Schmidt syndrome presenting as acute liver failure.

The Schmidt Syndrome (Type II Autoimmune-Syndrome) is characterised by an autoimmune adrenalitis in combination with a chronic lymphocellular thyreoiditis resulting in insufficiency of these organs in adulthood. Combination with diabetes is possible. The diagnosis is usually established by clinical examination and analysis of serum hormone levels (adrenocorticotropin hormone [ACTH], cortisol, thyroid stimulating hormone [TSH], triiodothyronine [fT3], thyroxine [fT4]). In the present case, initial diagnosis was rapid progressive liver failure of unknown origin with consecutive multiple organ dysfunction syndrome including dysfunction of heart, lungs, and kidneys. Frequent and less frequent causes of liver failure were ruled out, e.g. viral or autoimmune hepatitis, Budd-Chiari-syndrome, toxic, or drug induced liver failure. In retrospect, the multiple organ dysfunction syndrome was caused by hypoperfusion due to severe hypovolemia and hypoperfusion was induced by adrenocortical insufficiency proven by endocrinological testing. The clinical course of this case stresses the importance of the hormone balance in the critical ill patient. The guideline for treatment of patients with assumed hormonal dysregulation should include a full hormone status prior to substitution. The present case report also illustrates the importance of clinical signs and careful consideration of the medical history in detecting an autoimmune endocrine disease.

Non-functioning pituitary adenomas: endocrinological and clinical outcome after transsphenoidal and transcranial surgery.

AIM: To study improvement of anterior pituitary function after transsphenoidal and transcranial surgery of non-functioning (NF) pituitary macro- and microadenomas. METHODS: We retrospectively examined 155 patients with NF adenomas preoperatively and 3 months, 1 year and 2 years postoperatively. 130 patients harboured a macroadenoma, 109 underwent transsphenoidal (group one), 21 transcranial surgery (group two). 25 patients presented a microadenoma (transsphenoidal surgery, group three). Endocrine studies included basal serum levels and dynamic testing of anterior pituitary partial function. Clinical symptoms and hormone replacement therapy were documented. RESULTS: Preoperatively, in group one, two and three, somatotropic function was impaired in 85, 90 and 80 %, gonadotropic in 61, 57 and 24 %, corticotropic in 31, 38 and 28 %, thyreotropic in 32, 38 and 12 % and lactotropic in 22, 38 and 32 % cases, respectively. Pituitary functions did not improve significantly after transsphenoidal or transcranial surgery. Presurgically, 63, 62 and 0 % patients complained about visual impairments, 60, 48 and 40 % about headache, 53, 24 and 36 % about fatigue and 28, 33 and 20 % about disturbance of cycle or potency. After transsphenoidal surgery, impaired vision, headache and fatigue improved within 3 months; after transcranial surgery, only headache improved. Preoperatively, pituitary malfunctions were treated adequately. Postsurgically, more patients received adrenal and thyroid hormone substitution, less patients received sex hormones than examinations proved necessary. CONCLUSION: Anterior pituitary function of NF adenoma patients did not improve significantly after transsphenoidal or transcranial surgery. After transsphenoidal surgery, most clinical symptoms normalised within 3 months. In some of the patients, substitution was not optimally adjusted to hormonal impairments.