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Multiple cystic tuberculous lesions in the skeleton (Jüngling disease): a case report.

STUDY DESIGN: A case is reported. OBJECTIVES: To report and discuss a case of multiple cystic tuberculous skeletal lesions. SUMMARY OF BACKGROUND DATA: Multiple cystic tuberculosis lesions of the skeleton, or Jüngling disease, is a rare variety of tuberculosis reported extensively 30 to 40 years ago. No such severe type of tuberculosis has been reported in recent years. The patient was a 43-year-old woman with a history of steroid treatment, whose cervical lesions caused C2 and C3 destruction and spinal cord impingement. METHODS: Medical history, laboratory data, radiographs, bone scan, and MRI were studied. The bone scan showed lesions in both the axial and peripheral skeletons. As shown on the radiographs and MRI, the C2-C3 vertebral bodies were extensively destroyed. RESULTS: An open biopsy of the right clavicle confirmed the diagnosis of tuberculosis. Because of the neurologic involvement, the patient was treated with C2-C3 anterior radical resection and fusion combined with standard antituberculosis chemotherapy. During a follow-up period of 12 months, no progression in other skeletal lesions was observed. Follow-up radiographs showed fusion in the C2-C3 lesions. CONCLUSIONS: Although the prognosis of Jüngling disease is good, radical surgical treatment should be chosen when there is neurologic involvement. To prevent such a severe type of tuberculosis, it is recommended that steroid therapy should be withheld until tuberculosis is excluded in the diagnosis.

Adult↗

Age structure and selected pathological aspects of a series of skeletons of late medieval Bernau (Brandenburg, Germany).

The age distribution of an excavation site from the late Middle Ages (beginning of the 13th century until 1598) in Bernau (Brandenburg, Germany) that contained 252 skeletons shows a mortality maximum at the age classes infants I and mature. The lowest mortality was calculated for the adult age class. 51.6% of the individuals died before the age of 20 years. The mortality rate of young women was higher than that of young men. The life expectancy of the total population was 25 years. Altogether, 87 adult skeletons were examined for degenerative joint diseases. Many of the examined joints showed indications of beginning osteoarthritis; the intensity of the disease was low or medium severe. The highest values were found for the hip joint, followed by the elbow and the knee. For all joints examined, women were less often and to a lesser extent affected than men, which was noticeable best in the upper extremity. Harris lines were found in 75% of the children, 4.7 per average individual. No such lines were found by the age of one year, the peak value of the frequency being recorded at the age between 2 and 3 years. This can be correlated to the weaning period. The comparison to a rural population yielded a higher load with Harris lines in the small village, which, however, was not correlated to a higher mortality. Altogether, the population of Bernau is characterised by good living conditions and a considerable chance of survival for the children, as well as by a labour system, dominated by crafts and farming.

Adolescent↗

[Usefulness of semi-skeletonized right gastroepiploic artery].

The right gastroepiploic artery (RGEA) could be harvested easily and safely by using an ultrasonic scalpel. It is easier and faster not to remove a satellite vein from RGEA than to skeletonize it fully. And blood flow of the vein is important, because it is the flow of vasa vasorum of RGEA. Among 70 patients who have bypassed to right coronary arteries (RCA), 25 patients were treated with semi-skeletonized RGEA (SSK-RGEA), the other 45 patients were operated with pedicled RGEA or (if pedicled RGEA was short and small) with the other grafts. An availability of SSK-RGEA for RCA was 100%. And that of pedicled RGEA was 47% (p < 0.001). Twenty-eight patients were operated without a pump. Twenty-three of them (82%) were bypassed with RGEA. In the on-pump cases (42 patients), RGEA were used for 13 (31%) cases (p < 0.05). Early post-operative angiographies revealed 1 occlusion. But the site of occlusion was the origin of RGEA branch from a gastro-duodenal artery, and the anastomotic site was patent. This graft was supposed to be occluded post-operatively by arteriosclerosis. Flow competition occurred in two grafts. In conclusion, the SSK-RGEA was useful for RCA bypass grafting. The reliability of RGEA should increase the indication of off-pump coronary artery bypass grafting.

Cardiopulmonary Bypass↗

[Study of skeleton gravitation physiology and problem of osteoporosis].

Main osteoporosis definitions and some results of bone tissue research in Russian astronauts, patients, and healthy subjects, using modern osteodensitometry, are presented. Bone mineral density (BMD) was regularly decreased at lower segments of skeleton. In the skull bone and some other sites of upper part of skeleton, a tendency was revealed for an increase of the bone mineral content (BMC). The mean value of bone loss was within the normal range and not correlated with duration of space flight; it revealed a high individual variability and in some cases was clinically qualified as local osteopenia. On the ground of analysis of own results and animal and bone cultural experiments data in microgravity conditions, the described changes seem to be reflecting a deceleration of bone formation as an adaptive response of bone tissue to the mechanical unloading. The response is realized mainly on the tissue level. It does not exclude bone resorption activity as a result of changes in hierarchy of water and electrolytes metabolism as reflected by body fluid redistribution in cranial direction. The results obtained broaden our notions on pathogenesis of some types of osteoporosis in clinic.

Absorptiometry, Photon↗

[Detection of the Köbner phenomenon of the skeleton of patients with psoriasis].

Psoriasis is a humorally controlled systemic disease. The degree of "eruptive strength" of manifestation results from hereditary factors (disposition) and environmental factors (provocation). We were able to demonstrate that the well-known Köbner phenomenon of the skin also occurs on the skeleton of patients suffering from psoriasis. We analysed 83 patients in whom bone scans were carried out. Our results indicate that provocation factors such as bacterial foci and/or trauma correlate with a significantly higher number of pathological scintigraphic findings, ranging up to "hot spots". Furthermore, not only did bone fractures remained scintigraphically positive for an unusually long time, traumas of the end phalanx could be demonstrated in 70% of psoriatic patients compared with 21% of a control group. Obviously, one factor alone or a combination of factors triggers the involvement of the skeleton as a "deep Köbner phenomenon". In psoriatic patients the response of bone metabolism to disturbance differs from that of non-psoriatic patients in that there is a long-lasting dysregulation. This explains the high correlation between skin and skeletal manifestation in psoriatics. Therefore the manifestation of psoriatic disease is due not to a single-stranded linear causal interrelation but to a multicausal "network pathogenesis". Bone scintigraphy is the diagnostic method of choice in patients with psoriatic osteoarthropathy and allows an objective evaluation of therapeutic success.

Adult↗

[Criminalistic-identification aspect of image diagnosis of skeleton pathology characteristics].

In this paper the author showed possibilities for adaptation of the most present-day medical achievements in the field of noninvasive diagnosis of individual characteristics of human skeleton pathology--at crime detection and during criminalistic identification particularly. The identification value of image diagnosis for individual characteristics of the pathologically changed skeleton has been exemplified with: X-ray radiography, both conventional and computer-assisted tomography, scintigraphy, ultrasonography and nuclear magnetic resonance tomography. The paper's conclusion is formulating of new definitions for identification method of individuals, based on image diagnosis--named osteopathoscopy--which can be an alternative method to controversial radiology.

Autopsy↗

Reconstruction of root morphology in skeletonized remains with postmortem dental loss.

A reversible technique is presented in which the root morphology of missing teeth in skeletonized human remains can be reconstructed for the purpose of radiographic comparison and postmortem identification. In this technique, which is based upon pilot studies with skeletonized mandibles of archival remains, the alveolar socket walls are sealed with a coat of cyanoacrylate cement and injected with a mixture of vinyl polysiloxane and barium sulfate. Radiographs are produced with the radiopaque mixture in place, which highlights the antemortem morphology of the roots. Subsequently, the impression material is removed, resulting in no gross alteration of the evidence. The radiographs made with this technique, as well as the impressions, can be stored for later use at a trial or pending the discovery of antemortem dental evidence.

Adult↗

[Facial skeleton traumas in farmers in east-central region of Poland (part I)].

On the base of medical documentation, we presented epidemiological analysis of 215 patients treated for traumas of facial skeleton, which occurred during agricultural tasks. It was concluded that the injuries mentioned above occurred more frequently in males at the age between 18-40. The traumas in this group occurred mainly during use of jig saw or other tools and machinery used in agriculture. However in the female population injuries of facial skeleton resulted mainly from zoogenous trauma and accidental fall downs.

Accidents, Home↗

[Use of skeletonized double mammary arteries for emergency/urgency myocardial revascularization in unstable angina: technical aspects and long-term outcome].

BACKGROUND: Surgical revascularization employing bilateral internal mammary arteries (IMAs) is an excellent procedure in elective surgery, but its role in urgent/emergent procedures is still debating. This retrospective study evaluates the feasibility, safety and surgical early outcomes of employing double skeletonized IMAs in patients with unstable angina (UA) undergoing urgent/emergent revascularization. METHODS: From January 1997 to May 2004, 824 patients (491 males, 333 females, mean age 64 +/- 12 years) underwent urgent revascularization for UA. Bilateral IMAs were employed in 346 (42%) patients (group B) and isolated and/or saphenous vein grafts in the remaining 478 (58%) patients (group M). There were no significant differences in preoperative risk factors between the two groups (mean EuroSCORE value). RESULTS: Postoperative stay was free from complications in 87% of patients of group B and 91% of group M. In-hospital mortality (group B 5.9%, group M 5.3%, p = NS) and perioperative myocardial infarction (group B 2.2%, group M 1.96%, p = NS), mean coronary care unit stay and total hospital stay were similar in both groups. Actuarial survival at 1, 3, 5 and 7 years was respectively 98.7, 97.5, 96.9 and 96.1% in group B and 99, 93.4, 92.1 and 88.4% in group M (p < 0.05). At a mean follow-up of 6.6 years the event-free survival (p = 0.021) and reoperation-free cardiac survival (p = 0.003) were better in group B. Multivariate analysis identified that age > 65 years (p = 0.01), congestive heart failure (p = 0.001), left ventricular ejection fraction < 35% (p = 0.03), and > 1 ischemic irreversible area (p = 0.02) are negative predictors for reoperation-free cardiac survival. The employment of the left IMA (p = 0.006) and of both IMAs (p = 0.001) were positive predictors for the overall survival and reoperation-free cardiac survival. CONCLUSIONS: Our results show that the use of skeletonized bilateral IMAs is associated with an acceptable risk and a lower incidence of postoperative complications in patients with UA, improving late outcomes in this group of patients.

Actuarial Analysis↗

An integrated approach to assess structure-to-function relationships in the skeleton.

In general, the disciplines of biomechanics, morphology, densitometry, biochemistry, cell biology and molecular biology have advanced independently of one another. In spite of this fragmentation, there have been incremental increases in our understanding of the organization, mechanical properties, growth, remodeling and repair of the tissues comprising the skeleton. As a practical application, this increased knowledge has greatly improved our capabilities for early diagnosis of bone loss and has proven similarly useful in determining the efficacy of interventions to prevent osteoporosis. This approach, however, has been much less successful in countering several other important musculoskeletal disorders, including arthritis. In the immediate future, a major emphasis will be placed on tissue regeneration (engineering) to restore lost mechanical function to a compromised skeleton. To accomplish this goal, it will be necessary to employ much more sophisticated approaches toward evaluating the structure-to-function relationships, ones which will include integration of the respective contributions of gene expression, cell number and activity, matrix composition and architecture to achieve adequate tissue function.

Journal Article↗

Symmetrical fracturing of the skull from midline contact gunshot wounds: reconstruction of individual death histories from skeletonized human remains.

This paper reports a bilaterally symmetrical cranio-facial fracture pattern that is observed in self-inflicted, midline gunshot wounds. Five cases of self-inflicted gunshots wounds are presented as follows: two high-powered rifle cases, two shotgun cases, and one handgun case. In all five cases the remains were either decomposing or skeletonized and submitted to forensic anthropologists. Following identification, the main focus of the anthropological examination was the analysis of perimortem trauma to the skeleton. In each case, the skull was submitted in a highly fragmented state. Nevertheless, by focusing on the pattern of perimortem cranio-facial fractures, the anthropologists contributed key information regarding the circumstances of death. The observed symmetrical cranio-facial fracture patterns in the above cases are described in detail and interpreted. The specific location of the linear fractures is discussed, as well as the theoretical rationale behind the location in terms of skeletal architecture, such as buttresses, struts, and sutures. The interpretive framework provided by this paper may prove helpful to others who are faced with similar cases of cranio-facial fracturing.

Adult↗

[Skeleton reconstruction of oblique facial clefts using mandibular outer table].

OBJECTIVE: This paper presents a new method of skeleton reconstruction for oblique facial clefts using autogenous bone of the mandibular outer table. METHODS: In the operation, the mandibular outer table was harvested through the intraoral approach. Assisted with internal rigid fixation technique, the mandibular outer table was used to reconstruct the naso-orbital framework as inlay or onlay bone graft. RESULTS: From 1993 to 2001, seven cases of oblique facial clefts were repaired with mandibular outer table bone graft. Postoperative follow-up for 6 months to 3 years demonstrated that the grafted bone healed well with the adjacent bones. No obvious bone resorption was observed. The facial appearance was improved greatly. CONCLUSIONS: The mandibular outer table, with similar bone density to the calvarium, is easy to harvest without donor site scar. The method is quite ideal for skeleton reconstruction of oblique facial clefts.

Adolescent↗

[Clinical application of skeleton reconstruction in human hand allograft].

OBJECTIVE: To study and summarize the clinical experience and significance of the skeleton reconstruction of human hand allografts. METHODS: From January 2001 to October 2003, human hand allografts were applied to treat 4 cases of traumatic hand defect (6 hands) at different levels. During operation, the ulna and radius were reduced anatomically and fixed firmly with 3.5 mm AO-plates and screws according to AO internal fixation principle. The X-ray films were taken periodically and the function recovery of hand allografts was observed and estimated. RESULTS: The 4 cases were followed up for 4-36 months postoperatively. The clinical healing of fracture in 4 cases (6 hands) was achieved after 9 weeks, and by means of comprehensive assessment including the joint function, muscle strength, sensation, appearance, sequela and the ability of work, the satisfactory effects were gained eventually. CONCLUSION: It is significant for human hand allografts to reconstruct skeleton firmly.

Adult↗

Steps for targeting exercise towards the skeleton to increase bone strength.

Osteoporosis is a disease of bone fragility resulting mostly from low bone mass and a concomitant increase in the risk for fracture. Exercise is a commonly prescribed intervention for osteoporosis because bone tissue is mechanosensitive. The ability of mechanical stimuli to influence bone biology has been known for over a century, but it has been only in the past several decades that great gains have been made in terms of understanding factors that influence this response. By understanding these factors, steps can be developed to maximize the osteogenic effects of exercise on the skeleton and potentially reduce the incidence of bone fractures. This paper outlines these steps. They include: 1) starting exercise when young while the skeleton is most responsive to mechanical stimuli; 2) selecting exercises that are dynamic and high-impact to maximize osteogenic responses, such as jumping for the lower extremity and racquet sports for the upper extremity; 3) exercising the specific skeletal regions you want to strengthen as the bone response to mechanical loading is highly site-specific; 4) exercising briefly, yet often to offset the desensitization of skeletal mechanotransduction pathways; and 5) continuing to exercise as you age to prevent bone loss and reduce the risk of falls. Following these steps will help to promote skeletal health at all ages and may reduce an individuals risk for fracture by augmenting bone mass and size during youth, while reducing age-related bone loss and the risk for falls in adulthood.

Journal Article↗

[Development and congenital malformations of the skeleton].

The article presents current knowledge on skeleton development, which is initiated by mesenchymal cells condensation. The mesenchymal to epithelial transformation, vascular regression, cartilage formation and ossification are involved in normal skeletogenesis. All the processes are influenced by various genes and regulatory molecules, e.g., N-cadherin, vascular endothelial growth factor, transforming growth factor-beta, syndecan-3 and others. The chondrogenesis depends on the Sox genes expression - Sox5, Sox6 and especially Sox9, which continued action produce persistent cartilage. The essential role in osteogenesis is played by the transcription factor Runx2 that directly activates expression of genes coding typical bone proteins like collagen type I, osteocalcin, osteopontin and osteonectin. Development of fibrous, cartilaginous and synovial joints, bone maturation and remodeling, and mechanisms leading to skeleton dysmorphogenesis are also discussed.

Antigens, CD↗

Changing of facial skeleton for treatment of obstructive sleep apnoea syndrome.

Obstructive sleep apnoea syndrome (OSAS) is a potentially life-threatening disorder. It is characterized by at least five episodes of apnoea or hypopnoea during sleep lasting for more than 10 seconds. Apnoea or hypopnoea are accompanied by respiratory efforts. Changes of the facial skeleton by mandibular or maxillo-mandibular advancement belong to surgical techniques which might affect moderate and severe OSAS. In the surgical procedure mandible alone or the upper and lower jaws are moved forward by at least 10 mm. Thus also muscles fixed to the facial skeleton and upper airway dilatators are moved forward. The discussion also mentions possible complications and limitations of this surgical technique.

Humans↗

The genetic disorders of the red cell skeleton.

The genetic disorders of the red cell skeleton encompass hereditary spherocytosis, hereditary elliptocytosis and an array of ill-defined haemolytic anaemias. Protein chemistry and molecular genetics have illuminated the supramolecular arrangement of the skeleton, the sequence and three-dimensional structure of its protein components, the exon-intron organization of the corresponding genes, the complex splicing undergone by their transcripts. Basically, hereditary spherocytosis is often due to a defect of ankyrin, hereditary elliptocytosis usually results from alterations of spectrin or protein 4.1. Other conditions are related to changes in the anion transporter or protein 4.2. The heterogeneity of the genomic changes, their ultimate consequences at the protein level open windows on fundamental problems concerning alternative splicing of mRNAs and structure-function relationships in proteins.

Anemia, Hemolytic↗

Membrane skeleton protein 4.1 in inner segments of retinal cones.

Retinal cone photoreceptors are polarized cells with discrete functional compartments. Little is known of the membrane-skeleton components in cones responsible for the maintenance of cellular morphology and compartmentalization of integral membrane proteins. The authors describe a high density of the membrane-skeleton protein 4.1 in the myoid region of cones in retinas of developing and adult frogs, and adult lizards, turtles, and chicks. In the developing retina, a discrete locus of protein 4.1 is first detected by immunocytochemistry in the cone myoid after the completion of photoreceptor synaptogenesis. Protein 4.1 immunoreactivity expands to line the plasma membrane of the myoid after cones attain their adult proportions. Nonerythroid alpha-spectrin and protein 4.1 colocalize in the cone myoid, and both are in close proximity to long bundles of f-actin that traverse the myoid and extend from the cone outer segment to the external limiting membrane. By analogy to the erythrocyte, where protein 4.1 modulates the interaction of spectrin and actin to regulate cell shape, the authors propose that protein 4.1 interacts with nonerythroid alpha-spectrin and f-actin in cones and plays a role in the maintenance of cone inner segment morphology.

Actins↗