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July 1971, Vol 122, No. 1 >
Article | July 1971

Glucose-6-Phosphate Dehydrogenase Deficiency and Hyperbilirubinemia in the Newborn FREE

Rafael Lopez, MD; Jack M. Cooperman, PhD
[+] Author Affiliations

Received for publication Nov 12, 1970; accepted April 14, 1971.

Reprint requests to New York Medical College, Flower and Fifth Ave Hospital, 1249 Fifth Ave, New York 10029 (Dr. Lopez).


Am J Dis Child. 1971;122(1):66-70. doi:10.1001/archpedi.1971.02110010102020.
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Comments

In four cases of newborns with hyperbilirubinemia and no blood group incompatibilities between mother and infant, neither mothers nor infants had previously received drugs associated with hemolytic anemia. Investigations of the infants and their families revealed that the infants had glucose-6-phosphate dehydrogenase (G-6-PD) deficiency in the red blood cells (RBC). Prematurity, acidosis, hypoxia, and factors normally associated with the newborn in addition to the inability of the RBC deficient in G-6-PD to metabolize hydrogen peroxide may cause acute hemolytic episode in these infants even in the absence of drug ingestion or infection. Infants, especially those from ethnic groups known to have a high incidence of G-6-PD deficiency, with hyperbilirubinemia and no evidence of blood group incompatibilities should be screened for G-6-PD deficiency.

REFERENCES

1 +
Smith GD, Vella F:  Erythrocyte enzyme deficiency in unexplained kernicterus . Lancet 1:1133-1134, 1960;.
2 +
Panizon F:  Erythrocyte enzyme deficiency in unexplained kernicterus . Lancet 2:1093, 1960;.
3 +
Gilles HM, Taylor BG:  The existence of glucose-6-phosphate dehydrogenase deficiency trait in Nigeria and its clinical implications . Ann Trop Med Parasit 55:64-69, 1961;.
4 +
Fessas PH, Doxiadis SA, Valaes T:  Neonatal jaundice in glucose-6-phopshate dehydrogenase deficient infants . Brit Med J 2:1359-1362, 1962;.
5 +
Flatz G, Sringam S, Komkris V:  Neonatal jaundice in glucose-6-phosphate dehydrogenase deficiency . Lancet 1:1382-1383, 1963;.
6 +
Schettini F, Meloni T, Corda G, et al:  Red cells survival in jaundiced newborn children with erythrocyte enzyme deficiency . Lancet 2:1012, 1963;.
7 +
Levin SE, Charlton RW, Frieman I:  Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice in South African Bantu infants . J Pediat 65:757-764, 1964;.
8 +
Doxiadis SA, Valaes T, Karaklis A, et al:  Risk of severe jaundice in glucose-6-phosphate dehydrogenase deficiency of the newborn: Differences in population groups . Lancet 2:1210-1212, 1964;.
9 +
Yue PCK, Strickland M:  Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice in Chinese male infants in Hong Kong . Lancet 1:350-351, 1965;.
10 +
Lu TC, Wei H, Blackwell RQ:  Increased incidence of severe hyperbilirubinemia among newborn Chinese infants with glucose-6-phosphate dehydrogenase deficiency . Pediatrics 37:994-999, 1966;.
11 +
Brown WR, Wong HB:  Hyperbilirubinemia and kernicterus in glucose-6-phosphate dehydrogenase deficient infants in Singapore . Pediatrics 41:1055-1062, 1968;.
12 +
Phornphutkul C, Whithaker JA, Worathumrong N:  Severe hyperbilirubenemia in Thai newborns in association with erythrocyte glucose-6-phosphate dehydrogenase deficiency . Clin Pediat 8:275-278, 1969;.
13 +
O'Flyn M, Hsia DYY:  Serum bilirubin levels and glucose-6-phosphate dehydrogenase deficiency in newborn American Negroes . J Pediat 63:160-161, 1963;.
14 +
Zinkham WH:  Peripheral blood and bilirubin values in normal full-term primaquine sensitive Negro infants . Pediatrics 31:983-995, 1963;.
15 +
Wolff JA, Grossman BH, Paya K:  Neonatal serum bilirubin and glucose-6-phosphate dehydrogenase: Relationship of various perinatal factors to hyperbilirubinemia . Amer J Dis Child 113:251-254, 1967;.
16 +
Dacie JV, Lewis SM: Practical Hematology , ed 3. New York, Grune & Stratton Inc, 1963;, pp 390-394.
17 +
Oski FA, Growney PM:  A simplified micromethod for the detection of glucose-6-phosphate dehydrogenase deficiency . J Pediat 66:90-93, 1965;.
18 +
Zinkham WH:  An in vitro abnormality of glutathione metabolism in erythrocyte from normal newborns: Mechanism and clinical significance . Pediatrics 23:18-32, 1969;.
19 +
Oski FA, Brigandi EP:  Red cell metabolism in the premature infant . Pediatrics 39:689-695, 1967;.
20 +
Gross RT, Hurwitz RE:  The pentose phosphate pathway in human erythrocytes . Pediatrics 22:453-460, 1958;.
21 +
Brown AK, San Marco A, Hall R:  Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice . Southern Med J 57:1459, 1963;.
22 +
Brown AK:  Erythrocyte metabolism and hemolysis in the newborn . Pediat Clin N Amer 13:879-903, 1966;.
23 +
Jones PEH, McCance RA:  Enzyme activities in the blood of infants and adults . Biochem J 45:464-467, 1949;.
24 +
Ross JD:  Deficient activity of DPNH-dependent methemoglobin diaphorase in cord blood erythrocytes . Blood 21:51-62, 1963;.
25 +
Gordon HH, Nitowsky HM, Cornblath M:  Studies of tocopherol deficiency in infants and childhood: I. Hemolysis of the erythrocytes in hydrogen peroxide . Amer J Dis Child 90:669-681, 1955;.
26 +
Taylor DW, Wiseman R:  Rate of oxygen uptake of erythrocytes of vitamin E deficient rats . Nature 196:1102-1103, 1962;.
27 +
Namil BM, Munks B, Moyer E, et al:  Vitamin C in the blood and urine of the newborn and in the cord and maternal blood . Amer J Dis Child 74:417-433, 1947;.
28 +
Szeinber A, Marks PA:  Substances stimulating glucose catabolism by the oxidative reactions of the pentose phosphate pathway in human erythrocytes . J Clin Invest 40:914-924, 1961;.
29 +
Tarlov AR, Brewer GJ, Carson PE, et al:  Primaquine sensitivity: Glucose-6-phosphate dehydrogenase deficiency: An inborn error of metabolism of medical and biological significance . Arch Intern Med 109:209-214, 1962;.
30 +
Eshaghpour E, Oski FA, Williams M:  The relationship of erythrocyte glucose-6-phosphate dehydrogenase deficiency to hyperbilirubinemia in Negro premature infants . J Pediat 70:595-601, 1967;.
31 +
Oski FA, Naiman JL: Hematological Problems in the Newborn . Philadelphia and London, WB Saunders Co, 1966;, pp 81-95.

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

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References

1 +
Smith GD, Vella F:  Erythrocyte enzyme deficiency in unexplained kernicterus . Lancet 1:1133-1134, 1960;.
2 +
Panizon F:  Erythrocyte enzyme deficiency in unexplained kernicterus . Lancet 2:1093, 1960;.
3 +
Gilles HM, Taylor BG:  The existence of glucose-6-phosphate dehydrogenase deficiency trait in Nigeria and its clinical implications . Ann Trop Med Parasit 55:64-69, 1961;.
4 +
Fessas PH, Doxiadis SA, Valaes T:  Neonatal jaundice in glucose-6-phopshate dehydrogenase deficient infants . Brit Med J 2:1359-1362, 1962;.
5 +
Flatz G, Sringam S, Komkris V:  Neonatal jaundice in glucose-6-phosphate dehydrogenase deficiency . Lancet 1:1382-1383, 1963;.
6 +
Schettini F, Meloni T, Corda G, et al:  Red cells survival in jaundiced newborn children with erythrocyte enzyme deficiency . Lancet 2:1012, 1963;.
7 +
Levin SE, Charlton RW, Frieman I:  Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice in South African Bantu infants . J Pediat 65:757-764, 1964;.
8 +
Doxiadis SA, Valaes T, Karaklis A, et al:  Risk of severe jaundice in glucose-6-phosphate dehydrogenase deficiency of the newborn: Differences in population groups . Lancet 2:1210-1212, 1964;.
9 +
Yue PCK, Strickland M:  Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice in Chinese male infants in Hong Kong . Lancet 1:350-351, 1965;.
10 +
Lu TC, Wei H, Blackwell RQ:  Increased incidence of severe hyperbilirubinemia among newborn Chinese infants with glucose-6-phosphate dehydrogenase deficiency . Pediatrics 37:994-999, 1966;.
11 +
Brown WR, Wong HB:  Hyperbilirubinemia and kernicterus in glucose-6-phosphate dehydrogenase deficient infants in Singapore . Pediatrics 41:1055-1062, 1968;.
12 +
Phornphutkul C, Whithaker JA, Worathumrong N:  Severe hyperbilirubenemia in Thai newborns in association with erythrocyte glucose-6-phosphate dehydrogenase deficiency . Clin Pediat 8:275-278, 1969;.
13 +
O'Flyn M, Hsia DYY:  Serum bilirubin levels and glucose-6-phosphate dehydrogenase deficiency in newborn American Negroes . J Pediat 63:160-161, 1963;.
14 +
Zinkham WH:  Peripheral blood and bilirubin values in normal full-term primaquine sensitive Negro infants . Pediatrics 31:983-995, 1963;.
15 +
Wolff JA, Grossman BH, Paya K:  Neonatal serum bilirubin and glucose-6-phosphate dehydrogenase: Relationship of various perinatal factors to hyperbilirubinemia . Amer J Dis Child 113:251-254, 1967;.
16 +
Dacie JV, Lewis SM: Practical Hematology , ed 3. New York, Grune & Stratton Inc, 1963;, pp 390-394.
17 +
Oski FA, Growney PM:  A simplified micromethod for the detection of glucose-6-phosphate dehydrogenase deficiency . J Pediat 66:90-93, 1965;.
18 +
Zinkham WH:  An in vitro abnormality of glutathione metabolism in erythrocyte from normal newborns: Mechanism and clinical significance . Pediatrics 23:18-32, 1969;.
19 +
Oski FA, Brigandi EP:  Red cell metabolism in the premature infant . Pediatrics 39:689-695, 1967;.
20 +
Gross RT, Hurwitz RE:  The pentose phosphate pathway in human erythrocytes . Pediatrics 22:453-460, 1958;.
21 +
Brown AK, San Marco A, Hall R:  Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice . Southern Med J 57:1459, 1963;.
22 +
Brown AK:  Erythrocyte metabolism and hemolysis in the newborn . Pediat Clin N Amer 13:879-903, 1966;.
23 +
Jones PEH, McCance RA:  Enzyme activities in the blood of infants and adults . Biochem J 45:464-467, 1949;.
24 +
Ross JD:  Deficient activity of DPNH-dependent methemoglobin diaphorase in cord blood erythrocytes . Blood 21:51-62, 1963;.
25 +
Gordon HH, Nitowsky HM, Cornblath M:  Studies of tocopherol deficiency in infants and childhood: I. Hemolysis of the erythrocytes in hydrogen peroxide . Amer J Dis Child 90:669-681, 1955;.
26 +
Taylor DW, Wiseman R:  Rate of oxygen uptake of erythrocytes of vitamin E deficient rats . Nature 196:1102-1103, 1962;.
27 +
Namil BM, Munks B, Moyer E, et al:  Vitamin C in the blood and urine of the newborn and in the cord and maternal blood . Amer J Dis Child 74:417-433, 1947;.
28 +
Szeinber A, Marks PA:  Substances stimulating glucose catabolism by the oxidative reactions of the pentose phosphate pathway in human erythrocytes . J Clin Invest 40:914-924, 1961;.
29 +
Tarlov AR, Brewer GJ, Carson PE, et al:  Primaquine sensitivity: Glucose-6-phosphate dehydrogenase deficiency: An inborn error of metabolism of medical and biological significance . Arch Intern Med 109:209-214, 1962;.
30 +
Eshaghpour E, Oski FA, Williams M:  The relationship of erythrocyte glucose-6-phosphate dehydrogenase deficiency to hyperbilirubinemia in Negro premature infants . J Pediat 70:595-601, 1967;.
31 +
Oski FA, Naiman JL: Hematological Problems in the Newborn . Philadelphia and London, WB Saunders Co, 1966;, pp 81-95.

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