Mortality Rate in Mechanically Ventilated Neonates: A Developing Country Experience

Background: Although a great improvement has been achieved in the outcome of sick neonates with the effectivewidespread introduction of mechanical ventilation in neonatal intensive care units, a significant proportion of highfatality is still detected among such patients. This study was conducted to identify the complications, outcome, andcauses of death among a sample of critically ill Egyptian neonates subjected to mechanical ventilation using pressurecontrolledmode.  Methods: The statistical population of this study were prospectively enrolled in the current study and consisted of 240 eligible patients fulfilling the study inclusion criteria. Those with multiple congenital malformations or post-surgical cases were excluded from the study. The data regarding medical record, clinical examination, indication for mechanicalventilation, complications related to mechanical ventilation, and outcome were collected, recorded, and analyzed. Results: The incidence of complications related to mechanical ventilation was obtained at 104 (43.3%), and VAP observedan increase (20%). Furthermore, the complications related to disease patterns were reported to be 56.7% and 22.5% forsepsis and septic shock. It has been revealed that 124 (51.7%) of the studied neonates had favorable outcomes; however,the remaining 116 (48.3%) of the subjects passed away with a significant increase in the incidence of VAP (P=0.013). Itwas reported that smaller gestational age and lower birth weight were the most significant risk factors. Conclusion: In Egypt; as a developing country, the mortality rate among critically ill neonates undergoing mechanicalventilation with pressure-controlled mode was significantly high, approaching 48.3%, with VAP being the mostcommon cause of death

Prothrombotic risk factors and antithrombotic therapy in children with ischemic stroke

Objective: Congenital and acquired prothrombotic disorders have been highlighted in a recent series of cerebrovascular stroke (CVS), with a controversial role in pathogenesis. The aim is to study some prothrombotic risk factors [activated protein C (APC) resistance, von Willebrand factor (vWF), anticardiolpin (ACL) antibodies and plasma homocysteine] in children with ischemic stroke, and to evaluate the role of aspirin and low molecular weight heparin (LMWH) in its management in relation to outcome. Methods: A total of 37 cases aged from 1 month to 15 years ( mean ± standard deviation 26.2 ± 35.7 months), diagnosed as ischemic stroke (>24 hours) were recruited. Complete blood count, prothrombin time and concentration, partial thromboplastin time, serum electrolytes, random blood sugar, C-reactive protein, electrocardiogram and echocardiography were done. Levels of APC resistance, vWF, ACL antibodies [immunoglobulin G (IgG) and immunoglobulin M (IgM)] and plasma homocysteine were estimated. A total of 25 cases received aspirin 3–5 mg /kg/d and 12 patients received LMWH as initial dose at 75 international units (IU)/kg subcutaneously (SC) then 10–25 IU/kg/day for 15 days in a nonrandomized fashion. Results: The levels of APC resistance, vWF, ACL antibodies (IgG and IgM) and plasma homocysteine were significantly higher in stroke cases than in controls. There was no significant difference between cases treated with aspirin and those with LMWH in all prothrombotic factors. Significant positive correlations were found between vWF and ACL antibodies (IgG and IgM) levels before treatment. Significant decrease in cognitive function was detected between cases treated with LMWH and those treated with aspirin. Conclusion: Ischemic CVS in children is multifactorial. Thrombophilia testing should be performed in any child with CVS. Early use of aspirin improves the prognosis and has less effect on cognitive function

A comparative study of endoscopic ultrasonography versus endoscopic retrograde cholangiopancreatography in children with chronic liver disease

Background: Endoscopic ultrasonography (EUS) is a less invasive modality and may be equal or superior to endoscopic retrograde cholangiopancreatography (ERCP) in visualizing the biliary tree. Its role and feasibility in children need to be accurately defined. Aim: This study aimed at evaluation of EUS in assessment of children with chronic liver disease (CLD) in comparison with ERCP. Materials and Methods: The present study was carried out between September 2004 and February 2006 on 40 children suffering from CLD. Patients were selected from the Pediatric Hepatology Unit, Cairo University Children′s Hospital, Egypt. They were included if they had: sonographic (n = 8) or histopathological evidence of biliary pathology (n = 2); autoimmune hepatitis with high gamma glutammyl transpeptidase (GGT) levels and/or not responding to immunosuppressive therapy (n = 15); cryptogenic CLD (n = 13); neonatal cholestasis with relapsing or persistent course (n = 2). They all underwent EUS and ERCP. Results: Three of six cases with intrahepatic biliary radicle dilatation had Caroli′s disease by EUS and ERCP; and the other 3 had sclerosing cholangitis. EUS was equal to ERCP in diagnosis of biliary pathology. However, one false positive case was described to have dilatation and tortuosity of the pancreatic duct by EUS as compared to ERCP. EUS could detect early pancreatitis in 5 cases. One case with cryptogenic liver disease proved to have sclerosing cholangitis by both EUS and ERCP. Conclusion: EUS is an important diagnostic tool for biliary pathology and pancreatitis in children with pancreatico-biliary pathology. ERCP should be reserved for therapeutic purposes

Study of non-organ-specific antibodies in children with Genotype 4 chronic Hepatitis C

Background/Aim: Adult studies established a relationship between hepatitis C virus (HCV) infection and the presence of non-organ-specific antibodies (NOSAs). Most studies were carried out on genotypes 1 and 2. Only a few studies addressed that issue in pediatrics. No studies have been carried out on autoimmunity and genotype 4 in children. We aim to investigate NOSAs in 80 Egyptian children with chronic HCV infection along with studying the underlying genotype of HCV, and correlating autoimmunity with the epidemiological, clinical, biochemical, and virological features. Materials and Methods: HCV-RNA was assayed by the polymerase chain reaction and viral genotypes were determined. NOSAs were measured and liver biopsies were taken for histopathological examination. Results:Genotype 4 was the only detected genotype in the included 80 patients. Anti-smooth muscle antibodies (ASMA) were the only detected antibodies in 32 (40%) patients, always with V specificity (vessels only) at titers ranging from 1:20 and 1:160. Anti-nuclear antibodies (ANA) and liver-kidney microsomal antibodies-1 (LKMA-1) were not detected in any of our patients. Epidemiologic and clinical features did not significantly differ between autoantibody-positive and -negative patients. Among biochemical features, significantly high levels of total bilirubin, albumin, immunoglobulins, alkaline phosphatase, and gamma-glutamyl transpeptidase were found in the antibody-positive group. Conclusion: Genotype 4 HCV is the prevailing genotype in Egyptian children with chronic HCV infection. A consistent proportion of these children with chronic HCV infection circulate non-organ-specific autoantibodies. The prevalence of ASMA and the absence of ANA and LKMA-1 might be related to the unique situation in Egypt with unique prevalence of genotype 4. More studies are warranted on larger pediatric population to validate these findings

Sjogren–Larsson Syndrome: A case series of five members from an extended family with a novel mutation

Abstract Backgroundd Sjogren–Larsson syndrome (SLS) is a rare autosomal recessive disorder, characterized by a triad of spastic tetraplegia or diplegia, congenital ichthyosis, and intellectual disability. Methods We report a seven‐years‐old female born to consanguineous parents who presented with erythematous dry scaly skin all over the body sparing the face, without collodion membrane which started since birth. There were associated with global developmental delay and seizure disorder. SLS was suspected and hence sequence analysis of the ALDH3A2 gene by next‐generation sequencing was performed for the patient. Results A novel nucleotide exchange in homozygous state at position c.1320 in exon 9 of the ALDH3A2 gene (c.1320T>A), leading to a stop of the protein sequence (p.Tyr440) was detected in the patient. Genetic testing of the patient's extended family revealed another four affected family members with the same mutation. Conclusions SLS should be suspected in any patient with a triad of ichthyosis, intellectual disability and spastic di/tetraplegia. Molecular genetic testing of the ALDH3A2 gene should be performed to confirm the diagnosis. Extended family screening is highly recommended

Outpatient Blind Percutaneous Liver Biopsy in Infants and Children: Is it Safe?

Background/Aim: We aim to investigate the safety of outpatient blind percutaneous liver biopsy (BPLB) in infants and children with chronic liver disease (CLD). Patients and Methods: BPLB was performed as an outpatient procedure using the aspiration Menghini technique in 80 infants and children, aged 2 months to 14 yrs, for diagnosis of their CLD. Patients were divided into three groups: Group 1 (6 hrs due to oversedation in 2.5%. There was a statistically significant rise in the 1-hr post-biopsy mean heart and respiratory rates, but the rise was non-significant at 6 and 24 hrs except for group 2 where heart rate and respiratory rates significantly dropped at 24 hrs. No statistically significant difference was noted between the mean pre-biopsy and the 1, 6, and 24-hrs post-biopsy values of blood pressure in all groups. The 24-hrs post-biopsy mean hemoglobin and hematocrit showed a significant decrease, while the 24-hrs post-biopsy mean total leucocyte and platelet counts showed non-significant changes. The 24-hrs post-biopsy mean liver enzymes were non-significantly changed except the 24-hrs post-biopsy mean PT which was found to be significantly prolonged, for a yet unknown reason(s). Conclusions: Outpatient BPLB performed by the Menghini technique is safe and well tolerated even in infants and young children. Frequent, close monitoring of patients is strongly recommended to achieve optimal patient safety and avoid potential complications

Knowledge of Neonatal Hyperbilirubinemia Among Primary Health Care Physicians: A Single-Center Experience

Background and objectives: To evaluate the knowledge of the primary health care physicians (PHCP) in Kalubia governorate, Egypt, about the causes, diagnosis, complications, and treatment of neonatal hyperbilirubinemia (NHB). Methods: Cross-sectional survey distributed by interview to 500 physicians working in the primary health care (PHC) sector in Kalubia. Results: Out of 500 distributed surveys, 419 (84%) PHCP completed the questionnaire. They represent 174 (90%) out of 193 PHC units and centers. About 18% were males and 82% females with mean age of 28.5 ± 5.2 years, and mean duration of work was 3.3 ± 4.4 years. All of the respondents have patients with NHB in their daily practice. The knowledge of the PHCP was good in some aspects about NHB; however, it was poor and may be even hazardous in other aspects. Conclusions: Many areas of defects are detected in PHCP knowledge about NHB. Pre-service and continuous training of the PHCP about the diagnosis and management of NHB are essential

NT-proBNP as Early Marker of Subclinical Late Cardiotoxicity after Doxorubicin Therapy and Mediastinal Irradiation in Childhood Cancer Survivors

Background. Childhood cancer survivors treated with anthracyclines and mediastinal irradiation are at risk for late onset cardiotoxicity. Aims of the Study. To assess the role of N-terminal pro-brain natriuretic peptide (NT-proBNP) and tissue Doppler imaging (TDI) as early predictors of late onset cardiotoxicity in asymptomatic survivors of childhood cancer treated with doxorubicin with or without mediastinal irradiation. Methods. A cross-sectional study on 58 asymptomatic survivors of childhood cancer who received doxorubicin in their treatment protocols and 32 asymptomatic Hodgkin’s lymphoma survivors who received anthracycline and mediastinal irradiation. Levels of NT-proBNP, TDI, and conventional echocardiography were determined. Results. Thirty percent of survivors had abnormal NT-proBNP levels. It was significantly related to age at diagnosis, duration of follow-up, and cumulative dose of doxorubicin. TDI detected myocardial affection in 20% more than conventional echocardiography. Furthermore, abnormalities in TDI and NT-pro-BNP levels were more common in Hodgkin lymphoma survivors receiving both chemotherapy and radiotherapy. Conclusions. TDI could detect early cardiac dysfunction even in those with normal conventional echocardiography. Measurement of NT-proBNP represents an interesting strategy for detecting subclinical cardiotoxicity. We recommend prospective and multicenter studies to validate the role of NT-proBNP as an early marker for late onset doxorubicin-induced cardiotoxicity