Last week we learnt the difference between a Syndrome and a Disease.
Our hyperactive, skinny four-year-old son, Aki, had just recovered from a cough & cold, when he suddenly started looking a lot healthier. His cheeks filled out and his clothes started getting tighter. We were rather pleased at first -- until we realized that he had been putting on about 300g everyday without any significant change in his diet.
Our pediatrician recommended three tests (blood, urine and chest x-ray), and told us to immediately cut down on Aki's salt and fat consumption while increasing his intake of proteins - especially egg whites. The tests soon confirmed what he suspected: loss of protein in urine (proteinuria), retention of water within the body (edema), high cholesterol and creatine levels -- a classic case of Nephrotic Syndrome (NS).
A syndrome is a set of symptoms or conditions that suggest the presence of a disease. In the case of Nephrotic Syndrome, all the symptoms do not even point to a disease but merely signal the onset of a sudden change in the functioning of one set of cells within our kidneys, the nephrons.
The nephrons contain two parts - a wool-ball shaped Glomerulus, and a long, coiled Tubule. Blood is filtered by the glomerulus and the filterate goes down the tubule which takes back useful stuff and excretes the harmful ones as urine. Under NS, the glomeruli - quite inexplicably - start removing protein molecules from the blood. This is unusual, not only because proteins are usually large, heavy molecules, but due to the fact that proteins are rarely wasted; the body needs them build and repair tissues.
Aki's body had swelled up from 14kg to 18.5kg in less than ten days. So the doctor prescribed diuretics to reduce accumulated body fluid, and, rather disconcertingly, an extended three-month dosage of steroids. Sensing our alarm, the doctor said that we had two options: Prednisolone, a corticosteroid that had been tried & tested for the past 35 years. Possible side-effects included stomach pain, high BP, change in body appearance, increased hair and retardation of growth. The alternative was to go for a more recently developed drug with possibly milder side-effects.
So the Rx was:
- Deflazacort - Defcort 18mg (twice a day * 7 days) - Glucocorticoid
- Lasix 20mg (once a day * 2 days) - Diuretic
- Multivitamins, calcium supplements, antacids
Two days later, when the swelling refused to subside, we went for a second-opinion to a specialist Pediatric Nephrologist at Apollo Hospital. The elderly doctor felt that the diagnosis was spot-on but the prescription had to be changed. He handed us a useful booklet titled "Nephrotic Syndrome in Children", and reverted to the tried-and-tested protocol:
- Prednisolone (Omnacortil / Wysolone - 20mg morning; 15mg evening) - Glucocorticoid
- Zylynix one 2.5mg dose followed by Lasix 40mg (once day * 3 days)
- Continuation of the multivitamins (Zincovit), calcium (Ostocalcium) and antacid (Digene)
The effects were immediate. Almost overnight, Aki's body-weight came down from 18.5kg to 16kg. The side effects too were just as we were warned, especially the stomach aches, the tendency to be "excessively happy, quiet or abnormally active".
We are now one week down with the drugs, with five more to go...
- How was NS treated before the discovery of steroids?
- Why are cortocosteroid tablets so cheap? (not that I'm complaining!) - The new corticosteroid, Defcort-18mg cost Rs.19/tab while the older formulation costs just few paise!
- How do the steroids plug the sieves in the Glomeruli? What else does it do within the body to other organs?
- What makes the tablets so bitter?
LINKS & REFERENCES:
* Srivastava, RN (1975): Nephrotic Syndrome in Indian Children, Archives of Disease in Childhood, 1975 - url - http://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC1545499&blobtype=pdf
* Comparing Glucocorticoids: Prednisolone vs. Deflazacort - http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2763756/