Case report

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Nesrine Radwan
Lecturer in Paediatrics, Ain Shams University, Egypt.

Background: CGD is an immunodeficiency disease caused by mutations in genes encoding subunits of the leukocyte NADPH oxidase complex causing defective “respiratory burst”, and thus failure of clearance of micro-organisms. Its prevalence is 1∶250,000. The majority of CGD patients suffer from severe recurrent bacterial and fungal infections, dysregulated Th-17-lymphocyte-controlled inflammation causing granuloma formationand autoimmune phenomena. The most common offending organisms are Staphylococcus aureus, and Aspergillus. The leukocyte NADPH oxidase, consists of subunits, four of which are important for CGD; gp91phox ,p22phox (located in membranes) and p47phox, p67phox ( located in the cytosol). gp91phox gene mutations are responsible for 70% of CGD cases which are inherited as X-linked recessive. Mutations in the other subunits are autosomal recessive in inheritance (figure 1)1. Treatment includes long-term prophylactic antibiotics and antifungals and curative stem-cell transplantation.

El-Sayed ZA¹, Hossny EM¹, Reda SM¹, El-Owaidy RH¹, Aly N¹, Yahia RM²
¹Pediatric Allergy and Immunology unit, Children's Hospital, Ain Shams University, Cairo, Egypt
²Mohammed A Hamid Teaching Hospital for Paediatrics, Khartoum, Sudan.

Background: Chronic granulomatous disease (CGD) is a genetically heterogeneous disorder characterized by recurrent life-threatening bacterial and fungal infections and granuloma formation. Selective IgA deficiency (SIgAD) is one of the most common types of primary immunodeficiency, characterized by low serum levels of IgA (<5 mg/dL) in the presence of normal IgG and IgM. Coexistence of CGD and SIgAD is an interesting issue that is very rarely reported. Immunodeficiency and autoimmune phenomena may occur concomitantly in the same individual. The incidence of autoimmune disease in SIgAD varies between 7% and 36%, especially affecting the lungs and hematological parameters. Due to the persistent infections, patients with CGD could have high levels of immunoglobulins with several autoantibodies. More than 15% of the children with CGD suffer from autoimmune diseases

Traduit de l’anglais par Jeddane L (Maroc)

Contexte: La granulomatose septique chronique (CGD) est un trouble génétique hétérogène caractérisé par des infections récurrentes mortelles bactériennes et fongiques et la formation de granulomes. Le déficit isolé en IgA (SIgAD) est l'un des déficits immunitaires les plus fréquents, caractérisé par des taux sériques faibles d’IgA (<5 mg / dL) en présence de taux normaux d'IgG et IgM. La coexistence de CGD et SIgAD est une occurence intéressante qui est très rarement rapportée. Déficit immunitaire et autoimmunité peuvent se produire en même temps chez le même individu. L'incidence de maladies auto-immunes dans le SIgAD varie entre 7% et 36%, affectant en particulier les poumons et les paramètres hématologiques. En raison des infections persistantes, les patients atteints de CGD peuvent avoir des taux élevés d'immunoglobulines avec plusieurs auto-anticorps. Plus de 15% des enfants atteints de CGD souffrent de maladies auto-immunes.

Yousif Sulaiman, Idriss Matoug, Abdulla Alteer, Hanan Alsalheen, Salah Alamroni, Fatma Altajori.
Children's Hospital, Faculty of Medicine, Benghazi University, Benghazi, Libya.

Mucocutaneous leishmaniasis is one of different forms of leishmainiasis due to parasitic infection transmitted by sand fly bite. It is caused by L. braziliensis, L. panamensisand, less frequently, L. amazonensis. The infection characterized by lesions of the skin and mucous membranes in the mouth and nose (1,2).