A neuroimaging measure to capture heterogeneous patterns of atrophy in Parkinson's disease and dementia with Lewy bodies.

INTRODUCTION: Parkinson's disease (PD) and Dementia with Lewy bodies (DLB) show heterogeneous brain atrophy patterns which group-average analyses fail to capture. Neuroanatomical normative modelling overcomes this by comparing individuals to a large reference cohort. Patient-specific atrophy patterns are measured objectively and summarised to index overall neurodegeneration (the 'total outlier count'). We aimed to quantify patterns of neurodegenerative dissimilarity in participants with PD and DLB and evaluate the potential clinical relevance of total outlier count by testing its association with key clinical measures in PD and DLB. MATERIALS AND METHODS: We included 108 participants with PD and 61 with DLB. PD participants were subclassified into high and low visual performers as this has previously been shown to stratify those at increased dementia risk. We generated z-scores from T1w-MRI scans for each participant relative to normative regional cortical thickness and subcortical volumes, modelled in a reference cohort (n = 58,836). Outliers (z < -1.96) were aggregated across 169 brain regions per participant. To measure dissimilarity, individuals' Hamming distance scores were calculated. We also examined total outlier counts between high versus low visual performance in PD; and PD versus DLB; and tested associations between these and cognition. RESULTS: There was significantly greater inter-individual dissimilarity in brain-outlier patterns in PD poor compared to high visual performers (W = 522.5; p < 0.01) and in DLB compared to PD (W = 5649; p < 0.01). PD poor visual performers had significantly greater total outlier counts compared to high (ß = -4.73 (SE = 1.30); t = -3.64; p < 0.01) whereas a conventional group-level GLM failed to identify differences. Higher total outlier counts were associated with poorer MoCA (ß = -0.55 (SE = 0.27), t = -2.04, p = 0.05) and composite cognitive scores (ß = -2.01 (SE = 0.79); t = -2.54; p = 0.02) in DLB, and visuoperception (ß = -0.67 (SE = 0.19); t = -3.59; p < 0.01), in PD. CONCLUSIONS: Neuroanatomical normative modelling shows promise as a clinically informative technique in PD and DLB, where patterns of atrophy are variable.

A call for innovation in fetal monitoring during fetal surgery.

PURPOSE: A growing number of fetal procedures are performed at specialized fetal care centers for congenital problems that classically would have poor outcomes despite advanced postnatal management. Consistent fetal monitoring is integral to the safety of these challenging and innovative surgeries. However, standardization of fetal monitoring during various forms of fetal surgery has yet to be established. MATERIALS AND METHODS: We searched all articles on literature platforms until August 2019 using the terms "fetal surgery," "fetal monitoring," and "fetal interventions." Titles and abstracts were screened by our coauthors to determine the type of fetal monitoring used in these cases. RESULTS: The search identified 1,625 citations, of which the 50 citations considered most pertinent were included in this review. CONCLUSIONS: Fetal monitoring during in utero fetal surgeries continues to be challenging because of limited physical fetal access and technological aspects. Innovations in fetal cardiac monitoring during fetal surgeries have the potential for continuous and high-fidelity hemodynamic and physiologic monitoring, with the goal of early detection and treatment of fetal compromise.

Toward Eliminating Perinatal Comfort Care for Prenatally Diagnosed Severe Congenital Heart Defects: A Vision.

Over the past 40 years, the medical and surgical management of congenital heart disease has advanced considerably. However, substantial room for improvement remains for certain lesions that have high rates of morbidity and mortality. Although most congenital cardiac conditions are well tolerated during fetal development, certain abnormalities progress in severity over the course of gestation and impair the development of other organs, such as the lungs or airways. It follows that intervention during gestation could potentially slow or reverse elements of disease progression and improve prognosis for certain congenital heart defects. In this review, we detail specific congenital cardiac lesions that may benefit from fetal intervention, some of which already have documented improved outcomes with fetal interventions, and the state-of-the-science in each of these areas. This review includes the most relevant studies from a PubMed database search from 1970 to the present using key words such as fetal cardiac, fetal intervention, fetal surgery, and EXIT procedure. Fetal intervention in congenital cardiac surgery is an exciting frontier that promises further improvement in congenital heart disease outcomes. When fetuses who can benefit from fetal intervention are identified and appropriately referred to centers of excellence in this area, patient care will improve.

Fetal Surgery in the Era of SARS-CoV-2 Pandemic: A Single-Institution Review.

OBJECTIVE: To cope with the changing health care services in the era of SARS-CoV-2 pandemic. We share the institutional framework for the management of anomalous fetuses requiring fetal intervention at Mayo Clinic, Rochester, Minnesota. To assess the success of our program during this time, we compare intraoperative outcomes of fetal interventions performed during the pandemic with the previous year. PATIENTS: We implemented our testing protocol on patients undergoing fetal intervention at our institution between March 1, and May 15, 2020, and we compared it with same period a year before. A total of 17 pregnant patients with anomalous fetuses who met criteria for fetal intervention were included: 8 from 2019 and 9 from 2020. METHODS: Our testing protocol was designed based on our institutional perinatal guidelines, surgical requirements from the infection prevention and control (IPAC) committee, and input from our fetal surgery team, with focus on urgency of procedure and maternal SARS-CoV-2 screening status. We compared the indications, types of procedures, maternal age, gestational age at procedure, type of anesthesia used, and duration of procedure for cases performed at our institution between March 1, 2020, and May 15, 2020, and for the same period in 2019. RESULTS: There were no statistically significant differences among the number of cases, indications, types of procedures, maternal age, gestational age, types of anesthesia, and duration of procedures (P values were all >.05) between the pre-SARS-CoV-2 pandemic in 2019 and the SARS-CoV-2 pandemic in 2020. CONCLUSIONS: Adoption of new institutional protocols during SARS-CoV-2 pandemic, with appropriate screening and case selection, allows provision of necessary fetal intervention with maximal benefit to mother, fetus, and health care provider.

Toxicity evaluation of nanocrystalline silver-impregnated coated dressing on the life cycle of worm Caenorhabditis elegans.

In recent times, however, due to the emergence of bacterial strains with resistance to conventional antibiotics, silver has again gained attention as an alternative for developing new efficient bactericides, including the use of silver nanoparticles (AgNPs). However, the improper disposal of these items after use may cause toxicological effects on organisms in the environment. To evaluate the potential environmental hazard of nanosilver-coated dressings, the nematode Caenorhabditis elegans was chosen as a test organism. The assays were conducted in 24-well plates that contain four different sizes of coated dressing to obtain different concentrations. L1 and L4 C. elegans larval stages were exposed to these nanosilver concentrations. Dressing cutouts were arranged between two layers of agar for 3 days and Escherichia coli (OP 50 strain) was added as food source for the worms. After the exposure period, growth, reproduction, fertility, silver concentration in the medium and the concentration of reactive oxygen species (ROS) in the worms were evaluated. Scanning and transmission electron microscopy analyses were performed on the coated dressings, as well as analyses of zeta potential, ionic release and antibacterial power in two bacterial strains (Pseudomonas aeruginosa and Staphylococcus aureus). It was verified the antibacterial power of the coated dressing, in both bacteria strains tested. Characterization of the coated dressing indicated heterogeneous nanoparticles, as well as distinct zeta potentials for the medium in water and saline medium (0.9% NaCl). L1 larval worms exposed to nanosilver-coated dressing showed a high ROS concentration and reductions in growth, fertility and reproduction. Worms exposed to the coated dressing during the L4 stage showed almost no response. Overall, the obtained results indicate the potential environmental hazard of nanosilver-coated dressings.

Individual assessment of bovine embryo development using a homemade chamber reveals kinetic patterns of success and failure to reach blastocyst stage.

Most early developmental data are lost in bovine embryo culture systems. We developed and validated a method for culture of bovine embryos in groups that allow individual assessment. An autoclavable low-cost multiembryo chamber (MEC) was prepared using a polyester mesh fixed to a glass coverslip. Embryonic development was not affected by MEC. Compared to conventional bovine culture system (oil-covered drops, control), cleavage (C, 71.2 ± 7.8%; MEC, 74.3 ± 6.0%), blastocyst rate (C, 29.9 ± 4.4%; MEC, 28.3 ± 5.0%) and blastocyst cell number (C, 94.1 ± 9.7; MEC, 92.9 ± 5.3) were similar. Caspase 3 positive cell index in blastocysts was increased in MEC group, but apoptosis rate was below 5% (C, 2.9 ± 0.5; MEC, 4.6 ± 0.6). Using MEC, we performed a retrospective analysis for 'failure' and 'success' embryos, based on their ability to reach the blastocyst stage. We detected the majority of 'success' embryos displayed 8 cells at 48 h post-insemination (hpi) (48.7%), but blastocysts derived from this pattern presented lower cell numbers (91.3 ± 4.2 vs. 107.9 ± 4.9) and higher apoptosis index (6.2 ± 0.6 vs. 4.4 ± 0.5) than blastocysts from 4-cell embryos at 48 hpi. Most (72.0%) embryos that were at morula stage 120 hpi reached blastocyst stage at 168 hpi. Those blastocysts presented more number of cells than blastocysts derived from embryos exhibiting 16 cells at 120 hpi (108.6 ± 4.1 vs. 83.9 ± 4.8). Combination of embryo kinetics data at 48 and 120 hpi revealed high chances of blastocyst formation for patterns: 8 cells/morula, 4 cells/morula, 8 cells/16 cells and 4 cells/16 cells. Blastocysts formed from 4-cell/morula and 8-cell/morula patterns represented 69% of all 168 hpi blastocysts. Blastocysts derived from 4 cells/16 cells displayed decreased apoptosis (3.1 ± 0.6). Our results suggest that MEC can be used for bovine embryo culture without detrimental effects on development and can help to predict blastocyst formation and quality of in vitro fertilization (IVF) embryos. Abbreviations: BSA: bovine serum albumine; COC: cumulus-oocyte complex; FERT-TALP: Tyrode's albumin lactate pyruvate fertilization; FBS: fetal bovine serum; IVF: in vitro fertilization; MEC: multiembryo chamber; PBS: phosphate buffered saline; SOF-AA: synthetic oviductal fluid with amino acids medium; TCM: Tissue Culture Medium.

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.

BDNF and Hippocampal Synaptic Plasticity.

Brain-derived neurotrophic factor (BDNF) belongs to a family of small secreted proteins that also include nerve growth factor, neurotrophin 3, and neurotrophin 4. BDNF stands out among all neurotrophins by its high expression levels in the brain and its potent effects at synapses. Several aspects of BDNF biology such as transcription, processing, and secretion are regulated by synaptic activity. Such observations prompted the suggestion that BDNF may regulate activity-dependent forms of synaptic plasticity such as long-term potentiation (LTP), a sustained enhancement of excitatory synaptic efficacy thought to underlie learning and memory. Here, we will review the evidence pointing to a fundamental role of this neurotrophin in LTP, especially within the hippocampus. Prominent questions in the field, including the release and action sites of BDNF during LTP, as well as the signaling and molecular mechanisms involved, will also be addressed. The diverse effects of BDNF at excitatory synapses are determined by the activation of TrkB receptors and downstream signaling pathways, and the functions, typically opposing in nature, of its immature form (proBDNF). The activation of p75NTR receptors by proBDNF and the implications for long-term depression will also be addressed. Finally, we discuss the synergy between TrkB and glucocorticoid receptor signaling to determine cellular responses to stress.

Language improvement one week after thrombolysis in acute stroke.

OBJECTIVES: Language recovery following acute stroke is difficult to predict due to several evaluation factors and time constraints. We aimed to investigate the predictors of aphasia recovery and to identify the National Institute of Health and Stroke Scale (NIHSS) items that best reflect linguistic performance, 1 week after thrombolysis. MATERIALS AND METHODS: We retrieved data from a prospective registry of patients with aphasia secondary to left middle cerebral artery (MCA) stroke treated with intravenous thrombolysis. Complete recovery at day 7 (D7) was measured in a composite verbal score (CVS) (Σ Language+Questions+Commands NIHSS scores). Lesion size was categorized by the Alberta Stroke Program Early CT score (ASPECTS) and vascular patency by ultrasound. CVS was correlated with standardized aphasia testing if both were performed within a two-day interval. RESULTS: Of 228 patients included (age average 67.32 years, 131 men), 72% presented some language improvement that was complete in 31%. Total recovery was predicted by ASPECTS (OR=1.65; 95% CI, 1.295-2.108; P < 0.00) and baseline aphasia severity (OR=0.439; 95% CI, 0.242-0.796; P < 0.007). CVS correlated better with standardized aphasia measures (aphasia quotient, severity, comprehension) than NIHSS_Language item. CONCLUSIONS: Lesion size and initial aphasia severity are the main predictors of aphasia recovery one week after thrombolysis. A NIHSS composite verbal score seems to capture the global linguistic performance better than the language item alone.

Breeding of White-tailed Tropicbirds (Phaethon lepturus) in the western South Atlantic / Biologia reprodutiva, tamanho populacional e conservação do rabo-de-palha-de-bico-laranja no Atlântico Sul

Abstract Basic information on natural history is crucial for assessing the viability of populations, but is often lacking for many species of conservation concern. One such species is the White-tailed Tropicbird, Phaethon lepturus (Mathews, 1915). Here, we address this shortfall by providing detailed information on reproductive biology, distribution and threats on the Fernando de Noronha archipelago, Brazil – the largest colony of P. lepturus in the South Atlantic. We assessed reproduction from August 2011 to January 2012 by monitoring tropicbird nests and their contents. A population estimate was obtained through a combination of active searches for nests and by census at sea between 2010 and 2012. Breeding success was calculated by traditional methods. The growth curve of chicks and life table were also calculated. Additional information on nest and mate fidelity and on age of breeding birds was obtained from the banded birds. Our results indicate that the unusual nest form (limestone pinnacles) and predation by crabs may be responsible for the observed patterns of hatching and fledging success. Although the Fernando de Noronha population appears to be stable (at between 100-300 birds), a long term monitoring program would be desirable to assess fluctuations in this globally important population. Conservation strategies should focus on controlling predation by land crabs and tegu lizards.

Resumo Informações básicas sobre história natural são cruciais para acessar a viabilidade de populações, mas são ausentes para muitas espécies que necessitam de conservação. Uma destas espécies é o rabo-de-palha-de-bico-laranja, Phaethon lepturus Daudin, 1802. Aqui, vamos abordar o déficit de dados para esta espécie, fornecendo informações detalhadas sobre a biologia reprodutiva, tamanho da população, distribuição e ameaças em Fernando de Noronha, Brasil – a maior colônia de P. lepturus no Atlântico Sul. Acompanhamos a reprodução do rabo-de-palha-de-bico-laranja de Agosto de 2010 a Janeiro de 2011 monitorando ninhos e seus conteúdos. A estimativa da população foi obtida através de uma combinação de busca ativa de ninhos e censo no mar entre 2010 e 2012. O sucesso reprodutivo foi avaliado por métodos tradicionais. A curva de crescimento da coorte e a tabela de vida também foram obtidas. Além disso, informações sobre fidelidade ao ninho e parceiro e, a idade de reprodutores foi obtida a partir das aves anilhadas anteriormente. Nossos resultados indicam que a forma incomum de ninho (pináculos de calcário) e a predação por caranguejos podem ser responsáveis pelo sucesso observado de eclosão e recrutamento. A população de Fernando de Noronha parece estar estável entre 100-300 aves. No entanto, um programa de monitoramento a longo prazo seria desejável para avaliar as flutuações desta população globalmente importante. As estratégias de conservação devem se concentrar em controlar a predação por caranguejos e lagartos teiú.

Therapeutic efficacy of Chymotrypsin in acute bovine mastitis / Eficacia terapéutica de la quimotripsina en mastitis aguda bovina

ABSTRACT Objective . To evaluate the therapeutic efficacy of a proteolytic drug "chymotrypsin" combined with beta-lactam antibiotics in cows with acute mastitis. Material and Methods. Fourteen cows with acute mastitis. Three cows were treated with a beta-latam antibiotic (BLA) and the other eleven cows were treated with chymotrypsin plus beta-lactam antibiotic (C+BLA). The response was evaluated according to the semiological findings, somatic cell count (SCC) and a microbiological culture. Results. There was a therapeutic efficacy comparing the pre and post treatment period (SCC reduction, p<0.01) and a reduction of clinical signs in 84.7% of treated quarters in the first day of treatment (C+BLA) compared with (BLA). Conclusions. Chymotrypsin improves the treatment of acute mastitis when is combined with BLA, controlling the infected mammary glands, compared with the group treated only with amoxicilina and clavulanic acid.

RESUMEN Objetivo. Evaluar la eficacia terapéutica del fármaco proteolítico "quimotripsina", en tratamientos conjuntos con un antibiótico betalactámico en vacas con mastitis aguda. Material y métodos. Se evaluaron el conteo de células somáticas (CCS) y los hallazgos semiológicos, comparando la eficacia con un grupo de animales en donde sólo fue utilizado el antibiótico. Resultados. Los resultados revelaron eficacia clínica (disminución del CCS, p<0.01) y ausencia de signos clínicos agudos en el 84.7% de los cuartos observados, incluso observándose respuesta anti-inflamatoria desde las primeras horas del tratamiento. El restante 15.3% también presentó eficacia clínica a la terapia aunque la respuesta fue moderada en comparación con el 84.7% de los casos que tuvieron la respuesta acelerada. Conclusiones. Lo anterior permite concluir que el uso de quimotripsina en casos de mastitis aguda, acelera la respuesta en la glándula mamaria infectada e inflamada, con mayor eficacia que los tratamientos con sólo amoxicilina + ácido clavulánico.

Breeding of White-tailed Tropicbirds (Phaethon lepturus) in the western South Atlantic.

Basic information on natural history is crucial for assessing the viability of populations, but is often lacking for many species of conservation concern. One such species is the White-tailed Tropicbird, Phaethon lepturus (Mathews, 1915). Here, we address this shortfall by providing detailed information on reproductive biology, distribution and threats on the Fernando de Noronha archipelago, Brazil - the largest colony of P. lepturus in the South Atlantic. We assessed reproduction from August 2011 to January 2012 by monitoring tropicbird nests and their contents. A population estimate was obtained through a combination of active searches for nests and by census at sea between 2010 and 2012. Breeding success was calculated by traditional methods. The growth curve of chicks and life table were also calculated. Additional information on nest and mate fidelity and on age of breeding birds was obtained from the banded birds. Our results indicate that the unusual nest form (limestone pinnacles) and predation by crabs may be responsible for the observed patterns of hatching and fledging success. Although the Fernando de Noronha population appears to be stable (at between 100-300 birds), a long term monitoring program would be desirable to assess fluctuations in this globally important population. Conservation strategies should focus on controlling predation by land crabs and tegu lizards.

En bloc resection of a C-1 lateral mass osteosarcoma: technical note.

Osteosarcoma is an aggressive primary bone tumor. It is currently treated with multimodality therapy including en bloc resection, which has been demonstrated to confer a survival benefit over intralesional resection. The authors present the case of an 8-year-old girl with a C-1 lateral mass osteosarcoma, which was treated with a 4-stage en bloc resection and spinal reconstruction. While technically complex, the feasibility of en bloc resection for spinal osteosarcoma should be explored in the pediatric population.

Short Communication Impact of early enzyme-replacement therapy for mucopolysaccharidosis VI: results of a long-term follow-up of Brazilian siblings.

Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive multisystem lysosomal storage disorder, which is characterized by the deficiency of the enzyme arylsulfatase B encoded by the ARSB gene. Treatment of this disease with enzyme-replacement therapy (ERT) improves the clinical status of and generates hope for MPS VI patients. However, only few reports on patients with MPS VI treated before 5 years of age have been published. Thus, the objective of this study was to compare the clinical parameters of two sisters affected by MPS VI who started ERT at different ages (9 years and 1 year 5 months, respectively) and to determine the most relevant clinical impacts of early treatment after 85 months of evaluation. The treatment was well tolerated by both siblings. ERT in the younger sibling resulted in increased growth, an improved 6-minute walk test, less coarse face, slower progression of cardiac valve disease, and the absence of compressive myelopathy compared to that in her older sister. On the other hand, the older sibling had typical MPS VI phenotypic features before the commencement of ERT. Corneal clouding, clawed hands, and progressive skeletal changes were observed in both siblings despite the treatment. Both siblings displayed reduced frequencies of upper respiratory infections and apnea indices. This study emphasizes that early diagnosis and treatment of MPS VI are critical for a better disease outcome and to enhance the quality of life for these patients.

Subclinical left ventricular dysfunction in childhood-onset systemic lupus erythematosus: a two-dimensional speckle-tracking echocardiographic study.

OBJECTIVES: The main purpose of the study was to investigate left ventricular (LV) subclinical systolic and diastolic dysfunction in childhood-onset systemic lupus erythematosus (c-SLE) patients using two-dimensional speckle-tracking (2DST) echocardiography. We also interrogated possible correlations between impairment of myocardial deformation and the SLE Disease Activity Index 2000 (SLEDAI-2K), as well as the presence of traditional and disease-related cardiovascular risk factors (CRFs). METHOD: A total of 50 asymptomatic patients and 50 controls (age 14.74 vs. 14.82 years, p = 0.83) were evaluated by standard and 2DST echocardiography. RESULTS: Despite a normal ejection fraction (EF), there was reduction in all parameters of LV longitudinal and radial deformation in patients compared to controls: peak longitudinal systolic strain (PLSS) [-20.3 (-11 to -26) vs. -22 (-17.8 to -30.4)%, p < 0.0001], PLSS rate [-1.19 ± 0.21 vs. -1.3 ± 0.25 s(-1), p = 0.0005], longitudinal strain rate in early diastole [1.7 (0.99-2.95) vs. 2 (1.08-3.00) s(-1), p = 0.0034], peak radial systolic strain [33.09 ± 8.6 vs. 44.36 ± 8.72%, p < 0.0001], peak radial systolic strain rate [1.98 ± 0.53 vs. 2.49 ± 0.68 s(-1), p < 0.0001], and radial strain rate in early diastole [-2.31 ± 0.88 vs. -2.75 ± 0.97 s(-1), p = 0.02]. Peak circumferential systolic strain [-23.67 ± 3.46 vs. -24.6 ± 2.86%, p = 0.43] and circumferential strain in early diastole [0.37 ± 0.17 vs. 0.41 ± 0.15, p = 0.27] were similar between patients and controls, although peak circumferential systolic strain rate [-1.5 ± 0.3 vs. -1.6 ± 0.3 s(-1), p = 0.036] was reduced in c-SLE. Further analysis of patients revealed a negative correlation between LV PLSS and SLEDAI-2K (r = -0.52, p < 0.0001), and also between LV PLSS and the number of CRFs per patient (r = -0.32, p = 0.024). CONCLUSIONS: 2DST echocardiography has identified subclinical LV deformation impairment in c-SLE patients. Disease activity and cumulative exposure to CRFs contribute to myocardial compromise.

Cerebral Hemodynamics Patterns by Transcranial Doppler in Patients With Acute Liver Failure.

INTRODUCTION: About half of patients with acute liver failure (ALF) show clinical signs of cerebral edema and intracranial hypertension. Neuroimaging diagnostics and electroencephalography have poor correlation with intracranial pressure measurement. OBJECTIVE: The objective of this study was to characterize the cerebral hemodynamics patterns with transcranial Doppler (TCD) sonography in patients with ALF. METHOD: We studied 21 patients diagnosed with ALF, admitted to the intensive care unit (ICU) at the Centro de Investigaciones Médico Quirúrgicas of Cuba. All of these patients had a TCD performed on arrival at ICU, evaluating the following: systolic (SV), diastolic (DV), and medium (MV) flows velocities and pulsatility index (PI) in right middle cerebral artery (RMCA) via temporal windows. RESULTS: The sonographic patterns of cerebral hemodynamics were as follows: low-flow, 12 patients (57.1%); high resistance, 5 patients (23.8%); and hyperemic, 4 patients (19%). Patients who died while waiting had lower MV RMCA (56.1 vs 58.1 cm/s) and higher PI (1.71 vs 1.41) than patients who could undergo transplantation (P = .800 and P = .787, respectively). CONCLUSIONS: In patients diagnosed with ALF admitted to the ICU the predominating cerebral hemodynamic pattern was low-flow with resistance increase. The TCD was shown to be a useful tool in the initial evaluation for prognosis and treatment.

Subclinical right ventricle systolic dysfunction in childhood-onset systemic lupus erythematosus: insights from two-dimensional speckle-tracking echocardiography.

OBJECTIVE: The objective of this article is to evaluate right ventricle strain imaging by two-dimensional speckle-tracking (2DST) in childhood-onset systemic lupus erythematosus (c-SLE). METHODS: Thirty-five c-SLE patients with no signs or symptoms of heart failure and 33 healthy volunteers were evaluated by standard echocardiogram and 2DST. Conventional parameters included tricuspid annular plane systolic excursion (TAPSE), RV tissue-Doppler-derived Tei index and systolic pulmonary artery pressure. Global peak longitudinal systolic strain (PLSS) and strain rate (PLSSR) of RV were obtained by 2DST. Demographic/clinical features, SLEDAI-2K/SLICC/ACR-DI and treatment were also assessed. RESULTS: The median current age was similar in patients and controls (14.75 vs. 14.88 years, p = 0.62). RV PLSS was significantly reduced in c-SLE (-24.5 ± 5.09 vs. -27.62 ± 3.02%, p = 0.003). Similar findings were observed after excluding patients with pulmonary hypertension (-24.62 ± 4.87% vs. -27.62 ± 3.02%, p = 0.0041). RV PLSS was positively correlated with TAPSE (r = +0.49, p = 0.0027) and negatively correlated with Tei index (r = -0.34, p = 0.04) in c-SLE. RV PLSSR was not different comparing patients and controls (-0.65 s(-1 )± 0.47 vs. -1.87 ± 0.49 s(-1), p = 0.07). Further analysis of c-SLE patients revealed higher frequencies of neuropsychiatric manifestations (39% vs. 0%, p = 0.007) and antiphospholipid antibodies (55% vs. 18%, p = 0.035) in those with RV PLSS ≤ -23.7% vs >-23.7%. No differences were evidenced in demographic data, disease activity/damage or treatments (p > 0.05). CONCLUSIONS: The present study, using a new and more sensitive technique, revealed subclinical RV systolic dysfunction in c-SLE patients that may have future prognostic implications. The novel association of asymptomatic RV dysfunction with neuropsychiatric manifestations and antiphospholipid antibodies may suggest common physiopathological pathways.