Abstract: Recurrent endobronchial infection in cystic fibrosis requires treatment with intravenous antibiotics for several weeks usually in hospital, affecting health costs and quality of life for patients and their families.
Abstract: ABSTRACT: BACKGROUND: The notion of Palliative Care (PC) in neonatal and perinatal medicine has largely developed in recent decades. Our aim was to systematically review the literature on this topic, summarise the evolution of care and, based on the available data, suggest a current standard for this type of care. METHODS: Data sources included Medline, the Cochrane Library, CINAHL, and the bibliographies of the papers retrieved. Articles focusing on neonatal/perinatal hospices or PC were included. A qualitative analysis of the content was performed, and data on the lead author, country, year, type of article or design, and direct and indirect subjects were obtained. RESULTS: Among the 1558 articles retrieved, we did not find any quantitative empirical study. To study the evolution of the model of care, we ultimately included 101 studies, most of which were from the USA. Fifty of these were comments/reflections, and only 30 were classifiable as clinical studies (half of these were case reports). The analysis revealed a gradual conceptual evolution of the model, which includes the notions of family-centered care, comprehensive care (including bereavement) and early and integrative care (also including the antenatal period). A subset of 27 articles that made special mention of antenatal aspects showed a similar distribution. In this subset, the results of the four descriptive clinical studies showed that, in the context of specific programmes, a significant number of couples (between 37 and 87%) opted for PC and to continue with the pregnancy when the foetus has been diagnosed with a lethal illness. CONCLUSIONS: Despite the interest that PC has aroused in perinatal medicine, there are no evidence-based empirical studies to indicate the best model of care for this clinical setting. The very notion of PC has evolved to encompass perinatal PC, which includes, among other things, the idea of comprehensive care, and early and integrative care initiated antenatally.
Abstract: It is common for patients who are faced with physical or psychological suffering, particularly those in the advanced stages of a disease, to have some kind of wish to hasten death (WTHD). This paper reviews and summarises the current state of knowledge about the WTHD among people with end-stage disease, doing so from a clinical perspective and on the basis of published clinical research. Studies were identified through a search strategy applied to the main scientific databases. Clinical studies show that the WTHD has a multi-factor aetiology. The literature review suggests-perhaps in line with better management of physical pain-that psychological and spiritual aspects, including social factors, are the most important cause of such a wish. One of the difficulties facing clinical research is the lack of terminological and conceptual precision in defining the construct. Indeed, studies frequently blur the distinction between a generic wish to die, a WTDH (whether sporadic or persistent over time), the explicit expression of a wish to die, and a request for euthanasia or physician-assisted suicide. A notable contribution to knowledge in this field has been made by scales designed to evaluate the WTHD, although the problems of conceptual definition may once again limit the conclusions, which can be drawn from the results. Studies using qualitative methodology have also provided new information that can help in understanding such wishes. Further clinical research is needed to provide a complete understanding of this phenomenon and to foster the development of suitable care plans.
Abstract: The objective of this study was to validate the Spanish version of the SMiLE (Schedule for Meaning in Life Evaluation). The SMiLE is a respondent-generated instrument: respondents are first asked to list three to seven areas, which provide meaning to their lives, and then to rate their current satisfaction with the listed areas, as well as the individual importance of each one. Indices of total weighting (IoW), total satisfaction (IoS), and total weighted satisfaction (IoWS) are calculated.
Abstract: There is a need for a better etiologic classification of preterm births and for tools to help to determine the possible etiologies of these births.
Abstract: A review of the evidence on treatment of acute bronchiolitis is presented. There is sufficient evidence on the lack of effectiveness of most interventions tested in bronchiolitis. Apart from oxygen therapy, fluid therapy, aspiration of secretions and ventilation support, few treatment options will be beneficial. There are doubts about the efficacy of inhaled bronchodilators (salbutamol or adrenaline), with or without hypertonic saline solution, suggesting that these options should be selectively used as therapeutic trials in moderate-severe bronchiolitis. Heliox and non-invasive ventilation techniques, methylxanthine could be used in cases with respiratory failure, in patients with apnea, and surfactant and inhaled ribavirin in intubated critically ill patients. The available evidence does not recommend the use of oral salbutamol, subcutaneous adrenaline, anticholinergic drugs, inhaled or systemic corticosteroids, antibiotics, aerosolized o intravenous immunoglobulin, respiratory physiotherapy and others (nitric oxide, recombinant human deoxyribonuclease, recombinant interferon, nebulised furosemide and so on).
Abstract: Idiopathic hypercalciuria is an inherited metabolic abnormality characterised by excessive amounts of calcium excreted into the urine in patients with normal serum levels of calcium. The morbidity of hypercalciuria is related to kidney stone disease and bone demineralization. In children, hypercalciuria can cause recurrent haematuria, frequency-dysuria syndrome, urinary tract infection and abdominal and lumbar pain. Several pharmacological treatments have been described that can decrease the levels of urinary calcium or its index of urinary crystallization.
Abstract: The etiology of preterm birth is difficult to classify. It is usually divided into three clinical types according to its clinical presentation: medically indicated; caused by ruptured membranes; and spontaneous or idiopathic. However, this classification is controversial, imprecise and can result in multiple interpretations when applied. OBJECTIVE: To design an etiologically based classification of preterm birth, and to design a system to easily assign each case during the perinatal period. METHODS: Review of literature, qualitative analysis using consensus methods through nominal group technique, and quantitative analysis of a pilot study using a first version of the algorithm. RESULTS: A classification is made to establish a general division between the "primary cause" and "associated causes" of preterm birth, that allows remote causes or risk factors to be included. The primary cause includes seven categories: inflammatory (ruptured membranes and related); vascular (intrauterine growth restriction and related); maternal-local; maternal-systemic; fetal pathology; fetal distress; idiopathic. The medically indicated preterm birth is defined as a previous or independent category and so is compatible with the other, previously mentioned causes . An algorithm was designed to make it easier to classify the primary cause of preterm birth using a flowchart. CONCLUSIONS: A pragmatic classification of preterm birth is proposed that may help to achieve better precision and agreement between clinicians.
Abstract: OBJECTIVE: To verify the usefulness and reliability of a software tool we developed to help to apply the American Academy of Pediatrics Guidelines 2004 on hyperbilirubinemia according to the infant's age in hours and their clinical risk factors. DESIGN: Randomised cross-over controlled trial with 20 simulated clinical cases comparing the "manual"application of the guidelines with our software application. PARTICIPANTS: Fifteen doctors (eight final-year residents and seven consultants) from two hospitals in Spain. MAIN OUTCOME MEASURES: Major errors (defined a priori as any deviation from the AAP guideline that involves a risk of morbidity or mortality for the patient), minor errors (those that cause discomfort and/or, in extremely rare cases, morbidity) and time spent. RESULTS: Fifteen doctors each managed 20 simulated cases, half by using the guideleines alone and half using the software tool. Without the software application, 42 "minor" errors were made. With it, only 25 errors were made. "Major" errors also decreased from 10 to 2 with the software. As a group, the residents benefited most: they made an average of 1.8 errors fewer per ten cases. Use of the software reduced the time taken by the residents to resolve the cases, although the mean reduction in time was not significant for the group of consultants. CONCLUSIONS: The use of simulated clinical cases revealed many errors in the routine management of hyperbilirubinemia. The software helped clinicians make fewer errors and saved time for residents, but not consultants.
Abstract: Intrauterine growth restriction (IUGR) remains an important cause of perinatal morbidity and mortality. Both IUGR and low birth weight have been identified as risk factors for increased incidence of cardiovascular disease, dyslipemia, and other diseases in the adulthood. Several animal models have been developed to study the underlying mechanisms of IUGR and its later consequences, with utero-placental ischemia by uterine artery ligation (UAL) being the most frequently used in rats. The relevance of this model lies in the fact that it induces altered placental perfusion, the main cause of IUGR in humans in Western countries. However, there is also controversy over the grade and homogeneity of IUGR obtained. In this study, we propose a new animal model of IUGR related to placental ischemia through the cauterization of meso-ovarian vessels. We aimed to test the feasibility of meso-ovarian vessel cauterization (CMO), and to compare it with uterine artery ligation (UAL). The CMO group had similar incidence of perinatal mortality, percentage of IUGR, and evolution of body weight during early extrauterine life to the UAL group, indicating that both methods are similarly efficient for inducing IUGR. Moreover, both of them affect the ratio of fetal to placental weight, and the weight of vital organs, supporting the hypothesis of a fetal compensatory response or "brain- and heart-sparing effect". Both operative models suffer approximately 50% perinatal mortality, implying that they are both more efficient in the production of IUGR when C-section is performed. On the other hand, CMO was significantly faster to perform than UAL and seemed to produce a more uniform ischemia throughout the uterus than the UAL method, resulting in a more homogeneous group of IUGR pups.
Abstract: Recurrent endobronchial infection in cystic fibrosis (CF) requires treatment with intravenous antibiotics for several weeks usually in hospital, affecting health costs and quality of life for patients and their families.
Abstract: The emotional states of pregnant women affect the course of their pregnancies, their deliveries and the behaviour and development of their infants. The aim of this study is to analyse the influence of positive and negative maternal emotional states on neonatal behaviour at 2-3 days after birth. A sample of 163 healthy full-term newborns was evaluated using the Neonatal Behavioral Assessment Scale. Maternal anxiety, perceived stress, and emotional stability during pregnancy were evaluated in the immediate postpartum period with the State Trait Anxiety Inventory and the Perceived Stress Scale. Moderate levels of anxiety during pregnancy alter infant orientation and self-regulation. These aspects of infant behaviour could lead to later attachment, behavioural and developmental problems. Maternal emotional stability during pregnancy improves infant self-regulation and several aspects of infant behaviour that may predispose them to better interactions with their parents.
Abstract: INTRODUCTION: Cochrane Collaboration (CC) contains detailed, critical and up-to-date systematic reviews (SR) of the best scientific evidence available. AIM: To analyse the bibliometric characteristics of the SR related to paediatric neurology published in the 50 Collaborative Review Groups (CRG) of the CC. MATERIALS AND METHODS: Bibliometric analysis of the Database of Systematic Reviews in Cochrane Library, Issue 2, 2005 (n = 2.231 SR). The variables recorded were: number of SR and protocols in any CRG, authors and clusters of secondary research, dates (late review and update), type of study, critical review of the SR and conclusions. RESULTS: Nine published SR about neuropaediatrics: the Epilepsy Group (24 SR), the Neuromuscular Disease Group (16), the Neonatal Group (16), the Developmental, Psychosocial and Learning Problems Group (10), the Pain, Palliative Care and Supportive Care Group (4), the Movement Disorders Group (3), the Injuries Group (3), the Infectious Disease Group (3) and the Acute Respiratory Infections Group (2). The three main thematic areas were treatment of epilepsy (pharmacologic and non-pharmacologic), neonatal neurology (mainly intraventricular haemorrhage and perinatal asphyxia) and miscellanea (autism spectrum disorder, headache, cerebral palsy, myasthenia gravis, Guillain-Barre syndrome, Bell's palsy and bacterial meningitis). All the SR were about treatment interventions. CONCLUSIONS: Paediatric neurology SR are infrequent (3.6% of the 2.231 SR published in CC), and helps an evidence-based decision-making in a few areas: pharmacologic treatment of epilepsy, management of intraventricular haemorrhage of preterm infants and bacterial meningitis. Many therapies in paediatric neurology persist with no supporting evidence, and we detected no SR about important neurological issues in childhood as attention-deficit hyperactivity disorder, mental retardation and hypotonia.
Abstract: BACKGROUND: Several body positions other than standard supine position have been used in patients undergoing intensive care for reducing the incidence of pressure ulcers of the skin, contractures or ankylosis and for improving the patient's well being. In patients from different age groups undergoing mechanical ventilation (MV), it has been observed that particular positions, such as prone position, may improve some respiratory parameters. Benefits from these positions have not been clearly defined in critically ill newborns who may require mechanical ventilation for extended periods of time. OBJECTIVES: To assess the effects of different positioning of newborn infants receiving MV on short term respiratory outcomes and complications of prematurity. SEARCH STRATEGY: Databases searched (up to May 2006) were the Oxford Database of Perinatal Trials, CINAHL, MEDLINE, EMBASE and Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 2, 2006). Hand searches of proceedings of the Society for Pediatric Research from 1990 to May 2006 were used to identify unpublished studies. SELECTION CRITERIA: Randomised or quasi randomised clinical trials comparing different positions in newborns receiving mechanical ventilation. DATA COLLECTION AND ANALYSIS: Three independent and unblinded reviewers assessed the trials for inclusion in the review and extracted the data. Data were double-checked and entered into the Review Manager software. MAIN RESULTS: Eleven trials involving 206 infants were included in this review. Several positions were compared: prone vs. supine, prone vs. lateral right, lateral right vs. supine, lateral left vs. supine, lateral right vs. lateral left and good lung dependent vs. good lung uppermost. Apart from one of the two studies that compared lateral right vs. lateral left positions, and one comparing prone vs. supine position, all the included studies had a crossover design. Comparing prone vs. supine position, an increase in arterial P02 in the prone position of between 2.75 and 9.72 mm Hg (95% CI) was observed (one trial). When % hemoglobin oxygen saturation was measured with pulse oximetry, improvement in the prone position was from 1.18 to 4.36% (typical effect based on four trials). In addition, there was a slight improvement in the number of episodes of desaturation. It was not possible to establish whether this effect remained once the intervention was stopped. Negative effects from the interventions were not described, although this issue was not studied in sufficient detail. Effects of position on other outcomes were not statistically significant; however, either positive nor negative effects cannot be excluded considering the small numbers that were studied. AUTHORS' CONCLUSIONS: The prone position was found to slightly improve the oxygenation in neonates undergoing mechanical ventilation. However, we found no evidence concerning whether particular body positions during mechanical ventilation of the neonate are effective in producing sustained and clinically relevant improvements.
Abstract: The Cochrane Collaboration provides growing and readily accessible resources to help ensure that medical decision-making is based on detailed, methodical, and up-to-date reviews of the best available evidence. We analyzed systematic reviews in the field of pediatric cardiology published by the Cochrane Collaboration's 50 Collaborative Review Groups. We found a total of 20 systematic reviews: 13 published by the Cochrane Neonatal Group, 6 by the Cochrane Heart Group, and 1 by the Cochrane Peripheral Vascular Disease Group. Systematic reviews in pediatric cardiology appear infrequently. They only concern evidence-based decision-making in the therapeutic management of patent ductus arteriosus and arterial hypotension in preterm infants, and in the management of children with Kawasaki disease. The quality of the clinical trials contained in the systematic reviews of acute rheumatic fever or obesity in children is limited. Consequently, the reviewers' conclusions provide an inadequate basis for inferring probable effects in clinical practice. In pediatric cardiology, many therapies continue to be used without supportive evidence. We found no systematic reviews of important cardiologic topics in childhood such as heart failure, shock, hypertension, congenital cardiopathy, and arrhythmia. Clinical practice guidelines complement systematic reviews, which can recommend only strategies that are supported by strong evidence or suggest further research when scientific evidence is inadequate.
Abstract: OBJECTIVE: To evaluate the clinical significance of renal calyceal microlithiasis (RCM) in children with idiopathic hypercalciuria (IHC). MATERIAL AND METHODS: RCM is a renal echographic finding defined as the presence of hyperechogenic spots < 3 mm in diameter in the renal calyces. These spots have been associated with the presence of nephrourological symptoms in children and are considered to represent a stage prior to urolithiasis. We reviewed the medical records of 103 children (63 girls, 40 boys; age range 1-14 years; mean age 6.57 years) referred for various complaints who had IHC. Renal echography was routinely performed. At diagnosis, 52 children had RCM, 35 showed normal echography, 14 had calculi and two presented nephrocalcinosis. A long-term follow-up study was carried out to compare the clinical manifestations, analytic data and renal echographic findings of patients with RCM and those with normal echography. RESULTS: The clinical manifestations and the results of biochemical studies did not differ significantly between the two groups. Renal sonographic findings during the follow-up period revealed that, of patients with initial RCM, 35 showed normalized sonographic findings, two developed calculi and 36 developed recurrent RCM. Of the children with normal initial echography, 17 developed RCM and three developed calculi. The risk of developing lithiasis was less in children with RCM than in those with normal initial renal echography (0.04 vs 0.09), the relative risk being 0.45 (95% CI 0.08-2.55). The clinical and analytic differences between the group of 14 children with initial lithiasis and the other two groups previously described were also analyzed and no significant differences were found. An ongoing echographic study of these patients showed that the echograph was normalized in 10 children at some point or other, while seven developed RCM (four unilateral, three bilateral). In 13 cases the lithiasis reappeared, and the relative risk of recurrent lithiasis compared with those who initially showed no lithiasis was 16.16 (CI 95% 6.81-38.31). CONCLUSION: Our results indicate that up to 85% of children with IHC presented RCM in follow-up sonographies. This echographic finding, which may appear and disappear at different points during follow-up, does not seem to indicate an increased risk of lithiasis.
Abstract: A hepatodiaphragmatic interposition of the colon, known as Chilaiditi's sign, is usually discovered by chance during the study of another event, given that its presentation is normally asymptomatic. When this finding is accompanied by clinical symptoms, either intermittent or persistent, it is known as Chilaiditi syndrome. It may be associated with intestinal obstruction due to twisting. The association of Chilaiditi syndrome and transverse colon volvulus is exceptional. To date only three cases have been reported, all in adult males. Among the common predisposing factors were anatomical alterations of the intestine such as elongation of the colon and a history of prior abdominal surgery. The clinical symptoms were due to the intestinal obstruction. We present the first description in the paediatric population of an association of transverse colon volvulus and Chilaiditi syndrome whose predisposing factors, clinical symptoms and treatment differed from those reported in the non-paediatric cases published to date.
Abstract: BACKGROUND: Many treatment errors in neonatal intensive care units are caused by the need to carry out a sequence of calculations to determine the dose and dilution of the drugs used. OBJECTIVES: To help in this task, we designed a spreadsheet (Neodosis) that helps clinicians and nurses to calculate the doses and standardize the dilutions of some of the drugs most commonly used in resuscitation and neonatal intensive care units. The aim of this study was to verify the usefulness and reliability of this software package. METHODS: A randomized, cross-over, controlled trial was conducted through simulated clinical cases in which the number of errors in the prescription data and the amount of time spent in making calculations, with and without the program, were evaluated. Fifty-four tests were performed by pediatricians, third- and fourth-year pediatric residents, and nurses. RESULTS: Without computer support, all three groups made errors (residents, pediatricians and nurses in descending order). When Neodosis was used, all the medical staff made significantly fewer errors. The greatest reduction was found in errors made by pediatric residents: minor errors decreased from 16 % to 2 % and major errors from 1.6 % to zero. When using the spreadsheet, the time spent by all groups in making the calculations was reduced by between one-third and one-half. CONCLUSIONS: The tests performed with simulated clinical cases revealed that the number of errors made by the healthcare personnel who participated in this study was not inconsiderable. The use of Neodosis helped physicians and nurses to make markedly fewer errors and also saved them time.
Abstract: INTRODUCTION: Pupil asymmetry is an alarm signal which should lead to investigation to rule out severe underlying neurological disorders. Among its causes are tumors, aneurysms and hernia of the uncus. The differential diagnosis should also include other conditions such as Adie's tonic pupil, the Pourfour de Petit syndrome and local disorders such as closed angle glaucoma or segmental spasm of the iris dilator muscle. In practice however, exposure to mydriatic substance is one of the commonest causes. Another cause of this sign is benign episodic unilateral mydriasis. This uncommon condition has been defined as an isolated benign cause of pupil asymmetry. The underlying physiopathology is not always clear and may involve either parasympathetic deficiency or sympathetic hyperactivity affecting the iris. Usually related to migraine, some authors classify it as a limited form of ophthalmoplegic migraine, although some cases have been described with no accompanying headache. CLINICAL CASE: We describe a case of benign episodic unilateral mydriasis in a six-year-old girl who presented with intermittent episodes of pupil asymmetry with no other neurological symptoms. CONCLUSION: We underline the rarity of this condition in children with no simultaneous headache.
Abstract: To investigate the long-term outcome of the frequency-dysuria syndrome (FDS) with hypercalciuria (HCU), 19 children (15 girls and 4 boys; age range 15 months to 10 years) who presented with FDS alone (N=9) or with other associated clinical features (N=10; 6 with gross hematuria, 3 with microscopic hematuria and 1 with abdominal pain) were followed-up over 720 patient-months. Calcium loading test indicated absorptive HCU in 12 patients, renal HCU in 2, and in 5 the test was inconclusive. All patients were treated with a standard protocol after diagnosis. During follow-up, FDS recurred in 4 children, gross hematuria in 3, lumbar pain in 5, and 7 children developed urolithiasis within 3 to 60 months. The latter 7 children (4 with absorptive HCU and 1 with renal HCU) tended to be older than the other 12 (7.14 vs 5.08 years; p=0.11) and required a longer time to normalize urinary calcium excretion (16 vs 7 months; p <0.01). The initial urinary calcium excretion was similar between the patients with and those without stones (5.53 vs 5.6 mg/kg/d). In all other parameters measured, there were no statistically significant differences between the patients who initially presented with FDS alone and those with FDS accompanied with other urinary symptoms. We conclude that HCU and FDS in children can vary considerably in the clinical mode of presentation as well as its sequels. Significant risk for urolithiasis burdens the children who require a longer time (>12 months) to normalize their hypercalciuria.
Abstract: OBJECTIVE: The purpose of this study was to investigate the clinical manifestations, outcome and risk factors for urolithiasis of untreated idiopathic hypercalciuria (IH) in children. PATIENTS AND METHODS: During a 5 year period, all children with hematuria, lower urinary tract symptoms (LUS), or abdominal pain who were observed to have IH (urinary calcium > 4 mg/kg/day) were included in the study. The relationship between some variables (age, gender, hypercalciuria subtype, and clinical features) and urolithisis was analyzed. RESULTS: We studied 76 children with IH (9 with renal IH, 49 with absorptive IH and 18 with undetermined IH). Hematuria (46%), LUS (27.6%), lumbar pain (22%) and abdominal pain (15.7%) were the most common initial symptoms. We found a significant difference between age and clinical presentation. LUS were found more frequently in young children (46.4%) and flank pain in older patients (47.8%). Hematuria was age dependent. Eighteen (23%) patients developed urolithiasis. These 18 patients tended to be older (9.3 vs 6 years) than the other 58 children. Age at diagnosis presented a linear relationship with the development of urolithiasis (p < 0.001). Significant relative risk for urolithiasis (4.3) was found in those children who initially presented with lumbar pain. In all other parameters measured (calciuria, uricosuria, oxaluria, citraturia) and clinical characteristics analyzed, there were no statistically significant differences between those with and without stones. CONCLUSIONS: An age-dependent clinical pattern can be established for children with hypercalciuria. Significant risk for urolithiasis accompanies those children who are diagnosed later (> 9 years) and who initially present with lumbar pain.
Abstract: Mucopolysaccharide storage disease (MPS) presents clinically with a broad spectrum of abnormalities, among which cardiovascular involvement has been described. The echocardiographic findings have recently been reported for the various types of MPS. Among these, asymmetric septal hypertrophy (ASH) has been documented. We present a case of a 9-year-old girl suffering from type I MPS, atypical variant, with echocardiographic signs of ASH. She was given a bone marrow transplant after which the hypertrophic cardiomyopathy regressed.
Abstract: Idiopathic infantile arterial calcification (IIAC) is a rare disease of unknown etiology, which causes widespread arterial calcification and usually leads to early death from coronary arterial occlusion. Periarticular calcification has been reported in some cases. Two new cases are reported. In addition to the usual features of the disease, one was found to have ear-lobe calcification and the other an aortic aneurysm and coarctation of the aorta. Therapy with diphosphonate was apparently successful in one patient.
Abstract: Very few cases of children with cardiac tamponade from central venous catheterization have been reported. Four cases are described. In 3 patients the diagnosis was suspected, a pericardiocentesis was performed and they survived. The other patient died and, at autopsy, diagnosis was made. The available literature on cardiac tamponade from central venous catheters in children is reviewed, preventive measures are considered in detail and the need for a high index of suspicion is highlighted.
