Communication during childhood cancer: Systematic review of patient perspectives.

Effective communication is challenging in childhood cancer, where decisions carry unpredictable and life-threatening implications. We aimed to describe patients' experiences of communicating with clinicians during treatment of childhood cancer. A systematic review of qualitative studies to April 2019 was performed. Eligible studies included patients diagnosed with cancer at age ≤ 18 years and reported their perspectives of communicating with clinicians during treatment of childhood cancer. Data were extracted from primary studies for thematic synthesis. From 101 articles across 25 countries involving 1870 participants who were diagnosed with cancer between ages 3 to 18 years, we identified 6 themes: 1) rendered invisible and powerless (displaced and undermined by adult authority; betrayed and distrustful; feeling neglected; helpless and intimidated; disempowered by lack of information); 2) fear and worry for the future (paralyzed by devastating news; uncertainty, anticipation, and dread; broaching intimate and private topics); 3) burdened with responsibility (pressured and unprepared; balancing external expectations; protecting hope); 4) therapeutic patient-provider relationships (emotional support and encouragement; validated personhood and companionship); 5) safety in trust (truthfulness and transparency; prepared by awareness and understanding; reassured by reliable expertise; depending on adults for protection and difficult decisions; security in expressing opinions and needs); and 6) empowerment and assertive agency (right to individual knowledge and choice; control over own life; partnership and respect; enhancing capacity for self-management). During treatment of childhood cancer, patients gain a sense of respect, safety, and control when they feel clinicians address their information and developmental needs. However, communication that is perceived to be parent-centered can be disempowering. Promoting child agency and partnership may improve care and outcomes for children with cancer.

Anaplastic Astrocytoma in a Child With Coffin-Siris Syndrome and a Germline SMARCE1 Mutation: A Case Report.

Coffin-Siris syndrome (CSS) is a rare congenital disorder with variable clinical phenotype consisting of developmental delay and characteristic facial features. It is caused by mutations in the chromatin remodeling switch/sucrose nonfermenting complex. Although SWI/SNF genes are widely implicated in tumorigenesis, only 8 cases of neoplasm have been reported in patients with CSS. We report a case of anaplastic astrocytoma (WHO grade III) in an 18-month-old child with CSS due to a de novo germline missense SMARCE1 mutation. Additional molecular features of the tumor are described as well. The role of missense SMARCE1 mutations in tumor predisposition in children with CSS should be further investigated to better inform genetic counselling.

Dose-dependent response to long-term clomiphene citrate in male functional hypogonadotropic hypogonadism.

Summary: Functional hypogonadotropic hypogonadism is a relatively common condition in middle-aged to elderly men that can significantly impair quality of life. Besides lifestyle optimisation, androgen replacement remains the mainstay of treatment; however, its adverse effects on spermatogenesis and testicular atrophy are undesirable. Clomiphene citrate is a selective oestrogen receptor modulator that acts centrally to increase endogenous testosterone without affecting fertility. Although it has demonstrated effectiveness in shorter-duration studies, its longer-term outcomes are less well-documented. In this study, we report the case of a 42-year-old male with functional hypogonadotropic hypogonadism who sustained an excellent dose-dependent, titratable clinical and biochemical response to clomiphene citrate with no known adverse effects for 7 years to date. This case highlights that clomiphene citrate has potential as a safe and titratable longer-term treatment option, and the need for further randomised control trials in therapy options to normalise androgen status. Learning points: Functional hypogonadotropic hypogonadism is a relatively common, but likely underdiagnosed, condition in middle-aged to older males. Testosterone replacement is the current mainstay of endocrine therapy but can cause sub-fertility and testicular atrophy. Clomiphene citrate is a serum oestrogen receptor modulator that acts centrally to increase endogenous testosterone production without affecting fertility. It has potential as a safe and efficacious longer-term treatment option that can be titrated to increase testosterone and relieve clinical symptoms in a dose-dependent manner. Longitudinal prospective studies as randomised control trials evaluating alternatives to exogenous testosterone are required.

Long-term morbidity of respiratory viral infections during chemotherapy in children with leukaemia.

BACKGROUND: Respiratory viruses are a common cause of infection in immunosuppressed children undergoing cancer therapy. Pulmonary sequelae have been documented following respiratory viral infections (RVIs) in hematopoietic stem cell transplant (HSCT) recipients; however potential late effects in children undergoing nonmyeloablative chemotherapy have not been investigated. AIM: To evaluate the long-term pulmonary morbidity of respiratory viral infections during chemotherapy in children with acute lymphoblastic leukemia (ALL). METHODS: Childhood ALL survivors, aged 7 to 18 years, greater than 6 months posttreatment were recruited. Exclusion criteria included HSCT or proven bacterial/fungal respiratory infection during treatment. Subjects were classified into "viral" or "control" groups according to retrospective medical records that documented the presence of laboratory-proven RVIs during chemotherapy. Symptom questionnaires (Liverpool, ISAAC) and lung function testing (spirometry, plethysmography, diffusing capacity, forced oscillation technique to ATS/ERS standards) were then performed cross-sectionally at the time of recruitment. RESULTS: Fifty-four patients (31 viral, 23 control) were recruited: median (range) age 11.2 (7.2-18.1) years, and at 4.9 (0.5-13) years posttherapy. Abnormalities were detected in 17 (31%) individuals (8 viral, 9 control), with the most common being DLCO impairment (3 viral, 4 control) and reduced respiratory reactance at 5 Hz (5 viral, 6 control). Children with RVIs during chemotherapy reported more current respiratory symptoms, particularly wheeze (odds ratio [OR], 3.0; 95% confidence interval [CI]: 0.9-10.0; P = .09) and cough (OR, 2.7; 95% CI: 0.8-9.5; P = .11). No differences in lung function tests were observed between the two groups. CONCLUSIONS: Our study found children with RVIs during chemotherapy developed more long-term respiratory symptoms than controls; however, differences did not reach statistical significance. No differences in static lung function were found between the two groups. Overall, pulmonary abnormalities and/or significant ongoing respiratory symptoms were detected in nearly a third of ALL survivors treated without HSCT. Larger, prospective studies are warranted to evaluate the etiology and clinical significance of these findings.