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.

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.

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.

[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.

[An atypical case of Marcus Gunn's syndrome]. / Un cas atypique de syndrome de Marcus Gunn.

The authors examine a clinical case of the Marcus Gunn phenomenon, characterized by the lid retraction when the patient closes her mouth. The peculiarity of this clinical case would consist in a partial synkinesis involving the masseter and temporal muscles.

[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.

[Hypothyroxinemia in the low birth-weight infant in the screening of congenital hypothyroidism]. / L'ipotiroxinemia del neonato di basso peso nello screening dell'ipotiroidismo congenito.

Inside a pilot screening program for Congenital Hypothyroidism, T4 and TSH have been tested in sick and healty preteam and fullterm low birth weight (LBW) newborns during the first two months of life, 36 newborns affected by respiratory distress syndrome and 15 by sepsis have been included in the study. Blood samples were collected by heel puncture on 3rd, 10th, 20th, 40th and, in some cases, up to 60th day of life, and adsorbed on filter paper. Our findings show that hypothyroxinaemia in LBW newborns is strictly related to gestational age. In fact, among preterm infants with GA less than or equal to 33 weeks, 25 subjects (69,44%) showed T4 levels less than or equal to 6 micrograms/dl and 5 infants (13,88%) had T4 concentrations less than or equal to 2 micrograms/dl. The incidence of subjects with T4 values less than or equal to 6 micrograms/dl falls to 42,18% in the group of infants with GA = 34-36 weeks and to 17,27% in the group of fullterm LBW infants. None of these newborns showed thyroxine levels less than or equal to 2 micrograms/dl. All the examined infants showed normal TSH levels. The low T4 values may appear soon after birth or later (3rd-20th day of life) and sometimes persist up to 40th or 60th day, despite of always normal TSH levels. The mean of low T4 values at each sampling time is strictly and directly related to gestational age. (ABSTRACT TRUNCATED AT 250 WORDS)

[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%.

[Surgical correction of esotropias with unstable angles (author's transl)]. / Die chirurgische Korrektur der Esotropien mit instabilem Winkel (retroäquatoriale Myopexie).

The authors report on the surgical procedure for retroequatorial myopexis, with and without rearward relocation. Analysis of the surgical procedure for retroequatorial myopexis of the rectus lateralis and medialis with and without rearward relocation: 1) simple myopexis - anchoring the muscle on the sclera 14 mm from the insertion of the rectus medialis (19 mm in the case of the rectus lateralis); 2) combined procedure (myopexis and rearward relocation). Forty patients with esotropia and unstable angle, as well as clear signs of a blockage, underwent surgery. In 47% of the patients sensoric orthophoria was achieved, in 43% a significant reduction of the angle; in 5% treatment resulted in overcorrection and in 5% the angle was unchanged.

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.

Congenital obstruction of the lacrimal passages in five consecutive generations.

Congenital epiphora due to absence or imperfection of lacrimal puncta and/or canaliculi was present in 21 members from five consecutive generations of a Sicilian family. The anomaly was transmitted as an autosomal-dominant characteristic with apparently complete penetrance and highly variable expressivity.

[Intrafamilial variation in congenital ophthalmoplegia: studies in a Sicilian family]. / Variabilite intrafamiliale de l'ophtalmoplegie externe congenitale: etude d'une famille sicilienne.

Nine members of a Sicilian family, in four successive generations, showed a congenital eye defect of much variable severity, from simple blepharoptosis to complete external ophthalmoplegia, without other ocular or extraocular abnormalities. The anomaly in this pedigree was transmitted as an autosomal dominant character with incomplete penetrance and extremely variable expressivity.