Care of the dead (and lack of it) at 19th century posts.
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History, Modern 1601-↗
Biomedical subjects
M L Williams
Publications and source records attributed to M L Williams.
Stratum corneum lipid abnormalities in ichthyosis. Detection by a new lipid microanalytical method.
Although the biochemical diagnosis of the ichthyoses is still in its infancy, the two recessively inherited types, recessive X-linked ichthyosis (RXLI) and nonbullous congenital ichthyosiform erythroderma (CIE), are accompanied by stratum corneum lipid abnormalities. However, in RXLI, cholesterol sulfate accumulates; in CIE, massive quantities of n-alkanes accumulate. The diagnosis of these disorders has required large quantities of scale for sequential, quantitative thin-layer chromatography (TLC). In this study, we sought to confirm the previously described lipid abnormalities with the use of a rapid, recently developed microchromatographic technique that employs silica gel-coated quartz rods and flame ionization detection (Iatroscan). The cholesterol sulfate content of RXLI (n = 5) scale and the n-alkane content of CIE (n = 8) scale were determined by both TLC and the microchromatographic technique. Less than 10 mg of scale and even single punch biopsy specimens sufficed for the microchromatographic technique, whereas more than 50 mg of scale were required for TLC. Since the microchromatographic technique can rapidly detect diagnostic biochemical abnormalities from readily obtainable, small tissue samples, this method could eventually supplant or supplement standard lipid biochemical techniques for the diagnosis of cutaneous lipidoses.
Alkanes↗
Acquired ichthyosis: a marker for internal disease.
When ichthyosis occurs in adulthood, it may signify internal disease, including sarcoidosis and malignancy,--especially Hodgkin's lymphoma. Other systemic diseases associated with acquired ichthyosis are leprosy, hypothyroidism, chronic renal failure and nutritional deficiencies.
Adult↗
Alopecia and periorificial dermatitis in biotin-responsive multiple carboxylase deficiency.
Three siblings with infantile-onset biotin-responsive multiple carboxylase deficiency are described. Recognition of the characteristic dermatologic manifestations, alopecia and periorificial dermatitis, should result in early diagnosis and institution of potentially lifesaving therapy with biotin. Other metabolic disorders may present a similar clinical picture. Immunologic dysfunction and/or aberration in lipid or branched chain amino acid metabolism may be the common pathophysiologic link in some or all of these disorders.
Alopecia↗
Rubella screening and immunization of health care personnel: critical appraisal of a voluntary program.
At our health care center 1161 personnel and students were offered rubella antibody testing. Ninety-two percent were serologically screened or had been previously immunized or previously proven seropositive. Males were more likely to refuse serologic screening than females (p less than 0.01). Sixteen percent of the seroscreened individuals were found seronegative to rubella. Eighty-five percent of seronegative women less than or equal to 24 years old accepted immunization, as compared to 62% of older females and 55% of all males (p less than 0.01). The total cost of the program was over $11,000. Literature and data review failed to support an increased risk of rubella transmission and/or major consequences to pregnant patients in the health care setting as compared to the community. Voluntary rubella immunization is well received by seronegative young women. Programs for immunization of other health care personnel are unlikely to decrease the number of cases of congenital rubella, are costly, and are not well accepted. Education and immunization of all young susceptible women remain the best methods to substantially decrease the incidence of congenital rubella in a cost-effective manner in the near future.
Adult↗
The ichthyoses--pathogenesis and prenatal diagnosis: a review of recent advances.
Disturbances in the process of normal cornification leading to pathologic scaling provide the pathophysiologic basis for the ichthyoses. These disturbances may result from either abnormalities in protein metabolism (keratinization) (i.e., the "bricks") or in lipid metabolism (i.e., the "mortar") (Fig. 1). The evidence linking the various ichthyoses to defects in protein or lipid metabolism have been reviewed. It is likely that future advances will lead not only to a more complete understanding of the pathogenesis of these disorders, but also will shed significant light on the normal stratum corneum functions of barrier formation and desquamation, as well as lead the way to more rational and effective therapies. In recent years, prenatal diagnosis has been successfully performed in several of the ichthyoses. It is likely that improvements in our ability to prenatally diagnose those disorders will advance hand-in-hand with further progress in unraveling their underlying causes.
Animals↗
Use of serum ferritin in evaluation of iron deficiency anemia in chronic fetal-maternal hemorrhage.
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Anemia, Hypochromic↗
Perinatal outcome in the absence of antepartum fetal heart rate acceleration.
The perinatal outcome of 27 pregnancies in which antepartum fetal activity testing revealed fetal heart rate acceleration to be either absent or less than 10 beats per minute for 80 minutes is presented. At delivery, each pregnancy was found to have one or more features consistent with uteroplacental insufficiency. These included fetal growth retardation (74%), oligohydramnios (81%), fetal acidosis (41%), meconium (30%), and placental infarction (93%). There were four fetal and seven neonatal deaths, for a perinatal mortality of 41%. Despite delivery of all live-born infants by cesarean section without labor, the infants who died during the neonatal period appeared to be in such poor condition as to preclude survival. The authors conclude that the inability of the fetus to accelerate its heart rate, when not due to maternal medications, is an ominous signal. Throughout the literature on fetal activity testing there are indications that other investigators have had similar experiences. The purpose of the present report is to direct attention to an abnormal fetal activity test result of which the significance is not widely recognized.
Female↗
Human stratum corneum lipids: characterization and regional variations.
The lipids of mammalian stratum corneum are known to be important regulators of skin permeability. Since the human stratum corneum displays remarkable regional variations in skin permeability, we assessed the total lipid concentration, the distribution of all major lipid species, and the fatty acid composition in Bligh-Dyer extracts from four skin sites (abdomen, leg, face, and sole) that are known to display widely disparate permeability. Statistically significant differences in lipid weight were found at the four sites that were inversely proportional to their known permeability. In all four sites, among the polar lipids, the stratum corneum contained negligible phospholipids, but substantially more cholesterol sulfate (1-7%) than previously appreciated. As in the stratum corneum from other mammals, the bulk of the lipids consisted of neutral (60-80%) and sphingolipids (15-35%). Of the neutral lipids, free sterols (4- to 5-times greater than esterified sterols), free fatty acids, triglycerides, and highly nonpolar species (n-alkanes and squalene) predominated. n-Alkanes, which were present in greater quantities than previously appreciated, comprised a homologous series of odd- and even-chained compounds ranging from C19 to C34. The sphingolipids comprised over 80% ceramides vs. lesser quantities of glycosphingolipids. In all four sites, the sphingolipids were the major repository of long-chain, saturated fatty acids. The neutral lipid:sphingolipid ratio generally was proportional to the known permeability of each site: higher neutral lipids and lower sphingolipids generally were associated with superior barrier properties. These studies provide: 1) the first detailed, quantitative analysis of human stratum corneum lipids and 2) information about the variability in lipid composition at four skin sites with known differences in permeability. The latter results suggest that variations in neutral lipids, rather than sphingolipids, may underlie local variations in skin permeability.
Alkanes↗
Human epidermal lipids: characterization and modulations during differentiation.
Using thin-layer chromatography and glass capillary gas-liquid chromatography, we have quantitated the lipids in the germinative, differentiating, and fully cornified layers in human epidermis. As previously noted in nonhuman species, we found progressive depletion of phospholipids coupled with repletion of sterols and sphingolipids during differentiation. The sphingolipids, present only in small quantities in the lower epidermis, accounted for about 20% of the lipid in the stratum corneum, and were the major repository for the long-chain fatty acids that predominate in the outer epidermis. Although the absolute quantities of sphingolipids increased in the outer epidermis, the glycolipid:ceramide ratio diminished in the stratum corneum, and glycolipids virtually disappeared in the outer stratum corneum. Squalene and n-alkanes were distributed evenly in all epidermal layers, suggesting that these hydrocarbons are not simply of environmental or pilosebaceous origin. Cholesterol sulfate, previously considered only a trace metabolite in epidermis, was found in significant quantities, with peak levels immediately beneath the stratum corneum in the stratum granulosum. These studies: 1) provide new quantitative data about human epidermal lipids; 2) implicate certain classes of lipids for specific functions of the stratum corneum; and, 3) shed light on possible product-precursor relationships of these lipids.
Cell Differentiation↗
Diazacholesterol-induced ichthyosis in the hairless mouse. I. Morphologic, histochemical, and lipid biochemical characterization of a new animal model.
Several drugs that interfere with sterol metabolism have been associated with hyperkeratosis in man. We found that 20,25-diazacholesterol (30 to 60 mg/kg/day), administered to hairless mice that were otherwise given normal laboratory chow and water ad libitum, consistently produced ichthyosis after 6 to 9 weeks, an effect that was reversible with removal of drug or with coadministration of a high cholesterol diet. Scaling was most pronounced over the tail, but some stratum corneum retention was noted over the entire skin surface. As measured in frozen sections, stratum corneum thickness was three to 10 times thicker in treated animals than in either controls or revertants. Oil red O-stained frozen sections and freeze fracture replicas revealed decreased stratum corneum membrane lipids in the diazacholesterol-treated animals, but this finding was not specific, since a similar deficit was found in control and revertant tail stratum corneum but not in the stratum corneum from other sites. Stratum corneum lipid extracts revealed reduced total free sterols, reduced cholesterol, accumulation of several normally absent sterol precursors, and increased glycosphingolipids on thin-layer chromatography and high pressure liquid chromatography. In summary, we describe a syndrome of drug-induced ichthyosis in hairless mice that parallels the drug-induced syndrome in man. This syndrome is reversible and accompanied by distinctive abnormalities in cutaneous sterol metabolism. The diazacholesterol model may further our understanding of the pathogenesis of human keratinizing disorders and may provide a valuable analogue for testing new forms of therapy, such as retinoids, for scaling dermatoses.
Animals↗
N-Alkanes in normal and pathological human scale.
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Alkanes↗
Treatment of neonatal hypertension with Captopril.
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Captopril↗
Nonimmunologic hydrops fetalis: a review of 61 cases.
Sixty-one cases of nonimmunologic hydrops fetalis were studied. The incidence of nonimmunologic hydrops fetalis, 1 in 3748 births, was unchanged in 2 decades. The mortality from nonimmunologic hydrops fetalis remained high (98%). Polyhydramnios (75%), anemia (45%), and preeclampsia (29%) were the commonest prenatal maternal complications, whereas postpartum hemorrhage or difficulty with placental delivery, or both, occurred in 64%. Ultrasound examination always was successful in detecting the hydropic state. Many presumed etiologic factors were noted, although in 38% no possible cause was found. A major congenital anomaly was present in 41%. The obstetric and neonatal care of nonimmunologic hydrops fetalis must be improved. If nonimmunologic hydrops fetalis is diagnosed during the investigation of polyhydramnios, consideration should be given to early delivery in the interest of the fetus.
Edema↗
Validation of an early language milestone scale in a high-risk population.
Detailed language evaluations were obtained by interviewing the parents of 191 healthy children aged 0 to 3 years, and by testing the children themselves. From these data, normative values were derived for 41 language milestones in the first 36 months of life. These values were used to construct the Early Language Milestone Scale (ELM Scale), a brief language assessment tool suitable for use by general pediatricians. Physician use of the ELM Scale in a population of 119 children considered at high risk for the presence of developmental disability yielded 97% sensitivity and 93% specificity for the ELM Scale as a detector of developmentally delayed children, when compared with more formal developmental measures as applied by a clinical psychologist or speech pathologist. Early language milestones are a sensitive indicator of developmental integrity; delayed achievement of early language milestones strongly suggests the presence of a significant underlying developmental disability. The ELM Scale may be adopted as a valid measure of developmental status among children considred at high risk for the presence of developmental disabilities.
Cerebral Palsy↗
Sibling reaction to cot death.
Forty-nine children (from 23 families) were assessed during the nine months after the death of a sibling from cot death (SIDS). The death had a major impact on the surviving siblings of all ages, and commonly resulted in difficulties in coping. Feelings of guilt, anger, anxiety and sadness frequently led to behavioural and physical symptoms; but some children without major symptoms were also felt to have significant psychological and emotional difficulties. Explanations given to many of the children were confusing and inadequate, and often aggravated these feelings. Competent counselling should be offered to all families, including those without surviving children, to assist the family in making a satisfactory adjustment. Mourning should be accepted as a healthy part of living, and is important for further psychological growth in the family.
Age Factors↗
Normalization of low-density-lipoprotein levels in heterozygous familial hypercholesterolemia with a combined drug regimen.
We studied the effect of th bite acid sequestrant colestipol, alone and in combination with clofibrate or niacin, in patients with heterozygous familial hypercholesterolemia who were given a diet low in cholesterol and saturated fat. With colestipol alone, mean cholesterol levels in serum decreased 16 to 25 per cent. The addition of clofibrate produced a total mean decrement of only 28 per cent. In contrast, serum cholesterol levels fell 45 per cent when colestipol as combined with niacin. Low-density-lipoprotein (LDL) cholesterol decreased 55 per cent with colestipol and niacin, whereas high-density-lipoprotein (HDL) cholesterol increased. Mean LDL cholesterol was lower in patients given this regimen than in matched normal controls eating an unrestricted diet. Tendinous xanthomas, measured by quantitative xeroradiography, were significantly reduced (P < 0.01), indicating that this regimen mobilized cholesterol from tissue pools with slow turnover. Colestipol plus niacin promises to be useful in the treatment of patients at high risk from elevated levels of LDL.
Adult↗
Analysis of the 1980 heat wave in Memphis.
During the heat wave of 1980, average daily temperatures in Memphis first rose above the mean on June 25 and remained elevated for 26 consecutive days. In July, 1980, 83 heat-related deaths were recorded as compared to non in July 1979. Most of these deaths occurred in elderly, poor, black, inner-city residents. There was a statistically significant increase in total mortality rates, death from natural causes, cardiovascular mortality rates, and the rate for persons dead on arrival. Virtually all the excess mortality was in persons over the age of 60. The rise in heat-related emergency room visits occurred three days prior to the rise in heat-related deaths. Local planning for future heat waves should focus on the inner-city black elderly. Heat-related deaths and emergency room visits should be reported to public health officials.
Adolescent↗