Relapse of rare diseases during COVID-19 pandemic: bicytopenia in an adult patient with thiamine-responsive megaloblastic anaemia.

Thiamine-responsive megaloblastic anaemia (TRMA) is a syndrome associated with megaloblastic anaemia, diabetes mellitus and sensorineural deafness, due to mutations in the SLC19A2gene, which codes for a thiamine carrier protein. Oral thiamine supplementation is the main treatment. We report the case of a 19-year-old man known for TRMA, who presented in the emergency department with bicytopenia (haemoglobin 5,4 g/dL, thrombocytes 38×109/L) revealed by dyspnea and chest pain. Investigations excluded bleeding, hemolysis, coagulopathy and iron deficiencies. A recent infection and an acute coronary syndrome have also been eliminated. We later found out that thiamine treatment had been discontinued three months before, due to general confinement in Tunisia during the COVID-19 pandemic. Parenteral administration of 100 mg of thiamine daily resulted in the recovery of haematopoiesis within three weeks.

Relapse of rare diseases during COVID-19 pandemic: bicytopenia in an adult patient with thiamine-responsive megaloblastic anaemia (TRMA)

Thiamine-responsive megaloblastic anaemia (TRMA) is a syndrome associated with megaloblastic anaemia, diabetes mellitus and sensorineural deafness, due to mutations in the SLC19A2 gene, which codes for a thiamine carrier protein. Oral thiamine supplementation is the main treatment. We report the case of a 19-year-old man known for TRMA, who presented in the emergency department with bicytopenia (haemoglobin 5,4 g/dL, thrombocytes 38×109/L) revealed by dyspnea and chest pain. Investigations excluded bleeding, hemolysis, coagulopathy and iron deficiencies. A recent infection and an acute coronary syndrome have also been eliminated. We later found out that thiamine treatment had been discontinued three months before, due to general confinement in Tunisia during the COVID-19 pandemic. Parenteral administration of 100 mg of thiamine daily resulted in the recovery of haematopoiesis within three weeks

Drug-adverse related events in emergency department : Epidemiological,clinical profile and prognosis.

INTRODUCTION: Adverse-drug events (ADe) are frequent in emergency medicine and remain misdiagnosed depending on the clinical polymorphism and the underlying comorbidities. Older patients with multiple comorbidities and polypharmacy are more frequently affected and makes poor prognosis. AIM OF THE STUDY: The aim of this study was to evaluate the epidemiology of ADe in the emergency department (ED) visits and to identify the prognostic value mortality within 30 days. METHODS: Prospective, monocentric study. Patients were included if they met criteria of aADe. We evaluated severity and mortality at 30 days. Moreover, misuse and preventabilty were studied. RESULTS: We included 159 cases within 113,272 ED visits. Mean age = 64 ± 19 years, sex ratio =0.6.The average number of drugs was 4.5 ± 3. Polymedication was found in 54%. In 10 cases, the prescription contained twice the same molecule. A double ADe was found in 11 patients. We identified 55 cases of misuse, 94% of them were due to physician. An interaction was found in 23 patients. Improper prescription with age, renal function or presence of contra-indications was found in 46 patients. In 41% cases, ADR events were preventable. An ADR event was considered severe in 44% of cases and 30-Days mortality's rate was 9.4%. Drugs n (%): Anticoagulants 53(34,6); Antihypertensive 29(19); Antiarrhythmic 15(10). Multivariate analysis of mortality at day 30: Misuse and polyapthology were independent predictors; Respective Odds ratios: (OR: 2.6; 95% CI [1.25-5.38]; p=0,001) and (OR 2.31; 95% CI [1,16 - 4,61]; p=0,017). CONCLUSION: Drug-related ED visits are common in elderly. ADe is severe in 44% cases and preventable in 41%. Mortality rate was 9,4%. Misuse, polypharmacy and comorbidities were independent predictors of severity and mortality.

[Nonresponse to hepatits B vaccine in health care workers]. / La non reponse a la vaccination antihepatitique chez le personnel soignant.

The authors report the results of an investigation of witness cases realised in collaboration between Occupational Medecine Service and Immunology Laboratory of Hedi CHAKER University Hospital SFAX during the year 2000. The purpose was to search the genetic control of the HLA class I system for the non-response to hepatitis B vaccine and to evaluate the contribution of other favorite factors as tabac, sex, age. Thus, in a population of 32 healthy agents found nonresponders to hepatitis B vaccine by the titers of anti HBs antibody, we have performed the HLA-A, -B phenotypes by the technique of complement dependent microcytotoxicity. The frequency of studied HLA class I antigens, was compared for the non-responders group, to the frequency observed in witnesses group, done with 52 responders healthy agents. The rate of nonresponsiveness hepatitis B vaccine was evaluated up to 5 %. Statistically significant difference was observed for HLA A1 and-B44 markers showing fraquencies which were considerably higher in the non-responders than in witnesses.