continuous glucose monitoring system [CGMS] in patients with beta-thalassemia major

Background: Blood transfusion-dependent beta Thalassemia Major [BTM] patients are at risk of hemosiderosis. Hemosiderosis of pancreas results in impaired glucose homeostasis tolerance and diabetes mellitus [DM]. Since glycosylated hemoglobin has limited role in patients with hemoglobinopathies, this study was conducted as a first attempt worldwide to understand glucose homeostasis and evaluate efficacy of Continuous Glucose Monitoring [CGM] system as a diagnostic tool of abnormal glucose homeostasis in these patients

Materials and Methods: A case series study of six non-diabetic, transfusion-dependent beta-thalassemia patients aged 9-13-year-old. Clinical and laboratory data were collected on admission for their monthly transfusion. Patients were connected to CGM systems for one day

Findings: Using CGM and based on American Diabetes Association guidelines, three patients were found to have abnormal glucose levels of diabetic range. The other three showed impaired glucose tolerance. Among all patients, glycosylated hemoglobin [HbA1C] readings have an inverse relationship to CGM and calibration readings

Conclusion: CGM could be a promising tool for evaluating BTM patients. Larger studies are recommended

Cerebral malaria and the prognostic indicators in children of central Sudan

Identifying children with cerebral Malaria [CM] who are at high risk of a fatal outcome depends on recognition of clinical signs, as well as laboratory variables. No comprehensive data on the clinical features and their importance in the prognosis of cerebral malaria in the Sudan are available. The aims of this study were to identify the clinical presentation of children with CM in Central Sudan and to evaluate the importance of clinical signs and laboratory investigations in determining the outcome of CM in children. 409 children [mean age 6.1 +/- 3.3 years] with CM were enrolled to the study. Comprehensive clinical and biochemical assessment were determined. The case fatality rate was 12% with the highest rate in younger children [< 2 years of age]. Univariate and multiple regression analysis identified six significant prognostic indicators in these children. These are, age /= 6.6 mmol/L [RR, 3.989; 95% CI, 2.359 - 6.746] and haemoglobin concentration

Segregation analysis of susceptibility/resistance to periportal fibrosis in Schistosoma mansoni infections in endemic area [Umzoukra village - Gezira State - Sudan]

We carried out segregation analysis of susceptibility to periportal fibrosis caused by Shistosoma mansoni. A regressive logistic model of the disease was applied to 42 pedigrees ascertained during screening of one the infection endemic area of Sudan. The affected status was defined as a presence of the fibrosis irrespectively to severity of injury. Transmission probability model indicated a significant contribution of a major gene in the control of the pathology. The susceptibility to periportal fibrosis could be described within the framework of a recessive major gene diallele model, assuming incomplete age and squared-age dependent penetrance of genotypes. According to this model, the disease was manifested in almost all homozygous carriers of the mutant allele, while the risk of the disease for normal homozygotes and heterozygotes did not exceed 0.31. The frequency of disease allele in the population studied was estimated as 0.59. We also found non-majorgene phenotypic correlations between parents and offspring in the incidence of periportal fibrosis caused by Schistosoma mansoni

Regression of liver fibrosis in Schistosoma mansoni infected Sudanese subjects after praziquantel treatment

1-To evaluate the effect of Praziquantel [PZQ] therapy on the regression of liver fibrosis in an endemic population. 2-To determine the factors controlling the regression of hepatic fibrosis [e.g. gender, age and grade of fibrosis]. An association study of a cohort of one hundred seventy seven Sudanese patients infected with Schistosoma mansoni [82 males 46%, 95 females 54%] was conducted to evaluate the factors controlling the regression of liver fibrosis 39 months after treatment with PZQ using ultrasound evaluation. SPSS [Statistical Package for Social Science] software was used for statistical analysis. Chi- Square was used to compare the two phenotypes [regression and progression] in the study subjects. PPF was regressed in 63 patients [36%] from higher grades of fibrosis to lower ones. While in 24 patients [13%] the disease progressed to higher grades. In addition, the grade of PPF did not change in 90 patients [51%]. The mean values of portal vein diameter [PVD], splenic vein diameter [SVD], and index liver size [ILS] in subjects in whom PPF regressed after treatment were significantly lower than in subjects in whom the disease was progressed [P <.0001, P =.031, and P =.003 respectively].The progression of hepatic fibrosis in males [n = 15.18%] was greater than that of females [n = 9.9%]. Patients who showed regression of PPF or progression of the disease tend to cluster in certain families. Our study indicated that regression and stabilization of PPF after PZQ therapy is controlled by gender, age, grade of fibrosis, and possibly inherited factors