1.
The role of N-acetylcysteine in the treatment of thrombotic thrombocytopenic purpura.
Rottenstreich, A, Hochberg-Klein, S, Rund, D, Kalish, Y
Journal of thrombosis and thrombolysis. 2016;(4):678-83
Abstract
Thrombotic thrombocytopenic purpura (TTP) is an acute, thrombotic microangiopathy with a high mortality rate if left untreated. Plasma exchange (PEX) is the current standard of care. However, a significant number of patients are refractory to this treatment. N-acetylcysteine (NAC) was recently suggested as a potential therapeutic adjunct for patients with TTP. This study reports a series of three patients with TTP successfully treated with NAC in addition to standard therapy. Detailed chart reviews on these patients were conducted. We discuss clinical features, laboratory findings and management of three patients who presented with microangiopathic hemolytic anemia and thrombocytopenia. Anti-ADAMTS13 antibodies and low levels of ADAMTS13 were detected and confirmed the diagnosis of acquired TTP. Based upon their severe presentation or lack of response to initial treatment with PEX, corticosteroids and other immunosuppressive agents, NAC was added. Under this combined treatment, all three patients hada significant clinical improvement of symptoms with concurrent normalization of platelet count and ADAMTS13 activity level. This report highlights the potential therapeutic utility of NAC in the treatment of TTP. Randomized controlled studies will be required to better characterize the risk-to-benefit ratio of NAC in the treatment of TTP.
2.
[Reversal of acute liver failure with N-acetylcysteine and prednisone in a patient with DRESS syndrome: a case report and literature review].
Pérez-Reyes, E, Casanova-Lara, A, Pérez-Torres, E, Córdova, J
Revista de gastroenterologia de Mexico. 2014;(3):208-10
3.
Successful experimental treatment of congenital ichthyosis in an infant.
Deffenbacher, B
BMJ case reports. 2013
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Abstract
Ichthyosis is a rare genetic disease that causes defects in skin keratinisation. Infants born with this disease have tight shiny skin that inhibits limb and ear mobilities, eyelid and lip deformities and poor hair and nail growths. In addition, the barrier properties of the skin are disrupted, which leads to dehydration, body temperature regulation difficulties and increased susceptibility to infection. The treatments currently available include topical keratolytics, emollients, and for severe disease systemic retinoids. Given the increased permeability of the skin and increased body surface area infants are particularly susceptible to accidental overdose from the topical keratolytic treatments currently available. An experimental emollient of 10% N-acetylcysteine (NAC) and 5% urea was recently used with success in Argentina. A newborn with congenital ichthyosis cared for in our clinic failed his initial treatment of topical emollients. He was subsequently treated successfully with off-label use of a topical 5% NAC and 5% urea emollient.
4.
Early presentation following overdose of modified-release paracetamol (Panadol Osteo) with biphasic and prolonged paracetamol absorption.
Graudins, A, Pham, HN, Salonikas, C, Naidoo, D, Chan, B
The New Zealand medical journal. 2009;(1300):64-71
Abstract
BACKGROUND Panadol Osteo (GlaxoSmithKline) is a modified-release paracetamol formulation marketed in Australia and New Zealand, comprising 33% immediate and 66% sustained-release fractions. In overdose, absorption may be delayed and the paracetamol treatment nomogram can miss potentially toxic paracetamol concentrations if only one serum estimate is taken. We report a massive ingestion of Panadol Osteo with biphasic, prolonged absorption requiring extended treatment with N-acetylcysteine. CASE REPORT A 72-year-old female presented 2 hours after ingesting 119x665 mg (1 g/kg) of Panadol Osteo and 5x30 mg mirtazepine. The patient was drowsy (GCS14). Activated charcoal was not administered. Her pulse was 70 bpm, BP 149/63 mmHg with an unremarkable physical examination. Two-hour paracetamol concentration was 2628 micromol/L falling to 2216 micromol/L, 4 hours post-ingestion. Admission acid-base status and liver function were normal. N-acetylcysteine was commenced using the standard 21-hour intravenous protocol and continued for 5 days (total dose 700 mg/kg) until paracetamol concentrations were undetectable. Serum paracetamol peaked a second time, 12 hours post-ingestion, at 3040 micromol/L and paracetamol absorption continued for 35 hours post-ingestion. Despite early administration of N-acetylcysteine, serum AST/ALT peaked at 384 and 541 IU/L on day 3 with normal coagulation profile. CONCLUSIONS Massive ingestion of modified-release paracetamol (Panadol Osteo) may result in biphasic and prolonged paracetamol absorption requiring extended administration of N-acetylcysteine. Current intravenous dosing regimens may not provide enough N-acetylcysteine to effectively metabolise paracetamol to non-toxic adducts.
5.
A case of haemodialysis-associated pseudoporphyria successfully treated with oral N-acetylcysteine.
Cooke, NS, McKenna, K
Clinical and experimental dermatology. 2007;(1):64-6
Abstract
We report a 33-year-old woman with haemodialysis-associated pseudoporphyria successfully responding to treatment with oral N-acetylcysteine. We briefly review the current literature on bullous skin disorders in end-stage renal disease, and compare and contrast the pathogenesis of pseudoporphyria and porphyria cutanea tarda in this context. We also discuss the antioxidant properties and clinical applications of N-acetylcysteine, including the treatment of haemodialysis-associated pseudoporphyria.
6.
The critically ill liver patient: fulminant hepatic failure.
McGuire, BM
Seminars in gastrointestinal disease. 2003;(1):39-42
Abstract
Fulminant hepatic failure is a challenging medical condition that requires intensive care management to prevent-major complications (cerebral edema, infections, and multi-system organ failure) and assistance from a liver transplant team when it is believed that liver regeneration is unlikely. Unfortunately, there are no specific medical therapies or devices to correct all of the functions of a liver. N-acetylcysteine is used for the treatment of acetaminophen overdose, but for most other causes of fulminant hepatic failure therapy is supportive care. This case illustrates many of the problems that are encountered during medical management of fulminant hepatic failure.
7.
Phytonadione therapy in a multiple-drug overdose involving warfarin.
Bates, D, Mintz, M
Pharmacotherapy. 2000;(10):1208-15
Abstract
We cared for a patient who ingested an unknown amount of acetaminophen with zopiclone and warfarin. The only liver function test that was abnormal was an increased international normalized ratio (INR), which remained elevated despite treatment with subcutaneous phytonadione and a prolonged infusion of N-acetylcysteine. An interaction between acetaminophen and warfarin may have decreased the hepatic metabolism of warfarin. The patient received numerous antibiotics that may have contributed to the increased INR. The prolonged elevation of INR also may have been due to infrequent administration of phytonadione.