Vitamin A status, hospitalizations, and other outcomes in young children with sickle cell disease

OBJECTIVE: To determine the relation of serum vitamin A status to growth, nutritional and hematologic status, and to the number of hospitalizations in children with sickle cell disease-SS (homozygous for the S allele, SCD-SS). STUDY DESIGN: Children (2-9.9 years of age) with SCD-SS were assessed for serum retinol, hemoglobin, hematocrit, reticulocyte count, height, weight, body mass index, and recalled dietary intake. Vitamin A status was defined on the basis of serum retinol concentration as suboptimal (<30 microg/dL) and normal (> or =30 microg/dL). Hospitalizations were determined for 1 year after vitamin A assessment. RESULTS: Mean serum retinol was 26.7 +/- 6.8 microg/dL in 66 subjects (39 girls) and was suboptimal in 66% of children. Compared with those with normal status, children with suboptimal vitamin A had significantly lower body mass index z score (-0.7 +/- 1.0 vs -0.1 +/- 0.6) and hemoglobin (7.9 +/- 1.1 vs 8.5 +/- 1.1), and hematocrit (23.3 +/- 3.0 vs 25.1 +/- 3.8) and significantly more hospitalizations (2.8 +/- 2.0 vs 0.7 +/- 0.8). After adjusting for age and sex, suboptimal vitamin A status was associated with a 10-fold increased risk for hospitalization (OR, 10.5; 95% CI, 2.3, 48.6) and with increased pain (OR,5.3; 95% CI, 1.3, 21.6) and fever episodes (OR, 6.4; 95% CI, 1.7, 24.9) requiring hospitalization. CONCLUSIONS: Suboptimal vitamin A status was prevalent in US children with SCD-SS and was associated with increased hospitalizations and poor growth and hematologic status.     

Wild-type transthyretin-derived amyloidosis in various ligaments and tendons

Transthyretin-derived amyloid deposition is commonly found in intercarpal ligaments of patients with senile systemic amyloidosis. However, the frequency of transthyretin-derived amyloid deposits in ligaments of other tissues remains to be elucidated. This study aimed to determine the frequency of amyloid deposition and the precursor proteins of amyloid found in orthopedic disorders. We studied 111 specimens from patients with carpal tunnel syndrome (flexor tenosynovium specimens), rotator cuff tears (rotator cuff tendon specimens), and lumbar canal stenosis (yellow ligament specimens). To identify amyloid precursor proteins, we used immunohistochemical staining with antibodies that react with transthyretin, immunoglobulin light chain, amyloid A protein, and β(2)-microglobulin. By means of Congo red staining, we identified 47 (42.3%) amyloid-positive samples, 39 of which contained transthyretin-derived amyloid (18 flexor tenosynovium specimens, 5 rotator cuff tendon specimens, and 16 yellow ligament specimens). Genetic testing and/or clinical findings suggested that all patients with transthyretin amyloid deposits did not have familial amyloidotic polyneuropathy. The occurrence of amyloid deposition in those tissues depended on age. These results suggest that transthyretin-derived amyloid deposits may occur more frequently in various ligaments and tendons than originally expected. In the future, such amyloid deposits may aid determination of the pathogenesis of ligament and tendon disorders in older patients.     

The menace of endocrine disruptors on thyroid hormone physiology and their impact on intrauterine development

Αbstract The delivery of the appropriate thyroid hormones quantity to target tissues in euthyroidism is the result of unopposed synthesis, transport, metabolism, and excretion of these hormones. Thyroid hormones homeostasis depends on the maintenance of the circulating ‘free’ thyroid hormone reserves and on the development of a dynamic balance between the ‘free’ hormones reserves and those of the ‘bound’ hormones with the transport proteins. Disturbance of this hormone system, which is in constant interaction with other hormone systems, leads to an adaptational counter-response targeting to re-establish a new homeostatic equilibrium. An excessive disturbance is likely to result, however, in hypo- or hyper- thyroid clinical states. Endocrine disruptors are chemical substances forming part of ‘natural’ contaminating agents found in most ecosystems. There is abundant evidence that several key components of the thyroid hormones homeostasis are susceptible to the action of endocrine disruptors. These chemicals include some chlorinated organic compounds, polycyclic aromatic hydrocarbons, herbicides, and pharmaceutical agents. Intrauterine exposure to endocrine disruptors that either mimic or antagonize thyroid hormones can produce permanent developmental disorders in the structure and functioning of the brain, leading to behavioral changes. Steroid receptors are important determinants of the consequences of endocrine disruptors. Their interaction with thyroid hormones complicates the effect of endocrine disruptors. The aim of this review is to present the effect of endocrine disruptors on thyroid hormones physiology and their potential impact on intrauterine development.     

Clinical profile of patients with cardiac amyloidosis in India

Limited data exists on patients with cardiac amyloidosis (CA) in India, due to underdiagnosis and late presentation. We present single centre data from 13 patients over a 4 year period with a median age of 65 years. A majority presented with symptomatic heart failure (69%) and eight patients had confirmed AL amyloidosis. At the end of the follow up period, 46% patients died, with 30% of the overall cohort dead within six months. Among the survivors, 71% continue to have NYHA grade III/IV symptoms. A suggested algorithm for earlier diagnosis in resource constrained settings is also presented.     

Transthyretin: More than meets the eye

Transthyretin (TTR) is a plasma protein mostly known for being the transporter of thyroxine and retinol. When mutated, TTR is also well-described as the cause of familial amyloid polyneuropathy, a neurodegenerative lethal disorder characterized by systemic deposition of TTR amyloid fibrils, particularly in the peripheral nervous system. Recent studies have determined that besides its carrier properties, TTR is an important protein in peripheral and central nervous system physiology, namely by participating in behavior, in the maintenance of normal cognitive processes during ageing, amidated neuropeptide processing and nerve regeneration. Additionally, it has been proposed that TTR is neuroprotective in Alzheimer's disease, by preventing the formation of amyloid beta fibrils. With the advent of powerful screening techniques, TTR has also been linked to a number of other pathological conditions, including Parkinson's disease, schizophrenia, depression, among others. These associations, together with the recently unraveled nervous system-related functions, suggest that the relevance of TTR in physiology, particularly in neurobiology, is undervalued and that additional research in this field is needed. The aim of this review is to integrate in a critical perspective the current scattered knowledge concerning TTR most and less acknowledged functions and its association with several neuropathologies.     

ZAAPS International Surveillance Program (2007) for linezolid resistance: results from 5591 Gram-positive clinical isolates in 23 countries

The 2007 ZAAPS Program reports the results from the 6th year of oxazolidinone (linezolid) resistance surveillance among Gram-positive pathogens from 23 nations. For 2007, a total of 5591 organisms were systematically sampled from Asia, Australia, Canada, Europe, and Latin America including Staphylococcus aureus (3000 isolates, 38.2% methicillin resistant), coagulase-negative staphylococci (CoNS, 716 isolates), enterococci (906 isolates), Streptococcus pneumoniae (452 isolates), viridans group streptococci (155 isolates), and β-hemolytic streptococci (362 isolates). The overall linezolid MIC distribution (MIC₅₀ and MIC₉₀ at 1 and 2 μg/mL, respectively) was unchanged since 2002. At published linezolid breakpoints (≤2 μg/mL), all streptococci were susceptible; however, resistance was observed very rarely among S. aureus (0.03%), CoNS (0.28%), and the enterococci (0.11%, 0.55% intermediate). These oxazolidinone-nonsusceptible isolates occurred in Ireland, Italy, China, and Brazil (9 strains), and the rate was not increased since 2006. The detected mechanism of resistance was G2576 target mutations; no cfr-mediated patterns were observed. Clonal outbreaks with patient-to-patient dissemination were documented in 1 Italian site. Linezolid appears to retain excellent activity against monitored Gram-positive pathogens at a level of >99.8%.     

LINQ和VSTO在医院设备采购系统中的应用

介绍了LINQ和VSTO技术在医院设备采购系统设计中的应用。LINQ提供一种一致的编程模型来处理任何类型的对象或数据源。VSTO为Office文档提供了丰富的Windows窗体控件和创建自定义任务窗格的能力。LINQ和VSTO使开发丰富的应用程序变得更有效率。     

Renal Infarction and Its Consequences for Renal Function in Patients With Cardiac Amyloidosis

ObjectiveTo describe the prevalence of and risk factors for renal infarction (RI) in patients with cardiac amyloidosis.Patients and MethodsWe evaluated 87 patients with cardiac amyloidosis who underwent renal technetium-99m-labeled dimercaptosuccinic acid scintigraphy in the Amyloidosis Referral Center of Henri-Mondor Hospital from October 1, 2015, through February 28, 2018.ResultsThree groups of patients were identified according to the underlying amyloidosis disorder: AL amyloidosis in 24 patients, mutated-transthyretin amyloidosis in 24 patients, and wild-type transthyretin amyloidosis in 39 patients. Patients with wild-type transthyretin amyloidosis were older (P<.001), more likely to be men (P=.02), to have arrhythmic heart diseases (P<.001), and to be receiving anticoagulation treatment (P<.001). Patients with AL amyloidosis had significantly higher N-terminal pro-B-type natriuretic peptide levels (P=.02) and were more likely to have nephrotic syndrome (P<.001). Renal infarction was detected in 18 patients (20.7%), at similar frequencies in the various groups. Baseline urinary protein to creatinine ratio was the only parameter for which a significant difference (P=.03) was found between patients with and without RI diagnoses. The likelihood of RI diagnosis was 47.1% (8 of 17) in the presence of AKI and 14.5% (10 of 69) in its absence (P=.003). Overall, heart transplant–censored patient survival did not differ significantly between patients with and without RI (P=.64), but death- and heart transplant–censored renal survival was significantly lower in patients with RI (P<.001).ConclusionOur study suggests that prevalence of RI in patients with cardiac amyloidosis is higher than previously thought, regardless of the underlying amyloidosis disorder. Acute kidney injury in a patient with cardiac amyloidosis should alert clinicians to the possibility of RI.