study on transfusion-transmitted viral infections in thalassemic children

Beta- thalassemia syndromes are the most common causes of chronic haemolytic anemia in Egypt. The disease appears early in life as a Variable degree of anaemia associated with splenomegaly, stunted growth, bone changes and mongoloid facies. Patients are usually treated with regular blood transfusion which leads to iron overload and therefore chelation therapy is very important to avoid iron overload and its complications. The patients may have immunological abnormalities mostly due to iron overload, repeated exposure to allogenic antigens and immunosuppressive viruses in blood transfusions, desferrioxamine chelation therapy and splenectomy. Infection has been reported to be one of the main causes of morbidity and mortality in beta-thalassemia. It is described as the second most common cause of death in these patients with a prevalence of 12-13%. Besides the well-known risks of blood-borne infections associated with multiple transfusions, a less familiar clinical problem is the increased susceptibility of these patients to infections, due to the coexistent immune deficiency. One of these infections may be parvovirus B19. Parvovirus B19 is a single-stranded DNA virus. The virus is classified as a member of the erythrovirus genus because replication occurs only in human erythrocyte precursors. This work was designed to study some transfusion related viral infections in thalassemic children attending the hematology unit of Pediatrics department of Assiut University and to discuss the possible predisposing and underlying factors. The study was carried out in the period between September 2004 and October 2005 in the departments of clinical pathology and pediatrics, Assiut university hospital, Egypt It included 50 individuals, 35 transfusion-dependant children with beta-thalassemia major, aged 2 to 15 years and 15 apparently healthy children as a control group. Patients recruited in the study had thorough history taking and complete clinical examination. In addition, the following laboratory investigations were performed for all cases and controls: complete blood picture including reticulocytic count and calculation of the reticulocytic index; liver functions, [iron status [including serum iron, TIBC and ferritin]; human parvovirus B19 IgG; Hepatitis C virus antigen by PCR and antibodies [HCV-Abs] by ELISA, hepatitis B virus surface antigen [HBsAg], and human immunodeficiency virus types 1 and 2 antibodies by ELISA. Thalassemic patients had significantly lower Hb, RBCs and TIBC and significantly higher reticulocytic count, reticulocytic index, serum iron, serum ferritin, serum bilirubin, AST and ALT than the controls. The studied patients had 83% positivity for Parvovirus lgG antibodies, 97% for Hepatitis C IgG antibodies, 80% for Hepatitis C antigen by PCR. Patients had significantly lower CD4 T lympocytes, higher CD8 T lymphocytes than the controls. CD4/CD8 ratio was also inverted in the patients. Parvovirus positive cases had significantly lower Hb, RBCs, reticulocytic count and index and significantly higher AST and ALT than parvovirus negative cases. Serum ferritin, parvovirus IgG, and CD8 T lymphocytes correlated positively with the number of blood transfusions. Parvovirus lgG correlated positively with AST and ALT and negatively with reticulocytic count. Infectious complications constitute an important part of the clinical spectrum of beta-thalassemia, being associated with significant morbidity and mortality. The recently recognized immune defects in these patients involve multiple components of the immune system and have been attributed to specific features of the disease, as well as to the therapeutic modalities applied. Iron overload, a primary complication of both thalassemia itself and transfusion therapy, is thought to be the main precipitating mechanism, due to the important immunoregulatory properties of iron and its binding proteins. Iron excess may derange the immune balance in favor of the growth of infectious organisms. Other factors include multiple transfusions, associated with constant allo-antigenic stimulation, as well as with transmission of immunosuppressive viruses including the parvovirus B19. Infection with this virus in thalassemic patients can lead to persistent anemia indicated by reticulocytpenia and decreased reticulocytic index. Surveillance for infections in patients with beta-thalassemia is crucial, while additional studies are required to establish more clearly the clinical significance of the suspected precipitating mechanisms, hence providing new methods for the further amelioration of the survival rate and quality of life. Blood or blood products intended for use in high-risk groups such as immunocompromised individuals and patients with underlying hematological problems should be screened for B19. New inactivation methods for blood or blood products should be implemented to reduce the transmission of the parvovirus B19 via blood transfusion. Screening of blood donors for B19 can be an alternative to viral inactivation. Regular chelation therapy is a must to prevent the effects of the iron overload on the immune response. lntroduction of parvovirus B19 vaccines particularly for the immunocompromised patients may be helpful in the near future

Role of some inflammatory markers in recent ischemic stroke

The onset of cerebral ischemia triggers a cascade of pro-inflammatory molecular and cellular events. Clinical studies suggest that the strength of this acute response is important in early and late clinical outcomes, early clinical worsening, and extent of brain damage. The aim of this work was to estimate the. role of some inflammatory markers in recent ischemic stroke, and to correlate these inflammatory markers with the short term outcome. Twenty sex patients presented with recent history of hemiplegia within 24 hours were included. The patient group was planned to contain 13 patients with the age between 20 and <40years and 13 patients with the age between 40 and 60 years. 15 subjects, [age and sex matched to the patients] were included in the study as control Neurological deficits were rated by Scandinavian Stroke Scale. Clinical assessments and serum levels of the inflammatory markers, Neopterin C -Reactive Protein [CRP], Tumor Necrosis Factor-alpha [TNF-alpha], Complement 4 [C-4], Interleukin-8 [IL-8], and Neopterin, were done at the time of admission, day 3, and day 7 from the stroke onset. Serum levels of neopterin started to increase from the 3 rd day and remained high to the end of the first week from the onset of the stroke. The increase was more obvious in the young aged patients. Serum levels of IL-8, raised rapidly in the acute phase of the stroke and then gradually decreased through the first wee and but still higher than the base line of the total patients and control. Serum levels of TNF-alpha and CRP had rapid significant increase from the first day to reach maximum levels in the 3 rd day and still significantly high till the end of the first week Serum CRP levels were higher in the old age group while serum C4 levels decreased from the 1 st day then gradually increased to reach maximum levels in the 7 th day, but still significantly lower than the control group. Also, there were statistically positive correlations between serum levels of neopterin and C-4 in the 3 rd sample with the Scandinavian scale in the 3 rd assessment. Brain ischemia induced an inflammatory cascade by the increase in serum levels of neopterin, IL-8, TNF-alpha, CRP and decrease in C4. This inflammatory response continued through the first week by the increase in the levels of complement -4. The inflammatory response was more obvious in the young aged patients. There were positive correlations between serum levels of neopterin, and C-4 with the short-term outcome of the stroke patients

Celiac disease, TB enterocolitis and giardiasis among children with chronic diarrhea: the accuracy of ANT-EMA and tTG expression in the diagnosis of celiac disease

Chronic diarrhea is one of the most common causes of referral to a gastroenterology clinic. Chronic diarrhea may result from many different causes; celiac disease is one of them. Other important causes in our locality are infections such as TB and Giardiasis. This work was planned to: 1-Determine the frequency of celiac disease, TB enteritis and Giardiasis among children referred to the gastroenterology unit with the complaint of chronic diarrhea and to evaluate the different methods used in the diagnosis of each disease. 2- Verifying the diagnostic accuracy of immunohistochemical tTG expression versus serum anti-endomysial antibodies [EMA] in celiac disease [CD] diagnosis. The study included 92 patients with chronic diarrhea. Their ages ranged from 6 months to 15 years. They were 56 males and 36 females, admitted to the Pediatric Gastroenterology Unit, Assiut University Hospital during the period from January 2005 to December 2006. Besides full history and thorough clinical examination, the following investigations were done for all cases: stool analysis for three consecutive days, CBC, ESR, total proteins and serum albumin, tuberculin test, accelerated BCG test [in tuberculin negative cases], serum anti-EMA. Upper GIT endoscopy with duodenal biopsy and aspiration and tissue staining by H and E and by Immunohistochemical [anti-tTG moAbs] to detect tTG antigens in biopsy specimens. Lower GIT endoscopy with biopsy sampling and histopathological examination of biopsy specimens was also done. Out of the total patients, 18 cases [19.5%] were positive for celiac disease AEM antibodies while 16 were positive by tTG immunostaining of biopsy specimens. Fourteen patients [15.2%] had tuberculous enterocolitis while 12 [13%] had biopsy proven Giardiasis. On the other hand 48 patients [52.1%] had other undiagnosed causes of chronic diarrhea. A very high index of suspicion for CD should be maintained for patients who present with chronic diarrhea or iron deficiency anemia. The best method for diagnosis of celiac disease in such patients is serological testing followed by a small-bowel biopsy. The diagnosis of intestinal tuberculosis is difficult due to the lack of specific symptoms and signs. Colonoscopy with ileoscopy is a useful method for diagnosis of intestinal TB. Gastrointestinal endoscopy with biopsy examination is an important method of diagnosis and follows up of children with Giardiasis

Are patients with polycystic ovary syndrome at risk for cardiovascular disease?

Polycystic Ovary Syndrome [PCOS] is considered not only a reproductive endocrinopathy but also a metabolic disorder associated with long term health risks, including diabetes mellitus and coronary artery disease and it shares some or all components of syndrome X. To assess and evaluate cardiovascular risk factors in patients with PCOS and to compare the various systolic and diastolic function indices hetween PCOS patients and regularly cycling control women. 45 patients with PCOS [mean age 26.16 +/- 6.47 years and mean BMI 28.18"5.47 kg/m[2]] were recruited for this study, in addition to 30 healthy volunteer women with regular menstrual cycles, their age and body mass index [BMI] matched with the patients group. A thorough history and clinical examination were performed. Transvaginal sonography was performed using a transvaginal probe 5 MHZ. Hormonal assay for [serum luteinizing hormone [LH], follicle stimulating hormone [FSH], prolactin and total testosteronel, lipid profile [high and low density lipoproteins [HDL and LDL], triglyceride [TG] and total cholesterol [T Chol]], uric acid, homocysteine and two hours post prandial serum glucose [PPSG] levels were determined for all studied participants. M-mode, two dimension and Doppler echocardiography were performed for evaluation of systolic and diastolic function parameters. We have studied indices of cardiac flow in women with PCOS in relation to BMI, blood levels of reproductive hormones [LH, FSH. testosterone and prolactin]. lipid profile, uric acid, PPSG and homocsteine levels. The mean serum levels of LH, total testosterone, T Chol, TG, homocysteine, uric acid, PPSG, LH/FSH ratio and T Chol./HDL ratio were significantly higher in patients with PCOS group [p <0.001] than control group. Patients with PCOS had significantly lower peak mitral flow velocity in early diastole [PEV], ratio between peak mitral flow velocity in early diastole [E] and late diastole [A] [B/A ratio], Ejection fraction [EF] and fraction of shortening [FS] [p < 0.001] and significantly longer isovolumic relaxation time [IVRT] and E-velocity deceleration time [EVDT] [p <0.001]. Patients with PPSG> 140 mg/dl had significantly lower B/A ratio [p < 0.05] and those with BMI > 25 kg/m[2] had significantly longer IVRT [p <0.01]. Moreover, we found significant negative correlation between E/A ratio and both PPSG [r = -0.349] and LDL [r = -0.382] and significant positive correlation between IVRT and BMI [r = 0.415]. We conclude that both diastolic and systolic dysfunction are common findings in population with PCOS and this dysfunction together with the increased serum homocysteine concentration, dyslipidemia and impaired glucose tolerance may contribute to increased risk of cardiovascular disease in these patients