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Clinical and laboratory study of infants and children with Bartter syndrome attending Alexandria University Children’s Hospital (10 years experience)
Mohamed Thabet, Ola A Sharaki, Hanan M Fathy, Ahmed Z.Z Ayoub
January-April 2017, 30(1):1-10
Background Bartter syndrome (BS) is a group of inherited, salt-losing tubulopathies presenting as metabolic alkalosis with normotensive hyperreninemia and hyperaldosteronism. It is a consequence of abnormal function of the kidneys, which become unable to properly regulate the volume and composition of body fluids because of defective reabsorption of NaCl in loop of Henle. A first consequence of the tubular defect in BS is polyuria, which is responsible for particular complications of pregnancy − that is, polyhydramnios and premature delivery. Low potassium levels in the blood may result from overactivity of the renin–angiotensin II–aldosterone hormone system that is essential in controlling blood pressure. To date, at least five genes have been linked to BS, and they characterize five types of BS. Aim The aim of this work is to study the clinical and laboratory data of infants and children with BS attending Alexandria University Children’s Hospital during a 10-year period (January 2004–December 2013). Participants and methods A retrospective analysis of children diagnosed with BS attending Alexandria University Children’s Hospital during a 10-year period (January 2004–December 2013) was carried out. On the basis of the clinical, biochemical features, and ultrasonographic findings, 20 children were diagnosed with BS. Their demographic, clinical, biochemical, and hormonal profiles were analyzed. Follow-up data and outcomes were recorded. Results The majority of cases had perinatal history of polyhydraminos. All cases had high urinary chloride in spot urine analysis. The mainstay of treatment in BS is potassium supplementation and indomethacin. Correction of hypokalemia and hyperprostaglandinemia are mandatory for improving the symptoms (vomiting, polyuria) and chronic sequalae such as FTT and growth retardation. Conclusions According to this study, BS should be suspected in any child with a history of failure to thrive and metabolic alkalosis. Early diagnosis and treatment with NSAIDs are life-saving.
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A comparative study of the efficacy of using piracetam with iron therapy versus iron therapy alone in children with breath-holding spells
Bayoumi A Ghareib, Mona Khalil, Dina M Gaber
January-April 2017, 30(1):26-31
Background Breath-holding spells (BHS) are apparently frightening events occurring in otherwise healthy children. Generally, no medical treatment is recommended and parental reassurance is believed to be enough; however, in some cases BHS can be very stressful for the parents and a pharmacological agent may be desired in some of these children. Objective The aim of this work was to determine the efficacy of using piracetam with iron therapy versus iron therapy alone in children with BHS associated with iron-deficiency anemia. Study design The study design was an interventional randomized controlled one. Patients and methods This study was conducted at the Neuro-Pediatric Outpatient Clinic at Alexandria University Children’s Hospital, Egypt. A total number of 70 children between 6 months and 5 years of age with a diagnosis of BHS and iron-deficiency anemia reporting to the clinic were randomly divided into two groups: group I included 35 children who were given only iron therapy at a dose of 6 mg/kg/day, and group II included 35 children who were given iron therapy at the same dose and piracetam at a dose of 40 mg/kg/day. Follow-up of the cases monthly for 3 months was scheduled to evaluate the frequency of the attacks and the improvement in iron deficiency by evaluating iron profile after 3 months (iron, ferritin, and total iron binding capacity). Results Seventy patients were enrolled in this study (42 boys and 28 girls) and completed the study. The ratio of boys to girls was 3 : 2. The patients’ ages ranged between 6 months and 5 years at the time of presentation with 50% above 2 years of age. The frequency of spells varied widely, ranging from 1 to 3 per day to 3 per month, and the mean frequency was six episodes of BHS in 1 month (about 2/week) for both groups. On comparing the improvement in the frequency of attacks between the two studied groups after treatment, there was a significant difference (P=0.001) between the two groups; the median number of attacks was two attacks per month for group I and one attack per month for group II. Conclusion Treatment with piracetam in addition to iron therapy is better than iron therapy alone in reducing the frequency of breath-holding attacks in children with BHS associated with iron-deficiency anemia.
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Prevalence and etiology of communication disorders in children attending Alexandria University Children’s Hospital, Egypt
Bayoumi A Gharib, Manal M El Banna, Mona Khalil, Mai M Abou Heikal
January-April 2017, 30(1):17-25
Background Communication development in children usually follows a fairly predictable pattern, paralleling general cognitive development. Any problem in communication is likely to have a significant effect on a child’s social and academic skills and behavior. Objective The aim of this study was to estimate the prevalence and determine the possible etiologies of communication disorders in children attending the Outpatient Clinic of Alexandria University Children’s Hospital. Patients and methods Children attending the Outpatient Clinic of Alexandria University Children’s Hospital during a period of 6 months within the age range of 3–6 years were included in the study. A noninterventional descriptive cross-sectional hospital-based study was used. A structured questionnaire was used to identify communication complaints and their types from the parents’ perspectives besides their sociodemographic data. Children with positive complaints were evaluated using National Health Services-Lothian Guidelines for Referral to Speech and Language Therapy. The studied children underwent neurodevelopmental assessment and physical examination to identify possible etiologies of communication disorders and their distribution. Results In the studied sample of 280 children, the prevalence of significant communication complaints was 23.2% and the prevalence of confirmed communication disorders was 10%. The total prevalence of confirmed delayed language development (DLD) in the studied sample was 6.4%; 1.8% of the had specific language impairment, 1.4% had environmental DLD, 1.4% had DLD due to mental retardation, 1.1% had DLD associated with attention deficit hyperactivity disorder, and 0.7% had DLD due to autism spectrum disorders. The total prevalence of confirmed speech disorders in the studied sample was 3.2%; motor and speech-sound defects constituted 2.1% of the studied sample, 0.7% had dysfluency, and 0.4% had postadenoidectomy hypernasality. The total prevalence of confirmed voice disorders in the studied sample was 0.4%, with vocal polyp. Moreover, the study showed that sex, history of perinatal/postnatal events, and presence of medical health problems had significant effects on children’s communication development. Conclusion Communication disorders are common in pediatric patients. Sex, history of perinatal events, and presence of significant comorbid medical conditions are significant risk factors for developing communication disorders. Parents’ awareness of communication development is poor, and pediatricians need to be more aware of communication development assessment, management plan of communication disorders, and referral indications.
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Role of brain-type natriuretic peptide in rapid diagnosis and prognosis of persistent pulmonary hypertension of the newborn
Mohamed T Abdel latif, Bahaa S Hammad, Neveen L Mikhael, Mary E Elmirahem
January-April 2017, 30(1):11-16
Objective The diagnosis of persistent pulmonary hypertension of the newborn (PPHN) can often be difficult to make, especially in a clinical setting in which pediatric echocardiography is not readily available. This study aims at investigating the value of plasma brain-type natriuretic peptide (BNP) level as a noninvasive test in the diagnosis, prognosis, and differentiation of PPHN from other respiratory diseases in newborns with respiratory distress (RD). Patients and methods We used a prospective cohort study with three groups. One group was diagnosed with PPHN by clinical and echocardiographic criteria (PPHN group: n=20). The second group had been diagnosed with respiratory disease; however, PPHN was ruled out by having no evidence of elevated pulmonary pressure by echocardiography (RD group: n=20). The third group had no respiratory disease and was breathing room air (RA group: n=20). Plasma BNP levels were measured by Ray Bio BNP Enzyme Immunoassay Kit at study entry for all groups and upon recovery for PPHN cases. Results There was no difference between groups regarding gestational age, postnatal age, sex, birth weight, and mode of delivery. Initial BNP levels (pg/ml) were significantly elevated in the PPHN group relative to both the RA and RD groups. There was no difference in the initial plasma BNP level between the RD and RA groups. Plasma BNP levels declined significantly in the PPHN group upon recovery. Plasma BNP levels correlated with the gradient of the tricuspid regurgitation jet and the mean pulmonary artery pressure. Plasma BNP levels were higher, although insignificant, in PPHN because of underdevelopment. The mean plasma BNP level in septic babies was higher; however, this was insignificant, still the number of cases was low (two cases). Conclusion Plasma BNP levels were elevated in infants with PPHN but not in infants with other forms of RD. Follow-up plasma BNP level showed significant decline after recovery of PPHN cases. Elevated plasma BNP levels in term or near-term infants with RD should increase the suspicion of PPHN.
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Hypochromic microcytic anemia: a clincopathological cross-sectional study
Mostafa A.S Salama, Maha Y Kamal, Doren N.A Younan, Gehad A.A Henish
January-April 2017, 30(1):37-43
Background Iron-deficiency anemia, the most common cause of microcytic anemia, is a worldwide nutritional problem; its prevalence in the developing countries is three times higher than in the developed countries. It is a common disease in Egypt with a very high prevalence rate. It affects all age groups and all socioeconomic levels of the society. Identification of infants who are at risk for iron deficiency anemia (IDA) is vital as it impairs psychomotor development and growth and reduces physical activity and resistance to infection. Aim The aim of this work was to assess the prevalence of IDA among infants aged 6 months and 2 years presenting with hypochromic microcytic anemia and to determine the risk factors of iron deficiency among this age group. Participant and methods A total of 40 infants (6 months and 2 years) presenting with hypochromic microcytic anemia attending Alexandria University Children’s Hospital were included in this study. All were subjected to complete blood count, iron profile [serum ferritin (SF), serum iron, and total iron-binding capacity], and finding occult blood in stool. Those having normal iron profile were further subjected to capillary hemoglobin (Hb) electrophoresis and PCR for α-chain gene mutations to exclude β-thalassemias and α-thalassemias. Results Overall, 77.5% (31/40) of the studied infants had IDA, which represents the main cause of microcytic anemia in this study, whereas 17.5% (7/40) had β-thalassemia trait, diagnosed by increased HbA2 more than 3.5% by capillary Hb electrophoresis, and 5% (2/40) had α-thalassemia trait of the homozygous α3.7 (−α3.7/−α3.7) deletional type, detected by PCR for α-gene mutations. Moreover, this study showed that infants with IDA had significantly higher frequencies of preterm deliveries (48.38%) compared with β-thalassemia trait (14.28%), and α-thalassemia trait (0%), P=0.023, KWχ2=4.849. A significant positive correlation was found between Hb, serum iron, and SF levels of infants with IDA and both maternal Hb during pregnancy (r=0.937, P<0.001; rs=0.796, P<0.001; and rs=0.780, P<0.001, respectively) and maternal age (r=0.791, P<0.001; rs=0.749, P<0.001; and rs=0.671, P<0.001, respectively). The mean±SD weight of infants with IDA (9.91±1.61 kg) was significantly lower than that of infants having other microcytic anemias, P=0.039, KWχ2=5.659. Among infants with IDA, only five started weaning at an appropriate age, i.e., between 4 and 6 months, and their SF levels ranged between 8 and 9.4 μg/l, with a mean±SD of 8.58±1.10, which was statistically significantly higher than the SF levels among those who started weaning later (>6 months) (n=26), whose SF levels ranged between 2.0 and 5.9 μg/l, with a mean±SD of 4.04±1.10, P=0.001. Conclusion High prevalence of IDA was detected among infants presenting with microcytic hypochromic anemia (77.5%). The main risk factors for developing IDA include inadequate iron intake, preterm delivery, delayed onset of weaning, occult blood loss in stools owing to either early introduction of cow’s milk or parasitic infestations, and finally, infants born to young or anemic mothers.
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Incidence of cardiac arrhythmia in children with dilated cardiomyopathy
Ali M Abd El Mohsen, Manal A Antonios, Mohamed M Elbiomy
January-April 2017, 30(1):32-36
Background To date, no study has accurately characterized the frequency of arrhythmia in children with dilated cardiomyopathy (DCM). Recent studies have reported that children with DCM are at a higher risk for life-threatening arrhythmias than was previously appreciated. Aim This study attempted to assess the incidence of and to identify the risk for life-threatening arrhythmias in children with DCM. Patients and methods All patients with DCM who were attending the cardiology clinic in Alexandria University Children’s Hospital were enrolled in the study. All data about demographics, presenting symptoms at time of diagnosis and medications were collected. Clinical, echocardiographic and ECG parameters, and 24-h Holter records of the patients were evaluated. Results Six patients experienced arrhythmia, representing a rate of 20.7%, with more prevalence among female patients. Only one patient experienced life-threatening arrhythmia (supra-ventricular tachycardia, SVT), representing a rate of 3.4%. Significant risk factors of arrhythmias were disturbed electrolytes levels (P=0.008), abnormal diagnostic left ventricular dimensions (P=0.006) and longer QRS duration (P<0.001). Conclusion Wider-scale studies should be conducted to further evaluate the role of patient’s age, age at diagnosis of DCM and diagnostic echocardiographic variables on the incidence of arrhythmia. The high incidence of arrhythmia associated with prolonged QRS duration should undergo further investigation.
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Effect of erythropoietin as adjunctive therapy with whole-body cooling for treatment of hypoxic-ischemic encephalopathy in newborns
Mohamed M Badr-El Din, Ahmed M.S.H Abougabal, Khalid M Saad, Hanan R Abdel-Salam
May-August 2017, 30(2):45-52
Background Hypothermia induced by whole-body cooling (WBC) and/or selective head cooling reduces brain injury after hypoxia-ischemia in newborns. Despite differences in approach (head cooling vs. total body cooling), there is a general agreement that hypothermia improves outcomes of moderately asphyxiated infants, decreasing the combined outcome of death and neurologic dysfunction from 60 to 45%. Subsequently, the search for adjuvant therapies that may provide long-lasting neuroprotection was mandatory. Aim of the present work The present study aimed to evaluate the short-term effects of erythropoietin (Epo) as a neuroprotective agent in adjunction with WBC to treat newborn infants with hypoxic-ischemic encephalopathy (HIE). Participants and methods This is a true interventional randomized controlled hospital-based study. A total of 33 full-term and late preterm newborn infants, delivered at El-Shatby Alexandria University Maternity Hospital, Egypt, were enrolled in the study. They fulfilled the criteria of HIE and received WBC during the first 6 h of life, in addition to the standard intensive care measures. Of the 33 studied babies, 11 received 500 IU/kg Epo every other day for 2 weeks as adjunctive therapy to WBC. Results The Epo plus WBC group (group I) had a significantly shorter period of hospital stay in comparison with the other group managed by WBC solely (group II), [mean hospital stay in days±SD: 6.20±2.39 and 8.40±3.94, respectively (P=0.039)]. The incidence of seizures was insignificantly lower among babies in group I. Extensive white and gray matter brain lesions were observed more among group II neonates, although the difference was statistically insignificant. Clinical and neurological improvements, as guided by Thomson score, were better achieved among group I babies, despite the difference being statistically insignificant. Conclusion Epo as an adjunctive therapy with WBC for treating asphyxiated neonates is a safe mode of therapy, which significantly shortens the period of hospital stay as well as it (marginally) improves their short-term outcome, both radiologically and clinically. WBC plus Epo lowers the rate of occurrence of clinically detectable seizures in the 1st week of life among asphyxiated neonates.
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Risk factors for febrile seizures in Benghazi, Libya: a case–control study
Amenh B Yousif, Lamia M Hafez, Fatma S Benkhaial
May-August 2017, 30(2):68-73
Background Febrile seizures (FS) are the most common seizures of childhood, occurring in 2–5% of children at 6 months to 5 years of age. The risk factors for FS include developmental delay, delayed discharge from the neonatal ICU, viral infections, a family history of FS, possibly iron deficiencies, and nonexclusive breastfeeding. Aim This study was carried out to identify and quantify the risk factors for FS in Libyan children. Patients and methods A case–control study was carried out including one hundred patients with FS aged 6–60 months, admitted to Benghazi Paediatrics Hospital, Libya, who were matched with another 100 children with fever, but without seizure of the same age as the control group. The control group was enrolled randomly and the study was carried out over 8 months from 1 October 2016 to 31 May 2017. Data were collected using a pretested questionnaire. Blood samples were collected from both cases and controls and a complete blood count was performed. χ2-Test was used to assess the significance of the risk factors. Univariate and multivariate logistic regression analyses were carried out to determine the strength of associations at a 5% level of significance. Results The majority of cases and the children in the control group were between 6 months and less than 25 months of age. It was found that 93% of the cases and 87% of the children in the control group had a temperature higher than 38°C. The most common types of FS for cases were simple FS, which was recorded in 86% of cases, and complex FS, recorded in 14% of cases. A positive family history of FS was reported in 48% of cases compared with 22% of the children in the control group [odds ratio (OR)=3.27, 95% confidence interval (CI): 1.77–6.05, P<0.001]. Iron-deficiency anemia was reported in 73% of cases compared with 33% of controls, and this was found to be a strong significant risk factor for FS (OR=5.48, 95% CI: 2.99–10.07, P<0.001). Furthermore, nonexclusive breast-feeding was reported in 66% of cases compared with 32% of controls (OR=4.12, 95% CI: 2.28–7.43, P<0.001); prematurity was reported in 29% of cases compared with 11% of children in the control group (OR=3.30, 95% CI: 1.54–7.07, P<0.001). Conclusion The results of the present study indicated that iron-deficiency anemia, nonexclusive breast-feeding, and a family history of FS and prematurity were the most important risk factors for FS. This study recommends educational programs for modifiable risk factors, encourages exclusive breast feeding in the first 6 months, and early diagnosis and treatment for iron-deficiency anemia in children.
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Implementation of ventilator associated pneumonia prevention bundle in the neonatal intinsive care unit at Alexandria University Children’s Hospital, Egypt
Reem M.A Tayel, Abd El Haleem A Badr El Din, Soad F Hafez, Bahaa S Hammad
May-August 2017, 30(2):74-83
Introduction Ventilator-associated pneumonia (VAP) is a nosocomial lung infection that develops at or later than 48 h after mechanical ventilation (MV). It is the second most frequent hospital-acquired infection in neonatal intensive care units (NICUs). It results in high morbidity, mortality, prolonged NICU length of stay, and increased cost of hospitalization. Egypt and other developing countries report higher VAP rates compared with developed countries. Furthermore, studies monitoring VAP rates and success of intervention strategies in Egyptian NICUs are few. Aim The aim of the present study was to estimate the incidence of VAP during the implementation of the prevention bundle and also to identify the causative agents and significant risk factors in the NICU at Alexandria University Children’s Hospital. Patients and methods A nonrandomized clinical trial with historical control was conducted. All neonates admitted to the NICU in the period from July 2015 till March 2016, who spent more than 48 h on MV, were subjected to a VAP prevention bundle. Eligible neonates who spent more than 48 h on MV were monitored closely for VAP development. A thorough assessment of history, clinical examination, routine investigations, and chest radiography were carried out on all enrolled infants. Neonates who developed clinically suspected VAP were further subjected to nonbronchoscopic bronchoalveolar lavage for bacteriological confirmation of the clinical diagnosis. A review of records was performed to determine the incidence of VAP in the 9 months before intervention. Oral swabs were taken to study the pattern of oral colonization in ventilated neonates to trace its role in VAP development. Also, cultures of residual gastric volume and water traps inserted into the ventilator circuits were studied. Results A total of 108 episodes of VAP were diagnosed, with a cumulative incidence of clinically diagnosed VAP equal to 37.6% (34.2 VAP cases/1000 ventilation days). The incidence of bacteriological-confirmed VAP in this study was 19.97/1000 ventilation days. The most important risk factors for the occurrence of VAP were prematurity, low birth weight, prolonged duration of ventilation, re-intubations, and enteral feeding. Gram-negative bacteria were the predominating cause of VAP in the NICU and Klebsiella was the most common pathogen isolated from nonbronchoscopic bronchoalveolar lavage cultures. Conclusion VAP is a severe complication of MV as it significantly increases neonatal mortality. The VAP preventive bundle implemented in the present work was associated with a reduction in the VAP rate in the NICU.
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Psychometric properties of a translated arabic version of cerebral palsy-quality of life questionnaire: primary caregiver form
Heba M.T El-Weshahi, Mona K Mohamed, Hayam M Abd-Elghany, Tarek E.I Omar, Alaa E Azzawi
May-August 2017, 30(2):53-60
Background In the context of child rehabilitation, quality of life (QOL) is an important health-related outcome measure that has a great clinical utility as it can guide practice. Study objectives The aim of this study was to develop a translated Arabic version of primary caregiver form of cerebral palsy QOL questionnaire for children aged 4–12 years (CP QOLـChild) and to assess its validity and reliability to be readily used in Arabic and Egyptian cultures. Patients and methods The original English version of CP-QOL questionnaire was translated into Arabic language using forward–backward translation method, and then a cross-sectional survey was conducted including 200 children with cerebral palsy aged 4–12 years and their caregivers. Caregivers of children were interviewed to collect data about their demographic characteristics, age, and sex of their children. They were asked to fill the translated Arabic version of QOL questionnaire. Assessment of psychometric properties including test–retest reliability, internal consistency, and item internal consistency was conducted. Results The translated questionnaire showed excellent test–retest reliability and good internal consistency as the intraclass correlation ranges from 0.88 to 0.97 and Cronbach’s α exceeds 0.7 except for the domains of family health and emotional well-being. The majority of the items have moderate to good correlation with their domain scores. Four out of seven domains showed significant correlation with Gross Motor Function Classification System. Conclusion The translated Arabic version of CP-QOL questionnaire (caregiver form) is valid and reliable and could be used to assess quality of life of children with cerebral palsy in Arab-speaking population.
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Organizational skills training, neurofeedback, and/or pharmacotherapy in the treatment of school-aged children with attention-deficit hyperactivity disorder
Hanan G Azouz, Faten I Abdel Latif, Tarek E.S Omar, Mona Khalil, Marwa S Abdel Maksoud
May-August 2017, 30(2):61-67
Background Attention deficit hyperactivity disorder (ADHD) is the most common developmental and neurobehavioral disorder in childhood. It is characterized by hyperactivity, impulsivity, and/or inattention. Objective To study the efficacy of implementation of different interventional modalities including medication (MED; atomoxetine) either alone or in combination with behavioral therapy (organization skills training; OST) or cognitive training (neurofeedback training; NFT) in alleviation of the core symptoms of ADHD and its comorbid conduct problem among school-aged children with ADHD. Participants and methods This was a multigroup design study with preassessment and postassessment with the Arabic form of Conners’ Parent Rating Scale short form (CPRS-48). Participants were 45 school-aged children (age range from 6 to 10 years) with a diagnosis of ADHD according to the Diagnostic and Statistical Manual of Mental Disorders-5 diagnostic criteria. Children were recruited from the Pediatric Behavioral Outpatient Clinic of Alexandria University Children Hospital, Alexandria, Egypt, and they were allocated into the groups: group OST and MED (n=15); group NFT and MED (n=15); and group MED (n=15). Results Combined OST and MED showed a significant decrease in hyperactivity/impulsivity (P=0.011) and comorbid conduct problem scores (P=0.030) on postinterventional assessment with CPRS-48. However, no statistically significant difference was found among the other studied groups, groups NFT and MED or group MED, either on ADHD core symptoms or its comorbid conduct problem. Conclusion Combined OST and MED showed significant efficacy in the alleviation of ADHD core symptoms and comorbid conduct problem.
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