signs

Abstract Context Information about adrenal medullary hyperplasia (AMH) is scarce. Objective To study a large cohort of AMHs. Design, Setting, and Participants Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately. Results Of 936 adrenalectomies, 2.1% had AMH. Mean age was 47.2 ± 15.1 years. Only two (11%) AMHs had no concurrent PCC or adrenocortical adenoma. In AMHs, a genetic syndrome was present in 58% vs 4% in PCCs (P < 0.001). The noradrenaline/metanephrine levels were lower in AMHs, whereas suppression of dexamethasone was less than in PCCs. Cushing syndrome was found in 11% of AMHs. More AMHs were found during screening and less as incidentalomas. PCC symptoms were less prevalent in AMHs. Surgical management was similar; however, fewer of the AMHs were pretreated with alpha-blockers. Adrenalectomy improved blood pressure slightly less in AMHs. The disappearance of glycemic disturbances was similar to the PPCs. During a period of 11.2 ± 9.4 years, a new PCC developed in 32% of patients with AMH, 11% died, but no PCC metastasis occurred (PCCs: 4%, P < 0.001; 14% and 5%). AMHs without PCC had milder symptoms but more often Cushing disease than patients with PCC, whereas AMH with PCC more often displayed a familiar syndrome with more PCC recurrences. Conclusion A total of 2.1% of all adrenalectomies displayed AMH. AMH seemed to be a PCC precursor. The symptoms and signs were milder than PCCs. AMHs were mainly found due to screening. Outcomes seemed favorable, but new PCCs developed in many during follow-up.

To determine the efficacy of bilateral inferior petrosal sinus sampling in differentiating Cushing disease from ectopic Cushing syndrome

2020 ◽  
pp. 1-11
Author(s):  
Muhammad Azeemuddin ◽  
Tanveer Ul Haq ◽  
Shahmeer Khan ◽  
Raza Sayani ◽  
Ayesha Shoukat Hussain ◽  
...  
Keyword(s):  
Cushing Syndrome ◽  
Case Series ◽  
Invasive Procedure ◽  
University Hospital ◽  
Inferior Petrosal Sinus ◽  
Cushing Disease ◽  
Post Contrast ◽  
Inferior Petrosal Sinus Sampling ◽  
Positive Results

Abstract Management of endogenous Cushing syndrome is based on its aetiology. Increased Adrenocorticotropic Hormone (ACTH) levels are the most common cause of this disorder and, therefore, it is critical to determine the source of ACTH before further management. Dynamic post contrast MRI is currently the most common investigation implied to diagnose pituitary adenoma; however, it comes with the drawback of low specificity and high false positive results. Inferior petrosal sinus sampling (IPSS) is an established invasive procedure performed to differentiate central versus peripheral source of ACTH which, in turn, results in hypercortesolaemia. This is a series of 14 patients who underwent IPSS at the Department of Radiology, Aga Khan University Hospital, Karachi, from January 2006 to December 2018. The case series emphasises the role of IPSS in the management of ACTH-dependent Cushing syndrome and combined efficacy of Dynamic post-contrast MRI and the procedure under focus. Continuous....

Early implementation of stereoelectroencephalography in children: a multiinstitutional case series

2021 ◽  
pp. 1-8
Author(s):  
Jacob R. Lepard ◽  
Irene Kim ◽  
Anastasia Arynchyna ◽  
Sean M. Lew ◽  
Robert J. Bollo ◽  
...  
Keyword(s):  
Case Series ◽  
The United States ◽  
Postoperative Hemorrhage ◽  
Pediatric Epilepsy ◽  
Continuous Variables ◽  
Early Implementation ◽  
Significant Difference ◽  
Normative Practice ◽  
The Mean

OBJECTIVE Pediatric stereoelectroencephalography (SEEG) has been increasingly performed in the United States, with published literature being limited primarily to large single-center case series. The purpose of this study was to evaluate the experience of pediatric epilepsy centers, where the technique has been adopted in the last several years, via a multicenter case series studying patient demographics, outcomes, and complications. METHODS A retrospective cohort methodology was used based on the STROBE criteria. ANOVA was used to evaluate for significant differences between the means of continuous variables among centers. Dichotomous outcomes were assessed between centers using a univariate and multivariate logistic regression. RESULTS A total of 170 SEEG insertion procedures were included in the study from 6 different level 4 pediatric epilepsy centers. The mean patient age at time of SEEG insertion was 12.3 ± 4.7 years. There was no significant difference between the mean age at the time of SEEG insertion between centers (p = 0.3). The mean number of SEEG trajectories per patient was 11.3 ± 3.6, with significant variation between centers (p < 0.001). Epileptogenic loci were identified in 84.7% of cases (144/170). Patients in 140 cases (140/170, 82.4%) underwent a follow-up surgical intervention, with 47.1% (66/140) being seizure free at a mean follow-up of 30.6 months. An overall postoperative hemorrhage rate of 5.3% (9/170) was noted, with patients in 4 of these cases (4/170, 2.4%) experiencing a symptomatic hemorrhage and patients in 3 of these cases (3/170, 1.8%) requiring operative evacuation of the hemorrhage. There were no mortalities or long-term complications. CONCLUSIONS As the first multicenter case series in pediatric SEEG, this study has aided in establishing normative practice patterns in the application of a novel surgical technique, provided a framework for anticipated outcomes that is generalizable and useful for patient selection, and allowed for discussion of what is an acceptable complication rate relative to the experiences of multiple institutions.

Intermediate-term Experience With the STAR Total Ankle in the United States

2018 ◽  
Vol 40 (3) ◽  
pp. 268-275 ◽  
Author(s):  
Evan M. Loewy ◽  
Thomas H. Sanders ◽  
Arthur K. Walling
Keyword(s):  
United States ◽  
Perioperative Complications ◽  
Case Series ◽  
Major Complication ◽  
The United States ◽  
Complication Rates ◽  
Level Of Evidence ◽  
Patient Reported ◽  
The Mean

Background: Limited intermediate and no real long-term follow-up data have been published for total ankle arthroplasty (TAA) in the United States. This is a report of clinical follow-up data of a prospective, consecutive cohort of patients who underwent TAA by a single surgeon from 1999 to 2013 with the Scandinavian Total Ankle Replacement (STAR) prosthesis. Methods: Patients undergoing TAA at a single US institution were enrolled into a prospective study. These patients were followed at regular intervals with history, physical examination, and radiographs; American Orthopaedic Foot & Ankle Society (AOFAS) Ankle-Hindfoot Scale scores were obtained and recorded. Primary outcomes included implant survivability and functional outcomes scores. Secondary outcomes included perioperative complications such as periprosthetic or polyethylene fracture. Between 1999 and 2013, a total of 138 STAR TAAs were performed in 131 patients; 81 patients were female. The mean age at surgery was 61.5 ± 12.3 years (range, 30-88 years). The mean duration of follow-up for living patients who retained both initial components at final follow-up was 8.8±4.3 years (range 2-16.9 years). Results: The mean change in AOFAS Ankle-Hindfoot scores from preoperative to final follow-up was 36.0 ± 16.8 ( P < .0001). There were 21 (15.2%) implant failures that occurred at a mean 4.9 ± 4.5 years postoperation. Ten polyethylene components in 9 TAAs (6.5%) required replacement for fracture at an average 8.9 ± 3.3 years postoperatively. Fourteen patients died with their initial implants in place. Conclusion: This cohort of patients with true intermediate follow-up after TAA with the STAR prosthesis had acceptable implant survival, maintenance of improved patient-reported outcome scores, and low major complication rates. Level of Evidence: Level IV, case series.

Early Outcomes and Complications of Synthetic Cartilage Implant for Treatment of Hallux Rigidus in the United States

2019 ◽  
Vol 40 (10) ◽  
pp. 1140-1148 ◽  
Author(s):  
Spenser J. Cassinelli ◽  
Stephanie Chen ◽  
Timothy P. Charlton ◽  
David B. Thordarson
Keyword(s):  
Patient Satisfaction ◽  
Case Series ◽  
Retrospective Chart Review ◽  
The United States ◽  
Reoperation Rate ◽  
Hallux Rigidus ◽  
Level Of Evidence ◽  
Physical Dysfunction ◽  
Magnetic Resonance Imaging Mri

Background: The aim of this study was to determine the early outcomes and complications following the implantation of a hydrogel synthetic cartilage implant (SCI, Cartiva) for the treatment of hallux rigidus by a single surgeon. Methods: A retrospective chart review was performed of consecutive patients who underwent treatment for hallux rigidus with an SCI between August 2016 and April 2018 by a single surgeon. Demographic information, radiographic assessment, and concomitant operative procedures performed were evaluated for all patients. Postoperatively, PROMIS physical function (PF) and pain interference (PI) scores, patient satisfaction, reoperation, conversion to arthrodesis, and need for further clinical treatment were collected. Sixty-four SCIs were implanted in 60 patients. Follow-up averaged 18.5 months (range 12-30 months). Results: 14% (9/64) of patients were very satisfied, 28% (18/64) satisfied, 20% (13/64) neutral, 11% (7/64) unsatisfied, and 27% (17/64) very unsatisfied with their outcome. In addition, 45% of patients underwent additional procedures at the time of SCI, and 23% had a history of surgery on the hallux before implantation. PROMIS PF scores averaged 42 and PROMIS PI scores averaged 60. Overall, 63% completed PROMIS PI, 66% completed PROMIS PF, and 100% completed a satisfaction survey. In addition, 30% (19/64) underwent magnetic resonance imaging (MRI) postoperatively due to pain. There was a 20% reoperation rate, which included an 8% rate of conversion to arthrodesis. Conclusion: Synthetic cartilage implantation yielded neutral patient satisfaction, mild pain, and physical dysfunction at early follow-up. We believe patient selection and counseling regarding early postoperative limitations are important before proceeding with SCI. Level of Evidence: Level IV, case series.

scholarly journals ACTH adenomas transforming their clinical expression: report of 5 cases

2015 ◽  
Vol 38 (2) ◽  
pp. E15 ◽  
Author(s):  
Matteo Zoli ◽  
Marco Faustini-Fustini ◽  
Diego Mazzatenta ◽  
Gianluca Marucci ◽  
Eugenio De Carlo ◽  
...  
Keyword(s):  
Pituitary Adenomas ◽  
Cushing Syndrome ◽  
Brain Mri ◽  
Gamma Knife Radiosurgery ◽  
Spontaneous Resolution ◽  
Ophthalmological Examination ◽  
Clinical Expression ◽  
Cell Adenoma ◽  
Cushing Disease

OBJECT Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon. METHODS The authors retrospectively reviewed a series of patients with pituitary adenoma and collected clinical, biohumoral, and neuroradiological data of those who presented with a transformation from silent ACTH adenomas to functioning tumors or vice versa. In all the cases, preoperative assessment consisted of brain MRI, ophthalmological examination, and complete baseline endocrinological investigation. In patients with clinical and/or biochemical findings suspicious for Cushing syndrome, a low-dose dexamethasone suppression test was performed to rule in or out this diagnosis. Endocrinological evaluations were repeated 1 month after surgery, 3 months after surgery, and every 6 months or annually thereafter. Ophthalmological evaluations and brain MRIs were repeated after 3 months and then every 6 or 12 months thereafter. RESULTS Five patients (2 men and 3 women) included in this series had corticotropic tumors that showed transformation from an endocrinologically silent form to manifest Cushing disease and vice versa. The mean age at presentation was 40 years (range 18–51 years). In 3 of these patients, a transformation from silent to functioning ACTH adenoma with manifest Cushing disease occurred. In 1 patient, the authors observed the transition from a functioning to a silent adenoma with spontaneous resolution of hypercortisolism. Another patient's silent adenoma “shifted” to a functioning adenoma and then regressed back to a silent form with spontaneous resolution of Cushing disease. This patient again developed hypercortisolism, which finally resolved spontaneously. In this series, the transformation occurred after a mean of 3.5 years (range 6 months to 7 years). The shift from an ACTH-silent to a functioning adenoma was observed in 9% of the ACTH-silent adenomas in this series (4 of 44 cases), and the spontaneous remission of Cushing disease to a silent corticotroph cell adenoma occurred in 1.5% of cases of this series (2 of 132 functioning ACTH adenomas). At follow-up (mean 107 months; range 60–177 months), cortisol levels were within normal limits in all 5 cases. However, 1 patient required Gamma Knife radiosurgery and eventually adrenalectomy for disease control to be achieved. CONCLUSIONS The ability of silent ACTH adenomas to transform their secretion pattern poses a challenge for neurosurgeons and endocrinologists. Because the transformation is often unexpected, the clinical and biochemical data can be underestimated. Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.

scholarly journals Evaluation of the Adequacy of Levothyroxine Replacement Therapy in Patients with Central Hypothyroidism1

1999 ◽  
Vol 84 (3) ◽  
pp. 924-929 ◽  
Author(s):  
Elisabetta Ferretti ◽  
Luca Persani ◽  
Marie-Lise Jaffrain-Rea ◽  
Salvatore Giambona ◽  
Guido Tamburrano ◽  
...  
Keyword(s):  
Thyroid Hormone ◽  
Replacement Therapy ◽  
Interleukin 2 ◽  
Hormone Action ◽  
Normal Range ◽  
Free T4 ◽  
Central Hypothyroidism ◽  
Thyroid Hormone Action ◽  
Daily Dose

As there are few data on the evaluation of the adequacy of levothyroxine (l-T4) therapy in patients with central hypothyroidism (CH), a prospective study was performed to assess the accuracy of various parameters in the follow-up of 37 CH patients. Total and free thyroid hormones, TSH, and a series of clinical and biochemical indexes of peripheral thyroid hormone action have been evaluated off and on l-T4 therapy. Samples were taken before the daily administration of l-T4. In all patients off therapy, clinical hypothyroidism and low levels of free T4 (FT4) were observed, whereas values of FT3, total T4, and total T3 were below the normal range in 73%, 57%, and 19% of cases, respectively. Most of the indexes of thyroid hormone action were significantly modified after l-T4 withdrawal and exhibited significant correlation with free thyroid hormone levels. During l-T4 replacement therapy, 32 patients had circulating levels of FT4 and FT3 and indexes within the normal range with a mean l-T4 daily dose of 1.5 ± 0.3 μg/kg BW. Despite normal serum FT4, 3 patients had borderline high values of FT3 and a clear elevation of serum-soluble interleukin-2 receptor concentrations, suggesting overtreatment. Low or borderline low FT4/FT3 levels indicated undertreatment in 2 patients. The clinical parameters lack the required specificity for the diagnosis or follow-up of CH patients. The l-T4 daily dose should be established, taking into account the weight, the age, and the presence of other hormone deficiencies or pharmacological treatment of CH patients. In conclusion, our results indicate that the diagnosis of CH is reached at best by measuring TSH and FT4 concentrations. In the evaluation of the adequacy of l-T4 replacement therapy, both FT4 and FT3 serum levels together with some biochemical indexes of thyroid hormone action are all necessary to a more accurate disclosure of over- or undertreated patients.

Pipeline Embolization Device for Treatment of Extracranial Internal Carotid Artery Pseudoaneurysms: A Multicenter Evaluation of Safety and Efficacy

Neurosurgery ◽  
2020 ◽  
Vol 87 (4) ◽  
pp. 770-778
Author(s):  
Oluwaseun O Akinduro ◽  
Neethu Gopal ◽  
Tasneem F Hasan ◽  
Emad Nourollah-Zadeh ◽  
Kunal Vakharia ◽  
...  
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Internal Carotid ◽  
Case Series ◽  
The United States ◽  
Pipeline Embolization Device ◽  
Safety And Efficacy ◽  
Complete Occlusion ◽  
Extracranial Ica

Abstract BACKGROUND There is a paucity of literature regarding treatment options for extracranial pseudoaneurysms of the internal carotid artery (ICA). To date, Pipeline Embolization Device (PED; Medtronic Inc) use for the treatment of extracranial pseudoaneurysms of the ICA has only been reported from single-center case series. OBJECTIVE To evaluate the safety and efficacy of PED for the treatment of extracranial ICA pseudoaneurysms. METHODS This is a multicenter retrospective study involving 6 high-volume tertiary academic institutions in the United States. We analyzed patients with extracranial ICA pseudoaneurysms treated with PED between January 1, 2011, and January 1, 2019. Clinical assessment was performed pre- and postintervention using the modified Rankin Scale (mRS) and National Institution of Health Stroke Scale (NIHSS) at a minimum of 4-mo follow-up. RESULTS A total of 28 pseudoaneurysms with a mean diameter of 17.7 mm (range: 4.1-52.5 mm) were treated with PED in 24 patients at 6 participating centers. The mean age was 52.1 yr (17-73) ± 14.3 with 14 females and 10 males. At a mean of 21-mo (range 4-66 mo) follow-up, complete occlusion was achieved in 89% (n = 25/28), with near-complete occlusion (&gt;90% occlusion) in the remainder. There were no periprocedural complications. Postprocedure NIHSS was 0 in 88% (n = 21/24) and 1 in 12% (n = 3/24) of patients, and mRS was 0 in 83% (n = 20/24) and 1 in 17% (n = 4/24) of patients. CONCLUSION The treatment of extracranial ICA pseudoaneurysms with PED is safe and effective in selected patients. Randomized controlled trials and prospective cohort studies are needed to establish the role of flow diversion for ICA pseudoaneurysms.

scholarly journals Persistent leukocytosis in polycythemia vera is associated with disease evolution but not thrombosis

Blood ◽  
2020 ◽  
Vol 135 (19) ◽  
pp. 1696-1703 ◽  
Author(s):  
Lukas Ronner ◽  
Nikolai Podoltsev ◽  
Jason Gotlib ◽  
Mark L. Heaney ◽  
Andrew T. Kuykendall ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Repeated Measures ◽  
Thrombotic Event ◽  
Laboratory Data ◽  
The United States ◽  
Disease Evolution ◽  
Repeated Measures Data ◽  
Retrospective Database ◽  
Relevant Covariates

Abstract There are unresolved questions regarding the association between persistent leukocytosis and risk of thrombosis and disease evolution in polycythemia vera (PV), as much of the published literature on the topic does not appropriately use repeated-measures data or time-dependent modeling to answer these questions. To address this knowledge gap, we analyzed a retrospective database of 520 PV patients seen at 10 academic institutions across the United States. Taking hematologic laboratory data at ∼3-month intervals (or as available) for all patients for duration of follow-up, we used group-based trajectory modeling to identify latent clusters of patients who follow distinct trajectories with regard to their leukocyte, hematocrit, and platelet counts over time. We then tested the association between trajectory membership and hazard of 2 major outcomes: thrombosis and disease evolution to myelofibrosis, myelodysplastic syndrome, or acute myeloid leukemia. Controlling for relevant covariates, we found that persistently elevated leukocyte trajectories were not associated with the hazard of a thrombotic event (P = .4163), but were significantly associated with increased hazard of disease evolution in an ascending stepwise manner (overall P = .0002). In addition, we found that neither hematocrit nor platelet count was significantly associated with the hazard of thrombosis or disease evolution.

The INFINITY Total Ankle Prosthesis: Outcomes at Short-Term Follow-up

2020 ◽  
pp. 193864002094619 ◽  
Author(s):  
Calvin J. Rushing ◽  
Kurt Kibbler ◽  
Christopher F. Hyer ◽  
Gregory C. Berlet
Keyword(s):  
Case Series ◽  
The United States ◽  
Fourth Generation ◽  
Low Grade ◽  
Single Institution ◽  
Short Term ◽  
Radiographic Outcomes ◽  
Early Revision ◽  
Grade 3

Background The INFINITY total ankle prothesis remains the most widely used fourth-generation total ankle arthroplasty (TAA) system in the United States. The purpose of the present study was to assess radiographic outcomes for INFINITY at short-term follow-up. Methods All patients who underwent primary TAA with INFINITY between August 2015 and June 2018 at a single institution and who were at least 1 year postoperative were included. A total of 55 ankles with a mean follow up 22 months (range, 12-43 months) met the criteria. Weightbearing radiographs were assessed using coronal and sagittal alignment parameters preoperatively, at 6 to 8 weeks postoperatively, and at the most recent follow-up. Medical records and charts were reviewed and revisions, reoperations, and complications were classified according to the criteria established by Vander Griend et al and Glazebrook at el, respectively. Results Overall, INFINITY survivorship was 98.2%. Coronal and sagittal tibiotalar alignment improved after surgery ( P < .001, P = .09, respectively), and was maintained during the latest follow-up ( P = .62, P = .47, respectively). Only 1 ankle (1.8%) required early revision; while 5 (9.1%) indicated non-implant-related revision; 85.5% required no reoperation. In nineteen ankles (34.5%), postoperative periprosthetic lucency was observed; most were not progressive (17 ankles, 89.5%). Twelve complications (3 high grade, 3 intermediate, and 6 low grade) in 11 ankles (20%) were recorded according to the Glazebrook classification system. Conclusion INFINITY survivorship was 98.2% at a mean of 1.8 years of follow-up, and only 1 ankle required prosthetic revision. Additional studies with longer follow-up are warranted for fourth-generation TAA prostheses. Levels of Evidence: Level IV: Case series

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