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PubMedDate not supplied

Computed tomography of the human developing anterior skull base.

The ossification of the anterior skull base, especially the lamina cribrosa, has been studied by computed tomography and histopathology. Sixteen human fetuses, (referred to our laboratory for pathological examination after spontaneous abortion between 18 and 32 weeks of gestation) and three infants, (1, 2 and 6 years of age, respectively) were examined. The cartilaginous preformation of the anterior skull base creates a 'pseudo-defect' on CT in the coronal plane, even with ultra thin sections and high resolution CT. By the age of 6 years of life this 'artefact' is no longer, as the present ossification of the lamina cribrosa is, by that time, complete.

Embryonic and Fetal Development↗
PubMed2004

5-azacytidine alters TGF-beta and BMP signaling and induces maturation in articular chondrocytes.

Maintenance of the articular surface depends on the function of articular chondrocytes (ACs) which produce matrix and are constrained from undergoing the maturation program seen in growth plate chondrocytes. Only during pathologic conditions, such as in osteoarthritis, are maturational constraints lost causing recapitulation of the process that occurs during endochondral ossification. With the aim of establishing a model to identify regulatory mechanisms that suppress AC hypertrophy, we examined the capability of 5-azacytidine (Aza) to have an impact on the maturational program of these cells. Primary ACs do not spontaneously express markers of maturation and are refractory to treatment by factors that normally regulate chondrocyte maturation. However, following exposure to Aza, ACs (i) were induced to express type X collagen (colX), Indian hedgehog, and alkaline phosphatase and (ii) showed altered colX and AP expression in response to bone morphogenetic protein-2 (BMP-2), transforming growth factor-beta (TGF-beta), and parathyroid hormone-related protein (PTHrP). Since Aza unmasked responsiveness of ACs to BMP-2 and TGF-beta, we examined the effect of Aza treatment on signaling via these pathways by assessing the expression of the TGF-beta Smads (2 and 3), the BMP-2 Smads (1 and 5), and the Smad2 and 3-degrading ubiquitin E3 ligase Smurf2. Aza-treated ACs displayed less Smad2 and 3 and increased Smad1, 5, and Smurf2 protein and showed a loss of TGF-beta signaling on the P3TP-luciferase reporter. Suggesting that Aza-induction of Smurf2 may be responsible for the loss of Smad2 and 3 protein via this pathway, immunoprecipitation and metabolic labeling experiments confirmed that Aza accelerated the ubiquitination and degradation of these targets. Overall, Aza-treated ACs represent a novel model for the study of mechanisms that regulate maturational potential of articular cartilage, with the data suggesting that maturation of these cells may be due to up-regulation of Smad1 and 5 coupled with a Smurf2-dependent degradation of Smad2 and 3 and loss of TGF-beta signaling.

Animals↗
PubMed2006

Generalised and conditional inactivation of Pex genes in mice.

During the past 10 years, several Pex genes have been knocked out in the mouse with the purpose to generate models to study the pathogenesis of peroxisome biogenesis disorders and/or to investigate the physiological importance of the Pex proteins. More recently, mice with selective inactivation of a Pex gene in particular cell types were created. The metabolic abnormalities in peroxisome deficient mice paralleled to a large extent those of Zellweger patients. Several but not all of the clinical and histological features reported in patients also occurred in peroxisome deficient mice as for example hypotonia, cortical and cerebellar malformations, endochondral ossification defects, hepatomegaly, liver fibrosis and ultrastructural abnormalities of mitochondria in hepatocytes. Although the molecular origins of the observed pathologies have not yet been resolved, several new insights on the importance of peroxisomes in different tissues have emerged.

Animals↗
PubMedDate not supplied

MR appearance of idiopathic synovial osteochondromatosis.

OBJECTIVE: A retrospective review of the MR examinations in 21 patients with idiopathic synovial osteochondromatosis (ISO) was performed to determine its MRI characteristics. MATERIALS AND METHODS: Twenty-one patients diagnosed with ISO had undergone MRI prior to surgery. The MR images were retrospectively evaluated for configuration and extent of lesion as well as for signal characteristics. RESULTS: Three distinct MR patterns were seen in ISO: A--lobulated homogeneous intraarticular signal isointense to slightly hyperintense to muscle on T1-weighted images and hyperintense on T2-weighted images (n = 3); B--pattern A plus foci of signal void on all pulse sequences (n = 17); and C--features of pattern A and B plus foci of peripheral low signal surrounding central fat-like signal (n = 2). The foci of signal void in pattern B and C corresponded to areas of calcification and the foci of peripheral low signal surrounding central fat-like signal in pattern C corresponded to areas of ossification. CONCLUSION: The MR appearance of ISO appears sufficiently unique to allow its differentiation from other causes of intraarticular pathology.

Adolescent↗
PubMed1992

Imaging of degenerative processes of the spine.

Because MR imaging provides accurate, clear, and noninvasive imaging for diseases of the spine and spinal cord, it has become the primary imaging modality in patients with spinal pathology. Degenerative processes of the spine are frequent, including disk degeneration and herniations, spondylosis, spinal canal stenoses, calcifications, and ossifications of the spinal ligaments. Several papers have been published recently on MR imaging in degenerative processes of the spine.

Humans↗
PubMed1976

[Lesions of the cervical region of the spinal cord in so-called infantile cerebral palsy].

A EMG study of the brachial muscles in 41 children with spastic paralyses of the lower extremities, weakness of the hands concomitant with a mild hypotrophy of the muscles, disturbed functions of the III, VII and vestibular nerves and satisfactory mental development revealed the following: there were signs of motoneuron excitation in the cervical part of the spinal cord (motor units at rest in a maximum muscle contraction and in an elaboration of tenden reflexes), their destruction (a decreased amplitude of potentials of volitional contraction in combination with muscle hypotrophy) and a desinhibition of spinal mechanisms. Causes of such damage may be intrauterine pathology, perinatal hypoxia, delivery complicated by an incorrect position of the foetus and a mechanical trauma in an incomplete ossification of the spine, in premature infants.

Adolescent↗
PubMed2005

Dendriform pulmonary ossification, a form of diffuse pulmonary ossification: report of a 26-year autopsy experience.

CONTEXT: Dendriform pulmonary ossification (DPO) is an uncommon form of diffuse pulmonary ossification that typically affects the pulmonary interstitium in a setting of interstitial fibrosis. OBJECTIVE: To determine the incidence and characteristics of DPO and correlate the clinical, radiographic, and pathologic features in order to better understand its pathogenesis and behavior and facilitate proper therapy. DESIGN: Adult autopsies performed at a tertiary care center from 1978 to 2004 were reviewed to identify cases of DPO. Clinical, radiographic, and pathologic findings, including ultrastructural studies in select cases, were analyzed and correlated. RESULTS: Eight cases of DPO were identified from 1393 adult autopsies. None of the cases was diagnosed antemortem. The prevalence of DPO was 0.5%, and the incidence was 0.28 cases per year. Most patients had a history of chronic lung disease, and all were 65 years of age or older. CONCLUSIONS: Dendriform pulmonary ossification is an uncommon and unusual entity seen incidentally at autopsy and associated with chronic lung disease. It is well demonstrated in postmortem examination, confirmed by microscopy and ultrastructural study, but rarely diagnosed and virtually never considered clinically. Clinical diagnoses include bronchiectasis and interstitial pneumonitis based on radiographic evidence. With its associated radiographic/pathologic findings, DPO should be considered in the differential diagnosis of chronic lung disease.

Aged↗
PubMed2002

Prenatal growth deficiency with narrowness of the cervical spine, subglottic stenosis, hip dislocation, and severe delayed bone ossification: a new skeletal dysplasia.

A boy with a severe prenatal onset dysplasia, prominent occiput, hypertelorism, epicanthus inversus, low-set ears, flat nasal bridge, small nares, cleft palate, subglottic stenosis, narrow cervical canal, undermodeled bones, cortical thinning of the diaphyses, hip dislocation, severely delayed bone ossification, and apparently normal intellectual development is described. Osseous histopathological studies were unremarkable. The boy's parents are first cousins, suggesting recessive inheritance. To the best of our knowledge, this association has not been reported before, and may be considered a novel syndrome.

Abnormalities, Multiple↗
PubMed1997

Expansive laminoplasty for multilevel cervical OPLL.

Expansive laminoplasty is a new technique for the management of cervical ossification of the posterior longitudinal ligament (OPLL). Twenty patients were followed up for an average 3-year period. Follow-up examinations were performed every 3 months and at 1-year intervals. On lateral radiographs, the average increase in the anteroposterior (AP) diameter of the canal was 42%. Bone fusion was documented on computed tomography (CT) studies 3 months after surgery in 96% of patients. An average 83% of normal range of motion was preserved on long-term follow-up (3 years on average). Postoperative improvement over the same interval increased from an average preoperative Japanese Orthopaedic Association score of 7.9 to 14.1 Expansive laminoplasty as described is safe and has a high fusion rate, while better preserving the normal range of motion.

Constriction, Pathologic↗
PubMed1994

Germ cell tumors of the sacrococcygeal region: radiologic-pathologic correlation.

Germ cell tumors of the sacrococcygeal region include mature and immature teratomas and endodermal sinus tumor. Most sacrococcygeal teratomas are discovered in the newborn period as an obvious mass, but they may be detected prenatally. Endodermal sinus tumors are usually discovered later in early childhood. Pathologically and radiologically, teratomas are either both cystic and solid, predominantly cystic, or rarely solid. Over 50% have calcification or ossification. Most malignant teratomas have substantial solid components and may contain calcification. Treatment is surgical excision including coccygectomy. Malignant tumors are treated with both surgery and chemotherapy. Prognosis is excellent for teratoma, although local recurrences may occur. Malignant teratomas have had a dismal prognosis in the past, which has been improved with multiagent chemotherapy.

Child↗
PubMedDate not supplied

[The Cogan syndrome].

Cogan's Syndrom is the clinical entity of non syphilitic interstitial keratitis with vestibuloauditory symptoms. Although the disease is not rate the pathohistological findings in temporal bones are only reported twice. Our communication deals with observations made on a temporal bone out of O. Mayer's collection. The most striking findings are: the endolymphatic hydrops, the degeneration of the organ of Corti, the pathology of the ductus reuniens, the fibrosis within the cisterna perilymphatica, the destruction of the maculae and the most uncommon ossification within the scala vestibuli of the basal turn. The vessels seem to show signs of angitis.

Acoustic Maculae↗
PubMed1980

[Use of tissue adhesives in the reconstruction of the frontal sinus].

Fractures of the anterior wall of the frontal sinus, complicated by impression of bone fragments, can cause serious functional and cosmetic disturbances. Surgeons often remove those fragments and obliterate the sinus. But disfigurement from the operation will be worse than that caused by original lesion and can only be corrected by further plastic surgery. Some surgeons use the technique of joining bone fragments by metal or thread sutures but it is only possible when the fragment are not too small. We present four patients treated over a period of 1975-1979 with the reconstruction of the frontal sinus from its fragments using cyanoacrylic tissue adhesive (Chirurcoll-Polfa). One patient was operated immediately after an accident, two patients one month after a trauma and another after six months. The adhesive tissue was applied in single drops directly on the edges of dry surface of bone fragments. The fragments can be joined only in some points because the tissue adhesive can not make an isolation layer between them. The tissue adhesive speeds up the processes of ossification. We observed no interference with wound healing, no detrimental effect of this method in the postoperative course and no pathologic local and general reactions. The adhesive tissue makes manipulation in the operative field easier. The functional and esthetic results were satisfactory and further corrective surgery was not necessary.

Adult↗
PubMed2000

Recent progress in the study of pathogenesis of ossification of the posterior longitudinal ligament.

The pathogenesis of ossification of the posterior longitudinal ligament (OPLL) is still unknown. Gene analysis and molecular biology have been introduced in recent years, and etiologic and pathological clarifications are being achieved. An important role of the genetic background in the development of this disease was demonstrated by pedigree survey, twin survey, and HLA haplotype study. The results of gene linkage study showed that patients with OPLL have a significantly higher incidence of genetic abnormalities found in the XI collagen (alpha)2 gene (COL11A2) region. From the gene mapping of this abnormality, the abnormal N-propeptide of the COL11A2 gene was found to be responsible. We are planning to undertake genetic analysis of the whole of chromosome VI to find the pathogenic genes responsible for OPLL in addition to COL11A2. A cell biological approach is also necessary to make clear the relationship between abnormalities of the COL11A2 gene and ossification of the ligament. In future, identification of the susceptible gene, elucidation of its function, and study toward the development of preventive and therapeutic drugs will advance.

Chromosome Mapping↗
PubMed2003

In vivo somatic cell gene transfer of an engineered Noggin mutein prevents BMP4-induced heterotopic ossification.

BACKGROUND: The formation of the skeleton requires inductive signals that are balanced with their antagonists in a highly regulated negative feedback system. Inappropriate or excessive expression of BMPs (bone morphogenetic proteins) or their antagonists results in genetic disorders affecting the skeleton, such as fibrodysplasia ossificans progressiva. BMP signaling mediated through binding to its receptors is a critical step in the induction of abnormal ossification. Therefore, we hypothesized that engineering more effective inhibitors of this BMP-signaling process may lead to the development of therapies for such conditions. METHODS: BMP4-induced heterotopic ossification was used as a model for testing the ability of the BMP antagonist Noggin to block de novo bone formation, either by local or systemic delivery. Since Noggin naturally acts locally, a Noggin mutein, hNOGDeltaB2, was engineered and was shown to circulate systemically, and its ability to block heterotopic ossification was tested in a mouse model with use of adenovirus-mediated somatic cell gene transfer. RESULTS: A mouse model of BMP4-induced heterotopic ossification was developed. Local delivery of wild-type NOG inhibited heterotopic ossification, but systemic administration was ineffective. In contrast, systemic delivery of the adenovirus encoding hNOGDeltaB2 resulted in systemic levels that persisted for more than two weeks and were sufficient to block BMP4-induced heterotopic ossification. CONCLUSIONS: BMP4-induced heterotopic ossification can be prevented in vivo either by local delivery of wild-type Noggin or after somatic cell gene transfer of a Noggin mutein, hNOGDeltaB2. Furthermore, the data in the present study provide proof of concept that a naturally occurring factor can be engineered for systemic delivery toward a desirable pharmacological outcome. CLINICAL RELEVANCE: Blocking bone formation is clinically relevant to disorders of heterotopic ossification in humans, such as fibrodysplasia ossificans progressiva. Furthermore, development of BMP antagonists as therapeutic agents may provide modalities for the treatment of other pathologic conditions that arise from aberrant expression of BMPs and/or from a lack of their antagonists.

Adenoviridae↗
PubMed1999

Heterotopic bone formation in association with pelvic fracture and urethral disruption.

PURPOSE: The initial and secondary management of pelvic fracture associated with disruption of the membranous urethra is the subject of a wide literature containing varied and controversial viewpoints. We have noted the presence of heterotopic bone formation surrounding the area of urethral injury in patients undergoing delayed repair. We investigated the etiology, incidence and risk factors associated with such an injury, as well as potential means of prophylaxis. MATERIALS AND METHODS: We reviewed the current literature on heterotopic bone formation with similar traumatic injury. While instances of severe urethral disruption of this type are fortunately rare in children we describe prepubertal boys with such an injury complicated by heterotopic ossification. RESULTS: The incidence of heterotopic ossification reported in children and adolescents is 3 to 15%, which is less than 15 to 80% reported in adults. Risk factors associated with traumatic heterotopic ossification include prolonged operating time, hematoma formation, degree of bony debris, devitalized muscle and concomitant infection. Prophylaxis with single low dose radiation or nonsteroidal anti-inflammatory drugs has been shown to be effective in the prevention of heterotopic ossification and may be beneficial in this patient population. CONCLUSIONS: Heterotopic bone formation associated with severe traumatic injury in the presence of devitalized tissue resulting in the pathological formation of new bone is rare. This complication is only associated with the most severe pelvic fractures. Prophylaxis in these most severe cases with low dose radiation or nonsteroidal anti-inflammatory drugs can prevent the formation of heterotopic bone.

Child↗
PubMed2001

Refractory heterotopic ossification with complications.

BACKGROUND: Heterotopic ossification (HO) following spinal cord injury can lead to various complications, including venous thrombosis, autonomic dysreflexia, and pressure ulcers. We report refractory, complicated HO in a 19-year-old man with C8 incomplete tetraplegia. He first presented at 9 weeks postinjury with fever and swelling of his right leg. Ultrasound indicated a deep venous thrombosis (DVT). Persistent symptoms prompted triple-phase bone scan and magnetic resonance imaging (MRI), which revealed HO compressing the right external iliac vein and no evidence of DVT. The HO was complicated by hypercoagulability. CLINICAL COURSE: The HO was refractory to oral indomethacin and etidronate; therefore, intravenous etidronate was instituted, resulting in only a transient decrease in alkaline phosphatase. Local irradiation of the right hip did not decrease the activity of HO. The patient was discharged on oral etidronate, indomethacin, and warfarin. This complicated case raises issues regarding early diagnosis and aggressive treatment of HO, as well as treatment of associated hypercoagulability.

Constriction, Pathologic↗