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Biomedical subjects

H Hadar

Publications and source records attributed to H Hadar.

At least 19 recordsLinked to original sources

Muscle computed tomography features of motor neuron disease in late-onset GM2 gangliosidosis.

Late-onset GM2 gangliosidosis, a rare inherited neuronal storage disease, is characterized by a variety of clinical manifestations. The common clinical picture comprises neuromuscular, spinocerebellar, extrapyramidal, cognitive, and psychiatric abnormalities. Details of the extent of muscle involvement have never been reported. Eight patients with this syndrome were evaluated for the existence and extent of motor neuron disease using routine electrodiagnosis and systematic evaluation of skeletal musculature by computed tomography. Motor neuron disease was present in each and every patient regardless of the clinical manifestations and to a degree beyond that suspected on neurological examination. Muscle imaging disclosed a diffuse wasting and fatty replacement of muscles with predilection of pelvic and thigh muscles, and especially the quadriceps group. It seems that progressive motor disability in this syndrome is mainly due to motor neuron disease, as manifested by muscle atrophy, which can be easily demonstrated by muscle computed tomography.

Adipose Tissue

[Immunotherapy for metastatic renal cell carcinoma: treatment with PLAK cells and low-dose interleukin-2].

During the past 5 years publications from the NIH (Rosenberg et al.) and other centers have reported encouraging results in the treatment of metastatic renal cell carcinoma. Adoptive immunotherapy was applied, using lymphocytes activated by interleukin-2 (LAK cells) plus high doses of interleukin (IL-2) systemically. The mean clinical response rate was 20-35%. Severe lifethreatening adverse reactions to high doses of IL-2 were noted, although they were all of short duration. Laboratory findings of Novogrodsky et al. from Beilinson Medical Center, Israel showed that oxidizing mitogens can induce lymphocyte activation (PLAK cells). Further studies suggested that a combination of such activated cells with low doses of IL-2 could produce effective toxicity to tumor cells without the need for high doses of IL-2 which could be very toxic for the patient. In the past year we treated 7 patients with PLAK cells and IL-2. 4 completed the treatment, of whom 1 responded partially (regression of more than 50% of lung metastases), 1 is stable and in 1 liver metastases regressed but metastases in lumbar vertebrae and in the pelvis progressed. 1 patient died a month after discharge from hospital, probably due to rapid progression of the disease. Our protocol follows that of the Phase II clinical study of 40 patients treated at the Rogosin Institute, New York Hospital--Cornell Medical Center. The mean clinical response rate was 23.6%. Toxicity of IL-2 is dose-dependent. In this protocol, the low doses of IL-2 gave significantly fewer adverse reactions.

Adult

Pitfalls in the follow-up of cervical and mediastinal goitres: role of CT imaging.

Four patients with cervical and mediastinal goitres were described. Routine investigation in these patients, including chest X-rays, oesophageal and tracheal X-rays and scintigraphy of the thyroid gland, failed to show significant changes over the years, or underestimated the true extent of the goitres. Three of the goitres caused severe mediastinal compression, evident only on computed tomography (CT) imaging, and requiring urgent surgery. The pros and cons of different diagnostic modalities for imaging cervical and mediastinal goitres are discussed, stressing the importance of CT imaging as exemplified in the cases presented.

Aged

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.

Adolescent

The effect of intravesical bacillus Calmette-Guerin therapy on the upper urinary tract.

A total of 66 patients with low grade, low stage transitional cell carcinoma of the bladder who were treated with intravesical bacillus Calmette-Guerin (BCG) underwent cystourethrography to detect vesicoureteral reflux. BCG was instilled weekly for 6 weeks and monthly thereafter for up to 24 months. Whenever vesicoureteral reflux was found or morphological abnormalities were detected on excretory urography (IVP) an isotope renal scan was performed to evaluate the relative renal function. Vesicoureteral reflux was found in 13 patients (19.7%): 10 had grades 1 and 2A, and 3 had grade 2B reflux. The number of BCG instillations ranged from 8 to 22. IVPs were normal in 11 patients. In 2 patients mild unilateral dilatation was present before BCG instillations, and this remained unchanged during and after therapy. None of the 13 patients with vesicoureteral reflux had IVP features suggestive of urinary tuberculosis. In 11 patients the refluxing renal systems had normal relative renal function (50 to 55%). Two patients had a decrease to 40% of the relative renal function with normal IVPs, suggesting a nonBCG related cause. We conclude that BCG therapy is safe in patients with minimal reflux.

Aged

Short pancreas in polysplenia syndrome.

Two cases with the polysplenia syndrome also had a short pancreas. To the best of our knowledge the radiologic characteristics of this anatomic anomaly have not yet been reported. Both cases were asymptomatic with regard to their anomaly-complex. The radiologic findings of the short "pancreas" are similar to the only pathologic description of this association. The awareness of this association led to the diagnosis of the second case after a few months only. A physician performing ERCP in the polysplenia syndrome should keep in mind the possible presence of the "short pancreas" when the whole length of the pancreatic duct is not visualized.

Abnormalities, Multiple

Air in vagina. Indicator of intrapelvic pathology on CT.

Computed tomography of the normal female pelvis may show the presence of a tiny air bubble within the vagina. Among 2,800 female abdomino-pelvic CTs, 27 were found to contain large amounts of intravaginal air. Twenty-three patients suffered from pelvic malignancy while in 4, non-tumorous pelvic pathology was present. Detection of air bubbles in the vagina is abnormal and indicates, in the majority of cases, an intrapelvic malignancy.

Adult

Evaluation of renal angiomyolipoma by traditional and modern imaging in a case of tuberous sclerosis.

Renal angiomyolipoma is known to affect mainly patients with tuberous sclerosis, although it can be found in a significant number of otherwise normal individuals. Few cases have been published in which angiomyolipoma and renal cell carcinoma occurred in the same kidney. In such cases the radiologist may be asked if it is possible to distinguish between the two tumors in the same kidney. The present case report describes systemic traditional and modern imaging of huge bilateral angiomyolipoma in tuberous sclerosis. The analysis of the data implies that, although the diagnosis of angiomyolipoma can be established with noninvasive techniques only, it is impossible to distinguish between the two mentioned tumors if they coexist in the same kidney, even if all available imaging techniques are used.

Adult

A comparative evaluation of lymphoscintigraphy versus lymphangiography and computerized tomography scanning in diagnosis of lymph node metastases in advanced bladder cancer.

The potential of lymphoscintigraphy to detect lymph node metastases compared to lymphangiography and computerized tomography scanning was evaluated in 26 patients who underwent radical cystectomy for invasive bladder cancer. Four-view images of the abdominoperineal area were taken 90 to 120 minutes after 99mtechnetium-rhenium sulfide was injected into 2 interdigital spaces in each foot. Results of lymphoscintigraphy interpretation correlated with surgical and histological findings: a correct diagnosis was made in 61.5 per cent of the patients, while 23.1 per cent had false positive and 15.4 per cent had false negative results. Although computerized tomography was the most accurate method to detect lymph node metastases (correct diagnosis in 73.1 per cent of the patients) no significant difference was found among the 3 diagnostic methods. False positive interpretation of lymphoscintigraphy was twice as common as that of the radiological studies (23 versus 11.5 per cent). The possibilities that may cause image variation interpreted as a false positive result are discussed. Because lymphoscintigraphy is an easier and less time-consuming study than lymphangiography, the former method is suggested to be an additional and sometimes (for example if short-term followup studies are required) preferred modality to evaluate the extent of lymph node involvement in cases of invasive bladder cancer.

Aged

Gaucher disease: assessment with MR imaging.

The skeletal system, spleen, and liver of five patients with proved Gaucher disease were studied with magnetic resonance (MR) imaging. Homogeneous, low intensity signals resulting from relaxation times different than normal (longer T1 and shorter T2 values) were found in the marrow of long bones, vertebrae, and hips in all patients. In three patients, normal signals were noted in the patella, epiphysis of the knee, and capital femoral epiphysis. In two patients with acute bone pain in the tibial region, a higher signal was received from the tibial marrow. This signal was related to increased accumulation of fluid following an avascular episode. Soft tissues in the same area were also involved. Liver and spleen enlargement was readily visible, especially on coronal images. T1 values of spleen were significantly shorter than normal. MR imaging provides an excellent assessment of the extent of involvement of the liver, spleen, and bone marrow in Gaucher disease.

Adolescent

Nuclear magnetic resonance proton imaging of bone pathology.

Thirty-two patients with diversified pathology were examined with a supraconductive NMR imager using spin echo with different TR and TE to obtain T1 and T2 weighted images. They included 20 tumors (12 primary, eight metastasis), six osteomyelitis, three fractures, two osteonecrosis, and one diffuse metabolic (Gaucher) disease. In all cases except for the stress fractures, the bone pathology was clearly visualized in spite of the normal lack of signal from the compact cortical bone. Nuclear magnetic resonance (NMR) imaging proved to be at least as sensitive as radionuclide scintigraphy but much more accurate than all other imaging procedures including computed tomography (CT) and angiography to assess the extension of the lesions, especially in tumors extended to soft tissue. This is due both to easy acquisition of sagittal and coronal sections and to different patterns of pathologic modifications of T1 and T2 which are beginning to be defined. It is hoped that more experience in clinical use of these patterns will help to discriminate between tumor extension and soft-tissue edema. We conclude that while radionuclide scintigraphy will probably remain the most sensitive and easy to perform screening test for bone pathology, NMR imaging, among noninvasive diagnostic procedures, appears to be at least as specific as CT. In addition, where the extension of the lesions is concerned, NMR imaging is much more informative than CT. In pathology of the spine, the easy visualization of the spinal cord should decrease the need for myelography.

Adolescent

Magnetic resonance imaging in orthopaedic surgery. A glimpse into the future.

Magnetic resonance images (MRI) were obtained of 10 healthy volunteers and 70 patients suffering from various orthopaedic disorders. Selected images of soft tissue, joint, bone and spinal abnormalities are presented and their interpretation is described. Although we have been using MRI for only a very short time, it is already possible to see its advantages: it provides good images of soft-tissues, detailed pictures of bone marrow, and excellent visualisation of the spine and spinal cord. The decision-making process in surgical procedures will in the future be influenced by this technique.

Bone Diseases

Computed tomography of renal agenesis and ectopy.

The position and shape of the organs normally adjacent to the kidney were evaluated by computed tomography in seven patients with renal agenesis and in two patients with unilateral renal ectopy. The major findings were displacement of colonic flexures, small intestine loops, duodenum, spleen, and tail of the pancreas. The shape and position of the adrenal gland was found abnormal in cases with left-sided anomaly. Genital abnormalities such as absent seminal vesicle were frequently encountered. The presence of abnormally shaped adrenal glands in patients with left renal agenesis and ectopy is a new diagnostic radiologic sign for these conditions.

Adrenal Glands

Positional relations of colon and kidney determined by perirenal fat.

The anatomic relations of the colon in regard to the kidney at three levels were studied with CT in 70 male and 70 female patients in various age groups. The study was initiated when two patients with large left-kidney masses had an unusual posterior location of the colon. Considering the increase in percutaneous uroradiologic invasive procedures, it was believed that such a study was needed. It was shown that the colon has a tendency to be displaced anteriorly to the kidney in elderly men, whereas it is laterally located in females of all ages. An outstanding and unique finding was the excessive accumulation of perinephric fat in males and its quite total absence in females. It is suggested that this gender-related peculiar fat distribution might explain the anterior displacement of the colon in males.

Adipose Tissue

Fatty replacement of lower paraspinal muscles: normal and neuromuscular disorders.

The physiologic replacement of the lower paraspinal muscles by fat was evaluated in 157 patients undergoing computed tomography for reasons unrelated to abnormalities of the locomotor system. Five patients with neuromuscular disorders were similarly evaluated. The changes were graded according to severity at three spinal levels: lower thoracic-upper lumbar, midlumbar, and lumbosacral. The results were analyzed in relation to age and gender. It was found that fatty replacement of paraspinal muscles is a normal age-progressive phenomenon most prominent in females. It progresses down the spine, being most advanced in the lumbosacral region. The severest changes in the five patients with neuromuscular disorders (three with poliomyelitis and two with progressive muscular dystrophy) consisted of complete muscle group replacement by fat. In postpoliomyelitis atrophy, the distribution was typically asymmetric and sometimes lacked clinical correlation. In muscular dystrophy, fatty replacement was symmetric, showing relative sparing of the psoas and multifidus muscles. In patients with neuromuscular diseases, computed tomography of muscles may be helpful in planning a better rehabilitation regimen.

Adipose Tissue

Calcification in the portal venous system demonstrated by computed tomography.

The CT appearance of calcification in the portal venous system in a patient with chronic alcoholic cirrhosis is presented. Report cases of radiologically detectable calcification in the portal system are rare, and most of them have been associated with longstanding portal hypertension. We presume that with CT this diagnosis will be made more frequently. In the presence of calcification in the portal venous system, portal vein thrombosis is highly probable. This information is of obvious importance to the surgeon contemplating a portal decompressive shunt procedure.

Calcinosis