Primary Prophylaxis for Gastrointestinal Bleeding in Children With Biliary Atresia and Portal Hypertension Candidates for Liver Transplantation: A Single-Center Experience.

BACKGROUND: Cirrhosis for biliary atresia (BA) is associated with risk of gastrointestinal bleeding (GB) from gastroesophageal varices due to portal hypertension. Primary prophylaxis of GB is controversial in children who are candidates for liver transplantation (LT). The aim of the study was to define the management of gastroesophageal varices and to identify the benefit of primary prophylaxis for GB in BA children waiting for LT. METHODS: A retrospective single-center study including all BA children listed for LT in 2008-2016. Clinical, endoscopical, and biochemical data were analyzed. RESULTS: Of 82 children, 50 (61%) did not receive primary prophylaxis and did not present any episode of bleeding, 16 (19.5%) underwent primary prophylaxis, and 16 (19.5%) presented spontaneous GB and received secondary prophylaxis. Children without primary prophylaxis and GB were younger than patients with primary prophylaxis and those with GB (7.7 years [range, 4.1-37.9 years] vs 11.2 years [range, 5.1-43 years]; P = .03 vs 10.7 years [range, 6.9-39.9 years], respectively; P = .004). Seventy-five percent of GB occurred in children older than 8 months. Fifteen (93.8%) children with GB presented esophageal varices (grade III = 10 [62.5%]) and 10 (62.5%) required endoscopic treatments, consisting mainly of sclerotherapy. Median time to LT was similar for children with or without bleeding (2 months [range, 0-17.7 months] vs 2.2 months [0-17.9 months], respectively; P = .89). After 45.5 months (range, 13.7-105.5 months) of follow-up, the overall patient survival was 97.6%. At the intention-to-treat analysis, the survival rate was 100% for patients without bleeding episode and 87.5% for children with GB (P = .16). CONCLUSIONS: Despite the risk of GB being not clinically predictable in children with BA waiting for LT, our experience suggests that primary prophylaxis of GB might be unnecessary in children younger than 6 months, while it should be considered in older children. Thus, the occurrence of GB does not delay the timing of transplantation.

Tumores de la cavidad oral y huesos maxilares en pediatría. Revisión de 122 pacientes / Oral cavity and maxillary bone tumors in pediatrics. Review of 122 patients

Se presenta un estudio retrospectivo de 122 pacientes con tumores que se manifestaron en la cavidad oral, con localización inicial en maxilares o partes blandas (se excluyeron los tumores de la cara sin compromiso bucal). La edad promedio fue 9 años y 6 meses (rango de 1 día a 17 años). El 62 por ciento se presentó en varones. La localización inicial de los tumores fue en hueso en el 53 por ciento de los casos y en partes blandas en el 47 por ciento; 82 pacientes tuvieron lesiones benignas y 40 lesiones malignas. Las manifestaciones al ingreso fueron: tumor palpable o visible (39 por ciento), tumor más dolor (22 por ciento), dolor (19 por ciento) y otros como caída de dientes, parálisis, fiebre o asímetría facial (20 por ciento). La rutina de estudio comprendió radiografía panorámica de maxilar, centellografía ósea (gammacámara con Tecnesio 99), tomografía axial computada (TAC) y resonancia nuclear magnética (RNM). Los pacientes fueron tratados en forma multidisciplinaria siendo la cirugía (punción aspiración con aguja fina, biopsia y/o resección)el procedimiento inicial en la mayoría de ellos. De acuerdo al algoritmo todos los pacientes con lesión ósea fueron estudiados con Rx simple y TAC, 89 por ciento de positividad en ambas, previas a la biopsia por punción. De igual menera en los tumores de partes blandas la TAC mantuvo su utilidad, no así la Rx simple que fue reeplazada por la ecografía cuando se detectó ausencia de compromiso óseo. Las lesiones benignas predominaron (78/122) a nivel de hueso o de partes blandas. la curación en ella fue la regla. En lo que respecta a los tumores, primarios de la región (11/40) correspondieron inicialmente a partes blandas y raramente a hueso, en los que fue frecuente el compromiso metastático o multicéntrico.

Patología tumoral de ovario en edad pediátrica: experiencia del Hospital de Niños "Superiora Sor María Ludovica" / Pediatric ovarian tumors

Se evalúan 44 pacientes ingresadas en el período comprendido entre 1984-2001 con patología ovárica. El 79,5 por ciento (n=35) correspondieron a tumores y el 20,5 por ciento (n=9) a lesiones quísticas. Dentro del primer grupo 31/35 correspondieron a tumores germinales y 16/31 fueron teratomas maduros. Luego de evaluación clínica, imaginológica y de marcadores tumorales en aquellos con marcadores positivos y contenido mixto o sólido se realizó Punción Aspiración con Aguja Fina (PAAF) para corroborar diagnóstico. La conducta inicial fue la cirugía en 32/34 tumores y en 9/9 lesiones quísticas no tumorales. El 67 por ciento de las últimas ingresó con cuadro abdominal agudo por lo que recibió cirugía de urgencia. De obtenerse el diagnóstico sin cuadro de abdomen agudo y con marcadores tumorales negativos se utilizó la vía laparoscópica en 3 pacientes (2 quistes simples y 1 teratoma maduro). De las 15 pacientes con tumores germinales malignos 11 recibieron quimioterapia de acuerdo a protocolos SIOP (consecutivos por el tiempo de estudio) y 2 recibieron radioterapia luego de haberse diagnosticado disgerminoma. Sólo 2 pacientes, por considerarse los tumores irresecables al ingreso, recibieron quimioterapia preoperatoria. La sobrevida global fue del 94.5 por ciento (33/35) y la sobrevida libre de reactivaciones del 90 por ciento (31/35). Las dos pacientes fallecidas correspondieron al período inicial del estudio (1984-5) ingresando con diagnóstico tardío. Cinco pacientes (34 por ciento) de las portadoras de tumores malignos y que recibieron quimioterapia se han casado y tres de ellas tienen actualmente 5 hijos sanos que se controlan en nuestro Hospital

Neuroblastoma IV-S. Cual es el mejor tratamiento? Experiencia en 12 casos / Neuroblastoma IV-S. Which is the best treatment?

El neuroblastoma IV-S tiene muy buen pronostico. Sin embargo puede haber una evolucion mortal debido al curso natural de la enfermedad o, mas frecuentemente, por complicaciones del tratamiento. Decidir cual es el tratamiento de eleccion es una tarea dificil. La cirugia del tumor primario es un punto controvertido

Retroperitoneal and deep-seated lipoblastoma: diagnosis by CT scan and fine-needle aspiration biopsy.

Lipoblastomas are most commonly superficially-located tumors. Our experience with three examples of deep-seated lipoblastomas is reported. The CT scans of all cases showed low-attenuation densities (less than 0 Hansfield units), strongly suggesting fatty composition. Fine-needle aspiration biopsy (FNAB) smears contained uni- and multivacuolated lipoblasts, myxoid areas, and a plexiform capillary network. The combination of CT scan and FNAB findings seems to be enough for definitive preoperative diagnosis of lipoblastoma, allowing differential diagnosis with other deep-seated tumors.

[Asymptomatic cholelithiasis: indications for cholecystectomy based on the levels of acute phase proteins]. / La litiasi colecistica asintomatica: guida alla colecistectomia dai valori delle proteine della fase acuta.

Prophylactic cholecystectomy for asymptomatic gallstones is still controversial. Aim of the study was to assess whether the determination of serum acute phase proteins (APP) could be utilized as a criterion for cholecystectomy, as they are suggestive of the presence in the blood of cytokines released from the inflamed gallbladder wall, even when clinical signs are missing. In 75 cases of gallstones, free from other coexistent inflammatory processes, red cell sedimentation rate, plasmatic cortisol, immunoglobulin (IgA, IgG, IgM), electrophoresis of the proteins, CPR, fibrinogen, haptoglobin, alfa-1-antitrypsin and bile culture have been detected. The patients have been subdivided into two groups: patients with asymptomatic or mildly symptomatic disease and patients with a clear clinical pattern of acute cholecystitis. In the latter alfa-1-globulin, alfa-2-globulin, ESR, CPR and cortisol turned out to be significantly elevated, while in 20-30% of the former CPR, beta-globulin and cortisol were increased, too. The study demonstrates that among the patients with asymptomatic gallstones there is a population having PFA values higher than normal. This is suggestive of a cytokines activation which, when other inflammatory processes can be excluded, is likely due to gallbladder inflammation and surgery will likely be indicated.

Rhabdomyosarcoma of extrahepatic biliary tree: initial treatment with chemotherapy and conservative surgery.

BACKGROUND: The very low frequency of rhabdomyosarcoma (RMS) of the extrahepatic biliary tree has impeded the development of a standardized form of treatment. PROCEDURE: Based on the good response of embryonal RMS to chemotherapy, we used a multi-drug protocol as the initial treatment of a 3-year-old girl after obtaining adequate transparietohepatic biliary drainage. The treatment achieved complete remission. Later conservative laparoscopic surgery revealed only residual scar tissue. The patient is alive and well 1 year after remission. DISCUSSION AND CONCLUSIONS: Management of this difficult tumor using modern less invasive techniques for diagnosis and treatment, with the help of preoperative chemotherapy, prevented extensive damage to the biliary tree and allowed complete recovery.

Cytologic characteristics of peripheral neuroectodermal tumors in fine-needle aspiration smears: a retrospective study of three pediatric cases.

Cytologic diagnosis of peripheral neuroectodermal tumors (PNT) on fine-needle aspiration (FNA) smears represents a challenge to the cytopathologist. Usually ancillary studies are used to achieve definitive diagnosis. We retrospectively examined FNA material from three cases of PNT with the aim of identifying their features. Positive and negative cytologic findings were recognized. Positive features for PNT included the presence of: rather uniform appearance of the cells, which display scant but almost always-present perinuclear clear cytoplasm (suggesting a bland epithelial tumor); nuclei with distinctively smooth nuclear membrane contour, finely granular chromatin, and one or two small nucleoli (suggesting neuroendocrine anlage); and organization of the cells singly or in cohesive clusters. Negative findings included the absence of: frequent mitotic figures, large nucleoli, nuclear pleomorphism, cellular debris, histiocytes, and polymorphonuclear leucocytes. The smears appeared clean, with small, uniform cells having features suggesting a neuroendocrine epithelial tumor. These findings may prove useful for accurate cytologic diagnosis and differentiation of PNT from other small blue round cell tumours (SBRCT) of soft tissues without the use of ancillary studies since, when properly evaluated, cytomorphology of the latter group of tumors is more heterogeneous than generally believed.

[Nonocclusive intestinal infarct and necrotizing enterocolitis in the adult]. / Infarto intestinale non occlusivo ed enterocolite necrotizzante dell'adulto.

A case of full-thickness necrosis of the small bowel, and colon, which required partial resection of the jejunum and total resection of the ileum and colon is reported. The case gives the chance for a review of the Literature on intestinal necrosis not caused by vascular occlusion. Nonocclusive intestinal ischemia, acute neonatal necrotizing enterocolitis and adult necrotizing enterocolitis including the Pig-bel disease, common in Papua-New Guinea, are examined. Resemblances and differences in etiology, pathophysiology and clinical findings are discussed. The hypothesis that the process of "bacterial translocation" plays a central role in the pathogenesis of bowel infarction, representing therefore a possible link between infective and vascular mechanisms, is emphasized. Important suggestions on massive intestinal necrosis management are also reported.

[Carcinomas of the right colon. A review of 60 consecutive cases]. / I carcinomi del colon destro. Revisione di 60 casi consecutivi.

Sixty consecutive cases of tumor of the right colon operated upon between 1981 and 1991 are reviewed. Age and sex distribution, clinical findings, preoperative diagnostics and system used staging, TNM classification, surgical management, operative mortality, follow up and survival were analyzed. Results are discussed and compared with published data. In the second of the two 5-year period considered an increased incidence of right-sided carcinoma compared with tumors of the left colon and rectum was observed. According to the TNM classification, more than 50% of patients were in stage III. Operative mortality was 5%. The overall 5-year survival rate was 58%, while the survival rate of patients treated with radical intent was 70%.

[Spontaneous pneumothorax. The therapeutic directions based on 117 cases]. / Il pneumotorace spontaneo. Orientamenti terapeutici sulla base di 117 casi.

117 cases of spontaneous pneumothorax are reviewed. The authors report their experience and consider surgical drain to be the best method in most cases to achieve a rapid recovery. Surgical management or conservative approach can also to be used in selected patients.

[Stoma complications. Experience of a care center for enterostomal patients]. / Complicanze delle stomie. Esperienza di un centro di assistenza per enterostomizzati.

Personal experience in an enterostomy centre over a 5-year period is report. The problems deriving from the presence of a stoma are examined with particular emphasis on surgical complications which are grouped into early and late forms.

[Celiac-mesenteric insufficiency. Apropos of a case of aorto-hepatic bypass]. / L'insufficienza celiaco-mesenterica. A proposito di un caso di by-pass aorto-epatico.

The Authors report a case of coeliac axis atheromatous obstruction, with superior mesenteric artery steal. The coeliac axis, stopped up the origin was indirectly revascularized by hepatic artery. The early diagnosis of chronic intestinal ischaemia should avoid the intestinal infarction. The development of intravenous digital angiography will certainly help an early diagnosis.

[Acute cholecystitis. Early diagnostic study using cholescintigraphy with Tc99m-IDA]. / La colecistite acuta. Indagine diagnostica precoce con la colescintigrafia con IDA-99mTc.

34 patients admitted for suspected acute cholecystitis were evaluated using 99mTc IDA cholescintigraphy. The results of these studies are reviewed and compared with other diagnostic tests and the subsequent clinical diagnosis. Cholescintigraphy proved to be a safe, simple, highly accurate and sensitive technique. Therefore, 99mTc-IDA cholescintigraphy is proposed as the initial procedure of choice in the evaluation of patients with suspected acute cholecystitis.

Autosomal dominant cone-rod dystrophy associated with sickle-cell trait in a Sicilian family.

Three Sicilian siblings had a typical cone-rod dystrophy. The same ocular anomaly was probably present in their mother and in the maternal grandmother. The three probands were also carriers of Hb S, but this last anomaly was also present in an unaffected brother. It is concluded that the cone-rod dystrophy of this family is inherited as an autosomal dominant character and that its association with sickle-cell trait is coincidental.