Sets Of Siblings Research Articles

Mobilized Peripheral Blood Stem Cells (PBSC) Compared with Bone Marrow (BM) as the Stem Cell Source for Unrelated Donor Allogeneic Transplantation with Reduced Intensity Conditioning (RIC-alloSCT) in Patients with Acute Myeloid Leukemia (AML) in Complete Remission (CR): a Retrospective Analysis From the ALWP of EBMT.

Abstract 527RIC-alloSCT is being increasingly used for AML patients with high comorbidities not eligible for standard myeloablative conditioning. Few published data advocate the use of PBSC instead of BM in RIC in the HLA identical sibling setting, however only scarce data is availabel in RIC-alloSCT using unrelated donors. Moreover, sometimes volunteer donors are reluctant to use G-CSF. Therefore, we compared outcomes of PBSC vs. BM RIC-alloSCT, using the EBMT ALWP data set. Between 2000 and 2008, 803 patients with AML in CR underwent RIC-alloSCT from HLA compatible unrelated donors with PBSC (690) or BM (113) grafts. Recipient‘s age was higher in the PBSC vs. BM groups 57y (range, 19-77) and 52y (range, 18-76), respectively (P<0.0001), while gender was comparable between the 2 groups. Leukemia features were also comparable between the PBSC vs. BM groups, such as WBC at diagnosis (10.4×109/L vs. 8.6×109/L), cytogenetics–good risk (6.7% vs. 9.6%), intermediate risk (73.9% vs. 75%) and poor risk (19.4% vs. 15.4%) as well as for FAB classification. Disease status at RIC-alloSCT was not statistically different between the 2 cohorts: in the BM group 57% were in first CR and 43% in second CR compared to 64% and 36% in the PBSC group, respectively (p=0.13). Time from diagnosis to transplant was 239d in PBSC group vs. 332d in the BM group (P<0.0001) and low-dose TBI-based conditioning was more frequently used in PBSC (33.2%) compared to 6.2% in the BM group (P<0.0001). Donor gender and CMV serostatus were not statistically different in both groups. There was a difference in year of transplant as it was 2006 (2000-2008) vs. 2005 (2000-2008) for the PBSC vs. BM RIC-alloSCT (P<0.001). Results: Engraftment was achieved in 95% of the patients in both groups. Acute GVHD grade > II was significantly higher in the PBSC group, 28% compared to 12% in BM group, (P<0.0001). Similarly, chronic GVHD (2y) was higher in the PBSC with 55±3% vs. 48±9% in the BM group, respectively (P=0.05). The 2-year probabilities of LFS and relapse incidence were 44±3% and 38±3% for the PBSC transplants in comparison to 42±6% and 51±7%, for the BM group (NS), while, non relapsed mortality was significantly higher for the PBSC vs. BM group, 29±3% vs. 14±5%, respectively (P=0.007). In multivariate analysis, after statistical adjustments of differences, the PBSC group was associated with a higher incidence of aGVHD (II-IV) (HR=3.2, p=0.01), higher NRM (HR=2.6, p=0.005), a trend of decreased relapse incidence (HR 1.47, p=0.06) and no statistical difference of LFS between the two groups (p=0.62). In conclusion: 1) The vast majority (86%) of the unrelated RIC - alloSCT for AML performed in Europe are from PBSC rather than from BM grafts. 2) Our results indicate significantly higher incidence of acute GVHD and NRM, but not statistically different LFS comparing unrelated PBSC to BM grafts following RIC conditioning. Therefore, there is no disadvantage for using unrelated BM rather than unrelated PBSC grafts in the RIC-alloSCT setting. Whether PBSC should be preferred in advanced phase of the disease, where outcome is dominated by relapse incidence, need to be further investigated. Disclosures:No relevant conflicts of interest to declare.

Complete complement deficiency in a large cohort of familial systemic lupus erythematosus

Genetic complete deficiency of the early complement components such as C1, C2 and C4 commonly results in a monogenetic form of systemic lupus erythematosus (SLE). However, previous studies have examined groups of complete complement deficient subjects for SLE, while a familial SLE cohort has not been studied for deficiencies of complement. Thus, we undertook the present study to determine the frequency of hereditary complete complement deficiencies among families with two or more SLE patients. All SLE patients from 544 such families had CH50 determined. Medical records were examined for past CH50 values. There were 66 individuals in whom all available CH50 values were zero. All but four of these had a SLE-affected relative with a non-zero CH50; thus, these families did not have monogenetic complement deficient related SLE. The four remaining SLE-affected subjects were in fact two sets of siblings in which three of the four SLE patients had onset of disease at <18 years of age. Both patients in one of these families had been determined to have C4 deficiency, while the other family had no clinical diagnosis of complement deficiency. In this second family, one of the SLE patients had had normal C4 and C3 values, indicating that either C1q or C2 deficiency was possible. Thus, only 2 of 544 SLE families had definite or possible complement deficiency; however, 1 of 7 families in which all SLE patients had pediatric onset and 2 of 85 families with at least 1 pediatric-onset SLE patent had complete complement deficiency. SLE is found commonly among families with hereditary complement deficiency but the reverse is not true. Complete complement deficiency is rare among families with two or more SLE patients, but is concentrated among families with onset of SLE prior to age 18.

Is conception method or maternal factor affecting perinatal outcome?

OBJECTIVE: To assess the factor involved in an increased risk of adverse perinatal following assisted conception. DESIGN: Retrospective analysis of ART couples with singleton deliveries were evaluated for identification of maternal factors involved in perinatal outcome. MATERIALS AND METHODS: Neonatal measures such as birth weight and gestational age were recorded. The average birth weight was adjusted per gestational age and gender. Underweight and overweight neonates were considered as those falling below or above 2 standard deviation. A subanalysis included couples that conceived in a sequential manner from standard IVF then ICSI. RESULTS: A total of 5,708 ART singletons were identified: 2,354 IVF and 3,354 ICSI. Only 43.0% (1,021) of the couples undergoing IVF and 71.0% (2,381) of ICSI had complete perinatal records and allowed inclusion. The average maternal age was comparable (35.8 ± 4 vs 35.7 ± 5 yrs, respectively). A total of 151 (14.9%) IVF and 351 (14.4%) ICSI neonates were delivered prematurely between 33 to 37 weeks of gestation. In addition, 14 (1.4%) IVF and 34 (1.4%) ICSI were delivered sooner than 32 weeks. When plotted on the adjusted growth curve, 11 (1.1%) IVF and 40 (1.7%) ICSI babies were low birth weight while 30 (3.0%) and 51 (2.1%), respectively, were large for gestational age. In the hetero-conceived sibling analysis that included 22 mothers, 6 sets of siblings were identified between the 5th and the 25th percentiles while 2 sets of children were above the 90th percentile. CONCLUSIONS: Once corrected for true singletons, birth weights were comparable for the two conception methods. From the adjusted growth curve we found that the occurrence of low birth weight were similar among standard IVF and ICSI at a rate below 2%. On the other hand, overweight babies ranged from 2 to 3%. From the hetero-sibling analysis, it appears that a maternal factor seems to recur as causative of the variation in neonatal outcomes.

How do young people find out about their parent's cancer diagnosis: A phenomenological study

Aim Grief and its many manifestations can irrevocably change family life and the multiple relationships within. Informed by this, one is led to consider the extent an adult nurse provides a family focus to cancer care. It arguably remains wanting, particularly in adult healthcare environments where ‘family’ can still infer ‘spouse’ and the needs of children and young people at home may as a result go unrecognized. Little attention has been paid to what or how young people should be told about their parent's cancer diagnosis, but from the limited research that has been undertaken, parents are seeking help with this aspect of communication. Methods An interpretative phenomenological approach was used to explore the research question: How do young people experience learning about their parent's cancer diagnosis? This was a single centre study recruiting seven participants, aged 14–18 years, which included two sets of siblings. Participants were interviewed on one occasion only. Results The interview data revealed six dimensions of the young person's experience of learning about a parental diagnosis: first hearing about a parent's diagnosis; vulnerability of self and others, communication within the family, feeling supported in experience, experience and support of school, experience and support of hospital. Young people overwhelmingly advocate honesty and openness in family communication about cancer. Conclusions Our participants demonstrated strength and resourcefulness in adapting to their parent's cancer. Meaning making was central to our study's intent and still today it offers a perspective of the diagnosis period that has not been fully explored in the extant literature.

Relatives as spouses: Preferences and opportunities for kin marriage in a Western society

This article investigates the determinants of kin marriage on the basis of a large-scale database covering a major rural part of The Netherlands during the period 1840-1922. We studied three types of kin marriage: first cousin marriage, deceased spouse's sibling marriage, and sibling set exchange marriage. Almost 2% of all marriages were between first cousins, 0.85% concerned the sibling of a former spouse, while 4.14% were sibling set exchange marriages. While the first two types generally declined across the study period, sibling set exchange marriage reached a high point of almost 5% between 1890 and 1900. We found evidence for three mechanisms explaining the choice for relatives as spouses, centering both on preferences and on opportunities for kin marriage. Among the higher and middle strata and among farmers, kin marriages were commonly practiced and played an important role in the process of social class formation in the late nineteenth century. An increased choice for cousin marriage as a means of enculturation was observed among orthodox Protestants in the Bible Belt area of The Netherlands. Finally, all studied types of kin marriage took place more often in the relatively isolated, inland provinces of The Netherlands. Sibling set exchange marriages were a consequence of the enlarged supply of same-generation kin as a result of the demographic transition.

Response and toxicity of donor lymphocyte infusions following T-cell depleted non-myeloablative allogeneic hematopoietic SCT from 3–6/6 HLA matched donors

We report the outcome of early donor lymphocyte infusions (DLIs) after T-cell depleted non-myeloablative transplantation using stem cells from HLA-matched or mismatched donors. Sixty-nine patients with high-risk hematologic malignancies received DLI following fludarabine, CY and alemtuzumab with infusion of stem cells from a matched sibling (52) or partially matched family member donor (17). Patients received the first infusion at a median of 50 days after transplant, and doses ranged from 1 x 10(4) CD3+ cells/kg to 3.27 x 10(8) CD3+ cells/kg, depending on clinical status and the physician's discretion. A median cell dose of 1 x 10(5) CD3+ cells/kg in the mismatched setting and 1 x 10(6) CD3+ cells/kg in the matched sibling setting appears safe with only 1 of 7 (14%) and 4 of 31 patients (13%), respectively, experiencing severe acute GVHD at these doses. Importantly, 38% of patients with persistent disease before DLI attained a remission after infusion. Nine of the 69 patients remain alive and disease-free 32-71 months after the first DLI. In conclusion, low doses of DLI can be safely provided soon after T-cell depleted non-myeloablative therapy and provide a chance of remission. However, long-term survival still remains poor, primarily because of relapse in these patients.

Long-Term Outcome of Total Colonic Aganglionosis

No single surgical procedure for total colonic aganglionosis has clearly been proven to be superior. We present our experience and the long-term results of a standard Duhamel's pull-through procedure in the management of this rare form of Hirschsprung's disease. A retrospective study of all cases with Hirschsprung's disease diagnosed in our institute from November 1983 to December 2004 was conducted. All operated patients with total colonic aganglionosis (TCA) had a diversion in the first week of life. Duhamel's pull-through was the definitive procedure for all operated patients and was performed between 6 months and 1 year of age. All patients and their parents were contacted for an extended follow-up, ranging between 30 months and 21 years. Incontinence was assessed on the basis of soiling in the diaper between two consecutive defecations in the younger group of patients and the need for a diaper in the older age group. Hirschsprung's enteritis was regarded as severe if the patient's condition did not improve on conservative management and the patient needed an ileostomy post-pull-through. Thirty-six cases with Hirschsprung's disease were diagnosed during the study period. Twelve patients (31.4 %) had total colonic aganglionosis, confirmed at laparotomy, and underwent a diversion. Eight underwent definitive surgery during the first year of life. All operated patients had a standard Duhamel's pull-through as the definitive procedure. Four cases were not operated: two had Waardenburg syndrome with lethal total aganglionosis, one had multiple anomalies, and the last patient with mid-jejunal involvement died while awaiting a definitive procedure. Familial incidence was apparent in two sets of siblings. Leakage followed by stricture occurred in one patient. Hirschsprung's enteritis was diagnosed in three patients. In two, the enteritis was mild, while one patient required an ileostomy which was performed elsewhere. Half of our patients have an almost normal number of daily bowel movements (1 - 2 times). The long-term results of a standard Duhamel's pull-through for TCA are good in terms of continence, bowel motion, and physical development.

Individual variations in behavior and free cortisol responses to acute stress in tiger pufferfishTakifugu rubripes

The aim of the present study was for individual variation in behavior to be characterized and related to differences in physiological stress responses in juvenile tiger pufferfish (fugu) Takifugu rubripes. A set of siblings (n=330) from wild parents was subjected to behavior tests, and some were classified into active and inactive groups (n=42 each group). The active animals consisted of those individuals that kept active swimming during 6 min after transfer from one tank to another, and the inactive animals were those that stopped swimming within 6 min. Time-course acute stress responses, which were elicited by anesthesia and subsequent removal of a pectoral fin, were compared between the active and inactive fish. The stress indicators used were free and total plasma cortisol, and plasma glucose. Although no significant difference was observed at each sampling point between groups, the inactive fish required shorter recovery time from acute stress in all three indicators than the active fish. The percentage of free to total plasma cortisol in the pre-stress condition was significantly lower in the inactive group than in the active group. Our findings indicate that fugu has behavioral variation that is associated with stress responsiveness. The inactive fish are expected to be more tolerant of stress and suitable for aquaculture.

Implementation of Positive Behavior Support With a Sibling Set in a Home Environment

This study provides a demonstration of the process of positive behavior support (PBS) within a home setting to address the challenging behavior of a sibling set within family routines. Although a growing data base is demonstrating the feasibility and effectiveness of conducting functional behavioral assessment and implementing assessment-based interventions for young children in family contexts, the vast majority of these studies have focused on dyadic parent— child interactions. The presence of more than one child, particularly when all children display challenging behaviors, can amplify levels of parent stress and complicate the logistics of assessment, planning, and systematic intervention. In this study, three young children presented challenging behaviors that were highly disruptive to home routines. Functional assessments were followed by implementation of multicomponent, family-centered PBS plans individualized to four activity routines. Multiple-baseline across-routines and quasiexperimental (A-B) within-routines designs demonstrated promising effects of the interventions in reducing aggregate levels of challenging behavior.

Splenic nodules in paediatric Gaucher disease treated by enzyme replacement therapy

The natural history of focal splenic lesions in paediatric Gaucher disease (GD) is unknown and these lesions are thought to persist despite enzyme replacement therapy (ERT). To assess the prevalence, evolution and resolution of splenic nodules in a cohort of paediatric Gaucher patients treated with ERT. The US findings in 37 children with GD were retrospectively reviewed. A total of 28 children underwent serial abdominal US examinations as part of their initial assessment and during routine follow-up. All patients received ERT. Six children (21%) had splenic nodules on US examination, either at presentation or on follow-up examination. In all six patients, the nodules had resolved on follow-up imaging, with resolution taking 17 months to 4 years 8 months. Two sets of siblings developed nodules that resolved over a similar time period. Disappearance of focal splenic lesions in children with GD during follow-up has not been previously reported. The development of new focal splenic lesions in children with GD whilst on ERT has not been previously documented.

Clustering and Dispersal of Siblings in the North-Holland Countryside, 1850-1940

Abstract : »Raumliche Nahe und Distanz von Geschwistern im landlichen Nord-Holland, 1850-1940« . Why are some families scattered over a larger area than others? In this article we use a dataset with the complete life courses of all children from 210 families, originating from the same village in the commer-cialized North-western part of The Netherlands. We experiment with multino-mial logistic regression on sibling sets to discover the factors behind geo-graphical sibling dispersal. The most important factors turn out to be the survival of the parents, the civil status of the siblings, and the size and gender composition of the sibling set. Keywords : migration, family territory, siblings, North-Holland, life course, multinomial logistic regression. Introduction Siblings are vital and durable elements in one’s kinship network. Ties with brothers and sisters are maintained across the life course. To be sure, relation-ships with siblings are generally perceived to be less important than relation-ships with parents or children. Also, the strength of ties to siblings diminishes with age. More important than age, however, are life course transitions that alter the family configuration. Thus, marriage loosens ties between siblings whereas, conversely, marriage dissolution is associated with reintensified con-tacts. Thus, siblings can be seen as “permanent but flexible members of our social networks, whose roles in our networks are renegotiated in light of chang-ing circumstances and competing obligations” (White 2001, p.557). In the analysis of sibling relationships, proximity turns out to be a major factor (Sweetser 1970, p.54; White 2001, p.558; Miner and Uhlenberg 1997; Bras and Van Tilburg 2007, p.316). Distance influences some forms of contact more than others. Contacts such as phone calls (or, in the past, letters) or financial support are not strongly affected by distance. However, with increasing dis-tance the possibilities for all forms of practical support are diminished. In the past, to preserve and enhance one’s well-being the support and proximity of

Exploring Multiple Trees through DAG Representations

We present a Directed Acyclic Graph visualisation designed to allow interaction with a set of multiple classification trees, specifically to find overlaps and differences between groups of trees and individual trees. The work is motivated by the need to find a representation for multiple trees that has the space-saving property of a general graph representation and the intuitive parent-child direction cues present in individual representation of trees. Using example taxonomic data sets, we describe augmentations to the common barycenter DAG layout method that reveal shared sets of child nodes between common parents in a clearer manner. Other interactions such as displaying the multiple ancestor paths of a node when it occurs in several trees, and revealing intersecting sibling sets within the context of a single DAG representation are also discussed.

LRRTM1 on chromosome 2p12 is a maternally suppressed gene that is associated paternally with handedness and schizophrenia.

Left-right asymmetrical brain function underlies much of human cognition, behavior and emotion. Abnormalities of cerebral asymmetry are associated with schizophrenia and other neuropsychiatric disorders. The molecular, developmental and evolutionary origins of human brain asymmetry are unknown. We found significant association of a haplotype upstream of the gene LRRTM1 (Leucine-rich repeat transmembrane neuronal 1) with a quantitative measure of human handedness in a set of dyslexic siblings, when the haplotype was inherited paternally (P=0.00002). While we were unable to find this effect in an epidemiological set of twin-based sibships, we did find that the same haplotype is overtransmitted paternally to individuals with schizophrenia/schizoaffective disorder in a study of 1002 affected families (P=0.0014). We then found direct confirmatory evidence that LRRTM1 is an imprinted gene in humans that shows a variable pattern of maternal downregulation. We also showed that LRRTM1 is expressed during the development of specific forebrain structures, and thus could influence neuronal differentiation and connectivity. This is the first potential genetic influence on human handedness to be identified, and the first putative genetic effect on variability in human brain asymmetry. LRRTM1 is a candidate gene for involvement in several common neurodevelopmental disorders, and may have played a role in human cognitive and behavioral evolution.

50 Years Ago in The Journal of Pediatrics: Neonatal hepatitis in siblings

Peterman MG. J Pediatr 1957;50:315-20 In 1957, M. G. Peterman reported two sets of siblings presenting in the neonatal period with cholestasis. With the limited available therapy, there was progression of disease leading to death. Common to all the cases was the presence of giant cell transformation of liver cells on post-mortem examination resulting in the diagnosis of neonatal hepatitis. Peterman described the diagnosis as a “general all-inclusive term, probably as satisfactory as any,” recognizing that the etiology was ill-defined. Over the last 50 years, cases of familial neonatal cholestasis have served as models to elucidate hepatic physiology, to identify specific genetic and molecular defects, and to expand the differential diagnosis of intrahepatic cholestasis presenting in the newborn period. This, in turn, has lead to improved diagnostic and therapeutic modalities. Many of the known genetic forms of intrahepatic cholestasis fall into broad categories of defects in embryogenesis, bile acid metabolism, and canalicular transport. Other causes of intrahepatic cholestasis include multi-organ disorders, metabolic diseases, and other clinically definable syndromes.1Balistreri W.F. Bezerra J.A. Whatever happened to “neonatal hepatitis”?.Clinics in Liver Disease. 2006; 10: 27-33Abstract Full Text Full Text PDF PubMed Scopus (80) Google Scholar Evaluation of infants presenting with conjugated hyperbilirubinemia has evolved to include recognition of the immature physiology of the newborn liver, evaluation for infection and metabolic disease, radiographic study of the biliary anatomy, and differentiation of intrahepatic cholestatic syndromes through a combination of clinical features, serum bile acid level, serum gamma glutamyl transpeptidase level, and liver biopsy. Diagnostic studies of the future include development of a gene chip, biomarkers of genetic defects, and targeted testing of molecular defects. Continued understanding of liver physiology and molecular pathophysiology of cholestatic disease has resulted in therapy to reduce symptoms, improve nutritional status, and delay progression of disease. These measures encompass medical therapy as well as surgical intervention including liver transplantation. The goal, however, should be to avoid the need for liver transplantation by defining and treating the underlying disease. Despite the progress over the last 50 years in diagnosis and treatment of neonatal hepatitis, approximately 15% of patients continue to be diagnosed with idiopathic disease.1Balistreri W.F. Bezerra J.A. Whatever happened to “neonatal hepatitis”?.Clinics in Liver Disease. 2006; 10: 27-33Abstract Full Text Full Text PDF PubMed Scopus (80) Google Scholar Clearer definition of phenotype, precise nomenclature of known diseases, and multi-center registries will be critical in improving diagnostic and treatment strategies in those with defined disease and in understanding the etiology of disease in those yet undiagnosed cases.

‘It's a connection you can't get away from’: Brothers, Sisters and Social Capital

Using the theoretical framework of social capital, this paper explores the resources and support children and young people derive from their siblings. A prevailing representation of siblings as passive dependents competing for limited reserves of family social capital is undermined by our empirical study of being and having a brother or sister. Drawing on qualitative interviews with 44 children and young people from 16 sibling sets, we show how brothers and sisters generate significant social resources for each other within and outside their families. In particular, we demonstrate how younger siblings rely on older brothers and sisters to cope with the demands of growing up and becoming adult.

Relative Contribution of Genetic and Nongenetic Modifiers to Intestinal Obstruction in Cystic Fibrosis

Neonatal intestinal obstruction (meconium ileus [MI]) occurs in 15% of patients with cystic fibrosis (CF). Our aim was to determine the relative contribution of genetic and nongenetic modifiers to the development of this major complication of CF. A total of 65 monozygous twin pairs, 23 dizygous twin/triplet sets, and 349 sets of siblings with CF were analyzed for MI status, significant covariates, and genome-wide linkage. Specific mutations in the CF transmembrane conductance regulator (CFTR), the gene responsible for CF, correlated with MI, indicating a role for CFTR genotype. Monozygous twins showed substantially greater concordance for MI than dizygous twins and siblings (P = 1 x 10(-5)), showing that modifier genes independent of CFTR contribute substantially to this trait. Regression analysis revealed that MI was correlated with distal intestinal obstruction syndrome (P = 8 x 10(-4)). Unlike MI, concordance analysis indicated that the risk for development of distal intestinal obstruction syndrome in CF patients is caused primarily by nongenetic factors. Regions of suggestive linkage (logarithm of the odds of linkage >2.0) for modifier genes that cause MI (chromosomes 4q35.1, 8p23.1, and 11q25) or protect from MI (chromosomes 20p11.22 and 21q22.3) were identified by genome-wide analyses. These analyses did not support the existence of a major modifier gene on chromosome 19 in a region previously linked to MI. The CFTR gene along with 2 or more modifier genes are the major determinants of intestinal obstruction in newborn CF patients, whereas intestinal obstruction in older CF patients is caused primarily by nongenetic factors.

Breast-feeding and overweight in adolescence: within-family analysis [corrected

Previous reports have found associations between having been breast-fed and a reduced risk of being overweight. These associations may be confounded by sociocultural determinants of both breast-feeding and obesity. We addressed this possibility by assessing the association of breast-feeding duration with adolescent obesity within sibling sets. We surveyed 5,614 siblings age 9 to 14 years and their mothers. These children were a subset of participants in the Growing Up Today Study, in which we had previously reported an inverse association of breast-feeding duration with overweight. We compared the prevalence of overweight (body mass index exceeding the age-sex-specific 85th percentile) in siblings who were breast-fed longer than the mean duration of their sibship with those who were breast-fed for a shorter period. Then we compared odds ratios from this within-family analysis with odds ratios from an overall (ie, not within-family) analysis. Mean +/- standard deviation breast-feeding duration was 6.4 +/- 4.0 months, and crude prevalence of overweight was 19%. On average, siblings who were breast-fed longer than their family mean had breast-feeding duration 3.7 months longer than their shorter-duration siblings. The adjusted odds ratio (OR) for overweight among siblings with longer breast-feeding duration, compared with shorter duration, was 0.92 (95% confidence interval = 0.76-1.11). In overall analyses, the adjusted OR was 0.94 (0.88-1.00) for each 3.7-month increment in breast-feeding duration. The estimated OR for the within-family analysis was close to the overall estimate, suggesting that the apparent protective effect of breast-feeding on later obesity was not highly confounded by unmeasured sociocultural factors. A larger study of siblings, however, would be needed to confirm this conclusion.

Cactophilic Drosophila in South America: A Model for Evolutionary Studies

The Drosophila buzzatii cluster is composed of seven cactophilic species and their known geographical distribution encompasses the open vegetation diagonal, which includes the morphoclimatic Domains of the Caatinga, Chaco and Cerrado, which are situated between the Amazon and the Atlantic forests. Besides these areas, these cactophilic species are also found in a narrow strip along the Atlantic coast from northeastern Brazil to the southern tip of the country. The hypothesis of vicariant events, defining the core areas of each species, is proposed to explain the historical diversification for the cluster. The intraspecific analysis for the cluster shows a population structure with gene flow restricted by distance, range expansion with secondary contact resulting in introgression and simpatry, especially in the limits of the species distribution, polytypic populations and assortative mating in inter population experiments. There is a variation related to these events that depends on the species and geographic origin of the population analyzed. These events are, hypothetically, described as the results of expansion and retraction of the population ranges, as a consequence of their association with cacti, which theoretically follow the expansion and retraction of dry areas during the paleoclimatic oscillations in South America, as that promoted by the glacial cycles of the Quaternary. The Drosophila buzzatii cluster is divided into two groups. The first one is composed of D. buzzatii, a species that has a broad geographic distribution and no significant differentiation between its populations. The second is the Drosophila serido sibling set, which encompasses the others species and is characterized by a significant potential for differentiation.

Killer Cell Immunoglobulin-like Receptor (KIR) Analysis in Adult Korean Patients with Acute Myeloid Leukemia

Background: The prevalent natural killer (NK) cells induce alloreaction against leukemic cells during post-transplant. NK cell alloreactivity depends on the compatibility of killer cell immunoglobulin-like receptors (KIR) epitopes for graft-versus-host disease. Genotypic expressions of inhibitory or activating KIR in patients with acute myelogenous leukemia (AML) and their HLA-matched sibling donors, as a model for Korean KIR haplotype diversity and NK alloreactivity, were investigated. Methods: Ninety-two patients in complete remission and their 76 HLA-matched sibling donors were enrolled in this study. All the patients were scheduled to receive allogeneic hematopoietic stem cell transplantations (HSCT). KIR PCR-SSP typing was performed for 19 different kinds of KIR genes and pseudogenes. The PCR data representing the KIR genotypes from both the patients and donors were compared. Results: We found 43 Korean KIR haplotypes. Thirty-three variable haplotypes for the AML patients, in addition to 25 haplotypes for the normal HSCT donors, were demonstrated. Of note, the expressions of specific genes such as 2DL2 (P=0.026), 2DS2 (P=0.042), and 2DS4 (P=0.037) revealed remarkable differences between the patients and the normal donors. Korean HLA-identical sibling pairs showed 38% KIR matches in terms of the gene content and allelic polymorphism. Although the KIR gene content was the same between the patients and the donors, 40% of those matched pairs of patients and donors showed allelic polymorphism, specifically in the context of 2DL5 and 2DS4 genes. Conclusion: These results indicate that the expressions of donor inhibitory and activating repertoire of KIR genotypes, even in the HLA-matched sibling setting, are unique parameters to be considered when we perform allogeneic sibling HSCT.

Demographic dynamics and kinship in anthropological populations.

Changes in fertility and mortality affect the size of surviving sibling sets and thus numbers of surviving kin. Because the genealogical generations specifying kinship relations are not temporal cohorts and most plausible demographic changes in anthropological populations are period shocks, the effect of such shocks on kin counts are complex. Shocks increasing fertility or decreasing mortality produce larger numbers of kin per ego and decrease the inequality of the distribution of kin and vice versa. Effects are more diffuse at more distant collateral ranges. Effects are stronger the more intense the shock and the longer its duration. Kinship distributions return to their initial state after the shock and as the original age structure of the population is ergodically reattained. Alternating shocks produce more complex patterns. Implications of these outcomes are that opportunities for political networking and consolidation by means of kinship are altered by demographic instabilities, as are the dynamics of kin selection. This analysis is limited for simplicity to unilineal agnatic reckoning of kin.