Necessity for long-term follow-up of patients with head and neck paraganglioma and mutation in the succinate dehydrogenase genes: an index case report and literature review.

Objective: To describe a patient with hereditary head and neck paraganglioma (HNPGL) and to review the literature on these rare tumors.

Methods: We review the English-language literature regarding SDH mutations, HNPGL, hereditary paraganglioma-pheochromocytoma syndrome, and the role of functional imaging in the follow-up of these tumors. We also describe the clinical findings, imaging results, and follow-up of a man who initially presented with HNPGL and subsequently developed metastatic pheochromocytoma 20 years later.

Development and intra-institutional and inter-institutional validation of a comprehensive new hepatobiliary software: Part 1--Liver and gallbladder function.

Objective: To develop a software tool for quantification of liver and gallbladder function, and to assess the repeatability and reproducibility of measurements made with it.

Materials And Methods: The software tool developed with the JAVA programming language uses the JAVA2 Standard Edition framework. After manual selection of the regions of interest on a 99mTc hepatic iminodiacetic acid study, the program calculates differential hepatic bile flow, basal duodeno-gastric bile reflux (B-DGBR), hepatic extraction fraction (HEF) of both the lobes with deconvolutional analysis and excretion half-time with nonlinear least squares fit.

Hereditary fibrinogen A alpha-chain amyloidosis: phenotypic characterization of a systemic disease and the role of liver transplantation.

Variants of fibrinogen A alpha-chain (AFib) cause the most common type of hereditary renal amyloidosis in Europe and, possibly, the United States as well. Variant fibrinogen is produced in the liver, and solitary renal allografts fail within 1 to 7 years with recurrent amyloidosis. We assessed 22 AFib patients for combined liver and kidney transplantation (LKT) and report the clinical features and outcome.

Midgut neuroendocrine tumours with liver metastases: results of the UKINETS study.

We intended to identify the prognostic factors and the results of interventions on patients with liver metastatic midgut carcinoids. Five institutions that are part of United Kingdom and Ireland neuroendocrine tumour (NET) group took part in this study. Patients were included if they had histology proven NET of midgut origin and liver metastases at the time of the study.

Hepatobiliary scintigraphy after Kasai procedure for biliary atresia: clinical correlation and prognostic value.

Background/purpose: Kasai portoenterostomy (KP) is regarded as first-line treatment for biliary atresia, although its postoperative course is often unpredictable. Hepatobiliary scintigraphy using technetium-labeled iminodiacetic acid derivatives offers a dynamic, objective assessment both of parenchymal liver function and restored biliary excretion. The value of postoperative radionuclide scans was assessed prospectively in a large population of post-KP infants.

A Monte Carlo analysis technique applied to cerebral perfusion SPECT scans.

Objective: Voxel-based techniques are used to provide objective analyses of SPECT scans. The aim of this study was to develop a voxel-based analysis technique that uses a Monte Carlo method of statistical inference and assess this analysis technique for application to cerebral perfusion SPECT scans.

Methods: Assessment of the validity of this non-parametric, Monte Carlo method of statistical inference has been performed for a range of study designs, image characteristics and analysis parameters using phantom SPECT and Gaussian images.

Cystatin C, an easy and reliable marker for assessment of renal dysfunction in children with liver disease and after liver transplantation.

Renal dysfunction of variable severity is being increasingly recognized as a major complication of calcineurin inhibitors (CI), in some patients even necessitating renal transplantation. Close and effective monitoring of the renal function is indicated. Current methods for this monitoring are calculation of the glomerular filtration rate (GFR) based on creatinine or exogenous substances like 51Cr-EDTA.

Long duration asymmetrical postural tremor is likely to predict development of Parkinson's disease and not essential tremor: clinical follow up study of 13 cases.

Background: Patients presenting with asymmetrical postural tremor with or without mild rest tremor may be diagnosed as having essential tremor (ET), although there is considerable diagnostic uncertainty as to the long term outcome of these patients.

Objective: In this study, retrospective observations were made on 13 patients presenting originally with asymmetrical postural tremor, initially thought to have ET based on tremor characteristics, alcohol responsiveness, and family history but who subsequently met the criteria of Parkinson's disease (PD).

Methods: The patients were observed and followed up clinically with ancillary imaging using dopamine transporter SPECT scan or levodopa challenge tests in some cases.

Bilateral orbital metastases from a neuroendocrine tumor.

When neuroendocrine tumors metastasize to the orbit, they usually do so as solitary lesions, sometimes involving an extraocular muscle. A 70-year-old woman with a known neuroendocrine tumor had bilateral painful proptosis, orbital soft tissue swelling, and ophthalmoplegia. Imaging showed masses within all the extraocular muscles.

Transdermal hormone replacement therapy improves vertebral bone density in primary biliary cirrhosis: results of a 1-year controlled trial.

Background: Retrospective studies have suggested that hormone replacement therapy may reduce the rate of bone loss in primary biliary cirrhosis, but no controlled data are available.

Methods: Forty-two post-menopausal women with primary biliary cirrhosis were treated with calcium and vitamin D, either alone (n = 21) or together with transdermal hormone replacement therapy (n = 21). Bone densitometry was performed at baseline and at 1 year, and serum and urinary markers of bone turnover were measured at three-monthly intervals.

Long-term outcome after gastrectomy for intractable diabetic gastroparesis.

Aims: To examine the long-term outlook for patients with intractable vomiting from diabetic gastroparesis who underwent major gastric surgery.

Methods: Of 18 patients with problems from vomiting referred to the King's Diabetes Centre during the years 1994-2000, seven were considered to suffer irreversible symptoms not alleviated by protracted periods of medical treatment. They were all Type 1 Caucasian diabetic women, mean age 32 years (range 28-37 years) with multiple symptoms of severe autonomic neuropathy.

Diurnal, week-to-week, and long-term variation in urine deoxypyridinoline cross-link excretion in healthy older women.

Objectives: To establish a reference range for morning and afternoon excretion of urinary deoxypyridinoline (DPD) in apparently healthy older women selected from a volunteer database. To assess the extent of diurnal variation and short and long-term within-subject longitudinal variation.

Design: Prospective, observational, cohort study.

Bone mineral density and height gain in children with chronic cholestatic liver disease undergoing transplantation.

Background: Osteodystrophy is a well-described complication of chronic liver disease. Previous reports in adults and children undergoing liver transplantation (LT) were discordant, with the former showing no improvement of bone disease in the first year after transplantation and the latter demonstrating remarkable benefit from it. Our aim was to perform a pilot study on osteodystrophy in children undergoing LT and evaluate the contribution of growth on bone mineral density (BMD) changes.

Cushing's syndrome associated with a chemodectoma and a carcinoid tumour.

We present a case of Cushing's syndrome where 111In-octreotide scanning provided evidence for the presence of two neuroendocrine tumours. Uptake in the right neck corresponded to a chemodectoma, but there was no change in the clinical condition or fall in ACTH levels following surgical resection. Uptake in the left chest was assumed to relate to a bronchial carcinoid, but a tumour could not initially be localized on magnetic resonance imaging (MRI), spiral CT scanning or on selective venous sampling.

Quantitative perfusion imaging in carotid artery stenosis using dynamic susceptibility contrast-enhanced magnetic resonance imaging.

Quantitative, multislice dynamic susceptibility contrast-enhanced MRI perfusion measurements were used to determine the patterns of cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time (MTT), and normalized first moment of the tissue deltaR2-time curve (N) in 11 subjects with carotid artery occlusion or stenosis. MTT correlated with degree of carotid stenosis, whereas a range of alterations in CBF and CBV were found presumably reflecting variables degrees of collateral flow. There was no significant correlation between MRI and SPET flow perfusion measurements, with increasing disparity between the two techniques at higher inter-hemispheric flow ratios.

Novel surgical treatment and gastric pathology in diabetic gastroparesis.

Aims: Observations are made on four Type 1 diabetic patients with the rare syndrome of intractable vomiting from confirmed gastroparesis, to determine whether radical surgery would alleviate their symptoms and subsequently to examine in detail the gastric histopathology.

Methods: The surgical approach consisted of an approximate 70% resection of the stomach, including the antrum and pylorus, with closure of the duodenum and restoration of gastrointestinal continuity with a 60-cm Roux-en-Y jejunal loop. Four longstanding Type 1 diabetic patients were examined and treated as described.

Pathophysiological assessment of nitric oxide (given as sodium nitroprusside) in acute ischaemic stroke.

Acute ischaemic stroke is characterised by reductions in local cerebral blood flow (CBF) and activation of circulating platelets and leucocytes. Nitric oxide is a vasodilator and can inhibit these circulating cells. The aim of this study was to assess the effect of nitric oxide on platelet function and regional CBF in patients with acute ischaemic stroke.

Effect of calcium intake and physical activity level on bone mass and turnover in healthy, white, postmenopausal women.

Calcium intake and physical activity level (PAL) were assessed by questionnaire in 124 healthy women aged 52-62 y to determine the effect of calcium intake and PAL on bone mass and turnover. Four groups were identified according to their different reported calcium intakes and PALs. Bone mineral density (BMD) at the spine, hip, and left os calcis was measured together with total bone mineral content (TBMC) with dual-energy X-ray absorptiometry.

The development of regional nuclear medicine audit in south Thames.

A programme of audit, including clinical audit, organizational audit and patient/clinician satisfaction surveys, has been implemented in nuclear medicine departments throughout the South Thames Regional Health Authority (RHA). The clinical topics which have been audited include. ventilation-perfusion lung imaging, bone imaging, liver imaging, thyroid imaging and renal static DMSA imaging.

Use of extracorporeal liver assist device and auxiliary liver transplantation in fulminant hepatic failure.

The case history of a 14-year-old boy with fulminant hepatic failure secondary to non-A, non-B hepatitis who fulfilled selection criteria for orthotopic liver transplantation is described. Two forms of liver support were used (extracorporeal liver assist device and an auxiliary partial orthotopic liver transplantation) to provide additional time to allow spontaneous recovery to occur. During the 66 h of extracorporeal haemoperfusion through the device, haemodynamic stability was maintained along with improvements in serum bilirubin (555 to 381 mumol/l), and international normalized ratio (INR) (3.

Reduced bone density in patients with inflammatory bowel disease.

Background: Reduced bone mineral density in patients with inflammatory bowel disease is thought to be due to disturbances in calcium homeostasis or the effects of corticosteroid treatment.

Aims: To assess the prevalence and mechanism of reduced bone mineral density in 79 patients with inflammatory bowel disease (44 with Crohn's disease, 35 with ulcerative colitis) who did not have significant risk factors for low bone densities.

Methods: Dual x ray absorptiometry was used to measure bone mineral density and serum and urinary markers of osteoblast (alkaline phosphatase, procollagen 1 carboxy terminal peptide and osteocalcin) and osteoclast (pyridinoline, deoxypyridinoline, and type 1 collagen carboxy terminal peptide) activities to assess bone turnover.

Imaging secondary neuroendocrine tumours of the liver: comparison of I123 metaiodobenzylguanidine (MIBG) and In111-labelled octreotide (Octreoscan).

Functional imaging of neuroendocrine tumours with Octreoscan and I123-metaiodobenzylguanidine (MIBG) is important for assessment prior to various therapies and assessing response. The two imaging methods have not been directly compared in hepatic neuroendocrine tumours. Patients (n = 18) were studied with both imaging techniques.

Use of neural networks in brain SPECT to diagnose Alzheimer's disease.

Unlabelled: The usefulness of artificial neural networks in the classification of 99mTc-HMPAO SPECT axial brain scans was investigated in a study group of Alzheimer's disease patients and age-matched normal subjects.

Methods: The cortical circumferential profiling (CCP) technique was used to extract information regarding patterns of cortical perfusion. Traditional analysis of the CCP data, taken from slices at the level of the basal ganglia, indicated significant perfusion deficits for Alzheimer's disease patients relative to normals, particularly in the left temporo-parietal and left posterior frontal areas of the cortex.