naif bawazeer. history this 40 yr old female, past medically free, para 2+0. patient cant speak or...
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History This 40 yr old female, past medically free,
para 2+0. patient cant speak or hear. She complain of headache especially in the
morning mainly frontal associated with blurred vision, amenorrhea and enlargement in hand and foot since 7 years.
Past 6 month symptoms progressed out of proportion and associated with lower limb weakness.
Otherwise unremarkable.
Physical Examination
Conscious, alert, with dysmorphic features including: protruded jaw, macroglossia, big hands and feet.
Otherwise unremarkable.
Investigation
CBC and chemistry: with normal range Glucose: 126 mg/dl Alk pho:154 U/L (high) Urea and creatinie: with normal range. Growth hormone: 40mcg/L (very high) Prolactin : 23.6 ng/mL (high) FSH: 6.3 mIU/ml ECG: Sinus rhythm.
Management
Surgical removal of tumor by:
Endoscopic Transsphenoidal Pituitary Surgery
Post- Operative: no cerebrospinal fluid leak or seizure Hormonal replacement therapy. Follow up.
Phases of the Operation
Nasal stage (approaching tumor). Sellar stage (resection of tumor). Reconstruction phase (closure).
Definition
Excessive growth hormone (GH) after epiphyseal plate closure at puberty. Causing enlargement of hands, feet, and facial features
Epidemiology
Incidence about 3-4 per million per year. Mean age at presentation 44 years. Extrapolation:
Incidence in Saudi Arabia
Prevalence in Saudi Arabia
Causes
Endogenous sources:1- pituitary adenoma (90%).
2- familial syndromes: multiple endocrine neoplasia (MEN) type I, McCune-Albright syndrome, Carney's syndrome.
3- pituitary carcinoma .
4- peripheral neuroendocrine tumors.
Exogenous sources.
History and Physical
In case of pituitary adenoma: Mass effect:
headaches, cranial nerve palsy, visual field defects
+ Hormonal effect:
GH: increase in soft tissue, CTS (60%)
Prolactin: Amenorrhoea.
TSH: Hyperthyroidism.
ACTH: Cushing's syndrome
Apoplexy: sudden deterioration.
History and Physical
Other causes of acromegaly: according on underlying cause will give
us the clinical picture.
+ Hormonal effect:
GH: increase in soft tissue, CTS (60%)
Diagnosis
Clinical suspicion Biochemical marker:
elevated insulin-like growth factor 1 (IGF-1)elevated growth hormone (GH < 1 ng/mL )
Radiology:
MRI of pituitary gland to detect tumor and evaluation.
Treatment American Association of Clinical Endocrinologists Medical
Guidelines for Clinical Practice for the Diagnosis and Treatment of Acromegaly-2011 Update
http://aace.metapress.com/content/5h1427154k550851/?id=5H1427154K550851
Treatment
In case of unresectable pituitary tumour (with neural or vascular impingement/invasion): Medical therapy:
somatostatin analogs dopamine agonists
growth hormone-receptor antagonist (GHRA)
Debulking surgery Radiotherapy
Follow up:
Hormonal replacement therapy. blood investigation for hormones levels. MRI (evaluate recurrence). Colonoscopy.
References 1- Cook DM, Ezzat S, Katznelson L, et al (AACE Acromegaly Guidelines Task Force). AACE
medical guidelines for clinical practice for the diagnosis And treatment of acromegaly [published corrections Appear in Endocr Pract. 2005;11:144 and Endocr Pract. 2008;14:802-803]. Endocr Pract. 2004;10:213-225.
Javer, A., Marglani, O., Lee, A., Matishak, M., & Genoway, K. (2008). Image-guided endoscopic transsphenoidal removal of pituitary tumours. Journal Of Otolaryngology - Head & Neck Surgery = Le Journal D'oto-Rhino-Laryngologie Et De Chirurgie Cervico-Faciale, 37(4), 474-480.
2- Johnson N. New approaches to the development and use of treatment guidelines. Formulary. 1998;33:665-678.
3. Mechanick JI, Bergman DA, Braithwaite SS, Palumbo PJ (American Association of Clinical Endocrinologists Ad Hoc Task Force for Standardized Production of Clinical Practice Guidelines). American Association of Clinical Endocrinologists protocol for
standardized production of clinical practice guidelines [published correction appears in Endocr Pract. 2008;14:802-803]. Endocr Pract. 2004;10:353-361.
4. Alexander L, Appleton D, Hall R, Ross WM, Wilkinson R. Epidemiology of acromegaly in the Newcastle region. Clin Endocrinol (Oxf). 1980;12:71-79.
5. Daly AF, Rixhon M, Adam C, Dempegioti A, Tichomirowa MA, Beckers A. High prevalence of pituitary adenomas: a cross-sectional study in the province of Liege, Belgium. J Clin Endocrinol Metab. 2006;91:47694775.
6. Avagnina P, Martini M, Terzolo M, et al. Assessment of functional liver mass and plasma flow in acromegaly before and after long-term treatment with octreotide. Metabolism. 1996;45:109-113.