Rituximab in the Management of Refractory Myasthenia Gravis and Variability of Its Efficacy in Anti-MuSK Positive and Anti-AChR Positive Myasthenia Gravis.

Myasthenia gravis affects the neuromuscular junction of the skeletal muscles. It results in muscle weakness involving skeletal muscles (diaphragm, extraocular muscles) and myasthenic crisis. Treatment options for myasthenia gravis management have expanded, including azathioprine, corticosteroids, plasma exchange, and tacrolimus. Unfortunately, a few cases of myasthenia gravis don't respond to conventional treatment modalities. Monoclonal antibodies, rituximab (RTX), are novel treatments that have garnered interest as of late due to their efficacy within the patient population presented with refractory form myasthenia gravis. This review aims to showcase how RTX is an effective treatment within different forms of myasthenia gravis. A limited review was performed using databases that include PubMed and Google Scholar. The following keywords were used: "myasthenia gravis," "rituximab," "monoclonal antibody," "anti-AChR antibody," and "refractory myasthenia." The review focused on case reports, human studies, or research surveys based on the inclusion criteria of human studies involving participants more than 18 years of age and published in English literature. Out of 69 articles, 14 were duplicates, and 29 were relevant and met the inclusion criteria. The findings from the study demonstrate that patients with refractory myasthenia gravis responded well to RTX treatment. Furthermore, RTX has been shown to decrease corticosteroid dependence, induce sustained remission, and have a favorable response to anti-MuSK antibody positive myasthenia gravis compared to anti-AChR antibody positive myasthenia gravis. This literature review suggests that patients with refractory myasthenia gravis can benefit from rituximab; however, it has a variable response in different forms of myasthenia gravis.

Biliary Atresia With Extrahepatic Cyst: A Diagnostic Dilemma.

Biliary atresia (BA) is a rare disorder that usually presents with cholestatic symptoms such as jaundice, pale stool, and dark urine shortly after birth. Intracranial hemorrhage is a rare manifestation. BA may sometimes be associated with a cyst in the biliary tree. Differentiating choledochal cyst with BA or cystic biliary atresia can be quite challenging yet very important as they differ significantly in their prognosis and treatment. Furthermore, clear mucoid fluid in the extrahepatic cyst is an unusual occurrence. This case report aims to raise awareness about this rare variant to facilitate appropriate and timely diagnosis and treatment. We report a case of an infant who presented with intracranial hemorrhage and conjugated hyperbilirubinemia. The ultrasonographic and magnetic resonance cholangiopancreatography (MRCP) findings suggested Type I choledochal cyst, but it was later diagnosed as biliary atresia with an extrahepatic cyst and was not corrected by Kasai's operation.

Diagnostic Scores and Treatment Options for Acute Disseminated Intravascular Coagulation in Children.

Disseminated intravascular coagulation (DIC) is a thrombo-hemorrhagic condition that commonly accompanies life-threatening illnesses in children and is associated with significant morbidity and mortality. Treatment of underlying conditions, hemodynamic support, and replacement therapy with blood components is the mainstay of DIC management. Limited research studies have supported the use of antithrombin (AT), recombinant thrombomodulin (rTM), and protein C concentrates (PrCC). Although there have been several studies and advancements in the DIC treatment in adults, data in pediatric patients are limited, and the consensus is lacking. Evidence validating the use of diagnostic scoring systems in the pediatric population is also limited. Since the hemostatic system differs significantly in children, especially in neonates, management of DIC is also different in children from that of adults, and there is a dire need for good quality research studies in this aspect. We reviewed more than 100 articles in PubMed, Cochrane database, and Google Scholar. This traditional review article discusses different scoring systems for diagnosing DIC in pediatric patients, and different pharmacological treatment options for acute DIC in this population. This study mainly focuses on papers published from 1990 to 2021 and includes papers in all languages involving humans only.

A Systematic Review of Effects of Vitamin E on the Cardiovascular System.

Vitamin E is a fat-soluble vitamin and an antioxidant that prevents the peroxidation of lipid in vitro. The antioxidant role of vitamin E in preventing adverse cardiovascular outcomes is controversial as some studies support it, while others reject it. Therefore, this review aims to determine whether there is an association between vitamin E and cardiovascular diseases (CVDs). An electronic search was done to find out relevant articles. Papers were shortlisted after the initial title and abstract screen. A full-text study was done, and inclusion and exclusion criteria were applied before the quality assessment of each paper was done. Only high-quality papers were selected for analysis. Full-text articles of the last ten years were included, while non-English articles, gray literature, and animal studies were excluded. The majority of the papers, including 75% of the total population in this review, suggested no role of vitamin E in preventing CVD and CVD mortality. Some studies also suggested that a high level of vitamin E can be associated with adverse cardiovascular outcomes. Thus, one should be prudent about taking vitamin E supplementation for cardiovascular risk prevention.

Ewing's Sarcoma of Rib in a Four Year Old: A Case Report.

Although rare, the Ewing's sarcoma family of tumors represents the second most common primary bone malignancy that typically occurs in adolescents and young adults aged 10-25 years, presenting with localized pain and swelling of a long bone. We report a case of Ewing's sarcoma of rib in a four years old child that presented with acute onset of fever, cough, shortness of breath and was initially treated as pneumonia. Although the patient did not belong to typical demography and the symptoms were suggestive of infective pathology, careful scrutiny of the radiographic findings led to further investigations and diagnosis of Ewing's sarcoma of rib after correlating computerized tomography scan findings with immunohistochemistry reports.

Vitamin B12 Deficiency Resembling Acute Leukemia: A Case Report.

Vitamin B12 deficiency in children can cause megaloblastic anemia, poor growth, and increased chances of infections. It is an important reversible cause of bone marrow suppression which at the time of presentation can mimic hematological malignancy. Therefore, it should be considered as a differential diagnosis in cases suspected of acute leukemia. We report a case of 14 months old child who had atypical presentation of vitamin B12 deficiency. He had chronic fever, decreased feeding and increased paleness for one year. Pancytopenia with severe anemia was present along with 19% reactive/atypical cells in peripheral blood smear suggesting acute leukemia. However, bone marrow aspiration and biopsy showed features of megaloblastic anemia. Vitamin B12 level measured was very low and treatment with cyanocobalamin caused drastic improvement in the child's condition.