Taiwan is the “kingdom of kidney washing”, the incidence and prevalence of end-stage kidney disease is among the best in the world, but there is a group of patients with kidney disease is not acquired disease caused, these people because of hereditary kidney disease, from the age of more than 20 years, the kidney will appear symptoms. Doctors urged those with a family history to take better control of their lives or risk a rapid decline in kidney function as a result of something as simple as not drinking enough water.
Autologous dominant polycystic kidney disease (ADPKD, also known as polycystic kidney disease, bubble kidney disease) is the most common inherited kidney disease with single gene mutation in nephrology. Compared with other long-term kidney diseases, people generally have less understanding of dominant polycystic kidney disease, but in fact it is the most common inherited kidney disease.
Ministry of Health and welfare department of the Kidney medicine chief Physician Ye Zheting pointed out that, in terms of statistics, every 400-1000 people will have a polycystic kidney patient, in the wash kidney patients accounted for 5%~10%.
Box: Polycystic kidney Disease File
Official name: Autologous dominant polycystic kidney disease, ADPKD.
Polycystic kidney, bubble kidney.
Symptoms: hypertension, bloating, low back pain, proteinuria, hematuria.
Complications: uremia, kidney failure, liver cysts, cerebral hemangioma (prone to stroke), urinary tract infection, heart valve problems.
Types: The most common types are type 1 (about 85%) and Type 2 (about 15%). Type 1 patients are around 50-60 years old (average 54 years old), and renal function may be destroyed to the extent that kidney washing is needed. Type 2 has mild symptoms and requires kidney washing around the age of 70 to 80 (average age of 74).
Odds: it’s a dominant heredity. If one of your parents gets sick, you have a 50% chance of getting sick.
The kidney bubbles! Invade the kidney structure and damage the health
The most obvious feature of a polycystic kidney, Ye explained, is that the kidneys appear to be full of blisters. The mechanism for blisters in polycystic kidneys is related to the hormone vasopressin, which acts on the epithelium of the urinary tract, increasing cell division and secreting watery fluid, so these blisters form and become increasingly filled with fluid.
“Blisters in the polycystic kidney grow more and more and invade the normal structure of the kidney, leading to worse and worse kidney function.” Ye zheting explained. Many people with polycystic kidney disease face kidney washing problems after age 50.
If you have high blood pressure at a young age, be careful if you can’t find a cause
Although people with polycystic kidney disease don’t begin to show symptoms until after age 40, many underlying disease processes begin in their 20s.
Ye said that not all patients with polycystic kidney disease experience back or lower back pain, and some patients with no symptoms are found to have poor renal function during the health examination, and further investigation into the reason was found. “For example, people who have high blood pressure clinically at a young age, if they can’t find the cause, they need to get checked for hematuria, albuminuria, or arrange for ultrasound, and that’s when they have the opportunity to find out what’s going on with pISId.”
Others may develop unexplained hematuria, or polycystic kidney disease after common causes such as tumors, infections, and stones have been ruled out.
Imaging data plus family history can be used to diagnose PCD
Although autologous dominant POLYcystic kidney disease is an inherited disease, its diagnosis does not necessarily require confirmation by genetic testing. Imaging studies such as ultrasound, computed tomography, or MRI can confirm this if multiple vesicles in the kidney meet imaging criteria and if the patient has a family history of the disease.
Ye pointed out that the routine examination is based on biochemical tests, such as blood and urine tests, plus imaging tests to confirm the diagnosis. “There’s usually no need for genetic testing, but in patients who have a lot of polycystic kidney disease imaging and don’t have a family history, we do genetic testing.” In about 5 percent of patients, he said, neither parent has the disease, but during fertilization, the gametes themselves or the fertilized egg develop a genetic mutation that causes polycystic kidney disease, a condition like this that can be identified by genetic testing.
In addition, the fact that there is no family history does not mean non-genetic, some of which are inherited, but because parents died earlier, or parents’ symptoms are not obvious, so there is no obvious family history.
Control 4 daily activities, patients with disease do not go to wash the kidney!
“There is increasing awareness of the disease. Clinically, children whose parents are washing their kidneys due to polycystic kidney disease have a 50% chance of inheriting the disease, so they are worried about their kidney health and ask for an examination.” Share by Ye Zheting.
Facing these anxious young patients, Ye zheting offers four suggestions. “We can’t control the disease, but we can control the rate at which it develops. Young people diagnosed with polycystic kidney disease are clinically treated as chronic kidney disease. And it needs to be tracked continuously, and now there are new drugs available, so not everyone is going to have to wash their kidneys in the future.”
- Blood pressure control: Controlling blood pressure is the most important thing, which is the same for all kidney diseases, says Ye. “With chronic hypertension, kidney function deteriorates rapidly and you may end up with kidney washing.”
People with POLYcystic kidney disease have very obvious hypertension problems at a young age. Active control of blood pressure, on the one hand, prevents rapid deterioration of the disease, and on the other hand, these patients may be complicated with other diseases, such as fatal hemangioma in the brain. Controlling blood pressure can also reduce the occurrence of these vascular diseases.
- Drink plenty of water: Ye urges patients to drink as much water as possible because polycystic kidneys are linked to vasopressin, and when you drink a lot of water, your body produces less of the hormone. The function of antidiuretic hormone is to keep water in the body. If you drink water all the time, your body will be fully hydrated, so the antidiuretic hormone will be reduced and the vesicles will grow more slowly.
These patients also often have problems with hematuria, stones, or urinary tract infections. Drinking a lot of water is like flushing a pipeline frequently, so there are fewer blockages and bacterial infections can be avoided. “Some people ignore it, but every infection, every time, disrupts kidney function.” Ye Zheting reminded.
He recommends that adults drink at least three liters of water a day. “Drinking water is just as important as medicine. We should treat water as medicine and enforce it as strictly as medicine.”
- Salt control: The purpose of salt control is to keep blood pressure stable. In addition, salt restriction can slow the deterioration of renal function and prevent the formation of stones. Generally speaking, the diet of much salt, polysaccharide is easy to bring about stone occurrence.
- When kidney conditions are less than ideal, consider protein restriction: how appropriate dietary protein intake should be. The physician will refer the patient to a nutritionist to establish a nutrition log to help control protein intake. “However, this part does not need to be controlled by itself in advance, and should only be severely restricted if the patient has proteinuria or poor renal function.” Ye zheting pointed out.
Polycystic kidney disease is an underrated disease! Medical advice: Start with a family history
‘Taiwan is the kingdom of kidney washing, but this hereditary kidney disease should be underestimated,’ Mr. Yip said. “Statistically, there will be one pCOS patient per 400 to 1,000 people, and the younger you are, the better.”
Dr. Yip suggested that tracing a patient’s family history would be the most efficient way to find out. Several patients with polycystic kidney disease in Nantou area were diagnosed in this way. “Controlling it from a young age, using it when you’re eligible, is a better way to control the disease.”
Ye Zheting a young patient, is because the mother also has to wash the kidney, after the patient examination found that he is also hereditary kidney disease. It has been under control for four or five years, and the new drug started being used as soon as it was eligible for medicare benefits last July.
Take medicine to cooperate to drink water actively, get rid of to wash kidney fatalism
For polycystic kidney disease, there are new drugs that can delay the decline of renal function. It has been more than a year since the national Health Insurance benefits were passed in July (2019). Ye zheting said that after the new drug is paid through the National Health Insurance, patients take the initiative to ask about the drug.
The drug works by suppressing the effects of vasopressin on the kidneys, requiring patients to drink lots of water and pee in the toilet frequently. “But that’s what people with the disease do. In fact, all of the patients I work with are willing to adjust their lifestyle.”
Although there is currently no radical cure for pCOS, doctors have ample experience to delay the disease. “Some patients will have an escape mentality, but as long as you are willing to go out to see a doctor, Taiwan’s doctors, drugs are in place, will accompany you to face!” Ye zheting encouraged.