Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome

We report on a 14 year old boy who presented with the symptoms abdominal pain, fever and proteinuria. A hematoma in the region of the right pararenal space was diagnosed. Prothrombin time and activated partial thromboplastin time were prolonged, lupus anticoagulant and anticardiolipin antibodies were positive and serum cortisol was normal. Ten days after admission the boy suddenly suffered generalized seizures due to low serum sodium. As well, the patient developed hemolytic anemia, acute elevated liver enzymes, hematuria and increased proteinuria. At this time a second hemorrhage of the left adrenal gland was documented. Adrenal function tests revealed adrenal insufficiency. We suspected microthromboses in the adrenals and secondary bleeding and treated the boy with hydrocortisone, fludrocortisone and phenprocoumon. CONCLUSION: Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms

Determination of nutrient salts by automatic methods both in seawater and brackish water: the phosphate blank

9 páginas, 2 tablas, 2 figurasThe main inconvenience in determining nutrients in seawater by automatic methods is simply solved: the preparation of a suitable blank which corrects the effect of the refractive index change on the recorded signal. Two procedures are proposed, one physical (a simple equation to estimate the effect) and the other chemical (removal of the dissolved phosphorus with ferric hydroxide).Support for this work came from CICYT (MAR88-0245 project) and Conselleria de Pesca de la Xunta de GaliciaPeer reviewe

The impact of the COVID-19 pandemic on pediatric developmental services: a cross-sectional study on overall burden and mental health status

Background The COVID-19 outbreak has taken a heavy toll on the mental well-being of healthcare workers, even those who have not been directly involved in the care of acutely ill patients. The aims of this study were to identify the overall burden and mental health status of healthcare workers in pediatric developmental services under the influence of the COVID-19 pandemic, and to identify the risk and protective factors associated with mental health. Methods This cross-sectional web-based study was part of a large multicenter VOICE study conducted among employees ((neuro-)pediatricians, psychologists, speech therapists, occupational therapists, etc.) from various pediatric developmental services between June and July 2020. A total of 1291 questionnaires regarding overall burden, mental health status (depression, generalized anxiety disorder and emotional exhaustion) and risk and protective factors for mental health (working conditions, potential problems during the COVID-19 pandemic and psychological resources) were analyzed. Descriptive statistics and multiple linear regression were used for data analysis. Results A total of 44.5% (574/1291) participants felt a high or very high overall burden during the COVID-19 pandemic. Of all the participants, 14.6% (171/1173) reported clinically significant levels of depressive symptoms, 17.0% (199/1173) reported generalized anxiety disorder symptoms and 44.6% (532/1192) reported emotional exhaustion. Multiple linear regression analyses identified several common risk and protective factors for mental health status variables. The burden of an increase in the quantity of work, fear of work and fear of becoming infected showed the strongest negative associations, whereas psychological resources and sufficient relaxation in leisure time exhibited the strongest positive associations. Conclusion Employees who were not directly involved in the care of acutely ill patients were also exposed to considerable stress, some of which was not different from that experienced by professionals who were directly affected. These employees should not be lost sight of and must be offered appropriate support

Effectiveness of antiepileptic therapy in patients with PCDH19 mutations

Purpose PCDH19 mutations cause epilepsy and mental retardation limited to females (EFMR) or Dravet-like syndromes. Especially in the first years of life, epilepsy is known to be highly pharmacoresistant. The aim of our study was to evaluate the effectiveness of antiepileptic therapy in patients with PCDH19 mutations. Methods We report a retrospective multicenter study of antiepileptic therapy in 58 female patients with PCDH19 mutations and epilepsy aged 2-27 years (mean age 10.6 years). Results The most effective drugs after 3 months were clobazam and bromide, with a responder rate of 68% and 67%, respectively, where response was defined as seizure reduction of at least 50%. Defining long-term response as the proportion of responders after 12 months of treatment with a given drug in relation to the number of patients treated for at least 3 months, the most effective drugs after 12 months were again bromide and clobazam, with a long-term response of 50% and 43%, respectively. Seventy-four percent of the patients became seizure-free for at least 3 months, 47% for at least one year. Significance The most effective drugs in patients with PCDH19 mutations were bromide and clobazam. Although epilepsy in PCDH19 mutations is often pharmacoresistant, three quarters of the patients became seizure-free for at least for 3 months and half of them for at least one year. However, assessing the effectiveness of the drugs is difficult because a possible age-dependent spontaneous seizure remission must be considered