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Shwartzman phenomenon, also known as Shwartzman reaction, is a rare reaction of a body to particular types of toxins, called endotoxins, which cause thrombosis in the affected tissue. A clearing of the thrombosis results in a reticuloendothelial blockade, which prevents re-clearing of the thrombosis caused by a repeat introduction of the toxin. That will cause tissue necrosis. Shwartzman phenomenon is usually observed during delivery or abortion, when foreign bodies are introduced into the tissues of the female reproductive system.
The Shwartzman phenomenon is named for Gregory Shwartzman, the doctor at Mount Sinai Hospital in New York City who was the first to develop the concept of immune system hypersensitivity in the 1920s.
This reaction was experimented using "Neisseria meningitidis" endotoxin.
This is notably seen with "Neisseria meningitidis".
Lucio's phenomenon is treated by anti-leprosy therapy (dapsone, rifampin, and clofazimine), optimal wound care, and treatment for bacteremia including antibiotics. In severe cases exchange transfusion may be helpful.
CREST is not easily diagnosed as it closely mimics symptoms of other connective tissue and autoimmune diseases. Diagnoses are usually given when a patient presents three or more of the five major clinical symptoms. Additionally, blood exams can be given to test for a positive ANAs and ACAs or skin biopsies can be given to help confirm a diagnosis.
CREST syndrome can be noted in up to 10% of patients with primary biliary cirrhosis.
It is important to distinguish Raynaud's "disease" (primary Raynaud's) from "phenomenon" (secondary Raynaud's). Looking for signs of arthritis or vasculitis as well as a number of laboratory tests may separate them. If suspected to be secondary to systemic sclerosis, one tool which may help aid in the prediction of systemic sclerosis is thermography.
A careful medical history will often reveal whether the condition is primary or secondary. Once this has been established, an examination is largely to identify or exclude possible secondary causes.
- Digital artery pressure: pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mmHg is diagnostic (positive).
- Doppler ultrasound: to assess blood flow.
- Full blood count: this may reveal a normocytic anaemia suggesting the anaemia of chronic disease or renal failure.
- Blood test for urea and electrolytes: this may reveal renal impairment.
- Thyroid function tests: this may reveal hypothyroidism.
- An autoantibody screen, tests for rheumatoid factor, Erythrocyte sedimentation rate, and C-reactive protein, which may reveal specific causative illnesses or a generalised inflammatory process.
- Nail fold vasculature: this can be examined under the microscope.
To aid in the diagnosis of Raynaud's phenomenon, multiple sets of diagnostic criteria have been proposed. Table 1 below provides a summary of these various diagnostic criteria.
Recently, International Consensus Criteria were developed for the diagnosis of primary Raynaud's phenomenon by a panel of multiple experts in the fields of rheumatology and dermatology.
Patient should seek a physician for skin tests. Typically, after a consultation with rheumatologist, the disease will be diagnosed. A dermatologist is also another specialist that can diagnose.
Blood studies and numerous other specialized tests depending upon which organs are affected.
Secondary Raynaud's is managed primarily by treating the underlying cause and as primary Raynaud's, avoiding triggers, such as cold, emotional and environmental stress, vibrations and repetitive motions, and avoiding smoking (including passive smoking) and sympathomimetic drugs.
The microscopic examination of tissue (histology) gives the definitive diagnosis. The diagnostic histopathologic finding is intravascular cholesterol crystals, which are seen as cholesterol clefts in routinely processed tissue (embedded in paraffin wax). The cholesterol crystals may be associated with macrophages, including giant cells, and eosinophils.
The sensitivity of small core biopsies is modest, due to sampling error, as the process is often patchy. Affected organs show the characteristic histologic changes in 50-75% of the clinically diagnosed cases. Non-specific tissue findings suggestive of a cholesterol embolization include ischemic changes, necrosis and unstable-appearing complex atherosclerotic plaques (that are cholesterol-laden and have a thin fibrous cap). While biopsy findings may not be diagnostic, they have significant value, as they help exclude alternate diagnoses, e.g. vasculitis, that often cannot be made confidently based on clinical criteria.
The main pathological features of this disease are a vasculitis affecting all cutaneous vessels.
There are by five characteristic features:
- colonisation of endothelial cells by acid-fast bacilli
- endothelial proliferation and marked thickening of vessel walls to the point of obliteration
- angiogenesis
- vascular ectasia
- thrombosis of the superficial and mid-dermal blood vessels
The likely pathogenesis is endothelial cell injury due to colonization/invasion followed by proliferation, angiogensis, thrombosis and vessel ectasia.
Tests for inflammation (C-reactive protein and the erythrocyte sedimentation rate) are typically elevated, and abnormal liver enzymes may be seen. If the kidneys are involved, tests of renal function (such as urea and creatinine) are elevated. The complete blood count may show particularly high numbers of a type of white blood cell known as "eosinophils" (more than 0.5 billion per liter); this occurs in only 60-80% of cases, so normal eosinophil counts do not rule out the diagnosis. Examination of the urine may show red blood cells (occasionally in casts as seen under the microscope) and increased levels of protein; in a third of the cases with kidney involvement, eosinophils can also be detected in the urine. If vasculitis is suspected, complement levels may be determined as reduced levels are often encountered in vasculitis; complement is a group of proteins that forms part of the innate immune system. Complement levels are frequently reduced in cholesterol embolism, limiting the use of this test in the distinction between vasculitis and cholesterol embolism.
Reynolds syndrome is a rare secondary laminopathy, consisting of the combination of primary biliary cirrhosis and progressive systemic sclerosis. In some patients this syndrome has also been associated with Sjögren's syndrome and hemolytic anemia. Typical clinical features include jaundice, elevated blood levels of alkaline phosphatase, calcinosis cutis, telangiectasias, and pruritus. This disease may cause white or yellow-ish spots on the arms or legs. The syndrome, a special case of scleroderma, is named after the American physician, Telfer B. Reynolds, MD (1921–2004), who first described it. He is also known for creating one of the world's first hepatology programs at the University of Southern California.
It should not be confused with the more common Raynaud's phenomenon.
There is no current cure. The only way to treat this disease is by treating symptoms. Commonly patients are prescribed immunosuppressive drugs. Another route would be to take collagen regulation drugs.
The Mazzotti reaction, first described in 1948, is a symptom complex seen in patients after undergoing treatment of onchocerciasis with the medication diethylcarbamazine (DEC). Mazzotti reactions can be life-threatening, and are characterized by fever, urticaria, swollen and tender lymph nodes, tachycardia, hypotension, arthralgias, oedema, and abdominal pain that occur within seven days of treatment of microfilariasis. The Mazzotti reaction correlates with intensity of infection; however, there are probably multiple infection intensity-dependent mechanisms responsible for mediating this complex reaction.
The phenomenon is so common when DEC is used for the treatment of onchocerciasis that this drug is the basis of a skin patch test used to confirm that diagnosis. The drug patch is placed on the skin, and if the patient is infected with the microfilaria of "O. volvulus", localized pruritus and urticaria are seen at the application site.
A case of the Mazzotti reaction has been reported after presumptive treatment of schistosomiasis and strongyloidiasis with ivermectin, praziquantel and albendazole. The patient had complete resolution of symptoms after intravenous therapy with methylprednisolone.
Mees' lines appear after an episode of poisoning with arsenic, thallium or other heavy metals, and can also appear if the subject is suffering from renal failure. They have been observed in chemotherapy patients.
In studies, white coat hypertension can be defined as the presence of a defined hypertensive average blood pressure in a clinic setting, although it isn't present when the patient is at home.
Diagnosis is made difficult as a result of the unreliable measures taken from the conventional methods of detection. These methods often involve an interface with health care professionals and frequently results are tarnished by a list of factors including variability in the individual’s blood pressure, technical inaccuracies, anxiety of the patient, recent ingestion of pressor substances, and talking, amongst many other factors. The most common measure of blood pressure is taken from a noninvasive instrument called a sphygmomanometer. "A survey showed that 96% of primary care physicians habitually use a cuff size too small," adding to the difficulty in making an informed diagnosis. For such reasons, white coat hypertension cannot be diagnosed with a standard clinical visit. It can be reduced (but not eliminated) with automated blood pressure measurements over 15 to 20 minutes in a quiet part of the office or clinic.
Patients with white coat hypertension do not exhibit the signs indicative of trepidation and their increased blood pressure is often not accompanied by tachycardia. This is supported by studies that repeatedly indicate that 15%–30% of those thought to have mild hypertension as a result of clinic or office recordings display normal blood pressure and no unusual response to pressure stimulus. These persons did not show any specific characteristics such as age that may be indicative of a higher susceptibility to white coat hypertension.
Ambulatory blood pressure monitoring and patient self-measurement using a home blood pressure monitoring device is being increasingly used to differentiate those with white coat hypertension or experiencing the white coat effect from those with chronic hypertension. This does not mean that these methods are without fault. Daytime ambulatory values, despite taking into account stresses of everyday life when taken during the patient's daily routine, are still susceptible to the effects of daily variables such as physical activity, stress and duration of sleep. Ambulatory monitoring has been found to be the more practical and reliable method in detecting patients with white coat hypertension and for the prediction of target organ damage. Even as such, the diagnosis and treatment of white coat hypertension remains controversial.
Recent studies showed that home blood pressure monitoring is as accurate as a 24-hour ambulatory monitoring in determining blood pressure levels. Researchers at the University of Turku, Finland studied 98 patients with untreated hypertension. They compared patients using a home blood pressure device and those wearing a 24-hour ambulatory monitor. Researcher Dr. Niiranen said that "home blood pressure measurement can be used effectively for guiding anti-hypertensive treatment". Dr. Stergiou added that home tracking of blood pressure "is more convenient and also less costly than ambulatory monitoring."
Use of breathing patterns has been proposed as a technique for identifying white coat hypertension.
In one Turkish study of 438 consecutive patients, 38% were normotensive, 43% had white coat hypertension, 2% had masked hypertension, and 15% had sustained hypertension. Even patients taking medication for sustained hypertension who are normotensive at home may exhibit white coat hypertension in the office setting.
Although a clear understanding of the various skin lesions in IgG4-related disease is a work in progress, skin lesions have been classified into subtypes based on documented cases:
- Angiolymphoid hyperplasia with eosinophilia (or lesions that mimic it) and cutaneous pseudolymphoma
- Cutaneous plasmacytosis
- Eyelid swelling (as part of Mikulicz's disease)
- Psoriasis-like eruptions
- Unspecified maculopapular or erythematous eruptions
- Hypergammaglobulinemic purpura and urticarial vasculitis
- Impaired blood supply to fingers or toes, leading to Raynaud's phenomenon or gangrene
Note:
In addition, Wells syndrome has also been reported in a case of IgG4-related disease.
This is rare and is usually due to mutations in the R-spondin 4 (RSPO4) gene which is located on the short arm of chromosome 20 (20p13). Clinically it is manifest by the absence (anonychia) or hypoplasia (hyponuchia) of finger- and/or toenails.
IgG4-related skin disease is the recommended name for skin manifestations in IgG4-related disease (IgG4-RD). Multiple different skin manifestations have been described.
Anonychia is the absence of nails, an anomaly, which may be the result of a congenital ectodermal defect, ichthyosis, severe infection, severe allergic contact dermatitis, self-inflicted trauma, Raynaud phenomenon, lichen planus, epidermolysis bullosa, or severe exfoliative diseases.
They are typically white bands traversing the width of the nail. As the nail grows they move towards the end, and finally disappear when trimmed.
The term "toxic abortion" was first used to identify this phenomenon in humans in the earliest studies of the effects of pollutants on pregnancy in 1928, "An Experimental Investigation Concerning Toxic Abortion Produced by Chemical Agents" by Morris M. Datnow M.D.
Toxic abortion chemicals studied at that time were:
Petrochemicals,
Heavy metals,
Organic solvents,
Tetrachloroethylene,
Glycol ethers,
2-Bromopropane,
Ethylene oxide,
Anesthetic gases, and
Antineoplastic drugs.
In 1932, the "Journal of State Medicine" reported on a natural variation, with the occurrence of "a considerable number of cases of toxic abortion" being caused by untreated dental caries.
Study of pollution-caused abortion in humans ceased for a considerable time, interest renewing in the 2000s. A 2009 study found that fossil fuels play a role, as "pregnant African-American women who live within a half mile of freeways and busy roads were three times more likely to have miscarriages than women who don't regularly breathe exhaust fumes." A 2011 study found a correlation between exposure to workplace toxins and spontaneous abortion, and called for further study. "Newsweek" magazine reported in May 2014 that a spike in stillborn babies in the town of Vernal, in Utah, had correlated with an increase in pollution from new gas and oil drilling. "Newsweek" reported that "Vernal’s rate of neonatal mortality appears to have climbed from about average in 2010 (relative to national figures) to six times the normal rate three years later." "Newsweek" quoted one expert's observation that "We know that pregnant women who breathe more air pollution have much higher rates of virtually every adverse pregnancy outcome that exists." A study published in the "Journal of Environmental Health" in October 2014 found tetrachloroethylene or PCE, to be "linked to increased risk for stillbirths and other pregnancy complications."
The PCE study found that "pregnancies with high exposure to PCE were 2.4 times more likely to end with stillborn babies and 1.4 times more likely to experience placental abruption — when the placenta peels away from uterine wall before delivery, causing the mother to bleed and the baby to lose oxygen — compared with pregnancies never exposed to PCE." Higher exposure lead to a 35 percent higher risk of abruption. PCE has also been tied to an increased risk for cancer. Children exposed to PCE as fetuses and toddlers are more likely to use drugs later in life. The toxin has been linked to mental illness, an increased risk of breast cancer and some birth defects. It has been tied to anxiety, depression, and impairments in cognition, memory and attention. PCE contamination has been found in the Massachusetts water supply and "on military bases across the country," and "water systems in California and Pennsylvania and have also been found to be contaminated with PCE."
In 2015, "Newsweek" reported that chemicals found in fast food wrappers multiply miscarriage risk by sixteen times.
Some instances have been reported of women intentionally seeking to induce toxic abortion, where circumstances make medical abortion difficult to obtain, by exposing themselves to environmental toxins.
Paraproteinemias may be categorized according to the type of monoclonal protein found in blood:
- Light chains only (or Bence Jones protein). This may be associated with multiple myeloma or AL amyloidosis.
- Heavy chains only (also known as "heavy chain disease");
- Whole immunoglobulins. In this case, the paraprotein goes under the name of "M-protein" ("M" for monoclonal). If immunoglobulins tend to precipitate within blood vessels with cold, that phenomenon takes the name of cryoglobulinaemia.
The three types of paraproteins may occur alone or in combination in a given individual. Note that while most heavy chains or whole immunoglobulins remain within blood vessels, light chains frequently escape and are excreted by the kidneys into urine, where they take the name of Bence Jones protein.
It is also possible for paraproteins (usually whole immunoglobulins) to form polymers by aggregating with each other; this takes the name of macroglobulinemia and may lead to further complications. For example, certain macroglobulins tend to precipitate within blood vessel with cold, a phenomenon known as cryoglobulinemia. Others may make blood too viscous to flow smoothly (usually with IgM pentamer macroglobulins), a phenomenon known as Waldenström macroglobulinemia.
Some mucoceles spontaneously resolve on their own after a short time. Others are chronic and require surgical removal. Recurrence may occur, and thus the adjacent salivary gland is excised as a preventive measure.
Several types of procedures are available for the surgical removal of mucoceles. These include laser and minimally-invasive techniques which means recovery times are reduced drastically.
Micro-marsupialization is an alternative procedure to surgical removal. Micro-marsupialization uses silk sutures in the dome of a cyst to allow new epithelialized drainage pathways. It is simpler, less traumatic, and well-tolerated by patients, especially children.
A non-surgical option that may be effective for a small or newly identified mucocele is to rinse the mouth thoroughly with salt water (one tablespoon of salt per cup) four to six times a day for a few days. This may draw out the fluid trapped underneath the skin without further damaging the surrounding tissue. If the mucocele persists, individuals should see a doctor to discuss further treatment.
Smaller cysts may be removed by laser treatment, larger cysts will have to be removed surgically in an operating room.
In general, individuals with white coat hypertension have lower morbidity than patients with sustained hypertension, but higher morbidity than the clinically normotensive.
However, it should be remembered that all the established published trials on the consequences of high blood pressure and the benefits of treating are based on one-time measurement in clinical settings rather than the generally slightly lower readings obtained from ambulatory recordings.
The debate and conflicting ideas revolve around whether or not it would be feasible to treat white coat hypertension, as there still is no conclusive evidence that a temporary rise in blood pressure during office visits has an adverse effect on health.
In fact, many cross sectional studies have shown that "target-organ damage (as exemplified by left ventricular hypertrophy) is less in white-coat hypertensive patients than in sustained hypertensive patients even after the allowance has been made for differences in clinic pressure". Many believe that patients with "white coat" hypertension do not require even very small doses of antihypertensive therapy as it may result in hypotension, but must still be careful as patients may show signs of vascular changes and may eventually develop hypertension. Even patients with established hypertension that is well-controlled based on home blood pressure monitoring may experience elevated readings during office visits.
The Lazarus phenomenon raises ethical issues for physicians, who must determine when medical death has occurred, resuscitation efforts should end, and postmortem procedures such as autopsies and organ harvesting may take place.
Medical literature has recommended observation of a patient's vital signs for five to ten minutes after cessation of resuscitation before certifying death.