Results: Fifty-two patients (3 6%) had SRs (6 SPT and 46 intr

\n\nResults: Fifty-two patients (3.6%) had SRs (6 SPT and 46 intradermal): 43 (83%)

were female, and 17 (33%) had asthma. Systemic symptoms included (SPT/intradermal) pruritic eyes, nose, or pharynx (0%/46%); worsening cough (50%/26%); sensation of difficulty swallowing (0%/20%); worsening nasal congestion (17%/15%); rhinorrhea (17%/13%); chest tightness or shortness of breath (33%/11%); generalized pruritus (17%/11%); sneezing (33%/9%); wheeze (0%/4%); and urticaria (17%/2%). No severe asthma, shock, hypotension, unconsciousness, or biphasic reactions occurred. All 52 patients received epinephrine intramuscularly, 48 (92%) oral prednisone, 9 (17%) oral prednisone to take 6 to 8 hours after a reaction, 50 (96%) oral antihistamine, and 6 (12%) nebulized beta-agonist.\n\nConclusions: Of QNZ cell line patients who underwent ST, SRs occur-red in 3.6% (0.4% for SPT and 3.2% for intradermal ST), all of whom readily responded to epinephrine Linsitinib supplier intramuscularly in the deltoid. This immediate administration of epinephrine seems to prevent more serious and

biphasic reactions. Ann Allergy Asthma Immunol. 2009;102:400-402.”
“Aim: The aim of this study was to investigate the influence of platelet-rich fibrin (PRF) on early wound healing and preservation of the alveolar ridge shape following tooth extraction.\n\nMethods: In this clinical trial, 20 symmetrical, premolar extraction sockets using split-mouth design were randomly selected with PRF or blood clot. The evaluations of wound healing, alveolar ridge contour changes, and crestal bone resorption

were performed in dental casts and periapical radiographs (T0, initial; T1, 1 week; T2, 2 weeks; T4, 4 weeks; T6, 6 weeks; T8, 8 weeks).\n\nResults: Platelet-rich fibrin clinically showed early healing of soft tissue covering socket orifices in the first 4 weeks. At the first week, the horizontal resorption on buccal aspect of PRF (1.07 +/- 0.31 mm) was significantly less than that of the control (1.81 +/- 0.88 mm). Platelet-rich fibrin demonstrated the tendency to enter the steady stage after the fourth week following tooth extraction, whereas in the control group the progression of buccal contour contraction was still detected through the eighth week. Radiographically, the overall click here resorption of marginal bone levels at mesial and distal to the extraction site in PRF (0.70, 1.23 mm) was comparable to that of the control (1.33, 1.14 mm). Although the PRF group demonstrated faster bone healing compared with the control, no statistically significant difference was detected.\n\nConclusions: This preliminary result demonstrated neither better alveolar ridge preservation nor enhanced bone formation of PRF in the extraction socket. The use of PRF revealed limited effectiveness by accelerated soft-tissue healing on the first 4 weeks.”
“For painful procedures in children, national recommendations are now available in France.

VpRFP1 was initially identified as an expressed sequence tag (EST

VpRFP1 was initially identified as an expressed sequence tag (EST) from

a cDNA library constructed from leaves of V. pseudoreticulata inoculated with the grapevine powdery mildew Uncinula necator. Sequence analysis of the deduced VpRFP1 protein STI571 cell line based on the full-length cDNA revealed an N-terminal nuclear localization signal (NLS) and a C-terminal C4C4-type RING finger motif with the consensus sequence Cys-X(2)-Cys-X(13)-Cys-X(1)-Cys-X(4)-Cys-X(2)-Cys-X(10)-Cys-X(2)-Cys. Upon inoculation with U. necator, expression of VpRFP1 was rapidly induced to higher levels in mildew-resistant V. pseudoreticulata plants. In contrast, expression of VpRFP1 was downregulated in mildew-susceptible MEK162 V. vinifera plants. Western blotting using an antibody raised against

VpRFP1 showed that VpRFP1 was also induced to higher levels in V. pseudoreticulata plants at 12-48 hours post-inoculation (hpi). However, there was only slight increase in VpRFP in V. vinifera plants in the same time frame, even though a more significant increase was observed at 96-144 hpi in these plants. Results from transactivation assays in yeast showed that the RING finger motif of VpRFP1 exhibited some activity of transcriptional activation; however, no activity was seen with the full-length VpRFP1. Overexpression of VpRFP1 in Arabidopsis plants was found to enhance resistance to Arabidopsis powdery mildew Golovinomyces cichoracearum, which seemed to be correlated with increased transcript levels of AtPR1 and AtPR2 in the pathogen-infected tissues. In addition, the Arabidopsis transgenic lines showed enhanced resistance to a virulent bacterial pathogen Pseudomonas syringae pv. tomato DC3000. Taken together, the results suggested that VpRFP1 may be a transcriptional activator of defence-related genes in grapevines.”
“BioAggregate

is a new product that was formulated for root-end filling, perforation repair, and pulp capping. This study examined the chemical differences between white mineral trioxide aggregate (MTA) and BioAggregate in both powder and set forms using X-ray diffraction. The results showed that white MTA and BioAggregate have a similar chemical composition Crenolanib clinical trial with some differences: BioAggregate contains a significant amount of tantalum oxide instead of bismuth oxide. In both groups, similar peaks were observed in the set and powder form, but sharper and stronger peaks were observed in the powder samples. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010; 109: 155-158)”
“Effects of alkalization, acetylation, and maleic anhydride grafted polypropylene (MAPP) treatments on the thermal and chemical properties of the wheat straw fiber were investigated using thermogravimetric analysis, infrared spectrophotometer, X-ray diffraction, and scanning electric microscopy techniques.

In addition to singly methylated pairs of glutamate and/or glutam

In addition to singly methylated pairs of glutamate and/or glutamine residues, we identified singly methylated aspartate-glutamate and alanine-glutamate pairs and doubly methylated glutamate

pairs. The largest methylatable regions detected in Htrs comprise six heptads along the coiled coil. One methylated glutamate residue was detected outside of such a region, in the signaling region of Htr14. Our analysis produced evidence supporting the predicted methyltransferase and methylesterase activities of halobacterial CheR and CheB, respectively. It furthermore FRAX597 supplier demonstrated that CheB is required for Htr deamidations, at least at a specific glutamine-glutamate pair in Htr2 and a specific aspartate-glutamine pair in Htr4. Compared to previously reported methods, the described approach significantly facilitates the identification of physiological transducer

modification sites. (C) 2008 Elsevier Ltd. All rights reserved.”
“Objective To identify associations between men’s self-reports of ill-fitting condoms and selected condom use problems, using an event-specific analysis.\n\nMethods A convenience sample of men was recruited via advertisements in newspapers (two urban and one small town) and a blog on the website of a condom sales company. Men completed a questionnaire posted on the website Bucladesine mouse of The Kinsey Institute for Research in Sex, Gender, and Reproduction. Inclusion criteria were: at least 18 years old, used condoms for penile-vaginal intercourse in the past 3 months and the ability to read English.\n\nResults In controlled, event-specific, analyses of 436 men, those reporting ill-fitting condoms (44.7%) were significantly more likely to report breakage (adjusted odds ratio (AOR) 2.6), slippage (AOR 2.7), difficulty reaching orgasm, both for their female partners (AOR 1.9) and for themselves (AOR 2.3). In addition, they were more likely to report irritation of the penis (AOR 5.0) and reduced sexual pleasure, both for their female partner (AOR 1.6) and for themselves (AOR SIS3 inhibitor 2.4). Furthermore, they were more likely to report that condoms interfered with erection (AOR

2.0), caused erection loss (AOR 2.3), or became dry during sex (AOR 1.9). Finally, they were more likely to report removing condoms before penile-vaginal sex ended (AOR 2.0).\n\nConclusions Men and their female sex partners may benefit from public health efforts designed to promote the improved fit of condoms.”
“Background: There is no sufficiently validated therapy for chronic pelvic pain syndrome (CPPS).\n\nObjective: To investigate the effects of extracorporeal shock wave therapy (ESWT) in 60 patients suffering from CPPS.\n\nDesign, setting, and participants: Sixty patients suffering from CPPS for at least 3 mo were investigated in two groups. Both groups were treated four times (once per week), each by 3000 impulses; group 2 was performed as a sham procedure.

Diffusional

Diffusional BI 6727 nmr kurtosis imaging can provide additional and complementary information to DTI on spinal cord pathology.”
“Foot-and-mouth disease (FMD) is a highly contagious, debilitating, and globally significant viral disease typically affecting cloven-hoofed hosts. The diagnosis of FMD in bears in Vietnam is described. The current study describes a confirmed case of FMD in a bear species, and the clinical signs compatible with FMD in a Malayan sun bear.

Thirteen Asiatic black bears (Ursus thibetanus) and 1 Malayan sun bear (Helarctos malayanus) were apparently affected. In August 2011, an adult bear became lethargic, and developed footpad vesicles. Over 15 days, 14 out of 17 bears developed similar signs; the remaining 3 co-housed bears and another 57 resident bears did not. All affected bears developed vesicles on all footpads, and most were lethargic for 24-48 hr. Nasal and oral lesions were noted in 6 and 3 cases, respectively. Within 1 month, all looked normal. Foot-and-mouth disease virus (FMDV) was detected by reverse transcription polymerase chain reaction, classified as serotype O, and isolated by virus isolation techniques. Phylogenetic analysis see more demonstrated clustering of 3 bear isolates, in a branch distinct from other FMDV type O isolates. The outbreak likely occurred due to indirect contact with livestock,

and was facilitated by the high density of captive bears. It showed that Asiatic black bears are capable of contracting FMDV and developing clinical disease, and that the virus spreads easily between bears in close contact.”
“Controlled wear testing of total hip replacements in hip joint simulators is a well-established and powerful method, giving an extensive prediction of the long-term NU7441 nmr clinical performance. To understand the wear behavior of a bearing and its limits under in vivo conditions, testing scenarios should be designed as physiologically as possible. Currently, the ISO standard protocol 14242 is the most common preclinical testing procedure for total hip replacements, based on a simplified gait cycle for normal walking conditions. However, in recent years, wear patterns have increasingly

been observed on retrievals that cannot be replicated by the current standard. The purpose of this study is to review the severe testing conditions that enable the generation of clinically relevant wear rates and phenomena. These conditions include changes in loading and activity, third-body wear, surface topography, edge wear and the role of aging of the bearing materials.”
“The epidemiological transition has produced a society with a growing number of the elderly affected by multiple chronic diseases which often requires polypharmacy. Despite being the main users of medications, the elderly are largely underrepresented in clinical trials, especially if they have multimorbidity. It follows that the results of these trials are not applicable in the real world and potentially harmful.

DesignA cross-sectional study was conducted of dental rec

\n\nDesign\n\nA cross-sectional study was conducted of dental records of 1133 patients (mean age 4.6years, SD +/- 1.4) attending one public

dental service clinic in Sweden. The clinic was located in an area with a large multiethnic community. All were born within the years 2002-2006 and had received a check-up in one of the years 2007-2009. A registry was made of missing primary canines where no reason could be found. In documented cases, information about ethnic origin was extracted. Statistical grouping was made according to known East African ethnicities.\n\nResults\n\nAt least 36 ethnicities were recorded. Twenty-four (2.1%) patients were missing one or more canines according to the criteria for IDE. Significant difference was seen when comparison was made between patients of known East African ethnicities, selleck of whom 20.8% (21/101) manifested findings consistent with the criteria, and the rest of the population (3/1032; P<0.001).\n\nConclusions\n\nPrevalence of cases suggestive of IDE among patients of East African origin points to a need for increased awareness within dental and healthcare communities.”
“Antidementia drugs – indication and duration of treatment\n\nTreatment strategies in dementia Selleck JNK-IN-8 have to be embedded in a multimodal array of interventional therapies. The present article focuses on the treatment with antidementia

drugs, i. e. cholinesterase inhibitors

and memantine. Their pharmacological properties, their efficacy and adverse events are mostly known and based on valid research data. check details Problems with respect to duration of treatment, necessity of changing substances, and discontinuation of treatment may not be solved on evidence-based criteria. Some consensus work, however, has been done to give advice in these therapeutic dilemmata during the course of long-term treatment in practice and is elaborated upon in the present article. In a nutshell, antidementia treatment in Alzheimer disease today is symptomatic and, hence, limited in efficacy. Adverse events, however, are predominantly mild and manageable, pharmacological interactions mostly detectable, and treatment effects quite often obvious and recognizable by patients and relatives.”
“Diabetic neuropathy (DN) is the most common peripheral neuropathy and long-term complication of diabetes. In view of the pathological basis for the treatment of DN, it is important to prevent nerve degeneration. Most of the current treatment strategies are symptomatic therapies. In this study, we evaluated the effectiveness of magnesium-25, carrying porphyrin-fullerene nanoparticles, on diabetes-induced neuropathy. Previous studies have suggested that dorsal root ganglion (DRG) neurons comprise a specific target and may be responsible for the known complications of DN.

The successful implementation of this technology for diagnosing g

The successful implementation of this technology for diagnosing genetic disorders

requires a rapid, user-friendly method to annotate variants and generate short lists of clinically relevant variants of interest. This report describes Omicia’s Opal platform, a new software tool designed for variant discovery and interpretation in a clinical laboratory environment. The software allows clinical scientists to process, analyze, interpret and report ATM/ATR targets on personal genome files. Materials & Methods: To demonstrate the software, the authors describe the interactive use of the system for the rapid discovery of disease-causing variants using three cases. Results & Conclusion: Here, the authors show the features of the Opal system and their use in uncovering variants of clinical significance.”
“Torrefaction is a mild pyrolysis process (usually up to 300 degrees C) that changes the chemical and physical properties of biomass. This process is a possible pre-treatment prior to further processes (transport, grinding, combustion, gasification, etc) to generate energy or biofuels. In this study, three eucalyptus wood species and bark were subjected to different torrefaction conditions to determine the alterations in their structural and energy find protocol properties. The most severe treatment (280 degrees C, 5 h) causes mass losses

of more than 35%, with severe damage to anatomical structure, and an increase of about 27% in the specific energy content. Bark is more sensitive to heat than wood. Energy yields are always higher than mass yields, thereby demonstrating the benefits Daporinad of torrefaction in concentrating biomass energy. The overall mass loss is proposed as a relevant parameter to synthesize the effect of torrefaction conditions (temperature and duration). Accordingly, all results are summarised by analytical expressions able to predict the energy properties as a function of the overall mass loss. These

expressions are intended to be used in any optimization procedure, from production in the field to the final use. (c) 2010 Elsevier Ltd. All rights reserved.”
“Increased wave reflection (augmented pressure (AP) and augmentation index (Alx)) and reduced muscle strength may increase cardiovascular risk in postmenopausal women. We evaluated the effects of whole-body vibration exercise training (WBVET) on aortic haemodynamics and leg muscle strength. Twenty-eight postmenopausal women (age, 56 +/- 3 years; brachial systolic blood pressure (SBP) 138 +/- 12 mm Hg; body mass index, 33.9 +/- 3.7 kg m(-2)) were randomized to 6 weeks of WBVET (n=15) or no-exercise control groups. Aortic SBP, diastolic blood pressure (DBP), pulse pressure (PP), AP, Alx, tension time index (TTI, myocardial oxygen demand) and leg press muscle strength were measured before and after 6 weeks.

Tumor etiology is evenly distributed between de novo origin and s

Tumor etiology is evenly distributed between de novo origin and surgical trauma. Treatment outcomes, although, cannot be determined from

the limited data currently available. Published by Elsevier Inc.”
“We examined the structure of personal life values as a representation of underlying motivation, in a Spanish sample of children and adolescents 12 – 16 years old. In general, results showed selleck screening library that youth put higher priority on intrinsic life goals (meaningful relationships, being physically healthy, self-acceptance) than extrinsic life goals (image, money, power). Gender differences were found in specific life goals. When comparing our results with another longitudinal American study using the same instrument and methodology,

we found similar results, although Spanish youth value priorities goals related to support rather than striving as m American adolescents. Cultural and age trend in life priorities are discussed.”
“Parkinson Disease (PD) is a common neurodegenerative disorder of intricate etiology, caused by progressive loss of aminergic neurons and accumulation of Lewy bodies. The predominant role of genetics in the etiology of the disease has emerged since the identification of the first pathogenetic mutation in SNCA (alpha-synuclein) gene, back in 1997. Mendelian parkinsonisms, a minority among all PD forms, have been deeply investigated, with 19 loci identified. More recently, genome wide association Nutlin-3 supplier studies have provided convincing evidence that variants in some of these genes, as well as in other genes, may confer an increased risk learn more for late onset, sporadic PD. Moreover, the finding that heterozygous mutations in the GBA gene (mutated in Gaucher disease) are among the strongest genetic susceptibility factors for PD, has widened the scenario of PD genetic background to enclose a number of genes previously associated to distinct

disorders, such as genes causative of spinocerebellar ataxias, mitochondrial disorders and fragile X syndrome. At present, the genetic basis of PD defines a continuum from purely mendelian forms (such as those caused by autosomal recessive genes) to multifactorial inheritance, resulting from the variable interplay of many distinct genetic variants and environmental factors.”
“A Ca2+ signaling model is proposed to consider the crosstalk of Ca2+ ions between endoplasmic reticulum (ER) and mitochondria within microdomains around inositol 1, 4, 5-trisphosphate receptors (IP3R) and the mitochondrial Ca2+ uniporter (MCU). Our model predicts that there is a critical IP3R-MCU distance at which 50% of the ER-released Ca2+ is taken up by mitochondria and that mitochondria modulate Ca2+ signals differently when outside of this critical distance. This study highlights the importance of the IP3R-MCU distance on Ca2+ signaling dynamics.

Those results are consistent with data from the most successful c

Those results are consistent with data from the most successful care coordination demonstration projects funded by the Centers for Medicare and Medicaid

Services. Specifically, Gundersen’s program has been associated with reduced hospital stays, lower costs for inpatients, less use of inpatient services, and increased check details patient satisfaction. Gundersen’s success is rooted in its team-based approach to coordinated care. Teams, led by a subspecialty-trained nurse, have regular, face-to-face contact with patients and their physicians in both inpatient and outpatient settings; involve patients deeply in care-related decisions; access a system-wide electronic medical record database

that tracks patients’ care; and take a macrolevel view of care-related factors and costs. Gundersen’s model offers specific take-home lessons for institutions BMS-777607 order interested in coordinated care as they design programs aimed at improving quality and lowering costs. This institutional case study provides a window into well-executed care coordination at a large health care system in an era when major changes in health care provision and reimbursement mechanisms are on the horizon. (C) 2013 Mayo Foundation for Medical Education and Research square Mayo Clin Proc. 2013;88(2):184-194″
“Methods: A consecutive series of patients with AMI without a previous history of HF (n = 569) were followed up for 8 years. At baseline, the patients had a blood sample for determination of BNP, a 24-hour Holter recording for evaluating heart rate variability (HRV) and heart rate turbulence (HRT), and an assessment SBI-0206965 manufacturer of baroreflex sensitivity (BRS) using phenylephrine test.\n\nResults: During the follow-up, 79 (14%)

patients were hospitalized due to HF. Increased baseline BNP, decreased HRV, HRT, and BRS had a significant association with HF hospitalization in univariate comparisons (P < 0.001 for all). After adjusting with all the relevant clinical parameters, BNP, HRV, and HRT still significantly predicted HF hospitalization (P < 0.001 for BNP and for the short-term scaling exponent alpha(1), P < 0.01 for turbulence slope). In the receiver operator characteristics curve analysis, the area under the curve for BNP was 0.77, for the short-term scaling exponent alpha(1) 0.69, for turbulence slope 0.71, and for BNP/standard deviation of all N-N intervals ratio 0.80.\n\nConclusion: Baseline increased BNP and impaired autonomic function after AMI yield significant information on the long-term risk for HF hospitalization.\n\nAnn Noninvasive Electrocardiol 2010;15(3):250-258.”
“Background: Osteoarthritis patients may exhibit different kinematics according to the disease stage. However, changes in the frontal and horizontal planes in each stage remain unclear.

Only a small subset of yet to be determined alerts appears suitab

Only a small subset of yet to be determined alerts appears suitable for automated display in clinical routine.”
“The isolation of influenza virus 80 years ago in 1933 very quickly led to the development of the first generation of live-attenuated vaccines. The first inactivated influenza vaccine was monovalent (influenza A). In 1942, a bivalent vaccine AZD1480 research buy was produced after the discovery of influenza B. It was later discovered that influenza viruses mutated leading to antigenic changes. Since 1973, the WHO has issued annual recommendations

for the composition of the influenza vaccine based on results from surveillance systems that identify currently circulating strains. In 1978, the first trivalent vaccine included two influenza A strains and one influenza B strain. Currently, there are two influenza B lineages circulating; in the latest WHO recommendations, it is suggested that a second B strain could be added to give a quadrivalent vaccine. The history of influenza vaccine and the associated technology shows how the vaccine has evolved to match the evolution of influenza viruses.”
“Introduction: New reconstructive and less invasive methods have been 10058-F4 datasheet searched to optimize bone formation and osseointegration of dental implants in maxillary sinus augmentation.\n\nPurpose: The aim of the presented ovine split-mouth study was to compare bovine bone mineral (BBM) alone and in combination with mesenchymal

URMC-099 inhibitor stem cells (MSCs) regarding their potential

in sinus augmentation.\n\nMaterial and Methods: Bilateral sinus floor augmentations were performed in six adult sheep. BBM and MSCs were placed into the test side and only BBM in the contra-lateral control side of each sheep. Animals were sacrificed after 8 and 16 weeks. Augmentation sites were analyzed by computed tomography, histology, and histomorphometry.\n\nResults: The initial volumes of both sides were similar and did not change significantly with time. A tight connection between the particles of BBM and the new bone was observed histologically. Bone formation was significantly (p = 0.027) faster by 49% in the test sides.\n\nConclusion: The combination of BBM and MSCs accelerated new bone formation in this model of maxillary sinus augmentation. This could allow early placement of implants.”
“Trigeminal neuropathic pain is a facial pain syndrome associated with trigeminal nerve injury. However, the mechanism of trigeminal neuropathic pain is poorly understood. This study aimed to determine the role of transient receptor potential vanilloid 1 (TRPV1) in heat hyperalgesia in a trigeminal neuropathic pain model. We evaluated nociceptive responses to mechanical and heat stimuli using a partial infraorbital nerve ligation (pIONL) model. Withdrawal responses to mechanical and heat stimuli to vibrissal pads (VP) were assessed using von Frey filaments and a thermal stimulator equipped with a heat probe, respectively.

The cases of strong concentration of microelement and weak

The cases of strong concentration of microelement and weak

concentration of microelement were considered. Our purpose from this study is to investigate LDN-193189 research buy the effects of the governing parameters, namely the suction/injection parameter, thermal stratification parameter, Prandtl number, vortex viscosity parameter and Reynolds number on the velocity profiles, pressure distributions, angular velocity profiles and temperature profiles as well as the skin friction coefficient, dimensionless wall couple stress and the Nusselt number. The numerical results are validated by favorable comparisons with previously published results. The results are shown graphically. The values of the skin friction coefficient, dimensionless wall couple stress and the Nusselt number are presented in tables. Copyright (C) 2011 John Wiley & Sons, Ltd.”
“Severe toxicity was detected in mussels from Bizerte Lagoon (Northern Tunisia) using routine mouse bioassays for detecting diarrheic and paralytic toxins not associated to classical phytoplankton blooming. The atypical toxicity was characterized by rapid mouse death. The aim of the present work was to understand the basis of such toxicity. Bioassay-guided

chromatographic separation and mass spectrometry were used to detect and characterize the fraction responsible for mussels’ toxicity. Only a C17-sphinganine analog mycotoxin (C17-SAMT), with a molecular mass of 287.289 Da, was found in contaminated shellfish. The doses of C17-SAMT that were lethal to 50% of mice were 750 and 150 g/kg following intraperitoneal SB203580 in vivo and intracerebroventricular NSC23766 manufacturer injections, respectively, and 900 g/kg following oral administration. The macroscopic general aspect of cultures and the morphological characteristics of the strains isolated from mussels revealed

that the toxicity episodes were associated to the presence of marine microfungi (Fusarium sp., Aspergillus sp. and Trichoderma sp.) in contaminated samples. The major in vivo effect of C17-SAMT on the mouse neuromuscular system was a dose- and time-dependent decrease of compound muscle action potential amplitude and an increased excitability threshold. In vitro, C17-SAMT caused a dose- and time-dependent block of directly- and indirectly-elicited isometric contraction of isolated mouse hemidiaphragms.”
“Alpha-mannosidosis is a rare lysosomal storage disorder with a heterogeneous clinical presentation. We describe a set of siblings with alpha-mannosidosis. The older child presented with a severe phenotype with multisystem involvement and had progressive deterioration in her motor and cognitive functioning. She had a poor outcome. The second child has a less severe disease course. He is being managed symptomatically only because of the family’s poor socioeconomic circumstances.