Osteitis fibrosa cystica, coeliac disease and Turner syndrome. A case report.

Today, osteitis fibrosa cystica is seldom present in primary hyperparathyroidism while it is mainly observed in uraemic osteodystrophy. We describe the case of a 54-year-old woman who was found to have huge bone cysts due to osteitis fibrosa cystica in the long bones. A parathyroid adenoma was identified and removed. Coeliac disease and Turner syndrome were diagnosed. Metabolic bone disease due to secondary hyperparathyroidism is common in coeliac disease; however, osteitis fibrosa cystica has not yet been described.

Recurrent Salmonella sepsis and aortitis in a patient with hepatocellular carcinoma.

A 62 years old man was admitted to hospital because of fever; a small superficial hepatic nodule was showed by ultrasonography. Blood cultures grew Salmonella enteritidis. Cefotaxime was administered for ten days. Fever promptly disappeared but one week later recurred with abdominal and back pain. Cultures grew again Salmonella enteritidis. Biopsy of the hepatic nodule showed hepatocarcinoma. Computed abdominal tomography showed a paraaortic mass. Angiography demonstrated hematoma communicating with the aortic lumen. The patient underwent revascularization of the involved aortic tract and resection of the hepatic nodule. Histology showed suppurative aortic endarteritis and a well-differentiated hepatocellular carcinoma with a large area of suppurative necrosis. The recovery of Salmonella species as of any uncommon bacteria from blood should warrant a through research of underlying disease, especially cancer.

Ultrasound guided percutaneous fine-needle biopsy in a case of eosinophilic gastroenteritis.

Eosinophilic gastroenteritis is a rare disease; clinical features depend on which intestinal layer is involved. In our report a 70-year-old woman presented with intestinal subocclusion and ascites. Endoscopic biopsies of gastric mucosa were negative. Ultrasound guided percutaneous fine-needle biopsy showed muscle infiltration by eosinophils of muscle layer of the stomach and jejunum. Muscular and serosal disease are usually diagnosed only by laparotomy or laparoscopy.

Fish oil supplementation in patients with heterozygous familial hypercholesterolemia.

Familial hypercholesterolemia is associated with premature coronary heart disease. In patients with familial hypercholesterolemia, monotherapy with hydroxymethylglutaril coenzyme. A reductase inhibitors rarely achieves the goal of desirable low-density lipoprotein levels. Epidemiological studies suggest that populations with a high dietary intake of marine n3 fatty acids are protected against coronary heart disease. Hepatic synthesis and secretion of very low density lipoproteins are reduced during fish oil supplementation while other effects on lipid and lipoprotein metabolism are controversial. Fourteen patients affected by familial heterozygous hypercholesterolemia on chronic treatment with simvastatin were enrolled in a double blind, placebo controlled, randomized crossover trial that evaluated the effect of fish oil ethyl ester (Esapent, 5.1 g/day) on lipid and lipoprotein serum concentrations. Total cholesterol, low density lipoprotein cholesterol, high density lipoprotein cholesterol, triglycerides, apoprotein B, apoprotein AI, lipoprotein (a) did not show any significant variation during the four week treatment period with fish oil ethyl ester. The present data suggest that the possible favourable influence of fish oil on the progression of atherosclerosis in these high-risk patients might involve mechanisms which are different from lipid metabolism.

Pravastatin-associated myopathy. Report of a case.

A case of acute inflammatory myopathy associated with the use of pravastatin, a new hydrophilic 3-hydroxy-3 methylglutaril coenzyme A reductase inhibitor, is reported. The patient, a 69-year-old man was affected by non-insulin-dependent diabetes mellitus and hypertension. He assumed pravastatin (20 mg/day) because of hypercholesterolemia. He was admitted with acute myopathy of the lower limbs which resolved in a few days after pravastatin discontinuation. A previously unknown hypothyroidism, probably due to chronic autoimmune thyroiditis, was evidenced. Muscle biopsy (left gastrocnemius) revealed a perimysial and endomysial inflammatory infiltrate with a prevalence of CD4+ lymphocytes. While lovastatin and simvastatin have been associated with toxic myopathy, pravastatin-associated myopathy could represent a distinct, inflammatory entity.

Effects of a gluten-free diet on serum lipids and lipoprotein (a) levels in a group of patients with celiac disease.

The influence of nutrient absorption, caloric content, and diet on lipoprotein (a) [Lp(a)] concentration is uncertain. To our knowledge, there are no reports on Lp(a) behavior in malabsorption. Serum lipids and Lp(a) concentrations were evaluated in 17 celiac patients (5 male and 12 female patients; age range, 1-24 years) when the diagnosis was established and after a 3-month gluten-free diet. Mean total and low-density lipoprotein cholesterol did not show significant change, while mean high-density lipoprotein cholesterol rose and triglycerides decreased significantly after the diet. The Lp(a) concentration remained unchanged in all patients (median values, 35 mg/L before and 40 mg/L after the diet). Our results suggest that, in our patients, the lipoprotein profile was influenced by the gluten-free diet, while the Lp(a) concentration was not modified.

Cardiac involvement in Churg-Strauss syndrome: a follow-up of three cases.

Three cases of Churg-Strauss syndrome with myocardial involvement are reported. Cardiac disease is known to be the major cause of death in Churg-Strauss syndrome. Aggressive therapy (steroids and cyclophosphamide) may cure the myocardial dysfunction associated with the disease.

Lipoprotein (a) in thyroid dysfunction before and after treatment.

Alterations of the lipid profile are a well known phenomenon in thyroid dysfunction. Thyroid hormones regulate lipid metabolism through various mechanisms, but a key role is played by the LDL receptor pathway. Thyroid hormone influence on Lipoprotein (a) (Lp[a]) metabolism is unknown; therefore we studied Lp(a) concentrations in a group of 29 hypothyroid patients with post-surgical hypothyroidism and in a group of 14 hyperthyroid subjects with Graves' disease before and after the thyroid function was normalized by treatment. In hypothyroid patients total and LDL-cholesterol markedly decreased after T4 treatment (342 +/- 78 mg/dl before and 193 +/- 46 mg/dl after; 225 +/- 72 mg/dl before, 111 +/- 43 mg/dl after respectively, p < 0.001). Also HDL-cholesterol and triglycerides decreased (from 75 +/- 22 mg/dl to 56 +/- 18 mg/dl and from 182 +/- 87 mg/dl to 112 +/- 42 mg/dl respectively, p < 0.001). Lp(a) showed minor but not significant variations (median values 80 mg/l before 55 mg/l after treatment, p: N.S.). In hyperthyroid patients total and LDL-cholesterol increased after methimazole treatment (from 148 +/- 49 mg/dl before to 254 +/- 67 mg/dl after and from 87 +/- 38 mg/dl before to 178 +/- 51 mg/dl after, p < 0.001). HDL-cholesterol increased (from 39 +/- 9 to 50 +/- 15, p < 0.01) while triglycerides were unchanged. Lp(a) levels slightly rose (median values 57 mg/l before 84 mg/l after treatment, p < 0.05). These data suggest that the influence of thyroid hormones on Lp(a) metabolism is of minor entity and probably does not operate through the LDL receptor pathway.

Arginine normalizes the growth hormone (GH) response to GH-releasing hormone in adult patients receiving chronic daily immunosuppressive glucocorticoid therapy.

Glucocorticoids are thought to inhibit GH secretion through an enhancement of endogenous somatostatin tone. The aim of our study was to evaluate the effect of arginine, a secretagogue that increases GH secretion acting at the hypothalamic level, probably by decreasing somatostatin tone, on GH-releasing hormone (GHRH)-induced GH secretion in three male and five female adult patients with nonendocrine disease who were receiving daily immunosuppressive glucocorticoid therapy. Six normal subjects (four males and two females) served as controls. GHRH-induced GH secretion was evaluated after 30-min iv infusion of saline (100 mL) or arginine (30 g) in 100 mL saline. After saline administration, steroid-treated patients showed a blunted GH response to GHRH (GH peak, 8.7 +/- 2.4 micrograms/L) compared to that of normal subjects (GH peak, 23.8 +/- 3.9 micrograms/L). The GH responses to GHRH increased (P less than 0.05) after pretreatment with arginine compared to saline pretreatment in both normal subjects (GH peak, 36.6 +/- 4.0 micrograms/L) and steroid-treated patients (GH peak, 28.4 +/- 5.5 micrograms/L). The GH responses to GHRH plus arginine were not significantly different in steroid-treated and normal subjects. Thus, arginine is able to normalize the GH response to GHRH in patients receiving chronic glucocorticoid treatment. Our data are evidence that the stimulatory action of arginine and the inhibitory action of glucocorticoids on GH secretion are mediated by opposite effects on hypothalamic somatostatin tone.

Reversible myocardial impairment in the Churg-Strauss syndrome: report of a case.

Heart disease is a major cause of morbidity and mortality in the Churg-Strauss syndrome. However, few clinical follow-ups have been published. In this case report, in which the diagnosis of Churg-Strauss syndrome was made based on histologic criteria and clinical features, heart failure with globally depressed left ventricular function was present. Aggressive therapy (prednisone and cyclophosphamide) was instituted. In the follow-up a prompt clinical response and eventual recovery of the indices of cardiac function as evaluated by echocardiography and radionuclide ventriculography were observed.

Septic polyarthritis in chronic tophaceous gout.

This paper reports the case of a 48 year old patient with chronic tophaceous gout who was admitted to the hospital with a pneumococcal polyarthritis affecting the same joints previously involved by gout attacks. The authors emphasize that the possibility of a septic arthritis should always be considered in the gouty patient, and not only in the elderly, as stressed in the literature, but also in younger patients.

Liver enzyme abnormalities in subacute thyroiditis.

In a retrospective study of liver function tests in subacute thyroiditis, 13 out of 22 patients with the disease demonstrated an elevation of alkaline phosphatase level. Less prevalent abnormalities were elevations in gamma-glutamyl transpeptidase (5 pts), alanine (4 pts) and aspartate (3 pts) aminotransferase activities. These enzyme alterations were not correlated with thyroid hormone levels, concomitant diseases, drugs or alcoholic intake, and normalized in six patients followed until recovery. A benign, short-lived and subclinical hepatic involvement is common in subacute thyroiditis.

[Skin involvement in Rosai-Dorfman disease. Description of a case]. / Interessamento cutaneo nella malattia di Rosai-Dorfman. Descrizione di un caso.

Sinus Histiocytosis with massive lymphadenopathy is an idiopathic, histologically distinctive disorder that characteristically produces painless cervical lymphadenopathy. Other node groups are frequently involved and mass lesions in extranodal sites occur in approximately 30% of patients. In most cases the disease is chronic and the course is often characterized by exacerbations and remission. We report the case of a 66 years old white woman affected by sinus histiocytosis with massive cervical and inguinal lymphadenopathy. Subcutaneous nodules appeared in her left arm and low back region with the histological features of sinus histiocytosis. Cellular infiltrate was composed predominantly of histiocytes aggregating in granuloma like clusters. These cells had large round or kidney shaped nuclei; cytoplasm was abundant, vacuolated, often containing phagocytized lymphocytes. Multinucleated cells were also observed; reactive plasmocytosis was present in the nodule periphery. During the follow-up the patient maintained good physical conditions, despite a moderate weight loss. The size of her nodules regressed within one year. Immunologic alterations, consisting of polyclonal hypergammaglobulinemia and persistent inversion of T4/T8 lymphocyte ratio, were observed. Sinus histiocytosis usually offers a good prognosis; in our patient infective and/or autoimmune complications could be favoured by advanced age, extranodal involvement and immunological depression.

Severe microvascular disease in type II diabetes of early onset. A family study.

Both early onset and late onset type II diabetes were present in one family of nine siblings. The three early onset type II diabetic siblings showed severe microvascular complications: proliferative retinopathy, diabetic nephropathy, and peripheral neuropathy. Early onset type II diabetes was not associated with any particular HLA haplotype. Early onset type II diabetes could be considered a clinical and genetic disease entity different from MODY type diabetes.