bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pteridines and mono-amines: relevance to neurological damage
Authors: Smith I , Howells D W , Hyland K 
Published: December 2008

SummaryPatients with phenylalanine hydroxylase deficiency show increased concentrations of biopterins and neopterins, and reduced concentrations of serotonin and catecholamines, when phenylalanine concentrations are raised. The pterin rise reflects increased synthesis of dihydroneopterin and tetrahydrobiopterin, and the amine fall a reduction in amine synthesis due to inhibition by phenylalanine of tyrosine and tryptophan transport into neurones. The pterin and amine changes appear to be independent of each other and are present in the central nervous system as well as the periphery; they disappear when phenylalanine concentrations are reduced to normal. Patients with arginase deficiency show a similar amine disturbance but have normal pterin levels. The amine changes probably contribute neurological symptoms but pterin disturbance is not known to affect brain function.Patients with defective biopterin metabolism exhibit severely impaired amine synthesis due to tetrahydrobiopterin deficiency. Pterin concentrations vary with the site of the defect. Symptoms include profound hypokinesis and other features of basal ganglia disease. Neither symptoms nor amine changes are relieved by controlling phenylalanine concentrations. Patients with dihydropteridine reductase (DHPR) deficiency accumulate dihydrobiopterins and develop secondary folate deficiency which resembles that occurring in patients with defective 5,10-methylene tetrahydrofolate reductase activity. The latter disorder is also associated with Parkinsonism and defective amine and pterin turnover in the central nervous system, and a demyelinating illness occurs in both disorders. In DHPR deficiency cerebral calcification may develop in a similar distribution to that seen in congenital folate malabsorption and methotrexate toxicity. Symptoms are ameliorated by therapy with 5-formyltetrahydrofolate but exacerbated by folic acid.
Plasma electrolytes in elderly patients taking fixed combination diuretics
Authors: Bayer A J , Farag R , Browne S , Pathy M S J 
Published: December 2008

Summary
Plasma potassium and sodium concentrations were measured in a group of elderly patients taking maintenance thiazide diuretic therapy alone, with a potassium supplement or in combination with a potassium sparing diuretic. Fixed dose combinations of a thiazide and potassium sparing diuretic did not significantly reduce the prevalence of hypokalaemia and the combination of amiloride-hydrochlorothiazide was associated with a disproportionate number of cases of hyponatraemia. The desirability of the current widespread use of fixed dose combination diuretics over less expensive single agents is questioned.
Record linkage study of hypokalaemia in hospitalized patients
Authors: Paice B J , Paterson K R , Onyanga-Omara F , Donnelly T , Gray J M B , Lawson D H 
Published: December 2008

Summary
Retrospective analysis of biochemical data from 58,167 hospital inpatients revealed that 21% developed hypokalaemia during hospitalization-in 5.2% the serum potassium was &lt; 3.0 mmol/l. Subsequent evaluation showed a positive correlation between hypokalaemia and both female sex and hospital mortality. Patients with leukaemia and lymphoid tumours, especially when receiving antibiotic or cytotoxic therapy, and patients with gastro-intestinal malignancy were amongst those most frequently experiencing hypokalaemia. There was no significant association with cardiovascular disease. Drug and intravenous fluid administration accounted for the hypokalaemia in 56% of patients. While drug-related hypokalaemia was most commonly seen with diuretics, it was also apparent following use of steroids, insulin and haematinics.
Bilateral salmonella salpingo-oophoritis
Authors: Ghose A R , Vella E J , Begg H B 
Published: December 2008

Summary
A previously healthy nulliparous caucasian female presented with lower abdominal pain and a history of diarrhoea and vomiting for 2 weeks. Repeated stool examinations and blood culture were negative. A lump in the lower abdomen became more apparent over the next 2 weeks and a subsequent laparotomy revealed bilateral tubo-ovarian abscess formation. One ovary which was endometriotic and both the tubes were excised and the pus yielded a heavy growth of Salmonella stanley.
Immobilization hypercalcaemia due to low bone formation and responding to intravenous sodium sulphate
Authors: Evans R A , Lawrence P J , Thanakrishnan G , Hills E , Wong S Y , Dunstan C R 
Published: December 2008

Summary
A young man developed acute renal failure and hypercalcaemia following severe burns. The hypercalcaemia was initially controlled by haemodialysis, but it persisted after return of renal function. Plasma PTH was inappropriately elevated, but the nephrogenous cyclic adenosine monophosphate level was low; thus the PTH was probably not biologically active, and may have been artefactually elevated by the moderate renal impairment. Bone histology, showed a normal resorbing surface, but a zero forming surface, implying that the bone dissolution leading to hypercalcaemia resulted from a failure of bone formation. Because of widespread infection and impaired renal function, the hypercalcaemia could not be treated by corticosteroid drugs, mithramycin or phosphate, and there was no response to salmon calcitonin. He was therefore treated with intravenous sodium sulphate, which increased urinary calcium excretion and reduced the plasma calcium. Sodium sulphate still has a role in the treatment of patients with hypercalcaemia.
Acute pancreatitis in hepatitis A infection
Authors: Lopez Morante A , Rodriguez de Lope C , San Miguel G , Pons Romero F 
Published: December 2008

Summary
Hepatitis viruses are an uncommon cause of acute pancreatitis. We present the case of a boy with acute pancreatitis complicating viral hepatitis with satisfactory recovery. The finding of IgM-anti HAV antibodies implicates hepatitis A virus as the cause.
Fatal xylenol self-poisoning
Authors: Watson I D , McBride D , Paterson K R 
Published: December 2008

SummaryA case of fatal xylenol ingestion by a long-stay mental hospital patient is described. The clinical course was similar to that observed in other phenolic poisonings with active bowel sounds, nausea and vomiting, severe metabolic acidosis, hypotension and cardiac and renal failure.The formulation of xylenol ingested contains alcohol which would facilitate absorption; due to the dangers of such poisonings care must be exercised as to access and exposure to xylenol sterilising agents.
Significance of the measurement of uric acid fractional clearance in diuretic induced hyponatraemia
Authors: Sonnenblick M , Rosin A J 
Published: December 2008

SummaryThe biochemical features of severe hyponatraemia due to thiazide administration in 7 non-oedematous patients were compared with those in hyponatraemia due to frusemide. Hypouricaemia has been shown to occur in hyponatraemia due to the syndrome of inappropriate antidiuretic hormone activity and this was measured along with fractional uric acid clearances in all the patients. Five of the patients had been on thiazides (or hydrochlorothiazide with amiloride) for only a few days to a few weeks. Fractional uric acid clearance was elevated and serum uric acid levels were low in five of them and returned to the normal range on restoration of serum sodium to normal. By contrast, the patients on frusemide did not show any abnormality in fractional uric acid clearance at any stage.These results are consistent with excess ADH activity as having caused hyponatraemia induced by thiazides in 5 of the 7 cases, whereas frusemide caused a sodium depletion syndrome. Treatment in the former cases is by water restriction, and in frusemide-induced salt depletion by saline supplementation.
Muscular (hypertrophic) subaortic stenosis (hypertrophic obstructive cardiomyopathy): the evidence for true obstruction to left ventricular outflow
Authors: Wigle E D , Henderson M , Rakowski H , Wilansky S 
Published: December 2008

Summary
The clinical and haemodynamic significance of the subaortic pressure gradient in patients with muscular (hypertrophic) subaortic stenosis (hypertrophic obstructive cardiomyopathy) has long been debated. In this report we summarize the evidence which indicates that true obstruction to left ventricular outflow exists in these patients.
Rapid left ventricular ejection, through an outflow tract narrowed by ventricular septal hypertrophy, results in Venturi forces causing systolic anterior motion of the anterior (or posterior) mitral leaflets. Mitral leaflet-septal contact results in obstruction to outflow and the accompanying mitral regurgitation. The time of onset of mitral leaflet-septal contact determines the magnitude of the pressure gradient and the severity of the mitral regurgitation, as well as the degree of prolongation of left ventricular ejection time and the percentage of left ventricular stroke volume that is ejected in the presence of an obstructive pressure gradient. Early and prolonged mitral leaflet-septal contact results in a large pressure gradient, significant mitral regurgitation, as well as dramatic prolongation of the ejection time and a large percentage of left ventricular stroke volume being obstructed. Late and short mitral leaflet-septal contact results in little haemodynamic perturbation. Hypertrophic cardiomyopathy patients with obstructive pressure gradients are significantly more symptomatic than those without. Thus the obstructive pressure gradients in hypertrophic cardiomyopathy are of clinical as well as haemodynamic significance. To deny the existence of obstruction to outflow in patients with muscular subaortic stenosis is to deny these patients appropriate medical and surgical therapy.
Phonoechocardiography and intracardiac phonocardiography in hypertrophic cardiomyopathy
Authors: Shaver J A , Alvares R F , Reddy P S , Salerni R 
Published: December 2008

Summary
The salient phonoechocardiographic features of patients having hypertrophic cardiomyopathy (HCM) with or without left ventricular outflow tract (LVOT) gradients are reviewed. Intracardiac sound and pressure recordings from high fidelity catheter-tipped micromanometers have documented that the precordial murmur is the summation of both the systolic ejection murmur (SEM) arising from the LVOT, as well as the mitral regurgitant murmur recorded from the left atrium. The intensity of the precordial murmur varies directly with the LVOT gradient, which in turn is determined primarily by the contractility and loading conditions of the left ventricle.
Reversed splitting of the second heart sound (S2) with paradoxical respiratory movement is a common finding in HCM, and when present, almost always denotes a significant LVOT gradient. It is due to marked lengthening of the left ventricular ejection time secondary to prolongation of the contraction and relaxation phases of left ventricular systole. The presence of a fourth heart sound (S4) is the rule in HCM when normal sinus rhythm is present, and is a reflection of a forceful left atrial contraction into a hypertrophied noncompliant left ventricle. A third heart sound (S3) is also common in HCM, and often the initial vibrations occur before the 0 point of the apexcardiogram (ACG) and continue giving the auscultatory impression of a diastolic rumble. When associated with a loud S1, which is frequently present, the clinical presentation may mimic mitral stenosis. This is particularly true when the patient has chronic atrial fibrillation. Careful attention to evidence of marked left ventricular hypertrophy as well as the typical echocardiographic findings of HCM preclude this diagnosis.
In conclusion, phonoechocardiography is a simple non-invasive technique which almost always makes the definitive diagnosis of HCM.
Surgical treatment of hypertrophic cardiomyopathy (Düsseldorf experience)
Authors: Bircks W 
Published: December 2008

Summary
Surgical treatment appears to be the therapy of choice in hypertrophic, obstructive cardiomyopathy, at least in patients who are in the advanced stage of the disease and refractory to medical treatment. In addition, strong evidence has been obtained that surgical treatment improved the prognosis at least in patients who required only a myectomy via a transaortic approach.
Serial cardiac function tests in myocarditis
Authors: Miklozek C L , Kingsley E M , Crumpaker C S , Modlin J F , Royal H D , Come P C , Mark R , Abelmann W H 
Published: December 2008

Summary
Sixteen patients (mean age 27 years, range 16 to 39 years) with the diagnosis of myopericarditis established by strict clinical criteria were evaluated following recovery 0.7 to 4.0 years (mean 2.7 years) later. Evidence of an acute viral infection was present in 44%. During the acute illness, the major clinical manifestations consisted of pericarditis in 10 patients, acute myocardial infarction in 5, right ventricular dysfunction in 5, bundle branch or hemiblock in 4, ventricular arrhythmias in 3, congestive heart failure in 3 and cardiogenic shock and inappropriate sinus tachycardia in one patient each. Holter monitoring, echophonocardiography and radionuclide ventriculography results were abnormal in 57, 67 and 64% of patients respectively. At follow-up, these tests were abnormal in 67, 7 and 73%. Focal wall motion abnormalities were present in five. Seventy-five percent of patients had one or more abnormal tests at last follow-up. Although 8 patients had improved by a scoring system, 5 patients had an increase in the number of abnormal tests, including one who died. These findings indicate that persistent abnormalities following recovery from myopericarditis are not rare and support the hypothesis that the syndrome of dilated cardiomyopathy may be a sequel of myopericarditis.
Glomus tumour of the hallux: diagnosis by Doppler-shift ultrasound and digital subtraction angiography
Authors: Kreel L , Thornton A , Pardy B J 
Published: December 2008

Summary
A case is presented of a glomangioma with typical history and clinical findings, proven by operation and histology. Unique radiographic features are demonstrated including visualization of the tumour on a soft tissue radiograph and associated hyperaemic bone changes, continuous wave Doppler results indicating hyperaemia and an arterio-venous malformation, and the clear demonstration of the tumour in both frontal and lateral views was possible by intra-arterial digital subtraction angiography (DSA) under local anaesthesia. Fibrous dysplasia of a femur was an incidental finding.
Cushing's syndrome—transitory immune deficiency state?
Authors: Würzburger M I , Prelević G M , Brkić S D , Vucković S , Pendić B 
Published: December 2008

Summary
A 28 year old female patient with Cushing's syndrome due to an adrenal adenoma also suffered from recurrent urinary infections (proteus), tonsillitis (streptococcus), permanent candidiasis and perimandibular abscess (Staphylococcus pyogenes). Suppression of cellular and humoral immunity was confirmed by in vitro tests. After successful right adrenalectomy the clinical signs of Cushing's syndrome disappeared and no evidence of either bacterial or fungal infection were noted one year postoperatively. Immunological tests showed the restitution of both cellular and humoral immunity.
The course of the disease in the patient supports the idea that Cushing's syndrome might be considered as a transitory immune deficiency state.
Insulin oedema
Authors: Evans D J , Pritchard-Jones K , Trotman-Dickenson B 
Published: December 2008

Summary
A 35 year old markedly underweight woman presented with uncontrolled diabetes. Following insulin therapy she developed gross fluid retention with extensive peripheral oedema, bilateral pleural effusions and weight gain of 18.8 kg in 22 days, accompanied by a fall in plasma albumin. She responded well to treatment with diuretics and salt-poor albumin, losing 10.3 kg in 6 days without recurrence of oedema. Severe insulin oedema is an uncommon complication of insulin therapy and may be due to effects of insulin on both vascular permeability and the renal tubule.
How does stress exert its effects—smoking, diet and obesity, physical activity?
Authors: Kornitzer M , Kittel F 
Published: December 2008

Summary
Using a job stress questionnaire a negative correlation was found between job stress and physical fitness and a positive one with Type A behaviour. No correlation was found between job stress and obesity, nutritional patterns or physical activity. Subjects with angina had higher scores on the job stress questionnaire than normal controls. The job stress score was not predictive of future coronary heart disease.
Stress mechanisms in hypertension
Authors: Steptoe A 
Published: December 2008

Summary
Recent studies of stress mechanisms in hypertension have focussed on the cardiovascular and neuroendocrine reactions to challenging or threatening psychosocial stimuli. Fixed hypertension may develop in some animal models following chronic exposure to psychosocial conflict. Acute experiments in humans show that marked sympathetically-mediated cardiovascular reactions accompany the performance of challenging tasks. Responses are more accentuated in hypertensives and in people at high risk for developing the disorder. The working hypothesis to emerge is that the haemodynamic responses that accompany attempts to cope with challenging environments may promote the spiral towards sustained hypertension in susceptible individuals.
DIDMOAD syndrome with megacystis and megaureter
Authors: Chu P , Staff W G , Morris J A , Polak J M 
Published: December 2008

SummaryA case of DIDMOAD syndrome (diabetes insipidus, diabetes mellitus, optic atrophy and nerve deafness) is described. There was unusually severe urinary tract dilatation which led to an ileal conduit diversion. Immunohistological study of the bladder wall and ureter revealed a marked diminution in nerve fibres, which may have been primary or secondary to the muscle hypertrophy. The possible pathogenesis of the urinary tract dilatation is discussed in relation to this finding.
Surgery for the acute abdomen in adults with leukaemia
Authors: Martell R W , Jacobs P 
Published: December 2008

Summary
In a retrospective analysis five of 104 patients with acute leukaemia undergoing cytotoxic chemotherapy developed abdominal signs requiring emergency surgical exploration. Two common features in these patients were the inclusion of cytosine arabinoside in their treatment regimen and a necrotizing lesion involving the terminal ileum, appendix, and right colon. Appendicectomy was performed in two and hemicolectomy in three. Of the patients receiving high-dose cytosine arabinoside one died in the immediate post-operative period and two after recovery from surgery but before marrow regeneration; the remaining two patients received conventional dose cytosine arabinoside, and both recovered. The inclusion of this agent is standard in modern treatment programmes, resulting in significant improvement in long-term disease-free survival, so that a greater awareness of this complication is necessary. Early surgery, utilizing modern support techniques, is recommended as feasible and appears to offer the only realistic chance of survival.
Recurrent sterile meningitis caused by a pituitary abscess
Authors: Ford J , Torres L F , Cox T , Hayward R 
Published: December 2008

Summary
The case of a 21 year old woman who presented with recurrent sterile meningitis and bitemporal hemianopia is described. Computed tomographic scan showed a large low density ring enhancing lesion above and within the pituitary fossa which at operation and on histological examination was found to be compatible with an abscess. The visual field defect recovered completely and the scan appearances returned to normal postoperatively.
Previously unreported abnormalities of dermal basement membranes and collagen fibrils in a patient with X-linked spondyloepiphyseal dysplasia tarda
Authors: Borsey D Q , Hopwood D , Montgomery A J , Walsh D B , Jung R T 
Published: December 2008

SummaryElectron microscopic findings of the skin biopsy of a boy with spondyloepiphyseal dysplasia tarda showed several novel features. The basement membrane of the epidermis showed focal irregular thickening and reduplication. The collagen fibres showed foci of fibrous long spacing collagen and marked variation in fibre diameter. Taken together these abnormalities have not been reported in any other condition.
Use of computerized tomography of the abdominal wall in the diagnosis of partial post-operative wound dehiscence
Authors: Smith-Behn J , Arnold M , Might J 
Published: December 2008

SummaryA patient had occult post-operative partial wound dehiscence which was accurately diagnosed by performing a CT scan of the abdomen. It is suggested that CT scan of the abdominal wall is useful for early diagnosis of occult abdominal wound dehiscence.
Serum uric acid, serum glucose and diabetes: relationships in a population study
Authors: Cook D G , Shaper A G , Thelle D S , Whitehead T P 
Published: December 2008

Summary
The relationships between serum uric acid, serum glucose and diabetes have been examined in a survey of 7735 middle-aged men drawn at random from general practices in 24 British towns. There was a positive relationship between serum glucose and serum uric acid concentrations up to about 8.0 mmol/l; at higher levels of glucose, serum uric acid decreased. Uric acid levels were significantly reduced in insulin-dependent diabetics and in those on oral hypoglycaemics and also in ‘non-diabetics’ with casual glucose levels &gt; 10 mmol/l.
Both uric acid and glucose concentrations were positively related to body mass index; only uric acid was positively related to alcohol intake. Men on antihypertensive treatment had raised levels of uric acid (significant) and glucose (non-significant). The positive relationship between serum uric acid and serum glucose could not be explained by associations with body mass index, alcohol intake, age, social class, gout or treatment for hypertension. It probably reflects the biochemical interaction between serum glucose and purine metabolism, with increased excretion of uric acid during hyperglycaemia and glycosuria.
Lumbar puncture and subarachnoid haemorrhage
Authors: Patel M K , Clarke M A 
Published: December 2008

Summary
Medical notes of 123 patients with subarachnoid haemorrhage were examined. No patient deteriorated at the time of lumbar puncture. All but 2 patients with associated intracerebral haematomas had markedly impaired consciousness or focal signs.
Two of 30 patients with meningitis were initially diagnosed as subarachnoid haemorrhage, and one died without lumbar puncture. If consciousness is not markedly impaired and focal signs are absent, lumbar puncture is safe in patients with subarachnoid haemorrhage.
Myocardial hypertrophy, fibrosis and infarction following exposure of the heart to radiation for Hodgkin's disease
Authors: O'Donnell L , O'Neill T , Toner M , O'Briain S , Graham I 
Published: December 2008

SummaryA 35 year old man was treated for stage IIA Hodgkin's disease by radiation to the upper thorax, axillae and neck. Three years later he presented with intractable and ultimately fatal congestive heart failure.Autopsy revealed massive biventricular hypertrophy with widespread subendocardial fibrosis and myocardial infarction, but with little coronary artery disease. Such a complex of features has not previously been described after radiation therapy and cannot be adequately explained by other known causes of heart muscle disease. Ventricular hypertrophy with extensive subendocardial fibrosis may be part of the spectrum of radiation heart disease.