Nonalcoholic fatty liver disease (NAFLD) is one of the health problems with great burden on the liver that may end with liver cirrhosis and hepatocellular carcinoma. The aim of this work was to assess serum vitamin D level in nonalcoholic fatty liver disease children This cross sectional case control study involved 47 patients with nonalcoholic fatty liver disease selected while recruiting the pediatric hepatology clinics. Their ages ranged from 5–15 years and were compared with 23 healthy age and sex matched children. All involved patients were subjected to careful history taking, clinical examination and for patients and control, anthropometric measures for body mass index (BMI) calculation (plotted on WHO percentile growth charts), aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma glutamyl transferase (GGT), bilirubin (total and direct), serum albumin, creatinine, triglycerides, cholesterol, high density lipoprotein (HDL),low density lipoprotein (LDL), fasting blood glucose and fasting insulin (for calculation of insulin resistance), C reactive protein and serum vitamin D all were assayed. NAFLD was detected by ultrasonography and graded as absent, mild, moderate and severe. Ninety-three percent of NAFLD patients were obese. Significant differences were found between patients and control regarding AST, ALT, ALP, GGT, total and direct bilirubin, serum albumin, creatinine, triglycerides, cholesterol, HDL, fasting blood glucose, fasting insulin, the homeostatic model assessment for insulin resistance (HOMA-IR) and serum vitamin D levels. Significant negative correlation was found between serum vitamin D level and grades of steatosis. Serum vitamin D level decreases in children with NAFLD. This low serum vitamin D level is associated with higher stages of steatosis but not with BMI.
Thalassemia is a major health problem that disturbs the lifestyle of the affected patient. The aim of this work is to detect the impact of thalassemia on the quality of life regarding physical, social, emotional, psychological scored assessment.A case-control survey was conducted in Minia University children's hospital on 64 patients recruiting pediatric hematology outpatient clinic from July 2014 to February 2017. PedsQL™ 4.0 Generic Core Scale (Arabic version) was used to assess HRQOL in 64 thalassemia patients between 8 and 18 years of ages. Other related clinical data of the involved patients were collected from the pediatric hematology records.Mean physical, emotional, social, school performance, psychological and total scores (- 36.9 ± 20.9, 49.4 ± 17, 47.2 ± 21.3, 38.5 ± 15.5, 45.3 ± 13.8, 47.9 ± 38.8 respectively) were significantly decreased compared with control (p = 0.001 for all). The younger age group had better scores regarding social, emotional, psychological and total scores compared to older ones (p = 0.01, 0.03, 0.01 and 0.009 respectively). Older age of starting transfusion was statistically significant protecting factor from poor physical QOL in thalassemia patients (OR = 0.96, p = 0.03). The presence of hepatomegaly was a statistically significant predictor for poor physical QOL (OR = 8.5, p = 0.02). Household income was the statistically significant predictor for poor emotional QOL (OR = 5.03, p = 0.04). High serum ferritin was the statistically significant predictor for poor social QOL (OR = 1.1, CI 95%=, p = 0.04). Regarding poor psychological QOL (OR = 0.94, p = 0.01) and total QOL (OR = 0.94, p = 0.01) scores, older age of starting transfusion was the statistically significant protecting factor.Scheduled programs giving psychosocial help and a network connecting between the patients, school officials, thalassemia caregivers and the physician is required especially in developing countries where the health services are not integrated with social organizations. Special school services for thalassemia patients are required to deal with the repeated absence and anemia induced low mental performance of thalassemia children.
Beta Thalassemia represents a major public health problem in Egypt.The carrier rate varies between 5.5% to > 9%.It is estimated that there are 1000/1.5 million per year live births born with beta thalassemia. (1)β thalassemia occurs when there is a quantitative reduction of β globin chains that are usually structurally normal. (2)They are caused by mutations that nearly all affect the β globin locus and are extremely heterogeneous.Almost every possible defect affecting gene expression at transcription or post-transcriptional level, including translation, have been identified in β thalassemia. (3)These genetic defects lead to a variable reduction in β globin output ranging from a minimal deficit (mild β+ thalassemia alleles) to complete absence (β° thalassemia).Aim of the work:We aimed in this study to assess the molecular changes in transfusion dependent Beta thalassemia patients and the correlation of these molecular changes with their clinical outcomes.Patients & methods: This study will include 40 transfusion dependent β thalassemia patients with age range of 2 -18 years, recruiting the Pediatric Hematology unit in Minia University children hospital.Study procedure: β-Thalassemia mutation identification of samples will be performed by the reverse dot blot hybridization technique (RDB).For RDB, a panel of primers and probes using the beta globin strip assay well be used (β-Globin Strip Assay MED kit, VIENNA lab
Abstract Background: Physiological, pathological, and metabolic problems brought on by infection are what define the syndrome of sepsis. Neutrophil granules generated at human sites of infection and inflammation were the source of the first discovery of neutrophil associated gelatinase associated lipocalin (NGAL). Objectives: to evaluate the predictive usefulness of serum NGAL in children with sepsis in the pediatric intensive care unit and to compare it with the clinical score (PRISM III score) that is most frequently used to predict mortality in critically unwell children. ). Patients and methods: In this study 75 patients were included according to the inclusion criteria and divided into three groups 25 each (sepsis, septic shock, and multi-organ failure), and serum NGAL level in those group was measured and compared to 25 healthy controls. Results: Serum NGAL was significantly higher in both septic shock and multi-organ failure groups than in the control group. Positive bacterial cultures were linked to the severe degrees of sepsis which were associated with higher serum NGAL levels. Significant difference in serum NGAL level was found between survivors and non survivors, while no significant difference was found regarding PRISM III score. Conclusion: Serum NGAL is significantly elevated in severe sepsis. Serum NGAL level was superior on the frequently used PRISM III score to predict mortality in critically ill patients with sepsis. So, serum NGAL can be used as a marker for severity and prognosis for mortality in septic pediatric patients.
Background: Thrombocytopenia is a prevalent presentation in childhood with a broad spectrum of etiologies, associated findings, and clinical outcomes. Establishing the cause of thrombocytopenia and its proper management have obvious clinical repercussions but may be challenging. This article provides an adaptation of the high-quality Clinical Practice Guidelines (CPGs) of pediatric thrombocytopenia management to suit Egypt’s health care context. Methods: The Adapted ADAPTE methodology was used to identify the high-quality CPGs published between 2010 and 2020. An expert panel screened, assessed and reviewed the CPGs and formulated the adapted consensus recommendations based on the best available evidence. Discussion: The final CPG document provides consensus recommendations and implementation tools on the management of isolated thrombocytopenia in children and adolescents in Egypt. There is a scarcity of evidence to support recommendations for various management protocols. In general, complete clinical assessment, full blood count, and expert analysis of the peripheral blood smear are indicated at initial diagnosis to confirm a bleeding disorder, exclude secondary causes of thrombocytopenia and choose the type of work up required. The International Society of Hemostasis and thrombosis–Bleeding assessment tool (ISTH-SCC BAT) could be used for initial screening of bleeding manifestations. The diagnosis of immune thrombocytopenic purpura (ITP) is based principally on the exclusion of other causes of isolated thrombocytopenia. Future research should report the outcome of this adapted guideline and include cost-analysis evaluations.
Iron over- load in patients with beta-thalassemia major lead to alterations in the arterial structures and in the thickness of the carotid arteries. Doppler ultrasound scanning of extra-cranial internal carotid arteries is non-invasive and fairly quick to perform and may identify children at increased risk of stroke who would otherwise be missed. Increased carotid artery intima media thickness (cIMT) is a structural marker for early atherosclerosis and it correlates with the vascular risk factors and to the severity and extent of coronary artery disease. Objective: To evaluate the role of carotid Doppler examination and cIMT measurement as a predictive to atherosclerotic changes in BTM children with iron overload. Patients and Methods: Sixty two children with (BTM) and 30 healthy normal controls of matched age and sex were included. Complete blood count, serum iron, ferritin, serum cholesterol and hemoglobin electrophoresis were performed as well as carotid Doppler ultrasonography to measure the (cIMT) in both patients and controls. Results: By using carotid Doppler we found that cIMT of thalassemic patients was significantly increased compared to normal controls and there was significant positive correlation between CIMT and patient's age, weight, height, BMI, duration of illness, serum cholesterol, iron overload parameters as serum iron, serum ferritin, frequent blood transfusion, irregular use of iron chelating agents and in those who had splenectomy. Significant negative correlation was found between CIMT and hematocrit value but no significant correlation was found between CIMT and Hb level. Conclusion: Carotid Doppler is very useful method in measurement of cIMT that increased in thalassemic patients which shows a strong relationship with features of iron overload. We recommend the routine use of Doppler measurement of cIMT in these patients to predict early atherosclerotic changes as well as in the follow-up to prevent progression of atherosclerosis.
To assess the utility of 64 MDCT in the diagnosis, staging and follow-up of HB patients. This study included 17 children, 9 males and 8 females with HB from multiple centers aged 6 months- 3 years. Patients were subjected to complete clinical examination and laboratory investigations at presentation and during follow-up. Patients were evaluated, staged and treated according to SIOPEL HB protocols for management. Imaging evaluation was conducted using abdominal U/S, bone scan and MDCT for chest and abdomen. All patients were presented with abdominal mass and elevated AFP level, Jaundice seen in only 1 patient. On MDCT, 13 patients had single focal lesion, 2 patients had multiple foci and 2 patients showed diffuse disease. MDCT was more accurate than US in detecting site, multiplicity, density and lymph nodes (LNs). Regarding tumor staging, MDCT findings were confirmed surgically. In follow-up of patients MDCT was detected with accuracy residual or recurrent lesions in 3 cases. MDCT in children with HB accurately displayed the extent of hepatic involvement by tumor, tumor staging and its proximity to vascular structures that help the surgeon to identify the tumor resectability and can be used in follow-up.