Complete regress of a low-differentiated recurrence of the bladder cancer as the answer on two cycles of neoadjuvant polychemotherapy

Complete regress of a low-differentiated recurrence of the bladder cancer as the answer on two cycles of neoadjuvant polychemotherap

Hormonal control during infancy and testicular adrenal rest tumor development in males with congenital adrenal hyperplasia: a retrospective multicenter cohort study

IMPORTANCE Testicular adrenal rest tumors (TARTs), often found in male patients with congenital adrenal hyperplasia (CAH), are benign lesions causing testicular damage and infertility. We hypothesize that chronically elevated adrenocorticotropic hormone exposure during early life may promote TART development. OBJECTIVE This study aimed to examine the association between commencing adequate glucocorticoid treatment early after birth and TART development. DESIGN AND PARTICIPANTS This retrospective multicenter (n = 22) open cohort study collected longitudinal clinical and biochemical data of the first 4 years of life using the I-CAH registry and included 188 male patients (median age 13 years; interquartile range: 10-17) with 21-hydroxylase deficiency (n = 181) or 11-hydroxylase deficiency (n = 7). All patients underwent at least 1 testicular ultrasound. RESULTS TART was detected in 72 (38%) of the patients. Prevalence varied between centers. When adjusted for CAH phenotype, a delayed CAH diagnosis of >1 year, compared with a diagnosis within 1 month of life, was associated with a 2.6 times higher risk of TART diagnosis. TART onset was not predicted by biochemical disease control or bone age advancement in the first 4 years of life, but increased height standard deviation scores at the end of the 4-year study period were associated with a 27% higher risk of TART diagnosis. CONCLUSIONS AND RELEVANCE A delayed CAH diagnosis of >1 year vs CAH diagnosis within 1 month after birth was associated with a higher risk of TART development, which may be attributed to poor disease control in early life

Euclidean Gibbs states of interacting quantum anharmonic oscillators

A rigorous description of the equilibrium thermodynamic properties of an infinite system of interacting $\nu$-dimensional quantum anharmonic oscillators is given. The oscillators are indexed by the elements of a countable set $\mathbb{L}\subset \mathbb{R}^d$, possibly irregular; the anharmonic potentials vary from site to site. The description is based on the representation of the Gibbs states in terms of path measures -- the so called Euclidean Gibbs measures. It is proven that: (a) the set of such measures $\mathcal{G}^{\rm t}$ is non-void and compact; (b) every $\mu \in \mathcal{G}^{\rm t}$ obeys an exponential integrability estimate, the same for the whole set $\mathcal{G}^{\rm t}$; (c) every $\mu \in \mathcal{G}^{\rm t}$ has a Lebowitz-Presutti type support; (d) $\mathcal{G}^{\rm t}$ is a singleton at high temperatures. In the case of attractive interaction and $\nu=1$ we prove that $|\mathcal{G}^{\rm t}|>1$ at low temperatures. The uniqueness of Gibbs measures due to quantum effects and at a nonzero external field are also proven in this case. Thereby, a qualitative theory of phase transitions and quantum effects, which interprets most important experimental data known for the corresponding physical objects, is developed. The mathematical result of the paper is a complete description of the set $\mathcal{G}^{\rm t}$, which refines and extends the results known for models of this type.Comment: 60 page

Gonadectomy in conditions affecting sex development: a registry-based cohort study

Objectives To determine trends in clinical practice for individuals with DSD requiring gonadectomy. Design Retrospective cohort study. Methods Information regarding age at gonadectomy according to diagnosis; reported sex; time of presentation to specialist centre; and location of centre from cases reported to the International DSD Registry and who were over 16 years old in January 2019. Results Data regarding gonadectomy were available in 668 (88%) individuals from 44 centres. Of these, 248 (37%) (median age (range) 24 (17, 75) years) were male and 420 (63%) (median age (range) 26 (16, 86) years) were female. Gonadectomy was reported from 36 centres in 351/668 cases (53%). Females were more likely to undergo gonadectomy (n = 311, P < 0.0001). The indication for gonadectomy was reported in 268 (76%). The most common indication was mitigation of tumour risk in 172 (64%). Variations in the practice of gonadectomy were observed; of the 351 cases from 36 centres, 17 (5%) at 9 centres had undergone gonadectomy before their first presentation to the specialist centre. Median age at gonadectomy of cases from high-income countries and low-/middle-income countries (LMIC) was 13.0 years (0.1, 68) years and 16.5 years (1, 28), respectively (P < 0.0001) with the likelihood of long-term retention of gonads being higher in LMIC countries. Conclusions The likelihood of gonadectomy depends on the underlying diagnosis, sex of rearing and the geographical setting. Clinical benchmarks, which can be studied across all forms of DSD will allow a better understanding of the variation in the practice of gonadectomy