Asked by Andy Blackwell Uncategorized
Why periorbital swelling is first sign in nephrotic syndrome?
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Asked in Conditions and Diseases
How is nephrotic syndrome diagnosed?
Congenital nephrotic syndrome?
Definition Congenital nephrotic syndrome is a disorder passed down through families in which a baby develops protein in the urine and swelling of the body. Congenital means it is present from birth. However, this group of disorders also includes nephrotic syndrome that occurs in the first 3 months of life. See also: Nephrotic syndrome Alternative Names Nephrotic syndrome - congenital Causes, incidence, and risk factors Congenital nephrotic syndrome is a very rare form of nephrotic syndrome. It occurs mostly in families of Finnish origin and develops shortly after birth. It is inherited, which means it is passed down through families. Children with this disorder have an abnormal form of a protein called nephrin. The kidney's filters (glomeruli) need this protein to function normally. Symptoms Cough Decreased urine output Foamy appearance of urine Low birth weight Poor appetite Swelling (total body) Signs and tests An ultrasound done on the pregnant mother before birth may show a larger-than-normal placenta. The placenta is the organ that develops during pregnancy to feed the growing baby. Pregnant mothers may have a screening test done during pregnancy to check for this condition. The test looks for higher-than-normal levels of alpha-fetoprotein in a sample of amniotic fluid. Genetic tests should be used to confirm the diagnosis if the screening test is positive. After birth, the infant will show signs of severe fluid retention and swelling. The health care provider will hear abnormal sounds when listening to the baby's heart and lungs with a stethoscope. Blood pressure may be high. There may be signs of malnutrition. A urinalysis reveals fat and large amounts of protein in the urine. Total protein in the blood may be low. Treatment Early and aggressive treatment is needed to control this disorder. Treatment may involve: Antibiotics to control infections Blood pressure medicines called ACE inhibitors and ARBs to reduce the amount of protein leaking into the urine Diuretics ("water pills") to remove excess fluid Nonsteroidal anti-inflammatory drugs (NSAIDs) such as indomethacin to reduce the amount of protein leaking into the urine Fluids may be limited to help control swelling. The health care provider may recommend removing the kidneys to stop protein loss. This may be followed by dialysis or a kidney transplant. Expectations (prognosis) The disorder often leads to infection, malnutrition, and kidney failure. It can lead to death by age 5, and many children die within the first year. Congenital nephrotic syndrome may be controlled in some cases with early and aggressive treatment, including an early kidney transplant. Complications Acute kidney failure Blood clots Chronic kidney failure End-stage kidney disease Frequent, severe infections Malnutrition and related diseases Calling your health care provider Call your health care provider if your child has symptoms of congenital nephrotic syndrome. References Nachman PH, Jennette JC, Falk RJ. Primary glomerular disease. In: Brenner BM, ed. Brenner and Rector's the Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 30. Pais P, Avner ED. Nephrotic syndrome. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap. 521. Reviewed By Review Date: 09/20/2011 David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Herbert Y. Lin, MD, PhD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
Asked in Health, Conditions and Diseases, Down Syndrome
Who was the first person to identify Down's syndrome?
Minimal change disease?
Definition Minimal change disease is a kidney disorder that can lead to nephrotic syndrome, although the nephrons of the kidney look normal under a regular microscope. Alternative Names Minimal change nephrotic syndrome; Nil disease; Lipoid nephrosis; Idiopathic nephrotic syndrome of childhood Causes, incidence, and risk factors Each kidney is made of more than a million units called nephrons, which filter blood and produce urine. In minimal change disease, there is damage to the glomeruli -- the tiny blood vessels inside the nephron where blood is filtered to make urine and waste is removed. The disease gets its name because this damage is not visible under a regular microscope. It can only be seen under an electron microscope. Minimal change disease is the most common cause of nephrotic syndrome in children. It causes about 80% of cases in young children. It is also seen in adults, but makes up only 10 to 15% of nephrotic syndrome cases. The cause is unknown, but the disease may occur after or be related to: Allergic reactions NSAID use Tumors Vaccinations Viral infections Symptoms There may be symptoms of nephrotic syndrome: Foamy appearance of the urine Poor appetite Swelling (especially around the eyes, feet, and ankles, and in the abdomen) Weight gain (from fluid retention) Minimal change disease does not reduce the amount of urine produced. It rarely progresses to kidney failure. Signs and tests The doctor may not be able to see any signs of the disease, other than swelling. Blood and urine tests reveal signs of nephrotic syndrome, including: High cholesterol High levels of protein in the urine Low levels of albumin in the blood A kidney biopsy and examination of the tissue with an electron microscope can show signs of minimal change disease. An immunofluorescence exam of the biopsied kidney tissue will be negative. Treatment Corticosteroids can cure minimal change disease in most children. Some patients may need to stay on steroids to keep the disease in remission. Adults do not respond to steroids as well as children, but many still find steroids effective. Adults may have more frequent relapses and become dependent on steroids. Patients who have three or more relapses may do better with cytotoxic therapy instead of steroids. In most cases, this involves a medication called cyclophosphamide. Other medicines that have been used include cyclosporine and chlorambucil. Swelling may be treated with: ACE inhibitor medicines Blood pressure control Diuretics (water pills) You may also be told to reduce the amount of salt in your diet. Expectations (prognosis) Children with minimal change disease usually respond better to corticosteroids than adults. Children often respond within the first month. A relapse can occur. However, patients may improve after long-term treatment with corticosteroids and medications that suppress the immune system (immunosuppressive medications). Complications Nephrotic syndrome Side effects of medications Calling your health care provider Call for an appointment with your health care provider if: You develop symptoms of minimal change disease You have this disorder and your symptoms get worse You develop new symptoms, including side effects from the medications used to treat the disorder Prevention There is no known prevention. References Appel GB. Glomerular disorders and nephrotic syndromes. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 122. Nachman PH, Jennette JC, Falk RJ. Primary glomerular disease. In: Brenner BM, ed. Brenner and Rector's the Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 30. Pais P, Avner ED. Nephrotic syndrome. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 521. Reviewed By Review Date: 09/20/2011 David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Herbert Y. Lin, MD, PHD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
Asked in Spinal Cord
When was partial spinal sensory syndrome developed?
How do you reduce swelling after having wisdom teeth removed?
Do women with Turner syndrome look like normal females?
Turner Syndrome occurs when a female has only one X chromosome. One of the first signs of Turner is short stature which becomes apparent at about age 5. Most Turner Syndrome women do not go through puberty unless given hormone therapy. They can have obvious skeletal deformities, a lower than normal hairline on the back of their necks, swelling of the hands and feet and additional folds on their necks.