John H. Githens

Thymic alymphoplasia with XX/XY lymphoid chimerism secondary to probable maternal-fetal transfusion†††

Karyotypes in a 5-month-old patient with thymic alymphoplasia revealed evidence for XX/XY chimerism in peripheral blood lymphocytes. These cells showed no immunologic competence, and there was no indication of a graft-versus-host reaction (GVHR). These findings suggest that placentally transfused maternal lymphocytes may persist in an immunologically incompetent fetus without having a demonstrable effect. Additional findings included adrenal hyperplasia, possible absence of parathyroids, and lack of evidence of immunologic function after attempts at grafting fetal thymic and hematopoietic tissue.

The autoerythrocyte sensitization syndrome as the primarymanifestation of systemic lupus erythematosus

GARDNER AND DIAMOND 1 first described the autoerythrocyte sensitization syndrome in four adult women who had the sudden onset of spontaneous, painful purpura. They found that the skin of the affected individuals reacted to injection of packed red blood cells or red blood cell stroma with similar painful purpura. Later investigations have focused either on the abnormal skin response to red cell Components = or on the abnormal psychiatric evaluation Of these patients, a, 4 Routine laboratory tests have been uniformly normal. I n one report, the syndrome was described in association w{th an immune complex nephritis? AES has been rarely noted in childhood. This report describes an l l -year-old girl who presented with the characteristic clinical findings of AES; however, further labora tory testing indicated a diagnosis of sy stemic lupus erythematosus.

Immunologic Reconstitution with Fetal Tissue

IT has been recognized for a number of years that infants with congenital T-cell defects (cellular immune deficiencies) will readily accept various allogeneic grafts. Immunologic reconstitution of ...

The carrier state in hemophilia A

Summary A simplified method for detecting decreasedAHG activity with the thromboplastin generation test has been described. Utilization of this method in the study of mothers of males with hemophilia has detected reduced AHG activity in approximately half of a group of known and probable carriers. The AHG activity in the “hemizygous” normal male has been shown to be approximately equal to that of the homozygous normal female in contrast to the reduced levels found in some heterozygous female carriers. Some of the unexplained genetic implications of these findings are discussed.

Anemias in childhood resulting from bone-marrow deficiencies. Nutritional, aplastic and refractory anemias.

The rapid growth in the first year of life is the major cause of iron-deficiency anemia in infancy. Premature infants especially need supplemental iron. Oral iron therapy is very satisfactory. Intramuscular irondextran complex has produced excellent results clinically, but sarcomas have developed in animals receiving repeated injections at the same site.Some aplastic or refractory anemias may be due to an inborn error of metabolism of tryptophan or nucleic acid, to an inhibitor produced in the spleen, or to a deficiency of erythropoietin.Chloramphenicol should never be used in pediatric practice unless it is the only antibiotic known to be effective against a specific serious infection.Cortisone and testosterone have proved to be of considerable value in selective cases of aplastic and hypoplastic anemias.