Search

We report a case of spuriously 'normal' haemoglobin A(1c) (HbA(1c)) result due to misidentification of HbG Taipei as HbA(o) by the Variant II built-in retention time algorithm. The defect was circumvented effectively by the implementation of a chromatographic system specific internal quality control mechanism for peak identity verification. HbA(1c) and estimated average glucose results were corrected from 4.7% to 8.2%, and 4.9 to 10.5 mmol/L, respectively. The results were consistent with the patient's concurrent and previous fasting blood glucose concentrations and existence of diabetes mellitus dermopathy, indicating poor glycaemic control. A review of currently available analytical systems showed that other than mass spectrometry, HbA(1c) measurements by these systems were generally affected by the presence of haemoglobin variants. The same haemoglobin variant may affect different analytical systems differently, resulting in the deviation of HbA(1c) results from the true value to different extents. Including the analytical principle of HbA(1c) measurement in the laboratory report can avoid inappropriate comparison of results obtained by different analytical systems. Moreover, since individual haemoglobinopathy may affect the degree of glucose binding to haemoglobin in a different way, this uncertainty limits the general application of same decision cut-off of established guidelines for glycaemic control monitoring. Adoption of an individualized monitoring system based on the critical difference or reference change value of HbA(1c) can be considered. [ABSTRACT FROM AUTHOR]

We report on an unusual case with asymptomatic bilateral external iliac vein non-thrombotic obstruction causing difficulty in delivery of the leadless permanent pacemaker and discuss on the strategies to overcome the problem., (© 2021 Wiley Periodicals LLC.)

Objectives: The aims of this study were to determine the prevalence of urinary incontinence (UI) in pregnant young women and adolescents, characterize UI subtype, and identify characteristics associated with UI., Methods: This was a cross-sectional study of pregnant females aged 25 years or below, presenting for routine obstetrical care at a New York City community hospital. Subjects were stratified into 2 groups: adolescents (age, ≤19 years) and young adults (age, >19 years). Demographic and obstetric data were collected. The 3 Incontinence Questions questionnaire was used to screen and evaluate UI symptoms., Results: A total of 98 young females with a mean age of 20.3 ± 2.6 years were enrolled. Most participants were nulliparous (64%). Of parous women, route of previous obstetric delivery was primarily vaginal (83%). Mean gestational age at recruitment was 34.5 ± 7.5 weeks. The prevalence of UI was 52%. Urinary incontinence was associated with the following conditions: strenuous activity, 73%; urinary urgency, 67%; and absence of either, 20%. However, the most predominant UI subtype was with strenuous activity (63%). There was no statistical difference detected in demographic characteristics (such as age, parity, mode of delivery, race, education, and trimester of pregnancy) between continent and incontinent pregnant females (P > 0.18). No differences were appreciated between pregnant adolescents and young adult females with UI (P > 0.18)., Conclusions: Urinary incontinence was present in 52% of pregnant females aged 25 years or below. By age group, approximately 50% of both adolescents and young adults reported UI during pregnancy. Continent and incontinent patients did not seem to differ demographically. Our study highlights the extent of UI in this segment of the population. This data may support the need for services targeting UI prevention and early intervention in this newly identified at-risk group.

Background: Early initiation of chronic dialysis (starting dialysis with higher vs lower kidney function) has risen rapidly in the past 2 decades in Canada and internationally, despite absence of established health benefits and higher costs. In 2014, a Canadian guideline on the timing of dialysis initiation, recommending an intent-to-defer approach, was published., Objective: The objective of this study is to evaluate the efficacy and safety of a knowledge translation intervention to promote the intent-to-defer approach in clinical practice., Design: This study is a multicenter, 2-arm parallel, cluster randomized trial., Setting: The study involves 55 advanced chronic kidney disease clinics across Canada., Patients: Patients older than 18 years who are managed by nephrologists for more than 3 months, and initiate dialysis in the follow-up period are included in the study., Measurements: Outcomes will be measured at the patient-level and enumerated within a cluster. Data on characteristics of each dialysis start will be determined by linkages with the Canadian Organ Replacement Register. Primary outcomes include the proportion of patients who start dialysis early with an estimated glomerular filtration rate greater than 10.5 mL/min/1.73 m 2 and start dialysis in hospital as inpatients or in an emergency room setting. Secondary outcomes include the rate of change in early dialysis starts; rates of hospitalizations, deaths, and cost of predialysis care (wherever available); quarterly proportion of new starts; and acceptability of the knowledge translation materials., Methods: We randomized 55 multidisciplinary chronic disease clinics (clusters) in Canada to receive either an active knowledge translation intervention or no intervention for the uptake of the guideline on the timing of dialysis initiation. The active knowledge translation intervention consists of audit and feedback as well as patient- and provider-directed educational tools delivered at a comprehensive in-person medical detailing visit. Control clinics are only exposed to guideline release without active dissemination. We hypothesize that the clinics randomized to the intervention group will have a lower proportion of early dialysis starts., Limitations: Limitations include passive dissemination of the guideline through publication, and lead-time and survivor bias, which favors delayed dialysis initiation., Conclusions: If successful, this active knowledge translation intervention will reduce early dialysis starts, lead to health and economic benefits, and provide a successful framework for evaluating and disseminating future guidelines., Trial Registration: ClinicalTrials.gov, NCT02183987., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Uric acid crystals, the causative agent of gout, have recently gained widespread attention due to their role as a natural endogenous adjuvant. Uric acid crystals, first sensed extracellularly by membrane lipid alterations, are internalized and subsequently activate the NLRP3 inflammasome. Currently, various aspects of this particular novel pathway are poorly defined. This short review will focus on some recent discoveries regarding this simple crystalline structure and address areas requiring further investigation. The fact that uric acid crystals activate innate host defense mechanisms, triggering robust inflammation and immune activation, may lead to engineering potent adjuvants for future vaccines. Furthermore, the elucidation of uric acid's mechanism of inflammation may lay the foundation for other solid inflammatory structures such as silica, asbestos, and alum.

We report a patient with isolated left ventricular non-compaction diagnosed by echocardiography and cardiac magnetic resonance imaging. She developed refractory congestive heart failure and subsequently underwent successful heart transplantation. This type of cardiomyopathy is thought to be caused by the arrest of normal embryogenesis of the endocardium and myocardium. The spectrum of clinical, radiological, and pathological abnormalities is discussed.

The effect of adenosine mediated suppression of immune cell activation has long been an important topic of study. While the protective benefits of such a signaling mechanism are well recognized, there remains a question as to how dendritic cells (DCs) bypass the high levels of adenosine during tissue stress and infection, and become fully activated. We report here that adenosine receptors on resting stage DCs, both in vivo and in vitro, are functionally desensitized after ligand binding. This desensitization lasts several hours during which DCs are "blind" to adenosine and are stimulated without this negative feedback. This effect is mediated by cAMP signaling. Our report suggests that there is a specific regulatory mechanism used by DCs to overcome adenosine mediated inhibition to become fully activated despite a general suppressive state.

Background: To test the hypothesis that chronic sildenafil treatment has similar functional and hemodynamic effects in patients with severe pulmonary arterial hypertension due to Eisenmenger syndrome as those due to idiopathic pulmonary arterial hypertension without intracardiac shunts., Methods: A prospective open-label study was carried out to compare the effects of sildenafil on the pulmonary hemodynamics between two groups of patients with severe pulmonary hypertension and similar baseline functional capacity--Eisenmenger syndrome (ES group) (n=7) versus idiopathic pulmonary arterial hypertension (IPAH group) (n=6)., Results: After 6 months of sildenafil, there was a significant improvement in the functional capacity, the arterial saturation and the pulmonary hemodynamics in the ES group, as shown by significant reduction in the systolic and mean pulmonary artery pressures and the pulmonary vascular resistance., Conclusion: Sildenafil increases pulmonary blood flow and improves cyanosis in patients with Eisenmenger syndrome. Efficacy of sildenafil as treatment for idiopathic pulmonary arterial hypertension may be extended to patients with Eisenmenger syndrome.

Inflammatory or noninfectious aortitis may be idiopathic or it may be part of a systemic autoimmune disease, such as Takayasu's arteritis, Behçet's disease, or giant cell arteritis. At the acute stage, there is thickening of the aortic wall with dilatation of the aorta, more commonly in the thoracic aorta. If it involves the aortic root, there may be annuloaortic ectasia or aortic regurgitation. At a later stage, there may be aneurysmal dilatation of the aorta and rarely dissection or rupture of the aorta. In Takayasu's arteritis, stenosing lesions can occur as well as aneurysmal dilatation of the aorta or arteries. Stenosing lesions may be treated with angioplasty with or without stenting, whereas aneurysmal dilatation of the aorta is treated by aneurys-mectomy with arterial reconstruction or conduit. Severe aortic regurgitation may require aortic valve surgery with or without replacement of the ascending aorta. Irrespective of the interventional procedure undertaken as appropriate for the lesion, control of inflammation with steroid therapy with or without other immunosuppressive agents is of paramount importance. Otherwise, prosthetic valve or graft dehiscence may occur after aortic surgery, and restenosis rate is also higher after percutaneous transluminal angioplasty or stenting.

From 1995 to 2004, 13 patients with severe aortic regurgitation due to non-infectious aortitis underwent aortic valve surgery at our center. Twenty-eight operations (18 aortic valve replacements and 10 Bentall procedures including 1 aortic root replacement with a homograft) were performed due to a high incidence of prosthetic valve or valved conduit dehiscence (54%). Steroid therapy reduced the incidence of re-operation for anastomotic dehiscence within one year from 90% to 18%. Strategies to prevent dehiscence of the prosthetic aortic valve, which include institution of immunosuppression, replacement of the diseased aorta, and monitoring inflammatory indices, are discussed.

Cardiac sarcoidosis is a rare but important differential diagnosis in patients who present with progressive heart failure and arrhythmia. It may be diagnosed on endomyocardial biopsy. An excellent response can be achieved with steroid therapy in the early acute inflammatory stage. Progression of the disease may lead to end-stage heart failure that requires implantation of a permanent pacemaker, implantable cardioverter-defibrillator, or mechanical circulatory support as a bridge to heart transplantation. We present three Hong Kong Chinese patients with cardiac sarcoidosis.

Management of primary pulmonary hypertension is usually difficult because the disease is uncommon and the aetiology of the disease is not well understood. The disease is potentially lethal because it can lead to failure of the right ventricle, low cardiac output, and ensuing multiple organ failure. We report the successful treatment of a case of low-output syndrome due to primary pulmonary hypertension using combined drug therapy and atrial septostomy. Latest developments in the treatment of this disease are also discussed.

A case in which the diagnosis of idiopathic giant cell myocarditis was obscured by the presence of severe coronary artery disease is described. A 47-year-old man presented with recurrent inferior myocardial infarction and complete heart block. Cardiac catheterization confirmed severe 2-vessel disease and left ventricular dysfunction. Incessant ventricular arrhythmia rapidly ensued, which did not respond to anti-arrhythmic therapy and overdrive pacing despite complete surgical revascularization. He eventually died. Autopsy revealed giant cell myocarditis superimposed on coronary artery disease. Acute myocarditis masquerading as myocardial infarction has been well known, but virtually all reported cases had normal coronary arteries. This case illustrated the fact that even in the presence of obvious coronary artery disease the remote possibility of myocarditis should not be entirely disregarded. Although giant cell myocarditis is a rare and frequently fatal disorder, recent studies suggest that combined immunosuppressive therapy may improve the prognosis.

Post-transplantation lymphoproliferative disease (PTLD) is an unique iatrogenic complication after bone marrow transplantation (BMT) and solid organ transplantation (SOTx). The pattern of EBV related lymphoma in Chinese is different from Caucasians. We surveyed the incidence, clinical and pathological spectrum of PTLD among 541 cases of allogeneic BMT, 145 cases of renal transplant, 35 cases of heart/lung transplantation and 146 cases of orthotopic liver transplantation (OLT). From 1994 to 2001, 13 consecutive cases of PTLD were diagnosed, ranging from disseminated NK cell lymphoma to localized plasmacytoma. Both donor and recipient derived PTLD was documented. Disease was often heralded by cytomegaloviral disease and antithymocyte globulin (ATG) usage. Two cases were diagnosed post-mortem, and six patients died of PTLD at a median of 3 months. Complete and partial remission was only achieved in 3 and 2 cases, respectively, despite a range of treatment (reduced immunosuppression, explantation, radiotherapy, combination chemotherapy, donor lymphocytes, autologous marrow infusion and rituximab). Most responding patients died subsequently of rejection, infection and graft versus host disease (GVHD). The incidence of PTLD is not increased in Chinese patients. However, some patients may be at increased risk, especially mismatched allogeneic BMT, parental OLT (especially involving young infants) and heavy ATG exposure.

Bland-White-Garland syndrome refers to the rare congenital cardiac abnormality whereby the left coronary artery arises from the pulmonary artery. The natural history of this condition is highly variable, ranging from death in early infancy to asymptomatic adult survival. It is sometimes diagnosed in adults with mitral regurgitation thought to be of ischaemic origin. We report a case of a 29-year-old man with Bland-White-Garland syndrome and concomitant mitral valve prolapse, and review the literature on the appropriate investigations and management of this abnormality. Recognition and diagnosis of this condition is important because of the potentially life-threatening complications, which may be prevented by surgical intervention.

Lutembacher syndrome refers to the rare combination of congenital atrial septal defect and acquired mitral stenosis. This condition is usually treated by mitral valve operation with concomitant closure of the atrial septal defect. We describe a case of Lutembacher syndrome that was treated successfully with percutaneous transcatheter mitral commissurotomy using the Inoue balloon and closure of the atrial septal defect with the Amplatzer atrial septal defect occluder.

Previous studies have demonstrated the efficacy and safety of outpatient cardiac catheterization on stable patients at low risk. We present our experience with four French catheters in 104 patients (72 male, 32 female, mean age 51 years old) with coronary angiography done as an outpatient procedure. No heparin was given during the procedure. After initial hemostasis had been attained, the patients returned to day center with vital signs monitored regularly. Patients were allowed ambulation after 4 hours' bed rest and were discharged the same afternoon. Normal coronary angiogram study was found in 76% of the study population. Single-vessel disease, double-vessel disease, triple-vessel disease, and left-main disease were found in 12%, 9.6%, 2%, and 1%, respectively. Nearly all of the patients demonstrated normal ventricular contraction (99%). Average procedural time was 20.2 +/- 4.4 minutes. Average hemostatic time was 8.4 +/- 3.1 minutes. No mortality directly attributed to the catheterization occurred in our study population. Moreover, there were no myocardial infarction, acute pulmonary edema, severe allergic reaction, and cerebrovascular accident. Femoral puncture site complication was only limited to superficial skin bruise. Quality of the cineangiogram was good in majority of the patients. Therefore, this study demonstrates that outpatient cardiac catheterization using four French Judkins catheter is a safe and cost-effective procedure.

49 patients with 51 left-sided accessory pathways underwent radiofrequency catheter ablation for symptomatic supraventricular tachycardia via the transeptal route using specialised long vascular sheaths with compound curves. The procedure was successful in 45 patients (92%). The mean fluroscopic time was 22.5 +/- 15.2 mins and the mean procedure time was 1.7 +/- 0.5 hours. Pericardial tamponade occurred in 2 patients (4%) and 2 patients (4%) required switching to the retrograde transaortic route for successful ablation of the pathways. During the period of follow-up of 16.8 +/- 6.9 months, clinical recurrence occurred in 2 patients (4%). In conclusion, the transeptal route of radiofrequency catheter ablation is a useful alternative strategy to the transaortic approach with good long term results. The use of specialised sheaths may help in stabilisation of the catheter during the procedure which can generate more adequate lesions and consequently a lower recurrence rate.

Graft-versus-host disease in solid organ transplantation is very rare, but the prognosis is poor when the condition causes pancytopenia. We report a case of graft-versus-host disease in a heart-lung transplant recipient who at 2 weeks after transplantation had development of features of graft-versus-host disease, including bone marrow aplasia, that could not be attributed to drugs or viral infections. The diagnosis was confirmed by skin biopsy and demonstration of chimerism of peripheral lymphocytes. Augmentation of immunosuppression with intravenous methylprednisolone resulted in improvement in liver function but had no effect on the pancytopenia. Mediastinal irradiation was given with increase in both white blood cell and platelet counts. Unfortunately the patient eventually died of gastrointestinal bleeding and fungemia.

The incidence of pericardial effusion and tamponade postatrial septal defect repair in adult patients are 16 and 1.5%, respectively. Small, medium, and large effusions progressed equally, and echocardiographic study on days 7, 14, and 28 best detects potentially significant effusion.

Primary amyloid light chain (AL) amyloidosis of the heart is a rare cause of congestive heart failure. Approximately 15% of patients with primary AL amyloidosis demonstrate no monoclonal proteins on serum or urine immunoelectrophoresis:(so-called nonsecretory immunoglobulin-derived amyloidosis). The histologic findings of endomyocardial biopsy from these patients may be indistinguishable from those with senile cardiac amyloidosis. However, the AL type may respond favourably to chemotherapy while the latter type does not. The prognosis is also better in the senile cardiac amyloid type. The precise diagnosis in the present case was made by applying immunohistochemical techniques on cardiac tissues.

Background: Pulmonary hypertension remains a risk factor for early postoperative mortality in heart transplantation and may reduce the long-term benefits of the procedure. This study was undertaken to assess the value of baseline hemodynamic studies with nitroprusside used to predict the degree of postoperative reversibility of pulmonary hypertension in cardiac transplant recipients and to identify clinical risk factors for fixed pulmonary hypertension., Methods and Results: Hemodynamic data from 55 consecutive patients who underwent orthotopic cardiac transplantation from June 1988 through September 1993 were analyzed. The effects of nitroprusside and transplantation on pulmonary artery pressure, cardiac output, and pulmonary vascular resistance were compared. Multiple regression analysis was used to identify the predictors of reversibility of pulmonary hypertension. Nitroprusside reduced pulmonary vascular resistance by increasing cardiac output and, to a lesser extent, by reducing the transpulmonary gradient. Pulmonary hypertension was less reversible in patients with ischemic heart disease (versus dilated cardiomyopathy) and in former smokers (versus nonsmokers). Patients with nonischemic heart failure and no smoking history had significantly lower posttransplant pulmonary vascular resistance (1.24 +/- 0.45 Wood units) than ischemic patients (who were all former smokers; 2.20 +/- 1.01 wood units) or nonischemic former smokers (1.72 +/- 0.70 Wood units). The correlation of pulmonary vascular resistance during nitroprusside challenge with posttransplant pulmonary vascular resistance was better than that of baseline pulmonary vascular resistance with posttransplant pulmonary vascular resistance., Conclusions: Nitroprusside testing improves the prediction of late posttransplant pulmonary vascular resistance; hence, it provides data that may be relevant to both early operative risk and later long-term effectiveness of cardiac transplantation. The finding of increased risk of fixed pulmonary hypertension associated with ischemic heart disease and smoking suggests that underlying atherosclerotic vascular disease may contribute to the irreversibility of pulmonary vascular resistance.

We report three young Chinese male patients who underwent aortic valve replacement for severe aortic regurgitation of subacute onset, complicated by recurrent dehiscence of the aortic valve prosthesis, due to aortitis. One of the three patients underwent aortic valve replacement three times and progression of the prosthesis dehiscence was arrested with the use of corticosteroids. A review of the literature on the prognosis and treatment of aortoarteritis with particular reference to the management of recurrent valvular dehiscence is discussed.

Background: Previous Registry studies have reported that heart transplantation with older donor hearts is associated with a more than twofold increase in early mortality., Methods: An analysis of 77 consecutive patients undergoing heart transplantations at our institution between June 1988 and July 1994 was performed to assess the effect of donor age on mortality and morbidity. Recipients were grouped into those receiving hearts from younger donors (aged < 40 years, n = 60) and those receiving hearts from older donors (aged > 40 years, n = 17)., Results: There were two deaths within the first 30 days in the younger donor group and no deaths in the other group. One-year survival rate was 95% and 100% for the "younger" and "older" groups, respectively. The mean recipient age of the younger donor group was lower (46 +/- 14 years) compared with the older donor group (57 +/- 7 years). Morbidity was compared between the two groups; the length of hospital stay (22.6 +/- 15.8 days versus 21.3 +/- 9.4 days), the graft ejection fraction at 1 week (64% +/- 5% versus 62% +/- 7%), and the mean number of rejection episodes within the first 3 months (0.79 versus 0.65) were not statistically different between the two groups. However, the incidence of chronotropic incompetence requiring permanent pacemaker implantation was significantly greater in the group who received older donor hearts (41.2% versus 10.3%, p < 0.05), independent of the ischemic time., Conclusions: This study shows that older donor hearts may be used safely in selected patients with excellent outcome, although there is an increased incidence of chronotropic incompetence requiring implantation of permanent pacemakers.

Objective: The purpose of this study is to correlate the result of the ultrasound guided transperineal aspiration biopsy with the clinical findings., Materials and Methods: We retrospectively studied 27 patients who had been subjected to ultrasound guided transperineal prostatic aspiration biopsy in our institution during the period 1990 to 1992. The patients were divided into two groups: Group A--those with clinically palpable nodules on digital examination, and Group B--those without palpable nodule but with clinical evidence of prostatic enlargement and/or bladder outlet obstruction., Results: There were 8 biopsy proven cancers, 4 of them were palpable lesions while the other 4 were clinically non-palpable. The overall positive predictive value was 29.6%; this increased to 40% if the lesions were palpable but decreased to 23.5% if the lesions were non-palpable., Conclusion: The overall positive predictive value of this technique was found to be consistent with other studies using different methods of biopsies. The positive predictive value was increased if the abnormality was clinically palpable. This technique was also found to be safe and no significant complication was observed in these patients.

Prominent periportal echogenicity was detected during sonographic examination of patients suffering from recurrent pyogenic cholangitis, hepatocellular carcinoma and acute cholecystitis. To document the finding, 140 normal individuals were studied to establish a norm for the evaluation of the periportal echogenicity. The significance of this sonographic finding and its possible aetiology are discussed.

Fifty cases of recurrent pyogenic cholangitis among Chinese in Hong Kong were studied by ultrasound and compared with endoscopic retrograde cholangiopancreatography. The sensitivity of ultrasound detection of both dilated intrahepatic biliary ducts and intrahepatic calculi is 67% as compared with endoscopic retrograde cholangiopancreatography. A negative study, therefore, does not rule out the presence of such pathological features. A dilated common duct is present in virtually every case of recurrent pyogenic cholangitis and ultrasound is very accurate in its detection. Furthermore, ultrasound is able to provide additional information which can be missed with endoscopic retrograde cholangiopancreatography. Prominent periportal echogenicity, concomitant abscesses and tumours are examples in this series. Such findings bear significance on the management of patients with recurrent pyogenic cholangitis.

Lobar attenuation difference of liver on computed tomography was seen in a case of portal pyemia complicating perforated appendicitis. Left portal vein thrombus was detected first by ultrasound and subsequently confirmed by computed tomography. The left lobe of the liver showed greater contrast enhancement during the dynamic computed tomography study. The possible cause of this lobar attenuation difference is discussed.

Pseudarthrosis, a functional false joint, is an important mechanical complication of advanced ankylosing spondylitis. For correct determination of prognosis and therapy, the presence and extent of the abnormality must be assessed accurately. CT findings in 18 patients with 22 pseudarthroses were compared with the results of conventional tomography. The condition was confirmed by surgery in nine patients (50%). CT scans clearly characterized the pseudarthrosis, providing data not provided by conventional tomography in 17 (77%) of the 22 lesions. CT showed irregular diskovertebral osteolysis with reactive sclerosis and more frequently detected the vacuum phenomenon and paraspinal swellings. In all cases, CT showed either a fracture of mobile facet joints in the posterior elements and allowed clear differentiation between the two. In four cases, such differentiation was impossible on conventional tomograms. Associated spinal stenosis was suspected on conventional tomograms in three lesions, but it was shown clearly by CT in 10 lesions. Our results suggest that CT offers considerable advantages over conventional tomography in the investigation of spinal pseudarthrosis complicating ankylosing spondylitis.