Studies In Sickle Cell Anemia Research Articles

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. XIV. Management of the child with sickle-cell anemia.

Sickle-cell anemia (SCA) is a systemic disease characterized by protean symptomatology and by periods of remission and exacerbation. The clinical manifestations are primarily dependent upon hemolysis, stasis, anemia, the age of the patient, and the nature of the precipitating or stressor agent such as infections. The clinical expression of disease often appears in the form of pain, fever, anorexia, debility, weakness, vomiting, pallor, lymphadenopathy, jaundice, impaired renal function (hyposthenuria), leukocytosis, and organ enlargement—particularly involving the heart, liver, and spleen. In infants and small children swelling of the small joints of the hands and feet is a fairly common occurrence. The most commonly encountered hemoglobinopathies in which sickling is of clinical significance in the pediatric age period are (1) homozygous S sickle-cell anemia, (2) sickle-cell-hemoglobin-C disease, and (3) sickle-cell thalassemia (microdepanocytosis). In our experience, 1 sickle-cell-hemoglobin-C disease, in the absence of severe osseous involvement (such as aseptic necrosis) is relatively mild

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

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. 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.

Observation on the genesis of crises in sickle cell anemia.

Case Reports1 June 1959OBSERVATION ON THE GENESIS OF CRISES IN SICKLE CELL ANEMIAJ. C. S. PATERSON, C. C. SPRAGUEJ. C. S. PATERSONSearch for more papers by this author, C. C. SPRAGUESearch for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-50-6-1502 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe periodic crises which punctuate the natural course of sickle cell anemia were separated by Singer and his colleagues17in 1950 into the following categories: (a) the "aplastic" crisis, (b) the "hyperhemolytic" crisis, and (c) "the aplastic-hyperhemolytic" crisis, in which there is both an abeyance of the delivery of cells from the marrow and an increase in the rate of hemolysis of the circulating red cells. This hematologic classification has been generally accepted, but with more emphasis put upon the "thrombotic," "infarctive" or "symptomatic" crisis, an episode of fever and pain, without any significant change in the hemoglobin level.9, 19,...Bibliography1. AndersonBusby GWT: Sickle cell anemia and pregnancy, Am. J. Obst. and Gynec. 58: 75, 1949. CrossrefMedlineGoogle Scholar2. Barcroft J: The respiratory function of the blood. Part II. Haemoglobin, 2nd Ed., 1928, Cambridge University Press, London. Google Scholar3. BauerFisher JLJ: Sickle cell disease with special regard to its nonanemic variety, Arch. Surg. 47: 553, 1943. CrossrefGoogle Scholar4. BeachamBeacham WDDW: Sickle-cell disease and pregnancy, Am. J. Obst. and Gynec. 60: 1217, 1950. CrossrefMedlineGoogle Scholar5. ConleyMartinRecinos JRMBA: Sickle cell anemia. Special report, Clin. Proc. Child. Hosp. 6: 101, 1950. Google Scholar6. Connell JH: Cerebral necrosis in sickle cell disease, J. A. M. A. 118: 893, 1942. CrossrefGoogle Scholar7. Diggs LW: The crisis in sickle cell anemia. Hematologic studies, Am. J. Clin. Path. 26: 1109, 1956. CrossrefMedlineGoogle Scholar8. DoneganMacIlwaineLeavell CCWABS: Hematologic studies on patients with sickle cell anemia following multiple transfusions, Am. J. Med. 17: 29, 1954. CrossrefMedlineGoogle Scholar9. FergusonCarringtonScott ADHTRB: Studies in sickle cell anemia. A clinical review, Med. Ann. District of Columbia 24: 517, 1955. MedlineGoogle Scholar10. Graham GS: A case of sickle cell anemia with necropsy, Arch. Int. Med. 34: 778, 1924. CrossrefGoogle Scholar11. HaldenSprouleClarkeMuirheadMiller ERBJRLEEWF: Effects of O2 breathing upon the sickling phenomenon in vivo in sickle cell anemia and its variants, communication to the Southern Society for Clinical Research, 11th Annual Meeting, January, 1957. Google Scholar12. HarrisBrewsterHamCastle JWHHTHWB: Studies on the destruction of red blood cells. X. The biophysics and biology of sickle-cell disease, Arch. Int. Med. 97: 145, 1956. CrossrefGoogle Scholar13. Henderson AB: Sickle cell anemia. Clinical study of fifty-four cases, Am. J. Med. 9: 757, 1950. CrossrefMedlineGoogle Scholar14. Herrick JB: Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia, Arch. Int. Med. 6: 517, 1910. CrossrefGoogle Scholar15. Huck JG: Sickle cell anaemia, Bull. Johns Hopkins Hosp. 34: 335, 1923. Google Scholar16. Singer K: The pathogenesis of sickle cell anemia. A review, Am. J. Clin. Path. 21: 858, 1951. CrossrefMedlineGoogle Scholar17. SingerMotulskyWile KAGSA: Aplastic crisis in sickle cell anemia. A study of its mechanism and its relationship to other types of hemolytic crisis, J. Lab. and Clin. Med. 35: 721, 1950. MedlineGoogle Scholar18. Tomlinson WJ: A study of the circulation of the spleen in sicklemia and sickle cell anemia, Am. J. Clin. Path. 11: 835, 1941. CrossrefGoogle Scholar19. Watson RJ: The hereditary anemias, Bull. New York Acad. Med. 30: 106, 1954. MedlineGoogle Scholar20. WatsonLichtmanShapiro RJHCHD: Splenomegaly in sickle cell anemia, Am. J. Med. 20: 196, 1956. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New Orleans, Louisiana*Received for publication October 22, 1957.From the Department of Medicine, Tulane University Medical School, New Orleans, Louisiana.†This observation was made in the course of studies undertaken in connection with an award to Dr. G. E. Burch, Chairman of the Department of Medicine, by the John Simon Guggenheim Memorial Foundation for studies of sickle cell anemia and of methods for its treatment.Requests for reprints should be addressed to J. C. S. Paterson, M.D., Department Tropical Medicine and Public Health, The Tulane University of Louisiana, 1430 Tulane Avenue, New Orleans 12, Louisiana. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byNew Concepts in the Immunology of Sickle Cell DiseaseAbnormal neutrophil adhesion in sickle cell anaemia and crisis: relationship to blood rheologyChronic tonsillitis, tonsillectomy and sickle cell crisesInfections in Sickle Cell DiseaseGenetic ScreeningDanger lists for the anaesthetist.Sickle-cell disease and its surgical implications in the African childDanger lists for the anaesthetistThe influence of hemoglobinopathies on reproductionSICKLE-CELL DISEASE IN PREGNANCY 1 June 1959Volume 50, Issue 6Page: 1502-1507KeywordsAnemiaAplastic anemiaBone marrowFeversHemoglobin ePublished: 1 December 2008 Issue Published: 1 June 1959 PDF downloadLoading ...

DISORDERS ASSOCIATED WITH THE METABOLISM OF PHENYLALANINE AND TYROSINE

DISORDERS ASSOCIATED WITH THE METABOLISM OF PHENYLALANINE AND TYROSINE

Studies in sickle cell anemia: Angella D. Ferguson, Henry T. Carrington, and Roland B. Scott. M. Ann. District of Columbia 24: 518, October, 1955

Studies in sickle cell anemia: Angella D. Ferguson, Henry T. Carrington, and Roland B. Scott. M. Ann. District of Columbia 24: 518, October, 1955

Studies in sickle-cell anemia. V. Sickle-cell hemoglobin C disease; report of two cases in siblings with clinical and genetic observations and a brief review of the literature.

INTRODUCTION Within the past few years, the application of newer knowledge, chiefly involving genetic, biochemical, and electrophoretic techniques, has resulted in the recognition of previously unknown intrinsic properties of human hemoglobin. The application of these newer tools to the study of sickle-cell anemia has revealed that the condition is not a simple disease entity but is in reality a hemolytic syndrome which may be separated into specific subtypes depending upon the variety of abnormal hemoglobin present. To date, the hemoglobins which have been identified by chemical and/or electrophoretic studies are as follows *: A—Normal adult hemoglobin F—Fetal hemoglobin S—Sickle-cell hemoglobin C—C hemoglobin D—D hemoglobin E—E hemoglobin G—G hemoglobin† The pathologic effects of various known combinations of abnormal hemoglobins are listed in Table 1. The current designation of sickle-cell anemia is reserved for the type in which the genes responsible for sickling are homozygous. Statistically, this is the commonest variety. The second

STUDIES IN SICKLE CELL ANEMIA<subtitle>Inheritance Factor, Including Effect of Interaction of Genes for Sicklemia and Thalassemia</subtitle>

THE HEREDITARY nature of the sickling of erythrocytes of certain persons has been recognized and accepted for many years. The exact pathogenesis of the genetic pattern, however, has been the source of controversy. Earlier investigators¹recognized no genetic difference between sickle cell disease and the asymptomatic trait (sicklemia). They regarded both conditions as being due to a single dominant gene which in some persons produced active disease and in others only an asymptomatic trait. More recently, Neel²and Beet³have taken issue with the older hypothesis and have presented an alternative concept. According to these workers, the asymptomatic sickle cell trait is due to a gene which is represented but once in the patient's genetic system (heterozygous state), whereas active sickle cell disease appears when the gene occurs twice (homozygous state). Moreover, the earlier investigators often found the sickling phenomenon lacking in the blood of one parent

Studies in sickle-cell anemia: II. Clinical manifestations of sickle-cell anemia in children (an analysis of thirty-seven cases with observation on the use of ACTH and cortisone in two additional cases)

Summary Thirty-seven cases of sickle-cell anemia have been reviewed. The disease occurred in twenty-seven males and ten females. The average age of onset of symptoms was 3.5 years while the average age of first admission to the hospital was 4.5 years. Abdominal and joint pains and anorexia were the most frequent symptoms observed in these cases. Respiratory infections, pallor, icterus, splenomegaly, and cardiac murmurs were the most outstanding physical findings. Some of the clinical difficulties encountered in the diagnosis of sickle-cell anemia are discussed. The parameter test of Winsor and Burch has been useful in the differential diagnosis of this disease. The therapy in these cases was essentially supportive, consisting chiefly of blood transfusions and measures to combat respiratory infections. There were five deaths in this series, representing a mortality of 13.5 per cent. The prognosis in sickle-cell anemia has improved since the advent of modern chemotherapy and antibiotics. Two cases in which ACTH and cortisone were successful in relieving joint pains have been reported. ACTH and cortisone give promise of value in the management of hemolytic crises by controlling severe joint and abdominal pains. Should future clinical observations confirm our preliminary impression regarding the value of ACTH and cortisone in the control of hemolytic crises, the questionable use of splenectomy with its attendant mortality may be discontinued in the management of this disease.

AUREOMYCIN IN STAPHYLOCOCCIC MENINGITIS COMPLICATING SUBARACHNOID HEMORRHAGE IN SICKLE CELL ANEMIA

The rare occurrence of a spontaneous subarachnoid hemorrhage in an 11 year old Negro boy with sickle cell anemia is recorded. Of the eight reported cases five have occurred in children. In the study of sickle cell anemia it is evident there is an increasing awareness of this as well as other neurologic complications. This patient developed staphylococcus albus meningitis which for 12 days resisted combined penicillin, streptomycin and sulfadiazine therapy. The response to aureomycin as a sole therapeutic agent was dramatic, and this represents the first reported case of meningitis treated with this drug. The use of aureomycin as the only antibiotic in this staphylococcic infection seems to have been fortunate in view of the laboratory findings. In vitro studies disclosed that when penicillin and aureomycin or streptomycin and aureomycin were combined, the result was a decrease in the effectiveness of either drug on staphylococci.