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Research paper thumbnail of Congenital Dyserythropoietic Anemia: An Etiopathological Study

Journal of evolution of medical and dental sciences, Dec 30, 2015

To evaluate the role of blood and bone marrow findings in the diagnosis of congenital dyserythrop... more To evaluate the role of blood and bone marrow findings in the diagnosis of congenital dyserythropoietic anaemias type I and type II. SETTINGS AND DESIGN Dyserythropoietic anaemia is suspected when there is a suboptimal reticulocyte response for the degree of anaemia. Congenital dyserythropoetic anaemias are a group of rare hereditary disorders of hematopoiesis. It is believed that CDA is often under diagnosed. Knowledge of CDA workup in a patient of haemolytic anaemia is often rewarding. MATERIALS AND METHODS All the cases diagnosed as CDA at a tertiary paediatric centre were reviewed with respect to clinical and serological data, peripheral smear and bone marrow study findings. The haematological changes in CDA were analyzed with respect to the diagnosis. RESULTS AND CONCLUSION The light microscopic findings of the peripheral smear and bone marrow aspirate were found to be highly specific for the diagnosis of CDA. Significant differences were also noted between type I and type II CDA.

Research paper thumbnail of Infantile Hemangioendothelioma of Liver: Case Report

Journal of evolution of medical and dental sciences, Sep 3, 2015

Infantile Hemangioendothelioma of the liver is an uncommon tumor. Two subtypes have been describe... more Infantile Hemangioendothelioma of the liver is an uncommon tumor. Two subtypes have been described. Type1 has recognizable but irregularly dilated vascular spaces in a fibromyxomatous stroma. Type 2 is described as having a more aggressive histology. It is marked by poorly formed and anatomising channels with papillae lined by atypical endothelial cells and is potentially malignant. Clinical presentation is variable, hepatomegaly or abdominal mass, some hematological abnormalities may be seen. In laboratory investigation, or lesion may be asymptomatic and discovered incidentally. We report a case of 45 day old female child who presented with abdominal distension. CT abdomen showed hypoattenuating mass in the left lobe of the liver along with specks of calcification.. The mass was excised and had large areas of gritty calcification simulating a teratoma. however histopathological diagnosis of infantile hemangioendothelioma type I of liver was made. Hemangioendothelioma of the liver has been reported as most common benign symptomatic vascular tumor of the liver in infancy.

Research paper thumbnail of Congenital Dyserythropoietic Anemia, Bone Marrow

Research paper thumbnail of Efficacy of the seven feature, fifteen point histological scoring system and CD56 in interpretation of liver biopsies in persistent neonatal cholestasis: a five-year study

Indian journal of pathology & microbiology

Neonatal cholestasis (NC) lasting more than 2 weeks affects one in 2500 live births. Extrahepatic... more Neonatal cholestasis (NC) lasting more than 2 weeks affects one in 2500 live births. Extrahepatic biliary atresia (EHBA) and idiopathic neonatal hepatitis account for about 70% of all cases of NC. Differentiating these two conditions is important as patient management is very different for both the conditions. To assess the usefulness of the seven-feature, 15-point histological scoring system in the interpretation of liver biopsy in NC and usefulness of immunostaining with CD56 (N-CAM) in EHBA. Retrospective study of 5 years' duration at a pediatric referral institute, where the case load of NC is high and definitive surgery for EHBA is undertaken after histological confirmation. The study is of a 5-year duration conducted between June 2007 and May 2012. A total of 210 cases of NC were clinically diagnosed during this period. All the slides were reviewed with reference to a seven-feature, 15-point histological scoring system assessing its usefulness in the interpretation of live...

Research paper thumbnail of Thoracic fetus in fetu

Journal of Indian Association of Pediatric Surgeons, 2012

A rare case of thoracic fetus in fetu is reported. Complete excision was curative.

Research paper thumbnail of CLE, Histopathology, Bronchial Cartilage, RAC

Research paper thumbnail of Congenital Lobar Emphysema, a Histopathological Study

Journal of Evolution of medical and Dental Sciences, 2015

Congenital lobar emphysema is a very rare congenital cystic malformation of the lung that can cau... more Congenital lobar emphysema is a very rare congenital cystic malformation of the lung that can cause acute respiratory distress early in life. This study reviews 19 cases of CLE over a period of 5 years at a Tertiary Pediatric Referral Center. The cases were studied with special emphasis on Histopathological findings of the lobectomy specimens, particularly bronchial cartilage abnormalties, radial alveolar count and their predictive value in pathogenesis of CLE. This condition can mimick other causes of respiratory distress in the neonatal period. Early diagnosis and effective surgical management is curatve.

Research paper thumbnail of Hemangioendothelioma, Liver, Vascular Tumor, Infant, Teratoma

Research paper thumbnail of Calretinin immunohistochemistry: A new cost-effective and easy method for diagnosis of Hirschsprung′s disease

Journal of Indian Association of Pediatric Surgeons, 2013

aganglionic HD colon biopsy specimens and correlate with the H and E, thereby exploring its utili... more aganglionic HD colon biopsy specimens and correlate with the H and E, thereby exploring its utility in suspicious cases of HD. MATERIALS AND METHODS Our standard protocol of care for a child presenting with HD is barium enema study at presentation followed by a laparotomy where multiple biopsies are taken from the spastic aganglionic segment, transition zone, and the normal colon. A colostomy is sited at the level of the junction between the normal colon and the transition zone. The definitive surgery of choice is Duhamel's pull-through after 6 months of age. The aim of this study was to evaluate the efficacy of calretinin immunostaining [5,7] in the ganglionic and

Research paper thumbnail of Congenital Dyserythropoietic Anemia: An Etiopathological Study

Journal of evolution of medical and dental sciences, Dec 30, 2015

To evaluate the role of blood and bone marrow findings in the diagnosis of congenital dyserythrop... more To evaluate the role of blood and bone marrow findings in the diagnosis of congenital dyserythropoietic anaemias type I and type II. SETTINGS AND DESIGN Dyserythropoietic anaemia is suspected when there is a suboptimal reticulocyte response for the degree of anaemia. Congenital dyserythropoetic anaemias are a group of rare hereditary disorders of hematopoiesis. It is believed that CDA is often under diagnosed. Knowledge of CDA workup in a patient of haemolytic anaemia is often rewarding. MATERIALS AND METHODS All the cases diagnosed as CDA at a tertiary paediatric centre were reviewed with respect to clinical and serological data, peripheral smear and bone marrow study findings. The haematological changes in CDA were analyzed with respect to the diagnosis. RESULTS AND CONCLUSION The light microscopic findings of the peripheral smear and bone marrow aspirate were found to be highly specific for the diagnosis of CDA. Significant differences were also noted between type I and type II CDA.

Research paper thumbnail of Infantile Hemangioendothelioma of Liver: Case Report

Journal of evolution of medical and dental sciences, Sep 3, 2015

Infantile Hemangioendothelioma of the liver is an uncommon tumor. Two subtypes have been describe... more Infantile Hemangioendothelioma of the liver is an uncommon tumor. Two subtypes have been described. Type1 has recognizable but irregularly dilated vascular spaces in a fibromyxomatous stroma. Type 2 is described as having a more aggressive histology. It is marked by poorly formed and anatomising channels with papillae lined by atypical endothelial cells and is potentially malignant. Clinical presentation is variable, hepatomegaly or abdominal mass, some hematological abnormalities may be seen. In laboratory investigation, or lesion may be asymptomatic and discovered incidentally. We report a case of 45 day old female child who presented with abdominal distension. CT abdomen showed hypoattenuating mass in the left lobe of the liver along with specks of calcification.. The mass was excised and had large areas of gritty calcification simulating a teratoma. however histopathological diagnosis of infantile hemangioendothelioma type I of liver was made. Hemangioendothelioma of the liver has been reported as most common benign symptomatic vascular tumor of the liver in infancy.

Research paper thumbnail of Congenital Dyserythropoietic Anemia, Bone Marrow

Research paper thumbnail of Efficacy of the seven feature, fifteen point histological scoring system and CD56 in interpretation of liver biopsies in persistent neonatal cholestasis: a five-year study

Indian journal of pathology & microbiology

Neonatal cholestasis (NC) lasting more than 2 weeks affects one in 2500 live births. Extrahepatic... more Neonatal cholestasis (NC) lasting more than 2 weeks affects one in 2500 live births. Extrahepatic biliary atresia (EHBA) and idiopathic neonatal hepatitis account for about 70% of all cases of NC. Differentiating these two conditions is important as patient management is very different for both the conditions. To assess the usefulness of the seven-feature, 15-point histological scoring system in the interpretation of liver biopsy in NC and usefulness of immunostaining with CD56 (N-CAM) in EHBA. Retrospective study of 5 years' duration at a pediatric referral institute, where the case load of NC is high and definitive surgery for EHBA is undertaken after histological confirmation. The study is of a 5-year duration conducted between June 2007 and May 2012. A total of 210 cases of NC were clinically diagnosed during this period. All the slides were reviewed with reference to a seven-feature, 15-point histological scoring system assessing its usefulness in the interpretation of live...

Research paper thumbnail of Thoracic fetus in fetu

Journal of Indian Association of Pediatric Surgeons, 2012

A rare case of thoracic fetus in fetu is reported. Complete excision was curative.

Research paper thumbnail of CLE, Histopathology, Bronchial Cartilage, RAC

Research paper thumbnail of Congenital Lobar Emphysema, a Histopathological Study

Journal of Evolution of medical and Dental Sciences, 2015

Congenital lobar emphysema is a very rare congenital cystic malformation of the lung that can cau... more Congenital lobar emphysema is a very rare congenital cystic malformation of the lung that can cause acute respiratory distress early in life. This study reviews 19 cases of CLE over a period of 5 years at a Tertiary Pediatric Referral Center. The cases were studied with special emphasis on Histopathological findings of the lobectomy specimens, particularly bronchial cartilage abnormalties, radial alveolar count and their predictive value in pathogenesis of CLE. This condition can mimick other causes of respiratory distress in the neonatal period. Early diagnosis and effective surgical management is curatve.

Research paper thumbnail of Hemangioendothelioma, Liver, Vascular Tumor, Infant, Teratoma

Research paper thumbnail of Calretinin immunohistochemistry: A new cost-effective and easy method for diagnosis of Hirschsprung′s disease

Journal of Indian Association of Pediatric Surgeons, 2013

aganglionic HD colon biopsy specimens and correlate with the H and E, thereby exploring its utili... more aganglionic HD colon biopsy specimens and correlate with the H and E, thereby exploring its utility in suspicious cases of HD. MATERIALS AND METHODS Our standard protocol of care for a child presenting with HD is barium enema study at presentation followed by a laparotomy where multiple biopsies are taken from the spastic aganglionic segment, transition zone, and the normal colon. A colostomy is sited at the level of the junction between the normal colon and the transition zone. The definitive surgery of choice is Duhamel's pull-through after 6 months of age. The aim of this study was to evaluate the efficacy of calretinin immunostaining [5,7] in the ganglionic and