Anesthetic considerations in the child with gaucher disease

Tobias, Joseph D.; Atwood, Robert; Löwe, Sandra; Holcomb, George

doi:10.1016/0952-8180(93)90144-4

Cited by 16 publications

(7 citation statements)

References 8 publications

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“…In cases when it is decided to switch to enteral feedings, a nasogastric tube or gastric tube insertion with gastrostomy are two acceptable approaches. Decisions regarding the most appropriate approach should take into account potential anesthesia- related complications during a gastrostomy or gastric tube insertion procedure [27]. …”

Section: The Management Of Patients With Type 2 Gaucher Diseasementioning

confidence: 99%

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The clinical management of type 2 Gaucher disease

Weiss

Gonzalez

Lopez

et al. 2015

Molecular Genetics and Metabolism

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Gaucher disease, the inherited deficiency of the enzyme glucocerebrosidase, is the most common of the lysosomal storage disorders. Type 2 Gaucher disease, the most severe and progressive form, manifests either prenatally or in the first months of life, followed by death within the first years of life. The rarity of the many lysosomal storage disorders makes their diagnosis a challenge, especially in the newborn period when the focus is often on more prevalent illnesses. Thus, a heightened awareness of the presentation of these rare diseases is necessary to ensure their timely consideration. This review, designed to serve as a guide to physicians treating newborns and infants with Gaucher disease, discusses the presenting manifestations of Type 2 Gaucher disease, the diagnostic work-up, associated genotypes and suggestions for management. We also address the ethical concerns that may arise with this progressive and lethal disorder, since currently available treatments may prolong life, but do not impact the neurological manifestations of the disease.

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Section: The Management Of Patients With Type 2 Gaucher Diseasementioning

confidence: 99%

“…These may be triggered by pre-anesthesia medications or manipulation of the airway [27]. Difficult airway access may require the need for smaller endotracheal tubes [27]. Pulmonary involvement in GD is another risk factor for anesthesia-related complications.…”

Section: The Management Of Patients With Type 2 Gaucher Diseasementioning

confidence: 99%

The clinical management of type 2 Gaucher disease

Weiss

Gonzalez

Lopez

et al. 2015

Molecular Genetics and Metabolism

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show abstract

“…Because GD is a disease with multiorgan involvement, preoperative assessment should be carried out in order to determine the extent of organ involvement, with particular emphasis on central nervous system, liver, spleen, bone, bone marrow and probably the lung (6,7). Hepatosplenomegaly is the most common sign of visceral involvement in GD and may be seen in all three types (8).…”

Section: Discussionmentioning

confidence: 99%

Anesthetic Management of Gaucher Disease

et al. 2013

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“…Previous reports in the literature described cases where neurological dysfunction primarily determined the mode of anesthesia, while our experience was in relatively short surgeries for non-life-threatening complications, and in patients without neurological involvement. 6,7 …”

Section: Discussionmentioning

confidence: 99%