Mowat-Wilson syndrome: growth charts

Background Mowat-Wilson syndrome (MWS; OMIM #235730) is a genetic condition caused by heterozygous mutations or deletions of theZEB2gene. It is characterized by moderate-severe intellectual disability, epilepsy, Hirschsprung disease and multiple organ malformations of which congenital heart defects and urogenital anomalies are the most frequent ones. To date, a clear description of the physical development of MWS patients does not exist. The aim of this study is to provide up-to-date growth charts specific for infants and children with MWS. Charts for males and females aged from 0 to 16 years were generated using a total of 2865 measurements from 99 MWS patients of different ancestries. All data were collected through extensive collaborations with the Italian MWS association (AIMW) and the MWS Foundation. The GAMLSS package for the R statistical computing software was used to model the growth charts. Height, weight, body mass index (BMI) and head circumference were compared to those from standard international growth charts for healthy children. Results In newborns, weight and length were distributed as in the general population, while head circumference was slightly smaller, with an average below the 30th centile. Up to the age of 7 years, weight and height distribution was shifted to slightly lower values than in the general population; after that, the difference increased further, with 50% of the affected children below the 5th centile of the general population. BMI distribution was similar to that of non-affected children until the age of 7 years, at which point values in MWS children increased with a less steep slope, particularly in males. Microcephaly was sometimes present at birth, but in most cases it developed gradually during infancy; many children had a small head circumference, between the 3rd and the 10th centile, rather than being truly microcephalic (at least 2 SD below the mean). Most patients were of slender build. Conclusions These charts contribute to the understanding of the natural history of MWS and should assist pediatricians and other caregivers in providing optimal care to MWS individuals who show problems related to physical growth. This is the first study on growth in patients with MWS

Therapeutic fascism: re-educating Communists in Nazi-occupied Serbia, 1942-44

This article probes the relationship between psychoanalysis and right-wing authoritarianism, and analyses a unique psychotherapeutic institution established by Serbia’s World War II collaborationist regime. The extraordinary Institute for compulsory re-education of high-school and university students affiliated with the Communist resistance movement emerged in the context of a brutal civil war and violent retaliations against Communist activists, but its openly psychoanalytic orientation was even more astonishing. In order to stem the rapid spread of Communism, the collaborationist state, led by its most extreme fascistic elements, officially embraced psychotherapy, the ‘talking cure’ and Freudianism, and conjured up its own theory of mental pathology and trauma – one that directly contradicted the Nazi concepts of society and the individual. In the course of the experiment, Serbia’s collaborationists moved away from the hitherto prevailing organicist, biomedical model of mental illness, and critiqued traditional psychiatry’s therapeutic pessimism