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Biomedical subjects

Jae Il Shin

Publications and source records attributed to Jae Il Shin.

At least 19 recordsLinked to original sources

Doppler ultrasonographic indices in diagnosing nutcracker syndrome in children.

To elucidate the Doppler ultrasonographic cut-off value of nutcracker syndrome causing hematuria in children, we analyzed Doppler spectral findings between 15 children with nutcracker syndrome and 15 age- and sex-matched normal control subjects. A follow-up Doppler ultrasound (US) was also performed in children with nutcracker syndrome when hematuria subsided completely after a median period of 1.7 years (range: 1.0-3.5 years) (relieved nutcracker syndrome). The peak velocity (PV) ratios of the left renal vein (LRV) were significantly higher in children with nutcracker syndrome than in those with relieved nutcracker syndrome (P<0.0001) and normal children (P<0.0001). The PV ratios of the LRV at the follow-up US were significantly higher than those in the control subjects (P=0.019). None of the 15 normal children showed PV ratios of the LRV>3.7, but five of the 15 children with relieved nutcracker syndrome without hematuria had PV ratios of 3.91-5.02. When we set the cut-off values for nutcracker syndrome at the mean+/-2 SD (mean: 2.95+/-0.92, range: 1.60-5.02) of 30 controls (normal children and relieved nutcracker without hematuria), the calculated cut-off value was 4.8, and the sensitivity and specificity were 100% and 93%, respectively. Given its high sensitivity, renal Doppler US can be used as a useful initial non-invasive test in the diagnosis of nutcracker syndrome in children with hematuria.

Adolescent↗

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.

Adolescent↗

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.

Acute Kidney Injury↗

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.

Azathioprine↗

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

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.

Calcium Metabolism Disorders↗

Factors affecting histological regression of crescentic Henoch-Schönlein nephritis in children.

To identify the factors affecting histological regression of crescentic Henoch-Schönlein nephritis (HSN), we retrospectively analyzed serially biopsied 20 children with crescentic HSN treated with immunosuppressants. They were classified into two groups according to the histological changes between the first and second biopsy: group I (n=10) with histological regression and group II (n=10) with no change or histological progression. Of the 20 patients, 19 showed a favorable outcome at the end of follow-up. Initial laboratory and histological findings did not differ between the two groups. Histological regression was associated with a younger age at onset (P=0.003), early treatment with immunosuppressants (P=0.044) and absent or decreased fibrinogen deposits at the second biopsy (P<0.0001) in a univariate analysis. Mesangial IgA and fibrinogen depositions at the second biopsy were reduced significantly in group I (P<0.05). In the multivariate analysis, a younger age was an independent determinant of histological regression (OR 1.44; 95% CI 1.03-2.02). The intensity of fibrinogen deposits at the second biopsy correlated positively with the age at onset (r=0.503, P=0.024), and the chronicity index at the second biopsy correlated positively with the time that immunosuppressive therapy was started (r=0.619, P=0.004).

Biopsy↗

Cyclosporin A therapy for severe Henoch-Schönlein nephritis with nephrotic syndrome.

To evaluate the efficacy of cyclosporin A (CyA) for treating severe Henoch-Schönlein nephritis (HSN), seven patients with nephrotic syndrome, aged 3.9-13.8 years (mean 6.5 years), were analyzed retrospectively. Mean follow-up times were 5.5 years (range 2-9 years). All underwent renal biopsy before treatment, and follow-up renal biopsy was performed in six of the seven patients. All patients improved, with 24-h protein declining from a mean of 9.2 g/m(2)/day (range 1.5-16 g/m(2)/day) to 0.3 g/m(2)/day (range 0.03-1.2 g/m(2)/day) (p=0.016) and serum albumin increasing from a mean of 2.1 g/dl (range 1.5-2.4 g/dl) to 4.6 g/dl (range 3.5-5.3 g/dl) (p=0.016) after CyA therapy. The activity index decreased significantly at the second renal biopsies obtained at a mean interval of 11.7 months after the first (6.4+/-3.3 vs 3.5+/-1.2, p=0.042, respectively), while the chronicity index and the tubulointerstitial scores did not change. On the immunofluorescent findings at the second biopsies, the degree of deposits of immunoglobulins such as IgA, IgM, C3, and fibrinogen decreased in five of the six patients. Although this case series is without controls, our study suggests that CyA may be beneficial to a subset of HSN patients with nephrotic syndrome.

Adolescent↗

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 x DeltaIgA/C3 + 4.78 (r = 0.635, p = 0.027); where DeltaIgA/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.

Adolescent↗