ABSTRACT
Oculodental digital dysplasia (ODDD) is an uncommon inherited disorder with characteristic facial abnormalities, defects in dentition and syndactyly; previous reports have described neurologic dysfunction. We present neuroimaging features of a mother and daughter with ODDD; despite only the mother having neurologic dysfunction, the MRI findings in both are strikingly similar.
Subject(s)
Brain/pathology , Dental Enamel Hypoplasia/genetics , Magnetic Resonance Imaging , Microphthalmos/genetics , Syndactyly/genetics , Adolescent , Adult , Chromosome Aberrations/genetics , Chromosome Disorders , Dental Enamel Hypoplasia/diagnosis , Female , Genes, Dominant/genetics , Humans , Microphthalmos/diagnosis , Neurologic Examination , Syndactyly/diagnosis , SyndromeABSTRACT
Four male patients with the age from 31--43 years with lymphogranulomatosis and a 23 year old patient with chronic myeloic leukemia developed aseptic necrosis of the femoral head 15 to 69 months after treatment with a combination of cytostatic substances and steroids. In a patient who received maximal prednisone applications aseptic necrosis of the humeral head has been observed also. With increasing life expectancy asceptic bone necrosis due to treatment can appear as a late complication. The early recognition is important. The advancing of this irreversible bone destruction and the associated morbidity can be avoided by immediate cessation of the steroid application. Conservative treatment is indicated.
Subject(s)
Antineoplastic Agents/adverse effects , Femur Head Necrosis/chemically induced , Hodgkin Disease/drug therapy , Leukemia, Myeloid/drug therapy , Prednisone/adverse effects , Adult , Antineoplastic Agents/therapeutic use , Femur Head Necrosis/diagnostic imaging , Femur Head Necrosis/therapy , Humans , Humerus , Male , Osteonecrosis/chemically induced , Prednisone/therapeutic use , RadiographyABSTRACT
Exploratory laparotomy with splenectomy was performed on 275 patients with hisologically confirmed Hodgkin's disease. In 188 patients the laparotomy was a primary one to determine more precisely the state of the disease. A secondary laparotomy was performed in 87 patients 1-12 years after diagnosis and radiotherapy. In 17.5% of patients the state had to be revised after laparotomy with splenectomy. In 38 the disease had further progressed, while in ten it had slighty regressed. In four cases clinical stage I proved to be stage III. Even prognostically more favourable forms may have progressed at first diagnosis. There was no correlation between B-symptoms and histological type, but there was between B-symptoms and spread of the disease. Calculated spleen weight provided no clue as to spleen involvement. There was no clear relationship between spleen involvement and histological subclassification. Risk-effect analysis indicate that laparotomy with splenectomy was useful because it makes optimal treatment possible.
