太湖地区动脉粥样硬化病人的中医证素分布

目的根据动脉粥样硬化(atherosclerosis,AS)病人的四诊信息,观察太湖地区AS病人中医证候要素(证素)的分布规律。方法依据AS纳入病例标准,随机选取AS住院病人119例,作为实验组,由专职主任中医师进行中医"证素"评估。结果 119例AS病人中,单证素者79例,多证素兼夹者40例。单证素组中痰浊30例(38.0%),阴虚16例(20.3%),气虚14例(17.7%),血瘀4例(5.1%),肝火14例(17.7%),阳虚1例(1.3%)。单证素与多证素兼夹AS病人的累计证素组中,痰浊60例(34.7%),阴虚47例(27.2%),气虚28例(16.2%),血瘀23例(13.3%),肝火14例(8.1%),阳虚1例(0.6%)。单证素组79例中,女性47例,痰浊证素为21例(44.7%),肝火证素12例(25.5%);男性32例,痰浊证素9例(28.1%),阴虚证素10例(31.3%)。结论太湖地区AS病人常见的中医"证素"有痰浊、阴虚、气虚、肝火、血瘀,其分布规律呈现虚实分布的特点,实性证素和虚性证素大体上均衡分布,体现了AS虚实夹杂的病理属性。痰浊可能是该地区AS病人最主要的病理因素;血瘀证素则多以兼夹证素的形式出现;而虚性证素中以气虚证素、阴虚证素最多见,阳虚证素出现极少;肝火证素也占了一定比例,提示火热之邪也可以是导致AS的病理因素。AS证素在性别分布上,男性病人以痰浊证素、阴虚证素出现几率较高,女性病人以肝火证素、痰浊证素出现的几率较高,提示对于AS研究应根据不同性别特点进一步深入研究。     

Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with post-myocardial infarction syndrome (Dressler's syndrome)

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurred in a patient with post-myocardial infarction syndrome (Dressler's syndrome). Although Sweet's syndrome has been described in association with leukemias, other malignant disorders, and a variety of chronic inflammatory disorders, it has not been reported associated with Dressler's syndrome. Sweet's syndrome is reviewed with regard to its associations and to its pathogenesis.     

Evans syndrome in adults - incidence,prevalence, and survival in a nationwide cohort

Patients with Evans syndrome have both immune thrombocytopenia and autoimmune hemolytic anemia, but little is known about the epidemiology of this rare syndrome. Evans syndrome can be primary or secondary. This nationwide retrospective study linked health registries to identify 242 patients with Evans syndrome in Denmark in 1977-2017. For comparison, we identified three age-matched and sex-matched cohorts of patients with only immune thrombocytopenia or only autoimmune hemolytic anemia, and a general population cohort. The Evans syndrome cohort had a mean age of 58.5 years at diagnosis, 51.2% were women, and 27.3% were classified as secondary Evans syndrome. The annual Evans syndrome incidence and prevalence rose significantly during the study period, to 1.8 per million person-years and 21.3 per million persons, respectively, in 2016. The median survival with Evans syndrome was 7.2 years (primary Evans syndrome: 10.9 years; secondary Evans syndrome: 1.7 years). Secondary Evans syndrome was associated with higher mortality rates than any of the other cohorts, with a 5-year survival of 38%. Among patients with Evans syndrome, the prevailing causes of death were bleeding, infections, and hematological cancer. In conclusion, we found that both primary and secondary Evans syndrome conferred a poor prognosis. Lethal complications probably derive primarily from manifestations of underlying autoimmune hemolytic anemia and immune thrombocytopenia. Our findings suggested that suspicion of Evans syndrome should prompt vigilant clinical follow-up. International collaborations are warranted to advance our knowledge of optimal management of this rare disease.     

Eosinophilic fasciitis is clinically distinguishable from the eosinophilia-myalgia syndrome and is not associated with L-tryptophan use

Induration of the skin develops in a majority of patients with the eosinophilia-myalgia syndrome associated with L-tryptophan, and bears striking clinical and histopathological resemblance to eosinophilic fasciitis (EF). These similarities have led to the suggestion that eosinophilia-myalgia syndrome and EF are the same disease. To study the relationship of eosinophilia-myalgia syndrome and EF, we ascertained the prevalence of L-tryptophan use in a cohort of patients with EF, and compared their clinical and laboratory findings to those of patients with eosinophilia-myalgia syndrome associated cutaneous involvement. None of 11 patients who were diagnosed as having EF between 1970 and 1989 used L-tryptophan containing preparations prior to the onset of their illness. Marked clinical and laboratory test differences were observed between patients with EF and eosinophilia-myalgia syndrome. Patients with eosinophilia-myalgia syndrome had a more acute onset, more severe symptoms, higher frequency of rash and of pulmonary, cardiac, gastrointestinal, neurologic, myopathic and thyroid involvement compared to patients with EF. Corticosteroid therapy resulted in improvement of cutaneous involvement in 88% of patients with EF but it was only partially successful in patients with eosinophilia-myalgia syndrome. Hospitalization and fatalities occurred only among patients with eosinophilia-myalgia syndrome. These observations demonstrate that eosinophilia-myalgia syndrome is a more severe disease with multisystemic involvement that can be clinically distinguished from EF. In contrast to eosinophilia-myalgia syndrome, EF is not associated with L-tryptophan ingestion.     

Acquired hypophosphatemia osteomalacia associated with Fanconi’s syndrome in Sjögren’s syndrome

Sjögren’s syndrome is an autoimmune disorder involving exocrine glands that occurs alone or in association with various autoimmune and connective tissue diseases. The severity of Sjögren’s syndrome ranges from isolated sicca syndrome to severe complications such as vasculitis, lung and renal involvement. Overt or latent renal tubular acidosis caused by autoimmune tubulointerstitial nephritis, is a common extraglandular manifestation in Sjögren’s syndrome. Osteomalacia is a rare complication of renal tubular acidosis, and it was reported to be associated with distal renal tubular acidosis in Sjögren’s syndrome. We report a 60-year-old woman who presented with multiple bone deformity and general muscle weakness. Osteomalacia was secondary to Fanconi’s syndrome, and the Fanconi’s syndrome was a result of renal involvement in Sjögren’s syndrome. Fanconi’s syndrome is a rare kidney manifestation in Sjögren’s syndrome. It may be latent and may precede the subjective sicca symptoms. These findings suggest that evidence for Sjögren’s syndrome should be sought in adult patients with unexplained osteomalacia and renal tubular acidosis, even in the absence of subjective sicca syndrome. Conversely, in patients with Sjögren’s syndrome, early investigation and treatment of renal tubular dysfunction may prevent future complications, such as osteomalacia.     

Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: a case report

Dubin-Johnson syndrome is a chronic, benign, intermittent jaundice, mostly of conjugated hyperbilirubinemia. The level of bilirubin is not expected to be more than 20 mg/dl in this syndrome. In this article, we report a patient who was evaluated for hyperbilirubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome coexisting with hereditary spherocytosis. We suggest that other diseases should be investigated if patients with Dubin-Johnson syndrome present with severe hyperbilirubinemia. Dubin-Johnson syndrome accompanied by hemolytic diseases might also have high coproporphyrin levels (as in Rotor's syndrome) than expected in pure Dubin-Johnson syndrome.     

Dropped head syndrome: report of two cases

PURPOSE: The dropped head syndrome is characterized by an abnormal bending of the head to the body, mainly affecting old people. It corresponds to an alteration of the cervical extensor muscles, revealing in some cases a neuromuscular disease. In some cases, the etiology of this syndrome remains unknown. EXEGESIS: We report here two cases with dropped head syndrome. The first clinical case concerned a 78-year old man, presenting a dropped head syndrome revealing a myasthenia. The syndrome disappeared with specific therapy. The second clinical case was a dropped head syndrome developed in the context of severe depressive syndrome in a 71-year old woman. The etiological screening did not reveal any underlying disease. Counteracting the syndrome was successfully obtained with early physiotherapy. CONCLUSION: The dropped head syndrome can reveal a general disease such as myasthenia or amyotrophic lateral sclerosis. Therefore, investigation needs first to eliminate underlying diseases. If no etiology is found, the dropped head syndrome is considered of an unknown neuromuscular origin or a psychosomatic disease. In this latter case, physiotherapy may be beneficial.     

High incidence of pacemaker syndrome in patients with sinus node dysfunction treated with ventricular-based pacing in the Mode Selection Trial (MOST)

OBJECTIVES: We evaluated the incidence, predictors, and treatment of pacemaker syndrome in patients with sinus node dysfunction treated with ventricular-based (VVIR) pacing in the Mode Selection Trial (MOST). BACKGROUND: Pacemaker syndrome, or intolerance to VVIR pacing, consists of cardiovascular signs and symptoms induced by VVIR pacing. METHODS: The definition of pacemaker syndrome required that a patient with single-chamber VVIR pacing develop either congestive signs and symptoms associated with retrograde conduction during VVIR pacing or a >or=20 mm Hg reduction of systolic blood pressure during VVIR pacing, associated with reproducible symptoms of weakness, lightheadedness, or syncope. RESULTS: Of 996 patients randomized to VVIR pacing, 182 (18.3%) met criteria for pacemaker syndrome in follow-up. Pacemaker syndrome occurred early in most patients (13.8% at 6 months, 16.0% at 1 year, increasing to 19.7% at 4 years). Baseline univariate predictors of pacemaker syndrome included a lower sinus rate and higher programmed pacemaker rate. Previous heart failure, ejection fraction, and drop in systolic blood pressure with VVIR pacing at implantation did not predict the development of pacemaker syndrome. Post-implantation predictors of pacemaker syndrome were a higher percentage of paced beats, higher programmed low rate, and slower underlying spontaneous sinus rate. Quality of life decreased at the time of diagnosis of pacemaker syndrome and improved with reprogramming to atrial-based pacing. CONCLUSIONS: Severe pacemaker syndrome developed in nearly 20% of VVIR-paced patients and improved with reprogramming to the dual-chamber pacing mode. Because prediction of pacemaker syndrome is difficult, the only way to prevent pacemaker syndrome is to implant atrial-based pacemakers in all patients.     

Antiphospholipid antibodies in HELLP syndrome: clinical and biological study in 68 women

PURPOSE: Pregnancy complicated by the HELLP syndrome and antiphospholipid syndrome have rarely been reported. We report a study on anticardiolipin antibodies in HELLP syndrome. METHODS: Between March 1996 and September 1999, anticardiolipin antibodies were checked in all women with HELLP syndrome hospitalised in a maternity of the North of France. The women with positive anticardiolipin antibodies were seen month later in a internal medicine department. RESULTS: In the period 68 women with HELLP syndrome were checked for anticardiolipin antibodies. Apl were present in 9 patients (Incidence 42.8/1000 HELLP Year). They persisted after the accident only in 3 patients. Antiphospholipid syndrome was diagnosed in 2 patients, prevalence between the HELLP syndrome for the 42 month period was 0.03. CONCLUSIONS: HELLP syndrome may be a manifestation linked to the antiphospholipid syndrome and may revealed it.     

Primary antiphospholipid syndrome presenting with abdominal angina and splenic infarction

The antiphospholipid syndrome is an autoimmune hypercoagulability syndrome in which a wide variety of thromboembolic diseases may occur. Gastrointestinal manifestations associated with vascular occlusion include Budd-Chiari syndrome, hepatic and splenic infarction, pancreatitis, omental and intestinal infarction, and esophageal variceal bleeding due to portal vein thrombosis, but chronic mesenteric ischemia associated with mesenteric arterial thrombosis is very rare in this syndrome. We experienced a female patient with primary antiphospholipid syndrome with abdominal angina and splenic infarction associated with celiac trunk and mesenteric arterial thromboses. This is the first report describing chronic mesenteric ischemia and splenic infarction in a patient with primary antiphospholipid syndrome.     

The metabolic syndrome and coronary heart disease in older women: findings from the British Women's Heart and Health Study.

AIMS: To compare two proposed definitions of the metabolic syndrome and to determine the clinical importance of the syndrome with respect to its association with coronary heart disease (CHD). METHODS: Cross-sectional study of 3770 women aged 60-79 years randomly selected from 23 British towns. RESULTS: The prevalence of the metabolic syndrome was high in this population and similar with both definitions: 28.2% (95% confidence interval 26.8, 29.7%) of the women had metabolic syndrome according to a modified version of the WHO definition, and 29.2% (27.7, 30.7%) had the ATP III-defined syndrome. There was reasonable agreement between the two definitions, with 79% of the participants being similarly classified by both definitions. The syndrome was associated with prevalent CHD, with the magnitude of the association with CHD being similar for both definitions. The odds ratio (95% confidence interval) for the age, smoking, physical activity, adult and childhood social class adjusted association of the WHO defined syndrome with prevalent CHD was 1.45 (1.19, 1.75) and for the ATP III-defined syndrome was 1.53 (1.27, 1.85). Insulin resistance alone, hypertension alone and dyslipidaemia alone were all associated with CHD, with the magnitudes of these associations being similar to those for the WHO and ATP III-defined syndrome with CHD. CONCLUSIONS: The prevalence of the metabolic syndrome is high in older British women and is associated with CHD. There is reasonable agreement between a modified version of the WHO definition and the ATP III definition of the syndrome, and both are similarly associated with CHD. Single components of the syndrome are associated with CHD to a similar magnitude as the syndrome.     

Autosomal-dominant giant platelet syndromes: a hint of the same genetic defect as in Fechtner syndrome owing to a similar genetic linkage to chromosome 22q11-13

Families with 3 different syndromes characterized by autosomal dominant inheritance of low platelet count and giant platelets were studied. Fechtner syndrome is an autosomal-dominant variant of Alport syndrome manifested by nephritis, sensorineural hearing loss, and cataract formation in addition to macrothrombocytopenia and polymorphonuclear inclusion bodies. Sebastian platelet syndrome is an autosomal-dominant macrothrombocytopenia combined with neutrophil inclusions that differ from those found in May-Hegglin syndrome or Chediak-Higashi syndrome or the Dohle bodies described in patients with sepsis. These inclusions are, however, similar to those described in Fechtner syndrome. Other features of Alport syndrome, though, including deafness, cataracts, and nephritis, are absent in Sebastian platelet syndrome. Epstein syndrome is characterized by macrothrombocytopenia without neutrophil inclusions, in addition to the classical Alport manifestations-deafness, cataracts, and nephritis-and it is also inherited in an autosomal-dominant mode. We mapped the disease-causing gene to the long arm of chromosome 22 in an Italian family with Fechtner syndrome, 2 German families with the Sebastian platelet syndrome, and an American family with the Epstein syndrome. Four markers on chromosome 22q yielded an LOD score greater than 2.76. A maximal 2-point LOD score of 3.41 was obtained with the marker D22S683 at a recombination fraction of 0.00. Recombination analysis placed the disease-causing gene in a 3.37-Mb interval between the markers D22S284 and D22S693. The disease-causing gene interval in these 3 syndromes is similar to the interval described recently in an Israeli family with a slightly different Fechtner syndrome than the one described here. Recombination analysis of these 3 syndromes refines the interval containing the disease-causing gene from 5.5 Mb to 3.37 Mb. The clinical likeness and the similar interval containing the disease-causing gene suggest that the 3 different syndromes may arise from a similar genetic defect.     

Severe thrombophilia with antiphospholipid syndrome and hyperhomocysteinemia in a patient with Schnitzler's syndrome

Schnitzler's syndrome is a rare condition with chronic urticaria, intermittent fever, bone pain, and a monoclonal IgM gammopathy. Most patients have a chronic and indolent course, but a small number ultimately progress to a lymphoplasmacytic malignancy. We describe a patient with Schnitzler's syndrome who entered an accelerated phase of clinical deterioration with repeated thromboses of the cerebral and coronary arteries that ultimately led to a fatal outcome. We found that the he fulfilled the Sapporo criteria for definite antiphospholipid syndrome and had elevated blood levels of homocysteine during the active clotting phase of the disease. We suggest that the association with immune-mediated or metabolic disorders, such as the antiphospholipid syndrome and hyperhomocysteinemia, may unfavorably affect the otherwise chronic and stable course of patients with Schnitzler's syndrome. The systemic clotting disorder, the association with the antiphospholipid syndrome and hyperhomocysteinemia, and the biclonal rather than monoclonal IgM gammopathy were striking features of this atypical case of Schnitzler's syndrome.     

Lacrimo-auriculo-dento-digital syndrome with QT prolongation

We present a case of a young boy in whom lacrimo-auriculo-dental-digital (LADD) syndrome with QT prolongation was detected. According to Bazett's formula, corrected QT was 504 ms. There were no published data about LADD syndrome with QT prolongation. This is the first case concerning LADD syndrome associated with QT prolongation. It is known that deafness and QT prolongation occur in potassium channel dysfunction. QT prolongation and deafness may be associated with potassium channel dysfunction in patients with LADD syndrome. As a result QT prolongation may be a new component of the LADD syndrome.     

Inflammatory biomarkers and oxidative stress measurements in patients with systemic lupus erythematosus with or without metabolic syndrome

The aims of the present study were to report the frequency of metabolic syndrome in systemic lupus erythematosus (SLE); to verify differences in inflammatory biomarkers and oxidative stress in SLE patients with or without metabolic syndrome; and to assess which metabolic syndrome components are associated with oxidative stress and disease activity. The study included 58 SLE patients and 105 controls. SLE patients were divided in two groups, with and without metabolic syndrome. 41.4% patients met the criteria for metabolic syndrome compared with 10.5% controls. Patients with SLE and metabolic syndrome had significantly raised serum uric acid, C-reactive protein (CRP), lipid hydroperoxides, and protein oxidation when compared with patients with SLE without metabolic syndrome. Lipid hydroperoxides were correlated with CRP, whereas protein oxidation was associated with waist circumference and uric acid. There was a positive association between serum C3 and C4 and glucose and between C3 and CRP. SLE disease activity index (SLEDAI) scores were positively correlated with body mass index (BMI) and waist circumference (WC). In conclusion, SLE patients have a high prevalence of metabolic syndrome and this syndrome directly contributes to increase inflammatory status and oxidative stress. Inflammatory processes, being overweight/obese, and uric acid may favor oxidative stress increases in patients with SLE and metabolic syndrome. C3 and C4 may have a positive acute-phase protein behavior in patients with SLE.     

Thrombotic microangiopathy with liver, gut, and bone infarction (catastrophic antiphospholipid syndrome) associated with HELLP syndrome

Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome is a thrombotic microangiopathy complicating pregnancy and shares many clinical and biological features with thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Thrombotic microangiopathy is also a pathological feature of catastrophic antiphospholipid syndrome (CAPS). An association between refractory HELLP syndrome and antiphospholipid syndrome (APS) has been reported in a few cases. We describe a 19-year-old woman with APS and multiorgan failure conforming to a diagnosis of CAPS who developed refractory HELLP syndrome.     

Toxic-shock-like-syndrome due to Streptococcus pneumoniae sinusitis

We describe a patient with Streptococcus pneumoniae sinusitis associated with a severe sepsis syndrome and desquamative rash whose clinical illness strongly resembled toxic-shock syndrome. Assay of convalescent serum for antibodies to toxic-shock syndrome toxin 1 was negative. This case suggests the possibility of an additional bacterial pathogen associated with toxic-shock syndrome.     

Postural Orthostatic Tachycardia in a Teenager with Klinefelter Syndrome

To our knowledge this is the first reported case of postural orthostatic tachycardia syndrome (POTS) in a patient with Klinefelter syndrome. We describe a classic clinical presentation of POTS in an adolescent male with Klinefelter syndrome. Although the etiology of POTS appears to be multifactorial, there is a strong female predominance that suggests a genetic basis. Our patient with Klinefelter syndrome may further support a link with POTS to the X chromosome.     

Clinical Characteristics and Outcomes of Patients With Takotsubo Syndrome

BackgroundCharacteristics and outcomes of patients with takotsubo syndrome remain to be defined. The goal of this study was to report the characteristics and long-term outcomes of patients presenting with takotsubo syndrome compared with other patients presenting with acute myocardial infarction (AMI) in a community-based population.MethodsThis retrospective population-based study included patients hospitalised for AMI from 2006 to 2016. Those patients with takotsubo syndrome were compared with the patients with AMI. The primary outcome was all-cause mortality. Matching was performed to assemble a cohort of patients with similar baseline characteristics.ResultsAmong 26,015 patients hospitalised with an initial diagnosis of AMI, 530 (2.0%) were diagnosed with takotsubo syndrome. Patients with takotsubo syndrome were older (68.3 ± 11.3 vs 65.6 ± 12.2 years) and more likely to be women (93.4% vs 30.7%). Concomitant hypothyroidism, rheumatologic disorders, and lung disease were more prevalent in the takotsubo syndrome group, whereas diabetes and hyperlipidemia were less prevalent. Mortality was lower in the takotsubo syndrome group (1-year mortality 4.0% vs 8.9%; P < 0.001). The 530 patients with takotsubo syndrome were matched with 1,315 AMI patients with similar baseline characteristics. At a follow-up of 5.4 ± 3.3 years, patients with takotsubo syndrome had a lower risk for all-cause death than other patients who presented with AMI (hazard ratio 0.59, 95% CI 0.47-0.76).ConclusionsAmong patients presenting with AMI, patients with takotsubo syndrome were older and more likely to be women. Patients with takotsubo syndrome had better long-term outcomes compared with matched AMI patients.     

What Do We Currently Know About Metabolic Syndrome and Atrial Fibrillation?

Metabolic syndrome represents a cluster of atherogenic risk factors including hypertension, insulin resistance, obesity, and dyslipidemia. Considering that all of these risk factors could influence the development of atrial fibrillation, an association between atrial fibrillation and the metabolic syndrome has been suggested. Additionally, oxidative stress and inflammation have been involved in the pathogenesis of both metabolic syndrome and atrial fibrillation. The mechanisms that relate metabolic syndrome to the increased risk of atrial fibrillation occurrence are not completely understood. Metabolic syndrome and atrial fibrillation are associated with increased cardiovascular morbidity and mortality. Because atrial fibrillation is the most common arrhythmia, and along with the prevalence of metabolic syndrome constantly increasing, it would be very important to determine the relationship between these 2 entities, especially due to the fact that the risk factors of metabolic syndrome are mainly correctable. This review focused on the available evidence supporting the association between metabolic syndrome components and metabolic syndrome as a clinical entity with atrial fibrillation.