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In 1767 at the time of his great "candlelight" paintings of science and the Enlightenment, The Orrery and The Air-Pump , Wright of Derby developed the illness that plagued him for the rest of his life and became a patient and life-long friend of Dr Erasmus Darwin. Wright's recorded complaints and the periodicity are highly suggestive of the major depressive illness, seasonal affective disorder.

Sir William Knighton went from general practitioner in Devon to close friend and adviser of King George IV. He contributed remarkably to the stability of the Crown, bringing this dysfunctional King's finances under control and enabling the work of government requiring Royal decision-making to proceed much more effectively than it might otherwise have done. Inevitably he was involved in the making and breaking of ministries but appears to have done so with some reluctance. His detractors appear to have been motivated mainly by envy, fear of loss of patronage and social prejudice. His Royal career echoes physicianly virtues of fidelity to trust, empathy and honesty. He made every effort to keep a low public profile in order to minimize envy and intrusion into his private life. His success in this regard may account for the fact that he is practically unknown today despite a unique career in British medicine.

H+-ATPases are ubiquitous in nature; V-ATPases pump protons against an electrochemical gradient, whereas F-ATPases reverse the process, synthesizing ATP. We demonstrate here that mutations in ATP6B1, encoding the B-subunit of the apical proton pump mediating distal nephron acid secretion, cause distal renal tubular acidosis, a condition characterized by impaired renal acid secretion resulting in metabolic acidosis. Patients with ATP6B1 mutations also have sensorineural hearing loss; consistent with this finding, we demonstrate expression of ATP6B1 in cochlea and endolymphatic sac. Our data, together with the known requirement for active proton secretion to maintain proper endolymph pH, implicate ATP6B1 in endolymph pH homeostasis and in normal auditory function. ATP6B1 is the first member of the H+-ATPase gene family in which mutations are shown to cause human disease.

This study was performed to compare the effect of one month's treatment with hydrochlorothiazide (25 mg) in combination with either amiloride (2.5 mg) or triamterene (50 mg) on plasma sodium levels in elderly people in institutional care. Fifty residents of NHS nursing or social service residential care established on diuretics for congestive cardiac failure and aged 64 years or over were recruited. Forty-one patients were included in the final data analysis. Patients on hydrochlorothiazide/amiloride had a significantly lower plasma sodium (137 vs 139 mmol/l, 95% confidence interval for difference between medians 0-2 mmol/l) than those on hydrochlorthiazide/triamterene (P = 0.01). In equivalent potassium-retaining doses, amiloride is associated with significantly lower plasma sodium levels than triamterene, when given in combination with hydrochlorothiazide in elderly patients with congestive cardiac failure. This finding adds weight to uncontrolled observations implicating thiazide/amiloride diuretic combinations in causing serious hyponatraemia. This danger, although uncommon, should perhaps influence prescribing habits in an at-risk population.

A 28-year-old woman presenting with systemic lupus erythematosus stuck two sewing needles into her pericardium while psychotic from cerebral lupus. We believe this is a unique cause of acute pericarditis as a complication of SLE.

This paper reports a retrospective study of the clinical value of percutaneous renal biopsy in secondary referral centres. Between 1984-90, 205 patients over the age of 16 had 218 biopsies at three district general hospitals. Adequate tissue was obtained in 194 patients (95%, 89% of the biopsies). Significant complications occurred in only four patients. In 170 patients (83%) the biopsy yielded information of diagnostic and prognostic value and influenced management. The main indications for biopsy were nephrotic syndrome, in 63 patients, and chronic renal failure, in 58 patients. The most frequent findings were minimal change disease, focal segmental glomerulosclerosis, IgA nephropathy, membranous glomerulonephritis and mesangiocapillary glomerulonephritis. The most obvious association between indication and histology was between haematuria and IgA nephropathy. Percutaneous renal biopsy in the district general hospital in patients selected by a nephrologist and performed by experienced or supervised operators is a safe procedure. There is a high yield of renal tissue which is of clinical value in patient care.

Twenty-eight patients (11 Caucasian, 17 black) whose blood pressure was more than 160/96 mmHg after 4 weeks on placebo added to atenolol 100 mg/day were randomly given, in addition, nisoldipine 10 mg or nifedipine 20 mg each twice a day for 8 weeks in a double-blind cross-over study. There was a statistically significant (P < 0.001) fall in blood pressure with no change in heart rate, both supine and erect, on both drugs. There were no significant differences between nisoldipine and nifedipine. Adverse effects were recorded in 15%, 17% and 35% of the patients available for safety comparison for placebo, nisoldipine and nifedipine, respectively. There were no significant differences between the black and Caucasian patients in blood pressure responses, although the study had only a low power to detect these. However, the fasting serum triglyceride levels at the end of both calcium antagonist treatment periods were highly significantly lower in the black patients compared with the Caucasian patients. Nisoldipine, which has a higher coronary vascular selectivity and less negative inotropism than nifedipine, is as effective and as well tolerated as nifedipine in patients whose hypertension is inadequately controlled on atenolol. It may have a special role in hypertensive patients with impaired left ventricular function.

A 42 year old man presented with accelerated hypertension and an abdominal mass that proved to be a primary malignant retroperitoneal germ cell tumour involving renal vessels. The hypertension resolved with chemotherapy. To our knowledge this is the first case of a primary malignant retroperitoneal germ cell tumour to present with accelerated hypertension.

Gastrointestinal complications of Legionnaires' disease are frequently reported. These include diarrhoea, paralytic ileus, acute appendicitis and jaundice. We would like to report the previously unrecorded and life-threatening complication of acalculous cholecystitis.

Granulomatous muscle disease is most commonly seen in sarcoidosis, but may be seen in association with a wide variety of other disorders or in isolation. Patients with granulomatous myositis usually present with slowly progressive muscle pain and weakness affecting mainly proximal muscles. There are, however, a few reports of granulomatous muscle disease presenting with flexion contractures of the limbs. Two further patients with granulomatous muscle disease and flexion contractures of the limbs, but with no evidence of systemic granulomatous disease, is presented. It is suggested that such patients represent a separate clinical entity that is distinct from idiopathic granulomatous myositis presenting with muscle pain and weakness. The association of contracturing granulomatous myositis with a long-standing vasculitis in one patient suggests that the two conditions may be related.

Our experience has suggested that not only do nurses on hospital wards waste time making too many unnecessary clinical observations, but that it is very difficult to persuade them to modify the pattern of their practice. We therefore conducted a survey by questionnaire among trained nurses in one health district of their everyday ward practice and attitudes to clinical observations. This showed that whilst a majority of nurses questioned believe in the importance of routine observations for patient care, there is a lack of consistency in the criteria and communication procedures used that must seriously limit the value of the information obtained by the individual nurse. We believe that uncritical faith in routine observations is a barrier to rationalizing procedures to optimize efficient use of the professional skills of ward nurses in this regard. There is need for more thought to be given to the problem and in particular it is important that doctors play a more active role in decision making.

Two patients with long-standing chronic bronchial suppuration developed antineutrophil cytoplasmic antibody (ANCA) positive Wegener's granulomatosis and microscopic polyarteritis respectively. There is published evidence of an association between previous suppurative respiratory disease and Wegener's granulomatosis. We believe that our cases provide further evidence that chronic lung infection may play an aetiological role in the development of ANCA-positive systemic necrotizing vasculitis in some individuals.

A prospective study of severe hyponatraemia in adult hospital inpatients showed that 44 patients had plasma sodium concentrations below 125 mmol(mEq)/1. Eighteen cases (41%) were iatrogenic, caused by diuretic treatment or postoperative administration of intravenous 5% dextrose, or both. Chest infection, a seldom-recognised and ill-understood cause of hyponatraemia, proved more common than carcinoma of the bronchus. Thirty-one patients had symptoms attributable to the hyponatraemia, but these were severe in only five cases. Analysis of blood and urine was of no value in distinguishing the different diagnostic groups in an emergency.

Ganglioside GM1 (100 mg) was given daily by intramuscular injection for 28 days in a double-blind placebo controlled trial of acute stroke. No significant difference was detected in a 6 month follow-up period between well matched control and active groups. Although the number of patients studied was small the findings are believed to indicate that GM1 is unlikely to be of value in the treatment of acute stroke in the dose and route of administration used.

Six patients are presented, all of whom had systemic lupus erythematosus or a "lupus-like" disease and who developed major thromboses with gangrene of the extremities. Four of the 6 patients had circulating antiphospholipid antibodies at some point during the course of their illness. These serological markers, which have been associated with a tendency to thrombosis, may have contributed to the development of gangrene in these patients. Histological examination of affected arteries in 4 patients did not show any evidence of vasculitis. One patient in whom antiphospholipid antibodies were negative showed healing vasculitis on histology.

The optic neuritis of systemic lupus erythematosus (S.L.E.) more frequently results in the persistence of a central scotoma or complete blindness after a single attack than demyelinating optic neuritis, although the initial clinical presentations may be identical. A significant number of patients, however, recover normal vision. Optic neuritis may be the presenting symptom of S.L.E. and as myelopathy may also occur in the course of the disease, confusion with multiple sclerosis may result, especially if there are no arthritic, cutaneous nor visceral manifestations. We report a case of lupus optic neuritis associated with anticardiolipin antibodies and a circulating lupus anticoagulant and suggest these may be a marker for vasculitic optic neuritis and play a role in its aetiology.

The effect of a six-week course of spironolactone 300 mg/day was examined in 25 unselected patients with essential hypertension. In the blood spironolactone produced a significant rise in urea and potassium concentrations and a fall in sodium and bicarbonate concentrations. In six patients blood pressure was normal at the end of the course, while in five patients there was almost no change. Studies of the effects of spironolactone on various indices usually affected by mineralocorticoids-namely, blood electrolytes, total body potassium, and rectal electrical properties-showed no differences between responding and non-responding patients. Mineralocorticoid excess therefore seems to be rarely responsible for essential hypertension and the influence of spironolactone cannot at present be fully explained.

Renovascular hypertension is an unusual complication of renal trauma, occurring in approximately 5% of cases. It occurs predominantly in young males following road traffic accidents (RTA) or blunt abdominal trauma. The interval between injury and development of hypertension varies from two days to 14 years but presentation may be acute with hypertensive encephalopathy. Hypertension developing within a few months of injury may be treated conservatively. Conservative treatment more than one year after injury is associated with an increased risk of persistent hypertension. All cases of renal trauma should be followed with regular blood pressure (BP) recording for at least the first year after injury.