Correction: Tuning the melting point of selected ionic liquids through adjustment of the cation's dipole moment.

Correction for 'Tuning the melting point of selected ionic liquids through adjustment of the cation's dipole moment' by Brooks D. Rabideau et al., Phys. Chem. Chem. Phys., 2020, 22, 12301-12311, https://doi.org/10.1039/D0CP01214A.

Tuning the melting point of selected ionic liquids through adjustment of the cation's dipole moment.

In previous work with thermally robust salts [Cassity et al., Phys. Chem. Chem. Phys., 2017, 19, 31560] it was noted that an increase in the dipole moment of the cation generally led to a decrease in the melting point. Molecular dynamics simulations of the liquid state revealed that an increased dipole moment reduces cation-cation repulsions through dipole-dipole alignment. This was believed to reduce the liquid phase enthalpy, which would tend to lower the melting point of the IL. In this work we further test this principle by replacing hydrogen atoms with fluorine atoms at selected positions within the cation. This allows us to alter the electrostatics of the cation without substantially affecting the sterics. Furthermore, the strength of the dipole moment can be controlled by choosing different positions within the cation for replacement. We studied variants of four different parent cations paired with bistriflimide and determined their melting points, and enthalpies and entropies of fusion through DSC experiments. The decreases in the melting point were determined to be enthalpically driven. We found that the dipole moment of the cation, as determined by quantum chemical calculations, is inversely correlated with the melting point of the given compound. Molecular dynamics simulations of the crystalline and solid states of two isomers showed differences in their enthalpies of fusion that closely matched those seen experimentally. Moreover, this reduction in the enthalpy of fusion was determined to be caused by an increase in the enthalpy of the crystalline state. We provide evidence that dipole-dipole interactions between cations leads to the formation of cationic domains in the crystalline state. These cationic associations partially block favourable cation-anion interactions, which are recovered upon melting. If, however, the dipole-dipole interactions between cations is too strong they have a tendency to form glasses. This study provides a design rule for lowering the melting point of structurally similar ILs by altering their dipole moment.

Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life.

BACKGROUND: With recent conservative strategies, prognosis of patients with desmoid-type fibromatosis (DTF) is about function preservation. We analyzed the long-term quality of life (QoL) of pediatric patients with DTF. METHODS: All French young patients (<21years) treated between 2005 and 2016 for a DTF in the EpSSG NRSTS-05 study were analyzed. A first wait-and-see strategy was recommended. Patients' QoL was analyzed with the internationally validated Child Health Questionnaire (CHQ). We focused on the relevant subscales scores: physical functioning (PF), role social limitations physical (RP), bodily pain (BP), general health perception (GH) and physical (PhS) and psychosocial (PsS) summary measures. RESULTS: Among the 81 patients, 52 families answered the CHQ (median delay since diagnosis = 6.2years; min2.2-max13.3 years). Median age at diagnosis was 11.5 years. Primary site: limbs (52%), head/neck (27%), or trunk (21%). Five year-Progression Free Survival was 39.1% (95%CI: 27.7-50.5%). As initial management for these 52 patients, 30 patients were first observed (57%), 13 had surgery (25%) and 9 received chemotherapy (18%). Total burden of therapy was exclusive surgery (9pts/18%), exclusive chemotherapy (18pts/35%), surgery + chemotherapy (13pts/25%), chemotherapy + radiotherapy (1 pt), surgery + chemotherapy + radiotherapy (1 pt), wait and see (10 pt). Regarding the parent forms, patients have significant lower PF (86.0vs.96.1; p = 0.03), RP (82.0vs.93.6; p = 0.04), GH (60vs.73; p < 0.005) and PhS (46.2 vs.53; p = 0.02) scores compared to healthy population. Comparison of QoL subscales scores according to initial strategy (wait-and-see vs.surgery/chemotherapy) did not reveal any difference (PF = 87.3vs.84.9; p = 0.80/RP = 83.4vs.78.7; p = 0.72/BP = 78.9vs.78.2; p = 0.95/GH = 59.7vs60; p = 0.97). Similar results were found using the children or adult forms. CONCLUSIONS: Initial wait-and-see strategy does not affect long term functional impairment.

Is alpha-fetoprotein decline a prognostic factor of childhood non-seminomatous germ cell tumours? Results of the French TGM95 study.

PURPOSE: In adults' non-seminomatous germ cell tumours (NS-GCT), alpha-fetoprotein (AFP) decline was identified as an important prognostic factor. We investigated its prognostic value in the French TGM95 study for childhood NS-GCT. PATIENTS AND METHODS: Three risk groups were defined: low risk (LR: localised and completely resected pS1, AFP<15000 ng/ml), with a 'wait-and-see' strategy; intermediate-risk (IR: localised incompletely resected, AFP<15000 ng/ml) with 3-5 vinblastine-bleomycine-cisplatin courses; high risk (HiR: AFP≥15000 ng/ml and/or metastatic) with 4-6 etoposide-ifosfamide-cisplatin courses. The multivariable prognostic analysis for progression-free survival (PFS) included age (±10 years), primary tumour site (1-testis, 2-ovary, 3-extragonadal), extent of disease (1-pS1, 2-loco-regional dissemination, 3-metastasis) and AFP (±10,000 ng/ml). AFP decline prognostic value was investigated in IR + HiR groups using predicted time to normalisation (TTN), AFP change, and difference between observed and expected (based on AFP half-life) area under the curve (O-E AUC). RESULTS: From January 1995 to December 2005, 239 patients (median age = 3years, 60 LR, 65 IR, 114 HiR) were included. Main sites were testis (n = 66), ovary (n = 77) and sacrococcygeal (n = 57). Five-year PFS and OS were 85% (95% confidence interval [CI] = 80-89%) and 93% (89-95%), respectively. Age ≥ 10 years (hazard ratio [HR] = 4.6, 95% CI = 2.1-10.1, p = 0.0001) and extragonadal primary (HR = 6.3, 95% CI = 2.0-19.9, p = 0.005) were significant prognostic factors. In AFP decline analysis (n = 151, 17 events), TTN (p = 0.61) and AFP change (p = 0.10) were not prognostic, whereas we showed a significant effect of O-E AUC (HR = 2.1, 95% CI = 1.0-4.2, p = 0.05). CONCLUSION: Age ≥ 10 years and extragonadal tumours remain as poor prognostic factors. Contrary to adults, TTN is not reliable in paediatric NS-GCT. The prognostic value of O-E AUC should be investigated in larger studies.

St Gallen molecular subtypes in screening-detected and symptomatic breast cancer in a prospective cohort with long-term follow-up.

BACKGROUND: Diagnosis by screening mammography is considered an independent positive prognostic factor, although the data are not fully in agreement. The aim of the study was to explore whether the mode of detection (screening-detected versus symptomatic) adds prognostic information to the St Gallen molecular subtypes of primary breast cancer, in terms of 10-year cumulative breast cancer mortality (BCM). METHODS: A prospective cohort of patients with primary breast cancer, who had regularly been invited to screening mammography, were included. Tissue microarrays were constructed from primary tumours and lymph node metastases, and evaluated by two independent pathologists. Primary tumours and lymph node metastases were classified into St Gallen molecular subtypes. Cause of death was retrieved from the Central Statistics Office. RESULTS: A total of 434 patients with primary breast cancer were included in the study. Some 370 primary tumours and 111 lymph node metastases were classified into St Gallen molecular subtypes. The luminal A-like subtype was more common among the screening-detected primary tumours (P = 0·035) and corresponding lymph node metastases (P = 0·114) than among symptomatic cancers. Patients with screening-detected tumours had a lower BCM (P = 0·017), and for those diagnosed with luminal A-like tumours the 10-year cumulative BCM was 3 per cent. For patients with luminal A-like lymph node metastases, there was no BCM. In a stepwise multivariable analysis, the prognostic information yielded by screening detection was hampered by stage and tumour biology. CONCLUSION: The prognosis was excellent for patients within the screening programme who were diagnosed with a luminal A-like primary tumour and/or lymph node metastases. Stage, molecular pathology and mode of detection help to define patients at low risk of death from breast cancer.

[Paratesticular rhabdomyosarcoma in children: a scrotal emergency]. / Le rhabdomyosarcome paratesticulaire de l'enfant : une urgence scrotale.

Paratesticular rhabdomyosarcoma is a rare and highly aggressive embryonal tumor in infancy and childhood. The tumor is intrascrotal, localized in the spermatic cord, the epididymis, or in the tunica vaginalis. Rhabdomyosarcoma represents 10% of testicular tumors of infancy. On physical examination, a painless scrotal tumefaction is observed. A surgical and pathological classification is used to group patients according to the extent of residual tumor after the initial surgical procedure: Intergroup Rhabdomyosarcoma Study classification (IRS). Multimodality therapy involving surgery, chemotherapy, and radiotherapy is necessary. Depending on the extent of disease and the staging group, the approach of treatment is different. The most important factors affecting treatment outcome are the stage, the pathological subtype of the tumor, and the age of the patient. Younger patients (<10 years) with a local tumor and with embryonal pathology have an excellent prognosis. We report the case of a 6-year-old boy admitted for a specialist consultation because of the recent appearance of a tumor in the right scrotum. On physical examination, a painless, solid, right scrotal mass was noted and the diagnosis of paratesticular rhabdomyosarcoma was made. This diagnosis can be suspected on physical and on ultrasound examinations, but only a pathological examination will confirm it. The authors discuss the therapeutic issues raised by this lesion and report one case of paratesticular rhabdomyosarcoma.

Head and neck manifestation and prognosis of Langerhans' cell histiocytosis in children.

OBJECTIVE: To appreciate the several head and neck manifestations of Langherans' cell histiocytosis (LCH) in children and their multidisciplinary management and outcome. STUDY DESIGN: Retrospective study. PATIENTS AND METHODS: Clinical reports of 42 patients with LCH treated in the Departments of Paediatric Haematology, Paediatric Oncology and Paediatric Otorhinolaryngology of a tertiary care center were analyzed. Only cases where the disease was localized to the head and neck were considered. The age at diagnosis, gender, clinical presentation, extension of disease as well as response to treatment and outcome were recorded from the charts of each of these patients. RESULTS: Of the 42 patient charts reviewed, 31 (73.8%) presented with head and neck localization. 10 of these had an exclusive head and neck presentation. Multisystem LCH was mostly found in infants under 3-year-old (mean age: 2-year-old), and bony manifestations in older. All treatments delivered to patients were well-tolerated and the evolution good. DISCUSSION AND CONCLUSION: Head and neck involvement is known to be very frequent in LCH. There is no consensus about treatment but authors highlight that all teams in charge of patients presenting with LCH agree to remain as conservative as possible. For solitary large lesions looking like a tumor which resection could result in functional or cosmetic morbidity, it would be important to get first a biopsy. For multisystemic LHC, therapeutic trials with chemotherapy agents still in process should increase the rate of success.

Fibrin glue application in the management of refractory chylous ascites in children.

The purpose of this retrospective review of the charts of 6 children who underwent surgical treatment of chylous ascites refractory to conservative measures between 1993 and 2006 was to evaluate the efficiency of fibrin glue application for control of lymph leakage. Five children had postoperative chylous ascites (neuroblastoma, 4; cystic lymphangioma, 1) and 1 had a congenital malformation. Surgical exploration revealed large areas of diffuse lymphatic leakage in all of the patients. Lymphatic fistula was not identified intraoperatively in any patient. Ingestion of lipophilic dye in a concentrated fatty meal was not helpful in locating a lymph fistula. Absorbable mesh was used in association with glue application in the last 3 patients treated. Control of ascites was achieved immediately in 2 patients and within 3 weeks in 2 patients. Repeat surgery was required in the remaining 2 patients. The mean follow-up time was 4.3 years. One patient died of tumor recurrence 12 months after surgical treatment without relapse of the ascites. Two mild late recurrences were observed at 6 and 11 months after surgery and were managed conservatively. The findings of this study show that fibrin glue application on absorbable mesh after dissection of the leakage zones is easy, safe, and effective. We recommend that surgery with glue application be repeated until control of ascites is achieved. We suggest fibrin glue application as a preventive measure against postoperative chylous ascites.

[Evaluation of a screening strategy after occurrence of two simultaneous contaminating tuberculosis cases in a pediatric oncology department]. / Stratégie de prise en charge de contages tuberculeux dans un service d'oncologie-pédiatrique.

BACKGROUND: A medical staff and an administrative staff of our paediatric oncology department have contracted a pulmonary tuberculosis. This is a rare situation and the management of this infection threat in a paediatric oncology department is not clearly defined. Recommendations tell we must treat all patients. Nevertheless, antituberculosis agent expose to increased toxic effects and immunocompromised patients have an increased risk of experiencing progression of latent mycobacterium tuberculosis infection to active tuberculosis disease. OBJECTIVE: This study aims at the evaluation of a screening and a treatment strategy adapted for a paediatric oncology department. METHOD: From April 2004 to April 2005, 80 children with a solid tumour were screened for tuberculosis according to a screening and treatment protocol established by a multidisciplinary committee. Two risk groups were defined according to age and immunodepression status. The "high risk" group is composed of less than 2 years old children and children who underwent an haematological peripheral stem cell transplantation. All other children were included in the "low risk" group. The screening was based on clinical, biological and radiological data performed three times spaced out by 2 or 3 months. At the end of each part of screening, the multidisciplinary committee analyzed the results and discussed the utility of an antituberculosis treatment. RESULTS: 80 children (31 boys and 49 girl) with a median age of 7,3 years (0,3-24) participated to the screening. Sixty children were still undergoing anticancer treatments. Twenty belonged to the high risk group. The complete screening was performed in 32% of the patients. Three antituberculosis' treatment were initiated: 2 for prophylaxis purpose and 1 for a tuberculosis prime-infection. A child had an additional check-up because of an abnormal chest X-ray. Our management strategy allowed us to treat significantly less patients when compared to national guidelines (3 vs 80 test Chi-2 p<0.001). No side effects of antituberculosis agents were noted. No tuberculosis has been observed in our population 28 months after the completion of the treatment. CONCLUSION: The proposed screening allowed us to treat a minimum of children and thus, to reduce the potential toxicity induced by antituberculosis' treatments.

[Kystic pleuropulmonary blastoma fortuitly discovered in an infant]. / Pleuropneumoblastome kystique de découverte fortuite chez un nourrisson.

Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, associated with a poor outcome. We report the case of a 7 week-old boy with a pleuropulmonary blastoma classified as type I. Disease was discovered at a chest X-ray performed as a work-up for a benign acute viral bronchiolitis. The final diagnosis was brought by pathology: a bronchopulmonary malformation had not been ruled out by clinical, radiological and macroscopic findings. Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, for which the poor outcome and tough diagnosis justify a surgical attitude when an intrathoracic bullous lesion is found in an infant.

[Antimitochondrial agents: a new class of anticancer agents]. / Contribution de la Société francophone de recherche en pédiatrie (SFRP). "Les agents antimitochondriaux: une nouvelle classe d'agents anticancéreux".

Over the last 2 decades, the role of apoptosis in anticancer agent cytotoxicity has become clear. Defects in the regulation of apoptosis (programmed cell death) make important contributions to the pathogenesis and progression of most cancers and leukemias. Apoptosis defects also have a key role in cell resistance to chemotherapy. Mitochondria play a central part in cell death in response to anticancer agents. Most of these agents target mitochondria via caspases or other regulator elements of the apoptotic machinery. Nevertheless, some anticancer agents, already in clinical use (paclitaxel, vinblastine, lonidamine, etoposide, arsenic trioxide) or in pre-clinical development (betulinic acid, MT21), directly target and permeabilize mitochondria. The acknowledgement of mitochondria as a new target for anticancer agents provides a new way to bypass cancer cell chemoresistance.

Allogeneic bone marrow transplantation for chronic myeloid leukemia in childhood: a report from the Société Française de Greffe de Moelle et de Thérapie Cellulaire (SFGM-TC).

To determine the results of allogeneic hematopoietic stem cell (HSC) transplantation for chronic myelogenous leukemia (CML) at various stages of the disease in children, a retrospective analysis was carried out on the outcome of transplants performed on 76 children and teenagers with CML between 1982 and 1998. In all, 60 patients were transplanted from a matched sibling donor (MSD) and 16 from a matched unrelated donor (MUD). There was a higher incidence of acute graft-versus-host disease after MUD transplantation (P<10(-3)). The main cause of death was transplant-related toxicity in both groups. In MSD recipients, the probability of relapse at 5 years for patients transplanted in the first chronic phase was lower than in patients transplanted in the advanced phase (relative risk (rr)=5.90; 95% confidence interval (CI), 1.85-18.82, P<0.01). The estimated 5-year event-free survival (EFS) rate was higher after MSD vs MUD transplantation (61% (95% CI, 48-73%) vs 27% (95% CI, 4-49%), rr=0.25, P<10(-3)). In children transplanted from MSD, the 5-year EFS was higher when transplantation was performed in the first chronic phase vs the advanced phases (73% (95% CI, 59-87%) vs 32% (95% CI, 10-54%), P<10(-3)). Disease status at transplantation was the unique factor influencing survival in patients undergoing transplantation from MSD with a better outcome for those transplanted in the first chronic phase. Allogeneic HSC offers a possibility of curing childhood CML with a significant advantage for patients transplanted in chronic phase using a human leukocyte antigen-identical sibling donor.

Pharmacological investigations of the vasodilator nerves supplying the duck's foot.

Vasodilator responses to electrical nerve stimulation and to various putative autonomic inhibitory neurotransmitters were studied in the Krebs-perfused foot of the domestic duck. Electrical stimulation resulted in frequency-dependent vasoconstrictor responses which were abolished by the infusion of guanethidine. After abolition of the vasoconstriction, electrical stimulation produced a frequency-dependent vasodilatation. The putative inhibitory transmitter substances tested were substance P, somatostatin, vasoactive intestinal polypeptide, adenosine triphosphate and dopamine. Of these only dopamine produced dilator responses similar in appearance to those following nerve stimulation. Infusion of metoclopramide (170 microM) greatly reduced dilator responses to nerve stimulation and to dopamine but not those to glyceryl trinitrate. These results suggest that the vasculature of the duck foot may be supplied by dopaminergic vasodilator nerves.

Catecholamines in the sympathetic nervous system of the domestic fowl.

In order to resolve some existing uncertainties regarding the identity of the sympathetic catecholamine neurotransmitter in birds, we have measured endogenous noradrenaline (NA), adrenaline (AD) and dopamine (DA) in various peripheral tissues and in the paravertebral sympathetic chain of the domestic fowl. In all visceral tissues examined - atrium, spleen, mesenteric artery, gizzard, intestine, rectal caecum and kidney--NA concentrations were much higher than those of AD or DA. The ratios of NA:AD:DA were approximately 100:10:1 for all tissues except spleen, in which DA represented about 10% of the total catecholamines. In the paravertebral chain, the ratio of NA:DA was about 4:1, with no consistent segmental differences. Ganglionic AD was generally less than 1% of the total catecholamine, but isolated ganglia sometimes contained quite large amounts of AD. This AD was probably in chromaffin cells. The results obtained indicate that, contrary to some previous reports, NA constitutes the predominant sympathetic neurotransmitter in the fowl, and neurons in which AD is a transmitter are rare or absent. Although the relatively large amounts of DA present in the ganglia could be due to presence of dopaminergic neurons, no evidence for peripheral projections of such neurons was obtained.