Presentation
Starring Clare Wren (TV's The Young Riders), Bruce Davison (Longtime Companion), along with genre film stars Stacy Haiduk (Luther the Geek) and David Naughton (An American Werewolf in London), Vinegar Syndrome is proud to present the Blu-ray debut of [vinegarsyndrome.com]
[…] this one was handwritten (cos i am currently suffering from rsi of the left wrist argh) and then laboriously typed out as a present for our tumblr bros, because fuckyeahweskerxchris reached 10 followers today! [fanfiction.net]
We discuss a case of a 35 year old male who presented to us with upper limb ischaemia. [jpma.org.pk]
Acronym STLS Synonyms Dislocated hips and radial heads, carpal coalition, scoliosis, and short stature Keywords Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. [uniprot.org]
Entire Body System
- Short Stature
Steel syndrome is a rare disorder of the skeleton characterized by facial dysmorphism, short stature, carpal coalition, dislocated radial heads, bilateral hip dislocation and vertical talus. [ncbi.nlm.nih.gov]
stature-scoliosis-carpal coalitions-pes cavus-facial dysmorphism syndrome steel syndrome has related synonym dislocated hips and radial heads, carpal coalition, scoliosis, and short stature STLS Term relations [ebi.ac.uk]
Definition A syndrome characterized by dislocated hips and radial heads, fusion of carpal bones, short stature, scoliosis, and cervical spine anomalies. [uniprot.org]
- Pain
Carpal tunnel syndrome causes weakness, tingling, pain, and numbness in your hand and arm. [steelcityspine.com]
He presented with new onset pain and numbness of the left hand of 3-week duration. The pain and numbness was limited to the medial three fingers and medial aspect of hand. Examination revealed an unremarkable well-developed upper arm AVF. [ukidney.com]
Medications Nonsteroidal anti-inflammatory medications ease the pain and reduce swelling. [boneandspine.com]
Clinical appearances vary in severity and include (a) from upper limb side - weakness, thirst, cold, decreased blood pressure of the respective arm, pain and (b) neurological - increase in the physical load of the respective hand: dizziness, syncope, [inis.iaea.org]
- Weakness
Carpal tunnel syndrome causes weakness, tingling, pain, and numbness in your hand and arm. [steelcityspine.com]
Clinical appearances vary in severity and include (a) from upper limb side - weakness, thirst, cold, decreased blood pressure of the respective arm, pain and (b) neurological - increase in the physical load of the respective hand: dizziness, syncope, [inis.iaea.org]
A physical examination was notable for blood pressures of 150/70 mm Hg in the right arm and 80/60 mm Hg in the left arm, with weak brachial and radial pulses on the left side. [nejm.org]
It is commoner on the left side with L:R ratio of ~3:1. ipsilateral upper limb weak or absent pulse decreased systolic blood pressure in the affected side; systolic blood pressure difference between the brachial arteries are more than 20 mmHg 3 arm claudication [radiopaedia.org]
Symptoms include Spasm of vessels of hand leading to pallor in one or more of fingers Thrombosis or embolism of the arteries leading to weak or no pulse in the affected arm, cold fingers, hands or arms Weakness of arm or neck Aneurysm Muscle atrophy, [boneandspine.com]
- Surgical Procedure
A surgical procedure that may be useful when there are swallowing disturbances is a gastrostomy to permit feeding directly into the stomach. [medicinenet.com]
Blalock Taussig shunt is a surgical procedure indicated to increase the blood flow to the lungs. The procedure involves placing a tube between the subclavian artery and the lung artery. [healthool.com]
Performed via advanced endoscopic technology, compared to traditional surgery, the procedure: Decreases unavoidable surgical trauma to the surrounding tissue Reduces risk of post-surgical infection Speeds healing and recovery time Most individuals who [steelcityspine.com]
A patient with kidney failure that requires haemodialysis has a surgical procedure to connect a large artery and vein, forming an arteriovenous fistula. [dermnetnz.org]
- Arm Pain
Clinical appearances vary in severity and include (a) from upper limb side - weakness, thirst, cold, decreased blood pressure of the respective arm, pain and (b) neurological - increase in the physical load of the respective hand: dizziness, syncope, [inis.iaea.org]
If the patient has a reproduction of arm pain or numbness, consider thoracic outlet syndrome. Radial artery palpation can also diminish in addition to this. [boneandspine.com]
[…] intermittent claudication of the arm. 8 Arm symptoms include ischaemic pain at rest, ulcers, muscle fatigue, and digital necrosis. [jpma.org.pk]
In the minority of patients who manifest symptoms related to subclavian stenosis, arm claudication is the most common complaint, consisting of exercise-induced arm pain or fatigue. [circ.ahajournals.org]
Cardiovascular
- Hypertension
In cases of venous hypertension or arterial steal, revision and banding using grafted sheets at the anastomosis site, were done to reduce AV overflow. [nmckk.jp]
Aithal, D.M., and Matthias Ulrich, M.D. 1 Citing Article A 68-year-old man with hypertension, dyslipidemia, and diabetes mellitus presented with dizziness, presyncope, and blurring of vision that occurred when he used his left hand while gardening. [nejm.org]
Risk factors include hypertension, diabetes, hypercholesterolemia, tobacco use and vigorous exercise of the affected extremity. [aafp.org]
We report a 56-year-old male with history of diabetes,hypertension and end stage renal disease (ESRD) receiving hemodialysis via a 3-year old mature left upper arm brachiocephalic arteriovenous fistula (AVF). [ukidney.com]
It is critically important to employ all the risk reduction measures known to slow the progression of atherosclerosis, including smoking cessation, blood lipid management, control of hypertension, weight management, exercise, and control of diabetes. [verywell.com]
Musculoskeletal
- Hip Dislocation
Steel syndrome is a rare disorder of the skeleton characterized by facial dysmorphism, short stature, carpal coalition, dislocated radial heads, bilateral hip dislocation and vertical talus. [ncbi.nlm.nih.gov]
[…] radial heads, bilateral hip dislocation and vertical talus. [manipal.pure.elsevier.com]
A rare genetic bone disease characterized by short stature, bilateral congenital hip dislocation, radial head dislocation, carpal coalition, scoliosis, pes cavus, and atlantoaxial subluxation. [ebi.ac.uk]
- Arm Weakness
Symptoms include Spasm of vessels of hand leading to pallor in one or more of fingers Thrombosis or embolism of the arteries leading to weak or no pulse in the affected arm, cold fingers, hands or arms Weakness of arm or neck Aneurysm Muscle atrophy, [boneandspine.com]
Neurologic
- Ataxia
Clinical appearances vary in severity and include (a) from upper limb side - weakness, thirst, cold, decreased blood pressure of the respective arm, pain and (b) neurological - increase in the physical load of the respective hand: dizziness, syncope, ataxia [inis.iaea.org]
Vertebrobasilar insufficiency may produce light-headedness, dizziness, ataxia, vertigo, visual disturbances, motor deficits, confusion, focal seizures, aphasia, headaches or syncope. [aafp.org]
[…] systolic blood pressure in the affected side; systolic blood pressure difference between the brachial arteries are more than 20 mmHg 3 arm claudication (rare due to collateral perfusion) neurological (exacerbated by arm exercise) dizziness/vertigo/syncope ataxia [radiopaedia.org]
Transient periods of ataxia, diplopia, dysphagia and dysarthria. Tingling or numbness of the face, sensory hemianaesthesia affecting the body or transient hemiparesis. [patient.info]
[…] carotid artery ischaemia; hemianaesthesia, hemi/monoparesis, head ache, dysphagia, visual field disturbance and confusion, and of vertebrobasilar ischaemia; dizziness/vertigo/light headedness, drop attacks/fainting spells, diplopia, dysarthria, dysphagia, ataxia [jpma.org.pk]
- Limb Weakness
It is commoner on the left side with L:R ratio of ~3:1. ipsilateral upper limb weak or absent pulse decreased systolic blood pressure in the affected side; systolic blood pressure difference between the brachial arteries are more than 20 mmHg 3 arm claudication [radiopaedia.org]
Workup
Workup for previous stroke revealed normal protein C and S and antithrombin iii levels but high homocysteine levels in the blood. Patient was taken to the OR and via left postero-lateral thoracotomy, the subclavian artery was exposed. [jpma.org.pk]
Treatment
CONTRIBUTING FACTORS ASSESSMENT FINDINGS CONTRIBUTING FACTORS ASSESSMENT FINDINGS CONTRIBUTING FACTORS NURSING DIAGNOSES Brush up on key concepts ASSESSMENT FINDINGS TREATMENT CONTRIBUTING FACTORS DIAGNOSTIC TEST RESULTS TREATMENT ASSESSMENT FINDINGS [books.google.de]
Endovascular treatment with its safety, efficacy, and relatively low cost has been proven to be an alternative to the treatment of stenocclusive disease in subclavian arteries. [inis.iaea.org]
The syndrome was described as a unique entity with dismal results after conventional treatment of dislocated hips. [ncbi.nlm.nih.gov]
There is currently no effective treatment for PSP. [medicinenet.com]
Effective treatments are available that can help many, and our understanding of the psychology, neurobiology, and clinical treatment of the disorder has advanced dramatically over the past 25 years. [books.google.es]
Prognosis
Since this kind of syndrome occurs very rare, the prognosis has not been clearly characterized. [syndromespedia.com]
Prognosis Patients eventually become bed bound; death in 5–10 years, often from upper respiratory tract infection. Segen's Medical Dictionary. © 2012 Farlex, Inc. [medical-dictionary.thefreedictionary.com]
[…] endovascular intervention subclavian artery stenosis or occlusion easily identified delayed filling of ipsilateral vertebral artery (which fills retrogradely) other intracranial or extracranial cerebral vascular lesions can also be identified Treatment and prognosis [radiopaedia.org]
Progressive supranuclear palsy: Management and prognosis. https://uptodate.com/contents/search. Accessed Jan. 19, 2022. Whitwell JL, et al. [18F]AV-1451 Tau positron emission tomography in progressive supranuclear palsy. [mayoclinic.org]
Prognosis Symptoms may spontaneously resolve due to the establishment of extracranial collaterals to the subclavian circulation. Treatment is therefore usually reserved for patients with debilitating vertebrobasilar transient ischaemic attacks. [patient.info]
Etiology
The application of whole-exome sequencing to disorders apparently segregating as Mendelian traits has proven to be an effective approach to disease gene identification for conditions with unknown molecular etiology. [uthealth.influuent.utsystem.edu]
PREVENTING VASS Prevention strategies for VASS rely predominantly on an understanding of the various etiologies. [evtoday.com]
Etiology atherosclerosis : ~95% vasculitides, e.g. [radiopaedia.org]
"Both disorders clearly have a complex underlying genetic architecture, and these two studies lay the foundation for understanding the underlying genetic etiology of Tourette syndrome and OCD," said Jeremiah Scharf, a neurologist at Massachusetts General [livescience.com]
Epidemiology
Relevant External Links for COL27A1 Genetic Association Database (GAD) COL27A1 Human Genome Epidemiology (HuGE) Navigator COL27A1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: COL27A1 No data available for Genatlas for COL27A1 Gene Identification [genecards.org]
Epidemiology Prevalence This is a relatively uncommon disorder. Its incidence has been reported as between 0.1% and 3.4% of adults. [ 1 ] SSP is seen as an incidental finding on sonography of the vertebral system in about 2-5% of those examined. [patient.info]
Logistic regression with case-control imbalance results in serious inflation of test statistics, particularly in the case of rare variants (see Ma et al., 2013 Genetic Epidemiology). [elifesciences.org]
Epidemiology Although peripheral arterial disease affects about 20-25% of Americans older than 70 years, the vessels of the upper extremity are affected much less often than those of the lower extremity are. [emedicine.medscape.com]
The epidemiology of subclavian stenosis and its association with markers of subclinical atherosclerosis: the Multi-Ethnic Study of Atherosclerosis (MESA). Atherosclerosis. 2010 ; 211 :266–270. Crossref Medline Google Scholar 6. [circ.ahajournals.org]
Pathophysiology
These symptoms are frequently associated with proximal arteriovenous accesses and are often pronounced during hemodialysis treatment.Distal extremity ischemia secondary to steal phenomenon is often considered as the underlying pathophysiologic mechanism [ukidney.com]
Pathophysiology The pathophysiologic mechanisms that govern DHIS are complex and poorly understood. [cjasn.asnjournals.org]
Thereby retrograde diastolic inflow into the fistula—the pathophysiologic principle of steal—is stopped. [academic.oup.com]
Our relatively poor understanding of the pathophysiology of VASS has resulted in a diverse menu of treatment options depending on the etiology of the "steal." This article reviews the current understanding of the causes and prevention of VASS. [evtoday.com]
Pathophysiology The upper limb is supplied primarily via the axillary artery, the continuation of the subclavian artery that exits the thoracic outlet. [emedicine.medscape.com]
Prevention
In the case of DVT and PE, ancestry awareness not race-based prescribing can prevent death. [anthrogenica.com]
PREVENTING VASS Prevention strategies for VASS rely predominantly on an understanding of the various etiologies. [evtoday.com]
Prevention PSP cannot be prevented. Resources Organizations American Academy of Neurology. 1080 Montreal Ave., St. Paul, MN 55116. (612) 695-1940. http://www.aan.com. Society for Progressive Supranuclear Palsy, Inc. [medical-dictionary.thefreedictionary.com]
Prevention For prevention of the Subclavian steal syndrome, it is advised that you do the following: Ancillary evaluation for peripheral or coronary artery disease Risk management for cardiovascular disease Undergoing regular re-evaluation for the unsuccessful [syndromespedia.com]
When an AV aneurysm is associated with infection, thrombo-embolism or taut skin, emergent surgery should be done, not only to prevent rupture or hemorrhage, but also to preserve blood access for further hemodialysis. [nmckk.jp]