An unusual presentation of familial Mediterranean fever with prolonged hip pain and amyloidosis.

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limiting attacks of joint, chest and abdominal associated with fever. We present an unusual case of FMF with prolonged arthritis and amyloidosis. Familial Mediterranean fever should be considered in the differential diagnosis of prolonged hip pain, even in the absence of symptoms or signs of FMF.

Pellagra: a sporadic pediatric case with a full triad of symptoms.

Pellagra is clinically manifested by a photosensitive dermatitis, diarrhea, and dementia. The full triad of symptoms is usually not well developed in infants and children. We report a case of a 14-year-old boy with classic symptoms of pellagra. All his symptoms responded to treatment with nicotinic acid.

Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF?

BACKGROUND: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. OBJECTIVES: To determine the special characteristics and the prognosis of PAN in FMF patients. METHODS: A questionnaire was used for the present survey. The setting was 7 referral centers from Turkey and Israel. Seventeen patients who were diagnosed with FMF and who developed PAN were included. PAN was diagnosed in those who met the Chapel Hill consensus criteria for microscopic polyarteritis or classic PAN. The clinical features of these 17 patients and the outcomes of their vasculitis were analyzed. RESULTS: The age at diagnosis of PAN in these FMF patients ranged from 3.5 to 37 years. All patients had constitutional symptoms, elevated acute phase reactants, and myalgia at the time PAN was diagnosed. The diagnosis of PAN was confirmed by renal angiography in 8 patients, by renal biopsy in 6 patients, and by muscle and/or nodule biopsies in 6 patients. A number of patients had definite features of both classic PAN and microscopic polyarteritis. CONCLUSIONS: When compared with other PAN patients, those with FMF tended to have a younger age at PAN onset, more frequent perirenal hematomas, and an overall better prognosis. The cases with overlapping features of microscopic and classic PAN pose a problem for the current classification of vasculitis. We suggest that the clinical representation of PAN in FMF patients has certain characteristics and may be a feature of FMF per se.

Giant adrenal myelolipoma: case report and review of the literature.

Myelolipoma is a tumor-like growth composed of mature fat tissue and bone marrow elements. It occurs in the adrenal gland or as an isolated soft tissue mass. It may be associated with endocrine disorders such as hermaphroditism, Cushing's disease, Addison's disease and obesity of unknown cause. These lesions rarely measure more than 5 cm in diameter, although giant tumors have been reported in the literature. The fifth largest surgically resected adrenal myelolipoma in the literature is reported and its clinical associations and, macroscopic and microscopic features are discussed.

Effect of vitamin E and pentoxifylline on glycerol-induced acute renal failure.

The pathogenesis of acute renal failure may involve, among other causes, ischemia, vascular congestion, arachidonic acid pathways, and reactive oxygen metabolites. The aim of this study is to evaluate the effects of pentoxifylline and vitamin E on the prevention of experimental acute renal failure induced by glycerol. Eighty-five Sprague-Dawley rats weighing 170-230 g were included in the study. The rats were randomly divided into four groups: group 1 was given 1 ml saline; group 2, glycerol; group 3, glycerol plus vitamin E, and group 4, glycerol plus pentoxifylline. Extent of histological renal tubular necrosis and regeneration in each animal were graded. Blood urea nitrogen, serum creatinine, and creatine kinase concentrations were measured. Mean blood urea nitrogen and serum creatinine concentrations and tubular injury scores were significantly lower in group 1 than in groups 2-4 (p < 0.001), but there were no significant differences among groups 2-4. We conclude that postinsult administration of vitamin E and pentoxifylline does not have a beneficial effect on prevention and severity of acute renal failure and that controlled, multicenter studies involving a large number of patients are needed to clarify this subject.

Behcet's disease and AA-type amyloidosis.

Behcet's disease (BD) is a multisystem disorder characterized by vasculitis. To our knowledge, 52 patients with BD and amyloidosis have previously been described in the literature. Nephrotic syndrome was the most common type of presentation of amyloidosis in these patients. The prognosis of patients with BD and amyloidosis has not been reported before. In this report, we present a patient with BD and AA-type amyloidosis and analyze the prognosis in these patients. Follow-up and prognosis have been reported in 23 patients. Ten of these 23 patients died and most of these deaths occurred within 3 months after the diagnosis of amyloidosis. End-stage renal disease developed shortly in 4 patients. BD should be considered in the differential diagnosis of AA amyloidosis. In conclusion, the cases with BD and amyloidosis carry poor prognosis.

Effects of Seprafilm (sodium hyaluranate-based bioresorbable), Sepracoat (0.4% hyaluronic acid), and Ringer's lactate on the prevention of postsurgical adhesion formation in rat models.

Forty adult female rats were randomly divided into four groups. Before the standard surgical procedure which consisted of creating a lesion with a lancet over the right uterine horn. Group I received no medication. Ringer's lactate (RL) solution was applied intraperitoneally to Group II at the end of the surgery. Sepracoat solution was applied intraperitoneally to Group III at the onset of the surgery. Group IV randomly received Seprafilm wrapped over the entire right uterine horn. Re-laparotomy was performed 3 weeks later to evaluate the adhesions. The mean adhesion scores were (mean+/-SD) 2.90+/-0.18, 1.00+/-0.36, 0.50+/-0.17 and 0.40+/-0.16 in Groups I, II, III, IV, respectively. The postsurgical adhesions were significantly less in the groups treated with Seprafilm and Sepracoat.

Hepatitis C in haemodialysis and nonuraemic patients: a histopathological study.

Hepatitis C virus (HCV) is the major cause of posttransfusion non-A, non-B hepatitis. Haemodialysis patients carry the risk of HCV infection. The aim of this study is to compare the morphological changes related to chronic HCV infection found in haemodialysis and nonuraemic patients. Liver biopsies from nine haemodialysis patients and 37 patients with normal renal function were studied. This study shows that haemodialysis patients may have less active and progressive chronic hepatitis C than patients with normal renal function. The number of patients in this study is limited, therefore further studies are needed for definite conclusion.

Cathepsin B, p53 expression and AgNORs in transitional cell carcinoma.

The aim of this study was to assess the relation between silver-strained nucleolar organizer regions (AgNOR), tumor stage, tumor grade and p53 expression with cathepsin B staining in transitional cell carcinoma of bladder. Tissue sections from 64 transitional cell carcinomas of the bladder were evaluated for the relation between AgNOR, tumor stage, tumor grade and p53 expression with cathepsin B staining in the neoplastic and stromal cells. Mean AgNOR values were significantly higher and the presence of p53 expression were different in cathepsin B positive and negative tumor and stromal cells. Although the number of cases is limited, this pilot study shows that cathepsin B staining may have a prognostic value in transitional cell carcinoma, but more studies are needed for a definite conclusion.

Primary hemangiopericytoma of bone located in the tibia.

Primary hemangiopericytoma of the bone is a rare tumor. We describe a patient with primary hemangiopericytoma of the tibia treated by surgery. The clinical and radiological features of this tumor are described. The radiological and histopathological different diagnosis of hemangiopericytoma is discussed.

Behçet's disease and amyloidosis. Review of the literature.

In this report, we present a patient with BD, amyloidosis and end-stage-renal-disease and discuss the literature on BD and amyloidosis. Forty-four patients with BD and amyloidosis are described in the literature. The type of amyloid fibrils were studied in 27 of this 44 patients and all were AA. The interval between the onset of first symptom of BD and the diagnosis of amyloidosis ranged from one to 27 years. This clinical observation and AA type amyloid fibrils in BD suggest that amyloidosis is secondary to inflammation. Behçet's disease should be considered in the differential diagnosis of AA amyloidosis.

Histopathological characteristics of chronic hepatitis C virus infection in Turkey.

Hepatitis C virus (HCV) is the major cause of posttransfusion non-A, non-B hepatitis. The aim of this study is to describe the morphological changes related to HCV infection found in Turkey and compare the similarities and differences with the previous reports. Liver biopsies from 44 patients with HCV infection were studied. Chronic active hepatitis was the most common (56.8%) histopathological lesion. Most of the cases (n = 15, 34.1%) with chronic active hepatitis showed mild activity and mean HAI was 5.5 in these cases. The presence of sinusoidal lymphocytes (79.5%) and Kupffer cell proliferation (68.2%) were the most common histological features. We could not find any publication about histopathological characteristics of HCV infection from Turkey in MEDLINE database. This study shows that histopathological changes seen in patients with chronic HCV infection in Turkey is comparable with the literature, but more histopathological studies including genotyping of HCV and objective criteria to evaluate histopathological changes of chronic HCV infection are needed for definite conclusion.