Coarctation of aorta in a 52-year-old female

Coarctation of the aorta is a complex vascular lesion that usually originates distal to the left subclavian artery. It accounts for 5-10% of all congenital cardiovascular malformations and carries a high morbidity and mortality risk. Symptoms depend on the severity of the disease and other coexisting anomalies. Early in life, diagnosis is usually based on significant symptoms and physical findings. Many patients remain asymptomatic until adulthood, with most being diagnosed in the 2nd or 3rd decade. In this case report, we present a late diagnosis of aortic coarctation with a bicuspid aortic valve in a 52-year-old female. Our patient was relatively asymptomatic until she presented with chest discomfort, fatigue and dyspnea in her fifth decade of life. Based on the clinical presentation and the markedly elevated gradient, the patient was referred for corrective therapy.     

Congenitally corrected transposition of the great arteries with coexisting stenosis of pulmonary trunk valve and ventricular septum defect in a 55-year-old woman--a case report

Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation. This anomaly is characterised by atrioventricular as well as ventriculoarterial discordance. Isolated CCTGA may cause no symptoms until adult life. Most CCTGA cases with concomitant cardiac abnormalities are symptomatic and are therefore usually diagnosed in childhood. In the majority of patients, congestive heart failure secondary to right ventricular dysfunction occurring by the fifth or sixth decade enables diagnosis. We present an oligosymptomatic 55-year-old woman with corrected transposition of the great arteries and coexisting stenosis of pulmonary trunk valve and ventricular septum defect.     

Eisenmenger syndrome: a case of survival after ventricular tachycardia due to inferior myocardial infarction in a 48-year-old patient with congenital large ventricular septal defect

Eisenmenger syndrome is the most common consequence of congenital cyanotic heart disease seen in adults; survival to the fifth decade of life is rare. Death is very difficult to predict: it is related to sudden cardiac ventricular arrhythmia, massive hemoptysis and right heart failure. In this paper, a patient with ventricular septal defect and Eisenmenger reaction is described. The patient was relatively well until 48 years of age, when she underwent surgery because of a cerebral abscess without cerebral complications but with some deterioration of her cardiac function. After discharge, the patient was readmitted to the hospital because the electrocardiogram showed persistent ST inferior elevation. Echocardiography demonstrated poor contractility and inferior akinesia. Sudden ventricular tachycardia occurred and the patient became unconscious. She was successfully resuscitated and, following a period of ventilation, the hemodynamics stabilized and she was discharged 17 days later. She remained well two years later.     

Colloid cyst of the third ventricle

Colloid cyst of the third ventricle is a rare, histologically benign but potentially lethal tumor. Two cases reflecting the variability in symptoms, physical findings, and outcome seen with these rare tumors are presented. One patient with classic age of onset in the third to fifth decade of life, symptoms of escalating intermittent headaches, and signs of papilledema and altered level of consciousness had successful surgical removal of the cyst. Another patient with nonspecific symptoms well outside the usual age of onset and without papilledema succumbed to sudden, complete impaction and was not diagnosed until autopsy.     

Graves' disease. Influence of age on clinical findings

To determine the influence of age on the signs and symptoms of hyperthyroidism we prospectively examined 880 patients and matched them by age, sex, race, and visit date with similarly examined euthyroid control subjects. Many signs and symptoms showed little change with age until after the fifth decade of life when they began to decrease gradually. Findings that increased with age were weight loss and atrial fibrillation, while those that decreased most markedly with age were increased appetite and weight gain. The diagnosis is more difficult as age progresses because there are fewer findings and because the significance of those present may not be appreciated. Identification of the most sensitive and specific signs and symptoms in each age decade should improve the early detection of hyperthyroidism.     

Some Variations in the Normal Haemoglobin Concentration

In a study of the haemoglobin concentrations of 408 male and 789 female blood donors aged 18–65, it was found that in both sexes the haemoglobin level, except for a peak in the middle of the third decade, remained constant until the fifth decade. From this period to the age of 65, there was a progressive and significant decrease of haemoglobin concentration in men and a progressive and significant increase of haemoglobin concentration in women. An initial difference in haemoglobin level, between men and women, of almost 2 g/100 ml in the early twenties was reduced to just over 1 g/100 ml in the middle sixties. Possible reasons for the above differences were discussed with emphasis on the effects of hormonal environment and iron deficiency.     

Natural history of Fabry disease in males: preliminary observations

A large cohort of patients with Fabry disease is being studied to determine the natural history of the disease and how this relates to the specific mutation involved and the amount of residual α-galactosidase A activity. To date, we have investigated the progression of cerebral lesions and stroke, as identified by magnetic resonance imaging, and renal disease. Results have shown that cerebral lesions do not appear until 23 years of age, but are present in all patients by55 years of age. The peak onset of proteinuria occurred in the fourth decade, and the peak onset of chronic renal insufficiency and end-stage renal disease occurred in the fifth decade of life. Renal outcome was related to the type of mutation and residual enzyme activity. Data from these studies in untreated patients will be important when assessing the long-term efficacy of enzyme replacement therapy.     

Physiotherapy management of cystic fibrosis

For many years physiotherapy for cystic fibrosis (CF) was synonymous with a daily routine of postural drainage and percussion. Improved longevity and a desire for independence, together with a clearer understanding of the pathophysiology of the disease, has led to the development of many airway clearance techniques. The contribution of 'chest' physiotherapy remained unchallenged until recent times when lack of robust evidence and the presentation of asymptomic infants and adults led some physiotherapists to question this traditional approach. As survival increases into the fourth and fifth decade unique complications have evolved and physiotherapy management is challenged with nonrespiratory aspects of treatment. This review will consider the evidence for, and current practice of airway clearance, the value of exercise and the emerging problems of musculoskeletal complications and incontinence.     

Corrected transposition of the great arteries without associated defects in adult patients: clinical profile and follow up.

OBJECTIVE--To assess the clinical course of adult patients with corrected transposition of the great arteries without associated anomalies. DESIGN--All patients with corrected transposition of the great arteries without associated anomalies were reviewed with complete clinical and echocardiographic assessment. The complications were evaluated in each decade. SETTING--Tertiary centre with a specific unit dealing with "grown-up" adolescent and adult congenital heart disease, designated as a quaternary centre and a general hospital with a referral centre for "grown-up" congenital heart disease. PATIENTS--18 patients (nine male and nine female) aged 16-61 years followed for 1-30 years (mean 10 years). RESULTS--There were no deaths. Six patients had a worsening ability index during follow up. Complications were: (a) complete heart block in seven, three of whom required pacemaker insertion; (b) significant left atrioventricular valve regurgitation in 50%, appearing only in the third decade (12%), with increasing frequency thereafter. Infective endocarditis was responsible for increasing left atrioventricular valve regurgitation in only one patient; (c) supraventricular arrhythmia appeared in the fifth decade, and occurred in all patients over the age of 60 years. One patient aged 61 had recurrent sustained ventricular tachycardia; and (d) congestive heart failure developed only after 50 years in 66%. One patient had severe left atrioventricular valve regurgitation; the function of the systemic ventricle was only moderately reduced in the other three. Three of the nine women had seven uneventful pregnancies. CONCLUSIONS--Patients with corrected transposition of the great arteries without associated defects may remain undiagnosed until adult life. Symptoms occur rarely before the fourth and fifth decades, when rhythm disturbance, left atrioventricular valve regurgitation, and moderately impaired systemic ventricular function cause congestive cardiac failure. The role of pacemaker insertion or surgery for left atrioventricular valve regurgitation needs further assessment.     

Quadricuspid aortic valve with a hidden left ostium: Case report and literature review

Quadricuspid aortic valve (QAV) is a rare congenital condition that frequently progresses to aortic regurgitation with clinical impact in adulthood. Surgical treatment is required in the fifth to sixth decade of life in about one fifth of patients.We describe the case of a 64-year-old woman with regular cardiological follow-up for severe aortic valve regurgitation who had suffered recent clinical and echocardiographic deterioration. Conventional open surgery was indicated. During the procedure, a QAV with leaflet retraction and central orifice was observed. The aortic valve was successfully replaced.     

Long-term survival of childhood coronary artery disease in a patient severely affected with familial hypercholesterolemia

This brief account of a patient severely affected with familial hypercholesterolemia and with coronary artery disease since age 13 reviews his therapy and survival into the fifth decade of life. This is heretofore unreported in phenotypically homozygous patients with familial hypercholesterolemia in the era before statin drugs, low-density lipoprotein apheresis, or their combination.     

Tricuspid and pulmonary atresia with coarctation of the aorta: a rare combination possibly explained by persistence of the fifth aortic arch with a systemic-to-pulmonary arterial connection

A ten-day old baby was found to have tricuspid and pulmonary atresia, and a "ductal-dependent" pulmonary circulation that was prostaglandin-sensitive. An unusual coarctation of the aorta was not recognised until an autopsy was performed, some 11 days after construction of an aortopulmonary shunt. A previously undefined anomaly in this neonate was persistence of the fifth aortic arch with a systemic-to-pulmonary artery connection on the same side as the definitive aorta. The histology of the fifth aortic arch is consistent with the pattern of an arterial duct.     

Gerovital-H3: a clinical trial as an antidepressant.

Twenty-five volunteers, all in their fifth decade or beyond, all with mild to moderate, nonpsychotic depression of at least several months' duration participated in a double-blind study of Gerovital vs placebo. There was no significant difference between the Gerovital and placebo groups; both groups showed significant improvement on self-rating as well as observer rating scales.     

Ultrasonographic evidence of association of polyps and stones with gallbladder cancer

OBJECTIVE: The purpose of this study was to evaluate the level of gallbladder cancer risk associated with polyps and stones of the gallbladder by ultrasound examinations. METHODS: We investigated abdominal ultrasonographic findings for gallstones and polyps of the gallbladder and the relationship of these findings to malignancy in 194,767 asymptomatic participants in health screening examinations. RESULTS: Gallstones were detected in 7,985 participants (4.1%), at a prevalence of 5.3% in male and 4.5% in female participants. The prevalence was highest in the participants' seventh decade, followed by the sixth and the fifth decades, in both male and female participants. Polyps were found in 10,926 (5.6%), at a prevalence of 6.9% in the male and 4.5% in the female participants. In the male participants, prevalence was highest in the fourth decade, followed by the third and the fifth decades, whereas in the female participants it was highest in the fifth decade, followed by the fourth and third. High prevalence of polyps > or = 10 mm in diameter was found in the fifth to seventh decades in both sexes. Nineteen participants (0.01%) were diagnosed as having gallbladder cancer, and many of them were in the sixth or seventh decade in both sexes. Patients with stones showed a higher prevalence of gallbladder cancer. This difference was shown to be statistically significant by age-adjusted analysis (p < 0.01). The age distribution of the participants with polyps > or = 10 mm in diameter was correlated with that of patients diagnosed with gallbladder cancer. Furthermore, polyp incidence was significantly related to cancer by statistical analysis (p < 0.01). CONCLUSION: These results suggest an association between gallstone or polyp of the gallbladder and increased risk of malignancy, implying that an etiological association may also exist.     

Long‐term outcomes in 3 cases of quadricuspid aortic valve,using a new classification system: A case series and literature review

Quadricuspid aortic valve has been classified based on leaflet size. However, no association is seen between classification and severity of aortic regurgitation (AR). Bicuspid aortic valve is classified according to the number of cusps, with significantly higher prevalence of AR in cases with a raphe. We classified cases according to raphe number. In 1 patient with no raphe, AR severity did not change into the eighth decade. However, AR severity worsened in patients with a raphe, in 1 case requiring aortic valve replacement in the fifth decade. Unequal shear stress may lead to leaflet fibrosis and progressive AR.     

Two- and three-dimensional echocardiography in primary antiphospholipid syndrome: misdiagnosis as rheumatic valve disease

This is a report of a woman in the fifth decade of life with primary antiphospholipid syndrome and involvement of a heart valve. Diagnosis was reached with echocardiography and serological studies.     

Cystic dystrophy in heterotopic pancreas:a rare indication for pancreaticoduodenectomy

BACKGROUND:Cystic dystrophy in heterotopic pancreas (CDHP)is a rare benign condition characterized by the presence of cysts in the wall of the digestive tract associated with inflammation and fibrosis,intermingled with heterotopic pancreatic tissue.Treatment options for CDHP are poorly defined. METHOD:We report a case of CDHP,and review the literature focusing on the diagnosis and management. RESULTS:CDHP is mainly encountered in men in the fifth decade of life in association with chronic pancreatitis secon...     

Outcome of lupus renal disease in the past two decades: a report from China

Objective: To evaluate the differences in the renal survival of lupus nephritis (LN) diagnosed either during 1985–1994 or 1995–2004 and to analyse the possible causes. Methods: Fifty‐two patients with biopsy‐confirmed LN were followed up between 1985 and 1994 and 130 patients were followed up between 1995 and 2004. Renal survival was studied with Kaplan‐Meier analysis and the log rank test. Status at diagnosis and treatment schedules were also analysed. Results: Renal survival was significantly better in the patients who were diagnosed between 1995 and 2004 (P = 0.0233). The mean time from initiation until first diagnosis of SLE, from the initiation of SLE until referral to our centre, and from first detection of proteinuria until kidney biopsy was significantly shorter in the later decade (P < 0.05). In the decade from 1995 to 2004 there was significantly lower rates of early renal dysfunction and fewer histological signs of chronicity at the time of diagnosis of LN than in the decade from 1985 to 1994. In treatment schedules, more patients received intravenous cyclophosphamide (CTX) in a bolus in the later decade group (P = 0.014). Besides CTX, some new immunosuppressive agents such as cyclosporin A or mycophenolate mofetil were received by more patients in the decade from 1995 to 2004. Conclusions: The outcomes for patients with LN was significantly better between 1995 and 2004 than between 1985 and 1994. The result is maybe attributable to earlier diagnosis, earlier treatment, intravenous bolus CTX and more immunosuppressive agents from which to select.     

Gradual Onset of Dysphagia: A Study of Patients with Oculopharyngeal Muscular Dystrophy

This study describes five patients with slowly developing dysphagia secondary to oculopharyngeal muscular dystrophy (OPMD), a progressive neurological disorder characterized by gradual onset of dysphagia, ptosis, and facial and trunk limb weakness. OPMD is a genetic disorder that affects formerly healthy adults who typically begin to experience symptoms in the fourth or fifth decade of life. Despite the debilitating nature of the disease, it is common for affected individuals to live to old age. Because of the gradual progression of dysphagia, as well as the deterioration of articulation, resonance, and breath support, patients with OPMD may come to the attention of physicians, nurses, and speech pathologists before a diagnosis is made. We hope to heighten awareness of how these subjects developed strategies to cope with their swallowing problems without medical intevention until the disease was producing marked symptoms. Patients with suspected dysphagia should be questioned about overt problems with eating and swallowing, but also about their adaptations and compensatory strategies. A Clinical Interview Questionnaire is included that may yield additional information about hidden dysphagia.     

Recent concepts of the organization and structure of human trabecular bone--results of combined 2- and 3-dimensional analysis

Every loss of bone mass caused by dysfunction of the endocrine glands and mechanically, respectively, is accompanied by changes of the bone structure. For the pathogenetic mechanisms which in man lead to the abolition of the bone structure in osteoporosis the knowledge of the physiological principles of construction is a basic prerequisite. Hitherto performed studies have shown that with the help of two-dimensional section-cuttings a tridimensional reconstruction is either not possible or can be carried out only by means of serial sections and extensive computer procedures in circumscribed parts. For the analysis of the tridimensional structure of the spongiosa a new method of preparation was developed, which renders possible the simultaneous two-dimensional and tridimensional evaluation of the bone tissue. Seven spinal columns of deceased without disease of the skeleton (donors of organs) between the second and seventh decade of life were evaluated from the dens to the fifth body of the lumbar vertebra. Before the fifth decade of life numerous plate-like structures are existing which in form of intermittent wall carriers lead to a stabilization of the vertebral bodies. With growing age a transformation into rod-shaped trabeculae takes place. This change of structure takes place by large perforations within these plates. Additionally, after the fifth decade of life formations of microcallus occur in a size not known up to now. Apparently an additional process of reparation is existing which shows falsely positive results, when using non-invasive techniques. The results demonstrate the occurrence of perforations with growing age and fundamental changes of the structure of the spongiosa.(ABSTRACT TRUNCATED AT 250 WORDS)