RESUMEN
Startle disease or hyperreflexia is an autosomal dominant neurological disorder, with a neonatal onset, characterized by muscular hypertonia and myoclonic jerks, exaggerated by the slightest stimulus. Low concentrations of free gamma-aminobutyric acid (GABA) have been found in the cerebrospinal fluid of two affected infants. The involvement of GABA or its receptors has been raised and the use of GABA-agonist drugs has been suggested. We report a newborn with startle disease who also had a low concentration of GABA in the cerebrospinal fluid. No clinical improvement was observed with progabide, a GABA agonist. Furthermore, a high dose (100 mg/kg) of gamma-hydroxybutyrate (GHB) did not improve muscular stiffness and failed to induce general anesthesia. GHB, currently used as an effective general anaesthetic, is a structural analogue of GABA. It is present naturally at low concentrations in the brain and is regarded as an inhibitory neurotransmitter. Two specific GHB receptors, distinct from the GABA receptors, have been identified in rat brain. Failure to induce general anesthesia with a high dose of GHB suggests that one of these receptors could be involved in startle disease.
Asunto(s)
Enfermedades Neuromusculares/tratamiento farmacológico , Enfermedades Neuromusculares/fisiopatología , Receptores de Superficie Celular/fisiología , Reflejo Anormal/fisiología , Reflejo de Sobresalto/fisiología , Oxibato de Sodio/uso terapéutico , Anestésicos , Anticonvulsivantes/uso terapéutico , Agonistas del GABA/uso terapéutico , Humanos , Recién Nacido , Masculino , Reflejo Anormal/efectos de los fármacos , Reflejo de Sobresalto/efectos de los fármacos , Oxibato de Sodio/farmacología , Ácido gamma-Aminobutírico/análogos & derivados , Ácido gamma-Aminobutírico/líquido cefalorraquídeo , Ácido gamma-Aminobutírico/uso terapéuticoAsunto(s)
Obstrucción Intestinal/tratamiento farmacológico , Opio/uso terapéutico , Extractos Vegetales/uso terapéutico , Complicaciones Posoperatorias/tratamiento farmacológico , Ensayos Clínicos como Asunto , Método Doble Ciego , Femenino , Homeopatía , Humanos , Masculino , Distribución Aleatoria , VerdurasRESUMEN
A review of clinical, pathological findings in 3 cases of necrotising enterocolitis is presented, as well as considerations about pathogenesis. The authors stress the benefits of hyperbarie oxygen in the treatment of the disease.
Asunto(s)
Enterocolitis Seudomembranosa/diagnóstico , Adulto , Anciano , Enterocolitis Seudomembranosa/etiología , Enterocolitis Seudomembranosa/terapia , Humanos , Oxigenoterapia Hiperbárica , Infecciones/complicaciones , Mucosa Intestinal/patología , Masculino , Persona de Mediana Edad , Enfermedades Vasculares/complicacionesRESUMEN
A so-called "phase II" clinical trial was carried out in 89 patients with digestive carcinomas who were treated either by chemotherapy alone or by a combination of surgery and chemotherapy. Thirty-five patients with inoperable tumours received chemotherapy alone without any noticeable improvement. Thirty-two patients had tumours which were operable but with inoperable extension. Palliative chemotherapy, given after surgery to reduce the size of the tumour, resulted in a prolongation of survival. Twenty-two patients with operable tumours without inoperable extension benefited from routine adjuvant chemotherapy.