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[PMID]: 29441456
[Au] Autor:Vaddi VK; Sahu JK; Dhawan SR; Suthar R; Sankhyan N
[Ad] Address:Division of Pediatric Neurology, Department of Pediatrics, Postgraduate Institute of Medical Education & Research, Chandigarh, 160012, India.
[Ti] Title:Knowledge, Attitude and Practice (KAP) Study of Pediatricians on Infantile Spasms.
[So] Source:Indian J Pediatr;, 2018 Feb 14.
[Is] ISSN:0973-7693
[Cp] Country of publication:India
[La] Language:eng
[Ab] Abstract:OBJECTIVE: To investigate the knowledge, attitude, and practice of Infantile Spasms among pediatricians. METHODS: A survey was carried out among pediatricians serving in Punjab, Haryana, Chandigarh, Himachal Pradesh and Delhi. The survey was done by Survey Monkey Software through emails by using a structured questionnaire between July 2016 and December 2017. RESULTS: A total of 236 pediatricians responded to the survey. Most of the respondents (95.5%) correctly considered Infantile Spasms as a seizure type. The most preferred investigation was Electroencephalogram by 91.8% pediatricians; however, only 57.7% considered it to decide the treatment. Perinatal asphyxia was the most recognized etiology (60.7% pediatricians). For treatment of Infantile Spasms, 66.8% follow Nelson textbook of Pediatrics. Adrenocorticotropic hormone was the most preferred first choice drug by 40% pediatricians. Alternate anti-epileptic drug was considered by 60.9% pediatricians when there is no clinical response. Only 24% pediatricians considered treatment response as a complete cessation of spasms. Majority (90%) of pediatricians felt that there is a necessity for increased awareness and 62% pediatricians felt that available information was insufficient. CONCLUSIONS: A substantial number of pediatricians lack precise knowledge on evidence-based practice of Infantile Spasms. In developing countries, where pediatricians provide the initial management of Infantile Spasms, there is need to empower them and develop simplified national guidelines/consensus statement for management of Infantile Spasms.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180214
[Lr] Last revision date:180214
[St] Status:Publisher
[do] DOI:10.1007/s12098-018-2630-3

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[PMID]: 29313180
[Au] Autor:Daniel E; Debono M; Caunt S; Girio-Fragkoulakis C; Walters SJ; Akker SA; Grossman AB; Trainer PJ; Newell-Price J
[Ad] Address:Department of Oncology and Metabolism, The Medical School, University of Sheffield, Beech Hill Road, Sheffield, S10 2RX, UK.
[Ti] Title:A prospective longitudinal study of Pasireotide in Nelson's syndrome.
[So] Source:Pituitary;, 2018 Jan 08.
[Is] ISSN:1573-7403
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:PURPOSE: Nelson's syndrome is a challenging condition that can develop following bilateral adrenalectomy for Cushing's disease, with high circulating ACTH levels, pigmentation and an invasive pituitary tumor. There is no established medical therapy. The aim of the study was to assess the effects of pasireotide on plasma ACTH and tumor volume in Nelson's syndrome. METHODS: Open labeled multicenter longitudinal trial in three steps: (1) a placebo-controlled acute response test; (2) 1 month pasireotide 300-600 µg s.c. twice-daily; (3) 6 months pasireotide long-acting-release (LAR) 40-60 mg monthly. RESULTS: Seven patients had s.c. treatment and 5 proceeded to LAR treatment. There was a significant reduction in morning plasma ACTH during treatment (mean ± SD; 1823 ± 1286 ng/l vs. 888.0 ± 812.8 ng/l during the s.c. phase vs. 829.0 ± 1171 ng/l during the LAR phase, p < 0.0001). Analysis of ACTH levels using a random intercept linear mixed-random effects longitudinal model showed that ACTH (before the morning dose of glucocorticoids) declined significantly by 26.1 ng/l per week during the 28-week of treatment (95% CI - 45.2 to - 7.1, p < 0.01). An acute response to a test dose predicted outcome in 4/5 patients. Overall, there was no significant change in tumor volumes (1.4 ± 0.9 vs. 1.3 ± 1.0, p = 0.86). Four patients withdrew during the study. Hyperglycemia occurred in 6 patients. CONCLUSIONS: Pasireotide lowers plasma ACTH levels in patients with Nelson's syndrome. A longer period of treatment may be needed to assess the effects of pasireotide on tumor volume. TRIAL REGISTRATION: Clinical Trials.gov ID, NCT01617733.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180207
[Lr] Last revision date:180207
[Cl] Clinical Trial:ClinicalTrial
[St] Status:Publisher
[do] DOI:10.1007/s11102-017-0853-3

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[PMID]: 29316995
[Au] Autor:Stopyra JP; Harper WS; Higgins TJ; Prokesova JV; Winslow JE; Nelson RD; Alson RL; Davis CA; Russell GB; Miller CD; Mahler SA
[Ad] Address:1Wake Forest School of Medicine,Winston-Salem,North CarolinaUSA.
[Ti] Title:Prehospital Modified HEART Score Predictive of 30-Day Adverse Cardiac Events.
[So] Source:Prehosp Disaster Med;33(1):58-62, 2018 Feb.
[Is] ISSN:1945-1938
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Introduction The History, Electrocardiogram (ECG), Age, Risk Factors, and Troponin (HEART) score is a decision aid designed to risk stratify emergency department (ED) patients with acute chest pain. It has been validated for ED use, but it has yet to be evaluated in a prehospital setting. Hypothesis A prehospital modified HEART score can predict major adverse cardiac events (MACE) among undifferentiated chest pain patients transported to the ED. METHODS: A retrospective cohort study of patients with chest pain transported by two county-based Emergency Medical Service (EMS) agencies to a tertiary care center was conducted. Adults without ST-elevation myocardial infarction (STEMI) were included. Inter-facility transfers and those without a prehospital 12-lead ECG or an ED troponin measurement were excluded. Modified HEART scores were calculated by study investigators using a standardized data collection tool for each patient. All MACE (death, myocardial infarction [MI], or coronary revascularization) were determined by record review at 30 days. The sensitivity and negative predictive values (NPVs) for MACE at 30 days were calculated. RESULTS: Over the study period, 794 patients met inclusion criteria. A MACE at 30 days was present in 10.7% (85/794) of patients with 12 deaths (1.5%), 66 MIs (8.3%), and 12 coronary revascularizations without MI (1.5%). The modified HEART score identified 33.2% (264/794) of patients as low risk. Among low-risk patients, 1.9% (5/264) had MACE (two MIs and three revascularizations without MI). The sensitivity and NPV for 30-day MACE was 94.1% (95% CI, 86.8-98.1) and 98.1% (95% CI, 95.6-99.4), respectively. CONCLUSIONS: Prehospital modified HEART scores have a high NPV for MACE at 30 days. A study in which prehospital providers prospectively apply this decision aid is warranted. Stopyra JP , Harper WS , Higgins TJ , Prokesova JV , Winslow JE , Nelson RD , Alson RL , Davis CA , Russell GB , Miller CD , Mahler SA . Prehospital modified HEART score predictive of 30-day adverse cardiac events. Prehosp Disaster Med. 2018;33(1):58-62.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180206
[Lr] Last revision date:180206
[St] Status:In-Process
[do] DOI:10.1017/S1049023X17007154

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[PMID]: 29172980
[Au] Autor:Rice JB; White A; Lopez A; Nelson WW
[Ad] Address:1 Analysis Group, Boston, Massachusetts.
[Ti] Title:High-Cost Sarcoidosis Patients in the United States: Patient Characteristics and Patterns of Health Care Resource Utilization.
[So] Source:J Manag Care Spec Pharm;23(12):1261-1269, 2017 Dec.
[Is] ISSN:2376-1032
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Sarcoidosis is a multisystem inflammatory disorder characterized by the presence of noncaseating granulomas in involved organs. Prior research has found that sarcoidosis imposes a significant economic burden to U.S. payers. However, the drivers of high health care costs among sarcoidosis patients are unknown. OBJECTIVE: To characterize sarcoidosis patients who were among the top 20% of total health care costs. METHODS: Patients with a first diagnosis of sarcoidosis between January 1, 1998, and March 31, 2015 (index date) were selected from a deidentified privately insured administrative claims database. Study patients must have at least 12 months of continuous health plan enrollment prior to the index date. High-cost patients were those in the top 20% of total health care costs during the 12 months following the index date (follow-up period), and the remaining patients were classified as lower-cost patients. Patient characteristics, comorbidities, health care resource use, and health care costs in the study period were compared between the high-cost and lower-cost patients. Multiple logistic regression was used to assess the relationship between patient characteristics and being a high-cost sarcoidosis patient. RESULTS: A total of 7,173 sarcoidosis patients met the selection criteria. The 20% of patients classified as high-cost patients accounted for approximately 72% of the total health care costs in the 12-month follow-up period. Compared with lower-cost patients, high-cost patients were slightly older (50.6 vs. 49.1 years) and had a higher comorbidity burden at baseline (Charlson Comorbidity Index = 1.8 vs. 0.7). Mean annual total health care cost for high-cost sarcoidosis patients was 10 times that of their lower-cost counterparts ($73,345 vs. $7,073). Mean annual health care cost was $119,878 for patients in the 95th-99th percentile and $375,436 for patients in the top 1% of spend. High-cost patients had greater medical resource use and costs across all places of service (i.e., inpatient, emergency department, outpatient, and other) compared with lower-cost patients. Findings showed that higher total health care cost resulted in a larger proportion of inpatient spend and a smaller proportion of outpatient and pharmacy spend. Adjusting for baseline characteristics, high-cost patients were associated with a number of factors with high ORs: the presence of comorbidities such as deficiency anemia (OR = 1.606; P < 0.001), depression (OR = 1.504; P < 0.001), or cardiac arrhythmia (OR = 1.493; P < 0.001); having an inpatient admission (OR = 9.771; P < 0.001); and use of biologic therapies adalimumab and/or infliximab (OR = 31.821; P < 0.001). CONCLUSIONS: This study described the characteristics of high-cost sarcoidosis patients and identified several high-cost indicators using contemporary administrative data. The health care cost distribution for sarcoidosis patients is highly skewed, making it a worthwhile endeavor to focus improvement efforts on patients in the top quintile. The study findings can help population health decision makers identify a subset of patients for targeted interventions aimed at improving quality of care and reducing overall costs. DISCLOSURES: This study was funded by Mallinckrodt Pharmaceuticals. Rice, White, and Lopez are employees of Analysis Group, which received funding from Mallinckrodt Pharmaceuticals to conduct this research. Nelson is an employee of Mallinckrodt Pharmaceuticals. Study concept and design were contributed by Rice, White, and Nelson, along with Lopez. Lopez took the lead in data collection, with assistance from Rice and White. Data interpretation was performed by all of the authors. The manuscript was written by Rice, Lopez, White, and Nelson and revised by Rice, along with White and Nelson.
[Mh] MeSH terms primary: Cost of Illness
Health Care Costs/statistics & numerical data
Health Resources/utilization
Sarcoidosis/therapy
[Mh] MeSH terms secundary: Adalimumab/administration & dosage
Adalimumab/economics
Adult
Comorbidity
Databases, Factual
Female
Follow-Up Studies
Humans
Infliximab/administration & dosage
Infliximab/economics
Logistic Models
Male
Middle Aged
Retrospective Studies
Sarcoidosis/economics
United States
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:B72HH48FLU (Infliximab); FYS6T7F842 (Adalimumab)
[Em] Entry month:1802
[Cu] Class update date: 180201
[Lr] Last revision date:180201
[Js] Journal subset:IM
[Da] Date of entry for processing:171128
[St] Status:MEDLINE
[do] DOI:10.18553/jmcp.2017.17203

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[PMID]: 28982703
[Au] Autor:Pérez-Rivas LG; Theodoropoulou M; Puar TH; Fazel J; Stieg MR; Ferraù F; Assié G; Gadelha MR; Deutschbein T; Fragoso MC; Kusters B; Saeger W; Honegger J; Buchfelder M; Korbonits M; Bertherat J; Stalla GK; Hermus AR; Beuschlein F; Reincke M
[Ad] Address:Medizinische Klinik und Poliklinik IVLudwig-Maximilians-Universität München, Munich, Germany.
[Ti] Title:Somatic mutations are frequent events in corticotroph tumor progression causing Nelson's tumor.
[So] Source:Eur J Endocrinol;178(1):59-65, 2018 Jan.
[Is] ISSN:1479-683X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:OBJECTIVE: Somatic mutations in the ubiquitin-specific protease 8 ( ) gene are frequent in corticotroph tumors causing Cushing's disease (CD). Corticotroph tumor progression, the so-called Nelson's syndrome (NS), is a potentially life-threatening complication of bilateral adrenalectomy in patients with refractory CD that is caused by the development of an ACTH-secreting tumor of the pituitary gland. Whether alterations are also present in progressive Nelson's tumors has not been studied in detail so far. DESIGN AND METHODS: Retrospective, multicenter study involving tumors from 33 patients with progressive corticotroph tumors (29 females) and screening for somatic mutations on the mutational hotspot of the gene in the exon 14 with Sanger sequencing. RESULTS: Fifteen out of 33 tumors (45%) presented with a mutation in the exon 14 of , with c.2159C>A (p.Pro720Gln) being the most frequent (9/33), followed by c.2155_2157delTCC (p.Ser718del, 4/33) and c.2152T>C (p.Ser718Pro, 2/33). This prevalence is similar to that previously reported for CD. Mutations were found exclusively in females. Other variables, such as age at diagnosis with NS, body mass index, hyperpigmentation, visual field defects, adenoma size or mortality, did not significantly differ between patients with wild-type and mutant tumors. Patients with mutant tumors exhibited higher levels of plasma ACTH after surgery (median: 640 vs 112 pg/mL, = 0.03). No differences were observed in ACTH normalization (<50 pg/mL) and tumor control after surgery for Nelson's tumor. CONCLUSION: Somatic mutations in are common in Nelson's tumors, indicating that they do not drive the corticotroph tumor progression that leads to NS, and may be associated with a less favorable biochemical outcome after surgery for Nelson's tumor.
[Mh] MeSH terms primary: Carcinogenesis/genetics
Disease Progression
Endopeptidases/genetics
Endosomal Sorting Complexes Required for Transport/genetics
Mutation/genetics
Nelson Syndrome/genetics
Ubiquitin Thiolesterase/genetics
[Mh] MeSH terms secundary: Adrenocorticotropic Hormone/blood
Adult
Carcinogenesis/metabolism
Cohort Studies
Corticotrophs/physiology
Female
Humans
Male
Nelson Syndrome/blood
Nelson Syndrome/surgery
Retrospective Studies
Young Adult
[Pt] Publication type:JOURNAL ARTICLE; MULTICENTER STUDY
[Nm] Name of substance:0 (Endosomal Sorting Complexes Required for Transport); 9002-60-2 (Adrenocorticotropic Hormone); EC 3.4.- (Endopeptidases); EC 3.4.19.12 (USP8 protein, human); EC 3.4.19.12 (Ubiquitin Thiolesterase)
[Em] Entry month:1711
[Cu] Class update date: 171128
[Lr] Last revision date:171128
[Js] Journal subset:IM
[Da] Date of entry for processing:171007
[St] Status:MEDLINE
[do] DOI:10.1530/EJE-17-0634

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[PMID]: 28972425
[Au] Autor:Pryse-Phillips W
[Ad] Address:William Pryse-Phillips, Memorial University of Newfoundland, St. John's, Newfoundland, Canada.
[Ti] Title:Lord Nelson's (1758-1805) left eye.
[So] Source:J Med Biogr;:967772015624391, 2017 Jan 01.
[Is] ISSN:1758-1087
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Following the loss of his right eye at Calvi in 1794, Lord Nelson suffered increasing left-sided visual loss, here considered most likely to have been due to the ocular inflammatory condition 'sympathetic ophthalmia'. It is also argued that his succeeding episodes of violent headaches with nausea and prostration, and possible depigmentation of hair, reflected the development of an uveomeningoencephalitic syndrome akin to that of Vogt-Koyanagi-Harada disease, which is best regarded as the same condition with a different aetiology.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 171003
[Lr] Last revision date:171003
[St] Status:Publisher
[do] DOI:10.1177/0967772015624391

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[PMID]: 28950324
[Au] Autor:Caruso JP; Patibandla MR; Xu Z; Vance ML; Sheehan JP
[Ad] Address:Department of Neurological Surgery, University of Virginia Health System, Charlottesville, Virginia.
[Ti] Title:A Long-Term Study of the Treatment of Nelson's Syndrome With Gamma Knife Radiosurgery.
[So] Source:Neurosurgery;, 2017 Aug 05.
[Is] ISSN:1524-4040
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Nelson's syndrome may be a devastating complication for patients with Cushing's disease who underwent a bilateral adrenalectomy. Previous studies have demonstrated that stereotactic radiosurgery (SRS) can be used to treat patients with Nelson's syndrome. OBJECTIVE: To report a retrospective study of patients with Nelson's syndrome treated with Gamma Knife radiosurgery to evaluate the effect of SRS on endocrine remission and tumor control. METHODS: Twenty-seven patients with Nelson's syndrome treated with Gamma Knife radiosurgery after bilateral adrenalectomy were included in this study. After radiosurgery, patients were followed with serial adrenocorticotropic hormone (ACTH) levels and MRI sequences to assess for endocrine remission and tumor control. Cox proportional hazards regression analysis was used to evaluate the relationship between the time to remission and potential prognostic factors. RESULTS: In 21 patients with elevated ACTH prior to SRS and endocrine follow-up data, 14 (67%) had decreased or stable ACTH levels, and 7 achieved a normal ACTH level at a median of 115 mo (range 7-272) post-SRS. Tumor volume was stable or reduced after SRS in 92.5% of patients (25/27) with radiological follow-up. Time to remission was not significantly associated with the ACTH prior to SRS ( P  = .252) or with the margin dose ( P  = .3). However, a shorter duration between the patient's immediate prior transsphenoidal resection and SRS was significantly associated with a shorter time to remission ( P  = .045). CONCLUSION: This retrospective analysis suggests that SRS is an effective means of achieving endocrine remission and tumor control in patients with Nelson's syndrome.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1709
[Cu] Class update date: 170926
[Lr] Last revision date:170926
[St] Status:Publisher
[do] DOI:10.1093/neuros/nyx426

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[PMID]: 28589518
[Au] Autor:Catterall WA
[Ad] Address:Department of Pharmacology, University of Washington, Seattle, WA, 98195-7280, USA. wcatt@uw.edu.
[Ti] Title:Forty Years of Sodium Channels: Structure, Function, Pharmacology, and Epilepsy.
[So] Source:Neurochem Res;42(9):2495-2504, 2017 Sep.
[Is] ISSN:1573-6903
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Voltage-gated sodium channels initiate action potentials in brain neurons. In the 1970s, much was known about the function of sodium channels from measurements of ionic currents using the voltage clamp method, but there was no information about the sodium channel molecules themselves. As a postdoctoral fellow and staff scientist at the National Institutes of Health, I developed neurotoxins as molecular probes of sodium channels in cultured neuroblastoma cells. During those years, Bruce Ransom and I crossed paths as members of the laboratories of Marshall Nirenberg and Philip Nelson and shared insights about sodium channels in neuroblastoma cells from my work and electrical excitability and synaptic transmission in cultured spinal cord neurons from Bruce's pioneering electrophysiological studies. When I established my laboratory at the University of Washington in 1977, my colleagues and I used those neurotoxins to identify the protein subunits of sodium channels, purify them, and reconstitute their ion conductance activity in pure form. Subsequent studies identified the molecular basis for the main functions of sodium channels-voltage-dependent activation, rapid and selective ion conductance, and fast inactivation. Bruce Ransom and I re-connected in the 1990s, as ski buddies at the Winter Conference on Brain Research and as faculty colleagues at the University of Washington when Bruce became our founding Chair of Neurology and provided visionary leadership of that department. In the past decade my work on sodium channels has evolved into structural biology. Molecular modeling and X-ray crystallographic studies have given new views of sodium channel function at atomic resolution. Sodium channels are also the molecular targets for genetic diseases, including Dravet Syndrome, an intractable pediatric epilepsy disorder with major co-morbidities of cognitive deficit, autistic-like behaviors, and premature death that is caused by loss-of-function mutations in the brain sodium channel Na 1.1. Our work on a mouse genetic model of this disease has shown that its multi-faceted pathophysiology and co-morbidities derive from selective loss of electrical excitability and action potential firing in GABAergic inhibitory neurons, which disinhibits neural circuits throughout the brain and leads directly to the epilepsy, premature death and complex co-morbidities of this disease. It has been rewarding for me to use our developing knowledge of sodium channels to help understand the pathophysiology and to suggest potential therapeutic approaches for this devastating childhood disease.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1706
[Cu] Class update date: 171119
[Lr] Last revision date:171119
[St] Status:In-Process
[do] DOI:10.1007/s11064-017-2314-9

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[PMID]: 28332874
[Au] Autor:Espinosa-de-Los-Monteros AL; Sosa-Eroza E; Espinosa E; Mendoza V; Arreola R; Mercado M
[Ti] Title:LONG-TERM OUTCOME OF THE DIFFERENT TREATMENT ALTERNATIVES FOR RECURRENT AND PERSISTENT CUSHING DISEASE.
[So] Source:Endocr Pract;23(7):759-767, 2017 Jul.
[Is] ISSN:1530-891X
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OBJECTIVE: Treatment alternatives for persistent and recurrent Cushing disease (CD) include pituitary surgical re-intervention, radiation therapy (RT), pharmacotherapy, and bilateral adrenalectomy (BA). The decision of which of these alternatives is better suited for the individual patient rests on clinical judgment and the availability of resources. This retrospective cohort study was performed at a referral center to evaluate the long-term efficacy of different secondary interventions for persistent and recurrent CD. METHODS: We evaluated the hospital charts of 84 patients (77 female, median age 34 years, median follow up 6.3 years) with CD diagnosed, treated, and followed at our multidisciplinary clinic according to a pre-established protocol. RESULTS: Of the 81 patients who were initially treated with transsphenoidal surgery (TSS), 61.7% had a long-lasting remission, 16% had persistent disease, and 22% achieved remission but relapsed during follow-up. The most frequently used secondary treatment was pituitary re-intervention, followed by ketoconazole, RT, and BA. Early remissions were observed in 66.6% of the re-operated and in 58.3% of the radiated patients; long-lasting remission was achieved in 33.3% and 41.6% of these patients, respectively. Nelson syndrome developed in 41.6% of the patients who underwent BA. Upon last follow-up, 88% of all the patients are in remission, and 9.5% are biochemically controlled with ketoconazole. CONCLUSION: The efficacy of treatment alternatives for recurrent or persistent CD varies considerably among patients and multiple interventions are often required to achieve long-lasting remission. ABBREVIATIONS: ACTH = adrenocorticotrophic hormone; BA = bilateral adrenalectomy; CBG = cabergoline; CD = Cushing disease; CV = coefficient of variation; DXM = dexamethasone; IQR = interquartile range; RT = radiation therapy; SRS = stereotactic radiosurgery; TSS = transsphenoidal surgery; UFC = urinary free cortisol; ULN = upper limit of normal.
[Mh] MeSH terms primary: ACTH-Secreting Pituitary Adenoma/therapy
Adenoma/therapy
Adrenalectomy
Antifungal Agents/therapeutic use
Ketoconazole/therapeutic use
Neoplasm Recurrence, Local/therapy
Neurosurgical Procedures
Pituitary ACTH Hypersecretion/therapy
Radiotherapy
[Mh] MeSH terms secundary: ACTH-Secreting Pituitary Adenoma/pathology
Adenoma/pathology
Adolescent
Adult
Cohort Studies
Female
Humans
Male
Middle Aged
Nelson Syndrome
Remission Induction
Retreatment
Retrospective Studies
Tumor Burden
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Antifungal Agents); R9400W927I (Ketoconazole)
[Em] Entry month:1709
[Cu] Class update date: 170912
[Lr] Last revision date:170912
[Js] Journal subset:IM
[Da] Date of entry for processing:170324
[St] Status:MEDLINE
[do] DOI:10.4158/EP171756.OR

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[PMID]: 28084914
[Au] Autor:Graffeo CS; Perry A; Carlstrom LP; Meyer FB; Atkinson JLD; Erickson D; Nippoldt TB; Young WF; Pollock BE; Van Gompel JJ
[Ad] Address:Department of Neurologic Surgery, and.
[Ti] Title:Characterizing and predicting the Nelson-Salassa syndrome.
[So] Source:J Neurosurg;:1-11, 2017 Jan 13.
[Is] ISSN:1933-0693
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OBJECTIVE Nelson-Salassa syndrome (NSS) is a rare consequence of bilateral adrenalectomy (ADX) for refractory hypercortisolism due to Cushing disease (CD). Although classically defined by rapid growth of a large, invasive, adrenocorticotropin hormone (ACTH)-secreting pituitary tumor after bilateral ADX that causes cutaneous hyperpigmentation, visual disturbance, and high levels of ACTH, clinical experience suggests more variability. METHODS The authors conducted a retrospective chart review of all patients 18 years and older with a history of bilateral ADX for CD, adequate pituitary MRI, and at least 2 years of clinical follow-up. Statistical tests included Student's t-test, chi-square test, Fisher's exact test, multivariate analysis, and derived receiver operating characteristic curves. RESULTS Between 1956 and 2015, 302 patients underwent bilateral ADX for the treatment of hypercortisolism caused by CD; 88 had requisite imaging and follow-up (mean 16 years). Forty-seven patients (53%) had radiographic progression of pituitary disease and were diagnosed with NSS. Compared with patients who did not experience progression, those who developed NSS were significantly younger at the time of CD diagnosis (33 vs 44 years, p = 0.007) and at the time of bilateral ADX (35 vs 49 years, p = 0.007), had larger tumors at the time of CD diagnosis (6 mm vs 1 mm, p = 0.03), and were more likely to have undergone external-beam radiation therapy (EBRT, 43% vs 12%, p = 0.005). Among NSS patients, the mean tumor growth was 7 mm/yr (SE 6 mm/yr); the median tumor growth was 3 mm/yr. Prevalence of pathognomonic symptoms was low; the classic triad occurred in 9%, while hyperpigmentation without visual field deficit was observed in 23%, and 68% remained asymptomatic despite radiographic disease progression. NSS required treatment in 14 patients (30%). CONCLUSIONS NSS is a prevalent sequela of CD after bilateral ADX and affects more than 50% of patients. However, although radiological evidence of NSS is common, it is most often clinically indolent, with only a small minority of patients developing the more aggressive disease phenotype characterized by clinically meaningful symptoms and indications for treatment. Young age at the time of CD diagnosis or treatment with bilateral ADX, large tumor size at CD diagnosis, and EBRT are associated with progression to NSS and may be markers of aggressiveness.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1701
[Cu] Class update date: 170926
[Lr] Last revision date:170926
[St] Status:Publisher
[do] DOI:10.3171/2016.9.JNS161163


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