Zollinger-Ellison syndrome associated with a history of alcohol abuse: coincidence or consequence?

BACKGROUND: This 47-year observational study suggests that sporadic Zollinger-Ellison (Z-E) syndrome, particularly duodenal wall gastrinomas (DWG), is associated with a history of alcohol abuse. METHODS: Thirty-nine consecutive Z-E patients were followed from 1962 through 2010. The drinking patterns of these patients were assessed and compared with 3,786 community controls. RESULTS: Thirty-five patients had extrapancreatic gastrinomas (34 DWG and/or paraduodenal lymph nodes, 1 antral gastrinoma). Total gastrectomy was done in 24; 9 underwent less extensive operations to remove DWG, and 2 patients had no operations. There were no deaths from tumor progression. Four patients presented with pancreatic gastrinoma (PG) and liver metastasis, all died from tumor progression. Alcohol abuse (>50 g/d) was documented in 81% of patients with DWG and/or paraduodenal lymph nodes. The drinking patterns (drinks per day) of DWG patients were significantly different: DWG vs community control-abstainers, 3% vs 24%; 1-2 drinks, 16% vs 62%; 3-5 drinks, 29% vs 12%; and ≥ 6 drinks, 52% vs 2.5% (P < .01). CONCLUSION: Alcohol abuse is strongly associated with and may be a risk factor for sporadic Z-E with extrapancreatic DWG. Liver metastases and tumor deaths were not observed in this subgroup, supporting the concept that DWG and PG are different tumor entities.

Primary hyperparathyroidism with a history of head and neck irradiation: the consequences of associated thyroid tumors.

BACKGROUND: Information on thyroid tumors is scant in patients with primary hyperparathyroidism (HPT) and history of head and neck irradiation. The study objective was to investigate thyroid pathology in primary HPT patients with irradiation history presenting for parathyroidectomy. METHODS: A prospective database of 1,020 parathyroidectomy patients was analyzed. 916 consecutive HPT patients were identified. History of radiation, neck ultrasound results, thyroid operations, and pathology was assessed. Patients with radiation history were compared to those with no radiation. RESULTS: Of the 916 HPT patients, 49 (5%) had a history of radiation and were more likely to have nodular thyroid disease (95% vs 52%), undergone a prior thyroidectomy (29% vs 4%), or had concurrent thyroidectomy (49% vs 26%). Nine of 49 (24%) had thyroid cancer. Of the 867 patients with no history of radiation, 259 underwent thyroid resection (32 prior and 227 concurrent) and 32 (12%) had thyroid cancer. CONCLUSION: Primary HPT patients with head and neck irradiation presenting for parathyroidectomy had marked increase in nodular thyroid disease: nearly 1 in 2 had concurrent thyroidectomy, and nearly 1 in 4 had thyroid carcinoma. High resolution ultrasound prior to parathyroidectomy detects associated thyroid pathology and allows the surgeon to plan the extent of thyroid resection.

Reoperative parathyroidectomy: an algorithm for imaging and monitoring of intraoperative parathyroid hormone levels that results in a successful focused approach.

BACKGROUND: Advances in preoperative imaging and use of intraoperative parathyroid hormone (IOPTH) levels are changing the approach to reoperative parathyroidectomy (ReopPTX). We sought to develop a protocol for imaging and IOPTH monitoring that allows for a focused, successful operative approach. METHODS: We reviewed our prospective database of consecutive patients with primary hyperparathyroidism who underwent ReopPTX with IOPTH monitoring between December 1999 and June 2007. RESULTS: Thirty-nine patients underwent 43 ReopPTXs for persistent (79%)/recurrent (21%) disease. All underwent ultrasonography and sestamibi imaging; 24 cases (56%) underwent additional imaging studies. Sensitivity of ultrasonography was 56%, sestamibi 53%, both studies 67%, computed tomography (CT) 48%, magnetic resonance imaging (MRI) 67%, and selective venous sampling (SVS) 50%. IOPTH monitoring predicted accurately cure in 100% and failure in 78%. A focused/unilateral approach was performed in 60%; median operative time was 45 minutes (range, 12-127). At last follow-up, 36 (92%) patients were normocalcemic. CONCLUSIONS: We propose that ultrasonography and sestamibi studies should be done before all ReopPTXs; failure to localize should prompt sequential CT, MRI, and SVS until localization is achieved. IOPTH monitoring defines cure and is recommended for all ReopPTXs. This algorithm allows for a focused operative approach in >50% of ReopPTXs with operative times comparable with first-time, minimally invasive parathyroidectomy.

The influence of surgery in MEN-1 syndrome: observations over 150 years.

BACKGROUND: Efficacy and timing of operative intervention in patients with multiple endocrine neoplasia type 1 (MEN-1) syndrome remains controversial. This report utilizes a novel approach to evaluate the influence of evolving operative interventions for patients with MEN-1 syndrome. METHODS: Six generations from a large MEN-1 family pedigree were studied. The number of operations for MEN-1 related pathology was recorded according to birth eras over 150 years. Length of life was a primary outcome measurement. RESULTS: Inheritance of the MEN-1 trait was near 50%. There were no instances of a skipped generation. Affected individuals born before 1900 died from gastrointestinal hemorrhage and without any surgical intervention. After 1900, there were increasing numbers of gastric, parathyroid, and pancreatic operations in successive eras. Death occurred >20 years earlier in MEN-1 individuals than unaffected family members in eras 1 and 2. Family members with MEN-1 lived longer in succeeding eras with increasing number of operative and pharmacologic interventions. CONCLUSION: MEN-1 family members invariably have pathologic changes in pituitary, parathyroid, and pancreatic islets when long lived, the "all-or-none" phenomenon. Patients are not cured with operative interventions, although they may live longer and without symptoms with a good quality of life. This model may allow better comparisons with other MEN-1 patients when evaluating outcomes of new medical and operative management schemes and long-term follow-up.

The role of parathyroid hormone measurements after surgery for primary hyperparathyroidism.

BACKGROUND: During parathyroidectomy for primary hyperparathyroidism (pHPT), intraoperative parathyroid hormone (IOPTH) levels are used to confirm removal of all hyperfunctioning parathyroid tissue. The phenomenon of elevated parathyroid hormone (PTH) levels with normocalcemia after curative parathyroidectomy, seen in up to 40% of patients, continues to be an unexpected and unexplained finding. We therefore investigated whether postoperative PTH levels are as reliable as IOPTH levels in predicting cure after surgery for pHPT. METHODS: We reviewed our prospective database of consecutive patients undergoing surgery for pHPT between December 1999 and November 2004. Curative parathyroidectomy was defined as normocalcemia 6 months or longer postoperatively. RESULTS: A total of 328 patients who underwent 330 operations for pHPT had IOPTH measurements and serum follow-up calcium levels at 6 months or longer. Surgery was curative in 315 (95.5%) operations. IOPTH levels correctly predicted operative success in 98.2% (positive predictive value [PPV]. Postoperatively, the PPV of a normal PTH level at 1 week, 3 months, and 6 months was 97.1%, 97.3%, and 96.5%, respectively. Of all patients with an elevated postoperative PTH level at 1 week, 3 months, or 6 months, only 13.7%, 14.3%, and 14%, respectively, were not cured. CONCLUSIONS: Normal postoperative PTH levels reliably predict operative success. However, they do not improve upon results predicted by IOPTH levels. Elevated postoperative PTH levels do not predict operative failure in most patients. We propose that PTH measurements after surgery for pHPT may be misleading, costly, and not indicated in normocalcemic patients.

Twenty-five years of advances in vascular access: bridging research to clinical practice.

Vascular access has become a key component for a multitude of IV therapies, including parenteral nutrition. Access of the central venous system has been long recognized for its associated complications of infection, thrombosis, and occlusion. Over the past 25 years, clinical practice based on research and innovation has attempted to decrease complication rates and therefore improve the safety of vascular access. This article highlights the research and its influence on catheter care procedures, technology, and education that has led to advances in vascular access. An improved understanding of the pathophysiology associated with catheter-related complications and an ongoing evaluation of new treatment modalities has provided clinicians today with new options for improved patient care and the ability to preserve vascular access options for patients.

Detection of multiple gland primary hyperparathyroidism in the era of minimally invasive parathyroidectomy.

BACKGROUND: A focused surgical approach for primary hyperparathyroidism relies on the ability of preoperative imaging and intraoperative parathyroid hormone monitoring (IOPTH) to detect multiple gland disease (MGD). The study objective was to determine the best predictor for MGD. METHODS: First time parathyroidectomy was performed on 233 patients with primary hyperparathyroidism who underwent preoperative sestamibi imaging, ultrasound, and IOPTH between December 1999 and January 2004. RESULTS: Single gland disease (SGD) was found in 204 (88%) and MGD in 23 (10%) patients. Hyperparathyroidism persisted in 6 of 233 patients (2.6%). For patients with MGD, sestamibi imaging correctly predicted MGD in 2 of 23 (9%) patients, incorrectly showed SGD in 9 of 23 (39%), and was negative in 12 of 23 (52%). Ultrasound correctly predicted MGD in 6 of 23 (26%) patients, incorrectly predicted SGD in 6 of 23 (39%), and was negative in 8 of 23 (35%). Together sestamibi imaging and ultrasound predicted MGD in 7 of 23 (30%) patients, incorrectly predicted SGD in 7 of 23 (30%), was negative in 7 of 23 (30%), and was discordant in 10 of 23 (5%). IOPTH indicated MGD in 15 of 18 (83%) patients but falsely predicted cure after single gland excision in 3 of 18 (17%). The combination of sestamibi imaging, ultrasound, and IOPTH detected MGD in 16 of 18 (89%) patients. CONCLUSION: Ultrasound was more sensitive for detecting MGD than sestamibi imaging. Ultrasound and sestamibi imaging together provided information warranting a bilateral approach in 70% of patients with MGD. IOPTH was the most sensitive for MGD, but combining all 3 tests was the best predictor, identifying the majority of patients with MGD.