Acute quadriplegic myopathy with myosin-deficient muscle fibres after liver transplantation: defining the clinical picture and delimiting the risk factors

BACKGROUND: In the last few years, rare cases of acute quadriplegic myopathy (AQM*) with myosin-deficient muscle fibres occurring after solid organ transplantation has been reported. The aim of the present study was to review all cases of AQM with myosin deficient fibres seen at our institution among a large series of patients after orthotopic liver transplants (OLT), with special attention to clinical aspects and associated risk factors. Additionally, an extensive review of all ultrastructurally demonstrated cases of AQM in transplant recipients is also included. PATIENTS AND METHODS: Among patients involved in 281 consecutive liver transplant procedures performed in a 4-year period, 3 men and 1 woman developed an arreflexic, flaccid quadriplegia in the immediate postoperative period of OLT. After ruling out other causes of weakness, a muscle biopsy was performed and a loss of thick (myosin) filaments was confirmed by ultrastructural analysis in all cases. Accurate clinical, epidemiological, and evolutive data were recorded. RESULTS: Corticosteroids had been used at usual dosage given to liver transplant recipients; all four patients had several intra- and postoperative complications leading to receiving significantly higher amounts of hemoderivates, to develop renal failure in all cases, and to require a significantly higher number of reoperations within a few days after transplantation than our contemporaneous global series of liver transplant recipients. AQM patients required a significantly longer intensive care unit and hospital stay. Muscular recovery was the rule, but currently a mild myopathic gait remains in three patients. These and other reported cases of AQM do not histologically and clinically differ from AQM seen in other critically ill patients who have not had transplants. CONCLUSIONS: Patients with a complicated intra- and postoperative course of OLT who develop newly acquired acute muscle weakness should be suspected as having acute AQM with myosin-deficient muscle fibres. In this setting, differential diagnosis with other causes of weakness should be carried out, because the prognosis of this myopathy is good with early muscle rehabilitation therapy.     

The use of combined high-frequency jet ventilation and intermittent positive pressure ventilation in bilateral bronchopleural fistulae

Dissatisfaction with the results of conventional respiratory support has led to the use of high-frequency jet ventilation in desperate clinical situations with severe acute respiratory failure. We report a case of a 77 year old man with bilateral bronchopleural fistulae, who was ventilated with a combination of intermittent positive pressure ventilation and high-frequency jet ventilation. The hemodynamic and respiratory advances of this combination are discussed in an overview of the literature.     

Diaphragmatic hernia after transhiatal esophagectomy

Iatrogenic herniation of abdominal contents after transhiatal esophagectomy for the treatment of esophageal cancer is a rare complication. Its appearance in the early postoperative period is usually accompanied by acute respiratory insufficiency; herniation may develop without symptoms and the patient may have to be readmitted with signs of perforation or strangulation of the intestinal loops in the chest cavity. We report the case of a patient in this situation who developed a clinical picture of acute respiratory insufficiency and who required several exploratory procedures (fiberoptic bronchoscopy and echography) before a firm diagnosis could be made. Early diagnosis and emergency treatment reduces complications and increases survival. A lower incidence of iatrogenic herniation of abdominal contents depends on correct closure of the diaphragm. We review correct surgical treatment to decrease the frequency of this complication.     

Life-threatening Dobrava hantavirus infection with unusually extended pulmonary involvement

In Europe, hantavirus infections usually present as hemorrhagic fever with renal syndrome and its mild form nephropathia epidemica, while clinical cases with severe pulmonary affections are extremely rare and appear to be confined to infections by New World hanta viruses in the Americas. We report on a female patient from Northern Germany, who suffered primarily from severe acute respiratory distress syndrome-like pulmonary failure due to Dobrava hantavirus infection that was complicated by acute renal insufficiency.     

Left ventricular pseudoaneurysm after acute influenza A myocardiopericarditis

We describe the successful management of a rare case regarding an adult Caucasian who developed a left inferior ventricular pseudoaneurysm as a consequence of an influenza A virus infection of the upper respiratory tract followed by acute myocardiopericarditis. The cardiovascular features of this case illustrate the importance of raising a sufficient clinical index of suspicion for this common, but potentially lethal, entity.     

ARDS as an unusual presentation of bronchiolitis obliterans organizing pneumonia

We report the case of a 46-year-old patient with liver transplantation who developed an acute respiratory distress syndrome (ARDS). The commonly associated clinical disorders, those associated with direct injury to the lung and those that cause indirect lung injury in the setting of a systemic process, were not responsible for the clinical picture. Finally, because of progressive clinical deterioration, an open-lung biopsy was performed and revealed a bronchiolitis obliterans with organizing pneumonia (BOOP). Physicians should be aware of this rare aetiology of ARDS.     

Surviving severe acute respiratory distress syndrome: utility of open-lung biopsy and large doses of dexamethasone

The etiology of acute respiratory distress syndrome is wide and mortality is extremely high. We describe a patient dying from severe acute respiratory distress syndrome who had a tremendous recovery after receiving dexamethasone (1 g daily). This patient required positive end-expiratory pressure (up to 18 mm/Hg) and fractional inspiratory oxygen (up to 100%). Thirty-six hours after the large dose of corticosteroids, the respiratory mechanics and oxygenation were acceptable for extubation. Acute respiratory distress syndrome was proven and other etiologies of respiratory failure were ruled out by a bedside open-lung biopsy. The biopsy proven acute respiratory distress syndrome dramatically resolved with this salvage therapy. High-dose usage of corticosteroids for acute respiratory distress syndrome has tremendous potential.     

Clinical features and outcomes of mechanically ventilated patients with acute respiratory failure during treatment with immunosuppressive drugs

We examined clinical features and outcomes of mechanically ventilated patient (n = 11) retrospectively who had developed acute respiratory failure during treatments with immunosuppressive drugs. The mean APACHE II score was 22.6, and the mean lung injury score was 2.9. In eight patients chest X-ray and computed tomography showed interstitial pneumonia. Fungus and/or cytomegalovirus were isolated most often from patients with interstitial pneumonia. Observed mortality (72.7%) was significantly (P < 0.001) higher than predicted mortality (45.1%) in the APACHE II score. Patients, who were complicated with septic shock caused by fungus infection, showed poor mortality. These results suggest that the fungus and cytomegalovirus infections might be associated with poor prognosis in patients with acute respiratory failure during treatment with immunosuppressive drugs.     

Défaillance cardiaque et neurologique secondaire à une intoxication accidentelle par l’hydrogène sulfuré : à propos d’un cas

We report the case of a patient who was victim of a hydrogen sulphide poisoning. This poisoning took place following a fall in sewers. The initial clinical presentation was dominated by an acute respiratory failure caused by an acute pulmonary oedema. In this context, non cardiogenic pulmonary oedema is usually evoked in the literature. Our observation supports a left ventricular systolic dysfunction as a possible cause of this cardiorespiratory complication. The initial presentation was also marked by a coma which is often associated with severe poisonings by hydrogen sulphide. Evolution was favourable under symptomatic treatment. We discuss the mechanism of heart and neurological failure.     

Fatal form of phaeochromocytoma presenting as acute pyelonephritis

We report the case of a young man who presented with a clinical picture of acute pyelonephritis. Within 3 h of admission, the patient developed acute respiratory distress associated with tachycardia and shock, and he was transferred to the intensive care unit. Mechanical ventilation of the lungs and symptomatic treatment were started immediately. Abdominal ultrasound revealed the presence of an adrenal tumour with central necrosis indicating a probable phaeochromocytoma. There was no sign of pyelonephritis. Ventricular fibrillation followed by asystole occurred soon after admission. The suddenness of the patient's death did not allow time for further investigation and therapy. The severity of the clinical signs was probably related to a massive release of catecholamines because of necrosis of the tumour, which may have been worsened by the diagnostic procedures performed to investigate the clinical symptoms and signs of acute pyelonephritis.     

Obstruction d'une bronche lobaire par un comprimé de sucralfate: une cause rare d'insuffisance respiratoire aiguë

Obstruction of a lobar bronchus by a sucralfate tablet: a rare cause of acute respiratory failure. We report two cases of patients with chronic respiratory disease who experienced an asphyxia after aspirating a sucralfate tablet that occluded a lobar bronchus. In adults, a foreign body is a rare cause of acute respiratory failure from tracheobronchial occlusion. The sucralfate tablet has the physical property of expanding rapidly when wet (contact with mucosa). After aspiration, the tablet expands to a larger size and can occlude a lobar bronchus, causing acute respiratory failure. In patients at risk of aspiration, we recommend the use of sucralfate in liquid or suspension form.     

Adult respiratory distress syndrome occurring in two patients undergoing cytoreductive surgery plus perioperative intraperitoneal chemotherapy: case reports and a review of the literature

Cytoreductive surgery and perioperative intraperitoneal chemotherapy with mitomycin C and 5-fluorouracil may be considered as an accepted treatment for appendiceal malignancy with mucinous peritoneal carcinomatosis or for pseudomyxoma peritonei. This aggressive approach has been successfully utilized in approximately 500 patients with an acceptable mortality (1.5%) and morbidity (27%). Although pulmonary complications are frequently recorded, life-endangering acute respiratory failure in the absence of pulmonary infection or an obvious source of systemic sepsis has not been previously described. An extensive clinical review of two patients who had a clinical course compatible with acute respiratory distress syndrome without obvious cause except for the cytoreductive surgery and perioperative intraperitoneal chemotherapy itself was undertaken. These two patients developed gradually increasing respiratory distress in the postoperative period. No bacterial or fungal infections of lungs or intra-abdominal sites or sepsis were discovered. These two patients were unusual in that they had extensive cytoreduction, maximal heat with the mitomycin C chemotherapy, and perfusion of both the abdominal cavity and the right pleural space. Reoperation in both patients failed to show a septic source within the abdomen for progressive adult respiratory distress syndrome. We conclude that aggressive cytoreductive surgery plus perioperative intraperitoneal and intrapleural chemotherapy was associated with life-endangering respiratory failure in two patients. No other cause for this condition was evident from an exhaustive review of the clinical course of these two patients. It is possible that this aggressive approach to appendix malignancy with carcinomatosis is sufficiently traumatic to be considered a cause of adult respiratory distress syndrome.     

Effect of sivelestat sodium on acute lung injury after acute aortic dissection

Acute lung injury is a frequent and serious complication in patients with acute aortic dissection (AAD). Elevated neutrophil elastase has been reported to be one of the major determinants occurring in AAD. On admission, we administered sivelestat sodium hydrate, a neutrophil elastase inhibitor, to 11 patients with AAD who were medically treated to prevent lung injury. We compared their clinical course with that of 12 patients of control group in which sivelestat was not used prophylacticaly. Although there were 5 patients (42%) who suffered from respiratory failure and needed mechanical ventilation in the control group, no one needed intubation in the sivelestat group. Our study suggested that sivelestat sodium hydrate could be effective in preventing intubation due to respiratory failure. Further prospective study is necessary to evaluate prophylactic administration of sivelestat sodium hydrate in AAD.     

移植肾切除术后急性心力衰竭合并呼吸衰竭的多学科综合诊疗

目的探讨多学科综合诊疗(MDT)在移植肾切除术后急性心力衰竭(AHF)合并呼吸衰竭治疗中的作用。方法对1例突发移植肾出血致失血性休克,行移植肾切除术后2周发生急性心肌梗死(AMI)合并AHF、急性肺淤血、肺部感染、急性呼吸衰竭患者进行MDT讨论,制定治疗方案并进行效果评价。结果综合MDT讨论意见,给予经鼻高流量氧疗、床旁连续性静脉-静脉血液透析滤过(CVVHDF)降低心脏负荷、抗凝、扩张血管、降低心肌耗氧量、改善心肌重塑、调脂、抗感染及营养支持等综合治疗,临床结局良好,患者恢复规律血液透析治疗。结论应用MDT模式可为移植肾切除术后AHF合并呼吸衰竭患者制定全面有效的个体化治疗方案,提高临床治疗效果,改善患者预后。     

Respiratory syndromes in acute poisoning

Respiratory syndromes in acute poisoning can refer to a wide range of specific clinical syndromes, from acute tracheobronchitis to acute pulmonary edema, chemical pneumonia, acute respiratory distress syndrome and respiratory failure, that occur as a result of direct or indirect effect of chemical substances, drugs and toxins on lungs and airways. Our study attempt to identify, during one-year retrospective study on patients diagnosed with acute poisoning, addressed to Medical Clinic of Emergency Clinic Hospital of Iasi, the respiratory syndromes commonly associated with acute poisoning. We found that the association of toxins, inhalation of gases or volatile substances have a high risk for appearance of respiratory syndromes. The outcome of these patients is influenced by the duration of exposure or the delay of presentation to the hospital after ingestion, and depends on the rapid and aggressive measures for basic life support and intensive care.     

Fatal outcome in a liver transplant recipient with COVID‐19

Liver injury is common in patients with COVID‐19, but little is known about its clinical presentation and severity in the context of liver transplant. We describe a case of COVID‐19 in a patient who underwent transplant 3 years ago for hepatocellular carcinoma. The patient came to clinic with symptoms of respiratory disease; pharyngeal swabs for severe acute respiratory syndrome coronavirus 2 were positive. His disease progressed rapidly from mild to critical illness and was complicated by several nosocomial infections and multiorgan failure. Despite multiple invasive procedures and rescue therapies, he died from the disease. The management of COVID‐19 in the posttransplant setting presents complex challenges, emphasizing the importance of strict prevention strategies.     

Femur fractures and lung complications: a prospective randomized study of reaming

Reaming the intramedullary canal during fixation of femoral shaft fractures may contribute to pulmonary morbidity in patients with trauma. The purpose of our study was to compare acute and late pulmonary complications after reamed or nonreamed nailing of femur fractures. Patients who had femoral shaft fractures were randomized prospectively to a reamed (n = 41) or nonreamed (n = 41) femoral nailing group. Arterial blood gases were measured before and after femur fixation. Ratios of PaO2/FiO2 and alveolar arterial gradients were calculated. Pulmonary complications (acute respiratory distress syndrome) (ARDS), pneumonia, and respiratory failure) were monitored. Age, gender, fracture site, fracture type, time to nailing, length of operation, Injury Severity Score, and Abbreviated Injury Scale-thorax were similar for the two groups. No significant differences were observed in the ratio of PaO2/FiO2 ratios or alveolar arterial (A-a) gradients before and after nailing. The overall incidence of pulmonary complications was 14.6% (eight patients who had reamed nailing and four patients who had nonreamed nailing), and given the sample size, definitive conclusions could not be reached because of inadequate statistical power. We were unable to document differences in pulmonary physiologic response or clinical outcome between patients having reamed and nonreamed femoral nailing. This study may serve as a pilot investigation for other clinical investigations.     

A porcine model of acute quadriplegic myopathy: a feasibility study

BACKGROUND: The mechanisms underlying acute quadriplegic myopathy (AQM) are poorly understood, partly as a result of the fact that patients are generally diagnosed at a late stage of the disease. Accordingly, there is a need for relevant experimental animal models aimed at identifying underlying mechanisms. METHODS: Pigs were mechanically ventilated and exposed to various combinations of agents, i.e. pharmacological neuromuscular blockade, corticosteroids and/or sepsis, for a period of 5 days. Electromyography and myofibrillar protein and mRNA expression were analysed using sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE), confocal microscopy, histochemistry and real-time polymerase chain reaction (PCR). RESULTS: A decreased compound muscle action potential, normal motor nerve conduction velocities, and intact sensory nerve function were observed. Messenger RNA expression, determined by real-time PCR, of the myofibrillar proteins myosin and actin decreased in spinal and cranial nerve innervated muscles, suggesting that the loss of myosin observed in AQM patients is not solely the result of myofibrillar protein degradation. CONCLUSION: The present porcine AQM model demonstrated findings largely in accordance with results previously reported in patients and offers a feasible approach to future mechanistic studies aimed at identifying underlying mechanisms and developing improved diagnostic tests and intervention strategies.     

Microbial inciters of acute asthma in urban Nigerian children.

BACKGROUND--In tropical Africa the role of microbial agents of acute respiratory infections in acute exacerbations of bronchial asthma remains largely unexplored. However, empirical antibacterial therapy is frequently initiated in moderate to severe cases of acute asthma with symptoms of acute respiratory infection. A study was set up to determine how often acute respiratory infection is associated with acute asthma, to identify the associated pathogens, and to proffer appropriate therapeutic suggestions. METHODS--Over a 16 month period, 86 episodes of acute asthma were studied for clinical and laboratory features of acute respiratory infection at the University College Hospital (UCH), Ibadan. Virological diagnosis was based on immunofluorescence studies of nasopharyngeal aspirates and/or serological tests using the microtitre complement fixation technique. Throat swabs and blood were cultured for bacterial agents. RESULTS--Of the 64 cases who presented with rhinorrhoea, 51 (79.7%) were pyrexial (T > or = 37.6 degrees C). Inflammatory changes (frequently interstitial streakiness) were identified in 10 (19.6%) of the 51 chest radiographs; only two of these had lobar shadowing. Significant bacterial isolates were made in only three (3.5%) of the throat swabs and two (2.4%) of the blood cultures from the 86 cases; none had clinical septicaemia. On the other hand, 55 viral agents were identified from 39 (53%) of the 74 subjects studied; 16 (41.0%) had dual viral identifications. Respiratory syncytial virus (RSV) accounted for 20 (36.4%) identifications, parainfluenza virus (PIV) type 3 for 15 (27.3%), and influenza type A (Flu A) for 12 (21.8%). Viral identifications were significantly higher in infants and preschool subjects (< 5 years) and in those presenting with either rhinorrhoea or pyrexia. CONCLUSIONS--The results of this study underscore the importance of viral upper respiratory infections in asthma exacerbations in a tropical setting. The paucity of clinical and investigative features of bacterial acute respiratory infection suggests that there is little rationale for routine antibiotic cover in children with acute exacerbations of asthma in the tropics.     

Awake proning in patients with COVID-19-related hypoxemic acute respiratory failure: A rapid practice guideline

This rapid practice guideline provides evidence-based recommendations for the use of awake proning in adult patients with acute hypoxemic respiratory failure due to COVID-19. The panel included 20 experts from 12 countries, including one patient representative, and used a strict conflict of interest policy for potential financial and intellectual conflicts of interest. Methodological support was provided by the guidelines in intensive care, development, and evaluation (GUIDE) group. Based on an updated systematic review, and the grading of recommendations, assessment, development, and evaluation (GRADE) method we evaluated the certainty of evidence and developed recommendations using the Evidence-to-Decision framework. We conducted an electronic vote, requiring >80% agreement amongst the panel for a recommendation to be adopted. The panel made a strong recommendation for a trial of awake proning in adult patients with COVID-19 related hypoxemic acute respiratory failure who are not invasively ventilated. Awake proning appears to reduce the risk of tracheal intubation, although it may not reduce mortality. The panel judged that most patients would want a trial of awake proning, although this may not be feasible in some patients and some patients may not tolerate it. However, given the high risk of clinical deterioration amongst these patients, awake proning should be conducted in an area where patients can be monitored by staff experienced in rapidly detecting and managing clinical deterioration. This RPG panel recommends a trial of awake prone positioning in patients with acute hypoxemic respiratory failure due to COVID-19.