Joint Malformation Research Articles

Adjuvant arthritis in the rat.

Adjuvant arthritis (AA) is an induced form of (sub)chronic arthritis. Strains of rats have a varying genetic susceptibility to AA, whereas mice generally are not susceptible. AA is most easily induced with mycobacteria suspended in oil, although in some strains of rats it can be induced with oily adjuvants in the absence of mycobacteria. The disease is a T cell-mediated autoimmune arthritis that is frequently used to study immunological aspects of rheumatoid arthritis (RA) and other arthritic or inflammatory diseases in humans. Furthermore, it is used as a model for developing and testing antiinflammatory drugs. There are no particularly well-defined autoantigens in AA; in this respect, the model resembles spontaneous arthritic diseases in humans. In all susceptible rat strains, the inflammatory process of AA is self remitting, although usually the disease is severe and leads to permanent joint malformations, including ankylosis; a time line for AA development is presented. This unit describes the induction and evaluation of AA and the preparation of adjuvant used to induce AA.

The anatomy of the pelvis in the exstrophy complex.

We compared computerized tomography scans of the pelvis of twenty-four patients who had exstrophy of the bladder with scans of age-matched controls in order to analyze the pelvic deformity that accompanies the variably severe manifestations of this condition. The patients who had classic exstrophy of the bladder were found to have a mean of 12 degrees of external rotation of the posterior aspect of the pelvis on each side, retroversion of the acetabula, a mean additional 18 degrees of external rotation and 30 per cent shortening of the pubic rami, and progressive diastasis of the symphysis pubis. The foot-progression angle demonstrated 20 to 30 degrees of external rotation beyond the normal limits seen in early childhood, but this improved with age. The patients who had exstrophy of the cloaca and the bladder not only had all of these pelvic deformities to a greater degree but also had asymmetry of measured parameters between the right and left sides of the pelvis, malformation of the sacro-iliac joints, and occasional dislocation of the hip. An understanding of the pelvic anatomy that accompanies exstrophy is essential when corrective approaches are planned. Such an understanding will improve the rate of success of both closure of the bladder and control of urinary continence postoperatively.

METACARPOPHALANGEAL JOINT IMPLANT ARTHROPLASTY

Rheumatoid arthritis (RA) usually requires lifelong treatment and sometimes surgery. Metacarpophalangeal joint (MCPJ) implant arthroplasty is one surgical treatment for patients with severe RA malformation of the finger joints. Although not a cure, MCPJ implant arthroplasty can enhance patients' quality of life by improving their performance of independent activities of daily living. The silicone implant acts as a spacer until tendons and connective tissues are able to control the joint's functions. Comprehensive preoperative and postoperative patient teaching and aggressive physical therapy are needed to achieve optimal outcomes in patients who undergo this surgical procedure.

Malformation of a cervical facet joint with inclusion of an arthrolith

The authors present the case of a 49-year-old man with an isolated malformation of the left cervical facet joint at C5-6, with secondary spondylarthrotic hypertrophy of the joint leading to involvement of the C-6 nerve root. The etiology of this cervical joint malformation is discussed.

Dislocation and/or congenital malformation of the shoulder joint. Observations on a Mediaeval skeleton from Denmark

The purpose of this paper is to present a Mediaeval skeleton of an approximately 16 year old boy, which was excavated at a Danish cemetery containing ca. 150 graves. The skeleton reveals several pathologic changes, probably due to congenital malformation. The most intriguing find is seen at both scapulae, and the changes are bilateral symmetric. Both the glenoid cavities are placed posterior but at the normal height of the bone. The joints are almost perpendicular to their normal direction. The size of the glenoid cavities is normal and the shape is rather flat in accordance to the development stage of the skeleton, where the epiphysis of the rim has not yet appeared to form the gently concave fossa as normally seen in adults. Both the surface and the borderlines of the glenoid cavities are, however, more irregular than normally at that age. The position of the joints may be caused by dislocation and/or congenital malformation which is discussed. Due to the shape of the cavities, to the symmetric bilaterality, and to the minor congenital malformations, it is primarily believed to be caused by congenital malformation. Probably the young man was not much affected by the malformation of the shoulder joints, which is indicated by the normal form and size of the humeri and the well-developed muscle attachments of the bones. The claviculae seem shorter and more twisted than normal, which may be caused by a twisting of the scapulae. So the glenoid cavities may have pointed almost in the normal direction in spite of the malformation. Other minor malformations are spina bifida of the atlas and the 5. lumbar vertebra, multiple minor changes of the joints of both feet and malformations of one metatarsal bone in both feet. Agenesi of the praemolars is also seen. Although our study of the literature, we have not succeeded in correlation our finds with any known congenital syndrome, and as far as we know no similar case has been described in clinical observation or in skeletal finds.

Embryonic behavior, hatching and neuromuscular development in the chick following a transient reduction of spontaneous motility and sensory input by neuromuscular blocking agents.

AbstractEmbryos were immobilized with neuromuscular blocking agents for one to four days between days 10 and 15 of incubation. This treatment reduces spontaneous motility, as well as movement‐initiated proprioceptive and cutaneous stimulation. Although the major aim of these experiments was to determine the effects of such treatment on subsequent behavioral development, several indices of neuromuscular and general morphological development were also examined.A single injection of curare on day 10 continues to depress spontaneous motility for virtually the entire remaining incubation period. This effect is due to the persistence of unmetabolized curare in the closed system of the egg. When a comparable dose of a rapidly metabolized neuromuscular blocking agent (succinylcholine) is given, this long term behavioral depression is not found. Embryos treated with SC can remain totally immobilized for up to 60 hours with no apparent repercussions on subsequent behavior; spontaneous embryonic motility, reflex sensitivity, hatching, and several general posthatching behaviors, all appeared normal following such treatment.Embryos immobilized for as little as 48 hours developed joint malformations and were retarded in general growth by about one day whereas 24 hours of paralysis was not sufficient to induce these effects.Twenty‐four hours of total paralysis from days 10 to 11, plus a 40–50% reduction in motility until days 15 or 16, does not appear to cause any abnormalities in muscle or spinal cord development. Muscle histology, motor endplates, cell number in the spinal cord and choline acetyltransferase and acetylcholinesterase activity in spinal cord and muscle were all comparable to controls. An 80–90% reduction in motility on days 11 through 15, however, induces an apparent alteration of the intensity and distribution of histochemically demonstrable AChE in the anterior and posterior latissimus dorsi muscles.The present findings suggest that the suppression of overt motility for a period comprising ca. 5–12% of the total incubation time (21 days) does not modify subsequent behavioral development or the underlying neurogenetic mechanisms. Though these findings provide some support for the notion that the embryonic nervous system develops in forward reference to and without benefit from function or sensory input, only a more complete reduction of neural function, especially CNS activity, can provide a critical test of this concept.

Hereditary malformation of the toes and fingers in a family with double translocation heterozygosity.

A hereditary malformation of the metatarsophalangeal joints, called here "tear drop" deformity, and a cut off appearance of the distal phalanges of the toes is described in a family with double translocation t (7; 12), t (2; 6) heterozygosity. A bracydactyly E was also found. These disorders seemed unrelated to the chromosomal aberration.

Oval window absence, bony closure of round window, and inner ear anomaly.

AbstractUnusual temporal bone findings are presented in a seven‐and‐one‐half‐year‐old female with marked hearing impairment and multiple congenital anomalies. The oval window was absent bilaterally, with no stapedio‐vestibular joint development on the right side and incomplete development on the left side. The round window membranes were normal, but on the left side the round window niche was walled off from the middle ear by bone continuous with the periosteum of the middle ear surface of the otic capsule. Surgically, this would have been interpreted as congenital absence of the round and oval windows. It should be recognized that the round window membrane may be present in the absence of an open round window niche. Bilaterally the cochlea consisted of 13/4 turns, and on the left side, the scalae tympani ended blindly in the second turn so that no helicotrema was present. Spiral ganglion cells were markedly reduced on the right side and entirely absent on the left, where there was marked thickening of the cribriform base of the cochlea. The organ of Corti was present in all turns. The bony semicircular canals were not formed.The embryological development and interference with development which probably resulted in these malformations are discussed.Oval window absence or congenital footplate fixation should probably be referred to as varying degrees of stapediovestibular joint malformation.

Feeding chicks high levels of L-methionine-supplemented diets in the study of the experimental aspects of homocystinuria

1. 1. Feeding high levels (1, 2 and 4 per cent) of l-methionine to White Rock male chicks, from 1 day to 16 weeks produced the following conditions: growth depression, reduction in feed conversion, hock joint malformation, poor motor co-ordination, seizures, rough feathering and discoloration of shank and eyelid. 2. 2. Complete remission of all these deformities was obtained within eight weeks (16 weeks of age) by supplying the chicks previously fed (0–8 weeks) the aforementioned levels of l-methionine, with a basal diet and a basal plus l-cystine diet.

Effect of feeding high levels ofl-phenylalanine to day-old chicks and its relationship to sex and breed

1.Feeding high levels ofL-phenylalanine (2, 4 and 8 per cent) on top of the basal diet given to day-old White Leghorn females and crossbred (Rhode Island Red × Barred Plymouth Rock) male chicks from 1 day of age to 4 weeks produced the following conditions: growth retardation, reduction in feed efficiency, toe and hock joint malformations, poor motor coordination, rough feathering, discoloration of the shank and eyelid and lowered organ weights. These physical alterations were pronounced at the 8 per cent level and minimal at the 2 and 4 per cent levels.2.At all levels of supplement the purebred females were more affected than the crossbred males.