Urinary Transforming Growth Factor ß-1 as a Marker of Renal Dysfunction in Sickle Cell Disease.

BACKGROUND: Sickle cell disease (SCD) is a genetic disorder that results in deformity of red blood cells. Renal dysfunction affects 5-18% of patients with SCD. To date, few studies have described urinary levels of transforming growth factor ß-1 (TGF-ß1), which is a marker of fibrosis, as a biomarker in identifying patients at risk of developing renal disease in SCD. The aim of this study is to determine prevalence of sickle cell nephropathy in Egyptian SCD patients. We aimed also to evaluate the association of urinary TGF-ß1 with other conventional biomarkers of renal damage in SCD patients to identify a novel renal biomarker for early diagnosis of sickle nephropathy. METHODS: We examined 40 SCD patients, 21 with sickle cell anemia, 16 sickle thalassemia, and three with sickle trait. We compared them to 20 control children with matched age and sex. The study was held in the time period between May 2013 and December 2013 in the Hematology Clinic, New Cairo University Children Hospital, Cairo, Egypt. RESULTS: Urinary excretion of TGF-ß1 was 7.07 ± 1.91 ng/mL in SCD patients versus 2.23 ± 0.76 ng/mL in control children (p < 0.001). SCD patients had elevated estimated glomerular filtration rate (177.44 ± 35.6 mL/min/1.73 m(2)), denoting a state of glomerular hyperfiltration. 47.5% of SCD patients had microalbuminuria. Urinary TGF-ß1 correlated positively with microalbuminuria and estimated glomerular filtration rate (p = 0.001 and p = 0.018, respectively). CONCLUSION: We concluded that urinary TGF-ß1 may serve as a marker of early renal injury in SCD.

Liver Enzymes in Children with beta-Thalassemia Major: Correlation with Iron Overload and Viral Hepatitis.

BACKGROUND: Beta Thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%) with an estimated carrier rate of 9-10.2%. Injury to the liver, whether acute or chronic, eventually results in an increase in serum concentrations of Alanine transaminase (ALT) and Aspartate transaminase (AST). AIM: Evaluating the potentiating effect of iron overload & viral hepatitis infection on the liver enzymes. PATIENTS AND METHODS: Eighty (80) thalassemia major patients were studied with respect to liver enzymes, ferritin, transferrin saturation, HBsAg, anti-HCV antibody and HCV-PCR for anti-HCV positive patients. RESULTS: Fifty % of the patients were anti-HCV positive and 55% of them were HCV-PCR positive. Patients with elevated ALT and AST levels had significantly higher mean serum ferritin than those with normal levels. Anti-HCV positive patients had higher mean serum ferritin, serum ALT, AST and GGT levels and higher age and duration of blood transfusion than the negative group. HCV-PCR positive patients had higher mean serum ferritin and serum ALT and also higher age and duration of blood transfusion than the negative group. CONCLUSION: Iron overload is a main leading cause of elevated liver enzymes, and presence of HCV infection is significantly related to the increased iron overload.

Damage index in childhood-onset systemic lupus erythematosus in Egypt.

BACKGROUND: To investigate the prevalence of cumulative organ damage among Egyptian children with juvenile-onset systemic lupus erythematosus (jSLE) and the relationships between the organ damage and the demographic data, clinical variables, and disease activity. METHODS: A total of 148 patients with jSLE have been followed in the pediatric rheumatology clinic and section at Cairo University. These patients were evaluated by retrospective chart review. The organ system damage due to SLE was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Risk factors for damage were also studied including demographic criteria as well as clinical and laboratory manifestations. RESULTS: Overall, 43.9% of the patients had damage within a mean of 6.57 ± 3.59 years of disease diagnosis. Neuropsychiatric (NPS-21%) and renal (16.9%) system involvement were observed most frequently, followed by cardiovascular (11.5%), skin (9.5%), pulmonary (6.1%), and ocular (4.8%), with a mean SDI score of 0.93 ± 1.37. In our study, the presence of neuropsychiatric manifestations at diagnosis showed the strongest association with the presence of later disease damage.The number of SLE diagnostic criteria at presentation was strongly associated with the total SDI score, and the renal damage was significantly more prevalent in patients with age at disease diagnosis below 10 years of age. A higher mean disease duration was found in patients with musculoskeletal damage. CONCLUSION: We found that cumulative organ damage, as measured by the SDI, was present in 43.9% of Egyptian patients with juvenile-onset SLE. The damage was significantly more likely in patients who had more SLE diagnostic criteria at time of disease presentation and NPS manifestations at the time of diagnosis.

Alloimmunization and erythrocyte autoimmunization in transfusion-dependent Egyptian thalassemic patients.

BACKGROUND: Alloimmunization to red blood cells' (RBCs) antigens and formation of autoantibodies against RBCs is a frequent complication among immunocompetent transfusion-dependent patients. Autoantibodies can result in clinical hemolysis and difficulty in cross-matching blood. The objective of this study was to evaluate the presence of alloantibodies and autoantibodies in regularly transfused ß-thalassemic patients and the factors influencing the development of alloantibodies. MATERIALS AND METHODS: The clinical and transfusion records of 95 Egyptian ß-thalassemic patients, with a mean age of 17.07 years, presenting to the National Blood Transfusion Centre for regular blood transfusion were evaluated for alloimmunization and antibody formation. RESULTS: Alloantibodies were encountered in 27 patients (28.4%). The most frequent alloantibodies encountered were anti-Kell (23.6%) and anti-E (23.6%). Patients with blood group O were the highest in developing antibodies (37.9%). Patients with blood phenotypes R2r Kell negative developed more alloantibodies. Autoantibodies were encountered in only 1 patient. CONCLUSIONS: Alloimmunization to RBCs' antigens is a frequent finding among Egyptian transfusion-dependent thalassemic patients, with the majority of patients being transfused with blood matched for ABO and D antigens only. Absence of phenotypically matched donors, except for a limited number of patients, may have contributed to this problem.

Profile of infections in newly diagnosed patients with acute leukemia during the induction phase of treatment.

BACKGROUND AND PURPOSE: Acute leukemia is the most common pediatric malignancy. Despite the significant progress in the treatment of infectious complications, infection-related morbidity and mortality continue to be of great importance. Prompt initiation of the appropriate empiric antibiotic treatment has improved infection outcome. The aim of the present study is to assess the type, frequency, and severity of infectious complications in a cohort of pediatric cancer patients treated at a single medical institution. We also aim to identify factors affecting bloodstream infections in newly diagnosed ALL and AML pediatric patients during the induction phase of treatment. PATIENTS AND METHODS: This study was carried out at the Department of Pediatric Oncology, National Cancer Institute, Cairo University, during the time period from January 1st to June 30th 2007. Inclusion criteria were pediatric age group (from 0-16 years), newly diagnosed acute leukemia, positive blood culture and documented site of infection. Data were analyzed using the SPSS package version 15. A p-value ≤0.05 was considered significant. RESULTS: This is a retrospective study including 100 newly diagnosed cases of acute leukemia. Fifty-four patients had ALL, and 46 patients had AML. 348 infectious episodes were recorded. Blood stream infections (BSI) occurred once or twice in 32%, 3-4 episodes in 58%, and five or more episodes in 10% of the cases. Gram-positive cocci were the most frequently observed cause of BSI, accounting for 77.9% of the total isolates followed by Gram negative organisms seen in 18.9% and mixed infections in 8%. The majority of the episodes (n= 208, 58.4%) responded to first-line empirical antibiotic therapy. CONCLUSION: Clinical and laboratory risk factors could be identified and can help prediction of serious BSI. KEY WORDS: Acute leukemia - Blood stream infection - Bacterial infection - Pediatric cancer patients.