- General information
- Typical symptoms of mast cell activation
- Some more keywords
Mast cell diseases cannot be identified from a uniform clinical picture. Each case presents itself differently. The symptom constellation is as changeable as a chameleon.
The symptoms often resemble an allergy, food poisoning, a cold or other infectious disease. They occur in particular in connection with food intake, but can also persist chronically or occur in episodes without the affected person recognising any connection with the diet. An enormously wide range of predominantly unspecific symptoms is possible. The symptoms that occur vary from person to person. The following typical cardinal symptoms may occur:
- Acute or chronic irritation, inflammation, pain, swelling
- Disorders of the nervous system: sleep disorders, fatigue, nervousness, exhaustion syndrome, concentration disorders, memory disorders, susceptibility to stress, depressive moods
- Indigestion: diarrhea or constipation, abdominal pain, flatulence, heartburn, bad-smelling intestinal winds, irritable bowel syndrome
- Chronic inflammatory diseases
- Itching, skin rash, skin redness, flushing (flush on face)
- Hot flushes, sweating, disturbed temperature sensation [Carlin et al. 2015]
- Tachycardia, cardiac arrhythmia, palpitations, drop in blood pressure
- Headaches, migraines, dizziness
- Nausea, vomiting
- Dysmenorrhea, menstrual cramps, painful periods
- Edema (swelling, fluid accumulation, e.g. swollen eyelids)
There is no uniform clinical picture!
Mast cell activation diseases (MCAD) cannot be identified from a uniform clinical picture. Each case presents itself differently. The symptom constellation is at least as changeable as a chameleon.
To find out whether one has a certain disease or not, everybod tends to compare his own symptoms with those of other affected persons or with those described in literature. This seems obvious, but unfortunately does not work with mast cell activation diseases. Every case is different:
- Everyone has their own symptoms. The symptoms mentioned here may or may not be present, and the list does not claim to be complete. Many symptoms occur only temporarily and not necessarily simultaneously. Thus the affected person often does not suspect that his/her various complaints have something to do with each other.
- The severity is very different. Everything is possible, from those who are easily affected, who only have a little diarrhoea, headaches or itching from time to time, to those who are severely affected by almost all the symptoms described here and are permanently unable to work, or who are even dependent on stationary care in a clinic. Fortunately, the vast majority are only slightly affected.
- The symptoms are also non-specific, i.e. there are many other diseases which can also cause such symptoms.
In short: The symptoms alone do not indicate whether it is an MCAD or not.
Very broad variety of symptoms possible
More than 200 different mediators (messengers) can be released from activated mast cells, which can influence countless processes in the body. Histamine alone - probably the most important symptom trigger - fulfils numerous functions in our body. If histamine is released excessively (histaminosis), these processes are incorrectly regulated. Depending on the part of the body into which pathologically altered mast cells have migrated, different tissues, organs or organ systems can be affected. The type and strength of the triggering stimulus also determines which symptoms occur. Therefore, a particularly wide range of symptoms is possible, but not every affected person shows the same symptoms, and a single affected person does not always have the same symptoms. Some symptoms are chronic or very common, others may be sporadic.
In some patients, acute symptoms dominate, which occur shortly after the confrontation with a trigger and quickly decrease again. In others, the course is rather chronic, so that a single trigger contributes only imperceptibly to the permanent symptom level and is therefore difficult to recognise as such. Of course there are not only these two extremes, but one can also lie somewhere in between or react to a trigger with acute symptoms as well as with other chronic symptoms. In addition, the nature of the symptoms also depends on the characteristics of the trigger in question.
The figure below illustrates the different course of symptoms over time using a highly simplified model calculation. The calculation example shows how the intensity of the symptoms increases when an incompatible meal is taken daily at 6 am, 12 am and 6 pm.
In the acute course of the disease, the amount of histamine rises sharply, but then quickly decreases again. In a chronic course, the amount of histamine after a single meal does not increase much compared to a healthy person. However, it only decreases very slowly. At the next meal, the new histamine dose is added to the remaining amount of histamine and thus increases from day to day until after several days an equilibrium concentration is reached where the increase and decrease are the same.
The concentration levels of released mast cell mediators (e.g. histamine) only reach their maximum a long time after the trigger concentration has already started to sink again. Reason: Even after the trigger has reached its maximum concentration and decreases again, it continues to release mediators. The mediator level therefore continues to rise as long as the release rate exceeds the elimination rate (hypothetical model calculation).
Triggers are difficult to identify
If the affected person succeeds at all in identifying some of the many triggers, then these are the most likely:
Foodstuffs: red wine, sparkling wine, champagne (“alcohol allergy”), well-ripened cheese, fish (especially tuna) and seafood, meat (sausages, dried meat), tomato, pizza (“pizza allergy”), nuts, sauerkraut, convenience food, additives (“additive intolerance”).
Active drug ingredients: acetylsalicylic acid (aspirin), X-ray contrast agents, painkillers, anaesthetics, (“drug allergy”).
Various degrees of severity
The severity of MCAD can be very different. Slightly affected persons or healthy persons may only notice symptoms in extreme situations, for example after eating sumptuous meals in combination with alcohol or medication. In very severe cases, the untreated metabolic disorder can lead to daily or even permanent inability to work, usually due to exhaustion. As long as they do not yet know and treat the disease, many sufferers are constantly a little ailing and severely restricted in their quality of life. They suffer from some kind of discomfort every now and then or permanently. Most of them are not really ill (in the sense of unable to work), but can lead a reasonably normal life.
Important: The symptoms listed here are mostly non-specific symptoms. This means that many other conditions may cause the same symptom(s) as well. It should therefore always be clarified whether other diseases are also possible (differential diagnosis). It is not possible to diagnose histaminosis on the basis of symptoms. However, if a patient suffers again and again from some of the listed symptoms without a clear cause or trigger being identifiable, a histaminosis (especially a mast cell activation disease) should be considered. See also our note on health topics.
Typical symptoms of mast cell activation
In scientific publications, the following symptoms are mentioned as the most important, which most reliably indicate mast cell activation:
|Typical symptoms of mast cell activation (according to scientific literature)|
|Skin redness, facial redness, flushing [Valent et al. 2012]|
|Itching (pruritus) [Afrin et al. 2017; Valent et al. 2012]|
|Hives (urticaria), certain forms of skin rashes [Afrin et al. 2017; Valent et al. 2012]|
|Swelling of the skin, painless, usually not itchy (angioedema, Quincke's edema) [Valent et al. 2012]|
|Stuffy nose (nasal constipation) [Valent et al. 2012]|
|Sneezing (nasal itching) [Valent et al. 2012]|
|Pipes / belts in the airways (pulmonary noises) [Valent et al. 2012]|
|Swelling of the upper respiratory tract [Valent et al. 2012]|
|Headaches [Afrin et al. 2017; Valent et al. 2012]|
|Blood pressure too low (hypotension) [Valent et al. 2012]|
|Diarrhea or constipation or both alternating [Afrin et al. 2017; Valent et al. 2012]|
|Most common symptoms of MCAS (according to scientific literature)||Frequency|
|Fatigue, tiredness, exhaustion [Afrin et al. 2017]||83%|
|Fibromyalgia-like pain [Afrin et al. 2017]||75%|
|Sudden brief unconsciousness (syncope); drowsiness, dizziness, near unconsciousness (presyncope) [Afrin et al. 2017]||71%|
|Headaches [Afrin et al. 2017]||63%|
|Itching (pruritus); hives (urticaria) [Afrin et al. 2017]||63%|
|Tingling, furry, "ant running", (paresthesia) [Afrin et al. 2017]||58%|
|Nausea, vomiting [Afrin et al. 2017]||57%|
|Muscle tremor, chills [Afrin et al. 2017]||56%|
|Swelling, fluid accumulation in tissue (edema) [Afrin et al. 2017]||56%|
|Eye irritations (ocular irritation) [Afrin et al. 2017]||53%|
|Breathlessness, shortness of breath (dyspnoea) [Afrin et al. 2017]||53%|
|Stomage acid upsetting, reflux of gastric acid into the esophagus (gastroesophageal reflux) [Afrin et al. 2017]||50%|
|Impairment of mental performance (cognitive dysfunction): memory disorders, concentration disorders, learning disorders [Afrin et al. 2017]||49%|
|Pathological skin changes (efflorescence): spots, stains, nodules, blisters, pustules, hives, pimples, blackheads (comedones), cysts, calluses [Afrin et al. 2017]||49%|
|Abdominal pain, stomachache [Afrin et al. 2017]||48%|
|Heart palpitations, cardiac stumbling, arrhythmia, tachycardia [Afrin et al. 2017]||47%|
|Profuse sweating, sweat outbreaks, outbreaks of perspiration [Afrin et al. 2017]||47%|
|Allergies [Afrin et al. 2017]||40%|
|Fever [Afrin et al. 2017]||40%|
|Chest pain [Afrin et al. 2017]||40%|
|Tendency to bleed, bruising, nosebleeds [Afrin et al. 2017]||39%|
|Diarrhoea and constipation alternately [Afrin et al. 2017]||36%|
|Swallowing disorders (proximal dysphagia) [Afrin et al. 2017]||35%|
|Sleeplessness (insomnia) [Afrin et al. 2017]||35%|
|Blushing (flush) [Afrin et al. 2017]||31%|
|Visual impairment [Afrin et al. 2017]||30%|
|Enlarged lymph nodes, inflammation of the glands (adenitis, adenopathy) [Afrin et al. 2017]||28%|
|Diarrhea (diarrhoea) [Afrin et al. 2017]||27%|
|Susceptibility to infections [Afrin et al. 2017]||27%|
|Impaired wound healing, excess scarring [Afrin et al. 2017]||23%|
|Sinusitis [Afrin et al. 2017]||17%|
|Cough [Afrin et al. 2017]||16%|
|Anxiety, panic attacks [Afrin et al. 2017]||16%|
However, in reports on their experiences, those affected also mention countless other symptoms, the cause of which they see in their mast cell disease. In the following, we list symptoms which, according to reports from affected persons, either decrease significantly or disappear completely under the therapy described on this website (mast cell-specific medication and/or elimination diet). Listing without claim to completeness. The symptoms listed here are optional i.e they may or may not occur:
Digestive tract, gastrointestinal system
The most common type of histaminosis is acute or chronic gastrointestinal discomfort. These complaints are often referred to as non-specific, as the often very extensive diagnostics do not show any pathological findings and many possible causes are possible. One speaks of an irritable stomach or irritable bowel. A direct connection between food intake and symptoms is often difficult to detect. This is due to the fact that the food components are only slowly absorbed into the body during the intestinal passage of several hours and that there is a delay before complaints occur. However, foods heavily contaminated or spoiled with histamine can also lead to abdominal cramps and acute diarrhoea within about 15-30 minutes.
- Digestive complaints, flatulence, stomach ache, stomach sting, stomach and intestinal cramps
- Frequent or chronic diarrhoea, morning diarrhoea, histamine increases the motility of the intestinal movements, whereby the food pulp passes through the intestine faster than normal and can only be digested incompletely due to the shortened residence time.
- More rarely also constipation or alternating diarrhoea and constipation
- Heartburn, stomach acid upsetting (gastroesophageal reflux)
- Inflammatory gastrointestinal diseases, irritable bowel syndrome (IBS)
- Nausea, vomiting
- Seasickness, motion sickness, travel sickness
- Chinese restaurant syndrome (controversial)
- Symptoms similar to gastrointestinal influenza (gastroenteritis)
- Drop in blood pressure, low blood pressure (hypotension, hypotonia)
- Irregular heartbeat (cardiac arrhythmia, “heart stumbling”)
- Palpitations (increase of the heart's power by releasing adrenaline)
- Rapid heartbeat, increase in heart rate (tachycardia), up to panic attacks
Skin, mucous membranes, respiratory tract
- “persistent cold”, swelling of the nasal mucous membrane, runny nose (rhinitis, rhinorrhoea), especially when taking meals, maybe even independent of the type and histamine content of the meal, possibly intensified by cold weather / smoke / smog / odors
- When blowing the nose, there may also be some blood (increased permeability of blood vessels).
- Severe sweating, night sweats (nocturnal sweating), sweaty feet (perspiring feet) / sweaty hands, hot flash (hot flushes)
- Flushing of the face after meals, feeling of heat, facial skin feels slightly inflamed
- Facial skin impurities, acne, pimples, blackheads, sebum overproduction, oily skin
- Rashes, skin redness, eczema, hives (urticaria)
- Itching (e.g. itchy scalp, itchy vaccination scars)
- Physical stimuli such as scratches, blows or heat cause reddening / skin rash and itching.
- Follicular keratosis, chicken skin, keratosis pilaris: Upper arms, sometimes also thighs, face etc. are covered with small red spots / pimples, occasionally keratinised or purulent (keratinisation disorders). Possible connection with pollen allergies? (picture on wikipedia.org)
- Burning and/or painful inflammation on the hands / bubbles / nodules / calluses (picture)
- “Sun allergy”: sensitivity to sunlight. The skin quickly turns red in the sun, but on the following day the “sunburn” is gone again.
- Dry lips
- Aphthae: Micro injuries of the oral mucosa become small yellow-white “holes” or “wounds” that hurt like hell and do not heal for days or months. In addition to the mucous membrane of the mouth, individual papillae on the tongue can also hurt like an aphtum.
- Chronic cough, permanent tickly throat, dry irritable cough, bronchitis, irritated bronchi
- Frequent clearing one's throat or coughing slightly, especially in stressful situations
- Sputum, phlegm: coughed-up material from the lower airways, hoarse voice, especially after sumptuous meals (similar to cystic fibrosis)
- Edema, oedema (= swelling due to fluid accumulation in the interstitial tissue), e.g. swollen eyelids, swollen legs
Histamine is a neurotransmitter and is also involved in the regulation of the sleep-wake cycle [Kim et al. 2015]. If the body is flooded with histamine and other mast cell mediators, this can also lead to a variety of neurological symptoms [Afrin et al. 2015, Pöhlau et al. 2015].
- Headache, migraine, feeling of pressure in the head, dizziness
- Fatigue, tiredness, listlessness, lack of energy, exhaustion
- Insomnia, sleeplessness, sleeping disorders, only superficial dozing without deep sleep, dreamlessness
- Concentration disorders, impairment of mental performance, slowness
- Forgetfulness, limited recall of memories
- Sensitivity to noise, search for calm and uneventfulness, susceptibility to sensory overload
- Susceptibility to stress, reduced resilience, burnout feeling (feeling of mental/nervous exhaustion or overwork), reduced labour productivity under pressure to perform
- Always coughing slightly, especially in stressful situations
- Nervousness (even without external cause), restlessness, tingling, feeling like a caffeine overdose or neurotoxin poisoning, hyped-up, over-excited
- Anxiety, panic attacks
- Muscle cramps, muscle twitches, tremors, cramped jaw muscles, teeth grinding, sanded teeth
- Symptoms like mild intoxication with a neurotoxin
- Melancholy, sadness, tearfulness, depressive moods, depressions (often for no apparent reason)
- Suicidal thoughts
- Temporary loss or impairment of the sense of smell
- Central and/or peripheral psychiatric and neurological disorders, e.g. personality changes [Afrin et al. 2015, Pöhlau et al. 2015]
- Menstrual cramps: Dysmenorrhea (= pain in periods, menstrual pain), cycle disorders
- Developmental disorders, failure to thrive
Susceptibility to inflammation, immune system
- Increased susceptibility to infections, frequently ill
- Increased susceptibility to inflammation, inflammable parts / areas
- Sore throat
- Flu-like symptoms without clear outbreak of disease, persistent feeling of illness, pain in limbs
- Sinusitis: inflammation of the maxillary sinus, inflammation of other sinuses such as the sphenoid sinus or the ethmoidal sinus
- Lymph nodes permanently swollen or painful
- Tonsillitis, growth of the pharyngeal tonsils (pharyngeal tonsil hyperplasia), possibly surgical removal of the tonsils. (The tonsils serve the immune defence. Their absence after the operation can increase the increased susceptibility to infection.)
- Feeling of a lump in the throat
- Connective tissue inflammation: tissue sites under the skin with inflammation pain or pressure sensitivity
- chronic (non-bacterial) inflammation of the brain (chronic encephalitis), feeling of heat and pressure inside the head
- Inflammation of the bladder (non-bacterial cystitis): painful and burning urinary bladder, urge to urinate, frequent passing of water (similar to bacterial cystitis)
- burning eyes, inflammation of the conjunctiva, reddened, scratchy eyes, watery eyes
- Yellow-white material accumulates in the inner corners of the eyes.
- Hazy vision, veil vision, blurred vision
- Joint pain
- Bone pain, sore bones, boneache (ostealgia)
- Inflammatory rheumatic diseases, articular rheumatism (e.g. finger joint rheumatism)
- Soft-tissue rheumatism: e.g. tendon or joint problems, back pain: back muscles hurt similarly to dislocation / muscle soreness (muscle rheumatism, muscle inflammation).
- Temporary toothache, inflamed gums or inflamed wisdom teeth
- Herpes simplex labialis (labial herpes, fever blisters) or herpes-like symptoms (e.g. angular cheilitis (Cheilitis angularis) = non-healing skin cracks in the corners of the mouth)
- Alcoholic beverages are incompatible or only tolerated in small quantities ("alcohol intolerance", "alcohol allergy")
- Binge hunger attacks, ravenous appetite, ravenousness
- Vitamin and nutrient deficiencies (especially iron deficiency, vitamin B12 deficiency) due to disturbed absorption mechanisms of the irritated / inflamed intestine
A persistently inflamed, irritable and weakened body may be more sensitive to minor environmental influences that go unnoticed by a healthy person.
- Weather sensitivity
- Electrosensitivity ??? [Belpomme, Campagnac and Irigaray 2015]
- Sensitivity to earth rays, water veins, force points, lattice structures and other phenomena of radiesthesia ???
Some more keywords
Other terms (most of which are not recognized by conventional medicine) that are often used for (or confused with) such “dubious” symptoms:
Somatisation disorder, somatoform disorders, multiple chemical sensitivity (MCS), chemical hypersensitivity syndrome, sick building syndrome (SBS), environmental allergy, ecological disease, idiopathic environmental intolerance, eco-syndrome, allergic toxaemia, cerebral allergy, total allergy syndrome, 20th century disease, allergy to the 20th century. Civilization diseases of the industrialized countries, Burnout syndrome, Chronic Fatigue syndrome CFS, Chronic exhaustion syndrome, Fibromyalgia syndrome, electrosensitivity (electrosmog sensitivity), amalgam poisoning, "detergent allergy", multi-organ dysesthesia, Alexithymia, psychosomatist, psychosomatic, hypochondria, internet-hypochondriac, infection phobia, imaginary patient.
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|Afrin et al. 2017||Afrin LB, Self S, Menk J, Lazarchick J: "Characterization of Mast Cell Activation Syndrome" The American journal of the medical sciences. 2017;353(3):207-215. doi:10.1016/j.amjms.2016.12.013.|
(A description of the clinical picture of systemic mast cell activation syndrome (MCAS))
|Afrin et al. 2015||Afrin LB, Pöhlau D, Raithel M, Haenisch B, Dumoulin FL, Homann J, Mauer UM, Harzer S, Molderings GJ.: "Mast Cell Activation Disease: An Underappreciated Cause of Neurologic and Psychiatric Symptoms and Diseases." Brain Behav Immun. 2015 Jul 7. pii: S0889-1591(15)00236-6. doi: 10.1016/j.bbi.2015.07.002.|
(“We describe MCAD's pathogenesis, presentation (focusing on [central and/or peripheral neurologic and/or psychiatric symptoms] (NPS)), and therapy, especially vis-à-vis neuropsychotropes. Since MCAD patients often present NPS, neurologists and psychiatrists have the opportunity, in recognizing the diagnostic possibility of MCAD, to short-circuit the often decades-long delay in establishing the correct diagnosis required to identify optimal therapy.”)
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|Belpomme, Campagnac and Irigaray 2015||Belpomme D, Campagnac C, Irigaray P: "Reliable disease biomarkers characterizing and identifying electrohypersensitivity and multiple chemical sensitivity as two etiopathogenic aspects of a unique pathological disorder." Rev Environ Health. 2015 Dec 1;30(4):251-71. doi: 10.1515/reveh-2015-0027.|
(Electrosensitivity, chemical sensitivity, blood-brain barrier, oxidative stress, nitrostress, chronic inflammation, autoimmune diseases, neuro-degenerative diseases:
“[...] we have prospectively investigated, clinically and biologically, 1216 consecutive electro-hypersensitivity (EHS) and/or multiple chemical sensitivity (MCS)-self reporting cases [...]. 521 (71.6%) were diagnosed with EHS, 52 (7.2%) with MCS, and 154 (21.2%) with both EHS and MCS. Two out of three patients with EHS and/or MCS were female; mean age (years) was 47. As inflammation appears to be a key process resulting from electromagnetic field (EMF) and/or chemical effects on tissues, and histamine release is potentially a major mediator of inflammation, we systematically measured histamine in the blood of patients. Near 40% had a increase in histaminemia (especially when both conditions were present), indicating a chronic inflammatory response can be detected in these patients. [...] Both disorders appear to involve inflammation-related hyper-histaminemia, oxidative stress, autoimmune response, capsulothalamic hypoperfusion and blood-brain-barrier opening, and a deficit in melatonin metabolic availability; suggesting a risk of chronic neurodegenerative disease. Finally the common co-occurrence of EHS and MCS strongly suggests a common pathological mechanism.”)
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|Carlin et al. 2015||Carlin JL, Tosh DK, Xiao C, Pinol RA, Chen Z, Salvemini D, Gavrilova O, Jacobson KA, Reitman ML: "Peripheral Adenosine A3 Receptor Activation Causes Regulated Hypothermia in Mice that is Dependent on Central Histamine H1 Receptors." J Pharmacol Exp Ther. 2015 Nov 25. pii: jpet.115.229872.|
(Mast cells are involved in the regulation of body temperature:
“A3AR agonist-induced hypothermia was attenuated by mast cell granule depletion, demonstrating that the A3AR hypothermia is mediated, at least in part, via mast cells. [...] Mast cells release histamine and blocking central histamine H1 (but not H2 or H4) receptors prevented the hypothermia. [...] These results support a mechanistic model for hypothermia in which A3AR agonists act on peripheral mast cells, causing histamine release, which stimulates central H1R to induce hypothermia. This mechanism suggests that A3AR agonists are not likely to be useful for clinical induction of hypothermia.”)
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|Kim et al. 2015||Kim YS, Kim YB, Kim WB, Yoon BE, Shen FY, Lee SW, Soong TW, Han HC, Colwell CS, Lee CJ, Kim YI: "Histamine resets the circadian clock in the suprachiasmatic nucleus through the H1R-CaV 1.3-RyR pathway in the mouse." Eur J Neurosci. 2015 Jul 28. doi: 10.1111/ejn.13030.|
“Histamine, a neurotransmitter / neuromodulator implicated in the control of arousal state, exerts a potent phase-shifting effect on the circadian clock in the rodent suprachiasmatic nucleus. [...] Collectively, these results indicate that, to reset the circadian clock, histamine increases [Ca2+ ]i in SCN neurons by activating CaV 1.3 channels through H1R, and secondarily by causing Ca2+ -induced Ca2+ release from RyR-mediated internal stores.”
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|Moura et al. 2014||Moura DS, Georgin-Lavialle S, Gaillard R, Hermine O: "Neuropsychological features of adult mastocytosis." Immunol Allergy Clin North Am. 2014 May;34(2):407-22. doi: 10.1016/j.iac.2014.02.001.|
“In approximately one third of cases, patients with mastocytosis may experience various general and neuropsychological disabling symptoms. General signs can have a major impact on quality of life. Neurological symptoms are less common. In most cases, the pathophysiology of these symptoms is unknown, but could be associated with mast cell infiltration, release of mast cell mediators, or both.”
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|nmi||www.nahrungsmittel-intoleranz.com > Home > Histamin > Symptome|
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|Pöhlau et al. 2015||Dr.med. Dieter Pöhlau, Prof. Dr. med. Martin Raithel, PD Dr. rer.nat. Britta Haenisch, Dipl.Phys. Sabrina Harzer, Prof. Dr. med. Gerhard J. Molderings: "Vermutlich oft gesehen, aber nur selten diagnostiziert: Neurologische und psychiatrische Symptome der systemischen MCAD" ("Probably seen often, but rarely diagnosed: Neurological and psychiatric symptoms of systemic MCAD") NeuroTransmitter 2015; 26 (9).|
“In neurology and psychiatry, there are often patients with central nervous symptoms who suffer permanently or episodically from many other complaints, the cause of which remains unclear despite extensive research and whose symptomatic therapies are largely frustrated. Systemic mast cell activation disease (MCAD) is rarely included in differential diagnostic considerations.”
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|SIGHI||Experience gathered from the community of our members|
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|Valent et al. 2012||Valent P, Akin C, Arock M, Brockow K, Butterfield JH, Carter MC, Castells M, Escribano L, Hartmann K, Lieberman P, Nedoszytko B, Orfao A, Schwartz LB, Sotlar K, Sperr WR, Triggiani M, Valenta R, Horny HP, Metcalfe DD.: "Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal". Int Arch Allergy Immunol. 2012;157(3):215-25. Epub 2011 Oct 27.|
(Propose a global unifying classification of all MC disorders and pathologic MC reactions. This classification includes three types of 'MCA syndromes' (MCASs), namely primary MCAS, secondary MCAS and idiopathic MCAS. MCA is now defined by robust and generally applicable criteria, including (1) typical clinical symptoms, (2) a substantial transient increase in serum total tryptase level or an increase in other MC-derived mediators, such as histamine or prostaglandin D(2), or their urinary metabolites, and (3) a response of clinical symptoms to agents that attenuate the production or activities of MC mediators.)