PubMed Health⌕ Search

Biomedical subjects

G Ciliv

Publications and source records attributed to G Ciliv.

At least 19 recordsLinked to original sources
PubMed2000

Glycogen storage disease type Ia: frequency and clinical course in Turkish children.

The aim of this study was to determine the relative frequency of type Ia in glycogen storage disease (GSD) with prominent liver involvement and to determine its clinical and laboratory findings and prognosis in Turkish children. From 1980 to 1998, 45 out of 100 GSD patients (27 male) with liver involvement had been diagnosed for type Ia. The files were retrospectively evaluated and clinical and laboratory features were documented. In addition to routine laboratory evaluations, urine albumin, calcium excretions, and plasma biotinidase activity were measured. Breast-feeding was continued in all infants. After 6 months of age, uncooked cornstarch was administered to the patients. The relative frequency of type Ia in GSD with liver involvement was 45%. The diagnosis was made in 71% of patients before 2 years of age (median 1 year). Main complaint was abdominal protruding (57.8%), and main physical finding was hepatomegaly (100%). Forty percent of the patients had growth retardation at diagnosis. Among laboratory parameters, hypertriglyceridemia (97.8%) and hypertransaminasemia (95.6%) were the most frequent findings following plasma biotinidase activity, which was elevated in all patients. Microalbuminuria was determined in 52.8% of the patients and hypercalciuria in 23.8%. Histopathological findings of the liver included fibrosis (75.6%), steatosis (37.8%), mosaicism (24.4%) and nuclear hyperglycogenation (15.6%). During follow-up period, the ratio of patients with growth retardation did not change. Transaminases were decreased in 48.7% of the patients. Although triglyceride and cholesterol levels decreased in the majority of the patients, they did not normalise. The prevalence of type Ia in GSD with prominent liver involvement was found higher than the other reports. Microalbuminuria was also higher than the previous reports.

Adolescent↗
PubMed2000

Short-term effect of captopril on microalbuminuria in children with glycogen storage disease type Ia.

Early signs of renal dysfunction in glycogen storage disease type Ia (GSD Ia) are glomerular hyperfiltration and proteinuria. In a non-randomized study, the effect of captopril on the improvement of proteinuria in GSD Ia patients with microalbuminuria was investigated. A positive effect has been shown for the insulin-dependent diabetes mellitus patients. Microalbuminuria was defined as albumin/creatinine ratio (mg/mmol) more than 2.5 in spot urine. Nineteen (52.7%) out of 36 patients had microalbuminuria, and 8 patients received captopril at a dose of 1 mg/kg per day. Microalbuminuria was evaluated periodically during the follow-up period. Of the captopril-treated patients, one was lost to follow-up. In the remaining 7 patients, urinary albumin excretion normalized in 3 patients (42.9%) and decreased at least by 50% in another 3 patients (42.8%) after 6 months of treatment. One patient, who was the oldest, did not have any benefit. In untreated patients, only two patients had a decrease in microalbuminuria of more than 50%. Patients with microalbuminuria had significantly higher blood lactate (p < 0.05) and plasma triglyceride (p < 0.01) concentrations and significantly lower blood bicarbonate concentration (p < 0.05) than those patients without it. Additionally, the patients with microalbuminuria had been diagnosed earlier than those without microalbuminuria (p < 0.05). Patients with microalbuminuria have more severe clinical and laboratory findings than those without microalbuminuria. Captopril at a dose of 1 mg/kg per day seems to be effective in at least 50% of GSD Ia patients with microalbuminuria.

Adolescent↗
PubMed2000

Serum adenosine deaminase levels in patients with psoriasis: a prospective case-control study.

Adenosine deaminase (ADA) activity is a nonspecific marker of T cell activation. T cell activation is thought to play an important role in the pathogenesis of psoriasis. Our purpose was to assess the significance of serum ADA activity in psoriasis and its relevance to disease activity. ADA activity was determined with an enzymatic method in 25 patients with psoriasis and in 15 healthy subjects. These measurements were repeated for 10 patients after either PUVA or cyclosporin A treatments. Disease activity was estimated by the PASI scoring system. Serum ADA level was significantly elevated in patients with psoriasis compared to healthy subjects (p<0.05). There was a significant decrease in the ADA levels after treatment compared to pretreatment values in the same patients (p<0.05). There was no correlation between ADA levels and PASI scores. These results support the evidence that T cell activation is involved in the pathogenesis of psoriasis and that ADA may be valuable in the assessment of disease activity in psoriasis.

Adenosine Deaminase↗
PubMed1999

Diagnostic value of adenosine deaminase activity in pericardial fluids.

BACKGROUND: The activity of adenosine deaminase (ADA) was determined in serum and pericardial fluid of 70 patients (ages 21 to 71 years) with pericardial effusions of various etiologies and in 15 control subjects. METHODS: The patients were subdivided into five groups on the basis of definite diagnosis: 1) 24 patients with tuberculosis; 2) 22 with malignancies; 3) 12 with uremic pericarditis; 4) 12 with purulent pericarditis; 5) 15 control individuals without pericardial disease. The activity of ADA was determined at the same time in serum and cell-free pericardial fluid according to the method of Karker with minor modification. RESULTS: Mean (+/-SD) ADA activity in pericardial fluid was 66.92+/-4.12 IU/L in group 1; 27.50+/-6.02 in group 2; 28.65+/-4.73 in group 3; 53.05+/-11.14 in group 4; and 5.67+/-1.99 in group 5. Comparing the level achieved in group 1 with all others, the difference is significant at the p<0.001 level. When the cut-off value of 50 IU/L is used the sensitivity of the test for diagnosis of tuberculous effusion is 1, and the specificity is 0.83. Statistical analysis showed that there was no correlation between serum ADA activity and ADA activity in pericardial fluid. CONCLUSIONS: We recommend that determinations of ADA activity in pathologic pericardial fluids seem to be of great value in the early diagnosis of tuberculous pericardial effusions. Levels above 50 IU/L in effusions indicate probable tuberculosis.

Adenosine Deaminase↗
PubMed1995

Comparison of halothane, enflurane and isoflurane kidney effects through alanine aminopeptidase/urine creatinine values.

The kidney effects of halothane, enflurane and isoflurane were evaluated by using the ratio of urinary excretion of alanine aminopeptidase (AAP) to urine creatinine. Thirty patients in ASA class 1 or 2 were studied. None had renal disease nor received nephrotoxic drugs. Groups 1, 2 and 3 received halothane, enflurane and isoflurane respectively. Creatinine and AAP activities in urine spot tests, serum creatinine and BUN levels were determined preoperatively and on the first and second postoperative days. Urine AAP activity and AAP/urine creatinine values increased significantly on the first and second postoperative days compared with the preoperative values in all groups (P < 0.05). The present study did not reveal any significant difference in the kidney effects of halothane, enflurane and isoflurane through AAP/creatinine in spot urine values.

Adult↗
PubMed1993

Effects of recombinant human erythropoietin on sodium balance in nondialysed children with chronic renal failure.

In this study rhEPO, 70 U/kg, was subcutaneously administered two times a week for eight weeks to twelve nondialysed patients with renal anaemia and chronic renal failure. Renal function tests, blood pressure, Hb, Hct, FENa and Na-K-ATPase enzyme activity before and after administration of rhEPO have been studied. We have searched for correlations, if any, between these parameters. After the treatment period mean Hb concentration and mean Hct values increased from 7.5 +/- 0.3 to 8.6 +/- 0.5 g/dl and from 22.5 +/- 1.2 to 26.7 +/- 1.6%, respectively (p < 0.05), whereas no significant differences between pretreatment and posttreatment Cr and CrCl values were found (p > 0.05). No changes in blood pressure were found throughout the study. While mean FENa decreased from 10.2 +/- 1.6 to 6.15 +/- 1.05% (p < 0.05), mean Na-K-ATPase enzyme activities decreased from 0.120 +/- 0.016 to 0.095 +/- 0.025 mumol Pi/h/mg protein (p > 0.05) after treatment. In conclusion, subcutaneous administration of rhEPO, at doses of 70 U/kg twice a week in nondialysed patients increased Hb and Hct values. A significant decrease of FENa was observed after rhEPO treatment and there was no correlation between Na-K-ATPase enzyme activity and FENa.

Adolescent↗
PubMed1992

Serum selenium status in children with iron deficiency anemia.

Serum selenium concentration was investigated in 40 children with iron deficiency anemia and in 40 control subjects matched for age, sex and geographical origin. A spectrofluorometric method was used for determination of the selenium level. It was found to be significantly lower (p < 0.001) in the patient group, which consisted of both normally developed and malnourished children. Patients also having pica had higher levels of selenium compared to patients without pica. There was no relation between the serum selenium concentration and hematological parameters such as hemoglobin, serum iron, serum iron binding capacity and unsaturated iron binding capacity. However the results of 15 patients followed during iron therapy indicated that the duration of the anemic period may affect the selenium concentration. This study also suggests the effectiveness of iron and selenium administration.

Adolescent↗
PubMed1990

Androgen receptors in laryngeal carcinoma.

Specific androgen receptors were assayed from the cytosol fraction of laryngeal carcinomas from 15 male patients. Specimens also included the non-malignant laryngeal tissues from 3 of these patients. A specific radioligand was used and involved (3H) dihydrotestosterone incubation and a dextran-coated charcoal technique. Twelve of the laryngeal carcinomas showed detectable levels of high affinity but limited capacity androgen receptors. The number of binding sites varied from zero to 23.3 fmol/mg cytosol protein, with the dissociation coefficient (Kd) ranging from 0.46 to 1.85 x 10(-9) M. Androgen receptors were also found in all three non-malignant laryngeal tissues. Binding sites for these latter androgen receptors were 4.09 to 8.16 fmol/mg cytosol protein, with a Kd of 0.81 to 0.35 x 10(-9) M. Although further studies are necessary, the presence of androgen receptors in laryngeal carcinomas suggests that antiandrogen therapy might be used in the future for treating such disease.

Binding Sites↗
PubMed1985

Studies on lymphocyte cell surface in ataxia-telangiectasia.

Lymphocyte surface proteins of patients with ataxia-telangiectasia were separated by polyacrylamide gel electrophoresis and compared by autoradiography. The patients lacked one of the two main bands (Band I at the origin). The second main band (Band II) was absent in some cases. All patients had one or two additional bands of smaller molecular weight than Band II except one case who had no band detectable. In the patients, alkaline phosphatase, total ATPase and Mg2+. ATPase were increased but 5'-nucleotidase was normal. The results suggest abnormality in the plasma membranes of the patients' lymphocytes.

5'-Nucleotidase↗
PubMed1982

Effects of diethylstilbestrol diphosphate on serum and tissue ribonuclease levels in prostatic carcinoma.

Thirty-six patients with prostatic carcinoma and benign prostatic hyperplasia (BPH) were studied. Transurethral resection (TUR) was performed in all, and serum and tissue ribonuclease (RNase) levels were determined with the spectrophotometric method using yeast RNA as the substrate. In patients with untreated prostatic carcinoma, statistically significant increased RNase levels were found in serum and tissue. Treatment with diethylstilbestrol diphosphate (DESDP) led to a decrease in RNase levels. In the same patients RNase serum levels after DESDP treatments also showed a parallel decrease with serum prostatic acid phosphatase (sPAP) levels. We have concluded that the serum RNase measurement may be useful for TNM classification and immunostaging.

Acid Phosphatase↗
PubMed1980

Tissue acid and alkaline phosphatase in prostatic carcinoma.

Twenty-eight patients admitted with infravesical obstruction symptoms were studied. Fourteen of these patients were suffering from prostatic carcinoma defined as inoperable. The remaining 14 patients were diagnosed as benign prostatic hyperplasia (BPH) and transurethral resection (TUR) was performed to all of them. Specimens obtained by TUR were used to analyze tissue activities of total and tartarate labil acid phosphatase, and alkaline phosphatase. Enzymatic levels in the tissue were estimated by using fluorimetric method (FU/mg). The estimated averages seemed to vary in favor of the malignant tissue, however this variation was not found to be statistically significant. Different inhibition levels were observed in benign and malignant tissue with the addition of L(+) tartarate. The average levels of the alkaline phosphatase were found to be lower than those of acid phosphatase, but the former showed no difference between the malignant and benign groups.

Acid Phosphatase↗
PubMed1979

Myeloperoxidase activity and bactericidal function of PMN in iron deficiency.

In children with iron deficiency anemia, bactericidal capacity of polymorphonuclear leukocytes (PMN) and serum opsonic activity were studied. Nitroblue tetrazolium test (NBT), hexose monophosphate (HMP) shunt activation, and myeloperoxidase (MPO) activity of PMN of these cases were also examined. Bactericidal capacity and HMP shunt activation were found to be decreased in iron deficiency anemia ( p less than 0.001). MPO activity, NBT test, and serum opsonic activity were found to be within normal limits. After 1 1/2 months of iron therapy there was an improvement in bactericidal capacity and it returned to a normal level after 3 months of therapy.

Adolescent↗