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Acquired Spherocytosis


Presentation

  • The Peripheral Blood Film, Second Edition presents the various concepts of the origin, development, and functions of blood cells.[books.google.de]
  • Employing a multidisciplinary approach, it presents the newest information available in the field, including new developments in technologies and the automation platforms on which measurements are performed.[books.google.de]
  • The purpose of the present article was to review the neonatal presentation of HS and to provide practical and up-to-date means of diagnosing and treating HS in neonates.[pediatrics.aappublications.org]
  • Most often, it presents in middle-aged and older individuals. Presentation Symptoms Symptoms are due to both anaemia and the underlying disorder. Patients with minimal or long-standing haemolytic anaemia can be asymptomatic.[patient.info]
  • This patient’s case demonstrates the clinical utility of high-density array karyotyping to define atypical clinical presentations and highlights the variable natural history of myelodysplastic syndromes.[bloodjournal.org]
Falling
  • That will tell you whether your patient’s anemia falls into the immune category or not. If the DAT is positive, you have an immune process.[pathologystudent.com]
  • The hemoglobin level will fall, and the body will not be able to produce many reticulocytes. It may take several days before the bone marrow is able to resume production of blood cells.[ihtc.org]
  • Oxygen saturation was difficult to maintain, with saturations falling to 80%. In order to oxygenate and ventilate the patient, an endotracheal tube was then inserted nasally after nasal spray with nasal decongestant and lubricant.[jove.com]
Lymphadenopathy
  • Lymphadenopathy or hepatosplenomegaly suggest an underlying lymphoproliferative disorder or malignancy; alternatively, an enlarged spleen may reflect hypersplenism causing hemolysis.[aafp.org]
Excitement
  • Includes a wealth of information on the exciting subject of omics ; these extraordinarily complex measurements reflect important changes in the body and have the potential to predict the onset of diseases such as diabetes mellitus.[books.google.de]
  • 30% of children with FXS are diagnosed with autism, and 2 - 5% of autistic children suffer from FXS. 6 Transcranial Magnetic Stimulation (a non-invasive neurostimulatory and neuromodulatory technique that can transiently or lastingly modulate cortical excitability[jove.com]

Workup

  • Diagnosis Diagnostic tests For all anemias: Because many therapies--especially erythrocyte transfusions--can interfere with the diagnostic workup, it is generally recommended that, if possible, relevant studies of whole blood, plasma, and serum are obtained[cancertherapyadvisor.com]

Treatment

  • Treatment The symptoms of HS are treatable, but the inherited red blood cell defect is not curable. The treatment for young children and those with mild HS may include oral folic acid which is needed to make RBC’s.[ihtc.org]
  • Treatment can also include dietary supplements. It is best to consult with doctor about the proper treatment plan for this disorder.[primehealthchannel.com]
  • TABLE 2 Laboratory Evaluation for HS in a Jaundiced Neonate Treatment Phototherapy should reduce the bilirubin level of jaundiced neonates with HS, and it is the mainstay of treatment in the first days after birth. 33 – 35 When a bilirubin level is found[pediatrics.aappublications.org]
  • There is no specific therapy other than treatment of the underlying infection and avoidance of implicated medications.[aafp.org]

Prognosis

  • When detected and cured early, patients of the condition show a good prognosis and the recovery time is much lesser in duration. References :[primehealthchannel.com]
  • The prognosis of an anemia is largely dictated by the prognosis of the corresponding underlying disorder. The patient should be continually monitored and supportive care provided while disease-specific therapy is administered.[cancertherapyadvisor.com]
  • What we see as the essence: "NRBC are often an only transient observation, but they indicate a poor prognosis, whether transient or persistent."[sysmex.de]
  • This protocol details the experimental and analytical procedure for a cell-based assay developed in our laboratory as a functional test to predict the prognosis of idiopathic scoliosis in asymptomatic and affected children.[jove.com]

Etiology

  • The patient's medical records provide an invaluable resource for differentiating congenital from acquired etiologies.[cancertherapyadvisor.com]
  • Once hemolysis has been identified, the etiology is sought.[msdmanuals.com]
  • The etiologies of hemolysis often are categorized as acquired or hereditary. Common acquired causes of hemolytic anemia are autoimmunity, microangiopathy, and infection.[aafp.org]
  • FIGURE 5 Evaluating a neonate with problematic jaundice in whom the etiology is unclear. Not all neonates who receive phototherapy for 2 days have hemolytic jaundice.[pediatrics.aappublications.org]

Epidemiology

  • Methods Statistics Trigonometry Medical & Nursing Anatomy Anesthesiology Audiology Bacteriology Biochemistry Bioethics Biomedical Science Cardiology Cardiovascular Childbirth Chiropractic Dentistry Dermatology Diagnostic Imaging Drugs Endocrinology Epidemiology[brainscape.com]
  • Epidemiology Risk factors are variable and depend on the underlying cause.[patient.info]
  • Epidemiology The wide variability in the reported prevalence of anemia results, in part, from the lack of standard universal definitions.[cancertherapyadvisor.com]
  • However, having the subjects report to a laboratory greatly reduces ecological validity, is not always doable in large scale epidemiological studies, and can be intimidating for young children.[jove.com]
Sex distribution
Age distribution

Pathophysiology

  • Paroxysmal Nocturnal Hemoglobinuria (PNH) Discuss the molecular and pathophysiologic defects in paroxysmal nocturnal hemoglobinuria (PNH) and explain the test used to diagnose this disorder. List complications of PNH.[hematology.org]
  • Some anemias may result from more than one pathophysiology (e.g., gastrointestinal losses and nutritional reticulocytopenia in alcoholics). Knowledge of the relevant pathophysiology can guide diagnostic and treatment strategies.[cancertherapyadvisor.com]
  • […] hemolytic transfusion reactions ABO hemolytic diseases of newborn/Rh hemolytic disease of newborn Hereditary spherocytosis Intravenous water infusion or drowning (fresh water) hypophosphatemia Bartonellosis Snake bite hyposplenism Rh-null phenotype Pathophysiology[en.wikipedia.org]
  • Disorders of hemoglobin synthesis Hb C disease Hb E disease Hb S-C disease Sickle cell disease Thalassemias Disorders of RBC metabolism Embden-Meyerhof pathway defects (eg, pyruvate kinase deficiency) Hexose monophosphate shunt defects (eg, G6PD deficiency) Pathophysiology[msdmanuals.com]
  • Pathophysiology There are two mechanisms of hemolysis. Intravascular hemolysis is the destruction of red blood cells in the circulation with the release of cell contents into the plasma.[aafp.org]

Prevention

  • Provides guidance on error detection , correction, and prevention, as well as cost-effective test selection. Features a full-color layout , illustrations and visual aids , and an organization based on organ system.[books.google.de]
  • Spherocytosis Prevention As this is an inherited disease, it is impossible to prevent its occurrence. However, regular screening and early treatment of high-risk individuals can help avoid the risk of complications of the condition.[primehealthchannel.com]
  • A splenectomy does not cure this disease, but it can cure the anemia and prevent the development of gallstones. Since the spleen is a filter of bacteria for the body, its removal can result in an increased risk of certain infections.[ihtc.org]
  • We predict that early suspicion, prompt diagnosis and treatment, and anticipatory guidance will prevent adverse outcomes in neonates with HS.[pediatrics.aappublications.org]
  • The mean corpuscular hemoglobin concentration frequently is elevated. 2 , 21 Splenectomy effectively arrests the extravascular hemolysis and prevents its long-term complications, such as cholelithiasis and aplastic crises.[aafp.org]

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