Search

Background: In international cooperation projects that are carried out in less developed and developing countries, a large number of children with disabilities present cerebral palsy (CP). Orthopaedic disorders are frequent complications associated with this disorder. Their prevention and early intervention are essential to achieve an appropriate therapeutic approach for children with PC and to improve their quality of life. Objective: To describe the treatment approach that is currently used in international cooperation projects for the rehabilitation management of the orthopaedic disorders in children with cerebral palsy. Methods: This is an observational, descriptive, cross-sectional study, carried out by means of an online questionnaire to professionals in the field of Physiotherapy and Rehabilitation working in international cooperation projects. The inclusion criteria were professionals working in the rehabilitation field in development aid, humanitarian action or emergency projects that provided rehabilitation services, working with children with cerebral palsy from 0 to 18 years old. Results: Ninety-eight questionnaires were analysed. The average age of the participants was 33.2 years, they were mainly working in development cooperation projects (83.33%) that were implemented in rehabilitation centres and through community-based rehabilitation services (60%). The projects were located in countries all over the world but mainly on the Asian continent (71.4%). Physiotherapists and orthopaedic technicians (72.22%) were the main professionals working in these projects, followed by occupational therapists and social workers (55.56%). The results indicated that the orthopaedic disorders were very frequent in the sample (66.67%), with hip subluxation (50%), scoliosis (77.78%), kyphosis (61.1%), clubfoot (88.7%) and varus foot (61.11%) standing out. The most commonly used treatment approaches were positioning (88.89%) and the Bobath concept (83.33%). The technical aids that were used by the professionals were ankle foot orthosis (AFO) (94.44%), bracing (66.67%), standing frames (83.33%), moulded seats (100%), corner seats (93.75%) and adapted seats (92.85%). Conclusions: In international cooperation projects, the rehabilitation treatment of children with cerebral palsy is based on a holistic approach. This is reflected in the interventions that are carried out to treat their orthopaedic disorders and in locally produced devices, awareness raising and community education. However, the professionals surveyed considered that the aids or orthoses used are insufficient in the treatment and prevention of orthopaedic disorders in cerebral palsy.

Objective The study aimed to describe the cases of neurological disease related to the outbreak of enterovirus (EV) in three regions in Northern Spain during 2016. Materials and Methods Multicenter retrospective observational study. Clinical, radiological, and microbiological data were analyzed from patients younger than 15 years with confirmed EV-associated neurological disease admitted to 10 hospitals of Asturias, Cantabria, and Castile and Leon between January 1 and December 31, 2016. Results Fifty-five patients were included. Median age was 24 months (interquartile range = 18.5 months). Fifteen patients were classified as aseptic meningitis (27.3%). In total, 37 cases presented brainstem encephalitis (67.3%), 25 of them due to EV-A71 with excellent prognosis (84.6% asymptomatic 2 months following the onset). Three cases of acute flaccid myelitis (5.5%) by EV-D68 were reported and presented persistent paresis 2 months following the onset. Microbiological diagnosis by reverse transcriptase polymerase chain reaction was performed in all cases, finding EV in cerebrospinal fluid in meningitis, but not in brainstem encephalitis and acute flaccid myelitis, where EV was found in respiratory or rectal samples. Step therapy was administrated with intravenous immunoglobulin (IVIG; 32.7%), methylprednisolone (10%), and plasmapheresis (3.6%). Four patients received fluoxetine (7.3%). Twenty patients needed to be admitted to pediatric intensive care unit (36.4%). Conclusion Clinical, microbiological, and radiological diagnosis is essential in outbreaks of EV neurological disease, taking into account that it can be difficult to identify EV-A71 and EV-D68 in CSF, requiring throat or rectal samples. There is not specific treatment to these conditions and the efficacy and understanding of the mechanism of action of immune-modulatory treatment (IVIG, corticosteroids, and plasmapheresis) is limited.

Purpose of Review This review presents an in-depth overview of the sleep–wake phenotype of patients with cirrhosis, together with available pharmacological and non-pharmacological treatment strategies. A set of simple, practical recommendations is also provided. Recent Findings The understanding of the pathophysiology of sleep disorders in this patient population has improved over the past decade, especially in relation to the interplay between homeostatic and circadian sleep regulation. In addition, new tools have been utilised for both screening and in-depth investigation of the sleep–wake profile of these patients. Finally, a number of studies have evaluated the efficacy of novel treatment strategies, often with encouraging results. Summary Since sleep disturbances are common in patients with cirrhosis, more so than in patients with other chronic diseases of similar severity, their assessment should become routine hepatological practice, along with the initiation of adequate treatment.

Treatment of congenital cytomegalovirus infection is mandatory in cases with severe systemic and/or neurological involvement. However, some patients are paucisymptomatic, with very subtle systemic manifestations and/or minimal brain alterations. Current international guidelines do not clearly state whether these children should be treated, and this decision is not straightforward for clinicians. Of a small series of six infants with congenital cytomegalovirus infection admitted to our neonatal unit between 2015 and 2019, half showed paucisymptomatic neurological manifestations. In these cases, the determination of s2-microglobulin in cerebrospinal fluid and magnetic resonance imaging aided in the decision-making concerning the therapeutic approach to follow.

Chronic leg ulcers can have a major impact on the quality of life of patients. These wounds can be complex and hard to heal, as several factors may affect the outcome. Underlying conditions, bacterial growth and excess moisture may prevent wounds from healing. We describe the case of a patient with known chronic venous disease, who was admitted to our hospital for several complex, irregular and infected chronic venous ulcers in his lower legs. The management was frustrating for several months, until we began to use methacrylate powder dressing for his hard-to-heal wounds.

BACKGROUND & AIMS: Sleep preparation/onset are associated with peripheral vasodilatation and a decrease in body temperature. The hyperdynamic syndrome exhibited by patients with cirrhosis may impinge on sleep preparation, thus contributing to their difficulties falling asleep. The aim of this study was the assessment of skin temperature, in relation to sleep-wake patterns, in patients with cirrhosis. METHODS: Fifty three subjects were initially recruited, and 46 completed the study. Of the final 46, twelve were outpatients with cirrhosis, 13 inpatients with cirrhosis, 11 inpatients without cirrhosis and 10 healthy volunteers. All underwent baseline sleep-wake evaluation and blood sampling for inflammatory markers and morning melatonin levels. Proximal/distal skin temperature and their gradient (DPG) were recorded for 24 hours by a wireless device. Over this period subjects kept a sleep-wake diary. RESULTS: Inpatients with cirrhosis slept significantly less well than the other groups. Inpatients and outpatients with cirrhosis had higher proximal temperature and blunted rhythmicity compared to the other groups. Inpatients with/without cirrhosis had higher distal temperature values and blunted rhythmicity compared to the other groups. Inpatients and outpatients with cirrhosis had significantly lower DPG values compared to the other groups, and DPG reached near-zero values several hours later. Significant correlations were observed between temperature and sleep-wake variables and inflammatory markers. CONCLUSIONS: Alterations of distal/proximal skin temperature, their gradient and their time-course were observed in patients with cirrhosis, which may contribute to their sleep disturbances. This article is protected by copyright. All rights reserved.

Little is known about the pathogenesis of cerebral sinovenous thrombosis (CSVT) in the neonate. Although thrombophilia has been described as increasing the risk of CSVT in adults, it remains controversial in pediatric patients, and prospective case–control studies regarding neonatal CSVT are lacking. From 2008 to 2017, all 26 consecutive newborn infants ≥35 weeks of gestation diagnosed with neonatal CSVT, and their mothers, were tested for factor V Leiden (FV) G1691A, FII G20210A, and methylenetetrahydrofolate reductase C677T (MTHFR C677T) mutations. Eighty-five mother–infant pairs were recruited as controls. All infants except 1 with CSVT were suspected due to clinical symptoms, mainly seizures (22/25). Magnetic resonance imaging was performed in 24/26 infants. Heterozygous FV G1691A, FII G20210A, and homozygous MTHFR C677T mutations were present in 1/26, 3/26, and 3/20 infants with CSVT, respectively. FII (odds ratio: 10.96; 95% confidence interval [CI]: 1.09-110.35) and male sex (3.93; 95% CI: 1.43-10.76) were associated with CSVT. When FII G20210A analysis was adjusted for sex, the OR for FII G20210A was 6.70 (95% CI: 0.65-69.22). No differences were found for FV G1691A or homozygous MTHFR mutations between neonates with CSVT and their mothers, compared to controls.

Gender is a crucial determinant of life span, but little is known about gender differences in free radical homeostasis and inflammatory signaling. The aim of the study was to determine gender-related differences concerning oxidative stress and inflammatory signaling of healthy neonates and mothers. Fifty-six mothers with normal gestational course and spontaneous delivery were selected. Blood samples were collected from the mother (at the beginning of delivery and start of expulsive period) and from neonate (from umbilical cord vein and artery). The mothers of girls featured a higher total antioxidant status and lower plasma hydroperoxides than the mother of boys. Regarding the neonates, the girls featured a higher total antioxidant status and lower plasma membrane hydroperoxides in umbilical cord artery together with higher catalase, glutathione peroxidase, and superoxide dismutase activities. Lower levels of interleukin 6, tumor necrosis factor alpha, and prostaglandin E2 were observed in the mothers of girls and higher level of soluble tumor necrosis factor receptor II. In the neonates, lower levels of interleukin 6 and tumor necrosis factor alpha were observed in umbilical artery and higher soluble tumor necrosis factor receptor II in umbilical cord vein and artery of girls. An association between gender, oxidative stress, and inflammation signaling exists, leading to a renewed interest in the neonate’s sex as a potential risk factor to several alterations.

UNLABELLED Hyperammonaemia/mild hepatic encephalopathy (HE) can be simulated by the oral administration of a so-called amino acid challenge (AAC). This study sought to assess the effects of the AAC alone and in combination with either ammonia-lowering [L-ornithine-L-aspartate (LOLA)] or vigilance-enhancing medication (caffeine). Six patients with cirrhosis (5 males; 61.3 ± 9.2 years; 5 Child A, 1 Child B) and six healthy volunteers (5 males; 49.8 ± 10.6 years) were studied between 08:00 and 19:00 on Monday of three consecutive weeks. The following indices were obtained: hourly capillary ammonia, hourly subjective sleepiness, paper & pencil/computerized psychometry and wake electroencephalography (EEG) at 12:00, i.e. at the time of the maximum expected effect of the AAC. RESULTS On average, patients had worse neuropsychological performance and slower EEG than healthy volunteers in all conditions but differences did not reach significance. In healthy volunteers, the post-AAC increase in capillary ammonia levels was contained by both the administration of LOLA and of caffeine (significant differences between 10:00 and 14:00 h). The administration of caffeine also resulted in a reduction in subjective sleepiness and in the amplitude of the EEG on several frontal/temporal-occipital sites (p

The article Assessment and Management of Sleep Disturbance in Cirrhosis, written by Chiara Formentin, Maria Garrido, and Sara Montagnese, was originally published electronically on the publisher’s internet portal (currently SpringerLink) on 13 February 2018 without open access.

Background Providing structured information for the understanding of hepatic encephalopathy (HE) might be relevant to the prevention and management of the syndrome. The aim of our study was to design a brief, structured educational intervention and evaluate its usefulness in preventing HE-related hospitalisation over time. Methods Thirty-nine cirrhotic outpatients with a history of HE were enrolled and randomly assigned to an intervention (group A; n=20) or control group (group B; n=19). All of them underwent evaluation of HE (clinical and quantitative neuropsychiatric assessment) and completed the Questionnaire on the Awareness of Encephalopathy. A 15 min educational session was then provided to patients in group A, including basic information on the pathophysiology, hygienic and medical management of HE. Results No demographic/clinical differences were observed at baseline between the two groups. Similarly, there were no significant differences in HE-related information available at baseline between the two groups; knowledge of HE was limited in both. The intervention was highly effective in increasing patients’ understanding of treatment of the condition (from 5% to 80%). The educational intervention also reduced the risk of developing an episode of HE over a period of 12 months. Conclusion The educational intervention confirmed the poor knowledge of patients with previous HE about their condition, served as a tool to increase patients’ awareness, and minimised HE-related readmission rates over a period of 1 year.

BackgroundApple allergy manifests as two main groups of clinical entities reflecting different patterns of allergen sensitization: oral allergy syndrome (OAS) and generalized symptoms (GS).ObjectiveWe analysed the sensitization profile to a wide panel of different components of food allergens (rMal d 1, Mal d 2, rMal d 3, rMal d 4, rPru p 3, rBet v 1 and Pho d 2) for a population of Mediterranean patients with OAS and GS to apple.MethodsPatients (N = 81) with a history of apple allergy that could be confirmed by positive prick-prick test and/or double-blind-placebo-controlled food challenge (DBPCFC), were included. Skin prick test (SPT) and ELISA were performed using a panel of inhalant, fruit and nut allergens. ELISA and ELISA inhibition studies were performed in order to analyse the sensitization patterns.ResultsThirty-five cases (43.2%) had OAS and 46 (56.8%) GS. SPT showed a significantly higher number of positive results with peach, cherry and hazelnut in those with GS. ELISA showed a significantly high percentage of positive cases to rMal d 3, rMal d 4, rPru p 3 and Pho d 2 in patients with OAS and GS compared to controls, and to rBet v 1 in patients with OAS vs controls and between OAS and GS patients. Three different patterns of recognition were detected: positive to LTP (rMal d 3 or rPru p 3), positive to profilin (rMal d 4 and Pho d 2), or positive to both. There were also patients with rMal d 1 recognition who showed cross-reactivity to rBet v 1.ConclusionIn an apple allergy population with a high incidence of pollinosis different patterns of sensitization may occur. LTP is most often involved in those with GS. Profilin, though more prevalent in patients with OAS, has been shown to sensitise patients with both types of symptoms.

Objective. The main objective of this study was to systematically review all the scientific studies that analyse the effectiveness of mud pack therapy on patients diagnosed with knee OA. Methods. One hundred and fifteen publications were identified through an electronic and manual search and 20 of those studies were chosen based on the inclusion criteria: randomized clinical trials, systematic reviews or meta-analyses whose objective was to analyse the effect of mud pack therapy on perceived pain, function and quality of life, with a sample size of 520 subjects, published since 2000 and showing conclusive results. Results. In the studies that met the inclusion criteria, 12 analyse functionality, 17 perceived pain, 5 quality of life and all showed a significant improvement in the three analysed variables. The methodological quality of the studies had a moderate risk of bias. Conclusion. Mud pack therapy is considered an alternative and effective therapy in the clinical management of knee OA. Studies with better methodology are needed to prove its scope.

A 100-gram oral glucose tolerance test (OGTT) was performed on a selected group of normal women at each trimester of pregnancy to establish reference values for hyperglycemia and hypoglycemia. Ninety three OGTT were performed in the first trimester, 121 in the second trimester, and 98 in the last trimester. The fasting serum glucose did not differ significantly between the trimesters. The values at 60 and 120 minutes were significantly different for the fifth, fiftieth, and ninety-fifth percentiles between each trimester of pregnancy. For the 180-minute readings, the fifth and fiftieth percentiles were not significantly different between the first and second trimester of pregnancy, but the results of the third trimester were significantly higher than those of the other trimesters. The mean fasting insulinemia remained relatively constant during pregnancy. Insulin response to OGTT increased during the progression of the pregnancy. The interpretation of the glucose tolerance tests during pregnancy, either to detect gestational diabetes or hypoglycemia, should take these physiologic changes into account.