A total of 422 patients with sickle cell disorders have been observed for 3,442 patient years. During this period, 53 episodes of septicemia or meningitis occurred, indicating a risk of 12.5% from these infections for each individual. If only patients with SS hemoglobinopathy (sickle cell anemia) (323 patients) are considered, the risk was 15.2%. The case fatality ratios for sepsis and meningitis were 35% and 10%, respectively. Disease due to Streptococcus pneumoniae occurred, almost exclusively, among children with SS hemoglobinopathy who were less than 5 years of age. After the first decade, illnesses among patients with all types of sickle cell disorders were frequently associated with an identifiable source of infection, a chronic course, and frequent involvement of Gram-negative organisms.

Adolescent↗

PubMed1977

Severe coagulation abnormalities in Reye syndrome.

Coagulation studies were performed on 13 children with clinical diagnoses of Reye syndrome. Four had abnormalities so severe that transfusion of coagulation factors and platelets were required prior to percutaneous liver biopsy. In one of these, abnormalities frequently found in association with acute hepatic failure were present. Disseminated intravascular coagulation was noted in two, while in the fourth child laboratory data were most consistent with that process. Although the coagulation defect associated with Reye syndrome is most often due to decreased production of all coagulation factors except VIII, these patients demonstrate that consumption can also occur. The severity of the coagulopathies found indicates that bleeding can become a major complication in the diagnosis and management of Reye syndrome.

Blood Coagulation Disorders↗

PubMed1977

Hb-E in combination with Hb-S and Hb-C in a black family.

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Adult↗

PubMed1977

On the birth of an infant with sickle cell anemia.

This is the first report in the English literature of the birth of an obligate SS infant whose parents both had sickle cell anemia. The young adult SS parents of this infant and their families did not wish to prevent the birth of this child but had, in fact, many positive reasons to continue the pregnancy to term even in the face of the increased personal risk of pregnancy for this mother. This unusual circumstance initiated a re-appraisal of the realistic expectations with respect to reproduction in patients with homozygous recessive genetic disorders. No definitive studies could be found which attempted to evaluate the influence of genetic counseling on reproductive behavior in patients with sickle cell anemia. The questions raised about the 'right to reproduce' and the realistic aims of genetic counseling in adult homozygote patients have been discussed. However, no conclusions seem warranted because of the paucity of available information.

Adult↗

PubMed1977

Congenital malaria: a rare cause of splenomegaly and anemia in an American infant.

A 38-day-old infant had fever, jaundice, hepatosplenomegaly, and a hemolytic anemia. A peripheral blood smear demonstrated intraerythrocytic malarial parasites identified as Plasmodium vivax. Maternal and infant sera contained antibodies to this species. A directed history revealed the mother had suffered several febrile illnesses in Mexico during her pregnancy. Malaria had not been diagnosed nor was it considered at the time of her delivery at this hospital. Review of this and six other cases of congenital malaria reported in this country since 1950 indicates clinical manifestations seldom appear before 3 weeks of age. Although these signs are more frequently associated with other transplacental infections, their occurrence in an infant whose mother is from or who has traveled in an endemic area should prompt consideration of the diagnosis of congenital malaria.

Adult↗

PubMed1977

Severe iron deficiency anemia.

Severe iron deficiency anemia remains a continuing major health hazard among inner city children in Los Angeles. Over a 24-month period, 60 children in whom hemoglobin values were below 7 grams per dl were admitted to hospital; 11 (18 percent) of them were in overt congestive heart failure. Contrary to the popular conceptions, two thirds of the anemic children were undernourished, approximating the 16th percentile for weight on the Iowa growth chart, and the frequency of premature birth was not greater than in the general population. There were no deaths in this series. A management protocol which included partial exchange transfusion of children in congestive heart failure and supportive transfusion for children with hemoglobin levels below 5 grams per dl was employed.

Anemia, Hypochromic↗

PubMed1977

Increased glutathione peroxidase activity in alpha-thalassemia.

Glutathione peroxidase (GSHPx) activity was found to be greatly elevated in members of a family with alpha-thalassemia. Eleven other families with proven alpha-thalassemia were investigated, and all but one subject with hemoglobin H disease had increased red cell GSHPx. Most persons with alpha-thalassemia trait also had increased activity of red cell GSHPx. In contrast, only very modest increases in glutathione peroxidase activity were observed in subjects with various forms of beta-thalassemia.

Chemical Phenomena↗

PubMed1976

Anxiety, self-concept, and personal and social adjustments in children with sickle cell anemia.

The psychologic effect of sickle cell anemia, a life-long chronic illness, on the self-concept, anxiety level, and personal and social adjustments of school-aged children was investigated by using a battery of standard psychologic tests. Two groups of children were evaluated: a study group of 29 children with sickle cell anemia (hemoglobin SS) and a comparison group of 26 black inner city schoolchildren without sickle cell disease or other known chronic illness. The youngsters with sickle cell anemia did not differ from a peer group of schoolchildren in personal, social, and total adjustments. The self-concept scores of the patient group were lower than those of the comparison group. An unexpected finding of the study was the observation that the anxiety scores (measuring acute anxiety) were significantly lower in the study group than those in the comparison group.

Adolescent↗

PubMed1976

A case with both hemoglobins C and N-Baltimore.

Hemoglobins C and N-Baltimore were detected in a 75-year-old black man. Although mild anemia and abnormal erythrocyte morphology resembling thalassemia were present, detection of this combination of hemoglobins at an advanced age suggests that it does not limit normal activities.

Aged↗

PubMed1975

A fetuin-like antigen from human nephroblastoma.

An antigen was detected in pooled human nephroblastomas using antiserum prepared in rabbits against an ethylemediaminetetra acetic acid (EDTA) extract of the tumors. This antigen was not found in normal human plasma or kidney extracts, and was not related to the ABO or Forssman blood groups. The antigen was detected in extracts of cultured nephroblastoma cells, but was not present in extracts of normal human fetal kidney cell cultures. The antigen is believed to be present at the cell surface, as cell viability was not significantly lowered during the extraction procedure. A reaction of complete identity was demonstrated by Ouchterlony double diffusion experiments with this antigen and purified bovine fetuin. The antigen was not found in extracts of human fetal spleen, thymus or kidney, nor in human fetal serum. Furthermore, the antigen does not possess determinants in common with the human alpha-fetoprotein of hepatomas, nor was it detected in human renal clear cell carcinoma. Initial characterization of the antigen showed it to be nondialysable, not sedimentable at 100,000 times g for 2 h, stable to repeated freeze-thawing and to incubation at 56 degrees C for 1 h, and water soluble over a wide pH range. The antigen was susceptible to digestion with pronase and trypsin and possibly hyaluronidase, but not to ribonuclease or neuraminidase. The protein portion is therefore of major importance to the structural integrity of this antigen. The relationship between this antigen and other abnormal materials reported previously in nephroblastoma patients is being studied.

Adolescent↗

PubMed1975

Familial neuroblastoma.

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Adrenal Gland Neoplasms↗

PubMed1975

The pediatrician and the dying child. "Physician, know thyself".

To deal with problems aroused in professional medical staff working with fatally ill children, a team of psychiatrics and oncologists not only deals with the problems of the children and their families, but also with problems of the medical staff themselves. Psychotherapy to the medical staff is offered only indirectly. The overriding difficulty which prevents the medical staff from maintaining role-appearance behavior is dealing with the theme of death. Often this is the hidden agenda behind a facade of other presenting problems. At times, the medical staff may be unable to deal with their own anger when conforted by demanding patients or hostile parents. At other times, medical staff will overidentify with the patient resulting in inappropriate role behavior. When medical results are poor despite good medical care, staff may feel inappropriately guilty. These issues can be dealt with means of a weekly mental health conference with the focus on the patient.

Adolescent↗

PubMed1975

Rapid diagnosis of sickle cell disease at birth by microcolumn chromatography.

Accurate specific diagnosis of sickle cell disease can now be made at birth on routinely obtained cord blood samples by microcolumn chromatography. The method uses a small column of a cation ion exchange resin, CM-Sephadex, and a single developer that allows definitive rapid distinction of hemoglobin SS, AS, AC, SC, and CC, within two hours. Seventy-five samples or more per day have been analyzed by one technician in a laboratory without special precautions or equipment. In a program which has been initiated on a large, totally unpredictable obstetrical service in Los Angeles, 10,401 consecutively born infants have been studied for hemoglobin type without regard to racial origin. Three SS infants, 1 SC, 143 AS infants, and 37 AC infants as well as several with other abnormal hemoglobins have been identified without interfering with the routine operation of the delivery rooms of the obstetrical service. The diagnosis of sickle cell disease has been confirmed on subsequent examinations of the infants. The feasibility of using microcolumn chromatography as a rapid, accurate, inexpensive, and easy method for the rapid diagnosis of sickle cell disease in newborns has now been established.

Anemia, Sickle Cell↗

PubMed1975

Microchromatography of hemoglobins. IV. An improved procedure for the detection of hemoglobins S and C at birth.

Substitution of CM-cellulose for CM-Sephadex had yielded a superior microchromatographic method for distinguishing the AS, AC, SS, SC, and CC conditions at birth. On the translucent columns of CM-Sephadex, the hemoglobin zones are somewhat diffuse. However, the compact, well-defined zones on the CM-cellulose column facilitate the interpretation of the results even though the amount of sample is only 20 per cent as great. The CM-cellulose method is as simple and rapid as the original CM-Sephadex procedure.

Chromatography↗