Leptin and plasminogen activator inhibitor-1 levels in children on chronic dialysis.

PURPOSE: In this study, it is aimed to compare the serum leptin and PAI-1 levels and evaluate their relationship in children on hemodialysis (HD) and peritoneal dialysis (PD). METHOD: Thirty-six patients on HD (mean age: 15.0 ± 2.8 years), 19 patients on PD (mean age: 13.0 ± 3.5 years) and 15 healthy subjects (mean age: 14.5 ± 2.7 years) were included in the study. Laboratory investigations included blood count, biochemical parameters, serum iron, iron binding capacity, parathormone, erythrocyte sedimentation rate, C-reactive protein (CRP), prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, serum leptin and PAI-1 levels. RESULTS: Serum leptin levels were significantly higher in HD group than in control group when the effects of BMI and sex were controlled, while PD and control groups had similar leptin levels. PAI-1 levels were also significantly higher in HD group than in control group, while there was no statistically significant difference in PAI-1 levels of PD and control group. PAI-1 levels and leptin levels were significantly correlated, which was independent of the effect of BMI in both HD and PD groups when they are evaluated separately. CONCLUSION: Results of our study showed that HD patients had higher leptin and PAI-1 levels and leptin and PAI-1 levels were correlated significantly in both patient groups. The effect of elevated serum leptin and PAI-1 levels on the cardiovascular complications remains to be established.

Does thrombocyte size give us an idea about thrombocytosis etiology?

In the presence of a pathogenetic mutation in JAK2 or MPL, a differential diagnosis of essential thrombocythemia (ET) from reactive causes is relatively simple. However, in patients with suspected ET who lack JAK2 and MPL mutations, the exclusion of secondary causes is especially important. The study was aimed to explore the clinical application of particularly mean platelet volume (MPV), hemoglobin, red blood cell indices, white blood cell, serum iron profile, and C-reactive protein level in the differential diagnosis of thrombocytosis. Medical records of 49 patients, consisting of reactive thrombocytosis (RT) and ET were retrospectively reviewed. The mean MPV level in RT group was 7.49 fL, and in ET group was 8.80 fL (P < 0.01). A cutoff point of <8.33 fL was found to have significant predictive value according to ROC curve analysis. This cutoff was associated with 83% positive predictive value (PPV) and 74% negative predictive value (NPV) in the diagnosis of ET and had a sensitivity of 65% and specificity of 89% for ET. Investigation of MPV is cheap, quick, and noninvasive, and may serve as a predictor of primary thrombocytosis. High sensitivity, specificity, PPV, and NPV enable this test an important tool and a possible surrogate marker in clinical practice.

Clinical and hematologic findings in Noonan syndrome patients with PTPN11 gene mutations.

Reports on Noonan syndrome (NS) have documented multiple types of coagulation defects and bleeding diathesis, and a wide range of clinical presentations. Early studies suggested that a large proportion of NS patients have coagulation defects, whereas more recent reports indicate low rates of coagulopathy. The aim of this study was to evaluate phenotypic characteristics, PTPN11 gene mutations, and hematological and coagulation parameters in 30 clinically diagnosed cases of NS. One of the NS patients had a history of easy bruising; however, his hematological and coagulation tests were normal. None of the other patients had clinical coagulation problems. In the NS group, values for platelet count, activity of factors XI, XII, and protein C were significantly lower than the corresponding means for the control group. However, the results of coagulation tests in the NS group were diagnostically inconclusive and only one patient had clinical signs of coagulopathy. Interestingly, two NS patients had low protein C activity. One of these children had an A1517C mutation and transient myelodysplasia. The other patient had a C1528G mutation in exon 13 that has not been reported previously. Neither of these individuals experienced a thrombotic event or any complication during approximately 3 years of follow-up. For all patients clinically diagnosed with NS, a thorough history of coagulation issues should be taken and first-line coagulation testing should be done to evaluate for bleeding diathesis. However, if these assessments reveal nothing abnormal, complications related to coagulation are unlikely and extensive testing is unnecessary.

Association of serum adiponectin levels and coronary flow reserve in women with normal coronary angiography.

BACKGROUND: Women may have atypical clinical presentations and atypical risk factors of coronary artery disease. Adiponectin has anti-insulin-resistant properties and antiatherogenic effects. We investigated the association between serum adiponectin levels and coronary flow reserve (CFR) in women with normal coronary arteries. METHODS: CFR was assessed in 45 consecutive women (mean age 54.2+/-9.2 years) with normal epicardial coronary arteries by coronary angiography. Serum adiponectin, C-reactive protein, insulin, and glucose levels were examined and Homeostasis Model Assessment for Insulin Resistance index was calculated. Peak diastolic coronary flow velocities were measured in distal left anterior descending artery at baseline and after dipyridamole infusion by transthoracic pulsed wave Doppler echocardiography. CFR was calculated as the ratio of hyperemic to baseline peak diastolic velocities. A CFR value > or =2 was accepted as normal. RESULTS: Adiponectin levels were lower in patients with impaired CFR than those with normal CFR (7.1+/-2.3 vs. 13.8+/-6.7 microg/ml P<0.001). Adiponectin levels were correlated with CFR (r=0.531, P<0.001) and inversely correlated with C-reactive protein (r=-0.308, P=0.047), insulin (r=-0.426, P=0.008), and Homeostasis Model Assessment for Insulin Resistance index (r=-0.442, P=0.004). Adiponectin levels of < or =8.5 muU/ml had 83% sensitivity and 93% specificity [receiver operating characteristic area 0.084, P<0.001, 95% confidence interval (0.56-1.08)] for predicting impaired CFR. CONCLUSION: Decreased adiponectin levels are associated with impaired CFR in women with normal epicardial coronary arteries and hypoadiponectinemia may be a risk factor for impaired CFR in women.

Food allergy after liver transplantation in children: a prospective study.

Food allergy has been increasingly reported in children who had orthotopic liver transplantation (OLT). We aimed to conduct a prospective study to investigate the prevalence of sensitizations and food allergy in pediatric OLT recipients. We also aimed to identify potential risk factors. The study group consisted of 28 children (14 male, 14 female, mean age 4.96 +/- 0.76 yrs) who had OLT. Total eosinophil count (TEC), total IgE, and specific IgEs were studied before and 3, 6, 12 months after OLT. Six patients (21%) developed multiple food allergies. Mean age of six patients at OLT who developed food allergy was younger compared to the non-food allergy group (10.2 months vs. 68.9 months, p < 0.05). Food allergy has been developed within 1 yr in 5, and in 20 months in one patient after OLT. All six patients had cow's milk and egg allergy after OLT. Five children developed wheat, one children developed lentil and another one developed peach allergy in addition to cow's milk and egg allergy. Out of six food-allergic patients after OLT, four children developed Epstein-Barr virus (EBV) infection prior to food allergy. Before OLT, TECs and total IgE levels were not differed among food allergic and non-food allergic patients (p > 0.05). Mean of TECs were significantly higher in food allergic group compared to non-food allergic group at each time point after OLT (p < 0.05). Though statistically insignificant, mean of total IgE levels were also higher in the food allergic group (p > 0.05). These findings suggest that food allergy should be considered after OLT in patients who are younger than 1 yr of age, who developed hypereosinophilia, high total IgE levels or EBV viremia.

Platelet Membrane Γ-Glutamyl Transferase-Specific Activity and the Clinical Course of Acute Coronary Syndrome.

γ-Glutamyl transferase (GGT) participates in oxidative and inflammatory reactions inside the atheroma plaque and platelets. We evaluated whether platelet membrane γ-glutamyl transferase (Plt-GGT) activity is a predictor of major adverse cardiac events (MACEs) during 3 months follow-up of patients with acute coronary syndrome (ACS; MACE-3M). We included 105 patients who were hospitalized consecutively with the diagnosis of ACS. Patients with an MACE-3M were older, more likely to have hypertension, hyperlipidemia, family history of coronary artery disease(CAD), thrombolysis in myocardial infarction (TIMI) risk score >4, higher Plt-GGT and serum GGT activities, serum C-reactive protein level, and lower left ventricular ejection fraction (LVEF) when compared to those without MACE-3M (all P values ≤.05). By receiver-operator characteristic (ROC) curve analysis, 265 mU/mg for Plt-GGT, 30 U/L for serum GGT, and 45% for LVEF were determined as cutoff values to discriminate MACEs. Platelet GGT activity >265 mU/mg, TIMI risk score >4, and family history of CAD were independent predictors of MACE-3M (all P values <.05). Platelet GGT activity was as an independent predictor for MACEs in patients with ACS during the 3 months follow-up.

Congenital Hypothyroidism and Bone Remodeling Cycle.

OBJECTIVE: The present study aimed to evaluate the biochemical markers of bone turnover in children with congenital hypothyroidism during the course of treatment as compared to healthy children selected as controls. METHODS: The study included 31 children with congenital hypothyroidism and 29 healthy children. In both groups, we evaluated serum procollagen type-1 N-terminal propeptide (PINP) and tartrate-resistant acid phosphatase type 5b isoform (TRACP 5b) levels as bone turnover markers. RESULTS: In both groups, thyroid hormone levels were within normal limits. The levels of vitamin D were significantly higher in the cases with congenital hypothyroidism. Although PINP levels were not found to be different, TRACP 5b levels which are related to osteoclastic activities were significantly higher in the control group. CONCLUSION: We did not detect an increase in bone resorption in patients with congenital hypothyroidism, despite long-term treatment with LT4. Our results suggest that with effective vitamin D treatment and thyroxin replacement, congenital hypothyroidism is not a deleterious factor for bone turnover.

Hyperleptinemia and its relation with peripheral C34(+)CD7(+) stem cells in renal transplant recipients.

OBJECTIVES: Leptin, the Ob gene product, centrally regulates weight control. Transplant recipients are exposed to many factors affecting body mass. Leptin has been reported to activate the peripheral immune system. In this study, we evaluated serum leptin levels and factors contributing to hyperleptinemia; the relationship between serum leptin levels and lymphoid stem and mature cells; and their role in the rejection process in renal transplant recipients. MATERIALS AND METHODS: Sixty-three renal transplant recipients were included in the study. Patients were grouped according to serum leptin percentiles as hypoleptinemic (n=17), normoleptinemic (n=32), and hyperleptinemic (n=14). We determined serum leptin levels by radioimmunoassay and absolute number of CD34(+), CD7(+), CD34(+)CD7(+) lymphoid stem cells, CD4(+) and CD8(+) lymphocytes in peripheral blood by flow cytometry. RESULTS: The hyperleptinemic patients constituted 22.3% of the transplant patient. The mean peripheral blood CD34(+)CD7(+) lymphocyte count was significantly higher in hyperleptinemic patients than in normo- or hypoleptinemic patients (6.9, 6.1, and 44.3 cells/mm(3), respectively, P<0.05). There were no significant differences in the mean CD34(+), CD7(+), CD8(+), and CD4(+) lymphocyte count and CD4/CD8 ratio among the groups with respect to serum leptin levels. CD34(+)CD7(+) lymphocyte count was positively correlated with serum leptin levels (r=0.416, P<0.05). CONCLUSIONS: Hyperleptinemia is not rare during the posttransplant period. Our data support the results of previous experimental studies that have demonstrated the effect of the leptin hormone on lymphoid stem cells. The central and peripheral effects of leptin may differ on lymphoid stem cells and a serum threshold level may apply for the central effects.

Buffered papaverine facilitates passage of intratympanic dexamethasone to the inner ear.

CONCLUSION: We conclude that when papaverine is intratympanically administered together with dexamethasone after buffering, passage of dexamethasone to the inner ear is increased. OBJECTIVE: To determine the influences of papaverine and buffered papaverine on passage of dexamethasone administered intratympanically to the inner ear. MATERIALS AND METHODS: Twenty-seven Vienna white rabbits were divided into five groups: eight received intratympanic dexamethasone (4 mg/ml) (group 1), seven received intratympanic papaverine (10 mg/ml) + dexamethasone (2 mg/ml) (group 2), six received buffered (with sodium bicarbonate 8.4%) intratympanic papaverine (7.5 mg/ml) + dexamethasone (1.5 mg/ml) (group 3), three had basal cortisol levels in venous blood and perilymph measured (group 4), and three received intravenous papaverine + dexamethasone (group 5). At 1 h after the administration of the drugs, dexamethasone levels in ipsilateral and contralateral perilymph and venous blood were measured by radioimmunoassay. RESULTS: Animals in group 3 demonstrated the highest levels of ipsilateral perilymph dexamethasone. Ipsilateral perilymph levels were significantly higher in groups 1 and 3 than they were in the other groups (p<0.05). Although the perilymph levels observed in animals in group 2 were slightly higher than those in group 4, no significant difference existed (p = 0.160).

Serum neuron-specific enolase levels in preterm and term newborns and in infants 1-3 months of age.

BACKGROUND: Elevated serum levels of neuron-specific enolase (NSE) was initially assumed to be specific to neuronal tumors (particularly neuroblastoma), but is now known to accompany nontumoral conditions and tumors other than neuroblastomas. There is a need to establish normal ranges for NSE, especially in early infancy. The aims of this study were to determine reference values for NSE in newborns and young infants and to assess whether NSE levels in early infancy (i.e., preterm infants and term infants) differ from the adult reference range for this enzyme. METHODS: We enrolled 140 healthy babies, which included 40 preterm newborns (3-15 days old and born at 28-42 weeks gestation), 40 term newborns (< 1 month old and born at term), and 60 young infants 1-3 months old (n = 20 per subgroup of 1-, 2-, and 3-month-old infants). The determination of NSE levels was performed by the electrochemiluminescence immunoassay (ECLIA) method using the Elecysys 2010 device (Roche Diagnostics, Mannheim, Germany). The mean serum NSE levels for the preterm newborns was 21.83 ± 15.06 ng/mL [95% confidence interval (95%CI), 16.95-26.71 ng/mL]; term newborns, 18.06 ± 12.83 ng/mL (95%CI, 13.94-22.19 ng/mL); and young infants, 9.09 ± 4.38 ng/mL (95%CI, 7.96-10.23 ng/mL). The mean serum NSE level for infants 1-3 months old was within the ECLIA kit's normal range (4.7-18 ng/mL for adults), whereas the corresponding means for the preterm and term newborns were higher (p < 0.001, for both). CONCLUSION: Our findings suggest that adult reference values should not be applied to the preterm and term age groups.

Serum S100B levels in children with simple febrile seizures.

PURPOSE: Recent studies have found that S100B is a useful marker for astroglial activation seen in various neurologic disorders. The purpose of this study was to evaluate whether simple febrile seizures (SFS) was associated with an elevation in serum S100B levels. METHODS: In this study the samples consisted of 39 patients with SFS ranging from 6 to 36 months of age, and age-matched and sex-matched controls including 30 patients with fever and 30 healthy subjects. Two serum samples were obtained for S100B from the study group at 0-1h and 6-24h following seizure. Serum samples were drawn once in the control group. The serum samples were then analyzed using ELISA. RESULTS: In the study group, the mean values of the serum S100B concentrations at 0-1h and 6-24h were 32.6±7.8pg/ml and 32.1±5.8pg/ml, respectively, while the concentrations were 32.1±8.8pg/ml and 29.5±7.8pg/ml in the control groups. No significant differences were detected in serum S100B levels at 0-1h or 6-24h in the study when compared to the control groups. CONCLUSIONS: These results suggest that SFS do not raise serum S100B concentration above the normal range.

Synovial apoptosis in temporomandibular joint disc displacement without reduction.

OBJECTIVE: Our hypothesis is that increased apoptosis in synovium might contribute to temporomandibular joint (TMJ) degeneration. To investigate this, we measured soluble Fas (sFas) and nuclear matrix protein (NMP) levels in TMJ synovial fluid from patients with disc displacement without reduction as indicators of apoptosis in the synovium. PATIENTS AND METHODS: Synovial fluid was obtained from 17 joints in 17 patients (11 female, 6 male; mean age, 31.5 +/- 11.9 years; range, 19 to 55). Patients were referred to our clinic because of limited mouth opening, joint sounds, or TMJ pain. Synovial fluid obtained by arthrocentesis for therapeutic reasons was analyzed by enzyme-linked immunosorbent assays for APO-1/Fas and cell death detection (NMP). RESULTS: We studied 12 left (71%) and 5 right (29%) joints with disc displacement without reduction. The chief complaint was pain on the affected side and limited mouth opening. Only 2 patients had a click in the affected joint, whereas 14 reported pain and 17 had the limited mouth opening. All patients experienced a significant (P < .01) increase in maximal mouth opening immediately after arthrocentesis. Mean sFas and NMP levels were 484.9 +/- 466.7 pg/mL (range, 17 to 1501) and 29.2 +/- 13.7 U/mL (range, 8 to 52.8) respectively. CONCLUSION: Considering reports that increased sFas blocks apoptosis by inhibiting binding of FasL to Fas on the cell membrane, low level of sFas in our patients' synovial fluid (compared with amounts reported in joint inflammation or degeneration) suggests vulnerability to apoptosis in patients with internal derangement.

Relation between epicardial fat thickness and coronary flow reserve in women with chest pain and angiographically normal coronary arteries.

OBJECTIVE: A significant proportion of women with angina-like chest pain and angiographically normal coronary arteries have microvascular dysfunction as detected by reduced coronary blood flow reserve (CFR). Classical clinical risk factors of atherosclerosis poorly predict this scenario. We sought to assess whether increased epicardial fat tissue, which is a metabolically active organ, could be associated with impaired CFR in these patients. METHODS: We enrolled 68 women who underwent coronary angiography and had no obstructive coronary artery disease. Data about classical risk factors, insulin resistance and serum levels of C-reactive protein (CRP) and adiponectin were obtained. Stress tests were evaluated. Coronary flow velocities at baseline and under-induced hyperemia and epicardial fat thickness (EFT) were measured by transthoracic echocardiography within 48 h of angiography. CFR >or=2.0 was considered normal. RESULTS: Forty percent of women had reduced CFR suggestive of microvascular dysfunction and 60% had normal CFR. Menopause, hypertension and abnormal stress tests were significantly more prevalent, adiponectin level was significantly decreased, CRP, insulin resistance, and EFT were significantly increased in women with microvascular dysfunction as compared with those without. On multivariate regression analysis EFT emerged as the only independent predictor of microvascular dysfunction (P<0.0001). EFT of >0.45 cm had 85% sensitivity and 75% specificity to detect CFR <2 (P<0.0001). Traditional risk factors for atherosclerosis did not predict women with abnormal microvascular function. CONCLUSIONS: EFT has the potential to be an additional and easy diagnostic tool for risk stratification of women with chest pain and angiographically normal coronary arteries.

Weight loss and hypernatremia in breast-fed babies: frequency in neonates with non-hemolytic jaundice.

OBJECTIVE: The aim of this study was to determine what proportion of newborns admitted with idiopathic non-hemolytic hyperbilirubinemia exhibit severe weight loss and hypernatremia. METHODS: The prospective study involved 115 infants >48 h old who were admitted with jaundice between July 2002 and July 2003, and had unconjugated bilirubin levels >12 mg/dL. Premature babies (gestational age <37 weeks) and those with hemolytic jaundice and other pathologic causes of non-hemolytic jaundice were excluded. Postnatal age (days) at admission, bodyweight at admission, weight change since birth (percentage weight loss calculated at admission) and mode of feeding (breast-feeding, formula feeding, mixed feeding) were recorded. Severe weight loss was defined in babies who showed >10% weight loss or had not regained enough to reach birthweight by postnatal day 10. Serum Na levels and breast-milk Na levels were also measured. RESULTS: Twenty-eight (33%) of the 86 newborns with idiopathic hyperbilirubinemia in the study exhibited severe weight loss. Almost all the 86 babies were exclusively breast-fed, and 10 babies (12%) had severe weight loss combined with hypernatremia. The group with severe weight loss and hypernatremia had higher breast-milk Na levels than the other infants. CONCLUSION: The results indicate that a large proportion of babies with non-hemolytic jaundice have severe weight loss, and that breast-fed newborns with the combination of weight loss and hypernatremia may present with non-hemolytic jaundice.

Correlations among serum leptin levels, complete blood count parameters and peripheral CD34(+) cell count in prepubertal obese children.

Leptin is a hormone produced by adipocytes that helps reduce body weight by depressing appetite and increasing metabolic activity. Leptin also promotes early hematopoiesis. The main aim of this study was to compare complete blood count (CBC) parameters and peripheral blood CD34(+) cell counts in prepubertal obese and nonobese children. Relationships between leptin levels and CBC parameters and peripheral CD34(+) progenitor cell counts in the obese group were also investigated. Thirty one healthy, prepubertal, obese children and 30 nonobese, age-matched prepubertal controls were included in the study. A fasting blood sample was collected from each subject, and CBC findings, serum leptin level, and peripheral blood CD34(+) progenitor cell count were recorded. In the obese group, the mean results for body mass index (BMI), BMI standard deviation score (BMI SDS), and serum leptin level were significantly higher than the corresponding control findings. There were no significant differences between the groups with respect to CBC parameters and CD34(+) cell percentage. In both the obese and control groups, the girls' serum leptin levels were significantly higher than the boys'. In the obese group, serum leptin level was strongly correlated with BMI and with BMI SDS (Pearson correlation coefficients r=0.70, p<0.001, and r=0.59, p<0.001, respectively) in both girls and boys. None of the CBC parameters nor CD34(+) progenitor cell percentage was correlated with leptin, BMI, or BMI SDS. The results indicate that serum leptin levels in obese children are positively correlated with BMI. However, in contrast to adults, high leptin level in childhood obesity does not seem to be associated with altered CBC parameters or increased peripheral CD34(+) progenitor cell count.