Table 1.
Patient clinical characteristics and disease course.
Characteristic | Number (%) |
---|---|
Patient number | 175 (100%) |
MEN 1 present (a) | 45 (26%) |
Gender | |
Male | 91 (52%) |
Female | 84 (48%) |
Race | |
White | 137 (78%) |
Black | 29 (17%) |
Hispanic | 6 (4%) |
Asian | 3 (1%) |
Age at ZES onset (yrs.) (b) | |
Mean ± SEM | 39.9 ± 0.9 |
(Range) | (12.9–65.0) |
Age at ZES diagnosis (yrs.) (c) | |
Mean ± SEM | 45.8 ± 0.9 |
(Range) | (14.2–70.6) |
Age at 1st NIH visit | |
Mean ± SEM | 47.8 ± 0.9 |
(Range) | (13.1–71.0) |
Age at the time of present study | |
Mean ± SEM | 53.8 ± 0.9 |
(Range) | (21.3–81.8) |
Presenting clinical symptoms/features (d) | |
Pain | 135 (77%) |
Ulcer history | 121 (63%) |
Diarrhea | 140 (80%) |
GERD (any) | 88 (50%) |
GERD(severe) | 24 (14%) |
GERD/PUD Complication (e) | 31 (18%) |
Bleeding | 46 (26%) |
Duration (yrs.) | |
From ZES onset to the present study | |
Mean ± SEM | 13.7 ± 0.6 |
(Range) | (0.5–42.1) |
From ZES onset to last follow-up/death (f) | |
Mean ± SEM | 19.4 ± 1.3 |
(Range) | (2.4–40.7) |
From 1st NIH visit to last follow-up/death (f) | |
Mean ± SEM | 11.4 ± 0.9 |
(Range) | (1.2–23.7) |
Abbreviations: ZES—Zollinger–Ellison syndrome; MEN1—Multiple Endocrine Neoplasia type 1; yrs.—years; NIH—National Institutes of Health; GERD—gastroesophageal reflux disease; PUD—peptic ulcer disease; and PMD—patient’s private medical doctor. (a) MEN1 was diagnosed using family history, serum calcium, prolactin, and PTH assays as described previously [100,156,157,158,159,160,161]. (b) Onset is defined as the onset of recurrent and/or persistent symptoms compatible with ZES as defined previously [106,162]. (c) Criteria for diagnosis of ZES required assessment of BAO/fasting gastrin/secretin test as described previously [104,150,163]. (d) Clinical symptoms were determined as described previously [164,165]. (e) Other GERD/PUD complications include nonbleeding complications (stricture [esophageal, duodenal, and small intestine], perforation, penetration, obstruction, and advanced Barrett’s esophagus) as defined previously [103,164,166]. (f) All patients were followed in the long term (≥5 years) at NIH and treated with gastric antisecretory drugs, except for patients who had an early death, surgical cure with low acid secretion [162,167,168,169], or returned to their PMD after diagnosis stabilization and evaluation [106]. During follow-up, 25 patients died, with 8 patients having a ZES-related death, and in 17 patients, the death was not ZES-related [101,170].