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A history of smoking, higher levels of systolic blood pressure, lower levels of high‐density lipoprotein cholesterol, and the presence of peripheral arterial diseases are associated with an increased risk of subclavian artery stenosis.
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Survival:
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Catatonia (DynaMed) is:
The Bottom Line: There is no significant increased risk of bleeding in patients with amyloidosis. Renal biopsy can be done safely on patients with amyloidosis who do not have a hemostatic disorder or uncontrolled hypertension (Soares et al, 2008).
References: Soares SM, Fervenza FC, Lager DJ, Gertz MA, et al. Bleeding complications after transcutaneous kidney biopsy in patients with systemic amyloidosis: single-center experience in 101 patients. Am J Kidney Dis. 2008 Dec;52(6):1079-83. Doi:10.1053/j.ajkd.2008.05.022.
Tondel C, Vikse BE, Bostad L, Svarstad E. Safety and complications of percutaneous kidney biopsies in 715 children and 8573 adults in Norway 1988-2010. Clin J Am Soc Nephrol. 2012 Oct;7(10):1591-7. Doi:10.2215/CJN.02150212.
Summary: A study at the Mayo Clinic (Soares et al, 2008) found the incidence of bleeding in patients with amyloidosis (9.9%) similar to the occurrence in patients without amyloidosis (10.6%).
Tondel et al’s 2012 research out of Norway supports this finding. Their study of 9288 renal biopsies (715 children, 8573 adults) found that a diagnosis of amyloidosis did not carry an increased risk for major complications, including blood transfusions, surgery, or angiographic embolizations (Tondel et al, 2012). They conclude that a renal biopsy should be considered a safe procedure for all ages.
The Bottom Line: Prognosis for AL amyloidosis is generally poor, especially if left untreated. It is progressive or fatal within 2 years in 80% of patients. Only 5-10% survive more than 10 years after diagnosis (DynaMed Plus, 2017).
References: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 114350, Amyloid light chain (AL) amyloidosis; [updated 2017 Aug 10, cited 2018 Sep 24]; [about 24 screens]. Emory login required.
Gillmore JD, Wechalekar A, Bird J, Cavenagh J, et al. Guidelines on the diagnosis and investigation of AL amyloidosis. Br J Haematol. 2015 Jan;168(2):207-18. Doi:10.1111/bjh.13156.
Summary: The prognosis according to stages of disease are:
(Gillmore et al, 2015)
For additional information on characteristics associated with poor or better prognosis, see page 216 of the “Guidelines on the diagnosis and investigation of AL amyloidosis.”
The Bottom Line: CJD is a rare neurodegenerative condition with a rapid disease course and 100% mortality rate. The most challenging aspect of CJD is its diagnosis (Manix et al, 2015). According to DynaMed Plus (2015), the most reliable diagnostic testing are:
References: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 114980, Creutzfeldt-Jakob disease; [updated 2015 May 01, cited 2018 Aug 13]; [about 17 screens]. Emory login required.
Manix M, Kalakoti P, Henry M, Thakur J, et al. Creutzfeldt-Jackob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy. Neurosurg Focus. 2015 Nov;39(5):E2. Doi:10.3171/2015.8.FOCUS15328.
The Bottom Line: Tamoxifen appears to exert its effect through inhibition and modulation of TGF-β. In vitro and animal models showed that Tamoxifen treatment blocked EMT induced by TGF-β, preserved the fibrinolytic activity and reduced the migration capacity of mesothelial cells leading to reduced fibrosis and reduced PD effluent levels of VEGF leading to reduced angiogenesis in the peritoneum. However, Tamoxifen has almost always been used in combination with corticosteroids, therefore the efficacy and safety of Tamoxifen alone in the treatment of EPS still remains to be evaluated.
Reference: Moinuddin Z, Summers A, Van Dellen D, Augustine T, et al. Encapsulating peritoneal sclerosis – a rare but devastating peritoneal disease. Frontiers in Physiology. 2014;5:470. Doi:10.3389/fphysi.2014.00470
Summary: Encapsulating peritoneal sclerosis (EPS) is a devastating but rare complication of long-term peritoneal dialysis. The disease is associated with extensive thickening and fibrosis of the peritoneum resulting in the formation of a fibrous cocoon encapsulating the bowel leading to intestinal obstruction. The incidence of EPS ranges between 0.7 and 3.3% and increases with duration of peritoneal dialysis therapy.
Tamoxifen is a Selective Estrogen Receptor Modulator (SERM) with antifibrotic properties and has been used in the treatment of various fibrotic disorders like retroperitoenal fibrosis, fibrosing mediastinitis, fibrosing cerivicitis, and desmoid tumors
A large retrospective Dutch study demonstrated significantly reduced mortality in EPS patients that were treated with Tamoxifen (45.8%) when compared to those that were not treated with Tamoxifen (74.4%).The potential side-effects of Tamoxifen (deep vein thrombosis, endometrial cancer, and calciphylaxis) also need to be considered.
The Bottom Line: Most commonly diagnosed in children, hand, foot, mouth disease (HFMD) infects approximately 11% of exposed adults, and less than 1% of those infected experience clinical manifestations of the disease (Ramirez-Fort et al, 2014). Most cases resolve on their own, yet supportive treatment can alleviate fever, pain and inflammation (DyanMed Plus, 2018).
References: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 116931, Hand-foot-and-mouth disease; [updated 2018 Feb 22, cited 2018 Jun 19]; [about 10 screens]. Available from http://www.dynamed.com/login.aspx?direct=true&site=DynaMed&id=116931. Emory login required.
Ramirez-Fort MK, Downing C, Hung Q, et al. Coxsackievirus A6 associated hand, foot and mouth disease in adults: Clinical presentation and review of the literature. J Clin Virol. 2014 Aug;60(4):381-6. Doi:10.1016/j.jcv.2014.04.023
Summary: Complications are rare but can include the following (DynaMed Plus, 2018):
The Bottom Line: There is an overall survival rate of less than 5 years.
Reference: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 114774, Non-small cell lung cancer; [updated 2018 May 01, cited 15 May 2018]; [about 31 screens]. Emory access required.
Summary: Five-year survival is only 5-8% in patients with unresectable NSCL cancer. Patients with untreated T1 tumors have a survival rate of 9%. Five-year mortality is higher in men than in women.
5-year survival for patients with lung and bronchus cancer by stage at diagnosis:
A study considering intervention and age found that older age and less intervention is associated with poorer survival:
Additional Information: DynaMed Plus provides breakdowns by stage, age, and other factors.
For information on the staging of lung cancer, see the IASLC Lung Cancer Staging Project.
The Bottom Line: Horner syndrome results from an interruption of sympathetic innervation to the eye. The classic triad is unilateral ptosis (slight narrowing of the ocular fissure), miosis (smaller pupil on the affected side), and anhidrosis (lack of perspiration on the forehead or face). Horner syndrome is not likely to cause any functional visual disturbance but is of great importance clinically as a “red flag” warning that the oculosympathetic pathway has been interrupted and thus there may be potentially life-threatening lesions in the head, neck, and chest.
Reference: Martin TJ. Horner syndrome: a clinical review. ACS Chem Neuroscience. 2018 Feb 21;9(2):177-186. Doi:10.1021/acschemneuro.7b00405
Summary: The first, and perhaps most important, aspect of evaluating suspected Horner syndrome is a careful history. The history often reveals an obvious cause, such as trauma or neck/chest surgery, or will show that the Horner syndrome had been present for many years and therefore may not require an extensive investigation. The history may also reveal other symptoms that would help localize the lesion causing the Horner syndrome. A careful examination is obviously also critical, as other causes of anisocoria or ptosis may need to be considered rather than Horner syndrome, and concomitant signs (for example, a sixth nerve paresis) may help localize a potential lesion. After this, pharmacologic testing can be helpful to confirm diagnosis. Many clinicians will elect not to evaluate Horner syndrome if it has been present for greater than two years.
Though often benign or idiopathic, the cause of Horner syndrome can be very threatening or even lethal, so understanding how to recognize, diagnose, and appropriately evaluate Horner syndrome is important to all clinicians.
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