Ruptured aneurysms of sinuses of Valsalva

At least one additional cardiac lesion was present in 18 consecutive patients with ruptured aneurysms of the sinuses of Valsalva who were investigated between 1956 and 1973 at the University of Alberta Hospital. Clinical diagnosis was made in 78% (14/18) of the patients. Confirmation at cardiac catheterization, operation or autopsy was obtained in all but one. The main sites of rupture were the right ventricle (seven cases), right atrium (five) and left ventricle (five). Fifty percent (9/18) are alive and well following prompt operative repair, an average of 8.2 years later (range, six months to 15 years). Replacement of the aortic valve was associated with a high mortality (50% early, 13% late, total 63%) which could be explained by the higher operative risk in this group of very ill patients. Eight patients (44.4%) had had bacterial endocarditis prior to presentation and this may have played a significant role in the rupture of the sinus of Valsalva aneurysm.     

Previous cytology in patients with invasive carcinoma of the cervix

The cytologic history of 346 patients presenting with invasive carcinoma of the cervix was reviewed. A total of 74 patients (21%) reported that cervical cytology had been performed in the five years prior to presentation; confirmation of the cytologic history was obtained in 65 cases. Smears were available for review in 34 cases; of 28 smears originally reported as negative, 20 were found to be abnormal on review. The possible reasons for the failure of cytologic detection in these patients are discussed.     

Complications of ileal conduit diversion in adults with cancer followed up for at least five years.

A total of 111 adults with malignant disease of the bladder were studied to determine the long term complications of ileal conduit diversion. Each patient had survived at least five years (mean 10 years) after cystectomy. At final follow up the radiological appearance of one or both kidneys had deteriorated in 50 (47%) of 107 patients: deterioration worsened significantly (p less than 0.01) with increasing duration of follow up. Eighteen patients (16%) developed biochemical evidence of impaired renal function, of whom four died of complications of renal failure. Bilateral upper tract dilatation was noted in 30 patients (28%), and in 21 its cause was obscure. Ten patients formed renal stones, and an additional 12 required further operations on the conduit or stoma. Despite the age of patients with bladder cancer and the poor prognosis of those with invasive tumours clinically important side effects were observed in a significant proportion of the long term survivors. Further efforts to determine the aetiology of upper tract dilatation in patients with an ileal conduit diversion are justified.     

Transition-Related Discussions Among Adolescent Females With Turner Syndrome: Current Practices and Associated Factors

ObjectiveTo examine the current transition practices and factors associated with the occurrence and timing of transition-related discussions among adolescents with Turner syndrome (TS).MethodsA retrospective chart review was conducted at a large pediatric academic center among females with TS seen between 12 and 25 years of age. Medical/developmental characteristics, the age at transition, documented transition-related discussions, and the utilization of transition readiness assessment tools were abstracted. Analyses were conducted to examine the age/occurrence of discussions and associated factors.ResultsRecords of 112 patients were reviewed. The average age of TS diagnosis was 7.6 ± 5.8 years, and the average age of those that transitioned from pediatric to adult care (n = 21) was 20.1 ± 2.0 years. Only 22% of individuals had documented discussions regarding transition to adult care, and no transition readiness tools were utilized. The majority of transition-related discussions began between 11 and 15 years. Estrogen and cardiovascular counseling were common (84% and 75%, respectively). Less than half of the subjects had reproductive (40%), lifestyle (43%), and psychosocial (12%) discussions. Cardiovascular conditions were associated with transition to adult care discussions, and neurodevelopmental conditions were associated with psychosocial counseling. In those that transitioned to adult care, transition-related discussions occurred in only half of patients.ConclusionOur findings emphasize the need for the development and implementation of a standardized transition process for adolescents with TS, with particular attention to transition to adult care and reproductive, lifestyle, and psychosocial counseling. The utilization of formal transition tools may also help prepare these girls for transition to adult care.     

DNA repair in human fibroblasts, as reflected by host-cell reactivation of a transfected UV-irradiated luciferase gene, is not related to donor age

The effect of donor age on the ability of mammalian cells to repair ultraviolet (UV)-induced DNA damage has been studied using several approaches, most recently via assays that measure the host-cell reactivation (HCR) of UV-irradiated reporter gene-containing plasmid vectors following their transfection into cells. Plasmid HCR assays indirectly quantify a cell line's ability to perform nucleotide excision repair (NER) by measuring the enzyme activity of the repaired reporter gene, e.g., chloramphenical acetyltransferase (cat) or luciferase (luc), and are useful in studies investigating whether increasing age may be a risk factor for the deficient repair of potentially cancer-causing, sunlight-induced, DNA lesions in skin cells. In our study, we quantified the DNA repair ability of cultured, nontransformed, human skin fibroblast lines through their HCR of a transfected UV-C-irradiated plasmid containing luc. HCR was measured at various times after transfection in five lines from normal donors of ages 21-96 years, and from one donor who had xeroderma pigmentosum (XP). The normal lines displayed increasing HCR at successive post-transfection time points and showed no significant correlation between HCR and donor age. The XP-A line, known to be markedly deficient in NER of UV-induced DNA damage, showed minimal evidence of HCR compared to the normal lines. To further assess potential variation in HCR with donor age, fibroblast lines from five old donors, ages 84-94 years, were compared with lines from five young donors, ages 17-26 years. While significant differences in HCR were found between some lines, no significant difference was found between the young and old age groups (P = 0.44). Our study provides no indication that the higher incidence of skin cancer observed with increasing age is due to an age-related decrease in the ability to repair UV-induced DNA damage.     

Long survival in acute myelogenous leukaemia: an international collaborative study.

A group of 82 adult patients with acute myelogenous leukaemia had survived in continuous first remission for more than three years was studied. These long-surviving patients were being treated at 12 referral centres in Europe and the USA, and they were compared with other patients with acute myelogenous leukaemia from 10 of these centres. There was no clear difference in the amount of induction chemotherapy or the time taken to achieve remission. Immunotherapy was not found to improve chances of long-term survival. The 82 patients were also compared with a group of 115 patients who had no appreciable difference in the number of blood or marrow myeloblasts between these two groups at presentation, but the long survivors had significantly higher initial platelet counts and were slightly younger. The long survivors also tended to have a lower total white cell count at presentation and lower granulocyte counts; there was no obvious explanation for these differences. Eight of the 82 patients relapsed from three to four years after remission and two (of 69 patients) after four to five year. Thereafter relapse was rare, and it seems likely that some of the 40 patients who have survived for five years or more are cured.     

Aspects cliniques, biologiques et thérapeutiques de la cryptorchidie diagnostiquée à l’âge adulte: analyse d’une série de 69 cas

Objective

To report the clinical, biological and therapeutic features of adult cryptorchidism and to determine whether orchidopexy at adulthood may improve male fertility.

Material and methods

We retrospectively studied the clinical pattern, biological and therapeutic features of 69 men aged of more than 18 years admitted for cryptorchidism at the urological department of Aristide-Le-Dantec teaching hospital between January 1999 and December 2007.

Results

The mean age of our patients was 31.04 ± 8.4 years. In the majority of cases, cryptorchidism was diagnosed in a context of infertility (38 cases), scrotum vacancy (21 cases) and testicular cancer (six cases). Cryptorchidism was unilateral in 69.5% and bilateral in 30.4% of cases. Semen analyses were done for 60 patients and showed azoospermia in 46.6%, oligozoospermia in 38.3% and a normal sperm count in 15% of cases. In all bilateral cryptorchidism cases, semen analyses showed azoospermia. At surgery, the undescended testis was found in 66 cases (95.6%) and orchidopexy was the most done surgical procedure. Seven patients (without testicular cancer) underwent unilateral orchiectomy and histology of specimens showed sertoli-cell-only syndrome with no spermatogenesis in all cases. The histological type of testicular cancer was seminoma (three cases) and embryonic carcinoma (three cases). In azoospermic men (28 cases) no induction of spermatogenesis was achieved after orchidopexy. In infertile patients, the improvement of sperm count was seen in three patients with unilateral cryptorchidism. Three patients with unilateral crytorchidism achieved pregnancy (pregnancy rate of 7.8%).

Conclusion

Orchidopexy for adult cryptorcidism had little impact in male fertility. Because of the risk of testicular cancer, orchidectomy was recommended as treatment of unilateral cryptorchidism. But with the recent development of TESE, orchidopexy appears as a reasonable treatment of adult cryptorchidism.     

Serial determination of cyclic citrullinated peptide autoantibodies predicted five-year radiological outcomes in a prospective cohort of patients with early rheumatoid arthritis

The objective of this study was to evaluate the potential of serially determined anti-cyclic citrullinated peptide (CCP) antibodies for predicting structural joint damage in patients with early rheumatoid arthritis (RA), compared to a single baseline determination. Ninety-nine RA patients with disease durations of less than one year and no history of disease-modifying antirheumatic drug therapy were followed prospectively for at least five years. Anti-CCP2 concentrations were measured using a second-generation ELISA. Sharp scores as modified by van der Heijde were determined on hand and foot radiographs. Anti-CCP2 antibodies were detected in 55.5% of patients at baseline and 63.6% at any time during the first three years. Presence of anti-CCP2 at any time during the first three years was associated with radiographic damage at baseline (odds ratio (OR), 3.66; 95% confidence interval (95% CI) 0.99–13.54) and with five year progression of the total Sharp score (OR, 3.17; 95% CI, 1.3–7.7), erosion score (OR, 5.3; 95% CI, 1.4–19.2) and joint space narrowing score (OR, 2.8; 95% CI, 1.15–6.8). The presence of anti-CCP2 or IgM RF at baseline did not predict these outcomes. Patients with negative anti-CCP2 tests throughout follow-up had less radiographic progression than patients with increasing anti-CCP2 concentrations; they did not differ from patients with decreasing anti-CCP2 antibody levels. HLADRB1* typing showed that progression of the mean modified Sharp score was not correlated with the presence of the shared epitope alleles. In conclusion, serially determined anti-CCP2 antibodies during the first three years of follow-up performs better than baseline determination for predicting radiographic progression in patients with early RA.     

Assessment of post extraction complications in Indians

Extraction is one of the commonest procedures in dentistry. Therefore, it is of interest to evaluate the post extraction complications in patients undergoing extractions of permanent teeth. A total of 70 adult patients who had undergone dental extractions and presented with post -operative complications were included in the study and evaluated. Data collected was statistically analyzed using SPSS software and results obtained. Most of the patients with post extraction complications were in the age group of 31-40 years (21.6%), followed by 21-30 (20.2%) and 61-70 years (20.2%). Dry socket (39.19%) was the common post extraction complication in our study especially in the age group of 31-40 years. There was a statistically significant association between age of the patients and the post extraction complications (p<0.001). In our study, post extraction complications were commonly observed in age group of 31-40 years with a predilection for males. Dry socket was the most common post extraction complication. Age of the patient has a significant effect on post extraction complications. However, gender, smoking habits and systemic diseases have no influence on post extraction complications.     

Comparison between blood hemoglobin concentration determined by point-of-care device and complete blood count in adult patients with dengue

BackgroundHematocrit measurement has been an indispensable tool for monitoring plasma leakage and bleeding in dengue patients. However, hematocrit measurement by automated methods is hampered by frequent venipunctures. Utility of point-of-care hemoglobin (POC-Hb) test for monitoring dengue patients has not been established. We evaluated the relationship between hemoglobin measured by POC-Hb testing and hematocrit measured by the automated method in adult dengue patients.Methodology and principal findingsAdult dengue patients were recruited at two university hospitals in Thailand from October 2019 to December 2020. POC-Hb test was performed using capillary blood simultaneously with venipuncture to obtain whole blood for an automated complete blood count (CBC) analysis. The correlation of hemoglobin and hematocrit measurement was evaluated. A total of 44 dengue patients were enrolled. Twenty-nine patients (65.9%) were female, with a median age of 31 years (interquartile range 22–41). Of the enrolled patients, 30 (68.2%), 11 (25.0%), and 3 (6.8%) were classified as dengue without warning signs, with warning signs, and severe dengue, respectively. Seven patients (15.9%) had hemoconcentration, and five patients (11.3%) had bleeding. A total of 216 pairs of POC-Hb and CBC were evaluated. A significant positive correlation was observed between hemoglobin measured by POC-Hb testing and hematocrit measured by an automated CBC (r = 0.869, p <0.001). Bland-Altman analysis between hemoglobin measured by POC-Hb testing and an automated CBC showed a bias of -0.43 (95% limit of agreement of -1.81 and 0.95). Using the cutoff of POC-Hb ≥20% as a criteria for hemoconcentration, the sensitivity and specificity of hemoconcentration detected by POC-Hb device were 71.4% and 100.0%, respectively.ConclusionsHemoglobin measurement by POC-Hb testing has a strong correlation with hematocrit in adult patients with dengue fever. However, the sensitivity in detecting hemoconcentration is fair. The adjunct use of capillary POC-Hb testing can decrease the frequency of venipuncture. Further study in children is encouraged.     

Emotional problems in children; the uses of drugs in therapeutic management

The repair of intracardiac defects under direct vision by opening the heart to expose the operative field, with the aid of hypothermia or a pump-oxygenator, is now a practical clinical method. Twelve patients were operated upon by this method. In eight patients an atrial septal defect was repaired during total circulatory occlusion under hypothermia. The seven patients in this group who had uncomplicated atrial defects survived the operation and are doing well after a short follow-up period. One patient with an unrecognized, associated ventricular defect died at the time of operation. Four patients were operated upon during total cardiac by-pass with the DeWall bubble-oxygenator. The first three patients survived operation and are continuing to do well after a brief follow-up period. In the fourth patient an atrioventricularis communis was repaired by the reconstruction of an atrial and ventricular septum with a plastic prosthesis. This patient died at the end of operation.     

MR Imaging Evaluation of Intracerebral Hemorrhages and T2 Hyperintense White Matter Lesions Appearing after Radiation Therapy in Adult Patients with Primary Brain Tumors

The purpose of our study was to determine the frequency and severity of intracerebral hemorrhages and T2 hyperintense white matter lesions (WMLs) following radiation therapy for brain tumors in adult patients. Of 648 adult brain tumor patients who received radiation therapy at our institute, magnetic resonance (MR) image data consisting of a gradient echo (GRE) and FLAIR T2-weighted image were available three and five years after radiation therapy in 81 patients. Intracerebral hemorrhage was defined as a hypointense dot lesion appearing on GRE images after radiation therapy. The number and size of the lesions were evaluated. The T2 hyperintense WMLs observed on the FLAIR sequences were graded according to the extent of the lesion. Intracerebral hemorrhage was detected in 21 (25.9%) and 35 (43.2) patients in the three- and five-year follow-up images, respectively. The number of intracerebral hemorrhages per patient tended to increase as the follow-up period increased, whereas the size of the intracerebral hemorrhages exhibited little variation over the course of follow-up. T2 hyperintense WMLs were observed in 27 (33.3%) and 32 (39.5) patients in the three and five year follow-up images, respectively. The age at the time of radiation therapy was significantly higher (p < 0.001) in the patients with T2 hyperintense WMLs than in those without lesions. Intracerebral hemorrhages are not uncommon in adult brain tumor patients undergoing radiation therapy. The incidence and number of intracerebral hemorrhages increased over the course of follow-up. T2 hyperintense WMLs were observed in more than one-third of the study population.     

Characteristics and management of congenital esophageal stenosis: findings from a multicenter study

Background

Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.

Methods

Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.

Results

Over 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p?<?0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p?=?0.27).

Conclusions

CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

     

Surgical Treatment of Mitral Insufficiency

Sixty-four patients with pure mitral insufficiency were operated upon. Thirty of them had torn chordae tendineae. It was possible to repair the mitral valve in 57 patients and there were five operative deaths. One patient had a femoral artery embolus and another had a cerebral embolus. The incidence of peripheral embolization was 4 per cent compared with 40 per cent reported for ball valve replacement.Forty-eight of the 57 patients with repair (84 per cent) were living and well with at most a grade II/VI apical systolic murmur up to seven and a half years after operation. There has been no evidence of recurrence in these patients.In approximately 90 per cent of patients with pure mitral insufficiency, repair should be performed. When feasible, repair is more satisfactory than valvular replacement, with not only excellent long-term results, but far less morbidity than is reported with ball valve replacement.     

Age at Transition from Pediatric to Adult Care Has No Relationship with Mortality for Childhood-Onset Type 1 Diabetes in Japan: Diabetes Epidemiology Research International (DERI) Mortality Study

Objective

To follow up Japanese patients with type 1 diabetes for a maximum of 40 years to examine when they transitioned from pediatric care to adult care and to explore whether the attending physician, i.e., pediatrician or internist, was associated with prognosis.

Methods

Participants consisted of 1,299 patients who had been diagnosed as having type 1 diabetes at less than 15 years old between 1965 and 1979 identified through two nationwide surveys. Patients were classified as having received either pediatric care or adult care at the age of 15 and 30, and were compared for differences in mortality associated with the attending physician.

Results

The attending physicians were confirmed for a total of 1,093 patients at the age of 15. Of these patients, 43.8% and 40.3% received pediatric care and adult care, respectively. Of the 569 patients receiving pediatric care, 74.2%, 56.6%, 53.4%, and 51.3% continued with pediatric care at 20, 30, 40, and 50 years old, respectively. The attending physicians (pediatrician or internist) at the age of 15 and 30 had no significant impact on their survival (P = 0. 892, 0.411, respectively).

Conclusions

More than half of the patients who had received pediatric care at the age of 15 continued to receive pediatric care even after the age of 30, suggesting that their transition was far from smooth, while the attending physician at the age of both 15 and 30 was not a prognostic factor for mortality. Thus, the timing for transition to adult care in these patients has no relationship with mortality in Japan.     

Restoration of genetic diversity in the dry forest tree Swietenia macrophylla (Meliaceae) after pasture abandonment in Costa Rica

We studied the levels of genetic diversity of Swietenia macrophylla (big leaf mahogany) in five successional plots in the Santa Rosa National Park, Guanacaste, Costa Rica. We selected sites with different lengths of time since the last major disturbance (typically fire): 6, 9, 15 and 20 years. In addition, we also included a patch of mature forest that had experienced selective logging and other human activity in the past 100 years. Genetic diversity was assessed using five polymorphic DNA microsatellite loci. We found a total of 21 alleles in the five loci examined, in which the number of alleles present varied among the five sites studied. Allelic diversity varied between sites ranging from 20 to 14 alleles, and our data revealed that earlier successional sites have more alleles than older sites. There was significant heterogeneity in allele frequencies between sites; however, genetic differentiation between populations was low (FST = 0.063) indicating that most of the variation was found within sites and extensive gene flow between sites. In addition, our analysis also showed that genetic diversity of adult trees does not solely determine the diversity of seedlings and saplings found around them, also supporting the existence of extensive gene flow. The impact of these findings for the design of conservation strategies for tropical dry forests trees is discussed.     

Coarctation of the aorta in adults.

Thirty-six patients, 19 men and 17 women, presented at age 18 or older between 1952 and 1974 with coarctation of the aorta. Of the 14 (39%) who had associated cardiovascular disease, 12 had aortic stenosis or insufficiency or both. Three patients had infections-two, endocarditis (aortic valve) and one, endarteritis. Three of the seven patients who did not undergo an operation are alive, two at more than 50 years of age. Five patients had myocardial infarctions, two at 35 years of age. Twenty-nine (80%) had operations; in eight instances the patient was over age 40. All 18 patients undergoing repair of isolated coarctation survived, while only 7 of the 11 patients with associated cardiovascular lesions who underwent repair recovered. Aortic valvular disease and myocardial infarction are serious complicating factors in coarctation of the aorta.     

Transition from paediatric to adult care of adolescent patients with congenital heart disease: a pathway to optimal care

Introduction

Adolescents with congenital heart disease transition from a paediatric to an adult setting. This is associated with loss-to-follow-up and suboptimal care. Increasing numbers of patients justify a special program. In this study we evaluated the cooperative program between paediatric and adult cardiology departments in a tertiary referral centre.

Methods

In this retrospective study, patients with congenital heart disease with at least one appointment scheduled at the transition program between January 2010 and January 2015 were included. They were seen by a paediatric cardiologist at the age of 15 years in the paediatric department and from age 18 to 25 in the adult department. Demographic and medical data were collected from the electronic patient files.

Results

A total of 193 patients (105 males, 88 females) were identified. Sex distribution was almost equal. Most patients were 18–21 years of age. The largest group, 128 patients (67?%), lived within 50 kilometres of our hospital. Paediatric cardiologists referred 157 (81?%) of patients. General practitioners and cardiologists from outside our centre were important referrers for patients lost to follow-up, together accounting for 9?%. A total of 34 (18?%) patients missed an appointment without notification. Repeat offenders, 16 of 34 patients, formed a significant minority within this group. A total of 114 (59?%) patients were attending school, 46 (24?%) were employed, and 33 (17?%) patients were inactive. Activities are in line with capabilities. A nurse practitioner was involved with the 7?% with complex and psychosocial problems. Moderately severe congenital heart defects formed the largest patient category of 102 (53?%) patients. In 3?% of patients the diagnosis had to be revised or was significantly incomplete. In 30 (16?%) patients, cardiac diagnosis was part of a syndrome. Of the 193 patients, 117 (92?%) were in NYHA class I, with 12 (6?%) and 4 (2?%) patients falling into classes II and III, respectively.

Conclusions

A viable transition program can be built by collaboration between paediatric and adult cardiology departments with the same treating physician taking care of patients between 15 and 25 years of age. General practitioners are important in returning lost-to-follow-up patients to specialised care. Nurse practitioners are essential in the care for patients with complex congenital heart disease.

     

Restenosis Following Mitral Valvoplasty

Nineteen seriously disabled patients were operated upon a second time (using closed-heart technique) for recurrence of symptoms of mitral stenosis. The hospital mortality rate was 10.5%; there was one late death. Ten patients (52%) were well and working six months to five years after the second operation. In considering the cause of restenosis it was noted that nine patients had inadequate opening of the mitral valve at first operation. In five cases (26%) rheumatic activity appeared to be solely responsible for restenosis. In a further four cases (21%) it was considered that rheumatic activity was a contributing cause of restenosis. The diagnosis of rheumatic activity and the place of open heart procedures in the treatment of restenosis are discussed.