Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In hereditary cystic disorders, renal injury begins with the formation of the first cyst. Renal injury may manifest as large kidneys, abdominal pain, hypertension and hematuria in children and young adults with autosomal dominant polycystic kidney disease (ADPKD). In autosomal recessive PKD (ARPKD) and ADPKD, cysts form primarily in collecting ducts and expand progressively. Collecting duct cysts that block urine flow have the potential to block urine formation in large numbers of upstream nephrons. In an ARPKD rat congenitally lacking vasopressin, only a few cysts developed until exogenous arginine vasopressin (AVP) was administered. AVP elevates cyclic AMP in vulnerable tubule cells to stimulate mitogenesis and fluid secretion, thereby causing cysts to form and enlarge indefinitely. The administration of an AVP-V2 receptor inhibitor or the consumption of sufficient water to persistently lower plasma AVP levels will ameliorate disease progression. Renal volume measurements provide the most reliable way to forecast long-term outcome in individual children and adult patients with ADPKD. Many drugs that have demonstrated efficacy in small clinical trials, preclinical trials and cell-based studies are in the treatment pipeline. Counseling, regular exercise, limitation of dietary calories, salt, protein and fat, increased fluid intake throughout the day and treatment of hypertension are components of a rational treatment program that can be offered at an early age to those with, or at risk for developing PKD.
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PMID:Rationale for early treatment of polycystic kidney disease. 2502 29

BACKGROUND The aim of this study was to describe the clinical characteristics of Chinese ADPKD inpatients and to identify the factors associated with disease severity. MATERIAL AND METHODS We included 167 hospitalized patients (inpatients) with ADPKD in this study. Multiple regression analyses were conducted to determine factors correlated with estimated glomerular filtration rate (eGFR). Patients were stratified into subgroups according to the presence of symptoms, in which clinical parameters were analyzed and compared. RESULTS The mean age of hospitalized ADPKD patients was 48.7 years old, lumbar and/or abdominal pain was seen in 40.12% of patients, following by nephrolithiasis (38.92%), hematuria (30.54%), and urinary tract infection (24.55%). Serum thrombocyte level and hemoglobin exhibited significant positive correlations with eGFR. Symptomatic patients accounted for 71.26% of the studied population. Patients with hypertension had increased risk of presence of symptoms (OR=2.794, 95%CI=1.341-5.822). Low thrombocyte and hemoglobin levels were observed in patients with hematuria. CONCLUSIONS Thrombocyte level was positively correlated with eGFR but was not associated with presence of PKD-related symptoms, suggesting thrombocyte level might be an independent serum biomarker for disease progression. Hypertension was associated with increased risk of symptom occurrence, indicating the relationship between hypertension and disease progression. This study reveals the clinical characteristics of inpatients with ADPKD in China and provides clinicians with useful insights into this intractable disease.
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PMID:Clinical Features of 167 Inpatients with Autosomal Dominant Polycystic Kidney Disease at a Single Center in China. 3021 20