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Chronic haemolytic anaemia associated with adenylate kinase (AK) deficiency is very rare and only seven cases in five families have been described. We present six children of one family who are deficient of this enzyme and in three of them a combined G6PD deficiency was found. AK deficiency was transmitted by an autosomal recessive gene and heterozygous state was not accompanied by disease, whereas homozygously affected individuals present a congenital chronic non-spherocytic haemolytic anaemia with haemoglobin levels of 8-9 g/dl. Patients also deficient in G6PD suffer from a more severe haemolytic anaemia with haemoglobin levels around 6 g/dl. The AK-deficient children are also mentally retarded. Splenectomy performed in five of the six patients resulted in complete remission of the haemolytic process.
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
January 1964,
Vox sanguinis,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
August 1963,
Acta haematologica,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
December 1967,
The New Zealand medical journal,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
July 1963,
Lancet (London, England),
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
June 1970,
La Presse medicale,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
January 1971,
Folia medica Neerlandica,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
January 1979,
British journal of haematology,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
January 1979,
British journal of haematology,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
January 1968,
Acta paediatrica Academiae Scientiarum Hungaricae,
A Toren, and
F Brok-Simoni, and
I Ben-Bassat, and
F Holtzman, and
M Mandel, and
Y Neumann, and
B Ramot, and
G Rechavi, and
G Kende
December 1997,
British journal of haematology,
