Spectrum of pediatric systemic lupus erythematosus at Queen Rania Al-Abdullah hospital for children

The aim of this study is to describe clinical signs, symptoms, laboratory characteristics, and medication used in pediatric Systemic lupus Erythematosus, both at presentation and during the course of the disease in Jordanian children at Queen Rania Al-Abdulla Hospital for children. This is a retrospective descriptive study that included patients managed over a period of 11 years, from January 2000 to December 2010. The charts of 25 patients from the pediatric Rheumatology unit at Queen Rania Al-Abdulla Hospital for children, who met four or more of the revised American College of Rheumatology classification criteria for Systemic lupus Erythematosus were reviewed. There were 22 females and three males with F: M ratio of 7.3:1. The mean age at diagnosis was 10.9 years [range 7-14 years] with only five patients [20%] below the age of 10 years. The mean time from the start of illness to diagnosis was 8.6 month [range 1-36 months]. At presentation cutaneous manifestations were found in 17 [68%] patients, 60% of patients had arthritis. Serositis and neurological manifestations were seen in 24% of cases. Hematological dysfunctions were present in 48%. Renal involvement was found in 40% of cases. Kidney biopsy was done for seven patients with renal manifestations. Three had class IV, two class III and two class I World Health Organization nephritis stage classification. No organ damage was found in 18 patients with Systemic lupus Erythematosus, while three patients developed end stage renal disease, two had neuropsychiatric disease [one cerebrovascular accident and one with chorea], one had cataract and one had peripheral vascular thrombosis, and gangrene of the hands and feet. Antinuclear Antibodies was positive in all patients. To the best of our knowledge this is the first review of Systemic lupus Erythematosus in pediatric population in Jordan. Comparison of our cohort with other reports from our region and other parts of the world confirmed that more or less the pediatric Systemic lupus Erythematosus behavior in presentation and laboratory findings is comparable

Juvenile dermatomyositis king hussein medical center experience

Juvenile dermatomyositis [JDM] is an uncommon, often chronic, and potentially serious childhoodsystemic autoimmune vasculopathy affecting primarily skin and muscles. It is characterized bypathognomonic rash, and symmetrical proximal muscle weakness.In this retrospective study, we reviewed the clinical, laboratory profiles, treatment and outcome ofJordanian children diagnosed with JDM in the past 8 years in a tertiary facility in Amman, Jordan.Sixteen [16] JDM patients, diagnosed based on criteria of Bohan and Peter, and have attended thepediatric rheumatology clinic in King Hussein Medical Center, from January 2006 to September 2013,were recruited. Their medical records were studied for clinical and biochemical profile, radiological andelectrophysiological data were studied as well. Treatment and outcome were also reviewed.Our cohort includes 16 patients, 9 [56%] males, and 7 [44%] females [M: F 1.3:1], their age rangesbetween 2 to 9 years, with average age at diagnosis of 5.4 years. Time to diagnosis varies from 2 monthsto 12 months, and averages at 4.6 months. Proximal muscle weakness was present at time of diagnosis in14 [87.5%] cases. Cutaneous signs in form of either poikiloderma in malar distribution, Gottron's signand /or heliotrope sign were apparent in all the 16 [100%] patients at time of diagnosis, periungualerythema was evident in 10 [63%] patients while abnormal nailbed capillaries pattern was only reported in6 [3 8%] cases.Skin and soft tissue calcification, crusting and ulceration were seen in 3 [19%] patients. Serum Lactatedehydrogenase, [LDH] was elevated in 94% and creatininine phosphokinase [CPK] in 88%. ElevatedSGOT [AST] was seen in almost all subjects, while ESR was high in 14 [87.5%] patients. All patientstreated with corticosteroids and methotrexate [MTX].Two [12.5%] patients died in our series, complete remission was achieved in 4[25%] of patients, whilepartial remission was seen in 8 [50%] of patients in our cohort.JDM is a rare disease that has the potential to cause physical disability, poor functional outcome, anddeath if not recognized early and treated properly. We focused in our study, on importance of earlyreferral, and aggressive therapy in improving outcome, aiming to increase awareness of families andgeneral pediatricians

Application of flow cytometry in the diagnosis of primary immunodeficiency disease [PID]: King Hussein Medical Center experience

To highlight some of the significant applications of flow cytometric immunophenotyping in the diagnosis of Primary Immunodeficiency Disease. We reviewed the medical records of 135 consecutive patients who were referred to the Immunology Clinic at King Hussein Medical Center with a flow cytometry based diagnosis of Primary Immunodeficiency Disease between January 2000 to August 2009. The medical records of fl5 patients with history of recurrent or persistent infections were reviewed. Seventy seven [57%] patients were males and 58[43%] were females. They aged between 2 and 120 months with a mean age of 13 months. Flow cytomerty-based diagnosis was identified in 68 [50.3%] patients. Predominant antibody deficiency was diagnosed in 114 [10.3%] patients. There were 35[26%] patients with T and B cell immunodeficiency. There were 6 patients' satisfied diagnostic criteria of possible HyperlgM lmmunodeficiency syndrome. Diagnosis of severe combined immunodeficiency was retrieved in 22[16.2%] patients. Primary phagocytic disorder was the diagnosis in 34 [25%] patients. Dihydrorhodamine flow cytomerty-based burst test was confirmatory for Chronic Granulomatous Diseases in one patient while in the other 14 patients diagnosis was based on nitroblue tetrazoleoum test and genetic mutation study. There were 8 [6%] patients with other well defined immunodeficiency syndromes; one patient with Wiskott Aldrich Syndrome, 5 patients with Ataxia Telangectasia, one with Bloom syndrome, and one with DiGeorge anomaly. Eight [6%] patients were found to have an immunedysregulation syndrome. There were 8[6%] patients with an undefined primary immunodeficiency. Post Bone marrow transplantation Immunereconstitution of T-, B-cells and Leukocyte adhesion molecules were identified in 14 patients with appropriate Flow cytomerty immunophenotyping assay. Flow cytometric immunophenotyping of leucocytes appears to be an efficient and rapid tool in the diagnosis and follow-up of immunodeficient patients, supporting early recognition, which is reflected on reduced morbidity and improved survival