In the event of a pregnancy where the fetus has the Rh-D blood antigen and the mother does not, the mothers immune system will respond to the red blood cells as foreign and create antibodies against the Rh-D antigen on the fetal blood cells. Rh disease develops in the event of a second pregnancy where the mothers immune system launches an attack, via IgG, against the infants Rh-D positive blood cells. The immune response results in hemolysis of fetal red blood cells causing severe anemia.Hemolysis caused by the Rh incompatibility, causes extramedullary hematopoiesis in the fetal liver and bone marrow. The push to make more erythroblasts to help compensate with the hemolysis over works the liver causing hepatomegaly. The resulting liver dysfunction decreases albumin output which in turn decreases oncotic pressure. Consequentially, the decrease in pressure results in overall peripheral edema and ascites.Rh disease is currently an uncommon cause of immune-mediated hydrops fetalis. Due to preventative methods developed in the 1970s, the incidence of Rh disease has markedly declined. Rh disease can be prevented by administration of anti-D IgG (Rho(D) Immune Globulin) injections to RhD-negative mothers during pregnancy and/or within 72 hours of the delivery. However, a small percentage of pregnant mothers are still susceptible to Rh disease even after receiving anti-D IgG (Rho(D) Immune Globulin). Non-immune pathophysiology
Severe anemia leads to hyperdynamic circulation, which means high-output cardiac failure causes the blood to circulate rapidly. The excessive pumping of blood causes the left side of the heart to fail leading to pulmonary edema.
Carglumic acid, sold under the brand name Carbaglu among others, is used for the treatment of hyperammonaemia.Carglumic acid is a carbamoyl phosphate synthetase 1 (CPS 1) activator.The most common adverse effects include vomiting, abdominal pain, pyrexia (fever), and tonsillitis, anemia, diarrhea, ear infection, other infections, nasopharyngitis, decreased hemoglobin levels, and headache.It was approved for medical use in the United States in March 2010. Carglumic acid is an orphan drug. Medical uses
Carglumic acid is indicated for the treatment of acute hyperammonemia and chronic hyperammonemia. References
External links
"Carglumic acid". Drug Information Portal. U.S. National Library of Medicine.