China
Africa
Explore chapters and articles related to this topic
Gastrointestinal system
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
6.30. A 2-month-old breast-fed infant is brought to see you because of persistent jaundice since birth. His stools are pale and his urine is highly coloured. Urine shows presence of bile but no urobilin. Which of the following is/are possible diagnosis/es?Hypothyroidism.Congenital viral infection.Hereditary spherocytosis.Congenital biliary atresia,Breast milk jaundice.
Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
In hereditary spherocytosis, in addition to the above-described signs and symptoms of chronic hemolysis, splenomegaly, hepatomegaly, and cholelithiasis are frequently observed. The osmotic fragility of RBCs is increased, the Coombs test is negative.
Miscellaneous Forms of Acquired Red Cell Aplasia and Erythropoietic Failure in Childhood
Published in Stephen A. Feig, Melvin H. Freedman, Clinical Disorders and Experimental Models of Erythropoietic Failure, 2019
Melvin H. Freedman, Stephen A. Feig
Patients with hemolytic anemias who are at risk of parvovirus-induced erythropoietic failure should ensure optimal nutrition, particularly with regard to iron, vitamin B]2, and folic acid. Interventions that decrease the need for accelerated erythropoiesis should also be considered. For example, the shortened red cell survival in patients with hereditary spherocytosis can be “cured” by splenectomy and they should be considered for this procedure. Because parvovirus-induced erythropoietic failure usually occurs only once, however, the occurrence of an aplastic crisis need not be considered an absolute indication for subsequent splenectomy.
Severe autoimmune hemolytic anemia complicating hereditary spherocytosis treated successfully with glucocorticoids and cyclosporine: a case report
Published in Hematology, 2023
Na Wang, Hongkai Lu, Linzhang Li, Ming Gong, Yongtong Cao
Hereditary spherocytosis (HS) encompasses a heterogeneous group of inherited anemias characterized by the presence of spherical RBCs on peripheral blood smears. It is prevalent worldwide, with a high incidence of 1:2000 in northern Europeans [6] and an incidence of about 1:100,000 in the Chinese population [7]. HS is characterized by anemia, jaundice, splenomegaly, and cholelithiasis [8]. In addition, abnormalities in the quantity and/or quality of erythrocyte membrane proteins can lead to the reduced deformability of erythrocytes, which can easily break down and cause hemolysis [9,10]. Five genes are associated with HS and involved in the interaction between the erythrocyte membrane and lipid bilayer: ankyrin (ANK1); β-spectrin (SPTB); α-spectrin (SPTA1); solute carrier family 4, member 1 (SLC4A1); and erythrocyte membrane protein 4.2 (EPB42) [11–13]. Mutations in one or more of these genes can cause membrane protein deficiency, leading to HS [14,15].
Consideration of Splenectomy in Unstable Hemoglobinopathy: A Case Report of Hb Hammersmith (HBB: c.128T>C)
Published in Hemoglobin, 2022
Michaela M. Pesce, Mandy M. Atkinson, Vaishnavi Sridhar, Erwood G. Edwards
The majority of patients with Hb Hammersmith have been reported to present with severe Heinz body hemolytic anemia, hyperbilirubinemia, splenomegaly and transfusion dependence [6]. In patients with more common forms of hemolytic anemia, such as hereditary spherocytosis and thalassemias, splenectomy is considered an acceptable treatment option when noninvasive therapy has failed. Several studies report the utility of splenectomy in these conditions, with symptomatic improvement as well as a significant increase in Hb levels after either subtotal or total splenectomy [7–9]. However, none of these studies explored the clinical benefit of splenectomy in cases of unstable Hb variants. A review of the literature revealed several case studies that include patients with Hb Hammersmith who have reportedly undergone splenectomy, but only four cases discuss whether there was clinical improvement of patients thereafter.
Successful treatment of a patient with refractory immune thrombocytopenic purpura in systemic lupus erythematosus with rituximab
Published in Immunological Medicine, 2019
Kazuya Abe, Yuichi Ishikawa, Junichi Ishikawa, Michio Fujiwara, Yasuhiko Kita
CSs are first-line therapy in SLE-ITP treatment; most patients respond relatively well and approximately 20% achieve remission after CS monotherapy [4]. When SLE-ITP is resistant to CS monotherapy, splenectomy or IVIG are sometimes used, similar to primary ITP; however, this has not been well-studied and randomized controlled trials are lacking [8]. In SLE-ITP patients with a high risk of hemorrhage, IVIG is a good therapeutic option because it results in a rapid increase in platelet count [9]. However, its effectiveness may be temporary and induce difficulty of tapering CSs [10]. In addition, IVIG is expensive and carries a small risk of anaphylactic and anaphylactoid reaction. On the other hand, splenectomy may induce a lupus flare or lupus nephritis, given that the spleen is responsible for clearance of circulating immune complexes [11]. In addition, patients who undergo splenectomy are at risk for postsplenectomy infection. A previous study has reported a lifelong risk of severe and potentially fatal infection in patients with hereditary spherocytosis after splenectomy [12]. This is a particular concern in SLE patients who may require long-term immunosuppressive therapy. Moreover, the splenectomy procedure itself may have a high risk in SLE patients receiving CSs because CSs increase the risk of perioperative infection and gastrointestinal hemorrhage and impair wound healing [13–15].