Leukemia is cancer that affects the blood formation in the bone marrow, which is characterized by an increase in the number of leucocytes (leukocytosis) in the blood and bone marrow. Leukemia has four major types, which include acute myeloid leukemia (AML), acute lymphoid leukemia (ALL), chronic myeloid leukemia (CML), and chronic lymphoid leukemia (CLL). Hematopoiesis is a process in which the stem cells differentiate into erythrocytes, megakaryocytes, and immune cells of myeloid, and lymphoid, in bone marrow or lymphatic tissues. The blood is made up of fluid called plasma and three types of cells and each type has special functions. White blood cells (also called WBCs or leukocytes) help the body fight infections and other diseases. Red blood cells (also called RBCs or erythrocytes) carry oxygen from the lungs to the body’s tissues and take carbon dioxide from the tissues back to the lungs. The red blood cells give blood its color. Platelets (also called thrombocytes) help form blood clots that control bleeding. Leukemias can affect any age and the incidence is different between each type. The Clinical presentation of leukemia cells may be mature, such as in chronic myeloid leukemia, and it may come in precursor form such as in acute myeloid leukemia It can also come in two forms (precursor and mature) such as chronic myeloid leukemia. Many risk factors have been identified, such as Klinefelter, Down syndromes, ataxia telangiectasia, Bloom syndrome, Viral infections from Epstein Barr virus, Human T-lymphotropic virus, ionizing radiation exposure, radiation therapy, environmental exposure with benzene, and smoking history. There is a strong relationship between nutrition status and leukemia which strongly affect the overall survival rate. This article aims to provide a brief introduction and the signs and symptoms of leukemia, its causes, epidemiology, nutrition profile, treatment prevention and good practice management advice. Keywords: Leukemia, Acute Myeloid Leukemia, Acute Lymphoid Leukemia, Chronic Myeloid Leukemia. Chronic Lymphoid Leukemia
Congenital enteropeptidase deficiency (CEP), also known as enterokinase deficiency. CEP is an uncommon autosomal recessive genetic disorder mostly characterized by severe chronic diarrhoea after delivery, hypoproteinemia, and failure to grow. Enteropeptidase activity is anticipated to play a significant role in protein digestion. For growth and appropriate development in newborns with a congenital lack of the enzyme, pancreatic enzyme replacement treatment or an amino acid combination must be given. Only 13 cases of enterokinase insufficiency have been recorded since it was originally characterized in 1969. Couples should be informed that prenatal screening is an option and that EKD has a favourable prognosis. However, if an EKD patient is born, parents should be aware of the feeding issue and offer the proper pancreatic exocrine secretion medication. One research found that all patients had been diagnosed as newborns 25 years ago. Even when the pancreatic-enzyme replacement was stopped, they appeared to lead regular lives as adults, free of gastrointestinal issues and with normal body weight. This can be explained by the fact that trypsin, once liberated from its precursor, encourages additional trypsinogen activation in a positive-feedback manner. Better pharmaceutical preparations such as enteric-coated minimicrospheres and delayed-release capsules are used for better results as it maintains the enzyme and prevents its breakdown by stomach acidity. This review aims to summarise current knowledge of pathophysiology, causes, and treatment of Intestinal Enterokinase Deficiency in Pediatrics
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