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1.
J Helminthol ; 98: e42, 2024 May 27.
Article in English | MEDLINE | ID: mdl-38798016

ABSTRACT

Four species of the genus Longidorus were recovered from southern (Bushehr province) and southeastern (Southern Khorasan province) Iran. The first species, L. paratabrizicus n. sp. represents a new member to the genus and is characterised by 4.8-5.6 mm long females with anteriorly flattened lip region separated from the rest of the body by depression, amphidial fovea pocket-shaped without lobes, tail conical, dorsally convex, ventrally almost straight with bluntly rounded tip and males in population. By having similar lip region and tail shape, the new species most closely resembles five species viz. L. artemisiae, L. globulicauda, L. patuxentensis, L. sturhani, and L. tabrizicus. It represents the cryptic form of the last species. The second species belongs to L. mirus, recovered in both southern and southeastern Iran, representing the first record of the species after its original description. As an update to the characteristics of this species, it's all juvenile developmental stages were recovered and described. The criteria to separate L. mirus from two closely related species, L. auratus and L. africanus, are discussed. The third species belongs to L. persicus, a new record in southern Iran. The fourth species, L. orientalis was recovered in high population density in association with date palm trees in Bushehr province. The phylogenetic relationships of the new species and recovered populations of L. mirus and L. persicus were reconstructed using two ribosomal markers and the resulted topologies were discussed.


Subject(s)
Phylogeny , Iran , Animals , Male , Female , Nematoda/classification , Nematoda/anatomy & histology , Nematoda/genetics , Microscopy
2.
Res Pharm Sci ; 8(1): 65-9, 2013 Jan.
Article in English | MEDLINE | ID: mdl-24459478

ABSTRACT

The essential oils of the aerial parts of A. armeniaca and A. incana, collected from Arasbaran area (East Azarbaijan province, Iran) were extracted by hydrodistillation and analyzed by GC-MS. In total, 16 and 40 constituents were identified and quantified in the oils of A. armeniaca and A. incana representing 80.5% and 84.6% of the oils, respectively. The essential oil of A. armeniaca was mainly composed of non-terpene hydrocarbons (24.8%). The major components of the oil were α-pinene (10.7%), nonadecane (10.0%), 6,10,14-trimethyl-2-pentadecanone (9.4%), spathulenol (7.8%) and Z-verbenol (5.8%). The essential oil of A. incana was dominated by oxygenated monoterpenes (41.6%), with camphor (20.4%), 1,8-cineol (10.3%), Z-verbenol (8.7%), ß-thujone (8.3%) and α-thujone (5.6%), as major components. The essential oils were also subjected to general toxicity assay using brine shrimp lethality method. The toxicity profile of both oils indicated some degree of toxicity in comparison with podophyllotoxin.

3.
Horm Res Paediatr ; 75(6): 441-6, 2011.
Article in English | MEDLINE | ID: mdl-21430362

ABSTRACT

BACKGROUND: Psychosis secondary to paediatric Cushing's disease (CD) is extremely rare and presents a significant management challenge. METHOD: We report a 14.7-year-old CD patient with acute psychosis and self-inflicted injuries following failed transsphenoidal pituitary surgery. Her mental state rapidly deteriorated precluding medical therapy. RESULTS: Emergency intravenous low-dose etomidate infusion (3-3.5 mg/h) with dose titration according to the serum cortisol combined with a hydrocortisone infusion, in an intensive care setting, was effective in controlling the hypercortisolaemia. Her mental state improved with normalisation of her cortisol levels enabling oral administration of ketoconazole and bilateral adrenalectomy to be performed. CONCLUSION: This case illustrates the safe and effective use of a low-dose etomidate infusion in an unusual case of paediatric CD.


Subject(s)
Anesthetics, Intravenous/therapeutic use , Cushing Syndrome/complications , Etomidate/therapeutic use , Pituitary ACTH Hypersecretion/complications , Psychotic Disorders/drug therapy , Psychotic Disorders/etiology , Adolescent , Female , Humans , Self-Injurious Behavior/etiology
4.
Eur J Endocrinol ; 164(5): 667-74, 2011 May.
Article in English | MEDLINE | ID: mdl-21310872

ABSTRACT

OBJECTIVE: There are few published comparisons between paediatric and adult-onset Cushing's disease (CD). We compare the epidemiology, diagnostic features and cure rate by transsphenoidal surgery (TSS) in these groups. DESIGN: Retrospective review of patient databases in a single university hospital centre. PATIENTS: Totally, 41 paediatric (mean age 12.3 ± 3.5 years; range 5.7-17.8) and 183 adult (mean age 40 ± 13 years; range 18.0-95.0) patients with CD were investigated. RESULTS: Paediatric CD was characterised by male (63%) and adult CD by a female predominance (79%, P<0.0001). There were small but significant differences in clinical presentation. Biochemical features of CD were comparable except the serum cortisol increase during a CRH test: mean change (105%, n=39) in paediatric and (54%, n=123) in adult subjects (P<0.0001). Macroadenomas were more common in adult (15%, 28/183) than in paediatric (2%, 1/41, P=0.04) CD. Corticotroph microadenomas were more easily visualised by pituitary magnetic resonance imaging (MRI) in adult (76%, 50/66) compared with paediatric (55%, 21/38, P=0.045) CD with poorer concordance of imaging with surgical findings in children (P=0.058). The incidence of ACTH lateralisation by bilateral simultaneous inferior petrosal sinus sampling was comparable in paediatric (76%, 25/33) and adult (79%, 46/58; P=0.95) patients with good surgical concordance in both (82% paediatric and 79% adult). Cure rates by TSS were comparable, with a paediatric cure rate of 69%. CONCLUSION: Several features of paediatric CD are distinct: increased frequency of prepubertal CD in males, the different clinical presentation, the decreased presence of macroadenomas and the frequent absence of radiological evidence of an adenoma on MRI.


Subject(s)
Pituitary ACTH Hypersecretion/epidemiology , Pituitary ACTH Hypersecretion/surgery , Sphenoid Sinus/surgery , Adolescent , Adult , Age of Onset , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Pituitary ACTH Hypersecretion/diagnosis , Retrospective Studies , Sphenoid Sinus/pathology , Treatment Outcome , Young Adult
5.
Nanoscale Res Lett ; 5(4): 709-13, 2010 Jan 20.
Article in English | MEDLINE | ID: mdl-20672036

ABSTRACT

Metal interconnections having a small cross-section and short length can be subjected to very large mass transport due to the passing of high current densities. As a result, nonlinear diffusion and electromigration effects which may result in device failure and electrical instabilities may be manifested. Various thicknesses of Pd were deposited over SrTiO3 substrate. Residual stress of the deposited film was evaluated by measuring the variation of d-spacing versus sin2ψ through conventional X-ray diffraction method. It has been found that the lattice misfit within film and substrate might be relaxed because of mass transport. Besides, the relation between residual intrinsic stress and oxygen diffusion through deposited film has been expressed. Consequently, appearance of oxide intermediate layer may adjust interfacial characteristics and suppress electrical conductivity by increasing electron scattering through metallic films.

6.
Eur J Endocrinol ; 162(3): 603-9, 2010 Mar.
Article in English | MEDLINE | ID: mdl-19952125

ABSTRACT

OBJECTIVES: Cushing's disease (CD) in prepubertal children is very rare and presents important diagnostic and therapeutic challenges. We report experience of the management of this subpopulation of CD patients. STUDY DESIGN/METHODS: Retrospective patient case note review. RESULTS: Between 1985 and 2008, 17 prepubertal children (13M, 4F), aged 5.7-14.1 years presented to our centre for diagnosis and management of CD. All children had subnormal linear growth and excessive weight gain at presentation. A high proportion (85% of males, 75% of females) had evidence of excessive virilisation. Striae and hypertension were seen in 41% of patients. The investigation with highest sensitivity (100%) for CD was excessive increase of serum cortisol to i.v. CRH (mean increase 113%). Pituitary imaging performed in all the patients showed poor concordance with findings at surgery (31%). In contrast bilateral simultaneous inferior petrosal sinus sampling (BSIPSS), performed in 11/16 subjects showed a high correlation with surgical findings (91%). In 16 patients, transsphenoidal selective adenomectomy (TSS) achieved a cure rate of 44%. However, in the 11 patients who had pre-operative BSIPSS, the cure rate was 64%. Of the 16 patients, 9 patients who were not cured by TSS received external pituitary radiotherapy. CONCLUSIONS: Prepubertal CD had distinctive features with increased frequency in males, abnormal auxology and excessive virilisation. The cortisol response to i.v. CRH administration was particularly exuberant and contributed to diagnosis. BSIPSS was much more helpful than pituitary imaging in localisation of the microadenoma and was associated with improved cure rate by TSS.


Subject(s)
Adenoma/therapy , Pituitary ACTH Hypersecretion/diagnosis , Pituitary ACTH Hypersecretion/therapy , Pituitary Neoplasms/therapy , Adenoma/complications , Adenoma/diagnosis , Adolescent , Child , Child, Preschool , Combined Modality Therapy , Corticotropin-Releasing Hormone , Female , Humans , Hydrocortisone/blood , Hypertension/etiology , Immunoassay , Magnetic Resonance Imaging , Male , Overweight/etiology , Pituitary ACTH Hypersecretion/complications , Pituitary Gland/physiopathology , Pituitary Neoplasms/complications , Pituitary Neoplasms/diagnosis , Retrospective Studies , Treatment Outcome , Virilism/etiology
7.
Br J Neurosurg ; 21(4): 399-402, 2007 Aug.
Article in English | MEDLINE | ID: mdl-17676462

ABSTRACT

Pituitary tumours usually present with visual field defects; extraocular palsy is usually a sign of cavernous sinus extension and is a sign of advanced disease. We report a case of a pituitary adenoma presenting with complete, bilateral oculomotor nerve palsies, but minimal loss of visual fields, and intact abducens and trochlear nerves.


Subject(s)
Nerve Compression Syndromes/etiology , Oculomotor Nerve/surgery , Ophthalmoplegia/etiology , Pituitary Apoplexy/etiology , Pituitary Neoplasms/complications , Aged , Female , Humans , Nerve Compression Syndromes/diagnosis , Ophthalmoplegia/diagnosis , Pituitary Apoplexy/diagnosis , Pituitary Neoplasms/diagnosis , Treatment Outcome
8.
Pak J Biol Sci ; 10(18): 3091-6, 2007 Sep 15.
Article in English | MEDLINE | ID: mdl-19090104

ABSTRACT

In order to assess clinico-radiological findings of urinary diversion, five adult healthy mongrel dogs of both sexes weighing between 25-40 kg and average age of 1.5 years, underwent continent urinary diversion surgery. In this approach fifteen centimeters of the descending colon with preservation of its mesenteric vessels was resected and this segment longitudinally was opened and flushed with an aqueous solution of povidin iodine 0.1% and the remaining colon re-anastomosed by seromuscular sutures. Then two ends of transected ureters were drawn into the resected colon by mosquito hemostatic forceps and simple interrupted sutures were placed between the ureter and the colonic mucosa for uretero-colonic anastomosis. The uretero-colonic part in a cap form transplanted to partially cystectomized bladder with one layer of cushing pattern suture. All animals survived after the operation. Clinically, all dogs were dull and depressed and passed blood tinged urine for first few postoperative days. There was pollakiuria in all of animals distinctly, but urinary incontinence was not observed. At the first few postoperative days, anorexia, nausea and vomiting were seen in three dogs. In all animals, polydipsia were seen and continued until the end of study. Intravenous urograms showed hydronephrosis and upper urinary tracts dilatation in all animals at 25th day but there were signs of improvement at 45th day distinctly. Unilateral ureteral obstruction was observed in two dogs. In regards to clinico-radiological findings can be concluded that this operation has some distinct sequela that some of them may be seen in radiographic assessments.


Subject(s)
Colon/pathology , Colon/surgery , Urinary Bladder/surgery , Anastomosis, Surgical , Animals , Colon/diagnostic imaging , Cystectomy , Dogs , Female , Male , Mucous Membrane/pathology , Radiography , Surgical Procedures, Operative/methods , Time Factors , Treatment Outcome , Ureter/pathology , Urinary Bladder/diagnostic imaging , Urinary Diversion/adverse effects , Urinary Incontinence
9.
Clin Endocrinol (Oxf) ; 62(4): 466-72, 2005 Apr.
Article in English | MEDLINE | ID: mdl-15807878

ABSTRACT

OBJECTIVE: Linear growth data after cure of paediatric Cushing's disease (CD) have been reported infrequently. We evaluated final adult height (FH) and body mass index (BMI) in a cohort of paediatric patients treated successfully for CD. PATIENTS AND METHODS: Fourteen patients (10 male, age range 6.4-16.6 years) fulfilled the diagnostic criteria for CD. All had had transsphenoidal surgery (TSS), combined with pituitary irradiation (RT) (45 Gy in 25 fractions) in six. All were cured (post-TSS cortisol < 50 nmol/l or mean cortisol post-RT < 150 nmol/l). Subjects analysed had bone ages at diagnosis of < 15 'years' (male) and < 13 'years' (female). RESULTS: At diagnosis, height SDS was [mean (range)]-2.5 (-4.2 to -0.8) and body mass index (BMI) SDS +2.7 (0.8-5.1). Following cure, 13 patients had GH deficiency (peak GH < 20 mU/l) and were treated with hGH (+ GnRH analogue in four). Height SDS at FH (n = 10) or latest assessment (n = 4) was -1.3 (-3.9-0.2) and increased compared to diagnosis (P < 0.01). The difference between final or latest height SDS and target height SDS was -1.2 (-3.3-0.5), that is less (P < 0.01) than the difference between the height SDS at diagnosis and target height SDS of -2.4 (-3.9 to -0.5). At final height or latest assessment, BMI SDS was +1.7 (0.4-6.2), being decreased compared to diagnosis (P < 0.05) but greater than the normal population (P < 0.01). CONCLUSION: Catch-up growth was demonstrated in paediatric patients cured from CD, with the majority achieving FH within target height range. Early diagnosis and treatment of GH deficiency is recommended to achieve optimal long-term growth. Excess adiposity remains a potential long-term complication.


Subject(s)
Body Height , Body Mass Index , Cushing Syndrome/surgery , Pituitary Gland/surgery , Adolescent , Child , Cushing Syndrome/physiopathology , Cushing Syndrome/radiotherapy , Female , Gonadotropin-Releasing Hormone/agonists , Goserelin/therapeutic use , Human Growth Hormone/therapeutic use , Humans , Male , Pituitary Irradiation , Retrospective Studies , Statistics, Nonparametric , Treatment Outcome
10.
Minim Invasive Neurosurg ; 47(4): 256-7, 2004 Aug.
Article in English | MEDLINE | ID: mdl-15346327

ABSTRACT

A 26-year-old male presented with acute mid-thoracic back pain following a witnessed grand mal seizure. There was no trauma and the patient was on steroids for systemic lupus erythematosus. X-rays and CT scans of the thoracic spine revealed compression fractures at T5 and T6, with 50 % loss of vertebral height and kyphosis. He underwent percutaneous kyphoplasty of both vertebrae, with symptomatic improvement. Non-traumatic compression fractures of the thoracic spine following seizures are a rare injury. This may be related to the compressive forces exerted on the vertebral column by the contractions of the truncal muscles, during a seizure. These compression fractures are suitable for treatment by minimally invasive techniques, such as kyphoplasty.


Subject(s)
Bone Cements/therapeutic use , Epilepsy, Tonic-Clonic/complications , Orthopedic Procedures/methods , Polymethyl Methacrylate/therapeutic use , Seizures/complications , Spinal Fractures/etiology , Spinal Fractures/surgery , Thoracic Vertebrae/injuries , Thoracic Vertebrae/surgery , Adult , Biomechanical Phenomena , Humans , Male , Treatment Outcome
11.
Clin Endocrinol (Oxf) ; 60(2): 169-74, 2004 Feb.
Article in English | MEDLINE | ID: mdl-14725677

ABSTRACT

OBJECTIVE: Although Cushing's disease (CD) rarely occurs in childhood, affected children commonly fail to achieve predicted adult height. Hypercortisolaemia results in reduced GH secretion and GH-deficiency may persist or demonstrate delayed recovery after successful treatment of CD in adults. Whether recovery of spontaneous GH secretion occurs following treatment of childhood CD has yet to be established. DESIGN AND PATIENTS: We performed a retrospective analysis of the GH status of 13 children (10 males; 12.8 +/- 1.0 years, mean +/- SE) who had undergone successful treatment of CD that occurred prior to the completion of linear growth. Each underwent transsphenoidal hypophysectomy, resulting in satisfactory control of glucocorticoid levels in 7/13 (54%). The remaining six patients (46%) received fractionated external beam irradiation (4500 Gy). At the time of GH assessment, circadian dynamics of cortisol were normal in eight patients and five were receiving titrated glucocorticoid replacement. MEASUREMENTS: GH status was assessed using the peak response to a provocative stimulus. Eleven out of 13 underwent testing with insulin-induced hypoglycaemia (nadir plasma glucose 30 mU/l. Intermediate values were taken to represent subnormal GH status. Assessment of GH status was performed 39 +/- 10 months (median +/- SE) following successful treatment (range 9-108 months). RESULTS: Using these criteria 4/13 (31%) patients had severe GH-deficiency. Only 2/13 (15%) had a normal response. 7/13 (54%) achieved peak GH levels in the subnormal range. Those with multiple pituitary hormone deficiencies were most likely to have lower peak GH levels, but there was no clear effect of pituitary irradiation or relationship between duration post cure and peak GH response. CONCLUSION: GH-deficiency is common and may persist for many years following successful treatment of CD prior to completion of linear growth. External radiotherapy does not necessarily result in severe GH-deficiency in the short term. Assessment of GH status and consideration of GH treatment should be considered following treatment of CD in childhood and adolescence in order to maximize the opportunities to achieve a satisfactory final adult height. In those with subnormal GH responses, continued assessment is necessary to determine whether the GH axis subsequently recovers or if these patients develop features of the adult GH-deficiency syndrome.


Subject(s)
Cushing Syndrome/therapy , Growth Hormone/deficiency , Adolescent , Child , Cushing Syndrome/blood , Cushing Syndrome/diagnosis , Female , Follow-Up Studies , Glucagon , Glucocorticoids/administration & dosage , Growth Hormone/blood , Humans , Hydrocortisone/blood , Insulin , Male , Pituitary Gland/surgery , Retrospective Studies
12.
Br J Neurosurg ; 18(5): 500-5, 2004 Oct.
Article in English | MEDLINE | ID: mdl-15799153

ABSTRACT

The case is described of a 21-year-old woman, who developed a malignant tumour arising from a craniopharyngioma 14 years after the original diagnosis. The remarkable response of this malignant tumour ex-craniopharyngioma to cis-platin based chemotherapy, together with other midline tumour characteristics of craniopharyngioma, raise the question as to whether craniopharyngioma should any longer be separately considered from suprasellar germ cell tumour. This subject is discussed.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Craniopharyngioma/drug therapy , Pituitary Neoplasms/drug therapy , Adolescent , Adult , Child , Chromosome Aberrations , Cisplatin/administration & dosage , Craniopharyngioma/genetics , Craniopharyngioma/pathology , Etoposide/administration & dosage , Fatal Outcome , Female , Follow-Up Studies , Humans , Magnetic Resonance Imaging , Neoplasm Recurrence, Local/drug therapy , Pituitary Neoplasms/genetics , Pituitary Neoplasms/pathology
13.
Br J Neurosurg ; 17(3): 260-2, 2003 Jun.
Article in English | MEDLINE | ID: mdl-14565527

ABSTRACT

Chordomas are relatively rare neoplasms that appear to originate from benign remnants of primitive notochord. They account for about 0.15% of all intracranial neoplasm and are the most common of extradural neoplasms that involve the clivus. Although they present mostly with signs and symptoms of other intracranial lesions they rarely present with high prolactin levels mimicking prolactinomas. We report a case of chordoma presenting as a pseudoprolactinoma. We discuss the presentation, radiological features and treatment of this rare presentation.


Subject(s)
Chordoma/diagnosis , Pituitary Neoplasms/diagnosis , Prolactinoma/diagnosis , Adult , Chordoma/surgery , Female , Headache Disorders/etiology , Humans , Pituitary Neoplasms/surgery , Prolactinoma/surgery , Treatment Outcome , Vision Disorders/etiology , Vision Disorders/surgery
14.
J Clin Endocrinol Metab ; 87(12): 5465-9, 2002 Dec.
Article in English | MEDLINE | ID: mdl-12466338

ABSTRACT

Total bilateral adrenalectomy remains the definitive procedure for cure in Cushing's disease. It is complicated by the development of Nelson's syndrome, the treatment of which remains troublesome. We report the long-term follow-up, median 17 yr (range, 8-22 yr), of 13 patients (3 males and 10 females) treated with pituitary surgery for Nelson's syndrome at a median age of 35 yr (range, 21-67 yr). The presence of a pituitary mass lesion necessitated neurosurgery in all. Preoperatively, the median plasma ACTH level was 664 pmol/liter (range, 92-3665 pmol/liter); this fell to 29 pmol/liter (range, <2 to 1124 pmol/liter) postoperatively (P < 0.0005). Cutaneous hyperpigmentation was reduced in all and resolved in 11 patients. The pituitary tumor bulk was clearly reduced in 12 patients. There was no perioperative mortality. No patient developed a visual field defect attributable to surgery. New anterior pituitary hormone deficiency occurred in seven patients, and permanent diabetes insipidus occurred in five patients. At latest follow-up, the median plasma ACTH is 73 pmol/liter (range, <2 to 7759 pmol/liter); six patients have levels of less than 44 pmol/liter and also have a resolution of their pigmentation and no evidence of a recurrence of a pituitary mass lesion. We conclude that pituitary surgery is an efficacious treatment for mass lesions associated with Nelson's syndrome, has long-term benefit with minimal side effects, and must be considered in the management of this distressing complication.


Subject(s)
Hypophysectomy , Nelson Syndrome/surgery , Adrenocorticotropic Hormone/blood , Adult , Aged , Diabetes Insipidus/etiology , Female , Follow-Up Studies , Hormones/deficiency , Humans , Hypophysectomy/adverse effects , Male , Middle Aged , Pituitary Gland, Anterior/metabolism , Time Factors
16.
J Clin Endocrinol Metab ; 86(12): 5711-4, 2001 Dec.
Article in English | MEDLINE | ID: mdl-11739426

ABSTRACT

Selective transsphenoidal microadenomectomy is the first line treatment of childhood Cushing's disease, with accurate preoperative localization of the corticotroph adenoma an important step in its investigation. Inferior petrosal sinus sampling (IPSS) for ACTH after CRH stimulation is a recognized investigation in adults, but there are few data in the pediatric age range. We report the relative contributions of IPSS and pituitary imaging in 11 patients, aged 10.7-18.8 yr, presenting with Cushing's disease. All underwent transsphenoidal surgery (TSS). IPSS was performed without complication. Sampling was from the inferior petrosal sinuses in 7 patients and the high jugular veins in 4 (patients 2, 4, 5, and 10). The central to peripheral ACTH (IPS/P) ratios were more than 2 (2.5-157.2) in 10 of 11 patients, confirming central ACTH secretion. In 3 patients with high jugular sampling, IPS/P ratio ranged from 2.5-21.1. In the fourth patient with high jugular sampling (IPS/P ratio, 0.95), a central adenoma was identified surgically, and the patient was cured after TSS. The interpetrosal sinus ACTH gradient (IPSG) was more than 1.4 (2.1-20.8) in 10 patients, indicating lateralization of ACTH secretion to the right side in 6 patients and to the left in 4. IPSG ratios were 2.1-8.5 in 3 patients with high jugular sampling. Pituitary imaging (computed tomography and or magnetic resonance imaging) was reported to identify an adenoma in 5 of 11 patients. At operation a tumor was visualized by the same surgeon in all 11 cases. In 9 patients with lateralization on IPSS, the correct side of the tumor was confirmed at surgery. In a 10th patient with a negative IPSG, a central tumor was present. Thus, IPSS gave a 91% prediction of correct tumor localization. In only 1 of 5 patients with an adenoma reported on pituitary imaging was this localization confirmed at surgery, a prediction rate of only 9%. After TSS, 8 patients were cured, 1 was in remission, and 2 required pituitary irradiation. In 73% of patients undergoing IPSS, localization of the adenoma was followed by surgical cure or remission. Pituitary scanning was therefore relatively unhelpful in localizing the adenoma. In experienced hands, however, IPSS was feasible in this age group, safe, and strongly predictive of the site of the adenoma, leading to a high rate of successful surgical outcome.


Subject(s)
Adrenocorticotropic Hormone/physiology , Cushing Syndrome/diagnosis , Cushing Syndrome/physiopathology , Petrosal Sinus Sampling , Adenoma/complications , Adenoma/diagnosis , Adenoma/metabolism , Adenoma/surgery , Adolescent , Adrenocorticotropic Hormone/metabolism , Child , Cushing Syndrome/etiology , Female , Humans , Magnetic Resonance Imaging , Male , Pituitary Gland/diagnostic imaging , Pituitary Gland/pathology , Pituitary Neoplasms/complications , Pituitary Neoplasms/diagnosis , Pituitary Neoplasms/metabolism , Pituitary Neoplasms/surgery , Tomography, X-Ray Computed , Treatment Outcome
17.
Horm Res ; 55 Suppl 1: 24-30, 2001.
Article in English | MEDLINE | ID: mdl-11408758

ABSTRACT

Seventeen patients with Cushing's disease (CD) were treated from 1978 to 2000. There were 11 males and 6 females aged 6.8-18.8 years (mean age 13.0 +/- 5.9 years). Presenting features were: weight gain (100%); growth failure (71%); hirsutism (53%); striae (53%); hypertension (47%). Mean age of patients with striae was 15.2 +/- 2.3 years, without striae 10.3 +/- 3.3 years. Median height SDS was -1.81 (range -0.28 to -4.17), 53% having height SDS < -1.8. The height velocity in 6 subjects was subnormal (0.9-3.8 cm/year). Median BMI SDS was 2.29 (range 1.72-5.06). Cushing's disease was confirmed by detectable serum ACTH, median 28 ng/l (range 12-99, NR <10-50) (n = 15); loss of cortisol circadian rhythm values at midnight ranging from 216 to 1,080 nmol/l (NR <50) (n = 15); lack of cortisol suppression (NV < 50 nmol/l) during low-dose dexamethasone suppression test (LDDST) (0.5 mg 6-hourly x 8) (n = 14); and >50% suppression of cortisol compared with the basal value during high-dose dexamethasone suppression test (HDDST) (2 mg 6-hourly x 8) (n = 14). A CRH test (1 microg/kg i.v.) showed an increase of cortisol from 12 to 217% (median 73.5%) (n = 16). Pituitary imaging (CT/MRI) showed an image consistent with microadenoma in 6/17 patients, but there was concordance between pituitary imaging and surgical findings in 1/11 patients (9%). Inferior petrosal sinus sampling (IPSS) for ACTH after CRH was performed in 11 subjects (age 10.7-18.8 years). Central to peripheral ACTH ratios were >2 (2.5-157.2) in 10/11 patients. The inter-petrosal sinus ACTH gradient was >1.4 in 10 patients (2.1-20.8), indicating lateralization of ACTH secretion. In 10 patients (91%), the side of the tumour on IPSS was predictive of findings at surgery. Therapy consisted of transsphenoidal microadenomectomy (TSS) in 16 patients and bilateral adrenalectomy (1978) in 1. Following TSS alone, 7 patients were cured (cortisol <50 nmol/l) and 2 were in remission (cortisol <300 nmol/l), i.e. 56%. Seven had persisting hypercortisolaemia and underwent pituitary irradiation (4,500 cGy). Therapeutic outcome for a median of 8 years (0.5-24 years) resulted in cure of CD in 14/17 patients (82%) and remission in 1. Linear growth after TSS +/- pituitary irradiation in 10 subjects showed no short-term catch-up growth, with peak growth hormone (GH) 0.5-20.9 mU/l to insulin tolerance test (ITT)/glucagon. Eight patients were treated with human growth hormone (hGH) (14 U/m(2)/week) combined in 3 with GnRH analogue. The mean final (n = 6) or latest (n = 4) height SDS was -1.36. The difference between final/latest height SDS and target height SDS was 0.93 +/- 1.13, i.e. less (p = 0.005) than the difference between height SDS and target height SDS at presentation, i.e. 1.72 +/- 1.26, indicating long-term catch-up growth.


Subject(s)
Cushing Syndrome/diagnosis , Cushing Syndrome/therapy , Adolescent , Child , Cushing Syndrome/complications , Cushing Syndrome/physiopathology , Dexamethasone , Female , Follow-Up Studies , Glucocorticoids , Growth , Humans , Magnetic Resonance Imaging , Male , Pituitary Gland/radiation effects , Pituitary Gland/surgery , Postoperative Period , Radiotherapy , Tomography, X-Ray Computed , Treatment Outcome
18.
Br J Neurosurg ; 15(2): 147-50, 2001 Apr.
Article in English | MEDLINE | ID: mdl-11360380

ABSTRACT

Chordoid glioma, a rare tumour of the third ventricle, has distinctive histological appearances. Fewer than 20 cases have been reported in the literature, all but three in females. This paper describes a 54-year-old man with a chordoid glioma and reviews the clinicopathological features of this lesion.


Subject(s)
Cerebral Ventricle Neoplasms/diagnosis , Glioma/diagnosis , Cerebral Ventricle Neoplasms/chemistry , Cerebral Ventricle Neoplasms/surgery , Glial Fibrillary Acidic Protein/analysis , Glioma/chemistry , Glioma/surgery , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Neurosurgical Procedures , S100 Proteins/analysis , Third Ventricle , Tomography, X-Ray Computed , Vimentin/analysis
19.
J Clin Endocrinol Metab ; 86(4): 1645-52, 2001 Apr.
Article in English | MEDLINE | ID: mdl-11297598

ABSTRACT

Acromegaly is associated with increased morbidity and mortality unless serum GH levels are persistently less than 5 mU/L ( approximately 2 ng/mL) after treatment. Transsphenoidal surgical resection is the best available treatment for restoring GH to such "safe" levels; however, criteria for the assessment of the response to treatment are not uniform. To determine the clinically most useful method of assessing disease activity postoperatively and identify predictors of a favorable response to surgical treatment, we have analyzed 67 patients with acromegaly who underwent transsphenoidal surgery between 1993 and 1998. We used three different definitions of a satisfactory or safe response: 1) a postoperative mean GH less than 5 mU/L obtained from averaging five serum GH values obtained throughout one day; 2) a random single GH less than 5 mU/L; or 3) a serum insulin-like growth factor I (IGF-I) level within the normal range. Relying on a single GH measurement alone, 9 of the 23 patients with a single postoperative mean GH level less than 5 mU/L obtained at least one GH value of more than 5 mU/L (false positive rate, 28%) and 8 of the patients with a postoperative mean GH value of more than 5 mU/L obtained a single GH value of less than 5 mU/L (false negative rate, 15%). Postoperatively, a significant increase in the fluctuation of random GH values around the mean was observed in patients who were rendered safe (coefficient of variation, from 26 +/- 2% to 53 +/- 6%; P < 0.001) compared with patients with persistence of inadequately controlled disease. However, 13% (3 of 23) of patients with mean postoperative GH levels of less than 5 mU/L had elevated serum IGF-I levels postoperatively, and 17% (8 of 44) of patients with mean serum GH levels more than 5 mU/L had postoperative IGF-I levels within the normal range. There was no difference in the rate of agreement between mean GH less than 5 mU/L and normalization of IGF-I in relation to the interval since operation when IGF-I levels were measured. Preoperative tumor size and pretreatment mean GH levels were the major determinants of the outcome of surgery, as patients who were rendered safe had significantly lower preoperative mean GH levels than patients who were not cured (median, 31 mU/L vs. 78.5 mU/L, P < 0.01). IGF-I levels were weakly correlated with tumor size and could not be used to predict the patients who would be rendered safe. Preoperative PRL levels were higher in patients who failed to achieve a surgical satisfactory outcome [498 mU/L (187-857) vs. 196 mU/L (136-315), P < 0.01]. In summary, although single random GH values and IGF-I values are both significantly correlated with mean GH levels, they should not be used as an alternative to averaging several GH values to assess disease activity, because of the pulsatile nature of GH secretion and the multiple factors that may influence serum IGF-I. Because significant discrepancies occur, particularly postoperatively, mean GH levels remain the more reliable indicator of surgical outcome and disease activity. As there is considerably more evidence relating long-term prognosis to serum GH levels than to IGF-I and discrepancies occur between GH levels and IGF-I, we suggest that mean serum GH levels and single IGF-I levels, measured early in the postoperative period, are currently the best biochemical guide to the adequacy of surgery and, hence, the need for further treatment.


Subject(s)
Acromegaly/blood , Acromegaly/surgery , Human Growth Hormone/blood , Acromegaly/diagnostic imaging , Acromegaly/physiopathology , Adolescent , Adult , Aged , Aged, 80 and over , Child , Human Growth Hormone/metabolism , Humans , Middle Aged , Pituitary Gland/physiopathology , Pituitary Neoplasms/metabolism , Postoperative Period , Prognosis , Prolactin/metabolism , Tomography, X-Ray Computed , Treatment Outcome
20.
J Clin Endocrinol Metab ; 85(9): 3262-5, 2000 Sep.
Article in English | MEDLINE | ID: mdl-10999819

ABSTRACT

Cushing's disease is associated with growth failure in childhood and adolescence. Growth and final height were analyzed in 10 patients who were cured or in remission after treatment of Cushing's disease. Seven males and 3 females, aged 6.8-17.6 yr (bone age, 3.3-15.4 yr), had transsphenoidal surgery, which was combined with pituitary irradiation (4,500 cGy in 25 fractions) in 5 patients. At presentation, 5 patients were prepubertal (males), and 5 were pubertal (2 males and 3 females). The mean height SD score was -2.15 +/-1.26 (range, -0.21 to -4.32) compared with mean target height SD score of -0.43 +/- 0.58. Height velocity in 6 patients was subnormal (0.9-3.8 cm/yr). After treatment, short-term height velocity, over a mean interval of 0.57 yr, in 8 patients not receiving human GH (hGH) therapy, was variable (range, 0.8-7.6 cm/yr). GH stimulation tests (insulin tolerance test/glucagon) in 9 subjects showed peak GH levels of 0.5-20.9 mU/L. Eight were treated with hGH (14 IU/m2 wk), combined in 2 girls and 1 boy with a GnRH analog. After 1 yr of hGH, the mean height SD score had increased from -2.45 +/- 1.0 at initiation of hGH to -2.07 +/- 1.2 (P = 0.01). GH therapy was continued until final height or latest assessment. The mean final height SD score (n = 6) was - 1.24 +/- 1.38, and at the latest assessment the mean height SD score (n = 4) was - 1.52 +/- 1.33. Combining these 2 groups, the mean height so score was -1.36 +/- 1.29. The difference between final or latest height SD score and target height SD score was 0.93 +/- 1.13, i.e. less (P = 0.005) than the difference between height and target height SD score of 1.72 +/- 1.26 at presentation. In conclusion, catch-up and favorable long-term growth was seen after treatment for Cushing's disease. Posttreatment GH deficiency was frequent, and early hGH replacement may have contributed to the encouraging outcome.


Subject(s)
Body Height/physiology , Cushing Syndrome/therapy , Growth/physiology , Adolescent , Child , Cushing Syndrome/radiotherapy , Cushing Syndrome/surgery , Female , Growth Hormone/therapeutic use , Human Growth Hormone/blood , Humans , Male , Pituitary Gland/surgery
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