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   Table of Contents - Current issue
September-December 2019
Volume 32 | Issue 3
Page Nos. 101-144

Online since Monday, April 27, 2020

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Clinical and radiological predictors of the outcome of hydrostatic reduction of primary intussusception in childhood p. 101
Ahmed Elrouby, Saber Waheeb, Ashraf Ettaby, Ahmed Elabany
Introduction Success of hydrostatic reduction of intussusception using saline enema could be affected by several factors. Aim This study aimed to detect the clinical and radiological predictors of successful hydrostatic reduction of primary intussusception. Patients and methods This retrospective cross-sectional study included all patients with primary intussusception from the age of 6 months to 3 years. Patients with signs of peritonitis or bowel perforation were excluded from the study. Patients’ age, sex, duration and type of symptoms, general, abdominal, and digital rectal examination findings as well as findings in ultrasound of the abdomen (the presence and the site of an abdominal mass) and plain radiograph (PXR) of the abdomen standing (air fluid levels) were recorded and analyzed by appropriate statistical tools with the software SPSS version 10.0. Results Factors that affected the success of hydrostatic reduction significantly included age at presentation (Student’s t test: 3.46, P=0.001), BMI (Student’s t test: 2.383, P=0.019), long duration of symptoms (Student’s t test: 8.812, P=0.000), passage of red currant jelly stools (χ2-test: 91.777, P=0.000), presence of palpable abdominal mass (χ2-test: 55.813, P=0.000), on the left side of the abdomen (χ2-test: 52.439, P=0.000), and positive findings in PXR abdomen standing (χ2-test: 59.911, P=0.000). Conclusion Primary intussusception in patients with younger age group, low BMI, long duration of symptoms, passage of red currant jelly stool, palpable abdominal mass on the left abdominal side being confirmed by ultrasound of the abdomen, and the presence of air fluid levels in PXR abdomen standing have a lower rate of successful hydrostatic reduction.
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Effects of two dosing cohorts of dexmedetomidine as a primary sedative in critically ill infants with respiratory failure p. 107
Jehan A Sayed, Mohamed A.F Riad, Hala S Abdel-Ghaffar
Background Despite being increasingly used, prospective randomized dose-finding studies on dexmedetomidine (DEX) sedation in infants are deficient. Aim The aim was to compare the efficacy of two fixed dosing cohorts of DEX as a primary sedative in the pediatric ICU. Patients and methods Thirty-seven pediatric ICU mechanically ventilated infants (1–12 months) with acute respiratory failure were randomly allocated to receive DEX infusion without a loading dose: 0.5 µg/kg/h (group I, intermediate dose, n=18) or 0.35 µg/kg/h (group II, low dose, n=19) up to 72 h. The primary outcome was University of Michigan Sedation Scale (UMSS). Secondary outcomes were supplemental sedation or analgesia, hemodynamics, withdrawal adverse effects, withdrawal assessment tool-version 1, and ICU and length of hospital stay. Results Duration of DEX infusion was (48.8±21.7 vs 37.8±21.3 h) in groups I and II (P=0.127). UMSS scores were significantly lower in group II. Supplemental sedation was needed in two patients in group II. Total ICU and hospital length of stay were 6.2±1.4 vs 3.6±1.9 days, P<0.000 and 9.9±2.2 vs 6.58±2.6 day, P=0.000, in groups I and II. Withdrawal effects in 12 h after DEX discontinuation were hypertension (1 vs1), tachycardia (1 vs 1), and agitation (1 vs 10) in groups I and II. The withdrawal assessment tool-version 1 score in 12th was (0.28±0.1 vs 0.89±0.2, P<0.02) and 24th (0.28±0.1 vs 0.79±0.2, P<0.02) in groups I and II. Conclusion Two doses produced adequate sedation with comparable opioid sparing. The low dose was associated with lower UMSS scores, more agitation following DEX discontinuation, and lower ICU and length of hospital stay.
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Study of the effect of different iron-chelating agents on early renal glomerular and tubular function markers in children with beta-thalassemia p. 116
Maha Y Kamal Zeid, Hoda M.A Hassab, Doreen N Younan, Omneya Arab
Background Advances in the management of patients of beta-thalassemia major (BTM) and the advent of effective chelators have led to the discovery of many renal complications. Mechanisms of renal impairment in BTM are still not fully investigated. Chronic anemia and hypoxia, iron overload and its complications, and direct nephrotoxic effects of some chelators are the commonly used explanations. Objectives In this cross-sectional study, the aim was to investigate the prevalence of renal impairment in patients with BTM using some of both conventional and early markers of glomerular and tubular renal function and to investigate the relation between them and iron overload (s. ferritin) and the use of a specific chelator. Patients and methods The study included 60 transfusion-dependent patients with BTM without overt renal disease or risk factors, for example, diabetes mellitus, and 30 age-matched and sex-matched healthy children as a control group (group C). Patients were divided into two equal groups according to type of chelator: group A children receiving standard deferoxamine (DFO) and/or deferiprone and group B children receiving deferasirox (new treatment). Serum levels of ferritin, creatinine, and blood urea nitrogen; albumin/creatinine ratio (ACR) in urine; estimated glomerular filtration rate using Schwartz formula; urinary beta 2 microglobulin (UB2MG); and urinary calcium/creatinine ratio were measured in all patients and healthy control children. Results Serum creatinine was within the normal range in most of the patients, and no significant differences were found between the two thalassemic groups and the control group (P=0.473). ACR in urine and UB2MG were significantly higher in the two thalassemic groups, and the deferasirox group showed significantly higher levels than both DFO and/or deferiprone group and control group (P1=0.05 and P2=0.005, respectively, for ACR and P1=0.025 and P2=0.000, respectively, for UB2MG). UB2MG was abnormally high in 67 and 76% of the DFO and/or deferiprone group and deferasirox group, respectively, followed by microalbuminuria (16 and 30%, respectively). Glomerular hyperfiltration was prevalent, but there were no significant differences in the median of the three groups (P=0.766). Hypercalciuria was also prevalent in the two thalassemic groups with abnormally elevated urinary calcium/creatinine ratio in 30% of both groups but without significant differences in the median of the three groups (P=0.123). Serum ferritin was significantly positively correlated with ACR (P=0.002) and with UB2MG (P=0.000). Conclusions ACR can be used as an early marker of glomerular function. Deferasirox use is probably safe in the usual doses in low-risk patients, keeping into consideration that high doses of deferasirox cause disruption of the renal function.
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Hepatic expression of integrin β-8 protein as a risk factor in infants with biliary atresia p. 124
Mohammed A Khedr, Nermin M Adawy, Behairy E Behairy, Nermine A Ehsan, Ahmed El-Refaei, Fatma E.-Z.S Abou-Shaaban
Introduction Biliary atresia (BA) is a progressive obliteration of the extrahepatic bile ducts that affects as many as 1 in 5000 children in certain areas of the world. Aim The aim was to study the expression of integrin β-8 in patients with BA and compare it with patients with non-BA cholestasis. Patients and methods This study was conducted on 30 infants with BA and 30 infants with cholestasis other than BA. Integrin β-8 immunohistochemical (IHC) staining was done to all studied groups. Results In this study, infants with BA had statistically significant more integrin β-8 expression than non-BA patients (P=0.001) with significantly higher expression at IHC score 3 (P<0.0001). While non-BA infants had significantly higher expression at low IHC score 1 (P<0.0001) integrin β-8 IHC score at a cutoff value greater than 2 could discriminate between BA and non-BA groups that had 0.729 area under the receiver-operating characteristic curve with 83.3% sensitivity, 60% specificity, and 71.6% accuracy. Conclusion Integrin β-8 expression is significantly more expressed in patients with BA than non-BA patients and can significantly discriminate BA at an IHC score of greater than 2 from other causes of neonatal cholestasis.
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Apparent life-threatening events in infants: a single-center experience p. 131
Mona A.L Alsayed, Ahmed M El Refaey, Shaimaa G Othman, Mohammed M Abo Elkheir
Background Apparent life-threatening events (ALTEs) occur with diverse underlying medical conditions. Objective We aimed to assess the clinico-epidemiological characteristics of ALTEs in our locality in the Delta region located in Northern Egypt. Patients and methods A prospective cohort study included 53 patients younger than 1 year, who presented with ALTEs in emergency department of Mansoura University Children’s Hospital. All patients were subjected to full history taking, physical examination, and basic laboratory panel. Further investigations were individualized. All patients had 1-year follow-up for recurrence and outcome. Results The study included 53 patients, comprising 28 (52.8%) females, and the median age was 6 weeks (range, 2 days–11 months). Thirty-nine (73.6%) patients were defined as high-risk age group (below 2 months). Most of the patients had normal vital signs and stable medical condition at the time of their examination in the emergency department (40 cases, 75.5%). Recurrent ALTE was observed in 35 (66%) patients. Cardiac arrest occurred in 20.8% of patients, and eight of them died. Multivariate analysis revealed a significant effect of the cardiac arrest (odds ratio, 24; 95% confidence interval, 3.8–152.2; P=0.001) and abnormal physical examination (odds ratio, 15.5; 95% confidence interval, 1.74–138.2; P=0.014) on the number of deaths. Conclusion ALTE in our locality turned out not to be a rare condition. Its recognition and appreciation of the abnormal symptoms reported by the parents are important to promptly define any underlying etiology. Structured history and examination with tailored investigations have a high diagnostic yield.
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A cross-sectional study of complications in children with glycogen storage disease: a single-center study p. 136
Mohammed M Abd El-Razzak, Khadiga M Ali, Mohammad S Al-Haggar, Mona A.L Alsayed
Background Glycogen storage diseases (GSDs) are a heterogeneous group of metabolic disorders that result from the deficiency of particular enzymes implicated in glycogen metabolism. Identification of the exact diagnosis is essential, as there are different treatments, complications, and natural histories for the various kinds of this disorder. However, with the lack of molecular and enzymatic assay owing to unaffordable cost, we aimed to investigate children diagnosed with GSD for detection of disease-related complications. Patients and methods This cross-sectional observational study included 43 children diagnosed as having GSD based on liver biopsy, with emphasis on the possible associated complications despite the lack of molecular subtyping. Patients were selected from the outpatient clinic of Hepatology Unit of Mansoura University Children’s Hospital during the period from February 2016 to December 2018. Results A total of 27 (62.8%) patients were males. Their age ranged from 1 to 18 years and median value was 7 years. Positive consanguinity was present in 26 (60.5%) cases. Overall, 46% showed delayed puberty. Thirty-five children developed complications in the form of type I diabetes mellitus in one (2.3%), chronic kidney disease in two (4.7%), osteopenia in 24 (68.5%), delayed motor development in 21 (49%), and cardiomyopathy in five (11.6%). Conclusion Development of complications in pediatric patient with GSD can occur even with the strict diet control. Anticipation of complications and regular checkup of their occurrence is mandatory to prevent their progression and allow prompt management.
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