Infección por citomegalovirus como causa de diarrea grave rebelde en un lactante inmunocompetente / Cytomegalovirus infection causing protracted diarrhea in an immunocompetent child

La infección por citomegalovirus (CMV) es una causa frecuente de complicaciones en pacientes inmunodeprimidos. En huéspedes sanos, la infección por CMV cursa generalmente de forma asintomática. A continuación se presenta el caso de un paciente de 3 meses de edad inmunocompetente, en el que se desarrolló un cuadro de diarrea grave que precisó nutrición parenteral. El cuadro se resolvió sin secuelas tras tratamiento con ganciclovir (AU)

Cytomegalovirus infection is a frequent cause of complications in immunodeficient patients. In healthy hosts, cytomegalovirus infection is usually asymptomatic. We describe a case of a 3 month old immunocompetent boy who suffered from protracted diarrhoea that required parenteral nutrition. After treatment with ganciclovir he recovered without any after effects (AU)

[Cytomegalovirus infection causing protracted diarrhea in an immunocompetent child]. / Infección por citomegalovirus como causa de diarrea grave rebelde en un lactante inmunocompetente.

Cytomegalovirus infection is a frequent cause of complications in immunodeficient patients. In healthy hosts, cytomegalovirus infection is usually asymptomatic. We describe a case of a 3 month old immunocompetent boy who suffered from protracted diarrhoea that required parenteral nutrition. After treatment with ganciclovir he recovered without any after effects.

Hepatectomía laparoscópica de lesión hiperplásica hepática / No disponible

No disponible

[Lymphoproliferative disorders of Waldeyer's ring]. / Linfomas primarios del anillo de Waldeyer.

OBJECTIVE: To investigate the incidence and characteristics of lymphoproliferative disorders of Waldeyer's ring in our pediatric patients. MATERIAL AND METHODS: We retrospectively reviewed 20 children under 14 years of age who underwent surgery in our department for adenoidectomy and/or tonsillectomy between 1 January 1996 and 30 November 2000. In non-immunocompromised children, surgical indication was the recent development of progressive unilateral tonsillar hyperplasia, and in immunocompromised patients indication was mainly the enlargement of adenoids and/or tonsils, although eradication of local Ebstein-Barr virus infection and recurrent acute tonsillitis were also indications. RESULTS: The mean age was 4.6+/-2.3 years (range: 16.9 months-13.9 years). Sixteen patients (80.0 %) were male. In the nine patients with unilateral hyperplasia, histopathological diagnosis was diffuse lymphoid hyperplasia. Of the 11 immunocompromised patients, 5 (45.5 %) had some type of lymphoproliferative disorder. DISCUSSION AND CONCLUSIONS: Lymphoproliferative disorders of Waldeyer's ring are relatively frequent in immunocompromised children who have undergone surgery of the adenoids and/or tonsils. Although no cases of unilateral hyperplasia of the tonsils were diagnosed in our series, tonsillectomy is indicated in patients with this diagnosis, independent of their immunological status.

Linfomas primarios del anillo de Waldeyer / Lymphoproliferative disorders of waldeyer's ring

Objetivo: Investigar la incidencia y características de los procesos linfoproliferativos del anillo de Waldeyer en pacientes pediátricos. Material y métodos: Se recogieron retrospectivamente 20 pacientes menores de 14 años intervenidos en nuestro servicio de adenoidectomía y/o amigdalectomía entre el 1 de enero de 1996 y el 30 de noviembre de 2000. La indicación en niños no inmunodeprimidos fue la aparición de una hiperplasia unilateral reciente y progresiva de amígdala y en el caso de inmunodeprimidos fue principalmente el crecimiento adeno y/o amigdalar, aunque también se intervinieron para erradicar infección focal por el virus de Epstein-Barr y por amigdalitis de repetición. Resultados: La media de edad fue de 4,6 ± 2,3 años (límites, 16,9 meses-13,9 años). Del total de los pacientes de nuestra serie, 16 fueron varones (80,0 %). El diagnóstico anatomopatológico de los 9 pacientes con hiperplasia unilateral de amígdala fue hiperplasia folicular linfoide. Por otro lado, en 5 de los 11 sujetos con inmunodeficiencias (45,5 %) se evidenció algún tipo de proceso linfoproliferativo. Discusión y conclusiones: Los procesos linfoproliferativos del anillo de Waldeyer son relativamente frecuentes en niños inmunodeprimidos intervenidos de adenoides y/o amígdalas. Aunque no se diagnosticó ningún caso en nuestra serie, consideramos conveniente operar a sujetos pediátricos con hiperplasia unilateral de amígdala, con independencia de su estado inmunológico (AU)

Crohn's disease limited to the appendix.

BACKGROUND: Crohn's disease confined to the vermiform appendix is rare. In our study, the incidence was 0.2% of all patients diagnosed with Crohn's disease at La Paz University Hospital, Madrid, Spain, in 20 years. METHODS: Here we review the clinical records of 10 patients with isolated appendiceal Crohn's disease. RESULTS: Preoperative diagnosis was acute appendicitis in all 10 cases, and all patients underwent appendectomy. Postoperative complications were limited to an enterocutaneous fistula in 1 patient. There was no evidence of recurrence during a mean follow-up period of 14.5 years (range 2 to 25 year). CONCLUSIONS: We conclude that Crohn's disease when confined to the appendix is less aggressive than in other sections of the intestine, with a low recurrence rate and incidence of postoperative fistula.

Melanoma anorrectal: aportación de 10 casos y revisión de la bibliografía / Anorectal melanoma: a report of 10 cases and review of the literature

Introducción. El melanoma es la neoplasia maligna más agresiva y menos frecuente del segmento anorrectal, entre el 1 y el 2 por ciento de todos los tumores de la región. El objetivo de este estudio ha sido revisar los casos de melanoma anorrectal atendidos en nuestro hospital. Pacientes y métodos. Estudio retrospectivo de los 10 pacientes con melanoma anal tratados entre 1966 y 1998. Resultados. La rectorragia fue el síntoma más frecuente, seguida por la proctalgia y el tenesmo. En todos los pacientes se realizó rectocolonoscopia y biopsia de la lesión. En el momento del diagnóstico 3 pacientes presentaban metástasis a distancia. Todos fueron intervenidos quirúrgicamente, realizándose extirpación local en seis y amputación abdominoperineal en cuatro. El tamaño medio del tumor fue de 4 cm (rango, 3-6 cm). La supervivencia media fue de 11 meses. Conclusiones. El melanoma anorrectal es un tumor infrecuente que presenta una clínica similar a la de otras lesiones benignas o malignas del canal anal, lo que dificulta y retrasa su diagnóstico. El tratamiento es fundamentalmente quirúrgico, no aportando beneficios ni la quimioterapia ni la radioterapia. La supervivencia depende del tamaño y del estadio de la enfermedad en el momento del diagnóstico, siendo la detección precoz de esta lesión el único factor que puede mejorar el pronóstico (AU)

Malignant stromal tumors of the stomach.

BACKGROUND/AIMS: The purpose of this paper is to expose our experience in malignant stromal tumors of the stomach. METHODOLOGY: We present 16 cases of malignant stromal tumors of the stomach, operated on in our hospital from 1977 to 1995. Age, sex, symptoms and signs, standard laboratory tests, diagnostic methods, location, size, infiltration of other organs, proliferating activity, treatment and survival rate are analyzed. Immune staining was performed using the avidin-biotin-peroxidase method, with the S-100 1/8000 and muscular actin 1/50 antiserum. All patients underwent surgical treatment, and neither chemotherapy nor radiotherapy were used. RESULTS: Thirteen patients died 5 to 78 months after surgery, and the other 3 are alive and free of disease 74, 48 and 38 months after resection, respectively. Five year survival rate was 37.5%. The most important prognostic factors were tumor size, infiltration of neighboring organs and high mitotic index. CONCLUSION: All the clinical data, survival rates and prognostic factors are similar to other published cases. Surgery is the first step in the treatment of these tumors, as the role of other therapeutic options is not well known yet.

[Association of cytomegalovirus colitis and the 1st episode of ulcerative colitis in an immunocompetent patient]. / Associación de colitis por citomegalovirus y primer brote de colitis ulcerosa en un paciente immunocompetente.

Colitis by cytomegalovirus (CMV) is an inflammation of the large bowel which leads to mucous and submucous ulceration producing bloody diarrhea of uncertain evolution. This disease is well known in immunosuppressed (IS) patients but is very rare in immunocompetent (IC) patients, particularly in association with the initiation of the ulcerative colitis (UC). The case of an immunocompetent male who developed UC following colitis by primary CMV is presented. Although the immunologic mechanism of UC is undoubtable, this case suggests the possibility of CMV being a triggering factor of UC.

[Histological lesions and Helicobacter pylori colonization in children]. / Lesión histológica y colonización por Helicobacter pylori en el niño.

We have assessed the relationship between histologic findings and the Helicobacter pylori (HP) colonization in 115 patients (age: 9.8 +/- 3.5 years) with HP infection. Normal antral histology was found in 8.7% of the patients. Signs of histological activity were observed in 58.2% of the patients and glandular lesions in 3.5%. A significantly greater HP colonization was observed in patients with signs of histological activity (p < 0.001). A significant correlation was found between the HP colonization score and histological score (rs = 0.62) with a significant association between the degree of HP colonization and the histologic categories (p < 0.001).

[Colloidal bismuth subcitrate and amoxicillin in the treatment of Helicobacter pylori infection in children]. / Tratamiento con subcitrato de bismuto coloidal más amoxicilina en la infección por Helicobacter pylori del niño.

We have analyzed the effect of treatment with colloidal bismuth subcitrate (CBS) and amoxicillin in a series of 60 children (age range: 2.9-18 years) infected by Helicobacter pylori (HP). The following results were found: clinical remission 70%; endoscopic normalization 55%; histological normalization 61.6% and HP eradication 53.3%. The improvement of histological activity (100% vs 11.7%) and histological normalization (90.6% vs 28.5%) were significantly more frequent in the HP eradicated group as compared with the persistent HP group (p < 0.001). The present study suggests that combined therapy with CBS and amoxicillin is not the most adequate treatment to eradicate HP in children and supports the role played by HP in the development of antral histologic lesions.

[Carcinoma of the Vater's ampulla and colonic carcinoma with synchronous onset]. / Carcinoma de ampolla de Vater y carcinoma colónico de aparición sincrónica.

We report the case of a female patient who presented with a very unusual association: an early invasive adenocarcinoma of the ampulla of Vater and a synchronous carcinoma of the right colon, in the absence of familial polyposis coli. Six years before a hysterectomy was performed because of uterine leiomyomata. The treatment was a Whipple's resection and, after three months, a right colectomy.

[Cystic mucinous tumor of the pancreas . A new case]. / Tumores quísticos mucinosos de páncreas. Un nuevo caso.

The case of a woman with a mucinous cystadenoma of the pancreas is presented. Caudal pancreatectomy was performed. Post-operative course was uneventful.

[The current state of Niemann-Pick disease: evaluation of six cases]. / Estado actual de la enfermedad de Niemann-Pick: valoración de seis casos.

Six pediatric patients with Niemann-Pick disease are reported. They have been studied at Hepatology Unit HI "La Paz" (Madrid) in the period of time between 1975-1988. They are one case of type A, one case of type B and four cases of type C. This group of pediatric patients serve us to make a revision of the disease attending to clinical and biochemical classification aspects, diagnosis and treatment. We insist on two aspects: 1) greater importance of enzymatic diagnose for the "Niemann-Pick complex", in view of the overlapping of clinical symptoms, 2) bone-marrow transplantation as therapeutic alternative and its indications.

[Viral infection and obstructive cholangiography in children]. / Infección vírica y colangiopatía obstructiva infantil.

In the last years it has been suggested that idiopathic obstructive cholangiopathy is a pathologic process with evolutive studies: extrahepatic biliary atresia and neonatal hepatitis. The possibility of a viral agent as the inciting factor has been investigated. This report shows four male and one female with confirmed idiopathic obstructive cholangiopathy with different clinical picture. There was evidence of cytomegalovirus infection in two patients, rubella infection in one patient, hepatitis B virus infection in one patient and Epstein-Barr virus infection in one patient. While other causes of neonatal cholestasis were excluded, we suggest that those viruses has an etiologic role in the initial injury and the progression of the disease.

[Alpha 1-antitrypsin deficiency with histologic expression of metabolic disease of carbohydrates]. / Déficit de alpha-1-antitripsina con expresividad histológica de enfermedad metabólica de hidratos de carbono.

A two months old male affected by alpha-1-AT PiZZ deficiency with severe transient neonatal cholestasis is presented. Two hepatic biopsies were practiced in neonatal period. There was no evidence of PAS positive globules, but an intense univacuolar steatosis and a rossetoid transformation of hepatocytes were observed. Both findings are identical to those found in the histopathologic study of the liver in certain metabolic diseases such as fructosemia and galactosemia. A third biopsy practiced at an age of two years confirmed diagnosis of alpha-1-AT deficit since presence of PAS positive globules was established. It must be pointed out that histopathological findings show great variability among different patients with alpha-1-AT deficit in the neonatal period, as well as the infrequent presence of PAS positive globules in hepatic biopsies of those c during the first months of life.

[Localized giant pseudopolyposis in Crohn disease]. / Pseudopoliposis gigante localizada en la enfermedad de Crohn.

A case is presented of Crohn's disease of prolonged evolution in a 32-year-old male, with clinical manifestations of intermittent obstruction due to localized giant pseudopolyposis in the colon. The bibliography is reviewed, anatomophathologic aspects are analyzed and the problems of differential diagnosis are remarked.