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At least 397 records · Page 22Linked to original sources
PubMed1996

Vincristine treatment triggering the expression of asymptomatic Charcot-Marie-Tooth disease.

A 16-year-old male suffering from Ewing's sarcoma of the pelvis was treated with vincristine as part of his chemotherapeutic protocol. The boy was never known to suffer from any neurological problems. His father had a mild limp, attributed to prolonged "taxi driving," that was never investigated medically. The first course of treatment, which included 2 mg of vincristine, resulted in clinical improvement. However, at the same time the patient developed severe weakness of both upper and lower limbs, areflexia, and gradually a pes cavus deformity. Nerve conduction studies were suggestive of severe peripheral sensorimotor neuropathy, axonal and demyelinative. A definite diagnosis of Charcot-Marie-Tooth was confirmed by molecular analysis showing the typical duplication of 1.5 megabases at 17 p11.2. This unique manifestation of vincristine neurotoxicity is reported and discussed.

Adolescent↗
PubMed1979

Internal hemipelvectomy--excision of the hemipelvis with limb preservation. An alternative to hemipelvectomy.

Our results indicate that it is technically possible to resect the hemipelvis and maintain a viable extremity. Functional results are remarkably good and tumor control has been equivalent to standard hemipelvectomy. Excision of the ilium with preservation of the extremity in selected cases would appear to be a reasonable alternative to the standard hemipelvectomy, which involves amputation of the extremity.

Adolescent↗
PubMed2005

A new and a reclassified ICF patient without mutations in DNMT3B and its interacting proteins SUMO-1 and UBC9.

The ICF syndrome (immunodeficiency, centromeric instability, facial anomalies) (OMIM#242860) is a rare autosomal, recessively inherited disorder. Another rare condition, ischiadic hypoplasia, renal dysgenesis, immunodeficiency, and polydactyly (IHRDIP, OMIM#243340), displays features that resemble those of the ICF syndrome. Due to the overlapping symptoms in both syndromes, we asked whether a shared underlying molecular defect exists. Two patients, each with the clinical characteristics of one of these syndromes, were subjected to conventional cytogenetic analysis and the determination of the methylation state of satellite II DNA. We found that both displayed the two hallmark features of the ICF syndrome, namely hypomethylation and centromeric instability of chromosomes 1 and 16. Therefore, we reclassified the patient previously diagnosed with the IHRDIP syndrome as an ICF patient. Since the majority of ICF patients are carriers of mutations in the methytransferase gene DNMT3B, we determined the sequence of its coding, splice site, and putative promoter region and analyzed its transcripts in both patients, without detecting any alterations. Similarly, the coding region of two DNMT3B-interacting proteins, SUMO-1 and UBC9, did not reveal mutations. With this study, the published number of patients that lack mutations in DNMT3B coding region increases to almost 40% of all ICF patients reported. It is, therefore, implied that a significant subset of ICF patients will have a yet unknown, alternative alteration, which may include the involvement of DNMT3B-interacting factors or aberrations of an independent pathway.

Abnormalities, Multiple↗
PubMed1975

Age changes in the pubic symphysis of Macaca mulatta.

Age changes in the pubic symphyses of 142 Cayo Santiago rhesus monkeys (known age, sex and maternal genealogy) are described. Symphyseal development is sexually dimorphic. Males generate a ridge and furrow system which is gradually replaced by a solid ankylosis at the mid-sagittal plane. Female development parallels that of the male until puberty. With pregnancy and delivery, relaxation and reaggregation of pelvic ligaments binding the symphysis destroy the symphyseal face. Continued bearing of offspring inhibits mid-sagittal ankylosis. Limited estimates of skeletal age can be made by using symphyseal development as an indicator of senescence.

Age Determination by Skeleton↗
PubMed1975

A new pelvic fragment from Swartkrans and the relationship between the robust and gracile australopithecines.

A recently discovered hominid pelvic fragment from Swartkrans (SK 3155) is described in detail with particular reference to the relationship of the two presently recognized forms of australopithecines in South Africa. Results of this examination and metrical analysis indicate that the acetabulum and iliac blade of the early hominids are similar to Homo sapiens except for a unique pattern of traits: a relatively small sacral articular surface, a relatively small acetabulum, a relatively large iliac fossa, and wide lateral splaying of the iliac blades. The new Swartkrans fossil expresses these traits more strongly than does the gracile australopithecine (Sts 14) and is therefore somewhat less similar to Homo sapiens but it is very unlike any pongid.

Acetabulum↗
PubMed1977

Aging and secular change in adult stature in rural Colombia.

Age-associated changes in adult stature of a cross-sectional sample of 634 adults from rural Colombia were estimated by multiple regression of stature and age controlling for subischial length, and from equations predicting maximum attained stature from subischial length. From these analyses the estimated rate of loss in stature due to aging per se for men was 0.12 cm/yr, while for women it was 0.03 cm/yr. When corrected for aging, several secular trends in adult stature were evident, although none were statistically significant.

Adult↗
PubMed1984

A hominine hip bone, KNM-ER 3228, from East Lake Turkana, Kenya.

A male hominine partial hip bone, KNM -ER 3228, from East Lake Turkana , Kenya is described. In most of its features this specimen resembles modern human male hip bones. This is especially true for functional features related to weight transfer from the trunk to the pelvis and within the pelvis, and to the effective action of musculature arising from the pelvis during the performance of the modern human type of bipedalism . KNM -ER 3228 is very similar to the Olduvai Hominid 28 and the Arago XLIV hip bones, both attributed to Homo erectus .

Acetabulum↗
PubMed1988

Evolution of the ischial spine and of the pelvic floor in the Hominoidea.

Study of the pelvis in 143 different mammals reveals that in quadrupeds the ischial spines are barely noticeable and are located posteriorly near the sacrum. In humans, the ischial spines are prominent and more anteriorly located. As a consequence of their position and size, the ischial spines in humans become an obstacle to parturition. Herein a theory is proposed to account for what appears to be an incongruous development and orientation of the ischial spines in humans. The pelvic diaphragm is a vertical pelvic "wall" in tailed mammals and is composed of muscles involved mostly with the motion of the tail. In humans, the muscles of the pelvic diaphragm have a very different anatomical orientation. They form a horizontal pelvic "floor," and their functions are first to support the abdominopelvic organs and resist intra-abdominal pressure that is exerted from above, and second, as levator ani, to control the anal sphincter. In humans the muscles and fascias of the pelvic diaphragm are inserted on the ischial spines either directly or indirectly through the sacrospinous ligament and the tendinous arch of the pelvic fascia. The result is a medial pull on the ischial spines to produce a more rigid and narrower pelvic floor. An inconstant ossification center for the ischial spines make them more prominent. The backward tilt of the sacrum placed the bispinal line in a diameter position. Pongids and even fossil hominids occupy an intermediate position between tailed mammals and Homo sapiens. The present form of the pelvis in Homo sapiens may be determined by a significant genetic component but may also be partly acquired during childhood and adolescence.

Adolescent↗
PubMed1995

Hominid fossil sample from Kanjera, Kenya: description, provenance, and implications of new and earlier discoveries.

Anatomically modern hominids were first collected from Kanjera, Kenya, by L.S.B. Leakey in the 1930s. Their apparent association with an archaic fauna was quickly challenged, throwing their age into doubt. Further unpublished hominid fragments were collected in 1974, 1975, 1981, and 1987. We review the context and morphology of the entire hominid sample. A minimum number of five individuals is represented by both cranial and postcranial elements. Several individuals have thickened cranial vaults, a characteristic originally thought to reflect their great antiquity. Vault thickening resulted from diploic expansion and may have been a response to acquired or inherited anemia. The entire hominid sample postdates the Kanjera Formation, deposited from the early into the middle Pleistocene. Most of the sample was derived from the black cotton soil capping the stratigraphic column. The morphology and context of the Kanjera hominids is consistent with human skeletal remains from nearby Holocene sites. Hominid 3 was probably an intrusive burial into an early Pleistocene bed.

Animals↗
PubMed1993

Morphometric changes in growth of the rat pelvis after papain administration.

The proteolytic enzyme, papain, was given systematically to evaluate the short- and long-term effects of inhibition of endochondral bone formation on pelvic growth, with emphasis on the innominate bone. Ninety-eight Lewis-strain male rats, used concurrently for craniofacial growth studies, were divided into two groups. Thirty rats from Group I (n = 48) received 2% crude papain i.p. daily from 25-40 days-of-age and were euthanized at 40, 54, and 70 days-of-age. Thirty-five rats from Group II (n = 50) were given papain at the same dose from 25-70 days-of-age and were euthanized at 26, 40, 54, 70, and 120 days. The remaining animals in both groups were the controls. Standardized dorsoventral pelvic radiographs were taken of all 98 animals. Ten linear dimensions were measured on each and the data evaluated statistically. A reduction both in size and rate of growth of the bony pelvis was found. All the anteroposterior and most of the transverse pelvic dimensions were significantly shorter, to a greater extent in the prolonged papain group. Bi-ischial width was increased, perhaps to compensate for pelvic shortening and to accommodate the pelvic contents. The findings may contribute to our better understanding of abnormal endochondral bone growth in the pelvis.

Acetabulum↗