Idiopathic

Intrinsic asthma, also called idiopathic asthma, usually develops in adulthood. In intrinsic asthma allergic factors are not demonstrable. Episodes of intrinsic asthma may be triggered by a variety of stimuli, eg, emotional state, exposure to cold air, or inert dusts. Both intrinsic and extrinsic asthmatics can be prone to exercise-induced attacks. Individuals who experience a combination of extrinsic and intrinsic asthmatic reactions have mixed asthma. Status asthmaticus refers to an especially acute life-threatening asthma attack which is resistant to normal treatments and which may require hospitalization in order to stabilize the patient. 

Malabsorption syndrome Steatorrhea Tropical sprue Idiopathic hypercalcemia... 

Hematologic diseases autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, pernicous anemia Kidney disease Goodpasture syndrom, lipoid nephroses, minimal change glomerulonephritis Diseases of the gastrointestinal tract autoimmune chronic active hepatitis, autoimmune atrophic gastritis, Crohn s disease, ulcerative colitis... 

Benign familial neonatal convulsion is an idiopathic form of epilepsy beginning within the first six months after birth. Seizures include generalized and mixed, starting with tonic posture, ocular symptoms, and apnea, and often progress to clonic movements and motor automatisms. 

Seizure disorders are generally categorized as idiopathic or acquired. Idiopathic seizures have no known cause acquired seizure disorders have a known cause, including high fever, electrolyte imbalances, uremia, hypoglycemia, hypoxia, brain tumors, and some drug withdrawal reactions. Once the cause is removed (if it can be removed), the seizures theoretically cease. 

Same as bumetanide plus ascites due to malignancy, idiopathic edema, lymphedema Same as bumetanide plus hypertension (PO)... 

Loop diuretics are used in the treatment of edema associated with CHF, cirrhosis of the liver, and renal disease, including the nephrotic syndrome. These drug s are particularly useful when a greater diuretic effect is desired. Furosemide is the drug of choice when a rapid diuresis is needed or if the patient has renal insufficiency. Furosemide and torsemide are also used to treat hypertension. Ethacrynic acid is also used for the short-term management of ascites caused by a malignancy, idiopathic edema, or lymphedema. 

Idiopathic or secondary thrombocytopenic purpura, hemolytic anemia, red blood cell anemia, congenital hypoplastic anemia... 

These disorders are all acquired conditions with no evidence of an hereditary basis. Most of them involve inflammation of the skeletal muscle itself (myositis) (Figure 17), though this may sometimes occur because of initial targeting of the muscle vasculature or connective tissue. Many instances of myositis are classed as idiopathic disorders, in that the precise mechanisms of muscle degeneration are not known, but is widely accepted that these syndromes are associated with abnormal function of the immune system. The syndromes of polymyositis (PM) and derma-... 

Fig. 8.14. a Sixty-eight-old-year woman with idiopathic thrombocytopenia that prevented any cosmetic surgical intervention, b One year after the performance of deep chemical peeling... 

Wiggins CA, Dykowicz MS, Patterson R Idiopathic anaphylaxis. Classification, evaluation and treatment of 123 patients. J Allergy Clin Immunol 1988 82 849-855. 

Adding another layer of complexity to the regulation of mast cell activation levels in vivo is the observation that activated mast cells can respond to, and in some cases produce, a myriad of mediators that may serve to amplify FceRI-induced responses. For example, stem cell factor (SCF), the ligand for KIT, both can enhance FceRI-dependent activation of mouse or human mast cells and, under certain circumstances, can directly induce mast cell degranulation [6, 25, 62]. Thus, elevated SCF levels and/or activating KIT mutations (such as those that occur in mastocytosis) may exacerbate mast cell-driven reactions. Indeed, patients (both adult and children) with extensive skin disease associated with mastocytosis are at increased risk to develop severe anaphylaxis [63]. Moreover, it was recently reported that cases of idiopathic anaphylaxis are... 

Brittain E, Noel P, Metcalfe DD Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with idiopathic anaphy- 72 laxis. Blood 2007 110 2331-2333. 

Systemic anaphylaxis in man is frequently accompanied by electrocardiographic alterations ischemic ST waves, arrhythmias and atrial fibrillation [6-11]. Anaphylactic reactions after insect stings can lead to coronary spasm or acute myocardial infarction [12, 13]. Myocardial infarction can also occur as a consequence of idiopathic... 

Patella V, Marino I, Arbustini E. Lamparter-Schummert B, Verga L, Adt M. Marone G Stem cell factor in mast cells and increased mast cell density in idiopathic and ischemic cardiomyopathy. Circulation 1998 97 971. 

In one study, 26% of anaphylactic reactions were reported to have developed after a combination of elicitors [4]. In other patients with mastocytosis, anaphylaxis remains idiopathic despite an extensive search for an allergic basis. 

In rare cases, initiation of specific immunotherapy with insect venom leads to recurrent anaphylaxis, even with antihistamine premedication. In those cases, comedication with omalizumab (anti-IgE) has been reported to induce tolerance. In a case of recurrent anaphylaxis to induction of specific immunotherapy, the injection of 300 mg of omalizumab between 4 days and 1 h reportedly led to tolerance [42]. This approach also appears worthy of consideration in patients with both idiopathic recurrent anaphylaxis and mastocytosis who do not respond to standard antimediator therapy, as has been described in 2 atopic patients with ISM [43]. Most patients with mastocytosis and idiopathic anaphylaxis, however, are sufficiently controlled by standard antimediator therapy with antihistamines with or without low-dose corticosteroids. 

Not all symptoms after RCM exposure do resemble a hypersensitivity reaction. Toxic reactions related to the toxicity of RCM, imspecific reactions of unknown origin and or factors unrelated to RCM, such as chronic idiopathic urticaria, may occur (fig. 1) [3]. Hypersensitivity reactions to RCM may both present either under the clinical picture of anaphylaxis with the potential to result in fataUties or as delayed occurring... 

Some patients with chronic idiopathic urticaria develop wheals and even angioedema after aspirin or NSAIDs. In others, aspirin causes an obvious increase in the underlying urticaria. The reaction may occur in just 15 min or up to 24 h following aspirin ingestion, but on average it develops within 1-4 h. Most cases resolve within a few hours, but in severe reactions bouts of multiform skin eruptions, covering most of the body, may continue for 10 days after aspirin intake [8,16,17]. 

Patients with chronic idiopathic urticaria, who develop cutaneous reactions in response to aspirin, display certain similarities in eicosanoid profile with AIA. The mechanism of the reactions is often related to COX-1 inhibition [18]. Therefore, aspirin and all drugs that inhibit COX-1 should be avoided in patients who already have had cutaneous reactions to NSAID. Coxibs are usually well tolerated, although occasional adverse reactions have been reported [19, 20]. For treatment of the reactions, antihistamines are usually sufficient, but in more severe cases adrenaline and corticosteroids may be warranted. 

Zembowicz A, Mastalerz L, Setkowicz M, Radzis-zewski W, Szczeklik A Safety of cyclooxygenase-2 inhibitors and increased leukotriene synthesis in chronic idiopathic urticaria with sensitivity to nonsteroidal anti-inflammatory drugs. Arch Dermatol 2003 139 1577-1582. 

Ring J, Darsow U Idiopathic anaphylaxis. Curr Allergy Asthma Rep 2002 2 40-45. 

Asbestosis Idiopathic Fibrosis Pulmonary Edema Sarcoidosis... 

Scheibel I, Veit T, Neves AG, Souza L, Prezzi S, Machado S, Kohem C, IcareUi M, Xavier R, Brenol JC, Chies JA (2008) Differential CCR5Delta32 aUehc frequencies in juvenile idiopathic arthritis subtypes evidence for different regulatory roles of CCR5 in rheumatological diseases. Scand J Rheumatol 37 13-17... 

Abbadie C, Lindia JA, Cumiskey AM, Peterson LB, Mudgett JS, Bayne EK, DeMartino JA, MacIntyre DE, Forrest MJ (2003) Impaired neuropathic pain responses in mice lacking the chemokine receptor CCR2. Proc Natl Acad Sci USA 100 7947-7952 Agostini C, Gurrieri C (2006) Chemokine/cytokine cocktail in idiopathic pulmonary fibrosis. Proc Am Thorac Soc 3 357-363... 

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