Effect of physical activity and vitamin D compared with vitamin D alone on muscle strength, back flexibility and aerobic activity in patients with chronic kidney disease: A comparative study from Pakistan.

BACKGROUND AND OBJECTIVES: To compare the differences in musculoskeletal health with vitamin D alone in comparison with vitamin D with physical activity (PA) among chronic kidney disease (CKD) patients. METHODS AND STUDY DESIGN: An open labeled, randomized, controlled trial was conducted at two tertiary care centers in Pakistan. Patients with CKD stage 2-4 and vitamin D deficiency (<20 ng/mL) were recruited in the trial. Both the arms were given oral vitamin D (cholecalciferol) drops (4000 IU) once daily for three months. One arm received only vitamin D (VD arm), while the second arm received vitamin D along with PA (VDPA arm). RESULTS: Of the 1,235 CKD stage 2-4 subjects contacted, forty-six subjects were enrolled. Eighteen were assigned to VD arm and twenty-eight were assigned to VDPA arm. Between groups comparison shows that bicep strength increases from 15 to 17 kg. Likewise, back flexibility and aerobic fitness also increased among those who receive vitamin D and physical activity, however these differences were not statistically significant (p>0.05). Sensitivity analysis within group comparison shows rise of bicep strength from 13.8 kg to 15.2 kg in the VD alone arm (p=0.05); however, in the VDPA arm, there is a greater difference of 14.3 kg to 17.2 kg (p<0.001). CONCLUSIONS: Targeted PA among CKD patients has potential to improve bicep strength and back flexibility. However, as the sample size was small, further studies would be required to suggest whether a PA should be included as part of the treatment regimen.

Uterine Transplant: A Risk to Life or a Chance for Life?

Being inherently different from any other lifesaving organ transplant, uterine transplantation does not aim at saving lives but supporting the possibility to generate life. Unlike the kidneys or the liver, the uterus is not specifically a vital organ. Given the non-lifesaving nature of this procedure, questions have been raised about its feasibility. The ethical dilemma revolves around whether it is worth placing two lives at risk related to surgery and immunosuppression, amongst others, to enable a woman with absolute uterine factor infertility to experience the presence of an organ enabling childbirth. In the year 2000, the first uterine transplantation, albeit unsuccessful, was performed in Saudi Arabia from where it has spread to the rest of the world including Sweden, the United States and now recently India. The procedure is, however, still in the preclinical stages and several ethical, legal, social and religious concerns are yet to be addressed before it can be integrated into the clinical setting as standard of care for women with absolute uterine factor infertility.

Breaking your heart-A review on CPR-related injuries.

Cardiopulmonary resuscitation (CPR) has been shown to increase survival after cardiac arrest, but is associated with the risk of acquired injuries to the patient. While traumatic chest wall injuries are most common, other injuries include upper airway, pulmonary and intra-abdominal injuries. This review discusses the risk factors and prevalence of CPR-related injuries.

Sanitary Worker's Death Unnerves Pakistan's Health Care Ethics to the Core.

Health care ethics is a sensitive domain, which if ignored, can lead to patient dissatisfaction, weakened doctor-patient interaction and episodes of violence. Little importance has been paid to medical ethics within undergraduate medical education in developing countries such as Pakistan. Three doctors in Pakistan are currently facing an official police complaint and arrest charges, following the death of a sanitary worker, who fell unconscious while cleaning a drain and was allegedly refused treatment as he was covered in sewage filth. The medical license of the doctors in question should be cancelled, if found guilty following a thorough investigation into the case. The 'right to life' has been universally assured by all moral, cultural and legal codes and no society can ever argue against the sacredness of a human life. It is quite clear that the aforesaid doctors' actions are not only against the core principles of the physicians' code, but also go against the doctrine of human rights. If serious efforts on an urgent basis are not made by the regulatory and governing bodies, one can definitely expect similar incidents for at least a few more decades before any noticeable change is seen.

Russell's Viper Envenomation-Associated Addisonian Crisis.

Snakebite envenomation is an important public health problem in tropical countries. We report a case of bilateral adrenal hemorrhage in a 28-y-old man with Russell's viper bite that occurred in the Sathyamangalam forest range in the Indian state of Tamil Nadu. In this case, a combination of early bite recognition, hospital-based supportive care, corticosteroid therapy, and timely administration of polyvalent antivenom resulted in a favorable clinical outcome.

Consented Autopsy and the Middle-East.

Consented autopsy is almost non-existent in the Middle-East where established social and cultural beliefs regarding the procedure might discourage family members from requesting a consented autopsy. Evidence suggests that new information is obtained from consented autopsies. It would not be in the best interest of medicine if social and cultural misconceptions succeed in erasing the existence of consented autopsies entirely.

Personalized Medicine in a New Genomic Era: Ethical and Legal Aspects.

The genome of two completely unrelated individuals is quite similar apart from minor variations called single nucleotide polymorphisms which contribute to the uniqueness of each and every person. These single nucleotide polymorphisms are of great interest clinically as they are useful in figuring out the susceptibility of certain individuals to particular diseases and for recognizing varied responses to pharmacological interventions. This gives rise to the idea of 'personalized medicine' as an exciting new therapeutic science in this genomic era. Personalized medicine suggests a unique treatment strategy based on an individual's genetic make-up. Its key principles revolve around applied pharmaco-genomics, pharmaco-kinetics and pharmaco-proteomics. Herein, the ethical and legal aspects of personalized medicine in a new genomic era are briefly addressed. The ultimate goal is to comprehensively recognize all relevant forms of genetic variation in each individual and be able to interpret this information in a clinically meaningful manner within the ambit of ethical and legal considerations. The authors of this article firmly believe that personalized medicine has the potential to revolutionize the current landscape of medicine as it makes its way into clinical practice.

Prolonged Disease Control Despite ALK Inhibitor Discontinuation in Advanced ALK-Positive NSCLC.

Introduction: EML4-ALK is an oncogenic driver, seen in around five per cent of advanced non-small-cell lung cancer (NSCLC) patients, which can be targeted with anaplastic lymphoma kinase tyrosine kinase inhibitors with great response rates. Disease flare refers to sudden rapid disease worsening on tyrosine kinase inhibitors (TKI) discontinuation, which is associated with shorter survival and worse outcomes. Here, we review cases previously published in the literature where patients developed disease flares, and contrast this with our patients who had prolonged survival despite TKI discontinuation. Case description: We report three different patients with advanced ALK-positive NSCLC seen at our institute, who had EML4-ALK translocation variant 1 oncogenic driver on next-generation sequencing. They received treatment with several different ALK inhibitors before opting to discontinue TKI. They were able to come off TKI safely without developing disease flare and had prolonged survival. Discussion: Shorter time to progression on TKI, presence of symptoms with disease progression or central nervous system/pleural metastasis have been previously linked with development of flare, although this was not seen in our case series. Tumour response at the time of treatment discontinuation, line of therapy, overall disease burden, fusion variant and co-alteration status can affect the prognosis of these patients after ALK TKI cessation. In particular, variant 1 and wild-type TP53 status may be a suitable patient population for dose optimisation strategies. Intermittent TKI dosing strategies may help to avoid acquiring resistance mutations and prevent long-term treatment toxicities. Conclusion: It is important for clinicians to identify patients at risk for developing disease flare on TKI discontinuation to improve outcomes. Intermittent TKI dosing strategies require further investigation. LEARNING POINTS: Patients who develop disease flare after cessation have poor survival and worse outcomes.Certain phenotypic and molecular characteristics of the tumour may help clinicians identify which patients are likely and which are unlikely to develop disease flare on TKI discontinuation.Advanced ALK-positive NSCLC with variant 1 and wild-type TP53 may be a suitable patient population for intermittent TKI dosing investigations.

Acetaminophen induced Steven Johnson syndrome-toxic epidermal necrolysis overlap.

Steven Johnson Syndrome and Toxic Epidermal Necrolysis are rare but severe form of hypersensitivity inflammatory reactions to multiple offending agents including drugs. Acetaminophen is extensively used due to its analgesic and anti-pyretic properties. It is rendered to be relatively safe, with hepatotoxicity considered to be the major adverse effect. However, very few cases of Steven Johnson Syndrome and Toxic Epidermal Necrolysis have been reported with acetaminophen usage in the past. We present the case of a 40 years old lady who developed an overlap of the two condition after taking several doses of acetaminophen for fever. She presented with widespread maculopapular rash, stinging in the eyes, oral mucosal ulcerations and high grade fever. She was successfully treated with corticosteroid therapy along with the supportive treatment. This case addresses the fact, that severe hypersensitivity reactions can occur with acetaminophen which can be potentially life threatening.

Angioimmunoblastic T-cell lymphoma with exuberant plasmacytosis and spontaneous tumor lysis syndrome.

We present a 67-year-old man with complaints of confusion, chills, night sweats, and several days of poor oral intake. He had severe plasmacytosis on lab work, which initially raised concern for plasma cell leukemia. However, further workup led to the diagnosis of angioimmunoblastic T-cell lymphoma. His initial hospital course was complicated by spontaneous tumor lysis syndrome. Early recognition and prompt interventions are pivotal to improve survival outcomes in such patients.

Acquired Amegakaryocytic Thrombocytopenia Progressing to Aplastic Anaemia.

Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare disorder of the bone marrow characterized by a lack of megakaryocytes and preservation of other cell lines. It can occur due to an intrinsic stem cell defect or secondary to viral infections, autoimmune disorders, lymphoproliferative disorders or environmental toxins. With time, it can progress to aplastic anaemia (AA) and can have a poor prognosis. No standard guidelines exist for the treatment of AAMT progressing to AA. Herein, we report a rare case of AAMT leading to AA and review the handful of cases previously published in the literature. LEARNING POINTS: Acquired amegakaryocytic thrombocytopenia can present as isolated severe thrombocytopenia which can initially be misdiagnosed as immune thrombocytopenia.Lack of response to steroids and intravenous immunoglobulin should raise suspicion for acquired amegakaryocytic thrombocytopenia.Over time, acquired amegakaryocytic thrombocytopenia can progress to aplastic anaemia, which confers a worse prognosis.

Extramedullary chronic lymphocytic leukemia disguised as urinary tract infection.

Extramedullary and nonlymphoid organ involvement in chronic lymphocytic leukemia (CLL) is exceedingly rare, with only a few cases of bladder infiltration reported to date. Here, we present a case of a 71-year-old man with advanced-stage CLL who presented with gross hematuria initially thought to be secondary to a urinary tract infection. However, a cystoscopy with biopsy confirmed the diagnosis of extramedullary CLL with bladder involvement. The patient was started on venetoclax with improvement in symptoms and no recurrence of hematuria.

The Impact of Atrial Fibrillation on hospitalization Outcomes for Patients With Chronic Lymphocytic Leukemia Using the National Inpatient Sample Database.

BACKGROUND: The incidence of atrial fibrillation (AF) in patients with chronic lymphocytic leukemia (CLL) has been on the rise. However, the excess burden added by AF to the morbidity and mortality of CLL patients especially in the hospitalized setting is undetermined. METHODS: The National Inpatient Sample (NIS) database was accessed to gather data of hospitalized CLL patients with AF from 2009 to 2018. Propensity-score matching (PSM) and logistic regression model were performed to control for baseline patient factors to match 7265 CLL patient admissions with AF and 7265 CLL patient admissions without AF. The primary outcome was all-cause mortality (ACM), while the secondary outcomes included acute coronary syndrome (ACS), acute myocardial infarction (AMI), and the need for percutaneous coronary intervention (PCI), acute heart failure (AHF), acute hypoxic respiratory failure (AHRF), cardiac arrest (CA), cardiogenic shock (CS), stroke, and the total cost of hospitalization. RESULTS: CLL patients with AF had a higher rate of ACM (6.06% vs 4.47%; odds ratio [OR] 1.39, 95% confidence interval [CI] 1.19-1.61; P =< .001). All other secondary outcomes including ACS, AMI, PCI, AHRF, CA, CS, and stroke were observed at a significantly higher rate in the AF group as well. The median total hospital cost was also higher in the AF group ($9097 vs. $7646; P value < .0001) CONCLUSION: CLL patients with AF are at a significantly increased risk of all-cause mortality, cardiac-related mortality, and stroke. For this population, a multidisciplinary approach should be orchestrated for better management and outcomes.

Leg ulcer with long-term hydroxyurea use.

Long-term use of hydroxyurea can cause leg ulcers which usually do not heal unless the drug is discontinued. Patients should be counseled regarding alternative lines of treatment like anagrelide and pegylated-interferon.

Adenovirus-Associated Disseminated Intravascular Coagulation.

A 21-year-old previously healthy Caucasian female presented to the emergency department (ED) in the pre-COVID-19 era for evaluation of thrombocytopenia after a flu-like illness. The patient reported fever, cough, headache and myalgias for one week. She was on oral contraceptive pills (OCPs) for five years but discontinued one week ago. She was found to be in disseminated intravascular coagulation (DIC) and her hospital course was complicated by intraparenchymal hemorrhage, deep vein thrombus (DVT) in the right arm veins, bilateral pulmonary embolus (PE) and multiple splenic infarcts. An extensive workup was negative but nasopharyngeal swab came back positive for adenovirus by polymerase chain reaction (PCR).

Quebec platelet disorder.

A case of Quebec platelet disorder is hereby reported. A 33 years old woman presented with history of epistaxis and gum bleeding since childhood and menorrhagia and bleeding per vaginum after puberty, also had history of excessive blood loss after birth of child. Her coagulation profile was normal but platelet function testing by platelet aggregation assay showed abnormal aggregation of platelet with epinephrine. This type of response is seen in "Quebec platelet disorder" which is a rare autosomal dominant disorder of platelet function characterized by increased bleeding after any injury or trauma.