Jee Min Park

Effect of renal Doppler ultrasound on the detection of nutcracker syndrome in children with hematuria.

To assess the detection rate of nutcracker syndrome in children with isolated hematuria, renal Doppler ultrasound examinations were routinely performed on 216 consecutive children (176 microscopic hematuria and 40 gross hematuria). Renal Doppler ultrasound was also performed on 32 healthy normal children. The peak velocity (PV) was measured at the hilar portion of the left renal vein (LRV) and at the LRV between the aorta and the superior mesenteric artery. The PV at the aortomesenteric portion (P=0.003) and the PV ratios of the LRV (P=0.003) were significantly higher in children with hematuria than in normal children, while the PV at the hilar portion was not different. If a PV ratio of the LRV of at least 4.1 (the cut-off level set at the mean ±2 SD of the value for the normal children) was defined as abnormal, 72 cases (33.3%) in children with hematuria and no cases in normal children were diagnosed as having nutcracker syndrome. The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal Doppler ultrasound as a screening examination has a substantial effect on the detection of nutcracker syndrome.

Successful use of cyclosporin A in severe Schönlein–Henoch nephritis resistant to both methylprednisolone pulse and azathioprine

Schönlein–Henoch nephritis (SHN) usually presents with micro-haematuria or mild proteinuria but can be associated with heavy proteinuria and nephrotic or acute nephritic syndrome. Although the treatment of SHN is still under debate, it has been suggested that cyclosporin A (CsA) may be beneficial in children with severe SHN, as reported by Ronkainen et al. (Pediatr Nephrol 18:1138–1142, 2003) and Someya et al. (Pediatr Int 46:111–113, 2004). However, histological effects of CsA on severe SHN had not been evaluated in their evaluations. We report the successful use of CsA in a male patient with severe SHN with nephrotic-range proteinuria, who failed to respond to methylprednisolone pulse and azathioprine therapy.

Caroli's Syndrome with Autosomal Recessive Polycystic Kidney Disease in a Two Month Old Infant

Carolis syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Carolis syndrome and ARPKD.

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

Correspondence: Jae Seung Lee, Department of Pediatrics, Yonsei University College of Medicine, 134 Shinchon-Dong, SeodaemunKu, 120 – 752, C.P.O. Box 8044, Seoul, Korea. Email: jsyonse@ yumc.yonsei.ac.kr Received 1 December 2004; revised 2 February 2005; accepted 7 March 2005. IgA nephropathy is the most common chronic glomerular disease worldwide with a prevalence of about 15% in hematuric children. 1 The prevalence of childhood nutcracker syndrome is not known, but it is increasing because of the development of non-invasive diagnostic methods. In this report we describe a patient whose nutcracker syndrome is combined with IgA nephropathy. Such a combination in childhood has not been reported so far.

Catastrophic antiphospholipid syndrome in a 7-year-old girl

Antiphospholipid syndrome (APS) has been recognized as the leading cause of vascular thrombosis in children. The syndrome may occur in isolation or in association with an underlying systemic disease, particularly systemic lupus erythematosus. Less than 1% of patients with APS present with a life-threatening condition resulting from thrombosis in multiple organs and subsequent multiorgan failure, which is defined as catastrophic APS. Early recognition of APS is essential because prompt and appropriate management can result in favorable outcome. We present the case of a 7-year-old girl with APS who presented with cerebral, femoral, and renal involvement in the second week of the disease progress. The patient presented with multiple thrombotic episodes and rapidly progressive renal failure. Renal cortical infarction was diagnosed by magnetic resonance imaging.

Serum IgA/C3 Ratio May Be a Useful Marker of Disease Activity in Severe Henoch-Schönlein Nephritis

Background/Aims: This study was designed to investigate whether the serum IgA/C3 ratio can be a serologic marker of disease activity in children with severe Henoch-Schönlein nephritis (HSN). Methods: Twelve HSN patients who were treated with steroids and cyclosporine were examined. The levels of serum IgA and C3 were measured using an international reference preparation (IFCC/CRM470) and a renal biopsy was performed in all patients before and after therapy. After therapy, patients were divided into 3 groups: complete remission (n = 6, group I), mild urinary abnormalities (n = 3, group II), and active renal disease (n = 3, group III). Results: The serum IgA/C3 ratio decreased significantly in groups I and II after therapy (2.62 ± 0.82 vs. 2.02 ± 0.52, p = 0.02), whereas the ratio in group III increased, although it was not statistically significant (2.13 ± 0.93 vs. 4.67 ± 1.71, p = 0.25). A follow-up renal biopsy revealed that the activity index was reduced in groups I and II (7.0 ± 2.4 vs. 3.6 ± 1.6, p = 0.016), and not changed in group III (7.3 ± 2.1 vs. 9.3 ± 2.5, p = 0.25). The activity index at a follow-up renal biopsy correlated positively with the changes of the serum IgA/C3 ratio: posttherapy activity index = 1.20 × ΔIgA/C3 + 4.78 (r = 0.635, p = 0.027); where ΔIgA/C3 is posttherapy IgA/C3 – pretherapy IgA/C3. Conclusion: These findings suggest that the serum IgA/C3 ratio may be a useful marker to predict disease activity and histologic severity in HSN.

Superimposition of nutcracker syndrome in a hematuric child with idiopathic hypercalciuria and urolithiasis

Abstract  We report a 5‐year‐old girl with idiopathic hypercalciuria who developed gross hematuria and left flank pain despite normalization of calciuria, a renal stone, and microscopic hematuria. She was found to have nutcracker syndrome by renal Doppler ultrasound, which revealed the significant differences of the peak blood flow velocities in the two portions of the left renal vein.

Doppler ultrasonographic detection of nutcracker syndrome in a young child with intussusception: A case report

UNLABELLED We report on a 2-y-old male with intussusception accompanying nutcracker syndrome detected by renal Doppler ultrasound. Renal Doppler ultrasound revealed a significant difference in the peak velocity between the hilar and aortomesenteric portions of the left renal vein. CONCLUSION Renal Doppler ultrasound has been very useful and effective in detecting nutcracker syndrome in a young child.

Morphologically improved nutcracker syndrome in an 11‐year‐old girl with hematuria

A 11-year-old girl was referred to Yonsei University Hospital for microscopic hematuria, which was detected through mass urinary screening. She was asymptomatic, normotensive, and physical examination was normal. She was 155 cm tall (90 – 97th percentile) and weighed 38 kg (50 – 75th percentile). Urinalysis showed only microscopic hematuria (10 – 20 red blood cells/high-power fi eld ). Laboratory data were all noncontributory. i.v. urography showed no abnormal fi ndings. 2-D ultrasonography indicated normal-sized kidneys, but abruptly narrowed LRV between the aorta and the SMA. The anteroposterior (AP) diameters of the hilar and aortomesenteric portions of the LRV were 7.4 and 1.3 mm, respectively, and the ratio of the AP diameters was 5.69 ( Fig. 1a ). Renal Doppler ultrasound was performed while the patient was lying down using an HDI 5000 sonography system with 4 – 6 MHz convex transducers (Philips, Ultrasound, Bothell, WA, USA). Peak velocity (PV) was measured at two points in the LRV, one at the lateral portion of the LRV near the hilum and the other where the LRV courses between the aorta and the SMA (aortomesenteric portion). The peak velocities in the LRV at the hilar and aortomesenteric portions were 12.5 cm/s and 100.1 cm/s, respectively, and the PV ratio of the LRV was 8.01 ( Fig. 1b,c ). Color Doppler demonstrated collateral veins around the LRV ( Fig. 1d ). However, we could not detect dilated left ovarian vein with reversed fl ow or pelvic varicosities with Doppler ultrasound. Based on these fi ndings, the diagnosis of nutcracker syndrome was made. She had been followed in a conservative fashion, that is, urinalysis because there were no symptoms. The hematuria gradually subsided over 14 months and a follow-up Doppler ultrasound was performed from the same position of the ultrasound probe. She was 165 cm tall (90 – 97th percentile) and weighed 44 kg (50th percentile) at this time. The dilated LRV was normalized and the PV at the aortomesenteric portion was decreased at the follow-up ultrasound. The AP diameters of the hilar and aortomesenteric portions of the LRV were 4.0 mm and 1.6 mm, respectively, and the ratio of the AP diameters was 2.50. The peak velocities in the LRV at the hilar and aortomesenteric portions were 23.8 cm/s and 51.8 cm/s, respectively, and the PV ratio of the LRV was 2.18 ( Fig. 2 ). Collateral veins observed at initial Doppler ultrasound disappeared at the follow-up ultrasound.

A five-year-old boy with nephrotic syndrome complicated with acute acalculous cholecystitis.

© 2007 Japan Pediatric Society Nephrotic syndrome is characterized by generalized edema, hypoalbuminemia (<2.5 g/dL), proteinuria (>40 mg/m 2 per h), and hypercholesterolemia (>200 mg/dL). There have been studies reporting that hypoalbuminemia, which is one of the four diagnostic criteria of nephrotic syndrome, is associated with gallbladder (GB) wall thickening. Acute acalculous cholecystitis (AAC) has been found to develop in association with numerous diseases. It should be diagnosed on the basis of history, symptoms, physical examinations, laboratory fi ndings, and wall thickening of the GB by ultrasonography (USG). To date, no studies showing an association between nephrotic syndrome and AAC have been reported. We report a patient with minimal change nephrotic syndrome who presented with acute abdominal pain and vomiting and was diagnosed as having AAC on USG.