Luc’s abscess as an unlucky complication of mastoiditis

Luc’s abscess is a rare but important complication of acute otitis media (AOM), whereby infection spreads from the middle ear, resulting in a subperiosteal collection beneath the temporal muscle. Unlike other extracranial abscesses relating to AOM, Luc’s abscess is not believed to involve the mastoid bone. We present the case of a patient with a Luc’s abscess with mastoid involvement and discuss its successful management. We believe that patients presenting with a subperiosteal collection beneath the temporal muscle and mastoiditis may represent a different group of patients to those described originally by Luc. These individuals can be differentiated using computed tomography (CT) of the temporal bones. We advocate CT in patients with Luc’s abscess and AOM; this aids preoperative surgical planning.     

Endoscopic management of postoperative bile leak

Significant bile leak is an uncommon but serious complication of biliary tract surgery. Of twenty-five patients presenting with postoperative bile leak, 11 had complete tie-off of common bile duct and required surgery, while the remaining 14 had injury without complete obstruction and could be managed by endoscopic methods. Of these 14 cases, bile leak occurred from the cystic duct in 11 patients and from the common hepatic duct, right hepatic duct and left hepatic duct in one patient each. Endoscopic procedures performed included sphincterotomy alone (four patients), sphincterotomy and stent placement (seven patients) and sphincterotomy followed by nasobiliary catheter drainage (three patients). There was no technical failure and bile leak was stopped in all patients. One patient died of haemobilia 5 days after stent placement. When technically feasible, postoperative bile leak can be managed safely and effectively by endoscopic methods, obviating the need for surgical reexploration.     

An immunocytochemical study of the distribution of proline-4- hydroxylase in normal, osteoarthritic and rheumatoid arthritic synovium at both the light and electron microscopic level

The monoclonal antibody 5B5 reacts with the beta subunit of proline-4- hydroxylase, the enzyme which catalyses the formation of 4-hydroxyl proline in collagen and other proteins with collagen-like amino acid sequences. This study aims to assess the production and tissue distribution of this enzyme in normal and diseased synovia from patients with various joint diseases, on the basis that it is a putative marker of collagen-producing cells and, therefore, in this context, of fibroblasts. Sections from five normal, 10 osteoarthritic (OA) and 26 rheumatoid arthritic (RA) synovia were labelled with a mouse monoclonal antibody to proline-4-hydroxylase. The enzyme was found to be expressed by a proportion of synovial intimal cells and by fibroblasts in the underlying connective tissue in normal, OA and RA synovia. Labelling was more pronounced in OA and RA cases. The intimal cells labelling positively showed type B synoviocyte morphology, which was confirmed by subsequent double immunolabelling with 5B5 and antibody against type IV collagen using immunocytochemistry and immunoelectron microscopy.      

Randomized comparison of busulfan and hydroxyurea in chronic myelogenous leukemia: prolongation of survival by hydroxyurea. The German CML Study Group

In a randomized multicenter study the influence of hydroxyurea versus busulfan on the duration of the chronic phase and on survival of chronic myelogenous leukemia (CML) was determined. In addition cross resistance and adverse reactions of the drugs were analyzed. From July 1983 to January 1991, 441 CML patients were randomized to receive hydroxyurea or busulfan. Of these, 90.7% were Philadelphia positive; 25.7% were low, 38.2% intermediate, and 36.2% high risk patients according to Sokal's score. The median survival of the busulfan treated Philadelphia-positive patients is 45.4 months and of the hydroxyurea group 58.2 months (P = .008). The survival advantage for the hydroxyurea treated patients is recognized in all risk groups. Sixty four patients reached therapy resistance before blast crisis and were crossed over to the alternative drug. The 23 patients with primary hydroxyurea had a median survival of 5.6 years, the 41 patients with primary busulfan therapy a median survival of 2.7 years (P = .02). Adverse reactions were less frequent with hydroxyurea with no severe adverse effects (lung fibrosis, long lasting bone marrow aplasia). The analysis of white blood cell counts in the course of treatment showed lower counts in the hydroxyurea patients. We conclude that hydroxyurea is superior to busulfan in therapy of CML in chronic phase and should be used as first line therapy. Busulfan may have a role as secondary therapy after hydroxyurea resistance or intolerance.     

Comparison of the effects of diuretic therapy and low sodium intake in isolated systolic hypertension

Of 103 patients with isolated systolic hypertension, 71 were treated with diuretics and another 32 with low-sodium diet. In the 71 who were treated with diuretics, body weight decreased from 69.48 ± 1.47 to 68.60 ± 1.45 kg (p < 0.0005) and systolic blood pressure from 178 ± 2 to 152 ± 2 mm Hg (p < 0.0005). Plasma renin activity increased from 1.78 ± 0.30 to 7.32 ± 1.78 ng/ml per hour (p < 0.005) and urinary aldosterone from 10 ± 1 to 23 ± 4 μg per 24 hours (p < 0.005). The greatest decrease in systolic blood pressure occurred in patients in the low-renin group (?32 ± 2 mm Hg), whereas it decreased by 24 ± 2 mm Hg (p < 0.04) in the normal-renin group; however, blood pressure did not change significantly in the high-renin group. In the 32 patients who were treated with low-sodium diet, the 24-hour urinary sodium excretion decreased from 143 ± 10 to 48 ± 5 meq (p < 0.005), body weight decreased from 71.18 ± 2.50 to 70.17 ± 2.47 kg (p < 0.005), systolic blood pressure decreased from 174 ± 2 to 156 ± 3 mm Hg (p < 0.0005), and diastolic blood pressure decreased from 90 ± 1 to 87 ± 1 mm Hg (p < 0.01). Plasma renin activity increased from 2.25 ± 0.33 to 4.27 ± 0.43 ng/ml per hour (p < 0.005) and urinary aldosterone from 9 ± 1 to 15 ± 2 μg per 24 hours (p < 0.005). The decrease in the systolic blood pressure was related to the pretreatment 24-hour urinary sodium excretion (r = 0.40, p < 0.05). The smallest decrease in systolic blood pressure occurred in the patients with high renin values (?1 ± 9 mm Hg, n = 5), whereas the decrease in systolic blood pressure in the low-renin (n = 12) and normal-renin groups (n = 15) was similar, ?22 ± 2 mm Hg and ?21 ± 3 mm Hg, respectively (p < 0.005 compared with the high-renin group). These results indicate that both diuretic therapy and low-sodium diet are effective antihypertensive means in most patients with isolated systolic hypertension and low or normal plasma renin activity.     

整合蛋白α5亚基表达与肝癌恶性表型

目的探讨整合蛋白α５亚基与原发性肝癌的关系。方法应用免疫组化技术（ＡＢＣ法）和Ｎｏｒｔｈｅｒｎｂｌｏｔ杂交检测整合蛋白α５亚基在原发性肝癌中的表达。结果发现在７９例癌与癌周组织α５阳性率分别为３２．９％和８１．０％，两者间差异有显著性（Ｐ＜０．０１）。直径≤５ｃｍ的肝癌α５阳性率高于直径＞１０ｃｍ的肝癌（５５．６％比１０．０％，Ｐ＜０．０１），分化较好的肝癌α５阳性率高于分化不良者（４０．６％比１６．０％，Ｐ＜０．０５），已发生明确肝内转移（包括肝内播散和门静脉癌栓形成）的肝癌α５阳性率低于未发生肝内转移者（２０．６％比４２．２％，Ｐ＜０．０５）。α５亚基表达与患者年龄、血清甲胎蛋白水平、乙型肝炎病毒感染、肝硬化有无等因素均无明显相关（Ｐ＞０．０５）。Ｎｏｒｔｈｅｒｎｂｌｏｔ杂交结果也同时显示，非侵袭性肝癌α５表达高于侵袭性肝癌。结论整合蛋白α５低表达与肝癌增大、分化程度低、侵袭转移发生等恶性表型相关，可能对这些恶性表型起负性调节作用。     

Molecular analysis of clonality and bcr rearrangements in Philadelphia chromosome-positive acute lymphoblastic leukemia

Philadelphia chromosome (Ph1)-positive chronic myelogenous leukemia (CML) patients consistently show a rearrangement in a 5.8-kilobase length of chromosome 22, referred to as the breakpoint cluster region (bcr). In Ph1-positive acute lymphoblastic leukemia (ALL), the breakpoint in chromosome 22 is more heterogeneous and, in some instances, does not occur within this region. In such cases the cell of origin of the neoplastic clone and the relationship of the disease to CML has remained obscure. We have analyzed the bcr rearrangement in the malignant cells from three patients who presented with Ph1-positive ALL and who in cytogenetic studies had shown evidence of variable involvement of myeloid cells in the Ph1-positive clone. Rearrangements in bcr typical of most cases of CML were detected in purified granulocyte preparations from two of the ALL patients (nos. 1 and 2) and in the blasts from patient 3 at the time of her terminal relapse. In the same analysis the simultaneously obtained granulocytes from patient 3, however, did not show any evidence of bcr rearrangement. Patient 3 was also heterozygous for the BamHI polymorphism in the X- linked hypoxanthine phosphoribosyltransferase (HPRT) gene, thus permitting a different method of clonal analysis based on methylation differences in active and inactive alleles. When DNA from her granulocytes that had shown no bcr rearrangement was hybridized to an HPRT probe, a pattern typical of a polyclonal population was seen. A similar pattern was exhibited by her marrow fibroblasts. In marked contrast, her simultaneously isolated blasts showed an unambiguous monoclonal pattern. These findings demonstrate the origin of the disease in the first two patients in a cell with myelopoietic as well as lymphopoietic potential and confirm the restricted lymphoid cell origin of the neoplastic clone in the third Ph1-positive ALL patient. Furthermore, they indicate that different target cells for transformation within the hematopoietic system may be affected by very similar bcr rearrangements.     

A rapid assay for evaluation of iron-chelating agents in rats

The animal assay of potential new iron-chelating agents is at present dependent on cumbersome and imprecise iron balance studies in hypertransfused rodents. We report the development of a radioisotope assay in intact rats based on the transient labeling by ferritin 59Fe of the main source of chelatable iron within hepatocytes. The isotope was maximally available to chelators during the first 6 hr after its injection, nearly all the excretion being in the bile. The bile 59Fe/total iron ratio was independent of both the chelator and its dose. However, in iron-loaded rats, the ratio was reduced, and the isotope excretion was a less sensitive measure of intrahepatic chelation. In the proposed assay, test chelators were given to normal rats 2 hr after an intravenous injection of 59Fe-ferritin. Four hours later, the radioiron in the liver and in the gut gave a sensitive measure of the mobilization of hepatic iron to the bile. In addition, chemical iron determinations identified a small alternative source of urinary chelate with agents known to promote urine excretion in man. The assay gave a rapid and precise screen for chelators given by parenteral and oral routes.     

Clinical and genetic analysis of 18 pancreatic carcinoma/melanoma‐prone families

Bartsch DK, Langer P, Habbe N, Matthäi E, Chaloupka B, Sina M, Hahn SA, Slater EP. Clinical and genetic analysis of 18 pancreatic carcinoma/melanoma‐prone families. Families with both melanoma and pancreatic cancer are extremely rare and some are affected with the autosomal dominant inherited familial atypical multiple mole melanoma‐pancreatic cancer (FAMMM‐PC) syndrome. The phenotypic and genotypic expressions of such pancreatic cancer–melanoma prone families are not well defined. The National Case Collection of Familial Pancreatic Cancer of the Deutsche Krebshilfe includes 110 pancreatic cancer families, 18 of which (16%) show an association of pancreatic cancer and melanoma. These 18 families were analysed regarding their phenotype and the prevalence of germline mutations in the candidate genes CDKN2A, BRCA2, CHEK2, NOD2, ARL11 and Palladin (PALLD). There were two types of families: five families with the FAMMM‐PC phenotype and 13 PC/melanoma families without the multiple mole phenotypes (PCMS). The prevalences of PC and melanoma in the two types of families were similar. The prevalence of other tumour types, especially breast carcinoma, was higher (11%) in PCMS‐ than in FAMMM‐PC families (2.4%, p = 0.02). CDKN2A mutations were identified in 2 of 18 (11%) PCMS families. A cosegregating BRCA2 mutation was detected in one PCMS family without breast cancer. None of the reported germline mutations in the NOD2, Palladin, ARL11 or CHEK2 genes were detected in either type of family. In conclusion, families with an accumulation of PC and melanoma show a large variety of phenotypic expression, which is not always consistent with the FAMMM‐PC phenotype. More PC/melanoma‐prone families need to be analysed to clarify whether such families represent variations of the FAMMM‐PC syndrome or two distinct hereditary cancer syndromes.