RESUMO
Pre-2000, the clinical management of keratoconus centred on rigid contact lens fitting when spectacle corrected acuity was no longer adequate, and transplantation where contact lens wear failed. Over the last decade, outcome data have accumulated for new interventions including corneal collagen crosslinking, intracorneal ring implantation, topographic phototherapeutic keratectomy, and phakic intraocular lens implantation. We review the current evidence base for these interventions and their place in new management pathways for keratoconus under two key headings: corneal shape stabilisation and visual rehabilitation.
Assuntos
Colágeno/metabolismo , Substância Própria/cirurgia , Reagentes de Ligações Cruzadas/uso terapêutico , Ceratocone/terapia , Implantação de Prótese , Terapia Combinada , Córnea/metabolismo , Substância Própria/metabolismo , Procedimentos Clínicos , Humanos , Ceratocone/fisiopatologia , Próteses e Implantes , Transtornos da Visão/reabilitaçãoRESUMO
PURPOSE: To report a case of presumed infective meningoencephalitis complicated by bilateral optic neuritis. METHODS: Interventional case report. RESULTS: A 7-year-old Pakistani girl presented with fever and multiple right-sided focal seizures. Despite empirical treatment with antibiotic, anti-viral and anti-tuberculous therapy for presumed infective meningoencephalitis, she further deteriorated, developing altered consciousness, hemiplegia and severe, bilateral optic neuritis. No infectious agent could be identified. Following the addition of high dose systemic corticosteroid therapy, her optic nerve function in both eyes began to recover. At 3-month follow-up, the hemiplegia had completely resolved, and visual acuity was 6/6 bilaterally with normal pupillary responses. CONCLUSIONS: This case demonstrates that meningoencephalitis of presumed infective origin may be complicated by acute, severe, bilateral optic neuritis, the latter often mediating profound visual loss. In the setting of rapidly deteriorating vision, aggressive corticosteroid treatment, with antimicrobial cover, albeit unproven, remains the mainstay of treatment.
Assuntos
Meningoencefalite/complicações , Neurite Óptica/etiologia , Criança , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imageamento por Ressonância Magnética , Meningoencefalite/diagnóstico , Neurite Óptica/diagnóstico , Neurite Óptica/tratamento farmacológicoRESUMO
This report describes the case of a mucinous cystadenocarcinoma of probable urachal origin that presented with mass effect, precipitating deep venous thrombosis and pulmonary embolism. The patient presented with acute symptoms of leg swelling, pain and dyspnoea, and a vague awareness of lower abdominal distension. Computer tomography showed a cystic mass closely related to the anterior abdominal wall and the superior aspect of the bladder. A 1500 cm(3) cyst adherent to the dome of the urinary bladder was resected on laparotomy. Partial cystectomy was not carried out in the belief that the cyst represented a benign lesion. Subsequent imaging has shown cystic changes in the anterior bladder wall, and the patient has been referred for partial cystectomy.
Assuntos
Cistadenocarcinoma Mucinoso/etiologia , Cisto do Úraco/complicações , Neoplasias da Bexiga Urinária/etiologia , Adulto , Cistadenocarcinoma Mucinoso/diagnóstico por imagem , Cistadenocarcinoma Mucinoso/patologia , Humanos , Masculino , Embolia Pulmonar/etiologia , Tomografia Computadorizada por Raios X , Neoplasias da Bexiga Urinária/diagnóstico por imagem , Neoplasias da Bexiga Urinária/patologia , Trombose Venosa/etiologiaRESUMO
We report four pedigrees of the group of Na(+)-K(+)-leaky red cell disorders of the 'hereditary stomatocytosis' class. Each showed pseudohyperkalaemia because of temperature-dependent loss of K(+) from red cells on storage of whole blood at room temperature. All pedigrees showed an abnormality in the temperature dependence of the 'passive leak' of the membrane to K(+). Two pedigrees, both of which showed a compensated haemolytic state with dehydrated red cells and target cells on the blood film, showed a novel pattern, in which the profile was flat between 37 degrees C and about 32 degrees C then dropped as the temperature was reduced to zero. The third showed the 'shallow slope' profile, with stomatocytes on the blood film and very markedly abnormal intracellular Na(+) and K(+) levels. Minimal haemolysis was present. The fourth pedigree, of Asian origin, showed the shoulder pattern (minimum at 32 degrees C, maximum at 12 degrees C) with essentially normal haematology. Both of these latter two forms have previously been seen in other pedigrees. The first variant represents a novel kind of temperature dependence of the passive leak found in these pedigrees presenting with pseudohyperkalaemia.
Assuntos
Anemia Hemolítica Congênita/sangue , Adulto , Cátions/metabolismo , Permeabilidade da Membrana Celular , Membrana Eritrocítica/metabolismo , Feminino , Humanos , Hiperpotassemia/sangue , Recém-Nascido , Transporte de Íons , Masculino , Linhagem , Fenótipo , Potássio/sangue , Sódio/sangue , TemperaturaRESUMO
The tendency for thrombosis to occur if haemolysis persists after splenectomy is especially marked in "hereditary stomatocytosis", in which the red cell membrane "leaks" Na and K. A 21-year-old woman, who was splenectomized in childhood for a congenital haemolytic state, presented with major pulmonary embolism that recurred despite anticoagulation. Tests showed a significant cation leak with a "shallow-slope" abnormality in temperature dependence. Allogeneic bone marrow transplantation caused the thrombophilic state to cease and subsequently anticoagulation was stopped without recurrence of thromboembolism. However, she died 9 months after transplantation: iron overload, intensified by the transfusion demands of the transplant, was a major factor.
Assuntos
Anemia Hemolítica/terapia , Transplante de Medula Óssea , Trombofilia/terapia , Adulto , Anemia Hemolítica/metabolismo , Anemia Hemolítica/cirurgia , Membrana Eritrocítica/metabolismo , Evolução Fatal , Feminino , Humanos , Sobrecarga de Ferro , Potássio/metabolismo , Embolia Pulmonar/etiologia , Embolia Pulmonar/metabolismo , Sódio/metabolismo , Esplenectomia , Trombofilia/metabolismo , Transplante HomólogoAssuntos
Confidencialidade/legislação & jurisprudência , Educação de Pós-Graduação em Medicina , Ética Médica/educação , Defesa do Paciente/legislação & jurisprudência , Adolescente , Idoso , Criança , Feminino , Infecções por HIV/diagnóstico , Humanos , Masculino , Responsabilidade Social , Revelação da VerdadeRESUMO
Inspiration in medical humanities can be intense and personal, and does not necessarily require the portrayal of health issues.