Diagnostic and treatment challenge of unrecognized subacute bacterial endocarditis associated with ANCA-PR3 positive immunocomplex glomerulonephritis: a case report and literature review.

BACKGROUND: Diagnosis and treatment of either ANCA disease or silent infection-related glomerulonephritis is complicated and is a huge treatment challenge when overlapping clinical manifestations occur. We report a case of ANCA-PR3 glomerulonephritis, nervous system involvement, hepatosplenomegaly and clinically silent subacute infectious endocarditis. CASE PRESENTATION: A 57-year-old man with known mitral valve prolaps was admitted for unexplained renal failure with signs of nephritic syndrome, hepatosplenomegaly, sudden unilateral hearing loss, vertigo, malaise, new onset hemolytic anemia and thrombocytopenia. Immunoserology revealed positive c-anti-neutrophil cytoplasm antibody (ANCA)/anti-proteinase 3 (anti-PR3), mixed type crioglobulinemia and lowered complement fraction C3. Head MRI showed many microscopic hemorrhages. Common site of infection, as well as solid malignoma were ruled out. In accordance with clinical and laboratory findings, systemic vasculitis was assumed, although the etiology remained uncertain (ANCA-associated, cryoglobulinemic or related to unrecognized infection). After kidney biopsy, clinical signs of sepsis appeared. Blood cultures revealed Streptococcus cristatus. Echocardiography showed mitral valve endocarditis. Kidney biopsy revealed proliferative, necrotizing immunocomplex glomerulonephritis. Half a year later, following intravenous immunoglobulins, glucocorticoids, antibiotic therapy and surgical valve repair, the creatinine level decreased and c-ANCA and cryoglobulins disappeared. A second kidney biopsy revealed no residual kidney disease. Four years after treatment, the patient is stable with no symptoms or signs of vasculitis recurrence. CONCLUSIONS: Here we describe the diagnostic and treatment challenge in a patient with unrecognized subacute bacterial endocarditis associated with ANCA-PR3 immunocomplex proliferative and crescentic glomerulonephritis. In patients with ANCA-PR3 immunocomplex glomerulonephritis and other overlapping manifestations suggesting systemic disease, it is important to recognize and aggressively treat any possible coexisting bacterial endocarditis, This is the most important step for a favorable patient outcome, including complete clinical and pathohistological resolution of the glomerulonephritis.

United Kingdom national paediatric bilateral project: Results of professional rating scales and parent questionnaires.

OBJECTIVES: This fourteen-centre project used professional rating scales and parent questionnaires to assess longitudinal outcomes in a large non-selected population of children receiving simultaneous and sequential bilateral cochlear implants. METHODS: This was an observational non-randomized service evaluation. Data were collected at four time points: before bilateral cochlear implants or before the sequential implant, one year, two years, and three years after. The measures reported are Categories of Auditory Performance II (CAPII), Speech Intelligibility Rating (SIR), Bilateral Listening Skills Profile (BLSP) and Parent Outcome Profile (POP). RESULTS: Thousand and one children aged from 8 months to almost 18 years were involved, although there were many missing data. In children receiving simultaneous implants after one, two, and three years respectively, median CAP scores were 4, 5, and 6; median SIR were 1, 2, and 3. Three years after receiving simultaneous bilateral cochlear implants, 61% of children were reported to understand conversation without lip-reading and 66% had intelligible speech if the listener concentrated hard. Auditory performance and speech intelligibility were significantly better in female children than males. Parents of children using sequential implants were generally positive about their child's well-being and behaviour since receiving the second device; those who were less positive about well-being changes also generally reported their children less willing to wear the second device. CONCLUSION: Data from 78% of paediatric cochlear implant centres in the United Kingdom provide a real-world picture of outcomes of children with bilateral implants in the UK. This large reference data set can be used to identify children in the lower quartile for targeted intervention.

United Kingdom national paediatric bilateral project: Demographics and results of localization and speech perception testing.

OBJECTIVES: To assess longitudinal outcomes in a large and varied population of children receiving bilateral cochlear implants both simultaneously and sequentially. METHODS: This observational non-randomized service evaluation collected localization and speech recognition in noise data from simultaneously and sequentially implanted children at four time points: before bilateral cochlear implants or before the sequential implant, 1 year, 2 years, and 3 years after bilateral implants. No inclusion criteria were applied, so children with additional difficulties, cochleovestibular anomalies, varying educational placements, 23 different home languages, a full range of outcomes and varying device use were included. RESULTS: 1001 children were included: 465 implanted simultaneously and 536 sequentially, representing just over 50% of children receiving bilateral implants in the UK in this period. In simultaneously implanted children the median age at implant was 2.1 years; 7% were implanted at less than 1 year of age. In sequentially implanted children the interval between implants ranged from 0.1 to 14.5 years. Children with simultaneous bilateral implants localized better than those with one implant. On average children receiving a second (sequential) cochlear implant showed improvement in localization and listening in background noise after 1 year of bilateral listening. The interval between sequential implants had no effect on localization improvement although a smaller interval gave more improvement in speech recognition in noise. Children with sequential implants on average were able to use their second device to obtain spatial release from masking after 2 years of bilateral listening. Although ranges were large, bilateral cochlear implants on average offered an improvement in localization and speech perception in noise over unilateral implants. CONCLUSION: These data represent the diverse population of children with bilateral cochlear implants in the UK from 2010 to 2012. Predictions of outcomes for individual patients are not possible from these data. However, there are no indications to preclude children with long inter-implant interval having the chance of a second cochlear implant.

United Kingdom national paediatric bilateral audit.

Prior to 2009, UK public funding was mainly only available for children to receive unilateral cochlear implants. In 2009, the National Institute for Health and Clinical Excellence (NICE) published guidance for cochlear implantation following their review. According to these guidelines, all suitable children are eligible to have simultaneous bilateral cochlear implants or a sequential bilateral cochlear implant if they had received the first before the guidelines were published. NICE stated that they would review this decision in 2011. In preparation for this review, 13 UK cochlear implant centres formed a consortium, and the decision was made to carry out a multi-centre audit. The audit involves collecting data from simultaneously and sequentially implanted children at three intervals: before bilateral cochlear implants or before the sequential implant, 1 year after bilateral implants, and 2 years after bilateral implants. The measures include localization, speech recognition in quiet and background noise, speech production, listening, vocabulary, parental perception, quality of life, and surgical data including complications. The audit has now passed the 1-year point, and data have been received on more than 400 children. Preliminary results will be available a year later.