الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Juvenile Idiopathic Arthritis (JIA) is a heterogeneous group of disorders of autoinflammatory nature that begin before the age of 16 year sharing arthritis as a clinical manifestation.
According to ACR classification criteria, three main disease subtypes are identified; oligoarticular, polyarticular and systemic onset JIA.[2]
The present study was mainly concerned with anemia as one of the commonest rheumatoid comorbidities; having a prevalence that ranges between 30 and 70%.[40]
Although anemia affecting rheumatoid patients is mainly AI, other types may exist; depending on the disease stage and treatments used with IDA being the most common amongst. [40]
Being a common cause of both short & long-term morbidity, beside the fact that it is treatable, has made diagnosis and treatment of IDA of great medical relevance.[40]
Unfortunately, in the presence of concomitant chronic illness or inflammation, the diagnosis of iron deficiency, using the conventional laboratory parameters, is a major medical challenge. Here, outstands the necessity of introducing newer diagnostic tools serving this purpose. Two of them are serum hepcidin level and reticulocyte hemoglobin content (CHr).[43]
Hepcidin, in its active form, is a 25-amino acid peptide hormone, synthesized mainly by the liver. It is now considered the key negative iron regulator as well as an acute phase reactant. Hepcidin, itself, is regulated by at least four different mechanisms. Inflammation and erythropoiesis are considered the most related to the current study.[52]
The aim of the present work was to study serum level of hepcidin and the hemoglobin content of reticulocytes (CHr) in children with JIA, assessing their utility in diagnosing and differentiating various types of anemia, possibly found in such patients with concomitant acute phase response. The study also aimed to find out any possible relation between these two parameters and disease activity.
In this context, the study was conducted on a total number of 63 children whose ages ranged between 3 and 16 years, divided into two main groups:
• group I: included 43 patients with documented diagnosis of JIA
• group II: included 20 healthy age & sex matched children with no history of anemia or other chronic illnesses.
All JIA patients were selected on a random basis from those attending the clinic of pediatric rheumatology of AUCH as well as those admitted to the inpatient wards.
from all subjects of the present study, blood samples were collected between 7:00 and 9:00 a.m., at least 4 weeks after blood transfusion or iron therapy. All cases with apparent infection were excluded.
JIA patients included in this study were subjected to:
• Detailed history taking, especially emphasizing on disease onset, course, symptomatology and management; taking into consideration iron therapy and blood transfusion.
• Thorough clinical examination with special emphasis on possible manifestations of JIA and anemia.
• Disease activity assessment; using DAS28.
Both patients and control were subjected to laboratory investigations that included:
• CBC including chr.
• Iron profile; serum iron, TIBC, TSAT% and serum ferritin.
• Acute phase response parameters; ESR and CRP.
• Liver function tests; PT, serum albumin, ALT and AST.
• Special laboratory test to assess serum hepcidin level by ELISA technique, using (DRG International Inc., USA).
Regardless the disease subtype, the mean age at onset of JIA in the studied patients was 5.68 ± 3.65 (years) with 1.7:1 female to male ratio.
Except for serum ferritin and TIBC, significant differences were found, between the two main studied groups, in almost all laboratory parameters related to anemia and disease activity.
In a great similarity to serum ferritin, serum hepcidin levels (ng/mL) showed a much wider range in patient group compared to the control yet the difference in between didn’t reach the value of statistical significance (p= 0.695).
Contrarily, the mean chr (pg) was significantly lower in patients than control (p< 0.001).
CHr showed a highly significant correlations with all CBC parameters related to anemia except serum ferritin & TIBC while hepcidin correlated significantly to neither except serum ferritin.
As regards disease activity parameters, hepcidin and chr both had significant correlations with the counts of total WBCs and neutrophils as well as CRP.
Serum ferritin was used as a gold standard with different cut-offs set according to the presence or absence of acute phase response. Accordingly, the patient group was subdivided into two main categories; depleted and replete iron stores.
Anemia, regardless the type, was found in 51.2% of the studied patients. Moreover, three main types of anemia could be identified; IDA, ACD and combined IDA/ACD.
Comparing different studied laboratory parameters among the three anemia subgroups & the control group revealed the following:
The mean chr was found significantly lower in IDA and Combined IDA/ACD than the control group (p< 0.001). Compared to ACD subgroup, significantly lower chr was found only in IDA/ACD (p= 0.032) with no significant difference found; comparing IDA either to IDA/ACD or ACD.
In regard to serum hepcidin, none of the three subgroups differed significantly from the control yet significantly lower hepcidin was found in IDA compared to both IDA/ACD & ACD subgroups (p= 0.032 and 0.034, respectively).
The diagnostic performance of both serum hepcidin and chr were tested. Serum hepcidin at ≤ 3.8ng/mL was found 80% sensitive and 88.24% specific; discriminating between JIA patients with IDA and those with ACD & IDA/ACD while chr at ≤ 24.4pg differentiated IDA/ACD from ACD with 80% sensitivity and 100% specificity.
Finally, we could conclude that chr and serum hepcidin, together, may offer help discriminating different types of anemia in patients with JIA.