|Year : 2020 | Volume
| Issue : 2 | Page : 84-85
Nephrotic syndrome and intussusception: A coincidence
Manoj Girdhari Matnani1, Mosam K Maroo2
1 Consultant Pediatric Nephrologist, Dr. D.Y. Patil Medical College, Pune, Maharashtra, India
2 Department of Pediatrics, Dr. D.Y. Patil Medical College, Pune, Maharashtra, India
|Date of Submission||08-Sep-2020|
|Date of Decision||06-Oct-2020|
|Date of Acceptance||27-Nov-2020|
|Date of Web Publication||31-Dec-2020|
Manoj Girdhari Matnani
Consultant Pediatric Nephrologist, Dr. D.Y. Patil Medical College, Pimpri, Pune - 411 017, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Matnani MG, Maroo MK. Nephrotic syndrome and intussusception: A coincidence. Asian J Pediatr Nephrol 2020;3:84-5
We present a case of the first episode of nephrotic syndrome that presented to us with unusual clinical features, leading to the diagnosis of intussusception, as an association not described with idiopathic nephrotic syndrome in children.
A 6-year-old boy presented with generalized swelling, low-grade fever, severe abdominal pain, vomiting, and decreased urine output. On examination, he had fever, tachypnea, and tachycardia with normal blood pressure. Abdominal examination revealed periumbilical tenderness and generalized guarding, without any palpable mass. Based on a diagnosis of acute abdomen, following initial stabilization, an ultrasonography of the abdomen was performed which revealed a right-sided target-like lesion in the small bowel, indicating ileocolic intussusception [Figure 1]. Based on serum and urinary biochemistry, a diagnosis of nephrotic syndrome was also made. The abdominal symptoms were transient and resolved subsequently with conservative management; he started passing stools and vomiting subsided, before radiological reduction of intussusception could be conducted. Repeat serial ultrasonography revealed resolving ileoileal intussusception. Given the atypical presentation, we also performed a kidney biopsy which confirmed minimal change disease. Once the abdominal symptoms abated completely, the child was initiated on therapy with prednisolone at 2 mg/kg daily which led to complete remission of proteinuria.
|Figure 1: The classical target sign of the small bowel loop on USG of the abdomen|
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Reports of intussusception at the first presentation with steroid-sensitive nephrotic syndrome are rare. A report in 1975 described a 19-year-old boy with steroid-resistant nephrotic syndrome who was detected to have ileocolic intussusception on postmortem autopsy. A 6-month-old English girl with diffuse mesangial sclerosis had first presented with intussusception that was refractory to reduction by barium enema and was reduced surgically. A 4-year-old girl presented with ileocolic intussusception, and a 2-year-old boy developed ileoileal intussusception during the first episode of minimal change nephrotic syndrome,, while a 5-year-old boy with IgM nephropathy was diagnosed with this complication during a relapse. Similarly, this complication was reported in a boy with steroid-dependent nephrotic syndrome and biopsy-proven minimal change disease who required open surgical reduction and in another boy with congenital nephrotic syndrome with diffuse mesangial sclerosis who improved following pneumatic reduction. In contrast, the index patient responded to conservative management with spontaneous resolution of the intussusception.
The classic triad of intussusception (i.e), abdominal pain, vomiting, and currant jelly-like stools were lacking in our patient. The full-blown picture of intussusception might have been masked by presentation with abdominal pain early in the course, or mild intussusception which resolved spontaneously, before currant jelly stools were formed. While the index case is not novel, it highlights a rare complication of nephrotic syndrome, wherein intussusception likely developed due to bowel wall edema, as a part of anasarca, related to hypoalbuminemia similar to anasarca. Unlikely causes include mesenteric lymphadenopathy or submucosal lymphoid hyperplasia, as part of enteritis or peritonitis, that might act as a lead point for intussusception. Rarely, lymphomas and/or paraneoplastic nephrotic syndrome may present with similar symptoms. A kidney biopsy that was performed to rule out secondary causes such as IgA nephropathy, could have probably been avoided.
The key learning point from the case is that a high index of suspicion for intussusception is necessary in a patient with nephrotic syndrome presenting with severe abdominal symptoms. While this complication is quite rare as compared to the more usual etiologies of acute abdomen in relapse state of nephrotic syndrome, namely mesenteric ischemia and spontaneous bacterial peritonitis, a careful ultrasonography is useful in patients with refractory symptoms.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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