The use of venous catheterization in the diagnosis and localization of bilateral phaeochromocytomas.

OBJECTIVE: The purpose was to assess the value of venous catheter sampling as a method for the location of phaeochromocytomas, particularly when imaging techniques have been equivocal or ambiguous. DESIGN: Venous catheter sampling was carried out in cases of suspected phaeochromocytoma, and compared with samples obtained from other patients without phaeochromocytomas undergoing adrenal venous catheterization. PATIENTS: Three patients had phaeochromocytomas (subsequently confirmed by histology); five patients had no clinical or biochemical evidence of phaeochromocytoma but were being investigated for other conditions. MEASUREMENTS: Catecholamine and cortisol assays were performed on plasma samples from the adrenal veins and elsewhere, and the noradrenaline to adrenaline (NA:AD) ratio was calculated. RESULTS In patients without phaeochromocytomas the NA:AD ratio was less than 1 in nine adrenal vein samples; in four adrenal vein samples with NA:AD ratio of greater than 1, the presence of a phaeochromocytoma was subsequently confirmed. An extra-adrenal tumour was also located by comparison of central and peripheral venous catecholamine concentrations. CONCLUSIONS: Venous catheterization with measurements of catecholamines, and determination of NA:AD ratios, allows for the rapid and confident diagnosis and localization of unilateral, bilateral, and extra-adrenal phaeochromocytomas

Respiratory infections in children: an appropriateness study of when parents should home care or seek medical help

Background Children with respiratory tract infections (RTIs) use more primary care appointments than any other group, but many parents are unsure if, and when, they should seek medical help and report that existing guidance is unclear. Aim To develop symptom-based criteria to support parental medical help seeking for children with RTIs. Design and setting A research and development/University of California Los Angeles (RAND/UCLA) appropriateness study to obtain consensus on children’s RTI symptoms appropriate for home, primary, or secondary health care in the UK. Method A multidisciplinary panel of 12 healthcare professionals — six GPs, two pharmacists, two NHS 111 nurses, and two emergency paediatric consultants — rated the appropriateness of care setting for 1134 scenarios in children aged >12 months. Results Panellists agreed that home care would be appropriate for children with ≤1 week of ‘normal’ infection symptoms (cough, sore throat, ear pain, and/or runny nose, with or without eating adequately and normal conscious level). The presence of ≥2 additional symptoms generally indicated the need for a same-day GP consultation, as did the presence of shortness of breath. Assessment in the emergency department was considered appropriate when ≥3 symptoms were present and included shortness of breath or wheezing. Conclusion The authors have defined the RTI symptoms that parents might regard as ‘normal’ and therefore suitable for care at home. These results could help parents decide when to home care and when to seek medical help for children with RTIs

Plasma and 24 h-urinary catecholamine concentrations in normal and patient populations.

Using a single HPLC-ECD methodology, plasma catecholamine data were compiled from 545 patients and 144 healthy subjects, and 24 h urinary free catecholamine data from 106 patients. From these data, normal reference ranges were derived for the clinical investigation of suspected phaeochromocytomas, specifically to address the question as to when a result is normal and when further investigation may be required. We have shown that noradrenaline and adrenaline concentrations in plasma and urine are not normally distributed, and basal plasma noradrenaline is significantly lower in normal volunteers than in hospital patients. We have also demonstrated that a reference range which does not take account of these factors will give a significant number of false-positive results. Age and sex did not appear to be significant variables for either plasma or urinary catecholamines. In the investigation of phaeochromocytoma, 95% confidence limits may be used as a warning to repeat sampling and 99% confidence limits as rendering the diagnosis extremely probable. In patients with phaeochromocytomas, urinary catecholamines had a 99.5% specificity and 100% sensitivity in the diagnosis, and thus provide for a rapid and efficient diagnostic screen

Bilateral phaeochromocytomas in von Hippel-Lindau disease: diagnosis by adrenal vein sampling and catecholamine assay.

Three unrelated patients with von Hippel-Lindau disease had phaeochromocytomas diagnosed as a result of screening in their third decade. All had raised 24-h urinary noradrenaline levels and obvious unilateral adrenal tumours on ultrasound scanning and computed tomography. The contralateral adrenal appeared either normal or equivocally abnormal using the same imaging techniques. Radiolabelled meta-iodo-benzylguanidine uptake and enhanced T2-weighted magnetic resonance signals confirmed the ipsilateral lesion, but showed no abnormality in the contralateral adrenal. Nevertheless, venous sampling demonstrated abnormally elevated noradrenaline:adrenaline ratios (normal < 1) in blood draining both adrenals in all three patients. All underwent bilateral adrenalectomy: histological examination confirmed bilateral phaeochromocytomas in all three patients, with the smaller adrenal showing tumours between 3 and 11 mm in diameter. A fourth patient with von Hippel-Lindau disease was also evaluated because of a left 'suprarenal' mass discovered by ultrasound scanning. Urinary and plasma catecholamines were normal. Computed tomography showed a normal right adrenal, with a left para-aortic mass lying superior to the left adrenal. Magnetic resonance scanning showed an enhanced T2 signal from the mass, which also showed positive uptake with radiolabelled mIBG. At operation, a non-secretory left para-aortic paraganglioma was found. Venous sampling suggested that the left adrenal was normal, and this was confirmed on histology. Venous sampling for catecholamine assays is effective in locating small bilateral phaeochromocytomas and defining a normal adrenal gland, and can be useful in preventing unnecessary or repeat operations in patients with non Hippel-Lindau disease