Gluten-sensitive recurrent aphthous stomatitis

Twenty selected patients with recurrent aphthous stomatitis in whom celiac disease had been specifically excluded were placed on a gluten-free diet. Five patients (25%) showed a favorable response to gluten withdrawal and a positive gluten challenge. Jejunal morphology was normal in all patients indicating gluten sensitivity without enteropathy. Gluten withdrawal provides a further method of treating some patients with recurrent aphthous stomatitis.     

Clonal dysregulation of the antibody response to tetanus-toxoid after bone marrow transplantation

After bone marrow transplantation (BMT), a prolonged dysregulation of humoral immunity can be observed. In the present study, we investigated whether this is reflected in an abnormal production of specific antibodies (Ab) to the T-cell-dependent recall antigen tetanus-toxoid (TT). The study group consisted of children receiving transplants of an unmodified allogeneic graft and of adults receiving either a T-cell- depleted allogeneic or an unmodified autologous BM graft. Findings were compared with those in healthy controls. In pediatric graft recipients, who were routinely revaccinated early after BMT, the Ab response was quantitatively superior to that in adult graft recipients who did not receive early revaccination. In the majority of graft recipients, the time period after vaccination required to reach the peak level of antibodies was prolonged and the number of responding TT-specific B- cell clones was markedly decreased in comparison with controls. In controls, a low frequency of dominant B-cell clones may produce low quantities of homogeneous Ab components (H-Ab) against a heterogeneous background. However, in BM graft recipients, "overshooting" of Ab production by separate B-cell clones was observed, resulting in the development of H-Ab at a relatively high concentration. These abnormalities were present up to 10 years after BMT, irrespective of either the age of the recipient, the modulation of the graft, or the vaccination schedule used. It is hypothesized that the dysregulated Ab production is the consequence of activation of a restricted number of resting memory B cells, present in germinal centers, repopulating gradually after BMT. Our data show that routine revaccination early after BMT improves the humoral immune response. However, because of a clonally dysregulated Ab production, long-lasting qualitative defects may be present even after normalization of Ab titers.     

Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: A report from the Children's Oncology Group

Background

Supratentorial PNETs (sPNET) are uncommon embryonal malignancies of the central nervous system whose prognosis has historically been poor. We evaluated the outcome and prognostic factors of children with sPNET treated prospectively on a Children's Oncology Group trial.

Procedure

Following surgery, patients received craniospinal radiotherapy with concurrent carboplatin followed by six months of maintenance chemotherapy with cyclophosphamide and vincristine.

Results

Five‐year overall survival (OS) and progression‐free survival (PFS) for all patients was 58 ± 7% and 48 ± 7%. For patients with pineoblastoma (n = 23), five‐year OS and PFS was 81 ± 9% and 62 ± 11%. Extent of resection but not M‐stage was prognostic. Five‐year OS and PFS for 37 patients with non‐pineal tumors (NPsPNET) was 44 ± 8% and 39 ± 8%, significantly worse than for PB (P = 0.055 and 0.009 respectively). Extent of resection and major radiotherapy deviations were prognostic. Five year OS was 59 +/? 11.4% for those undergoing complete resection versus 10.4 +/? 7% for those who did not (P = 0.017). Central pathologic review called 14 (38%) “classic” sPNET, 8 (22%) “undifferentiated” and 13 (35%) “malignant gliomas.” There was no significant difference between the subgroups, although survival distributions approached significance when the combined “classic” and “undifferentiated” group was compared to the “malignant gliomas.”

Conclusions

Carboplatin during RT followed by 6 months of non‐intensive chemotherapy is a feasible treatment strategy for patients with sPNET. Aggressive surgical resection should be attempted if feasible. The classification of supratentorial small cell malignancies can be difficult. Pediatr Blood Cancer 2015;62:776–783. © 2015 Wiley Periodicals, Inc.

     

Multidisciplinary diabetes team care: the experiences of young adults with Type 1 diabetes

Background

This research examined whether young adults with Type 1 diabetes engage with the multidisciplinary consultation process and if not, then why.

Methods

We designed a web‐based self‐reported survey, available online from February to May 2011, for Australian adults 18–35 years with Type 1 diabetes. Respondents were asked about which clinicians they consulted to assist with self‐management. To expand on the results of the survey, we interviewed 33 respondents.

Results

Survey: Respondents (n = 150) consulted with the following clinicians: endocrinologist and diabetes educators: 23.3%; endocrinologist only: 18.0%; endocrinologist, diabetes educators and dieticians: 14.6%; endocrinologist, diabetes educators, dietician and general practitioners (GP): 11.3%; endocrinologist and GP: 10.6%; GP only: 4.6%; all clinicians recommended to assist with self‐management: 1.3%; 2.7% did not consult any clinician. Interview: Participants (n = 33) reported eight key disincentives to consultation with multidisciplinary clinicians. These were time constraints; provision of conflicting advice; inaccessibility of health services; variation in service standards; cost constraints; failure of clinicians to refer to other clinicians; lack of opportunity to build a therapeutic relationship; and failure of clinicians to engage in shared decision making.

Conclusion

Our results indicate that high attrition rates of young adults with Type 1 diabetes from recommended diabetes health services is linked to the failure of those services to meet the needs and preferences of their patients. The identified needs and preferences included joint consultation with multi‐disciplinary team clinicians; flexible access to advice by email or telephone consultation; and shared decision making. Patient engagement in health‐service re‐design has implications for improved health‐service delivery and enhanced treatment outcomes.