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Eosinophilia Myalgia Syndrome [keywords]
- Successful extracorporeal liver dialysis for the treatment of trimethoprim-sulfamethoxazole-induced fulminant hepatic failure. [JOURNAL ARTICLE]
- Singapore Med J 2013 Apr 17.
Trimethoprim-sulfamethoxazole (TMP-SMZ) is a commonly used antibiotic that has been associated with drug rash with eosinophilia and systemic symptoms (DRESS) syndrome. DRESS syndrome is characterised by fever, rash, lymphadenopathy, eosinophilia and one or more major organ involvement. Although rare, TMP-SMZ is a recognised cause of fulminant hepatic failure. We report a case of a 17-year-old Chinese, male adolescent who presented with fever, myalgia, generalised maculopapular rash and lymphadenopathy after taking TMPSMZ for acne vulgaris. He subsequently developed hepatic encephalopathy and was worked up for urgent liver transplantation. He responded well to extracorporeal liver dialysis (originally intended as a bridging therapy) and subsequently recovered without the need for liver transplantation. This case report highlights the importance of early recognition of TMPSMZ-induced DRESS syndrome and the need for early discontinuation of the drug in the affected patient. Extracorporeal liver dialysis and transplantation should be considered in the management of TMP-SMZ-induced fulminant hepatic failure.
- Severe eosinophilic syndrome associated with the use of probiotic supplements: a new entity? [Journal Article]
- Case Report Rheumatol 2012.:934324.
Severe eosinophilic syndromes related to the administration or use of unsuspected immunogenic substances have been described previously. Many of these diseases presented initially as clusters or isolated cases. The spanish toxic oil syndrome, the eosinophilia myalgia syndrome, and nephrogenic systemic fibrosis are examples of such diseases. We describe 2 cases of a severe eosinophilic syndrome characterized by marked peripheral blood eosinophilia (>15,000 cells/ml), mononeuritis multiplex, and necrotizing vasculitis which developed in a close temporal association with the recent onset use of nonprescription probiotics. There was no history of a prior autoimmune disease. Although both cases had prompt response to immunosuppression with rapid resolution of peripheral blood eosinophilia and accompanying constitutional symptoms, they remained with permanent neurological deficits.
- Leukemoid reaction secondary to hypersensitivity syndrome to phenobarbital: a case report. [Journal Article]
- Int J Clin Exp Pathol 2013; 6(1):100-4.
The most important adverse effects of phenobarbital, an anticonvulsant drug, are behavior and cognitive alterations. Hypersensitivity syndrome caused by phenobarbital presenting with a leukemoid reaction is a rare side effect, which is rarely ever reported and needs to be known. We report on a 27-year-old Chinese woman who experienced hypersensitivity syndrome three weeks after the initiation of phenobarbital. The patient developed fever, skin rash, face swelling, lymphadenopathy, myalgia, hepatitis, eosinophilia, atypical lymphocytes and leukocytosis. Along with the pathological progress of the disease, the patient noticed a gradual exacerbation of her symptoms. And the highest leukocyte count was up to 127.2 x 10(9)/L. After discontinuing of phenobarbital and administration of methylprednisolone combined with the intravenous immunoglobulin shock therapy, all initial symptoms improved and the leukocyte count normalized. This case is reported because of its rarity of the leukemoid reaction secondary to hypersensitivity syndrome to phenobarbital.
- Effects and side effects associated with the non-nutritional use of tryptophan by humans. [Journal Article, Research Support, Non-U.S. Gov't, Review]
- J Nutr 2012 Dec; 142(12):2236S-2244S.
The daily nutritional requirement for L-tryptophan (Trp) is modest (5 mg/kg). However, many adults choose to consume much more, up to 4-5 g/d (60-70 mg/kg), typically to improve mood or sleep. Ingesting L-Trp raises brain tryptophan levels and stimulates its conversion to serotonin in neurons, which is thought to mediate its actions. Are there side effects from Trp supplementation? Some consider drowsiness a side effect, but not those who use it to improve sleep. Though the literature is thin, occasional side effects, seen mainly at higher doses (70-200 mg/kg), include tremor, nausea, and dizziness, and may occur when Trp is taken alone or with a drug that enhances serotonin function (e.g., antidepressants). In rare cases, the "serotonin syndrome" occurs, the result of too much serotonin stimulation when Trp is combined with serotonin drugs. Symptoms include delirium, myoclonus, hyperthermia, and coma. In 1989 a new syndrome appeared, dubbed eosinophilia myalgia syndrome (EMS), and was quickly linked to supplemental Trp use. Key symptoms included debilitating myalgia (muscle pain) and a high peripheral eosinophil count. The cause was shown not to be Trp but a contaminant in certain production batches. This is not surprising, because side effects long associated with Trp use were not those associated with the EMS. Over 5 decades, Trp has been taken as a supplement and as an adjunct to medications with occasional modest, short-lived side effects. Still, the database is small and largely anecdotal. A thorough, dose-related assessment of side effects remains to be conducted.
- Eosinophilic fasciitis (Shulman disease). [Journal Article, Review]
- Best Pract Res Clin Rheumatol 2012 Aug; 26(4):449-58.
The eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by symmetrical and painful swelling with a progressive induration and thickening of the skin and soft tissues. The diagnosis of EF is often based on the association of characteristic skin or subcutaneous abnormalities and a thickened fascia with an inflammatory infiltration, mostly composed of lymphocytes and eosinophils. A peripheral eosinophilia is frequently present, but is not mandatory for the EF diagnosis. The diagnosis might be helped by a muscle magnetic resonance imaging which typically may evidence an increased signal intensity within the fascia and marked fascia enhancement after gadolinium administration at the acute phase of the disease. Differential diagnoses should be ruled out, including eosinophilia-myalgia syndrome (EMS) after L-tryprophane ingestion, hypereosinophilic syndromes (HES), systemic sclerosis, Churg-Strauss syndrome, and/or peripheral T cell lymphomas with cutaneous involvement. Due to the scarcity of the EF disease, there is no consensual therapeutic strategy. However, oral corticosteroids remain the mainstay treatment and may be associated to an immunosuppressive drug such as methotrexate in patients with morphea-like lesions or an unsatisfactory response to corticosteroids alone.
- Environmental- and injury-related epidemic-assistance investigations, 1946-2005. [Historical Article, Journal Article]
- Am J Epidemiol 2011 Dec 1; 174(11 Suppl):S65-79.
This paper summarizes environmental investigations (n = 458) conducted during the first 60 years of the epidemic-assistance investigation program at the Centers for Disease Control and Prevention. These investigations were grouped into 10 categories: toxic chemicals (n = 102), indoor air quality and outdoor air toxics (n = 21), new or rare epidemic diseases and unexplained syndromes (n = 29), natural disasters (n = 81), terrorism and unintentional human-made disasters (n = 9), substance use and abuse (n = 13), environmental aspects of infectious disease (n = 132), those affecting neonates and infants (n = 11), violence and injuries (n = 51), and miscellaneous (n = 9). Among the most important or prominent were studies of lead and arsenic toxicity at smelters, mercury in paint and beauty creams, dioxin in waste oil in Missouri, polychlorinated biphenyls and multiple other toxic chemicals, global pesticide poisoning outbreaks, hepatic angiosarcoma among vinyl chloride workers, toxic oil syndrome in Spain, eosinophilia-myalgia syndrome from contaminated L-tryptophan, diethylene glycol poisoning in Haiti, aflatoxicosis in Kenya, Gulf War illness among veterans, impact and needs assessments during natural disasters (e.g., Hurricane Katrina (2005) and the Mount St. Helens volcano eruptions (1980)), risk factors for heat-related mortality, domestic and international terrorist attacks, Parkinsonism related to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine in California, and unintentional injury- and violence-related events.
- [Acute interstitial pneumonia: diagnostic approach and management]. [English Abstract, Journal Article, Review]
- Rev Mal Respir 2011 Jun; 28(6):809-22.
Acute interstitial pneumonia (AIP) encompasses a spectrum of pulmonary disorders characterized by involvement of the lung interstitium and distal airways (bronchioles and alveoli). The onset of respiratory symptoms is acute, most often within two weeks. Most AIP take place de novo, but sometimes represent an acute exacerbation of chronic lung disease. The clinical presentation of AIP comprises rapidly progressive dyspnoea, associated sometimes with cough, fever, myalgia and asthenia. Chest radiography shows diffuse pulmonary opacities. The associated hypoxemia may be severe enough to cause acute respiratory failure. Underlying aetiologies are numerous and variable, particularly in relation to the underlying immune status of the host. Various histopathological entities may be responsible for AIP although diffuse alveolar damage is the predominant pattern. The diagnostic approach to a patient presenting with AIP is to try to determine the most likely underlying histopathological pattern and to search for a precise aetiology. It relies mainly on a meticulous clinical evaluation and accurate biological investigation, essentially guided by the results of bronchoalveolar lavage performed in an area identified by abnormalities on high resolution computed tomography of the lungs. Initial therapeutic management includes symptomatic measures, broad-spectrum antibiotic treatment adapted to the clinical context, frequently combined with systemic corticosteroid therapy.
- Post-epidemic eosinophilia-myalgia syndrome associated with L-tryptophan. [Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't]
- Arthritis Rheum 2011 Nov; 63(11):3633-9.
Eosinophilia-myalgia syndrome (EMS) is characterized by subacute onset of myalgias and peripheral eosinophilia, followed by chronic neuropathy and skin induration. An epidemic of EMS in 1989 was linked to consumption of L-tryptophan that had originated from a single source. Following the ban by the Food and Drug Administration (FDA) on the sale of L-tryptophan, the incidence of EMS declined rapidly. Moreover, no new cases have been described since the FDA ban was lifted in 2005. We report the clinical, histopathologic, and immunogenetic features of a new case of L-tryptophan-associated EMS, along with evidence of activated transforming growth factor β and interleukin-4 signaling in the lesional skin.
- Churg-Strauss Syndrome following PTU Treatment. [Journal Article]
- Int J Rheumatol 2009.:504105.
Propylthiouracil (PTU) is a frequently prescribed drug in the treatment of hyperthyroidism. The use of PTU is, however, accompanied by numerous potentially serious side effects including vasculitis. PTU-related vasculitides can present as haematuria, pulmonary haemorrhage, or cutaneous lesion together with aspecific symptoms such as fever, myalgia, arthralgia, and fatigue. Cerebral involvement is seldom observed. We present a 49-year-old female with Graves' disease and asthma, who developed paresis of the proximal extremities, eosinophilia, pulmonary, and cutaneous lesions following treatment with PTU. A cerebral vasculitis consistent with Churg-Strauss syndrome (CSS) was suspected. Although cerebral involvement is seldom observed with PTU treatment, cerebral vasculitis should be considered in patients developing CNS symptoms.
- [Eosinophilia myalgia syndrome (EMS) caused by L-tryptophan product and toxic oil syndrome (TOS) caused by denatured rape-seed oil]. [Journal Article, Review]
- Shokuhin Eiseigaku Zasshi 2009 Dec; 50(6):279-91.