pituitrin and Anemia--Sickle-Cell

Topics: Anemia, Sickle Cell; Antisickling Agents; Azepines; Cell Membrane Permeability; Diuretics; Erythrocyte Membrane; Erythrocytes, Abnormal; Fluid Therapy; Hemoglobins; Humans; Ion Channels; Oxidation-Reduction; Vasopressins; Vitamin E; Zinc

Topics: Adolescent; Adult; Age Factors; Anemia, Sickle Cell; Child; Child, Preschool; Female; Hemoglobinometry; Hemoglobins; Humans; Kidney; Kidney Concentrating Ability; Male; Renal Tubular Transport, Inborn Errors; Sodium; Urea; Vasopressins

Topics: Adolescent; Adult; Amino Acids; Anemia, Sickle Cell; Blood Cell Count; Chromatography, Gas; Clinical Trials as Topic; Cyanates; Diuresis; Feeding and Eating Disorders; Female; Hemoglobins; Humans; Hydantoins; Injections, Intravenous; Male; Middle Aged; Reticulocytes; Thyrotropin; Time Factors; Valine; Vasopressins

Frequencies of single nucleotide polymorphisms (SNPs) from pain related candidate genes are available for individuals with sickle cell disease (SCD). One of those genes, the arginine vasopressin receptor 1A gene (AVPR1A) and one of its SNPs, rs10877969, has been associated with pain and disability in other pain populations. In patients with SCD, clinical factors such as pain and stress have been associated with increased health care utilization, but it is not known if the presence of the AVPR1A SNP plays a role in this observation. The study purpose was to explore the relationships between rs10877969 and self-reported pain, stress, and acute care utilization events among individuals with SCD.. In a cross-sectional investigation of outpatients with SCD, participants completed PAINReportIt®, a computerized pain measure, to describe their pain experience and contributed blood or saliva samples for genetic analysis. We extracted emergency department and acute care utilization from medical records.. The SNP genotype frequencies (%) for this sample were CC 30 (28%), CT 44 (41%), TT 33 (31%). Acute care utilization and stress as an aggravator of pain were significantly associated with the rs10877969 genotype (p = .02 and p = .002, respectively). The CT genotype had the highest mean utilization and CC genotype was associated with not citing stress as a pain aggravator. Chronic pain was not associated with rs10877969 (p = .41).. This study shows that rs10877969 is related to indicators of stress and acute pain. Further research is recommended with other measures of stress and acute pain.

Topics: Adult; Aged; Anemia, Sickle Cell; Female; Genetic Predisposition to Disease; Humans; Male; Middle Aged; Pain; Polymorphism, Single Nucleotide; Stress, Psychological; Vasopressins; Young Adult

Sickle cell disease (SCD)-induced urinary concentration defect has been proposed as caused by impaired ability of the occluded vasa recta due to red blood cell sickling to serve as countercurrent exchangers and renal tubules to absorb water and solutes. However, the exact molecular mechanisms remain largely unknown. The present studies were undertaken to determine the effects of SCD on vasopressin, aquaporin2 (AQP2), urea transporter A1 (UTA1), Na-K-Cl co-transporter 2 (NKCC2), epithelial Na channels (ENaC), aquaporin1 (AQP1), nuclear factor of activated T cells 5 (NFAT5) and Src homology region-2 domain-containing phosphatase-1 (SHP-1), an important regulator of NFAT5, in the Berkeley SCD mouse kidney medulla. Under water repletion, SCD only induced a minor urinary concentration defect associated with increased urinary vasopressin level alone with the well-known effects of vasopressin: protein abundance of AQP2, UTA1 and ENaC-β and apical targeting of AQP2 as compared with non-SCD. SCD did not significantly affect AQP1 protein level. Water restriction had no further significant effect on SCD urinary vasopressin. NFAT5 is also critical to urinary concentration. Instead, water restriction-activated NFAT5 associated with inhibition of SHP-1 in the SCD mice. Yet, water restriction only elevated urinary osmolality by 28% in these mice as opposed to 104% in non-SCD mice despite similar degree increases of protein abundance of AQP2, NKCC2 and AQP2-S256-P. Water-restriction had no significant effect on protein abundance of ENaC or AQP1 in either strain. In conclusion, under water repletion SCD, only induces a minor defect in urinary concentration because of compensation from the up-regulated vasopressin system. However, under water restriction, SCD mice struggle to concentrate urine despite activating NFAT5. SCD-induced urinary concentration defect appears to be resulted from the poor blood flow in vasa recta rather than the renal tubules' ability to absorb water and solutes.

Topics: Anemia, Sickle Cell; Animals; Aquaporin 1; Aquaporin 2; Epithelial Sodium Channels; Female; Kidney; Male; Membrane Transport Proteins; Mice; Protein Tyrosine Phosphatase, Non-Receptor Type 6; Solute Carrier Family 12, Member 1; Transcription Factors; Up-Regulation; Urea Transporters; Vasopressins; Water-Electrolyte Balance

A five-year-old girl with known sickle cell disease presented with severe hyponatremia and findings compatible with syndrome of inappropriate secretion of antidiuretic hormone (SIADH). She was found to have lead levels in the Class III category. By exclusion, we postulated that the SIADH was in some way related to the high lead levels, since this was the only abnormality the patient exhibited. The toxic lead levels and the elevated vasopressin levels rapidly responded to dimercaprol and calcium EDTA chelation therapy.

Topics: Anemia, Sickle Cell; Chelation Therapy; Child, Preschool; Dimercaprol; Edetic Acid; Female; Humans; Hyponatremia; Inappropriate ADH Syndrome; Lead; Lead Poisoning; Vasopressins

Topics: Anemia, Sickle Cell; Hematuria; Humans; Vasopressins

Topics: Anemia, Sickle Cell; Blood Urea Nitrogen; Diuresis; Hemoglobin, Sickle; Humans; Kidney; Urea; Vasopressins

Topics: Anemia, Sickle Cell; Chronic Disease; Diabetes Insipidus; Diet; Humans; Hydrogen-Ion Concentration; Hypernatremia; Hyponatremia; Kidney; Kidney Concentrating Ability; Kidney Diseases; Osmolar Concentration; Vasopressins; Water-Electrolyte Imbalance

Topics: Anemia, Sickle Cell; Child; Female; Humans; Hyponatremia; Syndrome; Vasopressins; Water-Electrolyte Imbalance

Topics: Adenine Nucleotides; Anemia, Sickle Cell; Animals; Cyclic AMP; Humans; Kidney; Kidney Concentrating Ability; Potassium Deficiency; Rats; Vasopressins

Topics: Adolescent; Adult; Age Factors; Aged; Anemia, Sickle Cell; Child; Child, Preschool; Creatinine; Female; Fetal Hemoglobin; Glomerular Filtration Rate; Hemoglobin C; Hemoglobin C Disease; Hemoglobinometry; Hemoglobinopathies; Homozygote; Humans; Kidney; Kidney Concentrating Ability; Male; Middle Aged; Osmolar Concentration; Reticulocytes; Vasopressins; Water-Electrolyte Balance

Topics: Adult; Anemia, Sickle Cell; Bicarbonates; Blood Cell Count; Creatinine; Cystoscopy; Diuresis; Erythrocytes; Ethacrynic Acid; Furosemide; Glucose; Hematocrit; Hematuria; Hemoglobins, Abnormal; Hemoglobinuria; Hemolysis; Humans; In Vitro Techniques; Injections, Intravenous; Male; Mannitol; Osmolar Concentration; Oxygen; Oxygen Inhalation Therapy; Proteinuria; Sodium; Tromethamine; Vasopressins; Water

Topics: Adolescent; Anemia; Anemia, Sickle Cell; Black People; Body Weight; Child; Dehydration; Humans; Urine; Vasopressins; Water-Electrolyte Balance

Topics: Anemia; Anemia, Sickle Cell; Child; Erythrocytes, Abnormal; Humans; Kidney; Potassium; Vasopressins