Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10mm deviation to the left from the midline. C-reactive protein (9.2mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.

BackgroundTransition care is the planned transfer of adolescents with chronic health conditions from children-centered to adult-oriented health systems. Its challenges include gaps in continuity of care, poor adherence, delays in adult services attendance, differences between pediatric and adult centers, difficulties in autonomy over care and unstable medical conditions. EULAR/PRES recently published recommendations, standards and quality indicators on transitional care for young people with rheumatic diseases, which serve as a guide for transitional outpatient clinics. In Brazil, few centers focus on transition from pediatric rheumatology. The Pediatric Rheumatology Unit of Hospital Geral de Fortaleza is a reference in North and Northeast of the country, intending to be pioneer in transitional medicine in region.ObjectivesTo evaluate the adolescents and their families’ perception regarding follow-up and autonomy in health care, as well as to identify the main risks inherent to this age group.MethodsA longitudinal and descriptive study was carried out through interviews, questionnaires and medical records analyzes. Adolescents between 15 and 18 years old, regularly followed, who had at least three planned visits/year were included. Patients with cognitive impairment or without definite diagnosis were excluded. Epidemiological and clinical data were collected; self-management and health care utilization skills were evaluated by Transitional Readiness Assessment Questionnaire (TRAQ), a self-administered questionnaire.ResultsEighty two patients were recruited into the transitional service (61 female [74.4%]). Patients’ ages ranged from 15.0 to 18.7 years (median 16.9). Regarding religion, 41 patients (54.7%) were Catholic and 27 were Protestant (36%). Concerning to ethnicity, most of them declared themselves brown (61%) or white (22%). Fifty-nine adolescents (72%) considered themselves able to take care of their own follow-up, whereas 39 relatives (50%) considered the patient capable of doing so. The most frequent diseases were juvenile idiopathic arthritis (31.7%) and systemic lupus erythematosus (29.3%), of which 37 (45.1%) were in remission. Median disease duration and follow-up were 48.5 (5 - 180) and 34.5 (1 - 175) months, respectively. The median TRAQ value was 3.27 (1.61 - 4.55). There were 191 transitional visits (median 2.0 visits/patient). Fifty three patients had 2 or more visits, out of which 42 (79.2%) had at least one risk identified. The most prevalent risks were: overweight or obesity (31.7%), sedentarism (26.4%), anxiety (22.6%), changes in family structure (22.6%), school problems (20.8%), depression (18.9%), suicidal ideation/self-mutilation (18.9%), low self-esteem (15.1%). Risks related to psychiatric diseases were identified in 23 patients (43.4%).ConclusionThis is one of the first studies on transition care in Brazil focused on risks identification in adolescence. Transition care allows improvement survival and quality of life due to advances in preventive medicine and treatment of chronic rheu...

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Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10mm deviation to the left from the midline. C-reactive protein (9.2mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.