Differential diagnostics critical illness myopathy and their medical management in children
DOI:
https://doi.org/10.15574/SP.2022.122.72Keywords:
critical illness myopathy, X-linked miotubular myopathy, spinal muscular atrophy, disease-modified therapyAbstract
Critical illness myopathy (CIM) is a challenging problem for identifying which impacts on verified diagnosis in time. CIM occurs often as one of respiratory failure cause for intensive care admission and invasive ventilation is needed and subsequent inability to wean from the ventilator. It also influences on the length period of patient’s staying in the intensive care unit (ICU).
Purpose - to provide the analysis of clinical signs of CIM in children and their medical management.
Materials and methods. We examined the group of children aged from 3 month up to 15 years old. Patients were needed critical care in ICU. We utilized clinical, instrumental and laboratory methods of investigation.
Results. We have carried out differential diagnostics and the causes of CIM in presented clinical cases which have been shown. Neuromuscular causes of weakness in the children have been identified. X-linked myotubular myopathy was the cause for CIM in the first patient. Muscular weakness in patient with spinal muscular atrophy (SMA) combined with pneumonia and sepsis was the reason for critical care in the next patient. Prescribed antibacterial therapy combined with symptomatic one in the patient with X-linked miotubular myopathy as well as the antibacterial therapy connected with the disease-modified medicine and respiratory support in the patient with SMA showed positive result of treatment.
Conclusions. Diffuse muscular hypotonia, hyporeflexia, muscular weakness were main clinical signs of CIM in the patient with X-linked miotubular myopathy as well as in the patient with SMA, type I. Presented symptoms appeared from the child’s newborn period. The prescribed disease-modified therapy combined with antibacterial one and respiratory support proved effectiveness and allowed us to wean the patient from the ventilator.
The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies.
No conflict of interests was declared by the authors.
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