Acrocallosal syndrome: report of five Turkish patients

Acrocallosal syndrome is characterized by agenesis of the corpus callosum, craniofacial anomalies, psychomotor retardation, and polydactyly. The clinical spectrum of this syndrome is broader than previous reports suggest. Five Turkish patients including dizygotic twins are reported.     

Evaluation of P-POSSUM and O-POSSUM scores in patients with gastric cancer undergoing resection

BACKGROUND/AIMS: The aim of this study was to evaluate the predictive accuracy of P-POSSUM and O-POSSUM models on patients undergoing elective gastric resection. METHODOLOGY: P-POSSUM and O-POSSUM predictor equations for mortality were applied retrospectively to 126 patients who had undergone elective gastrectomy for cancer. Observed mortality rates were compared with rates predicted by P-POSSUM and O-POSSUM using the Hosmer-Lemeshow goodness-of-fit test. Evaluation of the discriminative capability of both models was performed using receiver-operating characteristic (ROC) curve analysis. RESULTS: Overall fourteen deaths were observed. O-POSSUM predicted 15 deaths (chi2=14.61, p=0.13) and P-POSSUM predicted 20 deaths (chi2=25.41, p=0.002) using the Hosmer-Lemeshow test. ROC curves analysis revealed that O-POSSUM had better discriminatory power for mortality compared to P-POSSUM (area under curve=0.880, for O-POSSUM and area under curve=0.703 for P-POSSUM). CONCLUSIONS: These data suggest that O-POSSUM predicts mortality more accurately than P-POSSUM in patients undergoing elective gastrectomy for cancer.     

Plasma ionized magnesium levels in neonatal respiratory distress syndrome

Measurement of ionized magnesium (IMg) provides an accurate assessment of the free form of Mg, which is the physiologically active form and is most reflective of the biologically active and not easily measurable intracellular Mg fraction. Plasma levels of IMg were measured by ion-selective electrode method in premature newborns with respiratory distress syndrome (RDS), and relationships and correlations between IMg levels and various demographic, prognostic and laboratory characteristics were investigated by comparing the premature newborns with (study group; n = 19) and without RDS (control group; n = 20) in the present study. The values of the postnatal arterial pH and base excess and plasma IMg levels were significantly different between the study and control groups, and the number of newborns with any morbidity was significantly higher in the study group. Within the study group there were significant negative correlations between the plasma IMg levels and the values of the umbilical cord arterial pH (r = -0.621, p = 0.005) and base excess (r = -0.746, p = 0.001), and the value of the postnatal arterial base excess (r = -0.585, p = 0.008). The newborns who died later had higher plasma IMg levels than those who survived (0.89 +/- 0.45 vs. 0.63 +/- 0.24 mmol/l, p = 0.026). These findings suggest that increase of plasma IMg may be due to extracellular movement of Mg, which is a principally intracellular ion, as a result of acidosis, hypoxia and probable cellular injury during the early course of RDS. The exact pathophysiological mechanism responsible for IMg increase, and whether determination of plasma IMg level, including umbilical cord blood IMg measurement, can be used as an early or predictive indicator of RDS in the diagnosis remain to be determined in further large-scale studies.     

Inhibition of human cytochromes P450 by components of Ginkgo biloba

The extraction, isolation and characterization of 29 natural products contained in Ginkgo biloba have been described, which we have now tested for their in-vitro capacity to inhibit the five major human cytochrome P450 (CYP) isoforms in human liver microsomes. Weak or negligible inhibitory activity was found for the terpene trilactones (ginkgolides A, B, C and J, and bilobalide), and the flavonol glycosides. However 50% inhibitory activity (IC50) was found at concentrations less than 10 microg L(-1) for the flavonol aglycones (kaempferol, quercetin, apigenin, myricetin, tamarixetin) with CYP1A2 and CYP3A. Quercetin, the biflavone amentoflavone, sesamin, as well as (Z,Z)-4,4'-(1,4-pentadiene-1,5-diyl)diphenol and 3-nonadec-8-enyl-benzene-1,2-diol, were also inhibitors of CYP2C9. The IC50 of amentoflavone for CYP2C9 was 0.019 microg mL(-1) (0.035 microM). Thus, the principal components of Ginkgo biloba preparations in clinical use (terpene trilactones and flavonol glycosides) do not significantly inhibit these human CYPs in-vitro. However, flavonol aglycones, the biflavonol amentoflavone and several other non-glycosidic constituents are significant in-vitro inhibitors of CYP. The clinical importance of these potential inhibitors will depend on their amounts in ginkgo preparations sold to the public, and the extent to which their bioavailability allows them to reach the CYP enzymes in-situ.     

Influence of haematological parameters before coronary angioplasty on subsequent restenosis

OBJECTIVES: Restenosis is the major limitation of coronary interventions occurring in nearly a third of the patients undergoing percutaneous transluminal coronary angioplasty (PTCA) with no single, definite predictor demonstrated in an individual patient. Platelets are known to play an important role in the pathogenesis of subsequent restenosis. METHODS AND RESULTS: In a prospective study, follow-up coronary angiographies were performed in 102 consecutive patients with stable angina who underwent a successful PTCA for single-vessel coronary artery disease. Demographics, baseline lipid profiles (total cholesterol, HDL- and LDL-cholesterol, triglycerides) and haematological parameters (red cell, white cell and platelet counts, haemoglobin concentration, haematocrite %, mean platelet volume, platelet mass and fibrinogen levels) were compared between patients with and without restenosis. In the restenosis group, mean platelet volume (8.82 +/- 0.78 fl vs. 8.13 +/- 0.64 fl, p < 0.001), white cell count (8673 +/- 322 x 10(3)/microl vs. 7513 +/- 232 x 10(3)/microl, p < 0.01) and fibrinogen level (4.2 +/- 1.4 g/l vs 3.6 +/- 1.1 g/l) were significantly higher. The relative odds for developing angiographically defined restenosis were 2.49 times greater in diabetics (p = 0.11) and 2.54 times greater in men (p = 0.13). It is 1.43 times greater in patients with higher fibrinogen levels (p = 0.16). But, the relative odds for developing restenosis were 10.43 times greater in patients with larger pre-procedural mean platelet volumes (p < 0.01). CONCLUSIONS: There was a positive correlation between mean platelets volume and loss in luminal diameter between post-angioplasty and follow-up angiographies (r = +2.345, p = 0.01). There was no association between restenosis and haemoglobin, haematocrit, red cell count, white cell count, platelet count, platelet mass and plasma fibrinogen level. The development of restenosis after successful coronary angioplasty may be mainly influenced by the platelet size.     

The distribution of symptoms of attention-deficit/hyperactivity disorder and oppositional defiant disorder in school age children in Turkey

Abstract Objective The aim of this study was to determine the prevalence of attention-deficit/hyperactivity disorder (ADHD) and oppositional defiant disorder (ODD) in school age children in Sivas province centre and to examine the distribution of symptoms according to subtypes. Method The study population was made up of 1425 children between the ages of 6 and 15 from eight primary schools selected by random sampling method in Sivas province centre. All students were given a questionnaire prepared according to DSMIV criteria that was completed by their parents or teachers. This form includes 18 symptoms of ADHD and eight symptoms of ODD. Students t-test and variance analysis were used for statistical evaluation. Results A prevalence of 8.1% was found for ADHD in our study. According to subtypes, 32.2% of these were inattentive, 40% were hyperactive and 27.8% were combined type. A prevalence of 11.5% was found for ODD. The boy/girl ratio was > 1 for both ADHD and ODD, and 52.2% of those with ADHD were established as having ODD at the same time. Conclusions The prevalence of ADHD and ODD in primary school children was not considered to be small. Studies concerning the distribution of symptoms in different societies, cultures, and viewpoints and identification of children with this type of problem are beneficial for early diagnosis and prophylactic treatment.     

Evaluation of bone mineral density in children with cerebral palsy

In the present study, bone mineral density of 40 children with cerebral palsy (study group) and the effects of various risk factors on bone mineralization in these children were investigated by comparing with 40 age-matched healthy children (control group). Weight, height, skinfold thickness, body-mass index measurements, and serum levels of calcium, phosphorus, alkaline phosphatase and 25 OH vitamin D were not significantly different between the study and control groups (p>0.05). The mean bone mineral density value of the study group measured by dual-energy X-ray absorptiometry method at L2-L4 levels of lumbar vertebrae was significantly lower than that of the control group (p<0.05). When the patients in the study group were assessed with respect to ambulation status, pattern of involvement, calcium and energy intakes, and whether or not they had taken and/or were taking a regular physical therapy program, there was a significant difference only between the hemiplegic and tetraplegic patients (p<0.05), while there were no significant differences among the patients who were ambulant versus non-ambulant, who had sufficient versus insufficient calcium and energy intakes, and who did and did not take a regular physical therapy (p>0.05). Although the ambulatory status, quantity of calcium and energy intakes, and the presence or absence of a physical therapy program had no effects on bone mineral density values of the children with cerebral palsy in this study, the exact factors and mechanisms responsible for the reduced bone mineral density in children with cerebral palsy should be investigated in further large-scale studies considering the increased risk of pathological fractures in these patients.     

Underlying causes of recurrent pneumonia in Turkish children in a university hospital

The aim of this study was to determine the relative frequency of underlying illnesses for recurrent pneumonia in children. Children who had two or more episodes of pneumonia per year, or three or more episodes in a lifetime were investigated retrospectively at Ankara University Medical School, Department of Pediatric Infectious Diseases, between January 1997 and October 2002. Out of 788 children hospitalized for pneumonia, 71 (9 per cent) met the criteria for recurrent pneumonia. An underlying illness was demonstrated in 60 patients (85 per cent). In this group, the underlying illness was diagnosed prior to pneumonia in 11 patients (18.3 per cent), during the first episode in 12 patients (20 per cent), and during recurrence in 37 patients (61.7 per cent). Underlying diseases were bronchial asthma (32 per cent), gastroesophageal reflux (15 per cent), immune disorders (10 per cent), congenital heart defects (9 per cent), anomalies of the chest and lung (6 per cent), bronchopulmonary dysplasia (4 per cent), cystic fibrosis (3 per cent), tuberculosis (3 per cent), and aspiration syndrome (3 per cent). No predisposing illness could be demonstrated in 11 patients (15 per cent). In conclusion, approximately one-tenth of hospitalized children with pneumonia in our hospital had recurrent pneumonia. Most of these children had an underlying illness, which was demonstrated by intensive investigation. Bronchial asthma in children aged more than 2 years and gastroesophageal reflux in children aged less than 1 year were the most common underlying illnesses for recurrent pneumonia.     

Adult type granulosa cell tumor causing precocious pseudopuberty in a 6 year-old girl

Malignant ovarian tumors are responsible for 2-3% of all cases of precocious pseudopuberty (PP) in girls. The most common forms of ovarian tumors presenting as PP are the granulosa cell tumors (GCT). The clinical and pathological features of granulosa cell tumors that occur frequently in young girls, so-called juvenile granulosa cell tumors (JGCT), differ histologically from those occurring in adults. As a cause of PP in young girls, adult type granulosa cell tumors (AGCT) are extremely rare. We report a 6 year-old girl presenting with early breast development and vaginal bleeding due to a well encapsulated ovarian tumor. Microscopic features of the resected tumor were characteristic of AGCT.     

Ligneous conjunctivitis,hydrocephalus, hydrocele,and pulmonary involvement in a child with homozygous type I plasminogen deficiency

Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis which usually starts in early infancy. Plasminogen deficiency has recently been associated with ligneous conjunctivitis. The disease may be associated with pseudomembranous lesions of other mucous membranes in the mouth, nasopharynx, trachea, and female genital tract and also with congenital hydrocephalus. In this report, a 1-month-old Turkish boy who had pseudomembranous conjunctivitis, occlusive hydrocephalus, and hydrocele is presented. After surgery for ventriculo-peritoneal shunt establishment, he developed inspiratory stridor, respiratory distress, and pulmonary atelectasis. Tracheal pseudomembranes were also demonstrated by bronchoscopy. Plasminogen antigen level and plasminogen activity were very low. Genomic DNA from the patient was screened for mutations in the plasminogen gene and a homozygous L650fsX652 mutation (deletion of 2081C) was detected. Both of his parents were heterozygous for this mutation. He died due to respiratory failure during follow-up. CONCLUSION: Ligneous conjunctivitis related to type I plasminogen deficiency is relatively common in the Turkish population, however, mutations are heterogeneous and a common founder is unlikely.