Publications by authors named "WOOLF A"

The funding of United States's poison control centers is threatened. The following Commentary argues for support of the current outstanding poison control system by presenting the evidence for its cost-effectiveness.

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Renal fibromuscular dysplasia (FMD) can cause hypertension, and previous reports suggest that FMD is familial. We hypothesized that, in families containing an individual with proven FMD, relatives of index cases would have an increased risk of hypertension. ACTA2 mutations cause a spectrum of extra-renal arteriopathy, leading to our second hypothesis that mutations are implicated in FMD.

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Background And Objectives: Primary vesicoureteric reflux (VUR) can coexist with reflux nephropathy (RN) and impaired renal function. VUR appears to be an inherited condition and is reported in approximately one third of siblings of index cases. The objective was to establish a DNA collection and clinical database from U.

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Ciliopathies are caused by mutations in genes encoding proteins required for cilia organization or function. We show through colocalization with PCM-1, that OFD1 (the product of the gene mutated in oral-facial-digital syndrome 1) as well as BBS4 and CEP290 (proteins encoded by other ciliopathy genes) are primarily components of centriolar satellites, the particles surrounding centrosomes and basal bodies. RNA interference experiments reveal that satellite integrity is mutually dependent upon each of these proteins.

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Unlabelled: A 5-year follow-up study was performed in female RA patients with established disease looking at vertebral fractures, scored on spinal X-rays, and non-vertebral fractures. We found a high incidence rate of vertebral and non-vertebral fractures in these patients compared to population-based studies.

Introduction: The aim of this study is to investigate the incidence of vertebral and non-vertebral fractures over a 5-year period in a cohort of postmenopausal patients with established rheumatoid arthritis (RA).

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Introduction: Bronze drinking vessels famous for their intricate carvings and used by the aristocracy in the Chinese Shang dynasty (1555-1145 BCE) are known to have been fabricated with alloys containing soft metallic lead. The contribution of lead leaching from such vessels into the fermented grain wines drunk by the Chinese nobility in ancient times has not been previously estimated.

Methods: Three bronze vessels containing 8% lead by weight were fabricated to resemble the late Shang bronze goblets.

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The planar cell polarity (PCP) pathway, incorporating non-canonical Wnt signalling, controls embryonic convergent (CE) extension, polarized cell division and ciliary orientation. It also limits diameters of differentiating renal tubules, with mutation of certain components of the pathway causing cystic kidneys. Mutations in mouse Vangl genes encoding core PCP proteins cause neural tube defects (NTDs) and Vangl2 mutations also impair branching of embryonic mouse lung airways.

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Autosomal dominant polycystic kidney disease (ADPKD) in children is sometimes considered to be a benign condition, with morbidity manifesting in adulthood. Therefore, diagnostic screening of children at risk is controversial. The aim of our study was to to compare the manifestations of ADPKD in children diagnosed by postnatal ultrasound (US) screening versus those presenting with symptoms.

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Renal tract malformations (RTMs) account for about 40% of children with end-stage renal failure. RTMs can be caused by mutations of genes normally active in the developing kidney and lower renal tract. Moreover, some RTMs occur in the context of multi-organ malformation syndromes.

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Urinary voiding dysfunction in childhood, manifesting as incontinence, dysuria, and urinary frequency, is a common condition. Urofacial syndrome (UFS) is a rare autosomal recessive disease characterized by facial grimacing when attempting to smile and failure of the urinary bladder to void completely despite a lack of anatomical bladder outflow obstruction or overt neurological damage. UFS individuals often have reflux of infected urine from the bladder to the upper renal tract, with a risk of kidney damage and renal failure.

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Objective: The objective of this study was to examine 5-year change in cortical hand BMD in female RA patients with established disease. Further, possibly baseline predictors of 5-year loss in digital X-ray radiogrammetry (DXR)-BMD were studied.

Methods: This 5-year multicentre, longitudinal study included patients from Amsterdam (the Netherlands), Truro (UK) and Oslo (Norway).

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Acute encephalopathy from childhood lead poisoning is fortunately rare. However, in pediatric patients with developmental disabilities and pica, there is a risk of lead exposure at a dose commensurate with encephalopathy, coupled with a risk of delayed diagnosis because of difficulty in distinguishing between baseline and altered behavior. We report here a 4-year old autistic boy who presented to the pediatrician's office with gastrointestinal symptoms and behavioral changes and was at first thought to have a viral syndrome.

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Background: Significant lead poisoning has been associated with imported nonpaint products.

Objectives: To describe cases of pediatric lead intoxication from imported Indian spices and cultural powders, determine lead concentrations in these products, and predict effects of ingestion on pediatric blood lead levels (BLLs).

Patients And Methods: Cases and case-study information were obtained from patients followed by the Pediatric Environmental Health Center (Children's Hospital Boston).

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Low back pain is a major cause of morbidity in high-, middle- and low-income countries, yet to date it has been relatively under-prioritised and under-funded. One important reason may be the low ranking it has received relative to many other conditions included in the previous Global Burden of Disease studies, due in part to a lack of uniformity in how low back pain is defined and a paucity of suitable data. We present an overview of methods we have undertaken to ensure a more accurate estimate for low back pain in the Global Burden of Disease 2005 study.

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Article Synopsis
  • Work disability due to rheumatoid arthritis (RA) is influenced by various factors including demographic and societal variables, with evidence suggesting biologic agents may reduce disability rates, though this research is primarily from Western contexts.
  • The QUEST-RA study analyzed data from over 8,000 RA patients across various countries to assess work status and clinical outcomes.
  • Findings revealed that a significant percentage of initially employed individuals became disabled within a few years, but those who remained employed showed better overall health, regardless of their country’s economic status.
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The use of complementary and alternative therapies in children has recently shown explosive growth, despite little scientific evidence of benefit, a need for better regulatory oversight, and continuing gaps in the knowledge and attitudes of pediatric health professionals.

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An intact genome is essential for kidney growth and differentiation, but less is known about whether, and how, an altered fetal milieu modifies these processes. Maternal low-protein diets perturb growth of the metanephros, the precursor of the mature kidney. Fetal corticosteroid overexposure may, in part, mediate this, because such diets downregulate placental 11beta-hydroxysteroid dehydrogenase-2, which degrades maternal corticosteroids.

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Primary vesicoureteric reflux accounts for approximately 10% of kidney failure requiring dialysis or transplantation, and sibling studies suggest a large genetic component. Here, we report a whole-genome linkage and association scan in primary, nonsyndromic vesicoureteric reflux and reflux nephropathy. We used linkage and family-based association approaches to analyze 320 white families (661 affected individuals, generally from families with two affected siblings) from two populations (United Kingdom and Slovenian).

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After the basic shape of the mammalian ureter is established, its epithelia mature and a coat of smooth muscle cells differentiate around nascent urothelia. The ureter actively propels tubular fluid from the renal pelvis to the bladder, and this peristalsis, which starts in the fetal period, requires coordinated smooth muscle contraction. Teashirt-3 (Tshz3) is expressed in smooth muscle cell precursors that form the wall of the forming mammalian ureter.

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Purpose Of Review: The present review highlights general advances concerning angiopoietin (Ang) biology and then focuses on possible roles of these molecules within healthy and diseased glomeruli.

Recent Findings: Early experiments showed that Ang1 stabilizes newly formed vessels and reduces vascular permeability, with Ang2 blocking Ang1 activation of the Tie2 receptor. Recent studies found that Ang2 can directly stimulate Tie2 and that both Ang1 and Ang2 can be pro-inflammatory.

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