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PubMed1979

Articular pathology of ankylosing spondylitis.

The essential articular pathology of AS may be said to reflect the occurrence, severity, and overall bias of (a) synovitis which tends to produce articular erosion, and (b) an inflammatory enthesopathy which results in capsular ossification in diarthrodial joints and syndesmophyte formation in cartilaginous joints, both of which are primarily responsible for bony ankylosis. Nonspecific secondary mechanisms contribute to the final picture. These include enchondral ossification, which produces synostosis, osteoporosis and altered stress distribution which make the axial skeleton susceptible to trivial trauma, the destructive effects of which are sometimes described as spondylodiskitis.

Bone and Bones↗
PubMed1991

[Role of echography in the diagnosis and treatment monitoring of congenital acetabular dysplasia. Personal experience with 2000 examined children].

Since 1987 the authors have used sonography (US) as the means to detect dysplasia and congenital dislocation of infant hips, to define severity of the disease, and to monitor the results during treatment. In this paper the authors report their experience in 2000 examined babies. In 20 young patients, mainly females, 25 pathologic joints were detected and classified as 2c-4 hips. Orthopedic treatment employed Pavlick harness and, in case of partial success, Milgram abduction pillow too. This treatment has always been successful, except for a case with severe dislocation with echostructural changes in the acetabular cartilage. In this case, surgery was necessary. "Delayed ossification" (type 2b hips) and physiological immatury (type 2a hips) with alpha angle greater than or equal to 55 degrees were not splinted in abduction: normal maturity has always been attained without dysplastic involutions. Our experience confirmed US value in the early diagnosis of congenital acetabular dysplasia, when clinics exhibited evident limitations. Nonetheless, management and economical problems are still to be solved, due to the relationship of the widespread use of US in infant population and to its successful results, also considering the low incidence of the examined pathologic condition.

Acetabulum↗
PubMed1975

[Modern viewpoints of hip joint dysplasia, clinical and radiologic. III. Radiologic follow-up].

Dysplasia of the hip-joint can unequivocally be recognized at the age of 7 weeks as proved by further developments. Defects in the acetabular angle remain in spite of abductionsplints which may have to be continued until the acetabular roof including its angle have become normal. It must not be abandoned when the PDW has returned to normal. One ought to demand that treatment should be started before this stage has been reached i.e. before the 7th week of life. 1. Clinical and radiologic observations in the first 3 months prove that dysplasia of the hip-joint can be well recognized at this early stage. A diagnosis beyond the 3rd month is a late diagnosis. 2. Essential radiologic signs are: wedge-segments, delayed ossification of the acetabular angel, "Tailliation" at the angle, subchondral sclerosis of the acetabular roof, double contour of the angle in radiographs and, in severe cases, steep PDW. 3. The averages and standard deviations of the Tonnis statistics have proved too large for the radiographs of the first 2 months of life and useless as shown in follow-up. 4. The dysplastic acetabulum is not immature but pathologic. 5. The common mild dysplasias cannot be recognized with PDW but only when searching for structural and contour changes at the acetabular roof.

Acetabulum↗
PubMedDate not supplied

Intralabyrinthine osteogenesis in Cogan's syndrome.

Temporal bones from a 64-year-old man who had Cogan's syndrome were examined by light microscopy. Although tissues of the middle ear appeared unremarkable, extensive ectopic bone formation was observed bilaterally in the inner ear membranous labyrinth. Such osteogenesis completely obliterated the cochlear compartments apically. Only the scala tympani compartment of the basal cochlear turn remained patent. Ectopic bone tissue was observed also in the vestibular semicircular canals. Marked degeneration of eighth nerve fibers and associated ganglion cells also was observed. To our knowledge, this report represents only the fourth temporal bone findings of Cogan's syndrome and demonstrates a more advanced pathologic state of inner ear pathology than those reported previously.

Adult↗
PubMedDate not supplied

Inner ear degeneration in acoustic neurinoma.

The temporal bones of three cases of acoustic neurinoma are described to illustrate histopathological features of inner ear lesions due to chronic partial obstruction of blood circulation by the tumor in the internal auditory meatus. Degenerative changes in the inner ear due to acoustic neurinoma were evaluated and compared with changes in the opposite ear. The main pathological findings in the inner ear which were attributed to the tumor were degeneration of nerve fibers and of ganglion cells, degeneration of the stria vascularis, degeneration of the tectorial membrane, fibrosis and ossification of a semicircular canal. Fairly good preservation of sensory cells was observed in the presence of total degeneration of nerve fibers and ganglion cells and subtotal degeneration of the stria vascularis.

Aged↗
PubMed1981

[Osteoplastic pneumopathy (disseminated bone formation in the lung)].

8 cases of pneumophathia osteoplastica (ppo) of branching type observed at patients having no vascular deformities and one case of a focal ppo at a patient with mitral stenosis are reported. Pathogenesis of the ppo of branching type in the majority of cases could not be clarified since the process appeared to be in the phase of definitive bone formation. Nevertheless in one of the cases in a septum of Y shape in addition to collagen fibres ending in bone tissue numerous elastic fibres have also been revealed. This fact, considering the presence of a normal bronchus in the area seems to evidence vascular origin of the lesion. The latter hypothesis could be verified by the bone-formation in the media of a vessel wall in another case. Further, in a case of primary chronic polyarthritis with ppo, bone-formation could be seen in the perivascular lung tissue with necrotizing pulmonal arteritis. Considering this finding the possibility of the primary role of necrotizing pulmonal arthritis in the pathogenesis of ppo have to be taken into account. Ppo should be classified as one of the alveolocapillary block syndromes. In some cases it may have clinico-pathological significance. It may lead to bronchietasis, emphyseme or cor pulmonale chronicum.

Aged↗
PubMed2000

Bilateral ossification in the Achilles tendon: a case report.

Bilateral symmetrical Achilles tendon ossification is presented, with a fracture on one side. A 57 year old male patient was conservatively treated for bilateral clubfeet in his childhood. Part of his Achilles tendons were replaced by ectopic bony mass and on the left it fractured without any trauma. The fracture was treated surgically by vertical thinning, intraosseal suture "osteosynthesis", and tendon flap plasty. This pathological condition healed in three months. Seven years after the operation the X-ray revealed total bony rebuilding. The ectopic bone mass has the same biological behaviour as the anatomic bones.

Achilles Tendon↗
PubMed2002

The osteopetrotic mutation toothless (tl) is a loss-of-function frameshift mutation in the rat Csf1 gene: Evidence of a crucial role for CSF-1 in osteoclastogenesis and endochondral ossification.

The toothless (tl) mutation in the rat is a naturally occurring, autosomal recessive mutation resulting in a profound deficiency of bone-resorbing osteoclasts and peritoneal macrophages. The failure to resorb bone produces severe, unrelenting osteopetrosis, with a highly sclerotic skeleton, lack of marrow spaces, failure of tooth eruption, and other pathologies. Injections of CSF-1 improve some, but not all, of these. In this report we have used polymorphism mapping, sequencing, and expression studies to identify the genetic lesion in the tl rat. We found a 10-base insertion near the beginning of the open reading of the Csf1 gene that yields a truncated, nonfunctional protein and an early stop codon, thus rendering the tl rat CSF-1(null). All mutants were homozygous for the mutation and all carriers were heterozygous. No CSF-1 transcripts were identified in rat mRNA that would avoid the mutation via alternative splicing. The biology and actions of CSF-1 have been elucidated by many studies that use another naturally occurring mutation, the op mouse, in which a single base insertion also disrupts the reading frame. The op mouse has milder osteoclastopenia and osteopetrosis than the tl rat and recovers spontaneously over the first few months of life. Thus, the tl rat provides a second model in which the functions of CSF-1 can be studied. Understanding the similarities and differences in the phenotypes of these two models will be important to advancing our knowledge of the many actions of CSF-1.

Alternative Splicing↗
PubMed2005

Surgical experience with symptomatic thoracic ossification of the ligamentum flavum.

OBJECT: Symptomatic thoracic ossification of the ligamentum flavum (OLF) is rare, and its prognostic factors remain unclear. The authors retrospectively studied 24 patients with surgically treated thoracic OLF to delineate its prognostic factor. METHODS: The clinical manifestations, radiological studies, surgical records, and pathological findings were reviewed. Preoperative and postoperative neurological data were reappraised using the American Spinal Injury Association and modified Japanese Orthopaedic Association (JOA) scoring systems. Spearman rank-correlation coefficients and nonparametric tests were used to analyze the correlations between the variables of patient characteristics, preoperative duration of symptoms, preoperative neurological status, associated spinal disorder(s) other than thoracic OLF, and the final functional outcome. CONCLUSIONS: Decompressive surgery is indicated in patients in whom symptomatic thoracic spinal cord compression is caused by intruding OLF. Magnetic resonance imaging can provide sufficient clues for the diagnosis of thoracic OLF. Higher preoperative modified JOA scores of 3 and 4 are positively correlated with better postoperatiVe functional recovery than lower scores. Surgery should be performed as soon as possible before independent ambulatory function is impaired.

Aged↗
PubMed1992

[Influence of muscle tone and force on ossification in postcomatose patients].

68 post-traumatic brain-injured patients in postcomatose unawareness (PCU) states were admitted during 1982-1987. 32 of them also suffered from fractures of the long bones and pelvis (total of 51 limbs). The muscle tone and force of all limbs were examined and x-rays of the large joints and of the fractures were taken periodically. Periarticular new-bone formation appeared in 32 patients. Normal callus developed in 41 fractured limbs and hypertrophic callus in 18. We found that pathological muscular tone (hypertonus and hypotonus) and pathologically decreased muscle force (paresis and plegia) were closely correlated with the appearance of periarticular new-bone formation. The extent of callus formation was not correlated with muscle tone and force.

Biomechanical Phenomena↗
PubMed1990

Traumatic basal subarachnoid hemorrhages: autopsy material analysis.

Fourteen cases of traumatic basal subarachnoid hemorrhages, examined in the years 1980-1988, were presented. Bleeding was connected with the fracture of the transverse process of the atlas in eleven cases and with atlanto-occipital dislocation in three. The source of hemorrhage--vertebral artery rupture--was determined only four times. In nine cases (63%) atlases showed various developmental malformations (posterior ponticle and foramen arcuale, epitransverse process, incomplete ossification of the transverse process). The results were related to the literature of the subject. Special attention was paid to the mechanism of the rupture of the vertebral arteries in which developmental disorders and pathological conditions may play an important role.

Adolescent↗
PubMedDate not supplied

Little League survey: the Houston study.

1. In a study of 595 Little League pitchers, approximately 17% had a history of elbow symptoms: only 1% had elbow symptoms which had ever excluded them from pitching. There was no correlation between the presence of symptoms and years of pitching experience. 2. Some limitation of active extension of the elbow was seen in 12% of the pitchers; however, there was no correlation between elbow flexion contractures and years of pitching experience, symptoms, or roentgenographic pathology. No normal child had an elbow flexion contracture greater than 15 degrees. 3. A slight valgus carrying angle is considered a normal anatomic variant in the dominant arm. There was no correlation between valgus carrying angle and years of pitching experience or symptoms or roentgenographic pathology. 4. No roentgenographic evidence of avascular necrosis of the capitellum, radial head, or both was seen in this population. 5. Roentgenographic findings such as bony hypertrophy, enlargement of the medial epicondyle, and secondary ossification centers are normal anatomic variants not related to symptoms. Undisplaced stress fractures of the medial epicondyle respond well to conservative treatment, with no functional residual.

Adolescent↗
PubMed2004

Pharmacological management of aseptic osteonecrosis in children.

Aseptic osteonecrosis (AON) in children can progress during ossification of cartilage in periods of increased growth or excessive physical stain and may occur in various locations in the skeleton. Disturbance of blood supply to the bone has been suggested as the main pathological mechanism involved in AON, which is characterised by the death of bone marrow and trabecular bone. The extent and development of osteonecrosis and the duration of disease until restorative healing, depend on the formation of new blood vessels, the spreading of vessels in the affected bony areas, the absorption of osteonecrotic tissue and the structure of new bone. Conservative and operative treatment options for AON vary according to the location and development of the disease and the age of the patient. The goal of all treatment options currently used today is to achieve relief of physical load in the affected bone and to promote and regulate blood supply. Treatment should be started early in order to minimise the extent of osteonecrosis and allow restorative healing. As the processes of myelopoiesis, myelophthisis and fracture healing become more clear, interest has focused on advances in the utilisation of bioactive factors to salvage bone in children affected by AON. Such methods include the use of osteoinductive agents, such as cytokines and bone morphogenetic proteins, as well as factors that stimulate angiogenesis and regulate blood supply. Currently, the prostacyclin analogue, iloprost (Ilomedin, Schering AG), has been successfully used in a pilot study in children suffering from early stages of AON.

Child↗
PubMed1980

[Valvular insufficiency in Lobstein's disease. A review of the literature apropos of a patient operated on for aortic insufficiency].

Lobstein's disease, a hereditary disorder of connective tissue, may rarely be accompanied by aortic or mitral regurgitation due to valvular dystrophy. The case reported is that of a patient with severe aortic incompetence with dilatation of the ascending aorta, complicated by angina pectoris and left ventricular failure; the patient also had Lobstein's disease with numerous spontaneous fractures, transverse lines of ossification, blue sclera skeletal deformities, and a positive family history of blue sclera. At surgery, valvular dystrophy was confirmed and corrected by aortic valve replacement. The patient's brother died recently with the same pathological association of Lobstein's disease and aortic incompetence. 28 cases of valvular disease and Lobstein's disease have been reported 12 with pathological evidence (at operation) on the purely dystrophic origin of the valvular regurgitation, and 2 with histological diagnoses alone in two newly born children. Aortic incompetence with dilatation of the ascending aorta is commoner than mitral incompetence. The macroscopical and histological appearances are similar to Marfan's syndrome and account for the operative risk of valvular replacement and for the incidence of postoperative haemorrhage. The rarity of valvular dysfunction in Lobstein disease contrasts with its relatively common occurrence in Marfan's syndrome.

Adult↗
PubMed1997

Dyggve-Melchior-Clausen syndrome without mental retardation (Smith-McCort dysplasia): morphological findings in the growth plate of the iliac crest.

Dyggve-Melchior-Clausen syndrome without mental retardation (Smith-McCort dysplasia) (SM) has clinical and radiographic findings similar to those of Dyggve-Melchior-Clausen syndrome (DMC) except for mental retardation. Iliac crest biopsies from two patients with SM were examined. The lace-like appearance of the iliac crests, which is a characteristic radiological sign of SM and DMC, was caused by bone tissue deposited in a wavy pattern at the osteochondral junction. The growth plate showed abnormal enchondral ossification with no columnarization of chondrocytes. Electron microscopy demonstrated chondrocytes with dilated cisternae of rough endoplasmic reticulum containing fine granular or amorphous material, similar to those reported in cases of DMC. Thus, SM has pathologic changes in common with DMC as a rough endoplasmic reticulum storage disorder, even though the mental condition is different.

Cartilage↗
PubMed1991

Choroid plexus tumors in childhood. Histopathologic study and clinico-pathological correlation.

Choroid plexus tumors are rare and account for only 2.3% (8/352) of primary childhood intracranial neoplasms in our series. Most of our patients were under 2 years of age. The tumors had a predilection for the lateral ventricle. Calcification was found in half of these tumors, and ossification was seen in 1 case. Histological features of malignancy including invasion, loss of differentiation, and severe nuclear pleomorphism pointed to a poor prognosis. Such features were found in 2 cases. Neither a large number of mitoses nor necrosis was a constant feature in cases of malignancy. Transthyretin, a marker for choroid plexus tumors, was positive in all cases. However, negative S-100 or positive carcinoembryonic antigen was not necessarily associated with a more aggressive histological pattern. All the papillomas could be totally resected without recurrence, and all the patients with carcinoma died within a few months.

Adolescent↗