Physical Medicine, Rheumatology and Rehabilitation
Aya Hamed Mohamed;
Abstract
J
uvenileIdiopathicArthritisisthemostfrequentchronicrheumaticdiseaseinchildhood.MASisacomplicationofJIAwithelevatedmorbidityandmortality.
MASappearstooccurwithvaryingdegreesofseverity,rangingfromthehighlymorbidchildwithpersistenthighgradefever,hepatosplenomegaly,bleedingtendency,andCNSmanifestations.Laboratoryparametersshowingpancytopenia,coagulopathy,hepatic,andrenaldisorderstotheunwellchildwithpersistentfever,noorganomegaly,arelativedropinbloodcellcounting,andmildcoagulopathy.
Earlyrecognition,differentiationfromactivityoftheJIAitselfandtherapidintroductionofaggressivetreatmentcontributetobetterprognosis.TheprogressinunderstandingthepathophysiologybehindMASandidentificationofthepathwaysassociatedwiththeearlystagesofthissyndromecandevelopnewbiomarkersandtreatmentofMAS.
TheaimofthisstudyistoinvestigatethepresenceofmacrophageactivationsyndromeamongJIApatientstoassesstheextentoftheprobleminthosepatients.WeincludedinourstudyonehundredJIApatientsregisteredinpediatricdepartmentsovertheprevious2years.DiagnosisofMASwasdoneaccordingtopreliminarydiagnosticguidelinesforMAScomplicatingJIA.
uvenileIdiopathicArthritisisthemostfrequentchronicrheumaticdiseaseinchildhood.MASisacomplicationofJIAwithelevatedmorbidityandmortality.
MASappearstooccurwithvaryingdegreesofseverity,rangingfromthehighlymorbidchildwithpersistenthighgradefever,hepatosplenomegaly,bleedingtendency,andCNSmanifestations.Laboratoryparametersshowingpancytopenia,coagulopathy,hepatic,andrenaldisorderstotheunwellchildwithpersistentfever,noorganomegaly,arelativedropinbloodcellcounting,andmildcoagulopathy.
Earlyrecognition,differentiationfromactivityoftheJIAitselfandtherapidintroductionofaggressivetreatmentcontributetobetterprognosis.TheprogressinunderstandingthepathophysiologybehindMASandidentificationofthepathwaysassociatedwiththeearlystagesofthissyndromecandevelopnewbiomarkersandtreatmentofMAS.
TheaimofthisstudyistoinvestigatethepresenceofmacrophageactivationsyndromeamongJIApatientstoassesstheextentoftheprobleminthosepatients.WeincludedinourstudyonehundredJIApatientsregisteredinpediatricdepartmentsovertheprevious2years.DiagnosisofMASwasdoneaccordingtopreliminarydiagnosticguidelinesforMAScomplicatingJIA.
Other data
| Title | Physical Medicine, Rheumatology and Rehabilitation | Other Titles | حدوث متلازمة تنشيط البلاعم في التهاب مفاصل الأطفال مجهول السبب | Authors | Aya Hamed Mohamed | Issue Date | 2017 |
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