Adil Azezli

Surgical Management of Substernal Goiters: Clinical Experience of 170 Cases

PurposeTo discuss the presentation, diagnosis, treatment, histopathological findings, and complications of patients who underwent thyroidectomy for substernal goiter in our surgical clinic.MethodsWe retrospectively analyzed 170 patients with substernal goiters among 2650 patients undergoing surgical treatment for various thyroid diseases between 1990 and 2003. We evaluated the clinical data, preoperative diagnostic findings, surgical treatments, histopathological results, and postoperative complications.ResultsThe most common symptoms were a cervical mass (88%) and dyspnea (35%), but 26% of the patients were asymptomatic. Chest radiography provided the first evidence of a substernal goiter in 77% of the patients. We performed total or near total thyroidectomy and operated through a cervical incision in all but 12 of the patients. There was no operative mortality but 12 (7%) patients suffered temporary hypoparathyroidism and 4 suffered transient vocal cord paralysis (2%). Malignancy was diagnosed by histopathological examination in 22 (13%) patients.ConclusionWe think that the diagnosis of a substernal goiter is an indication for thyroidectomy, which is associated with very low postoperative morbidity.

Severe hyperthyroidism requiring therapeutic plasmapheresis in a patient with hydatidiform mole.

A 38-year-old woman had a 4-week history of vaginal bleeding, heat intolerance and palpitations. Levels of β-human chorionic gonadotropin and thyroid hormones were abnormally high. After ultrasound diagnosis of a molar pregnancy, evacuation of the mole was planned with preoperative treatment involving the use of antithyroid drugs and plasmapheresis. Plasmapheresis was used to prepare for surgery in our patient who needed more rapid hormonal control. In conclusion, early diagnosis of molar pregnancy results in decreased incidence of significant complications related to hyperthyroidism.

An unusual patient with hypercalciuria, recurrent nephrolithiasis, hypomagnesemia and G227R mutation of Paracellin-1. An unusual patient with hypercalciuria and hypomagnesemia unresponsive to thiazide diuretics.

A 19-year-old female patient with hypercalciuria and recurrent nephrolithiasis/urinary tract infection unresponsive to thiazide type diuretics is presented. The patient first experienced nephrolithiasis at the age of 4 years. Afterwards, recurrent passages of stones and urinary tract infection occurred. On diagnostic evaluation at the age of 19 years, she also had hypocitraturia and hypomagnesemia. Her serum calcium concentrations were near the lower limit of normal (8.5–8.8 mg/dl; normal range: 8.5–10.5), her serum magnesium concentrations were 1.15–1.24 mg/dl (normal range: 1.4–2.5) and urinary calcium excretion was 900 mg/24 h. PTH concentrations were increased (110–156 pg/ml; normal range: 10–65). We tried to treat the patient with hydrochlorothiazide at a dose of 50 mg/day. During treatment with thiazide diuretics, PTH concentration remained high and the patient had recurrent urinary tract infections and passages of stones. Serum magnesium concentration did not normalize even under the parenteral magnesium infusion. Her mother had a history of nephrolithiasis 20 years ago. Severe hypomagnesemia in association with hypercalciuria/urinary stones is reported as a rare autosomal recessive disorder caused by impaired reabsorption of magnesium and calcium in the thick assending limp of Henle’s loop. Recent studies showed that mutations in the CLDN16 gene encoding paracellin-1 cause the disorder. In exon 4, a homozygous nucleotide exchange (G679C) was identified for the patient. This results in a point mutation at position Glycine227, which is replaced by an Arginine residue (G227R). The mother was heterozygous for this mutation. G227 is located in the fourth transmembrane domain and is highly conserved in the claudin gene family. This case indicates the pathogenetic role of paracellin-1 mutation in familial hypomagnesemia with hypercalciuria and nephrocalcinosis and further underlines the risk of stone formation in heterozygous mutation carriers.

The Use of Konjac Glucomannan to Lower Serum Thyroid Hormones in Hyperthyroidism

Objective: Patients with hyperthyroidism occasionally need rapid restoration to the euthyroid state. In wiew of the increased enterohepatic circulation of thyroxine (T4) and triiodothyronine (T3) in thyrotoxicosis, and metabolic effects of konjac glucomannan in gastrointestinal system, we aimed to determine the activity of glucomannan in treatment of hyperthyroidism. Methods: A prospective, randomized, placebo-controlled, one-blind study design was used with newly diagnosed 48 hyperthyroid patients (30 patients with Graves’ disease and 12 with multinodulary goitre). They were assigned to one of the following treatment groups: I) methimazole 2×10mg, propranolol 2×20mg, and glucomannan (Propol) 2×1.3gr daily for two months; II) methimazole 2×10mg, propranolol 2×20mg, and placebo powder daily for two months. Results: No differences were detected from the point of view of the baseline thyroid hormone levels between groups (p > 0.05). Further analyses revealed that the patients receiving glucomannan at the end of the second, fourth and sixth weeks of the study had significantly lower serum T3, T4, FT3 and FT4 levels than the patients who received placebo (p < 0.05). TSH was not different between the two groups at any specific time (p > 0.05). At week 8, thyroid hormone levels were not shown any differences. The glucomannan-treated group had a more rapid decline in all four serum thyroid hormone levels than the placebo-treated group. Conclusions: We believe our preliminary results indicate that glucomannan may be a safe and easily tolerated adjunctive therapeutic agent in the treatment of thyrotoxicosis. This combination therapy seems most effect during first weeks of treatment of a hyperthyroid patient.

Pituitary macroadenoma in Addison's disease

Long-standing primary failure of pituitary-dependent endocrine glands may lead to hyperplasia of the pituitary cells. These changes in the pituitary gland may be correlated with the severity and duration of target-endocrine gland insufficiency. Production of adrenocorticotrophic hormone by the pituitary tumour and modest hyperprolactinaemia may develop due to adrenocortical insufficiency, but production of prolactin by the pituitary tumour due to primary adrenal insufficiency is rare. A case study is presented, with primary adrenal insufficiency associated with hyperprolactinaemia and pituitary macroadenoma (most probably prolactinoma). Plasma levels of prolactin were found to decrease after glucocorticoid, mineralocorticoid and bromocriptine therapy.

Relationship between adrenal cortex pathology in cushing's syndrome and its response to the dexamethasone suppression test

Cushings syndrome is a severely disabling condition which can cause death if left untreated. Endogenous Cushings syndrome can be ACTH-dependent or ACTH-independent. The ACTH-dependent type is more common and is usually caused by diffuse hyperplasia on the adrenal cortex. This study investigated the response to low- and high-dose dexamethasone suppression testing of 30 adrenalectomized patients with Cushings syndrome, average age 37.3 ± 9.7 years. Twenty-four (79.3%) patients were female, and six (20.7%) were male. Bilateral adrenalectomy was performed in 14 (48.2%) patients and unilateral adrenalectomy (nine and seven right adrenalectomy) in 16 (51.8%). Two of the bilateral adrenalectomies were applied via endoscopic surgical approach. In the histopathological evaluation, diffuse hyperplasia was diagnosed in 13 (44.8%) patients and nodular hyperplasia in eight (26.6%), three macronodular and five micronodular hyperplasia. Adrenal cell adenoma was diagnosed in nine (28.6%) patients. Classic dexamethasone suppression testing was performed on all patients. Plasma levels of cortisol were not significantly decreased after low-dose testing, but plasma levels of cortisol were significantly decreased after high-dose testing in the diffuse hyperplasia group. In summary, due to the pathological changes of the adrenal cortex, dexamethasone suppression testing can differentiate between the two types of Cushings syndrome.