bluuebunny/crossref_metadata_2025_split huggingface dataset:
Surgery versus surveillance of pancreaticoduodenal endocrine tumours in multiple endocrine neoplasia type 1
Authors: Bartsch D K , Langer P , Schilling T , Wild A , Celik I , Nies C , Rothmund M 
Published: March 2003

Abstract

Background
Management of pancreaticoduodenal endocrine tumours remains controversial in multiple endocrine neoplasia type 1 (MEN1). The authors' experience with MEN1-related pancreaticoduodenal tumours has been reviewed in order to evaluate a rational therapeutic approach.

Methods
Twenty-one patients with MEN1 with pancreaticoduodenal endocrine tumours were analysed with special regard to outcome of surgery and surveillance. In addition, a phenotype/genotype analysis was performed based on MEN1 gene mutation analysis.

Results
Twelve of 21 patients had multiple tumours. Nine patients had gastrinomas, six non-functioning tumours, three insulinomas, two insulinomas and gastrinomas, and one had a vipoma. Six tumours were malignant with lymph node metastases (five gastrinomas, one vipoma). Seventeen patients were treated initially by either pancreatic left resection with tumour enucleation of the duodenum and/or pancreatic head (n = 9), tumour enucleation alone (n = 7) or pylorus-preserving partial pancreatectomy (PPPD; n = 1). Sixteen of 17 patients were cured biochemically after initial surgery and 11 remained free of disease after a median follow-up of 55 (range 2–197) months. Six patients underwent reoperation for recurrence and/or lymph node metastases, including two PPPDs in patients with gastrinoma. All six patients remained asymptomatic and three remained biochemically free of disease. Three of the 17 operated patients died from either unrelated causes (n = 2) or postoperative complications (n = 1). On the other hand, four asymptomatic patients with either gastrinomas (n = 2) or non-functioning tumours (n = 2) were closely surveyed and showed no progress or metastases on somatostatin (SMS) scans and computed tomography after 9–110 months. The genotype/phenotype analysis revealed that patients with truncating nonsense or frameshift MEN1 gene mutations in the N- or C-terminal regions (exons 2, 9 or 10) had a significantly higher rate of malignant tumours (55 versus 8 per cent; P = 0·04) and tended to have shorter disease-free intervals (28 versus 120 months; P = 0·11) than patients with other mutations.

Conclusion
An aggressive surgical approach is justified for pancreaticoduodenal tumours in patients with MEN1 since symptom-free long-term survival can be achieved. However, MEN1 gene mutations in exons 3–8 seem to be associated with mild behaviour of pancreaticoduodenal tumours which might allow close surveillance in asymptomatic patients with MEN1.
Normal preoperative levels of parathyroid hormone signify a mild form of primary hyperparathyroidism
Authors: Bergenfelz A , Lindblom P , Westerdahl J 
Published: March 2003

Abstract

Background
Primary hyperparathyroidism (pHPT) is characterized by an inappropriate serum level of parathyroid hormone (PTH) in relation to the serum level of calcium. Nowadays, patients often are diagnosed with an overtly asymptomatic pHPT with mild or even intermittent hypercalcaemia. Indeed, health screening surveys have shown that a significant proportion of patients with pHPT is normocalcaemic. Most studies also demonstrate patients with PTH levels in the upper normal range. The aim of the present study was to define subgroups of patients with mild pHPT, which potentially could give valuable insights into the pathogenesis of the disease.

Methods
Two hundred and seven consecutive patients, 157 women and 50 men, operated for pHPT were investigated with biochemical variables known to reflect PTH activity and calcium metabolism, and bone mineral content. The preoperative diagnosis of pHPT was based on the presence of hypercalcaemia combined with an inappropriate serum concentration of PTH. Thus, no patient was persistently normocalcaemic. Mean(s.d.) age of the patients was 64(14) years and mean(s.d.) serum level of calcium was 2·78(0·19) mmol l−1. On the basis of serum levels of PTH and calcium the day before surgery, patients were divided into subgroups.

Results
One hundred and sixty-two patients (78 per cent) had raised levels of PTH and calcium the day before surgery (group 1), 25 patients (12 per cent) had a raised level of PTH and normal level of calcium (intermittent normocalcaemia) (group 2) and 20 patients (10 per cent) had a normal PTH level and raised calcium level (group 3). Between-group analyses demonstrated differences between groups in age, bone density, renal function and adenoma weight, and serum levels of alkaline phosphate, osteocalcin and urate. Except for lower adenoma weights, patients in group 2 did not essentially differ from those in group 1. However, patients in group 3 were younger, had better renal function, lower biochemical bone turnover and higher bone mineral content than patients in the other two groups.

Conclusion
These data support the existence of subgroups of mild pHPT. Patients with a serum level of PTH within the normal range were characterized by less biochemical derangement and better bone mineral content. This finding suggests that PTH rather than calcium reflects the severity of the disease.
Endoscopic endocrine neck surgery with carbon dioxide insufflation: the effect on intracranial pressure
Authors: Rubino F , Pamoukian V N , Zhu J F , Deutsch H , Inabnet W B , Gagner M 
Published: March 2003

Abstract

Background
The endoscopic approach to the thyroid and parathyroid glands requires insufflation with carbon dioxide at 10–15 mmHg. This may decrease the cerebral venous return and increase intracranial pressure (ICP). The aim of this study was to evaluate the effect of carbon dioxide neck insufflation on ICP and haemodynamic variables.

Methods
Twelve 25-kg pigs underwent endoscopic thyroid dissection using carbon dioxide insufflation at 0 mmHg (sham), 10 mmHg (G10), 15 mmHg (G15) and 20 mmHg (G20). Three pigs were used in each group. ICP, mean arterial pressure, central venous blood pressure (CVP), cardiac output and blood gas were measured at baseline, 30, 60 and 120 min.

Results
There were no differences in mean ICP between the sham group and G10. However, ICP significantly increased in G15 and G20. The partial pressure of carbon dioxide was similar in G10 and G15 (mean(s.d.) 49·8(2·4) and 49·8(5·2) mmHg respectively at 120 min), while it was dramatically increased in G20 (63·1(6·9) mmHg at 120 min). Haemodynamic parameters were stable in all groups except for G20 in which CVP significantly increased with respect to baseline (8·5(2·1) versus 1·3(1·5) mmHg; P &lt; 0·01). Jugular vein collapse occurred during endoscopic dissection at all insufflation pressures, but pigs operated at 10 mmHg were able to maintain an intermittent blood flow.

Conclusion
A severe increase in ICP occurs with insufflation pressures higher than 15 mmHg, possibly owing to decreased cervical venous blood flow. Carbon dioxide insufflation up to 10 mmHg does not alter ICP, and is recommended for clinical application in endoscopic endocrine neck surgery.
Management of incidentally discovered adrenal masses and risk of malignancy
Authors: Favia G , Lumachi F , Basso S , D'Amico D 
Published: March 2003

Abstract

Background
Incidentally discovered adrenal masses larger than 1 cm are relatively frequent, but the correct management of such lesions is not well established.

Methods
Between 1980 and 1999, 158 patients (73 men (46 per cent) and 85 women (54 per cent), of median age 53 years) with adrenal incidentalomas larger than 2 cm were observed. Sixty-eight patients (43 per cent) underwent adrenalectomy. The reasons for surgery were: (1) suspicious appearance on computed tomography (CT) or magnetic resonance imaging (MRI) or no uptake at 75-selenium-norcholesterol scintigraphy (NCS) (n = 22), (2) increase in size of more than 1 cm (n = 15), (3) subclinical endocrine hyperfunction (n = 14), (4) size greater than 5 cm (n = 12) and (5) suspicious imaging-guided fine-needle aspiration cytology (FNAC) (n = 5).

Results
Pathological examination showed 39 adrenocortical adenomas (57 per cent), 20 adrenal malignancies (29 per cent) (15 carcinomas, three unsuspected metastases and two non-functioning malignant phaeochromocytomas) and nine various benign lesions (13 per cent). All masses increased in size were benign. Seven malignant tumours were 3–4 cm and 14 benign lesions were 5–6 cm in size. Sensitivity and specificity in detection of malignancy were 100 and 100 per cent for NCS (n = 34) and FNAC (n = 9), 75 and 94 per cent for CT (n = 68), and 88 and 100 per cent for MRI (n = 26) respectively.

Conclusion
To differentiate benign and malignant incidentalomas NCS and FNAC are more sensitive than CT and MRI, while the size criteria are of little value.
Ultrasonography and sestamibi as the only preoperative imaging tests in reoperation for parathyroid adenoma
Authors: Feingold D , Alexander H R , Chen C , Libutti S K , Shawker T , Simonds W , Spiegel A , Marx S , Skarulis M C , Doppman J L , Bartlett D L 
Published: March 2003

Abstract

Background
Various non-invasive and invasive tests are used for the localization of parathyroid adenomas in the reoperative setting. To help determine the most efficient algorithm for the evaluation of these patients, use of a combination of ultrasonography and sestamibi as the only preoperative imaging tests was explored.

Methods
This was an analysis of outcomes of 62 consecutive patients (21 men; mean age 55 years) treated between January 1995 and May 1999, and referred for persistent hyperparathyroidism after one (n = 47) or more (n = 15) previous explorations at which no abnormal parathyroid glands were found. While all patients underwent ultrasonography (all interpreted by one radiologist), computed tomography (CT), magnetic resonance imaging and sestamibi, the success of localization and reoperation was analysed using only the results of ultrasonography and sestamibi. Intraoperative ultrasonography (IOUS) was available in all cases.

Results
Sixty-one patients (98 per cent) had curative reoperations with solitary adenomas confirmed pathologically. One adenoma was not found at operation. Forty-six adenomas (74 per cent) were ectopic including 14 in the tracheo-oesophageal groove and 12 in the anterior mediastinum. The sensitivity, positive predictive value and accuracy for ultrasonography were 91, 87 and 84 per cent respectively; the corresponding values for sestamibi were 75, 94 and 71 per cent. In 58 (94 per cent) of 62 patients preoperative ultrasonography and/or sestamibi accurately identified the adenoma. In three patients cured by operation, in whom combined ultrasonography and sestamibi was inaccurate, one gland was found by IOUS in strap muscle, one by blind cervical thymectomy and one by sternotomy based on CT findings. Thirty-seven patients (60 per cent) underwent IOUS. In nine of these patients IOUS was essential in guiding resection of the adenomas.

Conclusion
This study supports an algorithm of obtaining ultrasonography and sestamibi as the initial and perhaps only preoperative localization tests for patients with persistent hyperparathyroidism after failed operation at which no abnormal glands were found. Using this algorithm, reoperation should be successful in almost 95 per cent of cases.
Normalization of intraoperative parathyroid hormone does not predict normal postoperative parathyroid function
Authors: Starr F , Betlej T , DeCresce R , Prinz R 
Published: March 2003

Abstract

Background
Intraoperative measurement of intact parathyroid hormone (iPTH) has been used increasingly as a means of confirming complete removal of hyperfunctioning parathyroid tissue. It remains uncertain, however, whether normalization of iPTH levels at operation accurately predicts postoperative values of iPTH.

Methods
Forty-five consecutive patients with primary hyperparathyroidism underwent parathyroidectomy between March and November 1999. Baseline intraoperative iPTH levels were measured before and at manipulation, then subsequently at 5 and 10 min after tissue was removed. Follow-up values were measured at 1- and 3-month intervals.

Results
Before operation the mean(s.d.) iPTH concentration was 211(182) pg ml−1 with a mean serum calcium level of 11·4(1·0) mg dl−1. In all but four patients, the final intraoperative iPTH normalized to absolute values below 60 pg ml−1 (mean 38(36) pg ml−1). One week after operation, serum calcium levels had returned to normal (mean 9·2(0·9) mg dl−1) and this correlated directly with final intraoperative iPTH values when assessed by linear regression analysis (r = 0·373, P &lt; 0·03). By 1 month, all but two patients were normocalcaemic (mean 9·6(0·6) mg dl−1) with a mean iPTH of 81(99) pg ml−1. There was no correlation between the final intraoperative iPTH and postoperative iPTH values as determined by linear regression analysis (r = 0·035, P &lt; 0·881). Both patients with persistent hypercalcaemia at 1 month had appropriate intraoperative decreases in iPTH level.

Conclusion
Although there is significant correlation between intraoperative iPTH levels and postoperative serum calcium levels, this does not hold true for postoperative iPTH levels. There was a 4 per cent failure rate in correcting postoperative calcium levels and a 20 per cent failure rate in normalizing postoperative iPTH levels despite appropriate intraoperative decreases in iPTH.
Clinical and genetic analysis of patients with pancreatic neuroendocrine tumours associated with von Hippel-Lindau disease
Authors: Libutti S K , Linehan W M , Choyke P L , Glenn G , Zbar B , Klausner R D , Alexander H R , Bartlett D L , Lubensky I , Walther McC 
Published: March 2003

Abstract

Background
Patients with von Hippel-Lindau disease (VHL) are at risk of developing pancreatic cysts, microcystic adenomas and neuroendocrine tumours. Pancreatic neuroendocrine tumours (PNETs) are found in 19 per cent of families with VHL. These tumours can behave in a malignant fashion, with up to 17 per cent of patients developing distant disease. The authors' initial experience with the diagnosis and management of these lesions, with resection based on size greater than 3 cm in imaging studies, has been reported previously. This is a report on their prospective experience using these criteria as well as on the role of genotype/phenotype analysis of germline VHL gene mutations in predicting clinical course.

Methods
Some 389 patients with VHL were screened between December 1998 and December 1999. The diagnosis of PNET was made by either pathological analysis of tissues or by characteristic radiographic appearance on computed tomography and magnetic resonance imaging. VHL gene germline mutations were determined by quantitative Southern blotting to detect deletions of the gene, Southern blotting to detect gene rearrangements, fluorescence in situ hybridization to confirm deletions and complete gene sequencing.

Results
Forty-four patients with PNET have been identified. Of these, 25 have undergone definitive surgical resection, five were found to have metastatic disease and 14 are being followed prospectively. No patient who has undergone resection based on the aforementioned tumour size criteria has developed metastatic disease. These patients represent 36 families (19 per cent) of a total of 188 VHL families who have had germline mutation analysis. Compared with all 188 VHL families, those with PNETs are more likely to have missense mutations (58 per cent) and more likely to have intragenic mutations. Four of five patients with metastatic disease have mutations in exon 3 compared with 18 of 39 patients without metastatic disease.

Conclusion
An aggressive approach to imaging for detection and surgical resection based on established size criteria has resulted in the successful management of patients with VHL and PNETs. Analysis of germline mutations may help predict those patients with VHL who are at risk of developing a PNET and which patients with a PNET may benefit from earlier surgical intervention to prevent spread of disease.
The ‘false’ non-recurrent inferior laryngeal nerve
Authors: Rafaelli M , Henry J F 
Published: March 2003

Abstract

Background
A communication between the middle cervical sympathetic ganglion (MCSG) and the inferior laryngeal nerve (ILN) has been described. The anastomotic branch (sympathetic–inferior laryngeal anastomotic branch; SILAB) is usually thin, but is sometimes larger and has the same diameter as the ILN. The purpose of this study was to evaluate prospectively the frequency of this condition and its implications during neck exploration.

Methods
From November 1998 to October 1999, 791 neck explorations were performed: 677 for thyroid, 99 for parathyroid and 15 for concomitant lesions. Some 1253 ILNs were dissected: 656 (52·3 per cent) on the right and 597 (47·7 per cent) on the left side.

Results
The ILN was identified in all cases. On the right side a non-recurrent ILN (NRILN) was found in three patients (0·5 per cent) and a large SILAB in ten (1·5 per cent). No anomalous branch was found on the left side. The SILAB originated from the superior cervical sympathetic ganglion (SCSG) in two patients and directly from the sympathetic chain (SC) above the MCSG in eight. No branch originating from the MCSG was found. The SILAB connected with the ILN less than 2 cm from the cricoid in all patients.

Conclusion
The SILAB may originate not only from the MCSG but also from the SCSG and directly from the SC. When the SILAB is as large as the ILN, it could be mistaken for a NRILN. Before concluding that the anomalous branch is a NRILN, one should check if it originates from the vagus or from the cervical sympathetic system. Awareness of this anatomical condition during neck exploration may help the surgeon to avoid injuries of an ILN running in the usual pathway.
Long-term prognosis of children with papillary thyroid cancer presenting with pulmonary metastases
Authors: Brink J , van Heerden J , Farley D , McIver B , Thompson G B , Grant C S , Zimmerman D 
Published: March 2003

Abstract

Background
Papillary thyroid cancer (PTC) has a generally favourable prognosis, but elderly patients with distant metastases or extracapsular invasion fair poorly. In small studies with short follow-up, young patients presenting with such extracapsular invasion and pulmonary metastases have faired well. This retrospective study was undertaken to clarify the long-term prognosis of such patients with advanced PTC.

Methods
Twenty-one children and young adults (median age 14 (range 6–20) years) presenting with PTC and pulmonary metastases were treated at a single institution between 1947 and 1998. Mean maximal tumour diameter was 4·65 cm. Initial surgical treatment consisted of total thyroidectomy (n = 16), subtotal thyroidectomy (n = 4) and isthmectomy (n = 1), coupled with a variety of lymph node dissections (n = 20). After operation, 19 patients were treated with ablative and incremental doses of iodine-131 until disease free. All patients were placed on suppressive thyroid hormone after operation. Mean length of follow-up was 21 years (range 3 months to 47 years). Follow-up was less than 3 years in four patients. All patients have undergone post-treatment radionucleotide and radiological evaluation.

Results
Nine of the 21 patients developed recurrent disease. The risk of recurrence at 5 years was 39 (95 per cent confidence interval 14–57) per cent. Eight had cervical lymph node recurrence and no patient developed recurrent pulmonary disease. All patients with identifiable recurrent disease underwent selective lymph node resection, which involved multiple resections in four. At follow-up, 18 patients remain completely free of disease, one patient has recurrent cervical node disease and two patients have died. The disease-free survival at 5 years was 95 (95 per cent confidence interval 86–100) per cent. Cause-specific death occurred in a single patient who died from extensive local disease at age 29 years after 12 years of multiple cervical lymph node recurrences.

Conclusion
A stepwise treatment approach including total thyroidectomy, high-dose iodine-131 treatment and early surgical reintervention for suspected local recurrent disease allows long-term survival and frequent ‘cure’ for young patients with PTC and concomitant pulmonary metastases.
The national liver transplantation audit: an overview of patients presenting for liver transplantation from 1994 to 1998
Authors: Hartley P , Petruckevitch A , Reeves B , Rolles K 
Published: March 2003

Abstract

Background
The aim of this study was to describe current clinical practice in liver transplantation in the UK and Ireland, to provide overall 1-year graft and patient survival rates, and to study some preoperative risk factors.

Methods
All patients receiving a liver transplant in the UK or Ireland between 1 March 1994 and 30 September 1998 were included. Data were collected on patients at the time of transplantation, 3 months after grafting and annually thereafter until the patient's death. The main outcome measures were graft and patient survival at 1 year.

Results
A total of 3102 liver transplants were carried out, of which 87 per cent were first transplants. The mean age at first transplantation was 42 (range 0–76) years. The most common indications for transplantation were primary biliary cirrhosis, alcoholic cirrhosis and posthepatitis C cirrhosis, but variations existed between sexes and centres. Risk factors associated with lower graft and patient survival were the presence of acute disease, being transplanted from hospital, and the need for renal and/or ventilatory support before operation.

Conclusion
Donor and recipient demographics are consistent with data held by the European Liver Transplant Registry, as are 1-year graft and patient survival rates. Variation across centres in factors such as the primary indication for liver transplantation, population demographics, the clinical status of each patient, incidence of retransplantation and other risk factors contributes to the problem of adjusting for case mix.
Experimental models of Gram-negative sepsis
Authors: Parker S J , Watkins P E 
Published: March 2003

Abstract

Background
The mortality rate from sepsis has improved little over the past two decades. One reason for this has been the use of flawed or inappropriate experimental models in preclinical sepsis studies.

Methods
A literature review of animal models of sepsis was performed following a Medline search based on the following medical subject headings: disease models, endotoxin, inflammation, peritonitis and sepsis. Additional references were identified from the papers identified in the search.

Results and conclusion
Many animal models of sepsis have been described but none has proved to be superior. Extrapolation of results from endotoxicosis or bacterial infusion models should be regarded with caution. Peritonitis models should be accepted as the ‘gold standard’ but the use of appropriate virulent bacterial species needs to be ensured. A standardized panel of animal models for the preclinical assessment of immunomodulatory agents should be established, including at least one immuno- suppressed model to simulate the immunocompromised patient with sepsis. A uniform and valid definition of sepsis applicable to both small and large animal species is required.