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May 1955, Vol 89, No. 5 >
Article | May 1955

HYPOPLASTIC ANEMIA WITH MULTIPLE CONGENITAL ANOMALIES (FANCONI SYNDROME) FREE

RAPHAEL GOLDSTEIN, (MC)
AMA Am J Dis Child. 1955;89(5):618-622. doi:10.1001/archpedi.1955.02050110732022.
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IN THE YEAR 1927, Fanconi published three cases of a rather unusual combination of congenital defects associated with what he called at the time a pernicious-like anemia. The cases reported by him were found in three brothers of the same family. Since then a number of similar cases have been published in the European literature as well as in the literature this side of the Atlantic. Although all the cases bear a certain resemblance to each other, it is rather hard to give a precise definition to the syndrome, since there is no good explanation for its etiology, and, what is more, there seem to be a number of variations in the clinical and laboratory findings. There is, however, no doubt that the syndrome exists as a pathological entity.

In his original article, Fanconi described three siblings between the ages of 5 and 7 years with pernicious anemia but which

REFERENCES

1 +
Baumann, T.:  Konstitutionelle Panmyelophthise mit multiplen Abartungen (Fanconi-Syndrom) , Ann. pædiat. 177:65-76, 142-174, 1951;.
2 +
Beautyman, W.:  Case of Fanconi's Anæmia , Arch. Dis. Childhood 26:233-240, 1951;.
3 +
Dacie, J. V., and Gilpin, A.:  Refractory Anæmia (Fanconi Type): Its Incidence in Three Members of One Family, with in One Case a Relationship to Chronic Haemolytic Anaemia with Nocturnal Haemoglobinuria (Marchiafava-Micheli Disease or "Nocturnal Haemoglobinuria," Arch. Dis. Childhood 19:155-162, 1944;.
4 +
Diamond, D., in Nelson W. E., Editor: Mitchel-Nelson Textbook of Pediatrics , Ed. 5, Philadelphia, W. B. Saunders Company, 1950;, p. 1128.
5 +
Diamond, L. K., and Blackfan, K. D.:  Hypoplastic Anemia , Am. J. Dis. Child. 56:464-467, 1938;.
6 +
Fanconi, G.:  Familiäre infantile perniziosaartige Anämie (perniziöses Blutbild und Konstitution) , Jahrb. Kinderh. 117:257-280, 1927;.
7 +
Heuiger; Hurez, and Dauphin:  Myélose aplastique avec infantilisme et malformations , Arch. franc. pédiat. 2:121, 1945;.
8 +
Jimenez de Asua, F., and Escardo, F.:  Anemia constitucional infantil asociada con anomalias somáticas múltiples (síndrome de Fanconi) , Rev. chilena pediat. 18:977-992, 1947;.
9 +
Kohlbry, C. O.:  Congenital Hypoplastic Anemia: Case Report , J. Pediat. 19:662-667, 1941;.
10 +
Kunz, H. W.:  Hypoplastic Anemia with Multiple Congenital Defects (Fanconi Syndrome) , Pediatrics 10:286-291, 1952;.
11 +
Reinhold, J. D. L.; Neumark, E.; Lightwood, R., and Carter, C. O.:  Familial Hypoplastic Anemia with Congenital Abnormalities (Fanconi's Syndrome) , Blood 7:915-926, 1952;.
12 +
Rohr, K.:  Familial Panmyelophthisis: Fanconi Syndrome in Adults , Blood 4:130-141, 1949;.
13 +
Rubell, I.:  Hypoplastic Congenital Anemia , J. Pediat. 20:756-758, 1942;.
14 +
Silver, H. K.; Blair, W. C., and Kempe, C. H.:  Fanconi Syndrome: Multiple Congenital Anomalies with Hypoplastic Anemia , A. M. A. Am. J. Dis. Child. 83:14-25, 1952;.
15 +
van Leeuwen, H. C.:  Ein Fall von konstitutioneller infantiler Perniziosaartiger Anämie (Fanconi) , Folia haemat. 49:434, 1935;.
16 +
Washington, J. A.: in Holt, L. E., Jr., and McIntosh, R.: Holt Pediatrics , Ed. 12, New York, Appleton-Century-Crofts, Inc., 1953;, p. 646.
17 +
Weil, P. E.:  Myélose aplastique infantile familiale avec malformations et troubles endocriniens: Contribution à l'étude du syndrome de Fanconi , Sang 12:369-389, 1938;.

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References

1 +
Baumann, T.:  Konstitutionelle Panmyelophthise mit multiplen Abartungen (Fanconi-Syndrom) , Ann. pædiat. 177:65-76, 142-174, 1951;.
2 +
Beautyman, W.:  Case of Fanconi's Anæmia , Arch. Dis. Childhood 26:233-240, 1951;.
3 +
Dacie, J. V., and Gilpin, A.:  Refractory Anæmia (Fanconi Type): Its Incidence in Three Members of One Family, with in One Case a Relationship to Chronic Haemolytic Anaemia with Nocturnal Haemoglobinuria (Marchiafava-Micheli Disease or "Nocturnal Haemoglobinuria," Arch. Dis. Childhood 19:155-162, 1944;.
4 +
Diamond, D., in Nelson W. E., Editor: Mitchel-Nelson Textbook of Pediatrics , Ed. 5, Philadelphia, W. B. Saunders Company, 1950;, p. 1128.
5 +
Diamond, L. K., and Blackfan, K. D.:  Hypoplastic Anemia , Am. J. Dis. Child. 56:464-467, 1938;.
6 +
Fanconi, G.:  Familiäre infantile perniziosaartige Anämie (perniziöses Blutbild und Konstitution) , Jahrb. Kinderh. 117:257-280, 1927;.
7 +
Heuiger; Hurez, and Dauphin:  Myélose aplastique avec infantilisme et malformations , Arch. franc. pédiat. 2:121, 1945;.
8 +
Jimenez de Asua, F., and Escardo, F.:  Anemia constitucional infantil asociada con anomalias somáticas múltiples (síndrome de Fanconi) , Rev. chilena pediat. 18:977-992, 1947;.
9 +
Kohlbry, C. O.:  Congenital Hypoplastic Anemia: Case Report , J. Pediat. 19:662-667, 1941;.
10 +
Kunz, H. W.:  Hypoplastic Anemia with Multiple Congenital Defects (Fanconi Syndrome) , Pediatrics 10:286-291, 1952;.
11 +
Reinhold, J. D. L.; Neumark, E.; Lightwood, R., and Carter, C. O.:  Familial Hypoplastic Anemia with Congenital Abnormalities (Fanconi's Syndrome) , Blood 7:915-926, 1952;.
12 +
Rohr, K.:  Familial Panmyelophthisis: Fanconi Syndrome in Adults , Blood 4:130-141, 1949;.
13 +
Rubell, I.:  Hypoplastic Congenital Anemia , J. Pediat. 20:756-758, 1942;.
14 +
Silver, H. K.; Blair, W. C., and Kempe, C. H.:  Fanconi Syndrome: Multiple Congenital Anomalies with Hypoplastic Anemia , A. M. A. Am. J. Dis. Child. 83:14-25, 1952;.
15 +
van Leeuwen, H. C.:  Ein Fall von konstitutioneller infantiler Perniziosaartiger Anämie (Fanconi) , Folia haemat. 49:434, 1935;.
16 +
Washington, J. A.: in Holt, L. E., Jr., and McIntosh, R.: Holt Pediatrics , Ed. 12, New York, Appleton-Century-Crofts, Inc., 1953;, p. 646.
17 +
Weil, P. E.:  Myélose aplastique infantile familiale avec malformations et troubles endocriniens: Contribution à l'étude du syndrome de Fanconi , Sang 12:369-389, 1938;.

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