Anaesthetic problems

The incidence of significant abnormalities is high. In a review, 100 cases of Down's syndrome requiring surgery, 44 patients had lesions requiring cardiac surgery, and 41 others had abnormalities or diseases with anaesthetic implications (Kobel et al 1982). 1. Cervical spine abnormalities increase the risk of dislocation of certain cervical vertebrae on intubation, or when the patient is paralysed with muscle relaxants.Atlantoaxial subluxation and spinal cord compression were discovered in two...

Hereditary haemorrhagic telangiectasia HHT Osler WeberRendu disease

An autosomal dominant disease in which there are cutaneous, mucosal and visceral vascular anomalies, accompanied by a family history of the disorder. Recent research has shown mutations in the endoglin gene on the long arm of chromosome 9 (Shovlin & Letarte 1999). Many of the problems are functional consequences of direct communications between the pulmonary and systemic sides of the circulation. Affected individuals may present with epistaxis or gastrointestinal bleeding, often after the...

Info

Management of diabetes during elective surgery. Traditionally, it was recommended that diabetics be admitted 2 3 days in advance of surgery and appear first on a morning operating list, and that long-acting oral hypoglycaemic agents be discontinued 2 days in advance, or changed to short-acting ones. Similarly, long-acting insulins should be changed to either actrapid or other short-acting insulin. In practice, the anaesthetist is often presented with diabetic patients the evening before...

Prader Willi syndrome

A multisystem genetic disorder of unknown aetiology, in which obesity is associated with disturbances of carbohydrate and fat metabolism. In some cases, abnormalities of chromosome 15 are thought to be involved. It is a two-stage disorder and the clinical features change with age 0 36 months, and 3 years to adulthood. Consensus criteria have been agreed and diagnosis is based on a scoring system (Holm et al 1993). In infancy the disorder presents with hypotonia, feeding and respiratory...

Anaesthetic Management In Case Of Tvd Patient With Jejunal Stricture

Nowadays, the increased quality of monitoring means that the fulminant case is rarely seen. However, it also means that there is an increase in the number of aborted or doubtful cases. Since the early signs of MH are nonspecific, it is the responsibility of the anaesthetist, should MH be suspected, to gain as much information as possible at the time of the event. 2. What should be done if intraoperative signs occur which are not specific to MH, and are not life-threatening, but raise the...

Central anticholinergic syndrome

A term given to a syndrome of blockade of central cholinergic neurotransmission, probably involving muscarinic receptors, which produces a clinical picture similar to that of atropine intoxication (Schneck & Rupreht 1989). It may be caused by any drug that has central anticholinergic actions, sometimes even after normal doses. It occurs most commonly following general anaesthesia, but also after acute intoxication from self poisoning, in association with sedation on the ITU, or during acute...

Guillain Barre syndrome GBS

A collective name given to a group of acute ascending polyneuropathies in which motor involvement predominates. Some of these may have an autoimmune basis and antecedent infection, surgery and immunisations have all been implicated in their development.Two-thirds of patients have an URTI or a gastrointestinal infection 1-4 weeks before the onset of symptoms and Campylobacter jejuni is the most frequent antecedent pathogen, with CMV and EBV also being implicated (Hartung 1999). Almost...

Churg Strauss syndrome

A syndrome of asthma, allergic rhinitis, pulmonary and systemic small-vessel vasculitis and extravascular granulomas. Reid et al (1999) reported organ systems involvement as follows lungs (48 ), heart (44 ), kidney (48 ), nervous system (78 ),skin (48 ),bowel (30 ), joints (57 ), and muscle (57 ).The presence of severe gastrointestinal disease or myocardial involvement is associated with a poor prognosis (Guillevin et al 1999). It has sometimes been associated with the new leukotriene...

Albin Lumbar Laminectomy Cardiac Arrest

Three patients undergoing lumbar laminectomy in the prone position developed sudden air embolism (Albin et al 1991).This is a rare occurrence, but in each case a frame was used to retain the patient in a 'four-poster' position. In this posture, the pressure in the inferior vena cava is low and there is a gravitational gradient between the right side of the heart and the operative site. k) Transurethral resection of prostate. Fatal massive air embolism occurred after TURP...

Anaesthesia Pheohromocytoma

Fahmy N,Assaad M, Bathija P et al 1997 Postoperative pulmonary edema a rare presentation of h pheochromocytoma. Clinical Nephrology 48 o Fenje N,Lee LW,Jamieson WRE et al 1989 Phaeochromocytoma and mitral valve replacement. y Canadian Journal of Anaesthesia 36 198-9. Greaves DJ, Barrow PM 1989 Emergency resection of M phaeochromocytoma presenting with hyperamylasaemia and pulmonary oedema. Anaesthesia 44 841-2. Harioka T, Nomura K, Hosoi S et al 1999 Laparoscopic resection of unsuspected...

Malignant hyperthermia

A rare pharmacogenetic condition, or possibly a spectrum of conditions, of complex inheritance. Malignant hyperthermia MH usually presents during general anaesthesia with a syndrome indicative of greatly accelerated muscle metabolism. Occasionally, it has been induced by severe exercise Britt 1988, Hackl et al 1991 .The exact defect is unknown. Dysfunction of the sarcoplasmic reticulum and abnormalities of intracellular ionic calcium are thought to play an important role, with the secondary,...

Buergers disease

A type of occlusive peripheral vascular disease which, until recently, predominantly affected young men. Some changes in the disease pattern have been observed Olin et al 1990 .An increasing number of women, more common involvement of the upper extremity, and its occurrence in older patients are being reported Sasaki et al 1999 .There is a strong association with smoking.The disease primarily affects small vessels of the feet, legs, and hands, and is exacerbated by vasospasm.The aetiology is...

Duchenne muscular dystrophy DMD

An X-linked recessive, severe muscular dystrophy, that usually presents with proximal lower limb and pelvic muscle weakness.The weakness, which is progressive and varies between muscles, results from a decrease in the total number of muscle fibres.The young child, on attempting to rise, will use its arms to 'climb' up its own legs. Cardiac muscle disease occurs, with characteristic ECG changes and a hypertrophic cardiomyopathy. The condition steadily progresses to involve other muscles until...

Automatic implantable cardioverter defibrillator AICD m

Patients requiring surgery and anaesthesia A device used in the treatment of patients with recurrent tachyarrhythmias that are unresponsive to medical treatment.The automatic implantable cardioverter defibrillator AICD senses VT or VF and responds with countershocks to the heart. Although there are significant complications from the insertion of this device and its subsequent aftercare, in this high-risk group of patients the AICD has been found to be superior to antiarrhythmic drugs in...

Goodpastures syndrome

A rapidly progressive syndrome of glomerulonephritis and pulmonary haemorrhage, in association with antibodies to glomerular basement membrane anti-GBM type IV collagen, detectable in plasma by radioimmunoassay and by immunofluorescence techniques on muscle biopsy. These antibodies cross-react with alveolar basement membrane, although those individuals with lung haemorrhage are usually smokers, and those with isolated anti-GBM nephritis are nonsmokers. The term is often applied more loosely, to...

Multiple system atrophy MSA

formerly Shy-Drager syndrome see also Autonomic failure A progressive condition presenting in later life, in which autonomic failure or dysfunction is associated with, or precedes, the onset of widespread central neuronal degeneration.The old term 'Shy-Drager syndrome' has been replaced by the broader term multiple system atrophy MSA .This has been divided into three categories Schatz 1996 1. Striatonigral degeneration, which produces a Parkinsonian form with some cerebellar dysfunction. 2....

Preoperative abnormalities

The patient may be asymptomatic, and about one-third of cases present because of an abnormality found on CXR.With more advanced disease there may be variable degrees of respiratory impairment. CXR usually shows bilateral hilar lymphadenopathy with increased reticular shadowing in the lung fields. Lung function tests may be impaired. Restrictive gas transfer and obstructive defects may all occur at different stages of the disease. In advanced disease, pulmonary hypertension may develop. 2....

TURP syndrome

A syndrome which may occur during transurethral resection of the prostate, in which large quantities of glycine 1.5 irrigating fluid are absorbed into the circulation through open veins in the prostatic bed, although some is also absorbed more slowly from the retroperitoneal or perivesical spaces. Glycine 1.5 is a nonelectrolytic, slightly hypotonic solution 2.1 would be isotonic , which on absorption is mainly confined to the ECF. Plasma sodium levels are decreased by more than would be caused...

Thyrotoxic crisis or storm see also

The abrupt onset of symptoms of a severe hypermetabolic state, associated with the output of thyroxine, in a patient with pre-existing thyroid disease.This is a clinical, not a bichemical, diagnosis, and biochemically it is difficult to distinguish between the two. However, serum free T4 concentrations are significantly higher in a thyroid crisis compared with thyrotoxicosis. It may occur in a patient with occult thyroid disease, in whom a crisis may be precipitated by an acute medical,...

Laryngeal papillomatosis

Benign, warty tumours of the larynx that occur mainly in children, but sometimes in adults, and are caused by one or more of the human papillomaviruses, the majority type 11, but sometimes type 6.They arise most commonly on the true vocal cords with extension onto the ventricles. Frequently recurrent, they may be present in other parts of the respiratory tract. Extension into the subglottis is common, but involvement of the trachea, proximal bronchus and lung parenchyma is rare. In children,...

Acromegaly

A rare, chronic disease of insidious onset, which usually presents in middle life.An increased secretion of growth hormone GH , associated with high insulin-like growth factor somatomedin, IGF-1 levels, results in an overgrowth of bone, connective tissue, and viscera. It is most commonly caused by an adenoma of the eosinophil cells of the pituitary. Lifespan is considerably shortened, and deaths are primarily from cerebrovascular, respiratory and cardiovascular complications. Death from...

Zenkers diverticulum

A pharyngeal pouch, which is formed by an outpouching of pharyngeal mucosa in the posterior wall of the hypopharynx at its junction with the oesophagus through Killian's dehiscence. At this site there is an area of weakness that lies between the cricopharyngeus and the inferior pharyngeal constrictor muscles.There may be a functional component to the condition, because neuromuscular dysfunction, particularly in the relaxation of cricopharyngeus, has been found. 1. The main complaints are of...

Drowning and near drowning

Drowning is one of the commonest causes of accidental death in young people, but is potentially remediable if appropriate treatment is instituted without delay. Early animal studies led to undue emphasis being placed on the differences between immersion in salt or fresh water, the accompanying osmotic changes, and, in fresh water, the possibility of massive haemolysis. In practice, the inhaled volumes are much less than those induced experimentally, the haemolysis is not significant, and, in...

Autosomal dominant

Facioscapulohumeral Landouzy Dejerine, FSHD has onset in adolescence, first affecting the shoulders, facial weakness, winging of scapulae, proximal arm wasting. It is slowly progressive.There is an increased incidence of cardiac arrhythmias suggesting that conduction tissue may be involved. Uneventful anaesthesia using atracurium Dressner et al 1989 , and vecuronium Nitahara et al 1999 , showed no increased sensitivity, but more rapid recovery times than normal. 4. Oculopharyngeal Landrum...

Anaesthesia Management In Patient With Hereditary Spherocytosis

Surgery should be avoided in the presence of a haemolytic crisis. 2. Prophylaxis before and after splenectomy. Three vaccines should be given, preferably 2 weeks before splenectomy Pneumovax 0.5 ml sc or im, and repeat once between 5 and 10 years later. HIB vaccine 0.5 ml sc or im once only. Mengivac A C 0.5 ml sc or im, or Meningitec C 0.5 ml im. Repeat every 2 years. Continuous antibiotic prophylaxis benzylpenicillin 600 mg 12 hrly iv or oral penicillin V 250 mg 12 hrly. If penicillin...

Osborne Glycopyrrolate Agitation

Unexpected delayed recovery from anaesthesia, stupor, restlessness, agitation, amnesia, hallucinations, respiratory depression, confusion, or seizures. In the perioperative period, after premedication with morphine 10 mg and hyoscine 0.4 mg, a young man developed hyperthermia 39.3 C increasing to 42 C , tachycardia, and confusion which resolved immediately after physostigmine 3 mg Torline 1992 .A young woman given glycopyrrolate 0.2 mg became hot and dry and developed headache, tachycardia,...

Cd Co

Insulin resistance occurs 3 in a number of circumstances. Normally the u ratio of insulin to glucose required is 15 u insulin to 500 ml 10 dextrose. In patients with severe infection, obesity, liver disease, steroid therapy, or undergoing cardiopulmonary bypass, the dose of insulin may have to be increased by up to four times the normal ratio. 7. Impaired conscious level in diabetics. Causes include hypoglycaemia, diabetic ketoacidosis, hyperglycaemic hyperosmolar...

Pierre Robin syndrome and the

A rare syndrome in which the combination of severe micrognathia and posterior prolapse of the tongue results in respiratory obstruction in infancy, failure to thrive, and, occasionally, cor pulmonale. Other congenital abnormalities, such as cleft palate and oesophageal atresia, may occur. The 'Robin sequence', a term that is increasingly being used in the specialist surgical literature, describes a spectrum of anomalies, the common features of which include mandibular hypoplasia, glossoptosis...

Tuberous sclerosis

A neurocutaneous disease associated with hamartomas in multiple organ systems, skin lesions, and learning difficulties. Skeletal muscle is not involved. It is an autosomal dominant condition and recent genetic linkage studies have implicated abnormalities of the 9q34 protein encoded by the tuberin and hamartin genes. Both are tumour suppressor genes on chromosomes 9 and 16 O'Callaghan 1999 . For full diagnostic criteria, see Webb and Osbourne 1995 . 1. Cutaneous. Facial and ungual angiofibroma....

Sarcoidosis

A multisystem, granulomatous disorder, of variable natural history and prognosis, most frequently presenting in young adults with bilateral hilar lymphadenopathy, pulmonary infiltration, cutaneous and ocular lesions. In some, an acute onset may resolve spontaneously in others, slow onset may herald progressive disease with serious complications, such as pulmonary fibrosis, blindness, cardiac involvement, nephrocalcinosis, and renal failure. Heart and lung involvement are the most significant...

Management

In severe hypothyroidism, elective surgery should be postponed whilst treatment is instituted.There is some argument about the management of patients requiring coronary artery bypass surgery. Although suggestions have been made that treatment should not be given before surgery, for fear of precipitating cardiac ischaemia or heart failure Finlayson amp Kaplan 1982, Drucker amp Borrow 1985,Vanderpump et al 1996 , hypothyroidism is not protective in cardiovascular disease and untreated patients...

Mucopolysaccharidoses MPS

A group of inherited connective tissue syndromes that result from enzyme deficiencies. The mucopolysaccharides or glycoaminoglycans are constituents of connective tissue, and are made up of repeating disaccharide units connected to protein.They are normally broken down in the cell lysosomes to monosaccharides and amino acids. In the absence of certain enzymes, accumulation of intermediate products of degradation process takes place.These substances increase cell size and cause impairment of...

Cushings syndrome and Cushings disease

Cushing's syndrome is the general term used for a disorder caused by excess circulating glucocorticoid. Cushing's disease specifies one of its causes that of pituitary-dependent adrenal hyperplasia, secondary to ACTH secretion.This accounts for about 70 80 of cases of Cushing's syndrome. Other important causes are adrenal cortical tumour 5 10 , and ectopic ACTH producing tumour 5 10 .The diagnosis and treatment of Cushing's syndrome, particularly during pregnancy, is more complex than...

Failure To Control The Airway In A Patient With Hunter S Syndrome

As usual, and that it, like the oropharyngeal airway, can push the epiglottis over the laryngeal inlet causing obstruction.As has already been pointed out, the laryngeal mask airway has limited value if the obstruction lies at glottic level or below. 8. Local anaesthetic techniques should be considered. Spinal anaesthesia has been reported in a patient with a previous failed intubation Sj gren amp Pedersen 1986 . Combined continuous spinal analgesia and general anaesthesia were used for upper...

Marfan syndrome

An autosomal dominant, inherited condition involving a connective tissue deficit, secondary to mutations within the fibrillin gene on chromosome 15. It affects the microfibrillar component of elastic fibres.The abnormal fibrillin alters the functional relationship between blood flow and vascular endothelial cell response Westaby 1999 .The tensile strength of collagen is reduced, while its elasticity is increased and the wall becomes weakened. Skeletal, cardiovascular and ocular features occur....

Intraoperative precautions

Use of latex-free anaesthetic masks, ECG electrodes and wires, blood pressure cuffs, self-inflating bag, elastic bandages, catheters, ileostomy bags, instrument mats, adhesive tapes, dental instruments and material, rectal balloons, airborne latex proteins from glove powder. Powderless gloves should be used and drugs should not be drawn from ampoules with rubber stoppers. 2. Each department should have a database of latex-free equipment Dakin amp Yentis 1998 . Ballantine JC, Brown E 1995...

Coarctation of the aorta adult

A congenital narrowing of the aorta that may be pre- or post-ductal.The preductal form is usually a long, narrow segment, and is associated with other cardiac defects.This type generally presents with heart failure before the age of 1 year and requires treatment in a paediatric cardiac surgical unit. It will not be considered further here.The postductal form, however, is often asymptomatic, and the patient may present in later life for surgery of some other condition, or for correction of the...

Scleroderma

A spectrum of diseases involving abnormal collagen deposition and microvascular changes in the skin and other organs.There is synthesis and deposition of excessive extracellular matrix and the presence of vascular anti-GBM glomerular basement membrane antibodies McHugh 1994 .Autoantibodies define subsets of the disease.These include anti-centromere antibody ACA and anti-topoisomerase-1 antibody ATA . Limited cutaneous scleroderma, previously known as CREST syndrome Calcinosis, Raynaud's, o...

Gauchers disease

An inherited sphingolipid storage disease in which there is a deficiency of a lysosomal enzyme, glucocerebrosidase, resulting in pathological storage of glycolipid in the macrophages of the reticuloendothelial system. There are three types, each with different features type 1 about 99 of cases , type 2 infantile, results in early death , and type 3 juvenile .There is a high incidence in Ashkenazi Jews Zimran et al 1992 . Enzyme therapy decreases the signs and symptoms, but is associated with...

Vipoma

One of the APUDomas Amine Precursor Uptake and Decarboxylation that secretes vasoactive intestinal polypeptide VIP , but may also produce other hormones. Tumours in adults are most commonly of pancreatic endocrine origin, whereas in children they are usually ganglioneuroblastomas arising from the sympathetic chain.Vipomas can also occur as a manifestation of the MEN 1 syndrome Thakker 1998 .VIP is one of a number of hormones that can be secreted by bronchial carcinomas, neuroblastomas, and...

Mitral stenosis

Normal left ventricular filling is restricted by the decreased area across the stenosed valve.The normal area of the valve is 4 cm2. Symptoms appear when this is reduced to about 2.5 cm2 below 1 cm2 the symptoms are severe. Compensation is normally achieved by increasing the pressure gradient across the mitral valve, and is dependent upon atrial contraction and the duration of diastole. Decompensation often begins with the onset of atrial fibrillation, associated with a fast ventricular rate....

Masseter muscle rigidity MMR

Masseter muscle spasm is a clinical diagnosis it has been defined as jaw tightness to a degree that it interferes with tracheal intubation, and occurs despite usually adequate doses of suxamethonium Saddler et al 1990 . Since MMR occurs in 50 of patients who are MHS, it was assumed to be an exclusive sign presaging an MH crisis. However, it is now known that an increase in masseter muscle tone may be a normal response to suxamethonium. In a study of 50 healthy patients, the majority showed...

Wegeners granulomatosis

A systemic granulomatous vasculitis, usually affecting the age group 20 40 years, in which granulomas of the upper and lower respiratory tract are associated with a focal necrotising glomerulonephritis, and a widespread vasculitis involving other organs.The clinical features overlap with microscopic polyarteritis, and the antineutrophil cytoplasmic antibody is positive in both.A survey of 85 cases showed that all patients had either upper or lower respiratory tract involvement, and 85 had...

Hereditary spherocytosis

A familial haemolytic anaemia of autosomal dominant inheritance in which premature destruction of intrinsically abnormal erythrocytes occurs in the spleen. Specific molecular defects have been found.The clinical severity of the disease varies considerably Eber et al 1990 .Anaesthesia may be required for splenectomy and or cholecystectomy. However, a conservative approach to splenectomy is being adopted avoidance of surgery in those with mild to moderate disease is now recommended. After...

Air embolism

Air embolism can occur in any situation in which there is an open vein and a subatmospheric pressure.When a patient is in an upright position, air inadvertently entering the venous system will normally be carried to the right side of the heart, where it localises initially at the junction of the right atrium and the superior vena cava. Some air may remain in the upper part of the right atrium, while the rest is carried through the tricuspid valve and into the pulmonary artery. There is...

Presentation

Cardiovascular collapse usually takes place immediately after the epidural injection, although delays of up to 45 min have been reported Woerth et al 1977 .Three cases of total spinal anaesthesia occurred when epidural injections of local anaesthetic were given into the interspace adjacent to an inadvertent dural perforation Hodgkinson 1981 . In all three incidents, the patients were in active labour. It was suggested that frequent uterine contractions can result in...

Hypercalcaemia

When artefactual causes of an increased serum calcium level have been excluded, the commonest causes of hypercalcaemia are malignancy and hyperparathyroidism. Sarcoidosis, thyrotoxicosis and vitamin D toxicity are uncommon. Other causes are extremely rare. Occasionally a patient with hypercalcaemia may present for anaesthesia. Severe hypercalcaemia gt 3.2 mmol l 1 may be dangerous and, in consultation with a physician, urgent lowering of the level may be required.

Carotid body tumour

One of a group known as the paraganglionomas, which are associated with the sympathetic nervous system. The cells of the carotid body normally act as chemoreceptors, but in common with other APUD cells, can secrete a variety of amines and peptides. Most carotid body tumours are nonfunctional, but occasionally they secrete norepinephrine noradrenaline , dopamine, calcitonin, or ACTH.They may be malignant, may enlarge and invade locally, and can produce metastases....

Multiple endocrine neoplasia see

A group of dominantly inherited cancer syndromes associated with mutations of tumour suppressor genes. Multiple endocrine neoplasia MEN is defined as the occurrence of tumours in two or more endocrine glands Thakker 1998 . MEN 1 An autosomal dominant condition in which the tumour suppressor locus is on chromosome 11 and the gene 'menin' has been identified Heath 1998 . MEN 2A Caused by mutations of the receptor tyrosine kinase RET proto-oncogene on chromosome 10. MEN 2B Also caused by mutations...

Protein C deficiency

In the normal subject, protein C is an essential anticoagulant. It acts by selective inhibition of activated Factors V and VIII, and by stimulation of fibrinolysis. Synthesis occurs in the liver and is vitamin K dependent. Inherited and acquired deficiencies may occur. Since the first report in 1981 by Griffin et al, a number of families have been described in which relatively young members have had recurrent spontaneous venous thromboses in association with reduced levels between 35 and 65 of...

Turners syndrome

A syndrome associated with a sex chromosome abnormality, it includes gonadal dysgenesis, primary amenorrhoea, skeletal, renal, cardiovascular and other anomalies. Cardiovascular complications are the single source of increased mortality and may be associated with aortic wall weakness.Those with the 45,X karyotype were particularly susceptible Gotzsche et al 1994 .There is a high incidence of sternal abnormalities, insulin resistance, and neoplastic conditions Saenger 1996 . 1. Skeletal...

Carnitine palmitoyl transferase deficiency CPTD

An autosomal recessive, metabolic myopathy involving an inability to use fatty acids for energy production in muscle. It is secondary to deficencies of CPT1 or CPT2, enzymes involved mitochondrial fatty acid oxidation Schaefer et al 1997 . It is thought to be an underdiagnosed condition, because episodes of muscle cramps and red urine following intense exercise may be ignored Katzir et al 1996 . It is characterised by episodes of rhabdomyolysis, myoglobinuria, and lipid accumulation. Carnitine...

Hyponatraemia

Hyponatraemia is of concern because of increasing evidence that it is a cause of significant morbidity and mortality in the hospital population Arieff 1998, Gill amp Leese 1998, Knochel 1999, Lane amp Allen 1999 . Even more worrying is the iatrogenic contribution to its development. There have been several studies of the distribution of low sodium levels in hospital. Flear et al 1981 showed that 15.2 of patients had levels below 134 mmoll-1, 4.9 below 130mmoll-1, 1.2 below 125 mmoll-1 and 0.2...

Marcus Gunn jaw winking phenomenon

A rare congenital abnormality in which there appears to be abnormal connections between the external pterygoid and ocular muscles.This results in ptosis, which can be partly corrected by the patient either opening the jaw, or moving it to the contralateral side.A number of other abnormal reflexes may be present.The Marcus Gunn phenomenon can be associated with other defects, in particular in the congenital fibrosis syndrome, a form of myopathy associated with external ophthalmoplegia Brodsky...

Polycythaemia vera

Polycythaemia is a general term for an increased haemoglobin Hb gt 17gdl-1 in men and gt 16g dl-1 in women , an increased red cell count, or haematocrit gt 0.51 in men and gt 0.47 in women . It can be relative in which there is an increased Hb and PCV, a normal red cell mass but a reduction in plasma volume , apparent an increased Hb and PCV, a normal red cell mass and normal plasma volume , or absolute in which the increased Hb and PCV is associated with an increased red cell mass . Apparent...

Diagnosis

Diagnosis can be made in a variety of ways. It may be demonstrated on a CT scan, DVI, MRI, or an aortogram. A femoral catheter can be passed into the IVC to show increased pressure, oxygen content or saturation. Fibreoptic oximetry was used to confirm the diagnosis in a patient following resection of an unruptured aneurysm Khan et al 1992 .An oximetric flotation catheter was passed up the femoral vein.A damped arterial waveform was identified, that was maximal at 15 cm, and increasing oxygen...

Phenylketonuria

One of a group of inborn errors of amino acid metabolism of autosomal recessive inheritance, that involves the essential amino acid, phenylalanine.There is absence of phenylalanine hydroxylase, which catalyses the conversion of phenylalanine to tyrosine, a reaction that only takes place in the liver, kidney, and pancreas. There is impairment of protein synthesis, and the accumulation of phenylalanine in the blood and urine inhibits a number of enzyme systems. There are decreased catecholamine...

Kawasaki disease mucocutaneous

An acute inflammatory illness of young children manifesting as a systemic febrile vasculitis. In the UK it is relatively rare, but it is three times more common in the USA and 30 times more common in Japan.The aetiology is not known, but bacterial superantigen toxin may be involved, similar to the mechanism in staphylococcal toxic shock syndrome.A rapidly reversible process secondary to toxins or cytokines is suggested by the fact that myocardial contractility was seen to improve over a period...