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Outcomes of Long-Term Noninvasive Ventilation Use in Children with Neuromuscular Disease: Systematic Review and Meta-Analysis

AlBalawi MM, Castro-Codesal M, Featherstone R, Sebastianski M, Vandermeer B, Alkhaledi B, et al.

Ann Am Thorac Soc, 2022 01;19(1):109-119.
PMID: 34181865 DOI: 10.1513/AnnalsATS.202009-1089OC

Objectives: To determine whether children with neuromuscular disorders using long-term noninvasive ventilation (NIV), continuous or bilevel positive airway pressure, have improved health outcomes compared with alternative treatment strategies. Data Sources: This systematic review is an extension of a scoping review. The search strategy used Medical Subject Headings and free-text terms for "child" and "noninvasive ventilation." Studies of humans from 1990 onward were searched in MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed. The results were reviewed for articles reporting on neuromuscular disorders and health outcomes including mortality, hospitalization, quality of life, lung function, sleep study parameters, and healthcare costs. Data Extraction: Extracted data included study design, study duration, sample size, age, type of NIV, follow-up period, primary disease, and primary and secondary outcome measures. Studies were grouped by primary disease into three groups: spinal muscular atrophy, Duchenne muscular dystrophy, and other/multiple neuromuscular diseases. Data Synthesis: A total of 50 articles including 1,412 children across 36 different neuromuscular disorders are included in the review. Mortality is lower for children using long-term NIV compared with supportive care across all neuromuscular disease types. Overall, mortality does not differ when comparing the use of NIV with invasive mechanical ventilation, though heterogeneity suggests that mortality with NIV is higher for spinal muscular atrophy type 1 and lower for other/multiple neuromuscular diseases. The impact of long-term NIV on hospitalization rate differed by neuromuscular disease type with lower rates compared with supportive care but higher rates compared with supportive care use for spinal muscular atrophy type 1, and lower rates compared with before NIV for other/multiple neuromuscular diseases. Overall, lung function was unaltered and sleep study parameters were improved from baseline by long-term NIV use. There are few data to assess the impact of long-term NIV use on quality of life and healthcare costs. Conclusions: Long-term NIV for children provides benefit for mortality, hospitalizations, and sleep study parameters for some sub-groups of children with neuromuscular disorders. High risk of bias and low study quality preclude strong conclusions.

Matched MeSH terms: Respiratory Insufficiency*
High-dose pulse methylprednisolone vs. dexamethasone standard therapy for severe and critical COVID-19 pneumonia: Efficacy assessment in a retrospective single-centre experience from Malaysia

Soo CI, Poon KV, Ayub A, You HW, Tan CX, Loh KJJ, et al.

Med J Malaysia, 2024 Jan;79(1):15-20.
PMID: 38287752

INTRODUCTION: The use of dexamethasone (DXM) has been associated with decreased mortality in the patients with hypoxemia during the coronavirus disease-2019 (COVID-19) pandemic, while the outcomes with methylprednisolone (MTP) have been mixed. This real-life study aimed to evaluate the outcomes of patients with severe respiratory failure due to COVID-19 who were treated with high doses of MTP.
MATERIALS AND METHODS: This retrospective cohort study enrolled hospitalised patients between May 2021 and August 2021, aged 18 years and above, with severe respiratory failure defined by a ratio of oxygen saturation to fraction of inspired oxygen (SF ratio) of less than 235. The treatment protocol involved administering high-dose MTP for 3 days, followed by DXM, and the outcomes were compared with those of patients who received DXM alone (total treatment duration of 10 days for both groups).
RESULTS: A total of 99 patients were enrolled, with 79 (79.8%) receiving pulse MTP therapy and 20 (20.2%) being treated with DXM only. The SF ratio significantly improved from a mean of 144.49 (±45.16) at baseline to 208 (±85.19) at 72 hours (p < 0.05), with a mean difference of 63.51 (p < 0.001) in patients who received ≤750 mg of MTP. Additionally, in patients who received >750 mg of MTP, the SF ratio improved from a baseline mean of 130.39 (±34.53) to 208.44 (±86.61) at 72 hours (p < 0.05), with a mean difference of 78.05 (p = 0.001). In contrast, patients who received DXM only demonstrated an SF ratio of 132.85 (±44.1) at baseline, which changed minimally to 133.35 (±44.4) at 72 hours (p = 0.33), with a mean difference of 0.50 (p = 0.972). The incidence of nosocomial infection was higher in the MTP group compared with the DXM group (40.5% vs. 35%, p = 0.653), with a relative risk of 1.16 (95% CI: 0.60-2.23).
CONCLUSION: MTP did not demonstrate a significant reduction in intubation or intensive care unit admissions. Although a high dose of MTP improved gas exchange in patients with severe and critical COVID-19, it did not provide an overall mortality benefit compared to standard treatment.

Matched MeSH terms: Respiratory Insufficiency*
Fulltext The immediate outcome of ventilation for pulmonary diseases

Yaacob I, Mustafa M

Singapore Med J, 1994 Oct;35(5):512-4.
PMID: 7701374

Fifty-eight patients were ventilated for acute respiratory failure complicating respiratory diseases between 1985 to 1990. There were 19 cases of chronic obstructive airway disease (COAD), 17 cases of asthma, 16 cases of pneumonia and 6 cases with other diagnoses. Overall, 40% of patients survived and were discharged from the ward. Patients with pneumonia had the lowest survival rate (25%) whilst the survival rates for asthma and COAD were 47% and 42% respectively. Increasing age had an inverse relationship to survival rate (r = 0.96, p < 0.05) but the duration of ventilation did not correlate with survival. Patients who were electively ventilated before respiratory arrest had a better chance of survival (57%) compared with only 18% survival rate in patients who were ventilated as an emergency (x2 = 4.47, p < 0.05). Patients who developed other organ failure had higher mortality (71%) than those who did not (22%; x2 = 2.14, p < 0.05). We conclude that patients younger than 50 years of age, who were electively ventilated and without other organ failure had a better immediate survival after assisted ventilation.

Matched MeSH terms: Respiratory Insufficiency/etiology; Respiratory Insufficiency/mortality; Respiratory Insufficiency/therapy*
Fulltext Domiciliary non-invasive ventilatory devices--a general hospital experience

Norhaya MR, Wazi RA, Azhar AA

Med J Malaysia, 2009 Mar;64(1):77-9.
PMID: 19852329

Treatment for chronic respiratory failure has advanced since the introduction of domiciliary non-invasive ventilatory devices. This has given a new light of hope for patients with chronic respiratory failure secondary to various causes. We report a series of patients with respiratory failure of different origins and types of management that they received. Four patients received bilevel positive airway pressure (BiPAP) and one patient received continuous positive airway pressure (CPAP).

Matched MeSH terms: Respiratory Insufficiency/therapy*
Fulltext A tile factory worker presenting with breathlessness and dry cough

Khajotia R, Gupta ED

Aust Fam Physician, 2009 Oct;38(10):803-4.
PMID: 19893821

Jenny, a nonsmoker, 54 years of age, presents with 3 years of dry cough, progressive breathlessness and reducing exercise tolerance. Two years ago she was diagnosed with asthma and treated with inhaled bronchodilators (which have been marginally effective). Jenny has worked in a tile factory for 22 years; 15 years in the grinding department, transferring to the chipping department 7 years ago. On examination she is tachypnoeic with a prolonged expiratory phase. There are bilateral rhonchi and a few fine crepitations at the left infrascapular region. Jenny's full blood count and electrocardiogram are normal. Arterial blood gas show mild hypoxia with respiratory alkalosis. Spirometry demonstrates mixed moderate obstructive and restrictive impairment. The diffusion capacity for carbon monoxide is reduced. Mantoux is negative and erythrocyte sedimentation rate is 10 mm/hour. A chest X-ray is taken.

Matched MeSH terms: Respiratory Insufficiency/etiology*
Massive subcapsular air collection in the liver: a rare life-threatening complication following ERCP rendezvous procedure

Koh PS, Ho SH, Yoong BK, Abdullah BJ, Goh KL

J Dig Dis, 2013 Jun;14(6):337-9.
PMID: 23279747 DOI: 10.1111/1751-2980.12030
Matched MeSH terms: Respiratory Insufficiency/etiology
Extracorporeal carbon dioxide removal (ECCO2R) in respiratory deficiency and current investigations on its improvement: a review

Manap HH, Abdul Wahab AK

J Artif Organs, 2017 Mar;20(1):8-17.
PMID: 27193131 DOI: 10.1007/s10047-016-0905-x

The implementation of extracorporeal carbon dioxide removal (ECCO2R) as one of the extracorporeal life support system is getting more attention today. Thus, the objectives of this paper are to study the clinical practice of commercial ECCO2R system, current trend of its development and also the perspective on future improvement that can be done to the existing ECCO2R system. The strength of this article lies in its review scope, which focuses on the commercial ECCO2R therapy in the market based on membrane lung and current investigation to improve the efficiency of the ECCO2R system, in terms of surface modification by carbonic anhydrase (CA) immobilization technique and respiratory electrodialysis (R-ED). Our methodology approach involves the identification of relevant published literature from PubMed and Web of Sciences search engine using the terms Extracorporeal Carbon Dioxide Removal (ECCO2R), Extracorporeal life support, by combining terms between ECCO2R and CA and also ECCO2R with R-ED. This identification only limits articles in English language. Overall, several commercial ECCO2R systems are known and proven safe to be used in patients in terms of efficiency, safety and risk of complication. In addition, CA-modified hollow fiber for membrane lung and R-ED are proven to have good potential to be applied in conventional ECCO2R design. The detailed technique and current progress on CA immobilization and R-ED development were also reviewed in this article.

Matched MeSH terms: Respiratory Insufficiency/therapy*
High intensity non-invasive positive pressure ventilation for refractory decompensated acute hypercapnic respiratory failure in advanced chronic obstructive pulmonary disease

Lim KY, Rajah R, Ng BH, Soo CI

Med J Malaysia, 2020 07;75(4):430-432.
PMID: 32724009

Chronic obstructive pulmonary disease (COPD) is a debilitating progressive lung disease characterised by irreversible airflow obstruction. In addition to an increase in morbidity and mortality, exacerbation also results in frequent hospital visits, which place a burden on healthcare systems. Non-invasive positive pressure ventilation (NPPV) with conventional inspiratory pressures is the standard ventilatory support for patients in exacerbation. At present, the use of higher inspiratory pressures through high intensity noninvasive positive pressure ventilation (Hi-NPPV) during an exacerbation remains unknown. We describe a novel application of Hi-NPPV in a patient with acute exacerbation who was refractory to conventional NPPV.

Matched MeSH terms: Respiratory Insufficiency/etiology*
Management of respiratory insufficiency

MANAVALAN AS

Med J Malaya, 1961 Dec;16:125-35.
PMID: 14469124
Matched MeSH terms: Respiratory Insufficiency*
Fulltext Prevalence, clinical predictors and diagnosis of gastro-oesophageal reflux in children with persistent respiratory symptoms

Siti Mazliah K, Norzila MZ, Deng CT, Zulfiqar A, Azizi BHO

Med J Malaysia, 2000 Jun;55(2):180-7.
PMID: 19839146

Objectives: This was a cross sectional study conducted in the Paediatric Institute among infants and children with chronic respiratory symptoms with the following objectives: i) to determine the prevalence of gastro-oesophageal reflux in children with persistent respiratory symptoms, ii) to identify the clinical predictors of GOR (Gastro-oesophageal reflux) in children with persistent respiratory symptoms and iii) assess the validity of abdominal ultrasound, barium oesophagogram and chest radiograph in diagnosing GOR in these patients.
Materials and Methods: Forty-four patients were recruited over a period of six months. All the presenting symptoms were identified. The patients were subjected to chest radiograph, abdominal ultrasound, barium oesophagogram and 24-hour pH oesophageal monitoring.
The predictive validity of clinical symptoms, chest radiograph, abdominal ultrasound and barium oesophagogram were assessed. Twenty-four hours oesophageal pH was the gold standard to diagnose GOR.
Results: The mean age of patients was 9.1 months (1-58 months). Thirty-one patients (70.5%) were confirmed to have GOR by pH study. Respiratory symptoms alone were not useful to predict GOR. Cough had the highest sensitivity of 51.6%. stridor, wheeze and choking each had a specificity of 76%. Wheeze, vomiting, choking and stridor were identified to have high specificity (90-100%) in diagnosing GOR when any two symptoms were taken in combination.
Collapse/consolidation was the commonest radiological abnormality but had low sensitivity (35.5%) and specificity (53.8%). However hyperinflation on chest radiograph had a specificity of 92.3% with positive predictive value at 80% in diagnosing GOR. Barium oesophagogram has low sensitivity (37.9%) and moderate specificity (75%) in diagnosing GOR in children with respiratory symptoms.
Abdominal ultrasound was a valid mode of diagnosing GOR when there were three or more reflux episodes demonstrated during the screening period with a specificity of 90.9%. However the sensitivity was low ie 20-25%. The specificity increased to 90-100% when two positive tests were taken in combination (abdominal ultrasound and barium oesophagogram). However the sensitivity remained low (10-20%). Chest radiograph did not improve the predictive value when considered with the above tests. Combination of clinical symptoms were useful as clinical predictors of GOR. In the absence of a pH oesophageal monitoring, a combination of barium oesophagogram and ultrasound may be helpful in diagnosing GOR.

Matched MeSH terms: Respiratory Insufficiency/ethnology; Respiratory Insufficiency/physiopathology*; Respiratory Insufficiency/ultrasonography
Fulltext Emergency thyroid surgery: a surgical challenge

Rohana A, Hisham AN

Asian J Surg, 2009 Apr;32(2):81-4.
PMID: 19423453 DOI: 10.1016/S1015-9584(09)60015-4

BACKGROUND: Acute life threatening presentations of thyroid disease are uncommon. The aim of this study was to review our experience of emergency thyroid surgery with regard to the incidence, management and outcome to this approach.
METHODS: Forty patients who underwent emergency thyroid surgery from December 1998 to December 2007 were prospectively accrued in this study. There were 30 female and 10 male patients. The mean age was 63.8 years (range, 33-83 years). All patients had total thyroidectomy, except one patient who only had a right hemithyroidectomy for a dominant right multinodular goiter. Eight of the patients had been intubated before referring to our centre.
RESULTS: The mean weight of specimen was 219 g (range, 32-800 g). A histopathology report confirmed 16 patients had multinodular goiters, eight patients had papillary thyroid carcinomas, seven patients had anaplastic cancers, four patients had follicular thyroid carcinomas, two patients had thyroid lymphoma, one patient had medullary carcinoma, one patient had sarcoma and one patient had thyroid abscess. A total of 28 patients were discharged well postoperatively.
CONCLUSION: Acute life threatening presentations of thyroid disease are uncommon. However, early recognition of these problems is crucial and may life saving. Emergency thyroidectomy is important to release the critically obstructed airways. Nonetheless it is associated with higher risk of surgery and complication rate then any elective thyroid surgery.

Matched MeSH terms: Respiratory Insufficiency/etiology; Respiratory Insufficiency/surgery
Fulltext Fracture synthetic tracheostomy tube: an ENT emergency

Shashinder S, Tang IP, Kuljit S, Muthu K, Gopala KG, Jalaludin MA

Med J Malaysia, 2008 Aug;63(3):254-5.
PMID: 19248703 MyJurnal

A synthetic tracheostomy tube [non-metallic type] fracturing within three days of insertion is very rare but it could lead to serious complications such as acute asphyxia or sudden death. We report three such cases at our centre. Recommendations are made to closely observe patients on tracheostomy tube in the first seven days post-insertion of the tube.

Matched MeSH terms: Respiratory Insufficiency/etiology; Respiratory Insufficiency/therapy
Diaphragmatic hernia: an unusual cause of postpartum collapse

Sharifah H, Naidu A, Vimal K

BJOG, 2003 Jul;110(7):701-3.
PMID: 12842063
Matched MeSH terms: Respiratory Insufficiency/etiology*; Respiratory Insufficiency/therapy
Fulltext Respiratory failure requiring ventilation in acute bronchiolitis

Chan PWK, Goh AYT, Lum LCS

Med J Malaysia, 1999 Dec;54(4):487-91.
PMID: 11072467

Severe bronchiolitis requiring mechanical ventilation is uncommon and is associated with the risk of barotrauma. We report our experience with 25 (42%) of 60 infants admitted to the Paediatric Intensive Care Unit (PICU) with severe bronchiolitis who required mechanical ventilation. Eighteen patients (72%) had severe hypoxaemia (PaO2/FiO2 < 250). The mean airway pressure required ranged from 5.8 to 15.6 cmH2O with median ventilation duration of 4.0 days (range 2.0-14.0 days). Oxygenation improved significantly within 12 hours of intubation. There was only one death. Mechanical ventilation is required in a subset of patients for severe bronchiolitis and is effective and generally well tolerated.

Matched MeSH terms: Respiratory Insufficiency/etiology*; Respiratory Insufficiency/therapy*
Acquired tracheo-oesophageal fistula following tracheal intubation in a burned patient

Tan KK, Lee JK, Tan I, Sarvesvaran R

Burns, 1993 Aug;19(4):360-1.
PMID: 8357487

A 27-year old male sustained a 60 per cent TBSA burn with inhalation injury following a road traffic accident. He developed respiratory distress on day 3 postburn, and was intubated and ventilated. He was noted to have greenish aspirate from his trachea on day 17 of ventilation. He succumbed from sepsis and died on day 21 post injury. At post-mortem, a large tracheo-oesophageal fistula (TOF) was found at the level of the cuff of the nasotracheal tube.

Matched MeSH terms: Respiratory Insufficiency/etiology; Respiratory Insufficiency/therapy
Fulltext Nine fatal cases of puffer fish poisoning in Sabah, Malaysia

Kan SKP, Chan MKC, David P

Med J Malaysia, 1987 Sep;42(3):199-200.
PMID: 3506645

The puffer fish belongs to the family Tetraodontidae and has one identifying characteristic which is the ability to inflate itself by gulping in large quantities of air or water. They are commonly found in estuarine and inshore water. The viscera (ovaries, testes and liver) of puffer fish contain varying amounts of extremely potent toxins [tetraodontoxin). The most violent form of fish poisoning is produced by ingestion of tetraodontoid or puffer-like fishes and this has been reported. The putftoad, Tetradon maculatum, has caused fatal poisoning? Despite the high toxicity of this group of fish, the meat is commonly consumed by some fisherman in Sabah without much morbidity. In Japan, puffer fish, called "fuqu" commands the highest prices in food fish and are prepared and sold in special restaurants where specially trained cooks are employed to prepare the "fuqu". Nevertheless it is still the primary cause of food poisoning in Japan especially among the lower socio-economic classes who fail to take the necessary precautions. In Sabah, four cases of puffer fish poisoning resulting in one death have been documented." We report herewith another incidence of puffer fish poisoning in which 18 persons from seven families were affected with nine deaths from two families.

Matched MeSH terms: Respiratory Insufficiency/etiology; Respiratory Insufficiency/mortality*
Thirteen years of invasive and noninvasive home ventilation for children in a developing country: A retrospective study

Nathan AM, Loo HY, de Bruyne JA, Eg KP, Kee SY, Thavagnanam S, et al.

Pediatr Pulmonol, 2017 04;52(4):500-507.
PMID: 27712049 DOI: 10.1002/ppul.23569

INTRODUCTION: Home ventilation (HV) for children is growing rapidly worldwide. The aim was to describe (1) the sociodemographic characteristics of children on HV and (2) the indications for, means and outcome of initiating HV in children from a developing country.
METHODOLOGY: This retrospective study included patients sent home on noninvasive or invasive ventilation, over 13 years, by the pediatric respiratory unit in a single center. Children who declined treatment were excluded.
RESULTS: Seventy children were initiated on HV: 85.7% on noninvasive ventilation, 14.3% on invasive ventilation. There was about a threefold increase from 2001-2008 (n = 18) to 2009-2014 (n = 52). Median (range) age of initiating HV was 11 (1-169) months and 73% of children were <2 years old. Common indications for HV were respiratory (57.2%), chest/spine anomalies (11.4%), and neuromuscular (10.0%). Fifty-two percent came off their devices with a median (interquartile range) usage duration of 12 (4.8, 21.6) months. Ten children (14.3%) died with one avoidable death. Children with neuromuscular disease were less likely to come off their ventilator (0.0%) compared to children with respiratory disease (62.1%). Forty-one percent of parents bought their equipment, whereas 58.6% borrowed their equipment from the medical social work department and other sources.
CONCLUSION: HV in a resource-limited country is possible. Children with respiratory disease made up a significant proportion of those requiring HV and were more likely to be weaned off. The mortality rate was low. The social work department played an important role in facilitating early discharge. Pediatr Pulmonol. 2017;52:500-507. © 2016 Wiley Periodicals, Inc.

Matched MeSH terms: Respiratory Insufficiency/epidemiology*; Respiratory Insufficiency/therapy
Fulltext Stochastic Modelling of Respiratory System Elastance for Mechanically Ventilated Respiratory Failure Patients

Lee JWW, Chiew YS, Wang X, Tan CP, Mat Nor MB, Damanhuri NS, et al.

Ann Biomed Eng, 2021 Dec;49(12):3280-3295.
PMID: 34435276 DOI: 10.1007/s10439-021-02854-4

While lung protective mechanical ventilation (MV) guidelines have been developed to avoid ventilator-induced lung injury (VILI), a one-size-fits-all approach cannot benefit every individual patient. Hence, there is significant need for the ability to provide patient-specific MV settings to ensure safety, and optimise patient care. Model-based approaches enable patient-specific care by identifying time-varying patient-specific parameters, such as respiratory elastance, Ers, to capture inter- and intra-patient variability. However, patient-specific parameters evolve with time, as a function of disease progression and patient condition, making predicting their future values crucial for recommending patient-specific MV settings. This study employs stochastic modelling to predict future Ers values using retrospective patient data to develop and validate a model indicating future intra-patient variability of Ers. Cross validation results show stochastic modelling can predict future elastance ranges with 92.59 and 68.56% of predicted values within the 5-95% and the 25-75% range, respectively. This range can be used to ensure patients receive adequate minute ventilation should elastance rise and minimise the risk of VILI should elastance fall. The results show the potential for model-based protocols using stochastic model prediction of future Ers values to provide safe and patient-specific MV. These results warrant further investigation to validate its clinical utility.

Matched MeSH terms: Respiratory Insufficiency/physiopathology*; Respiratory Insufficiency/therapy*
Non-steady State Modeling of the Ventilatory Depressant Effect of Remifentanil in Awake Patients Experiencing Moderate-to-severe Obstructive Sleep Apnea

Doufas AG, Shafer SL, Rashid NHA, Kushida CA, Capasso R

Anesthesiology, 2019 02;130(2):213-226.
PMID: 30247202 DOI: 10.1097/ALN.0000000000002430

BACKGROUND: Evidence suggests that obstructive sleep apnea promotes postoperative pulmonary complications by enhancing vulnerability to opioid-induced ventilatory depression. We hypothesized that patients with moderate-to-severe obstructive sleep apnea are more sensitive to remifentanil-induced ventilatory depression than controls.
METHODS: After institutional approval and written informed consent, patients received a brief remifentanil infusion during continuous monitoring of ventilation. We compared minute ventilation in 30 patients with moderate-to-severe obstructive sleep apnea diagnosed by polysomnography and 20 controls with no to mild obstructive sleep apnea per polysomnography. Effect site concentrations were estimated by a published pharmacologic model. We modeled minute ventilation as a function of effect site concentration and the estimated carbon dioxide. Obstructive sleep apnea status, body mass index, sex, age, use of continuous positive airway pressure, apnea/hypopnea events per hour of sleep, and minimum nocturnal oxygen saturation measured by pulse oximetry in polysomnography were tested as covariates for remifentanil effect site concentration at half-maximal depression of minute ventilation (Ce50) and included in the model if a threshold of 6.63 (P < 0.01) in the reduction of objective function was reached and improved model fit.
RESULTS: Our model described the observed minute ventilation with reasonable accuracy (22% median absolute error). We estimated a remifentanil Ce50 of 2.20 ng · ml (95% CI, 2.09 to 2.33). The estimated value for Ce50 was 2.1 ng · ml (95% CI, 1.9 to 2.3) in patients without obstructive sleep apnea and 2.3 ng · ml (95% CI, 2.2 to 2.5) in patients with obstructive sleep apnea, a statistically nonsignificant difference (P = 0.081). None of the tested covariates demonstrated a significant effect on Ce50. Likelihood profiling with the model including obstructive sleep apnea suggested that the effect of obstructive sleep apnea on remifentanil Ce50 was less than 5%.
CONCLUSIONS: Obstructive sleep apnea status, apnea/hypopnea events per hour of sleep, or minimum nocturnal oxygen saturation measured by pulse oximetry did not influence the sensitivity to remifentanil-induced ventilatory depression in awake patients receiving a remifentanil infusion of 0.2 μg · kg of ideal body weight per minute.

Matched MeSH terms: Respiratory Insufficiency/chemically induced*; Respiratory Insufficiency/physiopathology*
Juvenile acid maltase deficiency

Loi, H.D.K., Wong, K.T., Choo, K.E.

Malaysian Journal of Paediatrics and Child Health, 2005;14(1):58-63.
MyJurnal

An eight-year-old Chinese girl presented with a slowly progressive generalized muscle weakness and wasting, complicated by respiratory failure. She had many hospital admissions requiring ventilator support. Eventually tracheostomy tube was inserted. Initial investigations failed to elicit a diagnosis but a muscle biopsy and histological study confirmed the diagnosis of juvenile acid maltase deficiency.

Matched MeSH terms: Respiratory Insufficiency

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