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Objective: It has been theorized that changes in the lower extremity mechanics may lead to develop Patellofemoral Pain (PFP) in the young population. The present study aims to investigate the effects of lower extremity mechanics on Patellofemoral Pain Syndrome in private university students. Material and Methods: Kujala Pain Score (KPS) of 400 Yeditepe University students ages of 18-30 years was performed. Students having a less or equal point of 85 in KPS (n=30) and healthy groups randomly selected in students with KPS=100 (n=30) were measured in terms of Feiss Line, navicular drop, subtalar angle, tibial torsion, knee valgus angle, Q angle in standing and supine position and hamstring tightness. Results: The prevalence of PFP among students was found to be 10.5%(n = 42). In case and control groups were found 2nd Pes Planus (PP) as 16 (%53,3) and 3 (%10) respectively. A statistically significant difference was found between groups in navicular drop, subtalar angle, tibial rotation, Q angle in supine and hamstring tightness (p-value

Introduction We performed a comparison of cell blocks prepared with the collodion bag and HistoGel to evaluate the ease of embedding and cutting, performance with low cellularity specimens, time and cost per specimen, and value to support immunohistochemistry and molecular diagnostics. Materials and methods We processed 11 fresh, unfixed effusions using both the collodion bag and the HistoGel cell block preparation methods. Six immunohistochemistry stains were tested on 2 of the body fluids. DNA was extracted and quantified, and polymerase chain reaction cycle thresholds were evaluated from cell blocks prepared from 5 of the body fluids. The comparison parameters included embedding difficulty, cutting resistance, adequacy, cell yield, cell preservation, immunohistochemistry stain quality, DNA quantity, integrity, and purity. The time and cost to prepare each specimen was compared using normalized values for preparation of specimen, cost per year, and cost per specimen. Results Each parameter was assessed for both cell block preparation methods. All 3 of the samples with moderate or poor cell yield were low-volume (5-mL) samples prepared with the HistoGel method. In contrast, the collodion bag method produced a good yield with all three 5-mL samples. DNA recovery was greater in the collodion bag method. Similar crossing threshold values in purity reactions indicated equally high-quality matrix properties for the collodion bag and HistoGel preparations. Preparation of the specimen was 10 minutes faster with the collodion bag method, and the cost for the collodion bag method was $0.24 more expensive per cell block than using the HistoGel. Conclusions The collodion bag method produced superior cell blocks for both morphologic and molecular studies more consistently, with lower volume specimens and with less time per specimen.

Hemoglobin D-Los Angeles, a recessively inherited hemoglobin variant, has been introduced as hematologically silent in the heterozygous state. However, as its compound heterozygosity with other hemoglobinopathies may lead to a severe clinical phenotype, with hemoglobin S being the most serious, the detection of carriers is important. To clarify the hematologic picture, we assessed erythrocyte parameters in D carriers and compared values in healthy controls and beta-thalassemia carriers. Although values in D carriers, in the absence of confounding factors, significantly differed from thalassemia carriers (P

This paper presents a method to estimate the cutting forces and vibrations at the milling tool from accelerometers mounted on the spindle housing. The frequency response functions between the spindle nose and accelerometer locations are identified once with impact modal tests. Cutting tool and holder is assembled with the speed and preload dependent spindle structure dynamics using receptance coupling. The distortion of signal transmission from tool to the accelerometer location is compensated with a Kalman filter to estimate the forces and displacements at the tool tip. The proposed method is experimentally demonstrated in milling tests. The cutting force and displacement estimations were within 80% of the directly measured values.

This paper proposes a model to keep the cutting load induced tool tip position and tool orientation errors of five-axis CNC machines within the desired tolerance limit by scheduling the feed along the tool paths. The cutting forces at the tool tip are transmitted to three translational and two rotary servo drives of the machine tool using its kinematic model. Tracking errors of each axis contributed by cutting forces are modeled using the disturbance transfer function of servo drives. The tracking errors are mapped to the workpiece coordinate system to evaluate the tool tip position and tool axis orientation errors at discrete tool path locations. The cutting forces, therefore the tracking errors, are modeled as a linear function of tangential feed velocity. The feed is adjusted to constrain the tool tip position and tool axis orientation errors at the desired tolerance levels along the tool path. The proposed algorithm has been experimentally validated by milling a curved tool path on a five-axis commercial CNC machine tool. It is shown that the proposed algorithm can significantly improve the part machining accuracy without increasing the machining time.

This paper presents an on-line chatter detection method in milling using drive motor current commands supplied by the CNC system in real-time. The methodology is described by using the spindle drive motor current command although it has been applied to the feed drives as well which is demonstrated in the results section. The transfer function of spindle velocity controller is constructed by reading the control law parameters and measuring the Frequency Response Function (FRF) of the system automatically using an external computer communicating with the CNC in real time. By subtracting the rigid body based FRF from the measured FRF of the velocity controller that includes the flexibilities, the structural modes of the spindle drive are identified. The closed loop transfer function between the cutting torque at the tool and corresponding noise free digital current commanded by the CNC is formed. The effects of structural dynamic modes of the spindle are compensated via a proposed observer. The bandwidth of the compensated FRF of the current command over cutting torque disturbance has been increased to 2.5 kHz with 10 kHz communication speed limit of the CNC with external PC. After removing the forced vibration components, the frequency and presence of chatter are detected from the Fourier Spectrum of the current commands supplied by CNC in real time. The proposed system is experimentally validated in milling tests.

Five-axis CNC machine tools are widely used in machining parts with free form surfaces. This paper presents optimal placement of parts on the five-axis machine tool tables to minimize the tracking errors of the rotary servo drives. The cutting forces along the tool path are first simulated at the workpiece coordinate system in Computer-Aided-Manufacture (CAM) environment. The cutting torques transmitted to the rotary and translational drives are predicted using the location of the part on the table and kinematic configuration of the machine tool. The optimal location of the part on the table is identified by minimizing the forces transmitted to the rotary drives as torque disturbances. The proposed model has been experimentally validated on a five-axis machine with tilt-table configuration. It has been shown that the tracking and contouring errors can be significantly reduced with the proposed strategy, which can be used by process planners in digital simulation environment.

Background: Klebsiella pneumoniae is an important nosocomial pathogen that can lead to high morbidity and mortality. ESBL and carbapenamase producing strains may cause epidemic situations. The aim of our study was to investigate the molecular epidemiology and clonal relationship between carbapenem resistant K. pneumoniae strains isolated from our hospital during a three month period. Methods: Fourteen carbapenem resistant K. pneumoniae strains isolated during April 1st–June 30th 2013 were included. The identification and the antibiotic susceptibility of the strains were studied by Vitek 2 Compact (Biomerieux, France) system. The carbapenemase production of the isolates were investigated by Modified Hodge assay. The blaOXA of the strains was investigated by in house PCR. The clonal relationship between the isolates were studied by pulsed-field gel electrophoresis (PFGE) and automatized repetitive extragenic palindromic PCR (Rep-PCR, DiversiLab sistemi, Biomerieux, France) methods. Results: All the K. pneumoniae isolates were carbapenem resistant; they were all susceptible to gentamycin and colistin. All of them had blaOXA-48. The genotyping analysis revealed that eight isolates were in the same cluster both by Rep-PCR (similarity border ≥95%) and PFGE (Tennover criteriae) analysis. The other isolates did not belong to any other clusters. The strains that are in the same cluster are isolated from the Anesthesiology Intensive Care Unit during a three month period. The cluster ration by both methods was 57%. Conclusions: All K. pneumoniae strains possessed blaOXA-48. The clonal spreading was particularly detected in Anesthesiology Intensive Care Unit. Molecular epidemiological monitorization of nosocomial pathogens may prevent the spread of these multidrug resistant pathogens. Keywords: Carbapenem resistant K. pneumoniae, Molecular epidemiology, blaOXA-48

Hyperferritinemia-cataract syndrome, characterized by high serum ferritin concentration and cataracts in early life, remains a less-known rare disease, with fewer than 100 families reported worldwide. Though benign, high ferritin levels frequently result in misdiagnosis with iron storage disease, and patients can be exposed to unnecessary, even invasive, evaluation and treatment procedures. The presence of cataract together with isolated serum ferritin elevation should alert clinicians to consider this syndrome. We herein present a new family with hyperferritinemia-cataract syndrome to increase clinical awareness.

This paper presents a virtually assisted on-line milling process control and monitoring system. A part machining process is simulated to predict the cutting forces, torque, power, chip load and other process states. The simulated machining states are accessed by a real time monitoring system which detects the tool failure and adaptively adjusts the feed by predicting the forces from the feed and spindle drive motor current supplied by CNC. The integration of virtual simulation with real time measurements avoids false tool failure detection and transient overloads of the tools during adaptive control. The system has been implemented on a CNC machining centre for use in production.

We investigated the feasibility of using serum alpha-fetoprotein (AFP) levels as a screening test for prenatal diagnosis of Fanconi anemia (FA). Serial measurements in maternal serum were recorded. Parents, both heterozygous for FA, had declined prenatal molecular testing. The infant was born with no somatic abnormalities, and FA was confirmed by postnatal molecular analysis. Maternal serum AFP levels during each trimester of pregnancy were normal indicating that these levels cannot be used as a screening test in prenatal diagnosis. Three-year follow-up after birth showed constantly elevated serum levels in the patient from the start, suggesting a lack of postnatal inhibition on AFP gene.

Introduction The impact of uterine fibroids on fertility and the beneficial role of surgery is controversial. Current data suggest that submucosal and intramural fibroids that distort the endometrial cavity decrease pregnancy and implantation rates. However, the impact of intramural fibroids without intracavitary component is unclear. The aim of this study is to clarify the impact of the uterine myomas and myomectomy in patients undergoing assisted reproductive techniques (ART). Methods Retrospective cohort study performed in Instituto Universitario Dexeus including 1072 cycles of IVF performed in infertile patients: 473 in patients with uterine myomas not treated before ART, 79 in patients undergoing ART after myomectomy and 520 in patients included as a control group. Results Our results show that there is a significant 64% reduction in the clinical pregnancy rate in women with cavity-distorting intramural fibroids, a 51% significant reduction in the clinical pregnancy rate and a 53% significant reduction in the delivery rate in patients with non-distorting intramural fibroids. Conclusions Our study supports the opinion that non-cavity distorting intramural fibroids have a detrimental effect on live births, clinical pregnancy, implantation and delivery rates in patients undergoing ART.

This research was originally published in Blood Online. Tummala, H., et al. (2018). "Homozygous OB-fold variants in telomere protein TPP1 are associated with dyskeratosis congenita–like phenotypes." Blood 132(12): 1349-1353. Title. Blood. Prepublished Sept 20 2018; DOI https://doi.org/10.1182/blood-2018-03-837799

Congenital hypotransferrinemia (OMIM 209300) is an extremely rare disorder of inherited iron metabolism. Since its description in 1961, only 16 cases have been reported. The defective gene and molecular defect causing this disorder and clinicolaboratory findings seen in the homozygous and heterozygous states have been documented in both humans and mice. However, due to the lack of follow-up studies of the described cases, the long-term prognosis remains unknown. We present a 10-year observational follow-up of a patient previously diagnosed on a molecular basis who was treated with a unique therapy of plasma transfusion fortified with oral iron, with satisfactory clinicolaboratory responses.

Inherited giant platelet disorders are a subgroup of congenital thrombocytopenias characterized by decreased platelet counts along with macroplatelets and variable bleeding symptoms. Harris platelet syndrome, a newly described rare entity, is a subtype of inherited giant platelet disorders and is characterized by mild-to-severe thrombocytopenia, macroplatelets, and no bleeding manifestations. This entity was observed incidentally in healthy blood donors from India in the early 2000s, and the reported cases to date have without exception originated from the same region of the Indian subcontinent. We herein report the occurrence of Harris platelet syndrome in patients from a different ethnogeographic origin.

We describe a Turkish boy newly diagnosed with Fanconi anemia with mutation in the FANCA gene. The patient, with normal clinical phenotype and negative chromosomal breakage test result, presented with macrocytosis. No clinical or laboratory changes were observed in a follow-up period of 4 years. The diagnosis was confirmed molecularly after a prolonged and exhaustive investigation. He was found to be a compound heterozygote for 2 mutations in the FANCA gene (1 of which is novel, c. 4261-2A > C). We present this experience to alert physicians that Fanconi anemia should be considered in the differential diagnosis of otherwise unexplained macrocytosis during childhood.

Klebsiella pneumoniae is an important nosocomial pathogen that can lead to high morbidity and mortality. ESBL and carbapenamase producing strains may cause epidemic situations. The aim of our study was to investigate the molecular epidemiology and clonal relationship between carbapenem resistant K. pneumoniae strains isolated from our hospital during a three month period.Fourteen carbapenem resistant K. pneumoniae strains isolated during April 1st-June 30th 2013 were included. The identification and the antibiotic susceptibility of the strains were studied by Vitek 2 Compact (Biomerieux, France) system. The carbapenemase production of the isolates were investigated by Modified Hodge assay. The blaAll the K. pneumoniae isolates were carbapenem resistant; they were all susceptible to gentamycin and colistin. All of them had blaAll K. pneumoniae strains possessed bla

One of the most common silent β-thalassemia mutations is the CT substitution at position -101 within the distal CACCC box, which leads to a mild reduction in the expression level of the β-globin gene. Carriers of this mutation have a normal hematologic picture without microcytosis and borderline hemoglobin A2 values, and may be missed during screening. Co-occurrence of this mutation with one of the classical β-thalassemia mutations leads to β-thalassemia intermedia, and this is important for Mediterranean populations where β-thalassemia is frequent. Awareness of this mutation, which may have a heterogeneous clinical presentation, is required. We herein present the unusual hematologic findings of a Turkish family carrying this mutation.

Objective: We aimed to evaluate the recognition level of bladder cancer in the society by conducting a survey with regards to social awareness in early diagnosis of bladder cancer in this study. Materials and Methods: The survey was conducted on 100 randomly selected patients who were admitted to our clinic in May 2016 for any complaints. In the survey, the main focus was hematuria which is the first and the most common symptom of bladder cancer and questions and statements on this subject was used. Results: Of 100 patients, 67 (66.7%) were male, and 33 (33.3%) were female. Thirty six of the patients were younger than 50 (36%), and 64 of them (64%) were 50 years and older. Education level of 40 (40%) patients was found to be university level, and 60 (60%) patients we high school graduates or lower. Twenty seven (27%) patients had complains about blood in the urine, while 67 (67%) of them had no such complaint. Of 27 patients that had complaint about hematuria, which is the most important symptom of bladder cancer 22 (81%) were male and 5 (19%) were female. We divided the patients into two groups based on 50 age limit. Group 1 included patients who were below 50, while the group 2 consisted of patients who were 50 years old and above. The rates of immediate consultation were determined to be significantly higher in group 2 than group 1. The rate of consulting urology department in the presence of hematuria, and the rates of considering the risk of bladder cancer as a possible diagnosis were higher in group 2, but the difference was not statistically significant. There was no significant difference found between the two groups who were separated by age in terms of required diagnostic tests. The patients were divided into two more groups based on their education level. Group 3 included patients of university graduates, and group 4 included patients with high school graduates or lower. The rates of immediate consultation were significantly higher in group 4. In the event of hematuria, there was no significant difference between the two groups in terms of consulting urology department, considering the risk of bladder cancer as a possible diagnosis, or required diagnostic tests. Conclusion: We observed that a certain level of awareness has been formed in the society regarding hematuria on early diagnosis of the bladder cancer; however, there are still some problems managing this issue. The absence of significant difference between the groups based on education level points out the significance of further actions to be taken to raise social awareness in whole community for this matter.

Oculoectodermal syndrome (OMIM 600268) is rare and characterized by aplasia cutis congenita, epibulbar dermoids, and other abnormalities. We report herein on a newly recognized patient with oculoectodermal syndrome, which is the 19th reported patient with OES. The boy aged six years demonstrated a broad clinical spectrum of this condition, including aplasia cutis congenita, epibulbar dermoids, hyperkeratotic papule, mildly enlarged cisterna magna, and an enlarged fluid space in the quadrigeminal cistern, suggesting a cyst. He also manifested anomalies not reported associated with this disorder, including systematized epidermal nevus following Blaschko's lines, hypopigmented skin lesions, and mild digital anomaly. (c) 2014 Wiley Periodicals, Inc.

This paper reports the findings of a study evaluating demographic and offence characteristics of 230 offenders who had been convicted either of possessing indecent images (Internet offenders n = 74) or committing actual direct abuse of children (contact offenders n = 118) or committing both offences (Internet-contact offenders n = 38). All data were collected in the Greater London Offender Management Service. The group of Internet-contact offenders exhibited more diverse characteristics than the Internet and contact sex offenders and they were more likely to have been accused of a prior contact offence without being charged. The majority of the Internet offenders were white male, younger than the contact child sex offenders, more likely to be single and to have never been married, were better educated and more likely to have stable employment. They were also less likely to have had previous convictions. Contact offenders were more likely to report childhood difficulties including sexual abuse; there were ...

Vitiligo-like lesions, although rare, are believed to be a prognostic factor in malignant melanoma. While a predictive role for such lesions was shown with immunomodulatory therapies, this relation was not demonstrated with temozolomide. We present 3 patients with metastatic malignant melanoma who developed vitiligo-like skin lesions accompanying good response to treatment and prolonged survival. Onset of vitiligo-like lesions with temozolomide in metastatic malignant melanoma may predict long-term response for this treatment.

Grinding process is generally used to improve the tolerance integrity and surface quality of a workpiece. However, in case of some hard-to-machine materials grinding can also be a cost effective alternative even for roughing operations. It is crucial to know process forces since they are necessary to identify the conditions for surface burn which is one of the most important issues in grinding applications. In this paper, a new semi-analytical force model for grinding process is developed by modeling abrasive grits and their interaction with the workpiece individually. Grits are examined to determine their geometrical properties and distribution on the grinding wheel. Semi-analytical equations for total normal and tangential force components as well as average force per grit are established by using the micro milling analogy. Fundamental parameters such as shear stress and friction coefficient between the grits and the work material are identified. The model can then be used in prediction of the forces for different cases involving the same material and the abrasive grain however with different conditions. The model predictions are verified by several experiments and also using Johnson-Cook material model.

The aim of this study is to present the limbal stem cell deficiency (LSCD) cases with features resembling dyskeratosis congenita (DC), a heritable disease of stem cells principally caused by telomerase deficiency. The clinical, laboratory and molecular findings of four cases are presented. A complete systemic examination was performed in a standardized manner for each patient. Laboratory measurements included investigations of the tests used for screening DC. All eight known disease-causing genes in DC (DKC1, TERC, TERT, NOP10, NHP2, TINF2, C16orf57, and TCAB1) were screened for mutations. The family members of the cases were also assessed, when possible. In all four patients, multisystem involvement was present, along with the disorder affecting corneal LSCs. The affected tissues were mainly the skin and its adnexa, the oral cavity and the hematopoietic system, which are rapidly renewing tissues, consistent with the presence of a stem cell disorder. Similarly affected cases were seen in different generations in families, suggesting an underlying inherited disorder. No mutation was detected in any of the known disease-causing genes in these patients. Based on the presented cases and with the contribution of the review of previously reported DC cases available, we suggest that DC is one of the inherited causes of LSCD and that those cases presenting with LSCD might represent a subgroup of DC caused by mutations in an as yet undefined gene.

Understanding why indecent images of children are produced and collected involves issues beyond the legal definition of child pornography. An over-emphasis on a legal approach will not necessarily assist in the development of preventative and control strategies. This article provides a brief overview of the way in which the Internet can be misused for sexual gratification and has identified some of the issues that seem to be significant in understanding the nature and dimensions of Internet sexual offending. The main descriptive typologies that underpin research relating to Internet child sex offenders have been reviewed and critically evaluated. As discussed throughout this article, one of the most challenging issues continues to be the difficulty in placing Internet sex offenders into categories. The foregoing discussion indicates that Internet sex offenders comprise a heterogeneous population and that there appears to be a lack of a coherent and agreed framework for defining their activity.

We report herein on our observation of recessive congenital methemoglobinemia (type I), an autosomal recessive disorder, in immediate generations (in a mother and her daughter). Molecular analysis revealed a mechanism of inheritance not reported previously, despite the high probability of occurrence in autosomal recessive disorders. This report is also the first publication describing an extremely rare mutation (Arg50Gln) causing this disorder in the Turkish population.

Hereditary atransferrinemia is a very rare disorder characterized by microcytic anemia and iron overload. It has been reported in only 10 patients in 8 families. The molecular basis of atransferrinemia has been determined in only 3 human cases. We now report a new patient with this rare disorder, who is the first known case in Turkey, the 11th patient reported in the published literature and only the 4th case of human atransferrinemia characterized on a molecular basis. DNA analysis of the serum transferrin gene in the patient revealed a previously undescribed mutation in exon 4, a G] A transition at cDNA 410( Cys137Tyr). A number of previously known polymorphisms and a previously undescribed mutation at IVS10(-23)C -> T, presumably a polymorphism, were also documented. Copyright (c) 2007 S. Karger AG, Basel.

A 7-month-old Turkish boy presented with partial albinism and typical clinical features of an accelerated phase, suggesting the diagnosis of Griscelli syndrome. The diagnosis was confirmed by light microscopic evaluation of hair and a peripheral blood smear. Genetic analysis identified a mutation in the Rab27A gene. He was initiated immunosuppressive treatment but accelerated phase could not be ameliorated. He unfortunately died from multiorgan failure. The finding of partial albinism in children should alert clinicians to consider Griscelli syndrome since simple methods can confirm the diagnosis and early diagnosis is life-saving.

This study assessed whether glycosylated hemoglobin could be used as an index to distinguish between iron-deficiency anemia and thalassemia minor. Glycosylated hemoglobin was measured by high- pressure liquid chromatography in 40 beta-thalassemia minor patients, 20 iron-deficiency anemia patients, and 38 healthy controls, all nondiabetic. Median glycosylated hemoglobin was lower in thalassemia minor than in the iron-deficiency and control groups (p =. 000). There was no difference between iron-deficiency patients and healthy controls (p =.095). Glycosylated hemoglobin was not different in iron- replete and iron- deficient traits (p > .05). A cutoff value of 5% has provided a sensitivity of 95% and specificity of 75.7% for distinguishing between these two entities. Positive and negative predictive value were 96.6 and 67.9%. These values were superior to the traditional discriminants' values calculated on the same individuals. Glycosylated hemoglobin could be useful in discriminating between iron- deficiency anemia and thalassemia minor. Further studies are needed, but the point that it can also be used when both conditions exist simultaneously seems to be clinically important.

Human parvovirus B19 (PVB19) infection may cause mild pancytopenia characterized by transient and spontaneous recovery in healthy subjects. Severe aplastic anemia associated with PVB19 infection in patients without an underlying disease has been described in a number of reports. Here, a previous healthy, 10-year-old girl with severe aplastic anemia associated with PVB19 infection is described. The patient underwent bone marrow transplantation from her HLA-identical sibling resulting in complete recovery. PVB19 infection should be considered as one of the causes of aplastic anemia in patients without an underlying disease.

Eight-year event-free survival (EFS) was evaluated in 205 patients with acute lymphoblastic leukemia (ALL), to consider the efficacy of high-dose methylprednisolone (HDMP) given during remission induction chemotherapy between 1 and 29 days. The St Jude Total XI Study protocol was used after some minor modifications in this trial. Patients were randomized into two groups. Group A (n = 108) received conventional dose (60 mg/m(2)/day orally) prednisolone and group B (n = 97) received HDMP (Prednol-L, 900-600 mg/m(2) orally) during remission induction chemotherapy. Complete remission was obtained in 95% of the 205 patients who were followed-up for 11 years; median follow-up was 72 months (range 60-129) and 8-year EFS rate was 60% overall (53% in group A, 66% in group 13). The EFS rate of group B was significantly higher than of group A (P = 0.05). The 8-year EFS rate of groups A and B in the high-risk groups was 39% vs 63% (P = 0.002). When we compared 8-year EFS rate in groups A and B in the high-risk subgroup for both ages together less than or equal to2 or greater than or equal to10 years, it was 44% vs 74%, respectively. Among patients in the high-risk subgroup with a WBC count greater than or equal to50 x 10(9)/l, the 8-year EFS was 38% in group A vs 58% in group B. During the 11-year follow-up period, a total of 64 relapses occurred in 205 patients. In group A relapses were higher (39%) than in group B (23%) (P = 0.05). These results suggest that HDMP during remission-induction chemotherapy improves the EFS rate significantly for high-risk patients in terms of the chances of cure.

Summary Rapid leukemic cell kill at initial diagnosis of patients with acute lymphoblastic leukemia (ALL) has been shown to be associated with a favorable outcome. The aim of the present study was to investigate the effect of high dose methylprednisolone (HDMP) on in vivo blast cell apoptosis in children with ALL. Annexin V-binding and Fas (CD95), Fas ligand (FasL; CD95L), and Bcl-2 expression in PB blasts were determined in newly diagnosed children with ALL before and 4, 24, 96 h after initiation of HDMP treatment (n=20) or conventional dose steroids (CDS) (n=10) as the control group. A decrease in absolute blast count (from 40.8 × 09 to 21.4 × 109/l) associated with an increase in apoptosis (14.2 to 26.9%) (P

The authors have previously demonstrated a favorable effect of high-dose methylprednisolone (HDMP), which can induce differentiation and apoptosis of leukemic cells in children with acute myeloblastic leukemia (AML). Here, they evaluate the effect of short-course HDMP in 2 children with acute myeloblastic leukemia (AML-M2) presented with myeloid tumor (MT). Methylprednisolone (20 or 30 mg/kg/day) was given orally, in a single dose, without using other antileukemic agents. Rapid cytoreduction in MT, peripheral blood, and bone marrow blasts was observed in both children following short-course (4 or 7 days) HDMP treatment, possibly due to HDMP-induced differentiation and apoptosis of leukemic cells. The effects of HDMP should be explored in patients with other subtypes of AML who present with MT.

Recently, measurement of serum alpha-fetoprotein (sAFP) was introduced as a preliminary test for diagnosis of Fanconi's anemia (FA). In the present study, sAFP levels were measured in order to determine its sensitivity and specificity in 33 Turkish FA patients (17 males and 16 females) with a mean age of 11.6 +/- 7.7 (1.0-28.0) (median 10.0). Complementation groups were available in 12 patients. Nineteen age-matched healthy children, 17 patients with bone marrow failure syndromes, 37 FA heterozygotes, and 37 children with acute leukemia served as negative control groups. The sAFP was measured by particle immunoassay. The level of sAFP was found to be higher than the cut-off value, 8 IU/mL in 46% and was within normal limits in 54% of the FA patients. The AFP values were within normal limits in all of the subjects belonging to the control groups. This method provided 46% sensitivity and 100% specificity in the diagnosis of FA. The sAFP values were high in 4 of 17 (24%) FA patients who did not receive any androgen therapy, while the sAFP level was high in 7 of 9 (78%) patients who received such a therapy. The statistical analysis of incidence of a high sAFP level between these two groups indicated a significant difference (P = 0.014), suggesting that androgen therapy might be a contributing factor for elevation of sAFP. The comparison of several clinical and laboratory parameters between FA patients with high and normal levels of AFP revealed no statistically significant differences. The level of sAFP was elevated in only 5 of the 11 patients with complementation group A; in addition, variable levels of sAFP were noted among the affected members in 4 families, indicating that complementation groups, type of mutation, or familial factors were not responsible for elevation of sAFP.