Thymus transplantation in complete DiGeorge anomaly.

[omenn syndrome]

Complete DiGeorge anomaly is characterized by athymia , congenital heart disease , and hypoparathyroidism . This congenital disease is fatal by age 2 years unless immune reconstitution is successful . There are multiple underlying syndromes associated with complete DiGeorge anomaly including 22 q 11 hemizygosity in approximately 50 %, CHARGE association in approximately 25 %, and diabetic embryopathy in approximately 15 %. Approximately one -third of patients present with rash and lymphadenopathy associated with oligoclonal "host " T cells . This condition resembles Omenn syndrome . Immunosuppression is necessary to control the oligoclonal T cells . The results of thymus transplantation are reported for a series of 50 patients , of whom 36 survive . The survivors develop naïve T cells and a diverse T cell repertoire .

Diseases
Validation

Diseases presenting "heart disease" symptom

22q11.2 deletion syndrome

achondroplasia

acute rheumatic fever

adrenal incidentaloma

child syndrome

classical phenylketonuria

cohen syndrome

congenital diaphragmatic hernia

dentinogenesis imperfecta

esophageal adenocarcinoma

fabry disease

familial mediterranean fever

heparin-induced thrombocytopenia

hirschsprung disease

holt-oram syndrome

homocystinuria without methylmalonic aciduria

kabuki syndrome

monosomy 21

omenn syndrome

phenylketonuria

sneddon syndrome

systemic capillary leak syndrome

wiskott-aldrich syndrome

wolf-hirschhorn syndrome

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