الفهرس 
Chronic kidney diseaseOnly 14 pages are availabe for public view
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Abstract
Chronic kidney disease (CKD) refers to a state of irreversible kidney damage and/or reduction of kidney function that can lead to a progressive decrease in kidney function. It is now the accepted term in the pediatric nephrology community, replacing the clinical terms of chronic renal failure (CRF) and chronic renal insufficiency (CRI), which describe renal dysfunction of varying degrees from severe to mild in nature. CKD more clearly defines renal dysfunction as a continuum, rather than a discrete change in renal function.
Von Willebrand factor (VWF) is a multimeric glycoprotein which mediates platelet adhesion and aggregation. VWF function is partly regulated by the VWF protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) also known as von Willebrand factor-cleaving protease (VWFCP).So, ADAMTS13 is defined as a zinc-containing metalloprotease enzyme that cleaves von Willebrand Factor.
ADAMTS13 cleaves ultra-large VWF multimers into smaller multimers that are less procoagulant Therefore, the imbalance between VWF and ADAMTS13 is an important indicator of a prothrombotic state
Evidence on the association between prothrombotic factors and kidney function is scarce.
Given the dependency of kidney function on the adequate blood flow to the glomerulus, the kidney is one of the most susceptible organs to thrombotic events in its microcirculation. The imbalance between VWF and ADAMTS13 may promote thrombosis in kidney vessels.
Evaluation of ADAMTS13 in patients with chronic kidney diseases has improved our understanding in its role in thrombotic events that affect them.
This has taken on greater importance as the role of VWF in inflammation, angiogenesis, and in thrombotic diseases, such as thrombotic thrombocytopenic purpura and other forms of VWF-mediated thrombosis.
This is one of the hallmark clinical characteristics of thrombotic thrombocytopenic purpura (TTP), which is caused by a complete deficiency of ADAMTS13.
In TTP patients, ADAMTS13 deficiency results in microthrombi formation in the circulation as well as the small vessels of the kidney, contributing to renal insufficiency.
A disintegrin-like and metalloprotease with thrombospondin type 1 repeats 13 (ADAMTS13) is a specific VWF-cleaving protease which responsible for the proteolysis of ultra-large von Willebrand factor (VWF) multimers into smaller multimers.
Severe deficiency of this protease leads to the excessive accumulation of ultra large VWF multimers and platelet aggregation with micro thrombus formation, and is the main cause of the thrombotic events in CKD patients.
The aim of this work was to assess plasma concentration of von Willebrand factor cleaving protease activity in pediatric patient with chronic kidney disease as a marker of thrombosis.
This case control study was conducted on patients attending Pediatric Nephrology unit and outpatient nephrology Clinic of Benha University Hospitals from January 2020 to November 2020. Apparently healthy control children were taken from outpatient clinic. Laboratory work was conducted in Clinical Pathology Department, Benha University.
They were divided as following:
• Patient group (group I): twenty children previously diagnosed with chronic kidney disease on hemodialysis.
• Patient group (group II): twenty children previously diagnosed with chronic kidney disease and on conservative therapy according to Schwartz formula.
• Control group (group III): twenty children apparently healthy from outpatient clinic.
Inclusion criteria:
• All children diagnosed with chronic kidney disease less than 18 years.
• Chronic kidney diseases patients on dialysis and conservative therapy.
• Both sexes were included.
Exclusion criteria:
• Multiple congenital anomalies
• Metabolic disorders.
• Malignancy.
• Sepsis.
Ethical Consideration:
Ethical permission for the study was obtained from the parents who was fully informed about all study procedures and their consent was obtained prior to the children enrollment in the study. This study was approved by the ethical committee of the faculty of Medicine, Benha University Hospitals.
Methods:
Children were subjected to history taking to fulfill needed data:
(1) History taking:
1. Full history taking including the onset of diagnosis of renal disease.
2. Drugs used and their doses.
3. History suggestive of acute metabolic complications, as (sweating, headache, blurring of vision, tremors, convulsions, and coma).
(2) Clinical examination:
General examination:
• Vital signs: pulse, blood pressure, respiratory rate and body temperature.
• Anthropometric measures: weight, height and mid arm circumference.
• Upper limb and lower limb examination.
Local examination:
• Full examination of heart, chest and abdomen.
• Full neurological examination.
(3) Laboratory investigations:
• Complete blood count.
• Blood Urea Nitrogen (BUN).
• Serum creatinine.
• Microalbumin in urine.
• Random blood glucose.
• Serum electrolytes (sodium, potassium, calcium, phosphorus).
• Von Willebrand factor cleaving protease enzyme was assayed once by enzyme-linked immunosorbent assay (ELISA) technique in patients and control groups.
The results of our present study can be summarized as follows:
Weight percentiles is common affected among the studied cases as decreased among cases than controls.
Height percentiles is affected among the studied cases than controls
There was statistically significant difference between group I, group II and group III regarding SBP, DBP. As it was higher among (group I and group II) than group III.
There was statistically significant decrease in hemoglobin level among group I and group II than group III.
There was no statistically significant difference between group I, group II and group III regarding Platelets.
There is a significant decrease in total leucocytic count (TLC) of patients when compared to controls.
There was significant increase in serum creatinine, blood urea and BUN in the studied patients when compared to the control group
There is a significant decrease in patients GFR when compared to control group (p value=0.000).
There were statistically significant increase in potassium among group I, group II than group III. (p value=0.000).
There was a highly significant decrease in calcium level in patients as compared to controls.
There were statistically significant increase in phosphorus among group I, group II than group III
There was statistically significant difference between group I, group II and group III regarding PH and HCO3.Metabolic acidosis was present among group I, group II.
There was statistically significant difference between group I, group II and group III regarding Von Willebrand factor cleaving protease enzyme. Mean value of Von Willebrand factor cleaving protease enzyme was lower among group I than group II.Mean value of Von Willebrand factor cleaving protease enzyme was lower among group I than group III. Mean value of Von Willebrand factor cleaving protease enzyme was lower among group II than group III.
Mean value of Von Willebrand factor cleaving protease enzyme was lower among cases had thrombosis among group I than cases hadn’t thrombosis among group I .
There were statistically significant negative correlation between Von Willebrand factor cleaving protease enzyme and age.
There were statistically significant negative correlation between Von Willebrand factor cleaving protease enzyme and duration of renal failure.
There were statistically significant positive correlation between Von Willebrand factor cleaving protease enzyme and DBP.
There were statistically significant negative correlation between Von Willebrand factor cleaving protease enzyme and (Urea Creatinine, BUN).
There were statistically significant positive correlation between Von Willebrand factor cleaving protease enzyme and calcium.
There were statistically significant negative correlation between Von Willebrand factor cleaving protease enzyme and RBS.