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We report a previously well paediatric patient with two distinct presentations of invasive group A streptococcus (GAS) infection resulting in significant morbidity. The first episode, following GAS pharyngitis, involved multiorgan dysfunction syndrome. This included cardiorespiratory and acute hepatorenal failure and purpura fulminans that eventually necessitated four-limb amputation. The second episode occurred 12 months later, from undetermined aetiology, and resulted in septic shock. Molecular analysis of the emm gene and PCR for Serum Opacity Factor revealed that the initial isolate was M Type 4 and sof gene positive while the second isolate was M Type 1 and sof gene negative. Immunological investigations, including CH50, quantitative IgA, IgM and IgG, and flow cytometry measuring lymphocyte subsets, and vaccine response to measles, mumps, rubella and pneumococcus were normal. This is the first report of recurrent bacteraemia from different strains of Streptococcus pyogenes infection in an apparently immunocompetent child.

Reporting of severe invasive group A streptococcal disease (IGAS) has increased in Canada over the past decade, highlighting the importance of optimal chemoprophylaxis and management strategies. Canadian guidelines have had variable uptake across Canada. This practice point updates relevant aspects of these guidelines, with a focus on chemoprophylaxis of contacts of IGAS cases and clinical management of IGAS. The importance of penicillin in treating group A streptococcal disease is reaffirmed, and the role of clindamycin is discussed. In situations in which chemoprophylaxis may be considered, preferred agents are summarized.

Les déclarations de graves infections invasives à streptocoque du groupe A (IISGA) ont augmenté au Canada depuis dix ans, ce qui fait ressortir l’importance d’une chimioprophylaxie et de stratégies thérapeutiques optimales. L’adoption des lignes directrices canadiennes a été inégale au pays. Le présent point de pratique contient une mise à jour des éléments pertinents de ces lignes directrices et s’attarde sur la chimioprophylaxie des contacts et sur la prise en charge clinique de l’IISGA. On y réitère l’importance de la pénicilline dans le traitement de l’infection à streptocoque du groupe A et on y aborde le rôle de la clindamycine. On présente également un résumé des médicaments à privilégier lorsqu’une chimioprophylaxie est envisagée.

Lyme disease is an emerging problem in Nova Scotia. Lyme arthritis is a late manifestation of Lyme disease.To describe the demographic characteristics, referral patterns and clinical course of children diagnosed with Lyme arthritis in a tertiary care pediatric rheumatology clinic in Nova Scotia.In the present retrospective chart review, subjects diagnosed with Lyme arthritis between 2006 and 2013 were identified through the clinic database. Demographic variables, referral patterns, clinical presentation and information regarding treatment course and outcome were collected.Seventeen patients were identified; 76% presented in 2012 and 2013. In 37.5% of cases, the referring physician suspected Lyme disease. Most patients presented with one or more painful and/or swollen joints; 94% had knee involvement. Only three of 17 patients had a history of erythema migrans and four of 17 recalled a tick bite. Five patients had a history of neurological manifestations consistent with Lyme disease, although, none had a diagnosis made at the time. Arthritis usually resolved after treatment with standard antibiotics; however, at last follow-up, two patients had antibiotic refractory Lyme arthritis, with one having joint damage despite aggressive arthritis treatment.A significant increase in cases of Lyme arthritis has recently been recognized in a pediatric rheumatology clinic in Nova Scotia. A history of a tick bite or erythema migrans were not sensitive markers of Lyme arthritis, and this diagnosis was often not considered by the referring physician. Educational initiatives should be undertaken to increase local awareness of this treatable cause of arthritis in children.La maladie de Lyme est un problème émergent en Nouvelle-Écosse. L’arthrite de Lyme en est une manifestation tardive.Décrire les caractéristiques démographiques, les profils d’aiguillage et l’évolution clinique des enfants recevant un diagnostic d’arthrite de Lyme dans une clinique de soins tertiaires en rhumatologie pédiatrique de la Nouvelle-Écosse.Dans la présente étude rétrospective des dossiers, les chercheurs ont extrait de la base de données cliniques les sujets qui ont reçu un diagnostic d’arthrite de Lyme entre 2006 et 2013. Ils ont colligé les variables démographiques, les profils d’aiguillage, la présentation clinique et l’information sur l’évolution et les résultats cliniques du traitement.Les chercheurs ont dépisté 17 patients, dont 76 % ont consulté en 2012 et 2013. Dans 37,5 % des cas, le médecin traitant présumait une maladie de Lyme. La plupart des patients avaient au moins une articulation douloureuse ou enflée et 94 %, une atteinte du genou. Seulement trois des 17 patients avaient des antécédents d’érythème migrateur et quatre se rappelaient s’être fait piquer par une tique. Cinq patients avaient des antécédents de manifestations neurologiques évocateurs de la maladie de Lyme, mais aucun n’avait alors reçu de diagnostic. En général, l’arthrite disparaissait après un traitement aux antibiotiques standards, mais au dernier suivi, deux patients souffraient d’une arthrite de Lyme réfractaire aux antibiotiques, dont l’un présentait une atteinte articulaire malgré un traitement vigoureux contre l’arthrite.On a récemment constaté une augmentation significative des cas d’arthrite de Lyme dans une clinique de rhumatologie pédiatrique de la Nouvelle-Écosse. Des antécédents de piqûre de tique ou d’érythème migrateur n’étaient pas des marqueurs sensibles de l’arthrite de Lyme, et il n’est pas rare que le médecin traitant ne l’envisage pas. Il fau-drait lancer des initiatives de formation locales pour mieux faire connaître cette cause traitable de l’arthrite chez les enfants.

Francisella philomiragiais a rare and opportunistic pathogen capable of producing invasive infection in patients with compromised neutrophil function and in patients that have survived a near-drowning. A case ofF philomiragiaadenitis and lung nodules, refractory to cephalosporin therapy, is reported in a 10-year-old boy with chronic granulomatous disease following a facial abrasion from a saltwater crab. To the authors' knowledge, this is the first Canadian clinical isolate to be reported. Genus and species identification was confirmed via 16S ribosomal RNA sequence analysis. A literature review revealed three groups at risk ofF philomiragiainfection: young patients with chronic granulomatous disease; adults with hematogenous malignancy; and near-drowning patients. Pneumonia, fever without an apparent source and sepsis are the main clinical presentations. Invasive procedures may be required to isolate this organism and ensure appropriate antimicrobial therapy. Limited awareness ofF philomiragiahas led to delayed identification, patient death and misidentification asFrancisella tularensis- a biosafety level three pathogen and potential bioterrorism agent.

Background/Purpose: Lyme disease (LD) is an emerging problem in the Canadian province of Nova Scotia with a sharp rise in reported cases over the past 2 years, including cases referred to the pediatric rheumatology clinic in Halifax, the sole pediatric rheumatology referral centre in the province. This increase is likely related to an increase in the number and size of endemic areas in the province; it is expected that cases will continue to rise. We sought to characterize the demographics, clinical presentation, treatment course and musculoskeletal outcome of children with LD in our clinic. Methods: Subjects with a diagnosis of LD since 2008 were identified in the pediatric rheumatology clinic database. The medical records of patients with LD were reviewed for demographic variables, referral patterns, clinical presentation, investigations, treatment course, and outcome. Results: Seventeen patients were identified; all patients were referred for evaluation of arthritis. 76% of patients presented in 2012 and 2013. Median age was 11.5 years (2.6–15.8), 16 were male. All lived in known endemic areas with Lunenburg county being most common (59%); 76% did not recall a tick bite. Subjects were referred by their family physician (7), emergency department (4), orthopedic surgery (4), and infectious diseases (2). The median number of physician visits for MSK symptoms prior to rheumatology consult was 2 (range 1 to >5). Only one patient was referred with a known diagnosis of LD; LD was suspected in 37.5% of the remaining cases based on referral information. Six patients had prior joint aspiration, 2 of whom were treated for septic arthritis. Pain and/or swelling were reported by all patients; in those with swelling 47% were persistent/53% episodic. The median number of joints involved was 1, with 94% having knee involvement. Patients reported MSK–symptoms for a median of 2 months (range 0.1–11) prior to referral to rheumatology. Four patients had a history of neurologic manifestations of LD prior to MSK presentation, none of which were recognized as LD. ECG was done in 13 cases and was normal. Three patients had a history of erythema migrans. 14/17 patients have completed antibiotic treatment. Arthritis resolved in 50% of patients after 1 course of antibiotics and 29% after 2 courses. A third course of antibiotics was required in 21% of patients for persistent arthritis. Two patients continued to have arthritis following completion of antibiotic therapy. At a median of 5 months (range 0–50) following treatment, there are 2 patients with persistent synovitis and functional limitations; one having evidence of joint damage despite extensive arthritis treatment (NSAID, steroid injection, DMARDs and a biologic). Conclusion: Increasing numbers of children with Lyme arthritis are being seen in the pediatric rheumatology clinic in Nova Scotia. LD was not considered as a diagnosis in the majority of cases by the referring physician. A quarter of patients also had a history of unrecognized neurologic manifestations of LD. In keeping with published North American reports, most patients had an excellent outcome however a proportion continued to have arthritis and disability following antibiotic therapy.

A previously healthy six-year-old girl had developed a fever five days before presentation. Two days later, she had an onset of diffuse abdominal pain, intermittent vomiting and loose, nonbloody stools. On the fourth day of fever, she described pain on moving her arms and legs, and the following day, she was brought to the IWK Health Centre in Halifax, Nova Scotia. Her history showed up-to-date immunizations, and the absence of medications or known allergies. There was no history of trauma, infectious contacts, travel outside the Maritime provinces, or consumption of unpasteurized dairy products or undercooked meats. She had no history of animal exposure, except for a white rat kept in a cage in the family basement. On examination, she was febrile (38.8°C), unwell looking and tachycardic, but was oriented, normotensive and in no respiratory distress. Her head and neck examinations revealed periorbital edema, marked erythema of the buccal mucosa, and a ‘strawberry tongue’ with no oral ulcers, tonsillar exudate, cervical adenopathy or conjunctival injection. A blanching erythematous maculopapular rash was visible on the face, trunk, arms and legs, being most prominent on the hands and feet. A palpable, nonblanching petechial exanthem was also present on the palms of the hands and soles of the feet, with marked periungual erythema and capillary vessel dilation at the nail beds. No puncture marks or scratches were visible. The wrists, elbows, knees and small joints of the hands were swollen and warm to palpation, but without erythema. Any motion of these joints was limited and painful. There was periarticular swelling of the ankles and feet. The precordium was hyperdynamic, with a 2/6 vibratory systolic ejection murmur at the upper left sternal border. There were no bruits. There was no hepatosplenomegaly or generalized adenopathy. The white blood cell count was 6.0×109/L (56% neutrophils, 12% bands and 13% lymphocytes), the hemoglobin level was normal and the platelet count was 31×109/L. The C-reactive protein level and erythrocyte sedimentation rate were elevated at 219 mg/L (reference less than 5.3 mg/L) and 65 mm/h (reference 0 mm/h to 9 mm/h), respectively. Renal function and liver enzymes tests were normal. Urinalysis revealed trace blood, and hyaline and granular casts. Her chest radiograph and echocardiogram were normal. Empirical antibiotic therapy was started. A blood culture taken on the day of presentation was positive at 15 h. What is the diagnosis?

Background: HSCT recipients are at high risk of late bloodstream infection (BSI). Controversy exists regarding the benefit of surveillance blood cultures in this immunosuppressed population. Despite the common use of this practice, its practical value is not well established in non-neutropenic children following HSCT. Methods: At the IWK Health Centre, a tertiary health care center serving 2.5 million in the three Maritime provinces of Canada, weekly surveillance blood cultures from central venous access lines (CVLs) are drawn from children following HSCT until CVL removal. We reviewed data from a prospectively collected CVL database in association with a health record review to determine the utility and cost of this practice. Eligible participants were non-neutropenic pediatric HSCT recipients with CVLs followed at the IWK between 1999 and 2005. Patients routinely received PCP and penicillin prophylaxis. Cultures were considered to be for surveillance if the child was afebrile without focus of infection. The cost of laboratory investigations, nursing time, hospital stay, and interventions for positive surveillance cultures was calculated. Results: 43 HSCTs were performed in 41 patients of whom 21 were male (49%). The median age was 7 years (range 1–18). Donors were related in 15 cases (35%), unrelated in 18 (42%) and autologous in 10 cases (23%). The most common underlying illness leading to HSCT was leukemia (19/41; 46%) followed by solid tumour (7; 17%), inborn errors of metabolism and Fanconi Anemia (4 each; 10%) and other (4;10%). The median length of study eligibility, defined as the point at which the patient returned to the IWK from the transplant centre until the time of central line removal, was 70 days (range 7–176). There were 316 patient contacts for surveillance cultures (mean 7 per transplant) and 577 central line lumens sampled. Three patients had potentially clinically significant surveillance blood cultures that were positive (3/43; 7%). The bacteria isolated were Klebsiella pneumoniae (n=2) and Corynebacterium jeikium (n=1). Repeat cultures were done prior to initiation of antimicrobial therapy and all were sterile. Patients were admitted for antimicrobial therapy if they were not already hospitalized for HSCT care. Median hospitalization for treatment of the positive culture was 5 days. All three patients had an uncomplicated course. The estimated total cost of BSI surveillance and treatment of asymptomatic infection over 6 years was $27,989. Conclusion: This study suggests that BSI surveillance in children post engraftment has a very low yield and significant cost. It is unclear that it contributes to improved patient outcomes.