Howard A. Pearson

Transient congenital leukemia in 7 infants with mongolism

3. Stein, W. H.: A chromatographic investigation of the amino acid constitutes of normal urine, J. Biol. Chem. 201: 45, 1953. 4. Folin, O.: Laws governing the chemical composition of urine, Am. J. Physiol. 13: 66, 1905. 5. Stambaugh, R., Davidson, D. T., Jr., and Elkinton, J. R.: Variation in excretion of certain amino acids with age, Clin. Chem. 9: 210, 1963. Brie[ clinical and laboratory observations 3 0 3

Marrow hypoplasia in anorexia nervosa

Hypoplasia of the bone marrow was observed in three patients with severe anorexia nervosa. Histologically, a marked paucity of hematopoietic elements and a relative increase of acid mucopolysaccaride ground substance were observed. Although peripheral blood counts were normal, ferrokinetic studies indicated erythroid hypoactivity in one patient. In another patient, persistent leukopenia was observed during pneumococcal pneumonia. If studies in other patients confirm these observations, a limited ability to respond to hematopoietic stress may be part of the clinical picture of severe anorexia nervosa and should be considered in their clinical management.

Disseminated intravascular coagulation associated with Aspergillus endocarditis: Fatal outcome following heparin therapy†

A syndrome of disseminated intravascular coagulation (DIC) was documented in a patient with postcardiotomy Aspergillus endocarditis. In vitro studies revealed no hemolytic or thromboplastic activity of the fungus, and so local effects of the bulky fungus tumor on the blood are postulated to have caused consumption coagulopathy. Shortly after heparin therapy was initiated, the patient developed signs of acute intracranial hemorrhage and subsequently died. Although anticoagulation has been shown to be of benefit in states of DIC, in some instances such therapy may be hazardous.

Normal response of sickle cell anemia patients to immunization with Salmonella vaccines

Patients with hemolytic states associated with sickle cell hemoglobin have a surprisingincidence of serious infections with Salmonella. The causes for this apparently increased susceptibility are unclear. This study was devised to examine the immune response of these patients to immunization with Salmonella vaccines. The incidence and titers of preimmunization agglutinins to a large number of Salmonella antigens showed no significant differences between sickle cell patients and appropriate controls. Thirteen patients who were immunized with various Salmonella vaccines showed quantitatively and qualitatively normal responses of their immunoglobulins. Therefore, the increased susceptibility of these patients to serious Salmonella infections does not appear to be due to either increased exposure or a deficient or qualitatively abnormal immune globulin response to these bacteria.

Purpura Factitia Attracting Attention by Self-Inflicted Lesions

T~ermatologic consultation raised the possibility of allergic purpura. However, no beneficial response followed an elimination diet and vitamin therapy. Five months after the onset of the purpura, she was studied again in another university hospital. Complete hematologic workup revealed no abnormalities; bone marrow aspiration yielded a dilute or hypocellular but &dquo;non-diagnostic&dquo; specimen. The parents and physician were informed of the normal results but were told that because of the history of thrombocytopenia and corticosteroid therapy, the possibility of leukemia in remission could not be excluded.

Effects of dietary and iron supplementation on hematocrit levels of preschool children

The effects of a 5 week period of dietary and medicinal iron supplementation on hematocrit levels of a group of preschool children from low socioeconomic backgrounds were studied. Two thirds of all children showed no change in hematocrit levels. Diet alone and diet plus 30 mg. of elemental iron per day were associated with statistically significant increases in mean hematocrit level. A slightly greater increase in mean hematocrit was seen in the iron-supplemented group. This study indicates that although severe nutritional anemia is not a common problem in this group of children, short-term improvement in nutrition is followed by significant increases in hematocrit. Only slight additive effect was obtained by the use of medicinal iron in the diet. It is suggested that an increase in hematocrit or hemoglobin level following therapy may constitute a better indicator of the prevalence of nutritional deficiency than a single measurement of such parameters as hemoglobin, hematocrit, or serum iron.

Delayed Onset of Hemolytic Anemia in a Child An Indicator of Ball Variance of Aortic Valve Prosthesis

Severe intravascular hemolysis is described as a complication of implantation of an aortic Starr-Edwards ball-valve prosthesis and insertion of a Teflon patch in the ascending aorta in a 9-year-old boy with severe aortic valvar stenosis. This progressive hemolysis of delayed onset was the sole indicator of extreme degeneration of the silicone rubber ball of the prosthetic aortic valve, a potentially lethal condition requiring surgical intervention. The phonocardiographic signs of aortic ball variance were not present, and there was no overt valvar incompetence.A lesser but noteworthy aspect in this case was the occurrence of an “aplastic crisis” superimposed on the severe hemolysis. This transient bone marrow, red cell aplasia, which was unrelated to drugs, precipitated an acutely severe anemia which accounted for the presenting symptoms of the patient and required transfusion.

Bone marrow plasmacyte values in childhood

Normal values for bone marrow plasmacytes were determined in over 50 patients, ranging in age from newborn infants to adults. Because large numbers of nucleated marrow elements were enumerated (5,000 cells), significant values were established. Comparisons were made between these values and those obtained in patients with disease states involving synthesis of immune globulin. Marrow plasmacyte values are low in normal infants and young children, but approach adult levels in later childhood, particularly after the age of 5 years. Because of the small numbers of plasmacytes normally present in the bone marrow in infancy and early childhood, the finding of low numbers of plasma cells in the bone marrow is not definitive evidence for the diagnosis of hypogammaglobulinemia in this age group.

Lack of influence of sickle cell trait on fertility and successful pregnancy

Abstract Hemoglobin types have been determined in more than 1,900 Negro girls and women from Alachua County, Florida. Sickle cell trait had no discernible effect on fertility or abortion. Because of increasing survival of patients with homozygous hemoglobin (Hgb) S disease, the frequency of the sickle gene will probably become fairly stable in the United States. Large-scale screening programs to identify carriers of the sickle gene, combined with genetic counseling and family planning, are indicated if cases of sickle cell anemia are to be prevented in the future.