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Renal-hepatic-pancreatic dysplasia syndrome (Ivemark's syndrome).

Vankalakunti M, Gupta K, Kakkar N, Das A - Diagn Pathol (2007)

Bottom Line: Both cases are unrelated and there is no history of any consanguineous marriage.These two cases are unrelated and are rare.In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. vkmahesh123@yahoo.co.in

ABSTRACT

Background: Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.

Case presentation: We describe two unrelated cases of Renal-Hepatic-Pancreatic dysplasia syndrome in stillborn babies. The characteristic microscopic features were present in both the cases. The second case illustrates the unique association lymphangiectasia with Renal-Hepatic-Pancreatic dysplasia syndrome. Both cases are unrelated and there is no history of any consanguineous marriage.

Conclusion: These two cases are unrelated and are rare. In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.

No MeSH data available.


Related in: MedlinePlus

Photomicrography of pancreas shows widely spaced ducts lined by undifferentiated cells with loss of acini (H&E ×100). Inset showing islet cells (H&E ×400).
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Figure 4: Photomicrography of pancreas shows widely spaced ducts lined by undifferentiated cells with loss of acini (H&E ×100). Inset showing islet cells (H&E ×400).

Mentions: At autopsy, the baby weighed 1.4 Kg with crown-to-heel length of 39 cm, foot length of 6 cm. There was congenital talipes equino-varus deformity. Serous effusions were noticed. Kidneys were normal sized and showed multiple tiny cysts measuring 0.2 to 0.8 cm diameter. Microscopy showed features of dysplasia in the form of primitive ducts surrounded by immature mesenchyme. Pancreas was firm and showed cysts grossly, ranging in size from 0.4 to 1.3 cm (Figure 3). Microscopy revealed predominant areas of immature mesenchyme surrounding the cysts which were lined by bland low cuboidal lining (Figure 4). Although reduced in number, foci of islets were present (Figure 4, inset). Liver displayed marked extra-medullary hematopoesis and focal mesenchymal dysplasia in the portal tracts. In addition, lungs showed dilated lymphatic channels in the pleural tissue and also in the inter-lobar and lobular fissure (Figure 5). Blood group of mother was 'O' and Rh positive.


Renal-hepatic-pancreatic dysplasia syndrome (Ivemark's syndrome).

Vankalakunti M, Gupta K, Kakkar N, Das A - Diagn Pathol (2007)

Photomicrography of pancreas shows widely spaced ducts lined by undifferentiated cells with loss of acini (H&E ×100). Inset showing islet cells (H&E ×400).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC1919354&req=5

Figure 4: Photomicrography of pancreas shows widely spaced ducts lined by undifferentiated cells with loss of acini (H&E ×100). Inset showing islet cells (H&E ×400).
Mentions: At autopsy, the baby weighed 1.4 Kg with crown-to-heel length of 39 cm, foot length of 6 cm. There was congenital talipes equino-varus deformity. Serous effusions were noticed. Kidneys were normal sized and showed multiple tiny cysts measuring 0.2 to 0.8 cm diameter. Microscopy showed features of dysplasia in the form of primitive ducts surrounded by immature mesenchyme. Pancreas was firm and showed cysts grossly, ranging in size from 0.4 to 1.3 cm (Figure 3). Microscopy revealed predominant areas of immature mesenchyme surrounding the cysts which were lined by bland low cuboidal lining (Figure 4). Although reduced in number, foci of islets were present (Figure 4, inset). Liver displayed marked extra-medullary hematopoesis and focal mesenchymal dysplasia in the portal tracts. In addition, lungs showed dilated lymphatic channels in the pleural tissue and also in the inter-lobar and lobular fissure (Figure 5). Blood group of mother was 'O' and Rh positive.

Bottom Line: Both cases are unrelated and there is no history of any consanguineous marriage.These two cases are unrelated and are rare.In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. vkmahesh123@yahoo.co.in

ABSTRACT

Background: Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.

Case presentation: We describe two unrelated cases of Renal-Hepatic-Pancreatic dysplasia syndrome in stillborn babies. The characteristic microscopic features were present in both the cases. The second case illustrates the unique association lymphangiectasia with Renal-Hepatic-Pancreatic dysplasia syndrome. Both cases are unrelated and there is no history of any consanguineous marriage.

Conclusion: These two cases are unrelated and are rare. In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.

No MeSH data available.


Related in: MedlinePlus