Sedaghatian congenital lethal metaphyseal chondrodysplasia--observations in a second Iranian family and histopathological studies.

In 1980, Sedaghatian described in two brothers and one sister a neonatally lethal disorder associated with slight rhizomelic limb shortness, mild platyspondyly, and severe metaphyseal dysplasia. Here data are presented on another Iranian infant with the Sedaghatian syndrome who died on day 4 and was found to have histologic evidence of severe epimetaphyseal dysplasia. The occurrence in children of both sexes in one instance, born to normal parents who were first cousins, and currently apparent confinement of the disorder to Iranians suggests that the Sedaghatian syndrome is an autosomal recessive trait with high gene frequency in Iranians. This may be a more complexly pleiotropic syndrome than suggested by the roentgenograms, since one of Sedaghatian's patients also had "microphthalmia, asymmetry of ears, depressed nasal bridge, broad nose, short neck, prominent sternum, and short lower extremities."

Changes in serum transaminases, SDH and liver morphology after treatment with trypanocidal diamidines in mice.

The effects of 2 recently developed trypanocidal agents, Diamidinophenylindol (DAPI) and Diimidazolinophenylindol (DIPI) at doses of 10-30 micrograms/g intraperitoneally (i.p.) on serum GOT, GPT and sorbitol dehydrogenase (SDH) levels and on liver morphology have been investigated in mice. Pentamidine served as reference drug. Both agents caused dose-dependent increases in serum transaminases and SDH, and discrete morphological changes of the liver, e.g., fatty degenerations, azinoperipheral vacuolisation and alterations of the nuclei, at least at the higher dosage.

[Cadmium-induced vertebral-column ankylosis in whitefish ]. / Cadmium-induzierte Wirbelsäulenankylose beim Blaufelchen.

In two healthy and two diseased whitefish (Coregonus Wartmanni) taken from Lake Constance (FRG), ankylosis of the vertebral column was investigated both roentgenologically and histologically. Subsequent to the collapse and necrosis of the "residual" spinal cord within the intervertebral spaces, the outside edges of the vertebral bodies come into direct contact. The compression and tensile forces that occur to an increased extent as a result of the instability, lead not only to a remodelling of the vertebral bodies, but also to the formation of spondylotic osteophytes at the edges of the vertebrae and, as a result of periosteal stimulation, to the development of cellular hyaline cartilage, which fills the intervertebral spaces. Finally, as a result of perichondral ossification, a bony ankylosis develops. The humping of the spine of the fish due to the stiffening and shortening of the vertebral column, is accompanied by a restriction in the animal's freedom of movement. Muscular atrophic processes and disordered food uptake give rise to poor growth and a reduction in the weight of the diseased fish. These remodelling processes in the spine resulting from instability are specific to the periosteum and may be equated with the changes seen in man in spondylosis deformans. The possible cause of this vertebral column ankylosis is cadmium poisoning. The accumulation of this heavy metal obviously leads primarily to an irreversible toxic degeneration of the cells of the chorda dorsalis.

[The solvent-dried dura mater. A new dura transplant in the animal experiment]. / Lösungsmittelgetrocknete Dura mater. Ein neues Dura-Transplantat im Tierversuch.

After the excision of 7 X 5 mm abdominal muscle sections in an experiment using rats, rehydrated, solvent dried dura grafts of the same size were implanted. After three hours the tissue reaction of the transplant areas were continuously examined under a light and electron microscope for 14 months. A gradual graft decomposition advancing in a coating form from the periphery to the center and a simultaneous replacement with collagenous connective tissue of the body resulted due to an absorbent granulation tissue rich in cells and blood vessels after the initial infiltration of neutrophil granular leukocytes caused by the operation. A fibrous-rich connective tissue plate, in part forming two layers and thereby serving as a reminder of the former dura graft is decomposed in the abdominal muscles of the rats without infection or rejection and replaced with endogenous connective tissue. The duration of this decomposition and transformation process is contingent on the size of the graft and the tissue reactivity in the surrounding transplant area.

[Relevance of predamage in fibulotalar ligament rupture. Clinical and histological studies]. / Zur Relevanz von Vorschäden bei fibulotalaren Bandrupturen. Klinisch-histologische Untersuchungen.

In 31 patients with a recent rupture of the fibulotalar ligament, the aetiology and the significance of prior traumatization were investigated clinically and histologically. History. All the patients reported that they had twisted the injured foot either once or on a number of occasions. Only five patients were able to identify a concrete accident. Intraoperatively, the anterior fibulotalar ligament showed scarring in addition to fresh rupture. Histologically, excisional biopsies (EB) obtained during surgery, also revealed fresh lesions affecting the collagenous tissue as well as granulation and/or scar tissue of varying age. Pre-traumatization of the fibulotalar ligament occurs as a result of simple twisting of the foot. This apparently insignificant trauma leads to microlesions which although they induce reparative processes that can be demonstrated histologically, produce a relevant diminishment of the resistance of the ligament to rupture. Thus, ligament rupture can readily be explained by the interaction of prior traumatization and a violent force. In comparison, prior degenerative lesions have no relevance. In the individual case, therefore, in addition to prior history, the nature of any accident and intraoperative findings, a histological examination should also be performed for an epicritical assessment of the aetiopathogenesis.

[Aging bones as a paradigm for individual differences in aging]. / Der Altersknochen als Paradigma für die Individualität des Alterns.

The physiological fate of every organism and its organ systems is aging which--as general opinion has it--after the age of 50 leads to a measurable mass of bone loss in the human skeletal system. This so-called physiological osteoporosis, however, affects the individual bones differently: Thus, the volumetric density in the neck of the femur and in the lower three lumbar vertebral bodies (LVB) decreases by 30%, but not in the lower three cervical vertebral bodies (CVB), while in the thyroid cartilage it increases constantly from the age of 15/20 years onwards. From the functional point of view, the so-called osteoporosis of old age is thus an expression of an aging bone adapting to the physical activity of the body, which, as such--just like the bone of the younger adult--merely reflects the current stressing of the cancellous bone by the locomotor system. Osteoporosis proper, in contrast, is a manifestation of disease of the bone causing clinical symptoms, in which underlying osteopenia results in fractures and spontaneous deformations.

[Ossification principle of the laryngeal skeleton of the human and mammals. Comparative anatomic studies]. / Zum Ossifikationsprinzip des Kehlkopfskelets von Mensch und Säugetieren. Vergleichende anatomische Untersuchungen.

In 3 oxen, 24 deer, 40 common hares and 27 rabbits, the laryngeal cartilages, freed from soft tissue, were examined roentgenologically for ossification, and compared with the well-known ossification pattern of the human thyroid cartilage. In the human, the thyroid cartilage ossifies in four preferential directions: horizontal-caudal, vertical-lateral, vertical-median, and obliquely tongue-shaped; in cattle ossification in the three directions: horizontal-caudal, vertical-median, and vertical-lateral; and in the deer in two directions: median-concentric and vertical-lateral. The cricoid cartilage in cattle and deer ossifies at the lamina and the upper border of the arch, the arytenoid cartilages ossify at the muscular process. In the common hare the thyroid cartilage ossifies only rarely, the other cartilages, in common with the entire laryngeal skeleton of the rabbit, never. The cause of ossification of the laryngeal cartilages is their deformation by the muscles of the larynx. The differences in the ossification patterns in humans, cattle and deer are due to the species-specific mechanical stress determined by the respective anatomy. The absence of ossification in the common hare and rabbit, on the other hand, is an expression of too small a deformational stress of their laryngeal skeleton. Ossification and nonossification are examples of the same principle, the self-regulating adaptation of connective and supportive tissues to mechanical stresses.

[Autoclaved bone grafts in prosthesis replacement of the hip]. / Autoklavierte Knochenspäne beim Prothesenwechsel an der Hüfte.

Bone defects of the acetabulum caused by loosening of the socket of a total hip prosthesis can be filled in with autoclaved homologous cancellous bone grafts. Five months after surgery a radiological change in the bony structure is visible. Histological examination shows that new bone is formed around the autoclaved bone.

[Endoscopic-histological differential diagnosis of rare benign endolaryngeal proliferations (author's transl)]. / Endoskopisch-histomorphologische Differentialdiagnose seltener gutartiger Proliferationen des Endolarynx

In the period from 1961 to August, 1975, 2,222 microlaryngoscopic examinations were carried out at the ENT Department of the University. A correlation was seen between the increase in the number of examination in the last few years and the increase in the number of malignant tumours of the larynx, while malignant growths in the hypopharynx were found to be only slightly increased. An anlysis of 1,027 microlaryngoscopies carried out under anaesthesia over the last 2 2/3 years showed almost 50% cancers and precancers. In 5% of the case at most more rare laryngeal findings were also seen. Of these, the granular cell tumour, plasmocytoma, oncocytic cystadenoma and amyloid-tumour of the larynx, but also tuberculous laryngitis, are represented. The modern endoscopic examinations and the fundamental bioptic histomorphological examination of proliferations allow an exact differentiation of these changes in the tissues of the endolarynx. In this connection, a valuable technical aid is the v. Stuckard low-power magnifying laryngoscope, that has been in use for the past six months.

[The structure of the spongy bone in lumbar vertebrae and the neck of the femur. A comparative analysis of the age-dependent remodelling process (author's transl)]. / Spongiosastruktur von Lendenwirbelkörpern und Schenkelhals Vergleichende Analyse des altersabhngigen Umbaues.

In 114 autopsy cases of both sexes, aged between 31 and 97 years and without bone disease, the influence of mechanical forces on the age-dependent remodelling of the spongy bone was determined in the 3rd and 5th lumbar vertebrae and the neck of the femur. For this purpose contrasty X-ray images of 100 mu thick polished bone sections were analysed using the LEITZ texture analysis system. The volumetric density and the surface density are highest in the neck of the femur, lowest in the 3rd lumbar vertebra, and almost as low in the 5th lumbar vertebra. The volumetric density decreases with increasing age by about one-third in all three bones. Correspondingly, the surface density also decreases in the lumbar vertebrae by one-third, but only by 18% in the neck of the femur. The specific surface reveals no age differences in the lumbar vertebrae, but increases by 19% in the neck of the femur. The constancy of the specific surface in the lumbar vertebrae can be explained by the fact that compensatory hypertrophy of the remaining trabeculae takes place. This compensatory growth of bone does not occur in the neck of the femur. The age-dependent decrease in spongy bone manifests itself in a residual spongiosa structure that depends on compressive forces in the case of the lumbar vertebrae and bending forces in the neck of the femur.

[Animal studies of the effects of distal occlusion on the TMJ]. / Tierexperimentelle Untersuchungen über den Einfluss des Distalbisses auf die Kiefergelenke.

In four adult monks of the "macaca fascicularis" genus we surgically shifted the mandible posteriorly by 5 mm in three animals and by 10 mm in one animal. After 83, 172, 273 and 354 experimental days, the animals were killed and the TM joints were prepared for patho-histological examination. Histomorphological changes could be observed above all in spongy and cortical bone situated close to the TMJ. There were no striking pathological findings, however, in condylar cartilage and the articular disk.

Structural changes of collagen fibrils in skeletal dysplasias. Ultrastructural findings in the iliac crest.

The skeletal dysplasias are constitutional, generalized or localized disorders of the skeletal system involving a disturbance of growth and/or bone density; their genetic transmission varies. Pathomorphologically, a combined functional-structural disturbance of the cartilaginous and/or bone tissue is present. Clinically, the result is varying degrees of dwarfism. Within the framework of a systematic examination of skeletal dysplasias, a total of 84 iliac crest specimens/biopsies obtained from stillborn infants and patients varying in age from a few days to 40 years, were investigated in the electron microscope. The sections prepared extended from the perichondrium through the proximal resting zone to the primary mineralization zone. The structure of the collagen fibrils was studied in diastrophic dysplasia, pseudoachondroplasia, the WOLCOTT-RALLISON syndrome, osteogenesis imperfecta, and idiopathic juvenile osteoporosis. In diastrophic dysplasia, pseudoachondroplasia and idiopathic osteoporosis, the cartilaginous ground substance contains collagen fibrils that can vary considerably in length, structure and diameter. In one case of WOLCOTT-RALLISON syndrome, the lacunae of the chondrocytes are found to contain very wide amianthoid-like and inadequately aggregated collagen fibrils. In numerous cases, osteogenesis imperfecta reveals very fine and also irregularly structured collagen fibrils with scarcely discernible cross-striation in the region of the osteoid, which is of varying width. In some of the cases, catechin has a favourable effect on the formation of collagen fibrils, resulting in broader and more densely packed fibrils. In addition, the conditions are associated with considerable intracellular changes in the rough endoplasmic reticulum, the Golgi apparatus and the mitochondria. The varying collagen fibril findings in the cartilage and bone tissue also represent a morphological marker of the combined functional-structural disorder of chondrocytes and/or osteoblasts, and an expression of the differing aetiopathogenesis.

[Long-term behavior of aluminumoxide ceramic implantations in the region of pneumatized cavities in animal experiments]. / Langzeitverhalten von Aluminiumoxidkeramik-Implantaten im Bereich von pneumatisierten Höhlen im Tierexperiment.

After bilateral resection of the facial wall of the maxillary sinus of five Göttingen minipigs and coverage of the defect with perforated Al2O3 ceramic material, the initial response to the implant was examined clinically and histomorphologically for periods of up to eight months. Except for a wound infection in the initial stages of healing in the case of one animal, no complications were observed after the operation. Three months after the operation, the implants were fixed in the connective tissue. After the fourth month, newly formed compact bone had developed and stabilized in the region of the attachment. The basically uncovered surface of the implant, which was turned toward the maxillary sinus, was covered with a non-irritating respiratory mucous membrane. No immunologic responses were observed. Bio-inert reaction, stable tissue-bony anchorage, and coverage with functioning mucous membrane justify the insertion of Al2O3 ceramic implants to cover defects in pneumatized cavities.

[General partial resection of the mandible in para-osteal cancers of the mouth and oropharynx]. / Zur Frage der generellen Unterkieferteilresektion bei knochennah gelegenen Karzinomen der Mundhöhle und des Oropharynx.

The mandibles of 24 patients with a squamous cell carcinoma of the floor of the mouth or the tongue or the tonsils (all were T2N1b and T3N1b), were histologically examined. It could histologically be shown, that the bone will be destroyed by 2 modalities: 1. Atrophy of the mandible, caused by the pressure of the tumor, leaving the periosteum intact 2. Infiltration of the tumor into the bone. Tumor cells could not be found in the lymphatic system of the periosteum. Since the periosteum is a barrier against tumor growth, the indicator for the resection of the mandible was made after shaved off the periosteum. Resulting from our findings we propose the following concept: 1. If the tumor reaches the mandible (distance tumor to bone = 0 mm) or is fixed to the periosteum and the compacta shows no errosion, after shaving off the periosteum, the mandible can be preserved. 2. If the tumor grows through the periosteum, a marginal resection of the mandible is necessary. 3. If the bone shows errosion, a partial resection of the anterior part of the mandible or a hemimandibulectomy is indicated.

[Animal experimental studies on the healing around ceramic implantation in bone lesions in the maxillary sinus region]. / Tierexperimentelle Untersuchungen über die Einheilung von Keramikimplantaten bei Knochendefekten im Kieferhöhlenbereich

Clinical and histological tests were made in five minipigs to study the hitherto unknown tissue behaviour of the receiver organism to unilaterally uncovered perforated ceramic implants in the bilaterally osteotomized facial maxillary sinus wall. The implants were covered with connective tissue and coated with respiratory mucosa on the uncovered side. After 11 weeks, they had all healed without irritation, and were solidly anchored in the surrounding tissue by the connective tissue which had grown into the perforations.