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ABSTRACT: INTRODUCTION: Being born large for gestational age (LGA) is a risk factor for development of metabolic syndrome (MS) in adolescents and adults. OBJECTIVE: To evaluate prepubertal children born idiopathic LGA to non-obese mothers without gestational diabetes or glucosuria with respect to the presence of MS antecedents. PATIENTS AND METHODS: We conducted a cross-sectional study to compare 40 (19 F) LGA-born prepubertal children of a mean age of 6.1 ± 2.5 yr and 49 (25 F) appropriate for gestational age (AGA)-born body mass index (BMI)-matched peers of a mean age of 5.4 ± 1.8 yr with respect to their anthropometric data, blood pressure measurements, fasting serum glucose and insulin levels, homeostasis model assessment-insulin resistance (HOMA-IR), and lipids and atherogenic index (AI) [triglyceride (TG)/high-density lipoprotein cholesterol (HDL-C)]. HOMA-IR > 2.5 was used to define IR. HDL-C ≤ 40 mg/dL and TG ≥ 110 mg/dL were used to define dyslipidemia. Both groups were further divided into subgroups as obese and non-obese according to their BMI percentiles and the analyses were repeated. RESULTS: Non-obese LGA children had higher waist circumference (WC) standard deviation scores (SDSs) than BMI-matched AGA-born peers (p = 0.024). There were no significant differences between pooled, obese and non-obese subgroups of LGA-born children and their AGA counterparts with respect to dyslipidemia and IR. AI was higher in non-obese LGA children than in AGA counterparts (p = 0.028). CONCLUSIONS: Non-obese idiopathic LGA-born children have higher AIs than AGA-born counterparts in the absence of IR. WC seems to be a good clinical screening tool in identifying at risk of non-obese LGA children. Further studies are needed to evaluate MS antecedents in idiopathic LGA-born children.
Pediatric Diabetes 04/2013; · 2.16 Impact Factor
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ABSTRACT: Objective: Premature thelarche (PT) refers to isolated onset of thelarche in girls younger than 8 years of age. Most cases have an onset under 2 years of age. We aimed to establish whether the onset of thelarche under 2 years of age certifies a transient clinical course, as suggested by several authors. Methods: Sixty-seven girls with an onset of PT under 2 years of age were classified as having early puberty (EP) or classical PT after one year of follow-up. Progression of pubertal findings or absolute growth velocity (GV) standard deviation score (SDS) above 1 SDS constituted the criteria for a diagnosis of EP. Results: Twenty (29.1%) girls were classified as having EP and 47 (70.1%) girls as having classical PT. Basal serum luteinizing hormone (LH; ICMA) values at a cut-off level of 0.3 IU/L were found to be a significant risk factor for having an atypical course [OR=7.8; CI (95%): 2.04- 30.4, p=0.003]. Conclusions: Onset of thelarche under 2 years of age does not assure a transient course in a remarkable proportion of girls with PT. An absolute GV value of >1 SDS or a basal LH level ≥0.3 IU/L are suggested as indicators for close follow-up.
Journal of Clinical Research in Pediatric Endocrinology 09/2012; 4(3):140-5.
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ABSTRACT: We aimed to determine whether precocious adrenarche (PA) has a different impact on screening tests for metabolic issues and pubertal timing in boys and girls born appropriate for gestational age (AGA). Puberty and initial metabolic screening results of 47 girls and 23 boys with PA born AGA followed up from our outpatient endocrinology clinic between May 2000 and October 2009 were reviewed. Initial anthropometric measurements except for body mass index standard deviation score (SDS) being higher in boys than girls (p = 0.01), bone age (BA) SDS, homeostasis model assessment of insulin resistance, and plasma lipids were similar between sexes. Hormone levels except for significantly higher dehydroepiandrosterone sulfate levels in boys than girls (p = 0.0006) were also similar between the sexes. BA SDS and BA/chronological age were significantly advanced (p < 0.05) with respect to initial evaluation in 28 girls at onset of gonadarche unlike the case in 13 boys with PA (p > 0.05). In conclusions, PA in children born AGA does not herald any significant differences with respect to adverse metabolic screening results between sexes, and it appears to be a discrete process from onset of puberty in girls unlike boys, in whom it is likely a variant of normal puberty.
European Journal of Pediatrics 07/2012; · 1.88 Impact Factor
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ABSTRACT: Permanent neonatal diabetes mellitus (PNDM) is a rare condition presenting before six months of age. Mutations in the genes encoding the ATP-sensitive potassium (KATP) channel are the most common causes. Sulfonylurea (SU) therapy leads to dramatic improvement in diabetes control and quality of life in most patients who carry these mutations. Here, we report the long-term follow-up results of two siblings with PNDM who were treated with insulin until ABCC8 gene mutation was identified, and were successfully transferred to oral SU therapy. After 3.5 years of follow-up on SU, one patient had a very good response, while the other one had a poor response. Bad compliance to diet was thought to be the most probable reason for poor glycemic control in this patient. In conclusion, molecular genetic diagnosis in all patients with PNDM is recommended. Compliance to treatment should be an important aspect of the follow-up of these patients.
Journal of Clinical Research in Pediatric Endocrinology 06/2012; 4(2):107-10.
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Firdevs Baş,
Hülya Kayserili,
Feyza Darendeliler,
Oya Uyguner,
Hülya Günöz,
Memnune Yüksel Apak,
Fatmahan Atalar,
Rüveyde Bundak,
Robert C Wilson,
Maria I New,
Bernd Wollnik, Nurçin Saka
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ABSTRACT: Congenital adrenal hyperplasia (CAH) due 21-hydroxylase deficiency (21-OHD) is a common autosomal recessive disorder. It is caused by defects in the CYP21A2 gene.
Our aim was to determine the frequency of common gene mutations and to evaluate genotype-phenotype correlations in Turkish 21-OHD patients.
Molecular analysis of the CYP21A2 gene was performed for the detection of the eight most common point mutations [p.P30L, IVS2-13C>G (IVS-2), p.I172N, exon 6 mutation cluster (p.I236N, p.V237E, p.M239K), p.V281L, p.Q318X, p.R356W, 8-bp-deletion], of large deletion and conversion by southern blotting, allele specific semi-quantitative PCR/enzyme restriction method and sequencing, in 56 patients with 21-OHD, from 52 families.
Disease-causing mutations were identified in 77 out of 91 alleles (84.6%) of the patients. Mutations were found in 34 of 43 alleles (79.1%) in salt wasting (SW; n=26), 32 of 36 alleles (88.8%) in simple virilizing (SV; n=24) and 11 of 12 alleles (91.6%) in non-classical (NC; n=6) form of CAH. The most frequent mutations were IVS-2 (22.0%), large conversion (14.3%), p.I172N (9.9%) p.R356W (8.8%), and large deletion (6.6%). In the SW form, the most frequent genotypes were homozygous for IVS-2 (11.5%) and homozygous for large conversion of the gene (11.5%). In the SV form, the most frequent genotype was homozygous for IVS-2 (20%), followed by compound heterozygous for p.I172N/8-bp del (10%). Homozygous for p.V281L (16.7%) was most common in NC. In most cases there was good correlation between genotype and phenotype. In the SW and NC forms, genotypes of all the patients correlated with their phenotypes.
This is the first comprehensive study on the molecular basis of CAH patients in the Turkish population. Based on these results, we propose a modified screening strategy to facilitate molecular testing of CAH patients in our population.
Journal of Clinical Research in Pediatric Endocrinology 03/2009; 1(3):116-28.
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ABSTRACT: 22q11 deletion is one of the most frequently encountered genetic syndromes. The phenotypic spectrum shows a wide variability. We report a boy who presented at age 11.9 years with seizures due to hypocalcemia as a result of hypoparathyroidism. FISH analysis revealed a heterozygote deletion at 22q11.2. Positive findings for the syndrome were delayed speech development due to velofacial dysfunction, recurrent croup attacks in early childhood due to latent hypocalcemia and mild dysmorphic features. The findings of this patient indicate that 22q11 deletion syndrome may present with a wide spectrum of clinical findings and that this diagnosis needs to be considered even in patients of older ages presenting with hypocalcemia.
Journal of Clinical Research in Pediatric Endocrinology 03/2009; 1(3):151-4.
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ABSTRACT: Assessment of pubertal stages should be related to updated and reliable referance data from the same background population.
The aim of this study was to provide normative data for the onset and tempo of puberty in Turkish girls and analyze the growth parameters in puberty.
The analyses are based on data that were collected and evaluated biannually on 1020 Turkish school children aged 8-18 years and a subsample of 101 girls who had reached final height (FH). The data were analyzed cross-sectionally in the total group and longitudinally in the subsample.
Mean age and height (Ht) at onset of puberty were 10.1 ± 1.0 years and 141.7 ± 7.6 cm, respectively. Peak height velocity (HtV) was 8.5 ± 1.0 cm/year. Total pubertal height gain was 16.0 ± 3.9 cm. The duration of puberty was 4.9 ± 1.2 years. Age at menarche was 12.2 ± 0.9 years. Height at onset of puberty was positively correlated with FH (p < 0.0001). Body size (weight and height) at onset of puberty and weight and height velocity before the year of onset of puberty correlated negatively with age at onset of puberty (p < 0.05).
In conclusion, these results provide normative data for pubertal stages and growth parameters in girls in puberty. Height at onset of puberty is the most important determinant of FH. There is no secular trend for the onset of puberty. Weight does seem to affect the onset of puberty but not FH.
Journal of Clinical Research in Pediatric Endocrinology 09/2008; 1(1):8-14.
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Kadir Babaoğlu,
Sükrü Hatun,
Ilknur Arslanoğlu,
Pinar Işgüven,
Firdevs Baş,
Oya Ercan,
Feyza Darendeliler,
Rüveyde Bundak, Nurçin Saka,
Hülya Günöz,
Abdullah Bereket,
Nihal Memioğlu,
Olcay Neyzi
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ABSTRACT: There is an increasing trend in the prevalence of type 2 diabetes mellitus (DM2) in childhood and adolescence, while positive family history of DM2 and obesity are the most important risk factors. To study the influence of family history and obesity on glucose intolerance in our country was the aim of this study.
A total of 105 children and adolescents aged 10-18 years (mean 13.3 +/- 2.5 years) were included in the study. All children and adolescents were divided into three groups according to positive family history of DM2 and obesity, and an oral glucose tolerance test (OGTT) was performed for all. Prediabetes was defined as impaired glucose tolerance (IGT) and/or impaired fasting glucose (IFG). Insulin secretion and insulin resistance were estimated using the insulinogenic index; and the homeostatic model assessment for insulin resistance (HOMA-IR) and Matsuda index, respectively.
The prevalence of prediabetes was 15.2% in the whole group, while it was 25.5% in obese children who also had a positive family history of DM2. The frequency of hyperinsulinism was 57.1% in all groups. Prediabetic children had significant insulin resistance (HOMA-IR 11.5 +/- 7.1 and 4.1 +/- 6.4, respectively, p = 0.034).
Obesity and glucose intolerance are also a problem in developing countries. The risk of prediabetes in children is highest in obese children who also have a positive family history of DM2. There is a need for a lifelong preventive program starting in childhood to avoid DM2 and decrease cardiovascular risk factors
Journal of pediatric endocrinology & metabolism: JPEM 12/2006; 19(11):1319-26. · 0.88 Impact Factor
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ABSTRACT: Worster-Drought syndrome (WDS) (congenital bilateral perisylvian syndrome, congenital pseudobulbar paresia) is characterized by neuronal migration defect, pseudobulbar paralysis, epilepsy, neuromotor retardation and perisylvian dysplasia. We report a patient with WDS associated with posterior pituitary ectopia, pituitary hypoplasia, partial empty sella and panhypopituitarism, not previously reported in the literature. The 16.4 year-old female patient had severe growth retardation with height SDS -4.5, delayed puberty, microcephaly, pes equinovarus deformity, developmental delay, speech disorder and epilepsy. Laboratory findings, which revealed abnormal electroencephalography and bilateral perisylvian cortical dysplasia on cranial magnetic resonance imaging (MRI) were consistent with WDS. Endocrinological evaluation revealed secondary hypothyroidism and combined deficiency of adrenocorticotropin, gonadotropin and growth hormone (GH). Sella MRI showed congenital empty sella, anterior pituitary hypoplasia, ectopic neurohypophysis, and stalk agenesis. Appropriate replacement therapy was started. GH treatment resulted in a final height of 150.3 cm, appropriate for her target height. This is the first reported patient with WDS associated with congenital structural hypothalamic-pituitary abnormalities, including empty sella, pituitary hypoplasia, posterior pituitary ectopia, stalk agenesis and panhypopituitarism. GH has been successful in the treatment of her short stature.
Journal of pediatric endocrinology & metabolism: JPEM 05/2006; 19(4):535-40. · 0.88 Impact Factor
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ABSTRACT: This retrospective study evaluated clinical characteristics of patients with constitutional delay of growth and puberty (CDGP) at presentation, during puberty and at final height. The records of 151 children (105 boys, 46 girls) with CDGP were reviewed and the results were evaluated with respect to findings in healthy Turkish schoolchildren. CDGP was twice as frequent in boys as in girls. Height and weight deficit and short sitting height of the children were evident at presentation and continued up to final height. Mean age of onset of puberty was retarded by 2.5 years in girls and by 3 years in boys. The time between onset of puberty and pubertal growth spurt was shorter in both girls and boys than in the controls. Peak growth velocity was compromised in both girls and boys. Forty-one patients (30 boys, 11 girls) reached final height (FH). Mean FH was shorter than both target height and predicted adult height. The Bayley-Pinneau method was found to be a better predictor of FH than either the Tanner-Whitehouse method or target height. FH also showed correlation with the father's height. There was no effect of testosterone treatment on final height. Height deficit at onset of puberty, shorter duration between onset of puberty and pubertal growth spurt, compromised peak growth velocity and short upper segment due to delayed puberty, are findings which may explain the decreased final height of children with CDGP.
Journal of pediatric endocrinology & metabolism: JPEM 03/2005; 18(2):171-9. · 0.88 Impact Factor
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ABSTRACT: Retesting of patients with growth hormone (GH) deficiency (GHD), especially those with idiopathic GHD, has yielded normalization of the results in several studies. The aim of this study was to reevaluate patients diagnosed as GHD at completion or reconfirm the diagnosis before completion of GH treatment by retesting with provocative tests, and to evaluate the value of IGF-I and IGFBP-3 levels in the diagnosis of GHD. Fifty (33 M, 17 F) patients with GHD (peak GH level <0.46 pmol/l (10 ng/ml]) in two pharmacological tests were retested and IGF-I and IGFBP-3 levels measured. The age of the patients at retest was 15.2+/-5.0 yr. Thirteen of 50 patients (26%) normalized their GH secretion. According to the initial diagnosis, 69% of those with partial GHD (peak GH level 0.32-0.46 pmol/l [7-10 ng/ml]), 43% with isolated GHD, 33% idiopathic and 11% of those with complete GHD (peak GH level <0.32 pmol/l [7 ng/ml]) normalized their GH level at retesting. None of the patients with multiple hormone deficiency and none with small pituitary on MRI normalized GH levels at retest. The sensitivities of IGF-I and of IGFBP-3 were 70% and 67%, respectively, and the specificities were 100%, when peak GH cutoff is taken as 0.46 pmol/l (10 ng/ml) for the diagnosis of GHD. The sensitivities of IGF-I and IGFBP-3 increased to 76.5% and 73.5% when the cutoff level for GHD is taken as 0.32 pmol/l (7 ng/ml). Those patients who normalized their GH levels at retest showed a satisfactory height velocity when GH therapy was discontinued. In conclusion, reevaluation of GH status may also be undertaken while patients are still on treatment as well as at completion of treatment, especially in patients with idiopathic, partial and isolated GHD, by retesting and by IGF-I and IGFBP-3 measurements. Lowering the cutoff level of GH peak at pharmacological tests to 0.32 pmol/l (7 ng/ml) will lower the number of false positive results in the diagnosis of GHD.
Journal of pediatric endocrinology & metabolism: JPEM 07/2004; 17(7):1007-12. · 0.88 Impact Factor
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ABSTRACT: Growth hormone (GH) treatment has been used in children with intrauterine growth retardation (IUGR) to promote growth with success in several short- and long-term clinical trials. Intermittent GH therapy has also been advocated in children with IUGR. This study was designed to evaluate the growth of children with IUGR after discontinuation of a two-year trial of GH treatment. Sixteen children (12 F, 4 M) who had received GH (Genotropin) at age 5.3 (1.3) years at a dose of 0.2 IU/kg/day for 2 years (Group 1) and 10 (6 F, 4 M) controls of age 4.3 (1.7) years without treatment (Group 2) were followed after completion of the trial over a median period of 4 years. Height SDS of the GH-treated group showed an increase from -3.0 (0.5) to -1.9 (0.7) (p <0.001) over 2 years of therapy. Off therapy, height SDS decreased to -3.5 (0.5) at a mean age of 11.2 (1.6) years. The difference between the initial and recent height SDS in this group was significantly different (p = 0.02). Height SDS of the control group, -2.7 (1.4) initially, did not change over the two-year observation period. At follow-up, seven control children received GH in a similar fashion for one year. In spite of an insignificant increase in height SDS on one year of GH, it decreased to -2.9 (1.6) at age 11.0 (2.1) years at the latest visit. There was no significant difference between the recent heights of the two groups at final examination. One girl in Group 1 developed acanthosis nigricans and type 2 diabetes mellitus at age 13.3 years, after the follow-up period. A second patient developed osteosarcoma in the left tibia at age 9.9 years, for which she received chemotherapy and surgery. In conclusion, height SDS showed a significant increase on GH therapy for 2 years in children with IUGR; however, it decelerated after discontinuation of therapy. At the final visit, GH therapy did not seem to have had any effect on height prognosis. This finding shows that GH should be given continuously to improve final height in children with IUGR.
Journal of pediatric endocrinology & metabolism: JPEM 06/2002; 15(6):795-800. · 0.88 Impact Factor
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ABSTRACT: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) can occur as a result of mutations in the subunits that form the ATP-sensitive potassium channel (K+ATP) in pancreatic beta-cells which play a major role in modulating insulin secretion from the beta-cells. Mutations have been shown in the genes for these subunits, namely for the plasma membrane sulfonylurea receptor (SUR1), ABCC8, and its associated inwardly rectifying potassium channel (KIR6.2) KCNJ11. Drugs which act on K+ATP channels, such as diazoxide, seem to need intact ABCC8 to be able to show their effects. Thus, it would be desirable to know the exact locus of the abnormality in the beta-cell to be able to choose the right therapeutic agent or to perform early pancreatectomy. The aim of this study was to search for the correlation between the mutations of the K+ATP channel and the outcome of therapeutic measures in patients with PHHI followed for a duration of 4 months to 7.3 years. Thirteen patients (5 F, 8 M) with PHHI with a median age of 2.5 months (8 days-12.1 years) were included in the study. Therapy for PHHI was initiated either with diazoxide (n = 9) or with calcium channel blocker (n = 4) as the agent of first choice. Three patients unresponsive to drugs underwent 95% pancreatectomy. Mutation analysis was performed by polymerase chain reaction (PCR) and single strand conformation polymorphism (SSCP) in DNA samples extracted from patients' peripheral leukocytes. The PCR products were directly sequenced. Screening of ABCC8 and KCNJ11 for mutations revealed abnormalities in the ABCC8 gene in three patients out of 13: homozygosity for the 155del1 mutation, compound heterozygosity for T267-->G/A4612-2-->G, and compound heterozygosity for G4310-->A/ R1494Q. No mutations in the KCNJ11 gene were identified. Of the three patients who underwent pancreatectomy, two had identified mutations and one did not have any known mutation. In two patients in whom hyperinsulinism recurred after surgery and in the rest of the children, therapy with either diazoxide or calcium channel blocker proved to be effective in controlling hypoglycemia over the follow-up period. Thus it may be concluded that mutations in the ABCC8 gene were not predictive of the response to drugs. Unidentified mutations in the K+ATP channels other than those screened or other functional abnormalities in these channels may account for the different therapeutic responses.
Journal of pediatric endocrinology & metabolism: JPEM 15(7):993-1000. · 0.88 Impact Factor
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ABSTRACT: It is known that type 1 diabetes mellitus (type 1 DM) may be associated with other autoimmune diseases. Recently, a patient with an association of type 1 DM and familial Mediterranean fever (FMF) was reported in the medical literature. A 10.5-year-old boy was brought to our clinic with complaints of polydipsia, polyuria and weight loss and was diagnosed as diabetic ketoacidosis due to autoimmune type 1 DM. Insulin therapy was started. Elevated thyroid antibodies associated with diffuse goiter and hypothyroidism led to the diagnosis of autoimmune thyroid disease (ATD), and elevated antiendomysial antibodies and abnormal intestinal biopsy findings led to the diagnosis of celiac disease (CD). L-thyroxine therapy and gluten-free diet were initiated accordingly. At the third-year of follow-up, acute attacks of fever, abdominal pain and chest pain developed. Laboratory investigations, which were normal between the attacks, revealed elevated erythrocyte sedimentation rate, fibrinogen, white blood cell count and pleural effusion on chest X-ray during the attacks. Molecular analysis for FMF revealed compound heterozygous M694I and V726A. The patient responded well to colchicine therapy started at a dose of 1.5 mg/day. We present the second patient with type 1 DM associated with FMF who also had ATD and CD.
The Turkish journal of pediatrics 51(2):183-6. · 0.44 Impact Factor
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ABSTRACT: The aim of this study was to analyze head circumference (HC) growth retrospectively in longitudinally followed growth hormone (GH)-deficient children on GH therapy. Data of 54 (25 F, 29 M) children with GH deficiency were analyzed by dividing the children into two groups: Group 1 with height age (HA) < or =5 years (yrs) (n:18) and Group 2 with HA >5 yrs (n:36). Anthropometric measurements were expressed as standard deviation score (SDS) for chronological age (CA), and HC was also expressed as SDS for CA and HA. Group 1, with CA 6.6 (2.9) yrs at onset of therapy, showed an increase in height SDS from -3.8 (1.4) to -2.4 (1.7) (p < 0.001) and in HC SDS for CA from -1.9 (1.5) to -1.3 (1.6) (p < 0.05) on 4.8 (3.5) yrs of therapy. Group 2, with CA 12.6(2.2) yrs, increased height SDS from -3.4 (1.3) to -2.5 (1.4) (p < 0.001) and HC SDS for CA from -1.2 (1.3) to -1.4(1.2) (NS). HC SDS for HA was -0.4(1.3) in Group 1 and -0.2 (1.1) in Group 2 and showed no significant change. When analyzed by quartiles for cumulative dose of GH, HC SDS for HA became 0.08(1.2) in the fourth dosage quartile (p = 0.043), not significantly different from the mean. HC is disproportionately small for age but normal for the height. GH treatment results in an increase in HC of the children towards normalization in younger children. An increase in cumulative GH dose is associated with an increase in HC, but this is not inappropriate.
The Turkish journal of pediatrics 50(4):331-5. · 0.44 Impact Factor
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Abdullah Bereket,
Serap Turan,
Nursel Elçioğlu,
Seniha Hacihanefioğlu,
Nihal Memioğlu,
Firdevs Baş,
Rüveyde Bundak,
Feyza Darendeliler,
Hülya Günöz, Nurçin Saka, [......],
Mahmut Coker,
Sükran Darcan,
Behzat Ozkan,
Zerrin Orbak,
Sitki Oztaş,
Sükrü Palandüz,
Ilhan Sezgin,
Emre Atabek,
Ibrahim Erkul,
Gürbüz Erdoğan
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ABSTRACT: Spontaneous adult height (AH) in Turner syndrome (TS) varies among populations. Population-specific AH data is essential to assess the efficacy of growth-promoting therapies in TS. A multicenter study was performed to establish AH of nongrowth hormone (GH)-treated Turkish patients with TS. One hundred ten patients with TS (diagnosed by karyotype) who reached AH (no growth in the previous year, or bone age > 15 years) without receiving GH treatment were included in the study. The average AH was found to be 141.6 +/- 7.0 cm at the age of 22.9 +/- 6.2 years, which is 18.4 cm below the population average and 16.4 cm below the patients' mid-parental heights. Bone age at start of estrogen replacement was 12.3 +/- 1.3 year. Karyotype distribution of the patients was 45X (43%), 45X/46XX (16%), 45X/46Xi (12%), 45XiXq (10%) and others (19%). When the patients were evaluated according to their karyotype as 45X and non-45X, no significant difference in AH was observed (142.4 +/- 6.9 cm vs 140.9 +/- 7.1 cm, respectively). Adult height of non-GH-treated Turkish TS patients obtained in this study was comparable to that of other Mediterranean populations, but shorter than that of Northern European patients. Karyotype does not seem to affect AH in TS.
The Turkish journal of pediatrics 50(5):415-7. · 0.44 Impact Factor
