Hemodialysis-associated pseudoporphyria resistant to N-acetylcysteine

Driss El Kabbaj1, Aziza Laalou, Zoubida Alouane

  • 1Service of Nephrology, Haemodialysis and Kidney Transplantation, Military Hospital, Mohammed V, Rabat, Morocco. delkabbaj@yahoo.fr

We report a 33-year-old female patient who had hemodialysis-associated pseudoporphyria which did not respond to treatment with oral N-acetylcysteine. She responded favorably to treatment with the anti-malarial drug, chloroquine. The case is being reported to highlight the difficulty in interpreting the urinary porphyrin assays in patients on hemodialysis. Additionally, the current literature on pseudoporphyria disorders in patients with end-stage renal disease is briefly discussed.

Related Concept Videos

Hemodialysis II: Procedure and Complications01:24

Hemodialysis II: Procedure and Complications

DialyzersA hemodialysis (HD) dialyzer is a plastic cartridge containing thousands of parallel hollow fibers, which serve as semipermeable membranes. These fibers are typically made from cellulose-based or other synthetic materials. During HD, blood is pumped into the top of the cartridge and distributed among these fibers. Simultaneously, dialysis fluid, known as dialysate, is introduced into the bottom of the cartridge, bathing the outside of the fibers. Across the semipermeable membrane,...
Peritoneal Dialysis III: Nursing Management01:25

Peritoneal Dialysis III: Nursing Management

Peritoneal dialysis, or PD, utilizes the peritoneal membrane as a filter to eliminate excess fluid and waste products. Effective nursing management is essential for ensuring patient safety, preventing complications, and promoting optimal function of the peritoneal dialysis process.Assessment and MonitoringNurses must thoroughly assess the patient before, during, and after each dialysis session. Regular monitoring includes vital signs, daily weight, fluid intake and output, and laboratory values...
Peritoneal Dialysis II: Peritoneal Dialysis Systems and Complications01:25

Peritoneal Dialysis II: Peritoneal Dialysis Systems and Complications

Peritoneal dialysis (PD) is a medical process that removes waste products and excess fluid from the body using the peritoneal membrane as a natural filter.Peritoneal Dialysis MethodsSeveral methods can be used for peritoneal dialysis, including Acute Intermittent Peritoneal Dialysis, Continuous Ambulatory Peritoneal Dialysis, and Automated Peritoneal Dialysis, also known as Continuous Cyclic Peritoneal Dialysis.Acute Intermittent Peritoneal Dialysis (AIPD) is used for patients with uremic...
Pharmacogenetics of Phase II Enzymes: N-acetyltransferase, Thiopurine S-methyltransferase, UDP-glucuronosyltransferase01:27

Pharmacogenetics of Phase II Enzymes: N-acetyltransferase, Thiopurine S-methyltransferase, UDP-glucuronosyltransferase

Phase II biotransformation reactions are essential for detoxifying and eliminating xenobiotics, including many pharmaceutical compounds. These reactions typically involve conjugation, the covalent attachment of polar endogenous groups such as glucuronic acid, sulfate, methyl, or acetyl moieties to functional groups introduced during Phase I metabolism. The resulting conjugates are more water-soluble, enabling efficient renal or biliary excretion.The major classes of Phase II enzymes include...
Diabetic Nephropathy01:28

Diabetic Nephropathy

Definition Diabetic nephropathy is a chronic kidney complication that results from prolonged hyperglycemia.Prevalence It is the most common cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide, affecting up to half of individuals with diabetes.Pathophysiology • Sustained hyperglycemia triggers multiple hemodynamic and metabolic changes in the kidney. • Early in the disease, increased renal blood flow and glomerular hyperfiltration occur due to afferent arteriolar...
Chronic Kidney Disease III: Interprofessional Care01:28

Chronic Kidney Disease III: Interprofessional Care

Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...