Rare Kidney Malformation Accompanied By Severe Hypotension, This May Save The Life Of The Child!

Bilateral renal agenesis (BRA) is a fatal congenital malformation caused by the failure of both kidneys to develop normally during early embryonic development. Despite advances in fetal and neonatal treatment, morbidity and mortality in infants with congenital BRA remain high.

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At the same time, children with BRA may be complicated by severe hypotension, and traditional vasopressors may develop resistance. Recently, PEDIATRICS published a case report on children with BRA [1], indicating that angiotensin II may become a new option for the treatment of refractory hypotension in infants with BRA.

After a pregnant woman was diagnosed prenatally with BRA in her fetus, clinicians performed a series of amnioinfusions according to the Fetal Treatment of Intraamniotic Renal Insufficiency Trial Protocol (NCT 03101891).

The pregnant woman received 11 amnioinfusions between the estimated gestational age (EGA) of 22 and 31 weeks. The pregnant woman developed fetal membrane rupture at 31+1 weeks and received betamethasone treatment. Then at 33+0 weeks, the pregnant woman successfully gave birth to a baby boy through cesarean section.

The child required high-frequency oscillatory ventilation and nitric oxide therapy due to pulmonary artery hypoplasia, pulmonary hypertension, and respiratory distress syndrome. The next day, the clinician started using Aqaudex (a new type of simple ultrafiltration device) on the child for renal replacement therapy. The child's initial cranial ultrasound showed grade III hemorrhage on the left side of the ventricle, accompanied by ventricular enlargement. On the 10th day of treatment, the child was separated from the oxygen tube and showed intermittent hypoxia, requiring timely supplementation of oxygen. During treatment, the child also received regular dopamine and vasopressin treatments and occasional midodrine injections.

At 3 months of age, the child developed intermittent hypotension despite treatment with midodrine and daily Aqaudex. Clinicians treated him with phenobarbital, fosphenytoin, and levetiracetam, but his hypotension worsened and he developed focal seizures that progressed to status epilepticus within 24 hours.

Clinicians examined the child's infectious causes, including cerebrospinal fluid analysis and intracranial ultrasound, and found no evidence of cerebral hemorrhage or hydrocephalus. It is worth mentioning that the child's previous intermittent hypotension persisted, and after measurement, the systolic blood pressure (DBP) was between 40 and 50 mmHg, and the mean arterial pressure (MAP) was between 25 and 35 mmHg.

The child received fluid resuscitation and was restarted on hydrocortisone, along with infusions of dopamine, vasopressin, and epinephrine. The child's cerebral blood oxygen saturation was monitored to be 30%-50% (previously 50%-70%), the body (flank) blood saturation was 80%, and the perfusion index (PI) was intermittently lower than 1%.

Due to the child's refractory hypotension, the clinician started injecting angiotensin II at 1.25 ng/kg/min on the 5th day of treatment and titrated it to 40 ng/kg/min within the next 4 days. Slowly reduce the dosage within a few days and discontinue use. At the same time, during the use of angiotensin II, continuous infusion of heparin is supplemented to prevent thrombosis.

It was found that the patient initially needed hydrocortisone (2mg/kg) every 6 hours, maximum dose injection of dopamine 13mcg/kg/min, vasopressin 0.7mU/kg/min, and epinephrine 0.5mcg/kg/min. After administration of angiotensin II, all pressor movements were successfully stopped without a significant decrease in MAP.

The child was treated with angiotensin II and eventually recovered and was discharged.

BRA is a rare disease in fetal development that seriously harms fetal health. Ultrasound showing oligohydramnios is often suggestive of BRA. Although the exact cause is unknown, BRA is associated with mutations in many kidney development genes.

BRA can be a single disease, or it can be a pathological change in certain syndromes, such as X-linked inheritance, autosomal recessive inheritance, or autosomal dominant inheritance.

During the embryonic period, the primitive kidneys include the pronephros, mesonephros, and metanephros. During development, most of the pronephros and mesonephros degenerate one after another. The mesonephric tubules are connected to the pronephric ducts to form Wolff's ducts, and the ureteral buds grow out, posteriorly. It grows in the kidney and induces the formation of metanephros.

The absence of a kidney is generally due to the failure of the mesonephric duct to grow ureteric buds, thus failing to induce the metanephric primordia to differentiate into metanephros. Therefore, the child has no urine production or the produced urine cannot be discharged into the amniotic cavity, resulting in oligohydramnios or even no amniotic fluid, resulting in pulmonary hypoplasia, which is the main cause of neonatal death.

Fetuses with BRA usually die within a short period after delivery. For the fetus to be delivered smoothly, amnioinfusion is usually given after prenatal diagnosis to avoid affecting the amniotic fluid circulation. Intraamniotic infusion therapy uses a puncture needle to inject an appropriate amount of normal saline into the amniotic cavity through the abdomen under the guidance of B-ultrasound to improve the condition of oligohydramnios. It is a safe, economical, and effective method.

Other ways to quickly replenish amniotic fluid include:

Take plenty of fluids

You can increase amniotic fluid by drinking a lot of water. Drinking 2000ml of water within 2 hours can increase the amount of amniotic fluid in a short period, but the amount of amniotic fluid will decrease after some time and cannot last for a long time.

Infusion and oxygenation

When the situation of low amniotic fluid is serious, infusion should be taken immediately to quickly increase the amniotic fluid, and oxygen should be supplemented immediately. This can increase the amount of amniotic fluid and prevent the fetus from being hypoxic due to low amniotic fluid.

Replenish blood

Many pregnant women suffer from insufficient amniotic fluid due to insufficient blood volume in themselves. Insufficient maternal blood volume leads to insufficient blood volume in the fetus, which indirectly affects amniotic fluid circulation and leads to insufficient amniotic fluid.

It is worth noting that a comprehensive evaluation must be carried out before these treatments are carried out, and the treatment must be carried out under close monitoring to prevent the occurrence of allergic reactions, bleeding tendencies, premature birth, miscarriage, infection, and other adverse reactions.

Pay attention to complications, new treatments need to be explored and cracked!

In addition, due to the underdevelopment of the kidneys, their function is damaged, resulting in complications such as an imbalance of water and salt metabolism, reduced blood volume, reduced cardiac output, and hypotension, which require prompt treatment.

In the above case, the child with BRA who had hypotension was successfully treated after using angiotensin II. Tracing back to the origin, when the angiotensin-converting enzyme acts on angiotensin I, endogenous angiotensin II is released, which has a vasoconstrictive effect and can increase MAP. However, it is metabolized in plasma and major organs by nonspecific esterases and has a short half-life (<1 minute).

Altered vascular tone and reduced angiotensin II levels are reasonable in infants with BRA. Generally speaking, patients without kidneys have lower renin levels and lower angiotensin I, which prevents the release of angiotensin II. At present, a large number of studies have shown that angiotensin II is more effective in treating hypotension (including refractory hypotension) in adults and older children.

Previously, in a large study of adults with vasodilatory shock (symptoms including hypotension), nearly 70% of patients were able to achieve MAP >75 mm Hg within 3 hours of angiotensin II treatment, compared with placebo-treated patients. At 23% of patients, fewer serious adverse events were reported in the treatment group. In addition, there are two case reports describing the use of angiotensin II in children.

However, the children in the cases described in this article were infants, and no relevant studies have been reported before. It is unclear when angiotensin II is beneficial in patients with BRA and refractory hypotension. Further research is needed on the synergistic effects of specific drugs such as antidiuretic hormone or hydrocortisone, the optimal time to initiate angiotensin II, methods of monitoring treatment effects, and recommendations for drug discontinuation.

In conclusion, angiotensin II improved well in a critically ill infant with BRA and refractory hypotension. To determine the optimal method, timing, and efficacy of this therapy in infants, further research is needed to explore and verify it. However, it is worth mentioning that the successful treatment of this child provides new enlightenment for the treatment of BRA accompanied by refractory hypotension.

How Does Cistanche Treat Kidney Disease?

Cistanche is a traditional Chinese herbal medicine used for centuries to treat various health conditions, including kidney disease. It is derived from the dried stems of Cistanche deserticola, a plant native to the deserts of China and Mongolia. The main active components of cistanche are phenylethanoid glycosides, echinacoside, and acteoside, which have been found to have beneficial effects on kidney health.

Kidney disease, also known as renal disease, refers to a condition in which the kidneys are not functioning properly. This can result in a buildup of waste products and toxins in the body, leading to various symptoms and complications. Cistanche may help treat kidney disease ase through several mechanisms.

Firstly, cistanche has been found to have diuretic properties, meaning it can increase urine production and help eliminate waste products from the body. This can help relieve the burden on the kidneys and prevent the buildup of toxins. By promoting diuresis, cistanche may also help Reduce high blood pressure, a common complication of kidney disease.

Moreover, cistanche has been shown to have antioxidant effects. Oxidative stress, caused by an imbalance between the production of free radicals and the body's antioxidant defenses, plays a key role in the progression of kidney disease. ies help neutralize free radicals and reduce Oxidative stress, thereby protecting the kidneys from damage. The phenylethanoid glycosides found in cistanche have been particularly effective in scavenging free radicals and inhibiting lipid peroxidation.

Additionally, cistanche has been found to have anti-inflammatory effects. Inflammation is another key factor in the development and progression of kidney disease. Cistanche's anti-inflammatory properties help reduce the production of pro-inflammatory cytokines and inhibit the activation of inflammation mandatory pathways, thus alleviating inflammation in the kidneys.

Furthermore, cistanche has been shown to have immunomodulatory effects. In kidney disease, the immune system can be dysregulated, leading to excessive inflammation and tissue damage. Cistanche helps regulate the immune response by modulating the production and activity of immune cells, such as T cells and macrophages. This immune regulation helps reduce inflammation and prevent further damage to the kidneys.

Moreover, cistanche has been found to improve renal function by promoting the regeneration of renal tubes with cells. Renal tubular epithelial cells play a crucial role in the filtration and reabsorption of waste products and electrolytes. In kidney disease, these cells can be damaged, leading to damaged renal function. Cistanche's ability to promote the regeneration of these cells helps restore proper renal function and improve overall kidney health.

In addition to these direct effects on the kidneys, cistanche has been found to have beneficial effects on other organs and systems in the body. This holistic approach to health is particularly important in kidney disease, as the condition often affects multiple organs and systems. che has been shown to have protective effects on the liver, heart, and blood vessels, which are commonly affected by kidney disease. By promoting the health of these organs, cistanche helps improve overall kidney function and prevent further complications.

In conclusion, cistanche is a traditional Chinese herbal medicine used for centuries to treat kidney disease. Its active components have diuretic, antioxidant, anti-inflammatory, immunomodulatory, and regenerative effects, which help improve renal function and protect the kidneys from further damage. Cistanche benefits other organs and systems, making it a holistic approach to treating kidney disease.