Biomedical subjects

B L Jensen

Publications and source records attributed to B L Jensen.

101 records · Page 6Linked to original sources

PubMedDate not supplied

A comparison of the craniofacial morphology in 2-month-old unoperated infants with unilateral complete cleft lip and palate, and unilateral incomplete cleft lip.

This paper reports a cephalometric analysis of the craniofacial morphology in infants with unoperated unilateral complete cleft lip and palate (UCCLP) and unoperated unilateral incomplete cleft lip (UICL). The purpose of the study was to determine the nature and extent of the craniofacial deviations in UCCLP as compared to the morphology in UICL, which has previously been shown to be close to normal. The samples comprised 82 infants with UCCLP (58 males and 24 females) and 75 with UICL (48 males and 27 females). The mean age was about 2 months in both groups. The cephalometric analysis of craniofacial morphology included the lateral, frontal, and axial projections. The data were presented as mean plots of the craniofacial region including the calvaria, cranial base, orbits, nasal bone, maxilla, mandible, cervical column, pharynx, and soft-tissue profile. The most pronounced deviations in the UCCLP group were observed in the maxillary complex and the mandible. The most striking findings were: markedly increased width of the maxilla, a short mandible, and bimaxillary retrognathia except for the premaxillary area, which was relatively protruding and asymmetric. The study did not support the hypothesis previously suggested in the literature that cleft lip and palate is a craniofacial anomaly as size and shape of the calvaria and cranial base were found to be normal. The etiology of cleft lip and palate is still incompletely understood. Based on the present study, we suggest that facial type may be a liability factor that could represent a developmental threshold increasing the probability of cleft lip and palate.

Anthropometry↗

PubMedDate not supplied

Craniofacial growth in subjects with unilateral complete cleft lip and palate, and unilateral incomplete cleft lip, from 2 to 22 months of age.

This paper reports a longitudinal quantitative cephalometric analysis of the craniofacial growth in subjects with unilateral complete cleft lip and palate (UCCLP), and unilateral incomplete cleft lip (UICL), from 2 to 22 months of age. The purpose of the study was to determine the amount and direction of growth in UCCLP compared to UICL (control group) from 2 months of age (just prior to lip repair) to 22 months of age, 20 months later. The sample comprised of 49 subjects with UCCLP (37 males and 11 females) and 45 with UICL (29 males and 16 females). The cephalometric analysis of the craniofacial morphology included lateral, frontal, and axial projections. The data were presented as mean plots of the craniofacial region including the calvaria, cranial base, orbits, nasal bone, maxilla, mandible, cervical column, pharynx, and soft-tissue profile. A valid common coordinate system (registration according to the n-s line in the lateral projection, latero-orbitale line in the frontal projection, and meatus acusticus externus line in the axial projection for the landmark positions at examination 1 and 2) was ascertained. The growth at a specific anatomical location in a patient was defined as the displacement vector from the coordinate of the corresponding landmark in the X-ray at examination 1 to its coordinate at examination 2, corrected for X-ray magnification. The growth of an anatomical region in a patient was assessed by investigating the growth pattern formed by a collection of individual growth vectors in that region. The amount of growth in the UCCLP and UICL group was very similar. The general craniofacial growth pattern, in terms of the direction of growth, was also fairly similar in the UCCLP group and the control group. However, the maxilla and mandible showed a more vertical growth pattern than that observed in the control group. This study confirms that UCCLP is a localized deviation, and not a craniofacial anomaly, due to the fact that a normal growth potential has been observed in all craniofacial regions, except where the growth had been directly influenced by surgical intervention. Furthermore, the vertical growth pattern of the maxilla and mandible supports the hypothesis of a special facial type in cleft lip and palate individuals, and the facial type as a liability factor increasing the probability of cleft lip and palate.

Cleft Lip↗

PubMedDate not supplied

Incidence and prognostic significance of skin disease in patients with HIV/AIDS: a 5-year observational study.

Mucocutaneous diseases are common in HIV-infected patients. The aim of the present study was to determine the observed frequency of skin diseases and their prognostic significance for the progression of HIV infection. In a cohort of 150 HIV-infected patients diagnosed from 1986 to 1987 the number of mucocutaneous diseases, the CD4+ cell count, time to development of AIDS and survival time were registered for a period of 5 years. Eight patients were lost for follow-up. In the remaining 142 patients the number of mucocutaneous findings increased markedly during the observation period. The most frequent registered diseases and these with the highest increase were oral candidiasis, hairy leukoplakia, seborrhoeic dermatitis and herpes simplex. On average, 5.2 different diagnoses were registered per patient at the end of the follow-up period. The study showed that the total number of mucocutaneous diseases and the CD4+ cell count were significantly correlated to survival time and time to development of AIDS. The number of mucocutaneous diseases, like the CD4+ count, is an indicator of the immune system and the prognosis of HIV infection.

AIDS-Related Opportunistic Infections↗

PubMedDate not supplied

Craniofacial growth in cleidocranial dysplasia--a roentgencephalometric study.

The purpose of this study was to analyze craniofacial growth in cleidocranial dysplasia (CCD) with emphasis on bone remodeling, mandibular condylar growth, and tooth eruption. Skull radiographs of 22 CCD children followed longitudinally were examined. The span of time investigated varied for each patient, but the period from 5 years to adult age was well covered. Five patients had metallic implants inserted in the jaws; the implants enabled detailed analysis of bone remodeling, jaw rotation, and tooth eruption. In the calvaria, an open anterior fontanelle area persisted in most cases but decreased with age. A frontal sinus failed to develop or was diminutive in all cases but one. In the cranial base, the size increase of the sella turcica was reduced as a result of modest resorption at the floor and the posterior wall; the clivus was flexed in most cases, but the flexion remained stable during the observation period. Growth in maxillary height was severely reduced, primarily because resorptive lowering of the nasal floor was minimal. The amount of bone apposition on the orbital floor and the alveolar process was smaller than expected. Condylar growth direction was vertical giving rise to a forward rotation of the mandible in relation to the anterior cranial base. The expected resorptive remodeling below the mandibular angle and anteriorly on the ramus was negligible. The low maxilla, in combination with a marked forward mandibular rotation in cases with unstable occlusion as a consequence of eruption problems in the permanent dentition, gave rise to a diminished anterior facial height.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMedDate not supplied

Cleidocranial dysplasia: craniofacial morphology in adult patients.

The aim of the study was to carry out a detailed quantitative analysis of craniofacial morphology in a relatively large sample (N = 35) of adult patients with cleidocranial dysplasia (CCD) in order to contribute to the understanding of the characteristic craniofacial abnormalities. Roentgencephalometric films in the lateral, frontal, and basal projections were analyzed and mean facial diagrams for the lateral and frontal films were produced based on registration of 216 reference points. In the calvaria an increased width and a relatively more prominent frontal region were found. In the cranial base the most significant findings were reduced width, a cranial displacement of the clivus and sella, and an anteriorly facing foramen magnum. The orbital openings were high and narrow and nearly all patients had hypoplasia or aplasia of the nasal bones. The maxilla and mandible were reduced in height and width and the mandible was anteriorly inclined. It was concluded that all craniofacial regions are affected in CCD. Based on the present and previous studies, it is suggested that the abnormal craniofacial morphology in CCD may be explained by the skeletal dysplasia, combined with early cranial deformation and dysplastic and compensatory skeletal growth. Thus, the findings lead to the comprehension that several primary and secondary causal factors are involved in craniofacial morphogenesis in CCD, and that knowledge of early development is important when etiology and pathogenesis in a syndrome is searched for.

Adolescent↗

PubMedDate not supplied

Development of the skull in infants with cleidocranial dysplasia.

The purpose of the present paper is to clarify some of the questions regarding pathogenesis in cleidocranial dysplasia (CCD) in order to improve the understanding of the mechanisms leading to the craniofacial abnormalities characteristic for young and adult patients with the syndrome. The neonatal skull is described and craniofacial development from birth to 7 years analyzed. Skull radiographs of 11 CCD patients ages 0-7 years were examined. Study methods included plain radiographs of the skull and roentgencephalometric films in the lateral, frontal, and axial projections. In one patient, CT scans of the skull with 3-D reconstructions were available. Common to the four patients examined within the first 6 months postnatally was a marked delay of skeletal maturity. In the calvaria a severe reduction of calcification in all bones was found. Corresponding to the expected sutural areas, gaping defects were recorded, indicating that calcification of calvarial bones in the newborn CCD individual had only reached a stage corresponding to around 20 weeks gestational age. Calvarial size corresponded to that of normal newborns and the morphology was relatively normal, taking into account the pronounced deformation that took place during parturition due to the soft, undermineralized skull. At birth bony islands could be observed in the periphery of the occipital, parietal, and frontal bones. Ossification of the maxilla and the mandible seemed to be within normal limits. Posteriorly to the foramen magnum, the chondral part of the supraoccipital bone remained unossified. In the cranial base an increased width of the spheno-occipital synchondrosis was found.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

PubMedDate not supplied

Craniofacial abnormalities in 52 school-age and adult patients with cleidocranial dysplasia.

The purpose of the present investigation is to describe and discuss the craniofacial abnormalities in young and adult patients with cleidocranial dysplasia (CCD). Skull radiographs of 52 CCD patients were examined. Thirty-six patients were adult, 16 were between 7 and 16 years old. Facial photographs were available in 30 cases, and in one adult case with cranial CT scans, 3-D reconstructions were carried out. High quality photographs of six CCD skulls were also described. The paranasal sinuses were absent or diminished in all children and most adult patients. An inverted pear-shape of the calvaria and an open anterior fontanelle were present in more than 60% of the cases. In the cranial base an upward distortion of the clivus, an anteverted foramen magnum, and extremely narrow choanae were frequent traits, together with small sella and bulbous dorsum sellae. Nearly all patients and dry skulls showed hypoplasia or absence of nasal bones. The zygomatic arches were diminished or incomplete in all cases. Mandibular morphology was judged to be syndrome specific with the slender, upward-backward-pointing coronoid process. We have concluded that a great part of the abnormal qualitative traits are present to a lesser degree in CCD children, indicating a progressive abnormal development with advancing age. From the present and previous studies on CCD we have hypothesized that on top of their generalized reduction of growth potential, these patients suffer from a combination of skeletal immaturity resulting in deformations and a defective bone remodeling. We have acknowledged the importance of longitudinal investigations in order to ameliorate the understanding of the pathogenesis of CCD and thereby to improve total patient care. Correction of the craniofacial abnormalities may be advocated in the most severe cases of hypertelorism and midfrontal groove, if psychosocial problems occur. Also, patients with major midface underdevelopment might benefit from combined orthodontic/surgical correction.

Adolescent↗

PubMedDate not supplied

Histological observations of teeth and peridental tissues in cleidocranial dysplasia imply increased activity of odontogenic epithelium and abnormal bone remodeling.

Cleidocranial dysplasia (CCD) is a heritable generalized bone dysplasia presenting with a variety of dental abnormalities. To delineate morphological features of the dental tissues, we have analyzed histologically the structure of teeth and peridental tissues obtained from four CCD patients. Dentin appeared regular, except when formed in response to caries, physiological attrition or root resorption. Both acellular and cellular cementum were present in deciduous teeth, whereas in permanent teeth, cellular cementum was virtually lacking and acellular cementum was partially hyperplastic. Enamel pearls were seen in the furcations of one deciduous and one permanent molar. The roots of most deciduous teeth were resorbed only to a slight extent. Morphologically, supernumerary teeth resembled their normal counterparts. Denticles as well as epithelial cell clusters were occasionally present in the root pulps of deciduous teeth. In the peridental tissues, epithelium was locally abundant, and the cytokeratin profile confirmed its odontogenic origin. While the organization pattern of the alveolar bone matrix related to unshed deciduous teeth and unerupted permanent teeth varied, woven bone was abundant. Both formative and resorption surfaces were seen. Also, reversal lines were prominent, suggesting that the bone had, in fact, undergone remodeling. While no direct association between the aberrant bone morphology and the multifarious histological abnormalities of dental tissues can be established, the local abundance of odontogenic epithelium in peridental tissues of developing/unerupted as well as fully developed teeth may be causally related to the formation of excess acellular cementum, enamel pearls and supernumerary teeth.

Adolescent↗

PubMedDate not supplied

Osteogenesis imperfecta: clinical, cephalometric, and biochemical investigations of OI types I, III, and IV.

The aim of the study was to analyze craniofacial development in 54 patients with osteogenesis imperfecta (OI), who were classified into OI types I, III, and IV according to clinical criteria, and to relate the findings to the abnormalities in collagen I production. In 33 patients, analysis of radioactively labelled procollagen was performed. Cephalometric radiographs, facial photographs, and CT-scans (a single case) were analyzed and mean facial diagrams for lateral and frontal films were produced based on registration of 221 reference points. Radiographs of 102 male and 51 female Danish students served as control material. In OI type I, size of the skull and jaws was generally slightly reduced, but morphology was within normal limits. In OI type IV and especially type III more severe abnormalities were found; the cranial base was flattened, the maxilla posteriorly inclined, and nearly all size-measurements were reduced. In OI type III the sagittal jaw relations were reduced and a mandibular overjet recorded. Three OI type I patients, whose fibroblasts produced structurally abnormal collagen I, had the stature and several features in the craniofacial region, which corresponded to those recorded for the OI type IV group. Also, in three OI type IV patients whose fibroblasts produced a reduced amount of normal collagen I, craniofacial morphology showed several features resembling type I patients. We conclude that structural abnormalities of collagen I generally give rise to more severe alterations of the craniofacial features than a quantitative defect of collagen I. OI type I patients are only slightly affected in their craniofacial region, while patients with OI type IV and especially type III are moderately to severely affected. The combined cephalometric and biochemical findings suggest that future classification of patients with osteogenesis imperfecta should be based on biochemical/molecular and radiological analyses in combination with clinical criteria rather than on clinical features alone.

Adolescent↗

PubMedDate not supplied

Final height and craniofacial development after surgical resection of craniopharyngioma.

Seventeen patients (twelve males and five females) with craniopharyngioma were studied by retrospective review (stature, bone age, and hormone therapy) and by follow-up assessment in all seventeen survivors (stature and craniofacial development). Roentgencephalometric films in the lateral and frontal projections were analyzed. Individual and mean facial diagrams were produced based on 221 reference points in the individual patients and compared to normative data. The posterior cranial base was significantly reduced in length and the cranial base angle was significantly increased. In the facial regions great variations in size and prognathy of the jaws were recorded; on average the patients' maxilla and especially the mandible were short and retrognathic in relation to the anterior cranial base when compared to average adults. Average size and shape of the calvaria, cranial base, and facial regions in the adult male craniopharyngioma group corresponded closely to the average male at the stage of maximum growth in body height, i.e., around 14 years of age. It was concluded that size and morphology of the sphenoid and basioccipital bones were severely affected, possibly as a result of the interfering growth of a craniopharyngioma in childhood. The retrusion of the facial regions might be present as a result of the flattening of the posterior cranial base, but the relatively short and retruded mandible could also be caused by growth hormone deficiency before diagnosis/operation and in periods of sub-optimal therapy. The close resemblance of craniofacial morphology between adult males with craniopharyngioma and normal boys at the time of peak height velocity might reflect the fact that imitation of the natural, optimal balance between growth hormone and sex steroid in puberty is difficult to obtain in therapy.

Adolescent↗

PubMedDate not supplied

Dental manifestations of osteogenesis imperfecta and abnormalities of collagen I metabolism.

The in vitro protein-chemical features and the molecular background of osteogenesis imperfecta (OI), a heritable disorder of collagen I metabolism, have been elucidated in recent years. The aim of our study was to find the prevalence of dentinogenesis imperfecta (DI) and other dental anomalies in 88 patients with OI, to compare clinical with radiologic abnormalities, and to correlate these clinical/radiologic findings with the results of gel electrophoresis and molecular studies of collagen I. Twenty-eight percent of OI patients had DI. Most patients with DI had radiologic abnormalities, but some patients had radiologic signs compatible with DI, but no clinical signs of DI. OI type I patients with DI were more severely affected by OI than those without DI. In OI type III and IV, in contrast, there was no difference in overall severity between patients with and without DI. DI was not associated with any particular molecular aberration in any OI type. If defining DI from the presence of both clinical and radiologic signs, collagen I produced by cultured fibroblasts was qualitatively abnormal from all OI patients with DI. Some OI patients had dental abnormalities not resembling DI. A qualitative collagen abnormality could not be found in any of these patients. Denticles, i.e., calcifications within the pulpal cavity, were found more frequently in OI patients than in control subjects.

Adolescent↗