Beta Thalassemia Carrier Rate: Problem Burden among High School Children.

Background & Aim of the Work: ß-Thalassemia (ßT) is highly prevalent in some countries like Egypt. Accurate data about actual disease prevalence and heavily prevalent geographic locations are essential to help in early detection and in setting up effective preventive programs. We aim for screening ßT carriers among Egyptian high school students in the Delta region. SUBJECTS AND METHODS: A cross-sectional multicenter study was carried out on 4320 randomly selected students from four governorates of the Nile Delta region, Egypt. All patients were to be tested for their complete blood count. Those with microcytic hypochromic anemia not caused by iron deficiency were tested for ßT carrier status using high-performance liquid chromatography. RESULTS: The total prevalence of ßT carrier rate was 6.13%. The highest prevalence was detected in Al-Sharkia Governorate, reaching 7.89%, followed by 6.90% in Al-Gharbia Governorate. Al- Dakahilia and Al-Menoufia showed lower rates of 4.86% and 3.73%, respectively. CONCLUSION: Despite the premarital national screening program for ßT in Egypt, the carrier rate is still high. More effort should be done into the proper implementation of national prevention programs.

Role of serum cystatin C in the prediction of acute kidney injury following pediatric cardiac surgeries: A single center experience.

Intense contemporary research is directed towards validating novel biomarkers to predict acute kidney injury (AKI) in children undergoing cardiothoracic surgeries. We aimed to evaluate the role of cystatin C in early prediction of AKI following cardiac surgery in children with congenital heart disease. Prospective observational cohort study was conducted on 40 children with congenital heart disease undergoing cardiac surgery. 40 healthy children with matched age and sex were enrolled as a control group. Children were subjected to physical examination, routine blood tests, echocardiography, and measurement of plasma cystatin C level on different occasions. The median age of the patients was 3.65 years, a range from 1 to 5 years with no significant difference regarding the age and sex of cases and control groups. The mean serum cystatin C level in patients was 0.75 ±â€…0.15, 1.35 ±â€…0.34 and 1.21 ±â€…0.38 mg/dL (preoperative, at 6 h and at 24 h postoperative, respectively) with statistically significant difference P < .05. 30% of the patients developed postoperative AKI with significantly higher serum cystatin C at 6 hours postoperative >1.33 mg/dL compared to preoperative level p P < .05. Serum cystatin C level was positively correlated with cardiac bypass time, ischemic time and length of hospital stay at 6 hours postoperative. Serum cystatin C is a sensitive marker for early detection of AKI following cardiac surgery in children with congenital heart disease and it was positively correlated with cardiac bypass time, ischemic time and length of hospital stay.

Assessment of cystatin C in pediatric sickle cell disease and ß-thalassemia as a marker of subclinical cardiovascular dysfunction: a case-control study.

Cardiovascular complications represent the main determinant of survival in patients with hemoglobinopathies. Serum cystatin C is a well-known marker of nephropathy in sickle cell disease (SCD) and ß-thalassemia patients that has recently emerged as a strong predictor of cardiovascular dysfunction in patients with and without kidney disease. We performed a case control study to determine the role of cystatin C as a predictor of subclinical cardiovascular dysfunction in SCD and ß-thalassemia patients. We enrolled 40 SCD patients with a mean age of 12.4 years, 40 ß-thalassemia patients with a mean age of 11.4 years and 40 age and sex-matched controls. We assessed hematological profile, serum ferritin, urinary albumin-creatinine ratio (UACR), serum cystatin C, echocardiography and carotid intima media thickness (CIMT). UACR, cystatin C and CIMT were higher in SCD and ß-thalassemia patients compared to controls (p < .001). Significantly higher cystatin C levels were observed in SCD and ß-thalassemia patients with nephropathy or left ventricular systolic dysfunction (shortening fraction <30%, or ejection fraction <55%; p < .001). Moreover, SCD patients with pulmonary hypertension had significantly higher cystatin C levels. Cystatin C levels were positively correlated with CIMT in SCD (p = .02) and ß-thalassemia patients (p < .001) while negatively correlated with ejection fraction and shortening fraction (p < .001). The cutoff values of cystatin C ≥ 16.03 and ≥ 13.2 (ng/mL) could detect subclinical cardiac dysfunction risk among SCD and ß-thalassemia patients respectively. Cystatin C appears to be a promising marker for subclinical cardiovascular dysfunction in SCD and ß-thalassemia patients.

Mean platelet volume in children with systemic lupus erythematosus.

BACKGROUND: Platelets are heterogeneous in size, density, metabolic, functional, and biochemical properties. Mean platelet volume (MPV) is a measure of the average size of platelet in a blood sample. AIM: We aimed to evaluate the mean platelet volume as a marker for disease activity in children with systemic lupus erythematosus. MATERIALS AND METHODS: This was a prospective case-control study, which included 50 patients with SLE and 50 age and sex, matched healthy controls. All subjects were subjected to history taking, physical examination and laboratory parameters in active and remission phases of the diseases. RESULTS: The MPV value in the SLE group was significantly higher than control group (9.6 ± 1.3 fL, 9.1 ± 0.57 fL, respectively, p = 0.04). There was a significant increase of weight, blood pressure, urea, creatinine, proteinuria, CRP, ESR, cholestrol, MPV values, SLEDAI-2K scores and significant decrease of HB, albumin, C3, mean platelet volume (MPC) in the active stage than in the remission stage. There was a significant negative correlation between MPV and MPC in active stage of the disease but the correlation was insignificant in remission stage. CONCLUSION: MPV increased in active phase of patients with SLE and can be an easy, rapid, inexpensive and simple method to assess disease activity in children with SLE.

Evaluation of the role of ischemia modified albumin in neonatal hypoxic-ischemic encephalopathy.

BACKGROUND: Birth asphyxia is a leading cause of neonatal mortality. Ischemia-modified albumin (IMA) levels may have a predictive role in the identification and prevention of hypoxic disorders, as they increase in cases of ischemia of the liver, heart, brain, bowel, and kidney. PURPOSE: This study aimed to assess the value of IMA levels as a diagnostic marker for neonatal hypoxic-ischemic encephalopathy (HIE). METHODS: Sixty newborns who fulfilled 3 or more of the clinical and biochemical criteria and developed HIE as defined by Levene staging were included in our study as the asphyxia group. Neonates with congenital malformation, systemic infection, intrauterine growth retardation, low-birth weight, cardiac or hemolytic disease, family history of neurological diseases, congenital or perinatal infections, preeclampsia, diabetes, and renal diseases were excluded from the study. Sixty healthy neonates matched for gestational age and with no maternal history of illness, established respiration at birth, and an Apgar score ≥7 at 1 and 5 minutes were included as the control group. IMA was determined by double-antibody enzymelinked immunosorbent assay of a cord blood sample collected within 30 minutes after birth. RESULTS: Cord blood IMA levels were higher in asphyxiated newborns than in controls (250.83±36.07 pmol/mL vs. 120.24±38.9 pmol/mL). Comparison of IMA levels by HIE stage revealed a highly significant difference among them (207.3±26.65, 259.28±11.68, 294.99±4.41 pmol/mL for mild, moderate, and severe, respectively). At a cutoff of 197.6 pmol/mL, the sensitivity was 84.5%, specificity was 86%, positive predictive value was 82.8%, negative predictive value was 88.3%, and area under the curve was 0.963 (P<0.001). CONCLUSION: IMA levels can be a reliable marker for the early diagnosis of neonatal HIE and can be a predictor of injury severity.

Evaluation of serum interleukin 17 and zinc levels in recalcitrant viral wart.

BACKGROUND: Warts are benign epithelial proliferations of the skin and mucosa caused by infection with HPV. Low IL-17 levels may contribute in occurrence, maintenance, severity, and recurrence of different types of cutaneous wart that depend mainly on the cell-mediated immunity defect. In a majority of the patients, zinc deficiency was associated with persistent, progressive, or recurrent viral warts. A careful dose of oral zinc sulfate may be helpful in the management of such patients. Zn deficiency negatively affects the Th17 cells. IL 6 induced STAT3 activation during chronic inflammation and Th17 development suppressed by Zn via attenuating this activation critically controls Th17-cell development. OBJECTIVES: To evaluate the role of interleukin 17 and zinc in recalcitrant warts. PATENTS AND METHODS: All studied patients were subjected to history taking and dermatological examination. The evaluation of serum IL-17 level was done by ELISA in 25 recalcitrant wart patients and 25 wart patients. The measurement of serum zinc level was determined by colorimetric methods, using Au 480 Beckman coulter chemistry analyzer. RESULTS: The results revealed a significant decrease in serum IL-17 and zinc levels in recalcitrant wart patients. CONCLUSION: Both IL-17 and zinc deficiency have a role in the pathogenesis of recalcitrant warts through the imbalance of immune system and deficiency of immune cells. There is no significant correlation between serum levels of IL-17 and zinc, suggesting that they have different mechanisms in affecting the immune system.

Soluble CD27 and MIF as possible serum biomarkers of vitiligo activity in Egyptian patients in Sharkia Governorate.

Contrary to other inflammatory skin disorders like psoriasis or atopic dermatitis, vitiligo does not present with distinct inflammatory symptoms that can be easily evaluated by clinical examination. Identification of a putative biomarker to inform early and accurate treatment responses could be of considerable value. This study aims to validate levels of serum soluble CD27 (sCD27) and macrophage Migration Inhibitory Factor (MIF) as biomarkers of vitiligo to improve the quality of disease management. This cross-sectional study was conducted on 32 vitiligo patients, stratified into two subgroups of 22 active and 10 stable vitiligo patients; the stable group containing 1 segmental and 9 nonsegmental presentations, and 32 matched healthy individuals as the control group. Of the 32 patients in the study, 21 were female and 11 were male with a median age of 30 years. The measurements of the study parameters of sCD27 and MIF in the serum were carried out through blood sampling and followed up for three months at onemonth intervals for stable vitiligo cases. Mean serum levels of sCD27 and MIF were significantly higher in vitiligo patients than in the control group. A positive correlation was observed in active vitiligo cases between both serum MIF and sCD27 levels and the spreading item of Vitiligo European Task Force (VETF) score as an indicator of disease activity. Serum sCD27 and MIF levels in vitiligo patients were observed to be higher than that of controls with greater correlation found for sCD27 with disease activity.

Study of glucocorticoid receptors in T lymphocytes (CD3/GCR) as predictor of steroid responsiveness in Egyptian children with idiopathic nephrotic syndrome.

Little was known about the relationships between the T lymphocytes (CD3+), expression of glucocorticoid receptors (GCR) and the response to GC treatment in children with the idiopathic nephrotic syndrome (INS). Our objective was to determine the relation between steroid responsiveness and GCR expression in T lymphocytes. The present study was carried out on 80 children with new-onset INS admitted in Pediatric Nephrology Units of Zagazig and Tanta University Hospitals and on 40 healthy children of the same age and sex who served as control group. The Subjects were subdivided into three groups as follows: Group 1 with 40 healthy children of comparable age and sex served as control group; Group 2 consisted of 60 patients diagnosed with INS with early response to steroid therapy [early responder (ER)] and Group 3 with 20 patients diagnosed with INS with late response to steroid therapy [late responder (LR)]. They were subjected to history taking, focusing on the pattern of response to steroids (ERs), clinical examination, routine laboratory investigations and the GCR/CD3% relationship. 75% of newly diagnosed INS cases were ER whereas 25% were LR. GCR/CD3% was significantly lower in LR group in comparison with ER and control groups, with a significant negative correlation between time of steroid responsiveness and GCR/CD3%. LR group showed lower GCR expression in T lymphocytes before starting therapy which may mean that GCR expression could be part of a pathophysiological mechanism of steroid responsiveness in these children and can be used as a useful diagnostic marker to predict steroid responsiveness in patients with INS.

ADAMTS-13 level in children with severe diarrhea-associated hemolytic uremic syndrome: Unmasking new association.

Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS). We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case-control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014. The study included 15 consecutive children with typical HUS as well as 15 healthy children as a control group. Routine laboratory investigations were performed. Assessment of serum ADAMTS-13 level was performed using the Quantikine human ADAMTS-13 ELISA kit. Data were analyzed using Statistical Package for Social Sciences version 16. Nonparametric values were expressed as median and range, and the median of two groups was tested by Mann-Whitney test. The serum ADAMTS-13 level was significantly lower in HUS patients when compared to the control group (P < 0.05). There were significant negative correlations between ADAMTS-13 level and duration on dialysis, as well as serum urea and creatinine. Furthermore, there were significant positive correlations between serum ADAMTS-13 level and both hemoglobin level and platelet count. Our study suggests that the ADAMTS-13 level was decreased in children with severe typical HUS and its deficiency correlated with disease severity.

Screening of renal dysfunction among Burkitt lymphoma survivors by novel markers.

BACKGROUND: Burkitt lymphoma (BL) represents the most common pathological type of non-Hodgkin lymphoma in our region. Recently, high success rates have been achieved in BL treatment. Little is known about long-term renal dysfunction in this vulnerable group. In the present study, we tried to detect early chronic kidney diseases (CKD) among BL survivors by using novel screening modalities. PATIENTS AND METHODS: we investigated 53 children (aged 10 ± 2.8 years, 34 boys) who successfully treated for Burkitt lymphoma, based on LMB96 protocol, as "patient group" and 30 children as control. All eligible participants were subjected to history taking, physical assessment, and routine laboratory investigations including urine analysis, serum creatinine. Estimated glomerular filtration rates using new Schwartz formula (GFRCKD) were calculated and chronic kidney disease prevalence was diagnosed accordingly. Also, serum Cystatin-C (Cys-C) and neutrophil-gelatinase-associated Lipocalin (NGAL) were determined as novel markers aiming at early and accurate detection of CKD in BL survivors. RESULTS: After 18.3 ± 5.2 months of BL cytotoxic therapy completion, almost one fifth of asymptomatic BL survivors showed evidence of subclinical CKD when estimated GFRCKD (16.9%), serum Cystatin-C (15%) and serum neutrophil-gelatinase-associated Lipocalin (18.8%) were used for kidney function monitoring. This prevalence was four to fivefolds higher than that detected by routine serum creatinine screening (3.7%). Significant persistent albuminuria was diagnosed at 4/53 (7.5.3%) of BL survivors and asymptomatic hypertension was reported in 1/53 (1.9%) of them compared to none of the controls. Positive correlation could be displayed between serum Cys-C and serum NGAL. Conversely, negative correlations between both of them and estimated GFRCKD were documented. CONCLUSION: Novel modalities such new Schwartz formula (GFRCKD) estimation, serum Cys-C, and serum NGAL assessment should be incorporated in the routine follow-up screening for CKD among BL survivors for accurate diagnosis of such detrimental morbidity.

The Role of Pancreatic Stone Protein in Diagnosis of Early Onset Neonatal Sepsis.

Introduction. Early diagnosis and treatment of neonatal sepsis may help decrease neonatal mortality. Aim of the Study. To evaluate the role of pancreatic stone protein as a marker for early onset neonatal sepsis. Methods. A hospital-based prospective study was conducted on 104 (52 uninfected and 52 infected neonates) admitted to the Neonatal Intensive Care Unit (NICU) of Zagazig University hospitals during the period from April 2014 to April 2015. All newborns were subjected to full history taking, careful neonatal assessment, blood, C-reactive protein (CRP), and serum pancreatic stone protein. Results. Serum PSP levels were significantly higher in the infected group than in the uninfected group. At a cutoff level of PSP 12.96 ng/mL, the sensitivity was 96.2%, the specificity was 88.5%, positive predictive value was 95.8%, negative predictive value was 89.3%, and area under the curve was 0.87. A significant positive correlation between CRP and PSP was found in infected group. Conclusion. The high negative predictive value of PSP (89.3%) indicates that the serum PSP level is a good marker for diagnosis of early onset neonatal sepsis and can be used to limit hospital stay and antibiotic use in neonates treated for suspected sepsis.

Plasma neutrophil gelatinase-associated lipocalin as a marker for the prediction of worsening renal function in children hospitalized for acute heart failure.

Acute heart failure (AHF) is frequently associated with worsening renal function in adult patients. Neutrophil gelatinase-associated lipocalin (NGAL) serves as an early marker for acute renal tubular injury. To assess the role of plasma NGAL in predicting worsening renal function (WRF) in children with AHF, we studied 30 children hospitalized for AHF; children with history of chronic renal disease or on nephrotoxic drugs were excluded. Twenty age- and sex-matched healthy children were included in the study as a control group. Echocardiographic examination was performed on admission. Blood urea nitrogen (BUN), serum creatinine, estimated glomerular filtration rate (eGFR) and plasma NGAL levels were measured on admission and 72 h later. Seventeen (56.6%) patients developed WRF within the three-day follow-up period. At presentation, plasma NGAL level was significantly elevated in children who developed WRF. Admission plasma NGAL level correlated with renal parameters (BUN, creatinine and eGFR) as well as with left ventricular systolic parameters (ejection fraction and fractional shortening). For prediction of WRF, admission plasma, NGAL level>27.5 µg/L had sensitivity and specificity of 90% and 68%, respectively. The area under the receiver-operator curve was higher for NGAL (0.869) than for BUN (0.569) or eGFR (0.684). We conclude that admission plasma NGAL level can predict WRF in children hospitalized for AHF.

Pediatric systemic lupus erythematosus in a single nephrology unit.

Clinical manifestations of systemic lupus erythematosus (SLE) are widely variable, and its course is unpredictable. SLE that begins in childhood has been considered more severe than SLE with onset during adulthood. Our aim was to determine the presentation and the outcome of SLE of 26 children (20 females and 6 males, with a female to male ratio of 3.8:1) with SLE in our center, their ages ranging from 5 - 18 years and followed from 2005 till October 2011. They were diagnosed according to the American Rheumatism Association's revised criteria. Complete blood count, erythrocyte sedimentation rate, C3, urine analysis, 24-h urinary protein, antinuclear antibodies, anti-ds DNA and renal biopsy were obtained for the patients. We found that the most extra-renal manifestation of SLE was fever (57.7%), while lupus nephritis (LN) was the most commonly affected organ (50%). Hemolytic anemia was the most common hematological abnormality (80.8%), while immunological characteristics were positive in all the patients. Remission in patients without LN was more than 5.3-times the remission in LN patients. The outcome of the patients without LN was better than the patients with LN.

Detection of early renal injury in children with solid tumors undergoing chemotherapy by urinary neutrophil gelatinase-associated lipocalin.

Acute kidney injury (AKI) is a complication in children with solid tumors undergoing chemotherapy, as it may prevent the use of therapy protocols and also hinder the supportive and diagnostic procedures. Thus, there is an urgent requirement for early predictive biomarkers of AKI. The most promising novel AKI biomarker is neutrophil gelatinase-associated lipocalin (NGAL). The aim of the present study was to compare the predictability of NGAL as a biomarker of AKI with creatinine as a traditional biomarker in children with solid tumors under chemotherapy. The study was performed on 30 patients with different types of solid tumors (reuroblastoma, Wilms tumor, medulloblastoma, rhabdomyosarcoma and Ewing sarcoma) and 20 control subjects. Urinary NGAL (uNGAL) and serum creatinine samples were taken three times: Baseline before the beginning of the treatment, one week after chemotherapy and at the end of the chemotherapy protocol. AKI is defined as a change in creatinine level by >50% of the baseline. The creatinine level only rises to this level in the third sample, while uNGAL increases significantly in the second and third samples with percentage of change 376.8 and 698.2%, respectively, which is highly significant (P<0.001). When comparing the predictive value of serum creatinine for AKI depending on the receiver operating characteristic curve with that of uNGAL, the area under the curve (AUC) for creatinine was 0.60 with a standard error (SE) of 0.086 and 95% confidence interval (CI) between 0.432 and 0.768, while that of uNGAL was highly predictive with an AUC of 0.847, SE 0.55 and 95% CI between 0.739 and 0.955. Depending only on the creatinine level for detecting the AKI will markedly delay the diagnosis; however, uNGAL is detected earlier, and is easier and more reliable as a marker for AKI in children with solid tumors undergoing chemotherapy.

Evaluation of different therapeutic approaches for spontaneous bacterial peritonitis.

BACKGROUND AND STUDY AIMS: Spontaneous bacterial peritonitis (SBP) is a significant cause of mortality in cirrhosis. Reducing toxic burden of infected ascitic fluid through paracentesis needs further studies as adjunctive therapy of SBP. We aimed to evaluate different therapies for SBP. PATIENTS AND METHODS: Thirty-six cirrhotic ascitic patients with SBP were examined and classified according to treatment modality (5-7 days) into: Group A received cefotaxime, group B received cefotaxime and albumin 1.5 g/kg body weight within 6h of SBP being diagnosed and 1g/kg body weight on day 3, group C received cefotaxime and paracentesis with volume dependent albumin infusion. Control group of 12 cirrhotic ascitic patients free from SBP were included. Routine laboratory tests, ascitic fluid analysis for leucocytes and culture were done, inflammatory mediators such as nitric oxide and tumour necrosis factor alpha were measured in serum and ascitic fluid. Duplex-Doppler assessment of portal flow volume and renal resistive index, Echocardiography to measure end diastolic and end systolic volumes, stroke volume and cardiac output were done. Tests were carried out before and after therapy. RESULTS: Treatment response was assessed by, cardiac haemodynamics, portal and renal flow and NO and TNF. All studied parameters; laboratory, cardiac, Doppler exhibited a significant improvement in group B in contrast to the other groups as demonstrated by post therapy reduction of (blood and ascitic fluid WBCs & PNLS, serum and ascitic NO & TNF and renal resistive index), elevation of (serum albumin and portal flow volume) and improvement of cardiac haemodynamic. CONCLUSION: Treatment of spontaneous bacterial peritonitis by cefotaxime and body weight based albumin infusion gave most favourable results compared to other regimens. Postulation of removing toxic burden through paracentesis has not been confirmed.

Prevalence of cytomegalovirus (CMV) infection among neonatal intensive care unit (NICU) and healthcare workers.

CMV is the most common cause of congenital and perinatal infection, most infections are asymptomatic at birth but later on develop handicaps, mainly neurological disturbances. The aim of the present work is to study the prevalence of CMV infection in NICU, to detect possible nosocomial transmission of CMV infection and determine possible risk factors for neonatal CMV infection. This study was carried on 175 neonates in NICU and 19 employees in the same unit. All members of the study were investigated for serum CMV-IgG and IgM by ELISA and CMV - DNA by PCR. The overall prevalence of CMV was 12.57%, 10 (5.71%) had congenital infection, while 12 cases (6.86%) had perinatal infection. In neonates with congenital CMV infection, the prevalence of breast milk feeding, congenital anomalies and blood transfusion were 80%, 30% and 60%, respectively. In neonates with perinatal CMV infection the prevalence of breast milk feeding, congenital anomalies and blood transfusion were 75%, 16.67% and 50%, respectively. On the other hand from the 19 employees, 2 (10.53%) were CMV-DNA positive by PCR, none of them was CMV-lgM positive and all of them were CMV-IgG positive. The risk factors related to CMV infection among neonates in NICU were, low birth weight, congenital anomalies and breast milk feeding, while CMV infection among employee was related to blood transfusion and employment period. In our results there was no statistical correlation between neonates in NICU and employee in the same unit. CMV infections are of more prevalence in premature and low birth weight neonates in NICU. No evidence of nosocomial CMV transmission to employee in NICU.