Cardiac events and cardiac T2[*] in Egyptian children and young adults with beta-thalassemia major taking deferoxamine

Cardiac events and death are not uncommon in adults with beta-thalassemia [beta-TM] taking deferoxamine [DFO] monotherapy because of poor compliance and possibly the less effectiveness of DFO in controlling cardiac iron overload. We sought to assess compliance with DFO, the percentage of shift to other iron chelators, and the occurrence of cardiac siderosis, and cardiac events and death in beta-TM patients on DFO monotherapy. Prospective, observational, 10-year follow-up of patients attending Ain Shams Thalassemia Unit, Cairo, Egypt. For all beta-TM patients aged 2-1 8 years attending the unit during January 1998 and taking DFO, we recorded all cardiac events [whether fatal or not] during January 2008. All patients still on DFO monotherapy and with a normal EKG and not showing symptoms or signs suggestive of heart failure [HF] were evaluated for cardiac siderosis byT2[*]. Of 412 patients, only 126 [31%] were still taking DFO monotherapy [only 43% of those were compliant], 136 were taking combined DFO and deferiprone [DFP], 72 were taking DFP and 32 were taking deferasirox [DFX]. Twenty-one were lost follow-up and 25 died [10 cardiac]. Eight of ten cardiac deaths and 12 of 15 non-cardiac deaths were in the DFO monotherapy group. Those taking DFO monotherapy with no HF and left ventricular ejection fraction [LVEF] by T2[*] >56% had a median age of 19 years and 56% were males; cardiac T2[*] was <20 ms in 30 [22%]; 10-20 ms in 20 [14.7%] and <10 ms in 10 [7.3%]. LVEF ranged from 58%-76% [median 64%]. Forty percent of T2* patients <10 ms were compliant with DFO. Fifty-eight percent of patients on DFO monotherapy were noncompliant, but even compliance did not prevent severe cardiac siderosis and most cardiac events [whether fatal or not] that occurred in the DFO monotherapy group

Cytogenetic study of some childhood haematological disorders in East Delta of Egypt

Our understand of the pattern of chromosomal changes with the affected cells of patients with various hematological disorders has expanded enormously in the past years. Therefore, the work was carried out at Pediatric and Histology Departments in Zagazig University Hospital to evaluate the particular patterns of chromosomal changes in some blood diseases of children. Sixty patients with some blood diseases, aged 5-15 years old of both sexes and 15 children as control were included. All patients were subjected to full clinical and laboratory investigations in addition to full family history. Blood samples were collected from all cases for chromosomal study preparation. Phytohaemoagglutinin were added to all culture tubes to stimulate cell division. After 72 hours mitosis was stopped at metaphase stage by adding colchicine, the metaphase spreads were stained by Geimsa technique. Karyotyping was performed to all examined cases. The metaphase spreads of the patients with leukemia revealed abnormal chromosomal pattern in 73.3% of 15 patients. The main numerical aberrations were polyploidy, while the main structural aberrations were duplications or deletion of one arm of chromosomes 4, 5 and 15. Increased breaks and translocation were noted in many leukaemic cases. While the patients suffering from idiopathic thrombocytopenic purpura showed abnormal chromosomal pattern of 46.7% of the cases. The main detected aberrations were duplication of the long arm of chromosome 5 and deletion of long arm of chromosome 8. 30% of 15 cases of aplastic anemia showed chromosomal abnormalities. The recorded aberrations were duplication of long arm of chromosome 6 and short arm of X chromosome. Lastly, the main recorded chromosomal aberrations in cases of favism were satellite formation of acrocentric chromosomes of group D and G in two cases out of 15 cases with glucose 6 phosphate dehydrogenase deficiceny. In conclusion, chromosomal abnormalities are common in childhood leukemia than in aplastic anemia and idiopathic thrombocytopenia

Chromosomal abnormalities of childhood acute lymphoblastic leukemia and its relation to response to induction therapy

Thirty children with acute lymphoblastic leukemia [ALL] of both sexes [18 males and 12 females] with ages ranged from 2 to 15 years old and 10 healthy children matched with age and sex were included in this work to evaluate chromosomal abnormalities as well as to evaluate its relationship to the response to induction therapy. All cases were subjected to full history, complete examination and full laboratory investigations. Blood samples were collected from all cases for chromosomal study. Induction therapy was started with vincristine, prednisone, adriamycin and intrathecal methotrexate for four-six weeks. Assessment of the patients for induction of remission was carried through complete clinical examination and full laboratory investigations. The results showed that 22 cases of ALL revealed chromosomal abnormalities. Most of these chromosomal abnormalities were numerical aberrations in the form of polyploidy 14/11. The main structural aberrations were duplications, deletions and translocations. The response to induction therapy showed complete response for 22 cases [8 with normal chromosomal pattern and l4 with polyploidy]. Failure to achieve complete response was observed in six cases were with structural aberrations. Two cases died during therapy

Some of acute phase reactants in malnourished infants

The usefulness of clinical features of infection as well as some of laboratory screening tests in the diagnosis of infection in malnourlished infants were evaluated in this study in Zagazig University Hospital in 1989. Fourty infants with PEM were included in this study. 25 cases were associated clinically with manifestations of infection and 15 cases without any manifestations. Chest was the commonest site of infection and followed by GIT. Fever was the commonest clinical presentation of infection in PEM. ESR, C-Reactive protein, total white blood cell count, absolute band cell count and Band/Segmented ratio were the laboratory tests, used for derection of infection in PEM. There was no significant difference between both groups as regard of C-reactive protein, absolute band count and band/segmented ratio