The scleroatrophic syndrome of Huriez: a cancer-prone genodermatosis

We report a 24-year-old woman, her 6-year-old son and her 17-month-old daughter, who all suffer from a rare congenital genodermatosis first delineated by Huriez et al. in the 1960s. The clinical features of this autosomal dominant condition include scleroatrophy of the hands and feet, nail hypoplasia, mild palmoplantar keratoderma and hypohidrosis. Histological changes are non-specific, but immunohistological and ultrastructural examination in our index patient revealed an almost complete absence of epidermal Langerhans cells in the affected skin. This new finding may be linked to the cancer proneness of the scleroatrophic skin. In this family, the grandmother had died at the age of 37 years from metastatic squamous cell carcinoma which had arisen on the thenar eminence.     

The role of gentle ventilation in prevention of subglottic stenosis in the newborn

Although stuttering has long been acknowledged as a familial disorder, the nature of a genetic component remains unclear. Most previous data used in genetic studies were obtained primarily from adults who stutter and may be biased in several respects. The purpose of this investigation was to quantify the frequency of stuttering in relatives of preschool-age children who stutter, and who were first seen close to the onset of the disorder. Detailed pedigrees (family trees), including first-, second-, and third-degree relatives, were obtained from parents of 69 children who stuttered. We found, as have previous studies, that more male than female relatives ever stuttered, but that female subjects who stuttered had more female relatives who ever stuttered than did male subjects. In order to identify the genetic model most consistent with the observed patterns of stuttering transmission, we conducted segregation analyses. Results from these analyses suggest that transmission of a single major genetic locus increasing the liability to stuttering best accounts for the transmission of stuttering in families of preschool-age children who stutter.     

Measurement of polyene antibiotic-mediated erythrocyte damage by release of hemoglobin and radioactive chromium

Polyene antifungal antibiotics produce various degrees of membrane damage in sheep erythrocytes in vitro. Mediocidin, filipin, amphotericin B, and candicidin were found to result in greater damage than nystatin, pimaricin, and amphotericin B methyl ester. The degree of sensitivity of the cells varied by 100-fold for mediocidin verus amphotericin B methyl ester as measured by curves of hemoglobin release versus drug concentration. In erythrocytes prelabeled with radioactive chromium, release of the isotope through polyene-damaged cell membranes was found to occur at lower drug concentrations than measurable hemoglobin release, and the percentage of isotope released at the highest drug dose was consistently greater than the percentage of hemoglobin released. Thus, the isotope assay is a more sensitive indicator of polyene-induced membrane damage in the test system. These significant differences in release of molecules through polyene-induced membrane lesions indicate the complex nature of the binding and further interactions of this class of drugs with the plasma membrane.     

Safety of dipyridamole testing in 73,806 patients: the Multicenter Dipyridamole Safety Study

Use of the acute hospital has markedly decreased over the past four decades for various reasons: the decentralization of diagnostic treatment technologies to out-of-hospital sites; the clinical substitutions of quick diagnostic testing of the ambulatory patient for the longer diagnostic testing of the hospitalized patient; the diminished use of hospital bed rest and the expanded use of exercise for treatment; the corporate organization of hospital work that emphasizes efficiency; and the group practice organization of generalists and specialists that avoids hospital use for the diagnosis of complex disorders in ambulatory patients. A smaller domain for hospital bed care and renewed attention to chronic disease and prevention in the community diminish the hold of the acute hospital on care. The evolution of more collaborative, decentralized arrangements promises to be a positive development for community care.     

Monitoring drug concentrations in a case of combined overdosage with primidone and methsuximide

We describe a case of fatal overdosage with primidone and methsuximide. During the early phase of the patient's hospital course we found concentrations of methsuximide, N-desmethylmethsuximide, and primidone in serum that far exceeded the usual therapeutic concentrations, as determined by gas-liquid chromatography. Determination of N-desmethylmethsuximide in peritoneal fluid demonstrated concentrations comparable to those in serum. This led to the therapeutic decision to manage the patient by dialysis. Subsequently, serum samples collected during the course of hospitalization were analyzed quantitatively by gas-liquid chromatography for methsuximide, N-desmethylmethsuximide, primidone, phenobarbital, and diphenylhydantoin. Selected serum specimens were also analyzed by gas chromatography-mass spectrometry, and N-methyl-2-hydroxymethyl-2-phenylsuccinimide, a metabolite of methsuximide not previously described in human serum, was identified by analysis of its mass spectrum.     

Binding of murine myeloma proteins of different Ig classes and subclasses to Fc-reactive surface structures in gram-positive cocci

The paper presents the results of a behavioural programme designed to increase social interaction and activity in the locked ward of a psychiatric hospital. The intervention used a method of information feedback to staff to encourage them to make informed choices of targets and methods of change. The rationale was that this would enhance generalization and programme maintenance. Evidence is presented to show the extent of change obtained after 6 months.     

Are the reciprocal sliding of protofibrils and the shortening of thick filaments during muscle contraction based on a common molecular mechanism?]

It is assumed that the back stroke of myosin bridges in a contracting fibre is determined by nucleotide binding. Then an inhibition of actomyosin dissociation can lead to the shortening of the thick filaments. Possible existence of a protein control system, suppressing this dissociation is discussed.