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PubMed1983

[Hypoplasia of the thymus gland in children].

Morphological changes of the thymus, tonsils, lymph nodes, spleen, and other organs in two observations of severe combined insufficiency (SCIN) in infants of the first year of life are described. In both cases there was hypoplasia of the thymus with the lack or occasional thymic bodies and poor content of thymocytes in the lobules. In one infant, hypoplasia of the thymus was combined with intestinal angiomatosis which suggested the syndrome of ataxia-teleangiectasia. In the other infant, SCIN was associated with severe granulocytopenia of the Kostman type. In this case epithelial cells of the lobules formed adenomatous structures. Both infants died with sepsis developing against the background of SCIN.

Agranulocytosis↗
PubMed1982

Association of bilateral renal agenesis and Di George syndrome in an infant of a diabetic mother.

We found at postmortem examination the association of bilateral renal agenesis and of apparently complete Di George syndrome in an infant whose mother was diabetic. Vertebral abnormalities and hallux duplication were present as well. There is a correlation between maternal diabetes and the bilateral renal agenesis-caudal dysplasia complex on one hand, and maternal diabetes and cardiac malformations on the other hand. Moreover, it has been suggested that the absence of thymus and parathyroids in Di George syndrome is causally linked to the cardiac malformation. Therefore we suggest that the association in our case is not coincidental; both sets of malformations may be due to the maternal diabetes.

DiGeorge Syndrome↗
PubMed1994

[Sensible diagnosis in suspected immune deficiency].

Patients reporting a marked proclivity for developing infections often present the care-providing physician with a diagnostic problem. If the available diagnostic possibilities are to be used rationally and with proper understanding, a thorough knowledge of the nature of an immune reaction is essential. The present paper contains a step-by-step scheme that may serve as an aid for the diagnostic approach. In addition to the clinical examination and routine laboratory investigations, simple tests such as Mérieux multitest and serum antibody titer serve to orientate the doctor on the patient's immune status. More sophisticated time-consuming and expensive tests, such as flow cytometric analysis of immune cells or functional in vitro investigations permit the identification of immune deficiency syndromes. These tests should, however, be undertaken only when severe immunodeficiency is suspected clinically or the orienting studies make it appear likely.

Antibody Formation↗
PubMed1983

[Di George syndrome, exemplary rhomboencephalic neurocristopathy].

Di George's syndrome associates hypocalcemia and hyperphosphoremia (phosphocalcic disorders from absence of the parathyroids), cell immunity disorders related to agenesis or hypoplasia of the thymus, and large vessels malformations (pulmonary artery, aorta) with cardiac lesions. The disorder is the result of deletion of rhomboencephalic neural crests cells, Di George's syndrome being an exemplary rhomboencephalic neurocristopathy. This organic syndrome is associated with early disorders of the same neurological region (the rhomboencephalon), expressed clinically by sucking and swallowing disturbances and cardiorespiratory regulatory disorders. Early rhomboencephalic lesions can lead to velopalatal division and microretrognathism secondary to a lingual motility disturbance. The association of this neurocristopathy with a neural tube lesion constitutes a dysneurulation. Fetal and neonatal retrognathism are evidence of bulbar involvement. Di George's and Pierre Robin's syndromes constitute two fairly similar clinical expressions of the same embryonic deficiency of rhomboencephalic neurulation.

Brain↗