L Harel

Schneider Children's Medical Center of Israel, Petah Tikva, Central District, Israel

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Publications (79)285.44 Total impact

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    ABSTRACT: Introduction. Periodic fever, aphthous stomatitis, pharyngitis and cervical adenopathy (PFAPA) is an autoinflammatory syndrome characterized by periodic fever with aphthous stomatitis, cervical lymphadenopathy, myalgia, and abdominal pain. Peripheral blood concentrations of selected cytokines of PFAPA patients during and between febrile episodes were analyzed in a search for PFAPA-specific molecular signature. Methods. 23 children with PFAPA (age 6.07 ± 2.94 years, range 5-9 years) and three control children with severe oropharyngeal infections (age 6.2 ± 7.95 years, range 1-17 years) participated in the study. Peripheral blood concentrations of IL-1β, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, IFN-γ, GM-CSF, TNF-α were measured using Luminex technology. Results. PFAPA febrile episodes were characterized by detection of GM-CSF - 134.07 ± 315.5 pg/mL; significant (P < 0.001), compared to baseline and controls, elevation of concentrations of IL-8 (3193.7 ± 2508 pg/mL vs. 100.36 ± 119. pg/mL vs. 2.04 ± 4.08 pg/mL, respectively), IL-6 (1355.38 ± 2026.53 pg/mL vs. 28.8 ± 44.2 pg/mL and 27.13 ± 26.42 pg/mL, respectively). IL-1β was detected only in febrile and afebrile PFAPA patients (922.8 ± 1639 pg/mL vs. 10.98 ± 19.4 pg/ml, P < 0.002, respectively), but not in controls. Peripheral blood concentration of TNFα did not differ significantly between study groups. IL-2, IL-4, IL-5, and IL-10 were negligible in all study subjects. Discussion. PFAPA febrile episodes are characterized by activation of GM-CSF and IL-8 with Th1 skewing. We propose a molecular mechanism governing this phenomenon.
    Modern Rheumatology 03/2014; · 1.72 Impact Factor
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    ABSTRACT: Background/Purpose:In our previous prospective study we have demonstrated that the administration of Dexamethasone as an adjuvant therapy lead to a significant clinical and laboratory improvement in children with septic arthritis. This therapy has become accepted in some of the pediatric wards at Schneider's medical center. The aim of this study was to evaluate the effect of adding Dexamethasone to antibiotic therapy on the clinical course of septic arthritis in children not under controlled study.Methods:A retrospective study included all children hospitalized with the diagnosis of septic arthritis at Schneider Children's Medical Center from 2008–2013 .Part of the patients were treated with antibiotics alone, whereas others were treated with Dexametasone in addition to antibiotics, according to the policy of the admitting ward.Results:116 medical records were included in our study. Of which, 26 (22.4%) patients were treated with Dexamethasone and antibiotics and 90(77.6%) were treated with antibiotics only. There was no significant difference between the groups in age, duration of symptoms prior to hospitalization, body temperature or levels of acute phase reactants. Bacteria were isolated from 23 patients (20%) without any difference between the two groups. Patients treated with Dexamethasone had a significantly shorter duration of fever (mean 1st day without fever 2.3 vs. 3.9) (p = 0.002), shorter duration of parenteral antibiotic treatment (mean of 7.1 vs. 11.4 days) (p < 0.001) and accelerated clinical improvement (mean 1stday without pain or any limitation in physical examination 6.3 vs. 10 days)(p<0.001).Likewise, C-reactive protein (CRP) levels declined faster below 1mg/dL in the Dexamethasone group (mean of 5.3 vs. 8.4 days) (p = 0.002) and the hospital stay was significantly shorter (mean of 8 vs. 10.7 days) (p = 0.004). No side effects of treatment were recorded in either group, but in the Dexamethasone group a short rebound of symptoms occurred in 3/26 (11.5%) patients after completing the course of steroids.Conclusion:A short course of Dexamethasone given early, in addition to antibiotics, leads to a significant clinical and laboratory improvement, shortens duration of treatment and accelerates recovery in children with septic arthritis.
    Arthritis & Rheumatology. 03/2014; 66(S11).
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    ABSTRACT: Background Polyarteritis nodosa is a systemic necrotizing vasculitis with a pathogenesis that is poorly understood. We identified six families with multiple cases of systemic and cutaneous polyarteritis nodosa, consistent with autosomal recessive inheritance. In most cases, onset of the disease occurred during childhood. Methods We carried out exome sequencing in persons from multiply affected families of Georgian Jewish or German ancestry. We performed targeted sequencing in additional family members and in unrelated affected persons, 3 of Georgian Jewish ancestry and 14 of Turkish ancestry. Mutations were assessed by testing their effect on enzymatic activity in serum specimens from patients, analysis of protein structure, expression in mammalian cells, and biophysical analysis of purified protein. Results In all the families, vasculitis was caused by recessive mutations in CECR1, the gene encoding adenosine deaminase 2 (ADA2). All the Georgian Jewish patients were homozygous for a mutation encoding a Gly47Arg substitution, The German patients were compound heterozygous for Arg169Gln and Pro251Leu mutations, and one Turkish patient was compound heterozygous for Gly47Val and Trp264Ser mutations. In the endogamous Georgian Jewish population, the Gly47Arg carrier frequency was 0.102, which is consistent with the high prevalence of disease. The other mutations either were found in only one family member or patient or were extremely rare. ADA2 activity was significantly reduced in serum specimens from patients. Expression in human embryonic kidney 293T cells revealed low amounts of mutant secreted protein. Conclusions Recessive loss-of-function mutations of ADA2, a growth factor that is the major extracellular adenosine deaminase, can cause polyarteritis nodosa vasculopathy with highly varied clinical expression. (Funded by the Shaare Zedek Medical Center and others.).
    New England Journal of Medicine 02/2014; · 54.42 Impact Factor
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    ABSTRACT: Cutaneous Polyarteritis Nodosa (cPAN) was first described in 1931. cPAN is considered a rare disease, its true incidence is unknown. The age of onset is diverse. Most studies have shown no significant gender predominance. cPAN presents with distinct skin findings, such as a maculopapular rash, subcutaneous nodules, livedoid vasculitis, panniculitis, ischemic finger lesions, or erythematous patchy rash. Etiology is unclear. It is still believed to be an immune complex-mediated disease, although a possible mechanism recently proposed relates a familial form of the disease to impaired activity of Adenosine Deaminase 2. cPAN may reflect an underlying disease, infection or medical treatment. There is no consensus as to initial treatment, dosage and length of treatment. Patients with constitutional symptoms, visceral involvement, a more severe course of the disease, or high acute phase reactants, were treated mainly with systemic corticosteroids and/or cytotoxic agents for varying durations. However, persistence of cutaneous lesions has been documented. We describe a 14 year old male suffering from persistent cPAN, with no constitutional symptoms or involvement of internal organs. The patient was treated with a local corticosteroid-based ointment during exacerbations, until complete remission. Although reported in only one study, treatment with topical corticosteroid compound may result in significant improvement or complete regression of skin lesions in cPAN patients.
    Pediatric rheumatology online journal. 01/2014; 12:46.
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    Pediatric Rheumatology 07/2012; 10(1). · 1.47 Impact Factor
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    ABSTRACT: Catastrophic antiphospholipid syndrome (APS) in pediatric medicine is rare. We report 3 adolescents who presented with acute onset of severe abdominal pain as the first manifestation of probable catastrophic APS. The 3 patients, 2 male patients and 1 female patient were 14 to 18 years old. One had been diagnosed with systemic lupus erythematosus in the past, but the other 2 had no previous relevant medical history. All presented with excruciating abdominal pain without additional symptoms. Physical examination was noncontributory. Laboratory results were remarkable for high inflammatory markers. Abdominal ultrasonography was normal, and abdominal computed tomography scan showed nonspecific findings of liver infiltration. Only computed tomography angiography revealed evidence of extensive multiorgan thrombosis. All patients had elevated titers of antiphospholipid antibodies. The patients were treated with full heparinization, high-dose steroids, and intravenous immunoglobulin with a resolution of symptoms. One patient was resistant to the treatment and was treated with rituximab. In conclusion, severe acute abdominal pain can be the first manifestation of a thromboembolic event owing to catastrophic APS even in previously healthy adolescents. Diagnosis requires a high index of suspicion with prompt evaluation and treatment to prevent severe morbidity and mortality.
    PEDIATRICS 06/2012; 130(1):e230-5. · 4.47 Impact Factor
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    ABSTRACT: We evaluated the effect of adding dexamethasone to antibiotic therapy in the clinical course of septic arthritis in children. A randomized double-blind placebo-controlled trial was performed. The study group included 49 children with septicarthritis. In addition to antibiotic therapy given, patients were randomly assigned to receive intravenous dexamethasone 0.15 mg/kg every 6 hours for 4 days or placebo. The groups were compared for clinical and laboratory parameters, length of hospital stay, and late sequelae. Mean age was 33±42 months (range: 6 to 161 mo). There was no significant difference between the dexamethasone and placebo groups in age, duration of symptoms, joint affected, or levels of acute phase reactants. Bacteria were isolated from joint fluid in 17 patients (35%) and from blood in 4 patients. Compared with the placebo group, patients treated with dexamethasone had a significantly shorter duration of fever (P=0.021; mean first day without fever 1.68 vs 2.83) and local inflammatory signs (P=0.021; mean first day without pain 7.18 vs 10.76), lower levels of acute phase reactants (P=0.003; mean last day of erythrocyte sedimentation rate>25 mm/h 3.76 vs 8.40), shorter duration of parenteral antibiotic treatment (P=0.007; mean of 9.91 d vs 12.60 d), and shorter hospital stay. No side effects of treatment were recorded in either group. A 4-day course of dexamethasone given at the start of antibiotic treatment in children with septic arthritis, is safe, and leads to a significantly more rapid clinical improvement, shortening duration of hospitalization compared with those treated with antibiotics alone. I.
    Journal of pediatric orthopedics 03/2011; 31(2):211-5. · 1.23 Impact Factor
  • Liora Harel
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    ABSTRACT: Growing pains are common among children aged 2-12 years and are characterized by recurrent, self-limited, bilateral lower extremity pain, mostly in the afternoon and evening, and even at night, in an otherwise healthy child. The etiology is unknown and prognosis is excellent. It is important to distinguish between this benign condition and other serious rheumatic or malignant diseases.
    Pediatric endocrinology reviews: PER 12/2010; 8(2):76-8.
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    ABSTRACT: To perform a retrospective study comparing clinical and laboratory aspects of patients with acute rheumatic fever (ARF) and patients with post-streptococcal reactive arthritis (PSRA), to discern whether these are 2 separate entities or varying clinical manifestations of the same disease. We located the records of 68 patients with ARF and 159 patients with PSRA, whose diseases were diagnosed with standardized criteria and treated by 8 pediatric rheumatologists in 7 medical centers, using the Israeli internet-based pediatric rheumatology registry. The medical records of these patients were reviewed for demographic, clinical, and laboratory variables, and the data were compared and analyzed with univariate, multivariate, and discriminatory analysis. Four variables were found to differ significantly between ARF and PSRA and serve also as predictors: sedimentation rate, C-reactive protein, duration of joint symptoms after starting anti-inflammatory treatment, and relapse of joint symptoms after cessation of treatment. A discriminative equation was derived that enabled us to correctly classify >80% of the patients. On the basis of simple clinical and laboratory variables, we were able to differentiate ARF from PSRA and correctly classify >80% of the patients. It appears that ARF and PSRA are distinct entities.
    The Journal of pediatrics 11/2008; 153(5):696-9. · 4.02 Impact Factor
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    ABSTRACT: To assess the level of antibiotic resistance of viridans streptococci in the oral flora of children with a history of rheumatic fever, receiving long-term monthly intramuscular benzathine penicillin G prophylaxis. Oral swabs from patients receiving monthly penicillin G prophylaxis for rheumatic fever were cultured and tested for viridans streptococci. The E-test was used to test susceptibility to penicillin G, clindamycin, clarithromycin and rifampin. Findings were compared with samples from healthy children who had not been exposed to antibiotic treatment for at least 2 months. Twenty-six patients and 20 control children were included in the study. Duration of intramuscular antibiotic treatment ranged from 5 months to 13.5 years. Sixty isolates of viridans streptococci species were obtained, with a similar distribution in the two groups. Intermediate resistance to penicillin (MIC 0.25-2 mg/L) was documented in 10 of the 32 isolates (31.2%) in the study group, and high resistance in none, compared to seven of 28 isolates (25%) with intermediate or high resistance in the control group (p=NS). All isolates in the study group and all but one in the control group were susceptible to clindamycin, and all isolates from both groups were susceptible to rifampin. One isolate (3.1%) in the study group and two (7.1%) in the control group were resistant to clarithromycin. Monthly Intramuscular penicillin prophylaxis has no effect on the antibiotic susceptibility of viridans streptococci in oral flora in children with a history of rheumatic fever, receiving secondary prophylaxis after rheumatic fever, regardless of the duration of treatment.
    The Journal of infection 05/2008; 56(4):244-8. · 4.13 Impact Factor
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    ABSTRACT: Hypoalbuminemia is a common finding in children with massive parapneumonic pleural effusion; however, its incidence and pathogenesis are unclear. The objective of this study was to assess the presence and severity of hypoalbuminemia in children with parapneumonic pleural effusion and to propose a possible pathophysiologic mechanism. The clinical charts of patients who were hospitalized in a tertiary pediatric center with bacterial pneumonia complicated by pleural effusion were reviewed. The volume of pleural fluid was assessed semiquantitatively and categorized as small, moderate, or large. The lowest serum albumin level was recorded, and caloric intake and protein loss were evaluated. Findings were compared with age- and gender-matched children who had bacterial pneumonia without pleural effusion and with children who had acute illnesses other than pneumonia. Of the 50 patients in the study group, 15 (30%) had small effusions, 16 (32%) had moderate effusions, and 19 (38%) had large effusions. Moderate-to-severe hypoalbuminemia was found in 52% of the study group, 6% of the patients with pneumonia without pleural effusion, and none of the patients with other illnesses. Mean serum albumin level was lower in patients with large pleural effusions than in patients with small effusions (2.66 +/- 0.37 vs 3.66 +/- 0.47 g/dL). There was no evidence of albumin loss or significant malnutrition. Estimation of the amount of albumin in the drained pleural fluid suggested an albumin shift from blood to pleural fluid. Significant hypoalbuminemia is common in children with parapneumonic pleural effusion. Large effusions are associated with low serum albumin levels, which might be explained in part by a shift from blood to pleural fluid.
    PEDIATRICS 04/2008; 121(3):e533-8. · 4.47 Impact Factor
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    ABSTRACT: Acne can be associated with severe musculoskeletal and constitutional symptoms. This is a case history of a 15 year-old boy with severe acne, treated with isotretinoin, who was admitted because of high fever and weight loss of 3 weeks duration. His complaints were of severe chest and back pain, and inability to walk. His laboratory results revealed elevated erythrocyte sedimentation rate and C-reactive protein. On a pelvic radiograph bilateral sacroiliitis was noticed. Radionuclide bone scan revealed increased uptake in the right sacroiliac joint, in small vertebral bodies and sternum. A diagnosis of acne-associated musculoskeletal syndrome was made and the patient was treated with high dose steroids. His symptoms resolved and his acne improved. This case report sheds light on the relationship between acne and associated musculoskeletal syndromes. Physicians should be aware of this association in order to provide prompt and efficient treatment.
    Harefuah 02/2008; 147(1):12-5, 96.
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    ABSTRACT: Intra-articular corticosteroid injection in juvenile idiopathic arthritis (JIA) is often associated with anxiety and pain. Recent reports advocate the use of nitrous oxide (NO), a volatile gas with analgesic, anxiolytic and sedative properties. To prospectively evaluate the effectiveness and safety of NO analgesia for intra-articular corticosteroid injection in JIA, and to assess patients and staff satisfaction with the treatment. NO was administered to JIA patients scheduled for joint injection. The patient, parent, physician and nurse completed visual-analog scores (VAS) (0-10) for pain, and a 5-point satisfaction scale. Change in heart rate (HR) during the procedure was recorded in order to examine physiologic response to pain and stress. Patient's behavior and adverse reactions were recorded. 54 procedures (72 joints) were performed, 41 females, 13 males; 39 Jewish, 13 Arab; mean age was 12.2 +/- 4.7 year. The median VAS pain score for patients, parents, physicians and nurses was 3. The HR increased >/= 15% in 10 patients. They had higher VAS scores as evaluated by the staff. The median satisfaction level of the parents and staff was 3.0 and 5.0 respectively. Adverse reactions were mild. NO provides effective and safe sedation for JIA children undergoing intra-articular injections.
    Pediatric Rheumatology 01/2008; 6:1. · 1.47 Impact Factor
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    ABSTRACT: The incidence of Haemophilus influenzae type B (HIB) infection, once the most common cause of periorbital cellulitis, declined dramatically after the introduction of HIB vaccine in 1990. The aim of the current study was to determine the predisposing factors and agents in the pathogenesis of periorbital cellulitis in hospitalized children in the post-HIB vaccination era. Children with clinical findings of periorbital inflammation who were hospitalized in a tertiary pediatric hospital in Israel in 2000-2001 were observed prospectively. Special attention was directed to the predisposing medical condition in each case. One hundred sixty-three patients had a final discharge diagnosis of periorbital cellulitis. Mean age was 34 months (median = 24 months). The predisposing conditions were conjunctivitis (42.9%), infected wound or trauma (20.9%), insect bites (9.8%), sinusitis (8%), dacryostenosis (4.9%), and Streptococcus pneumoniae bacteremia (0.6%). Children with conjunctivitis and sinusitis had the most severe inflammatory signs. None of the cultures was positive for HIB, although only 71% of the children had complete immunization. The epidemiology of periorbital cellulitis in children has changed in the post-HIB vaccine era. The most common predisposing medical conditions are conjunctivitis or an infected wound in the vicinity of the eye. Bacteremia is rarely a source of the disease. These findings have important clinical implications in terms of choice of treatment.
    Journal of Pediatric Ophthalmology & Strabismus 01/2008; 45(5):300-4. · 0.86 Impact Factor
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    ABSTRACT: An elevated expression of the alloantigen D8/17 on B lymphocytes has been previously proposed as a susceptibility marker in rheumatic fever. The aim of the study was to investigate the presence of the D8/17 marker on B lymphocytes in poststreptococcal reactive arthritis (PSRA). The study sample included 19 patients (15 boys, 4 girls; mean age 11.7 +/- 5.4 years) who were diagnosed with PSRA (mean age at diagnosis, 9.4 +/- 5.6 years) and 18 healthy controls (10 boys and 8 girls) matched for ethnic background. B-cell D8/17 expression was tested by flow cytometry assay using monoclonal antibodies. Laboratory results showed a higher expression of D8/17 in the patient group (23.1 +/- 10.4%, range 11.6-51.9%) than in the control group (17.1 +/- 8.2%, range 7.1-35.7) in controls; this difference was statistically significant after log transformation of the data (P = 0.035). The high rate of expression of the D8/17 marker in patients with PSRA suggests that RF and PSRA share the same genetic susceptibility.
    Rheumatology International 07/2007; 27(8):695-8. · 2.21 Impact Factor
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    ABSTRACT: Folate is essential for normal brain development. This report describes a 15-month-old boy who presented with generalized and focal seizures and a decline in mental status. Laboratory tests revealed low folate levels in blood (1.13 nmol/L) and cerebrospinal fluid, accompanied by pancytopenia. Bone marrow aspiration confirmed the presence of megaloblastic anemia. Treatment with high-dose intravenous folinic acid led to normalization of cerebrospinal folate levels. These findings apparently indicate a defect in folic acid transport to the central nervous system. A clinical picture of developmental arrest, seizures, somnolence, and megaloblastic anemia should alert physicians to the possibility of folate deficiency.
    Journal of Child Neurology 07/2007; 22(6):783-6. · 1.39 Impact Factor
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    ABSTRACT: Calcinosis is a devastating complication of juvenile dermatomyositis and a challenging therapeutic problem. We report the use of an external Ilizarov fixator for the treatment of Achilles tendon calcinosis causing severe disability in a young girl with juvenile dermatomyositis.
    Clinical and experimental rheumatology 01/2007; 25(5):763-5. · 2.66 Impact Factor
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    ABSTRACT: To present an analysis of patients with protracted febrile myalgia (PFM), a rarely reported manifestation of familial Mediterranean fever (FMF), and propose clinical criteria for working diagnosis. A multicenter retrospective cohort study of children with PFM was performed. Clinical and laboratory data were obtained by medical record review. The study group included 15 patients with PFM. PFM occurred as the presenting sign of FMF in 33%. FMF was diagnosed clinically in all and by genetic analysis in 93%. M694V allelic involvement was noted in 93% of the patients. PFM occurred at a mean age of 9 +/- 3.4 years and was characterized by severe generalized muscle pain in all patients and fever in 71%. Mean duration up to diagnosis was 15.5 +/- 6 days. Mean erythrocyte sedimentation rate was 104 +/- 26 mm/h; mean C-reactive protein was 15.4 +/- 6.3 mg%. Creatine kinase was normal. Treatment included corticosteroids (4 patients) and nonsteroidal anti-inflammatory drugs (NSAIDs) (9 patients) with a symptomatic relief achieved at a mean of 7.7 +/- 4.3 days and 5 +/- 3.8 days, respectively (p = 0.14) (mean severity score 3 and 2.2, respectively, p = 0.075). Symptomatic relief in 2 untreated patients was achieved at a mean of 45.5 days. Based on our data, we propose criteria for working diagnosis including: severe disabling myalgia of at least 5 days in a young patient with FMF, associated with fever, elevated levels of inflammatory markers and presence of at least one M694V mutation.
    Clinical and experimental rheumatology 01/2007; 25(4 Suppl 45):S114-7. · 2.66 Impact Factor
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    ABSTRACT: Few studies have addressed antiphospholipid syndrome (APS) among children. Our aims were to analyze the clinical and laboratory manifestations in a pediatric APS cohort and to assess the influence of inherited thrombophilia factors on the outcome of children with APS. This was a multicenter study of children with APS who had no previous systemic autoimmune disease. We retrospectively reviewed their clinical and laboratory data, including hereditary thrombophilic deficits and outcomes. The cohort comprised 28 patients (17 females, mean +/- SD age at onset 10.6 +/- 6.1 years). The most common initial manifestations of APS were venous thrombosis, stroke, and thrombocytopenia. Lupus anticoagulant was detected in 96% of those tested. After a mean +/- SD followup of 5.7 +/- 4.8 years, 16 children (57.1%) had central nervous system disease, 9 exhibited hematologic involvement, and 5 (all females) had systemic lupus erythematosus (SLE). None had renal, heart, or new skin disease. Seven of 24 patients exhibiting vascular thrombotic events had recurrences. Infants with perinatal stroke had monophasic disease, and other manifestations of APS did not develop later. Hereditary thrombophilia was more common in children who experienced a single episode of APS (8 [53.3%] of 15 patients) than in those who experienced recurrences (2 [28.6%] of 7 patients). However, only 2 patients in the latter group (28.6%) received anticoagulants after the first manifestation, compared with 12 (70.6%) of the 17 patients without recurrences. APS in children has unique features. SLE may develop in a significant percentage of girls presenting with APS. Hereditary thrombophilia did not predict recurrent thrombosis, whereas the preventive impact of anticoagulant treatment following the first thrombotic event was noteworthy.
    Arthritis & Rheumatology 01/2007; 55(6):850-5. · 7.48 Impact Factor
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    ABSTRACT: The aim of this study was to describe the clinical manifestations and outcomes of a national cohort of childhood systemic lupus erythematosus (cSLE). All cases of cSLE registered in the Israeli national registry of children with rheumatic diseases between 1987-2003 were examined for disease activity and damage by the SLE disease activity index (SLEDAI) and SLE collaborating clinics/American College of Rheumatology (SLICC/ACR) damage index. Demographic, clinical, laboratory and treatment factors were analysed for their effect on the outcome. One-hundred and two patients were identified, 81% females, with a mean age at diagnosis of 13.3 +/- 2.6 years. The mean SLEDAI score was 17.2 +/- 9.0 (range 2-60). Fifty four patients were followed for at least five years. The mean SLEDAI decreased to 7.6 +/- 6.3 (0-29) and the mean SLICC/ACR damage index was 0.7 +/- 1.6 (0-8). Five patients developed chronic renal failure. No patients died. No factors were found to be significantly associated with the outcome except the initial SLEDAI score. The five-year outcome of our national cSLE cohort was good; with relatively low activity and minimal damage in most patients. The initial SLEDAI predicted the development of late damage.
    Lupus 01/2007; 16(2):142-6. · 2.78 Impact Factor