[Liver cirrhosis in childhood. Considerations on 22 cases with different etiology]. / La cirrosi epatica in età pediatrica. Considerazioni su ventidue casi a differente eziologia.

Although rather uncommon and multifactorial in etiology, liver cirrhosis is a severe and often rapidly fatal disease in pediatrics. In our institution, during the last 15 years, 22 children with liver cirrhosis have been followed. The underlying predisposing condition was HBV infection (8 cases), CMV perinatal infection (2 cases), Wilson's disease (4 cases), chronic cholestasis (2 cases) and alcohol abuse (2 cases); in 4 cases no predisposing condition was evident. In all cases the histological examination of the liver was the diagnostic cornerstone. The mean age at diagnosis was 6 years and 8 months, with an early onset especially in the posthepatitis cirrhosis. In 10 out of 22 patients, cirrhosis was not preceded by an history of chronic liver disease. Poor subjective symptomatology was present in 13 of the cases, hepatomegaly in all, splenomegaly in 18 cases, signs of hepatic failure in 13 cases. In all patients various impairments of hepatocellular synthesis were detectable, especially during the period preceding the development of hepatic insufficiency. The mean time to cirrhosis was 5 years. The average duration of the follow up was 3 years and 4 months: during the follow up 6 patients improved, 5 patients showed no clinical or functional modifications of their hepatic disease, 3 patients worsened and 8 died. In order to perform suitable treatment of liver cirrhosis the need of early diagnosis and etiological definition should be emphasized.

The Berlin Questionnaire for assessment of sleep disordered breathing risk in parturients and non-pregnant women.

BACKGROUND: Pregnancy is associated with alteration in sleep patterns and quality. We wished to investigate whether pregnant women have a higher likelihood of a positive Berlin Questionnaire than non-pregnant women. METHODS: Pregnant women ages 18-45 years (n=4074) presenting for delivery, and non-pregnant women ages 18-45 years (n=490) presenting for outpatient surgery provided demographic information and completed the Berlin Questionnaire evaluating self-reported snoring and daytime sleepiness. For the pregnant patients, the infant's birth weight and Apgar scores were also recorded. RESULTS: Of the 1439 patients with a positive Berlin Questionnaire, 96 were in the non-pregnant control population versus 1343 in the pregnant population (20% vs. 33%, respectively, P<0.001; odds ratio 2.0 [95% CI: 1.6-2.5]). There was a positive correlation between infant weight and a positive Berlin Questionnaire. The incidence of preeclampsia was greater (odds ratio 3.9) in the pregnant patients with a positive Berlin Questionnaire as compared with the parturients with a negative Berlin Questionnaire (odds ratio 1.1). CONCLUSION: Parturients are more likely to have a positive Berlin Questionnaire than non-pregnant women. This may indicate an increased likelihood of sleep disordered breathing.

[The role of liver in muscular dystrophy]. / Il ruolo del fegato nella distrofia muscolare.

Epidemiological or anamnestical data may either help or confuse the differential diagnosis of various diseases mainly characterized by asymptomatic hypertransaminasemia. Occasional finding of transaminase elevation may lead to suppose chronic or persistent hepatopathy, particularly when the patient seems to be asymptomatic and presents anamnestic data suggesting intoxication, acquired infection from blood derivatives, origin from geographic areas with high prevalence of viral hepatitis. However, the true existence of hepatic damage, concurrent to a myopathy, may be also related to the primitive diseases. There is evidence, in fact, that in the presence of muscular dystrophy, a disease caused by structural defects of muscular membranes, also hepatocytes show ultrastructural defects. The present work reports the cases of 5 children, hospitalized at the 1st Clinic of Infectious Diseases of the University of Genoa, affected by persistent hypertransaminasemia and showing anamnestical data suggesting hepatitis; histological findings of hepatitis were effectively shown in 3 patients after needle biopsy. All patients proved to be affected by muscular dystrophy. Hepatic damage results cannot be correlated to known causes of hepatopathy. During disease courses heralded by asthenia and hypertransaminasemia, differential diagnosis must take into account non-hepatic diseases, like muscular dystrophy. Although this disease mainly affects the muscle, also the liver seems to be involved, as suggested by histological changes found in some patients.

Hepatitis B virus (HBV)-DNA-positive hepatocellular carcinoma following hepatitis B virus infection in a child.

Integrated hepatitis B virus (HBV) DNA sequences were found in neoplastic liver tissue of a hepatitis B surface antigen (HBsAg)-negative child who had previously suffered from HBsAg-positive chronic active hepatitis and was anti-HBs and anti-hepatitis B core (HBc) positive at the time of tumor development. Reintegration pattern was consistent with the presence of a single integration site of the HBV genome into cellular DNA, and clonal proliferation of such infected cells. A normal liver, tested in the same experiment with the same amount of total DNA, was negative for viral DNA sequences. These findings support the possible oncogenic role of HBV in the development of liver cancer, not only in adults, but also in children, even in patients who are negative for HBsAg at the time of tumor diagnosis.

HBV-DNA-related hepatocellular carcinoma occurring in childhood. Report of three cases.

In a series of 325 HBV chronically infected children observed over an 18-year period, three developed HCC. These three children were born in southern Italy, a region characterized by a high endemic HBV infection rate; each had been infected perinatally, developed an acute hepatitis, and became a chronic carrier. Two of the three with cirrhosis were HBsAg positive at the time their HCC was detected. The remaining case had seroconverted to HBsAb but HBV-DNA integration could be demonstrated in the absence of cirrhosis; moreover HBV antigens were not expressed in the tissue of this case. The interval between HBV infection and HCC appearance in these three cases ranged from six to 11 years. A similarity between these three Italian cases and the majority of HCC arising in chronically infected children in the Far East is noted.

Treatment of children with chronic hepatitis B with a combination of steroids and human lymphoblastoid interferon.

The efficacy of human lymphoblastoid interferon (Wellferon) therapy was measured in 20 children with chronic hepatitis B with or without pretreatment with prednisolone. Patients were randomised to receive 0.6 mg/kg/day prednisolone for 3 weeks, then at 0.3 mg/kg for a fourth week or placebo. All patients then received interferon 5 MU/m2 i.m. for 12 weeks; daily for 5 days then three times a week for the remaining 11 weeks. Preliminary results show that 25% of children had a permanent loss of viral markers of replication. However, response to interferon varied widely between individuals and a prolonged follow-up is required in order to determine the influence of prednisolone pretreatment on the efficacy of interferon therapy.

[The pediatric emergency department: assessment of clinical activity and interaction with local health authorities]. / Il pronto soccorso pediatrico: valutazione dell'attività e interazione con i servizi territoriali.

A perspective study was carried out at the Emergency Department of the Gaslini Institute on a sample of the children admitted from May to September 1990. The study aimed to describe types and modalities of admission. "G. Gaslini" Institute is a multidisciplinary children's hospital: 45% of patients admitted in the hospital come from the southern regions of Italy, while the admission at the Emergency Department were local in 82%. Among all children considered, 2080 (71.9% were admitted on request of their parents, who did not ask previously any physician; of those cases, 356 (17%) were hospitalized, 474 (25%) were either examined by specialist or treated and then discharged. In the remaining cases, no intervention was necessary. As a consequence, 58% of children were admitted without any clinically plausible reason. Parents' anxiety clearly played an important role in most cases. 840 children were addressed to the Emergency Department by a physician, 652 (22.3%) by the doctor in charge, 213 (32.7%) of which were sent back home by the doctor on duty, who did not think it necessary any treatment. Of the 120 (4.1%) children sent by different Emergency Departments, 95 (79.1%) were hospitalized. The reason why physicians addressed quite a large number of children to the Emergency Department, when they didn't need any intervention remain nucleo. Their diagnosis, in fact, agreed with those of the doctor on duty in 83% of cases. In conclusion, it is difficult to imagine a diagnostic or therapeutic problem which needs specific structures. Cooperative programs with physicians working in our region will be carried out in order to control, where possible, admission to Emergency Departments.

[Reactivation of virus replication during immunosuppressive therapy in children with chronic hepatitis B]. / Riattivazione della replicazione virale in corso di terapia immunosoppressiva in bambini affetti da epatite cronica tipo B.

The aim of the present work is to assess whether reactivation of viral replication occurred in children affected by chronic hepatitis undergoing long term immunosuppressive therapy. 123 serum samples belonging to 25 children were retrospectively evaluated for HBV-DNA and HBeAg. Sera were collected prior to and during the protocol treatment (steroids alone or with azathioprine). Presence of HBV-DNA was evaluated by means of molecular hybridization technique, using a radiolabelled probe of cloned HBV-DNA. Sera (100 1) were denatured and transferred into nylon membrane (spot) then prehybridized and hybridized. After washing in stringent conditions, the filter was exposed in autoradiographic cassette with Kodak film XOmat5. Positivity was semiquantitatively evaluated by blackening of the spot. Increase or appearance of HBV-DNA was observed in the sera from 24/25 pts. HBeAg became positive in 4/5 pts previously negative. Obtained data shows reactivation of viral replication during immunosuppressive therapy. Data are especially significant in those cases in which such activity has apparently ceased or not been detected; HBV may be considered as a latent virus.

[The Gaslini Medical-Surgical Emergency Service. Organizational and functional aspects]. / Il Pronto Soccorso Medico-Chirurgico del Gaslini. Aspetti organizzativi e funzionali.

The Authors take into account the organization of the medical emergency service at Gaslini children's hospital. Emergency medicine has been developing as a pediatric subspecialty, involving medical surgical and intensive care units to meet the peculiar needs of the acutely ill child. Moreover epidemiological data regarding all kinds of activity have been collected; they show a decrease in admitted patients and an increase in outpatients. These data undertime how effectively the service can act as a filter as well.

Evaluation of ceftazidime in the treatment of 80 infectious episodes in compromised children.

The efficacy of ceftazidime in the treatment of infections in compromised children was evaluated in 80 such episodes occurring in 64 patients with various underlying diseases. Among the patients treated, 9 were newborns with severe neonatal distress, 21 were children with cancer and neutropenia, 8 were surgical patients, 22 had cystic fibrosis and 4 were suffering from meningitis. The following types of infections were treated: 19 bacteriologically documented and 8 possible septicemias (the latter only in newborns and neutropenic cancer patients); 2 severe upper respiratory tract infections in cancer patients; 8 soft tissue or skin infections; 1 cholangitis; 1 pneumonia; 1 osteomyelitis; 1 mediastinitis; 35 infectious exacerbations of underlying pulmonary disease in cystic fibrosis patients; and 4 meningitides. In almost all cases ceftazidime was administered intravenously in combination with an aminoglycoside. In 2 cases it was also given intrathecally or intraventricularly. Bacteriological documentation was achieved in 70 out of 80 episodes. A successful outcome was obtained in 79% of the cases with slight and statistically nonsignificant differences between groups of patients with different etiological patterns in terms of prevalence of gram-positive microorganisms. Tolerance of the treatment was uniformly good, only one patient showing a mild, transient transaminase elevation.

Dual-centre, double-blind, randomised trial of lymphoblastoid interferon alpha with or without steroid pretreatment in children with chronic hepatitis B.

Thirty-five children with chronic HBV infection, HBV-DNA and eAg serum positivity, and HBcAg in liver tissue were treated with lymphoblastoid human interferon alpha with (16 cases) or without (19 cases) prednisolone pretreatment. The patients were double-blind randomized to receive steroid or placebo for 4 weeks, followed after 2 weeks by 5 or 10 MU/m2 interferon for 12 weeks. The e anti-e seroconversion rate reached 48%, which is much higher than the spontaneous seroconversion rate. The influence of "prednisolone priming" was not statistically significant. HBeAg clearance was similar in both groups (44% after prednisolone/interferon and 53% after interferon alone). The response to either treatment did not correlate with the pretreatment serum transaminase. HBV-DNA or degree of histological activity. Interferon was well tolerated, the side effects being less severe than in adults, and never led to suspension of the treatment.

[The activities of a pediatric emergency department. The emergency problems]. / L'attività di un pronto soccorso pediatrico. Problemi emergenti.

The authors reviewed records of admission at the Fist Ais-Emergency Service of "G. Gaslini" Children's Hospital, data referring both to in and outpatients. First of all we took into account epidemiological data analysing occurrence and types of diseases; at the same time a demographic study, which aimed to show a decrease in the child population in Genova, was performed. Secondly we compared these data with the real number of admitted patients: collected data showed that this service has been used excessively.