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Voice changes after thyroidectomy: role of the external laryngeal nerve.

Hitherto voice changes have been regarded as an infrequent complication of thyroidectomy and damage to the recurrent laryngeal nerve has been given as their major cause. Voice function was assessed in 325 patients after thyroidectomy. Permanent changes occurred in 35 (25%) after subtotal thyroidectomy and in 19 (11%) after lobectomy. The commonest cause of voice change appeared to be injury to the external laryngeal nerves on one or both sides. Damage to the recurrent laryngeal nerve, which was routinely identified and protected, was rarely a cause. When the external laryngeal nerves were identified and preserved, permanent voice changes occurred in only 5% of cases; this was similar to the incidence of 3% in controls after endotracheal intubation alone. The course of the external laryngeal nerve is variable, and consequently mass ligation of the vessels at the top of the upper pole will damage it in a high proportion of cases. To minimise this serious complication these nerves should be identified and protected as well as the recurrent nerves and voice function should be assessed early in the postoperative period by laryngoscopy and by a speech therapist.

Adolescent↗

Tomographic imaging of the human thyroid with a positron camera before and after partial thyroidectomy.

A high-density avalanche chamber positron camera was used for tomographic imaging of the human thyroid before and after partial thyroidectomy. Images were made between 6 and 24 h after oral administration of the positron-emitting radionuclide, Na-124I with activities varying between 0.1 and 0.3 mCi before the surgical intervention and with activities between 0.03 and 0.05 mCi following partial thyroidectomy. The results of thyroid imaging performed on 50 patients and their surgical relevance are discussed; as an illustration, one typical case is presented. As a consequence of the high spatial resolution of the camera (2.5 mm full width at half maximum), the functional volume of the thyroid may be estimated from the transaxial tomographic sections before and following partial thyroidectomy, correct to about 10%. The thyroid surface, defined by the contours from each transaxial section, may be displayed using three-dimensional shaded-graphics techniques. This new imaging technique makes possible a fully three-dimensional description of the thyroid in vivo and contributes significantly to the surgical follow-up.

Goiter, Nodular↗

Effect of thyroidectomy on 2,3,7,8-tetrachlorodibenzo-p-dioxin-induced lipid peroxidation.

Thyroid hormones have been implicated in the toxicity of 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD). Therefore, the effects of methimazole (MMI) and propylthiouracil (PTU) induced hypothyroidism and surgical thyroidectomy on several toxic manifestations of TCDD were investigated. Female rats were treated with MMI (0.50 mg/kg) for 10 or 28 days, or PTU (5.0 mg/kg) for 10 days. Other animals were surgically thyroidectomized. The animals received TCDD (100 micrograms/kg) orally or the corn oil vehicle 6 days prior to sacrifice. MMI and PTU decreased serum thyroxine (T4) levels by 27-33% while surgical thyroidectomy decreased T4 levels by 66%. TCDD alone decreased T4 levels by 67%, and similar effects occurred in MMI and PTU treated animals. TCDD produced a 9% increase in serum triiodothyronine (T3) concentrations, and neither MMI or PTU treatment for 10 days modified this effect. Neither antithyroid drug prevented TCDD induced weight loss. TCDD administration resulted in over a 300% increase in hepatic malondialdehyde (MDA) content and a 60% decrease in glutathione peroxidase activity. Neither antithyroid compound affected TCDD-induced alterations in these two parameters. TCDD enhanced MDA content by 220% and inhibited glutathione peroxidase activity by 39% in surgically thyroidectomized rats. Thus, only severe hypothyroidism produced by surgical thyroidectomy was able to partially prevent the effects of TCDD on hepatic MDA content and glutathione peroxidase activity.

Animals↗

Active prophylactic management of respiratory obstruction after standard thyroidectomy for giant goitre in the middle belt region of Nigeria.

Respiratory obstruction is a lethal complication of thyroidectomy for giant goitre. Prophylactic tracheal splintage by retaining the endotracheal tube in situ for 24 h post-operatively and its meticulous management in the intensive care unit (ICU) is a safe and rewarding practice. Over a 7-year period, 33 patients, all women who had standard thyroidectomy for giant goitre were managed accordingly. There was no incidence of post-operative respiratory distress, nor mortality in the series. The average duration of stay in ICU was 2 days for all patients and the average hospital stay was 6 days for 27 of the 33 patients (81.8%). We suggest that judicious post-thyroidectomy management of giant goitre patients in ICU with endotracheal tube in situ for 24 h improves their survival chances.

Adult↗

Effects of hypophysectomy, thyroidectomy, and thyroxine on specific prolactin receptor sites in kidneys and adrenals of male rats.

The effect of T4 on specific PRL binding in the kidneys and adrenals was measured in intact, hypophysectomized, and thyroidectomized male rats. PRL binding in the kidneys was reduced from 12.1 +/- 1.1% in the intact group to 4.5 +/- 0.5% after hypophysectomy; 4-day replacement with T4 returned PRL binding to intact values. T4 administered to intact rats significantly increased PRL binding above intact values. Thyroidectomy reduced PRL binding in the kidneys from 14.9 +/- 1.2% to 7.0 +/- 0.6%, and T4 treatment restored PRL binding to intact values. PRL binding was measured at 2, 3, 5, 7, and 10 days after thyroidectomy in kidneys and found to decrease progressively from 8.2+/-0.5% in the intact rats to 2.3+/-0.3% on day 10. A single injection of T4 doubled PRL recetor binding in kidneys of thyroidectomized rats at 12 h and returned binding to intact levels at 24 h. In contrast to the effects of T4 on kidney PRL binding, adrenal PRL binding was only slightly altered by thyroidectomy and T4. These results suggest that T4 has a specific role in regulating PRL receptors in the kidneys but not in the adrenals. Since PRL receptors in the adrenals were only slightly altered by thyroid status, it is believed that the changes in kidney receptors represent a specific rather than a general metabolic effect of T4.

Adrenal Glands↗

Painful Hashimoto's thyroiditis as an indication for thyroidectomy: clinical characteristics and outcome in seven patients.

Patients with chronic lymphocytic thyroiditis, or Hashimoto's thyroiditis (HT), usually present with goiter, hypothyroidism, or both. Thyroid pain and tenderness are rare and suggest an alternative diagnosis of subacute granulomatous thyroiditis or other forms of thyroiditis. We report seven patients with painful HT who had temporary or no relief from L-thyroxine replacement or steroid treatment and required surgical intervention for unremitting pain. Antithyroid antibodies were elevated in all except one patient, in whom fine-needle aspiration and surgical pathology were diagnostic of HT. All patients underwent either a subtotal or near-total thyroidectomy. Pathology showed lymphocytic thyroiditis in all specimens, with varying degrees of fibrosis. Giant cells and granulomas characteristic of subacute thyroiditis were not seen. After surgery, four patients obtained total and permanent relief of pain after subtotal (two patients) or near total (two patients) thyroidectomy, whereas one patient had minimal residual discomfort. One patient had relapse of pain, despite adjunct radioiodine ablation. In this patient with documented total thyroid ablation but persistent pain, the overall clinical picture suggested a strong psychological element in her medical problem. In conclusion, thyroidectomy is an uncommon but necessary therapy for patients with painful HT not responding to medical therapy.

Adult↗

Does thyroidectomy, radioactive iodine therapy, or antithyroid drug treatment alter reactivity of patients' T cells to epitopes of thyrotropin receptor in autoimmune thyroid diseases?

The effect of treatment on thyroid antibody production and T cell reactivity to thyroid antigens was studied in 15 patients with Graves' disease (GD) before and after thyroidectomy, 19 patients with GD before and after radioactive iodine (RAI) therapy, and 9 patients maintained euthyroid on antithyroid drugs (ATD). Twenty subjects matched for age and sex without known thyroid disease served as controls. In GD patients, the responses of peripheral blood mononuclear cells (PBMC) and TSH receptor (TSHR)-specific T cell lines to recombinant human TSHR extracellular domain, thyroglobulin, and TSHR peptides were examined on the day of surgery or RAI therapy (day 0) and also 6-8 weeks and 3-6 months thereafter. Reactivity to TSHR peptides before surgery was heterogeneous and spanned the entire extracellular domain. Six to 8 weeks after subtotal thyroidectomy, the number of patients' PBMC responding to any peptide and the average number of recognized peptides decreased. A further decrease in the T cell reactivity to TSHR peptides was observed 3-6 months after surgery. The responses of PBMC from Graves' patients before RAI therapy were less than those in the presurgical group. Six to 8 weeks after RAI therapy, the number of patients responding to any peptide and the average number of recognized peptides increased. Three to 6 months after RAI, T cell responses to TSHR peptides were less than those 6-8 weeks after RAI therapy, but still higher than the values on day 0. Responses of PBMC from patients with GD, maintained euthyroid on ATD, were lower than those before surgery or RAI therapy. The reactivity of T cell lines in different groups reflected a pattern similar to PBMC after treatment. TSHR antibody and microsomal antibody levels decreased after surgery, but increased after RAI therapy. The difference in the number of recognized peptides by patients' PBMC before RAI and surgery may reflect the effect of long term therapy with ATD in the patients before RAI vs. the shorter period in patients before surgery. The decreased T cell reactivity to thyroid antigens after thyroidectomy could be the result of removal of a major part of the thyroid gland or redistribution of suppressor-inducer T cells. The increased T cell response after RAI therapy is probably epitope specific, rather than a response to the whole TSHR molecule. Synchronous recognition of peptides 158-176 and 248-263 is important for the development of GD, and the loss of recognition of one of these epitopes may be an early sign of immune remission and a predictor of euthyroidism.

Adult↗

The role of needle aspiration biopsy in the selection of patients for thyroidectomy.

Fine needle aspiration (FNA) biopsy is gaining in popularity among all diagnostic tests used in the evaluation of patients for thyroidectomy. Its safety and accuracy have been well documented, but criteria regarding when FNA is appropriate are lacking. We retrospectively reviewed our experience with 408 consecutive patients evaluated for thyroid surgery. Two hundred seventy-three ultimately underwent thyroidectomy; 100 of these had preoperative FNA. An additional 135 patients underwent FNA without surgery. Patients who benefited from FNA were those in whom cytologic findings led to surgery for otherwise unsuspected malignancy (15), and those with asymptomatic benign nodules who avoided unnecessary thyroidectomy on the basis of FNA findings (96). FNA is superfluous when clinical conditions mandate surgery. These include nodules that arouse clinical suspicion of malignancy, massive nodules that cause aerodigestive tract compression or cosmetic disfigurement, and nodules extending into the mediastinum. All patients with asymptomatic, clinically benign thyroid nodules should undergo FNA biopsy, as approximately 50% can anticipate significant benefit from the procedure.

Biopsy, Needle↗

Safety of total thyroidectomy: review of 100 consecutive cases.

During a 6-year period (June 1982 to June 1988), 100 consecutive total thyroidectomies were performed at Mount Sinai Hospital, University of Toronto, for benign and malignant disease. There were five permanent complications: one deliberate sacrifice of the recurrent laryngeal nerve and four cases of persistent hypoparathyroidism. Four of these five complications occurred in extracapsular carcinoma and resulted from oncologic wide-field resection and/or deliberate sacrifice. One patient with benign disease suffered initially transient hypoparathyroidism which has now become permanent. Interestingly, two patients actually underwent deliberate sacrifice of a recurrent laryngeal nerve; one patient has made a surprising recovery at 16 months postoperative and now has full vocal cord mobility. Our experience suggests that the morbidity of total thyroidectomy relates primarily to the stage of malignancy and extracapsular extension, necessitating en bloc excision accompanied by additional lymph node dissection. The low incidence of permanent complications in benign thyroid disease suggests the feasibility of total thyroidectomy as the operation of choice when surgeons are familiar with the technique and indications.

Adult↗

[Clinical evaluations of subtotal thyroidectomy for Graves' disease].

An attempt was made to determine the clinical evaluation of subtotal thyroidectomy in 58 patients with Graves' disease. The weight of the remnant thyroid gland was measured during the operations. Postoperative thyroid volume was measured by ultrasonography (thyroid volume = pi abc/6; a is length, b width, and c thickness). Postoperative thyroid function including serum TSH, free triiodothyronine (FT3), free thyroxine (FT4), thyroglobulin (Tg), TSH receptor antibodies (TRAb), and antimicrosomal antibodies (MCHA) was examined. Fifty-eight patients were divided into the three groups, according to postoperative thyroid function; 39 (67.2%) in remission, 7 (12.1%) with relapse and 12 (20.7%) in a hypothyroid state. Postoperative thyroid volume in patients with relapse was significantly (p < 0.05) greater than that in patients in remission and in a hypothyroid state. Remnant thyroid weight in patients with relapse was significantly (p < 0.05) heavier than that in patients in remission and in a hypothyroid state. However, there was no significant difference in an estimated total thyroid weight among the three groups. A significant correlation was noted between the remnant thyroid weight and the postoperative thyroid volume (R = 0.58, p < 0.001). On the other hand, there was a significant correlation between serum Tg level and the postoperative thyroid volume (R = 0.45, p < 0.01). Serum level of Tg in patients with relapse was significantly (p < 0.05) higher than that in patients in remission and in a hypothyroid state. The prevalence of negative MCHA in patients in remission appeared relatively higher than that in patients with relapse and in a hypothyroid state. In patients with a remnant thyroid weight of less than 4.0 g, there was no recurrence following subtotal thyroidectomy, but a higher prevalence of hypothyroid state (43%) was observed compared to those of larger remnant thyroid weight. In patients with a remnant thyroid weight of 4.0 to 6.0 g, there was a lower prevalence of recurrence (5.9%) compared to those with a larger remnant thyroid weight, while most patients showed remission (73.5%). On the other hand, the highest prevalence of recurrence (23.5%) was obtained in patients with a remnant thyroid weight of more than 6.0 g. These observations indicate that postopertive thyroid state does not depend upon an estimated total thyroid weight, but depends upon a remnant thyroid weight. In addition, a remnant thyroid weight is closely associated with thyroid volume measured by ultrasonography after subtotal thyroidectomy, which reflected the serum level of Tg.

Graves Disease↗

Changes in serum TSH receptor antibody (TRAb) values in patients with Graves' disease after total or subtotal thyroidectomy.

TSH receptor antibodies (TRAb) are generally regarded as mediators of thyroid stimulation in Graves' disease. In addition, a high serum TRAb value during pregnancy is one of the risk factors for intrauterine death, prematurity, and fetal or neonatal hyperthyroidism. Recently, correlations between a high serum TRAb value and endocrine opthalmopathy were also suggested. Surgical resection of the thyroid is usually followed by a reduction of serum TRAb levels in variable degrees. The relation between the extent of the thyroidectomy and the degree of reduction is still controversial. In addition, the changes in the TRAb value after total thyroidectomy (TT) over a long period of time have never been studied. We studied the changes in serum TRAb values after TT and subtotal thyroidectomy (ST) for more than 7 years. Forty-one patients with Graves' disease underwent TT, and 99 patients underwent ST. The serum TRAb values and the ratio of the patients who achieved normal values among each group (normalization rates of TRAb) at 3 and 6 months, 1, 3, 5 and 7 years after surgery were compared between the TT group and ST group. The mean preoperative TRAb values were not significantly different between the TT and ST groups, and the mean TRAb values measured 3, 6 and 12 months after surgery were not significantly different between the groups. However, the TRAb values measured 3, 5 and 7 years after surgery were significantly (p<0.05) lower in the TT group than in the ST group (16.7 +/- 3.3% vs 28.0 +/- 2.6%, 12.6 +/- 3.4% vs 29.3 +/- 3.8%, 5.6 +/- 0.9% vs 25.4 +/- 4.1%, respectively). The normalization rates of TRAb were not significantly different between the groups until 1 year after surgery. However, the normalization rates 3, 5 and 7 years after surgery were significantly (p<0.05) higher in the TT group than in the ST group (65.7% vs 42.4%, 77.3% vs 46.7%, 100% vs 59.1%, respectively). The surgical complication rates of TT were similar to ST except for permanent hypoparathyroidism. TT is a treatment option for Graves' disease, especially in patients with a high TRAb value who wish to have children or who have Graves' opthalmopathy.

Adolescent↗

Rapid improvement of thyroid function by using glucocorticoid indicated for the preoperative preparation of subtotal thyroidectomy in Graves' disease.

Glucocorticoid therapy is not considered as an authentic method for obtaining euthyroid in Graves' disease. We tried the administration of prednisolone as a preoperative preparation for subtotal thyroidectomy in 4 hyperthyroid patients with Graves' disease who had suffered adverse effects of thionamide antithyroid drugs, including agranulocytosis, liver damage and skin eruptions. Following oral administration of a 30 mg daily dose of prednisolone, with or without other antithyroid reagents, both serum T4 and T3 concentrations decreased rapidly and reached the normal range within 2 weeks. The clinical signs and symptoms of hyperthyroidism also improved rapidly and subtotal thyroidectomies were performed uneventfully in all cases. These results suggest that 1) glucocorticoid medication can normalize the circulating hormone levels rapidly in Graves' disease, 2) it is a useful method as preoperative preparation for subtotal thyroidectomy, especially when other conventional methods are not available or effective in obtaining euthyroid, and 3) mechanisms other than thyroid stimulation by circulating immunoglobulin seem to play an important role in causing hyperfunction of the gland.

Adult↗

Effect of thyroidectomy upon seasonality in rams.

Experiments were conducted to examine whether seasonal breeding patterns of male sheep are abrogated by thyroidectomy. In Expt 1, Welsh Mountain rams were thyroidectomized in early autumn (September) and then maintained on either 16 h light: 8 h dark (long days; n = 6) or 8 h light: 16 h dark (short days; n = 6) for 8 months. Intact rams (n = 6 per group) were also housed in long or short days, or in natural photoperiods. Results were similar in animals housed on long or short days. In thyroidectomized rams, plasma FSH concentrations and scrotal circumference were maintained at values typical of the breeding season throughout the investigation, whereas in intact animals both reached a nadir in December and January. In Expt 2, a further 11 rams were thyroidectomized in March and, together with 23 intact animals, were maintained thereafter in natural photoperiods. In control rams, scrotal circumference increased slowly between May and September, whereas in thyroidectomized animals the circumference increased rapidly in the first 4 weeks following thyroidectomy (3.7 +/- 0.7 cm), with a further increase (5.9 +/- 1.0 cm) in the next 4 weeks. The scrotal circumference of thyroidectomized rams was therefore significantly (P < 0.01) greater than that in intact animals between April and August. Plasma FSH concentrations were significantly (P < 0.01) higher in thyroidectomized than in control rams by two weeks after surgery. These results indicate that thyroidectomy overcomes the seasonal (or photorefractory) inhibition of reproductive activity in rams and supports a key role for thyroid hormones in the expression of seasonal patterns of breeding activity.

Animals↗

Clinical and laboratorial correlation of postoperative hypocalcemia after extensive thyroidectomy.

The medical records of 84 patients submitted to extensive thyroidectomy from January 1991 to April 1995 were reviewed and the data was analyzed in order to verify a correlation between postoperative laboratories results and physical findings suggestive of hypocalcemia. It was verified that there was hypocalcemia in 51.2 percent of the patients, of which only 18.6 percent presented symptoms. It was concluded that asymptomatic hypocalcemia is frequent in extensive thyroidectomy and a routine screening for serum calcium in the postoperative period following thyroidectomy and calcium reposition must be systematic.

Adolescent↗

Effects of maternal iodine deficiency and thyroidectomy on basal neuroendocrine function in rat pups.

We have used in situ hybridization histochemistry to investigate the effects of maternal thyroidectomy and chronic maternal iodine deficiency on basal neuroendocrine function in rat pups. Specifically, we have measured hypothalamic thyrotrophin-releasing hormone (TRH) and pituitary thyroid-stimulating hormone (TSH) expression together with circulating levels of tri-iodothyronine (T3) in rat pups delivered from and suckled by thyroidectomized or iodine-deficient dams. Because of the close interaction between the thyroid, adrenal and growth hormone axes, we have also examined hypothalamic corticotrophin-releasing hormone (CRH) and growth hormone-releasing hormone (GRH) transcripts at the same time points: birth, 1 month and 2 months of age. Three weeks after surgical thyroidectomy, adult female Sprague-Dawley rats proved unable to carry pups to term and lactate successfully. Pups delivered from thyroidectomized dams given a small replacement dose of T3 during pregnancy were significantly lighter than controls (84 +/- 3%) and had markedly depressed plasma T3 levels (36 +/- 6% of control). Hypothalamic CRH and GRH transcript levels were significantly decreased in pups at birth (to 8 +/- 2.5% and 24 +/- 8% of control respectively) but had returned to normal by 1 month after delivery. Pituitary TSH transcript levels and hypothalamic levels of TRH transcripts, however, were similar to those of controls. Only one of seven dams fed a low-iodine diet for 6 months produced live pups, and these were too few in number to produce significant data. Dams fed a low-iodine diet from 4 months before mating, however, did produce live pups and although they were not significantly lighter than control pups at birth, by 1 month after birth, they were significantly lighter (72 +/- 3% of controls). Circulating T3 levels were not significantly different from control at any time point examined. Hypothalamic TRH levels were significantly elevated at birth (451 +/- 138% of control), but this difference was not maintained at 1 or 2 months after birth despite the lactating dams being maintained on the low-iodine diet. Pituitary TSH levels showed an upward trend at all time points that reached significance at 1 month after birth (204 +/- 19%; P < 0.05). Hypothalamic CRH and GRH transcript levels were not different from controls at any time point. In summary, chronic iodine deficiency or thyroidectomy with low-level T3 replacement in Sprague-Dawley rats markedly impaired fertility and the ability to carry pups to term, and produced an unexpectedly modest up-regulation of the hypothalamo-pituitary-thyroid axis and down-regulation of the hypothalamo-pituitary-adrenal axis.

Animals↗

Prevention of postoperative nausea and vomiting after thyroidectomy: combined antiemetic treatment with dexamethasone and ginger versus dexamethasone alone.

BACKGROUND: The aim of this study was to compare the prophylactic effects of dexamethasone plus ginger and dexamethasone alone on postoperative nausea and vomiting (PONV) in patients undergoing thyroidectomy. METHODS: One hundred and twenty patients undergoing general anaesthesia for thyroidectomy were enrolled in this randomised, double-blind study. Patients received oral diazepam 10mg with either oral placebo (group I) or 0.5g of ginger (group II) as premedication 1 hour prior to surgery. Standard general anaesthetic techniques and postoperative analgesia were employed. Both group I and group II received intravenous dexamethasone 150 microg/kg immediately before the induction of anaesthesia. Data were recorded over a 24-hour observation period after surgery. RESULTS: In the dexamethasone-treated group, 14 patients experienced nausea, two patients retched, three patients vomited once, two patients vomited repeatedly, and 14 patients required a rescue antiemetic. In the dexamethasone-plus-ginger-treated group, 12 patients experienced nausea, one patient retched, four patients vomited once, no patients vomited repeatedly, and 13 patients required a rescue antiemetic. Dexamethasone plus ginger did not significantly reduce nausea and vomiting compared with dexamethasone alone during the observation period. CONCLUSION: In conclusion, the prophylactic combination of antiemetic treatment with dexamethasone and ginger was not clinically or statistically superior to dexamethasone alone in preventing PONV in patients undergoing thyroidectomy.

Administration, Oral↗

Postoperative parathyroid hormone levels in conjunction with corrected calcium values as a predictor of post-thyroidectomy hypocalcemia: review of outcomes 1 year after the implementation of a new protocol.

OBJECTIVES: To determine the effectiveness of post-thyroidectomy parathyroid hormone (PTH) levels in conjunction with corrected calcium values as predictors of patients at risk of developing hypocalcemia. METHODS: This is a follow-up study reviewing the results of a newly implemented post-thyroidectomy algorithm. The changes in management from the previous protocol involve decision making based on the 12-hour corrected calcium and PTH levels, as well as the 1-hour PTH value. The study involved 120 patients separated into two groups: 60 prior to implementation of the protocol and 60 following the implementation of the protocol. Patients having completion thyroidectomy, neck dissections, or parathyroidectomy were excluded. RESULTS: Since the implementation of the new protocol, there has been a reduction in the rate of transient hypocalcemia (25% to 12%; p = .059), fewer blood tests (23 to 15 per patient), and earlier patient discharges. CONCLUSIONS: The new algorithm is effective in detecting patients who are not at risk of developing hypocalcemia at 12 hours. This has led to significant cost savings at our institution. Moreover, calcium supplementation based on the 1-hour PTH level has coincided with a reduction in cases of transient hypocalcemia.

Adult↗

Genetic testing and early thyroidectomy for inherited medullary thyroid carcinoma.

The recent identification of mutations in the RET proto-oncogene that are associated with multiple endocrine neoplasia type 2 (MEN 2) syndromes has allowed therapeutic intervention in affected individuals on the basis of direct genetic testing. The principal endocrine neoplasm that occurs in patients with the MEN 2 syndromes is medullary thyroid carcinoma. This thyroid neoplasm is the only consistently malignant feature of the MEN 2 syndromes and it is the most common cause of death in affected patients. Kindred members at risk for one of the MEN 2 syndromes can be studied by direct DNA analysis to determine whether they have inherited a RET mutation. Those with a positive test can be treated by early thyroidectomy and cured when the disease is microscopic and localized to the thyroid gland. Total thyroidectomy is performed as early as 5 years of age and is associated with minimal morbidity and virtually no mortality. Residual or persistent medullary thyroid carcinoma following thyroidectomy can best be determined by detecting increased levels of calcitonin following the administration of intravenous calcium gluconate and pentagastrin.

Adolescent↗