RESUMEN
INTRODUCTION: Following definitive pull-through for Hirschsprung's disease (HD), a minority of patients develop constipation, incontinence and enterocolitis. The cause for these symptoms in some of the patients is believed to be "internal sphincter achalasia" related to abnormal innervation of the sphincter, with an absent anorectal relaxation reflex. Transanal myectomy, posterior sagittal rectal myectomy, anal dilatation and even intrasphincter injection of botulinum toxin have been used to solve this problem. Nitric oxide (NO) has been identified as the chemical messenger of the intrinsic non-adrenergic, non-cholinergic pathway mediating relaxation of the normal internal anal sphincter when applied topically. BACKGROUND/PURPOSE: To evaluate the effect of topical isosorbide dinitrate (DTN) applied to the anus and its role in the management of patients with HD after pull-through who have ongoing difficulties in stool evacuation. MATERIAL AND METHODS: Four children, aged 2, 5, 7 and 13 years, who all underwent the Soave operation for Hirschsprung's disease, were assessed. Three patients had recurrent episodes of enterocolitis, and all had symptoms of difficulty in rectal/colonic evacuation. Conservative treatment of repeated anal dilatation under anaesthesia had failed to improve symptoms. A rectal myectomy and conversion of Soave to Duhamel procedure had been done in 2 without significant improvement in symptoms. In all patients, ano-rectal manometry was performed before and after application of DTN paste (1 mg/kg/day in two separate doses) which was continued for a minimum period of 3 weeks. Results. Marked symptom improvement was noted in all 4 children. On manometric assessment the median maximum pressure (pre-DTN application) was 165 mm Hg (range 96 - 250), the median sphincter length 2.7 cm (range 2.3 - 3.1) and the high-pressure zone (HPZ) median length 1.6 cm (range 1.2 - 2.1). After application of DTN paste, the maximum pressure dropped by a median of 88 mm Hg (range 46 - 90), total sphincter length shortened to a median of 2.1 cm and the HPZ by a median total length of 1.4 cm (range 0.01 - 0.9). In addition, vector volume was reduced by a median of 59 % (range 40.5 - 77). CONCLUSION: From these results, it is evident that DTN paste is not only an adjunct in the investigation, but can also be used as a temporary form of treatment of obstructive symptoms in Hirschsprung's disease patients.
Asunto(s)
Canal Anal/fisiopatología , Enfermedad de Hirschsprung/cirugía , Dinitrato de Isosorbide/administración & dosificación , Complicaciones Posoperatorias/tratamiento farmacológico , Vasodilatadores/administración & dosificación , Administración Tópica , Canal Anal/inervación , Niño , Preescolar , Humanos , Dinitrato de Isosorbide/uso terapéutico , Manometría , Complicaciones Posoperatorias/fisiopatología , Vasodilatadores/uso terapéuticoRESUMEN
Percutaneous endoscopic gastrostomy (PEG) has become the method of choice for long-term enteral access in the pediatric population. Since its introduction, several common complications have been described. Less well known is the danger of removing or replacing a PEG by cutting the device at skin level without endoscopic assistance to ensure the complete removal of all parts. The aim of the present work is to describe a patient in whom gastrostomy parts were retained after PEG removal, causing bowel obstruction and perforation.
Asunto(s)
Remoción de Dispositivos/efectos adversos , Cuerpos Extraños , Gastrostomía/métodos , Enfermedades del Íleon/etiología , Íleon , Obstrucción Intestinal/etiología , Perforación Intestinal/etiología , Preescolar , Remoción de Dispositivos/métodos , Humanos , MasculinoRESUMEN
A subgroup of self-injuring patients responds positively to the opiate-blocking agent naltrexone in acute, double-blind studies. In this study we examined the effects of naltrexone after acute treatment and the long-term effects of naltrexone on SIB. Rates of SIB were collected from pretreatment baseline; a second baseline a year after the acute trial; and a subsequent 12-month double-blind, placebo-controlled treatment. A subgroup of patients decreased SIB for a year without treatment after acute exposure to naltrexone. Five participants who decreased SIB by 70% after acute treatment increased SIB to the long-term treatment with naltrexone. In contrast, those for whom SIB increased over the one-year treatment hiatus decreased their SIB after the first long-term treatment. Discussion of these complex effects considered the role of background opioid levels, dosing, and treatment regimen of naltrexone and other factors limiting receptor adaptation among patients who exhibit SIB.
Asunto(s)
Naltrexona/administración & dosificación , Naltrexona/uso terapéutico , Antagonistas de Narcóticos/administración & dosificación , Antagonistas de Narcóticos/uso terapéutico , Conducta Autodestructiva/tratamiento farmacológico , Adulto , Protocolos Clínicos , Estudios Cruzados , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Femenino , Humanos , Masculino , Persona de Mediana Edad , Quimioterapia por Pulso , Resultado del TratamientoRESUMEN
We evaluated the outcome of children with ovarian mass operated on at our Center over an 8-year period. Thirty-four girls aged 1 day to 17 years were included in the study. Mean duration of follow-up was 39.5 months. Eighteen had a nonneoplastic mass and 16 a neoplastic mass, eight of which were malignant. Patients with a malignant tumor underwent adnexectomy of the affected side and appendectomy, without removal of the uterus or the other ovary and without partial omentectomy; only the one girl with bilateral malignant disease had bilateral adnexectomy. Five of the eight patients with malignant disease received chemotherapy. All patients are alive with no evidence of disease. Pediatric ovarian masses are rare but have a relatively high rate of malignancy. They differ from adult malignant tumors in many aspects. Conservative surgery should be applied to preserve fertility and combined, if necessary, with aggressive chemotherapy. A good prognosis may be expected in most cases, even with progressive disease.
Asunto(s)
Neoplasias Ováricas/cirugía , Anexos Uterinos/cirugía , Adolescente , Adulto , Antineoplásicos/uso terapéutico , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Procedimientos Quirúrgicos Menores , Estadificación de Neoplasias , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/tratamiento farmacológico , Ovariectomía , Tomografía Computarizada por Rayos XRESUMEN
A large, nonspecific, chronic ulcer was found in the sigmoid colon of a 13-year-old child with neglected, undiagnosed Hirschsprung's disease (HD). There is no known association between HD and colonic ulcers, suggesting that the ulcer was a true stercoraceous ulcer of the colon and not an intrinsic defect of the aganglionotic bowel.
Asunto(s)
Enfermedad de Hirschsprung/complicaciones , Enfermedades del Sigmoide/etiología , Úlcera , Adolescente , Humanos , MasculinoRESUMEN
We report the cases of two siblings who died at age 21 and 15 years respectively. Both had optic atrophy, pitressinsensitive diabetes insipidus, and insulin-dependent diabetes mellitus, with onset occurring in early childhood. Although there are now 21 patients from 15 families with this syndrome, this is the first time that necropsy findings have become available. They include the expected atrophy of hypothalamic nuclei and degeneration of the optic nerves, chiasm and tract, as well as a totally unexpected degeneration of the pons and cerebellum.
Asunto(s)
Diabetes Insípida/genética , Diabetes Mellitus Tipo 1/genética , Atrofia Óptica/genética , Adolescente , Insuficiencia Suprarrenal/patología , Adulto , Encéfalo/patología , Aberraciones Cromosómicas/genética , Trastornos de los Cromosomas , Diabetes Insípida/patología , Diabetes Mellitus Tipo 1/patología , Femenino , Genes Recesivos , Cardiopatías Congénitas/patología , Humanos , Masculino , Miocardio/patología , Atrofia Óptica/patología , Quiasma Óptico/patología , Nervio Óptico/patología , Hipófisis/anomalías , Síndrome , Sistema Urinario/anomalíasRESUMEN
Methaqualone produces dose- and time-dependent decreases in susceptibility to electrically, chemically, and sound-induced seizures. The antagonism of methaqualone to electroconvulsive shock can be dissociated from its effects on temperature regulation and plasma corticosterone. Studies with SKF525A, a drug known to block enzymes in the liver that metabolize drugs, suggest that methaqualone, rather than a metabolite produced in the liver, is responsible for its anticonvulsant effects. Tolerance to the anticonvulsant effects of methaqualone is also demonstrated.