ENFERMEDAD DE WISKOTT ALDRICH PDF

Wiskott Aldrich syndrome (WAS) is a disease with immunological deficiency and reduced ability to form blood clots. Signs and symptoms include easy bruising. Wiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by the clinical triad of microthrombocytopenia, eczema, and recurrent. Request PDF on ResearchGate | Síndrome de Wiskott-Aldrich de un paciente diagnosticado en la infancia con enfermedad de Bruton.

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Hypertrophic cardiomyopathy 7, 2 Nemaline myopathy 4, 5. Spinocerebellar ataxia 5 Hereditary spherocytosis 2, 3 Hereditary elliptocytosis 2, 3 Ankyrin: Skin fragility syndrome Arrhythmogenic right ventricular dysplasia 9 centrosome: Expert Opinion on Biological Therapy.

Ornithine transcarbamylase deficiency Oculocerebrorenal syndrome Dyslipidemia: Enfermedsd and other nonsteroidal anti-inflammatory drugs should be avoided, since these may interfere with platelet enfremedad which is already compromised. Red Blanchable Erythema Generalized drug eruptions viral exanthems toxic erythema systemic lupus erythematosus.

Retrieved from ” eenfermedad Please help improve this article by adding citations to reliable sources. Purine nucleoside phosphorylase deficiency Hyper IgM syndrome 1. The syndrome is named after Dr. The majority of children with WAS develop at least one autoimmune disorderand cancers mainly lymphoma and leukemia develop in up to a third of patients. This article needs additional citations for verification. The estimated incidence of Wiskott—Aldrich syndrome in the United States is one inlive male births.

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Gestionan tratamiento en Italia de un niño tarijeño con síndrome de Wiskott-Aldrich

Enlargement of the spleen is not uncommon. The Journal of Experimental Medicine. Dyskeratosis congenita Hypohidrotic ectodermal dysplasia EDA X-linked ichthyosis X-linked endothelial corneal dystrophy.

Charcot—Marie—Tooth disease 2A Hereditary spastic paraplegia Views Read Edit View history. Long QT syndrome 4. Long QT syndrome 4 Hereditary spherocytosis 1. Spontaneous nose bleeds and bloody diarrhea are also common and eczema typically develops within the first month of life.

Gestionan tratamiento en Italia de un niño tarijeño con síndrome de Wiskott-Aldrich

Wiskott—Aldrich syndrome WAS is a rare X-linked recessive disease characterized by eczemathrombocytopenia low platelet countimmune deficiencyalsrich bloody diarrhea secondary to the thrombocytopenia.

As WAS is primarily a disorder of the blood-forming tissues, a hematopoietic stem cell transplant, accomplished through a umbilical cord blood or bone marrow transplant offers the only current hope of cure.

Striate palmoplantar keratoderma 2 Carvajal syndrome Arrhythmogenic right ventricular dysplasia 8 plectin: This may be recommended for patients with HLA -identical donors, matched sibling donors, or even in cases of incomplete matches if the patient is age 5 or under. Epidermolysis bullosa simplex with muscular dystrophy Epidermolysis bullosa simplex of Ogna plakophilin: The rare disorder X-linked neutropenia has also been linked to a specific subset of WAS mutations.

Anemia from bleeding may require iron supplementation or blood transfusion. For patients with frequent infections, intravenous immunoglobulins IVIG can be given to boost the immune system. Aldrkch page was last edited on 14 Novemberat Often, leukemia may be suspected on the basis of low platelets and infections, and bone marrow biopsy may be performed. Wikipedia articles needing page number citations from July Articles needing additional references from April All articles needing additional references Infobox medical condition new.

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WAS is associated with mutations in a gene on the short arm of the X chromosome Xp WAS occurs most often in males due to its X-linked recessive pattern of inheritance, affecting between 1 and 10 males per million. D ICD – Lymphoid and complement immunodeficiency D80—D85 Studies of correcting Wiskott—Aldrich syndrome with gene therapy using a lentivirus have begun.

Alport syndrome Dent’s enfermedsd X-linked nephrogenic diabetes insipidus. Epidermal wart callus seborrheic keratosis acrochordon molluscum contagiosum actinic keratosis squamous-cell carcinoma basal-cell carcinoma Merkel-cell carcinoma nevus sebaceous trichoepithelioma.

Wiskott–Aldrich syndrome

X-linked agammaglobulinemia Transient hypogammaglobulinemia of infancy. Treatment of Wiskott—Aldrich syndrome is currently based on correcting symptoms. The New England Journal of Medicine. Decreased levels of WASp are typically observed. Allergy, Asthma, and Clinical Immunology.

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