Peritoneal dialysis resulting in discontinuance of recurring attacks of acute intermittent porphyria: A case report.

Acute intermittent porphyria is one of eight disorders arising from disturbances in heme biosynthesis where the precursors, 5-aminolevulinate and porphobilinogen, are elevated in plasma and urine. Attacks are characterized by severe abdominal pain, vomiting and/or obstipation, neurological manifestations, and psychological disturbances. The mainstay of treatment is hemin infusion to induce the negative feedback of heme synthesis. Hemodialysis is casuistically suggested as an alternative treatment. We present a case report of a 78-year-old male with acute intermittent porphyria and renal failure treated with peritoneal dialysis resulting in complete discontinuance of longstanding painful and disabling porphyria attacks.

Reference intervals for plasma cystatin C and plasma creatinine in adults using methods traceable to international calibrators and reference methods.

INTRODUCTION: The aim of this study was to establish reference intervals for plasma cystatin C and creatinine in adults using the Gentians cystatin C method traceable to the international calibrator standard ERM-DA471/IFCC and a creatinine method traceable to the IDMS (Isotope Dilution Mass Spectrometry) creatinine reference method. METHODS: Blood samples were collected from 304 healthy blood donors (152 men and 152 women between 17 and 66 years old) with 30-31 men and 30-31 women in each ten-year interval. Plasma cystatin C was analyzed using the Gentian Cystatin C assay on a Roche cobas c702 analyzer, and plasma creatinine was analyzed using the CREA Plus assay on the Roche Modular P analyzer. RESULTS: The nonparametric reference intervals for plasma cystatin C were 0.58-1.00 mg/L in women (median 0.78 mg/L, range 0.56-1.06 mg/L) and 0.62-1.04 mg/L in men (median 0.79 mg/L, range 0.61-1.07 mg/L). The Mann-Whitney U test revealed no gender-related difference in plasma cystatin C (P = .21). A common reference interval in women and men was calculated to be 0.61-1.01 mg/L (median 0.79 mg/L, range 0.56-1.07 mg/L). The nonparametric reference interval for plasma creatinine was 52-89 µmol/L in women (median 69 µmol/L, range 52-92 µmol/L) and 61-108 µmol/L in men (median 86 µmol/L, range 56-118 µmol/L). The Mann-Whitney U test revealed a gender-related difference in plasma creatinine (P < .0001). CONCLUSION: In conclusion, we have established reference intervals for plasma cystatin C and creatinine in adults using methods traceable to international standards.

Failure of remote ischemic preconditioning to reduce the risk of postoperative acute kidney injury in children undergoing operation for complex congenital heart disease: a randomized single-center study.

OBJECTIVE: The objective of this study was to evaluate whether remote ischemic preconditioning can protect kidney function in children undergoing operation for complex congenital heart disease. METHODS: Children (n = 113) aged 0 to 15 years admitted for complex congenital heart disease were randomly allocated according to age to remote ischemic preconditioning and control groups. After exclusion of 8 patients, we conducted the analysis on 105 patients (remote ischemic preconditioning group, n = 54; control group, n = 51). Before surgery, remote ischemic preconditioning was performed as 4 cycles of 5 minutes of ischemia by inflating a cuff around a leg to 40 mm Hg above the systolic pressure. End points were development of acute kidney injury, initiation of dialysis, plasma creatinine, estimated glomerular filtration rate, plasma cystatin C, plasma and urinary neutrophil gelatinase-associated lipocalin, and urinary output. Secondary end points included postoperative blood pressure, inotropic score, and mortality, as well as morbidity reflected by reoperation and stays in the intensive care unit and hospital. RESULTS: Overall, 57 of the children (54%) had acute kidney injury develop, with 27 (50%) in the remote ischemic preconditioning group and 30 (59%) in the control group (P > .2). Remote ischemic preconditioning was not associated with improvement in either any of the renal biomarkers or any of the secondary end points. CONCLUSIONS: We found no evidence that remote ischemic preconditioning provided protection of kidney function in children undergoing operation for complex congenital heart disease.