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Management often includes the use of beta blockers such as propranolol or if not tolerated calcium channel blockers or ACE inhibitors.
Since angiotensin II receptor antagonists (ARBs) also reduce TGF-β, these drugs have been tested in a small sample of young, severely affected people with Marfan syndrome. In some, the growth of the aorta was reduced. However, a recent study published in NEJM demonstrated similar cardiac outcomes between the ARB, losartan, and the more established beta blocker therapy, atenolol.
There is no cure for Marfan syndrome, but life expectancy has increased significantly over the last few decades and is now similar to that of the average person. Regular checkups by a cardiologist are needed to monitor the health of the heart valves and the aorta. The syndrome is treated by addressing each issue as it arises and, in particular, preventive medication even for young children to slow progression of aortic dilation. The goal of treatment is to slow the progression of aortic dilation and damage to heart valves by eliminating arrythmias, minimizing the heart rate, and minimizing blood pressure.
Treatment with lamotrigine has been reported.
Treatment by acupuncture has been reported.
The clinician must protect the patient against hypotension, renal failure, acidosis, hyperkalemia and hypocalcemia. Admission to an intensive care unit, preferably one experienced in trauma medicine, may be appropriate; even well-seeming patients need observation. Treat open wounds as surgically appropriate, with debridement, antibiotics and tetanus toxoid; apply ice to injured areas.
Intravenous hydration of up to 1.5 L/hour should continue to prevent hypotension. A urinary output of at least 300 ml/hour should be maintained with IV fluids and mannitol, and hemodialysis considered if this amount of diuresis is not achieved. Use intravenous sodium bicarbonate to keep the urine pH at 6.5 or greater, to prevent myoglobin and uric acid deposition in kidneys.
To prevent hyperkalemia/hypocalcemia, consider the following adult doses:
- calcium gluconate 10% 10ml or calcium chloride 10% 5 ml IV over 2 minutes
- sodium bicarbonate 1 meq/kg IV slow push
- regular insulin 5–10 U
- 50% glucose 1–2 ampules IV bolus
- kayexalate 25–50 g with sorbitol 20% 100 ml by mouth or rectum.
Even so, cardiac arrhythmias may develop; electrocardiographic monitoring is advised, and specific treatment begun promptly.
Brachioradial pruritus (BRP) is a localized pruritus of the dorsolateral aspect of the arm. BRP is an enigmatic condition with a controversial cause; some authors consider BRP to be a photodermatosis, whereas other authors attribute BRP to compression of cervical nerve roots.
BRP may be attributed to a neuropathy, such as chronic cervical radiculopathy. The possibility of an underlying neuropathy should be considered in the evaluation and treatment of all patients with BRP.
The main cause of BRP is not known, but there is evidence to suggest that BRP may arise in the nervous system. Cervical spine disease may be an important contributing factor.
Patients with BRP may have underlying cervical spine pathology. Whether this association is causal or coincidental remains to be determined.
There is controversy regarding the cause of brachioradial pruritus: is it caused by a nerve compression in the cervical spine or is it caused by a prolonged exposure to sunlight?
In many patients, itching of the arms or shoulders is seasonal. Some patients reported neck pain.
BRP can be linked to the thyroid.
Atrophia Maculosa Varioliformis Cutis (AMVC) is a condition involving spontaneous scarring, specifically depressed scars on the face occurring over a period of months to years. It appears to only affect children and young adults, is considered to be quite rare, normally occurs on the cheeks, temple area and forehead, and is not well understood nor presently treatable. Case reports indicate the scars deepen over time but remain relatively superficial, and with the frequency of new scar appearance diminishing over time.
AMVC is quite difficult to diagnose, for reasons including the depressed box and ice pick scars being very similar to that caused by Acne vulgaris. A confident diagnosis can be made if such scars recently appeared without present acne and without a history of acne. Otherwise the correct diagnosis is usually not made, and even doing so provides little benefit as there is no treatment. It has been suggested in case reports that the condition, although rare, is likely underreported.
The disease is chronic and often progresses slowly. Prognosis is generally poor when associated with glaucoma [1,2].
Due to the risk of crush syndrome, current recommendation to lay first-aiders (in the UK) is to not release victims of crush injury who have been trapped for more than 15 minutes. Treatment consists of not releasing the tourniquet and fluid overloading the patient with added Dextran 4000 iu and slow release of pressure. If pressure is released during first aid then fluid is restricted and an input-output chart for the patient is maintained, and proteins are decreased in the diet.
The Australian Resuscitation Council recommended in March 2001 that first-aiders in Australia, where safe to do so, release the crushing pressure as soon as possible, avoid using a tourniquet and continually monitor the vital signs of the patient. St John Ambulance Australia First Responders are trained in the same manner.
In terms of the prognosis of ulnar neuropathy early decompression of the nerve sees a return to normal ability (function). which should be immediate.Severe cubital tunnel syndrome tends to have a faster recovery process in individuals below the age of 70, as opposed to those above such an age. Finally, revisional surgery for cubital tunnel syndrome does not result well for those individuals over 50 years of age.
Penetrating karatoplasty and endothelial keratoplasty can be used as treatments for severe cases of ICE [2,8]. Because glaucoma and elevated intraocular pressure are often present in ICE patients, long term follow up may be needed to ensure adequate intraocular pressures are maintained [2,7]
The primary treatment strategy is to eliminate or discontinue the offensive agent. Supportive therapy, such as ice packs, may be provided to get the body temperature within physiologic range. In severe cases, when the fever is high enough (generally at or above ~104° F or 40° C), aggressive cooling such as an ice bath and pharmacologic therapy such as benzodiazepines may be deemed appropriate.
Ophthalmodynia periodica is also referred to as "ice-pick headache", "needle-in-the-eye syndrome", and "sharp short-lived head pain." Ophthalmodynia periodica is considered a primary headache disorder, so it is not caused by any other conditions. Another well-known name for ophthalmodynia periodica is "jabs and jolts syndrome."
Ophthalmodynia periodica does not have a confirmed cause, being a primary headache, but can be identified with other primary conditions. "As many as 40% of all individuals with ice pick headaches have also been diagnosed as suffering with some form of migraine headache."
Immediate treatment consists of rinsing the bite site in cold water. If not too painful, ice the bite site. This constricts the blood vessels so the venom does not spread. Also recommended is papain, an enzyme that breaks down protein. Papain can be found in meat tenderizer and papaya. This deactivates the majority of the centipede venom's proteins. Depending on the type of centipede and level of envenomation, this treatment may not degrade the entire venom dose and residual pain will remain.
Individuals who are bitten by centipedes are sometimes given a urine test to check for muscle tissue breakdown and/or an EKG to check for heart and vascular problems.
Reassurance and pain relief is often given in the form of painkillers, such as non-steroidal anti-inflammatory medications, antihistamines and anti-anxiety medications. In a severe case the affected limb can be elevated and administered diuretic medications.
Wound care principles and sometimes antibiotics are used to keep the wound itself from becoming infected or necrotic.
In Barbados, a folk remedy involves applying a freshly cut onion to the site of the injury "bite" for 10 minutes. Repeat until relief is obtained.
The first-line therapy in ColdU, as recommended by EAACI/GA2 LEN/EDF/WAO guidelines, is symptomatic relief with second-generation H1- antihistamines. if standard doses are ineffective increasing up to 4-fold is recommended to control symptoms.
The second-generation H1-antihistamine, rupatadine, was found to significantly reduce the development of chronic cold urticaria symptom without an increase in adverse effects using 20 and 40 mg.
Allergy medications containing antihistamines such as diphenhydramine (Benadryl), cetirizine (Zyrtec), loratidine (Claritin), cyproheptadine (Periactin), and fexofenadine (Allegra) may be taken orally to prevent and relieve some of the hives (depending on the severity of the allergy). For those who have severe anaphylactic reactions, a prescribed medicine such as doxepin, which is taken daily, should help to prevent and/or lessen the likelihood of a reaction and thus, anaphylaxis. There are also topical antihistamine creams which are used to help relieve hives in other conditions, but there is not any documentation stating it will relieve hives induced by cold temperature.
Cold hives can result in a potentially serious, or even fatal, systemic reaction (anaphylactic shock). People with cold hives may have to carry an injectable form of epinephrine (like Epi-pen or Twinject) for use in the event of a serious reaction.
The best treatment for this allergy is avoiding exposure to cold temperature.
Studies have found that Omalizumab (Xolair) may be an effective and safe treatment to cold urticaria for patient who do not sufficiently respond to standard treatments.
Ebastine has been proposed as an approach to prevent acquired cold urticaria.
The more poignant part of this disorder is the lack of desensitization for water and aqua intile injection as allergen even on repeated exposure. Avoidance of allergen as a general principle in any allergic disorder necessitates the evasion of water exposure. Topical application of antihistamines like 1% diphenhydramine before water exposure is reported to reduce the hives. Oil in water emulsion creams, petrolatum as barrier agents for water can be used prior to shower or bath with good control of symptoms. Therapeutic effectiveness of various classes of drugs differs from case to case.
Treatment varies. In most cases, the best treatment is to remove the cause of compression by modifying patient behavior, in combination with medical treatment to relieve inflammation and pain. Whatever the cause, typical treatment takes several weeks to months—depending on the degree of nerve damage. Typical treatment options include:
- Active Release Technique (ART) soft tissue treatment
- Wearing looser clothing and suspenders rather than belts
- Weight loss if obesity is present
- Non-steroidal anti-inflammatory drugs (NSAIDs) to reduce inflammatory pain if pain level limits motion and prevents sleep
- Reducing physical activity in relation to pain level. Acute pain may require absolute bed rest
- Deep tissue massage to reduce tension in the gluteal muscles, most commonly the gluteus maximus. The tensor fasciae latae may also be implicated.
The lateral cutaneous nerve of the thigh can occasionally be damaged during laparoscopic hernia repair, or scarring from the operation can lead to meralgia paraesthetica.
For lower pain levels, treatment may involve having the patient:
- Seek appropriate physical therapy, such as stretching and massage, which plays a large role in the management of pain
- Learn to perform inguinal ligament stretching (from a physical therapist or from a YouTube video) which can rapidly relieve symptoms
- Use rest periods to interrupt long periods of standing, walking, cycling, or other aggravating activity
- Lose weight, and exercise to strengthen abdominal muscles
- Wear clothing that is loose at the upper front hip area
- Apply heat, ice, or electrical stimulation
- Take nonsteroidal anti-inflammatory medications for 7–10 days
- Remove hair in affected area (shave)
- Lidocaine patches (must shave area first)
- Titanium dioxide patches to interfere with the electrostatic effect of the nerves on the surface of the skin
Pain may take significant time (weeks) to stop and, in some cases, numbness persists despite treatment. In severe cases, the physician might perform a local nerve block at the inguinal ligament, using a combination of local anaesthetic (lidocaine) and corticosteroids to provide relief that may last several weeks. Pain modifier drugs for neuralgic pain (such as amitriptyline, carbamazepine or gabapentin) may be tried, but are often not as helpful in the majority of patients.
Persistent and severe cases may require surgery to decompress the nerve or, as a last resort, to resect the nerve. The latter treatment leaves permanent numbness in the area.
Most hand injuries are minor and can heal without difficulty. However, any time the hand or finger is cut, crushed or the pain is ongoing, it is best to see a physician. Hand injuries when not treated on time can result in long term morbidity.
Antibiotics in simple hand injuries do not typically require antibiotics as they do not change the chance of infection.
Massage therapy using trigger-point release techniques may be effective in short-term pain relief. Physical therapy involving gentle stretching and exercise is useful for recovering full range of motion and motor coordination. Once the trigger points are gone, muscle strengthening exercise can begin, supporting long-term health of the local muscle system.
Myofascial release, which involves gentle fascia manipulation and massage, may improve or remediate the condition.
A systematic review concluded that dry needling for the treatment of myofascial pain syndrome in the lower back appeared to be a useful adjunct to standard therapies, but that clear recommendations could not be made because the published studies were small and of low quality.
Posture evaluation and ergonomics may provide significant relief in the early stages of treatment. Movement therapies such as Alexander Technique and Feldenkrais Method may also be helpful.
Gentle, sustained stretching exercises within a comfortable range of motion have been shown to decrease pain thresholds. Regular, non-intense activity is also encouraged.
Treatment for ulnar neuropathy can entail:
NSAID (non-steroidal anti-inflammatory) medicines. there is also the option of cortisone. Another possible option is splinting, to secure elbow, a conservative procedure endorsed by some. In cases where surgery is needed, cubital tunnel release, where the ligament of the cubital tunnel is cut, thereby alleviating pressure on nerve can be performed.
Treatment for the common occurrence of ulnar neuropathy resulting from overuse, with no fractures or structural abnormalities, is treatment massage, ice, and anti-inflammatories. Specifically, deep tissue massage to the triceps, myofascial release for the upper arm connective tissue, and cross-fiber friction to the triceps tendon.
Non-surgical therapies include:
- Shoe modifications: wearing shoes that have a wide toe box, and avoiding those with pointed toes or high heels.
- Oral nonsteroidal anti-inflammatory drugs may help in relieving the pain and inflammation.
- Injections of corticosteroid are commonly used to treat the inflammation.
- Bunionette pads placed over the affected area may help reduce pain.
- An ice pack may be applied to reduce pain and inflammation.
Surgery is often considered when pain continues for a long period with no improvement in these non-surgical therapies.
Familial cold urticaria (also properly known as familial cold autoinflammatory syndrome, FCAS) is an autosomal dominant condition characterized by rash, conjunctivitis, fever/chills and arthralgias elicited by exposure to cold - sometimes temperatures below 22 °C (72 °F).
It has been mapped to CIAS1 and is a slightly milder member of the disease family including Muckle–Wells syndrome and NOMID. It is rare and is estimated as having a prevalence of 1 per million people and mainly affects Americans and Europeans.
FCAS is one of the cryopyrin-associated periodic syndromes (CAPS) caused by mutations in the CIAS1/NALP3 (aka NLRP3) gene at location 1q44. The disease was described in The Lancet Volume 364 by Hoffman H.M. et al.
The effect of FCAS on the quality of life of patients is far reaching. A survey of patients in the United States in 2008 found, "To cope with their underlying disease and to try to avoid symptomatic, painful, flares patients reported limiting their work, school, family, and social activities. Seventy-eight percent of survey participants described an impact of the disease on their work, including absenteeism and impaired job advancement; frequently, they quit their job as a consequence of their disease".
Treatment using anakinra (Kineret) has been shown effective for FCAS, although this does mean daily injections of the immunosuppressant into an area such as the lower abdomen. The monoclonal antibody canakinumab (Ilaris) is also used.
After a disease-causing mutation has been identified in an index case (which is not always accomplished conclusively), the main task is genetic identification of carriers within a pedigree, a sequential process known as "cascade testing". Family members with the same mutation may show different severities of disease, a phenomenon known as "variable penetrance". As a result, some may remain asymptomatic, with little lifelong evidence of disease. Nevertheless, their children remain at risk of inheriting the disorder and potentially being more severely affected.
There is no treatment that will rid the patient of symptoms of aquagenic urticaria. Most treatments are used to lessen the effects of the disease to promote more comfort when the body must come in contact with water.
- Oral antihistamine: Antihistamines such as hydrochloride, hydroxyzine, terfenadine and cyproheptadine have frequently been used to reverse or minimize the effects of aquagenic urticaria. The therapeutic response to these medications will vary from patient to patient and the benefits of applying a histamine antagonist to the skin has not been found to create a direct link to the minimization of water based urticaria effects.
- Topical corticosteroids: Parenteral corticosteroids have been used to help treat aquagenic uricaria in the past. The actual effect of this medication and its benefits are not clear at this time.
- Epinephrine: Patients with severe bouts of urticarial that appear to be acute will frequently use this medication to help decrease the appearance of cutaneous vasodilation. This can also help inhibit mast cell degranulation which may contribute to the presence of aquagenic urticaria.
- PUVA therapy: In one test a 21-year-old woman was given PUVA therapy four times a week in increased doses to help manage the symptoms of aquagenic urticaria. As the dosage was increased the lesions and itching caused by the disease disappeared.
- Ultraviolet radiation: Radiation is commonly used alongside antihistamines to help rid the patient of lesions and outbreaks caused by aquagenic urticaria. This therapy will cause thickening of the epidermis which can prevent water from penetrating this layer and interacting with the cells underneath. Ultraviolet therapy may also cause mast cells to limit their response to stimuli and immunosuppression which can help prevent these reactions.
- Stanazolol: Treatments for the human immunodeficiency virus or HIV have been found to help with the symptoms of aqugenic urticaria as well.
- Capsaicin: This medication is often used for producing Zostrix, a cream applied to lessen pain caused by aquagenic urticaria.
- Barrier methods: In some circumstances an oil in water solution or emulsion cream can be applied to the skin to protect it from water exposure while washing or performing aquatic activities. There does not appear to be a side effect to this method and the application is easier than many other options. Doctors will also recommend that these patients use physical barriers such as an umbrella or protective clothing to avoid contact with water to protect patients from potential outbreaks. Activities such as swimming or visiting a water park will also need to be avoided to minimize the risk of an outbreak.
The causes of MPS are not fully documented or understood. At least one study rules out trigger points: "The theory of myofascial pain syndrome (MPS) caused by trigger points (TrPs) ... has been refuted. This is not to deny the existence of the clinical phenomena themselves, for which scientifically sound and logically plausible explanations based on known neurophysiological phenomena can be advanced." Some systemic diseases, such as connective tissue disease, can cause MPS. Poor posture and emotional disturbance might also instigate or contribute to MPS.