bluuebunny/crossref_metadata_2025_split huggingface dataset:
Late onset epilepsy in undiagnosed tuberous sclerosis
Authors: Gutowski N J , Murphy R P 
Published: December 2008

Summary
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

Summary
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

Summary
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.
17α-Hydroxylase deficiency with persistence of müllerian ducts in a genotypic male and paradoxical aldosterone secretion
Authors: Panesar N S , Yeung V T F , Chan J C N , Shek C C , Nicholls M G , Cockram C S 
Published: December 2008

Summary
We report a case of congenital adrenal hyperplasia due to 17 α-hydroxylase deficiency in a Chinese genotypic male patient. Despite the male genotype, normal female external genitalia were present and with the introduction of cyclical oestrogen therapy withdrawal bleeding occurred, confirming the presence of functional endometrial tissue. We believe this to be the first report of persistent Mullerian duct structures in a genotypic male with 17 alpha-hydroxylase deficiency. It could be explained by either impaired secretion or impaired action of anti-Mullerian hormone. Further, contrary to the usual finding of suppressed aldosterone secretion, this patient had measurable levels of plasma aldosterone.
Quality of life of hirsute women
Authors: Sonino Nicoletta , Fava Giovanni A , Mani Elisa , Belluardo Piera , Boscaro Marco 
Published: December 2008

Summary
Hirsutism is recognized to cause profound distress in affected women, due to cosmetic and psychosexual implications. It was evaluated in the present study by methods found to be valid and reliable in psychosomatic research. Fifty women with hirsutism belonging to the spectrum of disorders from idiopathic hirsutism to polycystic ovary syndrome, after complete medical work-up, underwent the same psychometric evaluation as 50 healthy non-hirsute women, matched for sociodemographic variables. Hirsute women had a Ferriman and Gallwey score ranging from 8 to 19. Psychometric evaluation for quality of life was carried out by the following methods: (a) Kellner's Brief Problem List, a 12 item self-rating list of psychosocial problems; (b) Kellner's Symptom Rating Test (SRT), a 46 item self-rating scale that yields a total score of distress as well as six subscales (anxiety, depression, somatic symptoms, anger-hostility, cognitive and psychotic symptoms); and (c) Marks' Social Situations Questionnaire (SSQ), a 30 item self-rating scale concerned with social phobia. Patients with hirsutism displayed significantly higher social fears at the SSQ than controls (P &lt; 0.01). They also showed more anxiety (P &lt; 0.01) and psychotic symptoms (P &lt; 0.01) at the SRT, whereas there were no significant differences in depression, somatization, anger-hostility and cognitive symptoms. These results suggest that the complex management of hirsute women, in addition to pharmacological and/or cosmetic measures, may require specific psychotherapy.
Age-related differences in simultaneous interarm blood pressure measurements
Authors: Fotherby Martin D , Panayiotou Barnabas , Potter John F 
Published: December 2008

Summary
Simultaneous noninvasive blood pressure measurement were recorded bilaterally in 40 young and 40 elderly subjects. Overall interarm blood pressure (BP) differences for the elderly and young groups were similar, the absolute interarm differences being for systolic blood pressure (SBP) elderly: 4.2 mmHg (95% CI 3.1-5.3 mmHg); young 3.3 mmHg(2.6-4.1 mmHg); diastolic blood pressure (DBP) elderly 3.6 mmHg(2.8-4.4 mmHg), young 2.7 mmHg(2.0-3.3 mmHg). However, the range of interarm BP differences was wide. Four (10%) of the elderly had an interarm SBP difference &gt; 10 mmHg compared to one (3%) of the young group. Interarm DBP differences &gt; 8 mmHg were found in three (8%) of the elderly and in none of the young group. Although age does not affect mean interarm BP differences, clinically important interarm BP differences exist in both young and elderly subjects. Blood pressure should be measured in both arms of all patients at initial assessment to avoid potential problems with misclassification of blood pressure status.
Obesity, albuminuria and hypertension among Hong Kong Chinese with non-insulin-dependent diabetes mellitus (NIDDM)
Authors: Chan J C N , Cheung C K , Swaminathan R , Nicholls M G , Cockram C S 
Published: December 2008

SummaryA total of 412 Hong Kong Chinese diabetic patients were studied on at least two occasions 8-16 weeks apart. Although 28% were insulin-treated, only 3.6% had insulin-dependent diabetes (IDDM). In the remaining 397 patients with non-insulin-dependent diabetes (NIDDM), the mean (s.d.) body mass index (BMI) was 24.4 +/- 3.2 kg/m2 in females and 24.2 +/- 3.2 kg/m2 in males. Obesity was present in 17% of males (BMI &gt; 27 kg/m2) and 40% of females (BMI &gt; 25 kg/m2). Established hypertension was present in 49%. Abnormal albuminuria, defined as a mean urinary albumin/creatinine (UA/Cr) ratio greater than 5.4 mg/mmol based on two random spot urine samples, was present in 47%. On stepwise multiple regression analysis, UA/Cr ratio (R2 = 0.34, F = 65.4, P &lt; 0.001) showed significant associations with systolic blood pressure (standardized regression coefficient beta = 0.40, P &lt; 0.001), plasma creatinine concentration (beta = 0.27, P &lt; 0.001) and glycosylated haemoglobin (beta = 0.20, P &lt; 0.001). While the prevalence of hypertension increased with increasing severity of proteinuria, 40% of normoalbuminuric patients had hypertension. Among patients diagnosed before the age of 35 (n = 67), 52% were insulin-treated although only 10% were insulin-dependent. Among these NIDDM patients of young onset (n = 59), obesity was present in 25% of males and 56% of females. Overall, 18% of these patients had a blood pressure greater than 140/90 mmHg and 27% had abnormal albuminuria. In Hong Kong Chinese, diabetes mellitus is predominantly non-insulin-dependent even in the young. Obesity is more prevalent among females. Abnormal albuminuria is relatively common and is closely associated with hypertension and glycaemic control. In the light of increasing prevalence of diabetes among overseas Chinese, our findings may have important implications in the management of Chinese diabetic patients.
Asymptomatic acute pyelonephritis as a cause of acute renal failure in the elderly
Authors: Woodrow G , Patel S , Berman P , Morgan A G , Burden R P 
Published: December 2008

Summary
Urinary tract infections in the elderly are common, often asymptomatic and usually benign. We report three patients who presented with acute renal failure due to acute pyelonephritis in the absence of clinical findings of infection or urinary tract obstruction. Blood and urine cultures grew Escherichia coli in two of the patients and in two patients renal biopsy confirmed acute pyogenic pyelonephritis. Antimicrobial therapy and haemodialysis led to improvement, though one patient subsequently died from an unrelated cause.
We suggest that acute bacterial pyelonephritis should be considered as a cause of acute renal failure in the elderly. Clinical features of infection may be absent despite bacteraemia. Prompt diagnosis and intervention may avoid chronic renal failure in a group that has a less favourable outcome with long-term dialysis.
Corticosteroid therapy and intercurrent illness: the need for continuing patient education
Authors: Peacey S R , Pope R M , Naik K S , Hardern R D , Page M D , Belchetz P E 
Published: December 2008

Summary
In patients receiving long-term therapeutic or replacement corticosteroids, delayed or inappropriate adjustment of steroid dosage during intercurrent illness may be fatal. We used a questionnaire to assess current levels of patient knowledge, awareness of the need for action during intercurrent illness and the frequency with which steroid warning cards and Medic Alert pendants were carried, in 61 patients on long-term replacement corticosteroids and in 40 patients receiving long-term therapeutic corticosteroids.
Only 67 of the 101 patients taking corticosteroids were carrying a steroid warning card. Eleven of the 21 Medic Alert owners wore their pendants. Only 18 of the 41 patients in the therapeutic group and 41 of the 60 patients in the replacement group would take appropriate action during an intercurrent illness (P &lt; 0.001).
Lack of patient knowledge in this important area emphasizes the need for continuing and effective education of these groups of patients during follow-up. An information sheet detailing the exact changes to be made during intercurrent illness may help reinforce verbal advice.
Complex neuromuscular disorder in a patient with chronic myeloid leukaemia
Authors: Bergin Peter S , Gale Andrew N 
Published: December 2008

SummaryWe report a 44 year old man who developed external ophthalmoplegia and predominantly respiratory, truncal and bulbar weakness with brisk reflexes, histological evidence of an inflammatory myopathy and a high titre of acetylcholine receptor antibodies, one month after starting hydroxyurea and allopurinol for chronic myeloid leukaemia. The temporal relationship suggests a possible association between this patient's unusual neuromuscular disorder and either the chronic myeloid leukaemia or its treatment.
Asynchronous ovarian torsion--the case for prophylactic oophoropexy
Authors: Grunewald B , Keating J , Brown S 
Published: December 2008

Summary
The case of an 11 year old girl who suffered torsion of the left ovary 8 months following right oophorectomy for a similar event is presented. The left ovary was saved by immediate surgical intervention. The value of prophylactic oophoropexy in unilateral ovarian torsion is discussed.
Invasive aspergillosis in two patients with acute lymphoblastic leukaemia in complete remission
Authors: Turner Marc L , Russell Lesley , Milne Leslie J R , Parker Alistair C 
Published: December 2008

Summary
Invasive aspergillosis is a disease of the immunosuppressed patient. We describe two patients with acute lymphoblastic leukaemia who attained complete remission, with partial or complete bone marrow recovery, but who went on to develop fatal invasive aspergillosis contemporaneous with recovery of neutrophil counts. Quantitative recovery of peripheral blood neutrophil counts does not guarantee control of Aspergillus infection, perhaps due to functional neutrophil deficiencies post-chemotherapy, and specific defensive strategies adopted by the organism itself.
Intraperitoneal haemorrhage from anterior abdominal wall varices
Authors: Hunt J B , Appleyard M , Thursz M , Carey P D , Guillou P J , Thomas H C 
Published: December 2008

Summary
Patients with oesophageal varices frequently present with gastrointestinal haemorrhage but bleeding from varices at other sites is rare. We present a patient with hepatitis C-induced cirrhosis and partial portal vein occlusion who developed spontaneous haemorrhage from anterior abdominal wall varices into the rectus abdominus muscle and peritoneal cavity.
Anterior pituitary function after adrenalectomy in patients with Cushing's syndrome
Authors: Marazuela M , Cuerda C , Lucas T , Vicente A , Blanco C , Estrada J 
Published: December 2008

Summary
We assessed anterior pituitary function in five patients with Cushing's syndrome before and after the removal of cortisol-secreting adrenal adenomas. Before surgery, all patients lacked response of growth hormone to hypoglycaemia, four had low responses of thyrotrophin to thyrotrophin releasing hormone, three had hypogonadism and two had low prolactin reserve. After successful removal of the adrenal adenoma, all patients developed postoperative hypoadrenocorticism and recovered all impaired anterior pituitary hormones within a period of 3 months. Our results point to a direct inhibiting action of glucocorticoids at the pituitary level as the explanation for the impaired anterior pituitary function. Moreover, direct gonadal suppression by glucocorticoids may be an additional mechanism of hypogonadism in some patients.
Systemic lambda light-chain deposition presenting with predominant cardiac involvement
Authors: Garton M J , Walton S , Ewen S W B 
Published: December 2008

Summary
An 82 year old woman with suspected Bence Jones myeloma developed intractable fluid retention presumed secondary to cardiac failure. In addition she experienced angina pectoris, and required permanent cardiac pacing for symptomatic sinus bradycardia. Postmortem studies revealed prominent myocardial and renal deposits of lambda light-chains which were Congo Red negative, and had a non-fibrillar ultrastructure. Non-amyloidotic light-chain deposition is uncommon, and a rare cause of cardiac disease. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features and treatment is reviewed.
Parathyroid hormone-related protein as a tumour marker in humoral hypercalcaemia associated with occult malignancy
Authors: Hutchesson A C J , Dunne F , Bundred N J , Gee H , Ratcliffe W A 
Published: December 2008

Summary
The tumour-derived factor PTH-related protein (PTHRP) is the primary humoral factor responsible for hypercalcaemia in patients with solid tumours. In a woman presenting with anaemia and hypercalcaemia, the finding of raised plasma PTHRP and undetectable serum PTH concentrations led to further investigations and the subsequent identification of a uterine tumour. No evidence of tumour spread was found at operation, and removal of the tumour resulted in normalization of both serum calcium and plasma PTHRP. Expression of PTHRP by the tumour was shown by immunohistochemistry and in situ hybridization. We conclude that the identification of an occult tumour in a patient with hypercalcaemia and raised plasma PTHRP provides evidence of the diagnostic utility of PTHRP immunoassays in the investigation of patients with hypercalcaemia and suspected malignancy.
Salivary gland involvement as initial presentation of Wegener's disease
Authors: Vanhauwaert Bert G , Roskams Tania A , Vanneste Stefan B , Knockaert Daniel C 
Published: December 2008

Summary
We describe a case of Wegener's disease with massive painful bilateral submandibular gland enlargement as the presenting symptom. The diagnosis was based on histologically documented nasal mucosa involvement, focal necrotizing glomerulonephritis, episcleritis and anti-neutrophil cytoplasmic antibody.
Comparison of clock drawing with Mini Mental State Examination as a screening test in elderly acute hospital admissions
Authors: Death John , Douglas Adam , Kenny Rose Anne 
Published: December 2008

Summary
Clock drawing is a quick, easy to remember test that is well received by patients. It is a good screening test for Alzheimer's disease in the outpatient setting. We evaluated its usefulness compared with the standard Mini Mental State Examination (MMSE) in elderly acute medical and surgical hospital admissions. Within 48 hours of admission, 117 patients over 70 years old were administered the MMSE and asked to draw a clock. Using the MMSE as the standard, clock drawing had a sensitivity of 77% and a specificity of 87%. Patients with discrepant scores were then further evaluated. The findings suggest that normal clock drawing ability reasonably excludes cognitive impairment or other causes of an abnormal MMSE in elderly acute medical and surgical hospital admissions, where cognitive impairment is common and frequently missed.
Liver/spleen scintigraphy for diagnosis of splenic infarction in cirrhotic patients
Authors: Chin J K T , McCormick P A , Hilson A J W , Burroughs A K , McIntyre N 
Published: December 2008

Summary
Splenic infarction is rare in cirrhotic patients. The diagnosis of the condition is based on clinical findings and splenic imaging. In recent years, ultrasonography and computed tomographic scan have gained popularity over the more classical scintigraphy as the preferred investigations for the diagnosis of splenic infarction. We report three cases of splenic infarction in patients with cirrhosis and portal hypertension. Computed tomographic scan, angiography and ultrasonography failed to identify the lesions and the diagnoses were finally made with the aid of liver--spleen scintigraphy. We suggest that scintigraphy is the investigation of choice if splenic infarction is suspected in patients with congestive splenomegaly secondary to liver cirrhosis.
Effects of octreotide on circulating islet B cell products in endogenous hyperinsulinism
Authors: Krentz Andrew J , Pace Jackie , Somerville Wendy , Clark Penelope M S , Nattrass Malcolm 
Published: December 2008

Summary
The role of somatostatin analogues in the medical management of insulinomas is unclear. We describe an elderly patient with clinical and biochemical features of endogenous hyperinsulinism attributable to a benign islet B cell disorder whose incapacitating neuroglycopaenic symptoms responded dramatically to octreotide 50 micrograms subcutaneously at 2200 h each night. Octreotide suppressed inappropriate plasma concentrations of insulin thereby preventing fasting hypoglycaemia. Fasting concentrations of proinsulin, and 32-33 split proinsulin, as determined by two-site monoclonal antibody-based immunoradiometric assays, were also suppressed by octreotide.
Spinal ganglioneuroblastoma--complete response to chemotherapy alone
Authors: Raina V , Kamble R , Tanwar R , Singh S P , Sharma S 
Published: December 2008

Summary
Ganglioneuroblastoma in the spinal region is rare, the treatment of choice being surgical excision. We present a 21 year old male who was diagnosed to have this condition in the dorsolumbar spinal region. The tumour was extending intraspinally and was unresectable. Combination chemotherapy with Adriamycin (doxorubicin hydrochloride), vincristine, cyclophosphamide, etoposide, ifosfamide and cisplatin resulted in histologically proven complete remission. No radiotherapy or curative resection was done. The patient is alive without evidence of disease 24 months later. Never before has chemotherapy been successfully used as the sole modality of treatment in this condition. Our report raises important questions about the management of this rare condition, particularly in a situation of unresectability.
Sexual abuse of the elderly mentally ill
Authors: Benbow Susan M , Haddad Peter M 
Published: December 2008

Summary
Sexual abuse of the elderly may occur more commonly than is recognized. Reasons for the neglect of this area and possible risk factors are discussed. A definition of elder sexual abuse is proposed, and four case histories, each of which raises various issues about the nature, detection and management of sexual abuse, are described. In the absence of procedures for dealing with elder sexual abuse, professional staff need to be open to its occurrence and to be prepared to carry out thorough, sympathetic, collaborative assessments of both parties involved where it is suspected.
Reporting ventilation-perfusion lung scintigraphy: impact on subsequent use of anticoagulation therapy
Authors: Kaboli P , Buscombe J R , Ell P J 
Published: December 2008

Summary
Ventilation and perfusion lung scintigraphy is an established and safe noninvasive technique which has been used for nearly 30 years to establish the diagnosis of pulmonary embolism. Recently, in common with other diagnostic tests, there has arisen the need to reassess the effectiveness of this test in the clinical setting. A retrospective analysis of 244 patients undergoing ventilation-perfusion lung scintigraphy was performed. A total of 203 case notes were available. It was found that the majority of reports (68.5%) were described as either high probability for the presence of pulmonary embolism or normal or low probability. A result of 'high probability' changed the clinical management of the patient in 28/46 (61%) of reported cases but in only 5/91 (5.5%) of patients reported as 'normal' or 'low probability'. During the study there were six deaths, five from other causes and one from pulmonary embolism; this patient died despite anticoagulation following a 'high probability' ventilation-perfusion lung scintigraphy. Ventilation-perfusion lung scintigraphy is a safe and effective noninvasive method to diagnose the presence of pulmonary embolism and a test which has a significant effect on patient management.