Physiology
Endocrine
What is the pathophysiology of a phaeochromocytoma:
Answer:
Phaeochromocytomas are catecholamine-secreting tumours.Phaeochromocytoma
Physiology / Endocrine / Adrenal Function
Last Updated: 21st April 2019
Phaeochromocytomas are catecholamine-secreting tumours which occur in about 0.1% of patients with hypertension. In about 90% of cases they arise from the adrenal medulla. The remaining 10%, which arise from extra-adrenal chromaffin tissue, are termed paragangliomas.
Clinical Features
Common presenting symptoms include one or more of headache, sweating, pallor and palpitations. Less commonly, patients describe anxiety, panic attacks and pyrexia. Hypertension, whether sustained or episodic, is present in at least 90% of patients. Left untreated phaeochromocytoma can occasionally lead to hypertensive crisis, encephalopathy, hyperglycaemia, pulmonary oedema, cardiac arrhythmias, or even death. Patients with undiagnosed phaeochromocytoma having routine surgery can develop severe hypertension or sudden death.
Diagnosis
Diagnosis relies on the biochemical confirmation of elevated catecholamines or their metabolites (metanephrines), followed by radiological localisation of the tumour.
Management
The definitive treatment is surgical excision. In advance of the surgery, it is mandatory that all patients are protected from the effects of catecholamine excess by alpha-blockade with or without beta-blockade. Alpha-blockade, conventionally administered as oral phenoxybenzamine, should be commenced before beta-blockade in order to avoid unopposed alpha-adrenergic stimulation and the risk of hypertensive crisis. Beta-blockers can be introduced subsequently to control reflex tachycardia.
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- Biochemistry
- Blood Gases
- Haematology
| Biochemistry | Normal Value |
|---|---|
| Sodium | 135 – 145 mmol/l |
| Potassium | 3.0 – 4.5 mmol/l |
| Urea | 2.5 – 7.5 mmol/l |
| Glucose | 3.5 – 5.0 mmol/l |
| Creatinine | 35 – 135 μmol/l |
| Alanine Aminotransferase (ALT) | 5 – 35 U/l |
| Gamma-glutamyl Transferase (GGT) | < 65 U/l |
| Alkaline Phosphatase (ALP) | 30 – 135 U/l |
| Aspartate Aminotransferase (AST) | < 40 U/l |
| Total Protein | 60 – 80 g/l |
| Albumin | 35 – 50 g/l |
| Globulin | 2.4 – 3.5 g/dl |
| Amylase | < 70 U/l |
| Total Bilirubin | 3 – 17 μmol/l |
| Calcium | 2.1 – 2.5 mmol/l |
| Chloride | 95 – 105 mmol/l |
| Phosphate | 0.8 – 1.4 mmol/l |
| Haematology | Normal Value |
|---|---|
| Haemoglobin | 11.5 – 16.6 g/dl |
| White Blood Cells | 4.0 – 11.0 x 109/l |
| Platelets | 150 – 450 x 109/l |
| MCV | 80 – 96 fl |
| MCHC | 32 – 36 g/dl |
| Neutrophils | 2.0 – 7.5 x 109/l |
| Lymphocytes | 1.5 – 4.0 x 109/l |
| Monocytes | 0.3 – 1.0 x 109/l |
| Eosinophils | 0.1 – 0.5 x 109/l |
| Basophils | < 0.2 x 109/l |
| Reticulocytes | < 2% |
| Haematocrit | 0.35 – 0.49 |
| Red Cell Distribution Width | 11 – 15% |
| Blood Gases | Normal Value |
|---|---|
| pH | 7.35 – 7.45 |
| pO2 | 11 – 14 kPa |
| pCO2 | 4.5 – 6.0 kPa |
| Base Excess | -2 – +2 mmol/l |
| Bicarbonate | 24 – 30 mmol/l |
| Lactate | < 2 mmol/l |
