Normalisation of total body iron load with very intensive combined chelation reverses cardiac and endocrine complications of thalassaemia major

Cardiac and endocrine disorders are common sequelae of iron overload in transfused thalassaemia patients. Combined chelation with desferrioxamine (DFO) and deferiprone (DFP) is well tolerated and produces an additive/synergistic effect superior to either drug alone. 52 thalassaemia major patients were transitioned from DFO to combined chelation with DFO and DFP. Serum ferritin, cardiac and hepatic iron levels were monitored regularly for up to 7 years, as were cardiac and endocrine function. Patients’ iron load normalized, as judged by ferritin and cardiac and hepatic magnetic resonance imaging findings. In all 12 patients receiving treatment for cardiac dysfunction, symptoms reversed following combined chelation, enabling nine patients to discontinue heart medications. In the 39 patients with abnormal glucose metabolism, 44% normalized. In 18 requiring thyroxine supplementation for hypothyroidism, 10 were able to discontinue, and four reduced their thyroxine dose. In 14 hypogonadal males on testosterone therapy, seven stopped treatment. Of the 19 females, who were hypogonadal on DFO monotherapy, six were able to conceive. Moreover, no patients developed de novo cardiac or endocrine complications. These results suggest that intensive combined chelation normalized patients’ iron load and thereby prevented and reversed cardiac and multiple endocrine complications associated with transfusion iron overload.     

Transcatheter closure of perimembranous ventricular septal defects in infants and children using the Amplatzer perimembranous ventricular septal defect occluder

There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.     

Cavernous sinus chondroma. Case report and review of the literature

Chondromas of the base of the skull are most commonly found in the parasellar and sellar regions, and present varying degrees of involvment of the cavernous sinus.However, those confined mainly to the cavernous sinus are rare, and only a few cases have been reported. A 50 year old man experienced left hemifacial pain followed by left abducens nerve palsy. Computerized tomography and magnetic resonance image depicted a well circumscribed mass in the left cavernous sinus. A pterional craniotomy was performed to approach this lesion intradurally. The tumour was subtotally removed Histologically the mass was diagnosed as a mature chondroma. Postoperatively, the left hemifacial pain disappeared and the diplopia improved from the first postoperative day. Successfull removal of lesions in the cavernous sinus requires individualisation of the case as well as choosing the correct surgical approach for the certain patient.     

Rare combination of unilateral renal agenesis, congenital obstructive posterior urethral membrane, and enlarged prostatic utricle, with absence of hydroureteronephrosis

The existence of congenital obstructive posterior urethral membrane (COPUM), extending proximally from the verumontanum toward the bladder neck, has been widely debated. Although COPUM often leads to chronic renal disease, a milder degree of obstruction and protective pressure pop-off mechanisms have been reported. We present a male infant with a rare combination of unilateral renal agenesis, COPUM proximally from the verumontanum to the bladder neck, and enlarged prostatic utricle. The posterior urethra was dilated, without bladder abnormalities or hydroureteronephrosis, indicating milder obstruction. Moreover, the enlarged prostatic utricle could contribute in the urinary tract protection, serving as an uncommon pressure pop-off mechanism.     

Evaluation of sexual function in hypertensive men receiving treatment: a review of current guidelines recommendation

IntroductionIt has been suggested that some classes of antihypertensive drugs may induce or exacerbate sexual and/or erectile dysfunction (ED) more than others. Sexually related side effects of antihypertensive treatment may compromise patient's and partner's quality of life. Often, these side effects can lead to withdrawal or poor compliance with therapy resulting in abnormal blood pressure and associated morbidity.AimThe aim of this study was to evaluate whether hypertension clinical practice guidelines (CPGs) address ED and/or other sexual issues as either an adverse outcome of chosen therapy or as a factor to consider in treatment decision.MethodsHypertension CPGs were identified by searching PubMed (from 2000 to current), the World Wide Web, bibliographies of retrieved guidelines, and official home pages of major medical societies.Main Outcome MeasuresThe main outcome measures used for this study were guidelines assessment using a set of author‐determined survey questions.ResultsTwelve CPGs were identified and analyzed. From these 12, only three emphasized the importance of assessing sexual function prior to initiation and/or follow‐up of antihypertensive therapy; only five described potential sexual side effects associated with some drugs; only two provided specific management recommendations on commencing antihypertensive therapy in sexually active men or those with preexisting ED and address the timeline of the potential drug‐induced impairment of sexual function.ConclusionsOnly a minority of CPGs for the treatment of hypertension consider ED or other sexual issues as either an adverse outcome or as a factor to consider in treatment. Sexual function is an important aspect of quality of life for both the individual and his partner. It is therefore imperative to select therapy with the least possible potential for causing sexual sequelae and enable the best achievable balance between therapeutic efficacy, quality of life, and therapeutic compliance. Based on these results, our proposed algorithm attempts to effectively apply available evidence to clinical practice. Karavitakis M, Komninos C, Theodorakis PN, Politis V, Lefakis G, Mitsios K, Koritsiadis S, and Doumanis G. Evaluation of sexual function in hypertensive men receiving treatment: A review of current guidelines recommendation. J Sex Med 2011;8:2405–2414.     

The natural history of Peyronie's disease: an ultrasonography-based study

OBJECTIVES: To define ultrasonographic patterns reflecting different states of Peyronie's disease (PD) and to use them to evaluate the natural history of the disease. MATERIAL AND METHODS: Diagnosis of PD was based on medical and sexual history, physical examination, intracavernosal injection test, and penile ultrasonography. Patients with penile fracture history were excluded from the study. Three groups were formed according to ultrasonographic patterns: solitary hyperechoic lesion without acoustic shadow (group A), moderately hyperechoic multiple scattered calcified lesions with acoustic shadows (group B), dense calcified hyperechoic plaque with acoustic shadow (group C). All patients entered a watchful waiting protocol for 1 yr followed by a new penile ultrasonography. RESULTS: Ninety-five 95 patients with PD were included in the study (mean age, 57.2+/-9.1 yr; mean duration of disease, 12.9+/-8.9 mo). Risk factors associated with cardiovascular disease were present in 79 of 95 patients (83.16%). Eleven (11.6%), 35 (36.8%), and 49 (51.6%) patients were classified into groups A, B and C, respectively. At the end of the study, in group A, reduction of fibrotic lesions and curvature angle was noticed in 9 of 11 (81.8%) patients, whereas plaque formation was noticed in 2 of 11 (18.2%) patients. In group B, plaque and curvature reduction was noticed in 15 of 35 (42.9%) and 12 of 35 (34.3%) respectively, whereas in the rest a dense calcified plaque was noticed. In group C, no ultrasonographic evidence of improvement was noticed; curvature angle was reduced in 4 of 49 (8.2%), owing to the extension of the plaque circumferentially. Significant hemodynamic changes were noticed at the two time points tested (30.53% diagnosed with vascular disease at baseline vs. 46.32% at the end of the study, p=0.03). CONCLUSIONS: Corporal ultrasonography in patients with PD allows objective evaluation and classification of disease. The density of echogenic areas and presence of acoustic shadows are predictors of disease stability.     

Reversal of deep vein reflux after successful stenting in a patient with venous hypertension after thigh access graft creation

Presented here is a case of reversal of deep vein reflux after successful stenting in a patient with venous hypertension and valve incompetence after thigh angioaccess creation. The patient with exhausted upper-extremity access sites underwent a loop graft in the upper thigh. Six months later, the patient developed leg edema and significant femoral vein reflux on duplex ultrasound. Fistulography revealed an iliac vein stenosis, which was treated successfully with stenting. The edema and reflux on duplex promptly resolved. In similar cases, reflux may be a consequence of functional valve incompetence and can be reverted by timely treating the underlying stenosis.     

Characteristics and lipid distribution of a large, high-risk, hypertensive population: the lipid-lowering component of the Antihypertensive and Lipid-Lowering Treatment to Prevent Heart Attack Trial (ALLHAT)

The Antihypertensive and Lipid-Lowering Treatment to Prevent Heart Attack Trial (ALLHAT) consisted of 42,418 participants randomized to one of four antihypertensive treatment groups: chlorthalidone, amlodipine, lisinopril, or doxazosin. A subset of these participants with fasting low-density lipoprotein cholesterol levels 100–189 mg/dL were randomized into a lipid-lowering component: 5170 to receive pravastatin (40 mg daily) and 5185 to receive usual care. This report describes the characteristics and lipid distribution of these participants. There were no important differences between the randomized treatment groups. Women had higher total cholesterol, low-density lipoprotein cholesterol, and high-density lipoprotein cholesterol than men. There was a similar finding for black participants compared with whites, except blacks had lower triglycerides. Diabetics had lower high-density lipoprotein cholesterol and higher triglycerides than nondiabetics, and patients with body mass index <25 kg/m² had higher high-density lipoprotein cholesterol but lower low-density lipoprotein cholesterol and triglycerides than patients with higher body mass index. The success of the randomization of this large, diverse population and the differences in the lipid distributions among its subgroups will allow further understanding of optimal lipid-lowering treatment.