الفهرس 
Sickle cell diseaseOnly 14 pages are availabe for public view
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Abstract
Sickle cell nephropathy is an important cause of morbidity in SCD patients, early clinical detection of the manifestation of these process as well as detection its risk factors may permit early prompt treatment and may halters further progression to ESRD .
The present study was intended to evaluate the effect of sickle hemoglobinopathy on renal function both glomerular and tubular, and to find out the prevalence of microalbuminuria in Pediatric patients with sickle cell disease, sickle β thalassemia and sickle cell trait. Evaluation of the effect of the drug angiotensine converting enzyme inhibitor (captopril) on microalbuminuria after 4 weeks of treatment was also done.
The study was conducted on 60 patients, 12 patients with sickle cell disease (group I ) , 28 patients with sickle beta thalassemia (group II) and 20 patients with sickle cell trait (group III) .
Group I, 12 patients with sickle cell disease 5 are females (41.7%) and 7 are males (58.3%). Their mean age was ( 10.71 ± 5.28).
Group II, 28 patients with sickle β thalassemia 10 are females (35.7%) and 18 are males (64.3%) .Their mean age was (12.82 ± 6.44)
Group III, 20 patients with sickle cell trait 12 are females (60%) and 8 are males (40%). Their mean age was (14.3 ± 4.95) served as a control group.
All patients were subjected to full history taking laying stress on Age of patient, Number of Blood transfusion setting, Symptoms of hypertension as headache, vomiting and blurring of vision, Urinary symptoms, Swelling of both lower limbs, Number of hemolytic crisis and Family history of co-morbid condition like diabetes , congestive heart failure and renal disease and thorough clinical examination including anthropometric measurements.
All patients were subjected to the following investigation as complete blood count, complete urine analysis, assessment of glomerular filtration rate utilizing creatinine clearance, measurement of microalbuminuria as a predictor of early nephropathy and measurement of blood and urine osmolality after 12 hours of water deprivation to evaluate tubular concentrating ability.
The study results showed that no significant difference between the three groups as regards the mean of age, sex distribution, consanguinity, weight and weight percentile and height and height percentile .
There was significant difference between three groups as regards pain crisis, which were significantly higher in sickle cell disease and sickle β thalassemia than sickle cell trait with no significant difference between both diseased groups.
A vascular necrosis of head of femur occurred in 1 patient (8.3%) with sickle cell disease, 1 patient (3.6%) with sickle β thalassemia and no one of sickle cell trait showed this complication. Comparison between 3 groups showed no significant difference as regards avascular necrosis of head of femur.
Acute chest syndrome occurred in 2 patients (16.7%) in group I , 1 patient (3.6%) in group II, and no one of group III showed this complication.5 patients (41.7%) of group I, 12 patients (42.9%) of group II had splenomegaly and no patients of group III showed splenomegaly. The difference between the 3 groups was statistically significant P<0.05.
4 patients (33.3%) of group I, 10 patients (35.7%) of group II had hepatomegaly and no patients of group III showed hepatomegaly. Both sickle cell disease and sickle β thalassemia groups showed significantly higher % of patients with hepatomegaly than sickle cell trait group. The difference between the 3 groups was statistically significant P<0.05.
Hematuria was detected in 4 patients (33.3%) of group I, 6 patients (21.4%) of group II and 4 patients (20%) group III .There was no statistically significant difference between the 3 groups as regards the distribution of hematuria P>0.05
UTI was detected in 6 patients (50%) of group I, 10 patients (35.7%) of group II and 7 patients (35%) group III. There was no statistically significant difference between the 3 groups as regards the distribution of UTI P>0.05
Group I received 5.5±3.66 times of blood transfusion / year while group II received 7±3.45 times of blood transfusion / year. The age of start of blood transfusion was 1.35±0.57 year in group I and 1.61±1.02 year in group II. regarding the number of pain crisis it was 1.75 ±1.36 in group I while it was 2.75±1.53 in group II .There was no statistically significant difference between group I and group II as regards the mean no of blood transfusion /year, age of start of blood transfusion and no of pain crisis P>0.05
There was statistically significant difference between group I, group II and group III as regards the mean of WBCs, Hb, HbA, HbS, HbA2, HbF, and Pl. P<0.05
Group I and group II showed a statistically significant lower mean of Hb, and HbA and a statistically significant higher mean WBCs, and platelets.
The mean of microalbuminuria was high in group I (93.03 ± 146.27) than in group II (63.82 ± 172.38) and it was normal in group III (18.75 ± 12.86)). The mean of creatinine clearance in group I was (131.94 ± 84.10) , in group II (137.78 ± 73.13 )and in group III it was (152.56 ± 46.11) . The mean of serum creatinine in group I was (0.49 ± 0.14), in group II ( 0.54 ± 0.29 )and in group III ( 0.46 ± 0.15) . The mean of urine creatinine in group I was (44.88 ± 16.05), in group II (48.99 ± 17.26) and in group III (60.73 ± 34.07). The mean of urine osmolality in group I was ( 207.08 ± 79.82), in group II (169.14 ± 77.49) and in group III (183.9 ± 43.13)
Microalbuminuria was detected in 13/40 patients (32.5%) of groups I and II and it was detected in 1/20 patients (5%) of group III. The difference between the two groups is statistically significant P<0.05. .
Level of microalbuminuria was significantly higher in group I (93.03 ±146.27) than in group II (63.82 ± 172.38) than in group III (18.75 ± 12.86)
Level of microalbuminuria was found to be higher in patients with hematuria and pain crisis than in patients without hematuria and pain crisis
Comparison between patients with positive microalbuminuria and negative microalbuminuria regarding all studied parameters revealed significant higher mean age (18.31 ± 14.48) versus ((9.24 ± 4.96), serum creatinine (0.63 ± 0.31) versus (0.47 ± 0.19 ) urine volume (1498.15 ± 627.2) versus (952.22 ± 398.82) , number of blood transfusion (7.08 ± 3.55), versus (5.11 ± 4.05), number of hemolytic crisis (2.38 ± 1.98) versus (1.11 ± 1.19) and number of pain crisis (2.92 ± 1.61) versus (2.22 ± 1.48) in patients with positive microalbuminuria than in patients with negative microalbuminuria . No significant difference could be detected regarding WBCs, Hb, HbF and HbS.
Odds Ratio = 6.5 (95% Confidence Interval = 0.91-46.23) i.e. group I and group II were subjected to risk of microalbuminuria 6.5 times than group III .
There was a statistically significant higher mean age, serum creatinine, and higher urine volume in cases with positive microalbuminuria P<0.05
There was a statistically significant higher mean number of blood transfusion / year, of hemolytic crisis and of pain crisis in presence of microalbuminuria.
Patients with positive microalbuminuria in group I and group II received angiotensin converting enzyme inhibitor (captopril ) for 4 weeks. There was significant decrease in % of affected patients from 13/40 patients (32.5%) to 3/40 patients (7.5%) with microalbuminuria after treatment p =0.005. Moreover there was a statistically significant DROP of level of microalbuminuria after treatment (27.11 ± 13.9) than before treatment (171.9 ± 251.6) p<0.05.
There was no statistically significant difference between group I, group II and group III as regards urine and serum osmolality P>0.05.
Creatinine clearance was high in 3 patients (25%) of group I, 11 patients (39.3%) of group II and 13 patients (65%) of group III.
comparison between the 3 studied groups as regards creatinine clearance revealed a statistically significant higher % of patients with high creatinine clearance in group III than in group I and group II P=0.05, while no statistically significant difference could be detected between group I and group II (P=0.49 ).
We could conclud that sickle cell nephropathy may manifest as proteinuria and occur in all forms of the disease, sickle cell disease, sickle β thalassemia and sickle cell trait. It was associated with the severity of the disease . Early detection of proteinuria may allow therapy and prevention of further progression.
ACE inhibitor therapy can reduce albuminuria, its long term efficacy for children with SCD should be evaluated in amelioration of proteinuria and in prevention of the development of renal insufficiency.