Dr. T. Tóth szerk.: Studia historico-anthropologica (Anthropologia Hungarica 21. Budapest, 1990)

5. The spatial location of cranial lesions Congenital haemolytic anaemias (Cooley-anaemia, sickle-cell anaemia etc.) stay remain the diseased persons for all their lives. Therefore, increased red blood cell production and hyperplastic marrow remain constant characteristics during the life span. PH may appear not exclusively on the parietal and frontal bones but on the maxilla, on the zygomatic bone and on the bony palate, etc. The face may take Mongoloid features with protruding teeth and protuberant os zygomaticum. The development and pneumatization of paranasal cavities fail to occur or are retarded. These cavities are often mere apertures even on the skulls of 12-15 years old individuals. Iron deficiency or nutritional anaemias usually bring about PH formation restricted to the bones of the neurocraniurrk The pneumatization of paranasal cavities is undisturbed (MOSELEY 1965). PH develops most often on the upper part of the orbital cavities, on parietal, on frontal and on temporal bones in a decreasing order of frequency. The area of lesion affected skull surfaces can reach 6-8 cm

2 per centre in congenital haemolithic anaemias but it remains smaller in iron deficiency and nutritional anaemias (LIE-INJO 1958). Macrocephaly develops at the age of 6-12 months with congenital hyperphosphatasy. An extremely large trabecular type spherostosis is formed on the skull from the forehead to the nape (EYRING & EISENBERG (1968). Bi-concave vertebrae, severe osteoporosis of tubular bones and thinned outer cortical layer are also characteristic. The duration and severity of the primary disease seem to be correlated not only with the size of the lesion but with the type of it as well. The trabecular type is dominant or almost exclusive in congenital haemolytic anaemias. Iron deficiency anaemias are accompanied with less severe and smaller size lesions, tf the presence of Wilson-bands and/or enamel hypoplasia of incisors or canines can be verified by microscopic examinations it points towards nutritional or iron deficiency anaemia (ROSE & al. 1985). Frontoparietal cranial deformation is usually incidental to congenital haemolytic anaemias as well as to PH caused by cyanotic vitium. Whereas the deformity of the occipital region is more characteristic for iron deficiency anaemias (SZÁLA11986). 6. Alterations of tubular bones There are no specific lesions on tubular bones in congenital, haemolytic anaemias. The expansion of the medullary cavity of diaphysis, the thinning of the cortical layer and the resorption or absence of medullary cavity trabeculae is clearly visible in X-ray pictures and/or on the sawn up bones. Vertebral corpus compression may occur in sickle-cell anaemia. In hypovitaminosis conditions (scurvy, rickets) macroscopically and radiographically characteristic alterations can be found in the growth zone of bones. Dietary disorders (and less frequently other reasons such as febrile diseases or endocrine disturbances) may temporarily arrest the growth of long bones and Harris lines are formed. The Harris lines are well defined, 0.5-2 mm wide, transverse lines of increased opacity. These radiographic patterns may occur in single or multiple form on both ends of the femur, tibia, humerus and of the radius. The position of Harris lines relative to the midshaft makes it possible to determine the etiological age when a child was affected by dietary disorder. Harris lines can be detected in recent populations, too. Their frequency is 1-5%. The absence of Harris lines may indicate the absence of any dietary disorder (milk anaemia, iron deficiency anemia). Their presence - especially in the multiplied form - renders dietary and/or deficiency anaemias probable. We observed the combined presence of PH and the radiographically detectable alterations of tubular bones. Half of the PH affected individuals also had Harris lines in their tibiae, femora and less frequently in other tubular bones as well (JÓZSA & PAP 1988). However, no significant association was found between PH and Harris lines by other authors in other materials (MARCSIK 1987). Renal diseases leading to chronic uraemia produce PH in a small percentage. When the lesion is formed as a result of such a disorder the traces of PH are detectable on tubular bones. Renal diseases leading to chronic uraemia always result in secondary hyperparathyreosis that brings about severe osteoporosis in the entire skeleton and may occasionally lead to osteodystrophia fibrosa