Biomedical subjects
Publications and source records attributed to G Amir.
We describe a 15-year-old boy with hemangiomatosis of bone and hypophosphatemic rickets. The rickets was ameliorated by irradiation of the skeletal lesions.
A 21-year-old female with autoimmune polyglandular failure (APG) manifested by insulin-dependent diabetes mellitus (IDDM), hypothyroidism and pernicious anaemia developed severe malabsorption due to exocrine pancreatic insufficiency. Supplemental pancreatic enzymes resulted in marked improvement of steatorrhea. There was also an incidental finding of gastric carcinoid tumour. We identified only 13 other patients in our institution with either type 2 or 3 APG, one of which had significant steatorrhoea. Another patient with IDDM, hypothyroidism and pernicious anaemia had an asymptomatic gastric carcinoid tumour. The possible mechanisms for malabsorption in APG are discussed.
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This is the first reported case of ABC in a reactive lesion of soft tissue. The ABC may be regarded as an unusual type of organization of hematoma encountered in a bone-producing milieu. This would support the current concept that ABC occurring in association with another condition represents a secondary change in a pre-existing lesion.
Radiation damage to the neuromuscular junctions (NMJs) in mouse tongues was studied using local x-irradiation of the tongues with the rest of the body shielded. Transmission electron microscopy (TEM) revealed no significant morphological changes in the fine structures and organelles of the NMJs given 4 Gy. A dose of 8 Gy produced degenevative morphological changes associated with oxon terminal sprouting as early as 2 and 7 days following irradiation. Subsequently, 1-11 weeks later, severe degenerative changes were observed. The number of mitochondria was significantly decreased with increased occurrence of degenerative membranal features. The number of synaptic footplates without terminals or with multiple small terminals within one groove increased gradually with time. Most of these pathological changes persisted for at least 3 months after irradiation. However, the myofibres, blood vessels and interstitial cells appeared to be unaffected throughout the period of follow-up. The present study substantiates our previous reports of ageing-like changes in the tongues' NMJs induced by their excessive exposure to free radicals.
AIMS: To determine whether remodelling of subchondral bone in osteoarthritis is related to anatomical and mechanical factors operating on the joint surface. METHODS: Ten femoral heads were examined. Patients due to have the femoral head removed because of osteoarthritis were given a double tetracycline label before surgery. The specimens were divided in three roughly equal parts, corresponding to the medial, central (weight bearing), and lateral aspects of the femoral heads. Undecalcified methylmethacrylate embedded sections were prepared. The subchondral bone was assessed for appositional bone formation by ultraviolet light microscopy, for resorptive activity by osteoclast count, and for trabecular bone volume by semiautomatic computerised image analysis. Appositional bone rate was also correlated with the presence or absence of overlying cartilage. RESULTS: Bone density was reduced in the medial aspect of the femoral heads but there was no significant difference between the appositional bone rate or the resorptive activity in the three areas. Nor was there any correlation between appositional bone rate in the subchondral bone and the presence or absence of overlying cartilage. CONCLUSION: Remodelling of subchondral bone in osteoarthritis, at least in the short term, is fairly constant and is not related to weight bearing.
Giant cell arteritis and polyarteritis nodosa are systemic diseases which rarely involve the breasts. Two cases are reported in which breast masses, clinically suspected to be malignant, were found to be isolated vasculitis--bilateral giant cell mammary arteritis in a 67-year-old woman and isolated mammary polyarteritis nodosa in a 45-year-old woman. Vasculitis should be considered in the differential diagnosis of breast masses.
The introduction of sensitive assays for the beta subunit of hCG (beta-hCG) and improved ultrasound technology for diagnosis and monitoring of ectopic pregnancy have changed the clinical approach to the management of ectopic pregnancy. Only a few reports have been published of patients with ectopic gestation and negative serum beta-hCG levels. None of these described rupture of ectopic pregnancy following the decline of beta-hCG levels to below 10 mIU/mL. We describe a case of ectopic pregnancy, managed expectantly, in which rupture and hemoperitoneum occurred after the decline of beta-hCG levels to below 10 mIU/mL.
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Obstructive jaundice developed in a patient concomitantly with the diagnosis of breast carcinoma. Abdominal exploration disclosed a metastatic tumor in the head of the pancreas, the distal bile duct, and the gallbladder. A cholecystectomy and choledochojejunostomy were performed and later, because of intestinal obstruction, the patient underwent gastrojejunostomy. Pathological examination demonstrated metastatic lobular carcinoma of breast with strongly positive staining for estradiol. Additional hormonal therapy has been given to the patient since the operation. The patient is alive 16 months after the diagnosis of her disease. This case suggests that a vigorous diagnostic approach should be adopted in every jaundiced patient with metastatic breast cancer in order to exclude causes of jaundice other than diffuse metastatic involvement of the liver. Patients with extrahepatic biliary metastasis should be treated by aggressive surgical treatment, combined with systemic therapy which can offer them significant palliation and better survival.
Systemic mastocytosis is a rare condition in which mast cells infiltrate various organs, including the skeleton. Because the mast cell secretes various bioactive substances that may induce bone resorption, this condition may cause generalized osteoporosis. We describe a case of a 28-year-old woman who presented with a painful thoracolumbar kyphosis due to generalized osteopenia and multiple pathological compression fractures and was found to have mastocytosis. She underwent operative stabilization of her kyphotic deformity with anterior interbody fusion and posterior Cotrel-Dubousset (CD) instrumentation and fusion. We conclude that mastocytosis should be suspected in an atypical case of so-called idiopathic osteoporosis.
Femoral and tibial bones of diabetic sand rats were compared to those of non-diabetic sand rats (Psammomys obesus) of the same age range. Three morphometric parameters were examined: trabecular bone volume, length of epiphyseal cartilage (proliferative and mature) and cortical thickness of mid-shaft of tibia. There were no statistically significant differences between the test and control animals for the last two parameters. By contrast the trabecular bone volume of the diabetic animals was less than half that of the control animals indicating a substantial loss of trabecular bone mass. This study suggests that Psammomys obesus is a suitable model for studying the effects of diabetes mellitus on bone mass, provided that weight bearing bones are used.
The scapula is a rare location for osteoid osteoma, which in most cases does not involve the flat bones. The en bloc excision in this uncommon location can be problematic since the surgical exposure is difficult, and shoulder joint function can be affected if the lesion is subchondral. In an 18-year-old man, an osteoid osteoma was located in the subchondral area of the glenoid. A guided needle biopsy of the nidus resulted in complete healing. This mode of treatment, with proper follow-up examination, is acceptable for a benign lesion situated in a problematic location.
The tidemark is a metabolically active zone in the calcified layer of the cartilage in which it is possible to show that there is calcifying activity even over a short period of time using a tetracycline labelling technique. The tidemark slowly advances in the direction of the non-calcified cartilage and analysis of double-tetracycline labelled cartilage shows that this is not an appositional phenomenon like that occurring in bone, but that, where present, several tidemarks can be labelled at the same time. Each tidemark may therefore be metabolically active and it is not just the tidemark adjacent to hyaline cartilage which incorporates calcium.
We report two cases of osteoclast-like giant cell tumour of urinary bladder associated with papillary transitional cell tumours. Both cases were morphologically identical to giant cell tumour of bone. The giant cells stained strongly for acid phosphatase which was resistant to tartrate digestion, a staining reaction typical of osteoclasts. In view of the ability of urinary bladder to induce metaplastic and neoplastic bone, we believe that these tumours may represent extraosseous giant cell tumours of bone.
We describe a 75-year-old patient who presented with severe cholestatic liver disease. He had been receiving methyldopa, 250 mg daily, for 6 years. An extensive evaluation failed to reveal extrahepatic obstruction or serologic evidence of viral hepatitis. A liver biopsy disclosed marked cholestasis, without hepatitis, and was compatible with a rare form of methyldopa-induced liver injury. Cessation of drug treatment was followed by a slow but complete recovery. Cholestasis is a rare manifestation of methyldopa hepatotoxicity. Although methyldopa-associated liver injury usually appears after about 4 weeks of treatment, a history of a much longer exposure does not exclude this entity.
A 76-yr-old woman complained of right lower quadrant abdominal pain, nausea, and fever. There was no history of antibiotic therapy or preceding diarrhea. At laparotomy, a cecal lesion simulating a tumor was found with an otherwise normal colon. Right hemicolectomy was performed. Pathological examination showed pseudomembranous colitis confined to the cecum. It is suggested that pseudomembranous colitis unrelated to prior exposure to antimicrobial agents can occur without diarrhea, and may be a localized process mimicking colonic carcinoma.
An infant with microcephaly and generalized cytomegalovirus infection presented with two unusual complications: cystadenoma of the pancreas and minimal change nephrotic syndrome (MCN). Possible pathogenetic mechanisms of these conditions are discussed.