The VASCERN-VASCA working group diagnostic and management pathways for lymphatic malformations

Lymphatic malformations (LMs) are developmental defects of lymphatic vessels. LMs are histologically benign lesions, however, due to localization, size, and unexpected swelling, they may cause serious complications that threaten vital functions such as compression of the airways. A large swelling of the face or neck may also be disfiguring and thus constitute a psychological strain for patients and their families. LMs are also highly immunologically reactive, and are prone to recurrent infections and inflammation causing pain as well as chronic oozing wounds.The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) is dedicated to gathering the best expertise in Europe. There are only few available guidelines on management and follow up of LMs, which commonly focus on very specific situations, such as head and neck LM (Zhou et al., 2011). It is still unclear, what constitutes an indication for treatment of LMs and how to follow up the patients. The Vascular Anomalies Working Group (VASCA-WG) of VASCERN decided to develop a diagnostic and management pathway for the management of LMs with a Nominal Group Technique (NGT), a well-established, structured, multistep, facilitated group meeting technique used to generate consensus statements. The pathway was drawn following 2 face-to-face meetings and multiple web meetings to facilitate discussion, and by mail to avoid the influence of most authoritative members.The VASCA-WG has produced this opinion statement reflecting strategies developed by experts and patient representatives on how to approach patients with lymphatic malformations in a practical manner; we present an algorithmic view of the results of our work.Peer reviewe

The VASCERN-VASCA working group diagnostic and management pathways for severe and/or rare infantile hemangiomas

The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN), is dedicated to gathering the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular dis-eases. Infantile Hemangiomas (IH) are benign vascular tumors of infancy that rapidly growth in the first weeks of life, followed by stabilization and spontaneous regression. In rare cases the extent, the localization or the number of lesions may cause severe complications that need specific and careful management. Severe IH may be life-threatening due to airway obstruction, liver or cardiac failure or may harbor a risk of functional impairment, severe pain, and/or significant and permanent disfigurement. Rare IHs include syndromic variants associated with extracutaneous abnormalities (PHACE and LUMBAR syndromes), and large segmental hemangiomas. There are publications that focus on evidence-based medicine on propranolol treatment for IH and consensus state -ments on the management of rare infantile hemangiomas mostly focused on PHACES syndrome. The Vascular Anomalies Working Group (VASCA-WG) decided to develop a diagnostic and management pathway for severe and rare IHs with a Nominal Group Technique (NGT), a well-established, structured, multistep, facilitated group meeting technique used to generate consensus statements. The pathway was drawn following two face-to-facePeer reviewe

The VASCERN-VASCA Working Group Diagnostic and Management Pathways for Venous Malformations.

UNLABELLED To elaborate expert consensus patient pathways to guide patients and physicians toward efficient diagnostics and management of patients with venous malformations. METHODS VASCERN-VASCA (https://vascern.eu/) is a European network of multidisciplinary centers for Vascular Anomalies. The Nominal Group Technique was used to establish the pathways. Two facilitators were identified: one to propose initial discussion points and draw the pathways, and another to chair the discussion. A dermatologist (AD) was chosen as first facilitator due to her specific clinical and research experience. The draft was subsequently discussed within VASCERN-VASCA monthly virtual meetings and annual face-to-face meetings. RESULTS The Pathway starts from the clinical suspicion of a venous type malformation (VM) and lists the clinical characteristics to look for to support this suspicion. Strategies for subsequent imaging and histopathology are suggested. These aim to inform on the diagnosis and to separate the patients into 4 subtypes: (1) sporadic single VMs or (2) multifocal, (3) familial, multifocal, and (4) combined and/or syndromic VMs. The management of each type is detailed in subsequent pages of the pathway, which are color coded to identify sections on (1) clinical evaluations, (2) investigations, (3) treatments, and (4) associated genes. Actions relevant to all types are marked in separate boxes, including when imaging is recommended. When definite diagnoses have been reached, the pathway also points toward disease-specific additional investigations and recommendations for follow up. Options for management are discussed for each subtype, including conservative and invasive treatments, as well as novel molecular therapies. CONCLUSION The collaborative efforts of VASCERN-VASCA, a network of the 9 Expert Centers, has led to a consensus Diagnostic and Management Pathways for VMs to assist clinicians and patients. It also emphasizes the role of multidisciplinary expert centers in the management of VM patients. This pathway will become available on the VASCERN website (http://vascern.eu/)

The effect of prenatal balanced energy and protein supplementation on gestational weight gain: An individual participant data meta-analysis in low- and middle-income countries

Background AU Understanding: Please confirm that all heading levels are represented correctly the effects of balanced energy and protein (BEP): supplements on gestational weight gain (GWG) and how the effects differ depending on maternal characteristics and the nutritional composition of the supplements will inform the implementation of prenatal BEP interventions. Methods and findings Individual participant data from 11 randomized controlled trials of prenatal BEP supplements (N = 12,549, with 5,693 in the BEP arm and 6,856 in the comparison arm) in low- and middle-income countries were used. The primary outcomes included GWG adequacy (%) and the estimated total GWG at delivery as continuous outcomes, and severely inadequate (125% adequacy) as binary outcomes; all variables were calculated based on the Institute of Medicine recommendations. Linear and log-binomial models were used to estimate study-specific mean differences or risk ratios (RRs), respectively, with 95% confidence intervals (CIs) of the effects of prenatal BEP on the GWG outcomes. The study-specific estimates were pooled using meta-analyses. Subgroup analyses were conducted by individual characteristics. Subgroup analyses and meta-regression were conducted for study-level characteristics. Compared to the comparison group, prenatal BEP led to a 6% greater GWG percent adequacy (95% CI: 2.18, 9.56; p = 0.002), a 0.59 kg greater estimated total GWG at delivery (95% CI, 0.12, 1.05; p = 0.014), a 10% lower risk of severely inadequate GWG (RR: 0.90; 95% CI: 0.83, 0.99; p = 0.025), and a 7% lower risk of inadequate GWG (RR: 0.93; 95% CI: 0.89, 0.97; p = 0.001). The effects of prenatal BEP on GWG outcomes were stronger in studies with a targeted approach, where BEP supplements were provided to participants in the intervention arm under specific criteria such as low body mass index or low GWG, compared to studies with an untargeted approach, where BEP supplements were provided to all participants allocated to the intervention arm. Conclusions Prenatal BEP supplements are effective in increasing GWG and reducing the risk of inadequate weight gain during pregnancy. BEP supplementation targeted toward pregnant women with undernutrition may be a promising approach to delivering the supplements

Prevention of non-communicable disease in a population in nutrition transition: Tehran Lipid and Glucose Study phase II

<p>Abstract</p> <p>Background</p> <p>The Tehran Lipid and Glucose Study (TLGS) is a long term integrated community-based program for prevention of non-communicable disorders (NCD) by development of a healthy lifestyle and reduction of NCD risk factors. The study begun in 1999, is ongoing, to be continued for at least 20 years. A primary survey was done to collect baseline data in 15005 individuals, over 3 years of age, selected from cohorts of three medical heath centers. A questionnaire for past medical history and data was completed during interviews; blood pressure, pulse rate, and anthropometrical measurements and a limited physical examination were performed and lipid profiles, fasting blood sugar and 2-hours-postload-glucose challenge were measured. A DNA bank was also collected. For those subjects aged over 30 years, Rose questionnaire was completed and an electrocardiogram was taken. Data collected were directly stored in computers as database software- computer assisted system. The aim of this study is to evaluate the feasibility and effectiveness of lifestyle modification in preventing or postponing the development of NCD risk factors and outcomes in the TLGS population.</p> <p>Design and methods</p> <p>In phase II of the TLGS, lifestyle interventions were implemented in 5630 people and 9375 individuals served as controls. Primary, secondary and tertiary interventions were designed based on specific target groups including schoolchildren, housewives, and high-risk persons. Officials of various sectors such as health, education, municipality, police, media, traders and community leaders were actively engaged as decision makers and collaborators. Interventional strategies were based on lifestyle modifications in diet, smoking and physical activity through face-to-face education, leaflets & brochures, school program alterations, training volunteers as health team and treating patients with NCD risk factors. Collection of demographic, clinical and laboratory data will be repeated every 3 years to assess the effects of different interventions in the intervention group as compared to control group.</p> <p>Conclusion</p> <p>This controlled community intervention will test the possibility of preventing or delaying the onset of non-communicable risk factors and disorders in a population in nutrition transition.</p> <p>Trial registration</p> <p>ISRCTN52588395</p

Structure of a Hollow-Cone Spray With and Without Combustion

A hollow-cone spray with a nominal cone angle of 30 degrees from a pressure-swirl fuel atomizer was used in a swirl-stabilized combustor. The combustor is circular in cross section, with a swirl plate and fuel nozzle axis coinciding with the axes of the chamber. kerosene is injected upward inside the chamber from the fuel nozzle. Separate swirl and dilution air flows are distributed into the chamber that pass through honeycomb flow straighteners and screens. A calculated swirl number of 1.5 is generated with the design swirl plate exit air velocity of 30 degrees with respect to the chamber axis. Effects of swirl and dilution air flow rates on the shape and stability of the flame are investigated. A Phase Doppler Particle Analyzer (PDPA) is used to measure drop size, mean and rms values of axial drop velocity, fuel volume flux, drop velocity and size distributions, and size-classified drop velocity profiles for two cases of with and without combustion and at six different axial locations from the nozzle. For the no-combustion case all air and fuel flow rates were kept at the same values as the combusting spray condition. Results for mean axial drop velocity profiles indicate widening of the spray, with slight increase in the magnitudes of the peak drop velocities due to combustion. Root mean square (RMS) values of drop velocity fluctuations decrease due to a combination of increase in gas kinematic viscosity and elimination of small drops at high temperatures. Sauter mean diameter (SMD) radial profiles at all axial locations increase with combustion due to preferential burning of small drops. Fuel volume flux profiles indicate negligible drop vaporization and/or burning up to a distance of 25mm from the nozzle. Velocity number distributions at different radial points for without combustion at an axial distance of 55mm from the atomizer are symmetric in shape only close to the peak of the mean drop velocity and show a bimodal shape around the maximum mean drop axial velocity gradient. Corresponding number distributions for the combustion case are fairly symmetric and quite different in behavior at all radial positions. Size-classified drop velocity profiles are also plotted and discussed.</jats:p

Evaluation of repetitive stimulation test (RST) in 30 patients with Myasthenia Gravis, who were previously confirmed by clinical sign and tensilon test 1996-99

est (RST) is the most commonly used electrodiagnostic test to asses the defect of neuromuscular transmission, which is reported to be positive in the diffuse and restricted ocular forms 60-95% and 14-50%, respectively. In a cross-sectional study, to determine the efficacy of repetitive stimulation test in myasthenia gravis, we evaluated the results in 30 cases who were hospitalized in Imam Khomeini Hospital during 1996-1999. Patients were first selected clinically and then confirmed by Tensilon test.Various clinical types including generalized and restricted ocular forms with different severity and duration were entered in this study. Considering the fact that the positiveness of the test is enhanced by assessment of more muscle groups, we evaluated decremental response in the facial, proximal and distal muscles of limbs. 90% of patients had the generalized form of the disease, whereas ocular myasthenia gravis was seen only in 10% of the cases. 74% of females and 73% of males showed positive response (overall: 73.3%). No significant association was found between the positive response, and age and sex. Peaks of incidences of the disease for the males were in fourth and sixth decades and for the females in thired decade

"A COMPARATIVE TRIAL OF LAMOTRIGINE AND CARBAMAZEPINE IN PATIENTS WITH PRIMARY GENERALIZED TONIC CLONIC SEIZURES"

Lamotrigine has been used widely in the treatment of partial and secondary generalized seizures. In this study use of lamotrigine as monotherapy for the newly diagnosed primary generalized tonic–clonic seizure has been investigated and compared with carbamazepine. After dose escalation (3 weeks for carbamazepine and 6 weeks for lamotrigin), patients were followed every 4 weeks for the first 16 weeks and then every 8 weeks for the next 32 weeks. Total number of patients was 91, randomly divided in two groups, 46 patients in lamotrigine group and 45 patients in carbamazepine group. The efficacy of the two drugs against primary generalized tonic clonic seizure was almost the same. The proportion of patients with seizure episodes in the last 40 weeks of treatment in both groups was similar (24.2% versus 24.6%). Overall, fewer patients in lamotrigine group than in the carbamazepine group withdrew because of adverse events (6.5% vs. 24.5%, P = 0.0216). The commonest side effect leading to withdrawal with lamotrigine was rash and with carbamazepine was drowsiness. More lamotrigine than carbamazepine recipients completed the study (88.9% vs. 73.3%, P = 0.0961). Lamotrigine and carbamazepine showed similar efficacy against primary eneralized tonic clonic seizure in newly diagnosed epilepsy. Lamotrigine, however, was better tolerated

"Footdrop in the farmers: Clinical and electromyographical study "

Footdrop is a relatively common deficit among the neurological disorders, which has different causes with various levels of involvement in neuromuscular system, including central nervous system (brain cortex, spinal cord), fifth lumbar root, peripheral nerves and muscles. Peroneal nerve injury at the fibular head has been reported to the most common cause of foot drop, which can be due to infarct, tumor or leprosy but the vast majority of lesions are traumatic. In this article, we report seventeen patients with foot drop in farmers. All of the patients except one, were male with age ranges between 15 to 25 years. They had been doing certain farming activities (harvesting or weeding) for 1-5 days before developing foot drop. Electrophysiological studies have been done in only seven of them due to patient’s unwillingness. Nerve conduction velocity and amplitudes distal to the fibular head were normal, but stimulation above the fibular head showed reduced nerve conduction velocity and amplitudes (mean 22.4 m/s in the abnormal side versus 51.5 m/s in the normal side, mean peak to peak amplitude 3.6 mv in the symptomatic side versus 10.4 in the contralateral side respectively). Forty-three percent of patients had also conduction block. F wave latency increased on the affected side in comparison to the normal side (mean 4.7 m/s). The new and perhaps interesting findings in our cases are unilateral involvement and occurrence of peroneal palsy on the side of dominant hand, indicating that type of the hand activity is probably more important in inducing foot drop than the position of seating during harvesting or weeding. We suggest further investigation in this setting in order to find the mechanisms of nerve injury and prophylactic measures