Roland B. Scott

Studies in sickle-cell anemia

Children with sickle cell anemia drink large amounts of fluid, have serum of low osmolality, and void large quantities of urine of low osmolality. These findings may be explained on the basis of habitual ingestion of excessive amounts of fluid rather than renal damage.

Growth and development of negro infants

Summary The time of eruption of the firstdeciduous tooth and the number of teeth present at one year of age in Negro and white infants was compared and related to the race, socioeconomic level, and sex of the infant. From this study the following conclusionsare made: 1.The mean time of eruption of thefirst deciduous tooth in Negro male and female infants was 27.7 and 28.9 weeks, respectively. The white male and female infants erupted their first tooth at 29.1 and 31.6 weeks, respectively. This earlier eruption time in Negro infants is possibly due to a racial tendency toward accelerated tooth eruption in this group. 2.The Negro male and female infants had a mean of 6.0 and 5.9 teeth at the age of one year as compared to 6.9 and 6.4 teeth for white male and female infants, respectively. The larger number of teeth found in the white infants at one year of age may be attributed to nutritional advantages, since it was of statistical significance only when the white infants from a middle socioeconomic level (private practice) were compared with Negro infants from a lower socioeconomic level (clinic infants). 3.The influence of sex is demonstratedby the slight acceleration in dentition in the male infants in all groups studied.

HLA-B35 is associated with red cell alloimmunization in sickle cell disease☆

HLA-A, -B, -C, and DR antigens were determined in 33 patients with sickle cell disease (SCD), who had received red blood cell (RBC) transfusions. Twenty-one patients formed red cell alloantibodies after transfusions (responders) while 12 multitransfused SCD patients did not form any RBC antibodies (non-responders). We found that 67% of the SCD responder participants had HLA-B35 versus 25% of the non-responders (chi 2 = 5.3079, P = 0.0212). The frequency of B35 in non-responder SCD patients was similar to that of a normal healthy Black population consisting of 139 individuals. Calculation of the relative risk showed that sickle cell patients with B35 are six times more likely to form RBC alloantibodies after transfusion than those lacking that HLA antigen. We found no significant increase or association between any HLA-DR antigens and sickle cell disease.

Studies in sickle cell anemia: XVI. Sudden death during sickle cell anemia crises in young children

Summary Ten cases of sudden death occurring in children with sickle cell anemia are reported. Seven of the deaths occurred within 45 minutes of hospitalization. The clinical picture common to the entire group was extreme pallor, weakness and lethargy, shocklike appearance, tachycardia, and cold clammy skin. The most striking necropsy finding in the 7 patients autopsied was congestion of the internal organs with sickled erythrocytes. A possible mechanism of stasis of red blood cells in the spleen, liver sinusoids, and/or splanchnic capillaries with peripheral vascular collapse and cerebral anoxia is proposed to explain sudden death in infants and small children who are already debilitated by anemia, hemolysis, infection, and the like. In order to prevent a fatal outcome it is often necessary to provide emergency measures such as immediate transfusion of whole blood or packed erythrocytes, oxygen inhalation, and antibiotics for associated infections.

Rupture of the Stomach in the Newborn Infant: Report of Three Cases and Review of the World Literature

nursery. He did well until the second day of life when vomiting occurred. A naso-gastric tube was again passed easily, a few ml. of clear gastric juice was aspirated, and the stomach lavaged with normal saline solution. On the third day jaundice and marked abdominal distention developed, along with fever of 101° F. Laboratory findings were normal. Kanamycin was started. An x-ray film of the abdomen revealed free air in the peritoneal cavity including the subdiaphragmatic region (Fig. 1). A laparotomy was performed approximately seven hours after the distention was first noticed. TABLE 1. STzozvir~~ number of reported cases, deatlas and survivals from 1825 to 1964. Notetlze increase in number of reported cases during recent years and the improvemertt in she ~prezntage of survival

Growth and development of Negro infants. VIII. Comparison of the deciduous dentition in Negro and white infants; a preliminary study.

Summary The time of eruption of the firstdeciduous tooth and the number of teeth present at one year of age in Negro and white infants was compared and related to the race, socioeconomic level, and sex of the infant. From this study the following conclusionsare made: 1. The mean time of eruption of thefirst deciduous tooth in Negro male and female infants was 27.7 and 28.9 weeks, respectively. The white male and female infants erupted their first tooth at 29.1 and 31.6 weeks, respectively. This earlier eruption time in Negro infants is possibly due to a racial tendency toward accelerated tooth eruption in this group. 2. The Negro male and female infants had a mean of 6.0 and 5.9 teeth at the age of one year as compared to 6.9 and 6.4 teeth for white male and female infants, respectively. The larger number of teeth found in the white infants at one year of age may be attributed to nutritional advantages, since it was of statistical significance only when the white infants from a middle socioeconomic level (private practice) were compared with Negro infants from a lower socioeconomic level (clinic infants). 3. The influence of sex is demonstratedby the slight acceleration in dentition in the male infants in all groups studied.

Studies in Sickle Cell Anemia XXII. Clinical Manifestations of Sickle Cell Anemia During the First Two Years of Life

first two years of life.~-3 The clinical expressions of this .condition are protean and are influenced to some extent by the age of the patient. This report summarizes the clinical picture in early life, based upon the findings in 18 patients who were diagnosed and followed during the first two years of life. Of these, 14 were males and four, females. It is believed that this uneven distribution of the sexes is accidental, since the disease in no way has been found to be sex-linked.

Common skin disorders in the newborn Negro infant. Observations based on the examination of 1,000 babies.

Observations were made on 1,000 newborn infants for the occurrence of skin disorders. Approximately 87 per cent of the subjects were full-term and 13 per cent were premature infants. Each infant was cleansed with pHisohex on admission to the nursery. Thereafter, pHisohex was used on premature babies whereas tap water was used for the daily skin care of full-term infants. The most commonly observed dermatoses were milia, miliaria crystalina, erythema toxicum, xerodermia, and perianal dermatitis. The occurrence rate of these conditions was 19, 17, 16, 15, and 7 per cent, respectively. The 2 most frequently observed incidental findings were Mongolian spots (75 per cent) and port-wine marks (nevus flammeus) which occurred in 23 per cent of the infants.

Studies in sickle cell anemia. XIX. Priapism as a complication in children.

Summary Priapism is a rare complication of sicklecell anemia in children. Its presence in a Negro subject should lead the clinician to consider sickle cell disease as a possible etiological factor. The condition represents a therapeutic challenge. Treatment is symptomatic and generally unsatisfactory; however, needle aspirations of the corpora cavernosa appear to yield the most satisfactory results. Inasmuch as this complication is usually associated with a thrombotic type of crisis in which the hemoglobin concentration and erythrocyte count show insignificant changes from their usual values in these patients, blood transfusions are of questionable value.

Studies in Sickle Cell Anemia: XXVII. Complications in Infants and Children in the United States

to transitory vascular occlusion and interfere with the circulation in the organ affected. Sickled erythrocytes have a short life span, contributing to anemia and hypoxia. The interaction of these two pathologic processes, anemia plus focal or generalized vascular occlusion, are responsible for the bizarre symptoms and the physical findings associated with sickle cell anemia (SCA) (Fig. 1). The symptoms and signs of SCA can be