Diffuse soft tissue calcification in tumoral calcinosis

Tumoral calcinosis is a rare disease characterized biochemically by hyperphosphatemia, normocalcemia, and reduced fractional excretion of phosphate. Radiographically, it has been defined by the presence of large, amorphous juxtaarticular calcific deposits. A 53-year-old woman with tumoral calcinosis was found to have unusual diffuse soft tissue calcification indistinguishable from that usually seen in collagen vascular disease and previously referred to as calcinosis universalis. It is suggested that tumoral calcinosis is a misnomer as the calcification seen in patients with this disease may be tumoral or diffuse.     

Tumoral calcinosis-like lesion of the proximal linea aspera

Tumoral calcinosis is presently a poorly defined disease. In its classic form, it consists of multiple large foci of benign mineralization in the soft tissue adjacent to bone near large joints. Patients are generally of African descent and are adolescents or young adults at presentation. Both metabolic and traumatic etiologies have been proposed. We report six adult Caucasian patients with lesions that pathologically resembled tumoral calcinosis. All lesions were small (less than 3 × 3 cm) and were located along the proximal linea aspera of the femur. All patients presented with pain. Because of the atypical patient population and the unusual size and location of the lesions, we refer to this process as a tumoral calcinosis-like lesion. A typical radiographic appearance and location, together with appropriate clinical history, can strongly suggest this diagnosis.     

Mixed-sclerosing-bone-dystrophy: Report of a case and review of the literature

We present clinical, laboratory, radiologic, genetic, and pathologic findings in a 49-year-old man with mixed-sclerosing-bone-dystrophy (MSBD), review the six cases previously reported as MSBD, and examine the nosology of this rare bone dysplasia. Our asymptomatic patient showed radiographic changes consistent with osteopoikilosis, osteopathia striata, and melorheostosis and had widespread osteosclerosis of the axial skeleton. Several previous reports of combined osteosclerotic disorders suggest the latter finding represents osteopetrosis, however, histologic examination of our patient's iliac crest excluded that diagnosis. Limited radiographic surveys of his eight children were unremarkable except for isolated bone islands in two sons. Literature review revealed that MSBD has actually been used generically to describe the association of a variety of osteosclerotic bone dysplasias when they occur together in a single patient.     

Three-dimensional display of myocardial perfusion detection of ischemic lesions using a new image subtraction method

A recent, commercially available computer program for the three-dimensional (3D) display of single-photon emission tomography (SPET) data was used to study myocardial perfusion in patients with coronary artery disease (CAD). To enable the detection of small ischemic lesions, the authors proposed a new distance-subtraction method: after suitable centering of the axial slices, 3D distance-shaded images of the stress study were subtracted from the corresponding views of the rest study. With this technique, small changes in surface-to-observer distance were highlighted, thus enabling us to detect nontransmural ischemic areas of the myocardium. General characteristics and possibilities of the subtraction technique were tested on a simple myocardial phantom. Some clinical results of the application of this method on CAD patients are presented and discussed. In CAD patients in whom only nontransmural ischemic lesions are present, the subtraction of distance-shaded images is decisive for a correct diagnosis.     

Die besonderen konstruktionsmerkmale des japanischen „Tan-to“-(Messers) als Kriterien für dessen Gefährlichkeit

Zusammenfassung Es wird auf die besondere Klingenform japanischer Blankwaffen und auf die Neuentwicklung eines Verwandlungs-Tan-to verwiesen. Bei letzterem läßt sich die Klinge in der Handgriffmitte um 90° drehen und arretieren, eine ideale Fixierung dieses Messers durch Faustschluß am Quergriff. Durch diese Bedingungen ist eine hohe Verletzungsgefährdung mit tödlichem Ausgang gegeben. Diese seit kurzer Zeit im Handel erhältlichen Messer sollten nach § 37 Waff G umgehend als verbotene Gegenstände definiert werden.     

The “dural tail” adjacent to meningiomas studied by dynamic contrast-enhanced MRI: a comparison with histopathology

We examined 32 patients with intracranial tumors (17 meningiomas, 8 neuromas, 7 pituitary adenomas) by conventional and dynamic contrast-enhanced MRI. Our aim was to clarify whether the pathological dural contrast enhancement adjacent to meningiomas (the dural tail) is specific to meningiomas and, more important, whether it represents neoplastic dural infiltration or hypervascularization as a tumor accompanying reaction. A dural tail was found in 9 of 17 meningiomas. None of the other extra-axial tumours (neuromas, pituitary adenomas) showed comparable dural enhancement. Dynamic examinations with an ultrafast single slice imaging technique (snapshot-FLASH) after a bolus injection of contrast medium showed a dural tail in seven out of these nine meningiomas, while in two cases the dural tail turned out to be a cortical vein with a characteristic dynamic contrast enhancement pattern. In the dynamic study all seven dural tails were found to have earlier, steeper contrast enhancement than the corresponding tumours. All the tumours and part of the adjacent dura mater in four of the seven meningiomas with dural enhancement were examined histopathologically. In none of these four cases was neoplastic tissue found more than 2 mm away from the main tumour. The results strongly support the suggestion that the dural tail adjacent to meningiomas represents a hypervascular, non-neoplastic dural reaction.     

Pulmonary Hypertension Among End-Stage Renal Failure Patients Following Hemodialysis Access Thrombectomy

Purpose: Percutaneous hemodialysis thrombectomy causes subclinical pulmonary emboli without short-term clinical consequence; the long-term effects on the pulmonary arterial vasculature are unknown. We compared the prevalence of pulmonary hypertension between patients who underwent one or more hemodialysis access thrombectomy procedures with controls without prior thrombectomy.Methods: A retrospective case-control study was performed. Cases (n=88) had undergone one or more hemodialysis graft thrombectomy procedures, with subsequent echocardiography during routine investigation of comorbid cardiovascular disease. Cases were compared with controls without end-stage renal disease (ESRD) (n=100, group 1), and controls with ESRD but no prior thrombectomy procedures (n=117, group 2). The presence and velocity of tricuspid regurgitation on echocardiography was used to determine the prevalence and grade of pulmonary hypertension; these were compared between cases and controls using the chi-square test and logistic regression.Results: The prevalence of pulmonary hypertension among cases was 52% (46/88), consisting of mild, moderate and severe in 26% (n=23), 10% (n=9) and 16% (n=14), respectively. Prevalence of pulmonary hypertension among group 1 controls was 26% (26/100), consisting of mild, moderate and severe pulmonary hypertension in 14%, 5% and 7%, respectively. Cases had 2.7 times greater odds of having pulmonary hypertension than group 1 controls (p=0.002). The prevalence of pulmonary hypertension among group 2 controls was 42% (49/117), consisting of mild, moderate and severe pulmonary arterial hypertension in 25% (n=49), 10% (n=12) and 4% (n=5), respectively. Cases were slightly more likely to have pulmonary hypertension than group 2 controls (OR=1.5), although this failed to reach statistical significance (p=0.14).Conclusion: Prior hemodialysis access thrombectomy does not appear to be a risk factor for pulmonary arterial hypertension. Patients with ESRD are more likely to have pulmonary hypertension.     

Calcinosis in juvenile dermatomyositis

A retrospective study of the radiographs and clinical records of 39 children with dermatomyositis, followed up at a referral centre for a mean period of eight years, revealed calcinosis in 29 children (18 giris, 11 boys). Clinical presentation of calcinosis was unpredictable and variable, ranging from four months to 12 years after onset of disease. Subcutaneous calcilication, often associated with subsequent ulceration of overlying skin, was observed more frequently than calcinosis in intermuscular fascial planes (29:7). Subcutaneous calcinosis was demonstrated most commonly about the knees and elbows and in the fingers and toes. Intermuscular calcinosis affected the fascial planes around the quadriceps, deltoid, biceps, and the gastrocnemius/soleus muscles.Calcinosis, predominantly of the subcutaneous lesions, regressed spontaneously in eight of 11 children where this could be assessed over a one to four year period. This high rate of spontaneous resolution must complicate evaluation of the efficacy of proposed anti-calcinotic treatments in juvenile dermatomyositis.While no apparent relationship was established between subcutaneous calcinosis, which was present in all 29 of the affected patients, and disease activity or severity, all seven children with the additional, extensive, and classical intermuscular fascial plane calcification developed this complication early and had severe disease.These findings are discussed in relation to previous studies.     

Das Problem der a-priori-Wahrscheinlichkeit bei Verwendung der Essen-Möller-Formel zur Berechnung der Vaterschaftswahrscheinlichkeit

Zusammenfassung Die Annahme der a-priori-Wahrscheinlichkeit, daß Nichtväter und wahre Väter gleichhäufig zur Begutachtung gelangen, wird an Hand von 408 Aktenfällen bestätigt.Die Berechnung der Vaterschaftswahrscheinlichkeit bietet, insbesondere bei Mehrverkehrsfällen, wertvolle Hinweise für die Vaterschaft oder Nichtvaterschaft eines Probanden, Hinweise, die durch die Angabe der Vaterschaftsausschlußchance aufgrund der Mutter-Kind-Konstellation nicht erbracht werden können. Auf Beziehungen zwischen der Vaterschaftsausschlußchance und der Vaterschaftswahrscheinlichkeit wird hingewiesen.

---

The assumption of prior probability computing the plausibility of paternity by the Essen-Möller equation and its problem

Summary Among objectivists the assumption of firsthand probability, that non-fathers and true-fathers are being given an opinion on with equal frequency, yields to a rejection in calculating the probability of fatherhood according to Essen-Möller. By means of 408 cases it is shown, that this assumption does meet the actual situation. Non-fathers and true-fathers are observed equally frequent. Calculation of fatherhood probability renders a valuable reference to paternity or nonpaternity of a subject in cases of multiple intercourses. These references cannot be brought on by the statement of the exclusion chance of fatherhood based on the mother-child-constellation. The relationship between the exclusion chance and the probability of fatherhood is pointed out.

     

The value of T1-weighted images in the differentiation between MS,white matter lesions,and subcortical arteriosclerotic encephalopathy (SAE)

Summary The aim of the study was to define reliable criteria for the differentiation of MR imaging between patients with MS and with vascular white matter lesions/SAE. We examined 35 patients with proven MS according to the Poser criteria and 35 patients with other white matter lesions and/or SAE. The result is that with MR a differentiation can be achieved provided that T1-weighted spin-echo sequences are included and the different pattern of distribution is considered. MS plaques are predominantly located in the subependymal region, vascular white matter lesions are mainly located in the water-shed of the superficial middle cerebral branches and the deep perforating long medullary vessels in the centrum semiovale. Infratentorial lesions are more often seen in MS. Confluence at the lateral ventricles is frequently accompanied by confluent abnormalities around the third ventricle, Sylvian aqueduct, and fourth ventricle, which is uncommon in SAE. In MS many lesions visible on T2-weighted images have a cellular or intracellular composition that renders them visible also on T1-weighted ones as regions with low signal intensity and more or less distinct boundary. Vascular white matter lesions and SAE mainly represent demyelination and can there-fore be seen on T2-weighted images, but corresponding low signal intensity lesions on T1-weighted images are uncommon. In some exceptions there are such lesions with low signal representing lacunar infarcts or widened Virchow-Robin-spaces.     

Calcified convexity dura mater and acute epidural haematoma mimicking calcified chronic subdural haematoma

We report a patient with calcification of the convexity dura mater and an acute epidural haematoma. CT revealed a calcified layer between the haematoma and brain parenchyma, which mimicked acute bleeding into a calcified chronic subdural haematoma. The appearance of a calcified haematoma does not always mean a chronic lesion, and that emergency operation should not be foregone, when there is a history of acute head trauma and progressive impairment of consciousness.     

Usefulness of different myocardial sampling zones for the postmortem diagnosis of myocardial infarction

Summary The diagnosis of myocardial infarction requires the use of a group of tests that are very efficient, quick and inexpensive. Another important consideration is the choice of myocardial sampling zones, especially in cases of differential diagnosis between a cardiac injury secondary to a trauma or violent asphyxia and others, secondary to myocardial infarction. The aim of this work was to choose, through discriminant analysis, the most useful zones of cardiac tissue for the quantification of free fatty acids and free carnitine and for the performance of the K/Na quotient, as biochemical parameters for the postmortem diagnosis of myocardial infarction. According to the discriminant analysis performed, seven zones of cardiac tissue are necessary to achieve a differential diagnosis among myocardial infarction, other natural deaths, and violent deaths with a 71.9% efficacy. Greater diagnostic efficacy was found (78.1%) for differentiating between natural deaths and violent deaths. Offprint requests to: E. Lachica     

Bone island (enostosis): current concept — a review

An enostosis or bone island represents a focus of mature compact (cortical) bone within the cancellous bone (spongiosa). Thought by some to be a tumor-like condition and by others a hamartoma, this benign lesion is probably congenital or developmental in origin and reflects failure of resorption during endochondral ossification. A bone island can be virtually diagnosed based on its characteristic clinical and radiologic features. Typically asymptomatic, the lesion is usually an incidental finding, with a preference for the pelvis, femur, and other long bones, although it may be found anywhere in the skeleton, including the spine. Plain radiography reveals a homogeneously dense, sclerotic focus in the cancellous bone with distinctive radiating bony streaks (thorny radiation) that blend with the trabeculae of the host bone, creating a feathered or brush-like border. On CT scan, a bone island appears as a low-attenuation focus, and on MRI sequences it shows low signal intensity like cortical bone. A distinguishing feature of bone islands is that they are usually cold on skeletal scintigraphy. Thus, bone scan has been and continues to be the means of differentiating bone islands from the more aggressive entities. However, reports of histologically confirmed bone islands that were scintigraphically active have raised a note of caution about relying on this modality in the differential consideration of lesions otherwise characteristic of bone islands. Guides to the correct diagnosis should be looked for in the individual clinical situation and in the morphologic features of the lesion on plain radiography, CT, and MRI, without regard to the lesion's activity on bone scan. If such a lesion, however, is symptomatic and hot on scintigraphy, it demands close observation with follow-up imaging studies.     

Sclerotic changes of the manubrium sterni

Six females with nearly identical sclerotic and hyperostotic changes of the manubrium sterni are reported. Malignancies, bacterial inflammatory processes, and Paget disease, which were first suspected, could be excluded. The youngest patients also had sclerotic changes of other bones, including the lumbar spine, the pubic bone, and the clavicle, and may be classified as having chronic recurrent multifocal osteomyelitis (CRMO). The two oldest patients had ossification of the costoclavicular ligament(s) and may be classified as having inter-sterno-costo-clavicular ossification (ISCCO). One had only hyperostotic and sclerotic changes as seen in sterno-costo-clavicular hyperostosis (SCCH). The pathogenesis of these uncommon diseases is unknown, but they are all frequently associated with pustulosis palmoplantaris and have similar clinical courses and laboratory abnormalities. None of the present patients had HLA-B₂₇. The similarity of the radiological abnormalities of the manubrium sterni suggests that the diseases themselves may be similar, but with different courses depending on age, CRMO being present in children and young adults and ISCCO or SCCH in older adults.     

Hemorrhage associated with “bone crisis” in Gaucher's disease identified by magnetic resonance imaging

Children suffering from Gaucher's disease were examined by magnetic resonance imaging (MRI) during a characteristic episode of bone crisis. An unexpectedly high intramedullary as well as subperiosteal signal was observed on both the T1 and T2-weighted sequences in 5 patients, suggesting a subacute hemorrhage or hematoma. It is conceivable that such a painful hemorrhage is an important component of the bone crisis phenomenon. Furthermore, in these cases this is a specific sign which may enable differentiation of bone crises from other types of bone pain associated with Gaucher's disease.     

Short-stop-munition

Zusammenfassung Immer wieder wird Munition zur Verteidigung unter Hinweis auf ihre weniger-tödliche bis nicht-tödliche Wirkung angeboten. Die Praxis zeigt jedoch, daß von diesen Munitionstypen entgegen den oft verharmlosenden Angaben der Hersteller sehr wohl eine konkrete Gefahr ausgeht. Einerseits können daraus schwerwiegende Folgen resultieren, andererseits kann als Einlassung vor Gericht die Vorhersehbarkeit solcher Folgen begrundet in Frage gestellt werden. Unter diesem Aspekt werden eine spezielle Schrotmunition für Revolver des Typs Speer 38/357 Shot Shells des Herstellers CCI, die Patrone Short-Stop der Fa. MB Associates in San Ramon (Kalifornien) and mit eigenem Fallbeispiel eine Munition vom Schock-Defense-Typ der Fa. Rauchalles in Offenburg vergleichend hinsichtlich Aufbau und Wirkung besprochen. Sonderdruckanfragen an: W.Eisenmenger     

Ossification and pseudoepiphysis formation in the “nonepiphyseal” end of bones of the hands and feet

Metacarpals, metatarsals, and phalanges were studied to assess the developmental morphology of secondary ossification in the nonepiphyseal ends of these bones as well as the formation of the pseudoepiphysis as an epiphyseal ossification variant. Both direct ossification extension from the metaphysis into the epiphysis and pseudoepiphysis formation preceded, and continued to be more mature than, formation and expansion of the classic epiphyseal (secondary) ossification center at the opposite end of each specific bone. Direct metaphyseal to epiphyseal ossification usually started centrally and expanded hemispherically, replacing both physeal and epiphyseal cartilage simultaneously. In contrast, when remnants of physis were retained, while juxtaposed epiphyseal cartilage was replaced, a pseudoepiphysis formed. There were three basic patterns of pseudoepiphysis formation. First, a central osseous bridge extended from the metaphysis across the physis into the epiphysis and subsequently expanded to create a mushroom-like osseous structure. In the second pattern a peripheral osseous bridge formed, creating either an osseous ring or an eccentric bridge between the metaphysis and the epiphysis. In the third pattern, multiple bridging occurred. In each situation the associated remnant physis lacked typical cell columns and was incapable of significantly contributing to the postnatal longitudinal growth of the involved bone. Pseudoepiphyses were well formed by 4–5 years and coalesced with the rest of the bone months of years before skeletal maturation was attained at the opposite epiphyseal end, which ossified in the typical pattern (i.e., formation of a secondary center de novo completely within the cartilaginous epiphysis). This process may also affect the development and appearance of ossification within the longitudinal epiphyseal bracket (delta phalanx).     

Positron emission tomography imaging of adrenal masses: <Superscript>18</Superscript>F-fluorodeoxyglucose and the 11β-hydroxylase tracer <Superscript>11</Superscript>C-metomidate

Purpose ¹¹C-metomidate (MTO), a marker of 11-hydroxylase, has been suggested as a novel positron emission tomography (PET) tracer for adrenocortical imaging. Up to now, experience with this very new tracer is limited. The aims of this study were (1) to evaluate this novel tracer, (2) to point out possible advantages in comparison with ¹⁸F-fluorodeoxyglucose (FDG) and (3) to investigate in vivo the expression of 11-hydroxylase in patients with primary aldosteronism.Methods Sixteen patients with adrenal masses were investigated using both MTO and FDG PET imaging. All patients except one were operated on. Five patients had non-functioning adrenal masses, while 11 had functioning tumours(Cushings syndrome, n=4; Conns syndrome, n=5; phaeochromocytoma, n=2). Thirteen patients had benign disease, whereas in three cases the adrenal mass was malignant (adrenocortical cancer, n=1; malignant phaeochromocytoma, n=1; adrenal metastasis of renal cancer, n=1).Results MTO imaging clearly distinguished cortical from non-cortical adrenal masses (median standardised uptake values of 18.6 and 1.9, respectively, p<0.01). MTO uptake was slightly lower in patients with Cushings syndrome than in those with Conns syndrome, but the difference did not reach statistical significance. The expression of 11-hydroxylase was not suppressed in the contralateral gland of patients with Conns syndrome, whereas in Cushings syndrome this was clearly the case. The single patient with adrenocortical carcinoma had MTO uptake in the lower range.Conclusion MTO could not definitely distinguish between benign and malignant disease. FDG PET, however, identified clearly all three study patients with malignant adrenal lesions. We conclude: (1) MTO is an excellent imaging tool to distinguish adrenocortical and non-cortical lesions; (2) the in vivo expression of 11-hydroxylase is lower in Cushings syndrome than in Conns syndrome, and there is no suppression of the contralateral gland in primary aldosteronism; (3) for the purpose of discriminating between benign and malignant lesions, FDG is the tracer of choice.     

Total dural calcification with secondary hyperparathyoidism: A rare entity

Summary A case of total, dense dural calcification in an adult female patient with secondary hyperparathroidism is presented. The often reported feature in the skull is that of a combination of osteopenia and osteosclerosis giving a granular bone texture described sometimes as grains of sand or salt and pepper appearance [6]. Extensive dural calcification with this condition has very rarely been reported [2, 3]. The purpose of this paper is to document the existence of the latter in a patient followed up for 19 years.     

γ-Glutamyltransferase test as a new tool for identification of seminal stains

Summary A simple qualitative method for identification of seminal stains based on a high activity of -glutamyltransferase (-GTP) in human semen is described. It employs the release of -naphthylamine from N--glutamyl--naphthylamide by the -GTP action; -naphthylamine couples with Fast Garnet GBC salt to produce a strong brownish-red color. The data on its simplicity, specificity, and stability show that the present method is suitable for medicolegal examination of seminal stains as a preliminary test.