leukotriene-b4 and Familial-Mediterranean-Fever

Acute right lower abdominal pain may present a diagnostic dilemma. Leukotrienes have been found to be elevated in familial Mediterranean fever (FMF), a disease manifesting with recurrent episodes of "acute abdomen.". To determine whether urine leukotriene B4 (LTB4) may differentiate between an FMF attack and some other forms of acute right lower abdominal pain.. The LTB4 level was determined, using a commercial enzyme-linked immunosorbent assay (ELISA), in urine samples obtained from 36 patients with acute (< 24 hours) right lower abdominal pain presenting to the emergency department, and from 18 healthy volunteers.. Compared with the healthy control subjects, LTB4 was significantly higher in those who had FMF (12 patients, p < 0.03). In other forms of acute right lower abdominal pain, including appendicitis (eight patients), urologic disorders (eight patients), and nonspecific abdominal pain (eight patients), intermediate levels of LTB4 were observed, not significantly different from those of either FMF patients or healthy control subjects.. In the samples tested, urine LTB4 levels were not instrumental in differentiating FMF from other acute right lower abdominal pain.

Topics: Abdominal Pain; Acute Disease; Adult; Appendicitis; Diagnosis, Differential; Familial Mediterranean Fever; Female; Humans; Leukotriene B4; Male; Middle Aged; Reference Values; Sensitivity and Specificity

Neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic, cutaneous, and articular syndrome is an autoinflammatory disease characterized by urticarial rash, arthropathy, and central nervous system inflammation.. To describe a 13-year-old girl with overlapping symptoms of NOMID and Muckle-Wells syndrome who has a mutation in cryopyrin (NALP3).. We examined neutrophil migration using transwell assay and time-lapse videomicroscopy. We also examined p38 mitogen-activated protein kinase (MAPK) activation in patient and control neutrophils using Western blot analysis.. Neutrophil defects in chemotactic migration were found to a variety of chemoattractants, including interleukin 8, N-formyl-methionyl-leucyl-phenylalanine, complement C5a, and leukotriene B4. Her neutrophils exhibited elevated basal and stimulated p38 MAPK activation in response to interleukin 8, N-formyl-methionyl-leucyl-phenylalanine, complement C5a, and leukotriene B4.. This study is the first, to our knowledge, to demonstrate defects in neutrophil chemotaxis and p38 MAPK signaling in a patient with NOMID and Muckle-Wells syndrome and a cryopyrin mutation.

Topics: Adolescent; Carrier Proteins; Chemotaxis, Leukocyte; Complement C5a; Familial Mediterranean Fever; Female; Humans; Inflammation; Interleukin-8; Leukotriene B4; Mutation; N-Formylmethionine Leucyl-Phenylalanine; Neutrophils; NLR Family, Pyrin Domain-Containing 3 Protein; p38 Mitogen-Activated Protein Kinases

The level of LTB4 and LTC4 in blood plasma of patients with familial mediterranean fever is significantly higher than in healthy donors: 53 + 10 pg/ml for LTB4 (normal - 25 + 5 pg/ml) and 175 + 22 pg/ml for LTC4 (normal 67 + 19 pg/ml). The more increase of the LTB4 and LTC4 content in plasma is observed during attacks of fever - 107 + 21 pg/ml (LTB4) and 249 + 34 pg/ml (LTC4). Hyperbaric oxygenation of patients, used to relieve pain and fever, reduces the level of leukotrienes.

Topics: Colchicine; Familial Mediterranean Fever; Humans; Hyperbaric Oxygenation; Leukotriene B4; SRS-A