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OBJECTIVES: The prevailing linear reductionist medical model seems unable to explain complex multisymptomatic illnesses such as fibromyalgia (FM) and similar maladies. Paradigms derived from the complexity theory may provide a coherent framework for these elusive illnesses. Along these lines is the proposal that FM represents a degradation of our main complex adaptive system (the autonomic nervous system, ANS), in a failed effort to adjust to a hostile environment. Healthy complex systems have fractal structures. Heart rate fractal-like variability reflects resilient ANS performance. Our aim was to measure the heart rate variability (HRV) fractal scaling index in FM patients and to correlate this index with clinical symptoms. METHOD: We studied 30 women with FM and 30 controls. All participants filled out questionnaires assessing the severity of FM. The HRV fractal scaling index was estimated during 24 h using detrended fluctuation analysis (DFA). RESULTS: The fractal scaling index alpha-1 was higher in FM patients than in controls (mean ± sd: 1.22 ± 0.10 vs. 1.16 ± 0.09; p = 0.031). There was a positive correlation between the fractal scaling index alpha-1 and the visual analogue scale (VAS) for depression (Spearman's ρ = 0.36, p = 0.04). CONCLUSIONS: The heart rate fractal exponent alpha-1 is altered in FM patients, suggesting a rigid ANS performance. This tangible non-linear finding supports the notion that FM may represent a degradation of our main complex adaptive system, namely the ANS.

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We describe the archeological and imaging findings of a unique specimen (skull and mandible) with leontiasis ossea (LO) that is on display in the National Museum of Anthropology and History in Mexico City. The specimen shows diffuse and irregular periosteal bone proliferation, which produces a grossly nodular appearance involving the neurocranium and the facial skeleton. Plain radiography and helical computed tomography revealed generalized hyperostosis obliterating the maxillary and sphenoidal sinuses and 2 exuberant bony masses arising from the maxilla with encroachment of the anterior nasal aperture.Currently, LO is a purely descriptive term applied to a variety of osseous conditions that have in common hyperostosis of craniofacial bones leading to a leonine appearance. Clinicians who see such lionlike facies should consider the main causes of LO, which include renal osteodystrophy, Paget disease and, as most likely in this specimen, fibrous dysplasia.

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OBJECTIVE: To evaluate whether the presence of glomerulonephritis is or is not associated with the extent of arterial wall inflammatory cell infiltrate in Takayasu arthritis (TA). METHODS: Retrospective chart and pathology review of large artery and kidney specimens of TA autopsy cases. Kidney specimens were classified, according to their histopathological findings, in those with specific glomerular entities and those with non-specific, ischemic and/or hypertensive, glomerular changes. A control group of autopsy kidney specimens was utilized for comparison. Morphometric analysis was used to assess the extent of the arterial inflammatory infiltrates; results were compared among the different groups with kidney lesions. RESULTS: We included 25 kidney specimens from 25 autopsies. Specific glomerular entities were present in 14 specimens; 10 (40%) were classified as diffuse mesangial proliferative glomerulonephritis (DMPG [Group A]), and 4 (16%) as other associated glomerulopathies (Group B). Non-specific changes were observed in 11 (44%) specimens (Group C). The arterial inflammatory infiltrate proportion was 9.4 % for group A, 1.4% for group B, and 2.7% for group C. Furthermore, a larger proportion of vascular inflammation was confirmed for group A when compared with the other groups (p<0.05). Group A patients were younger than those in groups B and C (p<0.005) and exhibited shorter disease duration. CONCLUSION: The presence of DMPG was associated with a larger extent of vascular inflammatory cell infiltrate, suggesting a relationship between both phenomena.

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We describe the palaeopathologic and radiographic findings of the human skeletal remains that belonged to a female who lived in Mexico's viceroyship period (seventeenth and eighteenth centuries A.D.). Radiographic studies showed numerous, radiodense, ovoid, small and well-defined foci in the long tubular bones, sacrum, scapulae and iliac bones. Computed tomography (CT) examination revealed multiple hyperdense foci located in the central marrow portion of the bones. Measurements of attenuation coefficient revealed +1548 HU. The findings are consistent with osteopoikilosis, an uncommon, benign sclerosing bone dysplasia transmitted in an autosomal dominant fashion, which in the clinical setting is important to set apart from different bone pathologies to avoid unnecessary interventions and treatments. To the best of our knowledge, this is the first report of osteopoikilosis in ancient human remains.

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BACKGROUND AND PURPOSE: Despite prominent neurological symptoms reported in Takayasu arteritis (TA), a complete evaluation of the cerebral circulation has not been consistently performed. The purpose of this study is to describe MR angiography (MRA), color Doppler flow imaging, and transcranial Doppler (TCD) findings in the extracranial and intracranial cerebral arteries in TA. METHODS: MRA, color Doppler flow imaging, and TCD were performed in 21 patients with TA. Intima-media thickness was measured in the common carotid artery. The correlation between noninvasive studies and panaorto-arteriography was examined for supraortic vessels. Cerebral angiography findings were compared with the noninvasive methods in 7 patients. Intracranial hemodynamic changes detected by TCD were compared with extracranial circulation lesions assessed by panaorto-arteriography. RESULTS: Noninvasive vascular techniques showed at least 1 abnormality in the extracranial and/or intracranial cerebral arteries in 20 of 21 patients (95%). Both MRA and color Doppler flow imaging showed a substantial correlation in the ability to detect obstructive lesions in supra-aortic vessels compared with panaorto-arteriography. High-resolution ultrasonography displayed common carotid artery wall thickening in 5 vessels that were considered normal by arteriography. In 24% of patients, MRA and TCD showed abnormalities consistent with stenosis of the basal cerebral arteries. In 10 patients with severe extracranial circulation involvement (detected by arteriography), TCD displayed intracranial hemodynamic changes consisting of dampened or blunted waveforms with low pulsatility. CONCLUSIONS: The comprehensive assessment of cerebral circulation in TA patients by noninvasive methods allowed the detection of a high rate of diverse vascular abnormalities in both extracranial and intracranial circulation.

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OBJECTIVE: Hypertrophic osteoarthropathy (HOA) is characterized by the coexistence of digital clubbing and periosteal proliferation of the tubular bones. Localized vascular proliferation associated with platelet/endothelial cell activation are recognized features of this syndrome. Current knowledge suggests that HOA develops from the presence in the systemic circulation of one or more growth factors that are normally inactivated in the lungs. The nature of these purported growth factors has not yet been identified. Vascular endothelial growth factor (VEGF) has several features that may fit in with the pathogenesis of HOA. The objective of our study was to measure serum and plasma levels of VEGF in different groups of patients with HOA. METHODS: We studied 24 patients with HOA; of these, in 12 the HOA was secondary to cyanotic congenital heart disease and in 7 to lung cancer, while 5 represented primary cases. As controls we studied 28 individuals without HOA; of these, 12 were apparently healthy individuals, 7 had cyanosis secondary to chronic obstructive pulmonary disease, and 9 had lung cancer. ELISA was used to measure serum and plasma levels of VEGF. RESULTS: Plasma levels of VEGF were significantly higher in the patients with primary HOA (median 46.2; range 19.4-398.8 pg/ml) and in those with lung cancer-HOA (median 75.5; range 24.6-166.7), compared to healthy controls (median 7.4; range: 0-26.1), p < 0.05. Serum VEGF levels were higher in patients with lung cancer and HOA (median 411.4; range 164.2-959.5 pg/ml) compared with lung cancer patients without HOA (median 74.5; range 13.2-205.4), p < 0.001. CONCLUSIONS: Patients with primary HOA and those with HOA and lung cancer have increased circulating levels of VEGF. This cytokine may play a role in the pathogenesis of HOA.

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The syndrome encompassing the combination of pericarditis, arthritis, and camptodactyly is a rarely described cause of pericardial constriction in children. It is likely that this association is being under-recognized. We report a new case in which the skeletal abnormalities were subtle. The syndrome should be included in the differential diagnosis of any child with persistent non-inflammatory pericardial effusion. A careful search at the bedside for the associated skeletal abnormalities should lead to the correct diagnosis. Pericardiectomy is the treatment of choice.

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The effects of the anti-inflammatory drugs diclofenac, piroxicam, indomethacin, naproxen, nabumetone, nimesulide, and meloxicam on mitochondrial respiration, ATP synthesis, and membrane potential were determined. Except for nabumetone and naproxen, the other drugs stimulated basal and uncoupled respiration, inhibited ATP synthesis, and collapsed membrane potential in mitochondria incubated in the presence of either glutamate + malate or succinate. Plots of membrane potential versus ATP synthesis (or respiration) showed proportional variations in both parameters, induced by different concentrations of nimesulide, meloxicam, piroxicam, or indomethacin, but not by diclofenac. The activity of the adenine nucleotide translocase was blocked by diclofenac and nimesulide; diclofenac also slightly inhibited mitochondrial ATPase activity. Naproxen did not affect any of the mitochondrial parameters measured. Nabumetone inhibited respiration, ATP synthesis, and membrane potential in the presence of glutamate + malate, but not with succinate. NADH oxidation in submitochondrial particles also was inhibited by nabumetone. Nabumetone inhibited O2 uptake in intact cells and in whole heart, whereas the other five drugs stimulated respiration. These observations revealed that in situ mitochondria are an accessible target. Except for diclofenac, a negative inotropic effect on cardiac contractility was induced by the drugs. The data indicated that nimesulide, meloxicam, piroxicam, and indomethacin behaved as mitochondrial uncouplers, whereas nabumetone exerted a specific inhibition of site 1 of the respiratory chain. Diclofenac was an uncoupler too, but it also affected the adenine nucleotide translocase and the H+-ATPase.

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OBJECTIVE: To determine the accumulated 24-hour cardiovascular autonomic modulation and its circadian variations in patients with fibromyalgia, by means of heart rate variability analysis. METHODS: Thirty patients with fibromyalgia and 30 age- and sex-matched controls were studied prospectively. Assessments included a 24-hour ambulatory recording of heart rate variability, time-domain analysis of the accumulated 24-hour R-R interval variations, and power spectral analysis to determine the sympatho/ vagal balance at different hours (calculated as the power spectral density of the low-frequency [0.04-0.15-Hz] sympathetic band divided by the power of the high-frequency [0.15-0.50-Hz] parasympathetic band). RESULTS: Fibromyalgia patients had diminished accumulated 24-hour heart rate variability, manifested by a decreased standard deviation of all R-R intervals (mean +/- SD 126 +/- 35 ms, versus 150 +/- 33 ms in controls; P = 0.008) and a decreased ratio of pairs of adjacent R-R intervals differing by >50 ms (mean +/- SD 12.0 +/- 9.0% versus 20.1 +/- 18.0%; P = 0.031). Patients lost the circadian variations of sympatho/vagal balance, with nocturnal values significantly higher than those of controls at time 0 (mean +/- SD 3.5 +/- 3.2 versus 1.2 +/- 1.0; P = 0.027) and at 3 hours (3.3 +/- 3.0 versus 1.6 +/- 1.4; P = 0.01). CONCLUSION: Individuals with fibromyalgia have diminished 24-hour heart rate variability due to an increased nocturnal predominance of the low-frequency band oscillations consistent with an exaggerated sympathetic modulation of the sinus node. This abnormal chronobiology could explain the sleep disturbances and fatigue that occur in this syndrome. Spectral analysis of heart rate variability may be a useful test to identify fibromyalgia patients who have dysautonomia.

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Current advances in the study of hypertrophic osteoarthropathy are discussed. An update of the classification of hypertrophic osteoarthropathy is given in which the POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is included among the associated diseases. Cyanotic heart disease is the internal illness most closely linked to hypertrophic osteoarthropathy. The bony alterations at the distal phalanxes and on the periosteum of the tubular bones leave a characteristic and indelible mark that can be diagnosed centuries after the death of the individual. Current thinking suggests that localized activation of endothelial cells by an abnormal platelet population, with the ensuing release of fibroblast growth factors, plays a central role in the pathogenesis of the acropachy. Antiphospholipid syndrome may be a feature of cardiogenic hypertrophic osteoarthropathy. Unraveling the mechanisms of hypertrophic osteoarthropathy may help in understanding the pathogenesis of the associated diseases.

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OBJECTIVE: Paleopathology helps to define the origin and the migration of diseases. It has been established that genetic and environmental factors play a role in the pathogenesis of ankylosing spondylitis (AS). We investigated whether AS was present in Mesoamerica before contact with the European civilization. METHODS: We studied the collection of Mesoamerican human skeletal remains preserved at the National Museum of Anthropology of Mexico, searching for features of AS. RESULTS: We found one incomplete specimen of a man of the Postclassic period (900-1521 AD). It showed fusion of the vertebral column from T8 to L5 due to ankylosis of the apophyseal joints and of the spinal processes. The pelvis was not preserved. Radiographs demonstrated ossification of both supraspinous and interspinous ligaments. CONCLUSION: Our findings suggest AS was present in Mesoamerica before the arrival of Europeans.

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Renal artery and venous thrombosis and thrombotic microangiopathy have been related to PAPS. This condition may lead to end-stage renal disease (ESRD). We present 2 patients with PAPS and ESRD who received a living unrelated kidney transplant. Despite intensive anticoagulant therapy, the disease recurred in the grafts.

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Hypertrophic osteoarthropathy is one of the earliest recognized disease entities in the history of medicine. It has a peculiar periosteal proliferation distinctive from other bone diseases. In its advanced stage, it leaves an indelible mark on the skeleton. It has been recently shown that digital clubbing is accompanied by a bone remodeling process of the underlying phalanges. Thus, theoretically, this entity can be recognized in ancient human skeletal remains. We studied part of the collection of skeletal remains from pre-Hispanic Mesoamerica preserved at the National Museum of Anthropology of Mexico City. We examined 1000 specimens and found 2 skeletons with widespread, bilateral, symmetric periosteal proliferation of the tubular bones in addition to the bone remodeling changes of the distal phalanges. One of the specimens was from the Formative period (2000 B.C. to 100 A.D.). We conclude that hypertrophic osteoarthropathy can be recognized in ancient human skeletal remains and that this disease was present in Mesoamerica near the time of the original description of clubbing by Hippocrates about 2500 years ago.

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The diagnosis of acute rheumatic fever has become difficult. A growing number of diseases that were not recognized in the past could fulfill its diagnostic criteria. We emphasize its changing incidence, current knowledge of its pathogenesis, and lesser known clinical features such as pneumonitis, encephalitis and glomerulonephritis.

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We reviewed the clinical features of 26 children with Takayasu's arteritis. In contrast to what is often seen in adults, in this age group inflammation of different systems is a conspicuous feature. Eighty five percent of cases had arterial hypertension. The high incidence of arthritis and heart failure provoked confusion with rheumatic fever. There was a high frequency of positive purified protein derivative (73%) and lymphadenopathy (38%) with a histological picture of caseating granulomas, suggesting a link between Takayasu's arteritis and an atypical mycobacterial infection. No improvement of the arterial obstruction was observed with corticosteroid therapy. Takayasu's arteritis in children presents as an aggressive often lethal disease. The mortality rate was 35% over an observation period of 5 years.

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Based in our own clinical studies, we state our point of view, regarding two controversial issues in the literature: 1) the relationship between digital clubbing and hypertrophic osteoartropathy (HOA), and 2) its pathogenic mechanisms. We agree with the theory which proposes that clubbing is the initial, and most conspicuous sing of a full syndrome, (HOA). These entities develop when the lung fails to inactivate a vasodilator and bone-remodeling substance normally present in the systemic venous circulation. This failure of the lung could be due to exclusion, as is the case in the right-to-left shunts, or because of functional deterioration that develops in conditions such as in lung cancer or cystic fibrosis.

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