Identification of multiple endocrine neoplasia type 1 in patients with apparent sporadic primary hyperparathyroidism

Yip, Linwah; Ogilvie, Jennifer B.; Challinor, Susan M.; Salata, Rose A.; Thull, Darcy L.; Yim, John H.; Carty, Sally E.

doi:10.1016/j.surg.2008.07.030

Cited by 31 publications

(17 citation statements)

References 18 publications

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“…Of note, the penetrance of HPT in MEN1 is almost complete at the age of 50 years. (4)(5)(6)(7)(8)15) Urolithiasis and reduced BMD/osteoporosis lead to a recommendation of PTX in SPHPT. (31) However, the optimal timing for PTX in HPT/MEN1 still remains controversial.…”

Section: Discussionmentioning

confidence: 99%

“…This division point also coincides with the age at which penetrance of HPT in MEN1 is almost complete. (4)(5)(6)(7)(8)15) Some definitions were used in this study. The term severe demineralization was applied to patients with at least one of the four analyzed bone sites with Z-scores of less than À3.0 (group A) or T-scores of less than À3.0 (group B).…”

Section: Biochemical Measurementsmentioning

confidence: 99%

“…In MEN1, primary HPT is frequently the first clinical manifestation ($75%), and its penetrance is almost complete (82% to 100%) at age 50 years. (4)(5)(6)(7)(8) HPT/MEN1 differs from sporadic HPT (SPHPT) in several aspects because it presents with multiglandular parathyroid hyperplasia instead of parathyroid adenoma, earlier disease onset (20 to 35 versus 50 to 70 years), a sex ratio of 1:1 in contrast to the 1:3 ratio in SPHPT, and higher recurrence rates after parathyroidectomy (PTX), and it is mostly associated with a germ-line MEN1 mutation that is absent in SPHPT patients. (4,6,9) The early recognition of HPT/MEN1 is crucial, and its optimal surgical management consists of multiglandular removal associated with a preventive thymectomy instead of the adenomectomy usually recommended in patients with SPHPT.…”

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confidence: 99%

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Early-onset, progressive, frequent, extensive, and severe bone mineral and renal complications in multiple endocrine neoplasia type 1–associated primary hyperparathyroidism

Lourenço

Coutinho

Toledo

et al. 2010

Journal of Bone and Mineral Research

102

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Differences in bone mineral density (BMD) patterns have been recently reported between multiple endocrine neoplasia type 1-related primary hyperparathyroidism (HPT/MEN1) and sporadic primary HPT. However, studies on the early and later outcomes of bone/renal complications in HPT/MEN1 are lacking. In this cross-sectional study, performed in a tertiary academic hospital, 36 patients cases with uncontrolled HPT from 8 unrelated MEN1 families underwent dual-energy X-ray absorptiometry (DXA) scanning of the proximal one-third of the distal radius (1/3DR), femoral neck, total hip, and lumbar spine (LS). The mean age of the patients was 38.9 AE 14.5 years. Parathyroid hormone (PTH)/calcium values were mildly elevated despite an overall high percentage of bone demineralization (77.8%). In the younger group (<50 years of age), demineralization in the 1/3DR was more frequent, more severe, and occurred earlier (40%; Z-score À1.81 AE 0.26). The older group (>50 years of age) had a higher frequency of bone demineralization at all sites ( p < .005) and a larger number of affected bone sites ( p < .0001), and BMD was more severely compromised in the 1/3DR ( p ¼ .007) and LS ( p ¼ .002). BMD values were lower in symptomatic (88.9%) than in asymptomatic HPT patients ( p < .006). Patients with long-standing HPT (>10 years) and gastrinoma/HPT presented significantly lower 1/3DR BMD values. Urolithiasis occurred earlier (<30 years) and more frequently (75%) and was associated with related renal comorbidities (50%) and renal insufficiency in the older group (33%). Bone mineral-and urolithiasis-related renal complications in HPT/MEN1 are early-onset, frequent, extensive, severe, and progressive. These data should be considered in the individualized clinical/surgical management of patients with MEN1-associated HPT. ß

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Section: Discussionmentioning

confidence: 99%

Section: Biochemical Measurementsmentioning

confidence: 99%

mentioning

confidence: 99%

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Early-onset, progressive, frequent, extensive, and severe bone mineral and renal complications in multiple endocrine neoplasia type 1–associated primary hyperparathyroidism

Lourenço

Coutinho

Toledo

et al. 2010

Journal of Bone and Mineral Research

102

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“…When we tested it for value, the 6Q panel was highly effective compared to family history. 16 MEN-1 was increasingly likely with each positive 6Q answer, and also with young age and male sex, which makes sense because MEN-1 is autosomal-dominant, whereas sporadic hyperparathyroidism is much more common in women. Moreover, 22% of patients with false-positive 6Q responses actually had jaw-tumor syndrome, which---candidate members---sets you up for doing the next study.…”

Section: You Can Evolve Endocrine Surgery Without a Pipettementioning

confidence: 99%

“…The 6 questions for MEN-1 are: Do you have a blood relative with kidney stones, ulcers, brain tumors, pancreatic tumors, high calcium levels, or neck surgery? 16 …”

Section: You Can Evolve Endocrine Surgery Without a Pipettementioning

confidence: 99%