[Diabetic neurotrophic osteoarthropathy].
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Biomedical subjects
Publications and source records attributed to K Fried.
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Neonatal rats were given capsaicin subcutaneously. Controls received vehicle only. Six months later the nerve fiber populations in the dorsal root L4, in the inferior alveolar nerve and in mandibular molar pulps were examined by electron microscopy. The proportion of unmyelinated axons was found to be markedly decreased in root and nerve specimens from capsaicin-treated rats. However, the pulpal nerves in tooth specimens from these rats showed no obvious alterations.
An infant suffering from failure to thrive, hepatosplenomegaly, developmental retardation and early infantile death is described. The proposita demonstrated a type 2 early infantile sialidosis with onset at birth, and death at 4 months. A culture of the proband's fibroblasts showed neuraminidase deficiency, and low activity of the enzyme was found in the lymphocytes of both parents. A previous female child, born prematurely, died 6 h after birth and had hepatosplenomegaly and foam cells in the placenta. There is strong evidence that the inheritance of the disease is autosomal recessive.
A large family with three children affected with the autosomal recessive disease of Cerebrotendinous Xanthomatosis (CTX) was studied for class I (HLA-A,B,C) and class II antigens (HLA-DR,D,SB), properdin factor B and glyoxalase. The extensive typing revealed an informative cross-over between HLA-B and Bf, indicating that Bf is located centromeric to the HLA-B locus and segregated in this family with HLA-D/DR. The parents in this family were first cousins and their parents were also first cousins. Three of their four haplotypes share B14, BfS, DR1, Dx and SB4 and may be identical by descent. The three affected children carried among them all four parental haplotypes, indicating that close linkage of the CTX locus to HLA is unlikely.
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Regeneration of the inferior alveolar nerve and mandibular incisor pulpal reinnervation was qualitatively and quantitatively examined by electron microscopy 2 days--11 months after intramandibular neurotomy in young adult cats. Fifteen millimeters central to the proximal stump moderate atrophic alterations of myelinated axons were observed 1--2 months after surgery. By 4--11 months a principally normal picture had been restored. The proportion of unmyelinated axons was increased 2--4 months after operation but had normalized by 11 months. In the distal stump the first regenerating axons were observed at 2 weeks. The regenerated myelinated axons failed to re-establish the previous fibre size range and normal axo-glial relations did not appear. A seemingly stable morphological pattern was reached 4--11 months postoperatively. In the late survival period the proportion of unmyelinated axons was subnormal. In the incisor pulps virtually all axons disappeared after surgery. By two weeks pulpal reinnervation had begun. From two months on, a structurally largely normal pulpal axon population was present except for some persisting unmyelinated axon degeneration. The findings are consistent with previous physiological data and suggest that structural normalization at proximal and preterminal levels follows upon re-establishment of peripheral contacts.
The number and size distribution of axons in the developing feline inferior alveolar nerve (IAN) were examined by electron microscopy. Seven cat fetuses and thirty kittens and cats aged from 25 days post conception (dpc) to 11 years were used. The total number of IAN axons increased from 4,400 to 16-17,000 between 25 and 40 dpc, and then decreased to about 13,000 before birth. This level was maintained up to at least 11 years. Myelinated axons first appeared by 45 dpc and constituted 28% at birth. The young adult proportion of about 45% myelinated axons was established at 2 months. In the old adult (11 years) 55% of the IAN axons were myelinated. Size measurements showed that unmyelinated axons had diameters of 0.1-0.5 micrometer at 25 dpc. From 55 dpc and on the size range extended from 0.1 micrometer to 1 micrometer. The size range of myelinated axons was 1-4.5 micrometers at birth and 1-8 micrometers 2 months postnatally. A bimodal size distribution first appeared by 2 months, and the range was 1-13 micrometers from 6 months and on. The findings were compared with age-related changes in the primary and permanent dentitions.
The relation between the number of myelin lamellae (nl) and axon size (d) was examined in the developing and adult feline inferior alveolar nerve (IAN). The internodal lengths (L) and total diameters (D) were measured on teased IAN specimens from kittens and cats. The results show that relations nl/d and L/D principally similar to those in young adult cats had been established 2 months after birth. This coincides with the maturation of the primary dentition. During the first 3 postnatal weeks signs of a developmental demyelination were common. Comparisons between the internodal elongation of early myelinating axons and the longitudinal growth of the IAN in the mandibular canal indicated that some 50% of all prospective large internodes must be removed. Between 2 months and the young adult stage, when the permanent dentition is established, the relations nl/d and L/D were essentially unaltered, but the ranges extended towards larger sizes. In the young adult the average g-value was 0.67. In the old adult cat the relation nl/d was less uniform than in young adults, and the average g-value had decreased to 0.59. In addition, successive short (100-150 micrometer) internodes were found, indicating a senescent de- and remyelination in the IAN. These alterations may be related to the age-dependent deterioration of the mandibular dentition.
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The qualitative structural development of the inferior alveolar nerve was studied by electron microscopy in 56 pre- and postnatal kittens and 21 young and old adult cats. At 25 days post conception the nerve was composed of a bundle of small axons enclosed by primitive sheath cells. Three weeks later myelination had been initiated. Axons measuring 2-3 micrometers underwent local demyelination from 2 weeks before to 3 weeks after birth. This was accompanied and followed by nodalization of larger axons. A typical perineurium was first apparent in the newborn kitten. Six to eight weeks postnatally, the nerve appeared qualitatively mature, although axonal growth was far from completed. This coincides with achievement of a fully mature primary dentition shortly after the weanling period. Apart from a continued size growth, no changes were observed in the nerve during the transition from the primary to the permanent dentition. In the inferior alveolar nerve of old cats, axonal and perineurial changes co-existed with signs of dental attrition and pathology.
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Juvenile aberrations of the vertebral bodies and intervertebral discs are abnormalities of the chondral, periosteal and apophyseal ossification. Differentiation of clinically insignificant variations and potentially or manifestly pathogenic dysspondylies is suggested. The occurrence of abnormalities of bodies and discs is different in particular parts of the spine. The semiology of eight dysspondylies in individual segments of the spine is described. M. Scheuermann presents the most frequent dysspondyly.
The ingrowth, maturation, and aging of pulpal axons was followed by electron microscopy in permanent mandibular incisors of 44 cats aged 35 days-11 years. The first axons entered the incisor pulps about 2 months after birth. Cells resembling Schwann cells, but lacking an axonal relation, were also observed at this time. Axonal ingrowth and maturation continued until 7 months, when the pulps contained 92-394 axons, 81-88% of which were unmyelinated. Between month 7 and 20 months the character of the incisor pulpal axons remained largely unchanged. In adult cats the myelinated pulpal axons had diameters of 1-6 micrometer. The relation between the number of myelin lamellae and the axon size correlated better with linear + logarithmic than linear functions. The g-ratios were higher than in most peripheral nerves, and tended to increase with axon diameter. In the old adult this tendency was more evident and the g-ratios were generally lower. From 3 years on the incisors displayed various age-related or pathological alterations. These were accompanied by axonal changes and axon loss. In contrast to earlier stages perineuriumlike sheaths were frequent in old teeth. The findings are discussed in relation to axon development in the inferior alveolar nerve and in primary incisor pulps.
The life history of pulpal axons in primary mandibular incisors was examined by light and electron microscopy in 56 kittens aged from 25 days postconception to 120 days after birth. Cells resembling Schwann cells preceded the first arrival of pulpal axons, 1 week postnatally. myelination was initiated during the second week. Two months after birth the incisors were fully grown and each pulp contained about 100 axons. Between 10 and 20% of these were myelinated and ranged in size from 1 to 5 micrometer. The relation between number of myelin lamellae and axon size appeared nonlinear and differed markedly from that in similarly sized inferior alveolar nerve axons. During the third month many unmyelinated axons showed signs of degeneration. With progress of root resorption an increasing proportion of both unmyelinated and myelinated axons degenerated. In highly resorbed incisors necrotic Schwann cells were associated with degenerating axons and there was a generalized pulpal tissue reaction. In some teeth with advanced root resorption pulpal axons were lacking. A progressive derangement of all pulpal tissue elements continued until shedding during the second half of the fourth month. In incisor nerve branches below the incisor teeth axon degeneration was very limited.
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The proposita was operated on in this hospital in 1980 for pyloric stenosis, at the age of 13 days, after vomiting had started 5 days previously, and the diagnosis had been confirmed on radiological investigation. Her older sister and two male cousins of the father had the same operations in Israel in infancy. The other nine affected individuals in the family were known to have had projectile vomiting for several months in infancy, and two of them died in infancy. They were all born in the Jewish community in Georgia, U.S.S.R. The male:female sex ratio was 2:2 for the operated cases, and 4:5 for those projectile vomiting history. There was no skipping of a generation. This family indicates that pyloric stenosis can exceptionally be inherited as a simple autosomal dominant trait.