Successful restoration of immunity in the DiGeorge syndrome with fetal thymic epithelial transplant.
نویسندگان
چکیده
A 13-month-old girl presented with right upper lobe pneumonia and hypocalcaemic seizures: investigations showed hypoparathyroidism and impaired cell-mediated immune responses. Other features of the DiGeorge syndrome included hypertelorism, short philtrum of the lip, right-sided aortic arch, and aberrant origin of the left subclavian artery. Successful restoration of the immunodeficiency was achieved by transplantation of fetal thymic epithelium.
منابع مشابه
Digeorge syndrome presenting with uncommon cardiac anomaly and hepatomegaly
Digeorge syndrome is caused by microdeletion of a large region of chromosome 22q11.2 lead to the abnormal development of the third and fourth pharyngeal pouches. This syndrome is characterized by hypoparathyroidism, cellular immune deficiency secondary to thymic hypoplasia, congenital heart disease and dysmorphic facial features. In this case report, we describe a 4month old boy who presented w...
متن کاملبررسی ایمونوهیستوشیمیایی تیموسهای آپلاستیک از نظر مارکرهای اپی‌تلیالی، لنفوسیتی و آپوپتوز و مقایسه آن با تیموسهای نرمال
Background: Immune deficiency is one of the major causes of morbidity and mortality in the modern world. Primary immunodeficiency comprises a wide range of disorders that mainly manifest in early childhood as devastating infections with opportunistic organisms. Thymic aplasia is found on autopsy of some patients afflicted with immune deficiency disorders, such as DiGeorge syndrome and severe co...
متن کاملDiGeorge's syndrome
DiGeorge's syndrome results from an interference in embryogenesis between the sixth and tenth week of intrauterine life, involving the third and fourth branchial arches; the first antasecond arches are often and the sixth arch occasionally involved. The association of congenital absence of the thymus with seizures in the newborn and abnormal third and fourth pharyngeal pouch development was rec...
متن کاملImmuno-reconstitution by thymic transplant in DiGeorge's syndrome.
Persistent hypocalcaemic tetany in a breast-fed neonate made us suspect DiGeorge's syndrome, particularly as the baby had an abnormal facies, and a ventricular septal defect. Immuno-reconstitution was successfully achieved by thymic transplant. Evidence of immuno-reconstitution on the basis of the histology of the post-transplant thymus has not been previously recorded.
متن کاملTreatment Results and Prognostic Indicators in Thymic Epithelial Tumors: A Clinicopathological Analysis of 45 Patients
Background: Thymomas are rare epithelial tumors arising from thymus gland. This study aims at investigating the clinical presentation, prognostic factors and treatment outcome of forty five patients with thymoma and thymic carcinoma. Methods: Forty-five patients being histologically diagnosed with thymoma or thymic carcinoma that were treated and followed-up at a tertiary academic hospital duri...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 53 7 شماره
صفحات -
تاریخ انتشار 1978