Global Respiratory Insufficiency Research Articles

Clinical Case 26—Is it possible to live with thrombosed left ventricular assist device (LVAD)?

Abstract LVAD HeartWare was implanted in a 63-year-old patient with end-stage dilated cardiomyopathy. The postoperative course was complicated by massive gastrointestinal (GI) bleeding that required surgical exploration of the GI tract. For the next 1.5 years, the patient has significantly improved functional capacity with proper LVAD function. A month before the incident, an alarm indicating a pump malfunction was recorded. The patient presents with congestive symptoms and an altered sound of LVAD. Laboratory indicates seven times elevated LDH values, low HGB and INR 3.03. Thrombophilia tests were negative. Echo examination does not indicate pump thrombosis, but the pump parameters show increased energy consumption (>6 W) and increased flow (>7 L/min). A diagnosis of LVAD thrombosis was made. Fibrinolytic therapy was contraindicated because of a history of GI bleeding, so a continuous infusion of heparin was started. Despite this, the patient's clinical condition deteriorates, and a decision was made to discontinue the LVAD, to prevent further hemolysis that led to acute kidney injury (AKI). The procedure was performed without endangering the patient. Massive hemolysis, however, leads to the progressive development of AKI requiring CVVHDF. Global respiratory insufficiency develops and mechanical ventilation was started. After six days of intensive treatment, the patient recovered and was discharged home with a thrombosed pump. For the next 2.5 years, the patient was in satisfactory condition during follow-ups, the pump was not explanted. Even sophisticated medical devices carry with them risks for the patient's life despite adequate management. Treatment of LVAD thrombosis depends on patient characteristics and pump-related factors.

EP179: Expanding the phenotype of CLCN6-associated early-onset neurodegeneration

The de novo c.1658A>G (p.Tyr553Cys) missense variant in CLCN6, which encodes the late endosomal Cl-/H+-exchanger ClC-6, was recently identified as a novel pathogenic variant associated with severe, early-onset neurodegenerative disease in 3 unrelated European children. Global developmental delay, generalized hypotonia, respiratory insufficiency, and diffusion restriction on MRI brain were described as diagnostic features. On a cellular level, functional studies demonstrated a gain-of-function in ClC-6 especially prominent at acidic external pH levels, such as those in late endosomes and lysosomes.

Pickwickian syndrome - "the tip of the iceberg" in extremely obese patients

Introduction. Pickwickian syndrome (PS), also known as hypoventilation syndrome in adults, consists of three factors: obesity [Body Mass Index (BMI) > 30 kg/m2], daytime hypercapnia and sleep-disordered breathing, after ruling out other disorders that may cause alveolar hypoventilation. Timely recognition of PS is of utmost importance because such patients have significant morbidity and mortality. However, recent data indicate that PS is under-recognized and under-treated. We report a case of early-identified PS prehospitally with a favorable outcome after hospital treatment. Case outline. A 67-year-old female patient was diagnosed prehospitally, and the diagnosis was later confirmed in hospital. Diagnostic criteria were as follows: BMI > 45,7 kg/m2 (height 170 cm, weight 132 kg), hypercapnia, hypoxemia and respiratory acidosis (pCO2 ? 41 mmHg, pO2 ? 56 mmHg, pH 7.45) in the absence of other causes of hypoventilation. During hospitalization, the following diagnostic procedures were performed: standard laboratory analyses, chest radiography, electrocardiography, abdomen and heart echocardiography. An attempted sleep study (polysomnography) was interrupted due to a drop in oxygen saturation levels. Non-invasive mechanical ventilation and a diet were used as the first line of therapy. However, due to the development of a global respiratory insufficiency, the patient was intubated and placed on a mechanical ventilator. After 30 days of hospital treatment, the patient was released in a satisfactory general condition with recommendations for weight reduction and symptomatic therapy. Conclusion. As obesity is becoming an epidemic of modern society, early recognition and treatment of PS is of crucial importance.

CF Lung Disease - a German S3 Guideline: Module 2: Diagnostics and Treatment in Chronic Infection with Pseudomonas aeruginosa

Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the epithelial lining fluid which leads to chronic inflammation of the airways. Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90 % of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. Pseudomonas aeruginosa (Pa) is the most relevant pathogen in the course of CF lung disease. Colonization and chronic infection are leading to additional loss of pulmonary function. There are many possibilities to treat Pa-infection. This is a S3-clinical guideline which implements a definition for chronic Pa-infection and demonstrates evidence-based diagnostic methods and medical treatment for Pa-infection in order to give guidance for individual treatment options.

Non-invasive respiratory unit in the Hungarian health care system

Treating patients with acute or chronic respiratory insufficiency still poses a major load on the healthcare system. Though there is evidence that treating these patients in high dependency respiratory units results in a shortening of hospital stay, reduces the need of intubation, and decreases mortality. In the Hungarian routine these patients are treated in general wards until the development of global respiratory insufficiency, when they are transferred to intensive care units. The authors present their first year experience on their novel Non-invasive Respiratory Unit established at Semmelweis University.

Emergency extracorporeal membrane oxygenation to treat massive aspiration during anaesthesia induction. A case report

Since first described in 1946 by Mendelson, aspiration of gastric content resulting in severe pulmonary complications is a known hazard of general anaesthesia. We report on a case of massive aspiration of gastric content during induction of general anaesthesia, resulting in severe prolonged hypoxaemia with cardiac arrest, followed by rapid onset of an acute respiratory distress syndrome (ARDS) associated with severe global respiratory insufficiency and severe hypoxia. ARDS was successfully treated using emergency extracorporeal membrane oxygenation within 3 h after the incident.

A ecocardiografia transesofágica nos cuidados intensivos polivalentes: a sua utilidade no doente crítico ventilado.

The use of the transoesophageal echocardiography (TEE) in the critically ill patient admitted to the general intensive care unit begins to turn out to be very important but it is still little spread. The full implementation of the echocardiography in the general intensive cares is compromised by the acoustic window. However, TEE can flyover this difficulty adding to the advantages widely described of the transthoracic echocardiography (TTE) other high values 1. The TEE manages to do the dynamic evaluation of the necessity of fluids, able to discriminate fluid responders, able to distinguish types of shock, assess systolic and diastolic function of both ventricles, able to evaluate other structures of the heart and be a guide to therapy. The use of the echocardiography in the ventilated patient admitted to the general intensive care unit still lack for some definition. This clinical commentary was carried out by the intention of revising the most relevant literature that values the use and efficiency of the TEE in the ventilated critically ill patient in order to explain its use and consequently helping to implement the TEE in clinical practice. So, one managed to define the application of the TEE in the critically ill patient in several clinical scenarios, the haemodynamically unstable patient, the patient with global respiratory insufficiency, the patient with hipoxémia.

Acute Respiratory Distress Syndrome as a presenting manifestation in young patients infected with H1N1 influenza virus

Introduction The new strain of influenza A (H1N1) 2009, often referred to colloquially as “swine flu”, which was first detected in April 2009, raised to a pandemic of which the impact was not completely predictable. As reported, numerous cases with severe respiratory failure were also seen among young previously healthy people. Patients In the present study, we report eight cases of influenza A (H1N1) 2009 admitted to our medical intensive care with severe respiratory failure between November and December 2009 and in January 2011. All patients were older than 30 but younger than 50 years, had clinical and radiological evidence of an Acute Respiratory Distress Syndrome (ARDS) and needed invasive ventilatory support. Results Six of the eight patients had no relevant underlying disease; one had a pre-existing idiopathic lung fibrosis and another had a chronic obstructive pulmonary disease (COPD), an abuse of alcohol and an adiposities grade 3. Four patients needed an extracorporeal membrane oxygenation (ECMO) due to severe respiratory failure with global respiratory insufficiency that could not be treated by conservative ventilatory support. The one patient with a pre-existing lung fibrosis died shortly after lung transplantation despite use of an extracorporeal membrane oxygenation. One other patient died due to a subarachnoidal bleeding under the anticoagulatory regime during ECMO therapy. The adipose COPD-patient died due to septic shock with multiple organ failure without possibility for ECMO support. Conclusions The clinical course of severe cases of influenza A (H1N1) 2009-infection is markedly different from the disease pattern seen during epidemics of seasonal influenza. Most of the patients admitted to our intensive care unit due to influenza A (H1N1) 2009 associated ARDS were previously healthy young people.

Hospitalisation Costs of Cystic Fibrosis

To calculate per-case hospital costs for patients with cystic fibrosis under routine conditions from a healthcare provider's perspective; identify the impact of different cost categories; investigate whether cases with cystic fibrosis can be grouped into homogenous cost groups according to defined severity levels; and determine the value of specific factors as predictors of hospital cost variations. All data were collected from cases (n = 131) admitted to an inpatient cystic fibrosis unit under routine conditions during a period of 6 months in 2004. All costs were calculated for the year 2004 and divided into categories with high and low impact on variation in hospitalisation costs between patients. Staff costs for patient care, laboratory costs and drug costs were defined as categories with high impact, thus the individual resource utilisation for each case was measured. Cost categories that were classified as having a low impact were measured as overhead costs. Cases were classified according to two different severity models; within each model, patients were classified according to three severity levels. The diagnosis-related model classifies patients with pulmonary hypertension and global respiratory insufficiency as having severe disease, patients with Pseudomonas aeruginosa as having moderate disease, and patients with no colonisation of the lungs as having mild disease. The lung-function-related model differentiates patients as having mild, moderate and severe disease when patients have forced expiratory volumes in 1 second (FEV(1)) that are > or =70%, between > or =40% and <70%, and <40%, respectively. Analysis of variance tests were performed to investigate the differences of mean costs between the groups. Ordinary least squares regression analysis was used to determine predictors for cost variation. The mean total costs per case were 7326 euro. Almost one-third of the total mean costs were attributable to drug costs (28% of total costs), while shares of staff costs for patient care and laboratory costs (both 9% of total costs) were relatively small. Most of the difference in costs between severity levels was attributable to the variation in overhead costs and drug costs. For both severity models differences in mean total costs of mild and severe cases were statistically significant (p < 0.01 and p < 0.05, respectively) when compared with the mean costs of non-mild and non-severe cases. However, in moderate cases, significant differences compared with cases that were not of moderate severity were only seen for certain cost categories. In the multiple regression model the variables 'diagnosis-related severity' and 'FEV(1)' explained 31% of the variance of 'Ln (total costs per case)' between severity levels (p < or = 0.01). This study shows that to a large extent hospitalisation costs for patients with cystic fibrosis vary according to the severity of their disease; drug costs play a major role in these differences. In the light of this variation it seems plausible to create separate reimbursement rates for two or three severity groups. Diagnoses as well as FEV(1) seem suitable criteria for such a classification.

Continuous Flow Ventilatory Support (Clinical Experience)

Background. The world literature contains no reports on the clinical application of continuous flow ventilatory support by an insufflation catheter. Despite the use of different forms of ventilatory support, disconnection of patients from artificial ventilation is unsuccessful in 10—30% of cases despite the fact that the clinical and biochemical criteria are met. Objective: to discuss the efficiency of the new ventilation regime — continuous flow ventilatory support in the clinical setting. Methods: continuous flow ventilatory support with an original licensed multi-jet insufflation catheter or a terminal one-orifice catheter nasally inserted into the trachea was applied to 70 patients. It was used in a subgroup of 64 patients with chronic obstructive lung disease (COLD) due to the occurrence of global respiratory insufficiency caused by infectious complications and in a group of 6 patients as a ventilatory regime for their disconnection from long-term artificial ventilation, whose disconnection other ventilatory regimens being used were unsuccessful. Results. None patient with COLD should be intubated, and just 30 minutes after the initiation of ventilatory support with a multi-jet catheter, there were decreases in the mean respiration rate from 33±2.8 to 27±2.5 cycles/min and in paCo 2 from 11.9±1.7 to 10.8±1.6 kPa and an increase in paCo 2 from 5.7±1.1 to 6.8±1.3 kPa at FiO 2 =0.3. Within 24 hours after the initiation of ventilatory support, blood gas levels changed in response to the values typical of partial respiratory insufficiency. The spontaneous ventilation rate decreased to 20±2.2, paCO 2 reduced to 6.4±1.2 kPa and pO 2 continuously increased up to the value 8.9±1.4 kPa (FiO 2 =0.3) at hour 24 of ventilatory support. Ventilatory support lasted an average of 5 days. Statistical comparison of the study parameters showed a significant improvement (p&lt;0.05) just 6 hours after ventilatory support and a marked improvement of the parameters (p&lt;0.01) following 72 hours. In the other group of patients, continuous flow ventilatory support was used due to failing disconnection of the patients from long-term artificial ventilation. After extubation and 30 minutes after the initiation of continuous flow ventilatory support, the ventilation rated decreased to 27±2.5 cycles/min, there was a continuous reduction in paCO 2 to 3.9±0.9 kPa as a manifestation of hyperventilation that had been likely to be induced by a continuous decrease of paCO 2 to 8.8±1.4 kPa. Only 60 minutes after the initiation of ventilatory support, with the equal ventilation rate, the values of blood gases (paO 2 =9.9±1.5 kPa, paCO 2 =5.2±1.1 kPa) increased, as did VT (0.38±0.30), which permitted one to proceed with continuous flow ventilatory support that could be interrupted following 48 hours. Conclusion. The findings lead to the conclusion that continuous flow ventilatory support is an effective ventilation regimen that is applicable to patients with chronic obstructive lung disease in global respiratory insufficiency and makes it possible to overcome the period of, for example, infectious complications without intubation and artificial ventilation. It may also be used as a non-invasive ventilation regime in the disconnection of patients from long-term artificial ventilation. Its application in acute respiratory failure (acute respiratory failure, acute respiratory distress syndrome) requires further prospective studies.

Corrective osteotomy of the humerus using perivascular axillary anesthesia according to Weber in a patient suffering from McCune-Albright syndrome

We report on a 20-year-old patient with McCune-Albright syndrome suffering from global respiratory insufficiency who required continuous mask ventilation and where intubation had to be avoided. Perivascular axillary anesthesia according to Weber was performed for a double corrective osteotomy of the humerus. During plexus anesthesia the patient was positioned on the non-anesthesized side in a 15 degrees Trendelenburg position. An extension of analgesia was observed up to the complete upper arm region. Using the modified positioning an extension of brachial plexus anesthesia is possible.

Global respiratory insufficiency due to proximal diabetic neuropathy

Sirs: Proximal diabetic neuropathy [6], also known as diabetic amyotrophy [4] or Bruns-Garland Syndrome [1] is characterized by proximal muscle weakness, predominantly located on the lower extremities as well as pain. Proximal diabetic neuropathy, however, can also affect the upper limbs and can be the cause of neuromuscular respiratory weakness [2, 5, 7]. The primary target of therapy should be the treatment of the underlying diabetic disease. Pathological findings in nerve biopsy specimens have shown that proximal diabetic neuropathy may have an immunemediated inflammatory basis resulting in an epineural microvessel vasculitis [4]. Therefore it has been suggested that immunomodulation with intravenous immunoglobulin (IVIG) might have a positive effect on the patients’ condition [3, 6]. We present the report of a patient suffering from respiratory failure due to proximal diabetic neuropathy: A 79 year old woman suffering from non insulin dependent diabetes mellitus (NIDDM), hypertension and lumbar vertebral canal stenosis was admitted to our hospital because of severe dyspnea and reduced walking distance, which had worsened over the past two weeks. The patient was a nonsmoker, had an adult healthy son, and had worked in a bookstore for more than 30 years. As the patient was an orphan, there was no further family history. Apart from the lumbar canal stenosis and the NIDDM diagnosed seven years earlier, the patient did not report other serious illnesses. On admission, the patient presented with orthopnea, symmetric weakness and wasting of shoulder girdle muscles, and a sensory polyneuropathy in both feet. There was a severe bilateral palsy of arm abduction (MRC grade 2/5) and a moderate palsy of shoulder elevation (MRC grade 3/5). Tendon reflexes of the legs were weak. Clinical and functional examination did not yield a cardiopulmonary explanation for orthopnea. Serum creatine kinase level was normal on admission (117 U/l, range 0–145 U/l). Chest radiography, however, showed an elevated diaphragm on the left side (Fig. 1). Arterial blood gas analysis proved a global respiratory insufficiency (pO2 54.2 mmHG, pCO2 70.9 mmHG, sO2 88.3 %). Pulmonary function test revealed a markedly reduced vital capacity (0.63l, 33 % of the normal value). With a HbA1C of 8.2 %, a positive urinary glucose, and a fasting serum glucose level of 226 mg/dl, her diabetes was not well controlled. The patient did not keep to a diabetes diet. EMG of the right deltoid muscle showed acute and chronic signs of denervation, and pulmonary fluoroscopy showed bilaterally reduced diaphragmatic motility. Neurography of the main extremity nerves showed a generalized axonal sensori-motor neuropathy (see Table 1). Biopsy of the left deltoid muscle yielded groups of atrophic, elongated fibers, a high grade variability in fiber caliber, targetLETTER TO THE EDITORS

Foudroyante Verläufe bei AIDS-bedingter CMV-Retinitis

Of 57 patients at Karlsruhe Municipal Eye Hospital with manifest acquired immune deficiency syndrome, ocular involvement was found in 34%. In the prefinal stage (on average 6 months ante finem), 25% of these patients developed typical, rapidly progressing cytomegalovirus retinitis with intraretinal hemorrhages and necrosis, which responded well to combined intravenous and intravitreal DHPG therapy. Almost all the patients died of global respiratory insufficiency caused by pneumocystis carinii or atypical mycobacteria.

The role of mesna in the treatment of status asthmaticus in children.

Status asthmaticus in children is a life-threatening condition that calls for a comprehensive set of therapeutic measures, viz. evacuation of tracheobronchial secretions, parenteral drug administration and improvement of alveolar gas exchange. In the authors' experience, that therapeutic procedure can be much improved by bronchial lavage with mesna. This is illustrated by the good results obtained in fourteen patients with status asthmaticus and in another forty-four cases of global respiratory insufficiency. Evaluation was by blood gas values and X-ray pictures. The method used for bronchial lavage is described.

Development of radiological and functional changes in silicosis during the long term follow-up

The report follows up the development of radiologic changes and functional disorders in 200 patients with silicosis who were repeatedly examined over a period of time. The patients were divided according to the X-ray findings, when first detected, into groups of simple silicosis, complicated silicosis, and simple silicosis passing into the complicated stage during the study. Each group was divided into 3 time periods of follow-up: 3 to 5 yrs, 6 to 10 yrs, and 11 to 17 yrs between the first and the last examinations. The results disclosed the following conclusions: 1. Changes of the radiologic picture evaluated according to the International Classification of Pneumoconiosis ILO 1971 proceeded in the course of time into more serious forms with respect to size and profusion of nodules. With complicated silicosis, possibly with the transient form, the auxiliary symbols increased with time, particularly “ax”, “pl”, “em”, “es”, “tbu”, and “tba”. The classification proved satisfactory for expressing shifts of X-ray changes; it did not suffice alone, however, for answering the question when a dust finding reaches the stage at which it is to be declared an occupational disease. This would require more criteria, chiefly tomography of the lungs and assessment of the dynamics of development in radiologic changes. 2. The trend of the ventilatory-function decrease differed only slightly in a mutual comparison of the groups. This means that in the intermediate and long time periods the decrease in ventilatory function is similar because the patients with severe functional disorders do not live long enough for long-term studies. This was proved by an analysis of the mortality rate in the groups. 3. We ascertained more significant differences in the ventilatory function within the individual groups in patients with signs of chronic bronchitis when compared with patients without bronchitis. The important influence especially of the obstructive form associated with complicated silicosis was evident here. 4. Part of the group was also studied from the aspect of blood gases and acid-base status over periods of 3 to 5 and 6 to 10 yrs. With both forms of silicosis the values of the first and last examinations differed only insignificantly. Pronounced changes of the respiratory function cannot be expected when the organism is able to compensate the disorder of ventilation. The finding of global respiratory insufficiency with rapid progress of silicosis always indicated a limited life expectancy.

ACUTE RESPIRATORY FAILURE

The acute respiratory insufficiency (ARI) is a complicating syndrome in many severe, primarily not the lungs affecting internal and chirurgical diseases. As result of activation of classic cascade-systems, gas diffusion disturbance, right-left shunt and lung restriction occur. The effecting global respiratory insufficiency is only to prevent by polypragmatic therapy but often lung fibrosis terminates medical efforts.