Saudi Journal of Kidney Diseases and Transplantation

LETTER TO THE EDITOR
Year
: 2016  |  Volume : 27  |  Issue : 1  |  Page : 177--178

Nephrotic syndrome and situs inversus - A chance association?


Vaishali B More, Amit Saxena, Ratna Sharma 
 201, M-5, C-Wing, Palm Acres CHS, Pratiksha Nagar, Sion, Mumbai - 400 022, India

Correspondence Address:
Dr. Vaishali B More
201, M-5, C-Wing, Palm Acres CHS, Pratiksha Nagar, Sion, Mumbai - 400 022
India




How to cite this article:
More VB, Saxena A, Sharma R. Nephrotic syndrome and situs inversus - A chance association?.Saudi J Kidney Dis Transpl 2016;27:177-178


How to cite this URL:
More VB, Saxena A, Sharma R. Nephrotic syndrome and situs inversus - A chance association?. Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2019 Dec 9 ];27:177-178
Available from: http://www.sjkdt.org/text.asp?2016/27/1/177/174215


Full Text

To the Editor,

A 3-year-old girl was brought with complaints of generalized edema and decreased urine output. She had a history of similar complaints more than six months earlier for which she had responded to the treatment given by a local pediatrician. Her vital parameters were normal. She complained of generalized edema and abdominal distension and her heart sounds were better heard on the right side. No murmur was heard. The liver was just palpable on the left and the spleen was not palpable. Her urine albumin to creatinine ratio was more than 0.2 g. The urine albumin on fresh morning sample was 4+ and she had high serum cholesterol of 345 mg/dL. Her chest X-ray confirmed the presence of dextrocardia [Figure 1]. A 2D-echo revealed dextrocardia with a normal heart and ultrasound of the abdomen revealed situs inversus with an anatomically normal spleen.{Figure 1}

After ruling out tuberculosis and hepatitis B, the patient was started on steroids in the form of oral prednisolone at a dose of 60 mg/m 2 / day. After seven days of treatment, the patient went into remission and the steroids were continued for six weeks, followed by alternateday steroids for another six weeks. The patient is on regular follow-up in our nephrology outpatient department and remains in remission.

Dextrocardia with situs inversus is rarely reported with nephrotic syndrome, although various other congenital malformations have been reported. We found a report of dextrocardia with focal and segmental glomerulosclerosis (FSGS) reported in an adult patient with nephrotic syndrome. [1] But, beyond this, there are no reports of nephrotic syndrome being associated with dextrocardia and situs inversus.

Situs inversus is a rare condition and is associated with many other congenital malformations. [1] The other congenital anomalies associated with nephrotic syndrome are hydronephrosis and hydroureters, which are also seen in FSGS. A kidney biopsy has not been performed in our patient as she is an infrequent relapser and hence is more likely to have minimal change disease. Transforming growth factor-beta superfamily molecules have been said to play a major role by affecting the cell by signaling through type I and II serine/ threonine kinase receptors. They are said to be responsible for both nephritic syndrome and situs inversus totalis in an 18-year-old adult female who was steroid sensitive, like our patient. [2]

Conflict of Interest: None declared.

References

1Vikrant S, Kumar S, Raina R, Sharma A. Nephrotic syndrome in a patient with situs inversus totalis. Clin Exp Nephrol 2008;12: 215-8.
2Domanski M, Domanski L, Ciechanowski K. Nephrotic syndrome and situs inversus viscerum: Correlation or coincidence. J Nephrol 2005;18: 623-5.