GLOMERULAR
FILTRATiON
RATE
IN
CHILDREN
WITH
SICKLE
CELL
DISEASE
By PHILIP L. CALCAGNO, M.D., JOHN MCLAvy, B.S., AND THOMAS KELLEY, M.D.
Canal Zone
S
TUDIES of kidney function in sickle cell anemia have not been previously reported. Diggs’ considered the degenerative and proliferative changes in kidneys to be aconstant feature of long standing evere cases of sickle cell anemia. Engorgement with
disfigured red blood corpuscles was encountered in the distended capillaries of the
kidneys in patients with sickle cell disease.2 Yater3 found sclerosis of the glomeruli in
the kidney on routine necropsy reports. It seemed reasonable that a study of the function-ing kidney might reveal information which would not consistently appear at pathologic
examination.
CLINICAL MATERIAL AND METHODS
Sodium thiosulfate was used to determine the rate of glomerular filtration as described by Newman et al.4 The patients were fasting in the basal state. Collections of specimens of blood and urine for blank determinations were made before injection of sodium thiosulfate. A 6% sodium
thiosulfate solution was injected within 10 to 15 mm. The dosage was from 0.3 to 1.0 gm./4.5 kg. body weight. Fifteen minutes were allowed for equilibration throughout body fluids. Blood samples were taken from the arm opposite the one into which the injection had been made. Urine was collected
from the bladder by a catheter and the bladder was washed out with 10 to 20 cc. of sterile water. The diagnosis of sickle cell anemia was made when 95 to 100% sickling was observed in
smears from the peripheral blood associated with abnormally high reticulocyte and leucocyte counts. Only patients with hemoglobin levels below 50% and those who had not received previous trans-fusions, except for Patient 8, were accepted for the study.
Clearances were measured on patients with the following disorders: 5 patients with sickle cell
anemia, 2 patients with the sickle cell trait and 1 patient with chronic anemia secondary to uncinariasis.
RESULTS
Results are shown in Table I which presents all the pertinent data. Five patients with sickle cell anemia presented below average glomerular filtration rates. All values were corrected to 1.73 sq. m. of body surface area. Two patients who exhibited the sickle cell trait only showed a normal glomerular filtration rate.
Patient 8, who was said to have a chronic anemia secondary to uncinariasis for 1 yr., showed
normal glomerular filtration. Since this patient received 2 transfusions prior to the measurement of the glomerular filtration rate, the significance of this rate was not fully understood.
DISCUSSION
It has been shown that a renal functional abnormality develops in chronic sickle cell
anemia. There may be several possible explanations regarding the pathogenesis for the
low filtration rates in these patients.
Bradley5 was impressed with the afferent arteriolar vasoconstriction in patients with
chronic anemia which was indicated after finding low filtration fractions and a larger reduction in renal blood flows. Whether reduced renal blood flow occurs in sickle cell
From Gorgas Hospital, Canal Zone.
12g
P. L. CA1LANO,
J.
McLAW
ANt
T. I(1tLEY
TABLE I
Subject Wt.
kg. Age Sex
Hgb. gm. Body surface sq.m. Periods Glomerular filtration ml./min./i.73 sq.m. Diagnosis 1. R.L. 2. L.N. 3. D.U. 4. L.F. 5.H.C. 6. G.S. 7. E.J. 8. G.DeL. 29.6 13.6 21.3 41.3 22.7 25.0 15.4 54. 5 9 4 9 15 12 7 6 19 M F M M F M F M 6.8 7.0 6.5 7.2 7.0 13.4 14.0 5 .9 1 .89 0.612 0.871 1.370 0.890 0.958 0.672
1.620
6 3 3 3 3 . 3 2 3 74. 3 30.7 69 6 94.0 70.6 120.7 150.0
144 .5
Sickle cell anemia
“ “ “ “ “ “ “ “ “ “ “ “ Sickle trait “ “ Anemia secondary to uncinariasis
Mean value glomerular filtration rate 131 ml./min./1.73 sq. m.
Normal male above 2 yrs.
Mean value glomerular filtration rate. . 117 ml./min./1.73 sq. m.
Normal female above 2 yrs.
Glomerular filtration rate 45-120/1.73 sq. m.
Range under 2 yrs.
anemia with a concomitant fall in filtration fraction will not be known until further
studies are accomplished. Circulatory stasis in the small vessels of internal organs, muscle
and other tissue has been demonstrated in patients with sickle cell traits ;6 yet two
patients with the sickle cell trait revealed a normal glomerular filtration rate. One would
presume the striking difference in the patients with sickle cell disease as compared to
the patients with the sickle cell trait may be due to the severe chronic anemia.
Congestive heart failure and shock7 were not present in these cases. Consequently, these
factors could not be used to explain the lowered glomerular filtration rate.
SUMMARY
Renal function has been studied quantitatively in five patients with sickle cell anemia,
all of whom were proved to have reduced glomerular filtration rates. Two patients with
sickle cell trait revealed normal glomerular filtration rates. Possible mechanisms
responsi-ble for reduced glomerular filtration flows are discussed.
REFERENCES
1. Diggs, L. W., and Ching, R. E., Pathology of sickle cell anemia, South M. J. 27:839, 1934.
2. Bauer, J., Sickle cell disease, Arch. Surg. 41 : 1344, 1940.
3. Yater, W. M., and Hansmann, G. H., Sickle cell anemia: New cause of cor-pulmonale, Am. J.M. Sc. 191:474, 1936.
4. Newman, E. V., Gilman, A., and Philips, F. S., Renal clearance of thiosulfate in man, Bull.
Johns Hopkins Hosp. 79:229, 1946.
GLOMERULAR FILTRATION RATE IN SICKLE CELL DISEASE 129
SPANISH ABSTRACT
Proporci#{243}n de Filtraci#{243}n Glomerular en Ni#{241}oscon Enfermedad Hemati#{233} Falciforme
Sc ha estudiado Ia funci#{243}n renal cuantitativamente en 5 acientes con anemia hemati#{233} falciforme, de
los cuales todos demostraron tener proporciones de filtraci#{243}n glomerular reducidas. Dos pacicntes con caractcrlsticas de hemati#{233} falciforme revelaron proporciones de filtraci#{243}n glomerular normales. Se
tratan los mecanismos que pueden ser responsables por las corrientes de filtraci#{243}n glomerular reducidas.
