Italian Journal of Pediatrics - Latest Articles

Mandibular distraction in neonates: indications, technique, results

Enrico Sesenna — 2012-02-02T00:00:00Z

Background: The Pierre Robin Sequence features were first described by Robin in 1923 and include micrognathia, glossoptosis and respiratory distress with an incidence estimated as 1:8,500 to 1:20,000 newborns. Upper airway obstruction and feeding difficulties are the main concerns related to the pathology. Mandibular distraction should be considered a treatment option (when other treatments result inadequate).Patiants and methods. Ten patients between the ages of 1 month and 2 years with severe micrognathia and airway obstruction were treated with Mandibular Distraction Osteogenesis (MDO).All patients underwent fibroscopic examination of the upper airway and a radiographic imaging and/or computed tomography scans to detect malformations and to confirm that the obstruction was caused by posterior tongue displacement. All patients were evaluated by a multidisciplinary team. Indications for surgery included frequent apneic episodes with severe desaturation (70%). Gavage therapy was employed in all patients since oral feeding was not possible. The two tracheotomy patients were 5 months and 2 years old respectively, and the distraction procedure was performed to remove the tracheotomy tube. All patients were treated with bilateral mandibular distraction: two cases with an external multivector distraction device, six cases with an internal non-resorbable device and two cases with an internal resorbable device. In one case, the patient with Goldenhar's Syndrome, the procedure was repeated. Results: The resolution of symptoms was obtained in all patients, and, when present, tracheotomy was removed without complications. Of the two patients with pre-existing tracheotomies, in the younger patient (5 months old) the tracheotomy was removed 7 days postoperatively. In the Goldenhar's syndrome case (2 years old) a Montgomery device was necessary for 6 months due to the presence of tracheotomy-inducted tracheomalacia. Patients were discharged when the endpoint was obtained: symptoms and signs of airway obstruction were resolved, PAS and maxillomandibular relationship improved, and tracheotomy, when present, removed. During the follow-up, no injury to the inferior alveolar nerve was noted and scarring was significant in only the two cases treated with external devices. Conclusion: Mandibular Distraction Osteogenesis is a good solution in solving respiratory distress when other procedures are failed in paediatric patients with severe micrognatia.

Development and validation of serum bilirubin nomogram to predict the absence of risk for severe hyperbilirubinaemia before discharge: a prospective, multicenter study

Costantino Romagnoli — 2012-02-01T00:00:00Z

ObjectiveTo elaborate a percentile-based hour specific total serum bilirubin (TSB) nomogram and to assess its ability to predict the absence of subsequent non physiologic severe hyperbilirubinaemia before discharge. Methods: A percentile-based hour-specific nomogram for TSB values was performed using TSB data of 1708 healthy full term neonates. The nomogram's predictive ability was then prospectively assessed in five different first level neonatal units, using a single TSB value determined before discharge. Results: The 75 th percentile of hour specific TSB nomogram allows to predict newborn babies without significant hyperbilirubinemia after 48 to 72 hours of life. In the first 48 hours of life the observation of false negative results did not permit a safe discharge from the hospital. Conclusion: The hour-specific TSB nomogram is able to predict all neonates without risk of non physiologic hyperbilirubinemia only after 48 to 72 hours of life. The combination of TSB determination and risk factors for hyperbilirubinemia could facilitate a safe discharge from the hospital and a targeted intervention and follow-up.

De-hospitalization of the pediatric day surgery by means of a freestanding surgery center: pilot study in the Lazio region

Giovanni Mangia — 2012-02-01T00:00:00Z

Background: Day surgery should take place in appropriate organizational settings. In the presence of high volumes, the organizational models of the Lazio Region are represented by either Day Surgery Units within continuous-cycle hospitals or traditional Day Surgery Centers. This pilot study presents the regional volumes provided in 2010 and the additional volumes that could be provided based on the best performance criterion with a view to suggesting the setting up of a regional Freestanding Center of Pediatric Day Surgery. Methods: This is an observational retrospective study. The activity volumes have been assessed by means of a DRG (Diagnosis Related Group)-specific indicator that measures the ratio of outpatients to the total number of treated patients (freestanding indicator, FI). The selected DRGs had an FI exceeding the 3rd quartile present in at least a health-care facility and a volume exceeding 0.5% of the total patients of the pediatric surgery and urology facilities of the Lazio Region. The relevant data have been provided by the Public Health Agency and relate to 2010. The best performance FI has been used to calculate the theoretical volume of transferability of the remaining facilities into freestanding surgery centers. Patients under six months of age and DRGs common to other disciplines have been excluded. The Chi Square test has been used to compare the FI of the health-care facilities and the FI of the places of origin of the patients. Results: The DRG provided in 2010 amounted to a total of 5768 belonging to 121 types of procedures. The criteria of inclusion and exclusion have led to the selection of seven final DRG categories of minor surgery amounting to 3522 cases. Out of this total number, there were 2828 outpatients and 694 inpatients. The application of the best performance determines a potential transfer of 497 cases. The total outpatient volume is 57%. The Chi Square test has pointed to a statistically significant difference of the facilities and to a non-significant difference of inferiority of the regional places of origin with respect to the city of Rome. Conclusions: The activity volumes would seem to support the setting up of a Freestanding Regional Center of Pediatric Day Surgery. This Center represents the healthcare facility that is most likely to allow a de-hospitalization process. Subsequent studies will be required to confirm the validity of this pilot study.

Obesity in patients with Acute Lymphoblastic Leukemia in childhood

Lorenzo Iughetti — 2012-01-27T00:00:00Z

Acute lymphoblastic leukemia is the most common malignancy in childhood. Continuous progress in risk-adapted treatment for childhood acute lymphoblastic leukemia has secured 5-year event-free survival rates of approximately 80% and 8-year survival rates approaching 90%. Almost 75% of survivors, however, have a chronic health condition negatively impacting on cardiovascular morbidity and mortality. Obesity can be considered one of the most important health chronic conditions in the general population, with an increasing incidence in patients treated for childhood cancers and especially in acute lymphoblastic leukemia survivors who are, at the same time, more at risk of experiencing precocious cardiovascular and metabolic co-morbidities. The hypothalamic-pituitary axis damage secondary to cancer therapies (cranial irradiation and chemotherapy) or to primary tumor together with lifestyle modifications and genetic factors could affect long-term outcomes. Nevertheless, the etiology of obesity in acute lymphoblastic leukemia is not yet fully understood. The present review has the aim of summarizing the published data and examining the most accepted mechanisms and main predisposing factors related to weight gain in this particular population.

Abilities of preschoolers: comparing different tools

Matteo Chiappedi — 2012-01-26T00:00:00Z

Background: There is a strong need for studies evaluating tests in terms both of psychometric properties (i.e. their efficacy or ability to be helpful in reaching a diagnosis) and of their cost-effectiveness (i.e. their efficiency). These data are essential for planning a correct evaluation to identify children's needs (both educational and abilitative). Methods: We evaluated 58 children attending for the first time the last year of the Scuola dell'Infanzia. Parental view was obtained with Child Behaviour Check-List and Conners' Rating Scales - Revised, and family socio-economic status was evaluated using Hollingshead's Four Factor Index; teacher compiled the IPDA questionnaire; children were administered Raven's Progressive Matrices, Modified Bell Cancellation Test, BVN 5-11 (a neuropsychological battery). Results: A correlational analysis was conducted using Spearman's Rho (since variables were not normally distributed). These asymptomatic children show a good global cognitive functioning, but also a deficit of attention and of Executive Functions. Some of the tests used seem more cost-effective than others and there are some redundancies in information obtained. Conclusions: Our data show that there are significant correlations between different neuropsychological and behavioural measures. It is therefore possible to rationalize diagnostic protocols without a significant information reduction. A deeper analysis will require a preliminary definition of the psychometric properties of used tools.

Effects of chest physiotherapy and aerobic exercise training on physical fitness in young children with cystic fibrosis.

Bulent Elbasan — 2012-01-10T00:00:00Z

Background: Cystic fibrosis is a multisystem disease where the main problems are existing in the respiratory system. Aerobic exercise programs are effective in increasing physical fitness and muscle endurance in addition to chest physiotherapy.ObjectiveThe aim of this study was to evaluate the effects of chest physiotherapy and aerobic exercise training on physical fitness in young children with cystic fibrosis. Methods: Sixteen patients with cystic fibrosis, between the ages 5-13 years, were included in this study. All children were assessed at the beginning and at the end of 6 week of the training. Modified Bruce protocol was used for assessing the cardiovascular endurance. The sit-up test was used to evaluate the dynamic endurance of abdominal muscles, standing long jump was used to test power, sit and reach, trunk lateral flexion, trunk hyperextension, trunk rotation and forward bending tests were used to assess flexibility, 20 m shuttle run test and 10-step stair climbing tests were used to assess power and agility. All patients received chest physiotherapy and aerobic training, three days a week for six weeks. Active cycle of breathing technique and aerobic exercise training program on a treadmill were applied. Results: By evaluating the results of the training, positive progressions in all parameters except 20 m shuttle run and 10 stairs climbing tests were observed (p < 0.05). Active cycle of breathing techniques were used together with exercise training in clinically stable cystic fibrosis patients increases thoracic mobility (p < 0.05) and the physical fitness parameters such as muscle endurance, strength and speed (p < 0.05). Comparison of the results in sit and reach and forward bending tests were not significant (p > 0.05). Conclusion: It is thought that in addition to medical approaches to the systems affected, the active cycle of breathing techniques along with aerobic training helps to enhance the aerobic performance, thoracic mobility and improves physical fitness in children with cystic fibrosis.

About the need to use specific population references in estimating paediatric hypertension: Sardinian blood pressure standards (age 11-14 years)

Pier Paolo Bassareo — 2012-01-10T00:00:00Z

Background: Previous Italian paediatric blood pressure (BP) tables overestimated the prevalence of hypertension in adolescents of specific geographic areas, such as Sardinia, an island in the Mediterranean Sea. This is probably due to a not very homogeneous distribution of the subjects studied, most from Middle and Northern Italy, and the long period from the survey. Methods: BPs were repeatedly measured over a period of 3 years in 839 children (52.6% males. Age range: from 11 to 14 years during this period), using a standard mercury sphygmomanometer. For each gender, the specific percentile curves of systolic and diastolic BP were constructed.Results (corrected by the 50th percentile of height)Males (11-14 years): mean systolic BP (50th centile): from 111 to 115 mmHg. Hypertensive systolic BP (>95th percentile): from 127 to 135 mmHg. Mean diastolic BP (50th centile): from 65 to 69 mmHg. Hypertensive diastolic BP (>95th percentile): from 78 to 82 mmHg.Females(11-14 years): mean systolic BP (50th centile): from 110 to 112 mmHg. Hypertensive systolic BP (>95th percentile): from 127 to 130 mmHg. Mean diastolic BP (50th centile): from 65 to 67. Hypertensive diastolic BP (>95th percentile): from 78 to 80 mmHg. Conclusions: Sardinian BP tables emphasizes the need to integrate the previous standards with more up-to-date and representative reports on Italian children, as periodically performed in the USA, in order to increase the number of subjects to be checked, and to obtain a national coverage better and more completely representative of every geographic area of our country.

Zinc, Ferritin, Magnesium and Copper in a group of Egyptian Children with Attention Deficit Hyperactivity Disorder

Magdy Mahmoud — 2011-12-29T00:00:00Z

Background: Attention deficit hyperactivity disorder is a behavioral syndrome of childhood characterized by inattention, hyperactivity and impulsivity. There were many etiological theories showed dysfunction of some brain areas that are implicated in inhibition of responses and functions of the brain. Minerals like zinc, ferritin, magnesium and copper may play a role in the pathogenesis and therefore the treatment of this disorder.ObjectiveThis study aimed to measure levels of zinc, ferritin, magnesium and copper in children with attention deficit hyperactivity disorder and comparing them to normal. Methods: This study included 58 children aged 5-15 years with attention deficit hyperactivity disorder attending Minia University Hospital from June 2008 to January 2010. They were classified into three sub-groups: sub-group I included 32 children with in-attentive type, sub-group II included 10 children with hyperactive type and sub-group III included 16 children with combined type according to the DSM-IV criteria of American Psychiatric Association, 2000. The control group included 25 apparently normal healthy children. Results: Zinc, ferritin and magnesium levels were significantly lower in children with attention deficit hyperactivity disorder than controls (p value 0.04, 0.03 and 0.02 respectively), while copper levels were not significantly different (p value 0.9). Children with inattentive type had significant lower levels of zinc and ferritin than controls (p value 0.001 and 0.01 respectively) with no significant difference between them as regards magnesium and copper levels (p value 0.4 and 0.6 respectively). Children with hyperactive type had significant lower levels of zinc, ferritin and magnesium than controls (p value 0.01, 0.02 and 0.02 respectively) with no significant difference between them as regards copper levels (p value 0.9). Children with combined type had significant lower levels of zinc and magnesium than controls (p value 0.001 and 0.004 respectively) with no significant difference between them as regards ferritin and copper levels (p value 0.7 and 0.6 respectively). Conclusions: Children with attention deficit hyperactivity disorder had lower levels of zinc, ferritin and magnesium than healthy children but had normal copper levels.

Quality of life measures in Italian Children with Atopic Dermatitis and their families

Fiorella Monti — 2011-12-22T00:00:00Z

Background: The impact of atopic dermatitis (AD) on children's quality of life (QoL) in US and European countries is relatively well known, though rarely evaluated in the Italian population. Moreover, the association between child age and QoL has not been enough investigated, even though few studies detected a worse QoL in youngest AD children. The aim of the study was to evaluate the QoL in an Italian sample of atopic children and their families, also exploring a possible association with child age. Methods: 60 AD children aged between 1-12 years and their mothers completed specific QoL questionnaires (IDQoL/CDLQI, DFI) and a clinician completed a measure of AD severity (SCORAD). Results: AD severity (Objective SCORAD) significantly correlated with QoL measures. Severe AD children showed higher IDQoL/CDLQI and DFI scores compared to mild and moderate AD groups (P = 0.006 and P < 0.0005, respectively), but only DFI scores differed in these last two conditions (P = 0.014). DFI scores negatively correlated with children's age (P = 0.046), but did not differ when considering child age ranges. Multiple linear regression analyses revealed a significant association between Objective SCORAD and QoL measures. Conclusions: A strong association between severe AD and poor QoL, both in children and mothers, was found in the Italian sample, in line with the international literature. Family's QoL scores were sensitively related to AD severity, more than the child's QoL, emphasising that the disease has a deep impact on the family. A significant association between age and QoL was only partially found and needs further investigation.

"Electro-clinical Syndromes" with onset in Paediatric Age: the highlights of the clinical-EEG, genetic and therapeutic advances

Pasquale Parisi — 2011-12-19T00:00:00Z

The genetic causes underlying epilepsy remain largely unknown, and the impact of available genetic data on the nosology of epilepsy is still limited. Thus, at present, classification of epileptic disorders should be mainly based on electroclinical features. Electro-clinical syndrome is a term used to identify a group of clinical entities showing a cluster of electro-clinical characteristics, with signs and symptoms that together define a distinctive, recognizable, clinical disorder. These often become the focus of treatment trials as well as of genetic, neuropsychological, and neuroimaging investigations. They are distinctive disorders identifiable on the basis of a typical age onset, specific EEG characteristics, seizure types, and often other features which, when taken together, permit a specific diagnosis which, in turn, often has implications for treatment, management, and prognosis. Each electro-clinical syndrome can be classified according to age at onset, cognitive and developmental antecedents and consequences, motor and sensory examinations, EEG features, provoking or triggering factors, and patterns of seizure occurrence with respect to sleep. Therefore, according to the age at onset, here we review the more frequently observed paediatric electro-clinical syndrome from their clinical-EEG, genetic and therapeutic point of views.