Pediatr. Rep., Volume 5, Issue 1 (February 2013) – 6 articles

Myasthenia gravis (MG) is an autoimmune disease marked by weakness of voluntary musculature. Medical and surgical therapy of adult myasthenia is well documented. There is little pediatric surgical evidence, only a few case reports being available. The aim of this paper is to verify whether the surgical and anesthesiological techniques can warrant an early and safe discharge from the operating room. The secondary aim is to assess the presence of perioperative indicators that can eventually be used as predictors of postoperative care. During the years 2006-2009, 10 pediatric patients were treated according to a surgical approach based on video assisted thoracoscopic extended thymectomy (VATET). Standard preoperative evaluation is integrated with functional respiratory tests. Anesthetic induction was made with propofol and fentanyl/remifentanyl and maintenance was obtained with sevoflurane/desflurane/propofol ± remifentanyl. A muscle relaxant was used in only one patient. Right or left double-lumen bronchial tube (Ruesch Bronchopart® Carlens) placement was performed. Six patients were transferred directly to the surgical ward while 4 were discharged to the intensive care unit (ICU); ICU stay was no longer than 24 h. Length of hospital stay was 4.4±0.51 days. No patient was readmitted to the hospital and no surgical complications were reported. Volatile and intravenous anesthetics do not affect ventilator weaning, extubation or the postoperative course. Paralyzing agents are not totally contraindicated, especially if short-lasting agents are used with neuromuscular monitoring devices and new reversal drugs. Perioperative evaluation of the myasthenic patient is mandatory to assess the need for postoperative respiratory support and also predict timely extubation with early transfer to the surgical department. Availability of new drugs and of reversal drugs, the current practice of mini-invasive surgical techniques, and the availability of post anesthesia care units are the keys to the safety and successful prognosis of patients affected by MG who undergo thymectomy. Full article

The relationship between Helicobacter pylori (Hp) gastritis and gastroesophageal reflux disease (GERD) remains controversial. The aim was to investigate the association between Hp infection and gastroesophageal reflux (GER) and the impact of Hp eradication on esophageal acid exposure and motility in adolescents and young adults with Hp gastritis and GERD. Sixty-four patients with symptoms suggestive for GERD, of which 40 Hp-positive (group A) and 24 Hp-negative (group B), underwent endoscopy-biopsy, esophageal manometry and 24-hour pH-metry. All group A patients received eradication treatment and were re-evaluated six months later again with 24-hour pH-metry, esophageal manometry, endoscopy-biopsy and clinical assessment. At inclusion, there were no significant differences between the two groups regarding sex, age, grade of endoscopic esophagitis, manometric and pH-metry findings. All Hp-positive patients had an antral predominant gastritis. Eradication of Hp was successful in all patients, and gastritis and esophagitis were healed in all patients. The mean lower esophageal sphincter pressure (LESP) increased significantly from 11.25 mmHg before to 11.71 mmHg after eradication (P<0.05). A significant decrease in reflux index was observed (mean RI 6.02% before versus 4.96% after eradication (P<0.05). However clinical symptoms of GER improved not significantly after 6 months follow up. Conclusively, in children and young adults with GER symptoms and GERD, the presence or absence of Hp has no impact on manometric and pH-metric findings. Eradication of Hp infection results in increase in LESP with a consequent decrease in esophageal acid exposure but not significant clinical improvement. Full article

Complications in pediatric anesthesia can happen, even in our modern hospitals with the most advanced equipment and skilled anesthesiologists. It is important, albeit in a tranquil and reassuring way, to inform parents of the possibility of complications and, in general, of the anesthetic risks. This is especially imperative when speaking to the parents of children who will be operated on for minor procedures: in our experience, they tend to think that the anesthesia will be a light anesthesia without risks. Often the surgeons tell them that the operation is very simple without stressing the fact that it will be done under general anesthesia which is identical to the one we give for major operations. Different is the scenario for the parents of children who are affected by malignant neoplasms: in these cases they already know that the illness is serious. They have this tremendous burden and we choose not to add another one by discussing anesthetic risks, so we usually go along with the examination of the child without bringing up the possibility of complications, unless there is some specific problem such as a mediastinal mass. Full article

Child development may be subject to forms of motor, physical, cognitive and self-representation impairments when complex congenital heart disease (CHD) occurs. In some cases, inadequacy of both self-representation as well as the family system are displayed. It seems to be important to search the likely internal and external resources of the CHD child, and the possible connections among such resources, which may help him/her to manage his/her own risk condition. The research project inquires the possible resources related to the self-representation and self-esteem levels of the CHD child, and those related to maternal self-perception as competent mothers. A group of 25 children (mean age = 10.2; SD=1.8) suffering from specific forms of CHD, and a group made up of their relative mothers (mean age = 38.2; SD=5) were studied. The tools used were the Human Figure Drawing, to investigate child body-related self-representation; the TMA scale (Self-esteem Multidimensional Test), to investigate the child’s self-esteem; and the Q-sort questionnaire, to assess how mothers perceived their maternal competence. Data concerning the likely correlations between the child’s self-representation and the maternal role competence show [that] positive correlations between some indicators of maternal competence, specific aspects of CHD children’s self-representation (mothers’ emotional coping and children’s self-image adequacy) and self-esteem (mothers’ emotional scaffolding and children’s self-esteem at an emotional level). By detecting the occurrence of specific correlations among resources of both child and mother, the study provides cardiologists with information that is useful for building a relationship with the families concerned, which would seem to enhance the quality of the process of the cure itself. Full article

Maternal vitamin D deficiency is not uncommon. The lack of vitamin D during pregnancy may result in poor fetal growth and altered neonatal development that may persist into later life. Recognition of risk factors and early detection of vitamin D deficiency during pregnancy is important in order to prevent neonatal vitamin D deficiency and related complications. The aim of the current study is to assess the effect of maternal vitamin D status on the neonatal vitamin D stores. A total of 92 pregnant women at the end of the 3rd trimester and their newborns were recruited from Al Khafji Joint Operation Hospital, Saudi Arabia, during the year 2011. Maternal and cord blood samples were taken for determination of serum levels of circulating 25-hydroxyvitamin D₃ [25(OH)D₃] concentration, serum calcium (Ca⁺⁺), phosphorus (PO₄) and alkaline phosphatase (ALP). Compared with pregnant women with adequate vitamin D levels, women deficient in vitamin D had infants with vitamin D deficiency (X±SD 33.44±18.33 nmol/L vs 55.39±17.37 nmol/L, P=0.01). Maternal and neonatal serum 25(OH)D₃ levels showed a positive correlation with serum Ca⁺⁺ and negative correlation with serum PO₄ and ALP. Neonatal 25(OH)D was related to maternal 3rd trimester levels (r=0.89, P=0.01). The newborn serum 25(OH)D₃ concentrations rely on maternal vitamin D status. Poor maternal vitamin D status may adversely affect neonatal vitamin D status and, consequently, calcium homeostasis. Full article

Birth defects, encountered frequently by pediatricians, are important causes of childhood morbidity and mortality. Birth defects can be classified according to their severity, pathogenic mechanism, or whether they are involving a single system or multiple systems. This hospital-based prospective descriptive study highlights the prevalence of Congenital Anomalies (CAs) in one year among live born neonates delivered in Zagazig University Hospital (Egypt). All women giving birth to viable babies were included. Demographic details, associated risk factors and the type of CAs in all babies were recorded. Diagnosis of CAs was based on clinical evaluation, radiographic examination, ultrasonography, echocardiography and chromosomal analysis of the newborn whenever recommended. The overall incidence of CAs among live born neonates was 2.5%, as most of the cases were referred to Zagazig University Hospital (Egypt) for delivery. The musculoskeletal system (23%) was the most commonly involved followed by the central nervous system (20.3%). Involvement of more than one system was observed in (28.6%) cases. Among maternal and fetal risk factors; parental consanguinity, maternal under nutrition and obesity, positive history of an anomaly in the family, low birth weight, and prematurity were significantly associated with higher frequency of CAs (P<0.05), with non-significant differences for maternal age and the sex of the neonates. The current study highlights the prevalence of congenital anomalies in one year in Zagazig University Hospital. It revealed a high prevalence of congenital anomalies in our locality and stressed the importance of carrying out a thorough clinical examination of all neonates at birth. Full article