Water in the terrestrial planet-forming zone of the PDS 70 disk.

Terrestrial and sub-Neptune planets are expected to form in the inner (less than 10 AU) regions of protoplanetary disks1. Water plays a key role in their formation2-4, although it is yet unclear whether water molecules are formed in situ or transported from the outer disk5,6. So far Spitzer Space Telescope observations have only provided water luminosity upper limits for dust-depleted inner disks7, similar to PDS 70, the first system with direct confirmation of protoplanet presence8,9. Here we report JWST observations of PDS 70, a benchmark target to search for water in a disk hosting a large (approximately 54 AU) planet-carved gap separating an inner and outer disk10,11. Our findings show water in the inner disk of PDS 70. This implies that potential terrestrial planets forming therein have access to a water reservoir. The column densities of water vapour suggest in-situ formation via a reaction sequence involving O, H2 and/or OH, and survival through water self-shielding5. This is also supported by the presence of CO2 emission, another molecule sensitive to ultraviolet photodissociation. Dust shielding, and replenishment of both gas and small dust from the outer disk, may also play a role in sustaining the water reservoir12. Our observations also reveal a strong variability of the mid-infrared spectral energy distribution, pointing to a change of inner disk geometry.

First Evidence of Axial Shape Asymmetry and Configuration Coexistence in

The excited states of N=44 ^{74}Zn were investigated via γ-ray spectroscopy following ^{74}Cu ß decay. By exploiting γ-γ angular correlation analysis, the 2_{2}^{+}, 3_{1}^{+}, 0_{2}^{+}, and 2_{3}^{+} states in ^{74}Zn were firmly established. The γ-ray branching and E2/M1 mixing ratios for transitions deexciting the 2_{2}^{+}, 3_{1}^{+}, and 2_{3}^{+} states were measured, allowing for the extraction of relative B(E2) values. In particular, the 2_{3}^{+}→0_{2}^{+} and 2_{3}^{+}→4_{1}^{+} transitions were observed for the first time. The results show excellent agreement with new microscopic large-scale shell-model calculations, and are discussed in terms of underlying shapes, as well as the role of neutron excitations across the N=40 gap. Enhanced axial shape asymmetry (triaxiality) is suggested to characterize ^{74}Zn in its ground state. Furthermore, an excited K=0 band with a significantly larger softness in its shape is identified. A shore of the N=40 "island of inversion" appears to manifest above Z=26, previously thought as its northern limit in the chart of the nuclides.

Breeding options for nematode resistance in Lacaune dairy sheep.

Due to progressing anthelmintic resistance of gastrointestinal nematodes (GIN), supportive measures are needed to control these parasites. In sheep, it has been shown that selection towards an increased nematode resistance is feasible and that faecal egg count (FEC) is the generally acknowledged trait for selection. However, a selection based on FEC would come with certain costs, therefore auxiliary, cheaper resistance traits would be most welcome. FAMACHA©, a colour classification of the eyelid, usually used to determine the manifestation of an infection with Haemonchus contortus, could serve as such. Therefore, we collected FAMACHA©, packed cell volume (PVC) and FEC phenotypes of approx. 1150 naturally infected Lacaune ewes on 15 commercial farms in Switzerland. The Haemonchus-proportion was determined on farm level. Phenotypic correlations of FEC and FAMACHA© as well as FAMACHA© and PCV were 0.25 (SE 0.03) and -0.35 (SE 0.08), respectively, and correspond well with the results of other studies. A multi-trait animal model was applied to estimate genetic parameters with FEC, FAMACHA©, PVC and milk yield as dependent variables. The heritabilities of FEC, FAMACHA©, PCV and milk yield were estimated to be moderate with values of 0.33 (SE 0.08), 0.30 (SE 0.08), 0.36 (SE 0.08) and 0.34 (SE 0.08), respectively. The genetic correlations between FEC and FAMACHA© and between FEC and PCV were estimated to be close to zero with values of 0.03 (SE 0.22) and 0.01 (SE 0.21), respectively. The average Haemonchus-proportion compared to other GIN was found to be 43%. The FAMACHA© classification of the Lacaune ewes seems to indicate a rather high worm challenge, with 38, 14 and 2% of observations classified to scores 3, 4 and 5, respectively. However, the worm challenge according to FEC was moderate. It has been suggested that the genetic correlation between FAMACHA© and FEC is more pronounced when FEC was high. It could therefore be that the lack of genetic correlation was due to an insufficient worm challenge, even though the Lacaune were grazing at least 70 days before phenotyping. The genetic correlation between FEC and milk yield was estimated to be 0.07 (SE 0.22, slightly unfavourable). We conclude that if FEC is used as trait, the Lacaune could be selected for lower susceptibility towards nematode infection. The use of FAMACHA© as an auxiliary trait for FEC is not feasible, due to an inexistent genetic correlation between these two traits.

Two faces of temporal binding: Action- and effect-binding are not correlated.

Research on the sense of agency has proliferated a range of explicit and implicit measures. However, the relation of different measures is poorly understood with especially mixed findings on the correlation between explicit judgments of agency and the implicit perceptual bias of temporal binding. Here, we add to the conundrum by showing that the two sub-components of temporal binding - action-binding and effect-binding, respectively - are not correlated across participants either, suggesting independent processes for both components. Research on inter-individual differences regarding the sense of agency is thus well-advised to rely on other implicit measures until the phenomenon of temporal binding is better understood.

Biparietal meander expansion technique for sagittal suture synostosis in patients older than 1 year of age-technical note.

OBJECTIVE: Sagittal suture synostosis (SSS) is the most common form of craniosynostosis. For older patients, the strategy for surgical correction needs to consider diminished growth dynamics of the skull and an active reconstruction cranioplasty aims to sustain stability for the active child. We describe our technique of biparietal meander expansion (BME) technique for SSS for patients older than 1 year and retrospectively reviewed the perioperative course as well as the subjective experience of patients and caregivers during follow-up. METHODS: The BME technique incorporates bilateral serpentine craniotomies and fixation of the consecutively expanded bone tongues with crossing sutures for patients with SSS older than 12 months of age at surgery. We reviewed patients undergoing this surgical technique for correction of SSS and collected data about the clinical course and performed a patients reported outcome measure (PROM) for patients or caregivers to evaluate subjective experience and outcome after surgical treatment. RESULTS: BME was performed in 31 patients (8 females; median age: 43 months; range 13-388). The mean length of operation was 172.7±43 minutes (range 115-294). Patients experienced no immediate complications or neurological morbidity after surgery. Considering a total of 21 completed PROM questionnaires, the head shape after surgery was evaluated as either "better" (57%) or "much better" (43%) compared to preoperatively. Eighty-one percent of patients or caregivers answered that the patient experiences no limitation in daily activities. Although 42.8% perceived the hospital as strenuous, 90.5% would choose to undergo this treatment again. CONCLUSION: BME is a feasible technique for older SSS patients resulting in immediate stability of the remodelled calvarium with a more normal head shape. The survey among caregivers or patients revealed a favourable subjectively experienced outcome after this type of surgical treatment of SSS in the more complex context of an older patient cohort.

Trimethylamine-N-oxide is elevated in the acute phase after ischaemic stroke and decreases within the first days.

BACKGROUND AND PURPOSE: Trimethylamine-N-oxide (TMAO) is a biomarker of the gut microbiome and correlates with the risk of cardiovascular diseases. However, conflicting data exist on the specific role of TMAO in ischaemic stroke patients. We aimed to analyze the time course of TMAO levels in stroke patients compared with controls. METHODS: In this prospective, case-control study, patients suffering from ischaemic stroke (onset <24 h) and control patients with less than two cardiovascular risk factors were enrolled. Plasma TMAO levels were analyzed on admission, after 48 h and after 3 months. The primary endpoint was the difference in TMAO levels on admission between stroke patients and controls. RESULTS: A total of 196 patients with ischaemic stroke and 100 controls were included between February 2018 and April 2019. Plasma TMAO levels on admission were significantly higher in stroke patients than in controls [median value 4.09 (2.87-6.49) vs. 3.16 (2.08-5.16) µmol/L, P = 0.001]. There was a significant decrease in TMAO levels in stroke patients after 48 h [median at 48 h, 3.49 (2.30-5.39) µmol/L, P = 0.027]. TMAO levels increased again 3 months after stroke [median 4.23 (2.92-8.13) µmol/L, P = 0.047]. In controls, TMAO levels did not change between admission and after 48 h [median at 48 h, 3.14 (1.63-4.61) µmol/L, P = 0.11]. An inverse correlation between TMAO values and kidney function was found (Spearman rho -0.334, P < 0.001). CONCLUSIONS: Our study emphasizes the importance of the time course of TMAO levels after ischaemic stroke. Future studies should define the time point of TMAO analysis, preferably in the acute phase (<24 h).

Sustainable bioenergy for climate mitigation: developing drought-tolerant trees and grasses.

BACKGROUND AND AIMS: Bioenergy crops are central to climate mitigation strategies that utilize biogenic carbon, such as BECCS (bioenergy with carbon capture and storage), alongside the use of biomass for heat, power, liquid fuels and, in the future, biorefining to chemicals. Several promising lignocellulosic crops are emerging that have no food role - fast-growing trees and grasses - but are well suited as bioenergy feedstocks, including Populus, Salix, Arundo, Miscanthus, Panicum and Sorghum. SCOPE: These promising crops remain largely undomesticated and, until recently, have had limited germplasm resources. In order to avoid competition with food crops for land and nature conservation, it is likely that future bioenergy crops will be grown on marginal land that is not needed for food production and is of poor quality and subject to drought stress. Thus, here we define an ideotype for drought tolerance that will enable biomass production to be maintained in the face of moderate drought stress. This includes traits that can readily be measured in wide populations of several hundred unique genotypes for genome-wide association studies, alongside traits that are informative but can only easily be assessed in limited numbers or training populations that may be more suitable for genomic selection. Phenotyping, not genotyping, is now the major bottleneck for progress, since in all lignocellulosic crops studied extensive use has been made of next-generation sequencing such that several thousand markers are now available and populations are emerging that will enable rapid progress for drought-tolerance breeding. The emergence of novel technologies for targeted genotyping by sequencing are particularly welcome. Genome editing has already been demonstrated for Populus and offers significant potential for rapid deployment of drought-tolerant crops through manipulation of ABA receptors, as demonstrated in Arabidopsis, with other gene targets yet to be tested. CONCLUSIONS: Bioenergy is predicted to be the fastest-developing renewable energy over the coming decade and significant investment over the past decade has been made in developing genomic resources and in collecting wild germplasm from within the natural ranges of several tree and grass crops. Harnessing these resources for climate-resilient crops for the future remains a challenge but one that is likely to be successful.

The effects of action choice on temporal binding, agency ratings, and their correlation.

The sense of agency, i.e., the feeling of control over one's own actions and their consequences in the environment, is a crucial part of action taking. In experimental studies, agency is most commonly measured either directly via explicit agency ratings or indirectly via implicit measures, e.g., temporal binding. In order to aid our interpretation of previous and future results, several studies have focused on relating implicit and explicit measures of agency to one another. However, possibly due to different methodological issues, results have been far from conclusive. In the present study, we therefore contribute to this discussion by further characterizing temporal binding and explicit agency ratings in their response to action choice as an experimental manipulation in a high-powered design, and by studying how temporal binding and agency ratings are related in different experimental conditions. Furthermore, we discuss the possible influence of the specific agency question regarding the participants' ratings.

Decay rates of Escherichia coli, Enterococcus spp., F-specific bacteriophage MS2, somatic coliphage and human adenovirus in facultative pond sludge.

The purpose of this study was to evaluate the efficacy of a waste stabilization pond (WSP) system to reduce pathogen contaminants in sludge. This included examining the factors that influence the fate and concentration of human pathogens and their indicators in two sludge layers. The decay rates of five study microorganisms were determined under in-situ conditions at a WSP. The background levels of fecal origin microorganisms were consistently detected (ranging: Escherichia coli 104 to 106, enterococci 101 to 103, F-specific bacteriophage (MS2) 101 to 103 and somatic coliphage 101 to 104 colony-forming units (CFU) mL-1, as well as 101 to 102 human adenovirus gene copies mL-1) in the primary facultative pond. Among microorganisms tested, the bacteria generally decayed faster than adenovirus and bacteriophage, particularly in the upper sludge layer. Due to the observed regrowth of E. coli, it may have a limited value as an indicator for pathogen removal in the wastewater stabilization ponds. The abundance of E. coli numbers within the pond biome followed changes in pond temperature over time. The results of the study suggest that viruses could survive for a long time, particularly in deeper layers (>1 metre) in the sludge, during winter months (T90 = 156 d). The presence of human pathogens in WSP sludge, in particular viruses, may be a barrier to its beneficial reuse in agriculture. The results indicate that additional treatment of sludge may be required to mitigate potential public health risks from reuse of sludge for agricultural purposes.

Resequencing of four novel alleles with nanopore technology.

Long nanopore reads allow phasing of heterozygous positions but show limitations in sequencing of homopolymers.

Quality of life in adolescents with hepatitis C treated with sofosbuvir and ribavirin.

Chronic HCV infection has been associated with impairment of HRQL in both adults and paediatric patients. Our aim was to assess the HRQL of HCV-positive children treated with SOF + RBV. The data for this post hoc analysis were collected in a phase 2 open-label multinational study that evaluated safety and efficacy of SOF (400 mg/day) plus RBV (weight-based up to 1400 mg/day) for 12 or 24 weeks in adolescents with chronic HCV (GS-US-334-1112). Patients and their parents/guardians completed the PedsQL-4.0-SF-15 questionnaire at baseline, at the end of treatment and in post-treatment follow-up. We included 50 adolescents with HCV genotype 2 and 3 without cirrhosis (14.8 ± 1.9 years; male: 58%; treatment-naïve: 82%; vertically transmitted HCV: 70%). After treatment, 100% of patients with HCV genotype 2 and 95% with genotype 3 achieved SVR-12. During treatment with SOF + RBV, there were no significant decrements in any of patients' self-reported or parent-proxy-reported PRO scores regardless of treatment duration (all P > .05). After treatment cessation, we recorded a statistically significant improvement in patients' self-reported Social Functioning score by post-treatment week 12: on average, +4.8 points on a 0-100 scale (P = .02). By post-treatment week 24, parent-proxy-reported School Functioning score increased by, on average, +13.0 points (P = .0065). In multivariate analysis, history of abdominal pain and psychiatric disorders were predictive of impaired HRQL in adolescents with HCV (P < .05). Adolescents with HCV do not seem to experience any HRQL decrement during treatment with SOF + RBV and experience some improvement of their HRQL scores after achieving SVR.

Valve exchange towards an adjustable differential pressure valve with gravitational unit, clinical outcome of a single-center study.

OBJECTIVE: Overdrainage in children is a long-term problem for shunted patients which might lead to chronic anatomical changes. In order to prevent these problems, valve exchange is performed on a regular basis in patients without hydrostatic units towards a valve with both an adjustable and a gravitational unit. The clinical outcome of these patients is reported in a retrospective study. METHODS: Between 2009 and 2014, the in-house database was analyzed for patients who received a valve exchange towards an adjustable differential pressure valve with gravitational unit. The study protocol included the patients shunt history, image analysis for ventricular width, and necessity of revision surgery after valve exchange. A questionnaire was sent to the patients in order to ask for their subjective experience for symptom changes and treatment experience. RESULTS: Forty-six patients were identified (26 girls, mean age 11.8 ± 6.1 years) with a mean follow-up of 36.3 ± 15 months. The ventricular width did increase after valve exchange as measured in frontal and occipital horn ratio (0.364 ± 0.032 vs. 0.402 ± 0.09, p = 0.0017). Of the patients suffering from acute symptoms, 89% improved after treatment. The shunt and valve survival rates were 88 and 95%, respectively, after 12 months. Comparing the total amount of revisions before and after valve exchange, a significant reduction was seen in total but a no significant difference was analyzed in amount of revisions to time ratio. CONCLUSION: Valve exchange might be cautiously decided if patients seem to perform clinically well. In our study, we were able to show that the strategy of valve exchange to prevent chronic overdrainage is well tolerated and seem to improve patient's clinical outcome in terms of ventricular width, symptom relieve, and revision rate.

Implementation of feeding guidelines in infants at risk of intestinal failure.

OBJECTIVE: To implement feeding guidelines to reduce advancement time and the incidence of parenteral nutrition-associated liver disease (PNALD) among intestinal surgical infants requiring parenteral nutrition (PN). STUDY DESIGN: Feeding guidelines with higher initial enteral nutrition (EN) volume and specific advancement criteria were implemented for surgical infants aged <6 months. Preimplementation and postimplementation outcomes were compared. RESULTS: There were 57 preimplementation and 33 postimplementation infants. The initial EN volume improved from 10 to 20 ml kg(-1) day(-1) (P<0.001). Time to reach 50% of goal calories from EN decreased by a median of 6 days (P=0.012) without a change in necrotizing enterocolitis incidence after resuming feeding. PNALD incidence decreased from 70% to 48% (P=0.046), and median peak direct bilirubin (DB) decreased from 5.6 to 2.3 mg dl(-1) (P=0.011). CONCLUSION: Feeding guideline implementation with higher initial feeding volume was well tolerated and resulted in faster achievement of 50% goal EN calories. PNALD incidence and peak DB were reduced.

Indirect selection of industrial tomato genotypes that are resistant to spider mites (Tetranychus urticae).

Acyl sugars present in the tomato Solanum lycopersicum 'LA-716' accession confer good levels of resistance to arthropod pests. The objective of the present study was to select F2 plants from the interspecific cross Solanum pennellii 'LA-716' x Solanum lycopersicum 'Redenção' to assess resistance to spider mites (Tetranychus urticae) based on the leaf acyl sugar content and repellence tests. Four genotypes were selected with high leaflet acyl sugar content (RVTA-2010 pl#31, RVTA-2010 pl#75, RVTA-2010 pl#83, and RVTA-2010 pl#94), and an additional three genotypes with low acyl sugar content were also selected (RVTA-2010 pl#33, RVTA-2010 pl#39, and RVTA-2010 pl#73). The results from the in vivo tests used to confirm the selection of plants resistant to mites indicated that the genotypes with high acyl sugars content did not differ from the resistant parent LA-716. The negative correlation between acyl sugar content and the distance run by the mite along the leaflet surface confirmed the association between high and low allelochemical content and resistance. The medium degree of dominance (MDD) was estimated (MDD = -0.83), indicating that the high acyl sugar content was due to incomplete dominance of a recessive allele. A value of 81.85% was found for the broad sense heritability estimate, which suggests that most among-plant variation in the F2 generation is genetically based. Furthermore, 0.69 genes were estimated, which presumably confirms monogenic inheritance. Thus, indirect selection was an efficient method used to obtain industrial tomato plants that are resistant to spider mites.

Variability of hepatitis E serologic assays in a pediatric liver transplant recipient: challenges to diagnosing hepatitis E virus infection in the United States.

Hepatitis E virus (HEV) is an emerging cause of viral hepatitis among immunocompromised individuals in developed countries. Yet the diagnosis of HEV infection in the United States remains challenging, because of the variable sensitivity and specificity of currently available tests, and the lack of a US Food and Drug Administration-approved test. We report a case of multiple discordant HEV serology results in a pediatric liver transplant recipient with idiopathic hepatitis, and review the challenges to diagnosis of HEV infection in the United States.

[Current overview on MERS-CoV]. / Aktueller Überblick zum MERS-Virus.

The Middle East respiratory syndrome coronavirus (MERS-CoV) was discovered for the first time in Saudi Arabia in 2012. On a global scale 1200 infections have been registered with approximately 50 % resulting in a fatal outcome. It is currently not fully clear how MERS-CoV is transmitted and insufficient research has so far been carried out. Taking the sudden increase of cases in South Korea into consideration, this article discusses which preventive measures should be recommended for international travelers in order to prevent infections. A literature search of the most recent publications indicated that appropriate standard hygiene measures are sufficient and no travel restrictions are therefore necessary.

Jumping-catalyst dynamics in nanowire growth.

Nanowire growth is generally considered a steady-state process, but oscillatory phenomena are known to often play a fundamental role. Here we identify a natural sequence of distinct growth modes, in two of which the catalyst droplet jumps periodically on and off a crystal facet. The oscillatory modes result from a mismatch between catalyst size and wire diameter; they enable growth of straight smooth-sided wires even when the droplet is too small to span the wire tip. Jumping-catalyst growth modes are seen both in computer simulations of vapor-liquid-solid growth, and in movies of Si nanowire growth obtained by in situ microscopy. Our simulations also provide new insight into nanowire kinking.

Decay of enteric microorganisms in biosolids-amended soil under wheat (Triticum aestivum) cultivation.

There is a growing need for better assessment of health risks associated with land-applied biosolids. This study investigated in-situ decay of seeded human adenovirus (HAdV), Salmonella enterica, Escherichia coli, and bacteriophage (MS2) in biosolids-amended soil under wheat cultivation. The biosolids seeded with microorganisms were placed in decay chambers which were then placed in the topsoil (10 cm depth) at three different sites. Sites were selected in arid wheat-growing regions of Australia with loamy-sand soil type (Western Australia) and sandy soil (South Australia). Seeded E. coli and S. enterica had a relatively short decay time (T90 = 4-56 days) in biosolids-amended soil compared to un-amended soil (T90 = 8-83 days). The decreasing soil moisture over the wheat-growing season significantly (P < 0.05) influenced survival time of both bacteria and MS2 at Western Australia (Moora) and South Australia (Mt Compass) sites, particularly in the un-amended soils. Increasing soil temperature also significantly (P < 0.05) influenced the decay of MS2 at these sites. In this study, no notable decline in HAdV numbers (PCR detectable units) was observed in both biosolids-amended and the un-amended soils at all three sites. The HAdV decay time (T90 ≥ 180 days) in biosolids-amended and un-amended soils was significantly higher than MS2 (T90 = 22-108 days). The results of this study suggest that adenovirus could survive for a longer period of time (>180 days) during the winter in biosolids-amended soil. The stability of adenovirus suggests that consideration towards biosolids amendment frequency, time, rates and appropriate withholding periods are necessary for risk mitigation.

A new functional assay for the diagnosis of X-linked inhibitor of apoptosis (XIAP) deficiency.

X-linked inhibitor of apoptosis (XIAP) deficiency, caused by mutations in BIRC4, is an immunodeficiency associated with immune dysregulation and a highly variable clinical presentation. Current diagnostic screening tests such as flow cytometry for XIAP expression or lymphocyte apoptosis assays have significant limitations. Based on recent evidence that XIAP is essential for nucleotide-binding and oligomerization domains (NOD)1/2 signalling, we evaluated the use of a simple flow cytometric assay assessing tumour necrosis factor (TNF) production of monocytes in response to NOD2 stimulation by muramyl dipeptides (L18-MDP) for the functional diagnosis of XIAP deficiency. We investigated 12 patients with XIAP deficiency, six female carriers and relevant disease controls. Irrespective of the diverse clinical phenotype, the extent of residual protein expression or the nature of the mutation, the TNF response was severely reduced in all patients. On average, L18-MDP induced TNF production in 25% of monocytes from healthy donors or female carriers, while fewer than 6% of monocytes responded in affected patients. Notably, the assay clearly discriminated affected patients from disease controls with other immunodeficiencies accompanied by lymphoproliferation, hypogammaglobulinaemia or inflammatory bowel disease. Functional testing of the NOD2 signalling pathway is an easy, fast and reliable assay in the diagnostic evaluation of patients with suspected XIAP deficiency.

Successful haploidentical hematopoietic stem cell transplantation in a patient with SCID due to CD3ε deficiency: need for IgG-substitution 6 years later.

BACKGROUND: The CD3 co-receptor complex is essential for signal transduction after specific binding of the T-cell receptor (TCR). CD3E encodes the CD3ε chain, one of the protein components (γ-, δ-, ε- and ζ-chain) of the CD3 co-receptor. As previously reported in one family CD3ε deficiency causes SCID. PATIENT: We report on a patient with SCID due to CD3ε deficiency treated by HLA-haploidentical stem cell transplantation (SCT) (donor: mother) 15 years ago which resulted in development of normal T- and B-cell immunity. Despite conditioning donor cell engraftment was confined to T cells, while all other blood cell lineages remained of patient origin (split chimerism). In spite of normal functions, T-cell numbers never reached normal levels and naïve CD45+RA+ T-cells remained low. At 6 years after SCT the patient developed signs of humoral immunodeficiency, requiring regular substitution of IgG. RESULTS: In a retrospective genetic work up 11 years after SCT, a homozygous splice site mutation in CD3E was identified resulting in the loss of CD3ε protein. The loss of B-cell function as observed in the patient was reflected by a lack of switched memory B cells. To rule out a primary role of CD3ε in B-cell function we studied expression of CD3E in B-cells which was found not to be expressed. DISCUSSION: The clinical presentation of a secondary loss of specific humoral immunity in this constellation of split chimerism after allogeneic haploidentical SCT is unusual and unexpected in a patient with a primary T-cell defect. A most likely explanation is the gradual loss of T-helper-cell function.