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September 20, 1985, Vol 254, No. 11 >
ARTICLE | September 20, 1985

Life-Threatening Complications of Sickle Cell Disease in Children FREE

Myron L. Mills, MD
[+] Author Affiliations

Reprint requests to Section of Emergency Medicine, Arizona Health Sciences Center, Tucson, AZ 85724 (Dr Mills).


JAMA. 1985;254(11):1487-1491. doi:10.1001/jama.1985.03360110077028
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Article
References

SINCE 1910, when sickle cell anemia (SCA) was first described by Dr James Herrick, a Chicago cardiologist, a prodigious amount of clinical and laboratory investigation has taken place in an effort to elucidate the pathogenesis, etiology, and treatment of SCA.1 In 1949, Pauling and Itano2 published their findings relating to the different electrophoretic properties of hemoglobin from patients with SCA. Later, in 1957, Ingram3 published the results of his work detailing the biochemical defect in hemoglobin S. From the early 1950s to the present time, the sickle erythrocyte and hemoglobin S molecule have been studied by sophisticated techniques that have revealed their structure and function at the molecular level. Techniques employed for these studies have included low-resolution x-ray crystallography, electron microscopy with image reconstruction, hemoglobin S polymerization kinetics, high-resolution proton nuclear magnetic resonance spectroscopy, and fiber x-ray diffraction.

The basic defect in hemoglobin S is the substitution

REFERENCES

1 +
Herrick JB:  Peculiar elongated and sickle-shaped red corpuscles in a case of severe anemia . Arch Intern Med 1910;;6:517.
2 +
Pauling L, Itano HA:  Sickle cell anemia, a molecular disease . Science 1949;;110:543.
3 +
Ingram VM:  A specific chemical difference between the globins of normal human and sickle cell anemia hemoglobin . Nature 1956;;178:792.
4 +
Lessin LS, Jensen WN:  Sickle cell anemia 1910-1973 . Arch Intern Med 1974;;133:529.
5 +
Motulsky AG:  Frequency of sickling disorders in US blacks . N Engl J Med 1973;;288:31.
6 +
Lehman H, Huntsman RG:  The hemoglobinopathies , in Stanburg JB, Wyngarden JB, Fredrickson DS (eds): The Metabolic Basis of Inherited Disease . New York, McGraw-Hill Book Co, 1982;, p 1404.
7 +
Pearson HA:  Sickle cell syndromes and other hemoglobinopathies , in Miller DR, Pearson HA (eds): Smith's Blood Diseases of Infancy and Childhood , ed 4. St Louis, CV Mosby Co, 1978;, pp 407-429.
8 +
Horne MK II:  Sickle cell anemia as a rheologic disease . Am J Med 1981;;70:288.
9 +
 National Institutes of Health: Improved methods of blood transfusion for patients with sickle cell anemia . JAMA 1983;;249:699.
10 +
Bertles JF, Milner PF:  Irreversibly sickled erythrocytes: A consequence of the heterogenous distribution of hemoglobin types in sickle-cell anemia . J Clin Invest 1968;;47:1731.
11 +
Serjeant GR, Milner P:  The irreversibly sickled cell: A determinant of haemolysis in sickle cell anemia . Br J Haematol 1969;;17:527.
12 +
Diggs LW:  The crisis in sickle cell anemia: Hematologic studies . Am J Clin Pathol 1956;; 26:1109.
13 +
Benjamin JT:  Fatal splenic sequestration crisis . Va Med 1979;;106:679.
14 +
Rao KP, Seeler RA:  Acute splenic sequestration crisis in an adolescent with S-C hemoglobinopathy . Postgrad Med 1979;;65:159.
15 +
Seeler RA, Shwiaki MZ:  Acute splenic sequestration crisis in young children with sickle cell anemia . Clin Pediatr 1972;;11:701.
16 +
Topley JM, Rogers DW, Stevens MC, et al:  Acute splenic sequestration crisis and hypersplenism in the first five years in homozygous sickle cell disease . Arch Dis Child 1981;;56:765.
17 +
Githens JH, Gross GP, Eife RF, et al:  Splenic sequestration syndrome at mountain altitudes in sickle/hemoglobin C disease . J Pediatr 1977;;90:203.
18 +
Gilman PA, Abel AS:  Acute splenic sequestration in hemoglobin sickle O-Arab disease . Johns Hopkins Med J 1980;;146:285.
19 +
Pearson HA:  Hemoglobin S-thalassemia syndrome in Negro children . Ann NY Acad Sci 1969;;165:83.
20 +
Ducos ER, Zusman J, Smith B, et al:  Emergencies in sickle cell anemia in children . J La State Med Soc 1980;;132:71.
21 +
Seeler RA:  Deaths in children with sickle cell anemia: A clinical analysis of 19 fatal instances in Chicago . Clin Pediatr 1972;;11:634.
22 +
Erickson WD, Burgert ED, Lynn HB:  The hazard of infection following splenectomy in children . AJDC 1968;;116:1.
23 +
Walker W:  Splenectomy in childhood: A review in England and Wales, 1960-1964 . Br J Surg 1976;;63:36.
24 +
Singer DB:  Post-splenectomy sepsis , in Rosenberg HA, Borlande RD (eds): Perspectives in Pediatric Pathology . Chicago, Year Book Medical Publishers, 1973;, vol 1, p 285.
25 +
Dickerman JD:  Splenectomy and sepsis: A warning . Pediatrics 1979;;63:938.
26 +
Pegelow CH, Wilson B, Overturf GD, et al:  Infection in splenectomized sickle cell disease patients . Clin Pediatr 1980;;19:102.
27 +
Rausen AR:  Acute splenic sequestration in sickle cell anemia: An indication for splenectomy . Proc Am Soc Pediatr Res , 1967;, p 106.
28 +
Edmond AM, Morais P, Venugopal S, et al:  Role of splenectomy in homozygous sickle cell disease in children . Lancet 1984;;1:88.
29 +
Platt O, Nathan DG:  Sickle cell disease , in Nathan DG, Oski FA (eds): Hematology of Infancy and Childhood . Philadelphia, WB Saunders Co, 1981;, p 706.
30 +
O'Brien RT, McIntosh S, Aspnes GT, et al:  Prospective study of sickle cell anemia in infancy . J Pediatr 1976;;89:205.
31 +
Pearson HA, Spencer RP, Cornelius EA:  Functional asplenia in sickle cell anemia . N Engl J Med 1969;;281:923.
32 +
MacIver JE, Parker-Williams EJ:  The aplastic crisis in sickle cell anemia . Lancet 1961;;1:1086.
33 +
Serjeant GR, Topley JM, Mason K, et al:  Outbreak of aplastic crisis in sickle cell anemia associated with parvovirus-like agent . Lancet 1981;;2:595.
34 +
Mann JR, Cotter KP, Walker RA, et al:  Anaemic crisis in sickle cell disease . J Clin Pathol 1975;;28:341.
35 +
Pardoll DM, Rodeheffer RJ, Smith RR, et al:  Aplastic crisis due to extensive bone marrow necrosis in sickle cell disease . Arch Intern Med 1982;;142:2223.
36 +
Pierce LE, Rath CE:  Evidence for folic acid deficiency in the genesis of anemic sickle cell crisis . Blood 1962;;20:19.
37 +
Rao KR, Patel AR, Anderson MJ, et al:  Infection with parvovirus-like virus and aplastic crisis in chronic hemolytic anemia . Ann Intern Med 1983;;98:930.
38 +
Pattison JR, Jones SE, Hodgson J, et al:  Parvovirus infections and hypoplastic crisis in sickle-cell anemia . Lancet 1981;;1:644.
39 +
Young N, Mortimer P:  Viruses and bone marrow failure . Blood 1984;;63:729.
40 +
McCurdy PR:  32-DFP 51-Cr for measurement of red cell life span in abnormal hemoglobin syndromes . Blood 1969;;33:214.
41 +
Sargeant GR:  The irreversibly sickled cell: A determinant of hemolysis in sickle cell anaemia . Br J Hematol 1969;;17:527.
42 +
Singer K, Fisher B:  Studies on abnormal hemoglobins: V. The distribution of type S sickle cell hemoglobin and type F alkali resistant hemoglobin within the red cell population in sickle cell anemia . Blood 1952;;7:1216.
43 +
Pearson HA, Diamond LK:  The critically ill child: Sickle cell disease crisis and their management . Pediatrics 1971;;48:629.
44 +
Sydenstricker VP, Mulherin WA, Houseal RW, et al:  Sickle cell anemia: Report of two cases in children with necropsy in one . AJDC 1923;; 26:132.
45 +
Portnoy BA, Herion JC:  Neurological manifestations in sickle cell disease . Ann Intern Med 1972;;75:643.
46 +
Wood DH:  Cerebrovascular complications of sickle cell anemia . Stroke 1978;;9:73.
47 +
Powars D:  The natural history of stroke in sickle cell disease . Am J Med 1978;;65:461.
48 +
Wertham F, Mitchell N, Angrist A, et al:  The brain in sickle cell anemia . Arch Neurol Psych 1942;;47:752.
49 +
Stockman JA, Nigro MA, Mishkin MM, et al:  Occlusions of large cerebral vessels in sickle cell anemia . N Engl J Med 1972;;287:846.
50 +
Gerald B, Sebes JI, Langston JW:  Cerebral infarction secondary to sickle cell disease: Arteriographic findings . AJR 1980;;134:1209.
51 +
Garza-Mercado R:  Pseudomoyamoya in sickle cell anemia . Surg Neurol 1982;;18:425.
52 +
Jeffries BF, Lipper MH, Kishore PR:  Major intracerebral arterial involvement in sickle cell disease . Surg Neurol 1980;;14:291.
53 +
Russell MO, Goldberg HI, Hodson A, et al:  Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease . Blood 1984;;63:162.
54 +
Seeler RA, Royal JE, Powe L, et al:  Moyamoya in children with sickle cell anemia and cerebrovascular occlusion . J Pediatr 1978;; 93:808.
55 +
Seeler RA, Royal JE:  Acute and chronic management of children with sickle cell anemia and cerebrovascular occlusive disease . Ill Med J 1977;;147:267.
56 +
Russell MO, Goldberg HI, Reis L, et al:  Transfusion therapy for cerebrovascular abnormalities in sickle cell disease . J Pediatr 1976;; 88:382.
57 +
Wilimas J, Goff JR, Anderson HR, et al:  Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents . J Pediatr 1980;; 96:205.
58 +
Seeler RA, Royal JE:  Commentary: Sickle cell anemia, stroke and transfusion . J Pediatr 1980;;96:243.
59 +
Lusher JM, Haghighat H, Khalita AS:  A prophylactic transfusion program for children with sickle cell anemia complicated by CNS infarction . Am J Hematol 1976;;1:265.
60 +
Buchanan GR, Bowman WP, Smith SJ:  Recurrent cerebral ischemia during hypertransfusion therapy . J Pediatr 1983;;103:921.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Herrick JB:  Peculiar elongated and sickle-shaped red corpuscles in a case of severe anemia . Arch Intern Med 1910;;6:517.
2 +
Pauling L, Itano HA:  Sickle cell anemia, a molecular disease . Science 1949;;110:543.
3 +
Ingram VM:  A specific chemical difference between the globins of normal human and sickle cell anemia hemoglobin . Nature 1956;;178:792.
4 +
Lessin LS, Jensen WN:  Sickle cell anemia 1910-1973 . Arch Intern Med 1974;;133:529.
5 +
Motulsky AG:  Frequency of sickling disorders in US blacks . N Engl J Med 1973;;288:31.
6 +
Lehman H, Huntsman RG:  The hemoglobinopathies , in Stanburg JB, Wyngarden JB, Fredrickson DS (eds): The Metabolic Basis of Inherited Disease . New York, McGraw-Hill Book Co, 1982;, p 1404.
7 +
Pearson HA:  Sickle cell syndromes and other hemoglobinopathies , in Miller DR, Pearson HA (eds): Smith's Blood Diseases of Infancy and Childhood , ed 4. St Louis, CV Mosby Co, 1978;, pp 407-429.
8 +
Horne MK II:  Sickle cell anemia as a rheologic disease . Am J Med 1981;;70:288.
9 +
 National Institutes of Health: Improved methods of blood transfusion for patients with sickle cell anemia . JAMA 1983;;249:699.
10 +
Bertles JF, Milner PF:  Irreversibly sickled erythrocytes: A consequence of the heterogenous distribution of hemoglobin types in sickle-cell anemia . J Clin Invest 1968;;47:1731.
11 +
Serjeant GR, Milner P:  The irreversibly sickled cell: A determinant of haemolysis in sickle cell anemia . Br J Haematol 1969;;17:527.
12 +
Diggs LW:  The crisis in sickle cell anemia: Hematologic studies . Am J Clin Pathol 1956;; 26:1109.
13 +
Benjamin JT:  Fatal splenic sequestration crisis . Va Med 1979;;106:679.
14 +
Rao KP, Seeler RA:  Acute splenic sequestration crisis in an adolescent with S-C hemoglobinopathy . Postgrad Med 1979;;65:159.
15 +
Seeler RA, Shwiaki MZ:  Acute splenic sequestration crisis in young children with sickle cell anemia . Clin Pediatr 1972;;11:701.
16 +
Topley JM, Rogers DW, Stevens MC, et al:  Acute splenic sequestration crisis and hypersplenism in the first five years in homozygous sickle cell disease . Arch Dis Child 1981;;56:765.
17 +
Githens JH, Gross GP, Eife RF, et al:  Splenic sequestration syndrome at mountain altitudes in sickle/hemoglobin C disease . J Pediatr 1977;;90:203.
18 +
Gilman PA, Abel AS:  Acute splenic sequestration in hemoglobin sickle O-Arab disease . Johns Hopkins Med J 1980;;146:285.
19 +
Pearson HA:  Hemoglobin S-thalassemia syndrome in Negro children . Ann NY Acad Sci 1969;;165:83.
20 +
Ducos ER, Zusman J, Smith B, et al:  Emergencies in sickle cell anemia in children . J La State Med Soc 1980;;132:71.
21 +
Seeler RA:  Deaths in children with sickle cell anemia: A clinical analysis of 19 fatal instances in Chicago . Clin Pediatr 1972;;11:634.
22 +
Erickson WD, Burgert ED, Lynn HB:  The hazard of infection following splenectomy in children . AJDC 1968;;116:1.
23 +
Walker W:  Splenectomy in childhood: A review in England and Wales, 1960-1964 . Br J Surg 1976;;63:36.
24 +
Singer DB:  Post-splenectomy sepsis , in Rosenberg HA, Borlande RD (eds): Perspectives in Pediatric Pathology . Chicago, Year Book Medical Publishers, 1973;, vol 1, p 285.
25 +
Dickerman JD:  Splenectomy and sepsis: A warning . Pediatrics 1979;;63:938.
26 +
Pegelow CH, Wilson B, Overturf GD, et al:  Infection in splenectomized sickle cell disease patients . Clin Pediatr 1980;;19:102.
27 +
Rausen AR:  Acute splenic sequestration in sickle cell anemia: An indication for splenectomy . Proc Am Soc Pediatr Res , 1967;, p 106.
28 +
Edmond AM, Morais P, Venugopal S, et al:  Role of splenectomy in homozygous sickle cell disease in children . Lancet 1984;;1:88.
29 +
Platt O, Nathan DG:  Sickle cell disease , in Nathan DG, Oski FA (eds): Hematology of Infancy and Childhood . Philadelphia, WB Saunders Co, 1981;, p 706.
30 +
O'Brien RT, McIntosh S, Aspnes GT, et al:  Prospective study of sickle cell anemia in infancy . J Pediatr 1976;;89:205.
31 +
Pearson HA, Spencer RP, Cornelius EA:  Functional asplenia in sickle cell anemia . N Engl J Med 1969;;281:923.
32 +
MacIver JE, Parker-Williams EJ:  The aplastic crisis in sickle cell anemia . Lancet 1961;;1:1086.
33 +
Serjeant GR, Topley JM, Mason K, et al:  Outbreak of aplastic crisis in sickle cell anemia associated with parvovirus-like agent . Lancet 1981;;2:595.
34 +
Mann JR, Cotter KP, Walker RA, et al:  Anaemic crisis in sickle cell disease . J Clin Pathol 1975;;28:341.
35 +
Pardoll DM, Rodeheffer RJ, Smith RR, et al:  Aplastic crisis due to extensive bone marrow necrosis in sickle cell disease . Arch Intern Med 1982;;142:2223.
36 +
Pierce LE, Rath CE:  Evidence for folic acid deficiency in the genesis of anemic sickle cell crisis . Blood 1962;;20:19.
37 +
Rao KR, Patel AR, Anderson MJ, et al:  Infection with parvovirus-like virus and aplastic crisis in chronic hemolytic anemia . Ann Intern Med 1983;;98:930.
38 +
Pattison JR, Jones SE, Hodgson J, et al:  Parvovirus infections and hypoplastic crisis in sickle-cell anemia . Lancet 1981;;1:644.
39 +
Young N, Mortimer P:  Viruses and bone marrow failure . Blood 1984;;63:729.
40 +
McCurdy PR:  32-DFP 51-Cr for measurement of red cell life span in abnormal hemoglobin syndromes . Blood 1969;;33:214.
41 +
Sargeant GR:  The irreversibly sickled cell: A determinant of hemolysis in sickle cell anaemia . Br J Hematol 1969;;17:527.
42 +
Singer K, Fisher B:  Studies on abnormal hemoglobins: V. The distribution of type S sickle cell hemoglobin and type F alkali resistant hemoglobin within the red cell population in sickle cell anemia . Blood 1952;;7:1216.
43 +
Pearson HA, Diamond LK:  The critically ill child: Sickle cell disease crisis and their management . Pediatrics 1971;;48:629.
44 +
Sydenstricker VP, Mulherin WA, Houseal RW, et al:  Sickle cell anemia: Report of two cases in children with necropsy in one . AJDC 1923;; 26:132.
45 +
Portnoy BA, Herion JC:  Neurological manifestations in sickle cell disease . Ann Intern Med 1972;;75:643.
46 +
Wood DH:  Cerebrovascular complications of sickle cell anemia . Stroke 1978;;9:73.
47 +
Powars D:  The natural history of stroke in sickle cell disease . Am J Med 1978;;65:461.
48 +
Wertham F, Mitchell N, Angrist A, et al:  The brain in sickle cell anemia . Arch Neurol Psych 1942;;47:752.
49 +
Stockman JA, Nigro MA, Mishkin MM, et al:  Occlusions of large cerebral vessels in sickle cell anemia . N Engl J Med 1972;;287:846.
50 +
Gerald B, Sebes JI, Langston JW:  Cerebral infarction secondary to sickle cell disease: Arteriographic findings . AJR 1980;;134:1209.
51 +
Garza-Mercado R:  Pseudomoyamoya in sickle cell anemia . Surg Neurol 1982;;18:425.
52 +
Jeffries BF, Lipper MH, Kishore PR:  Major intracerebral arterial involvement in sickle cell disease . Surg Neurol 1980;;14:291.
53 +
Russell MO, Goldberg HI, Hodson A, et al:  Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease . Blood 1984;;63:162.
54 +
Seeler RA, Royal JE, Powe L, et al:  Moyamoya in children with sickle cell anemia and cerebrovascular occlusion . J Pediatr 1978;; 93:808.
55 +
Seeler RA, Royal JE:  Acute and chronic management of children with sickle cell anemia and cerebrovascular occlusive disease . Ill Med J 1977;;147:267.
56 +
Russell MO, Goldberg HI, Reis L, et al:  Transfusion therapy for cerebrovascular abnormalities in sickle cell disease . J Pediatr 1976;; 88:382.
57 +
Wilimas J, Goff JR, Anderson HR, et al:  Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents . J Pediatr 1980;; 96:205.
58 +
Seeler RA, Royal JE:  Commentary: Sickle cell anemia, stroke and transfusion . J Pediatr 1980;;96:243.
59 +
Lusher JM, Haghighat H, Khalita AS:  A prophylactic transfusion program for children with sickle cell anemia complicated by CNS infarction . Am J Hematol 1976;;1:265.
60 +
Buchanan GR, Bowman WP, Smith SJ:  Recurrent cerebral ischemia during hypertransfusion therapy . J Pediatr 1983;;103:921.

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