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Table 2 Case mix, surgical and anesthetic characteristics in patients with glycogen storage disease

From: Patients with glycogen storage diseases undergoing anesthesia: a case series

Pt

Agea at Surgery, y

Surgery/Anesthesia duration, min/Airway/Anesthetic Agents

Fluid used

Disposition

GSD type 0

  

1

51–60

Muscle biopsy/120,P

LR

Outpatient

GSD type I (Von Gierke)

  

2

19–30

Liver transplant/360/P, C, S, Iso

NS; D5W; PRBC; Cryo; Albumin

ICU

3

11–18

Liver transplant/300/P, C, Iso

NS; D5W; Albumin

ICU

4

6–10

Liver transplant/330/P, V, Iso

NS; D5NS; PRBC; Albumin

ICU

5

1–2

Muscle biopsy/120/Sev, N2O

D5NS

Outpatient

1–2

Gastric tube placement/60/P, At, Iso

D10NS

Ward

6

<1

Gastric tube placement/100/P, At, Sev

LR; D10NS

Ward

3–5

Tonsillectomy/110/P, V

LR; D10NS

Ward

3–5

Myringotomy/ 100/P

LR; D10NS

Ward

7

11–18

Rectal abscess I&D/70/At, STP, N2O

D10W

Outpatient

11–18

Abscess I&D/120/P, At, N2O

LR; D10W

Outpatient

GSD type II (Pompe)

  

8

11–18

Anterior spinal fusion/300/STP, Iso, Sev, P

LR; PRBC; FFP; Platelets; Hetastarch; Albumin

ICU

11–18

Posterior spinal fusion/730/P, Sev, N2O

LR

ICU

9

<1

Muscle biopsy/75/P, N2O

LR

Outpatient

10

41–50

Muscle biopsy/MAC/60, P

LR; NS

Ward

41–50

Tracheostomy/80/E, S, Sev

LR

ICU

11

51–60

Muscle biopsy/MAC/90 mask, P

NS

Outpatient

 12

31–40

Myocardial biopsy/MAC/90, Mz, AL

LR

Outpatient

31–40

Parathyroidectomy/120/E, V, Iso

LR

Ward

13

41–50

Muscle biopsy/MAC/60,P

LR

ICU

14

41–50

Muscle biopsy/MAC/120/ Mask, P

NS; LR; Albumin

Outpatient

15

3–5

Myectomy (ICD)/ CPB/280,Sev, V, Iso

LR

ICU

 

3–5

Sternal wire removal/180/E, RO, Sev

 

Ward

Glycogen storage disease type IV (Andersen)

  

16

1–2

Liver biopsy/MAC/60/ Mask, Sev

LR

Outpatient

1–2

Myringotomy/GA/60/Sev, N2O

None

Outpatient

17

61–70

Cervical discectomy/200, P, RO

 

Ward

Glycogen storage disease type V (McArdle)

  

18

11–18

Muscle biopsy/60)/ LMA/Sev, N2O

LR

Outpatient

19

6–10

Muscle biopsy/60/P, Sev, N2O

LR

Outpatient

20

41–50

Whipple /GA /360/P, S, V, Iso

LR

Ward

21

41–50

Muscle biopsy/MAC/60/ Mask/P

LR

Outpatient

22

11–18

Muscle biopsy/MAC/90/ Mask/P, Sev

LR

Outpatient

23

19–30

Muscle biopsy/ MAC/105/ Mask, P

LR

Outpatient

24

19–30

Muscle biopsy/ MAC/60/ Mask, P

LR

Outpatient

25

6–10

Muscle biopsy/70/ LMA, P

LR

Outpatient

26

19–30

Muscle biopsy/ MAC/120/ Mask, P

LR

Outpatient

27

>70

Muscle biopsy/MAC/60/ Mask, P

LR

Outpatient

GSD type VI (Hers)

  

28

1–2

Liver biopsy/105/Sev, RO

D5NS

Outpatient

GSD type VII (Tarui)

  

29

61–70

Muscle biopsy/MAC/210/Mask, P

LR

Outpatient

Unidentified, likely glycolytic defect

  

30

31–40

Dilation & curettage/60/LMA/P, N2O

LR

Outpatient

31–40

Mammoplasty/240/P, V, N2O

LR

Ward

31–40

Breast hematoma evacuation/90/LMA/P, N2O

LR

Ward

  1. Abbreviations: At atracurium, BiPAP bilevel positive airway pressure, C cisatracurium, CPB cardiopulmonary bypass, D10NS 10% dextrose with 0.9% normal saline, D10W 10% dextrose with water, D5NS 5% dextrose with 0.9% normal saline, D5W 5% dextrose with water, E etomidate, F female, GA general anesthesia, ICD implantable cardioverter-defibrillator; ICU intensive care unit, I&D incision and drainage, Iso isoflurane, LMA laryngeal mask airway, M male, Mz midazolam, MAC monitored anesthesia care, N 2 O nitrous oxide, NA not available, NS 0.9% normal saline, P propofol, PRBC packed red blood cells transfusion, Pt patient, RO rocuronium, S succinylcholine, Sev sevoflurane, STP sodium thiopental, V vecuronium
  2. aAge is reported as a range (in year): <1 year; 1–2; 3–5; 6–10; 11–18; 19–30; 31–40; 41–50; 51–60; 61–70; >70
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BMC Anesthesiology

ISSN: 1471-2253

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