Dr. Sonia Sharma

KIDNEY OVERVIEW

 

The kidneys are bean-shaped organs, located just below the rib cage on each side. Every day, the kidneys filter about 120 to 180 L of water with the normal Glomerular filtration rate (GFR) above 90 ml/min/1.73 m2 . Normal urine output range from 0.5ml/kg/hr to 3 ml/kg/hr (400 ml/day to ≤2 litre/day), which is 1% of total blood filter per day (reabsorb proteins, maintaining electrolyte balance) in order to excrete solutes composed of wastes and extra fluid. Children produce less urine than adults and the amount produced depends on their age.

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Other than water balance, kidneys also maintain electrolytes and acid- base balance in the body. It produce hormone erythropoietin, responsible for maintain hemoglobin (deficiency leads to Anemia), active Vitamin D for maintaining stable calcium & phosphorous levels (healthy bones).

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The urinary tract is made up of the kidneys, ureters, bladder and urethra. Ureters are the thin tubes of muscles on each side of the bladder and carry urine from each of the kidneys to the bladder. The bladder stores urine until person urinates through urethra.

Any anatomical or physiological defect in urinary tract can affect children in various ways, ranging from treatable disorders without long-term consequences to life-threatening conditions. Some children are born with a condition that causes the urethra to narrow or become blocked. This prevents urine from leaving the body. Surgery is usually done to clear the blockage. Long-term treatment may include medication and additional surgery.

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NEPHROTIC SYNDROME

                                                                        

Appearance of sudden onset swelling over face, eyes and then to whole body with loss of protein in the urine suggest onset of Nephrotic syndrome.

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It can appear with or without upper respiratory tract infection symptoms. Diagnosis needs confirmation with the persistence of low albumin and high cholesterol levels in the blood investigations and documentation of high urinary protein to creatinine ratio of more than 2 or if any doubt then more than a gram of urine protein in 24-hour urine sample.

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Disease is very well known with the incidence of 3-4/100,000 children. Although commonest affected age group is 3-4 year but we also often see kids beyond this age group. Age of onset also determine the severity and response to treatment. If it presents in infant of less than three months of age (congenital nephrotic syndrome) then no treatment is advised other than support with albumin transfusion & diuretic treatment as and when required. Congenital nephrotic syndrome is often associated with genetic mutation. For Nephrotic syndrome, beyond this age group, oral steroids are the main stay of treatment, which are prescribed after documenting no hidden underlying infection.

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Parental support and counseling is required for the need to understand the importance of adequate dose, duration and side effect of steroid therapy. Child need to take prescribed medicine regularly as per the treatment advised. Also need to maintain a diary for regular urine protein monitoring at home. Now days, uristix are available to measure urine protein at home easily. Parents should learn it properly and should monitor their child urine protein. This will help in taking timely action for urine protein and avoiding hospitalization for swelling.

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Most parents understand this and do regular monitoring at home

Nephrotic syndrome course can be steroid sensitive or steroid resistance.

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Steroid sensitive course generally do well. Recurrence of swelling is common which can be infrequent or frequent and with or without any episode of infection. These are known as relapses. Frequent relapses need treatment with other immunosuppressive therapies. Regular follow-up is required for monitoring all clinical and laboratory parameters.

 

For steroid resistance course, kidney biopsy is required to know the underlying cause of nephrotic syndrome and initiation of calcineurin inhibitor therapy. It is a safe procedure and done by pediatric nephrologist in children.

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Treatment should be given under doctor care in children as dosage depends on weight. Inadequate dosage as well as extra dosage can cause harm to child. Steroids side effects include, cushingoid facies, hypertension, obesity and short stature etc. Child on immunosuppressive therapies is prone to infection so it is advised to take precautions.

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Vaccinations for such children need consultation as all live vaccines are contraindicated here

during active episodes. Oral OPV (pulse polio program), BCG, measles, MMR vaccine are examples of such vaccines.  Lastly concluding that disease not only needs adequate treatment but also lot of patience and understanding by parents.