ABSTRACT
Diarrhoeal diseases are one of the leading worldwide preventable causes of death among children under 5 years of age. Almost half of children do not receive optimal acute gastroenteritis (AGE) treatment in Jordan. With neither regional nor local guidelines available for AGE, consensus recommendations on the management of paediatric AGE in Jordan were developed by a panel of senior paediatricians and paediatric gastroenterologists and are endorsed by the Jordanian Paediatric Society. Recommendations are based on international guidelines and available relevant literature in relation to the AGE landscape and the healthcare system in Jordan. The prevention of diarrhoeal diseases should focus on the improvement of nutrition, hygiene, and sanitation, the introduction of routine vaccination against rotavirus, and the adoption of a standardised approach for AGE management (oral rehydration solution (ORS) use±adjunct therapies, continued feeding, and avoiding routine antibiotic use). Ondansetron, diosmectite, racecadotril, probiotics, and zinc can be considered adjunct to ORS, if needed. Local data gaps should be addressed. The clinical algorithm for the management of paediatric AGE could promote adherence to practice recommendations and by extension improve health outcomes in children.
ABSTRACT
BACKGROUND: Familial mediterranean fever (FMF) is a recessively inherited disease characterized by recurrent crises of fever, abdominal, articular and/or thoracic pain. The most severe complication is the development of renal amyloidosis. Over 35 mutations have been discovered so far in the gene responsible for the disease, MEFV. This article aims at determining a correlation between the MEFV genotype and the occurrence of amyloidosis in FMF patients, in addition to the study of the modifying effects of the SAA1 (type 1 serum amyloid A protein) and MICA (Major Histocompatibility Complex (MHC) class-I-chain-related gene A) genes on this severe complication. METHODS: Fourteen MEFV mutations were screened and the SAA1 and MICA polymorphisms tested in 30 FMF patients with amyloidosis and 40 FMF patients without amyloidosis. RESULTS: The M694V and V726A allelic frequencies were, respectively, significantly higher and lower in the group with amyloidosis, compared to the control FMF group. The beta and gamma SAA1 alleles were more frequently encountered in the group without amyloidosis, whereas the alpha allele was significantly more observed in FMF patients with amyloidosis (p < 0.025). All the MICA alleles were encountered in both patients' groups, but none of them was significantly associated with amyloidosis. CONCLUSIONS: The results suggest a protective effect of the SAA1 beta and gamma alleles on the development of amyloidosis and show the absence of a MICA modifying effect on amyloidosis development. Testing these polymorphisms on a larger sample will lead to more definite conclusions.
