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S Arslan

Publications and source records attributed to S Arslan.

At least 55 records · Page 3Linked to original sources

Familial Mediterranean fever in children: report of a large series and discussion of the risk and prognostic factors of amyloidosis.

UNLABELLED: Familial Mediterranean fever (FMF) is a genetically transmitted disease characterized by recurrent attacks of fever and serositis. The most important complication of this disease is the development of amyloidosis. We present our analysis of 425 FMF patients without and 180 with amyloidosis (123 FMF having amyloidosis type 1 and 57 FMF having amyloidosis type II). The male female ratio was higher in the amyloidosis population (111/69) when compared to the FMF population (225 200) (P = 0.048). Consanguinity rate was the same among FMF and amyloidosis groups. However, a family history of amyloidosis was significantly more frequent in the amyloidosis group (P = 0.00001). Multivariate analysis has revealed that in FMF patients, the presence of a family history of amyloidosis plus consanguinity has a 6.04 fold increased risk of amyloidosis (P < 0.0001). The 5-year chronic renal failure free survival was 43.1% and 18.7% in type I and type II amyloidosis, respectively. The time interval to develop chronic renal failure after the development of amyloidosis was 4.8 in type I and 3.0 years in type II. respectively. We found ten cases of Henoch-Schönlein Purpura and nine of polyarteritis nodosa among our patients. The significance of the association between FMF and vasculitis awaits to be clarified. Among the FMF patients put on colchicine therapy (435), only 10 (2.3%) have developed amyloidosis confirming that this drug protects from amyloidosis. CONCLUSION: Since the presence of a familial history of amyloidosis has been defined as the most important risk factor in the development of amyloidosis, we suggest that additional genetic factors may be operative in the development of amyloidosis.

Amyloidosis↗

Case of the month.

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Age of Onset↗

The effect of intravascular transfusion for severely anemic fetuses on umbilical artery Doppler flow velocity waveforms.

BACKGROUND: To evaluate whether the doppler flow velocity waveforms reflect the fetal hematocrit and to assess the changes of the umbilical artery doppler indices before and after the puncture of the umbilical cord. MATERIAL AND METHODS: In this prospective-study, Pulsed-Doppler examinations of blood flow velocities in the umbilical artery were carried out before and after 22 diagnostic cordocentesis and 14 fetal blood transfusions into the umbilical vein. RESULTS: There were decreases in the systolic/diastolic ratio (p < 0.001), the pulsatility index (PI)(p < 0.001), and the resistance index (RI)(p < 0.003) after cordocentesis but not after fetal blood transfusion. There were no correlations between the initial hematocrit and the umbilical artery doppler indices in both fetal blood transfusion group and fetal blood sampling group. There was no correlation between the increase in hematocrit and changes in umbilical artery blood-velocity waveform indices following fetal blood transfusion. The umbilical artery doppler flow-velocity indices did not predict the fetal hematocrit. CONCLUSION: These findings suggest that acute changes in blood viscosity following intravascular transfusion are not associated with an increase in resistance to flow as assessed by doppler velocimetry. Umbilical blood sampling per se may be associated with a humorally mediated reduction in placental vascular resistance to flow.

Blood Transfusion, Intrauterine↗

Bacteriology of antrum in children with chronic maxillary sinusitis.

Swabs from 93 chronically inflamed maxillary sinuses in children were taken from aerobic and anaerobic bacteria during endoscopy. Bacterial growth was present in 87/93 specimens (93%). Anaerobic bacteria were isolated in 81/87 culture-positive specimens (93%) and were recovered alone in 61 cases (70%) and mixed with aerobic or faculative bacteria in 20 (23%). Aerobic or facultative bacteria were present alone in six cases (7%). A total of 261 isolates (3/specimen), 19 (2.4/specimen) anaerobes and 69 (2.6/specimen) aerobes or facultatives, were isolated. The predominant anaerobic organisms were Bacteroides sp. and anaerobic cocci; the predominant aerobes or facultatives were Streptococcus sp. and Staphylococcus aureus. These findings indicate the important role of anaerobic organisms in chronic sinusitis.

Adolescent↗

Polycystic ovaries in association with müllerian anomalies.

The polycystic ovary syndrome, whose etiopathogenesis is not clearly understood, has a wide spectrum of clinical presentations, and may co-exist with other pathologic conditions. In this study, we evaluated the prevalence of ultrasound-defined polycystic ovaries (PCO) in patients with müllerian anomalies (n = 167), and those without müllerian anomalies (n = 3165) from 1990 to 1994, in a population markedly composed of infertility patients. PCO were found in 50 (29.9%) patients in the study group, compared to 637 (20.1%) patients in controls (P < 0.01). Müllerian anomalies were further grouped according to the American Fertility Society (AFS) classification and it was found that patients with the septate uteri and bicornuate uteri malformations had a higher prevalence of PCO than the controls (P < 0.001, P < 0.05, respectively). Although a difference existed in the percentage of PCO in patients with unicornuate uteri and didelphic uteri compared to controls, this difference did not reach statistical significance (P > 0.05). We conclude that, as PCO are more prevalent in certain müllerian anomalies, an embryogenetic defect may also be involved in the etiopathogenesis of PCO.

Female↗

The "pseudo-cholangiocarcinoma sign" in patients with cavernous transformation of the portal vein and its effect on the serum alkaline phosphatase and bilirubin levels.

OBJECTIVES: The goal of this study was to identify the underlying disorder responsible for portal venous thrombosis and cavernous transformation of the portal vein (CTPV). All patients with this finding underwent a thorough medical examination with intent to determine the cause and biochemical consequences of CTPV. METHODS: During an 8-yr period, a total of 1247 patients with clinical evidence of portal hypertension were examined using ultrasonography. Forty four of these 1247 patients were found to have CTPV. In each case, the finding of CTPV was confirmed by portography using either splenoportography or arterial portography, with digital subtraction angiography. These 44 patients were studied in an effort to determine the etiology of the cavernous transformation. In addition, the specific reason for the increased serum bilirubin and alkaline phosphatase levels in 35 of the 44 cases was evaluated by endoscopic retrograde cholangiopancreatography (ERCP) (34 patients), percutaneous transhepatic cholangiography (one patient), and by CT in 19. The surgical findings in 10 of these 44 patients, who ultimately underwent splenectomy and portal venous decompression for bleeding, were reviewed in light of the ultrasonographic, portographic, and ERCP findings in the same 10 patients. RESULTS: The underlying disorder responsible for cavernous transformation was found to be Behcet's disease in seven patients, chronic liver disease in four, congenital hepatic fibrosis in five, congenital protein C deficiency in one, and a prior abdominal operation for cholelithiasis in one patient. Despite a full clinical, radiological, hematological, and chemical evaluation, no etiology for CTPV was found in the remaining 26 patients. All of these later cases had no indication for liver biopsy or evidence for parenchymal liver disease. In these 26 patients, the serum levels of bilirubin and alkaline phosphatase ranged from mild to moderately increased compared with the moderately to markedly increased levels present in the 18 patients having an identifiable underlying liver disease. Irregular, undulating narrowing and nodular extrinsic defects, the so-called "pseudo-cholangiocarcinoma sign" was present in 33 of the 35 patients who underwent either ERCP or percutaneous transhepatic cholangiography. No such findings were observed in 10 control cirrhotic patients with portal hypertension but without CTPV, who also underwent ERCP. CONCLUSION: The results of this study indicate that mildly increased serum alkaline phosphatase and direct reacting bilirubin levels occur in cases with CTPV associated with a pseudo-cholangiocarcinoma sign. Presumably, these enzyme elevations are a result of compression of the biliary tree by the venous collaterals that run along the extrahepatic biliary tree. None of the 33 cases with this sign had cholangiolar carcinoma. Thus, when a patient with splenomegaly but without documentable parenchymal liver disease demonstrates an increase in the serum direct reacting bilirubin and alkaline phosphatase levels, CTPV and the presence of large extrahepatic venous collaterals partially obstructing the biliary tree should be suspected.

Adult↗

Compensatory mechanisms in rats with nasal obstructions.

This study, in rats was designed to demonstrate the changes in respiratory dynamics in compensation for nasal obstructions. The experiment was performed on 30 rats. Fifteen rats served as the study group while another 10 rats served as controls. Five of the 30 rats were operated on to find out whether they would live and if so, for how long. Mouth breathing caused acute respiratory acidosis and marked aerophagia, leading to spontaneous death of the five experimental animals 90 to 100 hours postoperatively. Stenosis of the oropharyngeal airway due to palatal-epiglottic approximation, is supposed to be responsible for the respiratory insufficiency in nasally-obstructed rats. The compensatory changes in respiratory mechanics caused by high oropharyngeal airway resistance, together with some possible reflex changes, may have caused air swallowing. It is supposed that the increased air volume in the stomach and guts, causing elevation of the diaphragm and a paralytic ileus, contributed to mortality. No signs of aspiration were observed in these animals. After the surgical obstruction of both nostrils, changes in acid-base balance occurred in the 15 study rats 24, 48, and 72 hours postoperatively. On the first, second, and third days, a decrease in pO2 and pH, an increase in pCO2, and a compensatory increase in serum bicarbonate was observed.

Animals↗

Effects of recombinant alpha interferon on chronic active hepatitis B: preliminary results.

This paper presents the preliminary results of a study designed to evaluate the effects of alpha interferon in chronic hepatitis B. After six months' treatment with interferon alfa-2b (5 million units (MU), three times weekly) 15 of 25 (60%) patients achieved seroconversion of hepatitis B e antigen, 17 (68%) normalised alanine aminotransferase (ALT) activity, and 15 (60%) showed a decrease in the inflammatory reaction on liver histology. No seroconversions occurred in the control group (n = 10), and none of the control patients achieved a normal ALT or showed a reduction in the inflammatory reaction. Adverse effects were experienced by most patients who received interferon but none warranted stopping the treatment.

Adult↗

Budd-Chiari syndrome: analysis of 30 cases.

The authors report their experience with 30 adult patients with Budd-Chiari syndrome (BCS), which is a rare and serious disorder, characterized by hepatic outflow obstruction caused by many different conditions. The diagnosis was based on the clinical data, ultrasonography (US), vena cavography and hepatic venography, computed tomography (CT), and liver biopsy. Behçet's disease (BD) was found in 10 patients with BCS as an underlying disease. Two patients used oral contraceptive drugs, 2 had liver tumor hepatocellular carcinoma and liver lymphoma, and 1 patient had chronic lymphocytic leukemia. Despite full investigation, the authors could not find any obvious underlying cause in the other 15 patients. The results suggest that (1) BCS must be considered as a possible complication in patients with Behçet's disease when they have hepatomegaly even if there were no cardinal manifestations of the disease at the time of admission, and BD is the most common etiologic factor in BCS (33%) in Turkey, where the incidence of Behçet's disease is relatively high. (2) Anti-aggregant treatment seems to be effective in many instances. (3) There were space-occupying lesion-like appearances in the liver of 7 cases by CT and US examination in the acute stage, and these disappeared on the follow-up CT and US in 5 patients but continued in 2. BCS should thus be differentiated from other liver lesions. (4) There were other great-vessel involvements in 43% of the cases, mostly venous, but only 1 pulmonary arterial occlusion.

Adolescent↗

Peritoneal hemosiderosis in pediatric patients with nephrogenic ascites.

Nephrogenic ascites associated with maintenance hemodialysis is a complex problem with poorly understood pathophysiology. We report 4 pediatric patients investigated between 1986 and 1990. All the patients treated with maintenance hemodialysis required multiple blood transfusions. Each patient was carefully evaluated for factors potentially relevant to ascites, and serum ferritin levels were found to be extremely high. Peritoneoscopy which was utilized in all patients led to a specific diagnosis of hemosiderosis in the peritoneum and liver biopsies. In 1 patient, lymph node biopsy also showed iron deposition. We believe that iron deposition played a role in changing the permeability of the peritoneum and is presumed to be a pathogenetic factor in nephrogenic ascites.

Ascites↗

Bile duct varices or "pseudo-cholangiocarcinoma sign" in portal hypertension due to cavernous transformation of the portal vein.

A total of 832 patients with portal hypertension resulting from different etiology was studied by ultrasonograph as a screening test. In 17 of the 832 patients, cavernous transformation of the portal vein was detected by means of ultrasonography. We have prospectively studied these 17 patients, and the diagnosis of cavernous transformation was confirmed by portography in all patients. To evaluate how much biliary tract has been affected from cavernous transformation of the portal vein, and to explain the cause of mildly increased alkaline phosphatase and serum bilirubin levels, endoscopic retrograde cholangiopancreatography (ERCP) was performed in 16 of the 17 patients. There were narrowing, irregularity, undulation and nodular extrinsic defects resulting from compression of thrombosis of the portal vein and the collateral vessels, mimicking cholangiocarcinoma spreading along the common bile duct on the extrahepatic biliary tract in all 16 patients who underwent ERCP. Similar ERCP findings were not found in six patients with portal hypertension due to liver cirrhosis. The ultrasonographic, portographic, and ERCP findings corresponded to surgical findings in six patients who had undergone splenectomy for either hypersplenism or bleeding from esophageal varices. The results indicate that cavernous transformation of the portal vein cause the above-mentioned radiographic findings that we propose to call "pseudo-cholangiocarcinoma sign."

Adolescent↗