Handwriting in Children with Developmental Coordination Disorder: Is Legibility the Only Indicator of a Poor Performance?

Research on the use of speed, as an indicator of motor handwriting problems, is controversial and the legibility of the text is the measure mostly examined in children. This case-control study compared handwriting legibility and speed in 25 children with Developmental Coordination Disorder (DCD) and 75 typically developing peers matched by school grade (third and fourth grade). Children with DCD achieved significantly lower scores than their peers in both legibility and speed measures, overall and in the third and in the fourth grade independently. These measures represent a good insight into children's writing abilities and could be both used to screen and monitor.

Effects of Pidotimod on recurrent respiratory infections in children with Down syndrome: a retrospective Italian study.

BACKGROUND: Children with Down syndrome (DS) show a high susceptibility to recurrent infections (RI), caused by immune defects and abnormalities of the airways. Our goal was to investigate the effects of Pidotimod on RI prevention in children with DS, comparing immune and clinical parameters before (T0) and after (T1) the treatment with Pidotimod. METHODS: The study was conducted at the Down syndrome outpatient Center of Bambino Gesù Children's Hospital, in Rome. We reviewed the medical records of all children with a positive history for RI and who received oral prophylaxis of Pidotimod from September 2016 to February 2017. RESULTS: Thirty-three children met the inclusion criteria (males: 51.5%; average age: 6 years ±SD: 3). We found a significant decrease in the number of children with upper respiratory infections (82% at T0 vs 24% at T1; p = 0,0001) and with lower respiratory infections (36% at T0 vs 9% at T1; p = 0.003) after treatment with Pidotimod. We also demonstrated a significant decrease in the number of children hospitalized for respiratory infections (18% at T0 vs 3% at T1; p = 0.03). We measured T and B cells in the peripheral blood and B cell function in vitro at T0 and T1. We found that the response to CpG improved at T1. A significant increase of B cell frequency (p = 0.0009), B cell proliferation (p = 0.0278) and IgM secretion (p = 0.0478) were observed in children with DS after treatment. CONCLUSIONS: Our results provided evidence that Pidotimod may be able to prevent RI in children with Down syndrome.

Varicella associated pneumoniae in a pediatric population.

BACKGROUND: Varicella pneumonia has been studied extensively in adults; it may also affect children and may require hospitalization. METHODS: We examined pneumonia complications in children hospitalized for varicella, over a 13 year period. RESULTS: Pneumonia occurred in 8.2% of children hospitalized for varicella. The median length of hospitalization was 6 days. No statistically significant difference in length of stay was detected between immunodepressed children and previously healthy children. The hospitalization was on average shorter in patients who started antiviral therapy within 24 h of varicella onset. None of the included patients had been previously immunized for varicella. CONCLUSIONS: Our results support the need for increased awareness of current varicella prevention recommendations among both immunocompetent and immunodepressed individuals. In children affected by varicella, prompt antiviral therapy may be indicated to reduce the number of days of hospitalization.

Acute cerebellitis in children: an eleven year retrospective multicentric study in Italy.

BACKGROUND: Acute cerebellitis (AC) and acute cerebellar ataxia (ACA) are the principal causes of acute cerebellar dysfunction in childhood. Nevertheless. there is no accepted consensus regarding the best management of children with AC/ACA: the aim of the study is both to assess clinical, neuroimaging and electrophysiologic features of children with AC/ACA and to evaluate the correlation between clinical parameters, therapy and outcome. METHODS: A multicentric retrospective study was conducted on children ≤ 18 years old admitted to 12 Italian paediatric hospitals for AC/ACA from 01/01/2003 to 31/12/2013. A score based on both cerebellar and extracerebellar signs/symptoms was computed for each patient. One point was given for each sign/symptom reported. Severity was divided in three classes: low, moderate, severe. RESULTS: A total of 124 children were included in the study. Of these, 118 children received a final diagnosis of ACA and 6 of AC. The most characteristic finding of AC/ACA was a broad-based gait disturbance. Other common symptoms included balance disturbances, slurred speech, vomiting, headache and fever. Neurological sequelae were reported in 6 cases (5%) There was no correlation among symptoms, cerebrospinal fluid findings, clinical outcome. There was no correlation between clinical manifestations and clinical score on admission and length of hospital stay, sex, age and EEG findings with sequelae (P > 0.05). Children with pathological magnetic resonance imaging (MRI) or computed tomography (CT) had a higher probability of having clinical sequelae. Treatment was decided independently case by case. Patients with a higher clinical score on admission had a higher probability of receiving intravenous steroids. CONCLUSIONS: We confirmed the literature data about the benign course of AC/ACA in most cases but we also highlighted a considerable rate of patients with neurological sequelae (5%). Pathological MRI or CT findings at admission correlate to neurological sequelae. These findings suggest the indication to perform an instrumental evaluation in all patients with AC/ACA at admission to identify those at higher risk of neurological outcome. These patients may benefit from a more aggressive therapeutic strategy and should have a closer follow-up. Randomized controlled trials are needed to confirm these observations. The ultimate goal of these studies could be to develop a standardized protocol on AC/ACA. The MRI/CT data, associated with the clinical manifestations, may allow us to define the class risk of patients for a neurological outcome.

Lack of institutional pathways for referral: results of a survey among pediatric surgeons on prenatal consultation for congenital anomalies.

OBJECTIVE: This paper aimed to assess pathways through which pediatric surgeons receive couples for prenatal consultation after prenatal diagnosis. METHOD: A questionnaire was mailed to pediatric surgical centers to assess the following: (1) surgical caseload per year; (2) number of centers in which prenatal consultation is offered; (3) presence of a 'structured' prenatal consultation clinic; (4) number of consultations per year; (5) pathways for referral to the pediatric surgeon; and (6) the availability of psychological counseling. RESULTS: Response rate was 81%, (42/52 centers). Thirty-eight centers (93%) offered prenatal consultation. Seven centers (18%) reported to have a 'structured' clinic in terms of time and location. In 13 centers (34%), 1-9 consultations were carried out, from 10 to 19 in 18 centers (47%), from 20 to over 50 in 7 centers (18%). In 34 centers, internal referrals from the obstetric departments were counseled, and in 28 centers, there were also external referrals. Eleven centers reported that couples were self-referred. Information regarding prenatal counseling was available on the institutional website in 10/38 (26%) centers. Psychological counseling was available in 36 centers. CONCLUSION: Despite the fact that the majority of pediatric surgical centers provides prenatal consultation, caseloads are very variable as are referral modalities.

Birth of a child with congenital heart disease: emotional reactions of mothers and fathers according to time of diagnosis.

OBJECTIVE: To evaluate emotional distress, depression and quality of life in parents of infants with severe congenital heart disease (CHD) during their first hospitalization. METHODS: A pilot study for 38 parental couples of infants with CHD hospitalized within the 3 months of life. Parents filled up three self-administered questionnaires. We compared differences in the variables measuring emotional distress, depression and quality of life between mothers and fathers, and between prenatal and postnatal diagnosis. RESULTS: Stress and depression levels were significantly higher in mothers than in fathers (stress: 81.8% mothers versus 60.6% fathers; depression: 45.7% mothers versus 20.0% fathers). No difference were found between prenatal and postnatal groups in any field tested but, in percentage, mothers receiving prenatal diagnosis were more depressed, whereas those receiving postnatal diagnosis were more stressed. Fathers showed same tendency. CONCLUSIONS: Parents of newborns with severe CHD, especially mothers, need psychological support during their child's hospitalization. Parents of children diagnosed prenatally may need counseling throughout pregnancy to help them recover from the loss of the imagined healthy child.

Evaluation of haemoglobin, haematocrit, haemolysis, residual protein content and leucocytes in 345 red blood cell concentrates used for the treatment of patients with ß-thalassaemia.

BACKGROUND: The aim of this study was to evaluate the quality of red blood cell concentrates obtained from donated whole blood, selected for transfusion therapy of thalassaemic patients, by measuring the following parameters: haemoglobin, haematocrit, percentage haemolysis, residual leucocyte count and residual protein content. MATERIALS AND METHODS: Overall 345 red cell concentrates were evaluated, of which 205 had been filtered in-line pre-storage and washed and 140 were buffy coat-depleted and used within 2 days of collection. Of the buffy coat-depleted concentrates, 62 were leucodepleted and 78 washed and leucodepleted post-storage all within 2 days of collection. The off-line filters used for the leucodepletion were gamma-irradiated polyester with a pore size of 200 µm. The washing procedure was automated (Haemonetics ACP 215, Braintree, MA, USA). The haematological parameters were evaluated by a blood cell counter (Coulter, Ramsey, IL, USA) and the white blood cell count by cytofluorimetry (FACScan). RESULTS: Ninety-five percent (194/205) of the red cell concentrates that had been filtered pre-storage and washed, 92% (57/62) of the red cell concentrates that had been leucodepleted post-storage and 94% (73/78) of the those subjected to both treatments had normal values of haemoglobin (>40 g/unit), haematocrit (between 50-70%), percentage haemolysis (<0.8/unit), white cell count (<1×10(6)) and residual protein content (<0.5 g/L). Five percent (11/205) of the red cell concentrates that had been filtered pre-storage and washed, 8% (5/62) of those leucodepleted post-storage after 2 days and 6% (5/78) of those that underwent both procedures had a haemoglobin content <40 g/unit and a haematocrit <50%. CONCLUSIONS: The preparation procedures had been carried out satisfactorily; nevertheless, transfusion therapy with some "low dose" normal units could be less effective and might, therefore, result in greater transfusion requirements in patients receiving such units.

Change in cognitive abilities over time during preschool age in low risk preterm children.

BACKGROUND: More information is needed on 'low-risk' preterm infants' neurological outcome so that they can be included in follow-up programs at least until school age. OBJECTIVE: To examine the neuropsychological outcome in a group of low-risk low birth weight (LBW) children without neurological impairment followed from birth to 5 years of age. PATIENTS: 26 intellectually normal children born preterm (30-34 weeks gestation) without major neurological disabilities and 23 control children born at term and matched for age, sex, and parental educational and occupational status. METHODS: Subjects already evaluated at 3 years of age underwent assessment again at 5 years using as neuropsychological outcome measures a wide range of tests including perceptual and visual-motor function, language comprehension and expression, and attention skills. RESULTS: When tested at 5 years, children born preterm still obtained significantly lower mean scores than controls on visual motor integration test (57 vs 64, p=0.01), visual perception test (41 vs 43, p=0.002) and a trend toward a lower score in the picture vocabulary test (81 vs 85.5, p=0.07). The group of premature infants and controls improved their performance over time in the neuropsychological abilities investigated and, in some skills such as visual perception. Children born preterm took longer than those born at term to reach similar performance levels, 5 versus 3 years. CONCLUSION: Ex low-risk children born preterm achieve lower scores over time in visual-motor and perceptual ability scales and in some language tests than children born at term. Like high-risk premature infants even those at low risk deserve regular follow-up with long-term programs.

Cognitive assessment of very preterm infants at 2-year corrected age: performance of the Italian version of the PARCA-R parent questionnaire.

BACKGROUND: Serial assessments of cognitive and language development are recommended for very preterm children, but standardized neuropsychological testing is time-consuming and expensive, as well as tiring for the child. AIMS: To validate the Italian version of the PARCA-R parent questionnaire and test its clinical effectiveness in assessing cognitive development of very preterm children at 2 years of corrected age. METHODS: 120 consecutive Italian very preterm children (mean gestational age 28.8 weeks, standard deviation 2.1) were assessed in four hospitals through the Mental Development Index (MDI) of the Bayley Scales of Infant Development (BSID-II). Parents completed the PARCA-R questionnaire, designed to measure children's non-verbal and verbal (vocabulary and sentence complexity) cognitive level. The correlation between the MDI and the PARCA-R Parent Report Composite (PRC) was tested through the Pearson correlation coefficient, and the receiver operating characteristic (ROC) curve was used to identify optimal PRC cut-offs. RESULTS: Significant correlation between the PRC score and MDI (r=0.60, p<0.001) indicated good concurrent validity. The area under the ROC curve was 0.83, and the cut-off of 46 lead to 72.7% sensitivity and 77.1% specificity in identifying children with moderate/severe cognitive delay (MDI<70). Negative predictive value was 96.6 (90.3-99.3). Screening through PARCA-R would reduce the number of children with MDI≥70 undergoing BSID-II or equivalent standardized tool from 109 to 25. CONCLUSIONS: The Italian version of PARCA-R retains good discriminative power for identifying cognitive delay in 2-year very preterm children. It is well accepted by parents, and represents a valid and efficient alternative for developmental screening and outcome measurement.

Long term memory profile of disorders associated with dysregulation of the RAS-MAPK signaling cascade.

In the present study we evaluated long term memory in twenty individuals with molecularly confirmed diagnosis of Noonan syndrome and LEOPARD syndrome, two disorders caused by mutations in genes coding transducers participating in the RAS-MAPK signaling cascade. The profile of explicit long term memory abilities was investigated using PROMEA, which includes a battery of tests specifically developed to assess memory and learning in verbal, visual and spatial domains. Ten individuals (50%) had impaired (≤5th percentile) or below average (≤15th percentile) performance on a delayed verbal free recall memory task, four (20%) on a delayed visual recognition memory task, and only one (5%) on a delayed spatial recognition memory task. Our data suggest that dysregulation of the RAS-MAPK cascade may be associated with a pattern of reduced verbal recall memory performance but relative sparing of visual and spatial recognition memory.

Parents, siblings and grandparents in the Neonatal Intensive Care Unit. A survey of policies in eight European countries.

OBJECTIVE: To describe policies towards family visiting in Neonatal Intensive Care Units (NICU) and compare findings with those of a survey carried out 10 years earlier. METHODS: A questionnaire on early developmental care practices was mailed to 362 units in eight European countries (Sweden, Denmark, the UK, the Netherlands, Belgium, France, Spain and Italy). Of them 78% responded, and among those responded, 175 reported caring for at least 50 very low birth weight infants every year and their responses were analysed further. RESULTS: A majority of all units allowed access at any time for both parents. This was almost universal in northern Europe and the UK, whereas it was the policy of less than one-third of NICU in Spain and Italy, with France in an intermediate position. Restrictions on visiting of grandparents, siblings and friends, as well as restricting parents' presence during medical rounds and procedures followed the same pattern. A composite visiting score was computed using all the variables related to family visiting. Lower median values and larger variability were obtained for the southern countries, indicating more restrictive attitudes and lack of national policy. CONCLUSIONS: The presence of parents and other family members in European NICUs has improved over a 10-year period. Several barriers, however, are still in place, particularly in the South European countries.

The effect of cinnarizine and cocculus indicus on simulator sickness.

Pensacola Simulator Sickness Questionnaire (SSQ) is a valuable method to analyse symptoms evoked by exposure to a flight simulator environment that can also be adopted to evaluate the effectiveness of preventive tools, aiming at reducing simulator sickness (SS). In this study we analysed SSQ data in subjects undergoing a standard ground based spatial disorientation training inside a flight simulator, in order to evaluate the SS prevention obtained with two different pharmacological tools. Twelve males volunteers participated to an experimental design based on a double-blind, balanced administration of either 30 mg cinnarizine (CIN), or Cocculus Indicus 6CH (COC), or placebo (PLC) before one trial of about one hour spent inside a spatial disorientation trainer. All subjects underwent the three different conditions (CIN, COC, PLC) during 3 non-consecutive days separated by at least 2 weeks. During each experimental day, all subjects filled in SSQ. In addition, both postural instability (with the use of a static stabilometric platform), and sleepiness symptoms were evaluated. All the tests were performed before and after the simulated flight, at different times, in one-and-half-hour intervals. Results indicated a strong increase of sickness after flight simulation that linearly decreased, showing pre-simulator scores after 1.30 hours. In contrast to both PLC and COC, CIN showed significant side effects immediately following flight simulation, with no benefit at the simultaneous SSQ scores. Globally, no highly significant differences between COC and PLC were observed, although a minor degree of postural instability could be detected after COC administration. As far as the present exposure to a simulator environment is concerned, none of the pharmacological tools administered in this study resulted effective in reducing SS symptoms as detected by the SSQ. Moreover, CIN significantly increased sleepiness and postural instability in most subjects.