Effect of Early Corticosteroid Therapy for Landau-Kleffner Syndrome

Four children treated for seizures between 1980 and 1986 were diagnosed as having Landau-Kleffner syndrome (acquired aphasia with convulsive disorder), following the onset of aphasia. They received early and prolonged ACTH or corticosteroid therapy, with high initial doses. In all four cases the EEG promptly became normal, with subsequent long-lasting remission of the aphasia and improvement of seizure control. Three to six years after discontinuation of hormone therapy the children are off medication and free from seizures and language disability.     

Effect of Bupivacaine and Levobupivacaine on Exocytotic Norepinephrine Release from Rat Atria

Background: The cardiotoxic mechanism of local anesthetics may include interruption of cardiac sympathetic reflexes. The authors undertook this investigation to determine if clinically relevant concentrations of bupivacaine and levobupivacaine interfere with exocytotic norepinephrine release from cardiac sympathetic nerve endings.

Methods: Rat atria were prepared for measurements of twitch contractile force and 3[H]-norepinephrine release. After nerve endings were loaded with 3[H]-norepinephrine, the tissue was electrically stimulated in 5-min episodes during 10 10-min sampling periods. After each period, a sample of bath fluid was analyzed for radioactivity and 3[H]-norepinephrine release was expressed as a fraction of tissue counts. Atria were exposed to buffer alone during sampling periods 1 and 2 (S1 and S2). Control atria received saline (100 [mu]l each, n = 6 atria) in S3-S10. Experimental groups (n = 6 per group) received either bupivacaine or levobupivacaine at concentrations (in [mu]M) of 5 (S3-S4), 10 (S5-S6), 30 (S7-S8), and 100 (S9-S10).

Results: Bupivacaine and levobupivacaine decreased stimulation-evoked fractional 3[H]-norepinephrine release with inhibitory concentration 50% values of 5.1 +/- 0.5 and 6.1 +/- 1.3 [mu]m. The inhibitory effect of both local anesthetics (~70%) approached that of tetrodotoxin. Local anesthetics abolished the twitch contractions of atria with inhibitory concentration 50% values of 12.6 +/- 5.0 [mu]m (bupivacaine) and 15.7 +/- 3.9 [mu]m (levobupivacaine). In separate experiments, tetrodotoxin inhibited twitch contractile force by only 30%.     

Inflammatory central nervous system disease in lupus-prone MRL/lpr mice: comparative histologic and immunohistochemical findings

The brains of pathogen-free autoimmune MRL/lpr, NZBWF1 and NZB mice were examined for central nervous system (CNS) inflammation in premoribund 8-week-old animals and at ages when active systemic lupus erythematosus (SLE) was present. CNS inflammation was observed only in MRL/lpr mice. Immunohistochemical studies of brains from young MRL/lpr mice found that infiltrates were composed primarily of CD4+ cells. Older MRL/lpr mice (22 and 26 weeks of age) had CD4+ cells predominantly, but CD8+ and B220+ cells were also present. Perivascular leakage of IgG was a prominent and unexpected finding in the MRL/lpr model. Congenic MRL/+ mice with late-onset autoimmunity had no inflammatory cells in brain tissue, and there was no perivascular staining with IgG or albumin. Our findings suggest that MRL/lpr mice are a useful model for studies of lupus-associated CNS inflammatory disease, and perivascular leakage may be a primary mechanism for entry of IgG into the brain.     

Acral Malignant Melanoma and Striated Palmoplantar Keratoderma (Brunauer-Fohs-Siemens Syndrome): A Fortuitous Association?

Background. Striated palmoplantar keratoderma or Brunauer-Fohs-Siemens syndrome is a very rare, focal, nonepidermolytic palmoplantar keratoderma with autosomal inheritance. Unlike other palmoplantar keratodermas, no association with visceral or skin cancer has ever been reported.
Objective. We report a case of malignant melanoma arising in the hyperkeratotic lesions on the right heel of a patient with striated palmoplantar keratoderma. The lesion was completely excised; our patient also underwent sentinel lymph node biopsy and then was treated with high-dose interferon adjuvant therapy.
Methods. Sentinel lymph node biopsy incision was made in elliptical fashion, long enough to harvest a full-thickness skin graft to cover the wide local excision defect. The skin graft was defatted by sharp dissection. Several perforations were made in graft and it was secured in place with sutures and bolster dressing.
Results. At follow-up, the grafted skin showed hyperkeratotic changes but no local or systemic signs of the disease was observed.
Conclusion. The association between striated palmoplantar keratoderma and acral melanoma is discussed.     

Health insurance and private providers: A study of the civil servants' medical benefit scheme in bangkok,Thailand

Compulsory health insurance schemes have received attention recently as a means of creating a reliable extra-budgetary source of health care funding. Yet, the full implications of such schemes in a developing country context are unclear; in particular, the impact on and relationship with private sector providers has infrequently been explored. This study examines patterns of public and private sector utilization under the Civil Servants' Medical Benefit Scheme (CSMBS) in Bangkok, Thailand. The CSMBS currently provides limited reimbursement for private inpatient care, but recent proposals suggest increasing benefit levels for care sought in the private sector. The study shows that despite high level of cost recovery in the public sector, charges were much lower than those in the private sector. Different patterns of diagnosis in the two sectors were found with private hospitals tending to treat a less complex case mix. Within the private sector, there was a limited tendency to specialize in certain types of care. It is concluded that under the current payment mechanism of fee-for-service reimbursement, measures to enhance access to private sector care should be approached with caution. In the long run, the scheme should merge with the recently established social security scheme.     

Promoting autonomy and independence for older people within nursing practice: a literature review

The principles of promoting autonomy and independence underpin many approaches to improving the quality of nursing care for older people in whatever setting, and are in line with wider developments in health care such as the Patient's Charter. However, these concepts require careful definition if nursing practices which might promote autonomy and independence are to be identified. Although the generalizability of the research-based literature in this field is limited by a focus upon older people in continuing-care settings, a review of the literature found a number of indicators associated with attempts to promote patient autonomy and independence. These were grouped into the following categories: systems of care delivery which promote comprehensive individualized assessment and multidisciplinary care planning; attempts to encourage patients/clients to participate in decisions about their care; patterns of communication which avoid exerting power and control over patients/clients and attempts to modify the environment to promote independence and minimize risk. It is suggested that the review identifies a number of principles for nursing practice which can be applied in a range of care settings in order to promote the autonomy and independence of older people.     

The unusual association of three autoimmune diseases in a patient with Noonan syndrome.

We report on a 26-year-old female affected by Noonan syndrome (NS), a congenital disorder characterized by various phenotypic features and congenital anomalies) associated with a variety of autoimmune diseases, including systemic lupus erythematosus, celiac disease, and Hashimoto thyroiditis. Autoimmunity is seldom described in NS and the association between this congenital disease and three autoimmune disorders has not been previously reported. Should the occurrence of autoimmune disorders in NS be confirmed, a relevant clinical and laboratory evaluation of NS patients should be performed in order to clarify whether the immune system involvement represents only an occasional event or is a feature of the disease.