Onset, progression, and plateau of skeletal lesions in fibrous dysplasia and the relationship to functional outcome.

Most lesions in FD and their attendant functional disability occur within the first decade; 90% of lesions are present by 15 years, and the median age when assistive devices are needed is 7 years. These findings have implications for prognosis and determining the timing and type of therapy. INTRODUCTION: Fibrous dysplasia of bone (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Variable amounts of skeletal involvement and disability occur. The age at which lesions are established, the pace at which the disease progresses, if (or when) the disease plateaus, and how these parameters relate to the onset of disability are unknown. To answer these questions, we performed a retrospective analysis of a group of subjects with FD. MATERIALS AND METHODS: One hundred nine subjects with a spectrum of FD were studied for up to 32 years. Disease progression was assessed in serial (99)Tc-MDP bone scans by determining the location and extent of FD lesions using a validated bone scan scoring tool. Physical function and the need for ambulatory aids were assessed. RESULTS: Ninety percent of the total body disease skeletal burden was established by age 15. Disease was established in a region-specific pattern; in the craniofacial region, 90% of the lesions were present by 3.4 yr, in the extremities, 90% were present by 13.7 yr, and in the axial skeleton, 90% were present by 15.5 yr. Twenty-five of 103 subjects eventually needed ambulatory aids. The median age at which assistance was needed was 7 yr (range, 1-43 yr). The median bone scan score for subjects needing assistance was 64.3 (range, 18.6-75) compared with 23.1 (range, 0.5-63.5) in the unassisted subjects (p < 0.0001). Among subjects needing assistance with ambulation, 92% showed this need by 17 yr. CONCLUSIONS: The majority of skeletal lesions and the associated functional disability occur within the first decade of life. The implication is that the window of time for preventative therapies is narrow. Likewise, therapeutic interventions must be tailored to where the patient is in the natural history of the disease (i.e., progressive disease [young] versus established disease [older subjects]). These findings have implications for prognosis, the timing and type of therapy, and the development of trials of new therapies and their interpretation.     

The myth of the final hazard assessment

Hazard assessment is a basic tool used in the evaluation and regulation of chemicals, but in spite of this there are many different interpretations of the scope and function of a hazard assessment. Starting from a discussion of the essential elements and conceptual frames involved, an attempt is made to identify the sources of uncertainty that are inherent in a hazard assessment. Problems identified include unambiguous identification of the chemical source, the routes of exposure, and the possible targets to be considered. Inadequacies in effects data are frequent, but unlike many of the other sources of uncertainty, these are often compensated for by the use of safety factors when setting quantitative limits for human or environmental exposure. The inherent uncertainties in the process make periodic revisions of any hazard assessment process inevitable, as new knowledge on routes of exposure and targets becomes available.     

Bacterial meningitis in the first three months of life.

A retrospective study of infants with bacterial meningitis admitted to our hospital during 1949-52, highlighted the lack of ''classical'' signs of meningitis in these infants. We carried out a similar review of 44 infants aged less than three months, admitted during 1982-91. We also determined the causative organisms and their antibiotic sensitivities. Symptoms and signs were similar in the two series. Forty infants in the later series were either febrile, irritable or had seizures on the day of admission. Overall mortality fell from 30% to 11%. Between 1982 and 1991 Group B Streptocococcus and Neisseria meningitidis were the commonest causes of meningitis. All organisms, except one, were sensitive to ampicillin and/or cefotaxime. Bacterial meningitis should be suspected in young infants who are febrile, irritable or having seizures. Initial treatment with ampicillin and cefotaxime is appropriate.     

An analysis of fluid loss during transcervical resection of submucous myomas

Objective: To determine the contribution of several variables to fluid loss during transcervical resection of submucous myomas.Design: An observational study using multiple linear regression analyses.Setting: A university-affiliated training hospital and a university department of clinical epidemiology and biostatistics.Patient(s): Patients with submucous myomas.Intervention(s): Transcervical resection of submucous myomas and monitoring of fluid loss.Main Outcome Measure(s): Patient age, uterine enlargement, treatment with GnRH analogues or 8-ornithine-vasopressin, type of anesthesia, number of myomas, intramural extension of the myoma (type of myoma), and operating time were tested as variables.Result(s): Only intramural extension of the myoma and operating time were obviously related to fluid loss. For the other variables, such a relation was weak at best. The relation between fluid loss and operating time was not modified by any of the other variables.Conclusion(s): Because fluid loss is an important limiting factor in the transcervical resection of submucous myomas, special attention should be paid to reduction of the operating time and preoperative assessment of the intramural extension of the myoma to guide appropriate patient selection.     

Ruptured petrous carotid aneurysm presenting with otorrhagia and epistaxis.

Aneurysm formation and rupture within the petrous internal carotid artery (ICA) is an extremely rare occurrence with approximately 10 such cases in the literature. Etiologies of petrous ICA aneurysms include atherosclerosis, closed head trauma, iatrogenic injury during mastoid surgery, chronic middle ear infections, and congenital causes. Therapeutic options include carotid artery ligation, aneurysm resection with or without reconstruction, and radiographically controlled vessel occlusion. The case of a patient who presented with otorrhagia, epistaxis, and transient focal neurologic signs due to a ruptured petrous ICA aneurysm is presented. The incidence, etiology, and anatomy of these aneurysms is reviewed, and the various tests for determining adequacy of collateral cerebral blood flow are described. Factors that affect the selection of surgical versus radiologic control of these lesions are also discussed.     

Induction of type II collagen-specific antibody production in blood lymphocyte cultures of rhesus monkeys (Macaca mulatta) with collagen-induced arthritis using the immobilized native antigen.

Peripheral blood mononuclear cells (PBMC) from Rhesus monkeys previously immunized with bovine type II collagen to induce arthritis were cultured with the same antigen. Because the native protein is poorly soluble in culture medium a heating step is often used. The antigen in this form induced PBMC proliferation, but epitopes for the induction of antibody production and arthritis were lost. To keep the native protein intact it was coated on affigel beads. With the immobilized antigen specific antibody production could be induced.     

Staging and prognostic factors in carcinoma of the base of the tongue.

During the period 1966-1985, 66 patients were submitted for curative treatment of a carcinoma of the base of the tongue in the Netherlands Cancer Institute. Treatment consisted of radiotherapy (59 patients), surgery and post-operative radiotherapy (4 patients) and surgery alone (3 patients). Patients were staged according to the UICC (1982) and UICC (1987)/AJCC (1988) criteria. Regrouping by the latter system caused enlargement of the N2-group and of stage IV. The crude 5-year survival was 22%, the 5-year tumour control was 36% and the locoregional control was 47%. The most important prognostic factors for the tumour-free interval are the T-category (P = 0.01) and stage grouping (UICC 1982) (P = 0.022). The same factors predict the locoregional control (P = 0.005 and 0.02 respectively). Crude survival is lower in smokers, and in patients in poor general condition (P = 0.04 and 0.007 respectively).