Shereen Reda - Academia.edu (original) (raw)

Papers by Shereen Reda

The Egyptian Journal of Pediatric Allergy and Immunology, Oct 3, 2020

Journal of Clinical Immunology, 2021

Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), rep... more Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), represent a heterogeneous group of genetic disorders that affect the development and/or function of cells of the immune system. These disorders manifest with increased susceptibility to infection, autoimmunity, autoinflammatory diseases, allergy, and/or malignancy. Unfortunately, the awareness of PIDs, worldwide, is low among physicians and general practitioners because PIDs are considered relatively rare and complex diseases. Consequently, many patients remain undiagnosed or experience delayed diagnosis leading to increased morbidity and mortality during childhood or later on in adulthood. Purpose: This oral presentation will demonstrate a systematic approach on when to suspect and how to diagnose PID in infants and children laying stress on symptoms and signs suggestive of these disorders, as well as the basic and advanced laboratory tests that are essential for proper diagnosis. Also, the importance of timely referral of suspected PID cases to specialized centers to confirm diagnosis by other more advanced laboratory and genetic tests and design the proper management plan will be highlighted.

Clinical and Translational Allergy, 2020

Reported COVID-19 deaths in Germany are relatively low as compared to many European countries. Am... more Reported COVID-19 deaths in Germany are relatively low as compared to many European countries. Among the several explanations proposed, an early and large testing of the population was put forward. Most current debates on COVID-19 focus on the differences among countries, but little attention has been given to regional differences and diet. The low-death rate European countries (e.g. Austria, Baltic States, Czech Republic, Finland, Norway, Poland, Slovakia) have used different quarantine and/or confinement times and methods and none have performed as many early tests as Germany. Among other factors that may be significant are the dietary habits. It seems that some foods largely used in these countries may reduce angiotensin-converting enzyme activity or are anti-oxidants. Among the many possible areas of research, it might be important to understand diet and angiotensin-converting enzyme-2 (ACE2) levels in populations with different COVID-19 death rates since dietary interventions m...

Allergy, 2020

Large differences in COVID-19 death rates exist between countries and between regions of the same... more Large differences in COVID-19 death rates exist between countries and between regions of the same country. Some very low death rate countries such as Eastern Asia, Central Europe or the Balkans have a common feature of eating large quantities of fermented foods. Although biases exist when examining ecological studies, fermented vegetables or cabbage were associated with low death rates in European countries. SARS-CoV-2 binds to its receptor, the angiotensin converting enzyme 2 (ACE2). As a result of SARS-Cov-2 binding, ACE2 downregulation enhances the angiotensin II receptor type 1 (AT 1 R) axis associated with oxidative stress. This leads to insulin resistanceas well as lung and endothelial damage, two severe outcomes of COVID-19. The nuclear factor (erythroid-derived 2)-like 2 (Nrf2) is the most potent antioxidant in humans and can block the AT 1 R axis. Cabbage contains precursors of sulforaphane, the most active natural activator of Nrf2. Fermented vegetables contain many lactobacilli, which are also potent Nrf2 activators. Three examples are given: Kimchi in Korea, westernized foods and the slum paradox. It is proposed that fermented cabbage is a proof-of-concept of dietary manipulations that may enhance Nrf2associated antioxidant effects helpful in mitigating COVID-19 severity.

The Journal of infectious diseases, 2014

Persons with primary immune deficiency disorders (PID), especially those disorders affecting the ... more Persons with primary immune deficiency disorders (PID), especially those disorders affecting the B-cell system, are at substantially increased risk of paralytic poliomyelitis and can excrete poliovirus chronically. However, the risk of prolonged or chronic excretion is not well characterized in developing countries. We present a summary of a country study series on poliovirus excretion among PID cases. Cases with PID from participating institutions were enrolled during the first year and after obtaining informed consent were tested for polioviruses in stool samples. Those cases excreting poliovirus were followed on a monthly basis during the second year until 2 negative stool samples were obtained. A total of 562 cases were enrolled in Bangladesh, China, Iran, Philippines, Russia, Sri Lanka, and Tunisia during 2008-2013. Of these, 17 (3%) shed poliovirus, including 2 cases with immunodeficient vaccine-derived poliovirus. Poliovirus was detected in a single sample from 5/17 (29%) cas...

Journal of Allergy and Clinical Immunology, 2014

Background-The recombination-activating gene (RAG) 1/2 proteins play a critical role in the devel... more Background-The recombination-activating gene (RAG) 1/2 proteins play a critical role in the development of T and B cells by initiating the VDJ recombination process that leads to generation of a broad T-cell receptor (TCR) and B-cell receptor repertoire. Pathogenic mutations in the RAG1/2 genes result in various forms of primary immunodeficiency, ranging from T − B − severe combined immune deficiency to delayed-onset disease with granuloma formation, autoimmunity, or both. It is not clear what contributes to such heterogeneity of phenotypes. Objective-We sought to investigate the molecular basis for phenotypic diversity presented in patients with various RAG1 mutations. Methods-We have developed a flow cytometry-based assay that allows analysis of RAG recombination activity based on green fluorescent protein expression and have assessed the induction of the Ighc locus rearrangements in mouse Rag1 −/− pro-B cells reconstituted with wildtype or mutant human RAG1 (hRAG1) using deep sequencing technology. Results-Here we demonstrate correlation between defective recombination activity of hRAG1 mutant proteins and severity of the clinical and immunologic phenotype and provide insights on the molecular mechanisms accounting for such phenotypic diversity. Conclusions-Using a sensitive assay to measure the RAG1 activity level of 79 mutations in a physiologic setting, we demonstrate correlation between recombination activity of RAG1 mutants and the severity of clinical presentation and show that RAG1 mutants can induce specific abnormalities of the VDJ recombination process.

Brain and Development, 1999

We describe the prevalence and nature of gastrointestinal (GI) symptoms in 58 children affected b... more We describe the prevalence and nature of gastrointestinal (GI) symptoms in 58 children affected by cerebral palsy (range: from 6 months to 12 years of age) referred to a pediatric neurology outpatient clinic. In each patient we assessed (GI) symptoms and de®ned the associated GI functional or structural abnormalities. Furthermore, we tried to correlate the type of GI dysfunction with ®ndings on computed tomography (CT) or magnetic resonance imaging (MRI) of the brain. Our results showed that 92% of children with cerebral palsy had clinically signi®cant gastrointestinal symptoms. Swallowing disorders were present in 60% of patients, regurgitation and/or vomiting in 32%, abdominal pain in 32%, episodes of chronic pulmonary aspiration in 41% and chronic constipation in 74%. Dysfunction of the oral and/or pharyngeal phase of swallowing was found in 28 of 30 (93%) patients with swallowing disorders. Of the 45 patients with symptoms suggesting gastroesophageal re¯ux, 41 (91%) had an abnormal pH-monitoring and/ or esophagitis. Furthermore, a signi®cant delay in the scintigraphic gastric emptying of liquids was found in 12 of 18 patients (67%) and an abnormal esophageal motility in 11 of the 18 (61%) investigated patients. In 25 patients with chronic constipation evaluation of colonic transit showed a delay at level of the proximal segments of the colon in 13 (52%), at level of the left colon and rectum in 9 (36%) and in 3 (12%) at level of the rectum only. Computed tomography and/or magnetic resonance imaging were normal in 5 (9%) and abnormal in 53 (91%) of the 58 children with cerebral palsy. No GI symptom was signi®cantly associated with any kind of abnormal neuroimaging. In conclusion, children with cerebral palsy exhibited diffuse GI clinical manifestations, mostly due to disorders of GI motility. The GI symptoms seemed not to be related to any speci®c ®nding on CT or MRI of the brain.

Allergy, 2008

Neurogenic inflammation may participate in the development of chronic inflammatory airway disease... more Neurogenic inflammation may participate in the development of chronic inflammatory airway diseases such as bronchial asthma. The molecular mechanisms underlying neurogenic inflammation are orchestrated by a large number of neuropeptides mainly including tachykinins, such as neurokinin A (NKA) and substance P (SP) and calcitonin gene-related peptide (1). Tachykinins belong to a family of neuropeptides, which are released from nonadrenergic, noncholinergic excitatory nerves of airways (2) after exposure to allergens. They are also produced by inflammatory cells such as eosinophils (3, 4). Neurokinin A and SP have potent effects on bronchomotor tone, airway secretions and bronchial circulation. In view of their potent effects on the airways, tachykinins have been put forward as possible mediators of asthma, and tachykinin receptor antagonists are a potential new class of antiasthmatic medications (3, 5-7). Assessing biological markers of airway inflammation for proper diagnosis, monitoring and treatment of bronchial asthma is essential (8). Sputum induction is a noninvasive test that monitors airway inflammation. Sputum markers tend to be more sensitive than blood tests when assessing airway inflammation (9). With this background, we were stimulated to investigate sputum NKA levels in children and adolescents with acute asthma exacerbations of varying severity. Methods Study population This case-control, follow-up study was conducted on 24 asthmatic children and adolescents recruited from the

Journal of Allergy and Clinical Immunology, 2000

Journal of Allergy and Clinical Immunology, 2014

Background-SCID is a syndrome characterized by profound T cell deficiency. BCG vaccine is contrai... more Background-SCID is a syndrome characterized by profound T cell deficiency. BCG vaccine is contraindicated in SCID patients. Because most countries encourage BCG vaccination at birth, a high percent of SCID patients are vaccinated before their immune defect is detected. Objectives-To describe the complications and risks associated with BCG vaccination in SCID patients. Methods-An extensive standardized questionnaire evaluating complications, therapeutics, and outcome regarding BCG in patients diagnosed with SCID was widely distributed. Summary statistics and association analysis was performed. Results-Data on 349 BCG vaccinated SCID patients from 28 centers in 17 countries was analyzed. Fifty-one percent of the patients developed BCG complications, 34% disseminated and 17% localized (a 33,000 and 400 fold increase, respectively, over the general population). Patients receiving early vaccination (≤ 1 month) showed an increased prevalence of complications (p=0.006) and death due to BCG complications (p<0.0001). The odds of experiencing complications among patients with T cells ≤ 250/uL at diagnosis was 2.1 times higher (95% CI, 1.4-3.4; p = 0.001) than among those with T cells > 250/uL. BCG complications were reported in 2/78 patients who received anti-mycobacterial therapy while asymptomatic and no deaths due to BCG complications occurred in this group. In contrast 46 BCG-associated deaths were reported among 160 patients treated with anti-mycobacterial therapy for a symptomatic BCG infection (p<0.0001). Conclusions-BCG vaccine has a very high rate of complications in SCID patients, which increase morbidity and mortality rates. Until safer and more efficient anti-tuberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.

The Egyptian Journal of Pediatric Allergy and Immunology

Journal of Clinical Immunology

Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), rep... more Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), represent a heterogeneous group of genetic disorders that affect the development and/or function of cells of the immune system. These disorders manifest with increased susceptibility to infection, autoimmunity, autoinflammatory diseases, allergy, and/or malignancy. Unfortunately, the awareness of PIDs, worldwide, is low among physicians and general practitioners because PIDs are considered relatively rare and complex diseases. Consequently, many patients remain undiagnosed or experience delayed diagnosis leading to increased morbidity and mortality during childhood or later on in adulthood. Purpose: This oral presentation will demonstrate a systematic approach on when to suspect and how to diagnose PID in infants and children laying stress on symptoms and signs suggestive of these disorders, as well as the basic and advanced laboratory tests that are essential for proper diagnosis. Also, the importance of timely referral of suspected PID cases to specialized centers to confirm diagnosis by other more advanced laboratory and genetic tests and design the proper management plan will be highlighted.

La Tunisie medicale, 2018

INTRODUCTION Primary Immunodeficiency (PIDs) is a set of 330 rare hereditary diseases that increa... more INTRODUCTION Primary Immunodeficiency (PIDs) is a set of 330 rare hereditary diseases that increase susceptibility to infections, allergies, autoimmunity, and neoplasia. North American registries give higher prevalence than Maghreb ones, whereas consanguinity is high. The purpose of this study is to compare prevalence and coverage rate of Maghreb PID registries with estimates based on USA. METHODS We searched the prevalence of PIDs in the Maghreb registers. Next, we estimated the expected values based on recent publications. Finally, we calculated the coverage rate of the Maghreb registries compared to the new estimates and we evaluated the impact of consanguinity. RESULTS The total number is N1 = 2456 patients. The current Maghreb PID Prevalence is 2.56 / 100,000 inhabitants (population of 94,804,694 Million in 2017). Tunisia leads with a prevalence of 8.70 followed by Morocco 2.09, Libya 1.65 and Algeria 1.46/100.000 habitants. We did not find values for Mauritania. If we extrapol...

Egyptian Journal of Pediatric Allergy and Immunology, 2006

Common variable immunodeficiency (CVID) refers to a heterogeneous immunodeficiency syndrome chara... more Common variable immunodeficiency (CVID) refers to a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections and a variety of immunological abnormalities. Affected persons are prone to recurrent bacterial infections, especially involving the upper and lower respiratory tracts. In addition, patients exhibit increased susceptibility to a protean array of autoimmune, gastrointestinal, neoplastic, and inflammatory disorders .

The Egyptian Journal of Otolaryngology, 2014

The current study showed a relatively low incidence of IgA deficiency among children with ROM and... more The current study showed a relatively low incidence of IgA deficiency among children with ROM and indicated other environmental risk factors that participated in the occurrence of OM in our community.

Background. Persons with primary immune deficiency disorders (PID), especially those disorders af... more Background. Persons with primary immune deficiency disorders (PID), especially those disorders affecting the B-cell system, are at substantially increased risk of paralytic poliomyelitis and can excrete poliovirus chronically. However, the risk of prolonged or chronic excretion is not well characterized in developing countries. We present a summary of a country study series on poliovirus excretion among PID cases. Methods. Cases with PID from participating institutions were enrolled during the first year and after obtaining informed consent were tested for polioviruses in stool samples. Those cases excreting poliovirus were followed on a monthly basis during the second year until 2 negative stool samples were obtained. Results. A total of 562 cases were enrolled in Bangladesh, China, Iran, Philippines, Russia, Sri Lanka, and Tunisia during 2008-2013. Of these, 17 (3%) shed poliovirus, including 2 cases with immunodeficient vaccine-derived poliovirus. Poliovirus was detected in a single sample from 5/17 (29%) cases. One case excreted for more than 6 months. None of the cases developed paralysis during the study period. Conclusions. Chronic polioviruses excretion remains a rare event even among individuals with PID. Nevertheless, because these individuals were not paralyzed they would have been missed by current surveillance; therefore, surveillance for polioviruses among PID should be established.

The Egyptian Journal of Pediatric Allergy and Immunology, Oct 3, 2020

Journal of Clinical Immunology, 2021

Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), rep... more Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), represent a heterogeneous group of genetic disorders that affect the development and/or function of cells of the immune system. These disorders manifest with increased susceptibility to infection, autoimmunity, autoinflammatory diseases, allergy, and/or malignancy. Unfortunately, the awareness of PIDs, worldwide, is low among physicians and general practitioners because PIDs are considered relatively rare and complex diseases. Consequently, many patients remain undiagnosed or experience delayed diagnosis leading to increased morbidity and mortality during childhood or later on in adulthood. Purpose: This oral presentation will demonstrate a systematic approach on when to suspect and how to diagnose PID in infants and children laying stress on symptoms and signs suggestive of these disorders, as well as the basic and advanced laboratory tests that are essential for proper diagnosis. Also, the importance of timely referral of suspected PID cases to specialized centers to confirm diagnosis by other more advanced laboratory and genetic tests and design the proper management plan will be highlighted.

Clinical and Translational Allergy, 2020

Reported COVID-19 deaths in Germany are relatively low as compared to many European countries. Am... more Reported COVID-19 deaths in Germany are relatively low as compared to many European countries. Among the several explanations proposed, an early and large testing of the population was put forward. Most current debates on COVID-19 focus on the differences among countries, but little attention has been given to regional differences and diet. The low-death rate European countries (e.g. Austria, Baltic States, Czech Republic, Finland, Norway, Poland, Slovakia) have used different quarantine and/or confinement times and methods and none have performed as many early tests as Germany. Among other factors that may be significant are the dietary habits. It seems that some foods largely used in these countries may reduce angiotensin-converting enzyme activity or are anti-oxidants. Among the many possible areas of research, it might be important to understand diet and angiotensin-converting enzyme-2 (ACE2) levels in populations with different COVID-19 death rates since dietary interventions m...

Allergy, 2020

Large differences in COVID-19 death rates exist between countries and between regions of the same... more Large differences in COVID-19 death rates exist between countries and between regions of the same country. Some very low death rate countries such as Eastern Asia, Central Europe or the Balkans have a common feature of eating large quantities of fermented foods. Although biases exist when examining ecological studies, fermented vegetables or cabbage were associated with low death rates in European countries. SARS-CoV-2 binds to its receptor, the angiotensin converting enzyme 2 (ACE2). As a result of SARS-Cov-2 binding, ACE2 downregulation enhances the angiotensin II receptor type 1 (AT 1 R) axis associated with oxidative stress. This leads to insulin resistanceas well as lung and endothelial damage, two severe outcomes of COVID-19. The nuclear factor (erythroid-derived 2)-like 2 (Nrf2) is the most potent antioxidant in humans and can block the AT 1 R axis. Cabbage contains precursors of sulforaphane, the most active natural activator of Nrf2. Fermented vegetables contain many lactobacilli, which are also potent Nrf2 activators. Three examples are given: Kimchi in Korea, westernized foods and the slum paradox. It is proposed that fermented cabbage is a proof-of-concept of dietary manipulations that may enhance Nrf2associated antioxidant effects helpful in mitigating COVID-19 severity.

The Journal of infectious diseases, 2014

Persons with primary immune deficiency disorders (PID), especially those disorders affecting the ... more Persons with primary immune deficiency disorders (PID), especially those disorders affecting the B-cell system, are at substantially increased risk of paralytic poliomyelitis and can excrete poliovirus chronically. However, the risk of prolonged or chronic excretion is not well characterized in developing countries. We present a summary of a country study series on poliovirus excretion among PID cases. Cases with PID from participating institutions were enrolled during the first year and after obtaining informed consent were tested for polioviruses in stool samples. Those cases excreting poliovirus were followed on a monthly basis during the second year until 2 negative stool samples were obtained. A total of 562 cases were enrolled in Bangladesh, China, Iran, Philippines, Russia, Sri Lanka, and Tunisia during 2008-2013. Of these, 17 (3%) shed poliovirus, including 2 cases with immunodeficient vaccine-derived poliovirus. Poliovirus was detected in a single sample from 5/17 (29%) cas...

Journal of Allergy and Clinical Immunology, 2014

Background-The recombination-activating gene (RAG) 1/2 proteins play a critical role in the devel... more Background-The recombination-activating gene (RAG) 1/2 proteins play a critical role in the development of T and B cells by initiating the VDJ recombination process that leads to generation of a broad T-cell receptor (TCR) and B-cell receptor repertoire. Pathogenic mutations in the RAG1/2 genes result in various forms of primary immunodeficiency, ranging from T − B − severe combined immune deficiency to delayed-onset disease with granuloma formation, autoimmunity, or both. It is not clear what contributes to such heterogeneity of phenotypes. Objective-We sought to investigate the molecular basis for phenotypic diversity presented in patients with various RAG1 mutations. Methods-We have developed a flow cytometry-based assay that allows analysis of RAG recombination activity based on green fluorescent protein expression and have assessed the induction of the Ighc locus rearrangements in mouse Rag1 −/− pro-B cells reconstituted with wildtype or mutant human RAG1 (hRAG1) using deep sequencing technology. Results-Here we demonstrate correlation between defective recombination activity of hRAG1 mutant proteins and severity of the clinical and immunologic phenotype and provide insights on the molecular mechanisms accounting for such phenotypic diversity. Conclusions-Using a sensitive assay to measure the RAG1 activity level of 79 mutations in a physiologic setting, we demonstrate correlation between recombination activity of RAG1 mutants and the severity of clinical presentation and show that RAG1 mutants can induce specific abnormalities of the VDJ recombination process.

Brain and Development, 1999

We describe the prevalence and nature of gastrointestinal (GI) symptoms in 58 children affected b... more We describe the prevalence and nature of gastrointestinal (GI) symptoms in 58 children affected by cerebral palsy (range: from 6 months to 12 years of age) referred to a pediatric neurology outpatient clinic. In each patient we assessed (GI) symptoms and de®ned the associated GI functional or structural abnormalities. Furthermore, we tried to correlate the type of GI dysfunction with ®ndings on computed tomography (CT) or magnetic resonance imaging (MRI) of the brain. Our results showed that 92% of children with cerebral palsy had clinically signi®cant gastrointestinal symptoms. Swallowing disorders were present in 60% of patients, regurgitation and/or vomiting in 32%, abdominal pain in 32%, episodes of chronic pulmonary aspiration in 41% and chronic constipation in 74%. Dysfunction of the oral and/or pharyngeal phase of swallowing was found in 28 of 30 (93%) patients with swallowing disorders. Of the 45 patients with symptoms suggesting gastroesophageal re¯ux, 41 (91%) had an abnormal pH-monitoring and/ or esophagitis. Furthermore, a signi®cant delay in the scintigraphic gastric emptying of liquids was found in 12 of 18 patients (67%) and an abnormal esophageal motility in 11 of the 18 (61%) investigated patients. In 25 patients with chronic constipation evaluation of colonic transit showed a delay at level of the proximal segments of the colon in 13 (52%), at level of the left colon and rectum in 9 (36%) and in 3 (12%) at level of the rectum only. Computed tomography and/or magnetic resonance imaging were normal in 5 (9%) and abnormal in 53 (91%) of the 58 children with cerebral palsy. No GI symptom was signi®cantly associated with any kind of abnormal neuroimaging. In conclusion, children with cerebral palsy exhibited diffuse GI clinical manifestations, mostly due to disorders of GI motility. The GI symptoms seemed not to be related to any speci®c ®nding on CT or MRI of the brain.

Allergy, 2008

Neurogenic inflammation may participate in the development of chronic inflammatory airway disease... more Neurogenic inflammation may participate in the development of chronic inflammatory airway diseases such as bronchial asthma. The molecular mechanisms underlying neurogenic inflammation are orchestrated by a large number of neuropeptides mainly including tachykinins, such as neurokinin A (NKA) and substance P (SP) and calcitonin gene-related peptide (1). Tachykinins belong to a family of neuropeptides, which are released from nonadrenergic, noncholinergic excitatory nerves of airways (2) after exposure to allergens. They are also produced by inflammatory cells such as eosinophils (3, 4). Neurokinin A and SP have potent effects on bronchomotor tone, airway secretions and bronchial circulation. In view of their potent effects on the airways, tachykinins have been put forward as possible mediators of asthma, and tachykinin receptor antagonists are a potential new class of antiasthmatic medications (3, 5-7). Assessing biological markers of airway inflammation for proper diagnosis, monitoring and treatment of bronchial asthma is essential (8). Sputum induction is a noninvasive test that monitors airway inflammation. Sputum markers tend to be more sensitive than blood tests when assessing airway inflammation (9). With this background, we were stimulated to investigate sputum NKA levels in children and adolescents with acute asthma exacerbations of varying severity. Methods Study population This case-control, follow-up study was conducted on 24 asthmatic children and adolescents recruited from the

Journal of Allergy and Clinical Immunology, 2000

Journal of Allergy and Clinical Immunology, 2014

Background-SCID is a syndrome characterized by profound T cell deficiency. BCG vaccine is contrai... more Background-SCID is a syndrome characterized by profound T cell deficiency. BCG vaccine is contraindicated in SCID patients. Because most countries encourage BCG vaccination at birth, a high percent of SCID patients are vaccinated before their immune defect is detected. Objectives-To describe the complications and risks associated with BCG vaccination in SCID patients. Methods-An extensive standardized questionnaire evaluating complications, therapeutics, and outcome regarding BCG in patients diagnosed with SCID was widely distributed. Summary statistics and association analysis was performed. Results-Data on 349 BCG vaccinated SCID patients from 28 centers in 17 countries was analyzed. Fifty-one percent of the patients developed BCG complications, 34% disseminated and 17% localized (a 33,000 and 400 fold increase, respectively, over the general population). Patients receiving early vaccination (≤ 1 month) showed an increased prevalence of complications (p=0.006) and death due to BCG complications (p<0.0001). The odds of experiencing complications among patients with T cells ≤ 250/uL at diagnosis was 2.1 times higher (95% CI, 1.4-3.4; p = 0.001) than among those with T cells > 250/uL. BCG complications were reported in 2/78 patients who received anti-mycobacterial therapy while asymptomatic and no deaths due to BCG complications occurred in this group. In contrast 46 BCG-associated deaths were reported among 160 patients treated with anti-mycobacterial therapy for a symptomatic BCG infection (p<0.0001). Conclusions-BCG vaccine has a very high rate of complications in SCID patients, which increase morbidity and mortality rates. Until safer and more efficient anti-tuberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.

The Egyptian Journal of Pediatric Allergy and Immunology

Journal of Clinical Immunology

Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), rep... more Background: Inborn errors of immunity (IEI), also known as primary immunodeficiencies (PIDs), represent a heterogeneous group of genetic disorders that affect the development and/or function of cells of the immune system. These disorders manifest with increased susceptibility to infection, autoimmunity, autoinflammatory diseases, allergy, and/or malignancy. Unfortunately, the awareness of PIDs, worldwide, is low among physicians and general practitioners because PIDs are considered relatively rare and complex diseases. Consequently, many patients remain undiagnosed or experience delayed diagnosis leading to increased morbidity and mortality during childhood or later on in adulthood. Purpose: This oral presentation will demonstrate a systematic approach on when to suspect and how to diagnose PID in infants and children laying stress on symptoms and signs suggestive of these disorders, as well as the basic and advanced laboratory tests that are essential for proper diagnosis. Also, the importance of timely referral of suspected PID cases to specialized centers to confirm diagnosis by other more advanced laboratory and genetic tests and design the proper management plan will be highlighted.

La Tunisie medicale, 2018

INTRODUCTION Primary Immunodeficiency (PIDs) is a set of 330 rare hereditary diseases that increa... more INTRODUCTION Primary Immunodeficiency (PIDs) is a set of 330 rare hereditary diseases that increase susceptibility to infections, allergies, autoimmunity, and neoplasia. North American registries give higher prevalence than Maghreb ones, whereas consanguinity is high. The purpose of this study is to compare prevalence and coverage rate of Maghreb PID registries with estimates based on USA. METHODS We searched the prevalence of PIDs in the Maghreb registers. Next, we estimated the expected values based on recent publications. Finally, we calculated the coverage rate of the Maghreb registries compared to the new estimates and we evaluated the impact of consanguinity. RESULTS The total number is N1 = 2456 patients. The current Maghreb PID Prevalence is 2.56 / 100,000 inhabitants (population of 94,804,694 Million in 2017). Tunisia leads with a prevalence of 8.70 followed by Morocco 2.09, Libya 1.65 and Algeria 1.46/100.000 habitants. We did not find values for Mauritania. If we extrapol...

Egyptian Journal of Pediatric Allergy and Immunology, 2006

Common variable immunodeficiency (CVID) refers to a heterogeneous immunodeficiency syndrome chara... more Common variable immunodeficiency (CVID) refers to a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections and a variety of immunological abnormalities. Affected persons are prone to recurrent bacterial infections, especially involving the upper and lower respiratory tracts. In addition, patients exhibit increased susceptibility to a protean array of autoimmune, gastrointestinal, neoplastic, and inflammatory disorders .

The Egyptian Journal of Otolaryngology, 2014

The current study showed a relatively low incidence of IgA deficiency among children with ROM and... more The current study showed a relatively low incidence of IgA deficiency among children with ROM and indicated other environmental risk factors that participated in the occurrence of OM in our community.

Background. Persons with primary immune deficiency disorders (PID), especially those disorders af... more Background. Persons with primary immune deficiency disorders (PID), especially those disorders affecting the B-cell system, are at substantially increased risk of paralytic poliomyelitis and can excrete poliovirus chronically. However, the risk of prolonged or chronic excretion is not well characterized in developing countries. We present a summary of a country study series on poliovirus excretion among PID cases. Methods. Cases with PID from participating institutions were enrolled during the first year and after obtaining informed consent were tested for polioviruses in stool samples. Those cases excreting poliovirus were followed on a monthly basis during the second year until 2 negative stool samples were obtained. Results. A total of 562 cases were enrolled in Bangladesh, China, Iran, Philippines, Russia, Sri Lanka, and Tunisia during 2008-2013. Of these, 17 (3%) shed poliovirus, including 2 cases with immunodeficient vaccine-derived poliovirus. Poliovirus was detected in a single sample from 5/17 (29%) cases. One case excreted for more than 6 months. None of the cases developed paralysis during the study period. Conclusions. Chronic polioviruses excretion remains a rare event even among individuals with PID. Nevertheless, because these individuals were not paralyzed they would have been missed by current surveillance; therefore, surveillance for polioviruses among PID should be established.