calca-protein--human and Familial-Mediterranean-Fever

It is well known that the serum procalcitonin (PCT) levels increase in severe bacterial infections. However, there is little information about the levels of PCT in diverse diseases except mainly the infectious diseases. The aim of this study was to investigate the progress of serum levels of PCT together with traditional acute phase reactants in children with familial Mediterranean fever (FMF) during the attack and attack-free periods and to test whether PCT could help to diagnose the attack in FMF patients. The study group comprised 21 FMF patients (mean age 10 ± 4.6 years) and 19 healthy controls (mean age 10.6 ± 4.2 years). Serum levels of PCT and traditional acute phase reactants were measured during the attack and attack-free periods. Blood samples were obtained within the first 6-24 h of the attack period, 7 days later, and at least 2 months after the attack. Traditional acute phase reactants (hs-CRP, ESR, and fibrinogen) during the attack period were significantly higher than the attack-free levels and controls. PCT levels of the FMF patients during the attack period were also significantly higher than the attack-free and control group levels (median values, 0.044 ng/ml vs. 0.028 ng/ml and 0.031 ng/ml, P = 0.04, respectively). Although this difference was statistically significant (P = 0.04), median PCT values of the attack, attack-free period, and healthy subjects were lower than 0.05 ng/ml. As a result, these findings suggested that PCT levels were not conspicuously affected from inflammation and could not be used as a descriptive marker for attack in FMF patients.

Topics: Acute-Phase Proteins; Adolescent; Biomarkers; C-Reactive Protein; Calcitonin; Calcitonin Gene-Related Peptide; Child; Child, Preschool; Familial Mediterranean Fever; Female; Humans; Male; Protein Precursors

This study was conducted to define the value of procalcitonin (PCT) levels in the differential diagnosis of abdominal familial Mediterranean fever (FMF) attacks from acute appendicitis. From October 2006 to January 2007, 28 FMF (12 males, 16 females) patients with acute abdominal attacks and 34 patients (18 males) with acute abdomen who underwent operation with the clinical diagnosis of acute appendicitis were consecutively enrolled in this study. FMF patients with concurrent infectious diseases were excluded. PCT values were measured by an immunofluorescent method using the B.R.A.H.M.S. PCT kit (B.R.A.H.M.S. Diagnostica, Berlin, Germany). Erythrocyte sedimentation rate (ESR), C-reactive proteins (CRP) and leucocyte levels were also noted. Mean disease duration in FMF patients was 9.6 +/- 8.1 years (range 2-33 years) and all were on colchicine therapy with a mean colchicine dosage of 1.2 +/- 0.4 mg/day. Among the operated patients, 5 were excluded: 3 patients had normal findings and 2 had intestinal perforation (PCT levels were 2.69 and 4.93 ng/ml, respectively) at operative and pathologic evaluation. There were no significant differences between the two groups with respect to gender and age (p was not significant (NS) for all). Acute phase reactants and PCT levels were increased in patients with FMF compared to patients with acute appendicitis (0.529[0.12 +/- 0.96] vs 0.095 [0.01-0.80] p < 0.001, respectively). PCT levels higher than 0.5 ng/ml were found in 11% (3/28) of FMF patients compared to 62% (18/29) of acute appendicitis patients (p < 0.001). Our results suggest that PCT could be a useful test in the differentiation of abdominal FMF attacks from acute appendicitis, though it should not supplant more conventional investigations.

Topics: Abdomen, Acute; Adult; Appendicitis; Biomarkers; Calcitonin; Calcitonin Gene-Related Peptide; Diagnosis, Differential; Familial Mediterranean Fever; Female; Fluoroimmunoassay; Glycoproteins; Humans; Male; Protein Precursors; Reproducibility of Results; Retrospective Studies

Classical type hyper-immunoglobulin D (IgD) syndrome (HIDS) is an hereditary auto-inflammatory disorder, characterized by recurrent episodes of fever, lymphadenopathy, abdominal distress and a high serum concentration of IgD. It is caused by mevalonate kinase deficiency.. To further characterize the acute phase response during fever attacks in HIDS in order to improve diagnosis.. Twenty-two mevalonate kinase-deficient HIDS patients.. Blood samples were drawn during and in between febrile attacks, and concentrations ofC-reactive protein (CRP), ferritin, procalcitonin, pentraxin 3, IgD and cholesterol in several lipoprotein fractions were determined.. The marked acute phase response at the time of a fever attack in classical type HIDS is reflected by a rise in CRP accompanied by a moderate but statistically significant rise in procalcitonin and pentraxin 3. In only two of 22 patients, procalcitonin concentration rose above 2 ng mL(-1) during fever attack, compatible with the noninfectious nature of these attacks. Ferritin does not reach the high concentrations found in adult-onset Still's disease. Despite the defect in mevalonate kinase, a component of cholesterol metabolism, serum cholesterol did not change during attacks. IgD concentration is elevated regardless of disease activity, although there is appreciable variation during life. Its role in HIDS remains unclear.. The combination of high CRP concentration plus procalcitonin concentration <2 ng mL(-1) in a symptomatic HIDS patient might indicate a febrile attack without (bacterial) infection; this observation warrants further investigation for its usefulness as a marker in clinical practice.

Topics: Acute-Phase Reaction; Adolescent; Adult; Biomarkers; C-Reactive Protein; Calcitonin; Calcitonin Gene-Related Peptide; Cholesterol; Familial Mediterranean Fever; Female; Humans; Hypergammaglobulinemia; Immunoglobulin D; Male; Middle Aged; Protein Precursors; Serum Amyloid P-Component