bluuebunny/crossref_metadata_2025 huggingface dataset:
Addison's disease, hypertension, renal and hepatic microthrombosis in ‘primary’ antiphospholipid syndrome
Authors: Inam S , Sidki K , al-Marshedy A R , Judzewitsch R
Published: December 2008

We describe a 14 year old boy with antiphospholipid syndrome who initially presented at the age of 10 with recurrent loin pain, fever, weight loss, leucocytosis, thrombocytopenia, hypertension and haematuria. He had primary adrenal insufficiency with bilaterally enlarged adrenals on computed tomographic (CT) scan consistent with adrenal infarction. Renal and liver biopsies showed microthrombi in the glomerular capillaries and hepatic sinusoids respectively. The case is unusual in that hypertension rather than hypotension was dominant and a CT scan was consistent with bilateral adrenal infarction without haemorrhage. He represented with evidence of persistent hypertension with glomerulosclerosis and glomerular microthrombi on repeat renal biopsy. He continues to have permanent adrenal insufficiency with complete atrophy of his adrenals.
Granulomatous enteropathy in common variable immunodeficiency: a cause of chronic diarrhoea
Authors: Mike N , Hansel T T , Newman J , Asquith P
Published: December 2008

Gastrointestinal disease is a well recognized feature in patients with common variable immunodeficiency, and is often due to infection with a variety of organisms. Symptoms usually improve with appropriate antibiotic therapy and replacement gammaglobulin. We describe three middle-aged female patients with common variable immunodeficiency who had protracted diarrhoea and weight loss. Despite extensive investigation no infectious cause was found.

All patients had granulomas distributed throughout the gastrointestinal tract, but no features of inflammatory bowel disease. There was a poor response to gammaglobulin replacement therapy, antibiotics or symptomatic treatment. We suggest that granulomatous enteropathy is another gastrointestinal manifestation of common variable immunodeficiency.
Low dose danazol in the treatment of the premenstrual syndrome
Authors: Deeny M , Hawthorn R , Hart D McKay
Published: December 2008

This double-blind, randomized, crossover study compared the efficacy and safety of danazol (100 mg twice daily) with matching placebo in the treatment of severe premenstrual syndrome. Nineteen patients were randomly allocated to receive danazol for 3 months followed by placebo, and 18 to receive treatment in the reverse order.

Assessments of overall condition showed improvement to be statistically significantly more likely with danazol than with placebo (P<0.001) after 3 months' treatment. Furthermore, daily visual analogue scale assessments demonstrated statistically significantly better premenstrual scores with danazol in comparison to placebo for breast discomfort, irritability, depression, anxiety, mood swings, crying, depressed libido and abdominal swelling.

It is concluded that danazol provides effective and generally well tolerated treatment for severe premenstrual syndrome.
Giant thymic carcinoid
Authors: John L C H , Hornick P , Lang S , Wallis J , Edmondson S J
Published: December 2008

Thymic carcinoid is a rare tumour. It may present with ectopic endocrine secretion or with symptoms of compression as a result of its size. A case is reported which presented with symptoms of compression where the size of the tumour was uniquely large such as to warrant the term giant thymic carcinoid. The typical histological features are described, together with its possible origin and its likely prognosis.
Atrial septal defect presenting as recurrent primary amoebic lung abscess
Authors: Sharma M , Mehta H , Sharma S K
Published: December 2008

A middle-aged female with an atrial septal defect (secundum type) presented with a primary pulmonary amoebic abscess. She was successfully treated with antiamoebic therapy. One year later she presented with a similar lung abscess which again responded to antiamboebic treatment. Attention is drawn to the fact that a patient with a left to right shunt can present with a recurrent rare primary parasitic infection of the lung.
Massive gastrointestinal haemorrhage associated with ileal lymphoid hyperplasia in Gaucher's disease
Authors: Jones D R , Hoffman J , Downie R , Haqqani M
Published: December 2008

A case report of adult type I Gaucher's disease is described, with profound terminal ileal haemorrhage in association with ileal lymphoid hyperplasia. Peroperative ileal endoscopy localized the segment involved allowing an appropriate resection and successful outcome for the patient.
Localized primary sclerosing cholangitis mimicking a cholecystectomy stricture relieved by an endoprosthesis
Authors: Oren R , Goldin E , Harats N , Libson E , Shouval D
Published: December 2008

Primary sclerosing cholangitis presenting as a localized stricture affecting a segment of the extrahepatic biliary tree is rarely found. We describe the case of a 39 year old woman with obstructive jaundice, in whom this diagnosis was proven by endoscopic retrograde cholangiography. An endoprosthesis was endoscopically introduced through the stenotic area which led to a dramatic improvement enabling a successful liver transplantation after 2 years.
The successful surgical repair of a ruptured interventricular septum following a myocardial infarct in an 89 year old man
Authors: Grocott-Mason R M , Yates A , Wray R
Published: December 2008

This case report describes the successful repair of an acquired ventricular septal defect following an anterior full thickness myocardial infarct in an 89 year old man. Four months after the infarct the patient was in severe congestive cardiac failure (NYHA Class IV), despite medical treatment, with signs of a ventricular septal defect. This was confirmed by echodoppler and cardiac catheterization. Surgical repair of the ventricular septal defect was performed. He made an uncomplicated recovery and two and a half years later is well and active. We believe he is the oldest patient who has had this operation successfully.
Crescentic transformation in primary membranous glomerulonephritis
Authors: Kwan J T C , Moore R H , Dodd S M , Cunningham J
Published: December 2008

A 31 year old man first developed steroid-resistant idiopathic membranous glomerulonephritis in 1981. Stable normal renal function was maintained until August 1988 when he suffered a clinical relapse with heavy proteinuria and declining renal function. Immunosuppressive therapy with prednisolone and cyclophosphamide was instituted in an attempt to arrest this relapse. Despite this, he later developed acute renal failure with histological evidence of crescentic transformation of his nephritis. This unusual transformation was not associated with features of systemic vasculitis or positive anti-glomerular basement membrane and anti-neutrophil cytoplasmic antibodies.
A resistant case of neurocystercercosis
Authors: Chong M S , Hawkins C P , Cook G C , Hawkes C H , Kocen R S
Published: December 2008

A case of neurocystercercosis resistant to treatment with repeated courses of albendazole and praziquantel (even in combination), leading to deterioration in clinical condition is described.
Permanent pacemakers in nonagenarians
Authors: Elizabeth J E , Green G J
Published: December 2008

We report our experience of 20 patients who have had permanent pacemakers implanted for the first time after the age of 90. The main indications were syncope or presyncope which occurred in 15 patients. Complete heart block was present in 14 patients. Syncopal and presyncopal symptoms were cured in 73%. Nonagenarians paced for complete heart block can expect to survive for as long as others of the same age without heart block. Permanent pacing is an effective and appropriate treatment in the very old.
Massive chloroquine overdose—a survivor
Authors: Stiff G , Robinson D , Cugnoni H L , Touquet R , Dalton A M
Published: December 2008

Large chloroquine overdoses are almost inevitably fatal. We describe the presentation and management of a patient with such an overdose who survived, due to general supportive care and new specific treatment.
Diverticulum of a Meckel's diverticulum containing a stone
Authors: Gibney E J
Published: December 2008

An 18 year old girl presented with acute abdominal pain and a calcified opacity in the pelvis. She proved to have a Meckel's diverticulum which had a secondary diverticulum at its apex, containing a stone. Meckel's stones are uncommon and diverticula of a Meckel's diverticulum exceedingly rare. This is only the second recorded case of such a diverticulum containing a stone, and the first to describe a calcified stone in this location.
T cell dominated inflammatory reactions in the bronchioles of asymptomatic asthmatics are also present in the nasal mucosa
Authors: Poulter L W , Norris A , Power C , Condez A , Burnes H , Schmekel B , Burke C
Published: December 2008

Endobronchial and nasal mucosa biopsies were obtained from 5 patients with clinically-stable, diagnosed asthma (ATS criteria). A comparison was made of the presence and distribution of immunocompetent lymphocytes and macrophages within each sample. The distribution of immunocompetent cells within the nasal biopsies of the asthmatic patients reflected a very similar inflammatory infiltrate to that seen in the bronchial biopsies. Significantly raised numbers of T lymphocytes, CD45RO + lymphocytes, RFD1 + macrophage-like cells and RFD7 + macrophages were seen in both the nasal mucosa and the bronchial biopsies. Increases in HLA-DR expression were also seen in the nasal mucosa biopsies from asthmatics although the increases over normal did not reach statistical significance. It is concluded that inflammation present in the nasal mucosa of asymptomatic asthmatics exhibits cellular characteristics also seen in endobronchial biopsies. This observation offers the possibility that mucosal biopsy may be an alternative and less invasive approach for studying the cells involved in the bronchial inflammatory reaction that possibly predisposes asthmatics to bronchial hyper-responsiveness.
Small bowel function after aortic surgery
Authors: Barker S G E , Dodds R D A , Middlemiss A , Bennett C E , Russell M H , Sellick B C , Thomas M H
Published: December 2008

One cause of post-operative morbidity in the elective repair of abdominal aortic aneurysms is the development of a paralytic or ‘adynamic’ ileus. In a series of 20 consecutive patients undergoing such a procedure, the maintenance of small bowel motility and absorptive capacity in the immediate post-operative period was assessed using barium sulphate and xylose passed down a naso-duodenal tube sited at the time of surgery. This simple study demonstrated that small bowel function was preserved in all cases, and hence that patients could be fed enterally via naso-duodenal tube (in particular using very low residue formulae) rather than using costly parenteral regimens, should an ileus persist.
Melioidosis in a patient from Bangladesh
Authors: Kibbler C C , Roberts C M , Ridgway G L , Spiro S G
Published: December 2008

A 54 year old Bangladeshi man presented with a history and chest X-ray appearances suggestive of pulmonary tuberculosis. Following deterioration 4 weeks later, he required ventilation. Although a blood culture isolate was subsequently found to be Pseudomonas pseudomallei, it was initially misidentified and dismissed as a contaminant. Further cultures demonstrated the organism, but the patient died, despite treatment with ceftazidime.

The case illustrates the importance of taking a detailed travel history and having a high index of suspicion in patients from South East Asia and the Indian sub-continent, including Bangladesh, where the disease has not previously been considered endemic.
Alcohol and Indian porphyrics
Authors: Saksena H C , Panwar R B , Rajvanshi P , Sabir M , Suri M
Published: December 2008

The role of alcohol as the precipitating factor in the induction of acute attacks of acute intermittent porphyria was studied in an Indian population. Thirty-four teetotal patients with acute intermittent porphyria, in remission, were given 60 ml of 30% ethanol. Except for two patients, all had negative Watson-Schwartz tests prior to the alcohol. Within 24 hours, the Watson-Schwartz test became positive in 16 of these 32 patients (50%). In 8 out of the 34 patients (23.5%) a clinical attack was precipitated, including both patients who had a positive Watson-Schwartz test prior to the alcohol.

It was concluded that alcohol does precipitate an acute attack in a significant percentage of patients of Indian origin with acute intermittent porphyria. Patients already excreting porphobilinogen are at a greater risk of developing an acute attack on alcohol ingestion. This study is the first from India and probably first of its kind to be reported from any country.
Three cases of alpha-1-antitrypsin deficiency in the elderly
Authors: Jack C I A , Evans C C
Published: December 2008

We report three elderly patients who presented with increasing dyspnoea on exertion and radiological evidence of basal bullous emphysema. On further investigation they were found to have alpha-1-antitrypsin deficiency.

We emphasize that alpha-1-antitrypsin deficiency can present much later than is usually the case especially if the patient is a life-long non-smoker.
The practice of rectal examination
Authors: Ng D P K , Mayberry J F , McIntyre A S , Long R G
Published: December 2008

Rectal (both digital and rigid sigmoidoscopic) examination is an important part of the clinical examination. The aim of this study was to find out the opinions of the patients to this routine examination at the time of referral by their general practitioner and during their first hospital interview. Assessment of the value for the rectal examination was also examined.

We questioned 103 patients attending their second out-patient interview through a simple questionnaire. We discovered that patients considered awareness and explanation important: patients preferred to be told of the possibility of rectal examination prior to hospital consultation. Explanation of the method and reason was expected from hospital doctors. Formal consent was expected; informed verbal consent should be sufficient. Where logistically possible, a chaperone should always be present. Rectal examination would have facilitated the diagnosis in 47% of the patients examined. Thus, rectal examination should be performed on all patients where symptoms are referable to the lower gastrointestinal tract and where a possible diagnosis may be made or facilitated.
Rapid onset of blindness due to meningeal carcinomatosis from an oesophageal adenocarcinoma
Authors: Teare J P , Whitehead M , Rake M O , Coker R J
Published: December 2008

We report a 49 year old man who presented with left leg weakness, frontal headache, impaired concentration and dysphagia. He was thought to have a benign oesophageal stricture on barium swallow and oesophagoscopy though this was not initially biopsied. During admission his vision deteriorated so that he could only recognize bright light. Repeat oesophagoscopy demonstrated an oesophageal adenocarcinoma. The diagnosis of meningeal carcinomatosis was confirmed at post-mortem.

Sudden bilateral blindness is a common feature of meningeal carcinomatosis but, as this is rare, it is not commonly considered in the differential diagnosis of visual loss. The visual loss can be explained by vascular insufficiency in association with tumour cuffing of the subarachnoid space of the optic nerve sheath, by neuronal toxins, or other, as yet unknown, mechanisms.
Bickerstaff's brainstem encephalitis associated with typhoid fever
Authors: Wali G M
Published: December 2008

A 14 year old boy developed the syndrome of Bickerstaff's brainstem encephalitis during the course of bacteriologically proved typhoid fever. The clinical course and the results of various neurological investigations are detailed. This report adds a further manifestation to the published neuropsychiatric complications of typhoid fever.
Coeliac disease presenting with cerebellar degeneration
Authors: Hermaszewski R A , Rigby S , Dalgleish A G
Published: December 2008

A case of rapidly progressive cerebellar degeneration with bilateral sixth nerve palsies is described in whom investigation revealed the presence of unsuspected coeliac disease. In spite of treatment with a gluten free diet, rapid fatal deterioration occurred. Coeliac disease should be considered in patients with encephalopathy of obscure origin.
The clinical features of late onset anorexia nervosa
Authors: Joughin N A , Crisp A H , Gowers S G , Bhat A V
Published: December 2008

This study examines clinical features of late onset anorexia nervosa. This involved the scrutiny of a large database of patients with anorexia nervosa comprising data gathered at standardized initial assessments over the period 1960–1990. Patients with a late onset were compared to other selected patient samples.

The population comprised 12 patients with a first onset of anorexia nervosa at or after the age of 30, 415 patients with an onset after 15 but before 20 and 9 patients with an onset after 15 but before 20 and matched for age at presentation with the late onset group.

Features studied included age at menarche, age at onset of anorexia nervosa, age at presentation, duration of illness, weight at presentation, lowest adult weight, highest weight, weight at onset of illness, marital status and parity.

Patients with an onset of anorexia nervosa after the age 30 comprised 2% of the total female patient sample. Though such patients were rare, their clinical features were very similar to those of typical patients with adolescent onset. Notably, young and late onset patients had similar durations of illness prior to presentation, and similar proportions had bulimia and defensive vomiting. Feared sexuality, no longer necessary for childbearing, emerged as being of apparent aetiological significance in the late onset group, with the disorder embodying its rejection, as often also seems to be the case with earlier onset. The late onset cases were hard to diagnose and had a poor outcome.

The study underlines the importance of considering the diagnosis of anorexia nervosa in older patients, even if there is no earlier history of anorexia nervosa. Such patients are likely to find it easier to conceal the psychological origins of their problem behind the possibility of a primary physical illness, or behind psychiatric diagnoses such as depression, the treatment of which may not threaten their avoidance of normal body weight.
Pneumoperitoneum without peritonitis
Authors: Daly B D , Guthrie J A , Couse N F
Published: December 2008

We present our experience with 5 patients, all of whom were noted to have radiological signs of pneumoperitoneum in the absence of clinical features of peritonitis. All 5 cases were eventually shown to be due to causes not requiring surgery. We review the numerous unusual causes of pneumoperitoneum which do not require urgent surgical intervention and emphasize their importance in cases where the absence of signs of peritonitis may cause diagnostic difficulty.
Lipoprotein lipase deficiency due to long-term heparinization presenting as severe hypertriglyceridaemia in pregnancy
Authors: Watts G F , Cameron J , Henderson A , Richmond W
Published: December 2008

A case of severe hypertriglyceridaemia presenting in the third trimester of pregnancy in a woman on long-term heparin prophylaxis is described. The hypertriglyceridaemia was attributed to impaired clearance of triglyceride-rich lipoprotein particles secondary to heparin-induced reduction in the activity of the lipolytic enzyme, lipoprotein lipase.
Fatal fulminant hepatic failure in a 'solvent abuser'
Authors: McIntyre A S , Long R G
Published: December 2008

The case of a 17 year old abuser of butane aerosols who developed fulminant hepatic failure after taking a proprietary engine or carburetor cleaner is described. Fatalities as a result of liver failure due to volatile hydrocarbons or solvents have not previously been reported. The likely toxins included isopropyl alcohol, methyl amyl alcohol, butylated hydroxytoluene as well as petroleum products, and evidence for their toxicity is reviewed. The possibility of increased susceptibility to hepatotoxins after enzyme induction by abuse of volatile agents is discussed. Hepatic dysfunction should be sought in patients who abuse solvents or volatile hydrocarbons and supportive measures instituted promptly when required.
Organ limited amyloidosis with lymphadenopathy
Authors: Dalton H R , Featherstone T , Athanasou N
Published: December 2008

Hilar and mediastinal lymphadenopathy associated with localized pulmonary amyloid is very rare. We describe two cases of this unusual combination, one of endobronchial amyloid with adenopathy and the other of nodular parenchymal amyloid with hilar nodes. In both these cases the nodes contained calcification, and in the nodular parenchymal case in particular, this appearance is highly suggestive of pulmonary amyloid.
Giant pre-sacral neurofibroma
Authors: Agarwal M , Azzopardi A , Mufti G R
Published: December 2008

We report a case of pre-sacral neurofibroma diagnosed on a routine medical examination. The pathological features and management criteria of these rare tumours are discussed.
Oesophageal tuberculosis: a review of eleven cases
Authors: Mokoena T , Shama D M , Ngakane H , Bryer J V
Published: December 2008

Tuberculous infection of the oesophagus is rare. This is confirmed by our present review of cases managed in our teaching hospitals over a period of 18 years which uncovered only 11 patients. The main presentation is that of dysphagia whose algorithm of investigation should seek to differentiate tuberculosis from carcinoma, the more common cause of this symptom. Of the 11 patients, 9 presented with dysphagia while 2 had haemorrhage; 7 had an abnormal plain chest radiograph, of whom 4 had a mediastinal mass lesion (3 were lymphadenopathy and one an abscess). All but one had an abnormal radio-contrast oesophagogram, including a mediastinal sinus in two and a traction diverticulum in another two. The mainstay of investigation was oesophagoscopy through which diagnostic biopsy material was obtained in half of the patients. In the other half diagnosis was by either biopsy of associated mediastinal (3) or cervical (1) lymph node masses or by acid fast bacilli positive sputum (1). The diagnosis was established post-mortem in one patient.

Treatment was primarily non-operative with standard anti-tuberculosis drug therapy. Two patients underwent a diagnostic thoracotomy and one a drainage of mediastinal abscess together with resection and repair of oesophago-mediastinal sinus during the early part of the series. Outcome of management was very rewarding in 9 patients and death occurred in 2 patients, one of whom had his anti-tuberculosis drug therapy interrupted by severe hepatitis B virus infection. The other death occurred in a patient whose haemorrhage from an aorta-oesophageal fistula was not established ante-mortem. It is recommended that when biopsy material of the oesophagus is unobtainable or non-diagnostic in patients with dysphagia, especially with an abnormal chest radiograph or human immunodeficiency virus infection, effort should be made to obtain biopsy material from associated lymph nodes, even by thoracotomy if necessary, or culture of biopsy from the radiologically abnormal part oesophagus and sputum for mycobacteria, in order to establish the diagnosis of this rare but eminently treatable cause of dysphagia. Clinicians should be aware of tuberculosis of the oesophagus as a possible cause of haematemesis in patients with otherwise unexplained upper gastrointestinal haemorrhage.
Epilepsy--another cause of intermittent fever with confusion
Authors: Chan K M
Published: December 2008

The problem of recurring episodes of fever and confusion in an 83 year old man was found to be due to epilepsy. He was treated with carbamazepine which abolished the attacks. Epilepsy-induced fever is a difficult diagnosis to make, and electroencephalogram can be invaluable in making the diagnosis.
Primary pericardial mesothelioma presenting as tuberculous pericarditis
Authors: Rose D S C , Vigneswaran W T , Bovill B A , Riordan J F , Sapsford R N , Stanbridge R DeL
Published: December 2008

Ante-mortem diagnosis of primary pericardial mesothelioma is very rare. We report a case which presented clinically as tuberculous constrictive pericarditis. The patient underwent pericardial resection with an immediate haemodynamic benefit, although the malignant process progressed, and he died 14 weeks later.
NonEBV-related B-cell lymphoma in a renal transplant patient responding to acyclovir and reduction in immunosuppression
Authors: Wong R C W , Harrison P
Published: December 2008

A case of polyclonal B-cell pharyngeal lymphoma in a renal transplant patient is reported. No evidence of Epstein-Barr virus involvement was detected, but the lymphoma underwent complete remission with acyclovir and reduction in immunosuppression.
Is Asacol as effective as sulphasalazine in maintaining remission of Crohn's disease and ulcerative colitis?
Authors: Nakshabendi Imad M , Duncan Andrew , Russell Robin I
Published: December 2008

To compare the efficacy of Asacol (mesalazine coated with Eudraget-S) as a maintenance therapy with that of sulphasalazine, relapse rates of patients with ulcerative colitis and Crohn's disease, treated with sulphasalazine or Asacol were assessed in a retrospective study. A total of 164 patients were investigated, 127 on sulphasalazine and 37 on Asacol. None of the patients on Asacol was changed from sulphasalazine because of lack of efficacy to sulphasalazine. Relapse rates were measured over a 4 year period. In ulcerative colitis these were sulphasalazine 10/77 (13.0%), Asacol 5/20 (25.0%), NS; in all Crohn's disease patients, sulphasalazine 12/50 (24.0%), Asacol 11/17 (64.7%); P less than 0.0025. In patients with Crohn's disease with ileal involvement, relapse rates were sulphasalazine 9/28 (32.1%), Asacol 9/11 (81.6%), P less than 0.0125; without ileal involvement, sulphasalazine 3/22 (13.6%), Asacol 2/6 (33.4%), NS.

This study suggests that Asacol is as effective as sulphasalazine in maintaining remission in ulcerative colitis and in patients with Crohn's disease without ileal involvement. Sulphasalazine seems to be more effective than Asacol in maintaining remission in patients with Crohn's disease with terminal ileal involvement.
Adult metachromatic leukodystrophy with an unusual relapsing-remitting course
Authors: Sadeh Menachem , Kuritzky Arieh , Ben-David Eitan , Goldhammer Yochanan
Published: December 2008

A 46 year old woman had a relapsing-remitting course of hemiparesis, disorientation, paraparesis and seizures, followed by progressive dementia, spasticity and ataxia. Computed tomography at onset showed a parietotemporal hypodense area with diffuse mottled enhancement obliterating the lateral ventricle. Subsequent scans demonstrated symmetric periventricular non-enhancing hypodensities, progressive ventricular enlargement and atrophy. Adult metachromatic leukodystrophy was diagnosed on the basis of low leukocyte arylsulphatase A level and metachromatic material accumulation at neural nerve biopsy.
Recovery of adrenocortical function following treatment of tuberculous Addison's disease
Authors: Penrice Juliet , Nussey S S
Published: December 2008

We report two cases of Addison's disease occurring in association with proven Mycobacterium tuberculosis infection. Both were treated with modern chemotherapeutic agents and were shown to have developed normal adrenal cortical function after several years. We suggest that adrenal function should be tested after modern anti-tuberculous therapy of this disease.
Use of the SeHCAT test in the investigation of diarrhoea
Authors: Ford G A , Preece J D , Davies I H , Wilkinson S P
Published: December 2008

The SeHCAT test was used to investigate possible bile acid malabsorption in 166 patients presenting to a district general hospital with chronic diarrhoea of uncertain cause. Eighty-four (51%) patients had impaired SeHCAT retention. These included 23 of 28 patients with a possible type I abnormality (terminal ileal resection or disease, previous pelvic radiotherapy), 20 of 74 with a possible type II abnormality (idiopathic diarrhoea), 32 of 45 with a possible type III abnormality (post-cholecystectomy, post-vagotomy), and 9 of 19 with diarrhoea associated with diabetes. Patients with severe bile acid malabsorption demonstrated a good response to cholestyramine whereas the response in patients with a mildly abnormal SeHCAT retention was variable. Bile acid malabsorption is an important cause of diarrhoea in patients presenting with unexplained chronic diarrhoea.
Falciparum malaria-induced hypoglycaemia in a diabetic patient
Authors: Shalev Oded , Tsur Anat , Rahav Galia
Published: December 2008

We report a patient with diabetes mellitus who suffered severe falciparum malaria complicated by profound and persistent hypoglycaemia. The hypoglycaemia evolved before therapy with quinine was begun and resolved with eradication of the parasitaemia. The patient reverted to her baseline hyperglycaemia despite continuation of quinine. This case illustrates the critical role of falciparum malaria in the pathogenesis of malaria-associated hypoglycaemia, rather than quinine-mediated mechanisms. Anticipation of hypoglycaemia in falciparum malaria and its vigorous treatment may improve the poor prognosis associated with this complication.
Pancreatic abscess due to Salmonella typhi
Authors: Garg Pradeep , Parashar Sunil
Published: December 2008

Isolated involvement of the pancreas in Salmonella typhi bacteraemia is rare. A case of pancreatic abscess due to S. typhi is reported which was managed conservatively.
Lymphomatoid granulomatosis presenting as angioedema
Authors: Torrelo Antonio , Martín Manuel , Rocamora Antonio , Allegue Francisco , Ledo Antonio
Published: December 2008

We describe a patient with severe eyelid and lip angioedema lesions in whom biopsy specimens from angioedematous labial mucosa disclosed features of lymphomatoid granulomatosis. To our knowledge, angioedema lesions with characteristic histological findings of lymphomatoid granulomatosis have not been previously described as a presenting sign of this disease.
A case of polycythaemia vera presenting with intramuscular chest wall haematoma
Authors: Kheterpal S , Sayegh F , Cader A
Published: December 2008

A 40 year old male, previously well, presented with a posterior chest wall haematoma. Computerized tomography and ultrasound showed this to be intramuscular. Haematological indices were consistent with a diagnosis of polycythaemia vera. The haematoma and indices responded to hydroxyurea and venesection. This rare presentation of polycythaemia vera in a young person is described and the haemorrhagic complications of polycythaemia vera discussed.
The role of faecal Candida albicans in the pathogenesis of food-intolerant irritable bowel syndrome
Authors: Middleton S. J. , Coley A. , Hunter J. O.
Published: December 2008

Candida albicans was sought in stool samples from 38 patients with irritable bowel syndrome and 20 healthy controls. In only three patients with irritable bowel syndrome was C. albicans discovered and these patients had either recently received antibiotics or the stool sample had been delayed more than 24 hours in transit. C. albicans was isolated from none of the control stool samples. We conclude that C. albicans is not involved in the aetiology of the irritable bowel syndrome.
Endoscopic therapy for bile duct stones in a geriatric population
Authors: Croker John R. , Williams Simon G. , Charlton Michael , Vaira Dino , Dowsett John
Published: December 2008

During the 5 year period to May 1988, 137 consecutive patients (age range, 65-102 years; median 84 years) with a diagnosis of choledocholithiasis, were referred to The Middlesex Hospital Geriatric Department. Endoscopic sphincterotomy was successful in 96.2% of cases and immediate biliary drainage was achieved in all but one of these patients. Stones were cleared endoscopically in 73.3% and surgically in 4.7% of cases. Long-term stenting was employed in 14.3% of patients. The 30 day mortality after endoscopic or surgical treatment was 4.7% (six deaths), although death was probably unrelated to therapy in half the cases. Deaths were due to procedural cardiorespiratory arrest (1), pancreatitis (1), pneumonia (2) and cerebrovascular accident (2). Endoscopic treatment is effective even in a high-risk geriatric population. After sphincterotomy patients with intact gall bladders should be managed expectantly.
Non-secretory multiple myeloma presenting as primary plasma cell leukaemia
Authors: Sureda A. , Pais J. R. , Pascual J. , Pérez Vaquero M. A. , Hernando J. C.
Published: December 2008

A case of non-secretory multiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. Bone pain was the initial clinical manifestation. Laboratory analysis showed 20% of circulating immature plasma cells. Despite the presence of osteolytic lesions, no M-component could be demonstrated in serum protein electrophoresis, and serum and urine immunoelectrophoresis. Bone marrow aspirate demonstrated an 83% infiltration of plasma cells showing various degrees of immaturity. Immunofluorescence with monoclonal antisera demonstrated intracytoplasmic kappa light chains in a high percentage of plasma cells. Immature plasma cells without cellular capacity to synthesize and excrete complete immunoglobulins could be more aggressive, leading to an initial leukaemic process. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features, and response to treatment of this extremely rare association are reviewed.
Acute intermittent porphyria treated by testosterone implant
Authors: Savage M. W. , Reed P. , Orrman-Rossiter S. L. , Weinkove C. , Anderson D. C.
Published: December 2008

The hereditary disorder acute intermittent porphyria is potentially fatal. Many more females present with active disease than males and some have attacks related to their menstrual cycle and pregnancy. We present a female patient who was diagnosed while pregnant at 19 years. She subsequently developed life-threatening attacks pre-menstrually at 24 years; these were associated with weight loss. Initial treatment was with high calorie feeding via a naso-gastric tube, followed by a gastrostomy. Subsequent gonadotrophin suppression with intranasal luteinizing hormone-releasing hormone analogue (buserelin) thrice daily met with limited success. We implanted 100 mg of testosterone subcutaneously in November 1989. The buserelin was discontinued in January 1990 and menses returned 3 months later. There have been no serious attacks since then. Repeat implantation was performed at 6 monthly intervals until her present pregnancy. Baseline biochemical parameters have remained high and unaltered despite treatment although the testosterone has clearly had a marked clinical benefit, without side effects.
Behçet's syndrome in Scotland
Authors: Jankowski J , Crombie I , Jankowski R
Published: December 2008

We present the clinical details and HLA typing of 15 Celtic Caucasian patients (four male, 11 female) with Behçet's syndrome (International Study Group criteria). The males affected were younger than the affected females, and three of these males had severe uveal involvement. Two of the 15 patients had the A2 Bw6 Dr4 haplotype but this did not confer family penetrance. Eight had gastrointestinal involvement: two females required ileostomy, two females had chronic diarrhoea, one female had severe ileitis and oesophageal lesions, two males had peptic ulcers, and one female had a peptic ulcer and primary biliary cirrhosis. All of those who developed gastrointestinal symptoms had either the Dr4 or the Dr7 antigens. This study is the largest HLA survey of Celtic Caucasians with Behçet's syndrome. The clinical features and HLA haplotypes are markedly different from 'Arab' and 'Japanese' varieties of Behçet's syndrome. The expression of the Dr4 and Dr7 antigens in those with gastrointestinal involvement possibly implicates class II antigens (Dr) in the pathogenesis of the manifestations of Behçet's disease in the bowel.
Empty sella developing during thyroxine therapy in a patient with primary hypothyroidism and hyperprolactinaemia
Authors: Kelestimur Fahrettin , Selçuklu Ahmet , Ozcan Nevzat
Published: December 2008

A 35 year old woman presented with severe primary hypothyroidism and galactorrhea. A very high prolactin level was also detected and computerized tomography scan of the sellar region demonstrated an enlarged pituitary gland associated with contrast enhancement. Replacement therapy with thyroxine corrected both biochemical and clinical abnormalities but empty sella developed during this therapy. It is concluded that empty sella may be related to thyroxine-induced shrinkage of lactotroph and/or thyrotroph cell hyperplasia.
Should clinicians accept management responsibilities?
Authors: Welsby P D
Published: December 2008

The British National Health Service is changing and clinicians are being vigorously encouraged to accept managerial roles in addition to patient management.
Stroke in adult polycystic kidney disease
Authors: Rivera Maite , Gonzalo Ana , Gobernado José M , Orte Luis , Quereda Carlos , Ortuño Joaquin
Published: December 2008

In order to assess the incidence of acute cerebrovascular events, 142 patients with adult polycystic kidney disease were retrospectively reviewed. Fourteen patients (9.8%) had 19 cerebral attacks. Six patients (4.2%) had intracranial haemorrhage attacks (three ruptured intracranial aneurysms and three cerebral haemorrhages). Ischaemic events occurred in nine patients (five cerebral infarctions and four transient ischaemic attacks). Patients with ischaemic attacks had a better outcome than patients with haemorrhagic events even when transient ischaemic attacks were excluded. Patients with ruptured intracranial aneurysms were younger. Cerebral complications are an important cause of morbidity and mortality in patients with adult polycystic kidney disease. They can prove disabling prior to or after dialysis and transplantation.
Absence of potentiation of the skin response to intradermal bradykinin by a long-acting angiotensin converting enzyme inhibitor, trandolapril, at conventional antihypertensive dosage in human volunteers: a double-blind, randomized, cross-over, placebo-controlled trial
Authors: Li Kam Wa T C , Cooke E D , Beacham Julie , Turner P
Published: December 2008

A double-blind, randomized, cross-over, placebo-controlled study was carried out to determine the extent and duration of potentiation of the action of bradykinin introduced intradermally by a long-acting novel angiotensin converting enzyme (ACE) inhibitor, trandolapril. The investigations were performed in a temperature and humidity-controlled laboratory. Intradermal injections of 1 microgram, 2.5 micrograms and 5 micrograms of bradykinin and normal saline (as control) were made into the forearm skin of eight healthy normotensive male volunteers aged 21-33 years (mean 28 years) at baseline, 2, 4, 8, 24, 48, 72 and 96 hours after either 2 mg trandolapril or placebo given orally. Skin blood flow outside the induced weal was monitored by laser Doppler flowmetry (mean of recordings at four sites adjacent to the weal within the flare area). Flare area and weal volume were also measured.

Trandolapril reduced the mean arterial pressure. However, there was no evidence that this activity was associated with a potentiation of the cutaneous action of bradykinin.

In conclusion, it would appear that potentiation of the action of bradykinin may not be an important contributing factor to the fall in total peripheral vascular resistance associated with ACE inhibition in humans in the control of hypertension.
Safe use of streptokinase in myocardial infarction in patients aged 75 and over
Authors: Kafetz Kalman , Luder Robert
Published: December 2008

We have given streptokinase to 73 patients aged 75 and over admitted to medical admission wards for elderly people using strict exclusion criteria. This was safe and free from significant adverse events by comparison with other studies. The average age of the patients was 78.2 years so they formed a relatively young group compared with all patients over 75. There was a relatively long delay between presentation at the hospital and receipt of treatment.
Intracerebral haemorrhage after thrombolytic therapy for acute myocardial infarction
Authors: More R S , Vincent R
Published: December 2008

Seven cases of intracerebral haemorrhage were identified following the use of thrombolytic therapy in a total of 710 patients with acute myocardial infarction. Each case was reviewed in detail with particular attention to the presenting symptoms. A confusional state was the commonest initial feature. Increased age and male sex were associated with increased risk of cerebral bleed. The possible role of intravenous heparin in increasing risk and the management of affected cases is discussed. Early recognition and action is emphasized.
Severity of alcoholic liver disease and markers of thyroid and steroid status
Authors: Burra P , Franklyn J A , Ramsden D B , Elias E , Sheppard M C
Published: December 2008

Alcoholic liver disease is associated with abnormalities in circulating levels of thyroid, adrenal and gonadal steroid hormones. The relative importance of ethanol consumption and severity of liver disease in the aetiology of these changes and their relationship to clinical abnormalities are unclear. We studied 31 subjects with alcohol-induced liver disease divided into three groups according to the severity of histological features: fatty change, hepatitis and cirrhosis.

Circulating concentrations of thyroid, adrenal and gonadal steroid hormones, together with their major binding proteins, were measured in all subjects, and changes related to histology and tests of liver function, as well as clinical endocrine status. A reduction in circulating free tri-iodothyronine (fT3) was seen in subjects with alcoholic hepatitis and cirrhosis, in association with normal or reduced levels of thyrotrophin (TSH).

The absence of abnormalities in subjects with fatty change despite similar ethanol intake to the other groups, and correlations between fT3 and liver function tests, suggest that changes in fT3 reflect the severity of underlying liver disease. Similarly, marked increases in circulating cortisol in the hepatitis and cirrhosis groups, and correlations between cortisol and liver function, suggest that changes largely reflect hepatic disease. The absence of clinical features of hypothyroidism or Cushing's syndrome in these groups, despite abnormalities of fT3 and cortisol, suggest an altered tissue sensitivity to hormone effects. In contrast, increases in circulating oestradiol and reductions in testosterone were found in all three groups in males. These findings suggest that both direct effects of ethanol and hepatic dysfunction determine changes in gonadal steroids in males.
Neuromuscular disease, respiratory failure and cor pulmonale
Authors: White John , Bullock R E , Hudgson P , Gibson G J
Published: December 2008

Respiratory muscle weakness is an uncommon cause of chronic respiratory failure and a rare cause of cor pulmonale. The problem may not be apparent unless specific physical signs are sought or appropriate investigations performed. We present three patients who presented diagnostic difficulty for prolonged periods until the presence of respiratory muscle weakness was considered. Once the diagnosis was established treatment with nocturnal nasal intermittent positive pressure ventilation produced a dramatic improvement in symptoms and allowed a return to a near normal lifestyle.
Brucellosis with nephrotic syndrome, nephritis and IgA nephropathy
Authors: Siegelmann Nava , Abraham Abraham S , Rudensky Bernard , Shemesh Ovadia
Published: December 2008

A patient with systemic brucellosis due to Brucella melitensis had severe renal involvement. Clinical features included hypertension, macroscopic haematuria, massive proteinuria of 10 g per 24 hours and azotaemia. Following treatment with antibiotics, the azotaemia resolved and proteinuria decreased to less than 0.5 g per 24 hours, but microscopic haematuria and hypertension persisted. Renal biopsy during recovery revealed IgA nephropathy with minimal mesangial changes, suggesting a causal relation between brucellosis and IgA nephropathy with a reversible nephrotic syndrome.
Further evidence for differential regulation of follicle-stimulating hormone (FSH) and luteinizing hormone (LH): increased FSH and decreased LH levels in a patient with familial pure gonadal dysgenesis
Authors: Popovic V , Micic D , Damjanovic S , Calovic L J , Rolovic Z , Mijovic A , Petakov M , Manojlovic D , Micic J
Published: December 2008

There is experimental evidence that a portion of follicle-stimulating hormone (FSH) secretion is independent of hypothalamic influences. A 29 year old woman with familial pure gonadal dysgenesis developed myelodysplastic syndrome. Endocrine investigations showed discrepancy between serum FSH and luteinizing hormone (LH) levels. FSH levels remained elevated while LH levels decreased. The FSH to LH ratio was 10 (normal 2-2.5). The fall in LH is likely to be due to factor(s) involved directly and specifically in LH synthesis and release. Exogenous LH releasing hormone administration as well as hormonal replacement treatment increased LH levels. The FSH to LH ratio decreased to 7. This case supports the hypothesis of differential regulation of FSH and LH, and that FSH secretion is at least partly autonomous.
Late onset epilepsy in undiagnosed tuberous sclerosis
Authors: Gutowski N J , Murphy R P
Published: December 2008

A 67 year old woman first developed left-sided sensorimotor seizures postoperatively in association with infection and mild hyponatraemia. She was found to have previously unrecognized tuberous sclerosis, with gross pathognomic features: periungual fibromata, facial angiofibromata, calcified subependymal nodules, cortical tubers and multiple renal angiomyolipomata. Her son who was epileptic was also found to have other mild features of tuberous sclerosis as a result. As far as we are aware our patient is the oldest for the first presentation of tuberous sclerosis.
Obstructive uropathy due to extramedullary haematopoiesis in beta thalassaemia/haemoglobin E
Authors: Intragumtornchai Tanin , Arjhansiri Kiat , Posayachinda Makumkrong , Kasantikul Vira
Published: December 2008

An 18 year old woman with beta thalassaemia/haemoglobin E developed a large pelvic tumour resulting in bilateral obstructive uropathy. Technetium-99m sulphur colloid marrow image, computed tomographic scan of the abdomen and needle biopsy of the mass confirmed the diagnosis of extramedullary haematopoiesis. Although radiation is the treatment of choice for decompression, the mass in this patient did not respond satisfactorily due to its multiple area of tumour autoinfarction. Obstructive uropathy due to extramedullary erythropoiesis has not to our knowledge been previously described.
Relationship between smoking, weight and attitudes to weight in adolescent schoolgirls
Authors: Halek C , Kerry S , Humphrey H , Crisp A H , Hughes J M
Published: December 2008

A total of 1,932 schoolgirls aged 11-18 from seven schools in the South London area were surveyed using questionnaires which addressed eating patterns, body weight history, attitudes to body weight and shape, menstrual history and current smoking behaviour. They were also weighed and their height was measured. Twelve per cent of the girls were regular smokers and 10% smoked seven or more cigarettes over a 4 day period. Amongst girls aged 14 and over, 15% smoked regularly and a further 9% occasionally. A significant relationship was found between smoking and weight. Smokers were more likely to be moderately overweight in relation to their peers and to have been worried about their weight at some stage. There were differences between girls in state schools and those in independent schools with regard to smoking behaviour and weight. The findings have implications for anti-smoking strategies and health education generally.