Nephrotic Syndrome

Kidneys receive approximately 1 liter of blood every minute. Of this, about 100 ml gets filtered in the kidney. This contains, small molecules and few large molecules. This is due to the intricate cellular structure that prevents proteins from getting filtered into the urinary space. A derangement of this filter results in passage of heavy amounts of proteins in urine. This condition is called heavy proteinuria or Nephrotic syndrome or Nephrosis.

Definition:

In the urine, the protein excretion is > 3.5 gm/day or in children, it is >50mg/kg of body weight. In most cases, the serum albumin is <3gm/dl (normal>4), and passage of lipids in the urine. The lipids in the blood increase and swelling of face and feet also occur.

Causes of Nephrotic Syndrome:

In children, most cases are due to Minimal Change Disease, FSGS, MPGN etc. In adults, most cases are due to primary illness while upto 30% may be due to secondary illness.

Clinical Features:

Apart from edema, there is a tendency towards clotting of the blood in blood vessels. If this involves a critical area, like heart, brain or kidneys complications may occur. Infections also are common. In addition, kidney function may be reduced due to either the disease or its treatment. Sometime the blood pressure may be elevated.

In some cases, continuation of excess proteins in the urine may lead to glomerular damage and Chronic kidney disease leading to renal failure.

Diagnosis:

After confirmation of excess protein in the urine, a kidney biopsy is required in most cases. In children between 2 to 8 years, a biopsy may be deferred if there is a good response to steroids as Minimal Change disease is presumed.

Treatment:

The treatment consists of steroids in primary cases and in case of poor response other immunosuppressants and ACE-Inhibitors. Disease often has remissions and relapses which need to be monitored and treated appropriately.

Diabetes Care

For patients suffering from type II /adult onset diabetes :

In type II diabetes, diagnosis is often late (after the disease has already been present for some time). In early days T2DM (short for the adult type of diabetes) are often without symptoms. Hence the delay in diagnosis. This means complications will be seen early after diagnosis.

Foot Care :

Foot disease in diabetes may lead to difficulty in walking, pain and may lead to infections. If neglected amputation may be required.

Inspect feet with a mirror before going to sleep every night.

Use well fitting shoewear.

Do not walk bare feet even in the house.

Socks should be clean and correct size.

In case of deformities of feet, special shoes can be worn.

Eye Care :

It is nowadays the commonest cause of blindness throughout the world.

Check for refractory error at the time of diagnosis and once sugars are controlled.  In T2DM every year examination for changes in retina are required. If there are already changes, check up is required more often.

Retina is checked after dilating pupil with an ophthalmoscope.

Kidneys :

It is one of the common causes of kidney failure leading to requirement of dialysis and renal transplant. Early treatment may reverse diabetic kidney disease or slow its progression.

In early cases urine is checked for passage of extra amounts of albumin. This can be quantified to see response to therapy. This test is called urine for microalbuminuria, and urine albumin /creatinine ratio. If the disease is already present, USG, S Cr and testing for voiding function (Uroflowmetry) etc may be useful.

In those without evidence of kidney disease these tests are carried out every year. Frequency otherwise may depend on the stage of involvement. More severe involvement necessitates more frequent reviews.

Nerves :

Nerve involvement is checked by touch, pain, vibration sense.

During winters, hot water should be checked with either a thermometer or elbows. If fingers are used for checking temperature, very hot water may lead to burns.

Precautions should be taken to avoid falls.

Sugar Levels :

HbA1C level may be done every 3 months to monitor overall sugar control in blood. In most cases a level of < 7 is desirable.

If hypoglycaemia is frequent , a level of < 8 may be  all that can be achieved safely. In cases of some microvascular complications, a level < 6.5 may help but is difficult to achieve. Home sugar monitoring is done from capillary blood. The levels are different from venous blood levels tested in laboratories. Only sides of fingers should be used and not the areas used for holding functions of the hand. In T2DM once weekly sugars may be enough. In T1DM almost daily or before each meal sugars are required. Levels for control should not be done within 2 hours of meals. Check if there is uncertainty about diagnosis of hypoglycaemia. Do not wait for giving sugars, if testing takes time or symptoms are severe (loss of consciousness, confused talk, fits etc.) Treatment presumptively may prevent permanent damage to the brain. However sugar is not the treatment in diabetics for every small symptom. Record times of sugar checking and dates in a notebook for planning of drugs. Lipids and Statins : If there are more cardiovascular risk factors, age > 50 yrs in man, smoking, lower levels of LDL cholesterol are desirable. Daily 75 or 150 mg  of aspirin or statins may decrease risk of heart attacks. Bleeding complications however increase with aspirin.

Regular exercise, monitoring weight, smoking cessation all are essential parts of diabetic care.

Stone Disease of the Kidneys

Stone Disease of the Kidneys.

Stone formation in the kidneys is a common disease. About 1in 8 men and 1 in 20 women suffer from the symptomatic stone disease. Stone formation without symptoms is even more common.

About ¾ of the stones contain calcium and 10% have uric acid. Rest are due to combinations and rare diseases.

Calcium Stones

These contain calcium oxalate or calcium phosphate. Former is more common.

The risk factors for stone formation are

In Urine 

Low volume , High calcium in urine, high acid concentration and low citrate levels.

In Diet

Water and fluid intake is low, fruits are seldom eaten, food has high oxalate level or if calcium content of the diet is on the lower side.

Other diseases with high risk for stone formation are overweight, gout, diabetes, recurrent urinary tract infection etc. Bariatric surgery is a very significant risk factor.

If kidneys have certain diseases like the inability to excrete acid, a stone formation may occur.

Symptoms: Stones if they are in the urinary path (ureters or urethra) pain colicky in nature often radiating from back to lower abdomen or upto urine passage opening (urethra) may occur. This pain is at times very severe necessitating urgent consultation.

Urine may be passed in drops and is often red in colour due to blood.

If the passage of urine is blocked due to stone at any site, high pressure develops upstream of the blockage. If not treated early this may permanently damage the kidney.

In India, an untreated stone disease is a common cause of permanent renal failure.

Stone analysis, various urine tests aid in finding the underlying cause of stone formation. A cause is found in about ½ of the cases.

Prevention

Stone disease can usually be prevented by adequate water intake, fruits or fruit product ingestion, enough calcium and lower salt in the diet and early complete treatment of urinary tract infection.

Eating lower oxalates in the diet is of questionable value.

Small stones pass spontaneously with more fluid ingestion and alpha blockers. Large stones need to be removed by surgery.

Polycystic Kidney Disease

Polycystic Kidney Disease

Introduction and Genetics:

This is one of the commonest genetic disorders affecting kidneys. The disease runs in families. The reported incidence is 1 in 400 to 1in 1000. Both man and woman are affected. If one parent is affected the children of the patient have 50% chance of inheriting the disease.

One-quarter of patients may not provide a history of the disease in parents or siblings because of death prior to diagnosis, an undiagnosed disease in the other members or insufficient investigation of the other family members.

The disease can be found by genetic testing in the fetus. However as the disease has normal mentation and causes problems in adult life, genetic testing in a fetus are rarely carried out.

The disease is usually of two types. 85% suffer from an abnormality of Chromosome 16 and rest from Chromosome no 4. Type PKD 1 (ch 16 variety) is the more severe form. It causes renal failure at about 55 Yrs of age while Type PKD II at 70 Yrs of age.

Those patients with hypertension, males, with larger kidney size develop renal failure early.

There are hundred to thousands of cysts distributed in both kidneys in all areas. Liver and pancreas, and sometimes lungs may also have cysts. A fewhave intracranial aneurysms( dilatation of blood vessels). Cardiac valvular defects are also common. Affected kidneys may have a stone formation or these can be infected.

Patients may have initial symptoms of abdominal pain due to large kidneys, high blood pressure or urinary tract infections. USG done for unrelated symptoms sometimes reveals the diagnosis.

In affected families, the  no of cysts at various age groups helps in ascertaining the probability of disease. It has to be distinguished in initial cases (probands) where family history is not available, from other cystic diseases of the kidneys.

Treatment includes control of high blood pressure, statins, lower protein intake and general measures. Patients may waste salt in their urine and may suffer from low sodium levels. ACE inhibitors and ARBs are often used for the control of BP while care is taken to look for their side effects and safety.

A vasopressin inhibitor has been used increasingly for slowing/ Stopping the disease progression in ADPKD patients. It is more useful if started early before the s cr is very high.

Death is more often due to cardiac or strokes rather than kidney failure.

Drugs after Renal Transplant: II

Drugs after Renal Transplant: II

There are a number of other drugs used in transplant recipients after they are discharged from the hospital.

In the initial period these include:

Antibiotics to prevent urinary tract infections. These may need to be continued for 6 months. These (Trimethoprim + Sulfas) may prevent pneumocystis infection of the lungs as well.

A common infection in transplant recipients is due to cytomegalovirus. This can be prevented by a drug called Valgancyclovir. It is usually given for 90 – 100 days. It is a costly drug and the total treatment may cost about 25000 to 45000 ₹ depending on the dose and duration of CMV prophylaxis.

Anti Hypertensive drugs, sugar lowering drugs may be required in cases of Hypertensives and diabetics.