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Thyroid and Parathyroid Glands  265

          be considered in any child with an unexplained growth spurt, sympathy   Hypothyroidism
          (e.g.,  muscle  weakness,  paraesthesia)  or  behavioural  problems.  The   Hypothyroidism  is  a  clinical  state  in  which  there  is  reduced  thyroid
          gland is uniformly enlarged, smooth, firm, and nontender. It may be so   hormonal  activity.  This  is  rarely  due  to  thyroid  hypofunction  sec-
          vascular that a bruit is audible over it.              ondary  to  reduced  TSH  stimulation  resulting  from  hypopituitarism.
            Laboratory  evaluation  generally  reveals  elevated  free  T   and   Hypothyroidism may result from a defect anywhere in the hypothalam-
                                                         4
          decreased  TSH  levels.  In  10–20%  of  patients,  only  the  T   level  is   ic-pituitary-thyroid axis (Table 40.1).
                                                      3
          elevated, a condition referred to as T  toxicosis. The diagnosis of Graves’
                                    3                            Table 40.1: Causes of hypothyroidism.
          disease is established by the presence of TSH-receptor antibodies.
                                                                  Type of
          Management                                              hypothyroidism  Cause
          The treatment of Graves’ disease is palliative, with the goal to allow
                                                                                 Following subtotal thyroidectomy
          natural resolution of the underlying autoimmune process. The natural
                                                18
          course of untreated Graves’ disease is unpredictable.  The treatment is   Hypophysectomy
                                                                  Iatrogenic
          designed to reduce the production and secretion of the thyroid hormone.   Radio-iodine treatment for thyroitoxicosis
          This could be specific or nonspecific. Specific measures include the use
                                                                                 Excessive ingestion of para-aminosalicylic (PAS) acids,
          of  antithyroid drugs (carbimazole, neomercazole, potassium perchlo-   phenylbutazone, or antithyroid drugs
          rate). Nonspecific measures include rest and sedation.
                                                                  Iodine deficiency  Area of endemic goiters
            Initial therapy is with methimazole or propylthiouracil, which reduces
          thyroid hormone production by inhibiting follicle cell organification of   Autoimmune   Secondary to thyroid antibodies
          iodide and the coupling of iodotyrosines. Propylthiouracil also inhibits   thyroiditis
          peripheral conversion of T  to T , and may be the drug of choice if rapid   Deficiency or absence of enzymes needed for thyroid
                             4   3                                Dyshormonogenesis
          alleviation of thyrotoxicosis is desired. Both agents may possess some   hormone synthesis
          immunosuppressive  activity.  Methimazole  is  preferred  in  most  cases
                                                                                 Absence of thyroid gland (very rare) or ectopic thyroid
          due to its increased potency, longer half-life, and associated improved   gland
          compliance.  The  initial  dose  in  adolescents  is  30  mg  once  daily,   Congenital  Antenatal goitrogens
          adjusted for younger patients. The dosage is reduced to 10 mg when the
                                                                                 Pituitary-hypothalamic disease
          patient becomes euthyroid with normal T  and T .
                                        3    4
            The thyroid gland decreases in size in about half of the patients.
          Thyroid enlargement with therapy signals either an intensification of   Congenital hypothyroidism
          the disease or hypothyroidism with overtreatment. 18
                                                                 Ninety percent of paediatric hypothyroidism is congenital, detected by
            In general, the disease remission rate is approximately 25% after 2
                                                         22
          years of treatment, with a further 25% remission every 2 years. The   neonatal screening programmes, and results from dysgenesis of the thy-
                                                                 roid gland. Screening programmes have dramatically altered detection
          resolution rate is decreased if TSH-receptor antibodies persist during
                                                                 and management of congenital hypothyroidism. In the United Kingdom,
          and after treatment.
                                                                 all newborns are screened for congenital hypothyroidism as part of a
          Surgery                                                national screening programme, which also includes tests to exclude phe-
          Surgery is usually contraindicated in children due to the high postopera-  nylketonuria and cystic fibrosis. The worldwide incidence of congenital
          tive incidence of hypothyroidism (35%), recurrence, tetany (17%), and   hypothyroidism is reported as 1 in 4,000 infants. However, the true inci-
          of permanent hypoparathyroidism (10%). 12              dence is lower in African Americans and higher in Hispanic and Native
            Indications for surgery in children with Grave’s disease include:   American populations.  Two-thirds of these babies have a rudimentary
                                                                                 19
           • idiosyncratic reaction to antithyroid drugs;        gland, and complete absence of thyroid tissue is noted in the rest of the
                                                                 patients. The rudimentary gland may be ectopic. 18
           • progressive enlargement of the gland, even in a euthyroid state;
                                                                   The severe form of hypothyroidism in children is cretinism, which is
           • contraindication to radioactive iodine;             also congenital, and the child may be born with or without a goiter. The
                                                                 child  is  usually  underdeveloped  both  physically  and  mentally. There
           • recurrent hyperthyroidism;
                                                                 may be associated deafness, mutism, and neuromotor disorders (e.g.,
           • patients who refuse radioiodine;                    spastic paraplegia, dysarthria). 12
           • failed medical therapy; and                         Evaluation
                                                                 Infants with congenital hypothyroidism are often normal size at birth,
           • large thyroid gland compressing the airway.
                                                                 which is a reflection of the fact that thyroid hormones do not appear
            Surgery  in  the  form  of  either  subtotal  thyroidectomy  or  total   to be necessary for foetal growth. Physical features are not apparent in
          thyroidectomy  in  the  suitably  prepared  patient  may  be  performed.   the first week of life. Prolonged neonatal jaundice is usually the first
          Preoperative antithyroid medication should be administered to decrease   symptom,  followed  by  feeding  problems,  lethargy,  constipation,  and
          T   and  T   levels  to  the  normal  range.  Beta-blocking  agents  such  as   poor tone. Examination often reveals coarse facies; a large protruding
           3     4
          propranolol  may  be  used  to  ameliorate  the  adrenergic  symptoms  of   tongue; large open fontanelles; a hoarse cry; coarse, dry, and mottled
          hyperthyroidism.                                       skin; umbilical hernia; and delayed growth. In severe cases, these fea-
            In addition, Lugol’s iodine solution, 5 to 10 drops per day, should   tures appear within 4 to 8 weeks of birth. 24
          be  administered  for  4  to  7  days  before  thyroidectomy  to  reduce  the   Serum  T ,  T ,  and  resin  uptake  are  decreased,  whereas  TSH  is
                                                                             3
                                                                          4
          vascularity of the gland.                              elevated. Assessment of skeletal age (by x-ray of the knee) may show
            The  incidence  of  hypothyroidism  after  subtotal  thyroidectomy  is   bone maturation of less than 36 weeks gestation, suggesting intrauterine
          12–54%,  and  the  hypothyroidism  may  be  subclinical  in  up  to  45%   hypothyroidism.
          of children.  The rate of recurrent hyperthyroidism is approximately   Treatment
                   23
          13%. The relapse rate may increase with time after surgery. Near total   Treatment  is  by  lifetime  thyroid  hormone  replacement.  Synthetic
          thyroidectomy is advocated by some authors. 2          (laevo-) thyroxin is used at a dosage of 10 mg/kg per day, starting with
                                                                 25 mg per day and increasing to 100 mg per day.
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