A 15 years old male with a primary diagnosis of Wiskott Aldrich syndrome presented a laryngeal B cell lymphoma associated with Epstein-Barr virus. A chemotherapy and a radiotherapy were started in association with an endoscopic debulking of the tumor. The child died of respiratory failure secondary to a lung infection. ...

Chemotherapy with bleomycin may cause a syndrome of pulmonary sensitivity to supranormal inspired oxygen concentrations that persists for an unknown period of time after exposure. We present a mentally retarded adolescent patient in whom supranormal inspired oxygen was temporarily necessary to manage her difficult airway. Subsequently her pulmonary function deteriorated ...

All-trans retinoic acid (ATRA) is a potent differentiation agent that is effective therapy in acute promyelocytic leukaemia. Although ATRA is generally well tolerated, some patients develop retinoic acid syndrome. This syndrome is manifested by unexplained fever, weight gain, respiratory distress, interstitial pulmonary infiltrates, pleural and pericardial effusion, episodic hypotension, and ...

Severe acute respiratory syndrome (SARS) is a recently recognized and highly contagious pneumonic illness, caused by a novel coronavirus. While developments in diagnostic, clinical and other aspects of SARS research are well underway, there is still great difficulty for frontline clinicians as validated rapid diagnostic tests or effective treatment regimens ...

Severe acute respiratory syndrome (SARS) is a severe pulmonary infection that has been identified in multiple outbreaks around the world after emerging from mainland China in early 2003. The syndrome is caused by SARS-associated coronavirus, a novel human infection. SARS-associated coronavirus is spread by multiple mechanisms, including direct contact and ...

Respiratory viruses cause a number of clinical 'syndromes' in the intensive care unit with different viruses being able to produce similar clinical pictures. Our main presenting problems are upper airway (e.g. croup and tracheitis), lower airway with intrapulmonary shunt (e.g. bronchitis and pneumonia), lower airway with dynamic hyperinflation (e.g. bronchiolitis ...

Acute respiratory distress syndrome (ARDS) is a heterogeneous process that results in diffuse alveolar damage. It is associated with a variety of causative factors that can be grouped into two general categories, those associated with direct lung injury through the airways and those associated with indirect lung injury through the ...

Between November 2002 and March 2003, 17 cases of malaria (1 fatal) were observed in illegal Chinese immigrants who traveled to Italy through Africa. A further cluster of 12 was reported in August, 2002. Several immigrants traveled by air, making the risk of introducing sudden acute respiratory syndrome a possibility ...

OBJECTIVE: To investigate the variable clinical picture of Möbius syndrome (MIM no. 157900) and to further understand the pathogenesis of the disorder. METHODS: A standardized questionnaire was submitted to 37 Dutch patients with Möbius syndrome. All underwent standardized neurologic examination with special attention to cranial nerve functions, motor skills, and ...

Propofol infusion syndrome (PRIS) is a rare and often fatal syndrome described in critically ill children undergoing long-term propofol infusion at high doses. Recently several cases have been reported in adults, too. The main features of the syndrome consist of cardiac failure, rhabdomyolysis, severe metabolic acidosis and renal failure. To ...

Acute respiratory distress syndrome/acute lung injury is characterised by profound hypoxaemia due to a permeability pulmonary oedema. In this setting, recruitment manoeuvres (RMs) can be a useful tool as adjuncts to lung protective ventilatory strategies to prevent cyclic alveolar stress and avoid alveolar collapse. Many experimental and physiological studies have ...

BACKGROUND: While the prevalence of obstructive sleep apnoea syndrome among children with Down syndrome is reported to vary from 30 to 50%, the nocturnal respiratory patterns of adults with Down syndrome is not well known. OBJECTIVES: The aim of this study is to evaluate sleep-related breathing disorders in a sample ...

Acute respiratory distress syndrome (ARDS) can be derived from two pathogenetic pathways: a direct insult on lung cells (pulmonary ARDS (ARDSp)) or indirectly (extrapulmonary ARDS (ARDSexp)). This review reports and discusses differences in biochemical activation, histology, morphological aspects, respiratory mechanics and response to different ventilatory strategies between ARDSp and ARDSexp. ...

Turpentine is a volatile hydrocarbon used in polishes, solvents, paints and textile industry. When hydrocarbons are aspirated into the lung, they cause chemical pneumonitis, acute respiratory distress syndrome (ARDS), and rarely pneumatoceles and pneumothorax. We report a 20-year old boy with turpentine-induced chemical pneumonitis that evolved into a bronchopleural fistula. ...

We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary lymphangiectasia. Hydrops fetalis may be present in newborns with the Hennekam ...

Severe acute respiratory syndrome (SARS) poses a threat to most countries because of easy and convenient travel across the globe in a matter of hours. SARS is a recently recognized infectious disease that may lead to death and severe pulmonary sequelae. It presents with non-specific signs and symptoms and because ...

Severe acute respiratory syndrome (SARS) is an infectious atypical pneumonia that has recently been recognized in the patients in 32 countries and regions. This brief review summarizes some of the initial etiologic findings, pathological description, and its lesions of digestive system caused by SARS virus. It is an attempt to ...

The vast majority of patients with asthma have rhinitis, and rhinitis is a major independent risk factor for asthma in cross-sectional and longitudinal studies. The relationships between rhinitis and asthma can be viewed under the concept that the 2 conditions are manifestations of one syndrome, the chronic allergic respiratory syndrome, ...

Severe acute respiratory syndrome is a new disease that is highly contagious and is spreading in the local community and worldwide. This report is of a hospital medical officer with severe acute respiratory syndrome. He presented with sudden onset of fever, chills, myalgia, headache, and dizziness in early March 2003. ...

Severe acute respiratory syndrome (SARS) is a recently recognized infectious disease associated with severe morbidity and mortality. It presents with non-specific signs and symptoms and because no definitive laboratory test is readily available, it poses a great risk to healthcare workers as well as difficulty in quarantine. The global response ...

Severe acute respiratory syndrome (SARS) is an emerging viral infectious disease. One of the largest outbreaks of SARS to date began in Singapore in March 2003. We describe the clinical, laboratory, and radiologic features of the index patient and the patient's initial contacts affected with probable SARS.

As of April 14, 2003, Taiwan had had 23 probable cases of severe acute respiratory syndrome (SARS), 19 of which were imported. Taiwan isolated all 23 patients in negative-pressure rooms; extensive personal protective equipment was used for healthcare workers and visitors. For the first 6 weeks of the SARS outbreak, ...

A 43-year-old man with adult respiratory distress syndrome, secondary to pneumonia, developed bilateral pneumothorax on the fifth post-tracheostomy day, when the tracheostomy tube was being changed to a larger size tube in order to control bleeding from the tracheostomy site. This case demonstrates the potential danger of changing the tube ...

The current investigation examines the potential importance of item selection in the ongoing discussion regarding the taxonicity of depression. Following J. C. Coyne (1994), we contrast the taxonicity of "distress" with the taxonicity of a syndrome more focused on somatic symptoms (i.e., the involuntary defeat syndrome). Using 4 samples of ...

The idiopathic hypereosinophilic syndrome, common in adults, is a leukoproliferative disorder marked by a predilection to damage specific organs. This report describes a unique case of an infant with tricuspid stenosis, a hypoplastic right ventricle, severe pulmonary stenosis, patent ductus arteriosus, and a patent foramen ovale associated with hypereosinophilic syndrome. ...

OBJECTIVE: To review the epidemiology of acute lung injury (ALI) with particular emphasis on its effect on public health. DATA SOURCES: Published studies on the definitions, incidence, and outcomes of ALI. DATA SUMMARY: ALI is a syndrome of acute hypoxemic respiratory failure that is not primarily cardiac in origin. The ...

Larsen syndrome is a rare congenital connective tissue disorder which is characterized by multiple dislocations of major joints, typical facial appearance, cervical spine narrowing and instability and respiratory difficulties secondary to laryngotracheomalacia. A case of general anaesthesia for laparoscopic hernia repair in a male child aged 16 months with this ...

Since late February 2003, CDC has been supporting the World Health Organization (WHO) in the investigation of a multicountry outbreak of atypical pneumonia of unknown etiology. The illness is being referred to as severe acute respiratory syndrome (SARS). This report describes the scope of the outbreak, preliminary case definition, and ...

A 56-year-old man with late amyotrophic sequelae from poliomyelitis experienced progressive dyspnoea requiring intubation and artificial ventilation in the intensive care unit. Repetitive stimulation studies showed a marked decrement of the trapezius muscle response reversible with edrophonium. Ventilatory function considerably and lastingly improved under anticholinesterase treatment. In the absence of ...

Early-onset respiratory distress and a radiographic appearance of an aspiration syndrome occurred in three neonates who had not passed meconium before delivery. In each case there was evidence of inhalation of blood, associated with very high plasma protein concentration in lung fluid. Blood aspiration syndrome is a distinct diagnostic entity ...

OBJECTIVE: An increased oxidative stress in the lower respiratory tract of individuals with acute respiratory distress syndrome is considered to be one mechanism of lung injury in these patients. Cell and tissue damage resulting from an oxidative stress can ultimately be the consequence of a disruption of normal iron metabolism ...

Congenital central alveolar hypoventilation syndrome (CCAHS) is a disorder of ventilatory control that occurs without neuromuscular blockade or pulmonary disease. It is characterized by a lack of response to habitual respiratory stimulants, especially hypercapnia. In this article, the management of this syndrome by diaphragmatic pacing is discussed. Paralysis of the ...

Acute respiratory distress syndrome (ARDS) is an acute lung injury of high mortality rate, and sepsis syndrome is one of the most frequent causes of ARDS. Metabolites of arachidonic acid, including thromboxanes and leukotrienes, are proinflammatory mediators and potentially involved in the development of ARDS. A key enzyme for the ...

Airway obstruction is common among patients with craniosynostosis. We describe an infant with a clinical and genetic diagnosis of Crouzon syndrome who presented with respiratory distress and heart murmur in early neonatal life. Cardiac evaluation revealed absent pulmonary valve syndrome. She needed intubation at age 1 month, and repeated trials ...

In a rare case of Good's syndrome with pernicious anemia, one year after thymectomy, recurrent respiratory infections developed. Both panhypoglobulinemia and pernicious anemia were disclosed. An immunological analysis revealed the absence of circulating B cells, but T cell numbers and mitogenic responses were normal. Regular gammaglobulin and vitamin B12 injections ...

OBJECTIVE: To test the hypothesis that elevated concentrations of interleukin-8 associated with anti-interleukin-8 autoantibodies (anti-interleukin-8:interleukin-8 complexes) are found in patients at risk for acute respiratory distress syndrome who developed the disease. DESIGN: Measurement of anti-interleukin-8:interleukin-8 complex concentrations in previously collected bronchoalveolar lavage fluids. These fluids were obtained from patients at ...

Risk factors for Kawasaki syndrome (KS) were evaluated through a case-control study during an investigation of a KS cluster in Denver, CO. KS was associated with a humidifier in the child's room (odds ratio, 7.3; 95% confidence interval, 1.8 to 29.3) and possibly with an antecedent respiratory illness. The use ...

Idiopathic congenital central hypoventilation syndrome (CCHS) is a rare disorder in which affected children have a decreased sensitivity of their respiratory centers to hypercarbia and hypoxia, as well as evidence for generalized autonomic nervous system dysfunction. A genetic origin has long been hypothesized for CCHS. Previous reports of the syndrome ...

A 7-year-old boy with Down syndrome developed severe acute respiratory distress syndrome after a respiratory infection with Mycoplasma pneumoniae with an unusually high agglutination titre (1:10240). Initially, mechanical ventilation and nitric oxide inhalation were used, but these did not improve the alveolar-arterial oxygen gradient. Extracorporeal membrane oxygenation for 152 h ...

Morquio's syndrome is an inherited disorder characterized by excessive excretion of keratan sulphate in the urine. The anaesthetic care of these patients should take into consideration respiratory, craniofacial, cardiac, skeletal, ocular and hepatic abnormalities. We report the case of a child with Morquio's syndrome who presented for stabilization of the ...

Ischemic optic neuropathy is a rare cause of blindness reported most commonly in association with collagen-vascular diseases, infectious processes, and systemic hypotension related to massive exsanguinating hemorrhage. We report what we believe to be the first case of posterior ischemic optic neuropathy due to perioperative hypotension in a patient who ...

OBJECTIVE: To describe the clinical presentation of propofol infusion syndrome in critically ill adults. DATA SOURCES: Clinical literature was accessed through MEDLINE (1966 - March 2001). Key search terms included Diprivan, propofol, and propofol infusion syndrome. Case reports and small case series evaluating the use and toxicity of propofol in ...

The use of low tidal volumes with permissive hypercapnia in patients with acute respiratory distress syndrome may require heavy sedation to allow them to tolerate mechanical ventilation. Administration of methadone for sedation is an alternative to using other opioids, given its longer elimination half-life and incomplete cross-tolerance with other mu-receptor-active ...

Using the self-reports of 300 university students, we replicated and extended the results of a 1971 study of Hersen by demonstrating a significant relationship between both nightmare frequency and nightmare distress with two dimensions of anxiety. Our results are not consistent with the 1990 findings of Wood and Bootzin who ...

Vocal cord dysfunction (VCD) has been frequently discussed as confounding the diagnosis of asthma, and it is recommended to examine vocal cord function in cases of difficult asthma. However, there is also strong evidence that asthma may be associated with VCD, sometimes masquerading as exercise-induced asthma. Gerhardt's syndrome frequently induces ...

OBJECTIVE: To ascertain the feasibility and the safety of percutaneous dilational tracheostomy in patients with acute respiratory distress syndrome receiving high-frequency oscillatory ventilation. DESIGN: Case series. SETTING: Tertiary adult intensive care unit in a university teaching hospital. PATIENTS: Five patients with acute respiratory distress syndrome. INTERVENTIONS: Percutaneous dilational tracheostomy during ...

Coccidioides immitis causes a benign upper respiratory tract infection in endemic areas. Acute respiratory distress syndrome is a rare complication that is associated with high mortality. Corticosteroids have been avoided as adjunctive therapy to antifungals in the treatment of acute respiratory distress syndrome secondary to coccidioidomycosis because of the fear ...

Discovery Laboratories Inc (formerly Acute Therapeutics Inc (ATI) is developing lucinactant, originally identified at the Scripps Research Institute and sublicensed from Johnson & Johnson, for the potential treatment of respiratory diseases [174059], [357077], [361765], [422819]. The company anticipated filing an NDA for lucinactant in 2002, and by March 2002, lucinactant ...

We describe a 10-month-old boy diagnosed with X-linked hyper-IgM syndrome (XHIM) after suffering from life-threatening acute respiratory distress syndrome (ARDS) caused by Pneumocystis carinii pneumonia (PCP), although his previous clinical history and first level laboratory tests investigating immunological function did not indicate immunodeficiency. When the patient's overall condition was good, ...

A 50 year old man presented with 3 weeks of exertional dyspnoea. His chest radiograph on admission revealed diffuse bilateral interstitial infiltrates. He did not respond to antibiotics but subsequently improved on high dose corticosteroids. Bronchoscopic examination with transbronchial biopsy specimens revealed the presence of non-necrotising granulomas. This case demonstrates ...