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Ultrasonography (US) enables accurate assessment of the cartilage cap of exostoses. The cartilage cap appears as a hypoechoic layer covering the hyperechoic surface of the calcified part. Measurements of cap thickness with US were compared with measurements performed on pathological specimens in 22 resected exostoses and 2 exostotic chondrosarcomas. The US measurements proved to be very accurate, with a mean measurement error of less than 2 mm for cartilage caps less than 2 cm thick. The detection rate and measurement accuracy of US were higher than with computed tomography (CT) and comparable to magnetic resonance imaging (MRI), which were available in 14 and 10 cases, respectively. US appears to be a good procedure for evaluating the cartilage cap, which is usually thin for a benign exostosis and thick for a malignancy. In addition, other complications--such as bursa formation--are easily recognizable. The sole limitation is that US cannot visualize the cartilage cap when it is inwardly orientated or deeply located in soft tissues, which are both, however, relatively uncommon situations.

We report the cytogenetic results in three cases of B-cell non-Hodgkin's lymphomas with morphologic and immunophenotypic features compatible with a non-follicle center cell lymphoma. In all cases, a chromosome breakpoint at 14q32 and structural abnormalities of 1p were found. Increased copies of chromosome segment 12q and structural rearrangements of chromosome 6 were found in two cases. Translocation (14;18)(q32;q21) with rearrangement of bcl-2 was found in one case. These lymphomas have a perifollicular growth pattern and IgM+, IgD+, CD10-, and CD22+ immunophenotype features typical of non-follicle center cell lymphomas and probably belong to the follicle mantle lymphomas described recently. Little cytogenetic data about this group of lymphomas is available, possibly because in the Working Formulation for the Classification of Lymphomas they are not separated from follicle center cell lymphomas.

A case of plasma cell granuloma of the lung is presented, and the clinical, radiological, histological, diagnostic and therapeutic data obtained from 97 published cases are reviewed. Since the disease is usually peripheral, the diagnosis rests on surgical biopsy and histological examination. In the case presented here, it was established on intrabronchial biopsy obtained by fibroscopy.

The phospholipid requirement for the optimal solubilization of carnitine acylcarnitine translocase from the inner membrane vesicles of rat liver mitochondria and for its reconstitution in liposomes was investigated. At the octylglucoside-solubilization step, the presence of cardiolipin proved superior to the other lipids tested. For reconstitution, a mixture having phosphatidylcholine, phosphatidylethanolamine and cardiolipin was found to be particularly effective. The requirement of cardiolipin at this step was met less effectively by other anionic phospholipids. Moreover, in intact mitochondria of rat liver and heart, the translocase activity was markedly inhibited by micromolar concentrations of doxorubicin, a specific cardiolipin-binding agent.

The histological alterations seen in HIV-related lymphadenopathy have been described with different terms by different authors. In order to facilitate comparisons of results from various laboratories, a group of pathologists of the European Lymphoma Study Group (later the European Association for Haematopathology) have proposed a histological classification for the evaluation of HIV related lymphadenopathy. Most observers agree that the morphological and immunohistochemical alterations in the follicles (germinal centres) are the most conspicuous and earliest changes seen. The follicular alterations were therefore used as the basis for the proposed classification. In addition, some features occasionally seen were also included, i.e. angioimmunoblastic hyperplasia, multicentric Castleman-like lesions, and vascular lesions, especially pretumorous and tumorous stages of Kaposi's sarcoma. The proposed classification was used by the members of the group of a collection of lymph nodes compiled from the various centres. The classification was shown to be reproducible and to have clinical relevance in staging of the patients. The prognostic relevance as to the survival of the patients has to be further substantiated by follow-up studies. The proposed classification offers a common terminology for the alterations previously described by different terms by various authors.

Four hundred and two cases considered or suspected to be lymphoepithelioid cell lymphoma (Lennert's lymphoma) were analyzed morphologically, immunohistochemically, and clinically. One hundred and eight of these cases, investigated in 180 biopsies, fulfilled the morphological and immunohistochemical criteria for lymphoepithelioid cell lymphoma and are herein reported. Cellular composition and histological structure are described in detail as a basis for discriminating Lennert's lymphoma from similar lymphomas with a high content of epithelioid cells (Hodgkin's disease, AILD type of T-cell lymphoma, lymphoplasmacytic lymphoma) and from inflammatory epithelioid cell reactions. Single typical Hodgkin cells were found in only 3.8% of biopsy specimens examined, and single typical Sternberg-Reed cells were found in only 2.2% of the biopsy specimens examined. Because these single Hodgkin and Sternberg-Reed cells were situated in a relatively monotonous lymphoid cellular pattern and because both these cells types also may occur in peripheral T-cell lymphomas, we include cases with such cells in the category of Lennert's lymphoma. Eight percent of the patients with lymphoepithelioid cell lymphoma showed transformation into a large-cell T-cell lymphoma without the prominent focal epithelioid cell component previously observed. Immunohistochemically, seven of the 69 biopsy specimens with detectable giant cells stained positively for the granulocyte-specific monoclonal antibody 3C4 (approximately CD15). Plasma cells were rare and always showed a polytypic immunoglobulin pattern. Lymphoepithelioid cell lymphoma, defined as a lymphoma of CD4+ T lymphocytes, marks the border between Hodgkin's disease and the non-Hodgkin's lymphomas. Today, absolute criteria for distinguishing between these two classes of lymphoma are lacking.

A method for the determination of picomole quantities of gamma-butyrobetaine and its application for the determination of gamma-butyrobetaine distribution in tissues are described. The method is based on the quantitative conversion of gamma-butyrobetaine into carnitine by using a 50-60%-satd.-(NH4)2SO4 fraction of rat liver supernatant as the source of gamma-butyrobetaine hydroxylase [4-trimethylaminobutyrate,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating), EC 1.14.11.1]; the carnitine formed is then measured enzymically. The mean gamma-butyrobetaine content, as nmol/g wet wt. of tissue, ranged from a low of 4.6 in livers to a high of 12.3 in hearts of normal fed male adult rats. Starvation for 48 h did not affect the gamma-butyrobetaine concentration in serum, liver and brain, but that in skeletal muscles, kidney and heart was increased. These data are in line with the present views that most tissues are able to produce gamma-butyrobetaine, and show that starvation enhances the synthesis and/or the retention of this compound in many tissues. The observed high affinity of gamma-butyrobetaine hydroxylase for gamma-butyrobetaine (Km 7 microM), the high activity of this enzyme and the low concentration of gamma-butyrobetaine in liver indicate that gamma-butyrobetaine availability is one of the factors that normally limit carnitine synthesis.

The photoreaction center from Rhodospirillum rubrum contains about 90% protein, 6% pigment, mere traces of lipids, and no cytochromes. It also contains at least 1 mol of ubiquinone and 1 iron atom per mol. Its three-component polypeptide chains were isolated by preparative electrophoresis, and their molar stoichiometry was established as 1:1:1. The amino acid composition of the photoreaction center from strain S1 and from its subunits is reported. The protein as a whole contains about 65% nonpolar residues, and the degree of hydrophobicity of its subunits is alpha less than beta less than gamma. The minimal molecular weight based on the extinction coefficient and on the amino acid content is 90 000. This corresponds to a half-cystine mole number of 6.

Carnitine acylcarnitine translocase has been solubilized from inverted inner membrane vesicles of rat liver mitochondria with octyl glucoside and reconstituted into asolectin liposomes. For both processes, optimization of the detergent to phospholipid ratio was found crucial for obtaining reconstitutively active liposomes. Reassembly of the solubilized carrier into asolectin liposomes was achieved either by the octyl glucoside dilution method or by Extracti-Gel D column chromatography. The reconstituted system catalyzed exchange diffusion of carnitine, exhibited the expected inhibitor and temperature sensitivity, and discriminated between stereoisomers of octanoylcarnitine. The activity of unidirectional import of carnitine was low compared to exchange diffusion. It showed high-temperature sensitivity and a loss of activity on prolonged sonication that was regained by an appropriate freeze-thaw step subsequently.

We have observed three cases of carpal tunnel syndrome (CTS) complicated by cutaneous ulcerations and acroosteolysis. Although our first patient had a mild case of CTS, he had a concomitant amphetamine addition. This led to a mania for gnawing away at the digits in the territory of the median nerve. Our other two patients had severe cases of CTS. One suffered from diabetes and the other from primary amyloidosis. Surgical decompression of the median nerve was carried out in these two cases and led to rapid healing of the cutaneous lesions. The latter patients suffered from the entity known in the french literature as carpal tunnel "ulcero-mutilating" (ulcero-osteolytic CTS). This paper discusses possible mechanisms for its pathogenesis. In one of our cases, for example, we were struck by the role played by the patient's behavioral habits whether it be conscious or subconscious in the self-perpetration of the cutaneous and osseous lesions.

Rates of carnitine palmitoyltransferase-catalyzed conversion of palmitoylcarnitine to palmitoyl-CoA are markedly decreased with the progress of this reaction presumably owing to the build up of inhibitory palmitoyl-CoA in the enzyme vicinity. High, above micellar, concentrations of palmitoylcarnitine, phosphatidylcholine liposomes and high KCl concentrations increased the activity, apparently by facilitating the removal of palmitoyl-CoA from the enzyme surface. The presence of cardiolipin was found to be inhibitory. The enzyme activity followed in the direction of palmitoylcarnitine formation with low palmitoyl-CoA concentration as substrate, was inhibited by phosphatidylcholine, but stimulated by cardiolipin. Both of these lipids markedly stimulated the enzyme activity followed by the isotope exchange procedure which requires progression of both the forward and the backward reactions. The results indicate that one of the effects of phospholipids on carnitine palmitoyltransferase activity is exerted from the ability of these substances to bind the amphipathic reactants of this enzyme, particularly long-chain acyl-CoA. The possibility that the activity of the membrane-bound carnitine palmitoyltransferase may at times be affected by changes in the concentrations and composition of the various phospholipids in the enzyme's vicinity is raised by these findings.

We studied 123 lymph node biopsies from 99 patients with Hodgkin's disease, mixed cellularity type, with a high content of epithelioid cells (HDMCep), by light microscopy using conventional histologic and immunohistochemical techniques. The cellular composition and histologic structure, which are described in detail, serve as a basis for discriminating HDMCep from similar lymphomas with a high content of epithelioid cells, especially from lymphoepithelioid cell lymphoma (Lennert's lymphoma, LeL) and angioimmunoblastic (LgX) type of peripheral T-cell lymphoma with a high content of epithelioid cells (AILD-PTCLep). In all lymph nodes from patients with HDMCep, the nodal architecture was effaced. In 11% it was partially replaced by a massive infiltration of epithelioid cells and small to medium-sized lymphoid cells occurring in varying proportions with some immunoblasts. In all biopsy specimens, some typical Sternberg-Reed (SR) and Hodgkin (H) cells were found. Plasma cells and plasma cell precursors were present in all biopsy specimens; in most cases, they were sparse to moderate in number. Eosinophils were always present; half of the biopsy specimens had rather large numbers of them. Formation of granulomas like those seen in sarcoidosis was noted in 7% of the specimens. Increased vascularity and an increased number of fibers were found in 53% and 63% of the biopsy specimens, respectively. Necrosis was seen in 20% of the specimens. Immunohistochemically in 30 of 67 (45%) biopsy specimens, giant cells stained positively for the granulocyte-specific monoclonal antibody 3C4 (approximately CD15). Plasma cells and plasma cell precursors in all 70 specimens exhibited a polyclonal Ig pattern. A comparison of the main clinical and laboratory data in these three entities reveals both similarities and differences. HDMCep--defined as a special variant of Hodgkin's disease, mixed cellularity type (HDMC)--marks apparently the border between Hodgkin's disease and non-Hodgkin's lymphomas.

A peripheral T-cell lymphoma with a high content of epithelioid cells--an entity that reveals morphologic and clinical characteristics resembling those seen in angioimmunoblastic lymphadenopathy (AILD)--was separated from lymphoepithelioid cell lymphoma (Lennert's lymphoma, or LeL); it was considered a variant of the AILD type of peripheral T-cell lymphoma (AILD-PTCLep). A histologic evaluation of 135 lymph node biopsies from 98 patients with AILD-PTCLep showed that AILD-PTCLep and LeL share certain features, but there are also differences between the two. A finding common to both lymphomas was the mixture of neoplastic T-lymphocytes and a large number of epithelioid cells, which accumulated mostly in small, poorly defined clusters. Additionally, typical Sternberg-Reed and Hodgkin cells as well as giant cells resembling them were observed only rarely in either of these lymphomas. In contrast to LeL, AILD-PTCLep showed a great increase in follicular dendritic cells, which were sometimes identifiable in routine slides as so-called burned-out germinal centers. In AILD-PTCLep, the number of small vessels (predominantly epithelioid venules) and fibers is also usually greatly increased, whereas in LeL this is not the case, or the increase is only slight. Hypocellular and lymphocyte-depleted areas and PAS-positive intercellular material were seen in AILD-PTCLep (18% and 34%, respectively), but not in LeL. Large numbers of eosinophils, plasma cells and plasma cell precursors, and diffuse neoplastic infiltration of the capsule and extranodal tissue were observed much more commonly in AILD-PTCLep. Corresponding to these differences in the histologic pictures are differences in the clinical pictures. Hyperimmune phenomena (pruritus, skin rash, hemolytic anemia, allergy to antibiotics) and hypergammaglobulinemia were much more common in AILD-PTCLep than in LeL. Allergy to chemotherapeutic agents was observed only in AILD-PTCLep patients. Carcinomas were observed as a second neoplasm in 11% of the patients with AILD-PTCLep, but not at all in LeL. The similarities between the two lymphomas include the age and sex distribution (peak in the seventh decade and slight predominance of the male sex) and a similar median survival time. Another similarity is the possibility that the disease will develop into a large-cell malignant lymphoma. This was observed in 13% of the cases of AILD-PTCLep and 8% of the cases of LeL.

Steroid sulfatase is recovered quantitatively from the 105,000 g h supernatant of human placental microsomes extracted with Triton X-100. The solubilized enzyme has been purified using conventional techniques. Throughout the purification procedure, steroid sulfatase appears to be heterogeneous as evidenced by certain, but not all, criteria. Following polyacrylamide gel electrophoresis in the presence of sodium dodecyl sulfate, the final preparation exhibits a major component and varying amounts of two minor ones. Antibodies raised in rabbits with the heterogeneous immunogen give rise to a single precipitation line when the native enzyme is analyzed by double immunodiffusion or by immunoelectrophoresis. In addition, using aged preparations of microsomes and immunoaffinity techniques, steroid sulfatase activity was found to be associated with the fastest migrating minor component. This finding would suggest that the apparent heterogeneity of purified steroid sulfatase is linked to degradation processes occurring within the microsomal preparations. Steroid sulfatase has a Stokes radius of 56 A, a sedimentation coefficient of 4.85 +/- 0.15S (in Triton-containing buffers) and binds 1.3 g of Triton X-100-per g of protein. The molecular weight of the Triton-protein complex was calculated to be 166,000 in which the glycoprotein portion contribution is about 43% (72,000). In contrast, the apparent molecular weight of the major polypeptide determined on calibrated SDS-gels is 62,000. The purified enzyme exhibits two pH optima with cholesterol sulfate as substrate, an acidic one at pH 5.0 and a second one at pH 7.5. The Km values for cholesterol sulfate, dehydroandrosterone sulfate and p-nitrophenylsulfate were 5.26, 14 and 1,320 microM, respectively.

Subcutaneous and periarticular dystrophic calcifications are well known to be associated with progressive systemic sclerosis (PSS). On the other hand, calcifications inside the joints are very rarely reported. We report a new case of this unusual complication of PSS. The observation of the complete radiological follow-up led us to propose a mechanical rather than a purely inflammatory pathogenetic mechanism of this complication.

Three cases of D-penicillamine (DPA) induced myasthenia gravis (MG) and one case of DPA-induced polymyositis (PM) are reported among four patients suffering from seropositive rheumatoid arthritis. The cumulative doses responsible for the three DPA-induced MG cases amounted respectively to 73, 117 and 467 g. The cumulative dose responsible for the DPA-induced PM case amounted to 465 g. All the patients were HLA DR1. All four cases healed completely after withdrawal of DPA. The aetiology of the cases is discussed and the literature is reviewed. These cases represent further instances of DPA-induced neuromuscular disorders.

Whole microsomal membrane preparations and Triton X-100-solubilized human placental steroid sulfatase were subjected to radiation inactivation analysis using gamma rays from a 60Co irradiator in order to assess the size of the physiological form of the enzyme. The data indicate that the enzyme exists as a monomer of molecular weight 78600 in Triton-containing buffers and as a polymer of molecular weight 533000 within the microsomal membrane.

Histologic staging of Dupuytren's lesions into three types is proposed: a proliferative type with high cellularity and mitosis, a fibrocellular type characterized by the presence of a reticulin network, and a fibrous type with few cells. Sixty-three patients (77 hands) who had selective fasciectomy as a primary procedure and whose histologic specimen was available were clinically reviewed for disease recurrence and extension. Twenty-two hands (29%) were free of disease; twenty-five (32%) were free from recurrence but showed an extension, and thirty (39%) had a recurrence. This histologic classification seems to have a prognostic value because the recurrence rate is higher in type I (70%) and lower in type III (18%). The risk of extension did not correlate with the histologic type.

Sjögren's syndrome is a relatively uncommon condition in the paediatric age group. The youngest child reported thus far was a 5-year-old girl. This article reports the case of a 2-year-old girl admitted for recurrent infections of the respiratory tract with diffuse pulmonary interstitial infiltrations and a progressive swelling of the parotid glands. The clinical features and the results of laboratory investigations, including parotid and hepatic biopsies, chest X-rays and sialography helped to establish the diagnosis of Sjögren's syndrome. Clinical, immunological and genetic characteristics of Sjögren's syndrome are reviewed.