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

Unusual presentation of juvenile psoriatic arthritis: a case report

Several recent surveys of arthritis in children that used similar diagnostic criteria have confirmed that Juvenile Psoriatic Arthritis account for approximately 7 percent of all cases of chronic childhood arthritis. It is uncommon for the disease to begin before the age of one year. We report a 5 month-old male infant who satisfied the clinical criteria for the diagnosis of Juvenile Psoriatic Arthritis. To the best of our knowledge this is the first case to be diagnosed in the first year of age in Jordan

comparison of efficiency and safety of a single dose of intramuscular dexamethasone acetate with oral prednisolone in the management of asthma exacerbations in children

To determine wether a single intramuscular dexamethasone acetate injection is as effective as five days of oral prednisolone in improving symptoms and preventing relapse in children with acute asthma. This is a prospective quasi, randomized, and non-blinded trial. We enrolled 30 children, aged 9 months to 14 years with known history of asthma who presented to the emergency department with moderate exacerbation. The primary outcome was to measure the rate of relapse, persistance, worsening, recurrence of symptoms and hospitalization in 5 and 21 days after discharge from emergency department. All patients completed the study. More patients in the prednisolone group continued to use bronchodilator than patients in the dexamethasone group [three patients in the former compared to one patient in the second group]. No patient in both groups had severe replase or needed hospitalization. Patients in the prednisolone group experienced difficulty in administration of the tablets and 3 of them missed 50-75% of the total dose. There were no significant local or systemic adverse effects in both groups. All patients in dexamethasone group were satisfied with the treatment while most parents in the prednisolone group preferred an alternative form of treatment. We found that IM dexamethasone acetate is more clinically effective than a 5-day course of oral prednisolone tables in treatment of moderate asthma exacerbations. It was well tolerated, and preferred by most parents. we recommend it as an alternative option especially in those who are not compliant to oral prednisolone