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Histopathological information about “anorectal malformations” is scarce and the pathogenesis still controversial. Autopsy specimens of 20 human fetuses and newborns with “main” types of the disorder were studied histologically. Supplemented with surgical-anatomical data from the literature and with information from our own and earlier embryological research in animal models as well as from recent observations on the normal development of the human perineum, the study allowed for a new reconstruction of the pathogenesis of the disorder. The histological analysis of the malformations in human fetuses and newborns showed a ventralward deviation of the anal canal as the principal deformity. Ano-urogenital communications and differently structured ectopic anocutaneous canals issued from anywhere between the bladder and the vestibular/urethral orifice (female urethra excluded) and between the orifice and the usual site of the anus, respectively, or they ended blindly, but with a suggestion of lost communication. They occurred isolated or in association with other primary or secondary regional anomalies. Patho-embryological data from animal models revealed that the deviation was caused by defective development of the dorsal cloaca and not by disturbances in a series of fusion processes inside and outside the cloaca, as is currently believed. This mechanism fits well into recent adjustments of ideas about the normal development of the perineum. The cause of the defect is still obscure, but a malfunctioning of cells ingressing from an end-stage primitive streak that affects the dorsal side of the prospective cloaca appears most likely. The data collected permit a new reconstruction of the pathogenesis of anal and ano-urogenital malformations.

Differentiation between cutaneous pseudo-T-cell lymphomas and cutaneous T-cell lymphomas (CTCLs) may be extremely difficult. In this study, it was investigated whether demonstration of an aberrant phenotype and detection of clonal T-cell receptor gamma (TCR gamma) gene rearrangements can be used as additional differential diagnostic criteria. Immunohistochemical studies and TCR gamma gene rearrangement analysis using a polymerase chain reaction with primers specific for V gamma 1-8 and V gamma 9 gene segments in combination with denaturing gradient gel electrophoresis (PCR/ DGGE) were performed on frozen material of 11 pseudo-T-cell lymphomas and 17 CTCLs, including 9 cases of mycosis fungoides (MF) and 8 pleomorphic CTCLs. Clonal TCR gamma gene rearrangements were found in 66% of patch/plaque-stage MF and 100% of tumor-stage MF and pleomorphic CTCL, but not in any of 10 pseudo-T-cell lymphomas studied. Aberrant expression of CD2, CD3, and/or CD5 antigens was noted in 3 of 6 (50%) cases of patch/plaque-stage MF, all three cases of tumor-stage MF, and 5 of 8 (62%) pleomorphic CTCLs, but not in any of the 11 pseudo-T-cell lymphomas. Moreover, in pseudo-T-cell lymphomas exhibiting a nodular or diffuse growth pattern, a considerable admixture with reactive CD8+ T cells (15 to 60%), B cells (up to 20%), and macrophages was a characteristic finding. In conclusion, the results of this study suggest that demonstration of clonal TCR gene rearrangements and an aberrant phenotype, as well as demonstration of many admixed CD8+ T cells and B cells can be considered as useful additional criteria in the differentiation between pseudo-T-cell lymphomas and CTCLs.

Twelve quasi-identical almshouses with an architectural-historic value were reno-vated, because of their high energy use, poor indoor comfort and numerous moisture problems. Aerogel plaster was applied for the hygrothermal upgrade of the uninsulated brick walls, while limiting the reduction of living space in these very small houses and keeping the monumental character of the facades in their original state. Several quality assurance tests were executed to evaluate the quality of the renovation of the building envelope and to compare the results with the initial theoretical design calculations. It appeared that the existing materials performed considerably better than the assumed conservative default values. On the other hand, the newly installed insulation materials performed somewhat less than declared, for example due to on-site processing. This resulted in a strong overestimation of the improvement in thermal performance by the renovation works. Field measurements of the existing situation can help to close this gap.

Current opinion about structure and pathogenesis of cloacal exstrophy was challenged by histopathological findings and new insights into the normal development. Autopsy specimens of common (n = 3) and covered cloacal exstrophy (n = 4) with single intraexstrophic and -perineal phallic structures and perineo-exstrophic canals have been analyzed histopathologically. The findings were correlated to normal development to reconstruct the pathogenesis. By identifying a specific cloaca-derived urethra field as distinct from allantois-derived bladder fields, the exstrophic area is found to reflect the original hindgut configuration in embryos of approximately 26–29 postovulatory days gestational age (2–4 mm). Correlation to normal development suggests malfunctioning of the primitive streak/caudal eminence as a primary fault that leads to a defective cloacal region in the hindgut disturbing cloacal-intestinal-allantoic dissociation and also causes lengthening of the intestinal region into a blind-ending colon, teratoma-like lesions, and vertebral and muscular anomalies. The current idea that membranes in “covered cloacal exstrophy” represent persisting cloacal membranes is dismissed by finding an amnion-like structure, which suggests dysfunction of an umbilical ring placode as a simultaneous 2nd fault. This malfunctioning may cause omphalocele by defective demarcation of the umbilical cord and may replace midline stroma of the infraumbilical abdominal wall by extraembryonic tissue that stretches into a weak temporary membrane, may leave a perineo-extrophic canal, and may allow the formation of a single perineal or intraexstrophic phallus. Malfunctioning without replacement may result in a purely epithelial “allantoic” membrane, which by disintegrating in combination with the cloacal membrane will expose common cloacal exstrophy.

Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized clinically by the evolution of circumscribed erythematous lax skin masses, especially in the body folds, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers. GSS is often associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis fungoides (MF) and Hodgkin's disease (HD). No effective treatment is known yet. Whether this entity is a benign disorder, a peculiar host reaction to a malignant lymphoma, a precursor of malignant lymphoma or an indolent cutaneous T-cell lymphoma (CTCL) in itself is still a matter of debate. The results of the patients with GSS from the Netherlands are compared with the cases reported in the world literature. A female patient had had GSS for 8 years without developing a secondary malignancy. In a second female patient with a histologically confirmed diagnosis of MF, GSS developed 18 years later in the axillary and inguinal folds which had previously been affected by plaque-stage MF lesions. A third male patient with a 6-year history of erythematosquamous skin disease diagnosed as CTCL developed GSS. Moreover, granuloma formation was also found in a facial basal cell carcinoma, in a cervical lymph node and the spleen. Clonal rearrangements of the T-cell receptor beta genes were found in the 2 female patients; the male patient could not be tested. GSS is a rare clinicopathological entity. Only 34 patients have been described so far. The development of GSS within plaque MF lesions has not been reported before. Our third case developed very extensive skin lesions and showed a strong propensity to develop granulomas as compared to cases reported before. The presence of a clonal T-cell population was demonstrated in all cases tested. Our cases support the idea that GSS is a very rare and rather indolent type of CTCL. Apparently, the disease is associated with a peculiar immune response, characterized by granuloma formation and disappearance of elastic fibers resulting in the lax skin. The relationship between GSS and other preexisting or subsequent lymphoproliferative diseases (diagnosed in approximately 50% of the cases) warrants a life-long follow-up.

A search for exotic decays of the Higgs boson ( ) with a mass of 125 to a pair of light pseudoscalars is performed in final states where one pseudoscalar decays to two quarks and the other to a pair of muons or leptons. A data sample of proton-proton collisions at corresponding to an integrated luminosity of 138 recorded with the CMS detector is analyzed. No statistically significant excess is observed over the standard model backgrounds. Upper limits are set at 95% confidence level ( ) on the Higgs boson branching fraction to and to via a pair of s. The limits depend on the pseudoscalar mass and are observed to be in the range (0.17-3.3)  and (1.7-7.7)  in the and final states, respectively. In the framework of models with two Higgs doublets and a complex scalar singlet (2HDM+S), the results of the two final states are combined to determine upper limits on the branching fraction at 95% , with being a muon or a lepton. For different types of 2HDM+S, upper bounds on the branching fraction are extracted from the combination of the two channels. In most of the Type II 2HDM+S parameter space, values above 0.23 are excluded at 95% for values between 15 and 60 .

The double differential cross sections of the Drell-Yan lepton pair ( , dielectron or dimuon) production are measured as functions of the invariant mass , transverse momentum , and . The observable, derived from angular measurements of the leptons and highly correlated with , is used to probe the low- region in a complementary way. Dilepton masses up to 1 are investigated. Additionally, a measurement is performed requiring at least one jet in the final state. To benefit from partial cancellation of the systematic uncertainty, the ratios of the differential cross sections for various ranges to those in the Z mass peak interval are presented. The collected data correspond to an integrated luminosity of 36.3 of proton-proton collisions recorded with the CMS detector at the LHC at a centre-of-mass energy of 13 . Measurements are compared with predictions based on perturbative quantum chromodynamics, including soft-gluon resummation.

The production of Z bosons associated with jets is measured in collisions at with data recorded with the CMS experiment at the LHC corresponding to an integrated luminosity of 36.3 . The multiplicity of jets with transverse momentum is measured for different regions of the Z boson's , from lower than 10 to higher than 100 . The azimuthal correlation between the Z boson and the leading jet, as well as the correlations between the two leading jets are measured in three regions of . The measurements are compared with several predictions at leading and next-to-leading orders, interfaced with parton showers. Predictions based on transverse-momentum dependent parton distributions and corresponding parton showers give a good description of the measurement in the regions where multiple parton interactions and higher jet multiplicities are not important. The effects of multiple parton interactions are shown to be important to correctly describe the measured spectra in the low regions.

A search for decays to invisible particles of Higgs bosons produced in association with a top-antitop quark pair or a vector boson, which both decay to a fully hadronic final state, has been performed using proton-proton collision data collected at by the CMS experiment at the LHC, corresponding to an integrated luminosity of 138 . The 95% confidence level upper limit set on the branching fraction of the 125 Higgs boson to invisible particles, , is 0.54 (0.39 expected), assuming standard model production cross sections. The results of this analysis are combined with previous searches carried out at , 8, and 13 in complementary production modes. The combined upper limit at 95% confidence level on is 0.15 (0.08 expected).

A search is reported for pairs of light Higgs bosons ( ) produced in supersymmetric cascade decays in final states with small missing transverse momentum. A data set of LHC collisions collected with the CMS detector at and corresponding to an integrated luminosity of 138 is used. The search targets events where both bosons decay into pairs that are reconstructed as large-radius jets using substructure techniques. No evidence is found for an excess of events beyond the background expectations of the standard model (SM). Results from the search are interpreted in the next-to-minimal supersymmetric extension of the SM, where a \"singlino\" of small mass leads to squark and gluino cascade decays that can predominantly end in a highly Lorentz-boosted singlet-like and a singlino-like neutralino of small transverse momentum. Upper limits are set on the product of the squark or gluino pair production cross section and the square of the branching fraction of the in a benchmark model containing almost mass-degenerate gluinos and light-flavour squarks. Under the assumption of an SM-like branching fraction, bosons with masses in the range 40-120 arising from the decays of squarks or gluinos with a mass of 1200-2500 are excluded at 95% confidence level.