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Biomedical subjects

F Afshar

Publications and source records attributed to F Afshar.

At least 19 recordsLinked to original sources

Investigation, management and therapeutic outcome in 12 cases of childhood and adolescent Cushing's syndrome.

OBJECTIVE: Cushing's syndrome in childhood and adolescence is rare. We analysed the clinical presentation, investigation, management and therapeutic outcome in 12 paediatric patients with Cushing's syndrome. DESIGN: Retrospective review of case notes. PATIENTS: Twelve patients, 7 males and 5 females, aged 7.6-17.8 years with Cushing's syndrome who were admitted to St Bartholomew's Hospital between 1978 and 1993, were studied. Aetiologies of the Cushing's syndrome patients were: Cushing's disease (9), adrenal adenoma (1), nodular adrenocortical dysplasia (1) and ectopic ACTH syndrome (1). One further male patient, aged 17.8 years who presented with Nelson's syndrome after bilateral adrenalectomy for Cushing's disease in 1978, is described. MEASUREMENTS: Presenting symptoms, endocrine tests for hypercortisolism, imaging studies, simultaneous bilateral inferior petrosal sinus sampling and therapeutic strategies are discussed. RESULTS: The dominant clinical features were obesity, short stature, virilization, headaches, fatigue and emotional lability. Investigations confirmed Cushing's syndrome by demonstrating absent cortisol circadian rhythm and impaired suppression on low dose dexamethasone test and differentiated Cushing's disease from other aetiologies by high dose dexamethasone and hCRH tests. In Cushing's disease, pituitary CT scan identified a microadenoma in 4 out of 9 subjects. In 5 of the 9 patients (3 with a normal pituitary CT, 2 with a suggested microadenoma), a pituitary MRI scan was performed and confirmed the CT findings. Inferior petrosal sinus catheterization for ACTH in 4 patients confirmed excess pituitary ACTH secretion, correctly lateralizing the tumour in all cases. Cushing's disease was treated by transsphenoidal surgery alone in 6 patients and combined with pituitary irradiation in 3 patients. Of these 9 patients, 7 are cured and 2 are in remission. The patient with Nelson's syndrome is cured after total hypophysectomy. CONCLUSIONS: This series describes the clinical features, aetiologies and management of juvenile Cushing's syndrome. Investigation with low and high-dose dexamethasone suppression tests and hCRH test identified the aetiology in each case. Collaboration between paediatric and adult endocrine units together with an experienced neurosurgeon and a radiotherapist contributed to the successful therapeutic outcome of these patients.

Adenoma

Diagnosis and selective cure of Cushing's disease during pregnancy by transsphenoidal surgery.

The diagnosis of Cushing's Disease during pregnancy is complex because the biochemical features are obscured by changes in the normal hypothalamo-pituitary-adrenal axis that occur during gestation. To date, treatment has not been successful and there is a high incidence of maternal and fetal complications. We report the case of a 24-year-old woman with Cushing's disease who presented during her 16th week of pregnancy. Diagnosis was confirmed by the finding of elevated serum and urinary free cortisol levels with loss of the normal circadian rhythm of serum cortisol. Cortisol levels failed to suppress after a low-dose dexamethasone test but suppressed after a high-dose test. There was an exaggerated serum cortisol and plasma adrenocorticotrophin (ACTH) response to corticotrophin-releasing hormone (CRH). Magnetic resonance (MR) scanning demonstrated a pituitary tumour and cure was effected by transsphenoidal surgery where tumour immunostaining for ACTH was removed. Postoperatively the patient made an uncomplicated recovery; serum cortisol and plasma ACTH levels were undetectable at 9 days following surgery and recovery of the hypothalamo-pituitary axis occurred at 99 days after surgery. Caesarean section was performed at 38 weeks of pregnancy and a healthy but small female infant was delivered. This case illustrates the biochemical features of Cushing's disease during pregnancy and is the first report of the use of CRH testing and MR scanning in this clinical situation. The cure by surgery and successful outcome for mother and infant, with preservation of normal anterior pituitary function, suggest that transsphenoidal surgery may be the treatment of choice.

Adrenocorticotropic Hormone

Delayed intracerebral haematomas in moderate to severe head injuries in young adults.

The incidence of delayed intracerebral haematoma formation in moderate to severely head-injured young adults who do not have signs of cerebral contusions on the initial post-trauma head CT scan has been studied. Thirty patients were reviewed and in 6 (20%) a delayed haematoma was diagnosed on a later CT scan, when signs of cerebral contusions were absent initially. Recurrence of intracranial hypertension within 3 to 4 h proved to be a significant indication for the development of these haematomas, despite the initial but inconsistent good response to osmotic diuretics. The average Glasgow outcome score was 3, including two mortalities. Cerebral perfusion pressure directly correlated with the outcome.

Adolescent

Endothelin-1 and big endothelin cause subarachnoid hemorrhage in the anesthetized rabbit.

Intra-arterial injection of endothelin-1 (ET-1) (1 nmol/kg; n = 8) or human big endothelin-1 (b-ET-1; 3 nmol/kg; n = 8) into anesthetized rabbits produced a significant rise in left ventricular systolic pressure (LVSP) and caused subarachnoid hemorrhage (SAH) in 75 +/- 17% and 88 +/- 12% of the experiments, respectively. In all animals, the SAH occurred in the subarachnoid space around the distal part of the basilar artery complex. The cyclooxygenase inhibitor indomethacin (5 mg/kg i.v.) significantly potentiated the pressor effect of both peptides, and all animals pretreated with indomethacin prior to ET-1 (n = 3) or b-ET (n = 3) developed SAH. In contrast, rabbits treated with vehicle (saline; n = 7), indomethacin alone (n = 3), or the carboxy-terminal fragment of b-ET (CT 22-38; 3 nmol/kg i.a.; n = 3) developed neither a rise in LVSP nor SAH. A rise in blood pressure alone is unlikely to account for the SAH brought about by the peptides for angiotensin II (1 nmol/kg/min for 30 min; n = 7) produced a significantly greater increment in LVSP than ET-1 or b-ET, but did not cause SAH. In addition, there was no significant correlation between the rise in LVSP produced by ET-1 or b-ET and the severity of the SAH.

Anesthesia

Giant cell tumour of the sphenoid bone with coincidental galactorrhoea--a case report.

A case of the rare giant cell tumour involving the sphenoid bone is reported. The usual presentation of these tumours is headache and cranial nerve deficits. This 25-year-old lady presented with oligomenorrhoea and galactorrhoea. Investigations demonstrated an asymptomatic erosive mass lesion in the sphenoid with suprasellar extension and extension into the nasopharynx. The tumour was partially resected via the transphenoidal route. The differential diagnosis and treatment of these lesions are discussed.

Adult

Paediatric brachytherapy. II. Brain implantation.

The case histories of four children with brain tumours, for whom stereotactic brachytherapy was indicated, are presented from the St Bartholomew's Hospital intracranial brachytherapy programme. The recent evolution of the computed-tomography-directed stereotactic technique is described and the uses of different radionuclides (198Au, 192Ir) are discussed. The future of brachytherapy for paediatric brain tumours is debated.

Adolescent

Chronic spontaneous tension pneumocephalus due to benign frontal sinus osteoma.

Spontaneous pneumocephalus secondary to neoplasia is a rare condition. It may present with headaches, rhinorrhoea, otorrhoea, or meningitis in various combinations. A case of unusual presentation, with several months delay in diagnosis, with remarkable postoperative improvement is reported and the literature reviewed.

Female

The effects of transsphenoidal surgery on endocrine function and visual fields in patients with functionless pituitary tumours.

Twenty-eight patients with functionless pituitary tumours have been treated by transsphenoidal surgery over the last 28 months. Mean age at presentation was 55 years. Tumor size was graded according to the extent of suprasellar extension on CT headscan from above the interclinoid line: four were small, 15 medium and nine large. Before surgery in 25 per cent of patients visual acuity and visual fields were normal. In the immediate postoperative period, 24 per cent of the patients with visual defects before surgery had normal vision, and in 38 per cent of the others it was improved. Six months after surgery, there had been further improvement; 43 per cent had normal vision and 48 per cent showed an overall improvement. In no patient did vision deteriorate following surgery. In the patients who presented with visual symptoms of one year or less, there was no correlation between the length of symptoms and the extent of visual recovery after surgery. Average age of the patients with full visual recovery was 47 +/- 4 years (mean +/- SEM); average age of the patients with only partial visual recovery was 63 +/- 3 years. Prolactin levels before surgery were elevated in 77 per cent of patients and fell significantly after operation, remaining elevated in 28 per cent of patients six months later. Fifty per cent of patients were treated with long-term hormone replacement therapy. Transsphenoidal surgery led to improved vision in the majority of patients, the results being comparable with those obtained with transfrontal surgery. Surgical complications were few, and long-term morbidity low. We suggest that the first-line treatment for patients with functionless pituitary tumours should be transsphenoidal surgery, even when large suprasellar extensions are present.

Adenoma

Immunoreactive human chorionic gonadotrophin from the cyst fluid and CSF of patients with craniopharyngioma.

A specific radioimmunoassay for the beta-chain of human chorionic gonadotrophin irHCG beta has demonstrated HCG-like material to be present in craniopharyngioma cyst fluid in nine consecutive patients with craniopharyngioma. There was no detectable LH/HCG bioactivity as assessed using testosterone production from isolated Leydig cells from rat testis in seven samples tested. One patient was also found to have measurable irHCG beta in the cerebrospinal fluid (CSF), which fell to undetectable levels following surgery; in this patient, there was clinical evidence that the cyst fluid had leaked into the CSF pre-operatively. Immunocytochemical staining for HCG beta and intact HCG was positive in five of the tumours. irHCG beta was not measurable in the serum of any of the patients.

Biological Assay

Computerised axial tomography and magnetic resonance scanning in the Tolosa-Hunt syndrome.

A 50-year-old Asian male presented with a left sixth nerve palsy, left temporal pain, and rapidly deteriorating visual acuity in the left eye. A high resolution CT scan and magnetic resonance scan showed a left retro-orbital enhancing lesion extending from the lateral margin of the cavernous sinus on to the greater wing of the sphenoid and into the left orbit. Arteriography was normal. On high dose steroid therapy there was total resolution of the lesion. The value of imaging techniques in this condition is discussed.

Dexamethasone

Transsphenoidal chiasmopexy for long standing visual failure in the secondary empty sella syndrome.

The development of an empty sella syndrome is a well recognized complication in the treatment of pituitary tumours and usually presents with visual failure. Transsphenoidal chiasmopexy has been successful in reversing the visual loss when performed in the acute stage. A case, where a delay of 18 months in the surgery does not appear to have affected the outcome, is presented and the literature reviewed.

Adenoma

Comparative study of brain abscess by computed tomography and magnetic resonance imaging: report of two cases.

Two cases of cerebral abscess with different presentations are reported. The source of the abscess remained obscure in one case while in the other arose from infection of the right frontal sinus. While in one case a magnetic resonance image was obtained preoperatively, both cases underwent parallel monitoring in the postsurgery period by computed tomography and magnetic resonance imaging. The relative merits of these two imaging techniques in the diagnosis and monitoring of such lesions are discussed, and the literature is reviewed.

Adult

The microsurgical treatment of lumbar disc protrusion. Follow-up of 60 cases.

The technique and results of microsurgical techniques for lumbar disc protrusions in 60 patients are presented. All patients were followed up for a minimum of 2.5 years, and 91% had good or excellent results. The only patient with a recurrent disc sequestration had a good result after a second operation.

Follow-Up Studies

Spinal cord astrocytoma with intracranial spread: detection by magnetic resonance imaging.

An astrocytoma of the thoracic spinal cord in a 9-year-old boy was treated by partial tumour removal and postoperative radiotherapy. The development of intracranial spread causing cranial nerve signs and diabetes insipidus was detectable only by Magnetic Resonance Imaging. The spread of these tumours and difficulties in diagnosis are discussed together with a review of the current literature.

Brain Neoplasms

Diagnosis of chronic subdural haematoma: the advantages of MR imaging compared with the CT-scan.

Two cases of chronic subdural haematoma are reported. Both highlight the difficulties associated with the diagnosis of such collections in the isodense phase on Computed Tomography. Magnetic Resonance Imaging proved its higher discriminating power by delineating the haematomas in both cases. The respective values of these two imaging techniques are discussed with a review of the current literature.

Adult

Metastatic carcinoid tumour of the CNS from two different primary sites.

Two cases of carcinoid tumour affecting the central nervous system through their metastases were treated surgically. The first case was carcinoid tumour of the lung with a single metastases in the right frontal lobe of the brain, while the second developed metastases of the sixth thoracic vertebra from a terminal ileum bowel primary causing spinal cord compression. Neither case developed carcinoid syndrome. The rarity of such metastases affecting the central nervous system is discussed together with a review of the literature.

Aged