Relation between clinical severity of haemophilia and the factor inhibitor

Alloantibodies occuring in haemophilia as a side effect of repeated treatment represents a severe complication. The antibody binds to a specific epitope within the factor molecule in a time dependent manner. If the alloantibody is present in excess, factor coagulant activity will be inactivated. Our study was conducted on 34 haemophilic patient and 10 control healthy children. All cases and control were subjected to the following: full history taking; complete clinical examination, laboratory investigation of Hb%, RBCs, WBCs, platelets counts, bleeding, coagulation, prothrombin and partial thromboplastin time. And assay of levels of FVIII and FIX, FVIII inhibitor level and FIX inhibitor.71% of haemophilia A were mild and 29% were moderate, 90% of haemophilia B were mild and 10% moderate. An inhibitor was detected in 16 cases [66.6%] out of 24 cases of hemophilia A, and 9 cases [90%] out of 10 cases of haemophilia B. These inhibitors were detected 2-11 years after the diagnosis. There was non significant correlation between inhibitor level in heamophiliaA to age, Hb% and FVIII% but there was negative correlation V.H.S [R< 0.001] to residual FVIII%. Also there was no correlation between inhibitor level in haemophilia B to age, Hb% and FVIII% but there was negative correlation V.H.S [R< 0.001] to residual FIX%. So we can conclude that development of inhibitor to FVIII and FIX although predominantly develop in severe heamophiliacs, they do occasionally appear in mild and moderate disease. Many of them are of low-level and perhaps transient. So we recommend to screen these patients at intervals for the inhibitor. The development of the inhibitor depends on the immunogenecity of the replacement therapy and also on a genetic predisposition

Screening tools detection of children at risk for lead exposure

Elevated blood lead levels are known to cause a wide range of health problems in infants and children. Recent studies suggest that blood lead levels previously considered safe may have deleterious effects on several neurobehavioural parameters. American center for disease control [CDC] [1991] considered that lead level at 10 micro g/dl or above is toxic. The CDC put guidline-quationair to outline risk groups, so that the selective screening for these groups only will be cost-effective than mass screening. To test CDC quationair sensitivity and its possible application in our community, 2000 children from schools and nurseries from Abu Hammad Sharkia governorate, aged 2-14 years old, 400 of them fulfilled the criteria of CDC quationair. [living in or visiting in a job with lead exposure, living near active lead smelter, traffics or battery recycling, the child or one of his family treated before from lead poisoning]. All children were subjected to full history, through clinical examination for signs of lead poisoning and blood lead determinations by atomic absorption spectrophotometry with Delves sampling techniques. The mean blood lead level for all children was 31.16 micro g/dl. 48.5% of children had blood lead level between 25-40 micro g/dI. There was highly significant increase of the mean blood level in the high risk groups, outlined by CDC control. The result showed the sensitivity of CDC quationair and its possible applications in our community to out-line the risk groups to be screened

study on red cell enzymes in acute lymphoblastic leukaemia in attack and remission

This study comprised 40 children and included two groups; the first group consisted of 20 healthy children as control, their ages ranged from 2-12 years. The second group comprised 20 cases, their ages ranged from 18 month to 11 years. This group was classified into 11 cases in the acute attack; from them 7 cases were diagnosed as ALL of L2 category according to FAB classification and 4 of the L1 category. 9 cases came in remission, all were diagnosed as LL before