Neuromuscular disorders: relevance to anaesthesia and intensive care

Neuromuscular diseases are relatively rare, but it is important for both anaesthetists and intensivists to have a working knowledge of the common diseases as they may complicate general anaesthesia and result in neurogenic respiratory failure. The most common neurological condition seen in the intensive care unit is that of critical illness neuropathy; this subject is covered elsewhere in the journal. The diseases most commonly encountered in general anaesthetic practice include motor neurone disease, Guillain-Barré syndrome, botulism, myasthenia gravis and the muscular dystrophies.     

Neuromuscular disorders: relevance to anaesthesia and intensive care

Although relatively rare, neuromuscular disease is important to both anaesthetists and intensivists as it may complicate general anaesthesia and result in neurogenic respiratory failure. The most common diseases that will be encountered in a general anaesthetic practice include motor neurone disease, Guillain–Barré syndrome, botulism, myasthenia gravis and the muscular dystrophies. The clinical features and anaesthetic implications for these conditions are discussed.     

Surgical complications of bone marrow transplantation

BACKGROUND: We wished to determine the type of diseases in patients who received bone marrow transplant (BMT) that potentially involve the general surgeon at our institution. METHODS: The records of 542 patients who underwent bone marrow transplant at Oregon Health and Sciences University between January 1990 and December 2000 were retrospectively reviewed. Gastrointestinal complications included in the study were gastrointestinal bleeding, venoocclusive disease of the liver, intestinal graft versus host disease, pneumatosis intestinalis, necrotizing enteritis, as well as other more common surgical diseases (eg, appendicitis). RESULTS: Gastrointestinal complications or surgical consultations were noted in 92 of 542 patients (17%). Of these, formal general surgical consultation was obtained in 48 patients (9%). The most common causes for surgical consult were cholecystitis (5), abdominal pain of unknown etiology (5), central line complications (5), small bowel obstruction (4), and appendicitis (4). Twenty-eight (58%) of these patients received an operation. Six patients (13%) died during the same hospitalization as their surgery consult. Forty-four patients with these gastrointestinal symptoms related to transplantation did not receive surgical consult. The mortality in this group was 45%. CONCLUSIONS: The majority of gastrointestinal complications after bone marrow transplant do not require surgical intervention. However, these conditions may overlap the more common reasons for surgical consult and must be identifiable by the general surgeon. Of patients who did require surgical intervention, it was primarily for common surgical diseases.     

Small fiber neuropathy: Diagnosis,causes, and treatment

Small fiber neuropathy, which affects the sensory Aδ and C fibers, is now a major diagnostic and therapeutic challenge. Nearly 7% of the general population have chronic neuropathic pain responsible for severe quality-of-life impairments. Awareness must therefore be raised among clinicians of the somatosensory and autonomic symptoms that can reveal small fiber neuropathy, appropriate diagnostic investigations, most common causes, and best treatment options for each patient profile. To help achieve this goal, the present review article discusses the clinical presentation of neuropathic pain and paresthesia and/or autonomic dysfunction due to involvement of nerves supplying exocrine glands and smooth muscle; normal findings from standard electrophysiological investigations; most informative diagnostic tests (epidermal nerve fiber density in a skin biopsy, laser-evoked potentials, heat- and cold-detection thresholds, electrochemical skin conductance); main causes, which consist chiefly of metabolic diseases (diabetes mellitus, glucose intolerance), dysimmunity syndromes (Sjögren's syndrome, sarcoidosis, monoclonal gammopathy), and genetic abnormalities (familial amyloidosis due to a transthyretin mutation, Fabry disease, sodium channel diseases); and the available symptomatic and etiological treatments.     

Perioperative care of the child with arthrogryposis

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a congenital symptom complex characterized by multiple joint contractures. AMC results from fetal akinesia due to extrinsic factors (oligohydramnios) or primary neurogenic or myopathic conditions of the fetus. METHODS: We retrospectively reviewed our perioperative experience in 12 patients with AMC. RESULTS: Intraoperative issues noted in our patients included difficulties with peripheral IV placement (n = 3), difficult tracheal intubation (n = 4), and intraoperative hyperthermia (n = 4). Difficulties with peripheral IV placement were more common in younger patients (2.3 +/- 0.6 vs 9.2 +/- 3.5 years). Although easy bag-valve-mask ventilation was present in all 12 patients, difficulty with tracheal intubation was noted in four patients. In these four patients, the airway was secured using an LMA (n = 3) or a facemask (n = 1). The patients in whom difficulties with tracheal intubation were encountered were older than patients in whom no difficulties were noted (11.0 +/- 2.9 vs 5.8 +/- 3.9 years). Four patients developed intraoperative temperatures > or =37 degrees C; however, there was no evidence of hypercarbia or tachycardia suggestive of malignant hyperthermia. Postoperative issues included stridor (n = 2) and postoperative atelectasis (n = 3). CONCLUSIONS: The perioperative care of patients with AMC should consider not only the physical manifestations of the disease process, but also the implications of the underlying neuromuscular disease. The most common perioperative issues included difficulties with airway management, problematic intravenous access, and intraoperative hyperthermia.     

A case of undifferentiated embryonic liver sarcoma mimicking cystic hydatid disease in an endemic region of the world

Undifferentiated embryonic liver sarcoma (UELS) is a rare highly malignant neoplasm that predominantly occurs in children between 5 and 10 years of age. The typical radiologic appearance on ultrasound and computed tomography of UELS shows a large septated mass having combined cystic and solid components. These radiographic features, however, are not specific to UELS and are shared by other more common and benign diseases of the liver. For example, cystic hydatid disease (CHD), caused by larvae of the Echinococcus tapeworm, is the most common indication for hepatic operations in children residing in endemic regions of the world. Undifferentiated embryonic liver sarcoma and CHD are 2 diseases that share overlapping presenting features of patient age, symptoms, and radiologic appearance. Surgeons who operate in Echinococcal endemic regions must be aware that not all hepatic cystic masses are necessarily CHD and may be other more rare malignant diseases such as UELS.     

Hospital outcomes and early readmission for the most common gastrointestinal and liver diseases in the United States: Implications for healthcare delivery

BACKGROUNDGastrointestinal (GI) and liver diseases contribute to substantial inpatient morbidity, mortality, and healthcare resource utilization. Finding ways to reduce the economic burden of healthcare costs and the impact of these diseases is of crucial importance. Thirty-day readmission rates and related hospital outcomes can serve as objective measures to assess the impact of and provide further insights into the most common GI ailments.AIMTo identify the thirty-day readmission rates with related predictors and outcomes of hospitalization of the most common GI and liver diseases in the United States.METHODSA cross-sectional analysis of the 2012 National Inpatient Sample was performed to identify the 13 most common GI diseases. The 2013 Nationwide Readmission Database was then queried with specific International Classification of Diseases, Ninth Revision, Clinical Modification codes. Primary outcomes were mortality (index admission, calendar-year), hospitalization costs, and thirty-day readmission and secondary outcomes were predictors of thirty-day readmission.RESULTSFor the year 2013, the thirteen most common GI diseases contributed to 2.4 million index hospitalizations accounting for about $25 billion. The thirty-day readmission rates were highest for chronic liver disease (25.4%), Clostridium difficile (C. difficile) infection (23.6%), functional/motility disorders (18.5%), inflammatory bowel disease (16.3%), and GI bleeding (15.5%). The highest index and subsequent calendar-year hospitalization mortality rates were chronic liver disease (6.1% and 12.6%), C. difficile infection (2.3% and 6.1%), and GI bleeding (2.2% and 5.0%), respectively. Thirty-day readmission correlated with any subsequent admission mortality (r = 0.798, P = 0.001). Medicare/Medicaid insurances, ≥ 3 Elixhauser comorbidities, and length of stay > 3 d were significantly associated with thirty-day readmission for all the thirteen GI diseases.CONCLUSIONPreventable and non-chronic GI disease contributed to a significant economic and health burden comparable to chronic GI conditions, providing a window of opportunity for improving healthcare delivery in reducing its burden.     

Onset and duration of mivacurium-induced neuromuscular block in patients with Duchenne muscular dystrophy

Background. To determine the response to mivacurium, we prospectivelystudied onset time and complete spontaneous recovery from mivacurium-inducedneuromuscular block in patients with Duchenne muscular dystrophy(DMD). Methods. Twelve boys with DMD, age 5–14 yr, seven of themwheelchair-bound, ASA II–III, and 12 age- and sex-matchedcontrols (ASA I) were enrolled in the study. Anaesthesia wasinduced with fentanyl 2–3 µg kg^–1 and propofol3–4 mg kg^–1 titrated to effect, and maintained bycontinuous i.v. infusion of propofol 8–12 mg kg^–1and remifentanil as required. The lungs were ventilated withoxygen in air. Neuromuscular transmission was assessed by acceleromyographyusing train-of-four (TOF) stimulation every 15 s. After baselinereadings, a single dose of mivacurium 0.2 mg kg^–1 wasgiven. The following variables were recorded: (i) lag time;(ii) onset time; (iii) peak effect; (iv) recovery of first twitchfrom the TOF response to 10, 25 and 90% (T₁₀, T₂₅, T₉₀) relativeto baseline; (v) recovery index (time between 25 and 75% recoveryof first twitch); and (vi) recovery time (time between 25% recoveryof first twitch and recovery of TOF ratio to 90%). For comparisonbetween the groups the Mann–Whitney U-test was applied. Results. There were no differences between the groups in lagtime, onset time and peak effect. However, all recorded recoveryindices were significantly (P<0.05) prolonged in the DMDgroup. The median (range) for time points T₁₀, T₂₅ and T₉₀ inthe DMD and control group was 12.0 (8–16) vs 8.4 (5–15)min, 14.1 (9–20) vs 10.5 (7–17) min and 26.9 (15–40)vs 15.9 (12–23) min, respectively. The recovery indexand recovery time were similarly prolonged in the DMD group. Conclusions. These results support the assumption that mivacurium-inducedneuromuscular block is prolonged in patients with DMD. This study was presented at the Annual Meeting of the AmericanSociety of Anaesthesiologists, Las Vegas, October 2004. These authors contributed equally to this work.     

Gestion de la curarisation par rocuronium et sugammadex pour chirurgie abdominale en urgence chez une patiente porteuse d’une myopathie facio-scapulo-humérale

In patients with neuromuscular diseases, the use of rocuronium in the general anesthesia rapid sequence induction provides safety intubation conditions, but induces a deep and prolonged neuromuscular blockade. We report dose reduction to 0.8 mg/kg for a 47-year-old female with Landouzy-Dejerine myopathy. Therefore, less dose of sugammadex was given to reverse the neuromuscular block.     

310例慢性肾小球肾炎患者辨证规律研究

目的：通过对慢性肾小球肾炎患者（CKD2～3期）的中医证候分型、临床症状、中药疗效以及临床指标间相关性研究,探讨本病的中医证型分布特点,为进一步探索中药临床研究提供依据。方法：回顾性收集2008年～2011年入住我院肾病科的慢性肾小球肾炎患者的病史资料、中医临床症状、相关临床指标,运用统计分析方法研究慢性肾小球肾炎的中医辨证分型规律以及治疗方法。结果：（1）慢性肾小球肾炎的中医证型中以气阴两虚型、肝肾阴虚型常见;（2）脾肾阳虚型蛋白尿水平最高,差异具有统计学意义（P〈0.05）;（3）脾肾阳虚型中药治疗有效率最低（66.7%）。结论：慢性肾小球肾炎的中医证候以气阴两虚最为常见,脾肾阳虚型蛋白尿水平最高,治疗有效率最低。     

Celiac disease in patients with systemic lupus erythematosus

ObjectivesCeliac disease (CD) is one of the most common chronic diseases. Celiac disease has been associated with several autoimmune disorders, but the association with systemic lupus erythematosus (SLE) as a systemic autoimmune disease is still controversial. In this study, we aimed to determine the prevalence of biopsy-proven CD in patients with SLE, and to determine the clinical symptoms and laboratory data in these patients.Material and methodsIn a cross-sectional study, SLE patients at a referral clinic were evaluated for gastrointestinal symptoms between March and December 2016. Patients were evaluated by a gastroenterologist, and upper gastrointestinal endoscopy with intestinal biopsy was performed if deemed necessary. The clinical symptoms, laboratory data, and endoscopy results were recorded and compared between groups.ResultsIn total, 130 patients were evaluated in this study. Gastrointestinal symptoms were present in 40% of the patients. Endoscopy was performed in all SLE patients with gastrointestinal symptoms. Four patients (3%) were diagnosed as having CD based on biopsy results and response to a gluten-free diet. Anti-endomysium antibody (AEA) was found to be 100% sensitive and 99.2% specific for the diagnosis of CD in SLE patients, and anti-gliadin antibody (AGA) had a 50% sensitivity and 98% specificity. Patients with comorbid CD and SLE were significantly more likely to have diarrhea, abdominal pain, nausea/vomiting, recurrent oral aphthosis, and anemia.ConclusionsThe results of this study suggest that a significant association is present between CD and SLE. We found a prevalence of 3% for biopsy-proven CD in patients with SLE, which is five times the prevalence of CD in the general population.     

Interdisciplinary lymphology: the best place for each discipline in a team

The term lymphology includes both the physiology and the pathology of the lymphatic system. Many disciplines are involved in the study of the lymphatic system, to correctly diagnose lymphatic diseases and to ultimate provide the best available treatment for the patient. Lymphedema is one of the most common lymphatic diseases, potentially causing significant problems for the patient and for the health system in general. The aim of this article is to discuss the best placement and role for each discipline within an interdisciplinary team in order to provide an effective management of lymphedema and related lymphatic diseases.     

Relevance and feasibility of a systematic screening of multimorbidities in patients with chronic inflammatory rheumatic diseases

Objectives

EULAR recently proposed to screen multimorbidities in chronic inflammatory rheumatic diseases. The aims of the study were to define the most common multimorbidities in chronic inflammatory rheumatic diseases, compare the screening approach performed in the clinic with the recent EULAR recommendations, validate the points to consider for the systematic standardized multimorbidity screening proposed by EULAR and assess feasibility of such a screening in a daily clinic.

豫琼两地原发性肾小球疾病病理类型的变迁对比及临床分析

目的 回顾性分析豫北地区及海南两地近12年原发性肾小球疾病疾病谱的构成演变特点及其意义.方法 分别收集2008年1月至2019年12月于新乡医学院第一附属医院及海南医学院第一附属医院经临床及肾活检诊断为原发性肾小球疾病3985例患者的临床及病理资料,并对其进行对比分析,根据年份分为2008年至2011年、2012年至2...     

Clinical manifestations of Whipple’s disease mimicking rheumatic disorders

Whipple’s disease is a rare, chronic, systemic disorder caused by Tropheryma whipplei infection. The most common symptoms are weight loss, arthralgia, diarrhea and abdominal pain. Other organ involvement can also occur in the patients. Joint manifestations may mimic rheumatoid arthritis or spondyloarthritis. Arthalgia, arthritis, spondylodiscitis, bursitis and/or tenosynovitis are seen in the majority of the patients. This explains why some of the symptoms are misdiagnosed as those of rheumatic diseases. Understanding of Whipple’s disease is important for differential diagnostics of several rheumatic symptoms.     

Hypertonia in children: How and when to treat

Opinion statement Hypertonia in children can be caused by many different diseases. The most common etiology is cerebral palsy. Spasticity and dystonia are the most common types of hypertonia. There are few options for treatment, and usually treatment has an incomplete effect. Therefore, it is necessary to prioritize goals in order to improve overall functional outcome. The use of any intervention will require the ability to verify the magnitude and importance of the outcome in order to ensure that therapy is efficacious. In general, a complex, flexible, and multifaceted approach will be necessary to improve the motor abilities of children with hypertonia.     

IgG4相关腹部疾病的临床及影像学表现

目的 探讨IgG4相关腹部疾病的临床及影像学表现,提高对该病的认识及诊断水平.方法 回顾性分析2012年10月至2021年1月首都医科大学附属北京佑安医院28例IgG4相关腹部疾病的临床及影像学资料.结果 腹部多个器官(2～5个)受累多见,胆管(100.0％)和胰腺(85.7％)最常受累.胆管受累复杂多样,胆总管下段受...     

1148例新疆地区肾活检病理资料分析

目的:分析肾活检资料的地域、种族特点,为肾脏疾病的特异性诊治提供依据.方法:回顾性分析新疆医科大学第一附属医院1 148例肾活检病例的临床资料,进行统计学分析.结果:原发性肾小球肾炎占76.1%,其常见病理类型为非IgA肾病、IgA肾病;继发性肾小球肾炎占13.0%,其中以狼疮性肾炎最为常见;各病理类型分布在维吾尔族与...     

Inflammatory and Infectious Lesions of the Sinonasal Tract

The sinonasal tract is frequently affected by nonneoplastic inflammatory diseases. Inflammatory lesions of the sinonasal tract can be divided into 3 main categories: chronic rhinosinusitis, which encompasses a heterogeneous group of entities, all of which result in mucosal inflammation with or without polyps-eosinophils; infectious diseases; and autoimmune diseases and vasculitides, which can result in midline necrosis and facial deformities. This article reviews the common inflammatory lesions of the sinonasal tract with emphasis on infectious diseases, vasculitis, iatrogenic, and diseases of unknown cause. Many of these lesions can result in midline destruction and result in facial deformity.     

Predictors of Mortality in Acute Renal Failure in a Developing Country: A Prospective Study

Acute renal failure (ARF) occurs in wide range of conditions, making the evaluation of its prognosis a difficult task. Data regarding prognostic factors in ARF in a general population in developing countries are scarce. The objective of the study was to describe predictors of mortality in ARF that are relevant in the developing world. This prospective study was carried out over a one-year period; all hospitalized adults with ARF were included in the study. Predictors of mortality studied included causes of ARF, pre-existing diseases, and severity as well as complications of ARF. Of 33,301 patients admitted during the study period, 294 (0.88%) were either admitted with or developed ARF after hospitalization. Mean age was 43.9 ± 16.9 (18–86 yrs). Sepsis was the most common cause (63.26%). Pre-existing diseases like cardiovascular disease (CVSD), respiratory system disease (RSD), central nervous system disease (CNSD), hypertension, diabetet mellitus (DM), and malignancy were significantly higher in elderly as compared to younger patients. On univariate analysis sepsis, hypoperfusion as a cause of ARF and hospital-acquired ARF were associated with higher mortality. Pre-existing diseases viz. RSD, CVSD, CNSD, and DM had higher mortality. Among the severity and complications of ARF, oliguria, bleeding and infection during the course of ARF and critical illness were predictors of poor outcome. Age >60 yrs was associated with significantly higher mortality. However, on multivariate analysis, only critical illness (odds ratio 37.3), age > 60 years (odds ratio of 5.6), and sepsis as cause of ARF (odds ratio of 2.6) were found to be independent predictors of mortality.