Rothia mucilaginosa bacteraemia in an immunocompetent paediatric patient: a new pathogen to take into account. A case report.

Rothia mucilaginosa, previously known as Stomatococcus mucilaginosus, is a Gram-positive coccus that is part of the oropharyngeal microbiota and upper respiratory tract. It is mainly related to infections in immunosuppressed patients. Given its complex microbiological identification, its prevalence may be underestimated. We describe in this article a case of bacteraemia by Rothia in an immunocompetent paediatric patient without epidemiological or medical relevant history. In the available literature no cases of bacteraemia by Rothia mucilaginosa in immunocompetent paediatric patients have been reported. Given the characteristics of our patient, the publication of this case is of interest. Once the diagnosis of Rothia mucilaginosa has been made, the correct functioning of the immune system of the patient should be checked.

Enfermedad de Crohn metastásica en pediatría / Metastatic Crohn’s disease in pediatrics

Introducción: la enfermedad de Crohn metastásica (ECM) constituye una manifestación extraintestinal de la enfermedad de Crohn, siendo fundamental la biopsia para su diagnóstico. Existen referencias escasas a ECM en la edad pediátrica, y en adultos se estima una incidencia del 0,5-1%. No hay consenso sobre su abordaje terapéutico. Nuestro objetivo es describir nuestra experiencia diagnóstica y terapéutica en ECM. Caso clínico: se describen 4 casos de ECM en seguimiento en una Unidad de Gastroenterología Infantil en un hospital pediátrico de tercer nivel. Edades al diagnóstico entre 7 y 13 años. Las lesiones aparecieron antes del diagnóstico de enfermedad de Crohn (EC) en tres de ellos y durante la evolución de la enfermedad en otro. Localización genital en tres pacientes y en región pretibial bilateral en el otro. Todos demostraron granulomas no caseificantes en la biopsia. Dos pacientes precisaron únicamente nutrición enteral exclusiva, observándose resolución completa, mientras que otros dos recibieron terapias combinadas (corticoides, azatioprina, tacrolimus, infliximab y adalimumab) por recurrencia. Solo un caso requirió cirugía por mal control clínico. Discusión: la ECM es una entidad rara aunque siempre debemos incluirla en el diagnóstico diferencial de las lesiones cutáneas en enfermedad de Crohn, teniendo en cuenta que puede ser el debut de la enfermedad. Nos basaremos en la biopsia en cualquier caso para el diagnóstico definitivo. En esta serie se verifica la región genital como la más habitual en niños. El abordaje terapéutico no difiere del manejo de la afectación intestinal (AU)

Introduction and objectives: Metastatic Crohn’s disease (MCD) is an extraintestinal manifestation of Crohn’s disease, with biopsy as fundamental diagnostic tool. There are few references to MCD in children, with a 0.5-1% estimated incidence in adults. There is no consensus about its therapeutic approach. We describe our diagnostic and therapeutic experience in MCD. Results: Four cases of MCD are described in our Pediatric Gastroenterology Unit in a tertiary care hospital. The age at diagnosis was between 7 and 13 years. Lesions appeared before the diagnosis of Crohn’s disease in three of them, and during the course of the disease in another one, with genital location in three patients and bilateral pretibial region in the other. All four cases demonstrated non-caseificant granulomas on biopsy. Only two patients used exclusive enteral nutrition therapy with complete resolution, while other two cases received a combination of therapies (corticosteroids, azathioprine, tacrolimus, infliximab and adalimumab) because of recurrence. Only one case required surgery after poor clinical control. Conclusion: The MCD is infrequent but must always be included in the differential diagnosis of cutaneous lesions in Crohn’s disease, considering it could be the debut of the disease. We will rely on biopsy anyway for definitive diagnosis. In this series the genital region is verified as the most commonly affected in children. The therapeutic approach does not differ from the management of intestinal involvement (AU)

Metastatic Crohn's disease in pediatrics.

INTRODUCTION AND OBJECTIVES: Metastatic Crohn's disease (MCD) is an extraintestinal manifestation of Crohn's disease, with biopsy as fundamental diagnostic tool. There are few references to MCD in children, with a 0.5-1% estimated incidence in adults. There is no consensus about its therapeutic approach. We describe our diagnostic and therapeutic experience in MCD. RESULTS: Four cases of MCD are described in our Pediatric Gastroenterology Unit in a tertiary care hospital. The age at diagnosis was between 7 and 13 years. Lesions appeared before the diagnosis of Crohn's disease in three of them, and during the course of the disease in another one, with genital location in three patients and bilateral pretibial region in the other. All four cases demonstrated non-caseificant granulomas on biopsy. Only two patients used exclusive enteral nutrition therapy with complete resolution, while other two cases received a combination of therapies (corticosteroids, azathioprine, tacrolimus, infliximab and adalimumab) because of recurrence. Only one case required surgery after poor clinical control. CONCLUSION: The MCD is infrequent but must always be included in the differential diagnosis of cutaneous lesions in Crohn's disease, considering it could be the debut of the disease. We will rely on biopsy anyway for definitive diagnosis. In this series the genital region is verified as the most commonly affected in children. The therapeutic approach does not differ from the management of intestinal involvement.

Successful Mercaptopurine Usage despite Azathioprine-Induced Pancreatitis in Paediatric Crohn's Disease.

BACKGROUND: Azathioprine [AZA] and mercaptopurine [MP] are recommended for maintenance of steroid-free remission in children with Crohn`s disease [CD]. Azathioprine-induced pancreatitis, an idiosyncratic and major side effect, has been considered as an absolute contraindication for the use of a second thiopurine in IBD patients. MATERIALS AND METHODS: We describe two children with CD in whom MP were successfully trialled after a confirmed azathioprine-induced pancreatitis, being well tolerated in both cases. RESULTS: Two boys [13 and 10 years old] started exclusive enteral nutrition after diagnosis of moderate (Pediatric Crohn's Disease Activity Index [wPCDAI] = 45) and mild [wPCDAI = 35] CD. Both developed an acute mild to moderate pancreatitis after 2 and 3 weeks, respectively, of AZA treatment but recovered fully in hospital after AZA withdrawal. They started on MP treatment without any adverse effect. They were tested for the presence of polymorphisms 238G>C, 460G>A, and 719A>G in the TPMT gene and 94C>A and 21>C in the ITPase. Both patients were wild-type for all tested polymorphisms. CONCLUSIONS: Azathioprine-induced acute pancreatitis should not be considered as an absolute contraindication for the use of MP. Further investigation is required to create a better understanding of the mechanism underlying the adverse events and to allow more possibilities for personalised therapy.

Successful treatment for ulcerative proctitis with rectal tacrolimus in an 8-year-old girl with intolerance to mesalamine.

Ulcerative colitis (UC) is defined as a chronic inflammatory condition causing continuous mucosal inflammation of the colon without granulomas on biopsy. It affects the rectum, and, to a variable extent, the colon in continuity and is characterized by a relapsing and remitting course. Oral 5-aminosalicylic acid (5-ASA) regimens are recommended as first-line induction therapy for mild to moderately active pediatric UC and for maintenance of remission regardless of other initial treatments. In large clinical trials in adults, mesalamine intolerance was found in 2-5 % of the patients. We present a case of an 8-year-old female patient with intolerance to mesalamine and proctitis resistant to conventional therapy who responded to rectal tacrolimus treatment. The patient started with a dose of 2 mg/day at night with an excellent response. She reported feeling better than any of the previously prescribed treatments and without feeling the discomfort of previously administered enemas. After four weeks of treatment, the dose was reduced to 2 mg/week with no relapses. Tacrolimus suppositories were very well tolerated, and no adverse effects have been reported. Although only very little data has been published, rectal tacrolimus seems to be safe and of efficacy in ulcerative proctitis resistant to standard therapy.