The diagnostic value of planar and SPET scintimammography in different age groups

Planar scintimammography (SMM) with 99Tcm-sestamibi (99Tcm-MIBI) has proved to be a useful method in the evaluation of breast lesions. The aim of our study was to determine the diagnostic value of planar and single photon emission tomograpic (SPET) SMM in different age group. We investigated women with breast lesions for which the surgeon recommended biopsy or careful follow-up. Seventy consecutive patients underwent supine planar SMM, while supine SPET was performed in 68 of the patients. Twenty-seven carcinomas in 23 patients and 35 benign lesions in 31 patients were confirmed histologically. In the remaining 16 patients, the findings were classified as benign at follow-up. The carcinomas and benign lesions had a mean (+/- S.D.) diameter of 25 +/- 17 and 17 +/- 11 mm respectively (P < 0.05). A sensitivity/specificity of 67/96% and 88/91% was obtained with the planar and SPET techniques respectively. The accuracy of both techniques was 90%. In patients aged 40 years or less, SPET increased the sensitivity from 50 to 100% (P < 0.01), whereas it decreased the specificity and accuracy from 94 to 81% and 91 to 81% respectively (N.S.). In patients aged 41-50 years, the sensitivity increased from 63 to 100% (P < 0.01), whereas there was a small decrease in specificity and an increase in accuracy (from 91 to 89% and 86 to 91% respectively, N.S.) We conclude that, in younger patients, a negative SPET SMM study indicates the need for careful follow-up rather than biopsy. However, the addition of SPET in supine scintimammography does-not change the overall accuracy significantly.     

DNA adducts, mutant frequencies and mutation spectra in lambda lacZ transgenic mice treated with N-nitrosodimethylamine

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an increasingly recognized autosomal dominant disorder that leads to cerebrovascular manifestations in early adulthood. This study delineates the phenotypic spectrum and the natural history of the disease in 102 affected individuals from 29 families with biopsy-proven CADASIL. Recurrent ischemic episodes (transient ischemic attack [TIA] or stroke) were the most frequent presentation found in 71% of the cases (mean age at onset, 46.1 years; range, 30-66 years; SD, 9.0 years). Forty-eight percent of the cases had developed cognitive deficits. Dementia (28%) was frequently accompanied by gait disturbance (90%), urinary incontinence (86%), and pseudobulbar palsy (52%). Thirty-nine patients (38%) had a history of migraine (mean age at onset, 26.0 years; SD, 8.2 years), which was classified as migraine with aura in 87% of the cases. Psychiatric disturbances were present in 30% of the cases, with adjustment disorder (24%) being the most frequent diagnosis. Ten patients (10%) had a history of epileptic seizures. To delineate the functional consequences of ischemic deficits, we studied the extent of disability in different age groups. The full spectrum of disability was seen in all groups older than age 45. Fifty-five percent of the patients older than age 60 were unable to walk without assistance. However, 14% in this age group exhibited no disability at all. Kaplan-Meier analysis disclosed median survival times of 64 years (males) and 69 years (females). An investigation of the 18 multiplex families revealed marked intrafamilial variations.     

Binding of properdin to solid-phase immune complexes: critical role of the classical activation pathway of complement

OBJECTIVE: Our objectives were to assess the clinical and microbiological aspects of septic osteoarthritis in children admitted to our center from 1987 until July 1997 and to determine the sensitivity of ultrasound in this age group. PATIENTS AND METHODS: The medical records of 36 children diagnosed as having septic osteoarthritis of the hip were reviewed retrospectively. The diagnosis had been based on clinical criteria, along with synovectomy and drainage of purulent material from the affected joint. An X-ray and/or ultrasound had been performed when the diagnosis was suspected. RESULTS: Nineteen children were diagnosed during the neonatal period, 8 between the ages of 1 and 12 months and 9 older than one year of age. Mean age at diagnosis was 16.8 +/- 6.2 months (median 29 days, range 6 days to 13 years). The hip was involved in 32 children, the ankle in 3 and the elbow in 1. A microbiological diagnosis was achieved in 22 cases (61%) by culture from blood, CSF, and/or synovial fluid. The most common isolates were Gram positive cocci (S. aureus in 9 cases and coagulase negative Staphylococcus in 3). The diagnostic value of the X-rays was very low (18%). The ultrasound was initially considered abnormal in 64.5% of the patients, with a lower sensitivity in the neonatal period. After a mean follow-up period of 36 months, the outcome was good in 86% of the cases, although three children continue with sequelae. Two preterm infants died due to sepsis associated with the osteoarthritis. CONCLUSIONS: At the time of diagnosis of septic arthritis of the hip, the ultrasound is frequently normal. Due to the poor outcome when there is a delay in surgery, we suggest immediate synovectomy and drainage when there is clinical suspicion of septic arthritis despite an apparently normal ultrasound.     

Alleles of the alpha-1-antitrypsin phenotype in patients with aortic aneurysms

Prader-Willi syndrome (PWS) and Angelman syndrome (AS) are distinct neurodevelopmental disorders with interrelated genetic mechanisms because genomic imprinting within the chromosome 15q11-13 region affects both the PWS and the AS locus. Methylation analysis is one method of distinguishing between the maternally and paternally inherited chromosome 15. Here we present clinical and molecular data on a large series of 258 referred patients, evaluated with methylation analysis: 115 with suspected PWS and 143 with suspected AS. In these patients, the clinical phenotype was graded into three groups: classical (group 1); not classical but possible (group 2); not classical and unlikely (group 3). For PWS, a fourth group consisted of hypotonic babies. DNA methylation analysis confirmed the diagnosis of PWS in 30 patients (26%) and AS in 28 patients (20%). For 21 PWS patients the mechanism was established: 15 had deletions, 4 had uniparental disomy (UPD) and 2 a presumed imprinting defect. Clinically all those with an abnormal methylation pattern had the classical phenotype and none of those with a normal methylation pattern had classical PWS. For 23 AS patients in whom a mechanism was established, 17 had a deletion, 3 had UPD and 3 had a presumed imprinting defect. There was greater clinical overlap in AS, with 26 classical AS patients having a normal methylation pattern while an abnormal methylation pattern was seen in one patient from group 2. In addition, there were a further 40 patients with a normal methylation pattern in whom AS was still a possible diagnosis. Our conclusion is that methylation analysis provides an excellent screening test for both syndromes, providing approximately 99% diagnosis for PWS and for AS, a 75% diagnostic rate, supplemented for the remaining 25% with an essential basic starting point to further investigations.     

Transduction of non-dividing adult human pancreatic beta cells by an integrating lentiviral vector

This study assesses the long-term (mean 52+/-24 months) performance of the St. Jude Medical (SJM) valve in 200 young (mean age 31+/-13 years) rheumatic patients on low-level warfarin anticoagulation combined with dipyridamole. Follow-up was 95% complete and comprised 867 patient-years. There were 33 deaths (3.8%/patient-year). Death was valve related in 12 cases and due to left ventricular dysfunction in 10. Death due to left ventricular dysfunction occurred earlier after surgery than death due to other causes (10+/-7 vs 29+/-18 months, p <0.005); these patients had larger preoperative left ventricular dimensions than the rest of the group (end-systolic diameter 51+/-13 vs 37+/-16 mm, end-diastolic diameter 66+/-13 vs 50+/-19 mm, p = 0.006). Actuarial probability of survival was 81% at 86 months and probability of event-free survival was 71%. The median international normalized ratio was 1.88+/-0.54. Thromboembolism (13 events) occurred at a linearized rate of 1.5%/patient-year. There were 11 major bleeding episodes (1.3%/patient-year), 4 cases of prosthetic valve endocarditis (0.8%/patient-year), and 12 paraprosthetic leaks (1.4%/patient-year). No valve obstructions or reoperations occurred. Thus, the SJM valve performs well on low-level anticoagulation combined with dipyridamole. Left ventricular dysfunction was a common cause of death in the early postoperative period.     

Long-term followup of all patients with muscle invasive (stages T2, T3 and T4) bladder carcinoma in a geographical region

PURPOSE: We studied the relationship between long-term survival and treatment of stages T2, T3 and T4 bladder carcinoma in an unselected patient population. MATERIALS AND METHODS: A total of 680 patients with the initial diagnosis of bladder carcinoma in 1987 to 1988 in Western Sweden was prospectively registered and followed until 1994. Of these patients 107 had stage T2 to T3 and 41 had stage T4 disease. RESULTS: Of the patients with stage T2 to T3 disease 30 (mean age 66) underwent radical cystectomy, 33 (mean age 75) full dose radiotherapy and 44 (mean age 81) nonradical therapy (mainly transurethral resection of the bladder). The 5-year crude survival rates were 33, 15 and 14%, respectively. Of the patients with stage T4 disease 6 (mean age 61) underwent radical cystectomy, 9 (mean age 73) full dose radiotherapy and 26 (mean age 81) nonradical therapy (mainly transurethral resection of the bladder). All except 1 patient died of disease within 4 years. CONCLUSIONS: More than 60% of the patients in the cohort were considered unsuitable for radical cystectomy and their survival was poor, whether treated with full dose radiotherapy or transurethral resection of the bladder alone.     

A second-generation cementless hip prosthesis: improved results over the first-generation prosthesis

Forty-two patients who had a Porous Coated Anatomic (PCA) "E" series, second-generation, cementless hip arthroplasty (Howmedica, Rutherford, NJ) were compared with 42 patients who had a first-generation PCA prosthesis. Patients were directly matched for age, sex, diagnosis, weight, Charnley functional status, and duration of follow-up. All of the operations were done by the same two surgeons, who used the same operative approach and the same postoperative rehabilitation plan. All of the patients were followed up for at least 5 years (range, 60 to 76 months). In the "E" series group, there were 41 of 42 (98%) good and excellent clinical results with a mean Harris hip score of 94 points (range, 46 to 100 points); the first-generation group had 34 of 42 (81%) good and excellent clinical results and a mean Harris score of 81 points (range, 42 to 100 points) (P = 0.001). There was one acetabular component revision in the "E" series group (2%), which can be compared with eight revisions (19%) in the first-generation group (P = 0.012). The incidence of femoral radiolucencies was 19% (eight hips) for the "E" series group compared with 50% (21 hips) in the first-generation group (P = 0.009). The radiolucencies in the "E" series group were small, nonprogressive, and confined typically to zone I. We believe that the improvements in design of the "E" series component may account for these differences.     

Solar UVR exposures of primary school children at three locations in Queensland

OBJECTIVES: To study mortality and survival of patients with myasthenia gravis. METHODS: 290 patients with myasthenia gravis were studied, including 212 incident cases identified during a comprehensive epidemiological study of myasthenia gravis in western Denmark 1975-89. Follow up was performed on 31 December 1994. Survival curves were constructed using the life table method. Patient data were compared with data from the public Danish population statistics. Death certificates were provided from the National Registry of Death. RESULTS: The annual average crude mortality rate was 1.8 per million (range 1.5-2.2). The myasthenia gravis related mortality rate (myasthenia gravis as underlying or contributory cause) was 1.4 per million (range 1.1-1.8). The age specific mortality rates were low below 50 years. After this age the mortality increased with age in both sexes; after 60 years more rapidly in men than in women. The overall survival rates three, five, 10, and 20 years from diagnosis were 85%, 81%, 69%, and 63% respectively. The survival of both sexes was shorter than that of the corresponding Danish population. Old age at diagnosis, a classification in Osserman-Genkins group IIB or III, and the presence of a thymoma were associated with a less favourable prognosis. The three, five, 10, and 20 year survival rates of thymectomised patients were 94%, 94%, 86%, and 79% respectively. The corresponding figures for the non-thymectomised patients were 78%, 71%, 56%, and 51%. A Cox regression analysis showed that this apparently significant effect of thymectomy was because the thymectomised patients were younger than the non-thymectomised patients. Furthermore, at the time of diagnosis of myasthenia gravis the non-thymectomised patients had a higher frequency of serious conditions associated with myasthenia gravis than the thymectomised patients. CONCLUSION: Patients with myasthenia gravis generally have a relative good prognosis although their survival is shorter than that of the corresponding population. Old age, a classification in Osserman-Genkins group III, and the presence of a thymoma are associated with a less favourable prognosis. In this study, the apparently significant effect of thymectomy was because the thymectomised patients were younger than non-thymectomised patients and because the non-thymctomised patients had a higher frequency of serious conditions associated with myasthenia gravis.     

Serotonin function and treatment response to clozapine in schizophrenic patients

OBJECTIVE: Clozapine is the only compound proven to be effective in the 20% of schizophrenic patients refractory to treatment with conventional neuroleptics. Although its mechanism of action has not been elucidated, clozapine appears, in contrast to most conventional neuroleptics, to be a potent serotonin (5-HT) antagonist. This study hypothesized that 5-HT function is increased in patients who benefit from clozapine treatment relative to patients who fail to improve on it. METHOD: The 5-HT receptor agonist m-chlorophenylpiperazine (MCPP) was used as a probe to examine 5-HT function. MCPP (0.35 mg/kg p.o.) was administered in a placebo-controlled design after a 3-week drug-free period to 19 schizophrenic patients. ACTH, prolactin, body temperature, behavior, and MCPP blood level were measured. Patients were then treated with a conventional neuroleptic, and, having failed to respond to it, were treated with clozapine for 5 weeks (up to 600 mg/day). RESULTS: Patients who responded to clozapine had significantly higher ACTH responses to MCPP during the drug-free state than the patients who failed to benefit from clozapine. Moreover, the degree of improvement with clozapine, particularly the improvement in psychotic symptoms, was strongly correlated with the magnitude of MCPP-induced ACTH release. Other MCPP-induced responses and MCPP blood level were similar for the two groups and did not correlate with the degree of symptomatic improvement with clozapine. CONCLUSIONS: Results of this study suggest that MCPP-induced ACTH release, and by inference 5-HT receptor function, may be increased in patients who benefit from treatment with clozapine relative to patients who fail to improve on this drug.     

Factors affecting length of hospitalization in a rheumatic disease unit

OBJECTIVE: To assess factors influencing length of stay in a 12-bed rheumatic disease unit of a university associated teaching hospital with secondary and tertiary care responsibilities for a geographically broad referral base. METHODS: Patient discharges over a 12-month period were studied and divided into 3 categories. RESULTS: In Category 1, 167 patients with a mean age of 47.12 had a hospital stay of 1-14 days. Within this group was a subgroup of 30 patients with systemic lupus erythematosus, mean age of 30.13 receiving iv pulse cyclophosphamide who had multiple admissions. Their omission elevated the mean age to 50.87. In Category 2, 81 patients with a mean age of 59, had a hospital stay of 15-28 days and in Category 3, 24 patients with a mean age of 50.9 were hospital stay of 15-28 days and in Category 3, 24 patients with a mean age of 50.9 were hospitalized more than 29 days. Principal diagnosis, major complications of disease, complications of procedure and treatment and concurrent nonrheumatologic pathologies were analyzed for each group. The presence of a large number of comorbid pathologies in Groups 1 and 2 relate to the tertiary referral nature of the practice. Category 2, the group with the higher mean age had more treatment related complications in the form of adverse reactions to 2nd line agents and nonsteroidal antiinflammatory drugs. The 24 patients in Category 3 showed an equal sex distribution and are noted for the presence of infectious agent arthritis, requiring longterm iv antibiotics, both as a principal diagnosis, and as a complication of other pathologies. Vasculitis as a complication of preexisting disease and as a principal diagnosis was also a feature of this group. CONCLUSION: Length of stay above the 14 day mean relates to disease severity and serious complications such as vasculitis or joint sepsis requiring prolonged treatment.     

Hepatitis C virus and liver diseases

Pigmented villonodular synovitis is an uncommon synovial disease which only rarely involves the hip. In a multicenter retrospective study, we identified 58 histologically-proven cases. There were 33 females and 25 males. Mean age at diagnosis was 38 years. In all but two cases, only one hip was involved; the right hip was affected somewhat more often (33 cases) than the left. Two patients probably had bilateral hip disease. Mean delay to diagnosis was four years. Pain was the presenting symptom in most cases. A palpable mass in the groin was found in six patients. Plain roentgenograms were considered normal in only three patients. Bony cysts were seen in 39 patients and kissing cysts in 19. Joint space narrowing was found in 40 patients and was diffuse in half the cases. Roentgenograms suggested pigmented villonodular synovitis in 63% of cases, osteoarthritis of the hip in 16%, and inflammatory hip disease in 14%. Additional imaging studies included opaque arthrography in 21 subjects, computed tomography in 23, magnetic resonance imaging in 11, and arthroscopy in 9. Initial treatment was osmic acid synoviorthesis in 14 patients, partial synovectomy in 9, and total synovectomy in 21; in addition, eight patients had insertion of a cup prosthesis and 13 had total arthroplasty of the hip. Treatment was successful in 65% of cases after a mean follow-up of three years; among the 35% of failures, there were seven recurrences (14%). Total hip arthroplasty was performed secondarily in nine patients. This study illustrates the diversity of roentgenological changes in pigmented villonodular synovitis of the hip and the high frequency of osteoarticular lesions precluding conservative treatment. Magnetic resonance imaging and/or arthroscopy should be used to establish the diagnosis at an early stage when conservative treatment with total synovectomy and synoviorthesis is most likely to be successful.     

A second primary malignancy in a cohort of patients with epithelial ovarian cancer--characteristics of diagnosis

OBJECTIVE: Our objective was to study the whooping cough cases diagnosed in a 15-year period in our hospital, referring to its clinical features, epidemiology and analytical findings. PATIENTS AND METHODS: A retrospective study based on 144 cases with a clinical diagnosis of whooping cough, from 1981 to 1995, was carried out. Special attention was paid to those cases with cultures positive for B. pertussis. RESULTS: Nasopharyngeal specimens for culture were taken in 119 cases. This was not carried-out in those with previous antibiotic therapy. Cultures were positive for B. pertussis in 46 patients (38.7%). The age varied between 20 days and 30 months. Twenty-one were female. The initial symptoms were: paroxysmal cough in 44 (95.7%), cyanosis in 34 (73.9%), inspiratory whoop in 27 (58.7%), respiratory distress in 12 (26.1%) and post-tussive vomiting in 10 (21.7%). Thirty-nine children (84.8%) of this group had not received any pertussis vaccine, the rest had received just the first dose. It had been contacted by persons having cough in 19 patients (41.3%). Leukocytosis (mean: 19,818/mm3), lymphocytosis (mean: 13,047/mm3) and high platelet count (mean 459,522/mm3) were common findings. CONCLUSIONS: Cultures were positive in 38.7% of the patients. There are similar findings by other authors. In this group, most of the children were unvaccinated, and less than 3 months of age. Typical symptoms of pertussis were observed in the majority of patients. In one forth of them respiratory distress was found, probably related to their young age. Vaccinated adults emerge as a contact group that should be studied.     

Serum concentrations of retinol, d-alpha-tocopherol and beta-carotene: effects of storage at -70 degrees C for five years

To investigate the effects of prolonged storage of serum samples at -70 degrees C on concentrations of micronutrients, we measured concentrations of retinol, d-alpha-tocopherol, and beta-carotene in serum samples drawn in 1986. We compared values we measured in 1991 to values we obtained in 1986, using the same analytical methods. The relative concentrations obtained in 1991 (mean +/- S.D.) were: retinol 99.7 +/- 12.6% (n = 23), d-alpha-tocopherol 100.7 +/- 6.4% (n = 19), and beta-carotene 103.4 +/- 13.7% (n = 28). Using these techniques of sample preparation and high-performance liquid chromatographic analysis, we found that the effects of storage of serum at -70 degrees C for five years appear insignificant in a small population of patients. However, we did identify clinically important changes in concentration (> 20% difference) in several individual subjects.     

Differential effects of anti-beta2-glycoprotein I and antiprothrombin antibodies on the anticoagulant activity of activated protein C

Antiprothrombin and anti-beta2-glycoprotein I (beta2-GPI) antibodies belong to the family of antiphospholipid (APL) antibodies and represent the phospholipid-dependent inhibitors of coagulation. They may be distinguished by analyzing the coagulation profiles generated by the comparison of the ratios of two coagulation tests, the Kaolin Clotting Time (KCT) and the dilute Russell's Viper Venom Time (dRVVT), commonly adopted for their diagnosis. The KCT profile is caused by antiprothrombin antibodies, whereas anti-beta2-GPI antibodies are responsible for the dRVVT coagulation profile. The presence of aPL antibodies is frequently associated with acquired resistance to activated Protein C (APC-R), but limited information is available regarding the role of the different antibodies in its development. We studied the time-course of activated Factor V (FVa) generation and inactivation in the plasma of 42 patients with well-defined phospholipid-dependent inhibitors of coagulation: 24 displayed the dRVVT coagulation profile, whereas the other 18 cases showed the KCT profile. In normal pooled plasma, the peak values of FVa (mean +/- standard deviation, [SD]: 16.307 +/- 4.372 U/mL) were reached in 4 to 5 minutes and an almost complete inactivation (0.088 +/- 0.123 U/mL) was obtained within 20 minutes. At this time point, values of residual FVa exceeding 2 SD the mean of controls (0.344 U/mL) were considered abnormal. Patients belonging to the KCT coagulation profile group reached the maximal amount of FVa in plasma (22.740 +/- 7.693 U/mL, P = not significant v controls) within 4 to 5 minutes; at 20 minutes, the residual amount of FVa in plasma ranged from 0 to 1.09 U/mL (0.293 +/- 0.298; P = .027), but it was found abnormal in only six of the 18 cases. The time-course of FVa in plasma of patients belonging to the dRVVT coagulation profile group differed from that of normal controls in that the peak values (10.955 +/- 5.092 U/mL) were reached at 10 minutes and the amount of residual FVa at 20 minutes ranged from 0.320 to 14.450 U/ml (2.544 +/- 3.580 U/mL; P = .0191 v normal controls and P = . 0114 v KCT group patients). Twenty of the 24 patients belonging to the dRVVT profile group had an abnormal inactivation of FVa (chi2 = 0.001 v KCT group patients). History of venous thrombosis was experienced by 15 patients: an abnormal rate of FVa inactivation was found in 11 of them (73%) versus 15 of the 27 cases without thrombosis (56%) (x2 = 0.2556). The effect of affinity-purified IgG phospholipid-dependent inhibitors of coagulation on the time-course of FVa generation and inactivation in normal plasma was also investigated. Anti-beta2-GPI, but not antiprothrombin antibodies, hampered the inactivation of FVa by endogenous APC, thus reproducing the behavior of the original plasmas. This effect was strictly beta2-GPI-dependent. In conclusion, our findings confirm that anti-beta2-GPI antibodies identify patients with phospholipid-dependent inhibitors of coagulation at increased risk of thrombosis and suggest acquired APC-R as a possible explanation of the pathogenesis of the thromboembolic events.     

Functional defects of Cx26 resulting from a heterozygous missense mutation in a family with dominant deaf-mutism and palmoplantar keratoderma

BACKGROUND: There is controversy about the impact on morbidity from delayed diagnoses of blunt hollow viscus injuries. A recent study suggested that the increased morbidity was primarily from delayed diagnosis of blunt duodenal injury (BDI). STUDY DESIGN: We studied the medical records from a 10-year period from June 1987 to June 1997 examining the data on 22,163 cases of blunt trauma. We assessed the incidence and consequences of delayed diagnoses of BDI, and identified preoperative factors associated with these delayed diagnoses. RESULTS: Thirty-five patients (0.2%) were identified in the retrospective study of the records from 22,163 blunt trauma patients to have sustained BDI. Of these, 25 patients (71%) were male. Ages ranged from 1 to 58 years (mean 18.8 years), and the predominant mechanism was motor vehicle accident in 18 patients (51%). Seven patients (20%) (group I) had a diagnostic delay of > 6 hours; 28 patients (80%) (group II) were diagnosed in < 6 hours. Six of the seven group I patients (86%) were evaluated initially with CT scans, and five (83%) showed findings suggestive of BDI. Among the 28 group II patients, 14 (50%) underwent initial diagnostic peritoneal lavage (DPL), and 14 (50%) had a CT scan. In seven of the group II patients (50%) who were initially evaluated by CT scan, there were findings suggestive of BDI. Diagnostic peritoneal lavage was initially equivocal (red blood cell count=5,000 to 100,000) in the remaining one group I patient compared with three of the group II patients who had DPL. Deterioration found on physical examinations prompted followup CT scans in 6 group I patients (86%), and the scans were diagnostic for BDI in all cases. CONCLUSIONS: Blunt duodenal injury is an uncommon entity. Despite the presence of suggestive CT and DPL findings, the diagnosis was delayed in 20% of the 35 patients whose records were examined in the study; this delayed diagnosis was associated with increased abdominal complications. Patients with persistent abdominal complaints and equivocal CT or DPL findings should undergo laparotomy or repeat CT scan evaluations.     

Study of 86 cases of Mediterranean boutonneuse fever hospitalized at a university hospital

OBJECTIVES: To study the clinical manifestations of 86 patients with Mediterranean Boutonneuse Fever who were admitted in a University General Hospital. PATIENTS AND METHODS: Between 1986 and 1994 we studied retrospectively the clinical manifestations, evolution and complications of 86 patients with Mediterranean Boutonneuse Fever. Diagnosis was based on clinical and serological findings. RESULTS: We studied a total of 86 patients (64 males; 22 females) with a mean age of 55 years. 88% of cases were diagnosed between June and September, and 89% of them had contact with dogs. 53% of patients had an underlying disease. All patients presented with fever and a generalized erythematous rash. 60% of patients had a initial lesion (tache noire) especially in legs. All patients were treated with doxicycline during one week. 22% of patients had complications such as renal failure, respiratory failure, gastrointestinal bleeding and stroke. Old patients and those with underlying disease had severe complications with a higher significant frequency. No patients died. CONCLUSIONS: 22% of patients with Mediterranean Boutonneuse Fever, especially those with advanced age or underlying disease, who were admitted in the Hospital had severe complications.     

Methods in the evaluation of medical students]

BACKGROUND: Delayed diagnosis, a high rate of histologically undifferentiated types of tumors, and rapid disease progression are frequently cited as the main reasons for the poor prognosis of gastric cancer in young patients. An improved prognosis has been anticipated for young gastric cancer patients because of recent improvements in digestive tract diagnostic techniques. This retrospective study was designed to determine whether these trends have had an impact on young Japanese patients with gastric cancer, and to further elucidate differences in clinicopathologic features between elderly and young patients. METHODS: From 1984 to 1995, 1654 patients with gastric cancer were admitted to our hospital. Of these, 86 patients (5.2%) were less than 40 years of age (young group). The clinicopathologic features of this young group were reviewed retrospectively, using hospital records, and compared with those of older patients (n = 499 [29.4%], 60 to 69 years of age). RESULTS: The young group contained significantly higher percentages of female patients, epigastric pain symptoms, depressed superficial type lesions, mucosal invasion, and poorly differentiated histology; percentages of hepatic metastasis and venous invasion were lower. Survival rates for all patients and for the resected cases were significantly better in the young group (p = 0.035 and 0.017 respectively). The percentage of early stage stomach cancers for the group less than 40 years of age was 49.0% in 1984-89, but had risen to 60.9% by 1990-95. CONCLUSIONS: Early diagnosis has improved the prognosis of young gastric cancer patients. Furthermore, these data show a recent shift in stage distribution; additional prognostic improvement is anticipated for young patients with early gastric cancer.     

Color-coded signal-enhanced duplex ultrasonography of space-occupying intramammary processes

PURPOSE: We evaluated the influence of three different dosages of methotrexate (MTX) during consolidation on the incidence of testicular relapse in children with acute lymphoblastic leukemia (ALL). PATIENTS AND METHODS: One thousand one hundred forty-four boys with newly diagnosed ALL, enrolled in three consecutive trials of the Berlin-Frankfurt-Münster group (ALL-BFM 81, 83, 86), were retrospectively evaluated for the influence of MTX on testicular relapse-free interval. The basic treatment design was similar in all trials. No intravenous MTX was used in trial ALL-BFM 81 in the group who received cranial irradiation (CRT) except for a part of standard risk patients. Four courses of intermediate dose MTX (IDM) (0.5 g/m2) were introduced for all patients in trial ALL-BFM 83 (IDM group), which were replaced by high dose MTX (HDM) (5 g/m2) in trial ALL-BFM 86 (HDM group). The media observation time was > 9 years. RESULTS: The cumulative incidence of isolated testicular relapses was significantly higher in the CRT group as compared to the IDM and HDM groups (6.7% versus 2.5% and 2.3%, p = 0.02 and 0.01). HDM decreased neither the incidence nor the rate of isolated testicular relapses any further. Event-free survival (EFS) for boys was similar between trial ALL-BFM 81 and 86 (64% versus 69%, p = 0.35), but differed significantly between trials ALL-BFM 83 and 86 (61% versus 69%, p = 0.0078). CONCLUSION: The introduction of IDM reduced the incidence of isolated testicular relapses significantly by a factor two, but had no significant influence on overall survival. HDM did not result in a more effective prevention of testicular relapses, but resulted in better systemic control and hence better survival than IDM.     

Sarcomas and related proliferative lesions of specialized prostatic stroma: a clinicopathologic study of 22 cases

Sarcomas and related proliferative lesions of the specialized prostatic stroma have been the subject of case reports and, thus, have not been well characterized. We reviewed the clinicopathologic features of 22 cases and studied the immunohistochemical profile of 9. Patient age ranged from 25 to 86 years; mean age was 54 years, and peak incidence was in the 6th and 7th decades. The most common clinical presentation was urinary retention, then abnormal results of digital rectal examination, hematuria or hematospermia, and a palpable rectal mass. The cases were grouped into two categories: prostatic stromal proliferation of uncertain malignant potential (PSPUMP, 18 cases) and prostatic stromal sarcoma (PSS, 4 cases) based on the degree of stromal cellularity and the presence of mitotic figures, necrosis, and stromal overgrowth. Four histologic patterns of PSPUMP were identified: (1) hypercellular stroma with scattered cytologically atypical cells associated with benign glands, (2) hypercellular stroma with minimal cytological atypia associated with benign glands, (3) hypercellular stroma with or without cytologically atypical cells, associated with benign glands in a "leaflike" growth pattern that resembled phyllodes tumors of the mammary gland, and (4) hypercellular stroma without cytologically atypical stromal cells and without glands. Prostatic stromal sarcoma showed greater cellularity, mitoses, necrosis, and stromal overgrowth than PSPUMP and consisted either of stromal elements with benign glands in a pattern that resembled malignant phyllodes tumors of the mammary gland (3 cases) or of purely stromal elements (1 case). Positive immunohistochemical reactions were noted using vimentin in 9 of 9 cases, CD34 in 8 of 8, HHF-35 in 2 of 8, smooth muscle actin in 3 of 9, desmin in 4 of 8, S-100 protein in 0 of 9, estrogen receptor in 1 of 7, and progesterone receptor in 6 of 7. None of the cases classified as PSS were positive for HHF-35, smooth muscle actin, or desmin. Of the 13 patients classified as having PSPUMP who did not undergo definitive local therapy at the time of diagnosis, recurrent signs or symptoms were seen in six (46%), necessitating additional therapy. Distant metastases to lung and bone developed in one patient classified as having PSS. Clinical and pathologic findings in this patient suggested a progression from PSPUMP to PSS. We conclude that sarcomas and related proliferative lesions of the specialized prostatic stroma encompass a spectrum of histologic features and may be grouped into two clinicopathologic categories: PSPUMP and PSS. Based on their distinctive histologic appearance and immunohistochemical profile, PSPUMP and PSS can be differentiated from other mesenchymal lesions of the prostate.     

Perthes disease--results of a containment-oriented therapy concept

INTRODUCTION: In a retrospective study a treatment concept for Perthes' disease dependent on the containment was applied. PATIENTS/METHODS: 49 hips of 41 children (9 female, 32 male) were treated between 01. 01. 1990 and 31. 12. 1995. In our concept of treatment a varus femoral osteotomy was performed in 28 cases with not contained hips or less than 4/5 coverage of the femoral head (X-ray/MRI). The other 21 well contained hips with 4/5 coverage or more were treated conservatively with physiotherapy and in case of joint effusion and pain additionally with the use of crutches (partial weight bearing) and anti-inflammatory medication. The average age in the non-operative group at the time of first investigation was 4 years and 9 months (3 y./1 m. to 7 y./1 m.) and 6 years and 3 months (4 y/2 m. to 10 y/0 m.) at our last examination (mean follow up 17.7 months, range of 6 to 72 months). At the time of indication for a varus femoral osteotomy the patients had an average age 6 years and 1 month (3 y./6 m. to 10 y./2 m.), the mean age at the last postoperative examination was 7 years and 11 months (4 y./8 m. to 12 y./5 m.) with an average follow up of 21.5 months (6 to 77 months). RESULTS: For the conservatively treated children we achieved good results (still well contained hips with 4/5 coverage, no decrease of function, no increase of pain) in 85.7% (18 of 21 cases). In 85.7% (24 of 28 cases) we found good results (well contained hips, increase of coverage, no decrease of function, no increase of pain) in the operation group. CONCLUSIONS: The presented concept of therapy in Perthes' disease was practicable for all patients and included the possibility of decision for operative or non-operative treatment. In both groups we achieved good results in 85.7% of the cases.