Quality of life in parents of children with cerebral palsy: is it influenced by the child's behaviour?

The aims of the present study were: to examine the quality of life (QOL) of parents of children with cerebral palsy (CP) and to establish the possible effect of behaviour problems on their QOL. One-hundred children with CP, aged between 4 and 10 years, and both their parents were included in the study. Both parents completed the WHOQOL-BREF, to assess their QOL. A sample of 60 parents of healthy children was used as control group. The primary caregiver also completed the CHILD BEHAVIOUR CHECKLIST (CBCL). Parents of children with CP showed lower scores on physical and psychological domains than the control group on QOL. In the psychological domain the mothers of children with hemiplegia had the lowest scores. The mothers reported lower scores than the fathers for the physical domain in the group of children with diplegia and quadriplegia and for the psychological domain in the group of children with hemiplegia. Children with hemiplegia showed externalizing scores at CBCL higher than the other groups, that could explain the poorer QOL scores of their mothers. In conclusions our results provide useful information on the QOL in families with different forms of CP, useful in planning interventions for the family of children with CP.

Experience with the Platelia Candida ELISA for the diagnosis of invasive candidosis in neonatal patients.

This preliminary study evaluated the use of the Platelia Candida antigen kit for the diagnosis of invasive candidosis in 70 of 184 pre-term infants admitted to a neonatal intensive care unit between March 2004 and March 2006. The frequency of confirmed candidaemia was 6.5%. The sensitivity and specificity of the assay were 94.4% and 94.2%, respectively, with a positive predictive value of 85% and a negative predictive value of 98%. These results suggest that the inclusion of regular serological surveillance for mannanaemia in some pre-term infants would complement blood cultures for the early detection of candidosis.

Infant neurological examination from 3 to 12 months: predictive value of the single items.

The prognostic value of the single items of a standardised neurological examination, the Hammersmith Infant Neurologic Examination (HINE), was explored longitudinally in 658 infants at 3, 6, 9 and 12 months post-term age. ROC curves were built based on the presence/absence of cerebral palsy at 2 years of age. Global HINE scores showed very high prediction (ROC curve areas above 0.9) at all ages. The items with the highest predictive value were always movement quality and quantity. In the first semester, among the most predictive items were those assessing tone, while beyond that time they were reflexes and reactions. Our results show that the high predictive value of the HINE across the first year of life is granted by the successful combination of different groups of items for each age-period. This should be recognised in clinical practice when assessing the significance of individual neurological profiles.

Inhibition of glucagon secretion in the human newborn by glucose infusion.

Plasma glucagon, serum insulin, and blood glucose responses during a 30-min glucose (1 g/kg body wt) or saline infusion were reported in 37 full-term and 35 preterm infants. They were studied either on the first day of life before feeding was initiated or during the first week of life. Glucose infusion promptly suppressed glucagon secretion in all infants even from the initial hours of extrauterine life. The mean maximal decrement in percentage in the full-term infants on the day of birth and older was 54 +/- 4% and 61 +/- 6%, respectively, while the maximal decrement in the preterm infants on day of birth and older was significantly lower (44 +/- 3% and 38 +/- 4%, respectively). The insulin response to glucose was variable in all infants and most of them showed a delayed rise of serum insulin. No change in plasma glucagon, blood glucose, and serum insulin was observed during and after saline infusion.

Dextrocardia with and without situs viscerum inversus in two sibs.

We described two sibs born to consanguineous Sicilian parents who died of severe congenital heart malformation. Both had dextrocardia; however, only the girl had situs viscerum inversus. At necropsy she was found to have a right spleen and right pulmonary isomerism (three lobes in each lung, as commonly found in the asplenia syndrome). This observation, together with other literature reports, suggest that isolated dextrocardia, situs viscerum inversus, and the asplenia-polysplenia complex may be different end results of a unique dysmorphogenetic process involving the embryonic midline.

Caudal regression syndrome and annular pancreas: a rare association.

A female infant with caudal regression syndrome and annular pancreas is described. This is the first time this association appears to have been described.

[Physiopathologic and therapeutic aspects of persistent fetal circulation. Review of the literature and personal histological observations]. / Aspetti fisiopatologici e terapeutici della persistenza della circolazione fetale. Revisione della letteratura ed osservazioni istologiche personali.

Persistence of the fetal circulation (PFC) is a syndrome characterized by failure of the cardiocirculatory system to adapt successfully to postnatal life. Its typical feature is persisting right-left shunt across fetal channels which determines cyanosis refractory to oxygen treatment. PFC can simulate cyanotic congenital cardiopathy. It has two forms: a primitive form and secondary one due to various causes especially perinatal asphyxia. Both forms have a common pathogenesis consisting of hypertension of the pulmonary arterial circulation. This article reviews the physiology of the main prenatal and postnatal circulatory characteristics and the factors which regulate the pulmonary circulation. It also reports the latest findings on PFC physiopathology and treatment indicating the prognostic factors and future perspectives.

[CHARGE association: report of a clinical case with anal atresia and rectovaginal fistula]. / Associazione CHARGE: descrizione di un caso clinico con atresia anale e fistola retto-vaginale.

CHARGE association is a non-random constellation of Coloboma, Heart Disease, Atresia of the choana, Retarded mental development and growth, Genital hypoplasia, Ear anomalies and deafness. We report on a newborn with CHARGE association. In addition to the typical features our case presented anal atresia and rectovaginal fistula. Our patient had congenital cardiopathy and feeding problems and died at the age of 3 months. Most case of CHARGE association appear to have a primary defect of pharyngeal incoordination that cause aspiration of secretion that is an important cause of death in the nonsurvivors.

[Variations of blood bilirubin levels in the newborn with and without retinopathy of prematurity (ROP)]. / Variazioni dei livelli bilirubinemici nei neonati con e senza retinopatia della prematurità (ROP).

Retinopathy of prematurity (ROP) is a multifactorial disease where production of free radicals is a pathogenic factor. Bilirubin is regarded today as the most powerful antioxidant substance "in vitro". To test such effect "in vivo" we studied 219 premature infants, admitted to our Neonatal Intensive Case Unit from April 1991 to October 1992, evaluating their serum bilirubin levels from day two to seven and mean bilirubin level of first week for each child. We also calculated the mean rate of daily increase of bilirubin. Our results show that bilirubin parameters considered are higher in neonates which will develop ROP of every stage than in the control ones. These results therefore do not support the concept that bilirubin could have a role in the prevention of ROP.

[A comparison of the efficacy of aminophylline and doxapram in preventing idiopathic apnea in preterm newborn infants]. / Comparazione dell'efficacia dell'Aminofillina e del Doxapram nella prevenzione della apnea idiopatica dei neonati pretermine.

Doxapram is an analeptic of the respiratory system that has been used in the last few years for the treatment of idiopathic apnea spells in infants who show resistance to methylxantine. In this study we have compared the efficacy of aminophylline and doxapram for the prevention of idiopathic apnea spells in two groups of preterm infants comparable for gestational age, birthweight and postnatal age. The two drugs resulted to be effective in preventing the spells of apnea in 66% and 60% of the cases respectively. In the cases in which there was a partial or negative response, the association of the two substances resulted in a noticeable reduction of the apnea spells. The positive effect of the association of aminophylline and doxapram is probably due to the different action mechanism on the stimulation of the respiratory system.

[Oral administration of doxapram in preterm neonates with aminophylline-resistant idiopathic apnea crisis]. / L'impiego del doxapram per os nei neonati pretermine con crisi d'apnea idiopatiche resistenti alla aminofillina.

Doxapram is an analeptic capable of stimulating both central and periferal areas of the respiratory system. During the last few years, intravenous infusion of doxapram has been carried out, with success, for the treatment of idiopathic apnea in preterm infants otherwise unresponsive to methylxantine. Since doxapram has a tendency to precipitate into various solution containing amino acids and calcium gluconate, oral administration has been suggested. The Authors in this study have seen that in 18 preterm infants suffering from idiopathic apnea unresponsive to amynofillina, an oral administration of doxapram at 12 mg/kg/6h resulted in a complete recovery from apnea spells in 66.7% of cases, while 22.2% gave a partial positive response and only 11.1% a negative result. Furthermore the Authors would like to stress that doxapram showed a good tolerance level when administered orally. In fact, no side effects which were previously reported in other publications were presented in the infants studied.

[Pena-Shokeir syndrome: report of a case with benign outcome]. / La sindrome di Pena-Shokeir: descrizione di un caso clinico ad evoluzione benigna.

Pena-Shokeir syndrome is a rare, often lethal disease, characterized by intrauterine growth retardation and by fetal akinesia or hypokinesia that leads to craniofacial anomalies, limb ankylosis, polyhydramnios and pulmonary hypoplasia. The case that we report had a favourable evolution, although there was at birth a severe respiratory distress. EMG studies revealed deficit of innervation. Contraceptive therapy, wrongly used by the mother in the first period of pregnancy, played, probably, a concomitant role in the pathogenesis of the syndrome.

[Realities and perspectives of perinatal care in Sicily. Experience at an outborn neonatal unit]. / Realtà e prospettive dell'assistenza perinatale in Sicilia. L'esperienza di un Centro con neonati esculsivamente "outborn".

The Authors studied some mortality indices in the neonatal intensive and subintensive care unit of the University of Catania in order to assess the degree of efficiency of perinatal assistance. This Unit is the largest in southeastern Sicily and admits only outborn newborns from the city of Catania, the Catania province and other provinces. Comparison of the 1991-92 and the 1993-94 data revealed a marked reduction in the mortality rate, however this fall was only marginal in newborns of, or under 1,000 g. There was a marked decrease in the mortality rate from respiratory distress, especially in ventilated newborns receiving additional surfactant. Comparison of the data showed that in both study periods the mortality rate was much higher in newborns over 6 hours of life, in those presenting hematic pH values under 7.25 at admission and in those coming from other provinces. These results underline that it is essential for the political authorities to boost neonatal assistance in the delivery room and to ensure adequate transport of distressed newborns.

[Concentration of blood ICAM-1s in the newborn at risk of infection. A prospective study in the first 2 weeks of life]. / Concentrazioni sieriche di ICAM-1s in neonati a rischio di infezione. Studio prospettico nelle prime due settimane di vita.

We prospectively determined serum concentrations of soluble intercellular adhesion molecule 1 (sICAM-1) in the first 2 weeks of life in 32 preterm newborns in an attempt to assess whether these concentrations are reliable markers of sepsis in newborns at risk of infection. Ten of the study group were normal and had been hospitalized only for low birth weight. The remaining 22 presented respiratory distress (RDS) and were at even higher risk of infection because they required assisted mechanical ventilation and central venous catheterisation for parenteral feeding and infusion therapy. Sepsis was diagnosed in 11/22 newborns with RDS: in 3 on day 3 and in 8 on day 7. Circulating sICAM-1 concentrations were significantly elevated in neonates with RDS (group II) and associated infection (group III) compared with normal newborns (group I). However, after day 3 of life sICAM-1 values were significantly higher in group III than in group II.

[Epidemiological and clinical findings in congenital heart diseases during the neonatal period. Report on personal cases]. / Rilievi epidemiologici e clinici sulle cardiopatie congenite in epoca neonatale. Contributo casistico personale.

Cardiovascular impairment is frequent in the neonatal period and can be linked to malformative and non malformative pathologies. The Authors performed a retrospective study on the incidence of these two types of pathologies in a population of 3326 newborns who underwent cardiological examination. Malformative cardiopathy was observed in 779 (23.4%) of them, while non malformative cardiopathy was present in 316 (9.5%). Acyanogen forms (86.7%) were more common than cyanogen forms (13.2%) in malformative, while cyanogen forms (64.8%) were more frequent than acyanogen ones (35.1%) in non malformative cardiopathies. The Authors reviewed the incidence of the single cardiac defects and report some clinical and physiopathological considerations that underline the need for prompt differential diagnosis and the validity of echocardiography in achieving this aim.

[Epidemiological,and physiopathological findings in retinopathy of prematurity: the authors' personal cases]. / Rilievi epidemiologici e fisiopatologici sulla retinopatia del prematuro: contributo casistico personale.

The authors present a retrospective epidemiological study on retinopathy of prematurity (ROP), performed on 738 newborns with a gestational age of 34 +/- 2.41 weeks and birth weight of 1971 +/- 351 g. They excluded all cases with severe respiratory distress and other pathological conditions. First stage of ROP shows no correlation with gestational age and birth weight, whereas second and third stages of ROP show an inverse correlation with the above mentioned factors, particularly birth weight. Among neonates with the same gestational age, the incidence of ROP is higher in those "small for date". Regarding the pathogenesis of ROP, the authors outline the importance of perinatal hypoxia.

[Decreased plasma fibronectin (pFN) level in preterm infants with infections]. / Riduzione dei livelli plasmatici di fibronectina (FNp) in neonati prematuri infetti.

Plasma fibronectic (pFN) is a high molecular weight multifunction glycoprotein, which augments neutrophil and macrofage phagocytosis and acts as a nonspecific opsonin for the reticuloendothelial system. In this study we have determined pFN concentrations in fifty eight preterm infants to discriminate infected from non infected ones. Concentrations of pFN decreased from baselin in babies with early or late onset infections. The changes in pFN concentrations were not found before sepsis, but on day 1. By day 5 pFN concentrations have increased and have been no longer different from controls. We have calculated sensitivity (73.68%), specificity (74.36%), positive (58.35%) and negative (85.29%) predictive values of pFN and of other markers of infections (C-reactive protein--CRP-, Immature/Mature neutrophil ratio--I/M n. ratio-). Adding these tests to pFN, provided equal specificity and positive predictive value, but increased sensitivity (94.73%) and negative predictive value (96.43%). Thus, low concentrations of pFN may be a valuable but not early marker for neonatal infections. The combination of pFN, CRP and I/M n. ratio increase the precision of diagnostic testing.

[The importance of using the cerebral function monitor (CFM) in the neurological prognosis of neonates in intensive care]. / Importanza dell'uso del Cerebral Function Monitor (CFM) nella prognosi neurologica dei neonati ricoverati in Terapia Intensiva.

Cerebral function monitor (CFM), unlike traditional EEG, allows a long-term evaluation of electric brain activity, without interfering with the nursing of the newborn in the intensive care unit. Our aim was to evaluate the prognostic value of CFM for neurological outcome. We studied 102 newborns (gestational age 34.5 +/- 4.36 weeks; weight 1980 +/- 720 grams) by Multitrace CFM (Lectromed) 5 hours daily in the first week following admission. The patients also underwent cerebral echography, EEG and neurological follow-up to the 24th month. CFM was found to correlate well with the EEG recorded 3 months later. The persistence for at least one week of an I.C. tracing or the normalization of initial tracing have a good prognostic value (positive predictive value 95.23%), a persistently pathologic registration has a negative prognostic value (negative predictive value 85.18%), that even increases if cerebral echographic alterations are demonstrated (98.57%). The association of CFM and ultrasound abnormalities determines a relative risk for neurological motor impairment of 69.14, whereas CFM alone gives a relative risk of 6.4.

[The VACTERL association: a report of a clinical case with hepatic cystic lymphangiectasis]. / Associazione VACTERL: descrizione di un caso clinico con linfangectasia cistica epatica.

VACTERL association includes three or more of the following six anomalies: V (vertebral anomalies), A (anal atresia), C (cardiac abnormalities), TE (tracheo-esophageal fistula and/or esophageal atresia), R (reno-urinary anomalies) and L (limb defects). VACTERL cases are classified as "associated" when other than the typical six defects are present in the same infant, or "isolated" when they are not. We report a case of VACTERL association "associated" that presents an hepatic cystic lymphangiectasia that was never described before in literature. We also consider the most important factors involved in the aetiology of the typical anomalies.