Page 453 - Medicine and Surgery
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                                                                   Chapter 11: Disorders of the parathyroids 449


                  Management                                    Clinical features
                  Total parathyroidectomy possibly with autotransplanta-  Hypocalcaemiaandalkalosiscauseincreasedneuromus-
                  tion of parathyroid tissue equivalent to a normal gland  cularexcitability:paraesthesiasofthefingertipsandtoes,
                  into the arm, where it can be readily accessed for further  tetany (spasms of muscles of extremities and face)
                  treatment. Calcium replacement, phosphate binders and     Trousseau’s sign: Inflating a blood pressure cuff to
                  alfacalcidol(1-alphahydroxyvitaminD 3 )toincreasecal-  above systolic BP for at least 2 minutes causes carpal
                  cium absorption and serum levels may be effective by  spasm, which does not relax for a few seconds after
                  negative feedback on the parathyroids.         deflation.
                                                                 Chvostek’s sign: Tapping the facial nerve anterior to

                                                                 the ear lobe causes twitching of the facial muscles.
                  Hypoparathyroidism                               Convulsions occur more commonly in young people.
                  Definition
                  Adeficiency of parathyroid hormone (PTH) char-  Investigations
                  acterised by hypocalcaemia and hyperphosphataemia,  Low calcium with normal or high phosphate with no
                  with normal renal function.                   detectable PTH on immunoassay. Alkaline phosphatase
                                                                is normal. U&Es should be normal, or a renal cause is
                                                                suspected.
                  Aetiology
                  Most commonly occurs following surgery with removal
                                                                Management
                  of abnormal parathyroid glands or removal of neck ma-
                                                                Replacementtherapywith1,25(OH) 2 D 3 (calcitriol,vita-
                  lignancies.Glandfailuremaybecausedbydirectdamage
                                                                min D 2 )or 1(OH)D 3 .Serum and urinary calcium must
                  to the glands or their blood supply.
                                                                be measured, as hypercalcaemia and hypercalciuria can
                  Idiopathic hypoparathyroidism:
                                                                occur. Vitamin D intoxication causes irreversible renal
                    Genetic abnormalities are usually autosomal recessive

                                                                damage. Thiazide diuretics which increase renal tubular
                    and manifest at an early age. Associated with autoan-
                                                                reabsorption of calcium may be useful in treating hyper-
                    tibodies specific for parathyroid and adrenal tissue.
                                                                calciuria.
                    Associated autoimmune syndromes include perni-

                    cious anaemia, ovarian failure, autoimmune thyroidi-
                    tis, and diabetes mellitus.                 Prognosis
                    Late onset idiopathic hypoparathyroidism occurs
                                                                Lifelong treatment and follow-up.
                    without circulating autoantibodies.
                  Functional hypoparathyroidism occurs in patients with  Pseudohypoparathyroidism
                  chronic hypomagnesaemia which results in a failure of
                  PTH release.                                  Definition
                                                                This is a rare condition in which there is impaired re-
                                                                sponse to circulating parathyroid hormone, and hence
                  Pathophysiology
                                                                hypocalcaemia and hyperphosphataemia.
                  PTH is normally released in response to hypocalcaemia,
                  to restore calcium levels. The consequences of reduced
                  PTH are decreased calcium levels, increased phosphate  Aetiology
                  levels, decreased 1,25(OH) 2 D 3 and alkalosis (due to de-  Failure of the target cell response to parathyroid hor-
                  creased bicarbonate excretion).               mone, thought to be due to a PTH receptor defect or
                    In chronic cases of hypoparathyroidism, calcification
                                                                its coupling to the second messenger system, adenylate
                    of the basal ganglia causing extrapyramidal signs and  cyclase.
                    calcification of cornea may occur.
                    Cardiovascular problems with prolongation of the QT  Clinical features

                    interval in ECGs associated with hypocalcaemia, hy-  Round face, short stature with short fourth and fifth
                    potension and refractory congestive heart failure.  metacarpals and metatarsals. Other features are the same
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