The Impact of Moderate-Dose Acetylsalicylic Acid in the Reduction of Inflammatory Cytokine and Prevention of Complication in Acute Phase of Kawasaki Disease: The Benefit of Moderate-Dose Acetylsalicylic Acid

Background: Acetylsalicylic acid (ASA) is part of the recommended treatment of Kawasaki disease (KD). Controversies remain regarding the optimal dose of ASA. We aimed to evaluate the impact of different doses of ASA on inflammation control while minimizing adverse effects in the acute phase treatment of KD. Methods: The enrolled 323 patients with KD were divided into three groups according to ASA dose: moderate-dose (30–50 mg/kg/day), high-dose (80–100 mg/kg/day), and non-ASA. Results: High-dose ASA group showed a significantly shorter duration of fever from the start of treatment to remission than other groups. Baseline level and delta score of interleukin (IL)-1, IL-6, IL-10, tumor necrosis factor-α, and transforming growth factor β were not statistically different among the groups. The number of patients who received additional treatments in the non-ASA group was more than other groups. Coronary artery dilatation was not significantly different among the groups. One patient with high-dose ASA was diagnosed with Reye syndrome. Conclusion: Different doses of ASA did not show any differences in changes of inflammatory bio-makers and cytokines. However, high-dose ASA showed occurrence of Reye syndrome, and non-ASA showed intravenous immunoglobulin refractoriness. We suggest that moderate-dose ASA may be beneficial for the treatment of patients in the acute phase of KD.

Aspirin rechallenge in an adult patient previously diagnosed with Reye syndrome

Purpose Aspirin has been the cornerstone of antiplatelet therapy in patients with acute coronary syndromes and is well accepted and recommended by several major healthcare organizations. A combination of aspirin and a P2Y12 inhibitor, commonly known as dual antiplatelet therapy, is recommended in patients with coronary stent implantation to reduce the risk of stent thrombosis and ischemic events. Summary We recently cared for an adult male who presented with an acute coronary syndrome who had a history of Reye syndrome during childhood. During this admission, he was rechallenged with low-dose aspirin for the first time since his diagnosis of Reye syndrome as a child after aspirin therapy. There have been various case reports in children and adults who have been rechallenged with aspirin within days to weeks after the initial diagnosis of Reye syndrome. These reports show mixed results in children and adults regarding the return of Reye syndrome upon aspirin rechallenge shortly after initial aspirin exposure. Conclusion This, to our knowledge, appears to be the first report of a low-dose aspirin rechallenge 30 years later in life in an adult patient with a history of Reye syndrome while receiving aspirin therapy during childhood.

Non Salicylate-Associated Reye Syndrome: A Case Report

Reye's syndrome (RS) is a rare disease, usually associated with consumption of salicylates during viral illness. In 1965, the first case of association between RS and salicylates was described in United Kingdom (UK). The incidence of RS decreased dramatically after warnings of UK and US health agencies against using aspirin in children. Patients with RS presented with neurologic compromise, cerebral edema, acute hepatitis, and liver failure-especially in the children. In this paper, a four-month-old boy with diagnosis of RS was described, who presented with malaise, cyanosis and decreased level of consciousness, but the history of salicylates consumption was negative for him and his mother. © 2019 Tehran University of Medical Sciences. All rights reserved. Acta Med Iran 2019;57(5):332-334.

United States Pharmacopeia Safety Review of Willow Bark

Willow bark (Salix spp.) is an ingredient in some dietary supplements. No serious adverse effects were reported from trials of willow bark extracts delivering 120 – 240 mg salicin (the purported active constituent) daily for up to 8 weeks. All studies involved adults only; none involved special subpopulations such as pregnant or breastfeeding women, or children. The most common adverse effects associated with willow bark are gastrointestinal; a few allergic reactions were also reported. Some publications advise caution when taking willow bark. There is a risk of increased bleeding in vulnerable individuals, salicylates cross the placenta and are eliminated slowly in newborns, some persons are sensitive or allergic to aspirin, and children are at risk of Reye syndrome. Concurrent use with other salicylate-containing medicines increases these risks. Metabolism of 240 mg salicin from willow bark could yield 113 mg of salicylic acid, yet dietary supplement products are not required to be labeled with warnings. In contrast, over-the-counter low-dose aspirin (81 mg strength), which delivers 62 mg salicylic acid, is required by law to include cautions, warnings, and contraindications related to its use in pregnant and nursing women, children, and other vulnerable subpopulations, e.g., those using anticoagulants. In the interest of protecting public health, the United States Pharmacopeia has included a cautionary labeling statement in the United States Pharmacopeia Salix Species monograph as follows: “Dosage forms prepared with this article should bear the following statement: ‘Not for use in children, women who are pregnant or nursing, or by persons with known sensitivity to aspirin.’”

First Case Report of Primary Carnitine Deficiency Manifested as Intellectual Disability and Autism Spectrum Disorder

Systemic primary carnitine deficiency (PCD) is a genetic disorder caused by decreased or absent organic cation transporter type 2 (OCTN2) carnitine transporter activity, resulting in low serum carnitine levels and decreased carnitine accumulation inside cells. In early life, PCD is usually diagnosed as a metabolic decompensation, presenting as hypoketotic hypoglycemia, Reye syndrome, or sudden infant death; in childhood, PCD presents with skeletal or cardiac myopathy. However, the clinical presentation of PCD characterized by autism spectrum disorder (ASD) with intellectual disability (ID) has seldom been reported in the literature. In this report, we describe the clinical features of a seven-year-old girl diagnosed with PCD who presented atypical features of the disease, including a developmental delay involving language skills, concentration, and attention span, as well as autistic features and brain alterations apparent in magnetic resonance imaging. We aim to highlight the difficulties related to the diagnostic and therapeutic approaches used to diagnose such patients. The case reported here presented typical signs of PCD, including frequent episodes of hypoglycemia, generalized muscle weakness, decreased muscle mass, and physical growth deficits. A molecular genetic study confirmed the definitive diagnosis of the disease (c.1345T>G (p.Y449D)) in gene SLC22A5, located in exon 8. PCD can be accompanied by less common clinical signs, which may delay its diagnosis because the resulting global clinical picture can closely resemble other metabolic disorders. In this case, the patient was prescribed a carnitine-enriched diet, as well as oral carnitine at a dose of 100 mg/kg/day. PCD has a better prognosis if it is diagnosed and treated early; however, a high level of clinical suspicion is required for its timely and accurate diagnosis.