Body composition analysis using dual-energy x-ray absorptiometry in an Egyptian pediatric sickle cell disease cohort.

BACKGROUND: Growth failure is commonly encountered in sickle cell disease (SCD). Tissue compartment growth and development are subsequently likely to be altered in such patients. OBJECTIVE: We aimed to analyze body composition in an Egyptian pediatric SCD cohort using dual-energy x-ray absorptiometry (DEXA), one of the most comprehensive and noninvasive assessment methods available. METHODS: Forty children with SCD ≤18 years and 40 healthy youngsters age- and gender-matched were enrolled. Patients' demographic, clinical, and laboratory parameters were obtained from their archived files. All patients and controls were subjected to body composition assessment using a MedixDR-Whole Body DEXA System. RESULTS: In SCD patients; weight and height relative to age Z scores were significantly lower (p < .001), total body lean was significantly higher (p = .006), and total body fat percentage was lower, yet the difference was not statistically significant (p = .09). There were no statistically significant variations in bone mineral density or content, basal metabolic rate, subcutaneous adipose tissue, android/gynoid fat ratio, and visceral adipose tissue. There were no significant gender disparities between SCD patients and controls. CONCLUSION: Faltering growth in children with SCD should be addressed with a multidisciplinary approach including nutritional support, correction of anemia, and proper medical care. Body composition parameters assessed using DEXA were comparable between cases and controls apart from total body lean. Further clinical studies are needed with multicenter cooperation and a larger sample size to assess the usefulness of DEXA as an assessment tool for body composition in children with SCD.

Antibiotics Prophylaxis Practice in Arthroplasty Surgeries.

Background Infection in orthopedic surgery is one of the most dreaded complications. It is associated with prolonged morbidity, disability, and increased mortality. One of the cornerstones of the prevention of infections is antibiotic prophylaxis. This study assessed the practice of antibiotic prophylaxis in arthroplasty surgeries in our local hospital. Methods One hundred and seventy-one elective joint replacement patients were retrospectively analyzed for documentation of antibiotic plan in postoperative instruction, choice of antibiotic, dose, and dosage. Compliance with the dosage (duration and frequency) of antibiotic prophylaxis was compared among patients who underwent different operations, among patients whose operation notes had antibiotics plans, and among those patients whose operation notes lacked this information. Results Ninety-six females and 75 males with a mean age of 71.4±9.8 years who underwent hip replacement, knee replacement, or shoulder replacement were included in this study. Preoperative and postoperative antibiotics were received by 100% and 94.7% of patients, respectively. In 19.3%, there was no instruction about postoperative antibiotics while 4% missed at least one postoperative dose. The dosage of postoperative prophylactic antibiotics was variable as 26.3% of the patients experienced delayed administration of doses. Not having intravenous access, failure to prescribe antibiotics, and prescribing antibiotics in the "once only" rather than "regular medication" section of the medication chart were the reasons for improper timing of antibiotic doses. Observing surgical safety checklist was effective in ensuring preoperative antibiotic administration, whereas failing to document antibiotic plan in operation note was associated with poor compliance with postoperative dosage. Interprofessional participation is crucial to compliance with antibiotic prophylaxis practice. Conclusion This study identified key areas for improvement in our antibiotics prophylaxis practice. It resulted in implementing strategies to improve staff's awareness about the importance of timely administration of prophylactic antibiotics and proper documentation by all team members.

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.

Serum Hepcidin as a Diagnostic Marker of Severe Iron Overload in Beta-thalassemia Major.

OBJECTIVES: To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with ß-thalassemia. METHODS: A study was conducted on 30 thalassemia major (TM), 30 thalassemia intermedia (TI) and 60 healthy children as controls. Serum hepcidin was measured by Human Hepcidin, ELISA Kit. RESULTS: ß-thalassemia patients had a higher serum hepcidin compared to the controls (p < 0.001). TM group had higher hepcidin and ferritin compared to the TI group (p = 0.034; < 0.001, respectively). Among controls, hepcidin did not correlate with age (r = 0.225, p = 0.084). Among ß-thalassemia patients, it correlated positively with age (r = 0.4; p = 0.001), disease duration (r = 0.5; p < 0.001), transfusion frequency (r = 0.35; p = 0.007), total number of transfusions (r = 0.4; p = 0.003), and ferritin (r = 0.3; p = 0.027). Total hemoglobin and serum ferritin were significantly related to hepcidin, which tended to increase by 0.514 ng/ml with each 1 g/dl rise in hemoglobin (p = 0.023) and by 0.002 ng/ml with each 1 ng/ml rise in serum ferritin (p = 0.002). Iron overload [serum ferritin (SF) ≥ 1500 ng/ml] was independently associated with TM (p = 0.001) and elevated serum hepcidin (p = 0.02). The overall predictability of serum hepcidin in severe iron overload was statistically significant when compared to hepcidin to serum ferritin ratio. CONCLUSIONS: Serum hepcidin is elevated in children with ß-thalassemia; but this elevation is more evident in TM patients with severe iron overload. Thus, hepcidin can be a potential marker of severe iron overload in patients with TM. Further studies are recommended to compare serum hepcidin and serum ferritin in the prediction of severe iron overload in steady state and during infection or inflammation.

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.

Epidemiology of primary nephrotic syndrome in Egyptian children.

BACKGROUND: Primary nephrotic syndrome is a common renal problem in pediatrics, with great variation in patients' characteristics in different regions of the world. The aim of this study was to define these characteristics in Egyptian children with primary nephrotic syndrome. METHODS: Records of 100 primary nephrotic syndrome patients were retrospectively reviewed. Demographic, clinical, histopathological data and response to therapy were analyzed. RESULTS: The mean age of onset was 4.43 ± 2.7 years. Thirty-four percent of patients were steroid resistant, and 66% showed initial steroid response; 46 of the latter were steroid dependent. Forty patients underwent a renal biopsy with minimal change nephrotic syndrome occurring in 30%, mesangioproliferative glomerulonephritis in 37.5% and focal segmental glomerulosclerosis in 30%. Nine percent of cases developed chronic renal insufficiency. Response to cyclophosphamide and cyclosporine occurred in 37.5% and 33.3% of steroid-resistant nephrotic syndrome patients, respectively. CONCLUSIONS: A greater percentage of steroid-resistant patients were found in our patients compared with those in other studies. Response to immunosuppressives was different from other studies, probably due to differences in the priority of selection for immunosuppressive therapy.

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.

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.