Decreased salivary output in patients with Gaucher disease.
BACKGROUND
Gaucher disease, the most common sphingolipid storage disease, results in accumulation of glucocerebroside in macrophages or "Gaucher cells". In a preliminary screening of 109 patients with type I disease, when asked specifically about dry mouth, approximately one quarter claimed to suffer from this symptom.


OBJECTIVE
To ascertain whether decreased salivary output is a feature of Gaucher disease.


METHODS
Prospective case-control study.


METHODS
Salivary output was measured in 65 adult patients and 65 healthy controls using the Saxon test with Hochberg's modification.


RESULTS
Mean salivary output was 1.91+/-1.19 g/min in the patient group vs. 2.74+/-1.17 g/min in the control group (p<0.001). This difference was greater among males. These results were not improved in the patients receiving enzyme replacement therapy, which is effective in ameliorating most Gaucher-related signs and symptoms.


CONCLUSIONS
Recent studies have implicated an association between sicca syndrome and viral hepatitis C infection, which may imply an immunological trigger for these findings, but in this specific cohort, only three patients were reactive for hepatitis C. Follow-up of patients, both untreated and receiving enzyme therapy, is needed to delineate the association with salivary hypofunction, and ascertain whether enzyme therapy may induce sicca symptoms.
Dayan
B
B
Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem 91031, Israel.
Elstein
D
D
Zimran
A
A
Nesher
G
G
D000328:Adult; D016022:Case-Control Studies; D005260:Female; D005776:Gaucher Disease / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012472:Salivation; D012720:Severity of Illness Index; D014987:Xerostomia / Q000209:etiology*