Mohamed Khattab

Pediatric rhabdomyosarcoma in Morocco.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the first two decades of life. There is, however, a paucity of reports on the pattern of its occurrence in Africa. This study analyses the epidemiological pattern, clinical features, histology, and outcome in Moroccan children presenting with RMS.

Improving the prognosis of pediatric hodgkin lymphoma in developing countries: A moroccan society of pediatric hematology and oncology study

The event‐free survival (EFS) of children with Hodgkin lymphoma (HL) exceeds 80% in high income countries (HIC), but little is known about this rate in developing countries.

Ovarian tumours in children : A review of 18 cases

BACKGROUND To review the experience of Childrens Hospital of Rabat in managing ovarian tumours in children. MATERIALS AND METHODS There were 18 patients between 2 and 15 years of age who presented with an ovarian tumour at Childrens Hospital of Rabat between January 2000 and December 2008. Data collected from the hospital medical records included age at diagnosis, patients history, presenting complaints, radiological examination, tumour markers, management, operative procedure, histopathological examination and outcome of the patients. RESULTS The most common presenting complaint was abdominal pain in 10 (55%) patient. 77% of ovarian tumours were germ cell tumours; 71% of these were teratomas which were benign in 66% of cases. Unilateral salpingo-oophorectomy was the most common surgical procedure performed in 15 patients (83%) through laparotomy. Laparoscopic ovarian cystectomy was carried out in 2 (11%) patients with benign cystic teratoma. Of the 7 (39%) patients with malignant tumours, three received postoperative chemotherapy. Outcome was good in most cases. There were no cases of resistance to treatment, or death. CONCLUSION Early diagnosis of ovarian tumours in children and adolescents is important. Since most of these tumours are benign, surgical treatment should be conservative to minimise the risk of subsequent infertility, while the treatment of malignant tumours should include complete staging, resection of the tumour, postoperative chemotherapy when indicated, to give the patient a chance for future childbearing.

Von Willebrand's disease: case report and review of literature

Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels < 50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD usually do not concern type 1 patients with the mildest VWF defects (VWF levels between 30 and 50IU/dL). Von willebrand factor is a complex multimeric protein with two functions: it forms a bridge between the platelets and areas of vascular damage and it binds to and stabilizes factor VIII, which is necessary for the clotting cascade. By taking a clinical history of bleeding (mucocutaneous bleeding symptoms suggestive of a primary haemostatic disorder, a quantitative or qualitative abnormality of VWF is possible) it is important to think about VWD and to make the appropriate diagnosis. If the VWD is suspected diagnostic tests should include an activated partial thromboplastin time, bleeding time, factor VIII: C Ristocetin cofactor and vWF antigen. Additional testing of ristocetin induced plattlet adhesion (RIPA) the multimeric structure and collagen binding test and genanalysis allow diagnosing the different types of von. Willebrand Disease. The treatment of choice in mild forms is the synthetic agent desmopressin. In patients with severe type 1, type 2B, 2N and type 3 or in people who do not response to desmopressin, the appropriate treatment is a factor VIII concentrate that is rich of VWF. We report a case of infant in 27-month-old boy who had been referred due to haemorrhagic shock. His birth histories, his families social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications. Prior to the symptoms, the child was on a normal diet and was thriving appropriately. The child presented one days before his admission trauma to the inner face of the lower lip that caused an external acute bleeding loss. The laboratory data showed unfortunately, the most severe form of Von Willebrands Disease; Type 3. The management was based on erythrocyte and fresh-frozen plasma (FFP) transfusions with administration of factor VII with good evolution.

A hemophagocytic syndrome revealing a Griscelli syndrome type 2

Griscelli syndrome type 2 is a rare autosomal recessive disorder, due to a mutation in RAB27A gene. It associates partial albinism, silver hair and immune deficiency. We report the case of a 6 year-old boy who was admitted to the Emergency department with severe sepsis complicated by hemophagocytic syndrome. Many clinical and biological criteria leads to the diagnosis of type 2 Griscelli syndrome: consanguineous family, recurrent infection, absence of psychomotor retardation, oculocutaneous albinism, silver hair, occurrence of hemophagocytic syndrome and especially the pathognomonic appearance on microscopic examination of the hair. The absence of giant organelles inclusion in all granulated cells eliminated Chediak-Higashi syndrome.

Advanced Orofacial Rhabdomyosarcoma: A Retrospective Study of 31 Cases

Introduction  Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma encountered in childhood and adolescence. Early diagnosis of pediatric cases is critical to improving outcomes, especially when socioeconomic status and geographical access to specialist services can reduce opportunities for early cancer detection and treatment. Objective  The objective of this study is to determine factors that can delay referral and treatment in specialist pediatric oncology center upon our population specificities. Methods  This retrospective study involved 31 children between 2003 and 2013. Children affected by histologically confirmed RMS occurring as a primary lesion in the orofacial area were included. Results  The median age was 8 ± 4.22 years (range: 3 months – 15 years). The male to female ratio was 1.8:1. Most of the patients had advanced stage disease at presentation (81.7% group had 3–4 pretreatment staging) with parameningeal involvement in 80.6% of the cases. The 2-year event-free survival rate was 17.7 ± 7.8% for all the patients. Delay of admission to our unit and abandonment of treatment seem to be important factors for the dismal prognosis. Conclusion  Patients location, socioeconomic status and health care coverage have had an impact on longer delays in seeking care and on follow-up. More studies are needed for implementation of a better management practices and a better supportive care upon specificities of our population.