Utilidad de la entero-resonancia magnética en el estudio de la enfermedad de Crohn pediátrica / MRI enterography in the assessment of paediatric Crohn’s disease

Objetivo: Evaluar la utilidad de la resonancia magnética intestinal con contraste oral (RMI) para el estudio de extensión y actividad de la enfermedad de Crohn (EC) pediátrica, comparando los hallazgos con índices clínicos, tests biológicos, endoscopia y otras técnicas de imagen. Pacientes y métodos: Fueron valoradas las RMI efectuadas en pacientes menores de 18 años diagnosticados de EC. Para la preparación se administró 500-1000ml de polietilenglicol una hora antes de las imágenes (1,5-TMR). Se realizaron secuencias T2 HASTE con o sin Fat SAT, T2 true-FISP, T1 Fat-SAT VIBE pre/posgadolinio, HASTE dinámico y difusión. Se valoraron el engrosamiento de pared intestinal, la hipercaptación mucosa y las complicaciones extraintestinales. Se establecieron 5 patrones de RMI: normal, fibrosis, actividad leve, moderada y severa-transmural. Los hallazgos se compararon con PCDAI, parámetros inflamatorios, resultados endoscópicos e histológicos. Resultados: Incluimos para la evaluación 22 estudios. El 82% presentaba una distensión intestinal óptima. Observamos efectos secundarios leves en el 12% de los pacientes. Encontramos una relación significativa entre los patrones de RMI versus PCDAI (p=0,002), VSG (p=0,006) y PCR (p=0,047); no hallamos relación estadísticamente significativa (p=0,571) con la histología. La RMI valoró correctamente la extensión de la enfermedad a nivel ileal (80%) y a nivel cólico (66%). Un 86,4% de los estudios mostraron complicaciones extraintestinales, sin presentar relación estadística con la clasificación de RMI (p=0,274). Conclusiones: Existe una relación estadísticamente significativa entre nuestros patrones de RMI y PCR, VSG y PCDAI. La RMI presenta excelente concordancia con las ileoscopias. La RMI valora zonas no accesibles mediante endoscopia y permite el diagnóstico de complicaciones extraintestinales sin irradiación (AU)

Objective: To determine the usefulness of MRI enterography for assessing the extension and activity of paediatric Crohn's disease. MRI findings were compared with clinical, biological, endoscopic and other imaging data. Patients and methods: Studies of MRI enterography use in patients younger than 18 years of age were reviewed. Patients received 500-1000mL of polyethylene glycol one hour before examination (1.5-TMR). T2 HASTE sequences with or without fat saturation, T2 true-FISP, T1 with fat saturation, pre- and post gadolinium-enhanced VIBE sequences, and dynamic and diffusion HASTE were acquired. Thickening of the bowel wall, mucosal enhancement, and extra-luminal complications were evaluated. Five MRI patterns (normal, fibrosis, mild, moderate, and severe transmural activity) were defined. Findings were compared with PCDAI scores, inflammatory parameters, and endoscopic and histological results. Results: Twenty-two studies were reviewed. Optimal intestinal distension was present in 82% of the cases. Mild side effects were observed in 12% of patients. There was a significant relationship between MRI patterns and PCDAI scores (P=0.002), sedimentation rate (P=0.006) and serum PCR levels (P=0.047) and a non-significant relationship with the histology (P=0.571). MRI enterography correctly assessed the ileal (80%) and colonic (66%) extension. Extra-luminal complications unrelated to MRI classification (P=0.274) were reported in 86.4% of studies. Conclusions: There was a significant relationship between MRI patterns and PCR, sedimentation rate, and PCDAI scores. MRI enterography showed excellent agreement with ileoscopies, and allowed endoscopically non-accessible areas to be assessed, as well as the diagnosis of extra-luminal complications without irradiation (AU)

[MRI enterography in the assessment of paediatric Crohn's disease]. / Utilidad de la entero-resonancia magnética en el estudio de la enfermedad de Crohn pediátrica.

OBJECTIVE: To determine the usefulness of MRI enterography for assessing the extension and activity of paediatric Crohn's disease. MRI findings were compared with clinical, biological, endoscopic and other imaging data. PATIENTS AND METHODS: Studies of MRI enterography use in patients younger than 18 years of age were reviewed. Patients received 500-1000mL of polyethylene glycol one hour before examination (1.5-TMR). T2 HASTE sequences with or without fat saturation, T2 true-FISP, T1 with fat saturation, pre- and post gadolinium-enhanced VIBE sequences, and dynamic and diffusion HASTE were acquired. Thickening of the bowel wall, mucosal enhancement, and extra-luminal complications were evaluated. Five MRI patterns (normal, fibrosis, mild, moderate, and severe transmural activity) were defined. Findings were compared with PCDAI scores, inflammatory parameters, and endoscopic and histological results. RESULTS: Twenty-two studies were reviewed. Optimal intestinal distension was present in 82% of the cases. Mild side effects were observed in 12% of patients. There was a significant relationship between MRI patterns and PCDAI scores (P=.002), sedimentation rate (P=.006) and serum PCR levels (P=.047) and a non-significant relationship with the histology (P=.571). MRI enterography correctly assessed the ileal (80%) and colonic (66%) extension. Extra-luminal complications unrelated to MRI classification (P=.274) were reported in 86.4% of studies. CONCLUSIONS: There was a significant relationship between MRI patterns and PCR, sedimentation rate, and PCDAI scores. MRI enterography showed excellent agreement with ileoscopies, and allowed endoscopically non-accessible areas to be assessed, as well as the diagnosis of extra-luminal complications without irradiation.

Síndrome de aorta media / Mid-aortic syndrome: case studies from a paediatric nephrology department

Introducción: El síndrome de aorta media (SAM) (o su término anglosajón, mid aortic syndrome) describe el estrechamiento segmentario de la aorta abdominal y ramas que de ella derivan. Un alto porcentaje involucrará a las arterias renales, siendo la hipertensión arterial (HTA) la manifestación clínica cardinal. Objetivo: Revisión de los casos diagnosticados de SAM del Servicio de Nefrología Pediátrica del Hospital Vall d’Hebron. Materiales y métodos: Estudio retrospectivo de 5 pacientes entre 8 días y 12 años. Se analiza debut, exploraciones diagnósticas, actitud terapéutica y evolución. Resultados: La hipertensión fue el motivo de estudio común. En 2 de los 5 casos se asoció a insuficiencia renal. El diagnóstico definitivo se alcanzo por arteriografía y angio-RM, observándose en todos los casos afectación de las arterias renales (en 3 casos unilateral). El tratamiento fue conservador en 3 casos, 1 caso requirió tratamiento quirúrgico y otro fallece a los 7 meses. Conclusiones: El SAM, aunque poco frecuente, constituye una causa importante de HTA en la infancia. La técnica diagnóstica de elección es la arteriografía. Es importante descartarla posible afectación cerebral ya que condiciona el pronóstico vital. La hipertensión es de difícil manejo médico. Son indicaciones de tratamiento quirúrgico HTA refractaria a tratamiento médico, claudicación intermitente, angina intestinal y compromiso renal por hipoperfusión. De lo contario, se optará siempre por tratamiento médico ya que la lesión es progresiva durante la infancia y tiende a estabilizarse en la pubertad (AU)

Introduction: Mid-aortic syndrome (MAS) describes a narrowing segment of the abdominal aorta and the branches arising from there. This narrowing frequently involves the renal arteries, with the only clinical sign being arterial hypertension. Objective: To review the cases of MAS diagnosed in the Paediatric Nephrology Department of the Vall D’Hebron Hospital (Barcelona). Methods and materials: A retrospective study was conducted on 5 patients aged from 8 days to 12 years old. We analysed the onset, diagnostic examinations, treatment and outcome. Results: Hypertension was the common reason behind the study. In two of the five cases, it was associated with renal failure. The definitive diagnostic was made using angiography and angio-MR, observing, in all the cases, that the renal arteries were affected (in 3 cases unilateral). The treatment was by medication in 3 cases, 1 required surgery and 1 died at 7 months. Conclusions: MAS, although uncommon, is a very important cause of AHT in infancy. Angiography is the only technique to diagnose it. It is important to rule out cerebral involvement, which could lead to a fatal outcome. Hypertension is very difficult to control through medication. Surgery is required only when the hypertension is not controllable through medication, when there is intermittent claudication, intestinal angina, and renal hypoperfusion. Ideally, it is better to opt for medication as treatment, as the narrowing is progressive up to the end of puberty, after which, the narrowing stabilizes (AU)

[Mid-aortic syndrome: case studies from a paediatric nephrology department]. / Síndrome de aorta media.

INTRODUCTION: Mid-aortic syndrome (MAS) describes a narrowing segment of the abdominal aorta and the branches arising from there. This narrowing frequently involves the renal arteries, with the only clinical sign being arterial hypertension. OBJECTIVE: To review the cases of MAS diagnosed in the Paediatric Nephrology Department of the Vall D'Hebron Hospital (Barcelona). METHODS AND MATERIALS: A retrospective study was conducted on 5 patients aged from 8 days to 12 years old. We analysed the onset, diagnostic examinations, treatment and outcome. RESULTS: Hypertension was the common reason behind the study. In two of the five cases, it was associated with renal failure. The definitive diagnostic was made using angiography and angio-MR, observing, in all the cases, that the renal arteries were affected (in 3 cases unilateral). The treatment was by medication in 3 cases, 1 required surgery and 1 died at 7 months. CONCLUSIONS: MAS, although uncommon, is a very important cause of AHT in infancy. Angiography is the only technique to diagnose it. It is important to rule out cerebral involvement, which could lead to a fatal outcome. Hypertension is very difficult to control through medication. Surgery is required only when the hypertension is not controllable through medication, when there is intermittent claudication, intestinal angina, and renal hypoperfusion. Ideally, it is better to opt for medication as treatment, as the narrowing is progressive up to the end of puberty, after which, the narrowing stabilises.

[Usefulness of nuclear magnetic resonance in the diagnosis of aortic coarctation and recoarctation]. / Utilidad de la resonancia nuclear magnética para el diagnóstico de la coartación y recoartación aórtica.

OBJECTIVE: To assess nuclear magnetic resonance (NMR) usefulness in aortic coarctation and recoarctation diagnosis. MATERIAL: 46 NMR performed on 43 patients: aortic coarctation was suspected in 30 patients and recoarctation in 13 by means of clinical, echocardiographic and Doppler examination. METHOD: Comparison of the NMR observations with the previous diagnosis and the surgical and therapeutic catheterism findings. RESULTS: 41 patients were diagnosed of aortic coarctation or recoarctation by NMR, although a second examination was needed in 3 cases. This diagnosis was confirmed in the 26 treated children (surgical treatment or by angioplasty). CONCLUSIONS: NMR offers excellent aortic images; positive diagnoses have a high safety level, but patients with negative diagnoses must be clinically followed; children suffering from native coarctation can be operated without previous angiographic examination.

[Chronic encephalopathy in patients with sex-linked agammaglobulinemia]. / Encefalopatía crónica en pacientes con agamaglobulinemia ligada al sexo.

The clinical and neuroradiological findings of two patients with X-linked agammaglobulinemia, who developed a chronic encephalopathy, are presented. The main neurological manifestations in both patients were: progressive spastic tetraparesis, cortico-subcortical type of dementia and seizures. No infectious agent was identified in either patient. A systematic review of the clinical findings of 37 patients reported in the literature with X-linked agammaglobulinemia and chronic encephalopathy allows the distinction of two subgroups of patients according to their form of presentation (acute or insidious). In each subgroup there are significant clinical differences. The clinical-neuroradiological similarities between this complication and the ones derived from the vertically transmitted form of the human immunodeficiency virus are pointed out. Finally, emphasis is made on the need for CSF viral cultures on patients with X-linked agammaglobulinemia as soon as a neurological complication is suspected.