Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study

OBJECTIVES: The aim of the present investigation was to redefine the clinicopathologic profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC), with special reference to disease progression and left ventricular (LV) involvement. BACKGROUND: Long-term follow-up data from clinical studies indicate that ARVC is a progressive heart muscle disease that with time may lead to more diffuse right ventricular (RV) involvement and LV abnormalities and culminate in heart failure. METHODS: Forty-two patients (27 male, 15 female; 9 to 65 years old, mean [+/-SD] age 29.6 +/- 18) from six collaborative medical centers, with a pathologic diagnosis of ARVC at autopsy or heart transplantation, and with the whole heart available, were studied according to a specific clinicomorphologic protocol. RESULTS: Thirty-four patients died suddenly (16 during effort); 4 underwent heart transplantation; 2 died as a result of advanced heart failure; and 2 died of other causes. Sudden death was the first sign of disease in 12 patients; the other 30 had palpitations, with syncope in 11, heart failure in 8 and stroke in 3. Twenty-seven patients experienced ventricular arrhythmias (ventricular tachycardia in 17), and 5 received a pacemaker. Ten patients had isolated RV involvement (group A); the remaining 32 (76%) also had fibrofatty LV involvement that was observed histologically only in 15 (group B) and histologically and macroscopically in 17 (group C). Patients in group C were significantly older than those in groups A and B (39 +/- 15 years vs. 20 +/- 8.8 and 25 +/- 9.7 years, respectively), had significantly longer clinical follow-up (9.3 +/- 7.3 years vs. 1.2 +/- 2.1 and 3.4 +/- 2.2 years, respectively) and developed heart failure significantly more often (47% vs. 0 and 0, respectively). Patients in groups B and C had warning symptoms (80% and 87%, respectively, vs. 30%) and clinical ventricular arrhythmias (73% and 82%, respectively, vs. 20%) significantly more often than patients in group A. Hearts from patients in group C weighed significantly more than those from patients in groups A and B (500 +/- 150 g vs. 328 +/- 40 and 380 +/- 95 g, respectively), whereas hearts from both group B and C patients had severe RV thinning (87% and 71%, respectively, vs. 20%) and inflammatory infiltrates (73% and 88%, respectively, vs. 30%) significantly more often than those from group A patients. CONCLUSIONS: LV involvement was found in 76% of hearts with ARVC, was age dependent and was associated with clinical arrhythmic events, more severe cardiomegaly, inflammatory infiltrates and heart failure. ARVC can no longer be regarded as an isolated disease of the right ventricle.     

Esophageal leiomyomatosis

PURPOSE: To reassess the clinical and radiologic findings in patients with esophageal leiomyomatosis. MATERIALS AND METHODS: A search of the authors' radiologic archives revealed six cases of esophageal leiomyomatosis in a 22-year period. The clinical findings and radiologic images were reviewed retrospectively. RESULTS: The average age of the patients was 10.8 years (range, 6-18 years). Five patients presented with slowly progressive dysphagia. Barium studies revealed smooth, tapered narrowing of the distal esophagus in five patients and characteristic defects on the superomedial aspect of the gastric fundus abutting the cardia, presumably due to bulging of this thickened mass of muscle into the stomach, in four patients. In two patients, computed tomography (CT) revealed marked thickening of the distal esophageal wall. CONCLUSION: Esophageal leiomyomatosis can be suggested in a pediatric patient with long-standing dysphagia in whom smooth, tapered distal esophageal narrowing is seen at barium study and circumferential esophageal wall thickening is seen at CT.     

Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.75 years and early seizure onset (6.8 months), whereas group B was composed of older patients (mean age, 15.3 years) with later onset of seizures (3.7 years). Neurologic disease was more severe in group A, but group B had more widespread structural brain defects - shown on computed tomographic scans and magnetic resonance imaging - and metabolic brain defects shown on hexamethylpropyleneamine oxime and [18F] fluorodeoxyglucose single photon emission computed tomographic scans. Six group A cases had hypoperfusion at baseline and five of nine had worsening of perfusion and glucose metabolism 1 year later. A total of 119 stroke-like episodes occurred in six group A cases and eight group B cases; there were 65% fewer strokes in children treated with aspirin. The data suggest that progressive hypoperfusion and glucose hypometabolism are associated with neurologic deterioration in Sturge-Weber syndrome. Longitudinal studies are needed to better define the natural history of disease and to evaluate the safety and efficacy of aspirin therapy.     

Randomised double-blind clinical trial of intermediate- versus high-dose chloral hydrate for neuroimaging of children

Orally administered chloral hydrate is the most widely used sedative in children undergoing MRI. We compared intermediate- and high-dose oral chloral hydrate in 97 consecutive children undergoing MRI in a prospective, controlled, double-blind, randomised clinical trial. There were 50 girls and 47 boys, mean weight (+/- SD) 14.7 +/- 6.4 kg, and mean age 38 +/- 31. The children were randomly allocated to receive chloral hydrate syrup either 70 mg/kg (group A, n = 50) or 100 mg/kg (group B, n = 47). These two groups were not significantly different in sex, weight, age, diagnosis or ambulatory medication. The mean initial dose (+/- SEM) was 64 +/- 2 mg/kg for group A and 93 +/- 2 mg/kg for group B. Because adequate sedation was not achieved, 14 patients in group A and 6 in group B required a second dose, giving a mean total dose of 70 +/- 2 mg/kg for group A and 96 +/- 2 mg/kg for group B. The percentage of successful examinations after the initial dose (A: 64%, B: 87%; p < 0.05) and the total dose (A: 92%, B:100%; p = 0.14) was higher in group B. Significant differences were found for the time of onset of sedation (A:28 +/- 2 min, B: 21 +/- 1 min; p < 0.05), but not for the time to spontaneous awakening after the completion of the examination. The rate of adverse reactions was similar (A: 20%, B: 21%; p = 1.00). We conclude that high-dose oral chloral hydrate improves the management of children undergoing MRI.     

Meningococcal disease in childhood--a regional study in Ireland

A retrospective study of microbiology laboratory records of culture-confirmed cases of meningococcal disease in children under 14 years of age, admitted to four Dublin hospitals, was conducted for the period 1981-1991 inclusive. The study aimed to describe the epidemiology of meningococcal disease in childhood and to assess the potential of meningococcal group A and C vaccine in preventing disease. There were 406 cases of meningococcal disease of which 319 (78.6%) were in children under 5 years of age. The meningitis to septicaemia ratio was almost 4:1 (320 vs. 86 cases). Of the 406 cases, 216 (53.2%) cases occurred during the period November to March. The age-specific annual incidence rates for the Eastern Health Board region served was 64.2 per 100,000 under 1 year of age and 24.6 per 100,000 under 5 years of age. A relative increase in disease caused by meningococcal serogroup C was observed during the period of the study. Only 15% all cases could have been prevented by a policy of universal meningococcal group A and C immunisation at 2 years of age. Such a policy, therefore, is not recommended. A vaccine for preventing disease caused by meningococcal group B is urgently required.     

Radiographic and histologic patterns of calcification in chondromyxoid fibroma

OBJECTIVE: To evaluate the frequency of radiologic and histologic manifestations of matrix calcification in chondromyxoid fibromas. PATIENTS: Forty-four cases of chondromyxoid fibroma were reviewed. The age range of the patients was 3-70 years (average 29 years). RESULTS: Calcification was found microscopically in 15 cases (34.1%). In five cases (12.5%) it was demonstrated on plain films or CT. The age range of the patients with microscopic evidence of calcified matrix was 14-70 years (mean 46 years), while that of the patients with non-calcified lesions was 3-59 years (average 21 years). All but two of the patients who showed microscopic calcification in the tumors were over 40 years of age. Four microscopic patterns of calcification were observed: coarse granular, circumscribed, trabecular, and "chicken-wire." CONCLUSIONS: Calcification in chondromyxoid fibroma was found more frequently than in previously reported studies. There was a tendency for this phenomenon to occur in the tumors of older patients, particularly those over 40 years old, and in chondromyxoid fibromas situated in flat bones, including ribs.     

Prognostic significance of coexistent bulky metastatic central nervous system disease in patients with leptomeningeal metastases

OBJECTIVE: To assess the clinical significance of bulky metastatic central nervous system disease in patients with leptomeningeal metastases. PATIENTS AND METHODS: Forty patients (24 women and 16 men) ranging in age from 32 to 74 years (median, 56.5 years) with cytologically documented leptomeningeal metastases were demonstrated by cranial or spinal magnetic resonance imaging to have either no bulky central nervous system metastatic disease (group A; 20 patients) or bulky central nervous system metastatic disease (group B; 20 patients). Twenty-nine patients were treated with involved-field radiotherapy, and all patients were treated with sequential intraventricular chemotherapy. RESULTS: Median survival was 7 months in group A (range, 5-12 months) as compared with 4 months in group B (range, 2-12 months) (P < .01; Mantel-Cox log rank analysis). Cause of death was similar in both patient groups. CONCLUSIONS: In patients with leptomeningeal metastases, neuroradiographic demonstration of bulky metastatic central nervous system disease independently predicts survival and is useful in determining which patients are candidates for intraventricular chemotherapy.     

Incidence and clinical profile of familial forms of Parkinson's disease. A study of 428 index-cases from a department of neurology]

Although the cause of Parkinson's disease remains to be determined, several lines of evidence favor the role of a genetic factor. We therefore studied a series of 428 index-cases of Parkinson's disease that were referred to our Department of Neurology between 1986 and 1993, with the aim to identify secondary family cases. Index-cases were divided into 2 groups. In groups A (279 cases), a retrospective analysis of patients records was performed. In group B (149 cases), a prospective study was carried out by 1992, which allowed a more complete investigation of family history. In group A, 31/279 index-cases (11.1 p. 100) had secondary familial cases of Parkinson's disease. This percentage increased up to 22.8 p. 100 among index-cases in group B (34/149 cases). In most instances, only one secondary case was detected, and very few proponents had 2, or 3 other family cases. No large family with numerous Parkinson's disease cases was disclosed. Age at onset of disease was similar in group A between sporadic and familial index-cases, whereas in group B age at onset was earlier index-cases with positive family history as compared to those without (53.9 +/- 10.4 years versus 59.7 +/- 12.1 years respectively). This may be due to the different sizes of groups A and B, whereas clinical profile analysis did not differentiate index-cases with positive family history from those without family history (sporadic cases). An anticipation of age at onset of illness of 13.9 +/- 12.2 years was found in 9 of the 15 index-cases from group B with first degree parental vertical inheritance, where clinical data were available for the second family case. These findings about age at onset may be at least partly explained by a more accurate estimation of age at onset in index-cases than that in secondary family cases. Further analysis on the possible mode of transmission of the disease among familial cases was consistent with the implication of a genetic factor in the ethiopathogenesis of the disease, with a mendelian autosomal dominant inheritence with reduced penetrance.     

Systemic mastocytosis. Study of 14 cases

We have analyzed the clinical, analytical and evolutive data of 14 cases of systemic mastocytosis (SM) diagnosed in our hospital between 1991-1996 and we have compared these results with those of other published series. Clinical parameters, analytical profiles, peripheral hematologic data, radiologic data and histological study were collected. Following Metcalfe's criteria, the patients were classified in 4 groups: a) group 1, indolent mastocytosis; b) group 2, hematologic disorders; c) group 3, aggressive lymphadenopathic mastocytosis with eosinophilia, and d) group 4, mastocytic leukemia. The average age at diagnosis was 52.4 years, range 25-83, and 64% were females. The mean follow-up was 2 yr. In most of the cases (71.4%) the initial complaint was urticaria pigmentosa. The predominant clinical features were pruriginous-eritematous skin lesions (in 11 cases), and digestive symptoms (in 10 patients). The most usual biochemical disorder was the rise of serum alkaline phosphatase level (in 8 patients), while lactate-dehydrogenase (LDH) was normal in all the cases. The most striking roentgenologic features were oteopenia, observed in 50% of our patients. Pheripherical hematological disorders were discovered in 8 patients (64.3%) and in one of them circulating mast-cells were observed. The bone marrow was involved in all patients (100%) and in two of them mielodysplasic features were found. The diagnostic of SM is difficult in the absence of skin lesions. The skin lesions are very common in systemic mastocytosis. Bone marrow involvement is constant, so its study has a high diagnostic rentability.     

Multiple primary tumours of the urogenital system: our cases and a review of the literature

The impacted palatal canine requires a combination of both surgical and orthodontic management. Two types of approach are commonly used: simple exposure, or exposure with bracketing at the time of surgery. In this study 104 consecutively treated patients with palatally impacted canines were examined at two centres, one at which the ectopic tooth was surgically exposed alone and the other where an orthodontic bracket was bonded to facilitate early traction, and the flap replaced. The aim was to compare the outcome and complication rate for each type of procedure. In 30.7 per cent of all cases exposed and bracketed a second surgical intervention was required, compared with 15.3 per cent in the simple exposure group. Bracketing, though effective, is a more costly and time-consuming procedure, and it is suggested that simple exposure provides an equally efficient and predictable method of managing the palatally impacted canine with obvious clinical and financial benefits. The long-term periodontal status of the teeth which have been exposed in these two ways, however, needs further investigation.     

The impacted maxillary canine. Further observations on aetiology, radiographic localization, prevention/interception of impaction, and when to suspect impaction

Adjacent anomalous or missing maxillary lateral incisors have been implicated in the aetiology of palatally displaced canines by not providing proper guidance to the canine during its eruption. However, a recent review of the literature suggests that the aetiology of palatally displaced canines is genetic in origin. The aetiology of labially impacted canines differs, being due to inadequate arch space. Vertex occlusal radiographs have been recommended for localization but have limitations, and a case is illustrated where this radiograph is deceptive. The prevention/interception of a palatally displaced canine by the extraction of the deciduous canine is best carried out as early as the displacement is detected, mostly soon after 10 years of age. Usually, prevention/interception will avoid the surgical and orthodontic treatment needed to align a palatally impacted canine and may help prevent resorption of the adjacent incisor root. Suspicions that an impaction could occur or has occurred arise a) before the age of 10 years if there is a familial history and/or the maxillary lateral incisors are anomalous or missing; b) after the age of 10 years if there is asymmetry in palpation or a pronounced difference in eruption of canines between the left and right side; or the canines cannot be palpated and occlusal development is advanced; or, the lateral incisor is proclined and tipped distally; and, on a panoramic radiograph of the late mixed dentition if the incisal up of the canine overlaps the root of the lateral incisor.     

National cancer clinical trials: children have equal access; adolescents do not

PURPOSE: To determine whether adolescents with cancer, who in comparison to younger patients have a higher cancer incidence and lower mortality reduction, have equal access to national cancer clinical trials. METHODS: The ethnic/racial distribution of 29,859 subjects < 20 years of age entered onto National Cancer Institute-sponsored clinical trials between January 1, 1991, and June 30, 1994, was compared with the expected distribution of patients of the same age in the United States. RESULTS: The Children's Cancer Group and Pediatric Oncology Group had 29,134 (97.6%) of the total study entries among < 20-year-old subjects during the 3.5 years of surveillance. The adult cooperative groups accounted for < 3% of the clinical trials entries in the 15-19-year age range. When analyzed nationally by region, the under-representation of the older adolescent subjects was universal. From other analyses, the two pediatric cooperative groups were estimated to have registered > 94% of the children < 15 years of age who were expected to have been diagnosed to have cancer, but only 21% of the cancer patients in the 15-19-year age group. CONCLUSIONS: The national pediatric cancer cooperative groups allow the majority of American children < 15 years of age and their families equal opportunity to access clinical cancer trials, regardless of race or ethnicity. Among patients 15-19 years of age, however, > 75% are not being enrolled by any cooperative group sponsored by the National Cancer Institute. Thus, older adolescents are disadvantaged with respect to access to the national clinical trials, regardless of their race or ethnicity.     

Blastomycosis in northeast Tennessee

STUDY OBJECTIVES: To study the epidemiologic and clinical features of blastomycosis in northeast Tennessee. DESIGN: Retrospective review of blastomycosis cases in the region from 1980 through 1995. SETTING: Hospitals located in the Tri-Cities region of northeast Tennessee. PATIENTS: Seventy-two patients with confirmed blastomycosis infection. INTERVENTIONS: None. RESULTS: During the 1980 to 1995 study period, we documented 72 cases of blastomycosis. The mean age was 52 years (range, 13 to 86 years), most were male (69.4%), and nine were immunocompromised. A possible environmental exposure was noted for 28 patients. Pulmonary involvement represented the most common site of infection (61 cases), but multiorgan involvement was common (17 cases). Most patients with pulmonary blastomycosis (66%) presented with a chronic illness, and radiologic findings usually revealed local consolidation or a mass-like lesion. Nine patients developed ARDS with an associated mortality rate of 89%, compared with a 10% mortality for non-ARDS pulmonary cases. Antifungal treatment regimens varied widely, with amphotericin B often used for sicker patients. An epidemiologic evaluation revealed that the mean yearly incidence rate for blastomycosis quadrupled between 1980 and 1987 (0.31 cases/ 100,000 population) and 1988 to 1995 (1.23 cases/100,000 population) (p=0.00001). Most new blastomycosis cases in the 1988 to 1995 period occurred in three counties in the region where significant new construction projects have been underway. CONCLUSION: Blastomycosis is endemic in northeast Tennessee and the number of cases is increasing, coinciding with major new construction in the region. Clinicians in the area must be alert to this condition.     

Outcomes of the surgical exposure, bonding and eruption of 82 impacted maxillary canines

BACKGROUND: Advances in bonding techniques and materials allow for reliable bracket placement on ectopically positioned teeth. This prospective study evaluates the outcome of forced orthodontic eruption of impacted canine teeth in both palatal and labial positions. METHODS: Eighty-two impacted maxillary canines in 54 patients were included in the study and were observed for 18 to 30 months after exposure. Following exposure by means of a palatal flap or an apically repositioned buccal flap, an orthodontic traction hook, with a ligation chain attached, was bonded to each impacted tooth using a light cured orthodontic resin cement. A periodontal dressing was placed over the surgical site for a period of time. RESULTS: All teeth were successfully erupted. Complications consisted of: failure of initial bond, at the time of surgery, which required rebonding; premature debonding at the time of pack removal and; debonding of brackets during orthodontic eruption. There was no infection, eruption failure, ankylosis, resorption or periodontal defect (pocket greater than 3 mm) associated with any of the exposed teeth. Attached gingiva of less than 3 mm was seen in only two of the buccally positioned canines (9%). CONCLUSION: Forced orthodontic eruption of impacted maxillary canines with a well bonded orthodontic traction hook and ligation chain, used in conjunction with a palatal flap or an apically repositioned labial flap, results in predictable orthodontic eruption with few complications.     

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.     

Gender and age at onset in schizophrenia: impact of family history

Between June and November 1993, a parasitological, clinical and ultrasonographical survey was carried out in the mid west of Madagascar, among the whole population of a village where Schistosoma mansoni was endemic. S. mansoni eggs were found in the stools of 61.8% of the 544 individuals examined; the highest prevalence of infection (85.7%) was observed in the 15-19 years old age group. The most intense infections were recorded in the 10-14 years age group, in which the geometric mean egg count was 265 eggs/g of faeces and 34.3% of the individuals excreted more than 400 eggs/g. The egg count decreased steadily from the age of 15 years. Ultrasonographical examination was performed in 482 inhabitants older than 2 years: 29% of them presented definite schistosome associated liver alterations according to the proposals for staging S. mansoni infection made by the World Health Organization Cairo working group. Stage 3 was never observed in subjects under 30 years old. This is consistent with the need for a long-standing infection before serious lesions occur. A significant relationship between egg count and degree of liver alterations was found only in the 20-29 years age group.     

Diagnostic difficulties of myocardial infarct in an admissions and emergency service: influence of age

OBJECTIVE: To analyze the clinical signs and electrocardiographic and enzyme data in patients admitted to an emergency ward for myocardial infarction in order to highlight diagnostic pitfalls. PATIENTS AND METHODS: All patients admitted to our emergency ward between October 1995 and October 1996 with elevated myoglobulin or creatinine phosphokinase (CPK) levels (n = 457 patients) were included in the study series. Patient files were randomly selected (n = 257 files) for review by an emergency ward specialist and a cardiologist to identify cases of myocardial infarction (n = 88 patients, mean age 73.4 +/- 15.2 years). Clinical, electrical and enzyme data (including CPK-MB) were analyzed. RESULTS: The patients had been referred for chest pain (69%), dyspnea (24%) or another disorder (7%). Pain was located in the retrosternal area in 51%, in the lower chest in 19% and elsewhere in 30%. Delay between onset of pain and transfer to the emergency ward was 5 h 20 min +/- 6 h. Signs of left heart failure were observed in 50% of the patients. The admission electrocardiogram showed complete criteria for myocardial infarction in 43% of the cases, incomplete criteria in 21% and was non-contributive in 36%. Enzyme results were elevated in 78% of the cases at the first assay and in 98.2% at the second assay. Both typical chest pain and ECG were observed in only 30% of the cases. Chest pain was present in 55% of the patients over 75 years of age and in 81% of those under 75 years (p = 0.007). CONCLUSION: A typical syndrome is observed in less than one-third of all patients with myocardial infarction admitted to emergency wards. The frequency of atypical presentations increases with age.     

Diastolic dysfunction in patients with chronic kidney failure on a hemodialysis program

OBJECTIVE: The aim of this study was to analyse different ultrasound parameters for the assessment of isolated left ventricular diastolic dysfunction (LVDD) in patients with chronic renal failure (CRF) on periodic hemodialysis (HD), comparing pulsed wave Doppler with pulsed tissue Doppler. MATERIALS AND METHODS: Forty-seven patients with CRF on HD (61% were male; mean age was 51.0 +/- 16.5 years, mean HD time--3.7 +/- 3.8 years, 38% had hypertension, 17% had diabetes) were studied by echocardiography (bidimensional, M-Mode, flow pulsed Doppler and tissue Doppler imaging). All patients had symptoms of left heart failure-class II NYHA, were in sinus rhythm and had no symptoms of ischemic heart disease. The presence of abnormal LV regional contractility was the exclusion criteria. According to their mitral inflow profile Doppler characteristics, patients were included in two groups: Group A (E/A > 1; n = 21) and B (E/A < 1; n = 26). We compared: LV dimensions and function, left atrial (LA) dimension. Gaasch index, LV mass index. E and A wave velocities (in flow pulsatile Doppler and tissue Doppler). E/N ratio in tissue Doppler, isovolumetric relaxation time (IVRT) and deceleration time (DT). RESULTS: There were no significant differences in the prevalence of age > or = 65 years male sex, hypertension or diabetes between group A and B patients, and almost all patients were on hemodialytic treatment for more than one year (81% vs 85%: NS). LV hypertrophy was present in almost all group A and B patients (A--95% vs B--85.5%; NS). Group A, compared with group B, had a difference in the Gaasch index (2.45 +/- 0.3 vs 2.08 +/- 0.4; p < 0.05), E wave velocity in flow pulsatile Doppler and tissue Doppler (cm/sec) (110 +/- 27 vs 62 +/- 20; p < 0.001 and 41 +/- 15 vs 28.5 +/- 16; p < 0.05), E/A ratio in tissue Doppler (1.3 +/- 0.4 vs 0.8 +/- 0.3; p < 0.001). IVRT (msec) (80.7 +/- 15.2 vs 113.5 +/- 28.3; p < 0.001) and DT (msec) (189.7 +/- 24 vs 278.2 +/- 17.9; p < 0.001). According to the E'/A' ratio in tissue Doppler, group A patients were divided in another two groups: E'/A' > 1 (13/21--62%) and < 1 (8/21--38%) and a significantly longer IVRT (75.8 +/- 9.3 vs 100.9 +/- 3.2; p < 0.001) and DT (178 +/- 15 vs 240 +/- 20; p < 0.001) and a greater LA dimension (37.6 +/- 6.9 vs 44.6 +/- 6.9; p < 0.05) were found. CONCLUSIONS: Pulsed wave Doppler is the most useful non invasive method for assessment of global diastolic dysfunction. In our study, 17% of the patients had E/A < 1 only in the tissue Doppler study. These patients probably had a pseudonormal mitral pattern.     

Synovial sarcoma in children and adolescents: the St Jude Children's Research Hospital experience

PURPOSE AND METHODS: We reviewed the clinical records and pathologic findings of 37 children and adolescents with synovial sarcoma treated at our institution over a 30-year period to evaluate the prognostic significance of tumor size, invasiveness, histology, and other features. RESULTS: The 20 male and 17 female patients with synovial sarcoma had a median age of 13.7 years at diagnosis. Primary tumor sites were the extremities (n = 27), trunk (n = 8), and head and neck (n = 2). Disease stage (clinical group) was as follows: group I, n = 21; group II, n = 7; group III, n = 4; and group IV, n = 5. Nineteen patients had invasive (T2) lesions, 20 had tumors more than 5 cm in diameter, and 14 had histologic grade 3 lesions. The estimated 5-year survival rate (+/- SE) for patients with group I or II disease was 80% +/- 9%, compared with 17% +/- 15% for those with group III or IV tumors (P = .0003). An exact log-rank test, adjusted for clinical group, showed that tumor invasiveness and grade independently predicted overall and progression-free survival (P < .05); tumor size was significantly correlated with progression-free survival. A borderline significant relationship with overall survival was found for both tumor size and histologic subtype (P = .09). CONCLUSION: A controlled trial of adjuvant chemotherapy is merited in children with resected synovial sarcoma (clinical group I or II) who present with unfavorable clinicopathologic features such as large, invasive, or grade 3 lesions. Children with unresected or metastatic disease fare poorly despite multimodality therapy and require novel treatment approaches.     

Analysis of the immunophenotype of children treated on the Medical Research Council United Kingdom Acute Lymphoblastic Leukaemia Trial XI (MRC UKALLXI). Medical Research Council Childhood Leukaemia Working Party

Despite many years of meticulous immunophenotyping of childhood acute lymphoblastic leukaemia (ALL) cases the prognostic significance of some subtypes remains unclear. The Medical Research Council UKALLXI trial (1990-1996) in which uniform treatment has been given to 2090 children with ALL below the age of 18 years and above the age of 1 year, has afforded the opportunity to review these issues. Children with ALL of mature B cell type were not entered into this trial. Immunophenotype analysis was performed in each individual trial centre, but results were centrally reviewed in all cases, and were both available and considered adequate in 1934 (93%) of the first 2090 patients entered. The main diagnostic categories were early pre-B or null reported in 60 cases (3.1%), common ALL in 1242 (64.2%), pre-B in 252 (13.0%), 'common' or pre-B in 172 (8.9%) and T cell in 207 (10.7%) cases. Children with T cell disease were significantly more likely to be over the age of 10 years, with central nervous system disease at diagnosis and to be CD34 negative. They also had a higher incidence of high white cell count and were more likely to be of the French-American-British (FAB) L2 morphological subtype. Patients with 'null' cell disease tended to be less than 2 years or greater than 10 years of age, and CD13 and CD33 positive. CD10 was associated with lower white cell count (WBC) at diagnosis, younger age and FAB L1 morphological subtype. The presence of cytoplasmic immunoglobulin in pre-B cells was not associated with any specific clinical or laboratory features. CD34 positivity was less common in T cell patients and was associated with low WBC. Disease-free survival (DFS) and 95% confidence intervals (CI) at 5 years from diagnosis was 52% (95% CI: 44-59%) for T cell disease, 58% (95% CI: 43-73%) for early pre-B (or null cell) disease and 65% (95% CI: 62-68%) for common or pre-B disease; there being no significant difference between common and pre-B disease with regard to disease outcome. Patients with T cell disease had a worse prognosis than any other immunophenotype group (P < 0.00005). However this worse outcome was no longer significant after allowing for the other principal prognostic factors of age, gender and white cell count at diagnosis except for the very small number with WBC <20 x 10(9)/l and T cell disease. Those with CD10-positive leukaemia did better than those who were CD10 negative (P < 0.00005), with DFS at 5 years 64% (95% CI: 62-67%) for positive vs 56% (95% CI: 49-62%) for CD10 negative. CD10 positivity did not have independent significance when white count, gender and age were taken into account. CD13, CD33, and cytoplasmic mu positivity carried no prognostic significance.