Giant renal cell carcinoma: a case report

Though renal cell carcinoma is a fairly common disease, it is extremely rare to encounter a case providing a resected mass as large as over 2,000 g in weight. We report a case in which a giant 2,200 g tumor was resected after renal arterial embolization and interferon treatment for reduction of its size. A forty-three-year-old woman was first seen at a certain hospital with a chief complaint of fever and right flank pain. Under suspicion of right renal tumor, nephrectomy by extraperitoneal flank incision was attempted. However, the tumor was too large to be resected. After transfer to our department, she was treated by renal arterial embolization with absolute ethanol and steel coil and by the administration of interferon (rIFN-alpha A). As the tumor shrank to 77% of its original size on CT measurements and became mobile, transperitoneal nephrectomy was performed. The resected mass was as large as 12 x 12 x 21 cm. Pathologically, it was a clear cell type tumor showing mucinous degeneration and hyaline degeneration of tumor cells. Interferon used amounted to as high as 864 x 10(6) units, but no particular side effect occurred. No recurrence has been detected so far.     

Gene locus for autosomal recessive distal myopathy with rimmed vacuoles maps to chromosome 9

Distal myopathy with rimmed vacuoles is an autosomal recessive muscular disorder, characterized clinically by weakness of the distal muscles in the lower limbs in early adulthood. Recently, the gene locus for familial vacuolar myopathy with autosomal recessive inheritance (hereditary inclusion body myopathy) was mapped to chromosome 9 by genome-wide linkage analysis of nine Persian-Jewish families. Since both disease conditions share similar clinical, genetic, and histopathological features, we analyzed seven families with distal myopathy with rimmed vacuoles using ten microsatellite markers within the region of the hereditary inclusion body myopathy locus. Significantly high cumulative pairwise lod scores were obtained with three markers: D9S248 (Z_max = 5.90 at Θ = 0), D9S43 (Z_max = 5.25 at Θ = 0), and D9S50 (Z_max = 4.23 at Θ = 0). Detection of obligate recombination events as well as multipoint linkage analysis revealed that the most likely location of the distal myopathy with rimmed vacuoles gene is in a 23.3-cM interval defined by D9S319 and D9S276 on chromosome 9. The results raise the possibility that distal myopathy with rimmed vacuoles and hereditary inclusio body myopathy in Persian Jews are allelic diseases.     

Intrarenal vascular sites of action of adenosine and glucagon

Our purpose was to localize the intrarenal vascular sites of action of adenosine and glucagon. Renal blood flow (RBF) and glomerular filtration rate (GFR) were measured in anesthetized dogs, and renal perfusion pressure (RPP) was varied by an adjustable aortic clamp. At normal RPP, RBF was increased by all agents. In contrast, GFR was increased by glucagon, decreased by adenosine and unchanged by acetylcholine (ACh) or adenosine plus glucagon. The increases in RBF by glucagon occurred only at RPPs within the autoregulatory pressure range, and renal autoregulatory capability was attenuated during the infusion of glucagon. In contrast, adenosine increased RBF at RPPs both within and below the autoregulatory pressure range, and the autoregulatory capability was not perceptibly impaired. Superimposition of glucagon to adenosine caused further vasodilation, and the autoregulatory efficiency was completely attenuated. There was no difference between the RPP-RBF or RPP-GFR relations obtained during infusion of adenosine plus glucagon and ACh, which dilates both the afferent and efferent arterioles. It is generally accepted that afferent arteriolar resistance attains a minimum value at RPP near the lower limit of the autoregulatory range. Thus, our data indicate that glucagon and adenosine preferentially dilate the afferent arteriole and the efferent arteriole, respectively.     

Circulating promyelocytes and low levels of CD16 expression on polymorphonuclear leukocytes accompany early-onset periodontitis.

Early-onset periodontitis (EOP) is characterized by rapidly progressive alveolar bone loss, chemotactic defects of neutrophils, and significant familial aggregation. We found immature myeloid lineage cells, defined as promyelocytes, in the peripheral blood in patients with EOP. A hematological examination of peripheral blood cells showed normal reference values regarding cell proportions. Flow cytometry revealed significantly lower expression of CD16, a glycosylphosphatidylinositol (GPI)-anchored protein, on peripheral neutrophils in patients compared with those in age- and sex-matched healthy controls, whereas the levels of CD11a and CD11b expression were similar. The chemotactic response of neutrophils was lower toward not only formyl-methionyl-leucyl-phenylalanine but also complement fragment C5a than that of healthy controls. The expression of another GPI-anchored protein, CD14, was equally expressed by controls and patients. Therefore, the low level of CD16 expression was not due to the incomplete synthesis of the GPI anchor. GPI anchors of CD16 on neutrophils from controls and patients were both partially resistant to phosphatidylinositol-specific phospholipase C. The presence of promyelocytes in peripheral blood, low expression of CD16, and low chemotactic response of neutrophils suggest that patients with EOP have an abnormal maturation system in myeloid lineage cells in the bone marrow, which may be associated with the onset and course of EOP.     

Acquired Fatty Replacement of the Body and Tail of the Pancreas Diagnosed by Endoscopic Ultrasonography —A Case Report—

Abstract: A 62-year old female was admitted for a detailed study of a pancreatic abnormality detected by abdominal ultrasonography. A CT and MRI revealed a fat deposition in the body and tail of the pancreas. An ERCP demonstrated the distal main pancreatic duct as 2 fine branches in the head, and accessory pancreatic ducts were visualized. An endoscopic ultrasonography revealed a swelling of the body. In the tail, a membrane-like hyperechoic structure was noted, and probably represented the pancreatic capsule. A angiography demonstrated branches of the dorsal and transverse pancreatic artery, and the diagnosis of acquired fatty replacement of the body and tail of the pancreas was confirmed. Endoscopic ultrasonography appears to be a useful method to confirm fatty replacement of the body and tail of the pancreas.     

Full flexion after total knee replacement in rheumatoid arthritis

Summary The results of the Y-S total knee replacement are reported in 152 patients with rheumatoid arthritis (227 knees). The follow up was from 2 to 4 years (average 2.7 years). The good range of motion generally achieved was attributed to the use of a calibrated distractor for soft tissue balancing, a tibial prosthesis with a flattened plateau and extensive postoperative rehabilitation. Fourteen patients (27 knees) were able to squat fully after operation. A good range of motion before operation was related to a good range after operation. The range and overall results did not differ in patients with rheumatoid arthritis or osteoarthritis. The knees which recovered full flexion were no different from those with less than full flexion as far as stability and radiographic findings were concerned.

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Résumé Les auteurs rapportent les résultats de 227 prothèses de genou Y. S. chez 152 malades atteints d'arthrite rhumatoïde avec un recul de 2 à 4 ans (moyenne 2, 7 ans), la bonne amplitude de mouvements obtenue est attribuée à l'utilisation d'un distracteur calibré pour un bon équilibre des parties molles, à un plateau tibial prothétique plat et à une rééducation post-opératoire intensive. Une bonne mobilité préopératoire est un facteur de bonne mobilité post-opératoire. L'amplitude des mouvements et les résultats globaux sont les mêmes dans les arthrites rhumatoïdes et dans les ostéoarthrites. La stabilité et l'aspect radiologique étaient analogues que les genoux aient récupéré une flexion complète ou non.