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Biomedical subjects

G Erdem

Publications and source records attributed to G Erdem.

At least 37 records · Page 2Linked to original sources
PubMed1996

A case of Pearson syndrome associated with multiple renal cysts.

A 41-day-old infant who had severe metabolic acidosis, anemia, bleeding, hypoglycemia, and proximal tubulopathy was diagnosed with Pearson syndrome. Fibrosis in the liver, severe iron deposition in hepatocytes, and multiple renal cortical cysts were found on postmortem examination. Southern blot analysis of mitochondrial DNA obtained from peripheral blood revealed a heteroplasmic deletion of approximately 3.5 kilobases.

DNA, Mitochondrial↗
PubMed1996

Investigation of ascorbate-Cu (II) induced cleavage of DNA by scanning tunneling microscopy.

Scanning Tunneling Microscopy (STM) was used for the investigation of oxidative DNA damage. A PCR amplified fragment of human beta-globin gene was used as a model for time dependent cleavage reaction by ascorbate and copper. Cleavage reactions were carried out in a medium containing 0.5 microgram/20 microliters DNA, 20 nM Tris-HC1 pH, 7.8 and ascorbate-Cu (II) in the final concentrations of 1 mM and 30 microM, respectively. The mixtures were incubated at 37 degrees C for 5, 15 and 30 min. For STM studies, 3 pg/5 microliters DNA samples were deposited on the gold coated mica and dried in a water flow vacuum drier. The STM was operated in air at atmospheric pressure with a tip-to-substrate bias of 100 mV and tunneling currents of < 10 pA. Etched tips of Pt/Ir wires were used in a constant current mode. The degradated DNA structure can be distinguished from the intact DNA and the sizes of the degradation products can be identified in the STM micrographs. The size of fragments decreased from approximately 3000 A to 34 A in ascorbate-Cu (II) medium, after 30 min of incubation.

Ascorbic Acid↗
PubMed1996

Long-term follow-up of indirect hyperbilirubinemia in full-term Turkish infants.

OBJECTIVE: This retrospective follow-up study was performed to evaluate the suitability of the recently reported exchange transfusion limits (serum indirect bilirubin level of 428-496 mumol/1, 25-29 mg/dl) for Turkey. MATERIAL AND METHODS: The study groups totalled 102 children, 8-13 years of age, who had been born at term with birthweights greater than 3000 g and had been treated for indirect hyperbilirubinemia during their newborn period; the control group consisted of 27 children of the same age-group without indirect hyperbilirubinemia. Children were grouped according to their maximum serum and bilirubin levels and direct Coomb's test results. Physical and neurological examinations, visual and brainstem auditory evoked potentials and the Wechsler Intelligence Scale for children--Revised for Turkish Children were performed. RESULTS: There was no difference between the groups with regard to mean visual and brainstem auditory evoked potential latencies. Children whose direct Coomb's tests were positive had significantly lower IQ scores and more prominent neurological abnormalities (p < 0.05). IQ scores and prominent neurological abnormalities did not differ among the other groups. Nine children had prominent neurological abnormalities associated with abnormal brainstem auditory evoked potentials. An important risk factor was the duration that the infant's serum indirect bilirubin level remained greater than 342 mumol/l (20 mg/dl). CONCLUSION: The current limit of 342 mumol/l should continue to be used for infants whose direct Coomb's tests are positive in our country. Until better criteria for exchange transfusion other than the indirect bilirubin level are established, the current limits should also still be followed for infants whose direct Coomb's tests are negative in Turkey, where regular neonatal follow-up examinations are not satisfactory.

Adolescent↗
PubMed1996

A novel (delta beta)(0)-thalassemia due to a approximately 30-kb deletion observed in a Turkish family.

A new deletion of the beta-globin gene cluster was characterized in a Turkish family. A 6-year-old male and his father were heterozygotes for this deletion. They presented with mild hypochromic microcytic anemia associated with elevated Hb F (15%) and normal Hb A2 levels (2.0%). This newly described Turkish type (delta beta)(0) thalassemia has a deletion of about 30 kb. The 5' breakpoint of this deletion starts approximately 1.5 kb downstream of an enhancer-like sequence of the A gamma-globin gene. The 3' endpoint is located in the L1 repeat sequence (Kpnl site) 3' to the beta-globin gene. The new deletion (Turkish type 3) is quite similar to that of the Indian (delta beta)(0)-thalassemia deletion in size and 5' breakpoint. However, the 3' endpoint in this new deletion is 2.5 kb shorter than the Indian type.

Adult↗
PubMed1996

Clinical and magnetic resonance imaging features of L-2-hydroxyglutaric acidemia: report of three cases in comparison with Canavan disease.

We report three cases of L-2-hydroxyglutaric acidemia and three cases of Canavan disease. The L-2-hydroxyglutaric acidemia cases are the first biochemically proven Turkish cases. Magnetic resonance imaging findings in the cases and similarities between the two diseases are emphasized. Both diseases are characterized by predominant subcortical white-matter involvement and dentate nuclei lesions with variable basal ganglia involvement. Canavan disease differs from L-2-hydroxyglutaric acidemia by the presence of typical brainstem involvement.

Canavan Disease↗
PubMed1995

Cerebrospinal fluid amino acid levels in newborn infants with intracranial hemorrhage.

Cerebrospinal fluid (CSF) amino acid levels including excitatory amino acids (i.e. glutamate and aspartate) in 25 preterm and 18 full-term newborn infants with no serious disease except intracranial hemorrhage (ICH) were measured. ICH was detected in 13 preterm and six full-term infants on the basis of the clinical, lumbar puncture (LP) and cranial ultrasonography (CraUSG) findings. Twelve preterm and 12 full-term infants who were neurologically healthy comprised the control group. The mean concentration of CSF amino acids did not differ between preterm and full-term infants. The CSF concentrations of taurine, threonine, glycine, alanine, valine, isoleucine, leucine, tyrosine and phenylalanine in preterm infants, and threonine, aspartic acid and alanine in full-term infants were significantly elevated in infants with ICH. These abnormalities, especially in preterm infants, are probably related to cerebral hypoxia in CSF amino acid concentrations in newborn infants with ICH.

Amino Acids↗
PubMed1994

Lymphoma with bilateral cavernous sinus involvement in early childhood.

A 4-year-old girl developed complete ophthalmoplegia with intact pupillary responses. Computed tomography and magnetic resonance imaging demonstrated a mass invading the cavernous and sphenoid sinuses and posterior ethmoidal cells. Biopsy revealed non-Hodgkin lymphoma. This patient is the youngest reported with malignant lymphoma of the cavernous sinus and the second reported with bilateral cavernous sinus involvement.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1994

Dermoid tumor with persistently low CSF glucose and unusual CT and MRI findings.

A patient with dermoid tumor, presenting with persistent hypoglycorrhachia in the absence of cerebrospinal fluid pleocytosis is reported. The presence of ring enhancements without infection, surrounding edema, and mass effect on computed tomography and magnetic resonance imaging are unusual findings for these tumors.

Blood Glucose↗
PubMed1994

Infantile spasms as the initial symptom of biotinidase deficiency.

Two patients with biotinidase deficiency had diagnoses of infantile spasms made at 1 month of age. Biotinidase deficiency may be seen early in the neonatal period without the characteristic findings such as alopecia and seborrheic dermatitis. This diagnosis should be considered in patients with infantile spasms.

Amidohydrolases↗
PubMed1993

Evoked potentials in full-term and premature infants: a comparative study.

In this study the maturation of the central nervous system of full-term and premature infants were investigated electrophysiologically. The subjects were 16 full-term and 15 premature infants. Neurologic examination, psychometric tests, and measurement of evoked potentials were carried out periodically in babies who had no birth trauma, metabolic disorder, or intrauterine infection. Neurophysiologic comparison of the results was evaluated. As the babies grew older, I-V interpeak latency became shorter according to the results of brainstem auditory evoked potentials; N1-P1 amplitude became higher and P1 latency shorter according to the results of visual evoked potentials. Central nervous system maturation of full-term babies and prematures appear to be alike at 6 months of age.

Brain Stem↗
PubMed1991

Relation between the birthweight and cord, maternal serum and amniotic fluid growth hormone levels.

Maternal and cord serum and amniotic fluid growth hormone (GH) levels were measured in 55 term pregnancies in order to evaluate the role of GH in prenatal fetal somatic growth. The study group consisted of patients who had small, large, and average for gestational age infants. The difference in mean maternal and cord serum and amniotic fluid GH levels between the three weight groups were found to be insignificant. It is concluded that GH does not influence fetal somatic growth.

Adolescent↗