brain hepatorenal syndrome
Introduction
Introduction to brain, liver and kidney syndrome Ceribro-Hepato-Renal Syndrome was first reported by Bowen, Lee, and Zellweger in 1964, and is also known as Bowen-Lee-Zellweger syndrome, which is characterized by multiple malformations, mainly involving the nervous system. Liver and kidney, but no abnormal karyotype. Both men and women can develop the disease, the ratio is 1:2:7. The pathogenesis of this disease is unknown, and some people believe that it is due to placental iron transport dysfunction. basic knowledge The proportion of illness: 20% Susceptible people: no special people Mode of infection: non-infectious Complications: gastrointestinal bleeding
Cause
Causes of brain, liver and kidney syndrome
The disease is mostly siblings, the parents of the patients are normal, no abnormal karyotype is found, so the genetic pattern of the disease is considered to be autosomal recessive.
The pathogenesis of this disease is unknown, and some people believe that it is due to placental iron transport dysfunction.
Histopathological examination showed abnormal brain tissue, septic white matter malnutrition; cirrhosis, intrahepatic gallbladder and dysplasia; polycystic kidney; patent ductus arteriosus, patent foramen ovale and other cardiac malformations; atelectasis And hemosiderin deposition; islet cell proliferation, thymic dysplasia.
Prevention
Prevention of brain, liver and kidney syndrome
The disease is an autosomal recessive genetic disease, so there is no special precautionary measure. Two-thirds of patients with HRS are tl81 due to a large number of discharges of ascites, spontaneous bacterial peritonitis or other infections, and upper gastrointestinal bleeding. Therefore, avoiding the above incentives and giving targeted comprehensive treatment is reasonable. Diet, rational use of diuretics to avoid the use of nephrotoxic drugs, according to the patient's condition to give appropriate amount of albumin and plasma, the application of vasoactive drugs, help prevent the occurrence of HRS and improve the prognosis of patients with HRs.
Complication
Complications of brain, liver and kidney syndrome Complications, gastrointestinal bleeding
Easy complicated with heart malformations, brain malformations, head and face deformities, growth and dysplasia and other diseases. Liver failure, gastrointestinal bleeding, infection and hyperkalemia.
1, liver failure
(1) The clinical syndrome in which hepatocytes are extensively and seriously damaged, and the metabolic function of the body is seriously disordered, referred to as liver failure. Liver failure occurs in many serious liver diseases, with sinister symptoms and poor prognosis.
(2) The patient has hepatic encephalopathy, jaundice, hemorrhage, cerebral edema, ascites and other symptoms.
2, gastrointestinal bleeding
The clinical symptoms are hematemesis (dark red or bright red) and black stool. In cirrhosis, due to liver tissue fibrosis and connective tissue hyperplasia, blood vessels in the liver are damaged, occlusion occurs, and portal blood flow is blocked. More, due to blood circulation, venous blood vessels, expansion, venous wall thinning, at this time if eating coarse food, chemical stimulation and increased intra-abdominal pressure and other factors can cause rupture and bleeding.
3, infection
In patients with hepatorenal syndrome, due to decreased resistance, the body's immune function is reduced, and it is susceptible to attack by bacteria and toxins.
4, high blood potassium
Serum potassium ion >5 mEq/L is called hyperkalemia, and 6-7 mEq/L is moderate hyperkalemia, greater than 7
mEq/L is severe hyperkalemia. The most common cause of hyperkalemia is renal failure, which is mainly characterized by fatigue and arrhythmia.
Symptom
Symptoms of brain, liver and kidney syndrome Common symptoms Edema proteinuria weakness jaundice facial deformity Hepatorenal syndrome convulsion
Increased binding of serum iron and iron is helpful for diagnosis, but not all patients have this change, and isotope kidney and pyelography can be seen abnormal.
Head and face deformities, such as external ear malformation, forehead protrusion, large cardia, occipital flat, internal ecdysis, cataract, periocular edema, etc., neurological symptoms are general muscle weakness, tight reflexes disappear, convulsions, flexion contracture, patients Multiple growth dysplasia, large liver, jaundice, some cases with bleeding; proteinuria, hypoglycemia, cartilage calcification (especially the tibia).
Examine
Examination of brain, liver and kidney syndrome
General physical examination, blood routine, X-ray, CT, urine routine, blood biochemical examination. Blood routine is the most common and basic blood test. Blood consists of two major parts, fluid and tangible cells, which are routinely tested for blood. Blood has three different functions of cells - red blood cells (commonly known as red blood cells), white blood cells (commonly known as white blood cells), and platelets. The disease is judged by observing the quantitative change and the morphological distribution. It is one of the commonly used auxiliary examination methods for doctors to diagnose the disease.
Diagnosis
Diagnosis and differentiation of brain, liver and kidney syndrome
It can be diagnosed based on medical history, clinical symptoms and laboratory tests.
Pay attention to the identification of jaundice caused by heart disease such as mitral stenosis and other causes.
Histopathological examination showed abnormal brain tissue, septic white matter malnutrition; cirrhosis, intrahepatic gallbladder and dysplasia; polycystic kidney disease; patent ductus arteriosus, patent foramen ovale and other cardiac malformations; atelectasis And hemosiderin deposition; islet cell proliferation, thymic dysplasia.
