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Signa Vitae

Journal of Anaesthesia, Intensive Care and Emergency Medicine

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Lethal respiratory failure due to a surfactant protein C gene mutation

Abstract

A case of a full-term male infant with recurrent episodes of „obstructive bronchitis” and „bilateral pneumonias”, refractory to conventional asthma therapy, is reported. When referred to the paediatric clinic at the age of 15 months, his clinical presentation was of acute respiratory distress syndrome with severe hypoxemia, which was easily corrected by the inhalation of oxygen. A series of further investigations, including lung biopsy, suggested the diagnosis of childhood interstitial lung disease (ILD). Due to progressive respiratory failure, the boy died after 3 months of mechanical ventilator support, at the age of 19 months. Subsequently performed genetic studies revealed that he was heterozygous for the I73T mutation of the surfactant protein C precursor protein. Thus, suspicion of childhood ILD due to genetic mutations that influence surfactant metabolism should be raised in children who present with unexplained respiratory distress or chronic respiratory symptoms. Less invasive testing, such as surfactant protein genetics, may be diagnostic in such cases.

 

Key words: child, interstitial lung disease, respiratory distress, surfactant deficiency, surfactant protein C

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Successful use of venovenous extracorporeal membranous oxygenation in a 22-month old boy with necrotizing pneumonia, osteomyelitis and septic shock caused by Panton Valentine leukocidin – producing Staphylococcus aureus

Abstract

Extracorporeal membrane oxygenation (ECMO) is a life saving treatment for patients with severe respiratory failure. We present a case of a young child with invasive Panton Valentine leukocidin-producing Staphylococcus aureus infection, which is responsible for severe and invasive infection with a high mortality rate, commonly associated with necrotizing pneumonia. Our patient presented with septic shock and necrotizing pneumonia leading to severe respiratory failure, refractory to conventional ventilation means. After 1-day of treatment, venovenous ECMO (VV ECMO) was successfully instituted and inotropic support was gradually decreased. Acute renal failure was managed with peritoneal dialysis and intermittent venovenous hemofiltration. The patient was weaned from ECMO 9-days later and was mechanically ventilated for another 3 weeks. Necrotizing pneumonia with pleuropulmonary complications was finally managed by videothoracoscopy with evacuation of debris and partial pleural decortication. Osteomyelitis was confirmed by positron emission tomography – computed tomography (PET-CT) and was surgically treated. The child was treated with antistaphylococcal antibiotic therapy for 54 days. Finally, he was discharged to a rehabilitation center without supplemental oxygen and with his neurologic status at his baseline. Our case shows that VV ECMO can be applied to children with severe bacterial pneumonia resistant to conventional ventilation strategies and with moderate circulatory failure.

Key words: extracorporeal membranous oxygenation, septic shock, Staphylococcus aureus pneumonia, Staphylococcus aureus, Panton Valentine leukocidin, child

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Diluted porcine surfactant lung lavages in children with severe ARDS

Abstract

Acute respiratory distress syndrome (ARDS) is characterized by damage to the arteriolar-capillary endothelium and alveolar epithelium that leads to surfactant deficiency and atelectasis.  Alveolar collapse and pulmonary edema will further induce surfactant inactivation. Surfactant supplementation has been suggested but results are unpredictable. Poor response may be due to inhibition of administered surfactant by plasma components filling the alveolar space, severity of lung injury, time of surfactant application and inadequate dose. We report the course of gas exchange and pulmonary mechanics after instillation  of surfactant in 14 children (3 months-7 years) with severe ARDS, defined as an oxygenation index (OI) > 30 and a partial pressure of oxygen/ fraction of Inspired oxygen (PaO2/FiO2) 200 for more than 12 hours.  Diluted surfactant lung lavages were able to increase blood gas exchange in all our patients despite previously severe gas exchange impairment.

Keywords: ARDS, pulmonary surfactant, bronchoalveolar lavages, child

 

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Pediatric Shock

Abstract

Millions of children die of shock due to various etiologies each year. Shock is a state of circulatory dysfunction where the metabolic demands of the tissue cannot be met by the circulation. Several different etiologies from hypovolemia to severe infection can result in shock. This review focuses on the definition of different types of shock seen in children and summarizes treatment strategies for the acute care practitioner based on pertinent recent literature. Early recognition and timely intervention are critical for successful treatment of pediatric shock. A strong index of suspicion by the treating clinician and early fluid resuscitation followed by ongoing assessment and timely transfer to a higher level of care can make the difference between life and death for the child who presents in shock.

Key words: shock, child, etiology, treatment

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Extracorporeal membranous oxygenation (ECMO) in neonates and children experiences of a multidisciplinary paediatric intensive care unit

Abstract

Extracorporeal membranous oxygenation ECMO was applied in 18 patients. All children had deep hypoxia and 80% probability of dying. Average duration of ECMO in newborns was 131 hours, and in older patients 253 hours. Seven patients were discharged from the intensive care unit (late survivors), 5 of them are in perfect somatic and mental condition.

Key words: extracorporeal membra-nous oxygenation, neonate, child

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