Craniofrontonasal dysplasia--a distinct entity with lethality in the male?
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Biomedical subjects
Publications and source records attributed to J R Moore.
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A female child with multiple vertebral and rib abnormalities is described.
An unusual form of hereditary spastic paraplegia is described. Affected females have a late-onset slowly progressive spastic paraparesis. Affected males show oligophrenia with a rapidly progressive spastic quadriplegia. The mode of inheritance is consistent with sex-linkage, with partial manifestation in female carriers.
We studied the effects of hydrocortisone, an inhibitor of phospholipase A2, on the secretion of mucin and release of prostaglandins from guinea pig gallbladder explants. We measured mucin using [3H]glucosamine as a precursor and prostaglandins by radioimmunoassay of 6-keto-prostaglandin F1 alpha. Mucin secretion and prostaglandin release were studied under basal conditions and after arachidonate stimulation. Hydrocortisone sodium succinate reversibly inhibited basal secretion of mucin by 24% at 10(-5) M (P less than 0.05 compared with control) and 34% at 10(-4) M (P less than 0.01). Hydrocortisone, 10(-4) M, also reversibly inhibited arachidonate-stimulated secretion of mucin (P less than 0.01 compared with controls incubated with arachidonate alone). Release of prostaglandin F1 alpha was significantly inhibited by hydrocortisone under basal (P less than 0.01) and arachidonate-stimulated (P less than 0.01) conditions. The inhibitory effect of hydrocortisone was mediated by inhibition of hydrolysis of arachidonate from membrane phospholipids, suggesting that exogenous arachidonate is incorporated into membrane phospholipids prior to conversion to prostaglandins.
This paper relates recent modes of dental practice to changes that the public and government are likely to ask the health care professions to make in the future. As usual they are asking for the best of all worlds. First, that we maintain the clinical model to the highest standards of personal dental care based and tested against the best research at our disposal, whilst we ensure there is no reduction in the high technical standards for which british dentists have a reputation. Second, that the profession is required to consider ways of providing care on the medicosocial model for the whole community at an economic level the country will afford. The broad changes in dental education have been reviewed, from the technical apprenticeship to the establishment of strong university departments in teaching hospitals. The importance of a sound biomedical foundation and of research both to education and the credibility of dental practice as a primary health care profession is stressed if the profession is to retain its position as a sister to medicine and not slide down to that of a technical ancillary.
We evaluated the efficacy of free tissue transfer in the treatment of osteomyelitis in thirty-three patients who were followed for an average of 41.4 months (range, twenty-four to fifty-nine months). The bone lesions were classified in two ways: first, according to whether there had been drainage for less or more than six months and second, according to whether the lesion was Type I, II, or III. The thirty-three patients underwent a total of thirty-seven free tissue transfers, and thirty (79 per cent) of the transfers survived. Major complications were encountered after 41 per cent of the surgical procedures, consisting of failure of the free tissue transfer in seven patients (21 per cent) and recurrent sepsis in six (20 per cent) at follow-up. Four of the seven patients in whom the free tissue transfer failed underwent an amputation. Six of the patients with a failed transfer and nine of the ten with recurrent sepsis had a Type-III lesion. Previously published reports have suggested that free tissue transfer is an extremely successful and reliable procedure for the treatment of osteomyelitis. In our series limited success was achieved in patients with a Type-I or II lesion, but six of the patients with a Type-III lesion had recurrence of infection despite a successful transfer.
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Osteocartilaginous lesions, which are considered to be one of the most common neoplasms of bone, are seldom found in the hand. These are distinct from subungual exostoses or turret exostoses. When present in the hand in children, these lesions may limit function, cause cosmetic deformity, alter the growth of a digit, and pose a problem in diagnosis. Experience with these osteocartilaginous lesions in 10 children, (nine boys and one girl) is reviewed. Six patients had some degree of deviation or early rotation of the involved digit secondary to abnormal bone growth. There was no definite history of antecedent trauma; however, most parents assumed this to be a causative factor. Patients were treated by excision of the lesion and joint contouring when necessary. Follow-up ranged from 1.5 to 13 years, and there was good functional restoration, minimal growth disturbance, and no recurrence in any patient.
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Forty-one autogenous vascularized bone grafts have been performed by the authors since 1976. Twenty-two free vascularized fibular grafts were performed in a lower extremity and ten in an upper extremity. Ten of these patients were treated for locally aggressive, benign, or low-grade malignant bone tumors, while the remainder of the patients were treated for massive trauma-derived, segmental bone defects. The average length of the bone defect was 14.9 cm for tumor cases and 16.2 cm for trauma cases. In four patients (12.5%), the operation was unsuccessful, resulting in amputation. Nine patients were treated by osteocutaneous groin flaps, with one failure, resulting in amputation. Vascularized autogenous bone grafts transferred by microvascular anastomoses have been found an effective method of treatment for massive segmental bone defects.
A five-year experience with ten cases of primary resection of aggressive bone lesions treated by immediate reconstruction with autologous fibula demonstrates the advantages of vascularized, compared with free bone, graft. The procedure was performed for locally aggressive bone tumors in seven cases, and for Type III congenital pseudarthroses of the tibia in three cases. In large intercalary bone grafts, the vascularized fibula actively participates in the healing process, rather than serving merely as a framework for inductive creeping substitution, as is the case in conventional bone grafts. With the exception of one case, the grafts united and progressively hypertrophied, and there was no tumor recurrence. For defects less than 6 cm in diameter, conventional bone grafts usually prove successful. However, for defects greater than 6 cm or in a poorly vascularized bed, the vascularized fibular graft can prevent such complications as fatigue fracture, failure of incorporation, and nonunion that are frequently seen with nonvascularized cortical and cancellous bone grafts.
Seven cases are reported to illustrate the various soft tissue procedures employed for reconstructing severely injured legs with tibial fractures. Microvascular free tissue transfer is a new and often successful solution to some of the most difficult of all lower limb open fractures.
This paper reports SP changes pre- and post-operatively in a series of 12 patients who have undergone ultrasonic labyrinthectomy for Menière's disorder. The unoperated ear in 7 of these patients also had pre- and post- operative ECochG. The SP in the operated ear was reduced in amplitude and duration in 50 per cent and 66 per cent of ears respectively. However, the SP was also reduced in the unoperated ears by similar proportions. Statistical analysis showed no significant difference in SP change between the two sets of ears. It is concluded that the value of the SP in prediction or assessment of results of treatment in Menière's disorder is not at present proven, and that future investigations require the use of controls.
Amputated or devascularized digits should be appropriately cooled as quickly as possible. The injured extremity is dressed, splinted and elevated. Careful selection of patients for replantation has yielded satisfying results to both patients and surgeons. Replantation is indicated for a thumb or multiple-digit amputation or proximal-level injuries. However, single-finger replantation (except in a child or when extenuating circumstances exist) may not be warranted. Success depends on function, not merely on making the digit viable.
In a 56-year-old woman with severely deforming rheumatoid arthritis, surgical exploration demonstrated partial rupture of one ulnar nerve and complete rupture of the other.
Cryosurgery in obstructive non-allergic vasomotor rhinitis is a simple outpatient procedure with as good results as the more time-consuming and elaborate procedure of submucosal diathermy.