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Results 401 - 450 of 471
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Pagliuca A - - 1989
We describe 10 cases of primary myelodysplastic syndrome in which marrow fibrosis was striking at presentation. All the cases showed trilineage dysplasia with increased megakaryopoiesis and marked reticulin fibrosis. Significant organomegaly was notably absent. This association has hitherto not been highlighted and it is important to distinguish these cases from ...
Crawford P J - - 1989
Rapp-Hodgkin syndrome is a rare form of ectodermal dysplasia involving the hair, eyes, sweat glands, nails, teeth, and palate. The case of a white girl with the condition is presented. The differential diagnosis is discussed, and the eight previously reported cases are reviewed. Another (ninth) previously reported case is considered ...
Barat M - - 1989
Fibrous dysplasia is a rare disease of unknown cause that affects one or multiple bones. In its monostotic form, only one bone is involved. The maxilla is the most commonly affected facial bone. Facial deformity with or without pain is the most common presentation of fibrous dysplasia affecting the craniofacial ...
Bacha L - - 1989
A new, dominantly inherited form of peripheral dysplasia in three members of an Algerian family is reported. Two further individuals, not investigated, are also probably affected. The disease is characterised by distinctive clinico-radiological findings. A case of acromesomelic dysplasia of Maroteaux is also reported and the differential diagnosis of peripheral ...
Loomer R L - - 1989
Hereditary oncycho-osteodysplasia is a well-defined clinical entity consisting of four characteristics: (1) complete or partial absence of finger and thumb nails typically more severe on the radial side of the hand; (2) bony dysplasia about the knee consisting of patellar and lateral femoral condylar hypoplasia; (3) bony dysplasia about the ...
Schajowicz F - - 1989
Three cases of adamantinoma of the tibia associated with and mimicking fibrous dysplasia or osteofibrous dysplasia are reported in children aged three, nine, and 16 years. The roentgenographic features were typical of intracortical fibrous dysplasia (osteofibrous dysplasia). These entities are not two distinct diseases, but rather are different histologic expressions ...
Perlman M D - - 1988
An unusual case of enchondroma located in the distal phalanx of the left third toe is presented with a review of literature. An enchondroma is a benign tumor centrally located in a bone. This osseous dysplasia is characterized by an excess of mature hypertrophic hyaline cartilage that has not resorbed ...
Gembruch U - - 1988
In a pregnant woman without increased genetic risk, the presence of distrophic dysplasia of the fetus was diagnosed sonographically at 31 weeks' gestation and definitively distinguished from other skeletal dysplasias. In all prenatal diagnosis of diastrophic dysplasia reported so far, this autosomal recessive congenital condition had occurred in the family's ...
Meredith J T - - 1988
An unusual case of a progressive, noninflammatory stenosing vasculopathy, arterial fibromuscular dysplasia is presented. The distinctive features of this particular case include onset in early childhood with a predominant involvement of the gastrointestinal system, sparing of the renal arteries, lack of hypertension, and no cutaneous features of progressive systemic sclerosis. ...
Zain R B - - 1988
This is a case report of a reactive lesion which occurs only in the gingiva. This lesion is of a long duration causing erosion of the superficial bone. Histologically it is similar to other epulides of the gingiva but in addition contains ossified tissue. The lesion's confusing terminology is being ...
Herold H Z - - 1988
Details of ten new cases of spondylothoracic dysplasia (the Jarcho-Levin syndrome) are presented. During follow-up, lasting between 8 and 21 years, two children in this group died. Our observations did not confirm Heilbronner's theory concerning the existence of two distinctive types of this syndrome: Type 1 or fatal, and Type ...
Wroble R R - - 1988
Histiocytosis X can exhibit a variety of musculoskeletal manifestations. Nonetheless, this case exhibits two striking features. Severe progressive scoliosis and massive osteolysis of both femoral shafts occurred in conjunction with the Hand-Schüller-Christian form of histiocytosis X. The scoliosis was treated successfully by posterior fusion and instrumentation. The clinical features of ...
Hammill W W - - 1988
Hidrotic ectodermal dysplasia represents a group of congenital or hereditary disorders that involve ectodermal derivatives. It is characterized by partial or complete alopecia, dystrophic nails, and dental abnormalities. Dilated cardiomyopathy has not previously been reported in association with this illness. We report the cases of three children with fatal dilated ...
Scott R W - - 1988
Cleidocranial dysplasia, a rare congenital disorder characterized by dysplasia, and even more rarely, by aplasia of the clavicles, largely goes unrecognized because patients with the condition seldom display functional limitation, pain, or other symptoms. The purposes of this article include describing aspects of cleidocranial dysplasia pertinent to physical therapists and ...
Castellote A - - 1988
Two cases of osteofibrous dysplasia of long bones are reported, one in a neonate with ipsilateral tibial and fibular involvement and the other in a 6-year-old patient with bilateral tibial lesions. The radiological features of the younger patient were remarkably different from those of the older one. These two different ...
Thomas R L - - 1987
Two cases of skeletal dysplasia with limb-shortening abnormalities are presented. In association with the skeletal abnormalities, gross hydramnios was noted. A review of the existing literature reveals that coexistent fetal skeletal dysplasia and hydramnios have an extremely poor prognosis, especially in the nonachondroplastic patient with singleton fetus. The two cases ...
Chang H H - - 1987
Benign fibrous polyps of the ureter are rare. A review of the English literature since 1930 revealed that 71 cases had been described. Three additional cases of fibrous ureteral polyps, one in a ureter with ureteritis cystica, are reported with a discussion of both the histologic findings and proposed treatment ...
Perlman M D - - 1987
The unusual case of fibrous dysplasia located in the distal phalanx of the hallux is presented with a review of literature. Fibrous dysplasia is a benign disorder that can affect one or many bones in any particular case. The bone involvement is characterized by the presence of fibro-osseous tissue within ...
Aleck K A - - 1987
The dyssegmental dysplasias are lethal forms of neonatal short-limbed dwarfism in which vertebral segmentation defects and short, thick, bowed long bones are the prominent radiographic features. Clinically, unusual facies, short neck, narrow thorax, cleft palate, and reduced joint mobility are commonly seen. To date, 18 cases of dyssegmental dysplasia have ...
Scuderi G - - 1987
Acetabular fractures in children are not uncommon, but traumatic disruption of the acetabular triradiate cartilage occurs infrequently and may be associated with acetabular dysplasia and subluxation of the hip. The triradiate cartilage is at risk from birth to closure. The earlier the age at the time of injury, the greater ...
Biagini R - - 1987
The authors report a case of Mazabraud's syndrome in a patient aged 57 years with a history of fibrous dysplasia of the skeletal system which had commenced at the age of 10. This became associated with myxomas of the soft tissues at the age of 42. This rare association was ...
Gilinsky N H - - 1986
Case reports of three teenage patients with nonfamilial diffuse juvenile polyposis are presented to illustrate the considerable morbidity and significant growth retardation that may occur. Although generally considered to have no malignant potential, histologic findings revealed features ranging from adenomatous change to severe dysplasia. It is suggested that a conservative ...
Borochowitz Z - - 1986
Recently hypochondrogenesis was described as a form of neonatally lethal dwarfism said to resemble spondyloepiphyseal dysplasia congenita radiographically and achondrogenesis II morphologically. Because of the difficulty in distinguishing radiographically between mild achondrogenesis II and severe hypochondrogenesis, we performed a clinical, radiographic, and morphologic study of 24 cases originally classified as ...
Rosati L A - - 1986
The histological and immunohistochemical features of 3 cases of cellular neurothekeomas of skin are presented. Based on the findings in these cases and comparisons made with others reported in the literature, it is proposed that neurothekeomas may be divided into two distinct types: the myxoid and cellular variants. The relationship ...
Cupceancu B - - 1985
In order to avoid secondary induction of estrogens caused by tamoxifen treatment, tamoxifen was associated with the progestative lynestrenol in 48 menstrual women with benign breast disease (adenoma or fibroadenoma, cystic, simple or complex dysplasia). In one variant tamoxifen dosage was 20 mg (2 tablets) daily taken orally from day ...
Gropper P T - - 1985
A case of monostotic fibrous dysplasia of a long bone of the hand is reported. There has been only one similar case recorded, though it is not uncommon in other long bones. The diagnosis was proved by histology, and the tumour treated by subperiosteal excision. There has been no recurrence ...
Silverman F N - - 1985
Eight patients are presented who have a generalized bone dysplasia that resembles severe cleidocranial dysplasia but lacks the cranial and clavicular features of that well-defined condition. Vertebral-body ossification is markedly defective, and ossification centers of the tubular bones are grossly enlarged. These patients also differ from those with variant forms ...
Wilhelmsson B - - 1985
The histological study of the non-tumours nasal mucosa in 22 wood-workers with ethmoidal adenocarcinoma was carried out and special attention was paid to the presence of cuboidal metaplasia with or without dysplasia. The workers had been exposed to wood dust for an average of 38 years (range 18 to 55 ...
Hiraide F - - 1985
Three cases of lymphangiectatic fibrous polyp of the palatine tonsil are reported. The polyp arose from the upper pole of the tonsil in two cases and from the middle-to-lower part in one case. They were all asymptomatic until the polyps grew larger. The polyps were removed, with or without the ...
Muller L M - - 1985
Reports on prenatal diagnosis in cases of skeletal dysplasia have mostly been in high-risk mothers with a suspect genetic background where the fetal lesion could probably be predetermined. We deal with routine ultrasonographic appraisal of the fetal skeleton when dysplasia is not initially suspected, and relate our experience of the ...
Schmidt J M - - 1985
A histological study was made to determine the cochlear neuronal populations of 20 human ears having hearing loss caused by developmental defects. The neuronal populations ranged from 7677 in an ear with Mondini dysplasia to 30 753 in an ear with DiGeorge's syndrome, the norm for young human subjects being ...
Fasanelli S - - 1985
Two cases of dyssegmental dysplasia, a rare lethal skeletal dysplasia in the newborn, are reported. Two different forms of dyssegmental dysplasia can be distinguished--the lethal Silverman type and the less severe Rolland-Desbuquois type. In this report, both cases are of the Silverman type. Histopathology of chondro-osseous tissue confirms the similarity ...
Halawa M - - 1984
Malignant transformation of fibrous dysplasia to chondrosarcoma is rare. We report a case in which malignancy developed in an area of fibrous dysplasia in the ilium. We believe this to be the second reported case at this site. Treatment was by excision of the hemipelvis including the ala of the ...
Cruz-Conde R - - 1984
A new case of dysplasia epiphysealis hemimelica is reported. It is the third case reported in Spain and presents involvement of almost all the epiphyses of the left side of the body. The involvement of the left hip following surgery is hard to explain. The patient also presents lengthening of ...
Akagi G - - 1984
Liver tissues of 223 autopsy cases of cirrhosis and hepatocellular carcinoma were examined for liver cell dysplasia in relation to hepatitis B surface antigen (HBsAg) detected with orcein stain. Liver cell dysplasia was found in 94 cases (42.2%): 37 were from cases of cirrhosis only, and 53 were from cases ...
Keng S B - - 1984
A case report of Hypohidrotic Ectodermal Dysplasia is presented. The oro-facial features are described together with the dental management of the condition. Children who have this condition should be treated early for their prosthodontic needs to aid mastication and appearance. Continued review and replacement of dentures provided should be instituted ...
Hall M B - - 1984
To our knowledge, this is the only reported case of Albright's syndrome with reactivation of the fibrous dysplasia in adulthood, a marked elevation of growth hormone secondary to a pituitary adenoma, and development of osteosarcoma within the reactivated fibrous dysplasia. In addition, this patient also had hyperthyroidism with normal TSH ...
Friedman L - - 1984
A case of lipoid proteinosis in an 18-year-old youth is presented. The typical clinical findings in patients with this rare disorder are discussed. Emphasis is placed on the radiographic and computed tomographic appearances. The bean-shaped calcific densities, which are located in the region of the hippocampi in this case, are ...
Tanaka T - - 1984
Two autopsy cases of thanatophoric dysplasia in male newborn infant are described. They have markedly short-limbs and narrow thoraxes. Histologically, generalized disruption of endochondral ossification with normal resting cartilage was seen at the epiphyseal region of femur. Differential diagnosis from other 3 types of lethal chondrodysplasia is described and the ...
Kozlowski K - - 1984
Fourteen patients with spondylo-costal dysplasia were analysed. 3 of them presented without obvious associated anomalies "pure" spondylo-costal dysplasias; 2 had several components consistent with Vater (Vacterl)-Association; 2 showed malformations which are often encountered in Vater (Vacterl)-Association; 4 presented with minor malformations; 3 had major associated malformations rarely seen in Vater ...
Olow-Nordenram M A - - 1984
Forty-three patients with maxillo-nasal dysplasia have been subjected to a radiographic examination of the cervical spine. In 44.2 per cent malformations of the cervical vertebrae of a minor or major type were revealed. Dysplasia of the vertebral bodies related to persistence of the chorda dorsalis, a very rare malformation, was ...
Wells M D - - 1983
A case of oculo-auriculo-vertebral dysplasia is presented, with histopathological findings in the temporal bone and eye, and some radiological features. The syndrome consists of anomalies of the first and second branchial arch derivatives associated with ophthalmic and vertebral anomalies. Although clinical features and radiological findings of oculo-auriculo-vertebral dysplasia are documented ...
Sondheimer S J - - 1983
To evaluate mild cervical dysplasia, the results of Papanicolaou smears were reviewed for a six-month period in an inner-city family planning program. Papanicolaou smear evidence of mild dysplasia was consistent with the biopsy diagnosis in most cases. A single Papanicolaou smear is not a reliable indication of condylomata, nor is ...
Langer L O LO - - 1983
We have studied a male Japanese infant with severe upper limb brachymesomelia, glomerulocystic renal dysplasia, abnormalities of the cranium and face, corneal opacities, and a possible congenital heart defect. He was born at term and died on the 10th day of heart and kidney failure. Review of the literature failed ...
Wetherington R K - - 1983
The form of epiphyseal-diaphyseal dysostosis in the phalanges of the hand having the appearance of a cone-and-crater (cone-shaped epiphysis) is a common variant in otherwise normal individuals. Dysplasia on the radial margins of the epiphysis and diaphysis, typically resulting in clinodactyly, is another variant and is also common in Down's ...
Kremens B - - 1982
The radiologic and morphologic features of thanatophoric dysplasia with cloverleaf-skull could be demonstrated in a neonate who died from asphyxia shortly after birth. Occipital bone hemangiomatosis, a typical histomorphologic finding in cloverleaf-skull, is reported for the first time in a thanatophoric dwarf. Our investigations and the discussion of 26 observations ...
Yaguchi T - - 1982
A case of Peutz-Jeghers syndrome in which one colonic polyp showed many foci of glandular dysplasia is reported. Peutz-Jeghers polyps are considered to be hamartomatous, and their malignant potential has been considered doubtful. This case is valuable because foci of glandular dysplasia existed within hyperplastic glands. Glands of Peutz-Jeghers polyps ...
Sillence D O - - 1982
We report two sporadic cases with a previously undescribed skeletal dysplasia lethal in the neonatal period. The syndrome is characterized clinically by striking rhizomelic shortness of the limbs and radiographically by absence or hypoplasia of the humeri, hypoplastic vertebrae, absent fibulae and ossification in only the distal phalanges of the ...
Ogden J A - - 1982
While accessory ossicles are relatively common in the feet, major developmental chondro-osseous variations in otherwise normal feet appear to be extremely unusual. The "bifid os calcis" has been reported infrequently since its first description by Sever in 1930. The majority of cases are fortuitously diagnosed, usually in children three years ...
Hall C M - - 1981
A child with Werner's mesomelic dysplasia is presented. She has five fingers on each hand with absent thumbs and seven toes on each foot. There is posterior dislocation at the knees and mild tibial dysplasia. The child also has a ventricular septal defect (VSD) and Hirschsprung's disease. This is the ...
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