Nephrology
Section 9: Nephrology
9.1 COMMON CONDITIONS
Abdominal Striae Secondary to Steroid Therapy
Figure 9.1.1: Abdominal striae secondary to steroid therapy
Photo Courtesy: Pankaj Deshpande, Mumbai
This twelve years old boy had steroid sensitive nephrotic syndrome for more than 7 years. He had received multiple courses of steroids; the abdominal striae that are an adverse effect of steroids are well seen here.
They can be painful to begin with and eventually leave marks that do not disappear. Occur due to stretching of skin.
Once they occur, there is very little that can be done to make them disappear. Hence, the aim should be to prevent their occurrence.
Use of steroids sparingly and use of other agents to prevent steroid adverse effects is very important.
Vitamin E has been used to improve the appearance of the striae.
Adverse Effects of Steroids on Height
Figure 9.1.2: Adverse effects of steroids on height
Photo Courtesy: Pankaj Deshpande, Mumbai
The boy has nephrotic syndrome and had multiple courses of steroids. The boy is 7 years old and the girl is his sister who was 5 years old when this photo was taken. As can be seen the boy is much shorter than her younger sister though prior to the onset of nephrotic syndrome, his height was on the 10th centile.
Monitor the height regularly on steroids. The height velocity would be more appropriate. Any effect on the height of children in nephrotic syndrome should prompt use of further agents. This boy’s height at this stage was well below the third centile though his height had been on the 10th centile prior to several years of nephrotic syndrome and steroids.
Bilateral Dilatation of the Pelvicalyceal System
Figure 9.1.3: Bilateral dilatation of the pelvicalyceal system
Photo Courtesy: Pankaj Deshpande, Mumbai
Renal ultrasound showing severe bilateral dilatation of the pelvicalyceal system in a 6 months old baby. Note the “Mickey-Mouse appearance”.
Severe dilatation of the
pelvicalyceal system should arouse the suspicion of pelvi-ureteric junction obstruction. A radioisotope scan—MAG3/EC/DTPA will be able to determine the drainage pattern. If the renal function in both kidneys is preserved, conservative management usually is required.
If the renal function in the affected kidney is reduced, surgical
intervention is required to preserve function.
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Delayed Presentation of Renal Tubular Acidosis
Figure 9.1.4: Delayed presentation of renal tubular acidosis
Photo Courtesy: Pankaj Deshpande, Mumbai
Twelve years old untreated patient of renal tubular acidosis. Note the severe stunting of height. Other pictures show the deformities.
Detection has to be done early.
Suspect renal tubular acidosis in patients with failure to thrive. Blood gas and serum electrolytes with normal renal function will provide a diagnosis.
DMSA Scan done at Two Months after Urinary Tract Infection (UTI)
Figure 9.1.5: DMSA scan done at two months after UTI
Photo Courtesy: Pankaj Deshpande, Mumbai
DMSA scan done 2 months after a UTI. Note the reduced uptake in the upper and lower poles of the left kidney. This was reported as scarring.
A DMSA scan is done in UTIs to look for chronic damage. Acute changes on DMSA can last for several months. Hence, a DMSA scan should not be done for at least four months after a UTI. In fact, the later, the better. Ideally, it would be better to do the scan after six months!
DMSA Scan done Six Months after a UTI
Figure 9.1.6: DMSA scan done six months after a UTI
Photo Courtesy: Pankaj Deshpande, Mumbai
The DMSA scan of the same boy as in Figure 9.1.5, repeated after six months. Completely normal with no
‘scarring’!
A DMSA scan is done in UTIs to look for chronic damage. Acute changes on DMSA can last for several months. Hence, a DMSA scan should not be done for at least four months after a UTI. In fact, the later, the better. Ideally, it would be better to do the scan after six months!
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DMSA Scan Showing Dysplastic Left Kidney with Poor Function
Figure 9.1.7: DMSA scan showing dysplastic left kidney with poor function
Photo Courtesy: Pankaj Deshpande, Mumbai
DMSA scan showing the presence of a dysplastic left kidney with poor function. This baby had presented with mild fever 4 months prior to the scan and was diagnosed to have a UTI that was treated. The interesting feature to note is that despite left kidney being dysplastic, the ultrasound scan showed both kidneys to be of equal size.
DMSA scan is used in UTIs to detect chronic damage but the distinction between scarring and dysplasia has to be made on history and clinical features. Normal sized kidneys on ultrasound does not rule out dysplasia. The loss of corticomedullary differentiation is a subtle marker of dysplasia. Long- term monitoring of renal function and proteinuria is mandatory in such cases. Remember they are completely asymptomatic!
Hyperpigmentation of Skin on Fingers due to Cyclophosphamide
Figure 9.1.8: Hyperpigmentation of skin on fingers due to cyclophosphamide
Photo Courtesy: Pankaj Deshpande, Mumbai
This girl had nephrotic syndrome and was frequently relapsing.
Hence, she was given a course of cyclophosphamide. On the therapy with oral cyclophosphamide, patients can develop
hyperpigmentation of the toes and fingers (darkening of skin). This can be seen in the pictures of her fingers and toes. This is a common complaint of the parents that the distal toes and fingers look darker!
Masterly inactivity! No medications are required! The hyperpigmentation disappears completely after the therapy of 12 weeks is completed!
Idiopathic Nephrotic Syndrome on Long-term Steroid Therapy
Figure 9.1.9: Idiopathic nephrotic syndrome on long-term steroid therapy
Photo Courtesy: Pankaj Deshpande, Mumbai
This one and half years old girl had nephrotic syndrome. She had received more than 3 months of daily steroids and was not clearly in remission, hence it is case of steroid resistant nephrotic syndrome. The Cushingoid features with swollen cheeks can be well seen as also the puffiness of the eyelids, indicating edema and nonresolution of the nephrotic syndrome.
Even if edema resolves, ensure that the nephrotic syndrome has resolved by checking a urine protein/creatinine ratio in a spot urine sample. If the ratio is high (normally less than 0.5), it may be steroid resistant nephrotic syndrome. These children need a kidney biopsy and further medications like cyclosporine. This girl had minimal change disease on biopsy and has done very well on cyclosporine.
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MCUG Showing Posterior Urethral Valves and Trabeculated Bladder with Right Grade 5 Reflux
Figure 9.1.12: MCUG showing posterior urethral valve and trabeculated bladder with right grade 5 reflux
Photo Courtesy: Fagun Shah, Surat
This three months old male child presented with recurrent episodes of UTI and poor urinary stream with visibly palpable swelling in suprapubic region. Micturating cystourethrogram shows dilated posterior urethra at bladder neck along with trabeculated bladder and right Grade 5 reflux.
Posterior urethral valves have to be diagnosed early in the newborn period. Antenatal ultrasound scans usually show pelvic dilatation and/
or large bladder. Posterior urethral valves are diagnosed by MCUG and need fulguration. Long-term follow-up for renal function and proteinuria is mandatory and crucial.
MCUG Showing Bilateral Grade 4 Vesicoureteric Reflux
Figure 9.1.11: MCUG showing bilateral grade 4 vesicoureteric reflux
Photo Courtesy: Fagun Shah, Surat
This one year old baby presented with recurrent episodes of urinary tract infections and bilateral hydroureteronephrosis on ultrasound examination.
Micturating cystourethrogram done during infection free period revealed presence of bilateral grade 4 vesicoureteric reflux.
Children with recurrent episodes of UTI, especially below 1 year of age need special care and investigations.
Apart from ultrasound, MCUG is required to diagnose and grade vesicoureteric reflux. Medical and surgical therapy has shown similar outcomes in children diagnosed with vesicoureteric reflux. Attention to local factors to prevent UTI is important along with chemoprophylaxis.
Idiopathic Nephrotic Syndrome with Cushingoid Features
Figure 9.1.10: Idiopathic nephrotic syndrome with Cushingoid features
Photo Courtesy: Pankaj Deshpande, Mumbai
This three and half years old boy had nephrotic syndrome but relapsed frequently. Hence, he had multiple courses of steroids. The cushingoid features are well appreciated here.
The “moon-facies” that come with long courses of large doses of steroids are remarkable. Needless to say, obesity also makes its presence felt.
Though the first episode of nephrotic syndrome may be sensitive to steroids, frequent relapsers (more than 2 relapses in 6 months) will need other medications to avoid steroid toxicity. Moon facies, obesity, risk of infections, osteoporosis, hypertension, abnormal glucose tolerance, stunting of height, cataracts are the few adverse effects to watch out for with steroids!
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Nephrotic Syndrome on Cyclosporine Looking Normal
Figure 9.1.13: Nephrotic syndrome on cyclosporine looking normal
Photo Courtesy: Pankaj Deshpande, Mumbai
This six years old girl had frequently relapsing Nephrotic syndrome.
This picture shows how well she is on cyclosporine. Often, hirsutism is considered as one of the major problems on cyclosporine for girls.
As cyclosporine dose is adjusted properly, there is no evidence of hirsutism in this picture! Needless to say, she has no cushingoid features or steroid side effects.
When used appropriately in the right doses, medications like Cyclosporine do a great job of preventing relapses in nephrotic syndrome without causing the known side effects of hirsutism or gingival hyperplasia. This girl is now off all medications and is doing very well.
Posterior Urethral Valves
Figure 9.1.14: Posterior urethral valves Photo Courtesy: Pankaj Deshpande, Mumbai
Voiding cystourethrogram showing the presence of dilated posterior urethra indicating the presence of minor posterior urethral valves.
This four years old boy presented with occasional episodes of passing urine after a very long duration, sometimes even 12 hours.
Otherwise, he used to void regularly and had a fairly good stream. When he had a long interval between voiding episodes, he would have to strain to void. Minor posterior urethral valves usually do not affect renal function but have to be surgically/endoscopically removed/
fulgurated.
Renal Tubular Acidosis—Severe Deformities of the Lower Limbs
Figure 9.1.15: Renal tubular acidosis—Severe deformities of the lower limbs
Photo Courtesy: Pankaj Deshpande, Mumbai
Severe deformities of the lower limbs as in the similar type of case as mentioned in Figure 9.1.4. As florid rickets has not being treated, there is malleolar widening, severe weakness with the girl being unable to sit and severe osteomalacia on X-ray along with rickets. This picture was taken earlier than the other one and improvement in her clinical status can be seen on treatment as she was able to stand and walk independently.
Normal anion gap—Metabolic acidosis with hypokalemia and hyperchloremia are the basis of diagnosis of renal tubular acidosis (RTA). Rickets in RTA is usually due to acidosis inactivating the vitamin D or uncommonly due to phosphate loss as in Fanconi’s syndrome. To prevent deformity, early detection is must.
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Bladder Diverticulum