Autoimmune cytopenias are defined by autoantibodies' immune destruction of one or more blood elements. Most often it is autoimmune hemolytic anemia or immune thrombocytopenia or both that define Evans syndrome. It may be secondary to infection or to underlying pathology such as systemic autoimmune disease or primary immunodeficiency, especially when it becomes chronic over several years.
View Article and Find Full Text PDFCentral pontine myelinolysis is a demyelinating disorder mainly affecting the central pons. In some cases, it is associated with extrapontine myelinolysis. It is usually caused by rapid correction of hyponatremia and osmotic shock.
View Article and Find Full Text PDFWe report the case of a 2.5-year-old child admitted for abdominal distension, whose imaging revealed a large posterior mediastinal cystic mass, with a tissue component, a calcification, and a minimal fat component. The ultrasound- guided biopsy led to the diagnosis of a benign extragonadal germ cell tumor, also called mature teratoma or dermoid cyst, whose mediastinal localization is rare, often localized in the anterior mediastinum, and rarely in the posterior mediastinum.
View Article and Find Full Text PDFPrimary intracranial germ cell tumors are rare, often affecting children and young patients. Germinomas are the most common type of germ cell tumors. We present the case of a 10-year-old child, who was admitted with decreased visual acuity, asthenia, polyuro-polydipsic syndrome, and gait disorder.
View Article and Find Full Text PDFSevere combined immunodeficiency (SCID) is a heterogeneous group of primary immunodeficiency diseases (PIDs) characterized by a lack of autologous T lymphocytes. This severe PID is rare, but has a higher prevalence in populations with high rates of consanguinity. The epidemiological, clinical, and immunological features of SCIDs in Moroccan patients have never been reported.
View Article and Find Full Text PDFMacrophage activation syndrome (MAS) is a multisystemic disorder resulting from an over-activation of the immune system leading to a more or less diffuse macrophagic infiltration into the tussues. Clinical signs including fever, hepatosplenomegaly, adenopathy are associated with abnormal values in laboratory test results (bi or pancytopenia, hepatic cytolysis, elevated LDH levels, coagulopathy) and hemophagocytosis. In children, it can be primary or secondary to several disorders.
View Article and Find Full Text PDFCutis laxa (CL) is a heterogeneous group of inherited and acquired connective tissue disorders characterized by a loose skin and variable systemic involvement (inguinal hernia, cardiopulmonary disease, and emphysema). Autosomal dominant, autosomal recessive and x-linked recessive patterns have been described in the inherited forms. Acquired forms of this disease have been associated with a previous inflammatory skin disorder (urticaria…).
View Article and Find Full Text PDFHemophagocytic lymphohistiocytosis (HLH) is a severe life-threatening disorder, responsible for extensive phagocytosis of hematopoietic cells and causing a multisystem organ failure. If lymphomas are common causes of HLH, the association with Hodgkin's lymphoma is rarely described in children. We report a case of a 9-year-old boy presenting with HLH as an initial manifestation of Hodgkin's lymphoma.
View Article and Find Full Text PDFCongenital lobar emphysema (CLE) is a rare cause of respiratory distress during the neonate period. It is characterized by overinflation of pulmonary lobe, most commonly the left upper lobe or the right middle lobe. We report a case of a 21-day-old baby with the severe respiratory distress.
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