Early detection of factor VIII inhibitors in hemophilia a patients with different treatment modalities

Hemophilia care in underdeveloped and developing countries is extremely inadequate and in some instances, completely absent. Persons with hemophilia battle the complications of both the disease and its treatment. One of the most serious of these problems is the development of the inhibitors. The development of the inhibitors influences their quality of life. This work was designed to: 1- elucidate the inhibitors status 2-to study the relation of factor VIII inhibitors and modes of therapy and duration of treatment 3- to clarify the cause of bleeding, is it related to insufficient treatment or development of inhibitors 4- to study the effect of the severity of hemophilia in the frequency of factor VIII inhibitors and their correlation with factor VIII level. The study included 63 hemophilic patients under different treatment modalities and were classified to four groups according to the types of treatment; they were group [I] included [7] patients with no previous anti hemophilic treatment and were considered as a control group, group [II] included [12] patients who were on treatment with fresh frozen plasma, group [III] consists of [16] patients who were under treatment with cryoprecipitate and group [IV] was [28] patients who were under treatment with concentrated factor VIII.To all groups of patients complete blood count, prothrombin time and prothrombin concentration, activated partial thromboplastin time, factor IX, von willebrand factor, factor VIII levels and factor VIII inhibitor levels were performed. Development of inhibitors was obvious in severe hemophilic patients especially in those who were receiving on demand factor VIII concentrate therapy

Evaluation of cardiovascular autonomic functions compared with clinical response in patients with malignant lymphomas before and after chemotherapy

54 patients presented with malignant lymphoma [20 cases with Hodgkin's disease, 11 with low grade non-HodgKin's lymphoma and 23 with high grade non-Hodgkin's lymphoma] were studied. All had advanced disease [stage II B, III and IV], requiring combination chemotherapy which included the use of vinca alkaloids. Clinical assessment and standard cardiovascular autonomic function tests were carried out prior to and following completion of chemotherapy treatment and 8 weeks after whenever possible. At presentation forty patients 40/54 [74%] had abnormal cardiovascular autonomic function tests and there was no correlation with the presence or absence of mediastinal disease. Significant improvement in autonomic scores at the end of treatment were recorded in 26/40 [65%] patients despite the use of drugs with known neurocardiac toxicity, but others 14/40 [35%] showed residual abnormalities of cardiovascular autonomic functions despite clinical resolution of the disease. After 8 weeks from completion of chemotherapy treatment autonomic scores showed further improvement in four patients [complete remission was achieved]. It was suggested that subclinical autonomic dysfunction is common in patients with malignant lymphoma and probably it may represent a paraneoplastic syndrome rather than toxic effects of the drugs used, but its pathogenesis and prevalence of which deserve further study. This phenomenon may predispose the patients with lymphoma to develop other autonomic dysfunction in gastrointestinal and genitourinary tract and should be considered during the evaluation of neurotoxicity of chemotherapy regimens used

value of qualitative cytochemistry of blood neutrophils in acute myeloid leukemia

Qualitative cytochemistry of blood neutrophils was studied at the diagnosis in thirty-one patients and was followed up in fifteen patients. At diagnosis, neutrophil myeloperoxidase [POX], chloroacetate esterase [CAE] and alkaline phosphatase [NAP] were significantly reduced in patients with AML compared with levels in ten normal controls. In remission period, the enzyme activities increased markedly for the fifteen patients who were followed up and when five cases with bone marrow aplasia were excluded, the other ten cases [with partial and complete remission] showed statistically higher values than the pretreatment period. The blood picture parameters were greatly affected by treatment as white blood cells and blast cells decreased markedly to normal values, while platelets increased markedly. It was concluded that peripheral hemogram and neutrophil enzyme activities can predict the state of remission [partial, complete or bone marrow aplasia] instead of doing the troublesome bone marrow aspirates

Early assessment of coronary revascularization by dobutamine stress echocardiography

In order to assess the use of dobutamine stress echocardiography [DSE]in the evaluation of patients early after coronary revascularization, the test was performed within one day before and from one-three days after angioplasty in 52 patients [30 with single vessel disease [SVD] and 22 with multivessel disease [MVD]]and in 25 patients one-three days before and ten-fourteen days after coronary artery bypass grafting [CABG]. Before angioplasty, 42 patients [77% SVD and 86% MVD] developed new wall motion abnormalities [WMA] compared with 11 [10% SDV and 36% MVD] after angioplasty. Before angioplasty, mean wall motion score index [WMSI] increased significantly from baseline to peak dobutamine. After angioplasty, there was insignificant increase in WMSI. The frequency of chest pain decreased after angioplasty, before angioplasty twenty-three patients developed chest pain compared with nine after angioplasty. Before angioplasty, thirteen patients developed ST changes and seven had ST changes after angioplasty. Before CABG, twenty-three patients developed new WMA compared with six after CABG. Before CABG, mean WMSI increased from baseline to peak dobutamine. After CABG, there was insignificant increase in WMSI. The frequency of chest pain decreased after CABG, before CABG thirteen patients developed chest pain compared with four after CABG. Before CABG, thirteen patients developed ST changes and three had ST changes after CABG. The results indicated that DSE is a feasible and safe method that accurately demonstrated an early improvement in stress-induced regional WMA

Renal artery stenosis as a cause of secondary treatable hypertension: Colour and duplex doppler evaluation, diagnostic criteria and efficacy

Renal artery stenosis is the cause of hypertension in a small percentage of all patients with hypertension, but is the only anatomic cause that can be treated by surgery or angioplasty. Recent studies indicated that Doppler characteristics aided with color Doppler imaging is an adequate screening method for such cases. This study aimed to evaluate the use and feasibility of color and duplex Doppler scanning and its criteria for detection and evaluation of renal artery stenosis as a cause of h hypertension. The renal artery was studied by this method all over its whole course in 32 patients, selected clinically with a hypertension suggested to be of renovascular origin. Another ten normal cases were studied as a representative of normal findings used for comparison. Transfemoral aortography [aortic flush angiography] was used for confirmation of the ultrasonography [US] findings and assessment of the degree of renal artery stenosis. The findings defined the use of three parameters concomitantly to give an accurate identification of the renal artery stenosis. Also, it showed that these parameters together [peak velocity, pulsatility index and resistivity index] had a high sensitivity, specificity, positive predictive value, negative predictive value and an overall accuracy of 100%, 86.96%, 84.21%, 95.2% and 92.3%, respectively, for renal artery disease detection. It was concluded that duplex aided with color Doppler US study of renal arteries of hypertensive patients can reliably detect renal artery stenosis, that can be therefore a valuable tool in selection of hypertensive patients for angiography and for either angioplasty or surgical management

Diagnosis of early subclinical central nervous system involvement with leukemia and lymphoma

Several recent studies have identified the increased incidence of central nervous system [CNS] involvement in patients with acute leukemia and malignant lymphoma [Miller et al., 1994]. Even though, increased occurrence of primary CNS disease, in Non-Hodgkin's lymphoma has been reported [OSullivan et al., 1991]. Different diagnostic procedures, have been used for early detection of CNS involvement but uptill now there is an urgent need for easy and clinically applicable methods. In the present study we have assessed the diagnostic value of the determination of cerebrospinal fluid [CSF] carcinoembryonic antigen [CEA], Beta[2] macroglobulin [Beta[2]-MG], beta-glucuronidase[Beta-Glu], lysozyme [Lys], lecithin cholesterol acyl transferase [LCAT] and lactate dehydrogenase [LDH] in detecting early CNS infiltration in patients with acute leukemia and malignant lymphoma and comparing their results wilt. Computerized axial tomography [CAT] for the brain in some of these patients. The study was cross-sectional at Assiut university hospital conducted on 25 cases with acute leukemia and 20 patients with malignant lymphoma. The results of CSF markers were compared with the corresponding values in 20 subjects who underwent lumbar puncture during minor surgical operations for intrathecal anesthesia. The present study revealed significant increment of all the markers studied in sera and CSF of patients with and acute leukemia in comparison with control group Patients with evidences of CNS involvement showed significant elevations of CEA [P<0.01] beta-Glu [P<0.01], Beta[2] MG [P<0.001], lysozyme [P<0.01], LCAT [P<0.01] and LDH [P<0.001] compared with patients without evidences of CNS involvement. Meanwhile, the sensitivity of CSF CEA, Beta-Glu, Beta-G,-MG, lysozyme, LCAT and LDH in diagnosis of patients with CNS involvement was 88 percent, 71 percent, 88 percent, 71 percent, 67 percent and 75% respectively. However, among patients without CNS involvement, 43 percent, 33 percent, 38 percent, 33 percent, 29 percent and 33 percent had elevated CSF levels of the previous markers respectively. These patients could have CNS involvement in the preclinical stage. Based on the present data and stability of the tumor markers studied, markers combination of choice in diagnosing CNS metastasis in patients with acute leukemia and lymphoma may be formed of CEA and Beta Glu. These markers are more easy to be done, of low cost and more applicable than CAT

Evaluation of CA125, thymidine kinase and haptoglobin as tumor markers in malignant lymphoma

This study included thirty-six patients with malignant lymphoma comprised of nineteen with non-Hodgkin's, eighteen with Hodgkin's lymphoma as well as nine patients with benign lymphadenopathy together with ten completely healthy subjects who were age matched to patients. The levels of the three markers were increased significantly in malignant lymphomas compared with healthy controls. The increase was presented in both HL and NHL. The levels of CA-125 and TK were significantly higher in malignant lymphoma compared with benign cause of lymphadenopathy. The sensitivity of CA-125, TK and haptoglobin for differentiating malignant from benign conditions was 81%, 94% and 89%, respectively. The specificity was 89%, 44% and 44%, respectively. The levels of CA- 125 reflected the tumor burden to a greater degree than TK and haptoglobin as clarified by higher levels with advancement of stage of disease, development of extranodal manifestations, hepatic or splenic involvement and greater size of lymph nodes

Skin manifestations associated with insulin dependent diabetes mellitus: HLA pattern

Forty insulin dependent diabetic patients with skin manifestations were among the diabetic patients attending the Diabetic Out-Patient Clinic, Assiut University Hospitals in 1991. These skin manifestations included allergic skin changes [70%]; fungal, candidal and pyogenic infections [50%]; vascular [45%]; neurologic [30%] and metabolic [17.5%] skin changes. Diabetic patients with skin manifestations showed an increased frequency of HLA-A10, B37, CW2 and DR2 compared to normal controls. There was no significant differences in the HLA pattern between diabetic patients with or without skin manifestations except for DR4 which was absent in the former group and was present in [82%] in the latter group and thus could be considered as a protective antigen against the occurrence of skin manifestation. The group of diabetic patients with infections showed an increased frequency of HLA-DR2 [R.R. 6.7], while patients with vascular skin changes showed a decreased frequency of Al [R.R. 0.29]. Patients with neurological skin changes showed an increased frequency of HLA-Al and CW4 [R.R. 5.4 and 4.6 respectively]. These results are discussed and prospects of further studies are referred to

Maternal and neonatal blood glycosylated hemoglobin with maternal diabetes

The study included 35 newborns of diabetic mothers. They were subjected to full clinical examination including maternal history of abortion and the mode of delivery, signs of hypoglycemia, respiratory distress, jaundice and birth weight. The blood sugar in newborns, glycosylated hemoglobin [HbA[1c]] were done to all cases. Also maternal blood sugar and HbA[1c]] were done. A significantly higher maternal blood sugar and HbA[1c] than controls were found indicating the poor control of diabetes in the last two months of pregnancy. Also there was positive correlation between maternal and infant HbA[1c]. This may reflect the state of metabolic changes in diabetes. Significantly higher newborn HbA[1c] in cases with signs of hypoglycemia was observed in our I.D.M. A significantly lower newborn blood sugar was found in cases with history of repeated abortion, cases with hypoglycemic signs and cases with history of R.D. were found