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Selective BRAF inhibitors such as vemurafenib have become a treatment option in patients with Langerhans cell Histiocytosis (LCH). To date, only 14 patients receiving vemurafenib for LCH have been reported. Although vemurafenib can stabilize the clinical condition of these patients, it does not seem to cure the patients, and it is unknown, when and how to stop vemurafenib treatment. We present a girl with severe multisystem LCH who responded only to vemurafenib. After 8 months of treatment, vemurafenib was tapered and replaced by prednisone and vinblastine, a strategy which has not been described to date. Despite chemotherapy, early relapse occurred, but remission was achieved by re-institution of vemurafenib. Further investigation needs to address the optimal duration of vemurafenib therapy in LCH and whether and which chemotherapeutic regimen may prevent disease relapse after cessation of vemurafenib.
Background: Standardization in clinical practice may lead to improved outcomes. Unfortunately, little is known about the variability of non-pharmacological anti-infective measures in children with cancer.
Design and Methods: A web-based survey assessed institutional recommendations regarding restrictions of social contacts, pets and food and instructions on wearing face masks in public for children with standard- risk acute lymphoblastic leuk emia and acute myeloid leukemia during intensive chemotherapy.
Results: A total of 336 institutions in 27 countries responded to the survey (range, 1-76 institutions per country; overall response rate 61%). Most institutions recommend that patients with acute myeloid leukemia avoid indoor public places and daycare, kindergarten and school, whereas recommendations for patients with acute lymphoblastic leukemia differ considerably by institution. In terms of restrictions related to pets, there was a wide variability between institutions for both acute lymphoblastic and acute myeloid leukemia patients. Most, but not all institutions do not allow children with either acute lymphoblastic or acute myeloid leukemia to eat raw meat, raw seafood or unpasteurized milk. Whereas most institutions do not routinely recommend that patients with acute lymphoblastic leukemia wear face masks in public, advice on this matter varies for patients with acute myeloid leukemia.
Conclusions: The survey demonstrates that there is a wide variation in recommendations on non-pharmacological anti-infective measures between different institutions, countries and continents. This information may be used to encourage harmonization of supportive care practices and future clinical trials.
Inguinal hernia repair (IHR) is a common procedure in childhood. Laparoscopic IHR has been evolving for the last three decades. Although clear advantages have been shown, adaptation in Germany has been slow. We aim to study the current status of pediatric laparoscopic IHR. A survey was sent to all 89 pediatric surgical departments in Germany on current practices and preferences of open versus laparoscopic IHR. Two nationwide databases of administrative claims data from 2019 were analyzed and correlated with responses from the survey. A total of 56% of the pediatric surgical departments supplied data through the quality reports. The recall of our survey was 58% of all pediatric surgery departments. According to the pooled data, laparoscopic IHR was performed in 8.2% of all inpatients treated. Laparoscopic IHR was considered a training procedure in 48% of the departments. Five different laparoscopic techniques were described (most commonly percutaneous closure of the hernia under laparoscopic vision). The choice between open and laparoscopic IHR was mainly determined by the child’s age. Currently, only a minority of German children undergo inguinal hernia repair by laparoscopy. More training opportunities in the form of hands-on and video workshops may lead to more widespread employment of the laparoscopic technique.
Response to upfront azacitidine in juvenile myelomonocytic leukemia in the AZA-JMML-001 trial
(2021)
Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative therapy for most children with juvenile myelomonocytic leukemia (JMML). Novel therapies controlling the disorder prior to HSCT are needed. We conducted a phase 2, multicenter, open-label study to evaluate the safety and antileukemic activity of azacitidine monotherapy prior to HSCT in newly diagnosed JMML patients. Eighteen patients enrolled from September 2015 to November 2017 were treated with azacitidine (75 mg/m2) administered IV once daily on days 1 to 7 of a 28-day cycle. The primary end point was the number of patients with clinical complete remission (cCR) or clinical partial remission (cPR) after 3 cycles of therapy. Pharmacokinetics, genome-wide DNA-methylation levels, and variant allele frequencies of leukemia-specific index mutations were also analyzed. Sixteen patients completed 3 cycles and 5 patients completed 6 cycles. After 3 cycles, 11 patients (61%) were in cPR and 7 (39%) had progressive disease. Six of 16 patients (38%) who needed platelet transfusions were transfusion-free after 3 cycles. All 7 patients with intermediate- or low-methylation signatures in genome-wide DNA-methylation studies achieved cPR. Seventeen patients received HSCT; 14 (82%) were leukemia-free at a median follow-up of 23.8 months (range, 7.0-39.3 months) after HSCT. Azacitidine was well tolerated and plasma concentration-–time profiles were similar to observed profiles in adults. In conclusion, azacitidine monotherapy is a suitable option for children with newly diagnosed JMML. Although long-term safety and efficacy remain to be fully elucidated in this population, these data demonstrate that azacitidine provides valuable clinical benefit to JMML patients prior to HSCT. This trial was registered at www.clinicaltrials.gov as #NCT02447666.
In Eurotransplant kidney allocation system (ETKAS), candidates can be considered unlimitedly for repeated re‐transplantation. Data on outcome and benefit are indeterminate. We performed a retrospective 15‐year patient and graft outcome data analysis from 1464 recipients of a third or fourth or higher sequential deceased donor renal transplantation (DDRT) from 42 transplant centers. Repeated re‐DDRT recipients were younger (mean 43.0 vs. 50.2 years) compared to first DDRT recipients. They received grafts with more favorable HLA matches (89.0% vs. 84.5%) but thereby no statistically significant improvement of patient and graft outcome was found as comparatively demonstrated in 1st DDRT. In the multivariate modeling accounting for confounding factors, mortality and graft loss after 3rd and ≥4th DDRT (P < 0.001 each) and death with functioning graft (DwFG) after 3rd DDRT (P = 0.001) were higher as compared to 1st DDRT. The incidence of primary nonfunction (PNF) was also significantly higher in re‐DDRT (12.7%) than in 1st DDRT (7.1%; P < 0.001). Facing organ shortage, increasing waiting time, and considerable mortality on dialysis, we question the current policy of repeated re‐DDRT. The data from this survey propose better HLA matching in first DDRT and second DDRT and careful selection of candidates, especially for ≥4th DDRT.
Background: The aim of this study was to evaluate post‐irradiation changes in the central nervous system (CNS) detected using magnetic resonance (MR) imaging.
Methods: Magnetic resonance images of 15 children with CNS tumors treated through whole‐brain irradiation over 10 years were reviewed retrospectively. Variables such as age at the time of irradiation, total radiation dose, treatment length, and time interval between irradiation and MR changes, were evaluated.
Results: All patients included in the study had imaging abnormalities of the CNS. Eight patients (53%) developed CNS abnormalities within a short period of time – only a few months after irradiation (mean 4.8 months). Seven patients (47%) developed CNS abnormalities within a long time interval after treatment (mean 4.6 years). In almost all patients, a T2 increase in supra‐ and infratentorial white matter was observed. Follow‐up examinations showed nine patients (60%) with cerebellar atrophy.
Conclusions: In this sample of pediatric patients who underwent whole‐brain irradiation, the time receiving irradiation was not related to the severity of the MR changes. A correlation between the age of the child or the length of the radiotherapy and the extent of the changes could not be confirmed. However, we observed a trend towards stronger brain parenchymal degeneration with cystic changes in the younger age group of children in our sample. Older children who received irradiation seem to be more susceptible to vascular dysplasia with cavernous hemangiomas and microbleeding.
Maziwa a pakifuwa ca mama
(2010)
Au moment où l’Afrique en général, et le Ghana, en particulier, s’apprêtent à célébrer le centenaire de la naissance d’un des grands hommes politiques de l’Afrique contemporaine, je voudrais, en réponse à mon fils, examiner ces questions : quelle réception peut-on faire aujourd’hui du panafricanisme ? Que sont devenus les grands idéologues de ce mouvement ? L’Afrique a-t-elle encore une chance de rayonner un jour ? Chacun peut continuer la liste des questions. Comme chacun sait, du moins, je suppose, Kwame Nkrumah est né le 21 septembre 1909 et est incontestablement une des figures centrales du panafricanisme. Pendant que j’étais en train de parcourir un journal, mon fils me demande après avoir lu le mot « panafricanisme » : « Qu’est-ce que le panafricanisme ? » Ce terme échappe à toute définition évidente, je veux dire claire, nette et précise. Lors du 7ème congrès de ce mouvement panafricain réuni à Kampala, en Ouganda en 1994, les congressistes n’ont pu livrer une définition univoque. « Deux tendances [se sont affrontées] : celle inspirée de Kwame Nkrumah et des luttes de libération anti-impérialistes, l’autre, plus modérée, prônant une relation ‘’amicale’’ avec l’Occident ». Pour ma part, ne laissant place à aucune ambiguïté je choisirai naturellement la première, non sans relativiser l’importance accordée à l’anti- impérialisme. L’Afrique ne souffre plus du colonialisme mais de ses propres fils. (...) Le panafricanisme de Nkrumah et ses amis est « mort » et notre Afrique contemporaine continue de le tuer. Pour mieux te faire comprendre ce que je veux te dire, je voudrais que tu suives avec moi 3 idées fortes qui vont peut-être te convaincre ou te dégoûter, peu importe. -------------------------------------------------------------------------------------------------- CRELAF (Cercle de Reflexion des Etudiants en Littératures Africaines), Département de Littératures Africaines, Université Omar Bongo, Gabon
Background: Due to the difficulties in the definite diagnosis, data on brain imaging in pediatric patients with central nervous system (CNS)-invasive mold infection (IMD) are scarce. Our aim was to describe brain imaging abnormalities seen in immunocompromised children with CNS-IMD, and to analyze retrospectively whether specific imaging findings and sequences have a prognostic value. Methods: In a retrospective study of 19 pediatric patients with proven or probable CNS-IMD, magnetic resonance imaging (MRI)-findings were described and analyzed. The results were correlated with outcome, namely death, severe sequelae, or no neurological sequelae. Results: 11 children and 8 adolescents (11/8 with proven/probable CNS-IMD) were included. Seven of the patients died and 12/19 children survived (63%): seven without major neurological sequelae and five with major neurological sequelae. Multifocal ring enhancement and diffusion restriction were the most common brain MRI changes. Diffusion restriction was mostly seen at the core of the lesion. No patient with disease limited to one lobe died. Perivascular microbleeding seen on susceptibility weighted imaging (SWI) and/or gradient-echo/T2* images, as well as infarction, were associated with poor prognosis. Conclusions: The presence of infarction was related to poor outcome. As early microbleeding seems to be associated with poor prognosis, we suggest including SWI in routine diagnostic evaluation of immunocompromised children with suspected CNS-IMD.
Whereas the clinical approach in pediatric cancer patients with febrile neutropenia is well established, data on non-neutropenic infectious episodes are limited. We therefore prospectively collected over a period of 4 years of data on all infectious complications in children treated for acute lymphoblastic or myeloid leukemia (ALL or AML) and non-Hodgkin lymphoma (NHL) at two major pediatric cancer centers. Infections were categorized as fever of unknown origin (FUO), and microbiologically or clinically documented infections. A total of 210 patients (median age 6 years; 142 ALL, 23 AML, 38 NHL, 7 leukemia relapse) experienced a total of 776 infectious episodes (571 during neutropenia, 205 without neutropenia). The distribution of FUO, microbiologically and clinically documented infections, did not significantly differ between neutropenic and non-neutropenic episodes. In contrast to neutropenic patients, corticosteroids did not have an impact on the infectious risk in non-neutropenic children. All but one bloodstream infection in non-neutropenic patients were due to Gram-positive pathogens. Three patients died in the context of non-neutropenic infectious episodes (mortality 1.4%). Our results well help to inform clinical practice guidelines in pediatric non-neutropenic cancer patients presenting with fever, in their attempt to safely restrict broad-spectrum antibiotics and improve the quality of life by decreasing hospitalization.