Nutcracker syndrome combined with IgA nephropathy in a child with recurrent hematuria

Shin, Jae Il; Park, Jee Min; Shin, Youn Ho; Lee, Jae Seung; Kim, Myung Joon; Jeong, Hyeon Joo

doi:10.1111/j.1442-200x.2006.02212.x

Cited by 18 publications

(8 citation statements)

References 8 publications

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“…Therefore, the terminology NCS should be restricted to patients with typical clinical symptoms and signs, especially hematuria, low back pain, proteinuria, male varicocele, and female pelvic congestion (3). NCS is an important diagnosis of unexplained hematuria due to the lack of clear diagnostic procedures and standards and the possibility of the coexistence of other glomerular diseases (5)(6)(7)(8), and its diagnosis is often delayed. The diagnosis of NCS is mainly based on imaging examinations of patients with typical symptoms and signs, including US, CTA, magnetic resonance angiography (MRA), and intravascular ultrasound (IVUS) (9).…”

Section: Discussionmentioning

confidence: 99%

Case Report: An Unusual Case of Nutcracker Syndrome and Literature Review

Feng

Shen

et al. 2022

Front. Urol.

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BackgroundHematuria is one of the common complaints of patients with kidney disease and often occurs concurrently with proteinuria. Hematuria caused by nutcracker syndrome (NCS) is relatively common, while hematuria caused by type I von Willebrand disease (VWD) is less common.Case PresentationA 12-year-old boy was admitted to our ward due to sudden gross hematuria that had lasted for 4 days. After admission, his gross hematuria lasted for 24 days without remission and was combined with nephrotic-range proteinuria. A series of blood biochemical and urine tests, as well as renal ultrasound, lower abdominal CT angiography, cystoscopy, kidney biopsy, and genetic testing, were completed, and he was eventually diagnosed with NCS combined with type 1 VWD. After nutritional support, oral angiotensin-converting enzyme inhibitor (ACEI), and plasma infusion treatment, gross hematuria and microhematuria disappeared, proteinuria turned negative, and there was no recurrence during the 10 months of follow-up.ConclusionsNCS and/or VWD may exist in patients with hematuria, and bleeding disorders such as VWD should generally be on the list of suspected diagnoses in patients with hematuria.

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Section: Discussionmentioning

confidence: 99%

Case Report: An Unusual Case of Nutcracker Syndrome and Literature Review

Feng

Shen

et al. 2022

Front. Urol.

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“…Some researchers have proposed that nutcracker should be defined only when the clinical symptoms are present along with compatible radiologic findings, [4] because asymptomatic dilatation of LRV frequently shown on ultrasound or computed tomography has been regarded as a finding of a normal variant [5] . However, LRV entrapment has been reported to coexist with some idiopathic glomerular disease in some case reports, among which coexistence with IgAN were the most commonly reported ones [6–11] . The combination increases the difficulty of differentiating the primary cause of renal manifestations and influences the choice of therapy.…”

Section: Introductionmentioning

confidence: 99%

“…[5] However, LRV entrapment has been reported to coexist with some idiopathic glomerular disease in some case reports, among which coexistence with IgAN were the most commonly reported ones. [6][7][8][9][10][11] The combination increases the difficulty of differentiating the primary cause of renal manifestations and influences the choice of therapy.…”

Section: Introductionmentioning

confidence: 99%

Coexisting nutcracker phenomenon and superior mesenteric artery syndrome in a patient with IgA nephropathy

Wang

Zhao

et al. 2021

Medicine

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Rationale: Nutcracker and superior mesenteric artery (SMA) syndrome share the same pathogenesis, but the simultaneous occurrence of both diseases is quite rare. A combination of the nutcracker syndrome and IgA nephropathy has previously been reported. Herein, we report what we believe is the first case of coexisting nutcracker and SMA syndrome in a patient with IgA nephropathy. Patient concerns: A 15-year-old Chinese boy who was diagnosed with IgA nephropathy at 8 years of age presented with gross hematuria, fatigue, anorexia, nausea, and recurrent abdominal distension for 1 week without any obvious evidence of preceding infection. Laboratory data showed macroscopic hematuria, heavy proteinuria, and relatively normal renal function. Doppler ultrasonography and upper gastrointestinal gastrografin study were performed, respectively. Since his renal function deteriorated after admission, repeated renal biopsy was performed. Diagnoses: IgA nephropathy with nutcracker phenomenon and SMA syndrome. Intervention: Immunosuppressive therapy combined with conservative therapy for superior mesenteric artery syndrome. Outcomes: One month later, his abdomen symptoms such as anorexia and abdominal distension eased a lot with body weight increase of about 3 kg. After 6 months of follow-up, his body weight increased to 57 kg, serum creatinine decreased to 63 μmol/L, and urine microscopy showed 75.5 RBC/high-power field with 0.3 g urine protein per day. Lessons: Although the association between vascular compression and IgA nephropathy (IgAN) has not been elucidated yet, combination of nutcracker syndrome and SMA syndrome should be considered in patients with IgAN. The combination may increase the complexity of the disease, and renal biopsy should not be hesitated for differential diagnosis.

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“…A 12-year-old German girl with microscopic haematuria was found to have left renal vein entrapment and mesangial deposits of IgA [ 1 ]. Similarly, left renal vein entrapment and mesangial deposits of IgA were reported in a 9-year-old Korean girl with isolated microscopic haematuria and recurrent gross haematuria [ 2 ] and in a 25-year old Taiwanese woman with recurrent gross haematuria and left flank pain [ 3 ]. Left renal vein entrapment and IgA nephropathy were also disclosed in a 20-year-old woman and in a 22-year-old Japanese man, who, in the context of a pharyngitis, developed gross haematuria and a tendency to microscopic haematuria with persisting proteinuria [ 4 ].…”

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confidence: 96%