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Two cases of Kaposi's sarcoma (KS) are described in patients of pure Eskimo trait from the northernmost part of the world. The clinical and histological findings corresponded to KS in other parts of the world. This report on KS in Eskimos from the sparsely populated Arctic areas may support the opinion that KS is more common outside Africa than the literature indicates.
OBJECTIVE: To assess the role of HLA-B27 and other class I histocompatibility antigens in overall risk and clinical manifestations of spondyloarthropathy (SpA) in Alaskan Eskimos. METHODS: Class I antigens were studied in 104 patients with SpA and in 111 controls. The frequencies of HLA-A, B, and Cw antigens were determined in patients with SpA with various clinical manifestations and compared to frequencies observed in controls. RESULTS: Only HLA-B27 differed significantly in cases and controls. Except for B27, no association of particular antigens with specific syndromes or disease features was found. Patients with B27 had more extraarticular manifestations than patients who lacked B27 antigen. Patients putatively homozygous for B27 did not appear to have more severe disease than those who were heterozygotic. B27 was most closely associated with ankylosing spondylitis [odds ratio (OR) = 210], less so with reactive arthritis (OR = 12.9) and undifferentiated SpA (OR = 4.6). CONCLUSION: Observations in other population groups that implicated B27 cross reactive group (CREG) and other A, B, and Cw antigens as risk factors for developing SpA were not confirmed in Alaskan Eskimos. Nor were CREG or other B antigens either alone or in combination with B27 associated with specific clinical syndromes. Only HLA-B27 was strongly associated with disease and with extraarticular manifestations.
Whole-body counts of Alaskan Eskimos during the summer of 1962 showed the presence of cesium-134 as well as cesium-137. Cesium-134 was also found in reindeer and caribou meat; this finding was confirmed through coincidence counting. There was generally about 1 percent as much cesium-134 as there was cesium-137. Cesium-134 was also found on air filters collected at Richland, Washington. The appearance of cesium-134 seems to be world-wide and continuing.
An explosive epidemic of tuberculosis in a small isolated settlement is described. This outbreak, in a population of 329 Eskimos and 16 whites, produced 80 cases of active tuberculosis, involving 55% of all the house-holds. Forty-five of these patients were children under 10 years of age and only five were persons over 30. These patients probably were infected by one highly infectious individual.The contributing causes of poor housing, overcrowding and poor nutrition, coupled with the debilitating effect of recent epidemics of virus diseases, measles, mumps and German measles, are discussed.Since this article was written, a third resurvey in the same year, conducted in November 1963, revealed 11 more cases among the 279 members of the population who underwent radiological examination; all are hospitalized.
The records of thirty-one patients with nasopharyngeal carcinoma (NPC) diagnosed from 1966 through 1976 among the Alaskan native population (Eskimo, Aleut, Indian) were reviewed. There were 25 males and six females, which results in relatively high incidence rates per 100,000 of 13.5 for males and 3.7 for females. Clinical and pathologic features were similar to those found among southern Chinese NPC patients. Five-year survival rate was 48%. Antibodies to Epstein-Barr virus were higher in NPC patients than in patients with other tumors or matched controls. On histocompatibility testing Sin-2 was not detected, nor was there significantly increased frequency of A2. Instead, BW40 and a second locus blank occurred more often among NPC patients than among other groups. In response to a questionnaire, NPC patients more often reported use of salt fish in the childhood diet, smoking of cigarettes, and exposure to noxious inhalants than did controls, but the differences were not statistically significant.
Ultrastructural investigation of 19 malignant lymphoepithelial salivary gland tumors in Greenland Eskimos disclosed evidence of epithelial origin of the tumor cells in 18 cases. Furthermore, tumor cells exhibited markers of squamous cell differentiation in 16 cases. Signs of adenomatous differentiation were never encountered. Thus, the malignant lymphoepithelial tumor of salivary glands is the ultrastructural counterpart to the nasopharyngeal anaplastic carcinoma (malignant lymphoepithelioma), both being high-grade squamous cell carcinomas.
To identify the times of emergence of the permanent teeth of Canadian Eskimos (Inuit), 368 children and adolescents were examined. The presence or absence of all permanent teeth except the third molars was recorded and these data subjected to probit analysis. Female emergence times were advanced over males. Generally, the Inuit of both sexes showed statistically significant earlier emergence times than Montreal children, except for the incisors. The present results do not support hypotheses indicating that premature extraction of the deciduous teeth advances the emergence of their succedaneous counterparts. There is some indication the controls of deciduous tooth emergence continue to play some part in emergence of the permanent dentition, especially the first permanent teeth that emerge.
Data on the permanent dentition of 63 coastal and 33 inland Alaskan Eskimos are presented. The number of cusps and groove pattern of the mandibular molars were recorded. Agenesis of the mandibular third molars was classified and the mesiodistal and buccolingual crown diameter was measured on the first and the second mandibular molars. The predominant pattern of the lower first molars was Y5, while for the second molar the dominating patterns were +5 and +4. In the lower third molar, +5 was found in the majority of cases. For M1 and M2, men showed a stronger tendency toward a conservative pattern than did women. In the case of M2, the inland population exhibited a more conservative trait than did the coastal population. No connection was seen between the groove pattern and agenesis of M3, however, a reduction in the mesiodistal crown diameter for the second molars was seen when the number of cusps is reduced from 5 to 4.
The bone mineral content (BMC), bone width, and cross-sectional moment of inertia (CSMI) of 141 Alaskan Eskimo tibias were measured using photon absorptiometry. The effects of age and sex on the bones' structural properties were studied. It was found that in women, BMC decreased by 50% between the third and sixth decades, but that of the males did not decline significantly with age. This was true of the CSMI as well, for bending in both the anteroposterior (AP) and mediolateral (ML) planes. This result is different than that in some other prehistoric native American populations, where tibia CSMI increases with age in both sexes. The CSMI values were significantly higher in men than in women. Also, in men the AP CSMI was 55% larger than the ML CSMI; in women this difference was only 25%, and declined with age. Since the tibia is preferentially loaded in the AP plane by locomotor activities, the platycnemic differences between the sexes may reflect sex-related differences in activity which become more pronounced with age. The non-destructive method for obtaining data on the cross-sectional geometry of dry bones which is described here may be useful in studying other archaeological collections.
On the basis of earlier findings with Easter Islanders suggesting a positive correlation between dermatoglyphic variables and hand anthropometric measurements, the present study was designed to determine if such a relationship could be generalized to another population, namely, Iñupiat (Eskimo). Since some dermatoglyphic and anthropometric variables were available for both sides of the body, the extension of this study to explore the question of asymmetry was also possible. The Iñupiat sample numbered 142 male and 176 female adult inhabitants of five Alaskan North Slope communities. The major findings of this study included, for males, significant negative correlations between left arm length and digital ridge counts and positive relationships between the palmar variable of axial index and hand length on both hands. For females, the hand breadth/length index was negatively related to most of the digital variables. Very little definitive information regarding the relationship of the asymmetry variables between the two types of measures was ascertained.
We analyzed the demographic, clinical, laboratory and histologic features of 13 patients who were diagnosed as having polyarteritis nodosa associated with hepatitis B virus infection over a 12-year period, 1974 to 1985. All 13 patients were Yupik Eskimos and resided in southwest Alaska, an area hyperendemic for hepatitis B virus infection. The annual incidence of hepatitis B virus-associated polyarteritis nodosa for this population is 7.7 cases per 100,000 population. All patients presented with multisystem disease, and all had biopsy or angiographic findings consistent with polyarteritis nodosa. All 13 were positive for hepatitis B surface antigen and hepatitis B e antigen at diagnosis. Two untreated patients and two of five patients who received corticosteroids died, vs. none of six who received corticosteroids plus cyclophosphamide. None of the patients who survived the initial bout of polyarteritis nodosa has relapsed after a mean follow-up of 55 months, but all have become chronic HBsAg carriers. In eight patients, clinical or serologic evidence indicated that polyarteritis nodosa followed recent hepatitis B virus infection. We concluded that hepatitis B virus-associated polyarteritis nodosa is a serious, life-threatening complication that occurs early in the course of hepatitis B virus infection, is ameliorated by immunosuppressive therapy and can be prevented by hepatitis B vaccine.
The epidemiology of Hepatitis A virus (HAV) and hepatitis B virus (HBV) infection was studied in a northern Canadian Inuit (Eskimo) settlement. Sera from 720 of the 850 inhabitants of Baker Lake, Canada, were tested for markers of HAV and HBV infection. Anti-HAV was present in 71% of the residents and its prevalence increased with age. Serologic evidence of HBV infections was found in 27% of residents. The prevalence increased with age, being uncommon under the age of 20 (6%) and almost universal over the age of 60 (93%). Among the 29 hepatitis B surface antigen (HBsAg) carriers identified, all were adults, all had low levels of HBsAg, and all were negative for hepatitis B e antigen (HBeAg) and DNA polymerase but positive for antibody to HBeAg. These data demonstrate a high prevalence of HAV and HBV infection in this population. Further, they suggest that a dramatic decrease in the transmission of HBV infection has occurred over the past 20-30 years.
An epidemiological study of the occurrence of angle-closure glaucoma was made under the Eskimos population of the Norton Sound and Bering Straits region of Alaska. Among 1673 Alaskan Natives examined 14 cases (0.8%) of angle-closure glaucoma were found, in 0.5% of the men and 1.2% of the women; for persons above the age of 40 years these figures were 2.1% and 5.5% respectively. Angle-closure glaucoma was found in 11.8% of women above the age of 60 years. A shallow anterior chamber was seen twice as frequently in women as in men. Above the age of 30 years the chamber angle was considered to be occludable in 2% of men and 7.5% of women on gonioscopic examination. The average intra-ocular pressure of the right eyes of men was 11.7 mm Hg (s.d. 3.3) and of women 12.0 mm Hg (s.d. 3.4). Primary open-angle glaucoma was not seen in the population being studied.
Metachromatic leukodystrophy (MLD) is an autosomal recessive disorder resulting from the inability to metabolize sulphatide, an important component of myelin. Although there is significant clinical variability between patients, most have the late-infantile form. It is one of the most common lysosomal disorders involving mental deterioration and is found throughout the world. The great majority of the cases have a deficiency of arylsulphatase A activity. Accurate diagnosis of MLD is complicated by the presence of so-called pseudodeficiency alleles and the need to receive specimens for biochemical testing within 24-48 h of collection. We report the identification of the mutation (a g-to-a transition in the first nucleotide of intron 4) in the arylsulphatase A gene causing late-infantile MLD among the Eskimo population of southern Alaska. As all patients and family members from living and deceased patients had the same mutation, a mutation-based test was developed to identify patients and carriers that can be done on dried blood spots sent via regular mail service. A possible genetic link between this population and the Navajo Indians of the southwestern United States is proposed.
An A-to-G transition in the second intron was the sole mutation detected in four Yupik Eskimo patients with salt-wasting congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency. Allele-specific hybridization should be an efficient means of performing prenatal diagnosis of the disease in this highly inbred population.
An infrared and x-ray diffraction study of osteoporotic and normal, archaeological Eskimo bones. Osteoporotic bone apatite is greater in crystal size and/or perfection and lower in CO3 than normal bone apatite.
Studies in man and laboratory animals suggest that omega 3 polyunsaturated fatty acid constituents of fish oils have antiatherosclerotic properties. We have studied the effects of several such polyunsaturated fatty acids for ability to modify the in vitro release of mitogens from human platelets. Such mitogens may produce the fibro-proliferative component of atherosclerotic plaques. Both 5,8,11,14,17-eicosapentaenoic acid (20:5 omega 3) and 4,7,10,13,16,19-docosahexaenoic acid (22:6 omega 3), major constituents of fish oils, inhibited adenosine diphosphate-induced aggregation of platelets and the accompanying release of mitogens. These effects are dose dependent. Linolenic acid (18:3 omega 3), the biosynthetic precursor of eicosapentaenoic acid, also inhibited platelet aggregation and mitogen release. Eicosapentaenoic acid also inhibited mitogen release from human monocyte-derived macrophages, which, in vivo, are an additional source of mitogens during atherogenesis. Potent inhibition of human platelet aggregation and mitogen release was also seen with dihomo-gamma-linolenic acid (8,11,14-eicosatrienoic acid 20:3 omega 6), whose levels are reportedly elevated in Eskimos subsisting on marine diets. We conclude that diets that elevate plasma and/or tissue levels of eicosapentaenoic acid, docosahexaenoic acid and dihomo-gamma-linolenic acid precursor gamma-linolenic acid (18:3 omega 6) may exert antiatherosclerotic effects by inhibiting the release of mitogens from platelets and other cells.
The total urinary excretion of tetranor prostaglandin metabolites, measured as tetranorprostanedioic acid (TPD), was quantified in traditionally living Greenland Eskimos (E) and compared with that in Caucasian Danes (D). TPD excretion (microgram/24h) was not significantly different between both groups, neither for males (331 +/- 62.4 (E) vs. 331 +/- 25.7 (D), mean +/- SEM, n = 9 and 10) nor for females (190 +/- 31.7 (E) vs. 264 +/- 27.4 (D), n = 11 and 10, P2 greater than 0.05). Since urinary prostaglandin metabolites are thought to reflect the total prostaglandin turnover in vivo, these results suggest that a long-term intake of relatively large amounts of polyunsaturated fatty acids of the (n-3) family does not alter total prostaglandin turnover in vivo. This is in contrast to stimulated prostanoid formation in vitro, and thus suggests a different regulatory role of dietary and tissue fatty acids for 'stimulated' and 'basal' prostaglandin production.