ABSTRACT
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
ABSTRACT
To describe the clinical experience of a single center in severe combined Immunodeficiency. a total of 14 patients who were admitted to the Pediatric Department at King Hussein Medical Center with a probable diagnosis of severe combined immunodeficiency over 6 years duration were studied. The study descried patients' population, clinical, family history, laboratory and radiological investigations, treatment options and prognosis. Twelve [86 percent] patients were males and two [14 percent] were females. Median age at presentation was 21 weeks. They all showed the same clinical presentation with few exceptions. We demonstrated maternofetal engraftment in two [14 percent] patients. Unusual post vaccination poliomyelitis type II was demonstrated in one patient. Skin abscess and deep-seated ulcers were seen in one patient. One patient with Viral Associated Hemophagocytosis Syndrome Dead siblings with similar illness were retrieved in most patients had anemia, lymphopnia, and severe pan-hypogammaglobulinemia at time of presentation. We confirmed neutropenia in only three [21 percent] patients. Blood cultures revealed heavy growth of Klebsiella and Pseudomonas organisms in nine [64 percent] patients. We found that intravenous immunoglobulins administration was partially useful. Bone marrow transplant was not done for any patient. One patient was under preparation for autologous bone marrow transplantation. Eleven patients died after a mean of four months after diagnosis. Two patients were in a relatively acceptable condition, and that last one was suffering from fulminant sepsis. The awareness of the referring physicians to immunodeficiency is sub optimal. Pediatricians are urged to pay attention to persistent Lymphopenia as a helpful clue for the diagnosis of severe combined Immunodeficiency in infants with unusual infections especially in families with positive family history. Live attenuated viral or bacterial immunization is contradicted in suspected immunodeficiency. Health education of families is an essential part of management of these patients