Evaluation of propofol-based procedural sedation in pediatric hemato-oncological patients provided by physician assistants in anesthesia.

BACKGROUND: Procedural sedation and analgesia are required for painful hemato-oncological procedures such as lumbar and bone marrow punctures. At our institution, sedation with propofol and alfentanil is usually provided by Physician Assistants in Anesthesia. We evaluated the adverse events during the PSA program for children, provided by Physician Assistants in Anesthesia. PATIENTS AND METHODS: We included pediatric patients meeting our criteria for deep sedation by a Physician Assistants in Anesthesia, scheduled for a hemato-oncological procedure at the Amalia Children's Hospital at the Radboudumc Nijmegen. The primary outcome was oxygen desaturation below 92% for more than 20 s. We prospectively collected data on demographics, current health problems, type of procedure, need for airway interventions, and hypotension. RESULTS: We collected data from 437 sedation sessions involving 71 patients. No oxygen desaturation below 92% lasting longer than 20 s occurred. In 2 cases, a jaw thrust was performed. No invasive airway techniques (oropharyngeal cannula, laryngeal mask, or intubation) were required. A significant drop in mean arterial pressure was seen in 2 out of 437 cases (0.5%). There was no occurrence of cardiopulmonary resuscitation or other adverse events such as aspiration or laryngeal spasm. CONCLUSIONS: Sedation and analgesia for short painful procedures in selected pediatric hemato-oncological units with a dedicated protocol may be safely provided by trained and certified Physician Assistants in Anesthesia.

The impact of maintenance therapy on sleep-wake rhythms and cancer-related fatigue in pediatric acute lymphoblastic leukemia.

PURPOSE: To assess the impact of maintenance therapy and the additional impact of dexamethasone treatment on cancer-related fatigue and sleep-wake rhythms in pediatric acute lymphoblastic leukemia (ALL) patients and to determine the association between these outcomes. METHODS: A national cohort of pediatric ALL patients (≥ 2 years) was included (± 1 year post-diagnosis). Patients receiving dexamethasone were assessed twice (assessment with and without dexamethasone). Actigraphy assessments were used to calculate sleep-wake outcomes with nonparametric methods. Cancer-related fatigue was assessed with the PedsQL Multidimensional Fatigue Scale. Sleep-wake rhythms and cancer-related fatigue were compared between patients participating in the assessment without dexamethasone and healthy children (linear regression) and between assessments with and without dexamethasone (mixed models). Using linear regression, associations between sleep-wake outcomes and cancer-related fatigue were determined during assessments with and without dexamethasone. RESULTS: Responses were collected for 125 patients (113 assessments with and 81 without dexamethasone). The sleep-wake rhythm was less stable (p = 0.03) and less robust (p = 0.01), with lower physical activity levels (p < 0.001) and higher cancer-related fatigue levels (p < 0.001) in ALL patients compared to healthy children. Physical activity was lower (p = 0.001) and cancer-related fatigue more severe (p ≤ 0.001) during assessments with dexamethasone compared to without dexamethasone. Sleep-wake outcomes were significantly associated with cancer-related fatigue during periods without dexamethasone, but not during periods with dexamethasone. CONCLUSION: Sleep-wake rhythms are disturbed, physical activity levels lower, and cancer-related fatigue levels higher during maintenance therapy. Interventions aimed to enhance sleep-wake rhythms during maintenance therapy could improve cancer-related fatigue. Families should be supported in coping with the additional burden of dexamethasone treatment to improve well-being of ALL patients.

[Throat and abdominal symptoms in malignant lymphoma; recognize the alarm signals in children]. / Keel- en buikklachten bij een maligne lymfoom.

In children, a malignant lymphoma can present with life-threatening complications arising from progressive tumour growth, warranting swift diagnosis and treatment. Early recognition can be challenging if a child with paediatric lymphoma presents with abdominal and throat symptoms because of the frequency, and frequently benign nature, of these symptoms in the general paediatric population. In these cases, it is essential for the physician to recognize the alarm signals for suspicion of malignant lymphoma, to allow timely diagnosis and treatment. We present two cases (children age 8 and 16 years) demonstrating the symptoms of malignant lymphoma that should raise alarm in children presenting with unilateral tonsil enlargement or intussusception, and illustrating the importance of timely recognition of these alarm signals, to prevent life-threatening complications due to tumour progression. In patients presenting with unilateral tonsil enlargement, malignant lymphoma should be considered if atypical macroscopic tonsil morphology, cervical lymphadenopathy, dysphagia, snoring, fever or weight loss is present. The presence of a lead point as the cause of intussusception should be considered in children aged > 2 years of age, in a child with an ileo-iliac intussusception and in patients with longstanding abdominal symptoms. In patients presenting with simultaneous symptoms and alarm signals for malignant lymphoma, the relationship between the presenting symptoms should be recognized. Interdisciplinary and intradisciplinary consultation with colleagues can help in the search for an overarching diagnosis, and it is crucial that this should happen without delay to prevent acute life-threatening situations.

[A young lady with shortness of breath during the coronavirus crisis]. / Een benauwde jongedame tijdens de coronacrisis.

A 16-year-old girl repeatedly visited a general practitioner during the coronavirus pandemic for progressive shortness of breath. Progressive orthopnoea was found as well. Her neck was swollen for two weeks and there was generalised itching for months. Given the nature of her symptoms, she was assessed at the coronavirus station. A diagnosis of coronavirus disease 2019 (COVID-19) was assumed. Due to limited testing capacity, the diagnosis was not confirmed. She was treated with supportive treatment that had no effect on her dyspnoea. Tunnel vision ensured that the symptoms that did not fit COVID-19, were not recognised. Moreover, a scheduled ultrasound of her neck was cancelled because of the coronavirus restrictions, which did not help matters. She was eventually admitted to the paediatric intensive care unit with respiratory failure associated with cervical and mediastinal Hodgkin lymphoma.

[Glucose-6-phosphate dehydrogenase deficiency: clinical presentation and eliciting factors]. / Glucose-6-fosfaatdehydrogenasedeficiëntie: klinische presentatie en uitlokkende factoren.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency, a hereditary X-linked disorder, is the most common enzymatic disorder of red blood cells in humans, affecting more than 200 million people worldwide. The prevalence is increasing in the Netherlands due to immigration of people from the Middle East and Africa. We present three different clinical manifestations of G6PD deficiency: neonatal jaundice, haemolysis provoked by infection and haemolysis caused by fava beans. The pathophysiology and treatment are discussed. Furthermore a recent update of chemicals which should be avoided in G6PD deficiency is provided.

Variation in baseline factor VIII concentration in a retrospective cohort of mild/moderate hemophilia A patients carrying identical F8 mutations.

Essentials Factor VIII levels vary in mild and moderate hemophilia A (MHA) patients with the same mutation. We aimed to estimate the variation and determinants of factor VIII levels among MHA patients. Age and genotype explain 59% of the observed inter-individual variation in factor VIII levels. Intra-individual variation accounted for 45% of the variation in the three largest mutation groups. SUMMARY: Background The bleeding phenotype in patients with mild/moderate hemophilia A (MHA) is inversely associated with the residual plasma concentration of factor VIII (FVIII:C). Within a group of patients with the same F8 missense mutation, baseline FVIII:C may vary, because, in healthy individuals, von Willebrand factor (VWF) levels, ABO blood group and age are also known to influence baseline FVIII:C. Our understanding of the pathophysiologic process of the causative genetic event leading to reduced baseline FVIII:C in MHA patients is still limited. Objectives To estimate the variation and determinants of baseline FVIII:C among MHA patients with the same F8 missense mutation. Methods Three hundred and forty-six patients carrying mutations that were present in at least 10 patients in the cohort were selected from the INSIGHT and the RISE studies, which are cohort studies including data of 3534 MHA patients from Europe, Canada, and Australia. Baseline FVIII:C was measured with a one-stage clotting assay. We used Levene's test, univariate and multivariate linear regression, and mixed-model analyses. Results For 59% of patients, the observed variation in baseline FVIII:C was explained by age and genotype. Compared to FVIII:C in patients with Arg612Cys, FVIII:C was significantly different in patients with eight other F8 missense mutations. Intra-individual variation explained 45% of the observed variance in baseline FVIII:C among patients with the same mutation. Conclusion Our results indicate that baseline FVIII:C levels are not exclusively determined by F8 genotype in MHA patients. Insights into other factors may provide potential novel targets for the treatment of MHA.

[Clinical characteristics and management of adolescents admitted to the emergency ward for alcohol intoxication in the region of The Hague during the period 1999-2001]. / Kliniek en beleid bij jongeren met alcoholintoxicatie op de afdelingen voor spoedeisende hulp in de regio Den Haag, 1999-2000.

OBJECTIVE: To analyse the findings in adolescents between the ages of 12 and 17 who were admitted to the emergency ward as a result of excessive alcohol consumption. DESIGN: Retrospective. METHOD: The clinical characteristics, laboratory findings and treatment policy were studied in adolescents between the ages of 12 and 17 who were admitted for alcohol intoxication (> or = 1/1000) to the emergency wards of Leiden University Medical Centre, Juliana Children's Hospital, Haaglanden Medical Centre (Westeinde location) or the Leyenburg Hospital (all hospitals in the area of The Hague, The Netherlands) during the period 1999-2001. RESULTS: In the three successive years, the number of patients was 19, 18 and 51, respectively; there were a total of 58 boys and 30 girls. Laboratory determinations and other investigations were carried out and/or recorded in varying numbers of patients. The cause of the intoxication was usually the consumption of spirits together with friends; 60% of the patients had a Glasgow coma score < 15 and 30% were admitted to hospital. In 25% the visit to the emergency ward was preceded by an accident in which alcohol played a part. Little attention seems to have been paid to aftercare in the form of a follow-up visit.

Sarcoma botryoides in an infant.

A 17-month-old girl with no medical history presented at our emergency room with abnormal vaginal bleeding and vaginal tissue loss with a "grape bunch" appearance. Physical examination showed no abnormalities, but gynaecological examination showed abnormal vaginal tissue protruding through the vagina introitus. Given the typical clinical presentation, the age of the girl and the location and aspect of the lesion, there was a high suspicion of the botryoid variant of embryonal rhabdomyosarcoma of the vagina. Histology of a biopsy of the lesion was consistent with embryonal rhabdomyosarcoma. As no metastases were detected, the girl received chemotherapy. This case report describes the importance of early recognition of the typical clinical symptoms of sarcoma botryoides, since a rapid diagnosis followed by treatment is necessary to prevent death.