Attack triggers


 

  1. Introduction
  2. Trigger overview

 

1. Introduction

In the literature, a host of triggers are mentioned that need to be avoided in the case of CPT 2 deficient patients, otherwise a rhabdomyolytic attack may occur. Conversely, if a rhabdomyolytic attack is already in progress, then it is essential that all triggers that are present and active should be contained, restricted or eliminated.

Please note that it is not the case that people invariably will get an attack if one of the triggers mentioned is at play. Nevertheless, prevention is always better than cure, and CPT 2 deficiency patients and their doctors should keep these triggers in mind, and avoid them if possible, or otherwise treat them.

 

2. Trigger overview

A (potential) trigger for a rhabdomyolysis attack in CPT 2 deficient patients is, in general, anything that increases the dependance on lipid metabolism. The following attack triggers are mentioned in the literature:

Illnesses:
Infections (viral)
Fever

Physical:
Prolonged physical exertion
Exposure to cold
Sleep deprevation
Heavy injuries
Operations (and other emergencies)

Dietary:
Fasting
High fat intake

Drug-induced (see further):
General anesthesia (most drugs, but not all)
Common drugs, such as Ibuprophen, Paracetamol, Diazepam
Alcohol

Emotional issues:
Stress
Trauma
Operations (and other emergencies)

 

 

 

1. Emergencies: Traumata, accidents, operations; medications to avoid

In the first place all such situations will challenge an individuals (fat) metabolism.

They may also require medication, emergency measures and operations.

It should be born in mind that there are a host of medications that could trigger a rhabdomyolytic attack. The following are mentioned in the literature:

(from E.B. Larbi, MB, PhD, FRCP)

TABLE 1. Drugs which can cause rhabdomyolisis.

Antipsychotics and antidepressants
Amitriptyline24
Amoxapine
Doxepine52
Fluoxetine53
Fluphenazine54
Haloperidol55
Lithium56
Protriptyline57

Phenelzine58
Perphenazine24
Promethazine59
Chlorpromazine
Loxapine60
Promazine59
Trifluoperazine


Sedative hypnotics
Benzodiazepines:22
–Diazepam66
–Nitrazepam71
–Flunitrazepam75


-Lorazepam22,24
-Triazolam66
Barbiturates21
Gluthetimide21

Antilipemic agents
Lovastatin65
Pravastatin11
Simvastatin
Bezafibrate68


Clozafibrate69
Ciprofibrate70
Clofibrate64

Drugs of addiction
Heroin 34,61
Cocaine25,26,62


Amphetamine63
Methadone84

Antihistamines36
Diphenhydramine66
Doxylamine67

 

Others

Anesthetics:

Halothane, Enflurane, Desflurane, Sevoflurane,ether, Methoxyflurane, Cycloprone

Alcohol17,19,72-74
Amphotericin B87
Azathioprine89
Butyrophenones55
Emetics
Epsilon-aminocaproic acid33

Laxatives
Moxalactam85
Narcotics20,21
Oxprenolol88
Paracetamol25
Penicillamine

Pentamidine86
Phencyclidine5
Phenytoin77
Phenylpropanolamine78
Quinidine76
Salicylates25
Strychnine79
Succinylcholine82
Theophyline80
Terbutaline
Thiazides81
Vasopressin90

 

 

The particular issue of concern is the use of certain anesthetics (such as mentioned in the above) that can trigger a similar response as in patients prone to Malignant Hyperthermia (MH).

Like in MH these anesthetics should be avoided in patients with CPT deficiency and the same protocol followed as with MH patients.

The protocol that follows is taken and modified from the protocol given by MHAUS (the Malignant Hyperthermia Association of the US)

 



Version Revision date Revised by Comment
0.1 14 august 2000 MHN Needs better layout