Anaesthetic management of an infant with spinal muscular atrophy [Type 1] for fundoplication and feeding gastrostomy

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چکیده

Anaesthetic management of an infant with spinal muscular atrophy [Type 1] for fundoplication and feeding gastrostomy - IJCA- Print ISSN No: 2394-4781 Online No:- 2394-4994 Article DOI 10.18231/j.ijca.2023.044, Indian Journal Clinical Anaesthesia-Indian J Clin Anaesth

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Respiratory management of the infant with type 1 spinal muscular atrophy.

A recent paper has highlighted the differences in the respiratory management offered to infants with type 1 spinal muscular atrophy (SMA-1). Current views appear polarised between those who would offer nothing, to those who would proceed as far even as tracheostomy and long term invasive ventilation for these infants. Here we offer a personal view, as a possible template for managing a vexed an...

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Tongue fasciculations in an infant with spinal muscular atrophy type 1

Muscular hypotonia in infants may be associated with several conditions, such as spinal muscular atrophy (SMA). We report on an infant with tongue fasciculations and a rare mutation of the SMN1 gene. The presence of tongue fasciculations in combination with a thorough history may be suggestive of SMA.

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Spinal muscular atrophy type 1: A noninvasive respiratory management approach.

STUDY OBJECTIVE To determine whether spinal muscular atrophy (SMA) type 1 can be managed without tracheostomy and to compare extubation outcomes using a respiratory muscle aid protocol vs conventional management. DESIGN A retrospective cohort study. METHODS Eleven SMA type 1 children were studied during episodes of respiratory failure. Nine children required multiple intubations. Along with...

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Survival of patients with spinal muscular atrophy type 1.

BACKGROUND Spinal muscular atrophy type 1 (SMA1) is a progressive disease and is usually fatal in the first year of life. METHODS A retrospective chart review was performed of SMA1 patients and their outcomes according to the following choices: letting nature take its course (NT); tracheostomy and invasive mechanical ventilation (TV); continuous noninvasive respiratory muscle aid (NRA), inclu...

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Eighteen Years with Spinal Muscular Atrophy (SMA) Type 1

INTRODUCTION SMA-1, also known as Werdnig-Hoffmann disease, is the most common neuromuscular disease of hypotonic newborns. Its incidence is over 1 per 8000 births and is a common cause of sudden infant death syndrome. It is characterized by the degeneration of the anterior horn cells of the spinal cord and of the motor nuclei in the lower brainstem (1). About 99% of patients have deletions in ...

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ژورنال

عنوان ژورنال: Indian Journal of Clinical Anaesthesia

سال: 2023

ISSN: ['2394-4781', '2394-4994']

DOI: https://doi.org/10.18231/j.ijca.2023.044