Umair Khalid

Hughes-Stovin Syndrome

Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. Less than 40 published cases of HSS have been described in English medical literature so far. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. HSS has also been considered as a variant of Behcets disease (BD). Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. The management of HSS can either be medical or surgical. Medical management includes the use of steroids and cytotoxic agents. Cyclophosphamide, in particular, is a favored therapeutic agent in this regard. Antibiotics have no proven role in HSS while anticoagulants and thombolytic agents are generally contraindicated due to an increased risk of fatal hemorrhage. However, their use may be considered with great care under special circumstances, for instance, intracardiac thrombi or massive pulmonary embolism. For cases of massive hemoptysis due to large pulmonary aneurysms or those with lesions confined to one segment or one lung, lobectomy or pneumectomy can be carried out. However, surgical risks merit serious consideration and must be discussed with the patient. Transcatheter arterial embolization has emerged as a less invasive alternative to surgery in selected cases of HSS. Overall, patients with HSS have a poor prognosis and aneurysmal rupture is the leading cause of death. However, early diagnosis and timely intervention is crucial in improving the prognosis. There is a need to clearly elucidate the genetic, etiologic and pathologic basis for HSS in the future. Although most of the evidence put forward to refute the role of an infectious agent in the etiology and pathogenesis of HSS is based on negative blood and other body fluid cultures, more robust objective assessment is needed through the use of electron microscopy or 16 sRNA studies. The development of better therapeutic agents is also needed to address and prevent the serious consequences arising from pulmonary arterial aneurysms seen in BD and HSS. Also, the issue of anticoagulation in these patients is challenging and requires further deliberation.

Evaluation of compliance and outcomes of a management protocol for massive postpartum hemorrhage at a tertiary care hospital in Pakistan

BackgroundMassive postpartum hemorrhage is a life threatening obstetric emergency. In order to prevent the complications associated with this condition, an organized and step-wise management protocol should be immediately initiated.MethodsAn evidence based management protocol for massive postpartum hemorrhage was implemented at Aga Khan University Hospital, Karachi, Pakistan after an audit in 2005. We sought to evaluate the compliance and outcomes associated with this management protocol 3 years after its implementation. A review of all deliveries with massive primary postpartum hemorrhage (blood loss ≥ 1500 ml) between January, 2008 to December, 2008 was carried out. Information regarding mortality, mode of delivery, possible cause of postpartum hemorrhage and medical or surgical intervention was collected. The estimation of blood loss was made via subjective and objective assessment.ResultsDuring 2008, massive postpartum hemorrhage occurred in 0.64% cases (26/4,052). No deaths were reported. The mean blood loss was 2431 ± 1817 ml (range: 1500 - 9000 ml). Emergency cesarean section was the most common mode of delivery (13/26; 50%) while uterine atony was the most common cause of massive postpartum hemorrhage (14/26; 54%). B-lynch suture (24%) and balloon tamponade (60%) were used more commonly as compared to our previously reported experience. Cesarean hysterectomy was performed in 3 cases (12%) for control of massive postpartum hemorrhage. More than 80% compliance was observed in 8 out of 10 steps of the management protocol. Initiation of blood transfusion at 1500 ml blood loss (89%) and overall documentation of management (92%) were favorably observed in most cases.ConclusionThis report details our experience with the practical implementation of a management protocol for massive postpartum hemorrhage at a tertiary care hospital in a developing country. With the exception of arterial embolization, relatively newer, simpler and potentially safer techniques are now being employed for the management of massive postpartum hemorrhage at our institution. Particular attention should be paid to the documentation of the management steps while ensuring a stricter adherence to the formulated protocols and guidelines in order to further ameliorate patient outcomes in emergency obstetrical practice. More audits like the one we performed are important to recognize and rectify any deficiencies in obstetrical practice in developing countries. Dissemination of the same is pivotal to enable an open discourse on the improvement of existing obstetrical strategies.

Supernumerary, ectopic tooth in the maxillary antrum presenting with recurrent haemoptysis

BackgroundEctopic eruption of teeth in non-dental sites is a rare phenomenon and can present in a variety of ways such as chronic or recurrent sinusitis, sepsis, nasolacrimal duct obstruction, headaches, ostiomeatal complex disease and facial numbness. However, presentation of such patients with recurrent haemoptysis has not been described in the literature so far. We have described a case of an ectopic, supernumerary molar tooth in the maxillary antrum in a patient who initially presented with haemoptysis.Case presentationA 45-year-old male presented with a 2-month history of episodic haemoptysis. A pedunculated growth from the inferior nasal turbinate was seen with fibre-optic visualization. Although the patient was empirically started on antibiotic and anti-allergic therapy, there was no improvement after a few weeks and the patient had recurrent episodes of haemoptysis. Fibre-optic visualization was repeated showing bilateral osteomeatal erythema. Computed tomography scan of the paranasal sinuses demonstrated complete opacification of the left maxillary antrum along with a focal area of density comparable to bone. An ectopic, supernumerary molar tooth was found in the left maxillary antrum on endoscopic examination and subsequently removed. In addition, copious purulent discharge was seen. Post-operatively, the patient was treated with a 10-day course of oral amoxicillin-clavulanate. On follow-up, he reported resolution of symptoms.ConclusionRecurrent haemoptysis has not been described as a presentation for a supernumerary, ectopic tooth in literature before. We recommend that in patients with sinusitis-type of opacification of maxillary antrum and whose condition is refractory to conventional medical treatment, consideration should be given to the investigation of possible underlying anomalies as the cause of such symptoms. Presence of foreign bodies and ectopic teeth in paranasal sinuses can be reliably excluded with the use of appropriate radiological imaging and endoscopic examination.

Geriatric patients' expectations of their physicians: findings from a tertiary care hospital in Pakistan

BackgroundGeriatric health is a neglected and under-explored area internationally and in Pakistan. We aimed to ascertain the expectations of the geriatric patients from their physicians and the factors associated with patient satisfaction in this particular age bracket.MethodsA cross-sectional survey was carried out at a tertiary care teaching hospital in Karachi, Pakistan. Data collection was carried out via face-to-face interviews based on structured, pre-tested questionnaires. All consenting individuals aged 65 years or above were recruited into the study. Convenience sampling was used to draw the sample. The data was analyzed using SPSS version 16. Geriatric patients expectations from physicians were elicited using a set of 11 questions that were graded on a scale of 1-3 where 1 = not important, 2 = important, 3 = very important.ResultsThree hundred and eighty geriatric patients were interviewed. The response rate of this study was 89.8%. The mean age of the respondents was 73.4 ± 6.8 years. Two hundred and forty eight respondents (65.3%) were female. Diabetes mellitus (53.7%), hypertension (59.5%), arthritis (40.5%) and renal disease (32.1%) were common ailments among geriatric patients. More than 50% of the patients were visiting their physicians once every two to three months. Discussing treatment options and letting patients make the final decision (79.2%), prescribing minimum possible medications (84.5%), physicians holistic knowledge about the spectrum of care issues for geriatric patients (79.2%), being given a realistic but optimistic picture of future health by physicians (85.5%) were ranked as very important expectations by patients from their physicians. Cumulative household income (p = 0.005), most important health complaint (p = 0.01) and frequency of experiencing health complaint (p < 0.001) emerged as independent predictors of the satisfaction of geriatric patients from care provided by physicians.ConclusionWe have documented the expectations of the geriatric patients from their physicians in a developing country. Physicians belonging to all disciplines should keep these expectations in mind during clinical encounters with geriatric patients.

Institutional review boards - a mixed blessing.

Institutional Review Boards (IRBs) are an important checkpoint for all types of research in medicine. Although these bodies originated primarily in the developed world, they have special contemporary consideration in the context of developing countries due to the large number of clinical trials being conducted in these regions with the financial support of large pharmaceutical companies. IRBs are vital to ensure that all scientific investigation is conducted in a manner that is transparent, scientifically feasible and ethically sound. However, they have also been variably criticized for introducing unnecessary and often protracted bureaucracy and red tape into the system. There is a need to reorganize and better delineate the exact functions of the IRBs in view of the dynamic changes in the realm of research so that they can function in a more efficient, judicious and effective fashion.

Paradigm shift: 'ABC' to 'CAB' for cardiac arrests

CPR has a proven role in improving survival in cardiac arrest victims, especially those who are outside the hospital. Guidelines published by the AHA have included CPR as a vital intervention for decades. The previous guidelines have focused on the maintenance of airway as the first step, there by delaying the provision of chest compressions. However, the 2010 AHA Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care corrects this by changing the A-B-C of CPR to C-A-B, acknowledging that chest compressions are the most important aspect of the cardiac arrest management.

Takotsubo cardiomyopathy vs acute myocardial infarction: diagnostic utility of subtle ECG differences.

The clinical findings of Takatsubo Cardiomyopathy and acute myocardial infarction can be very similar. While Takatsubo cardiomyopathy rarely leads to severe complications, acute myocardial infarction can be life threatening. Treatment of both these conditions is different and so it is imperative for clinicians to have a high index of suspicion for either. Several EKG differences between the two entities have been proposed. This article summarizes the EKG changes most likely seen in Takatsubo cardiomyopathy and compares them to those seen in Acute Myocardial infarction.

Large thymic carcinoma presenting with right ventricular failure: a case report.

Abstract Thymic carcinoma is an overall rare tumour with variable clinical manifestations. Right ventricular failure remains an uncommon occurrence and has not been reported in literature so far. A 40-year-old lady presented with the complaints of progressively worsening retrosternal chest pain, shortness of breath, easy fatigability and cough since 1 year. Computed tomography scan of the thorax revealed a mass measuring 12 × 10 cm in the anterior mediastinum. This mass appeared to be adherent to both lungs and pericardium and was impinging on the right atrium and right ventricle. It appeared to be infiltrating the ascending aorta, pulmonary arteries and superior vena cava. Ultrasound of the abdomen showed hepatomegaly and moderate ascites. Echocardiography showed evidence of right ventricular dysfunction as well as elevated right ventricular systolic pressures secondary to extrinsic compression. Percutaneous biopsy of the thymus was performed showing a malignant thymoma. Radical thymectomy with resection of pericardium was planned. Intra-operatively, the tumour was separated from the right and left lungs, pulmonary artery and aortic arch. Morphologically, immunochemically and clinically, the features were consistent with those seen in Masoka stage III thymic carcinoma. She also received six cycles of chemotherapy (PAC regimen) including cisplatin (50 mg/ m2), doxorubicin (50 mg/m2) and cyclophosphamide (500 mg/m2). Radiation therapy in the adjuvant setting was planned but the patient was lost to follow-up after 4 months. Although right ventricular failure is a very rare presentation of thymic carcinoma, clinicians should be aware of this presentation to appreciate the complete clinical spectrum of presentation of this neoplasm.

Pure red cell aplasia regression after thymus resection

Dear Sir, Thymomas are common tumors of the anterior mediastinum accounting for about 20% of all mediastinal tumors. Overall, however, it is a rare neoplasm with an incidence of 0.15 cases per 100,000 [1]. Three paraneoplastic disorders have been reported in association with thymomas including myasthenia gravis (MG), pure red cell aplasia (PRCA), and hypogammaglobulinemia [2]. MG, present in around 30% of thymomas, occurs secondary to autoantibodies to the acetylcholine receptors. Thymectomy has been shown to result in improvement in symptoms in MG [2]. However, there is little consensus with regards to the role of thymectomy for patients with thymoma-associated PRCA. We encountered a 35-year-old woman at our institution who presented with complaints of palpitations and generalized myalgias since 1 month. Her past medical history was significant for severe anemia requiring transfusions on multiple occasions; the cause of the anemia had not been investigated. These transfusions had failed to keep her hemoglobin level within the normal range. Laboratory investigations undertaken at the time of presentation to our institution showed severe anemia (hemoglobin: 4 gm/dl) along with reticulocytopenia, normal granulopoiesis and megakaryopoiesis. Evaluation for other possible causes of PRCA did not reveal any remarkable finding. Chest roentogram, performed as part of workup, showed a well-defined mass in the anterior mediastinum. A computed tomography scan was performed next, which showed a 5.4×4.6 cm soft tissue mass in the anterior mediastinum (Fig. 1). Biopsy of this mass revealed a thymoma. The thymoma was resected via a median sternotomy approach (Fig. 2). Final pathology confirmed the diagnosis of World Health Organization type ‘AB’ thymoma with a paraneoplastic syndrome in the form of PRCA. The patient’s hemoglobin level dramatically returned to normal age-appropriate level following the excision of the thymoma. At 3 years follow-up period, the patient has remained free of any symptoms without any evidence of recurrence of disease. The time interval from the diagnosis of PRCA to the diagnosis of thymoma in this patient was approximately 8 months. This considerable delay in diagnosis occurred because the patient belonged to a remote area and was not able to visit our institution regularly for further investigations. Once the thymoma was diagnosed, the patient was operated within 2 weeks. Being a relatively rare paraneoplastic syndrome, information on themanagement, clinical picture and outcomes of thymomaassociated PRCA is quite limited. Our patient showed a complete remission from PRCA following thymectomy. In a 50-year single institution experience of 13 patients at Mayo Clinic with thymoma-associated PRCA, surgical resection of the thymoma was insufficient for normalization of erythropoiesis in all cases [3]. In another series of five patients over a period of 10 years, none of the patients achieved complete remission after thymectomy [4]; steroids and cyclosporine were administered to these patients for U. Khalid : T. Saleem (*) Medical College, Aga Khan University, Stadium Road, Karachi 74800, Pakistan e-mail: taimur@gmail.com