[Peripartum cardiomyopathy, acute autoimmune pancreatitis and periadipose tissue inflammation - autoimmune reaction?]. / Kardiomiopatia pologowa a ostre zapalenie trzustki i tkanki tluszczowej - reakcja autoimmunologiczna?

A case of 26 year-old female with peripartum cardiomyopathy, acute pancreatitis, periadipose tissue inflammation due to unknown cause and multiple organ dysfunction syndrome complication is presented.

[Difficulties in the diagnosis of painful Hashimoto's thyroiditis--case report]. / Trudnosci w rozpoznawaniu postaci bólowej przewleklego zapalenia tarczycy--opis przypadku.

Painful Hashimoto's thyroiditis is an atypical variant of Hashimoto's thyroiditis characterised by thyroid pain and fever. We report the case of a 40-year-old female whose clinical manifestation so closely mimicked subacute thyroiditis that she was initially diagnosed as having de Quervain's disease. Only finding a lymphocytic infiltration in fine needle biopsy specimens of the thyroid gland and positive antithyroid antibodies allowed us to correctly interpret the clinical manifestation of our patient leading to the diagnosis of painful Hashimoto's thyroiditis. No beneficial effect was produced by nonsteroidal antiinflammatory drugs while some improvement was observed after treatment with propranolol. The most effective drug was thiamazole that not only normalised thyroid hormone levels but also caused disappearance of fever and markedly reduced pain intensity. The discussed case shows difficulties in diagnosis of inflammatory processes in the thyroid gland and indicates that antithyroid drugs may be effective agents in hyperthyroid patients with painful Hashimoto's thyroiditis.

[Atypical clinical presentation of autoimmune polyglandular syndrome type 4]. / Nietypowa manifestacja kliniczna autoimmunologicznego zespolu wielogruczolowego typu 4.

Autoimmune polyglandular syndromes (APS) are rarely diagnosed conditions characterised by the combination of two or more autoimmune endocrinopathies and nonendocrine autoimmunopathies. They comprise a wide spectrum of autoimmune disorders, differing in the immunologic features of their pathogenesis. Based on their clinical manifestation, APS are divided into four different types. Primary hypoparathyroidism is characteristic for APS type 1, the major disease components of which are adrenal insufficiency, hypoparathyroidism, and candidiasis. However, the literature is sparse regarding the presence of hypoparathyroidism in the remaining types of APS. In our article, we present a case of a young female with primary hypoparathyroidism and a family history of autoimmune disorders who after several years developed type 1 diabetes. She also had anti-transglutaminase and anti-parietal cell antibodies. This constellation of two endocrine disorders and non-endocrine abnormalities led to the diagnosis of APS type 4. We show in details diagnostic and treatment strategies undertaken in our patient and their impact on the course of APS.

[Primary aldosteronism in a patient after surgical treatment of primary hyperparathyroidism]. / Hiperaldosteronizm pierwotny u chorego z przebyta nadczynnoscia przytarczyc.

The presence of both parathyroid adenoma and primary aldosteronism in the same person has been reported in only few patients. Taking into account a relatively high prevalence of both primary hyperparathyroidism and primary aldosteronism in the general population, these findings may be explained by a simple coincidence of both these disorders. In the present paper, we report a man with a family history of multiple endocrine neoplasia type 1 (MEN-1), who developed primary aldosteronism eight years after removal of parathyroid adenoma. We report in details diagnostic and treatment strategies applied in our patient and their impact on the course and outcome of both these disorders. In our opinion, the described case of our patient represents an atypical presentation of MEN-1 syndrome, a hereditary syndrome characterized by a variety of endocrine neoplasias and hormone excess syndromes, particularly tumors of the parathyroid glands, pancreatic islet cells, and pituitary gland. We conclude that each person with MEN-1 syndrome and the presence of adrenal lesions should be assessed for the presence of excess mineralocorticoid activity.

Hypoparathyroidism in pregnancy.

Pregnancy and lactation are associated with remarkable changes in calcium homeostasis, resulting from pregnancy-induced alterations in the production, metabolism and excretion of calcium and calcitropic hormones. Therefore, the clinical course of disorders affecting the parathyroid glands during pregnancy and lactation is often atypical. As the symptoms experienced by patients with parathyroid disorders are not specific, their diagnosis during gestation and breastfeeding may be sometimes very difficult. If left untreated, parathyroid disorders may pose a significant risk to the mother and fetus associated with increased perinatal and maternal morbidity and mortality. Hypoparathyroidism is a disorder rarely observed during pregnancy, resulting in most cases from surgical thyroidectomy. In our article, we present the case of a 32-year-old female with primary hypoparathyroidism diagnosed for the first time during pregnancy. We describe in details the treatment strategies undertaken in this patient and their impact on the outcome of pregnancy and lactation.

[Right atrial tumour and right ventricular failure - diagnostic role of echocardiography and cardiovascular magnetic resonance]. / Guz prawego przedsionka i ciezka niewydolnosc prawej komory w badaniu echokardiograficznym i rezonansie magnetycznym serca.

A case of a 66 year-old female with advanced right ventricular failure is described. Echocardiography and MRI revealed the presence of right atrial tumour. The patient underwent successful surgery and histological examination revealed lymphoma.

Recurrent pregnancy-induced diabetes insipidus in a woman with hemochromatosis.

Diabetes insipidus is a rare disorder in pregnant women, predating pregnancy or appearing for the first time during gestation. In pregnancy it usually affects women with HELLP syndrome or acute fatty liver of pregnancy and results from the reduced hepatic degradation of placental vasopressinase leading to its increased activity. Although infiltrative diseases have been found to cause diabetes insipidus in non-pregnant population, very few studies showed that these disorders may manifest for the first time during gestation. We describe here the case of transient diabetes insipidus in two subsequent pregnancies of a female with hemochromatosis. The first symptoms of this disease appeared for the first time at the beginning of the third trimester of her second pregnancy, and diagnosis was established on the basis of typical clinical presentation, confirmed by a water deprivation test. Diabetes insipidus resulted from the increased activity of vasopressinase, caused by hemochromatosis-induced liver dysfunction, the presence of which was confirmed between the pregnancies by liver biopsy and identification of the HFE gene mutation. Subsequent desferrioxamine treatment resulted in a less severe clinical course of diabetes insipidus in the last patient's pregnancy. In both pregnancies, the patient was successfully treated with oral desmopressin, which is resistant to degradation by placental vasopressinase. Although unrecognized pituitary disorders may pose a serious health problem to the mother and fetus, hemochromatosis-induced diabetes insipidus, as the case of our patient demonstrates, if effectively diagnosed and treated, cannot be regarded as a contraindication for pregnancy.

[Pituitary disorders in pregnancy and after delivery]. / Choroby przysadki w ciazy i po porodzie.

Pregnancy is associated with a number of changes in endocrine physiology that substantially alter hormone levels and affect a number of disease processes. Consequently, both pregnancy and the postpartum period may influence the course of pre-existing pituitary disorders and lead to the development of those that are found exclusively or almost exclusively during these periods. Overlapping clinical and biochemical features of pregnancy sometimes cause that the diagnosis of pituitary disorders during pregnancy may be missed. Moreover, their clinical picture may range from non-specific symptoms to even life-threatening conditions, and the manifestation of the same pituitary disease often change from one patient to another. If not evaluated and managed appropriately, these disorders may negatively affect both mother and fetus. The purpose of this review is to provide an update on the pathophysiology, clinical picture, diagnosis and treatment of different pituitary disorders during pregnancy and after delivery. The reader is also provided with some practical recommendations on dealing with pregnant and nursing women suffering from these disorders.

[Rare disorders of glucocorticoid production, metabolism and action]. / Rzadkie schorzenia wydzielania, metabolizmu i dzialania receptorowego glukokortykoidów.

Glucocorticoids belong to the most important human hormones which are involved in the regulation of a variety of cardiovascular, metabolic and immunologic functions. Because they are necessary to sustain life and maintain homeostasis - adrenal disorders, if not detected in a timely fashion, can have serious consequences. The significant progress in the fields of hormonal assessment, imaging procedures and molecular biology made in recent years has brought about great improvement in the identification, differentiation and treatment of various adrenal disorders and has enabled the description of some new clinical entities. Abnormally high or abnormally low tissue glucocorticoid levels may either coexist with or result in deficiency or excess of mineralocorticoids and/or adrenal androgens. This review summarises the present state of knowledge on the pathogenesis, clinical picture, diagnosis and management of the selected rare disorders of glucocorticoid production, metabolism and their receptor action.

Left internal mammary artery improves 5-year survival in patients under 40 subjected to surgical revascularization.

BACKGROUND: The population of young patients under 40 requiring coronary bypass surgery is characterized by an extremely and unusually rapid progression of coronary heart disease. The aim of the present study was to assess the clinical status and quality of life in these patients after surgery in relation to the type of conduit used to revascularize the left anterior descending artery (LAD). METHODS: One hundred seventeen patients under 40 (range, 30-40 years) underwent coronary artery bypass grafting (CABG) at our institution between 1991 and 1999. Ninety-one patients received LIMA to LAD graft (group A), and in 26 patients the saphenous vein was used to graft this vessel (group B). Seventy-eight patients (63 in group A and 15 in group B) were assessed after a mean time of 71 +/- 26 months. They were asked to fill out a questionnaire aimed at their subjective assessment of their quality of life as compared with their preoperative status. RESULTS: Five-year actuarial survival was higher in patients with LIMA to LAD graft (log rank test: P < .004). The functional status of patients in group B was significantly worse in comparison to group A: respectively, CCS 2.2 +/- 1.1 versus 1.5 +/- 0.7; (P = .02), NYHA 2.2 +/- 1.1 versus 1.3 +/- 0.5; (P = .002). Patients in group B more frequently required reinstitution of nitroglycerine treatment (93% versus 56%; P = .025). We failed to show differences between the 2 groups as far as subjective quality of life is concerned. In summary, 63% of patients perceived it to be worse, 29% to be better, and 8% felt it had not changed. CONCLUSION: The use of LIMA is crucial in patients undergoing CABG under the age of 40 in order to achieve the best possible surgical results. Quicker recurrence of coronary disease symptoms is observed when a vein is used to graft the LAD. It may reflect an earlier progress of atherosclerosis in venous grafts.

Serum tumour necrosis factor-alpha, interleukin-2 and interleukin-10 activation in stable angina and acute coronary syndromes.

BACKGROUND: Dynamic instability of coronary atherosclerotic plaque results in the development of both unstable angina and myocardial infarction. The aim of the study was to investigate the dynamics of serum concentrations of tumour necrosis factor (TNF)alpha, interleukin (IL)-10, and IL-2 in patients with myocardial infarction (MI) and unstable angina (UA) as compared to stable angina (SA) patients and healthy volunteers. METHODS: A total of 189 patients with coronary artery disease (CAD) were studied: 100 patients with SA (class II/III according to CCS), 57 patients with UA (Braunwald class IIIB; determinations at 6, 24, and 48 h after chest pain), and 32 patients with MI (determinations at admission, on the 7th and 30th days after MI). Twenty healthy volunteers acted as controls. RESULTS: Serum TNFalpha levels were elevated in all CAD groups (SA: 17.3+/-4; UA: 18.7+/-4; MI: 22.0+/-3 pg/ml; p<0.001) in comparison to the controls (8.3+/-1.4 pg/ml). However, the highest values were characteristic of MI patients, especially values obtained at admission (p<0.01 versus SA and UA). Mean serum concentrations of IL-2 were significantly higher in patients with MI and UA (89.6+/-40; 87.0+/-24 pg/ml, respectively; p<0.01) when compared to SA and the control group (58.3+/-49; and 51.5+/-39, respectively). Serum IL-10 levels were also higher in MI and UA patients. Levels of IL-2 and IL-10 measured following chest pain in unstable patients, as well as their consecutive determinations in MI patients did not show any change dynamics, that is, they were persistently elevated. CONCLUSIONS: When compared to stable CAD and healthy subjects, acute coronary syndromes are associated with long-term increase of serum concentrations of pro- and anti-inflammatory cytokines. It seems likely that sudden CAD progression leading to acute coronary syndromes is triggered/accompanied by prolonged immune activation.