Clinical, radiologic, and endoscopic characteristics upon diagnosis of patients with prehepatic portal hypertension at the Instituto Nacional de Pediatría from 2001 to 2011.

BACKGROUND: Prehepatic portal hypertension in children can be asymptomatic for many years. Once diagnosed, the therapeutic measures (pharmacologic, endoscopic, and surgical) are conditioned by the specific characteristics of each patient. In Mexico, there are no recorded data on the incidence of the disease and patient characteristics. AIMS: To determine the main clinical, radiologic, and endoscopic characteristics upon diagnosis of these patients at the Instituto Nacional de Pediatría within the time frame of January 2001 and December 2011. METHODS: A cross-sectional, retrolective, descriptive, and observational study was conducted in which all the medical records of the patients with portal hypertension diagnosis were reviewed. RESULTS: There was a greater prevalence of prehepatic etiology (32/52) (61.5%) in the portal hypertension cases reviewed. Males (62.5%) predominated and 11 of the 32 patients were under 4 years of age. The primary reason for medical consultation was upper digestive tract bleeding with anemia (71.9%) and the main pathology was cavernomatous degeneration of the portal vein (65.6%). Splenoportography was carried out on 17 of the 32 patients. A total of 65.5% of the patients received the combination therapy of propranolol and a proton pump inhibitor. Initial endoscopy revealed esophageal varices in 96.9% of the patients, 12 of whom presented with gastroesophageal varices. Congestive gastropathy was found in 75% of the patients. The varices were ligated in 8 cases, sclerotherapy for esophageal varices was carried out in 5 cases (15.6%), and sclerotherapy for gastric varices was performed in 2 patients. Seventeen patients (53.1%) underwent portosystemic diversion: 10 of the procedures employed a mesocaval shunt and 7 a splenorenal shunt. Nine patients (28.1%) underwent total splenectomy. CONCLUSIONS: The primary cause of the disease was cavernomatous degeneration of the portal vein; it was predominant in males and the first symptom was variceal bleeding.

[Generalized microsporidiosis caused by Encephalitozoon sp. in a pediatric patient with Bruton's disease]. / Microsporidiosis generalizada por Encephalitozoon sp. en un paciente pediátrico con enfermedad de Bruton.

We present the case of a four-year-old boy with a history of repeated upper respiratory tract infections and pyoderma. He presented fever, seizures, inability to talk, loss of swallowing, fine tremor in the upper extremities; positive bilateral Babinski reflex and quadriparesis. The diagnosis of Bruton's disease and generalized microporidiosis was based on immunologic analysis, smear tests with chromotrope R2 stain and indirect immunofluorescense with monoclonal 3B6 antibody for Encephalitozoon species in samples of spinal fluid, bronchial and paranasal sinus aspirates and stool, which were all positive. The patient was treated with albendazol during 72 days; he left the hospital in a good condition, walking, talking and able to swallow. His laboratory test controls were negative; he is followed up in the outpatient department.

[Meckel's diverticulum. Study of 61 cases]. / Divertículo de Meckel. Estudio de 61 casos.

BACKGROUND: Meckel's diverticulum is a well known disease in children. The main clinical presentations are rectal bleeding and intestinal occlusion. OBJECTIVE: To investigate the frequency, main clinical symptoms, signs, complications, localization, histology and diagnosis in a population of mexican children. METHOD: We studied 61 children who were seen at the Instituto Nacional de Pediatria, Mexico City, during a period of 10 years. The age, sex, symptoms, complications, hematocrit, 99mTc-Pertechnetate scanning, histology, and localization of Meckel's diverticulum were analyzed in all of them. RESULTS: 15 children were female and 46 male (1:3). In infants the main clinical finding was rectal bleeding. Intestinal occlusion was the most common clinical expression in older children. Gastric mucosa was a common finding in the diverticulum of children with rectal bleeding in contrast to ileal mucosa in children with intestinal occlusion. 99mTc-pertechnetate scanning with previous administration of H2 blocking agent was the best diagnostic procedure. CONCLUSIONS: Meckel's diverticulum must be considered an emergency in children. Meckel's diverticulum, has to be ruled cut as in infants with significant bleeding, as well as in children with intestinal occlusion. The best method for diagnosis is 99mTc-pertechnetate scanning.

Esclerosis hepato-portal: 10 anos de experiencia el el Instituto Nacional de Pediatria / Hepatoportal sclerosis. 10 years of experience in the National Institute of Pediatrics

Objetive: To assess the frecuency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City. Background: Hepatoportal Sclerosis is a disease of unknown etiology. It's diagnosis is difficut. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts. Methods: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specifical findings of splenoportography and histologic changes in liver biopsy. Results: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenisc, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotheraphy. At the time of this report all children have shown a good clinical course.

Esclerosis hepato-portal: 10 anos de experiencia el el Instituto Nacional de Pediatria / Hepatoportal sclerosis. 10 years of experience in the National Institute of Pediatrics

Objetive: To assess the frecuency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City. Background: Hepatoportal Sclerosis is a disease of unknown etiology. Its diagnosis is difficut. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts. Methods: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specifical findings of splenoportography and histologic changes in liver biopsy. Results: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenisc, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotheraphy. At the time of this report all children have shown a good clinical course. (AU)

[Hepatoportal sclerosis. 10 years of experience at the National Institute of Pediatrics]. / Esclerosis hepato-portal. 10 años de experiencia en el Instituto Nacional de Pediatría.

OBJECTIVE: To assess the frequency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City. BACKGROUND: Hepatoportal Sclerosis is a disease of unknown etiology. It's diagnosis is difficult. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts. METHODS: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specific findings of splenoportography and histologic changes in liver biopsy. RESULTS: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenic, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotherapy. At the time of this report all children have shown a good clinical course.

[Shwachman's syndrome]. / Síndrome de Shwachman.

BACKGROUND: Shwachman syndrome has been recognized as the second most frequent cause of pancreatic insufficiency in children and its is related to hematological and bony abnormalities. AIMS: To know the main clinical symptoms, analytical data, radiological and sonographic findings in the Shwachman syndrome. MATERIALS AND METHODS: We present a 1 year 4 months old girl with Shwachman syndrome. We reviewed the clinical history taking into consideration height/weight relationship according to percentiles and physical examination. Analytical data: blood counts, fetal hemoglobin, vitamin B12 and folate levels, bone marrow examination, stool cultures and examination for ova and parasites, transaminase levels, sweat electrolytes, serologic tests for viral hepatitis. Radiological studies: bone series and bone age, abdominal sonography and computed tomography. Liver and intestinal biopsies. RESULTS: In this patient we confirmed the presence of exocrine pancreatic insufficiency, dysfunction of the bone marrow and bony abnormalities typical in Shwachman syndrome.

[Congenital hepatic fibrosis. Study of 26 cases]. / Fibrosis hepática congénita. Estudio de 26 casos.

We studied 26 children with congenital hepatic fibrosis during the period 1971-1993. About half of the children were about the 6 years old. Only two had brothers with same disease. The chief clinical manifestation was hematemesis associated or not with liver enlargement, predominantly of left lobe. Only one case showed fever and cholangitis. Liver function tests were usually normal. Twenty-two children had portal hypertension. Liver biopsy was of definitive for diagnosis. Seven children died.

Fibrosis hepática congénita. Estudio de 26 casos. / [Congenital hepatic fibrosis. Study of 26 cases]

We studied 26 children with congenital hepatic fibrosis during the period 1971-1993. About half of the children were about the 6 years old. Only two had brothers with same disease. The chief clinical manifestation was hematemesis associated or not with liver enlargement, predominantly of left lobe. Only one case showed fever and cholangitis. Liver function tests were usually normal. Twenty-two children had portal hypertension. Liver biopsy was of definitive for diagnosis. Seven children died.

[Peptic acid disease associated with Helicobacter pylori in children. Report of a case]. / Enfermedad ácido péptica asociada a Helicobacter pylori en niños. Reporte de un caso.

We studied a child with peptic acid disease which was associated with H. pylori. The identification of H. pylori was done by biopsy and for antibody test. Clinical improvement was observed only with the use of amoxocilin and Bismuth subsalycilate.

[Intestinal arteriovenous malformations in children]. / Malformaciones arteriovenosas del intestino en niños.

Four children with arteriovenous malformation were studied during a two year period (1989-1991). The main localization of these arteriovenous malformations were: sigmoid rectum, ascending colon, terminal ileum and apoendix. The diagnosis was made with colonoscopy and arteriography. The surgical resection was the procedure of choice.

[Ultrasonographic findings in hepatoblastoma]. / Hallazgos ultrasonográficos en hepatoblastoma.

At de National Institute of Pediatrics in Mexico City, the ultrasonographic findings of hepatoblastomas were analyzed in order to establish the echographic pattern of this malignant liver tumor. The ultrasonic images of patient's livers dating back six years were retrospectively reviewed. The hyperrecognisance of the hepatoblastoma as a solid, poorly limited mass, with or without the presence of calcifications, with the displacement of the portal vein and frequently found in the right lobe, were considered as the most relevant echographic characteristics of the hepatoblastoma. The echographic characteristics found of the hepatoblastoma can aid in the sonographic diagnosis of the disease. The leukemic liver infiltrations, the primary liver lymphoma and the metastasis, due to their hyperrecognisance, can be confused with the diagnosis of hepatoblastoma.

[Pancreatic pseudocyst. A case report and review of the literature]. / Pseudoquiste pancreático. Informe de un caso y revisión de la literatura.

This is the case of a 15 year old adolescent girl who refers recurrent chronic abdominal pain for a period of three months. She was diagnosed as having a chronic pancreatic pseudocyst, seen as a complication of an episode of hemorrhage acute pancreatitis secondary to the administration of L-asparaginase for the treatment of acute lymphoblastic leukemia. The abdominal ultrasonography allowed for a pre-operatory diagnosis to be made. An internal drainage and a cystogastrostomy were the procedures of choice. A review of the literature is included on the physiopathology, clinical history, diagnostic procedures and therapeutic conduct to be followed.

[Epiploon cysts. Report of 2 cases]. / Quistes de epiplón. Informe de dos casos.

This report includes two cases of omental cysts, with detailed clinical and radiological findings. The main clinical finding in these patients was a massive cystic growth, producing an increase in abdominal volume undistinguishable from ascites. In both cases the preoperative diagnosis was done using abdominal ultrasonography. Although omental cysts are infrequent, it should be thought of as a possibility in the differential diagnosis in children with intraabdominal fluid. Omental cysts; intraabdominal cystic masses; abdominal ultrasound.

[Rectosigmoidal angiodysplasia]. / Angiodisplasia de rectosigmoides.

A case of a three year old child with an angiodysplasia (arterial venous malformation) localized in the sigmoidal rectum is reported. The malformation caused chronic recurrent rectal hemorrhaging. Colonoscopy was the procedure used to diagnose the problem which was later corroborated by arteriography. The affected area was removed.

[Intestinal malacoplakia in childhood. Report of 5 cases]. / Malacoplasia intestinal en la infancia. Informe de cinco casos.

We studied 5 children with intestinal malakoplakia who were seen during a period of 10 years. Four were male and one female. The main clinical manifestations were: chronic bloody and mucous diarrhea, abdominal pain and polypoid masses detected by rectal maneuver. Either the radiological study as well as endoscopy showed the presence of stenosis and polypoid masses (pseudopolyps). Two patients underwent laparotomy and a tumoral infiltration at the level of rectal-sigmoid and cecal appendix was found. Histological diagnosis proves to be definitive in these case. Drug treatment has been with bethanechol, trimethoprim-sulfamethoxazole in three patients. We conclude that intestinal malakoplakia is more common that ulcerative colitis in our children population attending at this institute. Therefore, the presence of intestinal malakoplakia must be ruled out in every child having chronic bloody and mucous diarrhea, presenting as well pseudopolyps.