David Neubauer

The Risk of Lower Mental Outcome in Infantile Spasms Increases after Three Weeks of Hypsarrhythmia Duration

Summary:  To assess the correlation between hypsarrhythmia duration and mental outcome in infantile spasms (IS) the medical records of 48 infants with IS were reviewed retrospectively and psychological assessments undertaken at follow‐up at the age of 3 to 13 years. We found 18 (38%) cryptogenic IS cases with typical hypsarrhythmia and 30 symptomatic with modified hypsarrhythmia—further classified into 15 cases as multifocal, 10 as pseudoperiodic and 5 as unilateral hypsarrhythmia. A short treatment lag (one to two weeks) occurred in 25, three to four weeks in 10 cases. Spasms ceased within one month after treatment in 23 infants. At follow‐up 15 children had normal mental outcome (borderline included). A correlation between hypsarrhythmia duration longer than three weeks and lower mental outcome was found using the logistic regression model. The duration of hypsarrhythmia represents a sensitive prognostic parameter in IS; the risk of mental retardation increases after three weeks of hypsarrhythmia.

Congenital hypotonia: Is there an algorithm?

This study was performed with the aim of determining the diagnostic profile of newborns with hypotonia and of analyzing the usefulness of different procedures in the diagnostic process. One hundred thirty-eight hypotonic newborns were identified through the search of hospital records in a 10-year period: 121 (88%) had central hypotonia and 13 (9%) had peripheral hypotonia, whereas 4 (3%) remained unclassified. Analysis of the contribution of clinical data and results of investigations led to the construction of an algorithm, by which all cases in the group were diagnosed. Step 1, which included clinical data and results of examinations, solved 50% of all diagnosed cases. Neuroimaging techniques made up step 2 and contributed to the diagnosis in 13%. Step 3 was accomplished by a search through Oxford Medical Databases, which yielded the final diagnosis in 9%, whereas karyotyping and fluorescent in situ hybridization for Prader-Willi syndrome comprised step 4 and contributed to the diagnosis in 6.5%. Biochemical tests formed step 5 and contributed to the diagnosis in 6%. Step 6, which included specific investigations of muscle and nerve, was diagnostic in 6%. The remaining cases (6.5%) were diagnosed only after several follow-up examinations. These results could assist the neonatologist when deciding the diagnostic approach to floppy newborns. (J Child Neurol 2004;19:439-442).

Amiel-Tison Neurological Assessment at term age: clinical application, correlation with other methods, and outcome at 12 to 15 months

The aims of this study were: (1) to perform the Amiel‐Tison Neurological Assessment (ATNA) in a group of infants with different risk factors for brain damage; (2) to analyze the results of the examinations in light of the risk factors and presumed aetiology; (3) to compare results of examinations with results of cranial ultrasound, electroencephalography (EEG), and cerebral function monitoring (CFM); and (4) to evaluate neurological outcome at 12 to 15 months of age using the Amiel‐Tison and Gosselin method, and developmental outcome using the Bayley Scales of Infant Development. Participants were 52 term, newborn infants (31 males, 21 females) with risk factors for brain damage. Mean birthweight was 3288g (SD 661g) and mean gestational age was 39.4wks (SD 1.2wks); range 38 to 41.3wks. Mean age at admission to a neonatal special care unit was 75h, (SD 13.7h). The group with a dynamic (evolving) clinical profile differed significantly from the group with a static (stable) profile in terms of aetiology, while the group with signs of prenatal brain damage differed from the group without these signs regarding aetiology and the level of severity of neurological signs. Sensitivity of the ATNA to detect infants with abnormal ultrasound was 0.97, with EEG 0.89, and with CFM 0.88. At follow‐up at 12 to 15 months 47 children were examined: neurological examination was normal in 25 and five children had a minor, five a moderate, and 12 a severe neurological deficit. Agreement of the ATNA with neurological and developmental assessment at follow‐up was very good. Our findings suggest that the ATNA is also of value in assessing aetiology and timing of brain lesions.

Economic evaluation of Haemophilus influenzae type b vaccination in Slovenia.

The objective of the present study was to assess the economical impact of invasive Haemophilus influenzae type b infections in Slovenia, where the annual incidence of these infections is 16.4/100000 in children less than 5 years of age, and to compare it with the costs of a vaccination programme. The lifetime costs and benefits were estimated for the annual birth cohort of 18200 children. In the base-case model, the calculated benefit-to-cost ratios were 0.15, 0.98 and 1.38 taking into account 95% of savings in acute care costs, medical costs, and medical and non-medical costs, respectively. From the point of view of the Institute of Health Insurance of Slovenia, who pays all healthcare and vaccination costs, the vaccination programme per annual birth cohort of 18200 children would require an extra 7023 EUR or 0.40 EUR per cohort-child. The savings to society would represent 118410 EUR, indicating the rationale for inclusion of H. influenzae type b vaccination in the routine childhood immunisation programme in Slovenia.

A Comparison of Respiratory Patterns in Healthy Term Infants Placed in Car Safety Seats and Beds

OBJECTIVE: The purpose of this work was to compare the incidence of apnea, hypopnea, bradycardia, or oxygen desaturation in healthy term newborns placed in hospital cribs, infant car safety beds, or infant car safety seats. METHODS: A consecutive series of 200 newborns was recruited on the second day of life. Each subject was studied while placed in the hospital crib (30 minutes), car bed (60 minutes), and car seat (60 minutes). Physiologic data, including oxygen saturation, frequency, and type of apnea, hypopnea, and bradycardia were obtained and analyzed in a blinded manner. RESULTS: The mean oxygen saturation level was significantly different among all of the positions (97.9% for the hospital crib, 96.3% for the car bed, and 95.7% for the car seat; P < .001). The mean minimal oxygen saturation level was lower while in both safety devices (83.7% for the car bed and 83.6% for the car seat) compared with in the hospital crib (87.4%) (P < .001). The mean total time spent with an oxygen saturation level of <95% was significantly higher (P = .003) in both safety devices (car seat: 23.9%; car bed: 17.2%) when compared with the hospital crib (6.5%). A second study of 50 subjects in which each infant was placed in each position for 120 minutes yielded similar results. CONCLUSIONS: In healthy term newborns, significant desaturations were observed in both car beds and car seats as compared with hospital cribs. This study was limited by lack of documentation of sleep stage. Therefore, these safety devices should only be used for protection during travel and not as replacements for cribs.

Epidemiologic Features of Infantile Spasms in Slovenia

Summary:  Purpose: To determine the epidemiologic features of infantile spasms (ISs) in Slovenia.

Female genital mutilation: a hidden epidemic (statement from the European Academy of Paediatrics)

Female genital mutilation or female circumcision is frequently performed worldwide. It is estimated by the World Health Organisation that worldwide, 100–140 million girls and women currently have to live with the consequences of female genital mutilation. The article argues that the tradition is one of the causes, while another four possible reasons for undergoing such cruel mutilation of young girls exist. Today, there exists a classification of at least four different ways of such mutilation which has no health benefits, and it harms girls and women in many ways. Long-term consequences like recurrent urinary tract infections, dysmenorrhea, sexual problems, infertility and complications both for the mother and infant at delivery are mentioned. Female genital mutilation is a violation of the fundamental human rights, as well as a savage breach of the integrity and personality. Conclusion: The European Academy of Paediatrics advises its members to initiate appropriate counselling for parents and female adolescents regarding the risk of female genital mutilation and strongly condemns female genital mutilation and councils its members not to perform such procedures.

Prenatal mercury exposure, neurodevelopment and apolipoprotein E genetic polymorphism

Abstract The aim of the present study was to evaluate the association between prenatal exposure to mercury (Hg) and neurodevelopment of the child, taking into account genetic polymorphism of apolipoprotein E (Apoe) and other relevant confounders. Six hundred and one mother‐child pairs were recruited from the central Slovenia region and 243 from Rijeka, on the Croatian coast of the northern Adriatic. The total Hg in cord blood, Bayley Scales of Infant and Toddler Development, Third Edition (Bayley‐III) assessment at 18 months of age and Apoe genotyping was performed on 361 children; 237 of them were from Slovenia and 124 from Croatia. The results showed negative association between low‐to‐moderate Hg exposure in children with normal neurodevelopmental outcome and cognitive and fine motor scores at 18 months of age as assessed by Bayley III. The Hg‐related decrease in cognitive score was observed only in children carrying at least one Apoe &egr;4 allele, while the decrease in fine motor scores was independent of the Apoe genotype. Adjusting for selenium (Se) and lead (Pb) levels, a positive association between Se and the language score and a negative association between Pb and the motor score was observed, but not in the subgroup of children carrying the &egr;4 allele. HighlightsApoe &egr;4 carriers showed negative association between cord blood Hg and cognitive score.Fine motor score was negatively associated with cord blood Hg in all children.Gene‐environment interaction was confirmed for the cognitive score..Se and Pb levels confounded the association between Hg and neurodevelopment.

Region-specific reduction in brain volume in young adults with perinatal hypoxic-ischaemic encephalopathy

BACKGROUND A severe form of perinatal hypoxic-ischaemic encephalopathy (HIE) carries a high risk of perinatal death and severe neurological sequelae while in mild HIE only discrete cognitive disorders may occur. AIM To compare total brain volumes and region-specific cortical measurements between young adults with mild-moderate perinatal HIE and a healthy control group of the same age. METHODS MR imaging was performed in a cohort of 14 young adults (9 males, 5 females) with a history of mild or moderate perinatal HIE. The control group consisted of healthy participants, matched with HIE group by age and gender. Volumetric analysis was done after the processing of MR images using a fully automated CIVET pipeline. We measured gyrification indexes, total brain volume, volume of grey and white matter, and of cerebrospinal fluid. We also measured volume, thickness and area of the cerebral cortex in the parietal, occipital, frontal, and temporal lobe, and of the isthmus cinguli, parahippocampal and cingulated gyrus, and insula. RESULTS The HIE patient group showed smaller absolute volumetric data. Statistically significant (p < 0.05) reductions of gyrification index in the right hemisphere, of cortical areas in the right temporal lobe and parahippocampal gyrus, of cortical volumes in the right temporal lobe and of cortical thickness in the right isthmus of the cingulate gyrus were found. Comparison between the healthy group and the HIE group of the same gender showed statistically significant changes in the male HIE patients, where a significant reduction was found in whole brain volume; left parietal, bilateral temporal, and right parahippocampal gyrus cortical areas; and bilateral temporal lobe cortical volume. CONCLUSIONS Our analysis of total brain volumes and region-specific corticometric parameters suggests that mild-moderate forms of perinatal HIE lead to reductions in whole brain volumes. In the study reductions were most pronounced in temporal lobe and parahippocampal gyrus.

Motor activity during asymptomatic nocturnal hypoglycemia in adolescents with type 1 diabetes mellitus

Abstract.Nocturnal hypoglycemia is reported in 13%–56% of adolescents with type 1 diabetes mellitus. It may be asymptomatic in more than 50% of patients. No noninvasive method for detecting asymptomatic nocturnal hypoglycemia (ANH) has so far proven successful. The aim of the present study was to evaluate quantitative changes of motor activity by actigraphy during episodes of ANH in adolescents with type 1 diabetes mellitus. A total of 18 patients aged 10–16 years with a history of ANH were investigated. Blood was sampled at half-hourly intervals between 22.30 and 06.00 hours with a micropump, and an actigraph was fastened to the right wrist. Blood glucose concentrations were measured and compared to motor activity. Nocturnal hypoglycemia was recorded in 10 patients (55%), with blood glucose during periods of hypoglycemia of 3.00+0.17 mmol/l (range, 1.2–3.4 mmol/l), and duration of hypoglycemia of 1.95+1.34 hours (range, 0.5–5.0 hours). All periods of hypoglycemia were clinically asymptomatic. Regression analysis revealed a statistically significant linear correlation (p=0.03) between blood glucose concentration and the respective 30-min activity counts. Activity counts in patients with nocturnal hypoglycemia were significantly (ANOVA, p<0.02) higher than in patients with normoglycemia. We conclude that low blood glucose was significantly correlated with an increase in motor activity as detected by actigraphy. This implies the possibility of noninvasive screening of asymptomatic nocturnal hypoglycemia.