Zeinab Elsayed

Serum Anti-Myelin—Associated Glycoprotein Antibodies in Egyptian Autistic Children

Autoimmunity to brain could play an etiopathogenic role in a subgroup of autistic patients. The frequency of serum anti-myelin-associated glycoprotein antibodies, as an index for autoimmunity to brain, and their relation to family history of autoimmunity were investigated in 32 autistic and 32 healthy matched children. Autistic children had significantly higher serum anti-myelin-associated glycoprotein antibodies than healthy children (2100 [1995] and 1138 [87.5] Buhlmann titre unit, P < .001). Anti-myelin-associated glycoprotein positivity was elicited in 62.5% of autistic children. Family history of autoimmunity in autistic children (50%) was significantly higher than controls (9.4%). Anti-myelin-associated glycoprotein serum levels were significantly higher in autistic children with than those without such history (P < .05). In conclusion, autism could be, in part, one of the pediatric autoimmune neuropsychiatric disorders. Further studies are warranted to shed light on the etiopathogenic role of anti-myelin-associated glycoprotein antibodies and the role of immunotherapy in autism.

Cardiovascular autonomic function assessed by autonomic function tests and serum autonomic neuropeptides in Egyptian children and adolescents with rheumatic diseases

OBJECTIVE Cardiovascular autonomic neuropathy (CAN) in patients with rheumatic diseases may result in sudden death, possibly from arrhythmia and myocardial infarction due to its frequent association with microvascular disease. Autonomic dysfunction may contribute to initiation and perpetuation of rheumatic diseases. Thus, we aimed to assess cardiovascular autonomic function in lupus and juvenile idiopathic arthritis (JIA) patients. METHODS Assessment of cardiovascular autonomic function was done in 20 lupus and 20 JIA patients, aged 8-16 years, by five non-invasive autonomic function tests (AFTs) and serum levels of neuropeptide Y (NPY) and vasoactive intestinal peptide (VIP), as indicators of sympathetic and parasympathetic functions, respectively, in comparison with 40 matched healthy control subjects. RESULTS Clinical evidence of CAN was found in 65 and 40% of lupus and JIA patients, respectively, and in none of healthy controls. Lupus and JIA patients had significantly lower serum NPY and VIP than controls (P < 0.001). The five AFTs score had significant negative correlations to NPY and VIP (P < 0.001). Patients with CAN had significantly lower serum NPY and VIP than patients without (P < 0.001). Clinical evidence of CAN was found in 41.7 and 14.3% of asymptomatic lupus and JIA patients, respectively. There was significant positive association between CAN and important disease manifestations, including activity, in these patients. CONCLUSIONS CAN is common in lupus and JIA patients, even in absence of relevant symptoms. Thus, assessments of cardiac autonomic function, by AFTs and serum autonomic neuropeptides (NPY and VIP), and the therapeutic effects of NPY and VIP are recommended in these patients.

Evaluation of banana hypersensitivity among a group of atopic egyptian children: relation to parental/self reports.

PURPOSE To evaluate the frequency of banana sensitization and allergy among a group of atopic Egyptian children in relation to parental/self reports. METHODS This is a case-control study included 2 groups of allergic children with and without history of banana allergy, each included 40 patients. They were subjected to skin prick test (SPT) using commercial banana allergen extract and prick-prick test (PPT) using raw banana, in addition to measuring the serum banana-specific IgE. Oral banana challenge was performed in suspected cases. RESULTS Banana allergy was diagnosed in 3 (7.5%) patients based on positive history of allergy on exposure to banana, positive SPT/PPT and elevated banana-specific IgE. The 3 patients had bronchial asthma with exacerbation upon banana exposure. The PPT results conform with those of SPT both in diagnosis of banana allergy and in the skin reactivity to banana. Serum banana-specific IgE was detectable in the whole studied sample with higher serum level among those without history of banana allergy (P=0.005). Oral banana challenge was negative for 20 patients with history of banana allergy and positive serum banana-specific IgE but negative SPT and PPT. CONCLUSIONS Self/parental reports of banana allergy is high while the actual banana allergy is uncommon. The PPT seems as reliable as SPT in diagnosis of banana allergy unlike specific IgE which reflects sensitization rather than allergy. Oral food challenge remains the most helpful tool for diagnosis of food allergy in suspected cases.

A Rare Association Between Leukocyte Adhesion Deficiency Type I and Psoriasis in Humans

The β2 integrins are expressed exclusively on leukocytes and participate in many immune and inflammatory processes. This subfamily comprises four heterodimeric glycoproteins with a common β-subunit, designated β2 (CD18). Spontaneous mutations of the CD18 gene result in leukocyte adhesion deficiency type I (LAD-I). Low level of CD18 expression has also been implicated in the pathogenesis of psoriasis. We here describe a child with recurrent skin infections without pus formation, persistent gingivitis and periodontitis. His blood counts showed persistent leukocytosis (neutrophilia). CD11b expression was defective on neutrophils, while that of CD18 was normal. So, our patient represents a mild variant of LAD-I with possible dysfunctional CD18. Moreover, he developed psoriasis with reduced CD18 expression on CD4+ T-cells. Psoriasiform dermatitis has been described before in association with LAD-I, however, clinically and histologically confirmed psoriasis in association with LAD-I has been described only in CD18 hypomorphic mice. Therefore, our patient represents the first clinically and histopathologically documented association between LAD-I and psoriasis in humans. It lends support to the role of β2 integrins in the etiopathogenesis of psoriasis.

Poliovirus Excretion among Persons with Primary Immune DeficiencyDisorders: Summary of Data from Enhanced Poliovirus Surveillance in Egypt,2011-2014

Background: If exposed to oral poliovirus vaccine (OPV), persons with primary immune deficiency disorders (PID) are at increased risk of paralytic poliomyelitis; and can chronically excrete poliovirus. However, the risk of excretion of vaccine derived poliovirus among immunodeficient persons (iVDPV) is not well characterized. We present summary of data from poliovirus surveillance project among PID patients collected between 2011 and 2014 from 11 Egyptian Governorates. Methods: Stool was tested for polioviruses in suspected or confirmed PID children regardless of whether Acute Flaccid Paralysis (AFP) was present or not. Those excreting poliovirus were followed until three consecutive negative stool samples were obtained. Results: There were 122 patients with suspected or confirmed PID identified; 13/122 (11%) excreted poliovirus; of these, 6 excreted iVDPVs, the remaining 7 excreted Sabin virus. The duration of iVDPV excretion ranged from 1 to 21 months. AFP was detected in 3/6 (50%) of those excreting iVDPVs. All iVDPV excretors had history of receiving OPV. Conclusions: Chronic poliovirus excretion in PID patients is rare, however, poliovirus eradication requires removal of all polioviruses from circulation; and because PID individuals are not necessarily paralyzed they might be missed by current poliovirus surveillance based on detection of AFP. To achieve poliovirus eradication, surveillance for polioviruses among PID patients should be routinely conducted in all countries, and poliovirus antiviral therapy must be made available for those with chronic excretion.

Alpha beta double negative T cells in children with systemic lupus erythematosus: The relation to disease activity and characteristics

Abstract Objectives: We aimed to investigate alpha beta double negative (αβ DN) T-cell percentages in juvenile systemic lupus erythematosus (JSLE) and their relation to disease activity and organ involvement. Methods: This prospective study was carried out over a period of 12 months and included 21 JSLE patients and 20 healthy matched controls. SLE disease activity index 2000 (SLEDAI-2K) scores were recorded in addition to αβ DN T-cell percentages measurement at enrollment and after remission. Results: Enrolled patients (n = 21) were females with age range 10–17 years. Patients were followed up for a duration ranging 5–9 months. αβ DN T-cell percentages were higher in cases during activity [median (IQR): 3.7 (3.0–5.7)] versus remission [median (IQR): 1.4 (1.2–1.8)] and during activity and remission versus healthy controls [median (IQR): 1.0 (0.5–1.4)]. αβ DN T-cell percentages correlated positively with SLEDAI-2K score (p < .001). The mean αβ DN T-cell percentages varied significantly with different degrees of activity per SLEDAI-2K score (p = .002) and with the presence of neurological (p = .028) and hematological (p = .032) manifestations. Conclusion: αβ DNT cells percentages are elevated in patients with JSLE and their percentages correlate with disease activity. Further studies are needed in conjunction with the proinflammatory cytokine profile, apoptotic assays and histological findings.

Giant Cell Arteritis in a 12-Year-Old Girl Presenting with Nephrotic Syndrome

Giant cell arteritis (GCA) is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities.

Management of Low and Intermediate Risk Adult Rhabdomyosarcoma: A Pooled Survival Analysis of 553 Patients

This is the second-largest retrospective analysis addressing the controversy of whether adult rhabdomyosarcoma (RMS) should be treated with chemotherapy regimens adopted from pediatric RMS protocols or adult soft-tissue sarcoma protocols. A comprehensive database search identified 553 adults with primary non-metastatic RMS. Increasing age, intermediate-risk disease, no chemotherapy use, anthacycline-based and poor chemotherapy response were significant predictors of poor overall and progression-free survival. In contrast, combined cyclophosphamide-based, cyclophosphamide + anthracycline-based, or cyclophosphamide + ifosfamide + anthracycline-based regimens significantly improved outcomes. Intermediate-risk disease was a significant predictor of poor chemotherapy response. Overall survival of clinical group-III patients was significantly improved if they underwent delayed complete resection. Non-parameningeal clinical group-I patients had the best local control, which was not affected by additional adjuvant radiotherapy. This study highlights the superiority of chemotherapy regimens –adapted from pediatric protocols- compared to anthracycline-based regimens. There is lack of data to support the routine use of adjuvant radiotherapy for non-parameningeal group-I patients. Nonetheless, intensive local therapy should be always considered for those at high risk for local recurrence, including intermediate-risk disease, advanced IRS stage, large tumors or narrow surgical margins. Although practically difficult (due to tumor’s rarity), there is a pressing need for high quality randomized controlled trials to provide further guidance.

Publisher Correction: Management of Low and Intermediate Risk Adult Rhabdomyosarcoma: A Pooled Survival Analysis of 553 Patients

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Is fertility-preservation safe for adult non-metastatic gynecologic rhabdomyosarcoma patients? Systematic review and pooled survival analysis of 137 patients

PurposeRecently, conservative approaches such as wide local excisions and neoadjuvant chemotherapy are being considered to select young adult females with gynecologic RMS who have strong desire to preserve fertility. This analysis aims to identify prognosticators affecting survival outcomes and defining potential candidacy for fertility-preservation. Another focus is to explore the role of chemotherapy in reducing the need for aggressive surgery and the role of radiotherapy in decreasing rates of local failure.MethodsA comprehensive database search identified 137 females > 16 years old with primary non-metastatic gynecologic RMS, who were included in a multivariate survival analysis.Results5-year overall survival rate was 65%. Patients < 50 years old, with cervix uteri primaries, well-defined/polypoid presentations, embryonal histology and superficial tumors were more likely to survive. Deeply invasive disease and alveolar/pleomorphic histology significantly increased risks of treatment failure and tumor recurrence. Chemotherapy use was a significant multivariate predictor of better overall and metastasis-free survival. Radical surgery did not add local control or overall survival benefit for patients with superficial lesions (minimal/no cervical stromal invasion and no myometrial invasion).ConclusionsWhile high-quality clinical trial evidence is missing, existing evidence seems to support holding back on radical surgery for selected candidates with well-defined, polypoid, superficial, embryonal cervical/endometrial RMS lesions that could be completely excised with conservative surgery; further local resections with/without radiotherapy are then warranted based on margin status. Experience on the use of neoadjuvant chemotherapy in the conservative management of uterine RMS in adults is very limited, though this approach is golden-standard in pediatrics. A suggested scheme is introduced for the management of uterine RMS.