RESUMEN
BACKGROUND AND AIMS: Patients with cirrhosis and portal hypertension often develop complications relating to hepatic excretory dysfunction. The standard measurement of the hepatic excretion is the constant infusion indocyanine green clearance (ICGCI ) technique. The ICG 15-min retention test (ICG-r15) is faster, more patient friendly, and cheaper. The aims were to compare the ICG-r15 test with the standard method, to assess relations to patient characteristics and survival, and to assess the ICG-r15 level in healthy control subjects. METHODS: This study included 68 patients with cirrhosis and portal hypertension (Child class A/B/C:17/37/14). All patients underwent a full liver vein catheterization and hemodynamic evaluation with determination of ICG-r15 and ICGCI as the reference in a subset of 38 patients. Sixteen healthy control subjects were included for compiling a reference interval. RESULTS: The ICG-r15 was increased in the cirrhotic patients with increasing values in parallel with liver dysfunction (15/41/58%) in Child class A/B/C compared with 7% in the controls (P < 0.001). ICG-r15 correlated highly significantly with the ICGCI (r = -0.96, P < 0.0001) and in a multivariate regression analysis with hepatic venous pressure gradient, markers of liver dysfunction and hyperdynamic circulation (P < 0.05-0.005). In the control group, normal reference values ranged from 0% to 13%. In addition, ICG-r15 was significantly related to mortality in the patient group (P = 0.02). CONCLUSIONS: Indocyanine green-r15 reflects portal hypertension, the degree of hepatic failure, and survival and may replace the standard ICGCI . A more elaborated reference interval needs to be compiled, and the prognostic value of ICG-r15 should be validated.
Asunto(s)
Hipertensión Portal/diagnóstico , Verde de Indocianina , Cirrosis Hepática/diagnóstico , Pruebas de Función Hepática/métodos , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Hipertensión Portal/complicaciones , Hipertensión Portal/mortalidad , Cirrosis Hepática/complicaciones , Cirrosis Hepática/mortalidad , Fallo Hepático/diagnóstico , Masculino , Tasa de Depuración Metabólica , Persona de Mediana Edad , Sensibilidad y Especificidad , Índice de Severidad de la Enfermedad , Tasa de SupervivenciaRESUMEN
In this report, we describe a female child with dysmorphic features and developmental delay. Chromosome microarray analysis followed by conventional karyotyping revealed a ring chromosome 9 with a 12 Mb deletion at 9pter-p23 and a 540 kb deletion at 9q34.3-qter. Four percent of the analyzed cells had monosomy 9. The patient has the features of both the Kleefstra syndrome and the chromosome 9p-syndrome, including trigonocephaly, long philtrum, hypertelorism, and retro-/micronagthia. The deletion of the patient overlaps with several of the proposed critical regions for the 9p deletion syndrome.
Asunto(s)
Anomalías Múltiples/genética , Anomalías Craneofaciales/genética , Craneosinostosis/genética , Discapacidades del Desarrollo/genética , Cardiopatías Congénitas/genética , Hipertelorismo/genética , Discapacidad Intelectual/genética , Anomalías Múltiples/diagnóstico , Deleción Cromosómica , Cromosomas Humanos Par 9/genética , Hibridación Genómica Comparativa , Anomalías Craneofaciales/diagnóstico , Craneosinostosis/diagnóstico , Discapacidades del Desarrollo/diagnóstico , Femenino , Cardiopatías Congénitas/diagnóstico , Humanos , Hipertelorismo/diagnóstico , Hibridación Fluorescente in Situ , Recién Nacido , Discapacidad Intelectual/diagnóstico , Cariotipificación , Cromosomas en AnilloRESUMEN
INTRODUCTION: It has been reported that the survival of patients having sentinel node (SN) biopsy for melanoma the day after lymphoscintigraphy using Tc99m-nanocolloid is worse than that of patients having lymphoscintigraphy and SN biopsy on the same day [1,2]. A possible explanation suggested is that overnight migration of the tracer from SNs to 2nd-tier nodes occurs, causing failure to remove true SNs. MATERIALS AND METHODS: The possibility of overnight tracer migration leading to errors in SN-identification was investigated in 12 patients scheduled for lymphoscintigraphy the day before surgery by repeating SPECT-CT imaging the next morning, before their SN biopsy. The aim was to check whether onward migration of colloid from previously-identified SNs had occurred. RESULTS: No significant migration of Tc99m-nanocolloid occurred overnight in any patient. All nodes reported to be SNs on day 1 imaging were also present and regarded as SNs on day 2 images. No new foci were visualised on day 2, but some that had been identified on day 1 were not seen on day 2. CONCLUSIONS: Since migration of nanocolloid overnight did not occur, this cannot explain the reported survival disadvantage for patients undergoing SN biopsy the day after lymphoscintigraphy. A likely alternative possibility is that inadequate doses of radioisotope were used for next-day procedures, causing the mistaken removal of 2nd-tier nodes instead of true SNs more frequently. Further research is required to explain the reported reduction in survival of patients having next-day SN biopsy procedures, since the possibility has important clinical implications.