RESUMEN
BACKGROUND: Jejunoileitis (JI) is an unusual manifestation of Crohn's disease (CD) that has been associated with high morbidity and the frequent need for surgical intervention. Although the disease has been well-described in adults, the true prevalence and clinical phenotype in children is unknown. AIM: To compare the clinical course and nutritional impact of CD in children with and without proximal small bowel involvement. METHODS: Patients with either Crohn's jejunitis or JI with or without colonic involvement were identified through a clinical database (1996--2002). All radiologic studies were reviewed by an experienced radiologist blinded to the clinical diagnosis. Thirty-six patients with CD without histologic or radiologic signs of proximal small bowel involvement were used for comparison. All medical, surgical, and hematologic parameters were compared in both disease groups. RESULTS: Among the 134 patients with CD, 23 (17%) had radiologic signs of JI, including intestinal fold thickening (57%), luminal narrowing (31%), and skip lesions (13%). Enteric fistula (6%) and strictures (6%) were less common. Patients with JI were likely to be stunted at the time of diagnosis, require surgical intervention (P < 0.03) and nutritional therapy in the form of nasogastric tube feeds (P < 0.03). Nutritional therapy was also associated with an improvement in height in patients with proximal small bowel disease (OR:5.87). DISCUSSION: JI is a relatively common disease phenotype in children with CD that requires aggressive nutritional and surgical intervention. Future studies are required to determine if the early detection and use of immune modulators may lessen the morbidity associated with proximal small bowel disease.
Asunto(s)
Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/rehabilitación , Ileítis/etiología , Enfermedades del Yeyuno/etiología , Apoyo Nutricional , Niño , Femenino , Humanos , Ileítis/terapia , Enfermedades del Yeyuno/terapia , Masculino , Fenotipo , Pronóstico , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
Hepatic resection for benign pathology is rare. We report our experience in two patients with hepaticolithiasis. One patient underwent left hemihepatectomy with removal of segments II, III and IV for localized Caroli's disease with multiple intrahepatic calculi. The second patient underwent resection of segment II and III with Roux-en-Y hepaticojejunostomy for right and left intrahepatic calculi with an abscess in the left lobe of the liver and a choledochal cyst.
Asunto(s)
Colelitiasis/cirugía , Hepatectomía , Hepatopatías/cirugía , Adulto , Enfermedad de Caroli/complicaciones , Colelitiasis/complicaciones , Femenino , Conducto Hepático Común , Humanos , Hepatopatías/complicaciones , Persona de Mediana EdadRESUMEN
Advances in genetic testing have confirmed the presence of susceptibility loci on chromosomes 12 and 16 for UC and CD. These loci show a strong association with particular disease phenotypes that may explain the clinical heterogeneity of IBD. Whether multiple genotypes will be found to explain these phenotypes remains to be determined. Pharmacogenetic differences in 6-mercaptopurine metabolism can be used clinically to predict patient susceptibility to drug-induced toxicity. Novel treatment strategies are being developed at The Johns Hopkins Medical Center Hospital based on these inherent genetic differences. The aim is to improve treatment efficacy and clinical response times and prevent untoward drug-induced toxicity.
Asunto(s)
Enfermedades Inflamatorias del Intestino , Adolescente , Factores de Edad , Niño , Exposición a Riesgos Ambientales , Humanos , Terapia de Inmunosupresión , Enfermedades Inflamatorias del Intestino/diagnóstico , Enfermedades Inflamatorias del Intestino/genética , Enfermedades Inflamatorias del Intestino/inmunología , Enfermedades Inflamatorias del Intestino/terapiaRESUMEN
The association of tylosis with esophageal cancer has been extensively reported but association with gastric cancer is rare. We report a 55-year-old man with familial tylosis and carcinoma of the stomach for which radical gastrectomy was done. Repeat endoscopy 3 years later is normal.
Asunto(s)
Adenocarcinoma/complicaciones , Queratodermia Palmar y Plantar Difusa/complicaciones , Neoplasias Gástricas/complicaciones , Adenocarcinoma/genética , Humanos , Queratodermia Palmar y Plantar Difusa/genética , Masculino , Persona de Mediana Edad , Neoplasias Gástricas/genéticaRESUMEN
HYPOTHESIS: Maternal measles immunity in the United States today is primarily vaccine induced, with corresponding lower antibody titers in infants, as compared to infants born in an earlier era to mothers with naturally acquired measles immunity. We hypothesized that, due to lower titer of passively transferred maternal measles antibody, administration of measles vaccine at 12 months of age would result in seroconversion and antibody persistence comparable to vaccination at 15 months of age. POPULATION: Children at both an urban hospital and a suburban clinic. METHODS: Informed consent was obtained from mothers for the infants to receive M-M-R(R)II vaccine at either 12 or 15 months and to have serum samples obtained before vaccination and 4 weeks post-vaccination (PV). Between 9 and 39 months PV, a third serum sample was obtained from 28% of seroconverters. A diary of adverse experiences was kept for 3 weeks PV. Sera were assayed by a microneutralization assay (NT) and an enzyme immunoassay (EIA) for measles antibody. RESULTS: Both age groups tolerated vaccination well with minor and transient side effects. Forty-four of 47 (94%) 12-month-old infants seroconverted by NT, compared to 45 of 46 (98%) 15-month-olds (p=NS). There was no statistically significant decline in median NT titers or EIA titers in nineteen 12-month-olds and thirteen 15-month olds followed for 9-39 months PV. CONCLUSION: This study showed comparable serologic responses in 12- vs 15-month-old infants born to measles vaccine-immune mothers; however, the sample size was too small to have adequate power and further study is indicated. Titers of antibody were constant in both the 12-month-old and the 15-month-old infants, over a 9-39 month period, suggesting that waning immunity over this period of time is not a problem in either age group.
Asunto(s)
Vacuna Antisarampión/administración & dosificación , Vacuna Antisarampión/inmunología , Factores de Edad , Anticuerpos Antivirales/sangre , Femenino , Humanos , Inmunidad Materno-Adquirida , Esquemas de Inmunización , Inmunización Pasiva , Lactante , Virus del Sarampión/inmunología , Vacuna contra el Sarampión-Parotiditis-Rubéola , Vacuna contra la Parotiditis/administración & dosificación , Vacuna contra la Parotiditis/inmunología , Embarazo , Vacuna contra la Rubéola/administración & dosificación , Vacuna contra la Rubéola/inmunología , Vacunas Combinadas/administración & dosificación , Vacunas Combinadas/inmunologíaRESUMEN
Cerebrospinal fluid (CSF) analysis for free, bound and total N-Acetyl Neuraminic Acid (NANA) as well as serum NANA was done in 68 patients of bacterial meningitis, of which 37 cases were of pyrogenic meningitis and 31 of tuberculous meningitis. Ten patients were included in the control group. The free NANA levels were increased in only pyogenic meningitis, independent of protein levels but the bound form increased with the increase in CSF proteins. The increase of free NANA in CSF of pyogenic meningitis patients was not related to the cell count or sugar content in CSF or to the duration or severity of illness. This finding can be of great help in differentiating cases of pyogenic meningitis, particularly partially treated patients, who may have ambiguous pictures of CSF analysis, from the cases of tuberculous meningitis.