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Background and Aim: Spinal muscular atrophy (SMA) is a neuromuscular disease characterized by progressive symmetrical weakness and atrophy of proximal muscles causing from degeneration of anterior horn cells of spinal cord. Nusinersen must be administered intrathecally and this treatment is specially for spinal muscular atrophy. Procedural sedation is commonly enough for intrathecal treatment in children. In this retrospective study, the investigators aimed to present our experience in procedural sedation for the intrathecal treatment of patients with SMA 1,2 and 3 in our hospital.
Design: This research is a retrospective obsevational study. Methods: After approval Institutional Review Board, data from anesthesia charts and electronic medical records of 14 patients with SMA type I, II and III who underwent procedural sedation,such as; the demographics data of the patients (age, gender, weight, American Society of Anesthesiologist (ASA) Physical Status), which anesthetic agent was used and their doeses, during of anesthesia, presence of scoliosis, by whom the procedure was performed etc. was reviewed. All our patients with SMA type II and III who underwent intrathecal treatment are given sedation with different combination of anesthetic agent. Due to peripheral intravenous line was placed previously, intravenous induction was performed in all of our patients For procedural sedation, anesthesiologists used different combination of midazolam, ketamine, propofol, fentanyl or remifentanyl, depending on the patient's requirement. They were oxygenated with a face mask or nasal cannula while their spontaneous breathing continues. All patients were recovered in the recovery room.
Statistical analysis: The data were analyzed by using SPSS Statistics Software (SPSS 24, Chicago, IL, USA) Descriptive statistics were used in this study. Mean and standart deviation were calculated for Age (year), Weight (kilogram), Midazolam Dose (mg/kg), Fentanyl Dose (mcg/kg), Remifentanyl Dose (mcg/kg), Propofol Dose (mg/kg), Ketamine Dose (mg/kg) and Length of Stay in Recovery Room (minute) and frequency analysis were made for the number of intervention.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Retrospective observational study | Other | Data from anesthesia charts and electronic medical records of 14 patients with SMA type I, II and III who underwent procedural sedation,such as; the demographics data of the patients (age, gender, weight, American Society of Anesthesiologist (ASA) Physical Status), which anesthetic agent was used and their doeses, during of anesthesia, presence of scoliosis, by whom the procedure was performed etc. was reviewed. |
| Measure | Description | Time Frame |
|---|---|---|
| Procedural sedation | The number of pediatric patients with SMA I,II and III who underwent intrathecal treatment required procedural sedation | 4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| Anesthetics use | The anesthetic agents and their doses that used during procedure | 4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| Age | Age in year | 4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| Gender | Gender as female/male | 4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| Weight | Weight in kilograms | 4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| American Society of Anesthesiologists (ASA) physical status | ASA score | 4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| Type of SMA | SMA I, SMA II and SMA III | 4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| Measure | Description | Time Frame |
|---|---|---|
| Presence of scoliosis | Yes or no | 4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.)( |
| How many times intrathecal treatment was performed for each patient |
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Inclusion Criteria:
Exclusion Criteria:
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Pediatric patients younger than 18 years old with SMA 1,2 and 3 who were to undergo intrathecal treatment under procedural sedation in our clinic
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| Name | Affiliation | Role |
|---|---|---|
| Feyza Sever | Ankara City Hospital Bilkent | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Feyza Sever | Ankara | Çankaya | 06800 | Turkey (Türkiye) |
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| ID | Term |
|---|---|
| D009134 | Muscular Atrophy, Spinal |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
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The number of intrathecal treatments was administered for each patient over 4 years was recorded.
| 4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| By whom the procedure was performed | It was recorded who performed the lumbar puncture procedure from a neurologist, neurosurgeon, or anesthesiologist. | 4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| Length of time spent in recovery room | Recovery time in minutes | 4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.) |
| D019636 | Neurodegenerative Diseases |
| D009468 | Neuromuscular Diseases |