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The article discusses the case of a 48-year-old man who have undergone stem cell transplantation (SCT) after he has been diagnosed of cutaneous T-cell lymphoma (CTCL). Topics covered include histopathological examination of the patient's skin prior the treatment, T-cell receptor (TCR) rearrangement due to CTCL, and incidence of primary cutaneous aggressive cytotoxic epidermotropic CD8+ TCL (PCAE-TCL). The outcome of SCT procedure is also mentioned. more...

A series of 11,890 patients from the senior investigator's surgical service between 1949 and 1998 is analyzed for the significance of distinct risk factors for recurrence of, and survival from, atherosclerotic occlusive disease. Eight risk factors have been assessed for their importance in 4 defined arterial categories (the coronary arterial bed, the branches of the aorta, the abdominal visceral [celiac, superior mesenteric, and renal] arteries, and the terminal abdominal aorta and its major branches) in determining survival rate of the entire group and their impact on rate of recurrence of atherosclerosis in a subgroup of 5,568 patients who had > or =1 postoperative arteriogram, permitting precise identification of changes in the atherosclerotic process. Patients in these 2 groups were followed for > or =25 years; univariate and multivariate analyses were used. On admission all patients had symptomatic atherosclerotic occlusive disease in a single vascular category. Each patient was treated surgically for alleviation of the disease. Two primary outcomes are included: (1) survival, by atherosclerosis category, in all 11,890 patients; and (2) recurrence, also by category, in the subset of 5,568 patients. Multivariate results for recurrence showed little consistency across categories. Only 1 risk factor, diabetes, appeared in 2 of the 3 categories fully analyzed. Other variables that are significant in only a single category are male sex, cholesterol, hypertension, and smoking. Survival showed much greater consistency, with age, diabetes, and hypertension significant in all 3 categories, male sex and smoking in 2, and cholesterol in only Category I. Univariate results followed much the same trend. For recurrence and survival, the response of the arterial bed to the risk factors in each of the 4 categories is distinctly different, an observation that we have not found to be previously reported. [ABSTRACT FROM AUTHOR] more...

Background: Anxiety and depressive symptoms are commonly reported to have a high prevalence in advanced cancer patients. However, whether the severity of the symptoms change during a stay in a palliative care unit (PCU) and after discharge home has not been studied thus far. This prospective, longitudinal, single-center study screened for anxiety and depression as measured on the German version of Hospital Anxiety and Depression Scale (HADS-D) in a palliative care (PC) cancer cohort at three different time points., Methods: Consecutive patients (N=206) admitted to a PCU were evaluated of whom N=102 could be enrolled. Patients were screened for anxiety and depression using the HADS-D questionnaire: 24 h after admittance (P1), within 24 h before discharge (P2) and 2 weeks after discharge (P3). Longitudinal changes and influencing factors were determined., Results: Nearly 80% of all patients had at least at one time point a HADS score ≥8 indicating a clinically meaningful symptom burden. The P1 mean scores were 7.1±3.3 (anxiety) and 8.9±4.6 (depression). Depression was associated with underlying cancer type (P<0.05). Anxiety and depression stabilized during hospitalization (P2). However, a significant deterioration after discharge (P3) was observed (anxiety P=0.046; depression P=0.003), in particular in older patients (>65 years) and higher ECOG status (≥3). Patients with a short time since first diagnosis (<1 year) had significantly higher symptom burden compared to patients with a longer disease course. Participation was 50% emphasizing the difficulty to study PC patients. Most patients had advanced cancers (99%). Underlying cancer types consisted of a broad variety of solid tumors including 15% hematological cases. Median survival was 1.1 months., Conclusions: The high prevalence of anxiety and depressive symptoms points to the need for psychological support. All PC patients should be screened for psychological distress to identify those in need of further assessment and treatment. The deterioration at home suggests the need for improved outpatient management, including home-based psychological support. Caregivers should be aware of the psychological vulnerability of newly diagnosed cancer patients, patients with lower functional status and higher age. more...

MPV17 encodes a putative channel-forming protein of the inner mitochondrial membrane and is involved in mitochondrial deoxynucleotide homeostasis. MPV17 mutations were first reported in patients with Navajo neurohepatopathy, an autosomal recessive mitochondrial DNA depletion syndrome, characterized by early-onset liver failure, failure to thrive as well as central and peripheral neurological involvement. Recently, two patients with juvenile-onset peripheral sensorimotor neuropathy associated with an MVP17 c.122G>A (p.Arg41Gln) variant have been reported. Here, we describe five additional patients from two unrelated families with sensorimotor axonal neuropathy without hepatocerebral affection caused by homozygous MPV17 variants. Patients of the first family carried the known c.122G>A variant and affected individuals of the second family had a novel c.376-9T>G near-splice variant, which was shown to result in an in-frame deletion of 11 amino acids. This report provides further evidence that MPV17 mutations should be considered in patients with pure, non-syndromic axonal neuropathy., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.) more...

Objective: We aimed to delineate the neurodevelopmental spectrum associated with SYNGAP1 mutations and to investigate genotype-phenotype correlations., Methods: We sequenced the exome or screened the exons of SYNGAP1 in a total of 251 patients with neurodevelopmental disorders. Molecular and clinical data from patients with SYNGAP1 mutations from other centres were also collected, focusing on developmental aspects and the associated epilepsy phenotype. A review of SYNGAP1 mutations published in the literature was also performed., Results: We describe 17 unrelated affected individuals carrying 13 different novel loss-of-function SYNGAP1 mutations. Developmental delay was the first manifestation of SYNGAP1-related encephalopathy; intellectual disability became progressively obvious and was associated with autistic behaviours in eight patients. Hypotonia and unstable gait were frequent associated neurological features. With the exception of one patient who experienced a single seizure, all patients had epilepsy, characterised by falls or head drops due to atonic or myoclonic seizures, (myoclonic) absences and/or eyelid myoclonia. Triggers of seizures were frequent (n=7). Seizures were pharmacoresistant in half of the patients. The severity of the epilepsy did not correlate with the presence of autistic features or with the severity of cognitive impairment. Mutations were distributed throughout the gene, but spared spliced 3' and 5' exons. Seizures in patients with mutations in exons 4-5 were more pharmacoresponsive than in patients with mutations in exons 8-15., Conclusions: SYNGAP1 encephalopathy is characterised by early neurodevelopmental delay typically preceding the onset of a relatively recognisable epilepsy comprising generalised seizures (absences, myoclonic jerks) and frequent triggers., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/) more...

Different mutations occurring in the unstable CGG repeat in 5' untranslated region of FMR1 gene are responsible for three fragile X-associated disorders. An expansion of over ∼200 CGG repeats when associated with abnormal methylation and inactivation of the promoter is the mutation termed 'full mutation' and is responsible for fragile X syndrome (FXS), a neurodevelopmental disorder described as the most common cause of inherited intellectual impairment. The term 'abnormal methylation' is used here to distinguish the DNA methylation induced by the expanded repeat from the 'normal methylation' occurring on the inactive X chromosomes in females with normal, premutation, and full mutation alleles. All male and roughly half of the female full mutation carriers have FXS. Another anomaly termed 'premutation' is characterized by the presence of 55 to ∼200 CGGs without abnormal methylation, and is the cause of two other diseases with incomplete penetrance. One is fragile X-associated primary ovarian insufficiency (FXPOI), which is characterized by a large spectrum of ovarian dysfunction phenotypes and possible early menopause as the end stage. The other is fragile X-associated tremor/ataxia syndrome (FXTAS), which is a late onset neurodegenerative disorder affecting males and females. Because of the particular pattern and transmission of the CGG repeat, appropriate molecular testing and reporting is very important for the optimal genetic counselling in the three fragile X-associated disorders. Here, we describe best practice guidelines for genetic analysis and reporting in FXS, FXPOI, and FXTAS, including carrier and prenatal testing. more...

Background: People with diabetes mellitus are instructed to clean their skin prior to self-monitoring of blood glucose to remove any dirt or food residue that might affect the reading. Alcohol-based hand sanitizers have become popular when soap and water are not available. The aim of this study was to determine whether a hand sanitizer is compatible with glucose meter testing and effective for the removal of exogenous glucose., Methods: We enrolled 34 nonfasting subjects [14 male/20 female, mean ages 45 (standard deviation, 9.4)] years, 2 with diagnosed diabetes/32 without known diabetes]. Laboratory personnel prepared four separate fingers on one hand of each subject by (1) cleaning the second finger with soap and water and towel drying (i.e., control finger), (2) cleaning the third finger with an alcohol-based hand sanitizer, (3) coating the fourth finger with cola and allowing it to air dry, and (4) coating the fifth finger with cola and then cleaning it with the instant hand sanitizer after the cola had dried. Finger sticks were performed on each prepared finger and blood glucose was measured. Several in vitro studies were also performed to investigate the effectiveness of the hand sanitizer for removal of exogenous glucose.z, Results: Mean blood glucose values from fingers cleaned with instant hand sanitizer did not differ significantly from the control finger (p = .07 and .08, respectively) and resulted in 100% accurate results. Blood glucose data from the fourth (cola-coated) finger were substantially higher on average compared with the other finger conditions, but glucose data from the fifth finger (cola-coated then cleaned with hand sanitizer) was similar to the control finger. The data from in vitro experiments showed that the hand sanitizer did not adversely affect glucose meter results, but when an exogenous glucose interference was present, the effectiveness of the hand sanitizer on glucose bias (range: 6% to 212%) depended on the surface area and degree of dilution., Conclusions: In our study, use of an instant hand sanitizer was compatible with the results of a blood glucose monitor and did not affect finger stick blood glucose results. However, depending on surface area, hand sanitizers may not be adequate for cleaning the skin prior to glucose meter testing., (© 2011 Diabetes Technology Society.) more...

Brachmann-De Lange Syndrome (BDLS, MIM 122470) is a rare multiple congenital anomaly/mental retardation syndrome characterized by a variable phenotype including intrauterine fetal growth retardation, limb reduction and distinctive facial and skull features (low frontal hairline, synophrys, anteverted nostrils, long philtrum, downturned corners of the mouth, micro- and retrognathia, low-set ears and micro-/brachycephaly), as well as a significant psychological developmental delay. A proposed classification system for BDLS include a classic type with characteristic facial and skull changes, a mild type where similar changes may develop with time or may be partially expressed, and a third type including phenocopies, where phenotypic changes are casually related to chromosomal aneuploidies or teratogenic exposures. We report on a 22-week gestation fetus with BDLS, showing intrauterine fetal growth retardation, brachycephaly, micro-/retrognathia and monolateral single bone of the forearm, in a woman harboring diffuse large B-cell lymphoma. Meticulous family history was negative for malformations, syndromes, congenital anomalies or psychiatric disorders. There are very few reports of BDLS at early gestation, but to the best of our knowledge, this is the first case occurring simultaneously with a hematological neoplastic disease of the mother. more...

The aim of this study was to quantify the effects of three different configurations of cardiomyoplasty on coronary blood flow in an acute dog model. Thirteen dogs had both latissimus dorsi muscles harvested and transposed to the chest. Coronary blood flow was measured using Doppler cuff probes on the left anterior descending and circumflex coronary arteries during each of three cardiomyoplasty configurations: left posterior, right anterior, and double. Multiple beat measures were made of systolic and diastolic flow during a control protocol and a subsequent protocol with the muscle(s) paced. Significant flow reductions during pacing were observed in the left anterior descending coronary artery during left posterior (17%, p = 0.003), right anterior (29%, p < 0.0001), and double (35%, p = 0.0001) myoplasty. Similar reductions occurred in the circumflex artery (14%, p = 0.0009; 20%, p = 0.001; 27%, p = 0.0053). The net flow over an entire pacing cycle also was reduced significantly: left anterior descending artery (11%, p = 0.0035; 23%, p = 0.0001; 23%, p = 0.0047) and circumflex artery (10%, p = 0.0025; 17%, p = 0.0018; 21%, p = 0.0091). Thus, in the acute setting cardiomyoplasty depresses coronary blood flow. A chronic setting will be needed to determine the ultimate significance of these results. more...

Objectives: We hypothesized that by enhancing parasympathetic activity, low dose transdermal scopolamine would increase heart rate variability after myocardial infarction., Background: Low heart rate variability is associated with increased mortality after acute myocardial infarction., Methods: Conventional time domain heart rate variability was measured from 24-h Holter recordings of 61 consecutive male patients (mean age 58 +/- 10 years, left ventricular ejection fraction 44.7 +/- 15.5%) 6 days (median) after acute myocardial infarction. Patients were then randomly assigned to wear one patch of transdermal scopolamine or a matching placebo patch for 24 h, during which their 24-h heart rate variability was remeasured., Results: Compared with placebo, transdermal scopolamine caused a significant increase in time domain measures of 24-h heart rate variability by 26% to 35% above baseline. Transdermal scopolamine was well tolerated., Conclusions: Low dose transdermal scopolamine safely increases cardiac parasympathetic activity and short-term heart rate variability after acute myocardial infarction. Whether the effect of transdermal scopolamine on heart rate variability is a reasonable surrogate for improvement of long-term morbidity and mortality requires an appropriate designed investigation. more...

A new configuration of double cardiomyoplasty was designed according to studies of the length-tension properties of the linear latissimus dorsi muscle. Four dogs had both their right and left latissimus dorsi muscles dissected from the chest wall and attached to a tensiometer to measure force of contraction. The maximum active tension obtained with stimulation of the linear latissimus dorsi muscle was observed when the muscle was at its resting anatomic length and up to 5% above this length. Eight dogs had a double cardiomyoplasty in which the resting anatomic length of both muscles was maintained. Control hemodynamic parameters obtained with the muscles at rest were compared with stimulated muscle protocols. In a normal heart state, stimulation of the double cardiomyoplasty increased the cardiac output 32% (p < 0.05), the stroke volume 39% (p < 0.05), and the left ventricular pressure 42% (p < 0.05). When acute heart failure was induced with high-dose intravenous propranolol (5 mg/kg), stimulation of the double cardiomyoplasty increased the cardiac output 32% (p = 0.01), the stroke volume 32% (p < 0.05), rate of pressure rise 39% (p < 0.01), and myocardial thickening 39% (p < 0.01). The study demonstrated that this configuration of double cardiomyoplasty provides significant hemodynamic assistance in the normal and acutely failing canine heart. more...

To determine the long-term influence of the severity of preoperative diabetes mellitus on the results of coronary bypass, a review was made of 212 diabetics operated on between 1968 and 1973, of whom 87 patients (41%) were receiving no drugs, 108 patients (50.9%) were receiving oral hypoglycemic agents, and 17 patients (8%) were receiving insulin. They were compared with 1,222 nondiabetic patients operated on over the same period. Perioperative mortality was similar in the diabetics and nondiabetics: 7.1% vs 4.5%. Improvement in anginal symptoms was similar in all patient groups: 85.9% to 92.7%. Overall 15-year survival probability was .53 for the nondiabetic group, .43 for the diabetics not receiving drugs, .33 for those receiving oral agents, and .19 for the insulin-treated patients. Late graft patency ranged from 78% to 90% and was comparable in all groups. The preoperative blood glucose level was an important predictor of late mortality in all diabetic patients. Thus, coronary bypass surgery was effective in all groups of diabetic patients in long-term relief of anginal symptoms. Intermediate-term survival rates were good in all groups, but the initial severity of the diabetes was an important determinant of long-term survival rates. more...

A semiautomated system for the rapid evaluation of left ventricular regional wall motion is described. The system includes direct projection of the cineangiogram film on an X-Y digitizer which is interfaced with a PDP-9 computer. The ventricular model used to evaluate regional wall motion and the methods of data analysis are described. Validation of the accuracy of the technique and specific clinical applications are presented. It is concluded that this technique provides a rapid means of evaluating left ventricular regional wall motion and that the accuracy of the technique is acceptable for clinical application. more...

This report is concerned with the factors influencing survival in 920 consecutive patients submitted to operation for infrarenal abdominal aortic aneurysm during the past 25 years. Rupture had occurred in 60 patients (6.5%) and survival was 77%, which did not vary during the period of study. Of the 860 patients (93.5%) treated for nonrupture, 819 (95%) survived operation. The mortality rate in this group varied from 18%, in the earlier period to 1.43% , in recent years. Risk factors including heart disease, hypertension, and advanced age accounted for 95% of the deaths that occurred within 30 days however, the mortality rate in patients with these problems decreased from 19.2% to 1.9% during the period of study although the average number of patients treated each year with these risk factors increased tenfold. Improvements in operative techniques and experience were responsible for decreasing mortality up to about 1971. Subsequent decrease in mortality was due to improvements in anesthesia, monitoring, and other supportive measures during operation and the early recovery period. Complete survival information was obtained in 816 (99.6%) patients, 191 of whom had been treated for periods over 15 years. Factors influencing long-term survival were associated disease and age at time of operation. Survival in percentage in patients without associated heart disease of hypertension for 5, 10, and 15 years was 84, 49, and 21; with heart disease, it was 54, 34, and 17. The median age of patients in the series was 65.5 years and survival at above intervals according to quartile was less than or equal to 60; 71, 53, and 24; 60 less than age less than or equal to 71; 66, 38, and 18; less greater than 71; 43, 13, and 11. more...

To determine which preoperative variables were most predictive of long-term survival after coronary bypass surgery, the status of 1,448 consecutive patients was determined 10 to 14 years after operation. All patients were operated on at least 10 years ago and follow-up at 10 years was 93.8% complete. The overall 14-year survival probabilities were: for one-vessel disease, 73.3%; for two-vessel disease, 45.9%; for three-vessel disease, 34.2%; and for left main coronary artery disease, 41.9%. Patients with good left ventricular function had an overall survival rate of 53.3%, and patients with poor ventricular function had a survival rate of 31.9%. Preoperative variables predictive of greater risk of total mortality were: digoxin usage, multivessel disease, poor quality of left ventricular function, age at operation, electrocardiographic evidence of myocardial infarction, previous stroke, diabetes, heart failure, diuretic usage, cigarette smoking, and residual ungrafted coronary artery disease. The major determinants of long-term survival were variables associated with preoperative left ventricular function. Diabetes was the only important metabolic risk factor identified. This study suggests that unfavorable preoperative conventional risk factors should not be considered a contraindication to operation in patients with adequate coronary anatomy and left ventricular function. more...

In order to determine the late results of reconstructive surgery for renovascular disease, a review was made of a series of 505 consecutive patients who underwent operation over a 20-year period. There were 257 males (50.9%) with an age range of 3-80 years. Renal artery bypass grafts were used in 75.4% (471/625) and thromboendarterectomy and/or patch angioplasty in 15.0% (94/625) of reconstructions. Associated vascular procedures were performed in 38.0% (186/489) of patients. Operative mortality (30-day) was 1.8% (9/489) overall, and 4.8% (9/186) with associated procedures but there was no operative mortality in 303 consecutive isolated renal artery reconstructions. The blood pressure was normal or improved in 65% of patients at a mean follow-up interval of 49.3 months, range 1-240 months. The best response rate was obtained in younger patients with isolated renal lesions. Linear regression analysis showed age at operation to be the most important determinant of blood pressure response (p < 0.003) with the presence or absence of diffuse atherosclerosis as another but less powerful determinant of responsiveness (p < 0.07). Crude 15-year survival was 70% (340/489). The overall five- and ten-year actuarial survival probabilities were 80 and 62% respectively. The most common causes of death were myocardial infarction, stroke, and cancer. Cox regression analysis for variables influencing survival indicated that age at operation (p < 0.001), sex (p < 0.01) and the presence or absence of fibromuscular disease (p < 0.002) were the major determinants of late survival with persistent severe hypertension exerting an important but lesser influence. The results of this study indicate that about two-thirds of patients will experience long-term relief of hypertension after operation and that the best long-term survival and blood pressure relief will be obtained in patients less than 50 years of age. Because hypertension in females is better tolerated, younger, male patients appear to have the most to gain from successful renovascular reconstruction. more...

Numerous studies have demonstrated the superiority of membrane oxygenators (MO) over the bubble oxygenators (BO) when used for prolonged cardiopulmonary support. However, there is little information available evaluating the MO for routine, short-term cardiopulmonary bypass. In this study the 5MO314 Modulung-Teflo (MO) was compared to 5M30314 Miniprime Variflo (BO). The data of 91 patients (46 MO and 45 BO) were analyzed according to the duration of cardiopulmonary bypass (Group I less than 60 min., Group II 60-90 min. and Group III greater than 90 min.). Hemodynamic parameters, fluid and blood balance, as well as hematologic and blood gas studies were used for comparing the two oxygentors. The hemodynamic parameters were better, and the arterial blood gases were more physilogic with the MO. The postoperative blood loss was significantly less when using the MO. The other measurements documented the stability of the MO. All statements were based on statistical analysis with a DEC PDP-9 computer, using the MIIS language and operating system. Consequently, we are now using this MO for routine cardiopulmonary bypass. more...

Between July 1979 and December 1984, 785 patients received 815 St. Jude Medical valve prostheses. Valve-related mortality in the follow-up period was due to thromboembolism in seven cases, anticoagulant-related hemorrhage in three and perivalvular leak in two. Freedom from valve-related death or reoperation at 3 years was 96.4% for aortic valve replacement and 98.3% for mitral valve replacement. The overall rate of thromboembolism was 2.6%/patient-year with warfarin, 9.2%/patient-year with antiplatelet medication and 15.6%/patient-year in patients with no anticoagulant therapy. One episode of thrombotic obstruction of a mitral valve, in a patient receiving no anticoagulant therapy, resulted in an occurrence rate of such obstruction of 0.22%/patient-year. Valve replacement with the St. Jude valve produced excellent clinical results, but long-term anticoagulation with warfarin was required to minimize thromboembolic complications. The use of antiplatelet agents alone provided inadequate protection. more...

Graft inclusion and vessel reattachment to openings made in the graft were employed in the treatment of 605 patients with thoracoabdominal aortic aneurysms. These patients were divided into four groups on the basis of the extent of aneurysm. Group I consisted of those patients with involvement of most of the descending thoracic and upper abdominal aorta; group II involved most of the descending thoracic aorta and most or all of the abdominal aorta; group III involved the distal descending thoracic aorta and varying segments of abdominal aorta; and group IV involved most or all of the abdominal aorta including the segment from which the visceral vessels arose. The cause of aneurysm formation was medial degenerative disease in 80%, and dissection in 17%; other causes were responsible in the remaining 3%. The median age was 65 years and associated diseases including aneurysms involving other segments, atherosclerotic occlusive disease, heart disease, chronic obstructive pulmonary disease (COPD), hypertension, and renal insufficiency were frequent. The aneurysm was symptomatic in 70% of cases and rupture had occurred in 4% of cases. There were 54 (8.9%) early (30-day) deaths and 151 late deaths; 400 (66%) patients were still alive 3 months to 20 years after operation, including 60% at 5 years. Statistically significant pre- and intraoperative variables by univariate analysis that were predictive of increased risk of early death were advancing age, associated diseases that included COPD, renal artery occlusive disease, atherosclerotic heart disease, renal insufficiency, and long aortic clamp time. Three of these (age, clamp time, and the presence of COPD) retained significance by multivariate analysis. Variables predictive of risk of late death were age, dissection, extent of aneurysm, rupture, heart disease, cerebrovascular disease, COPD, hypertension, and poor renal function. Age, rupture, renal dysfunction, extent of aneurysm, and dissection retained their significance by multivariate analysis. Variables predictive of neurologic disturbances of the lower extremities included rupture, reattachment of intercostal and lumbar arteries, clamp time, dissection, extent and age. Rupture, reattachment of vessels, dissection, and extent of aneurysm retained significance by multivariate analysis. Thus, the risk of this complication was greatest in patients with extensive lesions (group II) with aortic dissection. The greatest risk of renal failure after operation that required dialysis was in patients who had impaired renal function before operation. Methods employed did not prevent these complications. more...

The records of 13,827 patients admitted on one or more occasions to The Methodist Hospital in Houston on the service of the senior author for the treatment of arterial atherosclerotic occlusive disease from 1948 to 1983 were analyzed. The data derived from this analysis are believed to support the concept that atherosclerotic occlusive disease tends to assume characteristic patterns that may be classified, by predominant site or distribution of the disease, into five major categories: (I) the coronary arterial bed, (II) the major branches of the aortic arch, (III) the visceral arterial branches of the abdominal aorta, (IV) the terminal abdominal aorta and its major branches, and (V) a combination of two or more of these categories occurring simultaneously. Category IV had the highest proportion of patients (about two-fifths), Category I the second highest (almost one-third), and Category III had the lowest percentage (3%). Atherosclerotic occlusive disease in all categories tends to be well localized and usually occurs in the proximal or midproximal portions of the arterial bed. Such lesions are amenable to effective surgical treatment directed toward restoration of normal circulation. Less commonly, however, the occlusive disease in all categories occurs predominantly in the distal portions of the arterial bed, and such lesions are usually not amenable to effective surgical treatment. Patients in Categories I and III were significantly younger than those in the other categories and, although males predominated in all categories, Categories II and III contained significantly more female patients than did the other categories. In general, however, female patients behaved like male patients in virtually all aspects of the study. The rates of progression of the disease may be classified into: rapid (0 to 36 months), moderate (37 to 120 months), and slow (more than 120 months). The rapid and moderate rates of progression occurred most frequently in Categories II and IV, and the moderate and slow rates occurred most frequently in Category I. The possibility for development of recurrence or progression of disease in the same category and in a new category was significantly greater in younger patients. The patient's sex had no significant influence in this regard. Among the various categories, patients in Category IV had the highest incidence of development of disease in a new category, and Category I had the lowest incidence. Patients originally in Category II had a somewhat greater tendency to development of disease in Category IV, and patients originally in Category IV, for development of disease in Category II.(ABSTRACT TRUNCATED AT 400 WORDS) more...

Over a 30-year period, 190 consecutive patients had repair of coarctation of the descending thoracic aorta at a mean age of 25 years (range, 1 to 60 years); 130 were male. Median preoperative blood pressure (BP) was as follows: systolic, 160 mm Hg (range, 94 to 300 mm Hg) and diastolic, 90 mm Hg (range, 50 to 160 mm Hg). Dacron grafts were used in 64.7% of patients. Follow-up was obtained at a mean interval of 84.9 months, range, one to 360 months. Survival status was established for 86.1% (163/190) of patients. Postoperatively, the median BP was 133 mm Hg systolic (range, 90 to 195 mm Hg) and 80 mm Hg diastolic (range, 50 to 120 mm Hg). Overall, 80% of patients were either normotensive or had mild hypertension after operation. The best BP response and late survival were in patients operated on under 13 years of age. This study further confirms that early operation (below 5 years of age) is desirable. However, contrary to some previous reports, patients operated on as adolescents, despite some mild residual hypertension, had an excellent long-term prognosis. Patients over 21 years of age at operation had a high rate of persistent hypertension and experienced other serious cardiovascular complications. more...

During a period of slightly over 25 years, 949 new patients with aortoiliac atherosclerotic occlusive disease--409 (43%) with associated distal disease--were submitted to various reconstructive operations for claudication in 719 and rest ischemic problems in 230 patients. The ages ranged from 21 to 91 years with a medial age of 59. Men outnumbered women 2.5 to 1. Associated diseases were present in 695 (75%); heart disease and diabetes were most common. The mortality rate from operation--50% of which was from heart disease--8% in first 5 years and 3% during the last 15 years. Good function, i.e., restoration of femoral pulses, leg salvage, and relief of symptoms, was achieved in 95% of cases early after treatment. Early results were best in patients with claudication and those without associated distal disease. The long-term survival rates were significant--50%, 30%, and 15% at 10, 15, and 20 years, respectively--and successful function was maintained in survivors in 79%, 70%, and 56% at the same intervals. Amputation was performed in only 23 (3%) patients with claudication and 33 (14%) with rest ischemia during the period of study. Survival, functional results, and incidence of amputation varied with the numerous factors described in detail herein. more...

The goal of pacemaker follow-up is not only to detect battery depletion but also to detect all malfunctions of the pacing system and, when possible, to correct such problems using programming. During one year, we discovered 61 such malfunctions in a clinic of 1065 patients (5.7%). These were more frequent in the first year (7.7%) than in the third to fifth years of follow-up (range 3.1-4.8%). The incidence rose again in the sixth and subsequent years (7-7.7%). Despite a significant occurrence of malfunctions (5.2%) among multiprogrammable pacemakers, the necessity for operative intervention for their correction was low (1.2%). Sensing problems were the most common (57%) and the most likely to be corrected by reprogramming (85%); problems involving loss of capture were less likely to be corrected by programming (38.5%). Battery depletion accounted for only 18% of malfunctions, occurring earliest in the forty-third month of follow-up. Pulse generator longevity of those devices reaching end of battery life during the study period was 68.6 +/- 16.7 months (mean + SD). We conclude that specialized pacemaker follow-up continues to be necessary despite improved pulse generator reliability and longevity. Indeed, with reprogramming, it presently plays an even more important role than in the past. Follow-up should be oriented not only to the detection of battery depletion but also toward a comprehensive surveillance of pacemaker system function. more...