Biomedical subjects

Ilmay Bilge

Publications and source records attributed to Ilmay Bilge.

8 recordsLinked to original sources

PubMed2006

Crescentic glomerulonephritis in a child with infective endocarditis.

Renal manifestations associated with infective endocarditis (IE) may present with different clinical patterns, and the most common renal histopathological finding is diffuse proliferative and exudative type of glomerulonephritis, leading to hematuria and/or proteinuria. Renal failure due to crescentic glomerulonephritis (CGN) in children with IE is a very rare condition. We report here a 6-year-old boy, who had a history of cardiac surgery for pulmonary atresia and ventricular septal defect, presenting with the clinical findings of IE and hematuria associated with renal failure due to CGN. He was treated with a combination of intravenous (IV) methylprednisolone pulses and appropriate antibiotics, but also received one dose of IV cyclophosphamide. Complete serological, biochemical, and clinical improvement was achieved after 2 months of follow-up. Antibiotic therapy is the essential part of the treatment of IE-associated glomerulonephritis; however, this case also highlights the importance of aggressive immunosuppressive therapy to suppress the immunological process related with infection in this life-threatening condition leading to renal failure.

Anti-Bacterial Agents↗

PubMed2005

Central nervous system vasculitis secondary to parvovirus B19 infection in a pediatric renal transplant patient.

Central nervous system (CNS) vasculitis secondary to chronic parvovirus B19 (B19) infection presenting with recurrent neurological findings is a very rare disorder during childhood. Here we report a 12-year-old boy with a renal transplant who had chronic B19 infection with skin eruptions and recurrent episodes of encephalopathy with focal neurological deficits. B19 DNA was detected in blood, bone marrow, and skin biopsy specimens. Repeat cranial magnetic resonance (MR) imaging during each episode of encephalopathy showed variable focal findings, and MR angiography revealed vasculitic changes with narrowing of the cerebral arteries. We hypothesized that the CNS vasculitis might be associated with the chronic B19 infection. At the time of his fourth presentation with the same clinical findings, we administered intravenous immunoglobulin (IVIG) (1 g/kg per day, 2 consecutive days), which we continued for 6 months, at monthly intervals. IVIG therapy resulted in remission and has been effective not only for the clearance of B19, but also for the improvement of clinical and radiological findings of CNS vasculitis. We suggest that chronic B19 infection should be considered in immunocompromised patients with suspected CNS vasculitis. IVIG should be considered as a part of the treatment.

Brain Diseases↗

PubMed2004

Biochemical bone markers in nephrotic children.

In this study we evaluated the effects of high-dose corticosteroid (CS) therapy and the character of the nephrotic syndrome (NS) itself on bones in patients with normal glomerular filtration rate. We measured serum osteocalcin (OC), alkaline phosphatase (ALP), intact parathyroid hormone (iPTH), 25-hydroxyvitamin D, calcium (Ca), phosphorus (P), and magnesium (Mg) levels, and urinary Ca and protein excretion in nephrotic children during the active phase before (group Ia) and after CS treatment (group Ib). The results were compared with age-matched control subjects. A significant increase in urinary Ca excretion was observed after CS treatment. Serum ALP, OC, and iPTH levels were within normal limits at the time of study entry. However, both serum OC and ALP levels showed a significant decrease after the completion of CS treatment (OC from 13.6+/-9.2 ng/ml to 6.7+/-5.2 ng/ml and ALP from 151.8+/-60.2 U/l to 116+/-43.8 U/l). 25-Hydroxyvitamin D levels increased to 17.2+/-8.9 microg/l from 9.9+/-6.9 microg/l after CS treatment. The effects of recurrent use of CSs were assessed by dividing nephrotic patients into two subgroups: infrequent relapsers (IFR) and frequent relapsers (FR). The cumulative dose of CS was 28,125 mg/m(2) for IFR and 105,000 mg/m(2) for FR. The changes in OC, ALP, and 25-hydroxyvitamin D levels after CS treatment were significantly different between IFR and FR. We conclude that high-dose CS treatment causes a decrease in bone formation, as shown by the changes in OC and ALP levels. 25-Hydroxyvitamin D levels remained lower than control subjects after CS therapy. The higher the cumulative dose of CS used the more marked the changes in biochemical bone markers. The contribution of FR to baseline 25-hydroxyvitamin D levels needs further study.

Biomarkers↗

PubMed2004

ACE I/D gene polymorphism in primary FSGS and steroid-sensitive nephrotic syndrome.

The role of angiotensin-converting enzyme (ACE) insertion/deletion (I/D) gene polymorphism in various renal disorders has been investigated. We evaluated the association between the clinical characteristics and ACE genotypes of Turkish children with primary focal segmental glomerulosclerosis (FSGS) and steroid-sensitive nephrotic syndrome (SSNS). Patients with FSGS ( n=30) were classified into two groups: one with remission together with stable renal function ( n=22) and the other without remission and with impaired renal function ( n=8). We classified children with SSNS ( n=43) that were followed for at least 4 years into two subgroups as having more frequent ( n=19) and less frequent relapses ( n=11). The DD genotype was more frequent in the SSNS group than that in controls (37% vs. 17%, chi(2)=4.98, P=0.025). However, among SSNS subgroups, the frequency of the DD genotype was not different. The distribution of ACE genotype was similar among patients with FSGS and SSNS. There was no difference in the ACE I/D distribution between children with FSGS and normal controls (II 10%, ID 60%, DD 30% vs. II 13%, ID 70%, DD 17%). The frequency of the DD genotype was higher in FSGS patients with declining renal function (63%) than in those with stable renal function (18%) ( P=0.031). Progressive renal impairment was significantly more frequent in patients with FSGS with the homozygous D allele compared with FSGS patients with ID and II genotypes. Our results indicate that the DD genotype may be a risk factor for the development of progressive renal impairment in children with FSGS; however, larger studies are required to confirm this.

Child↗

PubMedDate not supplied

Is paraoxonase 192 gene polymorphism a risk factor for membranoproliferative glomerulonephritis in children?

We investigated the effects of paraoxonase (PON1) 192 polymorphism on serum PON1 activity and the impact of phenotypic expression on the risk and prognosis of Turkish children with membranoproliferative glomerulonephritis (MPGN). Eighteen children with biopsy-proven Type I MPGN (10 boys, 8 girls) and age-matched 53 healthy controls were included in the study. PCR (polymerase chain reaction), RFLP (restriction fragment length polymorphism) and agarose gel electrophoresis techniques were used to determine the PON1 192 genotype. PON1 activity was measured by spectrophotometric assay of p-nitrophenol production following addition of paraoxon. We found that PON1 192 genotype distribution (AA, AB, BB) in MPGN patients were 61.1%, 22.3%, 16.6% and 15.1%, 35.8%, 49.1% in controls, respectively. The frequency of AA genotypes was significantly higher in the MPGN group (0.611) compared with the healthy controls (0.151) (p < 0.001). Although the serum PON1 activity was lower in MPGN patients (103.3 +/- 55.2 U/l) than the healthy controls (130.9 +/- 71.2 U/mol), the difference was not statistically significant (p = 0.0563). In the genotypes of patients and controls classified according to PON1 A/B polymorphism; serum PON1 activities were significantly increased (p < 0.001, ANOVA) in the order of PON1 AA, AB and BB in both MPGN patients (82.4, 91.7 and 173.6 U/l) and healthy controls (85.9, 119.9 and 193.1 U/l), respectively. There was a significant relationship between the poor prognosis and having AA genotype and low PON1 activity. Of the 8 patients with poor prognosis, 7 had genotype AA and the remaining one was AB heterozygote. Our results suggest that homozygosity for the A allele might have an important role on the risk for developing MPGN and may also be associated with the poor prognosis of disease. In conclusion, we suggest that the PON1 activities are affected by PON1 genetic variability in Turkish patients with MPGN.

Adolescent↗

PubMedDate not supplied

Pyelolithotomy in a patient with Glanzmann thrombasthenia and antiglycoprotein IIb/IIIa antibodies: the shortest possible duration of treatment with recombinant activated factor VII and platelet transfusions.

Transfusion of platelet concentrates remains the first-line therapy for Glanzmann thrombasthenia in case of bleeding or preparation for surgery. However, development of antibodies to platelet glycoprotein (Gp) IIb/IIIa complex or human leukocyte antigens (HLA) is frequent and the main cause of platelet refractoriness. Recombinant activated factor VII (rFVIIa) is a potent alternative for patients with Glanzmann thrombasthenia with anti-platelet antibodies. We describe a case of Glanzmann thrombasthenia with alloantibodies to platelet Gp IIb/IIIa complex who underwent a successful pyelolithotomy operation under the coverage of recombinant activated factor VIIa and platelet transfusions.

Child, Preschool↗

PubMedDate not supplied

Brain calcification due to secondary hyperparathyroidism in a child with chronic renal failure.

Secondary hyperparathyroidism (SHPT) has been better treated over the last decades, but the rate of metastatic calcifications, which were rarely seen before, was significantly increased in dialysis patients. The presence of uncontrolled SHPT, disorders of calcium (Ca) and phosphorus homeostasis and the common usage of large doses of vitamin D and Ca- containing phosphate binders may all contribute to the metastatic calcifications of soft tissues and vasculature leading to some life-threatening complications. Although the metastatic lung, heart, kidney, intestinal wall, skin, eye and soft tissue calcifications have been commonly reported in adults and also in children undergoing dialysis, the central nervous system calcification is a very rare condition. We report here a pediatric hemodialysis patient who presented with severe neurological findings due to the metastatic brain calcification secondary to his uncontrolled hyperparathyroidism.

Adolescent↗

PubMedDate not supplied

Steroid-induced psychosis in an adolescent: treatment and prophylaxis with risperidone.

Steroid-induced psychotic disorder is one of the serious adverse effects of corticosteroid therapy and is characterized by hallucinations and delusions. While the mechanism is unclear, treatment of steroid psychosis involves dosage reduction or discontinuation of prednisone. In cases where this cannot be done, typical treatment involves an antipsychotic medication. Although it is a well-known complication in adulthood, literature about steroid-induced psychotic disorder in children and adolescents is lacking. Here we report a 12-year-old case of steroid-induced psychotic disorder who was treated with an atypical antipsychotic, risperidone, and in whom the antipsychotic therapy was maintained because of continuation of her corticosteroid treatment for nephrotic syndrome. Pediatricians should be aware of this rare problem when prescribing corticosteroids in this age group. To our knowledge, this is the first reported case of steroid-induced psychosis successfully treated with risperidone in an adolescent with nephrotic syndrome.

Antipsychotic Agents↗