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עמוד בית
Thu, 18.07.24

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May 2012
A. Zamora-Ustaran, R.O. Escarcega-Alarcón, M. Garcia-Carrasco, E. Faugier, S. Mendieta-Zeron, C. Mendoza-Pinto, Á. Montiel-Jarquin, M. Muñoz-Guarneros, A. Lopez-Colombo and R. Cervera

Background: Data on pediatric antiphospholipid syndrome (APS) are very sparse.

Objectives: To describe the main clinical characteristics, laboratory data and complications of pediatric APS patients, and to analyze the differences between primary APS and APS associated with systemic lupus erythematosus (SLE).

Methods: We retrospectively reviewed clinical and laboratory data of 32 children at “Federico Gomez,” the children’s hospital of México. Nineteen patients had SLE, 12 (37.5%) had primary APS and 1 (3%) had immune thrombocytopenic purpura. We collected information on sociodemographic variables, vaccinations, age at onset, and family history of rheumatic disease, hematological disorders, skin disorders and non-thrombotic neurological disorders. Immunological features included immunoglobulin (Ig) G and M aCl antibodies, IgG and IgM b2 glycoprotein I, lupus anticoagulant, anti-dsDNA and antinuclear antibodies.

Results: The patients included 24 females and 8 males. The most common thrombotic events were small vessel thrombosis (44%), venous thrombosis (28%) mainly deep venous thrombosis (DVT) in lower extremities, and arterial thrombosis (25%). The most common clinical non-thrombotic manifestations were hematological (53%) and neurological disorders (22%). There were no significant differences between groups with regard to the site of thrombosis, non-thrombotic clinical manifestations or laboratory features.

Conclusions: There were some important differences between the clinical manifestations of APS in children compared with adults, but we found no significant differences between patients with primary and APS associated with SLE. Larger studies in Latin American APS children are necessary to determine whether there are differences between ethnic groups.

 


April 2012
U. Arad, E. Niv, D. Caspi and O. Elkayam

Monogenic periodic fever syndromes are characterized by recurrent episodes of fever, accompanied by localized inflammatory manifestations. Among them, familial Mediterranean fever (FMF) is the most studied and is by far the most prevalent periodic fever syndrome in Israel. We present a diagnostic workup of a patient suffering from a periodic fever syndrome, initially thought to be FMF and characterized by attacks of fever, severe abdominal pain, a migratory erythematous rash and conjunctivitis. The development of periorbital edema presenting as diplopia led to consideration of tumor necrosis factor receptor-1-associated periodic syndrome (TRAPS). Genetic tests confirmed the diagnosis. This case should alert us that even in Israel, a patient with periodic fever, not fully consistent with the typical features of FMF, should be evaluated for other periodic fever syndromes.

December 2011
A. Tenenbaum, P. Hertz, T. Dor, Y. Castiel, A. Sapir and I.D. Wexler

Background: Maternal exposure to alcohol during pregnancy can lead to a wide range of clinical manifestations in their offspring, termed fetal alcohol spectrum disorder (FASD). In Israel, relatively few cases of FASD have been diagnosed and the prevalence has not been systematically evaluated.

Objectives: To determine the number of children with FASD or at risk for FASD in a select population of high risk patients seen at a clinic evaluating foster and adopted children.

Methods: Israeli children under 2 years old who were candidates for domestic adoption or in foster care were prospectively evaluated for clinical manifestations of FASD, and information was obtained regarding parental use of alcohol or other illicit drugs.

Results: Of the 100 patients prospectively evaluated, 8 had mothers with a known history of alcohol consumption during pregnancy. Two of the children had fetal alcohol syndrome (FAS) without known maternal exposure to alcohol and two had partial FAS. Eleven other children were at risk for development of one of the diagnostic categories of FASD.

Conclusions: In a population of pre-adoption and foster children, 15% either had manifestations of FASD or were at risk for developing FASD. Although this is a select high risk population, the data from this study strongly suggest a greater prevalence of FASD than previously assumed. Under-diagnosis of FASD is detrimental to affected children who could benefit from interventions designed to meet the needs of FASD victims.
 

October 2011
September 2011
E. Avitan-Hersh, G. Berger and R. Bergman
August 2011
B. Knyazer, J. Levy, E. Rosenberg, T. Lifshitz and I. Lazar
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