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Renal manifestations of tuberous sclerosis among children: an Indian experience and review of the literature.

Korula S, Ekbote A, Kumar N, Danda S, Agarwal I, Chaturvedi S - Clin Kidney J (2014)

Bottom Line: Two children did not have imaging studies available and were excluded from the analysis.One child was found to have proteinuria.We conclude that all children with TSC should be screened for renal involvement and regular follow-up should be arranged.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatrics , Christian Medical College and Hospital , Vellore , Tamil Nadu , India.

ABSTRACT

Objective: The objective of this study was to describe the renal manifestations in children 0-18 years of age diagnosed with tuberous sclerosis complex (TSC) at a tertiary hospital in South India.

Methods: Data of children with TSC, who presented to Christian Medical College Vellore Hospital from January 2008 to January 2013, were analysed by a retrospective chart review. The cases were identified from outpatient records and underwent ultrasonography, urine analysis and examination of serum creatinine to recognize renal involvement.

Results: Twenty-five children with TSC were identified. Two children did not have imaging studies available and were excluded from the analysis. The age of the included children ranged from 5 days to 15 years with a median age of 8 years. Seventy-four per cent (17/23) were males. Ten of the 23 children had evidence of renal involvement (43.5%). Of the 10 children with renal involvement, 6 had angiomyolipoma (60%), 5 had renal cysts (50%) and 1 had suspected renal cell carcinoma. In two children both angiomyolipoma (AML) and cysts were noted. One child was found to have proteinuria. The rest of the children had no evidence of proteinuria and had normal creatinine clearance.

Conclusion: We conclude that all children with TSC should be screened for renal involvement and regular follow-up should be arranged.

No MeSH data available.


Related in: MedlinePlus

Abdominal CT scan (contrast) of patient 9 showing a large lobulated renal mass (12 × 8.3 × 6.4 cms) with few small areas of necrosis, suggestive of a renal cell carcinoma.
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SFT162F2: Abdominal CT scan (contrast) of patient 9 showing a large lobulated renal mass (12 × 8.3 × 6.4 cms) with few small areas of necrosis, suggestive of a renal cell carcinoma.

Mentions: Renal cell carcinoma was suspected in one child (1/10) in our study. This is much higher than the 1–2% quoted in the literature [7]. This could possibly be due to the smaller cohort of patients in our study (Table 2, Patient 9, Figure 2). Her blood pressure and urine exam was normal and she was planned for surgical intervention, however, she was lost to follow-up. RCC also occurs at an earlier age in TSC as compared to RCC in the general population. The average age of diagnosis of RCC in TSC is 28 years although case reports have even reported RCC in an infant [21, 22]. The usual treatment approach is surgical resection for RCC. Studies have shown an overall 5-year survival rate of 52% for all cases of RCC among children and 70% in the non-metastatic group, although there is no clear data regarding this in children with TSC [23].Figure 2:


Renal manifestations of tuberous sclerosis among children: an Indian experience and review of the literature.

Korula S, Ekbote A, Kumar N, Danda S, Agarwal I, Chaturvedi S - Clin Kidney J (2014)

Abdominal CT scan (contrast) of patient 9 showing a large lobulated renal mass (12 × 8.3 × 6.4 cms) with few small areas of necrosis, suggestive of a renal cell carcinoma.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4377768&req=5

SFT162F2: Abdominal CT scan (contrast) of patient 9 showing a large lobulated renal mass (12 × 8.3 × 6.4 cms) with few small areas of necrosis, suggestive of a renal cell carcinoma.
Mentions: Renal cell carcinoma was suspected in one child (1/10) in our study. This is much higher than the 1–2% quoted in the literature [7]. This could possibly be due to the smaller cohort of patients in our study (Table 2, Patient 9, Figure 2). Her blood pressure and urine exam was normal and she was planned for surgical intervention, however, she was lost to follow-up. RCC also occurs at an earlier age in TSC as compared to RCC in the general population. The average age of diagnosis of RCC in TSC is 28 years although case reports have even reported RCC in an infant [21, 22]. The usual treatment approach is surgical resection for RCC. Studies have shown an overall 5-year survival rate of 52% for all cases of RCC among children and 70% in the non-metastatic group, although there is no clear data regarding this in children with TSC [23].Figure 2:

Bottom Line: Two children did not have imaging studies available and were excluded from the analysis.One child was found to have proteinuria.We conclude that all children with TSC should be screened for renal involvement and regular follow-up should be arranged.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatrics , Christian Medical College and Hospital , Vellore , Tamil Nadu , India.

ABSTRACT

Objective: The objective of this study was to describe the renal manifestations in children 0-18 years of age diagnosed with tuberous sclerosis complex (TSC) at a tertiary hospital in South India.

Methods: Data of children with TSC, who presented to Christian Medical College Vellore Hospital from January 2008 to January 2013, were analysed by a retrospective chart review. The cases were identified from outpatient records and underwent ultrasonography, urine analysis and examination of serum creatinine to recognize renal involvement.

Results: Twenty-five children with TSC were identified. Two children did not have imaging studies available and were excluded from the analysis. The age of the included children ranged from 5 days to 15 years with a median age of 8 years. Seventy-four per cent (17/23) were males. Ten of the 23 children had evidence of renal involvement (43.5%). Of the 10 children with renal involvement, 6 had angiomyolipoma (60%), 5 had renal cysts (50%) and 1 had suspected renal cell carcinoma. In two children both angiomyolipoma (AML) and cysts were noted. One child was found to have proteinuria. The rest of the children had no evidence of proteinuria and had normal creatinine clearance.

Conclusion: We conclude that all children with TSC should be screened for renal involvement and regular follow-up should be arranged.

No MeSH data available.


Related in: MedlinePlus