Malignant pheochromocytoma with widespread bony and pulmonary metastases

Pheochromocytoma is a rare benign tumor of the adrenal gland. A select few cases may be malignant, and metastatic cases are exceedingly rare. It often presents with symptoms of catecholamine excess, such as sweating, palpitations, headaches, and characteristic paroxysmal hypertension. Due to its diffuse symptoms, it is difficult to diagnose and is often diagnosed late. We describe the unique case of a 44-year-old female patient who presented with uncontrolled hypertension and vomiting, accompanied by lower back pain. She was diagnosed with malignant pheochromocytoma with multiple metastases to the lungs, vertebrae, scapulae, and skull. Because of the advanced state of her disease, the patient was started on treatment with the chemotherapeutic combination of cyclophosphamide, vincristine, and dacarbazine. However, she had a complicated hospital course and died because of aspiration pneumonia and sepsis

Minimally invasive technique of placing a dual chamber permanent pacemaker in children

Objective: To share the experience of a minimally invasive technique in the implantation of a dual chamber permanent pacemaker in paediatric population. Methods: The retrospective study was conducted at Aga Khan University Hospital, Karachi, and comprised data of patients aged up to1 6 years who underwent epicardial dual chamber permanent pacemaker insertion via xiphisternal incision between April 2011 and August 2016. Demographic data included age, weight and gender of the patient. Indications for pacemaker insertion, electrocardiography findings, concomitant cardiac procedures and procedural complications were reviewed. Pacemaker thresholds and impedance at the time of implantation and throughout the course of follow-up were extracted from the clinical data. Results: Of the 10 patients, 5(50% were males and 5(50%) were females. The overall mean age was 3.43.8 years range:1 month - 13 years. The mean weight at the time of operation was 11.4 6.8 kg range: 4.3 27 kg. Indications for permanent pacemaker insertion included postoperative advanced or complete atrioventricular block in 7(70% and complete congenital heart block in 3(30% There was no reported morbidity. Conclusion: Dual chamber permanent pacemaker insertion via xiphisternal incision was found to be of benefit to the patients and the surgeons alike

Embodied energy analysis of aluminium-clad windows.

This article provides an overview of energy loads associated with the application of powder coated aluminium profiles on the exterior wooden surface of double glazed windows. The entire powder coating process has been analysed to measure its energy and environmental contribution towards window cladding. Materials involved in aluminium clad windows have been analysed for their energy impacts. The energy embodied in a standard double glazed 1.2 m x 1.2 m aluminium clad window has been calculated, and is quantified as 1459 MJ, 1967 MJ, and 5.96 GJ respectively for argon, krypton and xenon infill gases. The energy consumed in powder coating the window cladding has been estimated to be 27 MJ and the total embodied energy of adding aluminium cladding to the window has been evaluated as 724 MJ

Technical varieties of ross procedure: A case series.

Replacement of aortic valve with the pulmonary autograft is carried out through the Ross procedure due to its potential for growth, durability in pediatric population, and absence of anticoagulation. This case series reports the postoperative outcome of two technical variations of Ross procedure in eight patients who underwent surgery from January 2007 to December 2016. The dominant valvular hemodynamic indication was aortic regurgitation. The techniques employed for Ross procedure included free standing root replacement in six patients and modified root replacement with autograft stabilisation using Dacron interposition graft in two patients. Right ventricular outflow tract (RVOT) conduit reconstruction was achieved by utilisation of Contegra bio prosthesis in four patients; and use of manually constructed valved conduit comprising of PTFE (polytetrafluoroethelene) membrane and bovine pericardial tube in remaining four patients. There was no mortality and no re-intervention. The technical varieties of Ross procedure offer freedom from need of anti-coagulation, mortality, and RVOT conduit failure in younger adults

Contegra valved conduit in the paediatric population: an exciting prospect for right ventricle to pulmonary artery reconstruction; experience and outcomes at Aga Khan University

Objective: The focus of this study is to share the experience and outcomes of Contegra graft implantation in the paediatric and adult population in Pakistan. Methods: Between May 2007 and July 2011, 16 patients, underwent implantation of a Contegra valved conduit. All operations were performed through a median sternotomy with cardiopulmonary bypass. Indications included: Pulmonary atresia with ventricular septal defect (n=11), Tetralogy of Fallot with absent Pulmonary Valve (PV) syndrome (n=2), double outlet right ventricle, transposition of great arteries and pulmonary stenosis (n=1), isolated aortic valve disease (n=1) and a pseudo-aneurysm with infective endocarditis (n=1).Conduit sizes varied between 16-22 mm. Results: The three in hospital deaths were unrelated to the Contegra valved conduit. One patient was lost to follow up. Of the 12 survivors, 10 are currently free from re-operation or complications related to the conduit while one needed distal pulmonary artery dilatation owing to critical stenosis and another had severe Valvular regurgitation. Echocardiographic evaluation of the Contegra valved conduit demonstrated no haemodynamically significant valve regurgitation in 10 patients. Conclusion: In this small review of 16 operations using the Contegra valved conduit for Right Venticular Outflow Tract (RVOT) reconstruction in the paediatric population, we observed good post operative results concerning conduit function. The Contegra conduit provides an excellent substitute to the homograft with satisfactory early and mid-term results though long term results are awaited in Pakista

Anomalous origin of the left coronary artery from the pulmonary artery: A surgical certainty

This study aims to assess the clinical presentation and the outcomes of a surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A retrospective review was carried out on the charts of six patients admitted for ALCAPA surgery at Aga Khan University Hospital, Karachi from March 2017 to May 2018.Dyspnoea, palpitation, poor feeding, fatiguability, pallor and a murmur of mitral regurgitation were the main presenting features. The pre-operative median left ventricular ejection fraction (LVEF) was 64%. Coronary reimplantation was performed in all the patients with a mitral valve repair being done in only one patient. The mea n LV EF was 66 .3%,p ost- ope rativel y. Mitra l regurgitation (MR) improved in patients post-operatively with trace in 2 patients and mild MR in one. Surgical correction by coronary re-implantation yields favourable outcomes in ALCAPA and significantly reduces the morbidity and mortality rates associated with the disease

2016 Laboratory guidelines for postvasectomy semen analysis: Association of Biomedical Andrologists, the British Andrology Society and the British Association of Urological Surgeons

Post-vasectomy semen analysis (PVSA) is the procedure used to establish whether sperm are present in the semen following a vasectomy. PVSA is presently carried out by a wide variety of individuals, ranging from doctors and nurses in general practitioner (GP) surgeries to specialist scientists in andrology laboratories, with highly variable results.Key recommendations are that: (1) PVSA should take place a minimum of 12 weeks after surgery and after a minimum of 20 ejaculations. (2) Laboratories should routinely examine samples within 4 h of production if assessing for the presence of sperm. If non-motile sperm are observed, further samples must be examined within 1 h of production. (3) Assessment of a single sample is acceptable to confirm vasectomy success if all recommendations and laboratory methodology are met and no sperm are observed. Clearance can then be given. (4) The level for special clearance should be &lt;100 000/mL non-motile sperm. Special clearance cannot be provided if any motile sperm are observed and should only be given after assessment of two samples in full accordance with the methods contained within these guidelines. Surgeons are responsible both preoperatively and postoperatively for the counselling of patients and their partners regarding complications and the possibility of late recanalisation after clearance. These 2016 guidelines replace the 2002 British Andrology Society (BAS) laboratory guidelines and should be regarded as definitive for the UK in the provision of a quality PVSA service, accredited to ISO 15189:2012, as overseen by the United Kingdom Accreditation Service (UKAS).</jats:p

Antifibrotic synergy between phosphodiesterase type 5 inhibitors and selective oestrogen receptor modulators in Peyronie’s disease models

Background: Peyronie's disease (PD) is a fibrotic disorder of the penile tunica albuginea, characterised by the formation of a localised fibrous plaque that can lead to deformity and erectile dysfunction. Nonsurgical therapeutic options for PD are limited in efficacy and safety. Myofibroblasts are key cells in the pathogenesis of PD, and inhibition of myofibroblast transformation has been suggested as a therapeutic option. Objective: To identify potential drugs using a novel phenotypic assay and then to test them using in vitro and in vivo models of PD. Design, setting, and participants: We have developed and validated a phenotypic screening assay that measures myofibroblast transformation, by which we tested 21 compounds that were suggested to be efficacious in treating PD. The successful hits from this assay were further tested using in vitro and in vivo models of PD. Results and limitations: The new assay was able to detect transforming growth factor-β1–induced myofibroblast transformation. Using this assay, phosphodiesterase type 5 inhibitors (PDE5i) and selective oestrogen receptor modulators (SERMs) were identified to significantly inhibit myofibroblast transformation. A PDE5i (vardenafil) and an SERM (tamoxifen) inhibited myofibroblast transformation, collagen gel contraction, and extracellular matrix production in a synergistic fashion. In a rat model of PD, the antifibrotic effect of the combination of vardenafil and tamoxifen was greater than that of each drug alone. This study is limited by not providing a molecular mechanism for the proposed synergy. Conclusions: This is the first demonstration of a synergistic activity between a PDE5i and an SERM discovered through a phenotypic screening approach. Future clinical trials using a combination of these drugs should be considered during the active phase of PD, given the early evidence of benefit in both in vitro and in vivo models. Patient summary: This report suggests that the combination of a phosphodiesterase type 5 inhibitor and a selective oestrogen receptor modulator may be efficacious in treating Peyronie's disease in its active phase

Opposite changes in the expression of clathrin and caveolin-1 in normal and cancerous human prostate tissue: putative clathrin-mediated recycling of EGFR

Endocytosis, an important macromolecule uptake process in cells, is known to be dysregulated in cancer. Clathrin and caveolin-1 proteins play a major role in receptor-mediated endocytosis. We have used a quantitative, unbiased and semi-automated method to measure in situ protein expression of clathrin and caveolin-1 in cancerous and paired normal (cancer adjacent, non-cancerous) human prostate tissue. There was a significant (p &lt; 0.0001) increase in the expression of clathrin in prostate cancer samples (N = 29, n = 91) compared to normal tissue (N = 29, n = 67) (N = number of patients, n = number of cores in tissue arrays). Conversely, there was a significant (p &lt; 0.0001) decrease in expression of caveolin-1 in prostate cancer tissue compared to normal prostate tissue. The opposite change in expression of the two proteins was highly correlated to increasing cancer aggressiveness. There was also a concurrent increase in the expression of epidermal growth factor receptor (EGFR), a key receptor in carcinogenesis, with clathrin in prostate cancer tissue, indicating recycling of EGFR through clathrin-mediated endocytosis (CME). These results indicate that in prostate cancer, caveolin-1-mediated endocytosis (CavME) may be acting as a brake and increase in CME may facilitate tumorigenicity and aggressiveness of prostate cancer through recycling of EGFR. Changes in the expression of these proteins can also potentially be used as a biomarker for prostate cancer to aid in diagnosis and prognosis and clinical decision-making