Retroperitoneal Spindle Cell Rhabdomyosarcoma With Compression Features in a 48-Year-Old Male: A Case Report.

Spindle cell rhabdomyosarcoma (SC-RMS) is an unprecedented version of embryonal rhabdomyosarcomas (RMSs) that emerges from the mesenchyme with the capacity to differentiate into skeletal muscle cells. Retroperitoneal RMS is extremely rare in the adult population. We present the case of a primary spindle cell retroperitoneal RMS with compression features. Investigation-based diagnosis of RMS is difficult due to the lack of specificity of clinical findings. Radiology does not help in making an accurate diagnosis. Surgical removal of the tumor followed by chemotherapy and radiotherapy is the best possible treatment for RMS in adults. SC-RMS has a poor long-term prognosis. To our knowledge, such cases of retroperitoneal SC-RMS compressing the abdominal viscera and resulting in hydroureteronephrosis have never been reported before.

A Case of Thrombotic Thrombocytopenic Purpura Possibly Induced by Graves' Disease.

Thrombotic thrombocytopenic purpura (TTP) has historically been diagnosed with a pentad of features, i.e., thrombocytopenia, micro-angiopathic hemolytic anemia (MAHA), fever, neurological abnormalities, and kidney failure. Traditionally, TTP cases have been described in healthy adults. However, their association with autoimmune diseases is now well documented in the literature. There is limited availability of literature on the association between TTP and Graves' disease (GD). Here, we report a case of an adult female, a known case of Graves' disease, who has now been diagnosed with an acquired case of TTP. The presence of MAHA associated with thrombocytopenia was considered a clinical diagnosis of TTP and the patient immediately underwent plasma exchange (PEX), which led to the resolution of complaints. Hyperthyroidism cases should be adequately followed up as clinical severity could lead to the emergence of TTP.

Knowledge and Attitude Towards Cardiopulmonary Resuscitation Among Doctors of a Tertiary Care Hospital in Karachi.

Objective Cardiac arrest is an emergency, which can be managed effectively by sound knowledge and practice of basic life support (BLS) skills. However, it has been globally reported that the knowledge of doctors regarding cardiopulmonary resuscitation (CPR) and BLS is sub-standard. We conducted this study with the aim to assess the knowledge and attitude of doctors toward CPR in Dr. Ruth K.M. Pfau Civil Hospital, one of the largest tertiary care hospitals, in Pakistan. Methods We conducted a cross-sectional study, in Dr. Ruth K.M. Pfau Civil Hospital located in Karachi, Pakistan, using cluster sampling. A total of 285 doctors were interviewed. Results A majority of the doctors were unaware of the revised rate and depth of chest compressions (65.6% and 75.8% respectively). While many know the abbreviations of BLS and CPR (96.55% and 95.4%, respectively), 56.5% did not know what automated external defibrillator (AED) stood for. CPR was preferred over chest compression-only resuscitation (CCR) by 91.6% of the doctors. Half of the participants rated their knowledge as average. Most stated that they will not be reluctant to perform CPR in an emergency situation. The majority also agreed that BLS training should be an integral part of the medical curriculum. Conclusion There is an evident lack of knowledge of CPR among healthcare professionals, particularly regarding the updates made in the 2015 American Heart Association (AHA) guidelines. However, an overall positive attitude was observed.

Top 100 Most Influential Articles in the Field of Myeloid Neoplasms: A Bibliometric Study.

BACKGROUND: Bibliometric analyses are a tool employed by researchers and funding agencies to establish the most important areas of research in a particular field, and to determine which foci need increased research attention. Such analyses have been published in a variety of clinical specialties; however, a detailed literature search showed that no such study has been done for "myeloid neoplasms." In order to bridge this gap, we conducted a citation analysis of the 100 most influential articles on myeloid neoplasms. METHODS: Two independent researchers extracted relevant articles from the Scopus database. These articles were then ranked in descending order of citations and a list of the top 100 original articles was made. A further, more detailed list was created containing significant discriminating characteristics. RESULTS: The top cited articles were published over a period of 47 years, with most of them being published in the 5-year interval of 2001-2005. The citations ranged from 636 to 4,039. The articles originated from 28 different countries. Most of the articles were published in high-impact journals. CONCLUSION: Our analysis sheds light on the quality of work and driving trends, listing the most cited and impactful guideline articles within this field and aiding clinicians.

Xeroderma Pigmentosum - Cockayne Syndrome Complex (XP-CS) - Another case.

We present the case of a 3-year old girl with clinical manifestations typical of XP-CS, an extremely rare combination of Xeroderma Pigmentosum and Cockayne Syndrome. She had a swelling above the upper lip and multiple brown spots on her face, neck, arms and back. She was globally delayed, deaf, dumb and photophobic. MRI brain showed mild cerebral atrophy and bilateral demyelination. De Sanctis Cacchione variant (dSCS) and Rothmund Thomson syndrome (RTS), which were among the differential diagnosis were ruled out upon careful evaluation. Supportive treatment was given and regular checkups were recommended to monitor the progression of the disease but our patient did not show up for the follow up. This report shows that the diagnosis of XP-CS can be based on clinical features and MRI findings when the genetic testing is not available.

Assessment and Improvement of Quality of Life in Patients Undergoing Treatment for Head and Neck Cancer.

INTRODUCTION: We compared the pre and post-treatment quality of life in head and neck cancer (HNC) patients and identified factors that could improve the quality of life in such patients.   Methods: The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire C30 (EORTC QLQ-C30) questionnaire was administered to 84 patients' pre and post-treatment. Patients who had non-metastatic, measurable, and untreated HNCs were invited, provided that their age was below 80 years. We did not discriminate based on treatment modality, stage of cancer, or co-morbidities. Patients who were mentally incapacitated, with secondary or recurrent HNC, distant metastasis, skin cancer, congenital anomaly of the head and neck, chronic illness, or any previous or current psychiatric illness were excluded from the study. A high mean score on the functional scale and a low score on symptom scale signify a better quality of life. We used the dependent t-test to compare pre and post-treatment scores. RESULTS: We found no statistically significant differences in any variables, except the four symptom scales of diarrhoea, constipation, nausea/vomiting, and financial difficulty. All of these variables had increased mean scores with p values of < 0.001. Also, we found no statistical significance (p = 0.250) when comparing the pre-treatment (59.4 ± 18.3) and post-treatment (61.2 ± 16.2) scores for the global health status. CONCLUSION: We found no improvement in the quality of life in HNC patients despite intervention. In fact, diarrhoea, constipation, nausea/vomiting, and financial difficulty of these patients worsened post-treatment.