Liver infiltrating mononuclear cells in children with type 1 autoimmune hepatitis.

OBJECTIVE: To investigate infiltrating cells in the liver of children with type 1 autoimmune hepatitis (AH-1). METHODS: liver biopsies from 24 untreated AH-1 patients (14 children, 10 adults), five patients with hepatitis C virus related chronic hepatitis (HCV), and 10 control liver specimens (CL) were processed for immunohistochemical cell characterisation. RESULTS: Two different cell distribution patterns were detected in the liver of patients with AH-1: (1) CD4(+) and CD20(+) cells were found in the central areas of the portal tracts (portal distribution); (2) CD8(+) cells were observed at the periphery of the portal space (periportal distribution). Some cell subsets, like CD56, CD57, Fas-L, and Bak, showed a non-defined distribution pattern. The presence of two well defined patterns of cell distribution was not observed in HCV and CL (CD4(+), CD20(+), and CD8(+) cells were uniformly distributed in the portal space). In AH-1 and CL, the NK markers CD56 and CD57 were found scattered throughout the liver parenchyma. However, in HCV biopsies, CD56(+) cells were also clearly increased in both the portal and the periportal areas. Biopsies of AH-1 and HCV patients showed a uniform distribution of Fas-L and Bak in the portal and periportal areas, with Bak staining also detected in the hepatic parenchyma. CONCLUSIONS: Despite clinical and genetic differences, there was a similar distribution of liver infiltrating mononuclear cells in children and adults with AH-1. These results raise the possibility of reclassifying cryptogenic chronic hepatitis by immunohistochemical analysis of infiltrating liver cells.

[Barrett's esophagus: diagnosis and coexistent diseases in childhood]. / Esófago de Barrett: diagnóstico y enfermedades coexistentes en la edad pediátrica.

From 1990 to 1998, 12 patients with columnar metaplasia of the lower esophagus were diagnosed. Only 4 of them displayed a Barrett's epithelium (BE) with a specialized columnar epithelium and globet cells. As it is already published, a male predominance was observed. In three of these patients some predisposing factor to develop BE was detected (i.e. severe central nervous system damage, chronic pulmonary disease, esophageal atresia and chemotherapy). All patients had severe gastroesophageal reflux (GER) with abnormal pH probe. Diagnosis was suggested by characteristic endoscopy images in 2 patients and was confirmed by biopsy in all cases. All patients had been primary treated with proton-bomb inhibitors. Two patients were treated by a Nissen fundoplication, 4 and 6 month after diagnosis, respectively. One patient with severe neurological damage will undergo the same surgery soon. In another patient with caustic esophageal injury, the affected esophagus will be resected, restoring continuity with stomach or colon. In view of the potential oncogenous transformation of BE, the importance of not overlooking this anomaly in all patients with severe GER is highlighted. All cases with predisposing factors to develop BE should be closely followed by periodic examination and multiple biopsies.

Immunohistochemical evaluation of hepatoblastomas with use of the hepatocyte-specific marker, hepatocyte paraffin 1, and the polyclonal anti-carcinoembryonic antigen.

The distinction of hepatoblastoma, especially the embryonal type, from other small, round-cell tumors of childhood can sometimes be difficult. Polyclonal anticarcinoembryonic antigen (pCEA) and Hepatocyte Paraffin 1 (Hep Par 1) are immunohistochemical markers that are useful in the diagnosis of hepatocellular carcinomas. We immunohistochemically studied pCEA, monoclonal CEA (mCEA), and Hep Par 1 on 12 hepatoblastomas (3 fetal type, 2 embryonal type, and 7 mixed epithelial type). In addition, we studied the expression of Hep Par 1 on 27 other selected childhood tumors, including 1 hepatocellular carcinoma, 5 germ-cell tumors, 4 peripheral neuroectodermal tumors/Ewing's sarcomas, 3 rhabdomyosarcomas, 5 neuroblastomas, 2 rhabdoid tumors, 3 lymphomas, and 4 Wilms' tumors. All of the hepatoblastomas expressed Hep Par 1 with a characteristic granular intracytoplasmic pattern that was generally less intense in embryonal-type than in fetal-type hepatoblastomas, perhaps reflecting the degree of hepatocyte differentiation. All of the fetal-type hepatoblastomas expressed pCEA with both an intracytoplasmic and bile canalicular pattern. Embryonal type hepatoblastomas were more likely to be pCEA negative or to show focal or no canalicular pattern of expression, again possibly reflecting the degree of hepatocyte differentiation. All of the hepatoblastomas were mCEA negative. All of the nonhepatoblastomas were Hep Par 1 negative, except for the one hepatocellular carcinoma in this study, which was Hep Par 1 positive. We conclude that Hep Par 1 and pCEA are useful markers for hepatoblastomas, as they have been shown to be in hepatocellular carcinomas.

[Histologic factors as elements of prognostic value in biliary atresia]. / Factores histológicos como elemento de valor pronóstico en atresia de vías biliares.

Biliary atresia (BA) is one of the biliary tree anomaly more frequent. Occurs in about 0.8 to 1/10.000 live births. BA is defined as a progressive biliary tree. The prognosis depends on the age of the diagnosis and precocity surgery. We present the results of a retrospective analysis of 71 RA carried out at the Garrahan Hospital from 1987 to 1993. 47 were female and 24 were male. Age ranged from 45 to 120 days of life. This study involved a consecutive series of 58 patients with histopathologic study of Porta-hepatis (PH) and liver biopsy obtained during the Kasai. The purpose of this study was to determine the value of histological factors as type of PH and hepatocytic giant cell transformation (GCT). 82.8% had favorable type of PH and the CCT was mild in 84.5%. 72.4% had bad outcome and was independent of the type of PH. Neither of them were statistically significant with survive and evolution. In our service neither PH non CGT were predictors of a bad outcome. There were good outcome in 27.5%, died 37.9% and 10.3% undergo liver transplantation. The precocity in a diagnosis and surgical procedure before two months of age are the most important factors in correlation with survival. Others immunomorphologic factors must be studied in BA that explained the etiopathogenic process. Orthotopic liver transplantation is the successful therapy in children with BA.

La biopsia rectal en el diagnóstico de la enfermedad de Hirschsprung : su confiabilidad / Rectal biopsy for diagnosis of Hirschsprung's disease: it's reliability

Entre 1987 y 1992 fueron atendidos 88 pacientes con sospecha de enfermedad de Hirschsprung(EH). Las edades fueron de un día a 14 años, 68 varones y 20 mujeres, con una incidencia familias de 8,9 por ciento y 5,3 por ciento de malformaciones asociadas. Los síntomas fueron constipación (100 por ciento ), oclusión intestinal (14,5 por ciento ), enterocolitis (10,8 por ciento ) y peforación intestinal(5,4 por ciento ).En este período se realizaron 94 biopsias rectales por succión, de las cuales 33 descartaron EH y lo confirmaron en 61. De estos 61 pacientes, 58 fueron diagnosticados en la primera biopsia y 3 requirieron una segunda biopsia.El número de secciones histológicas fue de 50 a 80 cortes por especimen y teñidas con hematoxilina y eosina. En aislados casos realizamos técnica de inmunoperoxidasa para proteína S100 y enolasaneurono específica.En todas se visualizaron filetes nerviosos hepertróficos y ausencia de células ganglionares a nivel submucoso.En 6 pacientes de los 33 en que se descartó EH se realizó biopsia seromuscular por laparotomía ante la persistencia de la sintomatología.Los diagnósticos fueron: miopatía visceral (4 casos) ,ganglioneuromatosis (1 caso),Chagas (1 caso).Esta metodología mostró una sensibilidad del 95 por ciento ,especificidad del 96 por ciento , un valor predictivo positivo del 98,3 por ciento y un valor predictivo negativo del 100 por ciento . Todos los casos de EH fueron confirmados en los especímenes quirúrgicos.Los resultados obtenidos con esta técnica nos motiva a recomendar el empleo de la biopsia rectal por succión con cortes semiseriados y destacar su bajo costo,confiabilidad y eficacia diagnóstica.

La biopsia rectal en el diagnóstico de la enfermedad de Hirschsprung : su confiabilidad / Rectal biopsy for diagnosis of Hirschsprungs disease: its reliability

Entre 1987 y 1992 fueron atendidos 88 pacientes con sospecha de enfermedad de Hirschsprung(EH). Las edades fueron de un día a 14 años, 68 varones y 20 mujeres, con una incidencia familias de 8,9 por ciento y 5,3 por ciento de malformaciones asociadas. Los síntomas fueron constipación (100 por ciento ), oclusión intestinal (14,5 por ciento ), enterocolitis (10,8 por ciento ) y peforación intestinal(5,4 por ciento ).En este período se realizaron 94 biopsias rectales por succión, de las cuales 33 descartaron EH y lo confirmaron en 61. De estos 61 pacientes, 58 fueron diagnosticados en la primera biopsia y 3 requirieron una segunda biopsia.El número de secciones histológicas fue de 50 a 80 cortes por especimen y teñidas con hematoxilina y eosina. En aislados casos realizamos técnica de inmunoperoxidasa para proteína S100 y enolasaneurono específica.En todas se visualizaron filetes nerviosos hepertróficos y ausencia de células ganglionares a nivel submucoso.En 6 pacientes de los 33 en que se descartó EH se realizó biopsia seromuscular por laparotomía ante la persistencia de la sintomatología.Los diagnósticos fueron: miopatía visceral (4 casos) ,ganglioneuromatosis (1 caso),Chagas (1 caso).Esta metodología mostró una sensibilidad del 95 por ciento ,especificidad del 96 por ciento , un valor predictivo positivo del 98,3 por ciento y un valor predictivo negativo del 100 por ciento . Todos los casos de EH fueron confirmados en los especímenes quirúrgicos.Los resultados obtenidos con esta técnica nos motiva a recomendar el empleo de la biopsia rectal por succión con cortes semiseriados y destacar su bajo costo,confiabilidad y eficacia diagnóstica.

[Mitotic index of intraepithelial lymphocytes in children with celiac disease. Retrospective study]. / Indice mitótico de linfocitos intraepiteliales en niños con enfermedad celíaca. Estudio retrospectivo.

The mitotic activity of epithelial lymphocytes was determined in 69 jejunal biopsies performed in 23 children with celiac disease. The mitotic index (MI) exceeded 0.2% in 46 biopsies obtained in untreated celiac and during acute gluten challenge. In the 23 biopsies made during the period of gluten restriction the mitotic index fell down under 0.2%. This retrospective study confirm the reproducibility of the method proposed by Marsh. The high mitotic index in the first biopsy may be useful to reduce in the future the multiple biopsies currently necessary for the diagnosing celiac disease.

Indice mitótico de linfocitos intraepiteliales en niños con enfermedad celíaca. Estudio retrospectivo / [Mitotic index of intraepithelial lymphocytes in children with celiac disease. Retrospective study]

The mitotic activity of epithelial lymphocytes was determined in 69 jejunal biopsies performed in 23 children with celiac disease. The mitotic index (MI) exceeded 0.2

in 46 biopsies obtained in untreated celiac and during acute gluten challenge. In the 23 biopsies made during the period of gluten restriction the mitotic index fell down under 0.2

. This retrospective study confirm the reproducibility of the method proposed by Marsh. The high mitotic index in the first biopsy may be useful to reduce in the future the multiple biopsies currently necessary for the diagnosing celiac disease.

Indice mitótico de linfocitos intraepiteliales en niños con enfermedad celíaca. Estudio retrospectivo. / [Mitotic index of intraepithelial lymphocytes in children with celiac disease. Retrospective study]

The mitotic activity of epithelial lymphocytes was determined in 69 jejunal biopsies performed in 23 children with celiac disease. The mitotic index (MI) exceeded 0.2

in 46 biopsies obtained in untreated celiac and during acute gluten challenge. In the 23 biopsies made during the period of gluten restriction the mitotic index fell down under 0.2

. This retrospective study confirm the reproducibility of the method proposed by Marsh. The high mitotic index in the first biopsy may be useful to reduce in the future the multiple biopsies currently necessary for the diagnosing celiac disease.

[Intestinal polyps in childhood]. / Polipos intestinales en la infancia.

From 1965 to 1987, 230 polypectomies were performed in patients between 8 month to 16 years old, 147 boys and 83 girls. The most frequent symptom was rectal bleeding and the most frequent location was the rectosigmoid (96%). The polyps measured from 0.8 to 2 cm., in only one case the polyp measured more than 5 cm. causing duodenal obstruction and it was associated with a gastric polyp. Solitary polyps represented 93.9% of all cases, Peutz-Jeghers syndrome 1.73 and juvenile polyposis 0.87%. In that period 3 cases of colonic carcinoma in children over 9 years old were diagnosed. The histology of 200 polyps were reviewed. In 21 cases the diagnosis was modified (10.5%). The juvenile polyp (J.P.) was the most frequent type (93%). The usual histopathological pattern was observed in 71.5% of the J.P., early lesions (26.3%) and hyperplastic changes (2.1%). Six cases were tubular adenoma and one tubulovillous. The multiple juvenile polyposis was found in girls (13 and 14 years old). None of the patients with Peutz-Jeghers syndrome had gonadal tumor. Endoscopic polypectomy in children is a safe and effective treatment for colorectal polyps. This study provides information on the clinicopathologic findings in argentinian children with intestinal polyps treated by polypectomy over a 22 year period. It emphasizes that juvenile polyps is the most frequent pattern however other lesions can be present in gastrointestinal tract and also associated with others tumors. Patients with polyps should be followed at regular intervals which can be modified according to the histologic type and family history.

Polipos intestinales en la infancia. / [Intestinal polyps in childhood]

From 1965 to 1987, 230 polypectomies were performed in patients between 8 month to 16 years old, 147 boys and 83 girls. The most frequent symptom was rectal bleeding and the most frequent location was the rectosigmoid (96

). The polyps measured from 0.8 to 2 cm., in only one case the polyp measured more than 5 cm. causing duodenal obstruction and it was associated with a gastric polyp. Solitary polyps represented 93.9

of all cases, Peutz-Jeghers syndrome 1.73 and juvenile polyposis 0.87

. In that period 3 cases of colonic carcinoma in children over 9 years old were diagnosed. The histology of 200 polyps were reviewed. In 21 cases the diagnosis was modified (10.5

). The juvenile polyp (J.P.) was the most frequent type (93

). The usual histopathological pattern was observed in 71.5

of the J.P., early lesions (26.3

) and hyperplastic changes (2.1

). Six cases were tubular adenoma and one tubulovillous. The multiple juvenile polyposis was found in girls (13 and 14 years old). None of the patients with Peutz-Jeghers syndrome had gonadal tumor. Endoscopic polypectomy in children is a safe and effective treatment for colorectal polyps. This study provides information on the clinicopathologic findings in argentinian children with intestinal polyps treated by polypectomy over a 22 year period. It emphasizes that juvenile polyps is the most frequent pattern however other lesions can be present in gastrointestinal tract and also associated with others tumors. Patients with polyps should be followed at regular intervals which can be modified according to the histologic type and family history.

Fibrolamellar carcinoma of the liver in a child: ultrastructural and immunohistologic aspects.

We describe a fibrolamellar carcinoma of the liver in a 12-year-old girl and review the literature concerning the occurrence of this distinct hepatic neoplasm in children up to the age of 16 years. The results of our electron microscopic and immunohistologic studies suggest that some cells of this tumor exhibit neuroendocrine differentiation. This finding has important theoretical implications relative to the tumor's histogenesis and practical implications with respect to the search for tumor markers of clinical usefulness and possibly for therapy.

Glandular schwannoma in a 17-month-old child.

Glandular schwannoma is an infrequent tumor, especially in the pediatric age group. We report a case in which the tumor developed in the retroperitoneum of a 17-month-old girl without stigmata of Von Recklinghausen's disease (VRD). She presented a local recurrence and bone and pulmonary metastases at 11, 23, and 30 months, respectively, after tumor resection. The pathologic findings in the original tumor did not permit us to predict its potential to metastasize. The ultrastructural characteristics of both glandular and schwannian elements are described. The rarity of this neoplasia in children and the absence of histological parameters of aggressiveness are emphasized.