Childhood acute lymphoblastic leukemia in the Middle East and neighboring countries: A prospective multi-institutional international collaborative study (CALLME1) by the Middle East Childhood Cancer Alliance (MECCA)

Background: Little is known about childhood ALL in the Middle East. This study was undertaken by MECCA as initial efforts in collaborative data collection to provide clinical and demographic information on children with ALL in the Middle East. Procedure: Clinical and laboratory data for patients with ALL between January 2008 and April 2012 were prospectively collected from institutions in 14 Middle East countries and entered into a custom-built-database during induction phase. All laboratory studies including cytogenetics were done at local institutions. Results: The 1,171 voluntarily enrolled patients had a mean age of 6.1±3.9 years and 59.2 were boys. T-ALL represented 14.8 and 84.2 had B-precursor ALL. At diagnosis, 5.6 had CNS disease. The distribution of common genetic abnormalities reflected a similar percentage of hyperdiploidy (25.6), but a lower percentage of ETV6-RUNX1 translocation (14.7) compared to large series reported from Western populations. By clinical criteria, 47.1 were low/standard risk, 16.9 were intermediate risk, and 36 were high risk. Most patients received all their care at the same unit (96.9). Patients had excellent induction response to chemotherapy with an overall complete remission rate of 96. Induction toxicities were acceptable. Conclusions: This first collaborative study has established a process for prospective data collection and future multinational collaborative research in the Middle East. Despite the limitations of an incomplete population-based study, it provides the first comprehensive baseline data on clinical characteristics, laboratory evaluation, induction outcome, and toxicity. Further work is planned to uncover possible biologic differences of ALL in the region and to improve diagnosis and management. Pediatr Blood Cancer 2014; 61:1403-1410. © 2014 Wiley Periodicals, Inc

Testicular tumours in prepubertal children: About eight cases

Background: To analyze the spectrum of testicular tumors in prepubertal children and the therapeutic resultants in an unselected population. Materials and Methods: Our hospital database was analyzed for testicular tumors from January 1995 to December 2010 concerning clinical presentation, treatment and therapeutic results. Results: Eight patients were operated on because of testicular tumors.In six cases (75%) the tumor was benign: benign teratoma (four cases), epidermoid cyst (one case) and immature teratoma (one case). Two  patients (25%) had a malignant tumour: yolk-sac tumour (two cases). All this children underwent surgery. Radical inguinal orchidectomy was  performed in six cases and conservative surgery was performed in two cases. One patient has received adjuvant chemotherapy. Followup was uneventfully three years after primary surgery.Conclusion: In prepubertal children, most testicular tumours are benign. If tumour markers were negative testis-preserving surgery can be proposed,  complete excision of the tumour should be ascertained. In the case of testicular teratoma, the possibility of contralateral tumour should be    considered in the follow-up.Key words: Children, Testis, Tumour, Teratoma,yolk-sac tumou

[Listeriosis in Tunis: seven cases reports].

International audienceListeria monocytogenesis a Gram positive facultative intracellular bacterium that can be responsible for severe infections, affecting essentially pregnant women, immunocompromised patients at the early and later stages of life. In Tunisia, invasive L. monocytogenes infections are thought to be exceptional and limited data are available about listeriosis. We reported seven cases (five newborn children and two infants) of human listeriosis that occurred in Tunis from 2000 to 2008. The newborn children were hospitalized for suspicion of maternofoetal infections. The two infants were hospitalized for fever associated with digestive signs in one case and neurological signs in the other. L. monocytogenes-was isolated from culture of cerebrospinal fluid in four cases, peripheral samples in two cases and from blood culture in one case. Isolates identification was based on conventional methods. Antimicrobial susceptibility was realized according to the recommendation of the "Comité de l'antibiogramme de la Société française de microbiologie". All L. monocytogenes isolates were sensitive to amoxicillin and aminoside but resistant to 3rd generation cephalosporins. Investigations of the immune system were realized for the two infants including phenotypic analysis of peripheral blood cells by flow cytometry, lymphocyte proliferation assays, phagocytic cell functions and measurement of immunoglobulins as well as complement. All these explorations were normal for both infants. The outcome was fatal in only one case (a newborn child), and all the other patients recovered after adapted antibiotic treatment. In conclusion, our study shows that listeriosis is not exceptional in Tunis. Thus, it is necessary to know how to evoke this diagnosis, at any age, in order to establish an early and adapted antibiotic treatment and to avoid fatal outcome

Identification of patients with abetalipoproteinemia and homozygous familial hypobetalipoproteinemia in Tunisia

BACKGROUND: Abetalipoproteinemia (ABL) and Homozygous Familial Hypobetalipoproteinemia (Ho-FHBL) are rare monogenic diseases characterised by very low plasma levels of cholesterol and triglyceride and the absence or a great reduction of apolipoprotein B (apoB)-containing lipoproteins. ABL results from mutations in the MTP gene; Ho-FHBL may be due to mutations in the APOB gene. METHODS: We sequenced MTP and APOB genes in three Tunisian children, born from consanguineous marriage, with very low levels of plasma apoB-containing lipoproteins associated with severe intestinal fat malabsorption. RESULTS: Two of them were found to be homozygous for two novel mutations in intron 5 (c.619-3T>G) and in exon 8 (c.923 G>A) of the MTP gene, respectively. The c.619-3T>G substitution caused the formation of an abnormal mRNA devoid of exon 6, predicted to encode a truncated MTP of 233 amino acids. The c.923 G>A is a nonsense mutation resulting in a truncated MTP protein (p.W308X). The third patient was homozygous for a novel nucleotide deletion (c.2172delT) in exon 15 of APOB gene resulting in the formation of a truncated apoB of 706 amino acids (apoB-15.56). CONCLUSIONS: These mutations are expected to abolish the apoB lipidation and the assembly of apoB-containing lipoproteins in both liver and intestine